Perforation of choledochal cyst in a 12-year-old boy

Choledochal cyst is a rare congenital malformation that presents in children and young adults and is more frequent in women. Early diagnosis is essential to avoid possible complications. The method of choice for the initial diagnosis is ultrasound. Treatment consists of complete resection of the cyst. We describe a case of perforation of a choledochal cyst in a 12-year-old boy.     

Renal cell carcinoma originating in a renal cyst in a 12-year-old girl

We report a 12-year-old girl with renal cell carcinoma originating in a cyst of the left kidney. Ultrasonography revealed 2 small hyperechoic masses in the luminal side of a cyst. Although hypervascularity was not detected in the cyst by computed tomography, the possibility of malignancy could not be ruled out because of the presence of 2 solid masses. Therefore, partial left nephrectomy was performed. On histopathologic examination, the 2 solid masses within the cyst were found to be renal cell carcinoma. This patient remains disease-free at 4 years after partial nephrectomy.     

Long-term survival with carcinoma arising from a congenital choledochal cyst

We report a case of 11-year survival with carcinoma arising from a congenital choledochal cyst. A 34-year-old Japanese woman was admitted due to epigastric discomfort. Ultrasonography revealed a choledochal cyst involving a polypoid tumor. Endoscopic retrograde cholangiopancreatography revealed pancreaticobiliary maljunction in which the main pancreatic duct joined the dilated common bile duct 2 cm above the papilla of Vater. A choledochal cyst containing a polypoid tumor was resected with the regional lymph nodes. Grossly, a 2×2-cm papillary tumor was noted in the posterior wall of the choledochal cyst. Microscopically, the tumor was well-differentiated tubular adenocarcinoma invading the mucosa and fibromuscular layer. Neither anticancer chemotherapy nor radiotherapy was performed. The patient recovered uneventfully after the operation and has been well for 11 years without evidence of recurrent cancer. The good results in this patient may have been due to the early stage of the cancer.     

Biliary papillomatosis arising in a congenital choledochal cyst: report of a case

We report a rare case of biliary papillomatosis arising in a congenital choledochal cyst, with an anomalous junction of the pancreatobiliary ductal system (AJPBDS). A 50-year-old woman was admitted to our department with epigastralgia, and imaging studies showed two masses in the cystic common bile duct and an AJPBDS. We made a preoperative diagnosis of biliary tract cancer, considering the size of the masses and the presence of the AJPBDS, and performed a pylorus-preserving pancreatoduodenectomy. The resected specimen contained two papillary tumors, which were subsequently diagnosed as benign papillomas. Histopathological and oncological examinations indicated that the lesions were involved in the development and progression of carcinogenesis because a point mutation of the K-ras gene and overexpression of p53 protein were detected. These findings suggest that biliary papillomatosis is a precancerous lesion induced by an AJPBDS. Received: December 25, 2001 / Accepted: May 7, 2002 Reprint requests to: K. Kubota     

Urinary and gastrointestinal malakoplakia in a 12-year-old girl

Malakoplakia is a rare chronic inflammatory disease that was originally described in the urinary bladder but can involve many other organs and soft tissues. It is believed to be caused by an alteration in the bacterial phagocytic system. Clinically, it is described as single or multiple tumors that can appear in any part of the body. Histologically, the presence of Michaelis-Gutmann bodies is pathognomonic. Malakoplakia in children is rare. Few pediatric cases in the urinary tract, kidney, or gastrointestinal tract have been published. We present a case of urinary and gastrointestinal malakoplakia in a 12-year-old girl.     

Gastric adenomyoma mimicking gastric duplication cyst in a 5-year-old girl

Gastric adenomyoma in children is a rare benign tumor composed of a mixture of duct-like epithelial structures with smooth muscle bundles. It has been considered as a subtype of ectopic pancreas because the epithelial component resembles that in pancreatic ducts. However, it is now recognized as an independent pathologic lesion. Gastric adenomyoma is usually asymptomatic but could cause obstruction, inflammation, ulceration, and malignant transformation. Hence, to achieve diagnostic certainty and to control symptoms, complete operative resection is recommended. To the best of our knowledge, this is the first case of gastric adenomyoma mimicking a gastric duplication cyst in children.     

Squamous cell carcinoma arising in a dentigerous cyst in a 16-month-old girl.

Squamous cell carcinoma arising in a dentigerous cyst is a rare lesion with distinct histopathology and stringent criteria for diagnosis.(1,2) During the past century, <60 cases have been reported.(3) Of the previously documented cases, all have occurred in adults. We present the case of a squamous cell carcinoma arising in a dentigerous cyst in a 16-month-old girl. This case poses interesting questions regarding the pathophysiology of these tumors and highlights the importance of maintaining a high index of suspicion regardless of age when evaluating cystic lesions of the mandible and maxilla.     

Primary neurilemmoma of the thyroid gland in a 12-year-old girl

A case of a neurilemmoma of the thyroid gland in a 12-year-old girl is reported. The tumor was noted as a asymptomatic left lower neck swelling 9 months before admission. Ultrasound scan showed the solid lesion of predominantly hypoechoic structure measuring 17.2 × 12 × 26 mm within the left lobe. Pathologic examination of the excised lobe was consistent with neurilemmoma with both Antoni A and Antoni B histologic patterns. To date only 14 cases of this nonepithelial tumor of the thyroid gland have been described in the literature, and all occurred in adult patients. This is the first report of thyroid neurilemmoma in a child.