Epithelioid sarcoma. A clinical and pathologic study of nine cases

Our plain-English summary of Epithelioid sarcoma. A clinical and pathologic study of nine cases, by H. Santiago, L.K. Feinerman, R. Lattes (Human Pathology, 1972).

The study

• Authors: H. Santiago, L.K. Feinerman, R. Lattes
• Journal: Human Pathology
• Published: 1972;3(1):133-47
• Cited by: 88 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

Abstract

This is a report of nine cases of epitheloid sarcoma, a term introduced recently to designate a special variant of malignant soft tissue tumor generally involving tendons, aponeuroses, and more superficial tissues of the extremities. The histologic features of this tumor, which consists of a mixture of polygonal epithelioid cells and elongated fibroblastic cells with necrotizing foci, have often misled the pathologist into diagnoses of necrotizing granuloma, malignant melanoma, angiosarcoma, or metastatic carcinoma. The tumor is definitely malignant, is difficult to eradicate by local surgical excision, and often metastasizes to lymph nodes and via the blood. The histogenesis of this tumor is still controversial, but some light and electron microscopic features suggest a possible synovial origin.

PubMed has no abstract for this study. This is the authors' abstract, copied from the article.

In plain English

This article is not about asbestos. It reports nine cases of epithelioid sarcoma, a malignant soft tissue tumor that usually involves the limbs. The cases came from pathology consultation material and from patients treated at the Columbia Presbyterian Medical Center in New York. Mesothelioma appears only as one of the diagnoses suggested for some of these cases before they were recognized as epithelioid sarcoma. The patients were 21 to 64 years old at biopsy, with a median age of 33. Seven were male and two female. Five tumors were on the lower limb and four on the upper limb. Seven patients had amputations, four of them after local excision was followed by recurrence. Six patients had at least one local recurrence. Of four patients followed for more than five years, two died of metastatic disease, one was alive with metastases and one was alive and well. Three patients had no significant follow-up. The authors concluded that pathologists must recognize this tumor as a highly malignant sarcoma needing prompt radical treatment, and must not mistake it for carcinoma, melanoma or granulomatous lesions.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: case series (clinical and pathologic study of nine cases), with two case histories presented in detail
• Who was studied: Nine patients with epithelioid sarcoma, from consultation material sent to the Division of Surgical Pathology, College of Physicians and Surgeons, and from patients treated at the Columbia Presbyterian Medical Center. They were aged 21 to 64 years at biopsy (median 33); seven were male and two female.
• Main results:
  • Five tumors were on the lower limb (leg 2, knee 1, heel 1, gluteal region 1) and four on the upper limb (forearm 2, finger 1, wrist 1).
  • Mesothelioma was among the diagnoses suggested for these nine cases before they were identified as epithelioid sarcoma. Others included synovial sarcoma, malignant melanoma, sarcoidal granuloma and angiosarcoma.
  • Seven of the nine patients had amputations of the involved limb. Four of these had local excision first, and the limb was amputated because of local recurrences.
  • Of the four patients followed for more than five years, two died of metastatic disease, one was alive with metastases and one was alive and well. Of two patients followed for over one year, one was alive and well and one died with metastatic disease.
  • Six of the nine patients had at least one local recurrence, and three of these six also had lymph node or blood-borne metastases. The main metastatic sites were the lungs and regional lymph nodes, with metastases also to the scalp, cheek and alveolar ridge.
• The authors' conclusion: The authors concluded that pathologists must learn to recognize epithelioid sarcoma as a variant of the highly malignant soft tissue sarcomas needing prompt and adequate radical treatment, and must avoid confusing it with metastatic carcinoma, malignant melanoma or inflammatory granulomatous lesions. They suggested that some light and electron microscopic features point to a possible synovial origin.
• Limitations the authors state:
  • Three of the nine patients had no significant follow-up information.
  • The tissue origin of the tumor was described as still controversial.

Topics

Medical terms in this study, explained in our Lexicon: Epithelioid, Sarcoma, Granuloma, Carcinoma, Metastasis.

Related studies

• Soft tissue sarcomas of vulva — I. Davos, M.R. Abell (1976)
• Non-mesenchymal Mimics of Sarcoma — L.A. Doyle (2015)
• Metastatic proximal epithelioid sarcoma in pleural effusion: cytopathologic findings and differential diagnosis — H. Guan, F. Haque, et al. (2013)
• A case of pleural epithelioid sarcoma of proximal type presenting as malignant pleural mesothelioma — G.A. Madsen, T.R. Rasmussen, et al. (2013)
• Synovial sarcoma of the pleura: a clinical and pathologic study of three cases — A.S. Colwell, J. D'Cunha, et al. (2002)
• Primary pulmonary epithelioid sarcoma: a case report — E. Mizutani, R. Morita, et al. (2021)

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