Summary
Extraskeletal osteosarcoma (EOS) is a high grade soft tissue tumour characterised
by the production of malignant osteoid, without attachment/involvement of underlying
bone/periosteum. Rarely, EOS presents as a cutaneous tumour. The clinical behaviour
of primary cutaneous EOS (PC-EOS) remains incompletely characterised. Herein we present
the largest case series of PC-EOS reported to date. Sixteen PC-EOS cases from the
archives/consultation files were retrieved (male:female 1:1; age 31–96 years, mean
age 66 years). The tumours measured 1–10 cm (mean 3.2 cm) and were located on the
lower extremity (7), head (6), upper extremity (2), and trunk (1). They consisted
of pleomorphic, spindled-to-epithelioid cells, with fascicular, nodular, or sheet-like
growth patterns and foci of malignant osteoid. Immunohistochemistry did not reveal
specific lines of differentiation, and there was no evidence of other tumour types.
A literature review was conducted to identify all well characterised cases of PC-EOS.
A combined analysis of present and past cases was performed to determine overall trends
in clinical characteristics and outcomes. The mean follow-up period was 23.9 months,
during which 67.5% of patients experienced progression-free survival and 18% of patients
died of disease. Rates of local recurrence and metastasis were 10% and 25%, respectively,
approximately double past estimates. These data suggest that the prognosis of PC-EOS
is less favourable than previously thought. The differential diagnosis includes benign
entities (e.g., ossifying pyogenic granuloma) and malignant neoplasms with heterologous
osteosarcomatous differentiation (e.g., carcinosarcoma, transdifferentiated melanoma).
Wide excision remains the standard of care, and the role of chemotherapy and radiation
remains inconclusive. Recognition of this rare entity can facilitate prompt diagnosis
and appropriate treatment.
Key words
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Article info
Publication history
Published online: December 23, 2022
Accepted:
October 11,
2022
Received in revised form:
October 3,
2022
Received:
August 31,
2022
Identification
Copyright
© 2022 Royal College of Pathologists of Australasia. Published by Elsevier B.V. All rights reserved.
