CLINICAL INFORMATION

Medical diagnosis: Relapse of chondrosarcoma of thoracic wall at 2nd-3rd right ribs.

Medical history:

A 59 year old male, diagnosed with 2nd degree chondrosarcoma of the cervical spine originating from the hyaline cartilage. The lesion expanded to D2 and developed in right thoracic cavity and spinal endochannel with medullary compression.

The patient had the following surgical procedure: D2 bilateral laminectomy, removal of posterior spinal neoplasia with medullary decompression and stabilization of the spinal column using the CD Horizon system. A transthoracic approach was performed for removal of the intrathoracic element and partial removal of the D2 soma. Neoplastic infiltration of the posterior thoracic wall remained without the possibility of radical removal.

He was later hospitalized for a hemivertebrectomy in order to complete the previous surgical procedure and recieved continuous cycles of adjuvant fractionated proton beam radiation therapy, with intensity modulated radiotherapy (IMRT) in order to control the cervical cordoma locally.

CAT scan of the cervicodorsal spine and chest showed relapse of the chondrosarcoma at the right 2nd-3rd ribs.

A second surgery was done to block resection - arched right perisubscapular incision. A segment of the thoracic wall was sculpted including the neoplastic mass. Visceral pleura was adhering to the parietal pleura and was left to cover the tumor. Block resection of the mass with microscopically wide margins. The pulmonary parenchyma was covered with tissue in apnea.

CAT scan showed signs of surgical procedure in right thoracic wall and removal of 2nd and 3rd ribs likely due to hemorrhagic infarction and an area of calcic density, air bubbles in subscapular adipose tissue, and decreased expansion of right lung in apical area. Right thoracic double drainage with residual flap remnants of homolateral apical pneumothorax. No significant hilar and mediastinic adenopathies. No pulmonary parenchymal nodular formations. Thickening of right perivertebral soft tissue in the area of the vertebral prosthesis.

Medical questions referred to Medical Opinion online consultation service:

Do you suggest any other additional therapy?

What is the prognosis?

To Medical Opinion:

Thank you for the online referral and copy of the medical records for this patient.This is a second opinion based on the attached clinical information, as requested on behalf of the patient by Medical Opinion.

 

Case History:

A 59 yr old man, suffering from locally recurrent chondrosarcoma.

His disease was diagnosed as a second degree chondrosarcoma of the cervical spine originating from the hyaline cartilage, expanding to D2 and developing in right thoracic cavity and spinal endochannel with medullary compression.

Following diagnosis his treatment was composed of 3 locoregional interventions:

A) Surgery which included D2 bilateral laminectomy, removal of posterior spinal neoplasia, with medullary decompression and stabilization of the spinal column using the CD Horizon system. Removal of the intrathoracic element of the tumor and partial removal of the D2 soma. Neoplastic infiltration of the posterior thoracic wall remained without the possibility of radical removal.

B) Hemivertebrectomy in order to complete the previous surgical procedure.

C) Adjuvant fractionated proton beam radiation therapy, with intensity modulated radiotherapy (IMRT).

Recurrent disease was diagnosed based on a CAT scan of the cervicodorsal spine and chest, which showed a relapse at the right 2nd-3rd ribs. A third surgical procedure was carried out obtaining block resection of the re-growing mass with microscopically wide margins.

CAT scan showed signs of surgical procedure in right thoracic wall and removal of 2nd and 3rd ribs where there is residual tissue due to hemorrhagic infarction, air bubbles in subscapular adipose tissue. Decreased expansion of right lung in apical area. Right thoracic double drainage with residual flap remnants of homolateral apical pneumothorax. No significant hilar and mediastinic adenopathies. No pulmonary parenchymal nodular formations. Thickening of right perivertebral soft tissue in the area of the vertebral prosthesis.

Question 1. : Do you suggest any other additional therapy?

My opinion is: not at the present time. I fully agree with the treatment procedures applied until now. The medical treatment policy with regards to sarcoma with no proof of distant metastases, is by radical surgery and adjuvant irradiation. Both procedures have already been applied in the case of this patient. Also with regards to the local recurrence of chondrosarcoma the approach should be limited to local procedures. The last surgical intervention achieved complete removal of the recurrent disease. CAT scan findings should be interpreted as mere postsurgical changes, with no indication for further surgery at the present stage. Furthermore, since the recurrence occurred within a previously irradiated area there is no indication for additional irradiation at the present time.

The present recurrence in spite of best treatment administered indicates a high risk for future recurrence. However, as yet there is no additional treatment which is recognized/ established to be effective in the adjuvant setup for this type of disease. Therefore, I cannot advice any chemotherapy at the present stage.

Question 2. : What is the prognosis?

There is still a hope for cure in spite of the relapse. I hope that indeed the last surgical intervention achieved eradication of all existent disease., There is a risk of additional local recurrence of this recurrent chondrosarcoma. We should take in consideration the nature of the residual disease to develop tumour, manifesting in shorter times to progression. Accordingly, this patient should be under close follow up including imaging procedures. Every local recurrence should be considered for re-operation, both for prolonging survival and for symptomatic control, mainly for prevention of neurological damage correlated with the spinal/ para-spinal location. There is a risk of distant metastases as well. He should therefore undergo elective and repeated lung imaging. In case of metastases to the lungs, thoracotomy should be strongly advocated with curative attempt.

Additional medical treatments, should be considered in case that non resectable disease develops compromising the well being of the patient.

Following are references of several examples:

1) Lancet Oncol. 2007 Jun;8(6):513-524 Opportunities for improving the therapeutic ratio for patients with sarcoma. Wunder JS, Nielsen TO, Maki RG, O´sullivan B, Alman BA.

" Biological data and preclinical studies support trials using inhibitors of hedgehog signalling in chondrosarcoma."

2) BMC Cancer. 2007 Mar 17;7:49. Tyrosine kinase inhibitor SU6668 represses chondrosarcoma growth via antiangiogenesis in vivo. Klenke FM, Abdollahi A, Bertl E, Gebhard MM, Ewerbeck V, Huber PE, Sckell A.

3) Int J Cancer. 2006 Sep 1;119(5):980-4 Zoledronic acid slows down rat primary chondrosarcoma development, recurrent tumor progression

after intralesional curretage and increases overall survival. Gouin F, Ory B, Rédini F, Heymann D.

4) Clin Cancer Res. 2005 Nov 15;11(22):8028-35. Estrogen signaling is active in cartilaginous tumors: implications for antiestrogen therapy as treatment option of metastasized or irresectable chondrosarcoma. Cleton-Jansen AM, van Beerendonk HM, Baelde HJ, Bovée JV, Karperien M, Hogendoorn PC.

5) J Neurooncol. 2005 Feb;71(3):333-4. Successful treatment of a chemoresistant tumor with temozolomide in an adult patient: report of a recurrent intracranial mesenchymal chondrosarcoma. Aksoy S, Abali H, Kiliçkap S, Güler N.

I wish that the patient will be cured by the optimal multidisciplinary treatment administered to him so far and that all these additional options will not be necessary. However, if I can be of any further help to you, please contact me through Medical Opinion.

Sincerely yours,

Dr----

Senior Oncologist