- Research
- Open Access
Highly malignant soft tissue sarcoma of the extremity with a delayed diagnosis
https://doi.org/10.1186/1477-7819-8-84
© Park et al; licensee BioMed Central Ltd. 2010
- Received: 27 June 2010
- Accepted: 23 September 2010
- Published: 23 September 2010
Abstract
Purpose
To evaluate the characteristics of highly malignant soft tissue sarcoma of the extremity with a delayed diagnosis.
Materials and methods
The clinical and radiological characteristics of 18 cases of highly malignant soft tissue sarcomas of the extremity with a delayed diagnosis were determined.
Results
Ten 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.
Conclusions
Contrary 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.
Keywords
- Sarcoma
- Soft Tissue Sarcoma
- Synovial Sarcoma
- Malignant Fibrous Histiocytoma
- Clear Cell Sarcoma
Introduction
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]. Early diagnosis is extremely important for the successful treatment of soft tissue sarcoma. However, 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 doctor-associated and socioeconomic factors. 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 thus, 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. Furthermore, because tumor growth rates are subjective, no definition of a slow growing tumor exists in the literature. According to a study by Lawrence et al. [3], approximately 60% of patients with a soft tissue sarcoma knew they had a tumor, and these patients were diagnosed within 6 months of this realization. In this study, 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. Here, we describe the characteristics of these tumors and provide a review of the literature.
Materials and methods
Of 31 cases with a diagnosis of highly malignant (Grade III according to the WHO classification) soft tissue sarcoma of the limb treated at our hospital between July 1997 and December 2008, we analyzed 18 cases in which the delay between patient awareness of the tumor and diagnosis exceeded 1 year. Highly malignant soft tissue sarcoma was diagnosed based on entries in final pathology reports. The male to female ratio of our cohort was 10:8, and mean patient age was 44.8 years (range 15 to 79 years). The characteristics of the 18 cases of highly malignant soft tissue sarcoma were analyzed based on tumor types determined at final diagnosis, times from patient recognition to diagnosis, the anatomical locations of tumors in limbs, depths of tumor locations in tissue (dichotomized as deep or superficial), tumor sizes, and the characteristics of tumors as determined by plain radiography and MRI.
Results
A plain radiograph showing a well-defined soft tissue mass with marginal calcification posterior to the distal femur. There is no evidence of associated bone erosion in this patient. This is an example of a synovial sarcoma in one of its more common locations.
MRI images of an alveolar soft part sarcoma. (A) An axial T1-weighted fat-suppressed image and (B) an axial T2-weighted image. High signal on T1FS, T2WI with multiple signal voids are apparent.
Discussion
Clinical data of all materials
No | Gender | Age (Yrs) | Duration (Yrs) | Diagnosis | Pain | Location | Depth | Size | Previous biopsy |
---|---|---|---|---|---|---|---|---|---|
1 | M | 42 | 6 | S S | No | thigh | deep | 12 × 7 × 7 | N |
2 | M | 27 | 20 | S S | No | forearm | superficial | 3 × 2 × 2 | N |
3 | F | 53 | 2 | S S | mild | ankle | superficial | 1.8 × 1.2 × 0.5 | N |
4 | F | 53 | 10 | S S | mild | foot | superficial | 5 × 5 × 6 | Y |
5 | M | 15 | 1 | S S | No | ankle | superficial | 3 × 3 | N |
6 | F | 28 | 1 | S S | mild | forearm | superficial | 2 × 2 | N |
7 | M | 40 | 10 | S S | Yes | popliteal | superficial | 5 × 5 | N |
8 | M | 70 | 1 | MFH | No | thigh | superficial | 9 × 4 | Y |
9 | F | 79 | 3 | MFH | No | forearm | superficial | 15 × 7 | Y |
10 | M | 69 | 1 | MFH | No | lower leg | superficial | 5 × 2 | Y |
11 | F | 16 | 1 | Alv SS | mild | thigh | deep | 5 × 4 × 3 | Y |
12 | M | 27 | 3 | Alv SS | yes | lower leg | deep | 7 × 4 × 11 | Y |
13 | M | 32 | 3 | Alv SS | yes | lower leg | deep | 15 × 7 × 6 | Y |
14 | M | 72 | 3 | HGL | No | thigh | deep | 4 × 4 × 5 | Y |
15 | F | 68 | 3 | HGL | No | lower leg | superficial | 5 × 4 × 3 | N |
16 | F | 52 | 2 | MFS | mild | lower leg | superficial | 14 × 9 | Y |
17 | M | 31 | 1 | MFS | mild | lower leg | deep | 10 × 5 | Y |
18 | F | 50 | 1 | CS | No | forearm | superficial | 2 × 3 | N |
Since most soft tissue sarcomas lack defined radiological characteristics, it is not easy to differentiate sarcomas, even by MRI. Nonetheless, alveolar soft part sarcomas do have specific imaging results, and thus, imaging tests may play a decisive diagnostic role. The general characteristics of most sarcomas are that they grow quickly, are located deep within tissue, and are relatively large. As shown by the present study, the characteristics of some types of soft tissue sarcomas contrast with the traditionally held opinion that they are small slow-growing masses with a superficial location. Fortunately, non-specific soft tissue sarcomas are uncommon, and if preconceived notions are avoided, they can be diagnosed early. Synovial sarcoma cases occur primarily in adults in the vicinity of the knee joint and in the lower leg, and the tumors grow slowly and occur superficially. On the other hand, alveolar soft part sarcomas occur in adolescents and young adults, and are found preferentially around the knee joint and have specific MRI characteristics.
Conclusions
According to this study, some soft tissue tumors that grow slowly are painless, and some that occur in the superficial limbs may be highly malignant. The most important characteristic of soft tissue sarcomas is their non-specific nature. Thus, even when a slow growing, painless superficial mass is encountered in the limbs, after eliminating absolutely benign possibilities, such as, a ganglion of the wrist or lipoma of the back, physicians must consider the possibility of sarcoma and conduct a careful examination.
Declarations
Authors’ Affiliations
References
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