Malignant Peripheral Nerve Sheath Tumor of the Inguinum and Angiosarcoma of the Scalp in a Child with Neurofibromatosis Type 1.

Marija Milković Periša,Jasminka Stepan Giljević,Božo Krušlin,Tihana Džombeta

doi:10.1155/2017/7542825

Abstract

Benign and malignant tumors are common in the setting of neurofibromatosis type 1 (NF1). Malignant peripheral nerve sheath tumor (MPNST) and angiosarcoma are rare tumors in children and adolescents and mostly occur in young patients in relation to NF1. Both histological types can be present in the same tumor mass in patients with NF1. We present a case of 12.5-year-old girl with NF1 who first presented with MPNST of the right inguinal region and 1.5 years later with unrelated angiosarcoma of the scalp.

Highlights

Neurofibromatosis type 1 (NF1) is a common autosomal dominant disorder with the incidence rate of 1 in every 3500 individuals [1]
Benign and malignant tumors are common in the setting of neurofibromatosis type 1 (NF1)
We present a case of 12.5-year-old girl with NF1 who first presented with Malignant peripheral nerve sheath tumor (MPNST) of the right inguinal region and 1.5 years later with unrelated angiosarcoma of the scalp

Summary

Introduction

Neurofibromatosis type 1 (NF1) is a common autosomal dominant disorder with the incidence rate of 1 in every 3500 individuals [1]. The main characteristics of this disorder are “cafe-au-lait” spots, cutaneous neurofibromas, and hamartomas of the iris (Lisch nodules) [1]. Malignant peripheral nerve sheath tumors and angiosarcomas are uncommon in children and adolescents, even those suffering from NF1. Some authors described these two entities within the same tumor mass, since heterologous differentiation in the form of angiosarcoma may occur in MPNST, more commonly in patients with NF1 [3,4,5]. We report a case of MPNST of the inguinal region and unrelated angiosarcoma of the scalp in a girl with NF1

Case Report

Findings

Discussion