Distal extremities soft tissue sarcomas: Are they so different from other limb localizations?

Abstract

Background and Objectives Soft tissue sarcoma localization in distal extremities (DESTS) of the limbs (hand/fingers, and foot/toes) is unusual. The literature is scarce about their behavior and this study was designed to assess their epidemiological characteristics, outcomes, and prognosis compared to other limb localizations (OLSTS). Methods From 1980 to 2010, adult DESTS and OLSTS in 22 centers were included. Demographics, tumor type, treatment modalities, and latest follow‐up status were collected. Primary endpoints were overall survival and local/metastatic recurrence incidences. Results Two hundred five DESTS and 3001 OLSTS were included. The patients were younger, with more female and smaller tumors in DESTS. There were more clear cell/epithelioid sarcomas, synovial sarcomas, and myxoid liposarcomas vs more dedifferentiated liposarcomas in OLSTS. DESTS tumors were less irradiated and more often amputated (24.3% vs 3.4%). The five‐year survival rate was 78.2% compared to 68.6% in OLSTS and after multivariate analysis, STS localization did not impact survival or local/metastatic recurrence. Conclusion Though rare and smaller than other limb localizations, DESTS are to be considered as aggressive. Despite a higher amputation rate, the prognosis remains the same as in OLSTS. Limb sparing vs amputation should be carefully assessed in DESTS, especially if grade 3 or of a poor prognosis histological subtype.

Nomogram for predicting the overall survival and cancer-specific survival of patients with extremity liposarcoma: a population-based study

“…Previous studies have identified large tumor size as an indicator of poor prognosis for extremity STS patients [3,10,13,14,22], consistent with the present research. This is probably because a large tumor size is related to higher biologic malignancy, including regional invasiveness and metastatic potential.…”

“…A previous study on liposarcoma also reported that age was an independent prognostic predictor [3]; conversely, no clear association between age and survival was observed in a retrospective evaluation over 15 years [3]. Further studies demonstrated that younger patients were more likely to be diagnosed with smaller tumors (≤5 cm vs > 5 cm) [22], distal extremity STS (distal extremities vs other limb localizations) [10], and only pulmonary metastases (pulmonary lesions vs other lesions) [7], and these patients tended to be more easily cured and therefore had a better prognosis. Similarly, a distribution difference by age in terms of tumor location and metastatic sites was detected [7,10], which may also explain why males were associated with unfavorable outcomes.…”

“…Further studies demonstrated that younger patients were more likely to be diagnosed with smaller tumors (≤5 cm vs > 5 cm) [22], distal extremity STS (distal extremities vs other limb localizations) [10], and only pulmonary metastases (pulmonary lesions vs other lesions) [7], and these patients tended to be more easily cured and therefore had a better prognosis. Similarly, a distribution difference by age in terms of tumor location and metastatic sites was detected [7,10], which may also explain why males were associated with unfavorable outcomes. Nevertheless, whether there was a biological reason behind these age and sex distributions is unclear.…”

“…Previous studies also revealed that tumor grade was significantly associated with metastatic potential after surgery and therefore risk of death. However, tumor grade had poor value in predicting local recurrence, which was mainly correlated with suboptimal surgical procedures [10,23,24]. In clinical practice, patients with high-grade tumors or tumors with large diameters were selected for combination therapy with neoadjuvant chemotherapy to limit the risk of distant metastases [8].…”

“…Currently, the American Joint Committee on Cancer (AJCC) system, known as the TNM staging system, remains the gold standard for prognostic prediction for tumor patients. However, other elements that have been reported to be prognostic factors for extremity STS patients are not taken into consideration in the TNM staging system, such as patient factors (including age and sex), tumor characteristics (including tumor grade, histologic subtype, and tumor location), and treatment strategies (including surgery, radiotherapy, and chemotherapy) [7,[9][10][11][12][13][14][15]. More importantly, the TNM staging system is unable to meet the increasing need for precision medicine and cannot provide individual predictions of prognosis at specific times [16,17].…”