J Neurosurg 49:460-463, 1978
Intracranial solitary chondroma Case report
JOHN DUTTON, M.B., B.S., F.R.C.S.
University Department of Neurosurgery, Manchester Royal Infirmary, Manchester, England v' A case of massive solitary intracranial chondroma involvingthe base of the skull in a 15-month-old baby boy was successfullytreated by total excision. The diagnosis was greatly simplified by the use of computerized tomography, which materially helped in planning the surgical approach. KEy WORDS
skull neoplasm
9 chondroma 9 intracranial chondroma 9 computerized tomography
B
Y 1938, 11 cases of solitary intracranial chondroma had been recorded at autopsy2a and 16 at surgery. 6,7 It appears that only 58 tumors have been operated on, and ll found at autopsy. The history varies greatly according to the site of the tumor and its rate of growth; the course varies from 4 months to 7 years and in many cases is indistinguishable from that of a meningioma. In the case of convexity tumors, there has been epilepsy of focal type, a progressive hemiparesis, and later signs of raised intracranial pressure. Only three cases have shown an endostosis, and calcification on x-ray study is rare, accounting for the density which may sometimes be found by careful exposure or tomographic cuts through suspect areas. Case Report
This 15-month-old baby boy was brought in for evaluation of minimal left-sided proptosis and sixth nerve paralysis. Examination. He had no other abnormalities. X-ray films revealed expansion of 460
the left middle fossa. The enormous extent of the space-occupying lesion was shown on computerized tomography (CT) which revealed that the mass occupied the entire middle fossa on the left (Fig. 1 left and center) and extended one-third of the way across the right sphenoidal wing (Fig. 2). The density without contrast indicated areas of calcium. Carotid angiography displayed occlusion of the left internal carotid artery and confirmed massive brain shift to the right side. Operation. Staged removal resulted in complete excision with only transient thirdnerve paralysis (Fig. 1 right).
Pathological Examination The tumor capsule was composed of a collagenous membrane consistent with dura and deep to this was collagenous tissue containing areas of maturing cartilage, presumably the perichondrium. Much of the tumor was composed of well differentiated cartilage of the hyaline variety, developing from spindle-shaped fibroblastic tissue 3". Neurosurg. / Volume 49 / September, 1978
Intracranial solitary chondroma
FIG. 1. Left and Center: Computerized tomography scans reveal expansion of the middle fossa on the left side with forward displacement of the sphenoidal wing, resulting from a large, high-density lesion, extending from the middle fossa high into the parietal region, displacing the lateral and third ventricles to the right and extending one third of the way across the right sphenoidal wing. Right: Final appearance after total removal, showing that the midline structures had returned toward the center but there was still some shift to the right of the lateral and third ventricles with a surface fluid collection.
through immature cartilage to mature hyaline cartilage (Fig. 3). There were no mitotic figures and no multinucleate cells. No elastic fibers were demonstrated and no groups of stellate cells with vacuoles to suggest a chordomatous element were seen. Postoperative Course. The patient's recovery was satisfactory and a subsequent psychological report, using the Griffiths mental development scale, gave the developmental quotient as within normal limits, with no significant retardation. A later carotid angiogram still revealed no functional internal carotid artery arising from the left common carotid artery. The brain now appeared more correctly situated and CT scans showed that the midline structures had returned toward the center but that there was still some shift to the right of the lateral and third ventricles.
cribrosa, but truly intracranial neoplasms composed of cartilage usually present at four main sites. Their combined numbers are still relatively small and, although only about 60 cases have been submitted to surgery, it
Discussion Excluding those patients with more or less generalized chondromatosis, where occasional cases present with intracranial localization, TM the fnding of an intracranial chondroma is extremely rare. Those arising in the nasal septum and sinuses can penetrate through the skull, 1,17 especially the lamina
J. Neurosurg. / Volume 49 / September, 1978
FIG. 2. Artist's impression of operation findings. The composite picture was constructed from the full tomographic survey. 46]
J. Dutton
FIG. 3. Photomicrograph showing the tumor, composed of well differentiated cartilage of the hyaline variety, developing from spindle-shaped fibroblastic tissue through immature cartilage to mature hyaline cartilage. H & E, • 70.
appears that their incidence is showing an upward trend. Tumors arising in the dural partitions, 1~ the leptomeninges, 3,9,~,~6 or the choroid plexus 18 are slightly less common than those arising from the base of the skull. 4,8,u,~3,~,2u~7 Embryologically, the basal bones arise from cartilaginous matrices and these chondromas presumably arise from residua of the primordial cartilaginous cranium. The vault, in contrast, arises from membranous bone. Basal chondromas arise mainly from the region of the foramen lacerum or more precisely the synchondroses that converge upon it. ~ These tumors arise from embryonic residues where the spheno-petrosal, petrooccipital, and spheno-occipital synchondroses meet like a star. They lie extradurally, the majority being parasellar in the middle fossa? ~ Less commonly, they occur in the posterior fossa behind the clivus, or may extend into the cerebellopontine angle. ~,5'1~'~5'2~ Then again, they may occupy both areas simultaneously. As in our case, a parasellar chondroma may grow around and obstruct the internal carotid artery, which represents the third aortic arch. The fifth ganglion may be involved in the tumor and the third and sixth nerves displaced. They destroy the bone diffusely with no reparatory new bone formation, thus the sella or the tip of the petrous bone may be eroded. Such tumors may extend upward high above the sella, or 462
predominantly downward toward the nasopharynx. Chondromas arise by budding of the embryonal cartilaginous rests left in place in the base of the skull or misplaced into neighboring tissues, or, on the other hand, by development from persistent and aberrant foci of meningeal mesenchyme or from leptomeningeal fibroblasts. These tumors are usually hard, but may be soft, elastic, lobulated, rounded, and encapsulated, and frequently reach a large size. They have a milky, opaline surface, typical of cartilage. They may appear to have calcific deposits and yellow mucinous areas with cystic cavitation. They are extradural but ill-defined in relation to bone. Basal chondromas show a preponderance in females. 17 The patient age span is usually between 16 and 54 years 8 in cases not part of generalized chondromatosis. The youngest patient previously reported was 4 years old. .9 Although these tumors are invariably benign, in the past the long-term prognosis has been viewed with caution. At one time surgery was associated with a mortality rate approaching 50% and a high incidence of recurrence in the survivors. The main problem, especially with the parasellar tumors, is their situation, making total removal difficult, and it would appear that x-ray therapy is valueless in such cases. Total removal, which can be achieved much more simply with the so-called convexity tumors, especially with early diagnosis, will give lifelong cure. Acknowledgments Grateful acknowledgment is made to Dr. J. Occleshaw, Department of Neuroradiology, Manchester Royal Infirmary, Professor P. O. Yates and Dr. G. T. G. Knowlson, University Department of Neuro-pathology, and Mr. R. Neave, Medical Artist, University Department of Medical Illustration, Manchester Royal Infirmary. The author is indebted to Dr. Bruce Foster, F.F.A.R.C.S. who gave the anesthetic in this case. References 1. Acquaviva R, Tamic PM, Thevenot C, et al: Los chondromas intracraneales. Revisi6n de la literatura a prop6sito de dos easos. Rev Esp Otoneurooftalmol 24:15-34, 1965 2. Aronson HA, Otis RD: Intracranial chondroma involving the cerebellopontine angle. Report of a case. J Neurosurg 19:529-531, 1962 J. Neurosurg. / Volume 49 / September, 1978
Intracranial solitary chondroma 3. Artwifiski E: Sur un cas de chondrome du lobe frontal gauche oper6 avec succes. Neuroi Polska 84:16-17, 1935 4. Bagchi AK, Sen Gupta KP: Basicranial chondromas. Int Surg 51:132-137, 1969 5. Bakdash H, Alksne JF, Rand RW: Osteochondroma of the base of the skull causing an isolated oculomotor nerve paralysis. Case report emphasizing microsurgical techniques. J Neurosurg 31:230-233, 1969 6. Chorobski J, Jarzymski J, Ferens E: Intracranial solitary chondroma. Surg Gynecoi Obstet 68:677-686, 1939 7. De Busscher J: Deux chondromes intracraniens. J Beige Neurol Psychlatr 39:81-102, 1939 8. Falconer MA, Bailey IC, Duchen LW: Surgical treatment of chordoma and chondroma of the skull base. J Neurosurg 29:261-275, 1968 9. Forsythe RW, Baker GS, Dockerty MB, et al: Intracranial osteochondroma. Proc Staff Meet Mayo Clin 22:350-356, 1947 10. Froment J, Wertheimer P, Dechaume J: Chondrome de la faux du cerveau. (Avec projections.) Lyon Med 150:356-359, 1932 I I. Gabrielsen TO, Kingman AF Jr: Osteocartilaginous tumors of the base of the skull. Report of a unique case and review of the literature. Am J Roentgenol 91:1016-1023, 1964 12. Green MI, Childrey JL: Intracranial chondroma. A case report. J Nerv Ment Dis 89:650-652, 1939 13. Hardy J, Bertrand C, Maltais R, et al: Volumineux chondro-sarcome calcif6 de la r6gion sellaire. Ex6rese trans-sph6noidale sous contrble radioscopique t616vis6. Neuroehirurgie 12:491-502, 1966 14. Hartmann E: La Radiographie en Ophtalmologie. Atlas Clinique. Paris: Masson, 1936 15. Isamat F, Miranda AM, Ripoll M: Parajugular foramen chondroma. Case report. J Neurosurg 28:490-494, 1968 16. King LS, Butcher J: Osteochondroma of the base of the skull. Arch Pathol 37:282-285, 1944 17. Kleinsasser O: Pathologie der Geschw(ilste des Hirnsch~idels. Gutartige Knorpelgeschwiilste,
J. Neurosurg. / Volume 49 / September, 1978
18.
19. 20.
21. 22. 23. 24. 25. 26. 27. 28. 29.
in Olivecrona H, T~nnis W (eds): Handbuch der Neurochirurgie, Band IV, Teii 1. Berlin: Springer-Verlag, 1960, pp 386-395 Letterer E: l~ber heterotope Geschwfilste der Aderhautgeflechte. (Enchondrome, Gliom und Ganglioneurogliom.) Beitr Pathol Anat 67:370-415, 1920 List CF: Osteochondromas arising from the base of the skull. Surg Gynecoi Obstet 76:480-492, 1943 Mansuy L, Girard P, Bret P: Apropos de deux observations de tumeur du trou d6chir6 post6rieur. Rev Otoneuroophtalmnl 36:249254, 1964 Minagi H, Newton TH: Cartilaginous tumours of the base of skull. Am J Roentgennl 105:308-313, 1969 Neuman M: Volumineux chondrome intracranien. J Chir Ann Soe Brig Chir 26:157-162, 1927 Paleari A: Osteocondroma della base cranica con sindrome diencefalica. Rev Otoneuroophtalmol 15:59-71, 1938 Roukkula M: Roentgenologic findings in chondromas of the pontine angle. Acta Radiol (Diag) 2:120-128, 1964 Russell DS, Rubinstein L J: Pathology of Tumours of the Nervous System, ed 2. London: Edward Arnold, 1963, pp 54, 221 Smitt WGS: I:lber intrakraniale Chondrome. Dtsch Z Nervenheiik 109:170-177, 1929 Trillet M, Lapras C, Bady B, et al: Apropos d'une observation de chondrome de la F.C.P. Rev Otoneuroophtalmol 40:113-116, 1968 Verbrugghen A, Learmonth JR: Chondroma of the :falx cerebri. J Nerv Ment Dis 76:463-466, 1932 Yasargit MG, Krayenb[ihl HA: Chondromas, in Krayenb~ihl HA, Maspes PE, Sweet WH (eds): Progress in Neurological Surgery. Basel: S Karger, 1975, Vol. 6, pp 435-463
Address reprint requests to."John Dutton, Esq., F.R.C.S., Department of Neurosurgery, Manchester Royal Infirmary, Oxford Road, Manchester, M13 9WL, England.
463
Intracranial solitary chondroma Case report
JOHN DUTTON, M.B., B.S., F.R.C.S.
University Department of Neurosurgery, Manchester Royal Infirmary, Manchester, England v' A case of massive solitary intracranial chondroma involvingthe base of the skull in a 15-month-old baby boy was successfullytreated by total excision. The diagnosis was greatly simplified by the use of computerized tomography, which materially helped in planning the surgical approach. KEy WORDS
skull neoplasm
9 chondroma 9 intracranial chondroma 9 computerized tomography
B
Y 1938, 11 cases of solitary intracranial chondroma had been recorded at autopsy2a and 16 at surgery. 6,7 It appears that only 58 tumors have been operated on, and ll found at autopsy. The history varies greatly according to the site of the tumor and its rate of growth; the course varies from 4 months to 7 years and in many cases is indistinguishable from that of a meningioma. In the case of convexity tumors, there has been epilepsy of focal type, a progressive hemiparesis, and later signs of raised intracranial pressure. Only three cases have shown an endostosis, and calcification on x-ray study is rare, accounting for the density which may sometimes be found by careful exposure or tomographic cuts through suspect areas. Case Report
This 15-month-old baby boy was brought in for evaluation of minimal left-sided proptosis and sixth nerve paralysis. Examination. He had no other abnormalities. X-ray films revealed expansion of 460
the left middle fossa. The enormous extent of the space-occupying lesion was shown on computerized tomography (CT) which revealed that the mass occupied the entire middle fossa on the left (Fig. 1 left and center) and extended one-third of the way across the right sphenoidal wing (Fig. 2). The density without contrast indicated areas of calcium. Carotid angiography displayed occlusion of the left internal carotid artery and confirmed massive brain shift to the right side. Operation. Staged removal resulted in complete excision with only transient thirdnerve paralysis (Fig. 1 right).
Pathological Examination The tumor capsule was composed of a collagenous membrane consistent with dura and deep to this was collagenous tissue containing areas of maturing cartilage, presumably the perichondrium. Much of the tumor was composed of well differentiated cartilage of the hyaline variety, developing from spindle-shaped fibroblastic tissue 3". Neurosurg. / Volume 49 / September, 1978
Intracranial solitary chondroma
FIG. 1. Left and Center: Computerized tomography scans reveal expansion of the middle fossa on the left side with forward displacement of the sphenoidal wing, resulting from a large, high-density lesion, extending from the middle fossa high into the parietal region, displacing the lateral and third ventricles to the right and extending one third of the way across the right sphenoidal wing. Right: Final appearance after total removal, showing that the midline structures had returned toward the center but there was still some shift to the right of the lateral and third ventricles with a surface fluid collection.
through immature cartilage to mature hyaline cartilage (Fig. 3). There were no mitotic figures and no multinucleate cells. No elastic fibers were demonstrated and no groups of stellate cells with vacuoles to suggest a chordomatous element were seen. Postoperative Course. The patient's recovery was satisfactory and a subsequent psychological report, using the Griffiths mental development scale, gave the developmental quotient as within normal limits, with no significant retardation. A later carotid angiogram still revealed no functional internal carotid artery arising from the left common carotid artery. The brain now appeared more correctly situated and CT scans showed that the midline structures had returned toward the center but that there was still some shift to the right of the lateral and third ventricles.
cribrosa, but truly intracranial neoplasms composed of cartilage usually present at four main sites. Their combined numbers are still relatively small and, although only about 60 cases have been submitted to surgery, it
Discussion Excluding those patients with more or less generalized chondromatosis, where occasional cases present with intracranial localization, TM the fnding of an intracranial chondroma is extremely rare. Those arising in the nasal septum and sinuses can penetrate through the skull, 1,17 especially the lamina
J. Neurosurg. / Volume 49 / September, 1978
FIG. 2. Artist's impression of operation findings. The composite picture was constructed from the full tomographic survey. 46]
J. Dutton
FIG. 3. Photomicrograph showing the tumor, composed of well differentiated cartilage of the hyaline variety, developing from spindle-shaped fibroblastic tissue through immature cartilage to mature hyaline cartilage. H & E, • 70.
appears that their incidence is showing an upward trend. Tumors arising in the dural partitions, 1~ the leptomeninges, 3,9,~,~6 or the choroid plexus 18 are slightly less common than those arising from the base of the skull. 4,8,u,~3,~,2u~7 Embryologically, the basal bones arise from cartilaginous matrices and these chondromas presumably arise from residua of the primordial cartilaginous cranium. The vault, in contrast, arises from membranous bone. Basal chondromas arise mainly from the region of the foramen lacerum or more precisely the synchondroses that converge upon it. ~ These tumors arise from embryonic residues where the spheno-petrosal, petrooccipital, and spheno-occipital synchondroses meet like a star. They lie extradurally, the majority being parasellar in the middle fossa? ~ Less commonly, they occur in the posterior fossa behind the clivus, or may extend into the cerebellopontine angle. ~,5'1~'~5'2~ Then again, they may occupy both areas simultaneously. As in our case, a parasellar chondroma may grow around and obstruct the internal carotid artery, which represents the third aortic arch. The fifth ganglion may be involved in the tumor and the third and sixth nerves displaced. They destroy the bone diffusely with no reparatory new bone formation, thus the sella or the tip of the petrous bone may be eroded. Such tumors may extend upward high above the sella, or 462
predominantly downward toward the nasopharynx. Chondromas arise by budding of the embryonal cartilaginous rests left in place in the base of the skull or misplaced into neighboring tissues, or, on the other hand, by development from persistent and aberrant foci of meningeal mesenchyme or from leptomeningeal fibroblasts. These tumors are usually hard, but may be soft, elastic, lobulated, rounded, and encapsulated, and frequently reach a large size. They have a milky, opaline surface, typical of cartilage. They may appear to have calcific deposits and yellow mucinous areas with cystic cavitation. They are extradural but ill-defined in relation to bone. Basal chondromas show a preponderance in females. 17 The patient age span is usually between 16 and 54 years 8 in cases not part of generalized chondromatosis. The youngest patient previously reported was 4 years old. .9 Although these tumors are invariably benign, in the past the long-term prognosis has been viewed with caution. At one time surgery was associated with a mortality rate approaching 50% and a high incidence of recurrence in the survivors. The main problem, especially with the parasellar tumors, is their situation, making total removal difficult, and it would appear that x-ray therapy is valueless in such cases. Total removal, which can be achieved much more simply with the so-called convexity tumors, especially with early diagnosis, will give lifelong cure. Acknowledgments Grateful acknowledgment is made to Dr. J. Occleshaw, Department of Neuroradiology, Manchester Royal Infirmary, Professor P. O. Yates and Dr. G. T. G. Knowlson, University Department of Neuro-pathology, and Mr. R. Neave, Medical Artist, University Department of Medical Illustration, Manchester Royal Infirmary. The author is indebted to Dr. Bruce Foster, F.F.A.R.C.S. who gave the anesthetic in this case. References 1. Acquaviva R, Tamic PM, Thevenot C, et al: Los chondromas intracraneales. Revisi6n de la literatura a prop6sito de dos easos. Rev Esp Otoneurooftalmol 24:15-34, 1965 2. Aronson HA, Otis RD: Intracranial chondroma involving the cerebellopontine angle. Report of a case. J Neurosurg 19:529-531, 1962 J. Neurosurg. / Volume 49 / September, 1978
Intracranial solitary chondroma 3. Artwifiski E: Sur un cas de chondrome du lobe frontal gauche oper6 avec succes. Neuroi Polska 84:16-17, 1935 4. Bagchi AK, Sen Gupta KP: Basicranial chondromas. Int Surg 51:132-137, 1969 5. Bakdash H, Alksne JF, Rand RW: Osteochondroma of the base of the skull causing an isolated oculomotor nerve paralysis. Case report emphasizing microsurgical techniques. J Neurosurg 31:230-233, 1969 6. Chorobski J, Jarzymski J, Ferens E: Intracranial solitary chondroma. Surg Gynecoi Obstet 68:677-686, 1939 7. De Busscher J: Deux chondromes intracraniens. J Beige Neurol Psychlatr 39:81-102, 1939 8. Falconer MA, Bailey IC, Duchen LW: Surgical treatment of chordoma and chondroma of the skull base. J Neurosurg 29:261-275, 1968 9. Forsythe RW, Baker GS, Dockerty MB, et al: Intracranial osteochondroma. Proc Staff Meet Mayo Clin 22:350-356, 1947 10. Froment J, Wertheimer P, Dechaume J: Chondrome de la faux du cerveau. (Avec projections.) Lyon Med 150:356-359, 1932 I I. Gabrielsen TO, Kingman AF Jr: Osteocartilaginous tumors of the base of the skull. Report of a unique case and review of the literature. Am J Roentgenol 91:1016-1023, 1964 12. Green MI, Childrey JL: Intracranial chondroma. A case report. J Nerv Ment Dis 89:650-652, 1939 13. Hardy J, Bertrand C, Maltais R, et al: Volumineux chondro-sarcome calcif6 de la r6gion sellaire. Ex6rese trans-sph6noidale sous contrble radioscopique t616vis6. Neuroehirurgie 12:491-502, 1966 14. Hartmann E: La Radiographie en Ophtalmologie. Atlas Clinique. Paris: Masson, 1936 15. Isamat F, Miranda AM, Ripoll M: Parajugular foramen chondroma. Case report. J Neurosurg 28:490-494, 1968 16. King LS, Butcher J: Osteochondroma of the base of the skull. Arch Pathol 37:282-285, 1944 17. Kleinsasser O: Pathologie der Geschw(ilste des Hirnsch~idels. Gutartige Knorpelgeschwiilste,
J. Neurosurg. / Volume 49 / September, 1978
18.
19. 20.
21. 22. 23. 24. 25. 26. 27. 28. 29.
in Olivecrona H, T~nnis W (eds): Handbuch der Neurochirurgie, Band IV, Teii 1. Berlin: Springer-Verlag, 1960, pp 386-395 Letterer E: l~ber heterotope Geschwfilste der Aderhautgeflechte. (Enchondrome, Gliom und Ganglioneurogliom.) Beitr Pathol Anat 67:370-415, 1920 List CF: Osteochondromas arising from the base of the skull. Surg Gynecoi Obstet 76:480-492, 1943 Mansuy L, Girard P, Bret P: Apropos de deux observations de tumeur du trou d6chir6 post6rieur. Rev Otoneuroophtalmnl 36:249254, 1964 Minagi H, Newton TH: Cartilaginous tumours of the base of skull. Am J Roentgennl 105:308-313, 1969 Neuman M: Volumineux chondrome intracranien. J Chir Ann Soe Brig Chir 26:157-162, 1927 Paleari A: Osteocondroma della base cranica con sindrome diencefalica. Rev Otoneuroophtalmol 15:59-71, 1938 Roukkula M: Roentgenologic findings in chondromas of the pontine angle. Acta Radiol (Diag) 2:120-128, 1964 Russell DS, Rubinstein L J: Pathology of Tumours of the Nervous System, ed 2. London: Edward Arnold, 1963, pp 54, 221 Smitt WGS: I:lber intrakraniale Chondrome. Dtsch Z Nervenheiik 109:170-177, 1929 Trillet M, Lapras C, Bady B, et al: Apropos d'une observation de chondrome de la F.C.P. Rev Otoneuroophtalmol 40:113-116, 1968 Verbrugghen A, Learmonth JR: Chondroma of the :falx cerebri. J Nerv Ment Dis 76:463-466, 1932 Yasargit MG, Krayenb[ihl HA: Chondromas, in Krayenb~ihl HA, Maspes PE, Sweet WH (eds): Progress in Neurological Surgery. Basel: S Karger, 1975, Vol. 6, pp 435-463
Address reprint requests to."John Dutton, Esq., F.R.C.S., Department of Neurosurgery, Manchester Royal Infirmary, Oxford Road, Manchester, M13 9WL, England.
463
