Le carcinome tubulo-mucineux et fusiforme: une tumeur rénale rare

Abstract

Le carcinome tubulo-mucineux et fusiforme désigne une tumeur rare décrite dans la classification OMS 2004 comme une nouvelle entité. Elle est reconnue de comportement relativement indolent. Nous rapportons l'observation d'une femme âgée de 60 ans qui présentait des douleurs lombaires, et chez qui la tomodensitométrie a révélé la présence d'une masse rénale gauche mesurant 55 x 40 mm. La patiente a été traitée par néphrectomie partielle gauche dont l'examen macroscopique a montré la présence d'une tumeur bien limitée d'aspect charnu avec des remaniements hémorragiques et nécrotiques. L'étude histologique a confirmé le carcinome tubulo-mucineux et à cellules fusiformes de bas grade. L'immunohistochimie a révélé une positivité à la cytokératine (CK 7 et CK 19) et à l'antigène des membranes épithéliales (EMA), et une négativité au CD10. L'évolution était favorable avec un recul de 6 mois.Mucinous tubular and spindle cell carcinoma is a rare tumor defined in the 2004 WHO classification as a new entity. It is characterized by a relatively indolent behavior We report the case of a 60-year old woman presenting with lumbar pain. CT scan showed left renal mass measuring 55 x 40 mm. The patient underwent left partial nephrectomy. Macroscopic examination showed a well limited fleshy tumor with hemorrhagic or necrotic elements. Histological examination confirmed low grade mucinous tubular and spindle cell carcinoma. Immunohistochemistry revealed cytokeratin positive cells (CK 7 and CK 19), epithelial membrane antigen positive cells (EMA), and CD10-negativity cells. Patient's evolution was favorable, with a follow-up period of 6 months.

Mucinous tubular and spindle cell carcinoma: A rare kidney tumour

“…However, it can be revealed with some non-specific signs as hematuria, flank pain, nephrolithiasis and a palpable abdominal mass [1][2]. Unlike our patient, this tumor is found usually in the renal cortex and rarely in the medulla [1,[2][3][4][5].…”

“…Renal mucinous tubular and spindle cell carcinoma (MTSCC) is an epithelial tumour that accounts for less than 1% of all neoplasms of the kidney [1,2,[5][6]. It affects primarily adult patients aged between 13 and 81 years old (mean age: 58 years old) and shows a female predominance like our patient [1,2,[5][6]. MTSCC is usually incidentally discovered on abdominal imaging as our case [1][2].…”

“…MTSCC express distal nephron markers (EMA, CK19, CK7, E-Cadherin) and proximal tubule markers (RCC Ma, AMACR, CD15) [5][6].…”

“…The main differential diagnosis of MTSCC is papillary renal cell carcinoma (RCC) in its compact variant [1,2,3,[5][6]. Therefore, MTSCC may be distinguishable from papillary RCC by evaluating the presence of a trisomy for chromosomes 7 and 17 [1,2,[3][4][5]. Sarcomatoid carcinoma is a diagnosis to be made when the spindle cell component dominates [1,[2][3][4][5].…”

“…Therefore, MTSCC may be distinguishable from papillary RCC by evaluating the presence of a trisomy for chromosomes 7 and 17 [1,2,[3][4][5]. Sarcomatoid carcinoma is a diagnosis to be made when the spindle cell component dominates [1,[2][3][4][5]. It behaves aggressively with neoplastic spindle-shaped cells with large, hyperchromatic nuclei, mitosis and necrosis [1,[3][4][5].…”