|Year : 2021 | Volume
| Issue : 5 | Page : 1286-1288
Percutaneous ultrasound-guided radiofrequency ablation for giant desmoid tumors of the intra-abdominal cavity in a patient with Gardner syndrome
Liping Wang1, Dong Xu2, Liyu Chen2, Pintong Huang3
1 Department of Ultrasonography, The Second Affiliated Hospital of Zhejiang University School of Medicine; Department of Interventional Ultrasound, Cancer Hospital of the University of Chinese Academy of Sciences (Zhejiang Cancer Hospital), Hangzhou, Zhejiang, China
2 Department of Interventional Ultrasound, Cancer Hospital of the University of Chinese Academy of Sciences (Zhejiang Cancer Hospital), Hangzhou, Zhejiang, China
3 Department of Ultrasonography, The Second Affiliated Hospital of Zhejiang University School of Medicine, Zhejiang, China
|Date of Submission||23-Feb-2021|
|Date of Acceptance||07-Oct-2021|
|Date of Web Publication||27-Nov-2021|
Department of Ultrasonography, The Second Affiliated Hospital of Zhejiang University School of Medicine, No. 88 Jiefang Road, Hangzhou, Zhejiang - 310009
Source of Support: None, Conflict of Interest: None
Gardner syndrome (GS) is a rare variant of familial adenomatous polyposis, leading to numerous intra- and extracolonic lesions. Extracolonic lesions of GS are most common with desmoid tumors (DTs) in the abdominal wall, intra-abdominal cavity, and mesentery. Surgery remains the primary treatment for DTs; however, the patients are challenged with the high recurrence rate after surgical resection, and wide resection often results in debilitating loss of function. This study presents a case of a 47-year-old female with GS who had undergone total colectomy and ultra-low anastomosis of the ileal anal canal, and she developed giant DTs in the intra-abdominal cavity. The patient underwent ultrasound-guided percutaneous radiofrequency ablation (RFA) for intra-abdominal DTs in September 2014, October 2015, and January 2016. Palliative RFA significantly slowed the progression of the tumor and improved the symptoms of abdominal compression; thus, it is a possible therapeutic option for intra-abdominal unresectable DTs in patients with GS.
Keywords: Desmoid tumors, Gardner syndrome, radiofrequency ablation, ultrasound-guided
|How to cite this article:|
Wang L, Xu D, Chen L, Huang P. Percutaneous ultrasound-guided radiofrequency ablation for giant desmoid tumors of the intra-abdominal cavity in a patient with Gardner syndrome. J Can Res Ther 2021;17:1286-8
|How to cite this URL:|
Wang L, Xu D, Chen L, Huang P. Percutaneous ultrasound-guided radiofrequency ablation for giant desmoid tumors of the intra-abdominal cavity in a patient with Gardner syndrome. J Can Res Ther [serial online] 2021 [cited 2022 Jan 24];17:1286-8. Available from: https://www.cancerjournal.net/text.asp?2021/17/5/1286/331308
| > Introduction|| |
Gardner syndrome (GS) is a rare and intractable variant of familial adenomatous polyposis (FAP), characterized by the triad: colonic polyps, multiple osteomas, and desmoid tumors (DTs), while the malignant transformation of colonic polyps and intra-abdominal DTs has become a major cause of death in patients with GS., Surgical resection remains the primary therapy for DTs; however, DTs have high disease recurrence after surgery and debilitating loss of function due to wide resection. In this rare case, we have presented radiofrequency ablation (RFA) as a palliative treatment option for intra-abdominal unresectable DTs. To our best knowledge, no case of RFA for giant DTs of the intra-abdominal cavity in a patient with GS has ever been reported in previous studies.
| > Case Report|| |
A 47-year-old female diagnosed with colon cancer with FAP underwent total colectomy and ultra-low anastomosis of the ileal anal canal in Zhejiang Cancer Hospital in 2004. However, symptoms, such as abdominal distension, constipation, bloody stool, and high fever, have occurred since 2009, after that she returned to the hospital. Ultrasound imaging suggested multifocal lesions in the intra-abdominal cavity, and multiple intra-abdominal tumors were confirmed by laparotomy (mainly solid in the right middle and lower abdomen) with a size of 12 cm × 12 cm × 10 cm. The tumors' surface was porcelain white, and part of the small intestinal wall was invaded. The mass was fixed with a hard texture and developed upward to the root of the mesenteriolum, among which vascular pulsation could be felt. The small intestine in the right middle and lower abdomen circled the mass, and the mesenteriolum was distinctly shortened, showing no mesentery.
Part of the peritoneal thickening tissue was excised for intraoperative freezing, and the report is shown as follows: collagen fiber tissue in the left lower abdominal wall showed tumor-like hyperplasia. Intraoperative ultrasonography showed many blood vessels in the right middle and lower abdominal mass and several large blood vessels in the mass extending to the root of the mesenteriolum. Combined with a medical history and intraoperative conditions, intraoperative diagnosis considered FAP after surgery, and tumor-like hyperplasia occurring in the abdominal wall and mesenteriolum fibrous tissue considered GS [Figure 1].
|Figure 1: The final pathological result of the specimen of the peritoneal thickening tissue excised by laparotomy as follows: fibroblasts and myofibroblasts hyperplasia in bundles, accompanied by collagen fibers. Combined with the patient's medical history, it was diagnosed as Gander's syndrome|
Click here to view
During the operation, it was difficult to eradicate the tumor; otherwise, total small-bowel resection might be performed. The patient's state of illness was explained in detail to the patient's family, which they understood, and then the surgery shall be terminated. Since 2011, the patient had been experiencing edema of the lower limbs, numbness of both feet, and abdominal distension significantly worsened. In September 2014, the patient had stopped defecating, and then she was examined by an abdominal magnetic resonance, which indicated a huge soft tissue mass in the abdominal cavity (25 cm × 11 cm). The patient had various compression symptoms due to tumor enlargement and was getting worse, so she strongly demanded treatment. After discussion by the hospital MDT, the patient decided to receive palliative RFA therapy to delay tumor progression.
The patient underwent ultrasound-guided percutaneous RFA for intra-abdominal DTs in September 2014, October 2015, and January 2016. Deployment approaches of multielectrode and multipoint were applied in each ablation procedure. For safety, prominent blood vessels were intentionally avoided during electrode deployments [Figure 2]. Finally, RFA procedures were successful, and postoperative anal defecation and exhaust recovered without evident abdominal pain and distension. The patient is still alive and regularly followed up. Although the tumor has been growing slowly, the overall control was satisfactory, all ablated zones were significantly reduced, and some ablated zones were absorbed and even disappeared [Figure 3].
|Figure 2: Percutaneous ultrasound-guided radiofrequency ablation for giant desmoid tumors of the intra-abdominal cavity in a patient with Gardner syndrome. (a) The ultrasound scan before radiofrequency ablation showed that large and substantial tumors occupied the intra-abdominal cavity, the boundary of the tumors was unclear, and closely related to the mesentery; large blood vessels were seen at the bottom of the tumor. (b) Deployment approaches of multielectrode and multipoint were applied in each ablation procedure. For safety, prominent blood vessels were intentionally avoided when electrode deployments. (c) Contrast-enhanced ultrasound evaluation was performed after ablation, and the ablation area was contrast-agent-free, indicating that the tumor in the ablation area was inactivated|
Click here to view
|Figure 3: Follow-up after ablation for four years indicated that abdominal compression symptoms improved significantly, and magnetic resonance suggested that all ablated zones were significantly reduced, and some ablated zones were absorbed and even disappeared|
Click here to view
| > Discussion|| |
Extracolonic lesions of GS are most common with DTs in the abdominal wall, intra-abdominal cavity, and mesentery and may even be the first symptom. With incidence about 10%, DT is the leading cause of death from colon resection and one of the most difficult symptoms for doctors to deal with, and it occurs more often in women., Although DTs with locally aggressive behavior do not demonstrate malignant potential, morbidity can lead to physical impairment, and mortality from the disease is occasionally observed as a result of local infiltrative growth and tissue invasion, particularly with intra-abdominal DTs., In this case, DTs in the intra-abdominal cavity infiltrated adjacent organs, extended along the mesentery plane, compressed blood vessels, and small intestine, and resulted in serious symptoms, such as intestinal obstruction and severe abdominal distension.
At present, surgery and adjuvant radiotherapy remain the mainstream treatment for DTs; however, not all patients with DTs are amenable to radical surgery, and the surgical recurrence rate is as high as 80%. In addition, surgery may lead to severe complications, such as secondary bowel obstruction, intestinal ischemia, and mesenteric contracture, seriously decreasing the quality of life of patients., The patient's DT was huge, extensive, and inoperable in this case, while the patient's progressive compression symptoms were obvious, so a strong desire was exerted for treatment to improve symptoms.
RFA is a thermal ablation therapy, which uses high-frequency alternating current electromagnetic waves generated by the radiofrequency instrument that enters the tumor tissue through a puncture. This enables the ions and polar macromolecules in the surrounding tissue cells to vibrate to generate heat, causing coagulative necrosis of the tumor tissue. The body's immune system absorbs the necrotic tissue to reduce the tumor volume and improve the clinical symptoms. Although RFA could not achieve the goal of tumor eradication in this case, it can effectively reduce the tumor burden and accelerate the absorption of ablation lesions, which slows down or even reverses the overall volume growth of the tumor in the short term. There are few reports on the RFA treatment of DTs with relatively small volumes in superficial tissues, such as the abdominal wall, limbs, and trunk.,,, Although palliative treatment with RFA to delay tumor enlargement and relieve compression of such giant intra-abdominal DT has not been reported before, it produced a satisfactory therapeutic effect in our tentative treatment. Thus, RFA may have a good therapeutic effect on unresectable giant intra-abdominal DTs.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
This work was supported by National Natural Science Foundation of China (82071946) and Medical Health Science and Technology Project of Zhejiang Province (2019KY338).
Conflicts of interest
There are no conflicts of interest.
| > References|| |
Tayeb Tayeb C, Parc Y, Andre T, Lopez-Trabada Ataz D. Familial adenomatous polyposis, desmoid tumors and Gardner syndrome. Bull Cancer 2020;107:352-8.
Baldino ME, Koth VS, Silva DN, Figueiredo MA, Salum FG, Cherubini K. Gardner syndrome with maxillofacial manifestation: A case report. Spec Care Dentist 2019;39:65-71.
Desmoid Tumor Working Group. The management of desmoid tumours: A joint global consensus-based guideline approach for adult and paediatric patients. Eur J Cancer 2020;127:96-107.
Fotiadis C, Tsekouras DK, Antonakis P, Sfiniadakis J, Genetzakis M, Zografos GC. Gardner's syndrome: A case report and review of the literature. World J Gastroenterol 2005;11:5408-11.
Akbulut S, Koc C, Dirican A. Unusual complication in patient with Gardner's syndrome: Coexistence of triple gastrointestinal perforation and lower gastrointestinal bleeding: A case report and review of literature. World J Clin Cases 2018;6:393-7.
Guignard N, Cartier C, Crampette L, Akkari M. Gardner's syndrome presenting with a fibromatous tumour of the parotid. Eur Ann Otorhinolaryngol Head Neck Dis 2016;133:357-9.
de Camargo VP, Keohan ML, D'Adamo DR, Antonescu CR, Brennan MF, Singer S, et al.
Clinical outcomes of systemic therapy for patients with deep fibromatosis (desmoid tumor). Cancer 2010;116:2258-65.
Lazar AJ, Hajibashi S, Lev D. Desmoid tumor: From surgical extirpation to molecular dissection. Curr Opin Oncol 2009;21:352-9.
Barrow E, Newton K, Rajashanker B, Lee S, Evans DG, Hill J. Successful radiofrequency ablation of an anterior abdominal wall desmoid in familial adenomatous polyposis. Colorectal Dis 2013;15:e160-3.
Cobianchi L, Ravetta V, Viera FT, Filisetti C, Siri B, Segalini E, et al.
The challenge of extraabdominal desmoid tumour management in patients with Gardner's syndrome: Radiofrequency ablation, a promising option. World J Surg Oncol 2014;12:361.
Ilaslan H, Schils J, Joyce M, Marks K, Sundaram M. Radiofrequency ablation: Another treatment option for local control of desmoid tumors. Skeletal Radiol 2010;39:169-73.
[Figure 1], [Figure 2], [Figure 3]