Latent Subclinical Medullary Thyroid Carcinoma: Diagnosis and Treatment (original) (raw)
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The Journal of Clinical Endocrinology & Metabolism, 2004
The survival rate of patients with medullary thyroid carcinoma (MTC) is significantly better in patients diagnosed and treated when the tumor is limited to the thyroid. In a pioneer study carried out in 1991, we demonstrated that routine measurement of serum calcitonin (CT) in nodular thyroid disease allowed the preoperative diagnosis of unsuspected sporadic MTC with better accuracy than routine fine needle aspiration cytology (FNAC). This finding has been confirmed in subsequent studies. In the present study we report the results of CT screening in 10,864 patients with thyroid nodular disease seen in the years 1991-1998 (group 1). We analyzed the prevalence of MTC and compared their outcomes with those of a historical group of patients (group 2) diagnosed before the introduction of CT screening (1970 -1990). The prevalence of MTC found by CT screening in group 1 was 0.40% (44 patients). A positive CT test had a higher diagnostic sensitivity and specificity compared with FNAC. CT screening allowed the diag-nosis of MTC at an earlier stage compared with group 2 (P ؍ 0.004). Normalization of serum CT levels (undetectable) after surgery was more frequently observed in group 1. At the end of follow-up, complete remission was observed in 59% of group 1 and in 2.7% of group 2 (P ؍ 0.
Endocrine, 2014
To investigate whether further, diagnostic procedures should be recommended in patients with slight increase of preoperative serum basal calcitonin (bCT) levels in whom surgical treatment can be recommendable. Fourteen consecutive patients with nodular thyroid disease underwent thyroidectomy in our center for suspected medullary thyroid microcarcinoma (MTC) because their serum bCT levels were slightly higher than the upper limit of normal range. Serum bCT was measured by radioimmunoassay, normality range = 0-20 ng/L. Surgical specimens were examined by the same pathologist using histologic and immunohistochemistry techniques. An extensive search for parafollicular C-cell hyperplasia (CCH) and/or microscopic MTC foci was performed. At preoperative ultrasound, a single thyroid nodule was depicted in three patients while a multinodular goiter in 11. The bCT values ranged between 24.4 and 94.6 ng/L, median 42.2 ng/L while the pentagastrin-stimulated CT (sCT) values by pentagastrin test ranged between 61.5 and 1,262 ng/L, median 245.0 ng/L. Total thyroidectomy was performed in 13 patients, and lobectomy in the other one; central node dissection was also performed in eight cases. At histology, MTC was diagnosed in nine patients (64.3 %), showing a median maximum diameter of 6.1 mm (range, 1.5-17 mm); CCH was diagnosed in the other five patients (35.7 %). The pentagastrin stimulation test was obtained in all patients. It is worth noting that a very high increase of sCT [100 ng/mL was observed in 5/9 patients with MTC and in 2/4 patients with HCC, therefore suggesting the absence of a relationship between the entity of response to pentagastrin test with a specific pathology (MTC vs. HCC). In six patients, the MTC was the nodule on which preoperative FNAC had been performed, while in other three patients preoperative FNAC had been performed on a different nodule from the MTC. Based on our experience, in case of the pentagastrin stimulation test with sCT \100 ng/L and a single nodule, the CT assay on FNAC may be useful, subsequently lobectomy with definitive histological diagnosis is recommended. In case of the Pg test with sCT \100 ng/L and bilateral goiter, total thyroidectomy with histological diagnosis is recommended. In this way, as for the surgical procedure, total thyroidectomy is recommended in cases of bilateral goiter, while lobectomy can be offered for cases with single nodes with serum dosage of bCT in the strict follow up. In case of the pentagastrin stimulating test with sCT \100 ng/L and bilateral goiter, total thyroidectomy with histological diagnosis is recommended.
British journal of cancer, 2003
Imaging-detected relapses are observed in a significant proportion of patients with medullary thyroid carcinoma (MTC) with normal postoperative imaging studies. The aim of this study was to search for prognostic factors of imaging-detected relapse. This retrospective study was performed in 63 consecutive MTC patients with normal postoperative medical imaging. After surgery, the basal calcitonin (CT) level was undetectable in 35 patients and elevated in 28. During follow-up, 18 patients developed a clinical or imaging-detected relapse (29%) in the neck and/or at distant sites: 15 had an elevated postoperative basal CT level and three had an undetectable postoperative basal CT level. At multivariate analysis, the significant parameters predictive of imaging-detected relapse were the postoperative plasma CT level and the tumour extension (pT). The 3- and 5-year relapse-free survival rates were 94 and 90% in patients with an undetectable postoperative basal CT level, and 78 and 61% in p...
European Journal of Surgical Oncology (EJSO), 2007
Aim: The analysis of a 37-year retrospective study on diagnosis, prognostic variables, treatment and outcome of a large group of medullary thyroid cancer (MTC) patients was conducted, in order to plan a possible evidence-based management process. Methods: Between Jan 1967 to Dec 2004, 157 consecutive MTC patients underwent surgery in our centre: 60 males and 97 females, mean age 47.3 years (range 6e79). Total thyroidectomy was performed in 143 patients (91.1%); central compartment (CC) node dissection (level VI) in 41 patients; central plus lateral compartment (LC) node dissection (levels II, III, and IV) in 82 patients. Subtotal thyroidectomy was initially performed in 14 cases: 10 of them were re-operated because of persistence of elevated serum calcitonin levels. Results: After a median post-surgical follow-up of 68 months (range 2e440 months), 42.9% of patients were living disease-free, 39.8% were living with disease, 3.1% were deceased due to causes different from MTC, and 3.2% were deceased due to MTC. The overall 10-year survival rate was 72%. At uni-variate statistical analysis (a) patient's age at initial treatment (>45 years; 45 years), (b) sporadic vs. hereditary MTC, (c) disease stage, and (d) the extent of surgical approach resulted as significant variables. Instead, at multivariate statistical analysis, only (a) patient's age at initial diagnosis, (b) disease stage, and (c) the extent of surgery resulted as significant and independent prognostic variables influencing survival. Conclusion: The presence of lymph node and distant metastases at first diagnosis significantly worsened prognosis and survival rate in our series. Early diagnosis of MTC is very important, allowing complete surgical cure in Stages I and II patients. Due to the relatively bad prognosis of MTC, especially for disease Stages III and IV, it appears reasonable to recommend radical surgery including total thyroidectomy plus CC lymphoadenectomy as the treatment of choice, plus LC lymphoadenectomy in patients with palpable and/or ultrasound enlarged neck lymph nodes.
Langenbeck's Archives of Surgery, 2001
Background: The purpose of this investigation was to analyze the individual diagnostic and operative strategy in the treatment of medullary thyroid carcinoma (MTC) in international specialized centers and to assess whether standard procedures are carried out in practice everywhere. Methods: A questionnaire concerning diagnosis and treatment of patients with primary, persistent, or recurrent sporadic or familial MTC was sent to 263 members of the International Association of Endocrine Surgeons. Results: Primary treatment of MTC does not show significant differences for patients with sporadic or familial disease (Chi-square, n.s.), and standard procedures are performed in only 25-40% of patients. Computed tomography scan is the most common localization procedure in persistent or recurrent disease (52-72%), followed by scintigraphy (43-71%), ul-trasonography (41-56%), and magnetic resonance imaging (31-49%). In case of negative localization studies, 68-86% of colleagues do not recommend reoperation. In symptomatic patients with stage-IV tumors, however, 84% of colleagues advocate reoperation to provide relief from the tumor burden. Conclusions: Even with experienced endocrine surgeons, a consensus to uni-and/or bilateral neck dissection in primary MTC is lacking. The majority of authors supports at least total thyroidectomy with central lymph-node dissection. In recurrent disease, there is a general tendency to reoperate in case of positive localization studies and in case of symptomatic disease.
Medullary Thyroid Carcinoma: A Rare Diagnosis
İstanbul Kanuni Sultan Süleyman Tıp Dergisi, 2021
Objective: The aim of this study was to analyze the clinical, imaging, pathological and postoperative surveillance data of patients with medullary thyroid carcinoma (MTC). Method: We included 11 patients who underwent surgery. The surgical type was documented. Clinical characteristics and imaging findings as well as preoperative and postoperative calcitonin and carcinoembryonic antigen (CEA) levels and preoperative thyroid stimulating hormone (TSH) levels were retrospectively reviewed. The demographic characteristics of the patients were also noted. Results: The mean age of the patients was 53.7 ± 12.8 years, and the mean nodule size was 27 ± 18 (range, 9-70) mm. The mean preoperative TSH level was 1.1 ± 1.0 mIU/L, and the median calcitonin and CEA levels were 898 (range, 10.9-1747) pg/mL and 98.1 (range, 44-196) ng/mL, respectively. Total thyroidectomy and central/lateral lymph node dissection were performed in all patients. According to preoperative fine-needle aspiration biopsy results, two (18.2%) patients were classified as Bethesda 2 and one (9.1%) as Bethesda 4 and eight (72.7%) patients had MTC. Of the patients, seven (63.6%) were in the early stage and four (36.4%) were in the localized advance stage. One patient had ret proto-oncogene-positive hereditary MTC and 10 had sporadic MTC. During the follow-up, one (9.1%) patient died because of lung metastasis. Conclusion: For the preoperative diagnosis of MTC, clinical characteristics, imaging findings, cytology test results, and genetic test results as well as serum calcitonin and CEA levels should be comprehensively evaluated. As a clinical approach, obtaining preoperative calcitonin and CEA levels for patients with thyroid nodules scheduled for thyroidectomy is advisable.
Lymph node involvement in macroscopic medullary thyroid carcinoma
British Journal of Surgery, 2005
Background Medullary thyroid carcinoma (MTC) is a rare disease, with variable tendency to lymphatic spread. The aim of this retrospective study was to identify distinctive features of large MTC with and without nodal metastases. Methods Between 1993 and 2003, 28 consecutive patients underwent total thyroidectomy and neck node dissection for sporadic MTC larger than 10 mm in diameter. Results All tumours were confirmed to be malignant with a locally invasive pattern of growth. Lymph node metastases were present in 16 patients (N1) and absent in 12 (N0). There were no statistically significant differences between patients with N0 and N1 tumours concerning age (mean 52·1 versus 53·4 years), male:female ratio (0·7 versus 1·0), basal preoperative calcitonin concentration (mean 3238 versus 3076 pg/ml) and tumour size (23·3 versus 23·9 mm). There were differences in the incidence of tumour invasion (P < 0·001), vascular embolism (P = 0·011) and peritumoral thyroiditis (P = 0·039). Measu...
Sporadic medullary thyroid carcinoma: clinical data from a university hospital
Clinics, 2009
INTRODUCTION: Medullary thyroid carcinoma may occur in a sporadic (s-medullary thyroid carcinoma, 75%) or in a multiple endocrine neoplasia type 2 form (MEN2, 25%). These clinical forms differ in many ways, as s-medullary thyroid carcinoma cases are RET-negative in the germline and are typically diagnosed later than medullary thyroid carcinoma in MEN2 patients. In this study, a set of cases with s-medullary thyroid carcinoma are documented and explored. PURPOSE: To document the phenotypes observed in s-medullary thyroid carcinoma cases from a university group and to attempt to improve earlier diagnosis of s-medullary thyroid carcinoma. Some procedures for diagnostics are also recommended. METHOD: Patients (n=26) with apparent s-medullary thyroid carcinoma were studied. Their clinical data were reviewed and peripheral blood was collected and screened for RET germline mutations. RESULTS: The average age at diagnosis was 43.9 years (± 10.82 SD) and did not differ between males and females. Calcitonin levels were increased in all cases. Three patients presented values that were 100-fold greater than the normal upper limit. Most (61.54%) had values that were 20-fold below this limit. Carcinoembryonic antigen levels were high in 70.6% of cases. There was no significant association between age at diagnosis, basal calcitonin levels or time of disease onset with thyroid tumor size (0.6-15 cm). Routine thyroid cytology yielded disappointing diagnostic accuracy (46.7%) in this set of cases. After total thyroidectomy associated with extensive cervical lymph node resection, calcitonin values remained lower than 5 pg/mL for at least 12 months in eight of the cases (30.8%). Immunocyto- and histochemistry for calcitonin were positive in all analyzed cases. None of the 26 cases presented germline mutations in the classical hotspots of the RET proto-oncogene. CONCLUSION: Our cases were identified late. The basal calcitonin measurements and immunostaining for calcitonin were highly useful for diagnosing s-medullary thyroid carcinoma. The rate of complete patient recovery was low, and none of the parameters analyzed were useful predictors of the thyroid tumor size. Our findings support previous recommendations for routine serum calcitonin evaluation and immunostaining analysis involving single thyroid nodules.