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
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.
Available from: Kurt Weiss
- "Tumor size >10 cm at presentation, mitotic rate >19 per 10 HPF, marginal or intralesional excision, and lack of adjuvant radiotherapy were found to be significant risk factors for progression of leiomyosarcomas located in the extremities. In their series, 2-year and 5-year disease-free survival rates were 42.3% and 32.6%, respectively . Another series of 66 patients over 22 years with leiomyosarcoma arising from the soft tissues of the extremities found that only 16% were located in the upper extremities. "
[Show abstract] [Hide abstract]
ABSTRACT: Sarcoma development has been associated with genetics, irradiation, viral infections, and immunodeficiency. Reports of sarcomas arising in the setting of prior trauma, as in burn scars or fracture sites, are rare. We report a case of a leiomyosarcoma arising in an arm that had previously been replanted at the level of the elbow joint following traumatic amputation when the patient was eight years old. He presented twenty-four years later with a 10.8 cm mass in the replanted arm located on the volar forearm. The tumor was completely resected and pathology examination showed a high-grade, subfascial spindle cell sarcoma diagnosed as a grade 3 leiomyosarcoma with stage pT2bNxMx. The patient underwent treatment with brachytherapy, reconstruction with a free flap, and subsequently chemotherapy. To the best of our knowledge, this is the first case report of leiomyosarcoma developing in a replanted extremity. Development of leiomyosarcoma in this case could be related to revascularization, scar formation, or chronic injury after replantation. The patient remains healthy without signs of recurrence at three-year follow-up.
- "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. Survival for tumors smaller than 2 cm and larger than 5 cm has been reported to be 95% and 30% respectively. "
[Show abstract] [Hide abstract]
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.
Available from: R. Lor Randall
- "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  , but this correlation has not been true in other studies    . Interestingly, at least one-quarter  to one-third  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) , have a worse prognosis, and pose uniquely challenging clinical management issues. "
[Show abstract] [Hide abstract]
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.
Data provided are for informational purposes only. Although carefully collected, accuracy cannot be guaranteed. The impact factor represents a rough estimation of the journal's impact factor and does not reflect the actual current impact factor. Publisher conditions are provided by RoMEO. Differing provisions from the publisher's actual policy or licence agreement may be applicable.