ABSTRACT: Desmoids are a benign connective tissue tumor that can grow aggressively, causing discomfort and impairing function. Surgical resection can incur significant morbidity, and there is a high rate of recurrence due to microscopic infiltration despite negative margins. Cryoablation has potential to treat with decreased morbidity, shorter recovery times, less anesthesia, and a decreased requirement for postoperative narcotics. In prior series, cyroablation has demonstrated utility as both curative and salvage/palliative therapy for symptomatic desmoids. Here, we describe 3 patients with symptomatic extra-axial desmoids treated with cryoablation who had been passed over for surgical resection, and describe their postprocedural clinical courses to date. Our experience suggests cryoablation is a viable treatment alternative for desmoids of all sizes, and that staged treatment may be a practical approach to achieving no macroscopic disease in large desmoid lesions.
IO Learning: 2020;8:E34-E38. Epub 2020 April 14.
Key words: cryoablation, desmoid, fibromatosis
Desmoid fibromatosis is a locally aggressive and often infiltrative proliferation of myofibroblasts derived from connective tissue. Although considered benign, desmoids can present as a burdensome symptomatic growth that is difficult to treat surgically due to its infiltrative properties. Wide surgical resection with negative margins has yielded recurrence rates as high as 50%.1 Postoperative complications and morbidity are also important considerations when evaluating surgical intervention and alternatives. Locoregional therapies have been described as less-invasive alternatives for desmoid treatment, including thermal ablation, stereotactic radiation, and magnetic resonance (MR)-guided focused ultrasound. Cryoablation has demonstrated utility as both curative and salvage/palliative therapy, allowing ablation of large lesions with potential for less postoperative pain and morbidity.2 We describe 3 patients with symptomatic desmoids treated with cryoablation who had been passed over for surgical resection, and describe their postprocedural clinical courses to date.
Case #1. Our first case involved a 55-year-old woman with a history of follicular lymphoma in remission and left arthroscopic rotator cuff repair 2 years prior to presentation. She noted an “abnormal lump” behind her left shoulder 1 year prior to presentation. Over time, she experienced increasing discomfort and bulk symptoms restricting her range of motion. MR imaging demonstrated a 6.5 x 3.1 x 5.1 cm infiltrative soft tissue mass; later biopsy proved it to be a desmoid tumor (Figure 1A, area bracketed by arrows). The mass appeared to arise from the left deltoid muscle, invading or displacing the adjacent infraspinatus muscle. A CT-guided cryoablation was performed using the Icefx Cryoablation System (Boston Scientific). Two IceForce 2.1 CX needles and two IceRod 2.1 CX needles were utilized to perform a 10-minute/8-minute/10-minute freeze/thaw/freeze cycle (Figures 1B and 1C). In all cases, a bag or glove filled with warm saline was utilized to prevent injury to overlying skin. Following the procedure, the patient developed significant left arm lymphedema in addition to expected postprocedure pain. This resolved within weeks after physical therapy at our lymphedema clinic. One-month follow-up MR imaging demonstrated less than 10% residual viable tumor, with a large area of necrosis. The overall size of the mass had yet to noticeably decrease. There was a thin rim of enhancing soft tissue injury surrounding 6.1 cm of necrosis and hemorrhage, as well as one definitive focus of residual desmoid (Figure 1D; arrow).
At 4 months, the area of necrosis decreased to 1.9 cm, the adjacent infraspinatus and deltoid muscles demonstrated considerable healing, and two foci of residual tumor were visible in the deltoid (Figure 1E; arrows). The patient’s presenting pain had resolved and the range of motion returned to normal. MR imaging at 6.5 months (not shown) demonstrated slight interval growth of the residual lesions to 3.1 cm each. As such, repeat cryoablation was performed with three IceForce 2.1 CX needles (Figure 1F). Follow-up MR imaging at 6 weeks (Figure 1G) demonstrated a new 6.6 cm bilobed area of necrosis and hemorrhage, which nearly resolved at 14 weeks (Figure 1H). There was no nodular enhancement to suggest residual tumor. Muscle edema suggested continued healing, and the patient was nearly pain free.
Case #2: Our second patient was a 50-year-old man with hypertension and coronary artery disease who presented with a painful chest wall mass that restricted some motion and activities, including driving. He had no history of surgery or trauma to the area. CT demonstrated a 3.8 x 6.3 x 7.2 cm mass superficial to his left pectoralis major (Figure 2A; arrow). Ultrasound-guided biopsy confirmed a desmoid tumor. Cryoablation was performed under CT guidance using one IceForce 2.1 CX probe and two IcePearl 2.1 CX probes. A similar 10-minute/8-minute/10-minute freeze/thaw/freeze cycle was performed (Figure 2B; ice ball visible after probe removal). Six weeks post treatment, subtraction MR imaging showed more than 90% reduction in viable tumor volume without foci definitive for residual tumor (Figure 2C). At his 6-month postoperative evaluation, the patient demonstrated improved range of motion and minimal discomfort. Further follow-up imaging is pending.
Case #3: Our third patient was a 27-year-old woman with a large mass behind her right calf, without history of prior surgery or trauma. The mass had been growing since it was first noticed 2 years prior, and was misdiagnosed as either a hemorrhagic Baker’s cyst or hematoma. There was no improvement with physical therapy and attempted drainages, and eventual biopsy proved a desmoid. Systemic sorafenib was initiated; however, the patient developed skin eruptions consistent with erythema multiforme, requiring hospitalization. After resolution, she presented via oncology referral to our clinic for local therapy.
MR imaging 2 months prior to ablation demonstrated a 11.0 x 5.8 x 5.1 cm, heterogeneously enhancing, well-circumscribed mass within the gastrocnemius muscle, displacing the soleus anteriorly (Figures 3A and 3B). Of note, the mass displaced the medial sural cutaneous nerve posteriorly, which is immediately adjacent. Under CT guidance, three IceForce 2.1 CX and one IceRod 2.1 CX cryoablation probes were placed. A single 10-minute/8-minute/10-minute freeze/thaw/freeze cycle was performed (Figure 3C). The IceRod probe and a single IceForce probe were removed, and the remaining two IceForce probes were withdrawn into the more caudal portion of the mass; a second freeze/thaw/freeze cycle was performed and the remaining cryoprobes were then removed.
The patient was discharged the same day, with in-home physical therapy. Follow-up MR imaging 40 days later demonstrated necrosis of the majority of the mass (Figures 3D and 3E). The thin rim of circumferential tissue likely represents a combination of healing soft tissue and residual desmoid tumor (Figure 3E). At this time, the patient was ambulating without difficulty and had nearly symmetrical muscle strength; however, she reported a small area of numbness overlying the lateral ankle and extending down to the right fifth toe, with tingling at the margins. Future MR imaging scans are planned at 3-month follow-up, and we anticipate a second procedure to ablate any residual tumor.
Historically, desmoid tumors have been approached with difficulty and limited treatment options. Depending on location, surgical resection incurs significant morbidity and high recurrence rates due to microscopic infiltration despite negative margins. Radiation therapy and MR-guided focused ultrasound have been used for locoregional control, and have their own comorbidities. Cryoablation is a viable alternative to these therapies, with the potential for shorter recovery times, less anesthesia and narcotics intraoperatively and postoperatively, as well as improved precision with direct visualization of ice ball formation.
One of our patients exhibited significant postoperative lymphedema of the affected arm that resolved, while another patient demonstrated sensory neuropraxia, which was expected. None of our patients experienced motor neuropraxia; however, this remains a significant concern in treating extra-axial desmoids with ablation. In a larger, retrospective study of 18 patients with 31 ablations, no major complications were reported,3 while another study including 23 patients and 30 ablations resulted in 2 patients with significant neuropraxia.4
Overall, cryoablation appears to be an effective alternative to surgery for both primary and palliative treatment. Of note, there may be particular value in staging treatment in large desmoids, with an initial debulking ablation that induces necrosis in a large volume of the mass, allowing for healing and convalescence of the surrounding tissue and residual desmoid tumor, and a second ablation that aims to treat all tissue visible by MR. While microscopic infiltration makes local recurrence likely, close surveillance with ablations at appropriate intervals is a promising approach to control this difficult diagnosis.
Our patients demonstrated improved quality of life and good tumor control with low morbidity. In agreement with the conclusions of larger cohort studies,3-5 we consider cryoablation to be a feasible, effective, and low-morbidity option for the first-line treatment and management of desmoid tumors.
Desmoid fibromatosis is an aggressive benign neoplasm, difficult to treat due to its high recurrence rate and microscopic infiltration. In choosing a locoregional modality as a first-line or alternative therapy to surgical resection, image-guided cryoablation can decrease viable tumor and tumor bulk, improving symptoms with relatively low patient morbidity. Our patients continue to demonstrate improved tumor control and quality of life with comparatively low morbidity. These cases, taken in context with other recently published larger retrospective cohorts, suggest that cryoablation is a promising modality for low-morbidity treatment of symptomatic desmoid lesions for both curative and palliative outcomes.
Acknowledgment. Special thanks to Sean Maratto, MD, Suehyb Alkhatib, MD, MS, and Michael Chorney, MD for assistance with manuscript preparation.
1. Ghanouni P, Dobrotwir A, Bazzocchi A, et al. Magnetic resonance-guided focused US treatment for extra-abdominal desmoid tumors: a retrospective multicenter study. Eur Radiol. 2017;27:732-740.
2. Garnon J, Koch G, Caudrelier J, Tsoumakidou G, Cazzato RL, Gangi A. Expanding the borders: image-guided procedures for the treatment of musculoskeletal tumors. Diagn Interv Imaging. 2017;98:635-644.
3. Schmitz JJ, Schmit GD, Atwell TD, et al. Percutaneous cryoablation of extraabdominal desmoid tumors: a 10-year experience. Am J Roentgenol. 2016;207:190-195.
4. Tremblay K, Lea WB, Neilson JC, King DM, Tutton SM. Percutaneous cryoablation for the treatment of extra-abdominal desmoid tumors. J Surg Oncol. 2019;120:366-375.
5. Kujak KL, Liu PT, Johnson GB, Callstrom MR. Early experience with percutaneous cryoablation of extra-abdominal desmoid tumors. Skeletal Radiol. 2009;39:1785-1782.
From the Pennsylvania Hospital, the University of Pennsylvania Health System, Philadelphia, Pennsylvania.
Disclosure: The authors have completed and returned the ICMJE Form for Disclosure of Potential Conflicts of Interest. The authors report no conflicts of interest regarding the content herein.
Address for Correspondence: Ben Hammelman, MD, MedED, Pennsylvania Hospital, Scheidt Building, 2nd Floor, 800 Spruce Street, Philadelphia, PA 19107. Email: BenHammelman@gmail.com.