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Sample records for intradural extramedullary schwannoma

  1. Cervical spine intradural-extramedullary hematoma presenting as ipsilateral hemiparesis

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    Chih Ming Lin

    2011-08-01

    Full Text Available A 75-year-old Taiwanese man suffered from acute onset of right-sided extremity weakness while talking to his neighbors. He was transferred to the hospital within three-hour time after symptom onset. Initial acute ischemic cerebral infarct was diagnosed based on his symptom and cerebral computed tomography. Thrombolytic therapy was held after his symptom improved promptly and could not excluded other etiology. Thorough history taking unraveled previous Chinese medicine clinic visit because of neck sore. However, he received limited improvement after several times of massage treatment. Magnetic resnance imaging (MRI of the cervical spine demonstrated hematoma compressing right side intradural-extramedullary space at the C2/C3 level. Through his clinical course, muscle weakness was the sole neurological finding with sparing of sensory defects. Given the close anatomy relationship between sensory and motor lamina distribution in the cervical spinal cord, our patient presented a rare manifestation. Cases of cervical spine intradural-extramedullary hematoma are not often seen and only sporadic in the documented literature. We wish, through the report of this article, to inform the first- line physicians with the following information. Among the elderly, neck sore is a common symptom. Over- stretching or overt local massage is not suggested due to relatively fragile musculature. In the clinical diagnosis and localization of lesion, cerebral or cervical spine lesion could mimic with each other and manifest hemiparesis as their first symptom. Meticulous history taking, neurological/ physical examination and pertinent laboratory work-up should be done before initiation of intravenous thrombolytic therapy as it could cause catastrophic consequences if not used properly.

  2. Coexistence of intervertebral disc herniation with intradural schwannoma in a lumbar segment: a case report.

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    Pan, Jianjiang; Wang, Yue; Huang, Yazeng

    2016-04-18

    Lumbar intervertebral disc herniation and spinal tumor are major pathologies that may cause back pain and radiculopathy. Neurological symptoms resulting from disc herniation and intradural spinal tumor together, however, are very rare. We report a case of lumbar disc herniation which coexists with intradural schwannoma at the same spinal level in a 67-year-old man. The patient presented with persistent low back pain, sciatica, and weakness of the lower limbs. Contrast lumbar spine magnetic resonance (MR) imaging clearly delineated an intradural lesion and an extradural herniated disc at L3/4 level. Using a single posterior approach, both pathologies were addressed. Pathological studies confirmed the intradural lesion was schwannoma. The case report highlights a rare concomitance of two symptomatic pathologies in a lumbar spine, which deserves clinical attention. Complete history, careful physical examination, and investigative measures, such as contrast MR imaging, are helpful to establish throughout diagnoses.

  3. [Neurosurgical Outcomes of Intradural Extramedullary Spinal Tumors in 97 cases:Siberian Experience].

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    Byvaltsev, Vadim Anatolyevich; Damdinov, Bair Batiyevich; Belykh, Evgenii Georgiyevich; Ivanova, Kristina Olegovna; Sorokovikov, Vladimir Alekseevich; Tsunetoshi, Kenzo; Kitai, Ryuhei; Kikuta, Kenichiro

    2017-09-01

    We retrospectively investigated the clinical presentation and outcome of patients with spinal intradural extramedullary tumors, which had been surgically treated. A total of 97 consecutive patients(32 males and 65 females;mean age, 48 years;range, 17-79 years)underwent surgery between 2004 and 2014 at Irkutsk State Medical Academy and affiliated hospitals. To determine presentation and outcomes associated with these tumors, we evaluated the waiting period before surgery, neurological symptoms including motor, sensory, urinary, and rectal dysfunction, modified McCormick scale, Macnab's outcome assessment of patient satisfaction, surgical procedure, tumor location, and histological diagnosis. The most frequent preoperative symptom was myelopathy(52.6%)at presentation, followed by radiculopathy(20.6%), and a combination of both(26.8%). Surgical intervention produced highly successful results, in which 77 of 97 cases showed neurological improvement. However, the symptoms were not completely reversed, and there was improvement only the equivalent to one grade in the modified McCormick scale for most patients. Surgical outcomes were not influenced by tumor location, extent of lamina resection, or histological diagnosis, but there was a correlation between greater patient improvement and a shorter waiting period before surgery. As such, we recommend early total resection for symptomatic spinal intradural extramedullary tumors.

  4. Endoscope-assisted retrosigmoid intradural suprameatal approach for surgical treatment of trigeminal schwannomas.

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    Samii, Madjid; Alimohamadi, Maysam; Gerganov, Venelin

    2014-12-01

    Trigeminal schwannomas are the most common intracranial nonvestibular schwannomas, and the dumbbell-shaped subtype is the most challenging. To evaluate the efficiency and safety of the endoscope-assisted retrosigmoid intradural suprameatal approach (EA-RISA) for dumbbell trigeminal schwannomas and to compare EA-RISA with classic RISA. A retrospective study of all patients with trigeminal schwannomas was performed with a focus on dumbbell tumors. Tumors were classified according to a modified Samii classification. Extent of tumor removal, outcome, and morbidity rates in the 2 subgroups were compared. Twenty patients were enrolled: 8 had dumbbell-shaped tumors (type C1), 8 had middle fossa tumors (A1-3), 3 had extracranial extension (D2), and 1 had posterior fossa tumor. Gross total resection was achieved in 15 and near-total resection in 5 patients. In 4 patients with dumbbell tumors, the classic RISA (Samii approach) was used; EA-RISA was used in the other 4 patients. The extent of petrous apex drilling was determined individually on the basis of the anatomic variability of suprameatal tubercle and degree of tumor-induced petrous apex erosion; in 2 patients, only minimal drilling was needed. The endoscope was applied after microsurgical tumor removal and in 3 of 4 patients revealed a significant unrecognized tumor remnant in the anterolateral and superolateral aspects of the Meckel cave. Thus, the EA-RISA technique allowed gross total resection of the tumor. The EA-RISA enlarges the exposure obtained with the classic RISA. Its judicious use can help achieve safe and radical removal of dumbbell-shaped trigeminal schwannomas (C1 type).

  5. Cervical Hemilaminoplasty with Miniplates in Long Segment Intradural Extramedullary Ependymoma: Case Report and Technical Note.

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    Oral, Sukru; Tumturk, Abdulfettah; Kucuk, Ahmet; Menku, Ahmet

    2018-01-01

    The surgical approaches for spinal tumors, to a great extent, have been developed in accordance with the developments in medical technology. Today, many surgical techniques are implemented as anterior, anterolateral, posterior, posterolateral and combined approaches. Due to its low morbidity, the posterior approach is the more preferred one. Laminectomy is a widely used technique, especially in neoplastic lesions. However, following laminectomy, there are numerous complications such as instability, kyphotic deformity and scar formation. In this paper, the excision of a tumor that was located intradural-extramedullary at the C3-C7 level with the cervical hemilaminoplasty technique is described. A 47-year-old female patient presented to our clinic with increasing complaints of neck and left arm pain, left arm numbness and searing pain for the last 10 years. On examination, hypoesthesia at the C4-7 dermatomes in the left upper extremity, an increase in deep tendon reflexes, and bilateral positive Hoffmann reflexes were observed. C3-C7 laminae were opened unilaterally on the right side with a midline skin incision. The laminae were drilled with a high-speed drill to provide a wide opening, both on the midline obliquely and from the border of the lamina-facet joint. After the tumor was totally excised, hemilaminae were placed into the previous position and reconstructed with mini-plates and screws. Cervical hemilaminoplasty provides a wide field of vision in tumor surgery of this region. Besides, the reconstruction of hemilaminae is important for stability. As the integrity of the spinal canal is preserved during reoperations of this region, the risk of complications is decreased.

  6. Spinal perimedullary vein enlargement sign: an added value for the differentiation between intradural-extramedullary and intramedullary tumors on magnetic resonance imaging

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    Gong, Tao; Wang, Guangbin; Gao, Fei; Chen, Xin [Shandong University, Department of Shandong Medical Imaging Research Institute, Jinan (China); Liu, Yubo [Provincial Hospital Affiliated to Shandong University, Department of Radiology, Jinan (China); Yang, Li [Zhongshan Hospital, Department of Radiology, Shanghai (China); Chen, Weibo [Philips Healthcare, Shanghai (China)

    2016-11-15

    The purpose of this study was to determine the added value of the perimedullary spinal vein enlargement sign on magnetic resonance imaging (MRI) in distinguishing intradural-extramedullary tumors (IDEMTs) from intramedullary spinal tumors (IMTs). Two hundred and eight consecutive spinal intradural tumors with histopathologic confirmation (21 IMTs, 187 IDEMTs) were enrolled. Two readers blinded to the final pathological diagnosis and clinical data independently assessed the venous enlargement sign to determine the agreement between them and jointly distinguished IDEMTs from IMTs according to the common MRI findings. Sensitivity, specificity, and accuracy for the diagnosis of IDEMTs were calculated for the common MRI findings, vein enlargement sign, and a combination of both. Intraobserver agreement and interobserver agreement for both readers was excellent. The sensitivity, specificity, and accuracy of common MRI findings for differentiating IDEMTs from IMTs were 83.4, 95.2, and 89.3 %, respectively. Thirty-one IDEMTs were mistakenly diagnosed as IMTs, in which seven were cases with vein enlargement signs. By applying the vein enlargement sign to the common MRI findings, the specificity remained at 95.2 %, while the sensitivity improved to 89.3 % and the accuracy increased to 92.3 %. The spinal perimedullary vein enlargement sign is useful in assessing intradural tumors and to differentiate IDEMTs from IMTs. (orig.)

  7. Thoracic Intradural-Extramedullary Epidermoid Tumor: The Relevance for Resection of Classic Subarachnoid Space Microsurgical Anatomy in Modern Spinal Surgery. Technical Note and Review of the Literature.

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    Barbagallo, Giuseppe M V; Maione, Massimiliano; Raudino, Giuseppe; Certo, Francesco

    2017-12-01

    Intradural epidermoid tumors of the spinal cord are commonly associated with spinal cord dysraphism or invasive procedures. We report the particular relationships between spinal subarachnoid compartments and thoracic intradural-extramedullary epidermoid tumor, highlighting the relevant anatomic changes that may influence microsurgery. A 40-year-old woman from compressive myelopathy owing to a thoracic epidermoid tumor extending from T3 to T4 and not associated with spina bifida, trauma, previous surgery, or lumbar spinal puncture underwent microsurgical excision. Accurate tumor membrane dissection, respecting spinal arachnoidal compartments, was performed. Reposition of a laminoplasty plateau helped in restoring thoracic spine anatomic integrity. Safe gross total tumor resection was achieved. Complete neurologic recovery as well as absence of recurrent tumor was documented at 4-year follow-up. A literature review revealed only 2 other cases of "isolated" thoracic spine epidermoid tumor. However, description of the relationship between tumor membranes and spinal subarachnoid compartments was not available in either case. A thorough knowledge of spinal subarachnoid space anatomy is helpful to distinguish between tumor membranes and arachnoidal planes and to achieve a safe and complete resection to avoid recurrences. Copyright © 2017 Elsevier Inc. All rights reserved.

  8. Fatores prognósticos no tratamento dos tumores intradurais extramedulares: estudo de 44 casos Prognostic factors in the treatment of the intradural extramedullary tumors: a study of 44 cases

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    Daniel Monte-Serrat Prevedello

    2003-06-01

    Full Text Available Entre 1993 e 1999 foram estudados 44 pacientes que preencheram requisitos protocolares de seguimento adequado e que foram submetidos à ressecção de processo expansivo extramedular intradural. Os pacientes eram constituídos por 43,2% de mulheres e por 56,8% de homens. A média da idade foi 32,9 anos. A localização da lesão foi mais comum na região torácica, com 45,5% dos casos, seguida da lombar com 18,2%. A extensão tumoral variou de 1 a 7 segmentos vertebrais, com uma média de 2,5 níveis. O schwanoma foi o tipo histológico mais comum com 65,9%, seguido dos meningiomas com 20,5%. Houve 2 casos de neurofibroma e 1 caso de paraganglioma, cisto neuroentérico, metástase e schwanoma maligno. A evolução foi de melhora em 56,8%, estabilidade em 31,8% e de piora em 11,4%. Não houve mortalidade relacionada ao procedimento cirúrgico. Todos os casos de piora tiveram ressecção total e eram localizados no segmento torácico. A ressecção total é a modalidade ideal de tratamento cirúrgico. No entanto, no nível torácico, onde predominam as peculiaridades de irrigação da medula, a morbidade cirúrgica pode ser mais elevada (p=0,014.Between 1993 and 1999, 44 patients submitted to resection of an expansible intradural extramedullary lesion who filled protocol requirements of appropriate follow up were studied. Patients were constituted by 43.2% female and 56.8% male. The mean age was 32.9 years old. Lesion most common location was at the thoracic spine, with 45.5% of the cases, followed by the lumbar level with 18.2%. Tumor extension varied from 1 to 7 vertebral segments, with an average of 2.5 levels. Schwannoma, with 65,9% of the cases, was the most frequent lesion, followed by meningioma with 20.5%. There were 2 cases of neurofibroma and 1 case of paraganglioma, neuroenteric cyst, metastasis and malignant schwannoma. The evolution was of improvement in 56.8%, stability in 31.8% and of worsening in 11.4%. There was no mortality

  9. Ancient schwannoma of thoracic spine in a schizophrenic patient with somatic delusion

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    Ya-Ting Wen

    2016-03-01

    Full Text Available Ancient schwannoma is a rare variant of schwannoma characterized by histopathologic degenerative changes, which are thought to be the result of long-term tumor growth and aging. However, ancient schwannoma in the spinal canal is particularly rare. We report a case of thoracic spine intradural extramedullary ancient schwannoma in a schizophrenic patient, who kept saying that “something in his back was giving him electric shock” for a long time. Unfortunately, this complaint was misinterpreted as somatic delusion symptoms. A spinal cord tumor was taken into consideration only after paraparesis developed. We have highlighted this case to remind every clinician to remain alert about the possibility of organic disease while treating patients with psychotic disorder history. Thorough neurological examination is required to avoid misdiagnosis. Spinal canal schwannoma can be totally removed successfully with good functional outcome and prognosis.

  10. Distinctive Surgical Strategy for Removal of String of Beadlike Schwannomas of Cauda Equina.

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    Liao, Dengyong; Zhang, Jing; Li, Dan; Deng, Xueyun; Ren, Qingqing; Chen, Haifeng

    2018-03-01

    Spinal schwannomas are the most common intradural extramedullary tumors. However, a string of beadlike schwannomas is rare. In some cases, the beadlike tumors might present a multiple segmental growing pattern, often located in the lumbar spinal canal and on 1 nerve fiber. Despite its benign nature, the resection of this string of beadlike tumors could be a challenge to neurosurgeons, especially when the tumors extend to a long distance. A 50-year-old female was admitted to our hospital, and her diagnosis was beadlike schwannomas. We performed 2 small hemilaminectomies and pulled all the tumors out gently. The patient made a full recovery quickly after resection. In this case, we first reported a new surgical approach for the removal of a string of beadlike cauda equina schwannomas. We hypothesize that this procedure would be a potential addition to the present surgical methods under some circumstances. Copyright © 2017 Elsevier Inc. All rights reserved.

  11. Melanotic schwannoma of the lumbar spine: a case report and literature review

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    João Bernardo Sancio Rocha Rodrigues

    2015-06-01

    Full Text Available Schwannomas are benign tumors, usually solitary, encapsulated, slow-growing, which have their origin in differentiated neoplastic Schwann cells with extramedullary intradural usual development related to nerve roots. The melanotic schwannoma is a variant of these tumors whose location in almost one third of cases is on the posterior spinal nerve root, with a nonspecific clinical presentation. Magnetic resonance imaging is the most widely used test for the diagnosis, revealing hyperintense T1-weighted sequences and hypointense T2-weighted sequences. Diagnostic confirmation is obtained by histological and immunohistochemical studies, in which there is intense cytoplasmatic pigmentation. There are two distinct types of melanotic schwannomas: sporadic and psammomatous, the latter related to the called Carney complex, a form of multiple endocrine neoplasm with familiar character. In literature we found few cases of these neoplasms, the largest series consisting of five cases. The objective of this study is to report a rare case of melanotic schwannoma of the lumbar spine of the sporadic type of extramedullary location. We also present a brief review of the literature containing the main characteristics of the tumor, including its different forms, differential diagnoses, data from histological and immunohistochemical studies as well as the currently recommended approach in order to contribute to a better understanding of this neoplasm.

  12. Surgery for ventral intradural thoracic spinal tumors with a posterolateral transpedicular approach.

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    Ito, Kiyoshi; Aoyama, Tatsuro; Miyaoka, Yoshinari; Seguchi, Tatsuya; Horiuchi, Tetsuyoshi; Hongo, Kazuhiro

    2016-08-01

    Surgery for ventrally seated thoracic tumors requires an anatomically specific approach that is distinct from cervical or lumbar spinal cord surgery as the narrower spinal canal of the thoracic spinal cord makes it sensitive to surgical procedures. However, reports describing this operative technique are few. To obtain a wide operative field and minimize thoracic spinal cord retraction, we employed a posterolateral transpedicular approach in ventral-located tumors and investigated the efficacy and limitations of this technique. Eighteen patients with lesions (meningioma or neurinoma) located in the ventral intradural thoracic region were surgically treated between 2009 and 2014. The relationship among the clinical outcome, tumor location, and postoperative spinal alignment was analyzed. Postoperative neurological function improved in all patients, namely those with meningioma (p = 0.012) and schwannoma (p = 0.018). One patient who underwent removal of two facet joints suffered a postoperative compression fracture. Removal of two facet joints and pedicles resulted in a worsening of spinal alignment (p = 0.03), while this was not the case for the removal of one facet joint and pedicle (p = 0.72). This case series clarified the benefits of the posterolateral transpedicular approach for resection of ventral intradural extramedullary tumors. Removal of one pedicle and facet joint seems to be more beneficial.

  13. Intraspinal schwannoma and neurogenic bladder.

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    Yang, K S; Ho, C S; Tai, P A; Kung, W M

    2018-04-01

    Most lumbar intradural schwannomas present initially as radiculopathies with sensory disturbances. However, neurogenic bladder dysfunction may be one of the earliest manifestations and can cause long-term disability. We present the case of a patient with a L3-4 schwannoma (newly diagnosed owing to recurrent urinary retention and urinary tract infection) who finally underwent surgical resection. Improvement of bladder sensation was documented by urodynamic study and the patient was subsequently weaned off her Foley catheter with satisfactory outcome.

  14. Primary Intradural Hemangiopericytoma With Intramedullary Invasion

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    Chiang-Wei Chou

    2009-10-01

    Full Text Available Hemangiopericytoma (HPC is a rare tumor of the central nervous system and is usually found intracranially. Intraspinal HPCs are very rare and mostly involve the extradural bony structures. Primary intradural HPC has only been reported in 10 cases, all of which occurred in the extramedullary region. Intramedullary invasion has never been reported. Here, we describe a case of primary intradural HPC of the thoracic spine that presented initially with paresthesia and paraplegia of both legs. Magnetic resonance imaging of the thoracic spine showed an intradural dumbbell-shaped tumor at the T10 level. The initial impression was neurogenic tumor, meningioma, or metastasis. During operation, the tumor was found to have obvious intramedullary invasion. Gross-total removal was done, and the patient's neurological function improved; there was no recurrence at the 3-year follow-up. There is no consensus as to what constitutes the optimal treatment of HPC, but most neurosurgeons will advocate gross-total resection. A comparative analysis between intradural and extradural HPCs showed a higher chance of gross-total resection for intradural HPCs, while the recurrence rates showed no difference. The role of adjuvant radiotherapy remains uncertain. Due to the high risk of recurrence and metastasis of HPCs, close follow-up for a long period is mandatory. [J Chin Med Assoc 2009;72(10:536–541

  15. Melanotic Schwannomas Are Rarely Seen Pigmented Tumors with Unpredictable Prognosis and Challenging Diagnosis.

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    Keskin, Elif; Ekmekci, Sumeyye; Oztekin, Ozgur; Diniz, Gulden

    2017-01-01

    Melanotic Schwannoma (MS) is rarely seen and potentially malignant neoplasm that is categorized as a variant of Schwannoma. MS most frequently involves intracranial structures followed by posterior nerve roots in the spinal canal. Approximately 50% of the cases with MS have psammomatous calcifications and this type of MS is related to Carney complex with autosomal dominant inheritance. Most cases of MS are benign, though 10% of them are malignant with metastatic potential. MS mimics melanoma and the differential diagnosis should be made excluding other melanin producing neoplasms especially melanoma. Case 1 . A 42-year-old hypertensive male presented for checkup. He had a well-defined extraspinal oval lesion measuring 3.5 × 2.5 cm near right adrenal. Case 2 . A 22-year-old female presented with neurofibromatosis-2, bilateral acoustic schwannomas and café au lait lesions on sacrococcygeal region. She had an intradural extramedullary lesion measuring 6.1 × 2.0 cm at L1-2 level. MS is a rare neoplasm composed of Schwann cells and melanin pigment. These tumors are usually benign but they may become aggressive. The biologic behavior of MS is difficult to predict; the patients have to be followed up for a longer period due to its malignant potential.

  16. Extra and intradural spinal Hemangioblastoma Hemangioblastoma espinal extra e intradural Hemangioblastoma espinhal extra e intradural

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    Marcelo Campos Moraes Amato

    2012-09-01

    Full Text Available Hemangioblastomas of the central nervous system (CNS are low-grade highly vascularized tumors that may be sporadic or associated with Von Hippel-Lindau disease. Extradural hemangioblastomas are uncommon and those located extra and intradurally are even rarer. This study uses an illustrative case and literature review to discuss the difficulties to consider the correct diagnosis and to select the best surgical approach. A 57 years-old white male patient presented with myelopathy and right C5 radiculopathy. The images showed a lobulated, hourglass shaped, highly enhanced extra/intradural lesion that occupied the spinal canal and widened the C4-C5 right intervertebral foramen. Total resection of the intradural lesion was achieved through a posterior approach, but the extradural part could only be partially removed. Complete improvement was observed after four months of follow-up and the residual tumor has been followed up clinically and radiologically. Even though the preoperative impression was of a spinal schwannoma, the histopathological examination revealed grade I hemangioblastoma as per WHO. Despite their rarity, current complementary exams allow considering the diagnosis of hemangioblastoma preoperatively. That is essential to a better surgical planning in view of the particular surgical features of this lesion.Hemangioblastomas del sistema nervioso central (SNC son tumores altamente vascularizados, de grado bajo, que pueden ser esporádicos o vinculados a la enfermedad de Von Hippel-Lindau. Hemangioblastomas extradurales no son comunes, y aquellos localizados extra e intraduralmente son aún más raros. Este estudio usa un caso ilustrativo y la revisión de la literatura para analizar las dificultades cuanto a considerar el diagnóstico correcto y para seleccionar el mejor abordaje quirúrgico. Un paciente, hombre blanco de 57 años de edad, presentaba mielopatía con radiculopatía C5 derecha. Las imágenes mostraban lesión extra/intradural

  17. Thoracolumbar intradural disc herniation in eight dogs: clinical, low-field magnetic resonance imaging, and computed tomographic myelography findings.

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    Tamura, Shinji; Doi, Shoko; Tamura, Yumiko; Takahashi, Kuniaki; Enomoto, Hirokazu; Ozawa, Tsuyoshi; Uchida, Kazuyuki

    2015-01-01

    Intradural disc herniation is a rarely reported cause of neurologic deficits in dogs and few published studies have described comparative imaging characteristics. The purpose of this retrospective cross sectional study was to describe clinical and imaging findings in a group of dogs with confirmed thoracolumbar intradural disc herniation. Included dogs were referred to one of four clinics, had acute mono/paraparesis or paraplegia, had low field magnetic resonance imaging (MRI) and/or computed tomographic myelography, and were diagnosed with thoracolumbar intradural disc herniation during surgery. Eight dogs met inclusion criteria. The prevalence of thoracolumbar intradural disc herniation amongst the total population of dogs that developed a thoracolumbar intervertebral disc herniation and that were treated with a surgical procedure was 0.5%. Five dogs were examined using low-field MRI. Lesions that were suspected to be intervertebral disc herniations were observed; however, there were no specific findings indicating that the nucleus pulposus had penetrated into the subarachnoid space or into the spinal cord parenchyma. Thus, the dogs were misdiagnosed as having a conventional intervertebral disc herniation. An intradural extramedullary disc herniation (three cases) or intramedullary disc herniation (two cases) was confirmed during surgery. By using computed tomographic myelography (CTM) for the remaining three dogs, an intradural extramedullary mass surrounded by an accumulation of contrast medium was observed and confirmed during surgery. Findings from this small sample of eight dogs indicated that CTM may be more sensitive for diagnosing canine thoracolumbar intradural disc herniation than low-field MRI. © 2014 American College of Veterinary Radiology.

  18. Intradural cervical disc herniation: a case report

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    Chung, Seung Eun; Lee, Sang Ho; Jo, Byung June; Yoon, Deug Hee [Wooridul Spine Hospital, Seoul (Korea, Republic of); Kim, Tae Hong [Inje University College of Medicine, Inje (Korea, Republic of)

    2005-07-15

    Intradural cervical disc herniation is an extremely rare condition and its pathogenesis is not certain. We experienced a case of intradural cervical disc herniation at the C4-5 level in a 56-year-old man. The preoperative sagittal T1- and T2- weighted images revealed an intradural iso-intensity lesion, with the spinal cord behind the posterior longitudinal ligament at the C4-5 disc level. The post-contrast T1-weighted image revealed a peripheral enhanced intradural lesion. We report here on a case of an intradural cervical disc herniation that was diagnosed by radiological examination, and we include a review of the related literature.

  19. Gastric schwannoma.

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    Lin, Chen-Sung; Hsu, Han-Shui; Tsai, Chien-Ho; Li, Wing-Yin; Huang, Min-Hsiung

    2004-11-01

    Gastrointestinal mesenchymal tumors are a group of tumors originated from the mesenchymal stem cells of the gastrointestinal tract, consisting of gastrointestinal stromal tumors (GIST), leiomyomas or leiomyosarcomas or schwannomas. Gastric schwannoma is a very rare gastrointestinal mesenchymal tumor, which represents only 0.2% of all gastric tumors and 4% of all benign gastric neoplasms. We report a 24-year-old girl who suffered from an episode of upper gastrointestinal bleeding. The endoscopic examination showed a round submucosal tumor with a central ulceration and bleeding over the high body of the stomach. Surgical resection of the tumor was performed. The pathological examination revealed a picture of spindle cell tumor that was strongly positive for S-100 protein stain, and non-reactive for CD34, CD117, actin, HHF-35, desmin, melan-A and HMB-45, consistent with gastric schwannoma. The literature is reviewed.

  20. Intradural disc herniation: how I do it.

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    Rodrigo, Víctor; Claramonte, Marta; Martín, Mónica; Calatayud, Juan B

    2018-03-12

    Intradural disc herniation is a rare phenomenon in spine surgery. Diagnosis is difficult despite current neuroradiologic imaging techniques. We present a case of a 59-year-old man with lumbar and radicular pain and a recurrent lumbar herniation. A laminectomy was performed after no clear disc herniation in the epidural space was found and an intradural mass was palpable. A durotomy showed an intradural disc fragment that was removed, followed by an arthrodesis. Only intraoperative findings lead to a definitive diagnosis for intradural herniation. A durotomy needs to be performed. In this case, an arthrodesis was necessary to avoid complications of segmental instability.

  1. Intraoperative Spinal Navigation for the Removal of Intradural Tumors: Technical Notes.

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    Stefini, Roberto; Peron, Stefano; Mandelli, Jaime; Bianchini, Elena; Roccucci, Paolo

    2017-08-05

    In recent years, spinal surgery has incorporated the many advantages of navigation techniques to facilitate the placement of pedicle screws during osteosynthesis, mainly for degenerative diseases. However, spinal intradural tumors are not clearly visible by intraoperative fluoroscopy or computed tomography scans, thereby making navigation necessary. To evaluate the use of spinal navigation for the removal of intradural and spinal cord tumors using spinal magnetic resonance imaging (MRI) merged with intraoperative 3-dimensional (3-D) fluoro images. After fixing the patient reference frame on the spinous process, the 3-D fluoro images were obtained in the surgical room. Using this image as the reference, the preoperative volumetric MRI images and intraoperative 3-D fluoro images were merged using automated software or manually. From January to July 2016, we performed 10 navigated procedures for intradural spinal tumors by merging MRI and 3-D fluoro images. Nine patients had an intradural extramedullary tumor, 6 had neurinomas, and 3 had meningiomas; 1 patient had an intramedullary spinal cord metastasis. The surgically demonstrated benefits of spinal navigation for the removal of intradural tumors include the decreased risk of surgery at the wrong spinal level, a minimal length of skin incision and muscle strip, and a reduction in bone removal extension. Furthermore, this technique offers the advantage of opening the dura as much as is necessary and, in the case of intrinsic spinal cord tumors, it allows the tumor to be centered. Otherwise, this would not be visible, thus enabling the precise level and the posterior midline sulcus to be determined when performing a mielotomy. Copyright © 2017 by the Congress of Neurological Surgeons

  2. MRI of orbital schwannomas

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    Abe, T.; Kawamura, N.; Homma, H.; Sasaki, K.; Izumimaya, H.; Matsumoto, K. [Department of Neurosurgery, Showa University School of Medicine, 5-8 Hatanodai 1, Shinagawa-ku, Tokyo 142-8666 (Japan)

    2000-06-01

    The literature on MRI of orbital schwannomas is limited. The appearances in three patients with an orbital schwannoma were reviewed. A superior orbitotomy through a subfrontal craniotomy revealed a schwannoma in all cases. MRI characteristics of very low signal on T 1-weighted images and homogeneous postcontrast enhancement may be helpful for differentiating schwannomas from other intraconal masses. (orig.)

  3. Facial Schwannoma

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    Mohammadtaghi Khorsandi Ashtiani

    2005-06-01

    Full Text Available Background: Facial schwannoma is a rare tumor arising from any part of the nerve. Probable symptoms are partial or facial weakness, hearing loss, visible mass in the ear, otorrhea, loss of taste, rarely pain, and sometimes without any symptoms. Patients should undergo a complete neurotologic history, examination with documentation of facial and auditory function, specially C.T. scan or M.R.I. Surgery is the only treatment option although the decision of when to remove facial schwannoma in the presence of normal facial function is difficult. Case: A 19-year-old girl with all above symptoms in the right side except loss of taste is diagnosed having facial schwannoma with full examination, audiometric, and radiological tests. She underwent surgery. In follow-up facial function were mostly restored. Conclusion: The need for careful assessment of patients with Bell's palsy cannot be overemphasized. In spite of the negative results if still there is any suspicoin, total facial nerve exploration is necessary.

  4. Soft Tissue Extramedullary Plasmacytoma

    Directory of Open Access Journals (Sweden)

    Fernando Ruiz Santiago

    2010-01-01

    Full Text Available We present the uncommon case of a subcutaneous fascia-based extramedullary plasmacytoma in the leg, which was confirmed by the pathology report and followed up until its remission. We report the differential diagnosis with other more common soft tissue masses. Imaging findings are nonspecific but are important to determine the tumour extension and to plan the biopsy.

  5. Stereotactic radiosurgery for intradural spine tumors using cone-beam CT image guidance.

    Science.gov (United States)

    Monserrate, Andrés; Zussman, Benjamin; Ozpinar, Alp; Niranjan, Ajay; Flickinger, John C; Gerszten, Peter C

    2017-01-01

    OBJECTIVE Cone-beam CT (CBCT) image guidance technology has been widely adopted for spine radiosurgery delivery. There is relatively little experience with spine radiosurgery for intradural tumors using CBCT image guidance. This study prospectively evaluated a series of intradural spine tumors treated with radiosurgery. Patient setup accuracy for spine radiosurgery delivery using CBCT image guidance for intradural spine tumors was determined. METHODS Eighty-two patients with intradural tumors were treated and prospectively evaluated. The positioning deviations of the spine radiosurgery treatments in patients were recorded. Radiosurgery was delivered using a linear accelerator with a beam modulator and CBCT image guidance combined with a robotic couch that allows positioning correction in 3 translational and 3 rotational directions. To measure patient movement, 3 quality assurance CBCTs were performed and recorded in 30 patients: before, halfway, and after the radiosurgery treatment. The positioning data and fused images of planning CT and CBCT from the treatments were analyzed to determine intrafraction patient movements. From each of 3 CBCTs, 3 translational and 3 rotational coordinates were obtained. RESULTS The radiosurgery procedure was successfully completed for all patients. Lesion locations included cervical (22), thoracic (17), lumbar (38), and sacral (5). Tumor histologies included schwannoma (27), neurofibromas (18), meningioma (16), hemangioblastoma (8), and ependymoma (5). The mean prescription dose was 17 Gy (range 12-27 Gy) delivered in 1-3 fractions. At the halfway point of the radiation, the translational variations and standard deviations were 0.4 ± 0.5, 0.5 ± 0.8, and 0.4 ± 0.5 mm in the lateral (x), longitudinal (y), and anteroposterior (z) directions, respectively. Similarly, the variations immediately after treatment were 0.5 ± 0.4, 0.5 ± 0.6, and 0.6 ± 0.5 mm along x, y, and z directions, respectively. The mean rotational angles were 0

  6. MR imaging of schwannoma

    Energy Technology Data Exchange (ETDEWEB)

    Uchida, Masafumi; Nishimura, Hiroshi; Abe, Toshi; Fujimoto, Kiminori; Kojima, Kazuyuki; Tabuchi, Akinori; Koganemaru, Michihiko; Ohtake, Hisashi (Kurume Univ., Fukuoka (Japan). School of Medicine)

    1989-08-01

    We have obtained MR imagings of 9 patients with schwannoma. Five cases were benign and four cases were malignant schwannoma. All of malignant schwannomas were complicated with Von Recklinghausen's disease. In all patients, the surgical therapy and histopathologic diagnosis were performed. Generally, MRI were not helpful in identifying tissue type except for fatty tumors. But MRI was excellent for evaluation in demonstrating capsule, structure of the tumor, and anatomical relationship of the nerve sheath and the tumor. We thought that MRI is useful for diagnosis of schwannoma. (author).

  7. Spinal intradural hydatid cyst causing arachnoiditis: A rare etiology of cauda equina syndrome

    Directory of Open Access Journals (Sweden)

    Suyash Singh

    2016-01-01

    Full Text Available This study aims to focus on a rare presentation of spinal hydatid cyst as cauda equine syndrome and misdiagnosed as intradural extramedullary (IDEM benign lesion on magnetic resonance imaging. In this article, we report a case of spinal hydatid cyst masquerading as IDEM tumor, and intraoperatively, we accidently find clumped granuloma with severe arachnoiditis and hydatid cyst in lumber region, which was present as bilateral S1 radiculopathy with cauda equina syndrome. An 11 year old boy who presented with symptoms and signs of cauda equina syndrome and planned for surgical excision. His radiological impression was IDEM possibly neurofibroma. To our surprise, we found multiple intradural cystic lesions with arachnoiditis. Dissecting in plane cyst was flushed out, and surgical cavity was irrigated with 3% saline. Postoperatively histopathology and serum tests confirmed the diagnosis of hydatid cyst. Hydatid disease is rare cause of cauda equine syndrome which can be miss diagnosed on radiological investigations. A high index of suspicion should be kept especially in a young patient from the Indian subcontinent.

  8. Intradural lumbar disc herniation with intradural gas: report of three cases

    Energy Technology Data Exchange (ETDEWEB)

    Chung, Seung Eun; Lee, Sang Ho; Jo, Byung June [Wooridul Spine Hospital, Seoul (Korea, Republic of); Kim, Tae Hong [Inje University College of Medicine, Seoul (Korea, Republic of)

    2005-12-15

    This paper reports on three cases of an intradural lumbar disc herniation (IDLDH) that were diagnosed by a radiological examination. In all cases, an intradural vacuum (IDV) was detected on the CT scans, and the IDLDH showed iso- or lower signal intensity on the T2-weighted images. Enhanced MRI of one case revealed a small amount of air, but this was without enhancement. All the cases showed definite IDV on the CT scans, and this was an important clue for diagnosing IDLDH.

  9. Extramedullary plasmacytoma of the larynx

    Directory of Open Access Journals (Sweden)

    Pinto, José Antônio

    2012-01-01

    Full Text Available Introduction: The extramedullary plasmocytoma is one of the localized forms of malignancy of the plasma cells, which has multiple myeloma main diagnosis. Its main site to the head and neck, but with a rare presentation in the larynx. Objective: To describe a case of extramedullary plasmocytoma of the larynx, with literature review. Case Report: Patient female, 49, referring to intermittent dysphonia for 01 years with progressive worsening associated with vocal fatigue and vocal effort, with reddish lesion, smooth edges fold left ventricular endoscopy. Being subjected to excisional biopsy diagnosed with extramedullary histopathological plasmocytoma. Conclusion: Extramedullary Plasmocytoma must be considered in the differential diagnosis of rare tumors of the larynx. It is essential after the diagnosis of multiple myeloma research and a "follow up" appropriate.

  10. Extramedullary plasmacytoma of the larynx.

    Science.gov (United States)

    Pinto, José Antônio; Sônego, Thiago Branco; Artico, Marina Spadari; Leal, Carolina de Farias Aires; Bellotto, Silvana

    2012-07-01

     The extramedullary plasmocytoma is one of the localized forms of malignancy of the plasma cells, which has multiple myeloma main diagnosis. Its main site to the head and neck, but with a rare presentation in the larynx.  To describe a case of extramedullary plasmocytoma of the larynx, with literature review.  Patient female, 49, referring to intermittent dysphonia for 01 years with progressive worsening associated with vocal fatigue and vocal effort, with reddish lesion, smooth edges fold left ventricular endoscopy. Being subjected to excisional biopsy diagnosed with extramedullary histopathological plasmocytoma.  Extramedullary Plasmocytoma must be considered in the differential diagnosis of rare tumors of the larynx. It is essential after the diagnosis of multiple myeloma research and a "follow up" appropriate.

  11. Focal splenic masses of the extramedullary hematopoiesis

    International Nuclear Information System (INIS)

    Incedayi, M.; Sivrioglu, A.

    2012-01-01

    Full text: Extramedullary hematopoiesis arises from pleuripotential stem cells distributed throughout the body. It is most common in patients with congenital hemolytic anemia, such as thalassemia, sickle cell anemia and hereditary spherocytosis as a response to ineffective red blood cell formation. Although microscopic foci of Extramedullary hematopoiesis are commonly seen in the spleen and liver parenchyma, focal mass-like lesion of extramedullary hematopoiesis in the liver and spleen are rare. We report a case of intrasplenic focal extramedullary hematopoiesis lesions and the imaging features of extramedullary hematopoiesis on computed tomography and magnetic resonance imaging. Extramedullary hematopoiesis should always be considered as a diagnosis in a patient with a known hematological disorder

  12. Schwannoma of stomach.

    Science.gov (United States)

    Enshaei, Ali; Hajipour, Babak; Abbasi, Fariba; Doost, Pantea Rohani; Rezaei, Seyfolah

    2015-06-01

    Gastrointestinal mesenchymal tumours are a group of tumours originating from the mesenchymal stem cells of the gastrointestinal (GI) tract. Digestive tract Schwannomas are rare mesenchymal tumours occurring most frequently in the stomach. We report the case of a 40-year-old woman with gastric Schwannoma located at the posterior wall of the antrum.

  13. Intraosseous schwannoma in schwannomatosis

    Energy Technology Data Exchange (ETDEWEB)

    Kashima, T.G.; Gibbons, M.R.J.P.; Whitwell, D.; Gibbons, C.L.M.H.; Bradley, K.M.; Ostlere, S.J.; Athanasou, N.A. [University of Oxford, Nuffield Orthopaedic Centre, Department of Pathology, Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, Oxford (United Kingdom)

    2013-12-15

    This study investigates the clinical, radiological, and pathological features of two cases of intraosseous schwannoma that arose in patients with multiple soft tissue schwannomas. In both cases, the patients were adult females and the tibial bone was affected. Vestibular schwannomas were not identified, indicating that these were not cases of neurofibromatosis 2 (NF2). Radiographs showed a well-defined lytic lesion in the proximal tibia; in one case, this was associated with a pathological fracture. Histologically, both cases showed typical features of benign schwannoma. Molecular analysis of one of the excised tumors showed different alterations in the NF2 gene in keeping with a diagnosis of schwannomatosis. Our findings show for the first time that intraosseous schwannomas can occur in schwannomatosis. (orig.)

  14. MRI features of epidural extramedullary hematopoiesis

    Energy Technology Data Exchange (ETDEWEB)

    Alorainy, Ibrahim A. E-mail: alorainy@ksu.edu.sa; Al-Asmi, Abdullah R.; Carpio, Raquel del

    2000-07-01

    A case of {beta}-thalassemia intermedia with spinal cord compression due to extramedullary hematopoiesis, which was successfully treated by blood transfusion, is presented. Emphasis was made on the MRI appearance of extramedullary hematopoiesis on different pulse sequences. The theories that aimed to explain the involvement of the epidural space by extramedullary hematopoiesis are discussed.

  15. Intradural squamous cell carcinoma in the sacrum

    Directory of Open Access Journals (Sweden)

    Fujisawa Kozo

    2009-02-01

    Full Text Available Abstract Background Leptomeningeal carcinomatosis occurs in patients with cancer at the rate of approximately 5%; it develops particularly in patients with breast cancer, lung cancer, melanoma, leukemia, or malignant lymphoma. We describe a rare case of leptomeningeal carcinomatosis in which spinal intradural squamous cell carcinoma with no lesions in the cerebral meninges and leptomeninx, was the primary lesion. Methods A 64-year-old man complained of sacral pain. Although the patient was treated with analgesics, epidural block and nerve root block, sacral pain persisted. Since acute urinary retention occurred, he was operated on. The patient was diagnosed as having an intradural squamous cell carcinoma of unknown origin. Results Since the patient presented with a slightly decreased level of consciousness 2 months after surgery, he was subjected to MRI scanning of the brain and spinal cord, which revealed disseminated lesions in the medulla oblongata. The patient died of pneumonia and sepsis caused by methicillin-resistant Staphylococcus aureus 5 months after surgery. Conclusion We report the first case of a patient with intradural squamous cell carcinoma with unknown origin that developed independently in the sacrum.

  16. Nasal septum extramedullary plasmacytoma

    Directory of Open Access Journals (Sweden)

    Belić Branislav

    2013-01-01

    Full Text Available Introduction. Plasmacytomas are malignant tumors characterized by abnormal monoclonal proliferation of plasma cells. They originate in either bone - solitary osseous plasmacytoma, or in soft tissue - extramedullary plasmacytoma (EMP. EMP represents less than 1% of all head and neck malignancies. Case report. We presented a case of EMP of the nasal septum in a 44-year-old male who had progressive difficulty in breathing through the nose and frequent heavy epistaxis on the right side. Nasal endoscopy showed dark red, soft, polypoid tumor in the last third of the right nasal cavity arising from the nasal septum. The biopsy showed that it was plasmacytoma. Bence Jones protein in the urine, serum electrophoresis, bone marrow biopsy, skeletal survey and other screening tests failed to detect multiple myeloma. This confirmed the diagnosis of EMP. The mass was completely removed via an endoscopic approach, and then, 4 week later, radiotherapy was conducted with a radiation dose of 50 Gray. No recurrence was noted in a 3-year follow- up period. Conclusion. EMP of the nasal cavity, being rare and having long natural history, represents a diagnostic and therapeutic challenge for any ear, nose and throat surgeon. Depending on the resectability of the lesion, a combined therapy is the accepted treatment.

  17. Solitary eyelid schwannoma

    Directory of Open Access Journals (Sweden)

    Renu M Magdum

    2014-01-01

    Full Text Available Schwannomas are rare benign tumors arising from Schwann cells of peripheral nerves that form the neural sheath. While there have been reports of such tumors in the orbit, solitary schwannomas arising from the eyelids are very rare. There are reports of schwannomas being erroneously diagnosed as chalazion, inclusion cysts or even eyelid malignancy. We are reporting a case of a 20-year-old female who presented with a painless, non-tender, slow-growing mass in the upper eyelid of the right eye. The external appearance of the mass was suggestive of an implantation cyst of the eyelid and it could be completely excised as it had a well-defined capsule. Histopathological examination showed characteristic hypercellular and hypocellular areas with fusiform nuclei that tended to form palisades. The purpose of reporting this case of schwannoma in a young female is to recommend the inclusion of this entity as a differential diagnosis of well-defined lid tumors.

  18. Epidural extramedullary haemopoiesis in thalassaemia

    International Nuclear Information System (INIS)

    Boyacigil, S.; Ali, A.; Ardic, S.; Yuksel, E.

    2002-01-01

    lntrathoracic extramedullary haematopoiesis is a rare condition. Involvement of the spinal epidural space with haematopoietic tissue is rather unusual. A 31-year-old-man with a known diagnosis of β-thalassaemia was referred with focal back pain. Magnetic resonance imaging revealed diffuse bone-marrow changes, thoracic paraspinal masses and lobulated epidural masses, suggesting extramedullary haemopoiesis. The patient was treated with radiotherapy and blood transfusions. Follow-up MRI was performed for evaluation efficacy of the treatment. Copyright (2002) Blackwell Science Pty Ltd

  19. CT morphology of malignant schwannomas

    Energy Technology Data Exchange (ETDEWEB)

    Triebel, H.J.; Heller, M.; Schumann, R.; Langkowski, J.H.; Schaefer, H.J.; Weh, H.J.

    1988-10-01

    Thirty patients with histologically confirmed malignant schwannomas were examined. In five patients the lesions were malignant neurofibromas, as part of von Recklinghausen's disease, the others were solitary malignant schwannomas. CT appearances of malignant schwannomas are variable. Shape, structure and attenuation vary considerably. Most malignant schwannomas present with the CT criteria of benign tumours. Altogether neither the solitary schwannomas, nor those associated with von Recklinghausen's disease show specific diagnostic features, whether for the primary tumour or for recurrences. (orig./GDG).

  20. Sympathetic chain Schwannoma

    International Nuclear Information System (INIS)

    Al-Mashat, Faisal M.

    2009-01-01

    Schwannomas are rare, benign, slowly growing tumors arising from Schwann cells that line nerve sheaths. Schwannomas arising from the cervical sympathetic chain are extremely rare. Here, we report a case of a 70-year-old man who presented with only an asymptomatic neck mass. Physical examination revealed a left sided Horner syndrome and a neck mass with transmitted pulsation and anterior displacement of the carotid artery. Computed tomography (CT) showed a well-defined non-enhancing mass with vascular displacement. The nerve of origin of this encapsulated tumor was the sympathetic chain. The tumor was excised completely intact. The pathologic diagnosis was Schwannoma (Antoni type A and Antoni type B). The patient has been well and free of tumor recurrence for 14 months with persistence of asymptomatic left sided Horner syndrome. The clinical, radiological and pathological evaluations, therapy and postoperative complications of this tumor are discussed. (author)

  1. Thyroid enlargement due to extramedullary plasmacytoma.

    Science.gov (United States)

    De Schrijver, I; Smeets, P

    2004-01-01

    We report the case of a 69-year-old woman presenting with a rapidly enlarging thyroid mass as the first extramedullary symptom of plasmacytoma. Extramedullary locations are found in less than 5% of patients with multiple myeloma and can arise in any tissue. If the patient presents with extramedullary lesions and the radiologist is unaware of the underlying disease process the masses can be mistaken for disseminated metastatic carcinoma or non-Hodgkin's lymphoma.

  2. Extramedullary plasmocytoma relapsing at differents sites: an ...

    African Journals Online (AJOL)

    Extramedullary plasmocytoma relapsing at differents sites: an unusual presentation. Maryame Ahnach, Sofia Marouan, Mohamed Rachid, Abdellah Madani, Asmaa Quessar, Said Benchekroun, Meryem Quachouh ...

  3. Deregulated genes in sporadic vestibular schwannomas

    DEFF Research Database (Denmark)

    Cayé-Thomasen, Per; Helweg-Larsen, Rehannah Holga Andrea; Stangerup, Sven-Eric

    2010-01-01

    In search of genes associated with vestibular schwannoma tumorigenesis, this study examines the gene expression in human vestibular nerve versus vestibular schwannoma tissue samples using microarray technology....

  4. Extramedullary spinal cysts in dogs.

    Science.gov (United States)

    Lowrie, Mark L; Platt, Simon R; Garosi, Laurent S

    2014-08-01

    To (1) synthesize the terminology used to classify extramedullary spinal cysts in dogs to clarify some of the commonly reported misconceptions, and (2) propose a classification scheme to limit confusion with terminology. Literature review. An online bibliographic search was performed in January 2013 for articles relating to extramedullary spinal cysts in dogs using PubMed (http://www.pubmed.gov/) and Google Scholar (http://scholar.google.com/) databases. Only peer-reviewed clinical literature describing cystic lesions pertaining to the spinal cord and associated structures was included. From 1962 to 2013, 42 articles were identified; 25 (95 dogs) reported meningeal cysts, 10 (24 dogs) described 60 extradural cysts, 3 reports (18 dogs) described discal cysts or acute compressive hydrated nucleus pulposus extrusions (HNPE). Spinal cysts were categorized by location based on cross-sectional imaging as meningeal or extradural non-meningeal. Sub-classification was then performed based on surgical findings and pathology. Meningeal cysts included arachnoid diverticulae and Tarlov (perineural) cysts. Extradural non-meningeal cysts included intraspinal cysts of the vertebral joints, ligaments and discs. Discal cysts also fit this category and have been reported extensively in humans but appear rare in dogs. Extramedullary spinal cysts should be first classified according to location with a sub-classification according to pathologic and surgical findings. Previous canine cases of discal cysts appear to represent a different disease entity and the term acute compressive HNPE is therefore preferred. © Copyright 2014 by The American College of Veterinary Surgeons.

  5. how acoustic schwannomas?

    African Journals Online (AJOL)

    chemistry In the diagnosis of brain tumours and, furthermore, shows that acoustic schwannoma must be considered In the appropriate clinical setting, even in a group previously regarded as low-risk. S Atr Med J 1990; 78: 11-14. Studies from various parts of the world on the relative. freqIJency of primary intracranial tumours ...

  6. Deregulated genes in sporadic vestibular schwannomas

    DEFF Research Database (Denmark)

    Cayé-Thomasen, Per; Helweg-Larsen, Rehannah Holga Andrea; Stangerup, Sven-Eric

    2010-01-01

    In search of genes associated with vestibular schwannoma tumorigenesis, this study examines the gene expression in human vestibular nerve versus vestibular schwannoma tissue samples using microarray technology.......In search of genes associated with vestibular schwannoma tumorigenesis, this study examines the gene expression in human vestibular nerve versus vestibular schwannoma tissue samples using microarray technology....

  7. Extramedullary pasmacytoma masquerading as chalazion.

    Science.gov (United States)

    Maheshwari, Rajat; Maheshwari, Sejal

    2009-01-01

    Extramedullary plasmacytoma (EMP) is a rare entity that belongs to the category of non-Hodgkin lymphoma. EMP make up 4% of all plasma cell tumors and occur mainly in the upper aerodigestive tract. Primary plasmacytoma involving the orbit is rare. There is a relation between solitary plasmacytoma and subsequent development of multiple myeloma. As no predictors of progression have been identified patients need indefinite follow-up. The authors report a case of EMP involving the eyelid in an otherwise healthy adult female.

  8. Angiogenesis in vestibular schwannomas

    DEFF Research Database (Denmark)

    Møller, Martin Nue; Werther, Kim; Nalla, Amarnadh

    2010-01-01

    targets the angiogenic process by investigation of tumor expression of MMP-2, MMP-9, and tissue inhibitors of metalloproteinase (TIMP)-1. A possible correlation with gender, patient age, symptom duration, tumor size, and the absolute and relative growth rate is explored.......Vascular endothelial growth factor (VEGF) and matrix metalloproteinases (MMPs) are potent mediators of tumor angiogenesis. It has been demonstrated that vestibular schwannoma VEGF expression correlates with tumor growth pattern, whereas knowledge on the expression of MMPs is lacking. This study...

  9. Extramedullary plasmacytoma involving perirenal space accompanied by extramedullary hematopoiesis and amyloid deposition.

    Science.gov (United States)

    Mimura, Rie; Kamishima, Tamotsu; Kubota, Kanako C; Nakano, Fumihito; Yabe, Ichiro; Sasaki, Hidenao; Maruyama, Satoru; Shinohara, Nobuo; Harris, Ardene A; Haga, Hironori; Shirato, Hiroki; Terae, Satoshi

    2010-05-01

    A 62-year-old man was referred to us after unsuccessful treatment of bilateral weakness in his upper and lower extremities with paresthesia in both lower extremities. Computed tomography (CT) revealed soft tissue masses in the left kidney along the capsule and paraaortic region that were of relatively low attenuation with accompanying granular calcifications. Pathological diagnosis of the biopsy specimen was extramedullary plasmacytoma accompanied by extramedullary hematopoiesis and amyloid deposition. Although the CT findings correlated well with the pathological results, the case was extremely atypical for extramedullary plasmacytoma in respect to location and the accompaniment with extramedullary hematopoiesis.

  10. Minimally invasive approach for small ventrally located intradural lesions of the craniovertebral junction.

    Science.gov (United States)

    Eicker, Sven O; Mende, Klaus Christian; Dührsen, Lasse; Schmidt, Nils Ole

    2015-04-01

    The surgical management of lesions ventral to the neuraxis at the level of the craniovertebral junction (CVJ) and upper cervical spine is challenging. Here, the authors describe a minimally invasive dorsal approach for small ventrally located intradural lesions at the CVJ as an alternative for the more extensive classic transoral approach or variants of suboccipital approaches. Between 2012 and 2014, 6 symptomatic patients with a small lesion of the ventral aspect at the CVJ level were treated using a minimally invasive dorsal approach at the University Medical Center in Hamburg-Eppendorf, Germany. The anatomical distance between the posterior atlantooccipital membrane and the posterior atlantoaxial ligament, as determined by CT images, was assessed in the treated patients and in 100 untreated persons. The authors treated 6 patients (mean age 54.7 years) who had a clinical presentation of mild neurological symptoms that disappeared after resection. Minimally invasive surgical dorsal access was achieved by using tubular systems and using the natural space between the occiput (C-0) and C-1, and in 1 case between C-1 and C-2, without having to remove bony structures. The postoperative course in each of the 6 patients was uneventful. The neuropatho-logical findings confirmed a meningotheliomatous meningioma (WHO Grade I) in 5 cases and an extramedullary cavernous hemangioma in 1 case. MRI confirmed complete resection of all the lesions. The atlantooccipital distances ranged from 3 to 17 mm (mean 8.98 mm) in the supine neutral position, and the atlantoaxial distances ranged from 5 to 17 mm (mean 10.56 mm). There were no significant differences between women and men (atlantooccipital p = 0.14; atlantoaxial p = 0.72). The results of this study demonstrate that the minimally invasive dorsal approach using the space between C-0 and C-1 or C-1 and C-2 provides direct and sufficient exposure for the safe surgical resection of small ventrally located intradural lesions at the

  11. Schwannoma of the Colon

    Directory of Open Access Journals (Sweden)

    Ronaldo Nonose

    2009-09-01

    Full Text Available Schwannomas are neoplasms originating from Schwann cells, which are the cells forming nerve sheaths. These neoplasms generally involve peripheral nerves. They rarely affect the gastrointestinal tract and primary colon involvement is extremely rare. The objective of the present paper was to present a case of primary schwannoma of the sigmoid colon, unassociated with von Recklinghausen disease, that was histopathologically confirmed by means of an immunohistochemical panel. The patient was a 71-year-old woman who had had rectal bleeding when evacuating, with pain and tenesmus, for 4 months. She underwent colonoscopy, which identified a raised submucous lesion of 2.8 cm in diameter, located in the sigmoid colon, 30 cm from the anal margin. During examination, loop polypectomy with lesion excision was performed. Histopathological evaluation showed that this was a tumor of stromal origin. Its resection margins were compromised by neoplasia, and colon resection by means of videolaparoscopy was indicated. Conventional histopathological examination using the hematoxylin-eosin technique suggested that the neoplasm was of mesenchymal origin. An immunohistochemical panel was run for etiological confirmation, using anti-CD34 antibodies, desmin, cytokeratins (AE1/AE3, cKit, chromogranin and S-100 protein. The panel showed intense immunoexpression of S-100 protein. Investigation of the proliferative activity rate using Ki-67 antibodies showed that there was a low rate of mitotic activity, thus confirming the diagnosis of primary benign schwannoma of the colon. The patient’s postoperative evolution was uneventful and she remains in good health, without signs of tumor recurrence, 15 months after surgical excision.

  12. [Introsseous schwannoma of the mandible].

    Science.gov (United States)

    Bodner, Lipa; Manor, Esther; Woldenberg, Yitzhak; Kachko, Leonid; Brennan, Peter A; Lorincz, Adám

    2013-12-01

    Schwannoma is a benign neoplasm originating from the neural sheath and occuring most often in the soft tissues of the head and neck. Intraosseous schwannoma (IS) is extremely rare, most commonly occurring in the mandible. This paper documents a case of IS and the histopathology, karyotyping, CT, and MRI in the diagnostic work-up. Histologically it was a classic schwannoma. The karyotype was normal. CT demonstrated destruction of the mandibular canal while MRI detected encasement of the inferior alveolar nerve by the tumor, and consequently the need to resect the nerve together with the tumor. Using CT and MRI, the morbidity associated with the anticipated surgery can precisely be determined preoperatively.

  13. Schwannoma of the sigmoid colon

    OpenAIRE

    Çakır, Tuğrul; Aslaner, Arif; Yaz, Müjgan; Gündüz, Umut rıza

    2015-01-01

    Colonic schwannomas are very rare gastrointestinal tumours originating from Schwann cells, which form the neural sheath. Primary schwannomas of the lower gastrointestinal tract are very rare and usually benign in nature. However, if they are not surgically removed, malign degeneration can occur. We report a case of a 79-year-old woman who presented to our clinic with rectal bleeding and constipation. She underwent a lower gastrointestinal tract endoscopy. A mass subtotally obstructing the lum...

  14. Clinicopathologic Features of Gastric Schwannoma

    OpenAIRE

    Tao, Kaixiong; Chang, Weilong; Zhao, Ende; Deng, Rui; Gao, Jinbo; Cai, Kailin; Wang, Guobin; Zhang, Peng

    2015-01-01

    Abstract To explore the clinicopathologic characteristics, diagnosis, treatment, and prognosis of gastric schwannoma in the imatinib era. The clinicopathologic characteristics and postoperative outcomes of patients diagnosed with gastric schwannoma at our institution between January 2007 and February 2015 were retrospectively collected and analyzed. The main patient complaint was epigastric pain or discomfort. Tumor sizes ranged from 15 to 80?mm (mean, 57.1?mm). In 17 patients, the tumors wer...

  15. Plexiform schwannoma of the forearm

    Energy Technology Data Exchange (ETDEWEB)

    Katsumi, Keiichi; Ogose, Akira; Hotta, Tetsuo; Hatano, Hiroshi; Kawashima, Hiroyuki; Endo, Naoto [Division of Orthopedic Surgery, Department of Regenerative and Transplant Medicine, Graduate School of Medical and Dental Sciences, Niigata University, Asahimachi 1-751, 951-8510, Niigata (Japan); Umezu, Hajime [Division of Pathology, Niigata University Hospital, Niigata (Japan)

    2003-12-01

    We report a case of plexiform schwannoma located in the flexor muscles of the forearm in the absence of other signs of neurofibromatosis or schwannomatosis. Magnetic resonance examination revealed a multinodular irregular inhomogeneous mass. Some nodules displayed a peripheral, high intensity rim and a central low intensity (target sign) on T2-weighted images. Pre-operative diagnosis of the rare plexiform schwannoma may be possible with careful imaging examination for the target sign. (orig.)

  16. [Anterior skull-base schwannoma].

    Science.gov (United States)

    Esquivel-Miranda, Miguel; De la O Ríos, Elier; Vargas-Valenciano, Emmanuelle; Moreno-Medina, Eva

    Schwannomas are nerve sheath tumours that originate in Schwann cells. They are usually solitary and sporadic and manifest on peripheral, spinal or cranial nerves. Intracranial schwannomas tend to manifest on the eighth cranial nerve, particularly in patients with neurofibromatosis type2. Anterior skull-base schwannomas represent less than 1% of all intracranial schwannomas. They are more frequent in young people and are typically benign. These tumours represent a diagnostic challenge due to their rarity and difficult differential diagnosis, and numerous theories have been postulated concerning their origin and development. In this article, we present the case of a 13-year-old male with a single anterior cranial-base tumour not associated with neurofibromatosis who presented with headache, papilloedema, eye pain and loss of visual acuity. Complete resection of the tumour was performed, which was histopathologically diagnosed as a schwannoma. The patient made a complete clinical recovery with abatement of all symptoms. We conducted a review of the literature and found 66 cases worldwide with this diagnosis. We describe the most relevant epidemiological and clinical characteristics of this kind of tumour and its relation with the recently discovered and similar olfactory schwannoma. Copyright © 2017 Sociedad Española de Neurocirugía. Publicado por Elsevier España, S.L.U. All rights reserved.

  17. Vestibular Schwannoma (Acoustic Neuroma) and Neurofibromatosis

    Science.gov (United States)

    ... Infections, and Deafness Vestibular Schwannoma (Acoustic Neuroma) and Neurofibromatosis On this page: What is a vestibular schwannoma ( ... are usually associated with a genetic disorder called neurofibromatosis type 2 (NF2). Half of affected individuals have ...

  18. Intradural hemangiopericytoma of the lumbar spine: a rare entity.

    Science.gov (United States)

    Fitzpatrick, D; Mahajan, J; Lewkowitz, M; Black, K; Setton, A; Woldenberg, R

    2009-01-01

    We report a case of a patient with an intradural hemangiopericytoma of the lumbar spine and the unusual MR angiography (MRA) and spinal angiography findings of arteriovenous shunting with spinal venous congestion. We highlight the concordance of the unusual MRA and angiographic findings and their relationship to combined endovascular and surgical therapy.

  19. Mass-like extramedullary hematopoiesis: imaging features

    Energy Technology Data Exchange (ETDEWEB)

    Ginzel, Andrew W. [Synergy Radiology Associates, Houston, TX (United States); Kransdorf, Mark J.; Peterson, Jeffrey J.; Garner, Hillary W. [Mayo Clinic, Department of Radiology, Jacksonville, FL (United States); Murphey, Mark D. [American Institute for Radiologic Pathology, Silver Spring, MD (United States)

    2012-08-15

    To report the imaging appearances of mass-like extramedullary hematopoiesis (EMH), to identify those features that are sufficiently characteristic to allow a confident diagnosis, and to recognize the clinical conditions associated with EMH and the relative incidence of mass-like disease. We retrospectively identified 44 patients with EMH; 12 of which (27%) had focal mass-like lesions and formed the study group. The study group consisted of 6 male and 6 female subjects with a mean age of 58 years (range 13-80 years). All 12 patients underwent CT imaging and 3 of the 12 patients had undergone additional MR imaging. The imaging characteristics of the extramedullary hematopoiesis lesions in the study group were analyzed and recorded. The patient's clinical presentation, including any condition associated with extramedullary hematopoiesis, was also recorded. Ten of the 12 (83%) patients had one or more masses located along the axial skeleton. Of the 10 patients with axial masses, 9 (90%) had multiple masses and 7 (70%) demonstrated internal fat. Eight patients (80%) had paraspinal masses and 4 patients (40%) had presacral masses. Seven patients (70%) had splenomegaly. Eleven of the 12 patients had a clinical history available for review. A predisposing condition for extramedullary hematopoiesis was present in 10 patients and included various anemias (5 cases; 45%), myelofibrosis/myelodysplastic syndrome (4 cases; 36%), and marrow proliferative disorder (1 case; 9%). One patient had no known predisposing condition. Mass-like extramedullary hematopoiesis most commonly presents as multiple, fat-containing lesions localized to the axial skeleton. When these imaging features are identified, extramedullary hematopoiesis should be strongly considered, particularly when occurring in the setting of a predisposing medical condition. (orig.)

  20. Extramedullary paraspinal hematopoiesis in hereditary spherocytosis

    Directory of Open Access Journals (Sweden)

    Gogia P

    2008-01-01

    Full Text Available Hereditary spherocytosis (HS is a common inherited hemolytic anemia due to red cell membrane defects. Extramedullary hematopoiesis is a compensatory response to insufficient bone marrow blood cell production. The preferred sites of extramedullary hematopoietic involvement are the spleen, liver and lymph nodes; but in HS, the posterior paravertebral mediastinum is also commonly involved. We report a case of a 50-year-old male who presented to us in respiratory distress and with bilateral paravertebral posterior mediastinal masses, which on trucut biopsy were found to be extra-hematopoietic masses; and the patient was found to have hereditary spherocytosis.

  1. Solitary extramedullary plasmacytoma of the sinonasal region.

    Science.gov (United States)

    Hazarika, Produl; Balakrishnan, R; Singh, Rohit; Pujary, Kailesh; Aziz, Benazim

    2011-07-01

    Less than 10% of the patients with plasma cell neoplasms present with a solitary plasmacytoma. Though the nasal cavity is a common extramedullary site, the occurrence is extremely rare. Two cases of solitary extramedullary plasmacytoma of the sinonasal region are reported. The first of which is sinonasal plasmacytoma with concomitant HIV, an association that has been reported rarely in literature to date and is matter of much debate. In the second case report, we present an instance of surgical excision of the tumor using KTP 532 laser. The diagnosis was established using immunohistochemical techniques and multiple myeloma workups were negative in all cases.

  2. Clinicopathologic Features of Gastric Schwannoma

    Science.gov (United States)

    Tao, Kaixiong; Chang, Weilong; Zhao, Ende; Deng, Rui; Gao, Jinbo; Cai, Kailin; Wang, Guobin; Zhang, Peng

    2015-01-01

    Abstract To explore the clinicopathologic characteristics, diagnosis, treatment, and prognosis of gastric schwannoma in the imatinib era. The clinicopathologic characteristics and postoperative outcomes of patients diagnosed with gastric schwannoma at our institution between January 2007 and February 2015 were retrospectively collected and analyzed. The main patient complaint was epigastric pain or discomfort. Tumor sizes ranged from 15 to 80 mm (mean, 57.1 mm). In 17 patients, the tumors were located in the body of the stomach. A total of 20 patients were preoperatively misdiagnosed with a gastrointestinal stromal tumor. The rate of correct preoperative diagnosis was only 3.3%. All patients underwent surgical resection and showed strong S-100 protein positivity. Laparoscopic surgery for gastric schwannoma was associated with less blood loss and a shorter postoperative hospital stay than open surgery (P Gastric schwannoma is often preoperatively misdiagnosed as gastric gastrointestinal stromal tumor. Laparoscopic resection of gastric schwannoma is considered safe and effective, and it may be the preferred surgery for most small- and moderate-sized tumors. The long-term outcome is excellent, as this type of neoplasm is uniformly benign. PMID:26559271

  3. Retroperitoneal Schwannoma: A Rare Case

    Directory of Open Access Journals (Sweden)

    Murat Kalaycı

    2011-01-01

    Case Presentation. A 38-year-old woman was admitted to the emergency service with the complaints of progressive abdominal pain and nausea for the last 24 hours. Abdominal examination was compatible with acute abdomen. Acute appendicitis was diagnosed by CT. During CT evaluation, a round shaped soft-tissue mass at the retroperitoneal area inferior to the right kidney was detected, The mass was resected and histology revealed schwannoma. Conclusion. Rare tumoral lesions with benign course such as schwannoma can be detected incidentally.

  4. Gastric Schwannoma: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Kye Ho; Jee, Keum Nahn [Dankook University Cellege of Medicine, Seoul (Korea, Republic of)

    2006-03-15

    Gastric Schwannoma is a rare benign intramural tumor arising from the stomach, and it accounts for only 0.1% of all the different kinds of gastric neoplasms, and it's less than 4% of all the benign gastric tumors. This tumor is very difficult to differentiate from the other mesenchymal tumors by the clinical, endoscopic and radiologic findings. In this study, we demonstrate the appearance of this tumor on endoscopic ultrasound and contrast-enhanced abdomen CT. We also show the histopathologic findings of a surgically confirmed gastric Schwannoma that was located in the proper muscle layer.

  5. Schwannoma de párpado Eyelid schwannoma

    Directory of Open Access Journals (Sweden)

    E. Moretti

    2012-09-01

    Full Text Available El schwannoma es un tumor benigno de las células de Schwann de los nervios periféricos. Cuando se origina en ramas del V par craneal puede representar el 0,7 % de los tumores palpebrales. Aunque rara vez maligniza, la cirugía es el tratamiento de elección. Hasta donde conocemos, existen 11 casos de schwannoma palpebral publicados en la literatura. Nuestro objetivo es presentar un caso clínico de esta estirpe tumoral en una paciente de 51 años de edad, con antecedentes de extirpación transconjuntival previa de chalazión.Schwannoma is a benign tumour of Schwann cells of the peripheral nerves. When originated from branches of the V cranial nerve can represent 0.7 % of eyelid tumours. Although rarely malignant, surgery is the treatment of choice. Until we can know, there are 11 cases reported in the literature. Our goal is to present a case of eyelid schwannoma in a 51 years old patient with a history of previous transconjunctival excision of chalazion.

  6. Subcutaneous growth of human acoustic schwannomas in athymic nude mice

    DEFF Research Database (Denmark)

    Charabi, S.; Rygaard, J.; Klinken, Leif

    1994-01-01

    Neuropathology, acoustic schwannomas, subcutaneous implantation, nude mouse, growth, pcna, ki-67......Neuropathology, acoustic schwannomas, subcutaneous implantation, nude mouse, growth, pcna, ki-67...

  7. MR findings in intralabyrinthine schwannomas

    Energy Technology Data Exchange (ETDEWEB)

    Montague, Mary-Louise; Kishore, Ameet; Hadley, Donald M.; O' Reilly, Brian

    2002-05-01

    AIM: Intralabyrinthine schwannomas (ILS) are rare benign tumours. They are not always recognized on routine magnetic resonance imaging (MRI). We aimed to study the clinical presentation and MRI findings in our patients with ILS. MATERIALS AND METHODS: Retrospective analysis of patients with vestibular schwannomas treated at this center. RESULTS: Of 144 vestibular schwannomas studied at this centre, three patients had an ILS. The most common presenting symptoms were unilateral hearing loss, tinnitus and vertigo. Two patients demonstrated a progressive sensorineural hearing loss (SNHL). The third patient had a severe SNHL at presentation. MRI enhanced with contrast medium was positive in the two patients with progressive SNHL and negative in the patient with the severe SNHL. CONCLUSION: This series demonstrates the ability of MRI to identify schwannomas filling the labyrinth, and also its inability to identify extremely small ILS. It underlines the importance of sending the cristae of patients undergoing labyrinthectomy for presumed Meniere's disease for histological examination. Montague, M.-L. et al. (2002)

  8. [Dumbbell malignant dorsal schwannoma embolized and operated by single posterior approach].

    Science.gov (United States)

    Zabalo, Gorka; de Frutos, Daniel; García, Juan Carlos; Ortega, Rodrigo; Guelbenzu, Juan José; Zazpe, Idoya

    2018-02-19

    We report a case of a 41 years old patient complaining of chronic dorsalgia. MRI showed a well defined intradural extramedular dumbbell-shaped lesion, associated to a left paravertebral tumor at D5-D6 level. The tumor was embolizated prior to surgery. Following she underwent a D4-D6 laminotomy, left D5-D6 costotransversectomy and resection of the intracanal extradural part of the lesion with section of the left D5 nerve. Posteriorly, complete resection of the extracanal portion of the tumor was performed by a postero-lateral approach. The anatomopathologic diagnosis was a malignant schwannoma. After the surgery, the patient required adyuvant radiotherapic treatment. Malignant schwannoma is a very uncommon tumor which belongs to the malignant peripheral nerve sheath tumors (MPNST). It develops from Schwann cells. It is frequently associated with type 1 neurofibromatosis. MPNST usually present local recurrence and can metastatize. Copyright © 2018 Sociedad Española de Neurocirugía. Publicado por Elsevier España, S.L.U. All rights reserved.

  9. AN INTERESTING CASE OF ANCIENT SCHWANNOMA

    Directory of Open Access Journals (Sweden)

    Binu

    2015-01-01

    Full Text Available INTRODUCTION : Schwannoma is a common benign tumour of nerve sheath. Degenerating type of schwannoma is called ancient schwannoma. Ancient schwannomas of scalp are rare and are often misdiagnosed as sebaceous cyst or dermoid cyst. CASE REPORT : We present a thirty two year old male presented with scalp swel ling of eight years duration. X - ray showed no intracranial extension. He underwent excision of the tumour and histopathology was reported as ancient schwannoma. DISCUSSION : Histopathologically , ancient schwannomas charecterised by cellular Antoni type A ar eas and less cellular Antoni type - B areas. 9 th , 7 th , 11 th , 5 th and 4 th cranial nerves are often affected and may be associated with multiple neuro fibramatosis (Von - Recklinghausen’s disease. Impact : Case is presented for its rarity and possible pre - operative misdiagnosis

  10. Extramedullary paraspinal hematopoiesis in thalassemia: CT and MRI evaluation

    Energy Technology Data Exchange (ETDEWEB)

    Tsitouridis, J.; Stamos, S.; Hassapopoulou, E.; Tsitouridis, K.; Nikolopoulos, P

    1999-04-01

    We present a comparative CT and MRI study of the paraspinal extramedullary hematopoiesis in 32 thalassemic patients. The patients were classified into four groups according to the MRI and CT imaging findings. Active recent extramedullary paraspinal hematopoietic masses show soft tissue behavior in both CT and MRI. Older inactive masses reveal iron deposition or fatty replacement. Combined imaging findings of paraspinal extramedullary hematopoiesis revealed the phase of its evolution and the correct diagnosis.

  11. Gastric schwannoma coexists with peptic ulcer perforation

    Directory of Open Access Journals (Sweden)

    Volkan İnce

    2011-09-01

    Full Text Available Gastric schwannoma is a benign neoplasm that originates from sheet of nerve cell in stomach. Differential diagnosis of gastrointestinal stromal tumors, (GISTs which have malign potential, than these tumors, which definite diagnosis is determined by histopathological and immunohistochemical methods have clinical significance due to gastric schwannomas have excellent progress after surgical resection. We presented a case of gastric schwannoma coexists with peptic ulcer perforation with guide of literature in this study.

  12. A Case of Mediastinal Extramedullary Plasmacytoma Associated with Multiple Myeloma

    Energy Technology Data Exchange (ETDEWEB)

    Min, Ji Hye; Kim, Tae Sung; Ko, Young Hyeh; Kim, Ki Hyun [Samsung Medical Center, Sungkyunkwan University, Seoul (Korea, Republic of)

    2010-08-15

    Extramedullary plasmacytoma is a rare manifestation of multiple myeloma, and involvement of the mediastinum by extramedullary plasmacytoma is very rare. We report here on a rare case of a large mediastinal extramedullary plasmacytoma and several pleural nodules with pleural effusions in a 45-year-old male patient with multiple myeloma that involved the thoracic spine and the calvarium. The mediastinal extramedullary plasmacytoma manifested on CT as an 11 x 4.5 cm-sized, relatively homogeneous, mildly enhancing, anterior mediastinal mass with several pleural nodules, and this simulated malignant lymphoma or malignant thymic epithelial tumor.

  13. A Case of Mediastinal Extramedullary Plasmacytoma Associated with Multiple Myeloma

    International Nuclear Information System (INIS)

    Min, Ji Hye; Kim, Tae Sung; Ko, Young Hyeh; Kim, Ki Hyun

    2010-01-01

    Extramedullary plasmacytoma is a rare manifestation of multiple myeloma, and involvement of the mediastinum by extramedullary plasmacytoma is very rare. We report here on a rare case of a large mediastinal extramedullary plasmacytoma and several pleural nodules with pleural effusions in a 45-year-old male patient with multiple myeloma that involved the thoracic spine and the calvarium. The mediastinal extramedullary plasmacytoma manifested on CT as an 11 x 4.5 cm-sized, relatively homogeneous, mildly enhancing, anterior mediastinal mass with several pleural nodules, and this simulated malignant lymphoma or malignant thymic epithelial tumor

  14. Epidemiology and natural history of vestibular schwannomas

    DEFF Research Database (Denmark)

    Stangerup, Sven-Eric; Caye-Thomasen, Per

    2012-01-01

    This article describes various epidemiologic trends for vestibular schwannomas over the last 35 years, including a brief note on terminology. Additionally, it provides information on the natural history of tumor growth and hearing level following the diagnosis of a vestibular schwannoma. A treatm......This article describes various epidemiologic trends for vestibular schwannomas over the last 35 years, including a brief note on terminology. Additionally, it provides information on the natural history of tumor growth and hearing level following the diagnosis of a vestibular schwannoma....... A treatment strategy based on the natural history of tumor growth and hearing also is discussed....

  15. Solitary gastric melanotic schwannoma: sonographic findings.

    Science.gov (United States)

    Chen, Yang-Yuan; Yen, Hsu-Heng; Soon, Maw-Soan

    2007-01-01

    Solitary gastric schwannoma is rare, and solitary melanotic schwannoma is even rarer, posing a dilemma in diagnosis and treatment. We report the case of a 69-year-old woman with gastric melanotic schwannoma who presented with nausea, vomiting, and abdominal pain. Abdominal sonographic examination revealed a 5-cm hypoechoic mass in the epigastric area that was confirmed to be a gastric submucosal tumor on endoscopic examination. The diagnosis of melanotic schwannoma was confirmed via sonographically guided percutaneous core biopsy. The tumor was resected, and no recurrence has occurred in a 3-year follow-up.

  16. Renal extramedullary hematopoiesis: interstitial and glomerular pathology.

    Science.gov (United States)

    Alexander, Mariam P; Nasr, Samih H; Kurtin, Paul J; Casey, Edward T; Hernandez, Loren P Herrera; Fidler, Mary E; Sethi, Sanjeev; Cornell, Lynn D

    2015-12-01

    Renal extramedullary hematopoiesis is rarely recognized in the antemortem setting. We identified 14 patients with renal extramedullary hematopoiesis on antemortem specimens from 1994 to 2015. The mean age was 68 years (range 47-87 years); males predominated (M:F=9:5). All presented with renal insufficiency, including five (36%) with acute kidney injury. The mean serum creatinine at biopsy was 2.9 mg/dl (range 1.2-7.3 mg/dl). All had proteinuria (mean 7.9 g/24 h; range 0.5-28; n=13), including 9 with ≥3 g/24 h. Renal extramedullary hematopoiesis appeared histologically as an interstitial infiltrate (n=12) and/or a perirenal infiltrate (n=3) or mass-like lesion (n=1). Five were misdiagnosed as interstitial nephritis. Concurrent glomerular disease was prevalent and included fibrillary-like glomerulonephritis (n=3), chronic thrombotic microangiopathy (n=5), focal segmental glomerulosclerosis (n=6), and diabetic glomerulosclerosis (n=2). All patients had an underlying hematologic malignancy: primary myelofibrosis in 9, myeloproliferative neoplasm not otherwise specified in 1, essential thrombocythemia in 1, polycythemia vera in 1, and plasma cell myeloma in 2. Clinical follow-up was available in 12 patients, mean of 29 months (range 4-120 months). In 10 patients for whom treatment history could be obtained, 9 were treated with chemotherapy, and 1 was treated with steroids. The mean creatinine at last follow-up was 2 mg/dl (range 1.2-3.9 mg/dl) (n=9). Ten patients died in the follow-up period from their underlying hematological disease and had persistent renal disease. The two remaining patients had persistent chronic kidney disease. Renal extramedullary hematopoiesis should be considered in the differential diagnosis of interstitial infiltrates, particularly in the presence of a glomerulopathy and a hematologic malignancy.

  17. Stereotactic radiotherapy for vestibular schwannoma

    DEFF Research Database (Denmark)

    Muzevic, Dario; Legcevic, Jelena; Splavski, Bruno

    2014-01-01

    BACKGROUND: Vestibular schwannomas (acoustic neuromas) are common benign tumours that arise from the Schwann cells of the vestibular nerve. Management options include observation with neuroradiological follow-up, microsurgical resection and stereotactic radiotherapy. OBJECTIVES: To assess...... the effect of stereotactic radiotherapy compared to observation, microsurgical resection, any other treatment modality, or a combination of two or more of the above approaches for vestibular schwannoma. SEARCH METHODS: We searched the Cochrane Central Register of Controlled Trials; PubMed; EMBASE; CINAHL......; Web of Science; CAB Abstracts; ISRCTN and additional sources for published and unpublished trials. The date of the search was 24 July 2014. SELECTION CRITERIA: Randomised controlled trials (RCTs) exploring the efficacy of stereotactic radiotherapy compared with observation alone, microsurgical...

  18. Gastric schwannoma: a case report

    Directory of Open Access Journals (Sweden)

    Hayfa Romdhane

    2016-11-01

    Full Text Available Schwannomas are generally benign, slow growing tumors. They are rarely observed in the gastrointestinal tract with the most common site being the stomach. These tumors are usually asymptomatic. The preoperative diagnosis via endoscopy is a challenging issue due to the difficulty of differentiation from other submucosal tumors. A 54-year-old woman presented with epigastric pain persisting for the last 10 months. Upper endoscopy revealed an elevated submucosal mass of the gastric antrum. The overlying mucosa was normal. Biopsy specimens yielded only unspecific signs of mild inactive chronic inflammation. Endoscopic ultrasound examination noted a hypoechoic homogeneous mass lesion located in the gastric antrum. The mass appeared to arise from the muscularis propria, and there was no perigastric lymphadenopathy. A contrast-enhanced computed tomography scan identified a homogeneous round mass and arising from the antrum of the stomach. Submucosal tumor was suspected and surgical intervention was recommended. The patient underwent an elective laparoscopic partial gastrectomy. The histopathologic features and immunohistochemical-staining pattern were consistent with a benign gastric schwannoma. Our patient shows no recurrence with a follow-up of one year. The definitive diagnosis of gastric schwannomas requires immunohistochemical studies. Complete margin negative surgical resection, as in this case, is the curative treatment of choice. The clinical course is generally benign.

  19. Gastric Schwannoma: A Case Report.

    Science.gov (United States)

    Romdhane, Hayfa; Cheikh, Myriam; Mzoughi, Zeineb; Slama, Sana Ben; Ennaifer, Rym; Belhadj, Najet

    2016-10-24

    Schwannomas are generally benign, slow growing tumors. They are rarely observed in the gastrointestinal tract with the most common site being the stomach. These tumors are usually asymptomatic. The preoperative diagnosis via endoscopy is a challenging issue due to the difficulty of differentiation from other submucosal tumors. A 54-year-old woman presented with epigastric pain persisting for the last 10 months. Upper endoscopy revealed an elevated submucosal mass of the gastric antrum. The overlying mucosa was normal. Biopsy specimens yielded only unspecific signs of mild inactive chronic inflammation. Endoscopic ultrasound examination noted a hypoechoic homogeneous mass lesion located in the gastric antrum. The mass appeared to arise from the muscularis propria, and there was no perigastric lymphadenopathy. A contrast-enhanced computed tomography scan identified a homogeneous round mass and arising from the antrum of the stomach. Submucosal tumor was suspected and surgical intervention was recommended. The patient underwent an elective laparoscopic partial gastrectomy. The histopathologic features and immunohistochemical-staining pattern were consistent with a benign gastric schwannoma. Our patient shows no recurrence with a follow-up of one year. The definitive diagnosis of gastric schwannomas requires immunohistochemical studies. Complete margin negative surgical resection, as in this case, is the curative treatment of choice. The clinical course is generally benign.

  20. Cellular Schwannoma Arising from Sigmoid Mesocolon Presenting ...

    African Journals Online (AJOL)

    Immunostaining showed diffuse S‑100 positivity [Figure 2, inset] and no reactivity for CD117, CD34,. SMA, CD31, HMB‑45, and desmin. A diagnosis of cellular schwannoma with torsion was conferred. Discussion. The schwannomas are typically found as solitary tumors. Presentation associated with schwannomatosis or.

  1. A rare adrenal incidentaloma: adrenal schwannoma.

    Science.gov (United States)

    Adas, Mine; Ozulker, Filiz; Adas, Gokhan; Koc, Bora; Ozulker, Tamer; Sahin, Ilknur Mansuroglu

    2013-01-01

    Adrenal schwannoma is an extremely uncommon cause of incidentaloma. It originates from neural sheath Schwann cells of the adrenal gland. We report the case of a left adrenal schwannoma incidentally discovered in a 32-year-old woman during examination of bloated feeling and stomach ache. The patient was incidentally found to have a left adrenal mass of 9 cm on abdominal ultrasonography. Computed tomography (CT) of the abdomen and [(18)F] fluorodeoxyglucose positron emission tomography (PET) were also performed. Metabolic evaluation was unremarkable. Due to the large size of the tumor, left adrenalectomy was performed. The postoperative course was uneventful. Histological examination established the diagnosis of schwannoma. This diagnosis was supported by immunohistochemistry of S-100 and vimentin positivity. In conclusion, adrenal schwannoma is an extremely rare entity and can grow considerably in size. The present case report emphasizes that clinicians should be aware of the possibility of retroperitoneal schwannoma. Total excision of benign schwannoma is associated with a favorable outcome. To our knowledge, there are case reports of schwannoma with CT and magnetic resonance imaging findings in the literature, although this is the first schwannoma case with PET-CT imaging.

  2. Temporal fossa intra-extracranial dumbbell schwannoma.

    LENUS (Irish Health Repository)

    Wong, Limy

    2011-02-01

    Intra-extracranial schwannomas arising unrelated to major cranial nerves are exceedingly rare neoplasms. We report the case of a 23-year-old male who presented with a 9 month history of progressive temporal swelling which was excised and found histologically to be a schwannoma. A succinct review of the relevant literature is presented.

  3. Cellular schwannoma arising from sigmoid mesocolon presenting ...

    African Journals Online (AJOL)

    We present a case of a 58‑year‑old female with a massive twisted tumor arising from sigmoid mesocolon. The tumor was diagnosed to be a case of cellular schwannoma, an exceedingly rare tumor in this location with rare presentation. Keywords: Pelvis, Retroperitoneum, Schwannoma, Sigmoid mesocolon, Torsion, Tumor ...

  4. Temporal fossa intra-extracranial dumbbell schwannoma.

    LENUS (Irish Health Repository)

    Wong, Limy

    2012-02-01

    Intra-extracranial schwannomas arising unrelated to major cranial nerves are exceedingly rare neoplasms. We report the case of a 23-year-old male who presented with a 9 month history of progressive temporal swelling which was excised and found histologically to be a schwannoma. A succinct review of the relevant literature is presented.

  5. Surgical treatment of an unusual case of pelvic extramedullary hematopoiesis.

    Science.gov (United States)

    Khen-Dunlop, Naziha; Girot, Robert; Brunelle, Francis; Révillon, Yann; Nihoul-Fékété, Claire; Sarnacki, Sabine

    2006-07-01

    Extramedullary hematopoiesis affects about 15% of the patients treated for thalassemia intermedia. Usually seen in adulthood, the most common location is the paraspinal region. Diagnosis and treatment of extramedullary hematopoiesis located in the pelvis of a young 15-year-old girl is discussed. The young age of the patient and the uncommon site of the mass first lead to the diagnosis of an ovarian dermoid cyst. Because of the clinical history and the typical feature on computed tomography scan, extramedullary hematopoiesis was concluded. A specific treatment based on blood transfusion and hydroxyurea was first proposed but remained inefficient. Surgical excision was thus successfully performed. Whereas surgery is limited to spinal cord compression in paraspinal extramedullary hematopoiesis, this observation argues for surgical treatment in symptomatic intraabdominal extramedullary hematopoiesis when medical treatment fails.

  6. Schwannoma intercostal simulando neoplasia pulmonar

    Directory of Open Access Journals (Sweden)

    Henn L.A.

    1998-01-01

    Full Text Available Schwannoma é uma neoplasia mesenquimal, usualmente solitária, encontrada no mediastino, retroperitônio ou pelve, sendo rara sua apresentação na parede torácica. OBJETIVO: Relatar o caso de um paciente masculino, tabagista, com um nódulo pulmonar direito com características de malignidade, detectado em radiografia de tórax de rotina, cujo diagnóstico anatomopatológico foi de schwannoma benigno de nervo intercostal. MÉTODOS: Revisaram-se os dados referentes ao quadro clínico, exames laboratoriais e de imagem (radiografia e tomografia computadorizada de tórax do caso em estudo, assim como os exames anatomopatológico e imuno-histoquímico do espécime cirúrgico. RESULTADOS: O paciente foi submetido à toracotomia direita diagnóstica com ressecção da tumoração. O exame anatomopatológico convencional mostrou células tumorais de aspecto fusiforme, dispostas em paliçada, formando os corpos de Verocay, compatível com schwannoma intercostal. A imuno-histoquímica foi positiva para proteína S-100, vimentina e enolase, e negativa para neurofilamentos. CONCLUSÃO: O diagnóstico definitivo de schwannoma só é possível por meio da análise histopatológica e imuno-histoquímica da lesão. Seu aspecto celular, associado à atividade mitótica e a áreas de pleomorfismo, pode levar ao diagnóstico incorreto de malignidade. A imuno-histoquímica, por meio da proteína S-100, é útil na caracterização da benignidade da lesão, já que não é detectada nas lesões malignas. Os schwannomas de parede torácica podem simular neoplasias pulmonares na radiografia e tomografia computadorizada de tórax.

  7. Intracranial Extramedullary Hematopoiesis in Beta-Thalassemia

    Energy Technology Data Exchange (ETDEWEB)

    Karki, Bivek; Xu, Yi Kai; Wu, Yuan Kui [Nan fang Hospital, Southern Medical University, Guangzhou (China); Tamrakar, Karuna [Zhujiang Hospital, Southern Medical University, Guangzhou (China)

    2012-03-15

    Extramedullary hematopoiesis (EMH) represents tumor-like proliferation of hemopoietic tissue which complicates chronic hemoglobinopathy. Intracranial EMH is an extremely rare occurrence. Magnetic resonance imaging (MRI) offers a precise diagnosis. It is essential to distinguish EMH from other extradural central nervous system tumors, because treatment and prognosis are totally different. Herein, we report the imaging findings of beta-thalassemia in a 13-year-old boy complaining of weakness of left side of the body and gait disturbance; CT and MRI revealed an extradural mass in the right temporoparietal region.

  8. Intracranial Extramedullary Hematopoiesis in Beta-Thalassemia

    International Nuclear Information System (INIS)

    Karki, Bivek; Xu, Yi Kai; Wu, Yuan Kui; Tamrakar, Karuna

    2012-01-01

    Extramedullary hematopoiesis (EMH) represents tumor-like proliferation of hemopoietic tissue which complicates chronic hemoglobinopathy. Intracranial EMH is an extremely rare occurrence. Magnetic resonance imaging (MRI) offers a precise diagnosis. It is essential to distinguish EMH from other extradural central nervous system tumors, because treatment and prognosis are totally different. Herein, we report the imaging findings of beta-thalassemia in a 13-year-old boy complaining of weakness of left side of the body and gait disturbance; CT and MRI revealed an extradural mass in the right temporoparietal region.

  9. Intracranial extramedullary hematopoiesis in beta-thalassemia.

    Science.gov (United States)

    Karki, Bivek; Xu, Yi-Kai; Tamrakar, Karuna; Wu, Yuan-Kui

    2012-01-01

    Extramedullary hematopoiesis (EMH) represents tumor-like proliferation of hemopoietic tissue which complicates chronic hemoglobinopathy. Intracranial EMH is an extremely rare occurrence. Magnetic resonance imaging (MRI) offers a precise diagnosis. It is essential to distinguish EMH from other extradural central nervous system tumors, because treatment and prognosis are totally different. Herein, we report the imaging findings of beta-thalassemia in a 13-year-old boy complaining of weakness of left side of the body and gait disturbance; CT and MRI revealed an extradural mass in the right temporoparietal region.

  10. Schwannoma of the stomach: a case report.

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    Singh, Aminder; Mittal, Ankur; Garg, Bhavna; Sood, Neena

    2016-01-15

    Schwannomas, also known as neurilemmomas, are benign slow-growing neoplasms originating from a Schwann cell sheath. These neoplasms are rare among the mesenchymal tumors of the gastrointestinal tract. In the stomach, Schwannomas only represent 0.2 % of all gastric tumors; this makes the presentation of a schwannoma in the stomach of a man in his seventh decade unusual. This case report highlights the rarity of a schwannoma at the greater curvature of the stomach because only a few cases have been reported in the literature. This case describes the importance of including gastric schwannomas in the differential diagnosis when preoperative assessment reveals a submucosal gastric mass with gastrointestinal stromal tumor as a leading differential diagnosis because of its common occurrence at this site. A 72-year-old man of Indian origin presented with a painless abdominal mass with nonspecific gastrointestinal upset. An endoscopy showed a submucosal lesion in his stomach measuring 4 × 3 × 2 cm. Histology revealed a benign spindle cell tumor. Immunohistochemistry confirmed the diagnosis of gastric schwannoma. He is on regular follow-up and doing well. It should be remembered that a schwannoma can present as a mass lesion in the stomach and mimic gastrointestinal stromal tumor. Patients should undergo an endoscopy and a biopsy of the lesion should be done. Many patients do not undergo endoscopy which can delay diagnosis and management. As these tumors have an excellent prognosis, surgical removal is sufficient treatment. Surgeons, radiologists, pathologists and gastroenterologists must be aware of this entity.

  11. MRI Findings of Early-Stage Hyperacute Hemorrhage Causing Extramedullary Compression of the Cervical Spinal Cord in a Dog with Suspected Steroid-Responsive Meningitis-Arteritis

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    Adriano Wang-Leandro

    2017-09-01

    Full Text Available A 9-month-old female Weimaraner was presented to the emergency service due to episodes of fever and neck pain. Physical examination revealed a stiff neck posture and elevated body temperature. Shortly after clinical examination was performed, the dog developed peracute onset of non-ambulatory tetraparesis compatible with a C1–C5 spinal cord (SC lesion. Immediately thereafter (<1 h, MRI of the cervical SC was performed with a 3-T scanner. A left ventrolateral intradural-extramedullary SC compression caused by a round-shaped structure at the level of C3––C4 was evidenced. The structure was iso- to slightly hyperintense in T1-weighted (T1W sequences compared to SC parenchyma and hyperintense in T2-weighted, gradient echo, and fluid-attenuated inversion recovery. Moreover, the structure showed a strong homogeneous contrast uptake in T1W sequences. Cerebrospinal fluid (CSF analysis revealed a mixed pleocytosis, as well as elevated protein and erythrocyte count. Early-stage hyperacute extramedullary hemorrhage was suspected due to immune mediated vasculitis. The dog was maintained under general anesthesia and artificial ventilation for 24 h and long-term therapy with corticosteroids and physiotherapy was initiated. Eight weeks after initial presentation, the dog was ambulatory, slightly tetraparetic. Follow-up MRI showed a regression of the round-shaped structure and pleocytosis was not evident in CSF analysis. This report describes an early-stage hyperacute extramedullary hemorrhage, a condition rarely recorded in dogs even in experimental settings.

  12. Intracochlear Schwannoma: Diagnosis and Management

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    Bittencourt, Aline Gomes

    2014-01-01

    Full Text Available Introduction Schwannomas of the eighth cranial nerve are benign tumors that usually occur in the internal auditory canal or the cerebellopontine angle cistern. Rarely, these tumors may originate from the neural elements within the vestibule, cochlea, or semicircular canals and are called intralabyrinthine schwannomas. Intracochlear schwannomas (ICSs represent a small percentage of these tumors, and their diagnosis is based on high-resolution magnetic resonance imaging (MRI. Objectives To report the clinical and radiologic features and audiometric testing results of an ICS in a 48-year-old man after a 22-month follow-up period. Resumed Report A patient with an 8-year history of persistent tinnitus in his right ear, combined with ipsilateral progressive hearing loss and aural fullness. Audiometry revealed normal hearing in the left ear and a moderate to severe sensorineural hearing loss in the right ear, with decreased speech reception threshold and word recognition score, compared with the exam performed 5 years previously. MRI showed a small intracochlear nodular lesion in the modiolus, isointense on T1 with a high contrast enhancement on T1 postgadolinium images. During the follow-up period, there were no radiologic changes on imaging studies. Thus, a wait-and-scan policy was chosen as the lesion remained stable with no considerable growth and the patient still presents with residual hearing. Conclusions Once diagnosed, not all ICS patients require surgery. Treatment options for ICS include stereotactic radiotherapy and rescanning policy, depending on the tumor's size, evidence of the tumor's growth, degree of hearing loss, intractable vestibular symptoms, concern about the pathologic diagnosis, and the patient's other medical conditions.

  13. Gastric Schwannoma: A Case Report

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    S. Shariat-Torbaghan

    2008-02-01

    Full Text Available Gastrointestinal mesenchymal tumors are a group of tumors originating from the mesenchymal stem cells of the GI tract. Digestive tract Schwannomas are rare mesenchymal tumors which occur most frequently in the stomach.We report a 56-yearold woman who was examined endoscopically for dyspepsia which she had suffered from since 3 years ago.Around gastric antral mass was seen.Surgical resection was recommended.The pathological examination revealed a spindle cell tumor that was strongly positive for S-100 protein stain and non-reactive for other markers.The literature is reviewed. 

  14. Rapid progressive extramedullary plasmacytoma in the orbit.

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    Knecht, P; Schuler, R; Chaloupka, K

    2008-05-01

    Plasma cell tumors of the orbit are uncommon and mostly the first manifestation of a multiple myeloma (MM). Primary extramedullary plasmacytoma (EMP) of the orbit is exceedingly rare and only a few cases have been reported so far. Differentiation of an EMP from a MM is important, since 9.5 years have been reported as the mean survival of EMP, compared with a mean survival of 3 to 4 years for multiple myeloma. A 68-year-old man presented with a history of rapid progressive extramedullary plasmacytoma in the right orbit, increasing in size within a few weeks. MR imaging revealed an intraorbital mass measuring 5.2 x 4.7 x 3 cm. The earlier performed biopsy was positive for plasmacytoma of the lambda light chain type. Systemic work-up was negative for further tumor spread. Irradiation (a maximum of 80 Gy) and several chemotherapy cycles showed no effect. Exenteration with sparing of the upper and lower lid skin was performed. The residual defect was covered by a Thiersch graft. Although the orbit had been irradiated with the maximal dose, the Thiersch graft healed well. Normally, EMP is slow to increase in size. In our patient, marked growth of tumor was noted within one week. One underlying reason is the poorly differentiated, blastic morphology leading to this rapid tumor growth. However, particular molecular features, like a t(4;14), could not be demonstrated.

  15. The Specific Sagittal Magnetic Resonance Imaging of Intradural Extra-Arachnoid Lumbar Disc Herniation

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    Tatsuro Sasaji

    2012-01-01

    Full Text Available Intradural extra-arachnoid lumbar disc herniation is a rare disease. Few MRI findings have been reported. We experienced an intradural extra-arachnoid lumbar disc herniation. We reviewed the preoperative MRI findings. Lumbar spine T2-weighted sagittal MRI showed that one line of the ventral dura was divided into two by a disc herniation. We speculated that the two lines comprised the dura and arachnoid and that a disc herniation existed between them. We believe that division of the ventral dural line on T2-weighted sagittal images is a characteristic finding of intradural extra-arachnoid lumbar disc herniation. The division of ventral dural line seemed to be a “Y,” and, thus, we called it the “Y sign.” The “Y sign” may be useful for diagnosing intradural extra-arachnoid lumbar disc herniation.

  16. The Specific Sagittal Magnetic Resonance Imaging of Intradural Extra-Arachnoid Lumbar Disc Herniation

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    Sasaji, Tatsuro; Horaguchi, Kiyoshi; Yamada, Noboru; Iwai, Kazuo

    2012-01-01

    Intradural extra-arachnoid lumbar disc herniation is a rare disease. Few MRI findings have been reported. We experienced an intradural extra-arachnoid lumbar disc herniation. We reviewed the preoperative MRI findings. Lumbar spine T2-weighted sagittal MRI showed that one line of the ventral dura was divided into two by a disc herniation. We speculated that the two lines comprised the dura and arachnoid and that a disc herniation existed between them. We believe that division of the ventral dural line on T2-weighted sagittal images is a characteristic finding of intradural extra-arachnoid lumbar disc herniation. The division of ventral dural line seemed to be a “Y,” and, thus, we called it the “Y sign.” The “Y sign” may be useful for diagnosing intradural extra-arachnoid lumbar disc herniation. PMID:22431932

  17. The Specific Sagittal Magnetic Resonance Imaging of Intradural Extra-Arachnoid Lumbar Disc Herniation

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    Sasaji, Tatsuro; Horaguchi, Kiyoshi; Yamada, Noboru; Iwai, Kazuo

    2012-01-01

    Intradural extra-arachnoid lumbar disc herniation is a rare disease. Few MRI findings have been reported. We experienced an intradural extra-arachnoid lumbar disc herniation. We reviewed the preoperative MRI findings. Lumbar spine T2-weighted sagittal MRI showed that one line of the ventral dura was divided into two by a disc herniation. We speculated that the two lines comprised the dura and arachnoid and that a disc herniation existed between them. We believe that division of the ventral du...

  18. Episodic cauda equina compression from an intradural lumbar herniated disc: a case of 'floppy disc'.

    Science.gov (United States)

    Nagaria, J; Chan, Cc; Kamel, Mh; McEvoy, L; Bolger, C

    2011-09-01

    Intradural disc herniation (IDDH) is a rare complication of intervertebral disc disease and comprises 0.26-0.30% of all herniated discs, with 92% of them located in the lumbar region (1). We present a case of IDDH that presented with intermittent symptoms and signs of cauda equina compression. We were unable to find in the literature, any previously described cases of intermittent cauda equina compression from a herniated intradural disc fragment leading to a "floppy disc syndrome". © JSCR.

  19. Posterior Laminoplastic Laminotomy Combined with a Paraspinal Transmuscular Approach for Removing a Lumbar Dumbbell-shaped Schwannoma: A Technical Note.

    Science.gov (United States)

    Ngerageza, Japhet Gideon; Ito, Kiyoshi; Aoyama, Tatsuro; Murata, Takahiro; Horiuchi, Tetsuyoshi; Hongo, Kazuhiro

    2015-01-01

    The surgical strategies and methods used to treat dumbbell-shaped tumors located in the lumbar-foraminal region are controversial. Although a total facetectomy and combined intra- and extraspinal canal approach provide a wide operative field, facet fusion is required, which can be rather invasive. Here, we report a successful removal of a lumbar dumbbell-shaped schwannoma using a combined laminoplastic laminotomy with Wiltse's paraspinal surgical approach. This was performed under an operating microscope without a complete facetectomy, fusion, and posterior fixation. Briefly, we treated two patients with lumbar foraminal tumors, both dumbbell-shaped schwannomas located in the intra- and extradural portion. After a laminoplastic laminotomy, the intradural tumor was removed. The tumor located at the extracanalicular site was removed after drilling the pars interarticularis of the lamina, which was performed to enlarge the intervertebral foramen via Wiltse's paraspinal surgical approach. During surgery, facetectomy with posterior fixation was not needed to remove the intraforaminal component. There was no lumbar instability or complication after surgery. Our results suggest that a combined posterior laminoplastic laminotomy and Wiltse's paraspinal surgical approach is useful and less invasive for treating patients with lumbar foraminal tumors.

  20. Vestibular Schwannoma or acoustic neuroma

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    Hekmatara M

    1997-04-01

    Full Text Available Vestibular schwannoma is the most common tumor of the posterior fossa of the skull. Patients referred with the primary otologic symptoms such as hearing loss, tinnitus, vertigo, imbalance, and the cranial nerve palsy. Thirty-three patients were operated and treated by a team of otolaryngologist and neurosurgeon, anudiometrist, and internist. Patients'chiefcomplaint was due to 94% hearing loss and 27% tinnitus. They scarcely complain of vertigo. If a patient refers with the palsy or paralysis of facial nerve preoperation, we must think of the facial nerve schwannoma or hemangioma or congential cholestoma or malignant metastases rather than acoustic neuroma. The best way for preoperative diagnosis is audiometry, ABR (Auditory Brain Response, and SDS (speech discrimination score with 90% success, but computer Tomography (CT scan and MRI (Magnetic Resonance Image are the valuable anatomic diagnostic radiographic devices. The best method of operation is translabirynthine approach (TLA, since it has the advantages such as an easy access to nerve paths and being the nearest path to CPA (Cerebellopontine Angle. Physicians ought to talk to patients about the importance of the microscopic surgery, surgical methods, and their probable diverse effects such as hearing loss, facial nerve palsy, and intracranial problems.

  1. [Gastric schwannoma: a "typical" clinical course?].

    Science.gov (United States)

    Kessler, W; Schreiber, A; Glitsch, A; Evert, M; Puls, R; Patrzyk, M; Heidecke, C-D

    2009-01-01

    Intra-abdominal schwannoma is a rare tumor entity. Although often detected incidentally, its diagnosis and surgical planning are difficult-as with all intramural intra-abdominal tumors. Puncturing is often not satisfying due to the inhomogeneous proliferation rates of different regions of the tumor. We describe the procedure using the example of a gastric schwannoma that was found incidentally. The leading symptom was perforation of a peptic stomach ulcer.

  2. Gastric schwannoma: CT findings and clinicopathologic correlation.

    Science.gov (United States)

    Ji, Jian-song; Lu, Chen-ying; Mao, Wei-bo; Wang, Zu-fei; Xu, Min

    2015-06-01

    The purpose of this study was to evaluate the computed tomography (CT) imaging characteristics of gastric schwannoma. Eight cases of gastric schwannomas confirmed by surgery and pathology were retrospectively analyzed by CT. We reviewed the CT findings of gastric schwannomas for the following characteristics: tumor location, size, contour, margin, growth pattern, enhancement pattern, the presence or absence of necrosis, and perigastric lymph nodes. The tumors were located in the lesser curvature of gastric body (n = 5) and greater curvature of the gastric antrum (n = 3) with a median size of 4.8 cm (range 1.7-11.4 cm). Gastric schwannomas appeared as submucosal tumors with CT features of ovoid (7/8 patients), well-defined (8/8) and exophytic (4/8) or mixed (3/8) growth patterns. On dynamic CT examination, the tumors displayed homogeneous enhancement in seven cases and heterogeneous enhancement in one case. Solid parts of eight tumors demonstrated mild enhancement during the arterial phase and strengthened progressive enhancement during the venous and delayed phases. Two cases had perigastric lymph nodes. Gastric schwannomas typically manifested as ovoid, well-defined, exophytic, or mixed growth pattern masses on CT. Homogeneous progressive enhancement on dynamic CT is a characteristic finding of gastric schwannoma.

  3. Spinal extradural arteriovenous fistula with intradural drainage: three surgical cases with long-term follow-up.

    Science.gov (United States)

    Vázquez, Enrique J; Gefaell, Joaquín; Fernandez, Leticia; López, Eva; Areitio, Eduardo; Elexpuru, Jose A

    2017-06-01

    Spinal extradural arteriovenous fistulas (SEDAVF) with intradural drainage are uncommon vascular lesions that cause venous congestive myelopathy. We present three SEDAVF with intradural drainage treated with surgical disconnection of the intradural drainage via a single level laminectomy, followed by transarterial embolization (TAE) with Onyx if spontaneous thrombosis of the extradural fistula did not occur spontaneously. All patients improved their neurological condition and no congestive myelopathy recurrence was noted after mean follow-up of 84 months. We believe that SEDAVF with intradural drainage constitute a specific entity that should be included in the classifications of spinal arteriovenous malformations.

  4. Extramedullary hematopoiesis: A report of two cases.

    Science.gov (United States)

    Zhang, Huan-Zhu; Li, Ying; Liu, Xin; Chen, Bao-Rong; Yao, Guo-Hua; Peng, Yu-Na

    2016-12-01

    Extramedullary hematopoiesis (EMH) is defined as hematopoiesis occurring in organs outside of the bone marrow. The present report describes two cases of thalassemic patients with paraspinal medullary hematopoiesis and analyzes the clinical manifestations, imaging, pathology, diagnosis and treatment of EMH. In addition, a supplementary review of previously published cases is provided along with a review of the related literature. Computed tomography (CT) of the first case revealed multiple paraspinal masses, and the largest was 6.2×8.0 cm in diameter. Likewise, CT of the second patient revealed multiple paraspinal masses in the bottom of the left thoracic cavity, and the largest was measured 10.1×10.5 cm. The two cases underwent surgical biopsy and the findings were compatible with a diagnosis of EMH. In conclusion, EMH is a compatible and rare disease, and should be distinguished from other neoplasms. EMH must considered when masses with characteristic radiologic appearance are detected in patients with thalassemia intermedia.

  5. Fourth Ventricular Schwannoma: Identical Clinicopathologic Features as Schwann Cell-Derived Schwannoma with Unique Etiopathologic Origins

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    Tiffany R. Hodges

    2011-01-01

    Full Text Available Background. To our knowledge, this is the sixth reported case in the literature of fourth ventricular schwannoma. The etiology and natural history of intraventricular schwannomas is not well understood. A thorough review of potential etiopathogenic mechanisms is provided in this case report. Case Description. A 69-year-old man presented with an incidentally found fourth ventricular tumor during an evaluation for generalized weakness, gait instability, and memory disturbance. Magnetic resonance imaging (MRI revealed a heterogeneously enhancing lesion in the fourth ventricle. A suboccipital craniotomy was performed to resect the lesion. Histopathological examination confirmed the diagnosis of schwannoma (WHO grade I. Conclusions. Schwannomas should be considered in the differential diagnosis of intraventricular tumors. Although the embryologic origins may be different from nerve sheath-derived schwannomas, the histologic, clinical, and natural history appear identical and thus should be managed similarly.

  6. ANTERIOR CERVICAL INTRADURAL ARACHNOID CYST - A RARE CAUSE OF SPINAL CORD COMPRESSION

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    Kollam Chandra Sekhar

    2016-07-01

    Full Text Available BACKGROUND Arachnoid cysts of spinal cord are relatively uncommon lesions. Most of them arise dorsal to the cord, and anteriorly placed intradural arachnoid cyst is a rare cause of cervical cord compression. To the best of our knowledge, only 30 cases were reported in the literature. We present a case of anterior cervical intradural arachnoid cyst with review of literature. METHODS We performed a literature search for anteriorly placed intradural arachnoid cysts in the cervical spinal cord through http://pubmed.com, a well-known worldwide internet medical address. To the best of our knowledge, only 30 cases were reported in the literature. We reviewed the literature with illustration of our case. We present a case of a 40-year-old male patient who presented with insidious onset of radicular pain. MRI cervical spine demonstrated cervical intradural cystic lesion extending from C2 to upper border of C4, lying anteriorly with compression over the cord. Cervical laminectomy followed by wide cyst fenestration and subtotal excision of cyst was done. Histopathological diagnosis was arachnoid cyst. RESULTS Patient totally recovered from his pain and sensory symptoms within a week and motor symptoms improved gradually over a period of six to eight weeks. With two years followup, patient had no further complaints. CONCLUSION Anterior cervical intradural arachnoid cysts are rare. These are amenable to resection through posterior approach safely with good postoperative recovery.

  7. Diagnosis and management of retroperitoneal ancient schwannomas

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    Gusani Niraj J

    2009-02-01

    Full Text Available Abstract Background Ancient schwannomas are degenerate peripheral nerve sheath tumors that very rarely occur in the retroperitoneum. They generally reach large proportions before producing symptoms due to mass effect. We describe three cases of retroperitoneal ancient schwannomas and discuss the diagnosis and management of these tumors. Case presentations Three female patients with retroperitoneal ancient schwannomas were reviewed. One patient presented with several weeks of upper abdominal pain and lower chest discomfort, whereas back pain and leg pain with associated weakness were predominant symptoms in the remaining two. Abdominal imaging findings demonstrated heterogeneous masses in the retroperitoneum with demarcated margins, concerning for malignancy. The patients successfully had radical excision of their tumors. Histological examination showed encapsulated tumors that displayed alternating areas of dense cellularity and areas of myxoid matrix consistent with a diagnosis of ancient schwannoma. Conclusion A diagnosis of ancient schwannoma should be entertained for any heterogeneous, well encapsulated mass in the retroperitoneum. In these cases less radical surgical resection should be considered as malignant transformation of these tumors is extremely rare and recurrence is uncommon following excision.

  8. EXTRACRANIAL HEAD AND NECK SCHWANNOMA: CASE REPORT

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    Ashok

    2015-03-01

    Full Text Available Schwannoma is a benign nerve sheath tumour composed of Schwan cells which normally produce the insulating myelin sheath covering peripheral nerves. Schwannoma is a homogenous tumour, consisting only of schwan cells. The tumour cells stay outside the nerve, but the tumour itself may either push the nerve aside or up against a bony structure there by producing nerve damage. They arise from peripheral, cranial and autonomic nerves and usually present as solitary well demarcated lesions. Extracranial Head and Neck schwannomas are rare tumours. They may produce secondary symptoms like nasal obstruction, dysphagia, and hoarseness of voice depending upon the location of the tumour. FNAC, Ultra sound, CT, MRI may be of limited help in the diagnosis. Complete surgical excision is the treatment of choice. Post operative histopa thological examination establishes the final diagnosis

  9. Collet-Sicard Syndrome Attributable to Extramedullary Plasmacytoma of the Jugular Foramen.

    Science.gov (United States)

    Oushy, Soliman; Graffeo, Christopher S; Perry, Avital; Morris, Jonathan M; Carlson, Matthew L; Van Gompel, Jamie J

    2018-02-01

    Collet-Sicard syndrome is a rare manifestation of skull base disease involving the jugular and hypoglossal foramina. We report the first case of Collet-Sicard attributable to extramedullary plasmacytoma-multiple myeloma (EP-MM) and the second case of EP-MM precipitating a jugular foramen syndrome (JFS)-spectrum disorder. A 59-year-old woman presented with 4 months of left aural fullness and pulsatile tenderness, positional vertigo, hoarseness, and dysphagia. Examination identified left tongue weakness and nonspecific sensory abnormalities of the ear, pharynx, and throat localizing to cranial nerves IX-XII. Imaging revealed a 3.4 × 1.4 × 2.8 cm lytic lesion extending from the left jugular foramen into the posterior fossa, for which she was referred to neurosurgery and otolaryngology for consideration of resection. A second, much smaller (1.1-cm) lytic lesion in the left posterior occipital bone was incidentally discovered by the surgeon during preoperative consultation. A stereotactic biopsy of the occipital lesion was subsequently recommended, which identified plasma cell neoplasm. Serum studies and skeletal survey were consistent with MM, and a definitive pathologic diagnosis of MM with cranial EP was confirmed by bone marrow biopsy. Tumors of the jugular foramen present with a diverse array of lower cranial nerve deficits, including Collet-Sicard syndrome, a rare subset of JFS-spectrum diseases. Paragangliomas are the most common jugular foramen neoplasms, followed by schwannomas and meningiomas; however, many other rare entities have been reported as masqueraders, and diligent work-up with consideration for preliminary biopsy is recommended, particularly in the presence of additional lesions, equivocal imaging findings, or cases arousing high suspicion. Copyright © 2017 Elsevier Inc. All rights reserved.

  10. [Clinical and pathologic features of gastric schwannoma].

    Science.gov (United States)

    Wang, Zhan-bo; Shi, Huai-yin; Yuan, Jing; Chen, Wei; Wei, Li-xin

    2012-02-01

    To study the clinical and pathologic features of gastric schwannomas. The macroscopic and microscopic features of 9 cases of gastric schwannoma were analyzed. Immunohistochemical study for S-100 protein, CD117, CD34, neurofilament, desmin, nestin, glial fibrillary acidic protein, platelet derived growth factor-alpha (PDGFR-α) and vimentin was carried out. Mutation analysis of c-kit gene (exon 9, 11, 13 and 17) and PDGFR-α gene (exon 12 and 18) in 1 case was examined by PCR amplification and direct sequencing. The patients included 5 males and 4 females. The age of patients ranged from 42 to 81 years (median = 56.5 years). The size of the tumors ranged from 2 to 9 cm in greatest diameter. Follow-up data in 8 cases (from 1 month to 65 months) showed no evidence of recurrence or metastasis. Gross examination showed that gastric schwannomas were homogeneous, firm, yellow-white and bore no true fibrous capsule. Histologically, all cases were composed of fascicles of spindle cells associated with nuclear palisading, Verocay body formation and peripheral cuff of reactive lymphoid aggregates. Some of them showed degenerative changes including cyst formation, calcification, hemorrhage, necrosis and hyalinization. Immunohistochemical study showed that the tumor cells were strongly positive for S-100 protein and vimentin. There was various degree of staining for nestin (8/9) and glial fibrillary acidic protein (6/9). They were negative for CD117, CD34, neurofilament, desmin and smooth muscle actin. One case showed focal positivity for PDGFR-α (1/9), with no mutations found. Gastric schwannomas share similar histologic features with conventional soft tissue schwannomas, in addition to the presence a reactive lymphoid cuff. The clinical, macroscopic, histologic and immunohistochemical features of gastric schwannomas were different from those of gastrointestinal stromal tumors and leiomyomas.

  11. Conus medullaris syndrome due to an intradural disc herniation: A case report

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    Chaudhary Kshitij

    2008-01-01

    Full Text Available A 70-year-old male patient developed acute paraplegia due to conus medullaris compression secondary to extrusion of D12-L1 disc. After negative epidural examination intraoperatively, a durotomy was performed and an intradural disc fragment was excised. Patient did not regain ambulatory status at two-year follow-up. Intraoperative finding of negative extradural compression, tense swollen dura and CSF leak from ventral dura should alert the surgeon for the possibility of intradural disc herniation. A routine preoperative MRI is misleading and a high index of suspicion helps to avoid a missed diagnosis.

  12. [A case of cutaneous extramedullary hematopoiesis associated with idiopathic myelofibrosis].

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    Corella, F; Barnadas, M A; Bordes, R; Curell, R; Espinosa, I; Vergara, C; Alomar, A

    2008-05-01

    Cutaneous extramedullary hematopoiesis is a rare manifestation of chronic myeloproliferative processes, mainly chronic idiopathic myelofibrosis. In adults, it manifests as macules, papules, nodules, and ulcers on the trunk. The lesions usually appear soon after diagnosis and the possibility of a relationship between splenectomy and the appearance of extramedullary foci of hematopoiesis is still debated. Diagnosis is based on histopathology showing an infiltrate with different combinations of myeloid and erythroid cell precursors and megakaryocytes. Symptomatic treatment is provided alongside treatment of the underlying disease. We report a new case associated with chronic idiopathic myelofibrosis in which foci of cutaneous extramedullary hematopoiesis were observed 9 years after initial diagnosis. The lesions were progressive and the patient went on to develop acute myeloid leukemia.

  13. Extramedullary plasmacytoma in the trachea of a dog.

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    Chaffin, K; Cross, A R; Allen, S W; Mahaffey, E A; Watson, S K

    1998-05-15

    A 10-year-old spayed female mixed-breed dog was examined because of acute inspiratory dyspnea. Radiography and tracheoscopy revealed a discrete, solitary mass originating from the membranous portion of the trachea at the level of the thoracic inlet. Tracheal resection and anastomosis were performed, and on histologic examination of the resected tissue, extramedullary plasmacytoma was diagnosed. Although tracheal tumors are rare in dogs, they should be considered during evaluation of dogs with signs of airway obstruction. Prognosis is excellent for dogs with extramedullary plasmacytoma in which surgical excision is complete.

  14. THREE YEARS STUDY OF SCHWANNOMAS OF PERIPHERAL NERVES

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    Subha Dhua

    2017-02-01

    Full Text Available BACKGROUND In this paper authors present three cases of schwannomas including a case of multiple schwannomas without the features of neurofibromatosis (NF. There was no family history of neurofibromatosis. All the patients underwent surgical excision and improved from the symptomatic lesions. Histopathology confirmed these lesions as schwannomas. The authors recommend surgery for symptomatic lesions. Asymptomatic tumours can be monitored. Regular follow up is essential as they may develop fresh lesions at any time. The relevant literature is discussed. • Malignant transformation of the schwannomas is rare and has poor prognosis. It should be considered in the differential diagnosis of schwannomas. • We should distinguish between “ancient schwannoma” and malignant transformation of schwannoma since treatment and prognosis vary. • Imaging is not entirely reliable in differentiating benign from malignant peripheral nerve tumours. MATERIALS AND METHODS All the patients underwent surgical excision and improved from the symptomatic lesions. Histopathology confirmed these lesions as schwannomas. The authors recommend surgery for symptomatic lesions. RESULTS The histopathological studies confirmed the lesion as Flexi Schwannoma and surgery was considered to be the best option. CONCLUSION Schwannomas and meningiomas are usually benign tumours curable by complete removal. They occur either as single sporadic tumors in otherwise healthy individuals in the fourth to sixth decades of life or as multiple tumours at an early age as part of the autosomal dominant genetic disorder neurofibromatosis 2 (NF2. The hallmark feature of NF2 is bilateral vestibular schwannomas. Multiplicity, a lobular growth pattern, and invasiveness are typical features of NF2 schwannomas. The diagnosis of NF2 is difficult in a group of heterogeneous and poorly defined patients who do not have BVSs but present with other features suggestive of NF2, namely (1 multiple

  15. Complications of Microsurgery of Vestibular Schwannoma

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    Jan Betka

    2014-01-01

    Full Text Available Background. The aim of this study was to analyze complications of vestibular schwannoma (VS microsurgery. Material and Methods. A retrospective study was performed in 333 patients with unilateral vestibular schwannoma indicated for surgical treatment between January 1997 and December 2012. Postoperative complications were assessed immediately after VS surgery as well as during outpatient followup. Results. In all 333 patients microsurgical vestibular schwannoma (Koos grade 1: 12, grade 2: 34, grade 3: 62, and grade 4: 225 removal was performed. The main neurological complication was facial nerve dysfunction. The intermediate and poor function (HB III–VI was observed in 124 cases (45% immediately after surgery and in 104 cases (33% on the last followup. We encountered disordered vestibular compensation in 13%, permanent trigeminal nerve dysfunction in 1%, and transient lower cranial nerves (IX–XI deficit in 6%. Nonneurological complications included CSF leakage in 63% (lateral/medial variant: 99/1%, headache in 9%, and intracerebral hemorrhage in 5%. We did not encounter any case of meningitis. Conclusions. Our study demonstrates that despite the benefits of advanced high-tech equipment, refined microsurgical instruments, and highly developed neuroimaging technologies, there are still various and significant complications associated with vestibular schwannomas microsurgery.

  16. Spontaneous Cervical Intradural Disc Herniation Associated with Ossification of Posterior Longitudinal Ligament

    Directory of Open Access Journals (Sweden)

    Dachuan Wang

    2014-01-01

    Full Text Available Intradural herniation of a cervical disc is rare; less than 35 cases have been reported to date. A 52-year-old man with preexisting ossification of posterior longitudinal ligament developed severe neck pain with Lt hemiparesis while asleep. Neurological exam was consistent with Brown-Séquard syndrome. Magnetic resonance images showed a C5-6 herniated disc that was adjacent to the ossified ligament and indenting the cord. The mass was surrounded by cerebrospinal fluid signal intensity margin, and caudally the ventral dura line appears divided into two, consistent with the “Y-sign” described by Sasaji et al. Cord edema were noted. Because of preexisting canal stenosis and spinal cord at risk, a laminoplasty was performed, followed by an anterior C6 corpectomy. Spot-weld type adhesions of the posterior longitudinal ligament to the dura was noted, along with a longitudinal tear in the dura. An intradural extra-arachnoid fragment of herniated disc was removed. Clinical exam at 6 months after surgery revealed normal muscle strength but persistent mild paresthesias. It is difficult to make a definite diagnosis of intradural herniation preoperatively; however, the clinical findings and radiographic signs mentioned above are suggestive and should alert the surgeon to look for an intradural fragment.

  17. Intracranial schwannoma presenting as a subfrontal tumor: case report.

    Science.gov (United States)

    Huang, P P; Zagzag, D; Benjamin, V

    1997-01-01

    Intracerebral schwannomas not associated with cranial nerves account for less than 1% of surgically treated schwannomas of the central and peripheral nervous system. Subfrontal schwannomas are extremely rare, with only 15 cases reported to date. A 33-year-old man presented with a 4-month history of progressive headaches and lethargy. Radiographic studies revealed a large subfrontal tumor thought to be a meningioma preoperatively. The patient underwent a craniotomy for resection of his tumor. Intraoperatively, a large extra-axial tumor arising from the floor of the left frontal fossa was encountered. Microscopic examination of the tumor revealed a schwannoma. Several theories on the possible origin of intracerebral schwannomas have been considered. Because of the age of the patient at presentation, many authors have postulated a developmental origin for these lesions. However, extra-axial schwannomas not associated with cranial nerves often present later in life, suggesting a different pathogenesis for this subgroup.

  18. Solitary extramedullary plasmacytoma of the colon, rectum and anus ...

    African Journals Online (AJOL)

    We report an unusual case of SEP of the colon in an human immunodeficiency virus (HIV)-infected patient. The patient was managed with colonic resection and made an uneventful recovery. To our knowledge, this is the first reported case of extramedullary plasmacytoma of the colon and rectum in association with HIV ...

  19. Extramedullary leukemia in children with acute myeloid leukemia

    DEFF Research Database (Denmark)

    Støve, Heidi Kristine; Sandahl, Julie Damgaard; Abrahamsson, Jonas

    2017-01-01

    BACKGROUND: The prognostic significance of extramedullary leukemia (EML) in childhood acute myeloid leukemia is not clarified. PROCEDURE: This population-based study included 315 children from the NOPHO-AML 2004 trial. RESULTS: At diagnosis, 73 (23%) patients had EML: 39 (12%) had myeloid sarcoma...

  20. Intracranial extramedullary hematopoiesis: a rare cause of headaches.

    Science.gov (United States)

    Singer, Adam; Quencer, Robert

    2014-01-01

    Ectopic bone marrow production, known as extramedullary hematopoiesis, may result in symptoms due to compression on normal structures. We present the multimodality imaging findings and subsequent management of a rare case of symptomatic extramedullary hematopoiesis within the calvarium. Case report. A 54-year-old male with a history of myelofibrosis and no previous diagnosis of a headache disorder presented to the emergency department with worsening severe bilateral headaches. A nonenhanced CT of the brain was performed and diffuse extra-axial nodular hyperdensities were visualized. MRI of the brain demonstrated diffuse extra-axial avidly enhancing nodular masses, dural thickening and marked susceptibility. No paravertebral masses, typical for extramedullary hematopoiesis, were present in the chest or abdomen. Although the clinical team considered a biopsy to confirm the diagnosis, we suggested a noninvasive confirmatory test. The subsequent Tc99m sulfur colloid scan corroborated the diagnosis. The patient was then referred to radiation oncology for treatment. In summary, extramedullary hematopoiesis is a hematologic compensatory disorder that rarely occurs within the CNS and may cause neurological compromise due to compression on underlying structures. The diagnosis can be made with noninvasive imaging and treated with low dose radiation therapy. Copyright © 2013 by the American Society of Neuroimaging.

  1. Cervical Intramedullary Schwannoma: Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    José Omar Navarro Fernández

    2018-02-01

    Full Text Available Cervical intramedullary schwannomas are extraordinarily rare. Gross total resection is the best therapeutic option for these types of tumors. Although rare, intramedullary schwannomas should be considered as a differential diagnosis of intramedullary lesions since a good prognosis can be guaranteed to the majority of these patients. We present a case of a cervical intramedullary schwannoma surgically treated in a 19-year-old male patient who initially presented with motor neuron disease.

  2. H. Pylori in a gastric schwannoma: a case report

    OpenAIRE

    Lavy, Daniel S.; Paulin, Ethan T.; Parker, Mitchell I.; Zhang, Bin; Parker, Glenn S.; Schwartz, Mark R.

    2016-01-01

    Schwannomas are benign, often asymptomatic, slow-growing tumors that originate from Schwann cells of the neural sheath. Although H. Pylori has been associated with gastric adenocarcinoma, there has never been a recorded association with schwannoma formation. We present a 64-year-old woman who underwent a laparoscopic partial wedge gastrectomy for an incidentally discovered gastric mass. Histologic examination was consistent with schwannoma; however, chronic inflammation with microorganisms mo...

  3. Gastric schwannoma: a case report and literature review.

    Science.gov (United States)

    Atmatzidis, S; Chatzimavroudis, G; Dragoumis, D; Tsiaousis, P; Patsas, A; Atmatzidis, K

    2012-07-01

    Schwannomas are generally benign, slow growing tumors, which can originate from any nerve that has a Schwann cell sheath. Digestive tract schwannomas are rare and are usually asymptomatic. We present the case of a 48-year-old woman with a symptomatic submucosal tumour of the gastric antrum. The patient underwent partial gastrectomy and the histological and immunohistochemical findings of the resected specimen established the diagnosis of schwannoma.

  4. SCHWANNOMA ORIGINATING FROM LOWER CRANIAL NERVES: REPORT OF 4 CASES

    OpenAIRE

    OYAMA, HIROFUMI; KITO, AKIRA; MAKI, HIDEKI; HATTORI, KENICHI; NODA, TOMOYUKI; WADA, KENTARO

    2012-01-01

    ABSTRACT Four cases of schwannoma originating from the lower cranial nerves are presented. Case 1 is a schwannoma of the vagus nerve in the parapharyngeal space. The operation was performed by the transcervical approach. Although the tumor capsule was not dissected from the vagus nerve, hoarseness and dysphagia happened transiently after the operation. Case 2 is a schwannoma in the jugular foramen. The operation was performed by the infralabyrinthine approach. Although only the intracapsular ...

  5. Laryngeal schwannoma: a case report with emphasis on sonographic findings

    Energy Technology Data Exchange (ETDEWEB)

    Souza, Luis Ronan Marquez Ferreira de, E-mail: luisronan@gmail.com [Universidade Federal do Triangulo Mineiro (UFTM), Uberaba, MG (Brazil); De Nicola, Harley; Yamasaki, Rosiane; Pedroso, Jose Eduardo; Brasil, Osiris de Oliveira Campones do; Yamashita, Helio [Universidade Federal de Sao Paulo (EPM/UNIFESP), Sao Paulo, SP (Brazil). Escola Paulista de Medicina

    2014-05-15

    Schwannomas are benign nerve sheath tumors composed of Schwann cells, which normally produce the insulating myelin sheath covering peripheral, cranial and autonomic nerves. Twenty-five to forty-five percent of all schwannomas occur in the head and neck region, but location of such tumors in the larynx is rarely observed. The present report is aimed at describing a clinical case of laryngeal schwannoma, with emphasis on sonographic findings. (author)

  6. Solitary Schwannoma in the breast: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Lim, Yun Jung; Kim, Ji Young; Park, Kyeong Mee; Han, Se Hwan; Kim, Soung Hee; Jeong, Myeong Ja; Kim, Soo Hyun [Sanggye Paik Hospital, Inje University, Seoul (Korea, Republic of)

    2006-08-15

    Schwannoma (neurilemoma) is a benign nerve tumor derived from the nerve sheath. The most common locations are the flexor surfaces of the extremities, and the head and the neck. Schwannoma of the breast is unusual. To our knowledge, few studies have reported the radiologic appearance of Schwannoma in the breast and there has been only one report from Korea (1-3). This tumor can be clinically and radiologically considered to be fibroadenoma, which is a common benign tumor of the breast. We describe the mammographic and sonographic findings of a case of Schwannoma in the breast.

  7. Clinicopathological and Immunohistochemical Characterisation of Gastric Schwannomas in 29 Cases

    Science.gov (United States)

    Zheng, Lijun; Wu, Xiaojiang; Kreis, Martin E.; Yu, Zhen; Feng, Lijin; Chen, Chunqiu; Xu, Bin; Bu, Zhaode; Li, Ziyu; Ji, Jiafu

    2014-01-01

    Schwannomas are tumors arising from the nervous system that also occur infrequently in the gastrointestinal tract, most commonly in the stomach. This report characterizes 29 patients with benign or malignant gastric schwannomas. Surgical data and clinical follow-up information were available for 28 cases with a median postoperative duration of 57 months. Clinicopathological and immunohistochemical characteristics of benign and malignant schwannomas were analysed. Four cases (13.7%) were histologically diagnosed with malignant schwannoma. All tumors were positive for S-100 and CD56 proteins, displaying a diffuse staining pattern. Vimentin was expressed in 100% cases and all schwannomas were negative for smooth muscle actin, c-kit, and HMB-45. A significant difference was observed between the group of benign and malignant schwannomas as regards recurrences and metastasis after complete resection (P = 0.015). The survival time of patients with benign schwannomas was longer than the malignant group (P = 0.013), so gastric malignant schwannomas have a potential for recurrence and metastasis, with subsequently short survival. Complete resection with an attempt to remove all tumor tissue with negative margins is of paramount importance in the management of gastric schwannomas, particularly when they turn out to be malignant. PMID:24688535

  8. Clinicopathological and Immunohistochemical Characterisation of Gastric Schwannomas in 29 Cases

    Directory of Open Access Journals (Sweden)

    Lijun Zheng

    2014-01-01

    Full Text Available Schwannomas are tumors arising from the nervous system that also occur infrequently in the gastrointestinal tract, most commonly in the stomach. This report characterizes 29 patients with benign or malignant gastric schwannomas. Surgical data and clinical follow-up information were available for 28 cases with a median postoperative duration of 57 months. Clinicopathological and immunohistochemical characteristics of benign and malignant schwannomas were analysed. Four cases (13.7% were histologically diagnosed with malignant schwannoma. All tumors were positive for S-100 and CD56 proteins, displaying a diffuse staining pattern. Vimentin was expressed in 100% cases and all schwannomas were negative for smooth muscle actin, c-kit, and HMB-45. A significant difference was observed between the group of benign and malignant schwannomas as regards recurrences and metastasis after complete resection (P=0.015. The survival time of patients with benign schwannomas was longer than the malignant group (P=0.013, so gastric malignant schwannomas have a potential for recurrence and metastasis, with subsequently short survival. Complete resection with an attempt to remove all tumor tissue with negative margins is of paramount importance in the management of gastric schwannomas, particularly when they turn out to be malignant.

  9. An Unusual Cause of Cardiac Arrhythmias; Mediastinal Schwannoma

    Directory of Open Access Journals (Sweden)

    Serdar Ozkan

    2014-02-01

    Full Text Available Schwannomas are rare tumours, which originated from neural crest cells. Thoracic schwannomas are very rare and most commonly seen in posterior mediastinum. In a 39 year old female patient whose tumor story dated back to 4 years, a 14 cm extra parenchymal intrathoracic tumor was observed to put minimal pressure on the heart. The patient%u2019s arrhythmia, who had preoperative complaints of palpitations, was improved after tumor excision. Although schwannomas generally are asympthomatic masses, they can lead mass effect according to their localization. In this study, patient with thoracic schwannoma presented with cardiac arrhythmia which never reported in literature so far.

  10. H. Pylori in a gastric schwannoma: a case report.

    Science.gov (United States)

    Lavy, Daniel S; Paulin, Ethan T; Parker, Mitchell I; Zhang, Bin; Parker, Glenn S; Schwartz, Mark R

    2016-04-01

    Schwannomas are benign, often asymptomatic, slow-growing tumors that originate from Schwann cells of the neural sheath. Although H. Pylori has been associated with gastric adenocarcinoma, there has never been a recorded association with schwannoma formation. We present a 64-year-old woman who underwent a laparoscopic partial wedge gastrectomy for an incidentally discovered gastric mass. Histologic examination was consistent with schwannoma; however, chronic inflammation with microorganisms morphologically consistent with H. Pylori was also present. This case suggests the first recorded case of H. Pylori in an immunohistochemically confirmed gastric schwannoma.

  11. Peripheral Vestibular System Disease in Vestibular Schwannomas

    DEFF Research Database (Denmark)

    Møller, Martin Nue; Hansen, Søren; Caye-Thomasen, Per

    2015-01-01

    with VS. METHODS: Retrospective analysis of vestibular system histopathology in temporal bones from 17 patients with unilateral VS. The material was obtained from The Copenhagen Temporal Bone Collection. RESULTS: Vestibular schwannomas were associated with atrophy of the vestibular ganglion, loss of fiber...... density of the peripheral vestibular nerve branches, and atrophy of the neuroepithelium of the vestibular end organs. In cases with small tumors, peripheral disease occurred only in the tissue structures innervated by the specific nerve from which the tumor originated. CONCLUSION: Vestibular schwannomas...... are associated with distinctive disease of the peripheral vestibular tissue structures, suggesting anterograde degeneration and that dizziness in these patients may be caused by deficient peripheral vestibular nerve fibers, neurons, and end organs. In smaller tumors, a highly localized disease occurs, which...

  12. Parapharyngeal space schwannoma of hypoglossal nerve

    Directory of Open Access Journals (Sweden)

    Surya Kanta Pradhan

    2015-01-01

    Full Text Available Parapharyngeal schwannomas are rare benign neoplasms located in a difficult anatomical region. Most of them are asymptomatic and some presents late. Neurological deficit is a late finding, and it occurs only when the lesion is very large and compresses contiguous structures. Computed tomography (CT guided fine needle aspiration cytology along with preoperative CT and magnetic resonance imaging can detect and diagnose it correctly and helps in proper planning and management. Total surgical excision is the treatment of choice. The approach is different as per the site, but trans-cervical approach is preferred. Recurrence is rare after complete excision. We are presenting a very rare parapharyngeal schwannoma arising from the hypoglossal nerve that was excised by trans-cervical approach without any complications and less morbidity as compared to other described approaches.

  13. Intraparenchymal schwannoma of the frontal lobe.

    Directory of Open Access Journals (Sweden)

    Deogaonkar M

    1994-10-01

    Full Text Available A 45 year old woman with bifrontal headaches and progressive diminution in vision over 6 months was found to have bilateral papilloedema. CT scan showed large right frontal lesion with surrounding oedema. Right basal frontotemporal craniotomy was performed to excise the multinodular, intraparenchymatous tumor. Hispathology confirmed the diagnosis of schwannoma. Post-operative course was uneventful with disappearance of pre-operative signs and symptoms.

  14. Radiosurgery of spinal meningiomas and schwannomas.

    Science.gov (United States)

    Kufeld, M; Wowra, B; Muacevic, A; Zausinger, Stefan; Tonn, Jörg-Christian

    2012-02-01

    Purpose of this study is to analyze local control, clinical symptoms and toxicity after image-guided radiosurgery of spinal meningiomas and schwannomas. Standard treatment of benign spinal lesions is microsurgical resection. While a few publications have reported about radiosurgery for benign spinal lesions, this is the first study analyzing the outcome of robotic radiosurgery for benign spinal tumors, treated exclusively with a non-invasive, fiducial free, single-fraction setup. Thirty-six patients with spinal meningiomas or schwannomas were treated, utilizing a robotic radiosurgery system (CyberKnife®, Accuray Inc. Sunnyvale CA), and were followed prospectively. Medical history, histology, clinical symptoms and radiographic outcome were recorded. Thirty-nine spinal lesions were treated because of tumor recurrence, remnants after microsurgery, multiple lesions, or rejection of open surgery. Median age was 45 years (range 18-80 years). Median target volume was 3.4 cm(3) (range 0.2-43.4 cm(3)). Histology revealed 28 schwannomas and 11 meningiomas (WHO grade I). All spinal levels were affected. Median prescription dose was 14 Gray (95% C.I. 13.4-14 Gy) to the 70% isodose. After a median follow-up of 18 months (range 6-50 months) no local tumor progression was detected. 20 lesions (51%) remained stable, 19 tumors (49%) decreased in size. One patient with schwannomatosis was treated repeatedly for three new tumor locations. Pain was the initial symptom in 16 of 25 schwannoma patients, and in 3 of 11 patients with meningiomas. Pain levels decreased in 8/19 patients. All but one patient with motor deficits remained clinically stable. No myelopathic signs where found. Single-session radiosurgery for benign spinal tumors in selected patients has proven to inhibit tumor progression within the observed period without signs of early toxicity. Radiosurgery offers an additional treatment option, if microsurgery is not feasible in cases of tumor recurrence, post

  15. Complications of microsurgery of vestibular schwannoma

    Czech Academy of Sciences Publication Activity Database

    Betka, J.; Zvěřina, E.; Balogová, Zuzana; Profant, Oliver; Skřivan, J.; Kraus, J.; Lisý, J.; Syka, Josef; Chovanec, M.

    2014-01-01

    Roč. 2014, May 28 (2014), s. 315952 ISSN 2314-6133 R&D Projects: GA MZd NT12459 Grant - others:GA MZd(CZ) NT11543; GA MŠk(CZ) UNCE 204013; GA UK(CZ) SVV 266513; GA MŠk(CZ) Prvouk-P27/LF1/1 Institutional support: RVO:68378041 Keywords : acoustic neurona surgery * tumor surgery * vestibular schwannomas Subject RIV: FF - HEENT, Dentistry Impact factor: 1.579, year: 2014

  16. Pelvic schwannoma in the right parametrium

    Directory of Open Access Journals (Sweden)

    Machairiotis N

    2013-03-01

    Full Text Available Nikolaos Machairiotis,4 Paul Zarogoulidis,3 Aikaterini Stylianaki,1 Eleni Karatrasoglou,4 Georgia Sotiropoulou,4 Alvin Floreskou,4 Eleana Chatzi,4 Athanasia Karamani,4 Georgia Liapi,5 Eleni Papakonstantinou,5 Nikolaos Katsikogiannis,1 Nikolaos Courcoutsakis,2 Christodoulos Machairiotis4 1Surgery Department, 2Radiology Department, University General Hospital of Alexandroupolis, Democritus University of Thrace, Alexandroupolis, Greece; 3Pulmonary Department, G Papanikolaou General Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece; 4Obstetric Gynecology Department, 5Pathology Department, Thriasio General Hospital, Athens, Greece Abstract: Neurilemomas are benign usually encapsulated nerve sheath tumors derived from the Schwann cells. These tumors commonly arise from the cranial nerves as acoustic neurinomas but they are extremely rare in the pelvis and the retroperitoneal area (less than 0.5% of reported cases, unless they are combined with von Recklinghausen disease (type 1 neurofibromatosis. We report the case of a 58-year-old female with pelvic schwannoma, 6.5 × 5.5 cm in size, in the right parametrium. This is the first case reported in the literature. Based on the rarity of this tumor and in order to ensure optimum treatment and survival for our patient, we performed laparotomy with total abdominal hysterectomy and en-block tumor excision. A frozen section was taken during the surgery before complete resection of the mass, which was ambiguous. Because of the possibility of malignancy, complete excision of the mass was performed, with pelvic blunt dissection. Histological examination showed a benign neoplasm, originating from the cells of peripheral nerve sheaths; diagnosis was a schwannoma. There were degenerative areas, including cystic degeneration, hemorrhagic infiltrations, ischemic foci with pycnotic cells, and collagen replacement. Pelvic schwannomas are rare neoplasms that can be misdiagnosed. Laparoscopy is a safe

  17. MRI in a quiescent vestibular schwannoma

    International Nuclear Information System (INIS)

    Dutto, Santiago G.; Trepat, Jorgelina; Nagel, Jorge R.

    2003-01-01

    Vestibular schwannomas are benign neoplasms that take origin from Schwann cells, the majority arise from the vestibular branch of VIII cranial nerve. Unilateral sensorineural hearing loss is the most common symptom referred by patients who suffer this disease. With the advent and increasing use of MRI the diagnosis of this intra labyrinthine tumor has become more frequent. Paramagnetic contrast gadolinium has precise indication in the study protocol of this pathology. The high sensitivity of this method allows an early diagnosis. In patients with low auditory loss an excision of intra labyrinthine small tumors can offer a better therapeutic chance. We report a case of a 25 years old woman with progressive hearing loss. MRI with gadolinium enhancement demonstrated an intra vestibular schwannoma. In a follow-up control after 5 years MRI revealed no significant changes in tumor size or signal intensity. 3-D volumetric reconstruction offered complimentary information about this 'quiescent' schwannoma. Considering the tumoral behavior (without growth within five years) and the degree of hearing loss no invasive therapy was performed. (author)

  18. Gastric schwannoma exhibiting increased fluorodeoxyglucose uptake.

    Science.gov (United States)

    Komatsu, Daisuke; Koide, Naohiko; Hiraga, Risako; Furuya, Naoyuki; Akamatsu, Taiji; Uehara, Takeshi; Miyagawa, Shinichi

    2009-01-01

    This is the first case of gastric schwannoma that exhibited increased accumulation of [(18)F] fluorodeoxyglucose (FDG) on positron emission tomography (PET) imaging. The patient was a 60-year-old woman in whom esophagogastroduodenoscopy showed a submucosal tumor, about 25 mm in size, in the upper body of the stomach, with ulceration at the top of the tumor. Endoscopic ultrasonography revealed a well-defined hypoechoic mass located in the proper muscle layer of the stomach. The specimen taken from the tumor showed only inflammatory degenerative tissue. Abdominal computed tomography revealed a tumor in the upper body of the stomach. FDG-PET showed FDG uptake (standardized uptake value [SUV] max 5.8) coincident with the tumor. Hence, the tumor was diagnosed initially as a gastrointestinal stromal tumor of the stomach. Laparoscopic partial gastrectomy was performed. Pathological examination showed that the tumor consisted of spindle cells with large nuclei, and mitosis was absent. The Ki-67 labeling index of the tumor cells was 4%. Immunohistochemically, the tumor cells showed a positive reaction for S-100 protein, whereas they were negative for KIT, CD 34, and alpha-smooth muscle actin protein. The tumor was diagnosed as a benign gastric schwannoma. Gastric schwannoma should be included in the differential diagnosis of submucosal tumors of the stomach with FDG uptake.

  19. Long-term hearing preservation in vestibular schwannoma

    DEFF Research Database (Denmark)

    Stangerup, Sven-Eric; Thomsen, Jens; Tos, Mirko

    2010-01-01

    The aim of the present study was to evaluate the long-term hearing during "wait and scan" management of vestibular schwannomas.......The aim of the present study was to evaluate the long-term hearing during "wait and scan" management of vestibular schwannomas....

  20. Malignant Transformation of Vagal Nerve Schwannoma in to ...

    African Journals Online (AJOL)

    Malignant Transformation of Vagal Nerve Schwannoma in to Angiosarcoma: A Rare Event. Sangeet Kumar Agarwal, Manish Munjal, Devinder Rai, Seema Rao. Abstract. Schwannomas are benign, rare peripheral nerve sheath tumors that occur in the head and neck region. Some physicians opt to closely observe cases of ...

  1. A Case Report of Schwannoma Presenting as Sciatica

    Directory of Open Access Journals (Sweden)

    A Afshar Fard

    2012-08-01

    The patient underwent operation and sciatic nerve explored via posterior tight and unique neural branch of mass dissociated of sciatic and mass resected. Pathology report confirmed Schwannoma in the patient. Conclusion: In patients with sciatic pain, schwannoma nerve is one of the differential diagnosis and it needs to be considered in assessing of patients.

  2. Molecular characterization of chromosome 22 deletions in schwannomas

    NARCIS (Netherlands)

    Bijlsma, E. K.; Brouwer-Mladin, R.; Bosch, D. A.; Westerveld, A.; Hulsebos, T. J.

    1992-01-01

    Schwannomas are tumors of the cranial, spinal, and peripheral nerve sheaths that originate from Schwann cells. Acoustic neurinomas are the most frequent cranial schwannomas. They might develop sporadically or in the context of neurofibromatosis type 2 (NF2). Loss of part or all of chromosome 22 is

  3. Extramedullary Myeloid Cell Tumour Presenting As Leukaemia Cutis

    Directory of Open Access Journals (Sweden)

    Thappa Devinder Mohan

    2002-01-01

    Full Text Available We herewith report a case of extramedullary myeloid cell tumour presenting as leukaemia cutis for its rarity. It occurred in a 50 year old male patient who presented to us with a 40 days history of painless raised solid skin swellings over the trunk. Histopathological examination of the skin biopsy and bone marrow biopsy showed features suggestive of non-Hodgkin’s lymphoma. Immunophenotyping on skin biopsy specimens and bone marrow biopsy found tumour cells expressing CD43 and Tdt but were negative for CD3 and CD20. These features were consistent with extramedullary myeloid cell tumour involving skin and subcutis (cutaneous manifestation of acute myeloid leukaemia.

  4. Unusually large quiescent ancient schwannoma of hypoglossal nerve

    Directory of Open Access Journals (Sweden)

    Sangeeta P Wanjari

    2013-01-01

    Full Text Available Ancient schwannoma is considered as a variant of schwannoma, comprising about 10% of all schwanommas. Schwannoma is a benign neoplasm derived from the nerve sheath of peripheral motor, sensory and sympathetic nerves and from the cranial nerve pairs. It usually presents as a solitary soft-tissue lesion which is slow growing, encapsulated and is often associated with nerve attached peripherally. Diagnosis is often confirmed with the microscopic examination. The long standing schwannoma attributes to degenerative changes and is termed "ancient" schwannoma. Present case is of a 68-year-old female patient who reported with an asymptomatic large swelling below mandible on the left side since last 23 years. The lesion was surgically excised under general anesthesia.

  5. Cytologic findings of gastric schwannoma: a case report.

    Science.gov (United States)

    Rodriguez, Erika; Tellschow, Steven; Steinberg, David M; Montgomery, Elizabeth

    2014-02-01

    Spindle cell lesions of the stomach are rare. They usually affect the submucosa or muscularis propria and therefore can be sampled by endoscopic fine needle aspiration. The most common tumor in this category is gastrointestinal stromal tumor (GIST), followed by leiomyoma and gastric schwannoma. Gastric schwannoma is a benign tumor of neuroectodermal origin that has overlapping morphologic/cytologic features with GIST and leiomyoma. Gastric schwannomas differ from peripheral schwannomas by lacking a capsule, Verocay bodies, Antoni B areas, and thick-walled vessels. They are characterized morphologically by poorly defined borders, cuffs of lymphoid tissue and a haphazard spindle cell proliferation. We present here the cytologic and histopathologic features of a gastric schwannoma. The tumor was composed of spindle cells with delicate eosinophilic cytoplasm and wavy nuclei, with an associated conspicuous lymphoid backdrop. The latter feature raised the possibility of a lymphoid lesion, a problem cytopathologists should be aware of. Copyright © 2012 Wiley Periodicals, Inc.

  6. Thalassemia, extramedullary hematopoiesis, and spinal cord compression: A case report

    OpenAIRE

    Bukhari, Syed Sarmad; Junaid, Muhammad; Rashid, Mamoon Ur

    2016-01-01

    Background: Extramedullary hematopoiesis (EMH) refers to hematopoiesis outside of the medulla of the bone. Chronic anemia states such as thalassemia can cause hematopoietic tissue to expand in certain locations. We report a case of spinal cord compression due to recurrent spinal epidural EMH, which was treated with a combination of surgery and radiotherapy. Pakistan has one of the highest incidence and prevalence of thalassemia in the world. We describe published literature on diagnosis and m...

  7. Adrenal extramedullary hematopoiesis associated with β-thalassemia major

    OpenAIRE

    Bijan Keikhaei; Ahmad Soltani Shirazi; Mahboob Mohammad Pour

    2012-01-01

    The presence of apparently normal hematopoietic tissue outside of bone marrow cavity is defined as extramedullary hema - topoiesis (EMH). EMH is a rare complication in thalassemia major (TM) and adrenal gland as well. This report describes a case of adrenal EMH in a 26-year-old man with β-TM. He has been transfused with regular blood transfusion since 9 months. During the routine physical examination he was incidentally found to have a hypoechoic mass at his abdominal ultrasonography. Abdomin...

  8. Premature epiphyseal fusion and extramedullary hematopoiesis in thalassemia

    International Nuclear Information System (INIS)

    Colavita, N.; Orazi, C.; Danza, S.M.; Falappa, P.G.; Fabbri, R.

    1987-01-01

    The main skeletal abnormalities in β-thalassemia are widening of medullary spaces, rarefaction of bone trabeculae, thinning of cortical bone, and perpendicular periosteal spiculation. Premature epiphyseal fusion (PEF) and extramedullary hematopoiesis (EH) are found, though more rarely. The incidence of PEF and EH in 64 patients affected by β-thalassemia is reported. The different incidence of such complications in thalassemia major and intermedia is reported, and a possible correlation with transfusion regimen is also considered. (orig.)

  9. Extramedullary Plasmacytoma Mimicking Pancreatic Cancer: An Unusual Presentation

    Directory of Open Access Journals (Sweden)

    Daniela Sciancalepore

    2016-01-01

    Full Text Available Multiple myeloma is a plasma cell tumor that homes to and expands in the bone marrow and that, despite the new available drugs, remains incurable. Extramedullary plasmacytoma is a not frequent manifestation during the natural history of multiple myeloma and is frequently associated with plasma cell bone marrow infiltration. The most common locations for an EMP include the gastrointestinal tract, pleura, testis, skin, peritoneum, liver, endocrine glands, and lymph nodes. Primary involvement of the gallbladder fossa is exceedingly rare. In this report, we describe a patient with multiple myeloma who achieved a clinical and serological remission after autologous transplant but progressed rapidly at extramedullary site mimicking a second cancer (i.e., pancreatic or biliary cancer. In this case, the extramedullary localization was refractory to standard therapy, differently from bone marrow localization, but responded to lymphoma-like therapy. In this patient (i the particular site of developing plasmacytoma is the gallbladder fossa, (ii the timing of onset of this neoplasm is immediately after autologous transplant, and (iii its disjunction from primary myeloma is that it appears in clinical and serological remission phase which may be confounding during the diagnostic approach simulating a different tumor (solid tumor.

  10. F 18 FDG PET/CT of a Gastric Schwannoma

    Energy Technology Data Exchange (ETDEWEB)

    Hong, Il Ki; Kim, Deog Yoon [Kyung Hee Univ. School of Medicine, Seoul (Korea, Republic of)

    2011-09-15

    Schwannomas, also known as neurilemmomas, are tumors originating from nervous tissue; they have Schwann cell sheaths. According to a recent classification, about 80% of gastrointestinal mesenchymal tumors are gastrointestinal stromal tumors (GISTs). Gastrointestinal (GI) Schwannomas have been reported to represent only 3% of all GI mesenchymal tumors. These tumors make up only 0.2% of all gastric neoplasms. Schwannomas of the GI tract are distinctive from conventional Schwannomas that arise in soft tissue of the central nervous system. GI Schwannomas are hypothesized to arise from the myenteric plexus within the GI tract wall. These tumors are usually benign, slow growing and asymptomatic, and therefore most are discovered incidentally. The differentiation of Schwannomas from other submucosal tumors is very difficult. The main differential diagnosis for a mass arising in the wall of the gastointestinal tract is a GIST, which is a potentially malignant mesenchymal GI tumor that arises from the interstitial cells of Cajal, which help regulate peristalsis. The diagnostic determination of Schwannomas requires positive histological tests for S 100 protein and vimentin, but negative histological tests for smooth muscle actin and c KIT. In contrast, GISTs are C KIT positive and can be S 100 positive if they are located in small bowel. Because most patients with Schwannomas have excellent prognoses, surgical removal is sufficient for treatment. Gastric Schwannomas are normally benign, and malignant transformation is extremely rare. However, the current case illustrates that these tumors may exhibit avid F 18 FDG uptake. It remains unclear why high F 18 FDG uptake is found in benign tumors such as Schwannomas. F 18 FDG uptake in soft tissue and neural Schwannomas is variable but is frequently high, possibly due to over expression of the glucose transporter by tumor cells. In particular, glucose transporter type 3 is found in all human tissues and is the major glucose

  11. F 18 FDG PET/CT of a Gastric Schwannoma

    International Nuclear Information System (INIS)

    Hong, Il Ki; Kim, Deog Yoon

    2011-01-01

    Schwannomas, also known as neurilemmomas, are tumors originating from nervous tissue; they have Schwann cell sheaths. According to a recent classification, about 80% of gastrointestinal mesenchymal tumors are gastrointestinal stromal tumors (GISTs). Gastrointestinal (GI) Schwannomas have been reported to represent only 3% of all GI mesenchymal tumors. These tumors make up only 0.2% of all gastric neoplasms. Schwannomas of the GI tract are distinctive from conventional Schwannomas that arise in soft tissue of the central nervous system. GI Schwannomas are hypothesized to arise from the myenteric plexus within the GI tract wall. These tumors are usually benign, slow growing and asymptomatic, and therefore most are discovered incidentally. The differentiation of Schwannomas from other submucosal tumors is very difficult. The main differential diagnosis for a mass arising in the wall of the gastointestinal tract is a GIST, which is a potentially malignant mesenchymal GI tumor that arises from the interstitial cells of Cajal, which help regulate peristalsis. The diagnostic determination of Schwannomas requires positive histological tests for S 100 protein and vimentin, but negative histological tests for smooth muscle actin and c KIT. In contrast, GISTs are C KIT positive and can be S 100 positive if they are located in small bowel. Because most patients with Schwannomas have excellent prognoses, surgical removal is sufficient for treatment. Gastric Schwannomas are normally benign, and malignant transformation is extremely rare. However, the current case illustrates that these tumors may exhibit avid F 18 FDG uptake. It remains unclear why high F 18 FDG uptake is found in benign tumors such as Schwannomas. F 18 FDG uptake in soft tissue and neural Schwannomas is variable but is frequently high, possibly due to over expression of the glucose transporter by tumor cells. In particular, glucose transporter type 3 is found in all human tissues and is the major glucose

  12. Triple manifestation of extramedullary plasmacytoma in the upper airway: an unusual clinical entity.

    LENUS (Irish Health Repository)

    Morariu, I

    2012-02-01

    OBJECTIVE: We report an extremely rare case of extramedullary plasmacytoma. METHOD: Case report and review of the English-literature concerning extramedullary plasmacytoma and multiple myeloma. RESULT: We present an unusual case of multiple extramedullary plasmacytomas, which, over a protracted course of 30 years, presented on different occasions at three separate sites in the head and neck. The patient was managed surgically on all occasions, and was disease-free at the time of writing. CONCLUSION: Following review of the literature, we believe this to be the only case with this extremely unusual presentation. This case is noteworthy, not only because of the rarity of extramedullary plasmacytoma, but also because it highlights a number of important clinical issues. The diagnosis and management of extramedullary plasmacytoma require close cooperation between multiple disciplines.

  13. Schwannoma of floor of mouth: A case report

    Directory of Open Access Journals (Sweden)

    Anjum Farooqui

    2017-01-01

    Full Text Available Schwannoma (neurilemmoma is a benign nerve sheath tumor that is composed entirely of well-differentiated Schwann cells. It is important to highlight that schwannoma is usually found in the head and neck and rarely in the oral cavity. Schwannoma is a benign tumor that originates from the Schwann cells of the peripheral nerves. They are usually asymptomatic, do not recur, and malignant transformation is rare. Here, we report a case of neurilemmoma that was located at an unusual location, i.e., floor of the mouth.

  14. Solitary schwannoma of sciatic nerve. Diagnosis by CT

    Energy Technology Data Exchange (ETDEWEB)

    Sato, M.; Handa, J. (Shiga Univ. of Medical Science, Ohtsu, Shiga (Japan)); Koyama, T.

    1982-05-01

    A solitary schwannoma of the peripheral nerve may arise sporadically in patients who have no evidence of a genetic predetermination of von Recklinghausen's disease. In the leg, schwannomas usually appear on the flexor aspect, especially near the elbow, wrist and knee, and the feet are usually spared. A solitary schwannoma of the sciatic nerve is very rare as a case of a sciatic pain, and the CT diagnosis of such a lesion has not been previously reported. In the present case, the deeply situated, small lesion was clearly delineated with high resolution CT.

  15. Outcome after translabyrinthine surgery for vestibular schwannomas

    DEFF Research Database (Denmark)

    Springborg, Jacob Bertram; Fugleholm, Kåre; Poulsgaard, Lars

    2012-01-01

    The objective of this article is to study the outcome after translabyrinthine surgery for vestibular schwannomas, with special focus on the facial nerve function. The study design is a case series from a national centralized database and it is set in two University Hospitals in Denmark. Participa......The objective of this article is to study the outcome after translabyrinthine surgery for vestibular schwannomas, with special focus on the facial nerve function. The study design is a case series from a national centralized database and it is set in two University Hospitals in Denmark....... Participants were 1244 patients who underwent translabyrinthine surgery during a period of 33 years from 1976 to 2009. Main outcome measures were tumor removal, intraoperative facial nerve preservation, complications, and postoperative facial nerve function. In 84% patients, the tumor was totally resected...... and in ~85% the nerve was intact during surgery. During 33 years, 12 patients died from complications to surgery and ~14% had cerebrospinal fluid leakage. Before surgery, 74 patients had facial paresis and 46% of these improved after surgery. In patients with normal facial function, overall ~70% had a good...

  16. CT, MRI, and PET findings of gastric schwannoma.

    Science.gov (United States)

    Takeda, Minako; Amano, Yasuo; Machida, Tadashi; Kato, Shunji; Naito, Zenya; Kumita, Shinichiro

    2012-08-01

    Gastric schwannoma is a rare tumor that accounts for only 0.2 % of all gastric tumors. We report a case of gastric schwannoma that underwent computed tomography (CT), magnetic resonance imaging (MRI), and [(18)F]-fluorodeoxyglucose positron emission tomography (FDG-PET), and its histological confirmation was acquired. Gastric schwannoma showed high intensity on T2-weighted and diffusion-weighted MRI and high maximum standardized uptake on [(18)F]-FDG-PET. Lymphadenopathy close to the tumor was also found. Although diffusion-weighted MRI, [(18)F]-FDG-PET, and the presence of lymphadenopathy could suggest malignant tumors, the detail interpretation of the other CT and MRI findings may give a clue for the diagnosis of gastric schwannoma.

  17. Laparoscopic resection of a gastric schwannoma: A case report

    Directory of Open Access Journals (Sweden)

    Edgar Vargas Flores

    2016-01-01

    Conclusion: Gastric schwannomas should be included in the differential diagnosis of any gastric submucosal mass. Negative margin resection as seen with this patient is the standard surgical treatment as there is low malignant transformation potential.

  18. Vestibular schwannoma with contralateral facial pain – case report

    Directory of Open Access Journals (Sweden)

    Ghodsi Mohammad

    2003-03-01

    Full Text Available Abstract Background Vestibular schwannoma (acoustic neuroma most commonly presents with ipsilateral disturbances of acoustic, vestibular, trigeminal and facial nerves. Presentation of vestibular schwannoma with contralateral facial pain is quite uncommon. Case presentation Among 156 cases of operated vestibular schwannoma, we found one case with unusual presentation of contralateral hemifacial pain. Conclusion The presentation of contralateral facial pain in the vestibular schwannoma is rare. It seems that displacement and distortion of the brainstem and compression of the contralateral trigeminal nerve in Meckel's cave by the large mass lesion may lead to this atypical presentation. The best practice in these patients is removal of the tumour, although persistent contralateral pain after operation has been reported.

  19. Anterior inferior cerebellar artery aneurysms mimicking vestibular schwannomas.

    Science.gov (United States)

    Marchini, Aïda Kawkabani; Mosimann, Pascal J; Guichard, Jean-Pierre; Boukobza, Monique; Houdart, Emmanuel

    2014-01-01

    Unruptured anterior inferior cerebellar artery (AICA) aneurysms are rare but potentially lethal cerebellopontine angle (CPA) lesions that may be misdiagnosed as vestibular schwannomas when they present with vestibulo-cochlear symptoms. We report two cases of unruptured but symptomatic AICA aneurysms initially referred to us as atypical vestibular schwannomas requiring surgery. Two discriminant MR features are described. One patient refused treatment. The other was successfully treated by coil occlusion. Caution is advised before suspecting a CPA mass to be a purely extra-canalicular schwannoma, given its extreme rarity. Deafness and cerebellar ischemia may be prevented if AICA aneurysms are correctly identified preoperatively. In the absence of specific arterial imaging, two MR features may distinguish them from vestibular schwannomas: (1) the absence of internal auditory canal enlargement and (2) the "blurry dot sign," representing blood flow artefacts on pre- and postcontrast studies. Copyright © 2013 by the American Society of Neuroimaging.

  20. Sciatica due to Schwannoma at the Sciatic Notch

    Directory of Open Access Journals (Sweden)

    Yavuz Haspolat

    2013-01-01

    Full Text Available Schwannomas are rarely seen on the sciatic nerve and can cause sciatica. In this case report we aimed to present an unusual location of schwannoma along sciatic nerve that causes sciatica. A 60-years-old-man was admitted to us with complaints of pain on his thigh and paresthesia on his foot. Radiography of the patient revealed a solitary lesion on the sciatic nerve. The lesion was excised and the symptoms resolved after surgery.

  1. Schwannoma of the median nerve: diagnosis sometimes delayed.

    Science.gov (United States)

    Boufettal, Monsef; Azouz, Mohamed; Rhanim, Abdelkarim; Abouzahir, Mohamed; Mahfoud, Mustapha; Bardouni, Ahmed El; Berrada, Mohamed S; Yaacoubi, Moradh El

    2014-01-01

    Schwannoma is a tumor that develops from nerve sheath. The authors report an unusual case of schwannoma of the median nerve (MN) that remained asymptomatic for four years. The diagnosis was based on MRI (magnetic resonance imaging) and histopathological examination. Surgical removal is usually curative. The asymptomatic character of the tumor and its slow evolution remain an essential factor in diagnosis delays. This tumor has a good prognosis with a low recurrence rate and potential for malignant transformation.

  2. [Gastric Schwannoma: a case report and literature review].

    Science.gov (United States)

    Huesca-Jiménez, K; Medina-Franco, H

    2009-01-01

    We report a 49-year old male with diagnosis of gastric schwannoma who underwent subtotal gastrectomy. We describe clinicopathological and immunohistochemical characteristics of these tumors and performed an extensive literature review. Gastric schwannomas are very rare tumors but they should be taken into account in the differential diagnosis of more common mesenchimal neoplasms like gastrointestinal stromal tumors (GIST). The treatment of choice is surgical resection and their prognosis is very favourable.

  3. Optimising the image of the intradural nerve root: the value of MR radiculography

    Energy Technology Data Exchange (ETDEWEB)

    Hofman, P.A.M. [Department of Diagnostic Radiology, University Hospital Maastricht (Netherlands); Wilmink, J.T. [Department of Diagnostic Radiology, University Hospital Maastricht (Netherlands)

    1996-10-01

    We evaluated the additional value of MR radiculography for increasing the sensitivity and specificity of MRI with regard to nerve root compression in patients with sciatica. The single slices of a heavily T 2-weighted oblique coronal image set were reformatted with a maximum intensity projection protocol. This image resembles a classical contrast radiculogram and shows the intradural nerve root and its sleeve. In 43 patients studied with a standard MRI examination there was a need for further assessment of nerve root compression in 19 (44 %). In 13 (68 %) of these, MR radiculography made a definite verdict possible. (orig.). With 4 figs., 2 tabs.

  4. Intradural Procedural Time to Assess Technical Difficulty of Superciliary Keyhole and Pterional Approaches for Unruptured Middle Cerebral Artery Aneurysms.

    Science.gov (United States)

    Choi, Yeon-Ju; Son, Wonsoo; Park, Ki-Su; Park, Jaechan

    2016-11-01

    This study used the intradural procedural time to assess the overall technical difficulty involved in surgically clipping an unruptured middle cerebral artery (MCA) aneurysm via a pterional or superciliary approach. The clinical and radiological variables affecting the intradural procedural time were investigated, and the intradural procedural time compared between a superciliary keyhole approach and a pterional approach. During a 5.5-year period, patients with a single MCA aneurysm were enrolled in this retrospective study. The selection criteria for a superciliary keyhole approach included : 1) maximum diameter of the unruptured MCA aneurysm <15 mm, 2) neck diameter of the MCA aneurysm <10 mm, and 3) aneurysm location involving the sphenoidal or horizontal segment of MCA (M1) segment and MCA bifurcation, excluding aneurysms distal to the MCA genu. Meanwhile, the control comparison group included patients with the same selection criteria as for a superciliary approach, yet who preferred a pterional approach to avoid a postoperative facial wound or due to preoperative skin trouble in the supraorbital area. To determine the variables affecting the intradural procedural time, a multiple regression analysis was performed using such data as the patient age and gender, maximum aneurysm diameter, aneurysm neck diameter, and length of the pre-aneurysm M1 segment. In addition, the intradural procedural times were compared between the superciliary and pterional patient groups, along with the other variables. A total of 160 patients underwent a superciliary (n=124) or pterional (n=36) approach for an unruptured MCA aneurysm. In the multiple regression analysis, an increase in the diameter of the aneurysm neck ( p <0.001) was identified as a statistically significant factor increasing the intradural procedural time. A Pearson correlation analysis also showed a positive correlation (r=0.340) between the neck diameter and the intradural procedural time. When comparing the

  5. Endosonographic Features of Gastric Schwannoma: A Single Center Experience.

    Science.gov (United States)

    Yoon, Jong Min; Kim, Gwang Ha; Park, Do Youn; Shin, Na Ri; Ahn, Sangjeong; Park, Chul Hong; Lee, Jin Sung; Lee, Key Jo; Lee, Bong Eun; Song, Geun Am

    2016-11-01

    Gastric schwannomas are rare benign mesenchymal tumors that are difficult to differentiate from other mesenchymal tumors with malignant potential, such as gastrointestinal stromal tumors. This study aimed to evaluate the characteristic findings of gastric schwannomas via endoscopic ultrasonography (EUS). We retrospectively reviewed the EUS findings of 27 gastric schwannoma cases that underwent surgical excision at Pusan National University Hospital during 2007 to 2014. Gastric schwannomas were mainly located in the middle third of the stomach with a mean tumor size of 32 mm. All lesions exhibited hypoechoic echogenicity, and 24 lesions (88.9%) exhibited heterogeneous echogenicity. Seventeen lesions (63.0%) exhibited decreased echogenicity compared to the normal proper muscle layer. Distinct borders were observed in 24 lesions (88.9%), lobulated margins were observed in six lesions (22.2%), and marginal haloes were observed in 24 lesions (88.9%). Hyperechogenic spots were observed in 21 lesions (77.8%), calcifications were observed in one lesion (3.7%), and cystic changes were observed in two lesions (7.4%). During EUS, gastric schwannomas appear as heterogeneously hypoechoic lesions with decreased echogenicity compared to the normal proper muscle layer. These features may be helpful for differentiating gastric schwannomas from other mesenchymal tumors.

  6. Plexiform (multinodular) schwannoma of soft palate. Report of a case.

    Science.gov (United States)

    Kapetanakis, Stylianos; Vasileiadis, Ioannis; Petousis, Aristotelis; Fiska, Aliki; Stavrianaki, Anna

    2012-01-01

    Plexiform schwannoma is a rare benign neoplasm of the neural sheath characterized by a multinodular plexiform growth pattern. Only 5% of schwannomas have a plexiform or multinodular growth pattern. Schwannoma apparently derives from the Schwann cells. Extracranially, 25% of all schwannomas are located in the head and neck region, but only 1% show an intraoral origin. The intraoral lesions show a predilection for the tongue, followed by the palate, buccal mucosa, lip and gingival. Microscopic examination is necessary to confirm the diagnosis. Characteristic histological signs are the palisading of the spindle-shaped Schwann cells around the central acellular area, so called Verocay bodies. We report a case of a 21-year-old woman with a smooth mass of the soft palate that was gradually increasing. Surgical excision of the mass was done and the histopathology and immunohistochemistry study of the excised lesion revealed a multinodular plexiform schwannoma of the soft palate. The patient is under regular clinical control, with no signs of recurrence after 17 months. Plexiform schwannomas of the soft palate are mentioned very rarely in the English literature. This rare benign tumor is worthy of recognition because it can be misdiagnosed as plexiform neurofibroma.

  7. Solitary Extramedullary Plasmacytoma of the Cricoid Cartilage—Case Report

    Directory of Open Access Journals (Sweden)

    Simone Krebs

    2017-11-01

    Full Text Available Solitary plasmacytoma (SP is an extremely rare form of hematologic malignancy that can be classified as solitary bone plasmacytoma or solitary extramedullary plasmacytoma. Here, we report a patient who presented with progressive shortness of breath and foreign body sensation in his throat. Fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG-PET/CT demonstrated an abnormal FDG-avid soft tissue mass arising from the larynx involving the cricoid cartilage without additional suspicious lesions. Histology revealed an abundance of plasma cells; immunohistochemistry was positive for CD138 expression and lambda chains, and negative for CD20. Comprehensive imaging studies and panendoscopy of the ENT tract confirmed solitary disease involvement. Following additional systemic work-up, a diagnosis of extramedullary plasmacytoma was rendered. The patient underwent definitive radiotherapy using intensity-modulated radiation therapy (total dose of 46 Gy, divided in 23 fractions of 200 cGy. Serial PET/CT showed the stepwise resolution of abnormal FDG uptake and resolution of the cricoid cartilage lesion. With 22 months of follow-up, the patient remains free of disease. We describe the rare case of SP presenting as a FDG-avid hypermetabolic soft tissue mass in the cricoid cartilage, which should be considered in the differential diagnosis of laryngeal tumors. Of note, SP is radiosensitive; favorable outcome can be expected once treated with doses of 40–50 Gy. FDG PET/CT is helpful in diagnosis and response assessment for this disease.

  8. Spinal cord compression secondary to extramedullary hematopoiesis in a dog.

    Science.gov (United States)

    Williams, Lindsay M; Skeen, Todd M

    2013-03-15

    An 11-year-old spayed female Siberian Husky was evaluated because of a 2-week history of progressive paraparesis. Results of neurologic examination were consistent with a T3-L3 myelopathy. There were no abnormalities on CBC, and hypercalcemia was noted on serum biochemical analysis. Several hypoechoic splenic nodules were evident on abdominal ultrasonography, and results of fine-needle aspiration cytology were consistent with splenic extramedullary hematopoiesis (EMH). Two compressive, extradural masses in the dorsal epidural space of the thoracolumbar region of the spinal cord were seen on MRI images. A dorsal laminectomy was performed to remove the extradural spinal masses. Results of histologic examination of tissue samples were consistent with EMH. Following surgery, clinical signs of paraparesis resolved, and there was no recurrence of the masses 24 months after surgery. Extramedullary hematopoesis should be considered as a differential diagnosis in dogs in which results of diagnostic imaging indicate a epidural mass. In human patients, spinal EMH usually occurs secondary to an underlying hematologic disease, but it can also occur spontaneously. Treatment options reported for humans include surgical decompression, radiation therapy, chemotherapy, and blood transfusion. The dog of this report responded favorably to surgical decompression and was clinically normal 2 years after surgery.

  9. Myelomatous ascites as an initial manifestation of extramedullary involvement of multiple myeloma

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Seo Youn; Lee, Hae Kyung; Yi, Boem Ha; Lee, Min Hee; Kim, Hee Kyung; Park, Seong Kyu [Soonchunhyang University Bucheon Hospital, Bucheon (Korea, Republic of)

    2017-03-15

    Multiple myeloma is a common hematological malignancy. Aggressive myeloma invades the organs outside the bone marrow, lymph, or reticuloendothelial systems. Among the extramedullary involvements of multiple myeloma, myelomatous ascites are extremely rare and are associated with a poor prognosis. We describe a case of myelomatous ascites as an initial manifestation of extramedullary involvement of multiple myeloma in 39-year-old patient. The patient was treated with high-dose chemotherapy, but extensive extramedullary involvement progressed, and the patient expired approximately five months after the initial detection of ascites.

  10. Pediatric Isolated Sinonasal Schwannoma: A New Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Xiao-Hui Ma

    2016-01-01

    Full Text Available Schwannomas of the paranasal sinus are uncommon. Less than 4% of schwannomas involve the nasal cavity and paranasal sinuses, even less in the pediatric age group. A case of schwannoma arising in maxillary sinus in a 2.5-year-old Chinese boy is reported. The basis for discussion of this case is the exceptional rarity of sinonasal schwannoma in pediatric patients.

  11. A rare case of concomitant cervical disc herniation and intradural meningioma treated with one-stage posterior surgery.

    Science.gov (United States)

    Hu, Xiaojian; Chen, Zhong; Wang, Yue

    2017-12-14

    Case report. To present a rare case of cervical disc herniation concomitant with intradural meningioma which was successfully treated using a single one-stage posterior surgery of tumor resection and transdural discectomy. Coexistence of symptomatic disc herniation and intra-spinal tumor in the same cervical segment is extremely rare. Usually, two-stage anterior and posterior surgeries are needed to treat two conditions, respectively. One-stage posterior surgery to treat two pathologies simultaneously has not been reported in the literature. A 76-year-old man presented with leg weakness and numbness for 6 months and left arm pain for 2 months. Contrast MR imaging revealed C3/4 intervertebral disc herniation and a hyperintense intradural lesion at the right portion of C3 canal. A one-stage posterior surgery, including C3/4 laminectomy, intradural tumor resection, transdural C3/4 discectomy, and C3/4 lateral mass instrumentation and fusion, was performed to treat two distinct pathologies together. The patient's arm pain and numbness disappeared right after the surgery and symptoms of myelopathy fully recovered at 6-month follow-up. Histological studies confirmed a herniated disc and a meningioma. In rare case, intradural tumor coexists with cervical disc herniation. When suspicious findings were noticed, or clinical symptoms cannot be fully explained, contrast MR imaging is helpful in differential diagnosis. Microscopic transdural discectomy is safe, and could be used as an optional procedure for cervical disc herniation in some cases.

  12. Unstable trochanteric femoral fractures: extramedullary or intramedullary fixation. Review of literature

    NARCIS (Netherlands)

    Schipper, I. B.; Marti, R. K.; van der Werken, Chr

    2004-01-01

    For operative treatment of unstable trochanteric fractures two options exist: extramedullary or intramedullary stabilisation. A review of 18 international papers that compared two different treatment methods for trochanteric fractures, in prospective randomised clinical trials, is presented. In view

  13. A mosaic pattern of INI1/SMARCB1 protein expression distinguishes Schwannomatosis and NF2-associated peripheral schwannomas from solitary peripheral schwannomas and NF2-associated vestibular schwannomas.

    Science.gov (United States)

    Caltabiano, Rosario; Magro, Gaetano; Polizzi, Agata; Praticò, Andrea Domenico; Ortensi, Andrea; D'Orazi, Valerio; Panunzi, Andrea; Milone, Pietro; Maiolino, Luigi; Nicita, Francesco; Capone, Gabriele Lorenzo; Sestini, Roberta; Paganini, Irene; Muglia, Mariella; Cavallaro, Sebastiano; Lanzafame, Salvatore; Papi, Laura; Ruggieri, Martino

    2017-06-01

    The INI1/SMARCB1 gene protein product has been implicated in the direct pathogenesis of schwannomas from patients with one form of schwannomatosis [SWNTS1; MIM # 162091] showing a mosaic pattern of loss of protein expression by immunohistochemistry [93% in familial vs. 55% in sporadic cases]. To verify whether such INI1/SMARCB1 mosaic pattern could be extended to all schwannomas arising in the sporadic and familial schwannomatoses [i.e. to SMARCB1-related (SWNTS1) or LZTR1-related (SWNTS2) schwannomatosis or to SMARCB1/LZTR1-negative schwannomatosis] and whether it could be involved in classical NF2 or solitary peripheral schwannomas METHODS: We blindly analysed schwannoma samples obtained from a total of 22 patients including (a) 2 patients (2 males; aged 38 and 55 years) affected by non-familial SMARCB1-associated schwannomatosis (SWTNS1); (b) 1 patient (1 female; aged 33 years) affected by familial schwannomatosis (SWTNS1/ SMARCB1 germ line mutations); (c) 5 patients (3 males, 2 females; aged 33 to 35 years) affected by non-familial (sporadic) LZTR1-associated schwannomatosis (SWNTS2); (d) 3 patients (3 males; aged 35 to 47 years) affected by familial schwannomatosis (SWTNS2/ LZTR1 germ line mutations); (e) 2 patients (1 male, 1 female; aged 63 and 49 years, respectively) affected by non-familial schwannomatosis (SWTNS, negative for SMARCB1, LZTR1 and NF2 gene mutations); (f) 4 patients (3 males, 1 females; aged 15 to 24 years) affected by classical NF2 (NF2: harbouring NF2 germ line mutations; and (g) 5 patients (3 males, 2 females; aged 33 to 68 years) who had solitary schwannomas. [follow-up = 15-30 years; negative for constitutional/somatic mutation analysis for the SMARCB1, LZTR1 and NF2 genes] were (blindly) analyzed. The INI1/SMARCB1 immunostaining pattern was regarded as (1) diffuse positive nuclear staining [= retained expression] or (2) mosaic pattern [mixed positive/negative nuclei = loss of expression in a subset of tumour cells]. All

  14. Extramedullary versus intramedullary tibial cutting guides in megaprosthetic total knee replacement

    Directory of Open Access Journals (Sweden)

    Karade Vikas

    2012-10-01

    Full Text Available Abstract Background In a standard total knee replacement, tibial component alignment is a key factor for the long term success of the surgery. The purpose of this study is to compare the accuracy of extramedullary and intramedullary tibial cutting guides used in indigenous and imported implants respectively, in positioning of the tibial components in megaprosthetic knee replacements. Methods A comparative study of the accuracy of extramedullary and intramedullary tibial cutting guides was carried out in 92 megaprosthetic knee replacements for distal femoral tumors. For the proximal tibia cut for tibial component placement, an extramedullary guide was used in 65 patients and an intramedullary guide was used in 27 patients. Tibial component alignment angles were measured in postoperative X-rays with the help of CAD software. Results There was more varus placement in coronal plane with extramedullary cutting guide (−1.18 +/− 2.4 degrees than the intramedullary guide (−0.34 +/− 2.31 degrees but this did not reach statistical significance. The goal of 90 +/− 2 degrees alignment of tibial component was achieved in 54% of patients in the extramedullary group versus 67% in the intramedullary group. In terms of sagittal plane alignment, extramedullary guide showed less accurate results (2.09 +/− 2.4 degrees than intramedullary guide (0.50 +/− 3.80 degrees for tibial component alignment, though 78% of patients were aligned within the goal of 0–5 degrees of tibial slope angle in extramedullary group versus 63% in intramedullary group. The mean error in the measurements due to rotation of the knee during taking the X-rays was less than 0.1 degrees and distribution of the X-rays with the rotation of knee was similar in both the groups. Conclusions Overall, in megaprosthetic knee replacement intramedullary guides gave more accurate results in sagittal plane and exhibited similar variability as of extramedullary guides in coronal plane.

  15. Spinal cord compression due to epidural extramedullary haematopoiesis in thalassaemia: MRI

    Energy Technology Data Exchange (ETDEWEB)

    Aydingoez, Ue.; Oto, A.; Cila, A. [Department of Radiology, Hacettepe University School of Medicine, Ankara (Turkey)

    1997-12-01

    Spinal epidural extramedullary haematopoiesis is very rare in thalassaemia. A 27-year-old man with thalassaemia intermedia presented with symptoms and signs of spinal cord compression. MRI showed a thoracic spinal epidural mass, representing extramedullary haematopoietic tissue, compressing the spinal cord. Following radiotherapy, serial MRI revealed regression of the epidural mass and gradual resolution of spinal cord oedema. (orig.) With 3 figs., 6 refs.

  16. Adult-onset intradural spinal teratoma: report of 18 consecutive cases and outcomes in a single center.

    Science.gov (United States)

    Wan, Wei; Yang, Cheng; Yan, Wangjun; Liu, Tielong; Yang, Xinghai; Song, Dianwen; Xiao, Jianru

    2017-07-01

    Eighteen consecutive patients with adult-onset intradural spinal teratoma underwent surgical treatment in our center from 1998 to 2013. Teratoma is defined as a neoplasm composed of elements derived from three germ cell layers (ectoderm, endoderm and mesoderm). Intraspinal teratoma is extremely rare and accounts for 0.2-0.5% of all spinal cord tumors. Moreover, teratoma occurs primarily in neonates and young children. Adult-onset intradural spinal teratoma is even rare. The aim of this study was to discuss the clinical characteristics, diagnosis and therapeutic strategies of adult-onset intradural spinal teratoma. This retrospective study included 18 consecutive adult patients with intradural teratoma who were surgically treated in our center between 1998 and 2013. The clinical features, pathogenesis, diagnostic strategies and surgical outcomes were discussed. Neurological function outcomes were evaluated by the JOA scoring system. Of the 18 included patients, 4 patients received subtotal resection and the other 14 patients received total resection. All the 18 cases were diagnosed with mature teratoma. The mean follow-up period was 79.7 (median 60.5; range 27-208) months. Local recurrence occurred in two of the four patients who underwent subtotal resection and in no patient who underwent total resection. The neurologic status improved in 16 cases and remained unchanged in the other two patients. Adult-onset intradural spinal teratoma is extremely rare. To the best of our knowledge, this is the largest series of patients with this disease. Despite the slow-growth and indolent nature, radical resection remains the recommended treatment to reduce tumor recurrence.

  17. Plexiform schwannoma of the floor of the mouth: a case report

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    Mohammad Koochek Dezfuli

    2017-03-01

    Full Text Available Abstract Plexiform schwannoma is a type of schwannoma that can be occured at any age and any site of the oral cavity. In the gross and histopathological features, it shows plexiform or multinodular growth pattern. This variant of schwannoma is sometimes associated with neurofibromatosis type II or schwannomatosis. Histopathologically, plexiform schwannoma is composed of schwann cells that show Anthony A growth pattern. Mitosis are rare or absent.This study reports a case of a healthy 34 year old female with swelling in floor of her mouth; according to the clinical and histopathological findings, the diagnosis was Plexiform schwannoma.

  18. Pulmonary Extramedullary Hematopoiesis in a Patient with Chronic Asthma Resembling Lung Cancer: A Case Report

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    Massood Hosseinzadeh

    2012-01-01

    Full Text Available Background. Extramedullary hematopoiesis is most often seen in reticuloendothelial organs specially spleen, liver, or lymph nodes, and it is rarely seen in lung parenchyma. Almost all reported cases of pulmonary extramedullary hematopoiesis occurred following myeloproliferative disorders specially myelofibrosis. Other less common underlying causes are thalassemia syndromes and other hemoglobinopathies. There was not any reported case of pulmonary extramedullary hematopoiesis in asthmatic patients in the medical literature. Case. Here we reported a 65-year-old lady who was a known case of bronchial asthma with recent developed right lower lobe lung mass. Chest X-ray and CT studies showed an infiltrating mass resembling malignancy. Fine needle aspiration cytology of mass revealed pulmonary extramedullary hematopoiesis. The patient followed for 10 months with serial physical examination and laboratory evaluations which were unremarkable. Conclusion. Extramedullary hematopoiesis of lung parenchyma can be mistaken for lung cancer radiologically. Although previous reported cases occurred with myelofibrosis or hemoglobinopathies, we are reporting the first case of asthma-associated extramedullary hematopoiesis.

  19. Adrenal extramedullary hematopoiesis associated with β-thalassemia major

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    Bijan Keikhaei

    2012-01-01

    Full Text Available The presence of apparently normal hematopoietic tissue outside of bone marrow cavity is defined as extramedullary hema - topoiesis (EMH. EMH is a rare complication in thalassemia major (TM and adrenal gland as well. This report describes a case of adrenal EMH in a 26-year-old man with β-TM. He has been transfused with regular blood transfusion since 9 months. During the routine physical examination he was incidentally found to have a hypoechoic mass at his abdominal ultrasonography. Abdominal computed tomography scan revealed a right well-defined suprarenal mass 7.7¥7.3¥5.8 cm in size. The diagnosis of EMH was confirmed with ultrasonographic-guided fine needle biopsy. Treatment options which include intensified regular blood transfusion and hydroxyurea have been started.

  20. Adrenal extramedullary hematopoiesis associated with β-thalassemia major.

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    Keikhaei, Bijan; Shirazi, Ahmad Soltani; Pour, Mahboob Mohammad

    2012-05-10

    The presence of apparently normal hematopoietic tissue outside of bone marrow cavity is defined as extramedullary hematopoiesis (EMH). EMH is a rare complication in thalassemia major (TM) and adrenal gland as well. This report describes a case of adrenal EMH in a 26-year-old man with β-TM. He has been transfused with regular blood transfusion since 9 months. During the routine physical examination he was incidentally found to have a hypoechoic mass at his abdominal ultrasonography. Abdominal computed tomography scan revealed a right well-defined suprarenal mass 7.7×7.3×5.8 cm in size. The diagnosis of EMH was confirmed with ultrasonographic-guided fine needle biopsy. Treatment options which include intensified regular blood transfusion and hydroxyurea have been started.

  1. Extramedullary hematopoiesis: a rare occurrence in the sinonasal tract.

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    Bizzoni, Andrea; Lombardi, Davide; Maroldi, Roberto; Incardona, Paolo; Nicolai, Piero

    2010-04-01

    Extramedullary hematopoiesis (EMH) is a systemic reaction to inadequate hematopoiesis. We report two exceedingly rare cases of EMH involving the paranasal sinuses. The first patient, a 30-year-old man, presented with a maxillary sinus mass. The lesion was excised by endoscopic surgery: definitive histology identified foci of EMH within an inflammatory fibromyxoid pseudotumor. The second case occurred in a 29-year-old man affected by intermediate beta-thalassemia. He was hospitalized with a diagnosis of sphenoid sinus mucocele secondary to an ethmoid lesion. The patient underwent endoscopic excision of the mass and drainage of the sphenoid mucocele. At definitive histology, a diagnosis of EMH was established. Herein, the presenting modalities, imaging profile, and treatment options of this rare EMH localization are reviewed. Copyright (c) 2009 Elsevier Ireland Ltd. All rights reserved.

  2. Paraspinal extramedullary hematopoiesis in patients with thalassemia intermedia.

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    Haidar, Rachid; Mhaidli, Hani; Taher, Ali T

    2010-06-01

    Ineffective erythropoiesis in patients with thalassemia intermedia drives extramedullary hematopoietic tumor formation in several parts of the body. Paraspinal involvement has received increasing attention due to the associated morbidity secondary to spinal cord compression. Although the history and physical examination may help narrow the differential diagnosis, radiographic imaging remains essential to confirm the existence of hematopoietic tissue. Characteristic appearance has been observed mainly on magnetic resonance imaging. Several treatment options have been described, including transfusion therapy, laminectomy, radiotherapy, and the use of fetal hemoglobin inducing agents that decrease the hematopoietic drive. However, the ideal management scheme remains controversial. Until large prospective trials evaluate the efficacy and safety of the available treatment options, both in single and in combination therapy, an individualized approach should be entertained.

  3. Extramedullary spinal teratoma presenting with recurrent aseptic meningitis.

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    Mpayo, Lucy L; Liu, Xiao-Hong; Xu, Man; Wang, Kai; Wang, Jiao; Yang, Li

    2014-06-01

    Spinal teratomas are extremely rare; they constitute meningitis. A 7-year-old boy presented with paroxysmal abdominal pain and a history of recurrent aseptic meningitis. Kernig and Brudzinski signs were present. Lumber puncture revealed pleocytosis with no evidence of bacteria growth. Imaging of the spine revealed a cystic lesion in spinal cord at thoracic level 9-11. Endoscopic excision of the cyst was successfully performed. Surgical and histopathological findings confirmed extramedullary matured teratoma. As the symptomatic attacks of spontaneous rupture of spinal teratoma resemble presentations of Mollaret meningitis, spinal teratoma should be considered in the differential diagnosis of Mollaret meningitis. We describe a rare example of spinal teratoma causing recurrent meningitis. Spine imaging should be considered in individuals with recurrent aseptic meningitis as this promotes earlier diagnosis, more appropriate treatment, and improved neurological outcome. Copyright © 2014 Elsevier Inc. All rights reserved.

  4. Orbital Cystic Schwannoma Originating from the Frontal Nerve

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    Yasuhiko Hayashi

    2012-01-01

    Full Text Available Schwannomas of the orbit are very rare benign neoplasms. Intraorbital cystic schwannomas originating from the frontal nerve are even rarer, with only 1 case reported to date. This is most likely due to the fact that, in most cases, the origin of the orbital schwannoma cannot be identified intraoperatively. The nerve origin is usually speculated from histological examination of the specimen and the postoperative neurological deficits of the patient. Here, we present the case of a 65-year-old woman with a one-month history of exophthalmos, whose orbital cystic lesion was completely removed by microsurgical transcranial operation. Intraoperatively, the continuity between the tumor and frontal nerve was seen macroscopically, leading us to confirm the frontal nerve as an origin of the tumor, which was consistent with the postoperative neurological findings. The diagnosis of the tumor was established as schwannoma from the histological examination. As a differential diagnosis of the orbital cystic lesions, the possibility of schwannomas should be kept in mind.

  5. Gastric schwannomas: radiological features with endoscopic and pathological correlation

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    Hong, H.S. [Department of Radiology, Severance Hospital, Yonsei University College of Medicine, Seodaemoon-gu, Seoul (Korea, Republic of); Ha, H.K. [Department of Radiology, University of Ulsan College of Medicine, Songpa-gu, Seoul (Korea, Republic of)], E-mail: hkha@amc.seoul.kr; Won, H.J.; Byun, J.H.; Shin, Y.M.; Kim, A.Y.; Kim, P.N.; Lee, M.-G. [Department of Radiology, University of Ulsan College of Medicine, Songpa-gu, Seoul (Korea, Republic of); Lee, G.H. [Internal Medicine, University of Ulsan College of Medicine, Songpa-gu, Seoul (Korea, Republic of); Kim, M.J. [Pathology, Asan Medical Center, University of Ulsan College of Medicine, Songpa-gu, Seoul (Korea, Republic of)

    2008-05-15

    Aim: To describe the radiological, endoscopic, and pathological findings of gastric schwannomas in 16 patients. Materials and methods: The radiological, endoscopic, and pathological findings of 16 surgically proven cases of gastric schwannoma were retrospectively reviewed. All patients underwent computed tomography (CT) and four patients were evaluated with upper gastrointestinal series. Two radiologists reviewed the CT and upper gastrointestinal series images by consensus with regard to tumour size, contour, margin, and growth pattern, the presence or absence of ulcer, cystic change, and the CT enhancement pattern. Endoscopy was performed in eight of these 16 patients. Six patients underwent endoscopic ultrasonography. Pathological specimens were obtained from and reviewed in all 16 patients. Immunohistochemistry was performed for c-kit, CD34, smooth muscle actin, and S-100 protein. Results: On radiographic examination, gastric schwannomas appeared as submucosal tumours with the CT features of well-demarcated, homogeneous, and uncommonly ulcerated masses. Endoscopy with endoscopic ultrasonography demonstrated homogeneous, submucosal masses contiguous with the muscularis propria in all six examined cases. On pathological examination, gastric schwannomas appeared as well-circumscribed and homogeneous tumours in the muscularis propria and consisted microscopically of interlacing bundles of spindle cells. Strong positivity for S-100 protein was demonstrated in all 16 cases on immunohistochemistry. Conclusion: Gastric schwannomas appear as submucosal tumours of the stomach and have well-demarcated and homogeneous features on CT, endoscopic ultrasonography, and gross pathology. Immunohistochemistry consistently reveals positivity for S-100 protein in the tumours.

  6. Parathyroid Adenoma, Gastric Adenocarcinoma, and Intraabdominal Schwannoma in One Patient

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    Gülşah Elbüken

    2016-09-01

    Full Text Available Intraabdominal schwannomas, which occur quite rarely, are usually benign tumors. They are often discovered coincidentally when abdominal scans are performed for other reasons. We also coincidentally detected an intraabdominal schwannoma in addition to primary hyperparathyroidism related to a parathyroid adenoma and a gastric adenoma which caused partial pyloric obstruction in a 69-year-old female patient who was admitted to our emergency room with vomiting while we were further investigating hypercalcemia that was found during laboratory workup. It is rare to diagnose multiple tumors concurrently in a single patient which are components of certain neuroendocrine syndromes themselves. It is more interesting to see a rare tumor such as intraabdominal schwannoma coexisting with a parathyroid adenoma that can be a component of multiple endocrine neoplasia syndromes. In the literature, there are few case reports of the coexistence of intraabdominal schwannomas with adenocarcinoma of the gastrointestinal tract. Here, we present an unusual case of intraabdominal schwannoma coexisting with parathyroid adenoma and gastic adenocarcinoma, all diagnosed in a single patient. To our best knowledge, this is the first case report of such a rare coexistence which makes it rather interesting.

  7. Intracranial extramedullary hematopoiesis masquerading as progressive metastasis in a child with stage 4 neuroblastoma: Utility of sulfur colloid scan

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    Sajid S. Qureshi

    2016-10-01

    Full Text Available Extramedullary hematopoiesis is production of blood cells outside bone marrow which occurs in a variety of disorders including myelofibrosis. We present a 15 month old child with stage 4 neuroblastoma with secondary myelofibrosis which resulted in intracranial extramedullary hematopoiesis, posing a diagnostic and therapeutic dilemma. This was resolved with a sulfur colloid isotope scan which confirmed extramedullary hematopoiesis and not a progressing metastasis. This helped us continue therapy with curative intent. To the best of our knowledge we did not come across any report of neuroblastoma with myelofibrosis and intracranial extramedullary hematopoiesis mimicking progression of metastasis.

  8. [A Case of Pelvic Schwannoma, Mimicking Metastasis of Rectal Carcinoma].

    Science.gov (United States)

    Watanabe, Yuichiro; Baba, Hiroyuki; Matsuzawa, Takeaki; Fukuchi, Minoru; Kumagai, Youichi; Ishibashi, Keiichiro; Mochiki, Erito; Ishida, Hideyuki

    2015-11-01

    Schwannoma in the lateral lymph node region is extremely rare; however, this tumor has been reported to have relatively high SUV on PET-CT, suggestive of malignancy. A 67-year-old man with advanced lower rectal cancer had a small nodule with FDG accumulation (SUVmax 2.6) near the left internal iliac artery. His preoperative diagnosis was rectal cancer with lateral lymph node metastasis. He underwent super-low anterior resection with lateral lymph node dissection. Histopathological examination was conclusive for pT3 (A), with an Rt263D lymph node metastasis. Interestingly, a schwannoma was detected among the harvested lymph nodes. Although rectal cancer is known to involve pelvic lymph nodes, the role of preoperative diagnosis with FDG-PET is unclear. We should consider that schwannoma is associated with slight elevation of SUVmax and it may mimic lymph node metastasis.

  9. Retroperitoneal schwannoma: diagnostic imaging findings in 5 patients

    International Nuclear Information System (INIS)

    Baltazar, Alberto; Santamarina, Mario; Scalise, Gabriela; Ponce de Leon, Valeria; Bello, Lorena

    2003-01-01

    Purpose: To evaluate the different imaging findings (US, CT and MRI) in retroperitoneal schwannoma. Materials and methods: 5 patients (3 male and 2 females) with a diagnosis of retroperitoneal schwannoma were retrospectively evaluated. Ages ranged from 33 to 63 years (means 54 years). The images (US, CT and MR) were analyzed and correlated to histopathologic results. Results: The most frequent clinical finding was abdominal pain (60%). A presumptive diagnosis prior to surgery was suggested in 3/5 cases that had well-defined masses with a predominant cystic appearance. All patients underwent surgery with tumoral resection. Only 2 patients (40%) had recurrence within a three years period of follow-up. Conclusion: Retroperitoneal schwannoma is an infrequent tumor. In our series, no pathognomotic features were observed on US, CT or MRI. However, 3/5 tumors showed high signal intensity on T2-weighted images due to cystic areas. (author)

  10. Laparoscopic Resection of Schwannoma of the Ascending Colon

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    Yoshihiko Tashiro

    2015-01-01

    Full Text Available Schwannomas of the colon are rare and difficult to diagnose preoperatively. We report a case of schwannoma of the ascending colon that was resected laparoscopically. A 64-year-old woman was referred to our hospital by her local clinic for further evaluation and management of a submucosal tumor of the ascending colon. A definitive preoperative diagnosis could not be reached despite examinations. Gastrointestinal stromal tumor, leiomyoma and lymphoma were the differential diagnoses. We performed a laparoscopic right hemicolectomy with D2 lymph node dissection. Histological findings with hematoxylin-eosin staining revealed spindle-like tumor cells, and immunohistochemical analysis showed that the tumor was positive for S-100 but negative for c-kit, CD34, smooth muscle actin and desmin, with a Ki-67 index of <5%. Thus, the diagnosis in this case was benign schwannoma of the ascending colon.

  11. Gastric Schwannoma or GIST: accuracy of preoperative diagnosis?

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    Guthrie, G; Mullen, R; Moses, A

    2011-11-01

    Mesenchymal cell tumours of the gastrointestinal tract are rare in western society. Gastrointestinal stromal tumour (GIST) is the most common of this group of tumours. Gastric schwannoma is rarer, accounting for only 0.2% of all gastric tumours and 4% of all benign gastric neoplasms. Indeed, schwannoma has only been recognized as a primary gastrointestinal tumour in the last 20 years through advances in pathological techniques. We report a rare case of gastric schwannoma, the endoscopic and radiological features of which were indistinguishable from a GIST. Due to the diagnostic uncertainty, surgical resection is the treatment of choice. Development of more reliable diagnostic methods, such as endoscopic core biopsy, may help the accuracy of preoperative diagnosis.

  12. An Unusual Differential Diagnosis of Orbital Cavernous Hemangioma: Ancient Schwannoma

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    Sara Filipa Teixeira Ribeiro

    2017-05-01

    Full Text Available Schwannomas are rare lesions of the orbit that can be confused with cavernous hemangioma on imaging studies. We report the case of an 84-year-old woman with a 9-year history of a tumoral lesion in the inferolateral left orbit. The imaging studies did not reveal specific characteristics, only bone remodeling due to the long evolution of the tumor. The patient underwent complete excision of the tumor by anterior orbitotomy via the inferior conjunctival fornix. The histopathological examination revealed an ancient schwannoma, a variant of schwannoma with uncommon histological features. The follow-up was uneventful. The present case emphasizes the importance of considering neural tumors in the differential diagnosis of orbital masses with bone changes and degenerative alterations such as hemorrhagic areas, cysts, and/or calcifications.

  13. Large intradural craniospinal arachnoid cyst: A case report and review of literature

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    Souvagya Panigrahi

    2012-01-01

    Full Text Available Presence of an arachnoid cyst at craniospinal junction is not very common. This is a very rare anatomic site, with only seven other cases reported in the literature. We report a case of large intradural craniospinal arachnoid cyst presenting with obstructive hydrocephalus and cranial nerve palsy. A 39-year-old male presented with 8-month history of neck pain, headache, vomiting, visual disturbances, diminished taste sensation, and numbness of face. He had bilateral papilledema on ophthalmoscopy. Magnetic resonance imaging (MRI revealed a posterior fossa arachnoid cyst extending down to the lower border of C5 vertebra. Posterior decompression was done through C5 laminectomy. He made a full recovery and was asymptomatic at 6-month follow-up examination. The clinical features, diagnosis, and management of these rare craniospinal arachnoid cysts are discussed.

  14. Laparoscopic resection of a gastric schwannoma: A case report.

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    Vargas Flores, Edgar; Bevia Pérez, Francisco; Ramirez Mendoza, Pablo; Velázquez García, José Arturo; Ortega Román, Oscar Alejandro

    2016-01-01

    Mesenchymal tumors of the gastrointestinal tract are a group spindle cell tumors which include gastrointestinal stromal tumors, leiomyomas, leiomyosarcomas and schwannomas (Nishida and Hirota, 2000). Schwannomas generally present as a slow and asymptomatic growing mass in the gastrointestinal tract typically arising in the gastric submucosa accounting for up to 0.2% of gastric tumors (Melvin and Wilkinson, 1993; Sarlomo-Rikala M, Miettinen, 1995). with negative surgical margin resection (as approached in this case) is considered the standard treatment. A 60-year-old woman was referred to our general surgery service for dyspepsia. During her evaluation a gastric mass was incidentally found on upper GI endoscopy which showed a submucosal exophytic neoplasm at the gastric antrum. The patient was discharged following an uneventful recovery from a successful surgical laparoscopic tumor resection. Schwannomas are benign neurogenic tumors that originate from Schwann cells. They commonly occur in the head and neck but are rare in the GI tract (Menno et al., 2010). The differential diagnosis between gastric schwannomas and GISTs can be difficult in the preoperative assessment. With the advent of immunohistochemical staining techniques it is now possible to make a differential diagnosis based on their distinctive immunophenotypes. Gastric schwannomas are consistently positive for S-100 protein and negative for c-kit; conversely, 95% of GISTs are positive for c-kit and negative for S-100 protein in up to 98 to 99% of the cases. Gastric schwannomas should be included in the differential diagnosis of any gastric submucosal mass. Negative margin resection as seen with this patient is the standard surgical treatment as there is low malignant transformation potential. Copyright © 2016 The Author(s). Published by Elsevier Ltd.. All rights reserved.

  15. Gastric schwannomas revisited: has precise preoperative diagnosis become feasible?

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    Fujiwara, Shinichi; Nakajima, Kiyokazu; Nishida, Toshirou; Takahashi, Tsuyoshi; Kurokawa, Yukinori; Yamasaki, Makoto; Miyata, Hiroshi; Takiguchi, Shuji; Mori, Masaki; Doki, Yuichiro

    2013-07-01

    Gastric schwannomas are not common but are clinically important in terms of differential diagnosis from other submucosal lesions. The precise preoperative diagnosis, however, has been challenging mainly owing to the lack of specific findings in conventional imaging studies. The aim of this study was to revisit the possibilities and limitations of modern preoperative diagnostic modalities for gastric schwannomas. Fourteen consecutive patients with a final pathological diagnosis of gastric schwannoma were retrospectively analyzed. Data included demographics, preoperative imaging studies/diagnosis, surgery, histopathology, and follow-up results. The series included 6 males and 8 females, with a median age of 49 years (range 26-68 years). No symptoms were presented, except for 1 patient with epigastric pain. The tumors were located in the upper (n = 5), middle (3), and lower stomach (6), with a median size of 41 mm (range 20-75 mm). Twelve schwannomas (86%) showed homogeneous enhancement on computed tomography. Ulceration was seen on endoscopy in 4 of 12 available cases (33%). Positron emission tomography was performed in the last 4 patients, showing fluorodeoxy-glucose uptake in all cases (100%). A preoperative diagnosis of schwannoma was not obtained in the majority of cases (13/14, 93%); only 1 case was correctly diagnosed, by endoscopic aspiration cytology. Laparoscopic partial gastrectomy was attempted and completed in 13 cases. The patients have been followed up for 4.7 years (range 2.1-20.3 years), with no recurrencesor metastases and acceptable gastrointestinal function. The precise preoperative diagnosis of gastric schwannomas remains difficult even with modern imaging studies. Surgery, therefore, should be positively considered for patients without a conclusive preoperative diagnosis.

  16. Acute median nerve palsy due to hemorrhaged schwannoma: case report

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    Kotan Dilcan

    2007-09-01

    Full Text Available Abstract Schwannomas are common, benign nerve tumors originating from the sheath of peripheral nerves. In this article, a 54 year old woman suffered from sudden onset motor and sensory deficit at her first radial three fingers on her right hand. Radiological investigations were normal. Electromyography diagnosed a median nerve entrapment neuropathy and urgent surgery was performed. Interestingly, a hemorrhaged mass was detected in the median nevre at the proximal end of the carpal ligament and was resected totally. Histopathological diagnosis was Schwannoma. The patient maintained a healthy status for five years.

  17. A Usual Schwannoma at an Unusual Site-The Mesorectum

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    Jaya Manchanda

    2016-07-01

    Full Text Available The aim of the article is to present a rare case of mesorectal schwannoma diagnosed by fine needle aspiration cytology in a 36 year old man. Schwannomas are benign encapsulated tumors arising from Schwann cells of motor and peripheral nerves. They commonly present on the head, neck and trunk and on rare occasion arise in the retroperitoneum and in the lumbosacral region. It is however quite uncommon for a schwanomma to originate in the mesorectum. In evaluating such cases, FNAC provides a safe and effective method to resolve the great diagnostic dilemma.

  18. Gastric Schwannoma: A Postoperative Surprise A Case Report.

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    Abdelmounaim Ait Ali

    2014-06-01

    Full Text Available Gastric Schwannoma is a rare, slow-growing, and clinically non-specific submucosal tumor, originating from Schwann cells with excellent prognosis after surgical resection.We report a clinical case of a patient presented with gastric schwannoma revealed by non-specific gastric signs and of which the definitive diagnosis is done through immunohistochemistry of the resected specimen, showing strong S100 protein positivity. The evolution is favorable after a partial gastrectomy with a decline of two years. Through this case, we are trying to trace the rarity, strong similarities with gastric stromal tumors and especially, the weak index of suspicion for this diagnosis.

  19. Gastric Schwannoma: A Case Report and Review of Literature.

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    Sreevathsa, M R; Pipara, Gotam

    2015-06-01

    Schwannomas are usually benign, slow growing tumors, that originate from any nerve that has a Schwann cell sheath. Here, we report the case of a 40 year-old female patient with an incidentally noted submucosal gastric tumor while being evaluated for cervical lymphadenopathy as a part of workup for lymphoma. She underwent sleeve resection of the stomach under suspicion of a gastrointestinal stromal tumor, but postoperative histopathological and immunohistochemical findings confirmed the diagnosis of shwannoma. Although schwannomas are mostly benign, they are often indistinguishable preoperatively from malignant tumors such as gastrointestinal stromal tumors. Therefore, resection is the treatment of choice for all such tumors.

  20. Back pain in patients with degenerative spine disease and intradural spinal tumor: what to treat? when to treat?

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    Bellut, David; Mutter, Urs M; Sutter, Martin; Eggspuehler, Andreas; Mannion, Anne F; Porchet, François

    2014-04-01

    Back pain is common in industrialized countries and one of the most frequent causes of work incapacity. Successful treatment is, therefore, not only important for improving the symptoms and the quality of life of these patients but also for socioeconomic reasons. Back pain is frequently caused by degenerative spine disease. Intradural spinal tumors are rare with an annual incidence of 2-4/1,00,000 and are mostly associated with neurological deficits and radicular and nocturnal pain. Back pain is not commonly described as a concomitant symptom, such that in patients with both a tumor and degenerative spine disease, any back pain is typically attributed to the degeneration rather than the tumor. The aim of the present retrospective investigation was to study and analyze the impact of microsurgery on back/neck pain in patients with intradural spinal tumor in the presence of degenerative spinal disease in adjacent spinal segments. Fifty-eight consecutive patients underwent microsurgical, intradural tumor surgery using a standardized protocol assisted by multimodal intraoperative neuromonitoring. Clinical symptoms, complications and surgery characteristics were documented. Standardized questionnaires were used to measure outcome from the surgeon's and the patient's perspectives (Spine Tango Registry and Core Outcome Measures Index). Follow-up included clinical and neuroradiological examinations 6 weeks, 3 months and 1 year postoperatively. Back/neck pain as a leading symptom and coexisting degenerative spine disease was present in 27/58 (47 %) of the tumor patients, and these comprised to group under study. Patients underwent tumor surgery only, without addressing the degenerative spinal disease. Remission rate after tumor removal was 85 %. There were no major surgical complications. Back/neck pain as the leading symptom was eradicated in 67 % of patients. There were 7 % of patients who required further invasive therapy for their degenerative spinal disease. Intradural

  1. Stereotactic radiation therapy for large vestibular schwannomas

    International Nuclear Information System (INIS)

    Mandl, Ellen S.; Meijer, Otto W.M.; Slotman, Ben J.; Vandertop, W. Peter; Peerdeman, Saskia M.

    2010-01-01

    Background and purpose: To evaluate the morbidity and tumor-control rate in the treatment of large vestibular schwannomas (VS) after stereotactic radiation therapy in our institution. Material and methods: Twenty-five consecutive patients (17 men, 8 women) with large VS (diameter 3.0 cm or larger), treated with stereotactic radiotherapy (SRT) or stereotactic radiosurgery (SRS) between 1992 and 2007, were retrospectively studied after a mean follow-up period of three years with respect to tumor-control rate and complications. Results: Actuarial 5-year maintenance of pre-treatment hearing level probability of 30% was achieved. Five of 17 patients suffered permanent new facial nerve dysfunction. The actuarial 5-year facial nerve preservation probability was 80%. Permanent new trigeminal nerve neuropathy occurred in two of 15 patients, resulting in an actuarial 5-year trigeminal nerve preservation probability of 85%. Tumor progression occurred in four of 25 (16%) patients. The overall 5-year tumor control probability was 82%. Conclusion: Increased morbidity rates were found in patients with large VS treated with SRT or SRS compared to the published series on regular sized VS and other smaller retrospective studies on large VS.

  2. Hybrid schwannoma-perineurioma of the gastrointestinal tract: a clinicopathologic study of 2 cases and reappraisal of perineurial cells in gastrointestinal schwannomas.

    Science.gov (United States)

    Agaimy, Abbas; Michal, Michal

    2011-10-01

    Soft tissue neoplasms with features of both schwannoma and perineurioma (hybrid schwannoma-perineurioma) have been increasingly recognized in recent years. To date, only a single case of this entity has been documented in the gastrointestinal tract (sigmoid colon). We herein describe 2 new cases of this entity. For comparison, we reevaluated 12 classic gastrointestinal schwannomas for the perineurial cell component. The 2 hybrid schwannoma-perineuriomas were detected incidentally in the gastric antrum and the vermiform appendix in a 50-year-old woman and a 17-year-old man during surgery for gastric GIST and appendicitis-like symptoms, respectively. None of the patients had neurofibromatosis 1 or 2. Patients were alive with no evidence of recurrence or new tumors at 8 and 12 months, respectively. The tumors measured 1.2 cm and 1.5 cm in size. Histologically, they showed prominent storiform, lamellar, and fascicular patterns. Notably, both lacked peripheral lymphoid cuffs and the trabecular pattern of gastrointestinal schwannomas. Both tumors coexpressed protein S100 (≥80%), CD34 (80%), and the perineurial cell markers (20% to 40% of tumor cells). The perineurial cell component formed alternating fascicles with S100-positive cells throughout the neoplasm. Reevaluation of 12 classic gastrointestinal schwannomas showed isolated claudin-1/epithelial membrane antigen-positive cells. However, 4 schwannomas (33%) strongly expressed glucose transporter-1 in most of the tumor cells indicating its limited specificity in this setting. Compared with gastrointestinal schwannomas, CD34 expression was stronger and more diffuse in hybrid schwannoma-perineuriomas. We conclude that hybrid schwannoma-perineuriomas are distinct from gastrointestinal schwannoma, both histologically and immunohistochemically.

  3. Extracranial schwannoma in the carotid space: A retrospective review of 91 cases.

    Science.gov (United States)

    Zheng, Xiaoke; Guo, Kai; Wang, Hongshi; Li, Duanshu; Wu, Yi; Ji, Qinghai; Shen, Qiang; Sun, Tuanqi; Xiang, Jun; Zeng, Wei; Chen, Yaling; Wang, Zhuoying

    2017-01-01

    Schwannomas of the vagus nerve and cervical sympathetic nerve are rare; hence, only limited information exists regarding their diagnosis and clinical management. We conducted a retrospective review of the clinical features, imaging studies, and treatment results of patients with schwannoma of the vagus nerve and schwannoma of the sympathetic nerve. Of 91 patients, 91% (n = 83) were preoperatively diagnosed with schwannoma tumors. Using the hyoid bone as an anatomic landmark, the location of the schwannoma of the vagus nerve in the carotid space was significantly different to the location of schwannoma of the sympathetic nerve (p = .003). Although 52 of the 76 patients followed up (68%) had postoperative nerve weaknesses, 13 patients (50%) and 14 patients (53.8%), respectively, fully recovered from schwannoma of the vagus nerve and schwannoma of the sympathetic nerve. In the carotid space, schwannomas of the vagus nerve are usually located below the hyoid bone, whereas schwannomas of the sympathetic nerve more commonly arise from the suprahyoid compartment. Accurate preoperative diagnosis and the intracapsular enucleation surgical approach decreased the incidence of postoperative morbidity. © 2016 Wiley Periodicals, Head Neck 39: 42-47, 2017. © 2016 Wiley Periodicals, Inc.

  4. Gastric Schwannoma: A Rare but Important Differential Diagnosis of a Gastric Submucosal Mass

    Directory of Open Access Journals (Sweden)

    William Yoon

    2012-01-01

    Full Text Available Schwannomas are generally slow growing asymptomatic neoplasms that rarely occur in the GI tract. However, if found, the most common site is the stomach. Gastrointestinal stromal tumors (GISTs are the most common mesenchymal tumors of the gastrointestinal tract, and 60–70% of them occur in the stomach. Owing to their typical presentation as submucosal neoplasms, gastric schwannomas and GISTs appear grossly similar. Accordingly, the differential diagnosis for a gastric submucosal mass should include gastric schwannomas. Furthermore, GI schwannomas are benign neoplasms with excellent prognosis after surgical resection, whereas 10–30% of GISTs have malignant behavior. Hence, it is important to distinguish gastric schwannomas from GISTs to make an accurate diagnosis to optimally guide treatment options. Nevertheless, owing to the paucity of gastric schwannomas, the index of suspicion for this diagnosis is low. We report a rare case of gastric schwannoma in 53-year-old woman who underwent laparoscopic partial gastrectomy under the suspicion of a GIST preoperatively but confirmed to have a gastric schwannoma postoperatively. This case underscores the importance of including gastric schwannomas in the differential diagnosis when preoperative imaging studies reveal a submucosal, exophytic gastric mass. For a gastric schwannoma, complete margin negative surgical resection is the curative treatment of choice.

  5. Gastric schwannoma: a rare but important differential diagnosis of a gastric submucosal mass.

    Science.gov (United States)

    Yoon, William; Paulson, Kari; Mazzara, Paul; Nagori, Sweety; Barawi, Mohammed; Berri, Richard

    2012-01-01

    Schwannomas are generally slow growing asymptomatic neoplasms that rarely occur in the GI tract. However, if found, the most common site is the stomach. Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract, and 60-70% of them occur in the stomach. Owing to their typical presentation as submucosal neoplasms, gastric schwannomas and GISTs appear grossly similar. Accordingly, the differential diagnosis for a gastric submucosal mass should include gastric schwannomas. Furthermore, GI schwannomas are benign neoplasms with excellent prognosis after surgical resection, whereas 10-30% of GISTs have malignant behavior. Hence, it is important to distinguish gastric schwannomas from GISTs to make an accurate diagnosis to optimally guide treatment options. Nevertheless, owing to the paucity of gastric schwannomas, the index of suspicion for this diagnosis is low. We report a rare case of gastric schwannoma in 53-year-old woman who underwent laparoscopic partial gastrectomy under the suspicion of a GIST preoperatively but confirmed to have a gastric schwannoma postoperatively. This case underscores the importance of including gastric schwannomas in the differential diagnosis when preoperative imaging studies reveal a submucosal, exophytic gastric mass. For a gastric schwannoma, complete margin negative surgical resection is the curative treatment of choice.

  6. Intradural pathology and pathophysiology associated with Chiari I malformation in children and adults with and without syringomyelia.

    Science.gov (United States)

    Dlouhy, Brian J; Dawson, Jeffrey D; Menezes, Arnold H

    2017-12-01

    OBJECTIVE The pathophysiology underlying tonsillar herniation and CSF obstruction in Chiari malformation Type I (CM-I) is unclear, and the cause of CM-I-associated syringomyelia is not well understood. A better understanding of this pathophysiology is important for an improved treatment strategy. Therefore, the authors sought to identify, characterize, and examine the intradural pathology and CSF flow pathophysiology in the posterior fossa and at the level of the foramen magnum that occurs in the setting of CM-I. They determined the incidence of these intradural findings and assessed differences across age, with the degree of tonsillar herniation, and in the presence and absence of syringomyelia. METHODS A prospective database initiated in March 2003 recorded all intraoperative findings during surgical treatment of children and adults with CM-I with or without syringomyelia. A total of 389 surgeries for CM-I were performed in 379 patients between March 2003 and June 2016. A total of 109 surgeries were performed in 109 patients with CM-I (without osseoligamentous abnormalities) in whom both a posterior fossa extradural and intradural decompression with duraplasty was performed (first-time intradural procedures). Using a surgical microscope, intradural pathology and obstruction of CSF channels were identified and assessed. Student t-tests and Fisher's exact tests compared groups in a series of univariate analyses, followed by multivariate logistic regression. RESULTS The following intradural pathological entities were observed (prevalence noted in parentheses). These include those that did not obstruct CSF flow channels: opacified arachnoid (33.0%), thickened arachnoid (3.7%), ischemic and gliotic tonsils (40.4%), tonsillar cysts (0.9%), and inferior descent of the fourth ventricle and cervicomedullary junction (CMJ) (78.0%). The following intradural pathological entities were observed to obstruct CSF flow channels: medialized tonsils (100%), tonsil overlying and

  7. Fractionated stereotactic radiotherapy of vestibular schwannomas accelerates hearing loss

    DEFF Research Database (Denmark)

    Rasmussen, Rune; Claesson, Magnus; Stangerup, Sven-Eric

    2012-01-01

    To evaluate long-term tumor control and hearing preservation rates in patients with vestibular schwannoma treated with fractionated stereotactic radiotherapy (FSRT), comparing hearing preservation rates to an untreated control group. The relationship between radiation dose to the cochlea...... and hearing preservation was also investigated....

  8. Increasing annual incidence of vestibular schwannoma and age at diagnosis

    DEFF Research Database (Denmark)

    Stangerup, Sven-Eric; Tos, Mirko; Caye-Thomasen, Per

    2004-01-01

    During the last 26 years the annual number of diagnosed vestibular schwannomas (VS) has been increasing. The aim of this study is to describe and analyse this increase. Since 1976, 1446 new cases of VS have been diagnosed at the authors' centre. Special focus was on the age at diagnosis, the loca...

  9. Schwannoma of the Recurrent Laryngeal Nerve : A Rare Entity

    NARCIS (Netherlands)

    de Heer, Linda M; Teding van Berkhout, F; Priesterbach, Loudy; Buijsrogge, Marc P

    Neurogenic tumors are the most common posterior mediastinal tumors in adults. Schwannomas originating from the recurrent laryngeal nerve are rare. The present study describes a 46-year-old man with a tumor in the left superior mediastinum. Because of the narrow relationship with the aorta and the

  10. Varied Presentation of Schwannoma – A Case Study

    Directory of Open Access Journals (Sweden)

    Ruquaya Mir

    2010-10-01

    Full Text Available Schwannomas can occur anywhere in the body with unusual presentation. They are difficult to diagnose preoperatively. Fine needle aspiration biopsy does not appear to provide an accurate preoperative diagnosis. Complete excision of the mass should be the goal of surgical excision.

  11. Retropharyngeal Schwannoma Excised Through a Transoral Approach: A Case Report

    Directory of Open Access Journals (Sweden)

    Chia-Ying Hsieh

    2006-09-01

    Full Text Available The contents of the retropharyngeal space are limited to fat and retropharyngeal nodes. Primary tumors originating from the retropharyngeal space are rare. More than 25% of schwannomas are found in the head and neck region, and they are rarely found in the retropharyngeal space. Here, we report the case of a 44-year-old woman with a schwannoma confined to the left retropharyngeal space, who presented with snoring and a mild lump in the throat sensation. Physical examination revealed anterior bulging of the left oropharyngeal wall, with intact mucosa. Magnetic resonance imaging showed a well-defined, encapsulated tumor in the left retropharyngeal space with bright signal intensity on T2-weighted images and low signal intensity on T1-weighted images, which was strongly enhanced after gadolinium administration. The tumor was removed through a transoral approach, resulting in a short postoperative recovery time without complications. The pathologic diagnosis was schwannoma. The patient has been well and free of tumor recurrence for 2 years. From anatomic and physiologic viewpoints, excision through a transoral approach is a good choice for a confined retropharyngeal schwannoma.

  12. [Difficult and unusual diagnostic and therapeutic gastric Schwannoma case].

    Science.gov (United States)

    Frejlich, Ewelina; Rudno-Rudzińska, Julia; Hałoń, Agnieszka; Kielan, Wojciech; Grzebianiak, Zygmunt

    2013-01-01

    Schwannoma is a rare, usually benign, generally slow growing, asymptomatic mesenchymal neoplasm derived from nerve cells. In the gastrointestinal tract the most common localization is stomach and the gastric schwannomas represent about 0.2% of all gastric neoplasms. We present a case of 44-years-old male admitted to 2nd Department of General Surgery and Oncological Surgery Medical University for treatment of a submucosal gastric tumor detected in upper gastrointestinal endoscopy, endoscopic ultrasonography and computed tomography (CT). Patient presented with no gastrointestinal disorders and the random endoscopy revealed a gastric tumor, but the biopsy of the lesion showed no carcinomas' cells and the suspicion of GIST. CT confirms the presence of the 5 cm large gastric tumor and intraabdominal lymphadenopathy. The patient was directed to surgery because of the malignant risk. The subtotal gastrectomy with BII anastomosis was performed and no perioperative complications were observed. The postoperative histopathological examination revealed a typical morphology and immunophenotype of tumor. The neoplastic cells were immunoreactive with S-100 protein, but lacked immunoreactivity with CD 117, CD 34 and smooth-muscle actin (SMA). The histopathologic features and immunohistochemical staining pattern were consistent with a gastric schwannoma. The lymph nodes resected, during the operation revealed reactive inflammatory changes without evidence of neoplastic cells and any malignancy. 10-month after the surgery patient has no complains but the follow up will be continued. This case underscores the importance of including gastric schwannomas in the differential diagnosis when preoperative imaging studies reveal a submucosal, exophytic gastric mass.

  13. Gastric plexiform schwannoma in association with neurofibromatosis type 2.

    Science.gov (United States)

    Kudose, Satoru; Kyriakos, Michael; Awad, Michael Magdi

    2016-12-01

    Plexiform schwannoma (PS) is an uncommon variant of schwannoma characterized by a multinodular (plexiform) growth pattern. It comprises up to 5 % of all schwannomas. The association between PS and neurofibromatosis type 1 or type 2 (NF1/NF2) is only rarely reported. Most cases of PS occur in the skin and subcutaneous soft tissue, with only a few reports of digestive tract involvement. We describe an 18-year-old male with NF2 who had bilateral vestibular schwannomas and multiple cutaneous PSs, and a 3-year history of abdominal pain. The patient ultimately underwent a distal gastrectomy for a partially obstructing submucosal antral mass, associated with an overlying ulcer. Histopathologic examination showed the mass to be a PS. The patient is alive and well, without symptoms, 12 months postoperatively. A review of the English language medical literature yielded only ten examples of PS arising in the digestive tract. Our patient is the first to be reported to have a gastric PS, and only the second patient to be reported with a digestive tract PS to have NF2, and the only patient reported to have both digestive tract and cutaneous PSs. Despite its rare occurrence with NF2, the finding of PS at any site should stimulate an examination for other manifestations of this disorder. None.

  14. What is the real incidence of vestibular schwannoma?

    DEFF Research Database (Denmark)

    Tos, Mirko; Stangerup, Sven-Eric; Cayé-Thomasen, Per

    2004-01-01

    OBJECTIVES: To present the incidence of vestibular schwannoma (VS) in Denmark, compare the incidence with that of previous periods, and discuss the real incidence of VS. DESIGN, SETTING, AND PATIENTS: Prospective registration of all diagnosed VS in Denmark, with a population of 5.1 to 5.2 million...... of approximately 13 VS/1 million inhabitants per year....

  15. Persistent L5 lumbosacral radiculopathy caused by lumbosacral trunk schwannoma.

    Science.gov (United States)

    Sharifi, Guive; Jahanbakhshi, Amin

    2017-01-01

    Schwannomais, usually, benign tumor of nerve sheath that occurs evenly along the spinal cord. Intra-pelvic schwannoma is very rare entity that may arise from lumbosacral nerve roots or from sciatic nerve. Radicular pain of the lower limb as a presenting symptom of pelvic schwannoma is extremely rare. In the current report, the patient is presented with a right sided L5 radicular pain typical of lumbar discopathy. Interestingly, a herniated lumbar disc was noted on lumbosacral magnetic resonance imaging (MRI). In pre-operative studies a large pelvic mass was detected in the right pre-sacral area with solid and cystic components consistent with schwannoma. The patient underwent a low midline laparotomy to evacuate the retroperitoneal mass. Uniquely, we found the tumor to be arisen from lumbosacral trunk not from a root or peripheral nerve. Most cases with intra-pelvic schwannoma present so late with vague abdominal and pelvic discomfort or pain, low back pain, urinary and bowel symptoms because of compressive effect of the tumor, or incidentally following gynecologic work-ups; So, these patients are mostly referred to gynecologists and urologists. A neurosurgeon should have a high degree of suspicion to diagnose such an entity among his or her patients presented with pains typical for discopathy.

  16. Skull base surgery of non vestibular schwannomas of the posterior ...

    African Journals Online (AJOL)

    Results: Twenty one patients with intracranial schwannomas arising from cranial nerves other than the vestibulocochlear were surgically treated in the Neurosurgery department, Alexandria University, in the period between 2003 and 2008. There were 14 males and 7 females and the mean age was 38 years. Seven of our ...

  17. Focal Anterior Displacement of the Thoracic Spinal Cord without Evidence of Spinal Cord Herniation or an Intradural Mass

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Jong Yoon; Lee, Joon Woo; Lee, Guen Young; Kang, Heung Sik [Department of Radiology, Seoul National University Bundang Hospital, Seongnam 463-707 (Korea, Republic of)

    2014-07-01

    We report magnetic resonance imaging (MRI) findings on focal anterior displacement of the thoracic spinal cord in asymptomatic patients without a spinal cord herniation or intradural mass. We identified 12 patients (male:female = 6:6; mean age, 51.7; range, 15-83 years) between 2007 and 2011, with focal anterior displacement of the spinal cord and without evidence of an intradural mass or spinal cord herniation. Two radiologists retrospectively reviewed the MRI findings in consensus. An asymmetric spinal cord deformity with a focal dented appearance was seen on the posterior surface of the spinal cord in all patients, and it involved a length of 1 or 2 vertebral segments in the upper thoracic spine (thoracic vertebrae 1-6). Moreover, a focal widening of the posterior subarachnoid space was also observed in all cases. None of the patients had myelopathy symptoms, and they showed no focal T2-hyperintensity in the spinal cord with the exception of one patient. In addition, cerebrospinal fluid (CSF) flow artifacts were seen in the posterior subarachnoid space of the affected spinal cord level. Computed tomography myelography revealed preserved CSF flow in the two available patients. Focal anterior spinal cord indentation can be found in the upper thoracic level of asymptomatic patients without a spinal cord herniation or intradural mass.

  18. Focal Anterior Displacement of the Thoracic Spinal Cord without Evidence of Spinal Cord Herniation or an Intradural Mass

    International Nuclear Information System (INIS)

    Lee, Jong Yoon; Lee, Joon Woo; Lee, Guen Young; Kang, Heung Sik

    2014-01-01

    We report magnetic resonance imaging (MRI) findings on focal anterior displacement of the thoracic spinal cord in asymptomatic patients without a spinal cord herniation or intradural mass. We identified 12 patients (male:female = 6:6; mean age, 51.7; range, 15-83 years) between 2007 and 2011, with focal anterior displacement of the spinal cord and without evidence of an intradural mass or spinal cord herniation. Two radiologists retrospectively reviewed the MRI findings in consensus. An asymmetric spinal cord deformity with a focal dented appearance was seen on the posterior surface of the spinal cord in all patients, and it involved a length of 1 or 2 vertebral segments in the upper thoracic spine (thoracic vertebrae 1-6). Moreover, a focal widening of the posterior subarachnoid space was also observed in all cases. None of the patients had myelopathy symptoms, and they showed no focal T2-hyperintensity in the spinal cord with the exception of one patient. In addition, cerebrospinal fluid (CSF) flow artifacts were seen in the posterior subarachnoid space of the affected spinal cord level. Computed tomography myelography revealed preserved CSF flow in the two available patients. Focal anterior spinal cord indentation can be found in the upper thoracic level of asymptomatic patients without a spinal cord herniation or intradural mass

  19. Vestibular schwannoma and fitness to fly.

    Science.gov (United States)

    Pons, Yoann; Raynal, Marc; Hunkemöller, Iris; Lepage, Pierre; Kossowski, Michel

    2010-10-01

    When a pilot is referred for vestibular schwannoma (VS), his or her fitness to fly may be questioned. The objective of this retrospective study was to describe a series of VS cases in a pilot population and to discuss their fitness to fly options. Between September 2002 and March 2010, the ENT/Head and Neck Surgery Department of the National Pilot Expertise Center conducted nearly 120,000 expert consultations for 40,000 pilots. We examined the files of 10 pilots who were referred to our 2 national experts for VS. At the time of the expert consultation, hypoacusis was present in nine cases (four with total deafness), tinnitus in one case, and vertigo in nine cases. In our series, only 2 of the 10 pilots experienced a negative impact on their fitness to fly. Decisions on fitness to fly were based on several factors: minimally disturbed audition, i.e., less than a 35-dB hearing loss with a good speech discrimination score; good balance, i.e., no reported difficulties; no spontaneous nystagmus recorded on videonystagmography (VNG); no postural deviation; and a normal head-shaking test. The delay and the VS's evolution between diagnosis and expert consultation are important because the selection of a treatment to control VS is critical in minimizing the possible associated complications. When a pilot is referred for VS, his or her fitness to fly is determined by the size of the tumor, balance, auditory status, and the follow-up results of these findings. The complications that may arise from VS treatments must also be considered.

  20. Immunostimulatory CpG-oligodeoxynucleotides cause extramedullary murine hemopoiesis.

    Science.gov (United States)

    Sparwasser, T; Hültner, L; Koch, E S; Luz, A; Lipford, G B; Wagner, H

    1999-02-15

    Bacterial DNA and the synthetic CpG-oligodeoxynucleotides (ODNs) derived thereof have attracted attention because they activate cells of the adaptive immune system (lymphocytes) and the innate immune system (APCs) in a sequence-dependent manner. Here, we addressed whether CpG-ODNs affect hemopoiesis. Challenging mice with immunostimulatory CpG-ODN sequences led to transient splenomegaly, with a maximum increase of spleen weight at day 6. The induction of splenomegaly by CpG-ODNs was sequence-specific, dose-dependent, and associated with an increase in splenic cell count, in numbers of granulocyte-macrophage CFUs (GM-CFUs), and early erythroid progenitors (burst-forming units-erythroid). The transfer of spleen cells from CpG-ODN-pretreated animals into lethally irradiated syngeneic mice yielded an increase of spleen CFUs. Furthermore, the challenge of sublethally irradiated mice with CpG-ODNs caused radioprotective effects, in that recovery of GM-CFUs and cytotoxic T cell function was enhanced. The increase in GM-CFU and CTL function correlated with an enhanced resistance to Listeria infection in irradiated mice. We conclude from these data that CpG-ODNs trigger extramedullary hemopoiesis, and that this finding could be of therapeutic relevance in myelosuppression.

  1. Thalassemia, extramedullary hematopoiesis, and spinal cord compression: A case report.

    Science.gov (United States)

    Bukhari, Syed Sarmad; Junaid, Muhammad; Rashid, Mamoon Ur

    2016-01-01

    Extramedullary hematopoiesis (EMH) refers to hematopoiesis outside of the medulla of the bone. Chronic anemia states such as thalassemia can cause hematopoietic tissue to expand in certain locations. We report a case of spinal cord compression due to recurrent spinal epidural EMH, which was treated with a combination of surgery and radiotherapy. Pakistan has one of the highest incidence and prevalence of thalassemia in the world. We describe published literature on diagnosis and management of such cases. An 18-year-old male presented with bilateral lower limb paresis. He was a known case of homozygous beta thalassemia major. He had undergone surgery for spinal cord compression due to EMH 4 months prior to presentation. Symptom resolution was followed by deterioration 5 days later. He was operated again at our hospital with complete resection of the mass. He underwent local radiotherapy to prevent recurrence. At 2 years follow-up, he showed complete resolution of symptoms. Follow-up imaging demonstrated no residual mass. The possibility of EMH should be considered in every patient with ineffective erythropoiesis as a cause of spinal cord compression. Treatment of such cases is usually done with blood transfusions, which can reduce the hematopoietic drive for EMH. Other options include surgery, hydroxyurea, radiotherapy, or a combination of these on a case to case basis.

  2. Extramedullary Hematopoiesis in Uterine Leiomyoma Associated with Numerous Intravascular Thrombi

    Directory of Open Access Journals (Sweden)

    Xiaoyan Cui

    2014-01-01

    Full Text Available We report a case of extramedullary hematopoiesis (EMH in uterine leiomyoma and associated numerous intravascular thrombi. A 29-year-old nulliparous female presented with heavy vaginal bleeding and a hematocrit of 22%. No bone marrow biopsy has been performed. She had a history of uterine leiomyomata and menorrhagia for a year. A transvaginal ultrasound confirmed the presence of a uterine leiomyoma. The patient was treated conservatively with oral contraceptive pills due to desire for fertility. However, she continued to have heavy vaginal bleeding and developed bilateral upper extremity deep vein thrombosis and multiple superficial vein thromboses after two months. An exploratory laparotomy with uterine myomectomy was performed. Gross examination of the specimen revealed a single nodular mass measuring 10.0×9.5×7.5 cm with a white-tan swirling cut surface. Microscopic examination revealed benign smooth muscle consistent with leiomyoma and numerous intravascular thrombi both with areas of EMH. Immunohistochemical stains confirmed the presence of all three benign lineages of hematopoietic cells. Occurrence of EMH in uterine leiomyoma and intravascular thrombi is very rare. It may be related to systemic hematopoietic stimulation due to severe chronic anemia and local presence of hematopoietic growth factors and/or cytokines.

  3. Gastroduodenal intussusception due to gastric schwannoma treated by Billroth II distal gastrectomy: one case report.

    Science.gov (United States)

    Yang, Jia-Hua; Zhang, Min; Zhao, Zhi-Hua; Shu, Yu; Hong, Jun; Cao, Yi-Jun

    2015-02-21

    Schwannomas are rarely observed in the gastrointestinal tract. The most common symptoms of a gastric schwannoma are abdominal pain or dyspepsia, gastrointestinal bleeding, and an abdominal mass. Many gastric schwannomas are asymptomatic and are discovered incidentally or at postmortem. The diagnosis of a schwannoma is based on immunohistochemical positivity for S-100 protein. We present a case report of a rare complication of gastric schwannoma causing gastroduodenal intussusception that was successfully managed by a Billroth II distal gastrectomy. In this rare case, the patient had intermittent, colicky abdominal pain, nausea, and vomiting for over 4 wk accompanied by a weight loss. A diagnosis of gastric intussusception was made by computed tomography. A Billroth II distal gastrectomy was then performed, and complete en bloc removal (R0 resection) was achieved. Pathology confirmed a gastric schwannoma through positive immunohistochemical staining for S-100 protein.

  4. Special problems encountering surgical management of large retroperitoneal schwannomas

    Directory of Open Access Journals (Sweden)

    Kondi-Pafitis Agathi

    2008-10-01

    Full Text Available Abstract Background Retroperitoneal schwannomas are rare, usually benign tumors that originate in the neural sheath and account for only a small percentage of retroperitoneal tumors. The aim of this clinical study is to present our experience in managing retroperitoneal schwannomas with a review of the current literature and to point out the surgical technical difficulties we faced, due to the tumor's strange behavior that eroded the vertebra in two cases without causing malignant invasion. Methods We reviewed the medical files of 69 patients treated in our department for retroperitoneal tumors from January 1991 until December 2006. Five patients had retroperitoneal schwannomas according to pathology report. Results There were two male and three female patients, with a mean age of 56 years (range 44–67 years. All patients were asymptomatic and none suffered from von Recklinghausen disease. Imaging workup included ultrasonography, computed tomography and magnetic resonance imaging. One patient, after having a non-diagnostic computed tomography fine needle aspiration (CT-FNA, underwent exploratory laparotomy and incisional biopsy that established the diagnosis of schwannoma. After complete excision of the tumors, postoperative course was uneventful in all patients. Tumors' maximum diameter was 12.7 cm (range 7–20 cm. No recurrences were detected during the follow up period (6–75 months. Conclusion Preoperative establishment of diagnosis is difficult in case of retroperitoneal schwannomas, however close relationship of retroperitoneal tumors with adjacent neural structures in imaging studies should raise a suspicion. Complete surgical resection is the treatment of choice. Histology and Immunohistochemistry confirms the diagnosis.

  5. Endoscopic resection for gastric schwannoma with long-term outcomes.

    Science.gov (United States)

    Cai, Ming-Yan; Xu, Jia-Xin; Zhou, Ping-Hong; Xu, Mei-Dong; Chen, Shi-Yao; Hou, Jun; Zhong, Yun-Shi; Zhang, Yi-Qun; Ma, Li-Li

    2016-09-01

    Gastric schwannoma is not so recognized by clinicians as its counterparts. The efficacy of endoscopic resection has not been described yet. Our aim was to assess the efficacy and safety of endoscopic resection in the management of gastric schwannoma. Retrospective data were reviewed from January 2008 to December 2013 in our center. Fourteen patients who had endoscopic resection with the final pathology result of gastric schwannoma were included in the study. Of the 14 patients, there were 12 females and two males. The median age was 59 years (range 32-83). Thirteen tumors (92.9 %) were from the muscularis propria and one located in the submucosa. Endoscopic en bloc resection was achieved in 12 patients (12/14, 85.7 %), including seven cases of endoscopic full-thickness resection (EFTR). The mean resected tumor size was 1.73 ± 1.10 cm (range 0.3-4.0 cm). In one case, endoscopic resection was suspended due to the limited experience of EFTR during the early period of the study. In another case, due to the difficult tumor location (gastric angle) and extraluminal growth pattern, the patient was referred to laparoscopic surgery. In the 12 successful endoscopic resection cases, during the median follow-up time of 4 years (range 17-77 months, one patient lost), no tumor residue, recurrence or metastasis was found. Endoscopic resection is safe and effective in treating gastric schwannoma with excellent long-term outcomes. However, it should be performed with caution because schwannoma is mainly located in the deep muscular layer, which leads to the full-thickness resection of gastric wall.

  6. Electrochemotherapy treatment of oral extramedullary plasmacytoma of the tongue: a retrospective study of three dogs

    Directory of Open Access Journals (Sweden)

    Rúbia Monteiro de Castro Cunha

    2017-11-01

    Full Text Available ABSTRACT: Extramedullary plasmacytomas (EPs are responsible for 2.5% of neoplasms in dogs. They are solitary, smooth, elevated, pink or red nodules, of 1 to 2cm in diameter. Cutaneous and oral extramedullary plasmacytomas in dogs are usually benign tumors, treated with local therapies. Prognosis is generally good. Recurrence and metastatic rates are low. Electrochemotherapy is a local treatment that combines chemotherapy and electroporation and shows objective responses of 70% to 94% with few local and systemic side effects. This scientific communication has the objective to report treatment of three canine patients with oral extramedullary plasmacytoma. Nodules were located on the tongue and patients were submitted to one or two electrochemotherapy sessions, which preserved the tongue without mutilation and cured the patients.

  7. Extramedullary Relapse of Acute Lymphoblastic Leukemia Presenting as Abnormal Uterine Bleeding: A Case Report.

    Science.gov (United States)

    Robillard, Diana T; Kutny, Matthew A; Chewning, Joseph H; Arbuckle, Janeen L

    2017-06-01

    Acute lymphoblastic leukemia (ALL) is the most common childhood malignancy. Relapse of ALL occurs in 15%-20% of patients, with 2%-6% occurring exclusively in extramedullary sites. Relapse of ALL in gynecologic organs is extremely rare. We present a case of a 12-year-old girl with a history of ALL who was referred to the pediatric gynecology clinic with abnormal uterine bleeding. She was determined to have an extramedullary uterine relapse of her ALL. Abnormal uterine bleeding in the setting of childhood malignancy is a frequent reason for consultation to pediatric and adolescent gynecology services. This bleeding is commonly attributed to thrombocytopenia due to bone marrow suppressive chemotherapeutic agents. However, as shown in this report, abnormal uterine bleeding might be a manifestation of an extramedullary relapse. Copyright © 2017 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

  8. Extramedullary Plasmacytoma of the Nasal Cavity Report of Three Cases With Review of the Literature

    Science.gov (United States)

    Ashraf, Mohamad Javad; Azarpira, Negar; Khademi, Bighan; Abedi, Elham; Hakimzadeh, Afsoon; Valibeigi, Bita

    2013-01-01

    Extramedullary plasmacytoma is a rare neoplasm characterized by monoclonal proliferation of plasma cells. Most lesions occur in the head and neck, primarily in the upper aerodigestive tract. The nasal cavity and nasal septum are the most common sites of occurrence. In this report, three patients admitted in our clinic with history of nasal obstruction and/or epistaxis. Patients were diagnosed with extramedullary plasmacytoma and mass were completely excised. This entity usually occurred in 5th-6th decade of life. One of our patients, a young man, was completely asymptomatic and following a paroxysm of coughing, a polypoid mass was expectorated. The clinical and histopathologic findings of plasmacytoma are discussed. In order to exclude systemic involvement, systematic approach using clinical, laboratory, and radiologic investigations was performed. Extramedullary plasmacytoma of the nasal cavity is rare and should be considered in the differential diagnosis of nasal cavity masses especially in young age group. PMID:24083014

  9. Laryngeal schwannoma in an 8-year-old boy with inspiratory dyspnea.

    Science.gov (United States)

    Rognone, Elisa; Rossi, Andrea; Conte, Massimo; Nozza, Paolo; Tarantino, Vincenzo; Fibbi, Antonio; Saetti, Roberto; Cutrone, Cesare; Tortori-Donati, Paolo

    2007-10-01

    Schwannomas of the larynx are rare lesions in the pediatric age group. In this article, we report on the neuroimaging features of a schwannoma arising from the left aryepiglottic fold in an 8-year-old boy with a 6-month history of inspiratory dyspnea. Neuroimaging showed a well-defined, avoid mass originating from the left aryepiglottic fold. The lesion was removed endoscopically. Complete removal of laryngeal schwannomas is curative, and adjuvant treatment is not required.

  10. A rare cause of chronic sciatic pain: Schwannoma of the sciatic nerve.

    Science.gov (United States)

    Rhanim, Abdelkarim; El Zanati, Rachid; Mahfoud, Mustapha; Berrada, Mohammed Saleh; El Yaacoubi, Moradh

    2013-06-01

    Schwannomas are common, benign tumors of the shelth of peripheral nerves. Sciatic schwannomas are rare. Their symptomatology usually mimics sciatic pain due to a herniated disc, which can delay the diagnosis. If there is no lumbar pain and lumbar MRI is normal, the sciatic nerve must be clinically and radiologically examined all along its course. We report a case of sciatic nerve schwannoma presenting with chronic sciatica which was diagnosed and monitored radiologically for several years before successful surgical resection.

  11. Intrinsic brainstem schwannoma – A rare clinical entity and a histological enigma

    Directory of Open Access Journals (Sweden)

    Anil Kumar Sharma

    2016-01-01

    Full Text Available Intraparenchymal schwannomas arising in the brainstem are very rare, and only eight cases have been reported in literature till now. We report an intraparenchymal brainstem schwannoma presenting with the classical clinical presentation of an intrinsic brainstem lesion, and discuss its clinicoradiological characteristics and histological origins. We highlight the importance of an intraoperative frozen section diagnosis in such cases. Intraoperative tissue diagnosis significantly may alter the surgical strategy, which should be aimed at near total intracapsular decompression of the schwannoma.

  12. Contribution of p75NTR to Schwannoma Growth and Therapeutic Responses

    Science.gov (United States)

    2015-05-01

    284-291. Hansen MR, Roehm PC, Chatterjee P, Green SH (2006) Constitutive neuregulin-1/ErbB signaling contributes to human vestibular schwannoma... vestibular schwannoma. Human molecular genetics 3:347-350. Jung KM, Tan S, Landman N, Petrova K, Murray S, Lewis R, Kim PK, Kim DS, Ryu SH, Chao MV, Kim...culture and in animal models of human schwannoma disease . We find that the NF2 gene product, merlin, regulates p75NTR expression levels and signaling

  13. Gastric Schwannoma Mimicking Malignant Gastrointestinal Stromal Tumor Exhibiting Increased Fluorodeoxyglucose Uptake

    OpenAIRE

    Sung Jin Oh; Byoung Jo Suh; Jong Kwon Park

    2016-01-01

    A schwannoma is a kind of neurogenic tumor that rarely occurs in the gastrointestinal tract. Gastric schwannomas make up 0.2% of all gastric neoplasms. Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors and up to 60?70% of GIST occur in the stomach. Schwannoma and GIST are similar in clinical features, so they are difficult to differentiate preoperatively. Differential diagnosis of these two submucosal tumors is important because of the malignant potential of GIST a...

  14. Plexiform Schwannoma of the Stomach in Neurofibromatosis Type 2: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Hyun Jung [Dept. of Pathology, Sanggye Paik Hospital, Inje University College of Medicine, Seoul (Korea, Republic of); Yeom, Dong Heon; Cho, Hyun Sun; Cho, Woo Ho [Dept. of Radiology, Sanggye Paik Hospital, Inje University College of Medicine, Seoul (Korea, Republic of)

    2012-02-15

    Plexiform schwannoma is a relatively rare benign subepithelial tumor arising from the peripheral nerve sheath, and associated with Neurofibromatosis type 2 (NF2). There are a few reports of plexiform schwannomas arising from the gastrointestinal tract, and to our knowledge, there is no report of it arising from the stomach in a patient with NF2. Here we present the first case of a plexiform schwannoma of the stomach in an NF2 patient a submucosal tumor on radiologic finding.

  15. Plexiform Schwannoma of the Stomach in Neurofibromatosis Type 2: A Case Report

    International Nuclear Information System (INIS)

    Kim, Hyun Jung; Yeom, Dong Heon; Cho, Hyun Sun; Cho, Woo Ho

    2012-01-01

    Plexiform schwannoma is a relatively rare benign subepithelial tumor arising from the peripheral nerve sheath, and associated with Neurofibromatosis type 2 (NF2). There are a few reports of plexiform schwannomas arising from the gastrointestinal tract, and to our knowledge, there is no report of it arising from the stomach in a patient with NF2. Here we present the first case of a plexiform schwannoma of the stomach in an NF2 patient a submucosal tumor on radiologic finding.

  16. Rare case of palatal schwannoma: Case report and review of literature

    Directory of Open Access Journals (Sweden)

    Uday Shankar Yaga

    2015-01-01

    Full Text Available Schwannomas, also known as neurilemmoma, are benign, slow-growing nerve sheath tumors arising from Schwann cells. Approximately 25-40% of schwannomas occur in the head and neck region. However, schwannomas that present in the oral cavity are relatively rare, constituting around 1% of all described cases in the head and neck region. [1],[2] Here, we report a rare case of an intraoral schwannoma, in a 28-year-old male, with painless swelling located in the posterolateral aspect of the soft palate on the right side. Definitive diagnosis was obtained after histopathology examination. Surgical excision of the tumor was done with no complications or recurrences.

  17. Gastric schwannoma misdiagnosed as GIST: A case report with immunohistochemical and molecular study.

    Science.gov (United States)

    Tatangelo, Fabiana; Cantile, Monica; Collina, Francesca; Belli, Andrea; DE Franciscis, Silvia; Bianco, Franco; Botti, Gerardo

    2016-04-01

    Schwannomas are tumors derived from Schwann cells. Generally, they are benign and their typical site of origin is the subcutaneous tissue of the distal extremities or of the head and neck region. Gastrointestinal localization of schwannomas is extremely rare, and the stomach is the prevalent site. The present study describes the case of a gastric schwannoma in a 61-year-old male who underwent subtotal gastrectomy following a clinical diagnosis of a gastrointestinal stromal tumor (GIST). A histological, immunohistochemical and molecular study was performed to exclude the misdiagnosis of GIST. The histomorphological features of the lesion and absence of c-Kit and PDGFRA mutations indicated the diagnosis of gastric schwannoma.

  18. Bilateral Pleural Effusion in a Patient with an Extensive Extramedullary Hematopoietic Mass

    Directory of Open Access Journals (Sweden)

    Yun Luo

    2013-01-01

    Full Text Available We present a 56-year-old woman with bilateral pleural effusions, widespread enlarged lymph nodes, and soft tissue masses located within the renal pelvis. The initially working diagnosis was tuberculosis and lymphoma. Further pathological examination of the lymph node biopsy confirmed a diagnosis of extramedullary hematopoiesis, and a bone marrow biopsy revealed myelofibrosis. Unlike common treatment options such as radiotherapy and/or surgery, intrathoracic cisplatin and dexamethasone for the treatment of pleural effusions secondary to extramedullary hematopoiesis demonstrated an improvement in feasibility and efficacy in the present case.

  19. Renal Bleeding Due to Extramedullary Hematopoiesis in a Patient With Chronic Myelogenous Leukemia

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    Stephanie Zettner

    2014-11-01

    Full Text Available Chronic myelogenous leukemia (CML is a myeloproliferative disorder that normally presents in middle-aged adults. Renal infiltration and extramedullary hematopoiesis in renal tissue has been rarely reported. This case report presents a patient with CML and renal insufficiency who developed gross hematuria. Efforts at controlling the hematuria led to a cascade of events propelled by the underlying disorder that ultimately led to a radical nephrectomy, multiorgan failure, and prolonged hospitalization. We suggest that management of gross hematuria in clinically stable patients with CML, suspected of having extramedullary hematopoiesis, should prioritize treatment of the myeloproliferative disorder over efforts to control bleeding.

  20. Renal Bleeding Due to Extramedullary Hematopoiesis in a Patient With Chronic Myelogenous Leukemia.

    Science.gov (United States)

    Zettner, Stephanie; Mistry, Sandeep G

    2014-11-01

    Chronic myelogenous leukemia (CML) is a myeloproliferative disorder that normally presents in middle-aged adults. Renal infiltration and extramedullary hematopoiesis in renal tissue has been rarely reported. This case report presents a patient with CML and renal insufficiency who developed gross hematuria. Efforts at controlling the hematuria led to a cascade of events propelled by the underlying disorder that ultimately led to a radical nephrectomy, multiorgan failure, and prolonged hospitalization. We suggest that management of gross hematuria in clinically stable patients with CML, suspected of having extramedullary hematopoiesis, should prioritize treatment of the myeloproliferative disorder over efforts to control bleeding.

  1. Intradural disc herniation: radiographic findings and surgical results with a literature review.

    Science.gov (United States)

    Kobayashi, Kazuyoshi; Imagama, Shiro; Matsubara, Yuji; Yoshihara, Hisatake; Hirano, Kenichi; Ito, Zenya; Ando, Kei; Ukai, Junichi; Muramoto, Akio; Shinjo, Ryuichi; Matsumoto, Tomohiro; Nakashima, Hiroaki; Ishiguro, Naoki

    2014-10-01

    To report a series of four cases of intradural disc herniation (IDH) with a review of the literature. IDH is a rare type of disc herniation. Preoperative diagnosis is difficult and IDH is only confirmed during surgery in most cases. Here, we describe four cases of IDH, including three with lumbar hernia and one with thoracic hernia. A retrospective chart review, surgical database query, and review of radiology reports are presented for each case, along with a literature review of IDH. Two of the four patients had a history of surgery at the same spinal level. Ring enhancement in gadolinium-enhanced MRI, an air image in computed tomography, and complete block in myelography were observed in the series. Surgery was performed with a transdural approach in all patients. One patient underwent transforaminal lumbar interbody fusion after postoperative recurrence. Three patients with lumbar involvement had nerve root symptoms preoperatively, but showed symptomatic improvement in the early postoperative period. In contrast, the patient with thoracic involvement had preoperative muscle weakness due to myelopathy symptoms, and had residual symptoms after surgery. IDH is a rare disease and characteristic imaging findings can be useful for diagnosis. Intraoperative findings lead to a definitive diagnosis in many cases and recognition of the pathological characteristics of IDH is important. Copyright © 2014 Elsevier B.V. All rights reserved.

  2. Comparison of Electrophysiological Outcomes of Tethered Cord Syndrome and Spinal Intradural Tumors: A Retrospective Clinical Study.

    Science.gov (United States)

    Pusat, Serhat; Kural, Cahit; Solmaz, Ilker; Temiz, Caglar; Kacar, Yunus; Tehli, Ozkan; Kutlay, Murat; Daneyemez, Mehmet; Izci, Yusuf

    2017-01-01

    Electrophysiological evaluation of the outcomes of spinal procedures is important for neurosurgeons. Somatosensorial evoked potentials (SSEPs) are used for electrophysiological evaluation of tethered cord syndrome (TCS) and spinal intradural tumors (SIT). The aim of this study was to document the electrophysiological outcomes of surgery for TCS and SIT and to compare the results based on the preoperative diagnosis. The data of 30 patients, who were operated for TCS and SIT between 2011 and 2013, were reviewed retrospectively. Surgical release of the spinal cord was performed for TCS and tumor removal was performed for SIT. Median and tibial nerve SSEPs at the left and right sides were measured at preoperative, early and late postoperative periods and compared statistically based on the diagnosis and the time of electrophysiological assessment. The diagnosis was TCS in 12 (40%) patients and SIT in 18 (60%) patients. There was a significant difference between preoperative, and early and late postoperative SSEPs values. Tibial nerve latencies were prolonged in the early postoperative, but shortened in the late postoperative period. In contrast, median nerve latencies were shortened in the early postoperative, but prolonged in the late postoperative period. There was no significant difference between the TCS and SIT groups based on the surgical intervention. Tibial nerve latency may be prolonged in the early postoperative period of TCS and SIT patients. However, electrophysiological changes were not predictive for these patients. Further studies with more patients are needed for other spinal lesions.

  3. Surgical debulking combined with photodynamic therapy to manage residual extramedullary plasmacytoma of the nasopharynx

    NARCIS (Netherlands)

    Karakullukcu, Baris; de Boer, Jan Paul; van Veen, Robert; Wegman, Jurgen; Tan, Bing

    2011-01-01

    Extramedullary plasmacytomas (EMP) are rare plasma cell neoplasms that are mostly located in the mucosal surfaces of the upper aerodigestive tract. The standard treatment is radiotherapy (RT), with surgical resection reserved as salvage procedure. In this report a patient with a EMP, located in the

  4. Peritoneal carcinomatosis-like implants of extramedullary hematopoiesis. An insolite occurrence during splenectomy for myelofibrosis

    Directory of Open Access Journals (Sweden)

    Marco Casaccia

    Full Text Available Introduction: Primary myelofibrosis (MF is a myeloproliferative neoplasm that results in debilitating constitutional symptoms, splenomegaly, and cytopenias. In patients with symptomatic splenomegaly, splenectomy remains a viable treatment option for MF patients with medically refractory symptomatic splenomegaly that precludes the use of ruxolitinib. Case presentation: We present the clinical case of a patient who was admitted to our Department to perform a splenectomy in MF as a therapeutic step prior to an allogeneic stem cell transplantation (ASCT. A laparotomic splenectomy and excision of whitish wide-spread peritoneal and omental nodulations was performed. There were no operative complications and the surgery was completed with minimal blood loss. The histopathological exam revealed an extramedullary hematopoiesis in both spleen and peritoneal nodules. Conclusion: In primary myelofibrosis it must always be kept in mind the possible presence of peritoneal implants of extramedullary hematopoiesis and ascites of reactive genesis. We report a rare case of peritoneal carcinomatosis-like implants of extramedullary hematopoiesis found at splenectomy for MF. Keywords: Myelofibrosis, Splenomegaly, Splenectomy, Extramedullary hematopoiesis

  5. Extramedullary Manifestation in Multiple Myeloma Bears High Incidence of Poor Cytogenetic Aberration and Novel Agents Resistance

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    Xiaoyan Qu

    2015-01-01

    Full Text Available Extramedullary disease (EMD in multiple myeloma (MM patients is an uncommon event and more attention was directed toward the feature of these patients. Cytogenetic aberration is an important characteristic of MM and is associated with patients’ outcome. In this study, we aimed to compare the cytogenetic abnormality of patients with and without extramedullary manifestation, and to analyze the clinical outcomes of novel agents in EMD patients. We retrospectively investigated data from 41 MM patients. Our analyses showed del(17p13 in 31% of EMD versus 13% of medullary disease (P=0.03 and amp(1q21 in 55% versus 32% (P=0.019. No differences were shown in del(13q14 and t(4;14. 24/27 patients with EMD at diagnosis responded to the novel agents-containing regimens. However, when relapsed, 70% of patients did not benefit from the sequential use of novel agents as salvage therapy. In 14 patients who developed EMD at relapse phase, only 2 patients responded to novel agents therapy. Median overall survival of patients with extramedullary manifestations was 30 months, in comparison to 104 months for patients without EMD (P=0.002. Patients with extramedullary manifestation bore high incidence of poor cytogenetic aberration and novel agents resistance.

  6. Anaesthetic management of a case of schwannoma with intraoral extension

    Directory of Open Access Journals (Sweden)

    Mamta Bhardwaj

    2015-04-01

    Full Text Available Schwannoma is a benign nerve sheath tumour. This benign lesion frequently occurs in the soft tissues of head and neck region and has various complicated growth patterns. These patients can present a challenge to the anaesthesiologist due to intraoral extension, leading to difficult mask ventilation and intubation. We report a 16 year old male with mandibular nerve schwannoma with intraoral extension. Intraoral examination revealed a diffuse swelling in the left side of soft palate with deviation of uvula to right side. He was advised gargles with 4 ml of 2% xylocaine viscous and 2–3 puffs of 10% xylocaine spray done in oral cavity and oropharynx. Check laryngoscopy revealed Cormack and Lehane grade 1 view. Patient was intubated using standard induction technique and successfully managed

  7. Painful percutaneous transthoracic needle biopsy of Schwannoma: a case report

    International Nuclear Information System (INIS)

    Kim, Sung Hoon; Chun, Kyung Ah; Kim, Young Joo; Park, Seog Hee; Shin, Kyung Sub; Lee, Eun Jung

    1995-01-01

    Percutaneous aspiration needle biopsy of the intrathoracic disease is a safe, easy, and accurate diagnostic method. It usually causes mild pain or discomfort during the procedure. We had a patient who complained of severe sharp pain, well localized at the biopsy site of the target mass during CT-guided transthoracic aspiration biopsy. It was pathologically confirmed as an intrathoracic schwannoma after special staining. To our knowledge, there has been no published report of such a painful percutaneous needle biopsy in a patient with schwannoma in Korea. Two cases were reported in other radiologic journals. The severe sharp pain developed during the transthoracic aspiration needle biopsy is a reliable sign of neurogenic tumor, therefore the participating radiologist should recommend specific immumochemical stain for neurogenic tumor to pathologist

  8. Calcification of vestibular schwannoma: a case report and literature review

    Directory of Open Access Journals (Sweden)

    Zhang Yang

    2012-10-01

    Full Text Available Abstract Calcification rarely occurs in vestibular schwannoma (VS, and only seven cases of calcified VS have been reported in the literature. Here, we report a 48-year-old man with VS, who had a history of progressive left-sided hearing loss for 3 years. Neurological examination revealed that he had left-sided hearing loss and left cerebellar ataxia. Magnetic resonance imaging and computerized tomography angiography showed a mass with calcification in the left cerebellopontine angle (CPA. The tumor was successfully removed via suboccipital craniotomy, and postoperative histopathology showed that the tumor was a schwannoma. We reviewed seven cases of calcified VS that were previously reported in the literature, and we analyzed and summarized the characteristics of these tumors, including the calcification, texture, and blood supply. We conclude that calcification in VS is associated with its texture and blood supply, and these characteristics affect the surgical removal of the tumor.

  9. Gastric schwannoma as a rare differential diagnosis of pleural effusion.

    Science.gov (United States)

    Janowitz, P; Meier, F; Reisig, J

    2002-11-01

    We report a case of solitary gastric schwannoma that initially manifested with recurrent left pleural effusion caused by an inflammatory reaction. A 75-year-old female was primarily admitted with progressive dyspnoea and left sided effusion. History as well as clinical examination, gastroscopy, computed tomography (CT) and transabdominal ultrasound of the abdomen suggested the diagnosis of a benign tumour of the stomach. The tumour was resected and a fundectomy with a security distance of 3-5 cm performed. Histological assessment revealed a large intramural schwannoma of the gastric wall, arising from the submucosal layer. There was no evidence of malignancy. During a three year follow-up the patient has not shown any evidence of relapse or pleural effusion. This is a very rare manifestation of this benign tumour, representing a rare differential diagnosis in a case of left sided pleural effusion.

  10. Intramedullary versus extramedullary fixation in the management of subtrochanteric femur fractures: a meta-analysis

    Directory of Open Access Journals (Sweden)

    Liu PC

    2015-04-01

    Full Text Available Pengcheng Liu,1,2,* Xing Wu,1,* Hui Shi,1,2 Run Liu,1 Hexi Shu,1 JinPeng Gong,1 Yong Yang,1 Qi Sun,1 Jiezhou Wu,1,2 Xiaoyang Nie,1 Ming Cai1 1Department of Orthopedics, Shanghai Tenth People’s Hospital, Tongji University, School of Medicine, Shanghai, 2First Clinical Medical College, Nanjing Medical University, Nanjing, People’s Republic of China *These authors contributed equally to this work Background: Intramedullary and extramedullary fixation methods are used in the management of subtrochanteric femur fractures. However, whether intramedullary or extramedullary fixation is the primary treatment for subtrochanteric femur fractures in adults remains debatable.Level of evidence: Meta-analyses of prospective studies, level I.Materials and methods: The Cochrane library, Embase, Google Scholar, and PubMed databases were searched separately for all relevant studies published before January 1, 2015. No language restriction was applied. Prospective randomized controlled trials that compared intramedullary or extramedullary internal fixation to repair subtrochanteric femur fractures in adults were included. We determined intraoperative data, postoperative complications, fracture fixation complications, wound infection, hospital stay days, and final outcome measures to assess the relative effects of different internal fixation methods for the treatment of subtrochanteric femur fractures in adults.Results: Six studies were included in our meta-analysis. The relative risks (RRs of revision rate was 83% lower (RR, 0.17, 95% confidence interval [CI], 0.05 to 0.60; P=0.006, fixation failure rate was 64% lower (RR, 0.36, 95% CI, 0.12 to 1.08; P=0.07, non-union rate was 77% lower (RR, 0.23, 95% CI, 0.07 to 0.81; P=0.02 in the intramedullary group compared with the extramedullary group. No significant differences were found between the intramedullary group and extramedullary group for intraoperative data, postoperative complications, wound infection

  11. Management of Large Tongue Schwannoma – A Short Report

    Directory of Open Access Journals (Sweden)

    Jayanta Medhi

    2016-03-01

    Full Text Available Schwannoma is a benign nerve sheath tumor composed of schwann cells. Oral cavity is a rare site for schwannomas, tongue being the most common location. Here we are presenting a case of a young adult who presented with a huge swelling in the tongue which was removed by mandibulotomy approach and pre-operative tracheostomy. A 22-year-old male patient presented to the outpatient department with a history of swelling in the tongue for the last 4 years with progressive difficulty in swallowing food and change of voice over the last few months. Upon examination a large swelling was observed on the posterior part of the tongue compromising the oropharyngeal inlet. The approximate size of the swelling was 5cmx4cm. After proper clinical evaluation the patient was advised to obtain a magnetic resonance imaging (MRI study of the oral cavity, which showed it to be a nerve sheath tumor (Schwannoma originating from the hypoglossal nerve branch. The patient was admitted for surgery. As difficult intubation was anticipated, pre-operative tracheostomy was performed. The tongue mass was approached by right paramedian mandibulotomy using a transcervical lip split incision. Post operative histopathological examination of the removed specimen showed hypercellular ‘Antony A’ area with plump spindle cells and hypocellular ‘Antony B’ area in a Hematoxylin & eosin stain (200x. This confirmed the diagnosis for a schwannoma. As each and every case is unique in its presentation, so is the management. The idea of presenting the above case is to emphasise the role of selection for the proper approach and foresee the preventable complications while working around the airway.

  12. Schwannoma-like pleomorphic adenoma of the parotid

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    Birgit Weynand

    2011-10-01

    Full Text Available Pleomorphic adenoma is the most common benign salivary gland tumour. It can occur in any salivary gland, but is most frequently found in the parotid gland. Chondroid metaplasia is a frequent finding in pleomorphic adenoma. Other forms of metaplasia have been described, but are encountered less frequently. We report a rare case of unusual pleomorphic adenoma of the parotid gland with schwannoma- like feature.

  13. Leptomeningeal Carcinomatosis of Gastric Cancer Misdiagnosed as Vestibular Schwannoma

    OpenAIRE

    Kim, Shin-Jae; Kwon, Jeong-Taik; Mun, Seog-Kyun; Hong, Young-Ho

    2014-01-01

    Gastric cancer is one of the most common causes of cancer-related death in Asian countries, including Korea. We experienced a case of leptomeningeal carcinomatosis (LC) from gastric cancer that was originally misdiagnosed as vestibular schwannoma based on the similar radiological characteristics. To our knowledge, LC from gastric cancer is very rare. In conclusion, our experience with this case suggests that clinicians should consider the possibility of delayed leptomeningeal metastasis when ...

  14. Gastric schwannoma presenting as a casual ultrasonographic findings.

    Science.gov (United States)

    Álvarez Higueras, Francisco Javier; Pereñíguez López, Ana; Estrella Díez, Esther; Muñoz Tornero, María; Egea Valenzuela, Juan; Bas Bernal, Águeda; Garre Sánchez, Carmen; Vargas Acosta, Ángel; Sánchez Velasco, Eduardo; Carballo Álvarez, Luis Fernando

    2016-12-01

    We present the case of a patient under study due to ascites in which a mass located on the gastric wall was observed during ultrasonography. Further studies (upper endoscopy and computed tomography) confirmed this finding. After an ultrasound-guided percutaneous biopsy, diagnosis of gastric schwannoma was made as intense S-100 expression was found. Surgery was rejected due to the bad clinical situation of the patient and because the mass was an asymptomatic benign tumor.

  15. Leptomeningeal carcinomatosis of gastric cancer misdiagnosed as vestibular schwannoma.

    Science.gov (United States)

    Kim, Shin-Jae; Kwon, Jeong-Taik; Mun, Seog-Kyun; Hong, Young-Ho

    2014-07-01

    Gastric cancer is one of the most common causes of cancer-related death in Asian countries, including Korea. We experienced a case of leptomeningeal carcinomatosis (LC) from gastric cancer that was originally misdiagnosed as vestibular schwannoma based on the similar radiological characteristics. To our knowledge, LC from gastric cancer is very rare. In conclusion, our experience with this case suggests that clinicians should consider the possibility of delayed leptomeningeal metastasis when treating patients with gastric cancer.

  16. 99m-Technetium Sestamibi Uptake in a Gastric Schwannoma.

    Science.gov (United States)

    Shawgi, Mohamed; Ali, Tamir; Scott, Matthew; Petrides, George

    2018-01-01

    We report the case of a 74-year-old woman with primary hyperparathyroidism who underwent 99m-technetium-sestamibi single photon emission computed tomography-computed tomography for preoperative localization of parathyroid adenoma. Unexpected focal sestamibi uptake was observed at a 5 cm submucosal tumor arising from the greater curve of the stomach. The patient underwent partial gastrectomy and the histological and immunohistochemical findings were consistent with the diagnosis of gastric schwannoma.

  17. Association between vestibular schwannomas and mobile phone use

    OpenAIRE

    Moon, In Seok; Kim, Bo Gyung; Kim, Jinna; Lee, Jong Dae; Lee, Won-Sang

    2013-01-01

    Vestibular schwannomas (VSs) grow in the region where the energy from mobile phone use is absorbed. We examined the associations of VSs with mobile phone use. This study included 119 patients who had undergone surgical tumor removal. We used two approaches in this investigation. First, a case–control study for the association of mobile phone use and incidence of VSs was conducted. Both cases and controls were investigated with questions based on INTERPHONE guidelines. Amount of mobile phone u...

  18. Intra-oral schwannoma: Case report and literature review

    Directory of Open Access Journals (Sweden)

    Martins Manoela

    2009-01-01

    Full Text Available Schwannoma is a relatively uncommon, slow-growing benign tumor that is derived apparently from the Schwann cells. The tongue is the most common site, followed by the palate, floor of mouth, buccal mucosa, lips, and jaws. It can present itself at any age. Usually, this lesion is not taken into account during clinical practice and the differential diagnosis includes numerous benign neoformations based on epithelial and connective tissues. Immunohistochemical features can be useful in determining the neural differentiation. Anti-S100 protein is probably the most used antibody to identify this lesion. The schwannoma is usually a solitary lesion, and can be multiple when associated with neurofibromatosis. In the current study, authors report a case of an intraoral schwannoma situated at the vestibule with 20 years of evolution treated by complete surgical excision. The diagnosis was established based on clinical, histopathological, and immunohistochemical aspects. The patient is under clinical control, with no signs of recurrence even after four years.

  19. [A Case of Primary Schwannoma of the Urinary Bladder].

    Science.gov (United States)

    Matsumoto, Yoshitaka; Waku, Natsui; Kawai, Koji; Ikeda, Atsushi; Kimura, Tomokazu; Ishitsuka, Ryutaro; Kojima, Takahiro; Suetomi, Takahiro; Joraku, Akira; Miyazaki, Jun; Sakashita, Mai; Nishiyama, Hiroyuki

    2017-08-01

    A 68-year-old woman presented with a bladder tumor. She was asymptomatic, and the tumor was incidentally detected with radiological imaging performed during treatment of cervical cancer. Magnetic resonance imaging and computed tomography revealed a solitary submucosal tumor located in the anterior wall of the urinary bladder, with homogeneous contrast enhancement. Cystoscopy showed a submucosal tumor covered by normal mucosa. A paraganglioma was considered in the differential diagnosis, but symptoms suggesting hypercatecholaminemia were not apparent. Moreover, she did not have a family history or symptoms associated with neurofibromatosis-1 (NF-1). She underwent partial cystectomy with a preliminary diagnosis of submucosal bladder tumor. Histopathological diagnosis confirmed a schwannoma arising from the bladder wall. She was followed up without intravesical recurrence or metastases for 6 months. In the literature, only 12 cases of bladder schwannoma have been reported. There was no reported family history or symptoms associated with NF-1 in any of the cases. Although the number of cases is limited, literature review showed a favorable prognosis for bladder schwannoma with local tumor resection in patients without NF-1.

  20. Mini-invasive surgery of infratemporal fossa schwannomas.

    Science.gov (United States)

    Haidar, H; Deveze, A; Lavieille, J P

    2015-02-01

    Infratemporal fossa schwannomas are benign, encapsulated tumours of the trigeminal nerve limited to the infratemporal fossa. Because of the complications and significant morbidity associated with traditional surgical approaches to the infratemporal fossa, which include facial nerve dysfunction, hearing loss, dental malocclusion and cosmetic problems, less invasive alternatives have been sought. This paper reports two cases of infratemporal fossa schwannomas treated in 2012 using mini-invasive approaches. The literature regarding different infratemporal fossa approaches was reviewed. The first schwannoma was 30 mm in size and was removed completely by a preauricular subtemporal approach. The second one was 25 mm in size and was removed completely using a purely transnasal endoscopic approach. In both cases, there were no intra-operative or post-operative complications. These two approaches allow non-invasive and wide exposure of the infratemporal fossa as compared to classical approaches. Surgical approach should be selected according to the tumour's anatomical location with respect to the maxillary sinus posterior wall. The preauricular subtemporal approach is recommended for tumours localised posterolaterally with respect to the maxillary sinus posterior wall. Medial and anterior tumours near the maxillary sinus posterior wall can be best removed using a transnasal endoscopic approach.

  1. Radiosurgical treatment of sporadic vestibular schwannomas: A prospective cohort study

    International Nuclear Information System (INIS)

    Martel V, Freddy; Iniguez S, Rodrigo; Venencia M, Daniel; Tagle M, Patricio; Besa D, Pelayo; Lorenzoni S, Jose

    2008-01-01

    Objective: To analyze the preliminary experience of radiosurgery for Vestibular Schwannomas at the Pontificia Universidad Catolica de Chile. Material and methods: The first 17 patients with sporadic Vestibular Schwannomas treated by radiosurgery at our institution are reported. The marginal dose used was 12 to 12.5 Gy. prescribed at the 70 or 80 isodose fine. Patients were controlled at 6, 12 and 24 months with magnetic resonance, audiometric study and clinical examination. Results: In all of the 17 patients treated a decrease tumor enhancement on MR was demonstrated. In 16 patients (94%) a pattern of central tumor necrosis was observed during the firs year Actuarial useful hearing was maintained in 62.5% at 2 year after treatment. Facial nerve function was maintained in all of the 15 patients with normal function at treatment (100%). Trigeminal function was maintained in ah of the 14 patients (100%) with previous normal trigeminal function. The mean time to return to work or normal activities was 11.5 days after treatment. Conclusions: These preliminary results are comparable with results published in the literature and reinforce the demonstrate role of radiosurgery in the management of vestibular schwannomas

  2. An evidence-based case of acoustic/vestibular schwannoma

    Directory of Open Access Journals (Sweden)

    Girish Gupta

    2015-01-01

    Full Text Available A vestibular schwannoma, often called an acoustic neuroma/schwannoma, is a benign primary intracranial tumor of the myelin-forming cells of the vestibulo-cochlear nerve (8 th cranial nerve. This tumor arises from the Schwann cells responsible for the myelin sheath that helps keep peripheral nerves insulated. [1] Approximately, 3000 cases are diagnosed each year in the United States with a prevalence of about 1 in 100,000 worldwide. It comprises 5-10% of all intracranial neoplasms in adults. Incidence peaks in the fifth and sixth decades and both sexes are affected equally. Studies in Denmark published in 2004 show the incidence of 17.4/million. Most acoustic neuromas are diagnosed in patients between the ages of 30 and 60, and men and women appear to be affected equally. [2] The case illustrated here is a rare one of acoustic/vestibular schwannoma a surgical conditions, treated with Lycopodium, which produced improvement on both subjective and objective parameters.

  3. Schwannoma of the Lower Eyelid Resembling a Recurrent Chalazion : A Case Report

    OpenAIRE

    Fukuyama, Junichiro; Hayasaka, Seiji; Setogawa, Tomoichi

    1990-01-01

    A 55-year-old man complained of foreign body sensation and a solid mass in the lower eyelid of the right eye. Clinically, the lesion resembled a chalazion, and it was excised. Histopathologic examination of the excised specimen revealed a schwannoma (neurilemmoma). We believe that this is a rare case of a schwannoma of the lower eyelid simulating a chalazion.

  4. A rare case of benign isolated schwannoma in the inferior orbit

    Directory of Open Access Journals (Sweden)

    Garg Rajiv

    2008-01-01

    Full Text Available A rare case of unilateral orbital schwannoma arising from the infraorbital nerve is presented. An excision biopsy with complete removal of the mass in the inferior orbit was performed. A definitive diagnosis was made on histopathological examination. The clinical and histological features of schwannoma are discussed. A need for early removal of such tumors is recommended to prevent complications.

  5. Gastric Schwannoma: Case report from Tanzania and brief review of literature

    OpenAIRE

    Manji, Mohamed; Ismail, Ame; Komba, Ewaldo

    2015-01-01

    Key Clinical Message Upper gastrointestinal bleeding causes significant morbidity and mortality worldwide. We report a rare case of hematemesis secondary to a gastric schwannoma in a Tanzanian female. Gastric schwannomas should be considered in the differential diagnosis of gastric masses and distinguished from other etiologies, given their excellent postresection prognosis.

  6. Gastric Schwannoma: Case report from Tanzania and brief review of literature.

    Science.gov (United States)

    Manji, Mohamed; Ismail, Ame; Komba, Ewaldo

    2015-07-01

    Upper gastrointestinal bleeding causes significant morbidity and mortality worldwide. We report a rare case of hematemesis secondary to a gastric schwannoma in a Tanzanian female. Gastric schwannomas should be considered in the differential diagnosis of gastric masses and distinguished from other etiologies, given their excellent postresection prognosis.

  7. Gastric GIST or gastric schwannoma-A diagnostic dilemma in a young female.

    Science.gov (United States)

    Mohanty, Sudhir Kumar; Jena, Kumarmani; Mahapatra, Tanmaya; Dash, Jyoti Ranjan; Meher, Dibyasingh; John, Ajax; Nayak, Manjushree; Bano, Shafqat

    2016-01-01

    Gastrointestinal stromal tumor (GIST) is the commonest mesenchymal tumor of GI tract and 60-70% of it seen in the stomach, whereas Gastric schwannoma is a benign, slow growing and one of the rare neoplasms of stomach. Age distribution, clinical, radiological features and gross appearance of both tumors are similar. We report a rare case of gastric schwannoma in a 20-year-old girl, who underwent subtotal gastrectomy with the suspicion of a GIST preoperatively but later confirmed to be gastric schwannoma postoperatively after immunohistochemical study. Accordingly, the differential diagnosis for gastric submucosal mass should be gastric schwannoma. Furthermore, Gastric schwannoma is a benign neoplasm with excellent prognosis after surgical resection, whereas 10-30% of GIST has malignant behavior. Therefore, it is important to distinguish between gastric schwannoma and GIST so as to make an accurate diagnosis for optimally guide treatment options. Due to the paucity of gastric schwannoma, the index of suspicion for this diagnosis is low. So it is important to include gastric schwannoma in the differential diagnosis when preoperative imaging studies reveal submucosal exophytic gastric mass and after resection of the tumor with a negative margin, it should be sent for immunohistochemical study for confirmation of diagnosis. Copyright © 2016 The Author(s). Published by Elsevier Ltd.. All rights reserved.

  8. Intradural communication between dorsal rootlets of spinal nerves: their clinical significance.

    Science.gov (United States)

    Solmaz, Bilgehan; Tatarlı, Necati; Ceylan, Davut; Keleş, Evren; Çavdar, Safiye

    2015-06-01

    Anatomical and surgical textbooks give almost no attention to the intradural communications between dorsal rootlets of adjacent spinal nerves. These communications can be of significance in various neurosurgical procedures and clinical conditions of the region. The spinal cord of six formaldehyde-fixed cadavers was dissected from C1-S5. The dorsal rootlets of the spinal nerves were exposed via a posterior approach and communications between adjacent spinal nerves were documented. The frequency of communication between adjacent dorsal rootlets of the spinal nerves showed variations among spinal levels. Thirty-eight dorsal rootlet communications were observed in six cadavers (12 sides) and 20 (52.6%) were at cervical levels, 14 (36.8%) at thoracic levels, and four (10.5%) at lumbar levels. The majority of communications were observed on the left side (65.8%). Communications were most frequently observed at cervical (C4-C5, C5-C6) and upper thoracic (T1-T2) levels and seen least frequently at lower thoracic and lumbar levels. No communications were observed at sacral levels. Five types of communication were observed: I. oblique ascending, II. oblique descending III. short Y, IV. long Y and V shaped. None of the communication extended beyond one segment at any spinal level. The occurrence of such dorsal rootlet communications ranged from 3 to 7 for each cadaver and the mean was 4.8 ± 1.3. Histological sections from various levels of the dorsal rootlet communications showed that all consisted of myelinated fibers of varying diameters. Such communications may lead to misinterpretation of the pathology on the basis of clinical signs and symptoms and also should be considered in rhizotomy.

  9. Endovascular reconstruction of unruptured intradural vertebral artery dissecting aneurysms with the Pipeline embolization device.

    Science.gov (United States)

    Kühn, Anna Luisa; Kan, Peter; Massari, Francesco; Lozano, J Diego; Hou, Samuel Y; Howk, Mary; Gounis, Matthew J; Wakhloo, Ajay K; Puri, Ajit S

    2016-10-01

    Dissecting aneurysms of the vertebral artery (VA) are difficult to treat using current surgical and endovascular techniques. To analyze retrospectively the efficacy and safety of flow diverters in the treatment of dissecting aneurysms of the vertebral artery. We identified six patients with six unruptured VA dissecting aneurysms either arising from the V4 or V3-V4 junction that were treated with the Pipeline embolization device (PED) at our institution between July 2012 and February 2015. Among other parameters, technical feasibility of the procedure, procedure-related complications, angiographic results, and clinical outcome were evaluated. PED placement was achieved in all cases and immediate angiography follow-up demonstrated intra-aneurysmal contrast stasis with parent artery preservation. A temporary episode of dysarthria was noted in one patient. Major procedure-related complications were not observed. The 6-month follow-up (n=6) demonstrated complete/near-complete aneurysm obliteration in five patients and partial obliteration in one. At the 1-year follow-up (n=5) stable complete aneurysm occlusion was seen in two patients. Two cases showed progression from near complete occlusion and partial occlusion at 6 months to complete occlusion and near complete occlusion. One cases showed unchanged near complete occlusion. No aneurysmal bleeding, in-stent stenosis or thromboembolic complication was seen. National Institutes of Health Stroke Scale and modified Rankin scale scores remained unchanged from admission to discharge. Our preliminary experience with the use of PED for the treatment of intradural VA dissecting aneurysms shows promising short-term results, making this technique a feasible and safe treatment option in patients suitable for this approach. However, long-term and larger cohort studies are needed to validate these results. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to

  10. Condrosarcoma vertebral: A propósito de un caso recidivado con invasión intradural

    OpenAIRE

    Navarrete Faubel, Francisco Enrique; Escribá Roca, Ismael

    2000-01-01

    Presentamos un caso de condosarcoma primario vertebral en un paciente de 17 años, que, tras el tratamiento quirúrgico, radio y quimioterapia adyuvante, sufrió una recidiva con invasión intradural a los 5 años de evolución. El paciente fue reintervenido realizándose una descompresión medular y liberación radicular, pero debido a la extensión tumoral, no hubo recuperación neurológica, quedando un síndrome medular incompleto con nivel T10. La poca frecuencia de este tumor en la columna, la dific...

  11. Ancient schwannoma at the olfactory groove mimicking meningioma: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Heo, Young Jin; Jeong, Hae Woong [Dept. of Radiology, Busan Paik Hospital, Inje University College of Medicine, Busan (Korea, Republic of)

    2015-12-15

    Schwannomas are benign slow-growing nerve sheath tumors, which can develop in any peripheral or central nerve that contains Schwann cells. Schwannomas located near the olfactory groove are extremely rare and radiological diagnosis can be difficult. Moreover, ancient schwannoma is an uncommon variant, and radiologic findings are rarely reported. Herein, we reported a surgically confirmed case of ancient schwannoma at the olfactory groove in a 44-year-old woman presenting with headache and visual disturbance. Brain magnetic resonance imaging (MRI) showed a solid and cystic extra-axial mass located in the subfrontal area mimicking an olfactory groove meningioma. Histopathologic diagnosis of ancient schwannoma was confirmed by immunohistochemical staining for S100, CD56, vimentin, and other markers. Furthermore, we described the clinical manifestations, MRI characteristics, and histopathologic findings of the case, and presented a review of related literature.

  12. Gastric Schwannoma Mimicking Malignant Gastrointestinal Stromal Tumor Exhibiting Increased Fluorodeoxyglucose Uptake.

    Science.gov (United States)

    Oh, Sung Jin; Suh, Byoung Jo; Park, Jong Kwon

    2016-01-01

    A schwannoma is a kind of neurogenic tumor that rarely occurs in the gastrointestinal tract. Gastric schwannomas make up 0.2% of all gastric neoplasms. Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors and up to 60-70% of GIST occur in the stomach. Schwannoma and GIST are similar in clinical features, so they are difficult to differentiate preoperatively. Differential diagnosis of these two submucosal tumors is important because of the malignant potential of GIST and the relatively benign course of gastric schwannomas. We report a 49-year-old woman who was diagnosed after operation with a gastric schwannoma, which was suspected a malignant GIST by fluorine-18-fluorodeoxyglucose positron emission computed tomography imaging.

  13. Gastric Schwannoma Mimicking Malignant Gastrointestinal Stromal Tumor Exhibiting Increased Fluorodeoxyglucose Uptake

    Directory of Open Access Journals (Sweden)

    Sung Jin Oh

    2016-04-01

    Full Text Available A schwannoma is a kind of neurogenic tumor that rarely occurs in the gastrointestinal tract. Gastric schwannomas make up 0.2% of all gastric neoplasms. Gastrointestinal stromal tumors (GIST are the most common mesenchymal tumors and up to 60-70% of GIST occur in the stomach. Schwannoma and GIST are similar in clinical features, so they are difficult to differentiate preoperatively. Differential diagnosis of these two submucosal tumors is important because of the malignant potential of GIST and the relatively benign course of gastric schwannomas. We report a 49-year-old woman who was diagnosed after operation with a gastric schwannoma, which was suspected a malignant GIST by fluorine-18-fluorodeoxyglucose positron emission computed tomography imaging.

  14. Gastric schwannoma with adjacent external progression harbored aberrant NF2 gene.

    Science.gov (United States)

    Ogasawara, Naotaka; Sasaki, Makoto; Ishiguro, Hideyuki; Itoh, Yukimi; Nojiri, Syunsuke; Kubota, Eiji; Wada, Tsuneya; Kataoka, Hiromi; Kuwabara, Yoshiyuki; Joh, Takashi

    2009-07-01

    Gastric schwannomas are rare benign mesenchymal tumors. We describe a schwannoma of gastric origin with adjacent external progression. Sections showed a spindle cell tumor arranged in interlaced bundles and fascicles that was S-100 and CD34 positive but c-KIT protein negative. Histology and immunohistochemistry revealed the typical appearance of a gastric schwannoma. Genetic evaluation revealed that the tumor harbored a point mutation in exon 6 of the tumor suppressor neurofibromatosis 2 (NF2) gene, which resulted in an amino acid substitution of NF2 protein, and no mutation in exon 4b of the NF1 gene. In conclusion, we identified a rare mutation of the NF2 gene in gastric schwannoma. A diagnosis can only be definitive when based on histological and immunohistochemical findings. Digestive tract schwannomas are rare mesenchymal tumors that are differentiated from gastrointestinal stromal tumors by the absence of KIT protein. Follow up suggested that complete resection is an effective long-term treatment strategy.

  15. Acoustic schwannoma with intracochlear extension and primary intracochlear schwannoma: removal through translabyrinthine approach with facial bridge cochleostomy and transcanal approach.

    Science.gov (United States)

    Mazzoni, A; Zanoletti, E; Faccioli, C; Martini, A

    2017-05-01

    Intracochlear schwannomas can occur either as an extension of a larger tumor from the internal auditory canal, or as a solitary labyrinthine tumor. They are currently removed via a translabyrinthine approach extended to the basal turn, adding a transotic approach for tumors lying beyond the basal turn. Facial bridge cochleostomy may be associated with the translabyrinthine approach to enable the whole cochlea to be approached without sacrificing the external auditory canal and tympanum. We describe seven cases, five of which underwent cochlear schwannoma resection with facial bridge cochleostomy, one case with the same procedure for a suspect tumor and one, previously subjected to radical tympanomastoidectomy, who underwent schwannoma resection via a transotic approach. Facial bridge cochleostomy involved removing the bone between the labyrinthine and tympanic portions of the fallopian canal, and exposing the cochlea from the basal to the apical turn. Patients' recovery was uneventful, and long-term magnetic resonance imaging showed no residual tumor. Facial bridge cochleostomy can be a flexible extension of the translabyrinthine approach for tumors extending from the internal auditory canal to the cochlea. The transcanal approach is suitable for the primary exclusive intralabyrinthine tumor. The indications for the different approaches are discussed.

  16. Ischaemic colitis and lung infiltrates caused by extramedullary haematopoiesis in a patient with an acute erythroid leukaemia following polycythaemia vera

    NARCIS (Netherlands)

    Brada, SJL; de Wolf, JTM; Poppema, S; Vellenga, E

    A patient with 'spent' polycythaemia vera showed extensive extramedullary haematopoiesis (EMH) in non-haematopoietic tissue clinically resulting in an ischaemic colitis and respiratory symptoms due to lung infiltrates. On laboratory investigation, the EMH also included immature erythroblasts due to

  17. Association of Genetic Predisposition With Solitary Schwannoma or Meningioma in Children and Young Adults.

    Science.gov (United States)

    Pathmanaban, Omar N; Sadler, Katherine V; Kamaly-Asl, Ian D; King, Andrew T; Rutherford, Scott A; Hammerbeck-Ward, Charlotte; McCabe, Martin G; Kilday, John-Paul; Beetz, Christian; Poplawski, Nicola K; Evans, D Gareth; Smith, Miriam J

    2017-09-01

    Meningiomas and schwannomas are usually sporadic, isolated tumors occurring in adults older than 60 years and are rare in children and young adults. Multiple schwannomas and/or meningiomas are more frequently associated with a tumor suppressor syndrome and, accordingly, trigger genetic testing, whereas solitary tumors do not. Nevertheless, apparently sporadic tumors in young patients may herald a genetic syndrome. To determine the frequency of the known heritable meningioma- or schwannoma-predisposing mutations in children and young adults presenting with a solitary meningioma or schwannoma. Using the database of the Manchester Centre for Genomic Medicine, this cohort study analyzed lymphocyte DNA from young individuals prospectively referred to the clinic for genetic testing between January 1, 1990, and December 31, 2016, on presentation with a single meningioma (n = 42) or schwannoma (n = 135) before age 25 years. Sequencing data were also examined from an additional 39 patients with neurofibromatosis type 2 who were retrospectively identified as having a solitary tumor before age 25 years. Patients with schwannoma were screened for NF2, SMARCB1, and LZTR1 gene mutations, while patients with meningioma were screened for NF2, SMARCB1, SMARCE1, and SUFU. The type of underlying genetic mutation, or lack of a predisposing mutation, was associated with the presenting tumor type and subsequent development of additional tumors or other features of known schwannoma- and meningioma-predisposing syndromes. In 2 cohorts of patients who presented with an isolated meningioma (n = 42; median [range] age, 11 [1-24] years; 22 female) or schwannoma (n = 135; median [range] age, 18 [0.2-24] years; 60 female) before age 25 years, 16 of 42 patients (38%) had a predisposing mutation to meningioma and 27 of 135 patients (20%) to schwannoma, respectively. In the solitary meningioma cohort, 34 of 63 patients (54%) had a constitutional mutation in a known meningioma

  18. Endoscopic ultrasound features of gastric schwannomas with radiological correlation: a case series report.

    Science.gov (United States)

    Zhong, Dan-Dan; Wang, Cai-Hua; Xu, Jing-Hong; Chen, Miao-Yan; Cai, Jian-Ting

    2012-12-28

    Gastric schwannomas are rare mesenchymal tumors of the gastrointestinal tract. They are usually misdiagnosed as other submucosal tumors preoperatively. Experience of the imaging features of gastric schwannomas is extremely limited. In this report, we summarize the features of a series of endoscopic ultrasound (EUS) images of gastric schwannomas in an effort to improve the diagnosis and differential diagnosis rate. We retrospectively reviewed the endosonographic features of four patients with gastric schwannomas and their computed tomography imaging results. Gastric schwannomas had heterogeneous hypoechogenicity or isoechogenicity, and a well-demarcated margin. The tumors originated from the fourth layer. Cystic changes and calcification were uncommon. Marginal hypoechoic haloes were observed in two patients. The results described here were different from those of previous studies. In the EUS evaluation, the internal echogenicity of gastric schwannomas was heterogeneous and low, but slightly higher than that of muscularis propria. These features might help us differentiate gastric schwannomas from other submucosal tumors. Further investigation is needed to differentiate these mesenchymal tumors.

  19. Spinal Rosai–Dorfman disease: Case report of a rare disorde

    Directory of Open Access Journals (Sweden)

    A. Elsotouhy

    2015-12-01

    Conclusion: This is a rare case of spinal Rosai–Dorfman Disease with epidural and intradural components causing cord compression. To our knowledge, this represents the first case of combined epidural and intradural extramedullary lesions in the literature.

  20. Intramedullary versus extramedullary alignment of the tibial component in the Triathlon knee

    LENUS (Irish Health Repository)

    Cashman, James P

    2011-08-20

    Abstract Background Long term survivorship in total knee arthroplasty is significantly dependant on prosthesis alignment. Our aim was determine which alignment guide was more accurate in positioning of the tibial component in total knee arthroplasty. We also aimed to assess whether there was any difference in short term patient outcome. Method A comparison of intramedullary versus extramedullary alignment jig was performed. Radiological alignment of tibial components and patient outcomes of 103 Triathlon total knee arthroplasties were analysed. Results Use of the intramedullary was found to be significantly more accurate in determining coronal alignment (p = 0.02) while use of the extramedullary jig was found to give more accurate results in sagittal alignment (p = 0.04). There was no significant difference in WOMAC or SF-36 at six months. Conclusion Use of an intramedullary jig is preferable for positioning of the tibial component using this knee system.

  1. Intramedullary versus extramedullary alignment of the tibial component in the Triathlon knee

    Directory of Open Access Journals (Sweden)

    Synnott Keith

    2011-08-01

    Full Text Available Abstract Background Long term survivorship in total knee arthroplasty is significantly dependant on prosthesis alignment. Our aim was determine which alignment guide was more accurate in positioning of the tibial component in total knee arthroplasty. We also aimed to assess whether there was any difference in short term patient outcome. Method A comparison of intramedullary versus extramedullary alignment jig was performed. Radiological alignment of tibial components and patient outcomes of 103 Triathlon total knee arthroplasties were analysed. Results Use of the intramedullary was found to be significantly more accurate in determining coronal alignment (p = 0.02 while use of the extramedullary jig was found to give more accurate results in sagittal alignment (p = 0.04. There was no significant difference in WOMAC or SF-36 at six months. Conclusion Use of an intramedullary jig is preferable for positioning of the tibial component using this knee system.

  2. [Hearing restoration with cochlear implants after translabyrinthine vestibular schwannoma resection].

    Science.gov (United States)

    Bohr, C; Müller, S; Hornung, J; Hoppe, U; Iro, H

    2017-09-01

    Hearing restoration after translabyrinthine vestibular schwannoma resection is a challenge. Because the cochlea can begin to ossify a few months after cochlear or labyrinthine injury, the time interval for cochlear implant surgery is limited. To avoid complete ossification and to prolong the time interval until cochlear implantation, it is possible to insert a placeholder (depth gauge) into the cochlea and perform the cochlear implant surgery at a later time point (two-stage approach). The aim of this retrospective case series was to present the outcomes after restoration of hearing with cochlea implants in six patients and to evaluate the use of the depth gauge in practice. The hearing outcome of all patients with (n = 3) and without (n = 3) insertion of a depth gauge was measured with the Freiburg monosyllabic test without background noise at 65 dB. The first measurement was performed prior to the translabyrinthine vestibular schwannoma resection, the last measurement was performed up to 48 months after cochlear implantation. All 6 patients reached 22.5 ± 36.57% prior to vestibular schwannoma resection and 41.3 ± 26% 12 months after cochlear implantation. The understanding values of the patients with a depth gauge were 25.8 ± 16% after 12 months which is below the values of the other patients with 56.6 ± 25.0%. No severe intraoperative or postoperative complications occurred in any patient. The two-stage approach for cochlear implantation with depth gauge insertion following labrynthine incision and intact nerve appears to represent a very promising and safe variation for hearing restoration. Intensified research on this approach seems to be justified and necessary.

  3. Solitary extramedullary plasmacytoma of the maxillary antrum and orbit presenting as acute bacterial orbital cellulitis.

    Science.gov (United States)

    Kelly, S. P.; Lloyd, I. C.; Anderson, H.; Joyce, P. W.; Pace-Balzan, A.

    1991-01-01

    Orbital involvement by plasma cell tumours is rare. Orbital tumours do not generally present as an acute orbital inflammatory disease in adults, though tumours such as rhabdomyosarcoma may cause clinical signs similar to an acute orbital cellulitis in children. We describe a patient with bacterial orbital cellulitis and sinusitis who was found to have an extra-medullary plasmacytoma of the maxillary antrum and orbit and coexisting testicular seminoma. Images PMID:1854702

  4. Myelopathy due to Spinal Extramedullary Hematopoiesis in a Patient with Polycythemia Vera.

    Science.gov (United States)

    Ito, Shuhei; Fujita, Nobuyuki; Hosogane, Naobumi; Nagoshi, Narihito; Yagi, Mitsuru; Iwanami, Akio; Watanabe, Kota; Tsuji, Takashi; Nakamura, Masaya; Matsumoto, Morio; Ishii, Ken

    2017-01-01

    Extramedullary hematopoiesis (EMH) occasionally occurs in patients exhibiting hematological disorders with decreased hematopoietic efficacy. EMH is rarely observed in the spinal epidural space and patients are usually asymptomatic. In particular, in the patients with polycythemia vera, spinal cord compression due to EMH is extremely rare. We report a case of polycythemia vera, in which operative therapy proved to be an effective treatment for myelopathy caused by spinal EMH.

  5. Cord Compression due to Extramedullary Hematopoiesis in an Adolescent with Known Beta Thalassemia Major

    OpenAIRE

    Soman, Salil; Rosenfeld, David L; Roychowdhury, Sudipta; Drachtman, Richard A; Cohler, Alan

    2009-01-01

    We describe a 16 year-old male with ß thalassemia major and gait disturbances that had not been given blood transfusions due to a severe childhood transfusion reaction. Thoracic spine MRI demonstrated hematopoietic marrow throughout the spine and epidural masses causing cord compression consistent with extramedullary hematopoiesis (EMH). After treatment with steroids, radiotherapy and monitored blood transfusions, the patient demonstrated significant improvement of his paraspinal lesions a...

  6. Myelopathy due to Spinal Extramedullary Hematopoiesis in a Patient with Polycythemia Vera

    Directory of Open Access Journals (Sweden)

    Shuhei Ito

    2017-01-01

    Full Text Available Extramedullary hematopoiesis (EMH occasionally occurs in patients exhibiting hematological disorders with decreased hematopoietic efficacy. EMH is rarely observed in the spinal epidural space and patients are usually asymptomatic. In particular, in the patients with polycythemia vera, spinal cord compression due to EMH is extremely rare. We report a case of polycythemia vera, in which operative therapy proved to be an effective treatment for myelopathy caused by spinal EMH.

  7. Myelopathy due to Spinal Extramedullary Hematopoiesis in a Patient with Polycythemia Vera

    OpenAIRE

    Ito, Shuhei; Fujita, Nobuyuki; Hosogane, Naobumi; Nagoshi, Narihito; Yagi, Mitsuru; Iwanami, Akio; Watanabe, Kota; Tsuji, Takashi; Nakamura, Masaya; Matsumoto, Morio; Ishii, Ken

    2017-01-01

    Extramedullary hematopoiesis (EMH) occasionally occurs in patients exhibiting hematological disorders with decreased hematopoietic efficacy. EMH is rarely observed in the spinal epidural space and patients are usually asymptomatic. In particular, in the patients with polycythemia vera, spinal cord compression due to EMH is extremely rare. We report a case of polycythemia vera, in which operative therapy proved to be an effective treatment for myelopathy caused by spinal EMH.

  8. Renal Bleeding Due to Extramedullary Hematopoiesis in a Patient With Chronic Myelogenous Leukemia

    OpenAIRE

    Stephanie Zettner; Sandeep G. Mistry

    2014-01-01

    Chronic myelogenous leukemia (CML) is a myeloproliferative disorder that normally presents in middle-aged adults. Renal infiltration and extramedullary hematopoiesis in renal tissue has been rarely reported. This case report presents a patient with CML and renal insufficiency who developed gross hematuria. Efforts at controlling the hematuria led to a cascade of events propelled by the underlying disorder that ultimately led to a radical nephrectomy, multiorgan failure, and prolonged hospital...

  9. Treatment with hydroxyurea in thalassemia intermedia with paravertebral pseudotumors of extramedullary hematopoiesis.

    Science.gov (United States)

    Cario, H; Wegener, M; Debatin, K-M; Kohne, E

    2002-08-01

    Excessive ineffective erythropoiesis in thalassemia intermedia may cause paravertebral pseudotumors of extramedullary hematopoiesis. Due to the proximity to the spinal canal, these paravertebral masses carry the risk of severe neurological damage. Treatment strategies include hypertransfusion, radiotherapy, and laminectomy. Hydroxyurea, stimulating fetal hemoglobin synthesis, may represent an alternative therapeutic approach. We report on a 26-year-old patient suffering from thalassemia intermedia with progressive anemia symptoms and presenting multiple intrathoracic paravertebral pseudotumors of extramedullary hematopoiesis. Hypertransfusion therapy and splenectomy were followed by regular transfusion (baseline hemoglobin 10 g/dl) and chelation with desferrioxamine. With this treatment, clinical symptoms disappeared, paravertebral hematopoietic masses did not progress, but severe hemosiderosis developed within a few years. Hydroxyurea therapy was initiated to increase the efficacy of erythropoiesis, thereby reducing the required transfusion volume but suppressing concomitantly further expansion of extramedullary hematopoiesis, and finally leading to a reduction of transfusional iron load. Treatment was started with 4 mg/kg per day and stepwise increased to 12.5 mg/kg per day. The fetal hemoglobin concentration increased from 4.5 to 5.5 g/dl after 1 year and to 9.9 g/dl after 2 years of treatment. The yearly transfusion volume was halved during the 1st year of treatment. At present, after 26 months of treatment, the patient has been transfusion-independent for 10 months. Serum ferritin levels decreased from 2844 to 1335 ng/ml. Size and shape of paravertebral hematopoietic pseudotumors remained stable. No side effects of hydroxyurea have been observed. In thalassemia intermedia patients with extramedullary hematopoiesis, hydroxyurea may lead to independence from regular transfusion therapy without further expansion of ectopic hematopoietic tissue.

  10. Bilateral Pleural Effusion in a Patient with an Extensive Extramedullary Hematopoietic Mass

    OpenAIRE

    Yun Luo; Ying Zhang; Shi-feng Lou

    2013-01-01

    We present a 56-year-old woman with bilateral pleural effusions, widespread enlarged lymph nodes, and soft tissue masses located within the renal pelvis. The initially working diagnosis was tuberculosis and lymphoma. Further pathological examination of the lymph node biopsy confirmed a diagnosis of extramedullary hematopoiesis, and a bone marrow biopsy revealed myelofibrosis. Unlike common treatment options such as radiotherapy and/or surgery, intrathoracic cisplatin and dexamethasone for the...

  11. A Rare Presentation of Extramedullary Hematopoiesis in Post-polycythemic Myelofibrosis

    OpenAIRE

    Konca Degertekin, Ceyla; Özkurt, Zübeyde Nur; Akyürek, Nalan; Yağcı, Münci

    2012-01-01

    Polycythemia vera is a clonal proliferative disorder of the bone marrow that could possibly evolve into myelofibrosis in its natural course. Progression to myelofibrosis is usually a late stage complication and presents clinically with refractory cytopenias and extramedullary hematopoiesis (EMH). EMH can occur in any tissue during the course of post-polycythemic myelofibrosis. However, skin and cardiac involvements seems to be very rare. We present a 56-year-old woman with post-polycythemic m...

  12. Extramedullary haematopoiesis in Thalassaemia: results of radiotherapy: a report of three patients

    Energy Technology Data Exchange (ETDEWEB)

    Pistevou-Gompaki, K.; Paraskevopoulos, P.; Kotsa, K. [Theagenion Cancer Center, Thessaloniki (Greece); Skaragas, G.; Repanta, E. [Saint Paul`s Hospital, Thessaloniki (Greece)

    1996-10-01

    Extramedullary haematopoiesis is sometimes encountered in serve anaemia. Rarely, it may cause neurological symptoms, leading to spinal cord or cauda equina compression. Three patients with thalassaemia intermedia, who developed neurological complications, are described. The diagnoses were based on the clinical findings, computed tomography and magnetic resonance imaging. Small doses of radiotherapy (10-20 Gy in 5-10 fractions) relieved symptoms in all of these patients. Our experience supports the role of radiation therapy as a treatment for this complication. (Author).

  13. Gastric schwannoma: a benign tumor often misdiagnosed as gastrointestinal stromal tumor

    Directory of Open Access Journals (Sweden)

    Apurva S. Shah

    2015-10-01

    Full Text Available Gastric schwannomas are rare mesenchymal tumors that arise from the nerve plexus of gut wall. They present with nonspecific symptoms and are often detected incidentally. Preoperative investigation is not pathognomic and many are therefore misdiagnosed as gastrointestinal stromal tumors. We report a rare case of a 37-year old woman who underwent laparotomy for complex bilateral ovarian cyst with resection of gastric-gastrointestinal stromal tumor preoperatively, but confirmed to have a gastric schwannomas postoperatively. This case underscores the differential diagnosis of submucosal, exophytic gastric mass as schwannoma.

  14. Detection of synchronous gastric schwannoma on FDG PET/CT aided by discordant metabolic response.

    Science.gov (United States)

    Yap, June; Huang, Yi-Tung Tom; Lin, Michael

    2015-05-01

    This is a case of an unsuspected synchronous gastric schwannoma demonstrating increased F-FDG accumulation on PET/CT in a 65-year-old female patient diagnosed with non-Hodgkin lymphoma on the basis of different metabolic activity to other sites of disease at staging and discordant metabolic response to therapy. The gastric schwannoma was confirmed by histopathology and immunohistochemistry after surgical resection. This case adds to the limited literature on FDG-avid gastric schwannoma and highlights the importance of investigating differential metabolic activity and response on serial PET/CT imaging.

  15. Gastric Schwannoma: A Benign Tumor Often Misdiagnosed as Gastrointestinal Stromal Tumor.

    Science.gov (United States)

    Shah, Apurva S; Rathi, Pravin M; Somani, Vaibhav S; Mulani, Astha M

    2015-09-28

    Gastric schwannomas are rare mesenchymal tumors that arise from the nerve plexus of gut wall. They present with nonspecific symptoms and are often detected incidentally. Preoperative investigation is not pathognomic and many are therefore misdiagnosed as gastrointestinal stromal tumors. We report a rare case of a 37-year old woman who underwent laparotomy for complex bilateral ovarian cyst with resection of gastric-gastrointestinal stromal tumor preoperatively, but confirmed to have a gastric schwannomas postoperatively. This case underscores the differential diagnosis of submucosal, exophytic gastric mass as schwannoma.

  16. Nonvestibular schwannoma tumors in the cerebellopontine angle: A structured approach and management guidelines

    DEFF Research Database (Denmark)

    Springborg, J.B.; Poulsgaard, L.; Thomsen, Jens Christian

    2008-01-01

    The most common cerebellopontine angle (CPA) tumor is a vestibular schwannoma, but one in five CPA tumors are not vestibular schwannomas. These tumors may require different management strategies. Compared with vestibular schwannomas, symptoms and signs from cranial nerve VIII are less frequent......: other cranial nerve and cerebellar symptoms and signs predominate in patients with these less common CPA tumors. Computed tomography and magnetic resonance imaging often show features leading to the correct diagnosis. Treatment most often includes surgery, but a policy of observation or subtotal...

  17. Sociodemographic factors and vestibular schwannoma: a Danish nationwide cohort study

    DEFF Research Database (Denmark)

    Schüz, Joachim; Steding-Jessen, Marianne; Hansen, Søren

    2010-01-01

    Vestibular schwannoma (VS) (or acoustic neuroma) accounts for about 5%-6% of all intracranial tumors; little is known about the etiology. We investigated the association between various sociodemographic indicators and VS in a cohort of 3.26 million Danish residents, with 1087 cases identified in 35...... 308 974 person-years under risk, with data accrued from 1993 to 2006. Complete ascertainment of cases was ensured by using population-based and clinical cancer registries. Information on sociodemographic indicators was obtained on an annually updated individual level from Statistics Denmark. Log...

  18. Germinoma in the Internal Auditory Canal Mimicking a Vestibular Schwannoma

    Directory of Open Access Journals (Sweden)

    Rubén Martín-Hernández

    2014-01-01

    Full Text Available The appearance of a primary germinoma in the central nervous system but not on or near the midline or within the brain is exceptional. It may occur at any age; however, it is rare in patients over 50 years old. Only a handful of cases of germinomas located in the cerebellopontine angle were presented, but to our knowledge, there has been no description of an isolated germinoma in the internal auditory canal. We report a case of germinoma in the internal auditory canal in a 51-year-old man simulating the clinical and radiological characteristics of a vestibular schwannoma.

  19. Critical Airway Compromise due to a Massive Vagal Schwannoma

    LENUS (Irish Health Repository)

    McDermott, AM

    2016-05-01

    We describe the case of a 37-year-old man with a slowly enlarging neck lump and compressive symptoms. He presented to a separate institution 10 years prior where an observational approach was advocated. Following preoperative investigations and embolization, an 11cm vagal schwannoma was excised and vagus nerve was sacrificed. Although conservative management is appropriate for a select patient population, surgical excision is treatment of choice for cervical neurogenic tumours and paraganglionomas and must be considered in young patients or rapidly expanding tumours to avoid compressive symptoms, as in this case.

  20. Extramedullary hematopoiesis (EMH) in laboratory animals: offering an insight into stem cell research.

    Science.gov (United States)

    Chiu, Shao-Chih; Liu, Hua-Hsing; Chen, Chia-Ling; Chen, Pin-Ru; Liu, Ming-Chao; Lin, Shinn-Zong; Chang, Ko-Tung

    2015-01-01

    Extramedullary hematopoiesis (EMH) is a pathological process secondary to underlying bone marrow (BM) insufficiency in adults. It is characterized by the emergence of multipotent hematopoietic progenitors scattered around the affected tissue, most likely in the spleen, liver, and lymph node, etc. EMH in patients frequently receives less medical attention and is neglected unless a compressive or obstructive hematopoietic mass appears to endanger the patient's life. However, on a biological basis, EMH reflects the alteration of relationships among hematopoietic stem and progenitor cells (HSPCs) and their original and new microenvironments. The ability of hematopoietic stem cells (HSCs) to mobilize from the bone marrow and to accommodate and function in extramedullary tissues is rather complicated and far from our current understanding. Fortunately, many reports from the studies of drugs and genetics using animals have incidentally found EMH to be involved. Thereby, the molecular basis of EMH could further be elucidated from those animals after cross-comparison. A deeper understanding of the extramedullary hematopoietic niche could help expand stem cells in vitro and establish a better treatment in patients for stem cell transplantation.

  1. Peritoneal carcinomatosis-like implants of extramedullary hematopoiesis. An insolite occurrence during splenectomy for myelofibrosis.

    Science.gov (United States)

    Casaccia, Marco; Fornaro, Rosario; Frascio, Marco; Palombo, Denise; Stabilini, Cesare; Firpo, Emma; Gianetta, Ezio

    2017-01-01

    Primary myelofibrosis (MF) is a myeloproliferative neoplasm that results in debilitating constitutional symptoms, splenomegaly, and cytopenias. In patients with symptomatic splenomegaly, splenectomy remains a viable treatment option for MF patients with medically refractory symptomatic splenomegaly that precludes the use of ruxolitinib. We present the clinical case of a patient who was admitted to our Department to perform a splenectomy in MF as a therapeutic step prior to an allogeneic stem cell transplantation (ASCT). A laparotomic splenectomy and excision of whitish wide-spread peritoneal and omental nodulations was performed. There were no operative complications and the surgery was completed with minimal blood loss. The histopathological exam revealed an extramedullary hematopoiesis in both spleen and peritoneal nodules. In primary myelofibrosis it must always be kept in mind the possible presence of peritoneal implants of extramedullary hematopoiesis and ascites of reactive genesis. We report a rare case of peritoneal carcinomatosis-like implants of extramedullary hematopoiesis found at splenectomy for MF. Copyright © 2017 The Authors. Published by Elsevier Ltd.. All rights reserved.

  2. Increasing annual incidence of vestibular schwannoma and age at diagnosis

    DEFF Research Database (Denmark)

    Stangerup, Sven-Eric; Tos, Mirko; Caye-Thomasen, Per

    2004-01-01

    During the last 26 years the annual number of diagnosed vestibular schwannomas (VS) has been increasing. The aim of this study is to describe and analyse this increase. Since 1976, 1446 new cases of VS have been diagnosed at the authors' centre. Special focus was on the age at diagnosis, the loca......During the last 26 years the annual number of diagnosed vestibular schwannomas (VS) has been increasing. The aim of this study is to describe and analyse this increase. Since 1976, 1446 new cases of VS have been diagnosed at the authors' centre. Special focus was on the age at diagnosis......, the localization and the size of the tumour. The size of the tumour was registered as either intrameatal or with the largest extrameatal diameter. The annual number of diagnosed VS has increased from 26 in 1976 to 101 in 2001. The size of the diagnosed tumours has decreased from a median of 35 mm in 1979 to 10 mm...... incidence of VS can be explained only by earlier diagnosis and easier access to magnetic resonance (MR) scanning it should be expected that the median age at the time of diagnosis would decrease simultaneously. In this study, the median age at the time of diagnosis has been almost unchanged throughout...

  3. Transoral Robotic Surgery in Retrostyloid Parapharyngeal Space Schwannomas

    Science.gov (United States)

    Ansarin, Mohssen; Tagliabue, Marta; Chu, Francesco; Zorzi, Stefano; Proh, Michele; Preda, Lorenzo

    2014-01-01

    Parapharyngeal space (PPS) tumors are very rare, representing about 0.5% of head and neck neoplasms. An external surgical approach is mainly used. Several recent papers show how transoral robotic surgery (TORS) excision could be a prospective tool to remove mainly benign lesions in PPS; no cases of neurogenic tumors from the retrostyloid space treated with TORS have been reported. We present two cases which underwent TORS for schwannomas from the retrostyloid compartment of the parapharyngeal space. Clinical diagnosis of schwannoma was performed by magnetic resonance imaging (MRI). In the first case a 6 cm neurogenic tumor arose from the vagus nerve and in the second case a 5 cm mass from the sympathetic chain was observed. Both cases were treated successfully by the TORS approach using a new “J”-shaped incision through the mucosa and superior pharyngeal constrictor muscle. Left vocal cord palsy and the Claude Bernard Horner syndrome, respectively, were observed as expected postsurgical sequelae. In case 1 the first bite syndrome developed after three months, while no complications were observed in case 2. Both patients regained a normal swallowing function. TORS seems to be a feasible mini-invasive procedure for benign PPS masses including masses in the poststyloid space. PMID:25202464

  4. Obturator Nerve Schwannoma as a Mimic of Ovarian Malignancy

    Directory of Open Access Journals (Sweden)

    Tyler Gleason

    2017-01-01

    Full Text Available The obturator nerve is an extremely rare location for schwannomas to originate, and such diagnosis is typically not considered among the imaging diagnostic possibilities for a cystic-solid pelvic mass. A 63-year-old female with a known pelvic mass presented with increasing pelvic pain. The mass, which had been followed by serial imaging over five years, was described showing mixed solid and cystic components, likely arising from the left ovary. Although the key diagnosis to be excluded was a primary ovarian malignancy, the patient chose to pursue active surveillance. Over the five years of close observation, the lesion increased slowly, while her CA-125 level showed no significant elevation. Increase in size of the mass and worsening pain and concern for a gynecologic malignancy on MRI led her to ultimately consent to a hysterectomy with bilateral salpingooophorectomy. During the surgery, the mass was noted to be contiguous with the left obturator nerve. Pathologic evaluation revealed a schwannoma (WHO grade I. The patient’s postsurgical course was uneventful, without residual weakness in the left adductor muscles.

  5. Transoral Robotic Surgery in Retrostyloid Parapharyngeal Space Schwannomas

    Directory of Open Access Journals (Sweden)

    Mohssen Ansarin

    2014-01-01

    Full Text Available Parapharyngeal space (PPS tumors are very rare, representing about 0.5% of head and neck neoplasms. An external surgical approach is mainly used. Several recent papers show how transoral robotic surgery (TORS excision could be a prospective tool to remove mainly benign lesions in PPS; no cases of neurogenic tumors from the retrostyloid space treated with TORS have been reported. We present two cases which underwent TORS for schwannomas from the retrostyloid compartment of the parapharyngeal space. Clinical diagnosis of schwannoma was performed by magnetic resonance imaging (MRI. In the first case a 6 cm neurogenic tumor arose from the vagus nerve and in the second case a 5 cm mass from the sympathetic chain was observed. Both cases were treated successfully by the TORS approach using a new “J”-shaped incision through the mucosa and superior pharyngeal constrictor muscle. Left vocal cord palsy and the Claude Bernard Horner syndrome, respectively, were observed as expected postsurgical sequelae. In case 1 the first bite syndrome developed after three months, while no complications were observed in case 2. Both patients regained a normal swallowing function. TORS seems to be a feasible mini-invasive procedure for benign PPS masses including masses in the poststyloid space.

  6. Prevalence of hydrocephalus in 157 patients with vestibular schwannoma

    Energy Technology Data Exchange (ETDEWEB)

    Rogg, Jeffrey M.; Ahn, S.H.; Tung, G.A. [Rhode Island Hospital, Department of Diagnostic Imaging, Providence, Rhode Island (United States); Reinert, S.E. [Rhode Island Hospital, Lifespan Medical Computing, Providence, Rhode Island (United States); Noren, G. [Rhode Island Hospital, Department of Neurosurgery, Providence, Rhode Island (United States)

    2005-05-01

    The purpose of this study was to determine the prevalence of hydrocephalus in patients with vestibular schwannoma. A second objective was to investigate possible etiologies for hydrocephalus in this population by attempting to correlate the incidence and severity of hydrocephalus with tumor volume and extent of fourth ventricular compression. The MRI examinations of 157 adult patients with vestibular schwannoma were retrospectively reviewed. Tumor size was quantified, and the presence of accompanying hydrocephalus was assessed, categorized as communicating type or non-communicating type and then rated as mild, moderate or severe (grades 1-3). Next, the degree of fourth ventricular distortion caused by tumor mass effect was evaluated and categorized as mild, moderate or severe (grades 1-3). Spearman's rank correlation coefficient was used to test the relationships between tumor volume and (1) the extent of fourth ventricular effacement and (2) severity of hydrocephalus. Hydrocephalus was present in 28/157 (18%) cases and was categorized as mild in 11/28 (39%), moderate in 15/28 (54%) and severe in 2/28 (7%). Communicating-type hydrocephalus was present in 17/28 (61%) and non-communicating type in 11/28 (39%). There was a positive correlation between the grade of non-communicating hydrocephalus and tumor volume (r=0.38; P<0.001) and between the severity of fourth ventricular compression and extent of hydrocephalus in this group(r=0.43; P<0.001). (orig.)

  7. Preoperative predictive factors for hearing preservation in vestibular schwannoma surgery.

    Science.gov (United States)

    Rohit; Piccirillo, Enrico; Jain, Yogesh; Augurio, Angela; Sanna, Mario

    2006-01-01

    We performed a retrospective chart review to evaluate the various predictive factors for postoperative hearing preservation in the surgical management of vestibular schwannoma. Of 792 patients operated on for vestibular schwannoma between April 1987 and July 2002, 107 were candidates for hearing preservation surgery. These patients were divided into group 1 (hearing preserved) and group 2 (hearing not preserved), and both of these groups were evaluated for age, sex, pure tone average, sound discrimination score, tumor size, and auditory brain stem response parameters. A corrected chi2 test and a corrected t-test were used for statistical analysis. Multiple regression analysis was further done to evaluate independent predictive factors, either alone or in combination. The results were evaluated by use of the modified Sanna classification and the guidelines of the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS). Preoperative pure tone average and tumor size were the 2 predictive factors in our study. A Pearson correlation test showed that there was no multicollinearity between the factors. On multiple regression analysis by backward elimination of nonsignificant factors, we found that tumor size is an independent predictive factor for postoperative hearing. According to the modified Sanna classification, postoperative hearing was preserved in 11.2% of patients (equivalent to class A of AAO-HNS guidelines). In our series, preoperative pure tone average and tumor size were found to be predictors of postoperative hearing levels.

  8. Molecular and immunohistochemical distinction of equine sarcoid from schwannoma.

    Science.gov (United States)

    Bogaert, L; Heerden, M Van; Cock, H E V De; Martens, A; Chiers, K

    2011-05-01

    Ten equine skin tumors that had been classified as schwannomas on routine histological examination were analyzed by polymerase chain reaction for bovine papillomavirus DNA. All 10 were positive for bovine papillomavirus 1 or 2, and all 10 were immunohistochemically negative for S-100 protein and strongly positive for vimentin. Nine tumors were moderately positive for laminin and 8, for smooth muscle actin. Five tumors were variably and weakly positive for type IV collagen. The lack of S-100 protein expression made Schwann cells an unlikely cell of origin, as opposed to peripheral nerve sheath tumors, which typically express S-100 protein, at least in some neoplastic cells. The immunohistochemical reactivity is consistent with myofibroblastic origin of the neoplastic cells, although smooth muscle cell or pericyte origin cannot be ruled out. These tumors represent an atypical form of equine sarcoid. Polymerase chain reaction for bovine papillomavirus and S-100 immunohistochemistry are strongly recommended for all equine skin tumors with histological characteristics typical of schwannoma or peripheral nerve sheath tumor.

  9. The jugular foramen schwannomas: review of the large surgical series.

    Science.gov (United States)

    Bakar, Bulent

    2008-11-01

    Jugular foramen schwannomas are uncommon pathological conditions. This article is constituted for screening these tumors in a wide perspective. One-hundred-and-ninty-nine patients published in 19 articles between 1984 to 2007 years was collected from Medline/Index Medicus. The series consist of 83 male and 98 female. The mean age of 199 operated patients was 40.4 years. The lesion located on the right side in 32 patients and on the left side in 60 patients. The most common presenting clinical symptoms were hearing loss, tinnitus, disphagia, ataxia, and hoarseness. Complete tumor removal was achieved in 159 patients. In fourteen patients tumor reappeared unexpectedly. The tumor was thought to originate from the glossopharyngeal nerve in forty seven cases; vagal nerve in twenty six cases; and cranial accessory nerve in eleven cases. The most common postoperative complications were lower cranial nerve palsy and facial nerve palsy. Cerebrospinal fluid leakage, meningitis, aspiration pneumonia and mastoiditis were seen as other complications. This review shows that jugular foramen schwannomas still have prominently high morbidity and those complications caused by postoperative lower cranial nerve injury are life threat.

  10. Imaging-documented repeated intratumoral hemorrhage in vestibular schwannoma: a case report

    NARCIS (Netherlands)

    Mandl, E. S.; Vandertop, W. P.; Meijer, O. W. M.; Peerdeman, S. M.

    2009-01-01

    Intratumoral hemorrhage in vestibular schwannomas is rare. Symptoms often have an acute onset and include headache, nausea, vomiting, vertigo, and depressed consciousness. Intratumoral hemorrhage is probably caused by vascular fragility associated with tumor characteristics and growth. With

  11. Synchronous occurrence of colon adenocarcinoma and gastric schwannoma: case report and review of the literature.

    Science.gov (United States)

    Di Cataldo, Antonio; Trombatore, Claudia; Cocuzza, Aldo; Latino, Rosalia; Li Destri, Giovanni; Petrillo, Giuseppe

    2013-01-01

    We report a case of a 66-year-old man with a gastric schwannoma incidentally discovered during the treatment of a colon cancer. At the pre-operative computed tomography performed for the stadiation of the colonic tumor was incidentally noted the presence of a nodular tumor between the liver and the gastric wall. A wedge resection of this gastic tumor and the surgical resection of the left colon were performed all at once. The pathological examination of the gastric neoplasia revealed a picture consistent with gastric schwannoma. Gastrointestinal schwannomas are difficult but not impossible to diagnose preoperatively although they are often asymptomatic and radiologic findings are often nonspecific. Radiological features of Gastrointestinal schwannomas described in literature are reviewed. The treatment of choice is complete surgical excision with free margins because of diagnostic uncertainty, and the long-term outcome is excellent as these lesions are uniformly benign.

  12. ACOUSTIC SCHWANNOMA - Two Case Reports and An Insight into Diagnostic Imaging

    Directory of Open Access Journals (Sweden)

    Sunali Khanna

    2003-01-01

    Full Text Available Two unusual and interesting cases of acoustic (vestibular schwannoma are reported, The advances in diagnostic imaging and their importance in early detection of these tumours is also discussed.

  13. Dumbbell Shaped Schwannoma of the Lateral Chest Wall masquandering as a soft tissue sarcoma.

    Science.gov (United States)

    Krishnamurthy, Arvind; Raghunandhan, G C; Majhi, Urmila

    2015-09-01

    Schwannomas are benign, slow growing nerve sheath tumours of Schwann cell origin. They predominantly are known to involve the head, neck and the flexor surfaces of the extremities, retroperitoneum and the posterior spinal roots. The chest wall is a relatively uncommon location for a schwannoma, the vast majority of which are intra-thoracic, which are usually located in the posterior mediastinum and bulge into the thoracic cavity. Schwannomas arising from the lateral chest wall are relatively uncommon (chest wall i.e. with an intra-thoracic and extra-thoracic component, is extraordinarily rare and to the best of our knowledge only one case has been reported prior. We report possibly the second case of a dumbbell shaped lateral chest wall schwannoma in a 33-year-old female patient which masquandered as a case of soft tissue sarcoma.

  14. Extramedullary hematopoiesis presented as cytopenia and massive paraspinal masses leading to cord compression in a patient with hereditary persistence of fetal hemoglobin

    OpenAIRE

    Katchi, Tasleem; Kolandaivel, Krishna; Khattar, Pallavi; Farooq, Taliya; Islam, Humayun; Liu, Delong

    2016-01-01

    Background Extramedullary hematopoeisis (EMH) can occur in various physiological and pathologic states. The spleen is the most common site of EMH. Case presentation We report a case with hereditary persistence of fetal hemoglobin with extramedullary hematopoiesis presented as cord compression and cytopenia secondary to multi-paraspinal masses. Conclusion Treatment can be a challenge. Relapse is a possibility.

  15. Extramedullary hematopoiesis presented as cytopenia and massive paraspinal masses leading to cord compression in a patient with hereditary persistence of fetal hemoglobin.

    Science.gov (United States)

    Katchi, Tasleem; Kolandaivel, Krishna; Khattar, Pallavi; Farooq, Taliya; Islam, Humayun; Liu, Delong

    2016-01-01

    Extramedullary hematopoeisis (EMH) can occur in various physiological and pathologic states. The spleen is the most common site of EMH. We report a case with hereditary persistence of fetal hemoglobin with extramedullary hematopoiesis presented as cord compression and cytopenia secondary to multi-paraspinal masses. Treatment can be a challenge. Relapse is a possibility.

  16. A case of schwannoma of the common peroneal nerve in the knee

    Directory of Open Access Journals (Sweden)

    Tayyar Taylan Öz

    2017-02-01

    Full Text Available Schwannomas are benign neurogenic tumors of peripheral nerves that originate from neural sheath. The aim of this case report is to bring to mind the schwannoma in the patient with knee pain. A 39-year-old woman presented with a complaint of knee pain of three months history. After surgical intervention, the patient’s complaints completely disappeared in the post-operative period. This should come to mind in the differential diagnosis of knee pain.

  17. A Case of Schwannoma of the Common Peroneal Nerve in the Knee

    Science.gov (United States)

    Öz, Tayyar Taylan; Aktaş, Birol; Özkan, Korhan; Özturan, Burak; Kilic, Bulent; Demiroğlu, Murat

    2017-01-01

    Schwannomas are benign neurogenic tumors of peripheral nerves that originate from neural sheath. The aim of this case report is to bring to mind the schwannoma in the patient with knee pain. A 39-year-old woman presented with a complaint of knee pain of three months history. After surgical intervention, the patient’s complaints completely disappeared in the post-operative period. This should come to mind in the differential diagnosis of knee pain. PMID:28286620

  18. The influence of unilateral vestibular dysfunction on spatial orientation in patients after vestibular schwannoma surgery

    OpenAIRE

    Šponarová, Lenka

    2010-01-01

    The aim of this diploma thesis is to evaluate spatial orientation in the patients after vestibular schwannoma surgery. In the theoretical part, there are summarized the facts about vestibular system; there is described the clinical picture of unilateral vestibular dysfunction and the field of vestibular schwannoma and spatial orientation in general. In the practical part, we performed the investigation of the spatial orientation using the Blue Velvet Arena System and posturography using the p...

  19. Gastric Schwannoma: A Rare but Important Differential Diagnosis of a Gastric Submucosal Mass

    OpenAIRE

    Yoon, William; Paulson, Kari; Mazzara, Paul; Nagori, Sweety; Barawi, Mohammed; Berri, Richard

    2012-01-01

    Schwannomas are generally slow growing asymptomatic neoplasms that rarely occur in the GI tract. However, if found, the most common site is the stomach. Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract, and 60–70% of them occur in the stomach. Owing to their typical presentation as submucosal neoplasms, gastric schwannomas and GISTs appear grossly similar. Accordingly, the differential diagnosis for a gastric submucosal mass should i...

  20. Gastric GIST or gastric schwannoma?A diagnostic dilemma in a young female

    OpenAIRE

    Mohanty, Sudhir Kumar; Jena, Kumarmani; Mahapatra, Tanmaya; Dash, Jyoti Ranjan; Meher, Dibyasingh; John, Ajax; Nayak, Manjushree; Bano, Shafqat

    2016-01-01

    Introduction: Gastrointestinal stromal tumor (GIST) is the commonest mesenchymal tumor of GI tract and 60–70% of it seen in the stomach, whereas Gastric schwannoma is a benign, slow growing and one of the rare neoplasms of stomach. Age distribution, clinical, radiological features and gross appearance of both tumors are similar. Presentation of case: We report a rare case of gastric schwannoma in a 20-year-old girl, who underwent subtotal gastrectomy with the suspicion of a GIST preoperati...

  1. Intranodal Schwannoma Mimicking a Gastrointestinal Stromal Tumor of the Stomach: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Nam, Kyung Bum; Namkyoung, Sook; Kim, Heung Cheol [Dept. of Radiology, Chuncheon Scared Heart Hospital, Hallym University College of Medicine, Chuncheon (Korea, Republic of); Kim, Hae Sung; Ryu, Byoung Yoon [Dept. of General Surgery, Chuncheon Scared Heart Hospital, Hallym University College of Medicine, Chuncheon (Korea, Republic of); Cha, Young Hee [Dept. of Pathology, Chuncheon Scared Heart Hospital, Hallym University College of Medicine, Chuncheon (Korea, Republic of)

    2011-10-15

    A 66-year-old-woman is presented with intranodal schwannoma of the retroperitoneum. Ultrasonography (US) and computed tomography (CT) results demonstrated a large encapsulated mass with internal cystic or necrotic portions in the gastrosplenic space. The tumor abutted the greater curvature of the gastric body and slightly indented the proximal small bowel loops on a small bowel series. The observations suggested a gastrointestinal stromal tumor. The mass was surgically proven to be a retroperitoneal tumor and histopathologically intranodal ancient schwannoma.

  2. Scrotal extratesticular schwannoma: a case report and review of the literature.

    Science.gov (United States)

    Palleschi, Giovanni; Carbone, Antonio; Cacciotti, Jessica; Manfredonia, Giorgia; Porta, Natale; Fuschi, Andrea; de Nunzio, Cosimo; Petrozza, Vincenzo; Pastore, Antonio Luigi

    2014-04-28

    Schwannomas are tumours arising from Schwann cells, which sheath the peripheral nerves. Here, we report a rare case of left intrascrotal, extratesticular schwannoma. Although rare, scrotal localisation of schwannomas has been reported in male children, adult men, and elderly men. They are usually asymptomatic and are characterised by slow growth. Patients generally present with an intrascrotal mass that is not associated with pain or other clinical signs, and such cases are self-reported by most patients. Imaging modalities (such as ultrasonography, computed tomography, and magnetic resonance imaging) can be used to determine tumour size, exact localisation, and extension. However, the imaging findings of schwannoma are non-specific. Therefore, only complete surgical excision can result in diagnosis, based on histological and immunohistochemical analyses. If the tumour is not entirely removed, recurrences may develop, and, although malignant change is rare, this may occur, especially in patients with a long history of an untreated lesion. Thus, follow up examinations with clinical and imaging studies are recommended for scrotal schwannomas. A 52-year-old man presented with a 3-year history of asymptomatic scrotal swelling. Physical examination revealed a palpable, painless, soft mass in the left hemiscrotum. After surgical removal of the mass, its histological features indicated schwannoma. Schwannoma should be considered in cases of masses that are intrascrotal but extratesticular. Ultrasonography provides the best method of confirming the paratesticular localisation of the tumour, before surgical removal allows histopathological investigation and definitive diagnosis. Surgery is the standard therapeutic approach. To prevent recurrence, particular care should be taken to ensure complete excision. This case report includes a review of the literature on scrotal schwannomas.

  3. Case Report: Sciatic nerve schwannoma - a rare cause of sciatica [version 1; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    Sunil Munakomi

    2017-03-01

    Full Text Available Herein we report a rare case of a sciatic nerve schwannoma causing sciatica in a 69-year-old female. Sciatic nerve schwannoma is a rare entity. It should always be considered as a possible cause of sciatica in patients that present with symptoms of sciatica with no prolapsed disc in the lumbar spine and a negative crossed straight leg raise test. Timely diagnosis and complete excision of the lesion leads to complete resolution of the symptoms of such patients.

  4. Glucose transporter 3 and 1 may facilitate high uptake of 18F-FDG in gastric schwannoma.

    Science.gov (United States)

    Shimada, Yutaka; Sawada, Shigeaki; Hojo, Shozo; Okumura, Tomoyuki; Nagata, Takuya; Nomoto, Kazuhiro; Tsukada, Kazuhiro

    2013-11-01

    Recently, some gastric schwannomas have been reported to have high uptake of FDG. However, Glut-1 was reported to be negative in gastric schwannomas tested. A 64-year-old female patient received a laparoscopic partial gastrectomy for a FDG PET-positive submucosal tumor (SUVmax 6.61). The resected tumor was diagnosed as a benign gastric schwannoma. Glut family immunohistochemical examination revealed diffuse positive expression of Glut-3 and partial positive expression of Glut-1. On the other hand, Glut-2 and Glut-4 expression in the tumor were negative. This case suggested that Glut-3 and Glut-1 expression were facilitators of high FDG uptake in the benign gastric schwannoma.

  5. Ancient Schwannoma of Ansa Cervicalis: A Rare Clinical Entity and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Satyajit Rath

    2015-01-01

    Full Text Available Ancient schwannoma is an uncommon variant of schwannoma, a benign tumor arising from the nerve sheath. It is reported to arise from any nerves except optic and olfactory. However, only six cases of ancient schwannomas arising from ansa cervicalis nerve have been reported to date in English literature. Proper preoperative evaluation is necessary to rule out other causes of neck mass such as thyroid lesions, lymphadenopathy, and carotid body tumor. We report a case of ancient schwannoma arising from the ansa cervicalis nerve. The origin of the lesion from ansa cervicalis was confirmed by intraoperative finding. Postoperative histopathological examination revealed degenerative changes including pleomorphism, cellular atypia, large nuclei with prominent nucleoli, and paucity of mitotic figures. Periphery of the mass showed nuclear palisading with characteristic verocay bodies. Immunohistochemical evaluation for S-100 showed diffuse positivity of the tumor cells, thereby confirming the diagnosis of schwannoma. We consider that schwannoma of cervical region can have origin from any nerve and should try to identify the origin pre- and intraoperatively. The postoperative complications depend on the nerve of origin and the precision of the surgery performed.

  6. Gamma Knife radiosurgery for vestibular schwannoma: case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Fairbanks Robert K

    2009-12-01

    Full Text Available Abstract Vestibular schwannomas, also called acoustic neuromas, are benign tumors of the vestibulocochlear nerve. Patients with these tumours almost always present with signs of hearing loss, and many also experience tinnitus, vertigo, and equilibrium problems. Following diagnosis with contrast enhanced MRI, patients may choose to observe the tumour with subsequent scans or seek active treatment in the form of microsurgery, radiosurgery, or radiotherapy. Unfortunately, definitive guidelines for treating vestibular schwannomas are lacking, because of insufficient evidence comparing the outcomes of therapeutic modalities. We present a contemporary case report, describing the finding of a vestibular schwannoma in a patient who presented with dizziness and a "clicking" sensation in the ear, but no hearing deficit. Audible clicking is a symptom that, to our knowledge, has not been associated with vestibular schwannoma in the literature. We discuss the diagnosis and patient's decision-making process, which led to treatment with Gamma Knife radiosurgery. Treatment resulted in an excellent radiographic response and complete hearing preservation. This case highlights an atypical presentation of vestibular schwannoma, associated with audible "clicks" and normal hearing. We also provide a concise review of the available literature on modern vestibular schwannoma treatment, which may be useful in guiding treatment decisions.

  7. Role of endoscopic ultrasound and endoscopic resection for the treatment of gastric schwannoma.

    Science.gov (United States)

    Hu, Jinlong; Liu, Xiang; Ge, Nan; Wang, Sheng; Guo, Jintao; Wang, Guoxin; Sun, Siyu

    2017-06-01

    Endoscopic ultrasound (EUS) and endoscopic resection play an important role in gastric submucosal tumor. However, there were few articles regarding EUS and endoscopic resection of gastric schwannomas. Our aim was to evaluate the role of EUS and endoscopic resection in treating gastric schwannomas.We retrospectively reviewed 14 patients between March 2012 and April 2016 with gastric schwannomas and who received EUS and endoscopic resection. EUS characteristics, endoscopic resection, tumor features, and follow-up were evaluated in all the patients.Fourteen patients were enrolled in the present study. The patients' ages ranged from 25 to 72 years (mean age, 52.6 years). On EUS, all tumors were originating from muscularis propria and hypoechoic. Ten tumors have the extraluminal growth patterns and 4 tumors have the intraluminal growth patterns. Marginal halos were observed in 7 lesions. No cystic change and calcification were found inside the lesions. Complete endoscopic resection was performed in all the patients with no complications occurring in any patients. No recurrence or metastases was found in all patients during the follow-up period.Gastric schwannoma has some characteristics on EUS, but it is difficult to differentiate gastric schwannoma from gastrointestinal stromal tumor. Endoscopic resection is an effective and safe treatment for gastric schwannoma with an excellent follow-up outcome.

  8. A Computer Navigation System Analysis of the Accuracy of the Extramedullary (Tibial Alignment Technique in Total Knee Arthroplasty (TKA

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    EK Chee

    2010-07-01

    Full Text Available In total knee arthroplasty, mechanical alignment guides have improved the accuracy of implant alignment, but errors are not uncommon. In the present study, an image-free computer-assisted navigation system was used to analyse the accuracy of an extramedullary (tibial alignment system, which is based on predetermined, fixed anatomical landmarks. Comparisons were made between two surgeons, with different levels of competency in order to determine if experience affected the accuracy of extramedullary tibial jig placement, in either the coronal and sagittal planes or both planes. The results showed that the accuracy of the extramedullary tibial alignment system, in the coronal plane (in up to 80-87% of cases was much better than for posterior slope, and sagittal plane. Surgeon experience was not a significant factor.

  9. Intrathoracic extramedullary hematopoiesis presenting as tumor-simulating lesions of the mediastinum in α-thalassemia: A case report.

    Science.gov (United States)

    An, Jun; Weng, Yimin; He, Jinyuan; Li, Yun; Huang, Shaohong; Cai, Songwang; Zhang, Junhang

    2015-10-01

    Extramedullary hematopoiesis (EMH) is a rare disease, where hematological disorder drives extramedullary hematopoietic tumor formation in multiple regions of the body. The present study reports a case of EMH presenting as multiple tumor-like lesions of mediastinum in a 61-year-old male with α-thalassemia, which was subjected to a video-assisted thoracoscopic surgery tissue biopsy to differentiate it from other mediastinal tumors. To date, only three cases of EMH in patients with α-thalassemia have been described in the literature. Patients with EMH typically exhibit no hematological disorder preoperatively and therefore EMH is frequently misdiagnosed. In the present study, along with a literature review of the clinicopathological features of EMH, the diagnosis and treatment of this rare case was discussed, in order to differentiate diagnosis, and particularly to distinguish EHM from extramedullary myeloid sarcoma.

  10. Temporal bone extramedullary hematopoiesis as a causeof pediatric bilateral conductive hearing loss:Case report and review of the literature.

    Science.gov (United States)

    Lanigan, Alexander; Fordham, M Taylor

    2017-06-01

    Extramedullary hematopoiesis occurs in children with hemoglobinopathy and chronic anemia. The liver and spleen are often affected first, but other foci can develop to support erythrocyte demand. We report a case of a nine-year-old with beta thalassemia and temporal bone extramedullary hematopoiesis causing ossicular fixation and bilateral conductive hearing loss. There is only one case in the literature describing this phenomenon in pediatric patients, and this is the first case report of bilateral hearing loss from this physiologic phenomenon. Otolaryngologists should consider this etiology in patients with chronic anemia and conductive hearing loss in the absence of otitis media. Published by Elsevier B.V.

  11. Efficacy of endoscopic ultrasound-guided fine-needle aspiration for schwannoma: six cases of a retrospective study.

    Science.gov (United States)

    Takasumi, Mika; Hikichi, Takuto; Takagi, Tadayuki; Suzuki, Rei; Watanabe, Ko; Nakamura, Jun; Sugimoto, Mitsuru; Kikuchi, Hitomi; Konno, Naoki; Waragai, Yuichi; Asama, Hiroyuki; Obara, Katsutoshi; Ohira, Hiromasa

    2017-08-09

    Schwannomas are difficult to diagnose using imaging alone. Endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) is an effective and safe tissue sampling technique. Nevertheless, few reports have described EUS-FNA for schwannoma. This study evaluates the efficacy of EUS-FNA for diagnosing schwannoma. This retrospective study examined six consecutive schwannoma patients who were diagnosed as having schwannoma either from EUS-FNA results or from surgically resected specimens. The primary endpoint was diagnostic accuracy of EUS-FNA for schwannoma. The secondary endpoint was EUS-FNA safety. Based on cytomorphologic features and immunocytochemistry results after EUS-FNA, 4 out of 6 patients (66.7%) were diagnosed with schwannoma. The diagnoses before EUS-FNA were the following: 3 cases of gastric subepithelial lesion (SEL, suspicious for gastrointestinal stromal tumor), 1 case of intraperitoneal tumor, 1 case of retroperitoneal tumor, and 1 case of pancreatic tumor, with sizes of 15-44 mm (median 36 mm). No case was diagnosed as schwannoma solely based on image findings. Two cases of gastric SELs could not be diagnosed as schwannoma by EUS-FNA before surgery. Inadequate sampling and a lack of additional material for immunohistochemical studies could have engendered less-definite diagnoses in those cases. No procedural adverse events occurred. The diagnostic accuracy rate of EUS-FNA for schwannoma is somewhat low. However, tissue samples were obtained safely using this method. Moreover, it is an important procedure for diagnosing schwannoma, which cannot be diagnosed solely from image findings.

  12. Increasing annual incidence of vestibular schwannoma and age at diagnosis

    DEFF Research Database (Denmark)

    Stangerup, Sven-Eric; Tos, Mirko; Caye-Thomasen, Per

    2004-01-01

    During the last 26 years the annual number of diagnosed vestibular schwannomas (VS) has been increasing. The aim of this study is to describe and analyse this increase. Since 1976, 1446 new cases of VS have been diagnosed at the authors' centre. Special focus was on the age at diagnosis...... in 2001. In the first years large and giant tumours dominated, in contrast to recent years in which intrameatal and small tumours dominated. The median age at the time of diagnosis has been almost unchanged through the period (median 55 years). If the decreasing size of the tumour and the increasing...... incidence of VS can be explained only by earlier diagnosis and easier access to magnetic resonance (MR) scanning it should be expected that the median age at the time of diagnosis would decrease simultaneously. In this study, the median age at the time of diagnosis has been almost unchanged throughout...

  13. VESTIBULAR SCHWANNOMA (ACOUSTIC NEUROMA) MIMICKING TEMPOROMANDIBULAR DISORDERS: A CASE REPORT

    Science.gov (United States)

    Bisi, Maurício A.; Selaimen, Caio M. P.; Chaves, Karen D.; Bisi, Melissa C.; Grossi, Márcio L.

    2006-01-01

    Approximately 6 to 16% of patients with trigeminal neuralgia symptoms present intracranial tumors, the most common being the vestibular schwannoma (acoustic neuroma). Some symptoms reported by patients include hearing loss, tinnitus, headaches, vertigo and trigeminal disturbances. An increased muscle response in the surrounding head and neck musculature may also be observed, which mimics signs and symptoms of temporomandibular disorders. In these cases, magnetic resonance imaging (MRI) has proved to be a useful tool in tumor diagnosis. The differential diagnosis between myofascial and neuralgic pain is important, as both may present similar characteristics, while being of different origin, and demanding special treatment approaches. The purpose of this paper is to demonstrate the relationship among trigeminal neuralgia symptoms, intracranial tumors and temporomandibular dysfunction by presenting a clinical case. PMID:19089251

  14. Isolated cochlear neuritis from varicella reactivation mimicking a vestibular schwannoma

    Directory of Open Access Journals (Sweden)

    Adam D. Goodale

    2016-09-01

    Full Text Available We present a case of a patient with progressive unilateral sensorineural hearing loss and tinnitus with internal auditory canal enhancement on magnetic resonance imaging (MRI secondary to isolated cochlear neuritis from varicella reactivation. MRI following antiviral treatment showed resolution of enhancement. Varicella reactivation is commonly seen in the form of Ramsay Hunt syndrome, which is known to produce abnormal MRI enhancement from facial and vestibulocochlear neuritis; however, its characteristic clinical signs aid the diagnosis. This case is unique in that the only manifestation of varicella infection was unilateral hearing loss. This case outlines the importance of maintaining a broad differential diagnosis in the evaluation of unilateral hearing loss as well as recognizing the limited specificity of MRI. Keywords: Vestibular schwannoma, Acoustic neuroma, Vestibular neuritis, Ramsay Hunt syndrome, Varicella zoster virus

  15. Sociodemographic factors and vestibular schwannoma: a Danish nationwide cohort study

    DEFF Research Database (Denmark)

    Schüz, Joachim; Steding-Jessen, Marianne; Hansen, Søren

    2010-01-01

    Vestibular schwannoma (VS) (or acoustic neuroma) accounts for about 5%-6% of all intracranial tumors; little is known about the etiology. We investigated the association between various sociodemographic indicators and VS in a cohort of 3.26 million Danish residents, with 1087 cases identified in 35...... 308 974 person-years under risk, with data accrued from 1993 to 2006. Complete ascertainment of cases was ensured by using population-based and clinical cancer registries. Information on sociodemographic indicators was obtained on an annually updated individual level from Statistics Denmark. Log.......23-0.50) compared with married men with a higher education. Lower incidence rates were also observed among unemployed or early-retirement pensioners, whereas there were no differences in incidence rates across the broad groups of occupations and across the types of districts. Sociodemographic indicators were...

  16. Management of Intrathoracic Benign Schwannomas of the Brachial Plexus

    Directory of Open Access Journals (Sweden)

    Alessandro Bandiera

    2014-01-01

    Full Text Available Primary tumours of the brachial plexus are rare entities. They usually present as extrathoracic masses located in the supraclavicular region. This report describes two cases of benign schwannomas arising from the brachial plexus with an intrathoracic growth. In the first case the tumour was completely intrathoracic and it was hardly removed through a standard posterolateral thoracotomy. In the second case the tumour presented as a cervicomediastinal lesion and it was resected through a one-stage combined supraclavicular incision followed by left video-assisted thoracoscopic surgery. A brachial plexus tumour should be suspected not only in patients with a supraclavicular or cervicomediastinal mass but also in those with intrathoracic apical lesions. A preoperative magnetic resonance imaging study of brachial plexus should be performed in such cases in order to plan the correct surgical approach.

  17. Gastrik Schwannoma: Bilgisayarlı Tomografi Bulguları

    OpenAIRE

    Bulut, H.Taner; Ara, Cengiz; Yılmaz, Mehmet

    2015-01-01

    Schwannoma, gastrointestinal duvar nöral pleksusunun Schwann hücrelerinden kaynaklanan, sindirim sisteminin mezenkim kaynaklı, nadir tümörlerinden biridir. Üst gastrointestinal sistem endoskopisi ilk değerlendirme için önemli olmakla beraber ekzofitik uzanım gösteren subserozal lezyonlarda tanısal olmayabilir. Bu durumda; kesitsel görüntüleme yöntemleri tümörün karakterizasyonu ve komşu organlar ile olan ilişkisini göstermede faydalı olabilir. Biz 56 yaşındaki bir erkek hastad...

  18. [A new case of "olfactory schwannoma"; presentation and literature review].

    Science.gov (United States)

    Martínez-Soto, L; Alfaro-Baca, R; Torrecilla-Sardón, M V; Fernández-Vallejo, B; Ferreira-Muñóz, R; De Diego, T

    2009-06-01

    We report the case of a 54-year-old man who presented at the Emergency Department with intense headache of 6-days duration and sporadic nominal dysphasia. He did not present anosmia and the rest of the examination was normal. The emergency CT and the posterior cerebral MR showed a great subfrontal extra-axial mass of 7 x 6 x 5 cm, over the right side of the cribiform plate, hetereogeneously enhancing after gadolinium administration. Preoperative diagnosis was olfactory groove meningioma. After total removal by bifrontal craniotomy the histopathological diagnosis was schwannoma of the conventional type. Owing to the unusual frequency of this kind of tumors (26 to the date), we review the literature, the possible radiological differences with olfactory groove meningiomas and the different theories about their origin.

  19. Comparison of extramedullary and intramedullary devices for treatment of subtrochanteric femoral fractures at tertiary level center

    Directory of Open Access Journals (Sweden)

    Yadav Sanjay

    2014-06-01

    Full Text Available Objective: The treatment of subtrochanteric fractures is challenging and treatment modalities and implants are constantly evolving. This study attempts to revisit and compare extramedullary vs. intramedullary devices in relatively young population. Methods: Thirty patients with subtrochanteric fractures were enrolled and treated with extramedullary or intramedullary devices and follow-up continued one year for clinico-radiological assessment. Results: The mean age of patients was 37.53 years. Most were males between 21-40 years old. The dominant mode of injury was traffic accidents (66%. Fractures were classifi ed according to Russell-Taylor classifi cation. Forty percent were Russell-Taylor type IA, 37% type IB and 23% type IIA. Average time to surgery was 3.6 days from the time of admission to hospital. Mean duration of surgery was 45 minutes for intramedullary device (group A and 105 minutes for extramedullary device (group B. Average blood loss was 100 ml in group A and 200 ml in group B. Mean duration of radiation exposure was 130 seconds and 140 seconds for groups A and B, while average duration of hospital stay was 12 days and 16 days respectively. Excellent results were seen in 47% of cases in group A and 33% of cases in group B. Conclusion: Intramedullary device is a reliable implant for subtrochanteric fractures. It has high rates of union with minimal soft-tissue damage. Intramedullary fixation has biological and biomechanical advantages, but surgery is technically demanding. Gradual learning and patience is needed to make this method truly rewarding. Key words: Subtrochanteric fractures; Intramedullary; Dynamic hip screw

  20. Intradural spine surgery may not carry an increased risk of shunt revision compared with extradural spine surgery in pediatric patients with myelomeningocele.

    Science.gov (United States)

    Kuhn, Elizabeth N; Hopson, Betsy; Conklin, Michael J; Blount, Jeffrey P

    2018-01-01

    OBJECTIVE Patients with myelomeningocele are often affected by scoliosis and tethered cord syndrome, and frequently require spine surgery. Intradural spine surgeries may carry an inherently higher risk of inducing shunt malfunction due to entry into the subarachnoid space. In this study, the authors sought to compare rates of shunt malfunction after intradural and extradural spine surgeries among pediatric patients with myelomeningocele. METHODS The authors reviewed records of the National Spina Bifida Program Registry for Children's Hospital of Alabama. The Exago reporting function was used to identify patients who had received at least one of the following procedures: shunt revision, tethered cord release (TCR), or spinal fusion for deformity. The registry records were reviewed for all identified patients to determine if a shunt revision was performed within the 1st year after TCR or spinal fusion. RESULTS Final analyses included 117 patients, of whom 39 underwent spinal fusion and 78 underwent TCR. Among patients who underwent spinal fusion, shunt revision was performed within 30 days in 2 patients (5.1%), within 60 days in 2 (5.1%), within 90 days in 4 (10.3%), and within 1 year in 5 (12.8%). Among patients who underwent TCR, shunt revision was performed within 30 days in 7 patients (9.0%), within 60 days in 10 (12.8%), within 90 days in 11 (14.1%), and within 1 year in 17 (21.8%). Using the log-rank test, there was no significant difference in Kaplan-Meier curves between intradural and extradural groups (p = 0.59). CONCLUSIONS In a review of single-institution registry data, the authors found no statistically significant difference in the risk of shunt malfunction after intradural and extradural spine surgeries.

  1. Probabilistic Tractography of the Cranial Nerves in Vestibular Schwannoma.

    Science.gov (United States)

    Zolal, Amir; Juratli, Tareq A; Podlesek, Dino; Rieger, Bernhard; Kitzler, Hagen H; Linn, Jennifer; Schackert, Gabriele; Sobottka, Stephan B

    2017-11-01

    Multiple recent studies have reported on diffusion tensor-based fiber tracking of cranial nerves in vestibular schwannoma, with conflicting results as to the accuracy of the method and the occurrence of cochlear nerve depiction. Probabilistic nontensor-based tractography might offer advantages in terms of better extraction of directional information from the underlying data in cranial nerves, which are of subvoxel size. Twenty-one patients with large vestibular schwannomas were recruited. The probabilistic tracking was run preoperatively and the position of the potential depictions of the facial and cochlear nerves was estimated postoperatively by 3 independent observers in a blinded fashion. The true position of the nerve was determined intraoperatively by the surgeon. Thereafter, the imaging-based estimated position was compared with the intraoperatively determined position. Tumor size, cystic appearance, and postoperative House-Brackmann score were analyzed with regard to the accuracy of the depiction of the nerves. The probabilistic tracking showed a connection that correlated to the position of the facial nerve in 81% of the cases and to the position of the cochlear nerve in 33% of the cases. Altogether, the resulting depiction did not correspond to the intraoperative position of any of the nerves in 3 cases. In a majority of cases, the position of the facial nerve, but not of the cochlear nerve, could be estimated by evaluation of the probabilistic tracking results. However, false depictions not corresponding to any nerve do occur and cannot be discerned as such from the image only. Copyright © 2017 Elsevier Inc. All rights reserved.

  2. Extramedullary Hematopoiesis in a Man With β-Thalassemia: An Uncommon Cause of an Adrenal Mass

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    Zeighami

    2015-06-01

    Full Text Available Introduction Extramedullary hematopoiesis (EMH commonly occurs in the spleen, liver and lymph nodes. Rare cases of EMH in the adrenal gland have been reported. Case Presentation We report the case of a 33-year-old man from the South of Iran suffering from major β-thalassemia, who underwent open left adrenalectomy and the histopathology revealed EMH. Conclusions In patients in which a history of hematologic disorders exists, careful imaging and hormonal assay should be done to certify a diagnosis of EMH. However, the surgical management becomes inevitable in certain cases.

  3. [Extramedullary hematopoyesis: compensatory mechanism or clinic syndrome? Case report and review of literature].

    Science.gov (United States)

    Rosada, J; Bindi, M; Pinelli, M; Pandolfo, C; Cassetti, G; Castiglioni, M

    2007-02-01

    Extramedullary hematopoiesis (EMH) is a compensatory mechanism occurring in patients with chronic anemia. Liver, spleen, and lymph nodes are frequently involved. However, EMH may also develop in several sites such as thymus, kidneys, retroperitoneum, paravertebral areas of the thorax, lungs, bowel and others. Rarely symptomatic, it often shows a variety of clinical features. This condition, frequently, may be fatal. A correct early diagnosis of EHM might avoid, if possible, a bad prognosis. The Authors report a case where bone marrow cells were identified in centrifuge cerebrospinal fluid of a patient suffering from non-Hodgkin lymphoma.

  4. Incomplete paraplegia caused by extramedullary hematopoiesis in a patient with thalassemia intermedia.

    Science.gov (United States)

    Hisamud-Din, Nurhasyimah; Mustafah, Nadia Mohd; Fauzi, Aishah Ahmad; Hashim, Natiara Mohamad

    2017-01-01

    Extramedullary hematopoiesis (EMH) is the production of blood cell precursors outside the bone marrow that occur in various hematological diseases. In patients with thalassemia intermedia, ineffective erythropoiesis drives compensatory EMH in the liver, pancreas, pleura, spleen, ribs and spine. We describe a patient with thalassemia intermedia who presented with acute neurological symptoms caused by paraspinal EMH, which responded well to combination therapy of steroid, hypertransfusion, laminectomy and excision of pseudotumor and hydroxyurea therapy to boost the formation of fetal haemoglobin. Prompt recognition of EMH based on clinical presentation and typical radiological findings should be made. Early treatment is recommended to prevent irreversible damage to the spinal cord.

  5. Extramedullary hematopoiesis within the clivus: an unusual cause of lower cranial nerve palsy.

    Science.gov (United States)

    Reames, Davis L; Lindstrom, Katherine; Raghavan, Prashant; Jane, John

    2010-12-01

    We report one year follow up of a case of extramedullary hematopoiesis within the clivus. The imaging findings, brief clinical course, and endoscopic transphenoidal approach are described. A 29-year-old female with thalassemia developed worsening cranial nerve signs. After imaging studies discovered a large clival mass, she underwent endoscopic transphenoidal biopsy of the lesion. Neural compression from exuberant erythrogenesis within tissue normally quiescent of red blood cell production was found to be the etiology of her neural deficit. Treatment for this condition is generally non-operative unless significant neural compression is present. Radiotherapy and anti-neoplastic agents have been used with success.

  6. Extramedullary hematopoiesis of the conjunctiva presenting as active systemic disease in a patient with myelofibrosis.

    Science.gov (United States)

    Cuttler, Nirupa; Heidemann, David; Cendrowski, Christopher; Armin, Ali-Reza; Folberg, Robert

    2014-12-01

    To report the clinicopathological correlation of extramedullary hematopoiesis (EMH) of the conjunctiva in a patient with a history of myelofibrosis. Case report. Elevated pink conjunctival lesions developed bilaterally in a 73-year-old man who had been treated for myelofibrosis for 13 years. EMH was detected in the examination of tissue from the lesion of the inferior fornix of the right eye. The appearance of conjunctival lesions in a patient with myelofibrosis may indicate underlying pathology of EMH that may necessitate a change in systemic treatment of this condition.

  7. Intracranial involvement in extramedullary hematopoiesis: case report and review of the literature

    Energy Technology Data Exchange (ETDEWEB)

    Haidar, Salwa; Ortiz-Neira, Clara; Shroff, Manohar; Gilday, David; Blaser, Susan [Hospital for Sick Children, Department of Diagnostic Imaging, Toronto, ON (Canada)

    2005-06-01

    Intracranial involvement in extramedullary hematopoiesis (EMH) is rare, but it should be suspected in patients with myelofibrosis presenting with chronic severe headache. We present a 9-year-old girl with known myelofibrosis whose headaches were unresponsive to routine treatment. CT and MRI studies of the brain showed diffuse pachymeningeal thickening. CT examinations of the chest and abdomen had demonstrated bilateral thoracic paraspinal masses caused by EMH, suggesting the possibility that the intracranial involvement might also be related to EMH. The diagnosis was confirmed by sulfur colloid isotope scan. (orig.)

  8. Splenomegaly unresponsive to standard and salvage chemotherapy in Langerhans cell histiocytosis: a case of extramedullary hematopoiesis.

    Science.gov (United States)

    Christiansen, Ellen C; Ellwein, Marcine; Neglia, Joseph P

    2012-06-01

    Langerhans cell histiocytosis (LCH) is a proliferative disorder of dendritic cells which has evaded efforts to clearly define pathogenesis, diagnostic criteria, and therapeutic response markers. Strides have been made at classification with the recent development of a quantified score of disease severity. Splenic involvement is an indicator of poor prognosis, with spleen size its surrogate marker in evaluation and scoring. We describe a case of pediatric LCH with progressive splenomegaly despite treatment, which when examined at splenectomy revealed no LCH involvement but extramedullary hematopoiesis. These findings thus challenge our understanding of splenomegaly as a marker of disease. Copyright © 2011 Wiley Periodicals, Inc.

  9. Intracranial involvement in extramedullary hematopoiesis: case report and review of the literature

    International Nuclear Information System (INIS)

    Haidar, Salwa; Ortiz-Neira, Clara; Shroff, Manohar; Gilday, David; Blaser, Susan

    2005-01-01

    Intracranial involvement in extramedullary hematopoiesis (EMH) is rare, but it should be suspected in patients with myelofibrosis presenting with chronic severe headache. We present a 9-year-old girl with known myelofibrosis whose headaches were unresponsive to routine treatment. CT and MRI studies of the brain showed diffuse pachymeningeal thickening. CT examinations of the chest and abdomen had demonstrated bilateral thoracic paraspinal masses caused by EMH, suggesting the possibility that the intracranial involvement might also be related to EMH. The diagnosis was confirmed by sulfur colloid isotope scan. (orig.)

  10. Paraplegia due to extramedullary hematopoiesis in thalassemia treated successfully with radiation therapy.

    Science.gov (United States)

    Malik, Monica; Pillai, Lakshmi S; Gogia, Nidhi; Puri, Tarun; Mahapatra, M; Sharma, Daya Nand; Kumar, Rajat

    2007-03-01

    Spinal cord compression due to extramedullary hematopoiesis (EMH) is a rare complication of thalassemia and generally presents as paraparesis with sensory impairment. Complete paraplegia is extremely rare in EMH due to thalassemia although it is known to occur in polycythemia vera and sickle cell anemia. Treatment options mostly include surgery and/or radiotherapy. Whereas cases presenting with paraparesis have been treated with either surgery or radiotherapy with equal frequency and efficacy, almost all reported cases with paraplegia have been treated with surgery with or without radiation therapy. We hereby report a case of thalassemia intermedia with paraplegia treated successfully with radiotherapy.

  11. PET CT imaging in extramedullary hematopoiesis and lung cancer surprise in a case with thalassemia intermedia

    Directory of Open Access Journals (Sweden)

    Semra Paydaş

    2011-03-01

    Full Text Available Extramedullary hematopoiesis (EMH is the production of hematopoietic precursors outside the bone marrow cavity, and it causes mass effects according to its localization. Magnetic resonance imaging (MRI and/or computed tomography (CT scans are used most commonly to detect EMH foci. We report herein a case with thalassemia intermedia causing paravertebral mass associated with EMH detected by CT scan. We further evaluated the case with positron emission tomography (PET CT, and lung cancer, which was not revealed in the CT scan, was detected coincidentally.

  12. Spontaneous cervical intradural disc herniation presenting with Brown-Séquard and Horner's syndrome: lesson learned from a very unique case.

    Science.gov (United States)

    Baudracco, Irene; Grahovac, Gordan; Russo, Vittorio M

    2017-05-01

    Cervical spontaneous intradural disc herniation (IDH) is an extremely rare condition. We describe a unique case of a patient presenting with a Brown-Séquard syndrome (BSS) and Horner's syndrome (HS). This study aimed to report an unusual case of spontaneous cervical intradural disc herniation that presented with Horner's and Brown-Séquard syndrome (BSS) and discuss difficulties in preoperative diagnosis and treatment difficulties of intradural cervical disc. Notes and images review, and analysis of the relevant literature. A 45-year old female presented with acute Horner's syndrome and Brown-Séquard syndrome. The magnetic resonance imaging of cervical spine revealed C4-5 disc extrusion with cord compression. The patient underwent urgent decompression through an anterior cervical corpectomy and fusion. Patient fully recovered 6 months after disease onset. We would like to emphasize that prompt and anterior cervical decompression is the treatment of choice, as it directly address the problem and allows dura repair in spontaneous cervical disc herniation.

  13. [Gastric schwannoma: rare differenzial diagnosis of acute upper gastrointestinal (GI) bleeding].

    Science.gov (United States)

    Lyros, Orestis; Schickel, Stephan; Schierle, Katrin; Hoffmeister, Albrecht; Gockel, Ines

    2017-08-01

    Schwannomas are benign tumors derived from Schwann cells and their typical site of origin is the subcutaneous tissue of the extremities. Gastrointestinal localization of Schwannomas is extremely rare and the stomach is the prevalent site. Gastric schwannomas primarily occur in the gastric submucosa and are usually asymptomatic.We present a rare case of a solitary gastric schwannoma in a 51-year old male, which initially manifested with hematemesis by acute upper gastrointestinal (GI) bleeding. The upper GI-Endoscopy revealed a gastric submucosal tumor, 7 cm in size, located in the proximal corpus and fundus. In the endoscopical Ultrasound (EUS-Examination), the lesion appeared to arise from the fourth proper muscle layer (Muscularis propria). The fourth layer origin and the isoechogenicity, as compared to the normal muscle layer, are endoscopic ultrasonographic characteristics of gastric schwannomas and help in distinguishing them from gastrointestinal tumors (GIST). Because of the unclear histological identity, the patient underwent a "rendezvous" endoscopic-laparoscopic surgical resection of the tumor in toto. The histomorphological features of the lesion and the strong expression of S100 in combination with absence of DOG1 expression indicated the diagnosis of gastric schwannoma. There was no evidence of malignancy. The postoperative course was uncomplicated.This is a very rare manifestation of gastric schwannoma, representing a rare differenzial diagnosis in a case of acute upper GI-Bleeding. Only 14 % of gastric schwanommas are presented with gastrointestinal bleeding, including mainly melena rather than hematemesis. This case is considered to be worthy of presentation owing to the rare and unusual cause of upper GI bleeding implied in it. © Georg Thieme Verlag KG Stuttgart · New York.

  14. Long-term socio-economic impact of vestibular schwannoma for patients under observation and after surgery

    DEFF Research Database (Denmark)

    Tos, Tina; Caye-Thomasen, Per; Stangerup, Sven-Eric

    2003-01-01

    This study describes and compares the long-term socio-economic impact for patients diagnosed with a vestibular schwannoma and either operated on or observed. A consecutive sample of patients diagnosed with vestibular schwannoma in Denmark and either operated on (748 patients) or observed by the w...

  15. Gastric Schwannoma mimicking malignant gastrointestinal stromal tumor and misdiagnosed by (18)F-FDG PET/CT.

    Science.gov (United States)

    Zhang, Yiqiu; Li, Beilei; Cai, Liang; Hou, Xiaoguang; Shi, Hongcheng; Hou, Jun

    2015-01-01

    Gastric Schwannoma is a rare benign mesenchymal tumor that accounts for only 0.2% of all gastric tumors. The current study presents a case of gastric Schwannoma misdiagnosed as malignant gastrointestinal stromal tumor (GIST) by esophagogastroduodenoscopy, endoscopic ultrasonography, and contrast-enhanced or not enhanced and (18)F-FDG PET/CT imaging.

  16. Naso-ethmoid schwannoma with intracranial extension: case report Schwannoma naso-etmoidal com extensão intracraniana: relato de caso

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    Mario G. Siqueira

    2001-06-01

    Full Text Available Intranasal schwannomas are rare lesions, specially when they present with an intracranial extension. The fifth case in the medical literature of a naso-ethmoid schwannoma with extension into the anterior cranial fossa is presented. The magnetic resonance findings and the details of the combined intracranial / transfacial operative approach used are described. The possible origin and the clinical characteristics of this rare lesion are reviewed.Schwannomas intranasais são lesões raras, principalmente quando apresentam um extensão intracraniana. Estamos apresentando o quinto caso da literatura médica de um schwannoma naso-etmoidal com extensão para o interior da fossa craniana anterior. São descritos os achados da ressonância magnética e os detalhes da via de acesso cirúrgico combinada intracraniana/transfacial. A possível origem e as características clínicas dessa lesaõ rara são revistas.

  17. Clinical diagnosis and treatment outcomes for parapharyngeal space schwannomas: A single-institution review of 21 cases.

    Science.gov (United States)

    Sato, Yoichiro; Imanishi, Yorihisa; Tomita, Toshiki; Ozawa, Hiroyuki; Sakamoto, Koji; Fujii, Ryoichi; Shigetomi, Seiji; Habu, Noboru; Otsuka, Kuninori; Watanabe, Yoshihiro; Sekimizu, Mariko; Ogawa, Kaoru

    2018-03-01

    Because the incidence of schwannoma arising from the parapharyngeal space (PPS) is very low, no studies have analyzed extirpation methods and postoperative neurological complications exclusively in PPS schwannomas. The preoperative diagnosis and clinical outcomes of surgical treatment in 21 patients with PPS schwannoma who underwent surgery were investigated. Neurological deficit of the involved nerve developed in all patients regardless of the extirpation method used. However, the incidence of first bite syndrome in sympathetic chain schwannoma was significantly lower after intracapsular enucleation (40%) than after total resection (100%; P = .045). Furthermore, the incidence of postoperative complications unrelated to the involved nerve was lower after intracapsular enucleation (0%) than after total resection (42.9%; P = .055). Although postoperative neurological deficit of the involved nerve was unavoidable in PPS schwannoma, intracapsular enucleation could be beneficial by reducing its severity and the incidence of complications unrelated to the involved nerve. © 2017 Wiley Periodicals, Inc.

  18. Long-term mobile phone use and the risk of vestibular schwannoma: a Danish nationwide cohort study

    DEFF Research Database (Denmark)

    Schüz, Joachim; Steding-Jessen, Marianne; Hansen, Søren

    2011-01-01

    Vestibular schwannomas grow in the region within the brain where most of the energy by radiofrequency electromagnetic fields from using mobile phones is absorbed. The authors used 2 Danish nationwide cohort studies, one a study of all adult Danes subscribing for a mobile phone in 1995 or earlier...... and one on sociodemographic factors and cancer risk, and followed subjects included in both cohorts for occurrence of vestibular schwannoma up to 2006 inclusively. In this study including 2.9 million subjects, a long-term mobile phone subscription of =11 years was not related to an increased vestibular...... reported holding their mobile phone to the right ear. Vestibular schwannomas in long-term male subscribers were not of larger size than expected. Overall, no evidence was found that mobile phone use is related to the risk of vestibular schwannoma. Because of the usually slow growth of vestibular schwannoma...

  19. Long-term mobile phone use and the risk of vestibular schwannoma: a Danish nationwide cohort study

    DEFF Research Database (Denmark)

    Schüz, Joachim; Steding-Jessen, Marianne; Hansen, Søren

    2011-01-01

    Vestibular schwannomas grow in the region within the brain where most of the energy by radiofrequency electromagnetic fields from using mobile phones is absorbed. The authors used 2 Danish nationwide cohort studies, one a study of all adult Danes subscribing for a mobile phone in 1995 or earlier...... and one on sociodemographic factors and cancer risk, and followed subjects included in both cohorts for occurrence of vestibular schwannoma up to 2006 inclusively. In this study including 2.9 million subjects, a long-term mobile phone subscription of ≥11 years was not related to an increased vestibular...... reported holding their mobile phone to the right ear. Vestibular schwannomas in long-term male subscribers were not of larger size than expected. Overall, no evidence was found that mobile phone use is related to the risk of vestibular schwannoma. Because of the usually slow growth of vestibular schwannoma...

  20. DNMT3A mutation leads to leukemic extramedullary infiltration mediated by TWIST1

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    Jie Xu

    2016-10-01

    Full Text Available Abstract Background DNMT3A mutations are frequently discovered in acute myeloid leukemia (AML, associated with poor outcome. Recently, a relapse case report of AML extramedullary disease has showed that AML cells harboring DNMT3A variation were detected in the cerebral spinal fluid. However, whether a causal relationship exists between DNMT3A mutation (D3Amut and extramedullary infiltration (EMI is unclear. Methods We took advantage of DNMT3A (R882C mutation-carrying AML cell strain, that is, OCI-AML3, assessing its migration ability in vitro and in vivo. By RNA interfering technology and a xenograft mouse model, we evaluated the effect of DNMT3A mutation on cell mobility and explored the possible mechanism. Results OCI-AML3 displayed extraordinary migration ability in vitro and infiltrated into meninges of NOD/SCID mice after intravenous transfusion. We found that this leukemic migration or infiltration capacity was significantly compromised by the knockdown of DNMT3A mutant. Notably, TWIST1, a critical inducer of epithelial–mesenchymal transition, which underlies the metastasis of carcinomas, was highly expressed in association with R882 mutations. Abrogation of TWIST1 in DNMT3A mutated cells considerably weakened their mobility or infiltration. Conclusions Our results demonstrate that D3Amut in OCI-AML3 strain enhances leukemic aggressiveness by promoting EMI process, which is partially through upregulating TWIST1.

  1. Extramedullary hematopoiesis: Elucidating the function of the hematopoietic stem cell niche (Review).

    Science.gov (United States)

    Yamamoto, Kouhei; Miwa, Yukako; Abe-Suzuki, Shiho; Abe, Shinya; Kirimura, Susumu; Onishi, Iichiroh; Kitagawa, Masanobu; Kurata, Morito

    2016-01-01

    Extramedullary hematopoiesis (EMH) occurs under various circumstances, including during embryonic/developmental periods, pathological status secondary to insufficient bone marrow function or ineffective hematopoiesis, in hematological disorders, for example malignancies, as well as stromal disorders of the bone. EMH is characterized by hematopoietic cell accumulations in multiple body locations. Common EMH locations observed in clinical and pathological practice include the spleen, liver, lymph nodes and para‑vertebral regions. Among the various organs associated with EMH, the spleen offers a unique site for evaluation of hematopoietic stem cell (HSC)/niche interactions, as this organ is one of the most common sites of EMH. However, the spleen does not have a major role in embryonic/developmental hematopoiesis. A recent study by our group revealed that circulating HSCs may be trapped by chemokine (C‑X‑C motif) ligand 12 (CXCL12)‑positive cells at the margin of sinuses near CXCL12‑positive endothelial cells, resulting in the initiation of the first step of EMH, which is a similar mechanism to bone marrow hematopoiesis. The present review briefly discusses the environment of EMH in extramedullary spaces in order to investigate the mechanisms underlying HSC maintenance, and aid the elucidation of the niche‑stem cell interactions that occur in the bone marrow.

  2. Extramedullary Acute Myeloid Leukemia (AML: Leukemic Pleural Effusion, Case Report and Review of the Literature

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    Naveen ePemmaraju

    2014-06-01

    Full Text Available Objective and Importance: Malignant pleural effusions occur in the setting of both solid and hematologic malignancies. Pleural effusion caused by leukemic infiltration is an unusual extramedullary manifestation of acute myeloid leukemia (AML with fewer than 20 cases reported.1-11 We report a case of pericardial and pleural effusions in a patient with AML and review the literature. Clinical presentation: In this case, a 55 year old man with previous history of myeloproliferative neoplasm (MPN experienced transformation AML, heralded by appearance of leukemic pleural effusions. The patient was identified to have leukemic pleural effusion based upon extended cytogenetic analysis of the pleural fluid, as morphologic analysis alone was insufficient. Intervention: The patient was treated with hypomethylator-based and intensive chemotherapy strategies, both of which maintained resolution of the effusions in the remission setting. Conclusion: Due to the rarity of diagnosis of leukemic pleural effusions, both cytogenetic and fluorescence in situ hybridization (FISH testing are recommended. Futhermore, systemic chemotherapy directed at the AML can lead to complete resolution of leukemic pleural effusions. Objective and ImportancePleural effusion caused by leukemic infiltration is an unusual extramedullary manifestation of acute myeloid leukemia (AML, but may be more common than previously thought. Fewer than 20 cases have been reported.1-11 We report a case of pericardial and pleural effusions in a patient with AML and review the literature.

  3. Spinal cord compression secondary to extramedullary hematopoiesis: A rareness in a young adult with thalassemia major.

    Science.gov (United States)

    Fareed, Shehab; Soliman, Ashraf T; De Sanctis, Vincenzo; Kohla, Samah; Soliman, Dina; Khirfan, Diala; Tambuerello, Adriana; Talaat, Mohamed; Nashwan, Abdulqadir; Caparrotti, Palmira; Yassin, Mohamed A

    2017-08-23

    We report a case of a thalassemia major male patient with back pain associated to severe weakness in lower extremities resulting in the ability to ambulate only with assistance. An urgent magnetic resonance imaging (MRI) of  thoracic and lumbosacral spine was requested. A posterior intraspinal extradural mass lesion compressing the spinal cord at the level of thoracic T5-8 was present, suggesting an extramedullary hematopoietic centre, compressing the spinal cord. He was treated successfully with thalassemia major alone. The patient was treated with blood transfusion, dexamethasone, morphine and paracetamol, followed by radiotherapy in 10 fractions to the spine (daily fraction of 2Gy from T3 to T9, total dose 20 Gy). His pain and neurologic examination quickly improved. A new MRI of the spine, one week after radiotherapy, showed an improvement of the extramedullary hematopoietic mass compression. In conclusion, EMH should be considered in every patient with ineffective erythropoiesis and spinal cord symptoms. MRI is the most effective method of demonstrating EMH. The rapid recognition and treatment can dramatically alleviate symptoms. There is still considerable controversy regarding indications, benefits, and risks of each of modality of treatment due to the infrequency of this disorder.

  4. Intracranial extramedullary hematopoiesis in patients with thalassemia: a case report and review of the literature.

    Science.gov (United States)

    Eskazan, Ahmet Emre; Ar, Muhlis Cem; Baslar, Zafer

    2012-08-01

    Extramedullary hematopoiesis (EH) is a compensatory phenomenon that results in the production of blood cell precursors outside the marrow in patients with chronic hemolytic anemia and ineffective erythropoiesis. EH usually involves the liver, spleen, and lymph nodes. It can also be found at paravertebral, intrathoracic, or pelvic locations. Intracranial EH is a rare entity and often asymptomatic but can sometimes lead to symptomatic tumor-like masses. Treatment options are controversial and include hypertransfusion, surgical excision, radiotherapy, and hydroxyurea (HU). Successful treatment of an intracranial EH mass with HU and blood transfusions in a beta-thalassemia major patient was discussed along with a review of the published literature on intracranial EH in thalassemia. In our patient, the extramedullary hematopoietic mass in the interhemispheric fissure showed a marked improvement after 6 months of HU and hypertransfusion therapy. In the English literature, there are a few cases with intracranial EH and thalassemia, which were treated with different treatment modalities, with different outcomes. There is no standard treatment approach in patients with symptomatic EH. HU with hypertransfusion regimen is a reasonable first-choice modality in treating intracranial EH masses. © 2012 American Association of Blood Banks.

  5. Extramedullary Plasmacytoma Diagnosed in an HIV-Positive Patient by an Unusual Clinical Presentation

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    Paulo de Camargo Moraes

    2016-01-01

    Full Text Available The aim of this paper is to describe a case report of EMP in an HIV-positive patient. A 44-year-old, dark-skinned HIV-infected woman was referred to the Oral Diseases Treatment Center with a swelling at palate and left gingival fornix in the maxilla. Biopsy was taken and the oral lesion was diagnosed as EMP with well-differentiated plasma cells and restriction of the lambda light-chain. Skeletal survey was performed and no radiograph alterations were observed, thus supporting the diagnosis of EMP. Patient was referred to treatment and after two months of chemo and radiotherapy, an expanding lesion was observed in L5/S1 patient’s vertebrae. Biopsy of the spinal lesion was consistent with lymphoma with plasmocitary differentiation, supporting the diagnosis of multiple myeloma (MM. Regarding the medical history, the final diagnostic was an oral extramedullary plasmacytoma with rapid progression into multiple myeloma. It is crucial to emphasize the relevance of HIV infection as a risk factor for both aggressive clinical behavior and unusual clinical presentation of extramedullary plasmacytoma cases.

  6. Laryngeal schwannoma excised under a microlaryngoscope without tracheotomy: A case report

    Science.gov (United States)

    WANG, BAOXIN; DONG, PIN; SHEN, BIN; XU, HONGMING; ZHENG, JIN

    2014-01-01

    Schwannomas are benign encapsulated tumors arising from Schwann cells in the peripheral nervous system. Between 25 and 45% of schwannomas occur in the head and neck region. Schwannomas of the larynx are extremely rare with an incidence of 0.1–1.5% in all benign laryngeal tumors. Laryngeal schwannomas usually occur in females aged between their 4th and 5th decades. The most successful curative method is surgical resection. When selecting the surgical method, the size and location of the tumor, as well as the requests of the patient, should be considered. The present case report describes a 29-year-old female patient with symptoms of hoarseness and dyspnea on exertion. Through endoscopic biopsy, histopathology revealed a schwannoma. Considering the symptoms, age and preferences of the patient, the method of trans-oral microlaryngoscopic excision without tracheotomy was used to excise the tumor located in the aryepiglottic fold. Results from a short-term follow-up showed the postoperative result to be satisfactory. PMID:24669270

  7. A neurofibromatosis type 2 case with vestibular, trigeminal and facial schwannomas together: magnetic resonance imaging findings

    International Nuclear Information System (INIS)

    Akay, S.; Hamcan, S.; Kara, K.; Battal, B.; Tasar, M.

    2012-01-01

    Full text: Introduction: Neurofibromatosis type 2 (NF2) is characterized by the development of multiple nervous system tumors. This disorder is also called multiple inherited schwannomas (MIS), meningiomas (M), and ependymomas (E) (MISME) syndrome. Objectives and tasks: To discuss the magnetic resonance (MR) imaging findings of a NF2 case who has bilateral vestibular and trigeminal schwannomas, unilateral facial schwannoma, multiple meningiomas and cervical intramedullary spinal cord tumors. Materials and methods: A 23-year-old male patient complaining of tinnitus and imbalance for 3 years, came to Neck-Nose-Throat department of our hospital. After the physical examination, the patient was referred to our department for the further work up with MR imaging. Results: Brain MR imaging showed bilateral acoustic schwannoma which reach through the internal acoustic canals. Bilateral symmetric homogeneously enhanced masses were also detected in Meckel's caves. Similarly, one milimetric enhancing lesion was seen at the right facial nerve. Eight meningiomas in various locations were observed, as well. Additionally, two enhancing intramedullary well-defined small foci were detected in the proximal cervical spinal cord. Ependymomas or intraparanchimal schwannomas were primarily suspected. Conclusion: This case includes all the probable intracranial and spinal mass lesions which may be associated with NF2. Enhanced MR is very reliable imaging modality for the detailed evaluation of NF2 patients

  8. Expression of estrogen and progesterone receptors in vestibular schwannomas and their clinical significance

    Directory of Open Access Journals (Sweden)

    Pandey Rakesh

    2009-11-01

    Full Text Available Abstract Objective The objective was to determine the expression of estrogen and progesterone receptors in vestibular schwannomas as well as to determine predictive factors for estrogen and progesterone receptor positivity. Materials and methods The study included 100 cases of vestibular schwannomas operated from January 2006 to June 2009. The clinical details were noted from the medical case files. Formaldehyde-fixed parafiin-embedded archival vestibular schwannomas specimens were used for the immunohistochemical assessment of estrogen and progesterone receptors. Results Neither estrogen nor progesterone receptors could be detected in any of our cases by means of well known immunohistochemical method using well documented monoclonal antibodies. In the control specimens, a strongly positive reaction could be seen. Conclusion No estrogen and progesterone receptor could be found in any of our 100 cases of vestibular schwannomas. Hence our study does not support a causative role of estrogen and progesterone in the growth of vestibular schwannoma as well as hormonal manipulation in the treatment of this tumor.

  9. Clinicopathologic Features of Gastric Schwannoma: 8-Year Experience at a Single Institution in China.

    Science.gov (United States)

    Tao, Kaixiong; Chang, Weilong; Zhao, Ende; Deng, Rui; Gao, Jinbo; Cai, Kailin; Wang, Guobin; Zhang, Peng

    2015-11-01

    To explore the clinicopathologic characteristics, diagnosis, treatment, and prognosis of gastric schwannoma in the imatinib era.The clinicopathologic characteristics and postoperative outcomes of patients diagnosed with gastric schwannoma at our institution between January 2007 and February 2015 were retrospectively collected and analyzed.The main patient complaint was epigastric pain or discomfort. Tumor sizes ranged from 15 to 80 mm (mean, 57.1 mm). In 17 patients, the tumors were located in the body of the stomach. A total of 20 patients were preoperatively misdiagnosed with a gastrointestinal stromal tumor. The rate of correct preoperative diagnosis was only 3.3%. All patients underwent surgical resection and showed strong S-100 protein positivity. Laparoscopic surgery for gastric schwannoma was associated with less blood loss and a shorter postoperative hospital stay than open surgery (P Gastric schwannoma is often preoperatively misdiagnosed as gastric gastrointestinal stromal tumor. Laparoscopic resection of gastric schwannoma is considered safe and effective, and it may be the preferred surgery for most small- and moderate-sized tumors. The long-term outcome is excellent, as this type of neoplasm is uniformly benign.

  10. Emotional intelligence in association with quality of life in patients recently diagnosed with vestibular schwannoma.

    Science.gov (United States)

    van Leeuwen, Bibian M; Borst, Jacoba M; Putter, Hein; Jansen, Jeroen C; van der Mey, Andel G L; Kaptein, Adrian A

    2014-10-01

    The objective of this study was two-fold. First, to examine the levels of emotional intelligence in patients recently diagnosed with vestibular schwannoma, in comparison to those of healthy individuals and patients with other physical illness. Second, to evaluate the correlation between Emotional Intelligence and quality of life. Cross sectional study in a university tertiary referral center. Consecutive patients (mean age [range], 56.4 [17-85] yr) diagnosed with vestibular schwannoma between April 2011 and October 2012 (N = 254). Sociodemographic characteristics, clinical characteristics, disease-specific quality of life (PANQOL), and Emotional Intelligence (TEIQue-SF) were assessed by questionnaire before the start of medical treatment. Levels of Emotional Intelligence in patients with vestibular schwannoma (N = 178; response rate 70.1%) were significantly lower compared with healthy individuals and patients with cancer. Emotional Intelligence was highly positively correlated to disease-specific quality of life. Balance disorders and cranial nerve dysfunction made a significant negative contribution to the quality of life. For educational level, a significant positive contribution was found as well. The substantial impact of a vestibular schwannoma-diagnosis on a psychological measure (i.e., Emotional Intelligence) in the affected patients as demonstrated in our study has important clinical and research implications when developing guidelines about counselling of these patients. This also has to be taken into account when making clinical decisions about the proposed treatment. Addressing Emotional Intelligence may be helpful in the development of a self-management program for patients with vestibular schwannoma.

  11. Serial CT Findings of Resolving Extramedullary Hematopoiesis as Unilateral Posterior Mediastinal Mass after Splenectomy in Hereditary Spherocytosis: A Case Report

    International Nuclear Information System (INIS)

    Nam, Mi Yeon; Lee, Ju Won; Kim, Yeo Ju; Kim, Youn Jeong; Kang, Young Hye; Lee, Kyung Hee

    2012-01-01

    Intrathoracic extramedullary hematopoiesis (EMH) is a rare condition of the hereditary spherocytosis. EMH usually regresses or disappears after treatment; such as splenectomy in the case of spherocytosis. We report a case of hereditary spherocytosis. It is presented with an unilateral paravertebral posterior mediastinal mass. After splenectomy, it revealed shrinkage and fatty replacement on serial CT scans.

  12. Serial CT Findings of Resolving Extramedullary Hematopoiesis as Unilateral Posterior Mediastinal Mass after Splenectomy in Hereditary Spherocytosis: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Nam, Mi Yeon; Lee, Ju Won; Kim, Yeo Ju; Kim, Youn Jeong; Kang, Young Hye; Lee, Kyung Hee [Dept. of Radiology, Inha University Hospital, Incheon (Korea, Republic of)

    2012-03-15

    Intrathoracic extramedullary hematopoiesis (EMH) is a rare condition of the hereditary spherocytosis. EMH usually regresses or disappears after treatment; such as splenectomy in the case of spherocytosis. We report a case of hereditary spherocytosis. It is presented with an unilateral paravertebral posterior mediastinal mass. After splenectomy, it revealed shrinkage and fatty replacement on serial CT scans.

  13. Quantitative comparison of retrosigmoid intradural suprameatal approach and retrosigmoid transtentorial approach: implications for tumors in the petroclival region.

    Science.gov (United States)

    Ambekar, Sudheer; Amene, Chiazo; Sonig, Ashish; Guthikonda, Bharat; Nanda, Anil

    2013-10-01

    Background Retrosigmoid transtentorial (RTT) and retrosigmoid intradural suprameatal (RISA) approaches have been used in the treatment of petroclival tumors. Objective To compare the area of exposure of brainstem and petroclival region obtained through RTT and RISA in cadaveric specimens. Methods Five cadaveric specimens with a total of 10 sides were analyzed. RTT and RISA were performed on five sides each. Brainstem and petroclival surface exposure were measured using both the approaches. These values were compared between the two approaches. Results Brainstem area exposure with RTT was 441 ± 63 mm(2) and that with RISA was 311 ± 61 mm(2). Student's t-test revealed that the difference was significant (p = 0.01). The area of petroclival exposure medial to the Meckel cave through RTT was 696 ± 57 mm(2), and that through RISA was 716 ± 51 mm(2) (p = 0.69). The area of brainstem exposure between V and VII-VII complex through RTT and RISA was 387 ± 86 mm(2) and 378 ± 76 mm(2) (p = 0.87). Conclusion The RTT approach is an excellent approach to ventrolateral brainstem and petroclival region. It provides greater superoventral exposure of the ventrolateral brainstem than RISA and provides similar petroclival exposure.

  14. Solitary intramammary schwannoma mimicking phylloides tumor: Cytological clues in the diagnosis

    Directory of Open Access Journals (Sweden)

    M U Thejaswini

    2012-01-01

    Full Text Available A 30-year-old woman presented with a huge exophytic lobulated mass in the right breast, clinically and radiologically resembling phylloides tumor. Fine needle aspiration cytology smears showed clusters and singly scattered spindle cells along with structures reminiscent of verocay bodies. However, the epithelial elements were absent. A cytological diagnosis of benign mesenchymal spindle cell lesion, suggestive of schwannoma was rendered. Subsequent histopathological examination and strong S-100 positivity of tumor cells on immunohistochemistry confirmed the diagnosis.Breast is an uncommon location for benign schwannoma. The present case describes the unusual clinical picture, highlights cytological features and discusses the differential diagnoses of schwannoma from other benign spindle cells lesions of the breast on cytology smears.

  15. Evolution of vestibular schwannoma after removal of epidermoid cyst of the same location: case report.

    Science.gov (United States)

    Saito, Atsushi; Sugawara, Takayuki; Watanabe, Ryu; Akamatsu, Yousuke; Mikawa, Shigeki; Seki, Hirofumi

    2009-10-01

    A 71-year-old man presented with vestibular schwannoma manifesting as hearing disturbance and truncal ataxia 16 years after removal of a cerebellopontine angle (CPA) epidermoid cyst, and located adjacent to the remnant lesion. The patient first presented with a 6-month history of right trigeminal neuralgia. Neuroimaging demonstrated a right CPA lesion, suggestive of an epidermoid cyst. Right lateral suboccipital craniotomy was performed and the histological diagnosis was epidermoid cyst. A small lesion remained, but the symptoms were relieved. Sixteen years later, the patient presented with right auditory disturbance, vertigo, and truncal ataxia. Magnetic resonance imaging revealed a multiple cystic mass adjacent to the remnant epidermoid cyst in the right CPA. The lesion was removed and the histological diagnosis was vestibular schwannoma associated with the epidermoid cyst. The irritative effect of the remnant epidermoid cyst or surgical procedures may have caused the vestibular schwannoma, but no evidence of the evolution of the different types of tumors was found.

  16. Gastric Schwannoma Presenting as an Incidentaloma on CT-Scan and MRI.

    Science.gov (United States)

    Raber, Menno H; Ziedses des Plantes, Cathelijne M P; Vink, Robert; Klaase, Joost M

    2010-12-01

    A 67 year old female was referred because of an incidentaloma on CT-scan and MRI which showed a 5.0 cm large mass in the wall of the distal stomach. After an initial work-up which suggested a gastrointestinal stromal tumor (GIST), a partial gastrectomy with a Billroth II gastrojejunostomy was performed. The histological diagnosis was a schwannoma. Gastric schwannomas are rare tumors which comprise 0.2% of all gastric tumors and 4% of all benign gastric neoplasms with a peak of incidence in the 4th and 5th decade of life. Gastric schwannomas are usually asymptomatic, but can present with ulceration and/or gastrointestinal bleeding. Clinical, endoscopical, surgical, radiological and histological features of this case are described and the relevant literature is reviewed.

  17. Gastric malignant schwannoma presenting with upper gastrointestinal bleeding: a case report.

    Science.gov (United States)

    Takemura, Masashi; Yoshida, Kayo; Takii, Mamiko; Sakurai, Katsunobu; Kanazawa, Akishige

    2012-01-25

    We report a case of gastric malignant schwannoma presenting with gastrointestinal bleeding. A 70-year-old Japanese man presented with gastrointestinal bleeding to our hospital. Gastrointestinal endoscopy revealed a protruding lesion in the gastric body. Hematoxylin and eosin staining of biopsy specimens from this lesion revealed sheets of spindle cells. Immunohistochemistry revealed that these cells were positive for S-100 protein and negative for c-Kit and smooth muscle actin. Because mitosis was diffusely visible, this tumor was diagnosed as a gastric malignant schwannoma. Distal gastrectomy with lymph node dissection was performed and the patient's postoperative course was uneventful. However, five months after the surgery, he died from multiple liver metastases. Cases of gastric malignant schwannoma have rarely been reported. The efficacy of surgical resection and postoperative prognosis continues to remain unclear and should be investigated further.

  18. Gastric schwannoma presenting with perforation and abscess formation: a case report and literature review.

    Science.gov (United States)

    Euanorasetr, Chakrapan; Suwanthanma, Weerapat

    2011-11-01

    Gastric schwannoma represent only 0.2% of all gastric tumors and 4% of all benign gastric neoplasms. They are usually asymptomatic but can present with variable symptoms. The authors report a case of a 29-year-old male patient who presented with fever and abdominal pain with epigastric mass. Pre-operative diagnosis was gastric lymphoma with perforation and an abscess formation. Hemigastrectomy with Billroth II anastomosis was performed The pathologic examination and immunohistochemical studies confirmed gastric schawannoma as the diagnosis. The post-operative course was uneventful. Gastric schwannoma are difficult if not impossible to diagnose preoperatively as endoscopic and radiologic findings are nonspecific. The treatment of choice is complete surgical resection because of diagnostic uncertainty and the long-term outcome is excellent. This is the first report of gastric schwannoma presenting with concealed perforation and an abscess formation. The literature was reviewed.

  19. Cellular schwannoma arising from the gastric wall misdiagnosed as a gastric stromal tumor: A case report.

    Science.gov (United States)

    Wang, Guangyao; Chen, Ping; Zong, Liang; Shi, Lei; Zhao, Wei

    2014-02-01

    Cellular schwannomas have been previously described at almost every anatomic location of the human body, but reports in the gastric wall are rare. The current study presents a rare case of cellular schwannoma originating from the gastric wall. Computed tomography revealed a 5.6×5.3×4.0-cm 3 solid mass located in the posterior wall of the stomach. Open laparotomy confirmed its mesenchymal origin. Microscopically, the tissue was composed of spindle-shaped and fascicularly-arranged cells, but mitotic figures were rare. Immunohistochemical staining showed that the tumor was negative for cluster of differentiation (CD)117, CD34, smooth muscle actin and desmin, but positive for S-100 and Ki67. The patient presented no evidence of recurrence and metastasis during follow-up. Gastric cellular schwannomas may be diagnosed by clinical characteristics, histological observations and immunohistochemical markers.

  20. Small bud of probable gastrointestinal stromal tumor within a laparoscopically-resected gastric schwannoma.

    Science.gov (United States)

    Cho, Haruhiko; Watanabe, Takafumi; Aoyama, Toru; Hayashi, Tsutomu; Yamada, Takanobu; Ogata, Takashi; Yoshikawa, Takaki; Tsuburaya, Akira; Sekiguchi, Hironobu; Nakamura, Yoshiyasu; Sakuma, Yuji; Kameda, Yoichi; Miyagi, Yohei

    2012-06-01

    Submucosal tumors (SMTs) of the gastrointestinal (GI) tract can be potentially difficulty to diagnose pathologically. We report a case of a gastric SMT that was resected by laparoscopic partial gastrectomy. Although the initial histological and immunohistochemical examinations considered the tumor as a schwannoma, mRNA-based KIT genotyping indicated that the tumor included cells with KIT gene expression, and that a small number of cells carried a deletion mutation in exon 11. Additional histopathological investigations revealed small aggregates of enlarged spindle to epithelioid cells, which were positive for KIT, CD34 and DOG1, and negative for S-100, scattered among the S-100-positive schwannoma cells. We consider that the cells carrying the KIT gene mutation are microscopic buds of a gastrointestinal stroma tumor (GIST), and to the best of our knowledge, this is the first report of probable GIST tissues identified in a schwannoma. Our observations raised the significance of genotyping for diagnosis of GI tract SMTs.

  1. Gastric malignant schwannoma presenting with upper gastrointestinal bleeding: a case report

    Directory of Open Access Journals (Sweden)

    Takemura Masashi

    2012-01-01

    Full Text Available Abstract Introduction We report a case of gastric malignant schwannoma presenting with gastrointestinal bleeding. Case presentation A 70-year-old Japanese man presented with gastrointestinal bleeding to our hospital. Gastrointestinal endoscopy revealed a protruding lesion in the gastric body. Hematoxylin and eosin staining of biopsy specimens from this lesion revealed sheets of spindle cells. Immunohistochemistry revealed that these cells were positive for S-100 protein and negative for c-Kit and smooth muscle actin. Because mitosis was diffusely visible, this tumor was diagnosed as a gastric malignant schwannoma. Distal gastrectomy with lymph node dissection was performed and the patient's postoperative course was uneventful. However, five months after the surgery, he died from multiple liver metastases. Conclusion Cases of gastric malignant schwannoma have rarely been reported. The efficacy of surgical resection and postoperative prognosis continues to remain unclear and should be investigated further.

  2. Collet-Sicard Syndrome With Hypoglossal Nerve Schwannoma: A Case Report.

    Science.gov (United States)

    Lee, Seung Hun; Lee, Eun Shin; Yoon, Chul Ho; Shin, Heesuk; Lee, Chang Han

    2017-12-01

    Collet-Sicard syndrome is a rare syndrome that involves paralysis of 9th to 12th cranial nerves. We report an uncommon case of schwannoma of the hypoglossal nerve in a 39-year-old woman presented with slurred speech, hoarse voice, and swallowing difficulty. Physical examination revealed decreased gag reflex on the right side, decreased laryngeal elevation, tongue deviation to the right side, and weakness of right trapezius muscle. MRI revealed a mass lesion in the right parapharyngeal space below the jugular foramen. The tumor was surgically removed. It was confirmed as hypoglossal nerve schwannoma via pathologic examination. Videofluoroscopic swallowing study revealed aspiration of liquid food and severe bolus retention in the vallecula and piriform sinus. Laryngoscopy revealed right vocal cord palsy. Electrodiagnostic study revealed paralysis of the right 11th cranial nerve. In summary, we report an uncommon case of schwannoma of the hypoglossal nerve with 9th to 12th cranial nerve palsy presenting as Collet-Sicard syndrome.

  3. Clinical course of vestibular schwannoma in pediatric neurofibromatosis Type 2.

    Science.gov (United States)

    Choi, Jung Won; Lee, Ji Yeoun; Phi, Ji Hoon; Wang, Kyu-Chang; Chung, Hyun-Tai; Paek, Sun Ha; Kim, Dong Gyu; Park, Sung-Hye; Kim, Seung-Ki

    2014-06-01

    Neurofibromatosis Type 2 (NF2) is an autosomal-dominant inherited disease, characterized by multiple neoplasia syndromes, including meningioma, schwannoma, glioma, and ependymoma. In this report, the authors present their clinical experience with pediatric NF2 patients. In particular, they focused on the clinical course of vestibular schwannoma (VS), including the natural growth rate, tumor control, and functional hearing outcomes. From May 1988 to June 2012, the authors recruited patients who were younger than 18 years and fulfilled the Manchester criteria. In total, 25 patients were enrolled in this study. The authors analyzed the clinical course of these patients. In addition, they measured the natural growth rate of VS before any treatment in these children with NF2. Then, they evaluated the tumor control rate and functional hearing outcomes after the treatment of VS. The mean age at the onset of NF2-related symptoms was 9.9 ± 4.5 years (mean ± SD, range 1-17 years). The mean age at the diagnosis of NF2 was 12.9 ± 2.9 years (range 5-17 years). The mean follow-up period was 89.3 months (range 12-311 months). As initial manifestations, nonvestibular symptoms were frequently observed in pediatric patients with NF2. The mean natural growth rate of VS was 0.33 ± 0.41 cm(3)/year (range 0-1.35 cm(3)/year). The tumor control rate of VS was 35.3% at 3 years after Gamma Knife surgery (GKS). The actuarial rate of useful hearing preservation was 67% in the 1st year and 53% in the 5th year after GKS. Clinical manifestations in children with NF2 were highly variable, compared with their adult counterparts. The natural growth rate of VS in children is slow, and this oncological feature may explain the diverse clinical manifestations besides vestibular symptoms in children with NF2. The treatment outcome of GKS for VS in children with NF2 was not favorable compared with previous reports of affected adults.

  4. STATE ANXIETY, SUBJECTIVE IMBALANCE AND HANDICAP IN VESTIBULAR SCHWANNOMA

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    Yougan Saman

    2016-07-01

    Full Text Available ABSTRACTEvidence is emerging of a significant clinical and neuro-anatomical relationship between balance and anxiety. Research has suggested a potentially priming effect with anxiety symptoms predicting a worsening of balance function in patients with underlying balance dysfunction. We propose to show that a vestibular stimulus is responsible for an increase in state anxiety and there is a relationship between increased state anxiety and worsening balance function. Aims1.To quantify state anxiety following a vestibular stimulus in patients with a chronic vestibular deficit.2.To determine if state anxiety during a vestibular stimulus would correlate with the severity of chronic balance symptoms and handicap. MethodsTwo separate cohorts Vestibular Schwannoma (VS patients underwent vestibular tests (electronystagmography, cervical and ocular vestibular evoked myogenic potentials and caloric responses and questionnaire assessment (Vertigo handicap Questionnaire, Vertigo Symptom Scale, State Trait Anxiety InventoryFifteen post resection Vestibular schwannoma patients, with complete unilateral vestibular deafferentation, were assessed at a minimum of 6 months after surgery in Experiment 1 (Aim 1. Forty-five patients with VS in-situ and with preserved vestibular function formed the cohort for Experiment 2 (Aim 2. Experiment 1: VS subjects (N=15 with a complete post-resection unilateral vestibular deafferentation completed a State anxiety questionnaire before caloric assessment and again afterwards with the point of maximal vertigo as the reference (Aim 1. Experiment 2: State anxiety measured at the point of maximal vertigo following a caloric assessment was compared between two groups of presenting with balance symptoms (Group 1 N=26 and without balance symptoms (Group 2 N=11 (Aim 2. The presence of balance symptoms was defined as having a positive score on the VSS-VER.ResultsIn experiment 1, a significant difference (p<0.01 was found when comparing

  5. Schwannoma em pálpebra superior esquerda em criança de 10 anos

    Directory of Open Access Journals (Sweden)

    Marília de Sá Coutinho

    2014-04-01

    Full Text Available Schwannoma é um tumor neurogênico benigno raro, originário das células de Schwann da bainha de mielina dos nervos periféricos. Sua localização nos tecidos oculares não é comum, sendo a órbita o local afetado com maior frequência e o acometimento das pálpebras é muito raro. Há poucos relatos descritos na literatura sobre Schwannoma palpebral, apenas dois em crianças. Este é, em nosso conhecimento, o primeiro caso relatado no Brasil.

  6. Ancient Schwannoma of the Cauda Equina: Our Experience and Review of the Literature

    Science.gov (United States)

    Wierzbicki, Venceslao; Marrocco, Luigi; Piccione, Emanuele; Frati, Alessandro; Caruso, Riccardo

    2016-01-01

    Ancient schwannomas (AS) are exceedingly rare variant of common schwannomas (CS). Only two cases involving the cauda equina region have been previously reported in literature. AS are typically associated with a higher histological degree of degenerative changes (Antoni B areas). It is of peculiar importance, according to our opinion, to outline that, because of their extremely slow growth (which explains the increase of the degenerative changes in respect to the CS) and their typical soft consistency in respect to their standard counterparts, AS usually imply an even better prognosis. PMID:28101394

  7. Ectopic extramedullary hematopoiesis: evaluation and treatment of a rare and benign paraspinal/epidural tumor.

    Science.gov (United States)

    Mattei, Tobias A; Higgins, Michael; Joseph, Flynn; Mendel, Ehud

    2013-03-01

    Ectopic extramedullary hematopoiesis (EMH), defined as the formation of blood cells outside the bone marrow, usually occurs in a scenario of chronic anemia when, even after conversion of the bony yellow marrow to red marrow, the body is still unable to meet the demand for red blood cells. Ectopic extramedullary hematopoiesis most commonly occurs in the liver and spleen but may, in fact, occur almost anywhere in the body. Although previous reports have documented EMH presenting as paraspinal masses, such lesions have almost always been associated with a predisposing hematological disorder such as hemolytic anemia, myelofibrosis or myelodysplastic syndromes, thalassemia, polycythemia vera, leukemia, or lymphoma. The authors of this report describe the first reported instance of EMH in a patient presenting with a symptomatic epidural and paraspinal cervical lesion arising from the posterior spinal elements and no known predisposing hematological disease. Initial radiographs revealed a bony lesion arising posteriorly from the C2-3 laminae and spinous processes. Subsequent imaging suggested the diagnosis, which was confirmed by CT-guided biopsy, peripheral blood smears, and bone marrow aspirate. Despite epidural compression and slight displacement of the cervical cord and thecal sac, the patient's symptoms were limited to pain and diminished cervical range of motion. Therefore, surgery was deferred in favor of nonsurgical therapy. Several alternative modalities for the treatment of EMH have been suggested in the literature, including cytotoxic agents and radiotherapy. The authors opted for an approach utilizing directed low-dose radiotherapy of a total of 25 Gy divided in 2.5-Gy fractions. At the 3-month follow-up, the patient continued to be asymptomatic, and MRI demonstrated a significant reduction in the dimensions of the lesion. Extramedullary hematopoiesis with spinal cord compression in the absence of a preexisting hematological disorder has not been described in

  8. Transjugular Intrahepatic Porto-Systemic Stent-Shunt for Therapy of Bleeding Esophageal Varices Due to Extramedullary Hematopoiesis in Primary Myelofibrosis: A Case Report

    OpenAIRE

    Phillip, Veit;Berger, Hermann;Straub, Melanie;Saugel, Bernd;Treiber, Matthias;Einwächter, Henrik;Schmid, Roland M.;Huber, Wolfgang

    2016-01-01

    Background: Primary myelofibrosis belongs to the group of myeloproliferative syndromes. Extramedullary hematopoiesis in the liver can lead to portal hypertension. Patient and Methods: We report a case of a patient with life-threatening, endoscopically not treatable bleeding from esophageal varices due to extramedullary hematopoiesis of the liver that was successfully treated with placement of a transjugular intrahepatic porto-systemic stent-shunt (TIPS). Results: Therapy of variceal bleeding ...

  9. A giant plexiform schwannoma of the brachial plexus: case report

    Directory of Open Access Journals (Sweden)

    Kohyama Sho

    2011-11-01

    Full Text Available Abstract We report the case of a patient who noticed muscle weakness in his left arm 5 years earlier. On examination, a biloculate mass was observed in the left supraclavicular area, and Tinel's sign caused paresthesia in his left arm. Magnetic resonance imaging showed a continuous, multinodular, plexiform tumor from the left C5 to C7 nerve root along the course of the brachial plexus to the left brachia. Tumor excision was attempted. The median and musculocutaneous nerves were extremely enlarged by the tumor, which was approximately 40 cm in length, and showed no response to electric stimulation. We resected a part of the musculocutaneous nerve for biopsy and performed latissimus dorsi muscle transposition in order to repair elbow flexion. Morphologically, the tumor consisted of typical Antoni A areas, and immunohistochemistry revealed a Schwann cell origin of the tumor cells moreover, there was no sign of axon differentiation in the tumor. Therefore, the final diagnosis of plexiform Schwannoma was confirmed.

  10. Ruptured Pseudoaneurysm after Gamma Knife Surgery for Vestibular Schwannoma.

    Science.gov (United States)

    Murakami, Mamoru; Kawarabuki, Kentaro; Inoue, Yasuo; Ohta, Tsutomu

    2016-01-01

    Ruptured aneurysms of anterior inferior cerebellar artery (AICA) after radiotherapy for vestibular schwannoma (VS) are rare, and no definite treatment has been established for distal AICA pseudoaneurysms. We describe a 61-year-old man who underwent Gamma Knife surgery (GKS) for left VS. Follow-up magnetic resonance imaging (MRI) revealed partial regression of the tumor. Twelve years after GKS, he suffered from subarachnoid hemorrhage. Initial angiogram showed no vascular lesions; second left vertebral angiogram, 10 days after admission, demonstrated a pseudoaneurysm in the lateral pontine segment of the left AICA. The proximal portion of the AICA was occluded by a coil. Postoperative MRI revealed an infarction on the left side of the pons and brachium pontis. Although the patient suffered from mild postoperative cerebellar ataxia and facial and abducens nerve palsy, he was discharged 1 month postoperatively requiring no assistance with activities of daily living. Twelve months later, he recovered satisfactorily with a modified Rankin Scale grade of 1, and no recanalization of the aneurysm was found on MR angiography. Endovascular parent artery occlusion for ruptured aneurysms at distal AICA carries the risk of brain stem infarction, but should be considered when no other option is available such as after radiotherapy for VS.

  11. Association between vestibular schwannomas and mobile phone use.

    Science.gov (United States)

    Moon, In Seok; Kim, Bo Gyung; Kim, Jinna; Lee, Jong Dae; Lee, Won-Sang

    2014-01-01

    Vestibular schwannomas (VSs) grow in the region where the energy from mobile phone use is absorbed. We examined the associations of VSs with mobile phone use. This study included 119 patients who had undergone surgical tumor removal. We used two approaches in this investigation. First, a case-control study for the association of mobile phone use and incidence of VSs was conducted. Both cases and controls were investigated with questions based on INTERPHONE guidelines. Amount of mobile phone use according to duration, daily amount, and cumulative hours were compared between two groups. We also conducted a case-case study. The location and volume of the tumors were investigated by MRI. Associations between the estimated amount of mobile phone use and tumor volume and between the laterality of phone use and tumor location were analyzed. In a case-control study, the odds ratio (OR) of tumor incidence according to mobile phone use was 0.956. In the case-case study, tumor volume and estimated cumulative hours showed a strong correlation (r(2) = 0.144, p = 0.002), and regular mobile phone users showed tumors of a markedly larger volume than those of non-regular users (p mobile phones and tumor volume that showed strong correlation with amount of mobile phone use, thus there is a possibility that mobile phone use may affect tumor growth.

  12. Extramedullary relapse in acute promyelocytic leukemia treated with all-trans retinoic acid and chemotherapy.

    Science.gov (United States)

    de Botton, S; Sanz, M A; Chevret, S; Dombret, H; Martin, G; Thomas, X; Mediavilla, J D; Recher, C; Ades, L; Quesnel, B; Brault, P; Fey, M; Wandt, H; Machover, D; Guerci, A; Maloisel, F; Stoppa, A M; Rayon, C; Ribera, J M; Chomienne, C; Degos, L; Fenaux, P

    2006-01-01

    We analyzed the incidence, presenting features, risk factors of extramedullary (EM) relapse occurring in acute promyelocytic leukemia (APL) treated with all-trans retinoic acid (ATRA) and chemotherapy by using a competing-risk method. In total, 740/ 806 (92%) patients included in three multicenter trials (APL91, APL93 trials and PETHEMA 96) achieved CR, of whom 169 (23%) relapsed, including 10 EM relapses. Nine relapses involved the central nervous system (CNS) and one the skin, of which two were isolated EM relapse. In patients with EM disease, median WBC count was 26950/mm3 (7700-162000). The 3-year cumulative incidence of EM disease at first relapse was 5.0%. Univariate analysis identified age or = 10,000/ mm3) (P or = 10,000/mm3) and carries a poor prognosis. Whether CNS prophylaxis should be systematically performed in patients with WBC > or = 10,000/mm3 at diagnosis remains to be established.

  13. Intrathoracic extramedullary hematopoiesis: appearance on /sup 99m/Tc sulfur colloid marrow scan

    International Nuclear Information System (INIS)

    Bronn, L.J.; Paquelet, J.R.; Tetalman, M.R.

    1980-01-01

    Imaging of the bone marrow by radionuclide scanning was performed using colloids, which are phagocytized by the reticuloendothelial cells of the marrow, or radioiron, which is incorporated into reticulocytes. The use of the former radiopharmaceutical is based on the assumption, generally valid except in aplastic states or after irradiation, that the distribution of hematopoietic and reticuloendothelial tissue in the marrow is similar. Regardless of the method used, active adult marrow is normally distributed only in the axial skeleton and proximal humeri and femurs. Marrow imaging has been used in the evaluation of myeloproliferative disorders, leukemia, lymphoma, aplastic states, malignancy metastatic to marrow, and hemolytic anemia. We report a case of thalassemia major in which the diagnosis of intrathoracic extramedullary hematopoiesis was confirmed with the /sup 99m/Tc sulfur colloid bone marrow scan

  14. Cord Compression due to Extramedullary Hematopoiesis in an Adolescent with Known Beta Thalassemia Major

    Directory of Open Access Journals (Sweden)

    Alan COHLER

    2009-01-01

    Full Text Available We describe a 16 year-old male with ß thalassemia major and gait disturbances that had not been given blood transfusions due to a severe childhood transfusion reaction. Thoracic spine MRI demonstrated hematopoietic marrow throughout the spine and epidural masses causing cord compression consistent with extramedullary hematopoiesis (EMH. After treatment with steroids, radiotherapy and monitored blood transfusions, the patient demonstrated significant improvement of his paraspinal lesions and near complete resolution of his neurological symptoms. While EMH causing cord compression in adolescents is rare in the current era of bone marrow transplantation or chronic transfusions, it should be considered when thalassemia major patients present with neurological deficits. The well defined imaging features of EMH can play a central role in its diagnosis and management, especially because surgical and / or radiotherapeutic intervention are often considered in cases of failed medical treatment.

  15. Extramedullary hematopoiesis with spinal cord compression in a child with thalassemia intermedia.

    Science.gov (United States)

    Ileri, Talia; Azik, Fatih; Ertem, Mehmet; Uysal, Zumrut; Gozdasoglu, Sevgi

    2009-09-01

    Spinal cord compression due to extramedullary hematopoiesis is an extremely rare complication of thalassemia intermedia. No cases with this complication have been reported in the first decade of life, because masses of heterotropic marrow developed in patients as a result of continuous erythropoiesis. We report the 9-year-old patient suffering from thalassemia intermedia and presenting spinal cord compression. We also review the literature about treatment options, because there is no consensus about the optimal treatment of these patients. Our patient was successfully treated with radiation therapy followed by hydroxyurea. With this combination therapy, he had no recurrence during the 4-year follow-up period. Clinical awareness of this phenomenon with the early treatment is essential for optimizing the successful outcome.

  16. Maxillofacial extramedullary hematopoiesis in a child with sickle cell presenting as bilateral periorbital cellulitis.

    Science.gov (United States)

    Reiersen, David A; Mandava, Mamatha; Jeroudi, Majed; Gungor, Anil

    2014-07-01

    Review of a case of paraosseous extramedullary hematopoiesis (EMH) affecting the maxillary sinuses and retro-orbital spaces imitating bilateral orbital cellulitis. Maxillofacial EMH causes diagnostic/therapeutic challenges. This case report describes a 4-year-old African American male with sickle cell disease (HbSS) who presented with bilateral orbitofacial swelling. Diagnosis was made with imaging and confirmed with tissue sampling. Partial exchange transfusion was utilized to stop the progression of maxillofacial EMH and to treat the patient's chronic anemia. Follow-up MRI showed regression of orbital and retro-orbital involvement. Early treatment with conservative modalities and close observation may prevent need for more invasive treatments. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  17. A Rare Presentation of Extramedullary Hematopoiesis in Post-polycythemic Myelofibrosis.

    Science.gov (United States)

    Konca Degertekin, Ceyla; Ozkurt, Zübeyde Nur; Akyürek, Nalan; Yağcı, Münci

    2014-09-01

    Polycythemia vera is a clonal proliferative disorder of the bone marrow that could possibly evolve into myelofibrosis in its natural course. Progression to myelofibrosis is usually a late stage complication and presents clinically with refractory cytopenias and extramedullary hematopoiesis (EMH). EMH can occur in any tissue during the course of post-polycythemic myelofibrosis. However, skin and cardiac involvements seems to be very rare. We present a 56-year-old woman with post-polycythemic myelofibrosis refractory to treatment, developing EMH after splenectomy in various organs, exceptionally the skin and the heart. Along with the case, the clinical presentations, treatment options, prognostic significance of EMH and the role of cytogenetics is discussed in the light of the literature.

  18. [Spinal cord compression due to extramedullary hematopoiesis in a patient with myelofibrosis].

    Science.gov (United States)

    Hijikata, Yasuhiro; Ando, Tetsuo; Inagaki, Tomonori; Watanabe, Hirohisa; Ito, Mizuki; Sobue, Gen

    2014-01-01

    Development and growth of hematopoietic tissue outside of the bone marrow is termed extramedullary hematopoiesis (EMH). It occurs in patients with hematological diseases such as myelofibrosis and thalassemia. Liver and spleen are the usual sites of EMH. However, spinal cord compression caused by EMH is a rare complication. A 65-year-old man with myelofibrosis was admitted to our hospital with progressive paraparesis. Thoracic spine MRI revealed epidural masses causing cord compression. Histological examination of the epidural mass showed evidence of EMH consisting of megakaryocytic and erythroid hyperplasia. After surgical decompression and radiotherapy, lower limb weakness and sensory disturbance were significantly improved. MRI showed disappearance of the spinal cord compression. With this therapy, he had no recurrence until he died of myelofibrosis. Spinal EMH should be considered as a differential diagnosis in patients with hematological diseases presenting with paraparesis. Surgical decompression and radiotherapy are effective approaches for the treatment of paraparesis due to EMH.

  19. Extramedullary hematopoiesis and paraplegia in a patient with hemoglobin e-Beta thalassemia.

    Science.gov (United States)

    Alam, M R; Habib, M S; Dhakal, G P; Khan, M R; Rahim, M A; Chowdhury, A J; Mahmud, T K

    2010-07-01

    Extramedullary hematopoiesis (EMH) occurs in patients with various hematologic disorders involving a chronic increase in the production of red blood cells, and is often associated polycythemia vera and sickle cell anaemia, but is less common with thalassemia especially with hemoglobin E-beta thalassemia. Spinal cord compression due to EMH is a extremely rare complication of thalassemia and may present with paraparesis or paraplegia with or without sensory impairment. Treatment options mostly include surgery and/or radiotherapy. Whereas cases presenting with paraplegia have been treated with either surgery or radiotherapy with equal frequency and efficacy, almost all reported cases with paraplegia have been treated with surgery with or without radiation therapy. We hereby report a case of hemoglobin E-beta thalassemia with paraplegia treated successfully with radiotherapy.

  20. Cord Compression due to Extramedullary Hematopoiesis in an Adolescent with Known Beta Thalassemia Major

    Science.gov (United States)

    Soman, Salil; Rosenfeld, David L; Roychowdhury, Sudipta; Drachtman, Richard A; Cohler, Alan

    2009-01-01

    We describe a 16 year-old male with β thalassemia major and gait disturbances that had not been given blood transfusions due to a severe childhood transfusion reaction. Thoracic spine MRI demonstrated hematopoietic marrow throughout the spine and epidural masses causing cord compression consistent with extramedullary hematopoiesis (EMH). After treatment with steroids, radiotherapy and monitored blood transfusions, the patient demonstrated significant improvement of his paraspinal lesions and near complete resolution of his neurological symptoms. While EMH causing cord compression in adolescents is rare in the current era of bone marrow transplantation or chronic transfusions, it should be considered when thalassemia major patients present with neurological deficits. The well defined imaging features of EMH can play a central role in its diagnosis and management, especially because surgical and / or radiotherapeutic intervention are often considered in cases of failed medical treatment. PMID:22470615

  1. Flow-diverting stents allow efficient treatment of unruptured, intradural dissecting aneurysms of the vertebral artery: An explanatory approach using in vivo flow analysis.

    Science.gov (United States)

    Gölitz, Philipp; Struffert, Tobias; Hoelter, Philip; Eyüpoglu, Ilker; Knossalla, Frauke; Doerfler, Arnd

    2016-02-01

    Our study aimed to evaluate the efficiency of flow-diverting stents (FDS) in treating unruptured, intradural dissecting aneurysms of the vertebral artery (VADAs). Additionally, the effect of FDS on the aneurysmal flow pattern was investigated by performing in vivo flow analysis using parametric color coding (PCC). We evaluated 11 patients with unruptured, intradural VADAs, treated with FDS. Pre- and postinterventional DSA-series were postprocessed by PCC, and time-density curves were calculated. The parameters aneurysmal inflow-velocity, outflow-velocity and relative time-to-peak (rTTP) were calculated. Pre- and postinterventional values were compared and correlated with the occlusion rate after six months. Follow-up DSA detected 10 aneurysms occluded, meaning an occlusion rate of 91%. No procedure-related morbidity and mortality was found. Flow analyses revealed a significant reduction of aneurysmal inflow- velocity and prolongation of rTTP after FDS deployment. Concerning aneurysm occlusion, the postinterventional outflow-velocity turned out to be a marginally statistically significant predictor. A definite threshold value (-0.7 density change/s) could be determined for the outflow-velocity that allows prediction of complete aneurysm occlusion with high sensitivity and specificity (100%). Using FDS can be considered an efficient and safe therapy option in treating unruptured, intradural VADA. From in vivo flow analyses the postinterventional aneurysmal outflow-velocity turned out to be a potential predictor for later complete aneurysm occlusion. Here, it might be possible to determine a threshold value that allows prediction of aneurysm occlusion with high specificity and sensitivity. As fast, applicable and easy-to-handle tool, PCC could be used for procedural monitoring and might contribute to further treatment optimization. © The Author(s) 2015.

  2. Islets of Meningioma in an Acoustic Schwannoma in a Patient with Neurofibromatosis-2: Pathology and Magnetic Resonance Imaging Findings

    International Nuclear Information System (INIS)

    Gelal, F.; Rezanko, T.; Uyaroglu, M.A.; Tunakan, M.; Bezircioglu, H.

    2005-01-01

    Mixed tumors of the cerebellopontine angle, composed of meningioma and schwannoma components, are extremely rare; so far, only 12 cases have been reported in the literature. They are thought to be exclusively associated with neurofibromatosis-2. We present a mixed tumor of schwannoma and meningioma in a patient with neurofibromatosis-2 and discuss the pathology and magnetic resonance imaging (MRI) findings in relation to the literature. Review of the literature shows that a typical MRI pattern has not been established for mixed tumors and it seems unlikely that a meningioma component can be differentiated within a schwannoma preoperatively

  3. An Individual with Gastric Schwannoma with Pathologically Malignant Potential Surviving Two Years after Laparoscopy-Assisted Partial Gastrectomy

    Directory of Open Access Journals (Sweden)

    Akira Watanabe

    2011-08-01

    Full Text Available Schwannomas are a kind of neurogenic tumor. They are generally benign and originate primarily from the central and peripheral nerve. They rarely develop in the gastrointestinal tract: gastric schwannomas make up 0.2% of gastric neoplasms. A malignant gastric schwannoma is a comparatively rare tumor, a few cases have been reported until now. We present the case of a 34-year-old male patient diagnosed during medical examination. The patient was treated with surgical resection, and 2 years passed without recurrence.

  4. CXCR4-directed endoradiotherapy induces high response rates in extramedullary relapsed Multiple Myeloma.

    Science.gov (United States)

    Lapa, Constantin; Herrmann, Ken; Schirbel, Andreas; Hänscheid, Heribert; Lückerath, Katharina; Schottelius, Margret; Kircher, Malte; Werner, Rudolf A; Schreder, Martin; Samnick, Samuel; Kropf, Saskia; Knop, Stefan; Buck, Andreas K; Einsele, Hermann; Wester, Hans-Juergen; Kortüm, K Martin

    2017-01-01

    C-X-C-motif chemokine receptor 4 (CXCR4) is a key factor for tumor growth and metastasis in several types of human cancer. We have recently reported promising first-in-man experience with CXCR4-directed endoradiotherapy (ERT) in multiple myeloma (MM). Eight heavily pretreated MM patients underwent a total of 10 ERT cycles (7 patients with 1 cycle and a single patient with 3 cycles). ERT was administered in combination with chemotherapy and autologous stem cell support. End points were occurrence and timing of adverse events, progression-free and overall survival. ERT was overall well tolerated without any unexpected acute adverse events or changes in vital signs. With absorbed tumor doses >30-70 Gy in intra- or extramedullary lesions, significant anti-myeloma activity was observed with 1 patient achieving complete remission and 5/8 partial remission. Directly after ERT major infectious complications were seen in one patient who died from sepsis 22 days after ERT, another patient with high tumor burden experienced lethal tumor lysis syndrome. Median progression-free survival was 54 days (range, 13-175), median overall survival was 223 days (range, 13-313). During follow-up (6 patients available), one patient died from infectious complications, 2/8 from disease progression, the remaining 3/8 patients are still alive. CXCR4-directed ERT was well-tolerated and exerted anti-myeloma activity even at very advanced stage MM with presence of extramedullary disease. Further assessment of this novel treatment option is highly warranted.

  5. Value of SSTR2A and Claudin - 1 in Differentiating Meningioma from Schwannoma and Hemangiopericytoma.

    Science.gov (United States)

    Anis, Shady E; Lotfalla, Mira; Zain, Muhammad; Kamel, Nora N; Soliman, Ahmed A

    2018-02-15

    The distinction between meningioma, schwannoma and solitary fibrous tumour/ hemangiopericytoma can be challenging in some cases. This study evaluates the expression of Somatostatin receptor 2A (SSTR2A) and Claudin-1 in these different tumours. Thirty-five cases of meningioma, 10 cases of intracranial schwannoma and 10 cases of hemangiopericytoma were assessed. The immunohistochemical expression of SSTR2A and Claudin-1 was evaluated and scored according to the percentage of immunostained tumour cells (0: 1+, 2+ and 3). The intensity of staining was classified as weak, moderate and strong. Positivity for SSTR2A and Claudin-1 was encountered in 89% and 49% of meningiomas respectively. None of the schwannomas or hemangiopericytomas was positive for any of both markers. All grade I and II meningiomas were positive for SSTR2A, and only 20% of grade III showed positive staining (p < 0.05). Claudin-1 positivity was detected in 50%, 43% and 60% of grade I, II and III meningioma respectively, with significantly higher intensity in grade III (p < 0.05). SSTR2A is highly sensitive and specific for meningioma. Claudin-1 is highly specific for meningioma, with low sensitivity. The adjunctive use of both markers can be very helpful in the diagnosis of meningioma and its distinction from schwannoma and hemangiopericytoma.

  6. The Middle Fossa Approach for the Removal of a Trochlear Schwannoma

    Directory of Open Access Journals (Sweden)

    Andrew B. Boucher

    2014-01-01

    Full Text Available Objectives. Schwannomas originating from the trochlear nerve are extremely rare; only 30 cases have been reported in the literature. Many operative approaches have been utilized for lesion resection, but the advantages of the anterior transpetrosal approach are numerous and include excellent exposure, minimal extradural retraction of the temporal lobe, and minimal cerebrospinal fluid leaks. We report the second case of a trochlear schwannoma resected via the anterior transpetrosal approach. Setting. A 64-year-old male presented with 3-month history of diplopia and headaches. On physical examination, he was found to have a right fourth nerve palsy. Brain magnetic resonance imaging revealed a mass within the right ambient cistern compressing the adjacent midbrain. A right-sided anterior transpetrosal approach was used—which confirmed that the trochlear nerve entered the mass—to achieve gross total resection. Pathological examination confirmed diagnosis of schwannoma. The patient was discharged on postoperative day 3. He experienced a persistent fourth nerve palsy postoperatively with an otherwise normal neurological examination. Follow-up imaging confirmed complete removal of the tumor. Conclusion. The anterior transpetrosal approach is an excellent approach for removal of trochlear schwannomas involving the cisternal course of the trochlear nerve. It affords complete visualization of this anatomical region while introducing minimal morbidity.

  7. Residential traffic noise exposure and vestibular schwannoma - a Danish case-control study

    DEFF Research Database (Denmark)

    Roswall, Nina; Stangerup, Sven-Eric; Cayé-Thomasen, Per

    2017-01-01

    BACKGROUND: Few risk factors for sporadic vestibular schwannoma (VS) are known. Several studies have proposed an increased risk with occupational noise exposure, whereas no studies have investigated residential traffic noise exposure as a risk factor. The present study investigated if residential...

  8. Cervical Vestibular Evoked Myogenic Potential in Hypoglossal Nerve Schwannoma: A Case Report.

    Science.gov (United States)

    Rajasekaran, Aravind Kumar; Savardekar, Amey Rajan; Shivashankar, Nagaraja Rao

    2018-02-01

    Schwannoma of the hypoglossal nerve is rare. This case report documents an atypical abnormality of the cervical vestibular evoked myogenic potential (cVEMP) in a patient with schwannoma of the hypoglossal nerve. The observed abnormality was attributed to the proximity of the hypoglossal nerve to the spinal accessory nerve in the medullary cistern and base of the skull. To report cVEMP abnormality in a patient with hypoglossal nerve schwannoma and provide an anatomical correlation for this abnormality. Case report. A 44-yr-old woman. Pure-tone and speech audiometry, tympanometry, acoustic stapedial reflex, auditory brainstem response, and cVEMP testing were performed. The audiological test results were normal except for the absence of cVEMP on the lesion side (right). A cVEMP abnormality indicating a compromised spinal accessory nerve was observed in a patient with hypoglossal nerve schwannoma. This case report highlights the importance of recording cVEMP in relevant neurological conditions and provides clinical proof for the involvement of the spinal accessory nerve in the vestibulocollic reflex pathway. American Academy of Audiology

  9. Late Malignant Transformation of a Vestibular Schwannoma without Association to NFII or Radiotherapy

    DEFF Research Database (Denmark)

    Bashir, Asma; Poulsgaard, Lars; Broholm, Helle

    Late malignant transformation of vestibular schwannoma (VS) following irradiation has previously been reported 29 times in the literature. Here, the authors report the first late malignant transformation of VS unrelated to neurofibromatosis or radiation exposure. After undergoing a near-total exc...

  10. Late malignant transformation of vestibular schwannoma in the absence of irradiation

    DEFF Research Database (Denmark)

    Bashir, Asma; Poulsgaard, Lars; Broholm, Helle

    2016-01-01

    Late malignant transformation of vestibular schwannoma (VS) following irradiation has previously been reported 29 times in the literature. Here, the authors report the first late malignant transformation of VS unrelated to neurofibromatosis or radiation exposure. After undergoing a near-total exc...

  11. Vestibular schwannoma and cell-phones. Results, limits and perspectives of clinical studies.

    Science.gov (United States)

    Mornet, E; Kania, R; Sauvaget, E; Herman, P; Tran Ba Huy, P

    2013-11-01

    The widespread development of cell-phones entails novel user exposure to electromagnetic fields. Health impact is a public health issue and a source of anxiety in the population. Some clinical studies reported an association between cell and cordless phone use and vestibular schwannoma; others found none. A systematic review was performed of all published clinical studies (cohort, registry, case-control and validation studies), with analysis of results, to determine the nature of the association and the level of evidence. Cohort studies were inconclusive due to short exposure durations and poor representativeness. Registry studies showed no correlation between evolution of cell-phone use and incidence of vestibular schwannoma. Case-control studies reported contradictory results, with methodological flaws. Only a small number of subjects were included in long-term studies (>10 years), and these failed to demonstrate any indisputable causal relationship. Exposure assessment methods were debatable, and long-term assessment was lacking. An on-going prospective study should determine any major effect of electromagnetic fields; schwannoma being a rare pathology, absence of association will be difficult to prove. No clinical association has been demonstrated between cell and cordless phone use and vestibular schwannoma. Existing studies are limited by their retrospective assessment of exposure. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

  12. Schwannoma extending from the umbilical region to the mid-thigh ...

    African Journals Online (AJOL)

    cystic degeneration, mimicking an ovarian carcinoma, was reported,[14] and there was one report of a presacral neurilemoma. [15]. One pelvic retroperitoneal neurilemoma arose in the fallopian tube,[16] and one malignant schwannoma arose in the cervix. [17] Yadav et al.[18] reported one case of neurilemoma located in ...

  13. Facial nerve function after vestibular schwannoma surgery following failed conservative management

    DEFF Research Database (Denmark)

    Kaltoft, Mikkel; Stangerup, Sven-Eric; Cayé-Thomasen, Per

    2012-01-01

    BACKGROUND:: As only a limited proportion of vestibular schwannomas display growth following diagnosis, an increasing number of patients are managed conservatively. Tumor growth during "wait and scan" may, however, necessitate surgery. In these cases, increased tumor size is likely to increase th...

  14. Two epithelioid malignant schwannomas in a patient with neurofibromatosis : Cytology, histology and DNA-flow-cytometry

    NARCIS (Netherlands)

    Molenaar, W M; Ladde, B E; Koops, H Schraffordt; Dam-Meiring, A

    Cytological, histological and DNA-ploidy findings of 2 epithelioid malignant schwannomas arising in a patient with von Recklinghausen's neurofibromatosis are described. In both primary tumors, i.e. in the thigh and in the thoracic wall, origin from a neurofibromatous nerve could be established.

  15. Schwannoma extending from the umbilical region to the mid-thigh ...

    African Journals Online (AJOL)

    They commonly arise from the cranial nerves as acoustic schwannomas and are extremely rare in the pelvis and retroperitoneal area (<0.5% of reported cases) unless they are combined with Von Recklinghausen disease (type 1 neurofibromatosis). We report the case of a 23-year-old woman with a mass extending from the ...

  16. Minimally Invasive Surgery for Intracochlear Schwannoma Removal and Simultaneous Cochlear Implantation

    Directory of Open Access Journals (Sweden)

    Bento, Ricardo Ferreira

    2016-04-01

    Full Text Available Introduction Hearing preservation has not yet been reported in patients undergoing resection of intracochlear schwannomas. This study describes a minimally invasive procedure for intracochlear schwannoma resection with simultaneous cochlear implantation that resulted in good hearing. Objective This study aims to describe a minimally invasive procedure for intracochlear schwannoma resection with simultaneous cochlear implantation. Data Synthesis The technique described in this study was developed for a 55-year-old male with a 20-year history of bilateral progressive hearing loss and tinnitus that had a mass in the left apical turn of the cochlea measuring 0.3 cm. Surgery accessed the apical turn of the cochlea. We performed mastoidectomy and posterior tympanotomy and removed incus and tensor tympani muscle to expose the cochlear apex. The tumor was identified and completely resected. After the cochlea was anatomically preserved, it was implanted with a straight electrode via round window insertion. The histopathological examination confirmed intracochlear schwannoma. Speech perception test revealed 100% speech recognition with closed sentences and the average audiometric threshold (500 to 2000 Hz was 23 dB. Conclusion Our technique led to rehabilitation of the patient and improved hearing without damaging the intracochlear structure, making it possible to perform CI in the same procedure with good results.

  17. Need for facial reanimation after operations for vestibular schwannoma: patients perspective

    DEFF Research Database (Denmark)

    Tos, Tina; Caye-Thomasen, Per; Stangerup, Sven-Eric

    2003-01-01

    A total of 779 patients operated on for vestibular schwannoma mostly by the translabyrinthine approach in Denmark during the period 1976-2000 answered a questionnaire about various postoperative consequences. In this paper we describe the patients' facial function evaluated by professionals one y...

  18. Pontine extension of a tentorial schwannoma without cranial nerve involvement: a case report

    Directory of Open Access Journals (Sweden)

    D'Urso Pietro

    2011-12-01

    Full Text Available Abstract Introduction Intracranial schwannomas unrelated to the cranial nerves are uncommon. We report a new case of tentorial schwannoma unrelated to the cranial nerves, with extension into the pons. A literature review with discussion of the most relevant pathogenetic aspects is also performed. Case presentation A 42-year-old Caucasian man was admitted with right-sided paresthesias and weakness of his upper and lower extremities. The neurological examination revealed right hemiparesis and hemi-hypoesthesia. A brain magnetic resonance imaging scan revealed a cerebellopontine lesion, arising from the left free edge of the tentorium, and extending into his pons. A piecemeal removal was performed through a retrosigmoid approach. The lesion was not found to be associated with any cranial nerves. The histological examination revealed a schwannoma Antoni type A. His postoperative course was uneventful. At one year follow-up, the patient was neurologically intact and the magnetic resonance imaging of his brain performed at that time showed complete removal without signs of recurrence. Conclusion Tentorial schwannomas are rare clinical entities. Knowledge of their clinical, radiological and anatomical characteristics is very important for the correct diagnosis and management.

  19. Focal extra-axial hemorrahagic mass with subdural hemorrhage secondare to extramedullary hematopoiesis in idiopathic myelodysplastic sindrome.

    Science.gov (United States)

    Di Ieva, A; Di Lieva, A; Aimar, E; Tancioni, F; Levi, D; Debernardi, A; Pisano, P; Rahal, D; Nozza, A; Magagnoli, M; Gaetani, P

    2007-03-01

    Idiopathic myelodysplastic syndrome is a disease characterized by a clonal stem cell disorder in which megacaryocitic and granulocytic lineages are mainly involved; extramedullary myeloid metaplasia is due to abnormal location of myeloid tissue in other organs than bone marrow. Rarely the central nervous system is involved. When it happens, it is typical to find masses around the brain and pachymeningeal thickening, but it is very rare to find it associated with subdural haemorrhage, as in the case we describe in the present article. Considering our case and the literature we can suggest that radiological images associated with the clinical history of the patient suggestive for extramedullary hematopoiesis can be sufficient for a correct diagnosis and for a radiotherapy treatment, demanding surgery in the case of diagnostic doubts, massive hemorrahages or neurological decifits caused by the focal lesions.

  20. Fifteen-year follow-up of a patient with beta thalassaemia and extramedullary haematopoietic tissue compressing the spinal cord

    Energy Technology Data Exchange (ETDEWEB)

    Niggemann, P.; Krings, T.; Thron, A. [Dept. of Neuroradiology, RWTH-Aachen Hosital (Germany); Hans, F. [Dept. of Neurosurgery, RWTH Aachen Hospital (Germany); 1

    2005-04-01

    A long-term follow-up of a patient with beta thalassaemia with intra- and extraspinal extramedullary haematopoietic tissue compressing the spinal cord is presented. Extramedullary haematopoietic nodules are a rare cause of spinal cord compression and should be included in the differential diagnosis, especially in patients from Mediterranean countries. Treatment with radiation therapy solely failed, giving rise to the need of surgical intervention. Surgical decompression of the spine and the removal of the culprit lesion compressing the spine were performed. Postinterventional radiation therapy was applied to the spine. A relapse had to be treated again by surgical means combined with postinterventional radiation therapy. A complete relief of the symptoms and control of the lesion could be obtained.

  1. Fifteen-year follow-up of a patient with beta thalassaemia and extramedullary haematopoietic tissue compressing the spinal cord

    International Nuclear Information System (INIS)

    Niggemann, P.; Krings, T.; Thron, A.; Hans, F.

    2005-01-01

    A long-term follow-up of a patient with beta thalassaemia with intra- and extraspinal extramedullary haematopoietic tissue compressing the spinal cord is presented. Extramedullary haematopoietic nodules are a rare cause of spinal cord compression and should be included in the differential diagnosis, especially in patients from Mediterranean countries. Treatment with radiation therapy solely failed, giving rise to the need of surgical intervention. Surgical decompression of the spine and the removal of the culprit lesion compressing the spine were performed. Postinterventional radiation therapy was applied to the spine. A relapse had to be treated again by surgical means combined with postinterventional radiation therapy. A complete relief of the symptoms and control of the lesion could be obtained

  2. Computer navigation vs extramedullary guide for sagittal alignment of tibial components: radiographic study and meta-analysis.

    Science.gov (United States)

    Kuzyk, Paul R T; Higgins, Gordon A; Tunggal, James A W; Sellan, Michael E; Waddell, James P; Schemitsch, Emil H

    2012-04-01

    Sagittal alignment of tibial components using computer navigation was compared with conventional methods. A radiologic study was performed using 110 total knee arthroplasties from 3 groups: computer navigation, cutting block with extramedullary guide, and manual tilt of extramedullary guide. Posterior tibial slopes were measured from radiographs and compared using statistical methods. The cutting block method was the most accurate, and computer navigation was the most precise. The manual tilt group had the greatest variance, significantly greater than computer navigation. There was no significant difference between groups with respect to the percentage of knees with posterior slope within 3° of the desired slope. Meta-analysis of 10 studies found no reduction in outliers with computer navigation. Computer navigation offers greatest precision but does not reduce the number of outliers. Copyright © 2012 Elsevier Inc. All rights reserved.

  3. Magnetic resonance imaging surveillance following vestibular schwannoma resection.

    Science.gov (United States)

    Carlson, Matthew L; Van Abel, Kathryn M; Driscoll, Colin L; Neff, Brian A; Beatty, Charles W; Lane, John I; Castner, Marina L; Lohse, Christine M; Link, Michael J

    2012-02-01

    To describe the incidence, pattern, and course of postoperative enhancement within the operative bed using serial gadolinium-enhanced magnetic resonance imaging (MRI) following vestibular schwannoma (VS) resection and to identify clinical and radiologic variables associated with recurrence. Retrospective cohort study. All patients who underwent microsurgical resection of VS between January 2000 and January 2010 at a single tertiary referral center were reviewed. Postoperative enhancement patterns were characterized on serial MRI studies. Clinical follow-up and outcomes were recorded. During the last 10 years, 350 patients underwent microsurgical VS resection, and of these, 203 patients met study criteria (mean radiologic follow-up, 3.5 years). A total of 144 patients underwent gross total resection (GTR), 32 received near-total resection (NTR), and the remaining 27 underwent subtotal resection (STR); 98.5% of patients demonstrated enhancement within the operative bed following resection (58.5% linear, 41.5% nodular). Stable enhancement patterns were seen in 24.5% of patients, regression in 66.0%, and resolution in only 3.5% of patients on the most recent postoperative MRI. Twelve patients recurred a mean of 3.0 years following surgery. The average maximum linear diameter growth rate among recurrent tumors was 2.3 mm per year. Those receiving STR were more than nine times more likely to experience recurrence compared to those undergoing NTR or GTR (P assist the clinician in determining an appropriate postoperative MRI surveillance schedule. Future studies using standardized terminology and consistent study metrics are needed to further refine surveillance recommendations. Copyright © 2011 The American Laryngological, Rhinological, and Otological Society, Inc.

  4. A Xenograft Model of Vestibular Schwannoma and Hearing Loss.

    Science.gov (United States)

    Dinh, Christine T; Bracho, Olena; Mei, Christine; Bas, Esperanza; Fernandez-Valle, Cristina; Telischi, Fred; Liu, Xue-Zhong

    2018-03-19

    Microsurgical implantation of mouse merlin-deficient Schwann cells (MD-SC) into the cerebellopontine angle of immunodeficient rats will initiate tumor formation, hearing loss, and vestibular dysfunction. The progress in identifying effective drug therapies for treatment of Neurofibromatosis type II (NF2) is limited by the availability of animal models of VS that develop hearing loss and imbalance. A microsurgical technique for implanting MD-SCs onto the cochleovestibular nerve of rats was developed. Ten Rowett Nude rats were implanted with either ∼10 MD-SCs expressing luciferase (N = 5) or vehicle (N = 5). Rats received bioluminescence imaging, auditory brainstem response testing, and were observed for head tilt every 2 weeks after surgery, for a total of 6 weeks. Tumors were harvested and processed with hematoxylin & eosin staining and immunohistochemistry was performed for S100. Rats implanted with MD-SCs developed significantly higher tumor bioluminescence measurements and hearing threshold shifts at multiple frequencies by the 4th and 6th weeks post-implantation, compared with control rats. Rats implanted with MD-SCs also developed gross tumor. The tumor volume was significantly greater than nerve volumes obtained from rats in the control group. All rats with tumors developed a head tilt, while control rats had no signs of vestibular dysfunction. Tumors demonstrated histological features of schwannoma and express S100. Using this microsurgical technique, this xenograft rat model of VS develops tumors involving the cochleovestibular nerve, shifts in hearing thresholds, and vestibular dysfunction. This animal model can be used to investigate tumor-mediated hearing loss and perform preclinical drug studies for NF2.

  5. Dynamical analysis of balance in vestibular schwannoma patients.

    Science.gov (United States)

    Hoffmann, Charles P; Seigle, Benoît; Frère, Julien; Parietti-Winkler, Cécile

    2017-05-01

    The analysis of the complexity of postural fluctuations is a recent method for assessing postural control. Complexity relates to the irregularity of the center of pressure time series and characterizes the ability of postural control to meet a changing environment. In our study, we used the sample entropy (SampEn) parameter to evaluate the complexity of postural sway velocity time series in patients with vestibular schwannoma (n=19) compared to healthy controls (n=20), using the sensory organization test. Patients performed postural assessments three days before surgical ablation of the tumor, then three times after surgery, at eight, thirty, and ninety days. The control group underwent posturographic tests only once. Our results demonstrated that SampEn values distinguished both groups before surgery only in postural tasks where vestibular afferences significantly contribute to maintaining balance. We also found an immediate decrease of complexity after the surgical resection of the tumor. Our results are in line with the theory of complexity loss of physiological systems stating that reducing the number of their structural components or altering their coupling leads to a decrease in complexity. Finally, our findings showed that progressive restoration of complexity over time was such that no difference was found between the two groups ninety days after surgery, due to the implementation of central adaptive mechanisms and the substitution by other sensory afferences. Thus, the SampEn parameter can highlight the postural effects of vestibular pathology, and complexity analysis appears to be a valuable tool for investigating the temporal structure of CoP time series. Copyright © 2017 Elsevier B.V. All rights reserved.

  6. Prediction of Balance Compensation After Vestibular Schwannoma Surgery.

    Science.gov (United States)

    Parietti-Winkler, Cécile; Lion, Alexis; Frère, Julien; Perrin, Philippe P; Beurton, Renaud; Gauchard, Gérome C

    2016-06-01

    Background Balance compensation after vestibular schwannoma (VS) surgery is under the influence of specific preoperative patient and tumor characteristics. Objective To prospectively identify potential prognostic factors for balance recovery, we compared the respective influence of these preoperative characteristics on balance compensation after VS surgery. Methods In 50 patients scheduled for VS surgical ablation, we measured postural control before surgery (BS), 8 (AS8) days after, and 90 (AS90) days after surgery. Based on factors found previously in the literature, we evaluated age, body mass index and preoperative physical activity (PA), tumor grade, vestibular status, and preference for visual cues to control balance as potential prognostic factors using stepwise multiple regression models. Results An asymmetric vestibular function was the sole significant explanatory factor for impaired balance performance BS, whereas the preoperative PA alone significantly contributed to higher performance at AS8. An evaluation of patients' balance recovery over time showed that PA and vestibular status were the 2 significant predictive factors for short-term postural compensation (BS to AS8), whereas none of these preoperative factors was significantly predictive for medium-term postoperative postural recovery (AS8 to AS90). Conclusions We identified specific preoperative patient and vestibular function characteristics that may predict postoperative balance recovery after VS surgery. Better preoperative characterization of these factors in each patient could inform more personalized presurgical and postsurgical management, leading to a better, more rapid balance recovery, earlier return to normal daily activities and work, improved quality of life, and reduced medical and societal costs. © The Author(s) 2015.

  7. Long-Term Results for Trigeminal Schwannomas Treated With Gamma Knife Surgery

    Energy Technology Data Exchange (ETDEWEB)

    Hasegawa, Toshinori, E-mail: h-toshi@komakihp.gr.jp; Kato, Takenori; Iizuka, Hiroshi; Kida, Yoshihisa

    2013-12-01

    Purpose: Surgical resection is considered the desirable curative treatment for trigeminal schwannomas. However, complete resection without any complications remains challenging. During the last several decades, stereotactic radiosurgery (SRS) has emerged as a minimally invasive treatment modality. Information regarding long-term outcomes of SRS for patients harboring trigeminal schwannomas is limited because of the rarity of this tumor. The aim of this study was to evaluate long-term tumor control and functional outcomes in patients harboring trigeminal schwannomas treated with SRS, specifically with gamma knife surgery (GKS). Methods and Materials: Fifty-three patients harboring trigeminal schwannomas treated with GKS were evaluated. Of these, 2 patients (4%) had partial irradiation of the tumor, and 34 patients (64%) underwent GKS as the initial treatment. The median tumor volume was 6.0 cm{sup 3}. The median maximum and marginal doses were 28 Gy and 14 Gy, respectively. Results: The median follow-up period was 98 months. On the last follow-up image, 7 patients (13%) had tumor enlargement, including the 2 patients who had partial treatment. Excluding the 2 patients who had partial treatment, the actuarial 5- and 10-year progression-free survival (PFS) rates were 90% and 82%, respectively. Patients with tumors compressing the brainstem with deviation of the fourth ventricle had significantly lower PFS rates. If those patients with tumors compressing the brainstem with deviation of the fourth ventricle are excluded, the actuarial 5- and 10-year PFS rates increased to 95% and 90%, respectively. Ten percent of patients had worsened facial numbness or pain in spite of no tumor progression, indicating adverse radiation effect. Conclusions: GKS can be an acceptable alternative to surgical resection in patients with trigeminal schwannomas. However, large tumors that compress the brainstem with deviation of the fourth ventricle should be surgically removed first and then

  8. Quality of Life in 807 Patients with Vestibular Schwannoma: Comparing Treatment Modalities.

    Science.gov (United States)

    Soulier, Géke; van Leeuwen, Bibian M; Putter, Hein; Jansen, Jeroen C; Malessy, Martijn J A; van Benthem, Peter Paul G; van der Mey, Andel G L; Stiggelbout, Anne M

    2017-07-01

    Objective In vestibular schwannoma treatment, the choice among treatment modalities is controversial. The first aim of this study was to examine the quality of life of patients with vestibular schwannoma having undergone observation, radiation therapy, or microsurgical resection. The second aim was to examine the relationship between perceived symptoms and quality of life. Last, the association between quality of life and time since treatment was studied. Study Design Cross-sectional study. Setting Tertiary referral center. Subjects and Methods A total of 1208 patients treated for sporadic vestibular schwannoma between 2004 and 2014 were mailed the disease-specific Penn Acoustic Neuroma Quality of Life (PANQOL) questionnaire and additional questions on symptoms associated with vestibular schwannoma. Total and domain scores were calculated and compared among treatment groups. Propensity scores were used, and results were stratified according to tumor size to control for potential confounders. Correlations were calculated to examine the relationship between self-reported symptoms and quality of life, as well as between quality of life and time since treatment. Results Patients with small tumors (≤10 mm) under observation showed a higher PANQOL score when compared with the radiation therapy and microsurgical resection groups. A strong negative correlation was found between self-reported symptoms and quality of life, with balance problems and vertigo having the largest impact. No correlation was found between PANQOL score and time since treatment. Conclusion This study suggests that patients with small vestibular schwannomas experience better quality of life when managed with observation than do patients who have undergone active treatment.

  9. Gastric schwannoma: a clinicopathologic study of 51 cases and critical review of the literature.

    Science.gov (United States)

    Voltaggio, Lysandra; Murray, Rebecca; Lasota, Jerzy; Miettinen, Markku

    2012-05-01

    Schwannoma is a rare gastrointestinal mesenchymal tumor, as the vast majority of gastric mesenchymal tumors are gastrointestinal stromal tumors. In this study, we analyzed clinicopathologically 51 gastric schwannomas. These tumors predominantly occurred in older adults with a marked female predominance (40 women and 11 men; median and mean ages, 60 and 58 years). They variably presented with gastric discomfort, bleeding, or rarely gastric outlet obstruction; and many were incidental findings during other medical procedures. The tumors ranged from 1 to 10.5 cm (median, 4.5 cm). The typical histologic features included spindle cells usually with microtrabecular architecture and focal nuclear atypia, and peritumoral lymphoid cuff, whereas features of soft tissue schwannomas, such as encapsulation, nuclear palisading, vascular hyalinization, and dilatation, were absent or infrequent. Median mitotic count was 2/50 high-power fields, with the highest count being 13/50 high-power fields. No malignant variants were recognized, and long-term follow-up did not reveal recurrences or metastases. Immunohistochemically, all examined tumors were S100 protein positive and most were also GFAP positive, whereas CD34 and NF68 were encountered rarely and all tumors were negative for HMB45, KIT, DOG1/Ano 1, smooth muscle actin, desmin, and synaptophysin. None of the 9 tumors studied contained gastrointestinal stromal tumor-specific KIT or PDGFRA mutations. Fluorescence in situ hybridization studies revealed multiple signals with BCR probe (chromosome 22) and centromeric probes for chromosomes 2 and 18 suggesting polyploidy. These findings indicate that gastric schwannoma is a distinctive form of peripheral nerve sheath tumor that in many ways differs from soft tissue schwannoma. It should be distinguished from gastrointestinal stromal tumor and other mesenchymal tumors of the gastrointestinal tract, such as the S100 protein-positive gastrointestinal clear cell sarcoma and metastatic

  10. GASTRIC SCHWANNOMA – A CLINICOPATHOLOGIC STUDY OF 51 CASES AND CRITICAL REVIEW OF THE LITERATURE

    Science.gov (United States)

    Voltaggio, Lysandra; Murray, Rebecca; Lasota, Jerzy; Miettinen, Markku

    2011-01-01

    Schwannoma is a rare gastrointestinal mesenchymal tumor as the vast majority of gastric mesenchymal tumors are GISTs. In this study, we analyzed clinicopathologically 51 gastric schwannomas. These tumors predominantly occurred in older adults with a marked female predominance (40 women and 11 men, median and mean ages, 60 and 58 years). They variably presented with gastric discomfort, bleeding, or rarely by gastric outlet obstruction, and many were incidental findings during other medical procedures. The tumors ranged from 1–10.5 cm (median, 4.5 cm). The typical histologic features included spindle cells usually with microtrabecular architecture and focal nuclear atypia, and peritumoral lymphoid cuff, whereas features of soft tissue schwannomas, such as encapsulation, nuclear palisading, vascular hyalinization and dilatation, were absent or infrequent. Median mitotic count was 2/50 HPFs, with the highest count being 13/50 HPFs. No malignant variants were recognized, and long-term follow-up did not reveal recurrences or metastases. Immunohistochemically, all examined tumors were S100 protein positive and most were also GFAP positive, whereas CD34 and NF68 were encountered rarely and all tumors were negative for HMB45, KIT, DOG1/Ano 1, SMA, desmin, and synaptophysin. None of the 9 tumors studied contained GIST-specific KIT or PDGFRA mutations. FISH studies revealed multiple signals with BCR probe (chromosome 22) and centromeric probes for chromosomes 2 and 18 suggesting polyploidy. These findings indicate that gastric schwannoma is a distinctive form of peripheral nerve sheath tumor that in many ways differs from soft tissue schwannoma. It should be distinguished from GIST and other mesenchymal tumors of the gastrointestinal tract, such as the S100 protein-positive gastrointestinal clear cell sarcoma and metastatic melanoma. PMID:22137423

  11. Favorable Swallowing Outcomes following Vagus Nerve Sacrifice for Vagal Schwannoma Resection.

    Science.gov (United States)

    Patel, Mira A; Eytan, Danielle F; Bishop, Justin; Califano, Joseph A

    2017-02-01

    Objective To determine the impact of unilateral vagal sacrifice for vagal schwannoma on postoperative swallowing function. Study Design Case series, chart review. Setting Academic medical institution. Subjects and Methods Ten patients underwent vagus nerve sacrifice for vagal schwannoma resection. Archived pathology records dating from 1985 through 2012 at our institution were retrospectively queried for cases of vagal schwannoma with vagus nerve sacrifice. Medical records were abstracted for demographic and disease information as well as cranial nerve and swallowing function. Preoperative and postoperative cranial nerve function, subjective and objective measures of swallowing function, Functional Oral Intake Scale (FOIS) level, and need for vocal fold medialization were variables collected. Data were analyzed with summary statistics. Results The patients who underwent vagal sacrifice for vagal schwannoma at our institution had a mean age of 42.3 years (median, 44 years; range, 15-63 years) and follow-up of 35.6 months (median, 9 months; range, 1-115 months). Most presented with no preoperative cranial nerve deficit or difficulty swallowing. Immediately postoperatively, 90% had a vagus nerve deficit, but 50% had no subjective difficulty swallowing, and 70% had a FOIS level of 7 at postoperative hospital discharge. Within 1 month after surgery, 70% had normal swallowing function according to a modified barium swallow study. A full diet was tolerated by mouth within an average of 2.7 days (median, 2 days; range, 1-6 days) after surgery in this cohort. Seventy percent required vocal fold medialization postoperatively for incomplete glottic closure. Conclusion Vagal nerve sacrifice during resection of vagal schwannoma can be performed with normal postoperative swallowing function.

  12. Hearing outcomes of vestibular schwannoma patients managed with 'wait and scan': predictive value of hearing level at diagnosis

    DEFF Research Database (Denmark)

    Stangerup, S-E; Tos, M; Thomsen, J

    2010-01-01

    This study aimed to evaluate the predictive value of both hearing level (at various frequencies) and speech discrimination for forecasting hearing outcome after a period of observation, in patients with vestibular schwannoma....

  13. Testing the Role of p21 Activated Kinases in Schwannoma Formation Using a Novel Genetically Engineered Murine Model that Closely Phenocopies Human NF2 Disease

    Science.gov (United States)

    2017-06-01

    Kinases in Schwannoma Formation Using a Novel Genetically Engineered Murine Model that Closely Phenocopies Human NF2 Disease The views, opinions and...Role of p21 Activated Kinases in Schwannoma Formation Using a Novel Genetically Engineered Murine Model that Closely Phenocopies Human NF2 Disease Form...autosomal dominant genetic disease characterized by benign schwannomas that grow on the cranial and spinal nerves. While technically benign, the tumors are

  14. Visual outcome after fronto-temporo-orbito-zygomatic approach combined with early extradural and intradural optic nerve decompression in tuberculum and diaphragma sellae meningiomas.

    Science.gov (United States)

    Mortini, Pietro; Barzaghi, Lina Raffaella; Serra, Carlo; Orlandi, Vittoria; Bianchi, Stefania; Losa, Marco

    2012-07-01

    The surgical challenge of the treatment of tuberculum (TSMs) and diaphragma sellae meningiomas (DSMs) is to preserve or improve the visual function. Extradural and intradural optic nerve decompression should reduce surgical trauma of the nerve achieving a good visual result. We reported 37 consecutive TSMs and DSMs operated through fronto-temporo-orbito-zygomatic approach with extradural unroofing of the optical canal and early intradural incision of the dural sheath. Visual data were recorded measuring the visual impairment score (VIS), the visual acuity (VA), the visual field (VF) and the postoperative improvement. A good visual outcome (VIS improved or unchanged) was obtained in 97.2% of patients (35/36). The evaluation of 72 eyes showed a good outcome (VA and VF unchanged or improved) in 98.6% (71/72 eyes). The degree of preoperative VA and VF impairment was the only factor correlating with the postoperative improvement of VA (P<.001 and P=.018) and VF defect (P<.001). Worsening of visual function occurred in 1/37 patient (2.7%). Using this surgical technique we achieved a high improvement rate of visual defects and a low frequency of worsening. Copyright © 2011 Elsevier B.V. All rights reserved.

  15. MR imaging of a malignant schwannoma and an osteoblastoma with fluid-fluid levels. Report of two new cases

    International Nuclear Information System (INIS)

    Vilanova, J.C.; Dolz, J.L.; Aldoma, J.; Capdevila, A.; Maestro de Leon, J.L.; Aparicio, A.

    1998-01-01

    One case of malignant schwannoma of the sacrum and another of occipital osteoblastoma were evaluated by MR imaging. Both tumors showed fluid-fluid levels with different signal intensities in the sequences performed. Pathologic examination revealed hemmorhagic fluid in both tumors. Malignant schwannoma and osteoblastoma should be included in the list of bone and soft-tissue with fluid-fluid levels. Our data confirm the non-specificity of this finding, which only suggests the presence of previous intratumoral hemorrhage. (orig.) (orig.)

  16. Unilateral Vestibular Schwannomas in Childhood without Evidence of Neurofibromatosis: Experience of 10 Patients at a Single Institute.

    Science.gov (United States)

    Yin, Luxin; Ma, Zhenyu; Li, Chunde; Luo, Shiqi

    2017-01-01

    We investigated the clinical presentation and management issues of unilateral vestibular schwannomas in childhood without evidence of neurofibromatosis. Ten pediatric patients with unilateral vestibular schwannoma and no evidence of neurofibromatosis were treated by the senior author at Tiantan Hospital from January 1994 to December 2014. The clinical manifestations, neuroimaging findings, treatment methods, and therapeutic results were retrospectively reviewed. The study included 6 male and 4 female patients. The mean age of the patients was 13.9 years (range, 11-15 years). Common clinical features included increased intracranial pressure, hearing loss, and ataxia. The median diameter of the tumors was 5.15 cm. Gross total resection was achieved in 9 of 10 patients via a suboccipital retrosigmoid approach, and the perioperative mortality rate was 10%. The follow-up period ranged from 1 to 12 years. One patient with facial nerve injury recovered to House? Brackmann grade I postoperatively, 5 to grade II-III, and 2 to grade IV-V. Only 1 patient had preserved hearing. The clinical features of pediatric vestibular schwannomas are different from those of adult vestibular schwannomas, and cranial nerve preservation in children is more difficult. When a child presents with hearing loss as well as cognitive disability, a vestibular schwannoma should be highly suspected. Hypervascular vestibular schwannomas in childhood should be managed by multistaged surgeries.

  17. Detection of Extramedullary Multiple Myeloma in Liver by FDG-PET/CT

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    Kim, Daeweung; Kim, Woo Hyoung; Kim, Myoung Hyoun; Choi, Keum Ha; Kim, Chang Guhn [Wonkwang Univ. School of Medicine, Iksan (Korea, Republic of)

    2014-06-15

    We present the case of a 42-year-old man with a painful mass lesion in the right shoulder that was detected by contrast-enhanced computed tomography (CT) and {sup 18}F-fluoro-2-deoxyglucose ({sup 18}F-FDG) positron emission tomography (PET)/CT. Excisional biopsy revealed infiltration of plasma cells with anaplastic features, consistent with solitary plasmacytoma (PC). Serum analysis showed elevation of serum free lambda light chain levels (27.78 mg/l), with an abnormally high kappa:lambda ratio (2.33) and high total proteins (10.4 g/dl). Serum protein electrophoresis revealed an M spike in the gamma-globulin region (56.1 %=5.8 g/dl). Subsequently, {sup 18}F-FDG PET/CT revealed another hypermetabolic mass in the right lobe of the liver. CT-guided biopsy of the liver lesion revealed plasma cell myeloma, consistent with multiple myeloma. Multiple myeloma presenting as nodular liver masses is very rare in clinical practice. In a retrospective review of more than 2,000 patients, Talamo et al. reported only nine cases where there was nodular involvement of the liver by multiple myeloma. The organ most commonly involved was the liver, followed by pancreas, stomach, peritoneum with malignant ascites, colon, rectum, duodenum and ileum. Therefore, the literature published thus far has been limited to a few reports and case series. Among these reports, some had demonstrated the PET or PET/CT findings of nodular liver involvement of multiple myeloma. About 10 % of the solitary myelomas appeared as extramedullary PC or solitary PC of bone. In spite of the advances in therapy, the treatment of multiple myeloma is still palliative. However, solitary PC could be cured by resection or radiation therapy. Thus, differentiation between PC and multiple myeloma is essential in making a decision for the appropriate therapeutic regimen. {sup 18}F-FDG PET/CT has the unique ability to detect and characterize malignant lesions in one single examination. Schirrmeister et al. reported that

  18. Predictors of Preoperative Tinnitus in Unilateral Sporadic Vestibular Schwannoma

    Directory of Open Access Journals (Sweden)

    Georgios Naros

    2017-08-01

    Full Text Available ObjectiveNearly two-thirds of patients with vestibular schwannoma (VS are reporting a significantly impaired quality of life due to tinnitus. VS-associated tinnitus is attributed to an anatomical and physiological damage of the hearing nerve by displacing growth of the tumor. In contrast, the current pathophysiological concept of non-VS tinnitus hypothesizes a maladaptive neuroplasticity of the central nervous system to a (hidden hearing impairment resulting in a subjective misperception. However, it is unclear whether this concept fits to VS-associated tinnitus. This study aims to determine the clinical predictors of VS-associated tinnitus to ascertain the compatibility of both pathophysiological concepts.MethodsThis retrospective study includes a group of 478 neurosurgical patients with unilateral sporadic VS evaluated preoperatively regarding the occurrence of ipsilateral tinnitus depending on different clinical factors, i.e., age, gender, tumor side, tumor size (T1–T4 according to the Hannover classification, and hearing impairment (Gardner–Robertson classification, GR1–5, using a binary logistic regression.Results61.8% of patients complain about a preoperative tinnitus. The binary logistic regression analysis identified male gender [OR 1.90 (1.25–2.75; p = 0.002] and hearing impairment GR3 [OR 1.90 (1.08–3.35; p = 0.026] and GR4 [OR 8.21 (2.29–29.50; p = 0.001] as positive predictors. In contrast, patients with large T4 tumors [OR 0.33 (0.13–0.86; p = 0.024] and complete hearing loss GR5 [OR 0.36 (0.15–0.84; p = 0.017] were less likely to develop a tinnitus. Yet, 60% of the patients with good clinical hearing (GR1 and 25% of patients with complete hearing loss (GR5 suffered from tinnitus.ConclusionThese data are good accordance with literature about non-VS tinnitus indicating hearing impairment as main risk factor. In contrast, complete hearing loss appears a negative predictor for tinnitus. For the first

  19. State Anxiety Subjective Imbalance and Handicap in Vestibular Schwannoma.

    Science.gov (United States)

    Saman, Yougan; Mclellan, Lucie; Mckenna, Laurence; Dutia, Mayank B; Obholzer, Rupert; Libby, Gerald; Gleeson, Michael; Bamiou, Doris-Eva

    2016-01-01

    Evidence is emerging for a significant clinical and neuroanatomical relationship between balance and anxiety. Research has suggested a potentially priming effect with anxiety symptoms predicting a worsening of balance function in patients with underlying balance dysfunction. We propose to show that a vestibular stimulus is responsible for an increase in state anxiety, and there is a relationship between increased state anxiety and worsening balance function. (1) To quantify state anxiety following a vestibular stimulus in patients with a chronic vestibular deficit. (2) To determine if state anxiety during a vestibular stimulus would correlate with the severity of chronic balance symptoms and handicap. Two separate cohorts of vestibular schwannoma (VS) patients underwent vestibular tests (electronystagmography, cervical and ocular vestibular evoked myogenic potentials, and caloric responses) and questionnaire assessments [vertigo handicap questionnaire (VHQ), vertigo symptom scale (VSS), and state-trait anxiety inventory (STAIY)]. Fifteen post-resection VS patients, with complete unilateral vestibular deafferentation, were assessed at a minimum of 6 months after surgery in Experiment 1 (Aim 1). Forty-five patients with VS in situ formed the cohort for Experiment 2 (Aim 2). Experiment 1: VS subjects (N = 15) with a complete post-resection unilateral vestibular deafferentation completed a state anxiety questionnaire before caloric assessment and again afterward with the point of maximal vertigo as the reference (Aim 1). Experiment 2: state anxiety measured at the point of maximal vertigo following a caloric assessment was compared between two groups of patients with VS in situ presenting with balance symptoms (Group 1, N = 26) and without balance symptoms (Group 2, N = 11) (Aim 2). The presence of balance symptoms was defined as having a positive score on the VSS-VER. In Experiment 1, a significant difference (p vestibular deafferentation. In

  20. Are stage IV vestibular schwannomas preoperatively different from other stages?

    Science.gov (United States)

    Tringali, Stéphane; Dubreuil, Christian; Zaouche, Sandra; Ferber-Viart, Chantal

    2008-01-01

    The aim of this study was to focus on the clinical and paraclinical symptoms of patients suffering from Stage IV vestibular schwannomas (VSs). In this prospective study, we included 734 patients who have VS and candidates for operation. Patients were classified as having Stage I, II, III, or IV tumors according to Tos criteria as evaluated by magnetic resonance imaging. PREOPERATIVE CLINICAL EVALUATION: We recorded the occurrence of complaints (%) and duration (yr) of hearing loss, tinnitus, and balance disorder. Preoperative paraclinical evaluation included pure-tone (PTA) and speech audiometry, auditory brainstem response (ABR) patterns, and vestibular deficit at videonystamography (VNG). Continuous variables were compared between Stage IV and other stages using analysis of variance. Qualitative variables expressed as a percentage of presence were compared between Stage IV and other stages using percentage comparison. Quantitative Parameters. Patients with Stage IV VS were significantly younger as compared with patients with other stages. Stage IV hearing loss was greater compared with other stages at 250 and 500 Hz but smaller at 2,000 and 8,000 Hz. We found no difference in the loss of PTA between Stage IV and the other stages. Speech discriminancy score was smaller in Stage IV. The durations of hearing loss, tinnitus, and balance disorders were similar whatever the tumor stage. Auditory brainstem response patterns showed no difference in Wave III latency between Stage IV VS and other stages, whereas Wave V latency and V-I interval were higher in Stage IV. Both ABR threshold and VNG caloric deficit were higher in Stage IV VS compared with other stages. Qualitative Parameters. The percentage of patients with Stage IV was lower than that with Stages II and III. The percentage of men and women was similar in all stages. The occurrence of hearing loss was similar in all stages, whereas that of tinnitus was lower in Stage IV compared with Stages I and II. In

  1. Indications of Gamma knife radiosurgery for vestibular schwannomas

    International Nuclear Information System (INIS)

    Fukuoka, Seiji; Takanashi, Masami; Hojyo, Atsufumi; Tanaka, Chiharu; Konishi, Masanori; Nakamura, Hirohiko

    2007-01-01

    The purpose of this study was to investigate the indication of gamma knife radiosurgery for vestibular schwannomas by analyzing tumor control and possible complications using low marginal doses and conformal multiple shots to fit irregular tumor shapes. The authors evaluated 223 patients with followed-up periods ranging from 5 years to 15 years (mean 7.7 years, median 7.4 years). Marginal doses were 9 to 15 Gy (mean 12.5 Gy, median 12 Gy) with corresponding treatment volumes being between 0.1 and 18.7 cm 3 (mean 2.6 cm 3 , median 1.8 cm 3 ). The number of isocenters varied from 2 to 24 shots (mean 9, median 9.2). The actuarial tumor control rates were 95% at 5 years and 94% at 7 years, respectively. Larger tumors (p=0.0068) and those in younger patients (p=0.093) tended to recur significantly. The preservation rates of useful hearing were 84%, 71%, and 64% at 2, 4, and 7 years, respectively. The most deterioration seemed to occur in cases with elderly patients (p=0.0048). Facial and trigeminal functions were preserved at 100%, and 97.8%, respectively. Amongst all patients, 20.6% developed transient dizziness, with persistent dizziness remaining in 1.5% of the total. Fifty-six other patients not in the long-term evaluation consecutively underwent caloric testing and static stabilometry as well as neurological examinations to evaluate vestibular function in detail, both before and after gamma knife radiosurgery (GKRS). The results showed that 90% of the patients had already developed vestibular dysfunction before the treatment despite reported symptoms of dizziness. GKRS did not significantly affect vestibular function. Hydrocephalus was recognized in 5.5% of all patients, and seemed to occur primarily in cases with larger tumors (p=0.0189). GKRS provides a safe and effective therapy for small to medium sized tumors up to 8 cm 3 . Long-term hearing preservation rate may be affected by presbycusis in elderly patients. (author)

  2. Incidence of extramedullary relapse after haploidentical SCT for advanced AML/myelodysplastic syndrome.

    Science.gov (United States)

    Yoshihara, S; Ikegame, K; Kaida, K; Taniguchi, K; Kato, R; Inoue, T; Fujioka, T; Tamaki, H; Okada, M; Soma, T; Ogawa, H

    2012-05-01

    Extramedullary (EM) relapse of leukemia after allo-SCT in patients with AML/myelodysplastic syndrome has been increasingly reported. The reduced effectiveness of the GVL effect in EM sites, as compared with BM, has been suggested to underlie this problem. We retrospectively analyzed the pattern of relapse after haploidentical SCT (haplo-SCT), performed as the first or second SCT. Among 38 patients who received haplo-SCT as their first SCT, the cumulative incidences of BM and EM relapse at 3 years were 40.5 and 10.9%, respectively. Among 19 patients who received haplo-SCT as their second SCT, the cumulative incidences of BM and EM relapse were 30.9 and 31.9%, respectively. Moreover, most of the patients who underwent repeat haplo-SCT for the treatment of EM relapse had further EM relapse at other sites. Post-relapse survival did not differ significantly with different patterns of relapse. The frequent occurrence of EM relapse after haplo-SCT, particularly when performed as a second SCT, suggests that the potent GVL effect elicited by an HLA disparity also occurs preferentially in BM. Our findings emphasize the need for a treatment strategy for EM relapse that recognizes the reduced susceptibility of EM relapse to the GVL effect.

  3. Spinal cord compression due to extramedullary hematopoiesis in beta-thalassemia intermedia

    International Nuclear Information System (INIS)

    Munn, Rita K.; Kramer, Carol A.; Arnold, Susanne M.

    1998-01-01

    Background: Extramedullary hematopoiesis (EMH) occurs in many disorders, including thalassemias and other hemoglobinopathies, and commonly presents in the spleen and liver. We present a case of spinal cord compression in a patient with beta-thalassemia intermedia, and review the literature and available treatment options. Patient and Methods: A 35-year-old black female with beta-thalassemia intermedia presented with a 3-week history of back pain and lower extremity weakness. Neurologic examination was consistent with spinal cord compression, and gadolinium enhanced magnetic resonance imaging (MRI) confirmed this diagnosis. She was given intravenous steroids and radiotherapy was begun in 200 cGy fractions to a total dose of 2000 cGy. Results: At the completion of radiotherapy the patient was ambulatory with mild residual weakness. MRI scans 16 months later showed smaller, but persistent masses, and she remains asymptomatic 5 years from her diagnosis. Conclusion: Recognition of spinal cord EMH requires prompt physical examination and MRI for accurate diagnosis. EMH can be managed with radiation, surgery, transfusions, or a combination of these therapies. Radiation in conservative doses of (750-3500 cGy) is non-invasive, avoids the surgical risks of potentially severe hemorrhage and incomplete resection, and has a high complete remission rate in the majority of patients. Relapse rates are moderate (37.5%), but retreatment provides excellent chance for second remission

  4. Extramedullary Plasmacytoma of the Larynx Treated by a Surgical Endoscopic Approach and Radiotherapy

    Directory of Open Access Journals (Sweden)

    Massimiliano Pino

    2015-01-01

    Full Text Available Extramedullary plasmacytoma (EMP is a rare variant of plasma cell myeloma that affects soft tissues. The head and neck region are the most affected sites, although others have also been described. Herein we report an uncommon case of EMP of the larynx in a 65-year-old male who presented with a history of progressive dysphonia and hoarseness. Laryngeal fiberscopy evidenced a reddish pedicled voluminous mass in the left false cords and ventricle. Microscopic suspension laryngoscopy was performed under general anaesthesia and a 4 W Acublade CO2 Laser was used for transoral resection of the lesion. This was followed by adjuvant radiotherapy, with the widely recommended doses on the supraglottic region, to achieve better local control. Diagnosis of EMP is based on immunohistochemistry and the exclusion of systemic plasma cell proliferative disorders. Diagnosis of solitary EMP can be made only if studies for disseminated disease and X-ray and/or magnetic resonance imaging of the spine, pelvis, femurs, and humerus and bone marrow biopsy are negative. As there are no internationally established guidelines, treatment of EMP is mainly based on consensus of expert opinion.

  5. Extramedullary Plasmacytoma of the Larynx: A Case Report of Subglottic Localization

    Directory of Open Access Journals (Sweden)

    Jaqueline Ramírez-Anguiano

    2012-01-01

    Full Text Available Extramedullary plasmacytoma (EMP is a rare neoplasm of plasma cells, described in soft tissue outside the bone marrow. EMP of the larynx represents 0.04 to 0.45% of malignant tumors of the larynx. A male of 57 years old presented with hoarseness, dyspnea, and biphasic stridor of 2 months. The indirect laryngoscopy (IL revealed severe edema of the posterior commissure and a polypoid mass in the right posterior lateral subglottic wall. A biopsy of the subglottic mass was performed by a direct laryngoscopy (DL. The histopathologic diagnosis was EMP CD138+, therefore radiotherapy was given at 54 Gy in 30 sessions. The patient had an adequate postoperative clinical course and a new biopsy was performed having tumor-free margins. All laryngeal lesions should be biopsied prior to treatment to determine an accurate diagnosis to guide a proper management of the condition. Radiation therapy to the EMP is considered the treatment of choice, having local control rates of 80% to 100%. The subglottis is the least accessible area of view and the least frequent location of a laryngeal mass, nevertheless the otolaryngologist should always do a complete and systematic exam of the larynx when a tumor is suspected, to detect diagnoses such as a subglottic plasmacytoma.

  6. A rare case of cervical epidural extramedullary plasmacytoma presenting with monoparesis

    Directory of Open Access Journals (Sweden)

    Turk Okan

    2017-03-01

    Full Text Available Multiple myeloma and other plasma cell disorders are characterized by production of a large number of plasma cells in the bone marrow. On the other hand, plasmacytoma results from proliferation of abnormal plasma cells in the soft tissue or skeletal system. Neurological complications are frequently observed in these diseases. The most commonly known complications among those complications are spine fractures, spinal cord compressions, and peripheral neuropathies. Although neurological involvements are common in plasmacytomas, extramedullary spinal epidural localizations have been reported very rarely. In this case report, we aimed to present a plasmacytoma case that presented with acute onset of upper extremity monoparesis. A 40-year-old woman was admitted to our clinic with complaints of sudden weakness and numbness in her left arm following neck and left arm pain. Emergency cervical magnetic resonance imaging (MRI revealed an epidural mass and the patient underwent emergency surgery. The patient showed improvement post-operatively and the pathology was reported as plasmacytoma. Following hematology consultation, systemic chemotherapy was initiated and radiotherapy was planned after wound healing.

  7. Role of the spleen in cyclophosphamide-induced hematosuppression and extramedullary hematopoiesis in mice.

    Science.gov (United States)

    Wang, Yuli; Meng, Qinggang; Qiao, Haiquan; Jiang, Hongchi; Sun, Xueying

    2009-05-01

    Extramedullary hematopoiesis (EMH) is induced in spleens due to various diseases. The aim of this study is to investigate the role of spleen in cyclophosphamide (CTX)-induced hematosuppression and EMH in mice. Balb/c mice were IP injected with 300 mg/kg CTX 2 weeks after splenectomy or sham operation and randomly sacrificed 1, 3, 7, 14, and 21 days after injection. Blood samples were collected, and spleens were weighed, histologically analyzed, and then used for flow cytometry. There were significant differences in white blood count, red blood count, platelet numbers and hemoglobin concentration between the splenectomized and sham-operated mice after CTX injection. The cellularity of the spleen was reduced 3 days following CTX treatment but then rose 7 days after CTX treatment. The numbers of colony-forming units in the spleen reached a peak 7 days after CTX injection, then declined. Flow cytometry demonstrated the percentage of CD34(+) and CD117(+) cells in the spleen increased 7 days after CTX injection, indicating the hematopoietic stem and progenitor cells in the spleen. The study indicates that EMH occurs as a compensatory reaction to CTX-induced hematosuppression in the murine spleen, implying that conservation of the spleen may promote the recovery of cancer patients from chemotherapy-induced hematosuppression.

  8. Extramedullary hematopoiesis of the liver in a child with sickle cell disease: A rare complication.

    Science.gov (United States)

    Barrier, Angela; Willy, Simo; Slone, Jeremy S

    2015-08-01

    We present the case of a 7-year-old Cameroonian girl with sickle cell disease (SCD) who presented with progressive abdominal distension, fever, severe anemia, respiratory distress, and fatigue. Abdominal ultrasound showed a 15.3 cm × 11.5 cm × 15.5 cm solid echogenic mass within the left lobe of the liver. Fine-needle aspiration showed features of extramedullary hematopoiesis (EMH). Despite transfusions, antibiotics, and initiation of hydroxyurea the patient died of respiratory failure during the hospital stay. There is a paucity of information on EMH in the pediatric sickle cell population, especially from resource-limited settings such as western Africa. EMH, however, is a known complication of SCD and should be considered in patients presenting with mass lesions in the setting of chronic anemia. With limited therapeutic interventions for EMH, including radiation and hydroxyurea, the emphasis should be on improving overall treatment of patients with chronic and untreated hemolytic anemia, especially in low-income countries. © 2015 Japan Pediatric Society.

  9. Helical tomotherapy for extramedullary hematopoiesis involving the pericardium in a patient with chronic myeloid leukemia.

    Science.gov (United States)

    Toms, Daniel R; Cannick, Leander; Stuart, Robert K; Jenrette, Joseph M; Terwiliger, Lacy

    2010-07-01

    Extramedullary hematopoiesis (EMH) refers to the development of foci of hematopoiesis outside its normal location in the bone marrow. This occurs normally during fetal development but is abnormal postpartum. The most common sites of EMH are the spleen and liver. The phenomenon occurs in a number of disease states, notably in myelofibrosis, thalassemia, immune thrombocytopenic purpura, sickle cell anemia, polycythemia vera, and myelodysplastic syndrome. Affected patients often develop symptoms related to the location of the EMH. Reported treatments include red blood cell transfusions, surgical excision, decompressive laminectomy in cases of cord compression, chemotherapy, and irradiation. Radiation therapy is highly effective for treating hematopoietic tissue because such tissues are extremely radiosensitive. Megavoltage helical tomotherapy is a technical advance in the delivery of radiation therapy, allowing more conformal and precise treatments. The present case report describes a patient with the diagnosis of atypical chronic myeloid leukemia and myelofibrosis who subsequently developed EMH of the pericardium with effusion and tamponade. By utilizing tomotherapy we were able to treat the pericardium while sparing much of the myocardium. The patient tolerated treatment well without acute adverse effects. His symptoms were alleviated, but he died approximately 1 year later.

  10. 27-Hydroxycholesterol induces hematopoietic stem cell mobilization and extramedullary hematopoiesis during pregnancy.

    Science.gov (United States)

    Oguro, Hideyuki; McDonald, Jeffrey G; Zhao, Zhiyu; Umetani, Michihisa; Shaul, Philip W; Morrison, Sean J

    2017-09-01

    Extramedullary hematopoiesis (EMH) is induced during pregnancy to support rapid expansion of maternal blood volume. EMH activation requires hematopoietic stem cell (HSC) proliferation and mobilization, processes that depend upon estrogen receptor α (ERα) in HSCs. Here we show that treating mice with estradiol to model estradiol increases during pregnancy induced HSC proliferation in the bone marrow but not HSC mobilization. Treatment with the alternative ERα ligand 27-hydroxycholesterol (27HC) induced ERα-dependent HSC mobilization and EMH but not HSC division in the bone marrow. During pregnancy, 27HC levels increased in hematopoietic stem/progenitor cells as a result of CYP27A1, a cholesterol hydroxylase. Cyp27a1-deficient mice had significantly reduced 27HC levels, HSC mobilization, and EMH during pregnancy but normal bone marrow hematopoiesis and EMH in response to bleeding or G-CSF treatment. Distinct hematopoietic stresses thus induce EMH through different mechanisms. Two different ERα ligands, estradiol and 27HC, work together to promote EMH during pregnancy, revealing a collaboration of hormonal and metabolic mechanisms as well as a physiological function for 27HC in normal mice.

  11. Chronic Idiopathic Myelofibrosis Presenting as Cauda Equina Compression due to Extramedullary Hematopoiesis: A Case Report

    Science.gov (United States)

    Goh, Duck-Ho; Cho, Dae-Chul; Park, Seong-Hyun; Hwang, Jeong-Hyun; Sung, Joo-Kyung

    2007-01-01

    Extramedullary hematopoiesis (EMH) is occasionally reported in idiopathic myelofibrosis and is generally found in the liver, spleen, and lymph nodes several years after diagnosis. Myelofibrosis presenting as spinal cord compression, resulting from EMH tissue is very rare. A 39-yr-old man presented with back pain, subjective weakness and numbness in both legs. Sagittal magnetic resonance imaging showed multiple anterior epidural mass extending from L4 to S1 with compression of cauda equina and nerve root. The patient underwent gross total removal of the mass via L4, 5, and S1 laminectomy. Histological analysis showed islands of myelopoietic cells surrounded by fatty tissue, consistent with EMH, and bone marrow biopsy performed after surgery revealed hypercellular marrow and megakaryocytic hyperplasia and focal fibrosis. The final diagnosis was chronic idiopathic myelofibrosis leading to EMH in the lumbar spinal canal. Since there were no abnormal hematological findings except mild myelofibrosis, additional treatment such as radiothepary was not administered postoperatively for fear of radiotoxicity. On 6 month follow-up examination, the patient remained clinically stable without recurrence. This is the first case of chronic idiopathic myelofibrosis due to EMH tissue in the lumbar spinal canal in Korea. PMID:18162730

  12. The protumorigenic potential of FTY720 by promoting extramedullary hematopoiesis and MDSC accumulation.

    Science.gov (United States)

    Li, Y; Zhou, T; Wang, Y; Ning, C; Lv, Z; Han, G; Morris, J C; Taylor, E N; Wang, R; Xiao, H; Hou, C; Ma, Y; Shen, B; Feng, J; Guo, R; Li, Y; Chen, G

    2017-06-29

    FTY720 (also called fingolimod) is recognized as an immunosuppressant and has been approved by the Food and Drug Administration to treat refractory multiple sclerosis. However, long-term administration of FTY720 potentially increases the risk for cancer in recipients. The underlying mechanisms remain poorly understood. Herein, we provided evidence that FTY720 administration potentiated tumor growth. Mechanistically, FTY720 enhanced extramedullary hematopoiesis and massive accumulation of myeloid-derived suppressor cells (MDSCs), which actively suppressed antitumor immune responses. Granulocyte-macrophage colony-stimulating factor (GM-CSF), mainly produced by MDSCs, was identified as a key factor to mediate these effects of FTY720 in tumor microenvironment. Furthermore, we showed that FTY720 triggers MDSCs to release GM-CSF via S1P receptor 3 (S1pr3) through Rho kinase and extracellular signal-regulated kinase-dependent pathway. Thus, our findings provide mechanistic explanation for the protumorigenic potentials of FTY720 and suggest that targeting S1pr3 simultaneously may be beneficial for the patients receiving FTY720 treatment.

  13. CXCR4 Regulates Extra-Medullary Myeloma through Epithelial-Mesenchymal-Transition-like Transcriptional Activation

    Directory of Open Access Journals (Sweden)

    Aldo M. Roccaro

    2015-07-01

    Full Text Available Extra-medullary disease (EMD in multiple myeloma (MM is associated with poor prognosis and resistance to chemotherapy. However, molecular alterations that lead to EMD have not been well defined. We developed bone marrow (BM- and EMD-prone MM syngeneic cell lines; identified that epithelial-to-mesenchymal transition (EMT transcriptional patterns were significantly enriched in both clones compared to parental cells, together with higher levels of CXCR4 protein; and demonstrated that CXCR4 enhanced the acquisition of an EMT-like phenotype in MM cells with a phenotypic conversion for invasion, leading to higher bone metastasis and EMD dissemination in vivo. In contrast, CXCR4 silencing led to inhibited tumor growth and reduced survival. Ulocuplumab, a monoclonal anti-CXCR4 antibody, inhibited MM cell dissemination, supported by suppression of the CXCR4-driven EMT-like phenotype. These results suggest that targeting CXCR4 may act as a regulator of EMD through EMT-like transcriptional modulation, thus representing a potential therapeutic strategy to prevent MM disease progression.

  14. 27-Hydroxycholesterol induces hematopoietic stem cell mobilization and extramedullary hematopoiesis during pregnancy

    Science.gov (United States)

    McDonald, Jeffrey G.; Shaul, Philip W.; Morrison, Sean J.

    2017-01-01

    Extramedullary hematopoiesis (EMH) is induced during pregnancy to support rapid expansion of maternal blood volume. EMH activation requires hematopoietic stem cell (HSC) proliferation and mobilization, processes that depend upon estrogen receptor α (ERα) in HSCs. Here we show that treating mice with estradiol to model estradiol increases during pregnancy induced HSC proliferation in the bone marrow but not HSC mobilization. Treatment with the alternative ERα ligand 27-hydroxycholesterol (27HC) induced ERα-dependent HSC mobilization and EMH but not HSC division in the bone marrow. During pregnancy, 27HC levels increased in hematopoietic stem/progenitor cells as a result of CYP27A1, a cholesterol hydroxylase. Cyp27a1-deficient mice had significantly reduced 27HC levels, HSC mobilization, and EMH during pregnancy but normal bone marrow hematopoiesis and EMH in response to bleeding or G-CSF treatment. Distinct hematopoietic stresses thus induce EMH through different mechanisms. Two different ERα ligands, estradiol and 27HC, work together to promote EMH during pregnancy, revealing a collaboration of hormonal and metabolic mechanisms as well as a physiological function for 27HC in normal mice. PMID:28783041

  15. Use of Ir192 interstitial brachytherapy for an equine malignant dermal schwannoma : clinical communication

    Directory of Open Access Journals (Sweden)

    M.N. Saulez

    2009-05-01

    Full Text Available A 10-year-old Hanoverian mare was evaluated for a right buccal swelling that recurred 3 months following surgical resection. Ultrasonographic examination showed a broadly pedunculated subcutaneous mass at the level of 106-109 and 406-409 cheek teeth associated with an erosive mucosal lesion on the inside of the cheek. Histological examination of a biopsy specimen revealed a well-demarcated, malignant, dermal schwannoma. Following subcutaneous placement of platinum coated Ir192 wires under general anaesthesia, low-dose radiation of 5 gray per day was delivered for 14 days. Short-term complications included loss of patency of the right nasolacrimal duct, erythema, dermatitis, leukotrichia and left-sided deviation of the muzzle. Ten months later, there has been no tumour recurrence. Findings suggest that the use of interstitial brachytherapy should be considered for a malignant, dermal schwannoma that has recurred or is not amenable to surgery.

  16. Malignant Schwannomas of the Ethmoid Sinus and the Larynx-Case Report and Review of Literatures

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Kyu Chan; Choi, Myung Sun [Korea University College of Medicine, Seoul (Korea, Republic of)

    1992-06-15

    The incidence of malignant schwannoma in the head and neck is extremely rate. Most tumors appear as a rapidly expanding nonpainful mass and the symptoms are usually attributable to local expansion of the mass. About one half occurs in association with von Recklinghausen disease. Wide surgical excision is generally recommended as a primary treatment. Recently, there has been a trend to include postoperative radiation therapy as a primary modality. Prognosis of head and neck malignant schwannoma has been reported as particularly poor. However, recent authors advocate that prolonged survival is possible after adequate therapy including postoperative radiation therapy. We present our experience with these tumors on very rare locations such as the ethmoid sinus and the larynx, with the review of literatures.

  17. Synchronous triple occurrence of MALT lymphoma, schwannoma, and adenocarcinoma of the stomach.

    Science.gov (United States)

    Choi, Kyeong W; Joo, Mee; Kim, Han S; Lee, Woo Y

    2017-06-14

    We present a case of a 56-year-old man with 3 synchronous gastric tumors. The patient presented with melena, and 3 gastric abnormalities were detected on gastroduodenoscopic examination, including a small ulcerative lesion in the gastric antrum, a submucosal mass in the gastric body, and severe erosion in the fundus. Histological examination of biopsy samples yielded respective diagnoses of gastric adenocarcinoma, gastritis, and mucosa-associated lymphoid tissue (MALT) lymphoma. The patient first received medication to eradicate any underlying Helicobacter pylori infection, which might have been a cause of the MALT lymphoma. Four weeks later, after examination of repeat biopsy samples revealed that the MALT lymphoma had resolved, the patient underwent subtotal gastrectomy. Further histological examination of resected tissue confirmed the antrum lesion as adenocarcinoma and the body lesion as schwannoma. To our knowledge, this is the first reported case of synchronous triple primary gastric adenocarcinoma, MALT lymphoma, and schwannoma.

  18. Intrameatal thrombosed anterior inferior cerebellar artery aneurysm mimicking a vestibular schwannoma.

    Science.gov (United States)

    Päsler, Dennis; Baldauf, Jörg; Runge, Uwe; Schroeder, Henry W S

    2011-04-01

    Aneurysms of the anterior inferior cerebellar artery (AICA) are a rare entity. Purely intrameatal aneurysms are even rarer. The authors report an intrameatal thrombosed AICA aneurysm mimicking a vestibular schwannoma that was treated by resection and end-to-end anastomosis. This 22-year-old man presented with acute hearing loss, vertigo, and moderate facial palsy. Magnetic resonance imaging showed an atypical intrameatal lesion with dilation of the internal auditory canal. Microsurgical inspection via a retrosigmoid approach and drilling of the posterior wall of the internal auditory canal revealed a thrombosed AICA aneurysm. The aneurysm was excised, and an end-to-end suture was performed to restore AICA continuity. Intraoperative indocyanine green videoangiography as well as postoperative digital substraction angiography showed a good revascularization. Intrameatal AICA aneurysms may present with symptoms similar to vestibular schwannomas. End-to-end reanastomosis after aneurysm resection is a treatment option when clipping is impossible.

  19. Transjugular intrahepatic porto-systemic stent-shunt for therapy of bleeding esophageal varices due to extramedullary hematopoiesis in primary myelofibrosis: a case report.

    Science.gov (United States)

    Phillip, Veit; Berger, Hermann; Straub, Melanie; Saugel, Bernd; Treiber, Matthias; Einwächter, Henrik; Schmid, Roland M; Huber, Wolfgang

    2012-01-01

    Primary myelofibrosis belongs to the group of myeloproliferative syndromes. Extramedullary hematopoiesis in the liver can lead to portal hypertension. We report a case of a patient with life-threatening, endoscopically not treatable bleeding from esophageal varices due to extramedullary hematopoiesis of the liver that was successfully treated with placement of a transjugular intrahepatic porto-systemic stent-shunt (TIPS). Therapy of variceal bleeding by TIPS insertion was successful. During a 29-month follow-up, no hepatic failure, hepatic encephalopathy, or further variceal bleeding episode occurred. TIPS placement is a well-established procedure for the treatment of complications due to portal hypertension mainly due to liver cirrhosis. This report illustrates that TIPS placement can also be a promising treatment option in patients with primary myelofibrosis and portal hypertension due to extramedullary hematopoiesis. Copyright © 2012 S. Karger AG, Basel.

  20. Long-term quality of life and tumour control following gamma knife radiosurgery for vestibular schwannoma

    DEFF Research Database (Denmark)

    Wangerid, Theresa; Bartek, Jiri; Svensson, Mikael

    2014-01-01

    Gamma knife radiosurgery (GKRS) has for the last decades been an established treatment option for patients with small- or medium-sized vestibular schwannomas (VS), although little data is reported on long-term outcome regarding quality of life (QOL) and tumour control in this patient category....... The objective of this study was to investigate long-term QOL and tumour control in GKRS-treated VS patients at our institution....

  1. Benign Ancient Schwannoma of the abdominal wall: An unwanted birthday present

    Directory of Open Access Journals (Sweden)

    Ram Manisha

    2010-01-01

    Full Text Available Abstract Background There has been a recent growth in the use of whole body Computerised Tomography (CT scans in the private sector as a screening test for asymptomatic disease. This is despite scant evidence to show any positive effect on morbidity or mortality. There has been concern raised over the possible harms of the test in terms of radiation exposure as well as the risk and anxiety of further investigation and treatment for the large numbers of benign lesions identified. Case Presentation A healthy 64 year old lady received a privately funded whole body CT scan for her birthday which revealed an incidental mass in the right iliac fossa. This was investigated with further imaging and colonoscopy and as confident diagnosis could not be made, eventually excised. Histology demonstrated this to be a benign ancient schwannoma and we believe this to be the first reported case of an abdominal wall schwannoma in the English literature Conclusions Ancient schwannomas are rare tumours of the peripheral nerve sheaths more usually found in the head, neck and flexor surfaces of extremities. They are a subtype of classical schwannomas with a predominance of degenerative changes. Our case highlights the pitfalls of such screening tests in demonstrating benign disease and subjecting patients to what turns out to be unnecessary invasive investigation and treatment. It provides evidence as to the consequences of the large number of false positive results that are created by blind CT scanning of asymptomatic patients i.e. its tendency to detect pseudodiesease rather than affect survival rates. Should the number of scans increase there may be an unnecessary burden on NHS resources due to the large numbers of benign lesions picked up, that are then referred for further investigation.

  2. Angiogenesis in vestibular schwannomas: expression of extracellular matrix factors MMP-2, MMP-9, and TIMP-1

    DEFF Research Database (Denmark)

    Møller, Martin Nue; Werther, Kim; Nalla, Amarnadh

    2010-01-01

    targets the angiogenic process by investigation of tumor expression of MMP-2, MMP-9, and tissue inhibitors of metalloproteinase (TIMP)-1. A possible correlation with gender, patient age, symptom duration, tumor size, and the absolute and relative growth rate is explored.......Vascular endothelial growth factor (VEGF) and matrix metalloproteinases (MMPs) are potent mediators of tumor angiogenesis. It has been demonstrated that vestibular schwannoma VEGF expression correlates with tumor growth pattern, whereas knowledge on the expression of MMPs is lacking. This study...

  3. Therapeutic profile of single-fraction radiosurgery of vestibular schwannoma: unrelated malignancy predicts tumor control.

    Science.gov (United States)

    Wowra, Berndt; Muacevic, Alexander; Fürweger, Christoph; Schichor, Christian; Tonn, Jörg-Christian

    2012-07-01

    Radiosurgery has become an accepted treatment option for vestibular schwannomas. Nevertheless, predictors of tumor control and treatment toxicity in current radiosurgery of vestibular schwannomas are not well understood. To generate new information on predictors of tumor control and cranial nerve toxicity of single-fraction radiosurgery of vestibular schwannomas, we conducted a single-institution long-term observational study of radiosurgery for sporadic vestibular schwannomas. Minimum follow-up was 3 years. Investigated as potential predictors of tumor control and cranial nerve toxicity were treatment technology; tumor resection preceding radiosurgery; tumor size; gender; patient age; history of cancer, vascular disease, or metabolic disease; tumor volume; radiosurgical prescription dose; and isodose line. Three hundred eighty-six patients met inclusion criteria. Treatment failure was observed in 27 patients. History of unrelated cancer (strongest predictor) and prescription dose significantly predicted tumor control. The cumulative incidence of treatment failure was 30% after 6.5 years in patients with unrelated malignancy and 10% after ≥15 years in patients without such cancer (P < .02). Tumor volume was the only predictor of trigeminal neuropathy (observed in 6 patients). No predictor of facial nerve toxicity was found. On the House and Brackmann scale, 1 patient had a permanent one-level drop and 7 a transient drop of 1 to 3 levels. Serviceable hearing was preserved in 75.1%. Tumor hearing before radiosurgery, recurrence, and prescription isodose predicted ototoxicity. Unrelated malignancy is a strong predictor of tumor control. Tumor recurrence predominantly predicts ototoxicity. These findings potentially will aid future clinical decision making in ambiguous cases.

  4. Gastric malignant schwannoma presenting with upper gastrointestinal bleeding: a case report

    OpenAIRE

    Takemura, Masashi; Yoshida, Kayo; Takii, Mamiko; Sakurai, Katsunobu; Kanazawa, Akishige

    2012-01-01

    Abstract Introduction We report a case of gastric malignant schwannoma presenting with gastrointestinal bleeding. Case presentation A 70-year-old Japanese man presented with gastrointestinal bleeding to our hospital. Gastrointestinal endoscopy revealed a protruding lesion in the gastric body. Hematoxylin and eosin staining of biopsy specimens from this lesion revealed sheets of spindle cells. Immunohistochemistry revealed that these cells were positive for S-100 protein and negative for c-Kit...

  5. Endoscopic interventional treatment for gastric schwannoma: a single-center experience.

    Science.gov (United States)

    Li, Bin; Liang, Tiejun; Wei, Lili; Ma, Mingze; Huang, Ya; Xu, Hongwei; Shi, Xiuju; Qin, Chengyong

    2014-01-01

    Endoscopic Interventional Treatment is of little trauma and less complications in the treatment of gastric schwannoma and leads to faster recovery and fewer days of hospitalization. This study was aimed to investigate the safety and efficacy of endoscopic interventional therapy for gastric schwannoma, including endoscopic submucosal excavation, non-laparoscopic-assisted endoscopic full-thickness resection, endoscopic tunneling submucosal resection, and so on. Six patients of gastric schwannoma diagnosed by pathology examination were retrospectively analyzed ranging from Oct 2011 to Feb 2014 at Shandong Provincial Hospital affiliated to Shandong University. Five of the six patients accepted endoscopic interventional therapy. Among the five patients, there were four males and one female, aged from 48 to 65 years old (the average age was 58 ± 6.4). The lesions located at the fundus, the fundus-cardia, gastric body or gastric antrum, respectively, with the diameters ranged from 8 to 25 millimeter (the average was 17.1 ± 7.8 mm). All the patients were performed endoscopic interventional therapy successfully. Among five patients, one patient was treated by endoscopic tunneling submucosal resection, two by endoscopic submucosal excavation, and the other two were given endoscopic full-thickness resection. Operation duration was about 43 to 83 minutes (the average was 57.6 ± 16.1 minutes). The mass were completely removed, with limited bleeding. During the operation, perforation and pneumoperitoneum occurred in two patients, who finally recovered by endoscopic and conservative treatment. No bleeding, inflammation or infection occurred in these patients. The average follow-up time was (7.4 ± 4.4) months. Neither recurrence nor metastasis was found during follow-up. Endoscopic interventional therapy is a safe and effective treatment for gastric schwannoma.

  6. The clinical characteristics and treatment for sudden sensorineural hearing loss with vestibular schwannoma.

    Science.gov (United States)

    Lin, Chang; Gong, Qilin; Zuo, Wenjing; Zhang, Rong; Zhou, Aidong

    2015-04-01

    The aim of this study is to analyze the clinical characteristics and treatment of sudden sensorineural hearing loss (SSNHL) patients with vestibular schwannoma (VS). The clinical features of the VS patients were explored by retrospectively analyzing the clinical data from 542 cases of SSNHL patients between January 2008 and March 2013. There were 10 cases (10 ears) diagnosed with VS in 542 cases of SSNHL patients (10 ears, 1.85 %), 3 males, 7 females, with a range of 28-57 years. Among all the cases, eight patients with abnormal ABR, ten with ear ipsilateral stapedius reflexes which were completely not elicited and seven patients with healthy ear contralateral stapedius reflexes which were completely not elicited. Neuromas were classified by Koos grades according to size (8 of grade I, 1 of grade II, 1 of grade IV). Eight small VS  patients were taken waiting and MRI therapy strategies. Meanwhile, we used glucocorticoid treatment and timely and short-term medication to improve the microcirculation of the inner ear for these patients. And four cases' hearing was improved. Some vestibular schwannomas have SSNHL as initial symptoms, especially the small ones in internal auditory canal. To prevent misdiagnosis or leak-diagnosis, MRI should be performed as a routine test for SSNHL, and ABR is sometimes necessary for SSNHL patients. It is also necessary to give appropriate treatment to protect hearing of the small vestibular schwannoma patients whose first symptoms are diagnosed as SSNHL in acute phase.

  7. A Lip Lump: An Unexpected Histological Diagnosis of a Lip Schwannoma

    Directory of Open Access Journals (Sweden)

    Thomas Haigh

    2017-01-01

    Full Text Available Schwannomas are benign nerve sheath tumours arising from Schwann cells. They comprise 1% of all benign tumours. In the 2016 World Health Organisation Classification of Central Nervous System, they are classified as a tumour of the cranial and paraspinal nerves, Schwannoma 9560/0. A 23-year-old Caucasian lady presented with a seven-month history of a painless right upper lip lump. Examination revealed a small cystic 0.5 cm diameter lesion within the right upper lip. The clinical impression was that of a mucocele. Excision of the lip lesion was performed under local anaesthetic. Histological examination of the excised lesion demonstrated a circumscribed nodule consisting of spindle cells mixed with vascular spaces containing red blood cells and fibrin. Immunohistochemistry for S100 was strongly positive. The findings were consistent with that of a small benign schwannoma. The current consensus is for surgical excision of a conservative nature with no need for margins. If recurrence does occur one needs to consider whether complete enucleation was achieved or whether malignant transformation has occurred.

  8. Unique Surgical Issues in the Management of a Giant Retroperitoneal Schwannoma and Brief Review of Literature

    Directory of Open Access Journals (Sweden)

    Santhosh Kuriakose

    2014-01-01

    Full Text Available Ancient Schwannoma, though benign, can cause diagnostic dilemma because of its clinical presentation and imaging features. We report the management of a giant retroperitoneal schwannoma in a 19-year-old young lady who presented with lower abdominal distension. CT scan reported a large heterogenous lesion in the abdominopelvic retroperitoneum (42 cm × 16 cm × 16 cm as a malignant tumor. The unique problems we encountered were the enormous size, the location of major part of the tumor in the pelvis, the need for fertility preservation, the external iliac vessels stretching over the tumor making mobilization surgically demanding, and the prospects of neurological deficits. An en bloc resection of schwannoma with common iliac, external iliac and internal iliac veins, internal iliac artery, femoral and obturator nerves, and iliopsoas muscle was done maintaining oncological principles. External iliac artery that was cut to facilitate tumor mobilization was reanastomosed at the end of the procedure. Postoperatively patient had uneventful recovery with patchy sensory loss, foot drop, and quadriceps weakness which was rehabilitated with a foot drop splint and active physiotherapy.

  9. Unique surgical issues in the management of a giant retroperitoneal schwannoma and brief review of literature.

    Science.gov (United States)

    Kuriakose, Santhosh; Vikram, Syam; Salih, Surij; Balasubramanian, Satheesan; Mangalasseri Pareekutty, Nizamudeen; Nayanar, Sangeetha

    2014-01-01

    Ancient Schwannoma, though benign, can cause diagnostic dilemma because of its clinical presentation and imaging features. We report the management of a giant retroperitoneal schwannoma in a 19-year-old young lady who presented with lower abdominal distension. CT scan reported a large heterogenous lesion in the abdominopelvic retroperitoneum (42 cm × 16 cm × 16 cm) as a malignant tumor. The unique problems we encountered were the enormous size, the location of major part of the tumor in the pelvis, the need for fertility preservation, the external iliac vessels stretching over the tumor making mobilization surgically demanding, and the prospects of neurological deficits. An en bloc resection of schwannoma with common iliac, external iliac and internal iliac veins, internal iliac artery, femoral and obturator nerves, and iliopsoas muscle was done maintaining oncological principles. External iliac artery that was cut to facilitate tumor mobilization was reanastomosed at the end of the procedure. Postoperatively patient had uneventful recovery with patchy sensory loss, foot drop, and quadriceps weakness which was rehabilitated with a foot drop splint and active physiotherapy.

  10. Communicating Hydrocephalus Associated with Intracranial Schwannoma Treated by Gamma Knife Radiosurgery.

    Science.gov (United States)

    Park, Chang Kyu; Lee, Sung Ho; Choi, Man Kyu; Choi, Seok Keun; Park, Bong Jin; Lim, Young Jin

    2016-05-01

    Gamma knife radiosurgery (GKRS) has been established as an effective and safe treatment for intracranial schwannoma. However, serious complications can occur after GKRS, including hydrocephalus. The pathophysiology and risk factors of this disorder are not yet fully understood. The objective of the study was to assess potential risk factors for hydrocephalus after GKRS. We retrospectively reviewed the medical radiosurgical records of 244 patients who underwent GKRS to treat intracranial schwannoma. The following parameters were analyzed as potential risk factors for hydrocephalus after GKRS: age, sex, target volume, irradiation dose, prior tumor resection, treatment technique, and tumor enhancement pattern. The tumor enhancement pattern was divided into 2 groups: group A (homogeneous enhancement) and group B (heterogeneous or rim enhancement). Of the 244 patients, 14 of them (5.7%) developed communicating hydrocephalus. Communicating hydrocephalus occurred within 2 years after GKRS in most patients (92.8%). No significant association was observed between any of the parameters investigated and the development of hydrocephalus, with the exception of tumor enhancement pattern. Group B exhibited a statistically significant difference by univariate analysis (P = 0.002); this difference was also significant by multivariate analysis (P = 0.006). Because hydrocephalus is curable, patients should be closely monitored for the development of this disorder after GKRS. In particular, patients with intracranial schwannomas with irregular enhancement patterns or cysts should be meticulously observed. Copyright © 2015 Elsevier Inc. All rights reserved.

  11. Gastric schwannoma in a female patient with pulmonary tuberculosis - a clinicopathological assessment and diagnosis.

    Science.gov (United States)

    Tahir, Tariq Mahmood; Anwar, Sadia; Naseem, Nadia; Mansoor-Ul-Haq, Hafiz; Saqib, Muhammad

    2010-04-01

    Schwannomas, or neurinomas, are generally benign, slow-growing, asymptomatic neoplasms originating from the Schwann cells of a nerve sheath. As a part of spindle cell mesenchymal tumours, schwannomas arising from the gastrointestinal tract (GIT) are unusual; however, when they occur, the most common site involved is the stomach, which represents 0.2% of all gastric tumours. We report the case of a 35-year-old female patient with a history of pulmonary tuberculosis presenting with a large palpable abdominal mass reaching up to the peritoneal cavity. The initial clinical impression was a tuberculous abdominal mass, a cyst, or a teratoma. However, intra-operative findings during a subtotal gastrectomy revealed an exophytic gastric serosal mass, which suggested a gastrointestinal stromal tumour (GIST). Post-operative histopathological findings showed a fascicular arrangement of neoplastic spindle cells with pallisading nuclei that showed intense positivity for S-100 protein, and were negative for CD117 and desmin in immunohistochemistry studies. These results confirmed the final diagnosis of a gastric schwannoma.

  12. Gastric Schwannoma in a Female Patient with Pulmonary Tuberculosis — A Clinicopathological Assessment and Diagnosis

    Science.gov (United States)

    Tahir, Tariq Mahmood; Anwar, Sadia; Naseem, Nadia; Mansoor-Ul-Haq, Hafiz; Saqib, Muhammad

    2010-01-01

    Schwannomas, or neurinomas, are generally benign, slow-growing, asymptomatic neoplasms originating from the Schwann cells of a nerve sheath. As a part of spindle cell mesenchymal tumours, schwannomas arising from the gastrointestinal tract (GIT) are unusual; however, when they occur, the most common site involved is the stomach, which represents 0.2% of all gastric tumours. We report the case of a 35-year-old female patient with a history of pulmonary tuberculosis presenting with a large palpable abdominal mass reaching up to the peritoneal cavity. The initial clinical impression was a tuberculous abdominal mass, a cyst, or a teratoma. However, intra-operative findings during a subtotal gastrectomy revealed an exophytic gastric serosal mass, which suggested a gastrointestinal stromal tumour (GIST). Post-operative histopathological findings showed a fascicular arrangement of neoplastic spindle cells with pallisading nuclei that showed intense positivity for S-100 protein, and were negative for CD117 and desmin in immunohistochemistry studies. These results confirmed the final diagnosis of a gastric schwannoma. PMID:22135537

  13. mTORC1 inhibition delays growth of neurofibromatosis type 2 schwannoma

    Science.gov (United States)

    Giovannini, Marco; Bonne, Nicolas-Xavier; Vitte, Jeremie; Chareyre, Fabrice; Tanaka, Karo; Adams, Rocky; Fisher, Laurel M.; Valeyrie-Allanore, Laurence; Wolkenstein, Pierre; Goutagny, Stephane; Kalamarides, Michel

    2014-01-01

    Background Neurofibromatosis type 2 (NF2) is a rare autosomal dominant genetic disorder, resulting in a variety of neural tumors, with bilateral vestibular schwannomas as the most frequent manifestation. Recently, merlin, the NF2 tumor suppressor, has been identified as a novel negative regulator of mammalian target of rapamycin complex 1 (mTORC1); functional loss of merlin was shown to result in elevated mTORC1 signaling in NF2-related tumors. Thus, mTORC1 pathway inhibition may be a useful targeted therapeutic approach. Methods We studied in vitro cell models, cohorts of mice allografted with Nf2−/− Schwann cells, and a genetically modified mouse model of NF2 schwannoma in order to evaluate the efficacy of the proposed targeted therapy for NF2. Results We found that treatment with the mTORC1 inhibitor rapamycin reduced the severity of NF2-related Schwann cell tumorigenesis without significant toxicity. Consistent with these results, in an NF2 patient with growing vestibular schwannomas, the rapalog sirolimus induced tumor growth arrest. Conclusions Taken together, these results constitute definitive evidence that justifies proceeding with clinical trials using mTORC1-targeted agents in selected patients with NF2 and in patients with NF2-related sporadic tumors. PMID:24414536

  14. Hybrid neurofibroma/schwannoma is overrepresented among schwannomatosis and neurofibromatosis patients.

    Science.gov (United States)

    Harder, Anja; Wesemann, Martin; Hagel, Christian; Schittenhelm, Jens; Fischer, Susan; Tatagiba, Marcos; Nagel, Christoph; Jeibmann, Astrid; Bohring, Axel; Mautner, Victor-Felix; Paulus, Werner

    2012-05-01

    We analyzed the histologic features of peripheral nerve sheath tumors occurring in 14 patients with schwannomatosis. Among a total of 31 tumors, 19 tumors (61%) showed schwannoma-like nodules within a neurofibroma-like tumor, corresponding to hybrid neurofibroma/schwannoma. At least 1 hybrid tumor occurred in 10 of 14 (71%) schwannomatosis patients. We then retrieved cases of hybrid tumors without documented relation to schwannomatosis from our database and identified 41 tumors arising in 23 patients. More than half of these patients (14/23) were reported to suffer from multiple peripheral nerve sheath tumors, favoring a tumor syndrome. Indeed, analysis of clinical records revealed the diagnosis of neurofibromatosis type 2 (NF2) in 26% (6/23), neurofibromatosis type 1 (NF1) in 9% (2/23), definite schwannomatosis in 4% (1/23), and possible schwannomatosis in 13% (3/23) of patients with multiple nerve sheath tumors. Our findings suggest that hybrid neurofibroma/schwannoma represents a common tumor type in schwannomatosis and shows a striking association with neurofibromatoses.

  15. Vestibular compensation after vestibular schwannoma surgery: normalization of the subjective visual vertical and disability.

    Science.gov (United States)

    Batuecas-Caletrio, Angel; Santacruz-Ruiz, Santiago; Muñoz-Herrera, Angel; Sousa, Pablo; Otero, Alvaro; Perez-Fernandez, Nicolas

    2013-05-01

    The degree of caloric weakness before surgery influences faster or slower recovery of patients undergoing vestibular schwannoma surgery. The Dizziness Handicap Inventory (DHI) is a good index to show the recovery of patients as it relates directly to an improvement or not of the subjective visual vertical (SVV). To evaluate the process of recovery of patients as measured by the SVV and the DHI after surgical removal of vestibular schwannoma. We studied 24 consecutive patients of the University Hospital of Salamanca who underwent vestibular schwannoma surgery. We assessed age, tumour size, degree of canalicular weakness and preoperative SVV, and their relationship with DHI and SVV at discharge and also at 1, 3 and 6 months postoperatively. Patients with lesser degrees of caloric weakness took longer to normalize SVV than those with a higher caloric weakness before surgery (p < 0.05). There was a significant correlation between DHI and improvements in SVV with time. The differences disappeared in 6 months where all patients, with greater or lesser degree of caloric weakness, had the same results.

  16. Intra-extramedullary drainage as an effective option for treatment of intramedullary ependymal cyst of thoracic spine: technical note.

    Science.gov (United States)

    Landi, Alessandro; Pietrantonio, Andrea; Marotta, Nicola; Mancarella, Cristina; Delfini, Roberto

    2014-03-01

    Intramedullary neuroepithelial cysts are extremely rare and only 15 cases have been reported in the literature. Clinico-radiological features are not indicative of a specific diagnosis; for this reason, diagnosis is based mainly on the histological features. In the literature, total surgical removal is considered the treatment of choice. The risk of recurrence is higher after partial removal and in cases of occlusion of intra-extramedullary shunt. For this reason, a surgical strategy that ensures the shunt patency in case of incomplete removal of the cyst becomes a very safe option for treatment of this pathology. We report the case of a 51-year-old woman who was found to have a dorsal (D9) intramedullary neuroepithelial cyst. She underwent surgical treatment with partial removal and placement of a Nelaton drainage device (8 French) inside the intra-extramedullary shunt. The patient experienced a complete regression of preoperative symptoms and magnetic resonance imaging (MRI) follow-up showed no radiological evidence of recurrence 24 months after surgical treatment. Spinal ependymal cysts show a high frequency of recurrence, especially in cases of partial removal of the cyst wall. Unfortunately, the cyst walls are often closely adherent to the spinal cord, making total removal impossible. Intra-extramedullary shunting is a viable option, although there is a high frequency of recurrence in cases of obstruction of the shunt. Placing an 8 Ch Nelaton drain between the dorsal columns is a reliable technique, especially in cases of partial removal. In fact, it allows continuous drainage of cyst fluid and subsequent resolution of symptoms, and it decreases the incidence of recurrences due to obstruction of the shunt. Georg Thieme Verlag KG Stuttgart · New York.

  17. Isolated Extramedullary Relapse of Acute Leukemia after Allogeneic Stem Cell Transplantation: Different Kinetics and Better Prognosis than Systemic Relapse.

    Science.gov (United States)

    Shem-Tov, Noga; Saraceni, Francesco; Danylesko, Ivetta; Shouval, Roni; Yerushalmi, Ronit; Nagler, Arnon; Shimoni, Avichai

    2017-07-01

    Allogeneic stem cell transplantation (SCT) is curative treatment in patients with acute leukemia and myelodysplastic syndrome. However, recurrent disease is the major cause of treatment failure. Isolated extramedullary relapse (iEMR) after SCT is relatively rare and not well characterized. We performed a retrospective analysis of 566 consecutive patients with acute myeloid leukemia (n = 446) and acute lymphoblastic leukemia (ALL; n = 120) after SCT to study the incidence, risk factors, treatment options, and outcome of iEMR. The 5-year cumulative incidence of bone marrow relapse (BMR) and iEMR was 41.0% and 5.8%, respectively. iEMR occurred significantly later than BMR at 10 and 4 months, respectively (P BMR but did not protect against iEMR. Most patients with iEMR received systemic treatment combined with local radiation and donor lymphocyte infusions when feasible. The 3-year survival after relapse was 8.5% and 30.1% after BMR and iEMR, respectively (P = .002). Patients with a first iEMR continued to have recurrent EMRs, and only a minority progressed to BMR. Second iEMR was also common after first BMR and associated with longer survival than second BMR. iEMR is more frequent in patients with ALL and prior extramedullary disease. It occurs later than BMR and more commonly in patients with chronic GVHD, suggesting less effective graft-versus-leukemia effect in extramedullary sites. Second iEMR is common after a first iEMR but also after a first BMR. Long-term survival is feasible with aggressive treatment. Copyright © 2017 The American Society for Blood and Marrow Transplantation. Published by Elsevier Inc. All rights reserved.

  18. The role of radiation therapy in the management of spinal cord compression due to extramedullary haematopoiesis in thalassaemia

    International Nuclear Information System (INIS)

    Singhal, S.; Sharma, S.; Dixit, S.; De, S.; Chander, S.; Rath, G.K.; Mehta, V.S.

    1992-01-01

    Extramedullary haematopoiesis associated with thalassaemia leading to spinal cord compression is an extremely rare event in the course of the disease. The efficacy of radiation therapy is advocated in the management of such a complication. Two patients with thalassaemia, who had presented with spinal cord compression, were successfully treated by a modest dose of local radiotherapy. In one of the patients, however, radiotherapy was resorted to after an initial decompressive laminectomy and partial removal of the intraspinal haematopoietic mass proved unsuccessful. The other patient was managed solely by radiation therapy. (Author)

  19. Hydroxyurea as a first-line treatment of extramedullary hematopoiesis in patients with beta thalassemia: Four case reports.

    Science.gov (United States)

    Karimi, Mehran; Cohan, Nader; Pishdad, Parisa

    2015-01-01

    Extramedullary hematopoiesis (EMH) is evidenced by erythropoietic masses, which occurs as a compensatory mechanism to overcome hypoxia during chronic anemia. EMH masses in spinal cord could lead to cord compression and neurological symptoms. Besides transfusion, radiotherapy, and surgery, hydroxyurea (HU) is also a treatment strategy in EMH. We described four cases of beta thalassemia with EMH who were treated with HU as a monotherapy. INTERVENTION (AND TECHNIQUE): HU therapy was done in all patients without any transfusion during therapy. HU is a good treatment option for patients with EMH and it could be a substitute for radiotherapy and invasive surgery or regular blood transfusion.

  20. Imaging observations of a schwannoma of low malignant potential in the anterior abdominal wall: A case report.

    Science.gov (United States)

    Liu, Yongkang; Chen, Xiao; Wang, Tianyao; Wang, Zhongqiu

    2014-09-01

    Neurilemmoma, also known as schwannoma, is an uncommon benign neoplasm that is most commonly found in the trunk and head and neck regions. The present study reports the case of a 67-year-old female with schwannoma localized in the anterior abdominal wall and analyzes the ultrasound and computed tomography (CT) imaging observations of the schwannoma. A dynamic time-intensity curve was also recorded in the study. A well-defined, elliptic low echo level, heterogeneous mass was observed during ultrasound examination. The CT scan revealed a solid, heterogeneous, low-density mass in the abdominal wall. Contrast-enhanced scans showed a heterogeneously enhanced mass during the arterial and venous phase. Centripetal fill-in was demonstrated and the mass was markedly, homogenously enhanced relative to the muscles during the delayed phase. Peak enhancement was observed during the venous phase and then slowly declined. However, the mass was hyperattenuated during the delayed phase. The lesion was completely excised and no evidence of recurrence has been identified during the 3 months of follow-up. The present study suggested that a diagnosis of schwannoma should be considered for certain patients with masses in the abdominal wall. Peripheral enhancement during the arterial and venous phases and homogeneous enhancement in the delayed phase are the significant imaging findings of a schwannoma.

  1. Malignant transformation in a hybrid schwannoma/perineurioma: Addition to the spectrum of a malignant peripheral nerve sheath tumor

    Directory of Open Access Journals (Sweden)

    Bharat Rekhi

    2011-01-01

    Full Text Available Benign nerve sheath tumors include schwannomas, neurofibromas and perineuriomas. The malignant counterpart of a nerve sheath tumor is designated as a malignant peripheral nerve sheath tumor (MPNST. Lately, benign nerve sheath tumors comprising more than one component have been described, including hybrid schwannomas/perineuriomas. However, malignant transformation in a hybrid schwannoma/perineurioma has not been documented so far. Herein, we present a rare case of a young adult male who presented with a soft tissue mass in his right thigh that was excised elsewhere and submitted to us for histopathological review. One of the tissue sections displayed histopathological features of a hybrid schwannoma/perineurioma, including alternate arrangement of benign schwann and perineurial cells, reinforced with S100-P and epithelial membrane antigen positivity, respectively, along with low MIB1 and negative p53 immunostaining. The other two tissue sections showed a spindly sarcomatous tumor that was immunohistochemically positive for S100-P, CD34, p53 and exhibited high MIB1 (30-40%. Diagnosis of a MPNST arising in a hybrid schwannoma/perineurioma was made. This unusual case forms yet another addition to the spectrum of a MPNST.

  2. Foramen magnum schwannoma: review of the literature and report of a case; Schwannoma do forame magno: revisao e relato de caso

    Energy Technology Data Exchange (ETDEWEB)

    Nacif, Marcelo Souto; Caiado, Stella; Oliveira, Nidia di Paula Silva; Paula Neto, Walter Teixeira de [Fundacao Educacional Serra dos Orgaos, Teresopolis, RJ (Brazil). Centro de Ciencias Biomedicas. Faculdade de Medicina de Teresopolis]. E-mail: marcelonacif30@hotmail.com; Mello, Ricardo Andrade Fernandes de [Universidade Federal, Rio de Janeiro, RJ (brazil). Faculdade de Medicina. Dept. de Radiologia; Jauregui, Gustavo Federico [Hospital Geral de Bonsucesso, Rio de Janeiro, RJ (Brazil); Campos, Flavio do Amaral [Universidade Federal de Sao Paulo (UNIFESP/EPM), SP (Brazil). Servico de Radiologia; Santos, Alair Augusto Sarmet Moreira Damas dos [Instituto de Pos-graduacao Medica Carlos Chagas (IPGMCC), Niteroi, RJ (Brazil). Curso de Pos-graduacao em Radiologia

    2001-02-01

    The authors report an unusual presentation of a voluminous neck schwannoma in a 53-year-old female that presented with a three-year history of progressive weakness associated with impaired movement of the limbs. Neurological examination revealed postural instability, unstable and paraparetic gait, tetraparesis and dyspnea. A preoperative magnetic resonance imaging of the neck revealed an expansive, extradural, well-circumscribed lesion, with soft-tissue attenuation, at the level of C1-C2 vertebral bodies. During surgery, the tumor was found to be extradural, lateral to the cervical spinal cord, attached to the C1 left nerve root and extending upwards through the foramen magnum. Histopathological analysis of the resected specimen confirmed the diagnosis of schwannoma. The patient showed a favorable outcome with progressive improvement of the symptoms. Magnetic resonance imaging proved to be valuable in the detection and evaluation of the lesion, although the definite diagnosis was achieved only after histopathological studies. We concluded that magnetic resonance imaging for early diagnosis and prompt surgical resection seems to be the best approach to achieve good prognosis. (author)

  3. Extramedullary Relapse Following Total Marrow and Lymphoid Irradiation in Patients Undergoing Allogeneic Hematopoietic Cell Transplantation

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Ji Hyun [Department of Radiation Oncology, City of Hope National Medical Center, Duarte, California (United States); Stein, Anthony [Department of Hematology/Hematopoietic Cell Transplantation, City of Hope National Medical Center, Duarte, California (United States); Tsai, Nicole [Department of Biostatistics, City of Hope National Medical Center, Duarte, California (United States); Schultheiss, Timothy E. [Department of Radiation Oncology, City of Hope National Medical Center, Duarte, California (United States); Palmer, Joycelynne [Department of Biostatistics, City of Hope National Medical Center, Duarte, California (United States); Liu, An [Department of Radiation Oncology, City of Hope National Medical Center, Duarte, California (United States); Rosenthal, Joseph [Department of Hematology/Hematopoietic Cell Transplantation, City of Hope National Medical Center, Duarte, California (United States); Department of Pediatrics, City of Hope National Medical Center, Duarte, California (United States); Forman, Stephen J. [Department of Hematology/Hematopoietic Cell Transplantation, City of Hope National Medical Center, Duarte, California (United States); Wong, Jeffrey Y.C., E-mail: jwong@coh.org [Department of Radiation Oncology, City of Hope National Medical Center, Duarte, California (United States)

    2014-05-01

    Purpose: Approximately 5% to 20% of patients who undergo total body irradiation (TBI) in preparation for hematopoietic cell transplantation (HCT) can develop extramedullary (EM) relapse. Whereas total marrow and lymphoid irradiation (TMLI) provides a more conformally targeted radiation therapy for patients, organ sparing has the potential to place the patient at a higher risk for EM relapse than TBI. This study evaluated EM relapse in patients treated with TMLI at our institution. Methods and Materials: Patients eligible for analysis had been enrolled in 1 of 3 prospective TMLI trials between 2006 and 2012. The TMLI targeted bones, major lymph node chains, liver, spleen, testes, and brain, using image-guided tomotherapy with total dose ranging from 12 to 15 Gy. Results: A total of 101 patients with a median age of 47 years were studied. The median follow-up was 12.8 months. Incidence of EM relapse and bone marrow (BM) relapse were 12.9% and 25.7%, respectively. Of the 13 patients who had EM relapse, 4 also had BM relapse, and 7 had EM disease prior to HCT. There were a total of 19 EM relapse sites as the site of initial recurrence: 11 soft tissue, 6 lymph node, 2 skin. Nine of these sites were within the target region and received ≥12 Gy. Ten initial EM relapse sites were outside of the target region: 5 sites received 10.1 to 11.4 Gy while 5 sites received <10 Gy. Pretransplantation EM was the only significant predictor of subsequent EM relapse. The cumulative incidence of EM relapse was 4% at 1 year and 11.4% at 2 years. Conclusions: EM relapse incidence was as frequent in regions receiving ≥10 Gy as those receiving <10 Gy. EM relapse rates following TMLI that included HCT regimens were comparable to published results with regimens including TBI and suggest that TMLI is not associated with an increased EM relapse risk.

  4. Hematopoiese extramedular intratorácica: relato de um caso Intrathoracic extramedullary hematopoiesis: a case report

    Directory of Open Access Journals (Sweden)

    Luiza Beatriz Melo Moreira

    2001-06-01

    Full Text Available Os autores relatam o caso de um paciente do sexo masculino, de 40 anos de idade, portador de anemia falciforme, que desenvolveu a forma intratorácica de hematopoiese extramedular, apresentando lesões expansivas com densidade de partes moles, localizadas posteriormente, nas goteiras paravertebrais, na radiologia convencional. A tomografia computadorizada do tórax demonstrou massas bem delimitadas, de contornos lobulados, com densidade heterogênea à custa de áreas de tecido gorduroso e de densidade de partes moles, localizadas no terço inferior das goteiras paravertebrais. Os pulmões tinham coeficientes de atenuação preservados. A tomografia computadorizada de abdome revelou importante aumento da densidade e do volume do fígado, atribuídos à hemossiderose, e baço atrófico e calcificado devido a infartos esplênicos de repetição.The authors report a case of a 40-year-old male patient with sickle cell disease who presented with intra-thoracic extramedullary hematopoiesis and paravertebral expansive lesions that appeared as soft tissue density masses on conventional x-ray films. A computed tomography of the chest demonstrated soft tissue density masses in the lower third of the paravertebral spaces. These masses presented well-defined limits, lobulated edges and were heterogeneous due to areas of interposed adipose and soft density tissues. The lung parenchyma had no abnormalities. Computed tomography of the abdomen revealed diffuse hiperdensity and enlargement of the liver that was attributed to hemosiderosis, and an atrophic and calcified spleen due to repeated episodes of infarction.

  5. Nephrotic syndrome in primary myelofibrosis with renal extramedullary hematopoiesis and glomerulopathy in the JAK inhibitor era.

    Science.gov (United States)

    Del Sordo, Rachele; Brugnano, Rachele; Covarelli, Carla; Fiorucci, Gioia; Falzetti, Franca; Barbatelli, Giorgio; Nunzi, Emidio; Sidoni, Angelo

    2017-01-01

    Primary myelofibrosis (PMF) is an uncommon form of myeloproliferative neoplasm (MPN) characterized by a proliferation of predominantly megakaryocytes and granulocytes in the bone marrow that, in fully-developed disease, is associated with reactive deposition of fibrous connective tissue, extramedullary hematopoiesis (EMH), and splenomegaly. Kidney involvement is rare and clinically presents with proteinuria, nephrotic syndrome, and renal insufficiency. Renal damage can be due to EMH and glomerulopathy. Renal EMH presents three patterns: infiltration of the interstitium with possible renal failure caused by functional damage of parenchyma and vessels, infiltration of capsule and pericapsular adipose tissue, and sclerosing mass-like lesions that can cause hydronephrosis and hydroureter with obstructive uropathy and renal failure. Glomerulopathy associated with PMF is rarely described, ranging from 1 month to 18 years from diagnosis of the neoplasm to renal biopsy. It is characterized by expansion and hypercellularity mesangial, segmental sclerosis, features of chronic thrombotic microangiopathy (TMA), and intracapillary hematopoietic cells infiltrating in absence of immune-mediated glomerulonephritis. We present a nephrotic syndrome in PMF-related glomerulopathy, associated with EMH, without renal failure, in a patient under treatment for 2 years with JAK2 inhibitor ruxolitinib. Despite treatment, the patient died 7 months after renal biopsy. Nephrologists still know very little about this topic and there is no homogeneous data about incidence, pathogenesis, and optimal treatment of this poor prognostic PMF-associated nephrotic syndrome. We focus on data in the literature in the hope of stimulating hematologists, nephrologists, pathologists to future studies about the natural history of renal involvement, useful for optimal management of this rare pathology.

  6. [Symptomatic extramedullary haematopoiesis in β-thalassemia: A retrospective single centre study].

    Science.gov (United States)

    Maazoun, F; Gellen Dautremer, J; Boutekadjirt, A; Pissard, S; Habibi, A; Bachir, D; Rahmouni, A; Bartolucci, P; Debbache, K; Lagrange, J-L; Michel, M; Galacteros, F

    2016-01-01

    Symptomatic extramedullary hematopoiesis (EH) is a rare but potentially severe phenomenon which occurs in β-thalassemia. There are no treatment guidelines. Retrospective single centre study including the cases of symptomatic EH encountered between 1997 and 2014 in a unit specialised in red blood cell genetic disorders. Description of clinical, biological and radiological characteristics of the patients, treatments received, and outcomes. Among 182 β-thalassemia patients followed during the study period, 7 cases of symptomatic EH were diagnosed. They were 5 men and 2 women, and their mean age was 37 years. Four patients were splenectomised, two patients were regularly transfused, and four patients had already received erythropoietin. EH was localised in intravertebral areas and responsible for dorsal spinal cord compression in 5 patients, in paravertebral dorsal area in 1 patient, and in presacral area in 1 patient. The mean hemoglobin level at diagnosis was 7.9 g/dL. Treatment administered included: red cell transfusion in 6 cases, associated with hydroxyurea in 5 cases and/or radiotherapy in 3 patients. One patient was treated with surgery and HU. After a median follow-up of 41 months, clinical recovery was complete in 2 patients and partial in 5 patients. EH must be suspected in β-thalassemia in patients presenting clinical signs of organ compression, and a typical radiological aspect. The functional prognosis depends on the rapidity of treatment, which includes red blood cell transfusion, hydroxyurea, radiotherapy, and rarely surgery. Long-term outcome is uncertain. Copyright © 2015. Published by Elsevier SAS.

  7. Ginsenoside Rg1 improves bone marrow haematopoietic activity via extramedullary haematopoiesis of the spleen.

    Science.gov (United States)

    Liu, Hua-Hsing; Chen, Fei-Peng; Liu, Rong-Kai; Lin, Chun-Lin; Chang, Ko-Tung

    2015-11-01

    Cyclophosphamide (CY) is a chemotherapeutic agent used for cancer and immunological diseases. It induces cytotoxicity of bone marrow and causes myelosuppression and extramedullary haematopoiesis (EMH) in treated patients. EMH is characterized with the emergence of multipotent haematopoietic progenitors most likely in the spleen and liver. Previous studies indicated that a Chinese medicine, ginsenoside Rg1, confers a significant effect to elevate the number of lineage (Lin(-) ) Sca-1(+) c-Kit(+) haematopoietic stem and progenitor cells (HSPCs) and restore the function of bone marrow in CY-treated myelosuppressed mice. However, whether the amelioration of bone marrow by Rg1 accompanies an alleviation of EMH in the spleen was still unknown. In our study, the cellularity and weight of the spleen were significantly reduced after Rg1 treatment in CY-treated mice. Moreover, the number of c-Kit(+) HSPCs was significantly decreased but not as a result of apoptosis, indicating that Rg1 alleviated EMH of the spleen induced by CY. Unexpectedly, the proliferation activity of c-Kit(+) HSPCs was only up-regulated in the spleen, but not in the bone marrow, after Rg1 treatment in CY-treated mice. We also found that a fraction of c-Kit(+) /CD45(+) HSPCs was simultaneously increased in the circulation after Rg1 treatment. Interestingly, the effects of Rg1 on the elevation of HSPCs in bone marrow and in the peripheral blood were suppressed in CY-treated splenectomized mice. These results demonstrated that Rg1 improves myelosuppression induced by CY through its action on the proliferation of HSPCs in EMH of the spleen and migration of HSPCs from the spleen to the bone marrow. © 2015 The Authors. Journal of Cellular and Molecular Medicine published by John Wiley & Sons Ltd and Foundation for Cellular and Molecular Medicine.

  8. Reduced RAC1 activity inhibits cell proliferation and induces apoptosis in neurofibromatosis type 2(NF2)-associated schwannoma.

    Science.gov (United States)

    Wang, Ying; Wang, Bo; Li, Peng; Zhang, Qi; Liu, Pinan

    2017-12-01

    Objective To study the function and potential mechanism of RAC1 inhibitors in NF2-associated schwannoma. Methods In this study, we the downregulation of RAC1 activity and tumor cell phenotypes by RAC1 inhibitor NSC23766 in vitro. And we further validated the anti-proliferation effect by this RAC1 inhibitor in subcutaneous xenograft tumor model and sciatic nerve model. Results Pharmacological inhibition of RAC1 could significantly inhibit the proliferation of both RT4 cells and human NF2-associated primary schwannoma cells by inducing apoptosis. Pharmacological inhibition of RAC1 effectively reduced Rac1 activity and down-regulated the pathway downstream of Rac. Moreover, pharmacological inhibition of RAC1 showed a potential antitumor effect, with low toxicity in vivo. Conclusion RAC1 inhibitors may play a therapeutic role in patients with schwannoma.

  9. Multiple schwannomas, including a plexiform variant, in a patient in the absence of neurofibromatosis I or II

    Directory of Open Access Journals (Sweden)

    Snehlata R Hingway

    2015-01-01

    Full Text Available Multiple schwannomatosis, is defined as two or more, pathologically proven schwannomas, without radiographic evidence of vestibular nerve tumor, below 18 years, or absence of VIII nerve dysfunction beyond 30 years with absence of stigmata of neurofibromatosis 2 (NF2. The plexiform schwannoma (PS is a rare tumor. The gross appearance is an important diagnostic criterion for diagnosis of PSs. It is associated with NF2, which was ruled out in this case. Here we present a 50 year female patient with two nonintradermal schwannomas, in the supraclavicular and infraauricular deep soft tissues. One was plexiform, diagnosed on cytology and confirmed by histopathology. She also had multiple pulmonary and pleural lesions. Fine needle aspiration cytology smears from both supraclavicular and infraauricular lesions as well as the lung lesion sampled, showed spindle cells in a myxoid background, having wavy nuclei with pointed ends. Verocay body - like palisading of nuclei was noted.

  10. Communicating Hydrocephalus Associated with Small- to Medium-Sized Vestibular Schwannomas: Clinical Significance of the Tumor Apparent Diffusion Coefficient Map.

    Science.gov (United States)

    Taniguchi, Masaaki; Nakai, Tomoaki; Kohta, Masaaki; Kimura, Hidehito; Kohmura, Eiji

    2016-10-01

    The etiology of hydrocephalus associated with the small- to medium-sized vestibular schwannomas is still controversial. We investigated tumor-specific factors related to the association of hydrocephalus with small- to medium-sized vestibular schwannomas. Among the 77 patients with vestibular schwannoma smaller than 30 mm, 9 patients demonstrated associated communicating hydrocephalus. Patient medical records, radiologic data, and histopathologic specimens were reviewed retrospectively. The age of the patients, and size, mean apparent diffusion coefficient (ADC) value, and histologic features of the tumors were compared with those of patients without hydrocephalus. The symptoms related to hydrocephalus improved in all patients after tumor removal. Both the mean size and ADC values exhibited a statistically significant difference between the tumors with and without hydrocephalus (P hydrocephalus. The increased tumor ADC value was considered to be the result of degenerative change and suggested the involvement of protein sloughing in the etiology of the associated hydrocephalus. Copyright © 2016 Elsevier Inc. All rights reserved.

  11. Segmental neurofibromatosis type 2: discriminating two hit from four hit in a patient presenting multiple schwannomas confined to one limb.

    Science.gov (United States)

    Castellanos, Elisabeth; Bielsa, Isabel; Carrato, Cristina; Rosas, Imma; Solanes, Ares; Hostalot, Cristina; Amilibia, Emilio; Prades, José; Roca-Ribas, Francesc; Lázaro, Conxi; Blanco, Ignacio; Serra, Eduard

    2015-01-24

    A clinical overlap exists between mosaic Neurofibromatosis Type 2 and sporadic Schwannomatosis conditions. In these cases a molecular analysis of tumors is recommended for a proper genetic diagnostics. This analysis is challenged by the fact that schwannomas in both conditions bear a somatic double inactivation of the NF2 gene. However, SMARCB1-associated schwannomas follow a four-hit, three-step model, in which both alleles of SMARCB1 and NF2 genes are inactivated in the tumor, with one of the steps being always the loss of a big part of chromosome 22 involving both loci. Here we report a 36-year-old woman who only presented multiple subcutaneous schwannomas on her right leg. To help discriminate between both possible diagnoses, an exhaustive molecular genetic and genomic analysis was performed on two schwannomas of the patient, consisting in cDNA and DNA sequencing, MLPA, microsatellite multiplex PCR and SNP-array analyses. The loss of a big part of chromosome 22 (22q12.1q13.33) was identified in both tumors. However, this loss involved the NF2 but not the SMARCB1 locus. SNP-array analysis revealed the presence of the same deletion breakpoint in both schwannomas, indicating that this alteration was actually the first NF2 inactivating hit. In addition, a distinct NF2 point mutation in each tumor was identified, representing independent second hits. In accordance with these results, no deletions or point mutations in the SMARCB1 gene were identified. None of the mutations were present in the blood. Two of the patient's children inherited chromosome 22 deleted in schwannomas of the mother, but in its wild type form. These results conclusively confirm the segmental mosaic NF2 nature of the clinical phenotype presented.

  12. Solitary Extramedullary Plasmacytoma of the Maxillary Sinus, Progressing to Smoldering Multiple Myeloma with Multifocal Skeletal Involvement, which Resolved Completely Following Chemotherapy Alone.

    Science.gov (United States)

    Jeyaraj, Priya; Venkatesan, Manu; Nijhawan, V S

    2016-07-01

    Plasmacytoma is an uncommon malignant tumor originating either from plasma cells located in the bone marrow also known as the solitary bone plasmacytoma, or from plasma cells located outside the bone, for e.g. in mucosal surfaces, referred to as the extramedullary plasmacytoma also called the solitary extramedullary plasmacytoma. Both, solitary as well as extramedullary bone plasmacytomas may, particularly in later stages, be accompanied by other osteolytic bone lesions (multifocal bone involvement) and features such as anemia, hypercalcemia, or renal impairment attributable to and indicative of progression to multiple myeloma. These three distinct disorders together comprise the plasma cell neoplasms and essentially represent a continuum of related disease processes. Extramedullary and solitary bone plasmacytomas of the head and neck region are extremely uncommon, and amongst them plasmacytoma of the maxilla is extremely rare. Such a case is being reported here for its rarity. Also, it was associated with multifocal skeletal involvement, making a correct categorization difficult as well as imperative in order to institute the correct treatment. Radiotherapy is considered to be the treatment of choice of plasmacytoma, with adjuvant chemotherapy for multi focal involvement. Surgery is usually limited to biopsy and excision of any residual disease following radiotherapy. The case presented responded extremely well to chemotherapy alone, with a complete resolution of the maxillary tumor, obviating the need for radiotherapy.

  13. A Case of Action-Induced Clonus that Mimicked Action Tremors and was Associated with Cervical Schwannoma

    Directory of Open Access Journals (Sweden)

    Young-Hee Sung

    2010-10-01

    Full Text Available Clonus is the rhythmic muscle contraction which usually occurs in patients with lesions involving descending motor pathways. Sometimes, rhythmic oscillation of action induced clonus could be confused to action tremor. We report a case of action induced clonus associated with cervical schwannoma which was misdiagnosed as essential tremor. The patient had spasticity in all limbs with exaggerated tendon reflexes, and passive stretch-induced clonus. Imaging and histological examinations revealed a schwannoma extending from C2 to C7. The lesion was partially removed by surgery. Even though essential tremor is a common disease, clinician have to do sufficient neurologic examination considering differential diagnosis.

  14. MR imaging of a malignant schwannoma and an osteoblastoma with fluid-fluid levels. Report of two new cases

    Energy Technology Data Exchange (ETDEWEB)

    Vilanova, J.C.; Dolz, J.L.; Aldoma, J.; Capdevila, A. [Centre Diagnostic Pedralbes, Ressonancia Magnetica, Barcelona (Spain); Maestro de Leon, J.L.; Aparicio, A. [Department of Neurosurgery, Hospital Mutua de Terrassa, Barcelona (Spain)

    1998-10-01

    One case of malignant schwannoma of the sacrum and another of occipital osteoblastoma were evaluated by MR imaging. Both tumors showed fluid-fluid levels with different signal intensities in the sequences performed. Pathologic examination revealed hemmorhagic fluid in both tumors. Malignant schwannoma and osteoblastoma should be included in the list of bone and soft-tissue with fluid-fluid levels. Our data confirm the non-specificity of this finding, which only suggests the presence of previous intratumoral hemorrhage. (orig.) (orig.) With 2 figs., 2 tabs., 17 refs.

  15. Schwannoma do forame magno: revisão e relato de caso Foramen magnum schwannoma: review of the literature and report of a case

    Directory of Open Access Journals (Sweden)

    Marcelo Souto Nacif

    2005-02-01

    Full Text Available Os autores relatam um volumoso schwannoma cervical de apresentação incomum, numa paciente do sexo feminino, 53 anos de idade, que em fevereiro de 2002 apresentou queixa de fraqueza progressiva, iniciada há três anos, com dificuldade de mobilização das pernas e braços. Antes da cirurgia foi realizada ressonância magnética da região cervical. No exame físico apresentava postura com aumento da base de sustentação, marcha instável e paraparética, além de diminuição da força nos quatro membros, maior à esquerda, associada a dispnéia. Na ressonância magnética cervical evidenciou-se lesão expansiva extramedular de limites bem definidos, na altura de C1 e C2. Na cirurgia, o volumoso tumor encontrava-se ântero-lateralmente à medula, aderido à raiz esquerda de C1, estendendo-se superiormente através do forame magno, com localização extradural. O diagnóstico de schwannoma foi confirmado pela histopatologia. A paciente evoluiu satisfatoriamente, com melhora progressiva da hemiparesia e hemiparestesia esquerda. A ressonância magnética possibilita a detecção e avaliação da lesão, porém o diagnóstico definitivo só é feito com o exame histopatológico. Dessa forma, o diagnóstico precoce através da ressonância magnética e a exérese cirúrgica tornam-se a melhor forma de abordagem, com bom prognóstico.The authors report an unusual presentation of a voluminous neck schwannoma in a 53-year-old female that presented with a three-year history of progressive weakness associated with impaired movement of the limbs. Neurological examination revealed postural instability, unstable and paraparetic gait, tetraparesis and dyspnea. A preoperative magnetic resonance imaging of the neck revealed an expansive, extradural, well-circumscribed lesion, with soft-tissue attenuation, at the level of C1-C2 vertebral bodies. During surgery, the tumor was found to be extradural, lateral to the cervical spinal cord, attached to the C1 left

  16. Small-field fractionated radiotherapy with or without stereotactic boost for vestibular schwannoma

    International Nuclear Information System (INIS)

    Kagei, K.; Shirato, H.; Suzuki, K.; Isu, T.; Sawamura, Y.; Sakamoto, T.; Fukuda, S.; Nishioka, T.; Hashimoto, S.; Miyasaka, K.

    1999-01-01

    Purpose: To assess the efficacy and toxicity of small-field fractionated radiotherapy with or without stereotactic boost (SB) for vestibular schwannomas.Methods and materials: Thirty-nine patients with vestibular schwannoma were treated with irradiation between March 1991 and February 1996. Extra-meatal tumor diameters were under 30 mm. Thirty-three patients received small-field fractionated radiotherapy followed by SB. Basic dose schedule was 44 Gy in 22 fractions over 5 1/2 weeks plus 4 Gy in one session. Six patients received small-field fractionated radiotherapy only (40-44 Gy in 20-22 fractions over 5-5 1/2 weeks or 36 Gy in 20 fractions over 5 weeks).< Results: Follow-up ranged from 6 to 69 months (median, 24 months). Tumors decreased in size in 13 cases (33%), were unchanged in 25 (64%), and increased in one (3%). The actuarial 2-year tumor control rate was 97%. Fifteen patients had useful hearing (Gardner-Robertson class 1-2) and 25 patients had testable hearing (class 1-4) before irradiation. The 2-year actuarial rates of useful hearing preservation (free of deterioration from class 1-2 to class 3-5) were 78%. The 2-year actuarial rates of any testable hearing preservation (free of deterioration from class 1-4 to class 5) were 96%. No permanent facial and trigeminal neuropathy developed after irradiation. The 2-year actuarial incidences of facial and trigeminal neuropathies were 8% and 16%, respectively.Conclusions: Small-field fractionated radiotherapy with or without SB provides excellent short-term local control and a relatively low incidence of complications for vestibular schwannoma, although further follow-up is necessary to evaluate the long-term results. (Copyright (c) 1999 Elsevier Science B.V., Amsterdam. All rights reserved.)

  17. Schwanoma de plexo braquial: relato de dois casos Schwannoma of brachial plexus: report of two cases

    Directory of Open Access Journals (Sweden)

    Manoel Baldoíno Leal Filho

    2004-03-01

    Full Text Available Schwanomas, neurinomas ou neurilemomas são tumores benignos de nervos periféricos. Podem ocorrer em associação com a neurofibromatose tipo 2. Relatamos dois casos de tumor cervical originado em plexo braquial sem associação com neurofibromatose. Uma mulher, de 31 anos apresentando uma tumefação em região supraclavicular direita, dor irradiada para o membro ipsilateral e sinal de Tinel à percussão da região. Outra mulher, 52 anos, com cervicobraquialgia persistente à direita há um ano. Ambas foram submetidas a microcirurgia, com ressecção total da lesão. O estudo histopatológico foi compatível com schwanoma. As duas pacientes tiveram boa evolução neurológica, com desaparecimento dos sinais e sintomas.Schwannomas, neurinomas or neurilemmomas are benign peripheral nerve tumors. The literature report some cases associated with neurofibromatosis 2. We report two cases of cervical schwannoma originating from the brachial plexus unassociated with neurofibromatosis. A 31-year-old woman presented with a mass in the right supraclavicular region, irradiating pain and distal tingling to percussion (Tinel's sign for 6 months. And a 52-year-old woman presented with pain in the cervical region and right arm for one year. Both the patients underwent to a microsurgery with total resection of the lesion. Histology of the surgical specimen confirmed the diagnosis of schwannoma. Postoperatively, the patients had a good recovery.

  18. The vestibulo-ocular reflex and subjective balance after vestibular schwannoma surgery.

    Science.gov (United States)

    Batuecas-Caletrio, Angel; Santacruz-Ruiz, Santiago; Muñoz-Herrera, Angel; Perez-Fernandez, Nicolas

    2014-06-01

    To evaluate the vestibulo-ocular reflex and its relationship with subjective balance in a long-term follow-up after vestibular schwannoma surgery. Retrospective cohorts study in a tertiary referral hospital. Forty-nine consecutive patients on which vestibular schwannoma surgery was performed at least 1 year before. Patients are classified in accordance with the morphology of the vestibulo-ocular reflex (VOR) into two groups: Group A, in which covert and overt saccades always occur in an organized fashion; and group B, in which covert and overt saccades randomly occur during head impulse and once finished. We evaluate the presence of covert and overt saccades and the morphology of the VOR measured by the video head impulse test (vHIT) and its relationship with subjective balance measured by Dizziness Handicap Inventory (DHI). The group B patients returned a higher score in total DHI and all three subscales without any predominant subscale (P = 0,0002; t test). Group B patients were older than those in group A (P = 0,024; t test). No differences were found in sex distribution, tumor size, or side or time interval after surgery. Regarding the unaffected side, overt saccades were found to be more frequent in group B patients (P = 0.05; X(2)). Long-term follow-up after vestibular schwannoma surgery has shown that 22% of the patients display a particular abnormality in the VOR because refixation saccades occur in a random fashion after elicitation of the reflex in the HIT test. These patients report the higher level of vestibular disability and handicap. 2b. © 2013 The American Laryngological, Rhinological and Otological Society, Inc.

  19. Postural Stability Evaluation of Patients Undergoing Vestibular Schwannoma Microsurgery Employing the Inertial Measurement Unit

    Directory of Open Access Journals (Sweden)

    Patrik Kutilek

    2018-01-01

    Full Text Available The article focuses on a noninvasive method and system of quantifying postural stability of patients undergoing vestibular schwannoma microsurgery. Recent alternatives quantifying human postural stability are rather limited. The major drawback is that the posturography system can evaluate only two physical quantities of body movement and can be measured only on a transverse plane. A complex movement pattern can be, however, described more precisely while using three physical quantities of 3-D movement. This is the reason why an inertial measurement unit (Xsens MTx unit, through which we obtained 3-D data (three Euler angles or three orthogonal accelerations, was placed on the patient’s trunk. Having employed this novel method based on the volume of irregular polyhedron of 3-D body movement during quiet standing, it was possible to evaluate postural stability. To identify and evaluate pathological balance control of patients undergoing vestibular schwannoma microsurgery, it was necessary to calculate the volume polyhedron using the 3-D Leibniz method and to plot three variables against each other. For the needs of this study, measurements and statistical analysis were made on nine patients. The results obtained by the inertial measurement unit showed no evidence of improvement in postural stability shortly after surgery (4 days. The results were consistent with the results obtained by the posturography system. The evaluated translation variables (acceleration and rotary variables (angles measured by the inertial measurement unit correlate strongly with the results of the posturography system. The proposed method and application of the inertial measurement unit for the purpose of measuring patients with vestibular schwannoma appear to be suitable for medical practice. Moreover, the inertial measurement unit is portable and, when compared to other traditional posturography systems, economically affordable. Inertial measurement units can

  20. Schwannoma del nervio facial intraparotídeo. Un dilema terapéutico

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    Paula Barba-Recreo

    2015-07-01

    Full Text Available Los schwannomas del nervio facial intraparotídeos son tumores benignos poco frecuentes, suponiendo frecuentemente un reto diagnóstico y terapéutico. La mayoría de los pacientes presentan una masa parotídea asintomática y las pruebas de imagen y la punción con aguja fina no suelen ser concluyentes en el diagnóstico. Tras la revisión de la literatura a propósito de un caso, pretendemos proporcionar cierta guía para el tratamiento de esta rara patología.