Our Summary

This research paper discusses a case of a rare cancer called malignant peripheral nerve sheath tumor (MPNST) that started from a nerve in the right arm and then spread to major veins and the heart. The patient, a 39-year-old female, had a history of an inherited disorder called neurofibromatosis type 1. She was initially treated with chemotherapy, and surgery was done to remove the tumor and the affected nerve. After the surgery, she also received radiation therapy. However, two years later, a scan revealed that the cancer had come back and was now growing in a vein leading to the heart. This was a life-threatening situation, so doctors performed an emergency surgery to cut off the vein’s base and remove the cancerous clot that had reached the right side of the heart. Despite this, the cancer in the vein continued to grow, so the patient underwent a more radical surgery to remove the whole right upper limb to get rid of the cancer. One year after this surgery, the cancer spread to a muscle in the right side of the chest, which was also safely removed. The patient is currently alive and free of cancer, two years after her major surgery. The paper concludes that in cases where cancer has previously spread to veins, it can return as a large clot in major blood vessels.

FAQs

  1. What is a malignant peripheral nerve sheath tumor (MPNST)?
  2. How is a malignant peripheral nerve sheath tumor (MPNST) usually treated?
  3. What complications can arise from a malignant peripheral nerve sheath tumor (MPNST)?

Doctor’s Tip

A doctor might advise a patient undergoing tumor resection to follow up closely with their healthcare team to monitor for any signs of recurrence, especially in cases where there was previous venous invasion. It is important to report any new symptoms or changes in health status promptly to ensure timely intervention if needed. Additionally, maintaining a healthy lifestyle and following any recommended post-operative care instructions can help support recovery and reduce the risk of further complications.

Suitable For

Patients with malignant peripheral nerve sheath tumors (MPNST) are typically recommended tumor resection, especially if the tumor is large, invasive, or causing symptoms. In the case described above, the patient underwent wide tumor resection and concomitant radial nerve resection to remove the tumor and prevent further spread. In cases where the tumor has invaded major veins or is causing life-threatening complications, urgent surgical intervention may be necessary to remove the tumor thrombus and prevent further complications. Additionally, adjuvant chemotherapy and radiotherapy may be recommended to help reduce the risk of recurrence and improve outcomes.

Timeline

Before tumor resection:

  • Patient presents with symptoms of a soft tissue sarcoma, in this case a malignant peripheral nerve sheath tumor (MPNST)
  • Patient undergoes adjuvant chemotherapy
  • Patient undergoes wide tumor resection and radial nerve resection
  • Postoperative radiotherapy is performed
  • Histological evaluation reveals venous invasion
  • 2-year follow-up CT reveals recurrent tumor thrombus extending to the heart

After tumor resection:

  • Urgent operation is performed to remove the intravenous tumor thrombus extending to the heart
  • Remaining tumor in the subclavian vein increases in size 3 months after thrombectomy
  • Extended forequarter amputation is performed to achieve surgical remission
  • New metastasis to the right diaphragm is safely resected
  • Patient remains alive without evidence of disease 2 years after extended forequarter amputation

What to Ask Your Doctor

  1. What is the goal of tumor resection in my case?
  2. What are the risks and potential complications associated with the surgery?
  3. Will I need any additional treatments, such as chemotherapy or radiation therapy, after the resection?
  4. How long is the recovery process expected to be, and what can I expect during the recovery period?
  5. Are there any specific lifestyle changes or precautions I should take after the surgery?
  6. How frequently will I need follow-up appointments and imaging scans to monitor for any recurrence or complications?
  7. What is the likelihood of the tumor returning after the resection, and what are the signs and symptoms I should watch out for?
  8. Are there any clinical trials or alternative treatment options available for my specific type of tumor?
  9. Will the surgery affect my mobility or quality of life in any way?
  10. What is the long-term prognosis for my condition following the tumor resection?

Reference

Authors: Hirozane T, Nakayama R, Yamaguchi S, Mori T, Asano N, Asakura K, Kikuta K, Kawaida M, Sasaki A, Okita H, Nakatsuka S, Ito T. Journal: World J Surg Oncol. 2022 Jan 7;20(1):8. doi: 10.1186/s12957-021-02473-2. PMID: 34996471