| Abstract|| |
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 behaviour and histologic features. Glomangiopericytoma is a borderline low malignancy tumor with a good prognosis after complete surgical resection. We report a case of 60-year-old woman who presented with progressive nasal obstruction and frequent nasal bleeding and was diagnosed as glomangiopericytoma 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.
Keywords: Glomangiopericytoma, nasal cavity, sinonasal type hemangiopericytoma
|How to cite this article:|
Roy NP, Desai DP, Jain SA. Glomangiopericytoma of nasal cavity. Indian J Pathol Microbiol 2015;58:554-6
| Introduction|| |
Glomangiopericytoma is defined as a sinonasal tumor with perivascular myoid phenotype, which was first reportedas hemangiopericytoma in 1942 by Stout and Murray  as a soft tissue tumor with characteristic vascular proliferation including branching vessels and small vessel perivascular hyalinization. The World Health Organization (WHO) classified this tumor as glomangiopericytoma in 2005. It is rare and accounts for <0.5% of all sinonasal neoplasms. The etiology is not clear although past trauma, hypertension, pregnancy and use of corticosteroids may be involved. We present a case of glomangiopericytoma arising from the septum of the right nasal cavity, which was treated by transnasal endoscopic surgery.
| Case Report|| |
A 60-year-old female presented to ENT Department Surat Municipal Institute of Medical Education and Research Hospital with 3 months history of progressive right nasal blockage, nasal bleeding and difficulty in breathing and was clinically diagnosed as a nasal polyp. Rhinoscopy was done which revealed a friable grayish pink polypoidal mass, that bleed on manipulation which was occupying right nasal cavity.
Computed tomography (CT) scan paranasal sinus view showed large ill-defined heterogeneously enhancing lesion involving the right nasal cavity widely infiltrative (right ethmoid air cells, right half of nasopharynx, right maxillary ostium, right nasal turbinates and probably right half of uncinate process and right half of sphenoid bone) in surrounding spaces; with lytic-sclerotic changes [Figure 1]. Diagnosis of vascular lesion-hemangioma/hemangiopericytoma/other neoplastic – inflammatory pathology were given on CT scan.
|Figure 1: Computed tomography scan showing soft tissue density in the anterior part of the right nasal cavity|
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The biopsy was taken and sent to Histopathology Department. Microscopic examination showed squamous epithelial lining; subepithelial tissue showed the lobular arrangement of spindle-shaped cells admixed with vascular channels of variable size with areas of hemorrhage and secondary inflammatory infiltrate. The differential diagnosis of hemangiopericytoma and solitary fibrous tumor (SFT) was made, and immunohistochemistry (IHC) advised. After a week, excision biopsy specimen was received. Received tissue was in multiple pieces, grey-brown in color and collectively measured 5 cm × 5 cm × 1.5 cm. Histopathological examination of sections showed ciliated columnar epithelium-lined tissue comprising of hyper- and hypo-cellular areas having a spindle to oval cells arranged in fascicles, storiform, and diffuse pattern. Cells had elongated plump nuclei with indistinct cell border. The spindle cells were interspersed by rich vasculature having stag horn shaped and thin-walled vessels with areas of hemorrhage, focal necrosis and infiltration of lymphocytes, plasma cells and eosinophils at places. [Figure 2]. Individual cells showed mild pleomorphism and mitotic rate of 5–7/10 high-power field. On (IHC) tumor cells were positive for vimentin and actin; negative of desmin, S-100, melanosome. CD34 was positive in blood vessels. On the basis of above histological features and IHC findings diagnosis of glomangiopericytoma of the nasal passage with low malignant potential was given. Postoperative follow-up of the patient after 6 months is unremarkable.
|Figure 2: (a) Staghorn vessels separated by oedematous stroma and presence of perivascular hyalinization. (Hematoxylin and Eosin stain, x10) (b) Tumoral proliferation of uniform spindle-shaped cells arranged in fascicles with oval to spindle-shaped nuclei with mild nuclear pleomorphism and occasional mitosis. (Hematoxylin and Eosin stain, x40) (c) Diffuse positivity of Vimentin (d) Diffuse positivity of Actin|
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| Discussion|| |
The concept of hemangiopericytoma was first described in 1942 by Stout and Murray  as a soft tissue tumor with the characteristic of vascular proliferation including branching vessels and small vessel perivascular hyalinization with otherwise marked morphologic heterogeneity. This tumor arising from the sinonasal tract was first described by Compagno and Hyams in 1976 as "hemangiopericytoma-like." Glomangiopericytoma, previously known as hemangiopericytoma is defined as the tumor arising from pericytes which can occur in any location with capillaries. The clinical and histologic characteristics of sinonasal hemangiopericytoma differ from those of hemangiopericytoma developing elsewhere in the body. Therefore, glomangiopericytomais considered as a distinct entity. It is categorized to borderline and low malignant potential soft tissue tumors of the nose and paranasal sinuses and was defined by the WHO in 2005 as a sinonasal tumor demonstrating a perivascular myoid phenotype.
It is rare and accounts for <0.5% of all sinonasal neoplasms. People in a broad range of age may be affected, with a peak incidence in the seventh decade of life and a slight female predominance., The majority of patients presents with unilateral nasal obstruction and/or recurrent epistaxis. Clinically, glomangiopericytoma is polypoid, beefy red to grayish pink, soft, fleshy to friable and edematous to hemorrhagic in appearance which bleeds easily on touching., Regional lymph node involvement is rare. The etiology is still unclear, although past trauma, hypertension, pregnancy and use of corticosteroids may be the causative factors as described by Angouridakis et al.
Microscopically this tumor consistently displays myoid appearance without cellular pleomorphism. Hematoxylin and Eosin staining shows a subepithelial well-delineated but unencapsulated cellular tumor, surrounded by the normal respiratory epithelium, characterized by diffuse growth of closely packed cells, forming short fascicles and sometimes exhibiting storiform, whorled or palisaded pattern, interspersed with numerous thin-walled and branching staghorn vessels. The neoplastic cells are uniform and elongated to oval, with a round to spindle-shaped nuclei and lightly eosinophilic cytoplasm.,,,
Compared to soft tissue hemangiopericytoma; limited mitotic activity, atypia and less amount of hemorrhage and necrosis are seen in glomangiopericytoma  Also cells are usually arranged in certain patterns such as sheets, short fascicles, and long rows. Immunohistochemically, glomangiopericytoma can be distinguished from soft tissue hemangiopericytoma by the characteristic diffuse reactivity for actin, factor XIIIA and vimentin without strong diffuse staining for CD34.
Differential diagnosis of glomangiopericytomas are with lobular capillary hemangioma (LCH), SFT, leiomyoma, and angiofibroma; than from sarcomas, which are usually clearly malignant. In contrast to glomangiopericytomas, LCH has a distinct lobular architecture often with an identifiable large, central feeder vessel giving rise to branching, progressively smaller, and ultimately slitlike vascular spaces. The tumor cells lining slit like vascular spaces have more irregular nuclear contours and mitoses. Immunohistochemically, the neoplastic cells of LCHs show vascular endothelial differentiation with CD31 and CD34 positive stains. Similar to glomangiopericytomas, SFTs consist of a cellular spindle cell proliferation effacing submucosal structures with staghorn-type vessels. SFT account for <1% of all sinonasal neoplasms. In contrast to glomangiopericytomas, SFTs have a prominent collagenous stroma with spindle cells intimately associated with ropy collagen bundles. Cells are arranged more haphazardly than in glomangiopericytoma, with no distinct growth pattern. The tumor cells show more nuclear overlap and contour abnormalities. Immunohistochemically, the neoplastic cells of SFTs stain positively for CD34, with less consistent actin staining. Sinonasal leiomyomasis very uncommon amounting to <1% of the nonepithelial sinonasal neoplasm. Prominent fascicular growth is the characteristic of leiomyoma, and the tumor cells have a fibrillary eosinophilic cytoplasm. The nuclei are more elongated with coarser chromatin and with occasional perinuclear vacuoles. Immunohistochemically, desmin staining is typically strongly positive in these tumor cells, which helps to separate them from glomangiopericytomas. Angiofibroma has abundant stromal collagen, making them eosinophilic at low-power magnification, stellate shaped tumor cells, and prominent vascular stroma. In contrast, glomangiopericytomas contain spindle cells, lack of collagenous stroma and are much more cellular. Immunohistochemically, the nuclei of angiofibroma cells are positively stained for androgen receptor 17 and β-catenin 18. Other differential diagnosis includes glomus tumor, leiomyosarcoma, and synovial sarcomas. Glomus tumor is different from glomangiopericytomas morphologically as it does not have staghorn-like vessel proliferation. Leiomyosarcoma shows coagulative necrosis, hemorrhage, and mitosis both typical and atypical; are periodic acid-Schiff positive and strong desmin positivity. Synovial sarcoma is a biphasic tumor having both epithelial and mesenchymal components; epithelial component showing cytokeratin and epithelial membrane antigen positivity and only spindle cell component showing vimentin positivity.
Glomangiopericytoma has an outstanding overall survival rate which can be achieved by complete excision. The local recurrence rate is reported as 16.8%, but the recurrences may be a consequence of incomplete excision and considered as a residual disease. In our present case, the tumor was completely resected by transnasal endoscopic excision which leads to the low potential of recurrence; even so, the patient is still highly recommended to be regularly followed up with the systemic examination.
| Conclusion|| |
Glomangiopericytoma is an uncommon, indolent, unique sinonasal neoplasm of older adults with a perivascular myoid phenotype that differs from traditional soft tissue "hemangiopericytoma" in location, biologic behavior and morphology. Characteristic histologic features include a diffuse, subepithelial, cellular proliferation of bland, spindled cells and a distinctive vascular network composed of variably sized vascular channels, some with perivascular hyalinization. Entities such as LCH, SFT, sinonasal leiomyoma, and angiofibroma are commonly confused with this lesion. Complete transnasal endoscopic excision is the choice of treatment. The regular postoperative follow-up is recommended for early finding of tumor recurrence.
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Dr. Devangi Parthiv Desai
54, Vanita Park Bunglows, Bhatar Junction, Surat - 395 017, Gujarat
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2]