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Renal cancer
Published in Anju Sahdev, Sarah J. Vinnicombe, Husband & Reznek's Imaging in Oncology, 2020
Conrad von Stempel, Lee Alexander Grant, Miles Walkden, Navin Ramachandran
Collecting system carcinoma (carcinoma of the collecting ducts of Bellini) is a highly aggressive, infiltrative subtype of RCC. Over a third of patients present with metastatic disease at diagnosis. These lesions can have variable features on imaging, and large tumours can share appearances of the more common subtypes of RCC. Collecting-duct carcinoma can be considered if a tumour is centred on the medullary pyramid, and has marked low signal on T2W sequences attributed to intratumoural calcification (121).
Hereditary Papillary Renal Cell Cancer
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Tumors of the kidneys represent 2%–3% of all adult malignancies, with RCC alone accounting for 85% of kidney neoplasms. There is a male predominance among RCC patients (male:female ratio of 2:1). Among various RCC histologic types, ccRCC (75%) predominates. This is followed by pRCC (10%–15%), chRCC (4%–5%), collecting duct carcinoma (<1% of RCC cases), tRCC (<1% of RCC cases), and other histologic types. Interestingly, over 90% of RCC cases do not show family connection and appear to emerge from sporadic/de novo mutations, whereas 5%–8% of RCC cases are attributed to germline mutations that occur in families, leading to bilateral or multifocal tumors in patients of relatively young age (up to 46 years) [1]. HPRCC has an estimated incidence of <1:1500.00, and its rarity is highlighted by the fact that only about 35 affected families have been reported worldwide.
The Molecular Genetics and Pathology of Renal Cell Carcinoma
Published in Anthony R. Mundy, John M. Fitzpatrick, David E. Neal, Nicholas J. R. George, The Scientific Basis of Urology, 2010
Maxine G. B. Tran, Tim O’Brien, Patrick H. Maxwell
Collecting duct carcinoma (CDC) is a rare tumor and accounts for less than 1% of renal malignancies in surgical series. There is a slight male predominance and average age at presentation is 55 years. These tumors arise from the collecting ducts, especially the papillary ducts (Bellini ducts) hence they are also called Bellini duct carcinomas. They are centrally located in the kidney, averaging 5 cm though range in size from 2.5 to 12 cm, and the cut surface appears solid with a gray-white color. Patients may present with hematuria, flank pain and mass. Urine cytology may be positive (39) and imaging studies may suggest urothelial malignancy.
Pathology and systemic therapy of non-clear cell renal cell carcinoma: an overview
Published in Expert Review of Anticancer Therapy, 2021
Lothar Bergmann, Sarah Weber, Arndt Hartmann, Marit Ahrens
Important tumor syndromes include von-Hippel-Lindau (VHL) syndrome with associated ccRCC, Bird–Hogg–Dubé syndrome with associated hybrid oncocytic tumors, tuberose sclerosis (TSC) with associated angiomyolipomas, PEComa, and eosinophilic solid and cystic (ESC) RCC. RCC within-family tumor syndromes also include SDH-deficient RCC and FH-deficient RCC. New tumor entities with a specific molecular alteration are the transcription elongation Factor 1 (TCEB), mutant RCC, the MiTF-TFE family of RCCs (TFE3 translocation-associated RCC, TFEB-translocated or amplified RCC), SMARCB1/INI-deficient RCC and ALK translocation-associated RCC. These tumors are often found in younger patients and frequently show a particularly aggressive disease course, dedifferentiating with sarcomatoid and rhabdoid features [3,25]. The diagnosis of specific tumor entities in poorly differentiated or dedifferentiated RCC is often difficult. In particular, the distinction of collecting duct carcinoma from poorly differentiated urothelial carcinoma can be exceedingly challenging. Even after extensive histomorphological, immunohistochemical and molecular studies, a tumor may not be clearly diagnosable as one specific entity. For theses tumors, the WHO classification recommends the diagnosis RCC, NOS (not otherwise specified), in which highly aggressive tumors have to be distinguished from indolent low-grade neoplasia.
Impact of histological subtype on outcomes of renal cell carcinoma patients
Published in Journal of Drug Assessment, 2018
A total of 89,968 of patients with RCC diagnosed 2001–2013 were identified among this cohort, 68,318 (75.9%) of patients have clear cell RCC, 14,791 (16.4%) of patients have papillary RCC, 6458 (7.1%) of patients have chromophobe RCC and 401 (0.6%) of patients have collecting duct RCC. Table 1 summarizes baseline characteristics of different RCC histological subtypes. The majority of RCC patients among all histological subtypes are males, white race, married and diagnosed in the age group of 40–69. Most patients present at an earlier stage (stage I/II) except those with collecting duct carcinoma which present more at a more advanced stage. The predominant local treatment modality is total (radical) nephrectomy and radiotherapy has not been used in the majority of cases.
Molecular characterization and diagnostic criteria of renal cell carcinoma with emphasis on liquid biopsies
Published in Expert Review of Molecular Diagnostics, 2020
Alessia Cimadamore, Francesco Massari, Matteo Santoni, Veronica Mollica, Vincenzo Di Nunno, Liang Cheng, Antonio Lopez-Beltran, Marina Scarpelli, Rodolfo Montironi, Holger Moch
Among rare RCC, collecting duct carcinoma (CDC) is characterized by common mutation, also found in other histotypes, such as in NF2, SETD2, SMARCB1, FH, and CDKN2A [39]. Renal Medullary carcinoma is a rare and distinctive entity characterized by loss of heterozygosity (LOH) and balanced translocations or biallelic loss of SMARCB1/INI1 tumor suppressor protein [40]. Hereditary leiomyomatous and RCC syndrome (HLRCC) patients is linked to a germline mutation in the fumarate hydratase (FH) gene [41]. These tumors are characterized by aggressive clinical behavior, and adverse morphologic features [14] and represent separate tumor entities in 2016 WHO classification (Table 1).