Cystic lymphangioma of the adrenal gland: report of a case and review of the literature - Beyond the Abstract

This case report was written in order to reflect the difficulty and challenge that we faced to make the diagnosis of the described patient. The patient indeed presented with constant abdominal pain for several weeks. After normal laboratory tests, the first radiological images by ultrasonography and CT-scan showed a cystic lesion of the left adrenal gland giving a large differential diagnosis.

As no sign of malignancy or worrisome findings were noted, a symptomatic treatment with first level painkillers (paracetamol and non steroidal anti-inflammatory drugs as described by the World Health Organization pain relief ladder) was first undertaken. As the treatment was unfortunately unsuccessful and to have a more precise diagnosis, it was decided to perform an MRI and a CT-guided puncture of the cyst.

The MR images strongly evocated a cystic lymphangioma of the adrenal gland. The CT-guided puncture - in addition to show absence of malignant cells - also allowed aspirating a certain fluid quantity from the cyst. During the percutaneous procedure it was not possible to remove all the fluid because of a dysfunctional guide wire. At first, it seemed that the evacuation of the cyst liquid helped to relieve the symptoms, but unfortunately the pain reappeared quickly after the puncture. This confirmed that aspiration just brings a transient amelioration and that recurrence reappears rapidly. At that moment, as the cystic lymphangioma was symptomatic after failures of conservative treatment and percutaneous puncture, surgical removal of this lesion was indicated, and it permitted to suppress the patient symptomatology.

Cystic lymphangioma of the adrenals is a rare clinical entity that physicians seldom meet in clinical practice. The abdominal lymphangiomas account for only 5% of all lymphangiomas, and lymphangiomas of the adrenal glands only account for a minor part of all intra-abdominal lymphangiomas. Cystic lymphangioma of the adrenals should nevertheless be included in the differential diagnosis of a cystic adrenal tumor.

Surgery remains the definitive treatment of choice if the lesion is symptomatic, and it permits to avoid complications of the cyst, like intra-cystic hemorrhage or cyst enlargement.

This article based on a report of a case allowed us to perform a concise review of the current evidences of this rare clinical entity. We sincerely hope that this case report will help clinicians in the future when they encounter a patient with a cystic lesion of the adrenal glands.

Written by:
Gaƫtan-Romain Joliat, MD
Department of Visceral Surgery, University Hospital of Lausanne (CHUV), Lausanne, Switzerland.

Abstract: Cystic lymphangioma of the adrenal gland: report of a case and review of the literature