Medullary Carcinoma of the Thyroid

Medullary thyroid cancer (MTC) is a distinct C-cell tumor of the thyroid. We review the oncogenesis and management of both sporadic tumors and those tumors arising as part of specific inherited syndromes. The RET proto-oncogene plays a role in the development of inherited forms of MTC and has become important in the clinical management of patients and their families. The recognition of the high rate of regional nodal involvement has led to lymphadenectomy being strongly considered for patients undergoing thyroidectomy for MTC.

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Recurrent Medullary Carcinoma detected by Gallium-68 Positron Emission Tomography

World Journal of Endocrine Surgery ◽

10.5005/jp-journals-10002-1128 ◽

2013 ◽

Vol 5 (2) ◽

pp. 59-60

Author(s):

Cigdem Soydal ◽

Mine Araz ◽

Ozlem N Kucuk ◽

Elgin Ozkan ◽

Taner Demirer

Keyword(s):

Positron Emission Tomography ◽

Thyroid Cancer ◽

Medullary Thyroid Cancer ◽

Medullary Carcinoma ◽

Emission Tomography ◽

Thyroid Cancer Patient ◽

Medullary Thyroid ◽

Positron Emission ◽

Pet Ct ◽

Gallium 68

ABSTRACT In this case, we would like to share our experience of a recurrent medullary thyroid cancer patient whose recurrence was detected by Ga-68 DOTATATE PET/CT. How to cite this article Soydal C, Ozkan E, Araz M, Kucuk ON, Demirer T. Recurrent Medullary Carcinoma detected by Gallium-68 Positron Emission Tomography. World J Endoc Surg 2013;5(2):59-60.

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Twenty years of lesson learning: how does theRETgenetic screening test impact the clinical management of medullary thyroid cancer?

Clinical Endocrinology ◽

10.1111/cen.12686 ◽

2014 ◽

Vol 82 (6) ◽

pp. 892-899 ◽

Cited By ~ 32

Author(s):

Cristina Romei ◽

Alessia Tacito ◽

Eleonora Molinaro ◽

Laura Agate ◽

Valeria Bottici ◽

...

Keyword(s):

Thyroid Cancer ◽

Clinical Management ◽

Screening Test ◽

Medullary Thyroid Cancer ◽

Medullary Thyroid ◽

Test Impact

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Clinical Management of a Patient with a Locally Recurrent Medullary Thyroid Cancer and Asymptomatic Slowly Progressing Distant Metastases

Thyroid Cancer ◽

10.1007/978-3-030-61919-0_36 ◽

2020 ◽

pp. 327-335

Author(s):

Virginia Cappagli ◽

Valeria Bottici ◽

Rossella Elisei

Keyword(s):

Thyroid Cancer ◽

Clinical Management ◽

Medullary Thyroid Cancer ◽

Distant Metastases ◽

Medullary Thyroid ◽

Locally Recurrent

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Medullary Thyroid Cancer

Cancer Control ◽

10.1177/107327489700400103 ◽

1997 ◽

Vol 4 (1) ◽

pp. 25-29

Author(s):

Michael G. Franz

Keyword(s):

Thyroid Cancer ◽

Surgical Treatment ◽

Medullary Thyroid Cancer ◽

Early Stage ◽

Medullary Carcinoma ◽

Early Stage Disease ◽

Medullary Thyroid ◽

Central Lymph Node Dissection ◽

Familial Tumors ◽

Thyroid Malignancies

Background Medullary thyroid cancer, a tumor of the parafollicular C cells, accounts for approximately 10% of all thyroid malignancies. An estimated 75% of cases are sporadic, and the remaining 25% are familial. Methods The author reviews the clinical features and diagnostic tests for this entity, as well as the surgical treatment of recurrent or persistent medullary carcinoma. Results Sporadic medullary thyroid cancer typically presents as an isolated unilateral mass. Familial tumors tend to be multifocal and bilateral. In patients with multiple endocrine neoplasia type 2A, pheochromocytomas and parathyroid hyperplasia also may develop. Care is taken to avoid operating on a patient with occult pheochromocytoma. Total thyroidectomy and central lymph node dissection are the keys for successful surgical treatment. Conclusions Surgery is the cornerstone of treatment for medullary carcinoma of the thyroid. Genetic testing using the ret oncogene allows identification of individuals who are at risk for the disease or those with early-stage disease.

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Results of a screening program for c-cell disease (medullary thyroid cancer and c-cell hyperplasia)

Cancer ◽

10.1002/1097-0142(19900401)65:7<1570::aid-cncr2820650720>3.0.co;2-0 ◽

1990 ◽

Vol 65 (7) ◽

pp. 1570-1576 ◽

Cited By ~ 18

Author(s):

W. John Simpson ◽

John S. Carruthers ◽

Dina Malkin

Keyword(s):

Thyroid Cancer ◽

Medullary Thyroid Cancer ◽

Screening Program ◽

Cell Disease ◽

Cell Hyperplasia ◽

Medullary Thyroid ◽

C Cell Hyperplasia ◽

C Cell

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Measurement of Basal Serum Calcitonin for the Diagnosis of Medullary Thyroid Cancer

Hormone and Metabolic Research ◽

10.1055/s-0043-122237 ◽

2017 ◽

Vol 50 (01) ◽

pp. 23-28 ◽

Cited By ~ 5

Author(s):

Stephanie Allelein ◽

Margret Ehlers ◽

Corinna Morneau ◽

Katharina Schwartz ◽

Peter Goretzki ◽

...

Keyword(s):

Thyroid Cancer ◽

False Positive ◽

Medullary Thyroid Cancer ◽

False Negative ◽

Cell Hyperplasia ◽

Serum Calcitonin ◽

Medullary Thyroid ◽

C Cell Hyperplasia ◽

Basal Serum ◽

C Cell

AbstractCalcitonin (CT), a tumor marker for medullary thyroid cancer (MTC), can be stimulated with pentagastrin or calcium. Because of the unavailability of pentagastrin, basal CT measurement is frequently used for the preoperative diagnosis of MTC. The aim of the study was to define basal serum calcitonin (bCT) cut-off thresholds for diagnosing MTC. Within a retrospective analysis, 114 patients (51 males) were included fulfilling the criteria of an increased preoperative bCT level (>10 pg/ml) and the criteria of an available postoperative histology analysis. Based on a ROC plot analysis, the cut-off values for the diagnosis of MTC vs. non-malignancy (C cell hyperplasia and goiter) were identified. The most precise bCT thresholds for the identification of MTC were ≥46 pg/ml for males (sensitivity: 93.6%, specificity: 95.0%, PPV: 97%, NPV: 90%) and ≥35 pg/ml for females (sensitivity: 87.3%, specificity: 87.5%, PPV: 98%, NPV: 50%). Using these cut-offs, only 6% of male patients were not identified of having MTC, whereas 5% were false positive (having instead C cell hyperplasia). In females, the discrepancy was higher since 13% of female MTC patients were false negative by using the cut-off of ≥35 pg/ml, and 13% had false positive results (suffering from C cell hyperplasia). Gender-specific bCT cut-offs for the identification of MTC vs. C cell hyperplasia and non-malignancy were defined, which can be used in clinical routine. In female patients, however, the accuracy is much lower compared to males.

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Importance of gender-specific calcitonin thresholds in screening for occult sporadic medullary thyroid cancer

Endocrine Related Cancer ◽

10.1677/erc-09-0136 ◽

2009 ◽

Vol 16 (4) ◽

pp. 1291-1298 ◽

Cited By ~ 47

Author(s):

Andreas Machens ◽

Florian Hoffmann ◽

Carsten Sekulla ◽

Henning Dralle

Keyword(s):

Thyroid Cancer ◽

Medullary Thyroid Cancer ◽

Cell Mass ◽

Cell Hyperplasia ◽

Predictive Values ◽

Medullary Thyroid ◽

C Cell Hyperplasia ◽

C Cell ◽

Gender Specific ◽

Better Than

Men and women differ in thyroidal C-cell mass and calcitonin secretion. This difference may have implications for the definition of calcitonin thresholds to distinguish sporadic C-cell hyperplasia from occult medullary thyroid cancer. This retrospective study examined the hypothesis that gender-specific calcitonin thresholds predict occult medullary thyroid cancer more accurately among patients with increased basal calcitonin levels than unisex thresholds. A total of 100 consecutive patients were evaluated with occult sporadic C-cell disease no larger than 10 mm who were referred for increased basal calcitonin levels and underwent pentagastrin stimulation preoperatively at this institution. Altogether, gender-specific calcitonin thresholds predicted medullary thyroid cancer better than unisex thresholds. At lower (≤50 pg/ml basally; ≤500 pg/ml after stimulation), but not higher, calcitonin serum levels, women revealed medullary thyroid cancer four to eight times more often than men. Most discriminatory between C-cell hyperplasia and medullary thyroid cancer was a basal calcitonin threshold of 15 pg/ml (corrected 20 pg/ml) for women and 80 pg/ml (corrected 100 pg/ml) for men, based on the greatest accuracy at the lowest possible calcitonin level. The respective gender-specific stimulated peak calcitonin thresholds were 80 pg/ml (corrected 100 pg/ml) and 500 pg/ml. Corresponding positive predictive values for medullary thyroid cancer at these calcitonin thresholds were 89 and 90% for women, as opposed to 100% for men. To increase the positive predictive value for women to 100%, the respective calcitonin thresholds would have to be raised to 40 pg/ml (corrected 50 pg/ml) and 250 pg/ml. These findings indicate that gender-specific calcitonin thresholds predict sporadic occult medullary thyroid cancer better than unisex thresholds.

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Clinical Management of a Patient with a Locally Recurrent Medullary Thyroid Cancer and Asymptomatic Slowly Progressing Distant Metastases

Thyroid Cancer ◽

10.1007/978-3-319-22401-5_40 ◽

2016 ◽

pp. 347-354 ◽

Cited By ~ 1

Author(s):

Virginia Cappagli ◽

Valeria Bottici ◽

Rossella Elisei

Keyword(s):

Thyroid Cancer ◽

Clinical Management ◽

Medullary Thyroid Cancer ◽

Distant Metastases ◽

Medullary Thyroid ◽

Locally Recurrent

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Histopathological and molecular studies in patients with goiter and hypercalcitoninemia: reactive or neoplastic C-cell hyperplasia?

Endocrine Related Cancer ◽

10.1677/erc-06-0053 ◽

2007 ◽

Vol 14 (2) ◽

pp. 393-403 ◽

Cited By ~ 22

Author(s):

Uberta Verga ◽

Stefano Ferrero ◽

Leonardo Vicentini ◽

Tatiana Brambilla ◽

Valentina Cirello ◽

...

Keyword(s):

Thyroid Cancer ◽

Multinodular Goiter ◽

Medullary Thyroid Cancer ◽

Final Diagnosis ◽

C Cells ◽

Cell Hyperplasia ◽

Medullary Thyroid ◽

C Cell Hyperplasia ◽

C Cell ◽

Molecular Studies

The cut-off values able to differentiate between reactive or neoplastic C-cell hyperplasia (CCH) or to predict sporadic medullary thyroid cancer (MTC) are still debated both for basal and stimulated calcitonin (bCT and sCT). In the present study, the prevalence and the histological patterns of CCH in 15 patients with multinodular goiter (MNG), bCT>10 pg/ml and sCT levels >50 pg/ml were studied. As controls, 16 patients with MNG and bCT levels <10 pg/ml and 4 patients with familial (FMTC) were included. For each case, calcitonin (CT) immunoreactive cells were counted in 60 consecutive high-power fields (400×) and CCH classified as focal, diffuse, nodular, or neoplastic. RET genetic analyses were performed at the germline and tissue levels in MTC and CCH cases. In patients with MNG, sCT levels >50 pg/ml were associated with CCH or MTC, being the total number of C-cells/60 fields significantly higher than that found in MNG with normal bCT (P = 0.0008) and comparable with that detected in FMTCs. In the group with sCT>50 pg/ml, the C-cells displayed a neoplastic phenotype. Neither germline nor somatic RET mutations were found. In conclusion, sCT levels >50 pg/ml were always associated with CCH, without correlation between CT levels and the number of C-cells or the final diagnosis. The C-cells had a morphology and distribution pattern similar to those observed in FMTC. Thus, sCT levels >50 pg/ml indicate the presence of CCH with a possible preneoplastic potential, suggesting the opportunity to perform a prophylactic surgical treatment.

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