Endocrine Abstracts

A 76-year-old lady was admitted to a Spanish Hospital with vertigo and dizziness. Investigations revealed hypercalcaemia and a right adrenal mass on an MRI abdomen. On returning to the UK, she was referred to endocrinology. Additional symptoms included fatigue, memory loss and some leg weakness over the preceding months. She was noted to be hypertensive, controlled on Amlodipine 5 mg od. Investigations revealed an elevated serum corrected calcium of 2.85 mmol/l and serum PTH raised at 13.4 pmol/l. Mild hypercalcaemia had been present for five years. The Non-contrast CT adrenals showed a heterogeneous mass arising between the right kidney and the right adrenal (4.6 × 4 × 4.3 cm) with an average peak density of 35 Hounsfield Units. This mass was not present on a previous CT seven years earlier. Adrenal hormone investigations including urinary metanephrines and plasma Aldosterone: Renin ratio were unremarkable A sestamibi SPECT CT scan revealed a 12 mm parathyroid adenoma in the right inferior to the lower right thyroid lobe which was confirmed on ultrasound. Following adrenal MDT discussion she underwent CT scan- Chest Abdomen and Pelvis followed by a robotic right adrenalectomy. The histological examination of the adrenal mass revealed that the tumour is diffusely positive for smooth muscle myosin and caldesmon, and there is patchy positivity for smooth muscle actin and desmin. The proliferation fraction on ki67 stainings is around 5-10%. Features and immunoprofile were compatible with an adrenal leiomyosarcoma with incomplete local excision. Surgical parathyroidectomy has been delayed until a period of time has elapsed and reassured that all is stable from the sarcoma perspective.

Conclusion: Primary adrenal leiomyosarcoma (PAL) is extremely rare, with less than 50 cases reported worldwide. PAL is a mesenchymal tumour that originates from the smooth muscle wall of the adrenal vein. They are usually large and grow rapidly. The incidence increases with age. Most PAL tumours do not secret adrenal hormones. There are no specific tumour markers or imaging characteristics that easily enable a preoperative diagnosis. Surgery is the mainstay of treatment. Adjuvant chemotherapy or radiotherapy is often used for PAL patients with poor prognosis. postoperative adjuvant radiation therapy is recommended for the treatment of locally advanced malignancy. Hyperparathyroidism is an uncommon endocrine disorder. There is no known association between adrenal leiomyosarcoma and hyperparathyroidism. This case represents the first known case of these conditions existing simultaneously.