Sinonasal hemangiopericytoma: A rare case report with review of literature (original) (raw)

Sinonasal hemangiopericytomas: Clinicopathologic and imaging findings

Ear, nose, & throat journal

Hemangiopericytomas are rare soft-tissue neoplastic lesions that can arise in any part of the body. They are mesenchymal tumors that account for 3 to 5% of all soft-tissue sarcomas and 1% of all vascular tumors. They originate in extravascular cells (pericytes). Some 15 to 30% of all hemangiopericytomas occur in the head and neck; of these, approximately 5% occur in the sinonasal area. We describe our brief retrospective review of 7 histologically proven cases of sinonasal hemangiopericytoma, and we discuss the imaging characteristics and clinical and pathologic findings in these patients.

Sinonasal Hemangiopericytomas: A Clinicopathologic and DNA Content Study

Archives of Otolaryngology - Head and Neck Surgery, 1992

Hemangiopericytomas are rare soft-tissue neoplastic lesions that can arise in any part ofthe body. They are mesenchymal tumors that accountfor 3 to 5% ofall soft-tissue sarcomas and 1% ofall vascular tumors. They originate in extravascular cells (pericytes). Some 15 to 30% ofall hemangiopericytomas occur in the head and neck; ofthese, approximately 5% occur in the sinonasal area. Wedescrib e our brief retrospective review of 7 histologically proven cases ofsinonasal hetnangiop ericytoma, and we discuss the imaging characteristics and clinical and pathologic findin gs in these patients.

Sinonasal hemangiopericytoma: two case reports and review of literature

2022

Hemangiopericytoma is a rare tumor that arises from pericytes, classi ed as a low-grade malignant vascular tumor. It is characterised by a poorly clinical presentation and histologically, often confused with other brous tumors, which could pose di culties on the diagnosis. The nasal cavity is an uncommon site. The rarity of this tumor imply the absence of management guidelines. So far, it still controversial regarding wether the endonasal surgery could control the tumor resection despite the wide performance, nowdays, of endoscopic techniques. It still unclear also conserning the use of embolization before surgery removal and the place of chemotheray. The high rate of recurrence impose long-term follow-up. So that, we report our experience through two cases and we aimed to discuss the clinical features and the differents treatement procedures. In fact, they are two women who consulted for epistaxis and nasal obstruction. Vascular masses were visualized with nasal endoscopy-one in the left nasal cavity and the second one had a destructive mass of the right hemiface. CT and MRI helped identify the mass, its margins and its extirpability. DiagnosIs was con rmed by histology. In one patient, surgery was performed. The second patient had an agressive and invading tumor for which chemotherapy was performed. The rst patient remained free of disease after surgery. The second one had a fatal evolution.

Sinonasal-Type Hemangiopericytoma

The American Journal of Surgical Pathology, 2003

Sinonasal-type hemangiopericytoma is an uncommon upper aerodigestive tract tumor of uncertain cellular differentiation. We report 104 cases of sinonasal-type hemangiopericytoma diagnosed between 1970 and 1995 from the files of the Armed Forces Institute of Pathology. There were 57 females and 47 males ranging in age from 5 to 86 years (mean 62.6 years). The most common clinical presentation was airway obstruction (n ‫ס‬ 57) and/or epistaxis (n ‫ס‬ 54), with symptoms averaging 10 months in duration. The tumors involved the nasal cavity alone (n ‫ס‬ 47) or also a paranasal sinus (n ‫ס‬ 26), were polypoid, and measured an average of 3.1 cm. Histologically, the tumors were submucosal and unencapsulated and showed a diffuse growth with fascicular (n ‫ס‬ 37) to solid (n ‫ס‬ 50) to focally whorled (n ‫ס‬ 7) patterns. The tumor cells were uniform in appearance with minimal pleomorphism and had spindleshaped (n ‫ס‬ 82) to round/oval (n ‫ס‬ 18) nuclei with vesicular to hyperchromatic chromatin and eosinophilic to amphophilic to clear-appearing cytoplasm with indistinct cell borders. Multinucleated (tumor) giant cells were identified in a minority of cases (n ‫ס‬ 5). Mitotic figures were inconspicuous and necrosis was absent. The tumors were richly vascularized, including staghorn-appearing vessels that characteristically had prominent perivascular hyalinization (n ‫ס‬ 92). An associated inflammatory cell infiltrate that included mast cells and eosinophils was noted in the majority of cases (n ‫ס‬ 87). The immunohistochemical profile included reactivity with vimentin (98%), smooth muscle actin (92%), muscle specific actin (77%), factor XIIIa (78%), and laminin (52%). Surgery was the treatment of choice for all of the patients; adjunctive radiotherapy was given to four patients. Recurrences developed in 18 patients within 1-12 years from diagnosis. Ninety-seven patients were either alive (n ‫ס‬ 51, mean 16.5 years) or dead (n ‫ס‬ 46, mean 9.6 years) but free of disease. Four patients had disease at the last follow-up: three died with disease (mean 3.6 years) and one patient is alive with disease (28.3 years). Recurrent tumor (17.8%) can be managed by additional surgery. The majority of sinonasal-type hemangiopericytomas behave in a benign manner with excellent long-term prognosis (88% raw 5-year survival) following surgery alone. Sinonasal-type hemangiopericytomas have a characteristic light microscopic appearance with an immunophenotypic profile resembling that of glomus tumors.

Recurrent Sinonasal Hemangiopericytoma: A Rare Case Report

International Journal of Otolaryngology and Head & Neck Surgery, 2020

Hemangiopericytoma (HPC) is a rare vascular tumor arising from capillary pericytes. This tumor represents 3%-5% of all soft tissue sarcomas and 1% of all vascular tumors. Only 15%-30% of cases occur in the head and neck region, among which sinonasal hemangiopericytoma (HPC) is accounted for around 5% of cases. Hemangiopericytoma (HPC) has a tendency for recurrence. Here, a case of recurrent nasal hemangiopericytoma (HPC) after 12 years of treatment is reported.

Hemangiopericytoma of the neck

Head & face medicine, 2010

Hemangiopericytoma (HPC) is an exceedingly rare tumor of uncertain malignant potential. Approximately 300 cases of HPC have been reported since Stout and Murray described HPCs as "vascular tumors arising from Zimmerman's pericytes" in 1942. After further characterization, the WHO reclassified HPC as a fibroblastic/myofibroblastic tumor. Long term follow up is mandatory because the histologic criteria for prediction of biologic behavior are imprecise. There are reports of recurrence and metastasis many years after radical resection. The head and neck incidence is less than 20%, mostly in adults. We report herein a case of HPC resected from the neck of a 74-year-old woman, who presented in our department with a painless right-sided neck mass. The mass was well circumscribed, mobile and soft during the palpation. The skin over the tumor was intact and normal. Clinical diagnosis at this time was lipoma. A neck computer tomography scan showed a large submucosal mass in the ...

Hemangiopericytoma like tumor - a diagnostic challenge

Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of India, 2006

Haemangjopericytoma is a vascular tumour rarely seen in common practice, especially in head & neck. Hemangjopericytoma - like tumor is an even more rare entity and only 70 cases have been described arising from the nose & paranasal sinuses. The aim of this paper is to review the literature and highlight the ability of the tumor to masquerade as a less ominous, more commonly occurring benign growth of the region thereby making the diagnosis even more elusive.

Review Article Hemangiopericytoma: A Case Report and Mini-Review

2015

The term hemangiopericytoma was first used by Stout and Murray in 1942 describing a tumor which is distinguished histologically from other types of vascular neoplasm by its proliferation of pericytes. Hemangiopericytoma is a rare neoplasm that was originally described as a vascular tumor derived from the pericytes. They account for 2-3% of all soft tissue sarcomas in humans and they occur mainly in the musculoskeletal system. 15-30% of all hemangiopericytomas occur in the head and neck region. Only 5% are located in the sinonasal region, where they display a more benign behavior than in other parts of the body. Herein, we are presenting an extremely rare case report of hemangiopericytoma in a 32 year old male patient with a brief overview regarding its epidemiology, macro-and microscopical characteristics, the clinic-pathological findings and the treatment of this extremely rare vascular neoplasm.

Hemangiopericytoma– Rarest of the rare at uncommon site

IP Innovative Publication Pvt. Ltd., 2017

Hemangiopericytoma are vascular lesions are more commonly reported in the soft tissues when compared to reports in the hard tissues. These are considered to originate from the pericytes in the blood capillaries and hence can occur anywhere in the human body. The incidence of this entity among the other vascular lesion is only 0.1%. The most common locations reported are the brain, lower extremities, pelvic area, head, and neck. Hemangiopericytoma is most commonly located in the nasal cavity and paranasal sinuses in the head and neck region. Hemangiopericytoma are painless masses and may not have any associated symptoms but may turn symptomatic when other adjacent structures are affected. Though rare, their prognosis is better. This is because of the tendency to be less aggressive and not metastasizing. Here we attempt to report this pathology at zygomatic bone which is the rarest site of the occurrence of the vascular lesions as seen in literature.

Head and neck hemangiopericytoma in a child: case report

Sao Paulo Medical Journal, 2004

CONTEXT: Hemangiopericytoma is a relatively rare tumor, first described in 1942, with approximately 300 cases described in the literature to date. In most cases, it affects the trunk and lower extremities. The head and neck incidence is less than 20%, mostly in adults. We describe a case of malignant head and neck hemangiopericytoma in a child. TYPE OF STUDY: Case report. CASE REPORT: A twelve-year-old male patient noted the presence of a firm painless right-side retroauricular lymph node of 1 cm in diameter, which at first remained unchanged for six months, but subsequently enlarged progressively. He denied having had previous trauma at that site. In November 2000, he presented nasal obstruction and voluminous epistaxis that required hospitalization and blood transfusion. During dental treatment one month later, a cranial x-ray revealed bone alterations. A subsequent computed tomography scan showed an extensive lesion of soft tissue density that had invaded the maxillary fossa, eroding the skull base and middle and nasal fossa. The child was then referred to our service, where biopsy was performed, giving a diagnosis of hemangiopericytoma. Shortly afterwards, magnetic resonance imaging revealed that this lesion had undergone significant growth, while maintaining the same invasion pattern. The patient was submitted to conservative surgery in April 2001, with only partial resection of the tumor because of its extent. Histopathological examination of the specimen confirmed the presence of malignant hemangiopericytoma. Following the surgery, the patient presented fast regrowth of the lesion, with partial response to chemotherapy and radiotherapy.