Brachial Plexus Desmoid Tumor Care for Functional Preservation

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.

We report the case of a 72-year-old man with a desmoid tumor arising from the anterior chest wall which invaded neighboring organs extensively. The patient complained of dyspnea on exertion and appetite loss and was referred to our hospital. Chest computed tomography revealed an anterior chest wall tumor 12 cm in diameter adjacent to the right lung, diaphragm, and sternum. An ultrasound-guided biopsy was conducted, and the tumor was diagnosed as a desmoid tumor. He underwent right-sided anterior chest wall resection with combined resection of the right lung, diaphragm, and sternum body. The chest wall defect was reconstructed using an expanded polytetrafluorethylene mesh (dualmesh). Although initial active surveillance has recently been recommended for asymptomatic patients with non-progressing desmoid tumors, our patient underwent resection because of his symptoms.