Is MRI diffusion-weighted imaging a reliable tool for the diagnosis and post-therapeutic follow-up of extremity soft tissue neoplasms?

The aim of this study was to evaluate the benefit of using quantitative diffusion-weighted imaging (DWI) with apparent diffusion coefficient (ADC) mapping in the initial diagnosis and post-therapeutic follow-up of extremity soft tissue masses.This study included 90 patients with extremity soft tissue masses. The DWI was obtained with 3 b values, including 0, 400, and 800 s/mm2. Benign masses, fibromatosis, and malignant soft tissue masses had mean ADC values of 1.18 ñ 1.0191 ? 10-3 mm2/s; 1.31 ñ 0.245 ? 10-3 mm2/sec; and 1.3 ñ 0.7 ? 10-3 mm2/s, respectively. Analysis of the post-therapy follow-up studies of fibromatosis showed that lesions with favorable response to chemotherapy or radiotherapy (8/12) exhibited significantly lower ADC values than those showing progressive disease course. DWI with ADC mapping of extremity soft tissue tumors are so complicated that they alone may not be of much value in differentiating between benign and malignant tumors; however, it can be used as a tool for monitoring response to treatment.

A 36-year-old Caucasian female weighing 60?kg with Gardener’s syndrome with a history of abdominal surgery was presented to our department with progressive abdominal distention associated with paroxysmal pain. With the use of CT, the patient was diagnosed with a mass arising from the mesenterial region.The patient had normal kidney function and nonalcoholic steatohepatitis. The patient was indicated for MVTx. After 16 months on the waiting list, the patient received a multivisceral graft from a deceased donor. Following the restoration of graft vascular flow, the patient’s right kidney was removed and the DT dissected ex vivo before autotransplantation into the right pelvic fossa. The patient received immunosuppressive, antithrombotic, and antibiotic treatment. There was no acute rejection, though the patient experienced pulmonary infection, dysphagia, and oesophageal reflux with fungal infection. The patient had required temporary dialysis for acute renal failure for 75 days. One year after the surgery, nausea and violent vomiting caused delayed gastric emptying caused by spastic pylorus. Clinical improvement was achieved using gastric peroral endoscopic myotomy (G-POEM). MVTx with kidney autotransplantation is a feasible treatment option in patients with familiar adenomatous polyposis complicated by an abdominal DT. Precise tumour dissection with nephron-sparing surgery was carried ex vivo. G-POEM was used to relieve MVTx-related gastroparesis. The patient had no disease reoccurrence after one-year follow-up.

A patient in the fifth decade of life with a history of ductal carcinoma-in-situ, status post bilateral mastectomy and silicone implant placement, presented with a palpable mass in the left breast, core biopsy proven to be a desmoid tumor underneath the implant. The patient underwent two cryoablation procedures in a six-month period. During both procedures part of the implant was included in the ablation zone without any negative effects on the implant. Cryoablation is a feasible treatment option for desmoid tumors adjacent to silicone breast implants.

About 15 % of patients with familial adenomatous polyposis “PAF” develop one or more desmoid tumors in their lifetime. These tumors raise a therapeutic problem because of their heterogeneity and the absence of predictive biomarkers along illness evolution. The identification of prognostic biological and clinical factors would make easier the selection of patients requiring first-line treatment, as spontaneous remissions have also been observed in patients with FAP whom which an active surveillance could also be a valid therapeutic option. The particularity of desmoid tumors associated to PAF lies in their predominantly intra-abdominal location and the risk of complication. In the last ten years, surgery has largely given way to conservative treatments such as chemotherapy and more recently to tyrosine kinase inhibitors that have shown their efficacy with a significant improvement in progression-free survival of patients.

Locoregional therapy for advanced or recurrent desmoid tumor is part of our therapeutic armamentarium. Radiation therapy at a dose of 56Gy is feasible and has led to favorable results despite limited prospective data available. Cryoablation is a novel, very promising technique that may be applied in the same clinical picture of advanced, progressing or recurrent disease, in the hands of trained teams. All these options have to be discussed in sarcoma tumor boards due to the rarity of the disease and the expertise needed.

In this study, we investigated the expression of estrogen receptors (ER) alpha (1) and beta (2), progesterone receptor (PR), androgen receptor (AR), as well as Poly Adenosine Diphosphate Ribose Polymerase-1 (PARP-1) via immunohistochemistry and quantitative RT-PCR in 69 tissue samples of desmoid tumors. Immunohistochemistry was quantified using the Immunoreactivity Score (IRS). Overall expression patterns were correlated with clinical-pathologic parameters to determine their value as a prognostic factor. Among the investigated hormone receptors only ER Beta showed partial cytoplasmic reactivity. PARP-1 revealed variable nuclear positivity with IRS ranging from 0 to 6. Univariate survival analysis showed that higher expression of estrogen receptor 1 was associated with shorter disease-free survival (p?=?0.005). Uni- (p?=?0.03) and multivariate (p?=?0.003) analyses of mRNA data revealed that higher PARP-1 expression correlated with earlier recurrence. According to this study PARP-1 expression is associated with poorer prognosis, that is faster recurrence, highlighting the possibility of PARP-1-targeting agents as a therapeutic option. Hormone receptors were of minor prognostic relevance in this study.