Kankane et al., J Spine Neurosurg 2015, 4:2
http://dx.doi.org/10.4172/2325-9701.1000186
Journal of Spine &
Neurosurgery
Research Article
A SCITECHNOL JOURNAL
Spinal Neurofibroma in Pediatric
Patient Presenting as Trophic
Ulcer on Sole: A Rare Case with
Rarest Presentation
Vivek Kankane *, Tarun Gupta and Gaurav Jaiswal
occur both sporadically and in association with neurofibromatosis
1(NF1: von Recklinghausen's disease) [3]. Nerve sheath tumor
accounts for 40% of all intradural spinal cord tumors in adult [4]. In
spine, there is a male preponderance and male to female ratio is 1.25 to
1.5:1, in contrast to intracranial and peripheral sites where there is a
female predominance (1.5:1). The peak incidence of these tumors is in
the fourth to sixth decade. Most are solitary schwannoma and occur
proportionally throughout the spine.
Department of Neurosurgery, M.B. Hospital, R.N.T. Medical College Udaipur,
Rajasthan, India
Materials and Methods
*Corresponding author: Kankane Vivek, Department of Neurosurgery, M.B.
Hospital, R.N.T. Medical College Udaipur, Rajasthan, India, Tel: 0294-2528811 Email: [email protected]
Rec date: Jan 30, 2015, Acc date: Mar 11, 2015, Pub date: Mar 16, 2015
Abstract
Nerve sheath tumor is uncommon tumor in the general
population with an annual incidence of 0.3–0.4 per 100,000
person. Peak incidence of these tumors is in 4th to 6th decade.
They occur both sporadically and in association with
neurofibromatosis 1 (NF1; von Recklinghausen's disease).
Nerve sheath tumor account for 40% of all intradural spinal
cord tumors in adult. In spine, there is a male preponderance
and male to female ratio is 1.25 to 1.5:1. We reported a rare
case report; a twelve years old male child was referred to our
department. Patient presented with trophic ulcer on left sole of
10 months duration followed by backache and features of
conus syndrome of 8 months and 4 months duration
respectively. Referring physician applied below knee cast and
referred patient to our department for further management.
CEMRI of lower dorsal and lumbosacral spine revealed
intradural
extramedullary
tumor
with
homogenous
enhancement at D12 -L1 level with extradural extension. D12,
L1, L2 laminectomy with near total excision of tumor was done.
Tumor was well-circumscribed, lobulated, grayish blue, soft,
CUSA suckable, with moderate vascularity. Tumor
histopathology
was
suggestive
of
neurofibroma.
Immunohistochemistry and Molecular testing are not available
in our setup Patient bowel-bladder function improved
significantly in immediate post-operative period. Patient
became fully continent with significant healing of trophic ulcer
after follow-up of 6 month. Majority of spinal neurofibromas are
intradural extramedullary tumors and presents with radicular
pain and ascending weakness and peak incidence of these
tumor is in 4th to 6th decade and rare in pediatric group. In this
case spinal neurofibroma was associated with trophic ulcer in
pediatric patient which is a rare case with rarest presentation,
probably first case reported in world’s literature.
A twelve years old male child was referred to our department.
Patient presented with trophic ulcer on left sole (Figure 1) of 10
months duration followed by backache and features of conus
syndrome of 8 months and 4 months duration respectively. Trophic
ulcer not associated with spinal dysmorphisim, trauma, leprosy,
diabetes, neuropathy, peripheral vascular disease. Referring physician
applied below knee cast and referred patient to our department for
further management. CEMRI of lower dorsal and lumbosacral spine
revealed intradural extramedullary tumor with homogenous
enhancement at D12 -L1 level with extradural extension (Figures 2 &
3). D12, L1, L2 laminectomy with near total excision of tumor was
done. Tumor was well-circumscribed, lobulated, grayish blue, soft,
CUSA suckable, with moderate vascularity. Tumor histopathology was
suggestive of neurofibroma, histopathological examination shows
hyperplasia of interfascicular connective tissue and matrix is riched in
proteoglycans and there is numerous tightly packed collagen and
reticular fibers and predominant cells are elongated with elliptical
nuclei suggestive of neurofibroma (Figure 4). There are numerous area
of myxoid degeneration in the proliferation of connective tissue and
hyperplasia of vascular stroma. Immunohistochemistry and Molecular
testing are not available in our setup. Patient bowel-bladder function
improved significantly in immediate post-operative period. Patient
became fully continent with significant healing of trophic ulcer after
follow-up of 6 month.
Keywords: Spinal neurofibroma; Pediatric; Trophic ulcer
Introduction
Nerve sheath tumors are uncommon tumor in the general
population with an annual incidence of 0.3–0.4 per 100,000 person
[1,2]. Peak incidences of these tumors are in 4th to 6th decade. They
Figure 1: Photograph of trophic ulcer on sole
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Citation:
Kankane V, Gupta T, Jaiswal G (2015) Spinal Neurofibroma in Pediatric Patient Presenting as Trophic Ulcer on Sole: A Rare Case with Rarest
Presentation. J Spine Neurosurg 4:2.
doi:http://dx.doi.org/10.4172/2325-9701.1000186
Figure 2: Magnetic resonance sagittal pre & post contrast T1W images, of lower dorsal and lumbosacral spine revealed intradural
extramedullary tumor with homogenous inhancment at D12 -L1 level with extradural extension
Volume 4 • Issue 2 • 1000186
• Page 2 of 5 •
Citation:
Kankane V, Gupta T, Jaiswal G (2015) Spinal Neurofibroma in Pediatric Patient Presenting as Trophic Ulcer on Sole: A Rare Case with Rarest
Presentation. J Spine Neurosurg 4:2.
doi:http://dx.doi.org/10.4172/2325-9701.1000186
Figure 3: Magnetic resonance axial pre & post contrast T1W images of lower dorsal and lumbosacral spine revealed large lobulated soft tissue
intensity mass lesion with homogenous inhancment in the intradural as well as extradural at D12- L1 level & passing through the right neural
foramen & paravertebral region suggestive of nerve sheath tumour
Volume 4 • Issue 2 • 1000186
• Page 3 of 5 •
Citation:
Kankane V, Gupta T, Jaiswal G (2015) Spinal Neurofibroma in Pediatric Patient Presenting as Trophic Ulcer on Sole: A Rare Case with Rarest
Presentation. J Spine Neurosurg 4:2.
doi:http://dx.doi.org/10.4172/2325-9701.1000186
intraoperative diagnosis of schwannoma enabled us to carry out a total
excision of the tumor, which resulted in near complete recovery at 10
months follow-up. Although rare, this diagnosis should be considered
when a child presents with a solitary intramedullary tumor since its
total resection can be achieved improving surgical outcome [6].
Ranjan R, et al. reported Spinal intradural extramedullary
ependymal cyst is a very rare entity with only few cases reported in the
literature. Its association with congenital dermal sinus has not been
described so far. We present a unique report of a 3-year-old male child
who presented with spastic quadriparesis with a in the right great toe
of 1-year duration. He harbored a congenital dermal sinus in the
cervical spine since birth. Intraoperatively, the sinus was associated
with an intradural cyst which proved to be an ependymal cyst on
histopathological examination. The clinical profile along with review
of literature of this rare entity is presented [7].
Figure 4: Histology section showing hyperplasia of interfascicular
connective tissue and matrix and there is numerous tightly packed
collagen and reticular fibers and predominant cells are elongated
with elliptical nuclei suggestive of neurofibroma. There are
numerous area of myxoid degeneration ( H and E )
Discussion
Trophic ulcer is more common in intramedullary tumor and spinal
dysraphism. In world literature no single case found of extramedullary
intradural tumor presented as trophic ulcer.
With nerve sheath tumors, two tumor populations need to be
distinguished which are schwannoma and neurofibroma.
Schwannomas are more common and are the largest category of nerve
sheath tumors. Whereas Schwannomas are encountered in patient
with neurofibromatosis (NF-2) and in patient without NF,
neurofibromas are found in patients with NF-1 [1]. Most nerve sheath
tumors arise from a dorsal nerve root. Neurofibromas represent a
higher proportion of ventral root tumors and often exhibit a dumbbell configuration [2].
Liang WU reported a case of a Spinal intradural malignant
peripheral nerve sheath tumors (MPNSTs) in children are extremely
rare, with only five reported cases in the literature. A 9-year-old female
with neurofibromatosis type 2 (NF-2) has presented with right hip
pain and severe weakness of bilateral legs for 3 months. Magnetic
resonance (MR) imaging revealed multiple intradural masses at the
T11-L2, L4, and L5-S5 level respectively, and bilateral vestibular
schwannomas in the cerebellopontine angle. Partial tumor excision
with T11-L2 laminectomy was undertaken and the tumors in the
spinal cord were consistent with the diagnosis of epithelioid MPNSTs.
No adjuvant therapy was performed after surgery. No metastasis of the
tumor was found in the 6-month follow-up MR imaging. She died of
brain metastasis at 9 months after surgery. MPNSTs should be added
to the differential diagnosis of intradural tumors of the pediatric spine,
even in children with NF-2. Multidisciplinary treatment consisting of
total surgical removal and adjuvant radiotherapy should be considered
due to poor prognosis of this abnormality [5].
Eljebbouri B, et al. reported Pediatric intramedullary schwannoma
without neurofibromatosis is extremely rare with only five cases
reported so far. He presents this rare finding in a 10-year-old boy who
presented with a sudden onset of weakness in the lower limbs. An
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Diem E, et al. reported a case in a female patient which had been
operated shortly after birth because of lumbar myelomeningocele,
suddenly trophic ulcer of the toes of her right foot occurred. Rapid
healing was achieved after laminectomy and successful extirpation of
an epidural lipoma and a fibrous band which compressed the cauda
equina at the level L4 [17]
Koranne V, ulcers over both feet in a 24 years old male were initially
diagnosed as of leprous etiology. However detailed investigations
revealed spina bifida and lipomeningocoele over sacral region.
Importance of thorough neurological investigations in such cases is
stressed to avoid mis-diagnosis of leprosy [18].
All reported cases; no single case was found of extramedullary
intradural tumor, which was presenting as trophic ulcer in pediatric
age group.
Conclusion
Majority of spinal neurofibromas are intradural extramedullary
tumors and presents with radicular pain and ascending weakness and
peak incidence of these tumor is in 4th to 6th decade and rare in
pediatric group. In this case spinal neurofibroma was associated with
trophic ulcer in pediatric patient which is a rare case with rarest
presentation, probably first case reported in world’s literature.
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Citation:
Kankane V, Gupta T, Jaiswal G (2015) Spinal Neurofibroma in Pediatric Patient Presenting as Trophic Ulcer on Sole: A Rare Case with Rarest
Presentation. J Spine Neurosurg 4:2.
doi:http://dx.doi.org/10.4172/2325-9701.1000186
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