open access publication

Article, 2024

A rare case report of a primary lung cancer comprising adenocarcinoma and atypical carcinoid tumor, with the carcinoid component harboring EML4-ALK rearrangement

Translational Lung Cancer Research, ISSN 2226-4477, 2218-6751, Volume 13, 5, Pages 1150-1162, 10.21037/tlcr-24-352

Contributors

Hu, Wenbin [1] Zhao, Jiaming [1] Wang, Guoxia [1] Wang, Qihao [1] Deng, Mingming [1] Shen, Jie [2] Hofman, Paul [3] Urbanska, Edyta Maria 0000-0002-4578-9346 [4] Santoni-Rugiu, Eric 0000-0002-2283-3535 [4] Christopoulos, Petros 0000-0002-7966-8980 [5] Ramirez, Robert A. [6] Hida, Toyoaki [7] Lu, Xiaoqing [1] He, Binjun (Corresponding author) [1]

Affiliations

  1. [1] Shaoxing University
    2. [NORA names: China; Asia, East];
  2. [2] Dagong Law Firm, Shaoxing, China, ;
    3. [NORA names: China; Asia, East];
  3. [3] Hôpital Pasteur
    4. [NORA names: France; Europe, EU; OECD];
  4. [4] Rigshospitalet
    5. [NORA names: Capital Region of Denmark; Hospital; Denmark; Europe, EU; Nordic; OECD];
  5. [5] University Hospital Heidelberg
    6. [NORA names: Germany; Europe, EU; OECD];

Abstract

Background: The occurrence of pulmonary adenocarcinoma coexisting with atypical carcinoid tumors is a rare phenomenon. The presence of EML4-ALK fusion in an atypical carcinoid component of a histologically mixed tumor is even more uncommon. Due to their infrequency, the origin and pathogenesis of these mixed tumors remain largely unknown. The advances of therapy development in such patients are still limited and there is no standard treatment. We present a case of collision tumor in the lung consisting of atypical carcinoid and adenocarcinoma to better understand the clinical characteristics of this disease. Case Description: We report an extremely rare case of EML4-ALK rearrangement in a pulmonary atypical carcinoid tumor that coexisting with adenocarcinoma. A 58-year-old woman, who was asymptomatic, underwent pulmonary lobectomy due to the detection of a gradually enlarging solitary pulmonary nodule in the right upper lung. Histological examination of the resected tumor revealed the presence of both atypical carcinoid (approximately 80%) and adenocarcinoma (approximately 20%) components. Metastases by the carcinoid component were observed in mediastinal lymph nodes (station 2R and 4R) and in the primary tumor. Anaplastic lymphoma kinase (ALK) rearrangement was detected in both the primary and metastatic lesions of the carcinoid tumor. Four cycles of chemotherapy with etoposide and carboplatin were dispensed after surgery. Conclusions: This is the first reported case of coexisting pulmonary adenocarcinoma and atypical carcinoid tumor with an ALK fusion only detected in the carcinoid component. The presence of ALK rearrangement in pulmonary carcinoid tumor is very uncommon, and there is currently no standard treatment for advanced stages. Therefore, comprehensive molecular testing, including ALK rearrangement analysis, should be recommended for mixed tumors exhibiting features of atypical carcinoid. ALK inhibitors could represent a potential treatment strategy for selected patients.

Keywords

EML4-ALK rearrangement, adenocarcinoma, advanced stage, analysis, anaplastic lymphoma kinase, atypical carcinoid, atypical carcinoid tumor, cancer, carboplatin, carcinoid, carcinoid component, carcinoid tumors, case of collision tumor, case report, cases, characteristics, chemotherapy, clinical characteristics, collision tumor, components, comprehensive molecular testing, cycle, cycles of chemotherapy, detection, development, disease, etoposide, examination, features, fusion, histological examination, histology, infrequency, inhibitors, kinase, lesions, lobectomy, lung, lung cancer, lymph nodes, mediastinal lymph nodes, metastasis, metastatic lesions, mixed tumors, molecular testing, nodes, nodules, occurrence, origin, pathogenesis, patients, phenomenon, potential treatment strategy, presence, primary lung cancer, primary tumor, pulmonary adenocarcinoma, pulmonary atypical carcinoid tumor, pulmonary carcinoid tumors, pulmonary lobectomy, pulmonary nodules, rare case, rare case report, rare phenomenon, rearrangement, rearrangement analysis, reports, resected tumor, right upper lung, solitary pulmonary nodules, stage, standard treatment, strategies, surgery, test, therapy, therapy development, treatment, treatment strategies, tumor, upper lung, women

Data Provider: Digital Science

LINKS
-

Matching Records in NORA

SUBJECTS
+

METRICS
+