engleski

NASAL GLOMUS TUMOR

Glomus tumors or glomangiomas are rare benign neoplasms (2% of all soft tissue neoplasms) first described by Masson in 1924. They represent a vascular hamartomatous derivative of the glomus body, a normal intradermal arteriovenous anastomosis surrounded by con-nective tissue. Glomus body are present in the whole body but most numerous in fingers and toes. They have an abundant symphatetic innervation which is very important for their function – prevent blood flow to upper skin layers when the body is exposed to cold temperature. Glomus tumors are more common in women between 30 and 50 years of age and are not associated with any other condi-tion. Glomus tumors usually appear as solitar lesions. However, a multiple glomus tumor syndrome has been described. Glomangiosarcoma is an exceptionally rare malignant variant of the glomus tumor. Here, we report a case of a 9-year old patient with clinically diagno-sed apical nasal tumor. The recieved material was an irregular skin specimen (0.4 cm in diameter) with a yellowish formation on its sur-face (0.1 cm in diameter). Microscopically, the epidermal layer was intact, a formation built of vascular spaces covered with several layers of regular cuboid glomus cells was present in the dermal layer. The tumor was well circumscribed (Figure 1). Malignancy features, such as infiltrative growth, necrosis, nuclear pleomorphism or mito-tic activity, weren't found. Immunohistochemically, smooth muscle cells were confirmed by positive reaction to SMA (Figure 2 C-D) while endothelial cells were positive to CD31 (Figure 2 A-B). Tumor cells showed no positivity to S100 which excluded melanomas or Langerhans cells proliferation. The tumor was completely removed.Glomangiomas are usually located subungually on fingers and toes and extremely hurt when pressed. The SMA (smooth muscle actin) positivity was crucial for this diagnosis although the tumor appeared at very young age and wasn't located on its most common spot. Facial location is very rare but this entity should be considered in the differential diagnosis of facial nodules when sensitivity to temperature or pres-sure is present. In cases where no clinical signs can be found, MRI - although not specific - may be helpful in establishing the correct diagnosis.

glomus tumor; atypical location; skin lesion

nije evidentirano