Radiological features of desmoid-type fibromatosis: a two-institution retrospective study

OBJECTIVES: To characterize the radiological findings of desmoid-type fibromatosis (DF). METHODS: This two-institution retrospective study included 152 patients with pathologically confirmed DF who underwent computed tomography (CT), magnetic resonance imaging (MRI), or 18F-fluorodeoxyglucose-positron emission tomography (FDG-PET)/CT between January 2001 and February 2024. Two board-certified radiologists independently evaluated the CT, MRI, and FDG-PET/CT findings, and a third board-certified radiologist resolved discrepancies. Imaging was performed with and without contrast media: 70 patients underwent plain CT, 95 underwent contrast-enhanced (CE) CT, 115 patients underwent plain MRI examinations, 100 patients underwent CE-MRI, and 11 patients underwent FDG-PET/CT (most patients underwent several modalities). RESULTS: The median age of the patients was 40 years, with a female predominance (male, 39.5% vs female, 60.5%). Swelling or palpable mass was the most frequent symptom (78/152, 51.3%). Gross total resection of DF was performed in 57 patients, with a recurrence rate of 38.6% (22/57). Tumors were most frequently observed in the extra-abdominal region (79/152, 51.6%). Characteristic radiological features included intermediate intensity on T2-weighted imaging (112/113, 99.1%), intermediate-to-high intensity on T1-weighted imaging (109/111, 98.2%), substantial enhancement in the late phase on MRI (100/100, 100%), moderate to strong enhancement in the late phase on CT (18/20, 90%), and arterial penetration sign on CE-CT (25/96, 26.0%). The mean apparent diffusion coefficient (ADC) of DFs was 1.46 × 10-3 mm2/s (range, 1.00-2.20). CONCLUSION: This study highlights the unique imaging features of DF, including the arterial penetration sign and high mean ADC values, which can aid in differentiating DF from other soft tissue tumors. These findings may improve preoperative diagnostic accuracy and reduce the need for invasive procedures. KEY POINTS: Question Imaging findings of DF are not well-documented in large-scale studies. Findings This study identifies unique imaging features of DF, such as the arterial penetration sign and high mean ADC values. Clinical relevance These distinctive imaging characteristics improve diagnostic accuracy for DF and lead to appropriate patient management, as DF requires distinct treatment strategies.

Hidradenitis suppurativa (HS) is a poorly understood chronic inflammatory disorder of the hair follicle that affects apocrine gland-rich and intertriginous areas. HS is a clinical diagnosis characterized by deep seated, painful nodules, frequently in the axilla or groin, that may spontaneously rupture and drain malodorous, purulent, or sanguineous material. Drug-associated HS has been previously reported in the literature implicating new immunomodulating medications such adalimumab, infliximab, vemurafenib, rituximab, and tocilizumab.1 However, we present a case of HS secondary to an entirely new class of medication, gamma secretase (GS) inhibitors.

Mesenteric desmoid tumors (DTs) are rare, and there are no specific imaging features that can aid definitive diagnosis. This article presents the case of a 61-year-old woman found to have intra-abdominal DT located in the mesentery at two-year follow-up after laparoscopy-assisted anterior resection for stage I endometrial cancer. Computed tomography (CT) indicated the presence of a solitary tumor arising from the intestinal wall or mesentery. Subsequent magnetic resonance imaging and integrated positron emission tomography/CT with 2-18F-fluoro-2-deoxy-d-glucose suggested DT rather than recurrence, but surgical resection was chosen after considering the potential risks. Surgery confirmed the diagnosis, revealing a tumor at the level of the jejunal mesentery and approximately 5 cm in diameter. The mesenteric tumor was resected, along with part of the jejunum and the greater omentum. Histopathological examination of the surgical specimens confirmed the diagnosis of DT. As mesenteric DT after laparoscopic resection of stage I endometrial cancer is rare, a high degree of suspicion is necessary for diagnosis. Given that laparoscopic surgery is increasingly used in gynecological clinical practice, gynecologists and radiologists should be aware that DT should be considered in the differential diagnosis when a mesenteric mass is detected during follow-up after a surgical procedure, including laparoscopy.

The patient was a 47-year-old woman. She had been aware of abdominal distension for several months and visited our hospital. Contrast CT revealed a 30 cm tumor with uneven contrast effect. MRI showed uniform low signal intensity on T1 weighted images and a mixture of low and high signal intensity on T2 weighted images. She was diagnosed with a giant ovarian tumor and underwent surgery at the gynecology department. Laparotomy revealed that the tumor originated in the small intestine mesentery rather than the ovary, and she was referred to our department during surgery. The tumor had involved the ileocolic artery and vein, and was removed by right hemicolectomy. The specimen was 32×32×27 cm and weighed approximately 8 kg. Histopathological examination showed proliferation of spindle cells. Immunohistochemistry showed negative staining for c-kit and positive staining for β-catenin. The tumor was diagnosed as an intra-abdominal desmoid tumor. Intra-abdominal desmoid is a rare disease, and diagnosis and surgical procedure are often difficult. We report a case of resection of a giant mesenteric desmoid tumor with a review of the literature.

Breast desmoid tumour is a rare type of benign breast disease that presents like malignancy. Current guidelines are based on limited evidence derived from case reports and small case series and recommend resection with microscopically-negative margin (R0). There is a high risk of recurrence despite negative surgical margins. A review of the published cases of breast desmoid since 2000 was conducted using Medline and Embase to descriptively analyse the clinical presentation, diagnosis, treatment, and outcomes of this rare disease. After screening, we identified 46 patients from 39 articles. Most cases did not have risk factors, but 17/46 (37%) had prior procedures on the ipsilateral breast. Mammography was able to detect 65% of the cases, ultrasound detected 74%, and both CT and MRI detected all cases when used. Preoperative diagnosis was best performed using core needle biopsy showing typical histology and positive beta-catenin staining. 42/46 cases underwent definitive surgical management, with 8 cases of recurrence. Recurrence occurred within 3 years of the initial surgery. Median time of recurrence was 8 months, and the median follow-up of the recurrence-free patients was 12 months. There were no predictive factors identified for recurrence. There were 7 cases treated with a nonsurgical modality, with 3 showing at least a partial response.

none available