We describe what we believe is only the second reported case of primitive neuroectodermal tumor of the mandible. Our patient was successfully treated initially with surgery and adjuvant radiation and chemotherapy, but 18 months later she developed a fatal pulmonary metastasis. Although this tumor is aggressive and prone to recurrence and metastasis, early intervention might improve the prognosis in affectedpatients. In patients with unresectable disease, radiation and chemotherapy might have palliative value.
References
1.
KushnerB.H., HajduS.I., GulatiS.C.Extracranial primitive neuroectodermal tumors: The Memorial Sloan-Kettering Cancer Center experience. Cancer1991; 67: 1825–9.
2.
ChowdhuryK., ManoukianJ.J., RochonL., BeginL.R.Extracranial primitive neuroectodermal tumor of the head and neck. Arch Otolaryngol Head Neck Surg1990; 116: 475–8.
3.
JonesJ.E., McGillT.Peripheral primitive neuroectodermal tumors of the head and neck. Arch Otolaryngol Head Neck Surg1995; 121: 1392–5.
4.
JurgensH., BierV., HarmsD.Malignant peripheral neuroectodermal tumors: A retrospective analysis of 42 patients. Cancer1988; 61: 349–57.
5.
MarinaN.M., EtcubanasE., ParhamD.M.Peripheral primitive neuroectodermal tumor (peripheral neuroepithelioma) in children. A review of the St. Jude experience and controversies in diagnosis and management. Cancer1989; 64: 1952–60.
6.
MiserJ.S., KinsellaT.J., TricheT.J.Treatment of peripheral neuroepithelioma in children and young adults. J Clin Oncol1987; 5: 1752–8.
7.
SchmidtD., HarmsD., BurdachS.Malignant peripheral neuroectodermal tumours of childhood and adolescence. Vichows Arch A Pathol Anat Histopathol1985; 406: 351–65.
8.
StoutA.P.A tumor of the ulnar nerve. Proc N Y Pathol Soc1918; 18: 2–11.
9.
DehnerL.P.Primitive neuroectodermal tumor and Ewing's sarcoma. Am J Surg Pathol1993; 17: 1–13.
10.
SamuelA.W.Primitive neuroectodermal tumor arising in the ulnar nerve: A case report. Clin Orthop1982; 167: 236–8.
11.
AskinF.B., RosaiJ., SibleyR.K.Malignant small cell tumor of the thoracopulmonary region in childhood: A distinctive clinicopathologic entity of uncertain histogenesis. Cancer1979; 43: 2438–51.
12.
AkeysonE.W., McCutcheonI.E., PershouseM.A.Primitive neuroectodermal tumor of the median nerve. Case report with cytogenetic analysis. J Neurosurg1996; 85: 163–9.
13.
BatsakisJ.G., MackayB., el-NaggarA.K.Ewing's sarcoma and peripheral primitive neuroectodermal tumor: An interim report. Ann Otol Rhinol Laryngol1996; 105: 838–43.
14.
SinghA.D., HussonM., ShieldsC.L.Primitive neuroectodermal tumor of the orbit. Arch Ophthalmol1994; 112: 217–21.
15.
WilsonW.B., RoloffJ., WilsonH.L.Primary peripheral neuroepithelioma of the orbit with intracranial extension. Cancer1988; 62: 2595–601.
16.
HollisL.J., PooleS., HernJ., PatelK.S.Primitive neuroectodermal tumour of the masseter muscle. J Laryngol Otol1996; 110: 1179–81.
17.
ShahN., RoychoudhuryA., SarkarC.Primitive neuroectodermal tumor of maxilla in an adult. Oral Surg Oral Med Oral Pathol Oral Radiol Endod1995; 80: 683–6.
18.
DehnerL.P.Peripheral and central primitive neuroectodermal tumors: A nosologic concept seeking a consensus. Arch Pathol Lab Med1986; 110: 997–1005.
19.
GrierH.E.The Ewing family of tumors. Ewing's sarcoma and primitive neuroectodermal tumors. Pediatr Clin North Am1997; 44: 991–1004.
20.
ShishikuraA., UshigomeS., ShimodaT.Primitive neuroectodermal tumors of bone and soft tissue: Histological subclassification and clinicopathologic correlations. Acta Pathol Jpn1993; 43: 176–86.
BolenJ.W., ThorningD.Peripheral neuroepithelioma: A light and electron microscopic study. Cancer1980; 46: 2456–62.
23.
Llombart-BoschA., Terrier-LacombeM.J., Peydro-OlayaA., ContessoG.Peripheral neuroectodermal sarcoma of soft tissue (peripheral neuroepithelioma): A pathologic study of ten cases with differential diagnosis regarding other small, round-cell sarcomas. Hum Pathol1989; 20: 273–80.
24.
KahnH.J., ThornerP.S.Monoclonal antibody MB2: A potential marker for Ewing's sarcoma and primitive neuroectodermal tumor. Pediatr Pathol1989; 9: 153–62.
25.
SabateJ.M., FranquetT., ParelladaJ.A.Malignant neuroectodermal tumour of the chest wall (Askin tumour): CT and MR findings in eight patients. Clin Radiol1994; 49: 634–8.
26.
CohnI.Epithelial neoplasms of the peripheral and cranial nerves: Report of three cases, review of the literature. Arch Surg1928; 17: 117–60.
27.
von SchlippeM., WhelanJ.S.Primitive neuroectodermal tumour of the chest wall. Ann Oncol1995; 6: 395–401.