| Anne Smets | Coexistence of an adrenocortical |
| Koen Mortelé | |
| Nathalie De Wever | carcinoma with an abdominal |
| Yves Benoit | |
| Marleen Praet | ganglioneuroma in a child |
| Marc Kunnen |
Received: 15 January 1997 Accepted: 15 October 1997
A. Smets · K. Mortelé · M. Kunnen Department of Radiology, University Hospital Gent, 9000 Gent, Belgium
Y. Benoit Department of Paediatric Oncology, University Hospital Gent, 9000 Gent, Belgium
N. De Wever . M. Praet Department of Pathology, University Hospital Gent, 9000 Gent, Belgium
A. Smets ☒ Academic Medical Center, University of Amsterdam, Department of Radiology, P.O. Box 22700 1100 DE Amsterdam, The Netherlands
Abstract We report a 3-year, 5-month-old boy with an adrenocor- tical carcinoma. These tumours are rare and highly malignant in child- hood. In most cases they are func- tional, secreting adrenocortical hormones. In this case there was a misleading coexistence with a sec- ond abdominal neoplasm, which was a ganglioneuroma; this is a rare be- nign tumour arising from the sym- pathetic nervous system. The imaging investigations and their findings are discussed and corre- lated with pathology.
Introduction
Adrenocortical carcinoma is a rare tumour in childhood, although it occurs with a high incidence in Southern Bra- zil [1]. Association with the Li-Fraumeni and Beckwith- Wiedemann syndromes has been reported, as well as an increased risk of second primary cancers suggesting a strong genetic basis [2]. Ganglioneuromas are rare be- nign tumours arising from the sympathetic ganglia.
Case report
A 3-year, 5-month-old boy was referred to our hospital because of generalised seizures and coma. The patient had a 3-week history of headache, increase in weight and a bloated face. Physical examina-
tion revealed an unconscious child with generalised hypertonia and a blood pressure of 140/90 mmHg. Clinical examination of the heart, lungs and abdomen was normal. Cerebral CT was normal. Ul- trasound examination of the kidneys showed caudal displacement of the right kidney by a large hypoechoic, well-circumscribed mass (4.7 × 4.2 cm) with a hyperechoic centre. An intravenous pyelo- gram confirmed the displacement of the right kidney.
Abdominal CT showed a hypodense right suprarenal mass with slight inhomogeneous enhancement after intravenous contrast ad- ministration (Fig.1). There was also a homogeneous hypodense, small soft-tissue mass in the retrocrural and right para-aortic space, which was considered to be an enlarged lymph node. Laboratory data showed normal urinary catecholamines and metabolites as well as non-suppressible cortisol levels. A 120-g right adrenal tu- mour was removed during exploratory laparotomy. The histologi- cal diagnosis was an adrenocortical carcinoma. Because of tumour rupture during surgery, chemotherapy (cisplatinum, vincristine, cy- clophosphamide and etoposide) and radiotherapy were given.
JL
After 2 months of adjuvant therapy, the retrocrural mass was still present and had slightly increased in volume on CT examina- tion (Fig.2). A second exploratory laparotomy showed no signs of local tumour recurrence in the right adrenal fossa. A hard white mass with a mean diameter of 2 cm was removed from the retrocrural region. The histological diagnosis of this mass was gan- glioneuroma. Follow-up examination 2 months later showed recur- rence of the disease with hepatic metastatic infiltration and enlarged lymph nodes (Fig.3). Laboratory results showed active se- cretion of adrenocortical hormones (cortisol and androgens). Inter- mittent hypertensive encephalopathy was present. Treatment was restricted to the adrenocytolytic agent mitotane. The patient died of hypertensive encephalopathy 9 months after tumour resection.
Discussion
This case report concerns a child with coexisting adrenocortical carcinoma and abdominal ganglioneuro- ma which, to our knowledge, has not been previously re- corded. Adrenocortical carcinomas are rare tumours. They are more common in females than in males (2.3:1) and 75% of the patients are under the age of 5 years [3]. A significantly higher incidence has been re- ported in southern Brazil [1]. Adrenocortical carcinoma has been reported in association with clinical syndromes such as hemihypertrophy and the Beckwith-Wiedemann and Li-Fraumeni syndromes [3]. An increased risk of second primary cancers has also been described [2]. These associations suggest a strong genetic basis for childhood adrenocortical carcinoma. In most childhood cases, the tumours produce adrenocortical hormones, especially androgens causing precocious puberty and vi- rilisation [1]. Most of the tumours are large at the time of diagnosis and tend to be locally aggressive and inva- sive. Metastatic spread occurs to lung, liver, spleen, lymph nodes and bone. Radiographic studies are helpful in confirming the diagnosis and, more importantly, in lo- cating the tumour. Haemorrhage and necrosis are com- mon, causing an inhomogeneous appearance to the tumour on imaging studies. On ultrasound, a stellate echogenic lesion caused by radiating scars (‘scar-sign’) has been described [4]. Treatment consists of surgery, and the survival rate is good when total resection of the tumour is possible. Chemotherapy is given when the tu- mour is inoperable or only partially excised and in case of recurrence and metastatic disease.
The retrocrural space is a small and triangular region of the prevertebral space, delineated anteriorly by the two diaphragmatic crura. It contains the aorta, nerves, veins of the azygos system, lymph nodes, cisterna chyli and the thoracic duct [5]. Pathological lesions arising in the retrocrural space are restricted to processes origi- nating from these structures, which explains the pres- ence of a ganglioneuroma. In paediatric patients especially, differential diagnosis should include lymph- adenopathy and nerve sheath tumours [5]. Ganglio-
neuroma is a rare and benign tumour arising from the sympathetic nervous system. Ganglioneuromas are usu- ally found in the posterior mediastinum (43 % ), the ret- roperitoneal space (32%) or the neck (8%). Sixty percent of patients with ganglioneuroma are younger than 20 years. There is a slight female predominance. Most of the patients are asymptomatic [6]. The tumours usually have no hormonal activity and secrete no cate- cholamines or metabolites. On sonography, they pro- duce a low-level, uniform echogenicity with smooth, well-defined margins. CT is more sensitive in detecting calcification, which is an occasional finding (20%) in
ganglioneuromas and is usually amorphous and less fre- quently punctate [7]. The tumour does not enhance after contrast medium administration or shows only slight enhancement at its periphery. On MRI, the tu- mour is hypointense on T1-weighted images and mark- edly hyperintense on T2-weighted images [6].
In summary, the coexistence of two different tu- mours, adrenal carcinoma and an abdominal ganglio- neuroma, caused a radiological pitfall. There is an increased risk of other tumours with adrenocortical car- cinoma, but this coexistence has not been described be- fore.
References
1. Sabagga CC, Avilla SG, Sculz C, et al (1993) Adrenocortical carcinoma in children: clinical aspects and prognosis. J Pediatr Surg 28: 841-843
2. Wagner J, Portwine C, Rabin K, et al (1994) High frequency of germline p53 mutations in childhood adrenocor- tical cancer. J Natl Cancer Inst 86: 1707-1710
3. Lee PDK, Winter RJ, Green OC (1985) Virilizing adrenocortical tumors in childhood: eight cases and a review of the literature. Pediatrics 76: 437-444
4. Prando A, Wallace S, Marins JLC, et al (1990) Sonographic findings of adreno- cortical carcinomas in children. Pediatr Radiol 20: 163-165
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6. Ichikawa T, Koyama A, Fujimoto H, et al (1993) Retroperitoneal ganglio- neuroma extending across the midline: MR features. Clin Imaging 17: 19-21
7. Armstrong EA, Harwood-Nash DCF, Fitz CR, et al (1982) CT of neuroblas- tomas and ganglioneuromas in children. AJR 139: 571-576