Long-Term Follow-Up in a Male Patient with Micro-TSH-Oma Diagnosed at 8-Yr-Old

Abstract

Triiodothyronine (T3) suppression test is generally reserved for patients with inconclusive results in above tests, because genetic tests for detection of mutations in thyroid receptor (TR)α and TRβ genes are expensive. Administering long-acting somatostatin analogs has been proposed for distinguishing between thyrotropinomas and RTH, since patients with thyrotropinomas would be likely to show a significant reduction in free thyroxine (fT 4 ) and T 3 levels. Approximately one third of patients with TSH-oma were misdiagnosed as having primary hyperthyroidism and mistakenly treated with thyroidectomy or radioiodine [2]. The majority of TSH-omas is monoclonal in origin, like other types of pituitary adenomas. Pituitary-specific transcription factor-1 (Pit-1) may play a role in adenomatous

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

“…Another feature of our case is the only paediatric case not developing hypopituitarism or other hormone deficiencies after two surgeries and gamma knife radiotherapy which was the usual outcome of all reported paediatric cases [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22]. For that reason, our patient did not require any hormone replacement therapy and spontaneously entered puberty and attained a final height of 161.3 cm.…”

“…However, the results of surgery in children are not as satisfactory as in adults. Only 7 patients (43.7%) including three with microadenoma experienced a full recovery after surgery of the 16 reported cases with known outcomes [7, 9-11, 17, 18, 23]. Therefore, the delayed diagnosis due to subtle symptoms causes extensive tumoral expansion, making the complete resection of the tumour impossible, and adjunctive treatments such as radiotherapy and/or somatostatin analogues are usually required.…”

“…For that reason, our patient did not require any hormone replacement therapy and spontaneously entered puberty and attained a final height of 161.3 cm. The final height had been reported only in 3 paediatric TSHoma cases [7, 8, 18]. Two of them had received GH replacement therapy due to hypopituitarism after surgeries [7, 18].…”

“…The final height had been reported only in 3 paediatric TSHoma cases [7, 8, 18]. Two of them had received GH replacement therapy due to hypopituitarism after surgeries [7, 18]. One male patient had a final height of 158.5 cm at the age of 16 years with a BA of 18 years when he presented [8].…”

“…Long-term follow-up and disease outcome are reported in only a few patients, the longest being 8 years. Final height has been reported in only 3 patients (two with GH replacement after surgery) [7, 8, 18]. In paediatric TSHomas, long-term outcomes, progression of the disease, and bone age (BA) progression under treatment are not well described due to the rarity of the disease and heterogenous hormone expression of the tumour.…”