No abstract available.
Ganglioneuroblastoma*

Ganglioneuromas are benign tumors. Surgical excision is the treatment of choice with very good prognosis. However, neuroblastomatous malignant transformation of ganglioneuromas was previously reported. We report a patient with spinal neuroblastoma recurrent from a ganglioneuroblastoma after disease free survival of 13 years. This is one of the rare examples of spinal neuroblastoma and to our knowledge the second case report with malignant transformation from a ganglioneuroblastoma or a ganglioneuroma. The present case is the only report in the literature with further genetic investigations.
Disease-Free Survival ; Ganglioneuroblastoma* ; Ganglioneuroma ; Humans ; Neuroblastoma* ; Prognosis

A case of adrenal ganglioneuroblastoma is presented here. This adrenal ganglioneuroblastoma was found in a 27-year-old female 7 months after delivery. CT clarified that the tumor originated retroperitoneally and was large in size (11.4 cm x 9.4 cm). The tumor was surgically removed together with pancreatic body and tail, left kidney and spleen, and pathological diagnosis was adrenal ganglioneuroblastoma-intermixed. Adrenal ganglioneuroblastoma is extremely rare in adults, with only about 9 cases documented including this case.
Adrenal Glands ; Adult* ; Diagnosis ; Female ; Ganglioneuroblastoma* ; Humans ; Kidney ; Spleen ; Tail

Ganglioneuromas of retroperitoneal cavity which not originate from adrenal gland are very rare tumor, and usually recognized incidental to some other aliment or on a routine physical examination. Ganglioneuromas are generally considered to be the mature form of a triad of tumors arising from neural crest tissue which include malignant neuroblastoma and ganglioneuroblastoma. We report one case of retroperitoneal non-adrenal ganglioneuroma with review of literature.
Adrenal Glands ; Ganglioneuroblastoma ; Ganglioneuroma* ; Neural Crest ; Neuroblastoma ; Physical Examination

BACKGROUND: Neuroblastoma is the third most common malignancy of chidhood, and is the most common mediastinal mass in children under the age of 2 years. However, the results of surgical treatment have been seldomly reported in Korea. Therefore, we analyzed the results of surgical treatment in children with neuroblastoma and its influencing factors. MATERIAL AND METHOD: We studied the clinical characteristics and prognosis of 12 children, 11 makes and 1 female, whose primary thoracic neurobalstomas or ganglioneuroblastomas were diagnosed and operated between 1977 and 1997. Men age at presentation was 29.9 months. RESULT: Respiratory symptoms were the modes of performed in 9 patients. Complete excision, partial excision, and biopsy only were performed in 9, 2, and 1 patients respectively. Ten patients of thoractic neuroblastomas survived (83.3%) during follow-up period. CONCLUSIONS: The postoperatve 5-year survival of thoracic neuroblastoma was 76.4% and the prognosis was related to the stage of neuroblastoma. We suggest that complete resection should be considered as preferential method in the treatment of thoracic neuroblastoma in children, especially with early stage.
Biopsy ; Child* ; Female ; Follow-Up Studies ; Ganglioneuroblastoma ; Humans ; Korea ; Male ; Mediastinal Neoplasms ; Neuroblastoma* ; Prognosis

This paper presents a case of ganglioneuroblastoma in 3-year-old Korean boy which was arised from right adrenal gland. The tumors arising from the ganglion cells of the peripheral nervous system are ganglioneuroma, neurplastoma and ganglioneuroblastoma. We experienced a case of ganglioneuroblastoma recently which was confirmed histologically and excised successfully. Also we made a brief review of literatures.
Adrenal Glands ; Child, Preschool ; Ganglion Cysts ; Ganglioneuroblastoma* ; Ganglioneuroma ; Humans ; Male ; Peripheral Nervous System

Ganglioneuroblastoma ; complications ; Humans ; Infant ; Male ; Paraneoplastic Cerebellar Degeneration ; etiology ; Tomography, X-Ray Computed

The syndrome of watery diarrhea, hypokalemia, and achlorhydria (WDHA syndrome) is rare, and occasionally related with neurogenic tumors. A 20-month-old girl presenting symptoms of WDHA syndrome was transferred to our hospital. She had a ganglioneuroblastoma of adrenal gland. The serum level of VIP was elevated. After complete excision of tumor, all symptoms of WDHA syndrome were improved. The postoperative course was uneventful, and symptoms of WDHA syndrome disappeared. The serum level of VIP dropped to normal. The girl with postoperative chemotherapy and radiation therapy survived without evidence of disease for 33 months after complete resection.
Achlorhydria ; Adrenal Glands ; Diarrhea ; Drug Therapy ; Female ; Ganglioneuroblastoma* ; Humans ; Hypokalemia ; Infant ; Vipoma*

The presence and distribution of pan-neuroendocrine markers such as neuron-specific enolase (NSE), chromogranin (CG), and synaptophysin (SYP) were investigated by immunohistochemistry in 15 cases of neuroblastic tumors, including four cases of neuroblastomas, six cases of ganglioneuroblastomas, and five cases of ganglioneuromas. Three cases of normal sympathetic ganglion were used for the normal control group. NSE was observed in all cases and both in ganglion cells and in neuropils. NSE was detected not only in the majority of the neuroblasts showing signs of differentiation, but also in some poorly differentiated neuroblasts. All cases of neuroblastic tumors were positive for CG, however, some variability of staining intensity and distribution patterns were noted. CG was found mainly in differentiated neuroblasts with enlarged cytoplasm and nuclei along the periphery of the perikaria, and was also found in the perinuclear regions of some undifferentiated cells. SYP was positive in 9 of 11 cases. In all of the 9 cases, SYP was detected in some differentiating neuroblasts and differentiated neuroblasts, as well as the mature ganglion cells. However, it has scarcely stained in dot or granular pattern. Two CG-negative tumors were also negative for SYP. Our data indicate that antibodies against NSE and CG are helpful as a diagnostic aid for neuroblastic tumors.
Antibodies ; Cytoplasm ; Ganglia, Sympathetic ; Ganglion Cysts ; Ganglioneuroblastoma ; Ganglioneuroma ; Immunohistochemistry ; Neuroblastoma ; Neurons* ; Neuropil ; Phosphopyruvate Hydratase* ; Synaptophysin*

Crossed testicular ectopia is a rare anomaly, in which both testes migrate toward the same hemiscrotum. Fewer than 100 cases of this condition have been reported in the literature worldwide. Occasionally, conditions initially suspected to be crossed testicular ectopia have proven to be other anomalies, including hypospadias, renal agenesis, and seminal vesicle cysts. In this case, we diagnosed ganglioneuroblastoma in a patient with testicular ectopia.
Congenital Abnormalities ; Cryptorchidism ; Female ; Ganglioneuroblastoma ; Humans ; Hypospadias ; Kidney ; Kidney Diseases ; Male ; Seminal Vesicles ; Testis