Proximal-type epithelioid sarcoma: a new case report and literature review

Echchaoui, Abdelmoughit; Sadrati, Yassine; Elbir, Youssef; Elktaibi, Abderrahim; Benyachou, Malika; Mazouz, S. El; Gharib, N.; Abbassi, A.

doi:10.11604/pamj.2016.24.238.8535

Cited by 11 publications

(14 citation statements)

References 14 publications

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“…Histopathology and immunohistochemistry studies confirmed the diagnosis. Surgical resection was the only treatment, and the patient did not develop any recurrence of the lesion at 18 months of follow-up [ 15 ].…”

Section: Discussionmentioning

confidence: 99%

Epithelioid Sarcoma Presenting as Non-Healing Traumatic Ulcer: A Case Report and Review of the Literature

Mohammedali¹,

Quazi²,

Muneer³

et al. 2021

Cureus

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Epithelioid sarcoma is a rare soft tissue sarcoma. It is a slow-growing neoplasm, which usually presents as a painless mass in the extremities and typically grows along deep dermal and subcutaneous planes. In contrast to other types of sarcoma, it has a strong tendency for nodal metastasis and local metastasis adjacent to the primary lesion within the affected limb. In this article, we present a case of chronic traumatic ulcer in the upper extremity in an adolescent male that was subsequently diagnosed as epithelioid sarcoma, which is a very unusual mode of presentation of this particular tumour. The patient was treated with wide local excision and reconstruction with a free flap. Histopathological examination and immunochemistry studies confirmed the diagnosis and the patient underwent radiotherapy post-operatively as a part of the treatment regime. His post-treatment period was unremarkable, and he was put on regular surveillance to monitor the development of any signs of disease recurrence. Patients with epithelioid sarcomas often present late due to the slow-growing nature of the tumour. Unusual presentations like this will further delay the diagnosis and treatment, which will eventually worsen the prognosis. Awareness of such presentations can encourage primary care physicians to make early referrals to experts, which, in turn, may help the patients get early treatment and have a better prognosis.

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Section: Discussionmentioning

confidence: 99%

Epithelioid Sarcoma Presenting as Non-Healing Traumatic Ulcer: A Case Report and Review of the Literature

Mohammedali¹,

Quazi²,

Muneer³

et al. 2021

Cureus

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“…A greater rate of survival has also been shown to be associated with the more common of the two histological ES subtypes: the distal-type [20]. Distal ES is often seen in the extremities while proximal ES typically affects the trunk, axilla, and groin among other proximal sites [21]. Debate on whether the proximal-type is inherently worse than distal ES or whether it is due to their associations with the affected site persists [1,4].…”

Section: Discussionmentioning

confidence: 99%

Epithelioid sarcoma: half a century later

et al. 2019

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Background and objectives: Epithelioid sarcoma (ES) is an aggressive malignancy scarcely reported on due to its rarity. This study is a review of its traits and features of prognosis and survival by analyzing both the literature and a national cancer database. Methods: Data were acquired from both the Survival, Epidemiology, and End Results database and literature. 1, 5, and 10-year Disease Specific Survival rates and hazard ratios (HR) were determined. Data were split into pre-2000 (<2000) and post-2000 (>2000) groups. Overall survival, recurrence, and metastasis rates were obtained. Results: Ninety hundred and ninety eight and 992 cases of ES were identified from the database and literature, respectively. Age, anatomical site, grade, TNM staging, treatment modality and year of diagnosis were demonstrated to be independent predictors of survival. Overall 5-and 10-year survival were 60.4% and 50.2%, respectively. Overall recurrence and metastasis rates were 63.4% and 40.3%. Using cases diagnosed prior to 2000 as reference, those diagnosed after 2000 had a worse prognosis (HR: 1.55). Conclusions: We report using the largest cohort of ES to date. Despite ES's often dismal prognosis, there are factors associated with better outcomes. A worsening survival over the years warrants further investigation into this sarcoma.

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“…Proximal-type ESs are extremely rare and are notorious for high local recurrence rates and distant metastasis in up to 60% of cases (17,18). These tumors, when arising in proximal locations, have a very poor prognosis than those arising in distal locations.…”

Section: Discussionmentioning

confidence: 99%

Primary pleural epithelioid sarcoma of the proximal type: a diagnostic and therapeutic challenge

Ahmad¹,

Stanazai²,

Wright³

et al. 2019

Transl. Lung Cancer Res.

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Epithelioid sarcoma (ES) is an uncommon soft tissue neoplasm first described in 1970. It is a unique soft tissue neoplasm of adolescents and younger adults which usually presents as a subcutaneous and deep dermal mass in the distal portions of the extremities. The proximal-type variant of this rare soft tissue neoplasm was only recently reported. The proximal form typically arise in proximal extremities and in the deep parts of pelvis, perineum and genital tract. The proximal type variant has distinct histological characteristics and aggressive clinical course as compared to the distal ES. Inactivation of INI1 has been reported in both distal and proximal variants and can help to make the diagnosis. Furthermore, the proximal variant has a possible association with malignant rhabdoid neoplasm. We describe here a case of primary pleural ES of the proximal type and highlight its diagnostic and therapeutic challenges.

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Proximal-type epithelioid sarcoma: a new case report and literature review

Cited by 11 publications

References 14 publications

Epithelioid Sarcoma Presenting as Non-Healing Traumatic Ulcer: A Case Report and Review of the Literature

Epithelioid Sarcoma Presenting as Non-Healing Traumatic Ulcer: A Case Report and Review of the Literature

Epithelioid sarcoma: half a century later

Primary pleural epithelioid sarcoma of the proximal type: a diagnostic and therapeutic challenge

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