Clinicopathologic Classification of Renal Cell Carcinoma in Patients ≤40 Years Old From Peru

Abstract

Introduction There are scant data on renal cell carcinoma (RCC) from relatively younger patients in South America using contemporary classification. Methods Fifty-nine consecutively treated patients with RCC (≤40 years old) were assessed from the National Institute of Neoplastic Diseases in Peru from 2008 to 2020 (34 males; 25 females), age range of 13 to 40 years. Results Most common presenting symptoms were flank pain (n = 40), hematuria (n = 19), and weight loss (n = 12). Associated conditions included 4 patients with proven or presumed tuberous sclerosis and 1 patient with von Hippel Lindau syndrome, all with clear cell RCC. Tumor histopathology was clear cell RCC in 32 of 59 (54%), chromophobe RCC in 6 of 59 (10%), and 5 of 59 (8%) each of papillary RCC and MiT family translocation-associated RCC. Four of 59 (7%) were FH-deficient RCC and 2 of 59 (3%) remained unclassified. The remaining tumors were isolated examples of clear cell papillary renal cell tumor, eosinophilic solid and cystic RCC (ESC RCC), RCC with fibromyomatous stroma, sarcomatoid RCC, and sarcomatoid clear cell RCC. Of the 4 FH-deficient RCCs, none had the classic morphology. The 5 MiT family translocation RCCs had variable morphology. There were 41 tumors without recurrence or metastases, 3 tumors with local recurrence only, 8 tumors with metastases only, and 7 tumors with both local recurrence and metastases. Conclusions The current study demonstrates the importance of special studies in accurately classifying RCC in younger individuals. The distribution of RCC subtypes in younger individuals is similar between 2 representative large institutions of the United States and Peru.

A TFEB-Amplified Renal Cell Carcinoma with Long-Term, Complete Immunotherapy Response: Retrospective Support for the Value of Molecular Classification

“…TFEB-rearranged RCCs tend to occur in younger patients, with a median age in the fourth or early fifth decade, while TFEB -amplified RCCs occur in older patients with a median age in the seventh decade. 4,5 Moreover, TFEB -amplified RCCs tend to show more aggression, with the presentation at higher stage or with metastasis. 3,6…”

“…TFEB-rearranged RCCs tend to occur in younger patients, with a median age in the fourth or early fifth decade, while TFEB-amplified RCCs occur in older patients with a median age in the seventh decade. 4,5 Moreover, TFEB-amplified RCCs tend to show more aggression, with the presentation at higher stage or with metastasis. 3,6 In light of recent scholarship, functionally implicating TFEB itself as a mediator of immune evasion through induction of PDL1 expression, 7 as well as observations of PDL1 expression in TFEB-altered RCCs, 8,9 including most recently TFEB-amplified RCCs, 10 we performed retrospective searches of institutional pathologic databases, with the goal of characterizing PDL1 expression in our patient population.…”