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Presented by George Netto, M.D. and prepared by Zarir E. Karanjawala, M.D., Ph.D.
Case 2: A 21 year old gentleman presented with gross hematuria.
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1. Question
Week 326: Case 2
A 21 year old gentleman presented with gross hematuria. He was found to have bilateral renal neoplasms. During a right radical nephrectomy, a needle core biopsy from a left renal mass was performed. Three tumors were identified in the right kidney with identical morphology. The Left renal biopsy is also shown below.images/netto100107_2A.jpg
images/netto100107_2b.jpg
images/netto100107_2c.jpg
images/netto100107_2d.jpgCorrect
Answer: Hybrid oncocytic – Chromophobe renal neoplasm (Hybridoma)
Histology: All three right sided tumors and the left renal mass shared similar morphologic features. The tumors are composed of oncocytic epithelial proliferation arranged in solid nests. Background hyalinized – edematous stroma characteristic of renal oncocytomas is only focally noted. In many areas, the tumor cells exhibit cytologic features of chromophobe cell carcinoma such as the presence of “raisinoid” nuclear morphology, imparted by the irregular nuclear membranes and the frequent perinuclear halos.
Discussion: Hybrid oncocytic renal neoplasms are encountered in the setting of Birt-Hogg-Dube (BHD) syndrome. BHD is an autosomal dominant genodermatosis characterized by the development of benign hair follicle tumors (fibrofolliculomas), an increased risk of renal neoplasia and spontaneous pneumothorax. BHD patients can develop multiple, at time bilateral renal tumors with variable morphology to include hybridomas, chromophobe renal cell carcinomas and conventional clear cell carcinomas. In most patients, the non neoplastic renal parenchyma reveals microscopic evidence of “oncocytosis”. BHD gene is mapped to chromosome 17p11.2. It encodes a novel protein of yet unknown function (follicullin). Relatives of affected individual can be tested for the presence of a germline BHD mutation to determine if they are at increased risk to develop renal malignancies.
Reference(s):
– Pavlovich CP, Grubb RL 3rd, Hurley K, Glenn GM, Toro J, Schmidt LS, Torres-Cabala C, Merino MJ, Zbar B, Choyke P, Walther MM, Linehan WM. Evaluation and management of renal tumors in the Birt-Hogg-Dube syndrome. J Urol. (2005) 173:1482-6.
– Pavlovich CP, Walther MM, Eyler RA, Hewitt SM, Zbar B, Linehan WM, Merino MJ. Renal tumors in the Birt-Hogg-Dube syndrome. Am J Surg Pathol. (2002) 26:1542-52.Incorrect
Answer: Hybrid oncocytic – Chromophobe renal neoplasm (Hybridoma)
Histology: All three right sided tumors and the left renal mass shared similar morphologic features. The tumors are composed of oncocytic epithelial proliferation arranged in solid nests. Background hyalinized – edematous stroma characteristic of renal oncocytomas is only focally noted. In many areas, the tumor cells exhibit cytologic features of chromophobe cell carcinoma such as the presence of “raisinoid” nuclear morphology, imparted by the irregular nuclear membranes and the frequent perinuclear halos.
Discussion: Hybrid oncocytic renal neoplasms are encountered in the setting of Birt-Hogg-Dube (BHD) syndrome. BHD is an autosomal dominant genodermatosis characterized by the development of benign hair follicle tumors (fibrofolliculomas), an increased risk of renal neoplasia and spontaneous pneumothorax. BHD patients can develop multiple, at time bilateral renal tumors with variable morphology to include hybridomas, chromophobe renal cell carcinomas and conventional clear cell carcinomas. In most patients, the non neoplastic renal parenchyma reveals microscopic evidence of “oncocytosis”. BHD gene is mapped to chromosome 17p11.2. It encodes a novel protein of yet unknown function (follicullin). Relatives of affected individual can be tested for the presence of a germline BHD mutation to determine if they are at increased risk to develop renal malignancies.
Reference(s):
– Pavlovich CP, Grubb RL 3rd, Hurley K, Glenn GM, Toro J, Schmidt LS, Torres-Cabala C, Merino MJ, Zbar B, Choyke P, Walther MM, Linehan WM. Evaluation and management of renal tumors in the Birt-Hogg-Dube syndrome. J Urol. (2005) 173:1482-6.
– Pavlovich CP, Walther MM, Eyler RA, Hewitt SM, Zbar B, Linehan WM, Merino MJ. Renal tumors in the Birt-Hogg-Dube syndrome. Am J Surg Pathol. (2002) 26:1542-52.
