Showing papers on "Mucinous tubular and spindle cell carcinoma published in 2016"
TL;DR: Next-generation sequencing of a multi-institutional MTSCC cohort revealed recurrent chromosomal losses and somatic mutations in the Hippo signaling pathway genes leading to potential YAP1 activation, suggesting a common mechanistic basis for this disease.
Abstract: Mucinous tubular and spindle cell carcinoma (MTSCC) is a relatively rare subtype of renal cell carcinoma (RCC) with distinctive morphologic and cytogenetic features. Here, we carry out whole-exome and transcriptome sequencing of a multi-institutional cohort of MTSCC (n = 22). We demonstrate the presence of either biallelic loss of Hippo pathway tumor suppressor genes (TSG) and/or evidence of alteration of Hippo pathway genes in 85% of samples. PTPN14 (31%) and NF2 (22%) were the most commonly implicated Hippo pathway genes, whereas other genes such as SAV1 and HIPK2 were also involved in a mutually exclusive fashion. Mutations in the context of recurrent chromosomal losses amounted to biallelic alterations in these TSGs. As a readout of Hippo pathway inactivation, a majority of cases (90%) exhibited increased nuclear YAP1 protein expression. Taken together, nearly all cases of MTSCC exhibit some evidence of Hippo pathway dysregulation. Significance: MTSCC is a rare and relatively recently described subtype of RCC. Next-generation sequencing of a multi-institutional MTSCC cohort revealed recurrent chromosomal losses and somatic mutations in the Hippo signaling pathway genes leading to potential YAP1 activation. In virtually all cases of MTSCC, there was evidence of Hippo pathway dysregulation, suggesting a common mechanistic basis for this disease. Cancer Discov; 6(11); 1258–66. ©2016 AACR. This article is highlighted in the In This Issue feature, p. 1197
58 citations
TL;DR: Solid PRCC is a rare tumor with an incidence of less than 1% of all renal tumors, and in majority of the cases, tumors were composed of tightly compressed tubular structures and short abortive papillae that render a solid morphologic appearance.
19 citations
01 Jan 2016
TL;DR: The tumour was composed of cuboidal cells arranged in tubules and was making abrupt transitions to the spindle cell morphology in a myxoid stroma and must be differentiated from papillary renal cell carcinoma, especially that with sarcomatoid change.
Abstract: Mucinous tubular and spindle cell carcinoma of the kidney (MTSCC K) is an unusual renal tumor. It is important to increase the recognition of MTSCC K and improve the level of clinical diagnosis. We report a case of mucinous tubular and spindle cell carcinoma in a 50-year old woman. The tumour, located on the left kidney, was well circumscribed and a partial nephrectomy was applied. In microscopically, the tumour was composed of cuboidal cells arranged in tubules and was making abrupt transitions to the spindle cell morphology in a myxoid stroma. Due to it’s favourable prognosis, mucinous tubular and spindle cell carcinoma must be differentiated from papillary renal cell carcinoma, especially that with sarcomatoid change. Correspondence to: Y. Dehayni, Department of Urology, Military hospital Moulay Ismail, Meknès, Morocco, E-mail: dr.y.dehayni@gmail.com
1 citations
TL;DR: This is the first report of mucinous tubular and spindle cell carcinoma in a kidney allograft, and when considering multifocal RCC with discordant histology, it is likely that these represent independent tumourigenic events.
Abstract: Renal cell carcinoma (RCC) is the fifth most common malignancy in kidney transplant recipients, with increased risk arising due to immunosuppression. De novo RCC occurrence in kidney allografts is much less common when compared with the native kidneys. Multifocal RCC in allograft kidneys is rarely described. In this report, we discuss two cases of de novo multifocal renal neoplasms in allograft kidneys. Case 1 had three distinct neoplastic lesions of >5 mm, and case 2 had four. Using the World Health Organization 2016 classification of adult renal tumours, case 1 had one clear-cell (cc) RCC (grade 3) and two papillary adenomas; all confined to the kidney. Case 2 had a nodular lesion classified as ccRCC (grade 4) with focal rhabdoid differentiation and some infiltration of renal sinus fat; a cc tubulopapillary RCC; a multilocular cystic renal neoplasm of low malignant potential; and a mucinous tubular and spindle cell carcinoma; the last three all confined to the kidney. This is the first report of mucinous tubular and spindle cell carcinoma in a kidney allograft. When considering multifocal RCC with discordant histology, it is likely that these represent independent tumourigenic events.
1 citations
TL;DR: A case of ulcerative colitis (UC) in a patient during the first severe relapse with colonic dilatation and coexisting of giant renal tumor, a very rare malignant kidney tumor of low malignant potential and relative good prognosis is presented.
Abstract: We present a case of ulcerative colitis (UC) in a patient during the first severe relapse with colonic dilatation and coexisting of giant renal tumor. Kidney tumor was constricting on colon and imitating toxic megacolon (TM). The patient with severe acute colitis (SAC) was admitted to the Department of Gastroenterology and Hepatology with inflammatory bowel disease (IBD) diagnosed in the previous month, based on clinical symptoms, endoscopy, and histopathology results. Computed tomography (CT) revealed a giant left kidney tumor without evidence of infiltration, a dilatation of the transverse colon up to 6 cm, and narrow light of the descending colon. There were no signs of intestinal obstruction or perforation. The control X-rays revealed maintaining megacolon, with dilatation of splenic flexure projection up to 6.5 to 7.5 cm. The patient was treated conservatively with no apparent improvement and finally operated on. Intraoperatively, a large tumor of the kidney (12 cm) constricting intestine was revealed. Left-sided nephrectomy and partial resection of the colon with the emergence of a colostomy was performed. The histopathology exam revealed renal mucinous tubular and spindle cell carcinoma (RMTSCC), a very rare malignant kidney tumor of low malignant potential and relative good prognosis. It was identified in the past 20 years. To date, approximately 100 such cases of cancer have been described. How to cite this article Kukulska M, Smola I, Halon A, Paradowski L, Poniewierka E, Kempinski R, Annabhani A. A Case of Severe Ulcerative Colitis with Colonic Dilatation caused by Renal Mucinous Tubular and Spindle Cell Carcinoma. Euroasian J Hepato-Gastroenterol 2016;6(2):190-193.
01 Jan 2016
TL;DR: Several new subtypes of renal carcinoma have been described in recent years and represent a growing awareness of the subtleties of renal cancer morphology and their relationship to molecular alterations.
Abstract: Several new subtypes of renal carcinoma have been described in recent years and represent a growing awareness of the subtleties of renal cancer morphology and their relationship to molecular alterations. One example of this encompasses Xp11 translocation carcinomas, described in Chap. 7, where distinct molecular changes impact both morphological variation and prognosis. Several other nonconventional subtypes of renal cell carcinoma have been incorporated into standard classification schema for renal neoplasia, although some entities still require further study and are considered “emerging entities” (Srigley et al., Am J Surg Pathol, 2013; 37(10):1469–89). Although our knowledge surrounding these neoplasms continues to expand, it appears that at least some of these lesions are associated with distinct molecular alterations and clinical behavior. Given that many of these entities have only been recently described, there is limited knowledge about therapies that may be uniquely applied to these entities.
TL;DR: A 57-year-old man presenting with loin pain and dragging sensation was diagnosed with MTSCC, a subtype of renal cell carcinoma, which is characterized by a polymorphous histology, wherein the spindled epithelial cell is an inherent carcinomatous component.
Abstract: Mucinous tubular and spindle cell carcinoma (MTSCC) is a recent entity introduced in the World Health Organization 2004 Classification. It is a tumour of low malignant potential. MTSCC is a subtype of renal cell carcinoma (RCC), which is characterized by a polymorphous histology, wherein the spindled epithelial cell is an inherent carcinomatous component. We report the case of a 57-year-old man presenting with loin pain and dragging sensation. Imaging revealed a large mass arising from the left kidney. Radical nephrectomy was performed, and histopathology revealed spindle cell elements of MTSCC with low-grade cytology, which occasionally blended with tubular structures in variable mucinous stroma admixed with spindle sarcomatoid cells with marked nuclear pleomorphism, associated with significant necrosis and mitoses of up to 5/10 high-power field. A final diagnosis of MTSCC along with high-grade areas consistent with sarcomatoid dedifferentiation was made. Sarcomatoid dedifferentiation has been well documented in various subtypes of RCC, and its presence signifies a worse prognosis in RCC.