Case report: Surgical treatment of McCune-Albright syndrome with hyperthyroidism and retrosternal goiter: A case report and literature review

IntroductionMcCune-Albright syndrome (MAS) is a low-incidence syndrome consisting of the clinical triad of fibrous structural dysplasia of bone, endocrine disease, and skin pigmentation. Thyroid dysfunction is the second most common endocrine dysregulation in MAS. However, there are no treatment gui...

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Main Authors: Zhiwei Xing, Gongshuai Tao, Wanwan Pan, Delin Wu, Tingting Pan, Lingfeng Wan, Xiaopeng Ma, Yangyi Wang
Format: Article
Language:English
Published: Frontiers Media S.A. 2023-01-01
Series:Frontiers in Surgery
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fsurg.2022.921427/full
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author Zhiwei Xing
Zhiwei Xing
Gongshuai Tao
Gongshuai Tao
Wanwan Pan
Delin Wu
Tingting Pan
Lingfeng Wan
Xiaopeng Ma
Yangyi Wang
author_facet Zhiwei Xing
Zhiwei Xing
Gongshuai Tao
Gongshuai Tao
Wanwan Pan
Delin Wu
Tingting Pan
Lingfeng Wan
Xiaopeng Ma
Yangyi Wang
author_sort Zhiwei Xing
collection DOAJ
description IntroductionMcCune-Albright syndrome (MAS) is a low-incidence syndrome consisting of the clinical triad of fibrous structural dysplasia of bone, endocrine disease, and skin pigmentation. Thyroid dysfunction is the second most common endocrine dysregulation in MAS. However, there are no treatment guidelines for MAS complicated with hyperthyroidism. Notably, no case of MAS complicated with retrosternal goiter and hyperthyroidism has been reported to our knowledge.Case presentationWe report a 27-year-old man with MAS who developed the typical triad of bone fibrous dysplasia, skin pigmentation and hyperthyroidism, complaining of recent fast-growing neck mass and difficulty in breathing. Hyperthyrodism was under control by Thiamazole, and computed tomography showed an enlarged thyroid extending retrosternally. We performed a total thyroidectomy on the patient. At the 1-year follow-up, the patient's dyspnea, hyperthyroidism, and bone pain were all significantly alleviated.ReviewWe searched the literature for previous case reports concerning MAS patients complicated with thyroid dysregulation. A total of 17 articles and 22 patients were identified to form our database. Among them, 9 studies clearly mentioned surgical intervention in 11 patients, and prognoses were also reported. Surgery was the most common intervention chosen and indicated a satisfactory prognosis.ConclusionWe report a rare case of MAS patient complicated with retrosternal goiter and hyperthyroidism. Our review provides an overview of MAS cases requiring interventions on thyroid function, and total thyroidectomy should be a proper treatment for these patients.
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spelling doaj.art-9fd88329f98145c2885594f922a7e43f2023-01-06T11:13:25ZengFrontiers Media S.A.Frontiers in Surgery2296-875X2023-01-01910.3389/fsurg.2022.921427921427Case report: Surgical treatment of McCune-Albright syndrome with hyperthyroidism and retrosternal goiter: A case report and literature reviewZhiwei Xing0Zhiwei Xing1Gongshuai Tao2Gongshuai Tao3Wanwan Pan4Delin Wu5Tingting Pan6Lingfeng Wan7Xiaopeng Ma8Yangyi Wang9Graduate School, Wannan Medical College, Wuhu, ChinaDepartment of Thyroid and Breast Surgery, The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei, ChinaGraduate School, Wannan Medical College, Wuhu, ChinaDepartment of Thyroid and Breast Surgery, The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei, ChinaDepartment of Thyroid and Breast Surgery, The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei, ChinaDepartment of Thyroid and Breast Surgery, The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei, ChinaDepartment of Thyroid and Breast Surgery, The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei, ChinaDepartment of Thyroid and Breast Surgery, The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei, ChinaDepartment of Thyroid and Breast Surgery, The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei, ChinaDepartment of Thyroid and Breast Surgery, The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei, ChinaIntroductionMcCune-Albright syndrome (MAS) is a low-incidence syndrome consisting of the clinical triad of fibrous structural dysplasia of bone, endocrine disease, and skin pigmentation. Thyroid dysfunction is the second most common endocrine dysregulation in MAS. However, there are no treatment guidelines for MAS complicated with hyperthyroidism. Notably, no case of MAS complicated with retrosternal goiter and hyperthyroidism has been reported to our knowledge.Case presentationWe report a 27-year-old man with MAS who developed the typical triad of bone fibrous dysplasia, skin pigmentation and hyperthyroidism, complaining of recent fast-growing neck mass and difficulty in breathing. Hyperthyrodism was under control by Thiamazole, and computed tomography showed an enlarged thyroid extending retrosternally. We performed a total thyroidectomy on the patient. At the 1-year follow-up, the patient's dyspnea, hyperthyroidism, and bone pain were all significantly alleviated.ReviewWe searched the literature for previous case reports concerning MAS patients complicated with thyroid dysregulation. A total of 17 articles and 22 patients were identified to form our database. Among them, 9 studies clearly mentioned surgical intervention in 11 patients, and prognoses were also reported. Surgery was the most common intervention chosen and indicated a satisfactory prognosis.ConclusionWe report a rare case of MAS patient complicated with retrosternal goiter and hyperthyroidism. Our review provides an overview of MAS cases requiring interventions on thyroid function, and total thyroidectomy should be a proper treatment for these patients.https://www.frontiersin.org/articles/10.3389/fsurg.2022.921427/fullretrosternal goiterMcCune Albright syndromehyperthyroidismthyroidectomy131I treatmentcase report
spellingShingle Zhiwei Xing
Zhiwei Xing
Gongshuai Tao
Gongshuai Tao
Wanwan Pan
Delin Wu
Tingting Pan
Lingfeng Wan
Xiaopeng Ma
Yangyi Wang
Case report: Surgical treatment of McCune-Albright syndrome with hyperthyroidism and retrosternal goiter: A case report and literature review
Frontiers in Surgery
retrosternal goiter
McCune Albright syndrome
hyperthyroidism
thyroidectomy
131I treatment
case report
title Case report: Surgical treatment of McCune-Albright syndrome with hyperthyroidism and retrosternal goiter: A case report and literature review
title_full Case report: Surgical treatment of McCune-Albright syndrome with hyperthyroidism and retrosternal goiter: A case report and literature review
title_fullStr Case report: Surgical treatment of McCune-Albright syndrome with hyperthyroidism and retrosternal goiter: A case report and literature review
title_full_unstemmed Case report: Surgical treatment of McCune-Albright syndrome with hyperthyroidism and retrosternal goiter: A case report and literature review
title_short Case report: Surgical treatment of McCune-Albright syndrome with hyperthyroidism and retrosternal goiter: A case report and literature review
title_sort case report surgical treatment of mccune albright syndrome with hyperthyroidism and retrosternal goiter a case report and literature review
topic retrosternal goiter
McCune Albright syndrome
hyperthyroidism
thyroidectomy
131I treatment
case report
url https://www.frontiersin.org/articles/10.3389/fsurg.2022.921427/full
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