Cranial Fossa, Posterior ; Cranial Nerve Neoplasms ; diagnosis ; pathology ; Ganglioneuroma ; diagnosis ; pathology ; Humans ; Male ; Middle Aged ; Skull Base Neoplasms ; diagnosis ; pathology ; Trigeminal Ganglion

Colonic diffuse ganglioneuromatosis is a benign neoplastic condition characterized by disseminated, intramural, or transmural proliferation of neural elements involving the enteric plexuses, sometimes associated with von Recklinghausen's disease and other multiple tumor syndromes. Colonic diffuse ganglioneuromatosis is usually large, ranging from 1 to 17 cm, and thus can distort the surrounding tissue architecture as well as infiltrate the adjacent bowel wall. However, colonic diffuse ganglioneuromatosis is an exceptional finding in adults and only individual cases are reported in the literature. Herein, we report two unusual cases of adult patients with colonic diffuse transmural ganglioneuromatosis presenting as a large subepithelial tumor.
Adult ; Aged ; Colon/metabolism/*pathology ; Colonoscopy ; Ganglioneuroma/*diagnosis/metabolism/pathology ; Humans ; Immunohistochemistry ; Male ; S100 Proteins/metabolism ; Tomography, X-Ray Computed

OBJECTIVETo study the clinicopathologic characteristics of peripheral neuroblastic tumors and to evaluate the prognostic significance of these features.

METHODSThe clinical and pathologic findings were retrospectively reviewed in 121 cases of peripheral neuroblastic tumor. The clinical outcomes of patients were evaluated. The three-year event-free survival rate was analyzed, with respect to age of patients, Evan's staging, International Neuroblastoma Pathology Classification and mitosis-karyorrhexis index.

RESULTSThe median age at diagnosis was 2.7 years; and 96 cases (79.3%) occurred in patients younger than 5 years old. The number of cases in Evan's staging I, II, III, IV and IVs was 24, 39, 24, 29 and 5, respectively. There were 82 cases of neuroblastoma (NB) (including 2 cases of undifferentiated NB, 52 cases of poorly differentiated NB and 28 cases of differentiating NB), 9 cases of ganglioneuroblastoma, intermixed type (GNBi), 19 cases of ganglioneuroma, maturing type (GN) and 11 cases of ganglioneuroblastoma, nodular type (GNBn). Forty-nine cases were in the favorable histology subgroup and 72 cases in the unfavorable histology subgroup. The overall three-year event-free survival rate of the 121 cases was 73.0% ± 4.3%. The three-year event-free survival rates were associated with age (P = 0.002), Evan's staging (P = 0.000), histologic category (P = 0.000), mitosis-karyorrhexis index (P = 0.043), prognostic subgroup (P = 0.000).

CONCLUSIONSMost of the peripheral neuroblastic tumors occur in the children younger than 5 years old. It is composed of NB, GNBi, GN and GNBn. The three-year event-free survival rate is approximately 70%. Significant prognostic parameters include age of patients, Evan's staging, International Neuroblastoma Pathology Classification and mitosis-karyorrhexis index.

Age Factors ; Antigens, Nuclear ; metabolism ; Child ; Child, Preschool ; Disease-Free Survival ; Female ; Ganglioneuroblastoma ; metabolism ; pathology ; surgery ; Ganglioneuroma ; metabolism ; pathology ; surgery ; Humans ; Infant ; Infant, Newborn ; Male ; Neoplasm Staging ; Nerve Tissue Proteins ; metabolism ; Nestin ; metabolism ; Neuroblastoma ; metabolism ; pathology ; surgery ; Peripheral Nervous System Neoplasms ; metabolism ; pathology ; surgery ; Phosphopyruvate Hydratase ; metabolism ; Retrospective Studies ; S100 Proteins ; metabolism

OBJECTIVETo investigate the molecular genetic abnormalities of N-myc and C-myc, and their clinical pathological implications in pediatric neuroblastic tumors (NTs).

METHODSAbnormalities of N-myc were detected by interphase fluorescence in situ hybridization (FISH) technique in 246 cases of NTs, including neuroblastoma (NB,188 cases), ganglioneuroblastoma (GNB, 52 cases), ganglioneuroma (GN, 6 cases), and their association with the histological typing of the tumors and prognosis was analyzed. Abnormalities of C-myc were detected by FISH in 133 cases of NTs.

RESULTSOf the 246 cases of NTs, N-myc amplification was only found in 27 cases (11.0%, 27/246) of NB, but not in any cases of GNB or GN (P < 0.05). 89.0% (219/246) N-myc non-amplification were found in NTs, and it included N-myc gain in 175 cases (71.1%, 175/246) and normal N-myc in 44 cases (17.9%, 44/246). Univariate analysis indicated significantly (P = 0.012) poorer outcome in patients with N-myc amplification than N-myc non-amplification. However no significant difference was observed between N-myc gain cases and normal N-myc cases (P = 0.057). C-myc gain was found in 74 of 133 cases (55.6%) of NTs; no C-myc amplification or translocation was detected. Forty percent (6/15) of cases with N-myc amplification and 57.6% (68/118) of cases with N-myc non-amplification were accompanied by C-myc gain. The difference between N-myc amplification and non-amplification with C-myc gain was not significant (P > 0.05). Univariate analysis indicated that the outcome difference was not statistically significant between C-myc gain cases and normal C-myc cases (P = 0.357).

CONCLUSIONSThe incidence of N-myc amplification only found in NB is low in pediatric NTs in China. Patients with N-myc amplification predict poorer outcome. No amplification or translocation of C-myc is detected in NTs, whereas C-myc gain is relatively common in NTs. There is no obvious association between N-myc amplification and C-myc gain.

Adrenal Gland Neoplasms ; genetics ; pathology ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Ganglioneuroblastoma ; genetics ; pathology ; Ganglioneuroma ; genetics ; pathology ; Gene Amplification ; Genes, myc ; Humans ; In Situ Hybridization, Fluorescence ; Infant ; Male ; Mediastinal Neoplasms ; genetics ; pathology ; Neuroblastoma ; genetics ; pathology ; Survival Rate

A case of intracranial ganglioneuroma arising from the trigeminal nerve in the pontine and cerebellopontine angle cistern, in a 44-year-old female, is presented with an emphasis on diffusion-weighted imaging findings. We will discuss on how the tumor in the very unusual location should be differentiated particularly focused on diffusion-weighted imaging findings.
Adult ; Contrast Media/diagnostic use ; Diagnosis, Differential ; Diffusion Magnetic Resonance Imaging/*methods ; Female ; Ganglioneuroma/*pathology/surgery ; Humans ; Trigeminal Nerve/*pathology/surgery

OBJECTIVETo investigate the basic clinical characteristics of paraneoplastic neurological syndrome (PNS) in children.

METHODTo retrospectively analyze the clinical data of 12 PNS children who were hospitalized in neurology department in Beijing Children's Hospital from 2010 to 2011. Some patients were followed up after surgery.

RESULTIn 12 patients with PNS, 11 were male and 1 was female. The mean onset age were (30.5 ± 15.3) months. The mean duration from neurological symptom onset to finding out of tumor was (112.7 ± 154.4) days. The onset of the disease in 2 patients was acute, in 3 was subacute and in the other 7 was chronic (2 of 7 had 2 to 3 relapses). Of 12 patients, 11 had symptoms of ataxia (3 patients also had opsoclonus and myoclonus, OMS), 1 had weakness of limbs at onset and then had ataxia. Nine of 12 patients had surgery, and pathologic diagnosis was neuroblastoma and ganglioneuroma. Six patients were followed-up for 8 to 21 months. One patient had a little improvement and 5 almost recovered.

CONCLUSIONThe PNS children can have neurological symptoms only at the onset and there were no particular evidence of tumor. It is prone to misdiagnosis. The prognosis of PNS in children was poor.

Adrenocorticotropic Hormone ; therapeutic use ; Biomarkers, Tumor ; analysis ; Brain ; diagnostic imaging ; pathology ; Child, Preschool ; Female ; Ganglioneuroma ; diagnosis ; pathology ; therapy ; Humans ; Immunoglobulins, Intravenous ; therapeutic use ; Infant ; Magnetic Resonance Imaging ; Male ; Neuroblastoma ; diagnosis ; pathology ; therapy ; Opsoclonus-Myoclonus Syndrome ; diagnosis ; pathology ; therapy ; Paraneoplastic Syndromes, Nervous System ; diagnosis ; pathology ; therapy ; Prognosis ; Radiography ; Retrospective Studies

Age Factors ; Child ; Child, Preschool ; Ganglioneuroblastoma ; genetics ; metabolism ; pathology ; ultrastructure ; Ganglioneuroma ; genetics ; metabolism ; pathology ; ultrastructure ; Gene Amplification ; Gene Expression Regulation, Neoplastic ; Humans ; Infant ; N-Myc Proto-Oncogene Protein ; Neoplasm Staging ; Neuroblastoma ; genetics ; metabolism ; pathology ; ultrastructure ; Nuclear Proteins ; genetics ; metabolism ; Oncogene Proteins ; genetics ; metabolism ; Peripheral Nervous System Neoplasms ; classification ; genetics ; metabolism ; pathology ; ultrastructure ; Prognosis ; Proto-Oncogene Proteins c-myc ; metabolism ; Receptor, trkA ; metabolism ; S100 Proteins ; metabolism

Chromogranin A ; metabolism ; Diagnosis, Differential ; Duodenal Neoplasms ; metabolism ; pathology ; surgery ; Ganglioneuroma ; metabolism ; pathology ; Gastrointestinal Stromal Tumors ; metabolism ; pathology ; Humans ; Male ; Middle Aged ; Neurofibroma ; metabolism ; pathology ; Paraganglioma ; metabolism ; pathology ; surgery ; Phosphopyruvate Hydratase ; metabolism ; S100 Proteins ; metabolism

OBJECTIVE: To evaluate clinical feature, diagnosis, treatment and prognosis of ganglioneuroma in the neck. METHOD: The medical records of 6 patients with cervical ganglioneuroma which were confirmed by pathology between 1995 and 2009 were retrospectively reviewed. RESULT: Patients with ganglioneuroma in the neck typically present with an asymptomatic neck mass. Neither imaging procedures nor fine needle aspiration made a definite diagnosis before surgery. All cases were operated, and developed Horner syndrome. With a median follow-up time of 5.9 years, all cases survived without local recurrence or distant metastasis. CONCLUSION Ganglioneuroma in the neck is a rare well differentiated benign tumour. Definite diagnosis only can be made after pathology. Complete surgical excision is the treatment of choice, as it will ensure thorough sampling of the tumour and cure. Postoperative prognosis is favorable if total resection.
Biopsy, Fine-Needle ; Ganglioneuroma ; pathology ; surgery ; Head and Neck Neoplasms ; pathology ; surgery ; Horner Syndrome ; etiology ; Humans ; Neoplasm Recurrence, Local ; Postoperative Complications ; Prognosis ; Retrospective Studies

OBJECTIVETo study the clinicopathologic characteristics of peripheral neuroblastic tumors and to investigate the prognostic significance of International Neuroblastoma Pathology Classification (INPC).

METHODSOne hundred and thirty-five cases of peripheral neuroblastic tumors encountered in Shanghai Children's Medical Center were enrolled into the study. All the cases were classified according to INPC and International Neuroblastoma Staging System (INSS). The follow-up data were analyzed.

RESULTSThe consensus diagnoses of the 135 cases were as follows: 80 cases (59.2%) of neuroblastoma, 24 cases (17.8%) of ganglioneuroblastoma, intermixed, 17 cases (12.6%) of ganglioneuroma and 14 cases (10.4%) of ganglioneuroblastoma, nodular. The cases were subdivided into 2 subgroups: favorable histology (number = 90, 66.7%) and unfavorable histology (number = 45, 33.3%). According to INSS, the number of cases in stages I, II, III and IV was 22 (16.3%), 24 (17.8%), 34 (25.2%) and 55 (40.7%), respectively. The survival of peripheral neuroblastic tumors correlated with histologic diagnosis, INPC and INSS (P < 0.05).

CONCLUSIONDiagnostic categorization of peripheral neuroblastic tumors according to INPC is of prognostic value.

Adolescent ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Ganglioneuroblastoma ; pathology ; surgery ; Ganglioneuroma ; pathology ; surgery ; Humans ; Infant ; Infant, Newborn ; Male ; Neoplasm Staging ; Neuroblastoma ; classification ; pathology ; surgery ; Peripheral Nervous System Neoplasms ; classification ; pathology ; surgery ; Retrospective Studies ; Survival Rate