Endocrine Abstracts

Introduction: Adrenal schwannomas are very rare tumors that are difficult to diagnose properatively. They represent only 1–3% of all retroperitoneum masses.

Case report: A 30-year-old woman was referred to the outpatient to evaluate an adrenal mass incidentally found on abdominal ultrasonographic images obtained for self-limit abdominal pain. Her medical and family histories were unremarkable. Clinically asymptomatic, only referred amenorrhea for two months. Physical examination showed BMI 20 kg/m², blood pressure 125/75 mmHg, pulse rate 88 lpm. The rest of the exploration was within normal. The patient’s endocrinologic data were as follows: Plasma metanephrine 34 pg/ml (<65), plasma normetanephrine 52 pg/ml (<169), urinary metanephrine 240 μg/24 h (<341), urinary normetanephrine 189 μg/24 h (<444), urinary vanillymandelic acid 2.8 mg/24 h (< 9), Cromogranine A <5 ng/ml, DHEA-S 0.8 mcg/ml (<4), Testosterone 0.8 ng/ml (<0.48), urinary free cortisol 124.1 μg/24 h (<176), Aldosterone 217 pg/ml (<160), PRA > 11.5 ng/ml per hour (<5.7), LH 0.1 mUI/ml, FSH 0.1 mUI/ml, Estradiol 1502 pg/ml, Progesterone 39.9 ng/ml, hCG 161250 mUI/ml (compatible with pregnancy of 6–12 weeks). Ultrasonography of the abdomen demonstrated a well-circumscribed hypoechoic mass with cystic and necrotic components that measured 32×27 mm, dependent of the right adrenal gland. MRI without gadolinium (2nd trimester) showed a 40×32 mm heterogeneous adrenal mass with necrotic and cystic components, located in the right adrenal gland, low intensity on T1-weighted images and high intensity on T2-weighted images, suggestive of pheochromocytoma as the most likely option, without being able to rule out an adrenal carcinoma. With these results we request a gynecological examination; which showed a 6-week gestation. After much deliberations, a laparoscopic adrenalectomy was performed under the suspicion of a malignant tumor or a non-functioning pheocromocytoma, in week 22 of gestation. Microscopically, the surgial specimen showed a tumor formed by fusiform cells, without atypia, with a swirling pattern and degenerative areas.Tumor cells express s100 protein and the proliferative index ki67 was 1%. The diagnosis was an ancient adrenal schwannoma. After surgery, pregnancy and delivery developed normally.

Conclusions: Adrenal swchannomas are extremely rare tumors, specially during pregnancy. Surgical resection is the primary means of management of adrenal schwannomas, as it is not possible to distinguish the schwannoma from malignant entities or pheocromocithoma simply base on imaging. In pregnant woman, like our patient, adrenalectomy should be performed in the second trimester, between weeks 12 and 24 of gestation.