Primary malignant fibrous histiocytoma (MFH) of the pancreas is rare and a distinct clinical entity. We report a case of recurrence of pancreatic MFH with computed tomography (CT) and magnetic resonance imaging (MRI) ...Primary malignant fibrous histiocytoma (MFH) of the pancreas is rare and a distinct clinical entity. We report a case of recurrence of pancreatic MFH with computed tomography (CT) and magnetic resonance imaging (MRI) findings. A 67-year-old man presented with a history of decreased body weight over the past 6 mo. Abdominal CT revealed a large, multilocular cystic mass in the head of the pancreas with obvious atrophy in the body and tail of the pancreas. After 6 mo postoperatively, MRI demonstrated a recurrent large mass in the primary area of the head of the pancreas. The lesion was heterogeneous, hypointense to the liver on T1-weighted imaging, and heterogeneously hyperintense to the liver with a hypointense area in the central part of the tumor on fat-saturated T2-weighted imaging. Contrast- enhanced T1-weighted imaging demonstrated a large multilocular cystic mass with a cystic wall, fibrous septa and enhancement of solid components. To the best of our knowledge, this is the first report on recurrence of primary MFH of the pancreas, and the first with MRI findings.展开更多
Malignant fibrous histiocytoma (MFH) occurs most commonly in the extremities and trunk, but rarely in the intestine. Here we report two cases of primary intestinal MFH. The first case was a 70-year old man admitted fo...Malignant fibrous histiocytoma (MFH) occurs most commonly in the extremities and trunk, but rarely in the intestine. Here we report two cases of primary intestinal MFH. The first case was a 70-year old man admitted for recurrent right lower quadrant abdominal pain. At laparotomy, a tumor was found originating from the cecum, with a suspicious metastatic nodule on the surface of the right lobe of the liver. A right hemicolectomy was performed followed by an ileotransverse end-to-end anastomotic reconstruction. The second case was a 43-year old man with intussusceptions of the small intestine. An emergent laparotomy revealed 4 pedunculated masses in the small bowel and a partial resection of the small intestine was performed. Though the symptoms were not typical, based on histological and immunohistochemical studies, the patients were diagnosed as MFH of the intestine. They were not treated with chemotherapy or radiotherapy and both died within 3 mo. MFH of the intestine is an extremely rare neoplasm with an aggressive biological behavior. The pathogenesis of this disease has not been clarified to date. Complete surgical excision is preferred, adjuvant chemotherapy or radiotherapy may be advisable.展开更多
Aneurysmal fibrous histiocytoma is often clinically misdiagnosed.In this study,we put forward an insight on how to help diagnose this disease clinically.A retrospective chart review was performed on all patients diagn...Aneurysmal fibrous histiocytoma is often clinically misdiagnosed.In this study,we put forward an insight on how to help diagnose this disease clinically.A retrospective chart review was performed on all patients diagnosed with aneuiysmal fibrous histiocytoma from 2007 to 2017 in the Department of Dermatology,Union Hospital,China,and all clinical data were collected from the hospital archives.From a total of 418 patients diagnosed with cutaneous fibrous histiocytoma,only 30 patients were confirmed to have aneurysmal fibrous histiocytoma out of which only 2 patients were clinically diagnosed with aneurysmal fibrous histiocytoma.The remaining 28 patients were diagnosed with various types of vascular tumors although pathology classified them as having aneurysmal fibrous histiocytoma.Among the 30 patients,9 were male and 21 were female.There were following age groups:13-19(mean 16,n=4),20-29(mean 26.25,n=8),30-39(mean 33,n=7),40-49(mean 44,n=4),50-59(mean 56.75,n=4)5 60 and above(mean 61,n=3).Tumors were present on the head,neck,back,waist,hips and upper and lower extremities.After complete excision,there was no recurrence and no complications.Histologically,lesions showed the typical pseudoangiomatoid spaces without endothelial lining and infiltration of fibrohistiocytes in hemosiderotic pigmentation.It was suggested that although the prognosis of aneurysmal fibrous histiocytoma is good,accurate diagnosis is paramount to avoid clinical misdiagnosis and subsequent complications.展开更多
BACKGROUND Angiomatoid fibrous histiocytoma(AFH)is a rare,slow-growing soft tissue tumor.It appears mostly on the limbs and trunk in children and young adults.The biology of AFH remains unclear because of the small nu...BACKGROUND Angiomatoid fibrous histiocytoma(AFH)is a rare,slow-growing soft tissue tumor.It appears mostly on the limbs and trunk in children and young adults.The biology of AFH remains unclear because of the small number of reported cases.Diagnostic testing does not provide definitive results.It has two clinical forms,that differ in terms of gene expression and clinical prognosis.It is important to inform the laboratory which specific gene testing is necessary.Here,we describe a case of rare AFH in the submandibular region using a full genetic panel.CASE SUMMARY A 13-year-old boy who had been misdiagnosed in the past 6 mo by his dentist visited our clinic because of a lesion in the submandibular area on the right side.The lesion was homogeneous and painless upon palpation.No skin discoloration was observed.Due to the non-specific radiological picture computed tomography(CT),magnetic resonance imaging(MRI),cone-beam CT(CBCT),and ultrasoundguided biopsy were performed.A venous malformation was suspected on the MRI.None of the tests provided a definitive diagnosis.Owing to the non-specific radiological findings,the patient qualified for surgical treatment.The surgical procedure included an excisional biopsy.The diagnostic testing was extended using gene rearrangements.The most distinctive gene translocation in diagnosing AFH is within the EWS RNA-binding protein 1(EWSR1)-CREB-binding protein.However,in this case,the diagnosis was confirmed by a rearrangement within the EWSR1 gene testing.CONCLUSION AFH in the submandibular location is rare,and surgical treatment with genetic evaluation defines AFH type that affects subsequent procedures.展开更多
Malignant fibrous histiocytoma (MFH) is a pleomorphic mesenchynal sarcoma. It is uncommonly arises primarily from the intra-peritoneal cavity. Primary peritoneal MFH with tumor bleeding and rupture is rare. We describ...Malignant fibrous histiocytoma (MFH) is a pleomorphic mesenchynal sarcoma. It is uncommonly arises primarily from the intra-peritoneal cavity. Primary peritoneal MFH with tumor bleeding and rupture is rare. We describe the imaging features of a 70-year-old patient presenting with ruptured hemorrhagic peritoneal MFH at subhepatic area,accompanied by massive hemoperitoneum,mimicking a ruptured pedunculated hepatocellular carcinoma. Computed tomography (CT) revealed a large heterogeneous enhanced subhepatic mass with adjacent liver,gallbladder and colon invasion. Tumor hemorrhage and rupture complicated with peritoneal seeding and massive bloody ascites were also detected. Angiography showed a hypervascular tumor fed by enlarged right hepatic arteries,cystic artery and omental branches of gastroepiploic artery. The patient underwent laparotomy for tumor resection,but the tumor recurred one month after operation. To our knowledge,the CT appearance of ruptured intraperitoneal MFH complicated by hemoperitoneum has not been previously described.展开更多
Purpose: To define the sites and CT findings of 45 MFH Patients. Materials and Methods: The primary sites were:cranio-facial 16, trunk and extremity 12 and abdominal 17 (13 retroperitoneal). As seen on CT scans, the l...Purpose: To define the sites and CT findings of 45 MFH Patients. Materials and Methods: The primary sites were:cranio-facial 16, trunk and extremity 12 and abdominal 17 (13 retroperitoneal). As seen on CT scans, the lesion is clear-margined and even when small, but, when large (42/50,>5 cm), it is uneven in consistency and apt to invade the nearby organs (75.8%). The CT Findings of untreated and recurrent MFH are similar. Results:Enhanced CT scans may provide useful information such as medium to hyper-attenuation (78.8%), necrosis (60.6%)or involvement of para-nasal sinuses as expanding deformity (8/9 cases). Conclusions: CT scan is important to delineate the extent of MFH Iesions of which the diagnosis is best ascertained by combining CT scan and histopathology. Enhanced CT scans can reveal much useful information.展开更多
文摘Primary malignant fibrous histiocytoma (MFH) of the pancreas is rare and a distinct clinical entity. We report a case of recurrence of pancreatic MFH with computed tomography (CT) and magnetic resonance imaging (MRI) findings. A 67-year-old man presented with a history of decreased body weight over the past 6 mo. Abdominal CT revealed a large, multilocular cystic mass in the head of the pancreas with obvious atrophy in the body and tail of the pancreas. After 6 mo postoperatively, MRI demonstrated a recurrent large mass in the primary area of the head of the pancreas. The lesion was heterogeneous, hypointense to the liver on T1-weighted imaging, and heterogeneously hyperintense to the liver with a hypointense area in the central part of the tumor on fat-saturated T2-weighted imaging. Contrast- enhanced T1-weighted imaging demonstrated a large multilocular cystic mass with a cystic wall, fibrous septa and enhancement of solid components. To the best of our knowledge, this is the first report on recurrence of primary MFH of the pancreas, and the first with MRI findings.
文摘Malignant fibrous histiocytoma (MFH) occurs most commonly in the extremities and trunk, but rarely in the intestine. Here we report two cases of primary intestinal MFH. The first case was a 70-year old man admitted for recurrent right lower quadrant abdominal pain. At laparotomy, a tumor was found originating from the cecum, with a suspicious metastatic nodule on the surface of the right lobe of the liver. A right hemicolectomy was performed followed by an ileotransverse end-to-end anastomotic reconstruction. The second case was a 43-year old man with intussusceptions of the small intestine. An emergent laparotomy revealed 4 pedunculated masses in the small bowel and a partial resection of the small intestine was performed. Though the symptoms were not typical, based on histological and immunohistochemical studies, the patients were diagnosed as MFH of the intestine. They were not treated with chemotherapy or radiotherapy and both died within 3 mo. MFH of the intestine is an extremely rare neoplasm with an aggressive biological behavior. The pathogenesis of this disease has not been clarified to date. Complete surgical excision is preferred, adjuvant chemotherapy or radiotherapy may be advisable.
基金the Natural Science Foundation of China(No.81472886).
文摘Aneurysmal fibrous histiocytoma is often clinically misdiagnosed.In this study,we put forward an insight on how to help diagnose this disease clinically.A retrospective chart review was performed on all patients diagnosed with aneuiysmal fibrous histiocytoma from 2007 to 2017 in the Department of Dermatology,Union Hospital,China,and all clinical data were collected from the hospital archives.From a total of 418 patients diagnosed with cutaneous fibrous histiocytoma,only 30 patients were confirmed to have aneurysmal fibrous histiocytoma out of which only 2 patients were clinically diagnosed with aneurysmal fibrous histiocytoma.The remaining 28 patients were diagnosed with various types of vascular tumors although pathology classified them as having aneurysmal fibrous histiocytoma.Among the 30 patients,9 were male and 21 were female.There were following age groups:13-19(mean 16,n=4),20-29(mean 26.25,n=8),30-39(mean 33,n=7),40-49(mean 44,n=4),50-59(mean 56.75,n=4)5 60 and above(mean 61,n=3).Tumors were present on the head,neck,back,waist,hips and upper and lower extremities.After complete excision,there was no recurrence and no complications.Histologically,lesions showed the typical pseudoangiomatoid spaces without endothelial lining and infiltration of fibrohistiocytes in hemosiderotic pigmentation.It was suggested that although the prognosis of aneurysmal fibrous histiocytoma is good,accurate diagnosis is paramount to avoid clinical misdiagnosis and subsequent complications.
文摘BACKGROUND Angiomatoid fibrous histiocytoma(AFH)is a rare,slow-growing soft tissue tumor.It appears mostly on the limbs and trunk in children and young adults.The biology of AFH remains unclear because of the small number of reported cases.Diagnostic testing does not provide definitive results.It has two clinical forms,that differ in terms of gene expression and clinical prognosis.It is important to inform the laboratory which specific gene testing is necessary.Here,we describe a case of rare AFH in the submandibular region using a full genetic panel.CASE SUMMARY A 13-year-old boy who had been misdiagnosed in the past 6 mo by his dentist visited our clinic because of a lesion in the submandibular area on the right side.The lesion was homogeneous and painless upon palpation.No skin discoloration was observed.Due to the non-specific radiological picture computed tomography(CT),magnetic resonance imaging(MRI),cone-beam CT(CBCT),and ultrasoundguided biopsy were performed.A venous malformation was suspected on the MRI.None of the tests provided a definitive diagnosis.Owing to the non-specific radiological findings,the patient qualified for surgical treatment.The surgical procedure included an excisional biopsy.The diagnostic testing was extended using gene rearrangements.The most distinctive gene translocation in diagnosing AFH is within the EWS RNA-binding protein 1(EWSR1)-CREB-binding protein.However,in this case,the diagnosis was confirmed by a rearrangement within the EWSR1 gene testing.CONCLUSION AFH in the submandibular location is rare,and surgical treatment with genetic evaluation defines AFH type that affects subsequent procedures.
文摘Malignant fibrous histiocytoma (MFH) is a pleomorphic mesenchynal sarcoma. It is uncommonly arises primarily from the intra-peritoneal cavity. Primary peritoneal MFH with tumor bleeding and rupture is rare. We describe the imaging features of a 70-year-old patient presenting with ruptured hemorrhagic peritoneal MFH at subhepatic area,accompanied by massive hemoperitoneum,mimicking a ruptured pedunculated hepatocellular carcinoma. Computed tomography (CT) revealed a large heterogeneous enhanced subhepatic mass with adjacent liver,gallbladder and colon invasion. Tumor hemorrhage and rupture complicated with peritoneal seeding and massive bloody ascites were also detected. Angiography showed a hypervascular tumor fed by enlarged right hepatic arteries,cystic artery and omental branches of gastroepiploic artery. The patient underwent laparotomy for tumor resection,but the tumor recurred one month after operation. To our knowledge,the CT appearance of ruptured intraperitoneal MFH complicated by hemoperitoneum has not been previously described.
文摘Purpose: To define the sites and CT findings of 45 MFH Patients. Materials and Methods: The primary sites were:cranio-facial 16, trunk and extremity 12 and abdominal 17 (13 retroperitoneal). As seen on CT scans, the lesion is clear-margined and even when small, but, when large (42/50,>5 cm), it is uneven in consistency and apt to invade the nearby organs (75.8%). The CT Findings of untreated and recurrent MFH are similar. Results:Enhanced CT scans may provide useful information such as medium to hyper-attenuation (78.8%), necrosis (60.6%)or involvement of para-nasal sinuses as expanding deformity (8/9 cases). Conclusions: CT scan is important to delineate the extent of MFH Iesions of which the diagnosis is best ascertained by combining CT scan and histopathology. Enhanced CT scans can reveal much useful information.
