Abstract
PurposeTo evaluate the characteristics of highly malignant soft tissue sarcoma of the extremity with a delayed diagnosis.Materials and methodsThe clinical and radiological characteristics of 18 cases of highly malignant soft tissue sarcomas of the extremity with a delayed diagnosis were determined.ResultsTen men and eight women of mean age 44.8 years (range, 15-79 years) were included in this study. Seven cases of synovial sarcoma, three cases each of alveolar soft part sarcoma and malignant fibrous histiocytoma, two cases each of highly malignant leiomyosarcoma and myxofibrosarcoma, and one case of clear cell sarcoma were enrolled. Times from tumor detection to diagnosis ranged from 1 to 3 years in most cases; three of the seven synovial sarcoma cases took more than 10 years to diagnose. Of the seven cases of synovial sarcoma, five cases of small, superficial located masses were simply excised without a pre-surgical biopsy. Three cases of alveolar soft part sarcoma showed characteristic T1- and T2-weighted high signal intensities with signal voids in MR images. In addition, one synovial sarcoma patient and one alveolar soft part sarcoma patient showed evidence of calcification on plain radiographs. However, no general characteristic clinical findings were found to be common to the 18 cases.ConclusionsContrary to general expectations, some soft tissue tumors that grow slowly are painless, and those that occur in superficial limbs may be highly malignant. Thus, even when a slow growing, painless superficial mass is encountered in a limb, physicians should keep the possibility of highly malignant soft tissue sarcoma in mind.
Highlights
Soft tissue sarcomas that develop in the limbs and the axial area, even those that are diagnosed early using appropriate methods and are treated adequately, have a 5-year survival rate of between 62 and 84% [1,2]
Because a substantial proportion of soft tissue sarcomas can grow for a long time without pain, they are often misdiagnosed due to the belief held by a large number of clinicians and the general populations that malignant tumors are painful, grow rapidly, and adhere strongly to adjacent tissues, and slow growing, highly malignant soft tissue sarcomas may not be diagnosed, which is likely to result in poor outcomes and become the basis of disputes between physicians and the patient’s relatives
All three malignant fibrous histiocytoma cases occurred in patients in their 70 s, whereas the alveolar soft part sarcomas occurred in one child (>10 years old), and in one 20 and one 30 year old
Summary
Soft tissue sarcomas that develop in the limbs and the axial area, even those that are diagnosed early using appropriate methods and are treated adequately, have a 5-year survival rate of between 62 and 84% [1,2]. Delayed diagnosis of soft tissue sarcoma is common. The reasons for these delays are variable, and include, slow-growth, no pain, no palpation due to a deep-seated location, and doctorassociated and socioeconomic factors. We defined a slow-growing tumor as one that patients had been aware of for over a year, and using this criterion, we analyzed highly malignant, slowly growing soft tissue sarcoma cases clinically and radiologically. We describe the characteristics of these tumors and provide a review of the literature
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