Desmoid Tumor of the Porta Hepatis: A Rare Location With Unusual Clinical Presentation

Desmoid tumors, also known as aggressive fibromatosis, are uncommon mesenchymal neoplasms. They account for only 0.03% of all tumors and 3% of all soft-tissue neoplasms [[1]]. A pregnancy-associated desmoid tumor is an even less common disease very rarely reported in published articles. Although it grows by locally infiltrating its neighboring tissues and structures, it is devoid of metastatic evolution. It usually occurs between the ages of 25 and 40 years, with a strong prevalence among women in the fertile age group [[1]].

A Giant Mesenteric Fibromatosis Case Presenting with Mechanical Intestinal Obstruction and Successfully Resected with Partial Duodeno-Jejunectomy and Right Hemicolectomy

Desmoid Tumors in a Dutch Cohort of Patients With Familial Adenomatous Polyposis

Aggressive Fibromatosis of the Chest Associated With a Silicone Breast Implant

e23543 Background: Desmoid tumors are locally invasive mesenchymal neoplasms of fibroblastic origin arising in deep soft tissue. Despite a rare incidence of 2.4-4.3 per one million and an inability to metastasize, desmoid tumors can cause significant morbidity by invading surrounding structures, causing pain, anatomic deformities, and, in some cases, death. Due to the high post-excision recurrence rate (21-29%) and frequent spontaneous regression or stabilization (50-88%), active surveillance is the first-line treatment for desmoid tumors. Most therapeutic agents only achieve tumor stability and response rates of 10-30%. As such, a large subset of patients face disease progression without an effective therapeutic option. In this context, percutaneous cryoablation arises as a novel treatment for desmoid tumors. This retrospective chart review case series aims to describe the effectiveness of cryoablation in the treatment of desmoid tumors. Methods: We retrospectively reviewed medical records of patients with a pathologically confirmed desmoid tumor who received computed tomography (CT) guided percutaneous cryoablation at a single academic hospital between 2010 and 2021. We defined objective response rate (ORR) as the percentage of patients who have partial or complete response to cryoablation. Disease progression (PD), Stable Disease (SD), Partial Response (PR), and Complete Response (CR) were defined per mRECIST criteria. Results: We identified nine patients (8 female, mean age 33) with desmoid tumors who underwent percutaneous cryoablation. The most common tumor location was the abdominal wall (n = 5,) and the median longer axis tumor size was 9.5 cm (range: 4.5-16 cm). Seven patients received previous systemic treatments. Sorafenib (n = 6) was the most common systemic therapeutic option, followed by methotrexate/vinblastine, Adriamycin/dacarbazine, tamoxifen, nirogacestat, and sulindac. Before cryoablation, six of the seven patients receiving systematic therapies had PD; one had SD. After cryoablation, seven underwent post-cryoablation imaging. Mean time to initial post-op follow-up was 38.85 days (range: 22-60 days). Six patients achieved PR (66.67%), one patient maintained SD (11.11%), and two (22.22%) were lost to follow-up. Two patients with PR regressed to SD two and three months after initial post-cryoablation imaging, respectively, and one with SD improved to PR after eleven months. As such, ORR was 71.43% (95% CI: 0.352 to 1.08). Conclusions: Our data support cryoablation as an effective therapy for decreasing tumor burden in multi-treatment resistant desmoid tumor patients. Although larger studies are needed to assess efficacy and safety, with an ORR of over 70% and a disease control rate of over 75%, cryoablation demonstrates promising results without the toxicity of systemic therapy and thus may be an effective strategy for multi-treatment resistant desmoid tumors.

Desmoid tumors are benign proliferations of spindle cells originating in fibro-aponeurotic tissue. Many patients with familial adenomatous polyposis (FAP) die from desmoid tumors, which can arise spontaneously but often appear to be surgically induced by prophylactic colectomy. Desmoid tumors are the second most common cause of death in patients with FAP, second to colorectal cancer. Many patients can live a long life with desmoid tumors without symptoms, but when symptoms (ranging from bowel or ureteric obstruction to bowel perforation with abscess and fistula) appear or there is a risk of functional impairment, a wide spectrum of therapies (local and systemic) are valuable in improving the symptoms and controlling the disease. A half-Japanese, half-Caucasian male, who had been diagnosed with intra-abdominal desmoid tumors associated with FAP at age 13, was treated using abdominal wall incision for decompression and chemotherapy from the age of 38. The therapeutic outcome was progressive disease, based on the modified response evaluation criteria in solid tumors (mRECIST), and when he visited our hospital at age 41 the desmoid tumor had invaded the small bowel with a fistula to the abdominal wall. We performed a palliative operation to improve his symptoms, which were fever, abdominal pain, vomiting, and difficulty eating. As the tumor was extremely large and had invaded the small intestine, massive resection including the small intestine was required. To prepare for anticipated massive bleeding, a balloon catheter was placed in the superior mesenteric artery just prior to surgery. Although the operation was extremely difficult, following surgery the patient regained his ability to eat and when discharged was ambulatory and without short-bowel syndrome. We report our experience treating one of the largest reported intraperitoneal desmoid tumors. Resection resulted in a good postoperative course, with improved quality of life and prognosis.

Patient: Female, 63Final Diagnosis: Recurrent desmoid tumorSymptoms: Abdominal discomfort • abdominal fullnessMedication: —Clinical Procedure: AbdominoplastySpecialty: Plastic SurgeryObjective:Rare co-existance of disease or pathologyBackground:Desmoid tumors are fibrous neoplasms that originate from the musculoaponeurotic structures in the body. Abdominal wall desmoid tumors are rare, but they can be locally aggressive, with high incidence of recurrence. These tumors are more common in young, fertile women. They frequently occur during or after pregnancy.Case Report:We present the case of a 63-year-old post-menopausal woman with a desmoid tumor of the anterior abdominal wall. She had no relevant family history. During abdominoplasty, an incidental mass was excised and biopsied, and was identified as a desmoid tumor with free margins. One year later, the patient presented with vague abdominal discomfort and feeling of heaviness. An incision was made through the previous abdominoplasty scar to maintain the aesthetic outcome. A large mass, arising from the abdominal wall and extending intra-abdominally, was excised and was determined to be a recurrent desmoid tumor.Conclusions:Recurrent anterior abdominal wall desmoid tumors in post-menopausal women are rare and locally aggressive, with a high risk of recurrence. During abdominal wall repair in abdominoplasty, desmoid tumor filaments might seed deep intra-abdominally. Therefore, it is necessary to take adequate safe margins before abdominal wall repair. Post-operatively, surgeons should keep a high index of suspicion for tumor recurrence.

Background:Desmoid tumor is the most common abdominal wall tumor. It is frequently associated with either surgical trauma, such as abdominal or pelvic surgery, or physiologic trauma, such as pregnancy. So, Caesarean sections (C/S) is thought to be an important risk factor because it contains both factors.Aim and Objectives:Review our experience of sporadic abdominal desmoid tumors relate to C/S and analyze it.Materials and Methods:Between 1983 and 2010, 9 cases of abdominal wall desmoid tumor with prior history of C/S were included in the study. All of the 9 patients were female with the mean age of 30.2 (ranging from 27 to 35-year-old). The demographic data including sex, age, history of previous C/S, and outcomes were analyzed.Results:Between all the patients, one patient had three prior C/S; three patients had two and five patients had one. All of the patients received surgical excision with primary closure with clear margin. The postoperative courses were all smooth except one subsequently developed recurrence and underwent re-excision.Conclusion:Pre-operative biopsy to confirm the pathology and rule out the possibility of systemic disease is mandatory when making the diagnosis of desmoid tumor. Intervene the tumor concurrent with C/S or at postpartum period are both acceptable choices. One stage excision with wider margin and optional reconstructive modalities including mesh closure and autologous tissue reconstruction is the goal to achieve abdominal wall closure.

Volvulus of the large bowel is a relatively rare cause of colonic obstruction in Western countries, with an incidence of up to 6%.Volvulus of the colon is mostly located in the sigmoid (43-80%) and caecum (15-43%).Rarely, a volvulus of the transverse colon or splenic flexure occurs due to fixation of this part of the colon by the phrenicocolic, gastrocolic and splenocolic ligaments.Risk factors for developing large bowel volvulus are congenital anatomical variations such as increased colonic length and abnormal fixation, Chagas disease resulting in megacolon, chronic constipation, distal obstruction such as carcinoma of the sigmoid, previous abdominal surgery and pregnancy.There is a male predominance and a geographical variation, with a higher incidence in African, Asian and Eastern countries.A 39-year-old, mentally disabled man was presented to the emergency department with progressive abdominal distension and pain since day 1.He complained of nausea, vomiting and fever of up to 38.5°C.Time of last bowel movement was unclear.He had a medical history of chronic constipation, epilepsy and hydrocephalus with placement of two ventriculoperitoneal (VP) shunts at a young age.These drains needed revision because of dysfunction a few times thereafter.The patient lives in a sheltered home.During physical examination, a moderately ill man was seen, with an evident enlargement of the skull (circumference 73 cm).His consciousness was clear and there were no signs of meningitis.The abdomen was distended with high-

Background Desmoid tumors are rare locally invasive tumors, but without metastatic potential. We report a case of recurrent locally advanced desmoid tumor of the anterior abdominal wall. We discuss the place of surgery and systemic treatments in the management of desmoid tumors. Report A 51-year-old woman presented at the Visceral Surgery Department of The National University Hospital (CNHUHKM) in January 2021 for a recurrent anterior abdominal wall tumor. The recurrence occurred 3 years after a first resection performed in 2008. The mass was centered on the umbilicus and was estimated 24 cm, ulcerated and hemorrhagic. She underwent a wide resection. Pathology report concluded to an ulcerated desmoid tumor. The postoperative course was uneventful. Eight months after resection, she showed no clinical signs of recurrence. Conclusion We presented a case of recurrent desmoid tumor of the abdominal wall. Although the indications for surgery have been restricted in recent years, it remains essential treatment for well selected patients. Key words: Desmoid tumor, abdominal wall, wide resection.

Desmoid tumors are rare, musculoaponeurotic mesenchymal origin tumors arising from the proliferation of well-differentiated fibroblasts. Desmoid tumors may arise from any location with the abdominal cavity, abdominal wall and extremity locations being most frequent. We present the case of a 35-year-old female with a history of endometriosis who presented palpable abdominal mass and cyclic abdominal pain. Resection was performed for a presumed desmoid soft tissue tumor. Final pathology demonstrated desmoid histology admixed with abdominal wall endometriosis (endometrioma). This unique pathologic finding has only been rarely reported and is discussed with a brief review of the literature.

Background: Abdominal wall endometriosis is an uncommon extrapelvic form of endometriosis, most often developing at a previous gynecologic surgical scar. Patients typically report cyclical pain with or without a palpable mass, and diagnosis is frequently delayed because the presentation can mimic hernia, granuloma, lipoma, or desmoid tumor. Purpose: To describe the clinical presentation, imaging findings, perioperative optimization, surgical management, and histopathological confirmation of abdominal wall endometriosis coexisting with adenomyosis in a patient with significant medical comorbidities. Method: A case report was performed in a 45-year-old multiparous woman with a five-year history of progressive lower abdominal pain that worsened during menstruation and localized to a prior surgical scar. Pelvic ultrasonography identified adenomyosis and a 6.9 × 3.9 cm hypoechoic lesion in the anterior abdominal wall, raising suspicion of subcutaneous endometriosis. Results: Surgical exploration showed a lesion extending through the subcutaneous tissue and fascia, with focal involvement of the rectus muscle. Complete excision was achieved after preoperative optimization, and the postoperative course was uneventful. Histopathological examination of two specimens (9 × 6 × 4 cm and 5 × 2 × 1 cm) demonstrated fibrocollagenous tissue, adipose tissue, and skeletal muscle infiltrated by endometrial glands, confirming abdominal wall endometriosis. Conclusion: Abdominal wall endometriosis should be considered in women presenting with cyclical pain at a surgical scar. Imaging supports assessment of lesion extent and surgical planning, while histopathology remains essential to confirm the diagnosis and to exclude rare malignant transformation.

Case report: intra-abdominal fibromatosis in familial adenomatous polyposis

To evaluate the safety and feasibility of radical surgery for giant desmoid in abdominal wall accompanied with the repair of giant myofascial defect using synthetic prosthesis. We analyzed the clinical and follow-up data of 31 patients with giant desmoid in abdominal wall undergoing radical resection and immediate abdominal wall reconstruction with synthetic prosthesis from January 2007 to January 2017 retrospectively. Patients were recruited at the Diagnostic and Therapeutic Center of Hernia and Abdominal Wall Diseases, the First Affiliated Hospital of Chinese PLA General Hospital and the Department of General Surgery, Chinese PLA General Hospital. Operative conditions, morbidity of complication, short- and long-term outcomes were summarized. All the patients underwent radical resection and infiltrated organs or tissues were simultaneously treated. Synthetic prosthesis was used to perform primary-intention reconstruction of giant myofascial defect in anterior or lateral abdominal wall. Bridging repair procedure for incisional hernia was used to perform double border fixation between prosthesis border and myofascial defect border. Placement and fixation of prosthesis followed the idea of "conformal repair", then prosthesis was finally repaired as arch in accordance with original abdominal wall. Of 31 patients, 28 cases were female with mean age of 35.2 (16-58) years and 3 were male with mean age of 42.6 (20-79) years. Six initial cases (19.4%) were diagnosed by preoperative biopsy, and 25 recurrent cases (60.6%) were diagnosed by medical history. The mean minimal diameter of tumors was 18.2 (14-25) cm, and the mean maximal diameter was 45.3 (32-53) cm. All 31 patients underwent radical resection and immediate abdominal wall reconstruction using synthetic prosthesis in bridging fashion successfully, and rapid pathological examination showed that all resection margins were negative. The average operative time was 335 (245-610) min, and the average intra-operative blood loss was 1260 (500-3500) ml. The size of abdominal wall defect after removal of desmoid ranged from 21 cm × 23 cm to 35 cm × 60 cm. The defects in 29 patients were repaired with compound synthetic prosthesis and the defects in 2 patients were repaired with compound prosthesis and polypropylene mesh. Four patients(12.9%) developed postoperative infection, in whom 3 patients had prosthesis infection during 1 month postoperatively, then 1 case recovered with conservative therapy, the other 2 cases were healed after the removal of infected prosthesis at 2 weeks and 3 months postoperatively, respectively; 1 patient had infection of artificial vessel prosthesis and received a second operation to remove the infected artificial vessel. The other 27 patients recovered smoothly and got primary intention wound healing. These 31 patients were followed up for a median of 60.5 (10-121) months with complete data. No marginal recurrence, incisional hernia, and abdominal wall bulge happened. One patient undergoing removal of all anterior and lateral abdominal wall had difficult defecation and urination during the first month after operation, and recovered through practising chest breathing. Ten patients developed fresh desmoids in other body positions postoperatively within 1-3 years, in whom 3 patients died of intestinal obstruction due to rapid neoplasm development and 7 patients survived with tumor receiving conservative therapy. All the 28 survival patients could restore normal life and workand have appropriate sports. Radical resection and immediate reconstruction of giant myofascial defect using synthetic prosthesis for patients with giant desmoid in abdominal wall is safe and effective.

Desmoid tumor (DT) is a rare benign soft tissue neoplasm that develops in the musculoaponeurotic structures, one-third of which involve the abdominal wall. Due to local aggressive infiltration of DT, the recurrence rate is approximately 45%-77%, according to the locations of the tumors, and 25%-50% for those with unclear surgical margins. Limited by adverse effects of radiotherapy and chemotherapy, surgical excision is still the standard management recommended. Differing from traditional midline or abdominoplasty access, we applied a fleur-de-lis miniabdominoplasty access in a 37-year-old woman who had primary abdominal wall DT with less than 1 cm depth from the umbilicus. The approach not only provides a better surgical field for radical tumor excision but also eliminates redundant skin and dog-ear formation at bilateral flanks. An appropriate surgical margin could be processed simultaneously when the tumor was close to the skin surface. After abdominal wall reconstruction, the postoperative course was uneventful, and no DT recurrence or incisional hernia was noted during the follow-up. The patient was satisfied with the tumor treatment and aesthetic outcome.