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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.
The locomotor system
Published in C. Simon Herrington, Muir's Textbook of Pathology, 2020
Most soft tissue tumours are benign, and usually small, superficially situated lesions such as lipomas. In contrast, soft tissue sarcomas are typically large tumours within the deep soft tissues of the limbs and retroperitoneum. Soft tissue sarcomas are a heterogeneous group of tumours of varying histological type and biological behaviour. Although uncommon they cause considerable morbidity and mortality.
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.
A case of spindle cell dominant histiocytic sarcoma showing a complete remission after first-line chemotherapy with doxorubicin and ifosfamide
Published in Journal of Chemotherapy, 2020
Yusuke Nakamura, Akihiro Takemasa, Yoshitomo Kushima, Sayo Soda, Naoya Ikeda, Ryo Arai, Kazuyuki Chibana, Yoshimasa Nakazato, Tomoyuki Yokose, Yasuo Shimizu, Seiji Niho
The needle bone biopsy samples showed atypical spindle and giant cells on hematoxylin-eosin (HE) staining (Figure 2A–B). Trabecular bone was maintained without destruction, suggesting a metastatic tumor. Spindle cells were morphological predominant, positive immunostaining for vimentin, and negative immunostaining for cytokeratin (CK) 7, CK20, anti-pan cytokeratin AE1/3, thyroid transcription factor-1 (TTF-1), anti-tyrosinase antibody (S-100), desmin, smooth muscle actin (SMA), myoglobin, anti-muscle actin (HHF35), factor 8, cluster of differentiation (CD) 34, CD1a, myeloperoxidase (MPO), CD20, CD3, signal transducer and activator of transcription 6 (STAT6), and CD21. Therefore, the initial diagnosis was unclassified soft tissue sarcoma. After two courses chemotherapy, additional immunostainings were performed to make a definitive diagnosis. Tumor cells were positive for CD14, CD68, CD163, epithelial membrane antigen (EMA), CD99, CD4, and lysozyme. Positive immunostaining for CD14, CD68, CD163, and lysozyme were shown in Figure 2C–F (×400). The Ki-67 (Mib-1) proliferative index was low (about 15%) (Figure 2G). Because histiocyte markers were positive, and other diseases had been excluded, the final diagnosis was determined as HS. The programmed death-ligand 1 (PD-L1) tumor proportion score was <1%. The therapeutic biomarkers, real-time polymerase chain reaction (RT-PCR) of epidermal growth factor receptor (EGFR) and echinoderm microtubule-associated protein-like 4 - anaplastic lymphoma kinase (EML4-ALK) fusion gene from frozen storage cells were negative (analyzed by SRL Inc.).