Pulmonary Enteric Adenocarcinoma Research Articles

Primary pulmonary enteric adenocarcinoma: A case report and review of literature

Primary pulmonary enteric adenocarcinoma (P-PEAC) is an unusual subtype of invasive lung adenocarcinoma. The significance of such tumor lies in its similar histopathological and immunohistochemical characteristics to colorectal adenocarcinoma. Therefore, a detailed investigation is crucial to establish a definitive diagnosis. This paper reports a case of a 51-year-old Saudi female, diagnosed with a rare variant of lung carcinoma, P-PEAC.

PP01.127 A rare case of pulmonary enteric adenocarcinoma in a 26-year-old female non-smoker

PP01.127 A rare case of pulmonary enteric adenocarcinoma in a 26-year-old female non-smoker

PP01.127 A rare case of pulmonary enteric adenocarcinoma in a 26-year-old female non-smoker

PP01.127 A rare case of pulmonary enteric adenocarcinoma in a 26-year-old female non-smoker

A case description of pulmonary enteric adenocarcinoma with KRAS G12D mutation.

A case description of pulmonary enteric adenocarcinoma with KRAS G12D mutation.

Gefitinib, an effective treatment option for patients with pulmonary enteric adenocarcinoma harboring compound EGFR L858R and A871G mutation.

Pulmonary enteric adenocarcinoma (PEAC) is a rare lung adenocarcinoma with morphological and immunohistochemical similarities to colorectal adenocarcinoma and intestinal differentiation. PEAC belongs to the group of non-small-cell lung carcinoma (NSCLC) and is defined as having a more than 50% intestinal differentiation component. We report a postoperative (T4N2M0 stage IIIb) PEAC patient with EGFR L858R + A871G combined mutation. Following surgery, the patient underwent treatment with the first-generation EGFR-TKI, gefitinib, and achieved an impressive 5-year progression-free survival (PFS). This suggests that gefitinib may serve as an effective treatment option for PEAC patients with EGFR L858R + A871G compound mutations.

Bilateral Pulmonary Enteric Adenocarcinoma on FDG PET/CT.

Pulmonary enteric adenocarcinoma(PEAC) is a rare variant of pulmonary adenocarcinoma. We herein report FDG PET/CT findings of PEAC in a 76-year-old woman. The images showed widespread opacities in both lungs with intense FDG uptake. The pathology revealed pulmonary enteric adenocarcinoma.

Clinical features and prognosis of pulmonary enteric adenocarcinoma: A retrospective study in China and the SEER database.

Pulmonary enteric adenocarcinoma (PEAC) is a rare subtype of pulmonary adenocarcinoma that lacks effective treatment. The purpose of this research was to investigate the clinical characteristics, treatment, and prognosis of PEAC, as well as the impact of relevant factors on survival, thus providing a reference for the clinical management of patients with this disease. For this study, we gathered clinical data from 26 patients with PEAC in the Affiliated Cancer Hospital of Zhengzhou University from June 2014 to June 2021. We used SEER*Stat software V8.3.5 to download the PEAC patients from the Surveillance, Epidemiology, and End Results (SEER) database. In total, 20 patients were identified. Clinical data, including general information, imaging findings, and treatment protocols, were obtained, together with a follow-up of disease regression. The relevant clinical data were then analyzed. It included 12 males and 14 females out of 26 patients from China, whose mean age was (62.73 ± 11.89) years; 20 were in the lower lung, 11 were stage I-II, and 15 were stage III-IV. Five had EGFR mutations, and four had KRAS mutations. In terms of treatment, patients with stage I-II were primarily treated by surgery, and patients with stage III-IV were treated mostly by chemotherapy. We extended the follow-up date to January 2022. On completion of the follow-up visit, 11 patients died, and the remaining 15 patients survived. The overall survival (OS) of 26 patients was 2.0-76.0 months, while the mean was 53.1 months, and the median OS (mOS) was 38.0 months (95% CI:1.727-74.273). In the case of progression-free survival (PFS) times, it was 2.0-76.0 months, with a mean PFS of 31.0 months and a median PFS (mPFS) of 8.0 months (95% CI:4.333-11.667). The PFS of the 15 patients in stage III-IV was 2.0-17 months, while the mean PFS was 6.5 months and the mPFS was 6.0 months (95% CI:4.512-7.488). Out of the 20 patients identified in the SEER database, the average age was 69.9 years, with 14 males and 6 females. Of these patients, 8 were diagnosed with stage I-II, while the remaining 11 were diagnosed with stage III-IV. 10 underwent surgery, 4 received radiation therapy, and 9 received chemotherapy. The mean OS of the 20 patients was 67.5 months, mOS was 28.0 months (95% CI: 9.664- 46.336). For patients diagnosed with stage III-IV, the mean OS was 14.8 months and mOS was 20 months (95% CI: 4.713-35.287). PEAC is rare, and the prognosis is determined mainly by the stage; patients who undergo surgery in stage I-II have a better prognosis.

Genomic and transcriptomic insights into the precision treatment of pulmonary enteric adenocarcinoma

BackgroundPulmonary enteric adenocarcinoma (PEAC) is a rare subtype of lung adenocarcinoma. More investigations about precision therapy in PEAC were required to improve the prognosis. Methods: Twenty-four patients with PEAC were enrolled in this study. Tumor tissue samples were available from 17 patients for both DNA and RNA based next-generation sequencing, PD-L1 IHC staining and PCR-based microsatellite instability (MSI) analysis. Results: TP53 (70.6%) and KRAS (47.1%) were the most frequently mutated genes in PEAC. For KRAS mutations, the prevalence of G12D (37.5%) and G12V (37.5%) was higher than G12A (12.5%) and G12C (12.5%). Actionable mutations in receptor tyrosine kinase (including one EGFR and two ALK mutations), PI3K/mTOR, RAS/RAF/MEK, homologous recombination repair (HRR) and cell cycle signaling pathways were identified in 94.1% of patients with PEAC. While PD-L1 expression was observed in 17.6% (3/17) patients, no MSI-H patients were identified. Transcriptomic data showed that two patients with positive PD-L1 expression had relatively high immune infiltration. In addition, prolonged survival was obtained with the treatment of osimertinib, ensartinib, and immunotherapy combined with chemotherapy in two EGFR-mutated, one ALK-rearranged, and one PD-L1 expressed patients, respectively. Conclusion: PEAC is a disease of genetic heterogeneity. The administration of EGFR and ALK inhibitors was effective in patients with PEAC. PD-L1 expression and KRAS mutation type may be used as predictive biomarkers for immunotherapy in PEAC. This study provided both theoretical basis and clinical evidence for PEAC.

Pulmonary Enteric Adenocarcinoma in a Young Man.

Pulmonary Enteric Adenocarcinoma in a Young Man.

Clinicopathological and genetic analyses of pulmonary enteric adenocarcinoma

AimsPulmonary enteric adenocarcinoma (PEAC) is a rare variant of pulmonary adenocarcinoma. Due to its rarity, few pathological and molecular studies have been performed on PEAC. We herein conducted clinicopathological, immunohistochemical...

A Case of Pulmonary Enteric Adenocarcinoma with Interstitial Pneumonia Showing Various Consolidations

A Case of Pulmonary Enteric Adenocarcinoma with Interstitial Pneumonia Showing Various Consolidations

Genomic and epigenomic profiles distinguish pulmonary enteric adenocarcinoma from lung metastatic colorectal cancer.

SummaryBackgroundAs a rare subtype of lung adenocarcinoma, the diagnosis of pulmonary enteric adenocarcinoma (PEAC) remains challenging due to overlapping morphologic spectrum with lung metastatic colorectal cancer (lmCRC). However, the molecular features of PEAC as a separate lung cancer entity are poorly understood.MethodsWe performed whole-exome sequencing and targeted bisulfite sequencing of 32 PEAC and 30 lmCRC to improve differential molecular characterization of the two diseases. We used machine learning methods to select key markers and developed a diagnostic classifier. In addition, we validated the classifier in the internal test cohort and an independently recruited external validation cohort with 17 PEAC and 7 lmCRC.FindingsOur results showed that EGFR was the key driver mutation in PEAC but at a lower prevalence compared to typical lung adenocarcinomas, whereas ERBB2 and KRAS were more frequently observed in PEAC. By contrast, we observed significant enrichment of KRAS and APC mutations in lmCRC compared with PEAC. At the chromosome arm level, copy number variations in 13q, 14q, and 18p were the major chromosomal differences observed between PEAC and lmCRC. Furthermore, by comparing differentially methylated regions (DMRs), we established a neat DNA methylation-based classifier consisting of eight DMRs. This classifier correctly classified all samples in the training cohort and 95% of the samples in the internal test cohort. An external validation cohort of 24 cases recruited from multiple centers in China also reliably agreed with pathological diagnosis.InterpretationThese results provide solid evidence of PEAC-specific genomic characteristics and demonstrate the potential utility of DNA methylation markers for auxiliary diagnosis of PEAC and lmCRC.FundingThis work was supported by National key research and development project 2019YFC1315700, CAMS Key Laboratory of Translational Research on Lung Cancer (2018PT31035), and Beijing Natural Science Foundation (7222144).

Progress in pulmonary enteric adenocarcinoma

Pulmonary enteric adenocarcinoma (PEAC), as a rare histologic subtype of primary lung adenocarcinoma, is defined as an adenocarcinoma in which the enteric component exceeds 50%. It is named after its shared morphological and immunohistochemical features with colorectal cancer. While with such similarity, the differential diagnosis of PEAC and lung metastatic colorectal cancer is a great challenge in the clinic. PEAC may originate from the intestinal metaplasia of respiratory basal cells stimulated by risk factors such as smoking. Current studies have found that KRAS is a relatively high-frequency mutation gene, and other driver gene mutations are rare. In terms of immunohistochemistry, in pulmonary enteric adenocarcinoma, the positive rate was 88.2% (149/169) for CK7, 78.1% (132/169) for CDX2, 48.2% (82/170) for CK20 and 38.8% (66/170) for TTF1. As for clinical features, the average age of onset for pulmonary enteric adenocarcinoma was 62 years, male patients accounted for 56.5% (35/62), smokers accounted for 78.8% (41/52), and 41.4% (24/58) of the primary lesion was located in the upper lobe of the right lung. In terms of treatment, conventional non-small cell lung cancer (NSCLC) regimens rather than colorectal cancer regimens are now recommended. There is still an urgent need for more basic and clinical research, in-depth exploration of its molecular feature and pathogenesis from the level of omics and other aspects, to help diagnosis and differential diagnosis, and find the optimal chemotherapy regimen, possibly effective targeted therapy and even immunotherapy.

Pulmonary Enteric Adenocarcinoma Lung-Case Series and Approach to Diagnosis

Pulmonary Enteric Adenocarcinoma Lung-Case Series and Approach to Diagnosis

Genetic mutation profiles and immune microenvironment analysis of pulmonary enteric adenocarcinoma

BackgroundPulmonary enteric adenocarcinoma (PEAC) has distinctive clinical outcomes, radiographic, pathological and molecular characteristics. The prognosis of patients with PEAC was poor. However, molecular profiles and therapeutic biomarkers of PEAC remain elusive.MethodsIn the present study, the hospitalized patients with PEAC admitted to Tongji Hospital in Wuhan from January 1, 2014 to November 20, 2020 were retrospectively enrolled and followed until December 10, 2020. Comprehensive genomic profiling of tumor tissue from the PEAC patients were performed and compared with lung adenocarcinoma, colorectal cancer and metastatic colorectal carcinoma. Tumor immune microenvironment analysis were evaluated.ResultsThere were 10 patients with PEAC enrolled. 70% of patients were male and the median age of onset was 63 years (interquartile range, 55–72). There were six early-stage patients (Stage IA to IIB) and four stage IV patients. Molecular analysis revealed the most common gene mutations included TP53 (57%, 4/7) and KRAS (57%, 4/7) mutations. There were 40% mutations occurred in genes encoding receptor tyrosine kinases (RTKs). 100% of patients (8/8) were microsatellite stability (MSS). The median level of TMB was 6.0 (interquartile range, 4.5–7.0) mutations/Mb. Three of 10 patients showed low PD-L1 expression (tumor proportion score < 10%) and the others were PD-L1 negative. A small subset of CD8+, CD3+, CD68+ T cells were observed and were mainly distributed in the cancer stroma.ConclusionThis study demonstrated that PEAC was characterized by low-frequency RTK gene mutation, high KRAS mutation, low PD-L1 expression, low TMB, and low CD8+ T cells infiltration.

TTF-1 Immunoexpression in Primary Rectal Adenocarcinoma with Brain Metastasis

Abstract Introduction/Objective Rectal adenocarcinoma metastatic to the brain occurs in 0.6%-3% of cases and is associated with advanced-stage disease.TTF-1 expression in rectal adenocarcinoma is an uncommon finding and less than five cases of TTF-1 positive rectal adenocarcinomas have been reported in the literature. Herein, we report a primary rectal adenocarcinoma with biopsy-proven brain metastasis, radiographic evidence of hepatic and pulmonary involvement and unique expression of TTF-1, a marker with high sensitivity for primary pulmonary and thyroid lesions. Furthermore, we discuss the importance of distinguishing this entity from Pulmonary Enteric Adenocarcinoma (PEA). Methods/Case Report A 68-year-old male with hypertension presented with a two-day history of left facial drooping and dysarthria. Brain MRI revealed a 2.9 cm, contrast-enhancing, solitary, right insular mass. A pterional craniotomy and gross total resection of the lesion was performed. Microscopic examination revealed metastatic adenocarcinoma with immunohistochemical expression of CAM 5.2, CDX2, CK20, focal immunoreactivity with Napsin A and TTF-1, and lack of expression of CK7 and Synaptophysin. Radiographic investigation revealed a right posterior rectal lesion and multiple hepatic and bilateral pulmonary nodules. Sigmoidoscopy a fungating, partially circumferential, ulcerated, and friable rectal mass extending 6 cm proximally from the anal verge. Biopsy demonstrated invasive well- differentiated rectal adenocarcinoma with microsatellite stable phenotype, expression of CDX2, CD20, TTF-1 in the lesional cells, and lack of immunostaining with CK7. Given these findings, we favored a diagnosis of invasive well- differentiated rectal adenocarcinoma. Results (if a Case Study enter NA) N/A Conclusion TTF-1 positive rectal adenocarcinoma is an important differential diagnosis for PEA. As the two primary lesions share histomorphological features, clinical history, radiological findings and immunohistochemical staining with CK7 can aid in distinguishing between the two entities. Recent literature suggests a possible role for CDH17 and SATB2 immunostaining to increase the sensitivity and specificity of distinction between the two entities. Lack of expression of CK7 in both the rectal and brain lesion biopsies, radiological finding of numerous bilateral pulmonary infiltrates and one large, solitary rectal mass supports the diagnosis of advanced stage primary rectal adenocarcinoma with distal metastasis in our patient.

Diagnosis and treatment of primary pulmonary enteric adenocarcinoma: Report of Six cases

BACKGROUNDPrimary pulmonary enteric adenocarcinoma (PEAC) is a very rare subtype of invasive adenocarcinoma, and there have been no large studies on PEAC to date. Therefore, it is necessary to obtain much more information about the clinical and pathological features, diagnosis, differential diagnosis, and treatment of PEAC.CASE SUMMARYAll clinical data of six patients with confirmed PEAC from 2013 to 2018 were collected, and data on diagnosis, differential diagnosis, and treatment of PEAC are discussed combined with all the associated literature. The mean age of six patients was 64.0 ± 5.6 (59-73) years old. Their clinical manifestations were heterogeneous, and during their disease course, there were no gastrointestinal symptoms. There was no evidence from colonoscopy or imaging studies to suggest digestive tract tumors or new metastases. The most commonly mutated gene was KRAS (50.0%), and the pathological features of the six cases were similar to those of colorectal cancer. CDX2 (83.3%) and CK7 (66.7%) had the highest positive rates upon immunohistochemical examination. In the associated literature, 252 cases were identified, and the most commonly mutated gene was KRAS (42.9%). Additionally, CDX2 (68.3%) and CK7 (85.8%) had the highest positive rates. Patients mainly received surgery, chemotherapy, and radiotherapy, immunotherapy was not included.CONCLUSIONPositive results for CDX2 and CK7 play an important role in the diagnosis and differential diagnosis of PEAC, and immunotherapy or targeted therapy focused on KRAS needs to be further studied for the treatment of PEAC.

Clinicopathological and molecular features of pulmonary enteric adenocarcinoma

Objective: To investigate the clinicopathological and molecular characteristics of pulmonary enteric adenocarcinoma (PEAC). Methods: The clinical and pathological data of 19 cases of PEAC in the Affiliated Cancer Hospital of Zhengzhou University were retrospectively collected from 2015 to 2019. Immunohistochemistry (IHC) was used to detect the relevant immunophenotypes, amplification refractory mutation system (ARMS) and fluorescence in situ hybridization (FISH) were used to detect the expression of EGFR, KRAS and ALK genes. The patients were followed up, and the relevant literature was reviewed and analyzed. Results: There were 19 cases, including 10 males and 9 females, with a mean age of 58 years (range 33-71 years). Microscopically, the tumors showed moderately to highly differentiated adenoid and/or papillary growth patterns. The tumor cells were highly columnar and sometimes showed pseudostratification. Inflammatory necrosis and scattered nuclear fragmentation were seen in some glandular lumens. IHC showed variable expression of CK7 (19/19), TTF1 (8/19), Napsin A (6/19), villin (17/19), CK20 (16/19) and CDX2 (10/19). Molecular testing showed KRAS mutation in nine cases (9/19), EGFR mutation in one case (1/19), and positive ALK split signal in one case (1/19). In the literature, the reported mutation rate of KRAS in PEAC was much higher than that of EGFR and ALK. All 19 cases underwent surgical resection and 11 cases were subjected to chemotherapy or radiotherapy. Conclusions: PEAC is a rare variant of invasive pulmonary adenocarcinoma, and has similar histological and cytological features to that of colorectal adenocarcinoma. However, detailed medical history, histologic heterogeneity, an IHC combination of CK7(+)/villin(+) and high KRAS mutation rate are the key points of diagnosis. The prognosis needs long-term follow-up and big data statistics.

Pulmonary enteric adenocarcinoma: a literature review.

Pulmonary enteric adenocarcinoma (PEAC) is an extremely rare type of non-small cell lung cancer (NSCLC) with a histologic pattern that mimics metastatic colorectal cancer (MCC). The main clinical symptoms in PEAC patients are dyspnoea, coughing, hemoptysis, and chest and back pain. The first article about PEAC appeared in 1991 in the form of a case report. As a variant of invasive lung carcinoma, only a small number of case reports and clinical research studies have been carried out, and the only one guidance on diagnosis and treatment is the WHO Tumor Classification book. It is important for doctors to distinguish PEAC from MCC to extend survival time and improve the quality of life. We reviewed the existing literature regarding the diagnosis, treatment, and prognosis of PEAC to provide some valuable clinical references.

Genomic and epigenomic profiles to distinguish pulmonary enteric adenocarcinoma from lung metastatic colorectal cancer.

e13528 Background: As an extremely rare variant of lung adenocarcinoma, the diagnosis of pulmonary enteric adenocarcinoma (PEAC) remains challenging in the clinic due to shared morphological and immunohistochemical features with lung metastatic colorectal cancer (mCRC). Current differentiation of PEAC and mCRC mainly relies on clinical history and pathological examination while which still remain risks of misdiagnosis. Due to their distinct treatment regimens, effective molecular markers are essential for accurate diagnosis. However, comprehensive molecular features of PEAC is still poorly understood. Methods: We performed whole-exome sequencing and targeted bisulfite sequencing of 23 PEAC and 20 mCRC and matched normal tissue to improve molecular characterization. For DNA methylation profiling, differentially methylated regions (DMR) were analyzed by comparing PEAC with normal lung tissue and with mCRC. We also trained machine learning methods to distinguish PEAC from mCRC and validated the classifier in an independent cohort with 10 PEAC and 10 mCRC. Results: Mutations of KRAS, APC, and EGFR, alterations of chromosome arms 13q, 14q and 18p were found to be the major differential genetic alterations between PEAC and mCRC (P &lt; 0.05), yet not enough to aid clinical diagnosis. For epigenomic profile, we identified 524 DMRs (false discovery rate ≤0.05) which were further reduced to 30 DMRs according to importance rank by the random forest algorithm. Based on these DMR features, we developed a diagnostic classifier that correctly classified 95.1% of patients in this discovery cohort. We further validated this predictive model in the validation cohort, with a prediction accuracy of 90.0%. We demonstrated its clinical application in two cases with difficulties to diagnosis by traditional methods. Conclusions: We have illustrated the unique genetic and methylation profiles of PEAC and mCRC. Our approach for disease classification may have a substantial impact on diagnostic precision and therapeutic decision for PEAC.