Characterization of alveolar soft part sarcoma using a large national database

Hagerty, Brendan; Aversa, John; Diggs, Laurence P.; Dominguez, Dana A.; Ayabe, Reed I.; Blakely, Andrew M.; Davis, Jeremy L.; Luu, Carrie; Hernandez, Jonathan M.

doi:10.1016/j.surg.2020.06.007

Cited by 16 publications

(39 citation statements)

References 23 publications

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“…ASPS is characterized by a high incidence of brain metastasis compared to other subtypes of soft-tissue sarcoma [33]. In this study, brain metastasis was observed in 11% of patients with metastatic ASPS and occurred in 6% of patients with localized ASPS, which was comparable to the published literature [2,17]. These data suggest that intracranial imaging should be added to the routine imaging studies mentioned in the current practice guidelines [34][35][36].…”

Section: Discussionsupporting

confidence: 85%

“…Overall, the 5-year DSSs for localized and metastatic ASPS in this study were 86% and 62%, respectively, which are comparable to the previous studies (Table 5). For localized ASPS, a 5-year survival rate of approximately 60% was reported in 1989 by Lieberman et al [4], while similar results have been observed almost 30 years later in the more recent series [3,17,19,30]. In a recent study using the National Cancer Database (USA), a 5-year survival rate of 73% was reported in 83 patients with localized disease [17].…”

Section: Discussionmentioning

confidence: 55%

“…For localized ASPS, a 5-year survival rate of approximately 60% was reported in 1989 by Lieberman et al [4], while similar results have been observed almost 30 years later in the more recent series [3,17,19,30]. In a recent study using the National Cancer Database (USA), a 5-year survival rate of 73% was reported in 83 patients with localized disease [17]. These data urge the innovation of more effective neoadjuvant/adjuvant therapy for localized ASPS.…”

Section: Discussionmentioning

confidence: 61%

“…Thus, we could not evaluate the e cacy and safety of these therapies precisely based on the number of administrations. Second, the follow-up period was relatively short, with a mean period of 31.5 months; this appears to be common in studies using the large databases [3,17], and longer follow-up may lead to a decrease in disease-speci c death. Third, the information regarding which metastatic site was resected were unavailable if the patients had multiple metastases.…”

Section: Malouf Et Al Described Low E Cacy Of the Antiangiogenic Therapies For Brain Metastasis Of Asps [47]mentioning

confidence: 99%

“…ASPS commonly originates from deep soft tissues of the extremities [1,[7][8][9], predominantly the lower extremities, followed by the trunk, but may also arise from the head and neck, internal organs, tongue, and bone [5,7,[10][11][12][13][14][15]. Clinically, ASPS presents as a slow-growing, painless mass with high vascularity [6], carries a high rate of early distant metastasis [16,17],…”

Section: Introductionmentioning

confidence: 99%

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

Fujiwara

Nakata

Kunisada

et al. 2021

Preprint

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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.

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Section: Discussionsupporting

confidence: 85%

Section: Discussionmentioning

confidence: 55%

Section: Discussionmentioning

confidence: 61%

Section: Malouf Et Al Described Low E Cacy Of the Antiangiogenic Therapies For Brain Metastasis Of Asps [47]mentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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

Fujiwara

Nakata

Kunisada

et al. 2021

Preprint

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Primary alveolar soft part sarcoma of the chest wall: A case report

Salarzaei

Khoroushi

Aminzadeh

et al. 2022

Clinical Case Reports

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Estrogen associates with female predominance in Xp11.2 translocation renal cell carcinoma

Zhu

et al. 2023

Sci Rep

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Based on the epidemiological characteristics of susceptibility and age selectivity for women in Xp11.2 translocation renal cell carcinoma (Xp11.2 tRCC), we inferred that estrogen was to be blamed. Rad54 like 2 (Rad54l2) which might be one of key effector proteins of DNA damage mediated by estrogen was downregulated in numerous cancers, however, its role in epidemiological characteristics of Xp11.2 tRCC was needed to further study. We reviewed 1005 Xp11.2 tRCC cases and collected estrogen data and then compared the onset time of Xp11.2 tRCC cases in female with estrogen changing trend. An RNA-sequencing was performed in estrogen treated HK-2 cells and subsequently bioinformatic analysis was applied based on the Cancer Genome Atlas (TCGA) and GEO database. The male-to-female ratio of Xp11.2 tRCC was 1:1.4 and the median age of onset was 29.7 years old. The onset trend of female was similar to estrogen physiological rhythm (r = 0.67, p < 0.01). In Xp11.2 tRCC and HK-2 cells after estrogen treatment, Rad54l2 was downregulated, and GSEA showed that pathways significantly enriched in DNA damage repair and cancer related clusters after estrogen treated, as well as GO and KEGG analysis. Downregulation of Rad54l2 was in numerous cancers, including renal cell carcinoma (RCC), in which Rad54l2 expression was significantly decreased in male, age over 60 years old, T2&T3&T4 stages, pathologic SII&SIII&SIV stages as well as histologic G3&G4 grades, and cox regression analysis proved that Rad54l2 expression was a risk factor for overall survival, disease-specific survival and progression-free interval in univariate analysis. There existed female predominance in Xp11.2 tRCC and Rad54l2 might play vital role in estrogen mediating female predominance in Xp11.2 tRCC.

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Characterization of alveolar soft part sarcoma using a large national database

Cited by 16 publications

References 23 publications

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

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

Primary alveolar soft part sarcoma of the chest wall: A case report

Estrogen associates with female predominance in Xp11.2 translocation renal cell carcinoma

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