Unusual mode of spread and presentation of meningioma : a case report.

A Goel, A Mehta, S Gupta 
 Department of Neurosurgery, King Edward Memorial Hospital and Seth G. S. Medical College, Parel, Mumbai, 400012, India., India

Correspondence Address:
A Goel
Department of Neurosurgery, King Edward Memorial Hospital and Seth G. S. Medical College, Parel, Mumbai, 400012, India.
India

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::   Introduction

An extremely rare and interesting case of cranial meningioma with an unusual mode of presentation and paraorbital extension is reported. The literature on extracranial meningiomas is briefly reviewed.

::   Case report

A 40 year old man presented with a 2 year history of progressive growth of a soft tissue mass over the right half of the forehead [Figure. 1]. The swelling grew towards the upper eyelid which was completely involved and later bulged inferiorly to cover the lower eyelid. The bulge resulted in complete obscuration of the vision in this eye. When the swelling was smaller, the patient could elevate it and could see underneath it normally. However, for about a month he was unable to perform this manoeuver. The swelling resulted in local pain, which was the principle reason for his seeking medical treatment. The skin over the swelling was hypervascular, and the surface was multinodular. The skin could be pinched off the underlying mass. The swelling was firm, non-tender, non-compressible and fixed to the underlying bone. The superior and lateral orbital margins were not palpable. There was no neurological symptom or deficit. Computerised tomography (CT) scan showed a large enhancing predominantly extracranial lesion located in the right anterior frontotemporal region. The lesion involved the frontal and ethmoid sinuses and wrapped the eyeball from medial, lateral and anterior sides [Figure. 2]. The anterior frontotemporal convexity bone, a large part of the lesser and greater wings of sphenoid bone and lateral and superior walls of the orbit in relationship to the tumour were markedly thickened [Figure. 3]. A relatively thin sheet of tumour was seen on the intracranial aspect of the thickened bone in the region of frontal polar convexity and orbital roof. At surgery a firm, fleshy, infiltrative and highly vascular tumour was completely excised. A large portion of the thickened bone and the involved dura were resected. Reconstruction of the basal dural defect and the frontal convexity bone was carried out with vascularised pedicle pericranial flap obtained from the posterior temporoparietal region and based on temporalis muscle and its fascial layers.[1],[2]





The blood loss during the operation exceeded 2.5 liters. In the post-operative phase, the patient developed disseminated intravascular coagulation disorder and suffered bleeding at multiple sites including the area of surgery. Despite the efforts to replace blood and blood components the situation worsened and the patient died within 8 hours of surgery. Histology of the lesion was of a benign meningioma with large number of round and polyhedral cells arranged in whorls and separated by fibrovascular connective tissue [Figure. 4].

::   Discussion

Dandy[3] reviewed 31 cases of orbital tumours, 24 of which were subjected to surgery. Of these 31 cases, 9 (29.1%) were `dural meningiomas with hyperostosis of the skull', 2 (6.5%) were dural tumours without hyperostosis of the skull, and one (3.2%) was a dural meningioma of the nerve sheath (bilateral). Dandy specified, `for tumours palpable below the supraorbital ridge, the anterior orbital approach is preferred'. This historical reference suggests that, although rare, the phenomenon of extracranial paraorbital spread of skull based meningioma, and the surgical strategy of such lesions has been well documented in the literature. Extracranial meningiomas are rare in comparison to axial lesions. Previous studies have reported the incidence of extracranial-extraaxial meningiomas to range from 1% to 2% of all meningiomas.[4],[5] Hoye et al[6] sub-classified extracranial meningiomas on the basis of the major aetiologies proposed in the development of extracranial meningiomas. The most common variety of extracranial meningiomas is that which arises from intracranial dura and extends extracranially.[7],[8],[9] About forty percent of these grow from the sphenoidal region into the orbit.[4] A large majority of these lesions have associated hyperostosis of bone and present with proptosis and visual loss.[10],[11] Biologically, extracranial meningiomas have been observed to be benign and slow growing.[12] Although there are occasional reports of complete remission following radiation therapy,[13] surgical resection is the treatment of choice. Resection of the involved dura and hyperostosed bone along with the tumour have been recommended by various groups as appropriate surgical strategy.[11]



The origin of the tumour in the presented case was clearly from the dura of frontal convexity as suggested by the marked local hyperostosis and by the tumour involved dura. Even though the outcome was poor, our case had interesting features. The unusual spread of a meningioma in the form of soft tissue, anterior to the eyeball, has not been recorded in literature. The neglect of the growth of the mass by the patient despite the adverse cosmetic consequences was interesting. The tumour enlargement depicted the characteristics of extracranial growth and natural history of the tumour. The exact cause of the fatal coagulopathy could not be identified. The patient did not have any clinical or biochemical evidence of haematological disorder. Haemolytic response to multiple transfusions of banked blood appeared to be the most likely cause. There is no report suggesting higher incidence of postoperative coagulopathy in extracranial meningiomas.

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