An institutional review of 10 cases of spinal hemangiopericytoma/solitary fibrous tumor.

BACKGROUND: Spinal hemangiopericytoma is very rare tumors with only a few case reports and one case series. We have treated ten patients between 2004 and 2017 and, thus, present a retrospective review of our patients with a focus on clinical presentation, radiological features, management, pathology, and outcome. MATERIALS AND METHODS: Histopathological data were reviewed in all the cases and clinical and follow-up details were collected from data available in our department. RESULTS: There were five males and five females, including one pediatric patient. The mean age of the patients was 34.7 years (Range 12-52 years). Dorsal, cervical, and lumbar spine involvement were found in five, four, and one patient, respectively. Intradural extramedullary tumor was the most common tumor and all patients presented motor weaknesses. Gross total resection of the tumor was done in seven patients and six patients received postoperative radiotherapy. Histopathology showed anaplastic tumor in two cases with high MIB-1 labelling index. Most patients were positive for CD34, vimentin, mic-2, and bcl-2. While the seven patients who underwent gross total resection improved significantly and were self-ambulatory in the follow-up period, two patients who underwent subtotal resection expired due to tumor metastasis. CONCLUSION: Spinal hemangiopericytoma is a very rare tumor. We present a series of cases treated at our institute for the same. Gross total resection is the goal and radiotherapy should be given in case of residual tumor or high-grade tumors. Prognosis is good after gross total excision and functional recovery can be expected in most patients.

Hemangiopericitoma primario de pulmon como hallazgo incidental. reporte de dos casos / Primary pulmonary hemangiopericytoma as an incidental finding. two case report

El hemangiopericitoma intra-pulmonar es una neoplasia poco frecuente, que se origina a partir de lo pericitos que rodean la membrana basal de capilares y vénulas dentro del parénquima pulmonar. Presentar dos casos clínicos como hallazgo incidental en su presentación conocer comportamiento y manejo de dicha neopla Paciente masculino 40 años cuadro clínico caracterizado por tos seca persistente, cansancio con dificultad para respirar con progresión a disnea rápida a pequeños esfuerzo y pérdida de peso. Paciente femenina de 37 años cuadro clínico caracterizado por dolor torácico tipo opresivo en región latero posterior de hemitórax erecho, con irradiación al hombro, radiografía de tórax póstero-anterior, se descubrió múltiples imágenes nodulares parenquimatosas bilaterales a nivel central y sub-pleural. Segundo paciente se realiza radiografía de tórax póstero-anterior se observa patrón de masa tumoral localizada en el centro del pulmón derecho, las cuales fueron confirmadas por tomografía computada de tórax. Se procedió a la recesión quirúrgica de dicha lesión para biopsia, mientras que en la paciente femenina la conducta fue más radical se practicó umonectomía derecha. Evolucionan satisfactoriamente. Reporte de biopsia y de inmunohistoquímica: emangiopericitoma primario de pulmón/tumor fibroso solitario. Ambos pacientes fueron referidos al servicio de oncología médica para terapia adyuvante.Tumor poco frecuente, su patogénesis es todavía desconocida y aunque es un tumor en apariencia y comportamiento benigno, debe tratarse como potencialmente maligno, se debe ser radical en el tratamiento y realizar un seguimiento cuidadoso, continuo y estricto del paciente(AU)

Intrapulmonary hemangiopericitoma potentially is a rare malignancy, which originates from the pericytes that surround the basal membrane of the capillaries and venules in lung parenchyma. The aim is to resent two cases as an incidental finding in its presentation and also know the behavior and management of this neoplasm. A male patient of 40 years with clinical picture haracterized by persistent dry cough, fatigue with shortness of breath with rapid progression to small effort dyspnea and weight loss and a female patient aged 37 clinical picture characterized by chest pain ppressive kind in lateral region posterior right chest, radiating to the shoulder in the chest film, multiple nodular parenchymal bilateral central and sub-pleural. The second patient so poster anterior chest radiograph where a pattern of tumor mass located in the center of the right lung, which was confirmed by computed tomography of the chest, is observed is performed. In the patient he proceeded to the surgical recession of the injury to biopsy, while in the female patient's behavior was more radical right neumonectomy was practical. They are evolving satisfactorily. Report biopsy and mmunohistochemistry for primary lung: Hemangiopericitoma / SFT. Both patients were referred to medical oncology service for adjuvant therapy. Is a rare tumor, its pathogenesis is still unknown and although a tumor in appearance and benign behavior, should be treated as malignant potential, must be radical in treatment and careful monitoring, continuous and strict patient(AU)

Anaplastic hemangiopericytoma of eyelid: An unusual location.

Hemangiopericytomas (HPCs) are rare soft tissue tumors. The eyelid is a very uncommon site for these tumors, and an anaplastic variant of HPC in the eyelid has not been reported before. A 44-year-old male presented with complaints of slowly progressive, painless swelling on the inner aspect of the left upper eyelid for 9 months. He underwent local excision of the swelling and histopathology revealed a WHO Grade III anaplastic HPC. Whole body 18 F-fluorodeoxyglucose positron emission tomography-computed tomography done postoperatively did not show any evidence of local or distant disease. The patient was planned for adjuvant radiotherapy of 60 Gy in 30 fractions over 6 weeks in view of high grade of histopathology and doubtful margins. He is disease free at the time of the last follow-up. To the best of our knowledge, this is the first case of anaplastic HPC of eyelid being reported in English literature.

[Neurolaw: its relevance for forensic psychiatry]. / Neurolaw: de relevantie voor de forensische psychiatrie.

BACKGROUND: Neurolaw is a new interdisciplinary area of research which investigates, from different perspectives, the significance of the neurosciences for law. AIM: To clarify the relevance of neurolaw for forensic psychiatry. METHOD: The importance of neurolaw developments for forensic psychiatry was analysed on the basis of recent literature. RESULTS: Some of the developments in the field of neurolaw research concern issues that are currently evaluated by forensic psychiatrists, such as risk of recidivism and legal insanity. CONCLUSION: Developments in neurolaw are relevant for forensic psychiatry in a number of ways. An important problem, not yet resolved, is to what extent psychiatry will be prepared to help in shaping these developments.

Primary intracranial haemangiopericytoma: comparison of survival outcomes and metastatic potential in WHO grade II and III variants.

Primary intracranial haemangiopericytomas (HPC) are rare, highly vascular tumours with a high propensity for local recurrence and distant metastasis. Optimal treatment includes maximal surgical resection followed by adjuvant radiotherapy. In 2007, new histopathological grading criteria were introduced to differentiate between high grade (World Health Organization [WHO] grade III) and low grade (WHO grade II) tumours. Given the rarity of this tumour, there is a paucity of information regarding the prognostic significance of histological grade. We conducted a retrospective review of our 20 year experience in treating 27 patients with HPC at our institution. Statistical analysis to compare overall survival, local recurrence rate and metastatic potential between the two grades were conducted using Kaplan-Meier analysis. The estimated median survival for grade II HPC was 216 months and for grade III tumours was 142 months. On multivariate analysis, grade II tumours were associated with better survival than grade III lesions (hazard ratio=0.16, 95% confidence interval 0.26-0.95; p=0.044). During the study period, 33% of grade III tumours developed local recurrence compared to 21% of grade II tumours. Metastases were found in 36% of grade II patients and 25% of grade III patients. There was no significant statistical difference in local recurrence rate and metastasis between the two grades. Higher histological grading in HPC is associated with worse overall survival. However based on our series higher histological grading is not associated with higher local recurrence or distant metastatic rates.

Sellar and parasellar intravascular lymphoma mimicking pituitary apoplexy.

BACKGROUND: Intravascular lymphoma (IVL) is a rare subtype of large-cell non-Hodgkin lymphoma, characterized by proliferation of lymphoma cells within the lumina of small vessels. There are no previously reported cases of IVL involving the pituitary gland presenting with neuro-ophthalmic findings. METHODS: A 68-year-old female presented with headache, right third nerve palsy, and Horner syndrome. MRI showed a 1.4-cm sellar mass consistent with a pituitary macroadenoma. Two weeks later, despite treatment with dexamethasone, the patient developed complete bilateral ophthalmoplegia and ptosis. Repeat MRI showed invasion of the clivus and cavernous sinuses, and a transsphenoidal pituitary biopsy was undertaken. RESULTS: The preliminary histopathology was consistent with bland pituitary apoplexy, but subsequent examination of an incidentally biopsied nasal polyp revealed endovascular malignant lymphoid cells that, on further scrutiny, were also present in the pituitary tissue. The diagnosis of IVL was confirmed, and the patient had an excellent clinical and radiological response to cyclophosphamide, doxorubicin, vincristine, prednisolone, and rituximab (CHOP-R) chemotherapy. CONCLUSION: IVL may involve the pituitary gland, causing sellar mass effect, cavernous sinus infiltration, and pituitary ischemia, mimicking pituitary apoplexy with neuro-ophthalmic features. It can be effectively treated with CHOP-R chemotherapy.

F-18 FDG PET/CT findings of primary sinonasal hemangiopericytoma: rare location in a young adult patient.

Hemangiopericytoma is an unusual tumor that arises from vascular smooth muscle cells. It is mainly experienced in 4th to 5th decades of life and soft-tissue masses are frequently located in lower extremity, pelvis, and retroperitoneum. A 29-year-old man presented with a mass lesion in the right nasal cavity associated with shortness of breath as a result of nasal obstruction. An incisional biopsy was reported as sinonasal type hemangiopericytoma. The initial staging F-18 flourodeoxyglucose PET/CT successfully demonstrated severe hypermetabolism in the primary tumor location and mild-to-moderate hypermetabolism in the right upper jugular lymph node.

Temporal fossa hemangiopericytoma: a case series.

OBJECTIVE: Review clinical experience with temporal fossa hemangiopericytomas (HPCs). STUDY DESIGN: Retrospective case series review. SETTING: Tertiary referral center. PATIENTS: Intracranial HPCs within the temporal fossa. INTERVENTIONS: Craniotomy for either subtotal or gross total tumor excision. MAIN OUTCOME MEASURES: Determination of clinical outcome (alive with no evidence of disease, alive with disease, and died of disease). RESULTS: 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. CONCLUSION: 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.

A case report of tumor-induced osteomalacia: eight year followed-up.

Tumor-induced osteomalacia (TIO) is a rare but potentially curable disease. It is caused by excessive renal clearance ofphosphate induced by a substance secreted from the tumor Here, the authors report a Thai patient who presented with multiple pathologic fractures, low serum phosphorus, and low tubular maximum reabsorption of phosphorus/glomerular filtration rate (TmPO4/GFR). The clinical, biochemical and bone abnormalities improved 6 months after the surgery. Two years follow-up showed no recurrence of the disease. Physicians should be aware of this condition when encountering with adult onset osteomalacia.

Haemangiopericytoma presenting with acute intracerebral haemorrhage.

Clinical presentation of brain tumour by acute haemorrhage is well known and occurs in around 5% of the cases. Haemangiopericytoma (HPC) is a richly vascularized tumour, but its clinical manifestation is most frequently related to tumour mass effect or seizures. We present the eighth case reported of a patient with acute intracerebral bleeding caused by HPC. Though HPC represents only about 2% of intracranial meningeal neoplasms it must be included in the differential diagnosis of intracranial haemorrhage.

Lipomatous hemangiopericytoma of the skull base and parapharyngeal space.

OBJECTIVE: To discuss the diagnosis and clinical management of lipomatous hemangiopericytoma. STUDY DESIGN: Case report. SETTING: Tertiary referral center. PATIENT: A 36-year-old woman was encountered with symptoms of facial numbness, blurred vision, headache, and lightheadedness of 6 weeks' duration. Magnetic resonance imaging revealed a 5.0-cm mass in the right parapharyngeal space and skull base extending inferiorly to the level of the carotid bifurcation. The mass was consistent radiographically with a glomus jugulare tumor, and surgical extirpation was performed. INTERVENTION: The patient underwent a transtemporal approach to the right posterior fossa and jugular foramen including mastoidectomy and isolation and preservation of Cranial Nerves VII, X, XI, and XII. Microscopic analysis of the mass revealed a highly cellular spindled mesenchymal tumor with a pericytoma pattern. Almost half of the mass displayed a mature lipomatous component. These findings were consistent with a lipomatous hemangiopericytoma. RESULTS: The authors describe the first case of lipomatous hemangiopericytoma involving the skull base. This rare variant of the more common hemangiopericytoma has been described previously in the retroperitoneal and lower extremities. Although one case of lipomatous hemangiopericytoma has been described in the occipital region, this is the first report of this entity involving the parapharyngeal space, skull base, and jugular foramen. CONCLUSION: The authors demonstrate that lipomatous hemangiopericytoma can occur in the parapharyngeal space and skull base. Once thought to be an aggressive variant, this tumor has an extremely low propensity for distant or local recurrence. Adjuvant therapies such as radiation and chemotherapeutic agents are reserved for recurrent or metastatic lesions.

Extremity haemangiopericytoma, a case report from Nigeria.

Haemangiopericytoma is an uncommon soft tissue sarcoma of vascular origin. It occurs more frequently in the extremities than elsewhere in the body although it can arise in any organ. Wide surgical excision is the mainstay of treatment. However, adjuvant radiotherapy and chemotherapy are desirable because the malignant nature of this tumour is frequently unpredictable. Adjuvant therapy is recommended for metastases, recurrence and incomplete resection. Long term follow up is essential in all cases as recurrence can occur several years after treatment. Where little or no experience with managing this tumor exists, it is important to be aware of its clinical behaviour and the treatment options, hence this case reports.

Intraventricular hemangiopericytoma.

Hemangiopericytoma is a rare tumor of the central nervous system and has seldom been reported intraventricularly. A 55-year-old woman presented with gradual onset of left side weakness, gait ataxia, and tendency to miss objects in the left visual field of uncertain duration. Magnetic resonance imaging with contrast showed a tumor with homogenous enhancement in the right lateral ventricle. The patient underwent right temporoparietal stealth-assisted craniotomy and surgical removal of the tumor. Histopathologic examination with ancillary tests confirmed hemangiopericytoma. Awareness that hemangiopericytoma can occur as an intraventricular tumor is important for clinicians and pathologists. Because of radiologic similarity, this tumor is not to be confused with intraventricular meningioma because the prognosis is different.

Extensive tongue hemangiopericytoma in a child: modified combined modality of treatment to preserve well functioning tongue.

Hemangiopericytoma is a rare vascular tumor, usually occurring in adults. The tumor is believed to originate from pericytes, the contractile cells surrounding capillaries. It has predilection for the musculoskeletal system. Clinically, the tumor occurs at any age, with highest incidence between the third and sixth decades and without any sex predilection. Head and neck hemangiopericytoma incidence in all age groups ranges from 9.4 to 28%. In children, head and neck hemangiopericytoma is as frequent as 35%, with the highest frequency (46%) found in infants. We report a case of hemangiopericytoma of the tongue in a 6-year-old child resembling to the infantile/congenital type, which was treated by a combination of surgery, chemotherapy, and brachytherapy. This approach has resulted in a normal functioning tongue for the past 5 years.

[Hypoglycemia associated with the production of insulin-like growth factor (IGF)-II by a hemangiopericytoma]. / Hypoglykämien bei malignem Hämangioperizytom der Leber.

HISTORY AND ADMISSION FINDINGS: A seventy-seven year-old woman with an unclear tumor of the liver suffered from recurrent hypoglycemia and was therefore admitted to our hospital. As diabetes mellitus, hyperinsulinism and reactive forms of hypoglycemia could be excluded, the presumptive diagnosis was non-islet-cell tumor hypoglycemia (NICTH). INVESTIGATIONS: Postprandial glucose levels were normal. Fasting glucose levels were 30 - 50 mg/dl. Plasma insulin-like growth factor (IGF)-I was below the normal range, IGF-II was not elevated, although 34 % of plasma IGF-II was present as "big"-IGF-II. IGF-binding protein (IGFBP)-2 was extremely elevated, whereas IGFBP-3 was within the normal range. Histological examinations of the tumor revealed a hemangiopericytoma of the liver. TREATMENT AND COURSE: : After a 2-month treatment with steroids and an experimental antiangiogenetic therapy, the glucose metabolism became stable. The tumour did not grow. Simultaneously, plasma IGF-II and "big"-IGF-II remained constant and plasma IGF-I level improved slightly. IGFBP-2, which is presumable produced by the tumor, increased, IGFBP-3 fell below the normal range. CONCLUSION: NICTH is a rare but important differential diagnosis of recurrent hypoglycemia. The tumor derived IGF-II has a higher than normal molecular weight ("big"-IGF-II) and shows different interactions with binding proteins, thus resulting in an increased bioavailability. An increased glucose uptake in different tissues as well as inhibition of hepatic gluconeogenesis and lipolysis lead to severe hypoglycemia. If surgical therapy of the tumor is not possible, symptomatic treatment with steroids may represent an effective alternative to control severe hypoglycemia.

Hemangiopericitoma de órbita / Orbital hemangiopericytoma

Objetivo:Descrever um raro caso de hemangiopericitoma orbital. Métodos: Relato de caso de associaçäo entre hemangipericitoma orbital e blefaroptose. Resultados: A exérese da neoplasia normalizou o posicionamento palpebral. Conclusöes: Lesöes orbitais anteriores säo causas de blefaroptose por compressäo do músculo elevador palpebral.