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Establishment of a Primary Culture of Patient-derived Soft Tissue Sarcoma
Published on: April 11, 2018
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Primary Alveolar Soft-Part Sarcoma of the Lung: A Case Report
Jiangying Zhao1, Jiao Peng1, Jingtao Liu2
1Department of Pathology, 118385Mianyang Hospital of T.C.M, Mianyang, Sichuan 621000, P.R. China.
International Journal of Surgical Pathology
|May 6, 2022
Summary
Primary alveolar soft-part sarcoma of the lung is a rare malignancy. This case highlights diagnostic challenges and recommends TFE3 gene fusion detection for accurate diagnosis.
Area of Science:
- Oncology
- Pathology
- Genetics
Background:
- Alveolar soft-part sarcoma (ASPS) is a rare soft tissue malignancy.
- Primary lung ASPS is exceptionally rare, with limited documented cases.
- Accurate diagnosis can be challenging due to overlapping features with other tumors.
Observation:
- A 30-year-old male presented with chest pain and dyspnea.
- Imaging revealed a pulmonary mass with characteristic vacuolated tumor cells.
- Immunohistochemical markers were largely negative, complicating initial diagnosis.
Findings:
- The tumor cells tested positive for TFE3 and the ASPSCR1::TFE3 fusion gene.
- Whole-body scanning excluded metastatic disease, confirming primary lung origin.
- The final diagnosis was primary alveolar soft-part sarcoma of the lung.
Implications:
- Pathologists should consider ASPS in lung tumors exhibiting specific cellular morphology.
- TFE3 immunohistochemistry and ASPSCR1::TFE3 fusion gene analysis are crucial for diagnosis.
- This case adds to the literature on rare primary pulmonary ASPS and aids diagnostic strategies.
