Surgery

The Children's Oncology Group conducted a prospective, single-arm, risk-stratified, interventional study between 2006 and 2013 (ARAR0332 [NCT00304070]).[12][Level of evidence B4] Patients with stage I disease had small tumors limited to the adrenal gland and were treated with adrenalectomy alone. Patients with stage II disease had tumors larger than 200 mL or heavier than 100 g and were treated with adrenalectomy and retroperitoneal lymph node dissection (RPLND). Patients with stage III disease had incompletely resected primary tumors. Patients with stage IV disease had distant metastatic disease. Patients with stage III and stage IV disease were treated with surgery of the primary tumor, RPLND, cisplatin, etoposide, doxorubicin, and mitotane.The stated goal of the study was to determine if RPLND would improve outcomes for patients with stage II disease. The operative notes to assess the adequacy of the RPLND were available for 11 of 15 patients. The median number of lymph nodes resected was 4 (range, 1–30). In a multivariable analysis performed in a cohort of 283 adult patients with adrenocortical carcinoma, patients who underwent RPLND (defined as >5 nodes resected) had a significantly reduced recurrence risk and disease-related death rate than patients who did not undergo nodal dissection.[13] The authors speculate that the RPLNDs performed in this study may not have been as thorough as the procedures carried out in the adult series, which could confound conclusions about the potential value of RPLND.

The Children's Oncology Group conducted a prospective, single-arm, risk-stratified, interventional study between 2006 and 2013 (ARAR0332 [NCT00304070]).[12][Level of evidence B4] Patients with stage I disease had small tumors limited to the adrenal gland and were treated with adrenalectomy alone. Patients with stage II disease had tumors larger than 200 mL or heavier than 100 g and were treated with adrenalectomy and retroperitoneal lymph node dissection (RPLND). Patients with stage III disease had incompletely resected primary tumors. Patients with stage IV disease had distant metastatic disease. Patients with stage III and stage IV disease were treated with surgery of the primary tumor, RPLND, cisplatin, etoposide, doxorubicin, and mitotane.The stated goal of the study was to determine if RPLND would improve outcomes for patients with stage II disease. The operative notes to assess the adequacy of the RPLND were available for 11 of 15 patients. The median number of lymph nodes resected was 4 (range, 1–30). In a multivariable analysis performed in a cohort of 283 adult patients with adrenocortical carcinoma, patients who underwent RPLND (defined as >5 nodes resected) had a significantly reduced recurrence risk and disease-related death rate than patients who did not undergo nodal dissection.[13] The authors speculate that the RPLNDs performed in this study may not have been as thorough as the procedures carried out in the adult series, which could confound conclusions about the potential value of RPLND.

The 5-year event-free survival rate estimates were 86.2% for patients with stage I disease (n = 24), 53.3% for patients with stage II disease (n = 15), 81% for patients with stage III disease (n = 24), and 7.1% for patients with stage IV disease (n = 14).

The Children's Oncology Group conducted a prospective, single-arm, risk-stratified, interventional study between 2006 and 2013 (ARAR0332 [NCT00304070]).[12][Level of evidence B4] Patients with stage I disease had small tumors limited to the adrenal gland and were treated with adrenalectomy alone. Patients with stage II disease had tumors larger than 200 mL or heavier than 100 g and were treated with adrenalectomy and retroperitoneal lymph node dissection (RPLND). Patients with stage III disease had incompletely resected primary tumors. Patients with stage IV disease had distant metastatic disease. Patients with stage III and stage IV disease were treated with surgery of the primary tumor, RPLND, cisplatin, etoposide, doxorubicin, and mitotane.The stated goal of the study was to determine if RPLND would improve outcomes for patients with stage II disease. The operative notes to assess the adequacy of the RPLND were available for 11 of 15 patients. The median number of lymph nodes resected was 4 (range, 1–30). In a multivariable analysis performed in a cohort of 283 adult patients with adrenocortical carcinoma, patients who underwent RPLND (defined as >5 nodes resected) had a significantly reduced recurrence risk and disease-related death rate than patients who did not undergo nodal dissection.[13] The authors speculate that the RPLNDs performed in this study may not have been as thorough as the procedures carried out in the adult series, which could confound conclusions about the potential value of RPLND.

On multivariable analysis, only stage and age were significantly associated with outcome.

The Children's Oncology Group conducted a prospective, single-arm, risk-stratified, interventional study between 2006 and 2013 (ARAR0332 [NCT00304070]).[12][Level of evidence B4] Patients with stage I disease had small tumors limited to the adrenal gland and were treated with adrenalectomy alone. Patients with stage II disease had tumors larger than 200 mL or heavier than 100 g and were treated with adrenalectomy and retroperitoneal lymph node dissection (RPLND). Patients with stage III disease had incompletely resected primary tumors. Patients with stage IV disease had distant metastatic disease. Patients with stage III and stage IV disease were treated with surgery of the primary tumor, RPLND, cisplatin, etoposide, doxorubicin, and mitotane.The stated goal of the study was to determine if RPLND would improve outcomes for patients with stage II disease. The operative notes to assess the adequacy of the RPLND were available for 11 of 15 patients. The median number of lymph nodes resected was 4 (range, 1–30). In a multivariable analysis performed in a cohort of 283 adult patients with adrenocortical carcinoma, patients who underwent RPLND (defined as >5 nodes resected) had a significantly reduced recurrence risk and disease-related death rate than patients who did not undergo nodal dissection.[13] The authors speculate that the RPLNDs performed in this study may not have been as thorough as the procedures carried out in the adult series, which could confound conclusions about the potential value of RPLND.

The authors reported that the combination of mitotane and chemotherapy resulted in significant toxicity. One-third of patients with advanced disease could not complete the scheduled treatment.