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Introduction to Cancer
Published in David E. Thurston, Ilona Pysz, Chemistry and Pharmacology of Anticancer Drugs, 2021
The tumorigenic effects of chemicals and radiation have been previously discussed. Unfortunately, this means that certain chemotherapeutic agents (e.g., DNA-interactive agents) and radiation therapy given to patients (particularly children) with cancer can increase the risk of them developing a different type of cancer later in life. This often manifests as soft tissue sarcoma, a disease affecting mostly connective tissue and muscles. According to one study reported in the International Journal of Cancer in 2004, soft tissue sarcoma is one of the most common types of new malignant disease appearing in young adults and teenagers treated with anticancer drugs during childhood. Furthermore, researchers from the Gustave Roussy Institute (France) followed more than 4,000 patients who had survived a first cancer during their childhood and observed the occurrence of 16 soft tissue sarcomas at least 3 years after diagnosis of the first disease. Although this rate of occurrence appears low, it is more than 50 times greater than that observed in the general population. More significantly, 14 of the 16 sarcomas were found in close proximity to the area of treatment of the first cancer. Furthermore, the probability of soft tissue sarcoma occurring as a secondary cancer increased in proportion to the dose of radiation originally administered. The researchers also found that treatment with the DNA-methylating agent procarbazine appeared to raise the risk of sarcoma developing later in life.
Paper 4
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
The imaging features are suspicious for a soft tissue sarcoma. The most appropriate next step is a CT chest to assess for any evidence of disease elsewhere. An MRI pelvis may also be helpful to more accurately delineate the extent of the mass. Discussion at a tertiary sarcoma centre prior to biopsy is important; however imaging should be completed prior to this referral. There are frequently concerns regarding tumour seeding with sarcomatous lesions, and sometimes sarcoma centres will request to biopsy the lesion themselves. Identifying the most appropriate biopsy site may also be aided by completing imaging, as a more superficial lymph node could be more easily accessible than an intrapelvic or abdominal mass. 18F-FDG PET/CT may be helpful but would not be clearly indicated at this point.
Sarcomas
Published in Peter Hoskin, Peter Ostler, Clinical Oncology, 2020
Local effects owing to tumour size are the main problem associated with soft-tissue sarcomas. In general, paraneoplastic effects and systemic complications such as hypercalcaemia are not features of these tumours, although hypoglycaemia has been described as a rare association with massive retroperitoneal sarcomas.
Hyperthermia in the treatment of high-risk soft tissue sarcomas: a systematic review
Published in International Journal of Hyperthermia, 2023
Paraskevi Danai Veltsista, Eva Oberacker, Adela Ademaj, Stefanie Corradini, Franziska Eckert, Anne Flörcken, David Kaul, Lars H. Lindner, Rolf Issels, Oliver J. Ott, Daniel Pink, Vlatko Potkrajcic, Peter Reichardt, Siyer Roohani, Mateusz Jacek Spalek, Oliver Riesterer, Daniel Zips, Pirus Ghadjar
Patients bearing high-risk soft-tissue sarcomas (STS, >5 cm in size, deep location with grade 2-3 according to the Fédération Nationale des Centers de Lutte Contre le Cancer – FNCLCC) have an unfavorable prognosis, with approximately 50% of the patients dying within 5 years of diagnosis despite multimodal treatment approaches. Complete tumor resection remains essential to achieving a cure for the disease [1]. For sarcomas of the extremities and the superficial trunk wall, local control can be improved by the addition of neoadjuvant- or adjuvant radiotherapy (RT) [2,3]. The question of whether the use of neoadjuvant or adjuvant RT is preferred is still under debate, although the notion is moving toward neoadjuvant RT due to a favorable toxicity profile and similar oncological outcomes [4–6]. A recent multi-center, open-label, randomized, phase III trial (European Organization for Research and Treatment of Cancer - EORTC- 62092: STRASS) [7] evaluated the addition of neoadjuvant RT of 50.4 Gy for retroperitoneal sarcomas. The authors showed elevated toxicity and no improvement in outcome and concluded neoadjuvant RT to be a debatable approach for treating retroperitoneal sarcomas. Nevertheless, in the STREXIT study on liposarcomas (which constitute the most common subcategory of retroperitoneal sarcomas) [8] neoadjuvant RT was associated with enhanced abdominal-recurrence-free survival (ARFS) in patients with primary, well-differentiated liposarcoma (HR, 0.61; 95% CI, 0.42–0.89) as well as in patients bearing dedifferentiated liposarcoma grade 1 or 2 (HR, 0.63; 95% CI, 0.40–0.97).
OX40 and 4-1BB delineate distinct immune profiles in sarcoma
Published in OncoImmunology, 2022
MJ Melake, HG Smith, D Mansfield, E Davies, MT Dillon, AC Wilkins, EC Patin, M Pedersen, R Buus, AA Melcher, K Thway, AB Miah, SH Zaidi, AJ Hayes, TR Fenton, KJ Harrington, M McLaughlin
UPS is considered to be more resistant to radiotherapy.4 In the study referenced, 83% of UPS patients tumors had grade 3 disease, with well-differentiated liposarcoma, MLPS, MFS, and LMS ranging from 0 to 36%. The worst 5-year disease-specific survival (DSS) was observed for UPS at 60.1%. In contrast, LMS, MFS, and MLPS were 76.8%, 76.7%, and 84.9%, respectively. 69.2% of UPS patients in that study received radiotherapy, substantially higher that the overall rate of 48.2% across the sarcoma subtypes studied. MLPS has been shown to be radiosensitive relative to other soft tissue sarcomas.21 In that study, where all patients received radiotherapy and surgery, the 5-year overall survival for MLPS was 93.9%, compared to 76.4% for all other soft tissue sarcoma subtypes. In this study, we initially compared gene expression between UPS and MLPS in a retrospective cohort of patients receiving radiotherapy and surgery at our institute. This selection was based on the frequency with which subtypes have been shown to be treated at our center with radiotherapy.4 We sought to identify potential immunotherapy targets of relevance to UPS patients who progress after surgery and radiotherapy, as well as to improve our understanding of the immune contexture of sarcoma subtypes.
Presentation of Infantile Hemangiopericytoma/Solitary Fibrous Tumor as a Giant Extracranial Temporal Mass
Published in Fetal and Pediatric Pathology, 2021
Etkin Boynuyogun, Mert Calis, Altan Kavuncuoglu, Kemal Kosemehmetoglu, Gokhan Tuncbilek
A twenty-five day old girl was referred to our institution with congenital craniofacial soft tissue mass of her right temporal region. She was born via spontaneous vaginal delivery and her antenatal course was uncomplicated. Maternal medical history was noncontributory. Initial evaluation at the pediatric oncology clinic revealed a painless, hard 4x4 cm mass, without signs of inflammation and erythema. Her MRI revealed a highly vascular mass with hemorrhagic and a possible necrotic core without intracranial extension (Figure 1). A congenital soft tissue sarcoma was suspected. Based on her MRI findings, an excisional biopsy of the lesion under general anesthesia was performed after two weeks of clinical follow-up, in which the overall size of the mass had increased to 9x7 cm (Figure 2).