Chylous ascites in disseminated Kaposi sarcoma: an unusual manifestation as immune reconstitution inflammatory syndrome

Cipriano, Patrícia; Nabais, Inês; Melo, Nuno; Alves, Ana Rafaela

doi:10.1136/bcr-2018-228406

Cited by 5 publications

(8 citation statements)

References 21 publications

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“…Chyle contains a high concentration of triglycerides, proteins, and lymphocytes. The development of chylous effusions can lead to nutritional depletion, a decrease in immune function, and an increased susceptibility to infections, signi cantly increasing both mortality and morbidity [9]. Reported mortality rates for chylous ascites range between 40-70%, while the 90-day mortality rate for chylothorax varies from < 10-82% [7].…”

Section: Discussionmentioning

confidence: 99%

Chylothorax and Chylous Ascites in AIDS-Associated Kaposi's Sarcoma： A Case Report and Literature Review

Jiang,

Peng,

Zhang

et al. 2024

Preprint

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Background: Kaposi's sarcoma (KS) is one of the most common tumors in patients with AIDS, while the occurrence of chylous effusions in Kaposi's sarcoma patients is rare and often indicates a poor prognosis. This report presents a case of successful management of an AIDS patient with Kaposi's sarcoma who developed both chylothorax and chylous ascites, along with a review of the relevant literature. Case presentation: A 31-year-old male patient, who was recently diagnosed with HIV infection and oral Kaposi's sarcoma at our clinic center, returned two weeks post-discharge. He reported significant enlargement of the oral tumor, increased abdominal girth with accompanying abdominal distension and pain, and a dry cough over the past week. Upon readmission, CT scans indicated the presence of extensive pleural and abdominal effusions. Aspirates from both the pleural and abdominal effusions exhibited a milky chylous appearance. After eliminating other potential etiologies for the chylous effusion, we associated it with the progression of Kaposi's sarcoma. The patient's condition was ultimately successfully managed through a combination of antiretroviral therapy, systemic chemotherapy, and intermittent thoracoabdominal paracentesis for drainage. Conclusion: The emergence of chylothorax and chylous ascites should arouse suspicion of Kaposi's sarcoma, and the presence of chylous effusions in Kaposi's sarcoma patients is an indication of disease progression. Prompt diagnosis and systemic treatment are crucial for the prognosis.

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Section: Discussionmentioning

confidence: 99%

Chylothorax and Chylous Ascites in AIDS-Associated Kaposi's Sarcoma： A Case Report and Literature Review

Jiang,

Peng,

Zhang

et al. 2024

Preprint

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“…A short onset delay after ART initiation does not seem to have any predictive value, and KS-IRIS can remain a time-limited phenomenon, controlled with a limited course of early systemic chemotherapy [ 27 ] or continue to develop, despite several cycles of chemotherapy over a number of months [ 67 ].…”

Section: Clinical Presentations Of Ks-irismentioning

confidence: 99%

“…More atypical presentations of KS-IRIS have been reported, such as oral localization [ 75 ], chylous ascites and chylothorax [ 67 ]. Although rare, KS-IRIS can also be observed in children, as recently reported in a 9-year-old Guinean girl [ 76 ].…”

Section: Clinical Presentations Of Ks-irismentioning

confidence: 99%

Immune Reconstitution Inflammatory Syndrome Associated Kaposi Sarcoma

Poizot‐Martin

Brégigeon

Palich

et al. 2022

Cancers

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People living with HIV (PLWH) with advanced immunosuppression who initiate antiretroviral therapy (ART) are susceptible to the occurrence of an immune reconstitution inflammatory syndrome (IRIS). Although ART is responsible for AIDS- associated Kaposi sarcoma (KS) improvement and resolution, new onset (unmasking KS-IRIS) or sudden progression of preexisting KS (paradoxical KS-IRIS) can occur after a time delay of between a few days and 6 months after the initiation or resumption of ART, even in patients with a low degree of immunocompromise. KS-IRIS incidence varies from 2.4% to 39%, depending on study design, populations, and geographic regions. Risk factors for developing KS-IRIS include advanced KS tumor stage (T1), pre-treatment HIV viral load >5 log10 copies/mL, detectable pre-treatment plasma-KSHV, and initiation of ART alone without concurrent chemotherapy. Both paradoxical and unmasking KS-IRIS have been associated with significant morbidity and mortality, and thrombocytopenia (<100,000 platelets/mm3 at 12 weeks) has been associated with death. KS-IRIS is not to be considered as ART failure, and an ART regimen must be pursued. Systemic chemotherapy for KS in conjunction with ART is recommended and, in contrast with management of IRIS for other opportunistic infections, glucocorticoids are contra-indicated. Despite our preliminary results, the place of targeted therapies in the prevention or treatment of KS-IRIS needs further assessment.

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“…KS can also involve the thoracic duct and adjacent mediastinal structures, manifesting as chylothorax. The thoracic duct can serve as either a site of origin or a target of metastasis for KS ( 23 , 24 ). Doxorubicin is frequently the first-line therapy for systemic or extensive KS, but newer targeted therapies are showing promise in clinical trials ( 25 ).…”

Section: Intermediate (Rarely Metastasizing)mentioning

confidence: 99%

Vascular tumors of the mediastinum

Paral¹,

Krausz²

2020

Mediastinum

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Vascular tumors represent only a sliver of all tumors affecting the mediastinum, but they pose diagnostic challenges due to significant overlap among entities, ever-evolving classification schemes, and the exquisite rarity of some of the entities not only in the mediastinum but in pathology practice as a whole. Most of the vascular tumors are better known to the practice of soft tissue pathology, from which some of the knowledge of clinical behavior can be extrapolated. For example, the stratification of epithelioid hemangioendothelioma (EHE) into two biologically separate categories has effectively translated from the somatic soft tissues to the thorax. For other entities, the effective validation of soft tissue parameters is hindered by the small numbers of reported mediastinal cases. Many of the treatment modalities have also transferred over, with the key differences resting on the difficulty in achieving complete surgical resection for mediastinal tumors as opposed to those in the somatic soft tissues. Accordingly, systemic drug therapies have emerged as attractive options for some of the mediastinal vascular tumors, such as kaposiform hemangioendothelioma (KHE) and Kaposi sarcoma (KS). The categories presented herein mirror the classification scheme set forth by the 5th Edition WHO Classification of Soft Tissue and Bone Tumors. This review focuses on the biologically aggressive vascular neoplasms while limiting discussion of the benign entities to relevant comparisons in the differential diagnoses. While distinguishing among the benign entities has academic importance, in practice, the stakes are far higher for diagnosing the biologically aggressive tumors given their marked heterogeneity in clinical outcomes. Practical advice for problem areas in pathology will be reviewed alongside tumor pathobiology, including the latest in molecular diagnostics.

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Chylous ascites in disseminated Kaposi sarcoma: an unusual manifestation as immune reconstitution inflammatory syndrome

Cited by 5 publications

References 21 publications

Chylothorax and Chylous Ascites in AIDS-Associated Kaposi's Sarcoma： A Case Report and Literature Review

Chylothorax and Chylous Ascites in AIDS-Associated Kaposi's Sarcoma： A Case Report and Literature Review

Immune Reconstitution Inflammatory Syndrome Associated Kaposi Sarcoma

Vascular tumors of the mediastinum

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