Successful implementation of an international desmoid tumor virtual tumor board: A novel platform for the management of rare tumors [Full Article]

The Desmoid Tumor Research Foundation (DTRF) was founded in 2005 with the mission of facilitating and funding research toward a cure for DT and supporting patients with information (staging-dtrforg.kinsta.cloud). The DTRF constantly receives inquiries from healthcare professionals, patients, and family members for advice on the management of DT. Similarly, as is the case for other rare tumors, known experts in the field of DT are inundated with email queries from colleagues seeking guidance. These messages often contain limited information; yet, the recipient typically feels a moral obligation to respond resulting in recommendations that may or may not be appropriate. With the desire to meet the needs of patients with DT and physicians worldwide, the DTRF initially raised the idea with members of its Scientific and Medical Advisory Boards of creating a virtual forum for discussion of complex DT cases. The idea was then taken to the annual DTRF International Research Meeting in 2017 where the virtual tumor board was born. The mission of this virtual tumor board includes four key tenants: multidiscipline, resourceful, inclusive, and collaborative. A core group of committed discipline-specific expert panelists from pediatric and medical oncology, radiology, general and orthopedic surgical oncology, pathology and radiation oncology were identified. A web-based platform was selected with funding and operations provided by the DTRF.

We present the case of an 18-year-old woman with a desmoid tumor involving her brachial plexus. A non-oncological resection was performed, with a focus on functional preservation. Residual disease is being treated with beta-catenin inhibitor and monitored with serial MRI.

We retrospectively analyzed 13 familial adenomatous polyposis (FAP) patients with desmoid tumors (DTs). A surveillance protocol consisting of scheduled follow-up evaluations depending on tumor location and tissue thickening, abdominal computed tomography (CT) scan/Magnetic resonance imaging (MRI) allowed prompt intervention in 3/11 aggressive intra-abdominal DTs, while sparing further interventions in the remaining cases, despite worrisome features detected in three patients. Moreover, we identified a possible predictive marker of tumor aggressiveness, i.e., the “average monthly growth rate” (AMGR), which could distinguish patients with very aggressive/life-threatening tumor behavior (AMGR > 0.5) who need immediate active treatment, from those with stable DTs (AMGR < 0.1) in whom follow-up assessments could be delayed. Surveillance protocols may be a useful approach for DTs. Further studies on larger series are needed to confirm the usefulness of periodic CT scan/MRI and the value of AMGR as a prognostic tool to guide treatment strategies.

High-intensity focused ultrasound (HIFU) therapy has been identified as a novel non-invasive approach for desmoid-type fibromatosis (DF) treatment; however, the ultrasonic energy generated by HIFU can cause skin heat injury. A 31-year-old female patient with signs and symptoms of DF received treatment in our institution. The patient had undergone HIFU treatment six times from April 27, 2018, to August 21, 2019. After HIFU therapy for the third time, she had a third-degree skin burn showing as orange peel-like change and spent three months to promote the recovery of the skin lesions. An intermittent ice-cooling strategy was used to avoid skin damage during the fourth HIFU treatment. This patient did not have any apparent skin injury during the last three HIFU therapy and acquired satisfactory anti-tumor therapeutic effect. There are differences in the thermal selectivity of tumor tissues, which leads to different critical thermal injury temperature values that the tissue can tolerate. Ice-cooling can lower skin tissue temperature and reduce the thermal damage caused by HIFU treatment.

We treated a male patient with a head and neck recurrent desmoid tumor with proton beam therapy (PBT) at a dose of 60 Gy (RBE). This patient underwent surgical resection as initial treatment, but the tumor recurred only six months after surgery, and resection was performed again. After PBT, the tumor gradually shrank and complete remission has been achieved for 10 years without any severe late toxicity. Here, we report the details of this case, with a review of the literature. We suggest that PBT may reduce the incidence of second malignant tumors by reducing the dose exposure around the planning target volume.

Desmoid tumors are rare fibrous tumors of the soft tissues. Develops on the abdominal wall, in the abdomen, or extra-abdominal. Parietal location is very common among women. Hormonal factors are incriminated. They are rare lesions without any metastatic potential a strong tendency to invade locally and to recur. Hormonal factors are incriminated in this pathology. Imaging can characterize these tumors and their reports and guide the biopsy that is the key to diagnosis. The treatment is based on first-line surgery, but in cases where excision margins are reached, local recurrence or unresectable tumors, other therapeutic alterations are needed such as radiotherapy and chemotherapy and others. We report a periumbilical case in perimenopause.