Multimodality management of soft tissue sarcomas

Soft tissue sarcomas are a heterogeneous group of malignant tumours arising from connective tissues of mesenchymal origin. By definition sarcomas arising from bone or cartilage are excluded though they have much in common in their behaviour as well as in the approach to their management. A retrospective study of all cases of soft tissue sarcomas diagnosed and notified in the Maltese Islands over the period 1.1.87 to 31.10.89 was carried out. Information was obtained from the cancer registry, the patient’s hospital files and radiographic records. When there was reasonable doubt about the diagnosis a review of histological material submitted was carried out.peer-reviewe

Don't cancel the surgery just yet! A case report of positive preoperative pregnancy test due to a soft tissue sarcoma production of ectopic beta human chorionic gonadotropin.

Soft tissue sarcomas are a rare group of mesenchymal malignancies which can range from low to high grade. These tumors have different clinical, radiographic, and histopathological characteristics. Beta human chorionic gonadotropin is a naturally secreted hormone by placental syncytiotrophoblast cells during pregnancy. On very rare occasions, sarcomas can develop the ability to ectopically produce human chorionic gonadotropin. Very few cases exist in the literature of soft tissue sarcomas expressing this hormone. We report the case of a 55-year-old female who presented with a posterior thigh soft tissue sarcoma who on the day of surgical resection was found to have an unusually elevated serum human chorionic gonadotropin. Positive immunohistochemical staining of the resected mass confirmed the sarcoma as the source of the beta human chorionic gonadotropin

Intraduodenal sarcoma recurrence of retroperitoneal origin: an unusual cause for a duodenal obstruction.

Soft tissue sarcomas are uncommon tumors, and intraduodenal soft tissue sarcoma manifestation is even more rare. Only three cases of intraduodenal sarcomas have been reported in the literature thus far. Here, we report a case of an intraduodenal recurrence of a retroperitoneal sarcoma causing bowel obstruction. This unusual recurrence pattern likely relates to the patient's previous resection and radiation treatment, and highlights the benefits, limitations and follow-up strategies after multimodality treatment

ESF-EMBO symposium "molecular biology and innovative therapies in sarcomas of childhood and adolescence" Sept 29–Oct 4, Polonia Castle Pultusk, Poland

Rhabdomyosarcoma (RMS) and Ewing sarcoma (ES) are among the most common pediatric sarcomas (Arndt et al., 2012). Despite sarcomas representing a highly heterogeneous group of tumors, ES and alveolar RMS (ARMS) typically share one common genetic characteristic, namely a specific chromosomal translocation (Helman and Meltzer, 2003; Lessnick and Ladanyi, 2012). These translocations generate fusion proteins, which are composed of two transcription factors (TF). Typically, one TF is a developmentally regulated factor that is essential for proper specification of a given lineage and provides the DNA-binding domain, while the partner TF contributes a transactivation domain that drives aberrant expression of target genes. Based on these common genetic characteristics, the first ESF-EMBO research conference entitled “Molecular Biology and Innovative Therapies in Sarcomas of Childhood and Adolescence” with special focus on RMS and ES was held at the Polonia Castle in Pultusk, Poland. The conference gathered 70 participants from more than 15 countries and several continents representing most research groups that are active in this field

BMP2/BMPR1A is linked to tumour progression in dedifferentiated liposarcomas

Bone Morphogenic Protein 2 (BMP2) is a multipurpose cytokine, important in the development of bone and cartilage, and with a role in tumour initiation and progression. BMP2 signal transduction is dependent on two distinct classes of serine/threonine kinase known as the type I and type II receptors. Although the type I receptors (BMPR1A and BMPR1B) are largely thought to have overlapping functions, we find tissue and cellular compartment specific patterns of expression, suggesting potential for distinct BMP2 signalling outcomes dependent on tissue type. Herein, we utilise large publicly available datasets from The Cancer Genome Atlas (TCGA) and Protein Atlas to define a novel role for BMP2 in the progression of dedifferentiated liposarcomas. Using disease free survival as our primary endpoint, we find that BMP2 confers poor prognosis only within the context of high BMPR1A expression. Through further annotation of the TCGA sarcoma dataset, we localise this effect to dedifferentiated liposarcomas but find overall BMP2/BMP receptor expression is equal across subsets. Finally, through gene set enrichment analysis we link the BMP2/BMPR1A axis to increased transcriptional activity of the matrisome and general extracellular matrix remodelling. Our study highlights the importance of continued research into the tumorigenic properties of BMP2 and the potential disadvantages of recombinant human BMP2 (rhBMP2) use in orthopaedic surgery. For the first time, we identify high BMP2 expression within the context of high BMPR1A expression as a biomarker of disease relapse in dedifferentiated liposarcomas

The reverse protraction factor in the induction of bone sarcomas in radium-224 patients

More than 50 bone sarcomas have occurred among a collective of about 800 patients who had been injected in Germany after World War II with large activities of radium-224 for the intended treatment of bone tuberculosis and ankylosing spondylitis.^In an earlier analysis it was concluded that, at equal mean absorbed doses in the skeleton, patients with longer exposure time had a higher incidence of bone sarcomas.^The previous analysis was based on approximations; in particular, it did not account for the varying times at risk of the individual patients.^In view of the implications of a reverse protraction factor for basic considerations in radiation protection, the need was therefore felt to reevaluate the data from the continued follow-up by more rigorous statistical methods.^A first step of the analysis demonstrates the existence of the reverse dose-rate effect in terms of a suitably constructed rank-order test.^In a second step of the analysis it is concluded that the data are consistent with a linear no-threshold dose dependence under the condition of constant exposure time, while there is a steeper than linear dependence on dose when the exposure times increase proportionally to dose.^A maximum likelihood fit of the data is then performed in terms of a proportional hazards model that includes the individual parameters, dose, treatment duration, and age at treatment.^The fit indicates proportionality of the tumor rates to mean skeletal dose with an added factor (1 + 0.18.tau), where tau is the treatment time in months.^This indicates that a protraction of the injections over 15 months instead of 5 months doubles the risk of bone sarcoma

Ifosfamide with regional hyperthermia in soft-tissue sarcomas

For high-risk soft tissue sarcomas (HR-STS) of adults, new treatment strategies are needed to improve outcome with regard to local control and overall survival. Therefore, systemic chemotherapy has been integrated either after (adjuvant) or before (neoadjuvant) optimal local treatment by surgery and radiotherapy in HR-STS. The combination with regional hyperthermia as a new treatment strategy seems to open a new therapeutic window. Copyright (C) 2003 S. Karger AG, Basel