Mancini Alyssa J, Mathew Ribu, Oentoro Jaymie, Devine Alma M, Maxwell Carolyn, Kravets Igor
Division of Endocrinology and Metabolism, Department of Medicine, Renaissance School of Medicine at Stony Brook University, Stony Brook, New York.
Department of Pathology, Renaissance School of Medicine at Stony Brook University, Stony Brook, New York.
AACE Clin Case Rep. 2024 Apr 16;10(4):144-148. doi: 10.1016/j.aace.2024.04.003. eCollection 2024 Jul-Aug.
BACKGROUND/OBJECTIVE: Collision tumors composed of craniopharyngiomas and pituitary adenomas are extremely rare. We report a collision tumor formed by a papillary craniopharyngioma and a growth hormone-secreting pituitary adenoma, which is the first report of such a tumor, to the best of our knowledge.
A 49-year-old man presented with 2 months of headaches and blurry vision. An exam demonstrated frontal bossing, enlarged jaw and hands, macroglossia, and bitemporal hemianopsia, and magnetic resonance imaging (MRI) showed a 4.1 cm sellar/suprasellar mass with mass effect on the optic chiasm. The tumor was resected twice via a craniotomy, the second time due to interval growth, with the pathology after both surgeries showing a papillary craniopharyngioma. IGF-1 was 517 ng/mL (68-225) and growth hormone suppression test was positive. Repeat MRI showed residual tumor with ongoing mass effect on the optic chiasm and radiation therapy was initiated. MRI showed interval growth of the mass and IGF-1 rose to 700 ng/mL after which the patient underwent a transsphenoidal resection of the tumor; the pathology showed a residual papillary craniopharyngioma and a PIT1 lineage adenoma with most cells expressing growth hormone. After developing numerous complications, the patient passed away.
Collision tumors of the sella are often associated with an aggressive clinical course, as they often go undiagnosed preoperatively, thus reducing the likelihood of total resection and leading to higher rates of craniopharyngioma recurrence.
A pituitary mass with an aggressive clinical course should prompt a high index of suspicion for a sellar collision tumor, though prognosis remains poor.
背景/目的：由颅咽管瘤和垂体腺瘤组成的碰撞瘤极为罕见。据我们所知，我们报告了一例由乳头状颅咽管瘤和分泌生长激素的垂体腺瘤形成的碰撞瘤，这是此类肿瘤的首例报告。
一名49岁男性出现2个月的头痛和视力模糊。检查发现额部隆起、下颌和手部增大、巨舌症以及双颞侧偏盲，磁共振成像（MRI）显示鞍区/鞍上有一个4.1厘米的肿块，对视交叉有占位效应。该肿瘤通过开颅手术切除了两次，第二次是因为肿瘤间隔生长，两次手术后的病理检查均显示为乳头状颅咽管瘤。胰岛素样生长因子-1（IGF-1）为517纳克/毫升（68 - 225），生长激素抑制试验呈阳性。复查MRI显示有残留肿瘤，对视交叉仍有占位效应，遂开始进行放射治疗。MRI显示肿块间隔生长，IGF-1升至700纳克/毫升，之后患者接受了经蝶窦肿瘤切除术；病理检查显示残留乳头状颅咽管瘤和一个PIT1谱系腺瘤，大多数细胞表达生长激素。在出现多种并发症后，患者去世。
鞍区碰撞瘤通常与侵袭性临床病程相关，因为它们术前常未被诊断，从而降低了全切的可能性，并导致颅咽管瘤复发率更高。
具有侵袭性临床病程的垂体肿块应引起对鞍区碰撞瘤的高度怀疑，尽管预后仍然很差。
