Cutaneous meningiomas are extremely rare tumours and their diagnosis is difficult. We describe the case of a patient who developed a paranasal swelling after head trauma and associated fractures in the same region years before. Histopathological examination of an incisional biopsy revealed the diagnosis of cutaneous meningioma. After one and a half years' follow up, surgical excision was performed because of progressive growth of the tumour and associated aesthetic problems. Extracranial meningiomas can develop probably secondary to trapping of meningeal tissue after trauma. If there is no intracranial connection surgical removal can be considered

Borggreven, P.A.

Graaf, F.H. de

Leemans, C.R.

Valk, P. van der

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The Journal of Laryngology & Otology
March 2004, Vol. 118, pp. 228–230
Clinical Records
Post-traumatic cutaneous meningioma
Pepijn A. Borggreven, M.D., Floris H. de Graaf, M.D.*, Paul van der Valk, M.D., Ph.D.†,
C. Rene´ Leemans, M.D., Ph.D.
Abstract
Cutaneous meningiomas are extremely rare tumours and their diagnosis is difcult. We describe the case of a
patient who developed a paranasal swelling after head trauma and associated fractures in the same region years
before. Histopathological examination of an incisional biopsy revealed the diagnosis of cutaneous meningioma.
After one and a half years’ follow up, surgical excision was performed because of progressive growth of the
tumour and associated aesthetic problems. Extracranial meningiomas can develop probably secondary to
trapping of meningeal tissue after trauma. If there is no intracranial connection surgical removal can be
considered.
Key words: Meningioma; Face; Orbital Fractures
Introduction
Meningiomas are usually intracranial neoplasms. Extra-
cranial meningiomas are uncommon and only a few cases
have been sporadically reported in the literature.1–10 We
describe a case of cutaneous meningioma probably due to
post-traumatic trapping and discuss the histopathological
ndings, differential diagnosis and management.
Case report
A 68-year-old female presented to the otolaryngology
service with a progressive paranasal swelling on the right
side, which had existed for 14 years. The tumour began
following a right orbital fracture extending into a skull base
fracture complicated by an epidural haematoma, which
was evacuated by operation (Figure 1). Notably, ve years
earlier she had consulted a general surgeon who partially
excised the tumour. Histopathology did not show a
conclusive diagnosis, but there was no evidence of
malignancy. Because of continuing slow growth of the
tumour after that operation and fear of a malignancy, she
sought further medical advice.
Physical examination revealed a subcutaneous, rm
paranasal tumour just beneath the right lower eyelid
(Figure 2). Nasal examination was normal. Neurological
examination showed no cranial nerve palsies nor specic
ophthalmological abnormalities. There was no evidence of
cervical adenopathy on palpation.
Magnetic resonance imaging (MRI) and a computed
tomographic (CT) scan showed a sharply delineated
paranasal mass on the right with some calcication but
without any evidence of intracranial connection or bony
defect (Figure 3). Fine needle aspiration did not yield a
conclusive diagnosis. Therefore an incisional biopsy was
performed. The histopathological ndings are shown in
Figure 4. Histologically the lesion showed a pattern
consisting of whorls of round to oval-shaped meningothe-
lial cells mixed with spindle-shaped cells and variable
From the Departments of Otolaryngology – Head and Neck Surgery, Plastic Surgery*, and Pathology†, VU Medical Centre,
Amsterdam, The Netherlands.
Accepted for publication: 16 December 2003.
Fig. 1
CT scan. Non-contrast-enhanced axial CT image. Skull base
fracture line (arrow).
228
numbers of psammoma bodies. It was diagnosed as a
cutaneous meningioma. Initial conservative therapy was
suggested and preferred by our patient. After one and a
half years of follow up surgical excision was performed
because of growth of the tumour and related aesthetic
problems.
Discussion
Extracranial meningiomas are rare tumours. Of all
meningiomas an incidence of one per cent for extracranial
meningiomas, excluding spinal and orbital tumours, has
been reported.1 ,2 Cutaneous meningiomas are the most
common clinical manifestations of extracranial meningio-
mas in the head and the neck.3 They are derived from
meningo-epithelial cells, and may be encountered in skin
and soft tissue beyond the connes of the central nervous
system.4 Based on several clinical-pathological reports
over the last decades a classication into four groups can
be made:
(1) Cutaneous meningiomas, which are derived from
precursors of meningo-epithelial cells. During embryogen-
esis, these ectopic arachnoid cells are very likely to be
displaced into the dermis and are primarily located in the
subcutaneous tissue of the scalp or forehead but also in
skin along suture lines and paravertebral areas.4 ,5 Often
they are represented in epidermal cysts, neoplasms or
areas of alopecia areata. Frequently an occult connection
with the central nervous system exists that normally
involutes but, if it persists, leakage of spinal uid can
occur especially at the time of surgical exploration. This
group of meningiomas is dened as primary cutaneous
meningiomas.
(2) Cutaneous meningiomas, which develop out of
ectopic cells distributed around sensory organs especially
localized along cranial and spinal nerves. Sites of predilec-
tion are the orbital, nasal and, in some cases, auricular
region. In contrast to primary cutaneous meningiomas
these lesions are acquired and manifest principally at any
age.5
Fig. 2
Lateral photograph of the patient. Paranasal swelling on the
right side (arrow).
Fig. 3
MRI scan. T1-weighted axial MRI image with a 2-cm-
diameter subcutaneous paranasal mass (arrow) without bone
defects.
Fig. 4
Histological characteristics. Pattern consisting of whorls of
round to oval shaped meningothelial cells mixed with spindle-
shaped cells and a psammoma body (arrow) (H&E; 3 25).
clinical records 229
(3) Intracranial meningiomas that spread through the
foramina or extend through post-traumatic lesions and old
operative defects into adjacent subcutaneous and skin
areas.6 Typically, these lesions are often accompanied by
intracranial tumours shown on supplementary imaging. In
addition, extension of extracranial meningial tissue by
post-traumatic trapping secondary to disruption of the
craniofacial bony structure has been postulated
sporadically.7
(4) Finally dissemination in blood or lymphatic tissue by
intracranial tumours with sarcomatous, haemangiopericy-
tomatous or papillary characteristics has been reported.
This group of cutaneous meningiomas is also known as
anaplastic (malignant) meningiomas.8
Histopathology and classication of meningiomas are
difcult and complex, because of their different ways of
manifestation. The most common form is the meningothe-
lial type, with spindle cells, whorls of cells and sheets and
nests of polygonal cells as distinguishing features. The
whorl cells often show hyaline or calcication centres
known as typical psammoma bodies, which are very
helpful in the diagnosis. Less frequent are the broblastic,
transitional, anaplastic and papillary type of which the
latter two are clinically malignant. Many other variants
have been described, but most are of interest only to the
pathologist. Histological diagnostic support can be made
by a positive immunohistochemical staining with vimentin,
cytokeratin and epithelial membrane antigen (EMA).9
Differential diagnoses for heterotopic skin tumours,
include meningoceles, meningomyeloceles and menin-
goencephaloceles, nasal gliomas, neuroectodermal
tumours, metastatic neuroblastomas and ganglioneuromas.
Clinical features and essential CT and/or MRI are
supporting but often not conclusive. Cytology or ne
needle aspiration biopsy can be decisive but usually an
incisional biopsy is necessary for distinction between these
differential diagnostic options.10
If there is no indication of intracranial or intravertebral
attachment the primary and neurocranial ectopic cell
meningiomas can be treated in two ways; primarily
conservatively, principally if there are no complaints nor
side effects, or secondly, by radical excision depending on
their localization, especially when there is neighbouring
tissue compression or aesthetic problems. Expanding
intracranial tumours accompanied by cutaneous meningio-
mas as well as metastatic lesions can be treated much less
effectively than the aforementioned meningiomas, mainly
because of advanced stage at the time of presentation, or
the underlying cerebral mass. If detected early, excision
and, if necessary, additional treatment can be performed,
but unfortunately palliative treatment is the only possible
option in the majority of cases.
Cutaneous meningiomas of the head and neck are rare
and difcult to identify. Our case can be classied as a
post-traumatic entrapment meningioma (group 3). Notably
the tumour can develop at any age and will often be
presented to the surgeon after it has been present for a
long time. A detailed history, especially for past head and
neck trauma can be helpful in determining the nal
diagnosis. As long as there is no evidence of intracranial
connection conservative therapy is an obvious option
before surgical intervention is considered.
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3 Lopez DA, Silvers DN, Helwig EB. Cutaneous meningio-
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4 Sibley DA, Cooper PH. Rudimentary meningocele: a
variant of primary cutaneous meningioma. J Cutaneous
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5 Ragoowansi R, Thomas V, Powell BWEM. Cutaneous
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Address for correspondence:
Pepijn A. Borggreven, M.D.,
Department of Otolaryngology – Head and Neck Surgery,
VU Medical Centre,
P.O. Box 7057,
1007 MB, Amsterdam, The Netherlands.
Fax: +31 20 4443688
E-mail: pa.borggreven@vumc.nl
P. A. Borggreven takes responsibility for the integrity of the
content of the paper.
Competing interests: None declared
x Cutaneous extracranial meningiomas are rare
x In this case report a lesion in the paranasal skin of a
patient who had previously suffered an orbital and
anterior skull base fracture was found to be a
meningioma
x It is postulated that this was the result of meningeal
tissue being sequestrated at the time of injury
230 p. a. borggreven, f. h. de graaf, van der valk, c. r. leemans


Post-traumatic cutaneous meningioma.

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Post-traumatic cutaneous meningioma.

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