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Extracavitary Primary Effusion Lymphoma

MedGen UID:
1666925
•Concept ID:
C4725707
•
Neoplastic Process
Synonym: Extracavitary PEL

Definition

A rare HHV8-positive B-cell lymphoma indistinguishable from primary effusion lymphoma presenting as solid tumor mass. (WHO 2017) [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVExtracavitary Primary Effusion Lymphoma

Recent clinical studies

Etiology

KSHV-associated extracavitary primary effusion lymphoma in an HIV seronegative patient: a case report and review of the literature.
Ibrahim U, Saqib A, Mohammad F, Ding J, Hussein S, Atallah JP
Postgrad Med 2017 Apr;129(3):402-407. Epub 2017 Feb 13 doi: 10.1080/00325481.2017.1286925. PMID: 28122468
Classic and extracavitary primary effusion lymphoma in 51 HIV-infected patients from a single institution.
Guillet S, Gérard L, Meignin V, Agbalika F, Cuccini W, Denis B, Katlama C, Galicier L, Oksenhendler E
Am J Hematol 2016 Feb;91(2):233-7. doi: 10.1002/ajh.24251. PMID: 26799611
Unusual presentation of "extracavitary" primary effusion lymphoma in previously unknown HIV disease.
Medeiros BC, Maness LJ, Bauer FA, Ross JW, Kapur D
Conn Med 2000 Oct;64(10):591-4. PMID: 11100630

Diagnosis

Extracavitary Primary Effusion Lymphoma Affecting the Oral Cavity: A Rare Case Report.
López de Cáceres CVB, Sant'Ana MSP, Roman Tager EMJ, Burbano RMR, de Almeida OP, Vargas PA, Fonseca FP
Int J Surg Pathol 2024 Feb;32(1):119-132. Epub 2023 May 7 doi: 10.1177/10668969231167509. PMID: 37150962
Synchronous visceral Kaposi sarcoma and extracavitary primary effusion lymphoma in a patient with AIDS.
Bradshaw S, Oertling E, Rezigh A
BMJ Case Rep 2022 Mar 29;15(3) doi: 10.1136/bcr-2021-245306. PMID: 35351773Free PMC Article
Extracavitary primary effusion lymphoma recurring with syphilis in an HIV-infected patient.
Sohail Ahmed D, Poliquin M, Julien LA, Routy JP
BMJ Case Rep 2020 Nov 4;13(11) doi: 10.1136/bcr-2020-235204. PMID: 33148591Free PMC Article
Panniculitis-Like Presentation of Extracavitary Primary Effusion Lymphoma.
Saggini A, Di Prete M, Facchetti S, Rapisarda VM, Anemona L
Am J Dermatopathol 2020 Jun;42(6):446-451. doi: 10.1097/DAD.0000000000001539. PMID: 31592862
Classic and extracavitary primary effusion lymphoma in 51 HIV-infected patients from a single institution.
Guillet S, Gérard L, Meignin V, Agbalika F, Cuccini W, Denis B, Katlama C, Galicier L, Oksenhendler E
Am J Hematol 2016 Feb;91(2):233-7. doi: 10.1002/ajh.24251. PMID: 26799611

Therapy

Remission of an HHV8-related extracavitary primary effusion lymphoma in an HIV-positive patient during antiretroviral treatment containing dolutegravir.
Campogiani L, Cerva C, Maffongelli G, Teti E, Pupo L, Vaccarini S, Cantonetti M, Pennica A, Andreoni M, Sarmati L
AIDS Res Ther 2019 Jul 27;16(1):15. doi: 10.1186/s12981-019-0230-6. PMID: 31351487Free PMC Article
Brentuximab vedotin as frontline treatment for HIV-related extracavitary primary effusion lymphoma.
Sandoval-Sus JD, Brahim A, Khan A, Raphael B, Ansari-Lari A, Ruiz M
Int J Hematol 2019 May;109(5):622-626. Epub 2019 Jan 23 doi: 10.1007/s12185-019-02592-3. PMID: 30671911
Kaposi sarcoma-associated herpesvirus/human herpesvirus 8-associated extracavitary primary effusion lymphoma presenting as multiple lymphomatous polyposis.
Oster C, Stein T, Kitahara S, Alkan S, Huang Q
Hum Pathol 2018 Sep;79:177-183. Epub 2018 Mar 2 doi: 10.1016/j.humpath.2018.02.020. PMID: 29505765
KSHV-associated extracavitary primary effusion lymphoma in an HIV seronegative patient: a case report and review of the literature.
Ibrahim U, Saqib A, Mohammad F, Ding J, Hussein S, Atallah JP
Postgrad Med 2017 Apr;129(3):402-407. Epub 2017 Feb 13 doi: 10.1080/00325481.2017.1286925. PMID: 28122468
Classic and extracavitary primary effusion lymphoma in 51 HIV-infected patients from a single institution.
Guillet S, Gérard L, Meignin V, Agbalika F, Cuccini W, Denis B, Katlama C, Galicier L, Oksenhendler E
Am J Hematol 2016 Feb;91(2):233-7. doi: 10.1002/ajh.24251. PMID: 26799611

Prognosis

Remission of an HHV8-related extracavitary primary effusion lymphoma in an HIV-positive patient during antiretroviral treatment containing dolutegravir.
Campogiani L, Cerva C, Maffongelli G, Teti E, Pupo L, Vaccarini S, Cantonetti M, Pennica A, Andreoni M, Sarmati L
AIDS Res Ther 2019 Jul 27;16(1):15. doi: 10.1186/s12981-019-0230-6. PMID: 31351487Free PMC Article
Brentuximab vedotin as frontline treatment for HIV-related extracavitary primary effusion lymphoma.
Sandoval-Sus JD, Brahim A, Khan A, Raphael B, Ansari-Lari A, Ruiz M
Int J Hematol 2019 May;109(5):622-626. Epub 2019 Jan 23 doi: 10.1007/s12185-019-02592-3. PMID: 30671911
Multiple remissions of extracavitary primary effusion lymphoma treated with a single cycle of liposomal doxorubicin in a patient infected with HIV.
Chen J, Mehraj V, Szabo J, Routy B, Michel RP, Routy JP
Curr Oncol 2018 Dec;25(6):e592-e596. Epub 2018 Dec 1 doi: 10.3747/co.25.4119. PMID: 30607128Free PMC Article
Kaposi sarcoma-associated herpesvirus/human herpesvirus 8-associated extracavitary primary effusion lymphoma presenting as multiple lymphomatous polyposis.
Oster C, Stein T, Kitahara S, Alkan S, Huang Q
Hum Pathol 2018 Sep;79:177-183. Epub 2018 Mar 2 doi: 10.1016/j.humpath.2018.02.020. PMID: 29505765
Classic and extracavitary primary effusion lymphoma in 51 HIV-infected patients from a single institution.
Guillet S, Gérard L, Meignin V, Agbalika F, Cuccini W, Denis B, Katlama C, Galicier L, Oksenhendler E
Am J Hematol 2016 Feb;91(2):233-7. doi: 10.1002/ajh.24251. PMID: 26799611

Clinical prediction guides

Classic and extracavitary primary effusion lymphoma in 51 HIV-infected patients from a single institution.
Guillet S, Gérard L, Meignin V, Agbalika F, Cuccini W, Denis B, Katlama C, Galicier L, Oksenhendler E
Am J Hematol 2016 Feb;91(2):233-7. doi: 10.1002/ajh.24251. PMID: 26799611

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