Clear-Cell Sugar Tumors

MedGen UID:: 385284
•Concept ID:: C2350234
•: Neoplastic Process

Synonyms:

Clear Cell Sugar Tumor; Clear Cell Sugar Tumors; Clear-Cell Sugar Tumor; Sugar Tumor, Clear-Cell; Sugar Tumors, Clear-Cell; Tumor, Clear-Cell Sugar; Tumors, Clear-Cell Sugar

Definition

PEComas containing glycogen-rich clear cytoplasm and found in the lung and a wide range of extrapulmonary sites. [from MeSH]

Professional guidelines

PubMed

French recommendations for the diagnosis and management of lymphangioleiomyomatosis.

Cottin V, Blanchard E, Kerjouan M, Lazor R, Reynaud-Gaubert M, Taille C, Uzunhan Y, Wemeau L, Andrejak C, Baud D, Bonniaud P, Brillet PY, Calender A, Chalabreysse L, Court-Fortune I, Desbaillets NP, Ferretti G, Guillemot A, Hardelin L, Kambouchner M, Leclerc V, Lederlin M, Malinge MC, Mancel A, Marchand-Adam S, Maury JM, Naccache JM, Nasser M, Nunes H, Pagnoux G, Prévot G, Rousset-Jablonski C, Rouviere O, Si-Mohamed S, Touraine R, Traclet J, Turquier S, Vagnarelli S, Ahmad K; OrphaLung network
Respir Med Res 2023 Jun;83:101010. Epub 2023 Mar 24 doi: 10.1016/j.resmer.2023.101010. PMID: 37087906

Lymphangioleiomyomatosis: pathogenesis, clinical features, diagnosis, and management.

McCarthy C, Gupta N, Johnson SR, Yu JJ, McCormack FX
Lancet Respir Med 2021 Nov;9(11):1313-1327. Epub 2021 Aug 27 doi: 10.1016/S2213-2600(21)00228-9. PMID: 34461049

Update on the Diagnosis and Management of Renal Angiomyolipoma.

Flum AS, Hamoui N, Said MA, Yang XJ, Casalino DD, McGuire BB, Perry KT, Nadler RB
J Urol 2016 Apr;195(4 Pt 1):834-46. Epub 2015 Nov 21 doi: 10.1016/j.juro.2015.07.126. PMID: 26612197

See all (166)

Recent clinical studies

Therapy

Acute abdomen as an unusual presentation of hepatic PEComa. A case report.

Priola AM, Priola SM, Cataldi A, Marci V, Fava C
Tumori 2009 Jan-Feb;95(1):123-8. doi: 10.1177/030089160909500124. PMID: 19366072

See all (1)

Prognosis

Expanding the histomorphologic spectrum of TFE3-rearranged perivascular epithelioid cell tumors.

Maloney N, Giannikou K, Lefferts J, Bridge JA, Linos K
Hum Pathol 2018 Dec;82:125-130. Epub 2018 Apr 4 doi: 10.1016/j.humpath.2018.03.023. PMID: 29626599

Predicting the Behavior of Perivascular Epithelioid Cell Tumors of the Uterine Corpus.

Acosta AM, Adley BP
Arch Pathol Lab Med 2017 Mar;141(3):463-469. doi: 10.5858/arpa.2016-0092-RS. PMID: 28234575

Incidental perivascular epithelioid cell tumor in an inguinal hernia sac.

Wang T, Voogjarv H, Vajpeyi R
Pathol Res Pract 2013 Sep;209(9):593-5. Epub 2013 Jun 14 doi: 10.1016/j.prp.2013.06.003. PMID: 23870603

Acute abdomen as an unusual presentation of hepatic PEComa. A case report.

Priola AM, Priola SM, Cataldi A, Marci V, Fava C
Tumori 2009 Jan-Feb;95(1):123-8. doi: 10.1177/030089160909500124. PMID: 19366072

Clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres: a novel member of the perivascular epithelioid clear cell family of tumors with a predilection for children and young adults.

Folpe AL, Goodman ZD, Ishak KG, Paulino AF, Taboada EM, Meehan SA, Weiss SW
Am J Surg Pathol 2000 Sep;24(9):1239-46. doi: 10.1097/00000478-200009000-00007. PMID: 10976698

See all (7)