Oncocytic choroid plexus carcinoma: case report

• Published in: Clinical neuropathology Vol. 29; no. 1; p. 14
• Main Authors: Sav, A, Scheithauer, B W, Mazzola, C A, Ketterling, S R P, Thompson, S J, Reilly, M H
• Format: Journal Article
• Language: English
• Published: Germany 01-01-2010
• Subjects: Adenoma, Oxyphilic - pathology; Brain - metabolism; Brain - pathology; Carcinoma - metabolism; Carcinoma - pathology; Carcinoma - therapy; Choroid Plexus Neoplasms - metabolism; Choroid Plexus Neoplasms - pathology; Choroid Plexus Neoplasms - therapy; Family; Female; Humans; Infant; Magnetic Resonance Imaging; Oxyphil Cells - metabolism; Oxyphil Cells - pathology; Tomography, X-Ray Computed
• ISSN: 0722-5091
• DOI: 10.5414/NPP29014

Herein, we report an unusual choroid plexus carcinoma with extensive oncocytic transformation. A 13-month-old girl presented with acute lethargy which quickly progressed to coma. A CT scan of the head revealed impending herniation due to hemorrhage within an intracranial tumor. An MRI scan showed a large, partly cystic and highly vascular left lateral ventricular mass. A near total resection was achieved. Microsections revealed a WHO Grade III choroid plexus carcinoma with extensive oncocyti c transformation. A minor portion of the moderately to poorly differentiated tumor exhibited classical microscopic features of choroid plexus carcinoma, including marked nuclear atypia, brisk mitotic activity (78/10 HPF), a high MIB-1 labeling index (44%) and zones of necrosis. In contrast, the large, eosinophilic, cytologically malignant but granular-appearing oncocytes comprising the majority of the lesion showed scant (1/10 HPF) mitotic activity and only a low MIB-1 labeling index (5%). A subsequent recurrence at 1 year consisted entirely of non-oncocytic tumor. Choroid plexus carcinoma with oncocytic transformation has not been previously reported. The remarkable extent of this alteration and its clinical significance remains to be determined.