Malignant spiradenoma in a patient with Recklinghausen syndrome: a potential association
Surgical Pathology Department, Santo Spirito Hospital, Viale Giolitti 2, Casale Monferrato, Italy.
Research Article
GSC Biological and Pharmaceutical Sciences, 2020, 12(03), 236-240.
Article DOI: 10.30574/gscbps.2020.12.3.0306
Publication history:
Received on 20 September 2020; revised on 29 September 2020; accepted on 30 September 2020
Abstract:
Recklinghausen syndrome or neurofibromatosis type 1(NF1) is a hereditary condition transmitted with a dominant autosomal inheritance pattern. It’s a systemic disorder characterized by involvements of skin, nerves, bone, soft tissues, iris and nervous system. Specifically a mutation of one allele of the oncosoppressor gene NF1 is the cause of the syndrome. Manifestations of neurofibromatosis include: benign tumors like neurofibromas, pheocromocytoma, juvenile xanthogranulomas, Lisch nodules, café-au-lait macules and freckles. Neurofibromatosis also correlates with an increased risk for malignant tumors like malignant melanoma, leukemia, brain gliomas. In this manuscript we report the association of NF1 with a malignant spiradenoma. A literature review has been made to investigate a possible correlation between these two entities.
Keywords:
Neurofibromatosis type 1; Malignant spiradenoma; Neurofibromin; Ras-pathway; NF-kB pathway
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Copyright © 2020 Author(s) retain the copyright of this article. This article is published under the terms of the Creative Commons Attribution Liscense 4.0
