Neurosurg. R e v .
15 (199'2) 187-192
M a l i g n a n t tumors o f the anterior cranial skull base Uwe Neubauer 1, Rudolf Fahlbusch 1, Malte E. Wigand z, and Manfred Weidenbecher z 1Neurosurgical Clinic, 2Ear-Nose and Throat-University Clinic Ertangen-Niirnberg, Fed. Rep. of Germany
Abstract Malignant tumors of the anterior cranial skull base are still a challenge for radical surgical treatment. Several different techniques and approaches have been developed over the years and the results, with mortality rates over 50%, are still not encouraging. Here we present our results of an interdisciplinary, onestage, neurohino transfronto-transbasal surgical approach in twelve patients with such tumors. The leng-term survival rate in our patients is now 83% with a mean postoperative follow-up of 19.3 months. Two patients died due to early recurrencies and metastasis after 10 and 13 months after initial treatment and postoperative irradiation. Another five patients with a follow-up of 34.2 months have had tumor recurrencies diagnosed at 19.5 months postoperatively. These patients have survived their recurrent tumor for 14.7 months. Five patients, 41% of the whole group, have been living without evidence of tumor recurrence for between 2 and 35 months.
structures most authors utilized a combined transcranial-transfacial approach, either in one operation or in two with a time interval of several days. The principle of this procedure is that the separate approach to the intracranial and the extracranial space should lower the risk of infection. In the earlier years a better visualization of the operated structures was also achieved. In the era of microsurgery we beIieve this is no longer necessary. Tumor removal can be performed completely and safely by an interdisciplinary neurorhinosurgical team using a transfronto-transbasal approach, as the authors have already shown in the treatment of seven patients with esthesioneuroblastoma in the advanced stage [5]. Meanwhile several reports have been published indicating that the feasibility of this approach has been recognized and that it has found wider use in neurosurgical practice [1, 5, 17].
Keywords: Nasopharyngeal carcinoma, sarcoma, skull base, surgical approach.
2 Material and methods 2.1 Patients
1 Introduction Malignant tumors of the anterior skull base present several surgical problems. A m o n g these, the anatomical situation with the simultaneous involvement of the intra- and extracranial space is the most important, as these tumors carry the risk of postoperative infections. Tumors growing into areas such as the orbit, the optic canal, or the orbital fissure carry the risk of severe postoperative neurological deficits. Since the early fifties surgical approaches have been developed to radically resect these tumors, because irradiation therapy alone was unable to cure the disease [22]. To reach all the affected 9 1992by Walter de Gruyter & Co. Berlin - New York
In the Neurosurgical Department of the University of Erlangen-Nfirnberg (FRG), we treated 12 consecutive cases o f malignant tumors of the anterior skull base between 1984 and 1990 with this procedure. Ten patients complained of impaired nasal respiration and six had had several episodes of epistaxis. These patients were first examined by the E N T specialist. Endoscopy of the nasal cavity and paranasal sinuses revealed the tumor clearly and endonasal biopsy confirmed the histological diagnosis. The duration of symptoms in these cases ranged from weeks to months. Nine patients already had anosmia preoperatively.
188
Neubauer et al., Malignant tumors of the anterior cranial skull base
Two patients had a history typical of patients with frontal space-occupying lesions including longlasting severe headache and neuropsychological changes. The patients were disoriented, at times even confused. In five cases with involvement of the orbit, a proptosis of the eye was found. In three cases the tumor we dealt with was a recurrence. The patients had already undergone operation and irradiation two years, four years and in one case even 20 years before. Comparison of the histological examination revealed identical findings in these cases, so that the assumption of a recurrence was justified although in the last case a second independent manifestation of the same disease is conceivable. All but two cases had local destruction of the anterior skull base, but CSF leakage was never observed.
2.2 Radiology All patients were examined by plain X-rays of the skull and CT scan. M R I scan was performed in three cases only, because the CT scan shows the bony structures involved [26], which are most important in the surgical planning, better (Figure I).
In all 12 cases the tumor was identified in the nasal cavity and the ethmoid sinus. The sphenoid sinus was involved in nine cases, in four cases, and the maxillary sinus in five cases. In two patients the tumor had reached and partially destroyed the clivus. All patients had a subfrontal tumor but only three patients had a significant intracranial tumor growth with perifocal edema in the frontal white matter. Involvement of the orbit was found in five cases.
2.3 Operative technique The operative technique has been described in detail in a previous report [5] and thus will only be summarized here in short. With the patient in supine position a bifrontal craniotomy along the orbital roof is performed preserving a large pedicled pericranial flap. After opening of the dura, CSF is taken from the basal cisterns to relax the brain. Then the intracranial part of the tumor is resected. Next the dura of the skull base is reseeted lateral to the medial sagittal line of the orbit and posterior to the sphenoid wing leaving just a small rim of 2 - 3 m m for later suturing. The bony skull base is opened with a rosehead drill. With this access the tumor tissue in the frontal, ethmoidal and sphenoidal sinus can be removed. In cases with involvement of the orbit, the affected orbital wall is then resected and the intraorbital tumor removed. Next the tumor mass in the nasal cavity and the maxillary sinus is resected by ENTsurgeon. The whole procedure is carried out using an operative microscope and under antibiotic prophylaxis (600 mg Clindamycin) which is continued for three days postoperatively.
Figure 1. CT scan in axial view and frontal reconstruction of an adenoid-cystic carcinoma invading the anterior skull base, nasal cavity, and both orbits.
The closure of the skull base is performed with autologous material only. For the outer layer a graft of fascia lata taken from the patient is sutured watertight to the basal dura. For the inner layer the predicled periostal flap is placed over the entire floor of the anterior fossa and again sutured to the basal dura. No bone graft or arteficial material is used. The skull is closed as usual (Figure 2). Neurosurg. Rev. 15 (1992)
Neubauer et al., Malignant tumors of the anterior cranial skull base ........................ >_................. \
)
',,,( .........................
,
"~/
,
Oeriostal flap
oil base defect
189
exan, methotrexat and corticoids. In two patients, one with an undifferentiated carcinoma and one with an adenoid-cystic carcinoma, we started immunostimulation therapy with interferon-beta (fiblaferon) after operation for tumor recurrencies 12 and 25 months, respectively, after our initial treatement. Both again have tumor tissue visible in their control CT scans but have been living with it since 19 and 23 months.
3 Results and follow-up Figure 2. Schematic drawing of exposure and closure of the anterior skull base using the transcranial-transbasal approach.
The tumor type we found most often was adenoidcystic carcinoma (6 cases). Two patients had a squamous cell carcinoma and one an undifferentiated carcinoma. Sarcomas were found in the remaining three cases, a chondrosarcoma in a woman of 74 years and two rhabdomyosarcomas in children of six and seven years (Table I).
Table I. Malignant tumors of the anterior skullbase: Histology n
Adenoid cystic carcinoma Squarnous cell carcinoma Undifferentiated carcinoma Rhabdomyosarcoma Chondrosarcoma
2.5 Irradiation and chemotherapy Radiation therapy was carried out in all our cases. Single doses of 2 Gy were given five times pr week up a total dose of 60 Gy in the target volume. Usually an a. p.-portal and 1 - 2 lateral fields with wedge filters were applied. Irradiation was done postoperatively in ten patients as an adjuvant therapy. In the two children with a rhabdomyosarcoma irradiation and chemotherapy was carried out before surgery. Chemotherapy included systemic vincristin, actinomycin D, ifosfamid, and adriblastin and additional intrathecal administration of alNeurosurg. Rev. 15 (1992)
(10 out of 12). All patients had regular follow-up examinations including nasal endoscopy for recurrencies and CT examinations.
2.4 Histology
Type
The mean follow-up in our series is now 19.4 mouths with a minimum of four and a maximum of 42 months. The long-term survival rate is 83%
Two of our 12 patients died after an early recurrence. One of these had an adeno-cystic carcinoma which had already been operated on by the transfacial route and irradiated two years before. The recurrence we operated on involved all paranasal sinuses, the skull base, and the orbit. Further irradiation therapy at this time was not possible. The patient had another a local recurrence and died 10 months after operation. The second patient had a squamous cell carcinoma which recurred locally and produced a cervical metastasis after six months. This patient died 13 months postoperatively. Post mortem examination was not performed. The ten survivors have a mean follow-up of 21.1 months, raging from 4 to 40 months. Half of them have had tumor recurrencies diagnosed between 8 and 30 months after operation. Three were intracranial and have been removed, but only one completely. The fourth was endonasal and operated this way. The last is growing along the skull base down the clivus and was not operated, because the patient also has multiple pulmonary metastasis known since 15 months. These patients survived their recurrencies so far for 13.1 months (6 to 23 months), two of them under interferon treatment. Five survivors (41.6 %) are living without any evidence of recurrence since 19.6 months (range: 4 to 35 months). This group includes one patient with adenoidcystic and with squamous cell carcinoma, one with chondrosarcoma, and the two children with a thabdomyosarcoma.
190
Neubauer et al., Malignant tumors of the anterior cranial skull base
Table II. Malignant tumors of the anterior skull base Case
Age/sex
Local.*
Irrad.
Chemother.
Complic.
Outcome
I. Carcinoma
1
53 m
1, 2, 4, 6, 7
postop.
-
-
recurrence alive
30 mo 42 mo
2
52 m
1, 2, 6
postop.
--
-
alive
30 mo
3
31 f
1, 2, 4, 5, 6, 7
postop.
+
osteomyelitis
recurrence alive
25 mo 44 mo
4
29 m
1, 2, 4, 6
postop.
+
-
recurrence alive
12 mo 35 mo
5
51 m
1, 2, 6
postop.
-
-
recurrence alive
8 mo 26 mo
6
47 m
1, 2, 3, 4, 5, 6, 7
preop.
-
-
died
10 mo
7
53 m
1, 2, 5, 6
preop.
-
-
recurrence died
6 mo 13 mo
8
76 f
t, 2, 3, 4, 5, 6
postop.
-
ICH
recurrence alive
22 mo 28 mo
9
51 f
1, 2, 3, 4, 6, 7
postop.
-
-
alive
6 mo
6f
1, 2, 4
preop.
+
-
alive
35 mo
11
8f
1, 2, 4, 6
preop.
+
-
alive
23 mo
12
74 f
1, 2, 3, 4, 5, 6, 7
postop.
-
-
alive
2 mo
II. Sarcoma
l0
"
* localization 1) nasal cavity 2) ethmoidal sinus 3) frontal sinus 4) sphenoidal sinus 5) maxillary sinus 6) intracranial (subfrontal) 7) orbit
We h a d n o operative deaths in o u r series. The o n l y c o m p l i c a t i o n s we have seen were one infected b o n e flap, which h a d to be r e m o v e d a n d one f r o n t a l i n t r a c e r e b r a l h e m a t o m a that h a d to be e v a c u a t e d (fortunately w i t h o u t p r o d u c i n g a n y deficit) (Table II). 4 Discussion
U n t i l n o w the m a j o r i t y o f a u t h o r s [2, 7, 18, 23] have r e c o m m e n d e d the c o m b i n e d cranofacial app r o a c h to m a l i g n a n t t u m o r s o f the a n t e r i o r c r a n i a l skull base for v a r i o u s reasons. T h e m o s t i m p o r t a n t is the risk o f severe i n t r a c r a n i a l infections, which were m a i n l y responsible for the high c o m p l i c a t i o n
rate in the early reports o n o p e r a t i o n for this disease [12, 25]. A n o t h e r r e a s o n is r o o t e d in the history o f skull base surgery. Surgery o f m a l i g n a n t t u m o r s o f the a n t e r i o r skull base b e g a n with treatm e n t of m a l i g n a n c i e s o f the p a r a n a s a l sinuses by E N T specialists [15]. Since radical resection o f the a d j a c e n t p a r t o f the skull base b y this r o u t e alone is n o t possible [6], a n a d d i t i o n a l t r a n s c r a n i a l app r o a c h [10] was required. T h e p r o g n o s i s for patients u n d e r g o i n g this p r o c e d u r e is p o o r with fewer t h a n 2 0 % l o n g - t e r m survivors [15], a c o m p l i c a t i o n rate u p to 7 0 % , i n c l u d i n g a n operative m o r t a l i t y o f 6 % [12], a n d a n increase o f l o n g - t e r m survival o f o n l y a b o u t 30% [25]. The t r a n s c r a n i a l p a r t o f the p r o c e d u r e was p e r f o r m e d t h r o u g h a small Neurosurg. Rev. 15 (1992)
Neubauer et al., Malignant tumors of the anterior cranial skull base
191
frontal opening which made the inspection of the anterior cranial fossa and resection of the cribriform plate easier. Further development of the operative technique and better management of postoperative complications improved the results to a long-term survival rate of 49% [11] and 40% [2].
ing the VAIA protocol and combined with irradiation. Both live free of recurrence. A similar regime was followed by Roux [18] in 11 cases of ethmoidal carcinomas with a two year survival rate of 84%. These good results seem worth further clinical investigation.
DEROME [3] used the method of a primary transcranial-transbasal route to tumors of the anterior skull base and adjacent paranasal sinuses, which Loew had performed already in the 60ies [9], in a large number of patients.
Interferon therapy was performed as an adjuvant therapy in two patients with tumor recurrencies that could not be removed completely and who had already received a full irradiation treatment. There is increasing evidence that interferon beta might be able to reduce or even stop the progression of these tumors [14, 24].
This technique was utilized by several authors, but still combined with a second transfacial approach by some [19]. LESOIN [13] used the DEROME approach for operations in the frontal and ethmoid sinus only. Meanwhile this approach has established itself for the resection of tumors localized in the nasal cavity, the maxillary sinus and the orbit as well [1, 5]. In our series we found a longterm survival of 83% with a mean follow-up of 19.3 months compared to an average longterm survival of about 40% reported in available literature [2, 8, 11, 23]. But among these survivors are five patients (41% of the whole group) who have had a tumor recurrence, which could only be removed totally in two of them. Thus the results seem to get worse with a longer follow-up. A long-term survival without any evidence of recurrence has been achieved in 41% of the patients, in three of them for more than two years. CHEESMAN [2] found the tumor related death occurred early in the postoperative course, two thirds in the first year, all due to early recurrence of the tumor. This is supported by our data with the two deaths occuring after 10 and 13 months. The question of whether tumors with later recurrencies have more benign biological behavior and thus the patients a better chance of survival must be answered by a longer follow-up of the patients. In the two children with a rhabdomyosarcoma, preoperative chemotherapy was performed follow-
Our low complication rate and lack of CSF leakages or intracranial infections proves that the closure of the skull base defect with autologous fascia and a pedicled pericranial flap alone is sufficiant [16]. The use of a free bone graft [1, 4, 20, 21] is not necessary in our view. 5 Conclusion The authors believe that the interdisciplinary neurorhinosurgical transcranial-transbasal approach to malignant tumors of the anterior cranial skull base is a safe and effective method with a low operative morbidity and a comparatively good long-term survival rate. Acknowledgements: The authors would like to thank Prof. Dr. THIERAUF(Institut fiir Pathologic der Universit/it Erlangen) for his expert diagnosis, Prof. Dr. BECK and Dr. RICHTER(Kinderklinik der Universitfit Erlangen) who took care of, and administed chemotherapy to the children included in this paper, Dr. J. HONEGGER (Neurochir. Klinik der Universit/it Erlangen) who was responsible for the interferon treatment in two adult patients, and Prof. Dr. SAUERand coworkers (Strahlentherapeutische Klinik der Universitfit Erlangen) who performed the irradiation treatment in our patients. Remark: This work has been presented in part at poster session fo the 41st Annual Meeting of the German Society for Neurosurgery, May 2 7 - 30, 1990 in Diisseldorf, Fed. Rep. of Germany (41. Jahrestagung der Deutschen Gesellschaft f/ir Neurochirurgie).
References [1] ARITAN, M SHINTARO,M SANO,T HAYAKAWA,K NAKAO, N KANAI,H MOGAMI:Surgical treatment of tumors in the anterior skull base using the transbasal approach. Neurosurgery 24 (1989) 379-384 Neurosurg. Rev. 15 (1992)
[2] CHEESMANA, VJ LUND,DJ HOWARD:Craniofacial resection for tumors of the nasal cavity and paranasal sinuses. Head & Neck Surgery 8 (1986) 429435
192
Neubauer et al., Malignant tumors of the anterior cranial skull base
[3] D~ROM~P: Les tumeurs spheno-ethmoidales. Neurochirurgie 18 (1986) suppl 1 [4] DEROtr P: Transbasal approach to tumors invading the base of the skull. In: SCHMmEKHH, WH SWEET (eds): Operative neurosurgical techniques Volt, Grune & Stratton, New York 1984 [5] FAHLBUSCHR, U NEUBAUER,ME WIGAND,M WEIDENBECHER,G R()CKELEIN, P THIERAUF,U SAUER: Neuro-rhinosurgical treatment of aesthesioneuroblastoma. Acta Neurochir (Wien) 100 (1989) 9 3 100 [6] FRAZELLEL, JS LEWIS:Cancer of the nasal cavity and accessory sinuses. Cancer 16 (1963) 1293-1301 [7] JACKSONIT, ER LAWS,RD MARTIN:A craniofacial approach to advanced recurrent cancer of the central face. Head Neck Surg 5 (1983) 474--488 [8] JACKSON IT, IR MUNRO, T HIDE: Treatment of tumors involving the anterior cranial fossa. Head Neck Surg 6 (1984) 901-913 [9] JAKUMEIT HD: Neuroblastoma of the olfactory nerve. Acta Neurochir (Wien) 25 (1971) 99-108 [10] K~TCrtAM AS, RH WILKINS, JM VANBUR~N, RR SMI~: A combined intracranial facial approach to the paranasal sinuses. Am J Surg 106 (1963) 6 9 8 703 [11] KETCHAMAS, PB CHRETIEN,JM VANBUREN:The etbmoid sinuses: a re-evaluation of surgical resection. Am J Sure 126 (1973) 469-476 [12] K~TCHAMAS, RC HOVE, JM VANBUR~N,RH JOHNSON, RR SMITH:Complications of intracranial facial resection for tumors of the paranasal sinuses. Am J Sure 112 (1966) 591-596 [13] LESOINF, CE THOMASIII, L gILLETTE:The midline supra-orbital approach, using a large single free bone flap. Acta Neurochir 87 (1987) 8 6 - 8 9 [14] MERTENSR, JH KARSTENS,J AMMON,HW CLASSEN, CH MITTERMAYER:Bisherige Erfahrungen der Interferontherapie an 7 Patienten mit Nasopharynxkarzinom. In: WANNENMACHERM (ed): Nasopharynxtumoren, Urban und Schwarzenberg, Miinchen - W i e n - Baltimore 1984 [15] OSBORNDA, P WINSTON: Carcinoma of the paranasal sinuses. J Laryng & Otol 75 (1961) 387-405 [16] PRICE JC, M LOURY, B CARSON,ME JOHNS: The pericranial flap for reconstruction of anterior skull base defects. Laryngoscope 98 (1988) 1159-1164
[17] ROSENEM, FA SIMEONE,DA BRUCE: Single stage composite resection and reconstruction of malignant anterior skull base tumors. Neurosurgery 18 (1986) 7--11 [18] Roux FX, D BRASNU,H LACCOURREYE,A FABle, JP CHODKIEWICZ: Les ad6nocarcinomes ethmoidaux op6r6s en un temps par vole transfaciale et sous-frontale apr~s chimiotherapie d'induction. Neurochirurgie 33 (1987) 365-370 [19] SAMnM, W DRAF: Neurosurgical ENT Treatment of lesions of the skull base. In: FROWEIN RA, O WILCKE, A KARIMI-NEJAD,M BROCK, M KLINGER (eds): Advances in Neurosurgery Vol 5, Springer Verlag, Berlin-Heidelberg-New York 1978 [20] SCI-mAMMV: Anterior skull base surgery for benign and malignant disease. Laryngoscope 89 (1979) 1077-1091 [21] SCHWAABG, P MaRANDAS:Les probl6mes du traitement chirurgical des tumeurs malignes de l'ethmoide par abord mixte endocranien et facial. Ann Oto-Laryng (Paris) 100 (1983) 159-161 [22] SMITH RR, CT KLOPP, JM WILLIAMS: Surgical treatment of cancer of the frontal sinus and adjacent areas. Cancer 7 (1954) 991-994 [23] TERZ JJ, HF YOtrNG, W LAWRENCE: Combined craniofacial resection for locally advanced carcinoma of the head and neck. Am J Surg 140 (1980) 613-624 [24] TR~tJNBRJ, D NIETHAMMER,G DANNECKER,R HAGMANN,V NEEF, PH ttOFSCHNEIDER:Successful treatment of nasopharyngeal carcinoma with interferon. Lancet (1980) 817- 818 [25] VAN BURENJM, AK OMMAYA,AS KETCHAM:Ten years experience with radical combined cranio-facial resection of malignant tumors of the paranasal sinuses. J Neurosurg 28 (1968) 3 4 1 - 350 [26] VOLLEE, J TREISCH,C CLAUSSEN,HJ KAUFMANN: Lesions of the skull base observed on high resolution computed tomography. Acta Radiologica 30 (1989) 129-134 Submitted October 1, 1990. Accepted April 4, 1991. Dr. Uwe Neubauer Neurochirurgische Klinik der Universit/it Erlangen-Nfirnberg Schwabachanlage 6 W-8520 Erlangen Fed. Rep. of Germany
Neurosurg. Rev. 15 (1992)
15 (199'2) 187-192
M a l i g n a n t tumors o f the anterior cranial skull base Uwe Neubauer 1, Rudolf Fahlbusch 1, Malte E. Wigand z, and Manfred Weidenbecher z 1Neurosurgical Clinic, 2Ear-Nose and Throat-University Clinic Ertangen-Niirnberg, Fed. Rep. of Germany
Abstract Malignant tumors of the anterior cranial skull base are still a challenge for radical surgical treatment. Several different techniques and approaches have been developed over the years and the results, with mortality rates over 50%, are still not encouraging. Here we present our results of an interdisciplinary, onestage, neurohino transfronto-transbasal surgical approach in twelve patients with such tumors. The leng-term survival rate in our patients is now 83% with a mean postoperative follow-up of 19.3 months. Two patients died due to early recurrencies and metastasis after 10 and 13 months after initial treatment and postoperative irradiation. Another five patients with a follow-up of 34.2 months have had tumor recurrencies diagnosed at 19.5 months postoperatively. These patients have survived their recurrent tumor for 14.7 months. Five patients, 41% of the whole group, have been living without evidence of tumor recurrence for between 2 and 35 months.
structures most authors utilized a combined transcranial-transfacial approach, either in one operation or in two with a time interval of several days. The principle of this procedure is that the separate approach to the intracranial and the extracranial space should lower the risk of infection. In the earlier years a better visualization of the operated structures was also achieved. In the era of microsurgery we beIieve this is no longer necessary. Tumor removal can be performed completely and safely by an interdisciplinary neurorhinosurgical team using a transfronto-transbasal approach, as the authors have already shown in the treatment of seven patients with esthesioneuroblastoma in the advanced stage [5]. Meanwhile several reports have been published indicating that the feasibility of this approach has been recognized and that it has found wider use in neurosurgical practice [1, 5, 17].
Keywords: Nasopharyngeal carcinoma, sarcoma, skull base, surgical approach.
2 Material and methods 2.1 Patients
1 Introduction Malignant tumors of the anterior skull base present several surgical problems. A m o n g these, the anatomical situation with the simultaneous involvement of the intra- and extracranial space is the most important, as these tumors carry the risk of postoperative infections. Tumors growing into areas such as the orbit, the optic canal, or the orbital fissure carry the risk of severe postoperative neurological deficits. Since the early fifties surgical approaches have been developed to radically resect these tumors, because irradiation therapy alone was unable to cure the disease [22]. To reach all the affected 9 1992by Walter de Gruyter & Co. Berlin - New York
In the Neurosurgical Department of the University of Erlangen-Nfirnberg (FRG), we treated 12 consecutive cases o f malignant tumors of the anterior skull base between 1984 and 1990 with this procedure. Ten patients complained of impaired nasal respiration and six had had several episodes of epistaxis. These patients were first examined by the E N T specialist. Endoscopy of the nasal cavity and paranasal sinuses revealed the tumor clearly and endonasal biopsy confirmed the histological diagnosis. The duration of symptoms in these cases ranged from weeks to months. Nine patients already had anosmia preoperatively.
188
Neubauer et al., Malignant tumors of the anterior cranial skull base
Two patients had a history typical of patients with frontal space-occupying lesions including longlasting severe headache and neuropsychological changes. The patients were disoriented, at times even confused. In five cases with involvement of the orbit, a proptosis of the eye was found. In three cases the tumor we dealt with was a recurrence. The patients had already undergone operation and irradiation two years, four years and in one case even 20 years before. Comparison of the histological examination revealed identical findings in these cases, so that the assumption of a recurrence was justified although in the last case a second independent manifestation of the same disease is conceivable. All but two cases had local destruction of the anterior skull base, but CSF leakage was never observed.
2.2 Radiology All patients were examined by plain X-rays of the skull and CT scan. M R I scan was performed in three cases only, because the CT scan shows the bony structures involved [26], which are most important in the surgical planning, better (Figure I).
In all 12 cases the tumor was identified in the nasal cavity and the ethmoid sinus. The sphenoid sinus was involved in nine cases, in four cases, and the maxillary sinus in five cases. In two patients the tumor had reached and partially destroyed the clivus. All patients had a subfrontal tumor but only three patients had a significant intracranial tumor growth with perifocal edema in the frontal white matter. Involvement of the orbit was found in five cases.
2.3 Operative technique The operative technique has been described in detail in a previous report [5] and thus will only be summarized here in short. With the patient in supine position a bifrontal craniotomy along the orbital roof is performed preserving a large pedicled pericranial flap. After opening of the dura, CSF is taken from the basal cisterns to relax the brain. Then the intracranial part of the tumor is resected. Next the dura of the skull base is reseeted lateral to the medial sagittal line of the orbit and posterior to the sphenoid wing leaving just a small rim of 2 - 3 m m for later suturing. The bony skull base is opened with a rosehead drill. With this access the tumor tissue in the frontal, ethmoidal and sphenoidal sinus can be removed. In cases with involvement of the orbit, the affected orbital wall is then resected and the intraorbital tumor removed. Next the tumor mass in the nasal cavity and the maxillary sinus is resected by ENTsurgeon. The whole procedure is carried out using an operative microscope and under antibiotic prophylaxis (600 mg Clindamycin) which is continued for three days postoperatively.
Figure 1. CT scan in axial view and frontal reconstruction of an adenoid-cystic carcinoma invading the anterior skull base, nasal cavity, and both orbits.
The closure of the skull base is performed with autologous material only. For the outer layer a graft of fascia lata taken from the patient is sutured watertight to the basal dura. For the inner layer the predicled periostal flap is placed over the entire floor of the anterior fossa and again sutured to the basal dura. No bone graft or arteficial material is used. The skull is closed as usual (Figure 2). Neurosurg. Rev. 15 (1992)
Neubauer et al., Malignant tumors of the anterior cranial skull base ........................ >_................. \
)
',,,( .........................
,
"~/
,
Oeriostal flap
oil base defect
189
exan, methotrexat and corticoids. In two patients, one with an undifferentiated carcinoma and one with an adenoid-cystic carcinoma, we started immunostimulation therapy with interferon-beta (fiblaferon) after operation for tumor recurrencies 12 and 25 months, respectively, after our initial treatement. Both again have tumor tissue visible in their control CT scans but have been living with it since 19 and 23 months.
3 Results and follow-up Figure 2. Schematic drawing of exposure and closure of the anterior skull base using the transcranial-transbasal approach.
The tumor type we found most often was adenoidcystic carcinoma (6 cases). Two patients had a squamous cell carcinoma and one an undifferentiated carcinoma. Sarcomas were found in the remaining three cases, a chondrosarcoma in a woman of 74 years and two rhabdomyosarcomas in children of six and seven years (Table I).
Table I. Malignant tumors of the anterior skullbase: Histology n
Adenoid cystic carcinoma Squarnous cell carcinoma Undifferentiated carcinoma Rhabdomyosarcoma Chondrosarcoma
2.5 Irradiation and chemotherapy Radiation therapy was carried out in all our cases. Single doses of 2 Gy were given five times pr week up a total dose of 60 Gy in the target volume. Usually an a. p.-portal and 1 - 2 lateral fields with wedge filters were applied. Irradiation was done postoperatively in ten patients as an adjuvant therapy. In the two children with a rhabdomyosarcoma irradiation and chemotherapy was carried out before surgery. Chemotherapy included systemic vincristin, actinomycin D, ifosfamid, and adriblastin and additional intrathecal administration of alNeurosurg. Rev. 15 (1992)
(10 out of 12). All patients had regular follow-up examinations including nasal endoscopy for recurrencies and CT examinations.
2.4 Histology
Type
The mean follow-up in our series is now 19.4 mouths with a minimum of four and a maximum of 42 months. The long-term survival rate is 83%
Two of our 12 patients died after an early recurrence. One of these had an adeno-cystic carcinoma which had already been operated on by the transfacial route and irradiated two years before. The recurrence we operated on involved all paranasal sinuses, the skull base, and the orbit. Further irradiation therapy at this time was not possible. The patient had another a local recurrence and died 10 months after operation. The second patient had a squamous cell carcinoma which recurred locally and produced a cervical metastasis after six months. This patient died 13 months postoperatively. Post mortem examination was not performed. The ten survivors have a mean follow-up of 21.1 months, raging from 4 to 40 months. Half of them have had tumor recurrencies diagnosed between 8 and 30 months after operation. Three were intracranial and have been removed, but only one completely. The fourth was endonasal and operated this way. The last is growing along the skull base down the clivus and was not operated, because the patient also has multiple pulmonary metastasis known since 15 months. These patients survived their recurrencies so far for 13.1 months (6 to 23 months), two of them under interferon treatment. Five survivors (41.6 %) are living without any evidence of recurrence since 19.6 months (range: 4 to 35 months). This group includes one patient with adenoidcystic and with squamous cell carcinoma, one with chondrosarcoma, and the two children with a thabdomyosarcoma.
190
Neubauer et al., Malignant tumors of the anterior cranial skull base
Table II. Malignant tumors of the anterior skull base Case
Age/sex
Local.*
Irrad.
Chemother.
Complic.
Outcome
I. Carcinoma
1
53 m
1, 2, 4, 6, 7
postop.
-
-
recurrence alive
30 mo 42 mo
2
52 m
1, 2, 6
postop.
--
-
alive
30 mo
3
31 f
1, 2, 4, 5, 6, 7
postop.
+
osteomyelitis
recurrence alive
25 mo 44 mo
4
29 m
1, 2, 4, 6
postop.
+
-
recurrence alive
12 mo 35 mo
5
51 m
1, 2, 6
postop.
-
-
recurrence alive
8 mo 26 mo
6
47 m
1, 2, 3, 4, 5, 6, 7
preop.
-
-
died
10 mo
7
53 m
1, 2, 5, 6
preop.
-
-
recurrence died
6 mo 13 mo
8
76 f
t, 2, 3, 4, 5, 6
postop.
-
ICH
recurrence alive
22 mo 28 mo
9
51 f
1, 2, 3, 4, 6, 7
postop.
-
-
alive
6 mo
6f
1, 2, 4
preop.
+
-
alive
35 mo
11
8f
1, 2, 4, 6
preop.
+
-
alive
23 mo
12
74 f
1, 2, 3, 4, 5, 6, 7
postop.
-
-
alive
2 mo
II. Sarcoma
l0
"
* localization 1) nasal cavity 2) ethmoidal sinus 3) frontal sinus 4) sphenoidal sinus 5) maxillary sinus 6) intracranial (subfrontal) 7) orbit
We h a d n o operative deaths in o u r series. The o n l y c o m p l i c a t i o n s we have seen were one infected b o n e flap, which h a d to be r e m o v e d a n d one f r o n t a l i n t r a c e r e b r a l h e m a t o m a that h a d to be e v a c u a t e d (fortunately w i t h o u t p r o d u c i n g a n y deficit) (Table II). 4 Discussion
U n t i l n o w the m a j o r i t y o f a u t h o r s [2, 7, 18, 23] have r e c o m m e n d e d the c o m b i n e d cranofacial app r o a c h to m a l i g n a n t t u m o r s o f the a n t e r i o r c r a n i a l skull base for v a r i o u s reasons. T h e m o s t i m p o r t a n t is the risk o f severe i n t r a c r a n i a l infections, which were m a i n l y responsible for the high c o m p l i c a t i o n
rate in the early reports o n o p e r a t i o n for this disease [12, 25]. A n o t h e r r e a s o n is r o o t e d in the history o f skull base surgery. Surgery o f m a l i g n a n t t u m o r s o f the a n t e r i o r skull base b e g a n with treatm e n t of m a l i g n a n c i e s o f the p a r a n a s a l sinuses by E N T specialists [15]. Since radical resection o f the a d j a c e n t p a r t o f the skull base b y this r o u t e alone is n o t possible [6], a n a d d i t i o n a l t r a n s c r a n i a l app r o a c h [10] was required. T h e p r o g n o s i s for patients u n d e r g o i n g this p r o c e d u r e is p o o r with fewer t h a n 2 0 % l o n g - t e r m survivors [15], a c o m p l i c a t i o n rate u p to 7 0 % , i n c l u d i n g a n operative m o r t a l i t y o f 6 % [12], a n d a n increase o f l o n g - t e r m survival o f o n l y a b o u t 30% [25]. The t r a n s c r a n i a l p a r t o f the p r o c e d u r e was p e r f o r m e d t h r o u g h a small Neurosurg. Rev. 15 (1992)
Neubauer et al., Malignant tumors of the anterior cranial skull base
191
frontal opening which made the inspection of the anterior cranial fossa and resection of the cribriform plate easier. Further development of the operative technique and better management of postoperative complications improved the results to a long-term survival rate of 49% [11] and 40% [2].
ing the VAIA protocol and combined with irradiation. Both live free of recurrence. A similar regime was followed by Roux [18] in 11 cases of ethmoidal carcinomas with a two year survival rate of 84%. These good results seem worth further clinical investigation.
DEROME [3] used the method of a primary transcranial-transbasal route to tumors of the anterior skull base and adjacent paranasal sinuses, which Loew had performed already in the 60ies [9], in a large number of patients.
Interferon therapy was performed as an adjuvant therapy in two patients with tumor recurrencies that could not be removed completely and who had already received a full irradiation treatment. There is increasing evidence that interferon beta might be able to reduce or even stop the progression of these tumors [14, 24].
This technique was utilized by several authors, but still combined with a second transfacial approach by some [19]. LESOIN [13] used the DEROME approach for operations in the frontal and ethmoid sinus only. Meanwhile this approach has established itself for the resection of tumors localized in the nasal cavity, the maxillary sinus and the orbit as well [1, 5]. In our series we found a longterm survival of 83% with a mean follow-up of 19.3 months compared to an average longterm survival of about 40% reported in available literature [2, 8, 11, 23]. But among these survivors are five patients (41% of the whole group) who have had a tumor recurrence, which could only be removed totally in two of them. Thus the results seem to get worse with a longer follow-up. A long-term survival without any evidence of recurrence has been achieved in 41% of the patients, in three of them for more than two years. CHEESMAN [2] found the tumor related death occurred early in the postoperative course, two thirds in the first year, all due to early recurrence of the tumor. This is supported by our data with the two deaths occuring after 10 and 13 months. The question of whether tumors with later recurrencies have more benign biological behavior and thus the patients a better chance of survival must be answered by a longer follow-up of the patients. In the two children with a rhabdomyosarcoma, preoperative chemotherapy was performed follow-
Our low complication rate and lack of CSF leakages or intracranial infections proves that the closure of the skull base defect with autologous fascia and a pedicled pericranial flap alone is sufficiant [16]. The use of a free bone graft [1, 4, 20, 21] is not necessary in our view. 5 Conclusion The authors believe that the interdisciplinary neurorhinosurgical transcranial-transbasal approach to malignant tumors of the anterior cranial skull base is a safe and effective method with a low operative morbidity and a comparatively good long-term survival rate. Acknowledgements: The authors would like to thank Prof. Dr. THIERAUF(Institut fiir Pathologic der Universit/it Erlangen) for his expert diagnosis, Prof. Dr. BECK and Dr. RICHTER(Kinderklinik der Universitfit Erlangen) who took care of, and administed chemotherapy to the children included in this paper, Dr. J. HONEGGER (Neurochir. Klinik der Universit/it Erlangen) who was responsible for the interferon treatment in two adult patients, and Prof. Dr. SAUERand coworkers (Strahlentherapeutische Klinik der Universitfit Erlangen) who performed the irradiation treatment in our patients. Remark: This work has been presented in part at poster session fo the 41st Annual Meeting of the German Society for Neurosurgery, May 2 7 - 30, 1990 in Diisseldorf, Fed. Rep. of Germany (41. Jahrestagung der Deutschen Gesellschaft f/ir Neurochirurgie).
References [1] ARITAN, M SHINTARO,M SANO,T HAYAKAWA,K NAKAO, N KANAI,H MOGAMI:Surgical treatment of tumors in the anterior skull base using the transbasal approach. Neurosurgery 24 (1989) 379-384 Neurosurg. Rev. 15 (1992)
[2] CHEESMANA, VJ LUND,DJ HOWARD:Craniofacial resection for tumors of the nasal cavity and paranasal sinuses. Head & Neck Surgery 8 (1986) 429435
192
Neubauer et al., Malignant tumors of the anterior cranial skull base
[3] D~ROM~P: Les tumeurs spheno-ethmoidales. Neurochirurgie 18 (1986) suppl 1 [4] DEROtr P: Transbasal approach to tumors invading the base of the skull. In: SCHMmEKHH, WH SWEET (eds): Operative neurosurgical techniques Volt, Grune & Stratton, New York 1984 [5] FAHLBUSCHR, U NEUBAUER,ME WIGAND,M WEIDENBECHER,G R()CKELEIN, P THIERAUF,U SAUER: Neuro-rhinosurgical treatment of aesthesioneuroblastoma. Acta Neurochir (Wien) 100 (1989) 9 3 100 [6] FRAZELLEL, JS LEWIS:Cancer of the nasal cavity and accessory sinuses. Cancer 16 (1963) 1293-1301 [7] JACKSONIT, ER LAWS,RD MARTIN:A craniofacial approach to advanced recurrent cancer of the central face. Head Neck Surg 5 (1983) 474--488 [8] JACKSON IT, IR MUNRO, T HIDE: Treatment of tumors involving the anterior cranial fossa. Head Neck Surg 6 (1984) 901-913 [9] JAKUMEIT HD: Neuroblastoma of the olfactory nerve. Acta Neurochir (Wien) 25 (1971) 99-108 [10] K~TCrtAM AS, RH WILKINS, JM VANBUR~N, RR SMI~: A combined intracranial facial approach to the paranasal sinuses. Am J Surg 106 (1963) 6 9 8 703 [11] KETCHAMAS, PB CHRETIEN,JM VANBUREN:The etbmoid sinuses: a re-evaluation of surgical resection. Am J Sure 126 (1973) 469-476 [12] K~TCHAMAS, RC HOVE, JM VANBUR~N,RH JOHNSON, RR SMITH:Complications of intracranial facial resection for tumors of the paranasal sinuses. Am J Sure 112 (1966) 591-596 [13] LESOINF, CE THOMASIII, L gILLETTE:The midline supra-orbital approach, using a large single free bone flap. Acta Neurochir 87 (1987) 8 6 - 8 9 [14] MERTENSR, JH KARSTENS,J AMMON,HW CLASSEN, CH MITTERMAYER:Bisherige Erfahrungen der Interferontherapie an 7 Patienten mit Nasopharynxkarzinom. In: WANNENMACHERM (ed): Nasopharynxtumoren, Urban und Schwarzenberg, Miinchen - W i e n - Baltimore 1984 [15] OSBORNDA, P WINSTON: Carcinoma of the paranasal sinuses. J Laryng & Otol 75 (1961) 387-405 [16] PRICE JC, M LOURY, B CARSON,ME JOHNS: The pericranial flap for reconstruction of anterior skull base defects. Laryngoscope 98 (1988) 1159-1164
[17] ROSENEM, FA SIMEONE,DA BRUCE: Single stage composite resection and reconstruction of malignant anterior skull base tumors. Neurosurgery 18 (1986) 7--11 [18] Roux FX, D BRASNU,H LACCOURREYE,A FABle, JP CHODKIEWICZ: Les ad6nocarcinomes ethmoidaux op6r6s en un temps par vole transfaciale et sous-frontale apr~s chimiotherapie d'induction. Neurochirurgie 33 (1987) 365-370 [19] SAMnM, W DRAF: Neurosurgical ENT Treatment of lesions of the skull base. In: FROWEIN RA, O WILCKE, A KARIMI-NEJAD,M BROCK, M KLINGER (eds): Advances in Neurosurgery Vol 5, Springer Verlag, Berlin-Heidelberg-New York 1978 [20] SCI-mAMMV: Anterior skull base surgery for benign and malignant disease. Laryngoscope 89 (1979) 1077-1091 [21] SCHWAABG, P MaRANDAS:Les probl6mes du traitement chirurgical des tumeurs malignes de l'ethmoide par abord mixte endocranien et facial. Ann Oto-Laryng (Paris) 100 (1983) 159-161 [22] SMITH RR, CT KLOPP, JM WILLIAMS: Surgical treatment of cancer of the frontal sinus and adjacent areas. Cancer 7 (1954) 991-994 [23] TERZ JJ, HF YOtrNG, W LAWRENCE: Combined craniofacial resection for locally advanced carcinoma of the head and neck. Am J Surg 140 (1980) 613-624 [24] TR~tJNBRJ, D NIETHAMMER,G DANNECKER,R HAGMANN,V NEEF, PH ttOFSCHNEIDER:Successful treatment of nasopharyngeal carcinoma with interferon. Lancet (1980) 817- 818 [25] VAN BURENJM, AK OMMAYA,AS KETCHAM:Ten years experience with radical combined cranio-facial resection of malignant tumors of the paranasal sinuses. J Neurosurg 28 (1968) 3 4 1 - 350 [26] VOLLEE, J TREISCH,C CLAUSSEN,HJ KAUFMANN: Lesions of the skull base observed on high resolution computed tomography. Acta Radiologica 30 (1989) 129-134 Submitted October 1, 1990. Accepted April 4, 1991. Dr. Uwe Neubauer Neurochirurgische Klinik der Universit/it Erlangen-Nfirnberg Schwabachanlage 6 W-8520 Erlangen Fed. Rep. of Germany
Neurosurg. Rev. 15 (1992)
