Journal
AMERICAN JOURNAL OF CLINICAL PATHOLOGY
Volume 128, Issue 1, Pages 70-79Publisher
AMER SOC CLINICAL PATHOLOGY
DOI: 10.1309/LR5G1VMXPY3G0CUK
Keywords
translocation; renal; carcinoma; TFE3; TFEB; adult; aggressive; clinical
Categories
Ask authors/readers for more resources
Renal cell carcinomas associated with Xp IL 2 translocations (TFE3 genefusions) are rare tumors predominantly reported in children. We studied 5 cases of translocation carcinoma in adult patients, 18 years or older (mean age, 32.6 years). Tumors were examined histologically, immunohistochemically, and electron microscopically and correlated with the clinical picture. Most tumors showed solid sheets of clear to eosinophilic cells with rich vasculature and foci of papillary or pseudopapillary architecture. All cases showed strong nuclear positivity for TFE3. Vimentin and CD1 0 were positive in the cytoplasm. A panel of cytokeratin antibodies, smooth muscle actin, CD45, HMB45, and calretinin were negative. Patients had nonspecific initial complaints and were diagnosed with advanced disease, most with distant metastases. Various treatments met with minimal success. Unlike pediatric patients, the adult patients followed a rapidly terminal course, with a mean survival of 18 months after diagnosis (range, 10-24 months).
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available