2023 World Conference on Lung Cancer (Posters)-EP06.05. Subpleural Cystic Mesenchymal Neoplasm with Endometrial-type Stromal Proliferation: A Report of 3 Cases

EP06.05. Subpleural Cystic Mesenchymal Neoplasm with Endometrial-type Stromal Proliferation: A Report of 3 Cases - PDF(Slides)

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This report discusses three cases of subpleural cystic mesenchymal neoplasm with endometrial-type stromal proliferation. Endometrial stromal and related tumors (EST) are rare and include endometrial stromal nodules (ESN) and low and high-grade endometrial stromal sarcomas (ESS). Pulmonary metastases of ESS are rare and often occur years after initial diagnosis. Molecular alterations, including t7;17 translocations and rearrangement of the PHF7 gene, are common in EST. The most common primary pulmonary neoplasm with subpleural cystic change is lymphangioleiomyomatosis (LAM), caused by mutations in the TSC1 and TSC2 genes. <br /><br />The three patients in the study presented with well-demarcated tumors with cystic spaces lined by cuboidal-columnar non-ciliated mucinous or columnar non-mucinous epithelium. The stroma was infiltrated by small spindle cells with clear cytoplasm and angulated nuclei. The surface epithelial cells were positive for CK7 and TTF-1, while the stromal cells were negative. ER/PR and CD10 positivity suggests a gynecologic tract origin, but other diagnoses such as endometriosis, metastatic cellular leiomyoma, immature teratoma, and pulmonary hamartoma with stromal overgrowth were considered. Molecular studies and long-term follow-up are needed for further understanding of these tumors.<br /><br />The report concludes that the stromal cells resemble low-grade endometrial stromal sarcoma, with entrapped pneumocytes being TTF-1 positive. However, additional molecular studies and clinical follow-up are necessary for proper diagnosis and management.

Asset Subtitle

Peter Illei

Meta Tag

Speaker Peter Illei

Topic Pathology & Biomarkers

Keywords

subpleural cystic neoplasm

endometrial stromal proliferation

endometrial stromal nodules

endometrial stromal sarcomas

pulmonary metastases

molecular alterations

lymphangioleiomyomatosis

TSC1 gene mutations

TSC2 gene mutations

low-grade endometrial stromal sarcoma