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Ectomesenchymoma

MedGen UID:
98123
•Concept ID:
C0431111
•
Neoplastic Process
Synonyms: ectomesenchymoma; Malignant Ectomesenchymoma; malignant ectomesenchymoma; Rhabdomyosarcoma with ganglionic differentiation; rhabdomyosarcoma with ganglionic differentiation; rhabdomyosarcoma with ganglionic differentiation (morphologic abnormality); Sarcoma with Ganglionic or Neuroectodermal Differentiation; sarcoma with ganglionic or neuroectodermal differentiation
SNOMED CT: Ectomesenchymoma (128750008); Rhabdomyosarcoma with ganglionic differentiation (128750008); Ectomesenchymoma (1295216003); Rhabdomyosarcoma with ganglionic differentiation (1295216003)
 
Monarch Initiative: MONDO:0002855

Definition

An exceedingly rare, aggressive malignant mesenchymal neoplasm characterized by the presence of a sarcomatous component and a ganglionic or a neuroectodermal component. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEctomesenchymoma

Professional guidelines

PubMed

Multiplex RT-PCR assay for the differential diagnosis of alveolar rhabdomyosarcoma and Ewing's sarcoma.
Downing JR, Khandekar A, Shurtleff SA, Head DR, Parham DM, Webber BL, Pappo AS, Hulshof MG, Conn WP, Shapiro DN
Am J Pathol 1995 Mar;146(3):626-34. PMID: 7887445Free PMC Article

Recent clinical studies

Etiology

Malignant ectomesenchymoma in children: The European pediatric Soft tissue sarcoma Study Group experience.
Milano GM, Orbach D, Casanova M, Berlanga P, Schoot RA, Corradini N, Brennan B, Ramirez-Villar GL, Lyngsie Hjalgrim L, van Noesel MM, Alaggio R, Ferrari A
Pediatr Blood Cancer 2023 Feb;70(2):e30116. Epub 2022 Nov 28 doi: 10.1002/pbc.30116. PMID: 36440693
Primary intracranial malignant ectomesenchymoma in an adult: Report of a rare case and review of the literature.
Mahajan S, Suri V, Sharma MC, Kedia S, Sardana H, Nakra T
Neuropathology 2019 Jun;39(3):200-206. Epub 2019 Mar 25 doi: 10.1111/neup.12547. PMID: 30907031
Malignant Ectomesenchymoma: Series Analysis of a Histologically and Genetically Heterogeneous Tumor.
Griffin BB, Chou PM, George D, Jennings LJ, Arva NC
Int J Surg Pathol 2018 May;26(3):200-212. Epub 2017 Oct 10 doi: 10.1177/1066896917734915. PMID: 28994342
Frequent HRAS Mutations in Malignant Ectomesenchymoma: Overlapping Genetic Abnormalities With Embryonal Rhabdomyosarcoma.
Huang SC, Alaggio R, Sung YS, Chen CL, Zhang L, Kao YC, Agaram NP, Wexler LH, Antonescu CR
Am J Surg Pathol 2016 Jul;40(7):876-85. doi: 10.1097/PAS.0000000000000612. PMID: 26872011Free PMC Article
Spindle cell sarcoma of the kidney with ganglionic elements (malignant ectomesenchymoma) associated with chromosomal abnormalities and a review of the literature.
Goldsby RE, Bruggers CS, Brothman AR, Sorensen PH, Beckwith JB, Pysher TJ
J Pediatr Hematol Oncol 1998 Mar-Apr;20(2):160-4. doi: 10.1097/00043426-199803000-00015. PMID: 9544170

Diagnosis

Malignant ectomesenchymoma in children: The European pediatric Soft tissue sarcoma Study Group experience.
Milano GM, Orbach D, Casanova M, Berlanga P, Schoot RA, Corradini N, Brennan B, Ramirez-Villar GL, Lyngsie Hjalgrim L, van Noesel MM, Alaggio R, Ferrari A
Pediatr Blood Cancer 2023 Feb;70(2):e30116. Epub 2022 Nov 28 doi: 10.1002/pbc.30116. PMID: 36440693
Primary uterine ectomesenchymoma harboring a DICER1 mutation: case report with molecular analysis.
Davidson B, Kleinberg L, Børresen IM, Slettevoll F, Fangberget A, Hindosh D, Zahl Eriksson AG, Bjerkehagen B
Virchows Arch 2021 Aug;479(2):419-424. Epub 2021 Feb 17 doi: 10.1007/s00428-021-03057-x. PMID: 33595736Free PMC Article
Malignant ectomesenchymoma of the cerebrum. Case report and discussion of therapeutic options.
Weiss E, Albrecht CF, Herms J, Behnke-Mursch J, Pekrun A, Brockmann K, Hess CF
Eur J Pediatr 2005 Jun;164(6):345-9. Epub 2005 Mar 4 doi: 10.1007/s00431-005-1646-7. PMID: 15747131
Malignant ectomesenchymoma of the nose. Case report and review of the literature.
Brehmer D, Overhoff HM, Marx A
ORL J Otorhinolaryngol Relat Spec 2003 Jan-Feb;65(1):52-6. doi: 10.1159/000068666. PMID: 12624508
Disseminated malignant ectomesenchymoma (MEM): case report and review of the literature.
Müller HL, Marx A, Trusen M, Schneider P, Kühl J
Pediatr Hematol Oncol 2002 Jan-Feb;19(1):9-17. doi: 10.1080/088800102753356149. PMID: 11787870

Therapy

Malignant ectomesenchymoma in children and adolescents: report from the Cooperative Weichteilsarkom Studiengruppe (CWS).
Dantonello TM, Leuschner I, Vokuhl C, Gfroerer S, Schuck A, Kube S, Nathrath M, Bernbeck B, Kaatsch P, Pal N, Ljungman G, Bielack SS, Klingebiel T, Koscielniak E; CWS
Pediatr Blood Cancer 2013 Feb;60(2):224-9. Epub 2012 Apr 25 doi: 10.1002/pbc.24174. PMID: 22535600
Malignant ectomesenchymoma of the paranasal sinuses with proptosis.
Vinck AS, Lerut B, Hermans R, Nuyts S, Sciot R, Jorissen M
B-ENT 2011;7(3):201-4. PMID: 22026142
A rare case of malignant pediatric ectomesenchymoma arising from the falx cerebri.
Altenburger DL, Wagner AS, Eslin DE, Pearl GS, Pattisapu JV
J Neurosurg Pediatr 2011 Jan;7(1):94-7. doi: 10.3171/2010.10.PEDS10261. PMID: 21194292
Malignant ectomesenchymoma in the wrist of a child: case report and review of the literature.
Oppenheimer O, Athanasian E, Meyers P, Antonescu CR, Gorlick R
Int J Surg Pathol 2005 Jan;13(1):113-6. doi: 10.1177/106689690501300117. PMID: 15735865
Malignant ectomesenchymoma of soft tissue. Report of two cases and review of the literature.
Kawamoto EH, Weidner N, Agostini RM Jr, Jaffe R
Cancer 1987 May 15;59(10):1791-802. doi: 10.1002/1097-0142(19870515)59:10<1791::aid-cncr2820591018>3.0.co;2-#. PMID: 2950992

Prognosis

A rare case of malignant pediatric ectomesenchymoma arising from the cerebrum.
Kun Y, Duan Z, Mei X, Xu Y, Li J, Li S, Qi X
Int J Clin Exp Pathol 2015;8(7):8545-50. Epub 2015 Jul 1 PMID: 26339431Free PMC Article
Malignant ectomesenchymoma in children and adolescents: report from the Cooperative Weichteilsarkom Studiengruppe (CWS).
Dantonello TM, Leuschner I, Vokuhl C, Gfroerer S, Schuck A, Kube S, Nathrath M, Bernbeck B, Kaatsch P, Pal N, Ljungman G, Bielack SS, Klingebiel T, Koscielniak E; CWS
Pediatr Blood Cancer 2013 Feb;60(2):224-9. Epub 2012 Apr 25 doi: 10.1002/pbc.24174. PMID: 22535600
Malignant ectomesenchymoma in the wrist of a child: case report and review of the literature.
Oppenheimer O, Athanasian E, Meyers P, Antonescu CR, Gorlick R
Int J Surg Pathol 2005 Jan;13(1):113-6. doi: 10.1177/106689690501300117. PMID: 15735865
Malignant ectomesenchymoma of the nose. Case report and review of the literature.
Brehmer D, Overhoff HM, Marx A
ORL J Otorhinolaryngol Relat Spec 2003 Jan-Feb;65(1):52-6. doi: 10.1159/000068666. PMID: 12624508
Disseminated malignant ectomesenchymoma (MEM): case report and review of the literature.
Müller HL, Marx A, Trusen M, Schneider P, Kühl J
Pediatr Hematol Oncol 2002 Jan-Feb;19(1):9-17. doi: 10.1080/088800102753356149. PMID: 11787870

Clinical prediction guides

Primary intracranial malignant ectomesenchymoma in an adult: Report of a rare case and review of the literature.
Mahajan S, Suri V, Sharma MC, Kedia S, Sardana H, Nakra T
Neuropathology 2019 Jun;39(3):200-206. Epub 2019 Mar 25 doi: 10.1111/neup.12547. PMID: 30907031
Ectomesenchymoma with t(1;12)(p32;p13) evolving from embryonal rhabdomyosarcoma shows no rearrangement of ETV6.
Howley S, Stack D, Morris T, McDermott M, Capra M, Betts D, O'Sullivan MJ
Hum Pathol 2012 Feb;43(2):299-302. Epub 2011 Jul 30 doi: 10.1016/j.humpath.2011.03.010. PMID: 21803398
Relationship of DNA ploidy to histology and prognosis in rhabdomyosarcoma. Comparison of flow cytometry and image analysis.
Kilpatrick SE, Teot LA, Geisinger KR, Martin PL, Shumate DK, Zbieranski N, Russell GB, Fletcher CD
Cancer 1994 Dec 15;74(12):3227-33. doi: 10.1002/1097-0142(19941215)74:12<3227::aid-cncr2820741223>3.0.co;2-x. PMID: 7526971

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