Mandibular Reconstruction in Cervical Desmoid Tumors

Introduction: Desmoid tumors are benign soft tissue tumors, but their infiltration character is responsible for big morbidity, especially in the cervicofacial location. Their management, therefore, represents a real challenge. Clinical case: Reporting a case of a 4-year-old girl presenting a desmoid tumor at the expense of the mandibular corpus and the right ascending ramus, with loco-regional infiltration, and whose treatment consisted of surgical resection of the tumor with Hemi-mandibulectomy and reconstruction according to the Masquelet technique. Conclusion: The traditional treatment consists of a large local excision with negative surgical margins. Head and neck fibromatosis, while rare, present a complex problem as they are often aggressive, and invade local bone, nerve and muscles, and can leave patients with a cosmetic deformity. In our case, it was a mandibular reconstruction after a large resection of a desmoid tumor with a location on the head and neck. With good consolidation, absence of recurrence, and without aesthetic sequelae at the control.

Clinical and imaging features of desmoid tumors of the extremities

BACKGROUND Desmoid fibroma is a rare soft tissue tumor originating from the aponeurosis,fascia,and muscle,and it is also known as aponeurotic fibroma,invasive fibroma,or ligamentous fibroma.AIM To investigate the clinical and imaging features of desmoid tumors of the extremities.METHODS Thirteen patients with desmoid fibroma of the extremities admitted to our hospital from October 2016 to March 2021 were included.All patients underwent computed tomography(CT),magnetic resonance imaging(MRI),and pathological examination of the lesion.Data on the diameter and distribution of the lesion,the relationship between the lesion morphology and surrounding structures,MRI and CT findings,and pathological features were statistically analyzed.RESULTS The lesion diameter ranged from 1.7 to 8.9 cm,with an average of 5.35±2.39 cm.All lesions were located in the deep muscular space,with the left and right forearm each accounting for 23.08%of cases.Among the 13 patients with desmoid fibroma of the extremities,the lesions were"patchy"in 1 case,irregular in 10,and quasi-round in 2.The boundary between the lesion and surrounding soft tissue was blurred in 10 cases,and the focus infiltrated along the tissue space and invaded the adjacent structures.Furthermore,the edge of the lesion showed"beard-like"infiltration in 2 cases;bone resorption and damage were found in 8,and bending of the bone was present in 2;the boundary of the focus was clear in 1.According to the MRI examination,the lesions were larger than 5 cm(61.54%),round or fusiform in shape(84.62%),had an unclear boundary(76.92%),showed uniform signal(69.23%),inhomogeneous enhancement(84.62%),and"root"or"claw"infiltration(69.23%).Neurovascular tract invasion was present in 30.77%of cases.CT examination showed that the desmoid tumors had slightly a lower density(69.23%),higher enhancement(61.54%),and unclear boundary(84.62%);a CT value<50 Hu was present in 53.85%of lesions,and the enhancement was uneven in 53.85%of cases.Microscopically,fibroblasts and myofibroblasts were arranged in strands and bundles,without obvious atypia but with occasional karyotyping;cells were surrounded by collagen tissue.There were disparities in the proportion of collagen tissue in different regions,with abundant collagen tissue and few tumor cells in some areas,similar to the structure of aponeuroses or ligaments,and tumor cells invading the surrounding tissues.CONCLUSION Desmoid tumors of the extremities have certain imaging features on CT and MRI.The two imaging techniques can be combined to improve the diagnostic accuracy,achieve a comprehensive diagnosis of the disease in the clinical practice,and reduce the risk of missed diagnosis or misdiagnosis.In addition,their use can ensure timely diagnosis and treatment.

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.

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.

An unusual case of fatty liver in a patient with desmoid tumor

A desmoid tumor,also known as aggressive fibromatosis,is a rare benign neoplasm that arises from fascial or musculoaponeurotic tissues.It can occur in any anatomical location,most commonly the abdominal wall,shoulder girdle and retroperitoneum.The typical clinical presentation is a painless mass with a slow and progressive invasion of contiguous structures.It is associated with a high local recurrence rate after resection.Many issues regarding the optimal treatment of desmoid tumors remain controversial.Aggressive surgical resection with a wide margin(2-3 cm) remains the gold standard treatment with regard to preserving quality of life.Radiotherapy alone has been shown to be effective for the control of unresectable or recurrent lesions.Desmoid tumors tend to be locally infiltrative,therefore,the fields must be generous to prevent marginal recurrence.The radiation dose appropriate for treating desmoid tumors remains controversial.We present a 25-year-old Caucasian man with local recurrence of a desmoid tumor after repeated surgical resection,treated with radiotherapy.The patient achieved complete tumor regression at 4 mo after radiotherapy,and he is clinically free of disease at 12 mo after the end of treatment,with an acceptable quality of life.The patient developed short bowel syndrome as a complication of second surgical resection.Consequently,radiotherapy might have worsened an already present malabsorption and so led to steatohepatitis.

Efficacy of vinorelbine combined with low-dose methotrexate for treatment of inoperable desmoid tumor and prognostic factor analysis

Objective： To assess the efficacy of conservative chemotherapy for inoperable desmoid tumor（DT） and analyze the prognostic factors.Methods：From November 2008 to April 2016,71 patients of inoperable DT were treated with vinorelbine and low-dose methotrexate in the Department of Bone and Soft Tissue Tumors,Peking University Cancer Hospital＆Institute,and enrolled in this retrospective study.The chemotherapy duration is one year.The efficacy of chemotherapy and the prognosis were observed.Results：Of the 71 patients,55% were female.Age of onset varied from 1 to 47 years,and the median age was 14years.Only 11（15.5%）cases suffered primary tumor.The distribution of the site of tumors was：31（43.7%）in the trunk,36（50.7%）in the limbs,and 4（5.6%）in the peritoneal and pelvic cavity.The size of tumor（the maximum diameter）differed from 2 to 37 cm with a mean of 9.3 cm.The median follow-up duration was 28（range,6–87）months.Common side effects included：nausea and vomiting,liver injury,bone marrow suppression and oral ulcers.When the chemotherapy finished,1（1.4%）case achieved complete response,24（33.8%）achieved partial response,37（52.1%）achieved stable disease and 9（12.7%）had progressive disease.The overall response rate was 87.3%.The progression-free survival（PFS）of the participants were from 6 to 87 months,and the 2-,3-and 5-year PFS was 79.9%,68.4% and 36.3%,respectively.No significant difference was identified in PFS in subgroups of gender,age of onset,age of chemotherapy,tumor site and tumor size.Conclusions：For recurrent,inoperable and progressive DT,enough course of chemotherapy with vinorelbine combined with low-dose methotrexate was an optional choice for local control.

Postoperative retroperitoneal desmoid tumor mimics recurrent gastrointestinal stromal tumor:A case report

Desmoid tumor is a locally invasive,myofibroblastic,nonmetastatic tumor.Its pathogenesis remains unclear and it may involve genetic abnormalities,sex hormones and traumatic injury,including surgery.Postoperative intra-abdominal desmoid tumor is rare,especially in the retroperitoneum.We report a case of postoperative retroperitoneal desmoid tumor that developed 29 mo after the first excision of a gastrointestinal stromal tumor.Sporadic trauma-related intraabdominal desmoid tumors reported in the English literature are also reviewed.Despite an extremely low incidence,postoperative desmoid tumor should be considered in the differential diagnosis when a recurrent neoplasm is found at least one year after operation.However,it is a clinical challenge to distinguish recurrent malignant neoplasms from desmoid tumors,and surgical resection is the treatment option depending on the anatomic location.

Gardner's syndrome: A case report and review of the literature

Gardner's syndrome is an autosomal dominant disease characterized by the presence of colonic polyposis,osteomas and a multitude of soft tissue tumors. The syndrome may present at any age from 2 mo to 70 years with a variety of symptoms, either colonic or extracolonic.We present a case of a 11-year-old female patient with Gardner's syndrome who presented with a lumbar area desmoid tumor and treated with resection of the desmoid,restorative proctocolectomy and ileal pouch anal anastomosis,A review of the current literature has been performed.

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.