Malignant Ectomesenchymoma in Childhood

Mouton, S.

doi:10.1080/713601190

Cited by 6 publications

(15 citation statements)

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“…The 6-year-old's clinical course paralleled that of the older group. Overall, eight were male and seven female, contrary to prior reports suggesting a more marked male predilection [2,6].…”

Section: Resultscontrasting

confidence: 84%

“…One finding was a relatively wider pediatric age range (6 months to 15 years) then previously appreciated for EM [2,6]. Only 5/15 were infants (Յ1 year old), although 10/15 were Յ3 years old (6 males, 4 females).…”

Section: Resultsmentioning

confidence: 97%

“…Also, a small subset of primitive neuroectodermal tumors (PNET) have a few scattered desmin-reactive cells; although these are not necessarily identifiable as rhabdomyoblastic, are apparently myoD1-negative and of uncertain significance [24]. Further, some neuroblastoma cell lines are desmin positive [6]. It has been noted that some RMS have double minutes or evidence of Nmyc amplification [25].…”

Section: Discussionmentioning

confidence: 96%

“…Then again, antibody cross-reactivities may explain some of these observations. Given these caveats, we would propose that the diagnosis of EM be limited to tumors with histologic evidence of neural elements, typically within an RMS, and not simply show IHC reactivity for "neural" markers in otherwise "pure" RMS, which in some cases has led to a diagnosis of EM [6]. IHC stain results alone are probably insufficient evidence for divergent differentiation, given the enormous variabilities in their use [22] and the above-noted discrepancies, some suggestive of antibody cross-reactivity or other artifacts.…”

Section: Discussionmentioning

confidence: 97%

“…Typically, there was scattered focal evidence of neural differentiation noted on hematoxylin and eosin (H&E) staining, confirmed by immunohistochemistry (IHC) with or without ultrastructural confirmation. In children generally, the predominant mesenchymal element of the EM is RMS [2,6], whereas in adults other mesenchymal elements may predominate [11,13,15]. A synonymous term for EM that is sometimes used in the literature is gangliorhabdomyosarcoma [1,9], although this term applies less well to EM with predominantly more primitive neuroblastic elements (PNB).…”

Section: Introductionmentioning

confidence: 99%

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Clinicopathologic Study of Ectomesenchymomas from Intergroup Rhabdomyosarcoma Study Groups III and IV

et al. 2000

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Ectomesenchymomas (EM) are rare malignant neoplasms usually consisting of rhabdomyosarcoma (RMS) with a neural component. Only 21 cases have been previously reported. Here we extend the clinicopathologic spectrum of EM by describing our findings in 15 cases. Only 5 patients were infants; 10 were < or =3 years old and 5 were > or =6 years old. No male predilection was observed; 7 were female. The originating institutional diagnoses were; RMS (12), undifferentiated sarcoma (1), or EM (2), suggesting underdiagnosis of this entity. The primary tumor sites included external genital (5), pelvis/abdomen (6), head and neck (3), and extremity (1). The size of the primary neoplasm was usually > or =5 cm at diagnosis but dissemination only occurred in a minority. Local infiltration was not uncommon. These neoplasms were typically multilobate, thinly encapsulated, hemorrhagic, and necrotic. Light microscopic features were highly variable, but embryonal RMS with scattered or clustered ganglion cells, often in lacunae, was characteristic. In some cases, primitive neuroblastic or neuroectodermal areas were found and/or a component of alveolar RMS was seen. Focal anaplasia was occasionally observed. Mitotic activity appears higher than previously appreciated and some necrosis was invariably present. Electron microscopy was performed in 11 cases, which confirmed skeletal muscle +/- neural differentiation. Cytogenetic studies performed in five cases revealed no specific abnormality. Monoclonal neuron-specific enolase was the best marker of ganglion cells and primitive neural elements. MIC-2 (CD99) membrane expression was not definitively present in any of the six cases examined. A number of the above parameters appear to be of some prognostic significance, but overall, these neoplasms appear to have a similar outcome as would be predicted for their RMS element alone (exclusive of any neural component), with respect to the RMS subtype, age of the patient, and anatomic location of the neoplasm.

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“…The 6-year-old's clinical course paralleled that of the older group. Overall, eight were male and seven female, contrary to prior reports suggesting a more marked male predilection [2,6].…”

Section: Resultscontrasting

confidence: 84%

Section: Resultsmentioning

confidence: 97%