Xanthogranulomatous adrenalitis in a neonate: CT and US findings
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- Trinavarat, P., Sasiwimonphan, K., Sansopha, L. et al. Pediatr Radiol (2009) 39: 286. doi:10.1007/s00247-008-1075-7
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We report a 46-day-old female infant with xanthogranulomatous adrenalitis. Ultrasonography showed a complex, solid-cystic right suprarenal mass with poorly defined margins. Colour flow Doppler revealed the solid portion of the mass to be vascular. CT demonstrated a heterogeneous cystic and solid mass with some contrast enhancement in the inferior part of the lesion. There was compression of the adjacent upper pole of the right kidney and the lateral aspect of the inferior vena cava. The mass was completely removed; histological examination revealed xanthogranulomatous adrenalitis. At the time of this report she remained well 3 years following surgery.
Retroperitoneal xanthogranuloma is a benign process of retroperitoneal tissues, especially of the kidney has been well documented in the urological literature since the first report in the mid 1930s . Xanthogranuloma of the adrenal gland is extremely rare. Only a few cases have been reported in the medical literature, the first by Grimi et al.  in 1987 and then by Atiemo et al.  in 2003. Here we report a further case of xanthogranuloma of the adrenal gland in a 46-day-old girl.
A 46-day-old female infant was referred for further investigation and management having been found to have hepatosplenomegaly, anaemia, and a right adrenal mass. She was born following a normal delivery, birth weight 4,260 g, but with severe birth asphyxia and a left Erb palsy. Her APGAR scores at 1 and 5 min were 1 and 3, respectively. Her neonatal life was complicated by sepsis and jaundice. Her first admission to hospital was at 27 days of age. The second admission was 1 week later because of progressive jaundice, pale stool, and frequent vomiting. Ultrasonography and CT scan at age 42 days revealed a 4.6×3.4-cm right suprarenal mass, and a diagnosis of adrenal haemorrhage was made. Her cholestatic jaundice was shown to be due to neonatal hepatitis, and this was resolving spontaneously during the second admission. However, the hepatosplenomegaly persisted and she was documented to be anaemic (haematocrit 28%); hence the referral to our institution.
An adrenal tumour, particularly neuroblastoma, was considered. However, the poorly defined margin was considered unusual. Furthermore, other investigations revealed urinary vanillylmandelic acid to be not elevated (0.8 mg/24 h; normal 1.5–10 mg), skeletal survey was normal, and bone marrow aspirate showed normocellular marrow without metastasis, but with erythroid hyperplasia.
Open biopsy of the mass was undertaken at 63 days of age. On operation, the right adrenal mass looked like tumour rather than haemorrhage. It extended behind the inferior vena cava, as demonstrated by CT and US. There was no lymph node enlargement. The mass was not adherent to other structures and a solid 4×3×3-cm mass was totally removed without difficulty.
Hepatosplenomegaly was diagnosed to be due to congenital cytomegalovirus (CMV) infection; CMV IgM was positive. Liver biopsy was performed concurrently with the adrenalectomy. Hepatic architecture was intact, but with minimal fatty change. Masson’s trichrome stain demonstrated fibrous portal expansion. These changes were regarded as nonspecific.
The anaemia was hypochromic and normocytic with polychromasia, and was regarded as most likely due to physiological anaemia in the newborn exacerbated by the multiple venepunctures she had undergone. Adrenal haemorrhage, as evidenced by haemosiderin-laden macrophages within the resected adrenal mass, was another possible cause of blood loss. Following the administration of packed red cells the haematocrit increased from 22.6% to 34%. At 3 years of age her haematocrit was 36% without any medication; hepatosplenomegaly had resolved.
Xanthogranulomatous process is a recognized histopathological entity affecting various organs, and is characterized by the presence of granular histiocytes in the initial stage, foamy lipid-laden macrophages, plasma cells, suppurative foci and haemorrhage .
There have been several reports of retroperitoneal xanthogranulomatous disease in children, mainly affecting the kidney [5–7]—xanthogranulomatous pyelonephritis (XGP). XGP is an atypical form of chronic renal parenchymal infection that may show extrarenal extension to involve other retroperitoneal organs and adjacent structures [5, 7], but other retroperitoneal organs are rarely the origin of this inflammatory process. Leukocytosis, raised erythrocyte sedimentary rate, and anaemia are often present [5, 6]. There are only a few cases of xanthogranulomatous pancreatitis and xanthogranulomatous adrenalitis (XGA) in the medical literature and all have been in adults.
To the best of our knowledge, there have been only two reported cases of XGA, in 1987  and 2003 . The latter was a 59-year-old man who had undergone nephrectomy 15 months previously because of renal cell carcinoma, and the cause of the XGA was postulated to be from an inflammatory process in the postoperative period . However, the exact aetiology is unknown.
In children, retroperitoneal xanthogranulomatous inflammation from organs other than the kidney is extremely rare with no cases identifiable from a MEDLINE search.
In our case, the girl was found to have a large right adrenal mass at 42 days of age and surgery at 62 days revealed XGA. Looking back in her life, she had severe birth asphyxia, and that could have caused adrenal haemorrhage. The finding of scattered haemosiderin-laden macrophages supports adrenal haemorrhage. Furthermore, she had been systemically infected since birth and had been treated with antibiotics because of sepsis. The adrenal gland might have been infected from a very early age. We postulate that haemorrhage and prolonged infection of the adrenal gland were the contributing factors in the development of XGA in this case. Fibrosis, which had been histologically described in the reported adult case of XGA , was scantly found in this case; probably insufficient time had elapsed for its development.
From the clinical and imaging standpoints, the major differential diagnoses for an adrenal mass in the first few months of life are adrenal haemorrhage and neuroblastoma. Other possibilities are rare and include adrenal abscess due to group B streptococcus, Escherichia coli, or Staphylococcus aureus . Findings here that suggest infection or inflammation were the poorly defined margins of the mass, leukocytosis, and the history of systemic infection.
In conclusion, XGA is extremely rare, in both children and adults. It may mimic tumour on imaging. This case highlights the need for a broader differential diagnosis when approaching the adrenal mass, even in a very young infant.