Management of Malignant Tumors of the Anterior Skull Base: Experience with 76 Patients
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Neurosurg Focus 13 (4):Article 5, 2002, Click here to return to Table of Contents Management of malignant tumors of the anterior skull base: experience with 76 patients KETAN R. BULSARA, M.D., TAKANORI FUKUSHIMA, M.D., D.M.SC., AND ALLAN H. FRIEDMAN, M.D. Duke Division of Neurosurgery, Durham; and Carolina Neuroscience Institute, Raleigh, North Carolina As the management of anterior cranial fossa malignancies has undergone significant evolution, decreases in mor- bidity and mortality rates have occurred. In this article, the authors discuss the clinical presentation, neuroimaging find- ings, and management options for common anterior skull base malignancies. Also discussed are surgery-related indi- cations and principles. KEY WORDS • anterior skull base • transbasal • nasopharyngeal carcinoma • esthesioneuroblastoma • transfacial In 1941 Dandy described anterior craniofacial resection Anterior transfacial approaches were described as early of orbital tumors.20 Prior to this, Frazier27 championed re- as 1829 by Lizars. Modifications were described by Fer- moving orbital rims to gain improved surgical access. In gusson in 1842.9 In the 1920s, Portmann and Retrouvey 1943 Ray and McLean59 combined a transcranial and reported the midfacial degloving procedure.7 Casson and transfacial approach for resection of a retinoblastoma. The associates10 further modified this technique, which was first en bloc resection of an extensive paranasal sinus then popularized by Conley and Price16 and Price alone.55 malignancy was initially reported by Smith, et al.,71 in A subcranial approach obviating the need for the transfa- 1954 and subsequently by Ketcham, et al.,38 and Van Bu- cial approach was initially described by Raveh, et al.,58 in ren, et al.79 Although complete tumor resection had con- 1978 for the management of craniofacial fractures and siderable benefits, the toll was an 80% morbidity rate and subsequently performed for the management of anterior a 7% mortality rate.38,69 The major cause of morbidity was skull base tumors. intracranial contamination due to sinonasal tract commu- The management of malignant tumors of the anterior 64 nication. This complication was reduced when recon- skull base can be technically demanding and has been as- structive techniques involving pericranial grafts were in- sociated with high rates of morbidity and mortality. troduced in 1979 by Schramm, et al.,65 and modified later 37 Advances in neuroimaging leading to earlier diagnosis, a by Johns, et al. With improvements in surgical tech- better understanding of the anatomy, and collaboration niques, the morbidity rate decreased to less than 40% and 45,46 among experienced surgeons has led to significant pro- mortality to between 0 to 2%. gress in the management of these lesions. In 1960 Tessier, et al.,76 pioneered the transbasal ap- proach for the reduction of craniofacial abnormalities. Derome, alone,21 with Tessier,22 and with others23 used this MANAGEMENT OF ANTERIOR SKULL BASE approach for sphenoethmoidal tumor removal beginning MALIGANCIES in the 1970s. Modifications of this approach have led to increased anterior cranial fossa exposure requiring mini- Presentation and Neuroimaging Features mal brain retraction. Anterior fossa tumors can manifest as a number of dif- ferent symptoms, including nasal obstruction and conges- tion, epistaxis, rhinorrhea, hyposmia or anosmia, head- Abbreviations used in this paper: CA = carotid artery; CSF = cerebrospinal fluid; CT = computerized tomography; ICA = in- ache, seizure, and psychological changes. Paresthesias in ternal CA; MR = magnetic resonance; UCNT = undifferentiated the distribution of the V1 or V2 divisions of the trigemi- carcinoma of the nasopharyngeal type; WHO = World Health Or- nal nerve may occur. Invasion into the sella turcica can ganization. cause loss of endocrine function, or invasive pituitary tu- Neurosurg. Focus / Volume 13 / October, 2002 1 Unauthenticated | Downloaded 10/01/21 03:52 PM UTC K. R. Bulsara, T. Fukushima, and A. H. Friedman mors can be responsible for over production of cortico- tion with peaks in the second and sixth decades of life. steroids, growth hormone, or prolactin. Visual loss can be It occurs predominantly in males. It is divided into two uni- or bilateral. Associated ocular symptoms include dip- distinct histological entities: squamous cell carcinoma lopia, orbital pain, exophthalmos, and retroorbital head- and UCNT. The WHO classifies nasopharyngeal carcino- aches. Many of these tumors can be demonstrated on ma based on the degree of differentiation:67,70,81 Type I is nasopharyngeal examination. a keratinizing squamous cell carcinoma similar to that Diverse tumors can involve the anterior cranial fossa. found in the rest of the upper digestive tract; Type II is a Primary benign tumors with rare malignant potential in- nonkeratinizing epidermoid carcinoma; and Type III is clude meningiomas and pituitary adenomas. Primary ma- UCNT. Numerous staging schemes are present for maxil- lignant tumors include chordomas, chondrosarcomas, lary and ethmoid neoplasms; however, only the University osteogenic sarcomas, and invasive pituitary tumors. of Florida staging system can be applied to all parana- Malignant tumors with secondary involvement include sal neoplasms.50,52 In this classification system, T1 tumors carcinoma of the paranasal sinuses. Others include lym- are limited to a single site of origin such as a nasal wall phoma arising from the adenoids, minor salivary gland or ethmoids; T2 tumors extend to an adjacent sinus, or- neoplasms, ethmoid carcinoma, paragangliomas, olfactory bit, palate, nasopharynx, or pterygomaxillary sites; and neuroblastomas, mucoepidermoid carcinomas, and osteo- T3 tumors extend to or through the skull base. Resection sarcomas. Histologically benign tumors include angiofi- of T1 tumors is associated with the least morbidity and bromas and inverted papillomas. Metastases from renal, mortality breast, and lung carcinoma can also involve the anterior The UCNT differs from other head and neck squamous cranial fossa. Teratocarcinomas are rare in this region cell carcinomas by its histological features, epidemiologi- (Fig. 1). Lastly, paranasal sinus infections can extend into cal characteristics, and its relation with the Epstein–Barr the anterior skull base. virus. Of nasopharyngeal carcinomas, it is the more preva- Computerized tomography and MR imaging provide lent. It is endemic in Southeast Asia, parts of the Med- complementary information regarding skull base lesions. iterranean basin, North Africa, parts of the Caribbean and Whereas CT scanning is better at defining osseous anato- Alaska. A characteristic feature of the undifferentiated tu- my and calcifications, MR imaging is superior at demon- mor is massive lymphoid T-cell infiltration. strating the relationship of skull base masses to soft-tissue The UCNT is associated with the Epstein–Barr vir- structures such as cranial nerves, the CA, the jugular vein, us.35,75,78,80 Deletion of tumor suppressor genes such as p16 and the brain. In the case of parasellar masses, MR imag- or the activation of oncogenes such as bcl-2 may predis- ing is better in excluding CA aneurysms. pose individuals to the development of WHO Types II and Nasopharyngeal Carcinoma. Nasopharyngeal carcino- III carcinoma.47,48 Dietary factors such as salted fish high ma arises from the surface epithelium of the nasopharynx in volatile nitrosamines have also been implicated in and commonly metastasizes to the lymph nodes, with UCNT.82 some preference for those at the apex of the posterior cer- Squamous cell carcinoma (WHO Type I) is more com- vical triangle.18 As many as 82% of the tumors arise in the mon in the Western population. The use of tobacco and lateral wall of the nasopharynx in Rosenmüller fossa (pha- alcohol may play a role in the development of this well- ryngeal recess), 12% in the midline, and 6% in normal- differentiated tumor.13 It is rarely associated with the Ep- appearing mucosa.70 stein–Barr virus. Mutations of the p53 gene commonly Nasopharyngeal carcinoma has a bimodal age distribu- occur.26 Patients with nasopharyngeal carcinoma may present with variable nonspecific symptoms, the most common of which is painless posterior cervical adenopathy. Lateral pharyngeal node involvement may result in pain with ipsi- lateral neck rotation or ear pain. Unilateral conductive hearing loss resulting from serous effusions due to poor eustachian tube function commonly occurs. Nasal ob- struction occurs late in the course of the disease and pro- fuse epistaxis is rare. Dysphonia, difficulty swallowing, and diplopia may also occur. Physical examination may reveal maxillary nerve dysfunction. Extension of the tu- mor into the lateral pharynx results in ninth and 10th cra- nial nerve palsies. Proptosis and trismus are rarely associ- ated with these malignancies. Nasopharyngeal carcinoma is associated with bone de- struction demonstrated on CT scanning. Intense homoge- neous enhancement of the lesion is seen following con- trast administration. The lesion can invade the middle fossa either by erosion through the greater wing of the Fig. 1. Neuroimaging studies. Left: Pathological diagnosis sphenoid or extension through the foramen ovale. In ad- of this heterogeneously enhancing lesion was teratocarcinoma. vanced cases, the cancer can reach the clivus and violate Right: Two years following an extensive transbasal resection, no areas of the posterior fossa. Adenoid cystic carcinomas tumor recurrence is noted.