Endocrinol Metab.  2024 Apr;39(2):387-396. 10.3803/EnM.2023.1877.

Clinical Characteristics, Diagnosis, and Treatment of Thyroid Stimulating Hormone-Secreting Pituitary Neuroendocrine Tumor (TSH PitNET): A Single-Center Experience

• Heo J
• Suh YL
• Kim SH
• Kong DS
• Nam DH
• Lee WJ
• Kim ST
• Hong SD
• Ryu S
• Lee YB
• Kim G
• Jin SM
• Kim JH
• Hur KY

• KMID: 2554643
• DOI: http://doi.org/10.3803/EnM.2023.1877

Abstract

Background
Thyroid-stimulating hormone (TSH)-secreting pituitary neuroendocrine tumor (TSH PitNET) is a rare subtype of PitNET. We investigated the comprehensive characteristics and outcomes of TSH PitNET cases from a single medical center. Also, we compared diagnostic methods to determine which showed superior sensitivity.
Methods
A total of 17 patients diagnosed with TSH PitNET after surgery between 2002 and 2022 in Samsung Medical Center was retrospectively reviewed. Data on comprehensive characteristics and treatment outcomes were collected. The sensitivities of diagnostic methods were compared.
Results
Seven were male (41%), and the median age at diagnosis was 42 years (range, 21 to 65); the median follow-up duration was 37.4 months. The most common (59%) initial presentation was hyperthyroidism-related symptoms. Hormonal co-secretion was present in four (23%) patients. Elevated serum alpha-subunit (α-SU) showed the greatest diagnostic sensitivity (91%), followed by blunted response at thyrotropin-releasing hormone (TRH) stimulation (80%) and elevated sex hormone binding globulin (63%). Fourteen (82%) patients had macroadenoma, and a specimen of one patient with heavy calcification was negative for TSH. Among 15 patients who were followed up for more than 6 months, 10 (67%) achieved hormonal and structural remission within 6 months postoperatively. A case of growth hormone (GH)/TSH/prolactin (PRL) co-secreting mixed gangliocytoma-pituitary adenoma (MGPA) was discovered.
Conclusion
The majority of the TSH PitNET cases was macroadenoma, and 23% showed hormone co-secretion. A rare case of GH/TSH/PRL co-secreting MGPA was discovered. Serum α-SU and TRH stimulation tests showed great diagnostic sensitivity. Careful consideration is needed in diagnosing TSH PitNET. Achieving remission requires complete tumor resection. In case of nonremission, radiotherapy or medical therapy can improve the long-term remission rate.

Keyword

Thyrotropin-secreting pituitary neuroendocrine tumor; Pituitary neuroendocrine tumor; Central hyperthyroidism; Mixed gangliocytoma pituitary adenoma; alpha-Subunit

Figure

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  Fig. 1. Coronal T1-weighted pituitary magnetic resonance imaging of a 21-year-old man with growth hormone/thyroid-stimulating hormone/prolactin co-secreting mixed gangliocytoma-pituitary adenoma.

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