[A Case of Intra-Abdominal Desmoid Tumors Occuring Four Years after Open Radical Prostatectomy].

Desmoid tumor is one kind of fibromatosis, and much occurs the abdominal wall and outside the abdominal wall. Intra- abdominal desmoid tumor is rare at about 8%. We experienced a case of intra-abdominal desmoid tumors occurring 4 years after open radical prostatectomy with some literature review. A 72-year-old man had undergone open radical prostatectomy for prostate cancer. Four years after that resection, multiple intra-abdominal tumors measuring 56 mm in maximum diameter was identified on follow-up computed tomography, and he was referred to our department for management. We performed laparotomy and investigation of the biopsy. Immunohistochemistry of the resected specimen indicated the tumor cells were positive for vimentin and ß-catenin, and the diagnosis was desmoid. We performed partial resection of the small intestine and ileocecal resection. His postoperative course was uneventful and he was discharged on the 12th postoperative day. He has shown no sign of recurrence in the 4 months follow-up since surgery. In the past, an operation was the best treatment for intra-abdominal desmoid tumor. But it is reported that watchful waiting is also possible by the case which has no symptom and dysfunction in NCCN guidelines 2019. Further research is needed.

An Intra-abdominal Solid-cystic Desmoid That Emerged after Distal Gastrectomy.

Desmoid is a locally aggressive fibroblastic neoplasm, typically showing a heterogeneous solid mass, and its pathogenesis is multifactorial, including surgical scars. We herein report a rare case of an intra-abdominal desmoid, consisting of solid and cystic components covered with epithelial linings, that emerged after distal gastrectomy. The preoperative diagnosis was inconclusive, so laparotomy was performed. Histopathology of the solid component showed proliferating spindle cells, which were positive for beta-catenin in their nuclei. Clinicians need to bear in mind that desmoids can appear in a solid-cystic form, and immunostaining of beta-catenin should be applied for tumors that emerge around postoperative wounds.

Not all abdominal masses after colorectal cancer surgery are malignant: intra-abdominal fibromatosis masquerading as recurrence.

AIM: Intra-abdominal fibromatosis is an unusual mesenchymal tumour that can be locally aggressive without any metastatic potential. Fibromatosis may simulate cancer recurrence on imaging surveillance for colorectal cancer follow-up. The optimal treatment of recurrent peritoneal malignancy is cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). Confirmatory biopsy of lesions suspicious for colorectal cancer recurrence may not be feasible, thereby rendering surgery the safest option. Our aim was to determine the incidence of fibromatosis in a cohort of patients undergoing CRS and HIPEC for suspected colorectal cancer recurrence. METHODS: One hundred and seventy-one CRS and HIPEC cases were performed at our Peritoneal Malignancy Institute between February 2007 and October 2018 for colorectal peritoneal metastases and were included in a prospectively maintained database. RESULTS: A total of 49 (29%) of 171 cases were performed for primary colorectal cancer with peritoneal metastases, whereas 122 (71%) of 171 cases were performed for suspected colorectal cancer recurrence detected on surveillance imaging after primary colorectal cancer resection. On histological analysis of the resected specimen, five (4.1%) of 122 cases undergoing CRS and HIPEC for colorectal recurrence had fibromatosis. CONCLUSION: Fibromatosis can masquerade as colorectal cancer recurrence. In this series it occurred with an incidence of 4.1% among a cohort of patients undergoing CRS and HIPEC for probable recurrence. Surgical resection may be the only option to confirm the diagnosis and rule out malignancy.

Reconstruction of a large full-thickness abdominal wall defect with flow-through-based alt flaps: A case report.

An extensive 35 × 20 cm sized full-thickness abdominal wall defect was created after resection of aggressive abdominal fibromatosis in a 19-year-old male patient. Immediate reconstruction was not possible due to prolonged operation time and resulting severe bowel edema. A silicone sheet with NPWT was applied over the exposed viscera. After 1 week, silicone sheet was substituted with a composite mesh. Then, abdominal wall reconstruction with bilateral free anterolateral thigh (ALT) flaps (30 × 12 cm and 25 × 12 cm sized) was performed. Since there was only a single reliable recipient vessel available, we linked 2 ALT free flaps sequentially in a flow-through fashion to the left inferior epigastric artery and vein. Two donor sites were closed primarily. The flap fully survived and the defect was covered successfully without any complication for 11 months of follow up. Multiple flaps may be needed for large full-thickness abdominal wall defect coverage. Linked fasciocutaneous free flaps could be a solution with a less donor site morbidity even in the case of limited available recipient vessels. The purpose of this study is to introduce our experience of extensive full-thickness abdominal wall reconstruction using only ipsilateral deep inferior epigastric vessels.

Mesenteric fibromatosis.

Mesenteric fibromatosis is a rare, locally invasive, non-metastasizing type of intra-abdominal fibromatoses with a very high rate of recurrence. Because of rarity, these tumors pose a diagnostic and therapeutic challenge. These tumors may remain asymptomatic, but commonly present as a painless mass. There are no clear treatment guidelines. Surgical excision with wide margin is the preferred modality of treatment. Medical therapy is indicated for inoperable and recurrent tumors. There are several isolated case reports on mesenteric fibromatosis with different and unusual presentations and its complications. Though several studies have been published on extra-abdominal fibromatosis as well as on extramesenteric abdominal fibromatosis, but extensive research studies are still lacking on mesenteric fibromatosis. The aim of this article is to present current knowledge on mesenteric fibromatosis, our experience of four cases, and comprehensive review of available literature.

Subsequent intra-abdominal fibromatosis mimicking recurrent gastrointestinal stromal tumor.

Intra-abdominal fibromatosis (IAF) commonly develops in patients who had abdominal surgery. In rare instances, it occurs subsequent to gastrointestinal stromal tumor (GIST). This special situation has clinical significance in imatinib era. About 1000 patients with GIST in our institution from 1993 to 2010 were re-evaluated based on their clinical and pathological data, the treatment strategies and the follow-up information. We identified 2 patients who developed IAF after GIST resection. Patient 1 was a 54 year-old male and had 5 cm × 4.5 cm × 3.5 cm jejunal GIST excised on February 22, 1994. Three years later, an abdominal mass with 7 cm × 6 cm × 3 cm was identified. He was diagnosed as recurrent GIST from clinical point of view. After excision, the second tumor was confirmed to be IAF. Patient 2 was a 45-year-old male and had 6 cm × 4 cm × 3 cm duodenal GIST excised on August 19, 2008. One year later, a 4 cm mass was found at the original surgical site. The patient refused to take imatinib until the tumor increased to 8 cm six months later. The tumor continued to increase after 6 months' imatinib therapy, decision of surgical resection was made by multidisciplinary team. The second tumor was confirmed to be IAF with size of 17 cm × 13 cm × 11 cm. Although IAF subsequent to GIST is very rare, it is of clinical significance in imatinib era as an influencing factor for making clinical decision. VIRTUAL SLIDES: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1076715989961803.

Abdominal wall fibromatosis associated with previous laparoscopic hernia repair.

CASES: Two cases of desmoid-type fibromatosis developing after laparoscopic hernia repair are described: one in a young male 3 years after laparoscopic umbilical hernia repair and the other in a young female 1 year after laparoscopic incisional hernia repair. FINDINGS: The male patient presented with a slowly enlarging non-tender firm abdominal wall mass; the female patient had similar findings. Excision biopsy in the male and core biopsy in the female were consistent with fibromatosis. TREATMENT: The young male patient underwent resection of the fibromatosis, and the female patient has been managed conservatively. RELEVANCE TO CURRENT KNOWLEDGE: These are the first documented cases of fibromatosis developing after laparoscopic hernia surgery. Whilst the safety of hernia meshes has been assessed in animal studies, it may be that more detailed study of intraperitoneal placement of these meshes is required.

Successful radiofrequency ablation of an anterior abdominal wall desmoid in familial adenomatous polyposis.

BACKGROUND: Desmoid tumours are an important cause of mortality in familial adenomatous polyposis (FAP). There are few effective treatment strategies. This report describes the use of radiofrequency ablation to debulk and palliate an abdominal wall desmoid tumour in FAP. METHODS: A 22 year old woman with FAP developed a large abdominal wall desmoid tumour after restorative proctocolectomy. The tumour was treated with 16 separate radiofrequency ablations. The follow up was 36 months from the first ablation. RESULTS: The procedure was well tolerated with minor complications; mild superficial cellulitis and skin ulceration occurred following only one of the ablation sessions. Repeated radiofrequency treatments resulted in a sustained reduction in size and symptoms from the desmoid tumour. CONCLUSION: Given the low efficacy of treatments for desmoids in FAP, radiofrequency ablation appears to be a promising modality.

The association between Crohn's disease and desmoid tumors: a novel case and review of the literature.

Desmoids are rare tumors resulting from the proliferation of fibroblasts. They occur in association with familial adenomatous polyposis (FAP), but they may also occur in the post-traumatic peri-partum or post-abdominal surgery setting, and a few present spontaneously. Presenting features of desmoids are protean and largely relate to the anatomical area of involvement. We describe a 50 year old male not known to have Crohn's disease and without FAP who presented with multiple desmoids. Investigation of post-operative diarrhea confirmed a diagnosis of Crohn's disease. This is the first report of a male patient, who had never undergone prior abdominal surgery, presenting with Crohn's disease and abdominal desmoid tumors. The reasons why Crohn's disease and desmoids may be associated are explored, focusing particularly on alternations in the fibrogenic cytokine TGF-ß now known to be involved in the pathogenesis of both diseases.

Multimodality management of desmoid tumors: how important is a negative surgical margin?

Desmoid tumors are rare, locally invasive mesenchymal tumors without metastatic potential. Their clinical behavior is heterogeneous and characteristically unpredictable; outcomes are influenced by anatomic location, proximity to vital organs, association with familial adenomatous polyposis. Surgery is the main treatment modality, but the significance of positive resection margins remains controversial since they may not increase the risk of recurrence: in this setting re-resection, adjuvant radiation or close clinical follow-up could all be appropriate options. We reviewed the current evidence for multimodality therapy of desmoids, with a focus on the importance of resection margins, and present our own algorithm for treatment.

Risk factors for development of desmoid tumours in familial adenomatous polyposis.

BACKGROUND: Desmoid tumours (DTs) are the primary cause of death of patients with familial adenomatous polyposis (FAP) following restorative proctocolectomy. The aim of this study was to identify risk factors for DT in a French population. METHODS: Clinical data for 442 patients with FAP from 1983 to 2004 were reviewed retrospectively. RESULTS: A total of 124 DTs were documented in 50 patients (25 female). DT sites were mesenteric (73 tumours), abdominal wall (44) and extra-abdominal (seven). Female patients developed DT earlier than males. Although DTs appeared after colectomy in 34 patients, the type of surgery did not influence the risk of DT. An identified point mutation in the adenomatous polyposis coli (APC) gene after codon 1444 was a significant risk factor (hazard ratio 3.3 (95 per cent confidence interval 1.5 to 7.3)). Belonging to a family affected by DT did not increase the individual's risk in this population. CONCLUSION: No risk factor for life-threatening mesenteric DT could meaningfully modify the management of patients with FAP.

Sporadic desmoid tumor. An exceptional cause of cystic pancreatic lesion.

CONTEXT: Desmoid tumors are rare, benign soft tissue tumors, characterized by the proliferation of fibroblasts in an abundant collagen extra-cellular matrix. Intra-abdominal forms involve the mesentery and retroperitoneum and usually occur associated with familial adenomatous polyposis or Gardner's syndrome. Sporadic cases are more uncommon. Although desmoid tumors do not metastasize, their evolution can be life-threatening due to aggressive local invasion, such as mesentery involvement. CASE REPORT: We herein report a very rare location of sporadic desmoid tumors involving the pancreatic tail, presenting as a cystic lesion. A 51-year-old woman presented with recurrent abdominal pain and weight loss. The diagnosis of mucinous cystadenocarcinoma was suspected preoperatively and the patient underwent a splenopancreatectomy with en-bloc resection of the left colonic flexure, duodenojejunal junction and part of the posterior gastric wall. Pathological analysis revealed fibroblastic proliferation arising in musculoaponeurotic structures consistent with a desmoid tumor. The diagnosis was confirmed by immunocytochemical analysis and the assessment of the beta-catenin gene mutation. Perioperative examination reported gastric and small-bowel invasion. No treatment was given postoperatively to prevent desmoid tumor recurrence. After a 1-year follow-up, no recurrence was observed. CONCLUSION: Desmoid tumors are very rare in the pancreas and their diagnosis can be difficult, such as in our case where it presented as a cystic lesion. In contrast to intra-abdominal forms, sporadic pancreatic desmoid tumors are more frequent than those associated with familial adenomatous polyposis.

Updates on abdominal desmoid tumors.

Desmoid tumor is a monoclonal, fibroblastic proliferation arising in musculoaponeurotic structures. This connective tissue hyperplasia infiltrates locally, recurs frequently after resection but does not metastasize. Abdominal desmoid occurs sporadically, in association with some familial syndromes and often represents a clinical dilemma for surgeons. The enigmatic biology and anatomical location of abdominal desmoids make treatment recommendations difficult. This distinct pathological entity is reviewed with a specific focus on aetiology and management.

Current ideas in desmoid tumours.

Desmoid tumours are rare neoplasms of fibroblastic origin which arise with disproportionate frequency in patients with familial adenomatous polyposis (FAP). They are thought to develop in about 10-25% of FAP patients and may be the leading cause of death amongst those who have undergone colectomy. Risk factors include trauma, having a distal germline APC mutation, having a family history of desmoids, and probably oestrogens. In very high-risk individuals there may be a case for delay of colectomy or chemoprophylaxis at the time of surgery. Desmoids are now known to be true neoplasms but with normal telomere length and telomerase activity. FAP-associated tumours seem to carry biallelic APC mutations, one of which lies in the distal part of the gene. Such loss of wild-type APC seems to occur relatively late in tumour development. It is likely that beta-catenin plays an important role in tumourigenesis. FAP-associated desmoids tend to arise in the abdomen or abdominal wall. CT scanning gives the best information on tumour anatomy whilst T2-weighted MRI indicates likely behaviour. Treatment may simply consist of observation. Otherwise, usual first-line therapy is with sulindac with or without an anti-oestrogen. Cytotoxic chemotherapy is an option in unresectable tumours. Surgery is a reasonable first-line treatment in abdominal wall tumours but is risky for intra-abdominal tumours and may necessitate massive small bowel resection. Desmoids are the greatest remaining challenge in the management of FAP and further research into their aetiology needs to be combined with multicentre clinical trials of new treatments in order to improve management of the disease.

Proctocolectomía laparoscópica y reservorio íleo-anal en poliposis adenomatosa familiar: análisis de un caso / Laparoscopic proctocolectomy and ileoanal pouch in familiar adenomatous polyposis: analysis of a case

El objetivo de este trabajo es presentar el resultado de la primera proctocolectomía asistida por laparoscopía con reservorio íleo-anal efectuada en el Hospital Clínico de la Pontificia Universidad Católica de Chile, en una paciente de 21 años con poliposis adenomatosa familiar de novo diagnosticada por un síndrome disentérico amebiano intercurrente cuyo estudio con colonoscopía reveló una poliposis clásica de colon (>100 pólipos) con compromiso intenso del recto. Se decidió efectuar una proctocolectomía laparoscópica más un reservorio ileal en J e ileostomía en asa por una incisión de Pfannenstiel. La cirugía se realizó sin dificultades en un tiempo de 360 minutos. La paciente se realimento con líquidos al primer día y se dio de alta al quinto día postoperatorio sin complicaciones.

Evidence for genetic predisposition to desmoid tumours in familial adenomatous polyposis independent of the germline APC mutation.

BACKGROUND: Many patients with familial adenomatous polyposis (FAP) die from desmoid tumours which can arise spontaneously but often appear to be surgically induced by prophylactic colectomy. FAP results from germline adenomatous polyposis coli (APC) gene mutations and desmoids arise following biallelic APC mutation, with one change usually occurring distal to the second beta-catenin binding/degradation repeat of the gene (3' to codon 1399). We have suggested that because families with germline mutations in this region already have the requisite change, they are more likely to develop desmoids. However, there are families with 5' germline mutations where desmoids are common. PATIENTS AND METHODS: We examined desmoid risk dependent on germline APC mutation, sex, history of abdominal surgery, and family history in FAP patients from the St Mark's Hospital Polyposis Registry. RESULTS: Overall desmoid prevalence was 15%. Desmoids tended to cluster in susceptible individuals, irrespective of the germline APC mutation. Independent predictors of increased desmoid risk were: germline mutation distal to codon 1399; any family history of disease; and a strong family history of desmoids. A family history of multiple desmoids (>1) increased an individual's own risk of multiplicity. Females had twice the odds of developing desmoids compared with males. There was no significant interaction between any of the three explanatory variables. CONCLUSIONS: Our results indicate the influence of unknown genetic factors independent of APC in susceptibility to desmoid tumours in FAP. The data have implications in terms of clinical management of FAP patients and assessing the balance between chemoprevention and prophylactic colectomy.