Congenital pulmonary airway malformation (CPAM), previously known as congenital cystic adenomatoid malformation, is a rare developmental lung abnormality associated with rhabdomyosarcoma, pleuropulmonary blastoma, and mucinous adenocarcinoma of the lung. We report an unusual case of a 10-day-old male newborn with a left lower lobe pulmonary cyst who underwent lobectomy, which revealed type II CPAM complicated by multifocal mucinous adenocarcinoma. KRAS... |
Sex cord tumors with annular tubules are known to originate from the sex cord of embryonic gonads that synthesize Sertoli cells, Leydig cells, granulosa cells, and theca cells of the ovarian stroma, while ovarian small cell carcinoma of the hypercalcemic type is a type of neuroendocrine tumor. Both these tumors are uncommon, potentially malignant neoplasms in children. We report the... |
Renal cell carcinoma (RCC) arising from epithelial cells of the renal tubules is a highly aggressive and malignant tumor in all ages; however, it rarely occurs in children. the standard treatment for RCC is radical nephrectomy with lymph node dissection when the tumor is localized and can be completely resected. Adjuvant chemotherapy, radiotherapy, and immunotherapy are used for pediatric patients... |
Purpose : Alagille syndrome is an autosomal dominant disorder with developmental abnormalities affecting the liver, heart, eyes, vertebrae, and craniofacial region. The Jagged1(JAG1) gene, which encodes a ligand of Notch, has been found mutated in Alagille syndrome. The aim of the study was to investigate the mutation analysis of JAG1 gene in Korean patients with Alagille syndrome. Methods : Genomic DNA... |
Primary lung cancer is unusual in children; the squamous cell variant is extremely rare. Lung cancer is classified by histologic types into small-cell lung cancer, non-small cell lung caner, carcinoid, mucoepidermoid carcinoma, and adenoid cystic carcinoma. Furthermore, non-small cell lung cancer is subclassified into adenocarcinoma, large-cell carcinoma, and squamous cell carcinoma. The incidence of lung cancer is influenced by smoking,... |
Central diabetes insipidus is a rare disorder that can result as a consequence of diverse etiologies, including malformations, autoimmune, infiltrative(e.g. neoplastic or histiocytosis) or traumatic processes, as well as mutations in the gene encoding arginine vasopressin. Idiopathic central diabetes insipidus is a diagnosis of exclusion, one that has been made less frequently through the decades. Idiopathic central diabetes insipidus in... |
While the frequency of primary carcinoma of the lung has recently increased in adults, it is rare in the pediatric age group. The various adult types of bronchogenic carcinoma such as squamous cell, small cell, large cell, and adenocarcinoma, are extremely rare in children. Twenty to 30 cases of primary bronchogenic carcinoma of the lung in children under 15 years... |
Bronchial mucoepidermoid carcinoma is very rarely encountered in children. We report a case of bronchial mucoepidermoid carcinoma in a 10-year-old boy who presented with persistent cough and atelectasis. Bronchoscopic examination showed a tumor mass occluding the right bronchus intermedius, and the mass was removed by bronchoscopy. The results of the pathological examination revealed low-grade mucoepidermoid carcinoma. He underwent right middle... |
Thymic carcinoma is a malignant neoplasm arising from the thymic epithelium, and is known to be extremely uncommon in the pediatric population. The tumor is clinically highly aggressive with frequent local intrathoracic spread and extrathoracic metastases detected in the majority of patients at initial diagnosis and often shows minimal or no response to conventional chemotherapy. We experienced two pediatric patients... |
The appearance of mucoepidermoid carcinoma of the lung during in childhood is an uncommon neoplasm. Symptoms of progressive bronchial obstruction proceed the recognition of this lesion. Adequate surgical resection precludes the need for further radiation therapy or chemotherapy. The patient underwent bronchoscopy to investigate the cause of chronic coughing, which revealed a tumor. Histological examination confirmed the diagnosis of mucoepidermoid... |
Purpose : Surgical excision has been the primary treatment for hepatoblastoma and hepatocellular carcinoma. However, at presentation, only one third of such tumors are surgically resectable. Without operation, the disease is fatal. Therefore, neoadjuvant chemotherapy has been introduced for conversion of the unresectable tumors into the resectable ones. We studied th e effects of chemotherapy for hepatic malignancy in children. Methods... |
Gastric adenocarcinoma in childhood is an extremely rare disease with poor prognosis. We experienced a case in which a 14 year old female patient who was admitted with complaints of abdominal pain, indigestion and obstipation. Her physical examination revealed positive Blumer's rectal shelf, positive ascitic fluid cytology and typical radiologic and endoscopic findings suggesting gartric adenocarcinoma. She showed symptomatic improvement... |
Carcinomas in childhood differ from those in adults in their frequency, histologic type, and distribution of primary sites. In children, carcinomas are rare and few series have been published containing sufficient mumbers of cases to permit a reliable account of the incidence and survival of children with carcinoma. During a 10-year period, 1982-1991, we observed 40 patients less than 16... |
Authors experienced a case of primary hepatocellular carcinoma in a 7 year 9 month old male patient, who was admitted to our hospital due to abdominal pain on right upper quadrant and anorexia during last two months. On family history, his mother was HBs Ag and HBe Ag carrier. On past history, he was admitted to a hospital due to persistant jaundice at... |
Nasopharyngeal carcinoma is a primary malignancy of nasopharyngeal epithelium, firstly de- scribed as a separate entity by Regaud and Schmincke in 1921. Although it is a rare tumor, reprsent- ing less than 1% of pediatric malignancies, it accounts for 9% of the nasopharyngeal neoplasms of childhood, and has not been reported in Korean child. We experienced two cases of nasopharyngeal carcinoma in a 10... |
Adrenocortical carcinoma is a rare disease in childhood. Most of these carcinomas are functional, produc- ing endocrine abnormalities such as Cushing syndrome, virilization, precocious puberty, hyperaldosteronism or feminization. We experienced a case of adrenocortical carcinoma in a 12 month old male infant who exhibited features of Cushing syndrome, Virilization, precocious puberty and hyperaldosteronism, which were due to functional adrenocortical carcinoma. Microscopic examination revealed cellular pleomorphism... |
This is a case report of adrenal cortical carcinoma in a 2 year 7 month old male patient who was diagnosed by clinical features, endocrinologic studies, radiologic studies and pathologic examina- tions. Cushing syndrome and virilization caused by adrenal cortical carcinoma were found. Left total adrenalectomy was performed. No evidence of metastasis was found at the time of operation. 3 months later, lung metastases... |
In childhood, cancer of the ovary is highly unusual. It is responsible for only 1—2% of cancers found in patients under the age of 17. The frequency with which the various histologic types of ovarian neoplasm occur in childhood differs from that in adults. Epithelial tumors account for 70% to 80% of ovarian neoplasms in adults but only 20% in patients less than... |
We obtained following results by analysis of 12 cases of thyroid carcinoma in patient under 15 years of age who were admitted to Presbyterian Medical Center, Chon-ju from January 1975 to December 1984. 1) The ratio between male and female patients was 1 : 3. Patients over 12 years of age totalled 7 cases(58.3%). The youngest patient was 5 years and... |
Mucoepidermoid carcinoma of the bronchus in a 13-years-old boy was presented. The case was diagnosed by bronchoscopic examination and with the findings of light microscopy. There was no evidence of metastasis and tumor was completely removed by mid and lower lobectomy. Mucoepidermoid carcinoma is a very rare tumor especially in children arising from the bronchial gland. |
Malignant diseases of tte colon are rare during childhood. The etiology of disesaes has not not been clearly defined, but diet, exposure to farm chemical, carcinogens, bile acid, clostria, and genetic factors are associated with large bowel cancer. 50% of the adenocarcinoma in children are of the mucin producing variety. The prognosis of childhood cancer of the colon is very poor,... |
Primary hepatoma is relatively rare tumor in infants an children. The incidence among 1000 hospitalized children in pediatric clinic is about 0.05-0.06. The author experienced, a case of primary hepatoma occurred, in 3-months-old male who was admitted to the Pusan II Sin Women’s Hospital with symptom of progressively growing palpable mass on the right upper quadrant. Under the procedure of explorative laparatomy, the hard mass... |
Adrenal carcinoma is a rare disease both in adults and in children. Most of these tumors are functional, especially in children, producing endocrine syndromes such as Cushing syndrome, virilization, feminization or precocious puberty. The authors report a nineteen month old girl who exhibited features of Cushing syndrome and virilization caused by adrenal cortical carcinoma measuring 5.5X5.0X2.8 cm. She was successfully... |
We report a case of thyroid papillary adenocarcinoma in a 5 year 1 month old boy. His major problem was palpable thyroid nodule & cervical lymph nodes. Extensive bilateral pulmonary metastasis was noticed. The metastatic thyroid carcinoma was regressed after therapeutic doses of radioiodine without total thyroidectomy. |
Papaillary thyroid cancer is a rare lesion in children. It progresses slowly and its prognosis is relatively good. A painless nodule in the thyroid or in the neck is the usual first evidence of disease. Cervical lymph node involvement is usually present at the time of the initial diagnosis and is often bilateral. The lungs are the most common site... |
We experienced a Cushing’s syndrome which was caused by adrenocortical carcinoma in 8-year-old boy. He has suffered from obesity, moonface and growth retardation for 2 years. The diagnosis was confirmed by clinical symptoms, biochemical studies, X-rays and pathologic examinations. A total adrenalectomy was impossible because of far extended mass to adjacent organ, so only tissue biopay was done. After surgery, he received radiation... |
Carcinoma of the colon in childhood has remained extremely rare, and especially, primary carcinoma of colon was often escapes early diagnosis because of a lack of awarenss of its occurrence in pediatric age group. Recently we experienced a case of mucinous adenocarcinoma (Colloid carcinoma) of descending colon without predisposing conditions which may increased the risk of development of colorectal cancer in 13... |
A 8-yrs-old boy was admitted because of convulsion and coma. The diagnosis of Cushing's syndrome(key word) associated with hypertensive encephalopathy(key word) due to right adrenocortical carcinoma(key word) was made by clinical features, biochemical studies, radiological studies and pathological examination. The tumor was successfully resected by right adrenalectomy. But 22 months later after operation, he died of dyspnea and heart failure.... |