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Epithelioid rhabdomyosarcoma: a clinicopathological study of seven additional cases supporting a distinctive variant with aggressive biological behaviour

Yu, Lin1,2; Lao, I. Weng1,2; Wang, Jian1,2

Pathology - Journal of the RCPA: December 2015 - Volume 47 - Issue 7 - p 667–672
doi: 10.1097/PAT.0000000000000321

Summary: We present our experience with seven cases of epithelioid rhabdomyosarcoma (RMS) to further characterise its clinicopathological features. There were five males and two females with ages ranging from 19 to 84 years (mean 56 years). Four tumours occurred in the somatic soft tissue, two in organs and one in the bone. The mean tumour size was 10.7 cm (range 3.5–15 cm). Histologically, six tumours were characterised by sheet-like growth of uniform epithelioid cells with large vesicular nuclei, prominent nucleoli, high mitotic activity and moderate to abundant amphophilic-to-eosinophilic cytoplasm. One tumour was composed of dyscohesive cells with rhabdoid appearance embedded in a myxoid matrix. Features suggestive of rhabdomyoblastic differentiation were absent. However, immunohistochemical study revealed skeletal muscle differentiation in all cases. Of note, focal expression of epithelial markers with co-expression of neuroendocrine markers was noted in five and three cases, respectively. Of six patients with follow-up, one experienced local recurrence and three developed metastases. To date, three patients have died of disease within 14 months. This study further demonstrates that epithelioid RMS represents a distinct variant of RMS with an aggressive behaviour. It may be misdiagnosed as poorly differentiated neuroendocrine carcinoma due to co-expression of epithelial and neuroendocrine markers.

1Department of Pathology, Fudan University Shanghai Cancer Center, Fudan University

2Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, China

Address for correspondence: Jian Wang, MD, PhD, Department of Pathology, Fudan University Shanghai Cancer Center, 270 Dong An Street, Shanghai 200032, China. E-mail:

Received 14 January, 2015

Revised 2 June, 2015

Accepted 17 June, 2015

© 2015 Royal College of Pathologists of Australasia
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