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Adrenocortical Carcinoma Surgery

Open or laparoscopic radical adrenalectomy for primary adrenocortical malignancy.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Üroloji department. Book Appointment →

What is Adrenocortical Carcinoma Surgery?

Adrenocortical carcinoma (ACC) is a rare aggressive malignancy arising from the adrenal cortex, with bimodal age distribution (children <5 and adults 40-50). About 60% are functional, secreting cortisol (Cushing's syndrome), aldosterone (Conn's syndrome), androgens (virilization) or estrogens (feminization). Non-functional tumors typically present with mass effect or as incidentalomas.

Surgical resection is the only curative treatment. Open radical adrenalectomy is the standard for large (>6 cm), locally advanced or invasive ACC and includes en-bloc resection of the adrenal gland with periadrenal fat, regional lymph nodes and adjacent organs (kidney, spleen, pancreas tail, liver) when invaded. R0 resection (negative margins) is the strongest prognostic factor.

Minimally invasive (laparoscopic/robotic) adrenalectomy is reserved for select small (<6 cm), well-localized tumors without preoperative evidence of invasion. Tumor capsule disruption, port-site recurrence and incomplete lymphadenectomy concerns historically favored open approach. Adjuvant mitotane therapy is recommended for ENSAT stage III-IV or high-grade disease, with platinum-based chemotherapy for advanced cases.

Symptoms

Cushing's syndrome (rapid weight gain, hypertension)
Virilization (women) or feminization (men)
Conn's syndrome (hypertension, hypokalemia)
Abdominal pain or palpable mass
Incidental adrenal mass on imaging
Constitutional symptoms (weight loss, fatigue)
Metastatic symptoms (bone, lung, liver)

Risk Factors

Li-Fraumeni syndrome (TP53 mutation)
Lynch syndrome
Beckwith-Wiedemann syndrome
Multiple endocrine neoplasia type 1 (MEN1)
Carney complex
Family history of ACC
Childhood ACC (Brazilian R337H mutation)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Rapidly progressive Cushing's syndrome
  • Adrenal mass >4 cm with suspicious imaging
  • Functional adrenal tumor with virilization/feminization
  • Adrenal incidentaloma with growth >5 mm/year
  • Recurrence after primary surgery

Treatment Methods

01
Multidisciplinary endocrine tumor board
02
Hormonal workup and biochemical staging
03
Adrenal protocol CT/MRI and FDG-PET
04
Open radical adrenalectomy with en-bloc resection
05
Regional lymphadenectomy (paraaortic/paracaval)
06
Adjacent organ resection when invaded
07
Minimally invasive only for small localized tumors
08
Adjuvant mitotane for ENSAT III-IV or R1/Rx
09
Cisplatin/doxorubicin/etoposide chemotherapy for advanced disease
10
Long-term endocrine surveillance and substitution

Which Department to Visit?

You can visit our Üroloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.