Medullary thyroid carcinoma and pregnancy

Medullary thyroid carcinoma (MTC) is a rare neuroendocrine tumor of the parafollicular cells of the thyroid gland. The tumor occurs, either sporadically or in a hereditary form, as a component of the type 2 multiple endocrine neoplasia (MEN) syndromes, MEN2A and MEN2B, both with an autosomal dominant transmission. Due to its higher aggressiveness and urgency to treat, it poses a challenge when diagnosed during pregnancy. We performed a literature review searching medical databases as UpToDate and PubMed for relevant information about diagnosis, impact, treatment, and outcome of medullary thyroid carcinoma during pregnancy. Diagnosis of MTC is established by clinical and ultrasound evaluation, followed by a fine needle aspiration biopsy. Further analyses decide whether it is a sporadic or a hereditary disease. Surgery offers the curative solution, but careful postoperative follow-up is needed for the best outcome. The decision for surgery is taken considering the DNA mutation of RET oncogene and pregnancy status. A premature planned delivery is associated with potential complications for the newborn, but it is recommended when maternal status worsens. Conclusions. Complete resection of the thyroid tumor and any local and regional metastases is the only curative solution. Timing of the intervention is extremely important to decrease fetal outcomes. Close follow-up during the postoperative period, by both biochemical and imaging methods, is needed.

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Serum Calcitonin-Negative Medullary Thyroid Carcinoma: Role of CgA and CEA as Complementary Markers

The International Journal of Biological Markers ◽

10.1177/172460080802300212 ◽

2008 ◽

Vol 23 (2) ◽

pp. 129-131 ◽

Cited By ~ 18

Author(s):

L. Giovanella ◽

S. Crippa ◽

L. Ceriani

Keyword(s):

Thyroid Carcinoma ◽

Medullary Thyroid Carcinoma ◽

Needle Aspiration ◽

Radical Cure ◽

Serum Calcitonin ◽

Medullary Thyroid ◽

Preoperative Ct ◽

Serum Cea

The biochemical activity of medullary thyroid carcinoma (MTC) includes production of calcitonin (CT), chromogranin A (CgA) and carcinoembryonic antigen (CEA). Routine CT measurement has been proposed as part of the initial evaluation of thyroid nodules and its use could ultimately decrease the morbidity and mortality of MTC. We report on a 43-year-old female patient with a large MTC expressing CT, CgA and CEA on immunostains but with negative preoperative CT and CgA results. Serum CEA was slightly increased and its rapid disappearance predicted radical cure by surgery as confirmed by 2-year follow-up. Our report illustrates that a diagnosis of MTC cannot always be excluded by negative preoperative CT. Fine-needle aspiration with cytomorphological analysis and complementary immunocytochemistry remains an essential diagnostic tool. Finally, serum aliquots must be stored before thyroid surgery in order to measure circulating forms of complementary markers found by tissue immunostaining (CEA and CgA)

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Serum Markers Measured in FNA Fluids of Medullary Thyroid Carcinoma Occurring as a Cyst

The International Journal of Biological Markers ◽

10.5301/jbm.5000205 ◽

2016 ◽

Vol 31 (2) ◽

pp. 224-227 ◽

Cited By ~ 5

Author(s):

Pierpaolo Trimboli ◽

Leo Guidobaldi ◽

Nicola Locuratolo ◽

Francesca R. Piro ◽

Marco Giordano ◽

...

Keyword(s):

Thyroid Carcinoma ◽

Medullary Thyroid Carcinoma ◽

Needle Aspiration ◽

Serum Markers ◽

High Serum ◽

Thyroid Lesion ◽

Serum Calcitonin ◽

Medullary Thyroid ◽

Fine Needle ◽

Ultrasound Evaluation

Background Diagnosis of medullary thyroid carcinoma (MTC) is still a challenge, and the measurement of calcitonin in fine-needle aspiration (FNA) washout has been recently introduced. Rarely, MTC presents as a cyst and its preoperative detection is difficult. Methods A 66-year-old woman was referred to us with high serum calcitonin. Ultrasound evaluation showed one cystic and one solid nodule. FNA was performed in both nodules and calcitonin, procalcitonin and CEA were measured in the aspirate fluids. Results The markers showed elevated levels in the FNA washout of the cyst and the patient was operated on. Final histology demonstrated MTC of the cystic nodule. Four weeks later, all serum markers were undetectable. Conclusions In the presence of a cystic thyroid lesion the diagnosis of MTC should be taken into account, and measuring calcitonin in FNA fluid is relevant. The determination in FNA of other markers is technically feasible but its role should be evaluated in larger series.

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Management of Advanced Medullary Thyroid Carcinoma with Vandetanib: Case Series

Endocrinology and Disorders ◽

10.31579/2640-1045/065 ◽

2021 ◽

Vol 5 (1) ◽

pp. 01-07

Author(s):

Andrés Flórez R

Keyword(s):

Partial Response ◽

Adverse Events ◽

Thyroid Carcinoma ◽

Medullary Thyroid Carcinoma ◽

Stable Disease ◽

Case Series ◽

Medullary Thyroid ◽

The Mean ◽

Grade 3

Objective: To describe the tumor response and adverse events in patients with advanced medullary thyroid carcinoma (MTC) treated with vandetanib at the National Cancer Institute in Bogotá, Colombia. Materials and Methods: Case series including five patients with advanced MTC treated with vandetanib from April 2011 to August 2018 and a minimum follow-up of 6 months. Results: 5 patients met the inclusion criteria, including 3 women. The mean age was 49 years. A total of 4 patients underwent total thyroidectomy prior to starting vandetanib. The main indication for vandetanib was progression of liver metastasis (4 patients). Regarding treatment response, 3 patients presented stable disease, 1 patient showed partial response, and 1 had disease progression. The mean treatment duration was 16.5 months. Grade 3 or 4 adverse events were observed in three patients, 1 with diarrhea, 1 with hypertension, and 1 with rash. All symptoms improved with dose reduction or temporary suspension of vandetanib. Conclusions: The management of advanced MTC with vandetanib allows for prolonged disease control (stable disease or partial response). Although adverse events are frequent, most are mild and severe cases are manageable.

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Medullary thyroid carcinoma: recent advances in identification, treatment, and prognosis

Therapeutic Advances in Endocrinology and Metabolism ◽

10.1177/20420188211049611 ◽

2021 ◽

Vol 12 ◽

pp. 204201882110496

Author(s):

Marisa A. Bartz-Kurycki ◽

Omowunmi E. Oluwo ◽

Lilah F. Morris-Wiseman

Keyword(s):

Thyroid Carcinoma ◽

Medullary Thyroid Carcinoma ◽

Distant Metastases ◽

Hereditary Disease ◽

Patient Specific ◽

Medullary Thyroid ◽

Recent Advances ◽

Initial Surgery ◽

Biochemical Testing ◽

Thyroid Malignancies

Medullary thyroid carcinoma (MTC) is a neuroendocrine tumor that represents <5% of all thyroid malignancies and is generally more aggressive than differentiated thyroid cancer. The aim of this study is to provide an update, through review of clinical studies of patients with MTC published between January 1, 2016, and June 1, 2021, on recent advances in the diagnosis and treatment of MTC. This review focuses on updates in biochemical testing, imaging, hereditary disease, surgical management, adjuvant therapies, and prognosis. Recent advances reviewed herein have sought to diagnose MTC at earlier stages of disease, predict when patients with a hereditary syndrome may develop MTC, use functional imaging to assess for distant metastases, perform optimal initial surgery with appropriate lymphadenectomy, employ targeted systemic therapies for patients with progressive metastatic disease, and better predict patient-specific outcomes.

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Medullary thyroid carcinoma

Oxford Textbook of Endocrinology and Diabetes ◽

10.1093/med/9780199235292.003.3356 ◽

2011 ◽

pp. 625-630

Author(s):

Friedhelm Raue ◽

Karin Frank-Raue

Keyword(s):

Sex Ratio ◽

Thyroid Carcinoma ◽

Medullary Thyroid Carcinoma ◽

Hereditary Disease ◽

C Cells ◽

Biochemical Screening ◽

Medullary Thyroid ◽

Histological Features ◽

Sporadic Disease ◽

Male To Female

Medullary thyroid carcinoma (MTC) is a rare calcitonin-secreting tumour of the parafollicular or C cells of the thyroid. As the C cells originate from the embryonic neural crest, MTC often have the clinical and histological features of neuroendocrine tumours. They account for 8–12% of all thyroid carcinomas and occur in both sporadic and hereditary forms (1). The majority of patients have sporadic MTC (70%), while 30% have hereditary MTC. The sex ratio in sporadic MTC is 1:1.3 (male to female), while both sexes are nearly equally affected in the familial variety (2). The highest incidence of sporadic disease occurs in the fifth decade of life, while hereditary disease can be diagnosed earlier, depending on the possibility of genetic and biochemical screening.

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Circulating Neuron-Specific Enolase in Medullary Thyroid Cancer

The International Journal of Biological Markers ◽

10.1177/172460088600100205 ◽

1986 ◽

Vol 1 (2) ◽

pp. 85-88 ◽

Cited By ~ 4

Author(s):

Furio Pacini ◽

Rossella Elisei ◽

Stefano Anelli ◽

Lucia Gasperini ◽

Ernestina Schipani ◽

...

Keyword(s):

Thyroid Carcinoma ◽

Medullary Thyroid Carcinoma ◽

Time Course ◽

Neuron Specific Enolase ◽

High Serum ◽

Large Tumor ◽

Medullary Thyroid ◽

The Mean ◽

Metastatic Involvement

The utility of determining circulating neuron-specific enolase (NSE) in medullary thyroid carcinoma was assessed in 25 patients followed up for a mean period of 45.6 months. In 5 patients tested before any treatment serum NSE concentrations were in the normal range. After total thyroidectomy abnormally high serum NSE concentrations (more than 9.8 ng/ml) were found in 1/3 patients with normal calcitonin (CT) in remission, in 2/10 with elevated CT levels but no evidence of disease and in 9/12 with elevated CT levels and documented metastases. The mean (± SD) NSE value in this last group was 12.0 ± 12.6 ng/ml, significantly higher than in the other groups (p < 0.005). The time course of serum NSE in patients with long follow-up seems to indicate that serum NSE rises when a large tumor mass is present and usually parallels the pattern of circulating CT. Effective treatment of the metastases is usually followed by reduction of serum NSE. Thus, serum NSE can serve as an additional humoral marker for medullary thyroid carcinoma, its elevation being associated with important metastatic involvement and with a poor prognosis of the tumor.

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