RESUMO
A cutaneous metaplastic synovial cyst is a rare entity that is probably caused by trauma or surgery. We report the second case of cutaneous metaplastic synovial cyst in a child with Ehlers-Danlos syndrome. His father is also affected with Ehlers-Danlos syndrome, and his diagnosis is substantiated by the demonstration of reduced synthesis of collagen type V.
Assuntos
Síndrome de Ehlers-Danlos/complicações , Cisto Sinovial/complicações , Cisto Sinovial/patologia , Criança , Pré-Escolar , Colágeno Tipo V/biossíntese , Eletroforese em Gel de Poliacrilamida , Humanos , Lactente , Recém-Nascido , Masculino , LinhagemRESUMO
Clear cell sarcoma of the tendons and aponeuroses is an aggressive, rare soft tissue tumour that occurs predominantly in the extremities of young adults. Although it appears to be histogenetically related to melanoma, its clinical behaviour resembles soft tissue sarcoma. Prognosis is reported to be poor due to the great propensity of regional and distant metastases. The risk of metastases to regional lymph nodes is very high. We describe a case of clear cell sarcoma of the hand and evaluate the feasibility of the sentinel lymph node biopsy (SLNB) technique in this kind of tumour. Up to now, there are no reports that describe the use of SLNB in clear cell sarcoma. The SLNB technique was carried out with success and the sentinel lymphnode was easily identified. Histological examination of the lymphnode allowed identification of metastatic cells and thus provide for radical lymphadenectomy. We maintain that SLNB can be successfully used for this type of tumour although this result should be evaluated and confirmed by larger case studies.
