Journal of Pathology and Translational Medicine

Case Reports

Complex Conjunctival Choristoma in Linear Nevus Sebaceous Syndrome: A case report.: Jinyoung Yoo, Jae Kyung Koh, Seok Jin Kang, Byung Kee Kim; Korean J Pathol. 2000;34(9):680-684.

Abstract PDF: Linear nevus sebaceous syndrome is an uncommon neurocutaneous disorder characterized by nevus sebaceus, seizures, and mental retardation, and is often accompanied by ocular anormalies. We recently experienced a case of conjunctival choristoma associated with linear nevus sebaceous syndrome in a 17-month-old boy. The child was the product of a full-term uncomplicated pregnancy and normal delivery with normal karyotype of 46, XY. He was noted at birth to have a conjunctival mass on the right eye and linear nevi over the midline facial region and right temporoparietal scalp. Ophthalmologic examination revealed optic atrophy, peripapillary staphyloma, macular hypogenesis, and osseous choristoma of the choroid. A computed tomography scan demonstrated widening of the subarachnoid space and moderate atrophy of the cerebral and cerebellar hemispheres. He has been followed by ophthalmology and completely free of seizures since birth. The patient underwent excision of the conjunctival mass and biopsies of the skin lesion at 17 months of age. Histologic examination of the skin biopsies was characteristic for the linear sebaceous nevus of Jadassohn. Histologic findings of the conjunctival lesion showed a complex choristoma that contained lacrimal gland tissue and cartilage.

Squamous Cell Carcinoma Developed in Nevus Sebaceus: A Case Report.: Hyun Joo Choi, Jinyoung Yoo, Lee So Maeng, Seok Jin Kang, Chang Suk Kang; Korean J Pathol. 2004;38(1):60-63.

Abstract PDF: We report a case of squamous cell carcinoma developed within the nevus sebaceus (NS) and review the literature. An 82-year-old woman presented with a 3-month history of pain within the lifelong skin lesion on her right cheek. Examination revealed a 1.0 x 1.0 cm poorly marginated, slightly raised yellow-brown nodule. Microscopically, the skin nodule revealed the typical findings of NS. Squamous cell carcinoma developed contiguously within the keratin-filled infundibulocyst of NS disconnected from the epidermis. We suggest that squamous cell carcinoma is derived from the embryonal stratum germinativum de-differentiated from the primary epithelial germ cells in the infundibulocyst of NS. Most secondary carcinomas associated with NS grow slowly over a period of years. In contrast, squamous cell carcinoma developed in NS often grow rapidly with aggressive behavior. Thus, the accurate pathologic diagnosis is necessary.

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