Giant mesenteric fibromatosis associated with non-Hodgkin lymphoma. A case report and literature review.

BACKGROUND: Mesenteric fibromatosis is a benign locally-aggressive mesenchymal neoplasm that lacks the potential for metastasis. It is related to Gardner's Syndrome, previous trauma, abdominal surgery, and prolonged intake of oestrogen. Differentially diagnosing this from similar tumours is crucial in order for establishing the appropriate treatment and only immunohistochemical features can be used for a definitive diagnosis. Although medical therapies play a role in the treatment of mesenteric fibromatosis, surgical resection is the gold-standard procedure. METHODS: Our case study is a 40-year-old male with a concomitant diagnosis of non-Hodgkin lymphoma and mesenteric fibromatosis, not associated with any of the risk factors mentioned above. We performed CT and PET scans and observed a vascularised and well-defined mesenteric centre-abdominal hypermetabolic solid mass in contact with the gastric body, duodenum, body and tail of the pancreas, transverse colon, and spleen. An ultrasound-guided tru-cut biopsy revealed features suggestive of mesenteric fibromatosis. RESULTS: An elective laparotomy was carried out and a giant mass, arising from mesentery, was excised, including a partial gastrectomy and segmental resection of the transverse colon. Distal pancreatectomy, small bowel resection and successive splenectomy were performed due to a large hypertensive component. The postoperative period was uneventful. The histopathology of the surgical pieces was compatible with intra-abdominal desmoid fibromatosis. CONCLUSION: As far as we know from the literature, this is the largest mesenteric fibromatosis tumour ever to be excised. We also noticed that this is the first reported case of the concomitant presence of mesenteric fibromatosis and non-Hodgkin lymphoma that is not related to any of the described risk factors. Further research is needed to establish what type of association this presentation may indicate.

Transverse colonic volvulus due to mesenteric fibromatosis: a case report.

BACKGROUND: Colonic volvulus, a condition in which a colonic segment partially twists around its base, is the third leading cause of large bowel obstruction after colonic neoplasms and diverticular disease. However, volvulus of the transverse colon is the rarest type of large intestinal volvulus. Moreover, the occurrence of transverse colonic volvulus secondary to a benign tumor originating from outside the intestine has never been reported. We hereby report a case of transverse colonic volvulus caused by mesenteric fibromatosis. CASE PRESENTATION: A 53-year-old female with a history of rheumatoid arthritis and thyroid tumor presented with abdominal pain for 1 day. Abdominal computed tomography revealed intestinal torsion at the hepatic flexure. Twisted and obstructed mucosa of the transverse colon was observed during colonoscopy, but no tumor invasion of the mucosal surface was detected. A solid mass of a mesenteric origin with involvement of the transverse colon was observed during surgery. The mass was diagnosed surgically as transverse colonic volvulus induced by a mesenteric tumor. Hence, the patient underwent a right hemicolectomy. Histopathological results indicated mesenteric desmoid-type fibromatosis. The postoperative recovery was uneventful, and the patient was discharged 8 days after surgery. CONCLUSIONS: Although mesenteric fibromatosis is rare, this disease should be considered when managing transverse colonic volvulus resulting from nonmucosal tumors.

Multiple rapidly growing desmoid tumors that were difficult to distinguish from recurrence of rectal cancer.

BACKGROUND: Intra-abdominal desmoid tumors are usually slow growing and solitary, but multifocal desmoid tumors develop on rare occasions. Diagnosing desmoid tumors before histological examination of a surgical biopsy is often difficult. In particular, if a patient has a prior history of malignancy, it may be difficult to differentiate between these lesions and disease recurrence or metastasis. CASE PRESENTATION: We present a rare case of multiple rapidly growing intra-abdominal desmoid tumors after surgical trauma, without familial adenomatous polyposis. A 51-year-old male underwent abdominal perineal resection with lateral lymph node dissection after neoadjuvant chemotherapy for lower rectal cancer. Follow-up computed tomography (CT), performed 6 months after primary surgery, showed a 20-mm solitary mass in the pelvic mesentery. Another CT scan, performed 3 months later, revealed that the mass had grown to 35 mm in size and that two new masses had formed. Based on imaging studies and his medical history, it was difficult to distinguish the desmoid tumors from recurrence of rectal cancer. Curative resection was chosen for therapeutic diagnosis. The pathological diagnosis was multiple mesenteric desmoid tumors. CONCLUSIONS: Desmoid tumors should not be excluded as a differential diagnosis for intra-abdominal masses after intra-abdominal surgery, even in cases of rapidly growing multiple masses.

Partial trisomy of 11q23.3-q25 inherited from a maternal low-level mosaic unbalanced translocation.

Partial trisomy of 11q is characterized by pre/postnatal growth retardation, microcephaly, dysmorphic craniofacial features, cognitive disability, abnormal muscle tone, inguinal hernia, and possible congenital heart defects. Here, we describe a 17-year-old male with a 17.77 Mb-sized [arr 11q23.3-q25 (116,667,559 -134,434,130) ×3] partial trisomy resulting from the unbalanced translocation between chromosomes 11 and 22. The terminal translocation was detected using oligonucleotide array comparative genomic hybridization (CGH) with fluorescence in situ hybridization (FISH) confirmation. The partial trisomy was inherited from his mother who had the low-level (22.7%) mosaic unbalanced translocation and a normal phenotype. The patient showed most of the common features of partial trisomy 11q syndrome, with additional findings, including mesenteric fibromatosis.

Fibromatosis mimicking relapse of a neuroendocrine tumor at 68Ga-DOTATOC positron-emission tomography/computed tomography.

Mesenteric fibromatosis (desmoid tumor) is a locally aggressive fibroblastic lesion, characterized by a high recurrence rate that makes treatment challenging. Currently, there is no evidence-based treatment approach. We report the case of a female patient with a history of neuroendocrine tumor, who underwent 68Ga-DOTATOC positron-emission tomography/computed tomography (PET/CT), showing increased focal abdominal uptake suggestive of disease relapse. Histological examination revealed typical findings of fibromatosis. These findings indicate the expression of staining for somatostatin receptors (SSTRs) on fibromatosis cell surface and suggest to include fibromatosis among the potential causes of false-positive results at 68Ga PET/CT. Moreover, SSTRs expressed in desmoid tumors could be further investigated as a therapeutic target.

A Giant Solid-Cystic Mesenteric Fibromatosis with Unusual Synchronous Skin Fibromatosis.

Mesenteric fibromatosis (MF) is a rare, locally aggressive tumor without distant metastasis, which has a high recurrence rate. Based on its location, it is classified as intra-abdominal, from abdominal wall, and extra-abdominal. The incidence of cystic-solid, retroperitoneal tumors is very low in comparison to other MF forms. Intra-abdominal MFs are asymptomatic in early stages, but their symptoms appear late in the tumor course. There is no specific imaging finding since radiological diagnosis is mostly impossible. Thus, diagnosis is made histopathologically. Nowadays, there is no consensus about its treatment although surgical resection is widely used. In the present study, a very rare case of cystic-solid retroperitoneal MF associated with separate synchronous skin tumors is reported.

Sporadic intra-abdominal desmoid tumor with a very unusual onset: two case reports.

BACKGROUND: Intra-abdominal desmoid tumors are rare soft tissue tumors that arise mainly in the mesentery and pelvis. Their etiology may include genetic mutations, estrogen-associated changes after childbirth, and mechanical factors such as a history of abdominal surgery. However, there are cases of intra-abdominal desmoid tumors that develop in the absence of such causes. Since they are rare, diagnosis is often difficult based on clinical findings. We encountered two cases of patients with sporadic intra-abdominal desmoid tumors with a very unusual onset and contrasting features. CASE PRESENTATION: The first patient was a 51-year-old asian man who presented with sudden onset of abdominal pain. He was referred to our department because of a giant tumor detected on abdominal ultrasonography. Imaging revealed a 19-cm tumor with internal tumoral hemorrhage; however, no definitive diagnosis was made. Tumor resection was performed for diagnostic and therapeutic purposes. The second patient was a 41-year-old asian man, and right hydronephrosis was detected on abdominal ultrasonography during a periodic medical checkup. We diagnosed invasion of the primary mesenteric tumor into the right ureter using diagnostic imaging and performed ileocecal resection with partial right ureteral resection for a definitive diagnosis and therapeutic purposes. Although the tumors of both patients had developed from the ileal mesentery, the tumors were substantially different from each other based on their imaging findings, macroscopic morphology, and progression pattern. Meanwhile, they showed similar pathological characteristics. Both consisted of bundles of collagen fibrils of spindle-shaped fibroblasts with low cell atypia. Moreover, they were diagnosed as desmoid tumors using positive immunohistochemical staining for ß-catenin. CONCLUSIONS: Neither patient had susceptibility factors for desmoid tumors, and to our knowledge, there have been very few reports to date of intra-abdominal desmoid tumors that were diagnosed because of acute abdominal pain caused by tumoral hemorrhage or asymptomatic obstructive uropathy. Furthermore, it is clinically interesting that the two patients showed contrasting progression patterns and imaging findings. Intra-abdominal desmoid tumors are rare and may present with various symptoms and findings similar to those observed in our patients. Diagnosis therefore requires experience and knowledge that is not bound by preconceptions.

A giant mesenteric fibromatosis adherent to the appendix and colonic wall, case report.

BACKGROUND: Mesenteric fibromatosis, is a rare neoplasm arising usually from the bowel mesentery, with intermediate behavior and local invasion potential. They can be sporadic or related to multiple factors contributions. They usually presents as an asymptomatic growth of intraabdominal mass, and can reach a large diameter before symptoms appearance. Surgical excision is the definitive treatment when achievable. CASE PRESENTATION: In this case we present a case of 34 years old gentleman, presenting for painless abdominal distension, and found to have a giant mesenteric fibromatosis of 23 cm diameter and 4.5 kg arising from the appendix and colonic mesentery. Treated surgically, and was free of recurrence after 1 year follow up. CONCLUSION: We report this rare case to encourage physicians to keep this etiology in mind as part of the differential diagnosis of unspecific abdominal mass.

Ball in the Wall: Mesenteric Fibromatosis-a Rare Case Report.

INTRODUCTION: Mesenteric fibromatosis-desmoid tumor of mesentery is a rare benign soft tissue tumor of mesentery. On CT, it mimics gastrointestinal stromal tumor (GIST). CASE REPORT: A 44-year-old female with small intestinal mass, preoperatively diagnosed radiologically and pathologically as GIST. CONCLUSION: Mesenteric fibromatosis is a rare tumor often mistaken for GIST. Histopathology and immunohistochemistry is the key as management of both the tumors differs.

Peutz-Jeghers syndrome with mesenteric fibromatosis: A case report and review of literature.

BACKGROUND: Peutz-Jeghers syndrome (PJS) and mesenteric fibromatosis (MF) are rare diseases, and PJS accompanying MF has not been previously reported. Here, we report a case of a 36-year-old man with both PJS and MF, who underwent total colectomy and MF surgical excision without regular follow-up. Two years later, he sought treatment for recurrent acute abdominal pain. Emergency computed tomography showed multiple soft tissue masses in the abdominal and pelvic cavity, and adhesions in the small bowel and peritoneum. Partial intestinal resection and excision of the recurrent MF were performed to relieve the symptoms. CASE SUMMARY: A 36-year-old male patient underwent total colectomy for PJS with MF. No regular reexamination was performed after the operation. Two years later, due to intestinal obstruction caused by MF enveloping part of the small intestine and peritoneum, the patient came to our hospital for treatment. Extensive recurrence was observed in the abdomen and pelvic cavity. The MF had invaded the small intestine and could not be relieved intraoperatively. Finally, partial bowel resection, proximal stoma, and intravenous nutrition were performed to maintain life. CONCLUSION: Regular detection is the primary way to prevent deterioration from PJS. Although MF is a benign tumor, it has characteristics of invasive growth and ready recurrence. Therefore, close follow-up of both the history of MF and gastrointestinal surgery are advisable. Early detection and early treatment are the main means of improving patient prognosis.

Huge mesenteric fibromatosis presenting with intestinal perforation and acute diffuse peritonitis: a case report.

Mesenteric fibromatosis is a locally invasive myofibroblastic proliferation and rarely metastasize to other organs. Hollow organ perforation and acute diffuse peritonitis caused by mesenteric fibromatosis rarely occurred. Here we report a case of huge mesenteric fibromatosis who complained a paroxysmal epigastric pain, and CT scan showed a huge mass, pneumoperitoneum and ascites. An urgent laparotomy showed an intro-abdominal mass and perforation locating at the jejunum. Postoperative histology confirmed it to be mesenteric fibromatosis. With one-year follow-up, the patient had no recurrence. We wish to share our treating experience of this interesting case because it did not belong to a typical type but presenting with acute diffuse peritonitis, and total resection and R0 margin is a key to treat acute case. This atypical one has not been reported in the literature till now.

Successful treatment of giant mesenteric fibromatosis with surgery and tamoxifen: case report.

A 65-year-old man underwent excision of a giant mesenteric fibromatosis (MF) via combined splenectomy and partial transverse colectomy. Pathological examination confirmed the presence of MF, whereas genetic testing indicated that the tumor was sensitive to tamoxifen. Over a 1-year follow-up, no symptoms of abdominal discomfort or recurrence was noted.

Mesenteric fibromatosis misdiagnosed with lymph node metastasis after successful laparoscopic right hemicolectomy: a report of two cases with review of literature.

Fibromatosis is a rare type of tumor derived from the mesenchymal tissue. This is a benign tumor with infiltrating growth but may invade locally and recur following excision. As one type of fibromatosis, mesenteric fibromatosis (MF) accounts for a mere 8% of cases. Although studies have revealed that the etiology of MF is related to trauma, surgery, hormones, and heredity, the specific etiology of fibromatosis remains unclear. With such low incidence, MF has been rarely reported and tends to be misdiagnosed due to insufficient recognition. In this paper, we describe the cases of two patients with MF who were misdiagnosed with lymph node metastasis and who had previously undergone successful laparoscopic right hemicolectomy. We provide this information in order to broaden the clinical understanding of MF.

Diagnostic and therapeutic dilemmas in intra-abdominal desmoid tumors: A case report and literature review.

INTRODUCTION: Intra-abdominal desmoid tumors (DTs) are a rare and anatomically diverse group of locally-aggressive, benign neoplasms. They are often difficult to diagnose, even in patients who possess risk factors for the disease. Even after a diagnosis has been reached, the optimal therapy is often not well-defined. PRESENTATION OF CASE: The case discussed of a 33-year old male with a giant intra-abdominal desmoid is an example of both the diagnostic and therapeutic dilemmas that arise when confronted with a patient with a DT. Initial confusion over diagnosis led to ineffective therapy, but once the correct diagnosis was made, the patient went on to definitive surgical resection. DISCUSSION: The differential diagnosis of DTs is broad, and the diagnosis is often delayed due to nonspecific presentations. Immunohistochemistry is crucial in the accurate histological diagnosis, which guides treatment. Chemotherapy and radiation have a role in the management of both primary and recurrent lesions, but surgical resection remains the cornerstone of treatment. CONCLUSION: DTs present a clinical challenge in their diagnosis and management, and despite providing standard medical and surgical treatment, recurrence rates are high and continued surveillance is crucial.

Mesenteric Fibromatosis Mimicking Tumor Recurrence Following Radical Cystectomy and Bladder Replacement.

We report an unusual case of benign tumor mimicking tumor recurrence following radical cystectomy and bladder replacement for high grade urothelial carcinoma.

Mesenteric fibromatosis (desmoid tumour) - a rare case report.

Mesenteric Fibromatosis (MF) is a proliferative fibroblastic lesion of small intestinal mesentery. It constitutes 8% of all desmoid tumours, which represent 0.03% of all neoplasm. It is histologically benign but may invade locally and recur after excision. It occurs sporadically or in association with Familial adenomatous polyposis (FAP) mutation as a component of Gardner's syndrome. The presenting features of MF are asymptomatic abdominal mass, abdominal discomfort or pain, bowel or ureteral obstruction, intestinal perforation, fistula, functional impairment of ileoanal anastomosis following colectomy in FAP cases. A 29-year-old male presented with a swelling on the right side of the umbilicus for six months and dull aching pain for two months. Fine needle aspiration cytology, ultrasonography, contrast enhanced computerized tomography findings were inconclusive. After Exploratory laparotomy, a mass approx 6x5x4 cm in ileal mesentery was identified and excised along with 20cm of ileum. End to end anastomosis was done and specimen was sent for histopathology which confirmed the diagnosis of MF. Considering the rarity of this tumour and difficulties in diagnostic and therapeutic ambit, we believe it is justified to describe this case which came to our observation.

Sporadic giant mesenteric fibromatosis.

Mesenteric fibromatosis is an uncommon tumour which is locally aggressive without any metastatic potential and can occur as a sporadic event or in association with familial adenomatous polyposis syndrome. Giant mesenteric fibromatosis is very rare and is a diagnostic and therapeutic challenge. This is a case report of a rare presentation of deep fibromatosis as a sporadic giant intrabdominal mesenteric tumour in a 29 year old male managed by surgical excision and definitive diagnosis made on the basis of immunohistochemical findings.

Giant mesenteric fibromatosis: Report of a case and review of the literature.

Mesenteric fibromatosis poses a diagnostic and therapeutic challenge. This paper presents a 35-year-old female complaining of vague abdominal pain of 2 mo duration. Her computed tomography scan and magnetic resonance imaging revealed a pelvi-abdominal heterogenous mass with significant displacement of the small bowel and urinary bladder. She underwent surgical excision of the mass with resection and anastomosis of the involved loop of the small intestine. Histological examination confirmed mesenteric fibromatosis without infiltration of the bowel. The patient remained well during the 6 mo follow-up.

Mesenteric fibromatosis of the ileocolic area: a case report.

Fibromatosis is a kind of spindle cell tumor which is characterized by the remarkable proliferation and invasive growth of fibrous tissue. It often arises from the abdominal wall or the extremities and rarely from the mesentery and abdominal organs. The patient was 35 year old female whose major complaints were an abdominal mass and pain. She underwent a right hemicolectomy after the tumor was confirmed by abdominal ultrasonography and computerized tomography. Hereby the pathophysiology, diagnosis and the treatment of the disease is discussed.