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Pituitary blastoma: a unique embryonal tumor.

Bernd W Scheithauer1, E Horvath, T W Abel

  • 1Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street, SW Rochester, MN 55905, USA. scheithauer.bernd@mayo.edu

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Pituitary blastoma, a rare neonatal tumor, shows arrested development and unchecked proliferation. This study details three cases, highlighting varied cell types and proliferation rates, suggesting different tumor grades.

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Area of Science:

  • Endocrinology and Pathology
  • Pediatric Oncology
  • Neuro-oncology

Background:

  • Pituitary blastoma is a rare, recently described tumor of the neonatal pituitary.
  • It exhibits differentiation towards Rathke epithelium and adenohypophysial cells, reflecting arrested pituitary development.

Observation:

  • This study reports on three additional cases of pituitary blastoma, all ACTH-producing.
  • Cases presented with varied clinical features, including ophthalmoplegia and third nerve palsy, and suprasellar mass with cavernous sinus invasion.
  • Histological analysis revealed complex tumors with Rathke epithelium-like glands, blastema cells, and large secretory cells, with mucin-producing goblet cells in one case.

Findings:

  • Immunohistochemical studies showed immunoreactivity for synaptophysin, chromogranin, keratins, ACTH, and beta endorphin in secretory cells.
  • MGMT immunolabeling ranged from 40-60%, with moderate to high mitotic and MIB-1 labeling in two cases, suggesting higher-grade tumors.
  • Ultrastructural examination identified blastema cells, folliculostellate cells, and large corticotroph cells.

Implications:

  • The findings support the diagnosis of pituitary blastoma, aligning it with blastomas in other organs and suggesting an underlying genetic abnormality.
  • Variations in cellular proliferation indicate the existence of low- and higher-grade pituitary blastomas.
  • Variable MGMT reactivity may imply an incomplete response to temozolomide therapy in some cases.