下垂体性TSH分泌亢進症

BACKGROUND: Thyroid-stimulating hormone (TSH)-secreting pituitary adenoma (TSHoma) is a rare functional pituitary tumor that secretes TSH independently, thus stimulating the synthesis and secretion of thyroid hormone. Its clinical manifestation is central hyperthyroidism, and it is easily misdiagnosed as Graves' disease. At present, the treatment of TSHoma includes surgery, medication, and radiotherapy, and transsphenoidal pituitary surgery remains the first-line treatment. CASE PRESENTATION: This case involved a 61-year-old Chinese male with clinical manifestations of weight loss, heart palpitations, and shortness of breath, both thyroid hormone and TSH are elevated. The patient was diagnosed with a TSH/growth hormone (GH) co-secreting pituitary adenoma by serological examination, magnetic resonance imaging of the pituitary gland, and related functional tests. Due to thrombocytopenia and heart disease, the patient was deemed a poor candidate for immediate surgery and was therefore treated with octreotide. Thyroid function tests normalized after medication, and the diagnosis of TSHoma was verified. CONCLUSION: Although TSHoma is rare, it is not difficult to diagnose based on clinical presentation, ancillary examination, and functional testing. Thyroid hormone resistance syndrome is very similar to it and is therefore easily misdiagnosed. Early diagnosis and appropriate treatment can effectively control the disease and even achieve a clinical cure.

Concurrent thyrotropin (TSH) secreting and growth hormone (GH) secreting pituitary adenomas are rare entities. Although tumors of the PIT-1 lineage may coexpress TSH and GH, concurrent biologically active secretion resulting in central hyperthyroidism and acromegaly is uncommon. We report the case of a 42-year-old woman presenting with weight loss, palpitations, amenorrhea, and progressive acromegalic features. Biochemical evaluation revealed central hyperthyroidism with elevated free T4 and inappropriately normal TSH, markedly elevated insulin-like growth factor 1 (IGF-1) and GH, hyperprolactinemia, and elevated α-subunit. Pituitary magnetic resonance imaging demonstrated a 2.7-cm macroadenoma with cavernous sinus extension. She underwent transsphenoidal resection without complications. Pathology confirmed a mature plurihormonal PIT-1 lineage adenoma immunopositive for TSH, GH, and prolactin with a Ki-67 of 3%. Immediately postoperatively, thyrotoxicosis and hyperprolactinemia resolved, IGF-1 levels declined, acromegalic features improved, and menses resumed. Over the ensuing months, thyroid and prolactin tests remained within normal range, but the IGF-1 level remained elevated. This case illustrates the rare presentation of a PIT-1 lineage adenoma secreting biologically active TSH, GH, and prolactin. Comprehensive hormonal evaluation and long-term biochemical and radiologic surveillance are essential, given the risk of incomplete remission despite apparent surgical cure.

Pituitary adenomas are common intracranial neoplasms and represent a clinically relevant but frequently underrecognized cause of secondary osteoporosis and fragility fractures. Hormonal excess, coexisting pituitary deficiencies, and the iatrogenic burden of multimodal treatments act synergistically to impair bone remodeling, microarchitecture, and mechanical strength. Consequently, skeletal fragility may manifest at a relatively young age and is often inadequately predicted by dual-energy X-ray absorptiometry (DXA)-derived areal bone mineral density (aBMD) alone.This narrative review summarizes current evidence on skeletal involvement in functioning pituitary adenomas, including acromegaly, Cushing's disease (CD), prolactinomas, and thyrotropin [TSH]-secreting adenomas (TSHomas), with a specific focus on sex-related differences relevant to clinical practice. Although several pituitary adenomas are more prevalent in women, male patients frequently present with delayed diagnoses, harboring larger and more aggressive tumors. Consequently, men may be affected by a longer cumulative exposure to hormone excess and a higher prevalence and severity of panhypopituitarism, often exacerbated by the mass effect and multiline therapies. Across clinical cohorts, these interacting factors are consistently associated with an increased burden of fragility fractures, particularly in male patients.In pituitary disorders, fracture risk is commonly underestimated because DXA-derived aBMD does not adequately capture alterations in bone quality and structural integrity. Comprehensive skeletal evaluation, including systematic vertebral morphometry, trabecular bone score (TBS), and selected advanced imaging techniques, improves detection of occult fractures and refines fracture risk stratification. Moreover, the persistently low awareness, diagnosis, and treatment rates of osteoporosis in men represent a substantial and potentially modifiable gap in care, particularly in secondary forms related to endocrine diseases.This review highlights the need for a proactive, sex-aware clinical approach that integrates endocrine management with appropriate skeletal assessment and bone-directed therapy, with the aim of reducing fracture burden and improving long-term outcomes in patients with pituitary diseases.

BACKGROUND: Thyroid-stimulating hormone (TSH)-secreting pituitary adenomas (TSHomas) are rare lesions that result in secondary hyperthyroidism. MR elastography (MRE) is an emerging imaging modality capable of assessing the mechanical properties of tissues, offering valuable insights into tumor consistency for improved preoperative planning and resection. OBSERVATIONS: A 16-year-old female diagnosed with a giant TSHoma underwent endoscopic transnasal transsphenoidal excision. The mechanical properties of TSHoma were assessed using preoperative MRE and in vitro nanoindentation on excised specimen. Collagen content was also evaluated. Based on its appearance on MRE, a neuroradiologist predicted the tumor would be soft, and it was found to be soft during the surgery. Interestingly, the in vitro shear storage and loss moduli closely match the in vivo findings. Additionally, the tumor tissue has up to 20% collagen content in intratumoral areas. LESSONS: MRE is a reliable and clinically valuable tool for preoperative evaluation of tumor consistency in rare TSHomas. The strong concordance between MRE findings, intraoperative texture, and in vitro mechanical measurements underscores the potential of MRE to guide surgical planning, reduce operative complexity, and improve clinical outcomes, especially in uncommon and challenging cases. https://thejns.org/doi/10.3171/CASE25402.

PURPOSE: Immature PIT-1 lineage tumors are classified as high-risk pituitary adenomas/pituitary neuroendocrine tumors (PitNETs) under the 2022 WHO Classification, yet clinical outcome data remain scarce. This study aimed to characterize clinicopathological features and treatment outcomes of this rare entity. METHODS: We retrospectively analyzed 13 patients with pathologically confirmed immature PIT-1 lineage tumors who underwent surgery at our tertiary center between January 2022 and December 2024. Diagnoses were established according to WHO 2022 criteria using comprehensive transcription factor (TF) immunohistochemistry. Clinical, radiological, histopathological, treatment, and follow-up data were evaluated. RESULTS: Immature PIT-1 lineage tumors accounted for 4% (21/525) of surgically treated pituitary adenomas, of whom 13 with complete follow-up data constituted the primary analysis cohort. Median age was 37 years (range: 25-73), with female predominance (61.5%). Notably, 61.5% presented with hormonal hypersecretion: acromegaly (38.5%), TSH-secreting tumors (15.4%), and GH-PRL co-secretion (7.7%). All patients had macroadenomas (median diameter 28 mm (range: 15-59 mm)); 38.5% demonstrated cavernous sinus invasion (Knosp grades 3-4). Immunohistochemically defined plurihormonal phenotype was present in 77% of cases; cytologic atypia was identified in all cases. Despite maximal surgical resection, residual disease persisted in 46% and 31% required reoperation at 16-month follow-up (range: 4-51 months). The median Ki-67 proliferation index was 4% (IQR: 2-10%; range: 1-35%), and the median mitotic count was 4 per 2 mm² (IQR: 1-8; range: 1-20). CONCLUSION: Immature PIT-1 lineage tumors exhibit aggressive behavior with high residual disease rates, with 31% of patients requiring reoperation, 23% receiving adjuvant radiotherapy, and somatostatin analogue resistance observed in two patients. Routine TF immunohistochemistry is essential for accurate diagnosis; management requires maximal safe surgical resection and intensive long-term surveillance.