Abstract:Epithelioid sarcoma, first described by Enzinger in 1970, is a rare soft-tissue sarcoma typically presenting as a subcutaneous or deep dermal mass in distal portions of the extremities of adolescents and young adults. In 1997, Guillou et al. described a different type of epithelioid sarcoma, called proximal-type epithelioid sarcoma, which is found mostly in the pelvic and perineal regions and genital tracts of young to middle-aged adults. It is characterized by a proliferation of epithelioid-like cells with rh… Show more
“…A staging CT scan revealed multiple metastases in the lungs and brain. The tumour exhibited an aggressive clinical course, with rapid distant metastasis and death nine months after diagnosis [ 16 ]. Stang et al reported a case of an 11-year-old girl who presented with a month-old swelling over the distal phalanx of the left middle finger.…”
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.
“…A staging CT scan revealed multiple metastases in the lungs and brain. The tumour exhibited an aggressive clinical course, with rapid distant metastasis and death nine months after diagnosis [ 16 ]. Stang et al reported a case of an 11-year-old girl who presented with a month-old swelling over the distal phalanx of the left middle finger.…”
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.
“…It was first described by Enzinger in 1970, typically presenting as a subcutaneous nodule or deep dermal mass in the distal extrimities. 2 Proximal variant was first described in 1997 by Guillou et al 5 Incidence is 0.04/million/yearin 2005 with male: female ratio being 1.8:1,with females having better prognosis.…”
Epitheloid sarcoma is a rare soft tissue sarcoma classified under tumor of uncertain histogenesis. Incidence being 0.4/million/year, it is divided into proximal variant-where in the proximal parts of the body are more involved and distal variant which is commoner and less aggressive of the two predominantly involving distal parts of upper limb followed by distal lower extremities and proximal upper limb. It has a male preponderance (1.8:1), although females have better prognosis comparatively, also it has a tendency of lymphatic spread with 13% metastasis. Recurrence of 77% is reported. Definitive diagnosis is made only on tissue biopsy. Most common variant is pseudo granulomatous type. This case report is based on a case which presented with a single right anterior shoulder swelling and was found to be of epitheloid sarcoma on biopsy. A 60 years old female patient came to OPD of D. Y. Patil Medical College, Pune, with complaints of swelling over her right shoulder since 1 month, initially small and gradually increasing in size of swelling. No history of pain and loss of appetite or weight. On examination, a 4 3 cms firm to hard swelling present over anterior part of right shoulder. It was observed to be non-tender, well defined and mobile. Patient has been investigated with x-ray, FNAC, and ultrasound of the swelling. FNAC was significant other giant cell lesion of soft tissue hence taken up for excision biopsy which showed epitheloid sarcoma on histopathology with faint focal positivity for pancytokeratin and EMA and negative for LCA, CK-3 and S-100.
“…[9] Although the literature reports an older age group in affected patients, our patient was just 17 years of age at presentation. [10] It typically shows large cells with prominent nucleoli, marked cytological atypia and intracytoplasmic hyaline inclusions. [11] These together impart a "RHABDOID" appearance to this variant.…”
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.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.