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Primary ewing's Sarcoma of the skull
Primary Ewing's Sarcoma of the Skull James B. Mansfield, M.D.
An unusual case is described in which primary Ewing's sarcoma of bone presented in the skull. Mansfield JB: PrimaryEwing'ssarcomaof the skull. Surg Neurol 18:286-288, 1982 Ewing's sarcoma is an uncommon malignant neoplasm of bone that neurosurgeons encounter only infrequently. The following case report describes a most unusual presentation of this lesion. Case Report A 34-year-old man came to the emergency room on May 12, 1981, complaining of left-sided headaches and an enlarging mass in the left frontal region that had been present for four months. X-ray films of the skull revealed a lytic lesion with vague borders in the left frontal bone (Fig. 1). A computerized tomographic (CT) scan showed a huge left frontal tumor involving the bone, with compression of the underlying brain and a left-to-right shift of the ventricular system (Fig. 2). A technetium bone scan showed only the solitary left frontal lesion. Left common and selective external carotid angiography showed staining of the large lytic lesion by branches of the superficial temporal artery, with some pooling of contrast material within the lytic area. On May 22, 1981, the tumor was removed via a frontal craniectomy. The neoplasm had destroyed the involved left frontal bone and had invaded the dura mater. A dural graft was performed, as was a cranioplasty. Pathological examination revealed the tumor to be a Ewing's sarcoma (Figs. 3 and 4); this was verified on consultation with another institution. The patient was given postoperative radiation to the entire skull, more intensely in the left frontal region, and chemotherapy with cyclophosphamide, vincristine, adriamycin, and actinomycin D. He was last seen on March 20, 1982; at that time he had been observed to be confused for some time. Repeat CT head scan showed no recurrence and spinal fluid examination was unremarkable. Radiation
encephalopathy was believed to be the cause of his mental status. Discussion In 1921 James Ewing [2] described a lethal primary bone lesion that usually affects children and young adults and most frequently originates in the long bones of the legs [1, 3-5, 7-9]. Ewing mentioned metastases to the skull, but gave no discussion of neurological symptoms. Coley [1] described 149 patients seen at Memorial Hospital, 16% of whom had a neurological lesion. Most of these patients had symptoms and signs referable to involvement of the spine (root pain, urinary retention, and paraplegia) and a minority had secondary involvement of the skull with resultant blindness, ophthalmoplegia, proptosis, and twelfth-nerve paresis. Of 27 patients in Mehta and Hendrickson's series [8], 15 had one or more neurological symptoms. The most common neurological symptom was headache and the most common sign was papilledema. Paresthesias and pain along the spine were other very frequent symptoms, with paraparesis and hemiparesis found frequently. Similarly, Kulick and Mones [6] asserted that neurological involvement from Ewing's sarcoma is a common occurrence, being present in 32 of
From the Departmentof Neurosurgery,Abraham Lincoln School of Medicine, Chicago, IL, and Sherman Hospital, Elgin, IL. Address reprint request to Dr. Mansfield, 860 Summit St., Elgin, I[ 60120. Fig. 1. Roentgenogram of the skull reveals an ill.demarcated lytic leKey words: Ewingsarcoma; neoplasm; skull. sion in the left frontal bone. 286
0090-3019/82/100286-03501.25 O 1981 by Little, Brown and Company (Inc.)
Mansfield: Ewing's Sarcoma
Fig. 2. CT scan demonstrates a large left frontal neoplasm arising from the skull with effacement of the left lateral ventricle and a leftto-right shift.
their 100 cases. Papilledema and increased intracranial pressure--the most common neurological manifestations of Ewing's sarcoma--were seen in one-half of their cases. The cause of the increased intracranial pressure was not clear in all their patients. Dysfunction of the nerve roots occurred frequently, with peripheral nerve, brainstem, and cerebral involvement occurring le.ss often. Osseous metastases are the most frequent ones seen, although the lung is the most common single site involved [6, 7]. The present case is most: unusual in that the skull was the site of primary as opposed to secondary involvement. While Coley's [1] data indicate 3 instances of primary involvement of the skull out of 149 cases and Pritchard [9] reports 3 in 234 patients, the neurological literature is devoid of
Fig. 3. Photomicrograph showing invasion and replacement of bone by neoplastic cells. (H&E; ×lO0.)
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Surgical Neurology Vol 18 No 4 October 1982
reference to Ewing's sarcoma p r e s e n t i n g as a primary lesion of t h e skull. Ewing's sarcoma in its usual progression is an e x t r e m e l y lethal disease, w i t h five-year survival rates rep o r t e d to be 5 to 16% [1, 6, 7, 9]. W h e t h e r the unusual l o c a t i o n found in our p a t i e n t carries a m o r e favorable prognosis remains to be seen.
References 1. Coley BL: Neoplasms of Bone. New York: Hoeber, 1949, pp 297-318 2. Ewing J: Diffuse endothelioma of bone. Proc NY Pathol Soc 21:17-24, 1921 3. Huvos A: Ewing's sarcoma, in Bone Tumors, Diagnosis, Treatment and Prognosis. Philadelphia: Saunders, 1979, pp 322-340 4. Johnson E, Pomeroy C: Integrated therapy for Ewing's sarcoma. Am J Roentgenol 114:532-535, 1972
Fig. 4. Photomicrographshowing typical tumor cells. (H&E; ×400.)
5. Johnson E, Pomeroy C: Evaluation of therapeutic results in Ewing's sarcoma. Am J Roentgenol 123:583-587, 1975 6. Kulick A, Mones J: The neurological complications of Ewing's sarcoma: incidence of neurologic involvement and value of radiotherapy. Mt Sinai J Med (NY) 37:40-59, 1970 7. Macintosh DJ, Price CHG, Jeffree GM: Ewing's tumour. A study of behaviour and treatment in forty-seven cases. J Bone Joint Surg [Br] 57:331-340, 1975 8. Mehta Y, Hendrickson R: CNS involvement in Ewing's sarcoma. Cancer 33:859-862, 1974 9. Pritchard DJ, Dahlin DD, Dauphone RT, Taylor WF, Beabout JW: Ewing's sarcoma. A clinicopathological and statistical analysis of patients surviving five years or longer. J Bone Joint Surg [Am] 57:10-16, 1975
An unusual case is described in which primary Ewing's sarcoma of bone presented in the skull. Mansfield JB: PrimaryEwing'ssarcomaof the skull. Surg Neurol 18:286-288, 1982 Ewing's sarcoma is an uncommon malignant neoplasm of bone that neurosurgeons encounter only infrequently. The following case report describes a most unusual presentation of this lesion. Case Report A 34-year-old man came to the emergency room on May 12, 1981, complaining of left-sided headaches and an enlarging mass in the left frontal region that had been present for four months. X-ray films of the skull revealed a lytic lesion with vague borders in the left frontal bone (Fig. 1). A computerized tomographic (CT) scan showed a huge left frontal tumor involving the bone, with compression of the underlying brain and a left-to-right shift of the ventricular system (Fig. 2). A technetium bone scan showed only the solitary left frontal lesion. Left common and selective external carotid angiography showed staining of the large lytic lesion by branches of the superficial temporal artery, with some pooling of contrast material within the lytic area. On May 22, 1981, the tumor was removed via a frontal craniectomy. The neoplasm had destroyed the involved left frontal bone and had invaded the dura mater. A dural graft was performed, as was a cranioplasty. Pathological examination revealed the tumor to be a Ewing's sarcoma (Figs. 3 and 4); this was verified on consultation with another institution. The patient was given postoperative radiation to the entire skull, more intensely in the left frontal region, and chemotherapy with cyclophosphamide, vincristine, adriamycin, and actinomycin D. He was last seen on March 20, 1982; at that time he had been observed to be confused for some time. Repeat CT head scan showed no recurrence and spinal fluid examination was unremarkable. Radiation
encephalopathy was believed to be the cause of his mental status. Discussion In 1921 James Ewing [2] described a lethal primary bone lesion that usually affects children and young adults and most frequently originates in the long bones of the legs [1, 3-5, 7-9]. Ewing mentioned metastases to the skull, but gave no discussion of neurological symptoms. Coley [1] described 149 patients seen at Memorial Hospital, 16% of whom had a neurological lesion. Most of these patients had symptoms and signs referable to involvement of the spine (root pain, urinary retention, and paraplegia) and a minority had secondary involvement of the skull with resultant blindness, ophthalmoplegia, proptosis, and twelfth-nerve paresis. Of 27 patients in Mehta and Hendrickson's series [8], 15 had one or more neurological symptoms. The most common neurological symptom was headache and the most common sign was papilledema. Paresthesias and pain along the spine were other very frequent symptoms, with paraparesis and hemiparesis found frequently. Similarly, Kulick and Mones [6] asserted that neurological involvement from Ewing's sarcoma is a common occurrence, being present in 32 of
From the Departmentof Neurosurgery,Abraham Lincoln School of Medicine, Chicago, IL, and Sherman Hospital, Elgin, IL. Address reprint request to Dr. Mansfield, 860 Summit St., Elgin, I[ 60120. Fig. 1. Roentgenogram of the skull reveals an ill.demarcated lytic leKey words: Ewingsarcoma; neoplasm; skull. sion in the left frontal bone. 286
0090-3019/82/100286-03501.25 O 1981 by Little, Brown and Company (Inc.)
Mansfield: Ewing's Sarcoma
Fig. 2. CT scan demonstrates a large left frontal neoplasm arising from the skull with effacement of the left lateral ventricle and a leftto-right shift.
their 100 cases. Papilledema and increased intracranial pressure--the most common neurological manifestations of Ewing's sarcoma--were seen in one-half of their cases. The cause of the increased intracranial pressure was not clear in all their patients. Dysfunction of the nerve roots occurred frequently, with peripheral nerve, brainstem, and cerebral involvement occurring le.ss often. Osseous metastases are the most frequent ones seen, although the lung is the most common single site involved [6, 7]. The present case is most: unusual in that the skull was the site of primary as opposed to secondary involvement. While Coley's [1] data indicate 3 instances of primary involvement of the skull out of 149 cases and Pritchard [9] reports 3 in 234 patients, the neurological literature is devoid of
Fig. 3. Photomicrograph showing invasion and replacement of bone by neoplastic cells. (H&E; ×lO0.)
287
288
Surgical Neurology Vol 18 No 4 October 1982
reference to Ewing's sarcoma p r e s e n t i n g as a primary lesion of t h e skull. Ewing's sarcoma in its usual progression is an e x t r e m e l y lethal disease, w i t h five-year survival rates rep o r t e d to be 5 to 16% [1, 6, 7, 9]. W h e t h e r the unusual l o c a t i o n found in our p a t i e n t carries a m o r e favorable prognosis remains to be seen.
References 1. Coley BL: Neoplasms of Bone. New York: Hoeber, 1949, pp 297-318 2. Ewing J: Diffuse endothelioma of bone. Proc NY Pathol Soc 21:17-24, 1921 3. Huvos A: Ewing's sarcoma, in Bone Tumors, Diagnosis, Treatment and Prognosis. Philadelphia: Saunders, 1979, pp 322-340 4. Johnson E, Pomeroy C: Integrated therapy for Ewing's sarcoma. Am J Roentgenol 114:532-535, 1972
Fig. 4. Photomicrographshowing typical tumor cells. (H&E; ×400.)
5. Johnson E, Pomeroy C: Evaluation of therapeutic results in Ewing's sarcoma. Am J Roentgenol 123:583-587, 1975 6. Kulick A, Mones J: The neurological complications of Ewing's sarcoma: incidence of neurologic involvement and value of radiotherapy. Mt Sinai J Med (NY) 37:40-59, 1970 7. Macintosh DJ, Price CHG, Jeffree GM: Ewing's tumour. A study of behaviour and treatment in forty-seven cases. J Bone Joint Surg [Br] 57:331-340, 1975 8. Mehta Y, Hendrickson R: CNS involvement in Ewing's sarcoma. Cancer 33:859-862, 1974 9. Pritchard DJ, Dahlin DD, Dauphone RT, Taylor WF, Beabout JW: Ewing's sarcoma. A clinicopathological and statistical analysis of patients surviving five years or longer. J Bone Joint Surg [Am] 57:10-16, 1975