Kobayashi Ai, Ishinoda Yuki, Uto Asuka, Ogata Sho, Oshima Naoki
Department of Endocrinology, National Defense Medical College, Saitama, JPN.
Department of Laboratory Medicine, National Defense Medical College, Saitama, JPN.
Cureus. 2024 Mar 25;16(3):e56878. doi: 10.7759/cureus.56878. eCollection 2024 Mar.
I-metaiodobenzylguanidine (I-MIBG) scintigraphy is a highly sensitive and specific imaging test for the diagnosis of pheochromocytoma. Typical pheochromocytomas are positive on I-MIBG scintigraphy; however, cases of paragangliomas eliciting negative results have been reported. We encountered a case of hypertensive crisis resulting in extensive coagulative necrosis of a pheochromocytoma and negative findings on I-MIBG scintigraphy. A 50-year-old Japanese female presented with an acute onset of vomiting, epigastralgia, and abdominal pain. Immediately after contrast-enhanced CT, the patient developed respiratory failure and was intubated. The CT scan revealed a 5-cm left adrenal mass, and a pheochromocytoma crisis was suspected. The patient's condition stabilized following phentolamine administration. Regarding the assessment for pheochromocytoma, plasma metanephrine levels were not markedly increased, and I-MIBG scintigraphy was negative. However, a histological examination of the left adrenal mass revealed extensive coagulative necrosis of the entire adrenal mass, comprising trabecular and alveolar growth of large polygonal cells that were immunopositive for chromogranin A/synaptophysin, thereby suggesting a diagnosis of pheochromocytoma. There have been three reported cases of I-MIBG scintigraphy-negative pheochromocytomas because of pure avascular necrosis without hemorrhage or rupture. To the best of our knowledge, this is the first reported case of massive tumor necrosis due to hypertensive crisis exacerbated after contrast-enhanced CT imaging. In conclusion, pheochromocytoma cannot be ruled out even with negative findings on I-MIBG scintigraphy. Accordingly, clinical judgment must be made based on a comprehensive assessment of the clinical course and pathological diagnosis, especially for cases involving a hypertensive crisis.
间碘苄胍(I-MIBG)闪烁扫描术是诊断嗜铬细胞瘤的一种高度敏感且特异的影像学检查。典型的嗜铬细胞瘤在I-MIBG闪烁扫描术中表现为阳性;然而,也有副神经节瘤呈阴性结果的病例报道。我们遇到了一例因高血压危象导致嗜铬细胞瘤广泛凝固性坏死且I-MIBG闪烁扫描术结果为阴性的病例。一名50岁的日本女性出现急性呕吐、上腹部疼痛和腹痛。在增强CT检查后,患者立即出现呼吸衰竭并接受了插管。CT扫描显示左肾上腺有一个5厘米的肿块,怀疑为嗜铬细胞瘤危象。给予酚妥拉明后患者病情稳定。关于嗜铬细胞瘤的评估,血浆甲氧基肾上腺素水平未明显升高,I-MIBG闪烁扫描术结果为阴性。然而,对左肾上腺肿块的组织学检查显示整个肾上腺肿块广泛凝固性坏死,由大的多边形细胞呈小梁状和腺泡状生长,嗜铬粒蛋白A/突触素免疫阳性,从而提示诊断为嗜铬细胞瘤。已有3例因单纯无血管坏死且无出血或破裂导致I-MIBG闪烁扫描术阴性的嗜铬细胞瘤病例报道。据我们所知,这是首例因增强CT成像后高血压危象加重导致肿瘤大片坏死的病例报道。总之,即使I-MIBG闪烁扫描术结果为阴性也不能排除嗜铬细胞瘤。因此,必须基于对临床病程和病理诊断的综合评估进行临床判断,尤其是对于涉及高血压危象的病例。