scholarly journals Alveolar Soft Part Sarcoma: Progress Toward Improvement in Survival? A Population-Based Study

2021 ◽  
Author(s):  
Tomohiro Fujiwara ◽  
Eiji Nakata ◽  
Toshiyuki Kunisada ◽  
Toshifumi Ozaki ◽  
Akira Kawai
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Metastatic Disease ◽  
Soft Part ◽  
Oncological Outcome ◽  
Cytotoxic Chemotherapy ◽  
Prolonged Survival ◽  
Alveolar Soft Part Sarcoma ◽  
National Study ◽  
Study Cohort

Abstract Background: Alveolar soft part sarcoma (ASPS) is a rare histological subtype of soft-tissue sarcoma, which remains refractory to conventional cytotoxic chemotherapy. We aimed to characterize ASPS and investigate whether the oncological outcome has improved over the past decade.Methods: One hundred and twenty patients with newly diagnosed ASPS from 2006 to 2017 were identified from the Bone and Soft-Tissue Tumor Registry in Japan. The Kaplan–Meier estimate and Cox proportional hazard model were used to investigate the survival outcomes and factors affecting prognosis.Results: The study cohort comprised 34 (28%) patients with localized ASPS and 86 (72%) with metastatic disease at presentation. The 5-year disease-specific survival (DSS) was 68% for all patients and 86% and 62% for localized and metastatic disease, respectively (p = 0.019). Metastasis at presentation was the only adverse prognostic factor for DSS (hazard ratio [HR]: 7.65; p = 0.048). Patients who were >25 years (80%; p = 0.023), had deep-seated tumors (75%; p = 0.002), and tumors >5 cm (5–10 cm, 81%; >10 cm, 81%; p < 0.001) were more likely to have metastases at presentation. In patients with localized ASPS, adjuvant chemotherapy or radiotherapy did not affect survival, and 13 patients (45%) developed distant metastases in the lung (n = 12, 92%) and brain (n = 2, 15%). In patients with metastatic ASPS (lung, n = 85 [99%]; bone, n = 12 [14%]; and brain n = 9 [11%]), surgery for the primary or metastatic site did not affect survival. The use of pazopanib significantly prolonged survival in patients who received systemic treatment (p = 0.045), whereas doxorubicin-based cytotoxic chemotherapy did not. Overall, a trend toward improved DSS for metastatic ASPS has been observed since 2012 (5-year, 58%–65%; p = 0.117), when pazopanib was approved for use in advanced disease.Conclusion: The national study confirmed a unique feature of ASPS with frequent metastasis to the lung and brain but an indolent clinical course. An overall trend toward prolonged survival after the introduction of targeted drugs encourages continuous efforts to develop novel therapeutic options for this therapeutically resistant soft-tissue sarcoma.

scholarly journals Alveolar soft part sarcoma in children and adolescents: A report from the Soft-Tissue Sarcoma Italian Cooperative Group

2000 ◽  
Vol 11 (11) ◽  
pp. 1445-1449 ◽  
Author(s):  
M. Casanova ◽  
A. Ferrari ◽  
G. Bisogno ◽  
G. Cecchetto ◽  
E. Basso ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Children And Adolescents ◽  
Soft Part ◽  
Alveolar Soft Part Sarcoma ◽  
Cooperative Group

scholarly journals Alveolar Soft Part Sarcoma with Unusual Cardiac Metastasis: A Case Report and Review of the Literature

2017 ◽  
Vol 2017 ◽  
pp. 1-3
Author(s):  
Abhinav Tiwari ◽  
Bhavana Siddegowda Bangalore ◽  
Himani Sharma ◽  
Zaid Ammari ◽  
Mohammad S. Khan ◽  
...  
Keyword(s):  
Case Report ◽  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Soft Part ◽  
Vascular Tumor ◽  
Left Thigh ◽  
Alveolar Soft Part Sarcoma ◽  
Cardiac Metastasis ◽  
Review Of The Literature ◽  
History Of

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.

Alveolar soft part sarcoma in children and adolescents: The European Paediatric Soft Tissue Sarcoma study group prospective trial (EpSSG NRSTS 2005)

2017 ◽  
Vol 65 (4) ◽  
pp. e26942 ◽  
Author(s):  
Bernadette Brennan ◽  
Ilaria Zanetti ◽  
Daniel Orbach ◽  
Soledad Gallego ◽  
Nadine Francotte ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Children And Adolescents ◽  
Soft Part ◽  
Prospective Trial ◽  
Alveolar Soft Part Sarcoma ◽  
Study Group ◽  
European Paediatric

Efficacy and safety of anlotinib plus TQB2450 in patients with advanced soft tissue sarcoma: A multicenter, single armed, phase 1b trial.

2021 ◽  
Vol 39 (15_suppl) ◽  
pp. 11547-11547
Author(s):  
Jiayong Liu ◽  
Zhengfu Fan ◽  
Wei Guo ◽  
Tian Gao ◽  
Shu Li ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Kinase Inhibitor ◽  
Soft Part ◽  
Epithelioid Sarcoma ◽  
Objective Response ◽  
Progression Free Survival ◽  
Efficacy And Safety ◽  
Phase 1B ◽  
Anthracycline Chemotherapy

11547 Background: Anlotinib, a multitargeted tyrosine kinase inhibitor, had been prove to be effective for the treatment of advanced or metastatic soft tissue sarcoma(STS) faild anthracycline chemotherapy. With the lack of prospective data of combination of PDL-1 inhibitor and antiangiogenic agent, we designed a phase 1b study to investigated the efficacy and safety of anlotinib plus TQB2450 in patients with STS. Methods: Eligible patients (age 18-70, ECOG 0-1, with histopathologically confirmed advanced STS, at least one measurable lesion according to RECIST 1.1, and previously received front-line anthracycline chemotherapy) were included and received anlotinib (12mg qd, D1-14, 21d/cycle) plus TQB2450 (1200 mg, IV, D1, 21d/cycle) until disease progression or intolerable toxicities.The primary endpoint was objective response rate (ORR), secondary endpoints included safety, overall survival (OS), progression-free survival (PFS), disease control rate (DCR). Results: From January 2019 to January 2021, 30 pts were enrolled1, 12 alveolar soft part sarcoma and 18 others (7 synovial sarcoma, 4 leiomyosarcoma, 5 undifferentiated pleomorphic sarcoma, 1 fibrosarcoma and 1 epithelioid sarcoma). ORR by RECIST was 36.7%, DCR was 83.3%, 11/30 pts had PR, 14/30 (46.7%) had SD, 5/30 (16.7%) PD. Median PFS was 9.6 m in all pts and 4.9m. in non-ASPS, respectively. Median OS in non-ASPS was 10.27m, while mOS in all pts and both mPFS and mOS in ASPS had not been reached. Notably, to ASPS pts, ORR was 75%, and DCR was 100%. The most common 1-2 grade treatment-related adverse reaction (TRAE) was hypothyroidism (19/30,63.3%)，hypercholesterolemia (16/30, 53.3%) and hypertriglyceridemia (16/30, 53.3%), the most common ≥3 grade TRAEs were hypertriglyceridemia (3/30, 10%). 6 SAE (20%) occurred, including 2 pneumothorax, 1 Immune associated hapatic injury, 1 hypotension, 1 Immune myocarditis and 1 diabetic ketoacidosis. Conclusions: The combination of anlotinib and TQB2450 showed promising activity in second-line treatment of advanced STS, especially in ASPS, with well tolerance and acceptable toxicity.

scholarly journals A case of multiple synchronously diagnosed brain metastases from alveolar soft part sarcoma without concurrent lung involvement

2021 ◽  
Vol 12 ◽  
pp. 111
Author(s):  
Mark A. Damante ◽  
Kristin M. Huntoon ◽  
Joshua D. Palmer ◽  
David A. Liebner ◽  
James Bradley Elder
Keyword(s):  
Soft Tissue ◽  
Brain Metastasis ◽  
Lung Metastasis ◽  
Soft Tissue Mass ◽  
Soft Part ◽  
Primary Lesion ◽  
Alveolar Soft Part Sarcoma ◽  
Lung Involvement ◽  
Tissue Mass ◽  
Intracranial Involvement

Background: Alveolar soft part sarcoma (ASPS) is a rare soft-tissue sarcoma with a propensity for early hematogenous dissemination to the lungs and frequent brain metastasis. The development of lung metastasis almost invariably precedes intracranial involvement. There are no previously reported cases in which a patient was synchronously diagnosed with ASPS and multiple brain metastasis without lung involvement. Case Description: A 29-year-old gentleman was found to have three intracranial lesions following the onset of generalized seizures. Staging studies identified a soft-tissue mass in the left thigh and an adjacent femoral lesion. Biopsy of the soft-tissue mass was consistent with ASPS. The patient then underwent neoadjuvant stereotactic radiotherapy to all three brain lesions, followed by en bloc resection of the dominant lesion. The patient was then started on a programmed death-ligand 1 (PD-L1) inhibitor. Subsequent surgical resection of the primary lesion and femur metastasis demonstrates a histopathologic complete response of the bony metastasis and partial response of the primary lesion. At present, the patient has received 14 cycles of atezolizumab without recurrence of the primary or bony lesions and the irradiated intracranial disease has remained stable without recurrence of the resected dominant lesion. Conclusion: While intracranial involvement is relatively common in ASPS, a case with multiple, synchronously diagnosed brain metastasis without concurrent lung metastasis has not been described. The presented case discusses the safety and efficacy of aggressive management of intracranial disease in the setting of atezolizumab. Prospective evaluation of the efficacy of checkpoint inhibitors and the prognostic value of PD-L1 expression in ASPS with brain metastasis are necessary.

scholarly journals Alveolar Soft-Part Sarcoma in Olfactory Cleft: A Case Report and Clinicopathological Review

2020 ◽  
pp. 014556132095513
Author(s):  
Saud Alromaih ◽  
Saleh Alqaryan ◽  
Saleh Alabood ◽  
Somaya Alabaishi ◽  
Abdulrazag Ajlan ◽  
...  
Keyword(s):  
Case Report ◽  
Soft Tissue ◽  
Malignant Disease ◽  
Clinical Presentation ◽  
Soft Part ◽  
Soft Tissue Sarcomas ◽  
Alveolar Soft Part Sarcoma ◽  
Olfactory Cleft

Sinonasal alveolar soft-part sarcoma (ASPS) is a rare malignant disease that comprises 0.4% to 1.2% of all soft-tissue sarcomas. Alveolar soft-part sarcoma is usually difficult to diagnose because it has many clinical and pathological mimickers. In this case study, ASPS occurred in the olfactory cleft, representing the second case in this location in the literature. This article presents the clinical presentation, radiologic, and histopathological case, and reviews the literature regarding the differentials.

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