Giant Mediastinal Neurofibroma in a Child with Neurofibromatosis Type I

Koichiro Uchida, Tadao Okada, Shohei Honda, Hisayuki Miyagi, Ryouji Kobayashi, Akihiro Iguchi, Kanako C. Kubota, Taketomi Akinobu

Department of Gastroenterological Surgery I, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
Department of Surgical Pathology, Hokkaido University Hospital, Sapporo, Japan.

DOI: 10.4236/ss.2012.312111                

Abstract

Aim: There are a variety of malignant tumors related to neurofibromatosis type 1 (NF1). This report describes a rare pediatric NF1 case with an unresectable giant mediastinal tumor. Case: A 6-year-old girl with wheezing was admitted to our institution for the further evaluation of a right mediastinal mass on plain chest radiography. On examination, there were multiple café au lait spots mainly on the trunk, and a well-defined, immobile, painless mass was palpable on her neck. The mediastinal lesion was detected as nonuniform mass surrounding the aortic arch, pulmonary artery, and right main bronchus on the contrast-enhanced CT and MRI. Open biopsy was useful to rule out malignancy and revealed neurofibroma, and contributed to follow up and treatment. Discussion: Open biopsy was useful to rule out malignancy, such as malignant peripheral nerve sheath tumor, revealed neurofibroma, and also contributed to follow up and treatment. The authors report successful management by open biopsy and discuss several clinical points regarding mediastinal neurofibroma for NF1.

Keywords

Mediastinal Tumor; Neurofibromatosis Type I; Neurofibroma, Open Biopsy

Conflicts of Interest

The authors declare no conflicts of interest.

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