Primary pituitary neuroendocrine tumor: Case report and literature review

Abstract

Background:Neuroendocrine tumors (NET) originate from the diffuse neuroendocrine system. These can arise in almost every organ of the body, although they are most commonly found in the gastrointestinal tract and respiratory system. The skull base and sellar region are extremely rare sites for neuroendocrine carcinoma. Consequently, in this case, both diagnosis and definition of surgical goals, as well as further treatment strategies were challenging.Case Description:A 65-year-old woman was admitted to our Neurosurgery Department with a rapidly progressive visus reduction, drowsiness, polyuria, and polydipsia. Neuroimaging showed a sellar/suprasellar mass (diameter of 2 cm) with a heterogeneous signal compressing the optic chiasm and extending laterally toward the cavernous sinus. Differential diagnosis based on imaging included pituitary macroadenoma or metastasis. The patient underwent endoscopic endonasal transsphenoidal surgery. A total resection of the mass was impossible because of the infiltration of the optic chiasm and the intraoperative histological diagnosis of malignant epithelial neoplasm. Further histological evaluation revealed that the lesion was a NET with no other primary or metastatic sites detectable. Subsequently, the patient was successfully treated with fractioned stereotactic radiotherapy and polychemotherapy. Four years after the surgery, follow-up magnetic resonance imaging showed stability of the residual disease. Neurologic examination revealed a complete visual recovery.Conclusions:Primary pituitary NET, though rare, should be included in the differential diagnosis of sellar lesions. A multimodality treatment approach is needed. Finally, the present case highlights, that in the case of a pituitary lesion infiltrating the optic chiasm, including NET, the endoscopic endonasal transsphenoidal subtotal resection followed by fractioned stereotactic radiotherapy and chemotherapy may represent an effective and safe choice of treatment.

Downregulation of glucose‑6‑phosphate dehydrogenase by microRNA‑1 inhibits the growth of pituitary tumor cells

“…Pituitary tumors are considered as one of the most common intracranial neoplasms of the central nervous system (1,2). Although the majority of pituitary adenomas are benign tumors, ~25% of the tumors invade areas of the intracranial region (3).…”

Primary Intracranial Neuroendocrine Tumor of the Skull Base Complicated with Tension Pneumocephalus after Radiotherapy

“…4) Primary intracranial NETs are extremely rare entities, and to date, only 12 cases have been reported in the literature. 1,[4][5][6][7][8][9][10][11][12][13] Most were located in the skull base and sellar regions, except for one case each in the third ventricle, 1) frontal convexity, 6) parietal lobe, 9) and cerebellum. 13) Based on the pathological findings of the present case, we consider that the differential diagnosis of NETs should include esthesioneuroblastoma and ectopic sparsely granulated corticotroph adenoma (SGCA).…”

“…Esthesioneuroblastoma usually comprises neuroblasts with uniform circular nuclei and longitudinal cerebromedullary tubes in the dendrites, but NETs originate from the epithelium and lack neural features. [6][7][8]10,11) On electron microscopy, verification of the presence of intracytoplasmic neuroendocrine granules is highly valuable for the differential diagnosis of NETs. 10) Intraoperative findings in this case indicated that the sellar dura was preserved and there was no continuity between the tumor and the pituitary gland.…”

“…Some authors reported success with postoperative treatment consisting of radiotherapy, including IMRT or fractioned stereotactic radiotherapy, with or without chemotherapy. 1,8,9,11) Radiotherapy seems to be more effective in poorly differentiated NENs. 10) Given the advanced age of our patient, we performed only tumor biopsy via the nasal cavity, followed by focal radiotherapy; this led to a marked reduction in tumor size, which led to the development of tension pneumocephalus.…”

Matrine inhibits the proliferation of pituitary tumor cells by decreasing Foxo3a phosphorylation and promoting Foxo3a nuclear localization

“…Transsphenoidal surgery is usually considered as the first-choice therapy for most patients with pituitary tumors, which is an effective and safe treatment (2). Comprehensive therapy is gradually important among endocrinologists, ophthalmologists, and radiologists (3). Thus, it is helpful to clarify the pathogenesis and progression of pituitary tumors.…”