MALIGNANT ECTOMESENCHYMOMA
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ALTERNATE NAMES
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Mature Ectomesenchymoma; Gangliorhabdomyosarcoma; Rhabdomyosarcoma with Ganglionic
Differentiation
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DESCRIPTION
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Malignant Ectomesenchymoma is a rare, potentially aggressive tumor with sarcoma-like cellular features. Medical
or pathology reports may describe it as a primitive neuroectodermal tumor (PNET) or
embryonal tumor with multilayer rosettes (ETMR). It may originate in the central nervous
system or soft tissue of the head and neck, abdomen, perineum, scrotum or limbs. It
occurs mostly in children.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING
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Diagnostic testing: Testing for malignant ectomesenchymoma tumors includes:
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Imaging studies (CT, MRI, or nucleide scans such as liver-spleen scans);
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Spinal tap for cerebrospinal fluid cytology;
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Urinary catecholamines; and
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Physical findings: Depends on the location of the tumor.
ICD-9: 191.X and other codes, depending upon site
ICD-10: C71.9
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PROGRESSION
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These tumors largely affect children under 15 years of age and about 20% are found
in adults. Increasing experience with the treatment of malignant ectomesenchymoma
via appropriate surgical approaches, and combination chemotherapy and radiotherapy,
may offer a better prognosis for children presenting with this rare and complex tumor.
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TREATMENT
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The treatment of this type of tumor depends on the stage of the cancer at the time
of diagnosis, the amount of tumor remaining after surgery to remove it or whether
the tumor has spread to other places in the body (metastases), age of the individual
at the time of diagnosis, recurrence, and response to chemotherapy and/or radiation
therapy.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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Suggested MER for
Evaluation:
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Clinical history and examination that describes the diagnostic features of the impairment;
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Copies of imaging reports describing the type and stage of the tumor;
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Suggested Listings for Evaluation:
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DETERMINATION
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LISTING
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REMARKS
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Meets
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13.04
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Meets 13.04 in adults if originating outside of the brain and has metastases or is
recurrent.
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13.13 A 3
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Meets the criteria in listings 13.13 A 3 if originating in the brain and has documented
metastases; also meets 13.13 A 3 in adults if it is recurrent cancer.
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113.03
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Meets the criteria in listing 113.03 in children when originating outside of the brain.
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113.13 C
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Meets the criteria in 113.13 C in children if originating in the brain and has documented
metastases; also meets 113.13 C in children if it is recurrent cancer.
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Equals
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or the
listings suggested to evaluate the claim. However, the decision to allow or deny the
claim rests with the adjudicator.
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