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Skull Base Reconstruction in Cases of Intracranial Teratoma Extending into the Extracranial Structures Hajime Arai, M.D., Kiyoshi Sato, M.D., Yoshiaki Kadota, M.D., Masanori Ito, M.D., Kouichi Ishimoto, M.D., and Akira Yanai, M.D. Departments of Neurosurgery, Pediatrics, and Plastic Surgery, Juntendo University, Tokyo, Japan
Arai H, Sato K, Kadota Y, Ito M, Ishimoto K, Yanai A. Skull base reconstruction in cases of intracranial teratoma extending into the extracranial structures. Surg Neurol 1992;38:383-90.
Recently experienced were two cases of postnataUy diagnosed intracranial teratoma, one a mature teratoma and the other an immature teratoma, both extending into the extracranial structures. In each case, tumor resection was performed in which a barrier was created between the intracranial and extracranial spaces with a temporalis muscle flap. The technical aspect of skull base reconstruction is described, and previously reported cases of intracranial teratoma involving the skull base are reviewed. KEY WORDS:
Intracranial teratoma; Neonate; Skull base; Re-
construction
W h e n an intracranial t u m o r penetrating the cranial base is resected, dural and bony defects caused by the tumor's invasion should be repaired. If relatively small, the defects can be repaired by rather simple techniques such as rotation of the pericranial flap, the temporalis muscle, and/or its fascia. W h e n the skull base defects are large, however, other techniques using pedunculuted myocutaneous flaps or free muscle flaps concomitant with reconstruction o f blood circulation are required to reconstruct the defects. W e recently experienced two cases o f intracranial teratoma, one a mature teratoma in a child and the other an immature teratoma in a newborn, both extending into the extracranial structures. T u m o r excisions were p e r f o r m e d in both cases, and the skull base defects remaining were reconstructed using a temporalis muscle flap. Clinical features and the surgical technique used for skull base reconstruction are described.
Address reprint requests to: Hajime Arai, M.D., Department of Neurosurgery, Juntendo University, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113 Japan. Received April 3, 1992; accepted May 26, 1992.
© 1992 by Elsevier Science Publishing Co., Inc.
Case Reports
Case 1 A 19-day-old female baby was initially admitted to our institution due to bulging o f the left cheek associated with blepharoptosis and an abnormally enlarged head. She was born to a 28-year-old multigravida by normal vaginal delivery after an uneventful pregnancy. At admission, head circumference was 39 cm and the anterior fontanelle was slightly tense. Neurological examination revealed extraocular m o v e m e n t on the left side to be impaired except for lateral m o v e m e n t of the eye. T h e pupils were round in shape and equal in size, but the left pupil reacted sluggishly to light. T h e r e was no apparent m o t o r disturbance of the extremities. C o m p u t e d t o m o g r a p h y (CT) d e m o n s t r a t e d a large lobulated mass (3 x 4 x 4 cm) occupying the suprasellar area in association with the t u m o r extension to the anterior and middle cranial fossae on the left side (Figure 1). T h e intracranial t u m o r also was found to extend into the orbit and infratemporal fossa on the left side. Collection o f peritumoral fluid mimicking an arachnoid cyst was noted as well (Figure 1). Since C T indicated the t u m o r to have heterogeneous density with calcified foci, a tentative diagnosis of intracranial teratoma extending into the extracranial structures with skull base perforation, was made. Although serum ~-fetoprotein (AFP) and h u m a n chorionic gonadotropin ( H C G ) concentrations repeatedly were measured, their values remained within normal limits. In consideration o f the characteristic o f the t u m o r as indicated by C T and the age o f the patient, the t u m o r was clinically diagnosed, without histological verification, as a malignant teratoma. Radical t u m o r excision was thought not to be indicated because o f the p o o r prognosis in general o f malignant teratoma. T o prevent progressive enlargement of the head, the cyst associated with the t u m o r was treated by means of cystoperitoneal (CP) shunt. This was r e m o v e d a w e e k later because of infection, which was successfully treated with antibiotics, and she was discharged after a stay o f 2 months. She did 0090-3019/92/$5.00
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Figure 1. Case 1 (mature teratoma). Axial CT sections at age 1 month show that heterogeneous solid tumor localized in anterior and middle cranial fossae on left side is associated with multiple cystic lesions. Note tumor extension into left orbit and extracranial space.
very well until, at the age o f 1 year 10 months, she was readmitted because o f Streptococcus p n e u m o n i a e meningitis, which was medically cured with antibiotics. She was again hospitalized at the age o f 2 years 8 months because o f the recurrence o f meningitis due to S. sanguis and S. pneumoniae, complicated by brain abscess in the left parietal area (Figure 2). Although she suffered grave infection o f the central nervous system, her psychomotor development was not seriously compromised at age 3 years. She was able to walk alone, speak a few words, and have appropriate conversational communication
with her parents. Left vision seemed to be preserved, although blepharoptosis and impaired eye m o v e m e n t remained unchanged. Magnetic resonance imaging (MRI), which had just become available, precisely revealed extension o f the tumor, which occupied cerebral areas involving hypothalamus and frontal and temporal lobes, mainly on the left side. The extracranial portion o f the tumor was seen to occupy the orbit, paranasal sinuses, nasal cavity and infratemporal fossa on the left side, with perforation o f the anterior and middle cranial base (Figure 3). These MRI
Figure 2. Case 1. (Upper row) At 2 years 8 months, CT without enhancement, indicates brain abscess developing in left parietal region (arrowheads). (Lower row) At 2 years 10 months, CT shows that brain abscess has almost completely disappeared after 2 months of treatment.
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Figure 3. Case 1. Tl-weighted MRI. (Upper row) Coronal sections. (Lower row) Sagittal sections. Mass lesion with cysts is located in anterior and middle cranial fossae on left side. Tumor perforates the skull base and extends extracranially to left orbit, paranasal sinus, nasal cavity, and infratemporal fossa.
findings, together with the clinical results, strongly suggested that growth of the tumor was minimal or nonexistent, which contradicted our primary impression that the tumor was highly malignant. This led us to decide on tumor resection and skull base repair to eliminate communication between extracranial and intracranial spaces. After preparatory disinfection of the head, saline was infiltrated into the subcutaneous layer of the scalp to facilitate spontaneous dissection of temporoparietal fascia from the skin. A T-shaped skin incision was made in the left frontotemporoparietal area, skin flaps were turned, and the temporoparietal fascia was exposed (Figure 4). A galeal temporalis myofascial flap was first separated with superficial temporal artery intact (Figure 4). A frontotemporoparietal craniotomy was then performed, and the dura mater opened. The collected peritumoral fluid was evacuated, as much as possible of the tumor occupying the anterior and middle fossae was removed, and dissection of the tumor capsule from the frontal and temporal lobe was achieved. However, portions of the tumor intermingled with the hypothalamus and the optic nerve and chiasm, the carotid artery and its branches, and the oculomotor nerve, on the left side were not removed. T h e area of the skull base defect where the tumor extended through into the extracranial space was diffusely
covered by a thick layer of fibrous tissue. N o attempt was made to dissect this layer from the skull base. Since the exact site of communication between the extracranial and intracranial spaces could not be identified in this situation, the galeal temporalis myofascial flap previously prepared was positioned on the anterior and middle cranial bases, and the edge of the flap was sutured to the dura mater. A sufficient amount of fibrin glue was applied along the contact areas among the structures, including the edge of the flap, dura mater, and neural tissue exposed by tumor debulking, to seal the extracranial and intracranial communication. The tumor was histologically found, during surgery, to be a mature teratoma (Figure 5). N o portion of the tumor was histologically immature. Postoperatively, part of the skin flap became necrotic, and was resected and repaired with a combination of rotation skin flap and splitskin graft 4 weeks after the primary surgery. Two years have passed since the operation, during which there has been no relapse o f infection, and no residual tumor growth has been observed.
Case 2
A 4-week-old female was admitted to our institute because of right blepharoptosis which had occurred ap-
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Figure 4. Case 1, intraoperative photographs. (Left) T-shaped incision in left frontotemporoparietal area, with preserved superficial temporal artery adhering to temporalis muscle. (Right) Galeal temporalis myofascial flap with preserved superficial temporal artery (arrowheads) is used for skull base reconstruction.
proximately 1 week after birth. She was born to a 24year-old primigravida by normal vaginal delivery. Neurological examination at admission revealed that she had proptosis in association with the blepharoptosis, and impairment of extraocular movement and bulging of the face, on the right side. The serum AFP and H C G values were within normal limits. MRI of the head revealed the
presence of a heterogeneous mass in the middle and posterior cranial fossae on the right, which peforated the middle cranial base and extended extracranially into the infratemporal fossa (Figure 6). An inverted U-shaped skin incision was made in the right parietotemporal area, and the linea temporalis was exposed. A temporal craniotomy was performed, and
Figure 5. Case 1. Photomicrographs of mature teratoma. (A) Tumor containing cartilage, muscle, and adipose tissue. (B) Magnified view of ganglion. (Hematoxylin and eosin stain, original magnification × 180 in A and × 360 in B.)
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Figure 6. Case 2. Tl-weighted MRI. (Upper row) Axial sections. (Lower row) Coronal sections with gadolinium diethylenetriaminepentaacetic acid (GdDTPA). Tumor is located mainly in right middle cranial fossa and extends to infratemporal fossa through cranial base defect. Tumor consists of cystic and solid portions, the latter partly enhanced by Gd-DTPA. Note extension into posteriorfossa.
the temporalis muscle was turned without detachment from the cranial bone. The dura mater was opened, and a tumor soft in consistency and heterogeneous in nature was resected. It was not possible to resect a small portion of the tumor extending into the posterior fossa on the right side because the tumor intermingled with cranial nerves coursing in the paratentorial cistern and the region o f the cerebellopontine angle. The bone and dural defects were obliterated with a temporalis muscle-bone flap. T o effect two-layer repair, however, a portion of the reflected temporal bone attached to the temporalis muscle was fashioned to reverse the convexity and used to obliterate the skull base defect. The temporalis muscle thus covering the defect was sutured to the dura mater, and sufficient fibrin glue was applied to the area of attachment of muscle and dura. Histological examination of the tumor revealed it to be an immature teratoma (Figure 7). T h e postoperative course was uneventful, and there was neither intracranial infection nor spinal fluid leakage. Combination therapy involving intravenous administration o f cisplatin, vinblastine, and bleomycin was undertaken. A C T scan 2 weeks after surgery revealed no change in the size of the tumor left in the posterior fossa, but an abrupt growth
of tumor was noted thereafter, and the patient died approximately 7 weeks after surgery (Figure 8).
Discussion Teratoma accounts for 9 % of intracranial tumors in infants [6] and as many as 51% of brain tumors in neonates [24]. In general, the reported prognosis for neonatal intracranial teratoma has been poor [3,4,7,8,13,19, 28,29]. The 1-year survival rate for such patients was reported as 7.2% in a review o f 200 neonatal brain tumors by Wakai et al [29]. This is low in comparison with the 1-year survival rates for other types o f neonatal brain tumors [20]. The neonatal teratoma with both intracranial and extracranial involvement reported here has rarely been reported, and only 16 cases have been found in the English and G e r m a n literatures (Table 1) [ 2 , 5 , 1 4 , 1 6 18,21,22,27,30,31]. Furthermore, there were no primary teratomas o f the neck with intracranial extension among these 16 cases. T h r e e of the 16 cases were considered to be pharyngeal teratomas (epignathus) with intracranial extension [2,5,14]. T h e remaining 13 cases and the two cases reported here may be categorized as intra-
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Figure 7. Case 2, Photomicrographs of immature teratoma. (A) Tumor containing cartilage, bone, and clusters of small immature cells. (B) Magnified view of neuroepithelial rosette. (Hematoxylin and eosin stain, original magnification × 180 in A and ×360 in B.)
cranial teratoma with extracranial extension, even though the origin o f the tumor is uncertain in some cases. The prognosis for such teratoma was poor because 11 o f the 16 cases were either stillborn or died immediately after birth. T h e case reported by Nanda et al [18], in which the patient survived for 10 years after radical resection o f the tumor, is noteworthy. T h e sporadic reports on the outcome o f surgical treatment o f neonatal teratoma are by no means favorable [9,11,15,23-26,30]. H o w e v e r , long-term survival can be expected following surgical therapy of histologically defined mature teratoma [18,25]. From this point of view, the good progress made by our case 1 can be considered rare, whereas case 2 can be said to be typical.
The difference in clinical progress o f the two cases appears to be attributable to their histopathological differences, namely, mature teratoma and immature teratoma. Neuroradiological study, including MRI, however, is incapable o f differentiating one from the other. It seems important that a policy o f treating neonatal intracranial teratoma should include as much resection o f the tumor as possible and the establishment o f a histopathological diagnosis. Skull base reconstruction is mandatory in connection with the surgical treatment o f intracranial teratoma perforating the cranial base. Reconstruction o f the middle cranial base is achieved by using (1) the temporalis muscle flap, (2) pedunculated myocutaneous flap using the
Figure 8. Case 2. CT 4 weeks after operation. There was rapid growth of tumor remaining in posteriorfossa, but no regrowth of tumor in right middle fossa.
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T a b l e 1. Congenital Teratoma with Both Intracranial and Extracranial Involvement
Source
Gestation age at delivery (wk), sex
Breslau & Rindleisch [2] Kraus [14]
24, F NA, F
Ehrich [5]
20, F
Whittle & Simpson [30]
37, M
Vinters et al [27]
31, M
Lipman et al [16]
36.5, N A
22, M
Mamalis et al [17]
28, M
Yang et al [31]
40, F
Rostad et al [21]
37, M
21, M
Rueda-Pedraza et al [22]
42, M 28, M
Location Pituitary fossa, mouth Pituitary fossa, pharynx Filling the cranium, nasal cavity, mouth, pharynx Anterior & middle cranial fossae, It orbit Filling the cranium, orbit Rt middle & posterior fossae, neck, mandible Filling the cranium, It orbit, oral cavity, It hypopharynx Filling the cranium, It orbit Lt middle cranial fossa, It pharyngeal space Rt middle cranial fossa, rt mandible, rt neck Filling the cranium, It orbit, mouth, pharynx Cranioethmoid
40, F
Craniopharyngeal, intraoral Orbitocerebral
40, F
Orbitocerebral
Nanda et al [18]
40, M
Present report
40, F
Lt middle fossa, It orbit Lt middle cranial & anterior fossae, paranasal sinus, nasal cavity, It orbit, infratemporal fossa Rt middle & posterior fossae, infratemporal fossa
40, F
Histology
Operation
Outcome
Mature
None
Stillborn
Immature
None
Stillborn
Mature
None
Stillborn
Mature & immature
Subtotal resection (2 mo of age)
Died 8 mo after operation
Mature & immature Immature
None
Stillborn
None
Died 3 min after birth
Immature
None
Died 5 min after birth
Immature
None
Stillborn
Mature
Almost total removal
NA
Immature
None
Died a few min after birth
Immature
None
Stillborn
Mature
None
Stillborn
Mature & immature Mature & immature Mature & immature Mature
None
Died 1 d after birth
None
Died 12 d after birth
None
Died 69 d after birth
Total resection
Survived 10 yr
Mature
Partial removal & skull base reconstruction
Survived 5 yr
Immature
Subtotal removal & skull base reconstruction
Died 2 mo after birth
Abbreviations: F, female; NA, not available; M, male; It, left; rt, right.
trapezius or latissimus dorsi muscle, or (3) microvascular free flap of the rectus abdominis muscle where the nutrient artery and vein are anastomosed to the external carotid system [1,12]. T h e first is a very simple and useful method, but its indication is limited to cases in which bony and dural defects are relatively localized.
Furthermore, the use o f the temporalis muscle to obliterate a skull base defect should be restricted in pediatric cases because transposition o f masticatory muscles may have a deleterious effect on subsequent craniofacial growth [10]. T h e latter two methods are indicated in cases where bony and dural defects involve large areas.
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T h e surgical t e c h n i q u e is so e x t e n s i v e that it is n o t suitable for n e o n a t e s a n d infants. I n surgical r e s e c t i o n o f a t u m o r e x t e n d i n g f r o m the m i d d l e cranial fossa to the o u t s i d e o f the skull, a c o m b i n a t i o n o f transcranial a n d i n f r a t e m p o r a l approach is indicated. T h e skull base defect r e m a i n i n g after t u m o r resection should be r e c o n s t r u c t e d with p e d u n c u l a t e d m y o c u t a n e o u s flaps o r m i c r o v a s c u l a r free flaps as o u t l i n e d above. Such e x t e n s i v e and invasive p r o c e d u r e s c a n n o t be u n d e r t a k e n in the case o f n e o n a t e s and infants. For this reason, we chose the t e m p o r a l i s m u s c l e flap to close the skull base defect in b o t h cases. I n case 1, d e v e l o p m e n t o f the t e m p o r a l i s m u s c l e was especially p o o r d u e to o u t w a r d b u l g i n g o f the t e m p o r a l b o n e secondary to e x t e n s i v e t u m o r g r o w t h i n t o the intracranial and extracranial spaces o n the left side. C o n s e q u e n t l y , we m o d i fied the so-called t e m p o r a l i s m u s c l e flap to achieve g r e a t e r r e c o n s t r u c t i o n o f the skull base using p o o r l y d e v e l o p e d t e m p o r a l i s muscle. A galeal t e m p o r a l i s m y o fascial flap with p r e s e r v e d superficial t e m p o r a l artery was p r e p a r e d a n d used for skull base r e c o n s t r u c t i o n . I n case 2, a t e m p o r a l i s m u s c l e - b o n e flap was used to skull base r e c o n s t r u c t i o n , and two-layer repair o f the skull base defect was achieved. B e c a u s e t h e r e are few reports o f n e o n a t a l and infantile skull base t u m o r s i n v o l v i n g skull base p e n e t r a t i o n , f u r t h e r i n v e s t i g a t i o n o f the technical aspects o f skull base repair is r e q u i r e d .
References 1. Anan VK. Reconstruction in cranial-base surgery In: AI-Mefty O, ed. Surgery of the skull base. Boston: Kluwer Academic, 1989:297-314. 2. Breslau B, Rindfleisch E. Geburtsgeschichte und Untersuchung eines Falles von Foetus in Foetus. Virchows Arch Pathol Anat 1864;30:406-17. 3. Chen HP. Intracranial teratoma of a newborn, report ofan unusual case. J Neuropathol Exp Neurol 1958;17:599-603. 4. Dolkart LA, Balcom RJ, Eisinger G. Intracranial teratoma: prolonged neonatal survival after prenatal diagnosis. Am J Obstet Gynecol 1990;162:768-9. 5. Ehrich WE. Teratoid parasites of the mouth [episphenoids, epipalati (epurani), epiganathi]. Am J Orthodont 1945;31:650-9. 6. FarwellJR, Dohrmann GJ, FlanneryJT. Intracranial neoplasms in infants. Arch Neurol 1978;35:533-7. 7. Finck FM, Antin R. Intracranial teratoma of the newborn. Am J Dis Child 1965;109:439-42. 8. Greenhouse AH, Neubuerger KT. Intracranial teratoma of the newborn. Arch Neurol 1960;3:718-24.
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9. Hirsch LF, Rorke LB, Schmidek HH. Unusual cause of relapsing hydrocephalus. Arch Neurol 1977;34:505-8. 10. Hohl TH. Masticatory muscle transposition in primates: effects on craniofacial growth. J Maxillofacial Surg 1983;11:149-56. 11. Ingraham FD, Baily OT. Cystic teratoma and teratoid tumors of the central nervous system in infancy and childhood. J Neuosurg 1946;3:511-32. 12. Jones NF. Methods of cranial base reconstruction. In: Sekhar LN, Schram VL, eds. Tumors of the cranial base: diagnosis and treatment. Mount Kisco: Futura, 1987:233-43. 13. Jooma R, Kendall BE, Hayward RD. Intracranial tumors in neonate: a report of seventeen cases. Surg Neurol 1984;21:165-70. 14. Kraus EJ. lJber ein epignathisches Teratom der Hypophysengegend. Zugleich ein Beitrag zur Frage der Entstehung der Nebennierenveriinderung bei der Anenzephalie. Virchows Arch Pathol Anat 1929;271:546-55. 15. Lehman RA, Torres-Reyes E. Cystic inracranial teratoma in an infant. J Neurosurg 1970;33:334-8. 16. Lipman SP, Pretorius DH, Rumack CM, Manco-Johnson ML. Fetal intracranial teratoma: US diagnosis of three cases and review of the literature. Radiology 1985;157:491-4. 17. Mamalis N, Garland PE, Agel JC, Apple DJ. Congenital orbital teratoma: a review and report of two cases. Surv Ophthalmol 1985;30:41-6. 18. Nanda A, Schut L, Sutton LN. Congenital forms of intracranial teratoma. Childs Nerv Syst 1991;7:112-4. 19. Oberman B. Intracranial teratoma replacing brain. Arch Neurol 1964;11:423-6. 20. Oi S, Kokunai T, Matsumoto S. Congenital brain tumors in Japan (ISPN cooperative study): specific clinical features in neonates. Childs Nerv Syst 1990;6:86-91. 21. Rostad SBK, DeMasters K, Manchester DK. Two massive congenital intracranial immature teratoma with neck extension. Teratology 1985;32:163-9. 22. Rueda-Pedraza ME, Heifetz SA, Sesterhenn IA, Clark GB. Primary intracranial germ cell tumor in the first two decades of life. Perspect Pediatr Pathol 1987; 10:160-207. 23. Sato O, Tamura A, Sekino H, Sano K. Brain tumor of early infants No To Shinkei 1973;25:1447-53. 24. Takaku A, Kodama N, Ohara H, Hori S. Brain tumor in newborn babies. Childs Brain 1978;4:365-75. 25. Takaku A, Mita R, SuzukiJ. Intracranial teratoma in early infancy. J Neurosurg 1973;38:266-8. 26. Uken P, Sato Y, Smith W. MR finding of malignant intracranial teratoma in a neonate. Pediatr Radiol 1986;16:504-5. 27. Vinters HV, Murphy J, Wittman B, Norman MG. Intracranial teratoma: antenatal diagnosis at 31 weeks gestation by ultrasound. Acta Neuropathol (Berl) 1982;58:233-6. 28. Vraa-Jensen J. Massive congenital intracranial teratoma. Acta Neuropathol 1974;30:271-6. 29. Wakai S, Arai T, Nagal M. Congenital brain tumors. Surg Neurol 1984;21:597-609. 30. Whittle IR, Simpson DA. Surgical treatment for neonatal intracranial teratoma. Surg Neurol 1981;15:268-73. 31. Yang PJ, Graham AR, Carmody RF, Seeger JF, Capp MP. Intracranial mass in a neonate. Invest Radiol 1986;21:360-4.
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Skull Base Reconstruction in Cases of Intracranial Teratoma Extending into the Extracranial Structures Hajime Arai, M.D., Kiyoshi Sato, M.D., Yoshiaki Kadota, M.D., Masanori Ito, M.D., Kouichi Ishimoto, M.D., and Akira Yanai, M.D. Departments of Neurosurgery, Pediatrics, and Plastic Surgery, Juntendo University, Tokyo, Japan
Arai H, Sato K, Kadota Y, Ito M, Ishimoto K, Yanai A. Skull base reconstruction in cases of intracranial teratoma extending into the extracranial structures. Surg Neurol 1992;38:383-90.
Recently experienced were two cases of postnataUy diagnosed intracranial teratoma, one a mature teratoma and the other an immature teratoma, both extending into the extracranial structures. In each case, tumor resection was performed in which a barrier was created between the intracranial and extracranial spaces with a temporalis muscle flap. The technical aspect of skull base reconstruction is described, and previously reported cases of intracranial teratoma involving the skull base are reviewed. KEY WORDS:
Intracranial teratoma; Neonate; Skull base; Re-
construction
W h e n an intracranial t u m o r penetrating the cranial base is resected, dural and bony defects caused by the tumor's invasion should be repaired. If relatively small, the defects can be repaired by rather simple techniques such as rotation of the pericranial flap, the temporalis muscle, and/or its fascia. W h e n the skull base defects are large, however, other techniques using pedunculuted myocutaneous flaps or free muscle flaps concomitant with reconstruction o f blood circulation are required to reconstruct the defects. W e recently experienced two cases o f intracranial teratoma, one a mature teratoma in a child and the other an immature teratoma in a newborn, both extending into the extracranial structures. T u m o r excisions were p e r f o r m e d in both cases, and the skull base defects remaining were reconstructed using a temporalis muscle flap. Clinical features and the surgical technique used for skull base reconstruction are described.
Address reprint requests to: Hajime Arai, M.D., Department of Neurosurgery, Juntendo University, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113 Japan. Received April 3, 1992; accepted May 26, 1992.
© 1992 by Elsevier Science Publishing Co., Inc.
Case Reports
Case 1 A 19-day-old female baby was initially admitted to our institution due to bulging o f the left cheek associated with blepharoptosis and an abnormally enlarged head. She was born to a 28-year-old multigravida by normal vaginal delivery after an uneventful pregnancy. At admission, head circumference was 39 cm and the anterior fontanelle was slightly tense. Neurological examination revealed extraocular m o v e m e n t on the left side to be impaired except for lateral m o v e m e n t of the eye. T h e pupils were round in shape and equal in size, but the left pupil reacted sluggishly to light. T h e r e was no apparent m o t o r disturbance of the extremities. C o m p u t e d t o m o g r a p h y (CT) d e m o n s t r a t e d a large lobulated mass (3 x 4 x 4 cm) occupying the suprasellar area in association with the t u m o r extension to the anterior and middle cranial fossae on the left side (Figure 1). T h e intracranial t u m o r also was found to extend into the orbit and infratemporal fossa on the left side. Collection o f peritumoral fluid mimicking an arachnoid cyst was noted as well (Figure 1). Since C T indicated the t u m o r to have heterogeneous density with calcified foci, a tentative diagnosis of intracranial teratoma extending into the extracranial structures with skull base perforation, was made. Although serum ~-fetoprotein (AFP) and h u m a n chorionic gonadotropin ( H C G ) concentrations repeatedly were measured, their values remained within normal limits. In consideration o f the characteristic o f the t u m o r as indicated by C T and the age o f the patient, the t u m o r was clinically diagnosed, without histological verification, as a malignant teratoma. Radical t u m o r excision was thought not to be indicated because o f the p o o r prognosis in general o f malignant teratoma. T o prevent progressive enlargement of the head, the cyst associated with the t u m o r was treated by means of cystoperitoneal (CP) shunt. This was r e m o v e d a w e e k later because of infection, which was successfully treated with antibiotics, and she was discharged after a stay o f 2 months. She did 0090-3019/92/$5.00
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Figure 1. Case 1 (mature teratoma). Axial CT sections at age 1 month show that heterogeneous solid tumor localized in anterior and middle cranial fossae on left side is associated with multiple cystic lesions. Note tumor extension into left orbit and extracranial space.
very well until, at the age o f 1 year 10 months, she was readmitted because o f Streptococcus p n e u m o n i a e meningitis, which was medically cured with antibiotics. She was again hospitalized at the age o f 2 years 8 months because o f the recurrence o f meningitis due to S. sanguis and S. pneumoniae, complicated by brain abscess in the left parietal area (Figure 2). Although she suffered grave infection o f the central nervous system, her psychomotor development was not seriously compromised at age 3 years. She was able to walk alone, speak a few words, and have appropriate conversational communication
with her parents. Left vision seemed to be preserved, although blepharoptosis and impaired eye m o v e m e n t remained unchanged. Magnetic resonance imaging (MRI), which had just become available, precisely revealed extension o f the tumor, which occupied cerebral areas involving hypothalamus and frontal and temporal lobes, mainly on the left side. The extracranial portion o f the tumor was seen to occupy the orbit, paranasal sinuses, nasal cavity and infratemporal fossa on the left side, with perforation o f the anterior and middle cranial base (Figure 3). These MRI
Figure 2. Case 1. (Upper row) At 2 years 8 months, CT without enhancement, indicates brain abscess developing in left parietal region (arrowheads). (Lower row) At 2 years 10 months, CT shows that brain abscess has almost completely disappeared after 2 months of treatment.
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Figure 3. Case 1. Tl-weighted MRI. (Upper row) Coronal sections. (Lower row) Sagittal sections. Mass lesion with cysts is located in anterior and middle cranial fossae on left side. Tumor perforates the skull base and extends extracranially to left orbit, paranasal sinus, nasal cavity, and infratemporal fossa.
findings, together with the clinical results, strongly suggested that growth of the tumor was minimal or nonexistent, which contradicted our primary impression that the tumor was highly malignant. This led us to decide on tumor resection and skull base repair to eliminate communication between extracranial and intracranial spaces. After preparatory disinfection of the head, saline was infiltrated into the subcutaneous layer of the scalp to facilitate spontaneous dissection of temporoparietal fascia from the skin. A T-shaped skin incision was made in the left frontotemporoparietal area, skin flaps were turned, and the temporoparietal fascia was exposed (Figure 4). A galeal temporalis myofascial flap was first separated with superficial temporal artery intact (Figure 4). A frontotemporoparietal craniotomy was then performed, and the dura mater opened. The collected peritumoral fluid was evacuated, as much as possible of the tumor occupying the anterior and middle fossae was removed, and dissection of the tumor capsule from the frontal and temporal lobe was achieved. However, portions of the tumor intermingled with the hypothalamus and the optic nerve and chiasm, the carotid artery and its branches, and the oculomotor nerve, on the left side were not removed. T h e area of the skull base defect where the tumor extended through into the extracranial space was diffusely
covered by a thick layer of fibrous tissue. N o attempt was made to dissect this layer from the skull base. Since the exact site of communication between the extracranial and intracranial spaces could not be identified in this situation, the galeal temporalis myofascial flap previously prepared was positioned on the anterior and middle cranial bases, and the edge of the flap was sutured to the dura mater. A sufficient amount of fibrin glue was applied along the contact areas among the structures, including the edge of the flap, dura mater, and neural tissue exposed by tumor debulking, to seal the extracranial and intracranial communication. The tumor was histologically found, during surgery, to be a mature teratoma (Figure 5). N o portion of the tumor was histologically immature. Postoperatively, part of the skin flap became necrotic, and was resected and repaired with a combination of rotation skin flap and splitskin graft 4 weeks after the primary surgery. Two years have passed since the operation, during which there has been no relapse o f infection, and no residual tumor growth has been observed.
Case 2
A 4-week-old female was admitted to our institute because of right blepharoptosis which had occurred ap-
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Figure 4. Case 1, intraoperative photographs. (Left) T-shaped incision in left frontotemporoparietal area, with preserved superficial temporal artery adhering to temporalis muscle. (Right) Galeal temporalis myofascial flap with preserved superficial temporal artery (arrowheads) is used for skull base reconstruction.
proximately 1 week after birth. She was born to a 24year-old primigravida by normal vaginal delivery. Neurological examination at admission revealed that she had proptosis in association with the blepharoptosis, and impairment of extraocular movement and bulging of the face, on the right side. The serum AFP and H C G values were within normal limits. MRI of the head revealed the
presence of a heterogeneous mass in the middle and posterior cranial fossae on the right, which peforated the middle cranial base and extended extracranially into the infratemporal fossa (Figure 6). An inverted U-shaped skin incision was made in the right parietotemporal area, and the linea temporalis was exposed. A temporal craniotomy was performed, and
Figure 5. Case 1. Photomicrographs of mature teratoma. (A) Tumor containing cartilage, muscle, and adipose tissue. (B) Magnified view of ganglion. (Hematoxylin and eosin stain, original magnification × 180 in A and × 360 in B.)
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Figure 6. Case 2. Tl-weighted MRI. (Upper row) Axial sections. (Lower row) Coronal sections with gadolinium diethylenetriaminepentaacetic acid (GdDTPA). Tumor is located mainly in right middle cranial fossa and extends to infratemporal fossa through cranial base defect. Tumor consists of cystic and solid portions, the latter partly enhanced by Gd-DTPA. Note extension into posteriorfossa.
the temporalis muscle was turned without detachment from the cranial bone. The dura mater was opened, and a tumor soft in consistency and heterogeneous in nature was resected. It was not possible to resect a small portion of the tumor extending into the posterior fossa on the right side because the tumor intermingled with cranial nerves coursing in the paratentorial cistern and the region o f the cerebellopontine angle. The bone and dural defects were obliterated with a temporalis muscle-bone flap. T o effect two-layer repair, however, a portion of the reflected temporal bone attached to the temporalis muscle was fashioned to reverse the convexity and used to obliterate the skull base defect. The temporalis muscle thus covering the defect was sutured to the dura mater, and sufficient fibrin glue was applied to the area of attachment of muscle and dura. Histological examination of the tumor revealed it to be an immature teratoma (Figure 7). T h e postoperative course was uneventful, and there was neither intracranial infection nor spinal fluid leakage. Combination therapy involving intravenous administration o f cisplatin, vinblastine, and bleomycin was undertaken. A C T scan 2 weeks after surgery revealed no change in the size of the tumor left in the posterior fossa, but an abrupt growth
of tumor was noted thereafter, and the patient died approximately 7 weeks after surgery (Figure 8).
Discussion Teratoma accounts for 9 % of intracranial tumors in infants [6] and as many as 51% of brain tumors in neonates [24]. In general, the reported prognosis for neonatal intracranial teratoma has been poor [3,4,7,8,13,19, 28,29]. The 1-year survival rate for such patients was reported as 7.2% in a review o f 200 neonatal brain tumors by Wakai et al [29]. This is low in comparison with the 1-year survival rates for other types o f neonatal brain tumors [20]. The neonatal teratoma with both intracranial and extracranial involvement reported here has rarely been reported, and only 16 cases have been found in the English and G e r m a n literatures (Table 1) [ 2 , 5 , 1 4 , 1 6 18,21,22,27,30,31]. Furthermore, there were no primary teratomas o f the neck with intracranial extension among these 16 cases. T h r e e of the 16 cases were considered to be pharyngeal teratomas (epignathus) with intracranial extension [2,5,14]. T h e remaining 13 cases and the two cases reported here may be categorized as intra-
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Figure 7. Case 2, Photomicrographs of immature teratoma. (A) Tumor containing cartilage, bone, and clusters of small immature cells. (B) Magnified view of neuroepithelial rosette. (Hematoxylin and eosin stain, original magnification × 180 in A and ×360 in B.)
cranial teratoma with extracranial extension, even though the origin o f the tumor is uncertain in some cases. The prognosis for such teratoma was poor because 11 o f the 16 cases were either stillborn or died immediately after birth. T h e case reported by Nanda et al [18], in which the patient survived for 10 years after radical resection o f the tumor, is noteworthy. T h e sporadic reports on the outcome o f surgical treatment o f neonatal teratoma are by no means favorable [9,11,15,23-26,30]. H o w e v e r , long-term survival can be expected following surgical therapy of histologically defined mature teratoma [18,25]. From this point of view, the good progress made by our case 1 can be considered rare, whereas case 2 can be said to be typical.
The difference in clinical progress o f the two cases appears to be attributable to their histopathological differences, namely, mature teratoma and immature teratoma. Neuroradiological study, including MRI, however, is incapable o f differentiating one from the other. It seems important that a policy o f treating neonatal intracranial teratoma should include as much resection o f the tumor as possible and the establishment o f a histopathological diagnosis. Skull base reconstruction is mandatory in connection with the surgical treatment o f intracranial teratoma perforating the cranial base. Reconstruction o f the middle cranial base is achieved by using (1) the temporalis muscle flap, (2) pedunculated myocutaneous flap using the
Figure 8. Case 2. CT 4 weeks after operation. There was rapid growth of tumor remaining in posteriorfossa, but no regrowth of tumor in right middle fossa.
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T a b l e 1. Congenital Teratoma with Both Intracranial and Extracranial Involvement
Source
Gestation age at delivery (wk), sex
Breslau & Rindleisch [2] Kraus [14]
24, F NA, F
Ehrich [5]
20, F
Whittle & Simpson [30]
37, M
Vinters et al [27]
31, M
Lipman et al [16]
36.5, N A
22, M
Mamalis et al [17]
28, M
Yang et al [31]
40, F
Rostad et al [21]
37, M
21, M
Rueda-Pedraza et al [22]
42, M 28, M
Location Pituitary fossa, mouth Pituitary fossa, pharynx Filling the cranium, nasal cavity, mouth, pharynx Anterior & middle cranial fossae, It orbit Filling the cranium, orbit Rt middle & posterior fossae, neck, mandible Filling the cranium, It orbit, oral cavity, It hypopharynx Filling the cranium, It orbit Lt middle cranial fossa, It pharyngeal space Rt middle cranial fossa, rt mandible, rt neck Filling the cranium, It orbit, mouth, pharynx Cranioethmoid
40, F
Craniopharyngeal, intraoral Orbitocerebral
40, F
Orbitocerebral
Nanda et al [18]
40, M
Present report
40, F
Lt middle fossa, It orbit Lt middle cranial & anterior fossae, paranasal sinus, nasal cavity, It orbit, infratemporal fossa Rt middle & posterior fossae, infratemporal fossa
40, F
Histology
Operation
Outcome
Mature
None
Stillborn
Immature
None
Stillborn
Mature
None
Stillborn
Mature & immature
Subtotal resection (2 mo of age)
Died 8 mo after operation
Mature & immature Immature
None
Stillborn
None
Died 3 min after birth
Immature
None
Died 5 min after birth
Immature
None
Stillborn
Mature
Almost total removal
NA
Immature
None
Died a few min after birth
Immature
None
Stillborn
Mature
None
Stillborn
Mature & immature Mature & immature Mature & immature Mature
None
Died 1 d after birth
None
Died 12 d after birth
None
Died 69 d after birth
Total resection
Survived 10 yr
Mature
Partial removal & skull base reconstruction
Survived 5 yr
Immature
Subtotal removal & skull base reconstruction
Died 2 mo after birth
Abbreviations: F, female; NA, not available; M, male; It, left; rt, right.
trapezius or latissimus dorsi muscle, or (3) microvascular free flap of the rectus abdominis muscle where the nutrient artery and vein are anastomosed to the external carotid system [1,12]. T h e first is a very simple and useful method, but its indication is limited to cases in which bony and dural defects are relatively localized.
Furthermore, the use o f the temporalis muscle to obliterate a skull base defect should be restricted in pediatric cases because transposition o f masticatory muscles may have a deleterious effect on subsequent craniofacial growth [10]. T h e latter two methods are indicated in cases where bony and dural defects involve large areas.
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T h e surgical t e c h n i q u e is so e x t e n s i v e that it is n o t suitable for n e o n a t e s a n d infants. I n surgical r e s e c t i o n o f a t u m o r e x t e n d i n g f r o m the m i d d l e cranial fossa to the o u t s i d e o f the skull, a c o m b i n a t i o n o f transcranial a n d i n f r a t e m p o r a l approach is indicated. T h e skull base defect r e m a i n i n g after t u m o r resection should be r e c o n s t r u c t e d with p e d u n c u l a t e d m y o c u t a n e o u s flaps o r m i c r o v a s c u l a r free flaps as o u t l i n e d above. Such e x t e n s i v e and invasive p r o c e d u r e s c a n n o t be u n d e r t a k e n in the case o f n e o n a t e s and infants. For this reason, we chose the t e m p o r a l i s m u s c l e flap to close the skull base defect in b o t h cases. I n case 1, d e v e l o p m e n t o f the t e m p o r a l i s m u s c l e was especially p o o r d u e to o u t w a r d b u l g i n g o f the t e m p o r a l b o n e secondary to e x t e n s i v e t u m o r g r o w t h i n t o the intracranial and extracranial spaces o n the left side. C o n s e q u e n t l y , we m o d i fied the so-called t e m p o r a l i s m u s c l e flap to achieve g r e a t e r r e c o n s t r u c t i o n o f the skull base using p o o r l y d e v e l o p e d t e m p o r a l i s muscle. A galeal t e m p o r a l i s m y o fascial flap with p r e s e r v e d superficial t e m p o r a l artery was p r e p a r e d a n d used for skull base r e c o n s t r u c t i o n . I n case 2, a t e m p o r a l i s m u s c l e - b o n e flap was used to skull base r e c o n s t r u c t i o n , and two-layer repair o f the skull base defect was achieved. B e c a u s e t h e r e are few reports o f n e o n a t a l and infantile skull base t u m o r s i n v o l v i n g skull base p e n e t r a t i o n , f u r t h e r i n v e s t i g a t i o n o f the technical aspects o f skull base repair is r e q u i r e d .
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