Lundon Dara J, Kelly Brian D, Casby Caoimhe, Coyle David, Flaherty Rita A, Hynes Sean, McHale Teresa, Durkan Garrett C
Department of Urology, Galway University Hospital Galway, Ireland.
Anatomic Pathology Department, Galway University Hospital Galway, Ireland.
Am J Clin Exp Urol. 2023 Feb 25;11(1):75-78. eCollection 2023.
Mucinous tubular and spindle cell carcinoma (MTSCC) is a rare type of kidney tumor that has only recently been described, with less than eighty cases in the literature. This was only recognized as a specific entity in the World Health Organization 2004 classification of Renal Cell Carcinoma (RCC). MTSCCs are polymorphic renal neoplasms characterized by small, elongated tubules lined by cuboidal cells with cords of spindled cells separated by pale mucinous stroma. We report the case of a 57 year old lady who had an incidental finding of a mass in her right kidney. The radiological features were consistent with a RCC and following a multidisciplinary team discussion she underwent a laparoscopic radical nephrectomy. Macroscopic examination revealed a well circumscribed 6.5 × 6 × 6.5 cm right lower pole mass. Histologically it was composed of elongated tubules, small tubules and papillary structures with a necrotic centre. The cells demonstrated cuboidal and spindle cell morphology. Histological grade was Fuhrman grade 2. The majority of MTSCCs are indolent, and there are only two reports of distant metastases which responded favorably to adjuvant sunitinib. To date there is no international consensus on long term surveillance of these patients. Due of the favorable prognosis with this type of tumor, MTSCC must be differentiated from papillary renal cell carcinoma to avoid administration of excessive adjuvant treatment to patients.
黏液性小管状和梭形细胞癌(MTSCC)是一种罕见的肾肿瘤,直到最近才被描述,文献中报道的病例不到80例。它在2004年世界卫生组织肾细胞癌(RCC)分类中才被确认为一种特定的实体。MTSCC是多形性肾肿瘤,其特征是由立方体细胞排列的小而细长的小管,伴有梭形细胞索,其间为淡染的黏液性间质分隔。我们报告一例57岁女性,偶然发现右肾有一肿块。影像学特征符合肾细胞癌,经过多学科团队讨论后,她接受了腹腔镜根治性肾切除术。大体检查发现一个边界清楚的右下极肿块,大小为6.5×6×6.5cm。组织学上,它由细长的小管、小的小管和乳头结构组成,中心有坏死。细胞表现为立方体细胞和梭形细胞形态。组织学分级为Fuhrman 2级。大多数MTSCC生长缓慢,仅有两例远处转移的报告,对辅助性舒尼替尼反应良好。迄今为止,对于这些患者的长期监测尚无国际共识。由于这类肿瘤预后良好,必须将MTSCC与乳头状肾细胞癌区分开来,以避免对患者进行过度的辅助治疗。