Overview
Definition:
Adrenocortical carcinoma (ACC) is a rare, malignant tumor arising from the cortex of the adrenal gland
Open resection refers to the surgical removal of the adrenal gland and the tumor through a surgical incision, as opposed to laparoscopic or robotic approaches.
Epidemiology:
ACC is an uncommon endocrine malignancy with an incidence of 1-2 cases per million population per year
It can occur at any age, but there is a bimodal distribution with peaks in childhood and in the 4th-5th decades of life
There is no clear gender predilection, though some studies suggest a slight female predominance.
Clinical Significance:
ACC is an aggressive cancer with a poor prognosis, often presenting at advanced stages
Complete surgical resection is the cornerstone of curative treatment
Understanding the surgical nuances is critical for improving patient outcomes and achieving disease control, making this a vital topic for surgical residents preparing for examinations.
Indications
Indications For Surgery:
Surgical resection is indicated for all resectable ACCs, regardless of hormonal activity
Curative intent is the primary goal
In cases of unresectable or metastatic disease, palliative resection may be considered to alleviate symptoms related to hormone overproduction or mass effect.
Functional Vs Nonfunctional Tumors:
Functional ACCs secrete excess hormones (cortisol, aldosterone, androgens), leading to Cushing's syndrome, hyperaldosteronism, or virilization
Non-functional tumors are typically diagnosed due to their size or incidental findings on imaging, often presenting with mass effect or pain.
Tumor Staging And Resectability:
Resectability is determined by imaging (CT, MRI) and intraoperative assessment
Tumors are considered resectable if they are clearly demarcated, do not involve major vascular structures (e.g., vena cava, renal vein), and can be completely excised with clear margins
Staging (e.g., using TNM system) guides treatment decisions.
Preoperative Preparation
Preoperative Hormonal Management:
For functional ACCs, preoperative medical management is crucial to control hormonal excess and prevent complications
This often involves drugs like ketoconazole, metyrapone, or osilodrostat to inhibit steroidogenesis
Beta-blockers may be used for hypertension and tachycardia
Mineralocorticoid antagonists are used for hyperaldosteronism.
Imaging And Staging:
Contrast-enhanced CT and MRI of the abdomen and pelvis are essential for assessing tumor size, local invasion, vascular involvement, and presence of metastases
Bone scans, PET-CT, and chest CT may be performed for metastatic workup.
Anesthesia Considerations:
Anesthesiologists must be aware of the hormonal status of the patient to manage potential complications like hypertensive crisis, hypokalemia, or fluid shifts
Invasive monitoring (arterial line, central venous catheter) may be necessary for large tumors or significant comorbidities.
Patient Counseling And Consent:
Thorough discussion with the patient regarding the risks and benefits of open adrenalectomy, including potential complications, expected recovery, and the possibility of adrenal insufficiency postoperatively, is essential for informed consent.
Procedure Steps Open Adrenalectomy
Surgical Approaches:
Common open approaches include the flank (transabdominal or retroperitoneal) incision and the anterior subcostal (or thoracoabdominal) incision
The choice depends on tumor size, location, and surgeon preference
Transabdominal approaches offer wider exposure for larger tumors or bilateral disease.
Exposure And Dissection:
The adrenal gland is identified and dissected carefully from surrounding structures
Key steps involve ligating and dividing the adrenal vein early to prevent tumor embolization and hormonal surge
Dissection proceeds around the gland, meticulously identifying and preserving surrounding vital structures.
Tumor Excision And Margin Assessment:
The tumor is excised en bloc with the adrenal gland
Careful attention is paid to achieving negative surgical margins (R0 resection)
Frozen section analysis may be used intraoperatively to assess margin status if there is concern for local invasion.
Management Of Vascular Involvement:
If the tumor invades major vessels like the vena cava or renal vein, vascular reconstruction may be necessary
This is a complex scenario that requires expertise in vascular surgery and careful planning.
Closure:
After ensuring adequate hemostasis and achieving negative margins, the surgical site is closed in layers
Placement of a drain is common to monitor for bleeding or fluid collections.
Postoperative Care And Monitoring
Intensive Care Unit Monitoring:
Patients are typically monitored in the ICU postoperatively for hemodynamic stability, fluid balance, and electrolyte levels, especially those with functional tumors or large resections.
Hormonal Replacement Therapy:
Post-adrenalectomy, patients will likely require lifelong glucocorticoid replacement therapy (e.g., hydrocortisone) and possibly mineralocorticoid replacement (e.g., fludrocortisone), particularly after bilateral adrenalectomy or if the remaining adrenal tissue is insufficient.
Pain Management:
Adequate pain control is essential for patient comfort and early mobilization
This may involve patient-controlled analgesia (PCA) or multimodal analgesia.
Monitoring For Complications:
Close observation for signs of bleeding, infection, ileus, adrenal crisis, and vascular complications is crucial in the immediate postoperative period
Serial laboratory tests (electrolytes, cortisol levels) and imaging may be required.
Early Mobilization And Rehabilitation:
Early ambulation is encouraged to prevent thromboembolic events and promote recovery
A multidisciplinary team approach, including physical therapy, is beneficial.
Complications
Early Complications:
Hemorrhage from the adrenal bed or dissected vessels
Adrenal insufficiency (acute adrenal crisis) presenting with hypotension, hyponatremia, hyperkalemia, and shock
Surgical site infection
Ileus
Pancreatitis
Injury to adjacent organs (spleen, kidney, bowel)
Vascular thrombosis.
Late Complications:
Recurrence of ACC (local or metastatic)
Adrenal insufficiency requiring lifelong hormone replacement
Wound dehiscence
Incisional hernia
Adrenal rest formation.
Prevention Strategies:
Meticulous surgical technique to ensure hemostasis and avoid injury to surrounding organs
Careful preoperative medical management of hormonal excess
Prompt recognition and management of adrenal insufficiency with appropriate hormone replacement
Strict adherence to sterile protocols to prevent infections.
Prognosis
Factors Affecting Prognosis:
Stage at diagnosis is the most significant prognostic factor
Other important factors include completeness of surgical resection (R0 vs
R1/R2), tumor size, presence of venous or capsular invasion, mitotic rate, presence of necrosis, and functional status of the tumor.
Outcomes And Survival Rates:
The 5-year survival rate for localized ACC (Stage I-II) after complete resection can be as high as 80-90%
However, for advanced disease (Stage III-IV), survival rates drop significantly, with a 5-year survival of around 20-30%
Metastatic disease is generally incurable.
Recurrence And Follow Up:
Patients require lifelong follow-up due to the high risk of recurrence
This typically involves regular clinical examinations, biochemical monitoring for hormone levels, and imaging (CT scans) every 3-6 months for the first 2-3 years, then annually thereafter
Surveillance aims for early detection of recurrence or metastases.
Key Points
Exam Focus:
Open adrenalectomy for ACC is a key surgical procedure
Focus on indications, preoperative hormonal control, operative steps, essential postoperative hormone replacement, and common complications like adrenal crisis and recurrence.
Clinical Pearls:
Early ligation of the adrenal vein is critical
Meticulous dissection is paramount to achieve negative margins and avoid injury to vital structures
Always consider the hormonal status and its implications for anesthetic and postoperative management
Lifelong steroid replacement is usually required.
Common Mistakes:
Inadequate preoperative hormonal control leading to perioperative instability
Incomplete resection due to failure to achieve negative margins
Delayed recognition and management of adrenal insufficiency
Insufficient follow-up leading to missed recurrences.