Sarcome alvéolaire primitif du poumon

Trabelsi, Amel; Abdelkrim, Soumaya Ben; Yacoubi, M. T.; Mlika, S.; Hmissa, Sihem; Mokni, Moncef; Korbi, S.

doi:10.1016/s0761-8425(09)72591-4

Cited by 13 publications

(5 citation statements)

References 6 publications

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“…Clinically, pulmonary metastatic ASPS is more common than pulmonary primary ASPS. Only four cases of primary pulmonary ASPS have been reported in PubMed to date [5][6][7][8] . To our best knowledge, the current case is only the fifth case to be reported.…”

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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“…Primary pulmonary ASPS is a rare condition, with only three cases being reported thus far (3)(4)(5). Herein, we report on another case that represents possibly the earliest onset of this condition reported to date (Table 1).…”

Section: Discussionmentioning

confidence: 89%

“…When ASPS is diagnosed, it is usually metastatic in nature, and it is prone to lung, bone, and brain metastases, leading to a poor long-term prognosis. ASPS involving the lung is mostly metastatic, while primary ASPS involving the lung occurs rarely (3)(4)(5). At present, there are only a few cases of patients affected by this condition, and therefore, no standard or first-line recommended treatment has been proposed.…”

Section: Introductionmentioning

confidence: 99%

Case report: Primary alveolar soft-part sarcoma of the lung in a child

Wang²,

Mao³

et al. 2023

Front. Surg.

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Alveolar soft-part sarcoma involving the lung is mostly metastatic in nature, while primary alveolar soft-part sarcoma involving the lung occurs more rarely. Herein, we report a rare case of a patient with primary alveolar soft-part sarcoma of the lung, which may represent the earliest onset of this condition reported thus far. In this patient, surgery was performed to excise the lesion to the greatest extent possible, and the combination of surgery with chemoradiotherapy and an antiangiogenic agent may provide an important reference for the development of standard or first-line treatment for such pediatric patients.

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“…By extensive review of the literature, we found that most pulmonary alveolar soft-part sarcoma originated from distant soft tissue tumor metastasis. To the best of our knowledge, only three cases of primary pulmonary acinar soft tissue tumor have been reported, [11][12][13] making this the fourth case. Rare tumors occurring at unusual sites can provide great challenges to the pathologist.…”

Section: Discussionmentioning

confidence: 92%

Primary Alveolar Soft-Part Sarcoma of the Lung: A Case Report

et al. 2022

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Alveolar soft-part sarcoma is a rare type of soft tissue malignant tumor. Although the tumor can occur in many parts of the body, primary alveolar soft-part sarcoma of the lung is extremely rare. According to previous literature, only 3 cases of primary alveolar soft-part sarcoma of the lung were reported, and no comprehensive analysis was conducted on these cases. Here, we describe another case of alveolar soft-part sarcoma in the lung, where the negative results of immunohistochemical staining cause extreme difficulty in distinguishing this lesion from other tumors. A 30-year-old Chinese male presented with chest pain and dyspnea. Computed tomography revealed a pulmonary mass, and biopsy results showed vacuolar tumor cells with abundant eosinophilic cytoplasm. A number of immunohistochemical markers were negative, but the tumor cells were positive for TFE3 and ASPSCR1::TFE3 fusion gene. No other tumor masses were found in the patient after whole-body scanning. The final diagnosis was primary alveolar soft-part sarcoma of the lung. Pathologists should consider the possibility of alveolar soft-part sarcoma in lung tumors with typical "organ like" or "acinar like" cell nests, where the tumor cells are large, vacuolated, and the nucleolus is obvious. After excluding metastasis from other sites, TFE3 immunohistochemical staining and ASPSCR1::TFE3 fusion gene detection are recommended for the diagnosis of primary alveolar soft-part sarcoma.

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Sarcome alvéolaire primitif du poumon

Cited by 13 publications

References 6 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

Case report: Primary alveolar soft-part sarcoma of the lung in a child

Primary Alveolar Soft-Part Sarcoma of the Lung: A Case Report

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