ABSTRACT
We present a case of adrenal cortical carcinoma (ACC) with tumor thrombus involving the inferior vena cava (IVC) and right atrium in a morbidly obese, middle-aged female. ACC is a rare type of cancer with a poor outcome. Most cases present with metastasis at the time of initial presentation. This patient presented with breathing difficulty and flank pain. With endocrinology evaluation and collaborative effort of multidisciplinary teams, the patient successfully underwent staged procedures of atrial clot evacuation after thoracotomy with cardiopulmonary bypass (CBP) along with inferior vena cava thrombectomy (IVCT) and left radical nephroadrenalectomy. The two staged procedure reduces the mortality when compared with a single staged procedure. The patient underwent atrial clot evacuation, performed by cardiothoracic surgery team under CBP and deployment of IVC filter by interventional radiologist as a first staged procedure. At six months of follow-up, the patient had locoregional disease spread but with good functional status. This case report highlights that even a high-volume disease, with proper planning by an experienced surgical team, can be operated successfully with an acceptable post-surgery quality of life for the patients.
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Objective To investigate the effects of preoperative 8 am plasma total cortisol level (PTC) on the prognosis of adrenocortical carcinoma (ACC).Methods A total of 44 patients from January 2009 to October 2017 with ACC were included,PTC level within one week before surgery,clinical and pathological data were collected.The mean age of the patients was (45.2 ± 15.2) years.There were 27 females and 17 males.The mean tumor diameter was (63.6 ± 20.7) mm.There were 20 cases with functional ACC,in which 18 patients presented symptoms associated with Cushing syndrome.According to ENSAT stage,9 patients were staged as stage Ⅰ,and 35 were staged as stage Ⅱ.The median pre-operative 8 am PTC was 572.6 nmol/L,range from 89.3 to 1 118.7 nmol/L.Open adrenalectomy was employed in 23 patients and laparoscopic approach in 21 patients.The optimal cut-off value of pre-operative PTC was determined by receiver operating characteristic curve (ROC) and patients were divided into two groups with high PTC and low PTC values.Kaplan-Meier method was used to draw the survival curve,univariate and multivariate Cox regression were used to explore factors influencing the prognosis of ACC patients.Results The median follow-up duration was 42 months,range from 3-104 months.The optimal cut-off value was determined as 476.2 nmol/L,baseline and clinic-pathologic characteristics differences between two groups were not statistical significant (P ＞ 0.05).The overall survival (OS) in the high PTC group was shorter than that in the low PTC group [median 18months (20-104 months) vs.40 months (5-98 months),P =0.014],and the recurrence free survival (RFS) of patients in the high PTC group was also shorter than that in the low PTC [median 26 months (0-104 months) vs.50 months (5-98 months),P =0.028).In univariate analysis,age,gender,tumor stage,preoperative PTC,and symptoms were correlated with postoperative OS and RFS (P ＜ 0.05).After adjusting for age,gender,tumor stage,symptoms,multivariate Cox regression showed that pre-operative high PTC was an independent prognostic factor associated with a decreased OS (HR =2.086,95 ％ CI 1.495-2.287,P =0.014) and decreased RFS (HR =2.234,95％ CI 1.546-2.334,P =0.045).Conclusion The preoperative morning PTC is an independent risk factor for the prognosis of ACC.
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Objective To explore the effect of adriamycin on the characteristics of colony derived from human adrenal cortical carcinoma cells (ACC) SW-13.Methods Treatment with Adriamycin (ADM) was used in BALB/c-nude mouse tumor xenograft model established using the ACC cell line SW-13.The characteristic of colony was assessed for the formation rates,the percentagc of three colony types and growth curve of single cell.Hoechst33342 dyeing test was used to test drug resistance.Results The Single-cell colony formation rate of experimental group were significantly higher than control group (P ＜ 0.05),and the holoclone percentage of experimental group were significantly higher than control group (P ＜ 0.05).In the Hoechst33342 dyeing tcst,the fluorescence intensity of control was higher than experimental group.Conclusion The treatment of ADM in vivo is beneficial for the colony formations of ACC cell and the formations rate of holoclone,and can improve the ability of drug resistance of ACC cell SW-13.
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[Summary]_ This is a pedigree of multiple endocrine neoplasia type 1(MEN1). The proband pursuit medical assistance because of hypertension and weakness. Adrenal cortical carcinoma with possible Cushing's syndrome was diagnosed after a series of tests. During this process, the proband was found to have hypercalcemia, and he was diagnosed as primary hyperparathyroidism. Adrenal carcinoma plus primary hyperparathyroidism suggested MEN1, which was confirmed by MEN1 gene 400_401insC mutation. Pedigree investigation found six additional patients, including one with high parathyroid hormone level and two without clinical evidence of any MEN1 diseases. The proband died of metastatic malignancy 7 months after diagnosis while the other 3 patients with clinically confirmed MEN1 tumor responded well to surgery, including one with adrenal cortical carcinoma.
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Background: Adrenal cortical carcinoma (ACC) is an uncommon cancer. Materials and Methods: The author performed a literature review on the reports of ACC in Thailand in order to summarize the characteristics of this rare cancer among Thai patients. Results: According to this study, there have been at least seven reports in the literature of nine individual cases of ACC, of which none was lethal directly to ACC. Conclusions: Here, the ACC is sporadically reported in Thailand. The diagnosis of ACC is usually by detection adrenal mass using imaging technique and the confirmation for the nature of cancer is usually by histopathology. Nevertheless, among the Thai population, the prognostic outcome is usually good.
Subject(s)
Adolescent , Adrenocortical Carcinoma/diagnosis , Adrenocortical Carcinoma/epidemiology , Adrenocortical Carcinoma/pathology , Adult , Aged , Female , Humans , Male , Middle Aged , Thailand/epidemiology , Young AdultABSTRACT
An estrogen producing adrenocortical carcinoma is a rare, highly malignant tumor. Fewer than 100 cases of feminizing adrenocortical carcinomas in men have been reported. These tumors are usually large in size and show high levels of serum total estrogen and estradiol. Recently, two cases of estrogen producing adrenocortical carcinoma were experienced by our selves during in year. A unilateral adrenalectomy, with mass excision, was performed in both patients, with an ipsilateral nephrectomy performed in just one. Mitotane or cisplatin based adjuvant chemotherapy was also performed.
Subject(s)
Humans , Male , Adrenalectomy , Adrenocortical Carcinoma , Chemotherapy, Adjuvant , Cisplatin , Drug Therapy , Estradiol , Estrogens , Mitotane , NephrectomyABSTRACT
An adrenal cortical carcinoma is a rare malignancy associated with poor prognosis. On diagnosis, most patients present with large tumor masses, which are often detected at an advanced stage. The most effective treatment is a complete resection, which is the only curative treatment for adrenal cortical carcinomas. The most important prognostic factor is a successful resection of the primary tumor, as long as it is low-grade and has not spread to distant sites. However, with advanced adrenal cortical carcinomas, with distant metastasis, there is no strict effective treatment program, and the prognosis is poor. The case of a 50-year-old female patient, presenting with an adrenal cortical carcinoma and Cushing's syndrome, who had a long-term survival of 78 months, is reported. The mass was completely resected on diagnosis, but 16 months later liver metastasis was discovered. She had received chemotherapy, with cisplatin, etoposide and bleomycin, for the liver metastasis for a period of 15 months, but with no response, furthermore, the size had increased after 10 months. Afterward, she received her 10th session of intrahepatic artery cisplatin chemotherapy and her 3rd hepatic artery embolization. Although the patient had a large degree of liver metastasis, this was tolerated. The tumor mass is presently not aggravated, and she still survives after 78 months.
Subject(s)
Female , Humans , Middle Aged , Adrenocortical Carcinoma , Arteries , Bleomycin , Cisplatin , Cushing Syndrome , Diagnosis , Drug Therapy , Etoposide , Fibrinogen , Hepatic Artery , Liver , Neoplasm Metastasis , PrognosisABSTRACT
A 60 year-old woman presented with clinical signs and symptoms of liver abscess. She had a cystic mass on the right lobe of the liver. Liver needle aspiration was performed and anchovy-paste like material was obtained. She recovered after treatment with oral metronidazole even though investigations for Entamoeba histolytica were negative. Two and a half years later, she returned with the same clinical signs and symptoms. Liver needle aspirations were performed many times and the same anchovy-paste like material was obtained but no definite diagnosis was made. Finally, laparotomy showed an extrahepatic retroperitoneal cystic mass of the right adrenal gland adjacent to the liver. Intravenous pyelography, adrenal CT scan and adrenal angiography were performed. The tumor was removed and the final histopathological diagnosis was adrenal cortical carcinoma.
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Adrenal cortical carcinoma is a rare disease both in adults and in children. Most of these tumors are functional, especially in children, producing endocrine syndromes such as virilization, Cushing syndrome, hyperaldosteronism or feminization. We experienced a case of adrenal cortical carcinoma in a 7yr old boy who showed features of virilization such as rapid growth rate, penile enlargement, and pubic hair. This case was diagnosed with typical hormonal findings and abdominal MRI and confirmed by pathologic findings. He was successfully treated by total left adrenalectomy and has been followed up without problem over eight months.
Subject(s)
Adult , Child , Humans , Male , Adrenalectomy , Adrenocortical Carcinoma , Cushing Syndrome , Feminization , Hair , Hyperaldosteronism , Magnetic Resonance Imaging , Rare Diseases , VirilismABSTRACT
The adrenal cortical carcinoma is a rare cancer with an estimated incidence of about 1 case per 1,700,000 population. Further development of the tumor thrombi invasion of the inferior vena cava is very rare. The adrenal cortical carcinoma has poor prognosis due to delayed onset of symptoms and signs with regional and metastatic diseases are about 70M at the time of diagnosis. The diagnosis is made by hormonal and imaging studies. Adrenal cortical carcinoma is slightly more frequent in female and hormonally non-functioning tumor is more frequent. Traditionally, surgery and mitotane chemotherapy are known as a valuable therapeutic modality. But recently usefullness of mitotane is questioned due to low response rate and complication such as gastrointestinal trouble and there is not established optimal dosage and duration of treatment. Recently, in metastatic adrenal cortical carcinoma, some literature with combined chemotherapy had tried and reported good response. But, in general, combined chemotherapy has known as ineffective. We experienced and report adrenal cortical carcinoma with inferior vena caval invasion which is diagnosed by computed tomography and magnetic resonance imaging, and we treated surgery and postoperative chemotherapy.
Subject(s)
Female , Humans , Adrenocortical Carcinoma , Diagnosis , Drug Therapy , Incidence , Magnetic Resonance Imaging , Mitotane , Prognosis , Vena Cava, InferiorABSTRACT
Fine needle aspiration(FNA) biopsy has become the procedure of choice for initial diagnosis of adrenal masses. However, there have been relatively few reports discussing the FNA cytologic features of adrenal cortical carcinoma. Recently, we experienced a case of FNA cytology of bilateral adrenal cortical carcinoma in a 61-year old man. The smear revealed loosely cohesive pleomorphic tumor cells with hemorrhagic and necrotic background. The tumor cells showed oval to spindle hyperchromatic nuclei and prominent nucleoli with frequent mitotic figures. The cytoplasm of tumor cells was relatively abundant and sometimes vacuolated. These cytologic findings were interpreted as an adrenal cortical carcinoma, undifferentiated pattern.
Subject(s)
Humans , Middle Aged , Adrenocortical Carcinoma , Biopsy , Biopsy, Fine-Needle , Cytoplasm , Diagnosis , NeedlesABSTRACT
Adrenal cortical carcinoma is classified clinically as functioning and nonfunctioning and the latter is though to be extremely rare and causes generally mass effect or symptoms due to metastasis because of insufficient steroid secretion. We report a case of nonfunctioning adrenal cortical carcinoma, which is thought to be heterotopic origin.
Subject(s)
Adrenocortical Carcinoma , Neoplasm MetastasisABSTRACT
Nonfunctioning adrenal cortical carcinoma is extremely rare, which probably merely secreted insufficient steroid to cause signs and symptoms. We experienced a case of nonfunctioning adrenal cortical carcinoma without metastatic evidence, treated by only adrenalectomy.