Fibromatoses are a heterogeneous group of benign proliferating fibroblasts and myofibroblasts which have a high predilection for recurrence and local invasion, especially deep fibromatoses or desmoid fibromatosis. 18F-FDG PET/CT, the workhorse of oncological imaging in nuclear medicine, can be employed to figure out the nature and aggressiveness of the lesions and various sites of involvement and to monitor treatment response to systemic therapies like tyrosine kinase inhibitors in case of deep or desmoid fibromatoses which is shown in the current research work.

Mesenteric desmoid-type fibromatosis (DTF) is a rare benign yet aggressive neoplasm that has an unpredictable biological behavior ranging from spontaneous regression to extensive local infiltration and has a high tendency for recurrence. The presenting symptoms are usually nonspecific and mostly related to the large size of the tumor compressing adjacent organs. Imaging studies can be suggestive of the diagnosis, but confirmation is based on histopathological and immunohistochemical examination. The lack of knowledge on the etiology and pathogenetic behavior of this tumor leads to therapeutic and prognostic challenges. Future genetic studies may help in advancing our understanding of this neoplasm and in formulating the proper management and follow-up plan. Here we present a case of a 14-year-old female who presented to the emergency room complaining of diffuse abdominal pain and distention. A computed tomography (CT) scan showed a large mass occupying most of the abdominal cavity and compressing adjacent organs. Exploratory laparotomy with resection and anastomosis was performed, and the histopathological and immunohistochemical examination of the resected mass was consistent with mesenteric DTF.

Desmoid-type fibromatosis is a rare benign mesenchymal neoplasm. Only 8% of desmoid-type fibromatosis develops in the abdominal cavity. The mesentery is seldom affected and gastrointestinal stromal tumors need to be considered in the differential diagnosis, particularly when imaging examination shows a tumor containing gases in the abdominal cavity. Only a few cases of gas-containing mesenteric desmoid-type fibromatosis have been reported in the literature.

A 69-year-old male patient presented with hematochezia and intermittent upper abdominal pain.

Contrast-enhanced computed tomography revealed a 3.9 × 3.6 cm gas-containing mass infiltrating the third portion of the duodenum. The tumor was heterogeneous, with cysts and air bubbles. It showed heterogeneous weak-to-mild enhancement in the solid part. Postoperative pathological examination confirmed a final diagnosis of mesenteric desmoid-type fibromatosis.

The patient underwent surgical resection of intra-abdominal lesion.

No evidence of local recurrence was noted during the 6 months of follow-up.

Accurate preoperative diagnosis is difficult for an intra-abdominal gas-containing mass on computed tomography scan. The appearance of spiculated infiltrative margin suggests the diagnosis of desmoid-type fibromatosis. Further investigation of imaging evidence and treatment methods is necessary.

A 68-year-old man, without a family history of cancer, was treated for primary cutaneous melanoma of the scalp. Two years later, a right lateral cervical lymph recurrence was observed and he was treated with lymphadenectomy and adjuvant nivolumab for 1 year. Four years from the initial melanoma diagnosis, a computer tomography scan showed a solid nodular lesion of 26 × 40 × 75 mm inside the previously known inguinoscrotal hernia. A new recurrence of melanoma was the most probable diagnosis and a right inguinal hernioplasty was performed. Notably, the histopathological examination revealed a mesenteric fibromatosis with the typical immunohistochemical pattern (strong nuclear staining of β-catenin). Interestingly, this represents the first case of a patient with a mesenteric desmoid tumour presenting as an inguinal hernia masking a cutaneous melanoma recurrence.

A 49-year-old woman presented to surgery outpatient clinic with the awareness of a right lower abdomen asymptomatic lump for one week. Contrast-enhanced CT of the abdomen demonstrated a 10×11×15 cm heterogeneous lesion anterior to the ileocecal junction with the possibility of leiomyosarcoma. The patient was prepared for laparotomy and intraoperative there was a large tumour arising from the medial wall of cecum. Right haemicolectomy was performed, and histopathology came as a desmoid tumour of the cecum. The patient had an uneventful postoperative course and is well on 1-year follow-up.

Desmoid-type fibromatosis (DF) is a locally aggressive neoplasm characterized by mutations in the CTNNB1 gene, which encodes the β-catenin protein. We reviewed 85 cases of DF and performed Sanger sequencing for detecting mutations in CTNNB1 and immunostaining for detecting β-catenin localization. We included 70 DF samples, of which 56 cases demonstrated nuclear β-catenin localization and 43 cases harboured CTNNB1 mutations. CTNNB1-mutant DF samples consistently displayed nuclear β-catenin expression and were derived from larger-sized tumours compared to samples with wild-type CTNNB1. When we further classified DF cases into 2 subgroups based on the type of specimen, excised specimens with nuclear β-catenin expression frequently displayed CTNNB1 mutation and no statistical correlation between nuclear β-catenin expression and CTNNB1 mutation was observed in biopsies. When we classified CTNNB1 mutation cases into 2 subgroups (DF with T41A or T41I, and DF with S45F or S45P), T41A or T41I mutations were observed more frequently in males than in females. Additionally, DF tumours harbouring S45F or S45P mutations were located more frequently in the abdominal wall than tumours with T41A or T41I mutations. In conclusion, CTNNB1 mutation correlates with nuclear β-catenin expression in larger or excised DF tumours, and DF harbouring CTNNB1 mutations manifest variable clinical presentations.

Aggressive fibromatosis or desmoid tumor is a rare disease resulting from fibroblasts which do not metastasize. However, desmoid tumors belong to low-grade malignant tumors since they have high potential to infiltrate surrounding tissues, causing high local recurrence rates and may affect surrounding organs, threatening life quality and expectancy. Although surgery, watch and wait, radiotherapy, chemotherapy, high intensity focused ultrasound, ablation techniques or several agents have all been frequently investigated for the treatment of this type of disease, none are deemed as standard therapy for high recurrence rates that have been supported by any data. The present review retrieved literature on treatment options for desmoids to summarize the latest treatment modalities and refine their efficacy, as well as their side effects, in order to provide a more comprehensive treatment reference for clinicians.

Desmoid tumors, also known as aggressive fibromatosis, are extremely rare, accounting for less than 3% of soft-tissue sarcomas and less than 0,03% of all neoplasms. The diagnosis is usually delayed because of the lack of specific symptoms, and can sometimes lead to serious and, even fatal complications.

We report the case of a 27-year-old male patient presenting with the clinical picture of acute appendicitis. During the operation, we found a tumor in the jejunum with a necrotic zone and perforation on its surface, causing hemorrhagic effusion into the abdominal cavity and subsequent peritonitis. The tumor was removed with negative margins via resection of the small bowel. The final histological result showed aggressive fibromatosis.

Aggressive fibromatosis remains a serious problem with the possibility of locally aggressive behavior with high rates of recurrence. Sometimes, its clinical and macroscopic recognition can be immensely tricky. As shown by our patient, on rare occasions, desmoid tumors can lead to acute surgical abdomen requiring an emergency operation.

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.

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.

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.

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.

Intra-abdominal aggressive fibromatosis is a locally aggressive tumor mostly originating from the mesentery or retroperitoneal space, infiltrating adjacent tissues, and very rarely metastasizing to distant organs. There are only two case reports in the English language literature where intra-abdominal aggressive fibromatosis originated from the intestinal wall. In this study, we aimed to report a case of aggressive fibromatosis originating from the muscularis propria layer of the duodenum and invading pancreas. Another interesting aspect of this case is that a primary paraduodenal hydatid cyst was incidentally detected in the surgical specimen. A 46-year-old female patient presented to our clinic with postprandial nausea and vomiting. A contrast-enhanced abdominal computerized tomography revealed a mass lesion with a size of 100 mm × 80 mm which originated from the distal pancreas and compressed the gastric pilor externally. Upon exploration the distal part of duodenum, proximal jejunum, and pancreatic mass were noted to form a conglomerated structure. Therefore, the fourth part of the duodenum, a 25 cm part of the proximal jejunum, distal pancreas, and the spleen were excised en-bloc. The pathology report of the specimen indicated fibromatosis with a diameter of 55 mm that originated from the muscularis propria of the duodenum and extended into the pancreatic parenchyma. There was also an incidentally detected 10 mm paraduodenal hydatid cyst. No tumor recurrence was detected at a follow-up period of 24 mo. In conclusion, the most ideal treatment of desmoid-type fibromatosis is surgical resection of the mass lesion with clean surgical borders. Although rare, this tumor may originate from the intestinal wall. Histopathological verification is of great significance for a proper diagnosis.

Desmoid-type fibromatosis represents a rare, benign, soft tissue tumour that is locally invasive with high recurrence potential.

We encountered a case of retroperitoneal desmoid-type fibromatosis in a 45-year-old woman who presented with chief complaints of stomach ache and vomiting. She underwent total abdominal hysterectomy and left salpingo-oophorectomy due to uterine myoma and a paroophoritic cyst at 42 years of age. Abdominal computed tomography showed a 5-cm left retroperitoneal tumour and severe hydronephrosis of the left kidney. Multiple imaging studies failed to provide a definitive diagnosis. Therefore, we performed tumour resection, right salpingo-oophorectomy, ureterectomy, and ureterocystostomy. The tumour surrounded the left ureter and adhered to the left internal/external iliac artery, rectum, bladder, and the edge of the vagina. Histopathologic examination yielded a diagnosis of retroperitoneal desmoid-type fibromatosis. One month after the operation, transvaginal sonography showed a 2-cm mass in the pelvis. We suspected tumour recurrence and commenced pharmacotherapy with tranilast (300mg/day, three times per day). Four months after the operation, the mass disappeared.

There are minimal reports of postoperative intra-abdominal desmoid-type fibromatosis and preoperative diagnosis is difficult. To the best of our knowledge, there are no reported cases of desmoid-type fibromatosis that involved the ureter with severe hydronephrosis following a gynaecologic operation.

We experienced a case of retroperitoneal desmoid-type fibromatosis that involved a unilateral ureter after gynaecologic surgery.

Fibromatosis or desmoid tumour is a benign fibroblastic proliferation with aggressive infiltrative growth. High incidence of recurrence is noted after incomplete resection of the involved margins of the lesion. Pelvic fibromatosis is a rare condition and usually affects females. Patients frequently complain of pelvic pain, which can mimic gynaecological abnormalities. A case of pelvic fibromatosis involving pelvic floor muscles with unchanged appearance during 5 years of follow-up is presented.