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HIV

HIV-Positive Patient With an Obstructing Laryngeal Mass

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

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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Article Information

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 (ngmohyuddin@houstonmethodist.org).

Published Online: May 7, 2020. doi:10.1001/jamaoto.2020.0223

Conflict of Interest Disclosures: None reported.

References
1.
Martellotta  F , Berretta  M , Vaccher  E , Schioppa  O , Zanet  E , Tirelli  U .  AIDS-related Kaposi’s sarcoma: state of the art and therapeutic strategies.   Curr HIV Res. 2009;7(6):634-638. doi:10.2174/157016209789973619PubMedGoogle ScholarCrossref
2.
La Ferla  L , Pinzone  MR , Nunnari  G ,  et al.  Kaposi’s sarcoma in HIV-positive patients: the state of art in the HAART-era.   Eur Rev Med Pharmacol Sci. 2013;17(17):2354-2365.PubMedGoogle Scholar
3.
Fatahzadeh  M .  Kaposi sarcoma: review and medical management update.   Oral Surg Oral Med Oral Pathol Oral Radiol. 2012;113(1):2-16. doi:10.1016/j.tripleo.2011.05.011PubMedGoogle ScholarCrossref
4.
Aboulafia  DM .  The epidemiologic, pathologic, and clinical features of AIDS-associated pulmonary Kaposi’s sarcoma.   Chest. 2000;117(4):1128-1145. doi:10.1378/chest.117.4.1128PubMedGoogle ScholarCrossref
5.
Douglas  JL , Gustin  JK , Dezube  B , Pantanowitz  JL , Moses  AV .  Kaposi’s sarcoma: a model of both malignancy and chronic inflammation.   Panminerva Med. 2007;49(3):119-138.PubMedGoogle Scholar
6.
Ramírez-Amador  V , Anaya-Saavedra  G , Martínez-Mata  G .  Kaposi’s sarcoma of the head and neck: a review.   Oral Oncol. 2010;46(3):135-145. doi:10.1016/j.oraloncology.2009.12.006PubMedGoogle ScholarCrossref
7.
Mohanna  S , Bravo  F , Ferrufino  JC , Sanchez  J , Gotuzzo  E .  Classic Kaposi’s sarcoma presenting in the oral cavity of two HIV-negative Quechua patients.   Med Oral Patol Oral Cir Bucal. 2007;12(5):E365-E368.PubMedGoogle Scholar
8.
Pantanowitz  L , Dezube  BJ .  Kaposi sarcoma in unusual locations.   BMC Cancer. 2008;8(1):190. doi:10.1186/1471-2407-8-190PubMedGoogle ScholarCrossref
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