Dados do Trabalho

Título

MAMMARY ANALOGUE SECRETORY CARCINOMA (MASC) OF PAROTID IN A INDIGENOUS PATIENT: CASE REPORT AND LITERATURE REVIEW.

Apresentação do caso

A 23-year-old woman, indigenous to the Metuktire ethnic group, Kororote village, Mato Grosso (MT), started mandibular bulging to the right 4 years ago, with progressive worsening of otalgia and headache. Biopsy (BX) in 2015 was negative for neoplasia, being treated with alternative medicine by the shaman of the tribe, until June 2017 when she was referred to the Ambulatory of the Indian, submitted to new BX, and anatomopathological (AP) compatible with MASC. In August / 2017 a right parotidectomy with cervical emptying was performed, AP showing a tumor of 4.5 cm, deep margin compromised, without lymph nodes affected, and immunohistochemistry (IHQ) compatible with MASC (pT3pN0cM0). Indicated adjuvant radiotherapy (RT), but returns to the village due gestation. Returns only in March / 2018, in oncology when performed RT with 50cGy, currently under follow-up

Discussão

The MASC of salivary glands is a rare tumor with similar morphological, IHQ and molecular similarities to breast-secreting carcinoma. The most frequent site is the parotid gland (70%). It occur more in adult men (mean 45 years), presenting as painless mass, variable evolution and symptoms as pain and facial paralysis. Diagnosis is by imaging (USG or MRI), and confirmation by AP. Histologically (HE) is extraductal, non-encapsulated, somewhat infiltrative, solid, microcystic, tubular, papillocystic and riddling patterns, IHC with strong and diffuse expression of mamaglubulin and S100 protein, and fusion of ETV6-NTRK3 genes by FISH confirms the diagnosis . Local recurrences have been reported suggesting adjuvant RT and metastatization rarely occurs. Specific translocation may be focus for target therapy. Neoplasms in natives have low literature and underreporting, with evidence of worse prognosis due to late diagnosis, and in parts of Brazil, more cases of head and neck cancer have been described.

Comentários finais

Here we report the 1st case described in the literature of this rare tumor in the indigenous population and we highlight the difficulty of access to the diagnosis and specific treatment in this population, besides the difficult follow-up of the same ones

Palavras-chave

Mammary analogue secretory carcinoma