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Abstract. Epithelioid angiosarcomas are extremely rare malignant tumors formed from endothelial cells. The majority of studies reporting these tumors have been concerned with the clinical and pathological aspects, with limited reporting of radiological diagnosis. The aim of the present study was to provide a reference to improve understanding of diagnosis, treatment choice and prognosis assessment of epithelioid angiosarcoma. The current study reports the case of a 44-year-old woman with epithelioid angiosarcoma located in the deep soft tissue of the lower extremities. Physical examination of the right thigh revealed a palpable hard mass and movement was clearly restricted and painful. X-ray, computed tomography (CT) scans and magnetic resonance imaging (MRI) were used to evaluate the imaging features of the tumor. Using X-ray and CT scanning, an inhomogenous tumor with osteolytic osseous destruction was observed. MRI revealed that the bordering skeletal muscles were infiltrated. The patient was treated with palliative surgery and chemotherapy, but succumbed to disease 1 year later. IntroductionAngiosarcoma accounts for ~2% of soft tissue sarcomas (1). Epithelioid angiosarcoma is a rare type of angiosarcoma that was first reported by Ng et al in 1996 (2). Epithelioid angiosarcoma occurs in various sites, including the skin, head and neck, lungs, breasts, adrenal glands, bones and deep soft tissue in the extremities (3-8). Epithelioid angioma has a male predilection and typically occurs in adults, with the highest incidence being in the seventh decade of life (4). Treatment modalities include surgical resection of the primary tumor, radiation therapy and chemotherapy (4-6). Within 2-3 years of diagnosis, ~50% of patient succumb to disease, however, 20-30% of patients are free of disease (4,9). Advanced age, increased tumor size and a retroperitoneal primary site are considered to be adverse prognostic factors (9). Although pathological examination is the gold standard of diagnosis, radiological studies may provide assistance for determining the treatment choice and assessing the prognosis (10). Magnetic resonance imaging (MRI) reveals the infiltration of the lesion, and computed tomography (CT) angiography (CTA) reveals the involvement of the arteries in the lower extremities, which determines whether or not thrombosis is present (3,4). The current study presents the imaging analysis of a patient with epithelioid angiosarcoma that occurred in the deep soft tissue of the lower extremities. Written informed consent was obtained from the patient. Case reportA 44-year-old woman presented at the Department of Radiology, The Affiliated Hospital of Xuzhou Medical College (Jiangsu, China) on September 16, 2012 with soreness in the medial side of the right thigh, which had developed over 2 months. Almost 1 month later, a mass had evolved at the painful site; however, the pain was not associated with exertion. A physical examination revealed an increased temperature on the medial side, at the mid-shaft of the femur. The ma...
Abstract. Epithelioid angiosarcomas are extremely rare malignant tumors formed from endothelial cells. The majority of studies reporting these tumors have been concerned with the clinical and pathological aspects, with limited reporting of radiological diagnosis. The aim of the present study was to provide a reference to improve understanding of diagnosis, treatment choice and prognosis assessment of epithelioid angiosarcoma. The current study reports the case of a 44-year-old woman with epithelioid angiosarcoma located in the deep soft tissue of the lower extremities. Physical examination of the right thigh revealed a palpable hard mass and movement was clearly restricted and painful. X-ray, computed tomography (CT) scans and magnetic resonance imaging (MRI) were used to evaluate the imaging features of the tumor. Using X-ray and CT scanning, an inhomogenous tumor with osteolytic osseous destruction was observed. MRI revealed that the bordering skeletal muscles were infiltrated. The patient was treated with palliative surgery and chemotherapy, but succumbed to disease 1 year later. IntroductionAngiosarcoma accounts for ~2% of soft tissue sarcomas (1). Epithelioid angiosarcoma is a rare type of angiosarcoma that was first reported by Ng et al in 1996 (2). Epithelioid angiosarcoma occurs in various sites, including the skin, head and neck, lungs, breasts, adrenal glands, bones and deep soft tissue in the extremities (3-8). Epithelioid angioma has a male predilection and typically occurs in adults, with the highest incidence being in the seventh decade of life (4). Treatment modalities include surgical resection of the primary tumor, radiation therapy and chemotherapy (4-6). Within 2-3 years of diagnosis, ~50% of patient succumb to disease, however, 20-30% of patients are free of disease (4,9). Advanced age, increased tumor size and a retroperitoneal primary site are considered to be adverse prognostic factors (9). Although pathological examination is the gold standard of diagnosis, radiological studies may provide assistance for determining the treatment choice and assessing the prognosis (10). Magnetic resonance imaging (MRI) reveals the infiltration of the lesion, and computed tomography (CT) angiography (CTA) reveals the involvement of the arteries in the lower extremities, which determines whether or not thrombosis is present (3,4). The current study presents the imaging analysis of a patient with epithelioid angiosarcoma that occurred in the deep soft tissue of the lower extremities. Written informed consent was obtained from the patient. Case reportA 44-year-old woman presented at the Department of Radiology, The Affiliated Hospital of Xuzhou Medical College (Jiangsu, China) on September 16, 2012 with soreness in the medial side of the right thigh, which had developed over 2 months. Almost 1 month later, a mass had evolved at the painful site; however, the pain was not associated with exertion. A physical examination revealed an increased temperature on the medial side, at the mid-shaft of the femur. The ma...
Rationale:Epithelioid angiosarcoma (EAS) is an extremely rare malignant disease, which accounts no more than 1% of all soft tissue sarcomas. In this article, we would report a new case of EAS with multiple bone destruction and right femoral pathological fracture, which was an even rarer manifestation of EAS.Patient concerns:In this case, a 64-year-old man with right femoral fracture was reported. He had suffered from a progressive low back pain for about 8 months, and the imaging examinations prompted a multiple bone destruction in his vertebra and lower limbs. He then got a right femoral fracture without any obvious traumatic injury, and came to our hospital.Interventions:He underwent an operation of radical resection, bone cement filling and dynamic condylar screw internal fixation. During the operation, we found that the soft tissue around the fracture had a rotten fish change, which suggested a malignant disease.Diagnoses:The postoperative pathological diagnosis reported an EAS, which is extremely rare and highly malignant.Outcomes:The patient died in 83 days after the surgery, and the survival time from the symptoms started to the end was only 11 months, which showed a rapid progress and poor prognosis of EAS.Lessons:EAS is very hard to be diagnosed by clinical manifestation or radiological examinations. As in our case, pathological analysis is the final diagnosis. The images of the patient may offer some tips for the skeletal presentation of EAS, and do more help in future study of this disease.
Rationale:Epithelioid angiosarcoma (EA) is a rare, highly invasive tumor. The histopathological features of EA are not distinct and less reported in the literature, and most of the medical records are incomplete.Patient concerns:A 61-year-old woman who came to the hospital because of pain in her right hip. This patient had had surgery for right hip tuberculosis 30 years ago.Diagnoses:The present study reports a case of primary EA of bone with aneurysmal bone cyst (ABC) that was diagnosed by 3 experienced pathologists.Interventions:The patients had undergone 2 surgeries; however, an early recurrence of the tumor was caused the death of the patient.Outcomes:Ten EA cases from other literature were reviewed in this article; all the symptoms were found in different parts of bone, and the case data were relatively complete. The primary clinical features and nonspecific histopathological morphology of the disease were summarized from the 11 cases mentioned in the literature, and the main immunohistochemistry characteristics and diagnostic traps of EA were reviewed.Lessons:Because the tumor has no characteristic diagnostic index in imaging and laboratory examination, the histopathologic features are not typical, especially in the case of obvious secondary lesions. It is easy to miss and misdiagnose. If possible, the diagnosis should be combined with immunohistochemical results.
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