Sato Tetsuko, Wada Yoshihisa, Kamitani Nobuhiko, Yamamoto Takenobu, Aoyama Yumi, Fujimoto Wataru, Tanaka Ryo
Department of Dermatology, Kawasaki Medical School, Kurashiki, Japan.
Department of Nephrology/Hypertension, Kawasaki Medical School, Kurashiki, Japan.
Case Rep Oncol. 2020 Sep 30;13(3):1209-1214. doi: 10.1159/000510442. eCollection 2020 Sep-Dec.
Hypercalcemia of malignancy occurs in up to one third of patients at some point during the course of their advanced stage. The majority of them is caused by humoral hypercalcemia of malignancy due to systemic secretion of parathyroid hormone-related protein (PTHrP) by tumor cells. Extramammary Paget's disease is a slow-growing cutaneous malignancy commonly limited to the epidermis of the anogenital region, but rarely becomes invasive and metastatic to distant sites. Herein, we report a 70-year-old male patient with metastatic extramammary Paget's disease. He consulted our hospital with altered consciousness and tumor in his genital area. Physical examination revealed erythematous plaque with a tumor on the scrotum and perineum. It was diagnosed as extramammary Paget's disease (multiple liver metastases and multiple lymph node metastases by skin biopsy and image examination). Increases in serum-corrected calcium and PTHrP-intact levels (15.3 mg/dL and 66.1 pg/L, respectively) were confirmed. PTHrP immunohistochemistry showed positive staining in the tumor cells. We diagnosed humoral hypercalcemia of malignancy. We treated hypercalcemia with saline, furosemide, zoledronic acid, and elcatonin. Regarding the local control of the tumor, 30 Gy/10 Fr electron beam therapy was performed. However, treatment with zoledronic acid was only temporally effective to correct hypercalcemia, and an increased serum calcium level developed again. Concurrently, the liver metastases were rapidly enlarged, and his general condition gradually deteriorated. The patient died on day 55. When patients with extramammary Paget's disease show unconsciousness, serum calcium level should be measured and PTHrP-producing tumor distinguished.
恶性肿瘤高钙血症在高达三分之一的晚期患者病程中的某个阶段出现。其中大多数是由恶性肿瘤体液性高钙血症引起的，这是由于肿瘤细胞系统性分泌甲状旁腺激素相关蛋白（PTHrP）所致。乳房外佩吉特病是一种生长缓慢的皮肤恶性肿瘤，通常局限于肛门生殖器区域的表皮，但很少发生侵袭并转移至远处部位。在此，我们报告一名70岁男性乳房外佩吉特病转移患者。他因意识改变和生殖器区域肿物前来我院就诊。体格检查发现阴囊和会阴有红斑性斑块伴肿物。经皮肤活检和影像学检查诊断为乳房外佩吉特病（多发肝转移和多发淋巴结转移）。确认血清校正钙和完整PTHrP水平升高（分别为15.3mg/dL和66.1pg/L）。PTHrP免疫组化显示肿瘤细胞呈阳性染色。我们诊断为恶性肿瘤体液性高钙血症。我们用生理盐水、呋塞米、唑来膦酸和降钙素治疗高钙血症。关于肿瘤的局部控制，进行了30Gy/10次分割的电子束治疗。然而，唑来膦酸治疗仅在短期内有效纠正高钙血症，血清钙水平再次升高。同时，肝转移迅速增大，他的一般状况逐渐恶化。患者于第55天死亡。当乳房外佩吉特病患者出现意识不清时，应检测血清钙水平并鉴别产生PTHrP的肿瘤。
