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
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
Adrenocortical carcinoma(ACC)is a rare and highly aggressive endocrine malignancy with limited therapeutic options and a substantial risk of postoperative recurrence.Mitotane remains the only US Food and Drug Administ...Adrenocortical carcinoma(ACC)is a rare and highly aggressive endocrine malignancy with limited therapeutic options and a substantial risk of postoperative recurrence.Mitotane remains the only US Food and Drug Administration(USFDA)and the European Medicines Agency(EMA)approved agent specifically for ACC.Its antitumor activity involves disruption of cholesterol trafficking,inhibition of steroidogenic enzymes,and mitochondrial dysfunction,leading to selective cytotoxicity in adrenocortical cells.Current evidence indicates that maintaining therapeutic plasma concentrations between 14 mg/L and 20 mg/L is critical for clinical benefit,underscoring the importance of therapeutic drug monitoring.Mitotane is used in both adjuvant and advanced disease settings;however,its highly variable pharmacokinetics,broad endocrine disruptions,and frequent gastrointestinal and neurological toxicities require individualized dosing strategies and comprehensive supportive care.This review summarizes recent advances in the understanding of mitotane’s molecular mechanisms,pharmacogenetic determinants,clinical efficacy,and adverse-effect profile,with an emphasis on laboratory monitoring and practical considerations for optimizing treatment in ACC.展开更多
This review comprehensively assesses the treatment modalities and prognostic outcomes of pediatric adrenocortical carcinoma(ACC),a rare malignancy with significant clinical challenges.Through a systematic literature s...This review comprehensively assesses the treatment modalities and prognostic outcomes of pediatric adrenocortical carcinoma(ACC),a rare malignancy with significant clinical challenges.Through a systematic literature search of Pub-Med using the term"childhood adrenocortical carcinoma,"supplemented by EndNote-assisted duplicate removal and manual verification,we synthesize current evidence on the clinical characteristics,staging criteria,pathological diagnosis,and imaging features of this disease.The analysis highlights that surgical resection remains the cornerstone of treatment,particularly for stage Ⅰ/Ⅱ patients who may achieve long-term survival without recurrence.However,prognosis deteriorates markedly in advanced stages,with stage Ⅳ patients exhibiting distant metastases facing poor outcomes.Adjuvant therapies such as postoperative chemotherapy and mitotane are critical to improving survival,yet further research is needed to optimize therapeutic strategies.These findings aim to guide clinicians in tailoring diagnostic and management approaches for pediatric ACC.展开更多
We report a case of pituitary-dependent hyperadrenocorticism in a 10-year-old,female,terrier dog.The animal was admitted due to polyphagia,weight gain,polyuria,polydipsia,hair loss,exercise intolerance and panting at ...We report a case of pituitary-dependent hyperadrenocorticism in a 10-year-old,female,terrier dog.The animal was admitted due to polyphagia,weight gain,polyuria,polydipsia,hair loss,exercise intolerance and panting at rest.On physical examination,abdominal distention,truncal and bilaterally symmetric alopecia,thin hypotonic skin,comedones,bruising,hyperpigmentation and calcinosis cutis on the dorsal midline were observed.Hematologic investigations showed stress leukogram,high serum alkaline phosphatase activity,mild to moderate alanine aminotransferase activity,hypercholesterolemia,hypertriglyceridemia and hyperglycemia.Mild generalized interstitial lung patterns and hepatomegaly were detected in the radiographs.Bilaterally symmetric normal-sized adrenal glands were also diagnosed in ultrasonography.Diagnosis of pituitary-dependent hyperadrenocorticism was confirmed with low-dose dexamethasone suppression test.The dog was successfully treated with mitotane.展开更多
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.
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.
基金supported by the National Natural Science Foundation of China(Nos.82403817 and 82170797)Shanghai Municipal Health Commission,China(Nos.20224Y0088,2022YQ021 and 202440098)+1 种基金Shanghai Municipal Science and Technology Commission,China(No.24QA2705200)Shanghai Shenkang Hospital Development Center,China(Nos.SHDC2024CRI081,SHDC22022301,SHDC22025304 and SHDC2025CCS022).
文摘Adrenocortical carcinoma(ACC)is a rare and highly aggressive endocrine malignancy with limited therapeutic options and a substantial risk of postoperative recurrence.Mitotane remains the only US Food and Drug Administration(USFDA)and the European Medicines Agency(EMA)approved agent specifically for ACC.Its antitumor activity involves disruption of cholesterol trafficking,inhibition of steroidogenic enzymes,and mitochondrial dysfunction,leading to selective cytotoxicity in adrenocortical cells.Current evidence indicates that maintaining therapeutic plasma concentrations between 14 mg/L and 20 mg/L is critical for clinical benefit,underscoring the importance of therapeutic drug monitoring.Mitotane is used in both adjuvant and advanced disease settings;however,its highly variable pharmacokinetics,broad endocrine disruptions,and frequent gastrointestinal and neurological toxicities require individualized dosing strategies and comprehensive supportive care.This review summarizes recent advances in the understanding of mitotane’s molecular mechanisms,pharmacogenetic determinants,clinical efficacy,and adverse-effect profile,with an emphasis on laboratory monitoring and practical considerations for optimizing treatment in ACC.
文摘This review comprehensively assesses the treatment modalities and prognostic outcomes of pediatric adrenocortical carcinoma(ACC),a rare malignancy with significant clinical challenges.Through a systematic literature search of Pub-Med using the term"childhood adrenocortical carcinoma,"supplemented by EndNote-assisted duplicate removal and manual verification,we synthesize current evidence on the clinical characteristics,staging criteria,pathological diagnosis,and imaging features of this disease.The analysis highlights that surgical resection remains the cornerstone of treatment,particularly for stage Ⅰ/Ⅱ patients who may achieve long-term survival without recurrence.However,prognosis deteriorates markedly in advanced stages,with stage Ⅳ patients exhibiting distant metastases facing poor outcomes.Adjuvant therapies such as postoperative chemotherapy and mitotane are critical to improving survival,yet further research is needed to optimize therapeutic strategies.These findings aim to guide clinicians in tailoring diagnostic and management approaches for pediatric ACC.
基金Support by Shahid Bahonar University of Kerman,Research Council,Kerman,Iran(Grant No.92-GR-VS-02).
文摘We report a case of pituitary-dependent hyperadrenocorticism in a 10-year-old,female,terrier dog.The animal was admitted due to polyphagia,weight gain,polyuria,polydipsia,hair loss,exercise intolerance and panting at rest.On physical examination,abdominal distention,truncal and bilaterally symmetric alopecia,thin hypotonic skin,comedones,bruising,hyperpigmentation and calcinosis cutis on the dorsal midline were observed.Hematologic investigations showed stress leukogram,high serum alkaline phosphatase activity,mild to moderate alanine aminotransferase activity,hypercholesterolemia,hypertriglyceridemia and hyperglycemia.Mild generalized interstitial lung patterns and hepatomegaly were detected in the radiographs.Bilaterally symmetric normal-sized adrenal glands were also diagnosed in ultrasonography.Diagnosis of pituitary-dependent hyperadrenocorticism was confirmed with low-dose dexamethasone suppression test.The dog was successfully treated with mitotane.
