Desmoid tumor in pregnancy complicated by deep vein thrombosis: A case report.

Desmoid tumor is a rare, locally aggressive soft-tissue neoplasm that primarily arises from the abdominal wall. Deep vein thrombosis is more common in pregnancy due to hypercoagulability. The coexistence of desmoid tumor and pregnancy complicated by deep vein thrombosis is extremely rare and poses unique diagnostic and therapeutic challenges. A 29-year-old woman, gravida 2 para 1, with obesity, presented at 28 + 6 weeks of gestation for routine antenatal care. She had a history of left lower limb deep vein thrombosis and presented with progressive leg swelling. Ultrasonography revealed a 10.7 × 6.5 × 10.6 cm abdominal wall mass arising from the left rectus abdominis compressing the left external iliac vein consistent with chronic deep vein thrombosis. Because of financial constraints, warfarin was prescribed temporarily instead of low-molecular-weight heparin. At 38 + 5 weeks of gestation, elective cesarean delivery was performed with a modified surgical approach to avoid damage to the tumor. Resection was deferred due to the high risk of hemorrhage while on anticoagulation. A healthy female newborn was delivered. Postpartum biopsy confirmed desmoid-type fibromatosis with extension into adjacent muscles and iliac vessels. Radiotherapy was planned but delayed, again owing to financial constraints. Lifelong anticoagulation with warfarin and surveillance were planned for the patient. The case highlights the importance of considering abdominal wall tumors as a potential cause of pregnancy-related deep vein thrombosis. It also demonstrates how financial considerations can shape clinical decisions and underscores the role of multidisciplinary planning in optimizing maternal and fetal outcomes.

Purpose
Radical resection is a valid option for abdominal wall desmoid tumors. However, significant functional impairment of the abdominal wall is often followed by radical resection with aggressive wide margins. Sublay repair may be advantageous in comparison to other surgical techniques for giant abdominal defect repair. This retrospective study aimed to analyze the clinical results of abdominal wall reconstruction after radical resection of abdominal wall desmoid tumors using the sublay technique in 76 patients.
Method
We retrospectively analyzed the clinical data of 76 patients who underwent abdominal wall desmoid tumor resection with sublay mesh-reinforced reconstruction between June 2012 and June 2022.
Results
The surgery was successfully completed in all patients. The mean size of tumors was 50.40 ± 34.74 cm2 (range 6-300). The mean defect size was 106.80 ± 40.97 cm2 (range 40–300). The hospital stay after the operation was 7.7 ± 2.9 days (range 3–38 days). Postoperative complications included hematoma formation and tumor recurrence. After two years of follow-up, no incisional hernia was observed in any patient.
Conclusion
Abdominal wall reconstruction using sublay mesh reinforcement is both feasible and safe for selected patients who undergo radical resection of abdominal wall desmoid tumors.

BACKGROUND: Aggressive fibromatosis, also known as desmoid tumor (DT), is a locally aggressive myofibroblastic neoplasm originating from deep soft tissues, characterized by an infiltrative growth pattern with a tendency for local recurrence. DTs account for 0.03% of all neoplasms, and cases associated with familial adenomatous polyposis (FAP) account for 5-15% of DTs. ^p AIMS: The aim of this study was to report the prevalence of DTs in patients operated on for FAP, describe the epidemiological profile, and evaluate the risk factors for tumor development, treatments performed, associated complications, and follow-up. ^p METHODS: This retrospective study assessed the medical records of patients with FAP who underwent surgery between 1990 and 2021 and developed DTs during follow-up. ^p RESULTS: In the study period, 147 patients with FAP were operated on; of these, 97 underwent total proctocolectomy with ileal-pouch anal anastomosis, 33 underwent total colectomy with ileorectal anastomosis (IRA), 14 underwent total proctocolectomy with terminal ileostomy, and three underwent total colectomy with partial proctectomy and low IRA using an ileal-pouch. A total of 26 patients (17.7%) developed DT; most were female (61.5%), were White (73.1%), and had a family history (84.6%). The most frequent complications were intestinal and ureteral obstructions. Long-term follow-up showed that six patients were free of disease, 14 were stable and undergoing drug therapy, four died due to complications of the disease, and two were lost to follow-up. ^p CONCLUSIONS: The prevalence of DT tumor was relatively high and more commonly observed in patients with a family history of the tumor. The disease presented high rates of morbidity and mortality.

Desmoid tumors, known as aggressive fibromatosis, are derived from connective tissues, and local invasion is usually observed. Despite intensive efforts have been performed to investigate the novel anticancer agents in desmoid tumors, effective clinical management for treating desmoid tumors has not been developed yet. Additionally, the molecular mechanisms involved in the tumorigenesis of desmoid tumors have not been elucidated. In this study, given the frequent mutations of Wnt components and loss of function mutations in Trp53 in desmoid tumors, we developed the mouse models harboring Apc mutation with/without Trp53 knockout, Apc1638N/+, and Apc1638N/+/Trp53-/-, respectively. We then established two primary cells derived from desmoid tumors in Apc1638N/+ and Apc1638N/+/Trp53-/-. Next, we have screened 3120 chemicals from a Food and Drug Administration-approved chemical library and identified halofuginone hydrobromide (HH), a collagen I-targeting compound, as exhibiting the most significant growth inhibition effects on Apc1638N/+ and Apc1638N/+/Trp53-/- desmoid tumor cells. Notably, HH also showed dramatic anticancer effects on colorectal cancer cells and mouse tumor organoids derived from intestinal tumors (Apc1638N/+). Taken together, targeting collagen I is an effective therapeutic strategy for treating desmoid tumors and colorectal cancer patients.

A superficial or deep mass in the limbs or body walls may be a sarcoma, even if it has been present for a long time. It warrants caution, as inappropriate surgery can worsen the prognosis. A superficial tumor should be evaluated with an ultrasound: if the tumor is not perfectly fatty and homogeneous, a percutaneous biopsy is necessary. Deep tumors require an MRI followed by a percutaneous biopsy. The final histopathological diagnosis must be awaited, which may include molecular biology in addition to immunohistochemistry. If it is a sarcoma or a desmoid tumor, the therapeutic strategy must be decided in a specialized multidisciplinary team meeting. Sarcomas require wide excision, ensuring healthy margins by staying well away from the lesion. In France, there is a network of specialized centers for the treatment of sarcomas (NETSARC+).

Rare breast tumors include benign or borderline entities that are often clinically and radiologically misleading. Diagnosis relies in all cases on appropriate biopsy and cross-anatomical-radiological interpretation. Optimal management requires multidisciplinary discussion. Desmoid tumors are non-metastatic but locally aggressive, infiltrative fibroblastic lesions whose management is now based on active surveillance. Idiopathic granulomatous mastitis is a chronic inflammatory mastitis, probably of autoimmune origin, which generally responds to corticosteroid treatment. Phyllodes tumor is a rare fibro-epithelial proliferation, with variable behavior depending on its histological grade, requiring complete excision with negative margins. Radial scarring, a benign sclerosing lesion, can mimic a carcinoma, and sometimes warrants excision depending on the presence of atypia. The aim in managing all these rare tumors is to limit overtreatment, while ensuring personalized follow-up.