Unusual occurrence of supratentorial medulloepithelioma in a young female

Abstract

Medulloepithelioma is an extremely rare PNET in late adolescence and adults with only two cases noted in literature. These are WHO grade IV tumors with dismal prognosis. Only few cases survived beyond 5 months. We report a rare case of supratentorial medulloepithelioma in a 17 year old girl. She had presented with right sided weakness, headache and vomiting. Imaging showed an enhancing mass lesion in left parietal region which undergone gross total resection. After surgery, her headache, vomiting and right sided weakness improved. On histopathology, the tumor had characteristic trabecular, ribbon and palisaded arrangement with brisk mitotic activity, necrosis and calcification. Immuno-histochemistry revealed positivity for Synaptophysin, Vimentin and EMA while GFAP was negative. MIB-1 labeling was very high. Patient received postoperative radiotherapy. On follow up after 14 months, she was clinically asymptomatic with no recurrence on imaging.

Clinicoradiological features and surgical outcomes of primary intracranial medulloepitheliomas: a single-center experience and pooled analysis of individual patient data

“…However, patients older than 10 years have been reported. 6,9,19,21,29,31 No sex predominance was observed. Occasionally, some uncommon symptoms, such as increased head size, 16,30,35 anorexia, 13 and torticollis, 23,27 were reported.…”

“…The radiological features have been rarely described in prior studies. The morphology was reported in only 21 cases (38.2%), among which 6 cases were solid, 8,18,22,26,34 11 cases were solid-cystic, 1,5,7,10,15,18,20,29,32,33 and 4 cases were cystic-solid. 24,28,37 Combined with our cases, it could be speculated that cystic degeneration is a common trait of MEPL; however, the proportion of lesions with cystic FIG.…”

Intracranial medulloepithelioma in a child: a case report

“…There have been approximately 40 cases of this entity reported in the literature (4). Notably, only two adult patients have been reported (11).…”

“…Therefore, medulloepitheliomas may occur anywhere along the entire neural axis. However, only five cases of peripheral medulloepitheliomas have been reported (11). The cerebrum is the most common location, accounting for nearly 30% of all cases.…”

“…The cerebrum is the most common location, accounting for nearly 30% of all cases. Medulloepitheliomas are commonly found in the periventricular location, involving (in order of frequency) the temporal, parietal, occipital, and frontal lobes (7,11). Medulloepithelioma is known for its high malignancy, and the median survival time is 5 months (10).…”