Ewing’s sarcoma of the mandible
E
wing’s sarwma is a malignant neoplasm ~haracterizcd by a low incidence of owurrence hut having a high degree of malignancy. It was first described by Ewing,l in 192 1, as a tumor of cntlothelial origin. Because of certain eharac+cristivs, it should constitute a specific l~~ne neoplasm. Ewing stated that the tumor was not associated with any form of mr-cloma or osteogenic sarcoma, and he thus tcwnecl it a “round ccl1 sarcom;i.” 11~ recognized that it was a highly radiosensitirca tumor. ‘I%~ Iitcrature (~ocs not rcveaI significant fintlings as to the cause of the tumor, iIll< thrrc has lwcn ~onsiderahlc wiifusion in the arc3 of c*linical, radiologic, and I~isfo~~athologic fcnturrs hccansc of several msw that were crronconsly labeled as Bwause of the confusion, some investigators have questioned l3wing’s sarcoma. tllc> \alitlity of Ewing’s samma as a srparatt~ entity. Obcrling and associates,’ as well iIs others, tlisagrcwl with the original rlrwription and stated that the tumor originatctl from immature rctidum wlls, thus suggesting the term “rcticulosarof the tumor in the mandible has von~ii 01’ the nicdullary hone.” The txwIrr(~llcv ‘Surgeon, Hcatl and Neck Serviw. “Hcarl of the Head and Neck ScrCw. ***Resident. j..(. r’atl,ologist, ‘_’ ’ Hcxtl,
Department
of Radiodiagnosis.
89
I+{/.
1.
Fig. d.
hemoglobin of 1 I Gm. The llemosedimentation was Xl mm., and the vnnil mandclic acid was 1.li pg per milligram of creatinine. Chest roenfgmogrnm. was normal. R:~diograms of the mandible (Figs. 3 and 4) sl~o\r~d cstmsivft destruction of honcx, with a huge tumor growing in all directions and inwding soft in the form of “sun rays. ” ‘l%c~ features initially suggested a npurohlastoma metastasis, parts wit11 Exing’s tumor as a wrondary clroiw IZistopabholo,qic~ findings from a hiopss rcvealetl a malignant nwsen~11jmal nroplasm with ap:lrse conneetivc-tissue stroma. Neoplastic elements were predominantly rountl with sm:lll Il~perchromatic nuelvi. Tllwe were some mitotic figures, Lmd the cells in general were uniform and arranged in blocks. Sonrc~ foci of necrosis and hemorrhage were presrnt. Sonw osteogencsis of
a reactive
nnturf:
was
olwrved,
hut
no roscttc
or pseudorosette
structures
were
identififvl.
Sp form of AAdriamyciu was :ulministcrctl at, a dosage of 20 mg. lwr d:iv intraw110wl~ for 3 days. ‘1’110tumor was not sensitive to chrwotllrr:lpy aud gww rapidly, ulvc~r:ltiIlg t IIP ol,al muvos:~ ant1 vsposing tllcl m:Indible to oral fluids. Tlrcl paticwt Ilirvl 2 month :lftcSl atlmiesion. Laljoratory investigation did not reveal mctnstnscs.
DISCUSSION
Ewing’s sarcoma is a tumor of questional~le histogencsis, hut it is generally eonsitlercd to result from immature reticulum cells of the medulla.‘~ 5, i, h It is considered to he the most lethal of bone tumors, and localization in the mandible ~)rotl~~~~~s a clcprcssive tumoral mass with an incrcasc in vascularization of the SUMi’aco anal secondary anemia a11t1 l,vmph node tendcrncss. This suggests an inflamllliltory origin. ( :cschickter and C’opelancl,‘; in their series of nineteen cases of Ewing’s s;rrcom;t of the mandible (15 per cent of sarcomas), thought that in most cases
92
. 5. Biopsy sprcimc~n showing nests of round cells with l~yperchromatic nuclei : focus of necrosis. (Hematoxylin and rosin stain. Medium-power m:rgnification.) f;. The tumor sl~ows densely packed small cells with round nuclei and indistinct outlincx (Hematoxylin and rosin stain. High-power magnifirntion.)
tramma \vas tlescrilwd previous to the first symptoms. Fifty per cent of their scriw of patients had pain as the first symptom, and facia.1 distention occurred i 1120 per writ of the cases. Radiographic* fin(lings wt~c oftc~~ not diagnostic, but in the present case the tlraniatic~ aspects wcrc spectacular. Occasionally, J’lwing’s sarcoma masquerades as ;I benign lesion or as chronic osteomyelitis.!‘, I” It often produces an “onion ],(!(‘I ‘. al)pc%ranw lwc~~usc~of the espansi\-c growth and infiltration of cortical I,OllC~.
In cliff’crcntial diagnosis, mctastatic neuroblastoma generally occurs in paticijts untler 5 years of age, Jvhercas JGwing’s sarcoma occurs in patients over that iIR(‘,” ‘I’llcl~;lpcutic considerations gcncrally favor radiotherapy because the tumors
I. 2. ::, i. 5. Ii. i. “. !I. IO. I I. It’. 1:I. I I. ! .l. I Ii. IT. I h.
I !). “(I.
E
wing’s sarwma is a malignant neoplasm ~haracterizcd by a low incidence of owurrence hut having a high degree of malignancy. It was first described by Ewing,l in 192 1, as a tumor of cntlothelial origin. Because of certain eharac+cristivs, it should constitute a specific l~~ne neoplasm. Ewing stated that the tumor was not associated with any form of mr-cloma or osteogenic sarcoma, and he thus tcwnecl it a “round ccl1 sarcom;i.” 11~ recognized that it was a highly radiosensitirca tumor. ‘I%~ Iitcrature (~ocs not rcveaI significant fintlings as to the cause of the tumor, iIll< thrrc has lwcn ~onsiderahlc wiifusion in the arc3 of c*linical, radiologic, and I~isfo~~athologic fcnturrs hccansc of several msw that were crronconsly labeled as Bwause of the confusion, some investigators have questioned l3wing’s sarcoma. tllc> \alitlity of Ewing’s samma as a srparatt~ entity. Obcrling and associates,’ as well iIs others, tlisagrcwl with the original rlrwription and stated that the tumor originatctl from immature rctidum wlls, thus suggesting the term “rcticulosarof the tumor in the mandible has von~ii 01’ the nicdullary hone.” The txwIrr(~llcv ‘Surgeon, Hcatl and Neck Serviw. “Hcarl of the Head and Neck ScrCw. ***Resident. j..(. r’atl,ologist, ‘_’ ’ Hcxtl,
Department
of Radiodiagnosis.
89
I+{/.
1.
Fig. d.
hemoglobin of 1 I Gm. The llemosedimentation was Xl mm., and the vnnil mandclic acid was 1.li pg per milligram of creatinine. Chest roenfgmogrnm. was normal. R:~diograms of the mandible (Figs. 3 and 4) sl~o\r~d cstmsivft destruction of honcx, with a huge tumor growing in all directions and inwding soft in the form of “sun rays. ” ‘l%c~ features initially suggested a npurohlastoma metastasis, parts wit11 Exing’s tumor as a wrondary clroiw IZistopabholo,qic~ findings from a hiopss rcvealetl a malignant nwsen~11jmal nroplasm with ap:lrse conneetivc-tissue stroma. Neoplastic elements were predominantly rountl with sm:lll Il~perchromatic nuelvi. Tllwe were some mitotic figures, Lmd the cells in general were uniform and arranged in blocks. Sonrc~ foci of necrosis and hemorrhage were presrnt. Sonw osteogencsis of
a reactive
nnturf:
was
olwrved,
hut
no roscttc
or pseudorosette
structures
were
identififvl.
Sp form of AAdriamyciu was :ulministcrctl at, a dosage of 20 mg. lwr d:iv intraw110wl~ for 3 days. ‘1’110tumor was not sensitive to chrwotllrr:lpy aud gww rapidly, ulvc~r:ltiIlg t IIP ol,al muvos:~ ant1 vsposing tllcl m:Indible to oral fluids. Tlrcl paticwt Ilirvl 2 month :lftcSl atlmiesion. Laljoratory investigation did not reveal mctnstnscs.
DISCUSSION
Ewing’s sarcoma is a tumor of questional~le histogencsis, hut it is generally eonsitlercd to result from immature reticulum cells of the medulla.‘~ 5, i, h It is considered to he the most lethal of bone tumors, and localization in the mandible ~)rotl~~~~~s a clcprcssive tumoral mass with an incrcasc in vascularization of the SUMi’aco anal secondary anemia a11t1 l,vmph node tendcrncss. This suggests an inflamllliltory origin. ( :cschickter and C’opelancl,‘; in their series of nineteen cases of Ewing’s s;rrcom;t of the mandible (15 per cent of sarcomas), thought that in most cases
92
. 5. Biopsy sprcimc~n showing nests of round cells with l~yperchromatic nuclei : focus of necrosis. (Hematoxylin and rosin stain. Medium-power m:rgnification.) f;. The tumor sl~ows densely packed small cells with round nuclei and indistinct outlincx (Hematoxylin and rosin stain. High-power magnifirntion.)
tramma \vas tlescrilwd previous to the first symptoms. Fifty per cent of their scriw of patients had pain as the first symptom, and facia.1 distention occurred i 1120 per writ of the cases. Radiographic* fin(lings wt~c oftc~~ not diagnostic, but in the present case the tlraniatic~ aspects wcrc spectacular. Occasionally, J’lwing’s sarcoma masquerades as ;I benign lesion or as chronic osteomyelitis.!‘, I” It often produces an “onion ],(!(‘I ‘. al)pc%ranw lwc~~usc~of the espansi\-c growth and infiltration of cortical I,OllC~.
In cliff’crcntial diagnosis, mctastatic neuroblastoma generally occurs in paticijts untler 5 years of age, Jvhercas JGwing’s sarcoma occurs in patients over that iIR(‘,” ‘I’llcl~;lpcutic considerations gcncrally favor radiotherapy because the tumors
I. 2. ::, i. 5. Ii. i. “. !I. IO. I I. It’. 1:I. I I. ! .l. I Ii. IT. I h.
I !). “(I.
