Pituitary & hypothalamus

Posterior pituitary tumors: review of histogenetic continuum, molecular drivers and targetable therapies

PituitaryPublished review

Authors' conclusion

These genomic and immune discoveries provide a rational for targeted therapy for refractory PPTs.

Study context

Population
Posterior pituitary tumors (PPTs): pituicytomas, spindle cell oncocytomas (spindle cell oncocytoma (SCOs)), granular cell tumors (granular cell tumor (GCTs)) and ependymal pituicytomas
Outcome
Clinical presentation and treatment

Main finding

As reported in the abstract

  • PPTs may mimic non-functioning pituitary adenomas or present as suprasellar masses, and can cause neurological and endocrine deficits due to mass effect
  • Surgery remains the treatment of choice, with radiotherapy considered for selected patients

Further findings

Chromosomal copy number variations (CNV) and prognostic subtypes

CNV-negative pituicytomas and SCOs associated with more favorable outcomes than the CNV-positive subtypes, and a third group composed of GCTs that have favorable outcomes

Molecular drivers

Pituicytomas and SCOs are enriched for pathogenic variants in the mitogen-activated protein kinase (MAPK) and PI3K signaling pathways associated with downstream pERK activation, whereas GCTs are characterized by relative genomic stability

Tumor immune microenvironment

SCOs display an immune-active microenvironment, contrasting with the highly immunosuppressive profile observed in pituicytomas

Original publication

Posterior pituitary tumors: review of histogenetic continuum, molecular drivers and targetable therapies. Pituitary. Published 13 November 2026. doi:10.1007/s11102-026-01786-3

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