Beyond Mitotane in a Patient With Highly Aggressive Adrenocortical Carcinoma
https://doi.org/10.15605/jafes.041.S1
- Author:
Muhammad Shukri Johar
1
;
Siti Sanaa Wan Azman
1
;
Dorothy Maria Anthony Bernard
1
;
Foo Siew Hui
1
Author Information
1. Endocrine Unit, Medical Department, Hospital Selayang
- Publication Type:Journal Article
- MeSH:
Adrenocortical Carcinoma;
Mitotane
- From:
Journal of the ASEAN Federation of Endocrine Societies
2026;41(S1):30-31
- CountryPhilippines
- Language:English
-
Abstract:
Introduction:Adrenocortical carcinoma (ACC) is an aggressive malignancy with high rates of recurrence even after surgical
resection. Surgery remains the mainstay of treatment,
while adjuvant options are limited. Mitotane is the only
approved systemic therapy. Current guidelines recommend
stereotactic body radiotherapy (SBRT) alongside adjuvant
mitotane therapy in Rx, R1, R2 resections and in locally
advanced disease.
Case:We present a case of a 40-year-old female who presented
with abdominal pain and was found to have a large
heterogeneous left adrenal mass measuring 8.2 × 8.5 × 9.5
cm (Hounsfield Unit 63) on computed tomography (CT)
imaging. Clinically, she was obese with a body mass index
of 33.7 kg/m². No discriminatory feature of Cushing’s was
present. Hormonal evaluation demonstrated autonomous
cortisol secretion with failure of suppression on both
overnight and low-dose dexamethasone suppression tests
at 301 nmol/L and 313.6 nmol/L, respectively. DHEA,
testosterone, and urinary metanephrine were within range.
Hemoglobin A1c was 6.6%. She underwent open left
adrenalectomy. Intra-operatively, a 12 × 10 cm adrenal tumor
was identified with multiple areas of tumor rupture and
spillage during mobilization. HPE confirmed high-grade
ACC with high Weiss score of 8, Ki-67 index 60–80%, and
mitotic count 54/50 hpf (pT2Nx). Post-operative CT imaging
demonstrated a residual soft tissue lesion in the left adrenal
bed (largest diameter 3.8 cm) with fluorodeoxyglucose
avidity. We commenced adjuvant mitotane therapy,
titrated to 2 g TDS with supraphysiological hydrocortisone
replacement. Mitotane level was within therapeutic range
(16 mcg/mL). She was deemed unsuitable for repeat surgery
due to the proximity of the residual mass to the adjacent
vessel and was planned for SBRT therapy after a multidisciplinary team discussion.
Conclusion:High-risk ACC with suspected residual disease remains a
therapeutic challenge. While mitotane remains the cornerstone of adjuvant therapy, SBRT may represent a promising
adjunctive local treatment modality in carefully selected
patients. Further studies are required to define its role in
improving local control and outcomes in ACC.
- Full text:202607281413420714EP_A022.pdf
