Tumor glómico del septum nasal

Presentamos el caso clínico de una mujer de 58 años que consulta por episodios de epis-taxis autolimitadas por fosa nasal derecha asociados a hipersensibilidad al manipular dicha fosa nasal. A la exploración se evidenció en el septum nasal una lesión mucosa nodular, de consistencia dura y aspecto violáceo. Se llevó a cabo exéresis de la misma por vía endonasal, con resultado histopatológico de tumor glómico (TG) del septum nasal. El TG es una neoplasia benigna perivascular, derivada de las células musculares lisas presentes en el cuerpo glómico; estructura con función termorreguladora, formada por un shunt entre arteriolas y vénulas, sin presencia de red capilar. Los cuerpos glómicos están presentes, mayoritariamente, en extremidades (lecho ungueal). Los tumores glómicos son, verdaderamente, excepcionales en el área ORL, existiendo sólo una treintena de casos publicados hasta la fecha a nivel nasosinusal. La mayoría de casos se presentan entre la 5ᵃ y 6ᵃ década de la vida y más, frecuentemente, en mujeres. Plantea diagnóstico diferencial, fundamentalmente, con dos entidades: el paraganglioma (con el que no debe ser confundido desde el punto de vista terminológico ni histológico), y con el tumor fibroso solitario (antiguo hemangiopericitoma). El comportamiento es benigno y el tratamiento es la cirugía

We present the clinical case of a 58-year-old woman who consulted for episodes of self-limited epistaxis in the right nostril associated with hypersensitivity to manipulation of this nostril. Examination revealed a nodular mucosal lesion in the nasal septum, with a hard consistency and purplish appearance. The lesion was excised endonasally, with histopatho-logical result of a glomus tumor (GT) of the nasal septum. The GT is a benign perivascular neoplasm, derived from smooth muscle cells present in the glomus body; structure with thermoregulatory function, formed by a shunt between arterioles and venules, without the presence of capillary network. Glomus bodies are mostly present in the extremities (nail bed). Glomus tumors are truly exceptional in the ENT area, with only about thirty cases published to date at the nasosinusal level. Most cases occur between the 5th and 6th decade of life and more frequently in women. It raises differential diagnosis fundamentally with two entities: paraganglioma (with which it should not be confused from the terminological or histological point of view) and with solitary fibrous tumor (former hemangiopericytoma). The behavior is benign and the treatment is surgery.

A Case of Glomangiopericytoma in the Prevertebral Space / 대한이비인후과학회지

Glomanigiopericytoma is a rare vascular neoplasm with a perivascular myoid phenotype. It is rarely found in the neck but most commonly in the nose and paranasal sinuses. We experienced a case of 72-year-old man who presented a 5-year history of a right neck mass. The pathologic diagnosis was glomangiopericytoma. We removed the mass transcervically and the patient showed no postoperative problem. We report this case with a review of literature.

Glomangiopericytoma of the Spinal Cord

Glomangiopericytoma, also referred to as a hemangiopericytoma-like tumor or sinonasal type hemangiopericytoma, is a rare tumor of the nasal cavity and paranasal sinus that has never been reported in the spinal cord. Here, we report a case of a 47-year-old man who developed glomangiopericytoma in the thoracic spine. This lesion showed a heterogeneous high signal intensity compared to the spinal cord on a T2-weighted image and an easily enhanced isointense signal on a T1-weighted image.

Glomangiopericitoma: reporte de un caso / Glomangiopericytoma: a case report

El glomangiopericitoma es un tumor nasosinusal extremadamente raro, más frecuente en pacientes de edad avanzada y en mujeres. Si bien su etiopatogenia es desconocida, se reconocen ciertos factores predisponentes tales como hipertensión arterial, embarazo, trauma y uso de corticoides. Para su diagnóstico, resulta necesario recurrir tanto a estudio por imágenes como a la histología y técnicas de inmunohistoquímica. Microscópicamente se caracteriza por un prominente crecimiento perivascular de células uniformes ovales o fusiformes, dispuestas en fascículos cortos intercalados con capilares de diámetros variables ramificados en "asta de ciervo" y presenta inmuno-rreactividad positiva para actina, factor XIII-A y vimentina principalmente. Clínicamente este tumor presenta un comportamiento generalmente benigno, pero con una elevada tasa de recurrencia. Presentamos el caso de una mujer de 71 años, con antecedentes de hipertensión arterial, que acudió a nuestro centro por rinorrea purulenta y epistaxis unilateral derecha de 5 años de evolución. Al examen se observa masa polipoidea en fosa nasal derecha con abundante vascularización, sin otros hallazgos al examen físico. La lesión es resecada en su totalidad mediante cirugía endoscópica. El estudio histológico e inmunohistoquímico son compatibles con glomangiopericitoma. La paciente evoluciona con remisión de su sintomatología y a los dos meses desde la resección no ha presentado evidencias de recurrencia.

Glomangiopericytoma is an extremely rare sinonasal tumor, more common among elderly and women. Although its etiology and pathogeny are unknown, there are certain predisposing factors, such as arterial hypertension, pregnancy, trauma and corticosteroids. To diagnose this tumor, it is necessary imaging, histopathologic and inmunohistochemical studies. At the microscopic study, it is characterized by a prominent perivascular growth of oval-shaped or fusiform cells, arranged in short fascicles separated by capillary vessels of variable diameters, with staghorn-like ramifications, and lmmunohistochemistry positive mainly for actin, XIII-A factor and vimentin. Clinically, this tumor has a generally benign behavior, but with high recurrence percentage. We present the case of a 71 years old woman, with history of hypertension, who present in our center with a 5 years history of purulent rhinorrhea and right unilateral epistaxis, at the physical exam there is a polypoid mass in the right nasal fossa with rich vascularization, with no other findings. This tumor was resected entirely with endoscopic surgery. Histology and immunohistochemistry were compatible witch gomangiopericytoma. The patient evolve with remission of her symptoms and with no signs of recurrence at two-month follow-up.

Glomangiopericytoma: The sinonasal mimic of soft tissue hemangiopericytoma

@#A 42-year old Filipino male with a 10-month history of progressive left nasal obstruction and rhinorrhea and a clinical impression of nasal polyposis underwent endoscopic sinus surgery with partial ethmoidectomy and polypectomy. We received several dark-brown, irregular, rubbery tissue fragments with an aggregate diameter of 3 cm. Histopathologic examination shows sheets of spindly tumor cells beneath the respiratory epithelial lining. These spindle cells are closely packed and arranged in short fascicles and storiform clusters surrounding hyalinized large vessels or thin-walled submucosal blood vessels. (Figures 1 and 2) There is no atypia or necrosis. Immunohistochemical studies show strong immunoreactivity to muscle specific actin, and focal reactivity to S-100. (Figure 3) Stains for CD34, caldesmon, cytokeratin, and desmin, are negative. (Figure 4) Based on these features, we diagnosed the case as glomangiopericytoma. Glomangiopericytoma is a rare tumor arising from the pericytes surrounding capillaries, and accounts for less than 0.5% of all sinonasal tumors.1 It has a very slight female preponderance, with a peak incidence during the seventh decade of life. The most common symptom is nasal obstruction, or epistaxis, with accompanying difficulty breathing, sinusitis and headache. A mass, or polyp is the most common clinical finding.2 Hematoxylin–eosin staining shows a well-delineated but unencapsulated cellular tumor underneath the normal respiratory epithelium that effaces or surrounds adjacent normal structures.2 The tumor is composed of closely packed, uniform, oval to spindle-shaped cells, in short fascicles and in storiform, whorled or palisaded patterns. The cells surround numerous branching thin-walled, blood vessels, thus the morphologic resemblance to soft tissue hemangiopericytoma/solitary fibrous tumor. However, in contrast to hemangiopericytoma, glomangiopericytoma shows diffuse reactivity to muscle actins, and non-reactivity to CD34, while hemangiopericytoma shows the reverse reactions. Desmin and caldesmon are likewise non-reactive, distinguishing the tumor from leiomyomas or leiomyosarcomas of the upper aerodigestive tract. Cytokeratin non-reactivity distinguishes it from spindle cell carcinoma. S100, although typically negative, can be focally and weakly positive in a small percentage of tumor.3 Glomangiopericytoma is categorized as a borderline low malignancy tumor with an overall survival of >90% in 5 years but which tends to recur in up to 30% of cases. Strict follow-up is thus required, especially if complete resection is not achieved.1

Glomangiopericytoma of nasal cavity.

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.

A Case of Glomangiopericytoma of the Ethmoid Sinus / 대한이비인후과학회지

Glomangiopericytoma, a new term referring to the sinonasal type hemangiopericytoma, is a rare sinonasal tumor arising from modified perivascular glomus-like myoid cells. It is a borderline, low grade malignancy tumor, which has good prognosis following a complete surgical resection. Recently, we experienced a case of a glomangiopericytoma in a 68-year-old woman who presented with epistaxis and unilateral nasal obstruction. Endoscopic examinations revealed polypoid mass with bleeding tendency in the left middle meatus. The tumor was located mainly in the left ethmoid sinus and we removed it successfully by endoscopic approach. Histological examination and immunohistochemistry confirmed the diagnosis of glomangiopericytoma. We present the clinical and histological characteristics, therapeutic principles, and prognosis of this rare disease entity with a literature review.