Localized Myxofibrosarcomas: Roles of Surgical Margins and Adjuvant Radiation Therapy.

PURPOSE: The objective of this study was to describe the outcome and prognostic factors for adults treated for localized myxofibrosarcoma. METHODS AND MATERIALS: We conducted a retrospective multicenter study of 425 nonmetastatic patients who underwent surgery between January 1996 and December 2015 in French National Group and were enrolled in the Conticabase. Pathologic diagnosis was systematically reviewed by expert pathologists. The endpoints were relapse-free and metastasis-free survival. Log-rank tests and Cox models have been used to identified prognostic factors. RESULTS: Median age was 66 years; 53% were males; 85% of cases occurred in limbs or superficial trunk; median size was 60 mm; 47% and 39% were grades 2 and 3, respectively; 66% had R0 resection and 34% R1 resection. Adjuvant radiation therapy was given to 65% of patients, neoadjuvant radiation therapy to 3%, neoadjuvant chemotherapy to 7%, and adjuvant chemotherapy to 13%. The median follow-up was 51 months. The 5-year local relapse-free survival was 67%; independent prognostic factors for local relapse were R1 resection (hazard ratio [HR] = 1.26; P = .001) and adjuvant radiation therapy (HR = 0.35; P = .0001) (ie, R1 resection and no adjuvant radiation therapy increase the hazard ratio). In stratified analysis, adjuvant radiation therapy was beneficial after R0 resection (P = .0020) and after R1 resection (P = .0001). The 5-year overall survival was 80%. The 5-year metastasis-free survival was 83%. Independent prognostic factors for metastatic relapse were grade 3 disease (HR = 1.975; P = .0001) and tumor size (HR = 1.006; P = .001). CONCLUSIONS: This large series of myxofibrosarcoma confirms the high rate of local relapse. Combination of R0 resection and adjuvant radiation therapy provided the best local control. In parallel with an increasing rate of R0 resection and adjuvant radiation therapy, we observed a constant improvement in both metastatic and local relapse-free survival during the study.

Implications of staged reconstruction and adjuvant brachytherapy in the treatment of recurrent soft tissue sarcoma.

PURPOSE: Prior studies illustrated a reduction in wound complications with the use of staged reconstruction (SR) and negative pressure wound therapy when treating soft tissue sarcoma (STS) with surgical resection followed by high-dose-rate adjuvant brachytherapy. The purpose of this study is to compare the outcomes of SR and immediate reconstruction (IR) brachytherapy in recurrent STS. METHODS AND MATERIALS: A retrospective review of 40 patients with recurrent STS of the local extremity and trunk treated with resection followed by adjuvant brachytherapy alone. Margin status was defined as positive (SM(+)) if there was microscopic involvement (R1) or ≤1 mm margin and negative (SM(-)) if >1 mm margin was obtained. SR and IR were compared regarding toxicity, local control, and limb preservation. RESULTS: Median followup was 27 months. When comparing the SR (n = 22) and IR (n = 18) cohorts, there was a significantly lower final SM(+) rate in SR (32% vs. 83%, p < 0.01). A 2-year local control benefit seen with SR (80% vs. 34%; p = 0.012) and a final SM(-) (81% vs. 39%; p = 0.023). SR was associated with less toxicity on multivariate analysis, including a 90% decrease in persistent edema, an 80% decrease in wound dehiscence, and a 94% decrease in nonhealing wounds, when compared to IR. Ten of 31 (32%) extremity cases required eventual amputation from either chronic wound complications (n = 4) or local recurrence (n = 6). SR predicted for a benefit in 2-year limb preservation (88% vs. 50%; p = 0.008). CONCLUSION: In our series, the treatment with SR brachytherapy resulted in less morbidity and an improved final SM(-) rate. This technique translated to an improvement in both local control and limb preservation of recurrent STS.

Diagnosis and treatment of a periocular myxosarcoma in a bearded dragon (Pogona vitticeps).

A 5-year-old male Australian bearded dragon (Pogona vitticeps) was presented with a 2-month history of a periocular mass. The clinical evaluation included a physical examination, hematology, biochemistry, and radiographs. The mass was treated surgically and diagnosed as myxosarcoma. Strontium-90 plesiotherapy was attempted, but the mass recurred 5 mo later.

Diagnostic et traitement d'un myosarcome périoculaire chez un dragon barbu(Pogona vitticeps) . Un dragon barbu mâle âgé de 5 ans (Pogona vitticeps) a été présenté avec une anamnèse de masse périoculaire apparue depuis 2 mois. L'évaluation clinique a inclus un examen physique, une hématologie, une biochimie et des radiographies. La masse a été traitée par chirurgie et diagnostiquée comme un myosarcome. Une plésiothérapie au strontium-90 a été tentée, mais la masse est revenue 5 mois plus tard.(Traduit par Isabelle Vallières).

Myxoid chondrosarcoma of the sinonasal cavity in a child: a case report.

Chondrosarcomas are malignant tumors of cartilage that rarely involve the sinonasal region, and myxoid chondrosarcoma is a rare histologic variant of chondrosarcoma that usually occurs in the soft tissue of extremities. Although several case reports and results of small series of chondrosarcomas in the sinonasal region in children are available, myxoid type chondrosarcoma is extremely rare. We recently experienced a case of low grade myxoid chondrosarcoma involving the sinonasal cavity in a 10-year-old boy, and here we report its radiologic-pathologic findings. In this case, chondroid calcification on CT and septal and marginal enhancement on MRI suggested a chondrosarcoma. Whole body PET-CT demonstrated no definite metastatic lesion and a low peak standardized uptake value primary tumor. However, no definite distinguishing imaging features were observed that distinguished low grade myxoid chondrosarcoma from conventional chondrosarcoma.

Primary retroperitoneal tumours, in the material of our department.

After giving a brief survey of the literature, the authors discuss the symptomatology, diagnostics and management of retroperitoneal tumours. Four of their own cases treated between 1979 and 1985 summarized in tables are presented. All of them were operated and three of them received irradiation therapy. The fate of the first patient is unknown. The second patient survived for 24 months following the first operation, with additional three operations and irradiation on two occasions for recurrence of tumour. The third patient survived for 5 months. In the fourth, it could be revealed by ultrasound and angiography that the retroperitoneal tumour was independent of the kidney. Thus, the intact left kidney was preserved during operation. The patient died three months postoperatively at the department of pulmonology. The histological finding of the tumours of all patients was a malignant tumour of mesenchymal origin, a type of sarcoma. Based on their experiences, the authors stress the importance of the early recognition and removal of the tumour, since the patients have a poor chance of surviving these extremely malignant sarcomas in spite of the surgical and irradiation therapies.

Fast neutron radiotherapy for soft tissue sarcomas. University of Washington experience and review of the world's literature.

Sixteen patients with inoperable soft tissue sarcomas were treated definitively with fast neutrons at the University of Washington between August, 1970 and May, 1982. Eleven of these 16 patients were treated with curative intent and form the basis of this report. Actuarial plots are shown for local tumor control and survival. This work is placed in the context of worldwide experience in using fast neutrons to treat unresectable soft tissue sarcomas.

[Mulitiple myxoma of the eyelid]. / Multiple Myxome der Lider.

Case report of a man aged 41 with several myxomas of the lids. At the age of 10 years a myxoma of the nose, primarily interpreted as myxosarcoma, was removed surgically and irradiated. 7 years later a myxoma developed on his right lower lid and 31 years later 3 more myxomas appeared on his left upper lid. The histology of this rare kind of lid tumour is described and the unusual course discussed.

[Radiotherapy of the soft tissue sarcomas (author's transl)]. / Strahlentherapie der Weichteilsarkome.

Although 82,5% of the total body weight consist of mesenchymal tissues, only 1%--3% of all malignant tumors are soft tissue sarcomas. The necessary irradiation dose can only be determined after an exact histopathological diagnosis; therefore this diagnosis has to be made prior to the radiotherapy. Except the fibro- and liposarcomas, all sarcoma form very early lymphogenic metastases. All sarcomas show a remarquable tendency to form recurrences. Surgery is the primary therapy. After that, an irradiation of the tumor region and the regional lymph nodes should be effected under all circumstances. We have treated and checked up 749 patients. After this therapeutic method, the five-year survival rate in the stages T1 and T2 is 60% for the myxosarcoma, 67% for the angiosarcoma, 41% for the fasciculated sarcoma, 40% for the globocellular sarcoma, 30% for the polymorphous sarcoma, 69% for the fibrosarcoma, and 61% for the leiomyosarcoma.