Medullary thyroid carcinoma in children: current state of the art and future perspectives

2021 ◽  
Vol 0 (0) ◽  
Author(s):  
Andreas Kiriakopoulos ◽  
Anastasia Dimopoulou ◽  
Constantinos Nastos ◽  
Dimitra Dimopoulou ◽  
Konstantina Dimopoulou ◽  
...  
Keyword(s):  
Lymph Node ◽  
Thyroid Carcinoma ◽  
Medullary Thyroid Carcinoma ◽  
Lymph Node Dissection ◽  
Locally Advanced ◽  
Biological Behavior ◽  
Prophylactic Surgery ◽  
Node Dissection ◽  
Serum Calcitonin ◽  
Medullary Thyroid

Abstract Medullary thyroid carcinoma (MTC) is a distinct type of malignant thyroid tumor in cell origin, biological behavior, and natural history. It accounts for 1.6% of all thyroid cancers and presents either sporadically or as a hereditary disease, the latter occurring as a part of multiple endocrine neoplasia (MEN) 2A and MEN2B syndromes or as a familial MTC disease with no other manifestations. The gene responsible for the hereditary form is the rearranged during transfection (RET) gene, a proto-oncogene located to human chromosome 10. Most pediatric MTC cases have been discovered after genetic testing investigations, leading to the concept of prophylactic surgery in presymptomatic patients. Therefore, the genetic status of the child, along with serum calcitonin levels and ultrasonographic findings, determine the appropriate age for prophylactic surgical intervention. Nevertheless, a diagnosis at an early stage of MTC warrants total thyroidectomy and central lymph node dissection with the addition of lateral/contralateral lymph node dissection depending on the tumor size, ultrasonographic evidence of neck disease, or calcitonin levels. Conversely, locally advanced/unresectable or metastatic MTC is primarily treated with multikinase inhibitors, while more specific RET inhibitors are being tested in clinical trials with promising results.

scholarly journals Complications after medullary thyroid carcinoma surgery: multicentre study of the SQRTPA and EUROCRINE® databases

2021 ◽  
Vol 108 (6) ◽  
pp. 691-701
Author(s):  
D -J van Beek ◽  
M Almquist ◽  
A O Bergenfelz ◽  
T J Musholt ◽  
E Nordenström ◽  
...  
Keyword(s):  
Risk Factors ◽  
Lymph Node ◽  
Thyroid Carcinoma ◽  
Medullary Thyroid Carcinoma ◽  
Lymph Node Dissection ◽  
Node Dissection ◽  
Lateral Lymph Node ◽  
Medullary Thyroid ◽  
Curative Therapy ◽  
Central Lymph Node Dissection

Abstract Background Surgery is the curative therapy for patients with medullary thyroid carcinoma (MTC). In determining the extent of surgery, the risk of complications should be considered. The aim of this study was to assess procedure-specific outcomes and risk factors for complications after surgery for MTC. Methods Patients who underwent thyroid surgery for MTC were identified in two European prospective quality databases. Hypoparathyroidism was defined by treatment with calcium/active vitamin D. Recurrent laryngeal nerve (RLN) palsy was diagnosed on laryngoscopy. Complications were considered at least transient if present at last follow-up. Risk factors for at-least transient hypoparathyroidism and RLN palsy were identified by logistic regression analysis. Results A total of 650 patients underwent surgery in 69 centres at a median age of 56 years. Hypoparathyroidism, RLN palsy and bleeding requiring reoperation occurred in 170 (26·2 per cent), 62 (13·7 per cent) and 17 (2·6 per cent) respectively. Factors associated with hypoparathyroidism were central lymph node dissection (CLND) (odds ratio (OR) 2·20, 95 per cent c.i. 1·04 to 4·67), CLND plus unilateral lateral lymph node dissection (LLND) (OR 2·78, 1·20 to 6·43), CLND plus bilateral LLND (OR 2·83, 1·13 to 7·05) and four or more parathyroid glands observed (OR 4·18, 1·46 to 12·00). RLN palsy was associated with CLND plus LLND (OR 4·04, 1·12 to 14·58) and T4 tumours (OR 12·16, 4·46 to 33·18). After compartment-oriented lymph node dissection, N0 status was achieved in 248 of 537 patients (46·2 per cent). Conclusion Complications after surgery for MTC are procedure-specific and may relate to the unavoidable consequences of radical dissection needed in some patients.

scholarly journals Identification of occult metastases of medullary thyroid carcinoma by calcitonin measurement in washout fluid from fine needle aspiration of cervical lymph node

2009 ◽  
Vol 53 (4) ◽  
pp. 479-481 ◽  
Author(s):  
Debora Siqueira ◽  
Andreia Possati Rocha ◽  
Marcia Khaled Puñales ◽  
Ana Luiza Maia
Keyword(s):  
Lymph Node ◽  
Thyroid Carcinoma ◽  
Medullary Thyroid Carcinoma ◽  
Fine Needle Aspiration ◽  
Residual Disease ◽  
Needle Aspiration ◽  
Illustrative Case ◽  
Serum Calcitonin ◽  
Medullary Thyroid ◽  
Fine Needle

Medullary thyroid carcinoma (MTC) may occur sporadically or as a manifestation of an autosomal-dominant inherited syndrome, the multiple endocrine neoplasia type 2. DNA-based RET genotype analysis gained worldwide acceptance in the identification of asymptomatic gene carrier. MTC synthesize and secrete calcitonin, a well established tumor marker and postoperative level of serum calcitonin, indicates whether residual disease was left behind and whether reintervention is necessary. However, management is difficult when routine imaging studies for MTC are negative. This paper brings a report of an illustrative case of a patient with MTC diagnosed by molecular screening, who persisted with detectable levels of serum calcitonin after surgical procedure. After 48 months, an increase in serum calcitonin impelled us to investigate the disease focus. Cervical-US and calcitonin measurement in washout fluid from fine needle aspiration was successfully used to identify MCT metastasis in a lymph node, allowing appropriated reintervention and illustrating the potential clinical applicability of this method.

scholarly journals FNA of misclassified primary malignant neoplasms of the thyroid: Impact on clinical management

CytoJournal ◽  
2009 ◽  
Vol 6 ◽  
pp. 1 ◽  
Author(s):  
William C. Faquin ◽  
Roberto Izquierdo ◽  
Kamal K. Khurana
Keyword(s):  
Lymph Node ◽  
Thyroid Carcinoma ◽  
Lymph Node Dissection ◽  
Clinical Management ◽  
Medullary Carcinoma ◽  
Malignant Neoplasms ◽  
Node Dissection ◽  
Histologic Diagnosis ◽  
Serum Calcitonin ◽  
Additional Surgery

Background: Fine needle aspiration (FNA) cytology is a popular, reliable and cost effective technique for the diagnosis of thyroid lesions. The aim of our study was to review cases of misclassified primary malignant neoplasms of the thyroid by FNA, and assess the causes of cytologic misdiagnosis and their impact on clinical management. Methods: Clinical data, FNA smears and follow-up surgical specimens of cases diagnosed with primary thyroid carcinoma were reviewed. Results: Of the 365 cases with a malignant diagnosis by FNA over a period of 11 years, nine (2.4 %) were identified with discrepant histologic diagnosis with regard to the type of primary thyroid malignancy. In addition, four cases were added from the consultation files of the Massachusetts General Hospital. Areas of difficulty contributing to misclassification included overlapping cytologic features (n = 6), rarity of tumors (n = 3), and sampling limitations (n = 4). Of the 13 cases, 12 underwent total or near total thyroidectomy and one patient had concurrent surgical biopsy. Measurement of serum calcitonin levels in one case, with an initial cytologic diagnosis of medullary carcinoma, prevented unnecessary lymph node dissection. Misclassification of medullary carcinoma as papillary carcinoma precluded lymph node dissection in one case. Further management decisions were based on the final histologic diagnosis and did not require additional surgery. Two cases of undifferentiated (anaplastic) thyroid carcinoma were misdiagnosed as papillary thyroid carcinoma. Both patients received total thyroidectomies, which may not otherwise have been performed. Conclusions: A small subset of primary malignant neoplasms of the thyroid may be misclassified with regard to the type of malignancy on FNA. The majority of primary malignant neoplasms diagnosed on FNA require thyroidectomy. However, initial cytologic misclassification of medullary carcinoma or undifferentiated carcinoma as other malignant neoplasms or vice versa may have an impact on clinical management.

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