Secondary Hyperthyroidism due to an Ectopic Thyrotropin-Secreting Neuroendocrine Pituitary Tumor: A Case Report

Trummer, Christian; Reiher, Hannes; Theiler‐Schwetz, Verena; Pandis, Marlene; Gstettner, Christian; Potzinger, Peter; Keck, Tilman; Pieber, Thomas R.; Lax, Sigurd; Haybaeck, Johannes; Stepan, V. T.; Pilz, Stefan

doi:10.1159/000505020

Cited by 9 publications

(7 citation statements)

References 23 publications

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“…The primary differential diagnosis in this case is resistance to thyroid hormone (RTH), specifically peripheral resistance to thyroid hormone (PRTH), which has been previously described in case reports ( 7 , 13 , 14 ). RTH is characterized by reduced sensitivity to thyroid hormone and is caused by mutations in the thyroid hormone receptor β gene, resulting in intranuclear T3 receptor dysfunction.…”

Section: Discussionmentioning

confidence: 78%

“…Additionally, in hyperthyroidism caused by TSH-secreting tumors, peripheral tissues exhibit sensitivity to thyroid hormone, and certain serum markers can be helpful in diagnosis. Increased serum concentrations of sex hormone-binding globulin (SHBG) and bone turnover markers, and elevated α-GSU/TSH molar ratio, could indicate the presence of TSH-secreting tumors ( 13 , 23 ). In addition, some tumors are plurihormonal, expressing GH and/or PRL along with TSH; hence, the measurement of GH and PRL can be helpful in diagnosing TSH-secreting tumor.…”

Section: Discussionmentioning

confidence: 99%

“…In the case of TSH-secreting tumors, tumor cells typically express abundant somatostatin receptor subtype (SSTR) 2 and 5 on their surface. Therefore, 68 Ga-DOTATATE or 68 Ga-DOTANOC PET/CT has been demonstrated to be effective in the diagnosing ectopic TSH-secreting tumor, especially when distinguishing them from intrasellar adenoma ( 12 , 13 ). Case 10 was the first to report the use of 68 Ga-DOTATATE PET/CT for localizing the ectopic TSH-secreting tumor ( 12 ).…”

Section: Discussionmentioning

confidence: 99%

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Central hyperthyroidism due to an ectopic TSH-secreting pituitary tumor: a case report and literature review

Zhu,

Liu,

et al. 2024

Front. Endocrinol.

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Ectopic thyroid-stimulating hormone (TSH)-secreting tumors are extremely rare, with only 15 reported cases in the literature. Herein, we described a 60-year-old female patient with thyrotoxicosis and elevated or unsuppressed levels of TSH. Family history and laboratory and genetic tests did not support a diagnosis of resistance to thyroid hormone (RTH). Given the unsuppressed TSH, TSH-secreting tumor was suspected, and magnetic resonance imaging (MRI) of the pituitary gland was performed. Surprisingly, the MRI scans revealed a nodule in the nasopharynx rather than a pituitary tumor in the sella region. Further evaluation using Gallium-68 DOTATATE positron emission tomography/computed tomography (68Ga-DOTATATE PET/CT) demonstrated increased DOTATATE uptake in the nasopharyngeal nodule. Additionally, an octreotide suppression test (OST) revealed an obvious reduction in TSH levels, further supporting the suspicion of the nasopharyngeal mass as the cause of inappropriate TSH secretion. To prepare for surgery, the patient received preoperative administration of octreotide, resulting in the normalization of TSH and thyroid hormone levels. The patient subsequently underwent successful surgical removal of the nasopharyngeal mass. Following the procedure, the patient experienced complete resolution of hyperthyroidism symptoms, with TSH declined and thyroid hormone levels returned to normal. Histochemistry analysis of the tumor revealed positive staining for TSH, growth hormone (GH), prolactin (PRL), luteinizing hormone (LH), and somatostatin receptor 2 (SSTR2). We discussed differential diagnosis of hyperthyroidism due to inappropriate TSH secretion, with a particular emphasis on the importance of 68Ga-DOTATATE PET/CT in combination with OST for identifying ectopic pituitary tumors.

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Section: Discussionmentioning

confidence: 78%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Central hyperthyroidism due to an ectopic TSH-secreting pituitary tumor: a case report and literature review

Zhu,

Liu,

et al. 2024

Front. Endocrinol.

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“…Furthermore, 18F-FDG PET/CT could not detect functional TSH-secreting tissue in our case, but 68Ga-DOTA-TATE PET/ CT did. Additionally, ectopic TSH-secreting tissue has been uncovered in several cases including a 10-year-old girl by using 68Ga-DOTA-TATE PET/CT [38][39][40]. However, it can be conceivable that 68Ga-DOTA-TATE PET/ CT imaging is helpful to detect ectopic and/or post-surgical recurrent/residual pituitary adenomas, since the pituitary gland also shows 68Ga-DOTA-TATE uptake.…”

Section: Discussionmentioning

confidence: 99%

Challenges in the Management of a 7-Year-Old Child with Thyrotropin-Secreting Pituitary Adenoma and the Review of the Literature

et al. 2023

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Introduction: Thyrotropin-producing pituitary adenoma (TSHoma) is a very rare disease, representing less than 1% of the pituitary tumours, present with elevated thyroid hormones and normal/high TSH concentrations. Case Presentation: A 7-year-old boy with nervousness was referred by his psychiatrist for elevated free T4, T3 and TSH levels. Initial evaluation revealed an elevated -subunit.Pituitary MRI demonstrated a macroadenoma. The patient underwent a trans-sphenoidal tumour resection (TSS) which showed positive immunohistochemical staining for TSH, growth hormone, and prolactin in tumoral tissue. Euthyroidism was achieved for one year after TSS, then, recurrence of tumour with elevated TSH and thyroid hormone levels necessitated a re-operation with TSS followed by gamma-knife radiosurgery. The euthyroid state was achieved and lasted for 2.5 years this time, but, due to the recurrence, medical treatment had been commenced with cabergoline and octreotide. Euthyroidism was maintained for the last 4 years on monthly octreotide treatment. A repeat MRI demonstrated no pituitary mass but a mass in the sphenoidal sinus had been detected. Removal of this mass by surgery did not achieve euthyroidism. 68Ga-DOTA-TATE PET/CT showed residual tissue extending from the pituitary region to the sphenoid sinus. The patient’s bone age was advanced 2 years at diagnosis which became 4 years in one year after the diagnosis and remained so throughout follow-up, leading to a final height of -3.3 SDS below his target height at the age of 16 years. Conclusion: The diagnosis, treatment, and follow-up of TSHomas are challenging and short stature due to accelerated bone maturation is a complication of paediatric TSHomas.

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“…First, the initial case of IST caused by ectopic TSHoma was reported in 1996 [ 16 ]. To date, more than ten similar cases have been documented [ 17 ].…”

Section: Pathophysiology Of Istmentioning

confidence: 99%

An Update on the Pathophysiology and Diagnosis of Inappropriate Secretion of Thyroid-Stimulating Hormone

Ohba

2021

IJMS

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Inappropriate secretion of thyroid-stimulating hormone (IST), also known as central hyperthyroidism, is a clinical condition characterized by elevated free thyroxine and triiodothyronine concentrations concurrent with detectable thyroid-stimulating hormone (TSH) concentrations. Similarly, the term syndrome of IST (SITSH) is widely used in Japan to refer to a closely related condition; however, unlike that for IST, an elevated serum free triiodothyronine concentration is not a requisite criterion for SITSH diagnosis. IST or SITSH is an important indicator of resistance to thyroid hormone β (RTHβ) caused by germline mutations in genes encoding thyroid hormone receptor β (TRβ) and TSH-secreting pituitary adenoma. Recent evidence has accumulated for several conditions associated with IST, including RTH without mutations in the TRβ gene (non-TR-RTH), the phenomenon of hysteresis involving the hypothalamus-pituitary-thyroid axis (HPT-axis), methodological interference, and Cushing’s syndrome after surgical resection. However, little information is available on the systematic pathophysiological aspects of IST in previous review articles. This report presents an overview of the recent advances in our understanding of the etiological aspects of IST that are relevant for diagnosis and treatment. Moreover, the report focuses on the potential mechanism of IST caused by hysteresis in the HPT-axis (lagging TSH recovery) in terms of epigenetic regulation.

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Secondary Hyperthyroidism due to an Ectopic Thyrotropin-Secreting Neuroendocrine Pituitary Tumor: A Case Report

Cited by 9 publications

References 23 publications

Central hyperthyroidism due to an ectopic TSH-secreting pituitary tumor: a case report and literature review

Central hyperthyroidism due to an ectopic TSH-secreting pituitary tumor: a case report and literature review

Challenges in the Management of a 7-Year-Old Child with Thyrotropin-Secreting Pituitary Adenoma and the Review of the Literature

An Update on the Pathophysiology and Diagnosis of Inappropriate Secretion of Thyroid-Stimulating Hormone

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