Endocrine Abstracts

Oncocytic adrenocortical neoplasms (OAN) were first described by Kakimoto et al. in 1986. Since then, only 160 cases have been reported in the literature. There are 3 categories of OANs: pure oncocytic, mixed oncocytic and ordinary adrenocortical with focal oncocytic changes. Although the majority of OANs are considered benign tumors, certain OANs may present similar characteristics to adrenocortical carcinoma, correct histological caractherisation is therefore crucial. OANs should be assessed using the Lin-Weiss-Bisceglia (LWB) score as the Weiss score may overestimate the malignant nature of these tumors.

We report the case of a 52-year-old man without medical history, addressed for exploration of a large adrenal mass discovered on self-palpation. The patient was completely asymptomatic, with no clinical sign of hormonal hypersecretion. Hormonal testing showed very high DHEAS levels and slightly elevated 17-OH progesterone. CT scan found a 17 × 16 × 11 cm, heterogeneous left adrenal mass, with calcifications and 34 HU spontaneous density. Open left adrenalectomy was performed, with an anatomopathological examination in favor of a 25 × 16.5 × 15 cm OAN weighing 2800 g, considered malignant according to the LWB score. After 2 years of CT scan monitoring every 6 months, a 8 mm pulmonary nodule was detected. The FDG-PET-CT was consistent with increased uptake in the micronodule and in an infracentrimetric lumbo-aortic lymphadenopathy. Biopsy of the pulmonary micronodule confirmed the metastatic origin of the initial OAN. After discussion in the COMETE multidisciplinary meeting, radiofrequency ablation of the pulmonary nodule was performed and the patient was started on adjuvant Mitotane treatment. CT and PET-FDG assessment carried out 4 months later did not find any increased uptake in the lumbo-aortic lymph node previously described. Pure functional OAN diagnosis, management and follow-up are still controversial. Due to the uncertain evolution profile of these tumors, close monitoring is still recommended in order not to ignore local or distant recurrence. Given the rarity of these tumors, no studies concerning adjuvant therapies have been performed. Each case must be discussed in multidisciplinary meetings of expert networks.