Carcinoids associated with multiple endocrine neoplasia syndromes

Duh, Quan‐Yang; Hybarger, C. Patrick; Geist, Richard E.; Gamsu, G; Goodman, Philip C.; Gooding, Gretchen A. W.; Clark, Orlo H.

doi:10.1016/0002-9610(87)90305-9

Cited by 180 publications

(89 citation statements)

References 17 publications

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“…2,3 Compared with other tumors, neuroendocrine carcinomas are rare in the lung representing about 1% of all primary lung neoplasms 4 and are extremely rare in the thymic gland where they account for o5% of all mediastinal tumors. 5 Similar morphology and routine immunohistochemical markers often do not differentiate between neuroendocrine carcinomas of different locations, thus making it difficult to establish the exact site of origin in cases of metastatic tumors. Therefore, the objective of this study was to compare the staining patterns of two immunohistochemical markers associated with lung and thymic tumors, thyroid transcription factor-1 (TTF-1) and PAX8 in 25 cases of low and intermediate grade neuroendocrine carcinomas of pulmonary and thymic origin, respectively, in order to evaluate their use for the differential diagnosis of these tumors.…”

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confidence: 99%

Comparative immunohistochemical analysis of pulmonary and thymic neuroendocrine carcinomas using PAX8 and TTF-1

et al. 2013

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PAX8 is expressed in thymic epithelial neoplasms and a subset of neuroendocrine carcinomas of gastrointestinal origin but not pulmonary neuroendocrine carcinomas. Thyroid transcription factor 1 (TTF-1) is known to be positive in pulmonary neuroendocrine carcinomas, but studies investigating its expression in thymic neuroendocrine carcinomas are lacking. To date, there are no comprehensive studies focusing on the comparative expression of PAX8 or TTF-1 in pulmonary and thymic neuroendocrine carcinoma. Twenty-five cases of low and intermediate grade neuroendocrine carcinomas of pulmonary and thymic origin, respectively, were selected for immunohistochemical studies using antibodies directed against PAX8 and TTF-1. The percentage of positive tumor cells as well as the intensity of staining were evaluated and scored. Twenty-one of the pulmonary neuroendocrine carcinomas were classified as low grade (typical carcinoid) and 4 as intermediate grade (atypical carcinoid) tumors; the thymic tumors consisted of 8 low grade and 17 intermediate grade neuroendocrine carcinomas. Only 2 (8%) of the pulmonary tumors showed nuclear expression of PAX8 while 19 (76%) expressed TTF-1. Of the thymic tumors, 8 (32%) were positive for PAX8 and 2 (8%) showed TTF-1 positivity. Primary neuroendocrine carcinomas of the thymus are rare neoplasms that display a more aggressive clinical course than pulmonary neuroendocrine carcinomas, highlighting the importance of the separation of these tumors. To date, there are no specific immunomarkers to distinguish between neuroendocrine carcinomas of pulmonary and thymic origin. The differential expression of PAX8 and TTF-1 may prove useful in this context as a PAX8 þ /TTF-1 À immunophenotype appears to be more common in thymic neuroendocrine carcinomas, whereas the reverse (PAX8 À /TTF-1 þ ) is true for most pulmonary neuroendocrine carcinomas.

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confidence: 99%

Comparative immunohistochemical analysis of pulmonary and thymic neuroendocrine carcinomas using PAX8 and TTF-1

et al. 2013

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“…Our index patient presented with assumed PHPT, which is typically the first manifestation of MEN1 and occurs in 95% of cases 18, 19. In addition, he had a NET (formerly known as carcinoid), which have been reported to occur in 4% of MEN1 patients 16, 19, 20. In retrospect, the clinical MEN1 diagnosis was not clear‐cut, with several lines of evidence suggesting that our index patient was most likely a so‐called phenocopy, that is, a phenotype resembling MEN1 without being it 21, 22.…”

Section: Discussionmentioning

confidence: 95%

“…MEN1 is autosomal dominant inherited and is characterized by PHPT, pancreatic tumors, pituitary tumors, and more seldom NETs typically located to the adrenal gland, or derived from the embryonic foregut 16, 17. The clinical diagnosis is considered when more than one organ system is affected.…”

Section: Discussionmentioning

confidence: 99%

Multiple endocrine neoplasia phenocopy revealed as a co‐occurring neuroendocrine tumor and familial hypocalciuric hypercalcemia type 3

Hovden

Jespersen

Nissen

et al. 2016

Clinical Case Reports

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“…Since then, this term has been widely applied to a variety of welldifferentiated neuroendocrine neoplasms at various sites, including the gastrointestinal tract, lung, and thymus (9,55,56). Duh et al (8) stated that thymic carcinoids are clinically malignant in approximately 82% of cases, whereas bronchial carcinoids are malignant in only 26% of cases. Also, Dusmet and McKneally (57) stated that although bronchial carcinoids may not influence life expectancy, thymic carcinoids, regardless of their histopathologic features, have a poor prognosis.…”

Section: Discussionmentioning

confidence: 99%

Primary Neuroendocrine Carcinoma (Thymic Carcinoid) of the Thymus with Prominent Oncocytic Features: A Clinicopathologic Study of 22 Cases

Moran

Suster

2000

Modern Pathology

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Twenty-two cases of oncocytic thymic neuroendocrine carcinomas (carcinoid tumors) are presented. The patients were 17 men and 5 women between the ages of 26 and 84 years (median, 55 years). Nine were asymptomatic, and the tumor was found on routine examination; four patients presented with chest pain, two with weight loss, two with multiple endocrine neoplasia I syndrome, and one with Cushing's syndrome. Surgical resection of the mediastinal tumor was performed in all cases. The lesions were described as soft, light tan to brown, measuring from 3 to 20 cm in greatest diameter. On cut section, the tumors showed a homogeneous surface, soft consistency, and focal areas of hemorrhage. Microscopically, the lesions were characterized by nests or trabeculae of tumor cells that contained abundant granular to densely eosinophilic cytoplasm, with round to oval nuclei and in some areas prominent nucleoli. Mitotic figures ranged from 2 to 10 per 10 high-power fields; foci of comedonecrosis were seen in all cases. Immunohistochemical studies including broad spectrum keratin, CAM 5.2, chromogranin, synaptophysin, Leu-7, and p53 were performed in 12 cases. All of the tumors were strongly positive for CAM 5.2 lowmolecular-weight cytokeratin, 11 showed strong positive reaction for Leu-7, 10 for broad-spectrum keratin, 8 for chromogranin, 7 for synaptophysin, and only 1 case showed focal positive staining of the tumor cells for p53. Clinical follow-up of 14 patients showed that 10 were alive between 2 and 11 years, and 4 patients had died of tumor from 4 to 11 years after diagnosis. Patients with good clinical outcome were those whose tumors showed low mitotic activity and minimal nuclear pleomorphism, whereas those who had died of their tumors were those whose tumors were characterized by marked nuclear atypia and higher mitotic rates. Primary neuroendocrine carcinomas of the thymus are unusual neoplasms that may take the form of either moderately and well-differentiated tumors (thymic carcinoid) or poorly differentiated neoplasms (small cell neuroendocrine carcinoma of the thymus). Thymic neuroendocrine carcinomas may display a diverse spectrum of differentiation. Tumors composed predominantly of spindle cells (1, 2), with mucinous stroma (3), with divergent cell lines including mesenchymal elements and amyloid-like stroma (4 -6), and cases showing transitional areas with small cell carcinoma (7) have been described in the literature. We present a study of 22 cases of another unusual morphologic variant of primary neuroendocrine carcinoma of the thymus characterized by prominent oncocytic features. The clinicopathologic, immunohistochemical, and differential diagnostic features of these lesions are discussed. MATERIALS AND METHODSTwenty-two cases of primary thymic neuroendocrine tumors of the thymus (thymic carcinoid) with prominent oncocytic features were identified from

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Carcinoids associated with multiple endocrine neoplasia syndromes

Cited by 180 publications

References 17 publications

Comparative immunohistochemical analysis of pulmonary and thymic neuroendocrine carcinomas using PAX8 and TTF-1

Comparative immunohistochemical analysis of pulmonary and thymic neuroendocrine carcinomas using PAX8 and TTF-1

Multiple endocrine neoplasia phenocopy revealed as a co‐occurring neuroendocrine tumor and familial hypocalciuric hypercalcemia type 3

Primary Neuroendocrine Carcinoma (Thymic Carcinoid) of the Thymus with Prominent Oncocytic Features: A Clinicopathologic Study of 22 Cases

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