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Meningiomas are extraaxial tumors that arise from arachnoid cap cells
and can develop wherever these cells reside. Therefore, meningiomas are
typically attached to dural leaflets with potential extension and erosion into
surrounding tissue, including bone. Meningiomas arising primarily in bone are
rare. In this paper, we present the case of a 46-year-old female patient with a
primary intraosseous meningioma, characterized by extensive hyperostosis of the
calvarium and intracranial and extracranial soft tissue tumor extension.
Keywords:
Meningiomas, Intraosseous meningioma, Extracalvarial meningioma,
Arachnoid cells
INTRODUCTION
Meningiomas are typically benign lesions that arise from arachnoid cap
cells. These lesions are usually found in the intracranial and intraspinal
compartments. Intracranial meningiomas can be found in a variety of locations
including convexity as well as skull base following the dural folds. They can
occasionally be intraventricular as well specifically in the adult population.
Other rare reported locations of meningiomas include primarily intraosseus
meningiomas as well as outside the central nervous system such as lung,
mediastinum, adrenal gland [1]. We present in this paper a case of a primary
giant calvarial meningioma with local extension.
Case Report
The patient is a 46-year-old female who presented with a 15-year history of bi-parietal scalp swelling and a several month history of intermittent localized headaches. Her headaches were sharp in nature and typically in the right parietal region. The patient was reported to have a history of Paget disease. Additionally, she described frequent scalp “infections” which intermittently required drainage procedures.
Given the severity of these symptoms she was referred for an MRI of the brain (Figure 1) by her primary care physician, which revealed significant meningeal thickening and enhancement, marked irregular calvarial thickening, and enhancing soft tissue extending both within the widened diploïc space and extracranially into the subgaleal and subcutaneous scalp soft tissues.
The soft tissue mass extended 3.7 cm extracranially at one point, and 6
cm intracranially with signs of dural infiltration. There was as well concern
for a local occlusion of the middle third of the superior sagittal sinus on MRV
(Figure 2). A CT scan of the head (Figure 3) revealed spiculated reactive new bone that also radiated
perpendicularly from the skull surface toward the scalp. Of interest, there was
preservation of the bony structure along the sagittal suture as well as lack of
focal lytic lesions. On physical examination, the patient was neurologically
intact. She had marked fullness of the
scalp in the bifrontal and parietal regions with scattered areas of superficial
scalp nodularity and thickening. There were several areas with focal tenderness
to pressure.
In the setting of a presumed paget’s disease,
the imaging and clinical findings were concerning for osteosarcoma. Therefore,
the patient was taken for an en bloc biopsy of the right posterior frontal
portion of the lesion (Figure 4, Figure 5) which ultimately revealed a
WHO Grade I syncytial-type meningothelial neoplasm, which had infiltrated the
calvarium extracranially and intracranially. Treatment strategies were
discussed with the patient including surgical resection and adjuvant therapy.
Patient refused any further intervention due to esthetic side effects and was
therefore followed with serial imaging, initially with short follow-ups that
were later spaced out. Patient is currently 7 years out from her biopsy with
stable imaging and no new clinical complaints.
DISCUSSION
The first case of primary extradural
meningioma is thought to be reported in 1730 by Johan Salzmann [2]. The term
“Primary Extradural Meningioma” (PEM) was first coined by Lang et al. as a
designation of all meningiomas that arise away from the subdural space with no
dural attachment [3]. These include locations such as calvarial, mediastinal,
pulmonary, nasal and oral cavity, paranasal sinuses, neck, and abdomen [3-5].
PEM represent about 2% of all meningiomas the majority of which are located in
the cranial vault [6].
Whilst the cell of origin of the meningiomas
is the arachnoid cap cell in the meninges, the origin of PEM is not entirely
clear. Multiple hypotheses have been advanced. Cranial trauma with entrapment
of dural/arachnoid cells in the fracture lines is one such explanation [7].
Along the same lines,
arachnoid cells are thought to become entrapped in the cranial sutures during
the cranial molding as the baby passes through the birth canal [8,9]. However,
only 0.2 to 4% of patient with PEM report a history of trauma [3,7]. Some authors
have hypothesized that these PEM develop from pluripotent mesenchymal cells
(Fibroblasts, schwann cells…) located extradurally [3,10,11]. Another
hypothesis involve faulty arachnoid cell migration during the embryologic
development to extracranial locations [3,12,13].
PEM have a bimodal
distribution with the first peak in the second decade of life and a later peak
between the fifth and the seventh decades [6]. They typically present as a
progressively growing mass with rarely accompanying neurological symptoms. The
clinical manifestations depend on the size and location of the tumor with
varying symptoms such as deafness, painless expanding mass, proptosis [14,15].
In general, these slow growing meningiomas are usually asymptomatic and do not
show neurologic signs unless the lesion extends through the dura to exert mass
effect on intracranial structures. The most common radiologic finding for the
calvarial meningiomas is hyperostosis [9], followed by osteolysis with the
differential diagnosis therefore including
bony metastasis, osteogenic sarcoma, epidermoid cyst, hemangioma, bone
cyst, osteochondroma, multiple myeloma, eosinophilic granuloma, and fibrous
dysplasia. In our case, the chronic history of scalp swelling and head
enlargement led to a presumptive diagnosis of Paget disease before imaging was
performed. It is likely that the episodic “scalp infections” requiring drainage
were in fact areas of extracranial soft tissue tumor necrosis.
Lang et al.
proposed a classification for cranial PEM according to their relative location vis-à-vis
the skull. Purely extracranial tumors without bony attachment were classified
as type I. Type II lesions had purely bony location and type III were primarily
intraossesous tumors with extracalvarial extensions [3]. In 2006 Bassiouni et
al. [12] proposed another classification taking into account the dural
involvement as it has treatment and surgical implications. Type I tumors were
epidural, type II calvarial, type III extracalvarial and type IV mixed with
extension from the dura to the extracalvarial space.
The most common
reported histopathological subtype is the meningothelial meningioma followed by
transitional then psammomatous [6].
Atypical meningiomas constitute 3 to 5.6% of PEM while malignant
meningiomas comprise 3.9 to 8% of all PEM [3,6,7].
The management
strategy for a lesion of this nature is not all that dissimilar to that for
other slow growing tumors. Wide marginal excision can be curative, however,
given the diffuse infiltrative nature of the disease, a meaningful surgical
excision can be difficult to achieve. In case of dural involvement, resection
of the dural area is advised. Other options include radiotherapy, and medical
treatment options such as RU 486 [16,17] and hydroxyurea, [18,19] although some
studies have shown these agents to be of marginal benefit.
CONCLUSION
Although rare, intraosseous meningiomas should be part of the differential diagnoses in patients with a skull-based lesion with associated soft-tissue components, although unlike the case we presented in this paper, the soft tissue component may be relatively minor. Plain x-ray and CT are superior to MRI in visualizing osteoblastic changes in the calvarium, a key finding in making the diagnosis.
- Agrawal V,
Ludwig N, Agrawal A, Bulsara KR (2007) Intraosseous intracranial
meningioma. AJNR Am J Neuroradiol 28: 314-315.
- Kompanje EJ
(2004) A patient with a large intra- and extracranial tumor, most probably
a primary extradural meningioma, described in 1730. J Neurooncol 67:
123-125.
- Lang FF,
Macdonald OK, Fuller GN, DeMonte F (2009) Primary extradural meningiomas:
a report on nine cases and review of the literature from the era of
computerized tomography scanning. J Neurosurg 93: 940-950.
- Kaleem Z,
Fitzpatrick MM, Ritter JH (1997) Primary pulmonary meningioma. Report of a
case and review of the literature. Arch Pathol Lab Med 121: 631-636.
- van der Meij JJ,
Boomars KA, van den Bosch JM, van Boven WJ, de Bruin PC, et al. (2005)
Primary pulmonary malignant meningioma. Ann Thorac Surg 80: 1523-1525.
- Liu Y, Wang H,
Shao H, Wang C (2015) Primary extradural meningiomas in head: a report of
19 cases and review of literature. Int J Clin Exp Pathol 8: 5624-5632.
- Mattox A, Hughes
B, Oleson J, Reardon D, McLendon R, et al. (2011) Treatment
recommendations for primary extradural meningiomas. Cancer 117: 24-38.
- Shaw R, Kissun
D, Boyle M, Triantafyllou A (2004) Primary meningioma of the scalp as a
late complication of skull fracture: case report and literature review.
Int J Oral Maxillofac Surg 33: 509-511.
- Crawford TS,
Kleinschmidt-DeMasters BK, Lillehei KO (1995) Primary intraosseous
meningioma. Case report. J Neurosurg 83: 912-915.
- Tokgoz N, Oner
YA, Kaymaz M, Ucar M, Yilmaz G, et al. (2005) Primary intraosseous
meningioma: CT and MRI appearance. AJNR Am J Neuroradiol 26: 2053-2056.
- Ho KL (1980)
Primary meningioma of the nasal cavity and paranasal sinuses. Cancer 46:
1442-1447.
- Bassiouni H,
Asgari S, Hubschen U, Konig HJ, Stolke D (2006) Dural involvement in
primary extradural meningiomas of the cranial vault. J Neurosurg 105: 51-59.
- Thompson LD,
Gyure KA (2000) Extracranial sinonasal tract meningiomas: a
clinicopathologic study of 30 cases with a review of the literature. Am J
Surg Pathol 24: 640-650.
- Kumar G, Basu S,
Sen P, Kamal SA, Jiskoot PM (2006) Ectopic meningioma: a case report with
a literature review. Eur Arch Otorhinolaryngol 263: 426-429.
- Gunduz K, Kurt
RA, Erden E (2014) Ectopic orbital meningioma: report of two cases and
literature review. Surv Ophthalmol 59: 643-648.
- Grunberg SM,
Weiss MH, Russell CA, et al. (2006) Long-term administration of
mifepristone (RU486): clinical tolerance during extended treatment of
meningioma. Cancer Invest 24: 727-733.
- Lamberts SW,
Tanghe HL, Avezaat CJ, et al. (1992) Mifepristone (RU 486) treatment of
meningiomas. J Neurol Neurosurg Psychiatry 55: 486-490.
- Newton HB, Scott
SR, Volpi C (2004) Hydroxyurea chemotherapy for meningiomas: enlarged
cohort with extended follow-up. Br J Neurosurg 18: 495-499.
- Weston GJ, Martin AJ, Mufti GJ, Strong AJ, Gleeson MJ (2006)
Hydroxyurea treatment of meningiomas: a pilot study. Skull Base 16:
157-160.
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