Недавно описанные варианты RCC

Renal cell carcinoma. Edited by Nizar M. Tannir, Oxford University Press, 2014


С момента публикации ВОЗ классификации RCC (2004 г) было описано несколько типов RCC (Таблица 2.1), которые обсуждаются ниже.

В прошлом тубуло-кистозная RСС ошибочно упоминалась как низко-злокачественный CDC. Поверхность среза этих опухолей имеет характерный вид «пузырчатой обертки», поскольку они состоят из кист вариабельного размера в плотной строме. Эти кисты выстилаются клетками с цитоплазмой от прозрачной до эозинофильной и высокой степенью градации ядра по Фурману (FNG; обычно 3).

Acquired cystic disease-associated RCC was first reported in 2006 in a series of patients with end-stage renal disease (eSRD). These tumors occur exclusively in the setting of eSRD and have a strong association with dialysis.

These tumors have characteristic microcystic architecture with eosinophilic tumor cells and high-grade nuclei. Calcium oxalate crystal deposits are a frequent pathologic finding. These tumors can occasionally be aggressive, and metastases have been reported.

Clear-cell papillary RCC (CCPRCC)was first described in the setting of eSRD but now has been reported to occur in the sporadic setting. Tumors are generally partially or extensively cystic, small, and organ confined. These tumors have delicate papillae lined with clear cells that have low-grade apically located nuclei. These tumors are indolent, and metastatic tumors have not been reported.

Primary thyroid-like follicular carcinoma is histologically indistinguishable from follicular carcinoma of the thyroid gland and is composed of tubules with inspissated colloid-like material. Thyroid-specific immunohistochemical stains are negative, which helps with the diagnosis.

Hereditary leiomyomatosis-associated RCC affects young patients with germline mutations of the fumarate hydratase gene. Patients may also develop cutaneous and uterine leiomyomas or leiomyosarcomas. Histologically, they resemble type2 PRCC with extensive tubulopapillary architecture, eosinophilic cells, and prominent orangeophilic “viral-like” nuclear inclusions. Patients usually present with locally advanced tumors, and prognosis is poor.

Succinate dehydrogenase (SDH)-associated RCC is a recently described RCC type that is associated with germline mutations of the Krebs cycle enzyme, SDH, involving the SDH-B/C/D genes. The histology may be varied. SDH-B–associated RCCs frequently have an oncocytic appearance, while SDH-C–associated RCCs frequently show a clear-cell appearance. Patients often follow an aggressive clinical course.


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