Doege-Potter syndrome (DPS) is a rare paraneoplastic condition characterized by hypoinsulinemic hypoglycemia from a solitary fibrous tumor. The underlying mechanism is the secretion of a prohormone form of insulin-like growth factor II (IGF-II) by the tumor, which causes decreased release of glucose into the circulation. We report the case of a 27-year-old Filipino male with presumptive DPS from a recurrent right temporo-zygomatic hemangiopericytoma (HPC). The complexity of DPS requires a multidisciplinary approach. Early screening for metastases from HPC may prevent the undesirable sequelae of the disease process.
Hemangiopericytoma ; Hypoglycemia

To study the clinicopathologic characteristics,immunohistochemical features,differential diagnosis,and prognosis of solitary fibrous tumours(SFT)/hemangiopericytomas(HPC)in the maters(meninx). Methods A series of 7 cases previously diagnosed as SFT/HPC at the Department of Pathology,Peking Union Medical College Hospital,during the period from 2008 to 2018 were analyzed for clinical data,histopathology,and immunohistochemical findings.The patients were followed up and the relevant literatures were reviewed. Results These seven patients included two males and 5 females aged 22 to 77 years(mean,49 years).Headache was the most common symptom.The magnetic resonance imaging of SFT/HPC showed irregularly contoured masses and dural tail sign was observed at the periphery of the lesion in 4 cases.The major axis of the tumor ranged from 1.8 cm to 10 cm(mean,4 cm).The tumors were located in the mater in 6 cases and in the spinal meninx in 1 case.The tumors were surgically removed in all cases.Under light microscope,the tumors were formed by long round,oval or spindle cells,with rich branching vascular pattern and varying quantity of collagenous fibers bands in both sparse areas and dense areas.According the WHO classification,2 cases were in WHO grade Ⅰ,2 cases in WHO grade Ⅱ,and 3 cases in WHO grade Ⅲ.Immunohistochemistry of the paraffin-embedded tissues in all cases showed positive immunoreativity for CD34 and vimentin in all seven cases,along with positive signal transducer and activator of transcription 6 in 4 cases,negative epithelial membrane antigen and S-100 in 7 cases,and negative progestational hormone and somatostatin receptor 2 in 6 cases.The Ki-67 index ranged from 1% to 15%.Five patients with follow-up data(including 1 current case)were alive,while 2 patients were lost to follow-up. Conclusions The SFT/HPC are rare in the maters(meninx)and is clinically difficult to be differentiated from other meningioma.The combination of CD34 and signal transducer and activator of transcription 6 helps to diagnose this disease.
Adult ; Aged ; Antigens, CD34 ; metabolism ; Diagnosis, Differential ; Female ; Hemangiopericytoma ; diagnosis ; pathology ; Humans ; Immunohistochemistry ; Male ; Meninges ; pathology ; Middle Aged ; Prognosis ; STAT6 Transcription Factor ; metabolism ; Solitary Fibrous Tumors ; diagnosis ; pathology ; Young Adult

Solitary fibrous tumor (SFT) is a rare, soft tissue neoplasm that rarely presents in breast tissue, with only 27 previously reported cases. To our knowledge, only one case of malignant SFT has been reported in the English literature. A 75-year-old Caucasian woman presented to our institution with a 3-month history of a palpable left breast mass. No other symptoms, including nipple discharge or skin changes, were noted. She underwent 3 previous biopsies for right breast masses, all of which were benign, with no evidence of spindle cell neoplasm, atypical hyperplasia, or malignancy. Microscopic examination of the mass demonstrated a classic area of SFT with areas of high-grade anaplastic component. In these areas, the tumor showed atypical epithelioid cells arranged in hypercellular sheets with diminished branching vasculature, nuclear pleomorphism, and increased mitotic count (up to 9/10 high-power fields). This case represents the second case of malignant SFT in the breast.
Aged ; Biopsy ; Breast ; Epithelioid Cells ; Female ; Hemangiopericytoma ; Humans ; Hyperplasia ; Nipples ; Skin ; Soft Tissue Neoplasms ; Solitary Fibrous Tumors

A 53-year old man who had a left hemiparesis from head injury of traffic accident 20 years ago visited an emergency room with suddenly developed semi-comatose mental status. Brain CT showed 8.6-cm sized solid and cystic mass on right temporal lobe that was associated with hemorrhage. Solid lesion showed a strong enhancement after an administration of contrast media. Because of severe mass effect, emergency operation was performed. The mass was an intraparenchymal lesion with yellowish cystic fluid and the firm reddish-brown solid lesion was hemorrhagic. The lesion was totally resected. Pathologically, anaplastic solitary fibrous tumor/hemangiopericytoma was diagnosed with 70/10 high power fields. Postoperative radiotherapy of 50 Gy was done. Postoperative 2 months later, the patient was recovered to alert mental state. We report this unusual case of non-dural based intraparenchymal solitary fibrous tumor/hemangiopericytoma with high mitotic index and acute massive hemorrhage. Rapid tumor growth of hypervascular tumor might have a chance of bleeding.
Accidents, Traffic ; Brain ; Brain Neoplasms ; Central Nervous System ; Cerebral Hemorrhage ; Contrast Media ; Craniocerebral Trauma ; Emergencies ; Emergency Service, Hospital ; Hemangiopericytoma ; Hemorrhage ; Humans ; Mitotic Index ; Paresis ; Radiotherapy ; Solitary Fibrous Tumors ; Temporal Lobe

A 51-year-old woman presented with severe dizziness. The brain magnetic resonance image revealed a 5.5 cm multiloculated mass with a thick rim in the left temporal lobe. Cytological examination of frozen diagnosis of the mass showed hypercellular sheets of round and rhabdoid cells in a hemorrhagic background, and two mitotic figures were observed. Histologically, the excised dura-based mass consisted of predominantly round cells with small foci of rhabdoid tumor cells in a pseudoalveolar pattern in a hemorrhagic background, and the cells showed nuclear positivity for signal transducer and activator of transcription 6 as well as frequent mitosis. The mass was diagnosed as a grade 3 solitary fibrous tumor (SFT)/hemangiopericytoma (HPC). The cytological diagnosis of SFT/HPC is challenging because of the heterogeneous cytological findings, such as histological heterogeneity, and because there are no standardized cytological criteria for malignant SFT/HPC. Cytological findings, such as singly scattered small cells, hypercellularity, rare ropy collagen, and round and rhabdoid cells with pseudoalveolar pattern, may assist in the diagnosis of malignant SFT/HPC.
Brain ; Central Nervous System ; Collagen ; Diagnosis ; Dizziness ; Female ; Hemangiopericytoma ; Humans ; Middle Aged ; Mitosis ; Population Characteristics ; Rhabdoid Tumor ; Solitary Fibrous Tumors ; STAT6 Transcription Factor ; Temporal Lobe

Glomangiopericytoma, also referred to as a hemangiopericytoma-like tumor or sinonasal type hemangiopericytoma, is a rare tumor of the nasal cavity and paranasal sinus that has never been reported in the spinal cord. Here, we report a case of a 47-year-old man who developed glomangiopericytoma in the thoracic spine. This lesion showed a heterogeneous high signal intensity compared to the spinal cord on a T2-weighted image and an easily enhanced isointense signal on a T1-weighted image.
Hemangiopericytoma ; Humans ; Middle Aged ; Nasal Cavity ; Spinal Cord* ; Spine

Mesenchymal tumors in the liver, whether primary or metastatic, are rare. Meningeal hemangiopericytoma (HPC) is characteristically associated with delayed metastasis and the liver is one of the most common sites. Despite its consistent histological features, a pathological diagnosis of HPC in the liver is sometimes not straightforward due to its rarity and usually remote medical history of the primary meningeal tumor. In this report, the clinicopathological features of 5 cases of metastatic HPC to the liver were reviewed and described.
Adult ; Female ; Hemangiopericytoma/*pathology ; Humans ; Liver/pathology ; Liver Neoplasms/*pathology/secondary ; Male ; Meningeal Neoplasms/*pathology ; Middle Aged

OBJECTIVETo investigate the diagnostic role of STAT6 immunohistochemistry in solitary fibrous tumors (SFT)/meningeal hemangiopericytomas (HPC).

METHODEvaluated the expression of STAT6, vimentin, CD34, EMA, PR, S-100, CD56, GFAP and Ki-67 in a cohort of 37 SFT/meningeal HPC, 30 meningiomas and 30 schwannomas by immunohistochemistry staining.

RESULTSAll SFT/meningeal HPC demonstrated nuclear positivity for STAT6, and the proportion of positive tumor cells ranged from 60% to 95%, with no significant difference cases.Vimentin was strongly positive in all cases. CD34, EMA and PR positivity was found in 32 cases, 1 case and 4 cases, respectively.S-100 protein, CD56 and GFAP were negative; Ki-67 labeling index was 1%-8%. However, the meningiomas and schwannomas were negative for STAT6.

CONCLUSIONSSTAT6 is a relatively specific biomarker for SFT/meningeal HPC, and may be used in the diagnosis and differential diagnosis of SFT/meningeal HPC, especially for the atypical cases, and allows the precise pathologic diagnosis of SFT/meningeal HPC.

Antigens, CD ; analysis ; Antigens, CD34 ; analysis ; Antigens, Differentiation, T-Lymphocyte ; analysis ; Biomarkers, Tumor ; analysis ; Diagnosis, Differential ; Glial Fibrillary Acidic Protein ; analysis ; Hemangiopericytoma ; chemistry ; diagnosis ; Humans ; Immunohistochemistry ; Ki-67 Antigen ; analysis ; Meningeal Neoplasms ; chemistry ; diagnosis ; Meningioma ; chemistry ; diagnosis ; Neurilemmoma ; chemistry ; diagnosis ; S100 Proteins ; analysis ; STAT6 Transcription Factor ; analysis ; Solitary Fibrous Tumors ; chemistry ; diagnosis ; Vimentin ; analysis

OBJECTIVE: Preoperative endovascular embolization of intracranial tumors is performed to mitigate anticipated intraoperative blood loss. Although the usage of a wide array of embolic agents, particularly polyvinyl alcohol (PVA), has been described for a variety of tumors, literature detailing the efficacy, safety and complication rates for the usage of Onyx is relatively sparse. MATERIALS AND METHODS: We reviewed our single institutional experience with pre-surgical Onyx embolization of extra-axial tumors to evaluate its efficacy and safety and highlight nuances of individualized cases. RESULTS: Five patients underwent pre-surgical Onyx embolization of large or giant extra-axial tumors within 24 hours of surgical resection. Four patients harbored falcine or convexity meningiomas (grade I in 2 patients, grade II in 1 patient and grade III in one patient), and one patient had a grade II hemangiopericytoma. Embolization proceeded uneventfully in all cases and there were no complications. CONCLUSION: This series augments the expanding literature confirming the safety and efficacy of Onyx in the preoperative embolization of extra-axial tumors, underscoring its advantage of being able to attain extensive devascularization via only one supplying pedicle.
Hemangiopericytoma ; Humans ; Meningioma ; Polyvinyl Alcohol

A solitary fibrous tumor (SFT) is an extremely rare, distinct otological soft-tissue tumor. Only two such tumors in the external auditory canal have been reported. A SFT related to hemangiopericytomas (HPC), which commonly arise in the central nervous system. HPCs act malignant in many cases, while SFTs at other sites are mainly benign. A 25-year-old female presented with highly vascular tumor at the right external auditory meatus and bleeding from the mass when a crust was removed from its surface. After excisional biopsy followed by pre-operative embolization, this tumor confirmed with SFT. The recurrence rate of SFT is very low after complete resection, with a slightly increased risk of recurrence with extrathoracic tumors. We describe the third case of SFT in the external auditory canal and review the literature.
Adult ; Biopsy ; Central Nervous System ; Ear Canal* ; Female ; Hemangiopericytoma ; Hemorrhage ; Humans ; Recurrence ; Solitary Fibrous Tumors*