Non-Xp11 Translocation Associated Renal Cell Carcinoma in Patients Less Than 20 Years Old: A Clinicopathologic Study with Follow-Up.
Jason Wang, Bahig M Shehata, Simone M Langness, Angela Davis, Liang Cheng, Adeboye O Osunkoya. Emory University School of Medicine, Atlanta; Indiana University School of Medicine, Indianapolis
Background: Renal cell carcinoma (RCC) is a rare neoplasm in patients younger than 20 years old, accounting for less than 2% of all RCC cases and 5% of pediatric renal tumors. Non-translocation associated RCC are even more uncommon, in view of the fact that Xp11 translocation-associated carcinomas comprise a significant proportion of pediatric RCC. Very few large clinicopathologic studies of this subset of tumors in the pediatric population have been published to date.
Design: A search was made through the surgical pathology and consultation files of two academic institutions for non-Xp11 translocation associated RCC in patients less than 20 years old from 1995-2009. Patients with renal medullary carcinoma were excluded from the study. Clinicopathologic data including follow up was obtained.
Results: Clinical data: 12 cases of non-Xp11 translocation associated RCC were identified from 350 pediatric patients with renal tumors (prevalence = 3.4%). The patient population consists of 6/12 males (50%) and 6/12 females (50%). The mean age at diagnosis was 10 years (range 1-18 years). Pathologic data: 10/12 (83%) patients had radical nephrectomy, and 2/12 (17%) patients had partial nephrectomy. The mean tumor size was 6.8 cm (range 1.6-12 cm). 5 cases were papillary RCC (42%), 4 cases were clear cell RCC (33%), and 3 cases were chromophobe RCC (25%). A TFE-3 stain was performed and was negative in all cases. 9/12 cases (75%) were Fuhrman nuclear grade 2, and 3/12 cases (25%) were Fuhrman nuclear grade 3. Pathologic staging was as follows: pT1, 6/12 cases (50%); pT2, 3/12 cases (25%); pT3, 2/12 cases (17%); unknown, 1/12 case (8%). 3/12 cases (25%) had regional lymph node metastasis. Angiolymphatic invasion (ALI) was identified in 4 cases (33%). 1/12 case (8%) each had perinephric adipose tissue and renal sinus fat invasion. Tumor necrosis was present in 5/12 cases (42%). Follow-up data: Mean duration of follow-up was 33 months (range 11-55 months). All patients were alive at last follow-up, including 1 patient with lung metastasis, and another with local recurrence.
Conclusions: Pathologic parameters typically associated with poor outcome in adults, including metastasis/high tumor stage, high Fuhrman nuclear grade, ALI, and tumor necrosis did not lead to death in any of the patients in our series. Non-Xp11 translocation associated RCC though relatively rare, should be considered in the differential diagnosis of pediatric renal tumors, and may have a better outcome than in adults.
Category: Genitourinary (including renal tumors)
Monday, February 28, 2011 9:30 AM
Poster Session I Stowell-Orbison/Surgical Pathology/Autopsy Awards Poster Session # 111, Monday Morning