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Ewing's sarcoma of the temporal bone
Ewing’s sarcoma of the temporal bone A case report M. J. C. Davidson, BDS(Lond), Sussex, England DEPARTMENT
OF ORAL
SURGERY,
MB, BS(Lond), FDSRCS(Eng),
ROYAL
SUSSEX
COUNTY
FRCS(Edin),
HOSPITAL
An 1 l-year-old girl with a primary Ewing’s sarcoma in the squamous temporal bone is described, and differential diagnosis and management are discussed. The membranous bones of the skull are rare sites for a primary Ewing’s sarcoma; the squamous temporal bone has not been previously described as a primary site. (ORAL SURC ORAL MED ORAL PATHOL
1991;72:534-6)
E
wing’s sarcoma, a malignant tumor that develops in the medullary tissues of bones, was first described by J. E. Ewing in 1921 .l The tumor, uncommon in adulthood, is the second most common bone tumor in children, with a peak incidence in the second decade of life. It is approximately twice as common in males as in females. The incidence of this tumor in the United Kingdom is 0.6 per million of the population, indicating that it is an uncommon tumor.* Common primary sites include the diaphyses of long bones, the ribs, pelvis, and vertebrae. The tumor has traditionally carried a poor prognosis when only local radiotherapy or surgical treatment has been used, resulting in a 5-year survival rate of approximately 15%. The introduction of combination chemotherapy in multimodality therapy has dramatically improved the 5-year survival rate. Rosen,3 using intensive multiagent chemotherapy, reported a 5-year survival rate of 90% for distal tumors and 65% for axial skeletal tumors. The United Kingdom Children’s Cancer Study Group reported a 5-year survival rate of 39% in patients with Ewing’s sarcoma at any site.3 Patients with a poor prognosis (i.e., those with primary tumors in the axial skeleton, large tumors, or those with secondary deposits) are now given multiagent chemotherapy, and local treatment in the form of radiotherapy and surgery if the tumor is resectable. The case reported here is of a primary lesion arising in the squamous temporal bone, a site that does not appear to have been reported previously. CASEREPORT
An 1l-year-old white girl wasseenin the Accident and EmergencyDepartment, Royal SussexCounty Hospital, 7/12/28560 534
Brighton, with a facial swelling.The patient had sustained a minor muscularneck injury a weekbefore presentation, with no direct facial trauma. Shortly after the injury the child’smother noticeda swellingof the left temporalarea. The patient washealthy, with no history of recentsystemic upsetnor any relevant medicalor drug history. On examination no abnormal clinical findings were presentother than the left temporalswelling.Specificnegative findings were the absenceof febrile illness,lymphadenopathy,and organomegaly. A hard, nontenderswellingwaspresenton the posterior half of the zygomatic arch, extending onto the temporal bone.The overlying skinwasnormal.There wasno restriction of jaw movements,no occlusalderangement,and no demonstrableneurologicdeficit. Plain radiographsrevealedno abnormality; hematologic and biochemicalinvestigationsincluding skeletal profile were within normal limits. A surgical exploration of the swellingwith biopsy was undertaken.With the patient undergeneralanesthesia, the lesionwas explored through a temporal preauricular approach as describedby Al-Kayat and Bramley.4A hard bony massarising from the middle of the left squamous temporalboneextendingto the inferior borderof the zygomatic arch wasexposed.Severalbiopsiesof the obvioustumor were performed,and postoperativerecovery was uneventful. Specimensshoweda small-celltumor infiltrating proliferating bone.The tumor nuclei were neither oval or angulated, and werevariable in shapeand uniform in density. The cytoplasmwaseosinophilic, and no ddinite cell boundary was present. Many mitotic figures and occasional rosetteswereseen(Fig. 1). The initial differential diagnosison clinical and histologicgroundsincluded neuroblas-
toma, malignant lymphoma, and Ewing’s sarcoma. At the referral center,St. Bartholomew’sHospital, London, the histologic findings were reviewed and further stainingwasdone.Periodicacid-SchitT (PAS) stainswith and without diastasetreatment showedthe intracellular
Ewing’s sarcoma of temporal bone
Volume 72 Number 5 Table
535
1. Sequence of clinical events
Mo since presentation 0 2 3 8-9
10 28 43 46
Event
Presentations with left temporalmass Completion of investigations; diagnosis of nonmetastatic Ewing’ssarcoma Commencement of Ewing’ssarcomabad-risk protocol Radiotherapy to left temporalarea(5000Gy) Melphalanadministered with autologous bone rescue Recurrence in left orbit; treatedwith combinationchemotherapy (cisplatinandmethotrexate)andlocalradiotherapy Furtherprogression of localdisease treatedby palliativeradiotherapy Deathfromintracranialextension of disease
presenceof PAS-positive diastase-labilematerial. This activity is indicative of intracellular glycogenand favors the diagnosisof Ewing’ssarcomarather than metastaticneuroblastoma. The patient wasreferred to the Departmentof Pediatric Oncology, St. Bartholomew’sHospital, London, for treatment. Further investigationswere carried out, and histologic sectionswere reviewed. Computerizedtomographic scanof the headdemonstrateda bony massarisingfrom the left temporalboneinvadingthe sphenoidanteriorly, pterygoidplatesinferiorly, andthe lateral pterygoidmuscle(Fig. 2). Radiograph and computed tomographic scan of the chestwere normal.A technetiumbonescandemonstrated increasedradioisotopeuptake in the left zygomatic region only. Resultsof a 24-hoururine collectionfor vanillylmandelic acid were normal. After the diagnosisof nonmetastaticEwing’ssarcoma arisingin the left temporalbonewasconfirmed,the patient wasallocatedto the bad-risk protocol in view of the axial positionof the tumor, which wasunsuitablefor surgicalresection.The treatment protocol consistedof six coursesof multiagentchemotherapyat 2- to 3-weekintervals (cyclophosphamide,vincristine, and doxorubicin), followed by melphalanwith an autologousbonegraft and finally radiotherapy to the primary tumor site. The patient receiveda total of six coursesof chemotherapy; however,the plannedtreatment with melphalanand autologousbonegraft waspostponeduntil after radiotherapy becauseof the patient’sexposureto a viral illness.The clinical sequenceis summarizedin Table 1. DISCUSSION
Ewing’s sarcoma, although an uncommon tumor, accounts for approximately 6% of primary bone tumors.5 The occurrence of this tumor in the membranous bones of the skull is rare. A literature review revealed only two other cases of primary Ewing’s sarcoma in the flat skull bones. Mansfield6 described a case in the frontal bone as a primary site, and Fitzer and Steffey7 found the petrous temporal bone to be the
1. Photomicrographshowssheetsof uniform round cells with inconspicuouscytoplasm. (Hematoxylin-eosin stain; original magnification,X600.) Flg.
Fig. 2. Computedtomographiccoronalheadscanat level of petroustemporal bone.Large arrow indicatesareasof bone expansionand destruction; small arrow indicates soft-tissueextensionof tumor within area of left temporal bone.
primary site. Both these cases involved neurologic deficits. Ewing’s sarcoma has been described in the facial bones, mostly in the mandible.*1 9 The clinical feature attributed to the tumor is a painful, rapidly expanding mass associated with a low-grade pyrexia and malaise. These features were absent in this case. The plain radiographs did not show the features of laminated periosteal changes (onion skin) with associated bone destruction or a soft-tissue component, which occur in the typical radiographic description of Ewing’s sarcoma. Wood et
536
Davidson
al.‘O in a review of the world literature on the radiologic findings of Ewing’s sarcoma of the jaws, demonstrated that laminar periosteal new bone is not a typical radiologic feature. Histologic features in this case were typical of Ewing’s sarcoma. The differential histologic diagnosis was clarified by the demonstration of intracellular glycogen by PAS stain with diastase control, embryonic rhabdomyosarcoma being the only other tumor to demonstrate intracellular glycogen. Metastatic neuroblastoma was excluded by a normal 24-hour urinary vanillylmandelic acid excretion. The indolent course of this child’s illness, with local recurrence and ultimately death from intracranial extension of the tumor, is unfortunately not uncommon in this disease, which remains one of the more difficult children’s cancers to cure. Current trials may result in a continued improvement in the treatment of these sarcomas.
ORAL
SURG ORAL
PATHOL
November 1991 REFERENCES
1. Ewing JE. Diffuse endothelioma of bone. Proc N Y Path01 Sot 1921;21:17-24. 2. 3.
4. 5.
6. I.
Souhami RL. Management of primary malignant bone tumours. Hosp Update 1988;14:1109-15. Rosen G, Caparros B, Nirenberg A, et al. Ewing’s sarcoma: a ten-year experience with adjuvant chemotherapy. Cancer 1980;47:2204-13. Al-Kayat A, Bramley P. A modified approach to the temporomandibular and malar arch. Br J Oral Surg 1979;17:91-103. Dahlin D, Coventry MB, Scanlon PW. Ewing’s sarcoma: a critical analysis of 165 cases. J Bone Joint Surg [Am] 1978; 43A:185-95. Mansfield JB. Primary Ewing’s sarcoma of the skull. Surg Neurol 1982;18:286-8. Fitzer PM, Steffey WR. Brain and bone scans in primary Ewing’s sarcoma 12.
8.
of the petrous
bone. J Neurosurg
1976;44:608-
Rota AN, Smith JL, MacComb WS, Jing BS. Ewing’s sarcoma of the maxilla and mandible: study of six cases. ORAL SURG
ORAL
MED ORAL
PATHOL
1968;25: 194-203.
Bernstein P, Bone RC, Feldman PS. Ewing’s sarcoma of the mandible. Ann Otol 1979;8:105-8. 10. Wood RE, Nortje CJ, Hessling P, Grotepass F. Ewing’s tumor of the jaw. ORAL SURG ORAL MED ORAL PATHOL 1990; 9.
69:120-7.
I thank the following persons for permission to present their patient and for their help in preparing this article: Mr. P. Bathard-Smith, Consultant Oral Surgeon, Royal Sussex County Hospital, Brighton, and Prof. 3. S. Malpas and Dr. J. Kingston of the Department of Paediatric Medical Oncology, St. Bartholomew’s Hospital.
MED ORAL
Reprint
requests:
M. J. C. Davidson, FDSRCS, FRCS Department of Oral Surgery The Dental Hospital at Leeds Claredon Way, Leeds Yorkshire, U.K. LS2 9LU
OF ORAL
SURGERY,
MB, BS(Lond), FDSRCS(Eng),
ROYAL
SUSSEX
COUNTY
FRCS(Edin),
HOSPITAL
An 1 l-year-old girl with a primary Ewing’s sarcoma in the squamous temporal bone is described, and differential diagnosis and management are discussed. The membranous bones of the skull are rare sites for a primary Ewing’s sarcoma; the squamous temporal bone has not been previously described as a primary site. (ORAL SURC ORAL MED ORAL PATHOL
1991;72:534-6)
E
wing’s sarcoma, a malignant tumor that develops in the medullary tissues of bones, was first described by J. E. Ewing in 1921 .l The tumor, uncommon in adulthood, is the second most common bone tumor in children, with a peak incidence in the second decade of life. It is approximately twice as common in males as in females. The incidence of this tumor in the United Kingdom is 0.6 per million of the population, indicating that it is an uncommon tumor.* Common primary sites include the diaphyses of long bones, the ribs, pelvis, and vertebrae. The tumor has traditionally carried a poor prognosis when only local radiotherapy or surgical treatment has been used, resulting in a 5-year survival rate of approximately 15%. The introduction of combination chemotherapy in multimodality therapy has dramatically improved the 5-year survival rate. Rosen,3 using intensive multiagent chemotherapy, reported a 5-year survival rate of 90% for distal tumors and 65% for axial skeletal tumors. The United Kingdom Children’s Cancer Study Group reported a 5-year survival rate of 39% in patients with Ewing’s sarcoma at any site.3 Patients with a poor prognosis (i.e., those with primary tumors in the axial skeleton, large tumors, or those with secondary deposits) are now given multiagent chemotherapy, and local treatment in the form of radiotherapy and surgery if the tumor is resectable. The case reported here is of a primary lesion arising in the squamous temporal bone, a site that does not appear to have been reported previously. CASEREPORT
An 1l-year-old white girl wasseenin the Accident and EmergencyDepartment, Royal SussexCounty Hospital, 7/12/28560 534
Brighton, with a facial swelling.The patient had sustained a minor muscularneck injury a weekbefore presentation, with no direct facial trauma. Shortly after the injury the child’smother noticeda swellingof the left temporalarea. The patient washealthy, with no history of recentsystemic upsetnor any relevant medicalor drug history. On examination no abnormal clinical findings were presentother than the left temporalswelling.Specificnegative findings were the absenceof febrile illness,lymphadenopathy,and organomegaly. A hard, nontenderswellingwaspresenton the posterior half of the zygomatic arch, extending onto the temporal bone.The overlying skinwasnormal.There wasno restriction of jaw movements,no occlusalderangement,and no demonstrableneurologicdeficit. Plain radiographsrevealedno abnormality; hematologic and biochemicalinvestigationsincluding skeletal profile were within normal limits. A surgical exploration of the swellingwith biopsy was undertaken.With the patient undergeneralanesthesia, the lesionwas explored through a temporal preauricular approach as describedby Al-Kayat and Bramley.4A hard bony massarising from the middle of the left squamous temporalboneextendingto the inferior borderof the zygomatic arch wasexposed.Severalbiopsiesof the obvioustumor were performed,and postoperativerecovery was uneventful. Specimensshoweda small-celltumor infiltrating proliferating bone.The tumor nuclei were neither oval or angulated, and werevariable in shapeand uniform in density. The cytoplasmwaseosinophilic, and no ddinite cell boundary was present. Many mitotic figures and occasional rosetteswereseen(Fig. 1). The initial differential diagnosison clinical and histologicgroundsincluded neuroblas-
toma, malignant lymphoma, and Ewing’s sarcoma. At the referral center,St. Bartholomew’sHospital, London, the histologic findings were reviewed and further stainingwasdone.Periodicacid-SchitT (PAS) stainswith and without diastasetreatment showedthe intracellular
Ewing’s sarcoma of temporal bone
Volume 72 Number 5 Table
535
1. Sequence of clinical events
Mo since presentation 0 2 3 8-9
10 28 43 46
Event
Presentations with left temporalmass Completion of investigations; diagnosis of nonmetastatic Ewing’ssarcoma Commencement of Ewing’ssarcomabad-risk protocol Radiotherapy to left temporalarea(5000Gy) Melphalanadministered with autologous bone rescue Recurrence in left orbit; treatedwith combinationchemotherapy (cisplatinandmethotrexate)andlocalradiotherapy Furtherprogression of localdisease treatedby palliativeradiotherapy Deathfromintracranialextension of disease
presenceof PAS-positive diastase-labilematerial. This activity is indicative of intracellular glycogenand favors the diagnosisof Ewing’ssarcomarather than metastaticneuroblastoma. The patient wasreferred to the Departmentof Pediatric Oncology, St. Bartholomew’sHospital, London, for treatment. Further investigationswere carried out, and histologic sectionswere reviewed. Computerizedtomographic scanof the headdemonstrateda bony massarisingfrom the left temporalboneinvadingthe sphenoidanteriorly, pterygoidplatesinferiorly, andthe lateral pterygoidmuscle(Fig. 2). Radiograph and computed tomographic scan of the chestwere normal.A technetiumbonescandemonstrated increasedradioisotopeuptake in the left zygomatic region only. Resultsof a 24-hoururine collectionfor vanillylmandelic acid were normal. After the diagnosisof nonmetastaticEwing’ssarcoma arisingin the left temporalbonewasconfirmed,the patient wasallocatedto the bad-risk protocol in view of the axial positionof the tumor, which wasunsuitablefor surgicalresection.The treatment protocol consistedof six coursesof multiagentchemotherapyat 2- to 3-weekintervals (cyclophosphamide,vincristine, and doxorubicin), followed by melphalanwith an autologousbonegraft and finally radiotherapy to the primary tumor site. The patient receiveda total of six coursesof chemotherapy; however,the plannedtreatment with melphalanand autologousbonegraft waspostponeduntil after radiotherapy becauseof the patient’sexposureto a viral illness.The clinical sequenceis summarizedin Table 1. DISCUSSION
Ewing’s sarcoma, although an uncommon tumor, accounts for approximately 6% of primary bone tumors.5 The occurrence of this tumor in the membranous bones of the skull is rare. A literature review revealed only two other cases of primary Ewing’s sarcoma in the flat skull bones. Mansfield6 described a case in the frontal bone as a primary site, and Fitzer and Steffey7 found the petrous temporal bone to be the
1. Photomicrographshowssheetsof uniform round cells with inconspicuouscytoplasm. (Hematoxylin-eosin stain; original magnification,X600.) Flg.
Fig. 2. Computedtomographiccoronalheadscanat level of petroustemporal bone.Large arrow indicatesareasof bone expansionand destruction; small arrow indicates soft-tissueextensionof tumor within area of left temporal bone.
primary site. Both these cases involved neurologic deficits. Ewing’s sarcoma has been described in the facial bones, mostly in the mandible.*1 9 The clinical feature attributed to the tumor is a painful, rapidly expanding mass associated with a low-grade pyrexia and malaise. These features were absent in this case. The plain radiographs did not show the features of laminated periosteal changes (onion skin) with associated bone destruction or a soft-tissue component, which occur in the typical radiographic description of Ewing’s sarcoma. Wood et
536
Davidson
al.‘O in a review of the world literature on the radiologic findings of Ewing’s sarcoma of the jaws, demonstrated that laminar periosteal new bone is not a typical radiologic feature. Histologic features in this case were typical of Ewing’s sarcoma. The differential histologic diagnosis was clarified by the demonstration of intracellular glycogen by PAS stain with diastase control, embryonic rhabdomyosarcoma being the only other tumor to demonstrate intracellular glycogen. Metastatic neuroblastoma was excluded by a normal 24-hour urinary vanillylmandelic acid excretion. The indolent course of this child’s illness, with local recurrence and ultimately death from intracranial extension of the tumor, is unfortunately not uncommon in this disease, which remains one of the more difficult children’s cancers to cure. Current trials may result in a continued improvement in the treatment of these sarcomas.
ORAL
SURG ORAL
PATHOL
November 1991 REFERENCES
1. Ewing JE. Diffuse endothelioma of bone. Proc N Y Path01 Sot 1921;21:17-24. 2. 3.
4. 5.
6. I.
Souhami RL. Management of primary malignant bone tumours. Hosp Update 1988;14:1109-15. Rosen G, Caparros B, Nirenberg A, et al. Ewing’s sarcoma: a ten-year experience with adjuvant chemotherapy. Cancer 1980;47:2204-13. Al-Kayat A, Bramley P. A modified approach to the temporomandibular and malar arch. Br J Oral Surg 1979;17:91-103. Dahlin D, Coventry MB, Scanlon PW. Ewing’s sarcoma: a critical analysis of 165 cases. J Bone Joint Surg [Am] 1978; 43A:185-95. Mansfield JB. Primary Ewing’s sarcoma of the skull. Surg Neurol 1982;18:286-8. Fitzer PM, Steffey WR. Brain and bone scans in primary Ewing’s sarcoma 12.
8.
of the petrous
bone. J Neurosurg
1976;44:608-
Rota AN, Smith JL, MacComb WS, Jing BS. Ewing’s sarcoma of the maxilla and mandible: study of six cases. ORAL SURG
ORAL
MED ORAL
PATHOL
1968;25: 194-203.
Bernstein P, Bone RC, Feldman PS. Ewing’s sarcoma of the mandible. Ann Otol 1979;8:105-8. 10. Wood RE, Nortje CJ, Hessling P, Grotepass F. Ewing’s tumor of the jaw. ORAL SURG ORAL MED ORAL PATHOL 1990; 9.
69:120-7.
I thank the following persons for permission to present their patient and for their help in preparing this article: Mr. P. Bathard-Smith, Consultant Oral Surgeon, Royal Sussex County Hospital, Brighton, and Prof. 3. S. Malpas and Dr. J. Kingston of the Department of Paediatric Medical Oncology, St. Bartholomew’s Hospital.
MED ORAL
Reprint
requests:
M. J. C. Davidson, FDSRCS, FRCS Department of Oral Surgery The Dental Hospital at Leeds Claredon Way, Leeds Yorkshire, U.K. LS2 9LU