Treatment of Desmoid Tumors in 2019

Recently, there has been a paradigm shift in the treatment of Desmoid Tumor toward an initial approach of active surveillance, rather than surgery with or without radiation. This shift occurred because the behavior of DT is unpredictable, and spontaneous growth arrest and regression can occur. In addition, patients can be asymptomatic. Herein, we describe our approach to the management of DT and the evidence base for selecting therapies in specific clinical scenarios.

A Desmoid tumor in the neck is a rare tumor in an even more unusual site. Our patient was an 18-year-old primigravid Bedouin woman from northwest Egypt. She presented with a dormant neck nodule that grew explosively during her first pregnancy and stopped abruptly after delivery. The presentation was confusing at first, as the fixation of the tumor to the underlying tissues implied a malignancy, while a 1-year history of non-metastasis alluded to a benign process. Pre-operative tissue diagnosis revealed an Estrogen receptor-expressing desmoid tumor. This report aims to increase awareness of peripartum Desmoid tumors, as well as discuss the surgical-site, psychological and socioeconomic challenges in the peculiar case of this Bedouin woman, and our recommendations after this experience and literature review.

The objective of this study was to develop an alternative method of non-contrast monitoring of tissue ablation during focused ultrasound treatment. Desmoid tumors are benign but locally aggressive soft tissue tumors that arise from fibroblast cells. Magnetic resonance-guided focused ultrasound (MRgFUS) has emerged as an alternative to conventional therapies, showing promising results in reduction of tumor volume without significant side effects. The gold-standard assessment of the reduction of viable tumor volume post-treatment is non-perfused volume (NPV) and evaluation of NPV is typically performed with post-treatment gadolinium enhanced MR imaging. However, as gadolinium cannot be repeatedly administered during treatments, there is a need for alternative non-contrast monitoring of the tissue to prevent over and under treatment. Double-echo and multi-echo images were acquired before, during and after the MRgFUS treatment. T2 maps were generated with an exponential fit and T2 maps were compared to post-treatment post-contrast images. In all five MRgFUS treatment sessions, T2 mapping showed excellent qualitative agreement with the post-contrast NPV. T2 mapping may be used to visualize the extent of ablation with focused ultrasound and can be used as a predictor of NPV prior to the administration of contrast during the post-treatment assessment.

A 64-year-old man had an intra-abdominal mass that was detected in a follow-up examination after laparoscopic partial nephrectomy for renal cell carcinoma (RCC). CT showed an enhanced mass of 2.5-cm diameter near the right kidney, where partial nephrectomy had been performed. Local recurrence of RCC with duodenum invasion was suspected, and excision was performed. The final pathological diagnosis of desmoid tumor differed from the preoperative diagnosis. Therefore, we report this case as a rare example of intra-abdominal desmoid tumor mimicking local recurrence of RCC.

A 37-year-old man with type 1 diabetes mellitus was referred to the hand surgery unit due to increasing pain, numbness and weakness in his right arm developing over more than one year. An ulnar nerve neurography was inconclusive and the patient had a frozen shoulder on the right side. At examination, the clinical presentation did not correspond to an ulnar nerve entrapment why other causes were considered. A chest X-ray revealed a lesion in the apical part of the right lung. Consecutive CT scan, MRI and fine and core needle biopsies led to the diagnosis of a desmoid tumor. Surgery was deemed to be too mutilating and treatment was initiated with doxorubicin. Due to tumor and symptom progression the therapy was changed to PegIntron, then antiestrogen and NSAID and finally tyrosine kinase inhibitor (sorafenib) resulting in tumor shrinkage.