scholarly journals Elevated basal serum levels of calcitonin and simultaneous surgery of MEN2A-specific tumors

2020 ◽  
Author(s):  
Hai-Xiao Tang ◽  
Hao Yang ◽  
Feng Li ◽  
Zhi-Lie Cao ◽  
Yun-Teng Huang ◽  
...  
Keyword(s):  
Lymph Node ◽  
Histopathological Examination ◽  
Serum Levels ◽  
Early Screening ◽  
Neck Lymph Node ◽  
Serum Calcitonin ◽  
Basal Serum ◽  
Simultaneous Surgery ◽  
Cutaneous Lichen Amyloidosis

Abstract Background Multiple endocrine neoplasia type 2A (MEN2A) is a rare syndrome caused almost by germline RET mutation, and characterized by medullary thyroid carcinoma (MTC), in combination or not with pheochromocytoma (PHEO), hyperparathyroidism (HPTH), cutaneous lichen amyloidosis (CLA) and Hirschsprung’s disease (HD). The basal serum calcitonin (Ctn)/carcinoembryonic antigen (CEA) levels are significantly correlated with MTC stage. Metachronous surgery of MEN2A-specific tumors is a routine procedure. We aimed to explore the clinical significance of pro-gastrin-releasing peptide (proGRP) in MTC with elevated Ctn, and simultaneous surgery of MEN2A-specific tumors. Methods We retrospectively investigated 8 RET mutation carriers of 2 Chinese pedigrees with MEN2A. Clinical profiles, imaging examinations, preoperative and postoperative biochemical data, surgical procedures and follow-up records were evaluated. Results Three patients showed the levels of elevated Ctn, but normal proGRP. Among them, one patient (FAIII-6) in Family A (one for RET C634R mutation), diagnosed with bilateral MTC, left PHEO, bilateral HPTH and CLA, classified as MEN2A-related CLA subtype, underwent successfully simultaneous adrenal-sparing surgery (ASS), total thyroidectomy (TT) and parathyroidectomy, while TT of the other two patients (FBII-3 and FBIII-7) diagnosed with bilateral MTC in Family B (all for RET C618R mutation) were performed. Unexpectedly, absence of neck lymph node MTC metastasis was indicated by histopathological examination. Postoperatively, all had consistently “undetectable” or normal levels of Ctn/CEA during follow-up. Conclusions Patients with normal proGRP, despite high levels of Ctn, might have noregional lymph node MTC metastasis, and neck dissection should be avoided. Moreover, simultaneous surgery for coexistent PHEO, and either MTC or HPTH is an approach of choice to use as an alternative treatment pattern. Recognition of MEN2A-related CLA and subsequently early screening of RET mutation may be favorable for timely management of MEN2A-specific tumors.

Molecular diagnosis and treatment of multiple endocrine neoplasia type 2B in Ethnic Han Chinese

2020 ◽  
Vol 20 ◽  
Author(s):  
Zhe-Wei Zhang ◽  
Xiao Guo ◽  
Xiao-Ping Qi
Keyword(s):  
Multiple Endocrine Neoplasia ◽  
Multiple Endocrine Neoplasia Type ◽  
Histopathological Examination ◽  
Diagnosis And Treatment ◽  
De Novo Mutation ◽  
Neck Lymph Node ◽  
Serum Calcitonin ◽  
Endocrine Neoplasia ◽  
Endocrine Neoplasia Type ◽  
Men 2B

Background: Multiple endocrine neoplasia type 2B (MEN 2B) is mainly caused by M918T RET germline mutation, and characterized by medullary thyroid carcinoma (MTC), pheochromocytoma (PHEO) and non-endocrine features. However, the diagnosis and treatment are usually delayed. Materials & Methods: This study reports 5 Chinese pedigrees with 5 individuals harboring germline RET-M918T, and systematically reviewed previous Chinese literatures. Results: All 5 patients initially presented MTC, but none had biochemically cured postoperatively. 2 also presented bilateral PHEO after adrenal-sparing surgery, 1 needed steroid replacement. Further, totally 32 MEN 2B patients from literatures were clustered with 28 available for analysis. 26 (92.8%) were diagnosed by endocrine-related symptoms; the remaining 2 (7.2%) due to RET testing and oral symptoms respectively. 25 patients underwent thyroidectomy with/without neck lymph node dissection at mean age of (23.3 ± 10.4) years. Histopathological examination revealed MTC (100%). Of them, 17 had definite TNM stage, with 1 in stage Ⅲ and others in Ⅳ. Other information of MEN 2B-related symptoms included penetrance of PHEO (60.7%), constipation (32.1%), Hirschsprung disease (25%), alacrima (17.8%), mucosal ganglioneuroma (96.4%) and marfanoid habitus (71.4%). 19 patients were verified harboring RET-M918T (c.2753T>C), of whom 15 (78.9%) were de novo mutation. The other 9 were clinically diagnosed as MEN 2B. Discussion & Conclusion : The initial diagnosis of MEN 2B is relatively later, and diagnosed by non-endocrine components is extremely lower. Recognition of MEN 2B and its non-endocrine-related components is still the utmost requirement for a Chinese physician. Combined RET screening and serum calcitonin detection can facilitate early diagnosis.

Gastro-intestinal polypeptides in patients treated for medullary carcinoma of the thyroid

1984 ◽  
Vol 106 (1) ◽  
pp. 112-115 ◽  
Author(s):  
Bente Rasmusson
Keyword(s):  
Serum Gastrin ◽  
Clinical Course ◽  
Elevated Serum ◽  
Medullary Carcinoma ◽  
Serum Levels ◽  
High Serum ◽  
Serum Concentrations ◽  
Serum Calcitonin ◽  
Basal Serum ◽  
Previously Treated

Abstract. In 12 patients treated 2 to 58 months previously for medullary carcinoma of the thyroid, basal serum concentrations of calcitonin, gastrin, vasoactive intestinal polypeptide, glucagon, insulin, and pancreatic polypeptide were measured in search of any correlation between these and the clinical course of the disease. All patients had elevated serum calcitonin levels indicating present disease. One patient had increased serum concentrations of several hormones. Another had achlorhydria and high serum gastrin levels. No relationship between calcitonin and gastro-intestinal polypeptides was found in 11 patients. No correlations were found between serum levels of polypeptides and the occurrence of diarrhoea in 5 patients. It is concluded that gastro-intestinal polypeptides, which are produced by other apudomas, are not secreted in more than normal concentrations under basal conditions, by the majority of patients previously treated for medullary carcinoma of the thyroid.

scholarly journals Elevated basal serum levels of calcitonin and simultaneous surgery of MEN2A-specific tumors

Neoplasma ◽  
2021 ◽  
Author(s):  
Hai-Xiao Tang ◽  
Hao Yang ◽  
Feng Li ◽  
Zhi-Lie Cao ◽  
Yun-Teng Huang ◽  
...  
Keyword(s):  
Serum Levels ◽  
Basal Serum ◽  
Simultaneous Surgery

scholarly journals Neck Lymph Node Metastasis Detection in Patients with Differentiated Thyroid Carcinoma (DTC) in Long-Term Follow-up: a ¹³¹I-SPECT/CT Study

2020 ◽  
Author(s):  
Angela Spanu ◽  
Susanna Nuvoli ◽  
Andrea Marongiu ◽  
Ilaria Gelo ◽  
Luciana Mele ◽  
...  
Keyword(s):  
Lymph Node ◽  
Thyroid Carcinoma ◽  
Differentiated Thyroid Carcinoma ◽  
Whole Body ◽  
Whole Body Scan ◽  
Body Scan ◽  
Neck Lymph Node ◽  
Long Term Follow Up

Abstract Background: The identification of neck lymph node (LN) metastases represents a very important issue in the management of patients with differentiated thyroid carcinoma (DTC). To this purpose, in the present study, we used 131I-SPECT/CT as a diagnostic imaging procedure. Methods: A consecutive series of 224 DTC patients with ascertained neck radioiodine-avid foci at ¹³¹I-SPECT/CT during long-term follow-up was evaluated. All patients had already undergone total thyroidectomy and radioiodine therapy and had been classified as follows: 62 at high risk (H), 64 at low risk (L) and 98 at very low risk (VL). ¹³¹I-Whole body scan (WBS) followed by SPECT/CT was performed in all cases. Results: In the 224 patients, 449 neck iodine avid foci were ascertained at SPECT/CT, while 322 were evidenced at WBS in 165/224 patients. WBS classified as residues 263/322 foci and as unclear 59/322 foci; among the former foci SPECT/CT correctly characterized 8 LN metastases and 3 physiologic uptakes and among the latter, it pinpointed 26 LN metastases, 18 residues, and 15 physiologic uptakes. SPECT/CT also classified 127 foci occult at WBS as 59 LN metastases and 68 residues. Globally, SPECT/CT identified 93 LN metastases in 59 patients (26 H, 20 L, 13 VL), while WBS evidenced 34 in 25 cases. All 13 VL patients, T1aN0M0, 5 of whom with LN near sub-mandibular glands, had thyroglobulin undetectable or <2.5 ng/ml. Globally, SPECT/CT obtained an incremental value than WBS in 45.5 % of patients, a more correct patient classification changing therapeutic approach in 30.3 % of cases and identified WBS false-positive findings in 8% of cases. Conclusions: ¹³¹I-SPECT/CT proved to correctly detect and characterize neck LN metastases in DTC patients in long-term follow-up, improving the performance of planar WBS. SPECT/CT routine use is thus suggested; its role is particularly relevant in patients with WBS inconclusive, VL, T1aN0M0 and with undetectable or very low thyroglobulin levels. Keywords: Differentiated thyroid carcinoma (DTC), Neck lymph node metastases, Long-term follow-up, 131I-Whole body scan (WBS), 131I-SPECT/CT

scholarly journals Neck Lymph Node Metastasis Detection in Patients with Differentiated Thyroid Carcinoma (DTC) in Long-Term Follow-up: a ¹³¹I-SPECT/CT Study

2019 ◽  
Author(s):  
Angela Spanu ◽  
Susanna Nuvoli ◽  
Andrea Marongiu ◽  
Ilaria Gelo ◽  
Luciana Mele ◽  
...  
Keyword(s):  
Lymph Node ◽  
Thyroid Carcinoma ◽  
Differentiated Thyroid Carcinoma ◽  
Low Risk ◽  
Whole Body ◽  
Neck Lymph Node ◽  
Incremental Value ◽  
Long Term Follow Up

Abstract Background: The prognostic importance of neck lymph node (LN) metastases in differentiated thyroid carcinoma (DTC) is controversial even if their presence at surgery often correlates with tumor recurrences that it is mandatory to identify. To this purpose, in the present study, we used 131 I-SPECT/CT as diagnostic imaging procedure. Methods: We retrospectively evaluated 224 consecutive DTC patients already submitted to total thyroidectomy and radioiodine therapy, all of them with ascertained neck radioiodine-avid foci at ¹³¹I-SPECT/CT during long-term follow-up; at surgery, 62 patients were classified at high risk (H), 64 at low risk (L), 98 at very low risk. All patients underwent ¹³¹I-Whole body scan (WBS) followed by SPECT/CT. Result: In the 224 patients, 449 neck iodine avid foci were ascertained at SPECT/CT, while 322 were evidenced at WBS in 165/224 patients. WBS classified as residues 263/322 foci and as unclear 59/322 foci; among the former foci SPECT/CT correctly characterized 8 LN metastases and 3 physiologic uptakes and among the latter it pinpointed 26 LN metastases, 18 residues and 15 physiologic uptakes. SPECT/CT also classified 127 foci occult at WBS as 59 LN metastases and 68 residues. Globally, SPECT/CT identified 93 LN metastases in 59 patients (26 H, 20 L, 13 VL), while WBS evidenced 34 in 25 cases. All 13 VL patients,T1aN0M0, 5 of whom with LN near sub-mandibular glands, had thyroglobulin undetectable or <2.5 ng/ml. Globally, SPECT/CT obtained an incremental value than WBS in 45.5 % of patients, a more correct patient classification changing therapeutic approach in 30.3 % of cases and identified WBS false positive findings in 8% of cases. Conclusion: ¹³¹I-SPECT/CT proved to correctly detect and characterize neck LN metastases in DTC patients in long-term follow-up, improving WBS performance. SPECT/CT routine use is suggested, its role being particularly significant in patients with WBS inconclusive, VL, T1aN0M0 and with undetectable or very low thyroglobulin levels.

scholarly journals Metastatic Lymph Node Ratio for Predicting Recurrence in Medullary Thyroid Cancer

Cancers ◽  
2021 ◽  
Vol 13 (22) ◽  
pp. 5842
Author(s):  
Jinyoung Kim ◽  
Jun Park ◽  
Hyunju Park ◽  
Min Sun Choi ◽  
Hye Won Jang ◽  
...  
Keyword(s):  
Thyroid Cancer ◽  
Lymph Node ◽  
Medullary Thyroid Cancer ◽  
Lymph Node Ratio ◽  
Serum Calcitonin ◽  
Free Survival ◽  
Medullary Thyroid ◽  
Node Ratio ◽  
Postoperative Serum

The lymph node ratio (LNR) has been investigated as a prognostic factor in many different types of cancers, including differentiated thyroid cancer; however, reports regarding medullary thyroid cancer (MTC) are limited. Therefore, this study aims to evaluate LNR as a risk factor for structural recurrence in patients with MTC. Medical records of patients treated for MTC in a single tertiary center between 1995 and 2017 were retrospectively reviewed. LNR is defined as the number of metastatic lymph nodes or lymph node metastases (LNM) divided by the number of retrieved lymph nodes or lymph node yield (LNY). In the survival analysis, recurrence-free survival was defined as the time from the date of total thyroidectomy to recurrence or last follow-up. To identify risk factors influencing structural recurrence, univariable and multivariable Cox proportional hazard models were used. A total of 132 patients were enrolled. The mean age of study participants was 49.7 years, and 86 patients (65%) were women. Structural recurrence was identified in 39 patients at the end of the study period, and the median follow-up period was 8.7 years. In univariable analyses, gross extra thyroidal extension, N stage, postoperative serum calcitonin and carcinoembryonic antigen (CEA) levels, and LNR were significant (p < 0.05) predictors of structural recurrence. In multivariable analysis, postoperative serum calcitonin, postoperative serum CEA, and LNR were identified as a predictor of disease-free survival (p < 0.05). LNR can potentially predict structural recurrence as a quantitative evaluation tool for lymph node metastasis in patients with MTC.

scholarly journals Radiotherapy for localized sebaceous carcinoma of the eyelid: a retrospective analysis of 83 patients

2019 ◽  
Vol 60 (5) ◽  
pp. 622-629 ◽  
Author(s):  
Yoshiaki Takagawa ◽  
Wakana Tamaki ◽  
Shigenobu Suzuki ◽  
Koji Inaba ◽  
Naoya Murakami ◽  
...  
Keyword(s):  
Lymph Node ◽  
Tumor Size ◽  
Postoperative Radiotherapy ◽  
Late Toxicity ◽  
Sebaceous Carcinoma ◽  
Curative Intent ◽  
Neck Lymph Node ◽  
Lymph Node Recurrence ◽  
Grade 3

Abstract The current study retrospectively analyzed the results of radiotherapy for clinically localized sebaceous carcinoma of the eyelid.We reviewed records of 83 patients with histologically confirmed sebaceous carcinoma who were treated radiotherapeutically between 1983 and 2015. Sixty-five patients (78%) were initially treated with radiotherapy of curative intent, while the remaining 18 patients underwent postoperative radiotherapy due to tumor recurrence or positive surgical margins. Thirty-seven patients belonged to T1–2, while 46 belonged to T3–4. All 83 patients were treated with radiotherapy with a median radiation dose of 60 Gy. The median follow-up period was 92.1 months (range, 2.8–310.3 months). At the time of analysis, 13 patients (15.1%) died, and 36 patients (43.3%) had local recurrence. The 7-year overall survival, freedom from neck lymph node recurrence, and local control (LC) rates for all patients were 83.5%, 75.5%, and 52.3%, respectively. Patients with a tumor size ≤10 mm had a higher 7-year LC rate than those with a tumor size >10 mm (58.8% vs 46.6%, P = 0.054). Neck lymph node recurrence was observed in 17 patients (20%) and significantly related to the tumor size. Late toxicity of an eyelid dysfunction of grade 3 was observed in 1 patient with T3 tumor. Radiotherapy for sebaceous carcinoma of the eyelid is a reasonable alternative to surgical resection for tumors <10 mm in size with few severe complications, while larger tumors should be treated with surgery if feasible.

Prognostic variables of papillary and follicular thyroid carcinoma patients with lymph node metastases and without distant metastases.

1999 ◽  
pp. 109-115 ◽  
Author(s):  
J D Lin ◽  
M J Liou ◽  
T C Chao ◽  
H F Weng ◽  
Y S Ho
Keyword(s):  
Lymph Node ◽  
Thyroid Carcinoma ◽  
Lymph Node Metastases ◽  
Follicular Thyroid Carcinoma ◽  
Distant Metastases ◽  
Prognostic Variables ◽  
Neck Lymph Node ◽  
Clinical Variables ◽  
Node Metastases

From 1977 through 1995, 1,013 thyroid carcinoma patients received treatment and were followed up at Chang Gung Medical Center in Taiwan. To evaluate the prognostic variables of papillary and follicular thyroid carcinomas with limited lymph node metastases, a retrospective review of these patients was performed. Of these patients, 910 had papillary or follicular thyroid carcinoma, and 119 patients were categorized as clinical stage 2 with limited neck lymph node metastases only at the time of diagnosis. The patients were categorized into two groups as no recurrence and local recurrence or distant metastasis at the end of 1997. After the operations, radioactive iodide (131I) treatments were performed in 114 patients and external radiotherapy for neck region or distant metastases in 18 patients. The median follow-up period of these patients was 5.4 years. Clinical variables were coded in our computer for statistical analysis. After the treatments, 93 patients remained disease-free; 10 were in stage 2; 5 in stage 3; and 11 aggravated to stage 4. Of the clinical variables, age, post-operative first 1311 uptake scans, and 1-month post-operative thyroglobulin levels revealed statistically significant differences between the group which improved and the group which did not. During the follow-up period, five patients died; three patients died of thyroid cancer and two died of intercurrent diseases. Patients with papillary thyroid carcinoma revealed a higher percentage of lymph node metastases. Although limited lymph node metastases did not influence survival rate, patients with poor prognostic factors need more aggressive treatment to avoid progression of the cancer.

Sign in / Sign up

Export Citation Format

Share Document