Intermediate-Grade Neuroendocrine Mediastinal Tumor a 38-Years- Old Man: Case Report and Literature Review
Abstract
Introduction. Neuroendocrine tumors of the mediastinum (MNET) are rare. They represent 0.4% of all carcinoid tumors and 5% of all anterior mediastinal masses. Clinical case. A 38-year-old man has chest pain, dyspnea on moderate exertion, insomnia, hip pain, nausea and cough, jugular engorgement, plethoric faces, venous network in the thorax. The final diagnosis was intermediate-grade neuroendocrine mediastinal tumor with vena cava syndrome. He was not a candidate for surgery, so he continued medical management with radiotherapy and chemotherapy. He developed spinal syndrome and, finally, heart failure secondary to carcinoid syndrome and chemo toxicity. Discussion: The most common signs are respiratory manifestations, and in some cases, they begin with superior vena cava syndrome. Computerized tomography (CT), magnetic resonance imaging (MRI), immunohistochemistry with chromogranin A and synaptophysin establish the diagnosis. The therapy prescript is surgical resection and systemic therapy with neoadjuvant chemotherapy comprising cytotoxic drugs such as cisplatin/carboplatin as the ideal treatment. New therapies such as Peptide Receptor Radionuclide are helpful for extensive tumors, like systemic chemotherapy based on 177Lu-Dotatate. It is a neoplasm of difficult diagnosis and bad prognosis; with timely treatment, patients have a better quality of life.
Abbreviations: MNET (Mediastinal Neuroendocrine Tumor), ECOG (Eastern Cooperative Oncology Group), CgA (Chromogranin A).
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2. Quiroz Gutiérrez Fernando. Human anatomy. Volume III. Respiratory, Digestive, and Genitourinary Apparatus. Glands of internal secretion and Organs of the senses. Editorial Porrua, S.A. Mexico. Forty-third edition, 2012.
3. Patnaik S, Malempati A, Uppin M, Susarla R. Rare mediastinal masses - Imaging review. J Can Res Ther [Internet]. 2021; 17: 13-21.Available in: https://www.cancerjournal.net/text.asp?2021/17/1/13/297202.
4. Oronsky B, Ma P, Morgensztern D, Carter C. Nothing But NET: A Review of Neuroendocrine Tumors and Carcinomas. Neoplasia [Internet]. 2017;19(12):991-1002. Available in: https://www.sciencedirect.com/science/article/pii/S1476558617303470?via%3Dihub
5. Kosmas K, Vamvakais I, Klapsinou E, Psychogiou E, Riga D. Primary Mediastinal Neuroendocrine Tumor: A Case of Atypical Carcinoid. Cureus [Internet] 2021; 22;13(1):e12853. Available in: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7897398/
6. B Melosky. Advanced typical and atypical carcinoid tumors of the lung: management recommendations. Current Oncology. [Internet]. 2018;(Suppl 1): S86-S93. Available in: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6001761/
7. Fukushima T, Gomi D, Seno N, Gibo T, Kobayashi T, Sekiguchi N. Successful Salvage Chemotherapy with Streptozocin in a Patient with Mediastinal Atypical Carcinoid Tumor Who Had Relapsed after Various Prior Therapies. Case Reports in Oncology [Internet]. 2018;11(1):49-54. Available in: https://www.karger.com/Article/FullText/477163
8. Póvoa S, Azevedo D, Marques C, Barroca H, Costa A. Unknown primary large-cell neuroendocrine tumor. Autopsy Case Rep [Internet]. 2018;8(2):e2018025. Available in: http://dx.doi.org/10.4322/acr.2018.025
9. Adnan, Aadil; Kudachi, Shreyas; Ramesh, Sudha; Prabhash, Kumar; Basu, Sandip.Metastatic or locally advanced mediastinal neuroendocrine tumors: outcome with 177 Lu-DOTATATE-based peptide receptor radionuclide therapy and assessment of prognostic factors. [Internet]. 2019. Volumen 40 - Numero 9 - pp 947-957. Available in: https://journals.lww.com/nuclearmedicinecomm/Abstract/2019/09000/Metastatic_or_locally_advanced_mediastinal.11.aspx
10. Schwartz LH, Litière S, De Vries E. RECIST 1.1. Update and classification: From the RECIST committee. Eur J Cancer [Internet]. 2016;62:132-137. Available in: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5737828/pdf/nihms926904.pdf
11. Azam F, Latif MF, Farooq A, et al. Performance Status Assessment by Using ECOG (Eastern Cooperative Oncology Group) Score for Cancer Patients by Oncology Healthcare Professionals. Case Rep Oncol. [Internet] 2019;12(3):728-736. Available in: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6792426/pdf/cro-0012-0728.pdf
12. Ventura L, Gnetti L, Silini E, Rindi G, Carbognani P, Rusc. Primary atypical carcinoid tumor of the mediastinum: a very rare finding. Journal of Thoracic Disease. [Internet]. 2017;9(4):E367-E372. Available in: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5418266/
13. Costanzo L, Kewan, T, Kerwin K Daw H. Primary Mediastinal Small Cell Neuroendocrine Carcinoma Presenting with Superior Vena Cava Syndrome. Cureus. [Internet]. 2019. 11 (6), e4873. Available in: http://dx.doi.org/10.7759/cureus.4873
14. Bodei L, Sundin A, Kidd M, Prasad V, Modlin IM. The status of neuroendocrine tumor imaging: from darkness to light? Neuroendocrinology. [Internet]. 2015;101(1):1-17. Available in: https://pubmed.ncbi.nlm.nih.gov/25228173/
15. Rabiou S, Lakranbi M, Ghizlane T, Elfatemi H, Serraj M, Ouadnouni Y. Quelle chirurgie pour quelle tumeur du médiastin : expérience du service de chirurgie thoracique de CHU Hassan II de Fès. Revue de Pneumologie Clinique [Internet]. 2017;73(5):246-252. Available in: https://www.sciencedirect.com/science/article/pii/S0761841716301626
16. Diaz-Perez J, Currás-Freixes M. Chromogranin A and neuroendocrine tumors. Rev Endocrinología y Nutrició. [Internet]. 2013; Vol(7):pp386-395. Available in: https://www.elsevier.es/es-revista-endocrinologia-nutricion-12-articulo-cromogranina-a-tumores-neuroendocrinos-S1575092212003221
17. Kyriakopoulos G, Mavroeidi V, Chatzellis E, Kaltsas G y Alexandraki, K. Histopathological, immunohistochemical, genetic and molecular markers of neuroendocrine neoplasms. Annals of Translational Medicine [Internet], 2018; 6 (12), 252-252. Available in: https://atm.amegroups.com/article/view/19995/html
18. Nardini M, Dunning J, Migliore M, Cerfolio R. Robotic resection of a middle mediastinal mass. Journal of Visualized Surgery [Internet]. 2018;2018(4):113-113. Available in: http://jovs.amegroups.com/article/view/19627/19607
19. HidakaT, Okuzumi S, Matsuhashi A, Takahashi H, Hata K, Shimizu S and Iwasaki Y. Large Cell Neuroendocrine Carcinoma of the Mediastinum Successfully Treated with Systemic chemotherapy after Palliative Radiotherapy. Inter Med (Tokio, Japón). [Internet]. 2019. 58 (4), 563–568.Available in: http://dx.doi.org/10.2169/internalmedicine.0437-17
20. Basu S, Thapa P, Parghane R. 177Lu-DOTATATE peptide receptor radionuclide therapy in metastatic or advanced and inoperable primary neuroendocrine tumors of rare sites. World Journal of Nuclear Medicine [Internet]. 2017;16(3):223. Available in: http://www.wjnm.org/article.asp?issn=1450-1147;year=2017;volume=16;issue=3;spage=223;epage=228;aulast=Thapa
21. Hamaji M, Allen M, Cassivi S. The role of surgical management in recurrent thymic tumors. Ann Thorac Surg [Internet]. 2012; 94(1):247-254.Available in:https://www.annalsthoracicsurgery.org/action/showPdf?pii=S0003-4975%2812%2900454-7
22. Maurizi G, D'Andrilli A, Vanni C. Salvage resection of advanced mediastinal tumors. J Thorac Dis [Internet]. 2019;9;11(Suppl 13):S1653-S1661.Available in: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6706622/
Authors
JIMENEZ-BAEZ, M. V., Milián-Saavedra , L. V., Martínez-Castro, . L. R., Sánchez-Tun, . J. A., & Naranjo-Cruz , A. Y. (2021). Intermediate-Grade Neuroendocrine Mediastinal Tumor a 38-Years- Old Man: Case Report and Literature Review. Journal of Current Medical Research and Opinion, 4(06), 967–974. https://doi.org/10.15520/jcmro.v4i06.428
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