Hemangiopericytoma tumor

Non-islet cell tumor hypoglycemia (NICTH) is a rarely encountered cause of hypoglycemia. It is most often caused by tumor secretion of precursor insulin-like growth factor-2 (IGF-2) which, in high concentrations, binds to insulin receptors exerting insulin-like metabolic effects. It is often associated with mesenchymal and hepatic tumors. We describe 3 cases of NICTH: a 60-year-old man with an unresectable pelvic sarcoma and two women ages 43 and 57 with metastatic hemangiopericytoma. Biochemical assessment identified hypoglycemia associated with suppressed insulin, c-peptide, and beta-hydroxybutyrate levels...

Despite multiple intracranial and extracranial relapses associated with a widely metastatic meningeal solitary fibrous tumor (formerly classified as hemangiopericytoma), a 66-year-old type 2 diabetic man was first diagnosed with paraneoplastic hypoglycemia 23 years after the original diagnosis and 12 years after the onset of extracranial metastatic disease. An enlarging mass entirely replacing the left kidney measuring 11.6 × 10 × 28 cm, which had not been locally treated before, was considered to be the putative source of IGF-2 excess...

Objective: To investigate the clinicopathological features of perivascular epithelioid cell tumor (PEComa) of the lung. Methods: Eight PEComa cases of the lung diagnosed at the First Affiliated Hospital of Soochow University, Suzhou, China from July 2008 to December 2021 were collected and subject to immunohistochemical staining, fluorescence in situ hybridization and next generation sequencing. The relevant literature was reviewed and the clinicopathological features were analyzed. Results: There were 5 males and 3 females, aged from 18 to 70 years (mean 39 years)...

Solitary fibrous tumor (SFT) is a clinically rare tumor derived from mesenchymal spindle cells. Central nervous system SFT represents only 0.09% of tumors occurring on the meninges, while intracranial solitary fibrous tumors (ISFT) are even more rare. Due to the similar genetic characteristics it shares with hemangiopericytoma, in 2016, the World Health Organization (WHO) classified it as a single disease called solitary fibrous tumor (SFT)/hemangiopericytoma. We reported a case of a 60-year-old female with an intracranial solitary fibrous tumor (ISFT)...

Solitary fibrous tumors (SFTs) are rare soft tissue tumors that can arise in the abdomen, pleura, and central nervous system, among other sites. Surgical resection is the mainstay of management, although recurrence rates remain substantial. This case describes a 73-year-old male treated surgically for both a recurrent SFT and small bowel obstruction (SBO) secondary to adhesions. The patient had undergone numerous intra-abdominal operations for malignant SFT since 1994, highlighting the importance of meticulous resection at the initial presentation of local disease...

In the World Health Organization Classification of Brain Tumors Fifth Edition, mesenchymal non-meningothelial tumors involving the central nervous system are divided into three major categories: soft tissue tumors, chondro-osseous tumors, and notochordal tumors. Soft tissue tumors are classified into four groups: fibroblastic and myofibroblastic tumors, vascular tumors, skeletal muscle tumors, and tumors of uncertain differentiation. This article will focus on solitary fibrous tumors(SFTs), which are frequently encountered clinically and continue to undergo classification revisions in the 5th edition, and outline the three newly added histological diagnoses...

Classification and molecular diagnosis of benign brain tumors, focusing on cranial and pasaspinal nerve tumors, meningioma, mesenchymal, and non-meningothelial tumors involving the central nervous system(CNS)has been reviewed based on the 5th edition of the World Health Organization Classification of Tumors of the Central Nervous System. In sporadic schwannomas, the novel fusion gene SH3PXD2A-HTRA1 , which activates the MAPK pathway, has been discovered. Meningioma shows frequent chromosomal alterations, including at the NF2 locus...

A 78-year-old woman presented to the emergency department with mild headaches and a sudden onset of blurred vision. Computerized tomography scan and magnetic resonance imaging showed what was described at first as a meningioma invading and occluding the torcular Herophili, the posterior third of the superior sagittal sinus and the proximal part of the right transverse sinus. Gross total resection of the tumor was performed without reconstructing dural sinuses. The patient was discharged home without new neurological deficit...

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Hemangiopericytomas (HPCs) are rare vascular tumors, and head and neck hemangiopericytoma (HNHPC) accounts for 11% to 16% of all HPCs, possibly occurring at any age. However, according to a recent study, HNHPC was most frequently observed in middle-aged adults and had a slight predominance of female patients. In the present case, we report the successful treatment of HNHPC.

BACKGROUND: Solitary fibrous tumors, previously known as hemangiopericytomas, originate from mesenchymal tissue and can occur at many body sites, such as the thorax, head and neck, retroperitoneal space and abdomen. These tumors are generally rare and pelvic location is extremely uncommon. Consequently, pelvic solitary tumors could be mistaken for ovarian cancer in menopausal women. This report presents a case of pelvic solitary tumor to highlight the importance of considering this diagnosis in a postmenopausal woman presenting with a solid pelvic mass, normal tumor markers and no ascites...

Intracranial hemangiopericytoma is a rare invasive tumor originating from mesenchymal fibroblasts and is prone to local recurrence and distant metastasis. This study reports a case of a 27-year-old woman who presented with severe headache, nausea and vomiting for two weeks at thirty-three weeks of gestation. Cranial magnetic resonance imaging (MRI) demonstrated a giant lesion in the bilateral parietal lobe with a size of 5.12x9.19x6.03 cm and severe edema in the surrounding brain tissue. The patient underwent four operations and 3 gamma knife radiosurgery procedures and is recovering well now...

Solitary fibrous tumor/hemangiopericytoma (SFT/HPC) is a rare primary central nervous system (CNS) tumor, included in the World Health Organization (WHO) 2016 classification. Very few cases have been described in the literature so far, especially the infantile type. It is a mesenchymal tumor of the fibroblastic type, characterized by the fusion of NAB 2 and STAT 6 genes. A 10-month-old boy presented to our neurosurgery department with complaints of increasing head circumference since 1 month of age. The magnetic resonance imaging (MRI) showed a space-occupying lesion measuring 8...

We describe a case of a 65-year-old woman affected by hemangiopericytoma/solitary fibrous tumor of the right shoulder-subclavian region. Hemangiopericytoma/solitary fibrous tumor is a rare tumor of uncertain malignancy. She reports shoulder pain and inability to abduct the arm and elevate the shoulder. Imaging showed erosion of the scapula. The patient underwent 5 sessions of "on demand" embolization in the previous 2 years scheduled for recurrence of symptoms-swelling of tissues. Further 2 treatments were achieved through embolization via 2 different microballoon catheter combined with percutaneous cryoablation with 5 probes...

A Solitary Fibrous Tumor (SFT) is a rare, aggressive, and metastasis- and recurrence- prone mesenchymal tumor. In this case report and review, we describe a rare instance of intracranial SFT, discovered for the first time. It was discovered in 2008 and following total surgical removal, the pathology was categorized as hemangiopericytoma cell tumor (HPC) at the time by WHO tumor criteria. An imaging review 8 months after surgery revealed a tumor recurrence: combined radiation and gamma-knife therapy was continued throughout this time...

Hemangioblastomas (HBs) are highly vascularized, slow-growing, rare benign tumors (WHO grade I). They account for about 2% of intracranial neoplasms; however, they are the most common primary cerebellar tumors in adults. Another frequent seat is the spinal cord (2-10% of primary spinal cord tumors). HBs are constituted by stromal and capillary vascular cells; macroscopically, HBs appear as nodular tumors, with or without cystic components. Although most of the HBs are sporadic (57-75%), they represent a particular component of von Hippel-Lindau disease (VHL), an autosomal dominant syndrome with high penetrance, due to a germline pathogenic mutation in the VHL gene, which is a tumor suppressor with chromosomal location on the short arm of chromosome three...

Meningiomas develop from meningothelial cells and approximately account for more than 30 percent of central nervous system (CNS) tumors. They can occur anywhere in the dura, most often intracranially and at dural reflection sites. Half of the cases are usually at parasagittal/falcine and convexity locations; other common sites are sphenoid ridge, suprasellar, posterior fossa, and olfactory groove. The female-to-male ratio is approximately 2 or 3-1, and the median age at diagnosis is 65 years. Meningiomas are generally extremely slow-growing tumors; many are asymptomatic or paucisymptomatic at diagnosis and are discovered incidentally...

PURPOSE OF THE STUDY Soft tissue sarcomas of the popliteal fossa are extremely rare tumors of mesenchymal origin accounting for 3%-5% of all extremity sarcomas. However, data regarding the tumor type, neurovascular involvement, and administration of radiation therapy before or after resection are limited. This study aimed to report on popliteal fossa sarcomas analyzing data from two institutions based on a relatively large patient sample. MATERIAL AND METHODS Twenty-four patients (80%; 9 men and 15 women) with a popliteal fossa soft tissue sarcoma were included in this study...

Biphenotypic sinonasal sarcoma (BSNS) is a rare low-grade malignancy occurring in the sinonasal tract that is characterized by dual neural and myogenic differentiation. Rearrangements involving the PAX3 gene, usually with MAML3, are a hallmark of this tumor type and their identification are useful for diagnosis. Rarely, a MAML3 rearrangement without associated PAX3 rearrangement has been described. Other gene fusions have not been previously reported. Herein, we report a 22 year-old woman with a BSNS harboring a novel gene fusion involving the PAX7 gene (specifically PAX7::PPARGC1A), which is a paralogue of PAX3...

OBJECTIVE: The authors aimed to explore the clinical outcomes and risk factors related to recurrence of and survival from solitary fibrous tumors (SFTs) and hemangiopericytomas (HPCs) that were reclassified according to the 2021 WHO classification of central nervous system (CNS) tumors. METHODS: The authors retrospectively collected and analyzed the clinical and pathological data of SFTs and HPCs recorded from January 2007 to December 2021. Two neuropathologists reassessed pathological slides and regraded specimens on the basis of the 2021 WHO classification...