Massive Facial Neurofibromas: Review of the Literature, New Classification System and Case Presentations

Case Presentation

Ann Surg Perioper Care. 2016; 1(2): 1007.

Massive Facial Neurofibromas: Review of the Literature, New Classification System and Case Presentations

Bogdasarian RN¹, Fientisch AM¹, Langer PD² and Datiashvili RO¹*

¹Department of Surgery, Division of Plastic Surgery, Rutgers State University of New Jersey/New Jersey Medical School, USA

²Department of Ophthalmology and Visual Science, Rutgers State University of New Jersey/New Jersey Medical School, USA

*Corresponding author: Datiashvili RO, Department of Surgery, Division of Plastic Surgery, Rutgers State University of New Jersey/New Jersey Medical School, ACC Building E-1620, NewYark, NJ 07103, USA

Received: October 05, 2016; Accepted: October 21, 2016; Published: October 24, 2016

Abstract

Massive facial neurofibromas are rare, occurring in 1 in 300,000 individuals.

In the presented article, we review the literature and our recent experience with MFNs. We provide an overview of management strategies and develop a classification system and treatment algorithm based on modern treatment options.

In this comprehensive review, Ovid Medline and the Cochrane library were mined for the relevant literature. Articles were extracted from the bibliographies of selected papers. The literature search returned 52 articles including literature reviews, case series and case studies.

We developed a three tiered classification system, based on the anatomical structures affected by the MFN. Our treatment algorithm provides concise guidelines for preoperative planning.

Keywords: Neurofibromatosis; Facial Neurofibroma; Surgery; Treatment; Classification; Algorithm

Abbreviations

MFN: Massive Facial Neurofibroma; NF1: NeuroFibromatosis Type One

Introduction

Massive facial neurofibromas (MFNs) pose formidable challenges to the surgeon: aesthetic, functional, reconstructive, and risk of severe bleeding. We define MFNs as neurofibromas involving greater than 25% of the face.

MFNs are associated with Neurofibromatosis Type I (NF1) or Von Recklinghausen Syndrome, an autosomal dominant disorder of variable penetrance resulting in benign, yet often disfiguring hamARtomas of peripheral nerve sheaths. NF1 affects 1/3000 individuals. Of these individuals, 1% is affected by MFNs, or 1/300,000 of the general population.

MFNs are variable, most often located in the orbitotemporal region, but with a wide range of effects on skin quality and special structures such as the orbit, eye, lids, lips, oral commissure, facial muscles, nerves, skeleton, and so on. All MFNs are highly vascular, amorphous tumors. They are prone to recurrence, have potential for malignant transformation (3.5% in the adult), and are located in cosmetically sensitive areas [1-6]. A consensus, standardized management protocol does not exist. The algorithms that do exist are restricted to specific anatomical regions and lack guidelines for a comprehensive medical and surgical approach. Thus, outcomes may benefit from a comprehensive classification system and treatment algorithm to guide preoperative planning.

MFNs in children may demonstrate rapid growth and malignant transformation (up to 20% in one study), and thus should be biopsied and closely observed [7]. Resection of MFNs in children is typically deferred until the age of 18 or until the MFN stabilizes, unless evidence of malignancy presents. This review focuses on the treatment of adults.

The purpose of this article is to share a review of the literature and our experience with two cases of MFNs. We developed and present a new classification system and treatment algorithm for preoperative planning.

Case I

A 29 year old male with history of NF1 and prior tumor resection of the scalp presented with left sided MFN involving the temporopalpebral region and cheek (Figure 1, Left). Paresis of the frontal branch of the facial nerve and left eye blindness were present. There was a relatively good skin condition overlying the tumor (type Ia, IIb, IIIa; see Table 1). The patient underwent a two-stage reconstruction consisting of primary debulking of the tumor with local skin flap arrangement with subsequent secondary canthopexy and browpexy. The highly vascular nature of the patient’s tumor resulted in moderate blood loss, especially during the primary procedure. Bleeding was controlled with pressure, electrocautery, and clip ligation. Preoperative tumor embolization, banked autologous blood and cross-matched blood ensured the patient’s safety. There were no postoperative complications (Figure 1, Right) [8]. The patient is very satisfied with his outcome.