MALIGNANT ECTOMESENCHYMOMA

Mature Ectomesenchymoma; Gangliorhabdomyosarcoma; Rhabdomyosarcoma with Ganglionic Differentiation

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.

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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  Bone marrow biopsy.

Physical findings: Depends on the location of the tumor.

ICD-9: 191.X and other codes, depending upon site

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.

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.

SUGGESTED PROGRAMMATIC ASSESSMENT*

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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  Laboratory findings; and

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  Biopsy reports.

Suggested Listings for Evaluation:

REMARKS

Meets 13.04 in adults if originating outside of the brain and has metastases or is recurrent.

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.

Meets the criteria in listing 113.03 in children when originating outside of the brain.

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.