1/61. 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 = ganglioneuroblastoma (Clic here for more details about this article) |
2/61. 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 = 1keywords = ganglioneuroblastoma (Clic here for more details about this article) |
3/61. 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 = 1keywords = ganglioneuroblastoma (Clic here for more details about this article) |
4/61. 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 = ganglioneuroblastoma (Clic here for more details about this article) |
5/61. 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 = ganglioneuroblastoma (Clic here for more details about this article) |
6/61. 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 = 2keywords = ganglioneuroblastoma (Clic here for more details about this article) |
7/61. Intestinal ganglioneuroblastoma in a 22-week fetus.The clinical history and autopsy findings of a 22-week fetus with intestinal ganglioneuroblastoma, cardiac anomalies, omphalocele, and ileal atresia are presented. ganglioneuroblastoma was confined to the large intestine and was not suspected prenatally despite ultrasonographic examination. Although enteric ganglia share neural crest lineage with other sites of congenital neuroblastoma, this is the first report of a primary intestinal ganglioneuroblastoma in a fetus or child. Various hypotheses are discussed to explain the coexistence of cardiac malformation and congenital neuroblastoma in this fetus and other cases in the literature. It is hypothesized that other malformations evident in this fetus were caused by the tumor, possibly as teratogenic effects of neuroblastoma-derived catecholamines during embryogenesis.- - - - - - - - - - ranking = 6keywords = ganglioneuroblastoma (Clic here for more details about this article) |
8/61. Bilateral testicular neuroblastoma. Scintigraphic depiction and therapy with I-131 MIBG.The authors describe a 29-year-old man who had a 16-year history of neuroblastoma and uncommon manifestations. At age 13, he was diagnosed with stage III retroperitoneal ganglioneuroblastoma that was resected. Ten years later, bilateral testicular enlargement and a pelvic mass from infiltration of the neuroblastoma became palpable. Metastatic involvement was depicted with MIBG, a radiotracer that concentrates in tissues of the sympathetic nervous system. Using I-131 MIBG, the tumors were treated with therapeutic doses of radiation and a partial response was obtained. This case is unique because of the massive degree of bilateral testicular infiltration and its occurrence as a late manifestation of neuroblastoma in early adulthood.- - - - - - - - - - ranking = 1keywords = ganglioneuroblastoma (Clic here for more details about this article) |
9/61. Posterior mediastinal masses in children: ganglioneuroblastoma.ganglioneuroblastoma, although a rare neoplasm, is one of the most common causes of posterior mediastinal masses in children. This tumor occurs in children from one to 10 years of age. With intrathoracic involvement, the tumor may remain asymptomatic until it is large. The case reported illustrates a typical presentation of intrathoracic ganglioneuroblastoma.- - - - - - - - - - ranking = 5keywords = ganglioneuroblastoma (Clic here for more details about this article) |
10/61. Retroperitoneal ganglioneuroma encasing the celiac and superior mesenteric arteries.ganglioneuroma is a rare neoplasm arising from the sympathoadrenal neuroendocrine system and has anatomic distribution paralleling the sympathetic chain ganglia and the adrenal medulla. In some cases, ganglioneuroma is the end stage maturation of less-differentiated neoplasms such as neuroblastoma or ganglioneuroblastoma, but based on age at diagnosis (over 10 years of age) and anatomic location, many of these tumors appear to arise de novo. It must be included in the differential diagnosis of posterior mediastinal and retroperitoneal mass. We report a case of retroperitoneal ganglioneuroma involving the celiac axis and superior mesenteric arteries in a 40-year-old female.- - - - - - - - - - ranking = 1keywords = ganglioneuroblastoma (Clic here for more details about this article) |
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