Rare extracranial localization of primary intracranial neoplasm
© Arndt et al; licensee BioMed Central Ltd. 2008
Received: 06 March 2008
Accepted: 16 April 2008
Published: 16 April 2008
Meningioma, craniopharyngeoma and glioma are mainly intracranial lesions. Nevertheless, in rare cases these entities may occur solely as extracranial lesions that may present as intranasal/sinusoidal masses, with headaches and nasal obstruction. We present three cases of common intracranial tumors, with purely extracranial extension. The three described cases demonstrate, that preoperative MRI and CT imaging is important for differential diagnosis to exclude intracranial connections of the tumors. A definitive diagnosis requires specialized immunohistochemical examinations. In all cases of intranasal or pharyngeal neoplasm the diagnosis of meningioma, craniopharyngeoma and glioma should be considered as differential diagnosis to optimize the surgical procedure.
Meningiomas, craniopharyngiomas and gliomas are mainly intracranial lesions. Nevertheless, in rare cases these entities may occur solely as extracranial lesions that may present as intranasal or sinusoidal masses, with vision disturbances, headaches and nasal obstruction. We present three cases of common intracranial tumors, with purely extracranial extension. In all patients, the tumor became manifest in symptoms resembling a primary extradural entity, like chronic sinusitis, chordoma or nasal polyposis. The true diagnosis is based mainly on histopathological examination.
1. Case report
2. Case report
A 16-year-old girl presented to our department with nasal obstruction and headache in the occipital region. Her medical history was significant for two turbinoplasties within two months in the last year elsewhere with persistency of the disturbances. General ENT examination showed a soft tumor in the nasopharynx. The overlying mucosa was normal in appearance.
The patient underwent transnasal endoscopic biopsy that showed adamantinomatous craniopharyngioma with focal keratinization, corresponding to WHO grade I . Navigated microscopic/endoscopic sphenoidectomy and removal of the tumor from clivus and sellar floor with midfacial degloving was performed. The entire tumor was extradural. The tumor could be resected completely, safety margins were free of disease.
3. Case report
The three reports demonstrated that in all cases of intranasal or pharyngeal neoplasm the diagnosis of meningioma, craniopharyngioma and glioma should be considered as differential diagnosis. These three described tumors rarely appear in an extracranial localization.
Intracranial meningiomas are the most common adult benign intracranial neoplasms, whereas extracranial meningiomas are rare tumors comprising 1–2% of all meningiomas [4, 5]. The prognosis of menigiomas is generally favorable. However, in rare cases meningiomas are aggressive and can occur as a malignant meningioma. Avninder  described a case of a papillary meningioma as an aggressive histological variant, which accounts for 1.0–2.5% of all meningiomas.
Extracranial sinonasal tract meningiomas often demonstrate an erosion of the sinus wall, with extension to the surrounding soft tissue, to the orbit and occasionally to the skull base. Complete surgical extirpation of sinonasal meningioma is the treatment of choice without the need for adjuvant treatment. Relapses are possible in case of incomplete removal of the primary formation. The prognosis of extracranial meningioma is always good if the excision is complete .
Craniopharyngiomas are benign but aggressive tumors deriving from cell rests from the Rathke's pouch and account for about 3% of all intracranial tumors [8, 9]. Usually they are localized in the suprasellar region. The isolated infracranial localization was first described by Bock in 1924 . Infrasellar craniopharyngiomas are exceedingly rare because the sphenoid bone imposes a limitation on caudal tumor expansion. Less than 10 cases of infrasellar craniopharyngiomas in which the tumor had no sella involvement have been described . In contrast to patients with suprasellar craniopharyngiomas, generally presenting with headache and visual disturbance, patients with infrasellar craniopharyngiomas present usually only with nasal obstruction like in the present case. Surgical treatment of these tumors is indispensable. The approach is determined by the anatomic location of the tumor. Entirely infrasellar craniopharyngiomas may be removed completely, possibly offering a better prognosis than suprasellar craniopharyngiomas.
Nasal glial heterotopia are rare tumors, might derive from either separated neuroectodermal tissue during the closure of the covering brain, or from a nasal encephalocele which is covered by dura, pia, and arachnoid and later disconnected from the intracranial cavity during subsequent development. They manifest usually at birth or during early childhood and can cause a visible deformation, a nasal obstruction or chronic otitis media. Nasal gliomas may occur in extrananasal (60%) or intranasal localization (30%) or combined (10%) . The treatment of choice is complete surgical excision to avoid deformations of immature facial bones, cartilage necrosis as well as infections. A biopsy should not be performed because of the risk of provoking menigitis or injuring intact brain tissue. The overall outcome is good, depending on complete excision. Recurrences occur in 4–10%, most likely due to incomplete primary resection .
All three present cases demonstrate that preoperative MRI and CT imaging is important for differential diagnosis to exclude intracranial connections of the tumor. A definitive diagnosis requires histopathological and immunohistochemical examination. In all cases of intranasal or pharyngeal neoplasm the diagnosis of meningioma, craniopharyngeoma and glioma should be considered as a differential diagnosis, and intracranial connections should be excluded to optimize the surgical procedure.
In parts presented at the Joint Meeting of the European Skull Base Society and the German Society of Skull Base Surgery, May 2–5, 2007 Prague.
Written informed consent was obtained from the patients for publication and accompanying images.
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