T2-weighted Signal Intensity Correlates with Histology in Patients with Hereditary Leiomyomatosis and Renal Cell Cancer Syndrome

Thursday, October 11, 2012 — Poster Session III
10:00 a.m. – Noon	Natcher Conference Center, Building 45	NICHD	ENDOC-5

Authors

• KM Devine
• AY Armstrong
• JH Segars
• M Merino
• M Linehan
• A Venkatesan

Abstract

Objective: Hereditary Leiomyomatosis and Renal Cell Cancer (HLRCC) is an AD-inherited syndrome characterized by susceptibility to renal cell cancer, skin and cellular uterine leiomyomas, and possibly, leiomyosarcoma. We hypothesized an association between MRI signal intensity and HLRCC leiomyoma histopathology. Methods: Preoperative T2-weighted MRI signal intensity of leiomyomas was characterized (Funaki 2006) in five patients as: (1) low, similar to muscle (2) intermediate, between skeletal muscle and myometrium (3) high, myometrium. Microscopic evaluation of leiomyoma samples was performed. The radiologist analyzing MRIs was blinded to pathologic diagnosis. Results: 43 fibroids were Funaki 2/3 by T2 weighted MRI, i.e. the majority of fibroids overall and for each individual patient. Three patients underwent myomectomy. Two underwent hysterectomy. 26/26 fibroids sectioned were cellular/atypical. Specifically, specimens demonstrated increased cellularity, nuclear pleomorphism, and occasional mitotic figures. 7/26 fibroids from 2 patients were subcategorized as smooth muscle tumor of undetermined malignant potential. No leiomyosarcoma or any malignancy were diagnosed. Discussion: MRI T2 signal intensity in all patients was higher than observed in common, spontaneous fibroids, and all leiomyomas studied were assessed as atypical by surgical pathology. These results support a larger study to determine if MRI may enable surveillance of leiomyoma in HLRCC patients without surgical intervention.

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