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DOI: 10.1055/a-0859-7012
TTF-1 positive atypical carcinoid of the epiglottis mimicking medullary thyroid carcinoma
Imitation eines medullären Schilddrüsenkarzinoms durch ein TTF-1 positives atypisches Karzinoid der EpiglottisPublikationsverlauf
Publikationsdatum:
11. April 2019 (online)
![](https://www.thieme-connect.de/media/10.1055-s-00034924/201903/lookinside/thumbnails/10-1055-a-0859-7012-1.jpg)
Introduction
Primary neuroendocrine tumors of the larynx are a very rare disease comprising only ~0.6 % of laryngeal neoplasms [1]. According to the WHO [2], neuroendocrine tumors of the larynx are subdivided into paragangliomas, typical carcinoids, atypical carcinoids, small cell carcinomas (neuroendocrine type) and combined small cell carcinomas (neuroendocrine type). Atypical carcinoids are the most frequent neuroendocrine tumors of the larynx and are rather aggressive tumors with frequent lymph node metastases. They usually occur in older men with a history of heavy smoking and have a 5-year survival rate of only 50 % [3]. Immunohistochemistry is usually positive for chromogranin as well as for synaptophysin and shows a frequent overexpression of calcitonin [2]. Therefore, atypical carcinoids can be confounded with metastases or ectopic manifestations of the far more frequent medullary thyroid carcinoma (MTC) for which calcitonin is the most specific marker. MTCs show positivity for chromogranin and synaptophysin alike [2]. They account for 1–2 % of all thyroid carcinoma and occur either sporadically or hereditary, often between the fifth or sixth decade of life. Prognosis depends on tumor size and the presence of lymph node or distant metastases. Therefore, 10-year survival rates range between 100–71 % in local disease and are only 21 % in advanced disease setting [4].
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References
- 1 Wenig BM, Hyams VJ, Heffner DK. Moderately differentiated neuroendocrine carcinoma of the larynx. A clinicopathologic study of 54 cases. Cancer 1988; 15 (62) 2658-76
- 2 Barnes L, Eveson JW, Reichart P. et al. Pathology and genetics. Head and neck tumours. World Health Organization classification of tumours. 2005. IARC Press; Lyon: 135-139
- 3 Van der Laan TP, Plaat BE, van der Laan BF. et al. Clinical recommendations on the treatment of neuroendocrine carcinoma of the larynx: A meta-analysis of 436 reported cases. Head & Neck 2015; 37: 707-715
- 4 Wells SA, Asa SL, Dralle H. et al. Revised American Thyroid Association guidelines for the management of medullary thyroid carcinoma: the American Thyroid Association Guidelines Task Force on medullary thyroid carcinoma. Thyroid 2015; 25 (06) 567-610
- 5 Bozkurt MF, Virgolini I, Balogova S. et al. Guideline for PET/CT imaging of neuroendocrine neoplasms with 68Ga-DOTA-conjugated somatostatin receptor targeting peptides and 18F–DOPA. European journal of nuclear medicine and molecular imaging 2017; 44 (09) 1588-1601
- 6 Klubo-Gwiezdzinska J, Manes RP, Chia SH. et al. Ectopic cervical thyroid carcinoma – Review of the literature with illustrative case series. The Journal of Clinical Endocrinology & Metabolism 2011; 96 (09) 2684-2691
- 7 Hunt JL, Ferlito A, Hellquist H. et al. Differential Diagnosis in Neuroendocrine Neoplasms of the Larynx. Advances in anatomic pathology 2017; 21: 161-168
- 8 Hirsch MS, Faquin WC, Krane JF. Thyroid transcription factor-1, but not p53, is helpful in distinguishing moderately differentiated neuroendocrine carcinoma of the larynx from medullary carcinoma of the thyroid. Modern pathology 2004; 17: 631