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

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

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 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

BACKGROUND: The term solitary fibrous tumor (SFT) is preferred over meningeal hemangiopericytoma (HPC), because NAB2-STAT6 gene fusion has been observed in both intracranial and extracranial HPCs. HPCs are now considered cellular variants of SFTs. METHODS: This study analyzes 19 patients with STAT6-confirmed SFTs, who were followed for over 11 years in a single institution. Ten patients (10/19, 56.2%) had extracranial metastases (metastatic group), while the remainder (9/19) did not (non-metastatic group). These two groups were compared clinicopathologically. RESULTS: In the metastatic group, the primary metastatic sites were the lungs (n = 6), bone (n = 4), and liver (n = 3). There was a mean lag time of 14.2 years between the diagnosis of the initial meningeal tumor to that of systemic metastasis. The median age at initial tumor onset was 37.1 years in the metastatic group and 52.5 in the non-metastatic group. The 10-year survival rates of the metastatic- and non-metastatic groups were 100% and 33%, respectively. The significant prognostic factors for poor outcomes on univariate analysis included advanced age (≥45 years) and large initial tumor size (≥5 cm). In contrast, the patients with higher tumor grade, high mitotic rate (≥5/10 high-power fields), high Ki-67 index (≥5%), and the presence of necrosis or CD34 positivity showed tendency of poor prognosis but these parameters were not statistically significant poor prognostic markers. CONCLUSIONS: Among patients with SFTs, younger patients (<45 years) experienced longer survival times and paradoxically had more frequent extracranial metastases after long latent periods than did older patients. Therefore, young patients with SFTs require careful surveillance and follow-up for early detection of systemic metastases.
Central Nervous System ; Diagnosis ; Follow-Up Studies ; Gene Fusion ; Hemangiopericytoma ; Humans ; Liver ; Lung ; Meningeal Neoplasms ; Necrosis ; Neoplasm Metastasis* ; Prognosis ; Solitary Fibrous Tumors* ; Survival Rate

Primary meningeal hemangiopericytoma (HPC) is a rare, aggressive dura based tumor that remarkably mimics a meningioma clinically and radiologically. Its occurrence within the cerebellopontine angle (CPA) is exceptional, and establishing the exact diagnosis is of the utmost importance since total resection remains the cornerstone of treatment. A 42-year-old man presented with a three-month history of progressively worsening vertigo and difficulty in walking. On admission, his neurological examination revealed a right peripheral facial palsy, right abducens palsy and left hemiparesis, suggesting the diagnosis of Millard-Gubler syndrome. Computed tomography and magnetic resonance imaging demonstrated a homogeneously enhancing dura based lesion of the right CPA causing major brain stem compression. There was no widening of the ipsilateral internal auditory canal. A standard retrosigmoid craniotomy was performed to access the right CPA. Exposure of the lesion revealed a well-encapsulated, gray, fibrous lesion, which appeared to originate from the tentorium. Gross total resection was achieved and confirmed radiologically. The microscopic features and the immunohistochemical profile confirmed the diagnosis of a HPC, and adjuvant radiation therapy was administered. Ten years later, the patient presented with a severe neurological deficit due to a local recurrence, but at that time refused any second intervention. He died three months later. HPC can locate within the CPA and present as a Millard-Gubler syndrome. The diagnosis should be kept in mind in case of a CPA dura based tumor. Radical surgery plus radiation therapy can maximize the recurrence-free survival and close follow-up remains mandatory to spot recurrences early.
Adult ; Brain Stem ; Cerebellopontine Angle* ; Clothing* ; Craniotomy ; Diagnosis ; Facial Paralysis ; Follow-Up Studies ; Hemangiopericytoma* ; Humans ; Magnetic Resonance Imaging ; Meningioma ; Neurologic Examination ; Paralysis ; Paresis ; Recurrence ; Vertigo ; Walking ; Wolves*

Primary meningeal hemangiopericytoma (HPC) is a rare, aggressive dura based tumor that remarkably mimics a meningioma clinically and radiologically. Its occurrence within the cerebellopontine angle (CPA) is exceptional, and establishing the exact diagnosis is of the utmost importance since total resection remains the cornerstone of treatment. A 42-year-old man presented with a three-month history of progressively worsening vertigo and difficulty in walking. On admission, his neurological examination revealed a right peripheral facial palsy, right abducens palsy and left hemiparesis, suggesting the diagnosis of Millard-Gubler syndrome. Computed tomography and magnetic resonance imaging demonstrated a homogeneously enhancing dura based lesion of the right CPA causing major brain stem compression. There was no widening of the ipsilateral internal auditory canal. A standard retrosigmoid craniotomy was performed to access the right CPA. Exposure of the lesion revealed a well-encapsulated, gray, fibrous lesion, which appeared to originate from the tentorium. Gross total resection was achieved and confirmed radiologically. The microscopic features and the immunohistochemical profile confirmed the diagnosis of a HPC, and adjuvant radiation therapy was administered. Ten years later, the patient presented with a severe neurological deficit due to a local recurrence, but at that time refused any second intervention. He died three months later. HPC can locate within the CPA and present as a Millard-Gubler syndrome. The diagnosis should be kept in mind in case of a CPA dura based tumor. Radical surgery plus radiation therapy can maximize the recurrence-free survival and close follow-up remains mandatory to spot recurrences early.
Adult ; Brain Stem ; Cerebellopontine Angle* ; Clothing* ; Craniotomy ; Diagnosis ; Facial Paralysis ; Follow-Up Studies ; Hemangiopericytoma* ; Humans ; Magnetic Resonance Imaging ; Meningioma ; Neurologic Examination ; Paralysis ; Paresis ; Recurrence ; Vertigo ; Walking ; Wolves*