BackgroundGerm cell tumors are tumors composed of tissues derived from more than one of the three germinal layers. They are more common in the testes and ovaries, but can present in many different regions in the midline, including the sacral region, retroperitoneum, mediastinum, and brain. Testicular germ cell tumors generally metastasize to the retroperitoneum, lungs, and brain; metastases to soft tissue are very rare.Case presentationHere we describe a case of a single soft-tissue mass in the thigh of a 27-year-old man, with histology showing areas of mature teratoma tissues derived from the ectodermal and mesodermal lineages, and areas of immature teratoma tissue composed of small undifferentiated cells, with primitive neuroectodermal differentiation foci forming neuroepithelial elements – thus classified as immature teratoma. The patient had no other clinical or radiological evidence of involvement, besides the lymph nodes.ConclusionThe case presented suggests a rare and unexpected primary immature teratoma of the thigh.
A 13-year-old boy presented congenital, raised, yellowish colored plaques with fibroelastic consistency, which were velvety in appearance, alopecic, with clearly defined limits and sulci or well-marked meandering invaginations, a cerebriform appearance on the upper part of the right ear, accompanied by alopecia in the temporomandibular region-parietal. The lesion was asymptomatic. Histopathologic examination revealed acanthosis, hyperkeratosis, papillomatosis and follicular plugging in the epidermis. The upper dermis showed underdeveloped hair follicles with sebaceous glands, consistent with sebaceous nevus. There were no skeletal, cardiological, ophthalmological or neuropsychomotor changes; nor were there any records of relevant pathologies in the family history. The patient complained of his appearance, experiencing aesthetic and social issues. Following a diagnosis of cerebriform nevus sebaceous, complete excision was performed with excellent aesthetic results.
Este estudo relata o caso de um paciente portador de um cisto dermóide com características raras, localização frontobasal e grande extensão. Esta forma cística apresenta índice de crescimento linear, diferentemente dos tumores neoplásicos que são exponenciais, o que caracteriza as manifestações clínicas tardias, desencadeando crises convulsivas ou síndrome de hipertensão intracraniana, por efeito compressivo cerebral. No caso estudado, a localização dos cisto comprometia estruturas vitais, o que resultou na permanêcia de restos capsulares após a cirurgia.
SINOPSEEste artigo demonstra a raridade de um tumor epitelióide "proximal" quanto à sua localização em região occipitocervical, mimetizando tumores tipo schwannoma ou neurofibromatose, quando normalmente, os tumores epitelióides surgem em regiões perianais 6 . Mesmo submetido à cirurgia radical, o tumor epitelióide "proximal" é altamente agressivo, com piora prognóstica quanto mais axial, ocorrendo preferencialmente em adultos, como neste caso, apresentando metástases múltiplas em região pulmonar e lombar, sendo submetido à cirurgia radical e radioterapia.Palavras-chave: Tumor de base de crânio, Sarcoma epitelióide proximal, Schwannoma, neurofibromatose. ABSTRACTWe report the rarity of a proximal epithelioid tumor located in the occipitocervical region, mimicking schwannoma type tumor or neurofibromatosis, when normally epithelioid tumors arise in perianal areas. The proximal epithelioid tumor is highly aggressive, with a worse prognosis when more axially located; it occurs mainly in adults, as in this case, presenting with multiple metastasis in lung and lumbar regions, even when submitted to radical surgery and radiotherapy.
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