Tubulocystic carcinoma of the kidney: a new entity among renal tumors

Azoulay, Sandy; Vieillefond, Annick; Paraf, François; Pasquier, Dominique; Cussenot, Olivier; Callard, P; Sibony, Mathilde

doi:10.1007/s00428-007-0483-7

Cited by 88 publications

(69 citation statements)

References 13 publications

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“…Tubulocystic carcinomas of the kidney occur most commonly in the fifth and sixth decades of life and there is a strong male predominance [3,6,[10][11][12]. Most TCKs are discovered incidentally and are typically small in size (almost 40% are smaller than 2 cm) [3,13,14].…”

Section: Clinical Characteristicsmentioning

confidence: 99%

Review of tubulocystic carcinoma of the kidney with focus on clinical and pathobiological aspects

Matsumoto¹,

Ohe²,

Mikami³

et al. 2013

pjp

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Tubulocystic carcinoma of the kidney (TCK) is a recently established entity in renal neoplastic pathology. This review aims to give an overview of the clinical and pathobiological aspects of TCK. Grossly, the TCKs are well-demarcated multicystic lesions giving a "wrapped bubble" or "spongy" appearance. Microscopically, the tumors are composed of multiple, variably sized cysts separated by thin fibrous septa lacking ovarian stroma or desmoplastic reaction. The cysts are lined by tumor cells with eosinophilic cytoplasm and nuclear atypia of variable, but not infrequently of high grade corresponding to Fuhrman grade 3. A frequent association with papillary tumors has been reported. Recent molecular genetic studies of TCK have revealed distinct features separating this subset of renal cell carcinomas (RCCs) from other types of renal tumors including collecting duct carcinoma of Bellini and renal medullary carcinoma as well as pointing towards a close kinship with papillary RCC. Tubulocystic carcinoma of the kidney generally pursues an indolent clinical course. However, several cases with aggressive clinical behavior have been reported. We strongly feel that there is enough clinicopathological evidence to corroborate TCK as a separate entity and that it should be incorporated into the next WHO classification of renal tumors as a separate neoplastic category.

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Section: Clinical Characteristicsmentioning

confidence: 99%

Review of tubulocystic carcinoma of the kidney with focus on clinical and pathobiological aspects

Matsumoto¹,

Ohe²,

Mikami³

et al. 2013

pjp

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show abstract

“…57 Recently three major series of tubulocystic carcinoma have reported 55 cases. [58][59][60] Tubulocystic carcinoma is a tumor of adults with a wide age range, 30-94 years. Most patients are present in the fifth and sixth decade and there is a strong male predominance (male/female ratio of 7:1).…”

Section: Tubulocystic Carcinomamentioning

confidence: 99%

“…Most patients are present in the fifth and sixth decade and there is a strong male predominance (male/female ratio of 7:1). [58][59][60] Patients are often asymptomatic, although they may also present with abdominal pain, distention and hematuria. Reported tumors are more often left sided (27/44).…”

Section: Tubulocystic Carcinomamentioning

confidence: 99%

“…Most tubulocystic carcinomas are pT1 tumors, with occasional pT2 and pT3 lesions being reported. 60 Immunohistochemical studies [58][59][60] demonstrate a wide range of marker positivity with cytokeratins (CK8, CK18, CK19) and parvalbumin being consistently positive. CD10 and P504S (racemase) (Figure 17) are positive in greater than 90% of tumors.…”

Section: Tubulocystic Carcinomamentioning

confidence: 99%

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Uncommon and recently described renal carcinomas

2009

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Major consensus conferences held over a decade ago laid the foundations for the current (2004) WHO classification of renal carcinoma. Clear cell, papillary and chromophobe carcinomas account for 85-90% carcinomas seen in routine practice. The remaining 10-15% of carcinomas consist of rare sporadic and hereditary tumors, some of which had been long recognized, but many of which only emerged as distinct entities in the decade leading up to the WHO publication. Collecting-duct carcinoma is a rare, often lethal form of carcinoma. Medullary carcinoma associated with sickle cell trait, has emerged as a distinctive tumor showing some overlapping features with upper tract urothelial carcinoma. Mucinous tubular and spindle-cell carcinoma and tubulocystic carcinoma were earlier considered as patterns of low-grade collecting-duct carcinoma, but are now recognized as separate tumor entities. Carcinomas associated with somatic translocations of TFE3 and TFEB comprise a significant proportion of pediatric renal carcinomas. Oncocytoid renal carcinomas in neuroblastoma survivors was recognized as a unique tumor category in the WHO classification. Renal carcinoma associated with end-stage renal disease is now recognized as having distinct morphological patterns and behavior. In addition there is a group of rare recently described carcinomas, including clear cell papillary carcinoma, oncocytic papillary renal cell carcinoma, follicular renal carcinoma and leiomyomatous renal cell carcinoma. It behooves the surgical pathologist to not only be capable of diagnosing the common forms of renal cancer, but also to be aware of the rare types of renal carcinoma, many of which have emerged in recent years.

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“…Nucleoli are often very prominent. TCC are immunohistochemically reactive for CD10, AMACR (P504-S), CK8, CK18, and CK19 (3,6). Th ese entities can also be positive for PAX2, kidney-specifi c cadherin, carbonic anhydrase IX, and parvalbumin (3).…”

Section: Discussionmentioning

confidence: 99%