scholarly journals Medullary thyroid carcinoma: Sporadic, hereditary

2003 ◽  
Vol 50 (3) ◽  
pp. 113-120
Author(s):  
Ivan Paunovic ◽  
Aleksandar Diklic ◽  
Ksenija Krgovic ◽  
Vladan Zivaljevic ◽  
Svetislav Tatic ◽  
...  
Keyword(s):  
Lymph Nodes ◽  
Total Thyroidectomy ◽  
Thyroid Tumor ◽  
Endocrine Surgery ◽  
Medullary Thyroid ◽  
Complex Approach ◽  
Mean Size ◽  
General Data ◽  
Central Lymph ◽  
Worse Prognosis

Medullary thyroid cancer (MTC) is uncommon thyroid tumor with specific characteristics which undoubtedly divide this tumor from other thyroid malignances. Patients with sporadic or hereditary form of MTC differ in clinical presentation, recurrence of the disease and outcome. The aim of study was to establish surgical characteristics of MTC as well as clinical factors that influence surgical treatment. The study group consisted of 68 patients with MTC managed at the Center for Endocrine Surgery between 1987. and 1999. Retrospective analysis included clinical form of the disease, general data, histological and other tumor characteristics. Mean age of the patients were 47.3 years (female/male ratio: 1.5:1). Mean size of tumor was 80.5 cm3, 72.1% patients had tumor greater than 4 cm. in diameter or extrathyroid spread. The majority of patients were in II and III stadium of the disease. Primary operation (at least total thyroidectomy) was performed in 57 (84%) patients. 2(3%) had postoperatively temporally nerve palsy and 7(10.29%) temporally hypoparathyroidism. The overall survival was 46.8 + 9.9% after 9 years and 63.6 + 7.2% at 5 years. Postoperative calcitonin value is significant predictor of survival /Spearman's coefficient (R=0.7048)/, worse prognosis is in correlation with high postoperative calcitonin values. The treatment of choice is at least total thyroidectomy and central lymph nodes resection if enlarged lymph nodes are found. Precise operative technique lowers the risk of postoperative complications. Complex approach to the patient with MTC includes all available methods in pre and postoperative evaluation as well as surgeon's knowledge and skill.

scholarly journals SUN-921 Indolent Presentation of Medullary Thyroid Cancer in a Patient with MEN Type 2B Due to a Germline RET M918T Mutationo

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Stephanie Kim ◽  
Victoria C Hsiao ◽  
Carolyn Seib ◽  
Jessica Erin Gosnell ◽  
Chienying Liu
Keyword(s):  
Thyroid Cancer ◽  
Lymph Nodes ◽  
Total Thyroidectomy ◽  
Medullary Thyroid Cancer ◽  
Whole Body ◽  
Nodal Metastases ◽  
Medullary Thyroid ◽  
Phenotypic Features ◽  
Men 2B ◽  
Positive Lymph Nodes

Abstract Background: MEN type 2B is rare and most commonly due to a germline methionine-to-threonine substitution at codon 918 (M918T) of the RET proto-oncogene. Medullary thyroid cancer (MTC) occurs in 100% of the patients affected with the mutation. This mutation is considered the highest risk and is typically associated with aggressive disease and worse overall survival. We describe a case of a late diagnosis of MEN 2B in a patient and his son, both with a relatively indolent presentation of MTC. Clinical Case: A 39-year-old man presented to an outside institution with difficult to control hypertension, headaches and anxiety and was found to have bilateral pheochromocytomas (left, 5.8 x 5.5 x 3.8 cm and right, 9 x 5.2 x 7.3 cm). Upon presentation to our institution, he was noted to have classic phenotypic features of MEN 2B with a marfanoid habitus and multiple mucosal neuromas. Genetic testing confirmed RET M918T mutation. His family history was negative for similar features in his parents and siblings. However, one of his three children, age 12, had similar phenotypic features and was found to have the same mutation. The patient subsequently underwent a successful bilateral adrenalectomy and pathology confirmed pheochromocytomas. Thyroid ultrasound showed multiple nodules with calcifications but no lateral nodal metastases. Calcitonin and carcinoembryonic antigen (CEA) levels were elevated (170 pg/mL, normal ≤10, and 180.4 ug/L, normal <3.8, respectively). He underwent a total thyroidectomy and bilateral central node dissection, with pathology confirming bilateral MTC (2.7 cm and 1.0 cm), metastatic in 4 of 10 positive lymph nodes (largest focus 2 mm). Whole body PET/CT post-operatively did not show metastatic disease. The patient’s son also had multiple thyroid nodules on ultrasound without lateral nodal metastases and elevated calcitonin and CEA levels (3015 pg/ml, normal ≤10, and 433 ng/mL, normal <2.5, respectively). MRI of the abdomen and pelvis was negative for pheochromocytomas. He underwent total thyroidectomy and bilateral central neck dissection, with pathology showing bilateral MTC (2.7 cm and 1.0 cm) with 0 of 14 positive lymph nodes. For both the patient and his son, calcitonin and CEA levels normalized following thyroidectomy and surveillance over a year later reveals no evidence of disease. Conclusion: Early diagnosis of MEN type 2B is important as MTC develops early in life and is the leading cause of death in these patients. When diagnosed early, prophylactic thyroidectomy in childhood is indicated and can improve long-term survival. There are salient phenotypic features associated with this disease which were unfortunately not recognized early in this patient and his son. Fortunately, their MTC presentations appear to be relatively indolent despite their late diagnoses, and they will continue to be closely monitored for recurrent disease.

scholarly journals What Lies Beneath? - Medullary Thyroid Cancer in Nodular Graves’ Disease

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A904-A904
Author(s):  
Rongzhi Wang ◽  
Shirisha R Vallepu ◽  
Herbert Chen ◽  
Rajasree Nambron
Keyword(s):  
Thyroid Carcinoma ◽  
Total Thyroidectomy ◽  
Medullary Thyroid Carcinoma ◽  
The United States ◽  
Endocrine Surgery ◽  
Graves Disease ◽  
Hormone Production ◽  
Thyroid Cancers ◽  
Medullary Thyroid ◽  
Central Lymph Node Dissection

Abstract Background: Medullary thyroid carcinoma (MTC) accounts for 1%-2% of thyroid cancers in the United States (US). MTC originates from thyroid parafollicular C-cells, occurring either sporadically or hereditarily as part of type 2 multiple endocrine neoplasia (MEN) or familial MTC (FMTC). Hyperthyroidism is prevalent in approximately 1.2%, and Graves’ Disease (GD) is the most common cause of hyperthyroidism in the US. GD is an autoimmune disorder that results in increased thyroid hormone production due to the stimulation of TSH receptor by thyrotropin receptor antibodies (TRAb). Thyroid carcinoma in general is uncommon in GD patients, while MTC is extraordinarily rare. We report a case of sporadic MTC in GD, which is extremely rare. A recent publication stated that there were only 15 reported cases of MTC coexisting in GD until 2019. Clinical Case: A 62-year-old male with hypertension, diabetes and obesity presented to the endocrine surgery clinic with symptoms of diaphoresis, chest pain and fullness, shortness of breath, and palpitations. The patient had a brother with a pancreatic mass of unknown pathology and multiple family members with thyroid disease. On physical exam, there was a palpable non-tender left-sided nodule with no lymphadenopathy. His FT4 was 8.5 (0.76-1.46 ng/dL), TSH was <0.006 (0.36-3.74 μIU/dL) and thyroid-stimulating antibody was 1.25 (<0.10 IU/L). Ultrasound showed a multinodular goitre with a dominant nodule in the left lower pole measuring 2.3x1.9x1.5 cm. He was diagnosed with GD, treated with methimazole, and his symptoms improved. Subsequent nuclear medicine uptake scan showed diffusely increased uptake and a cold nodule in the left thyroid lobe. Repeat labs showed low TSH (<0.01μIU/dL) and elevated FT4, CEA, calcitonin, PTH, metanephrine, and normetanephrine (1.04 ng/dl, 5.2 (3-5 ng/ml), 796 (<18 pg/ml), 58.10 (18.40-88.00 pg/mL), 90 (< OR = 57 pg/ml) and 222 (< OR = 148 pg/ml), respectively). A thyroid fine-needle aspiration (FNA) was suggestive of MTC (Bethesda Category VI). Total thyroidectomy with left central lymph node dissection revealed a 2.5cm MTC confined in the left lobe with focal lymphovascular invasion. His postoperative course was uneventful, and CEA and calcitonin levels trended down (1.1 ng/ml and <2 pg/mL, respectively). Conclusion: The coexistence of medullary thyroid carcinoma and Graves’ Disease is rare, incidental, with five-year survival rates lower than other thyroid cancers. A delayed diagnosis of MTC would be detrimental. Patients with thyroid nodules and GD are five times more likely to be diagnosed with thyroid carcinoma. Radiation, chemotherapy and thyroidectomy are standard treatment options for MTC, with total thyroidectomy being the preferred option. It is necessary to evaluate patients with GD for possible thyroid cancers, especially in the presence of nodules.

scholarly journals Predictors of Central Compartment Involvement in Patients with Positive Lateral Cervical Lymph Nodes according to Clinical and/or Ultrasound Evaluation

2021 ◽  
Vol 10 (15) ◽  
pp. 3407
Author(s):  
Giuseppa Graceffa ◽  
Giuseppina Orlando ◽  
Gianfranco Cocorullo ◽  
Sergio Mazzola ◽  
Irene Vitale ◽  
...  
Keyword(s):  
Lymph Nodes ◽  
Univariate Analysis ◽  
Roc Curves ◽  
Central Compartment ◽  
Central Neck Dissection ◽  
Level Of Evidence ◽  
Cervical Lymph Nodes ◽  
Kaplan Meier ◽  
Ultrasound Evaluation ◽  
Central Lymph

Lymph node neck metastases are frequent in papillary thyroid carcinoma (PTC). Current guidelines state, on a weak level of evidence, that level VI dissection is mandatory in the presence of latero-cervical metastases. The aim of our study is to evaluate predictive factors for the absence of level VI involvement despite the presence of metastases to the lateral cervical stations in PTC. Eighty-eight patients operated for PTC with level II–V metastases were retrospectively enrolled in the study. Demographics, thyroid function, autoimmunity, nodule size and site, cancer variant, multifocality, Bethesda and EU-TIRADS, number of central and lateral lymph nodes removed, number of positive lymph nodes and outcome were recorded. At univariate analysis, PTC location and number of positive lateral lymph nodes were risk criteria for failure to cure. ROC curves demonstrated the association of the number of positive lateral lymph nodes and failure to cure. On multivariate analysis, the protective factors were PTC located in lobe center and number of positive lateral lymph nodes < 4. Kaplan–Meier curves confirmed the absence of central lymph nodes as a positive prognostic factor. In the selected cases, Central Neck Dissection (CND) could be avoided even in the presence of positive Lateralcervical Lymph Nodes (LLN+).

scholarly journals Predictors of thyroglobulin in the lymph nodes recurrence of papillary thyroid carcinoma undergoing total thyroidectomy

BMC Surgery ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Zhichao Xing ◽  
Yuxuan Qiu ◽  
Zhe Li ◽  
Lingyun Zhang ◽  
Yuan Fei ◽  
...  
Keyword(s):  
Papillary Thyroid Carcinoma ◽  
Thyroid Carcinoma ◽  
Lymph Nodes ◽  
Total Thyroidectomy ◽  
Radioactive Iodine ◽  
Independent Predictor ◽  
Central Neck Dissection ◽  
American Thyroid Association ◽  
Papillary Thyroid ◽  
Serum Thyroglobulin

Abstract Background To investigate the association between postoperative lymph nodes (LNs) recurrence and distinct serum thyroglobulin (Tg) levels in patients with papillary thyroid carcinoma (PTC). Methods This study included PTC patients who underwent total thyroidectomy (TT) with at least central neck dissection and then re-operated due to recurrence of LNs between January 2013 and June 2018. These patients were grouped by negative or positive serum Tg levels according to the American Thyroid Association guidelines. Results Of the 60 included patients, 49 underwent radioactive iodine (RAI) treatment. Maximum unstimulated Tg (uTg) ≥ 0.2 ng/mL were associated with larger diameter of recurrent LNs (P = 0.027), and higher rate of metastatic LNs (P < 0.001). Serum-stimulated Tg (off-Tg) ≥ 1 ng/mL (P = 0.047) and unstimulated Tg (on-Tg) ≥ 0.2 ng/Ml (P = 0.013) were associated with larger diameter of recurrent LNs. Number of metastatic LNs ≥ 8 was an independent predictor for postoperative maximum uTg ≥ 0.2 ng/mL (OR = 8.767; 95% CI = 1.392–55.216; P = 0.021). Ratio of metastatic LNs ≥ 25% was an independent predictor for off-Tg ≥ 1 ng/mL (OR = 20.997; 95% CI = 1.649–267.384; P = 0.019). Conclusion Postoperative Tg-positive status was associated with larger size of recurrent LNs. Number of metastatic LNs ≥ 8 and ratio of metastatic LNs ≥ 25% were independent predicators for uTg-positive and off-Tg-positive status, respectively.

Detection of normal mediastinal lymph nodes by ultrasonography

1997 ◽  
Vol 38 (6) ◽  
pp. 965-969 ◽  
Author(s):  
C. F. Dietrich ◽  
M. Liesen ◽  
R. Buhl ◽  
G. Herrmann ◽  
J. Kirchner ◽  
...  
Keyword(s):  
High Resolution ◽  
Lymph Nodes ◽  
Pathological Finding ◽  
Doppler Imaging ◽  
Colour Doppler Imaging ◽  
Asymptomatic Volunteers ◽  
Human Cadavers ◽  
Mean Size ◽  
Almost All ◽  
Node Diameter

Purpose: the detection by US (ín contrast to CT) of lymph nodes of any size in the mediastinum is usually considered to be a pathological finding. the aim of this study was to find out whether it was possible to detect normal lymph nodes by high-resolution mediastinal US Material and Methods: Six different mediastinal regions in 80 healthy asymptomatic volunteers and in 20 human cadavers were examined by means of US (with colour Doppler imaging) to assess US access to the respective regions and to demonstrate the number and size of detectable lymph nodes. All the cadaveric lymph nodes that were detected were examined histologically to exclude inflammatory or malignant infiltration Results: in almost all subjects, we obtained US access to the supra-aortic (100%), paratracheal (95%), prevascular (99%), and pencardial (98%) regions, and to the aorticopulmonary window (98%). US access to the subcarinal region was more difficult (75%). in the healthy subjects, lymph nodes were detected in the paratracheal region (in 35% of these subjects, mean lymph-node diameter 12×7 mm), in the aorticopulmonary window (45%, 14×8 mm), and in the subcarinal region (13%, 13×7 mm). in the cadavers, histologically normal lymph nodes were detected frequently in the paratracheal region (85%, mean size 11×6 mm) and in the aorticopulmonary window (90%, 11×5 mm) Conclusion: These results indicate that normal lymph nodes (and not only pathological lymph nodes) can be demonstrated by high-resolution mediastinal US

scholarly journals Surgical approach to medullary thyroid cancer

2007 ◽  
Vol 51 (5) ◽  
pp. 818-824 ◽  
Author(s):  
Catharina Ihre Lundgren ◽  
Leigh Delbridg ◽  
Diana Learoyd ◽  
Bruce Robinson
Keyword(s):  
Thyroid Cancer ◽  
Total Thyroidectomy ◽  
Medullary Thyroid Cancer ◽  
Prophylactic Surgery ◽  
Surgical Removal ◽  
Serum Calcitonin ◽  
Medullary Thyroid ◽  
Central Lymph Node Dissection ◽  
Familial Form ◽  
Men 2A

Medullary thyroid cancer (MTC) compromises 3-5% of all thyroid cancers and arises from parafollicular or calcitonin-producing C cells. It may be sporadic (75% of cases), or may occur as a manifestation of either the hereditary syndrome Multiple Endocrine Neoplasia type 2 (MEN 2A or MEN 2B) (25% of cases), or rarely as an isolated familial syndrome (FMTC). Complete surgical resection comprising in most cases total thyroidectomy with central lymph node dissection at an early stage of the disease is the only potential cure for MTC. The familial form of the disease, MEN-2A occupies a unique place in surgical history, having been the first disease where surgical removal of an affected organ was undertaken before the development of malignancy, solely on the basis of genetic testing. Total thyroidectomy prior to the development of invasive cancer completely avoids an otherwise lethal malignancy. Timing of prophylactic surgery is based on models that utilise genotype-phenotype correlations, which have now been stratified into three risk groups based on the specific codon involved. MTC should be followed with postoperative serial serum calcitonin levels to survey for persistent or recurrent disease as indicated by detectable levels. The challenge however, if calcitonin levels are increased, is to find the source of its production. The first localisation technique recommended would be ultrasound of the neck, since there is a high frequency of local recurrence and cervical node metastasis, followed by a total body CT scan and bone scintigraphy.

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