Coexistence of duodenum derived aggressive fibromatosis and paraduodenal hydatid cyst: A case report and review of literature

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, proximaljejunum, 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 enbloc. 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.

1 Case Clinical Report of Cranium Aggressive Fibromatosis and Literature Review

Objective: The purpose of this study is to explore the clinical characteristics and therapeutic methods of aggressive fibromatosis (AF) in skull. Methods: The clinical data and operative therapy of one case of aggressive fibromatosis in skull in our department were examined and reviewed. The associated literatures were reviewed and discussed. Results: It was indicated that the main manifestation of AF in skull was headache and skull tumor. There was prominent osteolytic destruction found in X-ray plain film for skull AF. And CT scanning showed that skull sclerotin was disorganized and inhomogeneous, with widen diploe. The skull fibromatosis constituted by fibroblasts and myofibroblas, which were mainly spindle-shaped without heteromorphism. Immunohistochemistry showed positive expression of β-catenin and Vim in these cells. The enlarged incision was adopted for the strategy of operation in this patient of skull AF. After follow-up, there was no recurrence of AF discovered. Conclusions: Skull AF is very rare in neurosurgical clinic. The clinical manifestation and iconography of AF were lack of specificity. Therefore, skull AF is hard to diagnose preoperatively. The effective diagnose is mainly dependant on histopathologic examination. As for treatment, operation is the most optimal method so far, which has a good therapeutic effect.

A case report on aggressive fibromatosis of the arm and shoulder

Background:Aggressive fibromatosis is a locally aggressive tumor that originates from the deep musculo-aponeurotic structures.In this report,we describe the case of an adolescent male with painless,progressively growing swelling in his right arm necessitated surgical excision and postoperative pathologic examination revealed aggressive fibromatosis.Case presentation:A 15-year-old boy presented with a painless,progressively growing swelling of his right arm.The swelling was firm,non-tender and with restricted mobility.MRI imaging revealed a well-defined,heterogenous,lobulated swelling extending beneath arm and shoulder muscles.Wide local excision of the swelling was done under general anesthesia and pathologic examination revealed aggressive fibromatosis of the arm.Adjuvant radiotherapy was provided to the patient to reduce the risk of local recurrence of the tumor.Conclusion:Aggressive fibromatosis of the upper limb presents as a painless,progressively growing swelling.Assessment with MRI is imperative for making a preliminary diagnosis.Wide local excision with safety margin with adjuvant radiotherapy is the main line of treatment to minimize the incidence of recurrence and preserve the limb.

Aggressive fibromatosis of the sigmoid colon:A case report

BACKGROUND Aggressive fibromatosis(AF),also known as desmoid tumor or desmoid-type fibromatosis,is a rare soft tissue neoplasm that can occur in almost any part of the body.Although it is a benign disease,AF is aggressive and infiltrative and has a high recurrence rate after surgery.Common sites for intra-abdominal AF are the small bowel mesentery,retroperitoneum,and pelvis.AF in the colon is extremely rare.CASE SUMMARY Here,we report the first case of sigmoid colon AF,which was accidentally discovered in a 27-year-old woman during laparoscopic myomectomy.Computed tomography confirmed a slightly enhanced mass in the sigmoid colon.Subsequent colonoscopy did not reveal a mass in the colonic lumen,but a suspected external compress was found in the sigmoid colon.Surgical disease involving a gastrointestinal stromal tumor was suspected.The patient underwent laparoscopic exploration,and sigmoidectomy with a negative margin was performed to excise the mass.Postoperative immunohistochemistry revealed that the mass was an AF.The patient recovered well and was recurrence-free at the 30-month follow-up without adjuvant therapy.CONCLUSION AF should be considered in the differential diagnosis of subepithelial colon masses.Radical resection alone can achieve good outcomes.

Comparison of clinical features of patients with or without severe gastrointestinal complications in aggressive gastrointestinal lymphomas

BACKGROUND Aggressive primary gastrointestinal non-Hodgkin lymphoma(PGINHL)is an uncommon and heterogeneous group of lymphoid malignancies,that differs from indolent lymphoma and has a high incidence of severe gastrointestinal complications(GICs).AIM To investigate and compare the clinicopathological characteristics,treatments and outcomes in the GICs and No-GICs group with aggressive PGINHL.METHODS This retrospective analysis was performed on aggressive PGINHL patients between January 2013 and December 2021 at our hospital.The independent influence factors of GICs were obtained by univariate and multivariate Logistic regression analysis,the selected variables significantly related to GICs were selected as the final predictors to construct nomogram.Kaplan-Meier curves further analyzed the survival of patients in GICs and No-GICs groups.Survival analysis of GICs group was performed using Cox regression.RESULTS We focused on 124 aggressive PGINHL cases,which had a relatively high incidence 48.4%(60/124 cases)of GICs,the most common histological type in GICs group was diffuse large B-cell lymphoma(DLBCL)(n=49,81.7%).In the GICs group,small intestine was the most common anatomic site of lesion(43.3%),followed by large intestine(31.7%),and then stomach and esophagus(25.0%).Multivariate Logistic regression analysis showed that the independent risk factors for GICs were the small intestine[odd ratio(OR)=3.33;95%confidence interval(CI):1.47-9.41;P=0.009),aggressive B-cell(OR=0.09;95%CI:0.01-0.83;P=0.033),maximum tumor diameter(OR=1.25;95%CI:1.07-1.47;P=0.005),invaded deep serous layer(OR=3.38;95%CI:1.24-9.19;P=0.017).We developed a nomogram to predict risk of GICs in aggressive PGINHL patients based on independent risk factors.The value of area under curve calculated by receiver operating characteristic curve was 0.815,and calibration curve and decision curve analysis further indicated that the prediction effect was superior.The majority of patients with GICs were given combination therapy(chemotherapy combined with surgery or radiation).Event-free survival and overall survival in GICs group were no worse than those in the No-GICs group.CONCLUSION The complication rate of GICs in patients with aggressive PGINHL was relatively high,particularly in PGI-DLBCL.The independent risk factors for GICs were the small intestine,PGI-TNKL,bulky tumor,and depth of invasion.A combination treatment,involving surgery,improved survival in the GICs group.

Desmoid type fibromatosis: A case report with an unusual etiology

Desmoid type fibromatosis(DTF) is a rare, locally invasive, non-metastasizing soft tissue tumor. We report an interesting case of DTF involving the pancreatic head of a 54-year-old woman. She presented with intermittent dysphagia and significant weight loss within a 3-mo period. Laboratory findings showed mild elevation of transaminases, significant elevation of alkaline phosphatase and direct hyperbilirubinemia, indicating obstructive jaundice. Computerized tomography of the abdomen revealed a mass in the head of the pancreas, dilated common bile duct, and dilated pancreatic duct. Endoscopic retrograde cholangiopancreatography and endoscopic ultrasound showed a large hypoechoic massin the head of the pancreas causing extrahepatic biliary obstruction and pancreatic ductal dilation. The patient underwent a successful partial pancreatico-duodenectomy and cholecystectomy. She received no additional therapy after surgery, and liver function tests were normalized within nine days after surgery. Currently, surgical resection is the recommended first line treatment. The patient will be followed for any recurrence.

The Relationship between Aggressiveness and Gender in Patients with Temporal Lobe Epilepsy

Purpose: To examine the impact of gender and social gender on the level and typology of interictal aggressiveness in patients with temporal lobe epilepsy. Material and Methods: 40 adult patients with Temporal Lobe Epilepsy (TLE) and 86 healthy individuals were included. The qualitative and quantitative aggressiveness assess- ment was made with Buss-Durkee Hostility Inventory. The gender role behavior was measured with Bem Sex Role Inventory. Results: Patients with TLE didn’t differ from healthy subjects on the total scores of motor and attitudinal hostility components, but scored higher on subscales “resentment” and “guilt”. The comparative gender analysis showed there were no phenomenological differences in people with TLE. Assault dominated in healthy male subjects, resentment—in healthy female subjects. The prevalence of feminine social gender type was significantly higher in people with TLE in comparison to healthy people (55% vs. 26%, p 0.01). In patients with TLE, the number of masculine traits positively correlated with indirect hostility subscale and attitudinal hostility component scores. In healthy subjects, the masculine traits positively correlated with assault.

Recurrent aggressive mesenteric desmoid tumor successfully treated with sorafenib: A case report and literature review

BACKGROUND Desmoid tumors(DT) are locally advanced but histologically benign monoclonal neoplasms that can occur from any musculoaponeurotic structure. The aim of this report is to analyze a rare clinical case of an aggressive intra-abdominal DT successfully treated with sorafenib.CASE SUMMARY A 36-year-old man presented with increasing colicky abdominal pain and a selfpalpable mass in his left abdomen. Fourteen years earlier he was diagnosed with a large intra-abdominal tumor, which adhered to the left colonic flexure, part of the major gastric curvature and the spleen. Subsequent exploratory laparotomy revealed a voluminous mass in the epigastrium, arising from the posterior surface of the stomach and invading the superior mesenteric vessels, transverse mesocolon and the small bowel mesentery. As the tumor was unresectable, a jejunojejunal bypass was performed. Traditional therapeutic interventions proved insufficient, and the patient was started on sorafenib with a subsequent fulldisease response.CONCLUSIONDT's pathogenesis has been associated with mutations in the adenomatous polyposis coli(APC) gene or beta-catenin gene CTNNB1, sex steroids or previous surgical trauma. Local treatment modalities, such as surgery or radiotherapy, are implemented in aggressively progressing or symptomatic patients. Sorafenib is a hopeful therapeutic option against DTs, while several pharmacological agents have been successfully used.

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.

Intermittent ice-cooling to prevent skin heat injury caused by highintensity focused ultrasound therapy targeting desmoid-type fibromatosis:A case report

Introduction:Desmoid-type fibromatosis(DF)is a fibrous tumor characterized by low-grade malignant and easy invasive growth and high recurrence.High-intensity focused ultrasound(HIFU)therapy has been identified as a novel non-invasive approach for DF treatment;however,the ultrasonic energy generated by HIFU can cause skin heat injury.Case:A 31-year-old female patient with signs and symptoms of DF received treatment in our institution.The patient had undergone HIFU treatment six times from April 27,2018,to August 21,2019.After HIFU therapy for the third time,she had a third-degree skin burn showing as orange peel-like change and spent three months to promote the recovery of the skin lesions.An intermittent ice-cooling strategy was used to avoid skin damage during the fourth HIFU treatment.This patient did not have any apparent skin injury during the last three HIFU therapy and acquired satisfactory anti-tumor therapeutic effect.Conclusions:There are differences in the thermal selectivity of tumor tissues,which leads to different critical thermal injury temperature values that the tissue can tolerate.Ice-cooling can lower skin tissue temperature and reduce the thermal damage caused by HIFU treatment.

Cholangiocarcinoma accompanied by desmoid-type fibromatosis

BACKGROUND:Cholangiocarcinoma complicated by intraabdominal desmoid-type fibromatosis(DTF)is uncommon. There are no reports on patients with this type of fibromatosis, in which the pre-operative treatment(including diagnosis), surgical approach,post-operative pathologic reports,and prognosis are discussed. METHOD:The clinicopathological features of a 49-year-old man were retrospectively analyzed. RESULTS:Cholangiocarcinoma located in the inferior segment of the bile duct was considered pre-operatively on the basis of clinical findings.At the time of pancreaticoduodenectomy,the mesojejunum was stiff without nodules or a mass at a distance of approximately 80 cm from the ligament of Treitz.Complete excision of the entire lesion of the intestinal mesenteric contracture and its subsidiary was performed.Post-operative pathologic findings confirmed an adenocarcinoma located at the extremity of the common bile duct and infiltrating the full thickness of the common bile duct as well as the deep muscular layer of the duodenum.The contracted jejunal mesentery was shown to have DTF.The patient was alive with no evidence of recurrence after a follow-up of 6 months. CONCLUSIONS:The patient had a rare hereditary disease with intra-abdominal DTF,which manifests the characteristics of an aggressive growth pattern and a high rate of local recurrence; conservative therapy is recommended.Complete excision of the fibromatous lesion during pancreaticoduodenectomy may maximally decrease the risk of local recurrence.

Effects of thymidine phosphorylase on tumor aggressiveness and 5-fluorouracil sensitivity in cholangiocarcinoma

AIM: To evaluate the role of thymidine phosphorylase (TP) in cholangiocarcinoma using small interfering RNA (siRNA). METHODS: A human cholangiocarcinoma-derived cell line KKU-M139, which has a naturally high level of endogenous TP, had TP expression transiently knocked down using siRNA. Cell growth, migration, in vitro angiogenesis, apoptosis, and cytotoxicity were assayed in TP knockdown and wild-type cell lines. RESULTS: TP mRNA and protein expression were decreased by 87.1% ± 0.49% and 72.5% ± 3.2%, respectively, compared with control cells. Inhibition of TP significantly decreased migration of KKU-M139, and suppressed migration and tube formation of human umbilical vein endothelial cells. siRNA also reduced the ability of TP to resist hypoxia-induced apoptosis, while suppression of TP reduced the sensitivity of KKU-M139 to 5-fluorouracil. CONCLUSION: Inhibition of TP may be beneficial in decreasing angiogenesis-dependent growth and migration of cholangiocarcinoma but may diminish the response to 5-fluorouracil chemotherapy.

Gingival fibromatosis with multiple unusual findings:report of a rare case

Gingival fibromatosis is a rare disease, especially its syndromic form. Here, we review the literatures on gingival fibromatosis and briefly summarize some characters on clinical, etiological, genetic and histopathological aspects. We also present a rare case of gingival fibromatosis with multiple unusual findings in a 21-year-old man. And we differentiate it from some well-known syndromes including gingival fibromatosis. Maybe it implies a new syndrome within the spectrum of those including gingival fibromatosis.

ABC transporters and the hallmarks of cancer:roles in cancer aggressiveness beyond multidrug resistance

The ATP-binding cassette transporters(ABC transporters)have been intensely studied over the past 50 years for their involvement in the multidrug resistance(MDR)phenotype,especially in cancer.They are frequently overexpressed in both naive and post-treatment tumors,and hinder effective chemotherapy by reducing drug accumulation in cancer cells.In the last decade however,several studies have established that ABC transporters have additional,fundamental roles in tumor biology;there is strong evidence that these proteins are involved in transporting tumor-enhancing molecules and/or in protein-protein interactions that impact cancer aggressiveness,progression,and patient prognosis.This review highlights these studies in relation to some well-described cancer hallmarks,in an effort to re-emphasize the need for further investigation into the physiological functions of ABC transporters that are critical for tum or development.Unraveling these new roles offers an opportunity to define new strategies and targets for therapy,which would include endogenous substrates or signaling pathways that regulate these proteins.

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.

Localized intra-abdominal fibromatosis of the small bowel mimicking a gastrointestinal stromal tumor: A case report

Intra-abdominal fibromatosis （IAF） is a benign mesenchymal lesion that can occur throughout the gastrointestinal tract. Although rare, it is the most common primary tumor of the mesentery and can develop at any age. We describe a rare case of primary IAF involving the mesentery and small bowel which clinically, macroscopically and histologically mimicked malignant gastrointestinal stromal tumor （GIST）. This report highlights the fact that benign IAF can be misdiagnosed as a malignant GIST localized in the mesentery or arising from the intestinal wall. Their diagnostic discrimination is essential because of their very different biological behaviors and the fact that the introduction of effective therapies involving tyrosine kinase inhibitor ST1571 （imatinib mesylate） has greatly changed the clinical approach to intra-abdominal stromal spindle cell tumors.

Colorectal cancer of the young displays distinct features of aggressive tumor biology:A single-center cohort study

BACKGROUND In recent years,a decrease in incidence and mortality of colorectal cancer(CRC)has been observed in developed nations,presumably through public disease awareness and increased screening efforts.However,a rising incidence of CRC in young patients below the age of 50 years has been reported in several studies.AIM To study tumor biology in CRC patients below 50 years of age.METHODS All patients with CRC were prospectively enrolled in our single-center oncologic database from January 2013 to December 2018 and were grouped and analyzed according to age(≥50 and<50 years).Clinical as well as histopathological features were analyzed and compared.The study was approved by the local Ethics Committee.Fisher’s exact test or t-test was used to test for differences between the groups,as appropriate.All statistical analysis was performed with IBM SPSS software Version 25(SPSS Inc,Armonk,NY,United States)and with RStudio using R Version 3.4.1(RStudio,Boston,MA,United States).RESULTS Seventeen percent of the 411 patients were younger than 50 years.Young patients were more often diagnosed with locally advanced T4-tumors and lymph node metastases(36.6%and 62%vs 17.7%and 42%;P<0.01).In addition,a higher frequency of poorly differentiated(G3)tumors(40%vs 22.4%P<0.05)was observed.More than every second patient below 40 years of age(51.8%)had distant metastases at diagnosis with a significant higher rate ring of signet cell differentiation compared to patients≥50 years(14.8%,P<0.05).Mutational status(KRAS,NRAS,BRAF,MSI)as well as selected behavioral risk factors showed no significant differences.CONCLUSION Distinct histopathologic features of increased biologic aggressiveness are found in patients with CRC of young-onset.Those patients present more frequently with more advanced tumor stages compared to older patients.Features of aggressive tumor biology underscore the need for earlier uptake of routine screening measures.

Breast Fibromatosis after Hydrophilic Polyacrylamide Gel Injection for Breast Augmentation: a Case Report and Review of the Literature

BREAST fibromatosis is a rare kind of lesion. The average incidence is about 2-4 per million every year} So far there have been about 100 cases reported altogether.2 In this report, we describe a case of breast fibromatosis developed after hydrophilic polyacrylamide gel （HPG） injection for breast augmentation. By reviewing the literature, the possible pathogenesis of this case and the proper treatment strategy are investigated.

Misdiagnosis of ligamentoid fibromatosis of the small mesenteric:A case report

BACKGROUND Ligamentoid fibromatosis is a rare borderline tumor that occurs in the muscles,fascia,and aponeurosis.It is a kind of soft tissue tumor of fibrous origin,also known as invasive fibromatosis,desmoid fibroma,neurofibromatosis,etc.The tumor is between benign and malignant tumors and rarely has distant metastasis.Its characteristics are mainly local invasion,destruction and growth and easy recurrence.The World Health Organization defines it as a fibroblast cloning value-added lesion originating from deep soft tissue,which causes local invasion and growth leading to tissue reconstruction,extrusion and destruction of important structures and organs.The incidence rate accounts for 0.03%of all tumors and less than 3%of all soft tissue tumors.Definite diagnosis mainly depends on postoperative pathology.Surgical resection is still the main way to treat the disease,and a variety of nonsurgical treatment methods are auxiliary.Combined treatment can effectively reduce the risk of postoperative recurrence.CASE SUMMARY The patient is a 57-year-old female.One week ago,she accidentally found a mass in the left upper abdomen while lying flat.There was no abdominal pain and abdominal distention,no fever,no black stool and blood in the stool and no nausea and vomiting.She had a 10-year history of glaucoma on the left side,underwent hysterectomy for uterine fibroids 5 years ago,had no hypertension,heart disease,diabetes,hepatitis or tuberculosis,had no history of smoking and had been drinking for 20 years.CONCLUSION Accurate preoperative diagnosis is difficult,surgical resection is the main treatment,and a variety of nonsurgical treatment methods are auxiliary.Combined treatment can effectively reduce the risk of postoperative recurrence.The prognosis is still good,and the risk of recurrence of secondary surgery is greatly increased.

Gingival Fibromatosis

Presented in this paper is a clinico-pathological report of 11 cases of gingival fibromatosis. Of the 11 patients in our series, 2 cases (18.2%) with family history began to have symptom from age of 3, which is manifested by the involvement of whole gum. 9 patients (81.8%), for which the etiology was unknown, had onset of the disease at the periods of deciduous dentition, mixed dentition or permanent dentition, with localized or overall involvement of gingiva. The disease is characterized by diffuse or localized proliferation of gum. Microscopically, the proliferating gum tissue revealed well-developed fibrotic structure with few cells; and the fibers were densely arranged without clear boundary. The fibrotic tissues showed a diminishing of blood vessels and the infiltration of inflammatory cells is not conspicuous.