Chondroma of the Falx Cerebri in an Older Woman

Pierpaolo Lunardi, MD
Carlo Conti, MD
Paolo Giovenali, MD
Rodolfo Corinaldesi, MD
Volume 18 - Number 10 - November/December, 2010

Introduction

Intracranial chondromas are rare, benign tumors, representing approximately 0.5% of all primary brain tumors. They usually originate from the base of the skull, with a predilection for the sphenoethmoidal recess.1-18 Less commonly, intracranial chondromas may arise as lesions occupying the intraparenchymal or intraventricular spaces.1-19

We present an unusual case of an intracranial chondroma originating from the falx cerebri in a 79-year-old patient. To our knowledge, this is the oldest patient with a chondroma of this origin described in the literature.

Case Presentation

A 79-year-old woman came to the neurosurgical department after she was diagnosed with a lesion occupying the parasagittal, left parietal space. The patient reported headache and dizziness. She was being treated with hydrochlorothiazide for hypertension. Findings from the physical examination were normal and revealed nothing significant, while the neurological examination showed apraxia and a sensitive hypoesthesia in the right hemisoma (sensory inattention, minimal pain and temperature loss, discriminatory sensory deficit), without motion deficit. A magnetic resonance imaging (MRI) scan of the brain with and without the administration of contrast medium showed a lesion in the parietal and left parasagittal area with a maximum diameter of approximately 26 mm. The lesion was characterized by a slightly inhomogenous signal, and was hypointense on T1-weighted images; normally, the lesion is isointense to the brain parenchyma, but in this case the lesion was hypointense because it was present a nucleus of calcification (Figure 1a). The lesion was hyperintense on T2-weighted images (Figure 1b); it was not associated with reactive edema. After the administration of gadolinium, the lesion was characterized by a late enhancement and was inhomogeneous in appearance (Figure 2). The angiographic venous sequence showed no alteration of flow.

The patient underwent surgical removal of the lesion. The tumor was well demarcated, of hard consistency, was almost calcified, and was adhering to the falx cerebri, but it did not invade the superior sagittal sinus. The patient’s postoperative course was normal. Microscopic examination of fragments of the lesion revealed a lobular architecture composed of cartilaginous tissue, mainly of hyaline type. It did not reveal any nuclear polymorphism, atypia, or signs of mitotic activity (Figure 3). The tumor was positive for vimentin and for S-100 protein, and was negative for the epithelial membrane antigen.

A postoperative MRI scan performed 1 year later documented the complete removal of the tumor and revealed no evidence of recurrence.

Discussion

The first case of an intracranial chondroma was reported by Hirschfield in 1851,19 with the first surgical resection performed successfully by Nixon in 1982.20 Intracranial chondromas are benign and slow-growing. It is estimated that their incidence is less than 1% of all intracranial tumors. They are usually found in the skull base, with a predilection for the sphenoethmoidal recess.1-18 It is thought that the development of these tumors from the residual embryonic cartilage of the skull base may explain the predilection for this location; however, there have been reported cases of intracranial chondromas originating from heterotypic chondrocytes or anaplastic fibroblasts of the dura mater of the convexity, falx, tentorium, choroid plexus, brain parenchyma, or the ventricular ependyma without an apparent union with the dura.1-19 Intracranial chondromas may be solitary lesions or result from Ollier disease (enchondromatosis with multiple enchondromas) and Maffucci syndrome (enchondromatosis associated with hemangiomas of soft tissue).3,18,20,21 A summary of the clinical features of falx chondroma cases described in the literature is provided in the Table, where it appears that in most reported cases, the tumor involved the anterior third of the falx cerebri and the affected patients are young or adult.1,3-5,7,9-12,14-18,22

Intracranial chondromas usually occur in young adults and have a peak incidence in the third decade of life. The range of patient ages reported in the literature is between 15 months and 60 years; there is no clear dominance of sex.1,6,17,23-25 Many theories have been formulated to provide a reasonable explanation of the pathogenetic mechanism for the genesis of intracranial chondromas. Several authors agree that most chondromas of the skull base develop from cartilage of the skull base or from residues of primordial cartilage of the skull.2,6,8,13,15,21,24 The etiology of injuries that result from the dura mater and choroid plexus without connections to the skull base is even more controversial. Some hypotheses consider the development of cancer as a secondary meningeal metaplasia of fibroblasts or perivascular mesenchymal tissue arising from residues of ectopic cartilage from embryonic stem cells, or from the connective tissue of meningeal stroma, which, following a traumatic or inflammatory insult, leads to the formation of cartilaginous elements for the activation of fibroblasts.8,13,15,18 The clinical manifestations of falx chondromas are nonspecific, and their symptomatology is associated with their anatomical location. Their slow-growing biology often makes them clinically silent for an extended period of time, so these tumors can reach significant dimensions before a correct diagnosis.16,18

In most cases, patients with falx chondromas manifest signs of intracranial hypertension or neurological deficits,18 or the symptoms may begin with seizures.1,9,16,19 Another distinctive feature is the absence of invasion or destruction of the surrounding brain parenchyma.18 The images of a falx chondroma or convexity are totally nonspecific. In computed tomography scans, they appear as well-demarcated tumors, are isodense, and 60% to 90% of cases have calcifications.3,19,26,27 They may be associated with hyperostosis and deformity of the skull adjacent to the lesion.3,4,8,28 The calcifications may be represented as small nodules or irregular opacities, or they may have the characteristic ring pattern.19 Generally, falx chondromas appear in MRI scans as isointense lesions in T1-weighted images, while T2-weighted images have a heterogeneous hyperintense signal. After the administration of gadolinium, there is a slight thin enhancement, similar to a perilesional ring.4,8,19,26,28 The angiographic studies always show the absolute avascularity of the lesion.1,2,4,9,14,15,19,26 Falx chondromas usually do not show perilesional edema, but, in cases where the lesion is large, this may be present to a minor degree.1,6,8,15,16,19,28,29 The differential diagnoses include meningiomas, oligodendroglioma, hemangioblastoma, chondrosarcoma, or a fungal infection.

The characteristics that occasionally enable the clinician to differentiate a chondroma from a meningioma are usually the lack of a homogeneous enhancement; the absence of the “dural tail” sign, which is a thickening of the dura mater that results in the appearance of a tail extending from the mass; and total avascularity of the lesion.30 However, the only method for a diagnosis of certainty is the histological examination. The complete removal of the lesion is the most appropriate treatment for falx chondroma. While the long-term prognosis is favorable, there have been cases of relapse even after a complete removal.2,7,10-12,14,15,17,22,27,31 Radiotherapy is currently not recommended for tumor residues or for patients with inoperable lesions; the chondroma does not respond to radiation therapy and there is concern that this could induce malignant degeneration.1,6,15

Conclusion

Many theories have been formulated to provide a reasonable explanation of the pathogenetic mechanism for the genesis of intracranial chondromas. The complete removal of the lesion is the most appropriate treatment for falx chondroma. A review of the literature of intracranial chondromas originating from the falx cerebri shows that the patient described in this report is the oldest individual in whom an intracranial chondroma originating from the falx cerebri has been reported.

The authors report no relevant financial relationships.

Drs. Lunardi and Corinaldesi are from the Department of Neurosurgery, University of Perugia, Perugia, Italy; Dr. Conti is from the Department of Neurosurgery, Terni General Hospital, Terni, Italy; and Dr. Giovenali is from S.S.D. Diagnostic Cytology and Histology, Perugia General Hospital, Perugia, Italy.

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Figure Legends

Figure 1. Preoperative sagittal T1-weighted magnetic resonance image of the brain showing a hypointense lesion with nucleus of calcification in the left parasagittal area (arrows; a). T2-weighted axial cut showing the light hyperintense lesion and no peritumoral edema (b).

Figure 2. Preoperative, contrast-enhanced, sagittal T1-weighted magnetic resonance image of the brain showing a late enhancement and uneven appearing lesion.

Figure 3. Histopathology images of the tumor showing mature cartilage tissue, with chondrocytes that have a slightly irregular disposition and are immersed in discrete quantities of hyaline matrix (hematoxylin and eosin staining, magnification x100). Weak cartilage contains only one chondrocyte without evidence of atypical cytology.