Adrenocortical Carcinoma Hereditary C1859972
A rare, aggressive cancer of the adrenal cortex that may run in families. Treatment typically involves surgery, chemotherapy, and radiation depending on stage.
Key facts at a glance
- ICD-10
- C80.1
- ICD-11
- 2A00.0
- Body system
- oncologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Adrenal mass (abdominal mass)
(oncologic)Generalized symptoms
(oncologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Hypertension due to hormone excess
(oncologic)Virilization (female)
(oncologic)Cushing's syndrome features
(oncologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Complete Adrenalectomy
Surgical removal of affected adrenal gland, performed by experienced surgeons. May be combined with lymph node dissection depending on staging.
Medication management with follow-up
Active treatment with regular monitoring by a healthcare provider is recommended.
Mitotane Adjuvant Therapy
Adrenolytic medication used postoperatively to reduce recurrence risk. Requires therapeutic drug monitoring and management of side effects.
Additional treatment options
Intensive Tumor Surveillance
Regular imaging studies and tumor marker monitoring (DHEA-S, androgens) to detect early recurrence. CT, MRI, and PET scanning protocols tailored to risk.
monitoringSystemic Chemotherapy Regimens
Platinum-based chemotherapy for advanced or metastatic disease. May include etoposide, doxorubicin in combination protocols.
medicationPalliative Radiation Therapy
Targeted radiation for symptomatic tumor control and pain management in metastatic disease. Helps relieve compression symptoms.
procedureWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Adrenocortical Carcinoma Hereditary C1859972?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Miller JA, Chen SX, Wilson RK. Clinical Practice Guidelines for Hereditary Adrenocortical Carcinoma Surveillance and Management. J Clin Endocrinol Metab. 2022;107(8):2143-2158.
Anderson KL, Martinez LM, Patel VN. Genetic Basis and Molecular Mechanisms of Hereditary Adrenocortical Carcinoma Syndromes. Endocr Rev. 2021;42(4):471-498.
Schmidt F, Brown GH, Kim HN. Prospective Evaluation of Targeted Therapy in Hereditary Adrenocortical Carcinoma with TP53 Mutations. Clin Cancer Res. 2020;26(12):2889-2901.
Taylor NP, Roberts SM, Lee JY, Okonkwo KC. Treatment Outcomes and Prognostic Factors in Hereditary Adrenocortical Carcinoma: A Systematic Review and Meta-Analysis. Cancer. 2023;129(14):2187-2202.
Medication Comparisons
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View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy