DI 23022.226:
Malignant Ectomesenchymoma
Effective Dates: 01/10/2014 - Present- Effective Dates: 11/20/2017 - Present
- TN 10 (01-14)
- DI 23022.226 Malignant Ectomesenchymoma
- COMPASSIONATE ALLOWANCE INFORMATION
MALIGNANT ECTOMESENCHYMOMA- MALIGNANT ECTOMESENCHYMOMA
- ALTERNATE NAMES
- Mature Ectomesenchymoma; Gangliorhabdomyosarcoma; Rhabdomyosarcoma with Ganglionic Differentiation
- DESCRIPTION
- Malignant Ectomesenchymoma is a rare, potentially aggressive tumor (a type of primitive neuroectodermal tumor or PNET). 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, PHYSICAL FINDINGS, AND ICD-9-CM CODING
- Diagnostic testing: Testing for malignant ectomesenchymoma tumors includes imaging studies (CT, MRI, or nucleide scans such as liver-spleen scans); spinal tap for cerebrospinal fluid cytology; urinary catecholamines; and bone marrow biopsy.
- Physical findings: Depends on the location of the tumor.
- ICD-9: 191.X and other codes, depending upon site
- ONSET AND PROGRESSION
- 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.
- TREATMENT
- 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:
- * Clinical history and examination that describes the diagnostic features of the impairment.
- * Copies of imaging reports describing the type and stage of the tumor.
- * Laboratory findings.
- * Biopsy reports.
- Suggested Listings for Evaluation:
- DETERMINATION
- LISTING
- REMARKS
- Meets Listing
- 13.04
13.13A113.13A2- 13.13A3
- 113.03
113.13- 113.13C
Meets 13.13A1/113.13 in adults and children if originating in the brain and has documented metastases. Meets 13.13A2 in adults if it is recurrent cancer. Meets 113.03 in children when originating outside of the brain. Meets 13.04 in adults if originating outside of the brain and has metastases.- Meets 13.13A3/113.13C in adults and children if originating in the brain and has documented metastases; also meets 13.13A3/113.13C in adults/children if it is recurrent cancer. Meets 113.03 in children when originating outside of the brain. Meets 13.04 in adults if originating outside of the brain and has metastases or is recurrent.
- Equals Listing
13.13A2Medically equals 13.13A2 in children if it is in the brain and recurrent.-
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- * Adjudicators may, at their discretion, use the Medical Evidence of Record or Listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.
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