Since the first description of intraductal papillary mucinous neoplasms(IPMNs)of the pancreas in the eighties,their identification has dramatically increased in the last decades,hand to hand with the improvements in d...Since the first description of intraductal papillary mucinous neoplasms(IPMNs)of the pancreas in the eighties,their identification has dramatically increased in the last decades,hand to hand with the improvements in diagnostic imaging and sampling techniques for the study of pancreatic diseases.However,the heterogeneity of IPMNs and their malignant potential make difficult the management of these lesions.The objective of this review is to identify the molecular characteristics of IPMNs in order to recognize potential markers for the discrimination of more aggressive IPMNs requiring surgical resection from benign IPMNs that could be observed.We briefly summarize recent research findings on the genetics and epigenetics of intraductal papillary mucinous neoplasms,identifying some genes,molecular mechanisms and cellular signaling pathways correlated to the pathogenesis of IPMNs and their progression to malignancy.The knowledge of molecular biology of IPMNs has impressively developed over the last few years.A great amount of genes functioning as oncogenes or tumor suppressor genes have been identified,in pancreatic juice or in blood or in the samples from the pancreatic resections,but further researches are required to use these informations for clinical intent,in order to better define the natural history of these diseases and to improve their management.展开更多
The BCR/ABL fusion gene or the Ph^1-chromosome in the t(9;22)(q34;q11)exerts a high tyrokinase acticity,which is the cause of chronic myeloid leukemia(CML).The1990 Hannover Bone Marrow Classification separated CML fro...The BCR/ABL fusion gene or the Ph^1-chromosome in the t(9;22)(q34;q11)exerts a high tyrokinase acticity,which is the cause of chronic myeloid leukemia(CML).The1990 Hannover Bone Marrow Classification separated CML from the myeloproliferative disorders essential thrombocythemia(ET),polycythemia vera(PV)and chronic megakaryocytic granulocytic myeloproliferation(CMGM).The 2006-2008 European Clinical Molecular and Pathological(ECMP)criteria discovered 3variants of thrombocythemia:ET with features of PV(prodromal PV),"true"ET and ET associated with CMGM.The 2008 World Health Organization(WHO)-ECMP and 2014 WHO-CMP classifications defined three phenotypes of JAK2^(V617F)mutated ET:normocellular ET(WHO-ET),hypercelluar ET due to increased erythropoiesis(prodromal PV)and ET with hypercellular megakaryocytic-granulocytic myeloproliferation.The JAK2^(V617F)mutation load in heterozygous WHO-ET is low and associated with normal life expectance.The hetero/homozygous JAK2^(V617F)mutation load in PV and myelofibrosis is related to myeloproliferative neoplasm(MPN)disease burden in terms of symptomaticsplenomegaly,constitutional symptoms,bone marrow hypercellularity and myelofibrosis.JAK2 exon 12mutated MPN presents as idiopathic eryhrocythemia and early stage PV.According to 2014 WHO-CMP criteria JAK2 wild type MPL^(515)mutated ET is the second distinct thrombocythemia featured by clustered giant megakaryocytes with hyperlobulated stag-horn-like nuclei,in a normocellular bone marrow consistent with the diagnosis of"true"ET.JAK2/MPL wild type,calreticulin mutated hypercellular ET appears to be the third distinct thrombocythemia characterized by clustered larged immature dysmorphic megakaryocytes and bulky(bulbous)hyperchromatic nuclei consistent with CMGM or primary megakaryocytic granulocytic myeloproliferation.展开更多
The Polycythemia Vera Study Group(PVSG),World Health Organization(WHO) and European Clinical,Molecular and Pathological(ECMP) classifications agree upon the diagnostic criteria for polycythemia vera(PV) and advanced p...The Polycythemia Vera Study Group(PVSG),World Health Organization(WHO) and European Clinical,Molecular and Pathological(ECMP) classifications agree upon the diagnostic criteria for polycythemia vera(PV) and advanced primary myelofibrosis(MF). Essential thrombocythemia(ET) according to PVSG and 2007/2008 WHO criteria comprises three variants of JAK2V617 F mutated ET when the ECMP criteria are applied. These include normocellular ET,hypercellular ET with features of early PV(prodromal PV),and hypercellular ET due to megakaryocytic,granulocytic myeloprolifera-tion(ET.MGM). Evolution of prodromal PV into overt PV is common. Development of MF is rare in normocellular ET(WHO-ET) but rather common in hypercellular ET.MGM. The JAK2V617 F mutation burden in heterozygous mutated normocellular ET and in heterozygous/homozygous or homozygous mutated PV and ET.MGM is of major prognostic significance. JAK2/MPL wild type ET associated with prefibrotic primary megakaryocytic and granulocytic myeloproliferation(PMGM) is characterized by densely clustered immature dysmorphic megakaryocytes with bulky(bulbous) hyperchromatic nuclei,which are never seen in JAK2V617 F mutated ET,and PV and also not in MPL515 mutated normocellular ET(WHO-ET). JAK2V617 mutation burden,spleen size,LDH,circulating CD34+ cells,and pre-treatment bone marrow histopathology are mandatory to stage the myeloproliferative neoplasms ET,PV,PMGM for proper prognosis assessment and therapeutic implications. MF itself is not a disease because reticulin fibrosis and reticulin/collagen fibrosis are secondary responses of activated polyclonal fibroblasts to cytokines released from the clonal myeloproliferative granulocytic and megakaryocytic progenitor cells in ET.MGM,PV and PMGM.展开更多
BACKGROUND Atypical spindle cell lipomatous tumor(ASLT)is a rare soft tissue neoplasm with a low potential for malignancy.ASLT frequently occurs in the limb and limb girdles.However,large retroperitoneal ASLTs are ext...BACKGROUND Atypical spindle cell lipomatous tumor(ASLT)is a rare soft tissue neoplasm with a low potential for malignancy.ASLT frequently occurs in the limb and limb girdles.However,large retroperitoneal ASLTs are extremely rare.There was no concrete case report of retroperitoneal ASLTs.CASE SUMMARY An 18-year-old woman presented with abdominal pain and a palpable mass.Abdominal computed tomography revealed a large fatty mass that was approximately 30 cm in size and filled the entire abdominal cavity.Surgical excision was indicated.The tumor did not invade the adjacent organs.The pelvic cavity was then too narrow to dissect smoothly.The mass was successfully excised without tumor rupture or adjacent organ injury.Microscopically,the neoplasm was a well-differentiated adipocytic neoplasm.Immunohistochemical staining showed that the spindle cells were positive for CD34 and desmin,in addition to multifocal positivity for S100 protein.These histological features were consistent with an ASLT.The patient’s postoperative course was uneventful.At the 12-mo followup,no evidence of recurrence or metastasis was observed.CONCLUSION To the best of our knowledge,our study is the first concrete report of a large retroperitoneal ASLT in the English literature.In the large retroperitoneal ASLT located in the pelvic cavity,which made it too narrow and tight to dissect,complete excision is difficult but very important because of recurrence risk.Although large retroperitoneal ASLTs are considered extremely rare,their detection is important for accurate evaluation and management.Owing to their significant rarity,retrospective multicenter case studies are required to determine the clinicopathologic characteristics.展开更多
BACKGROUND The majority of renal cell carcinomas are single lesions;unilateral synchronous multifocal renal carcinoma(USMRC)is rarely reported and poses a treatment challenge for urological oncologists.CASE SUMMARY A ...BACKGROUND The majority of renal cell carcinomas are single lesions;unilateral synchronous multifocal renal carcinoma(USMRC)is rarely reported and poses a treatment challenge for urological oncologists.CASE SUMMARY A 56-year-old man was hospitalized for pain and discomfort in the right kidney area for 6 d.Contrast-enhanced computed tomography demonstrated cT1a renal tumors at the lower pole of the right kidney and a cT1b renal tumor at the middle dorsal portion of the right kidney.The patient underwent retroperitoneal laparoscopic partial nephrectomy(RLPN).There were no complications peri-operatively.Histopathology revealed a low-grade,pathologic stage T1a(pT1a),clear cell renal cell carcinoma at the lower pole of the right kidney and a pT1b,chromophobe renal cell carcinoma at the middle dorsal portion of the right kidney.No tumor bed recurrence or metastasis was observed on imaging and his renal function remained stable during the 12-mo follow-up period.CONCLUSION RLPN is a safe,effective,and feasible for the management of USMRC,which can obtain equivalent oncological results with optimal renal function preservation.展开更多
Objective The aim of the study was to investigate the clinical manifestations,pathologic features,differentiation,and diagnosis of retroperitoneal paraganglioma.Methods The clinical manifestations,histopathologic morp...Objective The aim of the study was to investigate the clinical manifestations,pathologic features,differentiation,and diagnosis of retroperitoneal paraganglioma.Methods The clinical manifestations,histopathologic morphology,immunohistologic features,treatment,and prognosis were analyzed in 32 cases of retroperitoneal paraganglioma,and the literature was reviewed. Results Low back pain was the main symptom of retroperitoneal paraganglioma,and patients often presented with increased blood pressure. Blood vessels were abundant in the neoplasm of retroperitoneal paraganglioma,and pathologic diagnosis showed that the cells of tumors were rich in cytoplasm,and some were basophilic. Immunohistochemical study showed that Cg A,Syn,S-100,and Ki-67(2%–30%) were expressed,while Melan-A and CK were not. The patients were treated with surgical resection. Thirteen cases were followed up,and no recurrence or metastases have been observed.Conclusion The diagnosis of retroperitoneal paraganglioma should be confirmed by morphologic features and immunophenotype. Patients should be followed-up due to the potential for malignancy.Key words: retroperitoneal paraganglioma; immunohistochemistry;展开更多
Intraductal papillary mucinous neoplasm(IPMN) of the pancreas is a noninvasive epithelial neoplasm of mucinproducing cells arising in the main duct(MD) and/or branch ducts(BD) of the pancreas.Involved ducts are dilate...Intraductal papillary mucinous neoplasm(IPMN) of the pancreas is a noninvasive epithelial neoplasm of mucinproducing cells arising in the main duct(MD) and/or branch ducts(BD) of the pancreas.Involved ducts are dilated and filled with neoplastic papillae and mucus in variable intensity.IPMN lacks ovarian-type stroma,unlike mucinous cystic neoplasm,and is defined as a grossly visible entity(≥ 5 mm),unlike pancreatic intraepithelial neoplasm.With the use of high-resolution imaging techniques,very small IPMNs are increasingly being identified.Most IPMNs are solitary and located in the pancreatic head,although 20%-40% are multifocal.Macroscopic classification in MD type,BD type and mixed or combined type reflects biological differences with important prognostic and preoperative clinical management implications.Based on cytoarchitectural atypia,IPMN is classified into low-grade,intermediategrade and high-grade dysplasia.Based on histological features and mucin(MUC) immunophenotype,IPMNs are classified into gastric,intestinal,pancreatobiliary and oncocytic types.These different phenotypes can be observed together,with the IPMN classified according to the predominant type.Two pathways have been suggested:gastric phenotype corresponds to less aggressive uncommitted cells(MUC1-,MUC2-,MUC5 AC +,MUC6 +) with the capacity to evolve to intestinal phenotype(intestinal pathway)(MUC1-,MUC2 +,MUC5 AC +,MUC6- or weak +) or pancreatobiliary /oncocytic phenotypes(pyloropancreatic pathway)(MUC1 +,MUC 2-,MUC5 AC +,MUC 6 +) becoming more aggressive.Prognosis of IPMN is excellent but critically worsens when invasive carcinoma arises(about 40% of IPMNs),except in some cases of minimal invasion.The clinical challenge is to establish which IPMNs should be removed because of their higher risk of developing invasive cancer.Once resected,they must be extensively sampled or,much better,submitted in its entirety for microscopic study to completely rule out associated invasive carcinoma.展开更多
Intraductal papillary-mucinous neoplasm(IPMN) of the pancreas is a clinically and morphologically distinctive precursor lesion of pancreatic cancer,characterized by gradual progression through a sequence of neoplastic...Intraductal papillary-mucinous neoplasm(IPMN) of the pancreas is a clinically and morphologically distinctive precursor lesion of pancreatic cancer,characterized by gradual progression through a sequence of neoplastic changes.Based on the nature of the constituting neoplastic epithelium,degree of dysplasia and location within the pancreatic duct system,IPMNs are divided in several types which differ in their biological properties and clinical outcome.Molecular analysis and recent animal studies suggest that IPMNs develop in the context of a field-defect and reveal their possible relationship with other neoplastic precursor lesions of pancreatic cancer.展开更多
AIM: To analyze the surgical management of adult primary retroperitoneal tumors (APRT) and the factors influencing the outcome after operation. METHODS: Data of 143 cases of APRT from 1990 to 2003 in the First Affilia...AIM: To analyze the surgical management of adult primary retroperitoneal tumors (APRT) and the factors influencing the outcome after operation. METHODS: Data of 143 cases of APRT from 1990 to 2003 in the First Affiliated Hospital of China Medical University were evaluated retrospectively. RESULTS: A total of 143 cases of APRT were treated surgically. Among them, 122 (85.3%) underwent complete resection, 16 (11.2%) incomplete resection, and 3 (3%) surgical biopsies. Twenty-nine (20.2%) underwent tumor resection plus multiple organ resections. Ninety-five malignant cases were followed up for 1 mo to 5 years. The 1-year, 3-year, and 5-year survival rates of the patients subject to complete resection was 94.9%, 76.6% and 34.3% and that of patients with incomplete resection was 80.4%, 6.7%, and 0%, respectively (P < 0.001). The Cox multi-various regression analysis showed the completeness of tumor, sex and histological type were associated closely with local recurrence. CONCLUSION: Sufficient preoperative preparation and complete tumor resection play important roles in reducing recurrence and improving survival.展开更多
BACKGROUND Schwannomas are rare,often benign,tumors deriving from Schwann cells that have low incidence in the retroperitoneal region(0.5%to 5%).Their diagnosis is hardly confirmed at early stage due to lack of specif...BACKGROUND Schwannomas are rare,often benign,tumors deriving from Schwann cells that have low incidence in the retroperitoneal region(0.5%to 5%).Their diagnosis is hardly confirmed at early stage due to lack of specific features in signs,symptoms and imaging tests.Thus,it is necessary performing guided punch biopsy in order to identify it.Tumor removal is the treatment of choice due to its low sensitivity to radiation and chemotherapy.CASE SUMMARY Forty-seven years old female patient who was hospitalized for elective orthopedic procedure,without complications,evolved with severe pain in the lower limbs,thigh and gluteal region associated with neurogenic claudication.Persistent pain required magnetic resonance imaging(MRI)of the lumbar spine,which showed epidural collection with dural compression and massive heterogeneous perihepatic nodular lesion.The surgical team opted for draining the collection,as well as requested an MRI of the abdomen and pelvis,whose analysis showed expansive lesion in the retroperitoneum,in close contact with the inferior vena cava and with the right renal vein.A guided puncture was performed for diagnostic clarification,which showed immunohistochemical profile compatible with schwannoma.The selected treatment comprised retroperitoneum tumor resection in association with laparoscopic cholecystectomy.The patient was discharged and remained stable after 4-mo follow-up;she remains under followup to check the need of cancer investigations.CONCLUSION Schwannoma features requires surgical treatment based on laparoscopic resection,which often presents low relapse rates and optimistic prognosis.展开更多
BACKGROUND Retroperitoneal bronchogenic cyst(RBC)is an extremely rare developmental abnormality.Most are benign tumors but malignant transformation is possible.Because of their anatomical position,RBCs are easily misd...BACKGROUND Retroperitoneal bronchogenic cyst(RBC)is an extremely rare developmental abnormality.Most are benign tumors but malignant transformation is possible.Because of their anatomical position,RBCs are easily misdiagnosed as adrenal or pancreatic solid tumors on radiological evaluation.Here,we report a case of RBC,review the literature,and summarize some important features.CASE SUMMARY A 49-year-old woman was incidentally found to have a retroperitoneal tumor during a physical examination.Enhanced computed tomography and laboratory evaluations,including routine blood examination,blood biochemistry,24-h urine 17 ketones,17 hydroxyls,adrenocortical hormone,serum potassium concentration,serum amylase,lipase,and epithelial tumor markers,revealed a moderate density,54 mm×40 mm mass with a clear boundary near the left adrenal gland.The were no abnormalities in the blood and urine values.Because the patient had a history of hypertension and the location of the mass was adjacent to the adrenal gland,it was initially diagnosed as a left adrenal tumor and was resected by retroperitoneal laparoscopy.However,the pathological examination after surgery confirmed it to be a bronchogenic cyst.CONCLUSION Retroperitoneal laparoscopic surgery can be prioritized for symptomatic RBC patients.Conservative treatment is feasible for selected patients.展开更多
BACKGROUND Retroperitoneal sarcoma(RPS) is a rare malignancy arising from mesenchymal cells that most commonly presents as an abdominal mass and is associated with poor prognosis.Although several studies have assessed...BACKGROUND Retroperitoneal sarcoma(RPS) is a rare malignancy arising from mesenchymal cells that most commonly presents as an abdominal mass and is associated with poor prognosis.Although several studies have assessed the survival benefits of wide excision,few have reported detailed methods for achieving wide excision in patients with RPS.AIM To describe our experience with multidisciplinary surgical resection of RPS using intra-and extra-pelvic approaches.METHODS Multidisciplinary surgery is an anatomical approach that combines intra-and extra-peritoneal access within the same surgery to achieve complete RPS removal.This retrospective review of the records of patients who underwent multidisciplinary surgery for RPS analyzed surgical and survival outcomes.RESULTS Eight patients underwent 10 intra-and extra-pelvic surgical resections,and their median mass size was 12.75 cm(range,6-45.5 cm).Using an intrapelvic approach,laparoscopy-assisted surgery was performed in four cases and laparotomy surgery in six.Using an extrapelvic approach,ilioinguinal and posterior approaches were used in four cases each,and the prone position and midline skin incision were shared in one.All patients’ RPS masses were removed completely,and four achieved R0 resection through intra-and extra-pelvic surgery.The median estimated blood loss was 2000 m L(range,300-20000 m L) and the median hospitalization was 12.6 d(range,9-69 d).Reoperation was needed in two patients(one for wound necrosis and the other for bowel perforation and wound necrosis).The median overall survival rate and median progression-free survival were 64.6 and 13.7 mo,respectively.CONCLUSION RPS is therapeutically challenging because of its location and high risk of recurrence.Therefore,intra-and extra-pelvic surgical approaches can improve the macroscopic security of the surgical margin.展开更多
Objective Breast cancer is the most frequently diagnosed cancer in women. Accurate evaluation of the size and extent of the tumor is crucial in selecting a suitable surgical method for patients with breast cancer. Bot...Objective Breast cancer is the most frequently diagnosed cancer in women. Accurate evaluation of the size and extent of the tumor is crucial in selecting a suitable surgical method for patients with breast cancer. Both overestimation and underestimation have important adverse effects on patient care. This study aimed to evaluate the accuracy of breast magnetic resonance imaging(MRI) and ultrasound(US) examination for measuring the size and extent of early-stage breast neoplasms.Methods The longest diameter of breast tumors in patients with T_(1–2)N_(0–1)M_0 invasive breast cancer preparing for breast-conserving surgery(BCS) was measured preoperatively by using both MRI and US and their accuracy was compared with that of postoperative pathologic examination. If the diameter difference was within 2 mm, it was considered to be consistent with pathologic examination.Results A total of 36 patients were imaged using both MRI and US. The mean longest diameter of the tumors on MRI, US, and postoperative pathologic examination was 20.86 mm ± 4.09 mm(range: 11–27 mm), 16.14 mm ± 4.91 mm(range: 6–26 mm), and 18.36 mm ± 3.88 mm(range: 9–24 mm). US examination underestimated the size of the tumor compared to that determined using pathologic examination(t = 3.49, P < 0.01), while MRI overestimated it(t =-6.35, P < 0.01). The linear correlation coefficients between the image measurements and pathologic tumor size were r = 0.826(P < 0.01) for MRI and r = 0.645(P < 0.01) for US. The rate of consistency of MRI and US compared to that with pathologic examination was 88.89% and 80.65%, respectively, and there was no statistically significant difference between them(χ~2 = 0.80, P > 0.05).Conclusion MRI and US are both effective methods to assess the size of breast tumors, and they maintain good consistency with pathologic examination. MRI has a better correlation with pathology. However, we should be careful about the risk of inaccurate size estimation.展开更多
BACKGROUND Intraductal papillary mucinous neoplasm(IPMN)is a rare pancreatic tumor and has the potential to become malignant.Surgery is the most effective treatment at present,but there is no consensus on the site of ...BACKGROUND Intraductal papillary mucinous neoplasm(IPMN)is a rare pancreatic tumor and has the potential to become malignant.Surgery is the most effective treatment at present,but there is no consensus on the site of resection.Heterotopic pancreas occurs in the gastrointestinal tract,especially the stomach and duodenum but is asymptomatic and rare.We report a case of ectopic pancreas with IPMN located in the jejunum.CASE SUMMARY A 56-year-old male patient suffered from severe pain,nausea and vomiting due to a traffic accident and sought emergency treatment at our hospital.Contrast-enhanced computed tomography of the whole abdomen suggested splenic congestion,which was considered to be splenic rupture.Emergency laparotomy was performed,and the ruptured spleen was removed during the operation.Unexpectedly,a cauliflower-like mass of about 2.5 cm×2.5 cm in size was incidentally found about 80 cm from the ligament of Treitz during the operation.A partial small bowel resection was performed,and postoperative pathology confirmed the small bowel mass as heterotopic pancreas with low-grade IPMN.CONCLUSION Ectopic pancreas occurs in the jejunum and is pathologically confirmed as IPMN after surgical resection.展开更多
文摘Since the first description of intraductal papillary mucinous neoplasms(IPMNs)of the pancreas in the eighties,their identification has dramatically increased in the last decades,hand to hand with the improvements in diagnostic imaging and sampling techniques for the study of pancreatic diseases.However,the heterogeneity of IPMNs and their malignant potential make difficult the management of these lesions.The objective of this review is to identify the molecular characteristics of IPMNs in order to recognize potential markers for the discrimination of more aggressive IPMNs requiring surgical resection from benign IPMNs that could be observed.We briefly summarize recent research findings on the genetics and epigenetics of intraductal papillary mucinous neoplasms,identifying some genes,molecular mechanisms and cellular signaling pathways correlated to the pathogenesis of IPMNs and their progression to malignancy.The knowledge of molecular biology of IPMNs has impressively developed over the last few years.A great amount of genes functioning as oncogenes or tumor suppressor genes have been identified,in pancreatic juice or in blood or in the samples from the pancreatic resections,but further researches are required to use these informations for clinical intent,in order to better define the natural history of these diseases and to improve their management.
文摘The BCR/ABL fusion gene or the Ph^1-chromosome in the t(9;22)(q34;q11)exerts a high tyrokinase acticity,which is the cause of chronic myeloid leukemia(CML).The1990 Hannover Bone Marrow Classification separated CML from the myeloproliferative disorders essential thrombocythemia(ET),polycythemia vera(PV)and chronic megakaryocytic granulocytic myeloproliferation(CMGM).The 2006-2008 European Clinical Molecular and Pathological(ECMP)criteria discovered 3variants of thrombocythemia:ET with features of PV(prodromal PV),"true"ET and ET associated with CMGM.The 2008 World Health Organization(WHO)-ECMP and 2014 WHO-CMP classifications defined three phenotypes of JAK2^(V617F)mutated ET:normocellular ET(WHO-ET),hypercelluar ET due to increased erythropoiesis(prodromal PV)and ET with hypercellular megakaryocytic-granulocytic myeloproliferation.The JAK2^(V617F)mutation load in heterozygous WHO-ET is low and associated with normal life expectance.The hetero/homozygous JAK2^(V617F)mutation load in PV and myelofibrosis is related to myeloproliferative neoplasm(MPN)disease burden in terms of symptomaticsplenomegaly,constitutional symptoms,bone marrow hypercellularity and myelofibrosis.JAK2 exon 12mutated MPN presents as idiopathic eryhrocythemia and early stage PV.According to 2014 WHO-CMP criteria JAK2 wild type MPL^(515)mutated ET is the second distinct thrombocythemia featured by clustered giant megakaryocytes with hyperlobulated stag-horn-like nuclei,in a normocellular bone marrow consistent with the diagnosis of"true"ET.JAK2/MPL wild type,calreticulin mutated hypercellular ET appears to be the third distinct thrombocythemia characterized by clustered larged immature dysmorphic megakaryocytes and bulky(bulbous)hyperchromatic nuclei consistent with CMGM or primary megakaryocytic granulocytic myeloproliferation.
文摘The Polycythemia Vera Study Group(PVSG),World Health Organization(WHO) and European Clinical,Molecular and Pathological(ECMP) classifications agree upon the diagnostic criteria for polycythemia vera(PV) and advanced primary myelofibrosis(MF). Essential thrombocythemia(ET) according to PVSG and 2007/2008 WHO criteria comprises three variants of JAK2V617 F mutated ET when the ECMP criteria are applied. These include normocellular ET,hypercellular ET with features of early PV(prodromal PV),and hypercellular ET due to megakaryocytic,granulocytic myeloprolifera-tion(ET.MGM). Evolution of prodromal PV into overt PV is common. Development of MF is rare in normocellular ET(WHO-ET) but rather common in hypercellular ET.MGM. The JAK2V617 F mutation burden in heterozygous mutated normocellular ET and in heterozygous/homozygous or homozygous mutated PV and ET.MGM is of major prognostic significance. JAK2/MPL wild type ET associated with prefibrotic primary megakaryocytic and granulocytic myeloproliferation(PMGM) is characterized by densely clustered immature dysmorphic megakaryocytes with bulky(bulbous) hyperchromatic nuclei,which are never seen in JAK2V617 F mutated ET,and PV and also not in MPL515 mutated normocellular ET(WHO-ET). JAK2V617 mutation burden,spleen size,LDH,circulating CD34+ cells,and pre-treatment bone marrow histopathology are mandatory to stage the myeloproliferative neoplasms ET,PV,PMGM for proper prognosis assessment and therapeutic implications. MF itself is not a disease because reticulin fibrosis and reticulin/collagen fibrosis are secondary responses of activated polyclonal fibroblasts to cytokines released from the clonal myeloproliferative granulocytic and megakaryocytic progenitor cells in ET.MGM,PV and PMGM.
基金Supported by the 2016 Yeungnam University Research Grant
文摘BACKGROUND Atypical spindle cell lipomatous tumor(ASLT)is a rare soft tissue neoplasm with a low potential for malignancy.ASLT frequently occurs in the limb and limb girdles.However,large retroperitoneal ASLTs are extremely rare.There was no concrete case report of retroperitoneal ASLTs.CASE SUMMARY An 18-year-old woman presented with abdominal pain and a palpable mass.Abdominal computed tomography revealed a large fatty mass that was approximately 30 cm in size and filled the entire abdominal cavity.Surgical excision was indicated.The tumor did not invade the adjacent organs.The pelvic cavity was then too narrow to dissect smoothly.The mass was successfully excised without tumor rupture or adjacent organ injury.Microscopically,the neoplasm was a well-differentiated adipocytic neoplasm.Immunohistochemical staining showed that the spindle cells were positive for CD34 and desmin,in addition to multifocal positivity for S100 protein.These histological features were consistent with an ASLT.The patient’s postoperative course was uneventful.At the 12-mo followup,no evidence of recurrence or metastasis was observed.CONCLUSION To the best of our knowledge,our study is the first concrete report of a large retroperitoneal ASLT in the English literature.In the large retroperitoneal ASLT located in the pelvic cavity,which made it too narrow and tight to dissect,complete excision is difficult but very important because of recurrence risk.Although large retroperitoneal ASLTs are considered extremely rare,their detection is important for accurate evaluation and management.Owing to their significant rarity,retrospective multicenter case studies are required to determine the clinicopathologic characteristics.
基金Supported by the Incubation Project of Outstanding Young Scientist Fund of Sichuan Province,No.2019JDJQ0039the Key Research Foundation of Sichuan provincial health commission,No.19ZD015the Interdisciplinary Program of Shanghai Jiao Tong University,No.YG2021QN102.
文摘BACKGROUND The majority of renal cell carcinomas are single lesions;unilateral synchronous multifocal renal carcinoma(USMRC)is rarely reported and poses a treatment challenge for urological oncologists.CASE SUMMARY A 56-year-old man was hospitalized for pain and discomfort in the right kidney area for 6 d.Contrast-enhanced computed tomography demonstrated cT1a renal tumors at the lower pole of the right kidney and a cT1b renal tumor at the middle dorsal portion of the right kidney.The patient underwent retroperitoneal laparoscopic partial nephrectomy(RLPN).There were no complications peri-operatively.Histopathology revealed a low-grade,pathologic stage T1a(pT1a),clear cell renal cell carcinoma at the lower pole of the right kidney and a pT1b,chromophobe renal cell carcinoma at the middle dorsal portion of the right kidney.No tumor bed recurrence or metastasis was observed on imaging and his renal function remained stable during the 12-mo follow-up period.CONCLUSION RLPN is a safe,effective,and feasible for the management of USMRC,which can obtain equivalent oncological results with optimal renal function preservation.
文摘Objective The aim of the study was to investigate the clinical manifestations,pathologic features,differentiation,and diagnosis of retroperitoneal paraganglioma.Methods The clinical manifestations,histopathologic morphology,immunohistologic features,treatment,and prognosis were analyzed in 32 cases of retroperitoneal paraganglioma,and the literature was reviewed. Results Low back pain was the main symptom of retroperitoneal paraganglioma,and patients often presented with increased blood pressure. Blood vessels were abundant in the neoplasm of retroperitoneal paraganglioma,and pathologic diagnosis showed that the cells of tumors were rich in cytoplasm,and some were basophilic. Immunohistochemical study showed that Cg A,Syn,S-100,and Ki-67(2%–30%) were expressed,while Melan-A and CK were not. The patients were treated with surgical resection. Thirteen cases were followed up,and no recurrence or metastases have been observed.Conclusion The diagnosis of retroperitoneal paraganglioma should be confirmed by morphologic features and immunophenotype. Patients should be followed-up due to the potential for malignancy.Key words: retroperitoneal paraganglioma; immunohistochemistry;
文摘Intraductal papillary mucinous neoplasm(IPMN) of the pancreas is a noninvasive epithelial neoplasm of mucinproducing cells arising in the main duct(MD) and/or branch ducts(BD) of the pancreas.Involved ducts are dilated and filled with neoplastic papillae and mucus in variable intensity.IPMN lacks ovarian-type stroma,unlike mucinous cystic neoplasm,and is defined as a grossly visible entity(≥ 5 mm),unlike pancreatic intraepithelial neoplasm.With the use of high-resolution imaging techniques,very small IPMNs are increasingly being identified.Most IPMNs are solitary and located in the pancreatic head,although 20%-40% are multifocal.Macroscopic classification in MD type,BD type and mixed or combined type reflects biological differences with important prognostic and preoperative clinical management implications.Based on cytoarchitectural atypia,IPMN is classified into low-grade,intermediategrade and high-grade dysplasia.Based on histological features and mucin(MUC) immunophenotype,IPMNs are classified into gastric,intestinal,pancreatobiliary and oncocytic types.These different phenotypes can be observed together,with the IPMN classified according to the predominant type.Two pathways have been suggested:gastric phenotype corresponds to less aggressive uncommitted cells(MUC1-,MUC2-,MUC5 AC +,MUC6 +) with the capacity to evolve to intestinal phenotype(intestinal pathway)(MUC1-,MUC2 +,MUC5 AC +,MUC6- or weak +) or pancreatobiliary /oncocytic phenotypes(pyloropancreatic pathway)(MUC1 +,MUC 2-,MUC5 AC +,MUC 6 +) becoming more aggressive.Prognosis of IPMN is excellent but critically worsens when invasive carcinoma arises(about 40% of IPMNs),except in some cases of minimal invasion.The clinical challenge is to establish which IPMNs should be removed because of their higher risk of developing invasive cancer.Once resected,they must be extensively sampled or,much better,submitted in its entirety for microscopic study to completely rule out associated invasive carcinoma.
文摘Intraductal papillary-mucinous neoplasm(IPMN) of the pancreas is a clinically and morphologically distinctive precursor lesion of pancreatic cancer,characterized by gradual progression through a sequence of neoplastic changes.Based on the nature of the constituting neoplastic epithelium,degree of dysplasia and location within the pancreatic duct system,IPMNs are divided in several types which differ in their biological properties and clinical outcome.Molecular analysis and recent animal studies suggest that IPMNs develop in the context of a field-defect and reveal their possible relationship with other neoplastic precursor lesions of pancreatic cancer.
文摘AIM: To analyze the surgical management of adult primary retroperitoneal tumors (APRT) and the factors influencing the outcome after operation. METHODS: Data of 143 cases of APRT from 1990 to 2003 in the First Affiliated Hospital of China Medical University were evaluated retrospectively. RESULTS: A total of 143 cases of APRT were treated surgically. Among them, 122 (85.3%) underwent complete resection, 16 (11.2%) incomplete resection, and 3 (3%) surgical biopsies. Twenty-nine (20.2%) underwent tumor resection plus multiple organ resections. Ninety-five malignant cases were followed up for 1 mo to 5 years. The 1-year, 3-year, and 5-year survival rates of the patients subject to complete resection was 94.9%, 76.6% and 34.3% and that of patients with incomplete resection was 80.4%, 6.7%, and 0%, respectively (P < 0.001). The Cox multi-various regression analysis showed the completeness of tumor, sex and histological type were associated closely with local recurrence. CONCLUSION: Sufficient preoperative preparation and complete tumor resection play important roles in reducing recurrence and improving survival.
文摘BACKGROUND Schwannomas are rare,often benign,tumors deriving from Schwann cells that have low incidence in the retroperitoneal region(0.5%to 5%).Their diagnosis is hardly confirmed at early stage due to lack of specific features in signs,symptoms and imaging tests.Thus,it is necessary performing guided punch biopsy in order to identify it.Tumor removal is the treatment of choice due to its low sensitivity to radiation and chemotherapy.CASE SUMMARY Forty-seven years old female patient who was hospitalized for elective orthopedic procedure,without complications,evolved with severe pain in the lower limbs,thigh and gluteal region associated with neurogenic claudication.Persistent pain required magnetic resonance imaging(MRI)of the lumbar spine,which showed epidural collection with dural compression and massive heterogeneous perihepatic nodular lesion.The surgical team opted for draining the collection,as well as requested an MRI of the abdomen and pelvis,whose analysis showed expansive lesion in the retroperitoneum,in close contact with the inferior vena cava and with the right renal vein.A guided puncture was performed for diagnostic clarification,which showed immunohistochemical profile compatible with schwannoma.The selected treatment comprised retroperitoneum tumor resection in association with laparoscopic cholecystectomy.The patient was discharged and remained stable after 4-mo follow-up;she remains under followup to check the need of cancer investigations.CONCLUSION Schwannoma features requires surgical treatment based on laparoscopic resection,which often presents low relapse rates and optimistic prognosis.
文摘BACKGROUND Retroperitoneal bronchogenic cyst(RBC)is an extremely rare developmental abnormality.Most are benign tumors but malignant transformation is possible.Because of their anatomical position,RBCs are easily misdiagnosed as adrenal or pancreatic solid tumors on radiological evaluation.Here,we report a case of RBC,review the literature,and summarize some important features.CASE SUMMARY A 49-year-old woman was incidentally found to have a retroperitoneal tumor during a physical examination.Enhanced computed tomography and laboratory evaluations,including routine blood examination,blood biochemistry,24-h urine 17 ketones,17 hydroxyls,adrenocortical hormone,serum potassium concentration,serum amylase,lipase,and epithelial tumor markers,revealed a moderate density,54 mm×40 mm mass with a clear boundary near the left adrenal gland.The were no abnormalities in the blood and urine values.Because the patient had a history of hypertension and the location of the mass was adjacent to the adrenal gland,it was initially diagnosed as a left adrenal tumor and was resected by retroperitoneal laparoscopy.However,the pathological examination after surgery confirmed it to be a bronchogenic cyst.CONCLUSION Retroperitoneal laparoscopic surgery can be prioritized for symptomatic RBC patients.Conservative treatment is feasible for selected patients.
文摘BACKGROUND Retroperitoneal sarcoma(RPS) is a rare malignancy arising from mesenchymal cells that most commonly presents as an abdominal mass and is associated with poor prognosis.Although several studies have assessed the survival benefits of wide excision,few have reported detailed methods for achieving wide excision in patients with RPS.AIM To describe our experience with multidisciplinary surgical resection of RPS using intra-and extra-pelvic approaches.METHODS Multidisciplinary surgery is an anatomical approach that combines intra-and extra-peritoneal access within the same surgery to achieve complete RPS removal.This retrospective review of the records of patients who underwent multidisciplinary surgery for RPS analyzed surgical and survival outcomes.RESULTS Eight patients underwent 10 intra-and extra-pelvic surgical resections,and their median mass size was 12.75 cm(range,6-45.5 cm).Using an intrapelvic approach,laparoscopy-assisted surgery was performed in four cases and laparotomy surgery in six.Using an extrapelvic approach,ilioinguinal and posterior approaches were used in four cases each,and the prone position and midline skin incision were shared in one.All patients’ RPS masses were removed completely,and four achieved R0 resection through intra-and extra-pelvic surgery.The median estimated blood loss was 2000 m L(range,300-20000 m L) and the median hospitalization was 12.6 d(range,9-69 d).Reoperation was needed in two patients(one for wound necrosis and the other for bowel perforation and wound necrosis).The median overall survival rate and median progression-free survival were 64.6 and 13.7 mo,respectively.CONCLUSION RPS is therapeutically challenging because of its location and high risk of recurrence.Therefore,intra-and extra-pelvic surgical approaches can improve the macroscopic security of the surgical margin.
文摘Objective Breast cancer is the most frequently diagnosed cancer in women. Accurate evaluation of the size and extent of the tumor is crucial in selecting a suitable surgical method for patients with breast cancer. Both overestimation and underestimation have important adverse effects on patient care. This study aimed to evaluate the accuracy of breast magnetic resonance imaging(MRI) and ultrasound(US) examination for measuring the size and extent of early-stage breast neoplasms.Methods The longest diameter of breast tumors in patients with T_(1–2)N_(0–1)M_0 invasive breast cancer preparing for breast-conserving surgery(BCS) was measured preoperatively by using both MRI and US and their accuracy was compared with that of postoperative pathologic examination. If the diameter difference was within 2 mm, it was considered to be consistent with pathologic examination.Results A total of 36 patients were imaged using both MRI and US. The mean longest diameter of the tumors on MRI, US, and postoperative pathologic examination was 20.86 mm ± 4.09 mm(range: 11–27 mm), 16.14 mm ± 4.91 mm(range: 6–26 mm), and 18.36 mm ± 3.88 mm(range: 9–24 mm). US examination underestimated the size of the tumor compared to that determined using pathologic examination(t = 3.49, P < 0.01), while MRI overestimated it(t =-6.35, P < 0.01). The linear correlation coefficients between the image measurements and pathologic tumor size were r = 0.826(P < 0.01) for MRI and r = 0.645(P < 0.01) for US. The rate of consistency of MRI and US compared to that with pathologic examination was 88.89% and 80.65%, respectively, and there was no statistically significant difference between them(χ~2 = 0.80, P > 0.05).Conclusion MRI and US are both effective methods to assess the size of breast tumors, and they maintain good consistency with pathologic examination. MRI has a better correlation with pathology. However, we should be careful about the risk of inaccurate size estimation.
文摘BACKGROUND Intraductal papillary mucinous neoplasm(IPMN)is a rare pancreatic tumor and has the potential to become malignant.Surgery is the most effective treatment at present,but there is no consensus on the site of resection.Heterotopic pancreas occurs in the gastrointestinal tract,especially the stomach and duodenum but is asymptomatic and rare.We report a case of ectopic pancreas with IPMN located in the jejunum.CASE SUMMARY A 56-year-old male patient suffered from severe pain,nausea and vomiting due to a traffic accident and sought emergency treatment at our hospital.Contrast-enhanced computed tomography of the whole abdomen suggested splenic congestion,which was considered to be splenic rupture.Emergency laparotomy was performed,and the ruptured spleen was removed during the operation.Unexpectedly,a cauliflower-like mass of about 2.5 cm×2.5 cm in size was incidentally found about 80 cm from the ligament of Treitz during the operation.A partial small bowel resection was performed,and postoperative pathology confirmed the small bowel mass as heterotopic pancreas with low-grade IPMN.CONCLUSION Ectopic pancreas occurs in the jejunum and is pathologically confirmed as IPMN after surgical resection.