Clear cell papillary renal cell carcinoma

Differential diagnosis and extended immunohistochemical profile

Sean R. Williamson, John Eble, Liang Cheng, David Grignon

Research output: Contribution to journalArticle

76 Citations (Scopus)

Abstract

Clear cell papillary renal cell carcinoma is a recently recognized renal neoplasm, composed of cells with clear cytoplasm lining cystic, tubular, and papillary structures. These tumors have immunohistochemical and genetic profiles distinct from clear cell renal cell carcinoma and papillary renal cell carcinoma. We studied morphologic and immunohistochemical features (cytokeratin 7 (CK7), carbonic anhydrase IX (CAIX), CD10, alpha-methylacyl-CoA racemase, smooth muscle actin, desmin, estrogen and progesterone receptors) in 55 tumors from 34 patients, 8 of whom had end-stage renal disease. These tumors comprised 3% of all adult renal cell carcinoma resections over a period of 3 years. The patients' ages ranged from 33 to 87 years (mean 61). Multiple tumors (2-8) were present in 9 patients. Other renal tumors were present concurrently in four patients and subsequently in two patients, including: oncocytoma, clear cell renal cell carcinoma, and multilocular cystic renal cell carcinoma. Sizes ranged from 0.2 to 7.5 (mean 2.0) cm; 87% were Fuhrman grade 2, and 96% were stage pT1a. Papillary architecture was usually limited to focal branching papillae (51% of 55 tumors) or small, blunt papillae (35%). Large areas of extensively branched papillae were present in only 14% of tumors. Almost all tumors (98%) included cysts, and 18 tumors were extensively (≥90%) cystic. Immunoprofile showed CK7+, AMACR-, CD10-, CAIX+ in the tubular and papillary components of all tumors; however, CD10 labeled the apical cell membrane of cyst epithelium in 59%. The stroma was focally actin positive (94%), with infrequent desmin expression (13%). Estrogen receptor and progesterone receptor were negative. During a median follow-up period of 56 months, no patient developed local recurrence, distant or lymph-node metastasis, or cancer death. Branched tubules, small papillae, and the immunohistochemical and molecular profiles aid in distinguishing these tumors from clear cell renal cell carcinoma and multilocular cystic renal cell carcinoma.

Original languageEnglish
Pages (from-to)697-708
Number of pages12
JournalModern Pathology
Volume26
Issue number5
DOIs
StatePublished - May 2013

Fingerprint

Renal Cell Carcinoma
Differential Diagnosis
Neoplasms
Keratin-7
Desmin
Progesterone Receptors
Estrogen Receptors
Cysts
Actins
Oxyphilic Adenoma
Kidney Neoplasms
Chronic Kidney Failure
Smooth Muscle
Cytoplasm
Epithelium
Lymph Nodes
Cell Membrane
Neoplasm Metastasis
Kidney
Recurrence

Keywords

  • carbonic anhydrase IX
  • CD10
  • clear cell papillary renal cell carcinoma
  • cytokeratin 7
  • immunohistochemistry
  • kidney
  • neoplasia

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

Clear cell papillary renal cell carcinoma : Differential diagnosis and extended immunohistochemical profile. / Williamson, Sean R.; Eble, John; Cheng, Liang; Grignon, David.

In: Modern Pathology, Vol. 26, No. 5, 05.2013, p. 697-708.

Research output: Contribution to journalArticle

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abstract = "Clear cell papillary renal cell carcinoma is a recently recognized renal neoplasm, composed of cells with clear cytoplasm lining cystic, tubular, and papillary structures. These tumors have immunohistochemical and genetic profiles distinct from clear cell renal cell carcinoma and papillary renal cell carcinoma. We studied morphologic and immunohistochemical features (cytokeratin 7 (CK7), carbonic anhydrase IX (CAIX), CD10, alpha-methylacyl-CoA racemase, smooth muscle actin, desmin, estrogen and progesterone receptors) in 55 tumors from 34 patients, 8 of whom had end-stage renal disease. These tumors comprised 3{\%} of all adult renal cell carcinoma resections over a period of 3 years. The patients' ages ranged from 33 to 87 years (mean 61). Multiple tumors (2-8) were present in 9 patients. Other renal tumors were present concurrently in four patients and subsequently in two patients, including: oncocytoma, clear cell renal cell carcinoma, and multilocular cystic renal cell carcinoma. Sizes ranged from 0.2 to 7.5 (mean 2.0) cm; 87{\%} were Fuhrman grade 2, and 96{\%} were stage pT1a. Papillary architecture was usually limited to focal branching papillae (51{\%} of 55 tumors) or small, blunt papillae (35{\%}). Large areas of extensively branched papillae were present in only 14{\%} of tumors. Almost all tumors (98{\%}) included cysts, and 18 tumors were extensively (≥90{\%}) cystic. Immunoprofile showed CK7+, AMACR-, CD10-, CAIX+ in the tubular and papillary components of all tumors; however, CD10 labeled the apical cell membrane of cyst epithelium in 59{\%}. The stroma was focally actin positive (94{\%}), with infrequent desmin expression (13{\%}). Estrogen receptor and progesterone receptor were negative. During a median follow-up period of 56 months, no patient developed local recurrence, distant or lymph-node metastasis, or cancer death. Branched tubules, small papillae, and the immunohistochemical and molecular profiles aid in distinguishing these tumors from clear cell renal cell carcinoma and multilocular cystic renal cell carcinoma.",
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