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.

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

Large Dumbbell-Shaped C1 Schwannoma Presenting as a Foramen Magnum Mass

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Helms, Jody; Michael, Lattimore Madison

2012-01-01

Schwannomas involving the foramen magnum commonly originate from the lower cranial nerves, but they are rarely found arising from the first cervical root. To date, very few cases have been described in the literature. The majority involve either the intradural or extradural compartment but not both. We report the second case of a dumbbell-shaped schwannoma arising from the first cervical root. Our patient presented with hemisensory deficits secondary to brainstem compression at the level of the foramen magnum. The patient underwent a far lateral approach, and a gross total resection was achieved. Preoperative suspicion of the diagnosis is helpful in anticipating displacement and avoiding damage to the surrounding neurovascular structures. PMID:23946923

Intradural lipoma

International Nuclear Information System (INIS)

Schubert, F.

1996-01-01

An intradural lipoma consists of a localized collection of fat within the intradural space of the spinal canal. Microscopically, spinal lipomas consist of a homogeneous mass of adult adipose tissue (rarely, brown fat or mixed yellow and brown fat) that exhibits no evidence of malignancy. The fat is divided into lobules by thick connective-tissue strands which may be associated with smooth muscle fibres. Striated muscle fibres, angiomatous elements, calcification and ossification are less common. A case of intradural lipoma is presented and it is shown how both computer tomography and magnetic resonance imaging (MRI) assisted in an accurate diagnosis of this pathology. MR is the imaging modality of choice as it allows to fully evaluate the extent of the lipoma and its relationship to the neural placode, spinal cord and roots of the cord and/or cauda equina. 4 refs., 5 figs

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

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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.

A Large Asymptomatic Thoracic Spine Schwannoma Detected Incidentally by {sup 18}F-FDG PET/CT: A Case Report

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Bahk, Yong Whee [Seoul Sungae Hospital, Seoul (Korea, Republic of); Yoo, Ie Ryung [Catholic University Medical School Hospital, Seoul (Korea, Republic of)

2008-10-15

Schwannoma is not a rare tumor occurring anywhere where sheathed nerve fibers present. However the spinal involvement has been noted to be uncommon. The use of PET in the diagnosis of spinal schwannomas seemed to have only sporadically been reported and standardized uptake values (SUV) measured in peripheral nerves schwannomas varied according to cellularity. Most reported spinal schwannoms were symptomatic and relatively hypometabolic but ours differed in that despite considerable compression of the spinal cord it did not produce clinical symptoms or signs and had a relatively high FDG uptake value. The spine was operated. On opening a 1.0x1.5 cm tumor was found to be encapsulated and located in the right posterolateral aspect of the intradural space at the 4th thoracic spine level. It was completely resected along with the rootlet from which the tumor seemed to have originated not damaging the cord or causing bleeding. The spinal medulla compressed by and adherent to the tumor was decompressed and released following arachnoid adhesiolysis. The hospital stay was uneventful. Patient was discharged and is well and fine without complication eight months after surgery.

Extramedullary plasmocytomas

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Ben Salah, H.; Daoud, J.; Hdiji, S.; Elloumi, M.; Makni, S.; Boudawara, T.; Ghorbel, A.M.

2012-01-01

Purpose. - To study the localization, treatment and prognosis of extramedullary plasmocytoma through a series of eight patients and a literature review. Patients and methods. - Eight patients with extramedullary plasmocytoma were treated in the university's hospital of Sfax in Tunisia. The average age was 57.3 years. Female represented 75% of patients. The diagnosis of plasmocytoma was based on anatomo-pathology and immunohistochemistry of a biopsy or resected tumour. Extramedullary location was confirmed if biological and radiological exams and medullary biopsy were normal. The therapeutic decision was made after multidisciplinary meetings regarding tumour location and anterior treatment. Results. - Solitary extramedullary plasmocytoma was located in nasal cavity, cervical node, testis, ovary, bladder and the tongue. One patient was treated for three simultaneous locations of extramedullary plasmocytoma (node, bowel, pleura) without evidence of myeloma. Radiotherapy was proposed in six cases but refused in one case (plasmocytoma of the bladder is currently receiving radiotherapy). Treatment consisted in chemotherapy in two cases. Evaluation after treatment revealed complete remission in 86% of the cases. Nodal recurrence was noted in two cases. These two patients were lost to follow up. The five other patients were in complete remission after a mean follow up of 5.7 years. No local recurrence or myeloma was noted. Conclusion. - Extramedullary plasmocytoma is a rare affection. It can occur in any region of the body. Head and neck is most frequent localization. The treatment is irradiation or surgery in some localization. Progression to myeloma is the most important factor that influences the prognosis of the disease. (authors)

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

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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.

Multiple myeloma with extramedullary disease.

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Oriol, Albert

2011-11-01

Plasmacytoma is a tumor mass consisting of atypical plasma cells. Incidence of plasmacytomas associated with multiple myeloma range from 7% to 17% at diagnosis and from 6% to 20% during the course of the disease. In both situations, occurrence of extramedullary disease has been consistently associated with a poorer prognosis of myeloma. Extramedullary relapse or progression occurs in a variety of clinical circumstances and settings, and therefore requires individualization of treatment. Alkylating agents, bortezomib, and immunomodulatory drugs, along with corticoids, have been used to treat extramedullary relapse but, because of the relatively low frequency or detection rate of extramedullary relapse, no efficacy data are available from controlled studies in this setting.

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

Extramedullary plasmacytoma of the larynx

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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.

Extramedullary plasmacytoma of the larynx.

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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.

MRI features of epidural extramedullary hematopoiesis

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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.

MRI features of epidural extramedullary hematopoiesis

International Nuclear Information System (INIS)

Alorainy, Ibrahim A.; Al-Asmi, Abdullah R.; Carpio, Raquel del

2000-01-01

A case of β-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

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.

Intradural squamous cell carcinoma in the sacrum

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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.

Mass-like extramedullary hematopoiesis: imaging features

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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.)

Evaluation of intradural stimulation efficiency and selectivity in a computational model of spinal cord stimulation.

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Bryan Howell

Full Text Available Spinal cord stimulation (SCS is an alternative or adjunct therapy to treat chronic pain, a prevalent and clinically challenging condition. Although SCS has substantial clinical success, the therapy is still prone to failures, including lead breakage, lead migration, and poor pain relief. The goal of this study was to develop a computational model of SCS and use the model to compare activation of neural elements during intradural and extradural electrode placement. We constructed five patient-specific models of SCS. Stimulation thresholds predicted by the model were compared to stimulation thresholds measured intraoperatively, and we used these models to quantify the efficiency and selectivity of intradural and extradural SCS. Intradural placement dramatically increased stimulation efficiency and reduced the power required to stimulate the dorsal columns by more than 90%. Intradural placement also increased selectivity, allowing activation of a greater proportion of dorsal column fibers before spread of activation to dorsal root fibers, as well as more selective activation of individual dermatomes at different lateral deviations from the midline. Further, the results suggest that current electrode designs used for extradural SCS are not optimal for intradural SCS, and a novel azimuthal tripolar design increased stimulation selectivity, even beyond that achieved with an intradural paddle array. Increased stimulation efficiency is expected to increase the battery life of implantable pulse generators, increase the recharge interval of rechargeable implantable pulse generators, and potentially reduce stimulator volume. The greater selectivity of intradural stimulation may improve the success rate of SCS by mitigating the sensitivity of pain relief to malpositioning of the electrode. The outcome of this effort is a better quantitative understanding of how intradural electrode placement can potentially increase the selectivity and efficiency of SCS

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....

Solitary eyelid schwannoma

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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.

Tongue schwannomas associated with neurofibromatosis type 2

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Harusachi Kanazawa

2018-06-01

Full Text Available Schwannomas are typically solitary benign neural tumors; however, multiple lesions associated with the rare genetic disorder neurofibromatosis type 2 (NF2 have been reported in some cases. We present the case of a tongue schwannomas in a 36-year-old woman previously diagnosed with NF2 with bilateral vestibular schwannomas. To alleviate difficulties with swallowing, tongue nodular masses were surgically removed, and schwannoma was histologically diagnosed. Our patient represents the first case of histologically confirmed tongue schwannoma associated with NF2. This indicates that in patient with NF2, schwannoma may be detected in the oral cavity as well as in other parts of the body. Thus, careful clinical and histological examinations are warranted to identify schwannomas associated with NF2 even in the oral cavity. Keywords: Schwannoma, Tongue, Oral manifestation, Neurofibromatosis type 2 (NF2

A rare adrenal incidentaloma: adrenal schwannoma.

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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.

AN INTERESTING CASE OF ANCIENT SCHWANNOMA

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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

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

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.

Treatment of extramedullary plasmacytoma

International Nuclear Information System (INIS)

Wang Weihu; Li Suyan; Gao Li; Qu Yuan; Xu Guozhen; Li Yexiong

2004-01-01

Objective: To analyze the clinical feature of extramedullary plasmacytoma (EMP) and treatment results. Methods: From Jan. 1960 to Aug. 2000, 28 EMP patients accumulated in a period of 40 odd years were evaluated retrospectively. Sixteen of them were treated with radiotherapy alone (R group, 57.1%) with a median dose of 50 Gy, 11 with combined modality therapy (CMT group, 39.3%) and 1 with surgery alone (S group, 3.6%). Results: During a median follow-up of 90 months (4-280 months), 2 developed local recurrence and 2 were converted to multiple myeloma. The overall 3-, 5-, and 10-year survival rates were 92.6%, 75.6% and 75.6%, respectively. In the R group, the overall 3-, 5-, and 10-year survival rates were 93.8%, 77.3% and 77.3%, as compared with 90.9%, 72.7% and 72.7% in the CMT group (P>0.05). Conclusions: Extramedullary plasmacytoma may well be cured with small field and limited dose of radiation therapy alone. The diagnosis of extramedullary plasmacytoma requires a complete work-up (including MRI) and strict criteria to exclude multiple myeloma. Close follow-up is necessary in those converted to multiple myeloma. (authors)

A Case of Mediastinal Extramedullary Plasmacytoma Associated with Multiple Myeloma

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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.

Surgical treatment of an unusual case of pelvic extramedullary hematopoiesis.

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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.

Extramedullary paraspinal hematopoiesis in thalassemia: CT and MRI evaluation

International Nuclear Information System (INIS)

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

1999-01-01

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

Extramedullary paraspinal hematopoiesis in thalassemia: CT and MRI evaluation

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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.

Perineural Spread of Renal Cell Carcinoma: A Case Illustration with a Proposed Anatomic Mechanism and a Review of the Literature.

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Capek, Stepan; Krauss, William E; Amrami, Kimberly K; Parisi, Joseph E; Spinner, Robert J

2016-05-01

Perineural spread (PNS) is an unusual mechanism of tumor extension and has been typically reported in squamous cell carcinoma, adenocystic carcinoma, and desmoplastic melanoma. Our group has previously demonstrated PNS in rectal, prostate, bladder, and cervical cancer from the primary site along the autonomic nerves to the major somatic nerves and even intradurally. We believe similar principles apply to renal cell carcinoma (RCC) as well, despite the different anatomy. We performed a retrospective search to identify cases of intradural-extramedullary metastases of RCC caused by PNS. Strict anatomic and imaging inclusion criteria were defined: only lesions located between T6 and L3 were included, and PNS as a potential cause had to be supported by imaging evidence. Although 3 cases of spinal intradural metastases were identified, only one met our strict inclusion criteria. A 61-year-old woman developed a late intradural-extramedullary metastasis of RCC 16 years after the original diagnosis that we believe represents an example of visceral organ PNS. RCC can propagate via PNS from the primary tumor along the autonomic nerves to the aorticorenal, celiac, and mesenteric ganglia and then along the thoracic and lumbar splanchnic nerves to the corresponding spinal nerves and intradurally. We present radiologic evidence together with the review of the literature to support the premise that PNS of RCC not only occurs but goes unrecognized. Copyright © 2016 Elsevier Inc. All rights reserved.

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

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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.

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

CT findings of adrenal schwannoma

International Nuclear Information System (INIS)

Zhang, Y.-M.; Lei, P.-F.; Chen, M.-N.; Lv, X.-F.; Ling, Y.-H.; Cai, P.-Q.; Gao, J.-M.

2016-01-01

Aim: To analyse the computed tomography (CT) imaging features of patients with adrenal schwannoma. Materials and methods: Eight cases of adrenal schwannoma confirmed by histopathology were included in this study. All eight patients had undergone multiphase CT examinations. The features of the adrenal schwannoma in the CT images were analysed retrospectively in detail, including size, shape, margin, radiodensity, calcification, and enhancement pattern. Results: There were six male and two female patients, with a median age of 44.5 years (range, 25–52 years). Two patients complained of right flank pain, and two with left upper abdominal discomfort, while the remaining patients were diagnosed by routine ultrasound examinations. On unenhanced CT images, all cases of adrenal schwannoma were well circumscribed, rounded or oval, heterogeneous masses with cystic components, with two cases exhibiting calcification, and three cases with septa. On enhanced CT images, all cases displayed mild heterogeneous enhancement of the tumour during the arterial phase, and progressive enhancement during the portal venous phase and equilibrium phase. Conclusion: Adrenal schwannoma commonly presents as a well-defined unilateral mass with cystic degeneration, septa, and a characteristic progressive contrast-enhancement pattern on multiphase enhanced scans. - Highlights: • Adrenal schwannomas were extremely rare, and eight cases' medical data of this disease were collected in this study. • They usually presented a well-defined unilateral mass with cystic degeneration and sepations. • They manifested characteristic progressive contrast enhancement pattern on enhanced CT images.

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)

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 case of a massive mandibular schwannoma

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Shishir Ram Shetty

2011-01-01

Full Text Available Schwannoma, a benign nerve sheath tumor is relatively rare in occurrence and even rarer in sites, such as jaw bones. There are only 45 reported cases of intraosseous schwannoma of the jaws reported in the literature. We report a rare case of mandibular schwannoma in a 50-year-old Indian male. The clinical features resembled that of a residual cyst, fibro-osseous lesion or an odontogenic tumor/cyst. Radiological differential diagnoses of ameloblastoma or odontogenic keratocyst was made based on the findings of the orthopantomogram. The lesion was examined histopathologically and a final diagnosis of schwannoma arising from the inferior alveolar nerve was made. The aim of this report is to add information to the existing sparse literature on intraosseous schwannomas of the jaw.

Schwannomas of the head and neck

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Anastasios Kanatas

2011-12-01

Full Text Available Schwannomas are benign encapsulated nerve sheath tumors composed of Schwann cells. Malignant change in head and neck schwannomas is rare, with the incidence varying between 8 and 13.9%. In this review, we discuss the presentation and the management of head and neck schwannomas. The issues and difficulties based on our own experience as well as the experience of published reports from the literature are presented.

True incidence of vestibular schwannoma?

DEFF Research Database (Denmark)

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

2010-01-01

The incidence of diagnosed sporadic unilateral vestibular schwannomas (VS) has increased, due primarily to more widespread access to magnetic resonance imaging.......The incidence of diagnosed sporadic unilateral vestibular schwannomas (VS) has increased, due primarily to more widespread access to magnetic resonance imaging....

Metastatic disease of the spine: MR evaluation with Gd-DTPA

International Nuclear Information System (INIS)

Sze, G.; Abramson, A.; Krol, G.; Zimmerman, R.D.; Deck, M.D.F.

1987-01-01

Gd-DTPA was used in the MR evaluation of 22 patients with metastatic disease of the spine. Ten patients had epidural lesions; ten patients had intradural extramedullary spread of tumor; two patients had intramedullary metastases. A 1.5-T superconductive magnet with surface coils was used to obtain 3- or 5-mm sagittal and axial sections of the spine. All patients also received myelography and postmyelographic CT. Gd-DTPA was very sensitive to intradural extramedullary disease. Both nodular metastases and nerve root thickening enhanced. Lesions often poorly defined or not visible at all without contrast were easily seen after administration of gadolinium. Gadolinium was also of benefit in patients with intramedullary metastases. Although abnormalities of cord contour and signal were present in noncontrast studies, the region of enhancement was more localized and focal, suggesting that much of the cord enlargement was due to edema. In patients with extradural disease, gadolinium proved to be useful in selected cases. Generally, epidural tumor extension was visible without contrast. In specific cases, administration of gadolinium was beneficial, for example in localizing regions of tumor for biopsy or in distinguishing possible herniated nucleus pulposus from epidural disease. In conclusion, gadolinium is of significant benefit in intradural extramedullary disease. Metastatic lesions of the cord itself may also be more fully evaluated with contrast scans. However, epidural disease will probably prove to be adequately studied without contrast, although gadolinium may be of use in selected applications

Magnetic resonance imaging of the intradural prepontine chordoma mimicking an epidermoid cyst: Pathologic correlation

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Cho, Hyoun; Yu, In Kyu; Kim, Seung Min; Kim, Han Kyu [Eulji Univ. Hospital, Daejeon (Korea, Republic of)

2012-08-15

Intracranial chordomas, originating from remnants of the primitive notochord, are extradural tumors arising mostly at the sphenooccipital synchondrosis in the clivus. We present an unusual case of intradural chordoma at the prepontine cistern, with parenchymal compressive invasion to the pons. It was excised subtotally, followed by a second operation due to the increasing remnant tumor size during 8 months. A differential diagnosis for intradural chordoma must be considered when the preoperative MRI features are not consistent with an epidermoid cyst if there are multiple fine enhancing lesions on enhanced magnetic resonance images and no bright signal intensity on diffusion weighted images. This report is concerned with the radiological findings in the intradural chordoma and the differential diagnosis focused on the epidermoid cyst.

Magnetic resonance imaging of the intradural prepontine chordoma mimicking an epidermoid cyst: Pathologic correlation

International Nuclear Information System (INIS)

Cho, Hyoun; Yu, In Kyu; Kim, Seung Min; Kim, Han Kyu

2012-01-01

Intracranial chordomas, originating from remnants of the primitive notochord, are extradural tumors arising mostly at the sphenooccipital synchondrosis in the clivus. We present an unusual case of intradural chordoma at the prepontine cistern, with parenchymal compressive invasion to the pons. It was excised subtotally, followed by a second operation due to the increasing remnant tumor size during 8 months. A differential diagnosis for intradural chordoma must be considered when the preoperative MRI features are not consistent with an epidermoid cyst if there are multiple fine enhancing lesions on enhanced magnetic resonance images and no bright signal intensity on diffusion weighted images. This report is concerned with the radiological findings in the intradural chordoma and the differential diagnosis focused on the epidermoid cyst

MR findings in intralabyrinthine schwannomas

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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)

MR findings in intralabyrinthine schwannomas

International Nuclear Information System (INIS)

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

2002-01-01

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)

MR findings in intralabyrinthine schwannomas

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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)

Schwannoma Originating From the Periphereral Intercostal Nerves

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Yunus Aksoy

2017-06-01

Full Text Available Schwannomas are usually solitary, encapsulated, and asymptomatic, benign neurogenic tumors originating from the nerve sheath. Schwannomas rarely show malignant transformation, however, require close monitoring. They are primarily located in the thorax in the costovertebral sulcus, may rarely originate from peripheral intercostal nerves. Less than 10% of primary thoracic neurogenic tumors originate from the peripheral intercostal nerves. The main treatment and diagnosis of schwannomas are complete surgical resection. We report a rare case of a 40-year-old male with asymptomatic schwannoma originating from an intercostal nerve which was found incidentally on his chest X-ray and was treated with surgery.

Extramedullary Hematopoiesis: An Unusual Finding in Subdural Hematomas

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Rong Li

2011-01-01

Full Text Available We present a case of a 59-year-old man who was found to have clusters of hyperchromatic, small, round nucleated cells within a subdural hematoma removed after a skull fracture. Immunohistochemistry study confirmed that the cells were hematopoietic components predominantly composed of normoblasts. In this paper, we describe the clinical and pathological findings. A brief review of published information on extramedullary hematopoiesis in subdural hematoma and the mechanisms of pathogenesis are also discussed. While extramedullary hematopoiesis is seen anecdotally by neuropathologists in chronic subdural hematomas, only a few cases are documented in the literature. Furthermore, extramedullary hematopoiesis in subdural hematoma can pose a diagnostic challenge for general pathologists who encounter subdural hematoma evacuations seldom in their surgical pathology practices.

Schwannoma in the vestibule and cochlea

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Susilawati, S. [Fatmawati Hospital, Jakarta (Indonesia). Department of Ear, Nose and Throat; Adler, J. [Sutherland Imaging Centre, Sydney, NSW (Australia); Fagan, P. [St Vincents Hospital, Darlinghurst, NSW (Australia)

1997-05-01

Schwannoma of the vestibule or the cochlea is an unusual lesion. In the past, most examples have been found at autopsy or as unsuspected findings at surgery for vertigo. The symptoms of isolated labyrinthine schwannoma may be indistinguishable from advanced Meniere`s disease. Magnetic resonance imaging has led to pre-operative diagnosis in some cases. Two cases of schwannoma within the labyrinth from a series of 339 symptomatic acoustic tumours, are presented and the imaging findings are discussed. 8 refs., 2 figs.

Unusually large quiescent ancient schwannoma of hypoglossal nerve

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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.

An arachnoid cyst presenting as an intramedullary tumour

NARCIS (Netherlands)

Willems, P. W.; van den Bergh, W. M.; Vandertop, W. P.

2000-01-01

A case of thoracic intradural extramedullary arachnoid cyst is presented in which an intramedullary low grade glioma was suspected preoperatively. The cyst was widely fenestrated and postoperatively, the patient experienced considerable improvement in her symptoms. As postoperative MRI studies also

CT findings of esophageal schwannoma: A case report

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Lee, Man Ho; Ryu, Dae Shick; Eom, Dae Woon; Shin, Dong Rock; Choi, Soo Jung; Ahn, Jae Hong; Park, Man Soo; Yoo, Dong Kon [Gangneung Asan Hospital, College of Medicine, University of Ulsan, Gangneung (Korea, Republic of)

2015-03-15

Esophageal schwannomas are a relative rare benign neoplasm that usually occurs in the upper esophagus, in the middle aged women. We report a case of a 67-year-old man with a lower esophageal schwannoma. This lesion was composed of homogenous density, iso-attenuating with the chest wall muscle on pre- and post-contrast chest computed tomography (CT). The CT findings of the esophageal schwannoma are similar to those of esophageal leiomyoma. Hense, esophageal schwannoma may be a differential diagnosis with esophageal leiomyoma.

Atypical Manifestation of Vestibular Schwannoma

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Webster, Guilherme

2013-09-01

Full Text Available Introduction: Vestibular schwannoma (also known as acoustic neuroma is a benign tumor whose cells are derived from Schwann sheaths, which commonly occurs from the vestibular portion of the eighth cranial nerve. Furthermore, vestibular schwannomas account for ∼8% of intracranial tumors in adults and 80 to 90% of tumors of the cerebellopontine angle. Its symptoms are varied, but what stands out most is a unilateral sensorineural hearing loss, with a low index of speech recognition. Objective: Describe an atypical manifestation of vestibular schwannoma. Case Report: The 46-year-old woman had vertigo and binaural hearing loss and fullness, with ear, nose, and throat examination suggestive of cochlear injury. After 6 months, the patient developed worsening of symptoms and onset of right unilateral tinnitus. In further exams the signs of cochlear damage remained, except for the vestibular test (hyporeflexia. Magnetic resonance imaging showed an expansive lesion in the right cerebellopontine angle. Discussion: This report warns about the atypical manifestations of vestibular schwannoma, which must always be remembered in investigating and diagnosing hearing loss.

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

Clinicopathological and Immunohistochemical Characterisation of Gastric Schwannomas in 29 Cases

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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.

Intracranial extramedullary hematopoiesis: a rare cause of headaches.

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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.

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.

Extramedullary versus intramedullary tibial cutting guides in megaprosthetic total knee replacement

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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.

Solitary Schwannoma in the breast: a case report

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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.

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.

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.

Solitary extramedullary plasmacytoma of the sinonasal region.

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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.

Stereotactic radiosurgery: the preferred management for patients with nonvestibular schwannomas?

International Nuclear Information System (INIS)

Pollock, Bruce E.; Foote, Robert L.; Stafford, Scott L.

2002-01-01

Purpose: To review patient outcomes after radiosurgery of nonvestibular schwannomas. Methods and Materials: From April 1992 to February 2000, 23 patients had radiosurgery at our center for nonvestibular schwannomas. Affected cranial nerves included the trochlear (n=1), trigeminal (n=10), jugular foramen region (n=10), and hypoglossal (n=2). Nine patients had undergone one or more prior tumor resections. One patient had a malignant schwannoma; 2 patients had neurofibromatosis. The median prescription isodose volume was 8.9 cc (range, 0.2 to 17.6 cc). The median tumor margin dose was 18 Gy (range, 12 to 20 Gy); the median maximum dose was 36 Gy (range, 24 to 40 Gy). The median follow-up after radiosurgery was 43 months (range, 12 to 111 months). Results: Twenty-two of 23 tumors (96%) were either smaller (n=12) or unchanged in size (n=10) after radiosurgery. One patient with a malignant schwannoma had tumor progression outside the irradiated volume despite having both radiosurgery and fractionated radiation therapy (50.4 Gy); he died 4 years later. Morbidity related to radiosurgery occurred in 4 patients (17%). Three of 10 patients with trigeminal schwannomas suffered new or worsened trigeminal dysfunction after radiosurgery. One patient with a hypoglossal schwannoma had eustachian tube dysfunction after radiosurgery. No patient with a lower cranial nerve schwannoma developed any hearing loss, facial weakness, or swallowing difficulty after radiosurgery. Conclusions: Although the reported number of patients having radiosurgery for nonvestibular schwannomas is limited, the high tumor control rates demonstrated after vestibular schwannoma radiosurgery should apply to these rare tumors. Compared to historical controls treated with surgical resection, radiosurgery appears to have less treatment-associated morbidity for nonvestibular schwannomas, especially for schwannomas involving the lower cranial nerves

Obturator nerve schwannoma presenting as an adnexal mass: case report

Energy Technology Data Exchange (ETDEWEB)

Mehta, M.; Thurston, W.A.; Merchant, N. [The Toronto Hospital, Dept. of Medical Imaging, Toronto, Ontario (Canada); Murphy, K.J. [The Toronto Hospital, Dept. of Obstetrics and Gynecology, Toronto, Ontario (Canada)

1999-02-01

Schwannomas are relatively common, benign nerve-sheath tumours. They arise most commonly from either cranial nerves or the dorsal root of spinal nerves. Schwannomas have also been reported to occur in peripheral nerve-root trunks, although this location is much less common. We report a case of a 45-year-old woman with a large pelvic mass originally believed to be an ovarian tumour. Following surgical excision, the tumour was found to be a schwannoma of the obturator nerve. To our knowledge, there are no reported cases of an obturator nerve schwannoma. The imaging features of schwannomas are reviewed. (author)

Localized extramedullary relapse after autologous hematopoietic stem cell transplantation in multiple myeloma

International Nuclear Information System (INIS)

Erkus, Muhan; Meteoglu, Ibrahim; Bolaman, Zahit; Kadikoylu, Gurhan

2005-01-01

Extramedullary plasmacytomas are rare manifestation of plasma cell malignancies. After hematopoietic stem cell transplantation HSCT, presentation of localized plasmacytoma with extramedullary growth is very unusual. We report a case of a 56-year-old woman with Dune-Salmon stage IIIA immunoglobulin A-kappa multiple myeloma, which presented 120 days after autologous HSCT with extramedullary plasmacytoma arising from a lymph node in supraclavicular region. The patient had no pretransplant-history related with extramedullary disease. There was no increase of plasma cells in bone marrow or monoclonal protein in urine or serum. Aspiration smears of lymph node revealed a population of plasmacytoid cells at various stages of maturation. The patient was successfully treated with local radiotherapy and has remained progression-free for more than 20 months. (author)

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.

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.

CT and MR features of the intracranial Schwannomas

International Nuclear Information System (INIS)

Jung, So Lyung; Ro, Hee Jeong; Lee, Hong Jae; Jung, Seung Eun; Byun, Jae Young; Yang, Il Kwon; Lee, Han Jin; Choi, Kyu Ho; Kim, Jong Woo; Shinn, Kyung Sub

1996-01-01

To evaluate CT and MR findings of the intracranial schwannomas arising from variable cranial nerves. The authors retrospectively analyzed CT (n=21) and MR (n=15) findings of 24 cases in 23 patients (M : 7, F : 16) who had suffered from surgically-proven intracranial schwannomas over the previous five years. Schwannomas arose from the acoustic nerve(n=18), the trigeminal nerve(n=2), the glossopha-ryngeal-vagal-accessory nerve complex (n=2), and the olfactory nerve(n=1). Intracranial schwannomas were well defined, ,lobulated and inhomogeneously or homogeneously enhancing masses on CT and MR, and were located along the course of the specific cranial nerve. Acoustic schwannomas involved both the internal auditory canal(IAC) and the cerebellopontive angle(CPA) in 14 case, the IAC in three, and the SPA in two. Two trigeminal schwannomas involved both middle and posterior cranial fossa and were in the shape of a dumbbell. One of the two schwannomas that invelved lower cranial nerve complex(9-11th) was located in the medullary cistern and jugular foramen ; the other was located in the central posterior cranial fossa. A case of olfactory schwannoma was located in the right cribriform plate. The precontrast CT scan showed low density in 13 cases (62%), isodensity in seven(33%) and high density in one(5%). on postcontrast CT scan, enhancement was seen in 20 cases(95%). Of the 15 cases with MR, 2 had low signal intensity on T1 weighted image and 14 had high signal intensity on T2 weighted image. MR imaging after Gd-DTPA infusion showed enhancement in 14 cases. Enhancement was inhomogeneous in 14 cases on CT and in 13 on MR. Of 24 cases, intratumoral necrosis was seen in 19, ring enhancement in five and severe cystic change in one. Other findings were in tratumoral calcification (21%), hemorrhage(8%), pressure bony erosion(70.8%), midline shift(58%), peritumoral edema(29%) and hydrocephalus(33%). On MR, there was in all 15 cases a peritumoral low signal intensity rim on T1-and

Quantitative and qualitative temporal evolution of gadolinium enhancement of spinal lesion

International Nuclear Information System (INIS)

Sze, G.; Krol, G.

1988-01-01

Seventy gadolinium-enhanced studies of spinal lesions were reviewed, and 34 were selected for analysis of the temporal quantitative and qualitative aspects of enhancement. Thirteen patients had intradural extramedullary lesions, eight had intramedullary lesions, and 13 had extradural lesions. Data analysis was conducted by measuring intensities of normal and pathologic tissues on short repetition-time (TR) scans. The evolution of enhancement of spinal tumors was found to differ, depending on which compartment the tumor was located. Intradural extramedullary nodules showed their most prominent enhancement on immediate postcontrast scans. Extradural lesions varied in behavior. Some demonstrated an increase in enhancement, while others showed a decrease. Intramedullary tumors usually showed an increase in enhancement on delayed scans. In most cases, however, the quantitative increase was minor. The exception occurred in a case of necrotic cord glioma. Immediate postcontrast short-TR scans will nearly always be sufficient for the evaluation of suspected spinal lesions. Only the very occasional case may benefit from delayed scans

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.

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...

Vulval schwannoma: A cytological diagnosis

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Das Subhashish

2008-01-01

Full Text Available Schwannomas are the most common peripheral nerve sheath tumors, the frequent sites of involvement being the head and neck, the flexor aspect of the limbs, especially near the elbow, wrist, knee, and trunk. Involvement of the female genital tract is extremely rare. We present one such rare case of a solitary vulval schwannoma which was diagnosed by fine needle aspiration cytology in a 48 year-old female, and confirmed by histopathological examination.

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....

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

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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.

Extramedullary plasmacytoma in the trachea of a dog.

Science.gov (United States)

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.

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

Science.gov (United States)

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.

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.

Atypical presentations of retroperitoneal giant schwannomas

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Sait Ozbir

2011-06-01

Full Text Available Schwannomas are usually benign rare tumors that originating from Schwann cells of peripheral nerve sheaths. Presentation is generally varied and changed in a non-specific range from abdominal mass, flank pain to incidental findings. Herein we report 2 cases of retroperitoneal giant schwannomas with different clinical presentations, of whom one presented with vague abdominal pain, palpable abdominal mass for 4 years, swelling and bilateral hydronephrosis that caused by giant abdominal mass; the other one presented with right flank pain, rectal hemorrhage and lower extremities edema. Two patients were treated by complete surgical excision of masses. The histological and immunohistochemical diagnosis was reported as benign schwannoma. Both of patients are doing well and had no recurrence in 9 years and 28 months follow-up, respectively.

Soft Tissue Extramedullary Plasmacytoma

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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.

Retroperitoneal Schwannoma: A Rare Case

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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.

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.

Distinct spontaneous shrinkage of a sporadic vestibular schwannoma

DEFF Research Database (Denmark)

Huang, Xiaowen; Cayé-Thomasen, Per; Stangerup, Sven-Eric

2013-01-01

on "shrinkage" or "negative growth" or "regression" or "involution" of the tumor were selected, and the contents on the rate, extent and mechanism of spontaneous tumor shrinkage were extracted and reviewed. The reported rate of spontaneous shrinkage of vestibular schwannoma is 5-10% of patients managed......We present a case with outspoken spontaneous vestibular schwannoma shrinkage and review the related literature. The patient was initially diagnosed with a left-sided, intrameatal vestibular schwannoma, which subsequently grew into the cerebello-pontine angle (CPA), followed by total shrinkage...... of the CPA component without any intervention over a 12-year observation period. The literature on spontaneous tumor shrinkage was retrieved by searching the subject terms "vestibular schwannoma, conservative management" in PubMed/MEDLINE database, without a time limit. Of the published data, the articles...

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.

Primary extramedullary plasmacytoma of the penis: a case report.

Science.gov (United States)

Wang, Yao; Li, Hong-Yan; Liang, Ting-Ting; Han, Yu-Ping; Wang, Xue-Ju; Wei, Xin; Fan, Li; Wang, Wei-Hua

2013-10-01

Extramedullary plasmacytoma involving the penis is extremely rare. Here, we describe a case of primary extramedullary plasmacytoma of the penis in a 64-year-old man who presented with a palpable penile mass. Nuclear magnetic resonance imaging revealed the presence of a large, round non-encapsulated mass in the perineum. A contrast-enhanced computed tomography scan of the pelvis showed that the mass was located in the tunica albuginea and corpora cavernosa at the base of the penis. The mass encased the urethra and demonstrated no marked enhancement during the arterial phase. The patient underwent successful surgical resection of the tumor. Histologically, the tumor was composed primarily of neoplastic plasma cells that were positive for CD38, vimentin and Ki 67. Postoperatively, the patient recovered well and exhibited no evidence of development of multiple myeloma, local recurrence or distant metastasis at 2 months post-surgery. To the best of our knowledge, our case represents the first documented case of human primary extramedullary plasmacytoma of the penis.

Vestibular schwannoma with contralateral facial pain – case report

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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.

Rare presentation of pancreatic schwannoma: a case report

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Tofigh Arash

2008-08-01

Full Text Available Abstract Introduction Schwannoma is a rare tumor among pancreatic neoplasms. Schwannomas vary in size, and most of them are cystic, mimicking pancreatic cystic lesions. Generally, a definitive diagnosis is made at the time of histological analysis. The mainstay treatment is surgical resection. Case presentation We report an unusual presentation of pancreatic schwannoma with abdominal pain and several episodes of cholangitis in a 54-year-old Caucasian (Iranian man. The condition was not diagnosed pre-operatively and Whipple's procedure was performed. Conclusion Pancreatic schwannoma is an important clinical entity to include in the differential diagnosis of pancreatic lesions. Pre-operative diagnosis is difficult but computed tomographic findings may be helpful. The tumor may also have atypical and rare presentations, such as cholangitis and weight loss. For benign tumors, simple enucleation is usually adequate, whereas malignant tumors require standard oncological resection.

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

International Nuclear Information System (INIS)

Aydingoez, Ue.; Oto, A.; Cila, A.

1997-01-01

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.)

Management of advanced intracranial intradural juvenile nasopharyngeal angiofibroma: combined single-stage rhinosurgical and neurosurgical approach.

Science.gov (United States)

Naraghi, Mohsen; Saberi, Hooshang; Mirmohseni, Atefeh Sadat; Nikdad, Mohammad Sadegh; Afarideh, Mohsen

2015-07-01

Although intracranial extension of juvenile nasopharyngeal angiofibroma (JNA) occurs commonly, intradural penetration is extremely rare. Management of such tumors is a challenging issue in skull-base surgery, necessitating their removal via combined approaches. In this work, we share our experience in management of extensive intradural JNA. In a university hospital-based setting of 2 tertiary care academic centers, retrospective chart of 6 male patients (5 between 15 and 19 years old) was reviewed. Patients presented chiefly with nasal obstruction, epistaxis, and proptosis. One of them was an aggressive recurrent tumor in a 32-year-old patient. All cases underwent combined transnasal, transmaxillary, and craniotomy approaches assisted by the use of image-guided endoscopic surgery, with craniotomy preceding the rhinosurgical approach in 3 cases. Adding a transcranial approach to the transnasal and transmaxillary endoscopic approaches provided 2-sided exposure and appreciated access to the huge intradural JNAs. One postoperative cerebrospinal fluid leak and 1 postoperative recurrence at the site of infratemporal fossa were treated successfully. Otherwise, the course was uneventful in the remaining cases. Management of intracranial intradural JNA requires a multidisciplinary approach of combined open and endoscopic-assisted rhinosurgery and neurosurgery, because of greater risk for complications during the dissection. Carotid rupture and brain damage remain 2 catastrophic complications that should always be kept in mind. A combined rhinosurgical and neurosurgical approach also has the advantage of very modest cosmetic complications. © 2015 ARS-AAOA, LLC.

Gastric Schwannoma: a case report

International Nuclear Information System (INIS)

Lee, Kye Ho; Jee, Keum Nahn

2006-01-01

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

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.

MRI findings of extramedullary haemopoiesis

International Nuclear Information System (INIS)

Chourmouzi, D.; Pistevou-Gompaki, K.; Plataniotis, G.; Skaragas, G.; Papadopoulos, L.; Drevelegas, A.

2001-01-01

Extramedullary haemopoiesis (EH) is a compensatory process associated with chronic haemolytic anaemia. It is rare, however, for such an abnormality to cause spinal cord compression. We present two patients with known beta-thalassaemia intermedia who developed spinal cord compression due to masses of extramedullary haematopoietic tissue in the epidural space of the thoracic spine. The EH masses were diagnosed by MRI as an isointense epidural lesion on both T1- and T2-weighted images, compressing severely the spinal cord. After administration of a paramagnetic agent, an intermediate enhancement of the masses was evident. All the vertebral bodies had low to intermediate signal intensity as a result of displacement of fatty marrow by haematopoietic marrow. Expansion of thoracic ribs with bilateral paravertebral masses were characteristic. A small dose of radiotherapy was given and marked improvement in neurological symptoms was evident. An MRI examination established shrinkage of the mass and decompression of spinal cord. The role of MRI in diagnosis of EH masses is essential and radiation therapy is a very effective treatment for this rare complication. (orig.)

Extramedullary plasmacytomas in the context of multiple myeloma.

Science.gov (United States)

Aguado, Beatriz; Iñigo, Belen; Sastre, Jose L; Oriol, Albert

2011-11-01

Plasmacytoma is a frequent complication of multiple myeloma, either at diagnosis or within disease progression. The extramedullary disease confers a poorer prognosis and is biologically distinct with high-risk molecular and histological features, being resistant to conventional treatments. Radiation therapy remains the most effective treatment for extramedullary lesions to achieve local control. There are very limited data from randomized trials regarding the most appropriate systemic treatment. Case reports such as those presented here, as well as retrospective analysis of series, suggest that lenalidomide is an effective agent, in combination with dexamethasone, in this setting. Additional studies are needed to define the proper management of this condition.

Schwannoma of the adrenal gland

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Anunayi Jeshtadi

2014-07-01

Full Text Available Visceral schwannomas are extremely rare and are usually discov-ered incidentally on USG/CT-Scan. Primary schwannomas of the adrenal gland are extremely uncommon. It has been theorized that they originate from Schwann cells that insulate the nerve fi-bers innervating the adrenal medulla. Histopathological examina-tion coupled with immunohistochemistry provides the definitive diagnosis. A 55 year old normotensive female presented with pain in the right loin since 5 months. Her renal parameters were normal. Contrast enhanced computed tomography of abdomen showed a well delineated 6.5 x 5cms mass at upper pole of her right kidney. 24-hour urinary metanephrine was slightly elevated (3.07mg/24hrs. A decline in Serum cortisol levels was observed following a dexamethasone suppression test (18.89nmol/l. Histopathological examination revealed a spindle cell tumor. Immunohistochemistry showed strong and diffuse positive staining for S-100 with negative expression for CD-117, desmin, CD-34, HMB-45, synaptophysin, chromogranin, cytokeratin, and SMA. Ki-67 index was 2%.A diagnosis of cellular schwannoma of adrenal gland was confirmed.

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.

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

Functional Nerve Preservation in Extracranial Head and Neck Schwannoma Surgery.

Science.gov (United States)

Ijichi, Kei; Kawakita, Daisuke; Maseki, Shinichiro; Beppu, Shintaro; Takano, Gaku; Murakami, Shingo

2016-05-01

A schwannoma is an uncommon, benign neurogenic tumor of Schwann cells. Tumor enucleation is the recommended surgical method to preserve function of the original nerve, although enucleation does not guarantee completely intact nerve function after the operation. To establish a strategy for functional preservation in extracranial head and neck schwannoma treatment by using an electromyographic (EMG) system during tumor resection. A retrospective cohort study was conducted of 15 patients who underwent surgery for removal of schwannoma tumors between April 1, 2006, and March 31, 2015, at an academic tertiary referral center. Data analysis was conducted from April 3, 2006, to September 15, 2015. Neurogenic tumors were diagnosed according to preoperative findings, and during surgery tumors were exposed and given EMG-controlled electrical stimulation to analyze their origins. In motor nerve cases, the electrical activity of the muscle was measured and recorded by EMG. The tumor was then enucleated by incision along tumor fibers mapped using EMG stimulation. If a nerve bundle was visible, we incised along there and enucleated the tumor. A strategy using electrical stimulation to improve preservation of nerve function in extracranial head and neck schwannoma operations. Frequency and duration of postoperative neurologic complications associated with functional preservation surgery with tumor enucleation was evaluated using EMG monitoring according to tumor origin. Of the 15 patients with extracranial schwannoma, 9 (60%) were women (mean [SD] age, 36.3 [15.3] years). All 15 patients underwent surgery using a transcervical approach. The most common nerves of origin were the vagus nerve and the sympathetic chain. In sensory or sympathetic nerve cases, the EMG response was absent. Two of 5 patients with vagus schwannoma had postoperative temporary vocal nerve palsy. These symptoms showed improvement after 1 year. There was no tumor recurrence during the follow-up period in any

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

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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.

Malignant Transformation of Vagal Nerve Schwannoma in to ...

African Journals Online (AJOL)

Vagal schwannomas are benign, rare peripheral nerve sheath tumors in the head and neck region. Some physicians opt to closely observe cases of schwannoma of the neck on an outpatient basis rather than to perform radical surgery. However, there is a possibility, albeit rare, of malignant transformation of a.

46-year-old man with a spinal cord mass.

Science.gov (United States)

Sanders, Mary Ann; Vitaz, Todd; Rosenblum, Marc; Plaga, Alexis R; Parker, Joseph C; Parker, John R

2011-01-01

Medulloblastoma accounts for only 1% of all adult CNS tumors. Likewise, recurrence of adult medulloblastoma greater than 20 years after initial diagnosis is extremely rare.We describe a case of adult medulloblastoma with late relapse of disease. The patient was 24 years old when first diagnosed and was treated with total tumor resection and craniospinal radiation. At the age of 45, an enhancing 1.3 cm intradural extramedullary spinal cord lesion at T5 was discovered on MRI. This was presumed to be recurrent medulloblastoma in the form of drop metastasis and the patient was treated with spinal radiation. Several months following treatment, at the age of 46, a follow-up MRI demonstrated an enhancing 1.4 cm intradural extramedullary spinal cord lesion at T7. The lesion was resected and histopathologic examination was most consistent with medulloblastoma, late drop metastasis. Although rare, adult medulloblastoma recurring 20 years after initial diagnosis should always be considered in the main differential diagnosis when working up CNS lesions at or outside the primary tumor site.

Laryngeal schwannoma: a case report with emphasis on sonographic findings

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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)

Tracheal schwannoma: Completely resected with therapeutic bronchoscopic techniques

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Barney Thomas Jesudason Isaac

2015-01-01

Full Text Available Tracheal schwannomas are rare benign tumors of the trachea. There are only a few reported cases in the literature. Surgeons have generally resected these tumors, whereas bronchoscopists have attempted to remove them bronchoscopically. We report a case of tracheal schwannoma which was completely resected using bronchoscopic techniques.

Hérnias intradural de discos lombares: relato de dois casos

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Joel M. Guelmann

1972-09-01

Full Text Available São relatados dois casos de hérnia de disco lombar intradural. Os autores salientam a raridade desta ocorrência e fazem comentários sobre os fatores que podem determina-la. Ambos os pacientes foram operados, com extirpação total da hérnia. Houve completa recuperação dos pacientes.

Schwannoma of the sublingual gland: report of a case

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Lee, Eun Sook; Choi, Soon Chul; Park, Tae Won; You, Dong Soo [Dept. of Oral and Maxillofacial Radiology, College of Dentistry, Seoul National University, Seoul (Korea, Republic of)

1994-08-15

Schwannoma, also referred to as neurilemmoma, is a solitary, benign and slow growing tumor of nerve sheath origin. This rare lesion originates from Schwann cell of peripheral, autonomic and cranial nerve. Extracranial neurogenic tumor of the head and neck is uncommon. Schwannoma of the salivary gland is a particularly rare form of an extracranial neurogenic tumor, with most presenting in the parotid gland originating from a peripheral branch of the facial nerve. In this report, an unusual case of schwannoma in the sublingual region is presented and the literature concerning this subject is reviewed.

Malignant Transformation of Vagal Nerve Schwannoma in to ...

African Journals Online (AJOL)

Schwannomas are benign, rare peripheral nerve sheath tumors that occur in the head and neck region. Some physicians opt to closely observe cases of schwannoma of the neck on an outpatient basis rather than to perform radical surgery. However, there is a possibility, albeit rare, of malignant transformation of a benign ...

Facial Nerve Schwannoma of the Cerebellopontine Angle: A Diagnostic Challenge

OpenAIRE

Lassaletta, Luis; Roda, José María; Frutos, Remedios; Patrón, Mercedes; Gavilán, Javier

2002-01-01

Facial nerve schwannomas are rare lesions that may involve any segment of the facial nerve. Because of their rarity and the lack of a consistent clinical and radiological pattern, facial nerve schwannomas located at the cerebellopontine angle (CPA) and internal auditory canal (IAC) represent a diagnostic and therapeutic challenge for clinicians. In this report, a case of a CPA/IAC facial nerve schwannoma is presented. Contemporary diagnosis and management of this rare lesion are analyzed.

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

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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.

Synchronous infiltrating ductal carcinoma and primary extramedullary plasmacytoma of the breast

Directory of Open Access Journals (Sweden)

Liu Yan-Xue

2009-04-01

Full Text Available Abstract Background Extramedullary plasmacytomas are seldom solitary and usually progress to diffuse myelomatosis. Plasmacytomas of the breast are rare, especially when not associated multiple myeloma. Synchronous infiltrating ductal carcinoma and primary extramedullary plasmacytoma of the breast have not previously reported. Case presentation A 27-years-old woman with an untreated upper outer quadrant breast mass for 1-year was referred to our cancer hospital for surgical evaluation of increasing breast pain. Postoperatively, microscopic examination revealed an infiltrating ductal carcinoma complicated by an extramedullary plasmacytoma divided by fibrous tissue in one section. Following surgery, the patient received chemotherapy for the carcinoma and radiotherapy for the plasmacytoma. Conclusion In this case, careful histopathology examination was essential to make the correct diagnosis and therapy for these synchronous lesions. The patient finished chemotherapy and radiotherapy without significant adverse effects.

A Case Report of Schwannoma Presenting as Sciatica

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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.

Spinal accessory nerve schwannomas masquerading as a fourth ventricular lesion

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Shyam Sundar Krishnan

2015-01-01

Full Text Available Schwannomas are benign lesions that arise from the nerve sheath of cranial nerves. The most common schwannomas arise from the 8 th cranial nerve (the vestibulo-cochlear nerve followed by trigeminal and facial nerves and then from glossopharyngeal, vagus, and spinal accessory nerves. Schwannomas involving the oculomotor, trochlear, abducens and hypoglossal nerves are very rare. We report a very unusual spinal accessory nerve schwannoma which occupied the fourth ventricle and extended inferiorly to the upper cervical canal. The radiological features have been detailed. The diagnostic dilemma was due to its midline posterior location mimicking a fourth ventricular lesion like medulloblastoma and ependymoma. Total excision is the ideal treatment for these tumors. A brief review of literature with tabulations of the variants has been listed.

Translabyrinthine surgery for disabling vertigo in vestibular schwannoma patients

NARCIS (Netherlands)

Godefroy, W. P.; Hastan, D.; van der Mey, A. G. L.

2007-01-01

To determine the impact of translabyrinthine surgery on the quality of life in vestibular schwannoma patients with rotatory vertigo. Prospective study in 18 vestibular schwannoma patients. The study was conducted in a multispecialty tertiary care clinic. All 18 patients had a unilateral

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

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

Science.gov (United States)

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

Liver schwannoma incidentally discovered in a patient with breast cancer.

Science.gov (United States)

Akin, Murat; Bozkirli, Bahadir; Leventoglu, Sezai; Unal, Kemal; Kapucu, L Ozlem; Akyurek, Nalan; Sare, Mustafa

2009-01-01

Benign schwannomas, also referred to as neurilemomas, neurinomas, and perineural fibroblastomas, are encapsulated nerve sheath tumors. Primary schwannomas of the liver are extremely rare. We present a case of liver schwannoma, incidentally found in a patient with breast cancer. A 66-year-old female consulted her physician for a mass she palpated on her left breast. The abdominal ultrasonography (USG) revealed a 44 x 28 mm mass in the medial segment of the left lobe of her liver suspicious of a metastasis. An USG-guided biopsy was performed and the histo-pathological examination revealed a "peripheral nerve sheath tumor". Positron emission tomography (PET-CT) revealed a pathologic FDG uptake in the lesion that was previously defined in the liver. The tumor resected from the liver was 5 x 4 x 3 cm, yellowish, soft, and capsulated tumor. Microscopic examination revealed that the mass consisted of bundles of spindle cells with hypercellular and hypocellular areas. In immunohistochemistry, there was a strong positive staining for S-100. The tumor was diagnosed as benign liver schwannoma. Schwannomas are benign, encapsulated neoplasms. Symptoms and signs vary depending on the anatomical site and the size of the neoplasm; however, most schwannomas present as an asymptomatic or painless mass. Recurrence is unusual, despite of an incomplete removal, and malignant transformation is exceedingly rare (Fig. 4, Ref. 8). Full Text (Free, PDF) www.bmj.sk.

Schwannoma of the stomach: a case report

International Nuclear Information System (INIS)

Lee, Mi Suk; Lee, Jeong Min; Yun, Yeung Geum; Yang, Kee Hwa

1995-01-01

We report a case of exogastric schwannoma of the stomach in 41-years-old male patient with papable mass in left upper abdomen. Upper Gl series revealed extrinsic compression on the greater curvature of the stomach. CT scan showed a low density mass attached to greater curvature of the stomach with inhomogeneous contrast enhancement in the mass lesion. The mass was removed by surgery, and confirmed pathologically as schwannoma arising from the stomach

Laparoscopic resection of an obturator schwannoma: a case report

Institute of Scientific and Technical Information of China (English)

YI Ke; WANG Yu-mei; CHEN Jie

2010-01-01

@@ Schwannomas are usually benign tumors that arise from a nerve supporting the Schwann cells, those are mostly associated with the cranial or main peripheral nerves. The occurrence of retroperitoneal schwannoma is quite rare, and in such a case, surgical excision is the first option for treatment.

Extramedullary plasmacytoma of small bowel mesentery in associated with cecal cancer: a case report

International Nuclear Information System (INIS)

Kim, Sung Kyu; Kim, Yong Soo; Kim, Young Sun; Cho, On Koo; Koh, Byung Hee; Rhim, Hyun Chul; Park, Choog Ki; Park, Dong Woo; Park, Yong Wook; Oh, Young Ha

2005-01-01

Extramedullary plasmacytoma is a rare disease that is histopathologically defined as a solitary tumor composed of a monoclonal proliferation of cells with plasmacytic differentiation in an extramedullary site. Most of these tumors occur in the submucosa of the upper aerodigestive tract, and they rarely occur in the small bowel mesentery. We report here on a case of extramedullary plasmacytoma of the small bowel mesentery that was in association with a cecal cancer. Abdominal ultrasound and CT revealed a lobulated soft tissue mass with a cystic portion and peripheral calcification. In this case, the preoperative radiological diagnosis was difficult due to accompanying cecal cancer

Extramedullary plasmacytoma of small bowel mesentery in associated with cecal cancer: a case report

Energy Technology Data Exchange (ETDEWEB)

Kim, Sung Kyu; Kim, Yong Soo; Kim, Young Sun; Cho, On Koo; Koh, Byung Hee; Rhim, Hyun Chul; Park, Choog Ki; Park, Dong Woo [Hanyang University College of Medicine, Seoul (Korea, Republic of); Park, Yong Wook; Oh, Young Ha [Hanyang University Guri Hospital, Guri (Korea, Republic of)

2005-07-15

Extramedullary plasmacytoma is a rare disease that is histopathologically defined as a solitary tumor composed of a monoclonal proliferation of cells with plasmacytic differentiation in an extramedullary site. Most of these tumors occur in the submucosa of the upper aerodigestive tract, and they rarely occur in the small bowel mesentery. We report here on a case of extramedullary plasmacytoma of the small bowel mesentery that was in association with a cecal cancer. Abdominal ultrasound and CT revealed a lobulated soft tissue mass with a cystic portion and peripheral calcification. In this case, the preoperative radiological diagnosis was difficult due to accompanying cecal cancer.

CT and MR imaging of high cervical intradural lipomas

Energy Technology Data Exchange (ETDEWEB)

Oh, Joo Hyeong; Choi, Woo Suk; Lee, Sun Wha; Lim, Jae Hoon; Leem, Woon; Kim, Gook Ki; Rhee, Bong Arm [Kyung Hee University Hospital, Seoul (Korea, Republic of)

1988-04-15

Intradural spinal lipoma occurs in less than 1% of all spinal cord tumors. It has been described at every level of the spinal canal, although its most common location is the cervicothoracic and thoracic region. However, lipoma located in the high cervical region is very unusual. We described two cases, a teenager and an adult, with progressive neurologic deficit from such a lipomatous tumor, which were evaluated by CT scanning and MR imaging.

Benign intramural schwannoma of the esophagus ? case report

OpenAIRE

Kozak, Katarzyna; Kowalczyk, Mateusz; Jesionek-Kupnicka, Dorota; Kozak, J?zef

2015-01-01

Schwannomas of the esophagus are rare peripheral nerve tumors. A 37-year-old woman with dysphagia was found to have an intramural tumor of the upper esophagus. The lesion was revealed on computed tomography. Endoscopic ultrasound biopsy was nondiagnostic. Through right thoracotomy, the mass was enucleated from the wall of the esophagus. Benign schwannoma was diagnosed only after immunological staining examination.

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

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...

Schwannoma originating from lower cranial nerves: report of 4 cases.

Science.gov (United States)

Oyama, Hirofumi; Kito, Akira; Maki, Hideki; Hattori, Kenichi; Noda, Tomoyuki; Wada, Kentaro

2012-02-01

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 tumor was enucleated, facial palsy, hoarseness, dysphagia and paresis of the deltoid muscle occurred transiently after the operation. The patient's hearing had also slightly deteriorated. Case 3 is a dumbbell-typed schwannoma originating from the hypoglossal nerve. The hypoglossal canal was markedly enlarged by the tumor. As the hypoglossal nerves were embedded in the tumor, the tumor around the hypoglossal nerves was not resected. The tumor was significantly enlarged for a while after stereotactic irradiation. Case 4 is an intracranial cystic schwannoma originating from the IXth or Xth cranial nerves. The tumor was resected through the cerebello-medullary fissure. The tumor capsule attached to the brain stem was not removed. Hoarseness and dysphagia happened transiently after the operation. Cranial nerve palsy readily occurs after the removal of the schwannoma originating from the lower cranial nerves. Mechanical injury caused by retraction, extension and compression of the nerve and heat injury during the drilling of the petrous bone should be cautiously avoided.

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.

Atypical location of extramedullary hematopoietic masses in thalassemia

International Nuclear Information System (INIS)

Gemenis, T.; Philippou, A.; Gouliamos, A.; Kalovidouris, A.; Papavasiliou, C.; Papacharalambus, X.; Panani, A.; Chalevelakis, G.; Raptis, S.

1989-01-01

A case of β-thalassemia with multiple foci of extramedullary hematopoiesis (EH) is reported. EH masses were demonstrated in the presacral and the costovertebral space. EH foci were also encountered in the spleen. (orig.) [de

Angiogenesis in vestibular schwannomas

DEFF Research Database (Denmark)

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

2010-01-01

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...

A New Classification for Pathologies of Spinal Meninges-Part 2: Primary and Secondary Intradural Arachnoid Cysts.

Science.gov (United States)

Klekamp, Jörg

2017-08-01

Spinal intradural arachnoid cysts are rare causes of radiculopathy or myelopathy. Treatment options include resection, fenestration, or cyst drainage. To classify intradural spinal arachnoid cysts and present results of their treatment. Among 1519 patients with spinal space occupying lesions, 130 patients demonstrated intradural arachnoid cysts. Neuroradiological and surgical features were reviewed and clinical data analyzed. Twenty-one patients presented arachnoid cysts as a result of an inflammatory leptomeningeal reaction related to meningitis, subarachnoid hemorrhage, intrathecal injections, intradural surgery, or trauma, ie, secondary cysts. For the remaining 109 patients, no such history could be elucidated, ie, primary cysts. Forty-six percent of primary and 86% of secondary cysts were associated with syringomyelia. Patients presented after an average history of 53 ± 88 months. There were 122 thoracic and 7 lumbar cysts plus 1 cervical cyst. Fifty-nine patients with primary and 15 patients with secondary cysts underwent laminotomies with complete or partial cyst resection and duraplasty. Mean follow-up was 57 ± 52 months. In the first postoperative year, profound improvements for primary cysts were noted, in contrast to marginal changes for secondary cysts. Progression-free survival for 10 years following surgery was determined as 83% for primary compared to 15% for secondary cysts. Despite differences in clinical presentation, progression-free survival was almost identical for patients with or without syringomyelia. Complete or partial resection leads to favorable short- and long-term results for primary arachnoid cysts. For secondary cysts, surgery can only provide clinical stabilization for a limited time due to the often extensive arachnoiditis. Copyright © 2017 by the Congress of Neurological Surgeons

Ruptured cervical arteriovenous fistulas presenting with subarachnoid hemorrhage and quadriplegia: an uncommon case.

Science.gov (United States)

Liu, Chien-Liang; Su, Yung-Cheng; Chen, Chien-Chih; Chong, Chee-Fah; Wang, Tzong-Luen

2008-02-01

Nontraumatic subarachnoid hemorrhage is a neurologic emergency, and prompt treatment is necessary to avoid catastrophic result. We present a patient with subarachnoid hemorrhage caused by ruptured cervical intradural extramedullary arteriovenous fistulas, which rapidly progressed to quadriplegia. Because of the timely management, the patient had a good recovery. This is a rare but important case that emergency physicians should be aware of.

Magnetic Resonance Imaging Appearance of Schwannomas from Head to Toe: A Pictorial Review

Directory of Open Access Journals (Sweden)

Jamie Crist

2017-01-01

Full Text Available Schwannomas are benign soft-tissue tumors that arise from peripheral nerve sheaths throughout the body and are commonly encountered in patients with neurofibromatosis Type 2. The vast majority of schwannomas are benign, with rare cases of malignant transformation reported. In this pictorial review, we discuss the magnetic resonance imaging (MRI appearance of schwannomas by demonstrating a collection of tumors from different parts of the body that exhibit similar MRI characteristics. We review strategies to distinguish schwannomas from malignant soft-tissue tumors while exploring the anatomic and histologic origins of these tumors to discuss how this correlates with their imaging findings. Familiarity with the MRI appearance of schwannomas can help aid in the differential diagnosis of soft-tissue masses, especially in unexpected locations.

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.

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

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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

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

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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

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.

Gastric schwannomas: radiological features with endoscopic and pathological correlation

International Nuclear Information System (INIS)

Hong, H.S.; Ha, H.K.; Won, H.J.; Byun, J.H.; Shin, Y.M.; Kim, A.Y.; Kim, P.N.; Lee, M.-G.; Lee, G.H.; Kim, M.J.

2008-01-01

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

Extramedullary plasmacytoma of the larynx. A report of three cases

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Strojan, P.

2002-01-01

Purpose. To report three cases of extramedullary plasmacytoma of the larynx treated at the Institute of Oncology in Ljubljana between 1969-1999. Results. All three patients were treated with radiotherapy only, which resulted in permanent local and regional control of 7.8, 4.7 and 3.5 years. The function of the larynx was preserved in all of them. Two patients died, both to the causes other than plasmacytoma. In none of the patients disease progressed to multiple myeloma. Conclusions. Extramedullary plasmacytoma of the larynx is a rare disease, highly curable when radiotherapy is used. Moderate radiation doses and limited fields ensure excellent cosmetic and functional result. (author)

Spinal tumors

International Nuclear Information System (INIS)

Goethem, J.W.M. van; Hauwe, L. van den; Oezsarlak, Oe.; Schepper, A.M.A. de; Parizel, P.M.

2004-01-01

Spinal tumors are uncommon lesions but may cause significant morbidity in terms of limb dysfunction. In establishing the differential diagnosis for a spinal lesion, location is the most important feature, but the clinical presentation and the patient's age and gender are also important. Magnetic resonance (MR) imaging plays a central role in the imaging of spinal tumors, easily allowing tumors to be classified as extradural, intradural-extramedullary or intramedullary, which is very useful in tumor characterization. In the evaluation of lesions of the osseous spine both computed tomography (CT) and MR are important. We describe the most common spinal tumors in detail. In general, extradural lesions are the most common with metastasis being the most frequent. Intradural tumors are rare, and the majority is extramedullary, with meningiomas and nerve sheath tumors being the most frequent. Intramedullary tumors are uncommon spinal tumors. Astrocytomas and ependymomas comprise the majority of the intramedullary tumors. The most important tumors are documented with appropriate high quality CT or MR images and the characteristics of these tumors are also summarized in a comprehensive table. Finally we illustrate the use of the new World Health Organization (WHO) classification of neoplasms affecting the central nervous system

Extramedullary plasmacytoma. Fine needle aspiration findings.

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Kumar, P V; Owji, S M; Talei, A R; Malekhusseini, S A

1997-01-01

To determine the role of fine needle aspiration cytology in the diagnosis of extramedullary plasmacytoma. The study group consisted of 13 patients with palpable masses at various sites. The tumors were aspirated for cytologic study. The smears revealed groups of mature and immature plasma cells at various stages of maturation. Mature plasma cells showed an eccentric nucleus and abundant, deep, basophilic cytoplasm with a paranuclear halo. Plasmablasts (immature plasma cells) showed a prominent, eccentric nucleus with single, large nucleolus and abundant, deep, basophilic cytoplasm with no paranuclear halo. Binucleate and multinucleate forms were also seen quite often. The tumors were excised, and the histologic sections confirmed the cytologic diagnosis. All the patients received radiotherapy. One patient (18 years old) developed recurrence and died due to extensive infiltration into the maxilla and mandible. Two patients (57 and 62 years) developed multiple myeloma one to two years after the excision of tumors, and both died two to three months later. The remaining 10 patients were alive and well at this writing. The smears from all 13 patients were diagnosed as extramedullary plasmacytomas by fine needle aspiration cytology.

Schwannoma extending from the umbilical region to the mid-thigh ...

African Journals Online (AJOL)

... originating from the cells of peripheral nerve sheaths; the diagnosis was a schwannoma. Abdominal schwannomas are rare neoplasms that can be misdiagnosed. Laparoscopy is a safe and efficient option for approaching benign pelvic tumours and may offer the advantage of better visualisation of structures owing to the ...

Schwannoma in the porta hepatis - laparoscopic excision under laparoscopic ultrasound guidance.

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Sebastian, Maciej; Sroczyński, Maciej; Donizy, Piotr; Rudnicki, Jerzy

2017-09-01

Schwannomas are usually benign tumors attached to peripheral nerves and are rarely found in the gastrointestinal tract. Schwannomas in the porta hepatis are extremely rare, with only 15 cases described in the literature to date. A 22-year-old female patient presented with colicky upper abdominal pain lasting 3 months. Magnetic resonance imaging of the abdominal cavity revealed a tumor in the porta hepatis. The patient was qualified for laparoscopy. The tumor was totally excised laparoscopically under guidance of laparoscopic ultrasound without intra- or postoperative complications. Postoperative histopathological examination confirmed the porta hepatic schwannoma. The patient recovered uneventfully with very good cosmetic results. In the follow-up period of 5 months we have not observed any abdominal or general health problems. The present case is the first report in the world of laparoscopic ultrasound guided laparoscopic excision of a porta hepatic schwannoma.

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

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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.

Sciatica due to Schwannoma at the Sciatic Notch

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Haspolat, Yavuz; Ozkan, Feyza Unlu; Turkmen, Ismail; Kemah, Bahattin; Turhan, Yalcin; Sarar, Serhan; Ozkan, Korhan

2013-01-01

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. PMID:23762699

Sciatica due to Schwannoma at the Sciatic Notch

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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.

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)

Microsurgical and Endoscopic Anatomy for Intradural Temporal Bone Drilling and Applications of the Electromagnetic Navigation System: Various Extensions of the Retrosigmoid Approach.

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Matsushima, Ken; Komune, Noritaka; Matsuo, Satoshi; Kohno, Michihiro

2017-07-01

The use of the retrosigmoid approach has recently been expanded by several modifications, including the suprameatal, transmeatal, suprajugular, and inframeatal extensions. Intradural temporal bone drilling without damaging vital structures inside or beside the bone, such as the internal carotid artery and jugular bulb, is a key step for these extensions. This study aimed to examine the microsurgical and endoscopic anatomy of the extensions of the retrosigmoid approach and to evaluate the clinical feasibility of an electromagnetic navigation system during intradural temporal bone drilling. Five temporal bones and 8 cadaveric cerebellopontine angles were examined to clarify the anatomy of retrosigmoid intradural temporal bone drilling. Twenty additional cerebellopontine angles were dissected in a clinical setting with an electromagnetic navigation system while measuring the target registration errors at 8 surgical landmarks on and inside the temporal bone. Retrosigmoid intradural temporal bone drilling expanded the surgical exposure to allow access to the petroclival and parasellar regions (suprameatal), internal acoustic meatus (transmeatal), upper jugular foramen (suprajugular), and petrous apex (inframeatal). The electromagnetic navigation continuously guided the drilling without line of sight limitation, and its small devices were easily manipulated in the deep and narrow surgical field in the posterior fossa. Mean target registration error was less than 0.50 mm during these procedures. The combination of endoscopic and microsurgical techniques aids in achieving optimal exposure for retrosigmoid intradural temporal bone drilling. The electromagnetic navigation system had clear advantages with acceptable accuracy including the usability of small devices without line of sight limitation. Copyright © 2017 Elsevier Inc. All rights reserved.

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

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Arthurs, Benjamin J; Lamoreaux, Wayne T; Giddings, Neil A; Fairbanks, Robert K; Mackay, Alexander R; Demakas, John J; Cooke, Barton S; Lee, Christopher M

2009-12-18

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.

Gamma-knife radiosurgery in the treatment of trigeminal schwannomas

International Nuclear Information System (INIS)

Peker, S.; Bayrakli, F.; Kilic, T.; Pamir, M.N.

2007-01-01

Trigeminal nerve schwannomas account for 0.07 %-0.28 % of all intracranial tumors. Advances in skull base surgery have led to more aggressive resection of these tumors, but surgery may associated with development of new neurological deficits. In this report, we analyse the long-term results 15 patients with newly diagnosed or residual/recurrent trigeminal schwannoma who underwent gamma-knife treatment. During a mean 61 months of follow-up, MRI revealed reduction of tumor size in 13 and no size change in 2 patients. The tumor growth control rate was 100 % and only 1 patient had transient facial numbness and diplopia. For patients with small to moderate size trigeminal schwannomas, gamma-knife radiosurgery is associated with good tumor control and a minimal risk of adverse radiation effects. (author)

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

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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.

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.

An evidence-based case of acoustic/vestibular schwannoma

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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.

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

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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.

The bony crescent sign - a new sign of facial nerve schwannoma

International Nuclear Information System (INIS)

Watts, A.; Fagan, P.

1992-01-01

Schwannomas are relatively uncommon intracranial tumours. They most commonly involve the acoustic nerve followed in frequency by the trigeminal nerve. Other cranial nerves are rarely involved. Facial nerve schwannomas occurring within the petrous temporal bone are very rare. Their diagnosis may be missed prospectively even when appropriate computerized tomography (CT) scans are performed. Even in retrospect the site of abnormality may be difficult to identify, especially if there is an associated middle ear mass such as a cholesteatoma. In the 4 cases presented the facial nerve schwannoma was seen on high resolution CT as a soft tissue mass bounded anteriorly by a thin rim of bone. This bony crescent sign is a previously undescribed feature of facial nerve schwannoma which appears to be strongly indicative of the presence of this tumour. Recognition of this sign makes these tumours arising in the region of the geniculate ganglion easy to diagnose prospectively. 12 refs., 6 figs

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

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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.

Long-term mobile phone use and the risk of vestibular schwannoma: a Danish nationwide cohort study.

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Schüz, Joachim; Steding-Jessen, Marianne; Hansen, Søren; Stangerup, Sven-Eric; Cayé-Thomasen, Per; Poulsen, Aslak Harbo; Olsen, Jørgen H; Johansen, Christoffer

2011-08-15

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 schwannoma risk in men (relative risk estimate = 0.87, 95% confidence interval: 0.52, 1.46), and no vestibular schwannoma cases among long-term subscribers occurred in women versus 1.6 expected. Vestibular schwannomas did not occur more often on the right side of the head, although the majority of Danes 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 and possible diagnostic delay, further surveillance is indicated.

Clinical experience with Leksell gamma knife in the treatment of trigeminal schwannomas

Institute of Scientific and Technical Information of China (English)

WANG En-min; PAN Li; ZHANG Nan; ZHOU Liang-fu; WANG Bing-jiang; DONG Ya-fei; DAI Jia-zhong; CAI Pei-wu

2005-01-01

@@ Trigeminal nerve schwannomas, which are rare, slowly growing, benign tumors, account for 0.2% to 1.0% of all intracranial tumors and 0.8% to 8.0% of intracranial schwannomas.1-5 These tumors are treated surgically.1-4 The development of microsurgery and skull base surgery has made complete resection possible in most patients. Nevertheless, cranial nerve sequelae appear after complete resection of these tumors because they are located close to the cavernous sinus and usually adhere to the vital vascular and neural structures. As an alternative to microsurgical resection, Leksell gamma knife (LGK) radiosurgery has been performed for patients with intracranial schwannomas to minimize the treatment-related morbidity and achieve a long-term control of tumor growth.6,7 In this report, we describe our 6-year experience in the treatment of 38 patients with trigeminal schwannomas by LGK.

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

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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.

Retropharyngeal Schwannoma Excised Through a Transoral Approach: A Case Report

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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.

Pelvic schwannoma in the right parametrium

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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

MRI of perineural extramedullary granulocytic sarcoma

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Graham, A. [Rehabilitation Medicine, Hunters Moor Neurological Rehabilitation Centre, Newcastle-Upon-Tyne (United Kingdom); Hodgson, T. [Neuroradiology Dept., Royal Hallamshire Hospital, Sheffield (United Kingdom); Jacubowski, J. [Neurosurgical Dept., Royal Hallamshire Hospital, Sheffield (United Kingdom); Norfolk, D. [Haematology Department, Leeds General Infirmary, Leeds LS1 3EX (United Kingdom); Smith, C. [Pathology Dept., Royal Hallamshire Hospital, Sheffield (United Kingdom)

2001-06-01

Granulocytic sarcoma is an extramedullary solid tumour consisting of myelogenous leukaemic blast cells, usually seen in acute myeloid leukaemia and less commonly in patients with chronic myeloid leukaemia or myeloproliferative disorders. Blast cells have a predilection for periosteal and perineural regions and rarely precede evidence of systemic disease. We present two patients, aleukaemic on peripheral blood counts, both at presentation and during subsequent treatment. We present the MRI features of this rare but important condition. (orig.)

Neurophysiological intraoperative monitoring during an optic nerve schwannoma removal.

Science.gov (United States)

San-Juan, Daniel; Escanio Cortés, Manuel; Tena-Suck, Martha; Orozco Garduño, Adolfo Josué; López Pizano, Jesús Alejandro; Villanueva Domínguez, Jonathan; Fernández Gónzalez-Aragón, Maricarmen; Gómez-Amador, Juan Luis

2017-10-01

This paper reports the case of a patient with optic nerve schwannoma and the first use of neurophysiological intraoperative monitoring of visual evoked potentials during the removal of such tumor with no postoperative visual damage. Schwannomas are benign neoplasms of the peripheral nervous system arising from the neural crest-derived Schwann cells, these tumors are rarely located in the optic nerve and the treatment consists on surgical removal leading to high risk of damage to the visual pathway. Case report of a thirty-year-old woman with an optic nerve schwannoma. The patient underwent surgery for tumor removal on the left optic nerve through a left orbitozygomatic approach with intraoperative monitoring of left II and III cranial nerves. We used Nicolet Endeavour CR IOM (Carefusion, Middleton WI, USA) to performed visual evoked potentials stimulating binocularly with LED flash goggles with the patient´s eyes closed and direct epidural optic nerve stimulation delivering rostral to the tumor a rectangular current pulse. At follow up examinations 7 months later, the left eye visual acuity was 20/60; Ishihara score was 8/8 in both eyes; the right eye photomotor reflex was normal and left eye was mydriatic and arreflectic; optokinetic reflex and ocular conjugate movements were normal. In this case, the epidural direct electrical stimulation of optic nerve provided stable waveforms during optic nerve schwannoma resection without visual loss.

Imaging characteristics of intraparenchymal schwannoma and the related pathology

International Nuclear Information System (INIS)

Liu Shuyong; Geng Daoying; He Huijin

2007-01-01

Objective: To Analyze the imaging characteristics of intraparenchymal schwannoma and the related pathology, in order to improve the accuracy of diagnosis and be in favor of the clinics and the prognosis. Methods: Four cases were confirmed to be intraparenchymal schwannoma by pathological and immunohistochemistry examination. One case was examined with precontrast and enhanced CT scanning, one with unenhanced MRI scanning, two with unenhanced and enhanced CT and MRI scanning. Their images were retrospectively analyzed. Results: Of the four cases, three patients were less than 30 years old, with tumors located supratentorially. Cysts were found in all cases, with nodules on the wall in 3 cases. The nodules were enhanced markedly in two cases and moderately in one case. In addition, calcification was detected in one case and prominent peritumoral edema existed in 1 case. The picture of the pathology demonstrated Antoni type A and Antoni type B. Immunostaining showed intense immunoreactivity for S-100 protein and Vim and negative immunoreactivity for GFAP and EMA. Conclusions: Intraparenchymal schwannoma mostly occurred in juvenile, which located supratentorially in most cases. The presence of a cyst and peritumoral edema together with the tumor appears to be characteristic of intraparenchymal schwannoma. Calcification or the enhanced nodule is the helpful sign for the diagnosis. Combining the imaging findings with the pathology and immunohistochemistry results can gain the accurate diagnosis. (authors)

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.

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...

Microdeletion del(22(q12.2 encompassing the facial development-associated gene, MN1 (meningioma 1 in a child with Pierre-Robin sequence (including cleft palate and neurofibromatosis 2 (NF2: a case report and review of the literature

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Davidson Tom B

2012-03-01

Full Text Available Abstract Background Pierre-Robin sequence (PRS is defined by micro- and/or retrognathia, glossoptosis and cleft soft palate, either caused by deformational defect or part of a malformation syndrome. Neurofibromatosis type 2 (NF2 is an autosomal dominant syndrome caused by mutations in the NF2 gene on chromosome 22q12.2. NF2 is characterized by bilateral vestibular schwannomas, spinal cord schwannomas, meningiomas and ependymomas, and juvenile cataracts. To date, NF2 and PRS have not been described together in the same patient. Case presentation We report a female with PRS (micrognathia, cleft palate, microcephaly, ocular hypertelorism, mental retardation and bilateral hearing loss, who at age 15 was also diagnosed with severe NF2 (bilateral cerebellopontine schwannomas and multiple extramedullary/intradural spine tumors. This is the first published report of an individual with both diagnosed PRS and NF2. High resolution karyotype revealed 46, XX, del(22(q12.1q12.3, FISH confirmed a deletion encompassing NF2, and chromosomal microarray identified a 3,693 kb deletion encompassing multiple genes including NF2 and MN1 (meningioma 1. Five additional patients with craniofacial dysmorphism and deletion in chromosome 22-adjacent-to or containing NF2 were identified in PubMed and the DECIPHER clinical chromosomal database. Their shared chromosomal deletion encompassed MN1, PITPNB and TTC28. MN1, initially cloned from a patient with meningioma, is an oncogene in murine hematopoiesis and participates as a fusion gene (TEL/MN1 in human myeloid leukemias. Interestingly, Mn1-haploinsufficient mice have abnormal skull development and secondary cleft palate. Additionally, Mn1 regulates maturation and function of calvarial osteoblasts and is an upstream regulator of Tbx22, a gene associated with murine and human cleft palate. This suggests that deletion of MN1 in the six patients we describe may be causally linked to their cleft palates and/or craniofacial

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

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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.

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

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.

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

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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.

Current practices in vestibular schwannoma management: a survey of American and Canadian neurosurgeons.

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Fusco, Matthew R; Fisher, Winfield S; McGrew, Benjamin M; Walters, Beverly C

2014-12-01

Comprehensive therapy for vestibular schwannomas has changed dramatically over the past fifty years. Previously, neurosurgeons were most likely to treat these tumors via an independent surgical approach. Currently, many neurosurgeons treat vestibular schwannomas employing an interdisciplinary team approach with neuro-otologists and radiation oncologists. This survey aims to determine the current treatment paradigm for vestibular schwannomas among American and Canadian neurosurgeons, with particular attention to the utilization of a team approach to the surgical resection of these lesions. A seventeen part survey questionnaire was sent by electronic mail to residency trained members of the American Association of Neurological Surgeons currently practicing in Canada or the United States. Questions were divided into groups regarding physician background, overall practice history, recent practice history, opinions on treatment paradigms, and experience with an interdisciplinary team approach. Seven hundred and six responses were received. The vast majority of neurosurgeons surgically resect vestibular schwannomas as part of an interdisciplinary team (85.7%). Regional variations were observed in the use of an interdisciplinary team: 52.3% of responding neurosurgeons who surgically treat vestibular schwannomas without neuro-otologists currently practice in the South (no other region represented more than 15.4% of this group, p=0.02). Surgeons who have treated >50 vestibular schwannomas show a trend towards more frequent utilization of an interdisciplinary approach than less experienced surgeons, but this did not reach statistical significance. The majority of neurosurgeons in the United States and Canada surgically resect vestibular schwannomas via an interdisciplinary approach with the participation of a neuro-otologist. Neurosurgeons in the South appear more likely to surgically treat these tumors alone than neurosurgeons in other regions of the U.S. and Canada

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.

Schwannoma of the facial nerve involving the middle cranial fossa:case report

Institute of Scientific and Technical Information of China (English)

SAI Ke; CHEN Zhong-ping

2007-01-01

@@ Facial nerve schwannoma involving the middle cranial fossa is quite rare,and its accurate diagnosis is very difficult before SUrgery.Here we present a case of schwannoma of the facial nerve at the middle cranial fossa that was misdiagnosed previously at a local hospital and then cured in our hospital.

Natural history of vestibular schwannomas and hearing loss in NF2 patients.

Science.gov (United States)

Peyre, M; Bernardeschi, D; Sterkers, O; Kalamarides, M

2015-07-13

Bilateral vestibular schwannomas are the hallmark of neurofibromatosis 2 (NF2), occurring in 95% of patients. These tumors are associated with significant morbidity due to hearing loss, tinnitus, imbalance and facial weakness. As radiosurgery and chemotherapy have been recently introduced in the treatment armamentarium in addition to surgery, a thorough evaluation of vestibular schwannoma natural history is mandatory to determine the role and timing of each treatment modality. An exhaustive review of the literature was performed using the PubMed database concerning the natural history of tumor growth and hearing loss in NF2 patients with vestibular schwannomas. Although some aspects of vestibular schwannoma natural history remain uncertain (pattern of tumor growth, mean tumor growth rate), factors influencing growth such as age at presentation and paracrine factors are well established. Studies focusing on the natural history of hearing have highlighted different patterns of hearing loss and the possible role of intralabyrinthine tumors. The polyclonality of vestibular schwannomas in NF2 was recently unveiled, giving a new perspective to their growth mechanisms. An uniform evaluation of tumor growth using volumetric evaluation and hearing with standard classifications will ensure the use of common endpoints and should improve the quality of clinical trials as well as foster comparison among studies while ensuring more consistency in decision-making. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

Small vestibular schwannomas presenting with facial nerve palsy.

Science.gov (United States)

Espahbodi, Mana; Carlson, Matthew L; Fang, Te-Yung; Thompson, Reid C; Haynes, David S

2014-06-01

To describe the surgical management and convalescence of two patients presenting with severe facial nerve weakness associated with small intracanalicular vestibular schwannomas (VS). Retrospective review. Two adult female patients presenting with audiovestibular symptoms and subacute facial nerve paralysis (House-Brackmann Grade IV and V). In both cases, post-contrast T1-weighted magnetic resonance imaging revealed an enhancing lesion within the internal auditory canal without lateral extension beyond the fundus. Translabyrinthine exploration demonstrated vestibular nerve origin of tumor, extrinsic to the facial nerve, and frozen section pathology confirmed schwannoma. Gross total tumor resection with VIIth cranial nerve preservation and decompression of the labyrinthine segment of the facial nerve was performed. Both patients recovered full motor function between 6 and 8 months after surgery. Although rare, small VS may cause severe facial neuropathy, mimicking the presentation of facial nerve schwannomas and other less common pathologies. In the absence of labyrinthine extension on MRI, surgical exploration is the only reliable means of establishing a diagnosis. In the case of confirmed VS, early gross total resection with facial nerve preservation and labyrinthine segment decompression may afford full motor recovery-an outcome that cannot be achieved with facial nerve grafting.

The imaging manifestation of intracranial schwannomas not arising from cranial nerves

International Nuclear Information System (INIS)

Luo Boning; Sun Gengxi; Liang Kangfu

2003-01-01

Objective: To report 4 cases of operation-confirmed intracranial schwannomas not arising from cranial nerves, and to discuss the histogenesis and CT and MRI manifestation combining with the literature. Methods: CT and MRI findings of these 4 intracranial schwannomas were analyzed retrospectively and their pathologic characteristics were reviewed. Results: The tumors demonstrated low-iso mixed density with necrotic and cystic areas. Large degeneration even became the prominent characteristics on CT scan. Low-signal to iso-signal intensity on T 1 -weighted images and heterogeneous high-signal intensity on T 2 -weighted images were revealed. The solid portion and the wall of the tumors were moderately enhanced on enhanced CT and MRI scans. Hematoxylin and eosin-stained sections showed two corresponding histological patterns: Antoni Type A and Antoni Type B pattern. The tumor cells exhibited a strong positive staining pattern for s-100 protein. Conclusion: The imaging manifestations of these tumors were similar to that of cranial nerve schwannomas except that the former had higher cystic degeneration rate. We should take that diagnosis into consideration when the mass is not located on cranial nerve but its image feature resembles schwannomas

Schwannoma of the upper lip: A case report and literature review

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Peediackel Antony George

2017-01-01

Full Text Available Introduction. Schwannomas or neurilemmomas are well demarcated, benign neurogenic lesions arising by a fibroblastic proliferation of the nerve sheath cell (Schwann cell. They usually present as solitary encapsulated lesions with rare occurrence in the upper lip. Non-diagnosed or misdiagnosed schwannomas present a high risk for the tumor to continue growing and exerting pressure on surrounding nerves. These tumours based on their location could lead to facial weakness and paralysis, pressure in ears, tinnitus, hearing loss, balance loss, and could lead to a life-threatening situation. Case Outline. This case is a rare presentation of a schwannoma located in the upper lip of a 21-year-old male patient of Indian origin. The patient complained of a swelling in the mouth with a difficulty in keeping the mouth closed. The swelling was surgically excised and the patient healed completely. Conclusion. This case of occurrence of tumor on the upper lip points to the possibility of considering schwannoma as a possibility in the diagnosis of oral tumors in the future, as the location of the tumor was rare and had a high chance of misdiagnosis.

Extracranial Facial Nerve Schwannoma Treated by Hypo-fractionated CyberKnife Radiosurgery.

Science.gov (United States)

Sasaki, Ayaka; Miyazaki, Shinichiro; Hori, Tomokatsu

2016-09-21

Facial nerve schwannoma is a rare intracranial tumor. Treatment for this benign tumor has been controversial. Here, we report a case of extracranial facial nerve schwannoma treated successfully by hypo-fractionated CyberKnife (Accuray, Sunnyvale, CA) radiosurgery and discuss the efficacy of this treatment. A 34-year-old female noticed a swelling in her right mastoid process. The lesion enlarged over a seven-month period, and she experienced facial spasm on the right side. She was diagnosed with a facial schwannoma via a magnetic resonance imaging (MRI) scan of the head and neck and was told to wait until the facial nerve palsy subsides. She was referred to our hospital for radiation therapy. We planned a fractionated CyberKnife radiosurgery for three consecutive days. After CyberKnife radiosurgery, the mass in the right parotid gradually decreased in size, and the facial nerve palsy disappeared. At her eight-month follow-up, her facial spasm had completely disappeared. There has been no recurrence and the facial nerve function has been normal. We successfully demonstrated the efficacy of CyberKnife radiosurgery as an alternative treatment that also preserves neurofunction for facial nerve schwannomas.

Biomechanical Comparison of an Intramedullary and Extramedullary Free-Tissue Graft Reconstruction of the Acromioclavicular Joint Complex

Science.gov (United States)

Garg, Rishi; Javidan, Pooya; Lee, Thay Q.

2013-01-01

Background Several different surgical techniques have been described to address the coracoclavicular (CC) ligaments in acromioclavicular (AC) joint injuries. However, very few techniques focus on reconstructing the AC ligaments, despite its importance in providing stability. The purpose of our study was to compare the biomechanical properties of two free-tissue graft techniques that reconstruct both the AC and CC ligaments in cadaveric shoulders, one with an extramedullary AC reconstruction and the other with an intramedullary AC reconstruction. We hypothesized intramedullary AC reconstruction will provide greater anteroposterior translational stability and improved load to failure characteristics than an extramedullary technique. Methods Six matched cadaveric shoulders underwent translational testing at 10 N and 15 N in the anteroposterior and superoinferior directions, under AC joint compression loads of 10 N, 20 N, and 30 N. After the AC and CC ligaments were transected, one of the specimens was randomly assigned the intramedullary free-tissue graft reconstruction while its matched pair received the extramedullary graft reconstruction. Both reconstructed specimens then underwent repeat translational testing, followed by load to failure testing, via superior clavicle distraction, at a rate of 50 mm/min. Results Intramedullary reconstruction provided significantly greater translational stability in the anteroposterior direction than the extramedullary technique for four of six loading conditions (p < 0.05). There were no significant differences in translational stability in the superoinferior direction for any loading condition. The intramedullary reconstructed specimens demonstrated improved load to failure characteristics with the intramedullary reconstruction having a lower deformation at yield and a higher ultimate load than the extramedullary reconstruction (p < 0.05). Conclusions Intramedullary reconstruction of the AC joint provides greater stability in the

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

Directory of Open Access Journals (Sweden)

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.

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.

Resection of cervical vagal schwannoma via a post-auricular approach.

Science.gov (United States)

Roh, Jong-Lyel

2006-03-01

Cervical vagal schwannomas are extremely rare and gross total resection is the standard treatment modality. However, because the conventional cervical approach leaves an incision scar in a visible area, other approaches need to be developed for young women who want the postoperative scar to be invisible. A 28-year-old female underwent complete resection of a 4x4 cm tumor in her right upper neck via a post-auricular approach using an inverted V-shaped incision along the post-auricular sulcus and hairline. The tumor was a schwannoma originating from the right cervical vagus nerve. Postoperatively, right vocal cord paralysis developed despite careful dissection but completely recovered within 6 months after surgery. The patient was satisfied with an invisible external scar which was hidden by her auricle and hair. A cervical vagal schwannoma can be successfully removed by making an incision in a potentially invisible area.

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...... the OS. No patients relapsed at the primary site of the myeloid sarcoma despite management without radiotherapy....

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

African Journals Online (AJOL)

Solitary extramedullary plasmacytoma (SEP) is a neoplastic proliferation of a single clone of plasma cells that occur outside of the bone and bone marrow. It is rare, commonly occurring in the head and neck region, followed by the gastrointestinal tract. The aetiology, risk factors, natural history and consequent treatment are ...

Multiple schwannomas of cauda equine in the absence of von Recklinghausen's disease

International Nuclear Information System (INIS)

Kayaoglu, Cetin R.; Sengul, G.; Aydin, Ismail H.

2007-01-01

Multiple schwannomas in the absence of neurofibromatosis is rarely reported in the literature. We present a 56-year-old female with a history of severe leg and back pain on the left side for one year. Magnetic resonance imaging revealed 4 schwannomas located in the cauda equine in the absence of von Recklinghausen's disease. (author)

Frontoethmoidal Schwannoma with Exertional Cerebrospinal Fluid Rhinorrhea: Case Report and Review of Literature.

Science.gov (United States)

Yoneoka, Yuichiro; Akiyama, Katsuhiko; Seki, Yasuhiro; Hasegawa, Go; Kakita, Akiyoshi

2018-03-01

Frontoethmoidal schwannomas are rare. No case manifesting exertional cerebrospinal fluid (CSF) rhinorrhea has ever been reported to the best of our knowledge. In this report, we describe an extremely rare case of frontoethmoidal schwannoma extending through the olfactory groove with exertional CSF rhinorrhea as the initial symptom. A 50-year-old woman was presented to our clinic for frequent nasal discharge on exertion. A postcontrast computed tomographic scan demonstrated heterogeneously enhanced tumor from the anterior cranial fossa to the anterior ethmoid sinus. A gadolinium-enhanced T1-weighted magnetic resonance image revealed a well-defined heterogeneously enhanced tumor situated in the midline anterior cranial fossa and anterior ethmoid sinus. After the resection, the defect of the right anterior skull base was reconstructed with a fascia graft and adipose tissue taken from the abdomen, as well as a pedicle periosteum flap. A histologic examination revealed the tumor as schwannoma. Her rhinorrhea completely resolved. She regained her sense of smell and taste 1 month after the operation. According to previous reports, olfactory groove, and paraolfactory groove/periolfactory groove schwannomas can be divided into 4 types: subfrontal, nasoethmoidal, frontoethmoidal, and ethmofrontal. Among them, a frontoethmoidal schwannoma can manifest exertional CSF rhinorrhea as an initial symptom. Copyright © 2018 Elsevier Inc. All rights reserved.

Cervical Vestibular Evoked Myogenic Potential in Hypoglossal Nerve Schwannoma: A Case Report.

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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

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

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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.

Intracranial Extramedullary Hematopoiesis in Beta-Thalassemia

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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.

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.

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.

Primary intradural mesenchymal chondrosarcoma of the spine in a child

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Li, Yu-Hua [Shanghai Jiao Tong University, Department of Radiology, Xin Hua Hospital, School of Medicine, Shanghai (China); Yao, Xiao-Hong [Shanghai Jiao Tong University, Department of Pathology, Xin Hua Hospital, School of Medicine, Shanghai (China)

2007-11-15

We report a primary intradural mesenchymal chondrosarcoma of the spine in a 3-year-old girl. MRI revealed a markedly enhancing oval mass associated with focal areas of low signal intensity extending from T11 to L1. The lesion was located posterolateral to the right side of the spinal cord, pushing the conus medullaris and cauda equina anteriorly and to the left. The adjacent spinal cord also showed serpiginous areas of flow void. The mass was completely removed. Microscopic examination and immunohistochemical studies confirmed the diagnosis of mesenchymal chondrosarcoma. The patient was free of symptoms after surgery. (orig.)

Primary intradural mesenchymal chondrosarcoma of the spine in a child

International Nuclear Information System (INIS)

Li, Yu-Hua; Yao, Xiao-Hong

2007-01-01

We report a primary intradural mesenchymal chondrosarcoma of the spine in a 3-year-old girl. MRI revealed a markedly enhancing oval mass associated with focal areas of low signal intensity extending from T11 to L1. The lesion was located posterolateral to the right side of the spinal cord, pushing the conus medullaris and cauda equina anteriorly and to the left. The adjacent spinal cord also showed serpiginous areas of flow void. The mass was completely removed. Microscopic examination and immunohistochemical studies confirmed the diagnosis of mesenchymal chondrosarcoma. The patient was free of symptoms after surgery. (orig.)

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...

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...

Management of vestibular schwannomas with linear accelerator-based stereotactic radiosurgery: a single center experience.

Science.gov (United States)

Sager, Omer; Beyzadeoglu, Murat; Dincoglan, Ferrat; Demiral, Selcuk; Uysal, Bora; Gamsiz, Hakan; Oysul, Kaan; Dirican, Bahar; Sirin, Sait

2013-01-01

The primary goal of treatment for vestibular schwannoma is to achieve local control without comprimising regional cranial nerve function. Stereotactic radiosurgery has emerged as a viable therapeutic option for vestibular schwannoma. The aim of the study is to report our 15-year single center experience using linear accelerator-based stereotactic radiosurgery in the management of patients with vestibular schwannoma. Between July 1998 and January 2013, 68 patients with unilateral vestibular schwannoma were treated using stereotactic radiosurgery at the Department of Radiation Oncology, Gulhane Military Medical Academy. All patients underwent high-precision stereotactic radiosurgery using a linear accelerator with 6-MV photons. Median follow-up time was 51 months (range, 9-107). Median age was 45 years (range, 20-77). Median dose was 12 Gy (range, 10-13) prescribed to the 85%-95% isodose line encompassing the target volume. Local tumor control in patients with periodic follow-up imaging was 96.1%. Overall hearing preservation rate was 76.5%. Linear accelerator-based stereotactic radiosurgery offers a safe and effective treatment for patients with vestibular schwannoma by providing high local control rates along with improved quality of life through well-preserved hearing function.

Clinical features and surgical treatment of cauda equina schwannoma

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Jun-jun HUANG

2011-03-01

Full Text Available Objective To investigate the clinical features and surgical treatment method of cauda equina schwannoma.Methods Clinical symptoms,imaging characteristics and functional outcomes after surgical treatment of 32 patients with cauda equina schwannoma from June 2007 to March 2009 were retrospectively reviewed.Results Most patients(30 cases suffered lower extremity numbness/pain before operations and 18 of them suffered in both lower extremities.Physical examination showed bilateral nerve damage or unilateral multiple nerve damage in 25 cases.The initial symptom of 12 cases was simple lumbago.Ten cases were misdiagnosed as lumbar disc herniation.Six patients were scanned by computed tomography but none of them was diagnosed correctly.All the patients were definitely diagnosed by magnetic resonance imaging(MRI,the typical appearance of the schwannoma was a round or ovoid mass with isointense or slightly hyperintense on T1-weighted images,while on T2-weighted images with heterogeneous hyperintense in 25 cases,slightly hyperintense in 2,and homogeneous hyperintense in 5.Gadolinium-enhanced MRI was performed in 13 patients,of whom 10 cases showed heterogeneous or annular enhancing.In all patients,the tumor was resected completely,the functional outcomes were satisfactory except one with a giant tumor,in whom the neurological symptom was aggravated,and no recurrence was found during a short-term follow-up.Conclusions The characteristic features of cauda equina schwannoma were lower extremities numbness/pain and bi-or unilateral polyradiculopathy in physical examination.MRI is helpful for early diagnosis and recommended as the first choice.Functional outcomes are satisfactory in patients with tumor completely resected.

Laryngeal Schwannoma: A Case Presentation and Review of the Mayo Clinic Experience.

Science.gov (United States)

Romak, Jonathan J; Neel, H Bryan; Ekbom, Dale C

2017-01-01

The aim of this study was to clarify the nature of laryngeal schwannomas through review of the experience of a single institution during a 104-year period. This is a retrospective case series. The Mayo Clinic, Rochester, Minnesota clinical and surgical pathology database was reviewed for the years 1985-2011. Four cases of laryngeal schwannoma were identified. These cases were pooled with a previously published series of laryngeal schwannomas treated at our institution between 1907 and 1986. The characteristics of all 11 cases were studied, and relevant literature was reviewed. A total of 11 cases of schwannoma of the larynx were identified. The mean age at presentation was 48 years (range 12-73 years). The most common presenting symptoms were dysphonia and dysphagia. The most frequently involved primary site was the false vocal fold (six patients), followed by the aryepiglottic fold (three), epiglottis (two), subglottis (two), ventricle (one), true vocal fold (one) and postcricoid region (one). The mean maximal tumor diameter was 2.5 cm. In all but one case, surgical excision was curative with no recurrence during recorded follow up ranging from 1 to 17 years. Laryngeal schwannomas, although rare, should be considered in the differential diagnosis of laryngeal tumors. They occur most frequently in the false vocal fold and present most commonly with dysphonia and/or dysphagia. Surgical excision is the treatment of choice. Copyright © 2017 The Voice Foundation. Published by Elsevier Inc. All rights reserved.

Retroperitoneal Gastrointestinal Type Schwannoma Presenting as a Renal Mass

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Susan J. Hall

2015-11-01

Full Text Available Retroperitoneal schwannomas are extremely rare, and unreported in Urology. Often thought to be malignant from imaging the diagnosis is often delayed until Histology. We report a case of retroperitoneal schwanoma thought to be a malignant renal mass. Seventy three year old lady presented with abdominal pain. Imaging showed a mass attached to the renal pelvis thus she underwent a radical nephrectomy. Histology reported retroperitoneal schwannoma. Malignant forms are rare however treatment for these is surgical excision. Awareness of the existence of these tumors may help in avoiding unnecessary radical surgeries by opting for biopsy preoperatively.

The Middle Fossa Approach for the Removal of a Trochlear Schwannoma

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Boucher, Andrew B.; Michael, L. Madison

2014-01-01

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. PMID:24716021

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.

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

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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.

MR imaging of cranial nerve schwannomas

International Nuclear Information System (INIS)

Shapiro, M.; Peyster, R.; Cross, R.R.; Charles, J.; Murtagh, R.; Shapiro, R.; Chyatte, D.

1988-01-01

One of the major advantages of magnetic resonance (MR) imaging over other imaging modalities is direct visualization of the cranial nerves. This is best accomplished with thin-section, contiguous T1-weighted images. They report a series of 75 cranial nerve neuromas, including 47 of the eighth nerve and a mixture of schwannomas involving all other cranial nerves (excluding the fourth). All tumors demonstrated at least some area of increased signal (equal to or greater than that of cerebrospinal fluid) on T2-weighted images. This fact enabled them to differentiate schwannomas from neoplasms (lymphoma, meningioma, sarcoma) that may be isointense on T2-weighted pulse sequences. Many of the lesions had areas of low signal intermixed with predominantly high signal (on T2-weighted images). The pathologic evaluation of these areas of decreased signal revealed predominant fibrosis. In addition, some of the neuromas had a cystic component. Gadolinium-enhanced MR imaging may permit detection when the nerve is still normal in size

Benign giant mediastinal schwannoma presenting as cardiac tamponade in a woman: a case report

Directory of Open Access Journals (Sweden)

Sekiya Mitsuaki

2011-02-01

Full Text Available Abstract Introduction Mediastinal schwannomas are typically benign and asymptomatic, and generally present no immediate risks. We encountered a rare case of a giant benign posterior mediastinal schwannoma, complicated by life-threatening cardiac tamponade. Case presentation We report the case of a 72-year-old Japanese woman, who presented with cardiogenic shock. Computed tomography of the chest revealed a posterior mediastinal mass 150 cm in diameter, with pericardial effusion. The cardiac tamponade was treated with prompt pericardial fluid drainage. A biopsy was taken from the mass, and after histological examination, it was diagnosed as a benign schwannoma, a well-encapsulated non-infiltrating tumor, originating from the intrathoracic vagus nerve. It was successfully excised, restoring normal cardiac function. Conclusion Our case suggests that giant mediastinal schwannomas, although generally benign and asymptomatic, should be excised upon discovery to prevent the development of life-threatening cardiopulmonary complications.

Radiotherapy for Vestibular Schwannomas: A Critical Review

International Nuclear Information System (INIS)

Murphy, Erin S.; Suh, John H.

2011-01-01

Vestibular schwannomas are slow-growing tumors of the myelin-forming cells that cover cranial nerve VIII. The treatment options for patients with vestibular schwannoma include active observation, surgical management, and radiotherapy. However, the optimal treatment choice remains controversial. We have reviewed the available data and summarized the radiotherapeutic options, including single-session stereotactic radiosurgery, fractionated conventional radiotherapy, fractionated stereotactic radiotherapy, and proton beam therapy. The comparisons of the various radiotherapy modalities have been based on single-institution experiences, which have shown excellent tumor control rates of 91-100%. Both stereotactic radiosurgery and fractionated stereotactic radiotherapy have successfully improved cranial nerve V and VII preservation to >95%. The mixed data regarding the ideal hearing preservation therapy, inherent biases in patient selection, and differences in outcome analysis have made the comparison across radiotherapeutic modalities difficult. Early experience using proton therapy for vestibular schwannoma treatment demonstrated local control rates of 84-100% but disappointing hearing preservation rates of 33-42%. Efforts to improve radiotherapy delivery will focus on refined dosimetry with the goal of reducing the dose to the critical structures. As future randomized trials are unlikely, we suggest regimented pre- and post-treatment assessments, including validated evaluations of cranial nerves V, VII, and VIII, and quality of life assessments with long-term prospective follow-up. The results from such trials will enhance the understanding of therapy outcomes and improve our ability to inform patients.

Quality of Life in 807 Patients with Vestibular Schwannoma: Comparing Treatment Modalities.

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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.

Preclinical Validation of Anti-Nuclear Factor Kappa B Therapy against Vestibular Schwannoma and Neurofibromatosis Type II

Science.gov (United States)

2015-06-01

Thomsen, J., Nielsen, F.C., 2010. Deregulated genes in sporadic vestibular schwannomas. Otol. Neurotol. 31 (2), 256e266. Cioffi, J.A., Yue, W.Y., Mendolia...Thomas, R., Gope, M.L., Doddaballapur, S.K., Gope, R., 2006. Age dependent phosphorylation and deregulation of p53 in human vestibular schwannomas. Mol...tissue was visualized and imaged using a Carl Zeiss 2000 upright microscope (Carl Zeiss, Jena, Germany ). Schwann and schwannoma cell isolation and

Analysis on CT features of tumor-like gastric schwannomas

International Nuclear Information System (INIS)

Zhang Yu; Chen Jie

2011-01-01

Objective: To analyze CT imaging features of tumor-like gastric schwannomas. Methods: Ten patients with gastric schwannomas were retrospectively analyzed. All were scanned with pre-and pro-enhanced CT. Analysis of the CT findings included evaluation of the volume, number, location, contour, growth pattern, border, enhancement pattern, and enhancement grade as well as the presence of surface dimpling, integrity of overlying mucosa. All cases were confirmed by pathology. Results: In every case, simple tumor was present. The largest was about 5.7 cm in the diameter, the smallest was 2.3 cm. All tumors were round or oval, and one tumor was slightly lobulated. Endoluminal growth pattern was defined in two cases, exoluminal growth pattern was defined in one case, and a mixed growth pattern was noted in the rest. The borders of tumors were clear. In arterial phase, no visible enhancement was present in eight cases and mild enhancement in two cases. All cases were constantly enhanced in portal phase. Superficial ulcers were present in four cases. Conclusion: CT findings of tumor-like gastric schwannomas are distinctive to a certain degree. It can be used to guide clinical therapy. (authors)

Contribution of p75NTR to Schwannoma Growth and Therapeutic Responses

Science.gov (United States)

2017-05-01

reducing schwannoma cell growth in culture and in animal models of human schwannoma disease. We find that the NF2 gene product, merlin, regulates p75NTR...treatment period. B. Waterfall plots of change in tumor volume for each tumor. Task 6- Define the ability of simultaneous inhibition of p75NTR and mTOR...mTOR inhibitors in culture, we have not used these compounds in animal models . Changes that had a significant impact on expenditures- None

Extracranial Facial Nerve Schwannoma Treated by Hypo-fractionated CyberKnife Radiosurgery

OpenAIRE

Sasaki, Ayaka; Miyazaki, Shinichiro; Hori, Tomokatsu

2016-01-01

Facial nerve schwannoma is a rare intracranial tumor. Treatment for this benign tumor has been controversial. Here, we report a case of extracranial facial nerve schwannoma treated successfully by hypo-fractionated CyberKnife (Accuray, Sunnyvale, CA) radiosurgery?and discuss the efficacy of this treatment. A 34-year-old female noticed a swelling in her right mastoid process. The lesion enlarged over a seven-month period, and she experienced facial spasm on the right side. She was diagnosed wi...

Long-Term Results for Trigeminal Schwannomas Treated With Gamma Knife Surgery

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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

MRI of infections and neoplasms of the spine and spinal cord in 55 patients with AIDS

Energy Technology Data Exchange (ETDEWEB)

Thurnher, M.M. [Neuroradiology Section, Department of Radiology, University Hospital Vienna (Austria); Post, M.J.D. [Department of Radiology, Neuroradiology Section, University of Miami School of Medicine, Miami, FL (United States); Jinkins, J.R. [Neuroimaging Research, Department of Radiology, Nebraska Medical Center, Omaha, NE (United States)

2000-08-01

Our purpose was to describe the range of MRI findings in infectious and neoplastic involvement of the spine and spinal cord in symptomatic patients with the acquired immunodeficiency syndrome (AIDS). MRI studies in 55 patients with AIDS and neurological signs and symptoms thought to be related to the spine or spinal cord were reviewed. We categorized the findings according to the spinal compartment involved. There were 29 patients with extradural, 11 with intradural-extramedullary and 9 with intramedullary disease. In 6 patients more than one compartment was involved simultaneously, and patients presented with multiple lesions in the same compartment. The most common causes of extradural disease were bone lesions (28); an epidural mass was seen in 14 and spondylodiscitis in 4 patients. Cytomegalovirus polyradiculitis was the most common cause of intradural-extramedullary disease (in 10 cases); herpes radiculitis was seen in two, and tuberculous infection in another two. In three cases leptomeningeal contrast enhancement was due to lymphoma. Human immunodeficiency virus (HIV) myelitis was seen in two patients, presumed vacuolar myelopathy in two, toxoplasma myelitis in four, intramedullary lymphoma in one, and herpes myelitis in one. Familiarity with the various potential pathological entities that can affect the spine and spinal cord in the AIDS population and their imaging characteristics is crucial for initiation of further diagnostic tests and appropriate medical or surgical treatment. (orig.)

MRI of infections and neoplasms of the spine and spinal cord in 55 patients with AIDS

International Nuclear Information System (INIS)

Thurnher, M.M.; Post, M.J.D.; Jinkins, J.R.

2000-01-01

Our purpose was to describe the range of MRI findings in infectious and neoplastic involvement of the spine and spinal cord in symptomatic patients with the acquired immunodeficiency syndrome (AIDS). MRI studies in 55 patients with AIDS and neurological signs and symptoms thought to be related to the spine or spinal cord were reviewed. We categorized the findings according to the spinal compartment involved. There were 29 patients with extradural, 11 with intradural-extramedullary and 9 with intramedullary disease. In 6 patients more than one compartment was involved simultaneously, and patients presented with multiple lesions in the same compartment. The most common causes of extradural disease were bone lesions (28); an epidural mass was seen in 14 and spondylodiscitis in 4 patients. Cytomegalovirus polyradiculitis was the most common cause of intradural-extramedullary disease (in 10 cases); herpes radiculitis was seen in two, and tuberculous infection in another two. In three cases leptomeningeal contrast enhancement was due to lymphoma. Human immunodeficiency virus (HIV) myelitis was seen in two patients, presumed vacuolar myelopathy in two, toxoplasma myelitis in four, intramedullary lymphoma in one, and herpes myelitis in one. Familiarity with the various potential pathological entities that can affect the spine and spinal cord in the AIDS population and their imaging characteristics is crucial for initiation of further diagnostic tests and appropriate medical or surgical treatment. (orig.)

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.

Schwannoma of the left brachial plexus mimicking a ...

African Journals Online (AJOL)

Schwannoma of the left brachial plexus mimicking a cervicomediastinal ... Her voice was hoarse but there was no eye signs suggestive of thyrotoxicosis. ... A presumptive diagnosis of thyroid carcinoma with retrosternal extension was made.

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...

[Primary malignant schwannoma of the buccal branch of facial nerve].

Science.gov (United States)

Moumine, M; Thiery, G; Harroudi, T; Amrani, M; El Othmany, A; Rzin, A

2012-06-01

Primary malignant schwannomas are rare neoplasms of nerve sheath origin, especially in the location of the head and neck where few cases are described in the literature. We report the case of a 65-year-old male diagnosed with malignant schwannoma in the left cheek. The patient underwent surgery with wide local excision, reconstruction were made later by skin graft. The treatment of choice is radical excision of the lesion with wide margins. In fact, to reduce local tumor recurrence, the use of adjuvant radiation or chemotherapy is still controversial. Copyright © 2009 Elsevier Masson SAS. All rights reserved.

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.

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.)

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

2012-01-01

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 making in ambiguous cases. PMID:22561798

Vestibular Schwannoma or acoustic neuroma

Directory of Open Access Journals (Sweden)

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.

Removal of vestibular schwannoma and facial nerve preservation using small suboccipital retrosigmoid craniotomy

Institute of Scientific and Technical Information of China (English)

CHEN Ling; CHEN Li-hua; LING Feng; LIU Yun-sheng; Madjid Samii; Amir Samii

2010-01-01

Background Vestibular schwannoma, the commonest form of intracranial schwannoma, arises from the Schwann cells investing the vestibular nerve. At present, the surgery for vestibular schwannoma remains one of the most complicated operations demanding for surgical skills in neurosurgery. And the trend of minimal invasion should also be the major influence on the management of patients with vestibular schwannomas. We summarized the microsurgical removal experience in a recent series of vestibular schwannomas and presented the operative technique and cranial nerve preservation in order to improve the rates of total tumor removal and facial nerve preservation.Methods A retrospective analysis was performed in 145 patients over a 7-year period who suffered from vestibular schwannomas that had been microsurgicaily removed by suboccipital retrosigmoid transmeatus approach with small craniotomy. CT thinner scans revealed the tumor size in the internal auditory meatus and the relationship of the posterior wall of the internal acoustic meatus to the bone labyrinths preoperatively. Brain stem evoked potential was monitored intraoperatively. The posterior wall of the internal acoustic meatus was designedly drilled off. Patient records and operative reports, including data from the electrophysiological monitoring, follow-up audiometric examinations, and neuroradiological findings were analyzed.Results Total tumor resection was achieved in 140 cases (96.6%) and subtotal resection in 5 cases. The anatomical integrity of the facial nerve was preserved in 91.0% (132/145) of the cases. Intracranial end-to-end anastomosis of the facial nerve was performed in 7 cases. Functional preservation of the facial nerve was achieved in 115 patients (Grade Ⅰ and Grade Ⅱ, 79.3%). No patient died in this series. Preservation of nerves and vessels were as important as tumor removal dudng the operation. CT thinner scan could show the relationship between the posterior wall of the internal

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

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.

Facial Nerve Schwannoma: A Case Report, Radiological Features and Literature Review.

Science.gov (United States)

Pilloni, Giulia; Mico, Barbara Massa; Altieri, Roberto; Zenga, Francesco; Ducati, Alessandro; Garbossa, Diego; Tartara, Fulvio

2017-12-22

Facial nerve schwannoma localized in the middle fossa is a rare lesion. We report a case of a facial nerve schwannoma in a 30-year-old male presenting with facial nerve palsy. Magnetic resonance imaging (MRI) showed a 3 cm diameter tumor of the right middle fossa. The tumor was removed using a sub-temporal approach. Intraoperative monitoring allowed for identification of the facial nerve, so it was not damaged during the surgical excision. Neurological clinical examination at discharge demonstrated moderate facial nerve improvement (Grade III House-Brackmann).

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

Directory of Open Access Journals (Sweden)

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.

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...

[Extramedullary fixation combined with intramedullary fixation in the surgical reduction of sagittal mandibular condylar fractures].

Science.gov (United States)

Chuanjun, Chen; Xiaoyang, Chen; Jing, Chen

2016-10-01

This study aimed to evaluate the clinical effect of extramedullary fixation combined with intramedullary fixation during the surgical reduction of sagittal mandibular condylar fractures. Twenty-four sagittal fractures of the mandibular condyle in18 patients were fixed by two appliances: intramedullary with one long-screw osteosynthesis or Kirschner wire and extramedullary with one micro-plate. The radiologically-recorded post-operative stability-associated com-plications included the screw/micro-plate loosening, micro-plate twisting, micro-plate fractures, and fragment rotation. The occluding relations, the maximalinter-incisal distances upon mouth opening, and the mandibular deflection upon mouth opening were evaluated based on follow-up clinical examination. Postoperative panoramic X-ray and CT scans showed good repositioning of the fragment, with no redislocation or rotation, no screw/plate loosening, and no plate-twisting or fracture. Clinical examination showed that all patients regained normal mandibular movements, ideal occlusion, and normal maximal inter-incisal distances upon mouth opening. Extramedullary fixation combined with intramedullary fixation is highly recommended for sagittal condylar fractures because of the anti-rotation effect of the fragment and the reasonable place-ment of the fixation appliances.

Schwannoma of the descending loop of the hypoglossal nerve: Case report.

Science.gov (United States)

Illuminati, Giulio; Pizzardi, Giulia; Pasqua, Rocco; Palumbo, Piergaspare; Vietri, Francesco

2017-01-01

Schwannomas of the descending loop of the hypoglossal nerve are very rare. They are slow-growing tumors that may masquerade a carotid body tumor. A 60-year-old female was referred for a latero-cervical mass appearing as a chemodectoma at CT-scan. At operation, a 2cm mass arising from the descending loop of the hypoglossal nerve was resected en bloc with the loop itself and a functional lymphadenectomy was associated. Post-operative course was uneventful and the patient is free from disease recurrence at one year follow-up. En bloc resection remains the real curative treatment of Schwannomas, ensuring unlimited freedom from disease, although causing functional impairment which may be significant. Nonetheless recurrence should be prevented as, beside requiring reintervention, it may harbor a malignant evolution towards sarcoma. Schwannomas of the descending lop of the hypoglossal nerve may masquerade a chemodectoma of the carotid bifurcation and can be curatively resected without any functional impairment. Copyright © 2017 The Author(s). Published by Elsevier Ltd.. All rights reserved.

Cellular schwannoma arising from sigmoid mesocolon presenting ...

African Journals Online (AJOL)

Schwannomas are a type of peripheral nerve sheath tumors with clinically indolent behavior. Though, they can occur anywhere in body, the incidence in retroperitoneum, mediastinum, and pelvis is exceedingly rare. We present a case of a 58‑year‑old female with a massive twisted tumor arising from sigmoid mesocolon.

Cellular Schwannoma Arising from Sigmoid Mesocolon Presenting ...

African Journals Online (AJOL)

Schwannomas are a type of peripheral nerve sheath tumors with clinically indolent behavior. Though, they can occur anywhere in body, the incidence in retroperitoneum, mediastinum, and pelvis is exceedingly rare. We present a case of a 58‑year‑old female with a massive twisted tumor arising from sigmoid mesocolon.

Reporting success rates in the treatment of vestibular schwannomas: are we accounting for the natural history?

Science.gov (United States)

Miller, Timothy; Lau, Tsz; Vasan, Rohit; Danner, Christopher; Youssef, A Samy; van Loveren, Harry; Agazzi, Siviero

2014-06-01

Stereotactic radiosurgery is generally accepted as one of the best treatment options for vestibular schwannomas. We question whether growth control is an accurate measure of success in vestibular schwannoma treatment. We aim to clarify the success rate of stereotactic radiosurgery and adjust the reported results to the benign natural history of untreated tumors. All articles were taken from a PubMed search of the English literature from the years 2000-2011. Inclusion criteria were articles containing the number of patients treated, radiation technique, average tumor size, follow-up time, and percentage of tumors growing during follow-up. Data were extracted from 19 articles. Success rates were adjusted using published data that 17% to 30% of vestibular schwannomas grow. The average reported success rate for stereotactic radiosurgery across all articles was 95.5%. When considering 17% or 30% natural growth without intervention, the adjusted success rates became 78.2% and 86.9% respectively. These rates were obtained by applying the natural history growth percentages to any tumors not reported to be growing before radiosurgical intervention. Success in the treatment of vestibular schwannomas with stereotactic radiosurgery is often defined as lack of further growth. Recent data on the natural growth history of vestibular schwannomas raise the question of whether this is the best definition of success. We have identified a lack of continuity regarding the reporting of success and emphasize the importance of the clarification of the success of radiosurgery to make informed decisions regarding the best treatment options for vestibular schwannoma. Copyright © 2014 Elsevier Ltd. All rights reserved.

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.

Extradural spinal schwannoma at cervical spine in 12 year old child

Directory of Open Access Journals (Sweden)

Madoori Srinivas

2016-10-01

Full Text Available Spinal schwannomas are benign tumors arising from spinal nerve root sheaths. It is a primary spinal tumor which are rare in children. We report a case of a 12 year old girl who presented with weakness of all limbs and unable to walk. Imaging studies demonstrated an extradural spinal tumor at cervical spine. The patient was operated and tumor was totally removed. The postoperative course was uneventful. Histology confirmed the diagnosis of schwannoma. After surgery there was improvement in signs and symptoms. After two months of operation, child could able to walk normally.

Mixed phenotype (T/B/myeloid) extramedullary blast crisis as an initial presentation of chronic myelogenous leukemia.

Science.gov (United States)

Qing, Xin; Qing, Annie; Ji, Ping; French, Samuel W; Mason, Holli

2018-04-01

Chronic myelogenous leukemia (CML) is a myeloproliferative disorder characterized by the Philadelphia (Ph) chromosome generated by the reciprocal translocation t(9,22)(q34;q11). The natural progression of the disease follows a biphasic or triphasic course. Most cases of CML are diagnosed in the chronic phase. Extramedullary blast crisis rarely occurs during the course of CML, and is extremely rare as the initial presentation of CML. Here, we report the case of a 32-year-old female with enlarged neck lymph nodes and fatigue. She was diagnosed with B-lymphoblastic leukemia/lymphoma with possible mixed phenotype (B/myeloid) by right neck lymph node biopsy at an outside hospital. However, review of her peripheral blood smear and her bone marrow aspirate and biopsy showed features consistent with CML, which was confirmed by PCR and karyotyping. An ultrasound-guided right cervical lymph node core biopsy showed a diffuse infiltrate of blasts, near totally replacing the normal lymph node tissue, admixed with some hematopoietic cells including megakaryocytes, erythroid precursors and maturing myeloid cells. By flow cytometry and immunohistochemistry, the blasts expressed CD2, cytoplasmic CD3, CD5, CD7, CD56, TdT, CD10 (weak, subset), CD19 (subset), CD79a, PAX-5 (subset), CD34, CD38, CD117 (subset), HLA-DR (subset), CD11b, CD13 (subset), CD33 (subset), and weak cytoplasmic myeloperoxidase, without co-expression of surface CD3, CD4, CD8, CD20, CD22, CD14, CD15, CD16 and CD64, consistent with blasts with mixed phenotype (T/B/myeloid). A diagnosis of extramedullary blast crisis of CML was made. Chromosomal analysis performed on the lymph node biopsy tissue revealed multiple numerical and structural abnormalities including the Ph chromosome (46-49,XX,add(1)(p34),add(3)(p25),add(5)(q13),-6,t(9;22)(q34;q11.2),+10,-15,add(17)(p11.2),+19, +der(22)t(9;22),+mar[cp8]). After completion of one cycle of combined chemotherapy plus dasatinib treatment, she was transferred to City of Hope

Facial Nerve Schwannoma Involving Middle Cranial Fossa: When the Unilateral Sensorineural Hearing Loss Guide to the Correct Diagnosis

OpenAIRE

De Stefano, Alessandro; Dispenza, Francesco; Kulamarva, Gautham

2011-01-01

The Facial Nerve Schwannoma is a rare tumor and it seldom involved the middle cranial fossa. Facial nerve schwannoma has various manifestations, including facial palsy but unfortunately facial nerve is very resistant to compression and often facial nerve paralysis or a facial weakness are not present. We present a case of giant facial nerve schwannoma involved the middle cranial fossa without facial nerve paralysis. In these cases the unilateral hearing loss (if present) guide to a correct di...

Intralabyrinthine schwannoma shown by magnetic resonance imaging

International Nuclear Information System (INIS)

Saeed, S.R.; Birzgalis, A.R.; Ramsden, R.T.

1994-01-01

Intralabyrinthine schwannomas are rare benign tumours which present with progressive or fluctuant audiovestibular symptoms and may mimic Menieres disease. The size and position of these lesions make preoperative diagnosis unusual and most are discovered incidentally at labyrinthectomy. A case is reported which was diagnosed on magnetic resonance imaging and confirmed at surgery. (orig.)

Intratemporal and extratemporal facial nerve schwannoma: CT and MRI findings

Energy Technology Data Exchange (ETDEWEB)

Kim, Keum Won [Pohang Medical Center, Pohang (Korea, Republic of); Lee, Ho Kyu; Shin, Ji Hoon; Choi, Choong Gon; Suh, Dae Chul [Asan Medical Center, Ulsan Univ. College of Medicine, Seoul (Korea, Republic of); Cheong, Hae Kwan [Dongguk Univ. College of Medicine, Seoul (Korea, Republic of)

2001-05-01

To analyze the characteristics of CT and MRI findings of facial nerve schwannoma in ten patients. Ten patients with pathologically confirmed facial nerve schwannoma, underwent physical and radilolgic examination. The latter involved MRI in all ten and CT scanning in six. We analyzed the location (epicenter), extent and number of involved segments of tumors, tuumor morphology, and changes in adjacent bony structures. The major symptoms of facial nerve schwannoma were facial nerve paralysis in seven cases and hearing loss in six. Epicenters were detected at the intraparotid portion in five cases, the intracanalicular portion in two, the cisternal portion in one, and the intratemporal portion in two. The segment most frequently involved was the mastoid (n=6), followed by the parotid (n=5), intracanalicular (n=4), cisternal (n=2), the labyrinthine/geniculate ganglion (n=2) and the tympanic segment (n=1). Tumors affected two segments of the facial nerve in eight cases, only one segment in one, and four continuous segments in one. Morphologically, tumors were ice-cream cone shaped in the cisternal segment tumor (1/1), cone shaped in intracanalicular tumors (2/2), oval shaped in geniculate ganglion tumors (1/1), club shaped in intraparotid tumors (5/5) and bead shaped in the diffuse-type tumor (1/1). Changes in adjacent bony structures involved widening of the stylomastoid foramen in intraparotid tumors (5/5), widening of the internal auditary canal in intracanalicular and cisternal tumors (3/3), bony erosion of the geniculate fossa in geniculate ganglion tumors (2/2), and widening of the facial nerve canal in intratemporal and intraparotid tumors (6/6). The characteristic location, shape and change in adjacent bony structures revealed by facial schwannomas on CT and MR examination lead to correct diagnosis.

Intratemporal and extratemporal facial nerve schwannoma: CT and MRI findings

International Nuclear Information System (INIS)

Kim, Keum Won; Lee, Ho Kyu; Shin, Ji Hoon; Choi, Choong Gon; Suh, Dae Chul; Cheong, Hae Kwan

2001-01-01

To analyze the characteristics of CT and MRI findings of facial nerve schwannoma in ten patients. Ten patients with pathologically confirmed facial nerve schwannoma, underwent physical and radilolgic examination. The latter involved MRI in all ten and CT scanning in six. We analyzed the location (epicenter), extent and number of involved segments of tumors, tuumor morphology, and changes in adjacent bony structures. The major symptoms of facial nerve schwannoma were facial nerve paralysis in seven cases and hearing loss in six. Epicenters were detected at the intraparotid portion in five cases, the intracanalicular portion in two, the cisternal portion in one, and the intratemporal portion in two. The segment most frequently involved was the mastoid (n=6), followed by the parotid (n=5), intracanalicular (n=4), cisternal (n=2), the labyrinthine/geniculate ganglion (n=2) and the tympanic segment (n=1). Tumors affected two segments of the facial nerve in eight cases, only one segment in one, and four continuous segments in one. Morphologically, tumors were ice-cream cone shaped in the cisternal segment tumor (1/1), cone shaped in intracanalicular tumors (2/2), oval shaped in geniculate ganglion tumors (1/1), club shaped in intraparotid tumors (5/5) and bead shaped in the diffuse-type tumor (1/1). Changes in adjacent bony structures involved widening of the stylomastoid foramen in intraparotid tumors (5/5), widening of the internal auditary canal in intracanalicular and cisternal tumors (3/3), bony erosion of the geniculate fossa in geniculate ganglion tumors (2/2), and widening of the facial nerve canal in intratemporal and intraparotid tumors (6/6). The characteristic location, shape and change in adjacent bony structures revealed by facial schwannomas on CT and MR examination lead to correct diagnosis

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.

Improved results for vestibular schwannoma radiosurgery

Energy Technology Data Exchange (ETDEWEB)

Flickinger, J C; Kondziolka, D; Pollock, B; Lunsford, L D

1995-07-01

PURPOSE/OBJECTIVE: Treatment techniques in radiosurgery have changed since 1987. We reviewed patients who received radiosurgery for vestibular schwannoma to identify these changes and to investigate any differences in tumor control and complications. MATERIALS and METHODS: One hundred thirty-eight unilateral vestibular schwannoma patients with a minimum follow-up of two years after treatment with gamma knife radiosurgery between 1987 and 1992 were analyzed. The early treatment group consisted of 55 patients treated between 1987-1989 (median: tumor volume 3.63 cc, Dmin 18.1 Gy, Dmax 35.4 Gy, isocenters 2.3, follow-up 50.4 mos.). The later treatment group consisted of 83 patients treated between 1990-1992 (median: tumor volume 3.81 cc, Dmin 16.0 Gy, Dmax 31.6 Gy, isocenters 4.7, follow-up 35.8 mos.) RESULTS: Clinical tumor recurrence requiring surgical intervention occurred in one patient in each group. The overall actuarial clinical tumor control rate was 98%. Slight increases in tumor size (1 to 2 mm) were identified in five other patients not requiring intervention, because of no further tumor growth (n=4) or shrinkage (n=1). This led to an overall radiologic tumor control rate of 92% (not significantly different in either group). Compared to the early treatment group, the incidence of facial neuropathy (temporary or permanent) decreased in the later group (49% vs. 11%, p < 0.0001), as did trigeminal neuropathy (40% vs. 8%, p < 0.0001). Serviceable hearing preservation improved only slightly in the later group (27% vs. 40%, p = 0.70). CONCLUSION: We document a significant decrease in the morbidity of vestibular schwannoma radiosurgery over this time period with no decrease in the high rate of tumor control. This improvement is attributed to a) better conformal dose-planning with stereotactic MRI rather than CT, b) an increase in the number of isocenters used, and c) a reduction in the average dose administered by 2 Gy.

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.

Extramedullary plasmacytoma of the testicle.

Science.gov (United States)

Carrion, Diego M; Álvarez-Maestro, Mario; Gómez Rivas, Juan; González-Peramato, Pilar; Cisneros Ledo, Jesús

2017-12-01

We present the case of a patient diagnosed with a testicular extramedullary plasmacytoma (EMP), and perform a brief review of the literature of this pathology. A 64 year-old male patient, with history of multiple myeloma successfully treated three years before, presented with left testicular swelling. Initial work-up was compatible with a testicular tumor and radical inguinal orchiectomy was performed. Histologic examination of the testis revealed extensive intertubular infiltration by CD138 and CD56 atypical plasma cells, with diffuse staining for IgA, compatible with EMP. Invasion of the testis in multiple myeloma patients as a recurrence of the disease is an extremely rare condition, as EMPs are more common in other organ systems. Initial treatment should be the same as a primary testicular tumor with radical inguinal orchiectomy, and definitive diagnosis is established in histologic analysis.

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.

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

A neurofibromatosis type 2 case with vestibular, trigeminal and facial schwannomas together: magnetic resonance imaging findings

Energy Technology Data Exchange (ETDEWEB)

Akay, S; Hamcan, S; Kara, K; Battal, B; Tasar, M

2012-07-01

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.

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

Intramedullary Recurrence of a Thoracic Meningioma-Presentation of an Unusual Case and Review of the Literature.

Science.gov (United States)

Piazza, Matthew A; Ramayya, Ashwin G; Geiger, Geoffrey A; Alonso-Basanta, Michelle; Nasrallah, MacLean P; Welch, William C; Ozturk, Ali K

2016-08-01

Spinal meningiomas are typically extra-axial, slow-growing, benign tumors that arise from the arachnoid cap cells. Intramedullary spinal meningiomas are exceedingly rare with few cases reported in the literature. A 64-year-old man with a history of grade I thoracic meningioma at the T4 level resected initially in 1989 and who required reoperation in 2013 for intradural, extramedullary recurrence of tumor presented again in 2015 with gait difficulty. Magnetic resonance imaging revealed a soft tissue mass at the T3 to T4 levels on the left side of the canal that was mildly enhancing on T1 contrasted sequences. The patient was taken to the operating room, where a purely intramedullary recurrence was discovered without extramedullary extension or a dural-based attachment. The intramedullary tumor was completely resected, and postoperatively the patient recovered well and was at his neurologic baseline. The patient ultimately underwent proton beam radiotherapy because this tumor, although benign, had recurred twice. Intramedullary spinal meningiomas, particularly intramedullary low-grade recurrence of a previously extramedullary tumor, are rare phenomena. Although the pathogenic mechanisms are not well understood, intramedullary recurrence as described in this patient may reflect extrinsic factors related to prior surgical resections in addition to histologic progression. When operating on recurrent extramedullary lesions, aggressive arachnoid dissection may predispose patients to unusual patterns of recurrence. Copyright © 2016 Elsevier Inc. All rights reserved.

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.

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.

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

Jugular Foramen Collision Tumor (Schwannoma and Plasma Cell Pseudotumor), a Probable IgG4-Related Disease.

Science.gov (United States)

Bakhit, Mudathir S; Fujii, Masazumi; Jinguji, Shinya; Sato, Taku; Sakuma, Jun; Saito, Kiyoshi

2017-06-01

Lower cranial nerve sheath tumors are relatively rare. Cases of schwannoma collision tumors have rarely been reported, with most of the reported cases describing schwannoma and meningioma collision tumors. We report a very rare case of a cerebellopontine angle collision tumor of the ninth cranial nerve schwannoma with an IgG4 plasma cell pseudotumor. IgG4 plasma cell pseudotumors comprise a group of diseases called IgG4-related diseases (IgG4-RDs). These diseases usually affect organs such as the pancreas and salivary gland. Few cases of nervous system IgG4-RDs have been reported. Under intraoperative microscopy, the tumor in our case did not appear different from usual cases of schwannoma, but histopathology showed significant infiltration of IgG4 plasma cells. IgG4-RDs have a distinctive histopathologic pattern; however, their pathophysiology remains unclear. Special attention must be paid to the diagnosis of such diseases because they mimic other diseases and can be missed. Copyright © 2017 Elsevier Inc. All rights reserved.

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.

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.

Extramedullary plasmacytoma: clinical and histopathologic study.

Science.gov (United States)

Strojan, Primoz; Soba, Erika; Lamovec, Janez; Munda, Anton

2002-07-01

To review the histories of extramedullary plasmacytoma patients diagnosed in Slovenia between 1969 and 1999, to determine the relationship between radiotherapy (XRT) dose and local tumor control, and to clarify the role of elective nodal XRT and the prognostic value of Bartl's histologic grading criteria (originally devised for multiple myeloma [MM]). The database of the Cancer Registry of Slovenia was used for the identification of patients. The inclusion criteria were as follows: bone marrow biopsy showing less than 10% plasma cells, normal skeletal survey, and immunohistochemically determined tumor monoclonality. Simulation/portal films were reviewed to assess the extent of elective nodal XRT. Twenty-six patients with 31 tumors fulfilled the inclusion criteria. In 4 patients, nine metachronously appearing solitary tumors were diagnosed. The head-and-neck region and other body sites were the sites of origin of primary tumors in 84% and 16% of patients, respectively, whereas in the two regions, regional disease was seen in 15% and 60% of patients, respectively. Therapy was as follows: XRT, 12 patients; surgery and postoperative XRT, 15 patients; and surgery, 4 patients. Ultimate local and regional control rates were 90% and 97%, respectively, and MM developed in 2 (8%) patients. The 10-year disease-specific and overall survival rates were 87% and 61%, respectively. The analysis of the dose-effect relationship showed that more conservative treatment is justified: for macroscopic disease, 40-50 Gy (2 Gy/day), adjusted to the bulk of disease; for microscopic disease, 36-40 Gy; after R0 surgery, no XRT is required, but close observation is needed. No attempts should be made to treat uninvolved nodal regions. Using Bartl's histologic grading criteria, trends were detected in patients with higher tumor grades: regional lymph node involvement (p = 0.04) and shorter disease-specific survival (p = 0.08). Extramedullary plasmacytoma is a highly curable disease when XRT is

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...

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...

Solitary extramedullary plasmacytomas of thyroid in Hashimoto's thyroiditis: Mimicking benign cystic nodule on ultrasonography

International Nuclear Information System (INIS)

Kwon, Yohan; Kim, Soo Jin; Hur, Joon Ho; Park, Sung Hee; Lee, Sun Jin; Lee, Tae Jin

2013-01-01

Solitary extramedullary plasmacytoma (SEP) of the thyroid is uncommon and mostly occur in patients with a Hashimoto's thyroiditis (82%). We present a case on SEP of thyroid in Hashimoto's thyroiditis, which mimics growing benign cystic masses on serial ultrasonography.

Diagnostics and therapy of vestibular schwannomas – an interdisciplinary challenge

Science.gov (United States)

Rosahl, Steffen; Bohr, Christopher; Lell, Michael; Hamm, Klaus; Iro, Heinrich

2017-01-01

Vestibular schwannomas (VS) expand slowly in the internal auditory canal, in the cerebellopontine angle, inside the cochlear and the labyrinth. Larger tumors can displace and compress the brainstem. With an annual incidence of 1:100,000 vestibular schwannoma represent 6–7% of all intracranial tumors. In the cerebellopontine angle they are by far the most neoplasm with 90% of all lesions located in this region. Magnetic resonance imaging (MRI), audiometry, and vestibular diagnostics are the mainstays of the clinical workup for patients harboring tumors. The first part of this paper delivers an overview of tumor stages, the most common grading scales for facial nerve function and hearing as well as a short introduction to the examination of vestibular function. Upholding or improving quality of life is the central concern in counseling and treating a patient with vestibular schwannoma. Preservation of neuronal function is essential and the management options – watchful waiting, microsurgery and stereotactic radiation – should be custom-tailored to the individual situation of the patient. Continuing interdisciplinary exchange is important to monitor treatment quality and to improve treatment results. Recently, several articles and reviews have been published on the topic of vestibular schwannoma. On the occasion of the 88th annual meeting of the German Society of Oto-Rhino-Laryngology, Head and Neck surgery a special volume of the journal “HNO” will be printed. Hence this presentation has been designed to deviate from the traditional standard which commonly consists of a pure literature review. The current paper was conceptually woven around a series of interdisciplinary cases that outline examples for every stage of the disease that show characteristic results for management options to date. Systematic clinical decision pathways have been deduced from our experience and from results reported in the literature. These pathways are graphically outlined after

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 ...

Proton beam stereotactic radiosurgery of vestibular schwannomas

International Nuclear Information System (INIS)

Harsh, Griffith R.; Thornton, Allan F.; Chapman, Paul H.; Bussiere, Marc R.; Rabinov, James D.; Loeffler, Jay S.

2002-01-01

Purpose: The proton beam's Bragg peak permits highly conformal radiation of skull base tumors. This study, prompted by reports of transient (30% each) and permanent (10% each) facial and trigeminal neuropathy after stereotactic radiosurgery of vestibular schwannomas with marginal doses of 16-20 Gy, assessed whether proton beam radiosurgery using a marginal dose of only 12 Gy could control vestibular schwannomas while causing less neuropathy. Methods and Materials: Sixty-eight patients (mean age 67 years) were treated between 1992 and 1998. The mean tumor volume was 2.49 cm 3 . The dose to the tumor margin (70% isodose line) was 12 Gy. The prospectively specified follow-up consisted of neurologic evaluation and MRI at 6, 12, 24, and 36 months. Results: After a mean clinical follow-up of 44 months and imaging follow-up of 34 months in 64 patients, 35 tumors (54.7%) were smaller and 25 (39.1%) were unchanged (tumor control rate 94%; actuarial control rate 94% at 2 years and 84% at 5 years). Three tumors enlarged: one shrank after repeated radiosurgery, one remained enlarged at the time of unrelated death, and one had not been imaged for 4 years in a patient who remained asymptomatic at last follow-up. Intratumoral hemorrhage into one stable tumor required craniotomy that proved successful. Thus, 97% of tumors required no additional treatment. Three patients (4.7%) underwent shunting for hydrocephalus evident as increased ataxia. Of 6 patients with functional hearing ipsilaterally, 1 improved, 1 was unchanged, and 4 progressively lost hearing. Cranial neuropathies were infrequent: persistent facial hypesthesia (2 new, 1 exacerbated; 4.7%); intermittent facial paresthesias (5 new, 1 exacerbated; 9.4%); persistent facial weakness (2 new, 1 exacerbated; 4.7%) requiring oculoplasty; transient partial facial weakness (5 new, 1 exacerbated; 9.4%), and synkinesis (5 new, 1 exacerbated; 9.4%). Conclusion: Proton beam stereotactic radiosurgery of vestibular schwannomas at the

Schwannoma of the 6th nerve: case report and review of the literature

Institute of Scientific and Technical Information of China (English)

Xin Li; Jingjun Li; Jing Li; Zhen Wu

2015-01-01

Introduction: Schwannomas of the 6th nerve are extremely rare.Only 22 cases of intracranial schwannomas of the 6th cranial nerve pathologically confirmed have been reported.Case Presentation: Here, we report a case of a 14-year-old girl who presented with isolated progressive 6th nerve palsy.A cisternal type of schwannomawas found from neuro-imaging.Subtotal removal of the tumor was performed by a routine left subtemporal craniotomy with an petrosectomy.The attachment to the 6th nerve was found.After surgery, the patient's 6th nerve palsy remained.Histological images revealed a cellular schwannoma.Then the classification, clinical presentation, diagnosis, operation, stereotactic radiosurgery are reviewed.Conclusions: The location and the attachment to the 6th nerve is the key for diagnosis.Most cases are treated surgically.It looks like it is not easy to completely remove for CA type because of invasion of the cavernous sinus or firm adherence to the nerves.The 6th nerve function seldom completely recovered postoperatively.The direction of further research is to improve the diagnosis and therapy to have better nerve recovery.

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

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)

Acute necrosis after Gamma Knife surgery in vestibular schwannoma leading to multiple cranial nerve palsies.

Science.gov (United States)

Kapitza, Sandra; Pangalu, Athina; Horstmann, Gerhard A; van Eck, Albert T; Regli, Luca; Tarnutzer, Alexander A

2016-08-01

We discuss a rare acute complication after Gamma Knife therapy (Elekta AB, Stockholm, Sweden) in a single patient. A 52-year-old woman presented with vertigo, facial weakness and hearing loss emerging 48hours following Gamma Knife radiosurgery for a right-sided vestibular schwannoma. Neurological examination 6days after symptom onset showed right-sided facial palsy, spontaneous left-beating nystagmus and pathologic head-impulse testing to the right. Pure-tone audiogram revealed right-sided sensorineural hearing loss. A diagnosis of acute vestibulocochlear and facial neuropathy was made. Brain MRI demonstrated focal contrast sparing within the schwannoma, likely related to acute radiation necrosis. Acute multiple cranial neuropathies of the cerebellopontine angle after Gamma Knife treatment should raise suspicion of acute tissue damage within the schwannoma and should result in urgent MRI. Treatment with steroids may be considered based on accompanying swelling and edema. Copyright © 2016 Elsevier Ltd. All rights reserved.

Evaluation of spinal cord vessels using multi-slice CT angiography

International Nuclear Information System (INIS)

Chen Shuang; Zhu Ruijiang; Feng Xiaoyuan

2006-01-01

Objective: To evaluate the value of Multi-slice spiral CT angiography for spinal cord vessels. Methods: 11 adult subjects with suspected of myelopathy were performed with Multi-slice spiral CT angiography, An iodine contrast agent was injected at 3.5 ml/s, for total 100 ml. The parameters were axial 16 slice mode, 0.625 mm slice thickness, 0.8 s rotation, delay time depending on smartprep(15-25 s), multi-phase scan. The coronal and sagittal MPR and SSD were generated on a workstation compared with spinal digital subtraction angiography (DSA) to analyze normal or abnormal spinal cord vessels. Results: Normal findings at spinal CTA and digital subtraction angiography in six adult normal subjects and spinal cord vascular malformations (1 intradural extramedullary AVF, 4 dural AVFs) in five cases, Recognizable intradural vessels corresponding to anterior median (midline) veins and/or anterior spinal arteries were show in six adult normal subjects. Abnormal intradural vessels were detected in all five spinal cord vascular malformation with CT angiography, in comparison with digital subtraction angiography these vessels were primarily enlarged veins of the coronal venous plexus on the cord surface, radiculomedullary-dural arteries could not be clearly shown in four dural AVF, only one anterior spinal artery was detected in one patient with intradural medullary AVF, which direct shunt between anterior spinal artery and perimedullary vein with tortuous draining vessel. Conclusion: Multi-slice CT angiography is able to visualize the normal or abnormal spinal cord vessels. It could be used as a noninvasive method to screen the spinal cord vascular disease. (authors)

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.

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

International Nuclear Information System (INIS)

Hofman, P.A.M.; Wilmink, J.T.

1996-01-01

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

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.

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.

The role of T2*-weighted gradient echo in the diagnosis of tumefactive intrahepatic extramedullary hematopoiesis in myelodysplastic syndrome and diffuse hepatic iron overload: a case report and review of the literature.

Science.gov (United States)

Belay, Abel A; Bellizzi, Andrew M; Stolpen, Alan H

2018-01-15

Extramedullary hematopoiesis is the proliferation of hematopoietic cells outside bone marrow secondary to marrow hematopoiesis failure. Extramedullary hematopoiesis rarely presents as a mass-forming hepatic lesion; in this case, imaging-based differentiation from primary and metastatic hepatic neoplasms is difficult, often leading to biopsy for definitive diagnosis. We report a case of tumefactive hepatic extramedullary hematopoiesis in the setting of myelodysplastic syndrome with concurrent hepatic iron overload, and the role of T2*-weighted gradient-echo magnetic resonance imaging in differentiating extramedullary hematopoiesis from primary and metastatic hepatic lesions. To the best of our knowledge, T2*-weighted gradient-echo evaluation of extramedullary hematopoiesis in the setting of diffuse hepatic hemochromatosis has not been previously described. A 52-year-old white man with myelodysplastic syndrome and marrow fibrosis was found to have a 4 cm hepatic lesion on ultrasound during workup for bone marrow transplantation. Magnetic resonance imaging revealed diffuse hepatic iron overload and non-visualization of the lesion on T2* gradient-echo sequence suggesting the presence of iron deposition within the lesion similar to that in background hepatic parenchyma. Subsequent ultrasound-guided biopsy of the lesion revealed extramedullary hematopoiesis. Six months later, while still being evaluated for bone marrow transplant, our patient was found to have poor pulmonary function tests. Follow-up computed tomography angiogram showed a mass within his right main pulmonary artery. Bronchoscopic biopsy of this mass once again revealed extramedullary hematopoiesis. He received radiation therapy to his chest. However, 2 weeks later, he developed mediastinal hematoma and died shortly afterward, secondary to respiratory arrest. Mass-forming extramedullary hematopoiesis is rare; however, our report emphasizes that it needs to be considered in the initial differential

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

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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.

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.

Anteriorly placed tumors to the conus: removal by interradicular window.

Science.gov (United States)

Ciappetta, P; Taurone, S; Spoletini, M; Artico, M

2017-01-01

Tumors anteriorly situated to the medullary conus are rarely encountered and represent a true surgical challenge. We examined the literature on this topic, concluding that there are no previous reports on alternative surgical techniques different to the traditional one. We report two cases of intradural extramedullary tumor operated on by a technique performed through a window opened between the spinal roots, which allows an easy, effective and useful resection. We describe a new operative technique which ensures a complete removal of these tumors and discuss clinical implications in the light of the available literature on this topic.

INTRACRANIAL AND INTRASPINAL TUMOURS: REVIEW OF MRI FINDINGS IN A RARE NF - 2 CASE

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Sahoo

2015-02-01

Full Text Available Neurofibromatosis type 2 is a rare autosomal dominant syndrome, characterized by multiple intracranial and intraspinal tumours associated with ocular abnormalities. The most common tumor associated with the disease is the vestibule cochlear schwannoma, and as many as 10% of patients with this tumor have neurofibromatosis type 2. Based on clinical and imaging findings the diagnostic of neurofibromatosis type 2 can be made. In this report we aim to report a 24 - year - old male who was evaluated for progressive h earing loss, vertigo, ataxic gait and right lower limb weakness. During the workup, cranial CT, Brain and whole s pine MRI was done which showed all the findings in one patient including bilateral vestibulocochlear schwannoma, multiple meningiomas, and intr amedullary and extramedullary tumours in spinal cord.

Extramedullary plasmacytoma: clinical and histopathologic study

International Nuclear Information System (INIS)

Strojan, Primoz; Soba, Erika; Lamovec, Janez; Munda, Anton

2002-01-01

Purpose: To review the histories of extramedullary plasmacytoma patients diagnosed in Slovenia between 1969 and 1999, to determine the relationship between radiotherapy (XRT) dose and local tumor control, and to clarify the role of elective nodal XRT and the prognostic value of Bartl's histologic grading criteria (originally devised for multiple myeloma [MM]). Methods and Materials: The database of the Cancer Registry of Slovenia was used for the identification of patients. The inclusion criteria were as follows: bone marrow biopsy showing less than 10% plasma cells, normal skeletal survey, and immunohistochemically determined tumor monoclonality. Simulation/portal films were reviewed to assess the extent of elective nodal XRT. Results: Twenty-six patients with 31 tumors fulfilled the inclusion criteria. In 4 patients, nine metachronously appearing solitary tumors were diagnosed. The head-and-neck region and other body sites were the sites of origin of primary tumors in 84% and 16% of patients, respectively, whereas in the two regions, regional disease was seen in 15% and 60% of patients, respectively. Therapy was as follows: XRT, 12 patients; surgery and postoperative XRT, 15 patients; and surgery, 4 patients. Ultimate local and regional control rates were 90% and 97%, respectively, and MM developed in 2 (8%) patients. The 10-year disease-specific and overall survival rates were 87% and 61%, respectively. The analysis of the dose-effect relationship showed that more conservative treatment is justified: for macroscopic disease, 40-50 Gy (2 Gy/day), adjusted to the bulk of disease; for microscopic disease, 36-40 Gy; after R0 surgery, no XRT is required, but close observation is needed. No attempts should be made to treat uninvolved nodal regions. Using Bartl's histologic grading criteria, trends were detected in patients with higher tumor grades: regional lymph node involvement (p=0.04) and shorter disease-specific survival (p=0.08). Conclusions: Extramedullary

Successful radiotherapy at the rare extramedullary localization of a chemotherapy refractory relapse Burkitt-All

International Nuclear Information System (INIS)

Menschel, E.; Koller, E.; Bernhart, M.; Noesslinger, T.; Pfeilstoecker, M.

2003-01-01

The intensive therapy of the Burkitt-All achieved higher remission rates in the last years. Relapses and therapy refractory are a big medial problem. Radiotherapy in contribution with radiosensitizer used by an extramedullary large relapse of Burkitt-All is documented in this article as a successful therapy method. (boteke)

ROBOTIC SURGERY FOR GIANT PRESACRAL DUMBBELL-SHAPE SCHWANNOMA

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Farid Yudoyono

2015-03-01

Full Text Available Objective: To demonstrate the feasibility of using da Vinci robotic surgical system to perform spinal surgery. Methods: Magnetic resonance imaging (MRI of a 29-year-old female patient complaining right pelvic pain for 1 month revealed a 17x8x10 cm non-homogeneous dumbbell shape encapsulated mass with cystic change located in the pelvic cavity and caused an anterior displacement of urinary bladder and colon. Results: There was no systemic complication and pain decrease 24 hours after surgery and during 2 years of follow up. The patient started a diet 6 hours after the surgery and was discharged 72 hours after the surgery. The pathological diagnosis of the tumor was schwannoma. Conclusions: Giant dumbbell shape presacral schwannomas are rare tumours and their surgical treatment is challenging because of the complex anatomy of the presacral. Clinical application of da Vinci robotic surgical system in the spinal surgical field is currently confined to the treatment of some specific diseases or procedures. However, robotic surgery is expected to play a practical future role as it is minimally invasive. The advent of robotic technology will prove to be a boon to the neurosurgeon.

Radiotherapy of extramedullary plasmacytoma of the head and neck

International Nuclear Information System (INIS)

Harwood, A.R.; Knowling, M.A.; Bergsagel, D.E.

1981-01-01

The purpose of this study is to report the results of megavoltage irradiation with cobalt-60 in 23 previously unreported cases of extramedullary plasmacytoma of the head and neck. It has been found that 3500 cGy (rad) in three weeks provides good local control of disease with minimal morbidity and a significant proportion do not go on to multiple myeloma. Prognostic factors of significance with respect to subsequent development of multiple myeloma include site and presence or absence of bone destruction. The presence or absence of an M protein peak appears to be of no significance. (author)

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

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

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.

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.

Nasal septum extramedullary plasmacytoma

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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.

Vestibular schwannomas: Accuracy of tumor volume estimated by ice cream cone formula using thin-sliced MR images.

Science.gov (United States)

Ho, Hsing-Hao; Li, Ya-Hui; Lee, Jih-Chin; Wang, Chih-Wei; Yu, Yi-Lin; Hueng, Dueng-Yuan; Ma, Hsin-I; Hsu, Hsian-He; Juan, Chun-Jung

2018-01-01

We estimated the volume of vestibular schwannomas by an ice cream cone formula using thin-sliced magnetic resonance images (MRI) and compared the estimation accuracy among different estimating formulas and between different models. The study was approved by a local institutional review board. A total of 100 patients with vestibular schwannomas examined by MRI between January 2011 and November 2015 were enrolled retrospectively. Informed consent was waived. Volumes of vestibular schwannomas were estimated by cuboidal, ellipsoidal, and spherical formulas based on a one-component model, and cuboidal, ellipsoidal, Linskey's, and ice cream cone formulas based on a two-component model. The estimated volumes were compared to the volumes measured by planimetry. Intraobserver reproducibility and interobserver agreement was tested. Estimation error, including absolute percentage error (APE) and percentage error (PE), was calculated. Statistical analysis included intraclass correlation coefficient (ICC), linear regression analysis, one-way analysis of variance, and paired t-tests with P ice cream cone method, and ellipsoidal and Linskey's formulas significantly reduced the APE to 11.0%, 10.1%, and 12.5%, respectively (all P ice cream cone method and other two-component formulas including the ellipsoidal and Linskey's formulas allow for estimation of vestibular schwannoma volume more accurately than all one-component formulas.

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

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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.

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.

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.

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...

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...

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 ...

Hypofractionated stereotactic radiation therapy in three to five fractions for vestibular schwannoma

International Nuclear Information System (INIS)

Morimoto, Masahiro; Yoshioka, Yasuo; Kotsuma, Tadayuki

2013-01-01

The objective of this study was to retrospectively examine the outcomes of hypofractionated stereotactic radiation therapy in three to five fractions for vestibular schwannomas. Twenty-five patients with 26 vestibular schwannomas were treated with hypofractionated stereotactic radiation therapy using a CyberKnife. The vestibular schwannomas of 5 patients were associated with type II neurofibromatosis. The median follow-up time was 80 months (range: 6-167); the median planning target volume was 2.6 cm 3 (0.3-15.4); and the median prescribed dose (≥D90) was 21 Gy in three fractions (18-25 Gy in three to five fractions). Progression was defined as ≥2 mm 3-dimensional post-treatment tumor enlargement excluding transient expansion. Progression or any death was counted as an event in progression-free survival rates, whereas only progression was counted in progression-free rates. The 7-year progression-free survival and progression-free rates were 78 and 95%, respectively. Late adverse events (≥3 months) with grades based on Common Terminology Criteria for Adverse Events, v4.03 were observed in 6 patients: Grade 3 hydrocephalus in one patient, Grade 2 facial nerve disorders in two and Grade 1-2 tinnitus in three. In total, 12 out of 25 patients maintained pure tone averages ≤50 dB before hypofractionated stereotactic radiation therapy, and 6 of these 12 patients (50%) maintained pure tone averages at this level at the final audiometric follow-up after hypofractionated stereotactic radiation therapy. However, gradient deterioration of pure tone average was observed in 11 of these 12 patients. The mean pure tone averages before hypofractionated stereotactic radiation therapy and at the final follow-up for the aforementioned 12 patients were 29.8 and 57.1 dB, respectively. Treating vestibular schwannomas with hypofractionated stereotactic radiation therapy in three to five fractions may prevent tumor progression with tolerable toxicity. However, gradient

Hypofractionated stereotactic radiation therapy in three to five fractions for vestibular schwannoma

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Morimoto, Masahiro; Yoshioka, Yasuo [Osaka Univ., Graduate School of Medicine, Suita, Osaka (Japan); Kotsuma, Tadayuki [Kinki-chuo Chest Medical Center, Sakai, Osaka (Japan); others, and

2013-08-15

The objective of this study was to retrospectively examine the outcomes of hypofractionated stereotactic radiation therapy in three to five fractions for vestibular schwannomas. Twenty-five patients with 26 vestibular schwannomas were treated with hypofractionated stereotactic radiation therapy using a CyberKnife. The vestibular schwannomas of 5 patients were associated with type II neurofibromatosis. The median follow-up time was 80 months (range: 6-167); the median planning target volume was 2.6 cm{sup 3} (0.3-15.4); and the median prescribed dose ({>=}D90) was 21 Gy in three fractions (18-25 Gy in three to five fractions). Progression was defined as {>=}2 mm 3-dimensional post-treatment tumor enlargement excluding transient expansion. Progression or any death was counted as an event in progression-free survival rates, whereas only progression was counted in progression-free rates. The 7-year progression-free survival and progression-free rates were 78 and 95%, respectively. Late adverse events ({>=}3 months) with grades based on Common Terminology Criteria for Adverse Events, v4.03 were observed in 6 patients: Grade 3 hydrocephalus in one patient, Grade 2 facial nerve disorders in two and Grade 1-2 tinnitus in three. In total, 12 out of 25 patients maintained pure tone averages {<=}50 dB before hypofractionated stereotactic radiation therapy, and 6 of these 12 patients (50%) maintained pure tone averages at this level at the final audiometric follow-up after hypofractionated stereotactic radiation therapy. However, gradient deterioration of pure tone average was observed in 11 of these 12 patients. The mean pure tone averages before hypofractionated stereotactic radiation therapy and at the final follow-up for the aforementioned 12 patients were 29.8 and 57.1 dB, respectively. Treating vestibular schwannomas with hypofractionated stereotactic radiation therapy in three to five fractions may prevent tumor progression with tolerable toxicity. However, gradient

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.

Transoral Robotic Surgery in Retrostyloid Parapharyngeal Space Schwannomas

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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.

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

Solitary extramedullary plasmacytomas of thyroid in Hashimoto's thyroiditis: Mimicking benign cystic nodule on ultrasonography

Energy Technology Data Exchange (ETDEWEB)

Kwon, Yohan; Kim, Soo Jin; Hur, Joon Ho; Park, Sung Hee; Lee, Sun Jin; Lee, Tae Jin [Chung-Ang University Hospital, Chung-Ang University College of Medicine, Seoul (Korea, Republic of)

2013-11-15

Solitary extramedullary plasmacytoma (SEP) of the thyroid is uncommon and mostly occur in patients with a Hashimoto's thyroiditis (82%). We present a case on SEP of thyroid in Hashimoto's thyroiditis, which mimics growing benign cystic masses on serial ultrasonography.

Adrenal extramedullary hematopoiesis associated with β-thalassemia major.

Science.gov (United States)

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.

Paraspinal extramedullary hematopoiesis in patients with thalassemia intermedia.

Science.gov (United States)

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.

Peripheral Vestibular System Disease in Vestibular Schwannomas

DEFF Research Database (Denmark)

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

2015-01-01

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...

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.

Schwannoma of the Median Nerve at the Wrist and Palmar Regions of the Hand: A Rare Case Report

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Harun Kütahya

2013-01-01

Full Text Available Schwannomas are also known as neurolemmas that are usually originated from Schwann cells located in the peripheric nerve sheaths. They are the most common tumours of the hand (0.8–2%. They usually present solitary swelling along the course of the nerve however multiple lesions may be present in cases of NF type 1, familial neurofibromatosis, and sporadic schwannomatosis. Schwannomas are generally represented as an asymptomatic mass; however pain, numbness and fatigue may take place with the increasing size of the tumour. EMG (electromyelography, MRI (magnetic resonance imagination, and USG (ultrasound are helpful in the diagnosis. Surgical removal is usually curative. In this paper, we present a 24-year-old male referred to our clinic for a lump located at the volar side of the left wrist and a lump located in his left palm and numbness at his 3rd and 4th fingers. Total excision was performed for both lesions. Histopathological examination of the masses revealed typical features of schwannoma. At the 6th-month followup the patient was symptom-free except for slight paresthesia of the 3rd and the 4th fingers. For our knowledge, this is the second case in the literature presenting wrist and palm involvement of the median nerve schwannoma.

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.

Assessment of tumor blood flow and its correlation with histopathologic features in skull base meningiomas and schwannomas by using pseudo-continuous arterial spin labeling images

International Nuclear Information System (INIS)

Yamamoto, Tatsuya; Takeuchi, Hiroaki; Kinoshita, Kazuyuki; Kosaka, Nobuyuki; Kimura, Hirohiko

2014-01-01

Objective: We aimed to investigate whether pseudo-continuous arterial spin labeling (pcASL)-MRI can adequately evaluate tumor perfusion even if the tumors are located in the skull base region and evaluate the correlation between tumor blood flow (TBF) and the histopathologic features of skull base meningiomas and schwannomas. Materials and methods: We enrolled 31 patients with skull base meningioma (n = 14) and schwannoma (n = 17) who underwent surgical resection. TBF was calculated from pcASL. Tissue sections were stained with CD34 to evaluate microvessel area (MVA). TBF and MVA ratio were compared between meningiomas and schwannomas using Mann–Whitney U-test. The correlations between MVA ratio and TBF were evaluated in each tumor by using single linear regression analysis and Spearman's rank correlation coefficients (r s ). Results: MVA ratio and TBF were significantly higher in meningioma than in schwannoma (both p < 0.01). Correlation analyses revealed significant positive correlations between MVA ratio and both mean and max TBF for meningiomas (r s = 0.89, 0.81, both p < 0.01). There was a weak positive correlation between MVA ratio and mean TBF for schwannomas (r s = 0.43, p = 0.04). However, no significant correlation was found between MVA ratio and max TBF for schwannoma. Conclusions: pcASL-MRI is useful for evaluating tumor perfusion even if the tumors are located in the skull base region. Moreover, pcASL-TBF was significantly higher in most meningiomas compared to schwannomas, which can help in the differential diagnosis of the 2 tumor types even without the use of contrast material

Schwannoma intralaberíntico. Descripción de un caso

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José Antonio CÁMARA-ARNAZ

2016-03-01

Full Text Available Introducción y objetivos: El schwannoma intralaberíntico es un tumor benigno muy infrecuente, de lento crecimiento extradural, localizado primariamente en el laberinto membranoso. A través de un caso clínico presentamos las características de su evolución, intentamos ofrecer una explicación fisiopatológica de la sintomatología y de los hallazgos audio-vestibulares y describimos finalmente, las diferentes opciones terapéuticas. Descripción: Se trata de una mujer de 59 años, que comienza con síntomas inespecíficos del oído izquierdo a los que posteriormente se añade una pérdida auditiva. Al confirmar que se trata de una hipoacusia unilateral no justificada, solicitamos una RM que pone de manifiesto la presencia de un pequeño tumor de 2-3 mm intravestibular, al que revisaremos periódicamente. Discusión: El origen y la prevalencia del schwannoma intralaberíntico son desconocidos. Aunque la clínica se confunde con la de otros procesos otológicos más frecuentes, la realización de RM incrementaría el número de casos diagnosticados. En función del paciente, de su sintomatología, de la evolución y de las características tumorales, se decide el tipo de tratamiento Conclusión: El schwannoma intralaberíntico debe formar parte del diagnóstico diferencial en pacientes con síntomas audio-vestibulares unilaterales y solicitar una RM de alta resolución para obtener el diagnóstico definitivo. Presentando un pronóstico excelente, la observación es considerada como la mejor opción terapéutica.

Organising haematoma mimicking tumour on MRI following resection of acoustic neuroma

International Nuclear Information System (INIS)

Higgins, J.N.P.; Pigeon, C.N.; Moseley, I.F.

1995-01-01

We describe a 26-year-old woman in whom an enhancing, intradural extramedullary mass was found at the craniocervical junction on MRI 3 years after resection of a large acoustic neuroma. The radiological appearances suggested a new tumour, raising the possibility of neurofibromatosis 2, provoking a review of family members in an attempt to confirm the diagnosis, as well as further surgery. Histology of the lesion revealed an organising haematoma, with no evidence of malignancy. The imaging features can be explained by the process by which blood clot in the subarachnoid space is resorbed. Caution is advised in interpreting CT or MRI after neurosurgery. (orig.)

Collision tumor of the facial nerve: a synchronous seventh nerve schwannoma and neurofibroma.

Science.gov (United States)

Gross, Brian C; Carlson, Matthew L; Driscoll, Colin L; Moore, Eric J

2012-10-01

To report a novel case of a collision tumor involving an intraparotid neurofibroma and a mastoid segment facial nerve schwannoma. Clinical capsule report. Tertiary academic referral center. A 29-year-old woman with a 2-year history of an asymptomatic enlarging left infraauricular mass and normal FN function presented to a tertiary care referral center. Computed tomography and magnetic resonance imaging demonstrated a cystic lesion in the deep portion of the parotid gland extending into the stylomastoid foramen. The patient underwent superficial parotidectomy, and a cystic parotid mass was found to be intrinsic to the intraparotid facial nerve. A portion of the mass was biopsied, and intraoperative frozen section pathology was consistent with a neurofibroma. A mastoidectomy with FN decompression was then performed until a normal-appearing segment was identified just proximal to the second genu. After biopsy, proximal facial nerve stimulation failed to elicit evoked motor potentials, and en bloc resection was performed. Final pathology demonstrated a schwannoma involving the mastoid segment and a neurofibroma involving the proximal intraparotid facial nerve. We report the first case of a facial nerve collision tumor involving an intraparotid neurofibroma and a mastoid segment facial nerve schwannoma. Benign FN sheath tumors of the parotid gland are rare but should be considered in the differential diagnosis of a parotid mass.

Extramedullary plasmocytoma of the rhino pharynx associated to intrathoracic mass - a case report

International Nuclear Information System (INIS)

Souza, Andrea Silveira de; Fernandes, Artur da Rocha Correa; Yamashita, Helio Kiitiro

1996-01-01

The authors report a case of a 56-year-old while male, who had occipital headache, libido and visual acuity (bitemporal field) reduction as the clinical picture, with progressive evolution within a four years period. The image diagnosis methods (roentgenologic studies, (MRI) demonstrated extensive mass involving the sella, clivus and sphenoid sinus, and an intrathoracic mass on the right. The final diagnosis was extramedullary plasmocytoma with rhinopharynx origin. (author)

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...

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...

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.)

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.

Schwannoma do forame magno: revisão e relato de caso Foramen magnum schwannoma: review of the literature and report of a case

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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

Cost-effectiveness analysis of a non-contrast screening MRI protocol for vestibular schwannoma in patients with asymmetric sensorineural hearing loss

International Nuclear Information System (INIS)

Crowson, Matthew G.; Rocke, Daniel J.; Kaylie, David M.; Hoang, Jenny K.; Weissman, Jane L.

2017-01-01

We aimed to determine if a non-contrast screening MRI is cost-effective compared to a full MRI protocol with contrast for the evaluation of vestibular schwannomas. A decision tree was constructed to evaluate full MRI and screening MRI strategies for patients with asymmetric sensorineural hearing loss. If a patient were to have a positive screening MRI, s/he received a full MRI. Vestibular schwannoma prevalence, MRI specificity and sensitivity, and gadolinium anaphylaxis incidence were obtained through literature review. Institutional charge data were obtained using representative patient cohorts. One-way and probabilistic sensitivity analyses were completed to determine CE model threshold points for MRI performance characteristics and charges. The mean charge for a full MRI with contrast was significantly higher than a screening MRI ($4089 ± 1086 versus $2872 ± 741; p < 0.05). The screening MRI protocol was more cost-effective than a full MRI protocol with a willingness-to-pay from $0 to 20,000 USD. Sensitivity analyses determined that the screening protocol dominated when the screening MRI charge was less than $4678, and the imaging specificity exceeded 78.2%. The screening MRI protocol also dominated when vestibular schwannoma prevalence was varied between 0 and 1000 in 10,000 people. A screening MRI protocol is more cost-effective than a full MRI with contrast in the diagnostic evaluation of a vestibular schwannoma. A screening MRI likely also confers benefits of shorter exam time and no contrast use. Further investigation is needed to confirm the relative performance of screening protocols for vestibular schwannomas. (orig.)

Cost-effectiveness analysis of a non-contrast screening MRI protocol for vestibular schwannoma in patients with asymmetric sensorineural hearing loss

Energy Technology Data Exchange (ETDEWEB)

Crowson, Matthew G.; Rocke, Daniel J.; Kaylie, David M. [Duke University Medical Center, Division of Otolaryngology-Head and Neck Surgery, Durham, NC (United States); Hoang, Jenny K. [Duke University Medical Center, Department of Radiology, Durham, NC (United States); Weissman, Jane L. [Oregon Health Sciences University, Professor Emerita of Diagnostic Radiology, Portland, OR (United States)

2017-08-15

We aimed to determine if a non-contrast screening MRI is cost-effective compared to a full MRI protocol with contrast for the evaluation of vestibular schwannomas. A decision tree was constructed to evaluate full MRI and screening MRI strategies for patients with asymmetric sensorineural hearing loss. If a patient were to have a positive screening MRI, s/he received a full MRI. Vestibular schwannoma prevalence, MRI specificity and sensitivity, and gadolinium anaphylaxis incidence were obtained through literature review. Institutional charge data were obtained using representative patient cohorts. One-way and probabilistic sensitivity analyses were completed to determine CE model threshold points for MRI performance characteristics and charges. The mean charge for a full MRI with contrast was significantly higher than a screening MRI ($4089 ± 1086 versus $2872 ± 741; p < 0.05). The screening MRI protocol was more cost-effective than a full MRI protocol with a willingness-to-pay from $0 to 20,000 USD. Sensitivity analyses determined that the screening protocol dominated when the screening MRI charge was less than $4678, and the imaging specificity exceeded 78.2%. The screening MRI protocol also dominated when vestibular schwannoma prevalence was varied between 0 and 1000 in 10,000 people. A screening MRI protocol is more cost-effective than a full MRI with contrast in the diagnostic evaluation of a vestibular schwannoma. A screening MRI likely also confers benefits of shorter exam time and no contrast use. Further investigation is needed to confirm the relative performance of screening protocols for vestibular schwannomas. (orig.)

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

International Nuclear Information System (INIS)

Pistevou-Gompaki, K.; Paraskevopoulos, P.; Kotsa, K.; Skaragas, G.; Repanta, E.

1996-01-01

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)

Giant mediastinal schwannoma located in the lower right side of the ...

African Journals Online (AJOL)

2016-01-18

Jan 18, 2016 ... nerve. The tumor was coated with a white envelope and filled. Giant mediastinal schwannoma located in the lower right side of the chest. Y Wu, J Zhang, Y Chai. Department of Thoracic Surgery, School of Medicine, Second Affiliated Hospital, Zhejiang University, Hangzhou, China. Access this article online.

Immunohistochemical profile of cytokines and growth factors expressed in vestibular schwannoma and in normal vestibular nerve tissue.

Science.gov (United States)

Taurone, Samanta; Bianchi, Enrica; Attanasio, Giuseppe; Di Gioia, Cira; Ierinó, Rocco; Carubbi, Cecilia; Galli, Daniela; Pastore, Francesco Saverio; Giangaspero, Felice; Filipo, Roberto; Zanza, Christian; Artico, Marco

2015-07-01

Vestibular schwannomas, also known as acoustic neuromas, are benign tumors, which originate from myelin-forming Schwann cells. They develop in the vestibular branch of the eighth cranial nerve in the internal auditory canal or cerebellopontine angle. The clinical progression of the condition involves slow and progressive growth, eventually resulting in brainstem compression. The objective of the present study was to investigate the expression level and the localization of the pro-inflammatory cytokines, transforming growth factor-β1 (TGF-β1) interleukin (IL)-1β, IL-6 and tumor necrosis factor-α (TNF-α), as well as the adhesion molecules, intracellular adhesion molecule-1 and vascular endothelial growth factor (VEGF), in order to determine whether these factors are involved in the transformation and development of human vestibular schwannoma. The present study investigated whether changes in inflammation are involved in tumor growth and if so, the mechanisms underlying this process. The results of the current study demonstrated that pro-inflammatory cytokines, including TGF-β1, IL-1β and IL-6 exhibited increased expression in human vestibular schwannoma tissue compared with normal vestibular nerve samples. TNF-α was weakly expressed in Schwann cells, confirming that a lower level of this cytokine is involved in the proliferation of Schwann cells. Neoplastic Schwann cells produce pro-inflammatory cytokines that may act in an autocrine manner, stimulating cellular proliferation. In addition, the increased expression of VEGF in vestibular schwannoma compared with that in normal vestibular nerve tissue, suggests that this factor may induce neoplastic growth via the promotion of angiogenesis. The present findings suggest that inflammation may promote angiogenesis and consequently contribute to tumor progression. In conclusion, the results of the present study indicated that VEGF and pro-inflammatory cytokines may be potential therapeutic targets in vestibular

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.

Prognostic significance of electrophysiological tests for facial nerve outcome in vestibular schwannoma surgery.

Science.gov (United States)

van Dinther, J J S; Van Rompaey, V; Somers, T; Zarowski, A; Offeciers, F E

2011-01-01

To assess the prognostic significance of pre-operative electrophysiological tests for facial nerve outcome in vestibular schwannoma surgery. Retrospective study design in a tertiary referral neurology unit. We studied a total of 123 patients with unilateral vestibular schwannoma who underwent microsurgical removal of the lesion. Nine patients were excluded because they had clinically abnormal pre-operative facial function. Pre-operative electrophysiological facial nerve function testing (EPhT) was performed. Short-term (1 month) and long-term (1 year) post-operative clinical facial nerve function were assessed. When pre-operative facial nerve function, evaluated by EPhT, was normal, the outcome from clinical follow-up at 1-month post-operatively was excellent in 78% (i.e. HB I-II) of patients, moderate in 11% (i.e. HB III-IV), and bad in 11% (i.e. HB V-VI). After 1 year, 86% had excellent outcomes, 13% had moderate outcomes, and 1% had bad outcomes. Of all patients with normal clinical facial nerve function, 22% had an abnormal EPhT result and 78% had a normal result. No statistically significant differences could be observed in short-term and long-term post-operative facial function between the groups. In this study, electrophysiological tests were not able to predict facial nerve outcome after vestibular schwannoma surgery. Tumour size remains the best pre-operative prognostic indicator of facial nerve function outcome, i.e. a better outcome in smaller lesions.

Change in hearing during 'wait and scan' management of patients with vestibular schwannoma

DEFF Research Database (Denmark)

Stangerup, Sven-Eric; Caye-Thomasen, P.; Tos, M.

2008-01-01

: At the time of diagnosis, 334 patients (53 per cent) had good hearing and speech discrimination of better than 70 per cent; at the end of the 10-year observation period, this latter percentage was 31 per cent. In 17 per cent of the patients, speech discrimination at diagnosis was 100 per cent; of these, 88......Aim: To evaluate hearing changes during 'wait and scan' management of patients with vestibular schwannoma. Subjects: Over a 10-year period, 636 patients have prospectively been allocated to 'wait and scan' management, with annual magnetic resonance scanning and audiological examination. Results...... surgery and of radiation therapy with those of 'wait and scan' management, it appears that, in vestibular schwannoma patients with a small tumour and normal speech discrimination, the main indication for active treatment should be established tumour growth Udgivelsesdato: 2008/7...

Daunorubicin, Cytarabine, and Cladribine Regimen Plus Radiotherapy and Donor Lymphocyte Infusion for Extramedullary Relapse of Acute Myeloid Leukemia after Hematopoietic Stem Cell Transplantation

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Marco Sanna

2013-01-01

Full Text Available Myeloid sarcoma is a rare tumor consisting of myeloid blasts that involve anatomic sites outside the bone marrow. Fatal prognosis is inevitable in patients with extramedullary relapse after hematopoietic stem cell transplantation (HSCT, and no standard treatments are available yet. We report the first case of extramedullary relapse after HSCT treated with a combination of daunorubicin, cytarabine, and cladribine (DAC regimen plus radiotherapy and donor lymphocyte infusion (DLI. This treatment induced a new and durable remission in our patient. The favorable toxicity profile and the reduced cost make this combination worthy of further investigations.

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.

Risk factors associated with vestibulocochlear nerve schwannoma: systematic review Fatores de risco associados ao Schwannoma do nervo vestibular: revisão sistemática

Directory of Open Access Journals (Sweden)

Ana Paula Corona

2009-08-01

Full Text Available The vestibulocochlear nerve schwannoma (VS is a benign tumor that stems from the edge of the Schwann´s sheath. It is considered the most frequent intracranial benign tumor, of low lethality rate and unknown etiology. AIM: to identify risk factors associated with VS. STUDY DESIGN: systematic review. METHODS: electronic search of studies using the following key words: "risk", "schwannoma", "vestibular", "neuroma" and "acoustic". All original articles on epidemiological studies published in Portuguese, English or Spanish describing measures of association were included. RESULTS: twenty case-control studies were found, most of them published in the United States. The analysis of those studies shows educational level, household income, occupation, exposure to ionizing radiation and noise, allergic diseases as well as the use of both cellular and cordless phones as risk factors for the VS. CONCLUSION: methodological limitations and lack of precision in the findings impose limits to definitive conclusions concerning those risk factors. The current study contributes with information which can subsidize decisions related to the methodology to be used, having in mind new investigations on risk factors for VS. Therefore, it is of great help for knowledge improvement in this field.O Schwannoma do nervo vestibular (SV é um tumor benigno que se origina da bainha de Schwann do VIII par craniano. É o tumor benigno intracraniano mais frequente, de baixa letalidade e etiologia obscura. OBJETIVO: Identificar fatores de risco associados ao SV. DESENHO DO ESTUDO: Revisão sistemática. MATERIAL E MÉTODO: Identificação de estudos em bases de dados eletrônicos utilizando as palavras-chaves "risk", "risco", "schwannoma", "vestibular", "neuroma" e "acoustic". Incluíram-se artigos originais de pesquisa epidemiológica publicados em português, espanhol ou inglês, que referiam alguma medida de associação. Foram comparados e analisados aspectos metodológicos e

Extramedullary spinal teratoma presenting with recurrent aseptic meningitis.

Science.gov (United States)

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.

A Case of Action-Induced Clonus that Mimicked Action Tremors and was Associated with Cervical Schwannoma

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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.

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...

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...

Linear accelerator stereotactic radiosurgery for vestibular schwannomas: a UK series.

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Benghiat, H; Heyes, G; Nightingale, P; Hartley, A; Tiffany, M; Spooner, D; Geh, J I; Cruickshank, G; Irving, R M; Sanghera, P

2014-06-01

To evaluate non-auditory toxicity and local control after linear accelerator stereotactic radiosurgery (SRS) for the treatment of vestibular schwannomas. The institutional policy was to use SRS for radiologically progressing vestibular schwannomas. Case notes and plans were retrospectively reviewed for all patients undergoing SRS for vestibular schwannomas between September 2002 and June 2012. All patients were surgically immobilised using a BrainLab stereotactic head frame. The treatment plan was generated using BrainLab software (BrainScan 5.03). The aim was to deliver 12 Gy to the surface of the target with no margin. Patients with a minimum of 12 months of follow-up were included for toxicity and local control assessment. Radiological progression was defined as growth on imaging beyond 2 years of follow-up. Overall local control was defined in line with other series as absence of surgical salvage. Ninety-nine patients were identified. Two patients were lost to follow-up. After a median follow-up interval of 2.4 years, the actuarial radiological progression-free survival at 3 years was 100% and overall local control was also 100%. However, two patients progressed radiologically at 3.3 and 4.5 years, respectively. Twenty-one of 97 (22%) evaluable patients suffered trigeminal toxicity and this was persistent in 8/97 (8%). Two of 97 (2%) suffered long-term facial nerve toxicity (one with associated radiological progression causing hemi-facial spasm alone). One of 97 (1%) required intervention for obstructive hydrocephalus. No statistically significant dosimetric relationship could be shown to cause trigeminal or facial nerve toxicity. However, 7/8 patients with persistent trigeminal nerve toxicity had tumours in contact with the trigeminal nerve. SRS delivering 12 Gy using a linear accelerator leads to high local control rates, but only prospective evaluation will fully establish short-term toxicity. In this study, persistent trigeminal toxicity occurred almost

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

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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.)

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.)

Intracranial extramedullary hematopoiesis in patients with thalassemia: a case report and review of the literature.

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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.

Quality of life in patients after vestibular Schwannoma surgery

OpenAIRE

Hajná, Barbora

2011-01-01

TVestibular schwannoma is a benign tumor that arises from the Schwann cells of the vestibular nerve. Unilateral hearing loss, tinnitus, facial and trigeminal dysfunction and vertigo are the most common symptoms. Surgical removal of the tumor is one of the treatment modalities of this disease. Surgical excision usually involves the complete vestibular nerve resection and there is also a risk of cochlear and facial nerve lesion. This thesis deals with changes in quality of life in patients afte...

Communicating Hydrocephalus Associated with Small- to Medium-Sized Vestibular Schwannomas: Clinical Significance of the Tumor Apparent Diffusion Coefficient Map.

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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.

Hematopoietic stem cell transplantation for isolated extramedullary relapse of acute lymphoblastic leukemia in children.

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Gabelli, Maria; Zecca, Marco; Messina, Chiara; Carraro, Elisa; Buldini, Barbara; Rovelli, Attilio Maria; Fagioli, Franca; Bertaina, Alice; Lanino, Edoardo; Favre, Claudio; Rabusin, Marco; Prete, Arcangelo; Ripaldi, Mimmo; Barberi, Walter; Porta, Fulvio; Caniglia, Maurizio; Santarone, Stella; D'Angelo, Paolo; Basso, Giuseppe; Locatelli, Franco

2018-06-13

Relapse of acute lymphoblastic leukemia (ALL) may occur in extramedullary sites, mainly central nervous system (CNS) and testis. Optimal post-remissional treatment for isolated extramedullary relapse (IEMR) is still controversial. We collected data of children treated with hematopoietic stem cell transplantation (HSCT) for ALL IEMR from 1990 to 2015 in Italy. Among 281 patients, 167 had a relapse confined to CNS, 73 to testis, 14 to mediastinum, and 27 to other organs. Ninety-seven patients underwent autologous HSCT, 79 received allogeneic HSCT from a matched family donor, 75 from a matched unrelated donor, and 30 from an HLA-haploidentical donor. The 10-year overall survival was 56% and was not influenced by gender, ALL blast immune-phenotype, age, site of relapse, duration of first remission, and type of HSCT. In multivariable analysis, the only prognostic factors were disease status at HSCT and year of transplantation. Patients transplanted in third or subsequent complete remission (CR) had a risk of death 2.3 times greater than those in CR2. Children treated after 2000 had half the risk of death than those treated before that year. Our results suggest that both autologous and allogeneic HSCT may be considered for the treatment of pediatric ALL IEMR after the achievement of CR2.

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

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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.

Repeat Gamma Knife surgery for vestibular schwannomas

Science.gov (United States)

Lonneville, Sarah; Delbrouck, Carine; Renier, Cécile; Devriendt, Daniel; Massager, Nicolas

2015-01-01

Background: Gamma Knife (GK) surgery is a recognized treatment option for the management of small to medium-sized vestibular schwannoma (VS) associated with high-tumor control and low morbidity. When a radiosurgical treatment fails to stop tumor growth, repeat GK surgery can be proposed in selected cases. Methods: A series of 27 GK retreatments was performed in 25 patients with VS; 2 patients underwent three procedures. The median time interval between GK treatments was 45 months. The median margin dose used for the first, second, and third GK treatments was 12 Gy, 12 Gy, and 14 Gy, respectively. Six patients (4 patients for the second irradiation and 2 patients for the third irradiation) with partial tumor regrowth were treated only on the growing part of the tumor using a median margin dose of 13 Gy. The median tumor volume was 0.9, 2.3, and 0.7 cc for the first, second, and third treatments, respectively. Stereotactic positron emission tomography (PET) guidance was used for dose planning in 6 cases. Results: Mean follow-up duration was 46 months (range 24–110). At the last follow-up, 85% of schwannomas were controlled. The tumor volume decreased, remained unchanged, or increased after retreatment in 15, 8, and 4 cases, respectively. Four patients had PET during follow-up, and all showed a significant metabolic decrease of the tumor. Hearing was not preserved after retreatment in any patients. New facial or trigeminal palsy did not occur after retreatment. Conclusions: Our results support the long-term efficacy and low morbidity of repeat GK treatment for selected patients with tumor growth after initial treatment. PMID:26500799

Upper cervical bronchogenic cyst: A rare condition at a rare location

Directory of Open Access Journals (Sweden)

Ranjeet Kumar Jha

2018-01-01

Full Text Available Intraspinal bronchogenic cyst (SBC is a rare but important cause of spinal cord compression, commonly seen in the cervicothoracic spine. We report a case of a 43-year-old male, presenting with complaints of neck pain, radiating to right shoulder, with numbness of right hand and fingers. Magnetic resonance imaging of the cervical spine revealed an intradural extramedullary, ventral cystic lesion extending from C2 to C4 vertebral levels. Complete surgical excision was performed, and the patient had a complete relief of symptoms postoperatively. Only 11 cases of SBCs have been reported in literature. We discuss the peculiar location of this lesion, possible embryological reasons and the overall surgical outcome of SBC.

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.

Dose verification to cochlea during gamma knife radiosurgery of acoustic schwannoma using MOSFET dosimeter.

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Sharma, Sunil D; Kumar, Rajesh; Akhilesh, Philomina; Pendse, Anil M; Deshpande, Sudesh; Misra, Basant K

2012-01-01

Dose verification to cochlea using metal oxide semiconductor field effect transistor (MOSFET) dosimeter using a specially designed multi slice head and neck phantom during the treatment of acoustic schwannoma by Gamma Knife radiosurgery unit. A multi slice polystyrene head phantom was designed and fabricated for measurement of dose to cochlea during the treatment of the acoustic schwannoma. The phantom has provision to position the MOSFET dosimeters at the desired location precisely. MOSFET dosimeters of 0.2 mm x 0.2 mm x 0.5 μm were used to measure the dose to the cochlea. CT scans of the phantom with MOSFETs in situ were taken along with Leksell frame. The treatment plans of five patients treated earlier for acoustic schwannoma were transferred to the phantom. Dose and coordinates of maximum dose point inside the cochlea were derived. The phantom along with the MOSFET dosimeters was irradiated to deliver the planned treatment and dose received by cochlea were measured. The treatment planning system (TPS) estimated and measured dose to the cochlea were in the range of 7.4 - 8.4 Gy and 7.1 - 8 Gy, respectively. The maximum variation between TPS calculated and measured dose to cochlea was 5%. The measured dose values were found in good agreement with the dose values calculated using the TPS. The MOSFET dosimeter can be a suitable choice for routine dose verification in the Gamma Knife radiosurgery.

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

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.)

A Patient-Assessed Morbidity to Evaluate Outcome in Surgically Treated Vestibular Schwannomas.

Science.gov (United States)

Al-Shudifat, Abdul Rahman; Kahlon, Babar; Höglund, Peter; Lindberg, Sven; Magnusson, Måns; Siesjo, Peter

2016-10-01

Outcome after treatment of vestibular schwannomas can be evaluated by health providers as mortality, recurrence, performance, and morbidity. Because mortality and recurrence are rare events, evaluation has to focus on performance and morbidity. The latter has mostly been reported by health providers. In the present study, we validate 2 new scales for patient-assessed performance and morbidity in comparison with different outcome tools, such as quality of life (QOL) (European Quality of Life-5 dimensions [EQ-5D]), facial nerve score, and work capacity. There were 167 total patients in a retrospective (n = 90) and prospective (n = 50) cohort of surgically treated vestibular schwannomas. A new patient-assessed morbidity score (paMS), a patient-assessed Karnofsky score (paKPS), the patient-assessed QOL (EQ-5D) score, work capacity, and the House-Brackmann facial nerve score were used as outcome measures. Analysis of paMS components and their relation to other outcomes was done as uni- and multivariate analysis. All outcome instruments, except EQ-5D and paKPS, showed a significant decrease postoperatively. Only the facial nerve score (House-Brackmann facial nerve score) differed significantly between the retrospective and prospective cohorts. Out of the 16 components of the paMS, hearing dysfunction, tear dysfunction, balance dysfunction, and eye irritation were most often reported. Both paMS and EQ-5D correlated significantly with work capacity. Standard QOL and performance instruments may not be sufficiently sensitive or specific to measure outcome at the cohort level after surgical treatment of vestibular schwannomas. A morbidity score may yield more detailed information on symptoms that can be relevant for rehabilitation and occupational training after surgery. Copyright © 2016 Elsevier Inc. All rights reserved.

Stereotactic Radiosurgery versus Natural History in Patients with Growing Vestibular Schwannomas.

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Tu, Albert; Gooderham, Peter; Mick, Paul; Westerberg, Brian; Toyota, Brian; Akagami, Ryojo

2015-08-01

Objective To describe our experience with stereotactic radiosurgery and its efficacy on growing tumors, and then to compare this result with the natural history of a similar cohort of non-radiation-treated lesions. Study Design A retrospective chart review and cohort comparison. Methods The long-term control rates of patients having undergone radiosurgery were collected and calculated, and this population was then compared with a group of untreated patients from the same period of time with growing lesions. Results A total of 61 patients with growing vestibular schwannomas treated with radiosurgery were included. After a mean of 160 months, we observed a control rate of 85.2%. When compared with a group of 36 patients with growing tumors who were yet to receive treatment (previously published), we found a corrected control rate or relative risk reduction of only 76.8%. Conclusion Radiosurgery for growing vestibular schwannomas is less effective than previously reported in unselected series. Although radiosurgery still has a role in managing this disease, consideration should be given to the actual efficacy that may be calculated when the natural history is known. We hope other centers will similarly report their experience on this cohort of patients.

Intra-extramedullary drainage as an effective option for treatment of intramedullary ependymal cyst of thoracic spine: technical note.

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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.

Foramen magnum schwannoma: review of the literature and report of a case

International Nuclear Information System (INIS)

Nacif, Marcelo Souto; Caiado, Stella; Oliveira, Nidia di Paula Silva; Paula Neto, Walter Teixeira de; Campos, Flavio do Amaral; Santos, Alair Augusto Sarmet Moreira Damas dos

2001-01-01

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)

Surgical management of vestibular schwannoma: attempted preservation of hearing and facial function.

Science.gov (United States)

Youssef, T F; Matter, A; Ahmed, M R

2013-05-01

Vestibular schwannomas are benign tumours which usually originate from the vestibular portion of the VIIIth cranial nerve. Treatment options include observation with serial imaging, stereotactic radiation and microsurgical removal. The goal of surgery was complete eradication of tumour with preservation of hearing and facial nerve function. A retrospective review was undertaken of 24 cases of vestibular schwannoma jointly operated upon by a team of neurosurgeons and otologists at the Suez Canal University Hospital, with assessment of VIIth and VIIIth cranial nerve function, tumour size, and extent of growth. All surgery utilised a retromastoid, suboccipital approach. Complete tumour removal was achieved in 19 patients. Anatomical preservation of the facial nerve was possible in 66.6 per cent of patients. Pre-operative, useful hearing was present in four patients, and preserved in 80 per cent. Cerebrospinal fluid leakage was diagnosed in two (8.3 per cent) patients, who responded to conservative therapy. The retromastoid, suboccipital surgical approach to the skull base can be safely and successfully achieved using a microsurgical technique, with minimal or no damage to neurovascular structures, even for large tumours.

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

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.

Spinal cord compression secondary to extramedullary hematopoiesis: A rareness in a young adult with thalassemia major.

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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.

Incidence, clinical features, laboratory findings and outcome of patients with multiple myeloma presenting with extramedullary relapse.

Science.gov (United States)

Papanikolaou, Xenofon; Repousis, Panagiotis; Tzenou, Tatiana; Maltezas, Dimitris; Kotsopoulou, Maria; Megalakaki, Katerina; Angelopoulou, Maria; Dimitrakoloulou, Elektra; Koulieris, Efstathios; Bartzis, Vassiliki; Pangalis, Gerasimos; Panayotidis, Panagiotis; Kyrtsonis, Marie-Christine

2013-07-01

Extramedullary plasmacytomas constitute a rare and not well studied subset of multiple myeloma (MM) relapses. We report the incidence, clinical-laboratory features and outcome of patients with MM and extramedullary relapse (ExMeR). A total of 303 patients with symptomatic MM were recorded in a 13-year period in two institutions. Twenty-eight cases of ExMeR (9%) were recorded. There was an increased frequency of elevated lactate dehydrogenase (LDH) (p = 0.026), bone plasmacytomas (p = 0.001) and fractures (p = 0.002) at diagnosis, in patients with ExMeR compared to the others. ExMeR was associated with an ominous outcome, high LDH, constitutional symptoms and a statistically significant decrease of monoclonal paraprotein compared to levels at diagnosis (p = 0.009). Prior treatment with bortezomib was associated with a decreased hazard of ExMeR (p = 0.041). Overall survival (OS) was decreased in patients with ExMeR compared to the others (38 vs. 59 months, p = 0.006). Patients with MM with ExMeR have a lower OS and their clinical and laboratory features differ from those without.

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.

Predictors of vertigo in patients with untreated vestibular schwannoma.

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Andersen, Jan Fredrik; Nilsen, Kathrin Skorpa; Vassbotn, Flemming Slinning; Møller, Per; Myrseth, Erling; Lund-Johansen, Morten; Goplen, Frederik Kragerud

2015-04-01

Previous studies have shown that vertigo is the most powerful negative predictor of quality of life in patients with vestibular schwannomas, but the variability in vertigo symptom severity is still poorly understood. We wanted to find out whether vertigo could be related to objective parameters such as tumor size, location, vestibular nerve function, hearing, and postural stability in patients with untreated vestibular schwannomas. Baseline data from prospective cohort study. Tertiary referral center. Four hundred thirty-four consecutive patients with unilateral VS diagnosed on MRI. Mean age 56 years (range 16-84 yr). Fifty-three percent women. Diagnostic, with a medical history, otolaryngological examination, pure-tone and speech audiometry, MRI, posturography, and videonystagmography with bithermal caloric tests. Dizziness measured on a 100-mm visual analog scale (VAS). Secondary outcome measures were canal paresis and postural imbalance (static and dynamic posturography). Three hundred three patients (70%) completed the VAS. Severe dizziness, defined as VAS 75 or greater, was reported by 9% of the patients. Larger tumors were associated with higher risk of postural instability and canal paresis. Moderate to severe dizziness was associated with postural imbalance and canal paresis, and possibly with small to medium-sized tumors. Postural instability was related to tumor size and canal paresis when measured by dynamic, but not with static, posturography. A minority of VS patients experience severe vestibular symptoms related to canal paresis and postural instability. A curvilinear relationship is hypothesized between tumor size and dizziness.

Prevalence of hydrocephalus in 157 patients with vestibular schwannoma

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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.)

Detection of Spontaneous Schwannomas by MRI in a Transgenic Murine Model of Neurofibromatosis Type 2

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S.M. Messerli

2002-01-01

Full Text Available Spontaneous schwannomas were detected by magnetic resonance imaging (MRI in a transgenic murine model of neurofibromatosis type 2 (NF2 expressing a dominant mutant form of merlin under the Schwann cell-specific PO promoter. Approximately 85% of the investigated mice showed putative tumors by 24 months of age. Specifically, 21% of the mice showed tumors in the intercostal muscles, 14% in the limb muscles, 7% in the spinal cord and spinal ganglia, 7% in the external ear, 14% in the muscle of the abdominal region, and 7% in the intestine; 66% of the female mice had uterine tumors. Multiple tumors were detected by MRI in 21% of mice. The tumors were isointense with muscle by T1-weighted MRI, showed strong enhancement following administration of gadolinium-DTPA, and were markedly hyperintense by T2-weighted MRI, all hallmarks of the clinical manifestation. Hematoxylin and eosin staining and immunohistochemistry indicated that the tumors consisted of schwannomas and Schwann cell hyperplasias. The lesions stained positively for S-100 protein and a marker antigen for the mutated transgenic NF2 protein, confirming that the imaged tumors and areas of hyperplasia were of Schwann cell origin and expressed the mutated NF2 protein. Tumors were highly infectable with a recombinant herpes simplex virus type 1 vector, hrR3, which contains the reporter gene, lacZ. The ability to develop schwannoma growth with a noninvasive imaging technique will allow assessment of therapeutic interventions.

Intradural Solitary Fibrous Tumor of the Lumbar Spine: A Distinctive Case Report

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Recep Basaran

2015-01-01

Full Text Available Background. Solitary fibrous tumors are ubiquitous mesenchymal neoplasms of putative fibroblastic origin. They were originally described in the pleura but subsequently have been reported in many extraserosal sites. Solitary fibrous tumors may also occur in the meninges, central nervous system parenchyma, and spinal cord. Case. A 67-year-old male patient with progressive lower extremity weakness, urinary urgency, and sexual dysfunction has been admitted to our hospital. On his lumbar MRI, we detected an intradural lesion posterior to the L3 vertebral corpus. We resected the lesion by L3 total laminectomy. Immunohistological findings revealed strong and diffuse immunopositivity with vimentin, CD34, and bcl-2. Ki-67 proliferation index was 5–8%. We did not detect any recurrence 12 months after his operation. Conclusion. SFT is mostly seen in young and middle-aged patients and should be considered among differential diagnosis in cases suffering from pain, hypoesthesia, and urinary dysfunction. Gross total resection should be primary treatment. Tumors that have high Ki-67 labeling should be followed up for potential recurrences.

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.

Linear accelerator-based stereotactic radiosurgery for bilateral vestibular schwannomas in patients with neurofibromatosis type 2

NARCIS (Netherlands)

Meijer, Otto W. M.; Vandertop, W. Peter; Lagerwaard, Frank J.; Slotman, Ben J.

2008-01-01

OBJECTIVE: Patients with neurofibromatosis Type 2 (NF2) patients typically have bilateral vestibular schwannomas (VS) and are at risk for developing bilateral deafness, bilateral trigeminal, and bilateral facial nerve function loss. Previous reports suggested that treatment outcomes in these

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.

Single-fraction vs. fractionated linac-based stereotactic radiosurgery for vestibular schwannoma: a single-institution study

NARCIS (Netherlands)

Meijer, O. W. M.; Vandertop, W. P.; Baayen, J. C.; Slotman, B. J.

2003-01-01

PURPOSE: In this single-institution trial, we investigated whether fractionated stereotactic radiation therapy is superior to single-fraction linac-based radiosurgery with respect to treatment-related toxicity and local control in patients with vestibular schwannoma. METHODS AND MATERIALS: All 129

Microvascular Decompression for Treatment of Trigeminal Neuralgia in Patient with Facial Nerve Schwannoma.

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Marinelli, John P; Van Gompel, Jamie J; Link, Michael J; Carlson, Matthew L

2018-05-01

Secondary trigeminal neuralgia (TN) is uncommon. When a space-occupying lesion with mass effect is identified, the associated TN is often exclusively attributed to the tumor. This report illustrates the importance of considering coexistent actionable pathology when surgically treating secondary TN. A 51-year-old woman presented with abrupt-onset TN of the V2 and V3 nerve divisions with hypesthesia. She denied changes in hearing, balance, or facial nerve dysfunction. Magnetic resonance imaging revealed a 1.6-cm contrast-enhancing cerebellopontine angle tumor that effaced the trigeminal nerve, consistent with a vestibular schwannoma. In addition, a branch of the superior cerebellar artery abutted the cisternal segment of the trigeminal nerve on T2-weighted thin-slice magnetic resonance imaging. Intraoperative electrical stimulation of the tumor elicited a response from the facial nerve at low threshold over the entire accessible tumor surface, indicating that the tumor was a facial nerve schwannoma. Considering the patient's lack of facial nerve deficit and that the tumor exhibited no safe entry point for intracapsular debulking, tumor resection was not performed. Working between the tumor and tentorium, a branch of the superior cerebellar artery was identified and decompressed with a Teflon pad. At last follow-up, the patient exhibited resolution of her TN. Her hearing and facial nerve function remained intact. Despite obstruction from a medium-sized tumor, it is still possible to achieve microvascular decompression of the fifth cranial nerve. This emphasizes the importance of considering other actionable pathology during surgical management of presumed tumor-induced TN. Further, TN is relatively uncommon with medium-sized vestibular schwannomas and coexistent causes should be considered. Copyright © 2018 Elsevier Inc. All rights reserved.

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

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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.

Von Reckling-hausen disease associated to thyroid carcinoma and malignant schwannoma of the chest wall. A case

International Nuclear Information System (INIS)

Diaz P, J.; Tantalean, E.; Guzman, R.; Pomatanta P, J.; Grados M, J.; Vilela, C.

1999-01-01

The multiple neurofibromatosis is an autosomal dominant hereditary disease associated to malignant schwannoma in about 3% of the cases and very rarely to others cancers. The study provides information on the case of a 32 year-old woman who suffers from this disease and presented two synchronous cancers: a papillary carcinoma of thyroid and a malignant schwannoma of the chest wall. The thyroid tumour was managed with hemithyroidectomy, hormonotherapy and radiotherapy, and the lesion of the thoracic wall was treated with local radical resection application of Marlex mesh and rotation of a musculocutaneous flap of the dorsal muscle. A review of the literature on the clinical aspects of this association and the surgical techniques employed to cover the defect of the chest wall is presented. (authors)

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.

Fractionated Stereotactic Radiotherapy of Vestibular Schwannomas Accelerates Hearing Loss

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Rasmussen, Rune, E-mail: rune333@gmail.com [Department of Neurosurgery, Rigshospitalet, Copenhagen (Denmark); Claesson, Magnus [Department of Neurosurgery, Rigshospitalet, Copenhagen (Denmark); Stangerup, Sven-Eric [Ear, Nose, and Throat Department, Rigshospitalet, Copenhagen (Denmark); Roed, Henrik [Department of Radiation Oncology, Rigshospitalet, Copenhagen (Denmark); Christensen, Ib Jarle [Finsen Laboratory, Rigshospitalet, Copenhagen (Denmark); Caye-Thomasen, Per [Ear, Nose, and Throat Department, Rigshospitalet, Copenhagen (Denmark); Juhler, Marianne [Department of Neurosurgery, Rigshospitalet, Copenhagen (Denmark)

2012-08-01

Objective: 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. Methods and Materials: Forty-two patients receiving FSRT between 1997 and 2008 with a minimum follow-up of 2 years were included. All patients received 54 Gy in 27-30 fractions during 5.5-6.0 weeks. Clinical and audiometry data were collected prospectively. From a 'wait-and-scan' group, 409 patients were selected as control subjects, matched by initial audiometric parameters. Radiation dose to the cochlea was measured using the original treatment plan and then related to changes in acoustic parameters. Results: Actuarial 2-, 4-, and 10-year tumor control rates were 100%, 91.5%, and 85.0%, respectively. Twenty-one patients had serviceable hearing before FSRT, 8 of whom (38%) retained serviceable hearing at 2 years after FSRT. No patients retained serviceable hearing after 10 years. At 2 years, hearing preservation rates in the control group were 1.8 times higher compared with the group receiving FSRT (P=.007). Radiation dose to the cochlea was significantly correlated to deterioration of the speech reception threshold (P=.03) but not to discrimination loss. Conclusion: FSRT accelerates the naturally occurring hearing loss in patients with vestibular schwannoma. Our findings, using fractionation of radiotherapy, parallel results using single-dose radiation. The radiation dose to the cochlea is correlated to hearing loss measured as the speech reception threshold.

Fractionated Stereotactic Radiotherapy of Vestibular Schwannomas Accelerates Hearing Loss

International Nuclear Information System (INIS)

Rasmussen, Rune; Claesson, Magnus; Stangerup, Sven-Eric; Roed, Henrik; Christensen, Ib Jarle; Cayé-Thomasen, Per; Juhler, Marianne

2012-01-01

Objective: 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. Methods and Materials: Forty-two patients receiving FSRT between 1997 and 2008 with a minimum follow-up of 2 years were included. All patients received 54 Gy in 27-30 fractions during 5.5-6.0 weeks. Clinical and audiometry data were collected prospectively. From a “wait-and-scan” group, 409 patients were selected as control subjects, matched by initial audiometric parameters. Radiation dose to the cochlea was measured using the original treatment plan and then related to changes in acoustic parameters. Results: Actuarial 2-, 4-, and 10-year tumor control rates were 100%, 91.5%, and 85.0%, respectively. Twenty-one patients had serviceable hearing before FSRT, 8 of whom (38%) retained serviceable hearing at 2 years after FSRT. No patients retained serviceable hearing after 10 years. At 2 years, hearing preservation rates in the control group were 1.8 times higher compared with the group receiving FSRT (P=.007). Radiation dose to the cochlea was significantly correlated to deterioration of the speech reception threshold (P=.03) but not to discrimination loss. Conclusion: FSRT accelerates the naturally occurring hearing loss in patients with vestibular schwannoma. Our findings, using fractionation of radiotherapy, parallel results using single-dose radiation. The radiation dose to the cochlea is correlated to hearing loss measured as the speech reception threshold.

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.

Schwanoma de plexo braquial: relato de dois casos Schwannoma of brachial plexus: report of two cases

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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.

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...

Extramedullary plasmacytoma in the carotid space: Expanding the differential diagnosis

International Nuclear Information System (INIS)

Deshpande, Sneha Satish; Kane, Shubhada; Arya, Supreeta

2014-01-01

Plasma cell neoplasms have been classified into various types, with a range of clinical and radiological presentations. Extramedullary plasmacytoma (EMP) is a subset of plasma cell neoplasms which presents as an isolated non-osseous soft tissue mass. Though carotid space neoplasms are commonly encountered, EMP in the carotid space is rare and seldom considered in the initial differential diagnosis of a carotid space mass. These tumors can be treated by surgery or radiotherapy. On the other hand, the commonly encountered tumors in the carotid space are treated surgically. Also, it is mandatory to exclude multiple myeloma in the patients presenting with EMP. Hence, accurate and early diagnosis has therapeutic and prognostic implications. We report a rare case of EMP of the carotid space, describing the imaging features and the differential diagnoses with clues pointing to this rare entity

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.

Impact of video-endoscopy on the results of retrosigmoid-transmeatal microsurgery of vestibular schwannoma: prospective study

Czech Academy of Sciences Publication Activity Database

Chovanec, M.; Zvěřina, E.; Profant, Oliver; Skřivan, J.; Čakrt, O.; Lisý, J.; Betka, J.

2013-01-01

Roč. 270, č. 4 (2013), s. 1277-1284 ISSN 0937-4477 Grant - others:GA MZd(CZ) NS9909 Institutional support: RVO:68378041 Keywords : vestibular schwannoma * acoustic neuroma * endoscopy-assisted microsurgery Subject RIV: FH - Neurology Impact factor: 1.608, year: 2013

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

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...

Intradural approach to selective stimulation in the spinal cord for treatment of intractable pain: design principles and wireless protocol

Science.gov (United States)

Howard, M. A.; Utz, M.; Brennan, T. J.; Dalm, B. D.; Viljoen, S.; Jeffery, N. D.; Gillies, G. T.

2011-08-01

We introduce an intradural approach to spinal cord stimulation for the relief of intractable pain, and describe the biophysical rationale that underlies its design and performance requirements. The proposed device relies on wireless, inductive coupling between a pial surface implant and its epidural controller, and we present the results of benchtop experiments that demonstrate the ability to transmit and receive a frequency-modulated 1.6 MHz carrier signal between micro-coil antennae scaled to the ≈ 1 cm dimensions of the implant, at power levels of about 5 mW. Plans for materials selection, microfabrication, and other aspects of future development are presented and discussed.

Trigeminal Schwannoma with intra- and extracranial portions - a case report and review of the literature

International Nuclear Information System (INIS)

Souza, Ricardo Pires de; Setubal, Roger; Florencio, Filipe Toledo; Gomes, Marcio Rogerio Alcala; Mayo, Suzete Varela; Leiro, Luis Carlos Filgueira; Soares, Aldemir Humberto

1997-01-01

The authors report a case of a 40-year-old male patient presenting a mandibular branch Schwannoma of the trigeminal nerve with intra-and extracranial portions. The radiologic, computed tomographic and magnetic resonance imaging findings are discussed and a review of the literature is presented. (author)

Assessment of outcome in patients undergoing surgery for intradural spinal tumor using the multidimensional patient-rated Core Outcome Measures Index and the modified McCormick Scale.

Science.gov (United States)

Bellut, David; Burkhardt, Jan-Karl; Mannion, Anne F; Porchet, François

2015-08-01

OBJECT The aim of this study was to evaluate outcome in patients undergoing surgical treatment for intradural spinal tumor using a patient-oriented, self-rated, outcome instrument and a physician-based disease-specific instrument. METHODS Prospectively collected data from 63 patients with intradural spinal tumor were analyzed in relation to scores on the multidimensional patient-rated Core Outcome Measures Index (COMI) and the physician-rated modified McCormick Scale, before and at 3 and 12 months after surgery. RESULTS There was no statistically significant difference between the scores on the modified McCormick Scale preoperatively and at the 3-month follow-up, though there was a trend for improvement (p = 0.073); however, comparisons between the scores determined preoperatively and at the 12-month follow-up, as well as 3- versus 12-month follow-ups, showed a statistically significant improvement in each case (p 0.05) up to 12 months postoperatively. In contrast, the overall COMI score, "worst pain," quality of life, and social disability not only showed a significant reduction from before surgery to 3 months after surgery (p 0.05), but did show a significant improvement (p = 0.011) from 3 months to 12 months after surgery. At the 3- and 12-month follow-ups, 85.2% and 83.9% of patients, respectively, declared that the surgical procedure had helped/helped a lot; 95.1% and 95.2%, respectively, declared that they were satisfied/very satisfied with their care. CONCLUSIONS COMI is a feasible tool to use in the evaluation of baseline symptoms and outcome in patients undergoing surgery for intradural spinal tumor. COMI was able to detect changes in outcome at 3 months after surgery (before changes were apparent on the modified McCormick Scale) and on later postoperative follow-up. The COMI subdomains are valuable for monitoring the patient's reintegration into society and the work environment. The addition of an item that specifically covers neurological deficits may

Diagnosis and treatment of trigeminal schwannomas extending into both the middle and posterior cranial fossa

Institute of Scientific and Technical Information of China (English)

徐启武; 车晓明; 胡杰; 杨伯捷

2004-01-01

@@ Trigeminal schwannomas (TSs) account for 0.1%-0.4% of all intracranial tumors and 1%-8% of intracranial schwannomas.1,2 Yoshida and Kawase3 classified TSs into 6 types according to their locations: M (TS involving the middle cranial fossa), P (TS involving the posterior cranial fossa), E (TS located at extracranial space), MP (TS involving both the middle and the posterior cranial fossa), ME (TS involving the middle cranial fossa and the extracranial space), and MPE (TS involving the the middle and the posterior cranial fossa and the extracranial space). Of these types, MP is the commonest, but is difficult to be totally removed. Between January 1984 and June 2003, we surgically treated 28 patients with TSs of type MP and obtained satisfactory results. To make a correct diagnosis of TS, to select appropriate surgical approach, and to improve surgical outcome of TS of type MP, we analysed the related clinical data and experiences.

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...

Postural Stability Evaluation of Patients Undergoing Vestibular Schwannoma Microsurgery Employing the Inertial Measurement Unit

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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

Hearing outcomes of vestibular schwannoma patients managed with 'wait and scan': predictive value of hearing level at diagnosis

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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....

Lack of association between human herpesvirus and vestibular schwannoma: analysis of 121 cases.

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Bhimrao, Sanjiv K; Maguire, John; Garnis, Cathie; Tang, Patrick; Lea, Jane; Akagami, Ryojo; Westerberg, Brian D

2015-03-01

To assess for the presence of human herpesvirus (HHV) using immunohistochemical and polymerase chain reaction (PCR) assay in surgically excised vestibular schwannoma (VS) samples. Cross-sectional study. A retrospective laboratory-based study of tumors from patients with vestibular schwannoma. Tissue microarrays (TMAs) representing sporadic and NF2-associated VS from 121 patients, as well as appropriate positive and negative controls, were studied. TMA sections were immunostained using antibodies directed against HHV-1, HHV-2, HHV-3, HHV-4, HHV-5, and HHV-8. PCR was used for the detection of all 8 known human herpesviruses. There was no detectable HHV (HHV-1, HHV-2, HHV-3, HHV-4, HHV-5, HHV-8) by immunohistochemistry in any of the 121 cases of sporadic and NF2 cases analyzed. These data were further validated by DNA sequence analyses using PCR in a subset of the VS samples, all of which were found to be negative for all HHV. The data offer no support for an association between HHV and the development of sporadic or NF2-associated VS in humans. © American Academy of Otolaryngology—Head and Neck Surgery Foundation 2015.

Idiopathic myelofibrosis accompanied by peritoneal extramedullary hematopoiesis presenting as refractory ascites in a dog.

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Rautenbach, Yolandi; Goddard, Amelia; Clift, Sarah J

2017-03-01

A 2.5-year-old spayed female American Pit Bull Terrier dog presented with a primary complaint of chronic refractory ascites. The dog's CBC displayed a moderate to severe macrocytic, hypochromic, nonregenerative anemia, and a moderate leukopenia as result of a moderate neutropenia and monocytopenia. Microscopic examination of the blood smear showed marked anisocytosis, mild polychromasia, mild acanthocytosis and ovalocytosis, moderate schistocytosis and poikilocytosis, and 4 metarubricytes/100 WBC. Abdominal ultrasonography revealed a homogenous, mild to moderately hyperechoic appearing liver as well as marked amounts of speckled anechoic to slightly hypoechoic peritoneal fluid. Cytology of the ascitic fluid demonstrated a sterile transudate, with evidence of a chronic inflammatory reaction as well as erythroid and myeloid precursor cells, and a few megakaryocytes with occasional micromegakaryocytes. Histologic sections of bone marrow, spleen, and liver were examined, using routine H&E stains, as well as a variety of immunohistochemistry and other special stains. Histopathology of the bone marrow and spleen revealed varying degrees of fibrosis, erythroid, and myeloid hyperplasia, as well as multiple small hyperplastic clusters of megakaryocytes. The megakaryocytes displayed many features of atypia such as increased cytoplasmic basophilia and occasional abnormal chromatin clumping with mitoses. Histopathologic examination of the liver disclosed evidence of mild extramedullary hematopoiesis. This case represents the first report of canine idiopathic myelofibrosis associated with peritoneal extramedullary hematopoiesis, resulting in refractory ascites. Although idiopathic myelofibrosis is a relatively rare condition in dogs, this case demonstrates that ascites caused by peritoneal implants of hematopoietic tissue may be the initial manifestation of myelofibrosis. © 2016 American Society for Veterinary Clinical Pathology.

Ferrokinetic study of splenic erythropoiesis: Relationships among clinical diagnosis, myelofibrosis, splenomegaly, and extramedullary erythropoiesis

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Beguin, Y.; Fillet, G.; Bury, J.; Fairon, Y.

1989-01-01

Splenic erythropoiesis was demonstrated by surface counting of 59 Fe in 129 of 1,350 ferrokinetic studies performed over a 15 year period. These 129 studies were carried out in 108 patients, including 40 with chronic myelogenous leukemia (CML), 24 with agnogenic myeloid metaplasia (AMM), 18 with polycythemia vera (PV), six with a myelodysplastic syndrome, five with acute leukemia, three with prostate or breast carcinoma, two each with aplastic anemia or Hodgkin's disease, and one each with idiopathic thrombocythemia, multiple myeloma, chronic renal failure, or treated hypopituitarism. Splenomegaly was present in 83% of the studies and hepatomegaly in 72%. Grade II-III myelofibrosis was demonstrated in 62% of the cases. Hepatic erythropoiesis was present in 77% of the studies (only 38% in PV), and marrow erythropoiesis was undetectable in 33%. Total erythropoiesis was about twice normal (range 0.2 to 8 times normal) but was ineffective to varying degrees in 86% of the studies. Relationships between organomegaly, myelofibrosis, and extramedullary erythropoiesis, as well as differences among clinical disorders, are discussed. Differences observed between CML in chronic or blastic phase suggested that the erythroid cell line was involved in the proliferative process. It is concluded that splenic erythropoiesis (1) is encountered in a variety of clinical conditions; (2) is not necessarily associated with splenomegaly or myelofibrosis, even in the myeloproliferative disorders; (3) is part of a predominantly extramedullary (in the liver as well as in the spleen), expanded, and largely inefficient total erythropoiesis; and (4) can be evaluated in a semiquantitative manner by surface counting

Post-operative complications after removal of sporadic vestibular schwannoma via retrosigmoid-suboccipital approach: current diagnosis and management.

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Charalampakis, Stylianos; Koutsimpelas, Dimitrios; Gouveris, Haralampos; Mann, Wolf

2011-05-01

The retrosigmoid (suboccipital) approach is one of four surgical approaches for the treatment of vestibular schwannomas (acoustic neuromas). It is increasingly used by otologic surgeons, and in experienced hands is associated with improved results and more limited complications. Mortality rates are minimal and often zero, while postoperative sequelae, on the other hand, are not rare. In order to not only save the patient's life, but also to assure good quality of life after the surgery, one must consider many different aspects of management of the respective complications. In this review the issues of current management of CSF leak and meningitis, facial paresis, headache, hearing loss, unsteadiness, disequilibrium, vertigo, tinnitus, cerebellar and brain stem injuries or abscess, vascular complications and venous air embolism after retrosigmoid approach for removal of vestibular schwannomas are presented. © Springer-Verlag 2011

P12.01 Epidemiology in spinal tumors treated surgically at the South Central Hospital of High Specialty from PEMEX in Mexico

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Hernandez Resendiz, R.; Cordoba Mosqueda, M.; Guerra Mora, J.; Loya Aguilar, I.; Garcia Gonzalez, U.

2016-01-01

Abstract Introduction: The spinal tumors are rare neoplasms, they can be primary or metastatic; in the literature they are divided in extradural and intradural, extramedullary and intramedullary, from which extradural tumors are the most frequent and are usually metastatic, the intramedullary are generally gliomas. From the primary tumors up to 78% are benign and 22% malign, the histological stripe and the involvement to the spinal compartments are of great importance for the results and the treatment which is mainly surgical, individualized and meticulously planned with the support of technological resources such as the electrophysiological monitoring during the surgery. Methods and Materials: Observational study with a range of patients from March 1999- March 2016 to whom surgical resection of the spinal tumor was performed and reported on the Electronic Files of the South Central Hospital of High Specialty PEMEX. A Statistical analysis is made with the SPSS Statistic of disease of the Institution program. Results: 23 patients with spinal tumor surgical resection were found. The median age was 53 ± 10 years. The most common clinical manifestation was radiculopathy (65%). The Karnofsky scale was used for initial evaluation where a 43% of patients had a 90 score at the moment of the diagnosis, while 65% had an ECOG 1. The most frequent tumor was the Spinal Shwannoma (39%), followed in prevalence by the Condroid Cordoma (17%), where the intradural extramedullary location was the most prevalent (78%). The medium rate of survival after the surgical procedure was from 11 months. Conclusions: Our cases and the international statistics coincide. Radiculopathy as high prevalence initial manifestation conceals us to dismiss in the sixth decade of life any possibility for spinal tumor presentation. Most of spinal tumor patients do not have any clinical deterioration in their basal state, which indicates that performing a successful surgical procedure and the right

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

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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.

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.

Outcome after translabyrinthine surgery for vestibular schwannomas

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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....... 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...

Clinical, radiological, surgical, and pathological determinants of olfactory groove schwannoma

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Andi Sadayandi Ramesh

2014-01-01

Full Text Available Background: Olfactory groove schwannomas (OGS are rare anterior cranial fossa base tumors with only 41 cases reported in literature. Olfactory ensheathing cell schwannoma (OECS has similar clinico-radiological features as OGS, but a different cell of origin. In recent years, there is growing interest in OECS as more cases are being reported. Aims: The objective was to study the clinico-radiological features of OGS and define the histological differentiation from OECS. Materials and Methods: We retrospectively analyzed clinical, radiological, surgical and histopathological picture of all cases of OGS managed in our institute. Immuno histochemical studies were performed in these tumors for differentiating from OECS. A comprehensive review of articles published until date describing the operative treatment was done. Results: All three cases had presented with seizures, two had anosmia and papilledema. Gross-total resection was achieved in all our patients. One patient expired in the postoperative period due to septicemia. Positive expression to newer immuno histochemical biomarker CD57 (Leu7, with negative staining to smooth muscle α-actin (SMA was helpful in confirming the diagnosis of OGS and differentiating it from OECS in all our cases. Conclusions: OECS, though rare has to be differentiated from OGS using immuno histochemistry. Gross-total resection of OGS with preservation of olfactory function is often possible and curative. Although these tumors are commonly treated with microsurgical skull base approaches, an endoscopic endonasal approach can be considered in some cases, with repair using mucoperiosteal pedicled flap to prevent cerebrospinal fluid leak.

Extramedullary hematopoiesis with spinal cord compression in a child with thalassemia intermedia.

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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.

Extramedullary hematopoiesis and paraplegia in a patient with hemoglobin e-Beta thalassemia.

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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.

Serial CT Findings of Resolving Extramedullary Hematopoiesis as Unilateral Posterior Mediastinal Mass after Splenectomy in Hereditary Spherocytosis: A Case Report

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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.

Serial CT Findings of Resolving Extramedullary Hematopoiesis as Unilateral Posterior Mediastinal Mass after Splenectomy in Hereditary Spherocytosis: A Case Report

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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.

MRI Findings of Juvenile Xanthogranuloma of the Spinal Cord: A Case Report

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Kim, Se Young; Park, Hee Jin; Lee, So Yeon; Chung, Eun Chul; Park, Hae Won; Kook, Shin Ho; Rho, Myung Ho; Goo, Ji Hye

2013-01-01

Juvenile xanthogranuloma (JXG) is a proliferative histiocytic disorder experienced during childhood and adolescents. JXG commonly presents as a solitary cutaneous lesion. Despite the term 'juvenile', development of the disease during adulthood is possible, although spinal JXG is extremely rare in adults. We describe a 67-year-old female patient who presented with an intradural-extramedullary (IDEM) tumor of the spinal cord. Magnetic resonance imaging (MRI) findings indicative of JXG of the spinal cord were seen, which was then confirmed pathologically. A lumbar spinal MRI with contrast enhancement showed an oval-shaped, well-defined IDEM tumor at the L1 level. This tumor had mixed signal intensity on the T1-weighted image and high signal intensity on the T2-weighted image. Central homogenous enhancement was observed after contrast administration.

Anesthetic management of schwannoma of the base of the tongue

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Upma B Batra

2011-01-01

Full Text Available Schwannoma arising from the base of the tongue are very rare and only a few cases have been reported so far. Definitive diagnosis is always made after a histological examination. Apart from an anticipated difficult airway with a risk of airway obstruction upon induction of general anesthesia, anesthetic concerns also include possibility of trauma to the growth and bleeding with attendant risks. We discuss the awake fiberoptic technique used for endotracheal intubation in such a case. This case report highlights the importance of detailed history taking and clinical examination, with emphasis on airway assessment and preoperative planning.

Slip Interface Imaging Predicts Tumor-Brain Adhesion in Vestibular Schwannomas.

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Yin, Ziying; Glaser, Kevin J; Manduca, Armando; Van Gompel, Jamie J; Link, Michael J; Hughes, Joshua D; Romano, Anthony; Ehman, Richard L; Huston, John

2015-11-01

To test the clinical feasibility and usefulness of slip interface imaging (SII) to identify and quantify the degree of tumor-brain adhesion in patients with vestibular schwannomas. S With institutional review board approval and after obtaining written informed consent, SII examinations were performed in nine patients with vestibular schwannomas. During the SII acquisition, a low-amplitude mechanical vibration is applied to the head with a pillow-like device placed in the head coil and the resulting shear waves are imaged by using a phase-contrast pulse sequence with motion-encoding gradients synchronized with the applied vibration. Imaging was performed with a 3-T magnetic resonance (MR) system in less than 7 minutes. The acquired shear motion data were processed with two different algorithms (shear line analysis and calculation of octahedral shear strain [OSS]) to identify the degree of tumor-brain adhesion. Blinded to the SII results, neurosurgeons qualitatively assessed tumor adhesion at the time of tumor resection. Standard T2-weighted, fast imaging employing steady-state acquisition (FIESTA), and T2-weighted fluid-attenuated inversion recovery (FLAIR) imaging were reviewed to identify the presence of cerebral spinal fluid (CSF) clefts around the tumors. The performance of the use of the CSF cleft and SII to predict the degree of tumor adhesion was evaluated by using the κ coefficient and McNemar test. Among the nine patients, SII agreed with the intraoperative assessment of the degree of tumor adhesion in eight patients (88.9%; 95% confidence interval [CI]: 57%, 98%), with four of four, three of three, and one of two cases correctly predicted as no adhesion, partial adhesion, and complete adhesion, respectively. However, the T2-weighted, FIESTA, and T2-weighted FLAIR images that used the CSF cleft sign to predict adhesion agreed with surgical findings in only four cases (44.4% [four of nine]; 95% CI: 19%, 73%). The κ coefficients indicate good agreement (0

Extramedullary hematopoiesis in a case of benign mixed mammary tumor in a female dog: cytological and histopathological assessment

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Leão João

2010-09-01

Full Text Available Abstract Backgroud Extramedullary hematopoiesis (EMH is defined as the presence of hematopoietic stem cells such as erythroid and myeloid lineage plus megakaryocytes in extramedullary sites like liver, spleen and lymph nodes and is usually associated with either bone marrow or hematological disorders. Mammary EMH is a rare condition either in human and veterinary medicine and can be associated with benign mixed mammary tumors, similarly to that described in this case. Case presentation Hematopoietic stem cells were found in a benign mixed mammary tumor of a 7-year-old female mongrel dog that presents a nodule in the left inguinal mammary gland. The patient did not have any hematological abnormalities. Cytological evaluation demonstrated two distinct cell populations, composed of either epithelial or mesenchymal cells, sometimes associated with a fibrillar acidophilic matrix, apart from megakaryocytes, osteoclasts, metarubricytes, prorubricytes, rubricytes, rubriblasts, promyelocytes, myeloblasts. Histological examination confirmed the presence of an active hematopoietic bone marrow within the bone tissue of a benign mammary mixed tumor. Conclusions EMH is a rare condition described in veterinary medicine that can be associated with mammary mixed tumors. It's detection can be associated with several neoplastic and non-neoplastic mammary lesions, i.e. osteosarcomas, mixed tumors and bone metaplasia.

Long-Term Follow-up of Acoustic Schwannoma Radiosurgery With Marginal Tumor Doses of 12 to 13 Gy

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Chopra, Rahul; Kondziolka, Douglas; Niranjan, Ajay; Lunsford, L. Dade; Flickinger, John C.

2007-01-01

Purpose: To define long-term tumor control and clinical outcomes of radiosurgery with marginal tumor doses of 12 to 13 Gy for unilateral acoustic schwannoma. Methods and Materials: A total of 216 patients with previously untreated unilateral acoustic schwannoma underwent Gamma Knife radiosurgery between 1992 and 2000 with marginal tumor doses of 12 to 13 Gy (median, 13 Gy). Median follow-up was 5.7 years (maximum, 12 years; 41 patients with >8 years). Treatment volumes were 0.08-37.5 cm 3 (median, 1.3 cm 3 ). Results: The 10-year actuarial resection-free control rate was 98.3% ± 1.0%. Three patients required tumor resection: 2 for tumor growth and 1 partial resection for an enlarging adjacent subarachnoid cyst. Among 121 hearing patients with >3 years of follow-up, crude hearing preservation rates were 71% for keeping the same Gardner-Robertson hearing level, 74% for serviceable hearing, and 95% for any testable hearing. For 25 of these patients with intracanalicular tumors, the respective rates for preserving the same Gardner-Robertson level, serviceable hearing, and testable hearing were 80%, 88%, and 96%. Ten-year actuarial rates for preserving the same Gardner-Robertson hearing levels, serviceable hearing, any testable hearing, and unchanged facial and trigeminal nerve function were 44.0% ± 11.7%, 44.5% ± 10.5%, 85.3% ± 6.2%, 100%, and 94.9% ± 1.8%, respectively. Conclusions: Acoustic schwannoma radiosurgery with 12 to 13 Gy provides high rates of long-term tumor control and cranial nerve preservation after long-term follow-up

Vestibular schwannoma (acoustic neuroma mimicking temporomandibular disorders: a case report Schwannoma vestibular (neurinoma do acústico imitando desordens temporomandibulares: um relato de caso

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Maurício A. Bisi

2006-12-01

Full Text Available 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.Aproximadamente 6 a 16% dos pacientes com sintomas de neuralgia trigeminal apresentam tumores intracranianos, sendo mais comum o schwannoma vestibular (neurinoma do acústico. Alguns sintomas relatados pelos pacientes são perda da audição, zumbido, dores de cabeça, vertigens e distúrbios trigeminais. Uma resposta muscular aumentada na musculatura associada da cabeça e do pescoço também pode ser observada, o que pode mimetizar sinais e sintomas de desordens temporomandibulares. Nestes casos é de grande valia o uso de imagem de ressonância magnética (IRM para detecção de tumores. É importante, também, a diferenciação de dores miofasciais e neurálgicas, pois ambas podem apresentar características semelhantes, mas com origens e tratamentos diferentes. O objetivo desse trabalho foi demonstrar através de relato de caso clínico a associação entre sintomas de neuralgia trigeminal, tumores intracranianos e disfunção temporomandibular.

F-18-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography Appearance of Extramedullary Hematopoesis in a Case of Primary Myelofibrosis

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Mukherjee, Anirban; Bal, Chandrasekhar; Tripathi, Madhavi; Das, Chandan Jyoti; Shamim, Shamim Ahmed

2017-01-01

A 44-year-old female with known primary myelofibrosis presented with shortness of breath. High Resolution Computed Tomography thorax revealed large heterogeneously enhancing extraparenchymal soft tissue density mass involving bilateral lung fields. F-18-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography revealed mildly FDG avid soft tissue density mass with specks of calcification involving bilateral lung fields, liver, and spleen. Subsequent histopathologic evaluation from the right lung mass was suggestive of extramedullary hematopoesis. PMID:28533647

Intracranial schwannomas arising from cranial nerves: Case series and review of literature

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Rashmeet Kaur

2017-01-01

Full Text Available Tumors arising from the cranial nerve sheath are common intracranial neoplasms, with only few articles describing their imaging characteristics. In this present study of four cases of schwannomas originating from the cranial nerves in the head region, we are discussing the radiological features on imaging with the clinical presentation and contrasting them with other differentials in their respective locations. Magnetic resonance imaging and computed tomography were done in these cases and correlated with clinical and biopsy findings.

Preoperative Identification of Facial Nerve in Vestibular Schwannomas Surgery Using Diffusion Tensor Tractography

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Choi, Kyung-Sik; Kim, Min-Su; Kwon, Hyeok-Gyu; Jang, Sung-Ho; Kim, Oh-Lyong

2014-01-01

Objective Facial nerve palsy is a common complication of treatment for vestibular schwannoma (VS), so preserving facial nerve function is important. The preoperative visualization of the course of facial nerve in relation to VS could help prevent injury to the nerve during the surgery. In this study, we evaluate the accuracy of diffusion tensor tractography (DTT) for preoperative identification of facial nerve. Methods We prospectively collected data from 11 patients with VS, who underwent pr...

Age, gender and tumour size predict work capacity after surgical treatment of vestibular schwannomas.

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Al-Shudifat, Abdul Rahman; Kahlon, Babar; Höglund, Peter; Soliman, Ahmed Y; Lindskog, Kristoffer; Siesjo, Peter

2014-01-01

The aim of the present study was to identify predictive factors for outcome after surgery of vestibular schwannomas. This is a retrospective study with partially collected prospective data of patients who were surgically treated for vestibular schwannomas at a single institution from 1979 to 2000. Patients with recurrent tumours, NF2 and those incapable of answering questionnaires were excluded from the study. The short form 36 (SF36) questionnaire and a specific questionnaire regarding neurological status, work status and independent life (IL) status were sent to all eligible patients. The questionnaires were sent to 430 eligible patients (out of 537) and 395 (93%) responded. Scores for work capacity (WC) and IL were compared with SF36 scores as outcome estimates. Patients were divided into two groups (group age, gender and tumour diameter were independent predictive factors for postoperative WC in multivariate analysis. A high-risk group was identified in women with age >50 years and tumour diameter >25 mm. In patients ≥64, gender and tumour diameter were significant predictive factors for IL in univariate analysis. Perioperative and postoperative objective factors as length of surgery, blood loss and complications did not predict outcome in the multivariable analysis for any age group. Patients' assessment of change in balance function was the only neurological factor that showed significance both in univariate and multivariable analysis in both age cohorts. While SF36 scores were lower in surgically treated patients in relation to normograms for the general population, they did not correlate significantly to WC and IL. The SF36 questionnaire did not correlate to outcome measures as WC and IL in patients undergoing surgery for vestibular schwannomas. Women and patients above 50 years with larger tumours have a high risk for reduced WC after surgical treatment. These results question the validity of quality of life scores in assessment of outcome after surgery

Flow cytometric minimal residual disease assessment of peripheral blood in acute lymphoblastic leukaemia patients has potential for early detection of relapsed extramedullary disease.

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Keegan, Alissa; Charest, Karry; Schmidt, Ryan; Briggs, Debra; Deangelo, Daniel J; Li, Betty; Morgan, Elizabeth A; Pozdnyakova, Olga

2018-03-27

To evaluate peripheral blood (PB) for minimal residual disease (MRD) assessment in adults with acute lymphoblastic leukaemia (ALL). We analysed 76 matched bone marrow (BM) aspirate and PB specimens independently for the presence of ALL MRD by six-colour flow cytometry (FC). The overall rate of BM MRD-positivity was 24% (18/76) and PB was also MRD-positive in 22% (4/18) of BM-positive cases. We identified two cases with evidence of leukaemic cells in PB at the time of the extramedullary relapse that were interpreted as MRD-negative in BM. The use of PB MRD as a non-invasive method for monitoring of systemic relapse may have added clinical and diagnostic value in patients with high risk of extramedullary disease. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Detection of Extramedullary Multiple Myeloma in Liver by FDG-PET/CT

International Nuclear Information System (INIS)

Kim, Daeweung; Kim, Woo Hyoung; Kim, Myoung Hyoun; Choi, Keum Ha; Kim, Chang Guhn

2014-01-01

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 18 F-fluoro-2-deoxyglucose ( 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, 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. 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 F-FDG PET revealed

Delivery of Therapeutic Proteins via Extracellular Vesicles: Review and Potential Treatments for Parkinson's Disease, Glioma, and Schwannoma.

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Hall, Justin; Prabhakar, Shilpa; Balaj, Leonora; Lai, Charles P; Cerione, Richard A; Breakefield, Xandra O

2016-04-01

Extracellular vesicles present an attractive delivery vehicle for therapeutic proteins. They intrinsically contain many proteins which can provide information to other cells. Advantages include reduced immune reactivity, especially if derived from the same host, stability in biologic fluids, and ability to target uptake. Those from mesenchymal stem cells appear to be intrinsically therapeutic, while those from cancer cells promote tumor progression. Therapeutic proteins can be loaded into vesicles by overexpression in the donor cell, with oligomerization and membrane sequences increasing their loading. Examples of protein delivery for therapeutic benefit in pre-clinical models include delivery of: catalase for Parkinson's disease to reduce oxidative stress and thus help neurons to survive; prodrug activating enzymes which can convert a prodrug which crosses the blood-brain barrier into a toxic chemotherapeutic drug for schwannomas and gliomas; and the apoptosis-inducing enzyme, caspase-1 under a Schwann cell specific promoter for schwannoma. This therapeutic delivery strategy is novel and being explored for a number of diseases.

Rare case of primary spinal ependymomatosis occurring in a 26-year-old man: a case report

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Kaliaperumal Chandrasekaran

2009-10-01

Full Text Available Abstract Introduction The authors report a rare case of primary spinal ependymomatosis in a young adult man. Multiple primary ependymomatous lesions were seen on magnetic resonance imaging and no anaplasia was identified on the surgical-pathological analysis. The aetio-pathological mechanism and surgical significance of this rare occurrence is discussed. Case presentation A 26-year-old man of Polish origin presented with a ten-day history of pain in the left leg and lower back. This was followed by difficulty in urinating and a decrease in sensation in both legs. Examination revealed pyramidal signs and mild weakness in both lower limbs. He had early sphincter involvement requiring catheterization. Magnetic resonance imaging of the brain was normal. However, that of the spinal cord revealed multiple intradural spinal lesions, both intra- and extramedullary, extending from the cervical cord down to the cauda equina roots. T12-L1 laminectomy was performed. Multiple intradural, extra- and intra-medullary tumors were seen. After the operation, the patient deteriorated with a sensory level at T4. Post-operative cranio-spinal radiotherapy was administered but there was no clinical improvement in the lower limbs. Conclusion Primary spinal ependymomatosis is a rare phenomenon involving multiple spinal segments in the absence of a primary intracranial tumor. Radical excision is unrealistic in this condition. Biopsy followed by radiotherapy is the preferred method of treatment.

Ten-Year Follow-up on Tumor Growth and Hearing in Patients Observed With an Intracanalicular Vestibular Schwannoma

DEFF Research Database (Denmark)

Kirchmann, Malene; Karnov, Kirstine; Hansen, Søren

2017-01-01

BACKGROUND: Reports on the natural history of tumor growth and hearing in patients with a vestibular schwannoma (VS) are almost exclusively short-term data. Long-term data are needed for comparison with results of surgery and radiotherapy.  OBJECTIVE: To report the long-term occurrence of tumor g...

Place of Gamma Knife Stereotactic Radiosurgery in Grade 4 Vestibular Schwannoma Based on Case Series of 86 Patients with Long-Term Follow-Up.

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Lefranc, Michel; Da Roz, Leila Maria; Balossier, Anne; Thomassin, Jean Marc; Roche, Pierre Hugue; Regis, Jean

2018-06-01

Grade IV vestibular schwannoma (Koos classification) is generally considered to be an indication for microsurgical resection or combined radiosurgery-microsurgery. However, the place of Gamma Knife stereotactic surgery (GK-SRS), either as first-line treatment or when progression of residual tumor compresses the brainstem, has not been clearly evaluated. This article reports the results of a large case series of patients with grade 4 vestibular schwannoma treated by GK-SRS. All consecutive patients with grade IV vestibular schwannoma treated by GK-SRS in our department between 1996 and 2011 with a minimum follow-up of 3 years were included in this study. 86 patients were treated by GK-SRS with a minimum follow-up of 3 years. Mean follow-up was 6.2 years (3-16 years). The mean age of the patients at the time of GK-SRS was 54.6 years (range: 23-84) and the sex ratio was 0.6. At the time of radiosurgery, no patient presented brainstem dysfunction prior to GK-SRS. 38 patients had functional hearing before treatment. One patient presented mild trigeminal neuralgia before GK-SRS. Tumor control with no clinical deterioration was obtained in 78 patients (90.7%). No radiation-induced brainstem or cranial nerve toxicity was observed in any of these patients. Functional hearing was maintained in 25 patients. 8 (9.3%) patients presented tumor growth and required microsurgical resection in 7 cases and ventricular shunt in 1 case. On the basis of this large series, GK-SRS appears to be a safe and effective treatment option for grade IV vestibular schwannoma for patients with no signs of brainstem dysfunction. Copyright © 2018 Elsevier Inc. All rights reserved.

Anterolateral meningioma of the foramen magnum and high cervical spine presenting intradural and extradural growth in a child: case report and literature review.

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Athanasiou, Alkinoos; Magras, Ioannis; Sarlis, Panagiotis; Spyridopoulos, Evangelos; Polyzoidis, Konstantinos

2015-12-01

We report a rare case of anterolateral meningioma of the foramen magnum (FMM) and high cervical spine presenting both intradural and extradural growth in a 7.5-year-old boy. We also performed a review of the relevant peer-reviewed literature. The patient presented with progressive tetraparesis and gait instability. Neuroimaging revealed an anterolateral tumor of the foramen magnum, C1 and C2 cervical spine level. The patient was treated in two stages: During the first operation, the extradural part was resected while the intradural part was removed in a second operation. Following the second operation, the patient showed almost complete neurological recovery as a result of cervical spinal cord and brainstem decompression but was complicated with cerebrospinal fluid leakage and infection by Acinetobacter. He sustained two further operations for dural sealing and external ventricular drainage and was treated with intraventricular administration of antibiotics. Histopathology of the tumor confirmed a meningotheliomatous meningioma. At the 6-month post-op follow-up examination, the patient exhibited complete neurological recovery and no radiological tumor recurrence. To the authors' best knowledge, we report the third case of sporadic pediatric meningioma of the foramen magnum and high cervical compartments with an extradural growth. Accurate pre-operative estimation of possible extradural growth is crucial towards surgical planning and sufficient treatment. Treatment of choice is total resection in a single operating session to avoid re-operations and increased risk of complications. If not possible, a re-operation should always attempt to secure the desired result.

Hearing preservation after low-dose gamma knife radiosurgery of vestibular schwannomas

International Nuclear Information System (INIS)

Horiba, Ayako; Hayashi, Motohiro; Chernov, Mikhail; Kawamata, Takakazu; Okada, Yoshikazu

2016-01-01

The objective of the retrospective study was to evaluate the factors associated with hearing preservation after low-dose Gamma Knife radiosurgery (GKS) of vestibular schwannomas performed according to the modern standards. From January 2005 to September 2010, 141 consecutive patients underwent such treatment in Tokyo Women's Medical University. Mean marginal dose was 11.9 Gy (range, 11-12 Gy). The doses for the brain stem, cranial nerves (V, VII, and VHIII), and cochlea were kept below 14 Gy, 12 Gy, and 4 Gy, respectively. Out of the total cohort, 102 cases with at least 24 months follow-up were analyzed. Within the median follow-up of 56 months (range, 24-99 months) the crude tumor growth control was 92% (94 cases), whereas its actuarial rate at 5 years was 93%. Out of 49 patients with serviceable hearing on the side of the tumor before GKS, 28 (57%) demonstrated its preservation at the time of the last follow-up. No one evaluated factor, namely Gardner-Robertson hearing class before irradiation, Koos tumor stage, extension of the intrameatal part of the neoplasm up to fundus, nerve of tumor origin, presence of cystic changes in the neoplasm, and cochlea dose demonstrated statistically significant association with preservation of the serviceable hearing after radiosurgery. In conclusion, GKS of vestibular schwannomas performed according to the modern standards of treatment permits to preserve serviceable hearing on the side of the tumor in more than half of the patients. The actual causes of hearing deterioration after radiosurgery remain unclear. (author)

Epithelioid schwannoma of the skin displaying unique histopathological features: a teaching case giving rise to diagnostic difficulties on a morphological examination of a resected specimen, with a brief literature review.

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Yamada, Sohsuke; Kirishima, Mari; Hiraki, Tsubasa; Higashi, Michiyo; Hatanaka, Kazuhito; Tanimoto, Akihide

2017-01-19

Epithelioid schwannoma as a rare variant poses a challenge to all pathologists, as this uncommon entity is extremely difficult to conclusively diagnose by morphological analyses on a resected sample alone owing to its unique histopathological features. However, few papers have described the detailed clinicopathological characteristics of epithelioid schwannoma. A 65-year-old female presented with a history of a flat and slightly elevated firm and tan plaque accompanied by occasional tenderness, measuring 10 × 8 mm, in the right joint of her hand 1 year before resection. A gross examination of a locally resected specimen revealed an encapsulated nodular lesion, yellow-whitish in color, partly filled with blood. A microscopic examination showed that the tumor predominantly consisted of a solid proliferation of epithelioid cells having mildly enlarged and round to partially spindled nuclei and abundant vacuolated or clear cytoplasm with very few mitotic figures and modest nuclear size variation, associated with focal hyalinized, cystic and hemorrhagic degeneration. This well-demarcated tumor was surrounded by dense, hyalinized and layered fibrocollagenous stroma. Immunohistochemically, these tumor cells were diffusely positive for S-100 protein and had a very low MIB-1 labeling index, and type IV collagen was strongly reactive with reduplicated basal lamina of them. We ultimately made a diagnosis of cutaneous epithelioid schwannoma. We should be aware that, since pathologists might misinterpret epithelioid schwannoma as other soft tissue tumors, including its malignant counterpart, a wide panel of immunohistochemical antibodies can be powerful supplementary tools for identifying a very rare entity of conventional schwannoma.

Cervical Intradural Disc Herniation Causing Progressive Quadriparesis After Spinal Manipulation Therapy: A Case Report and Literature Review.

Science.gov (United States)

Yang, Hwan-Seo; Oh, Young-Min; Eun, Jong-Pil

2016-02-01

Cervical intradural disc herniation (IDH) is an extremely rare condition, comprising only 0.27% of all disc herniations. Three percent of IDHs occur in the cervical, 5% in the thoracic, and over 92% in the lumbar spinal canal. There have been a total of 31 cervical IDHs reported in the literature. The pathogenesis and imaging characteristics of IDH are not fully understood. A preoperative diagnosis is key to facilitating prompt intradural exploration in patients with ambivalent findings, as well as in preventing reoperation. The purpose of reporting our case is to remind clinicians to consider the possibility of cervical IDH during spinal manipulation therapy in patient with chronic neck pain.The patient signed informed consent for publication of this case report and any accompanying image. The ethical approval of this study was waived by the ethics committee of Chonbuk National University Hospital, because this study was case report and the number of patients was manipulation for chronic neck pain over the course of a month. The day prior, he had noticed neck pain and tingling in the bilateral upper and lower extremities during the manipulation procedure. The following day, he presented with bilateral weakness of all 4 extremities, which rendered him unable to walk. Neurological examination demonstrated a positive Hoffmann sign and ankle clonus bilaterally, hypoesthesia below the C5 dermatome, 3/5 strength in the bilateral upper extremities, and 2/5 strength in the lower extremities. This motor weakness was progressive, and he further complained of voiding difficulty.Urgent magnetic resonance imaging (MRI) of the cervical spine revealed large, central disc herniations at C4-C5 and C5-C6 that caused severe spinal cord compression and surrounding edema. We performed C4-C5-C6 anterior cervical discectomy and fusion.The patient's limb weakness improved rapidly within 1 day postoperatively, and he was discharged 4 weeks later. At his 12-month follow-up, the patient had

Schwanoma de laringe: relato de caso Schwannoma of the larynx: case report

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Erich Christiano M. Melo

2004-04-01

Full Text Available Schwanomas são tumores benignos, de crescimento lento, encapsulados, que surgem da bainha das células de Schwann de nervos motores, sensitivos ou cranianos, não contendo elementos nervosos. A localização na laringe é extremamente rara, existindo relatos esporádicos na literatura mundial. O objetivo deste trabalho é relatar um caso de schwanoma laríngeo atendido em nosso meio. O paciente de 82 anos de idade, sexo feminino, procurou nosso serviço com queixa de disfagia para sólidos de longa data e sensação de globus faríngeo. A laringoscopia com telescópio rígido de 70º evidenciou um tumor submucoso, arredondado, na região interaritenóidea. A tomografia computadorizada de pescoço mostrou tratar-se de lesão aparentemente sólida, sem necrose central e sem extensão para planos profundos, medindo aproximadamente 2,5 cm no maior diâmetro. A paciente foi submetida a microcirurgia de laringe, com ressecção completa da lesão. O exame histopatológico revelou tratar-se de um schwanoma laríngeo. A paciente evoluiu bem, sem sinais de tumor residual ou recidiva em dois anos de seguimento clínico.Schwannomas are benign, slow-growing, encapsulated tumors, which appear on the sheath of sensitive or cranial motor nerves Schwann cells, and having no nervous elements. They rarely appear on the larynx, and there are sporadic reports in the literature. This work has as purpose to report a case of laryngeal schwannoma. A 82 years-old woman, born and residing in the city of Sao Paulo, SP, went to the Hospital, suffering from slowly progressive, long-term dysphagia for solids and globus sensation. The indirect laryngoscopy with a 70º rigid telescope revealed a submucous tumor, rounded, at the interarytenoid region. A neck computed tomography was made, revealing an apparently solid lesion, with no central necrosis and no extension to deeper levels, the greatest diameter of which was of 2.5 cm. The patient underwent a larynx microsurgery with

Gamma knife radiosurgery for acoustic Schwannoma. Early effects and preservation of hearing

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Hirato, Masafumi; Inoue, Hiroshi; Nakamura, Masaru; Ohye, Chihiro; Hirato, Junko [Gunma Univ., Maebashi (Japan). School of Medicine; Shibazaki, Tohru; Andou, Yoshitaka

1995-10-01

The effects of relatively low dose gamma knife irradiation on acoustic Schwannoma were evaluated. The signal intensity change and tumor shrinkage on magnetic resonance (MR) images, change in hearing, and complications in 28 patients (mean age 47.0{+-}13.6 yrs) were studied. Three patients had bilateral tumors. Six were already deaf when treated. The maximum tumor diameter was 35 mm. The mean dose delivered to the tumor was 12.1{+-}1.6 Gy at the periphery, and 25.2{+-}4.3 Gy at the center. The mean follow-up time was 16 months and the longest 24 months. Lowering of the MR signal intensity in the tumor center appeared after 3 months at earliest but generally after 6 months. Signs of tumor shrinkage appeared within 12 months on average. Cyst in the tumor enlarged rapidly after treatment in two patients. The percentage of hearing preservation was 85% (17/20) at 3 months, 80% (16/20) at 6 months, 72% (13/18) at 9 months, 75% (12/16) at 12 months, 67% (8/12) at 15 months, 60% (6/10) at 18 months, and 50% (2/4) at 24 months. Subtle changes in hearing were detected by speech tone audiometry. Temporary facial numbness and weakness was seen in one patient each. No patient had lower cranial nerve paresis. Relatively low dose gamma knife radiosurgery is effective in suppressing growth of acoustic Schwannoma with preservation of hearing. (author).

Residential traffic noise exposure and vestibular schwannoma - a Danish case-control study.

Science.gov (United States)

Roswall, Nina; Stangerup, Sven-Eric; Cayé-Thomasen, Per; Schüz, Joachim; Johansen, Christoffer; Jensen, Steen Solvang; Raaschou-Nielsen, Ole; Sørensen, Mette

2017-10-01

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 traffic noise was associated with vestibular schwannoma in a large, population-based Danish case-control study. We identified 1454 VS cases, age above 30 years at diagnosis, between 1990 and 2007. For each case, we selected two random population controls, matched on sex and year of birth. Road and railway traffic noise at the residence was calculated for all present and historical addresses between 1987 and index date. Associations between traffic noise and risk for VS were estimated using conditional logistic regression, adjusted for education, disposable personal income, cohabitation status, railway noise exposure, municipal population density, and municipal income. A two-year time-weighted mean road traffic noise exposure was associated with an adjusted odds ratio of 0.92 (0.82-1.03) for developing VS, per 10 dB increment. There was no clear trend in categorical analyses. Similarly, linear and categorical analyses of residential railway noise did not suggest an association. We found no interaction with demographics, year of diagnosis, individual and municipal socioeconomic variables, and railway noise exposure. The results did not differ by tumor side, spread or size. The present study does not suggest an association between residential traffic noise and VS.

Gamma knife radiosurgery for acoustic Schwannoma. Early effects and preservation of hearing

International Nuclear Information System (INIS)

Hirato, Masafumi; Inoue, Hiroshi; Nakamura, Masaru; Ohye, Chihiro; Hirato, Junko; Shibazaki, Tohru; Andou, Yoshitaka.

1995-01-01

The effects of relatively low dose gamma knife irradiation on acoustic Schwannoma were evaluated. The signal intensity change and tumor shrinkage on magnetic resonance (MR) images, change in hearing, and complications in 28 patients (mean age 47.0±13.6 yrs) were studied. Three patients had bilateral tumors. Six were already deaf when treated. The maximum tumor diameter was 35 mm. The mean dose delivered to the tumor was 12.1±1.6 Gy at the periphery, and 25.2±4.3 Gy at the center. The mean follow-up time was 16 months and the longest 24 months. Lowering of the MR signal intensity in the tumor center appeared after 3 months at earliest but generally after 6 months. Signs of tumor shrinkage appeared within 12 months on average. Cyst in the tumor enlarged rapidly after treatment in two patients. The percentage of hearing preservation was 85% (17/20) at 3 months, 80% (16/20) at 6 months, 72% (13/18) at 9 months, 75% (12/16) at 12 months, 67% (8/12) at 15 months, 60% (6/10) at 18 months, and 50% (2/4) at 24 months. Subtle changes in hearing were detected by speech tone audiometry. Temporary facial numbness and weakness was seen in one patient each. No patient had lower cranial nerve paresis. Relatively low dose gamma knife radiosurgery is effective in suppressing growth of acoustic Schwannoma with preservation of hearing. (author)

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.

Radiotherapy for Extramedullary Plasmacytoma of the Head and Neck

International Nuclear Information System (INIS)

Creach, Kimberly M.; Foote, Robert L.; Neben-Wittich, Michelle A.; Kyle, Robert A.

2009-01-01

Purpose: To define the effectiveness of radiotherapy in the treatment of patients with extramedullary plasmacytoma of the head and neck (EMPHN). Methods and Materials: We searched the Mayo Clinic Rochester Department of Radiation Oncology electronic Tumor Registry and identified 18 consecutive patients with a diagnosis of solitary EMPHN. Sixteen patients were treated with radiotherapy at initial diagnosis and 2 received salvage radiotherapy for local failure after surgery. Median dose administered was 50.4 Gy. Median follow-up was 6.8 years. Results: One patient (6%) developed a marginal recurrence 12 months after treatment. Six patients (33%) developed multiple myeloma (2 patients) or plasmacytomas at distant sites (4 patients) at a median of 3.1 years after diagnosis (range, 0.02 to 9.6 years). Median and 5- and 10-year overall survival rates from the date of diagnosis are 12.5 years, 88%, and 55%, respectively. Two patients (11%) developed a radiation-induced malignancy at 6.5 and 6.9 years after treatment. Conclusions: Radiotherapy provides excellent local and regional tumor control and survival in patients with EMPHN. To the best of our knowledge, this is the first report of presumed radiation-induced malignancy in this patient population

Thorascopic resection of an apical paraspinal schwannoma using the da Vinci surgical system.

Science.gov (United States)

Finley, David; Sherman, Jonathan H; Avila, Edward; Bilsky, Mark

2014-01-01

Posterior mediastinal neurogenic tumors have traditionally been resected via an open posterolateral thoracotomy. Video-assisted thorascopic surgery has emerged as an alternative technique allowing for improved morbidity with decreased blood loss, less postoperative pain, and a shorter recovery period, among others. The da Vinci surgical system, as first described for urologic procedures, has recently been reported for lung lobectomy. This technique provides the advantages of instrumentation with 6 degrees of freedom, stable operating arms, and improved visualization with the three-dimensional high-definition camera. We describe the technique for thorascopic resection of an apical paraspinal schwannoma of the T1 nerve root with the da Vinci surgical system. This technique used a specialized intraoperative neuromonitoring probe for free-running electromyography (EMG) and triggered EMG. We demonstrate successful resection of a posterior paraspinal schwannoma with the da Vinci surgical system while preserving neurologic function. The patient displayed stable intraoperative monitoring of the T1 nerve root and full intrinsic hand strength postoperatively. The technique described in this article introduces robotic system accuracy and precludes the need for an open thoracotomy. In addition, this approach demonstrates the ability of the da Vinci surgical system to safely dissect tumors from their neural attachments and is applicable to other such lesions of similar size and location. Georg Thieme Verlag KG Stuttgart · New York.

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.

Electrical vestibular stimulation after vestibular deafferentation and in vestibular schwannoma.

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Swee Tin Aw

Full Text Available BACKGROUND: Vestibular reflexes, evoked by human electrical (galvanic vestibular stimulation (EVS, are utilized to assess vestibular function and investigate its pathways. Our study aimed to investigate the electrically-evoked vestibulo-ocular reflex (eVOR output after bilateral and unilateral vestibular deafferentations to determine the characteristics for interpreting unilateral lesions such as vestibular schwannomas. METHODS: EVOR was recorded with dual-search coils as binocular three-dimensional eye movements evoked by bipolar 100 ms-step at EVS intensities of [0.9, 2.5, 5.0, 7.5, 10.0] mA and unipolar 100 ms-step at 5 mA EVS intensity. Five bilateral vestibular deafferented (BVD, 12 unilateral vestibular deafferented (UVD, four unilateral vestibular schwannoma (UVS patients and 17 healthy subjects were tested with bipolar EVS, and five UVDs with unipolar EVS. RESULTS: After BVD, bipolar EVS elicited no eVOR. After UVD, bipolar EVS of one functioning ear elicited bidirectional, excitatory eVOR to cathodal EVS with 9 ms latency and inhibitory eVOR to anodal EVS, opposite in direction, at half the amplitude with 12 ms latency, exhibiting an excitatory-inhibitory asymmetry. The eVOR patterns from UVS were consistent with responses from UVD confirming the vestibular loss on the lesion side. Unexpectedly, unipolar EVS of the UVD ear, instead of absent response, evoked one-third the bipolar eVOR while unipolar EVS of the functioning ear evoked half the bipolar response. CONCLUSIONS: The bidirectional eVOR evoked by bipolar EVS from UVD with an excitatory-inhibitory asymmetry and the 3 ms latency difference between normal and lesion side may be useful for detecting vestibular lesions such as UVS. We suggest that current spread could account for the small eVOR to 5 mA unipolar EVS of the UVD ear.

Cervical sympathetic chain schwannoma masquerading as a carotid body tumour with a postoperative complication of first-bite syndrome.

LENUS (Irish Health Repository)

Casserly, Paula

2012-01-31

Carotid body tumours (CBT) are the most common tumours at the carotid bifurcation. Widening of the bifurcation is usually demonstrated on conventional angiography. This sign may also be produced by a schwannoma of the cervical sympathetic plexus. A 45-year-old patient presented with a neck mass. Investigations included contrast-enhanced CT, MRI and magnetic resonance arteriography with contrast enhancement. Radiologically, the mass was considered to be a CBT due to vascular enhancement and splaying of the internal and external carotid arteries. Intraoperatively, it was determined to be a cervical sympathetic chain schwannoma (CSCS). The patient had a postoperative complication of first-bite syndrome (FBS).Although rare, CSCS should be considered in the differential diagnosis for tumours at the carotid bifurcation. Damage to the sympathetic innervation to the parotid gland can result in severe postoperative pain characterised by FBS and should be considered in all patients undergoing surgery involving the parapharyngeal space.

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

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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.

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

Long-Term Facial Nerve Outcomes after Microsurgical Resection of Vestibular Schwannomas in Patients with Preoperative Facial Nerve Palsy.

Science.gov (United States)

Mooney, Michael A; Hendricks, Benjamin; Sarris, Christina E; Spetzler, Robert F; Almefty, Kaith K; Porter, Randall W

2018-06-01

Objectives  This study aimed at evaluating facial nerve outcomes in vestibular schwannoma patients presenting with preoperative facial nerve palsy. Design  A retrospective review. Setting  Single-institution cohort. Participants  Overall, 368 consecutive patients underwent vestibular schwannoma resection. Patients with prior microsurgery or radiosurgery were excluded. Main Outcome Measures  Incidence, House-Brackmann grade. Results  Of 368 patients, 9 had confirmed preoperative facial nerve dysfunction not caused by prior treatment, for an estimated incidence of 2.4%. Seven of these nine patients had Koos grade 4 tumors. Mean tumor diameter was 3.0 cm (range: 2.1-4.4 cm), and seven of nine tumors were subtotally resected. All nine patients were followed up clinically for ≥ 6 months. Of the six patients with a preoperative House-Brackmann grade of II, two improved to grade I, three were stable, and one patient worsened to grade III. Of the three patients with grade III or worse, all remained stable at last follow-up. Conclusions  Preoperative facial nerve palsy is rare in patients with vestibular schwannoma; it tends to occur in patients with relatively large lesions. Detailed long-term outcomes of facial nerve function after microsurgical resection for these patients have not been reported previously. We followed nine patients and found that eight (89%) of the nine patients had either stable or improved facial nerve outcomes after treatment. Management strategies varied for these patients, including rates of subtotal versus gross-total resection and the use of stereotactic radiosurgery in patients with residual tumor. These results can be used to help counsel patients preoperatively on expected outcomes of facial nerve function after treatment.

Spinal tumours in neurofibromatosis type 1: an MRI study of frequency, multiplicity and variety

International Nuclear Information System (INIS)

Thakkar, S.D.; Mautner, V.F.; Feigen, U.

1999-01-01

In neurofibromatosis type 1 (NF1) spinal tumours cause neurological symptoms in about 2 % of patients. Among over 1400 patients with NF1 we saw symptomatic spinal tumours in 23 (1.6 %). MRI of the entire spinal canal was obtained in 54 patients aged 5-56 years with NF1. The number, site, morphology and signal characteristics of the spinal tumours were recorded and analysed. There were 24 patients with symptoms such as sensory impairment or paralysis; 30 patients had no neurological deficits. Of the 24 symptomatic patients, 23 (96 %) had spinal tumours, while we saw spinal tumours in 12 (40 %) of the 30 patients without neurological deficits. No spinal segment was preferred in symptomatic or asymptomatic patients. Most intraspinal extramedullary tumours were primarily extradural and intraforaminal. MRI showed intramedullary tumours in 3 patients (6 %), intraspinal extramedullary tumours in 18 (33 %) and intraforaminal tumours in 31 (57 %). Only neurological deficits in patients with NF1 should prompt further diagnostic clarification. In patients with neurological symptoms there may be a multiplicity of masses in the spinal canal, which can lead to difficulties in attaching symptoms to a certain tumour. In patients who do not satisfy the NIH criteria, it can be a helpful observation that spinal tumours in NF1 are primarily intraforaminal, extending into the spinal canal, while in NF2 they are mostly intraspinal intradural tumours. (orig.)

Atypical Central Neurocytoma with Recurrent Spinal Dissemination over a Period of 20 Years: A Case Report and Review of the Literature

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Tareq A. Juratli

2013-01-01

Full Text Available We present an unusual case of a late recurrent central neurocytoma that was rediagnosed as an ependymoma and neurocytoma in accordance with changes in histological classifications. Case Description. A 56-year-old male teacher presented with incomplete transverse syndrome due to several intradural extramedullary tumors at the level of lumbar vertebrae 1–3. The histological diagnosis at the time was atypical ependymoma. One year later, two additional tumors were removed at the L5-S1 vertebral level. For 12 years, the patient remained tumor free on followup. Fourteen years after the initial diagnosis, the patient presented with thoracic paresthesias due to two new extramedullary tumors in the C7-T1 and the T8-T9 vertebral levels. After complete removal of the tumors, a radiological survey revealed an intracranial lesion in the third ventricle. Five months later, an additional lesion recurrence was removed surgically. The most recent histological diagnosis revealed an atypical central neurocytoma. In retrospect, the previous tumors were reclassified as neurocytoma according to the additional immunohistochemistry evidence. Discussion. There is no standard adjuvant treatment regimen for atypical neurocytoma; therefore, the patient is currently under close followup. Modern histopathological diagnosis is essential in these cases. Potential routes for dissemination of the tumor should be considered upon first recurrence.

Sciatica from a Foraminal Lumbar Root Schwannoma: Case Report and Review of Literature

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Tarush Rustagi

2012-01-01

This case report describes lumbar foraminal schwannoma as an unusual cause of radiculopathy, presenting clinically as a lumbar disc prolapse. The diagnosis was confirmed on MRI scan. Patient had complete symptomatic recovery following surgical enucleation of the tumour mass from the L5 nerve root. This case report is of particular interest as it highlights the diagnostic confusion, which is bound to arise, because the clinical presentation closely mimics a lumbar PID. This often leads to delay in diagnosis and “failure of conservative treatment.”

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

Extramedullary Solitary Plasmacytoma: Demonstrating the Role of 18F-FDG PET Imaging.

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Gautam, Archana; Sahu, Kamal Kant; Alamgir, Ahsan; Siddiqi, Imran; Ailawadhi, Sikander

2017-04-01

An Extramedullary Plasmacytoma (EMP) is characterized by a neoplastic proliferation of clonal plasma cells outside the medullary cavity. EMPs are a rare occurrence compared to other malignant plasma cell disorders and account for approximately 3-5% of plasma-cell neoplasms. Although most cases of EMP are not immediately life threatening at diagnosis, EMPs can progress to Multiple Myeloma (MM) and thus, warrant monitoring. Currently, there are no standard guidelines for when and how to monitor patients who are diagnosed with or treated for a solitary plasmacytoma. We present a case of solitary EMP who was treated adequately and definitively but developed a distinct, non-contiguous subsequent solitary EMP and was only discovered due to surveillance 18 F-Fludeoxyglucose Positron Emission Tomography ( 18 F-FDG) (PET) scan. Uniform surveillance guidelines should be developed and the potential benefits of PET and other imaging techniques as well as their cost should be considered.

Isolated spinal accessory neuropathy and intracisternal schwannomas of the spinal accessory nerve

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Abdullah M. Al-Ajmi

2015-03-01

Full Text Available We report a 40-year-old female patient presenting with isolated left spinal accessory neuropathy that developed insidiously over 6 years. She complained of ill-defined deep neck and shoulder pain. On examination, prominent sternocleidomastoid and trapezoid muscle weakness and atrophy, shoulder instability, and lateral scapular winging were observed. MRI identified a small mass of the cisternal portion of the spinal accessory nerve. Its appearance was typical of schwannoma. Surgical treatment was not offered because of the small tumor size, lack of mass effect and the questionable functional recovery in the presence of muscular atrophy.

Fine needle aspiration cytology in feline skeletal muscle as a diagnostic tool for extramedullary plasmacytoma

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D.B. Martins

Full Text Available ABSTRACT Extramedullary noncutaneous plasmacytoma (ENP is a myeloproliferative disorder of plasma cells that rarely affects cats. This paper describes an ENP case revealed by fine needle aspiration cytology (FNAC of the mass in the skeletal muscle of an 8-month-old, male, mixed breed cat, which had a nodule in the left hind limb. The rapid immunoassay test confirmed the presence of feline leukemia virus (FeLV. The animal necropsy macroscopically showed the nodule came from the semimembranosus muscle. Histopathological examination ratified the cytological findings. Thus, this paper alerts to the existence of plasmacytoma located in the skeletal muscle of feline species. FNAC is a quick and efficient method for diagnosis of ENP.

Tissue-Specific Ablation of Prkar1a Causes Schwannomas by Suppressing Neurofibromatosis Protein Production

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Georgette N. Jones

2008-11-01

Full Text Available Signaling events leading to Schwann cell tumor initiation have been extensively characterized in the context of neurofibromatosis (NF. Similar tumors are also observed in patients with the endocrine neoplasia syndrome Carney complex, which results from inactivating mutations in PRKAR1A. Loss of PRKAR1A causes enhanced protein kinase A activity, although the pathways leading to tumorigenesis are not well characterized. Tissue-specific ablation of Prkar1a in neural crest precursor cells (TEC3KO mice causes schwannomas with nearly 80% penetrance by 10 months. These heterogeneous neoplasms were clinically characterized as genetically engineered mouse schwannomas, grades II and III. At the molecular level, analysis of the tumors revealed almost complete loss of both NF proteins, despite the fact that transcript levels were increased, implying posttranscriptional regulation. Although Erk and Akt signaling are typically enhanced in NF-associated tumors, we observed no activation of either of these pathways in TEC3KO tumors. Furthermore, the small G proteins Ras, Rac1, and RhoA are all known to be involved with NF signaling. In TEC3KO tumors, all three molecules showed modest increases in total protein, but only Rac1 showed significant activation. These data suggest that dysregulated protein kinase A activation causes tumorigenesis through pathways that overlap but are distinct from those described in NF tumorigenesis.

Definitive radiotherapy for extramedullary plasmacytomas of the head and neck.

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Michalaki, V J; Hall, J; Henk, J M; Nutting, C M; Harrington, K J

2003-10-01

Extramedullary plasmacytoma of the head and neck region (EMPHN) is an uncommon malignant plasma cell neoplasm. In this study we conducted a retrospective analysis of our experience of EMPHN with particular emphasis on the role of definitive radiotherapy. From 1982 to 2001, 10 patients (6 males, 4 females) with EMPHN were treated in our institution. Of nine patients treated at initial diagnosis, all received definitive radiotherapy. One patient treated at relapse underwent surgical resection followed by post-operative radiotherapy. The median age at diagnosis was 55 years (range 35-84 years). The disease was most frequently localized in the paranasal sinuses (50%). All nine patients who received definitive radiotherapy at a dose of 40-50 Gy achieved a complete response. The median follow up period was 29 months (range 7-67 months). Four patients (40%) relapsed, three have died of their disease. Two patients (20%) with paranasal sinus disease subsequently relapsed with multiple myeloma at 10 months and 24 months, respectively. Our results indicate that treatment of EMPHN with radiotherapy achieves excellent rates of local control. The relapse rate in neck nodes of 10% does not justify elective irradiation of the uninvolved neck.

[Spinal cord compression due to extramedullary hematopoiesis in a patient with myelofibrosis].

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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.

Combination Therapy with c-Met and Src Inhibitors Induces Caspase-Dependent Apoptosis of Merlin-Deficient Schwann Cells and Suppresses Growth of Schwannoma Cells.

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Fuse, Marisa A; Plati, Stephani Klingeman; Burns, Sarah S; Dinh, Christine T; Bracho, Olena; Yan, Denise; Mittal, Rahul; Shen, Rulong; Soulakova, Julia N; Copik, Alicja J; Liu, Xue Zhong; Telischi, Fred F; Chang, Long-Sheng; Franco, Maria Clara; Fernandez-Valle, Cristina

2017-11-01

Neurofibromatosis type 2 (NF2) is a nervous system tumor disorder caused by inactivation of the merlin tumor suppressor encoded by the NF2 gene. Bilateral vestibular schwannomas are a diagnostic hallmark of NF2. Mainstream treatment options for NF2-associated tumors have been limited to surgery and radiotherapy; however, off-label uses of targeted molecular therapies are becoming increasingly common. Here, we investigated drugs targeting two kinases activated in NF2-associated schwannomas, c-Met and Src. We demonstrated that merlin-deficient mouse Schwann cells (MD-MSC) treated with the c-Met inhibitor, cabozantinib, or the Src kinase inhibitors, dasatinib and saracatinib, underwent a G 1 cell-cycle arrest. However, when MD-MSCs were treated with a combination of cabozantinib and saracatinib, they exhibited caspase-dependent apoptosis. The combination therapy also significantly reduced growth of MD-MSCs in an orthotopic allograft mouse model by greater than 80% of vehicle. Moreover, human vestibular schwannoma cells with NF2 mutations had a 40% decrease in cell viability when treated with cabozantinib and saracatinib together compared with the vehicle control. This study demonstrates that simultaneous inhibition of c-Met and Src signaling in MD-MSCs triggers apoptosis and reveals vulnerable pathways that could be exploited to develop NF2 therapies. Mol Cancer Ther; 16(11); 2387-98. ©2017 AACR . ©2017 American Association for Cancer Research.

A rare case of cervical epidural extramedullary plasmacytoma presenting with monoparesis

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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.

Extreme lateral approach in a case of acute-onset quadriplegia due to high cervical neurenteric cyst.

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Abhishek, Agrawal; Anushree, Agrawal; Patir, Rana; Sehgal, A D

2007-01-01

Spinal neurenteric cysts are very rare intradural developmental lesions, predominantly localized anterior to the cervical cord and arising from misplaced endodermal cells in the 3rd week of embryonic life. An acute onset of symptoms can occur due to hemorrhage, but has not as yet been reported in relation to infection in the cyst. We report an 18-year-old male who presented with a rapid onset of respiratory distress and quadriplegia over a period of 6 h requiring intubation and ventilatory support. There was no respiratory effort with a dense sensory level from the neck. MRI of the cervical spine revealed an intradural extramedullary ventrally located cystic lesion at the C(2-3) level. There was no evidence of systemic infection elsewhere in the body. The lesion was radically excised using an extreme lateral approach as it provided excellent visualization of the cyst, thus permitting safe dissection without retraction of the cord. The cyst was tense and the contents turbid, thick, creamy and mucinous without any evidence of fresh or altered blood. Histopathological examination confirmed the cyst to be a neurenteric cyst. The cystic fluid, which was sent for culture, grew coagulase-negative Staphylococcus aureus. This is the first documented case of rapid neurological deterioration due to infection in a neurenteric cyst. In spite of an early effective surgical management, outcome was poor due to the rapid and profound neurological deficit. Copyright (c) 2007 S. Karger AG, Basel.

Primary multifocal gliosarcoma of the spinal cord

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Ramesh M. Kumar

2016-03-01

Full Text Available Gliosarcoma (GS is a rare and exceedingly malignant neoplasm of the central nervous system. It displays clinical features similar to glioblastoma, yet is histologically unique as it harbors both gliomatous and sarcomatous cellular components. Involvement of the neuroaxis is predominantly limited to the cerebral parenchyma and meninges. Primary GS of the spinal cord is rarely encountered. We report a case of a 54 year old male who presented with 2 months of progressive, bilateral lower extremity sensory deficits. Magnetic resonance imaging of the neuro-axis revealed multiple intradural lesions involving the cervical and thoracic spinal cord without evidence of intracranial involvement. Surgical resection of a dural based, extramedullary cervical lesion and two exophytic, intramedullary thoracic lesions revealed gliosarcoma, WHO grade IV. The patient died approximately 11 months after presentation. This report confirms that GS is not limited to supratentorial involvement and can primarily affect the spinal cord.

Serial MRI of vestibular schwannoma presenting transient enlargement associated with intratumoral hemorrhage following radiosurgery. Two case reports

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Kikuchi, Yasuhiro; Watanabe, Kazuo; Goto, Hiromi; Isaki, Kenji; Sato, Sonomi

2010-01-01

Two cases of vestibular schwannoma showing transient enlargement with hemorrhage that was recognized on T2 or T2*-weighted MR image are reported. Case 1: A 72-year-old woman had a recurrent cystic lesion after resection and stereotactic radiosurgery (SRS). After retreatment by gamma knife radiosurgery, the cystic component further expanded with a fluid-fluid level, and T2 or T2*-weighted image showed markedly-low signal in the parenchymal component. Chronic hemorrhage from the cyst wall injured by irradiation is probably related to expansion of the cyst. Case 2: A 59-year-old woman received SRS. Serial MRI scans demonstrated a lesion continually expanding with homogeneous contrast enhancement on T1-weighted images. At 64 months after treatment, a central portion with high blood flow surrounded by a markedly-low intensity component of apparent hemorrhage on T2*-weighted MRI was identified. The lesion decreased in size after 73 months. Hemorrhage might occur from the neogenetic angioma-like tissue induced by irradiation effect in the tumor. Based on these observations, microvasculopathy associated with irradiation is a probable mechanism that induces bleeding in vestibular schwannoma following radiosurgery. (author)

Audiovestibular Function Deficits in Vestibular Schwannoma

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Constantin von Kirschbaum

2016-01-01

Full Text Available Introduction. Vestibular schwannomas (VS are benign tumours of the vestibular nerve and can lead to hearing loss, tinnitus, vertigo, facial palsy, and brainstem compression. Audiovestibular diagnostic tests are essential for detection and treatment planning. Methods. Medline was used to perform a systematic literature review with regard to how audiovestibular test parameters correlate with symptoms, tumour size, and tumour location. Results. The auditory brainstem response can be used to diagnose retrocochlear lesions caused by VS. Since hearing loss correlates poorly with tumour size, a retrocochlear lesion is probably not the only cause for hearing loss. Also cochlear mechanisms seem to play a role. This can be revealed by abnormal otoacoustic emissions, despite normal ABR and new MRI techniques which have demonstrated endolymphatic hydrops of the inner ear. Caloric and head impulse tests show frequency specific dynamics and vestibular evoked myogenic potentials may help to identify the location of the tumour regarding the involved nerve parts. Conclusion. In order to preserve audiovestibular function in VS, it is important to stop the growth of the tumour and to avoid degenerative changes in the inner ear. A detailed neurotological workup helps to diagnose VS of all sizes and can also provide useful prognostic information.

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

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

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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

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

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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.

Stereotactic radiosurgery versus stereotactic radiotherapy for patients with vestibular schwannoma: a Leksell Gamma Knife Society 2000 debate.

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Linskey, Mark E

2013-12-01

By definition, the term "radiosurgery" refers to the delivery of a therapeutic radiation dose in a single fraction, not simply the use of stereotaxy. Multiple-fraction delivery is better termed "stereotactic radiotherapy." There are compelling radiobiological principles supporting the biological superiority of single-fraction radiation for achieving an optimal therapeutic response for the slowly proliferating, late-responding, tissue of a schwannoma. It is axiomatic that complication avoidance requires precise three-dimensional conformality between treatment and tumor volumes. This degree of conformality can only be achieved through complex multiisocenter planning. Alternative radiosurgery devices are generally limited to delivering one to four isocenters in a single treatment session. Although they can reproduce dose plans similar in conformality to early gamma knife dose plans by using a similar number of isocenters, they cannot reproduce the conformality of modern gamma knife plans based on magnetic resonance image--targeted localization and five to 30 isocenters. A disturbing trend is developing in which institutions without nongamma knife radiosurgery (GKS) centers are championing and/or shifting to hypofractionated stereotactic radiotherapy for vestibular schwannomas. This trend appears to be driven by a desire to reduce complication rates to compete with modern GKS results by using complex multiisocenter planning. Aggressive advertising and marketing from some of these centers even paradoxically suggests biological superiority of hypofractionation approaches over single-dose radiosurgery for vestibular schwannomas. At the same time these centers continue to use the term radiosurgery to describe their hypofractionated radiotherapy approach in an apparent effort to benefit from a GKS "halo effect." It must be reemphasized that as neurosurgeons our primary duty is to achieve permanent tumor control for our patients and not to eliminate complications at the

Magnetic resonance cisternography using the fast spin echo method for the evaluation of vestibular schwannoma

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Nishizawa, Shigeru; Yokoyama, Tetsuo; Uemura, Kenichi [Hamamatsu Univ. School of Medicine, Shizuoka (Japan)

1999-04-01

Neuroimaging of vestibular schwannoma was performed with the fat-suppression spoiled gradient recalled acquisition in the steady state (SPGR) method and magnetic resonance (MR) cisternography, which is a fast spin echo method using a long echo train length, for the preoperative evaluation of the lateral extension of the tumor in the internal auditory canal, and the anatomical identification of the posterior semicircular canal and the nerves in the canal distal to the tumor. The SPGR method overestimated the lateral extension in eight cases, probably because of enhancement of the nerves adjacent to the tumor in the canal. The posterior semicircular canal could not be clearly identified, and the cranial nerves in the canal were shown only as a nerve bundle. In contrast, MR cisternography showed clear images of the lateral extension of the tumor and the facial and cochlear nerves adjacent to the tumor in the internal auditory canal. The anatomical location of the posterior semicircular canal was also clearly shown. These preoperative findings are very useful to plan the extent to which the internal auditory canal can be opened, and for intraoperative identification of the nerves in the canal. MR cisternography is less invasive since no contrast material or radiation is required, as with thin-slice high-resolution computed tomography (CT). MR cisternography should replace high-resolution CT for the preoperative neuroradiological evaluation of vestibular schwannoma. (author)

Increased signal intensity of the cochlea on pre- and post-contrast enhanced 3D-FLAIR in patients with vestibular schwannoma

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Yamazaki, Masahiro; Naganawa, Shinji; Kawai, Hisashi; Nihashi, Takashi [Nagoya University, Department of Radiology, Graduate School of Medicine, Nagoya (Japan); Fukatsu, Hiroshi [Aichi Medical University Hospital, Department of Medical Informatics, Nagakute (Japan); Nakashima, Tsutomu [Nagoya University, Department of Otorhinolaryngology, Graduate School of Medicine, Nagoya (Japan)

2009-12-15

In the vestibular schwannoma patients, the pathophysiologic mechanism of inner ear involvement is still unclear. We investigated the status of the cochleae in patients with vestibular schwannoma by evaluating the signal intensity of cochlear fluid on pre- and post-contrast enhanced thin section three-dimensional fluid-attenuated inversion recovery (3D-FLAIR). Twenty-eight patients were retrospectively analyzed. Post-contrast images were obtained in 18 patients, and 20 patients had the records of their pure-tone audiometry. Regions of interest of both cochleae (C) and of the medulla oblongata (M) were determined on 3D-FLAIR images by referring to 3D heavily T2-weighted images on a workstation. The signal intensity ratio between C and M on the 3D-FLAIR images (CM ratio) was then evaluated. In addition, correlation between the CM ratio and the hearing level was also evaluated. The CM ratio of the affected side was significantly higher than that of the unaffected side (p < 0.001). In the affected side, post-contrast signal elevation was observed (p < 0.005). In 13 patients (26 cochleae) who underwent both gadolinium injection and the pure-tone audiometry, the post-contrast CM ratio correlated with hearing level (p < 0.05). The results of the present study suggest that alteration of cochlear fluid composition and increased permeability of the blood-labyrinthine barrier exist in the affected side in patients with vestibular schwannoma. Furthermore, although weak, positive correlation between post-contrast cochlear signal intensity on 3D-FLAIR and hearing level warrants further study to clarify the relationship between 3D-FLAIR findings and prognosis of hearing preservation surgery. (orig.)

Increased signal intensity of the cochlea on pre- and post-contrast enhanced 3D-FLAIR in patients with vestibular schwannoma

International Nuclear Information System (INIS)

Yamazaki, Masahiro; Naganawa, Shinji; Kawai, Hisashi; Nihashi, Takashi; Fukatsu, Hiroshi; Nakashima, Tsutomu

2009-01-01

In the vestibular schwannoma patients, the pathophysiologic mechanism of inner ear involvement is still unclear. We investigated the status of the cochleae in patients with vestibular schwannoma by evaluating the signal intensity of cochlear fluid on pre- and post-contrast enhanced thin section three-dimensional fluid-attenuated inversion recovery (3D-FLAIR). Twenty-eight patients were retrospectively analyzed. Post-contrast images were obtained in 18 patients, and 20 patients had the records of their pure-tone audiometry. Regions of interest of both cochleae (C) and of the medulla oblongata (M) were determined on 3D-FLAIR images by referring to 3D heavily T2-weighted images on a workstation. The signal intensity ratio between C and M on the 3D-FLAIR images (CM ratio) was then evaluated. In addition, correlation between the CM ratio and the hearing level was also evaluated. The CM ratio of the affected side was significantly higher than that of the unaffected side (p < 0.001). In the affected side, post-contrast signal elevation was observed (p < 0.005). In 13 patients (26 cochleae) who underwent both gadolinium injection and the pure-tone audiometry, the post-contrast CM ratio correlated with hearing level (p < 0.05). The results of the present study suggest that alteration of cochlear fluid composition and increased permeability of the blood-labyrinthine barrier exist in the affected side in patients with vestibular schwannoma. Furthermore, although weak, positive correlation between post-contrast cochlear signal intensity on 3D-FLAIR and hearing level warrants further study to clarify the relationship between 3D-FLAIR findings and prognosis of hearing preservation surgery. (orig.)

Chondromyxoid fibroma of the mastoid facial nerve canal mimicking a facial nerve schwannoma.

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Thompson, Andrew L; Bharatha, Aditya; Aviv, Richard I; Nedzelski, Julian; Chen, Joseph; Bilbao, Juan M; Wong, John; Saad, Reda; Symons, Sean P

2009-07-01

Chondromyxoid fibroma of the skull base is a rare entity. Involvement of the temporal bone is particularly rare. We present an unusual case of progressive facial nerve paralysis with imaging and clinical findings most suggestive of a facial nerve schwannoma. The lesion was tubular in appearance, expanded the mastoid facial nerve canal, protruded out of the stylomastoid foramen, and enhanced homogeneously. The only unusual imaging feature was minor calcification within the tumor. Surgery revealed an irregular, cystic lesion. Pathology diagnosed a chondromyxoid fibroma involving the mastoid portion of the facial nerve canal, destroying the facial nerve.

Hydroxyurea as a first-line treatment of extramedullary hematopoiesis in patients with beta thalassemia: Four case reports.

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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.

Preclinical Validation of Anti-Nuclear Factor Kappa B Therapy Against Vestibular Schwannoma and Neurofibromatosis Type II

Science.gov (United States)

2016-06-01

renal failure. Pharmacologic treatment of NF2 represents a major unmet medical need. The hallmark of NF2 is bilateral vestibular schwannomas (VSs...it to test our leading hypothesis over the next year. Innovation: Our focus on NFκB as a potent pharmacologic target for NF2 is novel. Our work...diagnostic awareness among clinicians (4,5). Contrast-enhanced magnetic resonance imaging ( MRI ) of the brain is currently the gold standard for

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.

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

Long-term follow-up reveals low toxicity of radiosurgery for vestibular schwannoma

International Nuclear Information System (INIS)

Rutten, Isabelle; Baumert, Brigitta G.; Seidel, Laurence; Kotolenko, Snezana; Collignon, Jacques; Kaschten, Bruno; Albert, Adelin; Martin, Didier; Deneufbourg, Jean-Marie; Demanez, Jean-Pierre; Stevenaert, Achille

2007-01-01

Aim: The long-term effects of radiosurgery of vestibular schwannomas were investigated in a group of consecutively treated patients. Methods and materials: Between 1995 and 2001, 26 patients (median age: 67, range: 30-82) with a vestibular schwannoma were treated by Linac-based stereotactic radiosurgery (SRS). The median follow-up was 49 months (16-85 months). Only progressive tumours were treated. The median size of tumours was 18 mm (range 9-30 mm). Before SRS, 11 patients had a useful hearing (Gardner-Robertson classes 1 and 2). Single doses of 10-14 Gy were prescribed at the 80% isodose at the tumour margin. The follow-up consisted of regular imaging with MRI the first 3-6 months after the intervention, followed by additional yearly MRIs, a hearing test and a neurological examination. Result: The 5-year-probability of tumour control (defined as stabilization or decrease in size) was 95%. Five-year-probability of preservation of hearing and facial nerve function was 96% and 100%, respectively. Hearing was preserved in 10 out of 11 patients who had a normal or useful hearing at the time of treatment. Mild and transient trigeminal toxicity occurred in 2 (8%) patients. It appeared to be significantly correlated to the dose used (p = 0.044). However, only a tendency to significance could be demonstrated in the relationship between the two factors when using the Cox analysis (hazard ratio = 1.7; 95% CI: 0.7-3.9; p = 0.23). Conclusions: With the doses used, our study demonstrates that SRS provides an equivalent tumour control rate when compared to surgery, as well as on a long-term basis, an excellent preservation of the facial and the acoustic nerves. Although no permanent trigeminal toxicity was observed, our data confirm that doses below 14 Gy can avoid transient dysesthesias

Stereotactic radiosurgery vs. fractionated radiotherapy for tumor control in vestibular schwannoma patients

DEFF Research Database (Denmark)

Persson, Oscar; Bartek, Jiri; Shalom, Netanel Ben

2017-01-01

OBJECTIVE: Repeated controlled studies have revealed that stereotactic radiosurgery is better than microsurgery for patients with vestibular schwannoma (VS) ... to patients treated with fractionated stereotactic radiotherapy. RESULTS: No randomized controlled trial (RCT) was identified. None of the identified controlled studies comparing SRS with FSRT were eligible according to the inclusion criteria. Nineteen case series on SRS (n = 17) and FSRT (n = 2) were...... included in the systematic review. Loss of tumor control necessitating a new VS-targeted intervention was found in an average of 5.0% of the patients treated with SRS and in 4.8% treated with FSRT. Mean deterioration ratio for patients with serviceable hearing before treatment was 49% for SRS and 45...

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

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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.

Clinical experience with vestibular schwannomas: epidemiology, symptomatology, diagnosis, and surgical results.

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Tos, M; Charabi, S; Thomsen, J

1998-01-01

The Danish model for vestibular schwannoma (VS) surgery has been influenced by some historical otological events, taking its origin in the fact that the first attempt to remove CPA tumors was performed by an otologist in 1916. In approximately 50 years VS surgery was performed by neurosurgeons in a decentralized model. Highly specialized neuro- and otosurgeons have been included in our team since the early beginning of the centralized Danish model of VS surgery in 1976. Our surgical practice has always been performed on the basis of known and proven knowledge, but we spared no effort to search for innovative procedures. The present paper reflects the experience we have gained in two decades of VS surgery. Our studies on the incidence, symptomatology, diagnosis, expectancy and surgical results are presented.

Magnetic resonance imaging of intramedullary meningioma of the spinal cord: case report and review of the literature

International Nuclear Information System (INIS)

Covert, S.; Gandhi, D.; Goyal, M.; Woulfe, J.; Belanger, E.; Miller, W.; Modha, A.

2003-01-01

Intramedullary meningioma of the spinal cord is an extremely rare tumour. To the best of our knowledge, only 4 cases have been reported in the English literature, and limited information on imaging features is available. We present a further case and review the literature on this entity. Meningiomas are among the most common tumours that occur in the spinal canal, representing about 25% of all adult intraspinal tumours. They are commonly found in the thoracic segment of the spine and are most often intradural-extramedullary. Their location is explained by the way in which they develop. Meningiomas are derived from persistent arachnoid cell remnants in the spinal coverings, most commonly from arachnoid. They are usually adherent to, but do not arise from, dura. We present a case of intramedullary meningioma, a rare clinical entity, discuss the magnetic resonance imaging (MRI) findings and review the 4 cases that have been previously reported. (author)

Perianal pain as a presentation of lumbosacral neurofibroma: a case report.

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Moghaddasi, Mehdi; Aghaii, Mahboubeh; Mamarabadi, Mansoureh

2014-12-01

Rectal and perianal pain is a common problem. Most people have experienced it at least once in their lifetime. It usually manifests as mild discomfort, but sometimes the pain can be so severe that it is incapacitating. A 59-year-old woman admitted with a 2-year history of paroxysmal perianal pain underwent a full work-up including proctoscopy, sigmoidoscopy, full colonoscopy, and barium enema that were unremarkable. Lumbosacral magnetic resonance imaging with and without gadolinium showed an intradural-extramedullary lesion at the level of L5. The pathologic diagnosis was a neurofibroma. She underwent surgery, and after a few weeks she felt well and medication was no longer needed for her paroxysmal pain. Although one should consider the usual causes of colorectal pain such as hemorrhoids, anal fissure, proctalgia fugax, and chronic perianal pain syndrome, we should keep in mind that some referral pain may mimic local pathologies and should be evaluated properly.

Multifocal Spinal Cord Nephroblastoma in a Dog.

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Henker, L C; Bianchi, R M; Vargas, T P; de Oliveira, E C; Driemeier, D; Pavarini, S P

2018-01-01

A 1-year-old male American pit bull terrier was presented with a history of proprioceptive deficits and mild lameness of the right hindlimb, which progressed after 5 months to paraparesis, culminating in tetraparesis after 2 weeks. Necropsy findings were limited to the spinal cord and consisted of multiple, intradural, extramedullary, slightly red masses which produced segmental areas of medullary swelling located in the cervical intumescence, thoracolumbar column, sacral segment and cauda equina. Histological evaluation revealed a tumour, composed of epithelial, stromal and blastemal cells, with structures resembling tubules, acini and embryonic glomeruli. Immunohistochemical labelling for vimentin, cytokeratin and S100 was positive for the stromal, epithelial and blastemal cells, respectively. A final diagnosis of multifocal spinal cord nephroblastoma was established. This is the first report of such a tumour showing concomitant involvement of the cervicothoracic, thoracolumbar, sacral and cauda equina areas of the spinal cord. Copyright © 2017 Elsevier Ltd. All rights reserved.

Clinicopathological variables of sporadic schwannomas of peripheral nerve in 291 patients and expression of biologically relevant markers.

Science.gov (United States)

Young, Eric D; Ingram, Davis; Metcalf-Doetsch, William; Khan, Dilshad; Al Sannaa, Ghadah; Le Loarer, Francois; Lazar, Alexander J F; Slopis, John; Torres, Keila E; Lev, Dina; Pollock, Raphael E; McCutcheon, Ian E

2017-09-08

OBJECTIVE While sporadic peripheral schwannomas (SPSs) are generally well treated with surgery, their biology is not well understood. Consequently, treatment options are limited. The aim of this study was to provide a comprehensive description of SPS. The authors describe clinicopathological features and treatment outcomes of patients harboring these tumors, and they assess expression of biomarkers using a clinically annotated tissue microarray. Together, these data give new insight into the biology and management of SPS. METHODS Patients presenting with a primary SPS between 1993 and 2011 (n = 291) were selected from an institutional registry to construct a clinical database. All patients underwent follow-up, and short- and long-term outcomes were assessed. Expression of relevant biomarkers was assessed using a new tissue microarray (n = 121). RESULTS SPSs were generally large (mean 5.5 cm) and frequently painful at presentation (55%). Most patients were treated with surgery (80%), the majority of whom experienced complete resolution (52%) or improvement (18%) of their symptoms. Tumors that were completely resected (85%) did not recur. Some patients experienced short-term (16%) and long-term (4%) complications postoperatively. Schwannomas expressed higher levels of platelet-derived growth factor receptor-β (2.1) than malignant peripheral nerve sheath tumors (MPNSTs) (1.5, p = 0.004) and neurofibromas (1.33, p = 0.007). Expression of human epidermal growth factor receptor-2 was greater in SPSs (0.91) than in MPNSTs (0.33, p = 0.002) and neurofibromas (0.33, p = 0.026). Epidermal growth factor receptor was expressed in far fewer SPS cells (10%) than in MPNSTs (58%, p SPSs more frequently expressed cytoplasmic survivin (66% of tumor cells) than normal nerve (46% of cells), but SPS expressed nuclear survivin in fewer tumor cells than in MPNSTs (24% and 50%, respectively; p = 0.018). CONCLUSIONS Complete resection is curative for SPS. Left untreated, however, these

Infiltración extramedular en la leucemia mielomonocítica crónica (LMMC Extramedullary infiltration in chronic myelomonocytic leukemia (CMML

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Onel Ávila Cabrera

2008-12-01

Full Text Available La leucemia mielomonocítica crónica (LMMC constituye un proceso oncohematológico de naturaleza mixta, mieloproliferativa y mielodisplásica. Su forma habitual de presentación es consecuencia, generalmente, de las citopenias en sangre periférica (síndrome anémico, infecciones o diátesis hemorrágica. La infiltración extramedular en la LMMC frecuentemente involucra el bazo, hígado, piel y nódulos linfáticos; sin embargo, es poco frecuente su localización en otros sitios. Se describe un paciente de 70 años con el diagnóstico de LMMC con infiltración extramedular en piel (nódulos subcutáneos, paladar duro y testículos; se comentan aspectos diagnósticos, terapéuticos y evolutivos.Chronic myelomonocytic leukemia is an oncohematological process of mixed, myeloproliferative and myelodisplastic nature. Its habitual form of presentation generally results from cytopenias in peripheral blood (anemic syndrome, infections or hemorrhagic diathesis. The extramedullary infiltration in the CMML frequently involves the spleen, the liver, the skin and the lymphatic nodules; however, its localization in other sites is not usual. The case of a 70-year-old patient with diagnosis of CMML and with extramedullary infiltration in the skin (subcutaneous nodules, hard palate and testicles is described. Comments on diagnostic, therapeutic and evolutive aspects are made

Facial Nerve Paralysis due to a Pleomorphic Adenoma with the Imaging Characteristics of a Facial Nerve Schwannoma

OpenAIRE

Nader, Marc-Elie; Bell, Diana; Sturgis, Erich M.; Ginsberg, Lawrence E.; Gidley, Paul W.

2014-01-01

Background Facial nerve paralysis in a patient with a salivary gland mass usually denotes malignancy. However, facial paralysis can also be caused by benign salivary gland tumors. Methods We present a case of facial nerve paralysis due to a benign salivary gland tumor that had the imaging characteristics of an intraparotid facial nerve schwannoma. Results The patient presented to our clinic 4 years after the onset of facial nerve paralysis initially diagnosed as Bell palsy. Computed tomograph...

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.

C-X-C motif chemokine 12 influences the development of extramedullary hematopoiesis in the spleens of myelofibrosis patients.

Science.gov (United States)

Wang, Xiaoli; Cho, Sool Yeon; Hu, Cing Siang; Chen, Daniel; Roboz, John; Hoffman, Ronald

2015-02-01

Myelofibrosis (MF) is characterized by the constitutive mobilization of hematopoietic stem cells (HSC) and hematopoietic progenitor cells (HPC) and the establishment of extramedullary hematopoiesis. The mechanisms underlying this abnormal HSC/HPC trafficking pattern remain poorly understood. We demonstrated that both splenic and peripheral blood (PB) MF CD34(+) cells equally share a defective ability to home to the marrow, but not to the spleens, of NOD/LtSz-Prkdc(scid) mice. This trafficking pattern could not be attributed to discordant expression of integrins or chemokine receptors other than the downregulation of C-X-C chemokine receptor type 4 by both PB and splenic MF CD34(+) cells. The number of both splenic MF CD34(+) cells and HPCs that migrated toward splenic MF plasma was, however, significantly greater than the number that migrated toward PB MF plasma. The concentration of the intact HSC/HPC chemoattractant C-X-C motif chemokine 12 (CXCL12) was greater in splenic MF plasma than PB MF plasma, as quantified using mass spectrometry. Functionally inactive truncated products of CXCL12, which are the product of proteolytic degradation by serine proteases, were detected at similar levels in both splenic and PB MF plasma. Treatment with an anti-CXCL12 neutralizing antibody resulted in a reduction in the degree of migration of splenic MF CD34(+) cells toward both PB and splenic MF plasma, validating the role of CXCL12 as a functional chemoattractant. Our data indicate that the MF splenic microenvironment is characterized by increased levels of intact, functional CXCL12, which contributes to the localization of MF CD34(+) cells to the spleen and the establishment of extramedullary hematopoiesis. Copyright © 2015 ISEH - International Society for Experimental Hematology. Published by Elsevier Inc. All rights reserved.

Fluorine-18 fluorodeoxyglucose splenic uptake from extramedullary hematopoiesis after granulocyte colony-stimulating factor stimulation.

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Abdel-Dayem, H M; Rosen, G; El-Zeftawy, H; Naddaf, S; Kumar, M; Atay, S; Cacavio, A

1999-05-01

Two patients with sarcoma, one with recurrent osteosarcoma of the spine and the other with metastatic synovial cell sarcoma, were treated with high-dose chemotherapy that produced severe leukopenia. The patients received granulocyte colony-stimulating factor (G-CSF) to stimulate the bone marrow (480 mg given subcutaneously twice daily for 5 to 7 days); their responses were seen as a marked increase in peripheral leukocyte count with no change in the erythrocyte or platelet counts. The patients had fluorine-18 fluorodeoxyglucose (F-18 FDG) imaging 24 hours after the end of G-CSF treatment. Diffusely increased uptake of F-18 FDG was seen in the bone marrow in both patients. In addition, markedly increased uptake in the spleen was noted in both, indicating that the spleen was the site of extramedullary hematopoiesis. The patients had no evidence of splenic metastases. The first patient had a history of irradiation to the dorsal spine, which was less responsive to G-CSF administration than was the nonirradiated lumbar spine.

A persistent cough as atypical clinical presentation of intrathoracic extramedullary hematopoiesis (EMH) in a female with thalassemia intermedia.

Science.gov (United States)

Abdulla, Mohammad Aj; Yassin, Mohamed A; Abdelrazek, Mohamed; Mudawi, Deena; Ibrahim, Firyal; Soliman, Dina S; ElOmri, Halima; Nashwan, Abdulqadir J; Fernihough, Liam; De Sanctis, Vincenzo; Soliman, Ashraf T

2018-02-16

Extramedullary hematopoiesis (EMH) is a rare disorder, defined as the appearance of hematopoietic elements outside the bone marrow or peripheral blood. The most common sites of EMH are liver and spleen, but it has been documented in other organs such as the mediastinum, lymph nodes, breast, and central nervous system. EMH occurs as a compensatory mechanism for bone marrow dysfunction in severe thalassemia. We report a case of EMH presenting as a posterior mediastinal mass in a 34-year-old woman with thalassemia intermedia with chronic cough and shortness of breath on exertion. The diagnosis of EMH was confirmed by a CT-guided fine needle biopsy. All symptoms disappeared after surgical removal of the mass.

Extramedullary Plasmacytoma of the Larynx: A Case Report of Subglottic Localization

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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.

Selection of treatment and surgical approach for vestibular schwannomas

International Nuclear Information System (INIS)

Eguchi, Kuniki; Yamaguchi, Satoshi; Sakoda, Eiichiro

2007-01-01

Described are the present state of selection of stereotactic radiotherapy or surgical treatment and their combination for schwannomas in the title, and authors' policy of surgery as a first choice treatment. The policy stands on the concept that surgery is useful for the controllability thereafter of the tumor, of which size is at first larger than 25 mm diameter, and radiotherapy like a gamma-knife is applicable to the residual tissue grown after operation and to the tissue with less than the size before surgery because the smaller the size, the better is thought the control by the knife (reportedly 100% for the tissue of <14 mm diameter). The basis of authors' selection of two surgical approaches of through-lower lateral occiput and trans-labyrinthine, and their outcomes like hearing loss are described and discussed in details for 24 patients (two underwent radiotherapy before surgery) during the period Sep. 2003-Aug. 2006 of authors' hospital. Radiotherapy is thought essentially useful for the control of the tumor with a small or surgically reduced size. (R.T.)

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.

A giant plexiform schwannoma of the brachial plexus: case report

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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.

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.

Pediatric intraspinal neoplasms; Paediatrische intraspinale Neoplasien

Energy Technology Data Exchange (ETDEWEB)

Mueller, U.; Ahlhelm, F. [Kantonsspital Baden AG, Institut fuer Radiologie, Baden (Switzerland); Ulmer, S. [Medizinisch-Radiologisches Institut, Zuerich (Switzerland); Schlaeger, R. [Universitaetsspital Basel, Neurologische Klinik und Poliklinik, Basel (Switzerland); UCSF, Department of Neurology, San Francisco (United States)

2014-08-15

With an overall incidence of 10 % of all central nervous system tumors, spinal tumors are relatively rare in children. The majority of these tumors are astrocytomas and ependymomas (70 %) followed by rare non-glial tumor entities, such as gangliogliomas. They can be differentiated into intramedullary, extramedullary intradural and extramedullary extradural tumors according to their occurrence within the anatomical intraspinal compartments. The clinical presentation is generally unspecific. Longer lasting back pain or a gradually worsening scoliosis are often the first signs of the disease. Neurological deficits, such as gait disturbances and paresis often occur after a time delay. In rare cases increased intracranial pressure has been reported. Knowledge concerning potential organ manifestations, resulting complications and typical radiological presentation, especially in magnetic resonance imaging are mandatory for adequate diagnosis and treatment of affected patients. (orig.) [German] Spinale Tumoren umfassen bei Kindern etwa 10 % aller auftretenden Tumoren des Zentralnervensystems (ZNS) und sind damit relativ selten. Die ueberwiegende Mehrheit dieser Tumoren sind Astrozytome und Ependymome (70 %), gefolgt von selteneren nichtglialen Tumorentitaeten, wie z. B. Gangliogliomen. Differenziert werden koennen sie nach ihrem Auftreten innerhalb der anatomischen intraspinalen Kompartimente in intra- und extramedullaere sowie intra- und extradurale Tumoren. Ihre klinische Praesentation ist haeufig unspezifisch. Laenger bestehende Rueckenschmerzen oder eine progrediente Skoliose sind oft erste Hinweise auf eine Erkrankung. Neurologische Ausfallerscheinungen wie Gangstoerungen und progrediente Laehmungen treten meist zeitlich verzoegert auf. In Einzelfaellen wird ueber Hirndruckzeichen berichtet. Kenntnisse ueber die moegliche Organmanifestation, resultierende Komplikationen und die typischen radiologischen Befunde v. a. in der MRT sind entscheidend fuer die adaequate

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.

Extramedullary hematopoiesis of the liver in a child with sickle cell disease: A rare complication.

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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.

Subdural fluid collection and hydrocephalus following cervical schwannoma resection: hydrocephalus resolution after spinal pseudomeningocele repair: case report.

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Benedetto, Nicola; Cagnazzo, Federico; Gambacciani, Carlo; Perrini, Paolo

2016-12-01

The authors report the case of a 31-year-old man who developed neck pain and headache 2 months after the uncomplicated resection of a cervical schwannoma. MR imaging revealed infratentorial subdural fluid collections and obstructive hydrocephalus associated with cervical pseudomeningocele. The clinical symptoms, subdural fluid collections, and ventricular dilation resolved after surgical correction of the pseudomeningocele. This report emphasizes that hydrocephalus may be related to disorders of cerebrospinal fluid flow dynamics induced by cervical pseudomeningocele. In these rare cases, both the hydrocephalus and the symptoms are resolved by the simple correction of the pseudomeningocele.

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

Incidence of extramedullary relapse after haploidentical SCT for advanced AML/myelodysplastic syndrome.

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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.

The extracisternal approach in vestibular schwannoma surgery and facial nerve preservation

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Eduardo A. S. Vellutini

2014-12-01

Full Text Available The classical surgical technique for the resection of vestibular schwannomas (VS has emphasized the microsurgical anatomy of cranial nerves. We believe that the focus on preservation of the arachnoid membrane may serve as a safe guide for tumor removal. Method The extracisternal approach is described in detail. We reviewed charts from 120 patients treated with this technique between 2006 and 2012. Surgical results were evaluated based on the extension of resection, tumor relapse, and facial nerve function. Results Overall gross total resection was achieved in 81% of the patients. The overall postoperative facial nerve function House-Brackmann grades I-II at one year was 93%. There was no recurrence in 4.2 years mean follow up. Conclusion The extracisternal technique differs from other surgical descriptions on the treatment of VS by not requiring the identification of the facial nerve, as long as we preserve the arachnoid envelope in the total circumference of the tumor.

Predicting Nonauditory Adverse Radiation Effects Following Radiosurgery for Vestibular Schwannoma: A Volume and Dosimetric Analysis

International Nuclear Information System (INIS)

Hayhurst, Caroline; Monsalves, Eric; Bernstein, Mark; Gentili, Fred; Heydarian, Mostafa; Tsao, May; Schwartz, Michael; Prooijen, Monique van; Millar, Barbara-Ann; Ménard, Cynthia; Kulkarni, Abhaya V.; Laperriere, Norm; Zadeh, Gelareh

2012-01-01

Purpose: To define clinical and dosimetric predictors of nonauditory adverse radiation effects after radiosurgery for vestibular schwannoma treated with a 12 Gy prescription dose. Methods: We retrospectively reviewed our experience of vestibular schwannoma patients treated between September 2005 and December 2009. Two hundred patients were treated at a 12 Gy prescription dose; 80 had complete clinical and radiological follow-up for at least 24 months (median, 28.5 months). All treatment plans were reviewed for target volume and dosimetry characteristics; gradient index; homogeneity index, defined as the maximum dose in the treatment volume divided by the prescription dose; conformity index; brainstem; and trigeminal nerve dose. All adverse radiation effects (ARE) were recorded. Because the intent of our study was to focus on the nonauditory adverse effects, hearing outcome was not evaluated in this study. Results: Twenty-seven (33.8%) patients developed ARE, 5 (6%) developed hydrocephalus, 10 (12.5%) reported new ataxia, 17 (21%) developed trigeminal dysfunction, 3 (3.75%) had facial weakness, and 1 patient developed hemifacial spasm. The development of edema within the pons was significantly associated with ARE (p = 0.001). On multivariate analysis, only target volume is a significant predictor of ARE (p = 0.001). There is a target volume threshold of 5 cm3, above which ARE are more likely. The treatment plan dosimetric characteristics are not associated with ARE, although the maximum dose to the 5th nerve is a significant predictor of trigeminal dysfunction, with a threshold of 9 Gy. The overall 2-year tumor control rate was 96%. Conclusions: Target volume is the most important predictor of adverse radiation effects, and we identified the significant treatment volume threshold to be 5 cm3. We also established through our series that the maximum tolerable dose to the 5th nerve is 9 Gy.

Single Fraction Versus Fractionated Linac-Based Stereotactic Radiotherapy for Vestibular Schwannoma: A Single-Institution Experience

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Collen, Christine, E-mail: ccollen@uzbrussel.be [Department of Radiation Oncology, UZ Brussel, Vrije Universiteit Brussel (VUB), Laarbeeklaan 101, 1090 Brussels (Belgium); Ampe, Ben [Department of Neurosurgery, UZ Brussel, Vrije Universiteit Brussel (VUB), Laarbeeklaan 101, 1090 Brussels (Belgium); Gevaert, Thierry [Department of Radiation Oncology, UZ Brussel, Vrije Universiteit Brussel (VUB), Laarbeeklaan 101, 1090 Brussels (Belgium); Moens, Maarten [Department of Neurosurgery, UZ Brussel, Vrije Universiteit Brussel (VUB), Laarbeeklaan 101, 1090 Brussels (Belgium); Linthout, Nadine; De Ridder, Mark; Verellen, Dirk [Department of Radiation Oncology, UZ Brussel, Vrije Universiteit Brussel (VUB), Laarbeeklaan 101, 1090 Brussels (Belgium); D' Haens, Jean [Department of Neurosurgery, UZ Brussel, Vrije Universiteit Brussel (VUB), Laarbeeklaan 101, 1090 Brussels (Belgium); Storme, Guy [Department of Radiation Oncology, UZ Brussel, Vrije Universiteit Brussel (VUB), Laarbeeklaan 101, 1090 Brussels (Belgium)

2011-11-15

Purpose: To evaluate and compare outcomes for patients with vestibular schwannoma (VS) treated in a single institution with linac-based stereotactic radiosurgery (SRS) or by fractionated stereotactic radiotherapy (SRT). Methods and Materials: One hundred and nineteen patients (SRS = 78, SRT = 41) were treated. For both SRS and SRT, beam shaping is performed by a mini-multileaf collimator. For SRS, a median single dose of 12.5 Gy (range, 11-14 Gy), prescribed to the 80% isodose line encompassing the target, was applied. Of the 42 SRT treatments, 32 treatments consisted of 10 fractions of 3-4 Gy, and 10 patients received 25 sessions of 2 Gy, prescribed to the 100% with the 95% isodose line encompassing the planning target volume. Mean largest tumor diameter was 16.6 mm in the SRS and 24.6 mm in the SRT group. Local tumor control, cranial nerve toxicity, and preservation of useful hearing were recorded. Any new treatment-induced cranial nerve neuropathy was scored as a complication. Results: Median follow-up was 62 months (range, 6-136 months), 5 patients progressed, resulting in an overall 5-year local tumor control of 95%. The overall 5-year facial nerve preservation probability was 88% and facial nerve neuropathy was statistically significantly higher after SRS, after prior surgery, for larger tumors, and in Koos Grade {>=}3. The overall 5-year trigeminal nerve preservation probability was 96%, not significantly influenced by any of the risk factors. The overall 4-year probability of preservation of useful hearing (Gardner-Robertson score 1 or 2) was 68%, not significantly different between SRS or SRT (59% vs. 82%, p = 0.089, log rank). Conclusion: Linac-based RT results in good local control and acceptable clinical outcome in small to medium-sized vestibular schwannomas (VSs). Radiosurgery for large VSs (Koos Grade {>=}3) remains a challenge because of increased facial nerve neuropathy.

Predicting Nonauditory Adverse Radiation Effects Following Radiosurgery for Vestibular Schwannoma: A Volume and Dosimetric Analysis

Energy Technology Data Exchange (ETDEWEB)

Hayhurst, Caroline; Monsalves, Eric; Bernstein, Mark; Gentili, Fred [Gamma Knife Unit, Division of Neurosurgery, University Health Network, Toronto (Canada); Heydarian, Mostafa; Tsao, May [Radiation Medicine Program, Princess Margaret Hospital, Toronto (Canada); Schwartz, Michael [Radiation Oncology Program and Division of Neurosurgery, Sunnybrook Hospital, Toronto (Canada); Prooijen, Monique van [Radiation Medicine Program, Princess Margaret Hospital, Toronto (Canada); Millar, Barbara-Ann; Menard, Cynthia [Radiation Oncology Program, Princess Margaret Hospital, Toronto (Canada); Kulkarni, Abhaya V. [Division of Neurosurgery, Hospital for Sick Children, University of Toronto (Canada); Laperriere, Norm [Radiation Oncology Program, Princess Margaret Hospital, Toronto (Canada); Zadeh, Gelareh, E-mail: Gelareh.Zadeh@uhn.on.ca [Gamma Knife Unit, Division of Neurosurgery, University Health Network, Toronto (Canada)

2012-04-01

Purpose: To define clinical and dosimetric predictors of nonauditory adverse radiation effects after radiosurgery for vestibular schwannoma treated with a 12 Gy prescription dose. Methods: We retrospectively reviewed our experience of vestibular schwannoma patients treated between September 2005 and December 2009. Two hundred patients were treated at a 12 Gy prescription dose; 80 had complete clinical and radiological follow-up for at least 24 months (median, 28.5 months). All treatment plans were reviewed for target volume and dosimetry characteristics; gradient index; homogeneity index, defined as the maximum dose in the treatment volume divided by the prescription dose; conformity index; brainstem; and trigeminal nerve dose. All adverse radiation effects (ARE) were recorded. Because the intent of our study was to focus on the nonauditory adverse effects, hearing outcome was not evaluated in this study. Results: Twenty-seven (33.8%) patients developed ARE, 5 (6%) developed hydrocephalus, 10 (12.5%) reported new ataxia, 17 (21%) developed trigeminal dysfunction, 3 (3.75%) had facial weakness, and 1 patient developed hemifacial spasm. The development of edema within the pons was significantly associated with ARE (p = 0.001). On multivariate analysis, only target volume is a significant predictor of ARE (p = 0.001). There is a target volume threshold of 5 cm3, above which ARE are more likely. The treatment plan dosimetric characteristics are not associated with ARE, although the maximum dose to the 5th nerve is a significant predictor of trigeminal dysfunction, with a threshold of 9 Gy. The overall 2-year tumor control rate was 96%. Conclusions: Target volume is the most important predictor of adverse radiation effects, and we identified the significant treatment volume threshold to be 5 cm3. We also established through our series that the maximum tolerable dose to the 5th nerve is 9 Gy.

Immediate and Long-term Hearing Outcomes With the Middle Cranial Fossa Approach for Vestibular Schwannoma Resection.

Science.gov (United States)

Ahmed, Sameer; Arts, H Alexander; El-Kashlan, Hussam; Basura, Gregory J; Thompson, B Gregory; Telian, Steven A

2018-01-01

To analyze the immediate postoperative and long-term hearing outcome data in patients who have undergone hearing preservation attempts with the middle cranial fossa (MCF) approach for the resection of sporadic vestibular schwannoma. Retrospective review of a surgical patient cohort. Tertiary academic referral center. Adult patients with unilateral sporadic vestibular schwannoma. Surgical treatment with a MCF approach. Comparison of pre- and postoperative audiometric data in accordance with the 1995 and 2012 American Academy of Otolaryngology/Head and Neck Surgery guidelines on reporting hearing outcomes and with a word recognition score only scale. Long-term hearing outcome data were analyzed in the following postoperative groups: 3 to 5, 6 to 8, 9 to 11, and 12+ years after surgery. From 1999 to 2016, 174 patients underwent the MCF approach and 155 of them met inclusion criteria for analysis of hearing outcome data. Class A or B hearing was preserved in 70% of the entire cohort after recovery. Seventy-one patients with measurable hearing met criteria for long-term data analysis. The rate of American Academy of Otolaryngology/Head and Neck Surgery class A or B hearing preservation was 82% at 3 to 5 years and declined thereafter. The rate of word recognition score class I or II hearing preservation was 98% at 3 to 5 years and declined less rapidly thereafter. Patients with preoperative Class A hearing had significantly higher rates of successful hearing preservation at all postoperative intervals. Delayed hearing loss occurs in a progressively increasing fashion but speech understanding remains durable for a majority of patients whose hearing is initially preserved with the MCF approach.

Laparoscopic Non-clamping Tumor Enucleation of Renal Hilum Schwannoma in a Single Kidney: A Case Report

Directory of Open Access Journals (Sweden)

Fuminari Hanashima

2015-11-01

Full Text Available A 56-year-old woman underwent laparoscopic right nephrectomy due to pyonephrosis associated with right ureteral stones. Moreover, the patient developed a brain stem hemorrhage and became bedridden. At the time of nephrectomy, a renal tumor, with a size of 24 × 24 × 20 mm, was observed in the left renal hilum; the tumor did not show contrast enhancement on computed tomography. After 3 years, the tumor gradually grew to a size of 45 × 35 × 34 mm, and therefore, laparoscopic non-clamping tumor enucleation was performed. Pathological examination confirmed a diagnosis of renal schwannoma.