Lipoblastoma-like tumour of the vulva: Report of three cases of a distinctive mesenchymal neoplasm of adipocytic differentiation

M. E. Lae, P. F. Pereira, Gary Keeney, A. G. Nascimento

Research output: Contribution to journalArticle

24 Citations (Scopus)

Abstract

Aims: We sought to delineate and describe three cases of a distinctive mesenchymal neoplasm of the vulva showing adipocytic differentiation and affecting young patients. Methods and results: In three patients between 13 and 38 years of age, the vulvar tumours had well-circumscribed borders and ranged in size from 35 to 100 mm. Histologically, they were well circumscribed and lobulated. The lobules were separated by thin fibroconnective tissue septa and were composed of slender spindle cells showing slightly eosinophilic cytoplasm with indistinct boundaries, uniform nuclei with finely granular chromatin, and no nucleoli. The cells were embedded in a richly myxoid stroma. The background in all three tumours was a 'chicken-wire', capillary vascular network resembling that seen in myxoid liposarcomas. Two tumours had scattered signet-ring-type lipoblasts and the third a large number of such lipoblasts. Clusters of mature adipocytes were entrapped in the tumours. None had mitotic figures, necrosis, or pleomorphism. The neoplastic cells stained positively for vimentin and were negative for other immunohistochemical markers. Treatment for all three tumours was enucleation alone. After follow-up of 10 years, 7 years, and 1 year, all patients are well with no evidence of disease. Conclusions: The benign behaviour of these neoplasms militates against the diagnosis of liposarcoma. We believe these are benign lesions of adipocytic differentiation akin to infantile lipoblastomas.

Original languageEnglish (US)
Pages (from-to)505-509
Number of pages5
JournalHistopathology
Volume40
Issue number6
DOIs
StatePublished - 2002

Fingerprint

Lipoblastoma
Vulva
Neoplasms
Myxoid Liposarcoma
Vulvar Neoplasms
Liposarcoma
Vimentin
Adipocytes
Chromatin
Blood Vessels
Chickens
Cytoplasm
Necrosis

Keywords

  • Adipocytic differentiation
  • Lipoblastoma
  • Liposarcoma
  • Vulva

ASJC Scopus subject areas

  • Cell Biology
  • Anatomy
  • Pathology and Forensic Medicine

Cite this

Lipoblastoma-like tumour of the vulva : Report of three cases of a distinctive mesenchymal neoplasm of adipocytic differentiation. / Lae, M. E.; Pereira, P. F.; Keeney, Gary; Nascimento, A. G.

In: Histopathology, Vol. 40, No. 6, 2002, p. 505-509.

Research output: Contribution to journalArticle

@article{8f3dd27dac15497089bc9458290abb49,
title = "Lipoblastoma-like tumour of the vulva: Report of three cases of a distinctive mesenchymal neoplasm of adipocytic differentiation",
abstract = "Aims: We sought to delineate and describe three cases of a distinctive mesenchymal neoplasm of the vulva showing adipocytic differentiation and affecting young patients. Methods and results: In three patients between 13 and 38 years of age, the vulvar tumours had well-circumscribed borders and ranged in size from 35 to 100 mm. Histologically, they were well circumscribed and lobulated. The lobules were separated by thin fibroconnective tissue septa and were composed of slender spindle cells showing slightly eosinophilic cytoplasm with indistinct boundaries, uniform nuclei with finely granular chromatin, and no nucleoli. The cells were embedded in a richly myxoid stroma. The background in all three tumours was a 'chicken-wire', capillary vascular network resembling that seen in myxoid liposarcomas. Two tumours had scattered signet-ring-type lipoblasts and the third a large number of such lipoblasts. Clusters of mature adipocytes were entrapped in the tumours. None had mitotic figures, necrosis, or pleomorphism. The neoplastic cells stained positively for vimentin and were negative for other immunohistochemical markers. Treatment for all three tumours was enucleation alone. After follow-up of 10 years, 7 years, and 1 year, all patients are well with no evidence of disease. Conclusions: The benign behaviour of these neoplasms militates against the diagnosis of liposarcoma. We believe these are benign lesions of adipocytic differentiation akin to infantile lipoblastomas.",
keywords = "Adipocytic differentiation, Lipoblastoma, Liposarcoma, Vulva",
author = "Lae, {M. E.} and Pereira, {P. F.} and Gary Keeney and Nascimento, {A. G.}",
year = "2002",
doi = "10.1046/j.1365-2559.2002.01409.x",
language = "English (US)",
volume = "40",
pages = "505--509",
journal = "Histopathology",
issn = "0309-0167",
publisher = "Wiley-Blackwell",
number = "6",

}

TY - JOUR

T1 - Lipoblastoma-like tumour of the vulva

T2 - Report of three cases of a distinctive mesenchymal neoplasm of adipocytic differentiation

AU - Lae, M. E.

AU - Pereira, P. F.

AU - Keeney, Gary

AU - Nascimento, A. G.

PY - 2002

Y1 - 2002

N2 - Aims: We sought to delineate and describe three cases of a distinctive mesenchymal neoplasm of the vulva showing adipocytic differentiation and affecting young patients. Methods and results: In three patients between 13 and 38 years of age, the vulvar tumours had well-circumscribed borders and ranged in size from 35 to 100 mm. Histologically, they were well circumscribed and lobulated. The lobules were separated by thin fibroconnective tissue septa and were composed of slender spindle cells showing slightly eosinophilic cytoplasm with indistinct boundaries, uniform nuclei with finely granular chromatin, and no nucleoli. The cells were embedded in a richly myxoid stroma. The background in all three tumours was a 'chicken-wire', capillary vascular network resembling that seen in myxoid liposarcomas. Two tumours had scattered signet-ring-type lipoblasts and the third a large number of such lipoblasts. Clusters of mature adipocytes were entrapped in the tumours. None had mitotic figures, necrosis, or pleomorphism. The neoplastic cells stained positively for vimentin and were negative for other immunohistochemical markers. Treatment for all three tumours was enucleation alone. After follow-up of 10 years, 7 years, and 1 year, all patients are well with no evidence of disease. Conclusions: The benign behaviour of these neoplasms militates against the diagnosis of liposarcoma. We believe these are benign lesions of adipocytic differentiation akin to infantile lipoblastomas.

AB - Aims: We sought to delineate and describe three cases of a distinctive mesenchymal neoplasm of the vulva showing adipocytic differentiation and affecting young patients. Methods and results: In three patients between 13 and 38 years of age, the vulvar tumours had well-circumscribed borders and ranged in size from 35 to 100 mm. Histologically, they were well circumscribed and lobulated. The lobules were separated by thin fibroconnective tissue septa and were composed of slender spindle cells showing slightly eosinophilic cytoplasm with indistinct boundaries, uniform nuclei with finely granular chromatin, and no nucleoli. The cells were embedded in a richly myxoid stroma. The background in all three tumours was a 'chicken-wire', capillary vascular network resembling that seen in myxoid liposarcomas. Two tumours had scattered signet-ring-type lipoblasts and the third a large number of such lipoblasts. Clusters of mature adipocytes were entrapped in the tumours. None had mitotic figures, necrosis, or pleomorphism. The neoplastic cells stained positively for vimentin and were negative for other immunohistochemical markers. Treatment for all three tumours was enucleation alone. After follow-up of 10 years, 7 years, and 1 year, all patients are well with no evidence of disease. Conclusions: The benign behaviour of these neoplasms militates against the diagnosis of liposarcoma. We believe these are benign lesions of adipocytic differentiation akin to infantile lipoblastomas.

KW - Adipocytic differentiation

KW - Lipoblastoma

KW - Liposarcoma

KW - Vulva

UR - http://www.scopus.com/inward/record.url?scp=0036278424&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0036278424&partnerID=8YFLogxK

U2 - 10.1046/j.1365-2559.2002.01409.x

DO - 10.1046/j.1365-2559.2002.01409.x

M3 - Article

C2 - 12047760

AN - SCOPUS:0036278424

VL - 40

SP - 505

EP - 509

JO - Histopathology

JF - Histopathology

SN - 0309-0167

IS - 6

ER -