Unusual Presentation of an Uncommon Malignancy: A 74-Year-Old Woman with Aggressive Fibromatosis of the Large Intestine Presenting as a Liver Mass and the Therapeutic Management.

BACKGROUND Desmoid tumors are a fibroblastic proliferation of soft tissues, with an extreme inclination for local dissemination and recurrence. Surgical excision is the usual treatment choice, with data regarding pharmaceutical treatment being scarce. CASE REPORT A 74-year-old female patient was admitted to "Laikon" General Hospital of Athens, Greece presenting with acute kidney injury secondary to diarrhea. The ultrasound, CT, and abdominal MRI performed showed a 12×6×10 cm tumorous liver lesion. Biopsy of the lesion revealed loosely organized, mesenchymal tissue with spindle cells, and myxoid stroma. Immunochemistry was positive for SMA and b-catenin. Right hemicolectomy was performed with tumor-free surgical margins (R0 resection) and tamoxifen was initiated. Six months after the last MRI (3 months after the use of tamoxifen), a follow-up MRI was performed. The tumor had increased to 14.2×11×12.3 cm, and at the next follow-up it had grown to 20.3×19 cm maximal dimensions; no new metastases were found. The patient received sorafenib and pazopanib. Our patient had PFS with sorafenib for more than 2 years and remained in a good performance status (ECOG 1). For Pazopanid, the median PFS for this treatment option was 6.5 months. CONCLUSIONS The results were good and show a promising method for the treatment of this rare but severe malignancy.

Extra-abdominal desmoid fibromatosis: Cryoablation versus traditional therapies.

PURPOSE: To retrospectively review the various methods used to treat extra-abdominal desmoid fibromatosis (EDF) at our institution to compare treatment response and complications with those for the emerging option of percutaneous cryoablation therapy. METHODS: A single-center retrospective review was conducted to identify patients with EDF who underwent some form of treatment for EDF in any combination (including medical therapy, surgery, percutaneous ablation and radiation therapy) at our institution between January 2007 and January 2020. Patients with pathological evidence of EDF and pretreatment and posttreatment images were included. Medical records and imaging data were also reviewed. Treatment response assessment was based on tumor size on follow-up imaging. RESULTS: A total of 41 patients (30 women; mean age, 34 y; range, 18-79 y) were included in the study. The extremities (44%) and back (22%) were the most common locations of EDF. Patients underwent a variety of treatment methods, including medical therapy (31 patients), surgery (24 patients), cryoablation (7 patients), radiation therapy (6 patients), and radiofrequency ablation (4 patients). Reduction in lesion size after at least 3 months of follow-up was most common in patients who underwent surgery alone (5 patients) or cryoablation (4 patients). Among all study patients, there were 10 minor complications and 3 major complications. Complication rates were lowest in patients who underwent cryoablation (no complications). CONCLUSION: Although further work is needed, the early data in this study offers promising results regarding the clinical application of cryoablation for EDF, which appears safer than radiofrequency ablation and a potentially effective.

Intra-abdominal Desmoid-Type Fibromatosis Mimicking Diverticulitis With Abscess: A Case Report.

BACKGROUND: Intra-abdominal desmoid-type fibromatosis (DF) rarely necessitates emergency surgery. However, the condition is difficult to diagnose preoperatively and can become life-threatening if left untreated. CASE REPORT: A 46-year-old man complained of fever and right lower quadrant pain. In computed tomography, the mesenteric side of the ascending colon demonstrated air and fluid collections, suggesting diverticulitis with abscess. After 2 weeks of conservative treatment with fasting, the patient started to consume food; nonetheless, fever returned. Colonoscopy and contrast enema detected a fistula extending from the ascending colon to the abscess, with no surrounding lesions. Surgery was then performed because the abscess was refractory. During laparotomy, the scar tissue of the abscess was found to be attached to the lateral wall of the ascending colon. Hence, right colectomy combined with abscess resection was performed. Histopathological findings revealed DF in the mesentery. CONCLUSION: Although rare, DF should be included in the preoperative differential diagnosis of intra-abdominal abscesses.

Radiation Therapy as an Alternative Treatment for Desmoid Fibromatosis.

AIM: To determine the outcome after radiation therapy for desmoid fibromatosis. MATERIALS AND METHODS: A retrospective review of 50 patients treated between 1988 and 2016 in a specialised bone and soft tissue tumour clinic. RESULTS: The median age at the time of radiation therapy was 36.8 years (range 15.1-69.0) and the median follow-up time was 51 months. Forty-three patients underwent radiation therapy as the definitive treatment with a median dose of 56 Gy (range 30-58.8 Gy). The median dose for the seven patients treated with postoperative radiation therapy was 50.4 Gy (range 48-56 Gy). Eleven patients (22%) developed progressive disease after radiation therapy at a median time of 41 months (range 12-113 months). The recurrences were within the radiation therapy field in four patients and outside the field in seven patients. One patient developed a radiation-induced malignancy 20 years after treatment. CONCLUSIONS: Radiation therapy is an alternative treatment in the management of desmoid fibromatosis. It should be considered in patients for whom surgical resection is not feasible, or as adjuvant therapy after surgery with involved margins where any further recurrences would cause significant morbidity.

Radiologically guided percutaneous cryotherapy for soft tissue tumours: A promising treatment.

Studies of percutaneous cryotherapy in the treatment of benign or malignant soft tissue tumours are rare and mainly involve small populations. Nevertheless, results show cryotherapy's potential in terms of local control of tumours, analgesic efficacy, reduced intra- and postoperative complications, and reduction in the length of convalescence after the procedure. The objective of this update is to set out the short-term prospects for this technique in the treatment of soft tissue tumours, so that it may be more widely offered in these indications.

Activity of Sorafenib against desmoid tumor/deep fibromatosis.

BACKGROUND: Desmoid tumors (deep fibromatoses) are clonal connective tissue malignancies that do not metastasize, but have a significant risk of local recurrence, and are associated with morbidity and occasionally mortality. Responses of desmoid patients to sorafenib on an expanded access program led us to review our experience. METHODS: After Institutional Review Board (IRB) approval, we reviewed data for 26 patients with desmoid tumors treated with sorafenib. Sorafenib was administered at 400 mg oral daily and adjusted for toxicity. RESULTS: Sorafenib was the first-line therapy in 11/26 patients and the remaining 15/26 had received a median of 2 prior lines of therapy. Twenty-three of 26 patients had shown evidence of progressive disease by imaging, whereas 3 patients had achieved maximum benefit or toxicity with chemotherapy. Sixteen of 22 (∼70%) patients reported significant improvement of symptoms. At a median of 6 months (2-29) of treatment, the best response evaluation criteria in solid tumors (RECIST) 1.1 response included 6/24 (25%) patients with partial response (PR), 17/24 (70%) with stable disease, and 1 with progression and death. Twelve of 13 (92%) patients evaluated by MRI had > 30% decrease in T2 signal intensity, an indirect metric for increased fibrosis and loss of cellularity. Eighty percent of patients with radiological benefit had extra-abdominal desmoids. DISCUSSION: Sorafenib is active against desmoid tumors. A prospective, randomized clinical trial of sorafenib against other active agents is warranted. Loss of MRI T2 signal may be a useful surrogate for defining responses, but requires validation by examination of tumor pathology.

[Chest wall fibromatosis after mammary prosthesis implantation. A case report and review of the literature]. / Fibromatosi del torace dopo impianto di protesi mammaria. Caso clinico e revisione della letteratura.

Desmoid tumour of the breast is a rare lesion. Only 9 cases in which the origin of the tumour was linked to a breast implant had been published up to 2004. We report a case of aggressive fibromatosis which developed on the thoracic wall two years after implantation of a mammary prosthesis. This tumour was locally aggressive. Radical surgery is the treatment of choice and in this case required a wide surgical resection, including removal of the chest wall, to gain control. The application of adjuvant radiation therapy is controversial.

Fibromatosis agresiva de localización mamaria: una rara entidad / Aggressive fibromatosis of the breast: a rare entity

Presentamos 2 pacientes con un tumor mamario cuyo diagnóstico, tras su extirpación quirúrgica, ha sido el de fibromatosis agresiva de localización mamaria. Es característica la falta de microcalcificaciones en la mamografía, y el diagnóstico suele realizarse tras la extirpación de la tumoración. El tratamiento de elección es la exéresis quirúrgica amplia del tumor. El estudio patológico pone de manifiesto una proliferación de células fibroblásticas sin atipia ni mitosis, que se organizan en fascículos largos o haces que rodean los lobulillos y conductos lobulillares. En cuanto a la evolución clínica, la característica más importante es la alta tendencia a la recidiva (AU)

Treatment of extra-abdominal desmoid tumors with interferon-alpha with or without tretinoin.

BACKGROUND AND OBJECTIVES: Surgery is the main treatment for extra-abdominal desmoid tumors, but the results of further management remain uncertain. Therefore, a retrospective analysis was undertaken to evaluate the toxicity and efficacy of treatment with interferon-alpha (IFN-alpha) +/- tretinoin in this setting. METHODS: Thirteen patients with extra-abdominal desmoid tumors and a median age of 32 years (range, 15-73) received IFN-alpha. Seven of these patients received a combination of IFN-alpha and tretinoin in order to test further enhancement. RESULTS: After a mean observation period of 27 +/- 15 months (mean +/- standard deviation) under treatment with IFN-alpha +/- tretinoin, local control was seen in 11 of 13 patients (85%). Seven patients had no evidence of disease at a mean disease-free interval of 22 +/- 18 months; in two patients progressive disease occurred after only 7 and 9 months, respectively, of observation. In another four patients, progression of the desmoid tumor was stabilized. CONCLUSIONS: The data of this retrospective, nonrandomized study on therapy with IFN-alpha +/- tretinoin suggest that such treatment may be effective in prolonging the disease-free interval of patients after intralesional or marginal surgery. Because of the encouraging response rate, this regimen appears to be another nonsurgical treatment alternative.