1型神经纤维瘤病患者并发肾上腺皮质癌：一例报告。
Adrenocortical carcinoma in a patient with neurofibromatosis type 1: A case report.
作者信息
Najafi-Semnani Mohammad, Rajabi-Moghaddam Mahdieh, Abbaszadeh Hamid
机构信息
Department of Urology, School of Medicine, Birjand University of Medical Sciences, Birjand, Iran.
Department of Pathology, School of Medicine, Birjand University of Medical Sciences, Birjand, Iran.
出版信息
Caspian J Intern Med. 2021 Fall;12(4):613-617. doi: 10.22088/cjim.12.4.613.
BACKGROUND
Neurofibromatosis type 1 (NF-1) is a genetic disorder. A heterogeneous group of benign and malignant neoplasms are associated with NF-1. Adrenocortical carcinoma (ACC) is an extremely rare invasive malignancy. The association of ACC with NF1 is not well understood.
CASE PRESENTATION
We report a case of ACC in the context of NF1 in a 39- year-old woman who referred with the chief complaint of a mass in left abdomen. A left adrenal lesion was diagnosed by CT scan. Biochemical tests showed no abnormality. Adrenalectomy was done and histological and immunohistochemical findings confirmed ACC. Due to the absence of metastasis, clinical stage II was considered for the tumor. On follow-up after six months, she was still alive and well and with no evidence of metastasis. The age of patient and lack of secretion of adrenal cortical hormones in this case were unlike most ACCs.
CONCLUSION
Also, modified Weiss score for malignancy of adrenocortical neoplasms, clinical staging system and different modality of treatment is discussed.
背景
1型神经纤维瘤病（NF-1）是一种遗传性疾病。一组异质性的良性和恶性肿瘤与NF-1相关。肾上腺皮质癌（ACC）是一种极其罕见的侵袭性恶性肿瘤。ACC与NF1之间的关联尚不清楚。
病例报告
我们报告一例39岁女性在NF1背景下发生ACC的病例，该患者以左腹部肿块为主诉前来就诊。CT扫描诊断为左肾上腺病变。生化检查未发现异常。进行了肾上腺切除术，组织学和免疫组化结果证实为ACC。由于没有转移，该肿瘤被认为处于临床II期。随访6个月后，她仍然健在，没有转移的迹象。该病例中患者的年龄以及肾上腺皮质激素无分泌情况与大多数ACC不同。
结论
此外，还讨论了肾上腺皮质肿瘤恶性程度的改良Weiss评分、临床分期系统以及不同的治疗方式。
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