Fibrous Meningioma in a Patient with Encephalocraniocutaneous Lipomatosis: A Rare Case with Unique Features

Overview

Journal Int Med Case Rep J

Publisher Dove Medical Press

Specialty General Medicine

Date 2020 Sep 5

PMID 32884367

Citations 1

Authors

Majdi Al Qawasmeh

Belal Aldabbour

Kefah Alhayek

Khalid El-Salem

Affiliations

Soon will be listed here.

Abstract

Encephalocraniocutaneous lipomatosis "ECCL" is a rare, sporadic neurocutaneous disorder that results from a lethal autosomal mutation surviving by somatic mosaicism. It is characterized by unilateral involvement of skin, eyes and central nervous system in addition to a propensity for mesenchymal tumors. A 30-year-old male with previously controlled epilepsy presented with recurrent seizures. Brain imaging revealed a left parietal parasagittal enhancing tumor, in addition to left sided gyriform calcifications, and bilateral cerebral atrophy and ventricular dilatation more prominent on the left side. He also presented multiple left sided sebaceous nevi and abundant subcutaneous lipomas in addition to left mandibular condylar cysts. The brain tumor was excised, and cytopathology revealed a WHO grade I fibrous meningioma. After a thorough evaluation and exclusion of alternative diagnoses, the patient was diagnosed with definite encephalocraniocutaneous lipomatosis as per Moog's criteria. Several cases of ECCL recently presented with different intracranial neoplasms. Here we report the first case of ECCL in association with meningioma.

Citing Articles

Haberland Syndrome (Encephalocraniocutaneous Lipomatosis) with Development of Diffuse Leptomeningeal Glioneural Tumor (DL-GNT) during Adolescence.

Ferraciolli S, Tortora M, de Souza Godoy L, Reis Casal Y, Lucato L Clin Neuroradiol. 2024; 34(4):973-976.

PMID: 38353703 DOI: 10.1007/s00062-024-01389-0.

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