Unusual Clinical and MRI Features of a Cerebellopontine Angle Medulloepithelioma

Abstract

We describe for the first time an unusual location and clinical presentation of medulloepithelioma, a rare embryonal tumor. A 5-year-old child presented with sudden onset of bilateral hearing loss. On imaging, the lesion appeared to be extra axial and was located in the right cerebello-pontine (CP) angle, extending into middle fossa along the trigeminal ganglion and in front of the brain stem into the opposite CP angle. It did not show any enhancement following contrast administration and had restricted diffusion on diffusion-weighted imaging, simulating an epidermoid. However, in vivo localized proton MR spectroscopy revealed a creatine peak dominated by a large choline resonance, peak of glycine with lactate/lipid and invisible N-acetylaspartate suggestive of a neoplastic lesion and not an epidermoid. Only subtotal resection could be performed and the patient had a stormy post-operative course due to extensive dissemination of the disease.

Unusual occurrence of supratentorial medulloepithelioma in a young female

“…Similar to the previously reported cases, our case presented with signs of raised intracranial tension and showed a predominantly large hypointense lesion on T1WI, hyperintense on T2WI, in the left parietal region, with surrounding edema and mass effect with midline shift, and showed enhancement on contrast[27] On histopathology, multiple lines of differentiation, including, neuronal and mesenchymal elements, and glial differentiation were reported by employing immunohistochemistry. [126810] In the case reported here, evidence for neuronal and mesenchymal differentiation was evident and follow-up confirmed 14 months of recurrence-free survival.…”

“…They are mostly supratentorial in the periventricular location, involving in order of frequency, the temporal, parietal, occipital, and frontal lobes, however, peripheral locations like the cauda equina, sciatic nerve, and orbit have rarely been reported in literature. [2678] They display a highly malignant behavior, with early progression or recurrence and dismal prognosis. Most children die within five months, and only three cases have survived beyond five years.…”

“…Most children die within five months, and only three cases have survived beyond five years. [12679] There are only two previously reported cases in adults, aged 23 and 20 years, located in the fronto-temporal and temporal lobes. [34]…”

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

“…components versus solid components varied greatly. After administration of gadolinium in 14 prior cases, MEPL usually presented with nonhomogeneous enhancement (n = 8), 5,20,22,24,26,29,32,33 but no enhancement (n = 3) was seen in studies by Hayase et al, 15 Mekni et al, 21 and Syal et al, 34 which is similar to case 5 from our series. The radiological characteristics of MEPL are nonspecific and cannot directly imply the diagnosis of MEPL or distinguish MEPL from other solid-cystic lesions.…”

“…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.…”