Angiosarcoma of bone is an exceedingly rare primary bone malignancy that can present as an aggressive osteolytic lesion. This subset can radiologically mimic non-vascular neoplasms and impose serious challenges in rea...Angiosarcoma of bone is an exceedingly rare primary bone malignancy that can present as an aggressive osteolytic lesion. This subset can radiologically mimic non-vascular neoplasms and impose serious challenges in reaching the correct diagnosis. Meanwhile histological diagnosis can be extremely challenging too, as the pathological features often resemble that of aneurysmal bone cysts. We present an unusual case of a 22-year-old woman who presented with a rapidly growing humeral tumor of 8 months’ duration. The case of intraosseous angiosarcoma presented as a diagnostic dilemma and the relevant radiological and pathologic findings were discussed. We describe the clinical, radiological and pathological features of this unique case, and review the literature concerning Angiosarcoma of bone. Our case highlights the diagnostic difficulties for such very rare tumours and clinico-pathological correlation is of paramount importance to differential diagnosis.展开更多
Objective： To evaluate the functional outcome and complications of allograft replacement in management of bone tumors. Methods： Between March 1992 and September 2002, 164 patients underwent bone tumor resection and ...Objective： To evaluate the functional outcome and complications of allograft replacement in management of bone tumors. Methods： Between March 1992 and September 2002, 164 patients underwent bone tumor resection and massive allograft reconstruction of bone defects. The length of the resected part ranged from 5-35 cm. The resections were classified as marginal or wide resections of the tumor on the basis of the Musculoskeletal Tumor Society staging system. Fresh-frozen allografts were employed as osteoarticular grafts （n = 95）, hemi-condylar （n = 15）, massive （n = 23）, allograft-prosthesis composite （n = 12）, intercalary grafts （n = 15） or hemi-pelvic grafts （n = 4）. Most of the lesions were osteosarcoma and giant cell tumor of bone and located in proximal and distal femur, proximal tibia and humerus. Results： At a median follow-up of 47 months （range, 12 to 168 months） after the operation, 154 of the patients in the study were free of disease and 10 died of disease. Twenty-one （12.8%） patients had local recurrence and 38 （23.2%） nonunion. Late complications included 11 （6.7%） fractures of the allograft and 18 （11.0%） infections of the graft, instability of the joint in the form of subluxation was noted in 13 （7.9%） patients. Ten extremities were amputated due to local recurrence or severe infection. Conclusion： AIIografts can be used for reconstruction of bony defects after tumor resection. AIIograft has nearly similar shape, strength, osteo-inductivity and osteo-conductivity with host bone. AIIograft implantation is a high complication reconstruction method, and the dsk of recurrence increases when less surgical margin achieves.展开更多
Among plasma cell disorders, solitary plasmacytoma (solitany-plasmacytoma of bone, SPB and extramedullary plasmacytoma, EMP) is rare as compared with mulitiple myeloma (MM). Furthermore.the relationship between solita...Among plasma cell disorders, solitary plasmacytoma (solitany-plasmacytoma of bone, SPB and extramedullary plasmacytoma, EMP) is rare as compared with mulitiple myeloma (MM). Furthermore.the relationship between solitary plasmacytoma and MM remains unclear.Between 1960 and 1994, 24 patients with SPB and 20 with EMP were treated. The criteria for diagonosis were: (1) No evidence of other lesions based on clinical and radiologic examinations;(2) Biopsy evidence of a plasma cell neoplasm; (3) Bone marrow biopsy specimen with negative findings (less than 10% plasma cell); (4) No anemia, hypercalcemia or renal involvement. The average follow-up period was 112 months (from 6 to 360 months). Fifty-four percent of patients with SPB and 40% of patients with EMP developed MM, however, there was no significant statistical difference between SPB and EMP (P <0.05).We suggested that solitary plasmacytomas be classified as two types, latent and aggressive. The former was histologically well-differentiated plasmacytomas. The latter was poorly differentiated tumors which easily progress to MM. The treatment of choice is wide excision or thorough curettage, by cryogenic necrosis with liquid nitrogen or cautery of the bony wall with phenol and the cavity filled with bone grafts or cement. All patients with apparently isolated plasmacytoma should he given if the tumor turns out to be poorly differentiated, in order to delay their progression to MM.展开更多
Bone metastasis is a rare event in patients with gastric cancer, but pathologic fracture, paralysis, pain and hematological disorders associated with the bone metastasis may influence the quality of life. We report he...Bone metastasis is a rare event in patients with gastric cancer, but pathologic fracture, paralysis, pain and hematological disorders associated with the bone metastasis may influence the quality of life. We report herein the case of a 53-year-old man who presented with primary remnant gastric cancer with bone metastasis. The patient requested further investigations after detection of a metastatic lesion in the 2 nd lumbar vertebra during evaluation for back pain that had persisted for 3 mo. No other metastatic lesions were detected. He underwent total gastrectomy and palliative metastasectomy to aid in reduction of symptoms, and he received combination chemotherapy with tegafur(S-1) and cisplatin. The patient survived for about 60 mo after surgery. Currently, there is no treatment guideline for gastric cancer with bone metastasis, and we believe that gastrectomy plus metastasectomy may be an effective therapeutic option for improving qualityof life and survival in patients with resectable primary gastric cancer and bone metastasis.展开更多
Objective ：To evaluate the sensitivity of serum tartrate-resistant acid phosphatase 5b（Tracp5b） activity in monitoring bisphosphonate treatment results of bone metastasis in breast cancer（BC） patients. Methods：T...Objective ：To evaluate the sensitivity of serum tartrate-resistant acid phosphatase 5b（Tracp5b） activity in monitoring bisphosphonate treatment results of bone metastasis in breast cancer（BC） patients. Methods：The serum activities of Tracp5b, CEA, CA153 were measured in 58 BC patients, including 26 without bone metastasis, 32 with bone metastasis. The serum activities of TracpSb, CEA, CA153 were also measured in 19 patients with bone metastasis after 3 months of bisphosphonate treatment. Eighteen healthy women with age from 34 to 70 served as control. Results：Serum TracpSb was significantly elevated in patients with bone metastasis compared with that in all any other groups（P〈 0.05）. The sensitivity of TracpSb was 78.13% and the specificity was 86.36%. The sensitivity of CA153 was 37.50% and the specificity was 77.27%. The sensitivity of CEA was 21.88% and the specificity was 84.09%. The serum activity of TracpSb decreased significantly（P 〈 0.05） after 3 months of bisphosphonate treatment, while the levels of CA153 and CEA were unchanged. Conclusion：Serum Tracp5b activity is a useful diagnostic marker for bone metastasis in BC patients and can be used to evaluate the treatment results of bisphosphonate.展开更多
A rational analysis procedure for solitary lesions on whole bone scan-ning was offered. This study was undertaken to analyze retrospectively solitary le-sions which obtained final diagnose through the following aspect...A rational analysis procedure for solitary lesions on whole bone scan-ning was offered. This study was undertaken to analyze retrospectively solitary le-sions which obtained final diagnose through the following aspects: (1) diagnosis ofbone metastasis, (2) the incidence of bone metastasis in different tumor, (3) the mostpossible lesion sites indicating bone metastasis, (4) morphological analysis of solitarylesions. The results are: (1) The incidence of solitary lesions in 2465 cases on wholebone scanning is 15.3%. (2) The rate of bone metastasis is 24.8% in 282 patientswith primary malignancy. The rate of bone metastasis is 6.3% in 64 patients withoutprimary malignancy, and the total diagnostic rate of bone metastasis is 21.4% in 346patients. (3) In patients with primary malignancy, the incidence of bone metastasis ofsolitary lesions is as follows respectively: bronchi cancer 36.1%(22/61); breast cancer23.8%(20/84); prostate gland 17.2%(5/29); other urinary system cancer 22.2%(4/18):G.I. system cancer 16.9%(10/59); others 29.0%(9/31). There is no significant differ-ence in different cancer. (4) In patients without primary malignancy, 93.7%(60/64) ofsolitary lesions are benign. (5) From anatomical point of view, we found the diagnos-tic rate of bone metastasis is as follow: 30% in spine; 34.2% in pelvis; 36.4% in skull;10.8% in other bones. There are significant differences in four groups. It is concludedthat: (1) The diagnostic rate of bone metastasis in solitary lesions is 21.4%. (2) Themost possible solitary lesions indicating osseous tumor spread are at spine, pelvic andskull. (3) Special attention to "cold" and streak like lesions should be paid. (4) Aclinical analysis procedure for diagnosis of solitary lesions has been summarized outhere.展开更多
Objective： To investigate the risk factors of bone metastases in breast carcinoma. Methods： By cross sectional study, the data of 225 breast cancer patients who were inpatients in four hospitals in Hangzhou were ana...Objective： To investigate the risk factors of bone metastases in breast carcinoma. Methods： By cross sectional study, the data of 225 breast cancer patients who were inpatients in four hospitals in Hangzhou were analyzed. All patients underwent total body bone scan with single photon emission computed tomography （SPECT） at least once during 1995 to 2000. Results： All patients were followed-up to 294 months after operation, bone metastases were found in 113 cases, suspected bone metastases 3 cases, with a bone metastases rate of 50.9% （113/222）. Multivariate analysis by Cox＇s proportional hazards regression model showed that there were four risk factors of bone metastases in breast cancer： （1） clinical stage, Ⅰ～Ⅳ stages with a hazard ratio of bone metastases of 1.945, 95% confidence interval 1.396～2.710; （2） number of invaded axillary lymph nodes, with a hazard ratio of 1.039, 95% confidence interval 1.0142～1.068; （3） skeletal complications （yes vs. no）, with a hazard ratio of bone metastases of 1.722, 95% confidence interval 1.060～2.796; （4） age at the time of surgery or diagnosis, with a hazard ratio of 2.048, 95% confidence interval 1.123～3.876 for patients of age 40～50 y versus patients bellow 40 y of age and 2.837, 95% confidence interval 1.473～5.465 for patients of age above 50 y versus patients of ages between 40 and 50. Kaplan-Meier curves showed that for patients with more than 5 invasive axillary lymph nodes, compared with those with 1～5, the bone metastasis rates increased significantly （x^2 =6.3319, P=0.012）. Conclusion： The clinical stage, number of metastatic axillary lymph nodes, age at the time of operation and skeletal complications are essential risk factors of bone metastases.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
The skeleton is the most common metastatic organ in patients with prostate cancer （PCa）. Non-invasive biomarkers that can facilitate the detection and monitoring of bone metastases are highly desirable. We designed ...The skeleton is the most common metastatic organ in patients with prostate cancer （PCa）. Non-invasive biomarkers that can facilitate the detection and monitoring of bone metastases are highly desirable. We designed this study to assess the expression patterns of serum miR-141 in patients with bone-metastatic PCa. Serum samples were collected to measure the miR-141 level in 56 patients, including six with benign prostatic hyperplasia （BPH）, 20 with localized PCa and 30 with bone-metastatic PCa （10 with hormone-naive PCa, 10 with hormone-sensitive PCa and 10 with hormone-refractory PCa）. A bone scan was performed for each patient with PCa to assess the number of bone lesions. The quantification of serum miR-141 levels was assayed by specific TaqMan qRT-PCR. The results showed that serum miR-141 levels were elevated in patients with bone metastasis （P〈O.O01）. There was no statistically significant difference in the serum miR-141 levels between patients with BPH and patients with localized PCa. Using Kendall＇s bivariate correlation test, both the Gleason score and the number of bone-metastatic lesions were found to correlate with serum miR-141 levels （P=0.012 and P〈O.O01, respectively）. The serum miR-141 level was found to be positively correlated with alkaline phosphatase （ALP） level in patients with skeletal metastasis, using Pearson＇s bivariate correlation test. No relationship was found between the serum miR-141 level and the serum prostate-specific antigen （PSA） level. We concluded that serum miR-141 levels are elevated in patients with bone-metastatic PCa and that patients with higher levels of serum miR-141 developed more bone lesions. Furthermore, serum miR-141 levels are correlated with serum ALP levels but not serum PSA levels.展开更多
文摘Angiosarcoma of bone is an exceedingly rare primary bone malignancy that can present as an aggressive osteolytic lesion. This subset can radiologically mimic non-vascular neoplasms and impose serious challenges in reaching the correct diagnosis. Meanwhile histological diagnosis can be extremely challenging too, as the pathological features often resemble that of aneurysmal bone cysts. We present an unusual case of a 22-year-old woman who presented with a rapidly growing humeral tumor of 8 months’ duration. The case of intraosseous angiosarcoma presented as a diagnostic dilemma and the relevant radiological and pathologic findings were discussed. We describe the clinical, radiological and pathological features of this unique case, and review the literature concerning Angiosarcoma of bone. Our case highlights the diagnostic difficulties for such very rare tumours and clinico-pathological correlation is of paramount importance to differential diagnosis.
文摘Objective： To evaluate the functional outcome and complications of allograft replacement in management of bone tumors. Methods： Between March 1992 and September 2002, 164 patients underwent bone tumor resection and massive allograft reconstruction of bone defects. The length of the resected part ranged from 5-35 cm. The resections were classified as marginal or wide resections of the tumor on the basis of the Musculoskeletal Tumor Society staging system. Fresh-frozen allografts were employed as osteoarticular grafts （n = 95）, hemi-condylar （n = 15）, massive （n = 23）, allograft-prosthesis composite （n = 12）, intercalary grafts （n = 15） or hemi-pelvic grafts （n = 4）. Most of the lesions were osteosarcoma and giant cell tumor of bone and located in proximal and distal femur, proximal tibia and humerus. Results： At a median follow-up of 47 months （range, 12 to 168 months） after the operation, 154 of the patients in the study were free of disease and 10 died of disease. Twenty-one （12.8%） patients had local recurrence and 38 （23.2%） nonunion. Late complications included 11 （6.7%） fractures of the allograft and 18 （11.0%） infections of the graft, instability of the joint in the form of subluxation was noted in 13 （7.9%） patients. Ten extremities were amputated due to local recurrence or severe infection. Conclusion： AIIografts can be used for reconstruction of bony defects after tumor resection. AIIograft has nearly similar shape, strength, osteo-inductivity and osteo-conductivity with host bone. AIIograft implantation is a high complication reconstruction method, and the dsk of recurrence increases when less surgical margin achieves.
文摘Among plasma cell disorders, solitary plasmacytoma (solitany-plasmacytoma of bone, SPB and extramedullary plasmacytoma, EMP) is rare as compared with mulitiple myeloma (MM). Furthermore.the relationship between solitary plasmacytoma and MM remains unclear.Between 1960 and 1994, 24 patients with SPB and 20 with EMP were treated. The criteria for diagonosis were: (1) No evidence of other lesions based on clinical and radiologic examinations;(2) Biopsy evidence of a plasma cell neoplasm; (3) Bone marrow biopsy specimen with negative findings (less than 10% plasma cell); (4) No anemia, hypercalcemia or renal involvement. The average follow-up period was 112 months (from 6 to 360 months). Fifty-four percent of patients with SPB and 40% of patients with EMP developed MM, however, there was no significant statistical difference between SPB and EMP (P <0.05).We suggested that solitary plasmacytomas be classified as two types, latent and aggressive. The former was histologically well-differentiated plasmacytomas. The latter was poorly differentiated tumors which easily progress to MM. The treatment of choice is wide excision or thorough curettage, by cryogenic necrosis with liquid nitrogen or cautery of the bony wall with phenol and the cavity filled with bone grafts or cement. All patients with apparently isolated plasmacytoma should he given if the tumor turns out to be poorly differentiated, in order to delay their progression to MM.
文摘Bone metastasis is a rare event in patients with gastric cancer, but pathologic fracture, paralysis, pain and hematological disorders associated with the bone metastasis may influence the quality of life. We report herein the case of a 53-year-old man who presented with primary remnant gastric cancer with bone metastasis. The patient requested further investigations after detection of a metastatic lesion in the 2 nd lumbar vertebra during evaluation for back pain that had persisted for 3 mo. No other metastatic lesions were detected. He underwent total gastrectomy and palliative metastasectomy to aid in reduction of symptoms, and he received combination chemotherapy with tegafur(S-1) and cisplatin. The patient survived for about 60 mo after surgery. Currently, there is no treatment guideline for gastric cancer with bone metastasis, and we believe that gastrectomy plus metastasectomy may be an effective therapeutic option for improving qualityof life and survival in patients with resectable primary gastric cancer and bone metastasis.
文摘Objective ：To evaluate the sensitivity of serum tartrate-resistant acid phosphatase 5b（Tracp5b） activity in monitoring bisphosphonate treatment results of bone metastasis in breast cancer（BC） patients. Methods：The serum activities of Tracp5b, CEA, CA153 were measured in 58 BC patients, including 26 without bone metastasis, 32 with bone metastasis. The serum activities of TracpSb, CEA, CA153 were also measured in 19 patients with bone metastasis after 3 months of bisphosphonate treatment. Eighteen healthy women with age from 34 to 70 served as control. Results：Serum TracpSb was significantly elevated in patients with bone metastasis compared with that in all any other groups（P〈 0.05）. The sensitivity of TracpSb was 78.13% and the specificity was 86.36%. The sensitivity of CA153 was 37.50% and the specificity was 77.27%. The sensitivity of CEA was 21.88% and the specificity was 84.09%. The serum activity of TracpSb decreased significantly（P 〈 0.05） after 3 months of bisphosphonate treatment, while the levels of CA153 and CEA were unchanged. Conclusion：Serum Tracp5b activity is a useful diagnostic marker for bone metastasis in BC patients and can be used to evaluate the treatment results of bisphosphonate.
文摘A rational analysis procedure for solitary lesions on whole bone scan-ning was offered. This study was undertaken to analyze retrospectively solitary le-sions which obtained final diagnose through the following aspects: (1) diagnosis ofbone metastasis, (2) the incidence of bone metastasis in different tumor, (3) the mostpossible lesion sites indicating bone metastasis, (4) morphological analysis of solitarylesions. The results are: (1) The incidence of solitary lesions in 2465 cases on wholebone scanning is 15.3%. (2) The rate of bone metastasis is 24.8% in 282 patientswith primary malignancy. The rate of bone metastasis is 6.3% in 64 patients withoutprimary malignancy, and the total diagnostic rate of bone metastasis is 21.4% in 346patients. (3) In patients with primary malignancy, the incidence of bone metastasis ofsolitary lesions is as follows respectively: bronchi cancer 36.1%(22/61); breast cancer23.8%(20/84); prostate gland 17.2%(5/29); other urinary system cancer 22.2%(4/18):G.I. system cancer 16.9%(10/59); others 29.0%(9/31). There is no significant differ-ence in different cancer. (4) In patients without primary malignancy, 93.7%(60/64) ofsolitary lesions are benign. (5) From anatomical point of view, we found the diagnos-tic rate of bone metastasis is as follow: 30% in spine; 34.2% in pelvis; 36.4% in skull;10.8% in other bones. There are significant differences in four groups. It is concludedthat: (1) The diagnostic rate of bone metastasis in solitary lesions is 21.4%. (2) Themost possible solitary lesions indicating osseous tumor spread are at spine, pelvic andskull. (3) Special attention to "cold" and streak like lesions should be paid. (4) Aclinical analysis procedure for diagnosis of solitary lesions has been summarized outhere.
文摘Objective： To investigate the risk factors of bone metastases in breast carcinoma. Methods： By cross sectional study, the data of 225 breast cancer patients who were inpatients in four hospitals in Hangzhou were analyzed. All patients underwent total body bone scan with single photon emission computed tomography （SPECT） at least once during 1995 to 2000. Results： All patients were followed-up to 294 months after operation, bone metastases were found in 113 cases, suspected bone metastases 3 cases, with a bone metastases rate of 50.9% （113/222）. Multivariate analysis by Cox＇s proportional hazards regression model showed that there were four risk factors of bone metastases in breast cancer： （1） clinical stage, Ⅰ～Ⅳ stages with a hazard ratio of bone metastases of 1.945, 95% confidence interval 1.396～2.710; （2） number of invaded axillary lymph nodes, with a hazard ratio of 1.039, 95% confidence interval 1.0142～1.068; （3） skeletal complications （yes vs. no）, with a hazard ratio of bone metastases of 1.722, 95% confidence interval 1.060～2.796; （4） age at the time of surgery or diagnosis, with a hazard ratio of 2.048, 95% confidence interval 1.123～3.876 for patients of age 40～50 y versus patients bellow 40 y of age and 2.837, 95% confidence interval 1.473～5.465 for patients of age above 50 y versus patients of ages between 40 and 50. Kaplan-Meier curves showed that for patients with more than 5 invasive axillary lymph nodes, compared with those with 1～5, the bone metastasis rates increased significantly （x^2 =6.3319, P=0.012）. Conclusion： The clinical stage, number of metastatic axillary lymph nodes, age at the time of operation and skeletal complications are essential risk factors of bone metastases.
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.
文摘The skeleton is the most common metastatic organ in patients with prostate cancer （PCa）. Non-invasive biomarkers that can facilitate the detection and monitoring of bone metastases are highly desirable. We designed this study to assess the expression patterns of serum miR-141 in patients with bone-metastatic PCa. Serum samples were collected to measure the miR-141 level in 56 patients, including six with benign prostatic hyperplasia （BPH）, 20 with localized PCa and 30 with bone-metastatic PCa （10 with hormone-naive PCa, 10 with hormone-sensitive PCa and 10 with hormone-refractory PCa）. A bone scan was performed for each patient with PCa to assess the number of bone lesions. The quantification of serum miR-141 levels was assayed by specific TaqMan qRT-PCR. The results showed that serum miR-141 levels were elevated in patients with bone metastasis （P〈O.O01）. There was no statistically significant difference in the serum miR-141 levels between patients with BPH and patients with localized PCa. Using Kendall＇s bivariate correlation test, both the Gleason score and the number of bone-metastatic lesions were found to correlate with serum miR-141 levels （P=0.012 and P〈O.O01, respectively）. The serum miR-141 level was found to be positively correlated with alkaline phosphatase （ALP） level in patients with skeletal metastasis, using Pearson＇s bivariate correlation test. No relationship was found between the serum miR-141 level and the serum prostate-specific antigen （PSA） level. We concluded that serum miR-141 levels are elevated in patients with bone-metastatic PCa and that patients with higher levels of serum miR-141 developed more bone lesions. Furthermore, serum miR-141 levels are correlated with serum ALP levels but not serum PSA levels.
