Adrenal venous malformation is an uncommon disease. We report a case of an incidentally diagnosed adrenal cystic mass during an evaluation for gastritis in a child. A 14-year-old girl admitted to our pediatric department for epigastric pain. After the gastroscopy, she was diagnosed with hemorrhagic gastritis. A 5.5 cm-sized cystic mass was incidentally found adjacent to her left adrenal gland during an ultrasound examination for evaluating her abdominal symptoms. She underwent laparoscopic surgery for the diagnosis and treatment of this cystic mass, which was confirmed to be venous malformation at pathologic diagnosis.
Recently, the incidence of perinatally detected asymptomatic adrenal gland masses has increased because of widespread use of radiological diagnostic tools. However, optimal treatment of these masses has not been determined. The aim of this study is to elucidate the treatment guideline of perinatally diagnosed adrenal gland masses. The authors retrospectively reviewed the medical records of the 11 patients with asymptomatic adrenal gland mass, detected perinatally, between 1999 and 2004. Six cases were detected by prenatal ultrasound and 5 cases were incidentally detected by postnatal ultrasound. Six patients (surgery group) underwent mass excision. The pathologic diagnoses were neuroblastoma (n=4), adrenocortical adenoma (n=1) and adrenal pseudocyst (n=1). The indications for operation were suspicion of neuroblastoma (n=5) or absence of size decrease during observation (n=1). Three of the 5 suspicious cases of neuroblastoma and one case under observation were proven to be neuroblastoma. There was no surgical complication in the urgery group. All neuroblastoma patients have been well during the follow up period (24.4±14.4 month) without evidence of recurrence. Five cases (observation group) were closely observed because of the benign possibility or size decrease in follow up ultrasound. During the observation period (39±21 week), 4 cases showed complete spontaneous resolution and 1 case showed markedly decreased size of the mass but could not be followed up completely. Surgical resection of the perinatally diagnosed asymptomatic adrenal gland mass is a safe treatment method especially in case of suspicion of neuroblastoma, but closed observation can be applied.
Beckwith-Wiedemann syndrome presents with multisystemic patterns of congenital anomalies and macrosomia. This syndrome was independently described by Beckwith in 1963 and by Wiedemann in 1964. There is wide spectrum of clinical manifestations, including prenatal or postnatal overgrowth, neonatal hypoglycemia, macroglossia, visceromegaly, omphalocele, hemihypertrophy and a predisposition for embryonal tumors, most frequently Wilms' tumor. We managed a case of Beckwith-Wiedemann syndrome with left adrenal cortical neoplasm of undetermined malignancy.
Adrenal cortical tumors are rare in adults and children. Most are malignant and functional. The principal clinical features are virilization, Cushing's syndrome, hyperaldosteronism and feminization. Recently, we treated a case of virilizing adrenal cortical tumor in a 26 month-old boy. The diagnosis was made by hormone assay, abdominal CT and tissue pathology. Right adrenalectomy was successful performed. Pathologic examination revealed an adrenal cortical adenoma with vascular invasion.
Neonatal adrenal hemorrhage is frequently associated with birth trauma or perinatal hypoxia. Hemorrhagic necrosis of the adrenal glands is often found at autopsy and many small lesions are usually asymptomatic. A palpable abdominal mass and jaundice are the usual presenting signs. Ultrasound is very useful in the diagnosis of this lesion; however, if the mass has mixed echoic pattern, magnetic resonance imaging (MRI) is helpful for the differential diagnosis from neuroblastoma. We present the case of a female newborn who was found to have a abdominal mass on physical examination. The patient showed anemia and hyperbilirubinemia. An ultrasonogram disclosed a 3.8 × 3.0 cm suprarenal mass with mixed echoic pattern. The mass was initially suspected to be neuroblastoma. An abdominal computed tomogram was not able to differentiate the mass. Magnetic resonance imaging revealed markedly increased signal intensity on T1 and T2-weighted sequences. This findLl1g was consistent with adrenal hemorrhage. Serial sonogram demonstrated the mass that resolved completely by 12 weeks of age.
Neuroblastoma is the most common solid malignancy in childhood. However, a neuroblastoma presenting as bilateral and multifocal is very rare. A male newborn weighing 2.7 kg was born by normal vaginal delivery at 39 weeks' gestation to a 27-year-old mother who had a normal pregnancy. He was in good condition at birth but presented palpable masses in the both upper abdomen and both side of the neck. Ultrasound examination showed the lesions to be 3 × 3cm sized calcified mass in right suprarenal area and 5 × 3cm sized homogenous mass in left suprarenal area. The abdominal mass was also examined by computed tomography with similar findings. With the impression of bilateral neuroblastoma or metastatic spread, the laparotomy was performed on the 13th day of life. Frozen section of biopsy of the left neck mass was obtained, which showed neuroblastoma. Intraoperative findings revealed bilateral adrenal masses which were distinct and anatomically separate. Bilateral adrenalectomy was accomplished and the initial postoperative course was unevenful. The patient was discharged for hopeless and expired at age 45 days. We present bilateral adrenal neuroblastoma considered to be simultaneous occurrence rather than metastases from one site to another.