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1.
Arch Esp Urol ; 74(8): 782-789, 2021 Oct.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-34605409

RESUMO

OBJECTIVE: Adrenocortical carcinoma (ACC) is a rare and heterogeneous disease, with challenging management and poor prognosis. Surgery withcurative intent is the preferred treatment option for localized disease, with a reported 5-year survival rate of 55% for complete resections. However, owing to the high risk of recurrence there is a need for adjuvant therapies, such as mitotane, an adrenolytic drug, or irradiation, while in advanced disease the standard of careis a combined chemotherapy scheme. The aim of this study was to report our experience in the surgical management and outcomes of ACC patients. MATERIALS AND METHODS: A retrospective observational study was performed in a cohort of ACC patients who had undergone surgical resection (open or laparoscopic approach) and were followed up at our tertiary hospital. Patients with localized or locally advanced disease were included in the analysis. All medical records, including clinical, surgical, pathologic, and follow-updata, were collected and analyzed. RESULTS: A total of 19 ACC patients were managed at our center between August 1990 and August 2013. The median age at diagnosis was 50.5 years (range 19-72), and most patients were males. Abdominal pain was the most common clinical presentation (n=9,47.4%). Abdominal contrast-enhanced computed tomography (CT) was performed in all cases. Only 3 tumors (15.8%) were functional and most were stage II at diagnosis (n=9, 47.4%). No patient presented metastasis. Of the 19 patients, 18 (94.7%) under went surgerywith curative intent, while one (5.3%) received adjuvant radiotherapy (ART). The open approach was used in 17 patients (89.5%), while the remaining 2 (10.5%) underwent laparoscopy. Postoperative complications occurred in 8 patients (42.1%); none were of grade IV or V. Median follow-up was 66 months (range 3-312). The majority of patients (n=15, 78.9%) were disease free with surgery alone. None received adjuvant mitotane therapy (AMT). Four patients (21%) experienced metachronous metastases and 3 (15.8%) local recurrence after a median time of 10.5 months (range 2-60) and 9.3 months (range 1.5-30), respectively. The 5-year overall survival rate was 47.4%. CONCLUSIONS: Our findings confirm both the unpredictable nature of ACC and the accepted primary role of surgery. The use of adjuvant therapy was less frequent in this series than is supported currently. However, a multidiscipinary approach should be the initial step in the management of this rare malignancy.


Assuntos
Neoplasias do Córtex Suprarrenal , Carcinoma Adrenocortical , Neoplasias do Córtex Suprarrenal/cirurgia , Adrenalectomia , Carcinoma Adrenocortical/cirurgia , Adulto , Idoso , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
2.
Cir Cir ; 89(5): 664-668, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34665162

RESUMO

The adrenocortical carcinoma is rare and aggressive. It has a bimodal presentation, predominantly female, > 20% of cases will be diagnosed incidentally. 43-year-old male, with colic pain in the left flank, weight loss and intermittent fever. Computed tomography with a tumor on the left adrenal with liver metastases, block resection surgery was performed, pathological report of adrenocortical carcinoma with a 7 points of Weiss score and Ki67 40%. Adrenocortical carcinoma is a rare and aggressive neoplasm; the clinical presentation is variable. Systemic therapy is important even in patients with localized disease and independent of surgical approach.


Assuntos
Neoplasias do Córtex Suprarrenal , Carcinoma Adrenocortical , Neoplasias Hepáticas , Neoplasias do Córtex Suprarrenal/diagnóstico por imagem , Neoplasias do Córtex Suprarrenal/cirurgia , Carcinoma Adrenocortical/diagnóstico por imagem , Carcinoma Adrenocortical/cirurgia , Adulto , Feminino , Humanos , Neoplasias Hepáticas/diagnóstico por imagem , Masculino , Tomografia Computadorizada por Raios X
3.
Endocrine ; 74(3): 475-477, 2021 12.
Artigo em Inglês | MEDLINE | ID: mdl-34559356

RESUMO

Pediatric and adult adrenocortical carcinomas differ in many respects but treatment is often similar in both age groups. The Journal of Clinical Oncology recently published the results of a risk-stratified single-arm interventional trial conducted by the Children's Oncology Group in which 77 patients were treated in three different interventional cohorts. In this Point of View paper we comment on the treatment strategies adopted within the ARAR0332 trial in terms of surgery approach, duration of adjuvant therapies, and palliative chemotherapy. We focus on the differences in the treatment of pediatric ACC patients compared to the ESE/ENSAT and ESMO guidelines released in 2018 for adult patients. For example, patients in stratum 3 and 4 received 8 (instead of 6) cycles of EDP chemotherapy but 8 months (instead of 24) of mitotane adjuvant therapy. Bearing clearly in the mind that pediatric and adult ACC patients represent different settings, we wonder whether there could be some areas of intervention overlapping to constitute a continuum of disease across ages. Thus, pediatric and adult cohoperative groups should be encouraged to collaborate in order to reach common guidelines for the treatment of such a rare disease.


Assuntos
Neoplasias do Córtex Suprarrenal , Carcinoma Adrenocortical , Neoplasias do Córtex Suprarrenal/tratamento farmacológico , Neoplasias do Córtex Suprarrenal/cirurgia , Carcinoma Adrenocortical/tratamento farmacológico , Carcinoma Adrenocortical/cirurgia , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Criança , Terapia Combinada , Humanos , Mitotano/uso terapêutico
4.
J Surg Oncol ; 124(8): 1261-1271, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-34424540

RESUMO

INTRODUCTION: The standard diagnosis for adrenocortical carcinoma (ACC) is clinical diagnosis (CD) based on radiographic and biochemical studies. Biopsy diagnosis (BD) is seldom required for the suspicion of secondary malignancy. We aim to study the impact of BD in the context of underlying T1/T2 ACC on overall survival (OS) compared with CD. METHODS: National Cancer Database (NCDB) for endocrine malignancies was utilized. Only patients with non-metastatic ACC, whose method of diagnosis and local disease extension were reported, and received a surgical adrenalectomy with curative intent were included. Patients were divided by disease stage into T1/T2, T3, and T4 groups. A propensity score match was applied to those with T1/T2 disease who received CD versus BD and the Kaplan-Meier method was used to compare OS. RESULTS: In total, 4000 patients with ACC were reported in the database, 1410 met selection criteria. Eight hundred and thirty patients had T1/T2, 365 had T3, and 162 had T4 ACC. Of patients with T1/T2 ACC, 742 (89.4%) received CD versus 88 (11.6%) with BD. A propensity score was calculated per a multivariable regression model with 79 patients matched from each group. Exact matching was applied for margin status and adjuvant therapies. Kaplan-Meier analysis showed a significant difference in median OS between CD versus BD patients in the matched data set (103.89 ± 15.65 vs. 54.93 ± 8.22 months; p = 0.001). In all comers, patients with T1/T2 ACC and BD had comparable median OS to that of patients with T3 ACC (52.21 ± 9.69 vs. 36.01 ± 3.33 months; p = 0.446). CONCLUSION: BD in T1/T2 ACC could be associated with disease upstaging and worse OS outcomes.


Assuntos
Neoplasias do Córtex Suprarrenal/mortalidade , Adrenalectomia/mortalidade , Carcinoma Adrenocortical/mortalidade , Biópsia/mortalidade , Neoplasias do Córtex Suprarrenal/patologia , Neoplasias do Córtex Suprarrenal/cirurgia , Carcinoma Adrenocortical/patologia , Carcinoma Adrenocortical/cirurgia , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Pontuação de Propensão , Taxa de Sobrevida
5.
BMC Cancer ; 21(1): 961, 2021 Aug 26.
Artigo em Inglês | MEDLINE | ID: mdl-34445989

RESUMO

BACKGROUND: Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy with a poor prognosis. Given the limited treatment options, prognostic assessment of ACC is increasingly crucial. In this study, we aim to assess the correlation between preoperative serum albumin and prognosis in patients with ACC after primary resection. METHODS: We retrospectively collected and reviewed medical information about 71 ACC patients who underwent primary resection. Survival analysis was performed by Kaplan-Meier analysis with log-rank test or Breslow test. Receiver operating characteristic (ROC) curve and Jordan index was generated to explore optimal cut-off value of albumin. Univariate and multivariate analysis was conducted using Cox's hazards model. Statistical significance was defined as P < 0.05. RESULTS: Among included patients, 33 patients (46.5%) relapsed at the end of follow-up, while 39 patients (54.9%) died. The median overall survival (OS) of included patients was 17 (range 1-104) months, and median recurrence-free survival (RFS) was 10 (range 0-104) months. In univariate analysis, the albumin was significantly associated with OS (HR:0.491, 95% CI: 0.260-0.930, P = 0.029) and RFS (HR: 0.383, 95% CI: 0.192-0.766, P = 0.007). In multivariate analysis, serum albumin as an independent prognostic factor of OS was confirmed (HR: 0.351, 95% CI: 0.126-0.982, P = 0.046). CONCLUSIONS: Preoperative albumin might be a significant prognostic factor for ACC patients after primary resection. This result may be useful for risk stratification and management of this rare malignancy.


Assuntos
Neoplasias do Córtex Suprarrenal/patologia , Carcinoma Adrenocortical/patologia , Biomarcadores Tumorais/sangue , Recidiva Local de Neoplasia/patologia , Cuidados Pré-Operatórios , Albumina Sérica/análise , Adolescente , Neoplasias do Córtex Suprarrenal/sangue , Neoplasias do Córtex Suprarrenal/cirurgia , Carcinoma Adrenocortical/sangue , Carcinoma Adrenocortical/cirurgia , Adulto , Idoso , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/sangue , Recidiva Local de Neoplasia/cirurgia , Prognóstico , Curva ROC , Estudos Retrospectivos , Taxa de Sobrevida , Adulto Jovem
6.
J Pediatr Urol ; 17(5): 735.e1-735.e6, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34210620

RESUMO

INTRODUCTION: Pediatric adrenocortical carcinoma (ACC) is a rare malignancy, encompassing less than 0.2% of all childhood malignancies. Due to the scarcity of this diagnosis, it is often managed according to guidelines established for adults, as there is a lack of reliable evidence regarding optimal adjuvant treatment options for pediatric patients. It is our aim to identify recent treatment trends as well as clinical and tumor characteristics and their impact on overall survival. METHODS: Using the National Cancer Data Base (NCDB), this study identified 49 patients under 18 years old with localized ACC (M0) undergoing adrenalectomy from 2004 to 2017. Kaplan-Meier analysis was performed to determine overall survival (OS) from patient characteristics and treatments received. Comparison of survival was performed using the log rank test. RESULTS: The median age of our cohort was 3 years old with a slight female predominance of 61%. The median tumor size was 9.4 cm, and patients older than 4 years were significantly (p = 0.03) more likely to present with larger tumors (11.33 cm vs 8.76 cm). Adjuvant treatment in the form of systemic therapy was administered in 20 of 49 (41%) patients and radiation therapy in 2 of 49 (4%) patients. Three-year OS for patients 4 years old and younger was 92.6% vs 61.8% for those older than 4 years (p = 0.002). Patients presenting with tumor size ≥9 cm had worse three-year OS compared to those with tumors <9 cm (95.24% vs 67.1% respectively, p = 0.02, Fig. 1). In patients with tumors ≥ 9 cm, younger children age 0-4 years had significantly (p = 0.04) higher OS rates than older children age 5-17 years. CONCLUSIONS: ACC is a rare pediatric malignancy with a female predominance. Those older than 4 years and those with presenting tumor size ≥9 cm have decreased overall survival rates after adrenalectomy for localized disease. Additionally, children older than 4 have poorer prognosis, even after controlling for larger tumor size. This is the largest contemporary series of localized pediatric ACC to date. However, multi-institutional prospective cohort or randomized-controlled trials are necessary to better evaluate relevant prognostic factors and the role of adjuvant therapies following adrenalectomy.


Assuntos
Neoplasias do Córtex Suprarrenal , Carcinoma Adrenocortical , Adolescente , Neoplasias do Córtex Suprarrenal/cirurgia , Adrenalectomia , Carcinoma Adrenocortical/cirurgia , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Estimativa de Kaplan-Meier , Masculino , Prognóstico , Estudos Prospectivos , Estudos Retrospectivos , Taxa de Sobrevida
7.
Ann Ital Chir ; 92: 293-298, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34312326

RESUMO

INTRODUCTION: Oncocytic adrenocortical tumors represent a subtype of the adrenal cortex neoplasms. These tumors can be divided into oncocytomas, oncocytic neoplasms of uncertain malignancy and carcinomas (OACs). To date, only 34 cases of OAC have been reported. CASE EXPERIENCE: We reported a case of a 54-year-old male patient with a history of laparoscopic right adrenalectomy for an OAC and subsequent chemotherapy for a recurrence. He was referred to our emergency room for diffuse abdominal pain, vomit, change in bowel habits, fever, asthenia. He underwent a laparotomy and a complete excision of the known bilobate lesion. The histopathological findings matched the features of a recurrent OAC. No chemotherapy was administered after surgery and the patient was disease-free after a follow-up of twenty-eight months. RESULTS - DISCUSSION: The most questionable issue in treating adrenocortical oncocytic neoplasms is the determination of malignancy. According to the Helsinki Score, which is the best prognostic system, the primary lesion was an OAC. We also implemented the score systems to the recurrent lesion, that seemed to be malignant. We believe that the adjuvant treatment can delay a recurrent lesion development, but finally, radical surgical excision is necessary. Moreover, we reported the longest survival after the primary adrenalectomy. CONCLUSIONS: This study described the first case of recurrent oncocytic adrenocortical carcinoma with the longest followup. Adrenocortical oncocytoma is an extremely rare tumor of the adrenal gland with variable biological behavior without definitive consensus about diagnostic criteria. This was also the first case in which different histopathological criteria have been implemented in a recurrence. KEY WORDS: Oncocytic adrenocortical carcinoma, Surgical oncology, Survival.


Assuntos
Adenoma Oxífilo , Neoplasias do Córtex Suprarrenal , Carcinoma Adrenocortical , Recidiva Local de Neoplasia , Adenoma Oxífilo/diagnóstico , Adenoma Oxífilo/cirurgia , Neoplasias do Córtex Suprarrenal/diagnóstico , Neoplasias do Córtex Suprarrenal/cirurgia , Adrenalectomia , Carcinoma Adrenocortical/diagnóstico , Carcinoma Adrenocortical/cirurgia , Humanos , Laparoscopia , Laparotomia , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/diagnóstico , Recidiva Local de Neoplasia/cirurgia , Reoperação
9.
Am J Case Rep ; 22: e928875, 2021 Apr 04.
Artigo em Inglês | MEDLINE | ID: mdl-33813589

RESUMO

BACKGROUND Adrenocortical carcinomas are rare and aggressive tumors often diagnosed as incidentalomas. The malignancy can present with abnormal hormone secretion or the tumor may be non-functioning and present as a palpable mass causing discomfort. Here, we present a case of an adrenal cortical carcinoma originally identified as an incidentaloma. CASE REPORT A 63-year-old woman presented with abdominal pain and discomfort. A large abdominal mass, occupying the left upper and lower quadrant, was palpated. Imaging revealed a mass occupying the left abdomen between the stomach and the spleen, applying pressure on the pylorus, duodenum, splenic vessels, and pancreas. The mass size was 21.2×13×14.6 cm. Hormonal investigations were normal. Surgical exploration was performed, and the tumor was excised. Pathological analysis revealed an adrenocortical carcinoma and the patient underwent adjuvant chemotherapy. Twelve months later, the carcinoma recurred. The patient underwent a second operation in which the recurrent mass was excised along with the tail of the pancreas and a small part of the left lobe of the liver. The postoperative period was uneventful, and the patient was discharged home on the 7th postoperative day. No further adjuvant therapy was applied. The patient remains disease-free 18 months after the reoperation. CONCLUSIONS Giant adrenocortical carcinomas, although rare, pose a challenge to the surgical team both diagnostically and therapeutically. Surgical excision with the appropriate oncologic support can guarantee excellent outcomes.


Assuntos
Neoplasias do Córtex Suprarrenal , Carcinoma Adrenocortical , Neoplasias do Córtex Suprarrenal/diagnóstico por imagem , Neoplasias do Córtex Suprarrenal/cirurgia , Carcinoma Adrenocortical/diagnóstico por imagem , Carcinoma Adrenocortical/cirurgia , Terapia Combinada , Feminino , Humanos , Fígado , Pessoa de Meia-Idade , Recidiva Local de Neoplasia
10.
Clin Genitourin Cancer ; 19(5): 417-424, 2021 10.
Artigo em Inglês | MEDLINE | ID: mdl-33858789

RESUMO

INTRODUCTION: Adrenocortical carcinoma (ACC) is a rare cancer with few well-powered studies regarding epidemiology or outcomes and even fewer studies to guide an evidence-based management approach. The effect of radiation therapy (RT) is particularly understudied and remains unclear, outside of general oncologic principles. We sought to describe current care patterns in the management of ACC and how these practices affect survival outcomes using the Surveillance, Epidemiology, and End Results (SEER) database. METHODS: Patients with ACC who could be staged by the American Joint Committee on Cancer (AJCC) system diagnosed between 1988 and 2015 were included. A classification and regression tree analysis identified subgroups of patients who may have benefitted from RT. Multivariable Cox regression analysis was used to compare overall survival (OS). Propensity score adjustment was used to compare OS for a subset of node-negative, nonmetastatic patients who received adjuvant RT following radical surgery. RESULTS: Among 865 eligible patients included in the analysis, only 10.5% received RT. In stage III node-negative patients, adjuvant RT following radical surgery was associated with a significantly improved OS compared with surgery alone. CONCLUSION: Medically fit persons with stage III, node-negative ACC should receive adjuvant RT after surgical resection.


Assuntos
Neoplasias do Córtex Suprarrenal , Carcinoma Adrenocortical , Neoplasias do Córtex Suprarrenal/radioterapia , Neoplasias do Córtex Suprarrenal/cirurgia , Carcinoma Adrenocortical/radioterapia , Carcinoma Adrenocortical/cirurgia , Humanos , Estadiamento de Neoplasias , Radioterapia Adjuvante , Programa de SEER , Resultado do Tratamento
11.
Georgian Med News ; (310): 7-11, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-33658401

RESUMO

We present a case of androgen and glucocorticoid secreting adrenocortical carcinoma with concomitant myelolipoma. A giant adrenal tumor which was initially nonfunctional was reassessed four years later due to the patient's refusal to treat. The patient was a 48-year-old woman with hypertension and acne lesions on the face. Laboratory findings were consistent with glucocorticoid and androgen hypersecretion. Computed tomography revealed a heterogeneously contrasting mass of 145x118x100 mm with lobular contour and soft tissue areas. The patient underwent left laparoscopic transperitoneal adrenalectomy with three port technique. There were no complications in the perioperative period. The resected specimen weighed 850 grams. Pathological findings showed a combination of myelolipoma-adrenal cortical cancer. In the postoperative period, hypertension improved and the hormone panel was normalized. Postoperative computed tomography and PET-CT showed no residual mass and metastasis. Although imaging is compatible with benign masses such as myelolipoma, coexistence of ACC-myelolipoma should be kept in mind and functional evaluation should be performed.


Assuntos
Neoplasias do Córtex Suprarrenal , Neoplasias das Glândulas Suprarrenais , Carcinoma Adrenocortical , Mielolipoma , Neoplasias do Córtex Suprarrenal/complicações , Neoplasias do Córtex Suprarrenal/diagnóstico por imagem , Neoplasias do Córtex Suprarrenal/cirurgia , Neoplasias das Glândulas Suprarrenais/cirurgia , Carcinoma Adrenocortical/complicações , Carcinoma Adrenocortical/diagnóstico por imagem , Carcinoma Adrenocortical/cirurgia , Androgênios , Feminino , Glucocorticoides , Humanos , Pessoa de Meia-Idade , Mielolipoma/complicações , Mielolipoma/diagnóstico , Mielolipoma/cirurgia , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada
12.
Cancer Med ; 10(7): 2259-2267, 2021 04.
Artigo em Inglês | MEDLINE | ID: mdl-33687146

RESUMO

BACKGROUND: To evaluate outcomes and survival rates in patients with metastatic adrenocortical carcinoma (ACC) who were treated with image-guided locoregional treatments (IGLTs). PURPOSE: To evaluate the overall survival (OS) and clinical impact of IGLT in the management of patients with advanced metastatic ACC. METHODS: Retrospective review of 39 patients treated with IGLT between 1999 and 2018 was performed. Short- and long-term efficacy of treatments were defined based upon imaging and clinical data. Subgroup survival analysis was performed on patients with metastatic disease at diagnosis (N = 17) and compared with the same stage group from the most recent National Cancer Database (NCDB) report. Statistical analysis was performed using Cox proportional hazards model. RESULTS: Treatments were performed at different anatomic sites including liver (N = 46), lung (N = 14), retroperitoneum (N = 5), bone (N = 4), subcutaneous (N = 2), and intracaval (N = 1). Radiofrequency, microwave, cryoablation, or a combination of two modalities (45, 18, 3, 3, respectively) were used in 69 ablation sessions. Intra-arterial procedures were performed in 12 patients in 18 treatment cycles (range 1-3 per patient). As of a 2019 analysis, 11 patients were alive with a mean follow-up of 169 months (range 63-292 months) from diagnosis. Two- and 5-year OS rates for all patients were 84.5% and 51%, respectively, and 76.5% and 59% for patients with metastatic disease at diagnosis (N = 17). This compares favorably with an NCDB report of 35% 5-year survival rate for patients with metastatic disease. Female gender and longer time from diagnosis to first IGLT were found to be predictors of prolonged survival with hazard ratios of 0.23 (p < 0.001) and 0.66 (p = 0.001), respectively. CONCLUSION: IGLT may be associated with prolonged life expectancy in select patients with metastatic ACC.


Assuntos
Neoplasias do Córtex Suprarrenal/cirurgia , Carcinoma Adrenocortical/cirurgia , Criocirurgia/métodos , Embolização Terapêutica/métodos , Neoplasias do Córtex Suprarrenal/diagnóstico por imagem , Neoplasias do Córtex Suprarrenal/patologia , Carcinoma Adrenocortical/diagnóstico por imagem , Carcinoma Adrenocortical/patologia , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Ablação por Radiofrequência/métodos , Estudos Retrospectivos , Cirurgia Assistida por Computador/métodos , Taxa de Sobrevida , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento , Adulto Jovem
13.
BJS Open ; 5(1)2021 01 08.
Artigo em Inglês | MEDLINE | ID: mdl-33609384

RESUMO

BACKGROUND: Adrenocortical carcinomas (ACCs) carry a poor prognosis. This study assessed the comparative performance of existing nomograms in estimating the likelihood of survival, along with the value of conditional survival estimation for patients who had already survived for a given length of time after surgery. METHODS: This was an observational study based on a prospectively developed departmental database that recorded details of patients operated for ACC in a UK tertiary referral centre. RESULTS: Of 74 patients with ACC managed between 2001 and 2020, data were analysed for 62 patients (32 women and 30 men, mean(s.d.) age 51(17) years) who had primary surgical treatment in this unit. Laparoscopic (9) or open adrenalectomies (53) were performed alone or in association with a multivisceral resection (27). Most of the tumours were left-sided (40) and 18 were cortisol-secreting.Overall median survival was 33 months, with 1-, 3- and 5-year survival rates of 79, 49, and 41 per cent respectively. Age over 55 years, higher European Network for Study of Adrenal Tumours stage, and cortisol secretion were associated with poorer survival in univariable analyses. Four published nomograms suggested widely variable outcomes that did not correlate with observed overall survival at 1, 3 or 5 years after operation. The 3-year conditional survival at 2 years (probability of surviving to postoperative year 5) was 65 per cent, compared with a 5-year actuarial survival rate of 41 per cent calculated from the time of surgery. CONCLUSION: Survival of patients with ACC correlates with clinical parameters but not with published nomograms. Conditional survival might provide a more accurate estimate of survival for patients who have already survived for a certain amount of time after resection.


Assuntos
Neoplasias do Córtex Suprarrenal/mortalidade , Adrenalectomia , Carcinoma Adrenocortical/mortalidade , Nomogramas , Taxa de Sobrevida/tendências , Neoplasias do Córtex Suprarrenal/cirurgia , Carcinoma Adrenocortical/cirurgia , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Centros de Atenção Terciária , Resultado do Tratamento , Reino Unido
15.
J Surg Oncol ; 123(5): 1238-1245, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33577722

RESUMO

BACKGROUND: Adrenocortical carcinoma (ACC) is often a contraindication to minimally invasive adrenalectomy (MIA). We used an administrative data set to analyze postoperative outcomes. We hypothesized that small tumors would have better short- and long-term outcomes, independent of the operative approach. METHODS: The National Cancer Database (2010-2016) identified patients with ACC who underwent adrenalectomy. Tumors were grouped: <5 cm (n = 125), 5-10 cm (n = 431), and >10 cm (n = 443). The primary and secondary outcomes were margin positivity and overall survival, respectively. RESULTS: Nine hundred and ninety-nine patients were analyzed: 37% MIA and 63% open adrenalectomy (OA). As the size increased, the rate of attempted MIA decreased. Larger tumors were associated with conversion to open. Although tumors with local invasion and those which required conversion to open were associated with an increased likelihood of a positive margin, tumor size was not. Although "complete" MIA (vs. OA) and tumor size were not associated with differences in survival, conversion (HR = 1.83, p = .02), positive margins (HR = 1.54, p = .01), and local invasion (HR = 1.84, p < .001) were associated with poor survival. CONCLUSION: Positive margins are associated with poor survival in ACC. Tumors ≥ 5 cm were associated with an increased conversion rate and subsequent increase in margin positivity. MIA may be considered for select patients with small tumors but adequate oncologic resection is critical.


Assuntos
Neoplasias do Córtex Suprarrenal/patologia , Adrenalectomia/mortalidade , Carcinoma Adrenocortical/patologia , Laparoscopia/mortalidade , Margens de Excisão , Neoplasias do Córtex Suprarrenal/cirurgia , Carcinoma Adrenocortical/cirurgia , Adulto , Idoso , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida
16.
Ann Surg Oncol ; 28(11): 6551-6561, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-33586069

RESUMO

BACKGROUND: Adrenocortical carcinoma (ACC) is a rare but aggressive malignancy, and many prognostic factors that influence survival remain undefined. Individually, the GRAS (Grade, Resection status, Age, and Symptoms of hormone hypersecretion) parameters have demonstrated their prognostic value in ACC. This study aimed to assess the value of a cumulative GRAS score as a prognostic indicator after ACC resection. METHODS: A retrospective cohort study of adult patients who underwent surgical resection for ACC between 1993 and 2014 was performed using the United States Adrenocortical Carcinoma Group (US-ACCG) database. A sum GRAS score was calculated for each patient by adding one point each when the criteria were met for tumor grade (Weiss criteria ≥ 3 or Ki67 ≥ 20%), resection status (micro- or macroscopically positive margin), age (≥ 50 years), and preoperative symptoms of hormone hypersecretion (present). Overall survival (OS) and disease-free survival (DFS) by cumulative GRAS score were analyzed by the Kaplan-Meier method and log-rank test. RESULTS: Of the 265 patients in the US-ACCG database, 243 (92%) had sufficient data available to calculate a cumulative GRAS score and were included in this analysis. The 265 patients comprised 23 patients (10%) with a GRAS of 0, 52 patients (21%) with a GRAS of 1, 92 patients (38%) with a GRAS of 2, 63 patients (26%) with a GRAS of 3, and 13 patients (5%) with a GRAS of 4. An increasing GRAS score was associated with shortened OS (p < 0.01) and DFS (p < 0.01) after index resection. CONCLUSION: In this retrospective analysis, the cumulative GRAS score effectively stratified OS and DFS after index resection for ACC. Further prospective analysis is required to validate the cumulative GRAS score as a prognostic indicator for clinical use.


Assuntos
Neoplasias do Córtex Suprarrenal , Carcinoma Adrenocortical , Neoplasias do Córtex Suprarrenal/cirurgia , Carcinoma Adrenocortical/cirurgia , Adulto , Intervalo Livre de Doença , Humanos , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Estados Unidos/epidemiologia
18.
Langenbecks Arch Surg ; 406(5): 1599-1606, 2021 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-33521844

RESUMO

PURPOSE: Prior literature suggests that cancer patients with hyperglycemia and type 2 diabetes mellitus (DM) exhibit worse oncologic and overall outcomes. Tumor metabolism and anabolism pathophysiology may explain this association, although this has not been adequately studied in adrenocortical carcinoma (ACC). We hypothesized that DM would be associated with worse oncological outcomes in ACC, and we utilized data from a national database and institutional sources for multimodal analysis. METHODS: Both a multi-institutional database (the Collaborative Endocrine Surgery Quality Improvement Program or CESQIP) and a single-center longitudinal cohort (Dana Farber Cancer Institute or DFCI) were queried as unique retrospective cohorts to identify patients with ACC. Patient demographics, tumor characteristics, DM-specific variables, and oncologic outcome data were assessed. Results were analyzed via univariate analysis and multivariable linear regression analysis. Statistical significance was defined as p < 0.05. RESULTS: Forty-eight CESQIP patients met inclusion criteria; 16 (33.0%) had DM. DM patients had a higher frequency of recurrence on longitudinal follow-up (12.5% v 0.0%, p = 0.04). Persistent disease was observed in 68.8% of DM patients and 40.6% of non-DM patients (p = 0.06). Patients in the DFCI cohort with lower average glucose values (< 110 mg/dL) had a significant survival benefit (p < .0001). A mean serum glucose > 110 mg/dL had increased risk (HR 36.3, 95% confidence interval 1.6, 831.3) for all-cause mortality. CONCLUSIONS: This multi-institutional, multimodal analysis suggests that patients with DM have worse oncologic and overall outcomes for ACC. While further study is warranted, consideration should be given among clinicians to optimize glycemic control as part of their ACC management.


Assuntos
Neoplasias do Córtex Suprarrenal , Carcinoma Adrenocortical , Diabetes Mellitus Tipo 2 , Diabetes Mellitus , Hiperglicemia , Neoplasias do Córtex Suprarrenal/complicações , Neoplasias do Córtex Suprarrenal/cirurgia , Carcinoma Adrenocortical/complicações , Carcinoma Adrenocortical/cirurgia , Diabetes Mellitus Tipo 2/complicações , Diabetes Mellitus Tipo 2/epidemiologia , Humanos , Hiperglicemia/epidemiologia , Recidiva Local de Neoplasia , Estudos Retrospectivos
19.
World J Surg ; 45(5): 1457-1465, 2021 05.
Artigo em Inglês | MEDLINE | ID: mdl-33481081

RESUMO

BACKGROUND: Metastatic adrenocortical carcinoma (ACC) is an aggressive cancer with poor prognosis, with limited treatment options. The survival benefit of adrenal surgery in patients with synchronous metastatic disease has not been well explored. METHODS: Patients with ACC with synchronous metastases were identified from the Surveillance, Epidemiology, and End Results database (2010-2016). The effect of adrenal surgery on different patterns of distant metastases was assessed. The overall survival was estimated by the Kaplan-Meier method. Multivariable Cox regression analysis was performed to identify prognostic factors associated with survival outcome. RESULTS: A total of 202 patients with synchronous metastatic ACC were identified from the SEER database, 76 (37.6%) patients underwent adrenal surgery. Compared to nonsurgical patients, patients who underwent adrenal surgery had a better survival (median overall survival: 4 vs. 13 months, P < 0.001). In sub-analyses, except for patients with liver metastases (P = 0.670), adrenalectomy could consistently confer a significant survival benefit in patients with lung metastases (P = 0.003), bone metastases (P = 0.020), and multiple metastases (P = 0.002). Cox regression analysis revealed that in addition to adrenalectomy [hazard ratio (HR) = 0.64, 95% confidence interval (CI) 0.45-0.92; P = 0.017], metastasectomy (HR = 0.48, 95% CI 0.26-0.86; P = 0.013), and chemotherapy (HR = 0.59, 95% CI 0.42-0.82; P = 0.002) were also associated with improved survival. CONCLUSIONS: Our findings support the view that adrenal surgery may be associated with improved survival in patients with synchronous metastatic ACC (except for patients with liver metastases), and the metastatic sites have significant prognostic implications on survival outcomes with adrenal surgery.


Assuntos
Neoplasias do Córtex Suprarrenal , Neoplasias das Glândulas Suprarrenais , Carcinoma Adrenocortical , Metastasectomia , Neoplasias do Córtex Suprarrenal/cirurgia , Neoplasias das Glândulas Suprarrenais/cirurgia , Carcinoma Adrenocortical/cirurgia , Humanos , Prognóstico , Estudos Retrospectivos , Programa de SEER
20.
ANZ J Surg ; 91(5): 992-999, 2021 05.
Artigo em Inglês | MEDLINE | ID: mdl-33438328

RESUMO

BACKGROUND: Adrenocortical tumours (ACT) are rare tumours of childhood usually presenting with endocrine dysfunction. This retrospective study is designed to review our institutional experience in surgical management. METHODS: Records of children treated for ACT between 1999 and 2019 were reviewed retrospectively. RESULTS: The median age of 24 children was 78 months. Fourteen patients had adrenocortical carcinoma, nine had adrenocortical adenoma and one had neuroendocrine differentiation of ACT. Endocrine dysfunction was noted in 79% of the patients. Five patients had preoperative chemotherapy but none had a decrease in tumour size. Transabdominal approach was used in all but two patients who had thoracoabdominal incision for excision of giant tumours and ipsilateral lung metastases. Two patients had visceral excision to achieve R0 resection. Five patients, four of whom had spillage and one with partial resection died of widespread disease. Two patients with stage 4 adrenocortical carcinoma are still on chemotherapy. All patients with stage I-III disease who had total excision without spillage (n = 17) are disease-free for 2-170 months. CONCLUSIONS: Our results show the importance of excision in ACT without spillage for survival. However, multicentre prospective studies should enhance the knowledge of children about ACT and develop alternative therapies for stage III and IV cases.


Assuntos
Neoplasias do Córtex Suprarrenal , Carcinoma Adrenocortical , Neoplasias do Córtex Suprarrenal/cirurgia , Carcinoma Adrenocortical/cirurgia , Criança , Pré-Escolar , Humanos , Estudos Prospectivos , Estudos Retrospectivos , Centros de Atenção Terciária
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