RESUMO
The first report of granulosa cell tumor occurred in 1926, by Abrikossoff, after whom it was named “Abrikossoff’s tumor”. It is a benign, uncommon neoplasm, most frequently observed in the head, neck and tongue, being found in the vulva only in 6%-7% of the cases. Its histogenesis is uncertain, probably linked to Schwann cells. The treatment is surgical, with good prognosis. Relapses may occur, and there are descriptions of malignant transformation in the literature. The objective of this paper is to describe a case of granular cell tumor located in the vulva.
O primeiro relato do tumor de células granulosas ocorreu em 1926, por Abrikossoff, nome que deu origem à denominação “tumor de Abrikossoff”. Trata-se de neoplasia benigna, incomum, observada mais frequentemente na cabeça, no pescoço e na língua, sendo encontrado na vulva em apenas 6%-7% dos casos. Sua histogênese é incerta, provavelmente ligada às células de Schwann. O tratamento é cirúrgico, com bom prognóstico. Podem ocorrer recidivas, e existem descrições de malignização na literatura. O objetivo deste trabalho é descrever um caso de tumor de células granulares localizado na vulva.
RESUMO
Epidermodysplasia verruciformis (EV) is triggered by a variety of mechanisms that at least partly include genetic background. We present a Brazilian man with a 30-year history of flat, wart-like lesions with clinical, histopathological, and evolutive aspects consistent with papillomavirus (HPV)-associated EV. Histological analysis of the wart lesions showed epidermis with hyperkeratosis, regular acanthosis, hypergranulosis, and cells with abundant basophilic cytoplasm. Moreover, a perivascular lymphocytic infiltrate was found in the superficial dermis, consistent with a viral wart. Type-2-HPV DNA was detected in various fragments of skin-wart lesions using the polymerase chain reaction (PCR). Two years after the EV diagnosis, the patient presented with an anesthetic well-demarcated, erythematous and mildly scaly plaque on his right forearm. A histopathological analysis of this lesion demonstrated the presence of a compact tuberculoid granuloma. Ziehl-Neelsen staining demonstrated the presence of rare acid-fast bacilli and confirmed the tuberculoid leprosy diagnosis. The patient's Mitsuda Intradermal Reaction was positive. To elucidate the possible mechanism involved in this case of EV, we genotyped the HLA genes of this patient. DQB genotyping showed the polymorphic HLA alleles DQB1*0301 and 0501. The patient was treated with a paucibacillary multi-drug therapy scheme, and the disease was cured in six months. This report describes an EV patient with an M. leprae infection, confirming that tuberculoid leprosy patients possess a relatively specific and efficient cell-mediated immunity against the bacillus and, therefore, localized forms of the disease. Moreover, we show the possible involvement of the polymorphic HLA alleles DQB1*0301 and 0501 in EV induction mechanisms.