RESUMO
Medulloepithelioma is a rare and highly malignant tumor of infancy and early childhood, and classified as a primitive neuroectodermal tumor. Considering that most cases occur in the central nervous system, development in atypical sites associated with secondary comorbidities, such as peritoneal carcinomatosis, becomes an extremely rare association due to its high morbimortality. This study reports a rare case of peripheral medulloepithelioma with peritoneal carcinomatosis in an 11-year-old boy, with a 4-year history of intestinal constipation alternated with fecal incontinence, taken to the emergency room due to increasing abdominal pain and urinary retention. This report aims to contribute to a better understanding of this rare pathology, as well as assist in the establishment of early diagnosis and treatment.
RESUMO
A cutis verticis gyrata é uma condição do couro cabeludo caracterizada por sulcos e dobras profundas, causando o espessamento da pele que lhe dá uma aparência semelhante aos giros do córtex cerebral. Pode ser congênita ou adquirida, e predomina no sexo masculino. É classificada de acordo com sua etiologia primária (essencial e não essencial) e secundária. Descrevemos aqui um caso de cutis verticis gyrata secundária à paquidermoperiostose em paciente do sexo masculino jovem, apresentando suas manifestações clínicas, laboratoriais e radiológicas. Estudo descritivo constando de relato de caso e revisão de literatura nas bases de dados da Biblioteca Virtual de Saúde. Foram obtidos artigos originais científicos nas bases LILACS, SciELO, Scopus e PubMed, buscando-se pelos descritores: "couro cabeludo", "doenças da pele e do tecido conjuntivo", "anormalidades da pele" e "dermatopatias". O limite temporal foi 2010 e as línguas escolhidas foram português, inglês, italiano, espanhol e francês. Paciente do sexo masculino, 25 anos, apresentando hipertrofias lineares em couro cabeludo, acompanhado de dor nas mãos, tornozelos e articulação coxofemoral. Presença de baqueteamento digital e hiperidrose palmoplantar. Trata-se de uma doença rara e progressiva, cuja avaliação clínica é essencial para definir a forma da cutis verticis gyrata e excluir outras síndromes associadas.
Cutis verticis gyrata (CVG) is a scalp condition characterized ridges and deep folds causing thickening of the skin, giving an appearance similar to the cerebral cortex gyri. It can be congenital or acquired, and predominates in males. It is classified according to its primary and secondary etiology (essential and non-essential). A case of Cutis verticis gyrata secondary to pachydermoperiostosis (PQP) is described in a young male patient, with its clinical, laboratory and radiological findings. This is a descriptive study consisting of a case report and review of literature on the Virtual Health Library databases (BVS). Scientific original articles were obtained in LILAC, SciELO, Scopus and PubMed databases, searching the keywords: "scalp", "skin and connective tissue diseases", "skin abnormalities" and "skin diseases" .The time limit was year 2010, and the languages chosen were Portuguese, English, Italian, Spanish and French. Male patient, 25 years old, with linear hypertrophy on the scalp, accompanied by pain in the hands, ankles and hip joint. Presence of digital clubbing, and palmoplantar hyperhidrosis. It is a rare, progressive disease whose clinical assessment is essential to define the type of cutis verticis gyrata, and to rule out other associated syndromes.