Coexistence Tumor of Craniopharyngioma and Pituitary Neuroendocrine Tumor: A Case Report and Literature Review

TL;DR

A rare case of two brain tumors coexisting in the same patient is described, highlighting the importance of accurate diagnosis and tailored surgical approaches.

Contribution

This case report highlights the rare coexistence of craniopharyngioma and pituitary neuroendocrine tumor and emphasizes the importance of preoperative recognition for surgical planning.

Findings

A 72-year-old man had two distinct tumors: a craniopharyngioma and a pituitary neuroendocrine tumor.

Endoscopic surgery successfully removed both lesions, resulting in biochemical remission of acromegaly.

Preoperative recognition of separated tumors allowed tailored resection and preserved pituitary-hypothalamic function.

Abstract

Acromegaly most commonly results from excess growth hormone (GH) produced by pituitary neuroendocrine tumors (PitNETs). Craniopharyngioma (CP) is an uncommon suprasellar tumor characterized by cystic change and calcification. The true coexistence of PitNET and CP is rare and poses diagnostic and operative challenges, particularly when lesions are spatially separated.

A 72-year-old man was referred for a pituitary mass and clinical features suggestive of acromegaly (enlarged extremities, prominent supraorbital ridge, and jaw enlargement) without headache or visual complaints. MRI demonstrated two distinct lesions: a heterogeneous, mainly cystic suprasellar mass measuring 2.2×1.2×1.2 cm compressing the optic chiasm and extending toward the third ventricle, and a separate 1.7×1.2×1.1 cm intrasellar lesion extending into the sphenoid sinus. CT showed calcification within the suprasellar lesion. Baseline hormones revealed elevated GH (7.60 ng/mL) and IGF-1 (388 ng/mL; +5.7 SD) with inadequate GH suppression on oral glucose tolerance testing (nadir GH 2.68 ng/mL). An endoscopic endonasal transsphenoidal approach was undertaken. Intraoperatively, a white, soft intrasellar tumor breaching the dura and protruding into the sphenoid sinus was removed, and a separate, highly calcified suprasellar tumor was internally decompressed and dissected free; no macroscopic continuity was identified. Histopathology showed a densely granulated mixed somatotroph-lactotroph PitNET (immunoreactive for GH, prolactin, Pit-1, and alpha subunit; CAM5.2 perinuclear pattern <70%; Ki-67 <1%) and an adamantinomatous CP with wet keratin and calcified nests. Postoperatively, GH fell to 0.36 ng/mL and IGF-1 to 166 ng/mL (+1.2 SD). Transient diabetes insipidus occurred and was controlled with desmopressin. The patient was discharged without new neurological deficits, and an MRI at four months showed no recurrence.

This case underscores the value of recognizing “separated” coexistence of CP and PitNET, in which discrepant imaging features (calcified, cystic suprasellar mass versus enhancing intrasellar lesion) can suggest dual pathology before surgery. Clear preoperative identification facilitates tailored resection strategies, allows focused manipulation of each lesion, and helps balance the competing priorities of gross-total removal and preservation of pituitary-hypothalamic function. Early biochemical remission of acromegaly and an uncomplicated radiographic course support the efficacy and safety of an endoscopic endonasal approach in selected patients.

When intra- and suprasellar lesions demonstrate discordant radiologic characteristics in a patient with biochemical acromegaly, concomitant CP and PitNET should be considered. Distinguishing separated from admixed disease preoperatively can guide operative planning and reduce morbidity. Targeted endoscopic resection achieved prompt hormonal normalization and radiographic disease control in this patient, emphasizing the importance of individualized, anatomy-driven management and vigilant postoperative endocrine and imaging follow-up.

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