Sarcoma sinovial renal primario: presentación de un caso con estudio molecular: Influence Statistics

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Sarcoma sinovial renal primario: presentación de un caso con estudio molecular

Abstract

. ResumenEl sarcoma sinovial (SS) renal fue descrito por primera vez por Argani et al. en el año 2000. Presentamos un caso de SS renal monofásico de grado intermedio. Se trata de un varón de 42 años que presentaba un tumor renal sólido-quístico de 6cm bien delimitado, con un nódulo sólido de 1,5cm. Se realizó nefrectomía parcial. El tumor se caracterizaba por células fusiformes monomorfas y quistes revestidos por células epiteliales en tachuela. Se observó infiltración focal de la grasa perirrenal que rodeaba el nódulo sólido. Las células fusocelulares expresaban CD99, BCL2, EMA y SMA, y eran negativas para S-100, CD34, receptores de estrógenos y progesterona, desmina y WT1. Las células en tachuela expresaban CD10, PAX8, PAX2, EMA, CKAE1-AE3, CK7 y CK19, confirmando que eran epitelio renal atrapado. Se realizó FISH que demostró la translocación t(X;18), específica del SS. El paciente ha recibido 4 ciclos de quimioterapia adyuvante y continúa libre de recurrencia o metástasis 9 meses tras la intervención.AbstractSynovial sarcoma (SS) of the kidney was first reported by Argani et al. in 2000. We report a case of a renal monophasic SS of intermediate grade in 42-year-old man with a solid-cystic, well-circumscribed, 6cm renal mass containing a solid nodule of 1.5cm. Partial nephrectomy was performed. The tumour was characterized by monomorphic spindle cells and cysts lined by trapped «hobnail» epithelial cells. Sections showed focal infiltration of perinephric fat surrounding the solid nodule. Spindle cells expressed CD99, BCL2, EMA and SMA and were negative for S-100, CD34, oestrogen and progesterone receptors, desmin and WT1. The “hobnail epithelium” expressed CD10, PAX8, PAX2, EMA, CKAE1-AE3, CK7 and CK19, confirming its non-neoplastic and renal identity. FISH was performed and t(X;18) translocation, specific of SS, was demonstrated. The patient received 4 cycles of adjuvant chemotherapy and is alive without evidence of disease 9 months after surgery.