Abstract
Hemangiopericytomas are soft tissue tumors composed of pericytic cells that are characterized by their “staghorn” vascular branching and their variable clinical presentation. Fifteen to 25% of all HPC occur in the head and neck, with only 5% found in the nose or paranasal sinuses. Sinonasal hemangiopericytoma (SNHPC) is considered distinct from its soft tissue counterpart – the former showing a more uniform cellular organization, has convincing pericytic differentiation and is associated with a far better prognosis. With less than 200 cases of SNHPC reported in the literature, only limited assumptions can be made about this rare tumor. The purpose of this article is to add to the growing body of literature on this disease. We report two new cases of SNHCP – both in female patients who presented with epistaxis and anosmia. Pulsatile vascular masses were visualized with nasal endoscopy – one in the left middle meatus and the second one near the cribriform plate. CT and MRI studies show enhancing masses in the left nasal cavities with thinning and erosion of the skull base. Diagnoses were confirmed by pathology which reported spindle cell neoplasm staining positively for VEGF, NSE, factor XIIIa, S-100 protein, and CD34, and negative for actin, desmin, CD31, and pankeratin, consistent with hemangiopericytoma. In one patient, embolization of the sphenopalatine and labial artery as well as pre-operative radiation therapy was performed before complete endoscopic resection was undertaken. The second patient had a tumor invading the skull base, so a craniofacial resection was performed. Both patients remained free of disease two years after surgery. Review of the literature and treatment options are discussed.
1
Introduction
First described by Stout and Murray , hemangiopericytomas (HPCs) are benign or malignant soft tissue tumors composed of pericytic cells that are characterized by their plump spindle cells, “staghorn” vascular branching, and their variable clinical presentation . Fifteen to 25% of all HPC occur in the head and neck with only 5% found in the nose or paranasal sinuses . Sinonasal hemangiopericytoma (SNHPC), also termed glomangiopericytoma , which is considered distinct from its soft tissue counterpart – the former showing a more uniform cellular organization, has convincing pericytic differentiation and is associated with a far better prognosis . SNHPCs remain a rare, albeit benign, tumor, representing 3%–5% of all soft-tissue sarcomas and only 1% of all vascular tumors . It shows no gender or ethnic preference and has been tentatively associated with pregnancy, hypertension, long-term steroid use and trauma . With less than 200 cases of sinonasal hemangiopericytomas reported in the literature , only limited assumptions can be made about the tumor. Here we present another two cases of sinonasal hemangiopericytoma to add to the small but growing body of literature on this disease.
2
Case presentations
2.1
Patient 1
A 42-year-old Chinese woman presented with a 6-week history of epistaxis from the left side of her nose. Nasal endoscopy revealed a pulsatile, vascular mass in the left middle meatus. There was no active bleeding and no CSF rhinorrhea ( Fig. 1 ). She had no neurologic symptoms except for anosmia for 1 year, and no visual changes. A CT of the sinuses with contrast was obtained, which showed a well-defined homogeneously enhancing mass in the left nasal cavity with thinning and erosion of the left cribriform plate and the left lamina papyracea ( Fig. 2 ). Magnetic resonance imaging (MRI) of the sinuses with contrast showed a well-defined homogenously enhancing mass in the left nasal cavity with thinning of the left cribriform plate without intracranial extension ( Figs. 2 and 3 ). Because of its vascularity and the lack of visual changes, headache, or facial pain, the pre-operative diagnosis was a soft-tissue tumor or a lymphoma. (See Figs. 4–8 .)
The patient initially underwent an endoscopic biopsy in the operating room due to increased vascularity and the risk of bleeding. There was excessive bleeding intra-operatively, so total resection was not possible. Nasal packing was required for several days, and interventional radiology performed embolization of the sphenopalatine and labial artery.
Biopsy showed a spindle cell neoplasm consistent with hemangiopericytoma. Immunohistological analysis was performed with the tumor cells staining positive for VEGF, NSE, factor XIIIa, S-100 protein, and diffusely positive for CD34. Results were negative for actin, desmin, CD31 and pankeratin. The mitotic rate was 4 per 10 high-power fields with a MIB1 proliferative index of 15%. The tumor was pathologically diagnosed as a spindle cell tumor consistent with hemangiopericytoma with potentially aggressive behavior.
Given that some bleeding continued to occur following embolization, upfront radiation therapy was performed to help control bleeding and shrink the tumor to increase the likelihood of obtaining negative margins during surgical resection. Complete endoscopic resection was performed with negative margins, with 250 cm 3 of blood loss. The patient remained free of disease 2 years after surgery.
2.2
Patient 2
A 30-year-old Hispanic female presented to our institution with a nasal tumor which was discovered on an MRI taken because the patient had complained of headaches and progressive hyposmia. She had presented with epistaxis three and a half years prior to seeing us, was diagnosed with sinonasal hemangiopericytoma, and had underwent endoscopic resection. Three years after her initial resection, she again presented to her surgeon with recurrent epistaxis, was found have a recurrence of her tumor and underwent her second endoscopic resection of the tumor. During the 6 months prior to presentation, she began noticing headache, increasing hyposmia, and paresthesia over her left foot and toe. MRI of the head revealed a second recurrence of the tumor in the roof of the left ethmoid sinus with extension intracranially. She reports being a smoker but denied nasal obstruction, diplopia, seizures, cerebrospinal fluid leak or eye pain.
She underwent a cranio-facial surgical resection that included a frontal craniotomy for resection of the intracranial component and reconstruction using a pericranial flap. She also underwent an endoscopic resection of the intranasal tumor at the same setting. Follow-up at 18 months with imaging and endoscopic surveillance reveals no recurrence. Her only complaint following surgery was anosmia but her headaches and epistaxis had disappeared.
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
