Cryoablation of extra-abdominal desmoid tumours: initial experience and results

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1 Cryoablation of extra-abdominal desmoid tumours: initial experience and results Poster No.: C-2542 Congress: ECR 2013 Type: Scientific Exhibit Authors: G. Tsoumakidou, J. Garnon, I. Enescu, J.-P. Bergerat, J.-E. Kurtz, A. Gangi; Strasbourg/FR Keywords: Interventional non-vascular, Oncology, Musculoskeletal soft tissue, CT, MR, Ablation procedures DOI: /ecr2013/C-2542 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 44

2 Purpose Desmoid tumors (also called deep or aggressive fibromatosis) are uncommon mesenchymal neoplasms with a fibrotic bandlike consistency. Though considered benign, with no potential of distant metastases, they are locally aggressive, present an infiltrative growth and have the tendency to recur locally (World Health organization). Desmoid tumours (DT) may affect all sites, including the extremities, trunk, and abdomen. They are characterised as: extra-abdominal (EAD), abdominal (AD), and intra-abdominal (IAD) (often associated with Gardner syndrome and familial adenomatous polyposis-fap). Fig. 1: (Right Fig.) EAD of the anterior thoracic wall (local recurrence after surgical excision). (Left Fig.) Sagittal T1W image showing the voluminous local recurrence. Page 2 of 44

3 References: Interventional Radiology, University Hospital of Strasbourg - Strasbourg/ FR Imaging Features Imaging of the DT relies mainly on CT and MRI. CT shows a soft tissue mass of variable attenuation and enhancement. The margins of the tumour may be indistinct because of infiltration of adjacent structures. Heterogeneous attenuation may be seen because of necrosis or degeneration. MRI is best suited for optimum evaluation of DT because it allows accurate depiction of their relationship with the adjacent structures. On T2W and Proton density images the signal intensity is usually intermediate, at times with the presence of hypointense bands corresponding to collagen bundles. Actively growing DT tend to have higher T2 signal due to higher cellularity. Fig. 2: (A, B) Axial and coronal T2W images showing a cervical EAD with intermediate signal intensity and low signal bands corresponding to collagen bundles. (C) Coronal T1W image after gadolinium injection. The tumour presents homogeneous intense enhancement. References: Interventional Radiology, University Hospital of Strasbourg - Strasbourg/ FR Page 3 of 44

4 Although the imaging of DT may be suggestive, histopathologic confirmation is needed in all cases before definitive treatment. Treatment options In selected asymptomatic patients with stable size DT, watchful waiting is considered the most appropriate treatment. Symptomatic DT need to be treated. -If feasible, surgical excision with wide margins is the treatment of choice for symptomatic desmoids. Surgery is often associated with postoperative radiotherapy to reduce the local recurrence rate in cases of involved surgical margins. -If resection is not possible because of close association with vital structures, external beam radiation, brachytherapy and systemic therapy (chemotherapy/antihormonal therapy) with or without surgical excision should be considered. Recurrence after surgical excision of DT is common (17-77%) and more frequent with EAD (30-50%) than IAD (15-30%). Though molecular determinants of desmoid recurrence still remain obscure, the #-Catenin deregulation has been commonly identified in sporadic desmoids and the S45F mutation has been considered as an important predictor of recurrence after surgery of the primary tumour. Page 4 of 44

5 Fig. 3: (Right Fig.)50 years old female patient presenting with voluminous local recurrence after surgical excision of an EAD on the left shoulder. (Left Fig.) Axial T2-STIR image showing the hyperintense lesion with the hypointense bands corresponding to collagen bundles. References: Interventional Radiology, University Hospital of Strasbourg - Strasbourg/ FR Image guided cryoablation has been used for the curative and palliative treatment of a variety of benign and malignant tumors. The third generation cryoablation equipment currently in use involves 17-G probes and relies on the application of the Joule-Thomson effect of gases to freeze (argon) and thaw (helium) tissue. The destructive effects of cryoablation can be grouped into two major mechanisms: cellular injury and vascular injury. Page 5 of 44

6 Fig. 4: The damaging effects of cryoablation on cells begin gradually as temperature falls. A. Crystal formation first occurs in the extracellular spaces creating an hyperosmotic environment that withdraws water from the intracellular space (cellular dehydration). B. At temperatures below -15 C intracellular ice formation begins causing rupture of the cellular membrane and cell death. References: Interventional Radiology, University Hospital of Strasbourg - Strasbourg/ FR The cryoablation zone can be monitored with imaging: hypodense ice ball on CT and signal void zone on MRI. Compared to surgery, cryoablation is less invasive, requires less analgesia and patient recovery is quicker. Page 6 of 44

7 Fig. 5: The cryoablation probes contain expansion chambers in which argon gas cools to -180#C, freezing the adjacent tissue. References: Interventional Radiology, University Hospital of Strasbourg - Strasbourg/ FR Purpose We herein present our experience with image guided cryoablation of extra-abdominal desmoids in six symptomatic patients whose tumors have failed to respond to standard therapy. Page 7 of 44

8 Images for this section: Fig. 1: (Right Fig.) EAD of the anterior thoracic wall (local recurrence after surgical excision). (Left Fig.) Sagittal T1W image showing the voluminous local recurrence. Page 8 of 44

9 Fig. 2: (A, B) Axial and coronal T2W images showing a cervical EAD with intermediate signal intensity and low signal bands corresponding to collagen bundles. (C) Coronal T1W image after gadolinium injection. The tumour presents homogeneous intense enhancement. Fig. 3: (Right Fig.)50 years old female patient presenting with voluminous local recurrence after surgical excision of an EAD on the left shoulder. (Left Fig.) Axial T2- Page 9 of 44

10 STIR image showing the hyperintense lesion with the hypointense bands corresponding to collagen bundles. Fig. 4: The damaging effects of cryoablation on cells begin gradually as temperature falls. A. Crystal formation first occurs in the extracellular spaces creating an hyperosmotic environment that withdraws water from the intracellular space (cellular dehydration). B. At temperatures below -15 C intracellular ice formation begins causing rupture of the cellular membrane and cell death. Page 10 of 44

11 Fig. 5: The cryoablation probes contain expansion chambers in which argon gas cools to -180#C, freezing the adjacent tissue. Page 11 of 44

12 Methods and Materials Institutional review board approval was obtained for this retrospective study. Patient informed consent was obtained before the procedure. From October 2008 till today, we have treated six patients (range: years old) with EAD tumours (five female, one male) using image-guided cryoablation. All patients had histologically confirmed EAD (either with percutaneous core or surgical biopsy). Decision to treat the included patients was taken in a multidisciplinary basis. All patients have been referred for cryoablation in an attempt to achieve local control of EAD that had failed to surgical therapy. No patient had received external beam radiation or brachytherapy. No patient had a history of Gardner or FAP syndrome. Lesions were located: shoulder (n=1), neck (n=2), anterior thoracic wall (n=2), pelvic wall (n=1). Lesion size ranged from 8 to 26 cm. Procedure All procedures were performed under general anaesthesia. In total, 8 cryoablations were performed in six patients. Cryoablation was performed in two consecutive sessions in one patient, while one patient was retreated for a residual lesion. Image guidance: CT-guidance was used in six cases and MR-guidance in 2 cases. Page 12 of 44

13 Fig. 6: Local recurrence after surgical resection of a EAD on the left shoulder. Thirteen cryoprobes are placed under CT-guidance for ablation of the tumour. Note the hypodense ice ball covering the majority (>90%) of the lesion (arrows). References: Interventional Radiology, University Hospital of Strasbourg - Strasbourg/ FR In one patient with a local recurrence on the pelvic wall after surgical removal of a pelvic tumour, and due to the proximity of the tumour to the pelvic organs and the sciatic nerve, the ablation was performed using a combined open surgical and CT-guided approach during the same session. Page 13 of 44

14 Fig. 7: Recurrence of a pelvic DT. The tumour was initially located on the posterior pelvic wall. (A, B) Axial T2W and T1W+C images, showing the recurrence on the pelvis, in contact with the pelvic organs and sciatic nerve. (C, D) The cryoablation was performed with combination of an open surgical and CT guided approach.(e, F) The monitoring of the ice ball was done strictly under CT guidance. The above procedure was done in collaboration with Prof. Hervé Lang (Prof. of Urology, University Hospital of Strasbourg) and his team. References: Interventional Radiology, University Hospital of Strasbourg - Strasbourg/ FR Cryoablation was performed with a range of 6 to 16 cryoprobes (Galil Medical Yokneam, Israel) depending on tumour size. Page 14 of 44

15 - In 4 patients repositioning of the cryoprobes was needed in order to accomplish better lesion coverage. Fig. 10 on page 22 - Ablation was performed in two consecutive sessions in a female patient with a 26 cm EAD located on the neck and extending to the upper posterior thoracic wall. Fig. 8: (A) Sagittal T2W image showing the voluminous recurrence of EAD occuping the cervical and posterior thoracic wall. (B, C) Axial and coronal T2-Blade image during percutaneous cryoablation of the cervical part of the lesion. Note the signal void ice ball. (D) MR follow up showing the retraction of the ablated cervical part (arrow) and persistence of the thoracic (non-treated) part of the EAD which was ablated in a seconded session under CT guidance (E). References: Interventional Radiology, University Hospital of Strasbourg - Strasbourg/ FR Two 10 minutes freezing cycles, separated by a 10 minutes passive thawing cycle were performed in all cases. Page 15 of 44

16 Fig. 9: Percutaneous CT guided cryoablation of a EAD on the neck.the anteromedial surface of the lesion in contact with the trachea and the neurovascular sheath was not covered by the ice ball, so as to avoid complications. References: Interventional Radiology, University Hospital of Strasbourg - Strasbourg/ FR Thermal Insulation: Sterile gloves filled with warm saline were placed on the skin in order to prevent it from freezing. Page 16 of 44

17 Fig. 11: Sterile gloves filled with warm saline are placed on the skin in order to prevent it from freezing. References: Interventional Radiology, University Hospital of Strasbourg - Strasbourg/ FR Furthermore, warm saline was injected through 22-G spinal needles in order to displace and actively insulate healthy structures in contact with the ablation zone (ice-ball). In the case of the anterior thoracic AED CO2 dissection of the brachial plexus was performed in order to passively insulate the latter. Page 17 of 44

18 Fig. 12: (A) Local recurrence after surgical excision of an EAD on the right anterior thoracic wall. (B) A 22 G spinal needle (arrow) was positioned under MR guidance in contact with the brachial plexus for CO2 dissection (passive insulation) of the latter. (C) T2 Blade image showing the complete coverage of the lesion of the signal void ice ball. References: Interventional Radiology, University Hospital of Strasbourg - Strasbourg/ FR Clinical and Imaging Follow-up: Clinical and imaging follow-up was performed on the 1st, 3rd, 6th, 9th month and every 6 months thereafter. Follow-up ranged from 3 to 50 months. For the last patient included, only a three months follow-up was available at the time of the study. Page 18 of 44

19 Images for this section: Fig. 6: Local recurrence after surgical resection of a EAD on the left shoulder. Thirteen cryoprobes are placed under CT-guidance for ablation of the tumour. Note the hypodense ice ball covering the majority (>90%) of the lesion (arrows). Page 19 of 44

20 Fig. 7: Recurrence of a pelvic DT. The tumour was initially located on the posterior pelvic wall. (A, B) Axial T2W and T1W+C images, showing the recurrence on the pelvis, in contact with the pelvic organs and sciatic nerve. (C, D) The cryoablation was performed with combination of an open surgical and CT guided approach.(e, F) The monitoring of the ice ball was done strictly under CT guidance. The above procedure was done in collaboration with Prof. Hervé Lang (Prof. of Urology, University Hospital of Strasbourg) and his team. Page 20 of 44

21 Fig. 8: (A) Sagittal T2W image showing the voluminous recurrence of EAD occuping the cervical and posterior thoracic wall. (B, C) Axial and coronal T2-Blade image during percutaneous cryoablation of the cervical part of the lesion. Note the signal void ice ball. (D) MR follow up showing the retraction of the ablated cervical part (arrow) and persistence of the thoracic (non-treated) part of the EAD which was ablated in a seconded session under CT guidance (E). Page 21 of 44

22 Fig. 9: Percutaneous CT guided cryoablation of a EAD on the neck.the anteromedial surface of the lesion in contact with the trachea and the neurovascular sheath was not covered by the ice ball, so as to avoid complications. Fig. 10: Percutaneous cryoablation of a AED recurrence on the anterior thoracic wall. (A) Sixteen cryoprobes were positioned under CT guidance to cover the superior right side of the lesion. (b) The cryoprobes were retrieved in order to cover the most superficial part of the lesion. (C) Sagittal CT reconstructed image showing the hypodense ice ball. (D) The inferior-left part of the lesion was not ablated and will be treated in a second session. Page 22 of 44

23 Fig. 11: Sterile gloves filled with warm saline are placed on the skin in order to prevent it from freezing. Fig. 12: (A) Local recurrence after surgical excision of an EAD on the right anterior thoracic wall. (B) A 22 G spinal needle (arrow) was positioned under MR guidance in Page 23 of 44

24 contact with the brachial plexus for CO2 dissection (passive insulation) of the latter. (C) T2 Blade image showing the complete coverage of the lesion of the signal void ice ball. Page 24 of 44

25 Results Ablation procedure Complete coverage of the tumour (>90% of the total lesion volume) was possible in three patinets. In two patients (desmoid tumour on the neck and the pelvic wall) the tumour was encasing neighboring neural structures and in order to avoid major complications the tumour coverage by the ice ball was incomplete. Fig. 9 on page In the patient with the anterior thoracic wall voluminous lesion, so far we have performed ablation of the superior half of the lesion, while the inferior half will be treated in a second session. All patients experienced significant tumour size reduction (>75%) and in four patients complete resolution of the lesionwas observed. In one patient (neck EAD) we surprisingly noted necrosis even of parts of the desmoid that were not covered by the ice ball. Fig. 13: Patient treated for a neck EAD. During the 3 months FU we surprisingly noted retraction and partial necrosis even of parts of the desmoid that were not covered by the ice ball (anteromedial surface). References: Interventional Radiology, University Hospital of Strasbourg - Strasbourg/ FR Page 25 of 44

26 Fig. 14: Axial and coronal T1+Gado images before and 6 and 9 months post treatent. Significant size reduction was noted on the pelvic DT that was treated with combined open surgical and CT-guided cryoablation. References: Interventional Radiology, University Hospital of Strasbourg - Strasbourg/ FR Complications: -One patient with the anterior thoracic wall EAD presented with a skin ulcer which was attributed to impaired vascular circulation (due to significant post-ablation oedema). The patient had no sign of infection and was treated conservatively. -One patient with the cervical EAD presented a Horner syndrome a few days post ablation (ptosis, myosis, anhydrosis) which was persisting on 3 months follow-up. Fig. 20 on page 35 No other major complication was noted. Hospitalization ranged from 3 to 7 days. Page 26 of 44

27 Clinical Follow-up All patients reported significant reduction of the pain level and mass volume posttreatment, while four patients were complete symptom-free three months post-treatment. Fig. 15: Figures showing the retraction of the EAD after percutaneous cryoablation (patient treated in two consecutive sessions). The patient was symptom free on short and long term follow up (50 months). References: Interventional Radiology, University Hospital of Strasbourg - Strasbourg/ FR Page 27 of 44

28 Fig. 16: Figures showing the reduction of the lesion volume on 6 months follow-up. References: Interventional Radiology, University Hospital of Strasbourg - Strasbourg/ FR Imaging Follow-up In all patients the initials MRI controls post treatment showed peripheral enhancement, which was represented an inflammatory reaction and postablation granulation tissue. Complete necrosis of the ablated lesion with no residual lesion after gadolinium injection was noted in four patients. In the patient with the shoulder EAD, MRI reaveled a persisting residual lesion of one cm, that was further successfully treated with MR-guided percutaneous cryoablation. Page 28 of 44

29 Fig. 17: (A) Axial T1W+C image. Residual tumour after percutaneous cryoablation of EAD on the left shoulder. (B) The patient was retreated with cryoablation of the residual lesion under MR-guidance. (C) Axial subtracted image after gadolinium enhancement showing the complete necrosis of the lesion. References: Interventional Radiology, University Hospital of Strasbourg - Strasbourg/ FR The patient with the pelvic wall EAD showed persistence of the uncovered areas in proximity to the sciatic nerve that were stable in size between the controls. The patient reported significant pain improvement and decision to follow her up closely was taken. Fig. 18: MR control of the cervicothoracic EAD before and after the percutaneous cryoablation showing complete resolution of the lesion. Page 29 of 44

30 References: Interventional Radiology, University Hospital of Strasbourg - Strasbourg/ FR Fig. 19: MR follow of the patient with the shoulder EAD. Complete resolution of the lesion is noted on the subtracted gadolinium enhanced images. References: Interventional Radiology, University Hospital of Strasbourg - Strasbourg/ FR Fig. 21: MRI on 3 months FU showed significant reduction of tumour volume. No mass effect and displacement of the trachea are noted. References: Interventional Radiology, University Hospital of Strasbourg - Strasbourg/ FR Page 30 of 44

31 Images for this section: Fig. 13: Patient treated for a neck EAD. During the 3 months FU we surprisingly noted retraction and partial necrosis even of parts of the desmoid that were not covered by the ice ball (anteromedial surface). Page 31 of 44

32 Fig. 14: Axial and coronal T1+Gado images before and 6 and 9 months post treatent. Significant size reduction was noted on the pelvic DT that was treated with combined open surgical and CT-guided cryoablation. Page 32 of 44

33 Fig. 15: Figures showing the retraction of the EAD after percutaneous cryoablation (patient treated in two consecutive sessions). The patient was symptom free on short and long term follow up (50 months). Fig. 16: Figures showing the reduction of the lesion volume on 6 months follow-up. Page 33 of 44

34 Fig. 17: (A) Axial T1W+C image. Residual tumour after percutaneous cryoablation of EAD on the left shoulder. (B) The patient was retreated with cryoablation of the residual lesion under MR-guidance. (C) Axial subtracted image after gadolinium enhancement showing the complete necrosis of the lesion. Fig. 18: MR control of the cervicothoracic EAD before and after the percutaneous cryoablation showing complete resolution of the lesion. Page 34 of 44

35 Fig. 19: MR follow of the patient with the shoulder EAD. Complete resolution of the lesion is noted on the subtracted gadolinium enhanced images. Fig. 20: One patient with a cervical EAD presented a Horner synrdome post ablation (ptosis, myosis, anhydrosis). Page 35 of 44

36 Fig. 21: MRI on 3 months FU showed significant reduction of tumour volume. No mass effect and displacement of the trachea are noted. Page 36 of 44

37 Conclusion Image guided cryoablation is an alternative treatment of EAD, when conventional treatments have failed. Because of its minimally invasive nature, cryoablation causes less damage to surrounding tissues, when compared with surgery. The use of high quality image guidance ensures the satisfactory coverage of the lesion, while spairing the neighboring healthy structures. Significant tumour size reduction and symptom regression can be safely achieved. Further research with long term follow up and larger samples are needed in order to validate its efficacy. Fig. 25: Interventional Radiology and Urology, University Hospital of Strasbourg, France Page 37 of 44

38 References: Interventional Radiology, University Hospital of Strasbourg - Strasbourg/ FR Page 38 of 44

39 Images for this section: Fig. 22 Page 39 of 44

40 Fig. 23: Combined open surgical and CT guided cryoablation of a pelvic wall EAD. With the contribution of the Urology team (Prof. Hervé Lang) Page 40 of 44

41 Fig. 24: Open surgical, CT guided cryoablation of DT. With the contribution of the Urology team (Prof. Hervé Lang). Page 41 of 44

42 References Lev D, Kotilingam D, Wei C, et al. (2007) Optimizing treatment of desmoid tumors. J Clin Oncol 25: Alexander J.F, Daniel T, Shohrae H, et al. (2008) Specific Mutations in the #-Catenin Gene (CTNNB1) Correlate with Local Recurrence in Sporadic Desmoid Tumors Am J Pathol 173(5): Ballo MT, Zagars GK, Pollack A, et al. (1999) Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy. J Clin Oncol 17: Alman BA, Li C, Pajerski ME, et al. (1997) Increased b-catenin protein and somatic APC mutations in sporadic aggressive fibromatoses (desmoid tumors). Am J Pathol 151: Abbas AE, Deschamps C, Cassivi SD, et al. (2004) Chest-wall desmoid tumors: results of surgical intervention. Ann Thorac Surg. 78: Phillips SR, A'Hern R, Thomas JM. (2004) Aggressive fibromatosis of the abdominal wall, limbs and limb girdles. Br J Surg 91: Gega M, Yanagi H, Yoshikawa R, et al. (2006) Successful chemotherapeutic modality of doxorubicin plus dacarbazine for the treatment of desmoid tumors in association with familial adenomatous polyposis. J Clin Oncol. 24: Dafford K, Kim D, Nelson A, et al. (2007) Extraabdominal desmoid tumors. Neurosurg Focus 22:E21. Okuno S (2006) The enigma of desmoid tumors. Curr Treat Options Oncol 7: Dinauer PA, Brixey CJ, Moncur JT, et al. (2007) Pathologic and MR imaging features of benign fibrous softtissue tumors in adults. Radiographics 27: Sorensen A, Keller J, Nielsen OS, et al. (2002) Treatment of aggressive fibromatosis: a retrospective study of 72 patients followed for 1-27 years. Acta Orthop Scand 73: Hansmann A, Adolph C, Vogel T, et al. (2004) Highdose tamoxifen and sulindac as first-line treatment for desmoid tumors. Cancer 100: Melis M, Zager JS, Sondak VK. (2008) Multimodality management of desmoid tumors: how important is a negative surgical margin? J Surg Oncol 98: Atwell TD, Farrell MA, Leibovich BC, et al. (2008) Percutaneous renal cryoablation: experience treating 115 tumors. J Urol 179: ; discussion Hinshaw JL, Shadid AM, Nakada SY, et al. (2008) Comparison of percutaneous and laparoscopic cryoablation for the treatment of solid renal masses. AJR Am J Roentgenol 191: Littrup PJ, Freeman-Gibb L, Andea A, et al. (2005) Cryotherapy for Page 42 of 44

43 breast fibroadenomas. Radiology 234: Beland MD, Dupuy DE, Mayo-Smith WW. (2005) Percutaneous cryoablation of symptomatic extraabdominal metastatic disease: preliminary results. AJR Am J Roentgenol 184: Callstrom MR, Atwell TD, Charboneau JW, et al. (2006) Painful metastases involving bone: percutaneous image-guided cryoablation-prospective trial interim analysis. Radiology 241: Kujak JL, Liu PT, Johnson GB, et al. (2010) Early experience with percutaneous cryoablation of extra-abdominal desmoid tumors Skeletal Radiol 39: Kasper B, Strobel P, Hohenberger P. (2011) Desmoid tumours: Clinical features and treatment options for advanced disease. The Oncologist 16: Page 43 of 44

44 Personal Information Tsoumakidou Georgia, MD, Non-Vascular Interventional Radiology University Hospital of Strasbourg, France Fig. 26 References: Interventional Radiology, University Hospital of Strasbourg Strasbourg/FR Page 44 of 44

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