BACKGROUND Plexiform fibromyxoma(PF)is a rare mesenchymal tumor of the stomach.The clinical features of PF frequently include upper abdominal pain,abdominal discomfort,hematemesis,melena,pyloric obstruction and an upp...BACKGROUND Plexiform fibromyxoma(PF)is a rare mesenchymal tumor of the stomach.The clinical features of PF frequently include upper abdominal pain,abdominal discomfort,hematemesis,melena,pyloric obstruction and an upper abdominal mass.We herein report a case of PF resected by laparoscopic radical distal gastrectomy plus Roux-en-Y gastrojejunostomy.CASE SUMMARY The patient was admitted to hospital,due to a 1-wk history of an abdominal space-occupying lesion identified during a health examination.He underwent complete resection by laparoscopic radical distal gastrectomy plus Roux-en-Y gastrojejunostomy.During the operation,the tumor was located in the anterior wall of the gastric antrum(approximately 7 cm×6 cm×5.5 cm)and did not show evidence of invasion of the serosa.Histology showed that the tumor cells were oval fibroblast-like and spindle-shaped cells,with numerous thin-walled blood vessels and abundant myxoid stroma.Cellular atypia and mitosis were both rare.Immunohistochemistry showed that the tumor cells were immunoreactive for smooth muscle actin,S-100 and CD-10,but were negative for CD-117,CD-34,DOG-1,and ALK.In this case,S-100 was positive and no significant disease was observed during the follow-up period.CONCLUSION The fact that PF is a rare tumor with only a few cases in this region can lead to misdiagnosis of this entity and pose a real diagnostic challenge for general surgeons and pathologists when encountering such patients and differentiating PF from other primary tumors of gastric mesenchymal origin.Our report may help increase awareness of this rare,but important new disease entity.展开更多
BACKGROUND Plexiform fibromyxoma is a rare, special type of mesenchymal tumor. The most common presenting symptoms are anemia, hematemesis, and hematochezia, without sex or age predilection. The reported cases have ma...BACKGROUND Plexiform fibromyxoma is a rare, special type of mesenchymal tumor. The most common presenting symptoms are anemia, hematemesis, and hematochezia, without sex or age predilection. The reported cases have mainly occurred in the gastric antrum and pylorus region, with some cases in the duodenum. CASE SUMMARY We report here a case of plexiform fibromyxoma in the upper segment of the jejunum, which was continuously followed up for 3 years after surgical removal. Plexiform fibromyxoma showed multinodular or plexiform growth. The cells in the tumor node were spindle-shaped but few in number and mitotic figures. Small blood vessels and mucous matrix were found among the tumor cells. Immunohistochemistry revealed that the plexiform fibromyxoma cells were positive for smooth muscle actin, focally positive for CD10, and negative for cytokeratin, CD117, DOG-1(discovered on GIST-1) desmin, S-100, epithelial membrane antigen, and CD34. Ki-67 labeling index was < 5%. Plexiform fibromyxoma showed benign biological behavior. After 3 years of consecutive postoperative follow-up, no obvious signs of metastasis or recurrence were found by imaging examination. CONCLUSION Plexiform fibromyxoma is a rare type of mesenchymal tumor. The diagnosis mainly depends on pathological examination, and it should be distinguished from other gastrointestinal mesenchymal tumors.展开更多
A 66-year-old man was diagnosed with a gastric submucosal tumor. Endoscopic ultrasound(EUS) revealed an iso/hypoechoic mass in the third layer. No malignant cells were detected in a histological examination. Yearly fo...A 66-year-old man was diagnosed with a gastric submucosal tumor. Endoscopic ultrasound(EUS) revealed an iso/hypoechoic mass in the third layer. No malignant cells were detected in a histological examination. Yearly follow-up endoscopy and EUS showed the slow growth of the tumor. Endoscopic submucosal dissection(ESD) was performed and a glistening tumor was resected. The lesion showed a multinodular plexiform growth pattern consisting of spindle cells with an abundant fibromyxoid stroma that was rich in small vessels. The tumor was diagnosed as plexiform fibromyxoma(PF) by immunohistochemistry. Although difficulties are associated with reaching a diagnosis preoperatively, chronological changes on EUS may contribute to the diagnosis of PF. ESD may also be useful in the diagnosis and treatment of PF.展开更多
Plexiform fibromyxoma(PF)is a very rare mesenchymal neoplasm of the stomach that was first described in 2007 and was officially recognized as a subtype of gastric mesenchymal neoplasm by World Health Organization(WHO)...Plexiform fibromyxoma(PF)is a very rare mesenchymal neoplasm of the stomach that was first described in 2007 and was officially recognized as a subtype of gastric mesenchymal neoplasm by World Health Organization(WHO)in 2010.Histologically,PF is characterized by a plexiform growth of bland spindle to ovoid cells embedded in a myxoid stroma that is rich in small vessels.The lesion is usually paucicellular.While mucosal and vascular invasion have been documented,no metastasis or malignant transformation has been reported.Its pathogenesis is largely unknown and defining molecular alterations are not currently available.There are other mesenchymal tumors arising in the gastrointestinal tract that need to be differentiated from PF given their differing biologic behaviors and malignant potential.Histologic mimics with spindle cells include gastrointestinal stromal tumor,smooth muscle tumor,and nerve sheath tumor.Histologic mimics with myxoid stroma include myxoma and aggressive angiomyxoma.Molecular alterations that have been described in a subset of PF may be seen in gastroblastoma and malignant epithelioid tumor with gliomaassociated oncogene homologue 1(GLI1)rearrangement.The recent increase in publications on PF reflects growing recognition of this entity with expansion of clinical and pathologic findings in these cases.Herein we provide a review of PF in comparison to other mesenchymal tumors with histologic and molecular resemblance to raise the awareness of this enigmatic neoplasm.Also,we highlight the challenges pathologists face when the sample is small,or such rare entity is encountered intraoperatively.展开更多
Plexiform fibromyxoma is a very rare mesenchymal tumor of the stomach, found almost exclusively in the antrum/pylorus region. The most common presenting symptoms are anemia, hematemesis, nausea and unintentional weigh...Plexiform fibromyxoma is a very rare mesenchymal tumor of the stomach, found almost exclusively in the antrum/pylorus region. The most common presenting symptoms are anemia, hematemesis, nausea and unintentional weight loss, without sex or age predilection. We describe here two cases of plexiform fibromyxoma, involving a 16-year-old female and a 34-year-old male. Both patients underwent complete resection(R0) by distal gastrectomy and retrocolic gastrojejunostomy(according to Billroth 2); for both, the postoperative course was uneventful. Histology showed multiple intramural and subserosal nodules with characteristic plexiform growth, featuring bland spindle cells situated in an abundant myxoid stroma with low mitotic activity. Immunohistochemistry showed α-smooth muscle actin-positive spindle cells, focal positivity for CD10, and negative staining for KIT, DOG1, CD34, S100, β-catenin, STAT-6 and anaplastic lymphoma kinase. One of the cases showed focal positivity for h-caldesmon and desmin. Upon followup, no sign of disease was found. In the differential diagnosis of plexiform fibromyxoma, it is important to exclude the more common gastrointestinal stromal tumors as they have greater potential for aggressivebehavior. Other lesions, like neuronal and vascular tumors, inflammatory fibroid polyps, abdominal desmoid-type fibromatosis, solitary fibrous tumors and smooth muscle tumors, must also be excluded.展开更多
BACKGROUND Plexiform fibromyxoma(PF)is a rare mesenchymal tumor,with limited case reports worldwide.Common clinical symptoms are abdominal discomfort and bleeding signs,which frequently present slow-onset in reported ...BACKGROUND Plexiform fibromyxoma(PF)is a rare mesenchymal tumor,with limited case reports worldwide.Common clinical symptoms are abdominal discomfort and bleeding signs,which frequently present slow-onset in reported cases.Herein,we report a case of gastric PF presenting as acute onset and with pyemia accompanying tumor rupture.We resected the tumor as well as the distal gastric,bulbus duodeni and gallbladder for treatment in emergency surgery.Notably,before the onset of the disease,the patient received coronavirus disease 2019(COVID-19)vaccines.CASE SUMMARY A 26-year-old man was admitted to our hospital,due to abdominal pain and fever after having received COVID-19 vaccines.Laboratory examination indicated severe sepsis.Computed tomography scan revealed a large mass in the abdomen.Deformation of the gastrointestinal tract was seen during gastroscopy.After failure of anti-infective treatment and symptoms of shock developed,he received an emergency surgery.We found a huge and partly ruptured mass,with thick purulence.Microscopically,the mass was composed of spindle cells with clarified cytoplasm,accompanied by myxoid stroma and arborizing blood vessels.Immunohistochemistry showed the tumor cells as positive for smooth muscle actin and succinate dehydrogenase subunit B but negative for DOG-1 and CD117.Finally,the patient was diagnosed with gastric PF and discharged from the hospital.CONCLUSION Gastric PF manifesting as tumor rupture combined with pyemia is rare.Timely surgery is critical for optimal prognosis.展开更多
Superficial acral fibromyxoma (SAF) is a tumor that occurs on the distal phalanges of the digits. As it does not spontaneously regress and is often associated with pain, the primary treatment is surgical resection. It...Superficial acral fibromyxoma (SAF) is a tumor that occurs on the distal phalanges of the digits. As it does not spontaneously regress and is often associated with pain, the primary treatment is surgical resection. It is often associated with the nail component of the affected digit, and thus cosmesis is an important goal of the operation. We herein describe a case of SAF on the distal phalanges of the fifth digit of the foot beneath the nail, which was successfully resected with the nail component kept intact. Moreover, although SAF is most commonly CD34-positive, the present case was CD34-negative except for endothelial cells within the tumor. While CD34-negative SAF has been previously reported, the current case further indicates that CD34-positivity is not essential for the diagnosis of SAF.展开更多
Plexiform angiomyxoid myofibroblastic tumor of the stomach is a unique mesenchymal tumor that we first described in 2007.The tumor is very rare,and to date,only 18 cases confirmed by immunohistochemistry have been rep...Plexiform angiomyxoid myofibroblastic tumor of the stomach is a unique mesenchymal tumor that we first described in 2007.The tumor is very rare,and to date,only 18 cases confirmed by immunohistochemistry have been reported in the literature.The patients' ages ranged from 7 to 75 years(mean,43 years),and the male-to-female ratio was approximately 1:1.Representative clinical symptoms are ulceration,associated upper gastrointestinal bleeding(hematemesis),and anemia.The tumors are located at the antrum in all cases,and grossly,the tumor is whitish to brownish or reddish,and forms a lobulated submucosal or transmural mass.Microscopically,the tumor is characterized by a plexiform growth pattern,the proliferation of cytologically bland spindle cells,and a myxoid stroma that is rich in small vessels and positive for Alcian blue stain.Immunohistochemically,the tumor cells are positive for α-smooth muscle actin and negative for KIT and CD34.Differential diagnoses include gastrointestinal stromal tumor and other mesenchymal tumors of the gastrointestinal tract.Some authors proposed that this tumor should be designated as "plexiform fibromyxoma",but this designation might cause confusion.The tumor is probably benign and thus far,neither recurrence nor metastasis has been reported.展开更多
文摘BACKGROUND Plexiform fibromyxoma(PF)is a rare mesenchymal tumor of the stomach.The clinical features of PF frequently include upper abdominal pain,abdominal discomfort,hematemesis,melena,pyloric obstruction and an upper abdominal mass.We herein report a case of PF resected by laparoscopic radical distal gastrectomy plus Roux-en-Y gastrojejunostomy.CASE SUMMARY The patient was admitted to hospital,due to a 1-wk history of an abdominal space-occupying lesion identified during a health examination.He underwent complete resection by laparoscopic radical distal gastrectomy plus Roux-en-Y gastrojejunostomy.During the operation,the tumor was located in the anterior wall of the gastric antrum(approximately 7 cm×6 cm×5.5 cm)and did not show evidence of invasion of the serosa.Histology showed that the tumor cells were oval fibroblast-like and spindle-shaped cells,with numerous thin-walled blood vessels and abundant myxoid stroma.Cellular atypia and mitosis were both rare.Immunohistochemistry showed that the tumor cells were immunoreactive for smooth muscle actin,S-100 and CD-10,but were negative for CD-117,CD-34,DOG-1,and ALK.In this case,S-100 was positive and no significant disease was observed during the follow-up period.CONCLUSION The fact that PF is a rare tumor with only a few cases in this region can lead to misdiagnosis of this entity and pose a real diagnostic challenge for general surgeons and pathologists when encountering such patients and differentiating PF from other primary tumors of gastric mesenchymal origin.Our report may help increase awareness of this rare,but important new disease entity.
文摘BACKGROUND Plexiform fibromyxoma is a rare, special type of mesenchymal tumor. The most common presenting symptoms are anemia, hematemesis, and hematochezia, without sex or age predilection. The reported cases have mainly occurred in the gastric antrum and pylorus region, with some cases in the duodenum. CASE SUMMARY We report here a case of plexiform fibromyxoma in the upper segment of the jejunum, which was continuously followed up for 3 years after surgical removal. Plexiform fibromyxoma showed multinodular or plexiform growth. The cells in the tumor node were spindle-shaped but few in number and mitotic figures. Small blood vessels and mucous matrix were found among the tumor cells. Immunohistochemistry revealed that the plexiform fibromyxoma cells were positive for smooth muscle actin, focally positive for CD10, and negative for cytokeratin, CD117, DOG-1(discovered on GIST-1) desmin, S-100, epithelial membrane antigen, and CD34. Ki-67 labeling index was < 5%. Plexiform fibromyxoma showed benign biological behavior. After 3 years of consecutive postoperative follow-up, no obvious signs of metastasis or recurrence were found by imaging examination. CONCLUSION Plexiform fibromyxoma is a rare type of mesenchymal tumor. The diagnosis mainly depends on pathological examination, and it should be distinguished from other gastrointestinal mesenchymal tumors.
文摘A 66-year-old man was diagnosed with a gastric submucosal tumor. Endoscopic ultrasound(EUS) revealed an iso/hypoechoic mass in the third layer. No malignant cells were detected in a histological examination. Yearly follow-up endoscopy and EUS showed the slow growth of the tumor. Endoscopic submucosal dissection(ESD) was performed and a glistening tumor was resected. The lesion showed a multinodular plexiform growth pattern consisting of spindle cells with an abundant fibromyxoid stroma that was rich in small vessels. The tumor was diagnosed as plexiform fibromyxoma(PF) by immunohistochemistry. Although difficulties are associated with reaching a diagnosis preoperatively, chronological changes on EUS may contribute to the diagnosis of PF. ESD may also be useful in the diagnosis and treatment of PF.
文摘Plexiform fibromyxoma(PF)is a very rare mesenchymal neoplasm of the stomach that was first described in 2007 and was officially recognized as a subtype of gastric mesenchymal neoplasm by World Health Organization(WHO)in 2010.Histologically,PF is characterized by a plexiform growth of bland spindle to ovoid cells embedded in a myxoid stroma that is rich in small vessels.The lesion is usually paucicellular.While mucosal and vascular invasion have been documented,no metastasis or malignant transformation has been reported.Its pathogenesis is largely unknown and defining molecular alterations are not currently available.There are other mesenchymal tumors arising in the gastrointestinal tract that need to be differentiated from PF given their differing biologic behaviors and malignant potential.Histologic mimics with spindle cells include gastrointestinal stromal tumor,smooth muscle tumor,and nerve sheath tumor.Histologic mimics with myxoid stroma include myxoma and aggressive angiomyxoma.Molecular alterations that have been described in a subset of PF may be seen in gastroblastoma and malignant epithelioid tumor with gliomaassociated oncogene homologue 1(GLI1)rearrangement.The recent increase in publications on PF reflects growing recognition of this entity with expansion of clinical and pathologic findings in these cases.Herein we provide a review of PF in comparison to other mesenchymal tumors with histologic and molecular resemblance to raise the awareness of this enigmatic neoplasm.Also,we highlight the challenges pathologists face when the sample is small,or such rare entity is encountered intraoperatively.
文摘Plexiform fibromyxoma is a very rare mesenchymal tumor of the stomach, found almost exclusively in the antrum/pylorus region. The most common presenting symptoms are anemia, hematemesis, nausea and unintentional weight loss, without sex or age predilection. We describe here two cases of plexiform fibromyxoma, involving a 16-year-old female and a 34-year-old male. Both patients underwent complete resection(R0) by distal gastrectomy and retrocolic gastrojejunostomy(according to Billroth 2); for both, the postoperative course was uneventful. Histology showed multiple intramural and subserosal nodules with characteristic plexiform growth, featuring bland spindle cells situated in an abundant myxoid stroma with low mitotic activity. Immunohistochemistry showed α-smooth muscle actin-positive spindle cells, focal positivity for CD10, and negative staining for KIT, DOG1, CD34, S100, β-catenin, STAT-6 and anaplastic lymphoma kinase. One of the cases showed focal positivity for h-caldesmon and desmin. Upon followup, no sign of disease was found. In the differential diagnosis of plexiform fibromyxoma, it is important to exclude the more common gastrointestinal stromal tumors as they have greater potential for aggressivebehavior. Other lesions, like neuronal and vascular tumors, inflammatory fibroid polyps, abdominal desmoid-type fibromatosis, solitary fibrous tumors and smooth muscle tumors, must also be excluded.
文摘BACKGROUND Plexiform fibromyxoma(PF)is a rare mesenchymal tumor,with limited case reports worldwide.Common clinical symptoms are abdominal discomfort and bleeding signs,which frequently present slow-onset in reported cases.Herein,we report a case of gastric PF presenting as acute onset and with pyemia accompanying tumor rupture.We resected the tumor as well as the distal gastric,bulbus duodeni and gallbladder for treatment in emergency surgery.Notably,before the onset of the disease,the patient received coronavirus disease 2019(COVID-19)vaccines.CASE SUMMARY A 26-year-old man was admitted to our hospital,due to abdominal pain and fever after having received COVID-19 vaccines.Laboratory examination indicated severe sepsis.Computed tomography scan revealed a large mass in the abdomen.Deformation of the gastrointestinal tract was seen during gastroscopy.After failure of anti-infective treatment and symptoms of shock developed,he received an emergency surgery.We found a huge and partly ruptured mass,with thick purulence.Microscopically,the mass was composed of spindle cells with clarified cytoplasm,accompanied by myxoid stroma and arborizing blood vessels.Immunohistochemistry showed the tumor cells as positive for smooth muscle actin and succinate dehydrogenase subunit B but negative for DOG-1 and CD117.Finally,the patient was diagnosed with gastric PF and discharged from the hospital.CONCLUSION Gastric PF manifesting as tumor rupture combined with pyemia is rare.Timely surgery is critical for optimal prognosis.
文摘Superficial acral fibromyxoma (SAF) is a tumor that occurs on the distal phalanges of the digits. As it does not spontaneously regress and is often associated with pain, the primary treatment is surgical resection. It is often associated with the nail component of the affected digit, and thus cosmesis is an important goal of the operation. We herein describe a case of SAF on the distal phalanges of the fifth digit of the foot beneath the nail, which was successfully resected with the nail component kept intact. Moreover, although SAF is most commonly CD34-positive, the present case was CD34-negative except for endothelial cells within the tumor. While CD34-negative SAF has been previously reported, the current case further indicates that CD34-positivity is not essential for the diagnosis of SAF.
文摘Plexiform angiomyxoid myofibroblastic tumor of the stomach is a unique mesenchymal tumor that we first described in 2007.The tumor is very rare,and to date,only 18 cases confirmed by immunohistochemistry have been reported in the literature.The patients' ages ranged from 7 to 75 years(mean,43 years),and the male-to-female ratio was approximately 1:1.Representative clinical symptoms are ulceration,associated upper gastrointestinal bleeding(hematemesis),and anemia.The tumors are located at the antrum in all cases,and grossly,the tumor is whitish to brownish or reddish,and forms a lobulated submucosal or transmural mass.Microscopically,the tumor is characterized by a plexiform growth pattern,the proliferation of cytologically bland spindle cells,and a myxoid stroma that is rich in small vessels and positive for Alcian blue stain.Immunohistochemically,the tumor cells are positive for α-smooth muscle actin and negative for KIT and CD34.Differential diagnoses include gastrointestinal stromal tumor and other mesenchymal tumors of the gastrointestinal tract.Some authors proposed that this tumor should be designated as "plexiform fibromyxoma",but this designation might cause confusion.The tumor is probably benign and thus far,neither recurrence nor metastasis has been reported.
