An audit of medullary thyroid carcinoma from a tertiary care hospital in northwest India
IntroductionMedullary thyroid carcinoma (MTC) is a rare thyroid malignancy originating from parafollicular C cells. It accounts for 5%–10% of all thyroid malignancies.MethodsAn ambispective analysis of pathologically proven MTC presented in a tertiary care hospital in northwest India was performed a...
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2024-01-01
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author | Ananda Mohan Chakraborty Ashutosh Rai Rimesh Pal Soham Mukherjee Divya Dahiya Rajinder Kumar Uma Nahar Saikia Naresh Kumar Panda Sanjay Kumar Bhadada Pinaki Dutta |
author_facet | Ananda Mohan Chakraborty Ashutosh Rai Rimesh Pal Soham Mukherjee Divya Dahiya Rajinder Kumar Uma Nahar Saikia Naresh Kumar Panda Sanjay Kumar Bhadada Pinaki Dutta |
author_sort | Ananda Mohan Chakraborty |
collection | DOAJ |
description | IntroductionMedullary thyroid carcinoma (MTC) is a rare thyroid malignancy originating from parafollicular C cells. It accounts for 5%–10% of all thyroid malignancies.MethodsAn ambispective analysis of pathologically proven MTC presented in a tertiary care hospital in northwest India was performed after considering demography, clinical manifestation, RET mutation status, management, and outcome as denominators.ResultsAmong 2,735 thyroid malignancy cases who presented to our institute in the last 10 years (2012–2022), 78 (3%) had MTC with a mean age of presentation of 43 ± 11 years; 60% of them were female. The median duration of symptoms was 23 months (IQR 12–36 months). The most common presenting complaint was goiter with lymphadenopathy (80.8%). Among the atypical presentations, one each had ectopic Cushing’s syndrome, hypertensive crisis in pregnancy due to pheochromocytoma, synchronous chondrosarcoma, and Von Hippel–Lindau disease spectrum. Median calcitonin and carcinoembryonic antigen (CEA) levels at presentation were 1,274 pg/mL (n = 64) and 149 ng/mL (n = 39), respectively. Twenty-two patients were germline RET mutation-positive, and they presented at a younger age. Majority of the patients presented with stage IV disease. Surgery was the primary modality of therapy. Twenty-nine patients received radiotherapy and 25 patients received tyrosine kinase inhibitors (TKIs). Nine patients received peptide receptor radiotherapy (PRRT) with Lu-177 with neoadjuvant capecitabine. Median progression-free survival (PFS) was 60 months. Patients without structurally and biochemically residual disease and stable disease after the first modality of therapy (Log-rank 11.4; p = 0.004) had a better PFS. Female patients (Log-rank: 9.5; p = 0.002) had a better PFS than male patients.ConclusionThis study showed that MTC comprises 3% of thyroid malignancies with a female preponderance. RET mutation-positive patients had a younger age at presentation. Surgery was the first-line therapy. Radiotherapy, TKI, and PRRT were given as a part of second-line or third-line therapy due to persistent disease and/or disease recurrence. The median PFS was better in female patients and in patients who had no residual lesions and stable disease after the primary modality of therapy. |
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spelling | doaj.art-26a972733c204a8588ba37c2b87fa0712024-01-08T06:16:42ZengFrontiers Media S.A.Frontiers in Endocrinology1664-23922024-01-011410.3389/fendo.2023.12263481226348An audit of medullary thyroid carcinoma from a tertiary care hospital in northwest IndiaAnanda Mohan Chakraborty0Ashutosh Rai1Rimesh Pal2Soham Mukherjee3Divya Dahiya4Rajinder Kumar5Uma Nahar Saikia6Naresh Kumar Panda7Sanjay Kumar Bhadada8Pinaki Dutta9Department of Endocrinology, Postgraduate Institute of Medical Education and Research, Chandigarh, IndiaCentre for Endocrinology, William Harvey Research Institute, Queen Marry University of London, London, United KingdomDepartment of Endocrinology, Postgraduate Institute of Medical Education and Research, Chandigarh, IndiaDepartment of Endocrinology, Postgraduate Institute of Medical Education and Research, Chandigarh, IndiaDepartment of General Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh, IndiaDepartment of Nuclear Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, IndiaDepartment of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, IndiaDepartment of Otorhinolaryngology, Postgraduate Institute of Medical Education and Research, Chandigarh, IndiaDepartment of Endocrinology, Postgraduate Institute of Medical Education and Research, Chandigarh, IndiaDepartment of Endocrinology, Postgraduate Institute of Medical Education and Research, Chandigarh, IndiaIntroductionMedullary thyroid carcinoma (MTC) is a rare thyroid malignancy originating from parafollicular C cells. It accounts for 5%–10% of all thyroid malignancies.MethodsAn ambispective analysis of pathologically proven MTC presented in a tertiary care hospital in northwest India was performed after considering demography, clinical manifestation, RET mutation status, management, and outcome as denominators.ResultsAmong 2,735 thyroid malignancy cases who presented to our institute in the last 10 years (2012–2022), 78 (3%) had MTC with a mean age of presentation of 43 ± 11 years; 60% of them were female. The median duration of symptoms was 23 months (IQR 12–36 months). The most common presenting complaint was goiter with lymphadenopathy (80.8%). Among the atypical presentations, one each had ectopic Cushing’s syndrome, hypertensive crisis in pregnancy due to pheochromocytoma, synchronous chondrosarcoma, and Von Hippel–Lindau disease spectrum. Median calcitonin and carcinoembryonic antigen (CEA) levels at presentation were 1,274 pg/mL (n = 64) and 149 ng/mL (n = 39), respectively. Twenty-two patients were germline RET mutation-positive, and they presented at a younger age. Majority of the patients presented with stage IV disease. Surgery was the primary modality of therapy. Twenty-nine patients received radiotherapy and 25 patients received tyrosine kinase inhibitors (TKIs). Nine patients received peptide receptor radiotherapy (PRRT) with Lu-177 with neoadjuvant capecitabine. Median progression-free survival (PFS) was 60 months. Patients without structurally and biochemically residual disease and stable disease after the first modality of therapy (Log-rank 11.4; p = 0.004) had a better PFS. Female patients (Log-rank: 9.5; p = 0.002) had a better PFS than male patients.ConclusionThis study showed that MTC comprises 3% of thyroid malignancies with a female preponderance. RET mutation-positive patients had a younger age at presentation. Surgery was the first-line therapy. Radiotherapy, TKI, and PRRT were given as a part of second-line or third-line therapy due to persistent disease and/or disease recurrence. The median PFS was better in female patients and in patients who had no residual lesions and stable disease after the primary modality of therapy.https://www.frontiersin.org/articles/10.3389/fendo.2023.1226348/fullmedullary thyroid carcinomaneuroendocrine tumortyrosine kinase inhibitorcalcitoninRET mutation |
spellingShingle | Ananda Mohan Chakraborty Ashutosh Rai Rimesh Pal Soham Mukherjee Divya Dahiya Rajinder Kumar Uma Nahar Saikia Naresh Kumar Panda Sanjay Kumar Bhadada Pinaki Dutta An audit of medullary thyroid carcinoma from a tertiary care hospital in northwest India Frontiers in Endocrinology medullary thyroid carcinoma neuroendocrine tumor tyrosine kinase inhibitor calcitonin RET mutation |
title | An audit of medullary thyroid carcinoma from a tertiary care hospital in northwest India |
title_full | An audit of medullary thyroid carcinoma from a tertiary care hospital in northwest India |
title_fullStr | An audit of medullary thyroid carcinoma from a tertiary care hospital in northwest India |
title_full_unstemmed | An audit of medullary thyroid carcinoma from a tertiary care hospital in northwest India |
title_short | An audit of medullary thyroid carcinoma from a tertiary care hospital in northwest India |
title_sort | audit of medullary thyroid carcinoma from a tertiary care hospital in northwest india |
topic | medullary thyroid carcinoma neuroendocrine tumor tyrosine kinase inhibitor calcitonin RET mutation |
url | https://www.frontiersin.org/articles/10.3389/fendo.2023.1226348/full |
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