1/108. Metastatic neuroblastoma with ganglioneuromatous differentiation and mandibular involvement.Neuroblastic tumors comprise neuroblastoma (NB), ganglioneuroblastoma, and ganglioneuroma (GN). NBs have shown remarkable differentiation capabilities, which include spontaneous regression. In the last 3 decades, the prognosis for metastatic NB has improved significantly. The ability to distinguish prognostic subtypes based on clinical and biologic features allows for understanding of this disease process and development of management procedures. NBs are often asymptomatic and remain undetected until a large abdominal mass or metastasis is found. GN, the most differentiated variant, is a benign neoplasm with significant growth potential and ability to cause clinical complications. GN of the mandible has seldom been reported in the literature in English. We present a case of NB metastatic to the mandible exhibiting ganglioneuromatous differentiation. We discuss the current understanding of the biology, grading, classification, and prognostic implications of NB.- - - - - - - - - - ranking = 1keywords = neuroblastoma (Clic here for more details about this article) |
2/108. Late-onset central hypoventilation with hypothalamic dysfunction: a distinct clinical syndrome.Idiopathic central hypoventilation has occasionally been reported in previously well children after infancy. The relationship between this late-onset central hypoventilation syndrome (LO-CHS) and congenital central hypoventilation syndrome (CCHS) has not been established. Both CCHS and LO-CHS have been associated with neural crest tumors, such as ganglioneuroblastoma and ganglioneuroma, and they generally occur in the presence of a histologically normal central nervous system. At least 10 case reports of idiopathic LO-CHS featured evidence of hypothalamic dysfunction (HD), including hyperphagia, hypersomnolence, thermal dysregulation, emotional lability, and endocrinopathies. We report on a case of LO-CHS/HD successfully treated by nasal intermittent positive pressure ventilation (NIPPV). Despite the commonalties with CCHS, we propose that LO-CHS/HD is a distinct clinical syndrome. In addition to the markedly different age at presentation, features of hypothalamic dysfunction are not seen in CCHS. review of the literature was undertaken to further clarify the full spectrum of the disease.- - - - - - - - - - ranking = 0.16666666666667keywords = neuroblastoma (Clic here for more details about this article) |
3/108. Adrenal ganglioneuroma: a familial case.A 67-year-old woman was evaluated for asymptomatic microscopic hematuria. Intravenous urography and computed tomography demonstrated an adrenal mass, but the workup for a functional adrenal tumor was negative. The mass was surgically resected, with a histologic diagnosis of adrenal ganglioneuroma. No further treatment was necessary. After the diagnosis was made, the patient reported a family history positive for neuroblastic tumors. Two of her grandchildren had presented at early ages with a ganglioneuroblastoma and a third had presented with a ganglioneuroma. This presentation is unique because ganglioneuroma, especially that of adrenal origin, is rare in the adult population, and familial cases are extremely uncommon.- - - - - - - - - - ranking = 0.16666666666667keywords = neuroblastoma (Clic here for more details about this article) |
4/108. ganglioneuroblastoma associated with malignant mesenchymoma.A case of ganglioneuroblastoma associated with malignant mesenchymoma is reported. The tumor originated from the retroperitoneum and was comprised not only of neuroblastoma and ganglioneuroma, but also of typical rhabdomyosarcoma, liposarcoma, undifferentiated mesenchymoma, as well as cartilaginous tissue. No distinct border existed between thses different components. The histogenesis of the tumor is discussed. We suggest that the tumor derived from a remnant of neural crest (ectomesenchyme).- - - - - - - - - - ranking = 1keywords = neuroblastoma (Clic here for more details about this article) |
5/108. Olfactory neuroblastoma with epithelial and endocrine differentiation transformed into ganglioneuroma after chemoradiotherapy.We report a 56-year-old man in whom an olfactory neuroblastoma with epithelial and endocrine differentiation transformed into a mature ganglioneuroma after chemoradiotherapy. The tumor arising from the sphenoidal and maxillary sinuses showed rapid growth into the frontal lobe and metastasis to the cervical lymph nodes. The patient showed signs of a syndrome of inappropriate secretion of antidiuretic hormone (SIADH). A radical craniofacial resection of the primary tumor was performed after 16 Gy of local irradiation and systemic chemotherapy. Three months after the operation, the patient died of mediastinal metastasis. The biopsy before chemoradiotherapy showed a neuroblastoma with Homer-Wright rosettes, fibrillary matrix, Flexner-Wintersteiner rosettes and antidiuretic hormone production. After chemoradiotherapy, the histology changed to that of a ganglioneuroma consisting of large ganglion cells and schwann cells without immature neuroblastoma components. Although transformation to ganglioneuroma in an adrenal neuroblastoma is common, an olfactory neuroblastoma showing ganglioneuronal maturation after chemoradiotherapy has not been reported. The pluripotent progenitor cells of the olfactory neurons may be the origin and their existence explains why various neoplasms with neuronal and epithelial differentiation arise from the olfactory mucosa.- - - - - - - - - - ranking = 1.5keywords = neuroblastoma (Clic here for more details about this article) |
6/108. Mediastinal neuroblastoma and ganglioneuroma. The differentiation between primary and secondary involvement on the chest roentgenogram.The roentgenologic diagnosis and differentiation of mediastinal neurogenic tumors are possible on the chest roentgenogram as a rule. The soft tissue mass may be ill-defined and the tumor "ghost-like" in the case of primary neuroblastoma, but it is usually obvious in ganglioneuroma and metastatic disease. The presence of calcifications differentiates neurogenic tumors from other posterior mediastinal tumors of childhood. They are common in primary and rare in secondary disease. Rib erosions and displacement are striking in neuroblastoma (after a few months of age), more subtle in ganglioneuroma, and absent with secondary involvement. In 3 out of 7 posterior mediastinal neuroblastomas the diagnosis and treatment were delayed, as the adjacent rib changes were not appreciated for some time. "Dumbbell" shaped tumors are usually associated with vertebral changes and myelography is indicated even in the absence of neurologic deficit. Thoracic deformity and disability subsequent to laminectomy, radiation therapy, or both, are present in all survivors.- - - - - - - - - - ranking = 1.1666666666667keywords = neuroblastoma (Clic here for more details about this article) |
7/108. Central pancreatectomy for a pancreatic ganglioneuroma in a patient with previous neuroblastoma.This is a case presentation of a 28-year-old woman with a mass at the junction of the neck and the body of the pancreas and a childhood history of a resected adrenal neuroblastoma. A central pancreatectomy was performed in this patient. The pathology revealed a benign ganglioneuroma. Ganglioneuromas and their association with a neuroblastoma are briefly reviewed. The indications and contraindications for, as well as the advantages and disadvantages of, a central pancreatic resection are discussed.- - - - - - - - - - ranking = 1keywords = neuroblastoma (Clic here for more details about this article) |
8/108. ganglioneuroblastoma in adults.ganglioneuroblastoma is a rare neoplasm of adults. Some information about the natural history and response to therapy can be obtained from two cases seen recently at our hospital and 17 cases in the literature. These tumors occur in adults of any age and are usually located in the retroperitoneum, mediastinum, or neck. Evidence of the tumor may be present years before diagnosis. Elevations of urinary catecholamines were documented in three cases and the levels correlated with progression or resection of the neoplasm. The tumor spreads either by local invasion or distant metastasis. Attempts at radiotherapy and chemotherapy in the treatment of unresectable or disseminated tumor has not resulted in objective improvement. This contrasts with the widely reported objective successes and cures of ganglioneuroblastomas occurring in childhood. Surgical excision of localized disease was the only curative therapeutic modality in the cases we reviewed.- - - - - - - - - - ranking = 1keywords = neuroblastoma (Clic here for more details about this article) |
9/108. ganglioneuroma presenting as an asymptomatic huge posterior mediastinal and retroperitoneal tumor.ganglioneuroma is a rare, differentiated, benign and slow-growing tumor that commonly arises from sympathetic ganglion cells. Most of them are asymptomatic and found incidentally. We here report a quite rare case of silent huge ganglioneuroma growing in both posterior mediastinum and retroperitoneum occurring in a 3.5-year-old girl. The patient was relatively well before and incidentally found to have a huge chest mass by chest x-ray film at an episode of respiratory tract infection. Computed tomography showed a huge tumor extending from bilateral posterior mediastinum to the level of the adrenal gland in the retroperitoneum. Initially, neuroblastoma was highly suspected and 24-hour urine vanillyl mandelic acid was slightly elevated. Cytology by bone marrow aspiration revealed no tumor nests or clumps. biopsy and pathology proved it as ganglioneuroma (GN). Due to too extensive involvement of the tumor and compression of the vital vessels, surgical removal became difficult. The family of the patient refused surgery due to there being no significant symptoms. Because of the potential for growth of unresectable GN and because the component of neuroblasts could not be completely excluded, the patient was still in dangerous status. The only thing we can do is to keep the family alert and continue regular follow-up.- - - - - - - - - - ranking = 0.16666666666667keywords = neuroblastoma (Clic here for more details about this article) |
10/108. ganglioneuroblastoma with disseminated bone marrow infiltration in an adult.ganglioneuroblastoma is an extremely rare neoplasm of adults. We present a case of adrenal ganglioneuroblastoma in a 47-year-old female with disseminated bone marrow involvement. bone marrow involvement was the major manifestation which determined the mortality of this patient. This case suggests that the diagnosis of bone marrow involvement should be carefully considered even in adult-onset ganglioneuroblastoma.- - - - - - - - - - ranking = 1.1666666666667keywords = neuroblastoma (Clic here for more details about this article) |
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