[A Case of Desmoid-Type Fibromatosis of the Mesentery of Small Intestine].

Desmoid-type fibromatosis is a relatively rare disease, often associated with familial adenomatous polyposis and a history of abdominal surgery. A 43-year-old male patient presented with abdominal pain and contrast-enhanced CT showed a mass in the lower abdomen. The mass was a 4×4×3 cm white, dense tumor with a wreath-like arrangement of eosinophilic spindle-shaped cells. Immunostaining showed KIT(-), CD34(-), desmin(-), ß-catenin(+), SMA(few+), and the diagnosis was desmoid-type fibrosis. Six months after surgery, there was no apparent recurrence.

[A Case of Desmoid Fibromatosis of the Small Intestinal Mesentery Institutions].

The patient was a 27-year-old man. He was referred to our hospital because he was aware of a mass in his abdomen. An abdominal ultrasound showed a 70-mm mass lesion. Enhanced computed tomography showed a 70-mm mass with well- defined margins and heterogeneous internal enhancement near the proximal jejunum. The patient was diagnosed with a suspected primary submucosal tumor of the duodenum or small intestine, and surgery was planned to diagnose and treat the tumor. The tumor was located in the upper jejunal mesentery, and tumor resection and partial small bowel resection were performed. Histopathological examination revealed proliferation of spindle-shaped cells without karyomitosis, and mixed collagen fibers in the tissue. Immunohistochemistry showed ß-catenin(+), SMA(+), AE1/AE3(-), KIT(-), CD34(-), and S-100(-). Based on these findings, we diagnosed primary desmoid fibromatosis of the small intestinal mesentery. In this report, we describe a case of primary desmoid fibromatosis of the small intestinal mesentery with a review of the literature.

Treatment of a Desmoid Tumor That Enlarged During Pregnancy: A Case Report and Literature Review.

Desmoid tumors are rare soft-tissue tumors that exhibit locoregional aggressiveness and a high local recurrence rate following initial resection. No fixed recommendations have been established with regard to the timing and method of treatment for desmoid tumors that enlarge during pregnancy. Desmoid tumors tend to enlarge during pregnancy, and most do not regress spontaneously postpartum. Thus, surgery may be required even during pregnancy. We report a case of an abdominal wall desmoid tumor that grew to 90 mm during pregnancy and was resected at 17 weeks of gestation. Marginal resection was performed, and the surgical margin was microscopically positive. The postoperative course and the pregnancy were uneventful, and no recurrence was observed at the 15-month follow-up visit.

[A Case of Long-Term Survival with Tumor Resection and DOX plus DTIC Therapy for Desmoid Tumor after FAP Surgery].

The patient is a 22-year-old, female. She had a family history of familial adenomatous polyposis(FAP)and a prophylactic total colorectal resection was performed for FAP at age of 18. She presented with fever and abdominal distention and palpated a mass with tenderness in the right lower abdomen. Contrast-enhanced CT scan of the abdomen showed a heterogeneous contrast effect around the tumor margins. With the diagnosis of intra-abdominal desmoid tumor, a partial duodenal resection, small bowel mass resection, and right fallopian tube resection were performed along with the tumor, and an artificial anus was created with the jejunum. Contrast-enhanced CT scan of the abdomen 16 months after resection of desmoid tumor showed a 6.5 cm long desmoid tumor recurrence in the mesentery. She received 5 courses of doxorubicin (DOX)plus dacarbazine(DTIC)therapy followed by continued NSAIDs. Seven years after the operation, she has been able to maintain the shrinkage of the recurrent tumor and is still on medication. Long-term surveillance is necessary because of the possibility of the appearance of other associated lesions in the future.

Fibromatosis del recto anterior del abdomen. Reporte de caso / Fibromatosis of the rectus abdominis. Case report

La fibromatosis es un tumor fibroblástico benigno, poco frecuente. El músculo recto anterior del abdomen es uno de los músculos de la pared abdominal más habitualmente afectado. Es un tumor con una alta frecuencia en la invasión local y recurrencia. La marcación para ß-catenina en el inmunofenotipo certifica el diagnóstico. Los márgenes negativos microscópicos (R0) deben ser el objetivo de la resección quirúrgica. La edad joven, el gran tamaño tumoral y los márgenes positivos son parámetros asociados a la recurrencia local. Presentamos el caso clínico de una paciente joven a la cual en el contexto de una abdominoplastia se le diagnostica una tumoración en el sector medio del recto anterior del abdomen izquierdo. Se hizo una biopsia incisional que certificó que correspondía a una fibromatosis abdominal (FA). Confirmado el diagnóstico se realizó resección de la tumoración y reparación de la pared abdominal con malla de polipropileno.

Fibromatosis is a rare, benign fibroblastic tumor. The rectus abdominis muscle is one of the most commonly affected abdominal wall muscles. It is a tumor with a high frequency of local invasion and recurrence. Marking for ß-catenin in the immunophenotype certifies the diagnosis. Microscopic negative margins (R0) should be the target of surgical resection. Young age, large tumor size, and positive margins are parameters associated with local recurrence. We present the clinical case of a young patient who, in the context of an abdominoplasty, was diagnosed with a tumor in the middle sector of the left rectus abdominis. An incisional biopsy was performed, which certified that it corresponded to abdominal fibromatosis (AF). Once the diagnosis was confirmed, the tumor was resected and the abdominal wall repaired with polypropylene mesh

[A Case of Giant Abdominal Desmoid Tumor in a Young Man].

Desmoid tumor is a rare tumor of the soft tissue. The frequency of occurrence is 2.4 to 4.3 cases per year per million people, which is a very rare disease. We experienced a huge intra-abdominal desmoid tumor which is thought to be the primary mesentery. The case was a male in his 20s. He visited a nearby doctor with a complaint of abdominal bloating and abdominal pain. Abdominal contrast CT revealed a huge abdominal mass with a clear boundary of 35×25 cm in size extending from the upper right abdomen to the pelvis. Surgery was performed with a diagnosis of an intra-abdominal mass. Open abdominal tumor resection. Due to infiltration into the duodenum, transverse colon, and pancreas, right hemicolectomy and duodenal combined resection were performed. The pathological diagnosis was a diagnosis of desmoid tumor.

Surgical management of abdominal desmoids: a systematic review and meta-analysis.

BACKGROUND: Desmoid tumours are benign fibromatous tumours arising from dysregulated myofibroblast proliferation within musculoaponeurotic structures. They can occur sporadically but more commonly are associated with genetic syndromes such as familial adenomatous polyposis (Sakorafas et al. in Surg Oncol 16(2):131-142, 2007) (FAP). Mutations in either the Wnt, ß-catenin or APC genes are 'key' triggers for the development of these tumours (Howard and Pollock in Oncol Ther 4(1):57-72, 2016). Classically, these tumours do not metastasise; however, they are associated with significant morbidity and mortality due to their infiltrative pattern and/or local invasion. Historically, surgical resection was the cornerstone of treatment. There remains paucity of data regarding outcomes following the surgical management of abdominal desmoid tumours in terms of success, recurrence and morbidity. OBJECTIVES: The aim of this review was to assess the current evidence for surgical management of abdominal desmoid tumours in terms of success, recurrence and morbidity. METHODS: A systematic search of articles in PubMed, EMBASE and The Cochrane Library databases was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines for the period from January 2000 to November 2020. RESULTS: Twenty-three studies were included, of which, 749 patients had surgical resection (696 for primary and 53 for recurrent desmoids), 243 patients (18.8%) were medically managed and 353 patients (27.3%) underwent surveillance. Median follow-up was 51.4 months (range 1-372). Six-hundred and ninety-six of the 749 resections (92.9%) underwent primary desmoid resection, with the remaining 53 (7.1%) undergoing resection for recurrence. One-hundred and two surgically managed patients (19%) developed a (re)recurrence, with mesenteric involvement the commonest site for recurrence (55%). When comparing recurrence post-surgery to progression following medical therapy, there was a trend towards better outcomes with surgery, with 25% of surgical patients having a recurrence versus 50.5% having progression with medical therapy [OR 0.40 (95% CI 0.06-2.70), p = 0.35]. Major morbidity following surgery was 4.4% (n = 33) with 2% (n = 14) mortality within 30 days of resection. CONCLUSION: The management of desmoids has considerable heterogeneity. Surgical resection for abdominal desmoids remains a valid treatment option in highly selective cases where negative margins can be obtained, with low major morbidity and/or mortality.

Large Intra-Abdominal Desmoid Tumor in a Deployed Soldier Initially Presented With Chronic Diarrhea.

Diarrhea is a common condition seen among soldiers in both garrison and deployed environments. Although the vast majority of soldiers with diarrhea will recover uneventfully with supportive care, clinicians should also maintain suspicion for less common causes and perform a thorough physical exam. We report the case of a young, healthy soldier with chronic diarrhea and progressively worsening abdominal distention that began during his deployment to Honduras who was subsequently found to have a large intra-abdominal desmoid tumor. Desmoid tumor is a rare and benign neoplasm that typically appears on the extremity, abdominal wall, intra-abdominal space, and occasionally in the chest wall. This tumor may be associated with abdominal distension and gastrointestinal complaints. A large tumor can compress organs, causing local tissue damage and, in rare cases, death.

Intra-abdominal desmoid tumors in familial adenomatous polyposis: How much do clinical and surgical variables interfere with their development?

OBJECTIVE: Familial Adenomatous Polyposis is a complex hereditary disease that exposes the carrier to a great risk of Colorectal Cancer (CRC). After prophylactic surgery, intra-abdominal desmoid tumors are known to be one the most important cause of death. Therefore, recognition of increased-risk patients and modification of operative strategy may be crucial. AIM: The objective of this study was to estimate the desmoid tumor risk in relation to various surgical and clinical variables. METHODS: Patients who had undergone polyposis since 1958 were included in the study. After exclusion criteria were met, those who had developed desmoid tumors were selected to undergo further evaluation. RESULTS: The study revealed that the risk of developing desmoid tumors was associated with various factors such as sex ratio, colectomy, and reoperations. On the other hand, the type of surgery, family history, and surgical approach did not affect the risk of developing desmoid tumors. The data collected from 146 polyposis patients revealed that 16% had desmoid polyps. The sex ratio was 7:1, and the median age at colectomy was 28.6 years. Family history, multiple abdominal operations, and reoperations were some of the characteristics that were common in desmoid patients. CONCLUSION: Recognition of clinical (female sex) and surgical (timing of surgery and previous reoperations) data as unfavorable variables associated with greater risk may be useful during the decision-making process.

Unusual and rare cause of abdominal pain: abdominal desmoid fibromatosis masquerading as a postoperative haematoma.

Desmoid fibromatosis is a rare connective tissue malignancy. It can occur in a variety of locations, including the abdominal wall, extremities and abdominal cavity. There has been an association with development in a prior surgical scar. Common symptoms can vary depending on the location and can include being painless to having pain at the site, functional impairment and bowel obstruction from intra-abdominal masses. In the following report, we discuss a case in which a patient's abdominal pain was attributed to a postoperative haematoma based on CT radiographic features; however, further work-up and biopsy yielded desmoid fibromatosis, a rare locally aggressive malignancy.

Management of unresectable and recurrent intra-abdominal desmoid tumors treated with ultrasound-guided high-intensity focused ultrasound: A retrospective single-center study.

To assess the efficacy and safety of ultrasound (US)-guided high-intensity focused ultrasound (HIFU) ablation for treatment of unresectable and recurrent intra-abdominal desmoid tumors. From June 2014 to March 2020, 15 patients with consecutive unresectable and recurrent diseases that pathologically proven to be intra-abdominal desmoid tumors had undergone the treatment of US-guided HIFU ablation. All patients underwent contrast-enhanced magnetic resonance imaging before and after HIFU treatment. Nonperfused volume ratio was used to evaluate the effect of HIFU therapy. Intraprocedural and postprocedural adverse effects and complications are recorded to assess the safety of the therapy. Outcome of HIFU ablation has been investigated through serial contrast-enhanced imaging examinations during follow up. Out of 15 patients 14 of them have successfully completed the whole therapy, 1 patient is ineffective and gives up further treatment. The mean nonperfused volume ratio is 71.1% (95% confidence interval, 3% to 88.2%). During a mean follow up of 29 months (range from 8 to 61 months), the mean tumor volume was reduced by 59% (95% confidence interval, +49% to -100%). No tumor spreads along the treated area in all patients except one. Complications have occurred in 5 patients (33.3%), including bowel rupture (1 case), intra-abdominal abscess (1 case), slight injury to the femoral nerve (1 case), and bone injury (2 cases), the bowel rupture patient underwent surgery; the others have been cured during the follow up. US-guided HIFU ablation is an effective treatment modality for patients suffered from unresectable and recurrent intra-abdominal desmoid tumors.

Fibromatosis mesentérica solitaria. Reporte de caso y revisión de la literatura / Sporadic mesenteric fibromatosis. A case report. Literature review / Fibromatose mesentérica solitária. Relato de caso. Revisão da literatura

Resumen: La fibromatosis mesentérica es un subtipo profundo de tumor desmoide (TD), un tumor benigno de origen fibroblástico localmente agresivo por su tendencia a infiltrar los tejidos adyacentes. Son raros, esporádicos y pueden asociarse con el síndrome de Gardner. El tratamiento de elección es la resección completa, evitando la recurrencia local. Comunicamos el caso clínico de una paciente con fibromatosis intrabdominal mesentérica única, bien circunscripta, que simulaba por la imagenología una masa de origen pelviano.

Summary: Mesenteric fibromatosis is a deep sub-type of desmoid tumors consisting of a benign tumor of fibroblastic origin which is locally aggressive given its tendency to infiltrate adjacent tissues. They are unusual and sporadic, and may be associated to Gardner's Syndrome. Complete resection is the treatment of choice, avoiding local recurrence. The study reports the clinical case of a patient with intra-abdominal sporadic mesenteric fibromatosis, well circumscribed that appeared to be a pelvic mass in MR imaging.

Resumo: A fibromatose mesentérica é um subtipo profundo de tumor desmóide (DT); é um tumor benigno de origem fibroblástica que é localmente agressivo devido à sua tendência a infiltrar tecidos adjacentes. São raros, esporádicos e podem estar associados à síndrome de Gardner. O tratamento de escolha é a ressecção completa, evitando recidiva local. Relatamos o caso clínico de uma paciente com fibromatose mesentérica intra-abdominal única e bem circunscrita que simulava uma massa de origem pélvica na imagem.

Large desmoid tumour of the small bowel mesentery.

A 74-year-old man was being investigated for a pancreatic insulinoma when an incidental mesenteric mass measuring 2.6 cm x 2.5 cm was noticed on CT imaging. A wait-and-see approach was decided on. Thirty-nine months later, the patient presented with symptoms of abdominal obstruction. CT images revealed the mesenteric mass filled majority of the abdominal cavity and measured 29 cm x 26 cm x 16 cm. The patient underwent an open bypass gastrojejunostomy which stopped working a few weeks later due to further compression by the tumour. A debulking surgery was performed: a right hemicolectomy and small bowel resection with excision of the desmoid tumour and bypass gastrojejunostomy. The tumour measured 12.6 kg and was macroscopically visualised to have a white cut surface with a focal translucent area. Microscopic analysis revealed bland spindle cells with pale eosinophilic cytoplasm showing no cytological atypia, in keeping with a mesenteric desmoid tumour. Currently, two and a half years from the debulking surgery, the patient remains well and in remission with planned surveillance.

[A Case of Intra-Abdominal Desmoid Tumor after Laparoscopic Resection of Colon Cancer].

An 84-year-old man underwent laparoscopic descending colon resection for colon cancer with stage Ⅰ. Follow-up computed tomography(CT), 18 months after surgery showed a soft tissue density nodular mass, 30 mm in size, in the small intestinal mesentery. The surgical resection of the tumor was performed after a thorough examination. Histopathological examination showed spindle-shaped fibroblasts and abundant collagen fibers. Immunohistochemical staining was negative for c-kit and CD34 and positive for α-SMA and ß-catenin. From the above, this tumor was diagnosed as intra-abdominal desmoid tumor.