Epithelioid sarcoma

Feger, Joachim

doi:10.53347/rid-86975

Cited by 3 publications

(7 citation statements)

References 6 publications

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“…While treatment can include the use of chemotherapy, immunologics, and radiation, timely and adequate surgical resection of the primary tumor continues to be the only potentially curative therapy. 2 The role of adjuvant radiation therapy remains unclear. 18 Doxorubicin-ifosfamide combination chemotherapy has shown effectiveness in proximal-type ES and was used in our case with the patient being 16 months postinitial diagnosis.…”

Section: Discussionmentioning

confidence: 99%

“…Enzinger first described ES in 1970; previously the condition may have been misdiagnosed based on histopathologic features. 2 They represent less than 1% of soft tissue sarcomas and are made up of mesenchymal cells with epithelial differentiation, although the cell of origin remains unclear. There exists a weak predilection for males, and the tumor can present in a wide range of ages.…”

Section: Discussionmentioning

confidence: 99%

“…1 It typically affects the distal extremities and occurs twice as frequently in men as it does in women. 2 Most reported tumors occur in young males ranging in age from 20 to 40 years. 3 The diagnosis of ES can be challenging clinically and histologically, due to the resemblance to granulomatous disease, carcinomas, and synovial sarcoma, among others.…”

mentioning

confidence: 99%

“…This tumor is locally invasive and frequently metastasizes to lymph nodes (in 30% of cases), lungs, bones, and brain; less commonly, the tumor can metastasize to the scalp, gastrointestinal tract, liver, and kidneys have been described. 2,4,5 Although surgical resection can be curative, recurrence and distant spread can occur. 4 There are very rare instances of primary occurrence in the orbit and periocular region.…”

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confidence: 99%

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Metastatic Epithelioid Sarcoma of the Eyelid in a 47-Year-Old Man: A Case Report

Han¹,

Dermarkarian²,

Camacho³

et al. 2023

Ophthalmic Plastic &Amp; Reconstructive Surgery

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Epithelioid sarcoma is a rare soft tissue neoplasm of uncertain differentiation that typically affects the distal extremities. Primary orbital epithelioid sarcoma is rare, and no reports exist characterizing metastases of this tumor to the orbit and ocular adnexa. In this article, the authors describe a rare case of eyelid metastasis in a 47-year-old man with epithelioid sarcoma of the right fibula diagnosed 16 months earlier who was otherwise doing well on the adjuvant tazemetostat therapy. In addition, the authors perform a retrospective review of cases of primary orbital epithelioid sarcoma reported in the literature, of which 4 patients responded favorably to surgical excision whereas 2 others eventually died as a direct result of their disease.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

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Metastatic Epithelioid Sarcoma of the Eyelid in a 47-Year-Old Man: A Case Report

Han¹,

Dermarkarian²,

Camacho³

et al. 2023

Ophthalmic Plastic &Amp; Reconstructive Surgery

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“…This tumor is highly aggressive and responds poorly to standard anti-cancer treatments [6]. Like all localized soft tissue sarcomas, surgical resection with an R0 margin is the gold standard treatment [7]. Adjuvant radiotherapy can be indicated depending on prognostic factors [7].…”

Section: Introductionmentioning

confidence: 99%

Neck Epithelioid Sarcoma at an Unusual Location Mimicking Lymph Node Metastases of Nasopharyngeal Carcinoma: A Case Report

El Ouardani,

Chibani,

Rezzoug

et al. 2024

Cureus

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Epithelioid sarcoma (ES) is an uncommon soft tissue sarcoma. It is usually located in the extremities and exceptionally in the neck. Its diagnosis constitutes a real challenge which is based on histology and immunohistochemistry staining that must be interpreted with caution given the anatomopathological similarities to other tumors. In this article, we report a case of a 37-year-old man admitted for a locally advanced ES of the neck. There were suspicions of lymph node metastases of nasopharyngeal carcinoma at the first pathological examination. The patient received palliative chemotherapy and was referred to the supportive care department. Through this case, we will discuss the clinical and anatomopathological characteristics and therapeutic options of this extremely rare tumor which poses a diagnostic challenge.

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Metastatic proximal-type epithelioid sarcoma presenting as a chest lesion with a pathological fracture of the humeral neck

Kumara

Udupihille

Prabodini

et al. 2022

Int J Case Rep Images

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Introduction: Proximal-type epithelioid sarcoma is a rare soft tissue neoplasm which occurs mostly in young males and is much rarer than classic distal sub type. A young male, with a known diagnosis of neurofibromatosis type 1 (NF1) presenting with metastatic proximal-type epithelioid sarcoma is reported. Case Report: A 30-year-old male with known NF1, hypothyroidism, epilepsy, presented with a right shoulder pain of two weeks duration. Plain radiographs revealed an expansile lytic lesion, an aggressive periosteal reaction in right humeral head with an underlying pathological fracture. A well-defined extrapulmonary mass with a lobulated margin in upper zone of right hemithorax with inferior rib erosion of 3rd rib and the 3rd intercostal space widening were also observed. Contrast-enhanced computed tomography (CECT) chest and abdomen confirmed peripherally located poorly enhancing soft tissue mass with lobulated margins arising from posterior chest wall along with a subdiaphragmatic deposit. Multiple lytic lesions in right head of humerus with an underlying pathological fracture of surgical neck, T2, T6, and L3 vertebral bodies, and pelvic bones were observed too. Diagnosis of malignant peripheral nerve sheath tumor with metastases was made and ultrasound-guided biopsy of chest wall lesion was performed. Histology and immunohistochemistry (IHC) revealed a proximal type epithelioid sarcoma. The patient was managed with palliative surgery and oncological follow-up. Conclusion: Presentation of proximal-type epithelioid sarcoma with disseminated metastases in a patient with NF1 masquerading malignant peripheral nerve sheath tumor (MPNST) and the challenges of diagnosis on clinical, radiological, and histopathology as well as IHC perspectives of this rare disease entity is highlighted.

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Epithelioid sarcoma

Cited by 3 publications

References 6 publications

Metastatic Epithelioid Sarcoma of the Eyelid in a 47-Year-Old Man: A Case Report

Metastatic Epithelioid Sarcoma of the Eyelid in a 47-Year-Old Man: A Case Report

Neck Epithelioid Sarcoma at an Unusual Location Mimicking Lymph Node Metastases of Nasopharyngeal Carcinoma: A Case Report

Metastatic proximal-type epithelioid sarcoma presenting as a chest lesion with a pathological fracture of the humeral neck

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