N6-methyladenosine methylation(m6A)is a common type of epigenetic alteration that prominently affects the prognosis of tumor patients.However,it is unknown how the m6A regulator affects the tumor microenvironment(TME)...N6-methyladenosine methylation(m6A)is a common type of epigenetic alteration that prominently affects the prognosis of tumor patients.However,it is unknown how the m6A regulator affects the tumor microenvironment(TME)cell infiltration in adrenocortical carcinoma(ACC)and how it affects the prognosis of ACC patients yet.The m6A alteration patterns of 112 ACC patients were evaluated,furthermore,the association with immune infiltration cell features was investigated.The unsupervised clustering method was applied to typify the m6A alteration patterns of ACC patients.The principal component analysis(PCA)technique was taken to create the m6A score to assess the alteration pattern in specific malignancies.We found two independent patterns of m6A alteration in ACC patients.The TME cell infiltration features were significantly in accordance with phenotypes of tumor immune-inflamed and immune desert in both patterns.The m6Ascore also served as an independent predictive factor in ACC patients.The somatic copy number variation(CNV)and patients prognosis can be predicted by m6A alteration patterns.Moreover,the ACC patients with high m6A scores had better overall survival(OS)and higher efficiency in immune checkpoint blockade therapy.Our work demonstrated the significance of m6A alteration to the ACC patients immunotherapy.The individual m6A alteration patterns analysis might contribute to ACC patients prognosis prediction and immunotherapy choice.展开更多
BACKGROUND Adrenocortical carcinoma(ACC)is a rare malignant epithelial tumor originating from adrenocortical cells that carries a very poor prognosis.Metastatic or inoperable diseases are often considered incurable,an...BACKGROUND Adrenocortical carcinoma(ACC)is a rare malignant epithelial tumor originating from adrenocortical cells that carries a very poor prognosis.Metastatic or inoperable diseases are often considered incurable,and treatment remains a challenge.Especially for advanced cases such as ACC complicated with renal venous cancer thrombus,there are few cumulative cases in the literature.CASE SUMMARY The patient in this case was a 39-year-old middle-aged male who was admitted to the hospital for more than half a month due to dizziness and chest tightness.Computed tomography(CT)findings after admission revealed a left retroperitoneal malignant space-occupying lesion,but the origin of the formation of the left renal vein cancer thrombus remained to be determined.It was speculated that it originated from the left adrenal gland,perhaps a retroperitoneal source,and left adrenal mass+left nephrectomy+left renal vein tumor thrombus removal+angioplasty were performed under general anesthesia.Postoperative pathology results indicated a diagnosis of ACC.Postoperative steroid therapy was administered.At 3 mo after surgery,abdominal CT reexamination revealed multiple enlarged retroperitoneal lymph nodes and multiple low-density shadows in the liver,and palliative radiotherapy and mitotane were administered,considering the possibility of metastasis.The patient is currently being followed up.CONCLUSION ACC is a highly malignant tumor.Even if the tumor is removed surgically,there is still the possibility of recurrence.Postoperative mitotane and adjuvant chemoradiotherapy have certain benefits for patients,but they cannot fully offset the poor prognosis of this disease.展开更多
BACKGROUND Adrenocortical carcinoma(ACC)is a rare and highly invasive endocrine malignant tumor with a poor prognosis.Although surgical resection is the main treatment for ACC,postoperative recurrence and metastasis h...BACKGROUND Adrenocortical carcinoma(ACC)is a rare and highly invasive endocrine malignant tumor with a poor prognosis.Although surgical resection is the main treatment for ACC,postoperative recurrence and metastasis have become the important factors of death.Transcatheter arterial chemoembolization(TACE)is an important option for the treatment of advanced ACC with liver metastasis.However,due to the small number of patients treated for ACC,the safety of the operation is not completely clear and needs to be further studied.CASE SUMMARY A 47-year-old patient with ACC after surgery was admitted for reexamination by abdominal computerized tomography suggesting liver metastasis.Because the patient expressed reluctance to undergo surgery again,we treated her with TACE for the liver lesions.After treatment,symptoms of acute adrenal dysfunction such as decreased blood pressure,anorexia,and fatigue appeared,which were relieved after hydrocortisone treatment.To date,the patient's liver lesion is well controlled and no other metastases are observed.CONCLUSION We report a rare case of acute adrenal hypofunction after TACE.Glucocorticoid supplementation can alleviate the symptoms.展开更多
BACKGROUND Adrenocortical carcinoma(ACC),the second most aggressive malignant tumor,lacks epidemiological data worldwide;therefore,every new case can improve the understanding of the pathology and treatment of this ma...BACKGROUND Adrenocortical carcinoma(ACC),the second most aggressive malignant tumor,lacks epidemiological data worldwide;therefore,every new case can improve the understanding of the pathology and treatment of this malignancy.CASE SUMMARY We present the case of a 66-year-old Caucasian woman with a giant androgenproducing ACC(21 cm×17 cm×12 cm;2100 g),without metastases,which unusually presented with an acute onset of atrial flutter and congestive heart failure.The cardiac complications observed in our case support the hypothesis that androgen excess in women is a cardiovascular risk factor.Androgen excess in women can be a rare cause of reversible dilated cardiomyopathy,therefore a comprehensive approach to the patient is essential to improve the recognition of androgen-secreting ACC.The atrial flutter was remitted after initiation of drug treatment during admission.The severe heart failure was totally remitted at 6 mo after radical open surgery to remove the giant ACC.CONCLUSION Radical open surgery to remove a giant androgen-producing ACC was the firstline treatment to cure the excess of androgen,which determined the total remission of cardiac complications at 6 mo after surgery in the women of this case report.展开更多
Objective:To explore the gene biomarkers related to the diagnosis and prognosis of adrenocortical carcinoma(ACC)by bioinformatics.Methods:GEPIA online analysis tool was used to screen differentially expressed genes fo...Objective:To explore the gene biomarkers related to the diagnosis and prognosis of adrenocortical carcinoma(ACC)by bioinformatics.Methods:GEPIA online analysis tool was used to screen differentially expressed genes for sequencing data from patients with ACC and normal adrenal cortex.The overall survival rate and disease-free survival method were used to conduct batch survival analysis on differentially expressed genes,and the top 100 genes with HR values calculated by the two methods were obtained respectively.The intersection method is used to obtain core genes that play a key role in both overall survival and disease-free survival.GEPIA online analysis tool was used again to explore the relationship between the above-mentioned survival-related genes and the pathological stage of ACC.Use UALCAN online analysis tool to verify the survival-related genes again and draw the Kaplan-Meier survival curve.Finally,GSE33371 chip dataset of the GEO database was used to evaluate the diagnostic value of the above-mentioned survival-related genes.Results:514 differentially expressed genes were obtained by limma method.Batch analysis of differential genes was performed to obtain the top 100 genes most related to overall survival and disease-free survival,of which 13 genes were closely related to overall survival and disease-free survival.9 hub genes including TP73,SNHG1,PDE6D,GPC2,SUV39H2,HELLS,CLK2,COPS7B and CEP164 were finally obtained by exploring the relationship between their expression levels and pathological stage and resurvival analysis.At the same time,the results of ROC analysis suggest that the above hub genes have high diagnostic value for patients with adrenocortical carcinoma.Conclusion:By using GEPIA,UALCAN and the gene chip retrieved from GEO database,combined with the bioinformatics method,we analyzed and verified the new biomarkers that can be used to evaluate the prognosis of patients with ACC and to differential diagnosis of ACC,and provided the theoretical support of bioinformatics for exploring the occurrence,development of molecular mechanism and potential target of treatment of ACC.展开更多
ACC （adrenocortical carcinoma） is an uncommon and aggressive malignancy with an overall five year survival rate from less than 50%. The majority of cases are accidentally diagnosed by imaging studies done for other ...ACC （adrenocortical carcinoma） is an uncommon and aggressive malignancy with an overall five year survival rate from less than 50%. The majority of cases are accidentally diagnosed by imaging studies done for other reasons, so they are often diagnosed in the later stages. Due to the rarity of both neoplasms, the synchronous presentation and the lack of effective therapeutic options; we report a case concerning about a female patient with adrenal cancer with a low grade solitary fibrous tumor who underwent surgery, adjuvant radiotherapy and chemotherapy with good response.展开更多
Myxoid change in adrenocortical carcinoma is a rare phenomenon,and no case has been reported in Chinese population to date.We report here a case of myxoid adrenocortical carcinoma presenting as Cushing syndrome with a...Myxoid change in adrenocortical carcinoma is a rare phenomenon,and no case has been reported in Chinese population to date.We report here a case of myxoid adrenocortical carcinoma presenting as Cushing syndrome with an immunohistochemical study of the tumor and discuss the diagnosis with reference to the current literature.展开更多
Background:Clinical outcome of adrenocortical carcinoma(ACC)varies because of its heterogeneous nature and reliable prognostic prediction model for adult ACC patients is limited.The objective of this study was to deve...Background:Clinical outcome of adrenocortical carcinoma(ACC)varies because of its heterogeneous nature and reliable prognostic prediction model for adult ACC patients is limited.The objective of this study was to develop and externally validate a nomogram for overall survival(OS)prediction in adult patients with ACC after surgery.Methods:Based on the data from the Surveillance Epidemiology,and End Results(SEER)database,adults patients diagnosed with ACC between January 1988 and December 2015 were identified and classified into a training set,comprised of 404 patients diagnosed between January 2007 and December 2015,and an internal validation set,com-prised of 318 patients diagnosed between January 1988 and December 2006.The endpoint of this study was OS.The nomogram was developed using a multivariate Cox proportional hazards regression algorithm in the training set and its performance was evaluated in terms of its discriminative ability,calibration,and clinical usefulness.The nomogram was then validated using the internal SEER validation,also externally validated using the Cancer Genome Atlas set(TCGA,82 patients diagnosed between 1998 and 2012)and a Chinese multicenter cohort dataset(82 patients diag-nosed between December 2002 and May 2018),respectively.Results:Age at diagnosis,T stage,N stage,and M stage were identified as independent predictors for OS.A nomo-gram incorporating these four predictors was constructed using the training set and demonstrated good calibration and discrimination(C-index 95%confidence interval[CI],0.715[0.679-0.751]),which was validated in the internal validation set(C-index[95%CI],0.672[0.637-0.707]),the TCGA set(C-index[95%CI],0.810[0.732-0.888])and the Chi-nese multicenter set(C-index[95%CI],0.726[0.633-0.819]),respectively.Encouragingly,the nomogram was able to successfully distinguished patients with a high-risk of mortality in all enrolled patients and in the subgroup analyses.Decision curve analysis indicated that the nomogram was clinically useful and applicable.Conclusions:The study presents a nomogram that incorporates clinicopathological predictors,which can accurately predict the OS of adult ACC patients after surgery.This model and the corresponding risk classification system have the potential to guide therapy decisions after surgery.展开更多
Objective: Adrenocortical carcinoma (ACC) is a rare but highly malignant tumor, and its diagnosis is mostly delayed and prognosis is poor. We report estrogen receptor (ER) expression in this tumor and our clinical exp...Objective: Adrenocortical carcinoma (ACC) is a rare but highly malignant tumor, and its diagnosis is mostly delayed and prognosis is poor. We report estrogen receptor (ER) expression in this tumor and our clinical experiences with 17 ACC cases. Methods: The data of the 17 patients (9 females and 8 males, age range from 16 to 69 years, mean age of 42.6 years) with ACC were reviewed, and symptoms, diagnostic procedures, treatment, and results of follow-up were evaluated. Immunohistochemistry was used to detect ER expression in tumor samples from the 17 patients. Results: At the time of diagnosis, 4 tumors were classified as Stage I, 4 as Stage II, 3 as Stage III, and 6 as Stage IV. Eight patients demonstrated positive nuclear immunostaining of ER. The prognosis of patients with ER positive was significantly better (P<0.05) than that of patients with ER negative, with 1- and 5-year survival rates at 86% and 60% for ER-positive patients, and 38% and 0% for ER-negative patients, respectively. Conclusion: ER-positivity may be one of the factors associated with a worse prognosis of ACC.展开更多
Alternative splicing(AS)in the tumor biological process has provided a novel perspective on carcinogenesis.However,the clinical significance of individual AS patterns of adrenocortical carcinoma(ACC)has been underesti...Alternative splicing(AS)in the tumor biological process has provided a novel perspective on carcinogenesis.However,the clinical significance of individual AS patterns of adrenocortical carcinoma(ACC)has been underestimated,and in-depth investigations are lacking.We selected 76 ACC samples from the Cancer Genome Atlas(TCGA)SpliceSeq and SpliceAid2 databases,and 39 ACC samples from Fudan University Shanghai Cancer Center(FUSCC).Prognosis-related AS events(PASEs)and survival analysis were evaluated based on prediction models constructed by machine-learning algorithm.In total,23,984 AS events and 3,614 PASEs were detected in the patients with ACC.The predicted risk score of each patient suggested that eight PASEs groups were significantly correlated with the clinical outcomes of these patients(p<0.001).Prognostic models produced AUC values of 0.907 in all PASEs’groups.Eight splicing factors(SFs),including BAG2,CXorf56,DExD-Box Helicase 21(DDX21),HSPB1,MBNL3,MSI1,RBMXL2,and SEC31B,were identified in regulatory networks of ACC.DDX21 was identified and validated as a novel clinical promoter and therapeutic target in 115 patients with ACC from TCGA and FUSCC cohorts.In conclusion,the strict standards used in this study ensured the systematic discovery of profiles of AS events using genome-wide cohorts.Our findings contribute to a comprehensive understanding of the landscape and underlying mechanism of AS,providing valuable insights into the potential usages of DDX21 for predict-ing prognosis for patients with ACC.展开更多
Introduction: Adrenal tumours (AT) are commonly encountered in clinical practice. For any patient presenting an adrenal mass, there are two crucial points to consider: is the adrenal mass malignant and is it hormonall...Introduction: Adrenal tumours (AT) are commonly encountered in clinical practice. For any patient presenting an adrenal mass, there are two crucial points to consider: is the adrenal mass malignant and is it hormonally active? The objective of our study was to evaluate the epidemiological, diagnostic, therapeutic, and evolutionary aspects of AT in a developing country. Methodology: We conducted a retrospective study spanning 17 years (from January 2005 to October 2022) in four Dakar hospital services. We included all patient medical records with explored AT. Results: AT was diagnosed in 35 patients who had a mean age of 36.62 years (range: 12-79), and a female predominance (26 women to 9 men). Among these patients, 17.2 % had incidental AT. Hypertension was the commonest presenting symptom in 27 patients (77%), which was associated with Mnards triad in 14 patients (40%). Abdominal pain was reported by 20 patients (57%), and 14 presented with an abdominal mass. Among the patients, 29 had functional AT: 22 with pheochromocytoma, 5 with primary hyperaldosteronism, and 2 with hypercortisolism. Imaging evaluations revealed that 33 patients had unilateral AT and one had bilateral AT. Tumour sizes varied, 5 patients had tumours 10 cm. Two patients presented with metastases. The therapeutic approach involved adrenalectomy in 32 patients. Perioperative complications were observed in five patients, including haemorrhage (two patients), hypotension (two cases), and hypertensive crisis (one case). One patient with bilateral pheochromocytoma developed acute adrenal insufficiency postoperatively, followed by abdominal herniation. Histopathological examination confirmed pheochromocytoma in 21 patients, Conns adenoma in 5, adrenocortical carcinoma in 3, cortisol adenoma in 2, and adrenal cyst in 1 patient. Non-functional AT included adrenocortical carcinoma (three patients), adrenal cyst (one patient), and pheochromocytoma (two patients). After a one-year follow-up, 29 patients with benign tumours had favourable outcomes, while death occurred within six months of diagnosis in five cases. A patient who had bilateral pheochromocytomas, with complications, died before surgery. Conclusion: In our practice, AT are predominantly pheochromocytomas, being typically diagnosed when they become symptomatic large tumours and, due to this large size, laparotomy is the preferred surgical approach. Unfortunately, the prognosis for malignant tumours is poor.展开更多
基金Supporting Project Number(RSPD2023R725)King Saud University,Riyadh,Saud Arabia。
文摘N6-methyladenosine methylation(m6A)is a common type of epigenetic alteration that prominently affects the prognosis of tumor patients.However,it is unknown how the m6A regulator affects the tumor microenvironment(TME)cell infiltration in adrenocortical carcinoma(ACC)and how it affects the prognosis of ACC patients yet.The m6A alteration patterns of 112 ACC patients were evaluated,furthermore,the association with immune infiltration cell features was investigated.The unsupervised clustering method was applied to typify the m6A alteration patterns of ACC patients.The principal component analysis(PCA)technique was taken to create the m6A score to assess the alteration pattern in specific malignancies.We found two independent patterns of m6A alteration in ACC patients.The TME cell infiltration features were significantly in accordance with phenotypes of tumor immune-inflamed and immune desert in both patterns.The m6Ascore also served as an independent predictive factor in ACC patients.The somatic copy number variation(CNV)and patients prognosis can be predicted by m6A alteration patterns.Moreover,the ACC patients with high m6A scores had better overall survival(OS)and higher efficiency in immune checkpoint blockade therapy.Our work demonstrated the significance of m6A alteration to the ACC patients immunotherapy.The individual m6A alteration patterns analysis might contribute to ACC patients prognosis prediction and immunotherapy choice.
基金Supported by Foundation of Hunan Educational Committee,No.16A188Foundation of Hengyang Science and Technology Committee,No.2018KJ135.
文摘BACKGROUND Adrenocortical carcinoma(ACC)is a rare malignant epithelial tumor originating from adrenocortical cells that carries a very poor prognosis.Metastatic or inoperable diseases are often considered incurable,and treatment remains a challenge.Especially for advanced cases such as ACC complicated with renal venous cancer thrombus,there are few cumulative cases in the literature.CASE SUMMARY The patient in this case was a 39-year-old middle-aged male who was admitted to the hospital for more than half a month due to dizziness and chest tightness.Computed tomography(CT)findings after admission revealed a left retroperitoneal malignant space-occupying lesion,but the origin of the formation of the left renal vein cancer thrombus remained to be determined.It was speculated that it originated from the left adrenal gland,perhaps a retroperitoneal source,and left adrenal mass+left nephrectomy+left renal vein tumor thrombus removal+angioplasty were performed under general anesthesia.Postoperative pathology results indicated a diagnosis of ACC.Postoperative steroid therapy was administered.At 3 mo after surgery,abdominal CT reexamination revealed multiple enlarged retroperitoneal lymph nodes and multiple low-density shadows in the liver,and palliative radiotherapy and mitotane were administered,considering the possibility of metastasis.The patient is currently being followed up.CONCLUSION ACC is a highly malignant tumor.Even if the tumor is removed surgically,there is still the possibility of recurrence.Postoperative mitotane and adjuvant chemoradiotherapy have certain benefits for patients,but they cannot fully offset the poor prognosis of this disease.
文摘BACKGROUND Adrenocortical carcinoma(ACC)is a rare and highly invasive endocrine malignant tumor with a poor prognosis.Although surgical resection is the main treatment for ACC,postoperative recurrence and metastasis have become the important factors of death.Transcatheter arterial chemoembolization(TACE)is an important option for the treatment of advanced ACC with liver metastasis.However,due to the small number of patients treated for ACC,the safety of the operation is not completely clear and needs to be further studied.CASE SUMMARY A 47-year-old patient with ACC after surgery was admitted for reexamination by abdominal computerized tomography suggesting liver metastasis.Because the patient expressed reluctance to undergo surgery again,we treated her with TACE for the liver lesions.After treatment,symptoms of acute adrenal dysfunction such as decreased blood pressure,anorexia,and fatigue appeared,which were relieved after hydrocortisone treatment.To date,the patient's liver lesion is well controlled and no other metastases are observed.CONCLUSION We report a rare case of acute adrenal hypofunction after TACE.Glucocorticoid supplementation can alleviate the symptoms.
文摘BACKGROUND Adrenocortical carcinoma(ACC),the second most aggressive malignant tumor,lacks epidemiological data worldwide;therefore,every new case can improve the understanding of the pathology and treatment of this malignancy.CASE SUMMARY We present the case of a 66-year-old Caucasian woman with a giant androgenproducing ACC(21 cm×17 cm×12 cm;2100 g),without metastases,which unusually presented with an acute onset of atrial flutter and congestive heart failure.The cardiac complications observed in our case support the hypothesis that androgen excess in women is a cardiovascular risk factor.Androgen excess in women can be a rare cause of reversible dilated cardiomyopathy,therefore a comprehensive approach to the patient is essential to improve the recognition of androgen-secreting ACC.The atrial flutter was remitted after initiation of drug treatment during admission.The severe heart failure was totally remitted at 6 mo after radical open surgery to remove the giant ACC.CONCLUSION Radical open surgery to remove a giant androgen-producing ACC was the firstline treatment to cure the excess of androgen,which determined the total remission of cardiac complications at 6 mo after surgery in the women of this case report.
基金Special fund for basic scientific research business expenses of the Central Public Welfare Research Institute of the Chinese Academy of Medical Sciences(No.2019PT330003)。
文摘Objective:To explore the gene biomarkers related to the diagnosis and prognosis of adrenocortical carcinoma(ACC)by bioinformatics.Methods:GEPIA online analysis tool was used to screen differentially expressed genes for sequencing data from patients with ACC and normal adrenal cortex.The overall survival rate and disease-free survival method were used to conduct batch survival analysis on differentially expressed genes,and the top 100 genes with HR values calculated by the two methods were obtained respectively.The intersection method is used to obtain core genes that play a key role in both overall survival and disease-free survival.GEPIA online analysis tool was used again to explore the relationship between the above-mentioned survival-related genes and the pathological stage of ACC.Use UALCAN online analysis tool to verify the survival-related genes again and draw the Kaplan-Meier survival curve.Finally,GSE33371 chip dataset of the GEO database was used to evaluate the diagnostic value of the above-mentioned survival-related genes.Results:514 differentially expressed genes were obtained by limma method.Batch analysis of differential genes was performed to obtain the top 100 genes most related to overall survival and disease-free survival,of which 13 genes were closely related to overall survival and disease-free survival.9 hub genes including TP73,SNHG1,PDE6D,GPC2,SUV39H2,HELLS,CLK2,COPS7B and CEP164 were finally obtained by exploring the relationship between their expression levels and pathological stage and resurvival analysis.At the same time,the results of ROC analysis suggest that the above hub genes have high diagnostic value for patients with adrenocortical carcinoma.Conclusion:By using GEPIA,UALCAN and the gene chip retrieved from GEO database,combined with the bioinformatics method,we analyzed and verified the new biomarkers that can be used to evaluate the prognosis of patients with ACC and to differential diagnosis of ACC,and provided the theoretical support of bioinformatics for exploring the occurrence,development of molecular mechanism and potential target of treatment of ACC.
文摘ACC （adrenocortical carcinoma） is an uncommon and aggressive malignancy with an overall five year survival rate from less than 50%. The majority of cases are accidentally diagnosed by imaging studies done for other reasons, so they are often diagnosed in the later stages. Due to the rarity of both neoplasms, the synchronous presentation and the lack of effective therapeutic options; we report a case concerning about a female patient with adrenal cancer with a low grade solitary fibrous tumor who underwent surgery, adjuvant radiotherapy and chemotherapy with good response.
文摘Myxoid change in adrenocortical carcinoma is a rare phenomenon,and no case has been reported in Chinese population to date.We report here a case of myxoid adrenocortical carcinoma presenting as Cushing syndrome with an immunohistochemical study of the tumor and discuss the diagnosis with reference to the current literature.
基金This work was supported by the Natural Science Foundation of China(81572514,U1301221,81402106,81272808,81825016)the Natural Science Foundation of Guangdong,China(2016A030313244)Grant[2013]163 from Key Laboratory of Malignant Tumor Molecular Mechanism and Translational Medicine of Guangzhou Bureau of Science and Information Technology,Grant KLB09001 from the Key Laboratory of Malignant Tumor Gene Regulation and Target Therapy of Guangdong Higher Education Institutes,and grants from the Guangdong Science and Technology Department(2015B050501004,2017B020227007).
文摘Background:Clinical outcome of adrenocortical carcinoma(ACC)varies because of its heterogeneous nature and reliable prognostic prediction model for adult ACC patients is limited.The objective of this study was to develop and externally validate a nomogram for overall survival(OS)prediction in adult patients with ACC after surgery.Methods:Based on the data from the Surveillance Epidemiology,and End Results(SEER)database,adults patients diagnosed with ACC between January 1988 and December 2015 were identified and classified into a training set,comprised of 404 patients diagnosed between January 2007 and December 2015,and an internal validation set,com-prised of 318 patients diagnosed between January 1988 and December 2006.The endpoint of this study was OS.The nomogram was developed using a multivariate Cox proportional hazards regression algorithm in the training set and its performance was evaluated in terms of its discriminative ability,calibration,and clinical usefulness.The nomogram was then validated using the internal SEER validation,also externally validated using the Cancer Genome Atlas set(TCGA,82 patients diagnosed between 1998 and 2012)and a Chinese multicenter cohort dataset(82 patients diag-nosed between December 2002 and May 2018),respectively.Results:Age at diagnosis,T stage,N stage,and M stage were identified as independent predictors for OS.A nomo-gram incorporating these four predictors was constructed using the training set and demonstrated good calibration and discrimination(C-index 95%confidence interval[CI],0.715[0.679-0.751]),which was validated in the internal validation set(C-index[95%CI],0.672[0.637-0.707]),the TCGA set(C-index[95%CI],0.810[0.732-0.888])and the Chi-nese multicenter set(C-index[95%CI],0.726[0.633-0.819]),respectively.Encouragingly,the nomogram was able to successfully distinguished patients with a high-risk of mortality in all enrolled patients and in the subgroup analyses.Decision curve analysis indicated that the nomogram was clinically useful and applicable.Conclusions:The study presents a nomogram that incorporates clinicopathological predictors,which can accurately predict the OS of adult ACC patients after surgery.This model and the corresponding risk classification system have the potential to guide therapy decisions after surgery.
文摘Objective: Adrenocortical carcinoma (ACC) is a rare but highly malignant tumor, and its diagnosis is mostly delayed and prognosis is poor. We report estrogen receptor (ER) expression in this tumor and our clinical experiences with 17 ACC cases. Methods: The data of the 17 patients (9 females and 8 males, age range from 16 to 69 years, mean age of 42.6 years) with ACC were reviewed, and symptoms, diagnostic procedures, treatment, and results of follow-up were evaluated. Immunohistochemistry was used to detect ER expression in tumor samples from the 17 patients. Results: At the time of diagnosis, 4 tumors were classified as Stage I, 4 as Stage II, 3 as Stage III, and 6 as Stage IV. Eight patients demonstrated positive nuclear immunostaining of ER. The prognosis of patients with ER positive was significantly better (P<0.05) than that of patients with ER negative, with 1- and 5-year survival rates at 86% and 60% for ER-positive patients, and 38% and 0% for ER-negative patients, respectively. Conclusion: ER-positivity may be one of the factors associated with a worse prognosis of ACC.
基金supported by National Key Research and Development Program of China(No.2019YFC1316000)Fuqing Scholar Student Scientific Research Program of Shanghai Medical College,Fudan University(No.FQXZ202112B)+1 种基金Natural Science Foundation of Shanghai(No.20ZR1413100)Shanghai Municipal Health Bureau(No.2020CXJQ03).
文摘Alternative splicing(AS)in the tumor biological process has provided a novel perspective on carcinogenesis.However,the clinical significance of individual AS patterns of adrenocortical carcinoma(ACC)has been underestimated,and in-depth investigations are lacking.We selected 76 ACC samples from the Cancer Genome Atlas(TCGA)SpliceSeq and SpliceAid2 databases,and 39 ACC samples from Fudan University Shanghai Cancer Center(FUSCC).Prognosis-related AS events(PASEs)and survival analysis were evaluated based on prediction models constructed by machine-learning algorithm.In total,23,984 AS events and 3,614 PASEs were detected in the patients with ACC.The predicted risk score of each patient suggested that eight PASEs groups were significantly correlated with the clinical outcomes of these patients(p<0.001).Prognostic models produced AUC values of 0.907 in all PASEs’groups.Eight splicing factors(SFs),including BAG2,CXorf56,DExD-Box Helicase 21(DDX21),HSPB1,MBNL3,MSI1,RBMXL2,and SEC31B,were identified in regulatory networks of ACC.DDX21 was identified and validated as a novel clinical promoter and therapeutic target in 115 patients with ACC from TCGA and FUSCC cohorts.In conclusion,the strict standards used in this study ensured the systematic discovery of profiles of AS events using genome-wide cohorts.Our findings contribute to a comprehensive understanding of the landscape and underlying mechanism of AS,providing valuable insights into the potential usages of DDX21 for predict-ing prognosis for patients with ACC.
文摘Introduction: Adrenal tumours (AT) are commonly encountered in clinical practice. For any patient presenting an adrenal mass, there are two crucial points to consider: is the adrenal mass malignant and is it hormonally active? The objective of our study was to evaluate the epidemiological, diagnostic, therapeutic, and evolutionary aspects of AT in a developing country. Methodology: We conducted a retrospective study spanning 17 years (from January 2005 to October 2022) in four Dakar hospital services. We included all patient medical records with explored AT. Results: AT was diagnosed in 35 patients who had a mean age of 36.62 years (range: 12-79), and a female predominance (26 women to 9 men). Among these patients, 17.2 % had incidental AT. Hypertension was the commonest presenting symptom in 27 patients (77%), which was associated with Mnards triad in 14 patients (40%). Abdominal pain was reported by 20 patients (57%), and 14 presented with an abdominal mass. Among the patients, 29 had functional AT: 22 with pheochromocytoma, 5 with primary hyperaldosteronism, and 2 with hypercortisolism. Imaging evaluations revealed that 33 patients had unilateral AT and one had bilateral AT. Tumour sizes varied, 5 patients had tumours 10 cm. Two patients presented with metastases. The therapeutic approach involved adrenalectomy in 32 patients. Perioperative complications were observed in five patients, including haemorrhage (two patients), hypotension (two cases), and hypertensive crisis (one case). One patient with bilateral pheochromocytoma developed acute adrenal insufficiency postoperatively, followed by abdominal herniation. Histopathological examination confirmed pheochromocytoma in 21 patients, Conns adenoma in 5, adrenocortical carcinoma in 3, cortisol adenoma in 2, and adrenal cyst in 1 patient. Non-functional AT included adrenocortical carcinoma (three patients), adrenal cyst (one patient), and pheochromocytoma (two patients). After a one-year follow-up, 29 patients with benign tumours had favourable outcomes, while death occurred within six months of diagnosis in five cases. A patient who had bilateral pheochromocytomas, with complications, died before surgery. Conclusion: In our practice, AT are predominantly pheochromocytomas, being typically diagnosed when they become symptomatic large tumours and, due to this large size, laparotomy is the preferred surgical approach. Unfortunately, the prognosis for malignant tumours is poor.
