1/66. neuroblastoma metastatic to the liver in infants.Four infants are described who presented with rapid enlargement of the liver. This was found to be due to neuroblastoma which had metastasized to the liver; the condition was associated with high levels of urinary vanillylmandelic acid (VMA). In 3 infants the primary tumour was in the adrenal gland and in one it was not identified. One infant died after laparotomy and 2 infants survive disease free with normal VMA levels, one after adrenalectomy and hepatic irradiation and one after a short course of chemotherapy. The fourth patient responded initially to hepatic irradiation and chemotherapy, but relapsed 2 years later with recurrent disease; at the same time the VMA level which had been normal, again rose. The importance of screening for an abnormal VMA level in any infant with a rapidly enlarging liver in order to obtain an early diagnosis is stressed. Careful follow-up, with serial VMA estimations, is essential to detect recurrent disease. The prognosis for some infants with this distribution of neuroblastoma which has metastasized to the liver, but not to the bones or oribt, is good.- - - - - - - - - - ranking = 1keywords = neuroblastoma (Clic here for more details about this article) |
2/66. Spontaneous regression of neuroblastoma.Case of spontaneous regression of neuroblastoma continue to occur in the present multimodal therapy era at institutions where physicians are prepared to withhold treatment on certain patients with residual primary or metastatic disease. From a survey of the 22 member institutions of Children's Cancer Study Group, seven hospitals submitted data on 24 neuroblastoma patients whose disease underwent regression after minimal, unusual, or no treatment. An analysis of these patients and of 33 patients form two large series in the literature shows that the majority of patients are infants with Stage II or Stage IVS disease. The spontaneous regression usually consists of complete disappearance of the disease, but in some neuroblastomas, maturation to ganglioneuroma takes place. The various factors that may influence regression are discussed.- - - - - - - - - - ranking = 3.5keywords = neuroblastoma (Clic here for more details about this article) |
3/66. jaw and pulpal metastasis of an adrenal neuroblastoma.Hematogenous spread of malignant tumors to the dental pulp is very rare. A case of adrenal neuroblastoma in a 71/2-year-old boy which metastasized to the mandible and dental pulp is described. Tumor cells were found within the pulpal blood vessels of a deciduous molar tooth.- - - - - - - - - - ranking = 2.5keywords = neuroblastoma (Clic here for more details about this article) |
4/66. Metastatic neuroblastoma in the mandible. Report of a case.The case reported here is that of a metastatic neuroblastoma of the mandible in a 5-year-old boy. This kind of metastasis in the mandible is very rare. Osteolytic jaw defects and unexplainable looseness of the deciduous molars in children should induce a thorough examination with the possibility of a metastatic malignoma in mind. A summary of the literature and prognosis of these tumors has been given.- - - - - - - - - - ranking = 2.5keywords = neuroblastoma (Clic here for more details about this article) |
5/66. Secondary leukemia in a child with neuroblastoma while on oral etoposide: what is the cause?To date little has been reported about the risk of therapy-related leukaemia (t-AML) in children receiving oral etoposide therapy. The authors present a case of t-AML that developed in a child with metastatic neuroblastoma 18 months after he received oral etoposide, given for palliation purpose. The leukemic blasts were examined by morphological, immunohistochemical, cytogenetic, and molecular genetic analyses. Although the t-AML developed following oral etoposide therapy, the child had previously received high-dose, multiagent chemotherapy, and rearrangement of the MLL gene was not demonstrated. The use of modern multiagent therapy often makes it difficult to appropriately apportion blame for causation of specific side effects. Moreover, the etiology of t-AML and mechanism of leukemogenesis are likely to be multifactorial and complex. Further studies on the precise association with different therapies are thus needed. Oral etoposide remains an effective palliative agent and its usage should not be excluded without most careful consideration of the risks.- - - - - - - - - - ranking = 2.5keywords = neuroblastoma (Clic here for more details about this article) |
6/66. Radiographic features of olfactory neuroblastoma.Olfactory neuroblastomas are malignant, slowly growing neurogenic tumors originating from the olfactory mucosa of the nasal cavity. Fourteen patients with this tumor were evaluated and showed variable extension into adjacent structures such as the ethmoid and sphenoid sinuses, orbit, and anterior cranial cavity.- - - - - - - - - - ranking = 2.5keywords = neuroblastoma (Clic here for more details about this article) |
7/66. Primary sphenoid and petrous apex esthesioneuroblastoma: case report.A 62-year-old woman presented with raised intracranial pressure and features of a right cerebellopontine angle tumour with extension into the right middle cranial fossa. The patient died before a surgical excision could be performed. The autopsy revealed a primary esthesioneuroblastoma of the sphenoid sinus eroding the petrous bone and extending into the middle cranial fossa with metastatic tumour in the liver, and paratracheal and hilar lymph nodes. Although rare, esthesioneuroblastoma must be considered in the differential diagnosis of petrous-sphenoid lesions.- - - - - - - - - - ranking = 3keywords = neuroblastoma (Clic here for more details about this article) |
8/66. Disaloganglioside GD2 loss following monoclonal antibody therapy is rare in neuroblastoma.BACKGROUND: Gangliosicle GD2 is abundant on human neuroblastoma (NB). Monoclonal antibody 3F8 targeted to GD2 may have imaging and therapeutic potential. Antigen-negative clones can escape immune-mediated attack leading to clinical resistance or recurrence. PROCEDURE: Among 95 evaluable patients treated intravenously with 3F8 (94 Stage 4, 1 Stage 3), 66 received nonradiolabeled 3F8, 11 received 131-iodine-labeled-3F8 (8-28 mCi/kg) with autologous bone marrow rescue, and 18 received both forms of treatment. Prior to treatment, 90 patients tested positive for GD2 reactivity by bone marrow immunofluorescence (n = 68), tumor immunohistochemistry (n = 20), or diagnostic radioimmunoscintigraphy (n = 2). RESULTS: Of 62 patients who had refractory or recurrent neuroblastoma following 3F8 treatment, 61 (98%) tested positive for GD2 reactivity by bone marrow immunofluorescence (n = 51) or tumor immunohistochemistry (n = 10). The sole tumor that lost GD2 expression underwent phenotypic transformation into a pheochromocytoma-like tumor. CONCLUSIONS: The persistence of GD2 expression in refractory or recurrent NB suggests that complete antigen loss is an uncommon event and cannot account for treatment failure.- - - - - - - - - - ranking = 3keywords = neuroblastoma (Clic here for more details about this article) |
9/66. neuroblastoma in monozygotic twins--a case of probable twin-to-twin metastasis.Concordance for neuroblastoma in monozygotic twins has been reported only rarely, and the cause of the shared pathology has not been established. We describe a case of infant monozygotic twins developing tumours that were morphologically, clinically and molecularly indistinguishable, but with a delay of 6 months between times of presentation. Both tumours were metastatic and had amplification of MYCN and deletion at 1p36. Twin 1, who developed neuroblastoma first, had constitutional karyotype abnormalities in at least 5% of peripheral blood mononuclear cells involving 1p and 3p, and a deletion of 1q44 in 21% of cells. Twin 2 had a normal constitutional karyotype and lacked rearrangement or deletion of these regions. We propose an acquired neuroblastoma predisposition specific for twin 1, and in utero metastatic spread of tumour cells to twin 2 via the shared placental circulation.- - - - - - - - - - ranking = 1.5keywords = neuroblastoma (Clic here for more details about this article) |
10/66. Can the growth of a neuroblastoma be influenced by a child's nutritional state? Observations in a patient treated for kwashiorkor and later given a restricted diet.A 17-month-old Thai female with neuroblastoma presented with an abdominal mass and the classical findings of kwashiorkor. Concomitant with effective repair of the child's protein deficit, the mass enlarged dramatically and metastases were noted. This is the first known report of such an occurrence.- - - - - - - - - - ranking = 2.5keywords = neuroblastoma (Clic here for more details about this article) |
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