Zahir U. Sarwar Valerie L. Ward David P. Mooney Sylvia Testa George A. Taylor
Congenital adrenocortical adenoma: case report and review of literature
| Received: 31 March 2004 Revised: 21 May 2004 Accepted: 27 May 2004 Published online: 17 September 2004 @ Springer-Verlag 2004 Z. U. Sarwar ☒ · V. L. Ward D. P. Mooney . S. Testa . G. A. Taylor Department of Pediatric Radiology, Children's Hospital Boston, 300 Longwood Avenue, Boston, MA 02115, USA E-mail: zahir88@comcast.net | Abstract Congenital ardrenocortical neoplasms are exceedingly rare. Our review of the medical literature re- vealed 23 reported cases of adreno- cortical neoplasm including this one. Eighteen of these cases were adre- nocortical carcinoma and four were grouped as adrenocortical tumor. We have not found any reported case with a histological diagnosis of a congenital adrenocortical ade- noma. We present this case of a congenital adrenocortical neoplasm with histological findings consistent with an adrenocortical adenoma in a premature infant aged 27 weeks and 4 days who had a prenatal sonogram showing a cystic right abdominal mass and a physical examination demonstrating a palpable mass. | Keywords Adrenal . Neoplasm . Adrenocortical carcinoma · Adrenocortical adenoma · Congenital · Child |
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Introduction
The worldwide annual incidence of childhood adreno- cortical neoplasm ranges from 0.3 to 0.38 per million children less than 15 years old [1]. These neoplasms are even more unusual in infants and our search of the med- ical literature revealed 22 cases. [1-5]. In children these tumors differ significantly in epidemiology, clinical char- acteristics, and biologic features compared to adults. The inclusive term of adrenocortical neoplasm has been ap- plied to these tumors, because in children adrenal carci- noma and adrenal adenoma are difficult to distinguish histopathologically. Radiological distinction between adrenocortical carcinoma and adenoma is also difficult to make without evidence of metastasis or vascular invasion.
Case report
Our patient is a 1,240-g baby boy born at 27 weeks and 4 days gestational age by cesarean section performed for possible placental abruption and breech presentation. He had an outside prenatal sonogram and the report suggested a right abdominal cystic mass, but the films were not available. On delivery, a smooth, firm, non- tender right upper abdominal mass was visible and palpable on examination. He had no other obvious anomaly. Postnatally, he received high-flow ventilation and surfactant. Renal and liver function test results were normal. A radiograph of the abdomen showed a right abdominal mass displacing the bowel inferiorly and medially without evidence of calcification. A sonogram
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revealed an 8x5x6-cm heterogeneous predominantly solid right adrenal mass with a cystic component dis- placing the right kidney inferiorly into the pelvis. The cystic area had septations suggestive of necrosis (Fig. 1). There was no calcification. The mass was well encap- sulated and was separate from the right kidney, liver, and anterior abdominal wall (Fig. 2). There was no evidence of tumor invasion of the inferior vena cava (IVC) or of the spinal cord. Doppler evaluation showed normal flow in the IVC and renal vessels (Fig. 3). The patient failed to thrive despite aggressive nutritional support and at 2 weeks of age underwent surgical exploration. A large, smooth, and well-encapsulated mass was found arising from the right adrenal gland.
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There was no evidence of invasion into the adjacent structures and the mass was completely excised.
Histopathological examination of the mass confirmed the diagnosis of an adrenocortical neoplasm. The high level of differentiation, low mitotic activity, and absence of vascular capsular invasion were more consistent with the diagnosis of an adrenocortical adenoma. Chromo- some analysis of the neoplasm showed near triploidy and loss of chromosomes 11 and 17, and these findings were also consistent with an adrenocortical neoplasm.
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| Clinical feature | Patient (n=22) |
|---|---|
| Virilization | 15 |
| Palpable mass | 9 |
| Metastasis | 7 |
| Cushing's syndrome | 3 |
| Arterial hypertension | 3 |
Postoperatively the patient required ongoing resus- citation but made an uneventful recovery. Follow-up sonograms performed at 3 and 11 months postopera- tively showed no tumor recurrence.
Discussion
Differential diagnosis of fetal adrenal mass includes adrenal hemorrhage, neuroblastoma, and adrenocorti- cal neoplasm. Subdiaphragmatic pulmonary seques- tration, mesenteric cyst, enteric duplication cyst, and neurogenic cyst can present as a suprarenal subdia- phragmatic mass. Renal lesions like congenital Wilms’ tumor, mesoblastic nephroma, and dysplastic change of a multicystic dysplastic kidney can at times be in- cluded in the differential diagnosis of a fetal suprarenal mass.
Neoplasms arising from the adrenal cortex are typi- cally divided into carcinoma and adenoma. They are grouped together as adrenocortical neoplasm. Pediatric adrenocortical neoplasm behaves differently from the adult type. In the pediatric population, they typically occur in children under the age of 15 years [1]. In older children they affect girls more than the boys with a ratio of 5.3:1. The incidence of left and right side involvement is equal [1]. These neoplasms have been described to be associated with hemihypertrophy, Beckwith-Wiedemann syndrome, and Li-Fraumeni syndrome. Increased risks of adrenocortical neoplasm have been found with pes- ticide exposure in children engaged in agriculture [1].
Clinical manifestations of adrenocortical neoplasm such as virilization, precocious puberty, and Cushing’s syndrome are attributed to steroid produced by the neoplasm (Table 1). In one published series, serum dehydroepiandrosterone (DHEA) was abnormal in up to 90% of patients. [1]. A large adrenocortical neoplasm may present as an abdominal mass but this finding is usually not the dominant manifestation in older children [1, 5]. Congenital adrenocortical neoplasms appear to have a much higher incidence of presenting as a palpable abdominal mass [1].
Out of the 22 published cases, 15 patients presented with virilizing symptoms and palpable abdominal mass
was present in nine patients [1-5] (Table 1). Three patients had Cushing’s syndrome and another three patients developed arterial hypertension. Seven patients had metastasis at the time of presentation. Ten pa- tients were girls, nine were boys, and the gender of three patients was not reported [1-5]. The left adrenal gland was involved in seven cases and the right was involved in 11 cases. Laterality was not reported in four cases. Eight infants died in the postoperative period and all of these cases occurred before 1976 [2]. Four patients died from medical complications before definitive therapy [1, 2]. Six patients had complete remission after surgery. Out of these six patients, one patient had skin and liver metastases and another patient had a lung metastasis that went into regression following surgery and chemotherapy. One patient with skin and brain metastases had spontaneous regression of metastases within 4 months of resection of the adrenocortical tumor without chemotherapy. At 1 year after the procedure he was reported to be disease-free [4].
In the radiographic evaluation of adrenocortical neoplasm, plain film radiography may show an abdominal mass or calcification [6]. Advanced bone age compared to chronological age is an additional finding. Sonographic features of a hyperechoic or hypoechoic mass with tumor necrosis have been de- scribed. Calcification is an inconsistent finding that is better demonstrated by CT [6]. None of these finding can distinguish adenoma from carcinoma. The pres- ence of a radiating echo seen at sonography has been associated with carcinoma. There is no significant difference in signal intensity characteristics between an adenoma and a carcinoma on MRI. While an adult adrenal adenoma can be differentiated from carcinoma because of the high lipid content in adenoma, this has not been studied in children. A metastasis suggests carcinoma and is usually found in the liver, lung, and regional lymph nodes [1].
Surgery is the mainstay of treatment of adrenocor- tical neoplasm [1, 2, 4, 5]. Large tumor size is a bad prognostic indicator [2, 5]. Presence of a metastasis at diagnosis or failure to completely resect the neoplasm is associated with an extremely poor outcome. Com- plete resection of an adenoma has been associated with an excellent outcome and can be considered curative [2, 3].
Our patient is now1 year of age and, thus far, is free from any sonographic evidence of residual or recurrent disease. As such, our patient is expected to have a good clinical outcome. However, given the rarity of the condition, close follow-up is warranted.
References
1. Satge D, Philippe E, Ruppe M, et al (1988) Neonatal carcinoma: review of the literature apropos of a case. Bull Cancer 75:373-384
2. Sandrini R, Ribeiro RC, DeLacerda L (1997) Childhood adrenocortical tumors. J Clin Endocrinol Metab 82:2027-2031
3. Butler H, Bick R, Morrison S (1988) Unsuspected adrenal mass in the neo- nate: adrenal cortical carcinoma and neuroblastoma. Pediatr Radiol 18:237- 239
4. Saracco S, Abramowsky C, Taylor S, et al (1988) Spontaneously regressing adrenocortical carcinoma in a newborn: a case report with DNA ploidy analysis. Cancer 62:507-511
5. Kakkar N, Vasishta RK, Lamba A, et al (2000) Pathological case of the month. Arch Pediatr Adolesc Med 154:1267- 1268
6. Daneman A, Chan HS, Martin J (1983) Adrenal carcinoma and adenoma in children: a review of 17 patients. Pediatr Radiol 13:11-18