2021, Number 3
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Otorrinolaringología 2021; 66 (3)
Nasosinusal glomangiopericytoma
Plata-Sánchez JA
Language: Spanish
References: 20
Page: 240-244
PDF size: 256.00 Kb.
ABSTRACT
Background: Glomangiopericytoma is a mesenchymal neoplasm derived from
the Zimmerman pericytes described by Stout and Murray in 1942. The most frequent
presentation is in the pelvic limbs followed by the abdominal cavity and the retroperitoneum.
It is considered a low-grade, slow-growing sarcoma, with a malignancy
frequency of 15-20%. The presentation in the head and neck is 15-30% and of these,
only 5% is located at the sinonasal level. It occurs in middle-aged patients, affecting
both sexes equally. The calculated survival is 80% at 5 years. Its etiology is unknown.
Clinically, it presents as a pale, painless, slow-growing mass that leads to unilateral
nasal obstruction, hyaline rhinorrhea, and occasionally epistaxis. Its diagnosis is
made by histopathological and immunohistochemical study. Differential diagnoses
include solitary fibrous tumor, leiomyoma, leiomyosarcoma, angiofibroma, synovial
sarcoma, malignant schwannoma, etc. The treatment of choice is surgical excision
with lesion-free margins.
Clinical case: A 63-year-old female patient with a long-standing tumor in the right
nostril, with an initial diagnosis by biopsy and immunohistochemistry of Ewing’s sarcoma
that after surgical management was diagnosed as nasosinusal glomangiopericytoma.
Conclusions: Glomangiopericytoma is an infrequent tumor of mesenchymal origin
and extremely rare at the nasosinusal level, with slow growth and nonspecific clinical
manifestations that make its suspicion difficult.
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