Prognostic factors in soft tissue leiomyosarcoma of the extremities: a retrospective analysis of 42 cases

Department of Human Pathology and Oncology, University of Florence, Viale Morgagni, 85, 50134 Firenze, Italy.
European Journal of Surgical Oncology (Impact Factor: 3.01). 06/2004; 30(5):565-72. DOI: 10.1016/j.ejso.2004.03.002
Source: PubMed


Soft tissue leiomyosarcomas (LMS) are rare tumours which are associated with a poor prognosis. The goals of the present study were to describe the clinico-pathological and prognostic features of 42 patients affected by soft tissue LMS of the extremities.
The following clinical and pathological parameters were analysed: age, sex, site, size, depth, previous surgical procedures, stage, histological type, nuclear atypia, grade, mitotic activity, necrosis, surgical margins, therapy. Disease-free survival rates were calculated.
The overall 2-year and 5-year disease free survival rates were 42.3% and 32.6%, respectively. By univariate analysis, tumour size (< or =10 cm vs. >10 cm; p = 0.01), average mitotic rate (< or =19/10 HPF vs. >19/10 HPF; p = 0.05), type of excision (wide vs. marginal or intralesional; p = 0.001) and adjuvant radiotherapy (none vs. brachytherapy+external beam radiotherapy vs. external beam radiotherapy; p = 0.02) were significantly correlated with disease progression. By multivariate analysis, the only factor that was found to be an independent predictor of disease relapse was type of excision (p = 0.001).
Large tumour size and high mitotic rate resulted adverse prognostic factors. Adjuvant radiation therapy, in combination with wide surgical excision, allowed the best chance of cure.

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    • "Factors that have correlated with adverse prognosis in patients with subcutaneous leiomyosarcoma include tumor size, high mitotic rate, presence of necrosis, deep-seated tumors with fascia involvement and intratumoral vascular invasion.[89111523] Survival for tumors smaller than 2 cm and larger than 5 cm has been reported to be 95% and 30% respectively.[17] "
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    ABSTRACT: Superficial leiomyosarcomas are rare malignant smooth-muscle tumors accounting for 4-6.5% of all soft-tissue sarcomas, less than 2-3% of cutaneous soft-tissue neoplasms and 0.04% of all cancers. They are divided into cutaneous or dermal and subcutaneous leiomyosarcomas. Subcutaneous tumors have been reported to be associated with an increased risk of local recurrences and distant metastases, compared to their cutaneous counterparts. In this study, we describe a rare case of a recurrent subcutaneous trunk leiomyosarcoma in a 68-year-old male patient. Local recurrence developed two years after the complete surgical resection with wide margins and adjuvant postoperative radiotherapy. The management of the patient is discussed along with a review of the literature. We conclude that subcutaneous leiomyosarcoma is a rare clinical entity which may be associated with an atypical clinical presentation. Physicians should be aware of the misleading features of this tumor in order to avoid delay in diagnosis and treatment. Early complete surgical resection with wide margins of at least 2 cm is the cornerstone of treatment and has been reported to mostly influence the prognosis. However, the tumor has a high tendency to recur locally and metastasize. Recurrence may develop despite wide resection and radiotherapy. Long-term follow-up is mandatory.
    04/2013; 4(1):238-42. DOI:10.4103/0976-9668.107316
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    • "Increasing tumor size, grade, and depth correlates with decreased survival [8, 11, 17–20]. Increasing mitotic index has also been shown to adversely affect prognosis [20] [21], but this correlation has not been true in other studies [8] [11] [17] [18]. Interestingly, at least one-quarter [11] to one-third [17] of somatic LMSs originate from a vessel wall but these lesions are distinguished from the major vessel LMS group, which arises in large vessels, most commonly the inferior vena cava (IVC) [22], have a worse prognosis, and pose uniquely challenging clinical management issues. "
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    ABSTRACT: Leiomyosarcoma is a malignant smooth muscle neoplasm with a complicated histopathologic classification scheme and marked differences in clinical behavior depending on the anatomic site of origin. Overlapping morphologic features of benign and borderline malignant smooth muscle neoplasms further complicate the diagnostic process. Likewise, deciphering the complex and heterogeneous patterns of genetic changes which occur in this cancer has been challenging. Preliminary studies suggest that reproducible molecular classification may be possible in the near future and new prognostic markers are emerging. Robust recapitulation of leiomyosarcoma in mice with conditional deletion of Pten in smooth muscle and the simultaneous discovery of a novel role for Pten in genomic stability provide a fresh perspective on the mechanism of leiomyosarcomagenesis and promise for therapeutic intervention.
    Sarcoma 02/2012; 2012:380896. DOI:10.1155/2012/380896
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    • "Wide excision of the lesion is of outmost importance as it is strongly correlated with disease-free survival [1,2,8,10,12-15]. Massi et al. in a retrospective study of 62 patients with extremity soft tissue leiomyosarcoma reported that seven out of the nine patients who had developed one or more local recurrences had been surgically treated with either marginal or intralesional excision [13]. Similarly, Svarvar et al. in a large study of 225 patients with leiomyosarcoma which was based on Scandinavian Sarcoma Group Register showed that the inadequate local treatment was the main risk factor for local recurrence [15]. "
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    ABSTRACT: Background Superficial leiomyosarcoma is an exceedingly uncommon malignant tumor which could be located either to cutaneous or subcutaneous tissues. Increased mass size and depth, advanced tumor staging and inadequate surgical excision are the main prognostic factors for poor result. Case presentation We report a rare case of a 71-year-old man with an extensive exophytic lesion (12 × 10 cm) in the anterior-medial side of the proximal right tibia. The lesion was painless and consistently neglected by the patient until a skin trauma caused ulceration of the affected area. Magnetic Resonance Imaging revealed a soft-tissue mass which was well defined from the surrounding bone and muscles. As initial biopsy in another hospital hadn't clarified the true nature of the lesion, new samples were taken and the diagnosis of leiomyosarcoma was established. Laboratory examination showed no distant metastasis and wide excision of the neoplasm was decided. After tumor resection, the remaining soft tissue and skin defect was covered with a gastrocnemius myocutaneous flap. The postoperative period was uneventful and wound healing was followed by local radiotherapy and systemic chemotherapy. At 3 years follow up, no recurrence or metastasis was identified and the patient was able to walk and stand without impairment of his ambulatory status. Conclusion Proper surgical management of soft tissue leiomyosarcoma continues to remain the cornerstone of treatment efficacy and the most important prognostic factor for patients' survival. Reconstruction of the remaining soft tissue defect should be always performed at the same operative time when removal of giant size tumors leaves an uncovered cavity with an inadequate sleeve of muscular and skin tissues.
    International Seminars in Surgical Oncology 05/2008; 5(1). DOI:10.1186/1477-7800-5-11
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