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Case report: Remarkable efficacy of negative-pressure wound therapy in giant lower extremity elephantiasis neuromatosa for vascularization, skin grafting, and fluid control.
Int J Surg Case Rep
March 2024
Division of Plastic Reconstructive and Aesthetic Surgery, Department of Surgery, Faculty of Medicine, Universitas Padjadjaran, Bandung, Indonesia.
Introduction And Importance: Neurofibromatosis type 1 is a benign peripheral nerve tumor, often manifests as plexiform neurofibroma that may cause severe dysfunction, pain, and disfigurement. Bleeding has been reported as a complication of plexiform neurofibroma due to vascular fragility and vasculopathy that may develop into life-threatening bleeding especially after excision procedure. Consequently, post excision complications also include dehiscence and infection.
View Article and Find Full Text PDFClinical impact of selumetinib on pediatric elephantiasis neuromatosa.
Pediatr Dermatol
September 2022
Department of Hematology Oncology, Baylor College of Medicine, The Children's Hospital of San Antonio, San Antonio, Texas, USA.
Elephantiasis neuromatosa (EN) is a rare and extreme form of plexiform neurofibroma in patients with neurofibromatosis type 1 (NF1). EN is often associated with significant morbidity and remains difficult to treat. We present a case of an 11-year-old female with NF1 whose thoracolumbar plexiform neurofibroma and lower extremity EN exhibited clinical improvement from treatment with selumetinib, a selective MEK inhibitor.
View Article and Find Full Text PDFArteriovenous Malformations in the Setting of Giant Elephantiasis Neuromatosa.
Mayo Clin Proc
July 2021
Department of Radiology, Yantai Affiliated Hospital of Binzhou Medical University, Yantai, China. Electronic address:
A Boot Brace for Elephantiasis Neuromatosa Associated with Neurofibromatosis Type 1.
Plast Reconstr Surg Glob Open
August 2019
Department of Plastic and Reconstructive Surgery, Graduate School of Medicine and Faculty of Medicine, Kyoto University, Kyoto, Japan.
Giant elephantiasis neuromatosa in the setting of neurofibromatosis type 1: A case report.
Oncol Lett
June 2016
Department of Diagnostic and Clinical Medicine and Public Health, University of Modena and Reggio Emilia, I-41100 Modena, Italy.
Elephantiasis neuromatosa (EN) can arise from a plexiform neurofibroma of the superficial and deep nerves developing from a hyperproliferation of the perineural connective tissue infiltrating adjacent fat and muscles. To date, the clinical association between EN and neurofibromatosis type 1 (NF1) has been poorly defined, particularly with regard to the role of lymphatic alterations and the consequent lymphedema. The present study reports the clinical and biomolecular features of EN in a NF1 patient with the clear clinical diagnostic criteria of multiple cafè-au-lait macules, neurofibromas, EN, a positive family history and a novel NF1 germline c.
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