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

We report a case of a 46 year old female patient with nasal hemangiopericytoma. She complained of left nasal congestion, pus snot for 10 years, sometimes with left nasal bleeding. Physical examination: in the left nasal tract saw red soft neoplasm, roughness surface, easy bleeding when touched. Sinus CT shows: bilateral maxillary sinus, ethmoid sinus, sphenoid sinus and the left posterior nasal cavity lesions, considering inflammation with the formation of polyps, tumor not excluded. The left nasal cavity neoplasm biopsy shows: hemangioma of left nasal cavity. After admission in general anesthesia, we do transnasal endoscopic sinus openning operation and the left nasal cavity neoplasm resection. Postoperative pathological examination shows: the left nasal cavity hemangiopericytoma. Immunohistochemical showed: Vimentin(+), Smooth muscle actin(+), Desmin(-), endothelial cells CD31(-) and CD34(-). No postoperative radiotherapy or chemotherapy, no tumor recurrence. After one year of follow-up, the contact was lost.
Biopsy ; Endoscopy ; Epistaxis ; Ethmoid Sinus ; pathology ; Female ; Hemangiopericytoma ; diagnosis ; pathology ; Humans ; Inflammation ; pathology ; Middle Aged ; Nasal Cavity ; pathology ; Nasal Polyps ; pathology ; Neoplasm Recurrence, Local ; Nose Neoplasms ; diagnosis ; pathology ; Sphenoid Sinus ; pathology

Adult ; Angiomyolipoma ; pathology ; Antigens, CD34 ; metabolism ; Diagnosis, Differential ; Female ; Hemangiopericytoma ; pathology ; Humans ; Kidney Neoplasms ; metabolism ; pathology ; surgery ; Nephrectomy ; Sarcoma, Synovial ; pathology ; Solitary Fibrous Tumors ; metabolism ; pathology ; surgery ; Vimentin ; metabolism

OBJECTIVE: To improve the accuracy of diagnosis and treatment efficacy of hemangiopericytoma in nasal cavity and paranasal sinuses. METHOD: The clinical and pathological data of 5 cases with hemangiopericytoma in nasal cavity and paranasal sinuses verified by pathology were analyzed retrospectively. RESULT: Computed tomography scan revealed vascular in 5 cases. On CT scan, hemangiopericytoma generally appeared to be a uniform high density mass with obvious enhancement upon injection of contrast material. In pathological examination, there were plentiful capillaries which were like tree branch. The normal endocytes were in the inner wall of the vessel. The round, oval and spear-like pericytes scattered around the vessel. The split phase of the nucleus could be found in the tumor cell. All cases underwent surgical resection and were proved by pathological examination. The clinical data showed that the prognosis of sinonasal hemangiopericytoma was closely related to its histological grade. The recur rate in highly malignant hemangiopericytoma was obviously higher than that in middle and low malignant tumor. The rate of misdiagnosis was 80%. CONCLUSION Hemangiopericytoma is a potentially malignant tumor. Medical imaging can help to demonstrate the site, configuration, and characteristics of the tumors and contribute to the treatment. But there are not characteristic medical features. The final diagnosis must depend on the closely related to its pathological grade. The doctor should pay attention to the description of histological pathology. All the patients must be followed up carefully.
Adult ; Aged ; Female ; Follow-Up Studies ; Hemangiopericytoma ; diagnosis ; pathology ; Humans ; Male ; Middle Aged ; Nasal Cavity ; pathology ; Paranasal Sinus Neoplasms ; diagnosis ; pathology ; Retrospective Studies ; Young Adult

Actins ; metabolism ; Adult ; Antigens, CD34 ; metabolism ; Carcinoma, Renal Cell ; metabolism ; pathology ; Diagnosis, Differential ; Female ; Glomus Tumor ; metabolism ; pathology ; Hemangiopericytoma ; metabolism ; pathology ; Humans ; Hypertension ; etiology ; Juxtaglomerular Apparatus ; metabolism ; pathology ; surgery ; ultrastructure ; Kidney Neoplasms ; complications ; metabolism ; pathology ; surgery ; ultrastructure ; Nephrectomy ; Wilms Tumor ; metabolism ; pathology

Adolescent ; Antigens, CD34 ; metabolism ; Bone Neoplasms ; diagnostic imaging ; metabolism ; pathology ; Chondrosarcoma, Mesenchymal ; metabolism ; pathology ; Diagnosis, Differential ; Hemangiopericytoma ; diagnostic imaging ; metabolism ; pathology ; Humans ; Male ; Platelet Endothelial Cell Adhesion Molecule-1 ; metabolism ; Sarcoma, Synovial ; metabolism ; pathology ; Tomography, X-Ray Computed ; Vimentin ; metabolism

Diagnosis, Differential ; Fetus ; Fibroma ; metabolism ; pathology ; Fibrosarcoma ; congenital ; genetics ; metabolism ; secondary ; Hemangiopericytoma ; metabolism ; pathology ; Humans ; Kidney Neoplasms ; pathology ; secondary ; Liver Neoplasms ; pathology ; secondary ; Lung Neoplasms ; pathology ; secondary ; Male ; Nerve Sheath Neoplasms ; metabolism ; pathology ; Rhabdomyosarcoma, Embryonal ; metabolism ; pathology ; Soft Tissue Neoplasms ; congenital ; genetics ; metabolism ; pathology ; Vimentin ; metabolism

Adult ; Aged ; Antigens, CD34 ; metabolism ; Chondrosarcoma, Mesenchymal ; metabolism ; pathology ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Hemangiopericytoma ; metabolism ; pathology ; radiotherapy ; surgery ; Humans ; Male ; Meningeal Neoplasms ; metabolism ; pathology ; radiotherapy ; surgery ; Meningioma ; metabolism ; pathology ; Middle Aged ; Neoplasm Recurrence, Local ; Proto-Oncogene Proteins c-bcl-2 ; metabolism ; Solitary Fibrous Tumors ; metabolism ; pathology ; Vimentin ; metabolism ; Young Adult

Diagnosis, Differential ; Hemangiopericytoma ; metabolism ; pathology ; Hemangiosarcoma ; metabolism ; pathology ; Histiocytoma, Malignant Fibrous ; metabolism ; pathology ; Humans ; Immunohistochemistry ; Leiomyosarcoma ; metabolism ; pathology ; Nerve Sheath Neoplasms ; metabolism ; pathology ; Neuroectodermal Tumors, Primitive ; metabolism ; pathology ; Rhabdoid Tumor ; metabolism ; pathology ; Rhabdomyosarcoma ; metabolism ; pathology ; Soft Tissue Neoplasms ; metabolism ; pathology ; Urinary Bladder ; metabolism ; pathology

Adult ; Hemangiopericytoma ; diagnosis ; Humans ; Male ; Spinal Cord ; pathology