scholarly journals Multidisciplinary sarcoma tumor board: adolescent and young adult soft tissue sarcoma—myxoid liposarcoma and alveolar soft part sarcoma

2020 ◽  
Vol 9 (5) ◽  
pp. 67-67
Author(s):  
Priya Jayachandran ◽  
Dakshesh Patel ◽  
James Hu ◽  
Omar Ragab ◽  
Jennifer Ho ◽  
...  
2017 ◽  
Vol 35 (15_suppl) ◽  
pp. 3060-3060 ◽  
Author(s):  
Tahlia Scheinberg ◽  
Anna Lomax ◽  
Martin HN Tattersall ◽  
David Morgan Thomas ◽  
Geoffrey Brian McCowage ◽  
...  

3060 Background: Sarcomas represent 10–15% of cancers in adolescent and young adult (AYA) patients, and survival for those with metastatic disease or after relapse is poor. Immunotherapy with checkpoint inhibition has improved outcomes in multiple tumour types, but there are limited data on the efficacy of immunotherapy in advanced sarcomas, particularly within the AYA population. Methods: We retrospectively reviewed AYA patients with advanced bone and soft tissue sarcoma who received self-funded pembrolizumab at Chris O’Brien Lifehouse and Children’s Hospital Westmead. Initial response was evaluated after cycle three or four using RECIST v1.1 criteria. Results: Fourteen AYA patients with sarcoma received pembrolizumab 2mg/kg IV every 3 weeks from May 2015 to December 2016. Median age was 24 (14 – 35), male to female was 7:7, ECOG PS was 0 – 1 in 6 patients, 2 in 6 patients and 3 – 4 in 2 patients. Malignancy type included three patients with osteosarcoma (OS), five patients with Ewing sarcoma (ES), two patients with synovial sarcoma (SS), two patients with alveolar soft part sarcoma (ASPS), and one patient with each of embryonal rhabdomyosarcoma (RMS) and clear cell sarcoma (CCS). The median number of pembrolizumab doses was four (range 1 – 16), with one patient still receiving treatment at the time of last follow up. Treatment was generally very well tolerated with no G3-4 toxicity. One patient with ES had an excellent, sustained response; of the two patients with ASPS one had a radiological partial response with an excellent clinical response and one patient achieved stable disease. Three patients (two ES, one RMS) died of disease prior to first scheduled assessment and thus their response was not evaluable. The remaining 8 patients had progressive disease. Conclusions: Our data suggest further evaluation of the role of pembrolizumab in AYA patients with advanced sarcoma is warranted.


2020 ◽  
Author(s):  
Tahlia Scheinberg ◽  
Anna Lomax ◽  
Martin Tattersall ◽  
David Thomas ◽  
Geoff McCowage ◽  
...  

2017 ◽  
Vol 50 ◽  
pp. 150-157 ◽  
Author(s):  
Judy Y. Ou ◽  
Holly Spraker-Perlman ◽  
Andrew C. Dietz ◽  
Rochelle R. Smits-Seemann ◽  
Sapna Kaul ◽  
...  

Author(s):  
Takashi Fukushima ◽  
Koichi Ogura ◽  
Toru Akiyama ◽  
Katsushi Takeshita ◽  
Akira Kawai

Abstract Objective The relationship between the adolescent and young adult age groups and poor overall survival in soft tissue sarcoma and the risk factors for poor outcomes in adolescent and young adult patients with soft tissue sarcoma were analyzed. Methods The medical records of 7759 Japanese patients diagnosed with soft tissue sarcoma from 2006–13 were accessed from the Bone and Soft Tissue Tumor registry. The epidemiological features of adolescent and young adult patients were compared with those of other age groups. The cancer survival rates were calculated using the Kaplan-Meier method. The prognostic factors for cancer survival were analyzed with the Cox proportional hazards models. The primary endpoint for prognosis was tumor-related death. Results There were 210 children, 1467 adolescent and young adults, 2771 adults and 3311 elderly among the 7759 patients identified with soft tissue sarcoma. Compared with other age groups, the proportions of myxoid/round cell liposarcoma, synovial sarcoma, malignant peripheral nerve sheath tumor, primitive neuroectodermal tumor and rhabdomyosarcoma in adolescent and young adult patients were the highest, but none was significantly more prevalent in adolescent and young adult patients. On multivariate analysis, age was not a prognostic factor for poor cancer survival among adolescent and young adult patients with soft tissue sarcoma. The cancer survival rates of adolescent and young adult patients with malignant peripheral nerve sheath tumor were poorer than those of the other age groups; however, adolescent and young adult age was not a prognostic factor on multivariate analysis in malignant peripheral nerve sheath tumor patients. Conclusions Our study is the first to investigate soft tissue sarcoma in adolescent and young adult patients using the nationwide Bone and Soft Tissue Tumor registry. Adolescent and young adult age is not a prognostic factor for poor cancer survival among those with soft tissue sarcoma in Japan.


2000 ◽  
Vol 11 (11) ◽  
pp. 1445-1449 ◽  
Author(s):  
M. Casanova ◽  
A. Ferrari ◽  
G. Bisogno ◽  
G. Cecchetto ◽  
E. Basso ◽  
...  

2017 ◽  
Vol 2017 ◽  
pp. 1-3
Author(s):  
Abhinav Tiwari ◽  
Bhavana Siddegowda Bangalore ◽  
Himani Sharma ◽  
Zaid Ammari ◽  
Mohammad S. Khan ◽  
...  

Alveolar soft part sarcoma is a very uncommon soft tissue malignancy which accounts for <1% of soft tissue sarcoma. It is a malignant and highly vascular tumor arising most commonly in the musculature of the lower extremities, with metastasis primarily to the lungs, bones, and brain. Cardiac metastasis is very rare and only 5 cases have been reported in the literature so far. We report a case of a young woman with a history of surgically resected alveolar soft part sarcoma of left thigh who presented with persistent dry cough and was found to have a cardiac mass, which on biopsy proved to be alveolar soft part sarcoma.


Sarcoma ◽  
2021 ◽  
Vol 2021 ◽  
pp. 1-12
Author(s):  
Che-Jui Lee ◽  
Agnieszka Wozniak ◽  
Thomas Van Cann ◽  
Iris Timmermans ◽  
Jasmien Wellens ◽  
...  

Soft tissue sarcoma (STS) is a heterogeneous family of rare mesenchymal tumors, characterized by histopathological and molecular diversity. Tissue microarray (TMA) is a tool that allows performing research in orphan diseases in a more efficient and cost-effective way. TMAs are paraffin blocks consisting of multiple small representative tissue cores from biological samples, for example, from multiple donors, diverse sites of disease, or multiple different diseases. In 2015, we began constructing TMAs using archival tumor material from STS patients. Specimens were well annotated in terms of histopathological diagnosis, treatment, and clinical follow-up of the tissue donors. Each TMA block contains duplicate or triplicate 1.0–1.5 mm tissue cores from representative tumor areas selected by sarcoma pathologists. The construction of TMAs was performed with TMA Grand Master (3DHistech). So far, we have established disease-specific TMAs from 7 STS subtypes: gastrointestinal stromal tumor (72 cases included in the array), alveolar soft part sarcoma (n = 12 + 47), clear cell sarcoma (n = 22 + 32), leiomyosarcoma (n = 55), liposarcoma (n = 42), inflammatory myofibroblastic tumor (n = 12 + 21), and alveolar rhabdomyosarcoma (n = 24). We also constructed a multisarcoma TMA covering a representative number of important histopathological subtypes on arrays for screening purposes, namely, angiosarcoma, dedifferentiated liposarcoma, pleomorphic liposarcoma, and myxoid liposarcoma, leiomyosarcoma, malignant peripheral nerve sheath tumor, myxofibrosarcoma, rhabdomyosarcoma, synovial sarcoma, and undifferentiated pleomorphic sarcoma, with 7–11 individual cases per subtype. We are currently expanding the list of TMAs with additional sarcoma entities, considering the heterogeneity of this family of tumors. Our extensive STS TMA platform is suitable for rapid and cost-effective morphological, immunohistochemical, and molecular characterization of the tumor as well as for the identification of potential novel diagnostic markers and drug targets. It is readily available for collaborative projects with research partners.


2017 ◽  
Vol 65 (4) ◽  
pp. e26942 ◽  
Author(s):  
Bernadette Brennan ◽  
Ilaria Zanetti ◽  
Daniel Orbach ◽  
Soledad Gallego ◽  
Nadine Francotte ◽  
...  

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