Prognostic Factors for Survival in Patients with Epithelioid Sarcoma: 441 Cases from the SEER Database

Jawad, Muhammad Umar; Extein, Jason; Min, Elijah S.; Scully, Sean P.

doi:10.1007/s11999-009-0749-2

Cited by 97 publications

(86 citation statements)

References 41 publications

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“…23,24 In a recent review, positive prognostic indicators in epithelioid sarcomas included age younger than 16 years, local stage of disease, negative lymph node status and complete surgical resection. 25 The high rate of local recurrence and distant metastasis observed in epithelioid sarcoma necessitates the development of improved adjuvant systemic therapies. Given the presence of morphologic and immunophenotypic features suggesting epithelial differentiation and lack of effective therapies currently available, therapeutic strategies targeting EGFR are promising.…”

Section: Discussionmentioning

confidence: 99%

Epithelioid sarcoma expresses epidermal growth factor receptor but gene amplification and kinase domain mutations are rare

2010

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Epithelioid sarcoma is a rare, malignant, soft tissue neoplasm that can be classified into proximal, distal and fibroma-like subtypes. Regardless of subtype, epithelioid sarcoma often shows morphologic and immunophenotypic evidence of epithelial differentiation. Current therapeutic strategies include surgical resection, amputation, radiation or chemotherapy, although the overall prognosis remains poor. The epidermal growth factor receptor (EGFR) is a novel therapeutic target in carcinomas. In some carcinomas, EGFR kinase domain mutations or gene amplification may correlate with response to specific inhibitors. EGFR expression has been reported in some sarcoma types, but expression, amplification and mutations have not been studied in epithelioid sarcoma. We evaluated 15 cases of epithelioid sarcoma from 14 patients for EGFR expression using immunohistochemistry, EGFR copy number aberration using fluorescence in situ hybridization and screened for mutations in the tyrosine kinase domain of the EGFR gene using direct sequencing. In all, 14 of the 15 epithelioid sarcomas (93%) showed expression of EGFR by immunohistochemistry. A majority of the cases (n ¼ 11, 73%) showed strong (2 þ to 3 þ ) and homogeneous (475% of cells) membrane staining. No amplification or polysomy of the EGFR gene or mutations of the tyrosine kinase domain of EGFR (exons 18-21) were detected. These results imply that although EGFR is expressed in most epithelioid sarcomas regardless of subtype, gene amplification and activating mutations in the tyrosine kinase domain appear to be rare or absent. Thus, the benefit of targeted therapy against EGFR in patients with epithelioid sarcoma remains to be determined.

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Section: Discussionmentioning

confidence: 99%

Epithelioid sarcoma expresses epidermal growth factor receptor but gene amplification and kinase domain mutations are rare

2010

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“…Jawad et al studied the SEER database and found the overall survival of ES to be 68% at 5 years and 61% at 10 years. [7] Asano et al reported 30% local recurrence rate with the median interval between surgery and first local recurrence as 25 months. [8] The distant metastasis rate varies between 40% and 57%.…”

Section: Discussionmentioning

confidence: 99%

Epithelioid sarcoma: A case series of three different presentations

Jetly

Varadarajan

Naik

et al. 2017

CRCP

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Background: Epithelioid sarcoma is a rare malignancy that presents diagnostic difficulties. Cases: We report three cases of epithelioid sarcoma with different presentations and clinical course. One patient had a classicaltype epithelioid sarcoma while rest two suffered from the rarer proximal-type. All three underwent surgical excision while two of them also had postoperative adjuvant therapy. The diagnostic challenges and the final outcome has been discussed in detail. Conclusion: The diagnosis of epithelioid sarcoma needs a high index of clinical suspicion and immunohistochemistry is extremely valuable. Adequacy of index surgery affects the ultimate prognosis.

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“…The incidence of epitheloid carcoma is increasing, with a annual percentage change of 5.2% since 1973. In 2005, the incidence is estimated 0.4 case per million 11 . Epithelioid sarcoma affects mainly the subcutaneous tissues, fascia, or tendon sheaths of the extremities.…”

Section: Discussionmentioning

confidence: 99%

“…In epithelioid sarcoma, advanced age, male gender, proximal or axial location, depth, tumor size, mitotic figures, necrosis, vascular invasion, and tumor hemorrhage have been identified as adverse prognostic factors 11 . In addition, local recurrence, lymph node metastasis, and the extent of surgery were also associated with survival in some studies 6,11,12 .…”

Section: Discussionmentioning

confidence: 99%

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Endobronchial Metastasis of Epithelioid Sarcoma

Kim

Lee

et al. 2011

Tuberc Respir Dis

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Epithelioid sarcomas are rare soft tissue sarcomas with a high tumor grade and high local recurrence and metastasis rates. Although the lung is the most common site of metastasis, endobronchial metastasis hasn't been reported yet. We now report a case of epithelioid sarcoma with endobronchial metastasis. A 28-year-old man had recurrent pneumothorax and underwent wedge resection. He presented at our hospital with hemoptysis, dyspnea, and chest pain. Chest computed tomography revealed left pneumothorax, multiple lung nodules and endobronchial lesions at the right lower basal lobe. Bronchoscopy showed a hemorrhagic mass obstructing the bronchus of the right lower basal lobe. Magnetic resonance imaging revealed multiple nodular lesions in the left thigh muscles. The bronchoscopic biopsy of the endobronchial lesion and the muscle biopsy of the thigh showed the same feature epithelioid sarcoma. This is the first case report of an epithelioid sarcoma with endobronchial metastasis that was diagnosed by bronchoscopic biopsy.

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Prognostic Factors for Survival in Patients with Epithelioid Sarcoma: 441 Cases from the SEER Database

Cited by 97 publications

References 41 publications

Epithelioid sarcoma expresses epidermal growth factor receptor but gene amplification and kinase domain mutations are rare

Epithelioid sarcoma expresses epidermal growth factor receptor but gene amplification and kinase domain mutations are rare

Epithelioid sarcoma: A case series of three different presentations

Endobronchial Metastasis of Epithelioid Sarcoma

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