MP55-01 MIXED EPITHELIAL AND STROMAL TUMOR FAMILY: A SINGLE INSTITUTIONAL EXPERIENCE WITH THIS RARE RENAL ENTITY

Abstract

You have accessJournal of UrologyKidney Cancer: Localized: Surgical Therapy III1 Apr 2017MP55-01 MIXED EPITHELIAL AND STROMAL TUMOR FAMILY: A SINGLE INSTITUTIONAL EXPERIENCE WITH THIS RARE RENAL ENTITY Mahyar Kashan, Mazyar Ghanaat, Maria Becerra, Andreas M. Hötker, Brandon Manley, Jozefina Casuscelli, Nicole Benfante, Shawn Mendonca, Satish Tickoo, Oguz Akin, Paul Russo, Jonathan Coleman, and A Ari Hakimi Mahyar KashanMahyar Kashan More articles by this author , Mazyar GhanaatMazyar Ghanaat More articles by this author , Maria BecerraMaria Becerra More articles by this author , Andreas M. HötkerAndreas M. Hötker More articles by this author , Brandon ManleyBrandon Manley More articles by this author , Jozefina CasuscelliJozefina Casuscelli More articles by this author , Nicole BenfanteNicole Benfante More articles by this author , Shawn MendoncaShawn Mendonca More articles by this author , Satish TickooSatish Tickoo More articles by this author , Oguz AkinOguz Akin More articles by this author , Paul RussoPaul Russo More articles by this author , Jonathan ColemanJonathan Coleman More articles by this author , and A Ari HakimiA Ari Hakimi More articles by this author View All Author Informationhttps://doi.org/10.1016/j.juro.2017.02.1694AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookTwitterLinked InEmail INTRODUCTION AND OBJECTIVES In 2016 the World Health Organization coined the term mixed epithelial and stromal tumor family (MESTF) to encompass adult cystic nephroma (ACN) within the classification of mixed epithelial and stromal tumors (MESTs) on the basis of overlapping clinical and pathologic profiles. MESTs have traditionally been regarded as benign, with only a few reports of malignant transformation or recurrence in the literature. Diagnosis requires histopathological evaluation, as radiologic imaging cannot accurately determine whether such tumors are benign or malignant. We aim to characterize our institutional experience with this rare neoplasm. METHODS We identified all patients with a pathological diagnosis of MEST or ACN from our prospectively collected institutional database between Jan 1995 - Dec 2015. Available imaging was re-reviewed by a single expert radiologist (AH). Demographic, radiologic, and clinical characteristics were recorded. RESULTS Data was available for 40 patients. The median age at diagnosis was 48.8 years (31.5-73.4). Thirty-seven (92.5%) patients were female and three (7.5%) were male. Imaging was available for re-review for 29 (72.5%) patients. The mean diameter of the tumor on preoperative imaging was 5.8 cm (1.9-16.1). Patients presenting with symptoms at diagnosis had a mean diameter of 7.4 cm (4-16.1). On imaging, 25 (86.2%) tumors where characterized as Bosniak 3 lesions and four (13.8%) were described as Bosniak 4 lesions. All patients underwent surgical resection, with partial nephrectomy performed in 72.5% of cases. Mean pathological tumor size was 5.9 cm (1.5-15). Median follow-up was 71.6 months (1-217). Two (4.9%) patients died from non-tumor related causes. At last follow-up, all patients showed no evidence of disease. CONCLUSIONS MESTF are indolent tumors with a female predominance. They are usually detected incidentally as Bosniak 3 or 4 lesions. Partial nephrectomy should be performed whenever possible to resect the tumor and preserve renal function. Given the low likelihood of recurrence following excision, we believe that, once pathologically identified, patients have an excellent long-term prognosis and require minimal surveillance imaging on follow-up. Genomic characterization is currently underway. © 2017FiguresReferencesRelatedDetails Volume 197Issue 4SApril 2017Page: e730 Advertisement Copyright & Permissions© 2017MetricsAuthor Information Mahyar Kashan More articles by this author Mazyar Ghanaat More articles by this author Maria Becerra More articles by this author Andreas M. Hötker More articles by this author Brandon Manley More articles by this author Jozefina Casuscelli More articles by this author Nicole Benfante More articles by this author Shawn Mendonca More articles by this author Satish Tickoo More articles by this author Oguz Akin More articles by this author Paul Russo More articles by this author Jonathan Coleman More articles by this author A Ari Hakimi More articles by this author Expand All Advertisement Advertisement PDF downloadLoading ...