PEComa (Perivascular Epithelioid Cell Neoplasms) associated with synchronous bilateral papillary thyroid cancer

Abstract

Introduction:

Perivascular Epithelioid Cell Neoplasms (PEComas) are uncommon tumors of mesenchymal origin. These ones include the epithelioid angiomyolipoma (EAML), a tumor with malignant potential and whose simultaneous presentation with thyroid cancer is quite rare.

Presentation of case:

Forty years old man with fast-growing mass in relation to lower right thyroid pole and tracheal infiltration. Abdominopelvic contrast-enhanced CT shows multiple renal masses with dominant mass in the left upper pole. Thyroid histopathology showed a lesion in right lobe corresponding to tall cell variant of papillary thyroid cancer (PTC) and a second neoplasm in the contralateral lobe corresponding to classical variant of moderately differentiated PTC. In the kidney was found a tumor corresponding to EAML with epithelioid aspect cells equivalent to 40%.

Discussion:

This article corresponds to the first in the literature to describe the simultaneous presentation of EAML with synchronous bilateral PTC (SBiPTC) and its possible association. A common molecular pathway corresponding to the TSC/mTOR pathway is described, as well as SBiPTC prevalence and immunohistochemical markers for EAML diagnosis.