Sinonasal-type Hemangiopericytoma Research Articles

Glomangiopericytoma: A rare sinonasal tumor in a young female

Glomangiopericytoma (GPC)/sinonasal type hemangiopericytoma (HPC) is a rare sinonasal neoplasm arising from the pericytes surrounding capillaries and accounts for <0.5% of all sinonasal tumors. This tumor differs from conventional soft-tissue HPC in location, biologic behavior, and histologic features. GPC is a borderline tumor of low malignant potential with a good prognosis after complete surgical resection. We report a case of a 20-year-old woman who presented with progressive nasal obstruction and frequent nasal bleeding and was diagnosed as GPC on histopathological and immunohistochemistry findings. Histological characteristics, differential diagnosis, and prognosis of this tumor are discussed in this article. This case has been reported because of its rarity and an array of differential diagnosis.

Glomangiopericytoma of the Spinal Cord

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.

Update on Select Benign Mesenchymal and Meningothelial Sinonasal Tract Lesions.

Several benign and malignant mesenchymal and meningothelial lesions may preferentially affect or extend into the sinonasal tract. Glomangiopericytoma (GPC, formerly sinonasal-type hemangiopericytoma) is a specific tumor with a predilection to the sinonasal tract. Sinonasal tract polyps with stromal atypia (antrochoanal polyp) demonstrate unique histologic findings in the sinonasal tract. Juvenile nasopharyngeal angiofibroma (JNA) arises from specialized tissue in this location. Meningioma may develop as direct extension from its intracranial counterpart or as an ectopic tumor. Selected benign mesenchymal tumors may arise in the sinonasal tract and pose a unique differential diagnostic consideration, such as solitary fibrous tumor and GPC or lobular capillary hemangioma and JNA. Although benign and malignant vascular, fibrous, fatty, skeletal muscle, and nerve sheath tumors may occur in this location, this paper focuses on a highly select group of rare benign sinonasal tract tumors with their clinicopathological and molecular findings, and differential diagnosis.

Sinonasal Glomangiopericytoma : A Case Report

Glomangiopericytoma (sinonasal-type hemangiopericytoma) is a rare sinonasal neoplasm, arising from pericytes surrounding capillaries. This tumor accounts for less than 0.5% of all sinonasal tumors. It is a borderline or low grade malignant tumor and a radial surgical resection is considered. The endoscopic and imaging findings are nonspecific. The final diagnosis is based on histopathology and immunochemistry. A 71-year-old woman presented with glomangiopericytoma of the nasal cavity manifestating nasal obstruction and epistaxis. Complete resection of the mass was performed endoscopically. We report herein a case of sinonasal glomangiopericytoma. (J Clinical Otolaryngol 2015;26:87-91)

Nuclear expression and gain-of-function β-catenin mutation in glomangiopericytoma (sinonasal-type hemangiopericytoma): insight into pathogenesis and a diagnostic marker

Glomangiopericytoma (sinonasal-type hemangiopericytoma) is a rare mesenchymal neoplasm with myoid phenotype (smooth muscle actin-positive), which distinguishes this tumor from soft tissue hemangiopericytoma/solitary fibrous tumor. Molecular genetic changes underlying the pathogenesis of glomangiopericytoma are not known. In this study, 13 well-characterized glomangiopericytomas were immunohistochemically evaluated for β-catenin expression. All analyzed tumors showed strong expression and nuclear accumulation of β-catenin. Following this observation, β-catenin glycogen serine kinase-3 beta phosphorylation region, encoded by exon 3, was PCR amplified in all cases and evaluated for mutations using Sanger sequencing. Heterozygous mutations were identified in 12 of 13 tumors. All mutations consisted of single-nucleotide substitutions: three in codon 32 (c.94G>C (n=2) and c.95A>T), four in codon 33 (two each c.98C>G and c.98C>T), two in codon 37 (c.109T>G), one in codon 41 (c.121A>G), and two in codon 45 (c.133T>C). At the protein level, these substitutions would lead to p.D32H, p.D32V, p.S33C, p.S33F, p.S37A, p.T41A, and p.S45L mutations, respectively. Previously, similar mutations have been reported in different types of cancers and shown to trigger activation of β-catenin signaling. All analyzed glomangiopericytomas showed prominent nuclear expression of cyclin D1, as previously shown for tumors with nuclear expression of β-catenin as a sign of oncogenic activation. These results demonstrate that mutational activation of β-catenin and associated cyclin D1 overexpression may be central events in the pathogenesis of glomangiopericytoma. In additon, nuclear accumulation of β-catenin is a diagnostic marker for glomangiopericytoma.

Glomangiopericytoma Simulating Cavernous Meningioma

Glomangiopericytoma is an extremely rare tumor of the nose and paranasal sinuses, accounting for less than 0.5% of all sinonasal tumors. These tumors were previously considered under the entity of hemangiopericytomas. However, because of their distinct clinical and histological features, as well as their lower malignant and metastatic potential, they were classified independently as sinonasal-type hemangiopericytoma (glomangiopericytoma). The majority of these tumors tend to remain localized within the sinonasal regions. However, some of these tumors may grow and extend into the anterior skull base region, making preoperative diagnosis of these tumors extremely challenging. Herein, we report a case of a giant glomangiopericytoma localizing into the cavernous sinus in a 48-year-old female patient who presented with mile ptosis. An expanded endoscopic endonasal approach was used to debulk the portion of the lesion in the medial compartment of the cavernous sinus. Postoperatively, the patient's ptosis resolved completely and no new defects were sustained. To our knowledge, this is the only case of glomangiopericytoma localizing solely to the cavernous sinus.

内視鏡下に摘出しえたsinonasal-type hemangiopericytoma例

We report herein on a 75-year-old woman with sinonasal-type hemangiopericytoma (HPC). She complained of nasal bleeding and a nasal tumor originated from the left nasal septum was found. It was successfully resected using endoscopic sinus surgery without preoperative embolization, because we were uncertain of its feeding artery. Its pathological examination gave a diagnosis of sinonasal-type of HPC. The sinonasal-type HPC is a rare benign sinonasal tumor, but WHO has classified it as a borderline malignancy, because it may have multiple relapses. Our patient remained free of relapse 36 months after the treatment.

Endoscopic Modified Lothrop Approach for the Excision of Bilateral Frontal Sinus Tumors

We describe the use of an endoscopic modified Lothrop approach for clearance of an extensive sinonasal-type hemangiopericytoma of the nasal cavity and paranasal sinuses with bilateral frontal sinus involvement in a 44-year-old woman. The modified Lothrop approach is conventionally used to treat sinusitis, but with some slight modifications to the technique, it can also be used for tumor excision.

A Case of Glomangiopericytoma of the Nasal Cavity

The glomangiopericytoma (a sinonasal-type hemangiopericytoma) is a rare tumor arising from the pericytes surrounding capillaries and shows variable malignant potential. We describe herein a case of a 60-year-old female with a glomangiopericytoma of the nasal cavity. After preoperative embolization, it was successfully treated with endoscopic excision. Histological examination showed multiplication of short spindle-shaped cells which reacted to immunostaining for α-smooth muscle actin. She had an uneventful postoperative course and remains well 12 months after surgery, without any tumor recurrence. Endoscopic surgery can be considered an effective treatment for the resection of this tumor type, when the tumor base can be visualized and the disease extent accurately defined.

Glomangiopericytoma of Nasal Cavity: A Rare Sinonasal Perivascular Tumor

Glomangiopericytoma, sinonasal type hemangiopericytoma, is a rare sinonasal neoplasm arising from the pericytes surrounding capillaries, and accounts for less than 0.5% of all sinonasal tumors. This tumor differs from conventional soft tissue hemangiopericytoma in location, biologic behavior, and histologic features. Glomangiopericytoma is a borderline low malignancy tumor with a good prognosis after complete surgical resection. A 42-year-old man presented with progressive nasal obstruction and frequent nasal bleeding. Rhinoscopic examination and imaging studies showed a mass occupying the right nasal cavity without bone destruction. The tumor was removed successfully by transnasal endoscopic excision. The pathological examination with immunohistochemistry confirmed the diagnosis of glomangiopericytoma. Histological characteristics, therapeutic principles and prognosis of this tumor are discussed in this article.

IMMUNOHISTOCHEMISTRY IN DIAGNOSIS OF EXTRANASOPHARYNGEAL ANGIOFIBROMA ORIGINATING FROM NASAL CAVITY: CASE PRESENTATION AND REVIEW OF THE LITERATURE

Angiofibromas are rare vascular tumors which originate predominantly in the nasopharynx and occur typically in male adolescents. Extranasopharyngeal sites such as nasal cavity and paranasal sinuses are less frequent. This review article was undertaken to evaluate the incidence, clinical features and management of extranasopharyngeal angiofibromas originating exclusivelly from nasal cavity structures. Our focus of interest was to evaluate the significance of immunohistochemical analysis in diagnosis of such extremely rare neoplasms. In the PubMed and Google Search, we found only 39 cases of nasal angifibroma, 27 males and 12 females from 1980 to 2012. The most prevalent site of origin was nasal septum, followed by inferior and middle turbinate. The commonest symptoms were nasal obstruction and epistaxis. Nasal angiofibromas are clinically distinct from nasopharyneal angiofibromas and can therefore be misdiagnosed. The differential diagnosis includes other vascular lesions, such as lobular capillary hemangioma and sinonasal-type hemangiopericytoma. Although immunohistochemistry is not necessary for differentiation between angiofibroma and capillary hemangioma, that diagnostic procedure may be helpful in distinction from sinonasal hemangiopericytoma. As an ilustration for immunohistochemical analysis, we presented a case of an elderly woman with tumor arising from the middle turbinate, diagnosed as angiofibroma. The staining was positive for CD34, CD31, factor VIII, vimentin and smooth muscle alpha-actin, and negative for desmin.

Sinonasal Glomangiopericytoma

Glomangiopericytoma (sinonasal-type hemangiopericytoma) is a rare tumor arising from the periyctes surrounding capillaries and accounts for less than 0.5% of all sinonasal tumors. It falls within the category of borderline and low-malignant-potential soft tissue tumors of the nose and paranasal sinuses. It is defined by the World Health Organization as a sinonasal tumor demonstrating a perivascular myoid phenotype. It was noted that when this lesion arose in the nasal cavity, it tended to behave in a more indolent fashion than its soft tissue counterpart, suggesting that sinonasal hemangiopericytomas represented a distinct entity. We report a case of a 68-year-old female patient diagnosed with glomangiopericytoma arising in the left sinonasal cavity and discussed the condition in terms of differential diagnosis.

Glomangiopericytoma: An uncommon sinonasal perivascular tumor with particular characteristics

Glomangiopericytoma, sinonasal type hemangiopericytoma, is a rare sinonasal neoplasm with a perivascular myoid phenotype, and accounts for less than 0.5% of all sinonasal tumors. It is considered a distinct entity and differs from conventional soft-tissue hemangiopericytoma in location, clinical and histopathologic features. Glomangiopericytoma is a borderline low malignancy tumor with a good prognosis after complete surgical resection. We present the case of a 19-year-old girl presented with a progressive nasal obstruction, anosmia and epistaxis. Rhinoscopic examination and imaging studies showed a mass occupying the left nasal cavity without bone destruction. The tumor was removed successfully by endoscopic endonasal surgery. The anatomopathology examination with immunohistochemistry confirmed the diagnosis of glomangiopericytoma. In the light of this observation and a literature review, clinical aspects, histological characteristics, therapeutic principles and prognosis of this tumor will be discussed.

Sinonasal-type hemangiopericytoma of the nasal cavity and paranasal sinus

Sinonasal-type hemangiopericytoma is a very rare disease. A 64-year-old man was admitted to our hospital because of nasal obstruction. Nasal endoscopy showed a polyp in the right nasal cavity. Imaging modalities including CT and MRI revealed polypoid tumors in the right nasal cavity and right sphenoid sinus. Excision of the tumors was performed. Macroscopically, the nasal tumor was reddish and focally cystic, and the tumor of the sphenoid sinus was reddish and solid. Microscopically, round and polygonal cells were seen to proliferate monotonously in a medullary fashion. Each tumor cell had a vesicular nucleus and amphophilic cytoplasm. The cellularity was high and mitotic figures were recognized in 6 per 10 high-power fields. Many thin-walled vessels were embedded within the tumor. No collagenization was recognized. A silver stain showed that fine argyrophilic fibers encased individual cells and a few cell nests. Mild invasion into the surrounding tissue was recognized in focal areas. The tumor showed focal cystic and hemorrhagic changes. Immunohistochemically, the tumor cells were positive for vimentin, bcl-2 and factor XIIIa, and negative for cytokeratins, epithelial membrane antigen, CD34, desmin, α-smooth muscle antigen, myoglobin, myogenin, CD31, KIT, p53 protein, CD99, and factor VIII-related antigen. Ki-67 labeling was 17%. The pathological diagnosis was low-grade malignant sinonasal-type hemangiopericytoma. The patient was followed up, but no recurrence has been seen 4 years after the operation.

F-18 FDG PET/CT Findings of Primary Sinonasal Hemangiopericytoma

Hemangiopericytoma is an unusual tumor that arises from vascular smooth muscle cells. It is mainly experienced in 4th to 5th decades of life and soft-tissue masses are frequently located in lower extremity, pelvis, and retroperitoneum. A 29-year-old man presented with a mass lesion in the right nasal cavity associated with shortness of breath as a result of nasal obstruction. An incisional biopsy was reported as sinonasal type hemangiopericytoma. The initial staging F-18 flourodeoxyglucose PET/CT successfully demonstrated severe hypermetabolism in the primary tumor location and mild-to-moderate hypermetabolism in the right upper jugular lymph node.

FDG PET/CT Findings of a Glomangiopericytoma

Glomangiopericytoma (GPC) is a rare vascular neoplasm that arises almost exclusively from the nasal cavity or paranasal sinuses. GPC is also called sinonasal-type hemangiopericytoma, although current nomenclature, as well as classification in a group with myopericytomas, better emphasizes the relatively indolent behavior of this tumor. The authors present the FDG PET/CT findings of GPC in a 53-year-old with symptoms of nasal congestion and facial pressure. CT and MRI showed a nasal mass to extend along the sphenoid ridge from the posterior nasal cavity into the posterior nasopharynx. PET showed the mass to have uniformly low-grade FDG hypermetabolism. Pathologic examination of the surgical specimen showed classic features of GPC.

Sinonasal Glomangiopericytoma: Case Report With Emphasis on the Differential Diagnosis

Glomangiopericytoma (sinonasal-type hemangiopericytoma) is an uncommon sinonasal neoplasm with a perivascular myoid phenotype. This tumor differs from conventional soft tissue hemangiopericytoma in location, biologic behavior, and histologic features. The proposed cell of origin is a modified perivascular glomuslike myoid cell. Glomangiopericytoma is an indolent tumor that tends to arise in the sinonasal tract of older adults and has a low malignant potential with excellent prognosis after surgical resection. Histologically, this lesion is composed of a diffuse, subepithelial proliferation of bland, uniform, closely packed spindled cells growing in a variety of patterns. A distinctive vascular network composed of variably sized vascular channels, the smaller of which demonstrate perivascular hyalinization, is often present. We report the case of a 48-year-old woman with epistaxis and nasal obstruction who was diagnosed with glomangiopericytoma and discuss the histologic differential diagnosis.

Sinonasal glomangiopericytoma: case report with emphasis on the differential diagnosis.

Glomangiopericytoma (sinonasal-type hemangiopericytoma) is an uncommon sinonasal neoplasm with a perivascular myoid phenotype. This tumor differs from conventional soft tissue hemangiopericytoma in location, biologic behavior, and histologic features. The proposed cell of origin is a modified perivascular glomuslike myoid cell. Glomangiopericytoma is an indolent tumor that tends to arise in the sinonasal tract of older adults and has a low malignant potential with excellent prognosis after surgical resection. Histologically, this lesion is composed of a diffuse, subepithelial proliferation of bland, uniform, closely packed spindled cells growing in a variety of patterns. A distinctive vascular network composed of variably sized vascular channels, the smaller of which demonstrate perivascular hyalinization, is often present. We report the case of a 48-year-old woman with epistaxis and nasal obstruction who was diagnosed with glomangiopericytoma and discuss the histologic differential diagnosis.

D2-40 staining in sinonasal-type hemangiopericytoma—further evidence of distinction from conventional hemangiopericytoma and solitary fibrous tumor

D2-40 is a monoclonal antibody, which reacts with a fixative-resistant epitope of lymphatic endothelium. Sinonasal-type hemangiopericytoma (SHP) and tumors of the (conventional) hemangiopericytoma/solitary fibrous tumor family (HP/SFT) are characterized by prominent vasculature. However, data concerning D2-40 labeling of these tumors are very sparse. In the present study, we investigated D2-40 staining in tissue specimens of 17 patients with SHP (male to female ratio of 2.4:1, median age of 63 years) and compared the immunolabeling with 20 cases of HP/SFT, including three SFT cases from nasal mucosa. D2-40 was detected in vascular channels of all SHP patients examined. By contrast, all cases of HP/SFT did not reveal any vascular channel being positive for D2-40, neither in the nasal cases nor in the remaining patients. This study presented for the first time data on D2-40 labeling in a series of SHP, HP/SFT, and supports the distinction of SHP from HP/SFT.

Aggressive Sinonasal Hemangiopericytoma Presenting with Liver Metastasis : A Case Report

Hemangiopericytoma is an uncommon soft tissue sarcoma. The tumour represents 2-3 percent of all soft tissue sarcomas in humans. This sarcoma arises from Zimmerman's pericytes. Sinonasal type hemangiopericytoma is an uncommon tumour of the upper aerodigestive tract. The majority of sinonasal hemangiopericytoma behave in a benign manner with excellent long term prognosis. Here we report an aggressive case of sinonasal hemangiopericytoma presenting with liver metastasis. This 42 year old male presented with epistaxis, a recurrent mass obstructing the lumen of the nose and a lump in right hypochondrium. His diagnosis after biopsy was interpreted as hemangiopericytoma. Ultrasonography showed multiple metastasis in the liver, which were also positive for metastatic sarcoma. This case highlights the fact that the usually benign sinonasal hemangiopericytoma can present with metastasis.