CASE 10860 Published on 26.03.2013

Retroperitoneal haemorrhage secondary to spontaneous rupture of giant adrenal myelolipoma

Section

Abdominal imaging

Case Type

Clinical Cases

Authors

Javier Fernandez-Jara; Carlos Poyo Calvo; Nieves Alegre Bernal; Begoña Corral Ramos

Hospital Universitario Severo Ochoa,
Radiodiagnostico;
Avenida Orellana s/n
28911 Leganes, Spain;
Email:javierfernandez_jara@hotmail.com
Patient

36 years, male

Categories
Area of Interest Adrenals ; Imaging Technique CT, RIS
Clinical History
36-year-old male patient with no relevant medical history was admitted with right flank pain of sudden onset that radiated to upper quadrant and hypogastric, accompanied by nausea, vomiting and sweating. No fever.
On physical examination, a painful mass was palpable in the right upper quadrant. Laboratory tests were normal.
Imaging Findings
The CT showed a large heterogeneous mass located between the right kidney and liver of 15 x 16 cm in size. The right adrenal gland could not be identified. The relationship between the mass and the upper pole of the right kidney was unclear on axial images. Coronal and sagittal reconstructions showed a displaced and compressed but intact superior renal pole, confirming the right adrenal mass (Figs. 1 and 2).
The lesion had heterogeneous attenuation, with predominant areas of fat attenuation and high-density areas in relation to bleeding. Intravenous injection contrast extravasation showed active bleeding inside the tumour (Figs. 1 and 2).
Because of the patient´s haemodynamic instability, an emergency laparotomy was performed. It revealed a haemorrhagic adrenal tumour with pathological diagnosis of adrenal myelolipoma (Fig. 3).
Discussion
Adrenal myelolipomas are benign, rare, non-functioning tumours, composed of a variable mixture of fat and mature haematopoietic elements [2, 3, 5, 7, 8]. The large size tumours are rare clinical entities. They occur in patients between the fourth and sixth decade of life, although cases have been reported in all age groups [4]. It affects men and women similarly and they are usually unilateral and occasionally bilateral [1].
Although the vast majority of myelolipomas arise in the adrenal cortex, extraadrenal locations (lung, liver and retroperitoneum) have been described [1, 3, 5, 6]. The pathogenesis of these tumours is not clear.
A recent study described the translocation (3; 21) (q25, p11) associated with adrenal myelolipoma, possibly indicating that it is a real neoplasia originating from haematopoietic tissue [2].
They are usually asymptomatic, being diagnosed during imaging tests or surgical explorations, taken for another reason. Myelolipomas represent 9% of all incidentally discovered adrenal masses [3]. The larger ones can produce symptoms by compression of adjacent organs, tumour necrosis or haemorrhage. Exceptionally, retroperitoneal bleeding may occur by spontaneous rupture of larger myelolipomas [2], as in our case.
They are not endocrinologically functioning, but they are associated with hypertension and endocrine diseases, such as obesity, Cushing syndrome, adrenal insufficiency or Conn syndrome [1, 2, 5].
Imaging tests play a key role in managing the symptomatic myelolipomas. CT is recommended as the best way to distinguish haemorrhagic myelolipoma from other conditions that may also suffer spontaneous bleeding [1].
Treatment depends on the patient haemodynamic status [7, 8]. There is no consensus on the management of bleeding myelolipomas; Routhier et al. reported a case without emergency surgical resection [3]. In general, treatment in the absence of complications must be conservative [8]. Our patient was unstable. However, given the large size of the lesion and because of active bleeding on CT, with massive retroperitoneal bleeding risk, emergency laparotomy was performed.
Although nontraumatic rupture of adrenal myelolipoma with spontaneous bleeding is a very rare association, the diagnosis should be suspected in a patient with a large haemorrhagic adrenal mass. CT detects bleeding, and allows in the appropriate clinical context to make a differential diagnosis and plan the surgery.
Differential Diagnosis List
Retroperitoneal haemorrhage secondary to spontaneous rupture of giant adrenal myelolipoma
Renal angiomyolipoma
Pheochromocytoma
Liposarcoma
Lipoma
Adrenal carcinoma
Metastasis
Final Diagnosis
Retroperitoneal haemorrhage secondary to spontaneous rupture of giant adrenal myelolipoma
Case information
URL: https://www.eurorad.org/case/10860
DOI: 10.1594/EURORAD/CASE.10860
ISSN: 1563-4086