Sarcomatoid adrenal cortical carcinoma (SACC) is an uncommon variant of adrenocortical carcinoma (ACC incidence 0.5-2 cases/million population), with an aggressive nature and poor prognosis. We present a case of SACC in a 54-year-old woman, followed since 2019 for an incidentally found 2.8 cm right adrenal nodule, which increased in size to 10.6 cm in 1/2022. Biochemical work-up was unremarkable except a mildly elevated dopamine. An adrenalectomy specimen revealed a 484 gram, 12x9.6x7.1 cm, biphasic, mostly MFH-like sarcomatoid high-grade neoplasm with morphology and immunoprofile compatible with SACC (Table). SACC invaded into the adherent fragment of the diaphragm, liver parenchyma, and IVC wall. 1/2 lymph nodes were positive (Stage III, pT4 pN1 cM0). NGS performed at Tempus revealed one genomic potentially actionable variant, three biologically relevant variants, one FDA-approved therapy and one potential therapeutic option (Table). The patient underwent 6 cycles 1st-line cisplatin/etoposide combined with pembrolizumab immunotherapy and is on maintenance pembrolizumab q21d since 08/2022. CT scan in 7/2024 showed no evidence of disease, 2.5 years post SACC diagnosis. We are the first to report a case of SACC with actionable genomic features, and subsequent successful novel personalized treatment, a premise of personalized medicine. Further studies are needed to examine the influence of personalized treatment on the outcome of patients with this rare aggressive carcinoma.
Diagnosis of sarcomatoid adrenal cortical carcinoma in the era of personalized medicine
Diagnosis of sarcomatoid adrenal cortical carcinoma in the era of personalized medicine
1.
Kenyon K, Enomoto L, Faulkner N, Orucevic A. Diagnosis of sarcomatoid adrenal cortical carcinoma in the era of personalized medicine. IJCCD. Published online October 11, 2024. doi:10.53876/001c.124502