Primary neuroendocrine carcinoma of thymus: A rare cause of Cushing′s syndrome
Thymomas constitute majority of the thymic neoplasms. In contrast, neuroendocrine tumors (carcinoid and neuroendocrine carcinoma) of thymus are extremely rare. Thymic carcinoids may present rarely with Cushing′s syndrome due to the ectopic production of adrenocorticotropic hormone (ACTH)....
| Main Authors: | , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wolters Kluwer Medknow Publications
2010-01-01
|
| Series: | Indian Journal of Pathology and Microbiology |
| Subjects: | |
| Online Access: | http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2010;volume=53;issue=1;spage=148;epage=151;aulast=Arora |
| _version_ | 1818844200935882752 |
|---|---|
| author | Arora Raman Gupta Ruchika Sharma Alok Dinda Amit |
| author_facet | Arora Raman Gupta Ruchika Sharma Alok Dinda Amit |
| author_sort | Arora Raman |
| collection | DOAJ |
| description | Thymomas constitute majority of the thymic neoplasms. In contrast, neuroendocrine tumors (carcinoid and neuroendocrine carcinoma) of thymus are extremely rare. Thymic carcinoids may present rarely with Cushing′s syndrome due to the ectopic production of adrenocorticotropic hormone (ACTH). Recognition of this association is imperative for appropriate management of patients. We describe three cases of rare atypical carcinoid tumor (neuroendocrine carcinoma) of the thymus. Case 1, of a 26-year-old man presenting with Cushing′s syndrome, case 2 - a 23-year-old female with Cushingoid features, and Case 3 - a 39-year-old man complaining of progressively worsening dyspnea. Computed tomography (CT) scans of chest in all three patients revealed anterior mediastinal mass. Excision of tumors and histological examination of the three tumors showed a carcinoid tumor with nuclear pleomorphism, increased mitotic activity and focal necrosis. The features suggested a diagnosis of atypical carcinoid tumor in all the three cases. The tumor cells in Cases 1 and 2 showed focal immunohistochemical staining for ACTH. Atypical carcinoid (neuroendocrine carcinoma, well-differentiated and moderately-differentiated) of the thymus is a rare thymic tumor which carries a worse prognosis compared to thymoma and requires aggressive therapy. Hence, an accurate diagnosis is essential. |
| first_indexed | 2024-12-19T05:09:59Z |
| format | Article |
| id | doaj.art-3de78ca1f0c24c418572f480c56790e4 |
| institution | Directory Open Access Journal |
| issn | 0377-4929 0974-5130 |
| language | English |
| last_indexed | 2024-12-19T05:09:59Z |
| publishDate | 2010-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Indian Journal of Pathology and Microbiology |
| spelling | doaj.art-3de78ca1f0c24c418572f480c56790e42022-12-21T20:34:49ZengWolters Kluwer Medknow PublicationsIndian Journal of Pathology and Microbiology0377-49290974-51302010-01-01531148151Primary neuroendocrine carcinoma of thymus: A rare cause of Cushing′s syndromeArora RamanGupta RuchikaSharma AlokDinda AmitThymomas constitute majority of the thymic neoplasms. In contrast, neuroendocrine tumors (carcinoid and neuroendocrine carcinoma) of thymus are extremely rare. Thymic carcinoids may present rarely with Cushing′s syndrome due to the ectopic production of adrenocorticotropic hormone (ACTH). Recognition of this association is imperative for appropriate management of patients. We describe three cases of rare atypical carcinoid tumor (neuroendocrine carcinoma) of the thymus. Case 1, of a 26-year-old man presenting with Cushing′s syndrome, case 2 - a 23-year-old female with Cushingoid features, and Case 3 - a 39-year-old man complaining of progressively worsening dyspnea. Computed tomography (CT) scans of chest in all three patients revealed anterior mediastinal mass. Excision of tumors and histological examination of the three tumors showed a carcinoid tumor with nuclear pleomorphism, increased mitotic activity and focal necrosis. The features suggested a diagnosis of atypical carcinoid tumor in all the three cases. The tumor cells in Cases 1 and 2 showed focal immunohistochemical staining for ACTH. Atypical carcinoid (neuroendocrine carcinoma, well-differentiated and moderately-differentiated) of the thymus is a rare thymic tumor which carries a worse prognosis compared to thymoma and requires aggressive therapy. Hence, an accurate diagnosis is essential.http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2010;volume=53;issue=1;spage=148;epage=151;aulast=AroraAtypical carcinoidcushing′s syndromeneuroendocrine carcinomathymus |
| spellingShingle | Arora Raman Gupta Ruchika Sharma Alok Dinda Amit Primary neuroendocrine carcinoma of thymus: A rare cause of Cushing′s syndrome Indian Journal of Pathology and Microbiology Atypical carcinoid cushing′s syndrome neuroendocrine carcinoma thymus |
| title | Primary neuroendocrine carcinoma of thymus: A rare cause of Cushing′s syndrome |
| title_full | Primary neuroendocrine carcinoma of thymus: A rare cause of Cushing′s syndrome |
| title_fullStr | Primary neuroendocrine carcinoma of thymus: A rare cause of Cushing′s syndrome |
| title_full_unstemmed | Primary neuroendocrine carcinoma of thymus: A rare cause of Cushing′s syndrome |
| title_short | Primary neuroendocrine carcinoma of thymus: A rare cause of Cushing′s syndrome |
| title_sort | primary neuroendocrine carcinoma of thymus a rare cause of cushing x2032 s syndrome |
| topic | Atypical carcinoid cushing′s syndrome neuroendocrine carcinoma thymus |
| url | http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2010;volume=53;issue=1;spage=148;epage=151;aulast=Arora |
| work_keys_str_mv | AT aroraraman primaryneuroendocrinecarcinomaofthymusararecauseofcushingx2032ssyndrome AT guptaruchika primaryneuroendocrinecarcinomaofthymusararecauseofcushingx2032ssyndrome AT sharmaalok primaryneuroendocrinecarcinomaofthymusararecauseofcushingx2032ssyndrome AT dindaamit primaryneuroendocrinecarcinomaofthymusararecauseofcushingx2032ssyndrome |