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Review
. 2019 Oct 16;19(1):103.
doi: 10.1186/s12902-019-0435-7.

Medullary thyroid carcinoma with double negative calcitonin and CEA: a case report and update of literature review

Claudio Gambardella  1   2 , Chiara Offi  3 , Guglielmo Clarizia  3 , Roberto Maria Romano  3 , Immacolata Cozzolino  4 , Marco Montella  4 , Rosa Maria Di Crescenzo  5 , Massimo Mascolo  5 , Angelo Cangiano  3 , Sergio Di Martino  3 , Giancarlo Candela  3 , Giovanni Docimo  3
Affiliations
Review

Medullary thyroid carcinoma with double negative calcitonin and CEA: a case report and update of literature review

Claudio Gambardella et al. BMC Endocr Disord. .

Abstract

Background: Medullary thyroid carcinoma is a malignant uncommon and aggressive tumour of the parafollicular C cells. In about 75% of cases it is sporadic while, in case of RET mutation, it is associated to multiple endocrine neoplasia type 2 (25% of cases). The biochemical features of medullary thyroid carcinoma include the production of calcitonin and carcinoembryogenic antigen. The above-mentioned features are useful in the diagnostic process as well as in the follow up and in the prognostication of the disease. Even if calcitonin elevation is strongly associated to MTC, it can also be found increased in many pathological different conditions as pregnancy, lactation, C-cells hyperplasia, autoimmune thyroiditis, end stage renal disease, lung and prostate cancer and several neuroendocrine tumours. Major medullary thyroid tumours are usually connected to high doses of circulating calcitonin, in fact non-secretory variants have hardly been described.

Case presentation: We herein report the case of a 59 years old male, who had undergone total thyroidectomy for multinodular goiter with negative preoperative calcitonin, showing medullary thyroid carcinoma at definitive pathology. To the best of our knowledge, this is the first case documenting a non-secretory medullary thyroid carcinoma, with double negative markers at the time of diagnosis and at the relapse.

Conclusion: A Literature review underlining pathological hypothesis, differential diagnosis and alternative and innovative biomarkers to identify non-secretory medullary thyroid carcinoma was carried out.

Keywords: Calcitonin; Carcinoembryogenic antigen; Medullary thyroid carcinoma; Thyroid cancer.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Thyroid definitive pathology with microscopic and immunochemistry evaluation a) Low magnification showed nests of neoplastic cells separated by thick septa of fibrous tissue (haematoxylin and eosin, original magnification, x4); b) The neoplastic cells have a plasmacytoid appearance (haematoxylin and eosin, original magnification, x20); c-f) Phenotypic features with positivity for cytokeratin 19 (c: immunoperoxidase stain for anti-cytokeratin 19, original magnification, x 20), calcitonin (d: immunoperoxidase stain for anti-calcitonin, original magnification, x 20), weak expression of CD56 (e: immunoperoxidase stain for anti-CD56, original magnification, x 20) and negativity for thyroglobulin (f: immunoperoxidase stain for anti-thyroglobulin, original magnification, x 20)
Fig. 2
Fig. 2
Recurrent laterocervical lymph-node definitive pathology. a haematoxylin and eosin, original magnification, × 4. b haematoxylin and eosin, original magnification, × 20
See this image and copyright information in PMC

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