From the Guidelines
The treatment of gastrointestinal neuroectodermal tumor (GNET) primarily involves a multidisciplinary approach, including surgical resection with wide margins as the first-line approach, as emphasized by the most recent guidelines 1. The goal of treatment is to achieve complete surgical removal of the tumor with negative margins, which offers the best chance for long-term survival.
- For localized disease, surgery alone may be sufficient, but due to the aggressive nature of these rare tumors, a multidisciplinary approach is often necessary, involving pathologists, radiologists, surgeons, medical oncologists, and other specialists 1.
- Adjuvant chemotherapy regimens may be considered, although the specific regimen may vary depending on the tumor characteristics and patient factors.
- For metastatic or unresectable disease, targeted therapies may be considered, particularly those targeting the EWSR1-CREB1 fusion protein, which is characteristic of GNET.
- Radiation therapy may be employed for local control in cases where complete surgical resection is not possible or for recurrent disease.
- Close surveillance following treatment is essential, with imaging studies every 3-6 months for the first 2-3 years, as these tumors have a high recurrence rate.
- The treatment approach should be individualized based on tumor location, size, stage, and patient factors, ideally managed by a multidisciplinary team with experience in rare gastrointestinal malignancies 1. A multidisciplinary treatment planning is necessary, involving a team of specialists, to ensure the best possible outcome for patients with GNET 1.
From the Research
Treatment Options for GNET
The treatment for gastrointestinal neuroectodermal tumor (GNET) is primarily focused on surgical resection, with adjuvant therapy considered in certain cases. Key points to consider include:
- Surgical resection is the mainstay of treatment for GNET, as it provides the best chance for cure 2, 3.
- Adjuvant chemotherapy may be considered in cases where the tumor is malignant or has a high risk of recurrence, with platinum-based chemotherapy showing promise in some studies 4, 5.
- Targeted chemotherapy based on specific tumor pathways is being developed, which may offer new treatment options for GNET patients 3.
Chemotherapy Regimens
Some chemotherapy regimens that have been used to treat GNET include:
- Cisplatin and etoposide, which have shown efficacy in achieving disease-free survival in some patients 4.
- CADO-CVP (cyclophosphamide, vincristine, doxorubicin, cisplatin, and etoposide), which has been used in combination with surgical resection and other therapies to treat metastatic GNET 5.
- Platinum-anthracycline based chemotherapy, which has shown initial shrinkage of liver metastasis in some patients 5.
Challenges and Future Directions
Despite these treatment options, GNET remains a challenging disease to manage due to its rarity and aggressive nature. Further research is needed to develop standardized treatment protocols and explore novel therapeutic approaches for this malignancy 2, 3.