Occult solitary fibrous tumour of the pleura presenting as recurrent spontaneous pneumothorax.

A woman in her 30s, non-smoker, presented at the emergency department two times because of spontaneous pneumothorax. The first episode was treated with small bore catheter drainage, while during the second episode-occurring only 1 week later-thoracoscopic talcage was attempted. The postoperative course was characterised by slow clinical and radiological resolution, and recurrence 3 days after discharge. Eventually, multiportal video-assisted thoracoscopic exploration identified an interfissural solid mass. Resection and further work-up revealed the diagnosis of 'low-risk' solitary fibrous tumour (SFT) stage pT1N0M0. The interdisciplinary tumour board advised no adjuvant therapy. A CT thorax was scheduled in 1 year for follow-up. The patient was discharged without complications and has had no recurrences of pneumothorax at 6 months of follow-up. This report shows that SFT can easily be missed on initial presentation and should be considered in the differential diagnosis of pneumothorax, especially when frequently recurring.

Non-islet cell tumor-induced hypoglycemia in the setting of metastatic intracranial hemangiopericytoma: case report and review of the literature.

Intracranial hemangiopericytoma, particularly in the setting of extracranial metastatic disease, can cause severe hypoglycemia; but there is limited description of this phenomenon in the neurosurgical literature. We report the case of a male patient with recurrent meningeal hemangiopericytoma that had metastasized to multiple extracranial locations, who presented with severe, symptomatic hypoglycemia and abnormal levels of insulin, pro-insulin, insulin-like growth factors I and II, and C-peptide. This case highlights the association between hemangiopericytoma and abnormal levels of peptide hormones involved in glucose homeostasis, which may serve as a warning sign for hemangiopericytoma and/or metastatic disease.

Cerebral Hemangiopericytoma Manifesting as Epilepsia Partialis Continua: A Case Report.

Cerebral hemangiopericytomas are very rare mesenchymal tumours arising from pericytes surrounding the blood vessels in the brain. Most patients present with headaches, focal neurological findings and focal seizures with or without generalisation. Our patient chiefly complained of an uncontrollable movement of her right hand that was initially fleeting but later became continuous. Her symptoms were initially described as tremors. We found an intracranial tumour as a cause of her symptoms, suspected the tumour to be a meningioma and performed surgical extirpation which resulted in symptom resolution. Histopathology and immunohistochemistry of the excised mass revealed that the tumour was hemangiopericytoma. The patient is being closely monitored for recurrences and metastasis. Hemangiopericytomas are very rare and they rarely result in the abnormal movements of epilepsia partialis continua. Differentiation of the abnormal movements of epilepsia partialis continua from tremors is very important as is the differentiation of the tumour from meningioma. Keywords: case reports; epilepsia partialis continua; hemangiopericytoma; solitary fibrous tumors.

Successful multidisciplinary treatment of Doege-Potter syndrome: hypoglycaemia caused by paraneoplastic IGF-2 production by a metastatic haemangiopericytoma.

Hypoglycaemia due to insulin-like growth factor (IGF)-2 secretion is a paraneoplastic complication of malignancy with significant morbidity that can often go unrecognised due to its uncommon presentation. We report on a case of a 51-year-old man with metastatic haemangiopericytoma presenting with refractory hypoglycaemia, requiring continuous dextrose 10% infusion while in hospital. IGF-2 levels were significantly elevated, in keeping with a rare entity associated with solitary fibrous tumours, known as Doege-Potter syndrome. The patient was managed using uncooked cornstarch in conjunction with debulking of the hepatic tumour burden with bland IR-guided transarterial embolisation, and eventual surgical resection to treat his non-islet cell tumour hypoglycaemia (NICTH). The case highlights this rare paraneoplastic phenomenon that should be included in the differential for hypoglycaemia, especially if a history of a solitary fibrous tumour is elicited. Our case is the first to document a successful approach to treating the hypoglycaemia using preoperative transarterial bland embolisation.

Hipoglucemia tumoral dependiente y no dependiente de células ß-pancreáticas, un reto diagnóstico / Tumor and non-tumor mediated hypoglycemia by pancreatic beta cells, a diagnostic challenge

La hipoglucemia es una urgencia médica frecuente que en la mayoría de los casos es secundaria al uso de fármacos hipoglucemiantes, orales o inyectados, indicados en pacientes con diabetes mellitus. No obstante, puede presentarse en forma espontánea y severa relacionándose con múltiples condiciones clínicas, incluyendo las neoplasias. Ante una hipoglucemia de origen paraneoplásico se deben reconocer los mecanismos fisiopatológicos que la generan y establecer el diagnóstico oportuno y preciso para disminuir las complicaciones propias de este síndrome clínico. Presentamos dos pacientes con cuadro de hipoglucemia refractaria al manejo médico inicial, de aparición similar con patologías diferentes. El primer caso corresponde a un paciente con insulinoma y el segundo con un hemangiopericitoma.

Hypoglycemia is a common medical emergency which is mostly secondary to the use of oral or injected hypoglycemic drugs indicated in patients with diabetes mellitus. However, it can present spontaneously and severely in relation to multiple clinical conditions, including neoplasms. When faced with hypoglycemia associated with paraneoplastic disorders, the pathophysiological mechanisms of hypoglycemia must be recognized and a timely and accurate diagnosis must be established in order to diminish complications inherent to this clinical syndrome. We herein present two patients with hypoglycemia refractory to initial medical management, sharing similar appearance with other pathologies. The first case corresponds to a patient with an insulinoma and the second to a patient with a hemangiopericytoma.

Outcome of Epidural Hematoma: Lessons from Solitary Fibrous Tumor/Hemangiopericytoma.

BACKGROUND: Intracranial solitary fibrous tumor (SFT)/hemangiopericytoma (HPC) is rare. In this report, a case of epidural hematoma (EDH) that eventually evolved into SFT/HPC is presented. We describe the possible association between the 2 diseases, which has not been previously reported. CASE DESCRIPTION: A 40-year-old man suffered from an EDH in the right parietal area 12 years ago and accepted conservative treatment. Follow-up computed tomography (CT) scan shows that the density of the right EDH gradually changed from uniform slightly lower density to mixed density. A new CT scan revealed an epidural mass extending to the subcutaneous with local bone destruction. An operation was performed via a large right parietal craniotomy, and the final diagnosis was World Health Organization grade III SFT/HPC after histopathologic examination and immunohistochemical verification. The patient died of deterioration of brain disease 3 months after the final diagnosis. CONCLUSIONS: To our knowledge, this is the first report that HPC occurred in the epidural cavity. We are the first time to describe the possible association between EDH and HPC.

A Footprint-Like Intracranial Solitary Fibrous Tumor/Hemangiopericytoma With Extracranial Extension and Acute Intratumoral Hemorrhage.

BACKGROUND: Intracranial solitary fibrous tumors/hemangiopericytomas (ISFTs/HPCs) are extremely rare spindle-cell tumors that are mostly benign and derived from mesenchymal tissue. Extracranial metastasis and intratumoral hemorrhage are considered to be special manifestations of malignant tumors, of which extracranial metastases of ISFTs/HPCs have been reported, while tumor hemorrhage has rarely been described. Here, the authors present an interesting case of ISFTs/HPCs with acute intratumoral hemorrhage. CLINICAL PRESENTATION: A 72-year-old woman underwent brain magnetic resonance imaging (MRI) for recurrent headaches and nausea. The MRI scan showed a footprint-like space-occupying lesion in the bilateral parietooccipital lobe. This lesion had multiple cystic components and invaded the superior sagittal sinus, destroying adjacent cranial bones. While waiting for routine surgery, the patient suddenly developed acute tumor bleeding and then underwent emergency surgery. Postoperatively, the diagnosis of ISFT/HPC was confirmed by pathological and immunohistochemical analysis. DISCUSSIONS AND CONCLUSIONS: The diagnosis of ISFT/HPC mainly depends on pathological and immunohistochemical results. Malignant cases with cystic necrosis may be prone to acute hemorrhage. Early total surgical excision can provide a good clinical prognosis. Adjuvant radiotherapy is an effective supplement to surgical treatment. Metastasis and recurrence require long-term follow-up monitoring.

[Solitary fibrous tumor/hemangiopericytoma of central nervous system: a clinicopathological analysis of 60 cases].

Objective: To investigate the clinical manifestations, imaging features, clinicopathologic features, and differential diagnosis of solitary fibrous tumors/anginoblastomas (SFT/HPCs) originating in the central nervous system. Methods: Sixty cases of SFT/HPCs originating in the central nervous system were collected at Nanjing Jinling Hospital, from January 1, 2008 to December 31, 2016. The clinical data, imaging data, histomorphologic changes and immunohistochemical finding were analyzed in the sixty cases. Results: The 60 cases included 26 males and 34 females, aged 14 to 85 (median 49) years. The main clinical manifestations were headache, dizziness with nausea and vomiting. Radiologically, the tumors were large, enhancing, solid and cystic masses attached to the dura. Histopathologically, the neoplasms were composed of spindle cells with oval nuclei, inconspicuous nucleoli and moderate amount of eosinophilic cytoplasm arranged in fascicles with areas of hyalinized stroma, myxoid changes and a staghorn vascular pattern. Immunohistochemically, tumor cells of all cases were positive for vimentin (100.0%, 60/60), STAT6 (98.3%, 59/60), CD34 (61.7%, 37/60), and the tumor cells were typically positive for CD99, bcl-2, EMA and SSTR2 as well.Negative for S-100 protein, SOX10, E-cadherin, GFAP. Ki-67 index ranged from 1% to 50%. Forty cases were followed up for 6 to 82 months with average of 40 months, 30 patients were alive and 10 patients died. Conclusions: Central nervous system SFT/HPCs can be aggressive and relapses may occur several years after diagnosis. STAT6 is highly sensitive and specific for the diagnosis. Complete tumor resection is optional treatment followed by radiotherapy and chemotherapy. There is a correlation between the prognosis and the location of the disease, the histological grade, Ki-67 index, and fusion gene variants.

Non-islet-cell tumour hypoglycaemia (NICTH): About a series of 6 cases.

The purpose of this study was to analyse the characteristics of 6 patients managed in a university hospital between 1996 and 2016 for non-islet cell tumor hypoglycemia (NICTH), a form of hypoglycaemia due to the paraneoplastic secretion of IGF-2 or its related substances. RESULTS: Three of these 6 patients (50%), aged over 69 years, including 2 with acromegaloid phenotype, presented with a pleural solitary fibrous tumor (SFT), with median diameter 20 cm (interquartile range, 12.5-20.5) with a low median SUV (3.3 g/mL (QR, 2-7.5)) on 18F-FDG PET. The other 3 patients presented respectively neuroendocrine carcinoma (NEC) of the palate (70-year-old woman), retroperitoneal myxofibrosarcoma (66-year-old man) and meningeal hemangiopericytoma (36-year-old woman). All 3 were inoperable and did not respond to any therapy other than glucose solution. Corticosteroid therapy was effective in the 3 SFTs and the NEC. One of the SFTs recurred 10 years later with asymptomatic hypoglycemia, which resolved after reintervention. Median (IQR) blood glucose levels of the 6 patients was 0.4g/L (QR, 0.31-0.41), with hypoinsulinemia at 0.7mIU/L (QR 0.7-2.0), undetectable GH, low IGF-1, normal IGF-2 level in 5/6 cases, a high IGF-2:IGF-1 ratio at 26.9 (QR, 20.8-37.8), hypokalemia and hypomagnesemia. CONCLUSION: NICTH is a rare syndrome, which should be considered in the presence of hypoinsulinemic hypoglycemia with low GH and IGF-1, and a IGF-2:IGF-1 ratio>10. Corticosteroid therapy was effective in elderly subjects, particularly with solitary fibrous tumor, which was generally operable. Hemangiopericytoma and myxofibrosarcoma had poor prognosis in younger patients.

Metastatic craniocervical hemangiopericytoma presenting with hydrocephalus and papilledema - Case review.

Hemangiopericytomas are rare mesenchymal tumors with propensity to recur locally and metastasize. We report the unique case of a fifty-five-year-old male with recurrence of a previously resected craniocervical hemangiopericytoma presenting with obstructive hydrocephalus secondary to new metastatic cerebellar deposits. Emergent surgical resection of the cerebellar hemangiopericytomas was performed prior to adjuvant radiotherapy. Hemangiopericytomas are rare but important differentials for craniocervical junction lesions. Gross total resection remains the cornerstone of management with post-operative radiotherapy and chemotherapy as potential adjuncts. Tumors located in deep regions pose complex management challenges as safe maximal excision may be limited by proximal eloquent structures.

Solitary fibrous tumour/haemangiopericytoma of the spinal cord. / Tumor fibroso solitario/hemangiopericitoma de la médula espinal.

Solitary fibrous tumours/haemangiopericytomas (SFT/HPC) are fibroblastic mesenchymal neoplasms that harbour a genetic fusion of NAB2/STAT6. There are few reported cases in the central nervous system (CNS), of which spinal tumours are minority. We present a case of a 63-year-old woman with progressive paraparesis and a sensory level of T6. On the MRI we detected an intradural extramedullary lesion on T9-T10. We performed a laminectomy with an intraoperative ultrasound study in which we observed a heterogeneous lesion with an infiltrating pattern affecting the medulla at several points, and resection of the lesion. Immunohistological findings revealed positivity for vimentin, CD34 and STAT6; Ki-67 was 2%. A final diagnosis of SFT/HPC with SFT phenotype was made. SFT/HPC of the CNS is a rare neoplasm that should be taken into account in the differential diagnosis of these kinds of lesions, as clinical and MRI findings often lead to errors in diagnostic approach. The support of intraoperative ultrasound can assist in surgical decision-making.

CD34 + tumours of the orbit including solitary fibrous tumours: a six-case series.

BACKGROUND: To report six cases of CD34+ fibroblastic mesenchymal tumours, which are uncommon neoplasms in the orbit. CASE PRESENTATION: Six patients presenting with proptosis and palpable mass who were later diagnosed with fibrous solitary tumours, fibrous histocytoma or haemangiopericytoma in the orbit were included. All patients received radiologic examinations and surgical excision for histopathology and immunohistochemistry examinations. Five patients had no recurrence after a minimum follow-up of 12 months. One patient (case 6) experienced recurrence twice, and had debulking surgeries each time. At present, the patient still has remnant tumour in the orbit, but no growth has been detected during the past two years. The tumour size will be closely monitored. CONCLUSIONS: Even though fibroblastic tumours are rarely found in the orbit, they can present as a palpable mass with proptosis. Complete surgical excision is important for long-term prognosis, and immunohistochemical study is helpful for confirming pathologic diagnosis.

Tablets at the bedside - iPad-based visual field test used in the diagnosis of Intrasellar Haemangiopericytoma: a case report.

BACKGROUND: In the assessment of a pituitary mass, objective visual field testing represents a valuable means of evaluating mass effect, and thus in deciding whether surgical management is warranted. CASE PRESENTATION: In this vignette, we describe a 73 year-old lady who presented with a three-week history of frontal headache, and 'blurriness' in the left side of her vision, due to a WHO grade III anaplastic haemangiopericytoma compressing the optic chiasm. We report how timely investigations, including an iPad-based visual field test (Melbourne Rapid Field, (MRF)) conducted at the bedside aided swift and appropriate management of the patient. CONCLUSIONS: We envisage such a test having a role in assessing bed-bound patients in hospital where access to formal visual field testing is difficult, or indeed in rapid testing of visual fields at the bedside to screen for post-operative complications, such as haematoma.

Hemangiopericitoma pélvico. Una localización infrecuente de tumor vascular / Pelvic hemangiopericytoma. An unusual location of a vascular tumor

Introducción: el hemangiopericitoma es una neoplasia infrecuente de estirpe vascular. Presentamos un caso clínico de interés dada su escasa frecuencia, tamaño y localización. Caso clínico: se trata de una mujer de 63 años que consultó por dolor abdominal. Se realizaron TAC, RMN abdominal y arteriografía que mostraban una gran masa pélvica que ocupaba el espacio de Douglas y desplazaba útero, vejiga y sigma y presentaba vascularización dependiente de la arteria mesentérica interior y ambas arterias hipogástricas. Se embolizó selectivamente la vascularización propia de la neoplasia y seguidamente se resecó la masa, sin presentarse complicaciones ni intra ni postoperatorias. La anatomía patológica confirmó el diagnóstico de hemangiopericitoma. La paciente sigue controles en consultas externas sin signos de recidiva hasta la fecha. Discusión: el hemangiopericitoma procede de las células del pericito por lo que puede presentarse en cualquier localización. La localización pélvica es excepcional. Puede presentarse como dolor abdominal inespecífico, dar síntomas de compresión de órganos vecinos y, ocasionalmente, asociarse a síndromes paraneoplásicos. El diagnóstico de sospecha es mediante TAC y angiografía aunque la confirmación es histológica tras analizar la pieza de resección. El tratamiento de elección es quirúrgico, precediéndose en algunos casos de embolización preoperatoria de la vascularización de la masa. No hay acuerdo sobre la quimio/radioterapia como tratamiento primario del hemangiopericitoma, aunque se ha descrito la radioterapia adyuvante para mejorar del control local y disminuir las recurrencias. El pronóstico es bueno si se consigue una resección completa, con supervivencias a 5 y 10 años entre el 70 y el 80% según las series (AU)

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Radiosurgery for Secondary Trigeminal Neuralgia: Revisiting the Treatment Paradigm.

BACKGROUND: The mechanisms by which surgery and radiation elicit pain relief in trigeminal neuralgia (TN) secondary to mass lesions vary widely. We aimed to evaluate the outcomes of radiation to the nerve rather than to the lesion in the treatment of secondary TN. METHODS: We retrospectively reviewed all patients who underwent radiation at the University of California, Los Angeles for TN secondary to tumors. The Barrow Neurological Institute (BNI) pain score was used to evaluate pain outcomes. RESULTS: Twelve patients were identified; 4 were male and 8 were female. Their mean age at treatment was 59.8 years (range, 47.7-84.7 years). Tumor pathologies included meningioma (n = 8), squamous cell carcinoma (n = 2), vestibular schwannoma (n = 1), and hemangiopericytoma (n = 1). No patient suffered from multiple sclerosis. Ten patients underwent initial radiation targeting their tumors-radiosurgery in 3 and fractionated radiation therapy in 7 others. Only 6 among these 10 experienced at least partial relief, which lasted a mean 6 months. Radiosurgery targeting the trigeminal nerve was eventually performed. Overall, 10 of 12 (83.3%) patients experienced good initial pain relief, complete in 6 (50%) patients. Pain recurred in 6 (60%) patients, at a mean 41 months after radiosurgery to the trigeminal nerve. Three patients experienced facial sensory dysfunction postprocedurally at a mean follow-up duration of 57 months. CONCLUSION: In contrast to tumor radiation, radiosurgery to the trigeminal nerve root resulted in reasonable and longer pain reduction, on par with the literature regarding surgical resection, with low risk of additional complications.