European Journal of Radiology, 15 (1992) 193-195
193
0 1992 Elsevier Science Publishers B.V. All rights reserved. 0720-048X/92/$05.00
EURRAD
003 11
Primary osteogenic sarcoma of the falx cerebri: a case report T. Ali Zlrh”,
M. Necmettin
aDepartment of Neurosurgery,
Pamir”,
M. Memet
b Department of Radiology, and ‘Department
Ozek”,
Canan
of Pathology, Marmara
Erzenb
and Aydin
Sav’
University Faculty of Medicine, Istanbul,
Turkqv
(Received 8 January 1992; accepted after revision 18 April 1992)
Key words: Computed tomography,
falx cerebri; Brain, neoplasm;
Osteosarcoma.
Falx
Introduction Sarcomas account for 3 % of all primary intracranial tumors [l] and only 1.62% of osteogenic sarcomas are located in the skull [ 2,3]. Primary osteosarcoma of the meninges is extremely rare, with only three cases reported in the literature, and only one of them being a case of primary falx osteosarcoma [4-61. This rarity of the lesion and its uncertain neuroradiological features may cause some confusion in preoperative differential diagnosis. A case of primary falx osteosarcoma is presented and its neuroradiological features discussed in the light of relevant literature. Case report A 64-year-old male patient presented with headache and weakness on his right side. The medical history revealed that the symptoms began six months previously and worsened until his examination. At physical examination the findings were in normal range, as were laboratory findings, including calcium, phosphate and alkaline phosphatase levels. Neurologic examination revealed mild right hemiparesis with neither deep tendon reflex asymmetry nor positive Babinski Sign. Plain skull radiographs demonstrated calcification at the right frontoparietal region located from midline to laterally (Fig. 1). Correspondence to: T. Ali Zirh, MD, Marmara Universitesi Hastanesi, Norosirurji Anabilim Dali, Tophanelioglu Cad No: 13-15. Altunizade 81190. Istanbul, Turkey.
Fig. 1. Plain skull X-ray demonstrating calcification frontoparietal region.
at the right
Computed tomography (CT) scans revealed a hyperdense, regular-shaped mass with 1160 HU density adjacent to the falx. Moderate brain edema was apparent and there was no clear cleavage between the tumor and brain tissue; spiculated areas with moderate involvement of the soft tissue was detected. There was no difference between the infused and non-infused scans (Fig. 2a,b). On operation, following left parietal craniotomy, a greyish white, multilobulated, firm, gritty mass with dimensions of 7 x 3 x 3 cm originating from the falx cerebri was observed and totally resected. On histopathologic examination, frozen sections of the material showed a meningioma-like histopathologic picture with areas of mature bone tissue and osteoid.
a
b
Fig. 2. (a) A non-infused CT scan revealing a hyperdense, regular shaped mass adjacent to the falx. Note moderate brain edema and absence of clear cleavage between the tumor and brain tissue. (b) A CT scan at the same level after IV contrast infusion. No remarkable difference can be detected between non-infused and infused slices.
The coexistence of these two dissimilar histologic features, i.e., meningioma and osteoid-matrix, were interpreted as meningioma and/or primary osteoma of the meninges. But eventual examination of the residual tissue on permanent sections showed an enigmatic pattern. Serial sections taken from the tumor mass revealed a pleomorphic, highly anaplastic cell population with osteoid and tumor bone. However, in some areas of the tumor matrix elements blended together as chondroosteoid. Moreover, the septa in this particular tumor, besides containing multinucleated giant cells and lacelike osteoid, also demonstrated nuclear atypia and atypical mitotic figures, Meningioma-like areas of the tumor tissue lay adjacent to tumor bone tissue and osteoid. The postoperative period was uneventful. All skeletal survey radiographs and bone scintigraphy and repeated blood parameters were found to be in normal range; the case was considered to be primary falx osteosarcoma. The patient was discharged on the 10th postoperative day with mild right hemiparesis. On follow-up examinations, three and eight months after surgical intervention, the right hemiparesis persisted. There was no evidence of recurrence on follow-up CT scans. Discussion Primary osteosarcoma is predominantly a tumor of long bones with greatest predilection for the metaphy-
sis. It is extremely rare for a primary osteosarcoma to originate from the meninges. To our knowledge, only three cases have been reported in the literature, only one of which is a primary falx osteosarcoma [5-71. With skull osteosarcomas, the majority of patients are initially seen with a painless skull mass and neurologic symptoms are present in approximately 40% of patients at initial presentation [ 81. In contrast to this generalization, in this patient, due to the falx origin, headache and hemiparesis were the major landmarks of clinical presentation. This disease has been associated with Paget’s disease, fibrous dysplasia, irradiation, brain metastasis from osteogenic sarcoma of the skeletal bone [ 9-151. An association between trauma and osteogenic sarcoma has been considered [ 14,161, and viral etiology has also been suggested [ 171. With the exclusion of all these factors, as in our patient, the case is considered as primary osteogenic sarcoma. Besides, the fact that the membraneous elements of the calvaria, blood vessels and all layers of meninges are derivates of the primitive multipotential mesenchyme [ 181, and the primitive multipotential cell containment of meninges [7,19,20] could explain the origin of primary falx osteosarcoma. Neuroradiologic evaluation of primary osteosarcoma of the skull is important; radiologic assessment from the plain radiographs is very difficult and there is no preferred site for characteristic appearance [2,3,9]. As in the long bones, the lesion in the skull may be lytic, such as eosinophilic granuloma, metastasis, hemangioma, etc, or blastic, as was observed in our case, or of a mixed type [2,21,22]. CT plays an essential role in the neuroradiologic assessment in such tumors. Although the tumors originate from the falx, hyperdensity, calcification and the regular shape of the lesion can lead to a preoperative diagnosis of falx meningioma, whereas the unverified contrast enhancement of the lesion and its extremely high density of 1160 HU opposes this diagnosis. However, the presence of brain edema and spiculated areas with moderate involvement of the soft tissue and the absence of clear cleavage between the tumor and the brain tissue cast doubt on the benign character of the lesion. Moreover, the irregularity of the lesion on CT scans described as a CT sign of potentially malignant behavior of meningiomas [6,23,24], indicated the tumor’s possible malignant nature. In the light of these neuroradiologic findings and the literature we think that this case serves as a reminder to neuroradiologists that primary falx osteosarcoma is a rare entity that should be considered in the differential diagnosis of falx meningiomas.
195
References 1 Lee WR, Laurie J, Townsend
2
3
4
5
6
7
8 9
10 11 12
13
AL. Fine structure of a radiation induced osteogenic sarcoma. Cancer 1975; 76: 1414-1425. Huvos AG, Sundaresan N, Bretskys S, Butler A. Osteogenic sarcoma of the skull. A clinicopathologic study of 19 patients. Cancer 1985; 56: 1214-1221. Nora FE, Unnik K, Pritchard DJ, Dahlin DC. Osteosarcoma of extragnathic craniofacial bones. Mayo Clinic Proc 1973; 58: 26% 272. Lam RM, Malik GM, Chanson JL. Osteosarcoma of meninges. Clinical, light and ultrastructural observations of a case. Am J Surg Pathol 1981; 5: 203-208. Turner OA. Craig WMcK. Osteogenic sarcoma of meningeal origin. Report of a case of meningeal tumor with both osteoblastic and osteoclastic activity. Arch Path01 1941; 32: 103-l 11. Wang AM, Fitzgerald TS. Lichtman AH, Power TC, Zamani AA, Haykal HA, Rumba& CL. Neuroradiologic features of primary falx osteosarcoma AJNR 1986; 7: 729-732. Lam RM, Colah SA. Atypical fibrous histiocytoma with myxoid stroma. A rare lesion arising from dura mater of the brain. Cancer 1979; 43: 237-245. Arlen M, Higinbotham NL, Huvos AG, et al. Radiation induced sarcoma of the bone. Cancer, 1971; 28: 1087-1099. Caron AS, Hajdu SI, Strong EW. Osteogenic sarcoma of the facial and cranial bones: A review of forty-three cases. Am J Surg 1971; 122: 719-725. Dahlin DC, Coventry MB. Osteogenic sarcoma- a study of six hundred cases. J Bone Joint Surg 1967; 49A: 101-110. Garland LH: Osteogenic sarcoma of the skull. Radiology 1945; 45: 45-48. Harding WG II, Courville CB. Bone formation in metastases of osteogenic sarcoma. Report of a case with metastases to the brain. Am J Cancer 1934; 21: 787-794. McKenna RJ, Schwinn CP. Soong KY, Higinbotham NL. Sar-
14
15 16 17 18
19
20
21 22
23
24
comata of the osteogenic series (osteosarcoma, fibrosarcoma, chondrosarcoma, parosteal osteogenic sarcoma, and sarcomata arising in abnormal bone): An analysis of 552 cases. J Bone Joint Surg 1966; 48A: l-26. Thompson JB, Patterson RH Jr., Parsons H. Sarcomas of the calvaria: Surgical experience with 14 patients. J Neurosurg 1970; 32: 534-538. Vandenberg HJ Jr, Coley BL. Primary tumors of the cranial bones. Surg Gynecol Obstet 1950; 90: 602-612. Hendee RW Jr. Primary osteogenic sarcoma of the calvaria. Case report. J Neurosurg 1976; 45: 334-337. Finkelstein JB. Osteosarcoma ofthe jaw bones. Radio1 Clin North Am 1970; 8: 425-443. McLone DG, Bondareff W. Developmental morphology of the subarachnoidal space and contiguous structures in the mouse. Am J Anat 1975; 142: 273-294. Kepes JL, Kepes M, Sloik F. Fibrous xanthomas and xanthosarcomas of the meninges of the brain. Acta Neuropathol (Berl) 1973; 23: 187-199. Rubinstein LJ. Sarcomas of the nervous system. In Minkler (Ed): Pathology of the nervous system. New York, McGraw-Hill, Vol. 2, 1971; 2144-2164. Benson JE, Goske M, Han JS, Brodekey JS, Yoon JS. Primary osteogenic sarcoma of the calvaria. AJNR 1984; 5: 810-813. Sundaresan N, Galicich JH, Rosen G, Huvos AG. Primary osteogenic sarcoma of the skull. Five years disease free survival following surgery and a high dose methotrexate therapy. N Y State J Med 1985; 85: 598-599. New PFJ, Hesselink JR, O’Carroll CP. Kleinman GM. Malignant meningiomas: CT and histologic criteria including a new CT sign. AJNR 1982; 3: 267-276. Vassilouthis J, Ambrose J. Computerized tomography scanning appearances of intracranial meningiomas. An attempt to predict the histological features. J Neurosurg 1979; 50: 320-327.
193
0 1992 Elsevier Science Publishers B.V. All rights reserved. 0720-048X/92/$05.00
EURRAD
003 11
Primary osteogenic sarcoma of the falx cerebri: a case report T. Ali Zlrh”,
M. Necmettin
aDepartment of Neurosurgery,
Pamir”,
M. Memet
b Department of Radiology, and ‘Department
Ozek”,
Canan
of Pathology, Marmara
Erzenb
and Aydin
Sav’
University Faculty of Medicine, Istanbul,
Turkqv
(Received 8 January 1992; accepted after revision 18 April 1992)
Key words: Computed tomography,
falx cerebri; Brain, neoplasm;
Osteosarcoma.
Falx
Introduction Sarcomas account for 3 % of all primary intracranial tumors [l] and only 1.62% of osteogenic sarcomas are located in the skull [ 2,3]. Primary osteosarcoma of the meninges is extremely rare, with only three cases reported in the literature, and only one of them being a case of primary falx osteosarcoma [4-61. This rarity of the lesion and its uncertain neuroradiological features may cause some confusion in preoperative differential diagnosis. A case of primary falx osteosarcoma is presented and its neuroradiological features discussed in the light of relevant literature. Case report A 64-year-old male patient presented with headache and weakness on his right side. The medical history revealed that the symptoms began six months previously and worsened until his examination. At physical examination the findings were in normal range, as were laboratory findings, including calcium, phosphate and alkaline phosphatase levels. Neurologic examination revealed mild right hemiparesis with neither deep tendon reflex asymmetry nor positive Babinski Sign. Plain skull radiographs demonstrated calcification at the right frontoparietal region located from midline to laterally (Fig. 1). Correspondence to: T. Ali Zirh, MD, Marmara Universitesi Hastanesi, Norosirurji Anabilim Dali, Tophanelioglu Cad No: 13-15. Altunizade 81190. Istanbul, Turkey.
Fig. 1. Plain skull X-ray demonstrating calcification frontoparietal region.
at the right
Computed tomography (CT) scans revealed a hyperdense, regular-shaped mass with 1160 HU density adjacent to the falx. Moderate brain edema was apparent and there was no clear cleavage between the tumor and brain tissue; spiculated areas with moderate involvement of the soft tissue was detected. There was no difference between the infused and non-infused scans (Fig. 2a,b). On operation, following left parietal craniotomy, a greyish white, multilobulated, firm, gritty mass with dimensions of 7 x 3 x 3 cm originating from the falx cerebri was observed and totally resected. On histopathologic examination, frozen sections of the material showed a meningioma-like histopathologic picture with areas of mature bone tissue and osteoid.
a
b
Fig. 2. (a) A non-infused CT scan revealing a hyperdense, regular shaped mass adjacent to the falx. Note moderate brain edema and absence of clear cleavage between the tumor and brain tissue. (b) A CT scan at the same level after IV contrast infusion. No remarkable difference can be detected between non-infused and infused slices.
The coexistence of these two dissimilar histologic features, i.e., meningioma and osteoid-matrix, were interpreted as meningioma and/or primary osteoma of the meninges. But eventual examination of the residual tissue on permanent sections showed an enigmatic pattern. Serial sections taken from the tumor mass revealed a pleomorphic, highly anaplastic cell population with osteoid and tumor bone. However, in some areas of the tumor matrix elements blended together as chondroosteoid. Moreover, the septa in this particular tumor, besides containing multinucleated giant cells and lacelike osteoid, also demonstrated nuclear atypia and atypical mitotic figures, Meningioma-like areas of the tumor tissue lay adjacent to tumor bone tissue and osteoid. The postoperative period was uneventful. All skeletal survey radiographs and bone scintigraphy and repeated blood parameters were found to be in normal range; the case was considered to be primary falx osteosarcoma. The patient was discharged on the 10th postoperative day with mild right hemiparesis. On follow-up examinations, three and eight months after surgical intervention, the right hemiparesis persisted. There was no evidence of recurrence on follow-up CT scans. Discussion Primary osteosarcoma is predominantly a tumor of long bones with greatest predilection for the metaphy-
sis. It is extremely rare for a primary osteosarcoma to originate from the meninges. To our knowledge, only three cases have been reported in the literature, only one of which is a primary falx osteosarcoma [5-71. With skull osteosarcomas, the majority of patients are initially seen with a painless skull mass and neurologic symptoms are present in approximately 40% of patients at initial presentation [ 81. In contrast to this generalization, in this patient, due to the falx origin, headache and hemiparesis were the major landmarks of clinical presentation. This disease has been associated with Paget’s disease, fibrous dysplasia, irradiation, brain metastasis from osteogenic sarcoma of the skeletal bone [ 9-151. An association between trauma and osteogenic sarcoma has been considered [ 14,161, and viral etiology has also been suggested [ 171. With the exclusion of all these factors, as in our patient, the case is considered as primary osteogenic sarcoma. Besides, the fact that the membraneous elements of the calvaria, blood vessels and all layers of meninges are derivates of the primitive multipotential mesenchyme [ 181, and the primitive multipotential cell containment of meninges [7,19,20] could explain the origin of primary falx osteosarcoma. Neuroradiologic evaluation of primary osteosarcoma of the skull is important; radiologic assessment from the plain radiographs is very difficult and there is no preferred site for characteristic appearance [2,3,9]. As in the long bones, the lesion in the skull may be lytic, such as eosinophilic granuloma, metastasis, hemangioma, etc, or blastic, as was observed in our case, or of a mixed type [2,21,22]. CT plays an essential role in the neuroradiologic assessment in such tumors. Although the tumors originate from the falx, hyperdensity, calcification and the regular shape of the lesion can lead to a preoperative diagnosis of falx meningioma, whereas the unverified contrast enhancement of the lesion and its extremely high density of 1160 HU opposes this diagnosis. However, the presence of brain edema and spiculated areas with moderate involvement of the soft tissue and the absence of clear cleavage between the tumor and the brain tissue cast doubt on the benign character of the lesion. Moreover, the irregularity of the lesion on CT scans described as a CT sign of potentially malignant behavior of meningiomas [6,23,24], indicated the tumor’s possible malignant nature. In the light of these neuroradiologic findings and the literature we think that this case serves as a reminder to neuroradiologists that primary falx osteosarcoma is a rare entity that should be considered in the differential diagnosis of falx meningiomas.
195
References 1 Lee WR, Laurie J, Townsend
2
3
4
5
6
7
8 9
10 11 12
13
AL. Fine structure of a radiation induced osteogenic sarcoma. Cancer 1975; 76: 1414-1425. Huvos AG, Sundaresan N, Bretskys S, Butler A. Osteogenic sarcoma of the skull. A clinicopathologic study of 19 patients. Cancer 1985; 56: 1214-1221. Nora FE, Unnik K, Pritchard DJ, Dahlin DC. Osteosarcoma of extragnathic craniofacial bones. Mayo Clinic Proc 1973; 58: 26% 272. Lam RM, Malik GM, Chanson JL. Osteosarcoma of meninges. Clinical, light and ultrastructural observations of a case. Am J Surg Pathol 1981; 5: 203-208. Turner OA. Craig WMcK. Osteogenic sarcoma of meningeal origin. Report of a case of meningeal tumor with both osteoblastic and osteoclastic activity. Arch Path01 1941; 32: 103-l 11. Wang AM, Fitzgerald TS. Lichtman AH, Power TC, Zamani AA, Haykal HA, Rumba& CL. Neuroradiologic features of primary falx osteosarcoma AJNR 1986; 7: 729-732. Lam RM, Colah SA. Atypical fibrous histiocytoma with myxoid stroma. A rare lesion arising from dura mater of the brain. Cancer 1979; 43: 237-245. Arlen M, Higinbotham NL, Huvos AG, et al. Radiation induced sarcoma of the bone. Cancer, 1971; 28: 1087-1099. Caron AS, Hajdu SI, Strong EW. Osteogenic sarcoma of the facial and cranial bones: A review of forty-three cases. Am J Surg 1971; 122: 719-725. Dahlin DC, Coventry MB. Osteogenic sarcoma- a study of six hundred cases. J Bone Joint Surg 1967; 49A: 101-110. Garland LH: Osteogenic sarcoma of the skull. Radiology 1945; 45: 45-48. Harding WG II, Courville CB. Bone formation in metastases of osteogenic sarcoma. Report of a case with metastases to the brain. Am J Cancer 1934; 21: 787-794. McKenna RJ, Schwinn CP. Soong KY, Higinbotham NL. Sar-
14
15 16 17 18
19
20
21 22
23
24
comata of the osteogenic series (osteosarcoma, fibrosarcoma, chondrosarcoma, parosteal osteogenic sarcoma, and sarcomata arising in abnormal bone): An analysis of 552 cases. J Bone Joint Surg 1966; 48A: l-26. Thompson JB, Patterson RH Jr., Parsons H. Sarcomas of the calvaria: Surgical experience with 14 patients. J Neurosurg 1970; 32: 534-538. Vandenberg HJ Jr, Coley BL. Primary tumors of the cranial bones. Surg Gynecol Obstet 1950; 90: 602-612. Hendee RW Jr. Primary osteogenic sarcoma of the calvaria. Case report. J Neurosurg 1976; 45: 334-337. Finkelstein JB. Osteosarcoma ofthe jaw bones. Radio1 Clin North Am 1970; 8: 425-443. McLone DG, Bondareff W. Developmental morphology of the subarachnoidal space and contiguous structures in the mouse. Am J Anat 1975; 142: 273-294. Kepes JL, Kepes M, Sloik F. Fibrous xanthomas and xanthosarcomas of the meninges of the brain. Acta Neuropathol (Berl) 1973; 23: 187-199. Rubinstein LJ. Sarcomas of the nervous system. In Minkler (Ed): Pathology of the nervous system. New York, McGraw-Hill, Vol. 2, 1971; 2144-2164. Benson JE, Goske M, Han JS, Brodekey JS, Yoon JS. Primary osteogenic sarcoma of the calvaria. AJNR 1984; 5: 810-813. Sundaresan N, Galicich JH, Rosen G, Huvos AG. Primary osteogenic sarcoma of the skull. Five years disease free survival following surgery and a high dose methotrexate therapy. N Y State J Med 1985; 85: 598-599. New PFJ, Hesselink JR, O’Carroll CP. Kleinman GM. Malignant meningiomas: CT and histologic criteria including a new CT sign. AJNR 1982; 3: 267-276. Vassilouthis J, Ambrose J. Computerized tomography scanning appearances of intracranial meningiomas. An attempt to predict the histological features. J Neurosurg 1979; 50: 320-327.
