Классификация почечно-клеточной карциномы

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


За последнее столетие предложено несколько классифицирующих систем почечно-клеточной карциномы. Всемирная организация здравоохранения разработала самую актуальную классифицирующую систему (Таблица 2.1). Эта система основана на фундаменте двух более ранних работ (Heidelberg classification и American Joint Committee on Cancer [AJCC] и Union Internationale Contre le Cancer classification), обе из которых опубликованы в 1997. После издания ВОЗ классификации в 2004 году сообщено несколько уникальных типов RСC (Таблица 2.1). Характерные черты этих опухолей перечислены в Таблице 2.2.

Светлоклеточная карцинома почки

Clear-cell RCC (ccRCC) is the most common RCC type, representing 65%–75% of all RCCs reported in most series. Most patients present with a solitary tumor; however, multiple synchronous tumors occur rarely. The typical ccRCC is a solid tumor with a bright yellow or light orange cut surface.

Cyst formation, hemorrhage, and necrosis may be evident, depending on the tumor. Most ccRCCs are organ confined, while the rest are locally advanced with invasion into the perinephric adipose tissue, renal sinus adipose tissue, renal vein, or inferior vena cava. In some studies, the renal sinus has been identified as the main portal for the tumor to spread beyond the confines of the kidney. The presence of delicate, interconnecting, sinusoidal-type thin blood vessels is an important histologic feature seen on light microscopy.

These blood vessels separate tumor cells into variable-size nests and tubules.

The tumor cells usually have cells with clear cytoplasm, hence, the name clear-cell renal cell carcinoma, which is a result of accumulation of lipids and glycogen in the cell’s cytoplasm. However, some tumor cells may have eosinophilic cytoplasm; the number of cells with eosinophilic cytoplasm varies within individual tumors. Rare tumors maybe almost entirely composed of cells with eosinophilic cytoplasm. In some high-grade ccRCCs, tumor cells may have “rhabdoid” features, that is, single cells with large eccentrically placed nuclei and prominent eosinophilic cytoplasm. Considering the aforementioned, the morphologic diagnosis of ccRCC is not based solely the cell cytoplasm. Rather, the diagnosis is based on a combination of the pattern of cell growth, pattern of the vasculature, and the cytoplasmic characteristics of the tumor cells.

Таблица 2.1. Типы почечно-клеточной карциномы

Типы почечно-клеточной карциномы в классификации Всемирной организации здравоохранения 2004 года

  • Светлоклеточная карцинома почки
  • Мультилокулярная светлоклеточная карцинома почки
  • Папиллярная почечно-клеточная карцинома
  • Хромофобная почечно-клеточная карцинома
  • Карцинома собирательных протоков Беллини
  • Медуллярная карцинома почки
  • Карцинома транслокации Xp11
  • Муцинозная тубулярная и веретеноклеточная карцинома
  • Карцинома ассоциировала с нейробластомой
  • Неклассифицированная почечно-клеточная карцинома

Недавно описанные типы почечно-клеточной карциномы

  • Тубуло-кистозная почечно-клеточная карцинома
  • Приобретенная кистозная болезнь-ассоциированная почечно-клеточная карцинома
  • Светлоклеточная папиллярная почечно-клеточная карцинома
  • Первичная тиреоид-подобная фолликулярная карцинома
  • Наследственный лейомиоматоз-ассоциированная почечно-клеточная карцинома
  • Сукцинатдегидрогеназа-ассоциированная почечно-клеточная карцинома

Папиллярная почечно-клеточная карцинома

Papillary renal cell carcinoma (PRCC) is the second most common RCC type, representing 10%–15% of all RCCs. PRCC is the tumor that is most often multifocal and bilateral and likely to metastasize to regional lymph nodes. Rarely, metastases to regional lymph nodes form a mass larger than the primary tumor in the kidney. These tumors are most often soft and friable, usually with abundant hemorrhage and necrosis. PRCCs that appear cystic on radiographic studies tend to have solid-appearing tumor at the periphery, while most of the center has tumor cells suspended in hemorrhagic fluid. Papilla formation is the typical morphologic feature; however, tumor cells may also form tubulopapillary forms and tubules; rarely, the papillae are so close together that they appear to form solid nests. Based on morphologic features, PRCC is further divided into type 1 and type 2.3 Type 1 PRCCs have thin fibrovascular cores that are lined with a single layer of low cuboidal cells with scant pale cytoplasm and low nuclear-grade nuclei. Collections of foamy histiocytes and psammoma bodies (laminated calcifications) are more common in type 1 PRCCs. Type 2 PRCCs have tall columnar pseudostratified cells with abundant eosinophilic cytoplasm and high-grade nuclei. At present, there are limited data that show differences in clinical outcomes and genetic abnormalities between type 1 and type 2 PRCCs.

Таблица 2.2. Морфологические черты и иммуногистохимический профиль различных типов почечно-клеточной карциномы

RCC type Morphologic features Immunohistochemical profile
Clear RCC Nests, sheets, or tubular architecture; thin-walled sinusoidal blood vessels; optically clear-cell cytoplasm EMA, vimentin, RCC antigen, CD10, carbonic anhydrase IX positive; CK7, AMACR negative
Papillary RCC Papillary, tubular, or solid architecture; frequent hemorrhage and necrosis; foamy macrophages and psammomatous microcalcifications EMA, vimentin, RCC antigen, CK7, CD10, AMACR positive; type 2 tumors are frequently CK7 negative
Type 1: Cuboidal cells with scant cytoplasm and small or inconspicuous nucleoli
Type 2: Pseudostratified columnar cells with voluminous eosinophilic cytoplasm and prominent nucleoli
Chromophobe RCC Solid sheets, nests, or tubules; thick-walled blood vessels; clear to eosinophilic cytoplasm with prominent cell membranes; perinuclear clearing; wrinkled nuclear membrane eMA, CK7, CD117 positive; vimetin, CD10, RCC antigen negative
Collecting duct RCC Centrally located; glandular or tubulopapillary architecture; inflammatory and desmoplastic stroma; high-grade nuclei; dysplasia of adjacent collecting ducts Ulex europeaus lectin, HMWCK, CK7, p63, vimentin positive; CD10, RCC antigen negative
Renal medullary carcinoma Centrally located; tubulopapillary, reticular, or microcystic architecture; inflammatory and desmoplastic stroma with prominent neutrophilic infiltrate; high-grade nuclei; trepanocytes (sickled erythrocytes) Ulex europeaus lectin, HMWCK, CK7, p63, vimentin positive; CD10, RCC antigen negative
Xp11 translocation carcinoma Papillary and solid architecture; psammomatous microcalcifications; cells with voluminous clear to eosinophilic cytoplasm; eosinophilic cytoplasmic inclusions TFe-3, CD10, AMACR positive; eMA, CK7, low-molecular-weight CK, HMWCK negative
Mucinous tubular and spindle cell carcinoma Tubular and focally solid architecture; tubules lined with cuboidal cells; spindle cells with low-grade nuclei; mucinous extracellular matrix eMA, vimentin, RCC antigen, CK7, CD10, AMACR positive
Clear-cell papillary RCC Cystic and partly solid tumor; tubulopapillary architecture; clear cytoplasm; apically located, low-grade nuclei CK7, eMA, vimentin positive; AMACR, CD10 negative
Tubulocystic RCC “Bubble-wrap” gross appearance; variably sized cysts embedded in fibrous stroma; clear to eosinophilic cytoplasm; prominent nucleoli CK7, CD10, AMACR, vimentin positive; HMWCK negative
Primary thyroid-like follicular carcinoma Tubular architecture; eosinophilic colloid-like material in lumen; grooved nuclei; nuclear pseudoinclusions CK7, vimentin, PAX8 positive; TTF-1, thyroglobulin, RCC antigen negative

AMACR, alpha methyl acyl Co-racemase; CK, cytokeratin; eMA, epithelial membrane antigen; HMWCK, high-molecular-weight cytokeratin; RCC, renal cell carcinoma; TTF-1, thyroid transcription factor 1.

Хромофобная почечно-клеточная карцинома

Chromophobe renal cell carcinoma (ChRCC) is the third most common RCC type, yet accounts for only about 5% of all RCCs. First described in 1985, ChRCC has distinctive morphologic and genetic features that separate it from the other RCC types. ChRCCs are soft solid tumors with a distinctive tan or tan–gray color. Microscopically, based on tinctorial properties of the cell cytoplasm, ChRCCs are divided into typical ChRCC and the eosinophilic variant. Tumor cells form sheets, nests, or broad alveoli, with interspersed thick-walled blood vessels (in contrast to the thin-walled vessels in ccRCC). Most ChRCCs have two types of cells—cells with clear cytoplasm and cells with eosinophilic cytoplasm, with one cell type predominating. Typical ChRCC predominantly has clear cells, while the eosinophilic variant is predominantly composed of eosinophilic cells. Clear cells have abundant cytoplasm with a frothy, flocculent, or bubbly appearance compared with the optically clear cytoplasm in ccRCC. eosinophilic cells tend to be smaller with finely granular eosinophilic cytoplasm. In both cell types, cytoplasmic organelles are pushed to the cell periphery, forming a halo around the nucleus. Since the organelles are pushed to the periphery, cell membranes appear thick and prominent, superficially resembling plant cells. Tumor nuclei tend to be hyperchromatic, frequently binucleated, and usually have a wrinkled nuclear membrane. The perinuclear halo and wrinkled nuclei result in the cells looking like koilocytes.

Карцинома собирательных протоков

Карцинома собирательных протоков (CDC) — редкая, агрессивная форма RCC, которая составляет <1% опухолей почек. Гистологически CDC показывают железистый или тубулопапиллярный рост с high nuclear-grade клетками, которые ассоциированы с избыточным десмопластическим стромальным ответом. Агрессивные черты, такие как некроз, лимфоваскулярная инвазия и патологоанатомически высокая злокачественность, распространены. Полная выживаемость плохая.

Медуллярная карцинома почки

Медуллярная карцинома почки (RMC) поражает молодых людей с историей серповидно-клеточной анемии и категоризируется как серповидно-клеточная нефропaтия. Гистологически RMC напоминает CDC с железистыми, ретикулярными и микрокистозными чертами, иногда с внеклеточным муцином. У большинства пациентов болезнь диагностируется на поздних стадиях со средней выживаемостью <1 года.

Муцинозная тубулярная и веретеноклеточная карцинома

Mucinous tubular and spindle cell carcinoma (MTSCC) is a rare RCC with distinctive morphology that overlaps with that of type 1 PRCC. As the name implies, these tumors are composed of tubules lined with cuboidal cells, extracellular mucin, and a bland spindle cell component. Most MTSCCs follow an indolent course when they are small and organ confined; however, larger tumors may be aggressive and possess sarcomatoid dedifferentiation.

Почечно-клеточная карцинома, ассоциированная с транслокацией Xp11.2

Xp11.2 translocation carcinoma is a distinctive RCC that is characterized by the presence of various translocations involving the TFe-3 gene located on chromosome Xp11.2, which is considered to be a part of the MiTF-TFe3 family of tumors. While translocation RCC was first identified in a subset of pediatric RCCs, these tumors are now recognized as occurring in the adult population as well. The two most common fusion partners to the TFe-3 gene are the PRCC gene t(X;1)(p11.2;q21) and the ASPL gene t(X;17) (p11.2;q25). These RCCs have distinct morphologic features, with a papillary or nested architecture that often resembles a combination of ccRCC and PRCC. Tumor cells typically have abundant clear to eosinophilic cytoplasm, with some cells having prominent intracytoplasmic hyaline globules. Numerous psammomatous calcifications may be present. Demonstration of the translocation by fluorescence in situ hybridization or immunohistochemical detection of the nuclear TFe-3 protein is required for the diagnosis. These RCCs are generally aggressive, and patients often present with locally advanced disease.

Карцинома, ассоциированная с нейробластомой

RCC, ассоциированные с нейробластомой, являются редкими опухолями с отличительными гистологическими чертами. Опухолевые клетки типично имеют избыточную эозинофильную или онкоцитарную цитоплазму.

Неклассифицируемая почечно-клеточная карцинома

Unclassified RCC is a designation for RCC that does not fit into one of the known types and is not a distinct type by itself. Since 2004, new distinct RCC types have been reported. Unclassified RCCs also include tumors that are composites of known types, for example, ccRCC and PRCC, RCC with extensive necrosis and viable tumor that cannot be typed accurately, and RCC with sarcomatoid dedifferentiation in which the epithelial component cannot be readily assigned to one of the known RCC types.


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