Outcome of Surgery as Part of Palliative Care of Patients with Symptomatic Advanced or Metastatic Extra-Abdominal High-Grade Soft Tissue Sarcoma.

Background: The modern multimodal treatment of malignant tumors has increased disease-specific survival and decreased the burden of tumor-associated complications. The main focus of palliative surgery is not based primarily on quantitative success parameters of tumor response but is instead mainly on the question of quality of life. Aim: The current study was conducted to analyze the clinical and oncological outcomes of palliative patients with soft tissue sarcoma. Design: Of 309 patients with extra-abdominal high-grade soft tissue sarcoma treated between August 2012 and December 2014, our retrospective analysis revealed 33 palliative patients for this study. All patients were evaluated and managed by a multidisciplinary team with expertise and experience in sarcoma treatment. The survival analysis was made using the Kaplan-Meier method. Results: The main sarcoma symptoms were pain (27.3%) and ulcerated tumors or shortly before ulceration (24.2%). Thirteen patients (39.4%) were operated on with negative margins, 15 (45.5%) with positive margins, 2 with tumor debulking (6.1%), and 3 patients (9.1%) were treated only with palliative hyperthermic isolated limb perfusion. Ten pedicle flaps were performed after sarcoma resection. The median operation time was 85 minutes (range, 37-216 minutes). The median hospitalization stay was 9.5 days (range, 3-27 days). No patients died during hospitalization. Twelve-month disease-free survival was 48.5% (95% confidence interval: 45.4-51.6). Conclusions: Palliative surgery of metastatic or advanced soft tissue sarcoma can improve the wound care and quality of life. Closed noninfected wounds enable further treatment options, such as chemotherapy, immunotherapy, and radiotherapy. This surgery should be considered during the discussion on interdisciplinary tumor boards.

The internal mammary artery perforator flap for neck reconstruction after palliative resection of advanced anaplastic thyroid cancer: a case report.

INTRODUCTION: Defects of the neck after palliative resection of exulcerated tumors could be reconstructed with different skin flaps. CASE PRESENTATION: The present report describes the case of a 40-year-old Caucasian female patient with advanced anaplastic thyroid cancer. The exophytically growing, bad-smelling massive exulcerated tumor caused an esthetic defect, neck mobility restrictions, and mental state deterioration. PRIMARY DIAGNOSIS, INTERVENTIONS, AND OUTCOMES: Palliative debulking of the tumor was performed. The 10 × 5 cm skin defect of the neck was successfully reconstructed with an internal mammary artery perforator island flap. The donor site was closed primarily. The patient had an uneventful clinical course; the cosmetic results and mental state were very pleasing. CONCLUSIONS: The present case illustrates that palliative resection of the tumor and plastic reconstruction of the neck defect promoted other treatments such as radiation or chemotherapy due to the improved local situation.

[Reconstruction of posttraumatic soft tissue defects of the ankle using free fascial flaps from the anterolateral thigh]. / Rekonstruktion posttraumatischer sprunggelenknaher Weichteildefekte durch freie Faszienlappen aus dem anterolateralen Oberschenkel.

BACKGROUND: Free fascial flaps from the anterolateral thigh (ALT) were used to reconstruct soft tissue defects after trauma to the ankle. This modification was compared to the conventional fasciocutaneous method. MATERIAL AND METHODS: The defect size, the thickness of the subcutaneous fat layer on the thigh and the extent of the soft tissue covering the ankle were determined retrospectively. The evaluations were compared between fascial (Fo) and fasciocutaneous flaps (Fc). The foot and ankle outcome score (FAOS) was used. Esthetic outcome surveys were carried out. RESULTS: A total of 18 isolated fractures of the ankle were evaluated. In 94% of the cases a closed soft tissue damage predominated. After fracture fixation using a plate, soft tissue defects with a mean area of 40.4 ± 13.1 cm2 (28-76 cm2) developed. The thickness of the soft tissue covering over the affected malleoli increased significantly in both groups as a result of the flap surgery (4.5 ± 0.7 vs. 21.1 ± 6.4 mm, p < 0.05). A significant difference was found when comparing the body mass index (BMI) between the groups (Fc 26.3 ± 3.4 kg/m2 vs. Fo 30.1 ± 4.2 kg/m2, p < 0.05). For both groups there was a positive correlation (r = 0.843) between the BMI and the thickness of the epifascial fat layer of the thigh. The FOAS survey revealed 75.9 ± 28.9 and 47.9 ± 32.4 points, respectively, for "function in daily life" and "foot and ankle-related quality of life". The esthetic reconstruction result was rated as "acceptable" by 55% and as "good" by 45%. DISCUSSION: The modified method of a free fascial flap from the ALT can be useful in situations where a bulky flap makes it difficult to fit it into the defect.

Classic Type of Epithelioid Sarcoma of the Distal Upper Extremity: Clinical and Oncological Characteristics.

BACKGROUND: The classic type of epithelioid sarcoma (ES) is a rare, aggressive soft tissue neoplasm that most commonly affects the distal upper extremities of young patients. This study aimed to assess clinical features and provide a long-term report of the oncological outcome. METHODS: We retrospectively analyzed our clinical database for patients with ES of the distal upper extremities. RESULTS: Twenty-three patients with ES of the distal upper extremity were treated surgically between January 1990 and August 2018. ES affected most commonly the palmar side of young patients. The most common site affected by a sarcoma was the wrist in 47.8% of cases, followed by metacarpals and fingers with 34.8% and 17.4%, respectively. Most of the patients were treated according to the protocols of interdisciplinary tumor boards with multimodal therapy. A local recurrence was observed in 7 patients (30.4%). The 5 - and 10-year recurrence-free survival was 80.4% (95% confidence interval [CI]: 68.6-76.8) and 60.9% (95% CI: 53.5-68.3), respectively. The 5- and 10-years disease-specific survival was 89.9% (95% CI: 87-92.8) and 61.9% (95% CI: 56.5-67.3), respectively. Five patients (21.7%) had metastasis in regional lymph nodes. CONCLUSION: The classic type of ES represents a group of high-grade sarcomas, which affect the dominantly distal upper extremity. Specific clinical, diagnostic, and oncological characteristics make it difficult to diagnose and therapy. Wide tumor resection as a part of multimodal therapy remains a more viable and common treatment option for patients with ES on distal extremities. High rates of lymph node metastasis are typical for ES.

Optimization of the preoperative requirements of blood units for the surgical treatment of extra-abdominal soft tissue sarcoma: the TRANSAR score.

BACKGROUND AND OBJECTIVES: Excessive preoperative blood orders frequently occur during the preoperative planning of resections of sarcomas. We aimed to develop a prediction score model that would be able to identify a patient cohort in which the cross-matching could be safely evaded. PATIENTS AND METHODS: We retrospectively analyzed data of 309 consecutive patients with extra-abdominal soft tissue sarcomas treated between September 2012 and December 2014. Scorecard scores for variables were calculated and summarized to a total score that can be used for risk stratification. The score was used in a logistic regression model. Results of the optimized model were described as a receiver operating characteristic curve. RESULTS: Preoperative units of red blood cells were requested for 206 (66.7%) patients, of which only 31 (10%) received them. Five parameters were identified with high predictive power. In the visualized barplot, there was an increased risk of blood transfusion with a higher score of TRANSAR. CONCLUSION: A TRANSAR score is a new tool that can predict the probability of transfusion for patients with sarcoma. This may reduce the number of preoperative cross-matching and blood product ordering and associated costs without compromising patient care.

Plasma Sequencing for Patients with GIST-Limitations and Opportunities in an Academic Setting.

Circulating tumor DNA (ctDNA) from circulating free DNA (cfDNA) in GIST is of interest for the detection of heterogeneous resistance mutations and treatment monitoring. However, methodologies for use in a local setting are not standardized and are error-prone and difficult to interpret. We established a workflow to evaluate routine tumor tissue NGS (Illumina-based next generation sequencing) panels and pipelines for ctDNA sequencing in an academic setting. Regular blood collection (Sarstedt) EDTA tubes were sufficient for direct processing whereas specialized tubes (STRECK) were better for transportation. Mutation detection rate was higher in automatically extracted (AE) than manually extracted (ME) samples. Sensitivity and specificity for specific mutation detection was higher using digital droplet (dd)PCR compared to NGS. In a retrospective analysis of NGS and clinical data (133 samples from 38 patients), cfDNA concentration correlated with tumor load and mutation detection. A clinical routine pipeline and a novel research pipeline yielded different results, but known and resistance-mediating mutations were detected by both and correlated with the resistance spectrum of TKIs used. In conclusion, NGS routine panel analysis was not sensitive and specific enough to replace solid biopsies in GIST. However, more precise methods (hybridization capture NGS, ddPCR) may comprise important research tools to investigate resistance. Future clinical trials need to compare methodology and protocols.

"The Baltic Sea Germ": A Case Report of Necrotizing Fasciitis following Vibrio vulnificus Infection.

Reported is an 80-year-old patient with septic shock from necrotizing fasciitis secondary to a Vibrio vulnificus (Vv) infection. The patient reports having been swimming in the Baltic Sea after a minor trauma to the left leg. Emergency superficial necrosectomy followed by intensive medical therapy was performed. Antibiotic therapy was initiated with a third-generation cephalosporin and a tetracycline. Vv was detected in the intraoperative microbiological smears. Instead of a leg amputation and a flap, due to the patient's age, a split skin covering to consolidate the wound was performed. Vv is a gram-negative rod bacterium of the genus Vibrio. Vv occurs in warm, low-salinity seawater (brackish water). In Germany, Vv occurs primarily in river mouths of the low-salinity Baltic Sea. Infections by Vv can occur through open wounds or by eating raw infected seafood, especially oysters. Infection via wounds often take a fulminant lethal course. Patients with chronic diseases, weakened immune system, and open wounds are particularly at risk. Infections with Vv are rare, but occur worldwide. Global warming is expected to spread Vv as water temperature increases and the dilution effect of sea level rise further decreases ocean salinity, and natural disasters promote the spread of Vv.

Localized Angiosarcoma, Not One Disease: A Retrospective Single-Center Study on Prognosis Depending on the Primary Site and Etiology.

BACKGROUND: Angiosarcomas are rare and heterogeneous tumors with poor prognosis. The clinical subtypes are classified depending on the primary site and etiology. METHODS: We conducted a retrospective, monocentric study of 136 patients with localized AS between May 1985 and November 2018. Overall survival (OS), local recurrence-free survival (LRFS), and metastasis-free survival (MFS) were estimated using the Kaplan-Meier method. To identify prognostic factors, univariate and multivariate analyses were performed based on Cox regressions. RESULTS: The median age was 67 years (19-72.8 years). Primary sites were cutaneous (27.2%), breast (38.2%), and deep soft tissue (34.6%). The majority was primary angiosarcomas (55.9%) followed by postradiation (40.4%) and chronic lymphedema angiosarcomas (2.9%). Prognosis significantly differed depending on the primary site and etiology. Shortest median OS and MFS were observed in deep soft tissue angiosarcomas, whereas cutaneous angiosarcomas, angiosarcomas of the breast, and radiation-associated angiosarcomas displayed worse median LRFS. Univariate analyses showed better OS for tumor size <10 cm (p = 0.009), negative surgical margins (p = 0.021), and negative lymph node status (p = 0.007). LRFS and MFS were longer for tumor size <10 cm (p = 0.012 and p = 0.013). In multivariate analyses, age <70 years was the only independent positive prognostic factor for OS in all subgroups. For LRFS, secondary AS of the breast was a negative prognostic factor (HR: 2.35; p = 0.035). CONCLUSIONS: Different behaviors and prognoses depending on the primary site and etiology should be considered for the treatment of this heterogeneous disease. In cutaneous angiosarcomas of the head/neck and postradiation angiosarcomas of the breast, local recurrence seems to have a crucial impact on OS. Therefore, improved local therapies and local tumor staging may have to be implemented. However, in deep soft tissue angiosarcomas, distant recurrence seems to have a major influence on prognosis, which indicates a benefit of additional perioperative chemotherapy.

[Microvascular reconstruction of foot defects using a free femoral periostal flap]. / Mikrovaskuläre Rekonstruktion von Defekten am Fuß durch freien Femur-Periostlappen.

BACKGROUND: The reconstruction of defects in the foot region is a challenge due to the anatomical peculiarities of the soft tissue covering. OBJECTIVE: This article presents the results of the reconstruction of postoperative foot defects using the free femoral periosteal flap (FFPF). MATERIAL AND METHODS: In a patient collective (n = 10) with postoperative wound healing disorders of the foot, the defect zone was covered using a vascularized FFPF from the distal femoral region. The wound healing process was retrospectively analyzed. RESULTS: The mean follow-up time was 20.2 ± 8.22 months (7-35 months). All patients had a soft tissue defect in the foot region with a mean area of 17.9 ± 3.72 cm2 (12-24 cm2). On average, a wound healing disorder occurred 2 weeks after the primary surgery. In the group of patients followed up the vascularized FFPF enabled a stable, definitive and aesthetically pleasing reconstruction and contributed to the bony consolidation. The duration of inpatient care after defect closure was 8-10 days and was significantly less than the duration of wound management prior to transplantation of the FFPF. CONCLUSION: The FFPF is an effective and elegant method for the regenerative reconstruction of defects with accompanying osseous components in the foot region. The FFPF has the advantage of instant thin and pliable tissue coverage in contrast to many other reconstructive methods. The FFPF can promote wound consolidation through the regenerative properties of a vascularized periosteal sheath in the context of a one-step and permanent infection control.

Efficacy of hyperthermic isolated limb perfusion in the treatment of locally recurrent high-grade soft tissue sarcoma of the extremities.

BACKGROUND: This novel study compared the use of tumor necrosis factor (TNF)-alpha and melphalan-based isolated limb perfusion (TM-ILP) to the standard treatment of locally recurrent soft tissue extremity sarcoma. The aim was to assess whether TM-ILP positively influences the recurrence-free survival of locally recurrent high-grade soft tissue sarcoma (STS) of the extremities. METHODS: We retrospectively analyzed our clinical database for patients with STS. Variables were analyzed using chi-square test or Mann-Whitney rank-sum test. Furthermore, Kaplan-Meier survival plots were calculated and a proportional hazard regression model was developed. RESULTS: Out of 448 patients with extraabdominal STS treated between August 2012 and December 2015, 52 cases involving 47 patients had locally recurrent STS. Twenty-eight of these patients were treated with TM-ILP prior to surgical resection (TM-ILP-group), and 24 were treated with standard therapy (without TM-ILP). The 3-year recurrence-free survival for the TM-ILP-group was estimated at 75% (95% confidence interval (CI), 71.5-78.5). Local recurrence-free survival in the standard group was significantly lower (LRFS: 43.4%, 95% CI 38.7-48.1, p = 0.026). Multivariable analysis revealed resection with negative margins, lower number of previous recurrences, and TM-ILP as positive predictors for recurrence-free survival. CONCLUSIONS: TM-ILP and consecutive resection of residual tumor with negative resection margins significantly improves local recurrence-free survival for patients with a first local recurrence of high-grade STS in the extremities.

Secondary angiosarcoma: A fatal complication of chronic lymphedema.

Secondary Angiosarcoma (Stewart-Treves Syndrome) is a rare malignant cutaneous lesion, which arises in chronic lymphedema of the extremity, often observed after breast cancer treatment. We reviewed the history and the oncological outcome of two patients with this disease. Multimodal therapy including hyperthermic isolated limb perfusion with TNF-alpha and Melphalan, combined with radical resection of the affected skin and subcutaneous tissue including the fascia, with large safety margins may probably lead to better survival.

Extended ray resection for sarcoma of the hand: long-term survival and functional results.

In this retrospective study, we analysed the long-term oncological and functional results after extended ray resection for sarcoma of the hand. Recurrence-free and overall survivals were calculated using the Kaplan-Meier method. The function of the operated hand was assessed with the Michigan Hand Questionnaire and compared with the contralateral side. Extended ray resection was performed in 25 out of 168 consecutive patients with soft-tissue and bony sarcomas of the hand. The overall 5- and 10-year, disease-specific survival rates were 86% and 81%, respectively. Local recurrences were observed in two patients. The Michigan Hand Questionnaire score for the affected hand at follow-up in nine patients was 82 points versus 95 for the healthy contralateral hands. We conclude that extended ray resection of osseous sarcomas breaking through the bone into the soft tissue or for soft tissue sarcomas invading bone is a preferable alternative to hand ablation when excision can be achieved with tumour-free margins. Level of evidence: III.

Lower limb function and quality of life after ILP for soft-tissue sarcoma.

BACKGROUND: Isolated limb perfusion with TNF-alpha and melphalan (TM-ILP) in combination with complete tumor resection is an effective treatment option for non-resectable soft-tissue sarcoma of the extremities, with limb salvage rates greater than 80%. The aim of this study was to assess quality of life (QoL) after TM-ILP, also with regard to long-term survival. METHODS: We retrospectively examined 27 patients who had primarily non-resectable soft-tissue sarcoma of the leg and who had undergone TM-ILP and complete tumor resection (with limb-sparing intent) during their follow-up examinations using the Quality of Life Questionnaire (QLQ-C30) and the German Short Musculoskeletal Function Assessment (SMFA-D). The results from the QLQ-C30 were compared to the reference values for the general population, to the "all cancer patients" reference values (both reference values published by the European Organization for Research and Treatment of Cancer (EORTC)), and to the reference values of a historical amputation group from the literature. The results of the SMFA were compared with those from a reference group of healthy individuals. RESULTS: Surprisingly, we found that the global health status/QoL in the TM-ILP group was not significantly different from the general population or from patients with amputation, but it was higher than that of patients with cancer in general. Concerning the SMFA, we did find functional impairments in patients after TM-ILP compared to the reference group. With regard to long-term survival, we found no time-dependent deterioration in QoL for longer time intervals after treatment. CONCLUSIONS: These results support the use of TM-ILP in limb-sparing multimodal therapy settings from a quality-of-life perspective, but they also encourage further research on this matter.