Primary dedifferentiated liposarcoma of the orbit, a rare entity: Case report and review of literature

Giulio Gasparini, Andrea Torroni, Sandro Pelo, Torroni Andrea

Risultato della ricerca: Contributo in rivistaArticolo in rivistapeer review

1 Citazioni (Scopus)

Abstract

Head and neck liposarcomas are rare entities accounting for less than 5% of all liposarcomas. The primary orbital location is even rarer, with about 40 cases described in the English literature. According with the widely accepted classification of Enzinger and Weis there are 5 histologic variants of liposarcomas: well differentiated, myxoid, dedifferentiated, round cell and pleomorphic. The first two are considered low-grade and display a favourable prognosis (>90% 5-year DSS and OS), whereas the dedifferentiated, round cell, and pleomorphic are defined high-grade and burdened with poorer prognosis (5-year DSS ranging 45-73%). Dedifferentiated liposarcomas (DDL) of the head and neck region are exceedingly rare, therefore there are scattered and contrasting data regarding their clinical history, treatment modality, and prognosis. We presented a case of DDL arising in the the left orbit (fourth case of primary orbital DDL described), free from disease after 5-year follow-up. Clinical history, treatment, and characteristics of the presented case were described and discussed in the light of how reported in the literature, in the attempt to bring further insight in the nature and management of this rare pathological entity.
Lingua originaleEnglish
pagine (da-a)312-315
Numero di pagine4
RivistaSaudi Journal of Ophthalmology
Volume33
DOI
Stato di pubblicazionePubblicato - 2019

Keywords

  • Dedifferentiated liposarcoma
  • Head and neck
  • Orbit

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