S4810 Obstructive Conundrum: Desmoid Fibromatosis as a Rare Cause of Small Bowel Obstruction

Desmoid fibromatosis (DF) is a clonal proliferative disorder of myofibroblasts, which arises, with a low incidence, in soft tissue, including within the abdomen. The incidence of DF is associated with familial adenomatous polyposis (FAP), and is more common following FAP surgery. It is rare for a patient to make his/her first visit to hospital due to DF symptoms associated with FAP. In the present report, a case of mesenteric DF associated with FAP is described. This case also had incomplete intestinal obstruction due to DF. By summarizing previous studies examining DF and FAP treatment, combined with the disease characteristics of this patient, the clinical treatment strategy for DF associated with FAP was explored.

A collision tumor is a neoplastic lesion comprised of two or more distinct cell populations with distinct borders. Desmoid fibromatosis (DF) is a rare musculoaponeurotic tissue tumor that grows deep in the connective tissue and shows locally aggressive behavior. Only two cases of collision tumors with desmoid fibromatosis are reported in the English literature, albeit papillary thyroid carcinoma with desmoid fibromatosis-like stroma is regarded as a variant rather than a collision tumor. We present a unique case of collision tumor with desmoid fibromatosis surrounding intra-abdominal metastasis from urothelial carcinoma. A 65-year-old white male with history of bladder and left renal pelvis high-grade papillary urothelial carcinoma status post-nephrectomy was found to have a small bowel obstruction due to a soft tissue mass. Histology of the mass showed multiple matted lymph nodes with metastatic urothelial carcinoma admixed with a proliferation of spindle cells positive for nuclear beta-catenin, consistent with desmoid fibromatosis. While the prior surgical site likely acted as a nidus for development of desmoid fibromatosis, we also hypothesize that a dysregulation of beta-catenin signaling pathways within the cancer cells might have attributed to the spindle cell proliferation in the stroma surrounding the tumor. Our case emphasized the importance of clinical suspicion of desmoid fibromatosis in patients with metastatic cancer, requiring a prompt diagnosis and treatment to decrease the risk of complications and local recurrence.

Objective To study the clinical effect of the postoperative abdominal wall reconstruction with synthetic mesh for abdominal desmoid type fibromatosis (DTF). Methods Clinical data of 11 patients with abdominal desmoid type fibromatosis during February 2012 to February 2016 in Beijing Chao-Yang Hospital were retrospectively collected. The abdominal wall of the patients who underwent tumor radical surgery were reconstructed with synthetic mesh. Results All the patients were successfully treated with synthetic mesh to reconstruct the abdominal wall. The mean operation time was 97±33 min, and the mean hospitalization was 19.2±3.1 days. All the patients recovered uneventfully. No wound infections and foreign body feelings were recorded in our study. Subcutaneous hydrops occurred in 1 patient who finally recovered uneventfully by puncture aspiration. During the follow-up time of 7-60 months (the median follow-up time was 31.8±18.5 months), there were no mortalities, and 1 patient suffered tumor recurrence. There were no mesh infections and mesh-related complications. No incisional hernias and intestinal obstructions were observed. Conclusion Abdominal wall reconstruction with synthetic mesh shows favorable clinical effect. Key words: abdominal desmoid type fibromatosis; abdominal reconstruction; synthetic mesh

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...

Intra-abdominal desmoid fibromatosis (IADF) is a rare condition, comprising 8-15% of all presentations of desmoid fibromatosis (DF), most commonly arising from the small bowel mesentery. We report the case of a 34 year old male, who presented with a 7.4 x 6.5 cm right iliac fossa mass arising from the omentum. Percutaneous biopsy was inconclusive and the working diagnosis was desmoplastic small round cell cancer of appendiceal or urachal origin. He underwent a diagnostic debulking procedure; a dominant omental mass was removed and 2-3 mm peritoneal nodules on the distal small bowel mesentery and a bulky mass around the root of the superior mesenteric vessels were noted. Histopathological, immunohistochemical and gene mutation analysis confirmed IADF as the definitive diagnosis. We review the literature of this rare presentation and discuss challenges in diagnosis and subcategorization of DF.

In the following report, we present the case of a 31-year-old patient examined for a suspected small bowel tumour. Small bowel tumours are relatively rare and definitive diagnosis is often preceded by surgical resection. The same was true for our patient examined for a soft tissue lesion found with imaging techniques in close proximity to the small intestine. After ruling out hormonal activity, diagnostic laparotomy was performed. Desmoid fibromatosis, a rare tumour of the mesentery, was confirmed histopathologically. Although without metastatic potential, desmoid fibromatosis is a tumour with moderately malignant potential for aggressive growth into surrounding structures and organs and a large percentage of local recurrences.

Aggressive fibromatosis is a rare type of intra-abdominal desmoid tumour that usually involves the small bowel mesentery. It is a locally-invasive lesion, with a high rate of recurrence, but without metastatic potential. Aggressive fibromatosis is seen more often in young female patients. This case report presents the radiological, intraoperative and histopathological findings from a 37-year-old female patient that presented with epigastric pain and a palpable mass in the right hemiabdomen. Histological and immunohistochemical examinations of the resected tumour, including positive staining for beta-catenin, confirmed a postoperative diagnosis of desmoid type fibromatosis. This specific case showed that desmoid type fibromatosis of the colon can mimic gastrointestinal stromal tumours (GIST) based on its clinical presentation, computed tomography and magnetic resonance imaging findings. Differential diagnosis between desmoid type fibromatosis and GIST is clinically very important due to the different treatments and follow-up protocols that are implemented for these lesions.

This case report describes a 54-year-old man who presented with abdominal discomfort, nausea, early satiety, and constipation. A CT scan revealed a large mass infiltrating the mesentery and close to bowel loops, raising suspicion of intestinal sub-occlusion. Urgent surgery was performed, resulting in a segmental jejunum resection to remove the identified mesenteric mass. Histological examination revealed a diagnosis of desmoid fibromatosis which is a rare type of soft tissue tumor with borderline malignancy that can cause bowel obstruction when it develops intra-abdominally. It is often associated with genetic predispositions and risk factors such as previous surgery and pregnancy. The case highlights the importance of considering a patient's surgical history when encountering desmoid fibromatosis to determine the most appropriate treatment approach.

A desmoid tumor is a rare neoplasm that is derived from soft tissues. Although it shows benign characteristics pathologically, local recurrence can occur. We herein report the case of a patient with an intraabdominal desmoid tumor that developed 3 years after laparoscopic appendectomy for acute appendicitis. A 59-year-old male visited our emergency room with complaints of abdominal pain and fullness. Abdominal computed tomography revealed distention of the small intestine with a point of obstruction by an intraabdominal tumor-like region. Pathological findings showed that the tumor was compatible with desmoid fibromatosis. In cases with an intraabdominal tumor after laparoscopic surgery, it is important to consider the possibility of a desmoid tumor, since it is difficult to diagnose it accurately before surgery.

Desmoid fibromatosis is a rare benign neoplasm of the soft tissue. Primary desmoid neoplasms rarely occur in the small bowel and are primarily found in patients with a previous abdominal surgery or irradiation history. They are challenging to diagnose at the time of presentation due to a lower incidence and their non-specific presentation making it difficult to distinguish from other intra-abdominal neoplasms, such as gastrointestinal stromal tumors (GISTs), which may present with similar symptoms. We like to present a case of a 34-year-old male with a four-day history of abdominal pain with worsening severity and one episode of non-bloody vomiting. Physical examination was significant for generalized abdominal tenderness with positive rebound and board-like rigidity. A computed tomography (CT) scan of the abdomen showed the presence of a lower abdominal mass of unknown etiology with free air foci and free intraperitoneal fluid either due to rupture of the suspicious mass or secondary to infection by an air-producing organism. The patient was immediately taken for emergency surgery, the tumor was resected successfully, and a specimen collected was sent for histopathology, which came out to be a desmoid tumor. We aim to highlight the importance of keeping a broad differential diagnosis in a patient with acute abdomen and symptoms of peritonitis.

Introduction. Aggressive fibromatosis, also known as desmoid type fibromatosis (DF) is a locally aggressive fibroblastic neoplasm that can arise anywhere in the body with no potential for metastasis and a high recurrence rate after surgical resection. Mesenteric fibromatosis are locally aggressive DF of the mesentery with a high propensity for bowel involvement. The real etiology of these tumors remains unknown, occurring sporadically or in association with familial adenomatous polyposis (FAP), as Gardner?s syndrome. Case report. A 34-year-old female patient presented with a palpable solid tumefactive mass in the left hemiabdomen. Contrast enhanced computed tomography (CT) and magnetic resonance imaging (MRI) revealed multiple massive solid tumefactions in the mesentery and in between the small bowel loops. Colonoscopy confirmed the presence of multiple sessile polyps characteristic of FAP. Tissue samples of the mesenteric mass were acquired via ultrasound guided biopsy with histopathologic confirmation of desmoid fibromatosis with imunohistochemical analysis. The risk of surgery was deemed too high at the time due to the size of the mass and proximity to mesenteric vascular structures, therefore the patient was planned for chemotherapy with a potential for further surgical reevaluation. Conclusion. Mesenteric fibromatosis is a rare neoplasm that presents with a wide range of histologic and imaging features. CT and MRI play a crucial role in evaluation and planning an optimal treatment model for patients with mesenteric fibromatosis.

Small bowel faeces sign in patients without small bowel obstruction

49-Year-Old Woman With Acute Abdominal Pain and Nausea

The incidence of bowel obstruction in pregnancy is approximately 1 in 17 000 deliveries and is not increased in comparison to the non-pregnant population1. Approximately 50% of cases result from postsurgical adhesions, including those associated with Cesarean delivery1, 2. Small bowel obstruction is considered a catastrophic complication of pregnancy with a reported overall risk of fetal loss of 17% and a maternal mortality rate of 2%2. Sonography has been demonstrated to be superior to plain radiography in the assessment of non-pregnant patients with suspected small bowel obstruction3, however, diagnosis is considered more difficult during pregnancy2. Here we describe two cases of maternal small bowel obstruction diagnosed during pregnancy by point-of-care transabdominal ultrasound. A 30-year-old primiparous woman presented at 27 weeks' gestation, complaining of abdominal discomfort, nausea, vomiting and obstipation. Her medical history included right salpingectomy for a tubal pregnancy. Transabdominal ultrasound examination was performed and the fetus was considered appropriate in size for gestational age (AGA). Markedly dilated loops of maternal small bowel containing fluid and edematous bowel wall were observed (Figure 1). Prominent ‘to and fro’ peristalsis was visualized in the small bowel and free fluid was noted in the cul-de-sac. Maternal small bowel obstruction was suspected and was confirmed by computed tomography. Following unsuccessful conservative treatment, laparotomy was performed and a mechanical obstruction due to adhesions was identified and released. On the third postoperative day, the patient delivered precipitously a 1260-g infant. Both mother and infant were well at the time of writing. A 26-year-old primiparous woman presented at 34 weeks' gestation with worsening abdominal discomfort, nausea, vomiting and obstipation. Her medical history included laparoscopic cholecystectomy and right ovarian cystectomy. Serum levels of aspartate aminotransferase and alanine aminotransferase were elevated at 113 U/L and 175 U/L, respectively. Qualitative urine dipstick analysis revealed proteinuria of 2+ and ketonuria of 4+. Transabdominal ultrasound examination showed the fetus to be AGA. Sonography of the maternal upper abdomen revealed numerous dilated loops of small bowel, edematous small bowel walls (Figure 2), marked ‘to and fro’ peristalsis and free fluid in the peritoneal cavity. Small bowel obstruction was suspected and was confirmed by magnetic resonance imaging. The patient received 4 L of intravenous fluids and concurrently manifested hypertension, leading to a suspected diagnosis of pre-eclampsia. Following failed conservative treatment of the bowel obstruction, she was scheduled for exploratory laparotomy. Prior to surgery, elevated blood pressure of 170/110 mmHg confirmed severe pre-eclampsia. The patient requested and underwent Cesarean delivery of an infant weighing 2260 g. Following repair of the uterine incision, an internal hernia with an incarcerated segment (hyperemic, edematous, with punctate lesions and prenecrotic appearance) of small bowel, located within previous surgical adhesions, was released. Both mother and infant did well. Abdominal ultrasound performed at the patient's bedside (‘point of care’) for the diagnosis of small bowel obstruction is a recent application in the emergency room4. Specific sonographic findings associated with small bowel obstruction include diameter of the small bowel > 25 mm, small bowel wall edema, ‘to and fro’ peristalsis, intra-abdominal fluid and the presence of a sonographic transition point (defined as the location between dilated small bowel proximal to the obstruction and decompressed small bowel distal to the obstruction)5. In both our cases, all of these sonographic signs of small bowel obstruction, other than depiction of the transition point, were present despite the gravid uterus. In both patients, the transition/obstruction points that were confirmed at surgery were posterior to the gravid uterus, thus were obscured to transabdominal sonographic assessment. A systematic search (PubMed, MEDLINE) of articles published between 1966 and 2016 in English medical literature, utilizing the search terms ‘pregnancy’, ‘small bowel obstruction’, ‘mechanical ileus’, and ‘point-of-care sonography’, indicates that the diagnosis of small bowel obstruction during pregnancy utilizing point-of-care sonography has not been reported previously. Our cases suggest that point-of-care sonography, now advocated for patients with suspected small bowel obstruction, may also be beneficial in pregnant patients at relatively advanced gestational ages. D. M. Sherer*†, M. Dalloul†, A. Schwartzman‡, A. Strasburger§, R. A. Farrell†, H. Zinn¶ and O. Abulafia§ †Department of Obstetrics and Gynecology, Division of Maternal-Fetal Medicine, State University of New York (SUNY), Downstate Medical Center, 450 Clarkson Avenue, Box 24, Brooklyn, NY, USA; ‡Department of Surgery, State University of New York (SUNY), Downstate Medical Center, Brooklyn, NY, USA; §Department of Obstetrics and Gynecology, Division of Gynecological Oncology, State University of New York (SUNY), Downstate Medical Center,Brooklyn, NY, USA; ¶Department of Radiology, State University of New York (SUNY), Downstate Medical Center,Brooklyn, NY, USA *Correspondence. (e-mail: [email protected])

Strangulated Small Bowel Obstruction Following Endoscopic Retrograde Cholangiopancreatography