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Case Report
Neurology
A rare case of dysembryoplastic neuroepithelial tumor combined with encephalocraniocutaneous lipomatosis and intractable seizures
Jee-Yeon Han, Mi-Sun Yum, Eun-Hee Kim, Seokho Hong, Tae-Sung Ko
Clin Exp Pediatr. 2016;59(Suppl 1):S139-S144.   Published online November 30, 2016

Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous syndrome that affects ectomesodermal tissues (skin, eyes, adipose tissue, and brain). The neurologic manifestations associated with ECCL are various including seizures. However, ECCL patients very rarely develop brain tumors that originate from the neuroepithelium. This is the first described case of ECCL in combination with dysembryoplastic neuroepithelial tumor (DNET) that presented with intractable...

Gastroenterology
Granular cell tumor of the esophagus in an adolescent
Ji Sun Lee, Kyung Ok Ko, Jae Woo Lim, Eun Jeong Cheon, Young Jin Kim, Jang Sin Son, Jung Min Yoon
Clin Exp Pediatr. 2016;59(Suppl 1):S88-S91.   Published online November 30, 2016

Esophageal granular cell tumor (GCT) is a rare neoplasm originating from the Schwann cells of the submucosal neuronal plexus. Histology is the gold standard for its diagnosis. Endoscopic resection or surgical excision should be considered, depending on the potential for malignancy. Here, we report a case of an esophageal GCT in an adolescent. A 12-year-old boy presented with a 1-year...

Review Article
Endocrinology
Effects of early menarche on physical and psychosocial health problems in adolescent girls and adult women
Jae-Ho Yoo
Clin Exp Pediatr. 2016;59(9):355-361.   Published online September 21, 2016

The menarcheal age of Korean women has been rapidly decreasing for the last 50 years, and the average menarcheal age of women born in the 1990s is approaching 12.6 years. In addition, interest in early puberty has been increasing recently owing to the rapid increase in precocious puberty. Generally, out of concern for short stature and early menarche, idiopathic central...

Case Report
Oncology
Early onset of colorectal cancer in a 13-year-old girl with Lynch syndrome
Do Hee Ahn, Jung Hee Rho, Hann Tchah, In-Sang Jeon
Clin Exp Pediatr. 2016;59(1):40-42.   Published online January 22, 2016

Lynch syndrome is the most common inherited colon cancer syndrome. Patients with Lynch syndrome develop a range of cancers including colorectal cancer (CRC) and carry a mutation on one of the mismatched repair (MMR) genes. Although CRC usually occurs after the fourth decade in patients with Lynch syndrome harboring a heterozygous MMR gene mutation, it can occur in children with...

Original Article
Prognostic factors in children with extracranial germ cell tumors treated with cisplatin-based chemotherapy
Jinsup Kim, Na Hee Lee, Soo Hyun Lee, Keon Hee Yoo, Ki Woong Sung, Hong Hoe Koo, Jeong-Meen Seo, Suk-Koo Lee
Clin Exp Pediatr. 2015;58(10):386-391.   Published online October 21, 2015
Purpose

To evaluate the outcomes and prognostic factors in children with extracranial germ cell tumors (GCTs) treated at a single institution.

Methods

Sixty-six children diagnosed with extracranial GCTs between 1996 and 2012 were included in the study. Primary treatment was surgical excision, followed by six cycles of cisplatin-based chemotherapy. The survival rates were compared according to the International Germ Cell Cancer Cooperative Group...

Case Report
DiGeorge syndrome who developed lymphoproliferative mediastinal mass
Kyu Yeun Kim, Ji Ae Hur, Ki Hwan Kim, Yoon Jin Cha, Mi Jung Lee, Dong Soo Kim
Clin Exp Pediatr. 2015;58(3):108-111.   Published online March 20, 2015

DiGeorge syndrome is an immunodeficient disease associated with abnormal development of 3rd and 4th pharyngeal pouches. As a hemizygous deletion of chromosome 22q11.2 occurs, various clinical phenotypes are shown with a broad spectrum. Conotruncal cardiac anomalies, hypoplastic thymus, and hypocalcemia are the classic triad of DiGeorge syndrome. As this syndrome is characterized by hypoplastic or aplastic thymus, there are missing...

Review Article
Growth hormone treatment and risk of malignancy
Hyun-Wook Chae, Duk-Hee Kim, Ho-Seong Kim
Clin Exp Pediatr. 2015;58(2):41-46.   Published online February 28, 2015

Growth hormone (GH) treatment has been increasingly widely used for children with GH deficiencies as the survival rate of pediatric patients with malignancies has increased. Both GH and insulin-like growth factor-I have mitogenic and antiapoptotic activity, prompting concern that GH treatment may be associated with tumor development. In this review, the authors examined the relationship between GH treatment and cancer...

Original Article
Diencephalic syndrome: a frequently neglected cause of failure to thrive in infants
Ahlee Kim, Jin Soo Moon, Hye Ran Yang, Ju Young Chang, Jae Sung Ko, Jeong Kee Seo
Clin Exp Pediatr. 2015;58(1):28-32.   Published online January 31, 2015
Purpose

Diencephalic syndrome is an uncommon cause of failure to thrive in early childhood that is associated with central nervous system neoplasms in the hypothalamic-optic chiasmatic region. It is characterized by complex signs and symptoms related to hypothalamic dysfunction; such nonspecific clinical features may delay diagnosis of the brain tumor. In this study, we analyzed a series of cases in order...

Review Article
Advancements in the treatment of pediatric acute leukemia and brain tumor - continuous efforts for 100% cure
Hee Young Ju, Che Ry Hong, Hee Young Shin
Clin Exp Pediatr. 2014;57(10):434-439.   Published online October 31, 2014

Treatment outcomes of pediatric cancers have improved greatly with the development of improved treatment protocols, new drugs, and better supportive measures, resulting in overall survival rates greater than 70%. Survival rates are highest in acute lymphoblastic leukemia, reaching more than 90%, owing to risk-based treatment through multicenter clinical trials and protocols developed to prevent central nervous system relapse and testicular...

Case Report
Gastrointestinal hemangioma in childhood: a rare cause of gastrointestinal bleeding
Eon Chul Han, Soo-Hong Kim, Hyun-Young Kim, Sung-Eun Jung, Kwi-Won Park
Clin Exp Pediatr. 2014;57(5):245-249.   Published online May 31, 2014

Gastrointestinal (GI) hemangiomas are relatively rare benign vascular tumors. The choice of an appropriate diagnostic method depends on patient age, anatomic location, and presenting symptoms. However, GI hemangiomas are not a common suspected cause of GI bleeding in children because of their rarity. Based on medical history, laboratory results, and imaging study findings, the patient could be treated with either...

A sclerosing stromal tumor of the ovary with masculinization in a premenarchal girl
Soo Min Park, Young Nam Kim, Young Jong Woo, Ho Sun Choi, Ji Shin Lee, Suk Hee Heo, Chan Jong Kim
Clin Exp Pediatr. 2011;54(5):224-227.   Published online May 31, 2011

A sclerosing stromal tumor of the ovary is an extremely rare benign tumor; it usually is found during the second and third decades of life. Patients present with pelvic pain or a palpable abdominal mass. Hormonal effects such as masculinization are uncommon. Here, an 11-year old premenarchal girl presented with deepening of the voice. In addition, clitoromegaly and hirsutism with...

Original Article
Selective embolization of the internal iliac arteries for the treatment of intractable hemorrhage in children with malignancies
Sul Hee Bae, Dong Kyun Han, Hee Jo Baek, Sun Ju Park, Nam Kyu Chang, Hoon Kook, Tai Ju Hwang
Clin Exp Pediatr. 2011;54(4):169-175.   Published online April 30, 2011
Purpose

Acute internal hemorrhage is an occasionally life-threatening complication in pediatric cancer patients. Many therapeutic approaches have been used to control bleeding with various degrees of success. In this study, we evaluated the efficacy of selective internal iliac artery embolization for controlling acute intractable bleeding in children with malignancies.

Methods

We retrospectively evaluated the cases of 6 children with various malignancies (acute lymphoblastic...

Case Report
A case of testicular adrenal rest tumor in a male child with congenital adrenal hyperplasia
Joo Hwa Kim, Kyong Ah Yun, Choong Ho Shin, Sei Won Yang
Clin Exp Pediatr. 2008;51(9):1018-1022.   Published online September 15, 2008
Testicular adrenal rest tumors are a well-known complication in male patients with congenital adrenal hyperplasia. Corticosteroid suppressive therapy usually results in the regression of these tumors. We describe a patient with 21-hydroxylase deficiency who developed bilateral testicular masses. Despite steroid suppressive therapy, the tumors did not regress and hormonal control was poor. Consequently, bilateral partial orchiectomies were performed.
Original Article
The Tumor Suppressor Function of PTEN/MMAC1 through the Regulation of IGFs and IGFBPs
Ho Keun Yi, Dong Jin Hwang, Sun Young Kim, Dae Yeol Lee, Pyoung Han Hwang
Clin Exp Pediatr. 2004;47(8):884-891.   Published online August 15, 2004
Purpose : PTEN/MMAC1, a novel tumor suppressor gene, is mutated in a variety of advanced and metastatic cancers. It acts as a phosphatase, and thereby, regulates the PI-3 kinase/Akt pathway. In this study, we examined to evaluate the new function of anti-tumor effects of PTEN/MMAC1 through the regulation of the IGFs-IGFBPs in gastric cancer cells. Methods : PTEN/MMAC1 was expressed in...
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