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Background To analyze the characteristic features of deep fibromatosis on conventional and diffusion-weighted MR images. Result The lesions were growing along the musculoaponeurotic fascia, mostly invaded the muscles, and showed ill-defined margins, low T2 signal bands and areas, and facial tail sign. Diffusion images showed mostly high or high mixed with low signal; only 2 lesions showed a persistent low signal. The average mean and minimum ADC values were 1.41 ± 0.26 × 10−3 mm2/s and 0.79 ± 0.43 × 10−3 mm2/s respectively. Post-contrast and DWI detected synchronous lesions and extensions missed on T1 and T2 images. Conclusion The most frequent MR features of deep fibromatosis are low T2 signal bands and areas, fascial tail sign, ill or partially defined margins, and predominant restricted diffusion pattern in addition to areas of “T2-blackout effect.” Post-contrast and DWI are more valuable in local staging of the tumor.

Desmoid-type fibromatosis is a neoplasm with intermediate biological aggressiveness classified among fibroblastic-myofibroblastic tumors. It is also known as aggressive fibromatosis, deep fibromatosis, musculoaponeurotic fibromatosis, desmoid tumor, desmoid fibromatosis, and also simply desmoid. Along with superficial fibromatosis, DTF belongs to the family of fibromatoses.

Desmoid tumors are invasive and aggressive deep fibromatosis rare in children. They occur in muscles, their sheaths and fasciae. Medical imaging (CT and MRI) can suggest the diagnosis, guide the therapeutic management and the follow-up. We report a pediatric case of desmoid tumor of the pelvis through which we describe the clinical and radiological appearance of these tumors in children.

Fibrous, myofibroblastic, and fibrohistiocytic soft-tissue tumours are amongst the most common benign soft-tissue lesions encountered in clinical practice. They demonstrate varied biological behaviour and imaging characteristics. Benign fibroblastic lesions, such as nodular fasciitis, are small, have a self-limited course, and rarely recur after excision, whereas deep fibromatosis and plexiform fibrohistiocytic tumours tend to exhibit more aggressive features and often have high recurrence rates. MRI with its superior tissue contrast, multiplanar imaging capability, and lack of ionising radiation is regarded as the preferred method of tumour evaluation, tissue characterisation, and assessment of treatment response. Histopathological features are depicted at MRI, reflecting the amount and distribution of the cellular and fibrous matrix. Cellular tumours tend to show higher T2 signal intensity and post-contrast enhancement as compared to tumours with greater collagenous content, which appear dark and show less enhancement. Awareness of MR characteristics, pathological behaviour, and common sites of occurrence of fibrous soft-tissue tumours will help radiologists to determine the appropriate differential diagnosis and guide patient management.

Desmoid tumors (deep fibromatosis) of the mesentery are rare mesenchymal tumors. They are often misdiagnosed, especially with a previous history of resection for gastrointestinal stromal tumor (GIST). Immunohistochemistry can help differentiate between these two tumors. In this article, we present a case we had encountered: a Desmoid tumor developing in a patient with a history of GIST 3 years ago. It is the first case of GIST with subsequent development of Desmoid tumor to be reported in Saudi Arabia. We discuss the two entities of Desmoid tumor and GIST by comparing their definitions, clinical presentations, histological features, immunohistochemistry stains, molecular pathogenesis, prognosis, and treatment. We also discuss the relationship between GIST and the subsequent development of Desmoid tumors and compare our case with case reports in literature.

Desmoid tumor is a proliferation of a mesenchymal, fibroblastic or myofibroblastic tissue. It is a non-capsulated tumor. Despite of its benign nature, desmoids tumor have an aggressive behavior.Its localization in the parotid gland has been rarely reported in the literature.The particularity of this location in the head and neck is the proximity of Vascular-nervous structures, the facial nerve in the parotid location and the consequence, if affected, on the facial motility especially that this tumor often occur in young people. The clinical presentation is often about a slow-growing mass characterized by being: painless, ill defined, firm, non inflammatory, deeply located, and fixed to the underlying structures. Treatments strategies, made always within a multidisciplinary committee, are based on the biological natural behavior of this deep fibromatosis which is unpredictable and variable. Surgery could be undergone every time it is radical. Radiotherapy could also be played. But the most relevant point is that actually, most authors highlighted the conservative approach by the “wait and see” policy for primary as well as for recurrence of the disease after surgical resection or radiotherapy.

Mesenteric fibromatosis is an uncommon tumour which is locally aggressive without any metastatic potential and can occur as a sporadic event or in association with familial adenomatous polyposis syndrome. Giant mesenteric fibromatosis is very rare and is a diagnostic and therapeutic challenge. This is a case report of a rare presentation of deep fibromatosis as a sporadic giant intrabdominal mesenteric tumour in a 29 year old male managed by surgical excision and definitive diagnosis made on the basis of immunohistochemical findings.

BACKGROUND: Desmoid tumors are slow growing deep fibromatosis with aggressive infiltration of the adjacent tissue but without any metastatic potential.

Desmoid tumors are a rare group of locally aggressive, non malignant tumors of fibroblastic origin that can lead to significant morbidity due to local invasion. Despite advances in the understanding of these tumors, their natural history is incompletely understood and the optimal treatment is still a matter of debate. Local control is the main goal of treatment and there has been a change in philosophy regarding the management of these tumors from aggressive surgical resection to function preservation. A multidisciplinary approach is essential to plan local control with acceptable morbidity. The current Mayo Clinic algorithm for the treatment of these tumors is based on institutional experience and the available evidence in the literature: asymptomatic/non progressive lesions away from vital structures are managed with observation and regular imaging; primary or recurrent desmoid tumors which are symptomatic or progressive or near vital structures are managed with wide surgical resection when wide surgical margins are possible with minimal functional and cosmetic loss. When positive or close surgical margins are likely, surgical resection with adjuvant radiotherapy or definitive radiotherapy is preferred. If likely functional or cosmetic deficit is unacceptable, radiotherapy is the treatment of choice. Unresectable lesions are considered for radiotherapy, chemotherapy or newer modalities however an unresectable lesion associated with a painful, functionless, infected extremity is managed with an amputation.

Fibromatosis is a group of soft tissue tumours with unpredictable clinical presentation distinguished as locally belligerent and non metastasising. Deep fibromatosis is rarer than superficial fibromatosis. We report an unique case of intravascular extension of fibromatosis where the tumour epicentred in the pelvis reached the cavo-atrial junction enroute Inferior vena cava.

Desmoid tumors (fibromatosis) are intermediate grade, locally aggressive soft-tissue tumors. A rare case of chest wall fibromatosis in a girl with congenital muscular torticollis is being reported. This report also highlights the need for long-term surveillance in such cases, despite being a benign pathology.

Desmoid tumors, also called aggressive fibromatosis, have been described for the first time by John McFarlane in 1832; they are rare, but not exceptional. They are part of deep fibromatosis and described as infiltrating fibrous proliferations without metastasis, but with tendency to recur locally. Their benign histological structure contrasts with their local aggressivity and their therapy remains the major problem. We report three new cases diagnosed in an Internal Medicine Department in three women aged respectively of 54, 27 and 37 years. The respective locations were ovary, inguinal and parietal region of the scapular. Therapy was based mainly on surgery. The desmoid tumor is suspected on clinical and radiological signs and confirmation of diagnosis is pathological. Regular monitoring of patients is necessary because of the frequency of recurrences.

The report deals with the clinical features of a 26-year-old man with the extraabdominal form of deep fibromatosis, which became manifest at the age of 18 years as acute ileus caused by diffuse intraabdominal fibromatous tumours. In the later course of the disease extensive fibromatous lesions developed, mainly at the skin. In addition, multiple keloids occurred after excision or after negligible traumas. The pathogenetic events of the clinical picture described may be linked to the activation of mesenchymal cells leading to the generation of either fibromatous tumours or posttraumatic keloids.

OBJECTIVE. The purpose of this article is to illustrate the common locations of desmoid tumors (deep fibromatosis), complications of intra- and extraabdominal desmoids, and treatment-related changes in their imaging appearance. CONCLUSION. Desmoids are locally aggressive fibrous tumors with a tendency to recur. Desmoids can be intraabdominal, in the abdominal wall, or extraabdominal. Complications, such as compression or invasion of adjacent structures, and abscess formation can occur. Treatment options include observation, local treatment (surgery, radiotherapy), or systemic therapy (conventional chemotherapy, molecular targeted agents).

Desmoid tumor, also known as aggressive or deep fibromatosis or musculoaponeurotic fibromatosis, is a rare tumor. Desmoids are characterized by their ability to locally infiltrate; while frequently locally recurrent, they lack metastatic potential. Desmoids typically arise within the abdomen or abdominal wall, but can be extra-abdominal, most commonly in the proximal extremities. The infiltrative and recurrent nature of desmoid tumors can render surgical resection challenging if acceptable function and cosmesis is to be maintained. Consequently, desmoid management frequently involves a multidisciplinary approach that combines the expertise of several surgical specialists, for example, surgical oncologists and plastic surgeons, and also medical and radiation oncologists. As will be reviewed in this article, working together before undertaking any definitive therapies, such expert teams may be optimally positioned to offer patients superior functional and aesthetic outcomes based on maximizing therapeutic efficacy while minimizing treatment-related morbidities.

10013 Background: DT/DFs are clonal connective tissue malignancies that do not metastasize, but have a significant risk of local recurrence, and are often associated with morbidity and occasionally mortality. Antiestrogens and cytotoxic agents have activity against desmoids, but many patients (pts) fail to respond to such therapy. Imatinib has activity against DT/DF, but response rates are also low. A response of a patient to sorafenib on an expanded access program led us to review our experience with sorafenib for pts with DT/DF. Methods: After IRB approval, we collected and reviewed files for 13 patients with DT/DF treated with sorafenib. Results: We identified 13 pts who received sorafenib for DT/DF. Patient characteristics included: M/F: 6/7; extremity primary site in 4 (31%), abdomen/pelvis in 6 (46%), other in 3 (23%); recurrent disease in 8 (61%); 2 with FAP (15%); 3 with post-surgical DT/DF (23%); multifocal in 6 (46%). Pts had received a median of 2 lines of systemic therapy (range 0-5). Sorafeni...