Parathyroid hormone-related peptide-producing non-familial pheochromocytoma in a child
Parathyroid hormone-related peptide-producing non-familial pheochromocytoma in a child
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DOI：
10.1111/j.1442-2042.2010.02555.x
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发表时间：
2010-07-01
影响因子：
2.6
通讯作者：
Takahashi, Kota
中科院分区：
文献类型：
--
作者：
Takeda, Keisuke;Hara, Noboru;Takahashi, Kota
我们经历了一例产生甲状旁腺激素相关肽(PTHrP)的嗜铬细胞瘤，发现于一名12岁的高钙血症男孩。主要症状是腹痛，首次就诊时发现有严重的高血压和心动过速。他的病史和家族史并不引人注目。实验室检查显示高钙血症(3.3mmoL/L血钙)。CT显示右侧肾上腺内有一长约5.0厘米的不均匀肿块，123-I间苯基胍核素显像显示该肿块有异常摄取。血/尿儿茶酚胺显著升高，血清甲状旁腺素受体也显著升高(1.4pmol/L)。患者接受了腹腔镜右肾上腺切除术。组织学诊断为典型的嗜铬细胞瘤，免疫组织化学证实PTHrP的表达。术后血清甲状旁腺素水平恢复正常。术后12个月无病发生。目前尚无报道的儿童甲状旁腺素rp产生的嗜铬细胞瘤患者。我们的证据表明，现在的肿瘤是一种复杂的肿瘤，涉及多种神经内分泌活动，具有双重谱系分化。
We experienced a case of parathyroid hormone-related peptide (PTHrP)-producing pheochromocytoma, which was found in a 12-year-old boy with hypercalcemia. The leading symptom was abdominal pain, and severe hypertension and tachycardia were noticed at the initial visit. His medical and familial histories were unremarkable. Laboratory examinations showed hypercalcemia (3.3 mmol/L of serum-calcium). Computed tomography showed a heterogeneous mass measuring 5.0 cm in the right adrenal gland, which had abnormal uptake with 123-I metaiodobenzylguanidine scintigraphy. Serum/urine catecholamines were highly elevated, and serum PTHrP also increased (1.4 pmol/L). The patient underwent laparoscopic right adrenalectomy. The tumor was histologically diagnosed as typical pheochromocytoma and the expression of PTHrP was confirmed with immunohistochemistry. The serum PTHrP level was normalized after surgery. He was free of disease postoperatively for 12 months. There has been no described pediatric patient with PTHrP-producing pheochromocytoma. We showed evidence that the present tumor is a complex neoplasm involving various neuroendocrine activities with the dual-lineage differentiation.