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Vulvar Lipoblastoma-Like Tumor

MedGen UID:
1793809
•Concept ID:
C5557368
•
Neoplastic Process
Synonym: Vulvar Lipoblastoma-Like Neoplasm

Definition

A benign, well-defined, lobulated, myxoid or gelatinous tumor that arises from the vulva. It shares morphological features with lipoblastoma, myxoid liposarcoma, and spindle cell lipoma. It is characterized by a lobulated growth and is composed of mature adipocytes, lipoblasts, and spindle cells without atypia in a myxoid matrix. DDIT3 and PLAG1 gene rearrangements seen in lipoblastoma and myxoid liposarcoma are not present in this tumor. It can recur infrequently and there are no reports of metastasis. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVVulvar Lipoblastoma-Like Tumor

Recent clinical studies

Etiology

Update on Mesenchymal Lesions of the Lower Female Genital Tract.
Croce S, Perret R, Le Loarer F
Surg Pathol Clin 2022 Jun;15(2):341-367. Epub 2022 May 20 doi: 10.1016/j.path.2022.02.009. PMID: 35715165
A contemporary review of myxoid adipocytic tumors.
Creytens D
Semin Diagn Pathol 2019 Mar;36(2):129-141. Epub 2019 Feb 28 doi: 10.1053/j.semdp.2019.02.008. PMID: 30853315
Lipoblastoma-like tumour of the vulva: report of three cases of a distinctive mesenchymal neoplasm of adipocytic differentiation.
Lae ME, Pereira PF, Keeney GL, Nascimento AG
Histopathology 2002 Jun;40(6):505-9. doi: 10.1046/j.1365-2559.2002.01409.x. PMID: 12047760

Diagnosis

Update on Mesenchymal Lesions of the Lower Female Genital Tract.
Croce S, Perret R, Le Loarer F
Surg Pathol Clin 2022 Jun;15(2):341-367. Epub 2022 May 20 doi: 10.1016/j.path.2022.02.009. PMID: 35715165
A contemporary review of myxoid adipocytic tumors.
Creytens D
Semin Diagn Pathol 2019 Mar;36(2):129-141. Epub 2019 Feb 28 doi: 10.1053/j.semdp.2019.02.008. PMID: 30853315
Lipoblastoma-like tumor of the vulva: further characterization in 8 new cases.
Mirkovic J, Fletcher CD
Am J Surg Pathol 2015 Sep;39(9):1290-5. doi: 10.1097/PAS.0000000000000448. PMID: 25929353
Lipoblastoma-like tumour of the vulva: report of three cases of a distinctive mesenchymal neoplasm of adipocytic differentiation.
Lae ME, Pereira PF, Keeney GL, Nascimento AG
Histopathology 2002 Jun;40(6):505-9. doi: 10.1046/j.1365-2559.2002.01409.x. PMID: 12047760

Prognosis

Metastatic lipoblastoma-like tumour.
Chia MRH, Windsor M
ANZ J Surg 2023 Mar;93(3):689-691. Epub 2022 Jul 1 doi: 10.1111/ans.17884. PMID: 35775445
Lipoblastoma-like tumour of the vulva: report of three cases of a distinctive mesenchymal neoplasm of adipocytic differentiation.
Lae ME, Pereira PF, Keeney GL, Nascimento AG
Histopathology 2002 Jun;40(6):505-9. doi: 10.1046/j.1365-2559.2002.01409.x. PMID: 12047760

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