Epithelioid sarcoma, first described by Enzinger in 1970,
1classically presents in young adults and usually arises in the distal extremities. The proximal-type variant, first described in 1997 as a rare aggressive form of sarcoma, usually arises more proximally.
- Enzinger F.M.
Epithelioid sarcoma: a sarcoma simulating a granuloma or carcinoma.
Cancer. 1970; 26: 1029-1041
2It carries a higher mortality rate than classical limb epithelioid sarcoma and is often resistant to multimodal treatment.
- Guillou L.
- Wadden C.
- Coindre J.M.
- Krausz T.
- Fletcher C.M.
“Proximal-type” epithelioid sarcoma, a distinctive aggressive neoplasm showing rhabdoid features.
Am J Surg Pathol. 1997; 21: 130-146
3We report the case of a 27-year old male who had a delayed diagnosis of proximal-type epithelioid sarcoma of the forearm. This was originally thought to be a necrotising soft tissue infection and was unfortunately metastatic at the time of eventual diagnosis. The clinical and histopathological features of this challenging tumour are discussed and the relevant literature is reviewed.
- Rekhi B.R.
- Gorad B.D.
- Chinoy R.F.
Clinicopathological features with outcomes of a series of conventional and proximal-type epithelioid sarcomas, diagnosed over a period of 10 years at tertiary cancer hospital in India.
Virchows Arch. 2008; 453: 141-153
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- Epithelioid sarcoma: a sarcoma simulating a granuloma or carcinoma.Cancer. 1970; 26: 1029-1041
- “Proximal-type” epithelioid sarcoma, a distinctive aggressive neoplasm showing rhabdoid features.Am J Surg Pathol. 1997; 21: 130-146
- Clinicopathological features with outcomes of a series of conventional and proximal-type epithelioid sarcomas, diagnosed over a period of 10 years at tertiary cancer hospital in India.Virchows Arch. 2008; 453: 141-153
- Epithelioid sarcoma of the hand.Clin Orthop Relat Res. 2005; 431: 193-200
- Epithelioid sarcoma.Arch Pathol Lab Med. 2009; 133: 814-819
- Proximal-type epithelioid sarcoma: a clinicopathologic study of 20 cases.Mod Pathol. 2001; 14: 655-663
- Radiologic manifestations of proximal-type epithelioid sarcoma of the soft tissues.Am J Roentgenol. 2002;Oct; 179: 973-977
- Immunohistochemistry in diagnosis of soft tissue tumours.Histopathology. 2011;Jun; 58: 1001-1012
- Proximal-type epithelioid sarcoma in the groin presenting as a diagnostic dilemma.Pathol Oncol Res. 2010;Jun; 16: 181-188
- Prognostic factors for survival in patients with epithelioid sarcoma.Clin Orthop Relat Res. 2009; 467: 2939-2948
- Proximal-type epithelioid sarcoma – a rare, aggressive subtype of epithelioid sarcoma presenting as a recurrent perineal mass in a middle-aged male.World J Surg Oncol. 2007;Mar; 5: 28
Published online: January 13, 2012
Accepted: November 26, 2011
Received: July 19, 2011
© 2011 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Inc. All rights reserved.