Epithelioid sarcoma

Epithelioid sarcoma is a rare soft tissue tumour, which was first described in 1970. The tumour occurs mostly in young adults and is characterized by multiple recurrences and late metastases. Two patients with epithelioid sarcoma are presented. Clinical and histological features and treatment are discussed.     

头部上皮样肉瘤

报告1例头部上皮样肉瘤。患者女，30岁。右侧颞部结节11年，溃疡1个月。皮肤科检查：右侧颞部见一2.5cm×2.5cm大、深约1cm的溃疡，中央表面结深黑色痂，周围有脓性分泌物。皮损组织病理检查：真皮中下部胶原间有结节状和交错排列的上皮样细胞团块，可见较多核分裂相，部分团块有坏死和裂隙样结构形成、间质中有许多淋巴细胞及少量细胞浸润。免疫组化染色结果示肿瘤细胞角蛋白（CK）、上质膜抗原（EMA）、波形蛋白（vim）、CD34均阳性。     

上皮样肉瘤

报告1例上皮样肉瘤.患者男,19岁.右侧臀部出现结节并渐增多伴破溃4个月来诊.皮损组织病理检查显示真皮及皮下组织内有一肿瘤浸润,瘤细胞由上皮样细胞和梭形细胞构成,部分细胞有异形性,瘤团内见小的坏死灶.免疫组化染色显示肿瘤细胞表达角蛋白(CK)和波形蛋白(VIM),而不表达CD34、平滑肌肌动蛋白(SMA)、上皮膜抗原(EMA)、S-100蛋白及CD68.患者的临床表现、组织病理改变及免疫组化染色均符合上皮样肉瘤的特点.     

Epithelioid sarcoma associated with lung adenocarcinoma

Epithelioid sarcoma is an uncommon soft tissue malignancy which is often misdiagnosed as necrobiotic granuloma or chronic inflammation. Many patients have local recurrence and distant metastasis because of its infiltrating growth. We report a 49-year-old Japanese man who had an ulcer with surrounding erythema on the left forearm after he sustained a bruise on this spot. His mother and father had died of rectal and bladder carcinoma, respectively, and he had also had a seminoma previously. Therefore, there was familial accumulation of malignancies. A skin biopsy revealed polygonal, plump, or spindle-shaped epithelioid cells and lymphocytes infiltrating through the dermis. Immunohistochemical studies showed positive staining for cytokeratins, epithelial membrane antigen, CD34 and vimentin and negative staining for desmin, CD68, HHF-35, 1A4 and p53. These clinical and histological features were diagnostic of epithelioid sarcoma. CT scans detected nodules in the right lung. A lung biopsy revealed that he had well-differentiated adenocarcinoma which expressed p53. Mutations in the TP53 gene were not searched for. We selected conservative treatment because the patient did not want surgical wide resection or amputation. Therefore, radiation, thermotherapy, application of 0.5% doxorubicin hydrochloride ointment and right lower lobectomy with lymph node dissection were performed. The size of the ulcer and the tumor invasion along the fasciae of muscles were decreased, however, metastasis of the epithelioid sarcoma was detected in the lymph node of the left axilla four years after the diagnosis.     

Epithelioid sarcoma: a diagnostic challenge

Epithelioid sarcoma is an uncommon slow-growing soft tissue malignancy, associated with a high incidence of local recurrence and metastasis. We report a 26-year-old male with epithelioid sarcoma on the right palm with a long history of over seven years, which was initially misdiagnosed as cutaneous tuberculosis and epithelioid hemangioendothelioma, as a result of which the treatment was delayed. No metastasis was found in our patient. The patient was referred to the oncology centre where he underwent wide excision of the lesion followed by radiotherapy. The review of the literature including clinical and histological differential diagnosis is presented as it mimics inflammatory, benign tumors as well as other malignant conditions.     

Epithelioid sarcoma. A diagnostic problem

Twelve cases of epithelioid sarcoma have been reviewed, illustrating the diagnostic problems for which this rare tumor has become notorious and demonstrating varied rates of tumor progression. The histological features most helpful to the distinction of epithelioid sarcoma from other nodular skin lesions, including benign conditions such as rheumatoid nodule and granuloma annulare, are discussed. The ultrastructural appearances of two cases were similar to immature mesenchymal tissue.     

上皮样肉瘤1例

报告1例皮肤上皮样肉瘤,患者男,42岁,右胫前皮肤硬肿块伴疼痛12年,破溃2年,双侧腹股沟淋巴结肿大5年,皮损及淋巴结组织病理检查示上皮样细胞殿堂增生呈团块状或条索状分布,细胞民形性明显,见核丝分裂像.免疫组化检查波形蛋白( ),AE1( ).     

上皮样肉瘤

报告1例跖部上皮样肉瘤.患者男,40岁.右足跖部结节2年,溃疡4～5个月.皮肤科检查:右足跖中央一5 cm×2 cm、深约1 cm的溃疡,周边少许肉芽样组织生长.皮损组织病理检查:真皮中下部可见栅状肉芽肿样瘤细胞团块,中央有坏死表现,周边主要由上皮样细胞和梭形细胞组成,核分裂象增多.免疫组化染色显示肿瘤细胞表达细胞角蛋白(CK)、上皮膜抗原(EMA)、波形蛋白(vimentin)和CD34,而不表达CD31和S-100蛋白.患者的临床表现、组织病理改变及免疫组化染色均符合上皮样肉瘤的特点.     

Epithelioid sarcoma metastatic to the tongue: a rare entity

Epithelioid sarcoma (ES) typically arises as a firm nodule on the extremities of young men. The tumor is remarkable for diagnostic difficulties both clinically and histopathologically resulting in a high frequency of initial misdiagnosis. ES is also known to have a high rate of recurrence and high rate of metastasis predominantly to the lymph nodes, lungs, and scalp. Herein, the second case of ES with metastasis to the tongue is reported.     

Epithelioid sarcoma: a puzzling soft tissue neoplasm in a child

Epithelioid sarcoma (ES) is a rare soft tissue neoplasm that usually occurs on the upper extremity of adolescents and young adults. It rarely occurs during childhood. ES is a slowly growing tumor with a high propensity for recurrences and metastases. This neoplasm is likely to be confused with a variety of benign and malignant conditions. Recognition of ES is important. The treatment consists of wide excision. Misdiagnosis can lead to inappropriate treatment and local recurrences or metastases. We report the case of a 5-year-old girl with an ES on the right forefinger.     

Epithelioid sarcoma and isolated necrobiotic granuloma: a comparative immunocytochemical study

Epithelioid sarcoma (ES) occasionally may be confused, both clinically and histologically, with isolated necrobiotic granulomas (ING), leading to misdiagnosis and potential mismanagement of these conditions. We studied 11 cases of ES and 11 of ING (6 examples of deep granuloma annulare and 5 of rheumatoid nodule) immunohistochemically, in an attempt to determine whether they could be diagnostically separated by such means. Monoclonal antibodies to cytokeratin polypeptides (CK), epithelial membrane antigen (EMA), and leukocyte common antigen (LCA) were applied to formalin-fixed, paraffin-embedded sections in each case, using the avidin-biotin-peroxidase complex technique. All ES cases stained positively for CK, and 6 expressed EMA, while examples of ING were non-reactive for these antigens. Conversely, the large epithelioid histiocytic cells in cases of ING were immunoreactive with anti-LCA, whereas no case of ES displayed this determinant in tumor cells. In the latter lesions, reactive peritumoral inflammatory cells were LCA-positive, but were readily distinguished from neoplastic cells on morphological grounds, as well as by their negativity with anti-CK and anti-EMA. Based on these data, it is concluded that immunohistologic stains for epithelial and hematopoietic antigens are valuable in the conclusive diagnostic separation of epithelioid sarcoma and necrobiotic granulomas.     

Epithelioid sarcoma and squamous cell carcinoma arising in a burn scar

Development of a malignant tumor is a well known complication of a chronic burn scar. Most of these tumors are squamous cell carcinomas and only 28 cases of burn scar sarcomas have been reported in literature. We report the first occurrence of the combination of squamous cell carcinoma and epithelioid sarcoma arising in a burn scar.     

Epithelioid sarcoma. Enzymatic histochemical and electron microscopic evidence of histiocytic differentiation

A 63-year-old man had epithelioid sarcoma. Light microscopy showed the tumor to resemble granuloma annulare. Enzymatic histocytochemical and ultrastructural studies demonstrated the tumor's histiocytic features. Literature review of epithelioid sarcoma and granuloma annulare showed them to have a similar anatomic distribution.