• Archives of Craniofacial Surgery
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• v.21 no.1
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• pp.41-44
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• 2020

In general, patients with neurofibromatosis type I have a higher risk than those with other types of neurofibromatosis of developing soft-tissue sarcomas related to the nervous system. We here present a 42-year-old man with neurofibromatosis type I who developed a protruding mass over only 2 weeks. The histopathological diagnosis was epithelioid sarcoma. Epithelioid sarcomas are rare and, to the best of our knowledge, no epithelioid sarcomas have been reported in patients with neurofibromatosis type I. Radical excision of the primary lesion was performed and postoperative radiotherapy and chemotherapy administered, as is recommended for epithelioid sarcoma. Our case emphasizes that patients with neurofibromatosis type I may develop malignant tumors.

• The Journal of the Korean bone and joint tumor society
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• v.14 no.2
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• pp.172-177
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• 2008

The epithelioid sarcoma is a rare high grade soft tissue sarcoma that affects young usually male, adults or adolescents. The most common localization is the hand and the forearm, followed by the leg and the foot. In most cases, the sarcoma grows slowly, involves the dermis, subcutis, or deeper soft tissues in the distal extremities. Epithelioid sarcoma is a slowly growing tumor with a high propensity for local recurrences and lymph node metastases. This neoplasm is likely to be confused with a variety of benign and malignant conditions. The treatment consists of wide surgical excision, chemotherapy and radiotherapy. We report the cases of 16-year-old girl with an epithelioid sarcoma on the lower extremity.

• The Korean Journal of Cytopathology
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• v.15 no.1
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• pp.65-69
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• 2004

Epithelioid sarcoma is a malignant soft tissue neoplasm with an uncertain histogenesis. We report the imprint cytologic features of epithelioid sarcoma in the left shoulder of a 29-year-old male patient. Imprint cytologic findings showed dissociated and loose aggregates of anaplastic epithelioid cells on the necrotic, bloody, and inflammatory background. Tumor cells were round to polygonal shaped. Tumor cells had vesicular nuclei with abundant cytoplasm. The nuclei were irregular in shape and often eccentrically located. Some tumor cells were oval to spindle shaped. Binucleated and multinucleated cells were found. Intracytoplasmic vacuoles were present. On immunohistochemical stain, the tumor cells were positive for epithelial membrane antigen, vimentin, and CD34.

• Journal of Korean Foot and Ankle Society
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• v.9 no.2
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• pp.234-238
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• 2005

Epithelioid sarcoma is a rare tumor which is usually presented with a nontender nodule on a distal extremity. It is sometimes confused with granulomatous process or chronic inflammation. We report of a case of epithelioid sarcoma on a foot of an adult male, which progressed rapidly.

• The Korean Journal of Cytopathology
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• v.7 no.1
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• pp.84-87
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• 1996

Cytologic features of the fine needle aspiration of metastatic epithelioid sarcoma are presented. The patient was a 48 year-old male who had been sufferring from recurrent and metastatic tumors of the right upper extremity for 6 years. The aspirate from the axillary mass cytologically consisted of isolated large polygonal cells with abundant, cyanophilic, slightly granular cytoplasm. Eccentrically located large round nuclei showed finely dispersed chromatin, small nucleoli, occasional convolution and folding of nuclear membrane, and frequent binucleation. Although both histologic and cytologic similarity of epithelioid sarcoma to squamous cell carcinoma is well known, findings of cyanophilic granular cytoplasm, convoluted nuclei, and binucleation appeared to be the differential points.

• The Journal of the Korean bone and joint tumor society
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• v.7 no.2
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• pp.64-67
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• 2001

Epithelioid sarcoma has been well characterized as a distincitve entity. In the classic form, it occurs in the distal extremities of young adults. However, in the proximal form, it was found mostly in the pelvis, perineum, and genital tract of young to middle-aged adults. Morphologically, the tumor cells had promient epithelioid or rhabdoid features with marked cytologic atypia, and grew in a multinodular pattern. Immunohistochemically, cytokeratin, epithelial membrane antigen, and vimentin were positive in the tumor cells. This reports dealt with 32-year-old male lesion on his perineum. The tumor shows typical features of proximal type epithelioid sarcoma. We also discussed pathological differential diagnosis and prognosis on this particular disease.

• Tuberculosis and Respiratory Diseases
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• v.70 no.5
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• pp.423-427
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• 2011

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.

• Annals of Hepato-Biliary-Pancreatic Surgery
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• v.27 no.4
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• pp.329-341
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• 2023

Vascular tumors of the liver are mesenchymal lesions from endothelial cells. They range from common benign lesions such as haemangioma, intermediate tumors like Kaposi sarcoma, and perivascular epithelioid cell tumor to malignant tumors such as hepatic epithelioid hemangioendothelioma and hepatic angiosarcoma in adults. Pediatric vascular tumors of the liver also include benign, locally aggressive, borderline, and malignant masses with haemangiomas being the most common benign tumors and epithelioid hemangioendothelioma being an uncommon pediatric malignancy. The list of these lesions is completed by nodular regenerative hyperplasia, solitary fibrous tumour, and hepatic small vessel neoplasms (HSVN). Some of these tumors are uncommon and rare. This review article aimed to enumerate hepatic vascular tumors along with their imaging, histopathology, molecular findings for accurate diagnosis that can result in better management.

• The Journal of the Korean bone and joint tumor society
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• v.12 no.1
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• pp.30-36
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• 2006

Purpose: Epitheliod sarcoma is an obscure clinical entity. This study analyzes the correlation between the clinical course and the AJCC stage, presence of residual tumor and ezrin-expression. Materials and Methods: Twenty-three cases of epithelioid sarcoma were eligible. All the cases had operation. Fifteen cases had systemic chemotherapy and 6 cases had adjuvant radiotherapy. Immumohistochemical analysis was done for 15 cases. Analyzed factors were initial stage, adjuvant treatment, local recurrence, residual tumor immumohistochemical results and surgical margin. Results: The event free survival rate of 15 stage II, III cases was 47.4% at 129 months. The actual survival rate of 8 stage IV cases was 37.5% at 80 months. The presence of residual tumor tissue on re-excision specimen showed statistical significance on event free survival rate(P=0.03). Adjuvant therapy showed no impact on outcome. The stage IV and locally recurrent cases had a positive relation with Ezrin-positivity. Conclusion: Residual tumor showed correlation in the outcome of epitheliod sarcoma. Chemotherapy and radiation therapy did not affect the outcome. Further case collection and analysis is needed for the significance between Ezrin expression and clinical behavior.

• Journal of the Korean Society of Radiology
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• v.85 no.1
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• pp.240-246
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• 2024

Epithelioid angiosarcoma is a rare variant of angiosarcoma characterized by an epithelioid morphology that mimics carcinoma. Therefore, multicentric epithelioid angiosarcoma is easily misdiagnosed as bone metastasis from carcinoma and has an aggressive clinical course. Here, we present a rare case of a 61-year-old male with multicentric epithelioid angiosarcoma of the bone. Plain radiography, CT, and MRI revealed multiple osteolytic lesions in both femurs; some lesions showed soft tissue extension with cortical bone destruction. Interestingly, PET-CT revealed that the lesions were only distributed along the bones of the lower extremities, including the pelvic bones, femurs, and tibiae. Despite histological analysis initially suggesting metastatic carcinoma, after additional immunohistological staining, including that for vascular markers (CD31 and ERG), the final diagnosis was epithelioid angiosarcoma. A better understanding of the clinicoradiological features of this disease may help eliminate diagnostic confusion and provide better management.