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A man in his 30s with a history of treatment-naive HIV (CD4 count < 200 cells/mm3) and untreated syphilis presented to the emergency department with 3 weeks of progressively worsening dysphonia, dyspnea, and biphasic stridor. On arrival to the hospital, the patient had a computed tomography scan performed; results demonstrated an obstructive laryngeal mass but no signs of local invasion or lymphadenopathy. The patient was taken urgently to the operating room where an awake tracheostomy was performed without complication. Findings of a direct laryngoscopy demonstrated violaceous nodules of the base of the tongue as well as a mass of the infraglottis and subglottis, which was nearly completely obstructing the airway (Figure, A and B). Full physical examination findings also demonstrated violaceous nodules involving the patient’s lower extremities. Results of an excisional biopsy demonstrated a subepithelial spindle cell lesion with extravasation of erythrocytes and stained strongly for human herpesvirus-8 (Figure, C).
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A. Kaposi sarcoma
Kaposi sarcoma (KS), first described by Hungarian dermatologist Moritz Kaposi in the 19th century, is a neoplastic disorder of the vascular endothelium affecting mucocutaneous tissues with the potential for lymph node and visceral involvement. Kaposi initially described slowly growing purple plaques involving the extremities of older adult men from the Mediterranean region, which is now known as the classic form. Multiple other forms have since been described, including endemic, AIDS-related, and iatrogenic. The endemic form affects HIV-seronegative, predominantly male children and young adults in sub-Saharan Africa and displays a variable clinical course ranging from indolent skin disease to locally infiltrative lesions with visceral involvement. The iatrogenic form refers to disease associated with immunosuppressive agents and typically develops in liver transplant recipients, again with a variable clinical course. AIDS-associated KS is the most common and most aggressive form, with more than half of patients demonstrating visceral involvement, typically of the gastrointestinal tract and lungs.1 Prognostic factors for staging of AIDS-associated KS include the amount of tumor extension and degree of systemic involvement.2
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Corresponding Author: Nadia Mohyuddin, MD, Weill Cornell College of Medicine, Department of Surgery, Texas A&M University College of Medicine, Institute of Academic Medicine, Houston Methodist Hospital, Houston Methodist ENT Specialists, 6550 Fannin St, Ste 1723, Houston, TX 77030 (firstname.lastname@example.org).
Published Online: May 7, 2020. doi:10.1001/jamaoto.2020.0223
Conflict of Interest Disclosures: None reported.
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