ODP025 Adrenocortical Carcinoma: A Rare but Life-Threatening Malignancy
Authors: Ortiz-Rivera Monica , Ortega Nydia Burgos , Madera-Marin Luis , Burgos Alexandra Rosado , Cordero Nicole Hernandez , Musa-Pena Laurianne E , Nieves Yadiel Rivera , Vick Margarita Ramirez , Santiago Milliette Alvarado , Rodriguez Loida Gonzalez 
Published: November 2022

Introduction

Adrenocortical carcinoma (ACC) is a rare malignancy of the adrenal glands, but often presents an aggressive behavior with variable prognosis. Median age of diagnosis is in the fifth to sixth decade of life with a median survival rate of 3-4 years. Complete surgical resection is the first line and only curative therapy. In patients with high-risk ACC, adjuvant therapy with mitotane, alone or in combination with other cytotoxic agents, may be used.

Case description

Case of a 33-year-old female with history of thyroid nodules presenting with a one year history of amenorrhea and hirsutism. One year prior to evaluation, the patient developed an intense right flank pain requiring abdominal-pelvic CT. It showed a solid heterogeneous right flank mass measuring 16×16×12 cm, 44Hounsfield units without enhanced contrast washout. Hormonal workup at that time resulted with DHEA-S &gt; 10,000ng/mL(nl 350-4300), androstenedione 10.7 ng/mL (nl 0.35–2.78), total testosterone 3.21 ng/mL (nl &lt; 0.52), midnight salivary cortisol at 14 nmol/L (nl &lt; 3.6) and normal FSH, ACTH and serum morning cortisol. Staging chest CT exhibited bilateral pulmonary embolisms. Patient underwent right open total adrenalectomy of a necrotic/hemorrhagic mass measuring 20×16×9 cm with complete resection. Histology consistent with a low-grade ACC with a mitotic rate 18/50 HPF, with capsular invasion but no lymphovascular invasion and clear margins. It was stratified as stage T2N0M0. Immunohistochemistry's were positive for Melan-A, calretinin, synaptophysin, and inhibin. Ki67 was 80%. She was started on hydrocortisone replacement. PET/CT scan showed hypermetabolic focus at the right thyroid lobe (previously biopsied with benign findings), but otherwise no pathologic 18 FDG uptake related to known oncologic disease. Due to her high risk of recurrence, the patient was started on mitotane 2 g daily as adjuvant therapy but had to discontinue therapy after 1 month due to lack of availability. One year after initial presentation, she was evaluated at our clinics and after discussion with Oncology Service restarting mitotane was recommended. However, marked transaminitis was noted along with right abdominal discomfort. Abdominal MRI revealed hepatic hypo-enhancing masses suggestive of liver metastases, with the largest measuring 6.7cm. Patient underwent right partial hepatectomy and was found with extensive metastatic carcinoma and lymph node involvement. Therapy with mitotane was restarted, pending chemotherapy initiation.

Conclusion

The goal of any therapy for ACC is to alleviate symptoms and prolong survival. Mitotane, an adrenolytic drug used as palliative treatment, is among therapeutic options. Due to the rarity of the disease, data regarding its use is mixed due to limited randomized controlled trials. Nevertheless, it supports improvement in recurrence-free and possibly overall-survival after complete radical resection in stage I, II or III ACC. Unfortunately, there is still marked individual variation in outcomes.

Presentation: No date and time listed