Vulvar Kaposi Sarcoma

Kaposi sarcoma (formerly multiple hemorrhagic sarcoma) usually affects extremities; vulvar involvement is rare. Vulvar Kaposi sarcoma presents as nonspecific mass: initially blue-red macules or small nodules, slowly enlarging, increasing in number, fusing into larger nodules or polyps; may have vulvar pruritus, burning, or pain. Types: classic indolent, African/endemic, iatrogenic, AIDS-related. Associated with human herpesvirus 8 (HHV-8) infection.

Common Symptoms

Papule; polyp; vulvar burning; bone destruction; osteoporosis; nodule; cyst; secondary infection; pruritus

Causes and Pathogenesis

(1) Etiology: human herpesvirus-8 (KSHV) closely linked; serologically/biochemically linked to CMV; high incidence in AIDS supports viral theory. Other hypotheses (geographic/ethnic/climate/sex/genetic/trauma/occupational/infectious) not fully proven. Current view: immune impairment allows viral infection or reactivation of oncogenic virus causing KS. (2) Mechanism: dark red/purplish macules/papules/plaques/nodules (may be gray-yellow); irregular macules, fairly well-defined borders; uneven plaques; ulcerated surface with yellow exudate. Histology: mixed-cell, monomorphic, anaplastic; by vascular/spindle-cell ratio: angiomatous vs sarcomatous. Early: increased dermal vessels, stromal edema, inflammatory infiltrate, extravasated RBCs/hemosiderin. Progress: spindle-cell areas mix with angiomatous areas; slit-like spaces containing RBCs; PAS-positive hyaline globules; advanced: spindle nuclei enlarge/hyperchromatic, mitoses increase, high-grade sarcoma. EM: tumor cells + neoplastic vessels; Weibel-Palade bodies; basement membrane around pericytes. IHC: endothelial cells FVIII+, spindle cells partial+; CD34/CD31+.

Treatment

Surgery, cryotherapy, radiotherapy, alpha-interferon

Preventive Measures

Tertiary tumor prevention; regular physical exams; boost immunity; seek care early.

Department to Visit

It is recommended to visit the Gynecology department; an appointment can be booked on this platform before the visit.

Warm reminder: The above information is for health science reference only and cannot replace professional medical diagnosis. If you have related symptoms, please seek medical attention at a regular hospital promptly.