Short answer · Medically reviewed summary · Last updated: 2026-05-08
Adrenal Cortical Carcinoma (ACC) is classified under ICD-10 code C74.0 (Malignant neoplasm of cortex of adrenal gland). In the older ICD-9 clinical modification system, Adrenal Cortical Carcinoma was categorized under code 194.0. What is the clinical significance of Adrenal Cortical Carcinoma? Adrenal Cortical Carcinoma (ACC) is a rare, aggressive endocrine malignancy that arises from the cortex of the adrenal gland.
ICD10 code of Adrenal Cortical Carcinoma (ACC) and ICD9 code
ICD-10 and ICD-9 codes for Adrenal Cortical Carcinoma (ACC), with classification details for clinicians, coders and patients.
Adrenal Cortical Carcinoma (ACC) is classified under ICD-10 code C74.0 (Malignant neoplasm of cortex of adrenal gland). In the older ICD-9 clinical modification system, Adrenal Cortical Carcinoma was categorized under code 194.0.
What is the clinical significance of Adrenal Cortical Carcinoma?
Adrenal Cortical Carcinoma (ACC) is a rare, aggressive endocrine malignancy that arises from the cortex of the adrenal gland. Because Adrenal Cortical Carcinoma can be functional (secreting excess hormones like cortisol, androgens, or aldosterone), patients often present with Cushing’s syndrome or virilization. Our community at DiseaseMaps.org currently supports 265 individuals navigating the complexities of this diagnosis, highlighting the need for specialized oncological care.
How is Adrenal Cortical Carcinoma categorized for billing and research?
Standardized coding is vital for tracking the epidemiology of Adrenal Cortical Carcinoma. While ICD-10-CM code C74.0 is the primary diagnostic identifier, clinicians may also use supplemental codes to describe the functional status of the tumor. Accurate coding for Adrenal Cortical Carcinoma ensures that patients receive appropriate insurance coverage for high-cost treatments, such as mitotane therapy or surgical resection.
What are the common staging and diagnostic considerations?
The diagnosis and management of Adrenal Cortical Carcinoma involve a multidisciplinary approach. Because Adrenal Cortical Carcinoma is rare—with an estimated annual incidence of 0.7 to 2.0 cases per million population—it is critical that diagnostic workups are performed at centers of excellence. Key diagnostic steps include:
- Biochemical testing: 24-hour urinary steroid profiling and serum hormone levels.
- Imaging: Computed Tomography (CT) or MRI of the abdomen to assess tumor size and vascular invasion.
- Pathological review: Assessment using the Weiss score to confirm the malignancy of the adrenal tissue.
- Genetic counseling: Evaluation for Li-Fraumeni syndrome (TP53 mutation) if the patient is young or has a family history.
Next steps
- Consult an endocrine oncologist at a high-volume academic medical center.
- Connect with the 265 members at DiseaseMaps.org to share experiences and coping strategies.
- Discuss clinical trials regarding targeted therapies for Adrenal Cortical Carcinoma with your medical team.
- Ensure your pathology reports are reviewed by a pathologist specializing in adrenal tumors.
Medical disclaimer: This information is for educational purposes only and does not replace professional medical advice, diagnosis, or treatment from your healthcare provider.
References
- NIH Genetic and Rare Diseases Information Center (GARD): Adrenal Cortical Carcinoma
- Orphanet: Rare Adrenal Cortical Carcinoma (ORPHA:187)
- American Association of Endocrine Surgeons (AAES) Patient Education
- National Cancer Institute (NCI) PDQ® Adrenocortical Carcinoma Treatment
