Giant Neurofibroma of the Trunk: Surgical Approach and Pathology
Iris A. Seitz, MD, PhD, David H. Song, MD; Loren S. Schechter, MD; Madelyn Kahana,
MD; McKay McKinnon, MD
INTRODUCTION: Neurofibromatosis is an autosomal-dominant systemic disease, first
described by Von Recklinghausen in 1882(1) and can present with abnormalities of the
skin, nervous system, soft tissue, blood vessels and bones, ranging from small skin
nodules to deforming giant tumors (2). Surgical excision of neurofibromas (NF) is
recommended for tissue diagnosis, to treat rapidly growing, large, symptomatic
lesions and to reduce risk of malignant transformation. Due to increased risk of
uncontrollable hemorrhage from large vessels within the tumor, some giant NF are
considered unresectable. Despite the well-known systemic vasculopathy in
neurofibromatosis (3), little attention has been paid to the structural changes of
the vasculature in neurofibromas themselves (4, 5). The purpose of this study is to
examine the surgical treatment of two giant NF that far exceed any previously
reported in the literature.
METHODS: Two case reports of the surgical evaluation and treatment of giant
Neurofibroma (>75kg) are presented and prior reports of large NF resection were
analyzed. Preoperative evaluations included history and physical examination, CT,
MRI, angiography and cardiac echography. Surgical technique as well as
histopathology and long-term follow-up were reviewed.
RESULTS: Both patients survived resection of unprecedented tumors without
complication at two and five year respective follow-up. Surgical strategy to
control hemorrhage from the tumor included resection from normal periphery to
central tumor in a deep fascial plane and controlled isolation and ligation of large
tumor vessels. Aggressive intraoperative fluid resuscitation using blood products
was essential. A description of the vascular histopathology of NF is presented
which may explain bleeding propensity in NF.
Age
(years)
Gender
Tumor
location
Pathology of
specimen
Patient
1
40
Female
Trunk
Patient
2
46
Female
Trunk
Neurofibroma,
multiple
large tumor
vessels with
intimal
thickening,
thinning of
media
Neurofibroma,
large tumor
vessels
Tumor
weight
(kg)
90 kg
Time in OR
(hrs)
77 kg
10 h
16 h
Blood
products
(Units)
46 U PRBC,
46 U FFP,
6-packsPlatelets
x5
25 U PRBC,
25 U FFP
Table 1: Two patients with giant NF
CONCLUSIONS: We report for the first time the successful
sized tumors of Neurofibromatosis, the surgical strategy
resection of similar tumors, and a previously unreported
characteristic of large NF. These findings may stimulate
the growth and surgery of "unresectable" tumors.
resection of two giant
developed for safe
vascular pathology
further investigation into
Figure 1: Patient 1 with giant NF of the trunk
Figure 2. Patient 2 with giant NF of the trunk
Figure 3: Macroscopic (left upper image) and microscopic (right upper image)
appearance of giant NF with large tumor vessels (left lower image) and in higher
magnification a large tumor vessel with intraluminal thrombus, thickening of intima,
thinned out media and fragments of elastica (right lower image).
References:
1. Von Recklinghausen F.D. (1882) Über die multiplen Fibrome der Haut und ihrer
Beziehung zu den multiple Neuromen. Berlin.
2. Riccardi VM. Neurofibromatosis: clinical heterogeneity. Current Problems in
Cancer 1982; 7:1-34.
3. Salyer WR and Salyer DC. The vascular lesions of neurofibromatosis. Angiology
1974; 25: 510-519
4. White N, Gwanmesia I, Akhtar N, Withey SJ. Severe hemorrhage in neurofibroma: a
lesson. Br J Plast Surg. 2004; 57:456-7.
5. Francis DM, Mackie W. Life-threatening haemorrhage in patients with
neurofibromatosis.
Aust N Z J Surg. 1987; 57:679-82.