S5584 Fatal AIDS Cholangiopathy Secondary to Kaposi Sarcoma of Ampulla of Vater: A Rare Clinical Entity

Introduction: Kaposi sarcoma (KS) is a malignancy linked to human herpesvirus 8 (HHV-8) infection, common among immunocompromised patients. It commonly presents with cutaneous lesions, gastrointestinal involvement is rarely seen. A comprehensive review of the literature reveals that biliary tract involvement in Kaposi sarcoma is exceedingly rare, with only a limited number of cases reported to date. Here we present a very case of KS obstructing ampulla of vater. Case Description/Methods: A 26-year-old man with a history of HIV/AIDS and cutaneous Kaposi sarcoma was admitted for preseptal cellulitis. He is not adherent to HIV medications and did not receive chemotherapy for Kaposi sarcoma. Labs revealed Alkaline phosphatase 1646 unit/L, aspartate aminotransferase 134 unit/L, alanine aminotransferase 125 unit/L, total bilirubin 0.8 mg/dl, albumin 1.7 gm/dl, CD4 count is 109 with undetectable viral load. During hospitalization, the patient's bilirubin levels progressively worsened. MRCP demonstrated ill-defined hypo enhancing hepatic lesions and a 0.8 cm common bile duct with suspected ampullary stricture, consistent with AIDS cholangiopathy. HIDA scan findings supported cholestasis, most likely secondary to HIV-related cholangiopathy. ERCP revealed a large vascular mass at the ampulla, and biopsies were confirmative of KS. Given the complexity of the case, transfer to a tertiary care center was recommended for endoscopic ultrasound-guided choledochoduodenostomy to facilitate biliary drainage. His clinical condition deteriorated leading to multi-organ failure and disseminated intravascular coagulation and required intubation and pressors. Despite all the measures, the patient unfortunately passed away. Discussion: Biliary involvement in KS is usually asymptomatic but can present as cholangitis or, rarely, liver failure. KS is linked to AIDS-related cholangiopathy, marked by biliary strictures in advanced HIV, typically managed with endoscopic sphincterotomy and stenting. The mainstay of KS treatment is HAART, alone or with chemotherapy—liposomal anthracyclines like pegylated liposomal doxorubicin are first-line for advanced cases. Though KS incidence has declined with effective HIV therapy, vigilance remains crucial in untreated or non-adherent patients for timely diagnosis and management.

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Publication Details

Journal
The American Journal of Gastroenterology
Published
2025-10-01
DOI
https://doi.org/10.14309/01.ajg.0001149796.83251.53
Primary Topic
Cholangiocarcinoma and Gallbladder Cancer Studies
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article
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article

S5584 Fatal AIDS Cholangiopathy Secondary to Kaposi Sarcoma of Ampulla of Vater: A Rare Clinical Entity

Nadeem Ashraf, Charitha Karanam Ramapathy
The American Journal of Gastroenterology
Cholangiocarcinoma and Gallbladder Cancer Studies
article

S5584 Fatal AIDS Cholangiopathy Secondary to Kaposi Sarcoma of Ampulla of Vater: A Rare Clinical Entity

Nadeem Ashraf, Charitha Karanam Ramapathy
article en

Abstract

Introduction: Kaposi sarcoma (KS) is a malignancy linked to human herpesvirus 8 (HHV-8) infection, common among immunocompromised patients. It commonly presents with cutaneous lesions, gastrointestinal involvement is rarely seen. A comprehensive review of the literature reveals that biliary tract involvement in Kaposi sarcoma is exceedingly rare, with only a limited number of cases reported to date. Here we present a very case of KS obstructing ampulla of vater. Case Description/Methods: A 26-year-old man with a history of HIV/AIDS and cutaneous Kaposi sarcoma was admitted for preseptal cellulitis. He is not adherent to HIV medications and did not receive chemotherapy for Kaposi sarcoma. Labs revealed Alkaline phosphatase 1646 unit/L, aspartate aminotransferase 134 unit/L, alanine aminotransferase 125 unit/L, total bilirubin 0.8 mg/dl, albumin 1.7 gm/dl, CD4 count is 109 with undetectable viral load. During hospitalization, the patient's bilirubin levels progressively worsened. MRCP demonstrated ill-defined hypo enhancing hepatic lesions and a 0.8 cm common bile duct with suspected ampullary stricture, consistent with AIDS cholangiopathy. HIDA scan findings supported cholestasis, most likely secondary to HIV-related cholangiopathy. ERCP revealed a large vascular mass at the ampulla, and biopsies were confirmative of KS. Given the complexity of the case, transfer to a tertiary care center was recommended for endoscopic ultrasound-guided choledochoduodenostomy to facilitate biliary drainage. His clinical condition deteriorated leading to multi-organ failure and disseminated intravascular coagulation and required intubation and pressors. Despite all the measures, the patient unfortunately passed away. Discussion: Biliary involvement in KS is usually asymptomatic but can present as cholangitis or, rarely, liver failure. KS is linked to AIDS-related cholangiopathy, marked by biliary strictures in advanced HIV, typically managed with endoscopic sphincterotomy and stenting. The mainstay of KS treatment is HAART, alone or with chemotherapy—liposomal anthracyclines like pegylated liposomal doxorubicin are first-line for advanced cases. Though KS incidence has declined with effective HIV therapy, vigilance remains crucial in untreated or non-adherent patients for timely diagnosis and management.

The American Journal of GastroenterologyVol. 120(10S2)
Openalex Percentile: Top 41%
Cholangiocarcinoma and Gallbladder Cancer Studies
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