Craniopharyngiomas (CPs) are rare intracranial tumors arising from remnants of Rathke’s pouch. Despite their benign histology, CPs present considerable clinical challenges due to their tendency to exert mass effect and adherence to critical neurovascular structures. There remains no clear consensus on the most effective management of CPs. We explore the latest developments in targeted treatment approaches, examining how innovations in surgery, radiation therapy, and emerging therapies are improving outcomes and management for these challenging tumors. In addition to providing our experience, we reviewed previously reported case series and reviews relevant to CPs. Studies show a bimodal age distribution for CP diagnosis, with peak incidence occurring in children aged 5–14 years and in adults aged 50–74 years. Surgical resection is the typical initial treatment for CPs, and adjuncts, including radiation therapy and intracavitary treatments, have been proven effective for tumor control. Additionally, genetic mutations associated with CPs offer an opportunity for novel strategies that address the underlying molecular mechanisms driving tumor growth through targeting the Wnt/β-catenin and/or MAPK/ERK pathways to disrupt the aberrant signaling that promotes tumor proliferation and survival. Survival rates for CPs are generally favorable (five-year survival of 80%), with recent studies showing improved outcomes and higher survival rates in children. CPs remain rare and challenging tumors. Although surgical resection is the main treatment, surgeons must weigh the benefits of achieving a gross total resection with the risks of iatrogenic injury. Adjuncts, including intracavitary and radiation treatments, may assist with subtotal resections and recurrences, as well as approved BRAF inhibitor therapy for the papillary variant. Many improvements in diagnostic and therapeutic methods were made after Dr. Cushing coined the term “craniopharyngioma”. Ongoing experiments, investigations, and advances in radiation techniques and molecular targeted therapies will provide patients with promise for safer and more effective treatments.

Background: Autoimmune hypophysitis (AH), an uncommon inflammatory disorder of the pituitary gland, involves immune cell infiltration into pituitary tissue and often occurs concurrently with systemic autoimmune diseases. Although Hashimoto’s thyroiditis, systemic lupus erythematosus, and IgG4-related disease have been associated with AH, rheumatoid arthritis (RA)-associated AH remains exceedingly rare, particularly in seropositive cases. Clinical and imaging characteristics of AH mimic a wide range of infectious, neoplastic, and infiltrative pathologies. Biopsy remains the gold standard for definitive diagnosis, though it is rarely pursued. Methods: A retrospective chart review was performed to obtain the details regarding patient history, histopathological findings, and treatment course. Results: This rare case of biopsy-proven AH involved a 46-year-old female with seropositive RA. The patient presented with progressive headaches, small scotomas in both visual fields, and multiple endocrinopathies including central hypothyroidism and adrenal insufficiency. Magnetic resonance imaging revealed an infiltrative, contrast-enhancing suprasellar lesion. Surgical intervention was pursued for biopsy and debulking. Histopathology demonstrated pituitary tissue infiltrated by a heterogeneous population of inflammatory cells without evidence of well-formed granulomas. In the context of positive rheumatoid factor (RF) and anticyclic citrullinated peptide antibodies, the diagnosis of RA-associated AH was favored. The patient was treated with azathioprine with resolution of her headaches and scotomas and radiographic improvement. To our knowledge, this case represents the first biopsy-proven example of seropositive RA-associated AH. Conclusion: This case underscores the importance of maintaining a broad differential diagnosis when evaluating pituitary lesions, especially in patients with systemic autoimmune disorders, and highlights the role of tissue diagnosis and decompression in a symptomatic patient with a suprasellar lesion resulting in mass effect.