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Hematopoietic Stem-Cell Transplantation for Brain Tumors
Autologous HSCT allows for escalation of chemotherapy doses above those limited by myeloablation and has been tried in patients with high-risk brain tumors in an attempt to eradicate residual tumor cells and improve cure rates. The use of allogeneic HSCT for solid tumors does not rely on escalation of chemotherapy intensity and tumor reduction, but rather on a graft-versus-tumor effect. Allogeneic HSCT is uncommonly used in solid tumors, and may be used if an autologous source cannot be cleared of tumor or cannot be harvested.

CNS Embryonal Tumors
Classification of brain tumors is based on both histopathologic characteristics of the tumor and location in the brain. Central nervous system (CNS) embryonal tumors are more common in children and are the most common brain tumor in childhood. They are primarily composed of undifferentiated round cells, with divergent patterns of differentiation. It has been proposed that these tumors be merged under the term “primitive neuroectodermal tumor” (PNET), however, histologically similar tumors in different locations in the brain demonstrate different molecular genetic alterations. Embryonal tumors of the CNS include medulloblastoma, medulloepithelioma, supratentorial PNETs (pineoblastoma, cerebral neuroblastoma, ganglioneuroblastoma), ependymoblastoma, and atypical teratoid/rhabdoid tumor (AT/RT).

Medulloblastomas account for 20% of all childhood CNS tumors. The other types of embryonal tumors are rare by comparison. Surgical resection is the mainstay of therapy with the goal being gross total resection with adjuvant radiation therapy, as medulloblastomas are very radiosensitive. Treatment protocols are based on risk stratification, as average or high risk. The average-risk group includes children older than 3 years, without metastatic disease, and with tumors that are totally or near totally resected (<1.5 cm² of residual disease). The high-risk group includes children aged 3 years or younger, or with metastatic disease, and/or subtotal resection (>1.5 cm2 of residual disease).

Current standard treatment regimens for average-risk medulloblastoma (postoperative craniospinal irradiation with boost to the posterior fossa followed by 12 months of chemotherapy) have resulted in 5-year overall survival (OS) rates of 80% or better.  For high-risk medulloblastoma treated with conventional doses of chemotherapy and radiotherapy, the average event-free survival at 5 years ranges from 34%–40% across studies.  Fewer than 55% of children with high-risk disease survive longer than 5 years. The treatment of newly diagnosed medulloblastoma continues to evolve, and in children under the age of 3, because of the concern of the deleterious effects of craniospinal radiation on the immature nervous system, therapeutic approaches have attempted to delay and sometimes avoid the use of radiation, and have included trials of higher-dose chemotherapeutic regimens with autologous HSCT.

Supratentorial PNETs (sPNET) are most commonly located in the cerebral cortex and pineal region. The prognosis for these tumors is worse than for medulloblastoma, despite identical therapies.  After surgery, children are usually treated similarly to children with high-risk medulloblastoma. Three- to 5-year OS rates of 40%–50% have been reported, and for patients with disseminated disease, survival rates at 5 years range from 20%–30%.

Recurrent childhood CNS embryonal tumor is not uncommon, and depending on which type of treatment the patient initially received, autologous HSCT may be an option. For patients who receive high-dose chemotherapy and autologous HSCT for recurrent embryonal tumors, objective response is 50%–75%; however, long-term disease control is obtained in fewer than 30% of patients, and is seen primarily in patients in first relapse with localized disease at the time of relapse.

Ependymoma
Ependymoma is a neuroepithelial tumor that arises from the ependymal lining cell of the ventricles and is therefore usually contiguous with the ventricular system. In children, the tumor typically arises intracranially, while in adults, a spinal cord location is more common. Ependymomas have access to the cerebrospinal fluid and may spread throughout the entire neuroaxis.  Ependymomas are distinct from ependymoblastomas due to their more mature histologic differentiation. Initial treatment of ependymoma consists of maximal surgical resection followed by radiotherapy. Chemotherapy usually does not play a role in the initial treatment of ependymoma. However, disease relapse is common, typically occurring at the site of origin. Treatment of recurrence is problematic; further surgical resection or radiation therapy is usually not possible. Given the poor response to conventional-dose chemotherapy, high-dose chemotherapy with autologous HSCT has been investigated as a possible salvage therapy.

Note: Other CNS tumors include astrocytoma, oligodendroglioma, and glioblastoma multiforme. However, these tumors arise from glial cells and not neuroepithelial cells. Thus, they are not considered PNETs. These tumors are considered separately in the Autologous Stem-Cell Transplantation for Malignant Astrocytomas and Gliomas medical policy.

Note: Due to their neuroepithelial origin, peripheral neuroblastoma and Ewing's sarcoma may be considered PNETs. However, these peripheral tumors are considered separately in the Hematopoietic Stem-Cell Transplantation for Solid Tumors of Childhood medical policy.

Embryonal tumors of the CNS 

Autologous hematopoietic stem-cell transplantation may be considered medically necessary to treat recurrent embryonal tumors of the CNS.

Autologous hematopoietic stem-cell transplantation may be considered medically necessary as consolidation therapy for previously untreated embryonal tumors of the central nervous system (CNS) that show partial or complete response to induction chemotherapy, or stable disease after induction therapy (see Policy Guidelines).

Tandem autologous hematopoietic stem-cell transplant is investigational to treat embryonal tumors of the CNS.

Allogeneic hematopoietic stem-cell transplantation is investigational to treat embryonal tumors of the CNS.

Ependymoma 
Autologous, tandem autologous and allogeneic hematopoietic stem-cell transplant is investigational to treat ependymoma.

For State and School Employee subscribers, all bone marrow/stem cell transplants must be certified as medically necessary by the Plan’s Utilization Review Vendor, CareAllies. No benefits will be provided for any transplant procedure unless prior approval for the transplant is obtained from CareAllies.

In general, use of autologous hematopoietic stem-cell transplantation for previously untreated medulloblastoma has shown no survival benefit for those patients considered to be at average risk (i.e., patient age older than 3 years, without metastatic disease, and with total or near total surgical resection [<1.5 cm2 residual tumor]) when compared to conventional therapies.

Investigative service is defined as the use of any treatment procedure, facility, equipment, drug, device, or supply not yet recognized by certifying boards and/or approving or licensing agencies or published peer review criteria as standard, effective medical practice for the treatment of the condition being treated and as such therefore is not considered medically necessary.

The coverage guidelines outlined in the Medical Policy Manual should not be used in lieu of the Member's specific benefit plan language.

6/25/2004: Code Reference section completed

11/18/2004: Review by MPAC, no changes

10/26/2005: Code Reference section updated, CPT codes 38230, G0355 - G0364 added to the covered table, 38204, 86812 - 86822 added to the non-covered table, J9000 - J9999 deleted, ICD9 procedure codes 41.01, 41.09 added to the covered table, 41.02, 41.03 added to the non-covered table

3/21/2006: Coding updated. CPT4/HCPCS revisions added to policy

5/21/2007: Policy reviewed, description of syngeneic and allogeneic donor types added. No change to policy statements

12/20/2007: Coding updated per 2008 CPT/HCPCS revisions

5/21/2008: Policy reviewed, no changes

04/26/2010: Title changed from “High-Dose Chemotherapy with Autologous Stem-Cell Support for Primitive Neuroectodermal Tumors (PNET) of the CNS and Ependymoma” to “Hematopoietic Stem-Cell Transplantation for CNS Embryonal Tumors and Ependymoma.”  The term “PNET” was changed to “embryonal tumors” throughout the policy.  Policy description updated. Policy statement changed to state that autologous consolidation therapy in patients with previously untreated embryonal tumors showing complete or partial response to, or stable disease after, induction therapy is now considered medically necessary. Supporting explanation added to the policy guidelines. Policy statement reworded and ependymoma and embryonal CNS tumors are addressed separately. FEP and State and School Employee verbiage added to the Policy Exceptions section. Added new CPT codes 86825 and 86826.  Removed CPT codes 86812, 86813, 86816, 86817, 86821, and 86822 from the non-covered table. Added HCPCS S2140 and S2142 to the non-covered table. Deleted HCPCS G0265, G0266, and G0267 from the code section as these codes were deleted on 12/31/2007.

12/28/2010:  Policy reviewed; no changes.

01/17/2012:  Policy reviewed; no changes.

03/13/2013:  Policy reviewed; no changes.

The code(s) listed below are ONLY covered if the procedure is performed according to the "Policy" section of this document.  

Covered Codes

This is not an all-inclusive list of non-covered procedure codes.

The code(s) listed below and ANY code not listed in the previous section are considered non-covered for this procedure. 

Non-Covered Codes

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