Cases reported "Neuroblastoma"

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1/150. 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.
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2/150. A case of Down's syndrome associated with progressive extradural neuroblastoma.

    We describe a case of Down's syndrome associated with progressive extradural neuroblastoma. Postmortem aspiration of the bone marrow revealed diffuse infiltration by tumor cells, in which trisomy 21 was found by fluorescence in hybridization in situ.
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3/150. False-negative scintigraphy with Tc-99m sestamibi in stage IV neuroblastoma.

    Tc-99m sestamibi, originally developed for myocardial studies, has been used as a tumor-seeking agent. Recently, the agent also was reported to be a functional tracer to predict multidrug resistance-related p-glycoprotein expression in tumor tissue. The current report presents the authors' experience with sestamibi tumor scintigraphy in a neuroblastoma. Although I-131 MIBG tumor imaging and Tc-99m MDP bone scanning accurately demonstrated the extent of the disease, Tc-99m sestamibi showed no accumulation in primary and metastatic foci. Lack of sestamibi uptake was initially thought to be suggestive of failure to respond to chemotherapy because of p-glycoprotein expression. However, the patient responded well to chemotherapy and complete remission was achieved. The failure of Tc-99m sestamibi to detect a neuroblastoma and the lack of sestamibi accumulation in the tumor may not always be related to chemotherapy resistance.
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4/150. central nervous system relapse following bone marrow transplantation in stage IV neuroblastoma.

    neuroblastoma is the most common extracranial solid tumor in pediatrics. The disease-free survival rate for patients with stage IV neuroblastoma has improved over the past 10 years secondary to more aggressive induction chemotherapy regimens combined with autologous bone marrow transplantation. The usual sites of recurrence include the site of primary tumor, residual gross disease, bone, and bone narrow. The central nervous system, a rare site of relapse, is being involved with increasing frequency. The authors report two cases of patients with treated stage IV neuroblastoma who had relapses isolated to the CNS.
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5/150. Coexistence of several unbalanced translocations in a case of neuroblastoma: the contribution of multicolor spectral karyotyping.

    spectral karyotyping (SKY) is based on the simultaneous hybridization of a set of 24 chromosome-specific dna painting probes, each labeled with a different fluor combination. Automatic classification, based on the measurement of the spectrum for each chromosome, was applied to metaphases obtained from the affected bone marrow of a neuroblastoma case. spectral karyotyping allowed the identification of chromosomal aberrations that could not be identified by the use of the G-banding technique, and revealed a number of gains and unbalanced translocations.
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6/150. plasma exchange as successful treatment of thrombotic thrombocytopenic purpura post autologous bone marrow transplant in a child.

    We describe the case of a small child with stage IV neuroblastoma who developed thrombotic thromobocytopenic purpura (TTP) post autologous bone marrow transplant. Pneumococcal sepsis may have been the cause, a previously unreported association in transplant-associated TTP. Despite the child's size (10 kg) and the severity of the disease early intensive treatment with whole blood exchange and subsequently plasma exchange with cryosupernatant proved to be rapidly effective, in contrast to previous reports on its ineffectiveness in this setting.
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7/150. Angiomatoid (malignant) fibrous histiocytoma as a second tumour in a child with neuroblastoma.

    neuroblastoma occurring as a disseminated disease in children has a poor prognosis. Haematogenous metastases usually involve the marrow, bone, liver and skin. A second neoplasm may also develop. We describe a child with retroperitoneal neuroblastoma (stage 3) who developed a nodular mass in the inguinal area which was suspected to be a metastasis. Histopathology disclosed an angiomatoid (malignant) fibrous histiocytoma, and excision was curative. The occurrence of angiomatoid (malignant) fibrous histiocytoma as a second tumour in a patient with neuroblastoma has not previously been reported.
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8/150. incidence of occult cancer in children presenting with musculoskeletal symptoms: a 10-year survey in a pediatric rheumatology unit.

    OBJECTIVES: To assess the frequency and types of cancer found in children presenting to our Unit with musculoskeletal symptoms over a 10-year period. methods: The medical records of patients with musculoskeletal symptoms and a final diagnosis of cancer were reviewed. In each case age, gender, presenting symptoms, laboratory data, diagnostic procedures, provisional and final diagnoses, and time between clinical onset and correct diagnosis were reviewed. RESULTS: An underlying neoplasia was found in 10 of 1,254 patients (<1%) complaining of musculoskeletal symptoms. The types of malignancies found included acute lymphocytic leukemia (ALL) (6 cases), lymphoma (2 cases), neuroblastoma (1 case), and Ewing's sarcoma (1 case). The mean time between disease onset and final diagnosis was 3.2 months. The most common presenting feature was monoarthritis, involving the larger joints such as the elbows, knees or ankles. Juvenile idiopathic arthritis (JIA) was the most frequent provisional diagnosis. In the preliminary hematologic evaluation, eight patients had an increased erythrocyte sedimentation rate (ESR) or c-reactive protein (CRP) value. White blood cell (WBC) count was normal in almost all children, with a normal differential count. Lactic dehydrogenase (LDH) was raised in all children. Bone marrow aspirates and lymph node or bone biopsies were necessary to reach the final diagnosis. CONCLUSIONS: A malignancy should always be excluded in children with musculoskeletal symptoms, especially when the clinical pattern is not characteristic of a specific rheumatic disease. Routine laboratory tests may be misleading. The simultaneous presence of high LDH or alpha-hydroxybutyric dehydrogenase (alpha-HBDH) levels and raised ESR or CRP, even with normal blood cell counts, should lead to additional investigations. RELEVANCE: All patients presenting with arthritis or other musculoskeletal symptoms should have a thorough clinical examination. Disproportionate pain levels and an atypical pattern of "arthritis," especially in the presence of systemic manifestations, suggest a possible underlying malignancy.
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9/150. A case of infantile neuroblastoma with intramucosal metastasis in a paranasal sinus.

    An 8-year-old boy with neuroblastoma of the right adrenal gland is reported. His initial treatment included chemotherapy and surgery, with complete response (CR) being achieved at the initial site. A metastatic lesion was found in the right maxillary sinus 32 months after his initial treatment. A mass in the right soft palate was detected and was clinically suspected of being a metastasis. The results of biopsy were negative and the differential diagnosis from the imaging studies of CT included odontogenic disease, fungal infection, paranasal sinus cyst or hematoma, and benign tumors. Open transantral biopsy was done under general anesthesia, revealing severe inflammation in the right maxillary sinus as well as bone erosion. The histopathological diagnosis was metastatic neuroblastoma from the adrenal lesion. The local field was irradiated with 20 Gy of linear accelerator (linac) radiation, then the local field was eradicated. Extensive skeletal metastases were subsequently found by bone scintigraphy. Despite further treatment his general condition deteriorated rapidly and he died 24 months after starting treatment. We review the previous reports and discuss metastasis to the sinuses.
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10/150. Post-transplant lymphoproliferative disorder after autologous peripheral stem cell transplantation in a pediatric patient.

    Post-transplant lymphoproliferative disorder (PTLD) is a complication of allogeneic bone marrow transplantation (BMT). Rare cases of PTLD after autologous BMT have been reported only in adults. This case report is the first to describe PTLD in a pediatric patient after autologous peripheral stem cell transplantation (PSCT). This 2-year-old male with stage IV neuroblastoma underwent autologous PSCT. The post-PSCT course was complicated by fever with hematochezia and a lung mass. On day 94 post PSCT, colonoscopy revealed an ulcer due to a PTLD, monomorphic type, B cell phenotype, associated with Epstein-Barr virus. Fine needle aspiration identified the lung mass as neuroblastoma. PTLD can occur in pediatric autologous PSCT recipients, and may occur more frequently in autologous grafts manipulated by T cell depletion or CD34 cell selection.
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