Cardiac desmoid tumour

A 64-year-old woman presented with chest pain for 1 month. Computed tomography showed a cardiac apex mass. A 3.3___3.5-cm tumour was identified during surgery. Histological examination confirmed fibroblastic proliferation and spindle cell infiltration. This is the first report of a desmoid tumour in the heart.

Using broad spectrum metabolomics, differences were investigated between paired normal fibroblast and desmoid tumor cells from affected patients. There were differences identified, also, in the metabolomics profiles associated with the two beta-catenin mutations, T41A and S45F. Ongoing drug screening has identified currently available compounds which inhibited desmoid tumor cellular growth by more than 50% but did not affect normal fibroblast proliferation. Two drugs were investigated in this study, and Dasatinib and FAK Inhibitor 14 treatments resulted in unique metabolomics profiles for the normal fibroblast and desmoid tumor cells, in addition to the T41A and S45F. The biochemical pathways that differentiated the cell lines were aminoacyl-tRNA biosynthesis in mitochondria and cytoplasm and signal transduction amino acid-dependent mTORC1 activation. This study provides preliminary understanding of the metabolic differences of paired normal and desmoid tumors cells, their response to desmoid tumor therapeutics, and new pathways to target for therapy.

Clinical and follow-up data of 24 patients with giant desmoid in the abdominal wall underwent radical removal and immediate abdominal wall reconstruction in Diagnostic and Therapeutic Center of Hernia and Abdominal Wall Diseases, First Affiliated Hospital of People’s Liberation Army General Hospital from October 2006 to October 2016 were analyzed retrospectively. All of desmoids were removed radically and proved by the rapid pathologic examination. All of defects were repaired with compound synthetic prosthesis using bridging procedure. Radical removal for patients with giant desmoid in the abdominal wall is an ideal therapeutic method, and compound synthetic patch can be used to repair huge abdominal wall defect, even the defect compromised all of abdominal wall. The long-termed follow-up results showed these procedures had not put bad influence on the quality of patients’ life.

An overview of the most frequent benign abdominal soft tissue tumors and their common imaging features is given. Furthermore, other facts concerning the individual entities that are relevant to radiologic practice are outlined. Hemangiomas, desmoid tumors, lipomas and lipoma variants are presented. Typical characteristics and radiological signs (e._g., iris diaphragm sign, band sign, India ink artifact) are elucidated and illustrated by image examples. Despite the lack of distinctive imaging features, there are a few radiological characteristics suggestive for each of the entities that in many cases allow for correct diagnosis. In cases of doubt, biopsy or surgery is necessary to distinguish them from malignancies.

Patient-, tumor-, and treatment-related factors of 90 children and adolescents with aggressive fibromatosis (AF) treated on multiple prospective trials of the Cooperative Weichteilsarkom Studiengruppe (1981-2015) were analyzed with focus on response and outcome of systemic therapy (ST). ST seems appropriate if a primary complete resection is not feasible and at relapse/progression after resection.

The aim of this retrospective study was to analyze the local control rates focusing on the effect of surgical margins and radiotherapy. From 1981 to 2014, 44 patients had been treated. Fifty four therapies had been applied, in 50 cases surgery +/- radiation therapy, NSAIDs or chemotherapy. In 4 cases a conservative approach was chosen. Surgical margins are less important than keeping function. Radiotherapy might be an option in unresectable lesions, the role of adjuvant radiotherapy is controversially discussed.