Case of conjunctival Kaposi's Sarcoma reported in NEJM

Kaposi sarcoma (KS) is a vascular spindle cell tumor of endothelial cell origin which can present in the skin, viscera, or mucosa and find its name as it was first described in 1872 by Dr. Moritz Kaposi.Conjunctival Kaposi's sarcoma (KS) is a red to pink conjunctival tumor and a blue to purple eyelid tumor. It is typically found in patients with HIV acquired immunodeficiency syndrome (AIDS), but can occur in the elderly and immunocompromised patients like transplant patients. Classic Kaposi's sarcoma when occurs in the elderly is slowly progressive.

Conjunctival or adnexal Kaposi sarcoma is not commonly encountered by healthcare providers since the advent of highly active antiretroviral therapy. It is vital for all healthcare providers to have Kaposi sarcoma in the differential diagnosis of a hemorrhagic conjunctival or adnexal mass and be familiar with its association with HIV/AIDS. If suspected, appropriate counseling and testing should be performed.

According to history a 50-year-old man presented to the ophthalmology clinic with a large lesion on his left eye that had developed over the preceding month. Physical examination showed a red, mobile, nodular mass on the inferior bulbar conjunctiva and violaceous plaques on the patient's back and lower limbs.

Antibody testing for human immunodeficiency virus (HIV) type 1 was positive. The viral load was 147,000 copies per milliliter (reference range, <40), and the CD4 cell count was 116 per cubic millimeter (reference range, 500 to 1200).

A biopsy specimen of the skin showed a spindle-cell neoplasm, and immunohistochemical testing for human herpesvirus 8 was positive. These findings were consistent with Kaposi's sarcoma, a malignant vascular neoplasm that is related to human herpesvirus 8 infection and that typically occurs in patients with immunodeficiency.

Rapidly enlarging vascular or bleeding lesions in the eye in a patient with HIV infection should alert physicians to a possible diagnosis of conjunctival Kaposi's sarcoma. Antiretroviral therapy and doxorubicin were initiated. Five months after presentation, the conjunctival mass had resolved (Panel B), as had the skin lesions.

DOI: 10.1056/NEJMicm2105217