Pleomorphic dermal sarcoma: a more aggressive neoplasm than previously estimated.
BACKGROUND
Pleomorphic dermal sarcoma (PDS) is a rare neoplasm sharing pathological features with atypical fibroxanthoma, but adding tumor necrosis, invasion beyond superficial subcutis or vascular or perineural infiltration. Although its metastatic risk has been estimated to be less than 5%, its real outcome is presently uncertain because of its rarity and to the lack of homogeneous criteria used in reported cases.


METHODS
Retrospective clinicopathological study of 18 cases of PDS.


RESULTS
The lesions presented as tumors or plaques (size: 7-70 mm) on the head of elderly patients (median: 81 years), without a gender predominance. Histopathologically, they consisted of spindle cells arranged in a fascicular pattern, containing pleomorphic epithelioid and giant multinucleated cells in varying proportions, and usually exhibiting numerous mitotic figures and infiltrative tumor margins. No immunoexpression for cytokeratins, S100 protein, desmin or CD34 was observed. Necrosis and venous invasion were found in three tumors each (17%). Follow-up was available in 15 cases (median: 33 months). Three patients (20%) had local recurrences, all with incomplete primary surgical resections. Three patients (20%) developed distant metastases in the skin, regional lymph nodes and/or lungs and died from the disease.


CONCLUSIONS
Our data suggest that PDS may be a more aggressive neoplasm than previously estimated.
Department of Pathology, Hospital Universitario de Fuenlabrada, Madrid, Spain.
Juan C
JC
Tardío
Department of Pathology, Hospital Universitario Fundación Alcorcón, Madrid, Spain.
Fernando
F
Pinedo
Department of Pathology, Hospital Universitario de Getafe, Madrid, Spain.
José A
JA
Aramburu
Department of Pathology, Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain.
Dolores
D
Suárez-Massa
Department of Dermatology, Hospital Universitario Fundación Alcorcón, Madrid, Spain.
Ana
A
Pampín
Department of Dermatology, Hospital Universitario Fundación Jiménez Díaz, Madrid, Spain.
Luis
L
Requena
Department of Pathology, Hospital Universitario Fundación Jiménez Díaz, Madrid, Spain.|Department of Pathology, Hospital Universitario Infanta Sofía de San Sebastián de los Reyes, Madrid, Spain.
Carlos
C
Santonja
D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D005354:Fibrosarcoma / Q000378:metabolism* / Q000473:pathology* / Q000601:surgery; D006258:Head and Neck Neoplasms / Q000378:metabolism* / Q000473:pathology* / Q000601:surgery; D006801:Humans; D008297:Male; D009362:Neoplasm Metastasis; D012878:Skin Neoplasms / Q000378:metabolism* / Q000473:pathology* / Q000601:surgery
atypical fibroxanthoma; immunohistochemistry; pleomorphic dermal sarcoma; sarcoma; skin