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 aggres...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.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
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 aggress...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.展开更多
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 growi...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.展开更多
Behavioral problems have a high rate in patients with temporal lobe epilepsy but there have been few detailed reports about pediatric patients. We report two children with temporal lobe epilepsy who manifest behavior ...Behavioral problems have a high rate in patients with temporal lobe epilepsy but there have been few detailed reports about pediatric patients. We report two children with temporal lobe epilepsy who manifest behavior abnormality remaining after surgery. One child developed complex partial seizures at 10 years and 3 months of age caused by a left temporal ganglioglioma and manifested behavioral problems of lability, aggression, impulsivity and disinhibition. Seizures were relieved after temporal lobe resection at 10 years and 8 months of age but behavioral symptoms had been remained. The second child developed epilepsy at 3 years of age. He manifested similar behavioral problems at 7 years old. Seizures were relieved after temporal lobe resection at 13 years old but behavioral symptoms had been remained. Their courses suggested that aggressive behavior might be associated with not only temporal lesion but also other lesions such as frontal lobe or neural circuitry between both lobes. It is important to inform families of patients that behavioral disorders may not improve after epileptic surgery.展开更多
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 a...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.展开更多
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 intermitte...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.展开更多
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 no...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.展开更多
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 ri...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.展开更多
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,...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.展开更多
Aggressive fibromatosis is a rare disease. The pathological feature of tile disease is that the tumorcells appeal as benign in biological behavior. So structures such as bone, morphology, but aggressive in the tumor i...Aggressive fibromatosis is a rare disease. The pathological feature of tile disease is that the tumorcells appeal as benign in biological behavior. So structures such as bone, morphology, but aggressive in the tumor infiltrates adjacent vessels and nerves as well as having a high recurrence rate after surgical resection, but lacks the capacity for forming distant metastases. We here report a patient who underwent a series of imaging examinations and surgery.展开更多
基金Supported by Hangzhou Health Science and Technology Program,No.A20210271.
文摘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.
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.
文摘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.
文摘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.
文摘Behavioral problems have a high rate in patients with temporal lobe epilepsy but there have been few detailed reports about pediatric patients. We report two children with temporal lobe epilepsy who manifest behavior abnormality remaining after surgery. One child developed complex partial seizures at 10 years and 3 months of age caused by a left temporal ganglioglioma and manifested behavioral problems of lability, aggression, impulsivity and disinhibition. Seizures were relieved after temporal lobe resection at 10 years and 8 months of age but behavioral symptoms had been remained. The second child developed epilepsy at 3 years of age. He manifested similar behavioral problems at 7 years old. Seizures were relieved after temporal lobe resection at 13 years old but behavioral symptoms had been remained. Their courses suggested that aggressive behavior might be associated with not only temporal lesion but also other lesions such as frontal lobe or neural circuitry between both lobes. It is important to inform families of patients that behavioral disorders may not improve after epileptic surgery.
文摘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.
文摘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.
基金supported by the Medical and Health Science and Technology Project of Zhejiang Province(No.2019325236)the Appropriate Technology Cultivation and Promotion Project of Zhejiang Medical and Health Office(2019325236).
文摘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.
文摘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.
文摘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.
文摘Aggressive fibromatosis is a rare disease. The pathological feature of tile disease is that the tumorcells appeal as benign in biological behavior. So structures such as bone, morphology, but aggressive in the tumor infiltrates adjacent vessels and nerves as well as having a high recurrence rate after surgical resection, but lacks the capacity for forming distant metastases. We here report a patient who underwent a series of imaging examinations and surgery.