Real-world data of a cohort of patients with soft tissue sarcoma in a single institution of Colombia.

2020 ◽  
Vol 38 (15_suppl) ◽  
pp. e19259-e19259
Author(s):  
Luis Eduardo Pino ◽  
Ivan Camilo Triana ◽  
Aylen Vanessa Ospina Serrano ◽  
Javier Segovia ◽  
Diana Carolina Hennessey

e19259 Background: Soft Tissue Sarcomas (STS) are a group of neoplasm with huge histological diversity and biological behaviors. They have a low prevalence and lack of data, especially in Colombia where there is no specific report of this disease. The objective of this study is to describe clinical characteristics and outcomes of patients with soft tissue sarcoma at Fundación Santafe, a university hospital located in Bogotá. Methods: This is an observational study of a cohort of soft tissue sarcoma patients treated at a single institution with a follow-up of 4 years (2015 - 2019). Clinical, molecular and epidemiological variables were registered, and overall survival was calculated for stage IV sarcomas. For the survival analysis a Kaplan Meier model was used. Results: Twenty-four patients were included. The histologies reported were: Pleomorphic sarcoma 25.0%, Ewing's sarcoma 20.8%, liposarcoma 16.7%, chondrosarcoma 8.3%, leiomiosarcoma 8.3%, synovial sarcoma 8.3%, soft part alveolar sarcoma 8.3%, and dermatofibrosarcoma protuberans 4.3%. OSm for the whole stage IV group was: 30.22m, according to subtypes OSm was: Ewing's sarcoma 37.13 OSm, liposarcoma 11 OSm, chondrosarcoma 12.3 OSm. Only 3 of the cases (2 Ewing's sarcoma and 1 alveolar sarcoma) had multigenic platform information. In these cases, main mutations in BCL2, SOX9, SATB2 and TFE3 were described. In two of the cases PDL1 expression was done with a negative result ( < 1%) (pleomorphic sarcoma and Ewing's sarcoma). Ifosfamide and anthracyclines was the most frequent chemotherapy regimen used, but in two of the cases checkpoint inhibitors were initiated. Conclusions: This real-world cohort of STS have a similar clinical and epidemiological distribution to historic cohorts, but our OSm for Ewing's sarcoma stage IV is longer than reported, even with a case of complete remission after consolidation with autologous bone marrow transplant. Other histologies had a worse prognosis with a less than 12 m OSm. Genomic data were scarce and useless for directed therapies or immunotherapy as usual in STS. [Table: see text]

Author(s):  
Kunhi Mohammed K. P. ◽  
Snehasis Pradhan ◽  
Supratim Bhattacharyya ◽  
Prafulla Kumar Das ◽  
Muhammed Navas N. K.

Background: Soft tissue sarcomas are a rare and heterogeneous group of malignant tumors of mesenchymal origin that comprise less than 1 percent of all adult malignancies. Although they occur anywhere in the body, they involve most commonly in extremities, trunk, retroperitoneum and head and neck. The aim of the study was to analyze clinical and histopathological features of various soft tissue sarcomas.Methods: This was a retrospective study, conducted in tertiary cancer centre in Odisha during the period 2015 to 2018. We collected clinical parameters like age, sex, site of swelling, any associated pain and biopsy reports and these variables were correlated with final histopathology reports.Results: A total of 107 patients were included in the study, with male to female ratio of 2:1(71 and 36) and average age of 43.45 years. All of them presented with a swelling. The lower extremities were the most common sites i.e. 44.62%. Pleomorphic sarcoma was the most frequent histologic variety comprising 43% and less frequent variety were angiosarcoma, and myxoid sarcoma.Conclusions: Soft tissue sarcoma are predominant in males and middle aged population are frequently affected. Most common affected site is lower extremity and pleomorphic sarcoma is the prominent histologic type.


1998 ◽  
Vol 84 (2) ◽  
pp. 167-170 ◽  
Author(s):  
Enza Barbieri ◽  
Giovanni Frezza ◽  
Ombretta Martelli ◽  
Stefano Neri ◽  
Mario Mercuri ◽  
...  

In 1989 we started an accelerated hyperfractionated schedule of radiotherapy (two 1.6 Gy daily fractions) in standard risk localized Ewing's sarcoma of bone, with the aim at reducing late effects in young patients and at improving disease control through a better integration of treatment modalities. From 1991, the same schedule was used in preoperative radiotherapy of adult soft tissue sarcomas of the extremities: the main purpose was to reduce the time to surgery and to evaluate surgical complications in comparison with a previous experience of hypofractionated radiotherapy (one 3 Gy daily fraction). From 1991 to 1997, 76 patients with Ewing's sarcoma and 24 patients with soft tissue sarcoma were treated at our Institution. Results and complication rates are analyzed in comparison with historical data. In Ewing's sarcoma, a correct evaluation of improvement in local control was difficult because of changing treatment policy (bulky disease was not included in the present series). Late effects, as evaluated in patients with a minimum follow-up of 3 years, occurred with similar incidence, but at higher total dose levels in patients treated with accelerated hyperfractionation. In patients with soft tissue sarcomas, incidence of surgical complications is reduced as compared to historical experience. Major problems of wound healing were seen in association with intraoperative brachitherapy boost.


Author(s):  
Umid Ibragimov Umid Ibragimov ◽  
Sundetilla Sargelov Sundetilla Sargelov ◽  
Murat Ensepbaev Murat Ensepbaev ◽  
Amankul Taynekova Amankul Taynekova

Relevance: Peripheral primitive neuroectodermal tumor (PNET) belongs to the group of malignant tumors that develop from migrating embryonic neural crest cells. PNET includes a number of nosological forms: Askin's tumor, esthesioneuroblastoma, the very peripheral primitive neuroectodermal tumor, Ewing's sarcoma . PNET accounts for 3–9% of all soft tissue tumors in children and 19% of all soft tissue sarcomas. In European countries and the United States, the frequency of their occurrence is 3.4 cases per year per 1 million children under 15 years of age, in Kazakhstan - 0.6-1.2 cases per 1 million child population. Rapid tumor growth, malignancy and early metastasis to other organs and systems predetermines its special role in oncology. Objective of the study: to improve the quality and availability of early diagnosis of sarcomas in children in medical institutions of the general medical network. Results of the study: 35 cases of peripheral PNET in children were analyzed. The age of the patients is from 1.5 to 17 years. The average age is 9.3 years. There were 1.3 times more boys among patients than girls. Extra-skeletal localization was found in one patient (3.6%). Radiographically, it is often almost impossible to make a differential diagnosis between the manifestations of Ewing's sarcoma and primary chronic or "healed" (antibiotic) forms of acute hematogenous osteomyelitis in the initial phase of the process, before the formation of the extraosseous soft tissue component. The process of bone damage is more often localized in its diaphysis and subsequently spreads to the metaphyses of the bone.


2021 ◽  
Vol 23 (4) ◽  
pp. 357-359
Author(s):  
Raghav Yelamanchi ◽  
Parikshith Manjunath ◽  
Nikhil Gupta ◽  
CK Durga

Scalp soft tissue sarcomas (STS) are very rare accounting for less than 0.1% of all malignancies. We report a rare clinical image of advanced stage soft tissue sarcoma of the scalp. A 65 year woman had presented to the surgical department with complaints of a rapidly growing swelling over the scalp for three months. On examination there was huge 20 x 20 cm swelling over the scalp in the left temporoparietal region with variegated consistency. Computed tomography of head revealed a large soft tissue mass with necrosis invading the bone and underlying brain parenchyma. Histopathological finding from core needle biopsy revealed pleomorphic sarcoma. STS are highly malignant tumors which should be diagnosed and treated using multimodality approach. Recurrences are common even after complete resection and prognosis is poor.


2014 ◽  
Vol 4 ◽  
pp. 6 ◽  
Author(s):  
Stacey K Mardekian ◽  
Ashish Gandhe ◽  
Markku Miettinen ◽  
Svetlana Pack ◽  
Mark T Curtis ◽  
...  

Extraosseous Ewing's sarcoma/peripheral neuroectodermal tumors (ES/PNETs) are rare neoplasms that account for approximately 10%-15% of soft tissue sarcomas in children and 5% of soft tissue sarcomas in adults. Primary spinal, extraosseous, intradural ES/PNETs are even less common. The diagnosis of ES/PNET is extremely challenging, because the tumor can have a nonspecific radiologic appearance, and the histologic features are shared by many other “small round cell tumors.” Thus, ES/PNET should be included in the radiologic and pathologic differential diagnosis, even in older patients and in unusual tumor sites. We report two cases of spinal, extraosseous, intradural ES/PNETs in adults who presented with back pain. Magnetic resonance imaging revealed contrast enhancing, intradural lesions in the area of the conus medullaris. The tumor in Case 1 was partially intramedullary, while the tumor in Case 2 was exclusively extramedullary. In both cases, the radiologic and intraoperative surgical impression favored ependymoma. The diagnosis of ES/PNET was established in both cases by histopathologic, immunohistochemical, and molecular analysis.


2022 ◽  
Vol 11 ◽  
Author(s):  
Zhichao Liao ◽  
Chao Zhang ◽  
Tielong Yang ◽  
Haotian Liu ◽  
Songwei Yang ◽  
...  

PurposeOur previously study showed that recombinant human endostatin (Endostar) combined with chemotherapy had significant activity to increase the mPFS in patients with advanced sarcomas with tolerable side effects. However, the small cohort size and short follow-up time made it difficult to screen sensitive sarcoma subtypes and determine whether there is an overall survival benefit. With the largest sarcoma cohort to our knowledge, we try to confirm the efficacy and safety of chemotherapy combined with Endostar in stage IV sarcomas, with the specific purpose of finding out the sensitive sarcoma types for this combined treatment.MethodsAfter the exclusion of ineligible patients, 156 patients with stage IV bone and soft tissue sarcomas were included in this study according to the inclusion criteria.ResultsBy the end of follow-up, the ORR was 10.7% (9/84) vs 1.4% (1/72) (p=0.041), the DCR was 26.2% (22/84) vs 5.6% (4/72) (p=0.001) in the combined group and chemotherapy group, respectively. The mPFS of combined group was significantly longer than the chemotherapy group (10.42 vs 6.87 months, p=0.003). The mOS were 26.84 months and 23.56 months, without significant difference (p= 0.481). In osteogenic sarcoma, there was no statistically significant difference in the mPFS between the two groups (p=0.59), while in the soft tissue sarcoma, the mPFS in the combined group was significantly higher than that of the chemotherapy group (11.27 vs 8.05 months, p=0.004). Specifically, undifferentiated polymorphic sarcoma (UPS) was the possible sarcoma subtypes that benefited from the combined therapy. For the 38 UPS patients (28 patients in the combined group and 10 patients in the chemotherapy group), the mPFS in the combined group was up to 14.88 months, while it was only 7.1 months in the chemotherapy group, with a significant difference (p=0.006). The most common adverse events in the combined group were myelosuppression, gastrointestinal reactions and abnormal liver function, without significant difference in two groups.ConclusionChemotherapy plus Endostar could prolong mPFS and improve ORR and DCR in patients with stage IV soft tissue sarcoma, suggesting that the combined therapy could improve the patient prognosis in soft tissue sarcomas, especially the UPS patients.


Biomedicines ◽  
2021 ◽  
Vol 9 (7) ◽  
pp. 774
Author(s):  
Pietro Scicchitano ◽  
Maria Chiara Sergi ◽  
Matteo Cameli ◽  
Marcelo H. Miglioranza ◽  
Marco Matteo Ciccone ◽  
...  

Primary malignant cardiac tumors are rare, with a prevalence of about 0.01% among all cancer histotypes. At least 60% of them are primary soft tissue sarcomas of the heart (pSTS-h) that represent almost 1% of all STSs. The cardiac site of origin is the best way to classify pSTS-h as it is directly linked to the surgical approach for cancer removal. Indeed, histological differentiation should integrate the classification to provide insights into prognosis and survival expectancy of the patients. The prognosis of pSTS-h is severe and mostly influenced by the primary localization of the tumor, the difficulty in achieving complete surgical and pharmacological eradication, and the aggressive biological features of malignant cells. This review aims to provide a detailed literature overview of the most relevant issues on primary soft tissue sarcoma of the heart and highlight potential diagnostic and therapeutic future perspectives.


2021 ◽  
Vol 28 (1) ◽  
pp. 813-817
Author(s):  
Arielle Elkrief ◽  
Suzanne Kazandjian ◽  
Thierry Alcindor

Background: Myxofibrosarcoma is a type of soft-tissue sarcoma that is associated with high rates of local recurrence and distant metastases. The first-line treatment for metastatic soft-tissue sarcoma has conventionally been doxorubicin-based. Recent evidence suggests that myxofibrosarcoma may be molecularly similar to undifferentiated pleomorphic sarcoma (UPS), which is particularly sensitive to gemcitabine-based therapy. The goal of this study was to evaluate the activity of gemcitabine-containing regimens for the treatment of metastatic myxofibrosarcoma refractory to doxorubicin. Material and Methods: We retrospectively evaluated seven consecutive cases of metastatic myxofibrosarcoma at our institution treated with gemcitabine-based therapy in the second-line setting, after progression on doxorubicin. Baseline clinical and baseline characteristics were collected. Primary endpoints were objective response rate (ORR), progression-free survival (PFS) and overall survival (OS). Results: After progression on first-line doxorubicin, a partial, or complete radiological response was observed in four of seven patients who received gemcitabine-based chemotherapy. With a median follow-up of 14 months, median progression-free and overall survival were 8.5 months and 11.4 months, respectively. Conclusions: Gemcitabine-based chemotherapy was associated with encouraging response rates in this cohort, similar to those seen in UPS. Both entities could be studied together for novel gemcitabine-based regimens.


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