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Adrenal carcinoma — Clinical Guidelines

Overview

Adrenal carcinoma, primarily adrenal cortical carcinoma (ACC), is a rare and aggressive malignancy with varied clinical presentations including hormonal syndromes and non-functioning masses. Complete surgical resection offers the best prognosis, though recurrence is common and outcomes can be unpredictable 1.

Diagnosis

Clinical Presentation: Symptoms vary widely, including hormonal excess syndromes (e.g., hyperaldosteronism, hypercortisolism) and nonspecific symptoms like back pain 5 9.

Imaging: CT and MRI are crucial for tumor characterization and assessing local invasion; unusual imaging findings may indicate malignancy 8.

Pathological Assessment: Rigorous pathological evaluation is essential for accurate staging and risk stratification 1.

Hormonal Testing: Urinary steroid profiles can aid in diagnosing specific hormonal excesses due to abnormal steroid precursor synthesis 5.

Lymph Node Evaluation: Lymphography may be necessary for assessing lymph node metastases and guiding post-operative treatment planning 13.

Management

Primary Treatment: Complete surgical resection is the cornerstone of treatment 1.

Adjuvant Therapy: Considered based on risk stratification post-surgery, though specific regimens are not detailed in the abstracts 1.

Monitoring: Regular follow-up is critical due to high recurrence rates; imaging and hormonal assessments guide management 1.

Special Populations

Pregnancy: Not specifically addressed in the provided abstracts.

Pediatrics: No specific cases or recommendations noted.

Elderly: No distinct management strategies highlighted for elderly patients.

Comorbidities: Management considerations for comorbidities like venous thrombosis (e.g., caval occlusion) are important but not extensively detailed 10.

Key Recommendations

Surgical Resection: Complete resection of adrenal carcinoma should be pursued when feasible to optimize outcomes (Evidence: Strong 1).

Rigorous Pathological Assessment: Uniform and thorough pathological evaluation is crucial for accurate staging and guiding adjuvant therapy decisions (Evidence: Moderate 1).

Close Monitoring Post-Surgery: Regular follow-up with imaging and hormonal assessments is essential due to high recurrence rates (Evidence: Expert opinion).

References

1 Giordano TJ, Berney D, de Krijger RR, Erickson L, Fassnacht M, Mete O et al.. Data set for reporting of carcinoma of the adrenal cortex: explanations and recommendations of the guidelines from the International Collaboration on Cancer Reporting. Human pathology 2021. link 2 Sudhakar MK, Kallarakkal JT, Damodharan J, Sahib K, Mahajan A, Kannan R. Sweet's syndrome preceding carcinoma of the adrenal cortex. Journal of the Indian Medical Association 2005. link 3 Kumar U, Albala DM. Laparoscopic approach to adrenal carcinoma. Journal of endourology 2001. link 4 Albala DM. Laparoscopic nephrectomy and adrenalectomy. Seminars in surgical oncology 1994. link 5 Bijl M, Bakker AJ, Leemhuis MP. Mineralocorticoid excess due to precursors only in a patient with adrenal cortical carcinoma. The Netherlands journal of medicine 1992. link 6 Collina G, Maldarizzi F, Betts CM, Eusebi V. Primary sarcomatoid carcinoma of the adrenal gland. First case report. Virchows Archiv. A, Pathological anatomy and histopathology 1989. link 7 Brod SA, Spencer SS, Kim JH. Primary omphalomesenteric adrenocarcinoma with metastases to the brain. Surgical neurology 1988. link90183-8) 8 Pombo Felipe F, Marini M, Arrojo Suarez de Centi L, Capdevila A, Rodriguez Vila A. An unusual appearance of a left adrenal mass on computed tomography. Clinical radiology 1988. link80358-1) 9 Panesar NS, Tsao SY, Wheeler MJ, Cockram CS. Hyperaldosteronism combined with hypercortisolaemia in a patient with adrenal carcinoma. Postgraduate medical journal 1988. link 10 Päivänsalo M, Myllylä V, Mäkäräinen H. Caval occlusion caused by a metastasis of adrenal carcinoma. Rontgen-Blatter; Zeitschrift fur Rontgen-Technik und medizinisch-wissenschaftliche Photographie 1985. link 11 Palte SB, Pillans PI. Adrenal carcinoma. A case report. South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde 1985. link 12 Coutsoyannis Z, Androulakakis P. Non-functioning carcinoma of the adrenal cortex. International surgery 1979. link 13 Rauste J, Tallroth K, Wiljasalo M. Lymphographic changes caused by lymph node metastases in carcinoma of the suprarenal glands. Lymphology 1976. link

Adrenal carcinoma