Primary purely intradermal pleomorphic liposarcoma.
BACKGROUND
Pleomorphic liposarcoma (PLPS) is a high-grade pleomorphic sarcoma, containing multivacuolated lipoblasts, which usually develops during late adult life. It usually occurs in the deep soft tissues and uncommonly arises in the subcutis, the dermis representing an exceedingly rare site of occurrence.


METHODS
We describe a case of PLPS arising in the dorsal aspect of the nose of a 75-year-old woman. Preoperative duration was 9 months.


RESULTS
The lesion was intradermal well-circumscribed, dome-shaped, measuring 1.2 cm. It fulfilled the histologic criteria for inclusion in the PLPS category. The tumor cells focally expressed calretinin. A review of the literature yielded four cases documented, to which we add the present report. All the patients were adults with a mean age of 67 (range 39-95) years, and three of five cases arose on the scalp. Local recurrence occurred in one patient, but no distant metastases or disease-related deaths were observed.


CONCLUSIONS
PLPS very rarely arises in the dermis. In spite of high-grade morphology, the intradermal tumor shows a relatively favorable prognosis. Diagnostic consideration includes pleomorphic lipoma, clear cell atypical fibroxanthoma, balloon cell melanoma, and metastatic clear cell carcinoma of renal origin. Recognition of this distinctive and rare type of liposarcoma is essential to avoid under- or misdiagnosis and inappropriate treatment.
Val-Bernal
J Fernando
JF
Department of Anatomical Pathology, Marqués de Valdecilla University Hospital, Medical Faculty, University of Cantabria, Santander, Spain.
González-Vela
M Carmen
MC
Cuevas
Jorge
J
D000368:Aged; D014408:Biomarkers, Tumor / Q000032:analysis; D064032:Calbindin 2; D020405:Dermis / Q000737:chemistry / Q000473:pathology*; D005260:Female; D006801:Humans; D019394:Ki-67 Antigen / Q000032:analysis; D008080:Liposarcoma / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D009666:Nose / Q000473:pathology; D064030:S100 Calcium Binding Protein G / Q000032:analysis; D012878:Skin Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D016896:Treatment Outcome
D014408:Biomarkers, Tumor; C576764:CALB2 protein, human; D064032:Calbindin 2; D019394:Ki-67 Antigen; D064030:S100 Calcium Binding Protein G