Pediatric Radiology

, 39:286

Xanthogranulomatous adrenalitis in a neonate: CT and US findings

Authors

  • Panruethai Trinavarat
    • Department of Radiology, Faculty of MedicineChulalongkorn University, King Chulalongkorn Memorial Hospital
    • Department of Radiology, Faculty of MedicineChulalongkorn University, King Chulalongkorn Memorial Hospital
  • Lalana Sansopha
    • Department of Pathology, Faculty of MedicineChulalongkorn University, King Chulalongkorn Memorial Hospital
  • Paisarn Vejchapipat
    • Department of Surgery, Faculty of MedicineChulalongkorn University, King Chulalongkorn Memorial Hospital
  • Darintr Sosothikul
    • Department of Paediatrics, Faculty of MedicineChulalongkorn University, King Chulalongkorn Memorial Hospital
Case Report

DOI: 10.1007/s00247-008-1075-7

Cite this article as:
Trinavarat, P., Sasiwimonphan, K., Sansopha, L. et al. Pediatr Radiol (2009) 39: 286. doi:10.1007/s00247-008-1075-7

Abstract

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.

Keywords

AdrenalXanthogranulomatous adrenalitisNeonateCTUltrasound

Introduction

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 [1]. 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. [2] in 1987 and then by Atiemo et al. [3] in 2003. Here we report a further case of xanthogranuloma of the adrenal gland in a 46-day-old girl.

Case report

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.

At our hospital she was noted to be afebrile, not icteric, but pale. The abdomen was soft with a palpable liver 3 cm below the right costal margin and a palpable spleen 2 cm below the left costal margin. Complete blood count showed WBC 23,390/µl (neutrophils 39%, lymphocytes 50%, monocytes 9.9%), haemoglobin 8.1 g/dl, and haematocrit 24.8%. The previous CT scan was reviewed and was interpreted as inconsistent with adrenal haemorrhage. The right suprarenal mass had heterogeneous cystic and solid components with some contrast enhancement in its inferior part (Fig. 1). There was also soft-tissue extension of the lesion into the retrocaval area, either as a result of direct extension or from coalescence of the mass with an enlarged retrocaval node. Ultrasonography performed in our hospital at age 49 days showed a 5.4×3.1-cm right suprarenal mass having solid and cystic components with poorly defined margins. Colour Doppler US showed vascular flow within the solid portion (Fig. 2).
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Fig. 1

Contrast-enhanced CT images of the upper abdomen reveal a large, complex right suprarenal mass. a The upper portion of the mass has a large cystic component (arrows) with a smooth thick wall. The inferior vena cava (arrowhead) is anteromedial to the lesion and is mildly compressed. b, c The lower portion of the mass has a solid component (arrows) with heterogeneous enhancement. The perirenal fat plane is obliterated (double arrows). The inferior vena cava (arrowheads) is partially encased

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Fig. 2

US images of the upper right side of the abdomen in the sagittal (a) and transverse (b, c) planes show a complex, solid-cystic right suprarenal mass (arrows). b The superior cystic part has a thickened wall. c The inferior solid part shows no identifiable fat plane from the adjacent liver, kidney, or head of the pancreas (H). The inferior vena cava is compressed (double arrows). d Colour Doppler image demonstrates vascularity of the solid portion and its wall (arrow)

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.

The cut surface revealed a dark-brown solid component and friable yellow–orange tissue, without pus, cystic space, or necrosis. Histological examination (Fig. 3) showed a large area of inflammation containing abundant foamy histiocytes mixed with lymphocytes, plasma cells, and multinucleated giant cells. Adrenal tissue was observed at the periphery of the mass. Diffuse bright yellow haemosiderin pigments in macrophages were present throughout the inflamed area. Scant fibrosis was noted. Inflammation extended into the periadrenal fat. Pathological diagnosis was xanthogranulomatous inflammation of the adrenal gland and the culture showed no organisms.
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Fig. 3

Histology. a H&E ×40. Normal adrenal gland (1) is seen at the periphery of the mass. The adjacent tissue displays organizing inflammation (2) and xanthogranulomatous inflammation (3). b H&E ×400. The xanthogranulomatous inflammatory area shows abundant foamy histiocytes mixed with lymphocytes, plasma cells and some multinucleate giant cells. Some yellowish haemosiderin is seen in macrophages

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.

Discussion

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 [4].

There have been several reports of retroperitoneal xanthogranulomatous disease in children, mainly affecting the kidney [57]—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 [2] and 2003 [3]. 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 [3]. 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 [3], 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 [8]. 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.

Copyright information

© Springer-Verlag 2008