CASE REPORT
L.Y.J. Leung . W.Y. Leung . K.F. Chan . T.W. Fan K.W. Chung . C.H.S. Chan
Ruptured adrenocortical carcinoma as a cause of paediatric acute abdomen
Accepted: 18 April 2001 / Published online: 22 October 2002 @ Springer-Verlag 2002
Abstract Adrenocortical carcinoma (ACC) is rare in children. Its presentation is usually related to hormonal activity of the tumour. We report a case of childhood ACC that presented as an acute abdomen due to tumour rupture. This is the first reported case of a ruptured ACC as a cause of paediatric acute abdomen.
Keywords Adrenocortical carcinoma . Rupture . Acute abdomen · Children
Introduction
Adrenocortical carcinomas (ACC) are rare tumours in children that have different diagnostic characteristics than in adults. Most paediatric ACCs are functional, showing clinical evidence of hormonal disturbance. For non-functioning tumours, the presentations are usually related to the size of the tumour. ACCs seldom rupture. An acute abdomen due to tumour rupture as its initial presentation has not been reported. We report a case of a ruptured ACC as a cause of paediatric acute abdomen, with highlights on the radiological aspects.
Case report
A 5-year-old boy was admitted because of a sudden onset of ab- dominal pain. He was otherwise healthy; there was no history of trauma. The physical examination revealed diffuse tenderness on the right side of the abdomen with guarding. The vital signs were stable. The child was overweight (32 kg, >97th percentile), but
L.Y.J. Leung · K.F. Chan . T.W. Fan . C.H.S. Chan Department of Radiology and Imaging,
Queen Elizabeth Hospital,
8 Wylie Road, Hong Kong, China
E-mail: jleung8888@hotmail.com
W.Y. Leung . K.W. Chung Department of Surgery, Queen Elizabeth Hospital, 8 Wylie Road, Hong Kong, China
there was no evidence of Cushingoid features or hirsutism. Hae- matological studies showed a normal haemoglobin level, platelet count and neutrophil count, Biochemical tests revealed normal liver and renal function.
An abdominal radiograph showed a 5-cm mass lesion with a calcified rim in the right upper quadrant (Fig. 1). Sonographic (US) studies revealed a calcified mass in the right adrenal region, with fluid in the hepatorenal pouch (Fig. 2). Computed tomography (CT) confirmed a calcified right adrenal mass. Intraperitoneal hypodense fluid (50 Hu) suggesting a haemoperitoneum was noted (Fig. 3a). After IV contrast, there was evidence of contrast ex- travasation into the hepatorenal pouch in the arterial phase (Fig. 3b) and stasis of contrast in the delay phase suggestive of tumour rupture.
An emergency laparotomy was performed; a 4 × 6 × 9-cm ruptured right adrenal tumour with a haemoperitoneum was found. A right adrenalectomy was performed. Pathologic examin- ation revealed that the tumour was an ACC (Fig. 4). Adjuvant chemotherapy was given. The patient is currently being followed in
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the paediatric unit; no clinical or radiological evidence of tumour recurrence has been reported.
Discussion
Adrenocortical carcinoma is a rare tumour, with an annual incidence ranging from 0.5 to 2 cases per million population. Paediatric ACCs comprise only 0.2% of all childhood malignancies [6]. Most ACCs in children are hormonally active. The clinical presentation is usually related to steroid overproduction, including virilisation and Cushing’s syndrome [7, 8]. As there are no symp- toms from non-functioning tumours, these patients tend to present with abdominal pain, fullness, or a palpable mass only after the tumour has attained considerable size. It is rare for childhood ACC to present as an acute abdomen; the most common causes are appendicitis, mesenteric adenitis, intussusception, inflammatory bowel disease, Meckel’s diverticulitis, and gynaeological conditions (ovarian cyst, tubo-ovarian abscess).
Some of the radiological features of ACC were demonstrated in this case. The tumour was calcified, which can be seen in 30% of abdominal radiographs [3]. US is useful for confirmation of extrarenal and extra- hepatic locations [2]. CT has the added advantage of demonstrating contralateral adrenal and lung metastases [1]. Other radiological investigations include magnetic resonance imaging [5] and adrenal scintigraphy [4]. This case illustrates the importance of radiological
b Fig. 3a, b CT scan of abdomen. a Plain scan shows right adrenal mass (arrowhead). Intraperitoneal hypodense area suggests hae- moperitoneum (arrow). b Evidence of contrast extravasation to hepatorenal pouch (arrow) in arterial phase
investigations for diagnosis and providing guidance for surgical management of ACC.
References
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