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 di...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.展开更多
Desmoid-type fibromatosis (DF) is a rare, locally invasive, non-metastasizing soft tissue proliferation derived from mesenchymal progenitor cells. The incidence of DF is 2 to 4 per million per year in the general popu...Desmoid-type fibromatosis (DF) is a rare, locally invasive, non-metastasizing soft tissue proliferation derived from mesenchymal progenitor cells. The incidence of DF is 2 to 4 per million per year in the general population and typically affects adults between the ages of 35 - 40. Desmoid-type fibromatosis can either be sporadic or associated with mutation in the adenomatous polyposis coli gene. Trauma, surgery, pregnancy, and oral contraceptives have been identified as risk factors for the development of desmoid-type fibromatosis. MRI is the standard for image characterization, and CT image-guided core needle biopsy for diagnosis. “Wait and see” is the current management recommendation, and studies of y-secretase inhibitors and tyrosine kinase inhibitors have shown promise in the treatment of desmoid-type fibromatosis. This report presents a case of rare right shoulder desmoid type fibromatosis in a 48-year-old male that was missed on an initial workup including EMG/NCS and shoulder MRI, and demonstrates the importance of revisiting the diagnostic process if a former workup has yielded an unclear clinical picture.展开更多
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. The...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.展开更多
Desmoid-type fibromatosis (DF) is a rare monoclonal fibroblastic proliferation that is characterized by locally infiltrative but rarely metastatic lesions. Tyrosine kinase and γ-secretase inhibitors are primarily use...Desmoid-type fibromatosis (DF) is a rare monoclonal fibroblastic proliferation that is characterized by locally infiltrative but rarely metastatic lesions. Tyrosine kinase and γ-secretase inhibitors are primarily used in the targeted therapy of DF. The use of these drugs, however, is mainly based on the recommendations of retrospective studies with small sample sizes. Previous studies that focused on the mechanism, efficacy, and safety of targeted therapy for DF were reviewed to provide references for clinical applications and research. The efficacy and safety of targeted therapy were compared with those of other systemic therapy options. Targeted therapy does not provide considerable advantages in efficacy and safety over other medical treatments and is usually applied after the failure of antihormonal therapies, nonsteroidal anti-inflammatory drugs, and chemotherapy. Further studies are required to explore the mechanism, indications, and appropriate drug dosage of the targeted therapy of DF.展开更多
Desmoid-type fibromatoses are clonal fibroblastic proliferations that arise in deep soft tissues and are characterized by infiltrative growth and a tendency toward local recurrence. The molecular genetic studies show ...Desmoid-type fibromatoses are clonal fibroblastic proliferations that arise in deep soft tissues and are characterized by infiltrative growth and a tendency toward local recurrence. The molecular genetic studies show that there are chromosomal abnormalities relating to the pathogenesis of this lesion, such as trisomy 8. However, there are different results concerning this type of abnormality. Fluorescence in situ hybridization （FISH） is a sensitive and reproducible technique that combines molecular cytogenetics with morphologic information and brings them together in a single frame for evaluation. To explore the controversy of the aneuploid abnormality of chromosome 8, we collected specimens of desmoid-type fibromatosis to determine whether the existence of trisomy 8 chromosome abnormalities correlates with clinicopathologic features, as well as to investigate the feasibility of detecting trisomy 8 in FFPE tissue by FISH method.展开更多
目的探讨腹壁型韧带样纤维瘤病(abdominal wall type desmoids-type fibromatoses,DF)的MSCT表现,并评价MSCT在诊断DF及鉴别诊断的价值。方法回顾性分析经病理证实的DF患者12例MSCT表现,包括肿瘤部位、大小、形态、边缘、CT密度及强化...目的探讨腹壁型韧带样纤维瘤病(abdominal wall type desmoids-type fibromatoses,DF)的MSCT表现,并评价MSCT在诊断DF及鉴别诊断的价值。方法回顾性分析经病理证实的DF患者12例MSCT表现,包括肿瘤部位、大小、形态、边缘、CT密度及强化特征。结果 12例均位于腹前壁肌肉,横截面积最大约14.6cm×14.3cm,最小3.5cm×1.8cm,椭圆形8例,浅分叶状4例,边界清楚2例,模糊10例。MSCT平扫示12例全部呈稍低密度,密度较均匀,肿块均未见明显出血、囊变、坏死及钙化,有10例平扫可见少量条状稍高密度影。肿块增强后呈渐进性轻、中度强化,其中有6例增强后可见斑片状更高强化区,条状影未见明显强化。结论腹壁型韧带样纤维瘤MSCT表现具有特征性,正确的认识能为DF的诊断及鉴别提供重要价值。展开更多
文摘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.
文摘Desmoid-type fibromatosis (DF) is a rare, locally invasive, non-metastasizing soft tissue proliferation derived from mesenchymal progenitor cells. The incidence of DF is 2 to 4 per million per year in the general population and typically affects adults between the ages of 35 - 40. Desmoid-type fibromatosis can either be sporadic or associated with mutation in the adenomatous polyposis coli gene. Trauma, surgery, pregnancy, and oral contraceptives have been identified as risk factors for the development of desmoid-type fibromatosis. MRI is the standard for image characterization, and CT image-guided core needle biopsy for diagnosis. “Wait and see” is the current management recommendation, and studies of y-secretase inhibitors and tyrosine kinase inhibitors have shown promise in the treatment of desmoid-type fibromatosis. This report presents a case of rare right shoulder desmoid type fibromatosis in a 48-year-old male that was missed on an initial workup including EMG/NCS and shoulder MRI, and demonstrates the importance of revisiting the diagnostic process if a former workup has yielded an unclear clinical picture.
文摘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.
基金We would like to thank all faculty members who assisted us in this study. This work was supported by the Beijing Municipal Administration of Hospitals Clinical Medicine Development Special Funding Support (No. XMLX201708)the Capital Health Research and Development Special Funds (No. 2016-2-2151)+1 种基金Beijing Municipal Administration of Hospitafs Ascent Plan (No. DFL20181104)the National Natural Science Foundation of China (No. 31770836).
文摘Desmoid-type fibromatosis (DF) is a rare monoclonal fibroblastic proliferation that is characterized by locally infiltrative but rarely metastatic lesions. Tyrosine kinase and γ-secretase inhibitors are primarily used in the targeted therapy of DF. The use of these drugs, however, is mainly based on the recommendations of retrospective studies with small sample sizes. Previous studies that focused on the mechanism, efficacy, and safety of targeted therapy for DF were reviewed to provide references for clinical applications and research. The efficacy and safety of targeted therapy were compared with those of other systemic therapy options. Targeted therapy does not provide considerable advantages in efficacy and safety over other medical treatments and is usually applied after the failure of antihormonal therapies, nonsteroidal anti-inflammatory drugs, and chemotherapy. Further studies are required to explore the mechanism, indications, and appropriate drug dosage of the targeted therapy of DF.
文摘Desmoid-type fibromatoses are clonal fibroblastic proliferations that arise in deep soft tissues and are characterized by infiltrative growth and a tendency toward local recurrence. The molecular genetic studies show that there are chromosomal abnormalities relating to the pathogenesis of this lesion, such as trisomy 8. However, there are different results concerning this type of abnormality. Fluorescence in situ hybridization （FISH） is a sensitive and reproducible technique that combines molecular cytogenetics with morphologic information and brings them together in a single frame for evaluation. To explore the controversy of the aneuploid abnormality of chromosome 8, we collected specimens of desmoid-type fibromatosis to determine whether the existence of trisomy 8 chromosome abnormalities correlates with clinicopathologic features, as well as to investigate the feasibility of detecting trisomy 8 in FFPE tissue by FISH method.
