Dataset Information

[High-grade Fetal Adenocarcinoma of the Lung: A Case Report].

ABSTRACT: Fetal adenocarcinoma of the lung (FLAC) is an extremely rare subtype of lung cancer, accounting for only 0.1% to 0.5% of primary pulmonary malignancy. In 2011, international multidisciplinary classification of lung adenocarcinoma developed by the International Association for the Study of Lung Cancer (IALSC), the American Thoracic Society (ATS) and the European Respiratory Society (ERS) classified FLAC as a variant of invasive adenocarcinoma. FLAC has been further divided into low-grade fetal adenocarcinoma (L-FLAC) and high-grade fetal adenocarcinoma (H-FLAC) as these two categories exhibit different clinicopathological features and biological behaviors. Here we report a case of high-grade fetal adenocarcinoma and summarize clinicopathologic features of fetal lung adenocarcinoma.?.

SUBMITTER: Huang C

PROVIDER: S-EPMC6441118 | biostudies-literature | 2019 Mar

REPOSITORIES: biostudies-literature

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[High-grade Fetal Adenocarcinoma of the Lung: A Case Report].

Huang Chuan C Ma Chao C Wu Qingjun Q Wang Zheng Z Sun Yaoguang Y Jiao Peng P Tian Wenxin W Yu Hanbo H Tong Hongfeng H

Zhongguo fei ai za zhi = Chinese journal of lung cancer 20190301 3

Fetal adenocarcinoma of the lung (FLAC) is an extremely rare subtype of lung cancer, accounting for only 0.1% to 0.5% of primary pulmonary malignancy. In 2011, international multidisciplinary classification of lung adenocarcinoma developed by the International Association for the Study of Lung Cancer (IALSC), the American Thoracic Society (ATS) and the European Respiratory Society (ERS) classified FLAC as a variant of invasive adenocarcinoma. FLAC has been further divided into low-grade fetal ad ...[more]

PMID: 30910000

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Project description:BackgroundFetal adenocarcinoma of the lung is a rare variant of lung adenocarcinoma and is subcategorized into low-grade and high-grade (H-FLAC) fetal adenocarcinoma. We previously reported poor prognosis in pulmonary adenocarcinomas with an H-FLAC component; however, the genetic abnormalities involved in H-FLAC remain unclear. Therefore, this study aimed to elucidate molecular abnormalities as potential therapeutic targets for H-FLACs.MethodsWe performed immunohistochemical analysis and comprehensive genetic analyses using whole-exome sequencing in 16 lung cancer samples with an H-FLAC component. DNA was extracted from formalin-fixed paraffin-embedded tissues after macrodissection of the H-FLAC component.ResultsCancer-related mutations were identified in TP53 (7/16 cases), KMT2C (6/16 cases), KRAS (4/16 cases), NF1 (3/16 cases), STK11 (3/16 cases), CTNNB1 (2/16 cases), and EGFR (1/16 cases). A high tumor mutation burden of ≥10 mutations per megabase was observed in 3/16 cases. A high microsatellite instability was not detected in any case. Based on the cosine similarity with the Catalogue of Somatic Mutations in Cancer mutational signatures, H-FLACs were hierarchically clustered into three types: common adenocarcinoma-like (five cases), surfactant-deficient (ten cases), and signatures 2 and 13-related (one case). All common adenocarcinoma-like cases presented thyroid transcription factor-1 (TTF-1) expression, whereas surfactant-deficient cases often presented loss of TTF-1 and surfactant protein expression and included cases with mutations in the surfactant system genes NKX2-1 and SFTPC. H-FLACs displayed low programmed death ligand-1 (PD-L1) expression (1-49% of tumor cells) in 5/16 cases, and no case displayed high PD-L1 expression (≥50% of tumor cells).ConclusionsThis study indicates that lung cancers with an H-FLAC component rarely harbor currently targetable driver gene mutations for lung cancer but display a high frequency of KMT2C mutations. The microsatellite instability, tumor mutation burden, and PD-L1 expression status suggest a poor response to immune checkpoint therapy.

Project description:BackgroundSweet's syndrome is a rare inflammatory disease of unknown etiology, and its relationship with tumors is unknown at present. Sweet's syndrome in patients with solid tumors, especially adenocarcinoma of the lung, is extremely rare. At present, only 1 case of an operative patient has been reported in the literature. Diagnosing lung cancer with Sweet's syndrome is not easy, when there is a fever with an unknown cause and erythemas, especially when the erythemas do not disappear after antibiotic treatment, a skin biopsy is much important. Although the exact mechanism of the disease and its link to lung cancer are unknown, our case shows that the active surgical treatment of the primary disease and appropriate glucocorticoid therapy are effective means.Case descriptionWe report the first case of a patient with Sweet's syndrome and lung adenocarcinoma with a decrease in peripheral whole blood cells. A 66-year-old male patient presented, who had been suffering from a fever for >10 days and had multiple tender erythemas, erythemas were mainly on the limbs and upper chest. He was treated with a variety of antibiotics, but his symptoms did not improve significantly. The routine blood tests results show a decline in peripheral blood cells, a chest computed tomography (CT) examination showed a space occupying lesion in the middle lobe of the right lung, which was considered peripheral lung cancer. Sweet's syndrome was diagnosed after a skin biopsy, a pathological examination showed that a large number of neutrophils were infiltrating. The patient then underwent video-assisted thoracoscopic lobectomy associated with the systematic dissection of the mediastinal lymph node, and glucocorticoids were administered. After the operation, the tender erythemas and fever disappeared, at the 1-month follow-up, the chest CT showed no obvious tumor recurrence or metastasis.ConclusionsTo the best of our knowledge, this is the first report of Sweet's syndrome in a patient with lung adenocarcinoma with 3 cell lines reduced. The active surgical treatment of the primary disease and appropriate glucocorticoid therapy proved to be an effective treatment for this syndrome.

Project description:BACKGROUND:Well-differentiated fetal adenocarcinoma (WDFA) of the lung is a rare disease that resembles fetal lung tubules. Most of previous reports concerning WDFA have focused on histological features, while there are few reports describing radiological features. In addition, there are no reports evaluating the difficulty of intraoperative diagnosis of WDFA with frozen section. We report a case of WDFA and review the radiological features of WDFA including the findings of F-18 fluorodeoxyglucose positron emission tomography (FDG-PET) and assess the difficulty of intraoperative diagnosis with frozen section. CASE PRESENTATION:A chest radiography performed in a 20-year-old female revealed a mass in the hilum of the right lung. Computed tomography revealed a well-defined mass measuring 3.5 × 3.0?cm in diameter in the right upper lobe, whereas PET showed a high accumulation of FDG. The most likely diagnosis was clinical T2aN0M0 stage 1B non-small cell lung cancer. A right S3 segmentectomy was performed via thoracotomy, and a benign tumor that was possibly an adenoma was intraoperatively diagnosed based on frozen section analysis. The mass was a solid tumor measuring 2.9 × 2.5?cm in diameter. Microscopically, the tumor comprised abundant glands with single or double layers of nonciliated cells and bronchial structures resembling a fetal lung. Rounded morules of polygonal cells were frequently observed. Immunohistochemistry revealed that nuclei and cytoplasm of the tumor cell were positive for ?-catenin. Finally, the postoperative pathological diagnosis was well-differentiated fetal adenocarcinoma of the lung, and completion right upper lobectomy and mediastinal lymph node dissection were conducted 1?month after the initial segmentectomy. No residual tumor or lymph node metastasis was identified, and the final pathological stage was pT1cN0M0 stage 1A3. The patient did not wish to receive any adjuvant therapy. At the 1-year follow-up, no evidence of recurrence was noted. CONCLUSIONS:Here, we report a rare case of well-differentiated fetal adenocarcinoma of the lung that was difficult to diagnose based on radiological evaluations including FDG-PET and intraoperative diagnosis using frozen section analysis.

Dataset Information

[High-grade Fetal Adenocarcinoma of the Lung: A Case Report].

Publications

[High-grade Fetal Adenocarcinoma of the Lung: A Case Report].

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