Glomangiopericytoma in the Nasal Cavity: A Rare Case Report

INTRODUCTION

Glomangiopericytoma (GPC) is a rare tumor in the nasal cavity and paranasal sinuses (PNS) emerging from pericytes surrounding the capillaries and accounts for less than 0.5% of all sinonasal neoplasms.^1,2 Stout and Murray in 1942 reported its first case and classified it as hemangiopericytoma.³ Glomangiopericytoma was classified as a sinonasal soft tissue neoplasm of borderline or low malignant potential with an excellent survival rate by the World Health Organization (WHO) in 2005.^4,5 The possible etiologic factors of GPC are hypertension, pregnancy, trauma, and long-term corticosteroid use.² We report a case of GPC arising in the left nasal cavity presenting as nasal discharge, nasal obstruction, loss of sense of smell, headache, and one episode of nasal bleeding from the same nostril. This case report has been reported in line with the SCARE 2020 criteria.⁶

CASE PRESENTATION

A 64-year-old woman presented with nasal obstruction, nasal discharge, loss of sense of smell, headache, and one episode of nasal bleeding to the Otolaryngology department. She had a history of hypertension for which she was taking medications. She had a history of surgical intervention that took place 40 years ago for a mass lesion in the left nasal cavity. She did not have any documentation of the surgical procedure. She has a scar mark on the left lateral rhinotomy incision. The patient complaints had been better in the early postoperative period after surgery, but reappeared 6 months back. Her main complaints were nasal obstruction, left nasal discharge, loss of sense of smell, headache, and a single episode of nasal bleeding from the same nostril. On examination, there was a polypoid tissue originating from the lateral wall of the left nasal cavity. The nasal septum was deviated to the right side. On probing, the polypoid tissue bleeds on the touch. Posterior rhinoscopy test showed the absence of polypoid tissue in the nasopharynx. A contrast-enhanced computed tomography (CECT) scan of the nose and PNS reported a well-defined soft tissue density lesion of size 30 × 18 × 40 mm in the left anterior nasal cavity showing mild peripheral heterogeneous contrast enhancement. The lesion is closely abutting the walls of the nasal cavity, middle and inferior turbinate with a deviation of nasal septum toward the right side. No evidence of extension into the maxillary sinus and no evidence of bony erosion were noted (Fig. 1). A biopsy was taken preoperatively which reported a pedunculated vascular lesion with small capillaries arranged in a lobular fashion. Reparative changes are seen in the stroma. Mixed lymphoplasmacytic inflammation and granulation tissue are identified. Features were suggestive of lobular capillary hemangioma. After collecting the biopsy report, a complete surgical excision was planned under general anesthesia using external approach (Fig. 2). The nasal pack was removed 48 hours after the surgery. Postoperative histopathological examination reported a lesion covered by pseudostratified ciliated mucosa. The sub-mucosal lesion is comprised of sheets and fascicles of monomorphic spindled to round cells with minimal eosinophilic cytoplasm and inconspicuous nucleoli (Fig. 3). Embedded small caliber vessels are seen. Large stag-horn-shaped vessels are seen in the center of the lesion. Perivascular hyalinization and necrosis are identified in few foci (Fig. 4). These features are suggestive of GPC. Suture removal was done at the 7th postoperative day (Fig. 5). The postoperative follow-up was at 2 weeks and 6 weeks, and minimal incrustations were noted over the surgical site. The patient is in regular follow-up with no signs of recurrence to date.

DISCUSSION

Mass in the nasal cavity needs detailed history-taking and proper clinical examination prior to management. Here we present a case of unilateral, soft, insensitive, and grayish nasal mass that came to our outdoor patient department with CT scan findings suggestive of left nasal polyp. Meticulous clinical examination gave us the suspicion of nasal mass not being polyp as it was bleeding on probing. Preoperative biopsy was planned in the operating room which suggested the mass being lobular capillary hemangioma. Then complete surgical excision was done under general anesthesia via an external approach as it was the revision case operated 40 years back with the same approach. Postoperative histopathologic examination report shows the features suggestive of GPC. Glomangiopericytoma has an appearance similar to inflammatory nasal polyps, and therefore can create confusion for clinicians.⁷ Histopathological tissue examination is the gold standard for definitive diagnosis.⁸ Preferred management option includes complete surgical excision having 5-year survival rate of 88%.⁹

We found too many cases of GPC on reviewing the literature but many more case reports are needed to generate adequate data.

Teamwork is required for the proper diagnosis and management of such cases, hence we must be vigilant at every step to do the best for the patient.

So, the take-home lesson from this case is detailed history-taking, and meticulous clinical examination are the key to not missing the diagnosis.

CONCLUSION

Glomangiopericytoma is a rare sinonasal neoplasm with borderline or low malignant potential. As it is a rare tumor having ambiguous clinical and radiological signs, a high index of suspicion is required for the diagnosis of GPC. Complete surgical excision is the mainstay of treatment. Rare case reports are required to generate an adequate data pool and make guidelines for diagnosis and treatment outcomes.

REFERENCES

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