Abstract

BackgroundAdrenocortical carcinoma (ACC) is an uncommon malignancy with an estimated 15,400 new cases annually across the globe. The prognosis is generally poor as the disease is often already advanced at initial diagnosis due to non-specific symptoms. Even for local disease, recurrence after surgical resection is high. Treatment choices for advanced disease include mitotane, chemotherapy, ablation, chemoembolization, radioembolization, and external beam radiotherapy, with varying degrees of efficacy. To the best of our knowledge, there have only been two prior case studies of complete clinical and radiological response of stage 4 disease at 1 year and 2 years after yttrium-90 (90Y) microsphere selective internal radiation therapy (SIRT) of isolated hepatic metastases post-surgery and chemotherapy.Case presentationWe present a case of a 58-year-old man with metastatic ACC who was treated with 90Y resin microsphere (SIR-spheres) for local control of liver metastases leading to a surgically proven negative pathology after partial hepatectomy 7 months after SIRT. The patient was initially diagnosed with stage 1 ACC that progressed 6 years later to stage 4 disease with new liver metastases that were deemed unresectable at an outside institution. After review of the case at multidisciplinary tumor board, he was referred for liver directed therapy for local tumor control. Angiographic workup demonstrated partial extrahepatic supply to the tumors from the right inferior phrenic artery, which was successfully embolized on the day of SIRT for flow redistribution. As the patient was being treated with mitotane that suppresses steroid production, he developed post-SIRT adrenal crisis, which was successfully controlled with steroids, highlighting the need for pre SIRT stress dose steroids.ConclusionsThis case continues to add to the literature supporting 90Y radioembolization as an effective treatment for isolated hepatic ACC metastases. Our case is the first to demonstrate surgically proven negative pathology after radioembolization. Further prospective study is warranted to better establish efficacy as well as safety of SIRT for ACC liver metastases.

Highlights

  • Adrenocortical carcinoma (ACC) is an uncommon malignancy with an estimated 15,400 new cases annually across the globe

  • This case continues to add to the literature supporting 90Y radioembolization as an effective treatment for isolated hepatic ACC metastases

  • Three additional cases of 90Y selective internal radiation therapy (SIRT) from a single institution demonstrated prolonged overall survival in patients with hepatic ACC metastases when analyzed in combination with 2 cases of liver-directed therapy treated with Transcatheter arterial chemoembolization (TACE) [8]

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Summary

Conclusions

The goal of local treatment for ACC is curative, but this is only achieved 20% of the time. Options for systemic treatment of advanced ACC have been with an EDP-M schedule (etoposide, doxorubicin, and cisplatin plus mitotane) or a M-S schedule (mitotane plus streptozosin) [9]. These regimens have comparable overall survival (14.8 vs 12 months), but EDP-M has been favored due to improved response rates and progressionfree survival (5 vs 2.1 months) [2, 10]. Due to the acuity and severity of the condition, treatment should not be delayed by diagnostic tests This case continues to add to the literature supporting 90Y radioembolization as an effective treatment for isolated hepatic ACC metastases. Querel; GIST: Gastrointestinal stromal tumor; MBq: Megabecquerel; mg: Milligram; PET/CT: Positron emission tomography/computed tomography; PLT: Platelet; SIRT: Selective internal radiation therapy; SPECT: Singlephoton emission computed tomography; TACE: Transcatheter arterial chemoembolization

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