BACKGROUND Herlyn-Werner-Wunderlich(HWW)syndrome is a rare Müllerian duct anomaly,characterized by a combination of urogenital abnormalities.The occurrence of primary cervico-vaginal carcinomas in patients with H...BACKGROUND Herlyn-Werner-Wunderlich(HWW)syndrome is a rare Müllerian duct anomaly,characterized by a combination of urogenital abnormalities.The occurrence of primary cervico-vaginal carcinomas in patients with HWW syndrome is excep-tionally rare,posing significant challenges for screening,early diagnosis,and effective management.CASE SUMMARY We report a rare case of primary clear cell carcinoma of the vagina complicated in a 40-year-old woman with HWW syndrome.The patient presented with irregular vaginal bleeding for 4 years.On gynecological examination,an oblique vaginal septum was suspected.Surgical resection of the vaginal septum revealed a com-municating fistula and a tumor on the left vagina and the left side of the septum,which was confirmed as clear cell carcinoma.One month later,she underwent a radical hysterectomy,vaginectomy,bilateral salpingo-oophorectomy,and pelvic lymph node dissection.Due to significant side effects,she completed only one course of chemotherapy.A year later,lung metastasis was detected and continued to grow.A thoracoscopic wedge resection of the right upper lobe was performed 4 years after the initial surgery.We also conducted a systemic review of the lite-rature on primary cervical or vaginal carcinoma in HWW syndrome to explore this rare entity.CONCLUSION Cervico-vaginal adenocarcinomas in patients with HWW syndrome are occult,and require early surgical intervention and regular imaging surveillance.展开更多
BACKGROUND Clear cell adenocarcinoma of the urethra is a rare type of aggressive cancer with a poor prognosis.Clear cell carcinoma of the urethra represents less than 0.02%of all malignancies in women.Adenocarcinomas ...BACKGROUND Clear cell adenocarcinoma of the urethra is a rare type of aggressive cancer with a poor prognosis.Clear cell carcinoma of the urethra represents less than 0.02%of all malignancies in women.Adenocarcinomas account for 10%of female urethral carcinomas,of which 40%are the clear cell variant.Determining the presence or absence of certain mutations through genetic testing may predict whether a patient with cancer may benefit from a particular chemotherapy regimen.CASE SUMMARY A 40-year-old woman presented with a 3-year history of slow urinary flow and a 3-mo history of urinary urgency and frequency as well as gross hematuria.An abdominal and pelvic computed tomography scan demonstrated enlarged lymph nodes in the abdomen and pelvis.A biopsy of a left inguinal lymph node microscopically confirmed a metastatic adenocarcinoma of the urethra.Specialized genetic testing determined personalized chemotherapy.She was treated successfully with a non-platinum-based chemotherapy consisting of paclitaxel and bevacizumab.Following 3 cycles of paclitaxel and bevacizumab,she attained significant clinical improvement,and response by FDG-Positron emission tomography(PET)imaging showed a definite improvement in size and metabolic activity.She achieved complete response after 6 cycles of therapy by PET scan.The patient concluded 11 cycles of paclitaxel and bevacizumab,and a subsequent PET scan confirmed progression of metastatic disease.The patient was then treated with two cycles of doxorubicin after which a PET scan revealed a mixed response to the treatment.CONCLUSION We report the first case of a patient with metastatic clear cell adenocarcinoma of the urethra who underwent personalized chemotherapy after testing for cancer gene alterations.Our unique case represents the safe and effective use of nonplatinum-based chemotherapy in clear cell adenocarcinoma of the urethra.展开更多
BACKGROUND Epidermal growth factor receptor tyrosine kinase inhibitors(EGFR-TKIs)significantly improve the survival of patients with Epidermal growth factor receptor(EGFR)sensitive mutations in non-small cell lung can...BACKGROUND Epidermal growth factor receptor tyrosine kinase inhibitors(EGFR-TKIs)significantly improve the survival of patients with Epidermal growth factor receptor(EGFR)sensitive mutations in non-small cell lung cancer(NSCLC).CASE SUMMARY A 67-year-old female patient in advanced lung adenocarcinoma suffered from drug resistance after EGFR-TKIs treatment.Secondary pathological tissue biopsy confirmed squamous cell carcinoma(SCC)transformation.Patients inevitably encountered drug resistance issues after receiving EGFR-TKIs treatment for a certain period of time,while EGFR-TKIs can significantly improve the survival of patients with EGFR-sensitive mutations in NSCLC.Notably,EGFR-TKIs resistance includes primary and acquired.Pathological transformation is one of the mechanisms of acquired resistance in EGFR-TKIs,with SCC transformation being relatively rare.Our results provide more detailed results of the patient’s diagnosis and treatment process on SCC transformation after EGFR-TKIs treatment for lung adenocarcinoma.CONCLUSION Squamous cell carcinoma transformation is one of the acquired resistance mechanisms of EGFR-TKIs in advanced lung adenocarcinoma with EGFR mutations.展开更多
OBJECTIVE To assess the effect of 17 β-estradiol（E2） on cell proliferation, cell invasiveness and its regulation of MTA3, Snail and matrix metalloproteinase 2 （MMP-2） expression in the ovarian clear cell adenocar...OBJECTIVE To assess the effect of 17 β-estradiol（E2） on cell proliferation, cell invasiveness and its regulation of MTA3, Snail and matrix metalloproteinase 2 （MMP-2） expression in the ovarian clear cell adenocarcinoma cell line ES-2, and to further investigate the mechanism involved. METHODS We first investigated expression of ERα, ERβ, PR and E-cadherin of ES-2 cells by RT-PCR and Western blots. Before all experiments, the ES-2 cells were grown in medium depleted of steroid for more than 7 days. Following treatment with 10^-7,10^-8 and 10^-9 M E2, cell viability of the ES-2 cells was determined by the MTT method, and the cell cycle distribution and apoptosis were examined by flow cytometry （FCM）. Invasion and mobility assays were performed using modified Boyden chambers. MTA3, Snail and MMP-2 mRNA expression was measured by RT-PCR, and Snail, MMP-2 protein levels were determined by IHC. MMP-2 activity was assayed by zymography. RESULTS RT-PCR and Western Blots showed that theexpression of ERα and E-cadherin mRNA and protein in the ES-2 cells was negative, while ERβ and PR expression was positive. E2 at 10^-7,10^-8 or 10^-9M stimulated cell proliferation. A level of 10^-8M E2 reduced the proportion of G0-G1 phase cells and increased the proportion of cells in the S phase, but it had no effect on apoptosis. Invasiveness and mobility of the ES-2 cells was significantly increased by 10^-8M E2. Treatment with 10^-8M E2 led to reduced MTA3 mRNA expression, and elevated Snail and MMP-2 mRNA and protein levels. CONCLUSION E2 enhanced invasion by the ES-2 cells. The effects observed maybe mediated by down-regulation of MTA3 and up-reguation of Snail and MMP-2.展开更多
Humoral Hypercalcemia of Malignancy (HHM) has been reported in association with a number of malignancies. In gynecologic malignancies, ovarian Clear Cell Carcinoma (CCC) is one of the most commonhistologic subtypes, w...Humoral Hypercalcemia of Malignancy (HHM) has been reported in association with a number of malignancies. In gynecologic malignancies, ovarian Clear Cell Carcinoma (CCC) is one of the most commonhistologic subtypes, whereas HHM caused by endometrial CCC is very rare. We report a case of endometrial CCC with HHM, with a low serum intact PTH level, elevated serum PTH-related Peptide (PTH-rP), and immunohistochemically demonstrated PTH-rP in the neoplasm.展开更多
BACKGROUND The clinicopathological features and prognosis of gastric signet ring cell carcinoma(GSRC)remain controversial,particularly with regard to sensitivity to postoperative adjuvant therapy.AIM To compare the pa...BACKGROUND The clinicopathological features and prognosis of gastric signet ring cell carcinoma(GSRC)remain controversial,particularly with regard to sensitivity to postoperative adjuvant therapy.AIM To compare the pathological features of GSRC with those of gastric adenocarcinoma of different degrees of differentiation and the differences in survival prognosis between the different disease processes.METHODS By screening gastric cancer patients from 2010 to 2015 in the database of Surveillance,Epidemiology and End Results,and collecting the clinicopathological and prognostic data of gastric cancer patients who underwent surgery from January 2014 to December 2016 in the Second Affiliated Hospital of Nanchang University,we analyzed the general pathological characteristics of GSRC by the chi-square test.Univariate and multivariate analyses were conducted to compare the factors affecting the survival and prognosis of early and advanced gastric adenocarcinoma.The Kaplan-Meier curves were plotted to reveal the survival difference between early and advanced GSRC and different differentiated types of gastric adenocarcinoma.The prognosis model of advanced GSRC was established with R software,and the area under curve(AUC)and C-index were used to assess the accuracy of the model.RESULTS Analysis of pathological features revealed that signet ring-cell carcinoma(SRC)was more frequently seen in younger(<60 years),female,and White patients compared to non-SRC patients.SRC was less commonly associated with early gastric cancer(EGC)(23.60%vs 39.10%),lower N0(38.61%vs 61.03%),and larger tumour sizes>5 cm(31.15%vs 27.10%)compared to the differentiated type,while the opposite was true compared to the undifferentiated type.Survival prognostic analysis found no significant difference in the prognosis of SRC patients among EGC patients.In contrast,among advanced gastric cancer(AGC)patients,the prognosis of SRC patients was correlated with age,race,tumour size,AJCC stage,T-stage,and postoperative adjuvant therapy.The predictive model showed that the 3-year AUC was 0.787,5-year AUC was 0.806,and C-index was 0.766.Compared to non-SRC patients,patients with SRC had a better prognosis in EGC[hazard ratio(HR):0.626,95%confidence interval(CI):0.427-0.919,P<0.05]and a worse prognosis in AGC(HR:1.139,95%CI:1.030-1.258,P<0.05).When non-SRC was divided into differentiated and undifferentiated types for comparison,it was found that in EGC,SRC had a better prognosis than differentiated and undifferentiated types,while there was no significant difference between differentiated and undifferentiated types.In AGC,there was no significant difference in prognosis between SRC and undifferentiated types,both of which were worse than differentiated types.A prognostic analysis of postoperative adjuvant therapy for SRC in patients with AGC revealed that adjuvant postoperative radiotherapy or chemotherapy significantly improved patient survival(34.6%and 36.2%vs 18.6%,P<0.05).CONCLUSION The prognosis of SRC is better than that of undifferentiated type,especially in EGC,and its prognosis is even better than that of differentiated type.SRC patients can benefit from early detection,surgical resection,and aggressive adjuvant therapy.展开更多
A 62-year-old woman with a history of curatively treated mucinous ovarian cancer, presented with dyspnea, anorexia and right-upper-quadrant pain at consultation with her general practitioner. A CT scan revealed severa...A 62-year-old woman with a history of curatively treated mucinous ovarian cancer, presented with dyspnea, anorexia and right-upper-quadrant pain at consultation with her general practitioner. A CT scan revealed several lymph node metastases in lungs and abdomen as well as a tumor in Morrison’s pouch and biopsy revealed renal cell carcinoma. Therefore, she was referred to Department of Urology. The multidisciplinary team could not immediately reject that there could be an exophytic tumor in the right kidney but discrepancy between histology and imaging, led to several biopsies including laparoscopic procedure. Re-examination of the primary ovarian cancer showed that one percent was classified as clear cell carcinoma. The final diagnose was metastatic clear cell ovarian carcinoma. The patient was terminal and suffered of cachexia and pleural effusion. The patient deceased four months after first consultation.展开更多
AIM: To retrospectively analyze the computed tomography (CT) and magnetic resonance imaging (MRI) appearances of primary clear cell carcinoma of the liver (PCCCL) and compare the imaging appearances of PCCCL and commo...AIM: To retrospectively analyze the computed tomography (CT) and magnetic resonance imaging (MRI) appearances of primary clear cell carcinoma of the liver (PCCCL) and compare the imaging appearances of PCCCL and common type hepatocellular carcinoma (CHCC) to determine whether any differences exist between the two groups. METHODS: Twenty cases with pathologically proven PCCCL and 127 cases with CHCC in the Second Affiliated Hospital of Sun Yat-sen University were included in this study. CT or MRI images from these patients were retrospectively analyzed. The following imaging findings were reviewed: the presence of liver cirrhosis, tumor size, the enhancement pattern on dynamic contrast scanning, the presence of pseudo capsules, tumor rupture, portal vein thrombosis and lymph node metastasis. RESULTS: Both PCCCL and CHCC were prone to occur in patients with liver cirrhosis, the association rate of liver cirrhosis was 80.0% and 78.7%, respectively (P > 0.05). The mean sizes of PCCCL and CHCC tumors were (7.28 ± 4.25) cm and (6.96 ± 3.98) cm, respectively. Small HCCs were found in 25.0% (5/20) of PCCCL and 19.7% (25/127) of CHCC cases. No significant differences in mean size and ratio of small HCCs were found between the two groups (P = 0.658 and 0.803, respectively). Compared with CHCC patients, PCCCL patients were more prone to form pseudo capsules (49.6% vs 75.0%, P = 0.034). Tumor rupture, typical HCC enhancement patterns and portal vein tumor thrombosis were detected in 15.0% (3/20), 72.2% (13/18) and 20.0% (4/20) of patients with PCCCL and 3.1% (4/127), 83.6% (97/116) and 17.3% (22/127) of patients with CHCC, respectively. There were no significant differences between the two groups (all P > 0.05). No patients with PCCCL and 2.4% (3/127) of patients with CHCC showed signs of lymph node metastasis (P > 0.05). CONCLUSION: The imaging characteristics of PCCCL are similar to those of CHCC and could be useful for differentiating these from other liver tumors (such as hemangioma and hepatic metastases). PCCCLs are more prone than CHCCs to form pseudo capsules.展开更多
Study design: The authors report on a clear cell meningioma (CCM) of the sacrum revealed 17 years after a removal of a spinal lesion. A review of 29 spinal CCM previously reported was done. Objective: To focus on clin...Study design: The authors report on a clear cell meningioma (CCM) of the sacrum revealed 17 years after a removal of a spinal lesion. A review of 29 spinal CCM previously reported was done. Objective: To focus on clinical and biological behaviour of CCM. Summary of background data: CCM is a rare subtype characterrized by its inordinately aggressive clinical course despite its benign appearance. The tumour shows proclivity for the cerebellopontine angle and cauda equine region. Recurrence and metastasis have been commonly reported. Method: A 26 year-old man presented with low back pain related to a lytic lesion of the sacrum. He had a history of an intradural extramedullary meningioma of the cervical spine removed when he was 9 year-old. CT scan revealed a sacral osteolytic lesion with anterior and lateral extension. Open biopsy revealed a clear cell meningioma. That was the same pattern of the tumour removed 17 years before. Results: Our patient was managed conservatively by palliative radiation therapy. At 2 years follow-up, he experienced improvement of pain and walking. Conclusion: CCM is an aggressive tumour with a high risk of metastases through cerebrospinal fluid. A follow up with spinal MRI should be performed.展开更多
This report documents a rare case of complete spontaneous regression of clear cell carcinoma of the endometrium. An elderly woman with paranoid schizophrenia was admitted to the hospital because of vaginal bleeding. D...This report documents a rare case of complete spontaneous regression of clear cell carcinoma of the endometrium. An elderly woman with paranoid schizophrenia was admitted to the hospital because of vaginal bleeding. Diagnostic curetting and biopsy were performed and she was diagnosed with clear cell carcinoma of endometrium. Anti-tumour therapy was not possible because of her poor psychiatric status. 13 months later the woman died of a natural cause and at autopsy the endometrial tumour could no longer be identified. Myocardial infarction, aggravated by poor functional status of organism due to sepsis, was concluded to be the cause of death.展开更多
目的探讨骨转移性透明细胞性肾细胞癌(clear c ell renal cell carcinoma bone metastasis,CCRCC-BM)患者的临床病理学特征,提高对CCRCC-BM的认识。方法回顾性分析2011年1月—2023年7月福州市第二总医院病理科收集的33例CCRCC-BM患者的...目的探讨骨转移性透明细胞性肾细胞癌(clear c ell renal cell carcinoma bone metastasis,CCRCC-BM)患者的临床病理学特征,提高对CCRCC-BM的认识。方法回顾性分析2011年1月—2023年7月福州市第二总医院病理科收集的33例CCRCC-BM患者的临床病理资料及随访结果,并复习相关文献。结果33例患者中,男性27例,女性6例,发病年龄42~94岁,中位年龄65岁。影像学均表现为膨胀性、溶骨性骨质破坏,骨转移病灶共43处,常见的骨转移部位为股骨(15处)及肱骨(12处);单发骨转移23例,伴一处或以上其他部位转移占10例。33例均因骨相关事件(skeletal related event,SREs)为首发症状就诊后经病理诊断为CCRCC-BM,18例出现肿瘤性坏死,3例伴有肉瘤样分化。Fuhrman细胞核分级:Ⅰ级16例、Ⅱ级14例,Ⅲ级1例,Ⅳ级2例。免疫表型:肿瘤细胞表达成对盒基因8(paired box protein 8,Pax-8)(32/33)、白细胞分化抗原10(cluster of differentiation 10,CD10)(33/33)、波形蛋白(vimentin)、碳酸酐酶Ⅸ(carbonic anhydraseⅨ,CAⅨ)(30/33)、上皮细胞膜抗原(epithelial membrane antigen,EMA)(32/33)、广谱细胞角蛋白(pan cytokeratin,CKpan)(31/33)。33例均经手术切除病灶,术后随访2~108个月,死亡21例,生存12例。结论CCRCC-BM好发于中老年男性患者,股骨、肱骨是最常见的转移部位,预后不佳。对于临床上不明原因出现的病理性骨折、脊髓压迫、骨痛等症状的患者,应考虑肾细胞癌骨转移的可能,结合既往史、病理形态学及免疫组织化学染色等有助于诊断及鉴别诊断。展开更多
A 17-year-old female presented with rectal bleeding from an ulcerated sigmoid mass in 1994.Initial pathological evaluation revealed a rare clear cell neoplasm of the colon,possibly originating from kidneys,adrenals,lu...A 17-year-old female presented with rectal bleeding from an ulcerated sigmoid mass in 1994.Initial pathological evaluation revealed a rare clear cell neoplasm of the colon,possibly originating from kidneys,adrenals,lung or a gynecologic source as a metastatic lesion.Extensive imaging studies were negative,and over the next 15 years,she remained well with no recurrence.The original resected neoplasm was reviewed and reclassified as a perivascular epithelioid cell neoplasm (PEComa).Although the long-term natural history of PEComas requires definition,increased clinical and pathological awareness should lead to increased recognition of an apparently rare type of colonic neoplasm that likely occurs more often than is currently appreciated.展开更多
文摘BACKGROUND Herlyn-Werner-Wunderlich(HWW)syndrome is a rare Müllerian duct anomaly,characterized by a combination of urogenital abnormalities.The occurrence of primary cervico-vaginal carcinomas in patients with HWW syndrome is excep-tionally rare,posing significant challenges for screening,early diagnosis,and effective management.CASE SUMMARY We report a rare case of primary clear cell carcinoma of the vagina complicated in a 40-year-old woman with HWW syndrome.The patient presented with irregular vaginal bleeding for 4 years.On gynecological examination,an oblique vaginal septum was suspected.Surgical resection of the vaginal septum revealed a com-municating fistula and a tumor on the left vagina and the left side of the septum,which was confirmed as clear cell carcinoma.One month later,she underwent a radical hysterectomy,vaginectomy,bilateral salpingo-oophorectomy,and pelvic lymph node dissection.Due to significant side effects,she completed only one course of chemotherapy.A year later,lung metastasis was detected and continued to grow.A thoracoscopic wedge resection of the right upper lobe was performed 4 years after the initial surgery.We also conducted a systemic review of the lite-rature on primary cervical or vaginal carcinoma in HWW syndrome to explore this rare entity.CONCLUSION Cervico-vaginal adenocarcinomas in patients with HWW syndrome are occult,and require early surgical intervention and regular imaging surveillance.
文摘BACKGROUND Clear cell adenocarcinoma of the urethra is a rare type of aggressive cancer with a poor prognosis.Clear cell carcinoma of the urethra represents less than 0.02%of all malignancies in women.Adenocarcinomas account for 10%of female urethral carcinomas,of which 40%are the clear cell variant.Determining the presence or absence of certain mutations through genetic testing may predict whether a patient with cancer may benefit from a particular chemotherapy regimen.CASE SUMMARY A 40-year-old woman presented with a 3-year history of slow urinary flow and a 3-mo history of urinary urgency and frequency as well as gross hematuria.An abdominal and pelvic computed tomography scan demonstrated enlarged lymph nodes in the abdomen and pelvis.A biopsy of a left inguinal lymph node microscopically confirmed a metastatic adenocarcinoma of the urethra.Specialized genetic testing determined personalized chemotherapy.She was treated successfully with a non-platinum-based chemotherapy consisting of paclitaxel and bevacizumab.Following 3 cycles of paclitaxel and bevacizumab,she attained significant clinical improvement,and response by FDG-Positron emission tomography(PET)imaging showed a definite improvement in size and metabolic activity.She achieved complete response after 6 cycles of therapy by PET scan.The patient concluded 11 cycles of paclitaxel and bevacizumab,and a subsequent PET scan confirmed progression of metastatic disease.The patient was then treated with two cycles of doxorubicin after which a PET scan revealed a mixed response to the treatment.CONCLUSION We report the first case of a patient with metastatic clear cell adenocarcinoma of the urethra who underwent personalized chemotherapy after testing for cancer gene alterations.Our unique case represents the safe and effective use of nonplatinum-based chemotherapy in clear cell adenocarcinoma of the urethra.
文摘BACKGROUND Epidermal growth factor receptor tyrosine kinase inhibitors(EGFR-TKIs)significantly improve the survival of patients with Epidermal growth factor receptor(EGFR)sensitive mutations in non-small cell lung cancer(NSCLC).CASE SUMMARY A 67-year-old female patient in advanced lung adenocarcinoma suffered from drug resistance after EGFR-TKIs treatment.Secondary pathological tissue biopsy confirmed squamous cell carcinoma(SCC)transformation.Patients inevitably encountered drug resistance issues after receiving EGFR-TKIs treatment for a certain period of time,while EGFR-TKIs can significantly improve the survival of patients with EGFR-sensitive mutations in NSCLC.Notably,EGFR-TKIs resistance includes primary and acquired.Pathological transformation is one of the mechanisms of acquired resistance in EGFR-TKIs,with SCC transformation being relatively rare.Our results provide more detailed results of the patient’s diagnosis and treatment process on SCC transformation after EGFR-TKIs treatment for lung adenocarcinoma.CONCLUSION Squamous cell carcinoma transformation is one of the acquired resistance mechanisms of EGFR-TKIs in advanced lung adenocarcinoma with EGFR mutations.
文摘OBJECTIVE To assess the effect of 17 β-estradiol（E2） on cell proliferation, cell invasiveness and its regulation of MTA3, Snail and matrix metalloproteinase 2 （MMP-2） expression in the ovarian clear cell adenocarcinoma cell line ES-2, and to further investigate the mechanism involved. METHODS We first investigated expression of ERα, ERβ, PR and E-cadherin of ES-2 cells by RT-PCR and Western blots. Before all experiments, the ES-2 cells were grown in medium depleted of steroid for more than 7 days. Following treatment with 10^-7,10^-8 and 10^-9 M E2, cell viability of the ES-2 cells was determined by the MTT method, and the cell cycle distribution and apoptosis were examined by flow cytometry （FCM）. Invasion and mobility assays were performed using modified Boyden chambers. MTA3, Snail and MMP-2 mRNA expression was measured by RT-PCR, and Snail, MMP-2 protein levels were determined by IHC. MMP-2 activity was assayed by zymography. RESULTS RT-PCR and Western Blots showed that theexpression of ERα and E-cadherin mRNA and protein in the ES-2 cells was negative, while ERβ and PR expression was positive. E2 at 10^-7,10^-8 or 10^-9M stimulated cell proliferation. A level of 10^-8M E2 reduced the proportion of G0-G1 phase cells and increased the proportion of cells in the S phase, but it had no effect on apoptosis. Invasiveness and mobility of the ES-2 cells was significantly increased by 10^-8M E2. Treatment with 10^-8M E2 led to reduced MTA3 mRNA expression, and elevated Snail and MMP-2 mRNA and protein levels. CONCLUSION E2 enhanced invasion by the ES-2 cells. The effects observed maybe mediated by down-regulation of MTA3 and up-reguation of Snail and MMP-2.
文摘Humoral Hypercalcemia of Malignancy (HHM) has been reported in association with a number of malignancies. In gynecologic malignancies, ovarian Clear Cell Carcinoma (CCC) is one of the most commonhistologic subtypes, whereas HHM caused by endometrial CCC is very rare. We report a case of endometrial CCC with HHM, with a low serum intact PTH level, elevated serum PTH-related Peptide (PTH-rP), and immunohistochemically demonstrated PTH-rP in the neoplasm.
基金Supported by National Natural Science Foundation of China,No.81860433Natural Science Youth Foundation of Jiangxi Province,No.20192BAB215036+2 种基金Jiangxi Province Natural Science Key R&D Project-General Project,No.20202BBG73024Training Plan for Academic and Technical Young Leaders of Major Disciplines in Jiangxi Province,No.20204BCJ23021Jiangxi Provincial Education Department,Science and Technology Research Project-Youth Project,No.GJJ210252。
文摘BACKGROUND The clinicopathological features and prognosis of gastric signet ring cell carcinoma(GSRC)remain controversial,particularly with regard to sensitivity to postoperative adjuvant therapy.AIM To compare the pathological features of GSRC with those of gastric adenocarcinoma of different degrees of differentiation and the differences in survival prognosis between the different disease processes.METHODS By screening gastric cancer patients from 2010 to 2015 in the database of Surveillance,Epidemiology and End Results,and collecting the clinicopathological and prognostic data of gastric cancer patients who underwent surgery from January 2014 to December 2016 in the Second Affiliated Hospital of Nanchang University,we analyzed the general pathological characteristics of GSRC by the chi-square test.Univariate and multivariate analyses were conducted to compare the factors affecting the survival and prognosis of early and advanced gastric adenocarcinoma.The Kaplan-Meier curves were plotted to reveal the survival difference between early and advanced GSRC and different differentiated types of gastric adenocarcinoma.The prognosis model of advanced GSRC was established with R software,and the area under curve(AUC)and C-index were used to assess the accuracy of the model.RESULTS Analysis of pathological features revealed that signet ring-cell carcinoma(SRC)was more frequently seen in younger(<60 years),female,and White patients compared to non-SRC patients.SRC was less commonly associated with early gastric cancer(EGC)(23.60%vs 39.10%),lower N0(38.61%vs 61.03%),and larger tumour sizes>5 cm(31.15%vs 27.10%)compared to the differentiated type,while the opposite was true compared to the undifferentiated type.Survival prognostic analysis found no significant difference in the prognosis of SRC patients among EGC patients.In contrast,among advanced gastric cancer(AGC)patients,the prognosis of SRC patients was correlated with age,race,tumour size,AJCC stage,T-stage,and postoperative adjuvant therapy.The predictive model showed that the 3-year AUC was 0.787,5-year AUC was 0.806,and C-index was 0.766.Compared to non-SRC patients,patients with SRC had a better prognosis in EGC[hazard ratio(HR):0.626,95%confidence interval(CI):0.427-0.919,P<0.05]and a worse prognosis in AGC(HR:1.139,95%CI:1.030-1.258,P<0.05).When non-SRC was divided into differentiated and undifferentiated types for comparison,it was found that in EGC,SRC had a better prognosis than differentiated and undifferentiated types,while there was no significant difference between differentiated and undifferentiated types.In AGC,there was no significant difference in prognosis between SRC and undifferentiated types,both of which were worse than differentiated types.A prognostic analysis of postoperative adjuvant therapy for SRC in patients with AGC revealed that adjuvant postoperative radiotherapy or chemotherapy significantly improved patient survival(34.6%and 36.2%vs 18.6%,P<0.05).CONCLUSION The prognosis of SRC is better than that of undifferentiated type,especially in EGC,and its prognosis is even better than that of differentiated type.SRC patients can benefit from early detection,surgical resection,and aggressive adjuvant therapy.
文摘A 62-year-old woman with a history of curatively treated mucinous ovarian cancer, presented with dyspnea, anorexia and right-upper-quadrant pain at consultation with her general practitioner. A CT scan revealed several lymph node metastases in lungs and abdomen as well as a tumor in Morrison’s pouch and biopsy revealed renal cell carcinoma. Therefore, she was referred to Department of Urology. The multidisciplinary team could not immediately reject that there could be an exophytic tumor in the right kidney but discrepancy between histology and imaging, led to several biopsies including laparoscopic procedure. Re-examination of the primary ovarian cancer showed that one percent was classified as clear cell carcinoma. The final diagnose was metastatic clear cell ovarian carcinoma. The patient was terminal and suffered of cachexia and pleural effusion. The patient deceased four months after first consultation.
文摘AIM: To retrospectively analyze the computed tomography (CT) and magnetic resonance imaging (MRI) appearances of primary clear cell carcinoma of the liver (PCCCL) and compare the imaging appearances of PCCCL and common type hepatocellular carcinoma (CHCC) to determine whether any differences exist between the two groups. METHODS: Twenty cases with pathologically proven PCCCL and 127 cases with CHCC in the Second Affiliated Hospital of Sun Yat-sen University were included in this study. CT or MRI images from these patients were retrospectively analyzed. The following imaging findings were reviewed: the presence of liver cirrhosis, tumor size, the enhancement pattern on dynamic contrast scanning, the presence of pseudo capsules, tumor rupture, portal vein thrombosis and lymph node metastasis. RESULTS: Both PCCCL and CHCC were prone to occur in patients with liver cirrhosis, the association rate of liver cirrhosis was 80.0% and 78.7%, respectively (P > 0.05). The mean sizes of PCCCL and CHCC tumors were (7.28 ± 4.25) cm and (6.96 ± 3.98) cm, respectively. Small HCCs were found in 25.0% (5/20) of PCCCL and 19.7% (25/127) of CHCC cases. No significant differences in mean size and ratio of small HCCs were found between the two groups (P = 0.658 and 0.803, respectively). Compared with CHCC patients, PCCCL patients were more prone to form pseudo capsules (49.6% vs 75.0%, P = 0.034). Tumor rupture, typical HCC enhancement patterns and portal vein tumor thrombosis were detected in 15.0% (3/20), 72.2% (13/18) and 20.0% (4/20) of patients with PCCCL and 3.1% (4/127), 83.6% (97/116) and 17.3% (22/127) of patients with CHCC, respectively. There were no significant differences between the two groups (all P > 0.05). No patients with PCCCL and 2.4% (3/127) of patients with CHCC showed signs of lymph node metastasis (P > 0.05). CONCLUSION: The imaging characteristics of PCCCL are similar to those of CHCC and could be useful for differentiating these from other liver tumors (such as hemangioma and hepatic metastases). PCCCLs are more prone than CHCCs to form pseudo capsules.
文摘Study design: The authors report on a clear cell meningioma (CCM) of the sacrum revealed 17 years after a removal of a spinal lesion. A review of 29 spinal CCM previously reported was done. Objective: To focus on clinical and biological behaviour of CCM. Summary of background data: CCM is a rare subtype characterrized by its inordinately aggressive clinical course despite its benign appearance. The tumour shows proclivity for the cerebellopontine angle and cauda equine region. Recurrence and metastasis have been commonly reported. Method: A 26 year-old man presented with low back pain related to a lytic lesion of the sacrum. He had a history of an intradural extramedullary meningioma of the cervical spine removed when he was 9 year-old. CT scan revealed a sacral osteolytic lesion with anterior and lateral extension. Open biopsy revealed a clear cell meningioma. That was the same pattern of the tumour removed 17 years before. Results: Our patient was managed conservatively by palliative radiation therapy. At 2 years follow-up, he experienced improvement of pain and walking. Conclusion: CCM is an aggressive tumour with a high risk of metastases through cerebrospinal fluid. A follow up with spinal MRI should be performed.
文摘This report documents a rare case of complete spontaneous regression of clear cell carcinoma of the endometrium. An elderly woman with paranoid schizophrenia was admitted to the hospital because of vaginal bleeding. Diagnostic curetting and biopsy were performed and she was diagnosed with clear cell carcinoma of endometrium. Anti-tumour therapy was not possible because of her poor psychiatric status. 13 months later the woman died of a natural cause and at autopsy the endometrial tumour could no longer be identified. Myocardial infarction, aggravated by poor functional status of organism due to sepsis, was concluded to be the cause of death.
文摘目的:透明细胞乳头状肾细胞肿瘤(clear cell papillary renal cell tumor,CCPRCT)是一种少见但重要的肾肿瘤类型,与其他肾细胞肿瘤具有类似的形态学特征,易导致误诊。本研究旨在探讨CCPRCT的临床病理特征、诊断及鉴别诊断要点,以提高其病理诊断的准确性。方法:收集15例CCPRCT患者,观察其临床及影像学特点,分析其镜下形态、免疫表型,并复习相关文献。结果:15例患者中,男10例,女5例,年龄为(54±17)岁,10例患者肿瘤位于左肾,5例患者肿瘤位于右肾。组织学上肿瘤均由增厚的纤维囊包裹,并局限于肾实质内;肿瘤细胞排列成乳头状、管状、囊状、腺泡及实性等结构;乳头由小到中等大小、透明细胞质的单层细胞组成,肿瘤均为世界卫生组织/国际泌尿病理学会(World Health Organization/International Society of Urological Pathology,WHO/ISUP)1级或2级;细胞核反极性分布,即核上移,靠近腔面,远离基底,类似鲨鱼牙齿排列。免疫组织化学染色显示:细胞角蛋白7、碳酸酐酶9(carbonic anhydrase 9,CA9)和高分子量细胞角蛋白(34βE12)均为阳性表达;α甲基酰基辅酶A消旋酶、CD117、转录因子E3均为阴性表达;CCPRCT中典型的CA9表现方式是“U型”着色,即腔缘不表达,基底和侧面表达。患者术后随访均未见复发或转移。结论:CCPRCT是一种惰性肾细胞肿瘤,预后好,临床可能存在过诊断,其形态学为特征性的核朝向腔缘的线性排列,特殊的免疫表型CA9呈“U型”阳性,可以与其他肾细胞肿瘤区分开来,但仍然需要积累更多患者来阐释其预后。
文摘术前预测透明细胞肾细胞癌(clear cell renal cell carcinoma,ccRCC)的分级可有效评估患者的预后并指导临床治疗,但实现精准预测是目前本领域内的一项重要问题。该研究首先确定最优建模的CT类型与网络层数,提出了一种基于改进残差网络的ccRCC的CT影像分级模型,具体包括:利用大卷积操作对图像进行原始特征提取,利用混合注意力模块通过计算特征图中当前空间和临近空间以及当前空间和远距离空间之间的信息交互获取更多有用的特征,使得原始图像特征图在通道维度与空间维度上进行自适应特征细化,利用四个深度卷积网络层提取图像深度特征,并利用改进通道注意力模块产生通道注意力特征图信息,提取更多通道上的交互信息。实验结果表明,增强CT实质期图像和34层残差网络最有利于分级预测模型的开发,所提出的模型的总体加权准确率、AUC、精度、召回率和F1分数分别为90.8%、0.897、90.5%、90.8%、90.9%,各项指标优于其他常见网络结构,因此,该模型在预测ccRCC的国际泌尿病理学学会(International Society of Urological Pathology,ISUP)分级方面有良好的效能,对患者的临床辅助诊断和预后治疗具有重要的理论指导意义。
文摘目的探讨骨转移性透明细胞性肾细胞癌(clear c ell renal cell carcinoma bone metastasis,CCRCC-BM)患者的临床病理学特征,提高对CCRCC-BM的认识。方法回顾性分析2011年1月—2023年7月福州市第二总医院病理科收集的33例CCRCC-BM患者的临床病理资料及随访结果,并复习相关文献。结果33例患者中,男性27例,女性6例,发病年龄42~94岁,中位年龄65岁。影像学均表现为膨胀性、溶骨性骨质破坏,骨转移病灶共43处,常见的骨转移部位为股骨(15处)及肱骨(12处);单发骨转移23例,伴一处或以上其他部位转移占10例。33例均因骨相关事件(skeletal related event,SREs)为首发症状就诊后经病理诊断为CCRCC-BM,18例出现肿瘤性坏死,3例伴有肉瘤样分化。Fuhrman细胞核分级:Ⅰ级16例、Ⅱ级14例,Ⅲ级1例,Ⅳ级2例。免疫表型:肿瘤细胞表达成对盒基因8(paired box protein 8,Pax-8)(32/33)、白细胞分化抗原10(cluster of differentiation 10,CD10)(33/33)、波形蛋白(vimentin)、碳酸酐酶Ⅸ(carbonic anhydraseⅨ,CAⅨ)(30/33)、上皮细胞膜抗原(epithelial membrane antigen,EMA)(32/33)、广谱细胞角蛋白(pan cytokeratin,CKpan)(31/33)。33例均经手术切除病灶,术后随访2~108个月,死亡21例,生存12例。结论CCRCC-BM好发于中老年男性患者,股骨、肱骨是最常见的转移部位,预后不佳。对于临床上不明原因出现的病理性骨折、脊髓压迫、骨痛等症状的患者,应考虑肾细胞癌骨转移的可能,结合既往史、病理形态学及免疫组织化学染色等有助于诊断及鉴别诊断。
文摘A 17-year-old female presented with rectal bleeding from an ulcerated sigmoid mass in 1994.Initial pathological evaluation revealed a rare clear cell neoplasm of the colon,possibly originating from kidneys,adrenals,lung or a gynecologic source as a metastatic lesion.Extensive imaging studies were negative,and over the next 15 years,she remained well with no recurrence.The original resected neoplasm was reviewed and reclassified as a perivascular epithelioid cell neoplasm (PEComa).Although the long-term natural history of PEComas requires definition,increased clinical and pathological awareness should lead to increased recognition of an apparently rare type of colonic neoplasm that likely occurs more often than is currently appreciated.
