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Sample records for hemangiopericytoma

  1. Renal Hemangiopericytoma

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    İbrahim Halil Bozkurt

    2015-03-01

    Full Text Available Hemangiopericytoma is an uncommon perivascular tumor originating from pericytes in the pelvis, head and tneck, and the meninges; extremely rarely in the urinary system. We report a case of incidentally detected renal mass in which radiologic evaluation was suggestive of renal cell carcinoma. First, we performed partial nephrectomy, and then, radical nephrectomy because of positive surgical margins and the pathological examination of the surgical specimen that revealed a hemangiopericytoma. No additional treatment was administered.

  2. Thirty-nine cases of intracranial hemangiopericytoma and anaplastic hemangiopericytoma: A retrospective review of MRI features and pathological findings

    International Nuclear Information System (INIS)

    Zhou, Jun-lin; Liu, Jian-li; Zhang, Jing; Zhang, Ming

    2012-01-01

    Objective: To retrospectively review the imaging features of surgically and pathologically confirmed intracranial hemangiopericytoma and anaplastic hemangiopericytoma. Methods: Thirty-nine cases of surgically and pathologically confirmed hemangiopericytoma and anaplastic hemangiopericytoma were analyzed retrospectively. The MRI features were compared with pathological findings in all cases. Results: Of the 39 cases, 21 were anaplastic hemangiopericytoma (WHO grade III) and the remaining cases were hemangiopericytoma (WHO grade II); all lesions were solitary. MRI of anaplastic hemangiopericytoma showed that 20 cases were lobulated, and nine grew cross-leaf. The lesions showed mixed iso-high-low signal (n = 20) or iso-signal (n = 1) on plain T1WI, and mixed high-low signal (n = 20) or iso-signal (n = 1) on plain T2WI. After contrast injection, marked heterogeneous enhancement was seen in 19 cases. Significant necrosis and cystic changes were seen in 16 cases, and the “dural tail sign” was found in two cases. Ten cases had bony destruction, and 16 showed significant peritumoral edema. In 18 cases of hemangiopericytoma, nine were oval-shaped and three grew cross-leaf. The lesions showed mixed iso-low signal (n = 10) or iso-signal (n = 8) on plain T1WI, and mixed iso-high signal (n = 10) or iso-signal (n = 8) on plain T2WI. After contrast injection, significant uniform enhancement was seen in 10 cases. Significant necrosis and cystic changes were seen in seven cases, and “dural tail sign” was seen in six cases. Two cases had bony destruction. No case showed significant peritumoral edema. Pathological immunohistochemical Ki67 staining showed a concentration of ∼18.4% positive cells in anaplastic hemangiopericytoma, whereas in hemangiopericytoma it was 7.12%. Conclusion: Imaging findings of intracranial anaplastic hemangiopericytoma had more pronounced lobulation, cross-leaf growth tendency, more and easier bleeding, more necrosis, more cystic changes giving

  3. Meningeal hemangiopericytoma

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    Guang-zhi YANG

    2015-03-01

    Full Text Available Objective To investigate the clinical, neuroimaging and pathological features of meningeal hemangiopericytoma.  Methods One case of meningeal hemangiopericytoma was reported, and the relevant literatures were also reviewed.  Results A 40-year-old male had caught a headache for about 3 months with muscle weakness in the left limb, and became progressively serious for 2 weeks. Brain MRI displayed a space-occupying lesion in the right temporal lobe with equal signals in T1WI, mixed signals in T2WI and obvious enhancements. In surgery, the tumor was found to be located in the cranial fossa, and was completely removed. The tumor was large, with rich blood supply, and had no capsule. In histology, the neoplasm was composed of dense spindle cells with mild atypia. The boundary of the tumor cells was unclear. The nuclei were circular, oval or spindle with obvious mitoses (4/10 HPF. There were plenty of thick-wall blood vessels and blood sinuses with characteristic "staghorn" shape. In immunohistochemistry, CD34 and vimentin (Vim were positive, epithelial membrane antigen (EMA was focally positive and Ki-67 labeling index was 17%-20%. Postoperative radiotherapy was adopted and no relapse was found during the 20-month follow-up period. Conclusions The meningeal hemangiopericytoma is easy to be misdiagnosed as meningioma, however, the prognosis of meningeal hemangiopericytoma is quite worse, thus the differential diagnosis is very important. A clear diagnosis often depends on pathological examination. DOI: 10.3969/j.issn.1672-6731.2015.03.011

  4. Primary hemangiopericytoma of the lung: Case report

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    Seong, Hyun Lim; Yang, Jae Beom; Park, Chan Sup; Park, Yang Hee; Lee, Sang Seun

    1990-01-01

    Hemangiopericytoma may occur at any age and can arise in almost any part of the body, but seldom in the lung. Since the first report by Stout, around fifty cases of primary hemangiopericytoma of the lung have been reported in English literature journal up to 1987. We report a case of primary pulmonary hemangiopericytoma in a 19 year old man which manifest as a solitary nodule

  5. The orbital and sinonasal hemangiopericytoma, 2 case reports:

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    Maryam Maghbol

    2016-03-01

    Full Text Available Background & objective: Hemangiopericytoma is an uncommon vascular tumor derived from the pericytes of Zimmermann. . The most common location of the tumor is pelvic retroperitoneum and musculoskeletal system of the lower extremities. It is rarely seen in the head and neck area. In addition, only 0.8% to 3% of all orbital tumors and less than 5 % of primary sinonasal tumors are primary hemangiopericytoma, which demonstrates that the orbital and sinonasal cavities are rare locations for this tumor. Case: 2 cases of primary orbital and sinonasal hemangiopericytoma that were successfully treated through surgery were reported. Conclusion: Hemangiopericytoma is an uncommon vascular tumor which has a potentially malignant behavior. Histopathologic features alone do not predict the biologic behavior of the tumor but the increased cellularity, necrosis, hemorrhage and more than 4 mitotic figures per 10 high power field, may elicit a diagnosis of malignant hemangiopericytoma . The local recurrence and the distant metastasis might also occur with an incomplete excision.  The lung, bone and liver are the most common sites of distant metastasis. Thus, the long-term follow-up is recommended after the surgical removal of the tumor.

  6. Endoscopic Resection of Sinonasal Hemangiopericytoma following Preoperative Embolisation

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    Ledderose, Georg J; Gellrich, Donata; Holtmannspötter, Markus

    2013-01-01

    Objectives. Hemangiopericytoma is a rare tumor entity deriving from pericytes. Less than 5% of hemangiopericytoma occur in the nasal cavity and are characterised by a rather benign nature with low tendency of metastasis. However, as the recurrence rate in the literature ranges from 9.5% to 50%-de...

  7. Hemangiopericytoma of the spleen.

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    Illuminati, Giulio; Pizzardi, Giulia; Calio, Francesco; Pacilè, Maria A; Carboni, Fabio; Palumbo, Piergaspare; Vietri, Francesco

    2015-03-01

    Hemangiopericytoma of the spleen is a very rare tumor, with 14 isolated reports. It was our aim to review our experience and compare it with all the reported cases in an attempt to standardize surgical treatment, adjuvant treatment and follow-up protocol of this infrequent condition. A consecutive case series study, with a mean follow-up of 44 months. Five patients (mean age, 49 years) underwent simple splenectomy for hemangiopericytoma limited to the spleen followed by adriamycin-based chemotherapy in one patient. All the patients are alive and free from disease. For tumors confined to the spleen, simple splenectomy can be considered curative, without any need for further adjuvant treatment. On review of the medical literature, cure can still be achieved with complete resection of recurrences, when feasible, with adjuvant chemotherapy being also indicated. The slow-growing pattern of the tumor suggests a 10-year follow-up. Copyright © 2015 Surgical Associates Ltd. Published by Elsevier Ltd. All rights reserved.

  8. Multifocal Congenital Hemangiopericytoma.

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    Robl, Renata; Carvalho, Vânia Oliveira; Abagge, Kerstin Taniguchi; Uber, Marjorie; Lichtvan, Leniza Costa Lima; Werner, Betina; Mehrdad Nadji, Mehrdad

    2017-01-01

    Congenital hemangiopericytoma (HPC) is a rare mesenchymal tumor with less aggressive behavior and a more favorable prognosis than similar tumors in adults. Multifocal presentation is even less common than isolated HPC and hence its clinical and histologic recognition may be challenging. A newborn infant with multifocal congenital HPC causing severe deformity but with a favorable outcome after chemotherapy and surgical removal is reported. © 2016 Wiley Periodicals, Inc.

  9. Renal hemangiopericytoma secondary to refractory hypertension in a child: A case report.

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    Hu, Qingfeng; Fang, Zujun; Zhou, Zhongwen; Zheng, Jie

    2014-12-01

    Hemangiopericytoma is a rare perivascular tumor that often involves the extremities, pelvis, head and neck, and meninges, but rarely occurs in the kidney. The differentiation from renal cancer prior to surgery is extremely challenging; therefore, almost all cases of renal hemangiopericytoma are diagnosed by pathological examination. The majority of cases are identified in patients between the ages of 20 and 50 years of age, and a considerable proportion of patients exhibit hypertension, hypoglycaemia or additional paraneoplastic syndromes. The current study reports a rare case of renal hemangiopericytoma with drug refractory hypertension in a 14-year-old female. Following the complete resection of the tumor, the patient's blood pressure returned to normal. No evidence of recurrence or metastasis was observed during a follow-up of 12 months following surgery. The present case indicated that surgery provides satisfactory outcomes and appears to be the most effective modality of treatment for renal hemangiopericytoma. Furthermore, this case also demonstrated that secondary hypertension may also recover following tumor excision.

  10. Hemangiopericytoma - The need for a protocol-based treatment plan

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    Murugesan Krishnan

    2011-01-01

    Full Text Available Hemangiopericytoma is a vascular tumor which comprises only 1% of all vascular tumors. The frequency of occurrence in the head and neck accounts for about 16-33% of all hemangiopericytomas. In this paper we discuss the surgical management, the difficulties in decision-making and treatment-planning in a case of a maxillary tumor in a five-year-old boy with a two-year follow-up. A five-year-old boy presented with a large unilateral maxillary tumor with nasal obstruction. Computed tomography revealed a heterogeneous mass completely occupying the right maxillary sinus and displacing the lateral wall of the nose and nasal septum. The lesion was diagnosed as hemangiopericytoma after histopathological confirmation. The option of surgical resection (total maxillectomy was carried out after evaluating the available literature. Various treatment modalities like surgery, chemotherapy and radiotherapy were taken into consideration as the tumor has an aggressive nature. Due to the inadequate literature on definitive treatment options for these types of tumors, there was difficulty in arriving at a protocol-based treatment plan.

  11. Effect of radiation therapy against intracranial hemangiopericytoma

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    Uemura, Shozaburo; Kuratsu, Jun-ichi; Hamada, Jun-ichiro; Yoshioka, Susumu; Kochi, Masato; Ushio, Yukitaka [Kumamoto Univ. (Japan). School of Medicine; Nakahara, Tadashi; Kishida, Katsuaki

    1992-06-01

    Seven cases of intracranial hemangiopericytoma were studied retrospectively to investigate the efficacy of radiation therapy. Tumor response evaluated by computed tomography and magnetic resonance imaging was obvious after 20-30 Gy irradiation. The total reduction rate was 80-90% and continued as long as 5-7 months after treatment. In five patients receiving radiation therapy before radical removal, the tumors were easily removed without massive hemorrhage. Histological inspection of specimens after irradiation showed a significant disappearance of tumor cells. Pyknosis frequently occurred in endothelial cells, and proliferating vessels with hyalinoid degeneration were also seen. Reticulin fibers between tumor cells were fewer, split, or absent. Preoperative radiation therapy is useful in the treatment of hemangiopericytoma involving considerable surgical risk. Postoperative radiation therapy should be given even if removal is complete. (author).

  12. Effect of radiation therapy against intracranial hemangiopericytoma

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    Uemura, Shozaburo; Kuratsu, Jun-ichi; Hamada, Jun-ichiro; Yoshioka, Susumu; Kochi, Masato; Ushio, Yukitaka; Nakahara, Tadashi; Kishida, Katsuaki.

    1992-01-01

    Seven cases of intracranial hemangiopericytoma were studied retrospectively to investigate the efficacy of radiation therapy. Tumor response evaluated by computed tomography and magnetic resonance imaging was obvious after 20-30 Gy irradiation. The total reduction rate was 80-90% and continued as long as 5-7 months after treatment. In five patients receiving radiation therapy before radical removal, the tumors were easily removed without massive hemorrhage. Histological inspection of specimens after irradiation showed a significant disappearance of tumor cells. Pyknosis frequently occurred in endothelial cells, and proliferating vessels with hyalinoid degeneration were also seen. Reticulin fibers between tumor cells were fewer, split, or absent. Preoperative radiation therapy is useful in the treatment of hemangiopericytoma involving considerable surgical risk. Postoperative radiation therapy should be given even if removal is complete. (author)

  13. Primary epidural malignant hemangiopericytoma of thoracic spinal column causing cord compression: case report.

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    Mohammadianpanah, Mohammad; Torabinejad, Simin; Bagheri, Mohammad Hadi; Omidvari, Shapour; Mosalaei, Ahmad; Ahmadloo, Niloofar

    2004-09-02

    Hemangiopericytoma is an uncommon mesenchymal neoplasm that rarely affects the spinal canal. Primary malignant hemangiopericytoma of the spinal column is extremely rare. We report on a case of primary epidural malignant hemangiopericytoma of the thoracic spinal column that invaded vertebral bone and caused spinal cord compression in a 21-year-old man. The patient presented with progressive back pain over a four-month period that progressed to paraparesis, bilateral leg paresthesia and urinary incontinence. The surgical intervention involved laminectomy and subtotal resection of the tumor, with posterior vertebral fixation. Postoperative involved-field radiotherapy was administered. A marked neurological improvement was subsequently observed. We describe the clinical, radiological, and histological features of this tumor and review the literature.

  14. Primary epidural malignant hemangiopericytoma of thoracic spinal column causing cord compression: case report

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    Mohammad Mohammadianpanah

    Full Text Available CONTEXT: Hemangiopericytoma is an uncommon mesenchymal neoplasm that rarely affects the spinal canal. Primary malignant hemangiopericytoma of the spinal column is extremely rare. CASE REPORT: We report on a case of primary epidural malignant hemangiopericytoma of the thoracic spinal column that invaded vertebral bone and caused spinal cord compression in a 21-year-old man. The patient presented with progressive back pain over a four-month period that progressed to paraparesis, bilateral leg paresthesia and urinary incontinence. The surgical intervention involved laminectomy and subtotal resection of the tumor, with posterior vertebral fixation. Postoperative involved-field radiotherapy was administered. A marked neurological improvement was subsequently observed. We describe the clinical, radiological, and histological features of this tumor and review the literature.

  15. MR appearance of pelvic hemangiopericytoma

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    Kehagias, D.; Gouliamos, A.; Vlahos, L.

    1999-01-01

    A case of pelvic hemangiopericytoma in a 59-year-old woman is reported. The MR imaging features are presented. The tumor was unresectable and the patient received postoperative irradiation of 4200 cGy on the pelvis. One year after diagnosis, metastasis to a lumbar vertebra was discovered and additional irradiation of 3900 cGy was applied. One year later, CT showed control of the pelvic tumor and its metastasis. (orig.) (orig.)

  16. A five-week-old girl with inspiratory stridor due to infantile hemangiopericytoma

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    Hansen, Jonas K; Sørensen, Flemming Brandt; Christensen, Mogens Fjord

    2006-01-01

    UNLABELLED: A 5-week-old girl with inspiratory stridor is presented. No immediate cause of the stridor was found, but eventually a diagnosis of infantile hemangiopericytoma located in the rhinopharynx was made. After surgery all respiratory symptoms disappeared.CONCLUSION: Infantile hemangioperic......UNLABELLED: A 5-week-old girl with inspiratory stridor is presented. No immediate cause of the stridor was found, but eventually a diagnosis of infantile hemangiopericytoma located in the rhinopharynx was made. After surgery all respiratory symptoms disappeared.CONCLUSION: Infantile...

  17. Hemangiopericytoma in a young dog: Evaluation of histopathological and immunohistochemical features

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    Fatemeh Namazi

    2014-06-01

    Full Text Available In the present study, we describe a subcutaneous mass between the left flank and hip in a 2-year-old male Great Dane dog. Histopathologically, cells appeared to be spindle shaped around a central capillary together with a fingerprint pattern. Immunohistochemical analysis presented that the neoplastic cells expressed vimentin, but did not stain for S-100 protein. On the basis of histopathology and immunohistochemical findings, the present tumor was diagnosed as canine hemangiopericytoma. Hemangiopericytoma could be considered in differential diagnosis list of any mass in the skin (even in young dogs and must be identified histopathologically.

  18. MR appearance of pelvic hemangiopericytoma

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    Kehagias, D.; Gouliamos, A.; Vlahos, L. [Dept. of Radiology, University of Athens (Greece)

    1999-02-01

    A case of pelvic hemangiopericytoma in a 59-year-old woman is reported. The MR imaging features are presented. The tumor was unresectable and the patient received postoperative irradiation of 4200 cGy on the pelvis. One year after diagnosis, metastasis to a lumbar vertebra was discovered and additional irradiation of 3900 cGy was applied. One year later, CT showed control of the pelvic tumor and its metastasis. (orig.) (orig.) With 3 figs., 10 refs.

  19. Unpredicted Sudden Death due to Recurrent Infratentorial Hemangiopericytoma Presenting as Massive Intratumoral Hemorrhage: A Case Report and Review of the Literature

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    Toshihide Tanaka

    2014-01-01

    Full Text Available Unpredicted sudden death arising from hemangiopericytoma with massive intracranial hemorrhage is quite rare. We encountered a patient with recurrent infratentorial hemangiopericytoma presenting as life-threatening massive intracerebral hemorrhage. A 43-year-old man who had undergone craniotomy for total resection of an infratentorial hemangiopericytoma 17 months earlier presented with morning headache and generalized convulsions. Computed tomography revealed a massive hematoma in the right infratentorial region causing tonsillar herniation and emergency surgery was performed to evacuate the hematoma. Histological findings revealed hemangiopericytoma with hemorrhage. Neurological status remained unimproved and brain death was confirmed postoperatively. Hemangiopericytoma presenting as massive hemorrhage is quite rare. Since the risk of life-threatening massive hemorrhage should be considered, careful postoperative long-term follow-up is very important to identify tumor recurrences, particularly in the posterior cranial fossa, even if the tumor is completely removed.

  20. Radiation/hyperthermia in canine hemangiopericytomas: A large animal model for therapeutic response

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    Richardson, R.C.; Anderson, V.L.; Voorhees, W.D. III; Blevins, W.E.; Inskeep, T.K.; Janas, W.; Shupe, R.E.; Babbs, C.F.

    1984-01-01

    X-irradiation followed by microwave induced hyperthermia resulted in a 91% objective response rate in 11 dogs with naturally occurring hemangiopericytomas. The authors used a novel statistical procedure to quantitatively evaluate the clinical behavior of locally invasive, non-metastatic tumors undergoing therapy for control of local disease. Utilizing a small sample size, the procedure demonstrated distribution of the data and classical parametric and non-parametric statistical methods, including setting confidence limits on the population mean and placing tolerance limits on a population percentage. Similarities of canine and human hemangiopericytomas were observed. Application of the statistical methods to human and animal trials were apparent

  1. Meningeal Hemangiopericytoma with Intracranial Metastases in an HIV-Positive Male: Case Report and Review of the Literature

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    Zachary Beatty

    2012-03-01

    Full Text Available Background: Meningeal hemangiopericytoma is a rare, aggressive CNS tumor that tends to invade locally, metastasize, and has a high rate of recurrence. HIV classically increases the risk of 3 AIDS-defining malignancies: Kaposi’s sarcoma, non-Hodgkin’s lymphoma and invasive cervical cancer. More recently, considerable interest has been paid to the link between HIV and a wider range of non-AIDS-defining cancers. An HIV-positive patient with meningeal hemangiopericytoma is described. Case Description: A 36-year-old HIV-positive male presented with worsening headache and ataxia. The patient had experienced similar neurologic symptoms 4 months prior and MRI at that time had showed an extra-axial left cerebellar mass most consistent with benign meningioma. Repeat MRI showed the tumor had increased in size by a factor of greater than 20 in this 4-month period, with 4 small additional foci of similar enhancement. Subtotal resection was performed on the mass and final pathological diagnosis was meningeal hemangiopericytoma. Conclusions: This represents the first reported case of meningeal hemangiopericytoma in an HIV-positive patient. This is also the shortest time to intracranial metastasis ever reported for a meningeal hemangiopericytoma. Although the increased risk in the HIV-positive population of non-AIDS-defining cancers that has been observed in recent years can largely be attributed to cancers with a known viral pathogenesis, it is speculated that HIV infection in this patient may have contributed to the occurrence or unique behavior of this rare tumor.

  2. Cellular solitary fibrous tumor (hemangiopericytoma) with anaplasia at cerebellopontine angle--a case report.

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    Zeng, Jianying; Ogera, Patricia; Benardete, Ethan A; Nicastri, Anthony D; Rao, Chandrakant

    2012-08-15

    Cellular solitary fibrous tumor is currently considered a synonym for hemangiopericytoma, as it became increasingly clear that the morphological and immunohistochemical features that separate these two entities have become tenuous, and evidence for a unifying concept has emerged. Furthermore, as no evidence of pericytic differentiation is given in most cases of hemangiopericytoma, this diagnostic term is waning in popularity. We present here a case of cellular solitary fibrous tumor in a 22-year-old man. Neuroimaging revealed a right cerebellopontine angle tumor. Most of the tumor was cellular although some less cellular areas were seen. Sinusoidally dilated large vessels, including staghorn type, were seen. Nuclear pleomorphism and increased mitotic activity (5 mitosis/10 high power field) were regarded as evidence of anaplasia. Diffuse CD34 immunoreactivity and focal positivity for Factor XIIIa were seen in the tumor, which was negative for EMA and S100. The tumor also displayed rich reticulin network. Solitary fibrous tumor at cerebellopontine angle is rare, and 20 such cases (five reported as hemangiopericytoma) have been reported in the English literature. Copyright © 2012 Elsevier GmbH. All rights reserved.

  3. Treatment of naturally occuring hemangiopericytoma and oral squamous cell carcinoma in dogs using surgery and photodynamic therapy with HPPH as a photosensitizer

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    Payne, John T.; McCaw, Dudley L.; Rogers, Kevin J.; Tompson, Robert V.

    1995-05-01

    Pyropheophorbide-a-hexyl ether (HPPH) is a new photosensitizer for use with photodynamic therapy (PDT) that has shown promise in laboratory animals. PDT, using this drug, is being used to treat canine patients afflicted with hemangiopericytoma and oral squamous cell carcinoma (SCC) at the University of Missouri-Columbia College of Veterinary Medicine. To date, 11 dogs with hemangiopericytoma and 5 dogs with oral SCC have been treated using a combination of surgery and PDT. Thus far, there have been no serious complications attributable to the treatment. Two dogs have had recurrences of the hemangiopericytoma and there have been no recurrences of SCC with a median follow time of 5 months. Both recurrent hemangiopericytomas were in patients with large tumors that had previous surgery. This study is ongoing and no conclusions have been reached; however several observations are noted. It appears that PDT using HPPH is safe is dogs, and may decrease the recurrence rate of Hemangiopericytomas. In dogs with oral SCC, the treatment is effective is causing necrosis and sloughing of the tumor tissue, and recurrences have not been noted on follow-ups up to 6 months.

  4. Hemangiopericitoma de órbita Orbital hemangiopericytoma

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    Ana Paula Ximenes Alves

    2001-04-01

    Full Text Available Objetivo: Descrever um raro caso de hemangiopericitoma orbital. Métodos: Relato de caso de associação entre hemangipericitoma orbital e blefaroptose. Resultados: A exérese da neoplasia normalizou o posicionamento palpebral. Conclusões: Lesões orbitais anteriores são causas de blefaroptose por compressão do músculo elevador palpebral.Purpose: To describe a rare case of orbital hemangiope- ricytoma. Methods: Case report of an association of blepha-roptosis with orbital hemangiopericytoma. Results: When the lesion was surgically removed the position of the upper eyelid returned to normal. Conclusions: Anterior orbital lesions can lead to blepharoptosis by compression of the levator palpebrae muscle.

  5. Usefulness of Choline-PET for the detection of residual hemangiopericytoma in the skull base: comparison with FDG-PET

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    Ito Shin

    2012-02-01

    Full Text Available Abstract Background Choline is a new PET tracer that is useful for the detection of malignant tumor. Choline is a precursor of the biosynthesis of phosphatidylcholine, a major phospholipid in the cell membrane of eukaryotic cells. Malignant tumors have an elevated level of phosphatidylcholine in cell membrane. Thus, choline is a marker of tumor malignancy. Method The patient was a 51-year-old man with repeated recurrent hemangiopericytoma in the skull base. We performed Choline-PET in this patient after various treatments and compared findings with those of FDG-PET. Results Choline accumulated in this tumor, but FDG did not accumulate. We diagnosed this tumor as residual hemangiopericytoma and performed the resection of the residual tumor. FDG-PET is not appropriate for skull base tumor detection because uptake in the brain is very strong. Conclusion We emphasize the usefulness of Choline-PET for the detection of residual hemangiopericytoma in the skull base after various treatments, compared with FDG-PET.

  6. Multidisciplinary Approach to Hepatic Metastases of Intracranial Hemangiopericytoma: A Case Report and Review of the Literature

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    Dimitrios K. Manatakis

    2015-01-01

    Full Text Available Hemangiopericytoma is a rare primary tumor originating from Zimmerman’s pericytes, with significant metastatic potential. Hepatic metastatic disease requires an aggressive approach by a multidisciplinary team of dedicated oncology specialists, to prolong survival in selected patients. We report on a patient with recurrent hepatic metastases of grade II intracranial hemangiopericytoma 5 years after initial treatment, managed by a stepwise combination of liver resection, radiofrequency ablation, and transarterial embolization. Although metastatic disease implies hematogenous dissemination, long-term survival after liver resection has been reported and major hepatectomies are justified in patients with adequate local control. Liver resections combined with transarterial embolization are highly recommended, due to hypervascularity of the tumor.

  7. Gamma surgery for hemangiopericytomas

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    Payne, B.R.; Prasad, D.; Steiner, M.; Steiner, L.

    2000-01-01

    A retrospective analysis of a consecutive series of 12 patients with 15 intracranial hemangiopericytomas treated at the University of Virginia using gamma surgery is presented. Clinical and radiographic follow up of 3 to 56 months is available for 10 patients with 12 tumors. There was one tumor present at the time of initial gamma surgery in each patient. Two new tumors occurred in patients previously treated. Nine of the tumors decreased in volume and three remained stable. Four of the nine tumors that shrank later progressed at an average of 22 months after treatment. Of the tumors that decreased in volume and have not progressed, the response has been for an average of 11 months. The follow-up for two tumors that remained unchanged was 10 and 34 months (average 22 months). A third tumor was unchanged at 42 months but the patient died of new disease adjacent to the treated area in the anterior skull base. There were no complications and the quality of life following the procedure was maintained or improved in every case. Gamma surgery is effective in palliating the patients by decreasing tumor volume and delaying recurrence. (author)

  8. Oncogenic osteomalacia secondary to a hemangiopericytoma of the hip: case report

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    Baronofsky, S.I.; Kalbhen, C.L.; Demos, T.C.; Sizemore, G.W.

    1999-01-01

    Osteomalacia is characterized by abnormally increased unmineralized osteoid within the bone matrix. This metabolic bone disease is usually the result of decreased uptake or abnormal metabolism of vitamin D or of renal tubular phosphate loss. Dietary deficiency, malabsorption, cirrhosis, renal tubular acidosis and certain drugs can cause osteomalacia., Oncogenic osteomalacia - osteomalacia secondary to tumours - is rare, and the exact mechanisms by which neoplasms induce osteomalacia are not known. We describe a patient with chronic osteomalacia of unknown origin who was subsequently found to have oncogenic osteomalacia secondary to a hemangiopericytoma of the hip. (author)

  9. Oncogenic osteomalacia secondary to a hemangiopericytoma of the hip: case report

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    Baronofsky, S.I.; Kalbhen, C.L.; Demos, T.C.; Sizemore, G.W. [Loyola Univ. Medical Center, Dept. of Medicine, Maywood, IL (United States)

    1999-02-01

    Osteomalacia is characterized by abnormally increased unmineralized osteoid within the bone matrix. This metabolic bone disease is usually the result of decreased uptake or abnormal metabolism of vitamin D or of renal tubular phosphate loss. Dietary deficiency, malabsorption, cirrhosis, renal tubular acidosis and certain drugs can cause osteomalacia., Oncogenic osteomalacia - osteomalacia secondary to tumours - is rare, and the exact mechanisms by which neoplasms induce osteomalacia are not known. We describe a patient with chronic osteomalacia of unknown origin who was subsequently found to have oncogenic osteomalacia secondary to a hemangiopericytoma of the hip. (author)

  10. A reappraisal of hemangiopericytoma of bone; analysis of cases reclassified as synovial sarcoma and solitary fibrous tumor of bone

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    Verbeke, Sofie L J; Fletcher, Christopher D M; Alberghini, Marco

    2010-01-01

    Hemangiopericytoma (HPC) was first described as a neoplasm with distinct morphologic features, presumably composed of pericytes. In soft tissue, it is accepted that most such lesions are solitary fibrous tumors (SFTs), monophasic synovial sarcomas (SSs), or myofibromatoses. It is unclear whether...

  11. Malignant hemangiopericytoma. A correlative study of angiography, computed tomography, and pathology

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    Higa, Toshiaki; Kuroda, Yasumasa; Kobashi, Yoichiro; Ichijima, Kunio; Odori, Teruo; Torizuka, Kanji [Kyoto Univ. (Japan). Hospital

    1983-01-01

    Four cases of primary and secondary malignant hemangiopericytoma were correlatively studied using selective angiography, computed tomography, and pathologic specimens. One case was found in each of the peritoneal space (metastasis), the left middle cranial fossa, the left thigh, and the left retroperitoneal space. Basic angiographic features of the tumor were a few feeding arteries entering the tumor with radially arranged fine network throughout, early visualization of veins due to A-V shunting, and a long-standing tumor stain with avascular area (s). Correlative evaluation of angiograms, computed tomograms, and pathologic specimens revealed hemorrhage, central necrosis, and cystic degeneration for the avascular area (s) of the tumor stains on the angiograms. The more prominent those secondary changes, the less characteristic the angiographic features.

  12. Primary Intradural Hemangiopericytoma With Intramedullary Invasion

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    Chiang-Wei Chou

    2009-10-01

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

  13. Pazopanib efficacy in recurrent central nervous system hemangiopericytomas.

    Science.gov (United States)

    Apra, Caroline; Alentorn, Agusti; Mokhtari, Karima; Kalamarides, Michel; Sanson, Marc

    2018-04-26

    There is currently no treatment for solitary fibrous tumors/hemangiopericytomas (SFT/H) of the central nervous system recurring after multiple surgeries and radiotherapies. The NAB2-STAT6 gene fusion is the hallmark of these tumors, and upregulates Early Growth Factor, activating several growth pathways. We treated two patients presenting pluri-recurrent meningeal SFT/H with Pazopanib, a broad-spectrum tyrosine kinase inhibitor. We analyzed the exome and RNA sequencing data of one of them and, in addition to another meningeal SFT/H, compared it to the transcriptomic profiling of 5 systemic SFT/H. A dramatic clinical and radiological response was observed in both cases, respectively 84 and 43% decrease after 3 months. As a comparison, Pazopanib has only a stabilizing effect in systemic SFT/H. Indeed, central nervous system SFT/H show overexpression of different tyrosine kinases targeted by Pazopanib. Two consecutive patients with untreatable central nervous system SFT/H showed a spectacular partial response to Pazopanib, an unprecedented result in SFT/H. This result could be explained by differences in expression profiles and calls for a confirmation in a larger cohort of patients.

  14. Temporal fossa hemangiopericytoma: a case series.

    Science.gov (United States)

    Heiser, Marc A; Waldron, James S; Tihan, Tarik; Parsa, Andrew T; Cheung, Steven W

    2009-10-01

    Review clinical experience with temporal fossa hemangiopericytomas (HPCs). Retrospective case series review. Tertiary referral center. Intracranial HPCs within the temporal fossa. Craniotomy for either subtotal or gross total tumor excision. Determination of clinical outcome (alive with no evidence of disease, alive with disease, and died of disease). Five cases of HPC involving the temporal fossa were treated at our tertiary referral center for the period from 1995 to 2008. All but 1 patient were men. The age of presentation ranged from 31 to 62 years, and duration of follow-up ranged from 8 to 153 months. Clinical presentation was protean; headache was the most common symptom. Gross total tumor excision was achieved in 2 patients, whereas subtotal tumor excision was achieved in 3 patients. Reasons for subtotal resection included excessive intraoperative blood loss and inextricable tumor. Histologically, all tumors were composed of tightly packed, randomly oriented (jumbled-up) tumor cells with little intervening collagen. CD34 staining mostly highlighted the vascular background. One patient died of disease, 2 patients were alive with disease, and 2 patients had no evidence of disease. Management of temporal fossa HPC is challenging because clinical presentation is often late, and extent of tumor excision is constrained by vital structures in the cranial base and intracranial contents. A multidisciplinary approach with neurosurgery and neurotology undertaken to achieve the most complete tumor resection possible, whereas minimizing morbidity are likely to confer a longer period of symptom-free survival and improves curability of these difficult lesions.

  15. Acquired Ichthyosis Triggered by an Osseous Hemangiopericytoma: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Aikaterini Patsatsi

    2014-01-01

    Full Text Available Ichthyoses are a heterogeneous group of cutaneous keratinization disorders that can be congenital or acquired. Apart from neoplastic disorders, the acquired form of ichthyosis (AI has been associated with a variety of diseases including infections, autoimmune/inflammatory and endocrine/metabolic diseases as well as nutritional conditions, medications and others. However, malignancy accounts for half of the reported cases, most commonly including lymphoproliferative disorders. We present a case of AI as a paraneoplastic skin manifestation of a primary, osseous hemangiopericytoma (HP accompanied by multiple liver metastases. We also review the literature and discuss the necessity of investigating underlying diseases, especially malignancy, when adult-onset ichthyosis arises.

  16. Meningeal Hemangiopericytomas and Meningomas: a Comparative Immunohistochemical and Genetic Study.

    Science.gov (United States)

    Trabelsi, Saoussen; Mama, Nadia; Chourabi, Maroua; Mastouri, Maroua Haddaji; Ladib, Mohamed; Popov, Sergey; Burford, Anna; Mokni, Moncef; Tlili, Kalthoum; Krifa, Hedi; Jones, Chris; Yacoubi, Mohamed Tahar; Saad, Ali; Brahim, Dorra H'mida-Ben

    2015-01-01

    The meningeal hemangiopericytoma (MHPC) is a vascular tumor arising from pericytes. Most intracranial MHPCs resemble meningiomas (MNGs) in their clinical presentation and histological features and may therefore be misdiagnosed, despite important differences in prognosis. We report 8 cases of MHPC and 5 cases of MNG collected from 2007 to 2011 from the Neuro-Surgery and Histopathology departments. All 13 samples were re reviewed by two independent pathologists and investigated by immunohistochemistry (IHC) using mesenchymal, epithelial and neuro-glial markers. Additionally, we screened all tumors for a large panel of chromosomal alterations using multiplex ligation probe amplification (MLPA). Presence of the NAB2-STAT6 fusion gene was inferred by immunohistochemical staining for STAT6. Compared with MNG, MHPCs showed strong VIM (100% of cases), CD99 (62%), bcl-2 (87%), and p16 (75%) staining but only focal positivity with EMA (33%) and NSE (37%). The p21 antibody was positive in 62% of MHPC and less than 1% in all MNGs. MLPA data did not distinguish HPC from MNG, with PTEN loss and ERBB2 gain found in both. By contrast, STAT6 nuclear staining was observed in 3 MHPC cases and was absent from MNG. MNG and MHPC comprise a spectrum of tumors that cannot be easily differentiated based on histopathology. The presence of STAT6 nuclear positivity may however be a useful diagnostic marker.

  17. CT, MR and angiographic findings of hemangiopericytomas

    International Nuclear Information System (INIS)

    Lim, Soo Mee; Lee, Ho Kyu; Shin, Ji Hoon; Kim, Jae Kyun; Kim, Dae Hong; Choi, Choong Gon; Suh, Dae Chul

    1999-01-01

    Hemangiopericytoma(HP) exhibits its pathologic findings different from those of meningioma or other angiomatous tumor; and its clinical behavior is unique and prognosis worse than other cases. We reviewed the CT, MR and angiographic findings of HPs and evaluated differential radiologic points of comparison between typical meningiomas and meningeal HPs. MR(n=16), CT(n=5) and angiographic imaging(n=10) were performed in 18 patients(M:F = 12:6, mean age: 45 years) with histologically proven primary HPs. We evaluated the imaging findings of HPs with respect to site, shape, size, signal intensity, enhancement characteristics, vascular signal voids, calcification, bony and adjacent sinus involvement, and angiographic findings. HPs were meningeal in 14 cases and nonmeningeal in four. Meningeal HPs were located in the parasagittal region(n=8), convexity(n=3), intradural extramedullary space(n=1), choroid plexus(n=1), and olfactory groove(n=1). Nonmeningeal HPs were located in the masticator space(n=2), paraspinal area(n=1) and supraclavicular area(n=1). The mean maximal dimension of tumors was about 5.4cm and their shape was papillary(n=8) or lobulated(n=7). MR images showed high(n=13) or iso(n=3) signal intensities on T2W1, and heterogeneity on T2W1(n=9). Vascular signal voids in the mass were seen in all cases, while in two cases, CT scanning showed nodular dense calcification. Bone destruction was present in six cases, but no hyperostosis was found. In five cases, the superior sagittal sinus was involved. Angiographic images revealed highly vascular masses supplied by the internal carotid artery(n=5), external carotid artery(n=8), descending scapular artery(n=1) and radiculomedullary artery(n=1), with delayed tumor blush during the capillary and venous phase in which there was no arteriovenous shunt. HP is one of the extra-axial tumors in which there is hypervascularity, aggressive bony destruction arising in the meningeal and extrameningeal area, and heterogeneous high

  18. Intracranial solitary fibrous tumors/hemangiopericytomas: first report of malignant progression.

    Science.gov (United States)

    Apra, Caroline; Mokhtari, Karima; Cornu, Philippe; Peyre, Matthieu; Kalamarides, Michel

    2018-06-01

    OBJECTIVE Meningeal solitary fibrous tumors/hemangiopericytomas (MSFTs/HPCs) are rare intracranial tumors resembling meningiomas. Their classification was redefined in 2016 by the World Health Organization (WHO) as benign Grade I fibrohyaline type, intermediate Grade II hypercellular type, and malignant highly mitotic Grade III. This grouping is based on common histological features and identification of a common NAB2-STAT6 fusion. METHODS The authors retrospectively identified 49 cases of MSFT/HPC. Clinical data were obtained from the medical records, and all cases were analyzed according to this new 2016 WHO grading classification in order to identify malignant transformations. RESULTS Recurrent surgery was performed in 18 (37%) of 49 patients. Malignant progression was identified in 5 (28%) of these 18 cases, with 3 Grade I and 2 Grade II tumors progressing to Grade III, 3-13 years after the initial surgery. Of 31 Grade III tumors treated in this case series, 16% (5/31) were proved to be malignant progressions from lower-grade tumors. CONCLUSIONS Low-grade MSFTs/HPCs can transform into higher grades as shown in this first report of such progression. This is a decisive argument in favor of a common identity for MSFT and meningeal HPC. High-grade MSFTs/HPCs tend to recur more often and be associated with reduced overall survival. Malignant progression could be one mechanism explaining some recurrences or metastases, and justifying long-term follow-up, even for patients with Grade I tumors.

  19. Osteomalacia inducida por tumor: hemangiopericitoma rinosinusal Tumor-induced osteomalacia: rhinosinusal hemangiopericytoma

    Directory of Open Access Journals (Sweden)

    Enriqueta M. Serafini

    2013-02-01

    Full Text Available La osteomalacia inducida por tumor es una rara enfermedad del metabolismo óseo caracterizada por el aumento en la excreción de fosfato a nivel renal seguido de hipofosfatemia. Es causada por agentes fosfatúricos producidos por determinados tumores. La resección total del tumor resulta en la completa reversión de las anormalidades bioquímicas, la desaparición de las manifestaciones clínicas y los hallazgos en los estudios por imágenes. Presentamos el caso de un varón de 61 años con cuadro clínico y laboratorio compatibles con osteomalacia oncogénica inducida por tumor mesenquimático de localización rinosinusal. En nuestro caso el diagnóstico histológico correspondió a una neoplasia de tipo vascular: hemangiopericitoma.Tumor-induced osteomalacia is a rare disease of bone metabolism. The characteristic of this disease is an increase in phosphate excretion followed by hypophosphatemia, due to phosphaturic agents produced by different types of tumors. Tumor resection results in complete resolution of clinical, biochemical and radiological abnormalities. We present the case of a 61 year old man with signs, symptoms and laboratory findings consistent with oncogenic osteomalacia due to a rhino-sinusal mesenchymal tumor. The histological diagnosis showed a vascular neoplasm: hemangiopericytoma.

  20. Meningeal hemangiopericytoma treated with surgery and radiation therapy -case report-

    Energy Technology Data Exchange (ETDEWEB)

    Jang, Ji Young; Oh, Yoon Kyeong [College of Medicine, Chosun University, Gwangju (Korea, Republic of)

    2006-06-15

    Meningeal hemangiopericytoma (HPC) is an uncommon dura-based tumor and can recur not only locally but also distantly in the neural axis or extraneural sites. We report our experience of radiation therapy, one preoperative and one elective postoperative, in two patients with meningeal HPC and reviewed the role of radiation therapy. A 41-year-old man (Case 1) presented with a 3-month history of headache and right hemiparesis. The mass was nearly unresectable at the first and second operation and diagnosed as meningeal HPC. Preoperative radiation therapy was given with a total dose of 55.8 Gy/31 fractions to the large residual mass of left frontoparietal area. Follow-up computerized tomography (CT) showed marked regression of tumor after radiation therapy. The third operation was performed to remove the residual tumor at 6 months after the radiation therapy and a 2 x 2 cm sized tumor was encountered. The mass was totally removed. The serial follow-up CT showed no evidence of recurrence and he is alive without distant metastasis for 4 years and 10 months after the first operation. A 45-year-old woman (Case 2) presented with suddenly developed headache and visual impairment. Tumor mass occupying right frontal lobe was removed with the preoperative diagnosis of meningioma. It was totally removed with attached sagittal sinus and diagnosed as meningeal HPC. Elective postoperative radiation therapy was performed to reduce local recurrence with a total dose of 54 Gy/30 fractions to the involved area of right frontal lobe. She is alive for 5 years maintaining normal activity without local recurrence and distant metastasis.

  1. Meningeal hemangiopericytoma treated with surgery and radiation therapy -case report-

    International Nuclear Information System (INIS)

    Jang, Ji Young; Oh, Yoon Kyeong

    2006-01-01

    Meningeal hemangiopericytoma (HPC) is an uncommon dura-based tumor and can recur not only locally but also distantly in the neural axis or extraneural sites. We report our experience of radiation therapy, one preoperative and one elective postoperative, in two patients with meningeal HPC and reviewed the role of radiation therapy. A 41-year-old man (Case 1) presented with a 3-month history of headache and right hemiparesis. The mass was nearly unresectable at the first and second operation and diagnosed as meningeal HPC. Preoperative radiation therapy was given with a total dose of 55.8 Gy/31 fractions to the large residual mass of left frontoparietal area. Follow-up computerized tomography (CT) showed marked regression of tumor after radiation therapy. The third operation was performed to remove the residual tumor at 6 months after the radiation therapy and a 2 x 2 cm sized tumor was encountered. The mass was totally removed. The serial follow-up CT showed no evidence of recurrence and he is alive without distant metastasis for 4 years and 10 months after the first operation. A 45-year-old woman (Case 2) presented with suddenly developed headache and visual impairment. Tumor mass occupying right frontal lobe was removed with the preoperative diagnosis of meningioma. It was totally removed with attached sagittal sinus and diagnosed as meningeal HPC. Elective postoperative radiation therapy was performed to reduce local recurrence with a total dose of 54 Gy/30 fractions to the involved area of right frontal lobe. She is alive for 5 years maintaining normal activity without local recurrence and distant metastasis

  2. 'Papillary' solitary fibrous tumor/hemangiopericytoma with nuclear STAT6 expression and NAB2-STAT6 fusion.

    Science.gov (United States)

    Ishizawa, Keisuke; Tsukamoto, Yoshitane; Ikeda, Shunsuke; Suzuki, Tomonari; Homma, Taku; Mishima, Kazuhiko; Nishikawa, Ryo; Sasaki, Atsushi

    2016-04-01

    This report describes clinicopathological findings, including genetic data of STAT6, in a solitary fibrous tumor (SFT)/hemangiopericytoma (HPC) of the central nervous system in an 83-year-old woman with a bulge in the left forehead. She noticed it about 5 months before, and it had grown rapidly for the past 1 month. Neuroradiological studies disclosed a well-demarcated tumor that accompanied the destruction of the skull. The excised tumor showed a prominent papillary structure, where atypical cells were compactly arranged along the fibrovascular core ('pseudopapillary'). There was rich vasculature, some of which resembled 'staghorn' vessels. Mitotic figures were occasionally found. Whorls, psammoma bodies, or intra-nuclear pseudoinclusions were not identified. By immunohistochemistry, CD34 was strongly positive in the tumor cells, and STAT6 was localized in their nuclei. By reverse transcription-polymerase chain reaction (RT-PCR), an NAB2-STAT6 fusion gene, NAB2 exon6-STAT6 exon17, was detected, establishing a definite diagnosis of SFT/HPC. 'Papillary' SFT/HPC needs to be recognized as a possible morphological variant of SFT/HPC, and should be borne in mind in its diagnostic practice.

  3. "Cor Occidere": a novel strategy of targeting the tumor core by radiosurgery in a radio- and chemo-resistant intracranial hemangiopericytoma.

    Science.gov (United States)

    Li, You Quan; Chua, Eu Tiong; Chua, Kevin L M; Chua, Melvin L K

    2018-02-01

    Intracranial hemangiopericytomas (HPC) are chemotherapy- and radiotherapy (RT)-resistant. Here, we report on a novel stereotactic radiosurgery (SRS) technique-"Cor Occidere" (Latin), as a potential strategy of overcoming radioresistance of HPC. A 36-year old female presented to our clinic for consideration of a 3rd-course of RT for her recurrent cavernous sinus HPC, following previous cranial RT at 13 and 5 years prior, and a failed 9 months trial of bevacizumab/temozolomide. The tumor-adjacent brain stem and carotid artery risked substantial damage given the cumulative RT doses to these organs. We therefore designed an SRS plan targeting only the tumor core with 16 Gy single-fraction. Despite underdosing the tumor margin, we achieved stable disease over 25 months, contrasting her responses to systemic therapies. Achieving tumor control despite a suboptimal treatment that utilized high dose ablation of the tumor core suggests novel biological mechanisms to overcome radioresistance of HPC.

  4. [Solitary fibrous tumors and hemangiopericytomas of the meninges: Immunophenotype and histoprognosis in a series of 17 cases].

    Science.gov (United States)

    Savary, Caroline; Rousselet, Marie-Christine; Michalak, Sophie; Fournier, Henri-Dominique; Taris, Michaël; Loussouarn, Delphine; Rousseau, Audrey

    2016-08-01

    The 2007 World Health Organization (WHO) classification of tumors of the central nervous system distinguishes meningeal hemangiopericytomas (HPC) from solitary fibrous tumors (TFS). In the WHO classification of tumors of soft tissue and bone, those neoplasms are no longer separate entities since the discovery in 2013 of a common oncogenic event, i.e. the NAB2-STAT6 gene fusion. A shared histopronostic grading system, called "Marseille grading system", was recently proposed, based on hypercellularity, mitotic count and necrosis. We evaluated the immunophenotype and histoprognosis in a retrospective cohort of intracranial HPC and TFS. Fifteen initial tumors and 2 recurrences were evaluated by immunohistochemistry for STAT6, CD34, EMA, progesterone receptors and Ki67. The pronostic value of the WHO and the Marseille grading systems was tested on 12 patients with clinical follow-up. Initial tumors were 11 HPC and 4 SFT. STAT6 and CD34 were expressed in 16/17 tumors, EMA and progesterone receptors in 2 and 5 cases, respectively. The Ki67 labelling index was 6.25% in HPC and 3% in SFT. Half of the tumors recurred between 2 years and 9 years after initial diagnosis (mean time 5 years). No statistical difference in the risk of recurrence was associated with either grade (WHO or Marseille), in this small cohort. The diagnosis of HPC and TFS is facilitated by the almost constant immuno-expression of STAT6, and this justifies their common classification. The high rate of recurrence implies a very long-term follow-up because the current grading systems do not accurately predict the individual risk. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  5. Prognostic Factors in Patients with Primary Hemangiopericytomas of the Central Nervous System: A Series of 103 Cases at a Single Institution.

    Science.gov (United States)

    Zhu, Hongda; Duran, Daniel; Hua, Lingyang; Tang, Hailiang; Chen, Hong; Zhong, Ping; Zheng, Kang; Wang, Yongfei; Che, Xiaoming; Bao, Weimin; Wang, Yin; Xie, Qing; Gong, Ye

    2016-06-01

    Hemangiopericytoma (HPC) is a rare mesenchymal tumor that tends to affect the central nervous system and is associated with distant metastasis and a high recurrence rate. The purpose of this study was to analyze the prognostic factors in patients with primary HPC who received surgical treatment. This retrospective study reviewed all adult patients with primary HPC of the central nervous system treated from 2001 to 2009 at our institution. Clinical information, adjuvant radiation, and expression levels of Ki-67 and p53 were correlated with patient outcomes. The final analysis included 103 patients. The mean follow-up period was 75.9 months ± 36.5 (range, 1-165 months). There was a significant difference in progression-free survival (PFS) (P < 0.001) and overall survival (P = 0.014) between patients who underwent gross total resection versus subtotal resection. Expression of p53 was found in 48.5% of patients and showed utility as an independent unfavorable prognostic factor for PFS (P = 0.006). Multivariate analysis revealed that only extent of tumor resection (P = 0.004) and p53 expression (P = 0.024) were independent prognostic factors for PFS. Adjuvant radiation was found to extend PFS only in the p53-negative expression group (P = 0.044). Gross total resection significantly improves the outcome of patients with primary HPCs, whereas adjuvant radiation contributes significantly to PFS only in patients with negative p53 expression and in patients with incomplete resections. Extent of resection and p53 expression may serve as prognostic markers for the outcome of patients with primary HPC. Copyright © 2016 Elsevier Inc. All rights reserved.

  6. CT and magnetic resonance imaging finding of lipomatous hemanioperisytoma of skull base: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Hee Girl; Yu, In Kyu; Kim, Han Kyu; Kim, Seung Min; Kang, Dong Wook [Eulji University Hospital, Daejeon (Korea, Republic of)

    2013-07-15

    Lipomatous hemangiopericytoma (LHPC) is recently recognized as a rare hemangiopericytoma variant. To our knowledge, imaging features of LHPC involving skull base have not yet been reported. We present the imaging features of LHPC of skull base in a 44-year-old female, along with a literature review CT and magnetic resonance imagings showed well-enhanced fatty issues containing temporal skull base masses, with pressure bony erosions.

  7. Grading of meningeal solitary fibrous tumors/hemangiopericytomas: analysis of the prognostic value of the Marseille Grading System in a cohort of 132 patients.

    Science.gov (United States)

    Macagno, Nicolas; Vogels, Rob; Appay, Romain; Colin, Carole; Mokhtari, Karima; Küsters, Benno; Wesseling, Pieter; Figarella-Branger, Dominique; Flucke, Uta; Bouvier, Corinne

    2018-03-30

    The finding that meningeal solitary fibrous tumors (SFTs) and meningeal hemangiopericytomas (HPCs) are both characterized by NAB2-STAT6 gene fusion has pushed their inclusion in the WHO 2016 Classification of tumors of the central nervous system (CNS) as different manifestations of the same entity. Given that the clinical behavior of the CNS SFT/HPC spectrum ranges from benign to malignant, it is presently unclear whether the grading criteria are still adequate. Here, we present the results of a study that analyzed the prognostic value of an updated version of the Marseille Grading System (MGS) in a retrospectively assembled cohort of 132 primary meningeal SFTs/HPCs with nuclear overexpression of STAT6. The median patient follow-up was 64 months (range 4-274 months); 73 cases (55%) were MGS I, 50 cases (38%) MGS II and 9 cases (7%) were MGS III. Progression-free survival (PFS) and disease-specific survival (DSS) were investigated using univariate analysis: the prognostic factors for PFS included MGS, extent of surgery, radiotherapy, chemotherapy and mitotic activity ≥5/10 high-power field (HPF). Moreover, MGS, radiotherapy, mitotic activity ≥5/10 HPF, and necrosis were the prognostic factors measured for DSS. In multivariate analysis, extent of surgery, mitotic activity ≥5/10 HPF, MGS I and MGS III were the independent prognostic factors measured for PFS while necrosis, MGS III and radiotherapy were the independent prognostic factors for DSS. In conclusion, our results show that assessing the malignancy risk of SFT/HPC should not rely on one single criterion like mitotic activity. Therefore, MGS is useful as it combines the value of different criteria. In particular, the combination of a high mitotic activity and necrosis (MGS III) indicates a particularly poor prognosis. © 2018 International Society of Neuropathology.

  8. Aplicação de retalho cutâneo no tratamento cirúrgico do hemangiopericitoma canino Surgical treatment of canine hemangiopericytoma using skin flap

    Directory of Open Access Journals (Sweden)

    Julia Maria Matera

    1998-03-01

    Full Text Available O hemangiopericitoma canino é uma neoplasia cutânea relativamente comum, que surge principalmente nos membros. As formas de terapia mais indicadas são a amputação; ressecção local e radioterapia, sendo freqüente a ocorrência de recidivas (GOLDSCHIMIDT & SHOFER, 1992. Os registros de seis cães atendidos no Serviço de Técnica Cirúrgica do Hospital Veterinário da Faculdade de Medicina Veterinária da Universidade de São Paulo foram revisados no período de julho de 1994 a julho de 1996. Foram avaliados os dados referentes à formação cutânea, técnica de retalho cutâneo utilizada, e evolução pós-operatória dos animais. Os cães pertenciam a diferentes raças, sendo três machos e três fêmeas com idade média de 9 anos. Em cinco cães, as massas neoplásicas surgiram nos membros e, em um cão, na região perineal. As técnicas de retalhos cutâneos utilizadas foram a H-plastia (2, retalho por avanço pediculado simples(1, retalho por transposição(2 e W-plastia(1. A cicatrização da pele levou de 10 a 15 dias com sobrevivência de 100% do retalho em cinco cães e 89% do retalho em um cão. Um dos animais veio a óbito 15 meses após a cirurgia devido a causas não relacionadas à neoplasia. Não ocorreu recidiva num intervalo que variou de 4 a 20 meses, desde a cirurgia até a presente data. A excisão cirúrgica do hemangiopericitoma canino com amplas margens de segurança, parece ser uma forma de terapia eficiente, uma vez que não ocorreram recidivas. O domínio das técnicas de retalhos cutâneos tem permitido tal procedimento, pois não há o receio de não se conseguir reconstruir os tecidos locais. A recuperação dos animais é mais rápida e o custo é menor em relação às outras formas de terapia.Canine hemangiopericytoma is a relatively common cutaneous tumor frequently affecting the limbs. Treatment usually is local resection, limb amputation and radiation, with high rates of tumor recurrence. The medical

  9. Meningeal hemangiopericytoma and solitary fibrous tumors carry the NAB2-STAT6 fusion and can be diagnosed by nuclear expression of STAT6 protein.

    Science.gov (United States)

    Schweizer, Leonille; Koelsche, Christian; Sahm, Felix; Piro, Rosario M; Capper, David; Reuss, David E; Pusch, Stefan; Habel, Antje; Meyer, Jochen; Göck, Tanja; Jones, David T W; Mawrin, Christian; Schittenhelm, Jens; Becker, Albert; Heim, Stephanie; Simon, Matthias; Herold-Mende, Christel; Mechtersheimer, Gunhild; Paulus, Werner; König, Rainer; Wiestler, Otmar D; Pfister, Stefan M; von Deimling, Andreas

    2013-05-01

    Non-central nervous system hemangiopericytoma (HPC) and solitary fibrous tumor (SFT) are considered by pathologists as two variants of a single tumor entity now subsumed under the entity SFT. Recent detection of frequent NAB2-STAT6 fusions in both, HPC and SFT, provided additional support for this view. On the other hand, current neuropathological practice still distinguishes between HPC and SFT. The present study set out to identify genes involved in the formation of meningeal HPC. We performed exome sequencing and detected the NAB2-STAT6 fusion in DNA of 8/10 meningeal HPC thereby providing evidence of close relationship of these tumors with peripheral SFT. Due to the considerable effort required for exome sequencing, we sought to explore surrogate markers for the NAB2-STAT6 fusion protein. We adopted the Duolink proximity ligation assay and demonstrated the presence of NAB2-STAT6 fusion protein in 17/17 HPC and the absence in 15/15 meningiomas. More practical, presence of the NAB2-STAT6 fusion protein resulted in a strong nuclear signal in STAT6 immunohistochemistry. The nuclear reallocation of STAT6 was detected in 35/37 meningeal HPC and 25/25 meningeal SFT but not in 87 meningiomas representing the most important differential diagnosis. Tissues not harboring the NAB2-STAT6 fusion protein presented with nuclear expression of NAB2 and cytoplasmic expression of STAT6 proteins. In conclusion, we provide strong evidence for meningeal HPC and SFT to constitute variants of a single entity which is defined by NAB2-STAT6 fusion. In addition, we demonstrate that this fusion can be rapidly detected by STAT6 immunohistochemistry which shows a consistent nuclear reallocation. This immunohistochemical assay may prove valuable for the differentiation of HPC and SFT from other mesenchymal neoplasms.

  10. 国外文摘(英文)

    Institute of Scientific and Technical Information of China (English)

    2011-01-01

    Phase H study of subcutaneous octreotide in adults with recurrent or progressive meningioma and meningeal hemangiopericytoma.Neuro Oncol. 2011 May;13(5):530-5. Johnson DR, Kimmel DW, Butch PA, Cascino TL, Giannini C, Wu W, Buckner JC.

  11. Mesenchymal Chondrosarcoma of Posterior Maxilla: Report of a ...

    African Journals Online (AJOL)

    Histologically, the lesion must be differentiated from similar other lesions like hemangiopericytoma, Ewing sarcoma, leukemia/lymphoma, rhabdomyosarcoma, malignant melanoma and small cell osteosarcoma.[8]. The detection of type II collagen in the matrix component of MC was a sensitive marker for the identification of.

  12. Extracranial metastasizing solitary fibrous tumors (SFT) of meninges: histopathological features of a case with long-term follow-up.

    Science.gov (United States)

    Gessi, Marco; Gielen, Gerrit H; Roeder-Geyer, Eva-Dorette; Sommer, Clemens; Vieth, Michael; Braun, Veit; Kuchelmeister, Klaus; Pietsch, Torsten

    2013-02-01

    Extrapleural solitary fibrous tumors are uncommon mesenchymal neoplasms frequently observed in middle-aged adults and are classified, according to the WHO classification of soft tissue tumors, as part of the hemangiopericytoma tumor group. However, these two entities remain separated in the WHO classification of tumors of the central nervous system. In fact, meningeal solitary fibrous tumors are believed to be benign lesion and only in a minority of cases local relapses have been described, although detailed survival clinical studies on solitary fibrous tumors of meninges are rare. In contrast to hemangiopericytoma, which frequently shows distant extracranial metastases, such an event is exceptional in patients with meningeal solitary fibrous tumors and has been clinically reported in a handful of cases only and their histopathological features have not been investigated in detail. In this report, we describe the detailed clinico-pathological features of a meningeal solitary fibrous tumor presenting during a 17-year follow-up period, multiple intra-, extracranial relapses and lung metastases. © 2012 Japanese Society of Neuropathology.

  13. Browse Title Index

    African Journals Online (AJOL)

    Items 251 - 300 of 736 ... M El Baghouli, A Sahnoun, A Debbagh, A Joual, S Bennati, F Meziane. Vol 11, No 3 (2005):, Hemangiopericytoma of the prostate. a rare case report and novel treatment strategy, Abstract PDF. Sudhir Sukumar, H Sanjay Bhat, B Nair, CS Mohammed Saheed, KV Sanjeev. Vol 20, No 2 (2014), Herlyn ...

  14. Benign mediastinal hemangiopericytoma in an adult

    International Nuclear Information System (INIS)

    Viamonte Tapia, Maria N; Perez Cala, Armando; Navarro Cuervo, Carlos

    2011-01-01

    The clinical case of a man aged 55 undergoing polymiositis diagnosis as a paraneoplastic manifestation when he was alive is presented. Nonetheless, his health condition got worse and he died even after receiving the steroid therapy. The necropsy result confirmed the presence of gastric neoplasm. (author)

  15. Technetium tc 99m-labeled red blood cells in the preoperative diagnosis of cavernous hemangioma and other vascular orbital tumors.

    Science.gov (United States)

    Polito, Ennio; Burroni, Luca; Pichierri, Patrizia; Loffredo, Antonio; Vattimo, Angelo G

    2005-12-01

    To evaluate technetium Tc 99m (99mTc) red blood cell scintigraphy as a diagnostic tool for orbital cavernous hemangioma and to differentiate between orbital masses on the basis of their vascularization. We performed 99mTc red blood cell scintigraphy on 23 patients (8 female and 15 male; mean age, 47 years) affected by an orbital mass previously revealed with computed tomography (CT) and magnetic resonance imaging (MRI) and suggesting cavernous hemangioma. In our diagnosis, we considered the orbital increase delayed uptake with the typical scintigraphic pattern known as perfusion blood pool mismatch. The patients underwent biopsy or surgical treatment with transconjunctival cryosurgical extraction when possible. Single-photon emission tomography (SPET) showed intense focal uptake in the orbit corresponding to radiologic findings in 11 patients who underwent surgical treatment and pathologic evaluation (9 cavernous hemangiomas, 1 hemangiopericytoma, and 1 lymphangioma). Clinical or histologic examination of the remaining 22 patients revealed the presence of 5 lymphoid pseudotumors, 2 lymphomas, 2 pleomorphic adenomas of the lacrimal gland, 1 astrocytoma, 1 ophthalmic vein thrombosis, and 1 orbital varix. The confirmation of the preoperative diagnosis by 99mTc red blood cell scintigraphy shows that this technique is a reliable tool for differentiating cavernous hemangiomas from other orbital masses (sensitivity, 100%; specificity, 86%) when ultrasound, CT, and MRI are not diagnostic. Unfortunately, 99mTc red blood cell scintigraphy results were positive in 1 patient with hemangiopericytoma and 1 patient with lymphangioma, which showed increased uptake in the lesion on SPET images because of the vascular nature of these tumors. Therefore, in these cases, the SPET images have to be integrated with data regarding clinical preoperative evaluation and CT scans or MRI studies. On the basis of our study, a complete diagnostic picture, CT scans or MRI studies, and

  16. Interventional neuroradiology

    International Nuclear Information System (INIS)

    Mehta, B.A.; Quint, D.J.; Sanders, W.P.; Patel, S.C.; Boulos, R.S.; Burke, T.H.

    1987-01-01

    This presentation reviews the authors' angiographic approach to interventional cases and demonstrates examples of procedures we have performed including preoperative embolizations (dural, arteriovenous malformations, meningioma, juvenile angiofibroma, gliosarcoma, glomus tympanicum, hemangiopericytoma, and spinal hemangioma), therapeutic interventions (balloon occlusion of cavernous-carotid and vertebral fistulas, intracranial and extracranial aneurysms, and angioplasty of vertebral, external carotid, and subclavian arteries), and pain management (alcohol injection of spine metastases). Potential and actual complications are reviewed

  17. Yoga Therapy in Treating Patients With Malignant Brain Tumors

    Science.gov (United States)

    2017-07-27

    Adult Anaplastic Astrocytoma; Adult Anaplastic Ependymoma; Adult Anaplastic Meningioma; Adult Anaplastic Oligodendroglioma; Adult Brain Stem Glioma; Adult Choroid Plexus Tumor; Adult Diffuse Astrocytoma; Adult Ependymoblastoma; Adult Ependymoma; Adult Giant Cell Glioblastoma; Adult Glioblastoma; Adult Gliosarcoma; Adult Grade II Meningioma; Adult Medulloblastoma; Adult Meningeal Hemangiopericytoma; Adult Mixed Glioma; Adult Oligodendroglioma; Adult Papillary Meningioma; Adult Pineal Gland Astrocytoma; Adult Pineoblastoma; Adult Pineocytoma; Adult Supratentorial Primitive Neuroectodermal Tumor (PNET); Recurrent Adult Brain Tumor

  18. Immunohistochemistry in diagnosis of extranasopharyngeal angiofibroma originating from nasal cavity: case presentation and review of the literature.

    Science.gov (United States)

    Perić, Aleksandar; Sotirović, Jelena; Cerović, Snezana; Zivić, Ljubica

    2013-01-01

    Angiofibromas are rare vascular tumors which originate predominantly in the nasopharynx and occur typically in male adolescents. Extranasopharyngeal sites such as nasal cavity and paranasal sinuses are less frequent. This review article was undertaken to evaluate the incidence, clinical features and management of extranasopharyngeal angiofibromas originating exclusivelly from nasal cavity structures. Our focus of interest was to evaluate the significance of immunohistochemical analysis in diagnosis of such extremely rare neoplasms. In the PubMed and Google Search, we found only 39 cases of nasal angifibroma, 27 males and 12 females from 1980 to 2012. The most prevalent site of origin was nasal septum, followed by inferior and middle turbinate. The commonest symptoms were nasal obstruction and epistaxis. Nasal angiofibromas are clinically distinct from nasopharyneal angiofibromas and can therefore be misdiagnosed. The differential diagnosis includes other vascular lesions, such as lobular capillary hemangioma and sinonasal-type hemangiopericytoma. Although immunohistochemistry is not necessary for differentiation between angiofibroma and capillary hemangioma, that diagnostic procedure may be helpful in distinction from sinonasal hemangiopericytoma. As an ilustration for immunohistochemical analysis, we presented a case of an elderly woman with tumor arising from the middle turbinate, diagnosed as angiofibroma. The staining was positive for CD34, CD31, factor VIII, vimentin and smooth muscle alpha-actin, and negative for desmin.

  19. Nasopharyngeal Hemangiopericytoma in a Sudanese child: A ...

    African Journals Online (AJOL)

    These cells can be found surrounding all capillaries and are thought to be immature smooth muscle derived from mesenchyme. Pericytes add structural support to the capillaries and have a contractile capability playing a role in capillary blood flow regulation, modulating both flux and permeability1,3,4. Approximately 1% of ...

  20. Cystic solitary fibrous tumor arising from the left occipital meninges: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Oh, Bae Geun; Hwang, Yoon Joon; Cha, Soon Joo; Hur, Gham; Kim, Yong Hoon; Kim, Su Young; Seo, Jung Wook; Lee, Ji Young; Kim, Han Seung [Ilsan Paik Hospital, Inje University, School of Medicine, Goyang (Korea, Republic of)

    2007-02-15

    Solitary fibrous tumor (SFT) is a benign mesenchymal neoplasm of a spindle-cell origin, and it usually involves the pleura. It's occurrence in various organs of the body has recently been described. Meningeal SFT is very rare. Radiologically, it is a strongly enhancing solid mass and is undistinguishable from fibrous meningioma and hemangiopericytoma. Yet we report here on a case of SFT with massive cystic degeneration that arose from the meninges of the left occipital region.

  1. IMMUNOHISTOCHEMISTRY IN DIAGNOSIS OF EXTRANASOPHARYNGEAL ANGIOFIBROMA ORIGINATING FROM NASAL CAVITY: CASE PRESENTATION AND REVIEW OF THE LITERATURE

    Directory of Open Access Journals (Sweden)

    Aleksandar Perić

    2013-01-01

    Full Text Available Angiofibromas are rare vascular tumors which originate predominantly in the nasopharynx and occur typically in male adolescents. Extranasopharyngeal sites such as nasal cavity and paranasal sinuses are less frequent. This review article was undertaken to evaluate the incidence, clinical features and management of extranasopharyngeal angiofibromas originating exclusivelly from nasal cavity structures. Our focus of interest was to evaluate the significance of immunohistochemical analysis in diagnosis of such extremely rare neoplasms. In the PubMed and Google Search, we found only 39 cases of nasal angifibroma, 27 males and 12 females from 1980 to 2012. The most prevalent site of origin was nasal septum, followed by inferior and middle turbinate. The commonest symptoms were nasal obstruction and epistaxis. Nasal angiofibromas are clinically distinct from nasopharyneal angiofibromas and can therefore be misdiagnosed. The differential diagnosis includes other vascular lesions, such as lobular capillary hemangioma and sinonasal-type hemangiopericytoma. Although immunohistochemistry is not necessary for differentiation between angiofibroma and capillary hemangioma, that diagnostic procedure may be helpful in distinction from sinonasal hemangiopericytoma. As an ilustration for immunohistochemical analysis, we presented a case of an elderly woman with tumor arising from the middle turbinate, diagnosed as angiofibroma. The staining was positive for CD34, CD31, factor VIII, vimentin and smooth muscle α-actin, and negative for desmin.

  2. Treatment of Vascular Soft Tissue Sarcomas With Razoxane, Vindesine, and Radiation

    International Nuclear Information System (INIS)

    Rhomberg, Walter; Wink, Anna; Pokrajac, Boris; Eiter, Helmut; Hackl, Arnulf; Pakisch, Brigitte; Ginestet, Angela; Lukas, Peter; Poetter, Richard Prof.

    2009-01-01

    Purpose: In previous studies, razoxane and vindesine together with radiotherapy was proved to be effective in soft tissue sarcomas (STS). Because razoxane leads to a redifferentiation of pathological tumor blood vessels, it was of particular interest to study the influence of this drug combination in vascular soft tissue sarcomas. Methods and Materials: This open multicenter Phase II study was performed by the Austrian Society of Radiooncology. Among 13 evaluable patients (10 angiosarcomas and 3 hemangio-pericytomas), 9 had unresectable measurable disease, 3 showed microscopic residuals, and 1 had a resection with clear margins. They received a basic treatment with razoxane and vindesine supported by radiation therapy. Outcome measures were objective response rates, survival time, and the incidence of distant metastases. Results: In nine patients with measurable vascular soft tissue sarcomas (eight angiosarcomas and one hemangiopericytoma), 6 complete remissions, 2 partial remissions, and 1 minor remission were achieved, corresponding to a major response rate of 89%. A maintenance therapy with razoxane and vindesine of 1 year or longer led to a suppression of distant metastases. The median survival time from the start of the treatment is 23+ months (range, 3-120+) for 12 patients with macroscopic and microscopic residual disease. The progression-free survival at 6 months was 75%. The combined treatment was associated with a low general toxicity, but attention must be given to increased normal tissue reactions. Conclusions: This trimodal treatment leads to excellent response rates, and it suppresses distant metastases when given as maintenance therapy.

  3. Oncogenic osteomalacia diagnosed by blood pool scintigraphy

    International Nuclear Information System (INIS)

    Palaniswamy, Shanmuga Sundaram; Subramanyam, Padma; Kumar, Harish

    2011-01-01

    Oncogenic osteomalacia is a rare metabolic bone disease characterized by phosphaturia and hypophosphatemia. Certain tumors secrete a phosphaturic factor, which results in this metabolic abnormality; this factor called as phosphatonin, is in fact a fibroblast growth factor 23 (FGF-23) involved closely in phosphate homeostasis and skeletogenesis. Complete excision of these tumors facilitates reversal of the problem. We have reported here the case of a patient who was crippled with this disease and on thorough investigation revealed an oncogenic osteomalacia with tumor focus in the right tibia. The tumor was identified as a mesenchymal tumor, i.e., hemangiopericytoma. Tumor excision alleviated patient symptoms with rapid symptomatic and biochemical improvement

  4. Cytogenetic and molecular-genetic aberrations in malignant primary bone tumors

    International Nuclear Information System (INIS)

    Zoubek, A.; Kovar, H.; Gadner, H.

    1998-01-01

    Osteosarcoma, chondrosarcoma and tumors of the Ewing group are the most frequently observed primary malignant bone tumors. In an Internet homepage recently constructed for the Orthopedic Hospital Rizzoli Bologna, Italy, these tumors have represented the majority of 4423 malignant bone tumors in the archives of this institution since 1920 (http://www.tizeta.it/rizzoli). Malignant fibrous histiocytoma, fibrosarcoma, hemangioendothelioma, malignant hemangiopericytoma and giant-cell tumors are diagnosed less frequently. Since the introduction of modern molecular and cytogenic techniques, knowledge of genetic aberrations in malginant bone tumors has steadily increased. However, so far only for the group of Ewing tumors has a recurrent chromosomal marker, the translocation t(11; 22)(q24; q12), been identified. (orig.) [de

  5. Extramedullary intradural spinal tumors; Extramedullaere intradurale spinale Tumoren

    Energy Technology Data Exchange (ETDEWEB)

    Papanagiotou, P. [Universitaetsklinikum des Saarlandes, Klinik fuer Diagnostische und Interventionelle Neuroradiologie, Homburg/Saar (Germany)

    2011-12-15

    The category of extramedullary intradural tumors includes a variety of lesions ranging from meningiomas originating from meningeal cells and nerve sheath tumors (neurofibromas, schwannomas) to less common primary tumors, such as lipomas, ependymomas, hemangiopericytomas, epidermoid cysts and dermoid cysts. Extramedullary metastases can occur as transcoelomic metastases in tumors of the central nervous system (CNS) or metastasization from other tumors. Magnetic resonance imaging (MRI) is the method of choice for localization and characterization of these lesions before treatment. (orig.) [German] Die Kategorie der extramedullaeren intraduralen Tumoren enthaelt Laesionen, die von den Nervenhuellen (Schwannome und Neurofibrome) oder von den meningealen Zellen ausgehen (Meningeome). Ependymome, Lipome, Haemangioperizytome, Epidermoidzysten und Dermoidzysten entsprechen selteneren primaeren Tumoren. Extramedullaere Metastasen koennen als Abtropfmetastasen bei ZNS-Tumoren oder als Metastasierung anderer Karzinomerkrankungen auftreten. Die Magnetresonanztomographie (MRT) ist die Methode der Wahl zur Abklaerung einer intraduralen Raumforderung. (orig.)

  6. Oncogenic osteomalacia presenting as bilateral stress fractures of the tibia

    International Nuclear Information System (INIS)

    Ohashi, Kenjirou; Ohnishi, Takeshi; Ishikawa, Tohru; Tani, Haruo; Uesugi, Keisuke; Takagi, Masayuki

    1999-01-01

    We report on a patient with bilateral stress fractures of the tibia who subsequently showed classic biochemical features of oncogenic osteomalacia. Conventional radiographs were normal. MR imaging revealed symmetric, bilateral, band-like low-signal lesions perpendicular to the medial cortex of the tibiae and corresponding to the only lesions subsequently seen on the bone scan. A maxillary sinus lesion was subsequently detected and surgically removed resulting in prompt alleviation of symptoms and normalization of hypophosphatemia and low 1,25-(OH) 2 vitamin D 3 . The lesion was pathologically diagnosed as a hemangiopericytoma-like tumor. Patients with oncogenic osteomalacia may present with stress fractures limited to the tibia, as seen in athletes. The clue to the real diagnosis lies in paying close attention to the serum phosphate levels, especially in patients suffering generalized symptoms of weakness and not given to unusual physical activity. (orig.)

  7. High-grade primary pulmonary leiomyosarcoma

    Directory of Open Access Journals (Sweden)

    Rodrigo Afonso da Silva Sardenberg

    2011-12-01

    Full Text Available Primary sarcomas of the lung are rare and account for 0.5% of all primary lung tumors. There were approximately 300 cases described in the literature as of 2006. All histologic types of sarcoma were described, and the most common intrathoracic types reported were angiosarcoma, leiomyosarcoma, fibrosarcoma, hemangiopericytoma, and rhabdomyosarcoma. The biological behavior of these tumors is not well-known due to their low frequency. Leiomyosarcomas represent one of the most common subtypes encountered in the lungs, and usually occur during the sixth decade, with male predominance. Although the frequency of metastatic disease is not related to tumor size, prognosis was reported to be poorer in high-grade tumors. In comparison with other sarcomas, survival after complete resection of pulmonary leiomyosarcoma was reported as longer. We report on a patient with primary leiomyosarcoma originating from the bronchus with complete resection and long-term follow-up.

  8. Magnetic resonance imaging of intraorbital tumors

    International Nuclear Information System (INIS)

    Inoue, Tooru; Fukui, Masashi; Matsushima, Toshio; Fujii, Kiyotaka; Hasuo, Kanehiro

    1991-01-01

    Ten cases of histologically confirmed intraorbital tumors were studied with magnetic resonance imaging (MRI). Two meningiomas were nearly isointense on the T 1 -weighted image (T 1 WI) and the T 2 -weighted image (T 2 WI) relative on the cerebral cortex. The hemangiopericytoma, lacrimal gland tumor, optic glioma, and encephalocele were hypointense on the T 1 WI. The pseudotumor was hypoisointense on both the T 1 WI and the T 2 WI. The metastatic tumor (prostatic carcinoma) was hyperintense on both the T 1 WI and the T 2 WI. Gd-DTPA MRI was performed in five cases. The anatomical relationships between the tumor and the orbital tissue could be discriminated well by means of the coronal and sagittal views. MRI is thus found to be useful for the preoperative diagnosis of the intraorbital tumor and the selection of the surgical approach. (author)

  9. Oncogenic osteomalacia presenting as bilateral stress fractures of the tibia

    Energy Technology Data Exchange (ETDEWEB)

    Ohashi, Kenjirou; Ohnishi, Takeshi; Ishikawa, Tohru [Department of Radiology, St. Marianna University Hospital, Kanagawa (Japan); Tani, Haruo [Department of Internal Medicine III, St. Marianna University Hospital, Kawasaki City, Kanagawa (Japan); Uesugi, Keisuke [Department of Otolaryngology, St. Marianna University Hospital, Kawasaki City, Kanagawa (Japan); Takagi, Masayuki [Department of Pathology, St. Marianna University Hospital, Kawasaki City, Kanagawa (Japan)

    1999-01-01

    We report on a patient with bilateral stress fractures of the tibia who subsequently showed classic biochemical features of oncogenic osteomalacia. Conventional radiographs were normal. MR imaging revealed symmetric, bilateral, band-like low-signal lesions perpendicular to the medial cortex of the tibiae and corresponding to the only lesions subsequently seen on the bone scan. A maxillary sinus lesion was subsequently detected and surgically removed resulting in prompt alleviation of symptoms and normalization of hypophosphatemia and low 1,25-(OH){sub 2} vitamin D{sub 3}. The lesion was pathologically diagnosed as a hemangiopericytoma-like tumor. Patients with oncogenic osteomalacia may present with stress fractures limited to the tibia, as seen in athletes. The clue to the real diagnosis lies in paying close attention to the serum phosphate levels, especially in patients suffering generalized symptoms of weakness and not given to unusual physical activity. (orig.) With 4 figs., 6 refs.

  10. Magnetic resonance imaging of intraorbital tumors

    Energy Technology Data Exchange (ETDEWEB)

    Inoue, Tooru; Fukui, Masashi; Matsushima, Toshio; Fujii, Kiyotaka; Hasuo, Kanehiro (Kyushu Univ., Fukuoka (Japan). Faculty of Medicine)

    1991-12-01

    Ten cases of histologically confirmed intraorbital tumors were studied with magnetic resonance imaging (MRI). Two meningiomas were nearly isointense on the T[sub 1]-weighted image (T[sub 1]WI) and the T[sub 2]-weighted image (T[sub 2]WI) relative on the cerebral cortex. The hemangiopericytoma, lacrimal gland tumor, optic glioma, and encephalocele were hypointense on the T[sub 1]WI. The pseudotumor was hypoisointense on both the T[sub 1]WI and the T[sub 2]WI. The metastatic tumor (prostatic carcinoma) was hyperintense on both the T[sub 1]WI and the T[sub 2]WI. Gd-DTPA MRI was performed in five cases. The anatomical relationships between the tumor and the orbital tissue could be discriminated well by means of the coronal and sagittal views. MRI is thus found to be useful for the preoperative diagnosis of the intraorbital tumor and the selection of the surgical approach. (author).

  11. Oncogenic osteomalacia: a clinicopathologic study of 17 bone lesions.

    Science.gov (United States)

    Park, Y. K.; Unni, K. K.; Beabout, J. W.; Hodgson, S. F.

    1994-01-01

    Oncogenic osteomalacia is an unusual and rare clinicopathologic syndrome characterized by mesenchymal tumors that apparently produce osteomalacia and biochemical abnormalities consisting of hypophosphatemia, normocalcemia, and increased levels of alkaline phosphatase. We collected from the Mayo Clinic files and from our consultation files the records for 17 cases of osteomalacia associated with bone lesions. There were five cases of fibrous dysplasia, three of hemangiopericytoma, and two of phosphaturic mesenchymal tumor. There was one case each of osteosarcoma, chondroblastoma, chondromyxoid fibroma, malignant fibrous histiocytoma, giant cell tumor, metaphyseal fibrous defect, and hemangioma. In this study we can figure out that the most common characteristic histologic features of our cases were hemangiopericytomatous vascular proliferation, fine lace-like stromal calcification, and stromal giant cells. In most of the cases, the clinical and biochemical symptoms and signs resolved soon after complete resection of the lesion. When the lesion recurred or metastasized, the symptoms and signs also recurred. PMID:7848576

  12. The Rare Cancer Network: ongoing studies and future strategy

    Directory of Open Access Journals (Sweden)

    Mahmut Ozsahin

    2014-08-01

    Full Text Available The Rare Cancer Network (RCN was formed in the early 1990’s to create a global network that could pool knowledge and resources in the studies of rare malignancies whose infrequency prevented both their study with prospective clinical trials. To date, the RCN has initiated 74 studies resulting in 46 peer reviewed publications. The First International Symposium of the Rare Cancer Network took place in Nice in March of 2014. Status updates and proposals for new studies were heard for fifteen topics. Ongoing studies continue for cardiac sarcomas, thyroid cancers, glomus tumors, and adult medulloblastomas. New proposals were presented at the symposium for primary hepatic lymphoma, solitary fibrous tumors, Rosai-Dorfman disease, tumors of the ampulla of Vater, salivary gland tumors, anorectal melanoma, midline nuclear protein in testes carcinoma, pulmonary lymphoepithelioma-like carcinoma, adenoid cystic carcinoma of the trachea, osteosarcomas of the mandible, and extra-cranial hemangiopericytoma. This manuscript presents the abstracts of those proposals and updates on ongoing studies, as well a brief summary of the vision and future of the RCN.

  13. Differentiation of meningiomas from histologic mimics via the use of claudin-1

    International Nuclear Information System (INIS)

    Shalaby, A.M.R.; Naquib, S.M.

    2006-01-01

    Meningiomas may be occasionally difficult to distinguish pathologically from other tumors of the central nervous system. Claudin-1 is a tight junction-associated protein recently shown to be expressed in anaplastic meningiomas. This study aimed at assessment whether immunohistochemical staining for claudin-1 could help distinguish meningiomas from histologic mimics, compared with commonly used markers. Tissue sections from 20 meningothelial meningiomas, 40 fibrous meningiomas, 20 atypical meningiomas, 14 solitary fibrous tumors of the meninges, 10 meningeal hemangiopericytomas, and 14 vestibular schwannomas were stained immunohistochemically for claudin-1, epithelial membrane antigen, S-100 protein, CD34, and glial fibrillary acidic protein. In total, 42(53%) of 80 meningiomas were immunoreactive for claudin-1, whereas none of the other tumors were positive. In contrast, there was considerable overlap in the distribution of the other antibodies evaluated. Claudin-1 seems to be a specific marker for meningiomas in this context. Although its sensitivity is relatively low, claudin-1 may be helpful in a panel of immunostains to distinguish meningiomas from histologic mimics. (author)

  14. A well-known lesion in an unusual location: infantile myofibroma of the eyelid: a case report and review of literature.

    Science.gov (United States)

    Asadi Amoli, Fahimeh; Sina, Amir Hossein; Kasai, Aboulfazl; Ayan, Zahra

    2010-01-01

    Myofibroma is a neoplasia of myofibroblasts that can be solitary or multiple and it is found most commonly in the head & neck region including scalp, forehead, parotid region and oral cavity. In the eyelid it is rarely reported. It has a benign course in the solitary form and fatal in its multiple form. A 4 month male infant referred to Farabi hospital -the referral center for eye diseases- with a 2 month history of a mass in his eyelid with gradual enlargement with no other complaints. The only abnormal physical finding was a 2.5 cm mass in the eyelid. This mass was excised and sent to the hospital pathology laboratory. When confronting a spindle cell lesion with a nodular or multinodular growth pattern which appears biphasic due to alteration of light and dark staining areas, the surgical pathologist should think to the possibility of myofibroma. Its pattern of growth and architecture rules out the other differential diagnoses like nodular fasciitis, fibrous histiocytoma, infantile fibromatosis, and peripheral primitive neuroectodermal tumor, mesenchymal chondrosarcoma, malignant hemangiopericytoma, juvenile fibrosarcoma and poorly differentiated synovial sarcoma. In difficult cases immunohistochemical staining is helpful that is Vimentin & Actin positivity & Desmin, CK, EMA & S100 negativity.

  15. CT and MR imaging findings of sphenoidal masses

    Energy Technology Data Exchange (ETDEWEB)

    Takahashi, Shoki; Higano, Shuichi (Tohoku Univ., Sendai (Japan). School of Medicine); Ishii, Kiyoshi (and others)

    1994-07-01

    CT and MR imaging findings of 57 sphenoidal masses were retrospectively reviewed to assess the possibility of differential diagnosis between them. Various kinds of masses such as pituitary adenoma, epipharyngeal cancer, mucocele, chordoma, chondroma, chondrosarcoma, distant metastasis, multiple myeloma, fibrous dysplasia, craniopharyngioma, hemangiopericytoma, giant cell tumor, primary sphenoidal cancer, malignant melanoma, leukemia, histiocytosis X, and giant cell tumor were included in this series. CT scanning was performed in all cases using a spin-echo pulse sequence. The relative density of the masses, bony changes and calcification were evaluated on CT, and on MR images, signal intensity of the masses relative to the normal gray matter, contrast enhancement and extension/contour were evaluated. Although no single feature appeared to be specific to the masses, detection of calcification on CT, identification of the normal pituitary gland as deformed or displaced on T1-weighted images, signal intensity on T2-weighted images, and extension of the masses seemed to be useful and should be examined in terms of their ability to assist in differential diagnosis. Finally, accommodative classification of sphenoidal masses primarily based on presumed origin or mode of extension was attempted. (author).

  16. Transzygomatic approach with intraoperative neuromonitoring for resection of middle cranial fossa tumors.

    Science.gov (United States)

    Son, Byung Chul; Lee, Sang Won; Kim, Sup; Hong, Jae Taek; Sung, Jae Hoon; Yang, Seung-Ho

    2012-02-01

    The authors reviewed the surgical experience and operative technique in a series of 11 patients with middle fossa tumors who underwent surgery using the transzygomatic approach and intraoperative neuromonitoring (IOM) at a single institution. This approach was applied to trigeminal schwannomas (n = 3), cavernous angiomas (n = 3), sphenoid wing meningiomas (n = 3), a petroclival meningioma (n = 1), and a hemangiopericytoma (n = 1). An osteotomy of the zygoma, a low-positioned frontotemporal craniotomy, removal of the remaining squamous temporal bone, and extradural drilling of the sphenoid wing made a flat trajectory to the skull base. Total resection was achieved in 9 of 11 patients. Significant motor pathway damage can be avoided using a change in motor-evoked potentials as an early warning sign. Four patients experienced cranial nerve palsies postoperatively, even though free-running electromyography of cranial nerves showed normal responses during the surgical procedure. A simple transzygomatic approach provides a wide surgical corridor for accessing the cavernous sinus, petrous apex, and subtemporal regions. Knowledge of the middle fossa structures is essential for anatomic orientation and avoiding injuries to neurovascular structures, although a neuronavigation system and IOM helps orient neurosurgeons.

  17. Primary glomangiosarcoma of the lung: A case report

    Directory of Open Access Journals (Sweden)

    Konstantinou Eleftheria

    2010-10-01

    Full Text Available Abstract Background Glomus tumor is an uncommon neoplasm derived from cells of the neuromyoarterial glomus or glomus body. Most glomus tumours occur in the dermis and subcutaneous tissues. A case of a primary pulmonary glomus tumour originating in the right upper lobe is presented. Case presentation A 74-yr-old male was admitted with siccus cough, dyspnea and right-sided chest pain. Computed tomography of the thorax revealed a 4 cm growth of the right upper lobe. Fiberoptic bronchoscopy demonstrated an endobronchial hypervascular mass causing obstruction of the apical segmental bronchus. Pathology report was consistent with pulmonary glomus tumor. The patient underwent a typical right upper lobectomy with mediastinal lymph node dissection. Twelve months later he is free of disease. Conclusion Occasionally glomus tumors can occur in extracutaneous sites such as the gastrointestinal tract, bone, genitourinary system and respiratory tract. Primary pulmonary glomus tumors are very rare (our case is the 19th one presented in the international literature and are often confused with other solid neoplasms such as carcinoids, hemangiopericytomas and tumors belonging to the family of Ewing's sarcoma/primitive neuroectodermal tumours.

  18. Clinical use of a 15-W diode laser in small animal surgery: results in 30 varied procedures

    Science.gov (United States)

    Crowe, Dennis T.; Swalander, David; Hittenmiller, Donald; Newton, Jenifer

    1999-06-01

    The use of a 15-watt diode laser (CeramOptec)in 30 surgical procedures in dogs and cats was reviewed. Ease of use, operator safety, hemostasis control, wound healing, surgical time, complication rate, and pain control were observed and recorded. Procedures performed were partial pancreatectomy, nasal carcinoma ablation, medial meniscus channeling, perianal and anorectal mass removal (5), hemangioma and hemangiopericytoma removal from two legs, benign skin mass removal (7), liver lobectomy, partial prostatectomy, soft palate resection, partial arytenoidectomy, partial ablation of a thyroid carcinoma, photo-vaporization of the tumor bed following malignant tumor resection (4), neurosheath tumor removal from the tongue, tail sebaceous cyst resection, malignant mammary tumor and mast cell tumor removal. The laser was found to be very simple and safe to use. Hemostasis was excellent in all but the liver and prostate surgeries. The laser was particularly effective in preventing hemorrhage during perianal, anal, and tongue mass removal. It is estimated that a time and blood loss savings of 50% over that of conventional surgery occurred with the use of the laser. All external wounds made by laser appeared to heal faster and with less inflammation than those made with a conventional or electrosurgical scalpel.

  19. REVIEW OF NASA FIBROMYXOMA: Aggressive Behaviour? (Case Report

    Directory of Open Access Journals (Sweden)

    Rr. Suzy Indharti

    2015-12-01

    Full Text Available Background: Myxomas are rare benign tumors arising from mesenchymal tissues throughout the body. These tumors are usually seen in the atrium of the heart and the jawbone. Involvement of the skull base with intracranial extension is extremely rare, and only a few cases of primary intracranial myxomas have been described in the literature. This article presents a rare case of primary myxoma of the nasal bone. Methods: The patient underwent a skull base surgery with a pre-diagnosis of possible fibromyxomas. The tumor pathology revealed a diagnosis of myxoma with bone and meningeal involvement. Despite the debulking surgery, the tumor showed a local recurrence in five month. A second debulking surgery in piece meal was required. In the article, the etiology, histological and radiological findings as well as treatment options of this rare entity were briefly discussed under the highlights of the relevant literature. Such a localization and intracranial extension of myxomas is extremely unusual in clinical practice; the diagnosis therefore requires a high degree of suspicion and detailed histopathological examination. The differential diagnosis frequently includes chondrosarcomas, chordoma, metastatic tumors of the skull, hemangiopericytoma, meningioma and other neoplasms of the dura and skull base in this location.

  20. CT and MRI evaluation of orbital tumors: our experience

    International Nuclear Information System (INIS)

    Cabrini, Marcelo; Docampo, Jorge; Martinez, Manuel; Bruno, Claudio; Morales, Carlos

    2007-01-01

    Purpose: To show our experience in the evaluation of orbital masses on computed tomography (CT) and magnetic resonance imaging (MRI). To describe their most important findings and epidemiological features found on literature review, related to their differential diagnosis. Materials and methods: During a 48-months period of time, 26 patients (13 male, 13 female; age range, 3 to 75 years) with orbital tumors were evaluated. Seventeen patients underwent MR scans, 8 underwent CT scans, and one underwent both imaging methods. It was employed 0,5 and 1 Tesla MR scanners, and axial-helical CT scanners. Results: Benign lesions were found on 7 patients (cavernous hemangioma [n=2], meningioma [n=1], epidermoid cyst [n=1], dermoid cyst [n=1], lipoma [n=1], orbital vein deformity [n=1]). It was found lesions with undetermined behavior (optical nerve glioma [n=2]), and malignant ones were found on 17 patients (metastatic lesions [n=5], non- Hodgkin's lymphoma [n=3], hemangiopericytoma [n=2], retinoblastoma [n=2], rhabdomyosarcoma [n=2], melanoma [n=1], and lacrimal adenocarcinoma [n=1]). Conclusion: In our experience, 65.4% was malignant tumors (orbital metastasis was the most common; 19.2%). More than one-quarter was benign tumor, where cavernous hemangioma was the most frequent. (author) [es

  1. A Well-Known Lesion in An Unusual Location: Infantile Myofibroma of the Eyelid:A Case Report and Review of Literature

    Directory of Open Access Journals (Sweden)

    Fahimeh Asadi Amoli

    2010-11-01

    Full Text Available "nMyofibroma is a neoplasia of myofibroblasts that can be solitary or multiple and it is found most commonly in the head & neck region including scalp, forehead, parotid region and oral cavity. In the eyelid it is rarely reported. It has a benign course in the solitary form and fatal in its multiple form. A 4 month male infant referred to Farabi hospital -the referral center for eye diseases- with a 2 month history of a mass in his eyelid with gradual enlargement with no other complaints. The only abnormal physical finding was a 2.5 cm mass in the eyelid. This mass was excised and sent to the hospital pathology laboratory. When confronting a spindle cell lesion with a nodular or multinodular growth pattern which appears biphasic due to alteration of light and dark staining areas, the surgical pathologist should think to the possibility of myofibroma. Its pattern of growth and architecture rules out the other differential diagnoses like nodular fasciitis, fibrous histiocytoma, infantile fibromatosis, and peripheral primitive neuroectodermal tumor, mesenchymal chondrosarcoma, malignant hemangiopericytoma, juvenile fibrosarcoma and poorly differentiated synovial sarcoma. In difficult cases immunohistochemical staining is helpful that is Vimentin & Actin positivity & Desmin, CK, EMA & S100 negativity.

  2. Inflammatory fibroid polyp in the duodenum of a common marmoset (Callithrix jacchus).

    Science.gov (United States)

    Yokouchi, Yusuke; Imaoka, Masako; Sayama, Ayako; Jindo, Toshimasa; Sanbuissho, Atsushi

    2013-01-01

    A 32-month-old male common marmoset had a firm and white-colored mass in the duodenal wall. The cut surface was smooth and grayish white in color. Histologically, the mass consisted of a proliferation of spindle cells with an oval to spindle-shaped nucleus and scant eosinophilic cytoplasm in a loose myxoid or fibrotic background. Most of the lesion displayed no specific growth pattern whereas some of the cells concentrated around the vessels and created an onion-bulb structure. Additionally, marked inflammatory cellular infiltration, mainly eosinophils, was observed throughout the lesion. Immunohistochemically, the spindle cells were positive for vimentin, α-smooth muscle actin, fascin, and cyclin D1, and negative for S-100, factor VIII-related antigen, and c-kit. These histological and immunohistochemical features did not meet any differential diagnoses such as gastrointestinal stromal tumor, inflammatory myofibroblastic tumor, solitary fibrous tumor/hemangiopericytoma, smooth muscle tumor, schwannoma, and hemangiosarcoma. Collectively, the authors diagnosed the mass as a lesion that corresponded to an inflammatory fibroid polyp (IFP) in humans. IFP is defined as a mesenchymal proliferation composed of spindle stromal cells, small blood vessels, and inflammatory cells, particularly eosinophils, and is currently classified as a nonneoplastic lesion. To the best of our knowledge, this is the first case of spontaneous IFP in nonhuman primates.

  3. A Well-Known Lesion in An Unusual Location: Infantile Myofibroma of the Eyelid:A Case Report and Review of Literature

    Directory of Open Access Journals (Sweden)

    Fahimeh Asadi Amoli

    2010-12-01

    Full Text Available Myofibroma is a neoplasia of myofibroblasts that can be solitary or multiple and it is found most commonly in the head & neck region including scalp, forehead, parotid region and oral cavity. In the eyelid it is rarely reported. It has a benign course in the solitary form and fatal in its multiple form. A 4 month male infant referred to Farabi hospital -the referral center for eye diseases- with a 2 month history of a mass in his eyelid with gradual enlargement with no other complaints. The only abnormal physical finding was a 2.5 cm mass in the eyelid. This mass was excised and sent to the hospital pathology laboratory. When confronting a spindle cell lesion with a nodular or multinodular growth pattern which appears biphasic due to alteration of light and dark staining areas, the surgical pathologist should think to the possibility of myofibroma. Its pattern of growth and architecture rules out the other differential diagnoses like nodular fasciitis, fibrous histiocytoma, infantile fibromatosis, and peripheral primitive neuroectodermal tumor, mesenchymal chondrosarcoma, malignant hemangiopericytoma, juvenile fibrosarcoma and poorly differentiated synovial sarcoma. In difficult cases immunohistochemical staining is helpful that is Vimentin & Actin positivity & Desmin, CK, EMA & S100 negativity.

  4. A clinicopathological study of eyelid malignancies from central India

    Directory of Open Access Journals (Sweden)

    Jahagirdar Sameer

    2007-01-01

    Full Text Available Background: Eyelid malignancies are completely treatable if detected early. The treatment depends on the invasiveness of the cancer which in turn depends on the type of malignancy. Aim: The aim of the study was to characterize the distribution of the types of eyelid malignancies in central India. Settings and Design: The study was conducted in the Department of Plastic and Maxillofacial Surgery at a tertiary care hospital. Materials and Methods: We report a series of 27 cases of eyelid malignancies. In the same case series, we also include a case of malignant hemangiopericytoma which is an extremely rare form of eyelid malignancy worldwide. Statistical Analysis: Depending on the underlying statistical distribution, either analysis of variance (ANOVA or the Kruskal-Wallis (K-W test was used to assess the differential distribution of these variables across the types of eyelid malignancies observed in this study. Results: We observed that sebaceous cell carcinoma (~37% was almost as prevalent as basal cell carcinoma (~44% in the study subjects and had an earlier age of occurrence and a more rapid clinical course. Conclusions: Sebaceous cell carcinoma of the eyelid is almost as common as basal cell carcinoma in a large tertiary care centre in central India.

  5. [Tumor and tumor-like benign mesenchymal lesions of the breast].

    Science.gov (United States)

    Bisceglia, M; Nirchio, V; Carosi, I; Cappucci, U; Decata, A; Paragone, T; Di Mattia, A L

    1995-02-01

    All the spectrum is encompassed of those miscellaneous pathologic entities occurring in the mammary stroma which are on record up to date other than "mixed fibroepithelial" tumors (fibroadenomas and phyllodes tumors) and tumors both "pure" and "mixed" originating from myoepithelium (adenomyoepitheliomas and pleomorphic adenomas). Also they were excluded those dysreactive-autoimmune diseases (sarcoidosis, sclerosing lymphocytic lobulitis, lobular granulomatous mastitis) and those inflammatory-infectious conditions (tuberculosis, actinomycosis, foreign body reactions, Mondor's disease) which can mimick breast tumors clinically or on image analysis, but on the contrary not evoking the idea of a tumor on histology. Specifically, inflammatory pseudotumor, myofibroblastoma, leiomyoma, neurinoma/neurofibroma, benign fibrous histiocytoma, hemangiopericytoma, fibromatosis, nodular fascitis, variants of lipoma, mesenchymoma, amartoma and its variants, hemangiomas, pseudoangiomatous hyperplasia of stroma, amyloid tumor, granular cell tumor, are consecutively described and discussed, with a large list of references enclosed to each rubric. Most of the pictures are taken from personally observed lesions of the breast. Only few pictures referred to are from their analogue lesions which occurred in soft parts of other locations, with specific mention of that when it was the case. Of note after reviewing the literature the fact that no glomus tumor, nor Kaposi's sarcoma either sporadic or in the context of any immunodeficiency, nor myelolipoma has been recorded yet.

  6. Human BCAS3 expression in embryonic stem cells and vascular precursors suggests a role in human embryogenesis and tumor angiogenesis.

    Directory of Open Access Journals (Sweden)

    Kavitha Siva

    Full Text Available Cancer is often associated with multiple and progressive genetic alterations in genes that are important for normal development. BCAS3 (Breast Cancer Amplified Sequence 3 is a gene of unknown function on human chromosome 17q23, a region associated with breakpoints of several neoplasms. The normal expression pattern of BCAS3 has not been studied, though it is implicated in breast cancer progression. Rudhira, a murine WD40 domain protein that is 98% identical to BCAS3 is expressed in embryonic stem (ES cells, erythropoiesis and angiogenesis. This suggests that BCAS3 expression also may not be restricted to mammary tissue and may have important roles in other normal as well as malignant tissues. We show that BCAS3 is also expressed in human ES cells and during their differentiation into blood vascular precursors. We find that BCAS3 is aberrantly expressed in malignant human brain lesions. In glioblastoma, hemangiopericytoma and brain abscess we note high levels of BCAS3 expression in tumor cells and some blood vessels. BCAS3 may be associated with multiple cancerous and rapidly proliferating cells and hence the expression, function and regulation of this gene merits further investigation. We suggest that BCAS3 is mis-expressed in brain tumors and could serve as a human ES cell and tumor marker.

  7. Clinicopathological differences between variants of the NAB2-STAT6 fusion gene in solitary fibrous tumors of the meninges and extra-central nervous system.

    Science.gov (United States)

    Nakada, Satoko; Minato, Hiroshi; Nojima, Takayuki

    2016-07-01

    Investigations on the NAB2-STAT6 fusion gene in solitary fibrous tumors (SFTs) and hemangiopericytomas (HPCs) have increased since its discovery in 2013. Although several SFTs reported without NAB2-STAT6 fusion gene analysis, we reviewed 546 SFTs/HPCs with NAB2-STAT6 fusion gene analysis in this study and investigated differences between the gene variants. In total, 452 cases tested positive for the NAB2-STAT6 fusion gene, with more than 40 variants being detected. The most frequent of these were NAB2 exon 6-STAT6 exon 16/17/18 and NAB2 exon 4-STAT6 exon 2/3, with the former occurring most frequently in SFTs in meninges, soft tissues, and head and neck; the latter predominated in SFTs in the pleura and lung. There was no difference between the histology of SFTs and fusion gene variants. A follow-up analysis of SFTs showed that 51 of 202 cases had a recurrence, with 18 of 53 meningeal SFTs having a local recurrence and/or metastasis within 0-19 years. In meninges and soft tissue, SFTs with the NAB2 exon 6-STAT6 exon 16/17/18 tended to recur more frequently than SFTs with the NAB2 exon 4-STAT6 exon 2/3. Clinicopathological data, including yearly follow-ups, are required for meningeal SFTs/HPCs to define the correlation of variants of NAB2-STAT6 fusion gene.

  8. Phase I dose escalation clinical trial of phenylbutyrate sodium administered twice daily to patients with advanced solid tumors.

    Science.gov (United States)

    Camacho, Luis H; Olson, Jon; Tong, William P; Young, Charles W; Spriggs, David R; Malkin, Mark G

    2007-04-01

    Phenylbutyrate (PBA), and its metabolite phenylacetate (PAA), induce growth inhibition and cellular differentiation in multiple tumor models. However, despite their potential anti-cancer properties, several pharmacodynamic aspects remain unknown. We conducted a dose escalating trial to evaluate twice-daily intravenous PBA infusions for two consecutive weeks (Monday through Friday) every month at five dose levels (60-360 mg/kg/day). Twenty-one patients with the following malignancies were treated: colon carcinoma 4, non-small cell lung carcinoma 4; anaplastic astrocytoma 3, glioblastoma multiforme 3, bladder carcinoma 2, sarcoma 2, and ovarian carcinoma, rectal hemangiopericytoma, and pancreatic carcinoma 1 each. Conversion of PBA to PAA and phenylacetylglutamine (PAG) was documented without catabolic saturation. Plasma content of PBA > or =1 mM was documented for only 3 h following each dose at the top two dosages. The therapy was well tolerated overall. Common adverse effects included grade 1 nausea/vomiting, fatigue, and lightheadedness. Dose limiting toxicities were short-term memory loss, sedation, confusion, nausea, and vomiting. Two patients with anaplastic astrocytoma and a patient with glioblastoma remained stable without tumor progression for 5, 7, and 4 months respectively. Administration of PBA in a twice-daily infusion schedule is safe. The maximum tolerated dose is 300 mg/kg/day. Study designs with more convenient treatment schedules and specific molecular correlates may help to further delineate the mechanism of action of this compound. Future studies evaluating PBA's ability to induce histone acetylation and cell differentiation alone or in combination with other anti-neoplastics are recommended.

  9. A designated centre for people with disabilities operated by Caring and Sharing Association (CASA), Co. Dublin

    LENUS (Irish Health Repository)

    Bisceglia, Michele

    2011-09-01

    We reviewed the world literature on solitary fibrous tumors of the central nervous system from August 1996 to July 2011, focusing on both clinicopathological features and diagnostic findings. The anatomical distribution of the 220 cases reported so far reveals that most are intracranial and just over one-fifth are intraspinal. In decreasing frequency, intracranial tumors involve the supratentorial and infratentorial compartments, the pontocerebellar angle, the sellar and parasellar regions, and the cranial nerves. Intraspinal tumors are mainly located in the thoracic and cervical segments. Although most solitary fibrous tumors of the central nervous system are dural based, a small subset presents as subpial, intraparenchymal, intraventricular, or as tumors involving the nerve rootlets with no dural connection. Preoperative imaging and intraoperative findings suggest meningioma, schwannoma or neurofibroma, hemangiopericytoma, or pituitary tumors. Immunohistochemistry is critical to establish a definitive histopathological diagnosis. Vimentin, CD34, BCL2, and CD99 are the most consistently positive markers. The usual histologic type generally behaves in a benign manner if complete removal is achieved. Recurrence is anticipated when resection is subtotal or when the tumor exhibits atypical histology. The proliferative index as assessed by MIB1 labeling is of prognostic significance. Occasionally, tumors featuring conventional morphology may recur, perhaps because of minimal residual disease left behind during surgical extirpation. Rare extracranial metastases and tumor-related deaths are on record. Surgery is the treatment of choice. Stereotactic and external beam radiation therapy may be indicated for postsurgical tumor remnants and for unresectable recurrences. Long-term active surveillance of the patients is mandatory.

  10. Solitary fibrous tumor of the central nervous system: a 15-year literature survey of 220 cases (August 1996-July 2011).

    LENUS (Irish Health Repository)

    Bisceglia, Michele

    2011-09-01

    We reviewed the world literature on solitary fibrous tumors of the central nervous system from August 1996 to July 2011, focusing on both clinicopathological features and diagnostic findings. The anatomical distribution of the 220 cases reported so far reveals that most are intracranial and just over one-fifth are intraspinal. In decreasing frequency, intracranial tumors involve the supratentorial and infratentorial compartments, the pontocerebellar angle, the sellar and parasellar regions, and the cranial nerves. Intraspinal tumors are mainly located in the thoracic and cervical segments. Although most solitary fibrous tumors of the central nervous system are dural based, a small subset presents as subpial, intraparenchymal, intraventricular, or as tumors involving the nerve rootlets with no dural connection. Preoperative imaging and intraoperative findings suggest meningioma, schwannoma or neurofibroma, hemangiopericytoma, or pituitary tumors. Immunohistochemistry is critical to establish a definitive histopathological diagnosis. Vimentin, CD34, BCL2, and CD99 are the most consistently positive markers. The usual histologic type generally behaves in a benign manner if complete removal is achieved. Recurrence is anticipated when resection is subtotal or when the tumor exhibits atypical histology. The proliferative index as assessed by MIB1 labeling is of prognostic significance. Occasionally, tumors featuring conventional morphology may recur, perhaps because of minimal residual disease left behind during surgical extirpation. Rare extracranial metastases and tumor-related deaths are on record. Surgery is the treatment of choice. Stereotactic and external beam radiation therapy may be indicated for postsurgical tumor remnants and for unresectable recurrences. Long-term active surveillance of the patients is mandatory.

  11. Diagnostic importance of contrast enhanced 18F-fluorodeoxyglucose positron emission computed tomography in patients with tumor induced osteomalacia: Our experience

    International Nuclear Information System (INIS)

    Jain, Avani S.; Shelley, Simon; Muthukrishnan, Indirani; Kalal, Shilpa; Amalachandran, Jaykanth; Chandran, Sureshkumar

    2016-01-01

    To assess the diagnostic utility of contrast-enhanced 18 F-fluorodeoxyglucose positron emission tomography-computed tomography (FDG PET-ceCT) in localization of tumors in patients with clinical diagnosis of tumor-induced osteomalacia (TIO), in correlation with histopathological results. Eight patients (five male and three female) aged 24–60 (mean 42) years with a clinical diagnosis of TIO were included in this prospective study. They underwent whole body (head to toe) FDG PET-ceCT following a standard protocol on Philips GEMINI TF PET-CT scanner. The FDG PET-ceCT results were correlated with postoperative histology findings and clinical follow-up. All the patients had an abnormal PET-ceCT study. The sensitivity of PET-ceCT was 87.5%, and positive predictive value was 100%. The tumor was located in the craniofacial region in 6/8 patients and in bone in 2/8 patients. Hemangiopericytoma was the most common reported histology. All patients underwent surgery, following which they demonstrated clinical improvement. However, one patient with atypical findings on histology did not show any clinical improvement, hence, underwent 68 Gallium-DOTANOC PET-ceCT scan for relocalization of the site of the tumor. The tumors causing TIO are small in size and usually located in obscure sites in the body. Hence, head to toe protocol should be followed for FDG PET-ceCT scans with the inclusion of upper limbs. Once the tumor is localized, regional magnetic resonance imaging can be performed for better characterization of soft tissue lesion. Imaging with FDG PET-ceCT plays an important role in detecting the site of the tumor and thereby facilitating timely management

  12. Preoperative embolization of hypervascular thoracic, lumbar, and sacral spinal column tumors: technique and outcomes from a single center.

    Science.gov (United States)

    Nair, Sreejit; Gobin, Y Pierre; Leng, Lewis Z; Marcus, Joshua D; Bilsky, Mark; Laufer, Ilya; Patsalides, Athos

    2013-09-01

    The existing literature on preoperative spine tumor embolization is limited in size of patient cohorts and diversity of tumor histologies. This report presents our experience with preoperative embolization of hypervascular thoracic, lumbar, and sacral spinal column tumors in the largest series to date. We conducted a retrospective review of 228 angiograms and 188 pre-operative embolizations for tumors involving thoracic, lumbar and sacral spinal column. Tumor vascularity was evaluated with conventional spinal angiography and was graded from 0 (same as normal adjacent vertebral body) to 3 (severe tumor blush with arteriovenous shunting). Embolic materials included poly vinyl alcohol (PVA) particles and detachable platinum coils and rarely, liquid embolics. The degree of embolization was graded as complete, near-complete, or partial. Anesthesia records were reviewed to document blood loss during surgery. Renal cell carcinoma (44.2%), thyroid carcinoma (9.2%), and leiomyosarcoma (6.6%) were the most common tumors out of a total of 40 tumor histologies. Hemangiopericytoma had the highest mean vascularity (2.6) of all tumor types with at least five representative cases followed by renal cell carcinoma (2.0) and thyroid carcinoma (2.0). PVA particles were used in 100% of cases. Detachable platinum coils were used in 51.6% of cases. Complete, near-complete, and partial embolizations were achieved in 86.1%, 12.7%, and 1.2% of all cases, respectively. There were no new post-procedure neurologic deficits or other complications with long-term morbidity. The mean intra-operative blood loss for the hypervascular tumors treated with pre-operative embolization was 1745 cc. Preoperative embolization of hypervascular thoracic, lumbar, and sacral spine tumors can be performed with high success rates and a high degree of safety at high volume centers.

  13. The Radiological Spectrum of Orbital Pathologies that Involve the Lacrimal Gland and the Lacrimal Fossa

    Energy Technology Data Exchange (ETDEWEB)

    Jung, Won Sang; Ahn, Kook Jin; Park, Mi Ra; Kim, Ji Young; Choi, Jae Jeong; Kim, Bum Soo; Hahn, Seong Tai [Catholic University College of Medicine, Seoul (Korea, Republic of)

    2007-08-15

    CT and MRI are utilized to differentiate between different types of masses and to determine the extent of lesions involving the lacrimal gland and the fossa. In lacrimal gland epithelial tumors, benign pleomorphic adenomas are seen most commonly with a well defined benign appearance, and a malignant adenoid cystic carcinoma is seen with a typical invasive malignant appearance. However, a malignant myoepithelial carcinoma is seen with a benign looking appearance. Lymphomatous lesions of the lacrimal gland include a broad spectrum ranging from reactive hyperplasia to malignant lymphoma. These lesions can be very difficult to differentiate both radiologically and pathologically. Generally, lymphomas tend to occur in older patients. The developmental cystic lesions found in the lacrimal fossa such as dermoid and epidermoid cysts can be diagnosed when the cyst involves the superior temporal quadrant of the orbit and manifests as a nonenhancing cystic mass and, in case of a lipoma, it is diagnosed as a total fatty mass. However, masses of granulocytic sarcoma and xanthogranuloma, as well as vascular masses, such as a hemangiopericytoma, are difficult to diagnose correctly on the basis of preoperative imaging findings alone. Clinically, the lesions of the lacrimal gland and fossa are found as palpable masses in the superior lateral aspect of the orbit, and these lesions constitute about 5-13% of all of the orbital masses confirmed by biopsy. Many different pathological entities arise from the lacrimal gland and fossa, and as each of them requires a different therapeutic approach, the radiological characterization of each lesion is important. Approximately, half of the lacrimal gland masses are tumors of epithelial origin and the rest are lesions that arise from lymphoid or inflammatory diseases. Pseudotumors, metastatic masses and developmental cysts may also occur in the lacrimal fossa. This paper aims to discuss the radiologic findings of the lesions that are found in

  14. The Radiological Spectrum of Orbital Pathologies that Involve the Lacrimal Gland and the Lacrimal Fossa

    International Nuclear Information System (INIS)

    Jung, Won Sang; Ahn, Kook Jin; Park, Mi Ra; Kim, Ji Young; Choi, Jae Jeong; Kim, Bum Soo; Hahn, Seong Tai

    2007-01-01

    CT and MRI are utilized to differentiate between different types of masses and to determine the extent of lesions involving the lacrimal gland and the fossa. In lacrimal gland epithelial tumors, benign pleomorphic adenomas are seen most commonly with a well defined benign appearance, and a malignant adenoid cystic carcinoma is seen with a typical invasive malignant appearance. However, a malignant myoepithelial carcinoma is seen with a benign looking appearance. Lymphomatous lesions of the lacrimal gland include a broad spectrum ranging from reactive hyperplasia to malignant lymphoma. These lesions can be very difficult to differentiate both radiologically and pathologically. Generally, lymphomas tend to occur in older patients. The developmental cystic lesions found in the lacrimal fossa such as dermoid and epidermoid cysts can be diagnosed when the cyst involves the superior temporal quadrant of the orbit and manifests as a nonenhancing cystic mass and, in case of a lipoma, it is diagnosed as a total fatty mass. However, masses of granulocytic sarcoma and xanthogranuloma, as well as vascular masses, such as a hemangiopericytoma, are difficult to diagnose correctly on the basis of preoperative imaging findings alone. Clinically, the lesions of the lacrimal gland and fossa are found as palpable masses in the superior lateral aspect of the orbit, and these lesions constitute about 5-13% of all of the orbital masses confirmed by biopsy. Many different pathological entities arise from the lacrimal gland and fossa, and as each of them requires a different therapeutic approach, the radiological characterization of each lesion is important. Approximately, half of the lacrimal gland masses are tumors of epithelial origin and the rest are lesions that arise from lymphoid or inflammatory diseases. Pseudotumors, metastatic masses and developmental cysts may also occur in the lacrimal fossa. This paper aims to discuss the radiologic findings of the lesions that are found in

  15. Rapid recurrence and bilateral lungs, multiple bone metastasis of malignant solitary fibrous tumor of the right occipital lobe: report of a case and review.

    Science.gov (United States)

    Wu, Zhengrong; Yang, Hongjun; Weng, Desheng; Ding, Yanqing

    2015-07-09

    Intracranial malignant solitary fibrous tumor (MSFT) is extremely rare. The authors report a case of MSFT of the right occipital lobe with a rapid recurrence and bilateral lung, multiple bone metastasis. The patient was a 25-year-old male presenting with headache, nausea and visual disturbances without obvious cause. Three times right-side occipital craniotomies were performed and two times postoperative conformal radiotherapy were administered within one year. 4 months after the third time of right-side occipital craniotomy, the patient felt right chest pain and neck pain. Positron emission tomography/computed tomography (PET/CT) showed tumor recurrence of the right occipital lobe and bilateral lung metastasis, multiple bone metastasis including: vertebrae, libs, the left iliac wing, sacrum, the right ischium and upper parts of both femurs. Ultrasound guided puncture biopsy of left-side back of the neck and CT guided puncture biopsy of the third lumbar vertebra were performed. General sample showed grayish white or grayish red with irregular shape. Histopathologically, the tumor was composed of areas of alternating hypercellularity and hypocellularity with spindle-shaped cells, which arranged as fascicular, storiform pattern or patternless pattern, with intervening irregular eosinophilic collagen bundles. Some areas showed hemangiopericytoma-like perivascular pattern and perivascular hyalinization. Tumor cells were pleomorphic with mitotic counts of more than 4 per 10 high power fields and showed coagulative necrosis. Immunohistochemically, tumor cells were diffusely positive for vimentin and CD99, focal positive for CD34, bcl-2 and Actin. Ki-67 labelling index was more than 40%. The final pathological diagnosis was MSFT of the right occipital lobe, metastatic MSFT of left-side back of the neck and the third lumbar vertebra. The MSFT of the right occipital lobe with recurrence and bilateral lung, multiple bone metastasis is extremely rare. Although intracranial

  16. Efficacy of 68Ga-DOTATOC Positron Emission Tomography (PET) CT in Children and Young Adults With Brain Tumors

    Science.gov (United States)

    2017-04-27

    Acoustic Schwannoma; Adult Anaplastic Astrocytoma; Adult Anaplastic Ependymoma; Adult Anaplastic Meningioma; Adult Anaplastic Oligodendroglioma; Adult Brain Stem Glioma; Adult Choroid Plexus Tumor; Adult Craniopharyngioma; Adult Diffuse Astrocytoma; Adult Ependymoblastoma; Adult Ependymoma; Adult Giant Cell Glioblastoma; Adult Glioblastoma; Adult Gliosarcoma; Adult Grade I Meningioma; Adult Grade II Meningioma; Adult Medulloblastoma; Adult Meningeal Hemangiopericytoma; Adult Mixed Glioma; Adult Myxopapillary Ependymoma; Adult Oligodendroglioma; Adult Papillary Meningioma; Adult Pilocytic Astrocytoma; Adult Pineal Gland Astrocytoma; Adult Pineoblastoma; Adult Pineocytoma; Adult Subependymal Giant Cell Astrocytoma; Adult Subependymoma; Adult Supratentorial Primitive Neuroectodermal Tumor (PNET); Childhood Choroid Plexus Tumor; Childhood Craniopharyngioma; Childhood Ependymoblastoma; Childhood Grade I Meningioma; Childhood Grade II Meningioma; Childhood Grade III Meningioma; Childhood High-grade Cerebellar Astrocytoma; Childhood High-grade Cerebral Astrocytoma; Childhood Infratentorial Ependymoma; Childhood Low-grade Cerebellar Astrocytoma; Childhood Low-grade Cerebral Astrocytoma; Childhood Medulloepithelioma; Childhood Supratentorial Ependymoma; Meningeal Melanocytoma; Newly Diagnosed Childhood Ependymoma; Recurrent Adult Brain Tumor; Recurrent Childhood Anaplastic Astrocytoma; Recurrent Childhood Anaplastic Oligoastrocytoma; Recurrent Childhood Anaplastic Oligodendroglioma; Recurrent Childhood Brain Stem Glioma; Recurrent Childhood Cerebellar Astrocytoma; Recurrent Childhood Cerebral Astrocytoma; Recurrent Childhood Diffuse Astrocytoma; Recurrent Childhood Ependymoma; Recurrent Childhood Fibrillary Astrocytoma; Recurrent Childhood Gemistocytic Astrocytoma; Recurrent Childhood Giant Cell Glioblastoma; Recurrent Childhood Glioblastoma; Recurrent Childhood Gliomatosis Cerebri; Recurrent Childhood Gliosarcoma; Recurrent Childhood Medulloblastoma; Recurrent Childhood

  17. Imaging manifestations and its clinical significance in patients with oncogenic osteomalacia

    International Nuclear Information System (INIS)

    Yu Wei; Lin Qiang; Zhang Yunqing; Jiang Bo; Jin Jin; Jiang Yan; Li Mei; Li Fang

    2006-01-01

    Objective: To compare images from different modality for detecting lesions in patients with oncogenic osteomalacia. Methods: Eight patients with oncogenic osteomalacia were recruited in this study. The age ranged from 28 to 69 years (mean age 44.1, 5 men and 3 women). All patients were diagnosed as osteomalacia according to their clinical and radiographic manifestations. Main laboratory tests included serum calcium, phosphorus, alkaline phosphatase activity, parathyroid hormone, urinary phosphorus as well as liver and renal functions. Octreotide scans were performed for all patients according to clinical request for confirming the oncogenic osteomalacia. Further examinations of MR imaging in 8 patients, spiral CT in four patients and conventional radiography in four patients were obtained after the octreotide scans respectively. All patients had operation for their tumor resections and for the pathologic diagnostic findings. Results: Abnormal laboratory findings in all patients included low serum phosphorus level (ranged from 0.29 to 0.65 mmol·L -1 ), elevated alkaline phosphatase activity (ranged from 36. 6 to 310.6 μmol·s -1 ·L -1 ) as well as urinary phosphorus level (ranged from 11.5 to 40. 9 mmol·L -1 ). Normal results included parathyroid hormone level, liver and renal functions. Pathology confirmed the diagnosis of 4 soft tissue tumors including 1 hemangiomas, 1 giant-cell tumor of tendon sheath, 1 hemangiopericytoma and 1 mesenchymal tumor, as well as 4 bone tumors including 1 malignant neurofibroma, 2 mesenchymal tumors and 1 fibroblastoma. All lesions were shown abnormal region of increasing uptake tracer on octreotide scans. However, the octreotide scans could not determine where (bone or soft tissues) the lesions located. MR imaging could differentiate the lesions within the bone or within the soft tissues in all patients. All lesions had hypo- or iso- signal intensity on T 1 WI and high signal intensity on T 2 WI with heterogeneous in 6 tumors and

  18. Interventional neuroradiology of the head and neck.

    Science.gov (United States)

    Turowski, Bernd; Zanella, Friedhelm E

    2003-08-01

    interventions are: a hemangioma of the hard palate, a juvenile angiofibroma, a hemangiopericytoma, a malignant meningioma, a malignant fibrous histiocytoma, and a glomus tumor. Effective treatment of vascular malformations, such as AV fistulas or angiomas, needs exact occlusion of the fistula or the angiomatous nidus, which is demonstrated in the case of an AV angioma of the base of the tongue. Chemotherapy with local intra-arterial cisplatin combined with intravenous administration of sodium thiosulfate as antidote is indicated as an adjuvant modality in a multimodal regimen of oropharyngeal squamous cell carcinoma or as palliative treatment of recurrent and otherwise untreatable malignant tumors of the head and neck. Examples are a carcinoma of the alveolar ridge, a squamous cell carcinoma of the floor of the mouth, and a nasopharyngeal lymphoepithelioma. Palliative treatment of a bleeding oropharyngeal cancer is another example of interventional treatment. Selective treatment, either occluding or pharmacologic, may be preoperative, palliative, or curative. The objective is reduction of surgical risk, improvement of quality of life, or curative therapy of a lesion. Thus, the interventional treatment should not be associated with morbidity or mortality. Major complications, such as cerebral stroke, blindness, or cranial nerve palsies, can result from application of inappropriate techniques or poor evaluation of angiographic findings and should be avoided in the majority of cases. Sometimes collateral lesions are unavoidable. These include inflammation, necrosis, and nerve damage. Based on neuroradiologic experience, most of those risks are predictable. The benefits, risks, and expected damages of neuroradiologic interventions must be balanced during the informed consent procedure with the patient. Avoiding complications, handling unavoidable problems, and dealing with complications is a skill founded on knowledge and experience. Continuing education is a firm basis to push the