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Int J Clin Exp Pathol 2013;6(12):2936-2942

Original Article
Clear cell papillary renal cell carcinoma: a clinicopathological study emphasizing
ultrastructural features and cytogenetic heterogeneity

Shan-Shan Shi, Qin Shen, Qiu-Yuan Xia, Pin Tu, Qun-Li Shi, Xiao-Jun Zhou, Qiu Rao

Department of Pathology, Nanjing Jinling Hospital, Nanjing University School of Medicine, Nanjing, China. Equal contributors.

Received September 12, 2013; Accepted September 27, 2013; Epub November 15, 2013; Published December 1, 2013

Abstract: Clear cell papillary renal cell carcinoma (CCPRCC) is a recently recognized renal neoplasm, which was initially described in end-
stage renal disease (ESRD), but some cases have been reported in otherwise normal kidneys. We report a series of 11 CCPRCC (age range,
33-72 years; male-to-female ratio, 8:3). Follow-up was available for 8 patients. No patients developed local recurrence, distant or lymph-node
metastasis, or cancer death. Histologically, all tumors exhibit morphologic features typical of CCPRCC including a mixture of cystic and
papillary components, covered by small to medium-sized cuboidal cells with abundant clear cytoplasm. All 11 cases exhibited moderate to
strong positivity for CK7, CA9, Vim, and HIF-1α, coupled with negative reactions for CD10, P504S, and RCC. We did not find any VHL gene
mutations in all 11 cases. Losses of chromosomes 3 (monoploid chromosome 3) was detected in 3 cases. Ultrastructurally, the tumor cells
composed of numerous glycogens with scanty cell organelles, reminiscent of clear cell renal cell carcinoma (CCRCC). In conclusion, the
coexpression of CA9 and HIF-1α in the absence of VHL gene abnormalities in CCPRCC suggests activation of the HIF pathway by
mechanisms independent of VHL gene mutation. Losses of chromosomes 3 (monosomies chromosome 3) was detected in 3 cases
suggesting that at least some of these lesions have demonstrated abnormalities of chromosomes 3. Ultrastructurally, CCPRCC composed of
numerous glycogens with scanty cell organelles, reminiscent of CCRCC suggesting the close pathogenesis relationship of CCPRCC with
CCRCC. (IJCEP1309033).

Keywords: Renal cell carcinoma, clear cell papillary renal cell carcinoma, VHL, immunohistochemistry, fluorescence in situ hybridization,
ultrastructure

Address correspondence to: Dr. Qiu Rao and Dr. Xiao-Jun Zhou, Department of Pathology, Nanjing Jinling Hospital, Nanjing University School
of Medicine, Nanjing, China. Tel: 86-25-80860191; Fax: 86-25-80860191; E-mail: raoqiu1103@126.com (Qiu Rao); zhouxj1@126.com (Xiao-
Jun Zhou)