A giant mesenteric desmoid tumor revealed by acute pulmonary embolism due to compression of the inferior vena cava.

We report the case of a 69-year-old man who presented to our hospital with an acute pulmonary embolism. The patient had a past history of colic surgery for a polyp with a high-grade dysplasia. Pulmonary angiography showed partial occlusion of the right superior lobe artery and partial occlusion of the middle lobe artery. The patient was given thrombolytic therapy. Abdominal computerized tomography revealed a mesenterial giant mass with compression of the inferior vena cava (IVC). A biopsy of the mass, confirming aggressive fibromatosis. This report underlines the potential of imaging investigations of abdomen and vena cava if pulmonary embolism is suspected, especially when there is no evidence of peripheral venous thrombosis or other predisposing factors.

This report describes an 18-year-old woman presenting with abdominal distension, left flank pain, and hypertension. She had a huge abdominal mass, diagnosed as a mesenteric desmoid-type fibromatosis, causing compression of the left external iliac vessels and ureter, as well as elevated renin concentration and hypertension. After surgical removal of the mass, all signs improved including hypertension.

Purpose: To estimate the maximum tolerated dose (MTD) for continuous oral administration of the gamma-secretase inhibitor PF-03084014, determine the recommended phase 2 dose (RP2D), and evaluate safety and preliminary activity in patients with advanced solid tumors. A total of 64 patients received treatment. The MTD was estimated to be 220 mg BID. The RP2D was determined to be 150 mg BID, based on the better safety profile versus the 220-mg BID dose, given comparable NOTCH-related target inhibition. The most common treatment-related AEs were diarrhea, nausea, fatigue, hypophosphatemia, vomiting, rash, and decreased appetite, which were generally mild to moderate in severity. One patient with advanced thyroid cancer had a complete response and five of seven response-evaluable patients with desmoid tumor achieved a partial response (71.4% objective response rate). Tumor responses were mostly durable, ranging from 1.74+ to 24+ months. PF-03084014 demonstrated a generally dose-dependent pharmacokinetic profile at doses ranging from 20 to 330 mg BID. Consistent down-modulation of NOTCH-related HES4 gene expression was observed in peripheral blood from all evaluable patients. Conclusions: Further development of PF-03084014 for the treatment of patients with advanced solid tumors is warranted and currently under evaluation.

Desmoid type fibromatosis is an uncommon benign disease entity of which its etiology is currently unknown. It constitutes 0.3% of all solid neoplasms, but it is rarely seen in the breast and even more scarcely reported to develop in association with breast implant. We present ultrasonography and magnetic resonance imaging findings of a 29-year-old female patient with fibromatosis after breast implant surgery. Knowledge of imaging findings of breast fibromatosis associated with implant will be helpful for accurate diagnosis and appropriate management.

The medical records of 16 patients (9 female and 7 male; age range, 5-66 years) with 24 lesions that had a histologic diagnosis of desmoid tumors were reviewed at our institution. Six cases were extra-abdominal, 4 intra-abdominal, and 6 in the abdominal wall. Sonography can show a desmoid tumor’s site, size, contour, margin, echogenicity, homogeneity, vascularity, and resistive index value in detail.

We conducted a systematic review to determine the optimal treatment options in patients with desmoid tumours who have declined observational management. Significant toxicities were reported for all treatment modalities, including surgical morbidity, and rt- and chemotherapy-related toxicities. In patients who have declined observational management, the local control rate was higher with surgery plus rt than with surgery alone. However, the additional rt-related complications should be considered in treatment decision-making. Surgery, rt, and systemic therapy are all reasonable treatment options for patients with desmoid tumours.