Patterns of Metastatic Recurrence of Genetically Confirmed Myxoid Liposarcoma.

BACKGROUND: Most sarcomas metastasize predominantly to the lungs, and chest x-ray, or computed tomography, is the most commonly used staging investigation. Myxoid liposarcomas (MLSs) are rare tumors with a tendency to metastasize to extrapulmonary loci. The aim of this study was to assess the locations of the first metastases in MLS patients, to guide the design of effective staging and follow-up imaging protocols. METHODS: Patients treated for MLS between 1987 and 2017 were identified in a prospectively maintained register. Histology of the tumors was reassessed. In addition, the presence of one of the pathognomonic gene translocations was confirmed, uniquely for a retrospective series. The surgical and oncological outcomes were reviewed. A comprehensive review of the literature was performed on the metastatic pattern of MLS, including series with 10 or more MLS patients with metastatic disease. RESULTS: A total of 32 patients with genetically confirmed MLS were identified, with a median follow-up of 7.6 years. Seven patients (22%) developed metastatic disease, five initially intra-abdominally and only one to the lungs. The comprehensive review included 14 series with 1853 patients, 348 (19%) of whom had metastases. The location of the first metastases was soft tissues in 32% of patients, intra-abdominal in 26%, pulmonary in 24%, and bone in 17%. CONCLUSIONS: MLSs metastasize often intra-abdominally and to extra-abdominal soft tissues. Thus, whole-body imaging may be indicated during the initial assessment and follow-up of these patients.

Chondrosarcoma of the Chest Wall: A Single Institution Review of 50 Cases.

INTRODUCTION: Chondrosarcoma (CS) is most common primary osseous tumor of the chest wall. The aim of this study was to report results from surgical procedures and evaluate clinical factors predicting survival of patients with chest wall CSs treated in a single tertiary sarcoma center. MATERIALS AND METHODS: Fifty patients with primary CS located in the ribs and sternum were included. Details of the clinical data and oncological outcomes, including local recurrence (LR) and disease-specific survival (DSS), were collected. RESULTS: The tumor was primarily originated in the sternum in 6 patients (12.5%) and in ribs 2 to 11 in the remaining patients. Specimens were histologically graded 1 in 13 patients (26%), 2 in 28 (56%), 3 in 8 (16%), and 1 (2%) as mesenchymal grade 3 CS. R0 margins were obtained in all cases. Reconstruction was warranted in 47 (94%) cases. Local recurrence developed in 3 (6%) patients, and the median time to LR was 17 (range, 16-68) months. Eight (16%) patients developed metastasis. Increasing tumor volume was a statistically significant factor for reduction of DSS. CONCLUSIONS: Chondrosarcoma of the chest wall can be treated effectively with clear margins, resulting in lower LR rate and higher DSS than CS of the extremities and pelvis. Metastasis of the chest wall mostly occurs in high-grade tumors, and the locations of the metastases differ greatly from those observed in CS of the extremities and pelvis. Metastases are commonly extrapulmonary, indicating the need for postoperative follow-up with multiple imaging modalities to monitor recurrence and metastases.

Reconstruction of the superficial femoral vessels with muscle flap coverage for soft tissue sarcomas of the proximal thigh.

BACKGROUND: Surgical resection of soft tissue sarcoma with a margin of healthy tissue may necessitate resection and reconstruction of major blood vessels together with soft tissues of the proximal thigh to preserve the limb. The long-term functional outcomes of these reconstructions remain unestablished. The aim of this report was to assess the vascular and functional outcomes of soft tissue sarcoma patients with femoral vessel reconstructions. PATIENTS AND METHODS: Patients who had undergone oncovascular reconstruction during the treatment of proximal thigh soft tissue sarcoma in 2014-2020 were reviewed for details of the vascular and soft tissue reconstructions, and the oncological and functional outcomes. This included eight patients of a median age 59 (range 19-77) years. All had a reconstruction of at least the superficial femoral artery and vein as well as soft tissue reconstruction with a muscle flap. All vessel reconstructions were done with either autologous vein (six grafts/four patients) or allograft (10 grafts/six patients). A microvascular latissimus dorsi flap, with a skin island, was incorporated to cover the vascular grafts in five patients. A pedicled sartorius or gracilis muscle flap was used to fill the defect in three patients. RESULTS: Graft patency was assessed in seven patients with a median follow-up of 48 (1-76) months. The arterial graft was patent in 6/8 and the vein graft in 2/8 patients. The gait had returned to normal in five of the six patients assessed. The median MTSS was 70 (43-87)% and the TESS 90 (75-100)%. No local recurrence of the sarcoma was detected. CONCLUSIONS: Vascular reconstruction combined with soft tissue reconstruction enables limb-sparing surgery in patients with soft tissue sarcoma involving proximal femoral vessels. Although the surgeries are complex with high early morbidity, the achieved long-term functional outcomes are worthwhile.

Soft Tissue Sarcoma of Lower Extremity: Functional Outcome and Quality of Life.

BACKGROUND: Few studies have focused on patient-related factors in analyzing long-term functional outcome and health-related quality of life (HRQoL) in patients with postoperative lower extremity soft tissue sarcoma (STS). OBJECTIVE: The purpose of this study was to investigate factors associated with postoperative functional outcome and HRQoL in patients with lower extremity STS. METHODS: This cross-sectional study was performed in a tertiary referral center using the Toronto Extremity Salvage Score (TESS), Quality-of-Life Questionnaire (QLQ)-C30 and 15 Dimension (15D) measures. Functional outcome and HRQoL data were collected prospectively. All patients were treated by a multidisciplinary team according to a written treatment protocol. RESULTS: A total of 141 patients who had undergone limb-salvage surgery were included. Depending on the outcome measure used, 19-51% of patients were completely asymptomatic and 13-14% of patients had an unimpaired HRQoL. The mean score for TESS, 15D mobility score, and QLQ-C30 Physical Functioning scale were 86, 0.83, and 75, respectively, while the mean score for 15D was 0.88, and 73 for QLQ-C30 QoL. Lower functional outcome was statistically significantly associated with higher age, higher body mass index (BMI), and the need for reconstructive surgery and radiotherapy, while lower HRQoL was statistically significantly associated with higher age, higher BMI, and reconstructive surgery. CONCLUSION: Functional outcome and HRQoL were generally high in this cross-sectional study of patients with STS in the lower extremity. Both tumor- and treatment-related factors had an impact but patient-related factors such as age and BMI were the major determinants of both functional outcome and HRQoL.

Treatment and Prognosis of Radiation-Associated Breast Angiosarcoma in a Nationwide Population.

BACKGROUND: Radiation-associated angiosarcoma of the breast (RAASB) is an aggressive malignancy that is increasing in incidence. Only a few previous population-based studies have reported the results of RAASB treatment. METHODS: A search for RAASB patients was carried out in the Finnish Cancer Registry, and treatment data were collected to identify prognostic factors for survival. RESULTS: Overall, 50 RAASB patients were identified. The median follow-up time was 5.4 years (range 0.4-15.6), and the 5-year overall survival rate was 69%. Forty-seven (94%) patients were operated on with curative intent. Among these patients, the 5-year local recurrence-free survival, distant recurrence-free survival, and overall survival rates were 62%, 75%, and 74%, respectively. A larger planned surgical margin was associated with improved survival. CONCLUSIONS: We found that the majority of RAASB patients were eligible for radical surgical management in this population-based analysis. With radical surgery, the prognosis is relatively good.

Single centre 30-year experience in treating retroperitoneal liposarcomas.

BACKGROUND AND OBJECTIVES: Liposarcomas form a diverse group of tumors that represent the majority of retroperitoneal soft tissue sarcomas. Radical excision of these retroperitoneal liposarcomas is often challenging due to their large size and proximity to visceral organs and major vessels. Here we present the 30-year experience of our multidisciplinary sarcoma team in the treatment of these tumors and analysis of factors influencing survival. METHODS: Patients with retroperitoneal liposarcomas treated in Helsinki University Hospital from 1987 to 2017 were reviewed. Local recurrence-free survival, metastases-free survival, and disease-specific survival were assessed with Kaplan-Meier analysis, and factors influencing survival were evaluated with Cox regression. RESULTS: A total of 107 patients were identified. The median follow-up time was 5.4 years (interquartile range: 2.2-8.8 years). Local recurrence developed in 72% and metastases in 15% during follow-up. The 5-year disease-free survival was 31% and disease-specific survival was 66%. The multifactorial analysis revealed histological type and grade as predictors of disease-specific survival (P < .01) while multifocality carried a poor prognosis for local recurrence (P = .02) and higher histological grade for metastases (P < .01). CONCLUSIONS: Retroperitoneal liposarcomas rarely metastasize but tend to recur locally. For tumors that have been resected with macroscopically clear margins, histological, type, and grade are significant predictors of survival.

Multidisciplinary Oncovascular Surgery is Safe and Effective in the Treatment of Intra-abdominal and Retroperitoneal Sarcomas: A Retrospective Single Centre Cohort Study and a Comprehensive Literature Review.

OBJECTIVE: Radical excision of retroperitoneal or intra-abdominal soft tissue sarcomas may necessitate vessel resection and reconstruction. The aim of this study was to assess surgical results of retroperitoneal or intra-abdominal sarcomas involving major blood vessels. METHODS: This was a retrospective single centre cohort study and a comprehensive review of literature. Patients with retroperitoneal or intra-abdominal sarcomas treated by the oncovascular team in Helsinki University Hospital from 2010 to 2018 were reviewed for vascular and oncological outcomes. A comprehensive literature review of vascular reconstructions in patients with retroperitoneal sarcoma was performed. RESULTS: Vascular reconstruction was performed in 17 patients, 11 of whom required arterial reconstructions. Sixteen of the operations were sarcoma resections; the post-operative diagnosis for one patient was thrombosis instead of the presumed recurrent leiomyosarcoma. Early graft thrombosis occurred in two venous and one arterial reconstruction. Late thrombosis was detected in three (18%). The median follow up was 27 (range 0-82) months. Of the patients with sarcoma resections 5 (31%) died of sarcoma and further 4 (25%) developed local recurrence or new distant metastases. The comprehensive review of literature identified 37 articles with 110 patients, 89 of whom had inferior vena cava reconstruction only. Eight arterial reconstructions were described. Late graft thrombosis occurred in 14%. The follow up was 0-181 months, during which 57% remained disease free and 7% died of sarcoma. CONCLUSION: Vascular reconstructions enable radical resection of retroperitoneal and intra-abdominal sarcomas in patients with advanced disease. The complex operations are associated with an acceptable rate of serious peri-operative complications and symptomatic thrombosis of the repaired vessel is rare. However, further studies are needed to assess the performance of the vascular reconstructions in the long term.

Forequarter Amputation and Reconstructive Options.

OBJECTIVE: This study aimed to present the results of a series of forequarter amputations (FQAs) and to evaluate the reconstructive methods used. SUMMARY BACKGROUND DATA: Although FQA has become a rare procedure in the era of limb-sparing treatment of extremity malignancies, it is a useful option when resection of a shoulder girdle or proximal upper extremity tumor cannot be performed so as to retain a functional limb. METHODS: Thirty-four patients were treated with FQA in 1989 to 2017. Various reconstructive techniques were used, including free fillet flaps from the amputated extremity. RESULTS: All patients presented with intractable symptoms such as severe pain, motor or sensory deficit, or limb edema. Seventeen patients were treated with palliative intent. Chest wall resection was performed in 9 patients. Free flap reconstruction was necessary for 15 patients, with 11 free flaps harvested from the amputated extremity. There was no operative mortality, and no free flaps were lost. In curatively treated patients, estimated 5-year disease-specific survival was 60%. Median survival in the palliatively treated group was 13 months (1-35 months). CONCLUSIONS: Limb-sparing treatment is preferable for most shoulder girdle and proximal upper extremity tumors. Sometimes, FQA is the only option enabling curative treatment. In palliative indications, considerable disease-free intervals and relief from disabling symptoms can be achieved. The extensive tissue defects caused by extended FQA can be safely and reliably reconstructed by means of free flaps, preferably harvested from the amputated extremity.

Assessment of the structural validity of three foot and ankle specific patient-reported outcome measures.

BACKGROUND: The structural validity of the Lower extremity functional scale (LEFS), the Visual analogue scale foot and ankle (VAS-FA), and the Western Ontario and McMaster Universities osteoarthritis index (WOMAC) has not been compared earlier in patients after foot and ankle surgery. METHODS: Altogether 165 previously operated patients completed the foot and ankle specific instruments, the 15D health-related quality of life (HRQoL) instrument, and general health (VAS). RESULTS: The LEFS, the VAS-FA and the WOMAC had slight differences in their measurement properties. The VAS-FA had the best targeting and coverage. All three foot and ankle measures accounted for mobility and usual activities when compared to the different aspects of generic HRQoL. CONCLUSIONS: The LEFS, the VAS-FA and the WOMAC have relatively similar psychometric properties among foot and ankle patients, yet the VAS-FA provides the best targeting and coverage.

Functional Outcome Measurement in Patients with Lower-Extremity Soft Tissue Sarcoma: A Systematic Literature Review.

BACKGROUND: The importance of functional outcome (FO) in the treatment of patients with extremity soft tissue sarcoma (STS) has been increasingly recognized in the last three decades. This systematic review aimed to investigate how FO is measured in surgically treated lower-extremity STS patients. METHODS: A systematic search of PubMed, Web of Science, and Scopus was performed based on the PRISMA guidelines. The methodologic quality of the publications was measured using the MINORS tool. The results from the included studies examining measurement types, measures, and time of FO measurement were compiled. The FO pooled mean and standard deviation were calculated as a weighted average for the groups. The validity of the applied measures is reported. RESULTS: The literature search found 3461 publications, 37 of which met the inclusion criteria. The measurement types used were clinician-reported outcomes (n = 27), patient-reported outcomes (n = 20), and observer-reported outcomes (n = 2). The most frequently used measures were the Toronto Extremity Salvage Score (TESS) (n = 16) and the Musculoskeletal Tumor Society (MSTS) score 1993 (n = 12). The postoperative FO was relatively good. The pooled mean TESS and MSTS 1993 scores were respectively 83.3 and 86.2 (out of 100). Of the 10 previously reported measures, 3 provide validated FO scores. The methodologic quality of publications was generally low. CONCLUSIONS: Based on this systematic review, several different methods exist for assessing FO in patients with lower-extremity sarcoma. The most frequently used measure is a validated TESS. The postoperative FO of patients with lower-extremity STS seems to increase to the preoperative baseline level during long-term follow-up evaluation.