Alveolar soft‐part sarcoma: Primary metastatic disease and metastatic relapse occurring during long‐term follow‐up

Sparber‐Sauer, Monika; Seitz, Guido; Kalle, Thekla von; Vokuhl, Christian; Scheer, Monika; Münter, Marc W.; Bielack, Stefan; Kazanowska, Bernarda; Ladenstein, Ruth; Niggli, Felix; Klingebiel, Thomas; Fuchs, Joerg; Kościelniak, Ewa

doi:10.1002/pbc.27405

Cited by 16 publications

(5 citation statements)

References 30 publications

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“…The 5-year survival rate of patients with localized ASPS who undergo surgical resection is approximately 80%, whereas that of patients who do not undergo resection is only 10% [9] . ASPS is not sensitive to traditional radiotherapy and chemotherapy [10][11][12][13] ; chemotherapy only has an efficacy of less than 10% [13] . Meanwhile, the benefit of radiotherapy on ASPS is controversial.…”

Section: Discussionmentioning

confidence: 99%

Combined immunotherapy and targeted treatment for primary alveolar soft part sarcoma of the lung: case report and literature review

et al. 2021

Invest New Drugs

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Primary acinar soft part sarcoma of the lung is a rare malignancy with unique cellular structure and clinical and genetic characteristics. Most patients have no obvious clinical symptoms, and only a few develop respiratory symptoms. The typical histological characteristics are acinoid or organ-like structure. Immunofluorescence in situ hybridization suggests a rearrangement of the transcription factor E3 gene. Patients with primary acinar soft part sarcoma of the lung respond poorly to chemotherapy and are thus primarily treated with surgery and targeted therapy. We report herein a unique case of primary alveolar soft part sarcoma of the lung. The patient was a 24-year-old man who underwent targeted treatment combined with immunotherapy. Some of the lesions completely disappeared after treatment, indicating significant efficacy. The treatment benefit observed in the current case provides preliminary evidence that targeted therapy combined with immunotherapy maybe a safe and effective modality in patients with primary pulmonary ASPS, thus warranting further investigation.

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Section: Discussionmentioning

confidence: 99%

Combined immunotherapy and targeted treatment for primary alveolar soft part sarcoma of the lung: case report and literature review

et al. 2021

Invest New Drugs

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“…A review of the published literature related has shown that the response rate of ASPS to chemotherapy is extremely low, with an objective remission rate of approximately 10%. 21 , 22 In addition, the patient and his family members were unwilling to tolerate the severe toxicity and side-effects; and they subsequently refused follow-up systemic chemotherapy. Therefore, based on the patient's wishes and the finding from the relevant literature, we considered changing the regimen for the patient to anlotinib.…”

Section: Discussionmentioning

confidence: 99%

Treatment of a case of primary alveolar soft part sarcoma of the lung: Case report and literature review

Zhao

et al. 2023

J Int Med Res

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Alveolar soft part sarcoma (ASPS) is an extremely rare type of soft tissue sarcoma. The primary sites of ASPS are mostly located in the extremities and trunk. Primary pulmonary ASPS is extremely rare. A search of the PubMed® database identified only five cases of primary pulmonary ASPS. This current case report describes the sixth case of ASPS in a 15-year-old male that presented with recurrent headaches. Head computed tomography showed space-occupying lesions in the left parietal lobe. Positron emission tomography–computed tomography confirmed the space-occupying lesions in the left parietal lobe and showed multiple nodules and masses in the two lungs and pleura, which were considered to be low-grade malignant mesenchymal tumours. The case report presents the clinical characteristics, diagnosis and treatment process. Programmed cell death protein 1 monoclonal antibody (sintilimab) combined with a tyrosine kinase inhibitor (anlotinib hydrochloride) achieved a good therapeutic effect, indicating that this combination therapy is worth exploring further. Large-scale prospective studies are needed to explore and develop standardized treatments for ASPS.

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“…The extent of negative margin may differ according to the behavior of the tumor: low‐ or intermediate‐grade tumors might require more limited surgical margins than large, high‐grade tumors. The possibility of complete resection at diagnosis varies among histotypes (Table 1 ) 9 , 10 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 and by tumor grade, but may also be affected by the size of the patient. For instance, infantile fibrosarcoma (IFS) may have a low likelihood of complete resection because it often presents as a relatively large tumor in babies (median age: 1.9 months), rather than being a particularly locally invasive tumor such as desmoid type fibromatosis.…”

Section: The Oncology Perspective: What Clinical Classification Syste...mentioning

confidence: 99%

The significance of margins in pediatric Non‐Rhabdomyosarcoma soft tissue sarcomas: Consensus on surgical margin definition harmonization from the INternational Soft Tissue SaRcoma ConsorTium (INSTRuCT)

et al. 2023

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Background Margin status following surgery in children, adolescents, and young adults with soft tissue sarcomas is controversial and has been defined differently by various specialties, with definitions changing over time and by cooperative group. The International Soft Tissue Sarcoma Consortium (INSTRuCT) is a collaboration of the Children's Oncology Group (COG) Soft Tissue Sarcoma Committee, European pediatric Soft Tissue sarcoma Study Group (EpSSG), and the European Cooperative Weichteilsarkom Studiengruppe (CWS) devoted to improving patient outcomes by pooling and mining cooperative group clinical trial data. Methods The INSTRuCT non‐rhabdomyosarcoma soft tissue sarcoma (NRSTS) working group aimed to develop international harmonized recommendations regarding surgical margin assessment and definitions in children and adolescents with soft tissue tumors. Results and Conclusion This review addresses accepted principles and areas of controversy, including the perspectives of surgeons, pathologists, radiation oncologists, and pediatric oncologists, to develop a framework for building common guidelines for future research.

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Alveolar soft‐part sarcoma: Primary metastatic disease and metastatic relapse occurring during long‐term follow‐up

Abstract: Complete tumor resection correlates with long-term survival in patients with primary and relapsed MD.

Cited by 16 publications

References 30 publications

Combined immunotherapy and targeted treatment for primary alveolar soft part sarcoma of the lung: case report and literature review

Combined immunotherapy and targeted treatment for primary alveolar soft part sarcoma of the lung: case report and literature review

Treatment of a case of primary alveolar soft part sarcoma of the lung: Case report and literature review

The significance of margins in pediatric Non‐Rhabdomyosarcoma soft tissue sarcomas: Consensus on surgical margin definition harmonization from the INternational Soft Tissue SaRcoma ConsorTium (INSTRuCT)

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