|Year : 2019 | Volume
| Issue : 1 | Page : 38-41
Glomangiopericytoma: A rare sinonasal tumor in a young female
Sankha Subhra Sinha, Dipanwita Nag, Nandini Das, Aparajita Samaddar
Department of Pathology, Medical College and Hospital, Kolkata, West Bengal, India
|Date of Web Publication||22-Aug-2019|
Dr. Sankha Subhra Sinha
Department of Pathology, Medical College Kolkata, 88 College Street, Kolkata - 700 073, West Bengal
Source of Support: None, Conflict of Interest: None
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.
Keywords: Endoscopic sinus surgery, hemangiopericytoma-like, immunohistochemistry, sinonasal
|How to cite this article:|
Sinha SS, Nag D, Das N, Samaddar A. Glomangiopericytoma: A rare sinonasal tumor in a young female. Ann Indian Acad Otorhinolaryngol Head Neck Surg 2019;3:38-41
|How to cite this URL:|
Sinha SS, Nag D, Das N, Samaddar A. Glomangiopericytoma: A rare sinonasal tumor in a young female. Ann Indian Acad Otorhinolaryngol Head Neck Surg [serial online] 2019 [cited 2020 May 29];3:38-41. Available from: http://www.aiaohns.in/text.asp?2019/3/1/38/265138
| Introduction|| |
Glomangiopericytoma (GPC) is a rare vascular neoplasm of the nasal cavity and paranasal sinuses (PNS) characterized by a pattern of prominent perivascular growth. Synonyms include sinonasal-type hemangiopericytoma (HPC), HPC-like tumor, and HPC of sinonasal origin.
GPC was first reported as HPC in 1942. HPC of sinonasal origin is clinically and pathologically different from the soft-tissue HPC. Thompson et al. analyzed 104 cases of HPC over 25 years located in the sinonasal cavity and stated the first sinonasal-type HPC description. The World Health Organization (WHO) classified this tumor as GPC in 2005. The etiology of GPC remains unknown; however, past trauma, hypertension, pregnancy, and use of corticosteroids are considered predisposing factors.
| Case Report|| |
A 20-year-old nondiabetic, nonhypertensive, euthyroid female reported with the symptoms of repeated bleeding from the nose and progressive nasal obstruction along with heaviness of head for the past 2 weeks. She had a similar episode of bleeding 1 month back. On thorough history taking, she was not having any other bleeding manifestations or symptoms of increased bleeding tendencies. She did not have symptoms of rhinitis and she denied any recent history of local trauma. She was not on any oral anticoagulants or oral contraceptive pills. Local examination revealed a polypoid mass in the left nasal cavity. Her initial laboratory workup was essentially normal except for a mild anemia. Computed tomography (CT) scan of the PNS showed features suggestive of an antrochoanal polyp [Figure 1] and [Figure 2]. On endoscopy, a left nasal angiomatous mass was seen arising from the medial to the middle turbinate. It was endoscopically removed, nasal packing was done, and the mass was sent for histopathological examination.
|Figure 1: Computed tomography scan of the paranasal sinuses coronal view showing a mass filling up the left nasal cavity|
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|Figure 2: Computed tomography scan of the paranasal sinuses axial view showing a mass filling up the left nasal cavity|
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Grossly, the specimen comprised of multiple fragmented brownish tissue pieces measuring 3 cc in volume and was all embedded in paraffin block [Figure 3].
Light microscopic examination of hematoxylin and eosin (H and E)-stained tissue sections show plenty of thin-walled vascular spaces lined by endothelium [Figure 4] and [Figure 5]. In between the vascular spaces, cords and sheets of monomorphic round tumor cells are seen, having monomorphic round vesicular nucleus and abundant eosinophilic cytoplasm with indistinct cell margins [Figure 6]. These cells are interspersed with mast cells having dense nucleus and eosinophilic cytoplasm. Some of the vascular spaces have staghorn pattern [Figure 5]. Mitosis is inconspicuous.
|Figure 6: Cords and sheets of monomorphic round tumor cells seen having round vesicular nucleus and abundant eosinophilic cytoplasm with indistinct cell margins (H and E, ×400)|
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On immunohistochemistry (IHC), tumor cell cytoplasm shows strong positivity for smooth muscle actin [Figure 7] and vimentin [Figure 8]. Tumor cells are negative for S100 and pan cytokeratin. Tumor cells are negative, but the blood vessel walls are positive for CD34 [Figure 9].
|Figure 7: Immunohistochemistry smooth muscle actin showing strong cytoplasmic positivity|
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|Figure 9: Immunohistochemistry CD34 showing positivity in endothelial cells but not in tumor cells|
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On the basis of above histological features and IHC findings, diagnosis of GPC of the nasal cavity with low malignant potential was given. Postoperative follow-up of the patient after 6 months is unremarkable.
| Discussion|| |
GPC, also called sinonasal HPC, is defined as a sinonasal tumor showing a perivascular myoid phenotype. Stout and Murray first reported it as HPC in 1942, but this definition has been questioned. Later on, Compagno in 1976 described this tumor arising from the sinonasal tract as “HPC-like” because of the lower rates of metastasis and mortality. Most recently, the WHO classified this tumor as GPC in 2005.
Architecturally, the clinical and histologic characteristics of sinonasal HPC differ from those of HPC developing elsewhere in the body. Hence, it is considered as a distinct entity.,
GPC develops in the nasal cavity and/or PNS, where it represents <0.5% of all sinonasal neoplasms., There is a very slight female preponderance. All ages can be affected, but the peak is in the seventh decade.,
Usually, patients present with unilateral nasal obstruction and/or recurrent epistaxis. Difficulty in breathing, visual disturbance, pain, and headache are less frequent complaints.,
Anatomically, GPC is polypoid, beefy red to grayish pink, soft, fleshy to friable, and edematous to hemorrhagic in appearance with bleeding on touching., Regional lymph node involvement is rare.
Imaging studies (CT scan and magnetic resonance imaging) demonstrate noncalcified soft-tissue mass in a nasal fossa or a paranasal sinus. The vascular nature can be demonstrated by contrast-enhanced imaging.,,
The diagnosis of a GPC is based on histopathology. H and E staining reveals a subepithelial circumscribed but unencapsulated cellular tumor, surrounded by a normal respiratory epithelium and is comprised tightly packed small cells interspersed with many vascular channels. A prominent perivascular hyalinization is characteristic. The tumor cells are uniform, elongated to oval, with round-to-spindle-shaped nuclei, and lightly eosinophilic cytoplasm.,,,, Compared to soft-tissue HPC, no or minimal mitotic activity and atypia, less hemorrhage, and necrosis are seen in sinonasal HPC., Immunohistochemically, GPC can also be distinctly different from soft-tissue HPC by characteristic diffuse reactivity for actins and vimentins, but no strong diffuse staining for CD34,,, whereas HPC will show positivity for all the three markers.
Differential diagnosis includes solitary fibrous tumor, myopericytoma, glomus tumor, and synovial sarcoma.
Solitary fibrous tumor is rich in collagen and different from GPC, and tumor cells are positive to Bcl-2 and negative to CD34 and actin.
The tumor cells of myopericytoma have a myxoid stroma with a solid growth pattern. It is differentiated from GPC by its morphologic character as having concentric perivascular proliferation of spindle cells.
Glomus tumor is different from GPC morphologically as it does not have staghorn-like vessel proliferation and it is usually localized in distal extremities.
Synovial sarcoma is a biphasic tumor that has both epithelial and mesenchymal components.
GPC is categorized as a borderline low malignancy tumor by the WHO 2017 Classification of Tumours, with an excellent overall survival being achieved with complete surgical excision.,, Recently, endoscopic endonasal removal has been selected as a less invasive tumor resection method. Recurrence, often due to incomplete tumor resection, develops in up to 30% of cases, making necessary a regular postoperative follow-up.,
| Conclusion|| |
GPC, or sinonasal HPC, is an uncommon unique indolent sinonasal tumor of older adults, characterized by a perivascular myoid phenotype. It differs from traditional soft-tissue HPC in location, biologic behavior, and morphology. Entities such as solitary fibrous tumor, myopericytoma, glomus tumor, and synovial sarcoma are commonly confused with this lesion. It is important to recognize GPC s as they tend to behave in a benign fashion. Complete endonasal excision is the treatment of choice. Regular postoperative follow-up is recommended to diagnose a possible recurrence.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9]