Intracranial epidermoid cyst with proliferative folliculosebaceous epithelium: Report of a rare case and discussion on pathogenesis

• Published in: Neuropathology 2018-10, Vol.38 (5), p.510-515
• Main Authors: Okada, Tomu, Fujitsu, Kazuhiko, Ichikawa, Teruo, Miyahara, Kousuke, Tanino, Shin, Uriu, Yasuhiro, Hataoka, Synsuke, Tanaka, Yuusuke, Suzuki, Kouji, Niino, Hitosi, Yagishita, Saburou, Kato, Ikuma
• Format: Article
• Language: English
• Subjects: Activities of daily living; Aged; Cerebellar Diseases - pathology; Cerebellopontine Angle - pathology; Cysts; Diagnosis; Epidermal Cyst - pathology; Epidermis; epidermoid cyst; Epithelium; Epithelium - pathology; Follicles; Gait; Hair; Hair Follicle - pathology; Humans; Male; malignant transformation; Movement disorders; proliferative folliculosebaceous epithelium; Recurrence; Sebaceous gland; Sebaceous Glands - pathology; Surgery
• ISSN: 0919-6544; 1440-1789
• DOI: 10.1111/neup.12481

Intracranial epidermoid cysts are rarely known to increase in size over a brief period. While malignant transformations of epidermoid cyst have been previously described, no reports to date have described rapid proliferation accompanied by mature hair follicles and sebaceous glands without malignant transformations. The present case involved a 71‐year‐old man who visited a local physician with disturbance of equilibrium. A mass lesion was detected at the left cerebellopontine angle and was subsequently removed. Histopathological diagnosis of this lesion was as an epidermoid cyst. Seventeen years after this initial surgery, worsened left hypoacusis, disorientation, eating disorder and gait disturbance appeared, and the patient visited the physician again. Local recurrence of the lesion was observed, and the patient was referred to our hospital for its removal. Intraoperative findings revealed a pearly white tumor, histopathologically diagnosed as an epidermoid cyst. Three years later, local recurrence was observed, and the cyst was again removed. Intraoperative findings revealed a partially pearly white tumor similar to the tumor observed during the second surgery, but the majority of the tumor was non‐shiny, ochre‐colored and suckable. Histopathological diagnosis was an epidermoid cyst with an epidermis‐like structure. Postoperative activities of daily living were independent thereafter, but from March of the following year, he began to experience increasing difficulty in walking and subsequently visited our hospital again. Tumor recurrence was observed and was removed again in November of the same year. Intraoperative findings revealed a tumor that was primarily ochre‐colored, non‐shiny, brittle and suckable. The histopathological diagnosis was folliculosebaceous epithelial proliferative lesion accompanied by an epidermis‐like epithelium and a differentiation into hair follicles and sebaceous gland,s and the tumor was determined to be an epidermoid cyst with proliferative folliculosebaceous epithelium.