ACTA NEUROCHIRURGICA
Acta Neurochirurgica 51, 97-- 104 (1979)
9 by Springer-Verlag 1979
Servicio de Neurocirug;a, Cllnica Puerta de Hierro, Universidad Aut6noma, Madrid, Spain
Primary Osteogenic Sarcoma of the Skull By
J. Vaquero, R. Carrillo, G. Leunda, and J. M. Cabezudo With 7 Figures
Summary A case of primary osteogenic sarcoma of the skull with intracraniai involvement is presented. The rarity of a skull location for osteogenic sarcoma is stressed. The clinical, diagnostic, and therapeutic aspects of this entity are discussed. Keywords: Osteogenic sarcoma; skull tumours.
Osteogenic sarcomas of the skull are v e r y rare 4. Their occurrence as a p r i m a r y g r o w t h in this location has been discussed is. I n 1936 Geschicker 7 studied 500 bone tumours, 13 of which were located in the skull, 8 of these being diagnosed as osteogenic sarcomas. C a d e ~ p o i n t e d out the occurrence of 2 osteogenic sarcomas in the skull a m o n g 133 patients w i t h this illness. A t the M a y o Clinic C o v e n t r y et al. 5 reviewed 2,276 bone tumours, and they f o u n d 430 osteogenic sarcomas, o f which o n l y 3 affected the skull. A t the Massachusetts General H o s p i t a l Weinfeld and D u d l e y 15 f o u n d o n l y one case of a skull location a m o n g 94 cases o f osteogenic sarcoma. C o l e y and V a n d e n b e r g ~, 14 r e p o r t an incidence of skull location in 888 m a l i g n a n t bone t u m o u r s of 0 . 8 % . We present an additional case of p r i m a r y osteogenic sarcoma of the skull.
Case Report This 34-year-old man was seen for evaluation of a left temporo-occipital regular mass, measuring 5 cm in its largest diameter. He had complained of headaches in that area for the previous three years. At first he noticed a small nodule, the size of a pea, which later grew slowly to reach a size of 5 cm diameter. 7
Acta Neurochirurgica, Vol. 51, Fasc. ]--2
0001-6268/79/0051/0097/$ 01.60
98
,1. Vaquero et al.:
An X-ray film taken three years previously in another hospital demonstrated a small radiolucent lesion in the left temporo-occipital area, with an anterior band of increased density (Fig. 1). On admission, physical examination revealed a healthy young man with a hard and tender mass in the left temporo-occipital area. The skin over it was normal. Laboratory investigations were normal, but alkaline phosphatase was 230 mU/ml. Skull X-ray films revealed a turnout with local destruction and new bone formation (Figs. 2 and 3).
Fig. 1. Plain lateral projection of the skull obtained three years prior to the present admission. An irregular band of increased density, ending in a small radiolucent area, is seen to extend backwards from the petrous bone Left carotid angiography showed the blood supply of the tumour via an enlarged posterior auricular artery and from meningeal rami (Fig. 4). A C T scan revealed intracranial growth of the turnout (Fig. 5). At operation it was difficult to separate the skin from the turnout due to profuse bleeding and to adhesions between them. This exposure showed an extraordinary hard bony turnout protruding from the skull. It was blueish in colour. During attempted mobilization venous bleeding occurred from the margin. Five burr holes were placed round the mass, and, after they had been joined by saw cuts, the bony flap was lifted slightly. The tumour was very adherent to dura mater. The dura mater was opened, and then it was possible to remove the specimen composed of turnout, surrounding bone, and dura mater. The transverse sinus was torn, resulting in profuse bleeding which could be controlled.
Fig. 2 Fig. 3 Figs. 2 and 3. Frontal and lateral projections show, at the time of admission, the changes from the previous finding in Fig. 1. A sizeable lesion featuring a permeative pattern surrounded by sclerotic bony areas can be seen. The latter correspond to the margins of an eroded outer table and also to the partial periphery of the intracranially expanded mass
e~
o
O
%
O
o
100
J. Vaquero
et at. :
Fig. 4. Carotid angiogram, with almost selective filling of the external carotid artery, showing an early tortuous posterior auricular artery (upper). A meningeal feeding artery is seen at a later phase round the upper margin of the turnout (lower)
Macroscopic observation of the specimen (Fig. 6) showed no invasion of the dura mater by the turnout. The histological diagnosis of the tumour, after examination by three different pathologists, was osteogenic sarcoma (Fig. 7). The patient did not receive a course of local radiotherapy, and he remains asymptomatic one year after operation.
Primary Osteogenic Sarcoma of the Skull
101
Fig. 5. Computed tomography showing, at standard window settings (upper), the global shape of the lesion. Here is a moderate shift of midline structures. No abnormal intracranial components, other than the calcium density mass, are present. By using bone window settings (lower) the area of skull erosion and the lower attenuated extracranial and intracranial expansions can be clearly demonstrated, as well as the sclerotic inner portions of the mass
Discussion The diagnostic criterion for osteogenic sarcoma is the demonstration in any part of the tumour of sarcomatous cells which produce an osteoid substance 4, 11, 15 There is an incidence peak between 15-25 years 1% although in considering degeneration of Paget's
102
J. Vaquero
et al.:
disease, a second incidence peak is found in late decades 10, la. 1~ Sarcomatous degeneration of Paget's disease is not common ,a. It has been estimated to be between in 10-15% s0. Previous X-ray exposure of the skull was encountered in 4 of the 14 skull sarcomas reviewed by Thompson et al. in 1970 53 and it could be another aetiological
Fig. 6. Extracranial (upper) and intracranial (lower) aspects of the excised specimen
factor *, l~. Previous head trauma is not considered today as an aetiological factor la The extraordinary rarity of a primary skull location for a osteogenic sarcoma, in the absence of previous factors, can be due, according to Vandenberg and Coley 1% to the fact that the skull bones ossify directly from primitive connective tissue instead of going through a complicated process of cartilaginous degeneration and resorption, which is characteristic of most bones.
Primary Osteogenic Sarcoma of the Skull
103
In the skull the osteogenic sarcoma is located mainly in the posterior areas so, being abundantly fed by rami of the external carotid artery, generally via the superficial temporal artery and middle meningeal artery 8, 9 An elevated alkaline phosphatase can give a clue to the nature of the process, although it is not an specific finding because alkaline phosphatase can be elevated also in metastasis, myeloma, and eosinophilic granuloma 6
Fig. 7. Histological aspect of tumour shows sarcomatous cells and osteoid material. H. E. technique
Although the best treatment for osteogenic sarcoma elsewhere is under discussion 15, in the skull it is accepted that the best form of treatment is radical surgery followed by radiotherapy 8, 18. External carotid artery ligature, prior to removal, has been proposed as a way to reduce operative bleeding s, 9. We think that this is not necessary although it can make the surgical procedure a little shorter. The biggest technical difficulty is the possible intracranial growth of the tumour, which in cases as the one of Kosary 9 and our case, can be adherent to or invade venous sinuses. Late survival is estimated to be under 10% so. It is possible that different anatomical locations of this malignant growth can give it some differences in its biological behaviour 25. In this sense, Thompson et al. 23 have followed for more than 10 years a case of primary skull osteogenic
104
J. Vaquero et al.: Primary Osteogenic Sarcoma of the Skull
sarcoma, without any sign of recurrence. In our case, we should point out the very slow rate of growth of the tumour, three years at least, from the first X - r a y picture until the moment the patient was seen by us. Acknowledgement We wish to thank Dr. Carlos Parera for providing the radiological material and descriptions. References 1. Berg, N., Landberg, T., Lindgren, M., Osteonecrosis and sarcoma following external irradiation of intracranial tumors. Acta Radiol. Ther. (Stockholm) 4 (1966), 417--436. 2. Cade, S., Osteogenic sarcoma: a study based on 133 patients. J. Roy. Coll. Surg. Edinb. 1 (1955), 79--111. 3. Coley, B. L., Tumors of the skull. In: Neoplasms of bone and related conditions, ed. 2, pp. 495--503 (Coley, B. L., ed.). New York: P. B. Hoeber. 1960. 4. Courville, C. B., Deeb, P., Marsh, C., Notes on the pathology of cranial tumors. 7. Osteogenic sarcomas of the cranial vault with particular reference to those associated with osteitis deformans (Paget's disease) and their tendency to involve the dura and brain. Bull. Los Angeles neurol. Soc. 27 (1962), 57--74. 5. Coventry, M. B., Dahlin, D. C., Osteogenic sarcoma. A critical analysis of 430 cases. J. Bone Jr. Surg. 39 A (1957), 741--758. 6. Gerlach, J., Tumours of the cranial vault. In: Handbook of clinical neurology. Tumours of the brain and skull, part II, Vol. 17, pp. 104--135 (Vinken, P. J., Bruyn, B. W., eds.). Amsterdam: Am. Elsevier Publish. 1974. 7. Geschickter, C. F., Primary tumors of the cranial bones. Amer. J. Cancer 26 (1936), 155--180. 8. Hendee, R. W., jr., Primary osteogenic sarcoma of the calvaria. Case report. J. Neurosurg. 45 (1976), 334--337. 9. Kosary, I. Z., Braham, J., Bubis, J. J., Primary osteogenic sarcoma of the skull. Case report. J. Neurosurg. 25 (1966), 87--89. 10. Long, D. M., Kieffer, S. A., Chou, S. N., Tumorous lesions of the skull. In: Neurological surgery, p. 1239 (Youmans, J. R., ed.). Philadelphia: W. B. Saunders. 1973. 11. Osbourne, R. L., The differential radiologic diagnosis of bone tumors. CA (N.Y.) 24 (1974), 194--211. 12. Rowbotham, G. F., Neoplasms that grow from the bone-forming elements of the skull. A survey of 20 cases. Brit. J. Surg. 45 (1957), 123--134. 13. Thompson, J. B., Patterson, R. H., jr., Parsons, H., Sarcomas of the calvaria. Surgical experience with 14 patients. J. Neurosurg. 32 (1970), 534--538. 14. Vandenberg, H. J., jr., Coley, B. L., Primary tumors of the cranial bones. Surg. Gynec. Obstet. 90 (1950), 602--612. 15. Weinfeld, M. S., Dudley, H. R., Osteogenic sarcoma: A follow-up study of the ninety-four cases observed at the Massachusetts General Hospital from 1920-1960. J. Bone Jt. Surg. 44 A (1962), 269--276. Authors' address: J. Vaquero Crespo, Servicio de Neurocirugia, Clfnica Pnerta de Hierro, San Martin de Porres, 4, Madrid-35, Spain.
Acta Neurochirurgica 51, 97-- 104 (1979)
9 by Springer-Verlag 1979
Servicio de Neurocirug;a, Cllnica Puerta de Hierro, Universidad Aut6noma, Madrid, Spain
Primary Osteogenic Sarcoma of the Skull By
J. Vaquero, R. Carrillo, G. Leunda, and J. M. Cabezudo With 7 Figures
Summary A case of primary osteogenic sarcoma of the skull with intracraniai involvement is presented. The rarity of a skull location for osteogenic sarcoma is stressed. The clinical, diagnostic, and therapeutic aspects of this entity are discussed. Keywords: Osteogenic sarcoma; skull tumours.
Osteogenic sarcomas of the skull are v e r y rare 4. Their occurrence as a p r i m a r y g r o w t h in this location has been discussed is. I n 1936 Geschicker 7 studied 500 bone tumours, 13 of which were located in the skull, 8 of these being diagnosed as osteogenic sarcomas. C a d e ~ p o i n t e d out the occurrence of 2 osteogenic sarcomas in the skull a m o n g 133 patients w i t h this illness. A t the M a y o Clinic C o v e n t r y et al. 5 reviewed 2,276 bone tumours, and they f o u n d 430 osteogenic sarcomas, o f which o n l y 3 affected the skull. A t the Massachusetts General H o s p i t a l Weinfeld and D u d l e y 15 f o u n d o n l y one case of a skull location a m o n g 94 cases o f osteogenic sarcoma. C o l e y and V a n d e n b e r g ~, 14 r e p o r t an incidence of skull location in 888 m a l i g n a n t bone t u m o u r s of 0 . 8 % . We present an additional case of p r i m a r y osteogenic sarcoma of the skull.
Case Report This 34-year-old man was seen for evaluation of a left temporo-occipital regular mass, measuring 5 cm in its largest diameter. He had complained of headaches in that area for the previous three years. At first he noticed a small nodule, the size of a pea, which later grew slowly to reach a size of 5 cm diameter. 7
Acta Neurochirurgica, Vol. 51, Fasc. ]--2
0001-6268/79/0051/0097/$ 01.60
98
,1. Vaquero et al.:
An X-ray film taken three years previously in another hospital demonstrated a small radiolucent lesion in the left temporo-occipital area, with an anterior band of increased density (Fig. 1). On admission, physical examination revealed a healthy young man with a hard and tender mass in the left temporo-occipital area. The skin over it was normal. Laboratory investigations were normal, but alkaline phosphatase was 230 mU/ml. Skull X-ray films revealed a turnout with local destruction and new bone formation (Figs. 2 and 3).
Fig. 1. Plain lateral projection of the skull obtained three years prior to the present admission. An irregular band of increased density, ending in a small radiolucent area, is seen to extend backwards from the petrous bone Left carotid angiography showed the blood supply of the tumour via an enlarged posterior auricular artery and from meningeal rami (Fig. 4). A C T scan revealed intracranial growth of the turnout (Fig. 5). At operation it was difficult to separate the skin from the turnout due to profuse bleeding and to adhesions between them. This exposure showed an extraordinary hard bony turnout protruding from the skull. It was blueish in colour. During attempted mobilization venous bleeding occurred from the margin. Five burr holes were placed round the mass, and, after they had been joined by saw cuts, the bony flap was lifted slightly. The tumour was very adherent to dura mater. The dura mater was opened, and then it was possible to remove the specimen composed of turnout, surrounding bone, and dura mater. The transverse sinus was torn, resulting in profuse bleeding which could be controlled.
Fig. 2 Fig. 3 Figs. 2 and 3. Frontal and lateral projections show, at the time of admission, the changes from the previous finding in Fig. 1. A sizeable lesion featuring a permeative pattern surrounded by sclerotic bony areas can be seen. The latter correspond to the margins of an eroded outer table and also to the partial periphery of the intracranially expanded mass
e~
o
O
%
O
o
100
J. Vaquero
et at. :
Fig. 4. Carotid angiogram, with almost selective filling of the external carotid artery, showing an early tortuous posterior auricular artery (upper). A meningeal feeding artery is seen at a later phase round the upper margin of the turnout (lower)
Macroscopic observation of the specimen (Fig. 6) showed no invasion of the dura mater by the turnout. The histological diagnosis of the tumour, after examination by three different pathologists, was osteogenic sarcoma (Fig. 7). The patient did not receive a course of local radiotherapy, and he remains asymptomatic one year after operation.
Primary Osteogenic Sarcoma of the Skull
101
Fig. 5. Computed tomography showing, at standard window settings (upper), the global shape of the lesion. Here is a moderate shift of midline structures. No abnormal intracranial components, other than the calcium density mass, are present. By using bone window settings (lower) the area of skull erosion and the lower attenuated extracranial and intracranial expansions can be clearly demonstrated, as well as the sclerotic inner portions of the mass
Discussion The diagnostic criterion for osteogenic sarcoma is the demonstration in any part of the tumour of sarcomatous cells which produce an osteoid substance 4, 11, 15 There is an incidence peak between 15-25 years 1% although in considering degeneration of Paget's
102
J. Vaquero
et al.:
disease, a second incidence peak is found in late decades 10, la. 1~ Sarcomatous degeneration of Paget's disease is not common ,a. It has been estimated to be between in 10-15% s0. Previous X-ray exposure of the skull was encountered in 4 of the 14 skull sarcomas reviewed by Thompson et al. in 1970 53 and it could be another aetiological
Fig. 6. Extracranial (upper) and intracranial (lower) aspects of the excised specimen
factor *, l~. Previous head trauma is not considered today as an aetiological factor la The extraordinary rarity of a primary skull location for a osteogenic sarcoma, in the absence of previous factors, can be due, according to Vandenberg and Coley 1% to the fact that the skull bones ossify directly from primitive connective tissue instead of going through a complicated process of cartilaginous degeneration and resorption, which is characteristic of most bones.
Primary Osteogenic Sarcoma of the Skull
103
In the skull the osteogenic sarcoma is located mainly in the posterior areas so, being abundantly fed by rami of the external carotid artery, generally via the superficial temporal artery and middle meningeal artery 8, 9 An elevated alkaline phosphatase can give a clue to the nature of the process, although it is not an specific finding because alkaline phosphatase can be elevated also in metastasis, myeloma, and eosinophilic granuloma 6
Fig. 7. Histological aspect of tumour shows sarcomatous cells and osteoid material. H. E. technique
Although the best treatment for osteogenic sarcoma elsewhere is under discussion 15, in the skull it is accepted that the best form of treatment is radical surgery followed by radiotherapy 8, 18. External carotid artery ligature, prior to removal, has been proposed as a way to reduce operative bleeding s, 9. We think that this is not necessary although it can make the surgical procedure a little shorter. The biggest technical difficulty is the possible intracranial growth of the tumour, which in cases as the one of Kosary 9 and our case, can be adherent to or invade venous sinuses. Late survival is estimated to be under 10% so. It is possible that different anatomical locations of this malignant growth can give it some differences in its biological behaviour 25. In this sense, Thompson et al. 23 have followed for more than 10 years a case of primary skull osteogenic
104
J. Vaquero et al.: Primary Osteogenic Sarcoma of the Skull
sarcoma, without any sign of recurrence. In our case, we should point out the very slow rate of growth of the tumour, three years at least, from the first X - r a y picture until the moment the patient was seen by us. Acknowledgement We wish to thank Dr. Carlos Parera for providing the radiological material and descriptions. References 1. Berg, N., Landberg, T., Lindgren, M., Osteonecrosis and sarcoma following external irradiation of intracranial tumors. Acta Radiol. Ther. (Stockholm) 4 (1966), 417--436. 2. Cade, S., Osteogenic sarcoma: a study based on 133 patients. J. Roy. Coll. Surg. Edinb. 1 (1955), 79--111. 3. Coley, B. L., Tumors of the skull. In: Neoplasms of bone and related conditions, ed. 2, pp. 495--503 (Coley, B. L., ed.). New York: P. B. Hoeber. 1960. 4. Courville, C. B., Deeb, P., Marsh, C., Notes on the pathology of cranial tumors. 7. Osteogenic sarcomas of the cranial vault with particular reference to those associated with osteitis deformans (Paget's disease) and their tendency to involve the dura and brain. Bull. Los Angeles neurol. Soc. 27 (1962), 57--74. 5. Coventry, M. B., Dahlin, D. C., Osteogenic sarcoma. A critical analysis of 430 cases. J. Bone Jr. Surg. 39 A (1957), 741--758. 6. Gerlach, J., Tumours of the cranial vault. In: Handbook of clinical neurology. Tumours of the brain and skull, part II, Vol. 17, pp. 104--135 (Vinken, P. J., Bruyn, B. W., eds.). Amsterdam: Am. Elsevier Publish. 1974. 7. Geschickter, C. F., Primary tumors of the cranial bones. Amer. J. Cancer 26 (1936), 155--180. 8. Hendee, R. W., jr., Primary osteogenic sarcoma of the calvaria. Case report. J. Neurosurg. 45 (1976), 334--337. 9. Kosary, I. Z., Braham, J., Bubis, J. J., Primary osteogenic sarcoma of the skull. Case report. J. Neurosurg. 25 (1966), 87--89. 10. Long, D. M., Kieffer, S. A., Chou, S. N., Tumorous lesions of the skull. In: Neurological surgery, p. 1239 (Youmans, J. R., ed.). Philadelphia: W. B. Saunders. 1973. 11. Osbourne, R. L., The differential radiologic diagnosis of bone tumors. CA (N.Y.) 24 (1974), 194--211. 12. Rowbotham, G. F., Neoplasms that grow from the bone-forming elements of the skull. A survey of 20 cases. Brit. J. Surg. 45 (1957), 123--134. 13. Thompson, J. B., Patterson, R. H., jr., Parsons, H., Sarcomas of the calvaria. Surgical experience with 14 patients. J. Neurosurg. 32 (1970), 534--538. 14. Vandenberg, H. J., jr., Coley, B. L., Primary tumors of the cranial bones. Surg. Gynec. Obstet. 90 (1950), 602--612. 15. Weinfeld, M. S., Dudley, H. R., Osteogenic sarcoma: A follow-up study of the ninety-four cases observed at the Massachusetts General Hospital from 1920-1960. J. Bone Jt. Surg. 44 A (1962), 269--276. Authors' address: J. Vaquero Crespo, Servicio de Neurocirugia, Clfnica Pnerta de Hierro, San Martin de Porres, 4, Madrid-35, Spain.
