Caspian Journal of Internal Medicine
Babol University of Medical Sciences
Fri, Feb 7, 2025
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Volume 12, Issue 4 (Autumn 2021)
Caspian J Intern Med 2021, 12(4): 613-617 |
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Najafi-Semnani M, Rajabi-Moghaddam M, Abbaszadeh H. Adrenocortical carcinoma in a patient with Neurofibromatosis type 1: a case report. Caspian J Intern Med 2021; 12 (4) :613-617
URL: http://caspjim.com/article-1-2752-en.html
URL: http://caspjim.com/article-1-2752-en.html
Department of Urology, School of Medicine, Birjand University of Medical Sciences, Birjand, Iran , abbaszadehbidokhty@gmail.com
Abstract: (2695 Views)
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.
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.
Type of Study: case report |
Subject:
Oncology
Received: 2021/02/4 | Accepted: 2021/04/10 | Published: 2021/09/19
Received: 2021/02/4 | Accepted: 2021/04/10 | Published: 2021/09/19
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