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
We report three rare cases of sclerosing angiomatoid nodular transformation(SANT) in the spleen.We compared the conventional and contrast-enhanced ultrasonographic appearance.The conventional sonographic examinations ...We report three rare cases of sclerosing angiomatoid nodular transformation(SANT) in the spleen.We compared the conventional and contrast-enhanced ultrasonographic appearance.The conventional sonographic examinations exhibited solitary lesions without common respects,while contrast-enhanced ultrasonography(CEUS) revealed nodular appearance mimicking its pathologic characteristics.It suggests that CEUS can provide morphologic information for diagnosing SANT.展开更多
BACKGROUND Sclerosing angiomatoid nodular transformation(SANT)is a rare benign disease of the spleen with unknown origin.Clinical symptoms are inhomogeneous,and suspicious splenic lesion often found incidentally,leadi...BACKGROUND Sclerosing angiomatoid nodular transformation(SANT)is a rare benign disease of the spleen with unknown origin.Clinical symptoms are inhomogeneous,and suspicious splenic lesion often found incidentally,leading to splenectomy,as malignancy cannot securely be ruled out.Diagnosis is made histologically after resection.CASE SUMMARY Two cases of German,white,non-smoking,and non-drinking patients of normal weight are presented.The first one is a 26-year-old man without medical history who was exhibiting an undesired weight loss of 10 kg and recurring vomiting for about 18 mo.The second one is a 65-year-old woman with hypertension who had previously undergone gynecological surgery,suffering from a lasting feeling of abdominal fullness.Both showed radiologically an inhomogeneous splenic lesion leading to splenectomy approximately 6 and 9 wk after surgical presentation.Both diagnoses of SANT were made histologically.Follow-up went well,and both were treated according to the recommendation for asplenic patients.CONCLUSION SANT is a rare cause of splenectomy and an incidental histological finding.Further research should focus on clinical and radiological diagnosis of SANT as well as on treatment of patients with asymptomatic and small findings.展开更多
BACKGROUND Sclerosing angiomatoid nodular transformation(SANT)is a rare disease of the spleen.It has unique pathological features and mimics splenic tumor on radiological imaging.CASE SUMMARY A 47-year-old woman was i...BACKGROUND Sclerosing angiomatoid nodular transformation(SANT)is a rare disease of the spleen.It has unique pathological features and mimics splenic tumor on radiological imaging.CASE SUMMARY A 47-year-old woman was incidentally found to have a splenic mass on abdominal ultrasound.She had a 10-cm postoperative scar in the lower abdomen due to previous cesarean sections.The patient had a past history of anemia of unknown etiology for 20 years.The patient underwent laparoscopic splenectomy.The postoperative course was uneventful,with a hospital stay of 7 d.The histopathological examination of the spleen revealed SANT.At the 6-mo followup,the patient remained disease-free.CONCLUSION SANT is a rare benign disease mimicking a malignant tumor.A definitive diagnosis can be made only on histopathology.展开更多
BACKGROUND Many clinicians and surgeons are unfamiliar with the sclerosing angiomatoid nodular transformation(SANT),which is gaining recognition as a benign splenic tumor.We challenge that SANT is rare and whether sur...BACKGROUND Many clinicians and surgeons are unfamiliar with the sclerosing angiomatoid nodular transformation(SANT),which is gaining recognition as a benign splenic tumor.We challenge that SANT is rare and whether surgical intervention could be avoided through critical imaging review.AIM To evaluate the incidence of SANT among splenic tumors and the decisionmaking process of SANT management.METHODS Twenty hospitalized patients who underwent splenectomy in 2018 and 2019 in a tertiary university hospital were retrospectively reviewed,and their data on imaging,diagnosis,surgical indications,and courses were recorded.All pathology results were confirmed by pathologist.Discriminative features differentiating SANT from other non-SANT splenic tumors were descriptively analyzed in this case series.RESULTS Fourteen out of 20 patients who underwent splenectomy had splenic tumors,including 3 SANTs(21%splenic tumors),6 non-SANT benign lesions(43%),2 metastatic tumors,and 3 lymphomas.Hypointensity on T2-weighted magnetic resonance imaging(MRI),spoke wheel enhancing pattern in contrasted computed tomography or MRI,and cold spot(low fluorodeoxyglucose uptake)in positron emission tomography(PET)scan helped establish the diagnosis of SANT.Lymphoma,presenting with a hot spot on the PET scan were differentiated from SANT.Surgical indications were reformatted for splenic tumors.Splenectomy need not be performed in patients with typical imaging features of SANT.CONCLUSION SANT is not a rare disease entity in clinical practice.Splenectomy should not be routinely indicated as the only management option for SANT with typical imaging features.展开更多
Sclerosing angiomatoid nodular transformation （SANT） of the spleen, a newly defined primary lesion of the spleen, is very rare. Immunohistochemistry is the only way to confirm the diagnosis of SANT. We present the c...Sclerosing angiomatoid nodular transformation （SANT） of the spleen, a newly defined primary lesion of the spleen, is very rare. Immunohistochemistry is the only way to confirm the diagnosis of SANT. We present the clinical characteristics and postoperative outcomes of five SANT cases that underwent splenectomy from January 2007 to October 2010. Although SANT had specific imaging findings, differential diagnosis from other splenic tuomrs or malignant lesions preoperatively was difficult. The hand-assisted laparoscopic splenectomy was a useful and effective technique for the management and postoperative diagnosis of SANT. All SANT patients had good prognosis without recurrence after splenectomy.展开更多
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We report three rare cases of sclerosing angiomatoid nodular transformation(SANT) in the spleen.We compared the conventional and contrast-enhanced ultrasonographic appearance.The conventional sonographic examinations exhibited solitary lesions without common respects,while contrast-enhanced ultrasonography(CEUS) revealed nodular appearance mimicking its pathologic characteristics.It suggests that CEUS can provide morphologic information for diagnosing SANT.
文摘BACKGROUND Sclerosing angiomatoid nodular transformation(SANT)is a rare benign disease of the spleen with unknown origin.Clinical symptoms are inhomogeneous,and suspicious splenic lesion often found incidentally,leading to splenectomy,as malignancy cannot securely be ruled out.Diagnosis is made histologically after resection.CASE SUMMARY Two cases of German,white,non-smoking,and non-drinking patients of normal weight are presented.The first one is a 26-year-old man without medical history who was exhibiting an undesired weight loss of 10 kg and recurring vomiting for about 18 mo.The second one is a 65-year-old woman with hypertension who had previously undergone gynecological surgery,suffering from a lasting feeling of abdominal fullness.Both showed radiologically an inhomogeneous splenic lesion leading to splenectomy approximately 6 and 9 wk after surgical presentation.Both diagnoses of SANT were made histologically.Follow-up went well,and both were treated according to the recommendation for asplenic patients.CONCLUSION SANT is a rare cause of splenectomy and an incidental histological finding.Further research should focus on clinical and radiological diagnosis of SANT as well as on treatment of patients with asymptomatic and small findings.
文摘BACKGROUND Sclerosing angiomatoid nodular transformation(SANT)is a rare disease of the spleen.It has unique pathological features and mimics splenic tumor on radiological imaging.CASE SUMMARY A 47-year-old woman was incidentally found to have a splenic mass on abdominal ultrasound.She had a 10-cm postoperative scar in the lower abdomen due to previous cesarean sections.The patient had a past history of anemia of unknown etiology for 20 years.The patient underwent laparoscopic splenectomy.The postoperative course was uneventful,with a hospital stay of 7 d.The histopathological examination of the spleen revealed SANT.At the 6-mo followup,the patient remained disease-free.CONCLUSION SANT is a rare benign disease mimicking a malignant tumor.A definitive diagnosis can be made only on histopathology.
文摘BACKGROUND Many clinicians and surgeons are unfamiliar with the sclerosing angiomatoid nodular transformation(SANT),which is gaining recognition as a benign splenic tumor.We challenge that SANT is rare and whether surgical intervention could be avoided through critical imaging review.AIM To evaluate the incidence of SANT among splenic tumors and the decisionmaking process of SANT management.METHODS Twenty hospitalized patients who underwent splenectomy in 2018 and 2019 in a tertiary university hospital were retrospectively reviewed,and their data on imaging,diagnosis,surgical indications,and courses were recorded.All pathology results were confirmed by pathologist.Discriminative features differentiating SANT from other non-SANT splenic tumors were descriptively analyzed in this case series.RESULTS Fourteen out of 20 patients who underwent splenectomy had splenic tumors,including 3 SANTs(21%splenic tumors),6 non-SANT benign lesions(43%),2 metastatic tumors,and 3 lymphomas.Hypointensity on T2-weighted magnetic resonance imaging(MRI),spoke wheel enhancing pattern in contrasted computed tomography or MRI,and cold spot(low fluorodeoxyglucose uptake)in positron emission tomography(PET)scan helped establish the diagnosis of SANT.Lymphoma,presenting with a hot spot on the PET scan were differentiated from SANT.Surgical indications were reformatted for splenic tumors.Splenectomy need not be performed in patients with typical imaging features of SANT.CONCLUSION SANT is not a rare disease entity in clinical practice.Splenectomy should not be routinely indicated as the only management option for SANT with typical imaging features.
文摘Sclerosing angiomatoid nodular transformation （SANT） of the spleen, a newly defined primary lesion of the spleen, is very rare. Immunohistochemistry is the only way to confirm the diagnosis of SANT. We present the clinical characteristics and postoperative outcomes of five SANT cases that underwent splenectomy from January 2007 to October 2010. Although SANT had specific imaging findings, differential diagnosis from other splenic tuomrs or malignant lesions preoperatively was difficult. The hand-assisted laparoscopic splenectomy was a useful and effective technique for the management and postoperative diagnosis of SANT. All SANT patients had good prognosis without recurrence after splenectomy.
