Leiomyosarcoma of the inferior vena cava posing as an adrenal incidentaloma

Correspondence to Dr Sinduja Ramanan; [email protected]

Background

An Adrenal incidentaloma is often called ‘disease of modern technology’.¹ True to its name, it is an incidentally detected lesion when a patient undergoes imaging for a non-adrenal disease or most often for non-specific symptoms. Once found on imaging, it adds onto the anxiety of the patient as well as to the burden of the treating doctor as malignancy and adrenal hypersecretion has to be ruled out. While the prevalence of these lesions in imaging ranges between 0.2% and 4.4%, the mean prevalence among autopsy studies was 2.3%, ranging between 1% and 8.7%, indicating that it is a more frequent finding and may not always warrant intervention.¹ These adrenal incidentalomas have a variety of differential diagnoses with the most common being a non-functioning adenoma (69%) followed by pheochromocytoma (10%), metastasis (7%), myelolipoma (8%), cortisol producing adenoma (5%), adrenocortical carcinoma(11%) adrenal cyst (5%) and ganglioneuroma (4%).²

Our patient was a 22-year-old woman who was incidentally found to have a retroperitoneal mass, which was provisionally diagnosed as a non-functioning adrenal tumour. However, postoperative biopsy report was leiomyosarcoma arising from inferior vena cava (IVC). These IVC leiomyosarcomas are rare tumours with an approximate incidence of 1:100 000.³ Just like other retroperitoneal tumours, they either remain asymptomatic for a long time and maybe incidentally diagnosed while undergoing imaging for other complaints or may present with non-specific symptoms such as vague abdominal pain, nausea, dyspnoea, easy fatiguability or weight loss. The only positive prognostic factor for this tumour is achieving negative margin through surgical resection of the tumour.

Case presentation

A 22-year-old woman presented with complaints of menorrhagia for 1 month with no other symptoms. She had no other complaints with no significant personal or family history.

Her general and systemic examinations were normal.

Investigations

Her ultrasound abdomen, done for her non-specific complaints, showed a lesion in the right suprarenal region for which she underwent contrast-enhanced CT (CECT). Her CECT showed a 10.8×9.2×10.6 cm heterogeneously enhancing soft tissue density mass (around 30 HU) in the right suprarenal region displacing and abutting segments 4, 5 and 6 of liver compressing and displacing IVC superiorly, with no evidence any metastasis elsewhere (figures 1 and 2).

Her plasma cortisol (midnight cortisol 2.46...