J Neurosurg 47:785-787, 1977

Primary fibrosarcoma of the skull Case report

JAMES B. MANSFIELD, M . D .

Department of Neurosurgery, Abraham Lincoln School of Medicine, Chicago, and Sherman Hospital, Elgin, Illinois u, A case of primary fibrosarcoma of the skull is presented. Only one other case with this uncommon site of occurrence of this tumor has been previously reported. KEY WORDS

9 fibrosarcoma

F

IBROSARCOMAS are malignant primary bone tumors that usually occur in the long bones of the legs, especially in the area of the knee. Only one case has been reported previously of this neoplasm located in the skull with no associated pathological process? ~

9 osteosarcoma

9 skull tumor

demonstrated ala area of increased radionuclide localization peripherally in the region of the left frontotemporoparietal junction. Operations. An excisional trephine biopsy was performed. The outer table of the skull was eroded by a soft, grayish tumor, while the

C a s e Report

A 35-year-old w o m a n was evaluated because of increasing pain deep in the left ear region for 2 years. She had been seeing an otolaryngologist for 10 months and was being treated for chronic bilateral otitis media without significant improvement. Examination. Sinus x-ray films revealed a lytic region in the left parietal area. Physical examination showed only bilateral perforated tympanic membranes with small tubes present in both ear canals and decreased auditory acuity bilaterally. Skull x-ray films demonstrated a 2-cm radiolucent defect in the left parietotemporal region, with a sharp anterior but indistinct posterior border (Fig. 1). Routine laboratory tests were within normal limits. An electroencephalogram showed small sharp seizure activity in the left anterior and mid-temporal regions, while a brain scan

J. Neurosurg. / Volume 47 / November, 1977

FIG. 1. Left lateral skull film shows the lytic lesion. 785

J. B. Mansfield

Fix. 2. Representative section of tumor demonstrating cells with spindle-shaped nuclei arranged in an interweaving pattern. H & E, • 100.

inner table was intact. Pathological interpretation of the specimen was that of a Grade II fibrosarcoma, confirmed by a second consultation (Fig. 2). For this reason, a more extensive craniectomy was carried out at a second operation. Resection of the underlying dura and its replacement with a Silastic graft followed by an acrylic cranioplasty completed the treatment. The patient tolerated both procedures well and her subsequent skull xray films have shown no change over 1 year. Discussion Fibrosarcoma of bone is most c o m m o n in the second through fourth decades: symptoms and signs of the tumor are usually longstanding and not specific, with complaints of pain or a mass in the region of the tumor. The x-ray a p p e a r a n c e of the t u m o r is not characteristic2 ,16,x9 It is often difficult to dis786

tinguish the lesion from an osteolytic osteosarcoma when it appears in its frequent location in the long bones. There is a poorly defined central or eccentric zone of lysis and often an associated periosteal reaction. Primary fibrosarcoma of bone can arise from the scalp, periosteum, dura, or supportive connective tissue o f the bone marrow, and is made up entirely of fibrous elements. 1'2'1~ When arising at the site of preexisting disease, the tumor is termed a secondary fibrosarcoma. This occurs chiefly in areas involved by Paget's disease, other predisposing conditions including sarcoma, and otherwise typical giant-cell tumors, fibromas, aneurysmal bone cysts, fibrous dysplasia, benign osteoblastoma, bone subjected to prior radiation, sites of osteomyelitis, and bone infarcts. 5'7'9,x7a9 In 1959, Jelsma stated that he had not seen a case, or a reference to a case, involving the calvaria. 14 He mentioned that Dandy had reported a primary fibroma of the skullP .pancoast, et al., TM asserted that fibrosarcomas involve the cranium, but they were speaking of soft-tissue tumors that arose from without and invaded the bone. Several large series of fibrosarcomas include no examples involving the skull? ,3,8,'1,~3,~6 Dahlin and Ivins 5 reported on 114 cases examined at the Mayo Clinic before 1968. Four of these are indicated as occurring in the skull, but they were all s e c o n d a r y fibrosarcomas. Several series of cranial tumors refer to primary fibrosarcomas, but only Geschickter reports on a specific case? ~ This 34-year-old woman noted a painful mass associated with headache 21/2 months before excision of the tumor. Death occurred 5 years later from intracranial extension. Microscopically the typical cell produces collagen in variable amounts and has spindleshaped nuclei. Differential diagnosis includes benign fibroblastic regions of bone and osteogenic sarcoma with prominent fibroblastic components, rare spindle-cell sarcomas of soft tissue, fibrous dysplasia, and metastatic tumors which may have a spindlecell pattern2 ,19 The studies cited above agree that grading according to the degree of cellular anaplasia appears to have a significant bearing on prognosis2 ,x~aa,x9 In the series of Dahlins and Ivins, 5 the 5year survival rate for patients with Grade I and II tumors combined was 38.5%, and J. Neurosurg. / Volume 47 / November, 1977

Primary fibrosarcoma of the skull 24.5% for those with Grade III and IV tumors?

Most of the patients with fibrosarcoma involving an extremity have been treated with

amputation, although radical local excision has also been employed. N o effective chemotherapeutic agents are available. 19 Radiation therapy has produced definite suppression of the t u m o r ' s growth in several instances. Long, et al., TM assert that radiation combined with radical surgery is the treatment of choice, but provide no data to support this claim. Recently, Thompson, et a l . ) ~ have reported 10-year survival in two cases of primary cranial osteogenic sarcoma treated with aggressive surgery, and Hendee TM has presented a similar case with a current 2-year survival. The present case is reported so as to call the attention of neurosurgeons to this rare cause of an osteolytic skull lesion. References

1. Conley J, Stout AP, Healey WE: Clinicopathologic analysis of eighty-four patients with an original diagnosis of fibrosarcoma of the head and neck. Am J Surg 114:564-569, 1967 2. Courville CB, Deeb P, Marsh C: Notes on the pathology of cranial tumors. Bull Los Angeles Neurol Soc 27:57-74, 1962 3. Cunningham MP, Arlen M: Medullary fibrosarcoma of bone. Cancer 21:31-37, 1968 4. Dahlin DC: Personal communication, 1976 5. Dahlin DC, Ivins JC: Fibrosarcoma of bone. A study of 114 cases. Cancer 23:35-41, 1969 6. Dandy WE: Surgery of the brain, in Lewis D (ed): Lewis' Practice of Surgery, Volume 12. Hagerstown, Md: WF Prior, 1932 7. Dorfman HD, Norman A, Wolff H: Fibrosarcoma complicating bone infarction in a caisson worker. A case report. J Bone Joint Surg 48A:528-532, 1966 8. Erye-Brook AL, Price CHG: Fibrosarcoma of bone. Review of fifty consecutive cases from the Bristol Bone Turnout Registry. J Bone Joint Surg 51B:20-37, 1969

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9. Furey JG, Ferrer-Torells M,,Reagan JW: Fibrosarcoma arising at the site of bone infarcts. A report of two cases. J Bone Joint Surg 42A:802-810, 1960 10. Geschickter CF: Primary tumors of the cranial bones. Am J Cancer 26:155-180, 1936 11. Gilmer WS Jr, MacEwen GD: Central (medullary) fibrosarcoma of bone. J Bone Joint Surg 40A:121-141, 1958 12. Hendee RW Jr: Primary osteogenic sarcoma of the calvaria. Case report. J Neurosurg 45:334-337, 1976 13. Jaffe HL: Tumors and Tumorous Conditions of the Bone and Joints. Philadelphia: Lea and Febiger, 1958, pp 298-313 14. Jelsma F: Primary Tumors of the Calvaria. Springfield, Ill: Charles C Thomas, 1959 15. Long DM, Kieffer SA, Chou SN: Tumorous lesions of the skull, in Youmans JR (ed): Neurological Surgery, Volume 3. Philadelphia: WB Saunders, 1973, pp 1231-1274 16. McLeod J J, Dahlin DC, Ivins JC: Fibrosarcoma of bone. Am J Surg 94:431-437, 1957 17. Morris JM, Lucas DB: Fibrosarcoma within a sinus tract of chronic draining osteomyelitis. Case. report and review of the literature. J Bone Joint Surg 46A:853-857, 1964 i8. Pancoast HK, Pendergrass PE, Schaeffer JP: The Head and Neck in Roentgen Diagnosis. Springfield, Ill: Charles C Thomas, 1940, 976 pp (see p 251) 19. Spjut HJ, Dorfman HD, Fechner RE, et al: Fibrosarcoma, in Atlas of Tumor Pathology. Second Series, Fascicle 5: Tumors of Bone and Cartilage. Washington, DC: Armed Forces Institute of Pathology, 1971 20. Thompson JB, Patterson RH Jr, Parsons H: Sarcomas of the calvaria. Surgical experience with 14 patients. J Neurosurg 32:534-538, 1970 21. Vandenberg HJ Jr, Coley BL: Primary tumors of the cranial bones. Surg Gynecol Obstet 90:602-612, 1950

Address reprint requests to: James B. Mansfield, M.D., Elgin Neurosurgery, Ltd., 860 Summit Street, Elgin, Illinois 60120.

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Primary fibrosarcoma of the skull. Case report.

J Neurosurg 47:785-787, 1977 Primary fibrosarcoma of the skull Case report JAMES B. MANSFIELD, M . D . Department of Neurosurgery, Abraham Lincoln...
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