Malignant ectomesenchymoma is an uncommon neoplasm composed of neuroectodermal elements and one or more mesenchymal neoplastic elements. It is believed to arise from remnants of migratory neural crest cells (ectomesenchyme). The authors report the clinical and pathological findings of a 3-year-old girl with malignant ectomesenchymoma of the CNS. Embryogenesis of this tumor is discussed, and a review of the literature with 39 other cases is done.

Keywords:
Brain tumor, Ectomesenchyme, Ectomesenchymoma, Neural crest
1.
Baker CVH, Bronner-Fraser M, Le Douarin NM, Teillet MA: Early- and late-migrating cranial neural crest cell populations have equivalent developmental potential in vivo. Development 1997;124:3077–3087.
2.
Selleck MA, Bronner-Fraser M: The genesis of avian neural crest cells: A classic embryonic induction. Proc Natl Acad Sci USA 1996;93:9352–9357.
3.
Le Douarin NM, Dupin E, Ziller C: Genetic and epigenetic control in neural crest development. Curr Opin Genet Dev 1994;4:685–695.
4.
Dupin E, Sextier-Sainte-Claire Deville F, Nataf V, Le Douarin NM: The ontogeny of the neural crest. C R Acad Sci III 1993;316:1062–1081.
5.
Erickson CA, Perris R: The role of cell-cell and cell-matrix interactions in the morphogenesis of the neural crest. Dev Biol 1993;159:60–74.
6.
Couly G, Grapin-Botton A, Coltey P, Le Douarin NM: The regeneration of the cephalic neural crest, a problem revisited: The regenerating cells originate from the contralateral or from the anterior and posterior neural fold. Development 1996;122:3393–3407.
7.
Katschenko N: Zur Entwicklungsgeschichte der Selachierembryos. Anat Anz 1888;3:445–467.
8.
Goronowitsch N: Die axiale und die laterale Kopfmetamerie der Vögelembryonen. Die Rolle der sog. ‘Ganglienleisten’ im Aufbau der Nervenstämme. Anat Anz 1892;7:454–464.
9.
Goronowitsch N: Untersuchungen über die Entwicklung der sogenannten ‘Ganglienleisten’ im Kopfe der Vögelembryonen. Morphol Jahrb 1893;20:187–259.
10.
Platt JB: Ectodermic origin of the cartilage of the head. Anat Anz 1893;8:506–509.
11.
Platt JB: The development of cartilaginous skull and of the branchial and hypoglossal musculature in Necturus. Morphol Jahrb 1897;25:377–464.
12.
Hörstadius S: Introduction: The neural crest; in Hörstadius S (ed): The Neural Crest: Its Properties and Derivatives in the Light of Experimental Research. New York, The Hafner Publishing Company, 1969, pp 1–9.
13.
Jesuthasan S: Contact inhibition/collapse and pathfinding of neural crest cells in the zebrafish trunk. Development 1996;122:381–389.
14.
Bronner-Fraser M: Neural crest cell formation and migration in the developing embryo. FASEB J 1994;8:699–706.
15.
Thorogood P, Smith L: Neural crest cells: The role of extracellular matrix in their differentiation and migration. Prog Clin Biol Res 1984;151:171–185.
16.
Wachtler F: On the differentiation and migration of some non-neuronal neural crest derived cell types. Anat Embryol (Berl) 1984;170:161–168.
17.
Le Douarin NM: Differentiation of the neural crest. Influence of growth factors. Ann Endocrinol (Paris) 1988;49:245–269.
18.
Poirier V, Boisseau S, Poujeoul C, Simonneau M: Environmental signals and neural crest cells. J Physiol (Paris) 1994;88:265–269.
19.
Le Douarin NM, Dulac C, Dupin E, Cameron-Curry P: Glial cell lineages in the neural crest. Glia 1991;4:175–184.
20.
Murphy M, Reid K, Ford M, Furness JB, Bartlett PF: FGF2 regulates proliferation of neural crest cells, with subsequent neuronal differentiation regulated by LIF or related factors. Development 1994;120:3519–3528.
21.
Stern HM, Lin-Jones J, Hauschka SD: Synergistic interactions between bFGF and a TGF-beta family member may mediate myogenic signals from the neural tube. Development 1997;124:3511–3523.
22.
Bittinger A, Rossberg C, Rodehuser M: Primary malignant ectomesenchymoma of the orbit. Gen Diagn Pathol 1997;142:221–225.
23.
Cozzutto C, Cormeli A, Bandelloni R: Ectomesenchymoma: Report of two cases. Virchows Arch A Pathol Anat Histopathol 1982;398:185–195.
24.
Shuangshoti S, Kasantikul V, Suwangool P, Chittmittrapap S: Malignant neoplasm of mixed mesenchymal and neuroepithelial origin (ectomesenchymoma) of thigh. J Surg Oncol 1984;27:208–213.
25.
Kawamoto EH, Weidner N, Agostini RM Jr, Jaffe R: Malignant ectomesenchymoma of soft tissue. Report of two cases and review of the literature. Cancer 1987;59:1791–1802.
26.
Matsko TH, Schmidt RA, Milam AH, Orcutt JC: Primary malignant ectomesenchymoma of the orbit. Br J Ophthalmol 1992;76:438–441.
27.
Hajivassiliou CA, Carachi R, Simpson E, Patrick WJ, Young DG: Ectomesenchymoma: One or two tumors? Case report and review of the literature. J Pediatr Surg 1997;32:1351–1355.
28.
Ingraham FD, Bailey OT: Cystic teratomas and teratoid tumors of the central nervous system in infancy and childhood. J Neurosurg 1946;3:511–532.
29.
Holimon JL, Rosenblum WI: ‘Gangliorhabdomyosarcoma’: A tumor of ectomesenchyme. J Neurosurg 1971;34:417–422.
30.
Naka A, Matsumoto S, Shirai T, Itoh T: Ganglioneuroblastoma associated with malignant mesenchymoma. Cancer 1975;36:1050–1056.
31.
Karcioglu Z, Someren A, Mates SJ: Ectomesenchymoma: A malignant tumor of migratory neural crest (ectomesenchyme) remnants showing ganglionic, Schwannian, melanocytic, and rhabdomyoblastic differentiation. Cancer 1977;39:2486–2496.
32.
Strother DR, Parham DM, Houghton PJ: Expression of the 5.1 H11 antigen, a fetal muscle surface antigen, in normal and neoplastic tissue. Arch Pathol Lab Med 1990; 114:593–596.
33.
Tsokos M: Peripheral primitive neuroectodermal tumors. Diagnosis, classification, and prognosis. Perspect Pediatr Pathol 1992;16:27–98.
34.
Shuangshoti S, Chutchavaree A: Parapharyngeal neoplasm of mixed mesenchymal and neuroepithelial origin. Arch Otoryngol 1980;106:361–364.
35.
Schmidt D, Mackay B, Osborne BM, Jaffe N: Recurring congenital lesion of the cheek. Ultrastruct Pathol 1982;3:85–90.
36.
Sirikulchayanota V, Wongwaisayawan S: Malignant ectomesenchymoma (neoplasm of mixed mesenchymal and neuroepithelial origin) of wrist joint. J Med Assoc Thai 1984;67:356–361.
37.
Kodet R, Kashuri N, Marsden HB, Road NAG, Raafat F: Gangliorhabdomyosarcoma: A histopathological and immunohistochemical study of three cases. Histopathology 1986;10:181–193.
38.
Kasantikul V, Shuangshoti S, Cutchavaree A, Bunyaphiphat P: Parapharyngeal malignant ectomesenchymoma: Combined malignant fibrous histiocytoma and primitive neuroectodermal tumor with neuroglial differentiation. J Laryngol Otol 1987;101:508–515.
39.
Paulus W, Slowik F, Jellinger K: Primary intracranial sarcomas: histopathological features of 19 cases. Histopathology 1991;18:395–402.
40.
Fellinger EJ, Garin-Chesa P, Triche TJ, Huvos AG, Rettig WJ: Immunohistochemical analysis of Ewing’s sarcoma cell surface antigen p30/32MIC2. Am J Pathol 1991;139:317–325.
41.
Garin-Chesa P, Fellinger EJ, Huvos AG, Beresford HR, Melamed MR, Triche TJ, Rettig WJ: Immunohistochemical analysis of neural cell adhesion molecules. Differential expression in small round cell tumors of childhood and adolescence. Am J Pathol 1991;139:275–286.
42.
Dias P, Parham DM, Shapiro DN, Tapscott SJ, Houghton PJ: Monoclonal antibodies to the myogenic regulatory protein MyoD1: Epitope mapping and diagnostic utility. Cancer Res 1992;52:6431–6439.
43.
McCune BK, Patterson K, Chandra RS, Kapur S, Sporn MB, Tsokos M: Expression of transforming growth factor-beta isoforms in small round cell tumors of childhood. An immunohistochemical study. Am J Pathol 1993;142:49–58.
44.
Pellin A, Boix J, Blesa JR, Noguera R, Carda C, Llombart-Bosch A: EWS/FLI-1 rearrangement in small round cell sarcomas of bone and soft tissue detected by reverse transcriptase polymerase chain reaction amplification. Eur J Cancer 1994;30:827–831.
45.
Kogner P, Bjork O, Theodorsson E: Plasma neuropeptide Y in healthy children: Influence of age, anesthesia and the establishment of an age-adjusted reference interval. Acta Paediatr 1994;83:423–427.
46.
Kilpatrick SE, Teot LA, Geisinger KR, Martin PL, Shumate DK, Zbieranski N, Russell GB, Fletcher CD: Relationship of DNA ploidy to histology and prognosis in rhabdomyosarcoma. Comparison of flow cytometry and image analysis. Cancer 1994;74:3227–3233.
47.
Apostolides PJ, Spetzler RF, Johnson PC: Ectomesenchymal hamartoma (benign ‘ectomesenchymoma’) of the VIIIth nerve: case report. Neurosurgery 1995;37:1204–1207.
48.
Mouton SC, Rosenberg HS, Cohen MC, Drut R, Emms M, Kaschula RO: Malignant ectomesenchymoma in childhood. Pediatr Pathol Lab Med 1996;16:607–624.
49.
Goldsby RE, Bruggers CS, Brothman AR, Sorensen PH, Beckwith JB, Pysher TJ: Spindle cell sarcoma of the kidney with ganglionic elements (malignant ectomesenchymoma) associated with chromosomal abnormalities and a review of the literature. J Pediatr Hematol Oncol 1998;20:160–164.
50.
Hibshoosh H, Lattes R: Immunohistochemical and molecular genetic approaches to soft tissue tumor diagnosis: A primer. Semin Oncol 1997;24:515–525.
51.
Bridge JA: Cytogenetics of bone and soft tissue tumors. Cancer J 1996;9:3.
52.
Muro-Cacho CA: The role of immunohistochemistry in the differential diagnosis of soft-tissue tumors. Cancer Control: JMCC 1998;5:53–63.
53.
Brooks JS: Immunohistochemistry in the differential diagnosis of soft tissue tumors. Monogr Pathol 1996;38:65–128.
1999
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