Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In summary, carcinoma is the most frequent cancer that metastasizes to the skin; lung cancer in men and breast cancer in women. Clinically distinctive patterns of cutaneous metastasis of epithelial origin include alopecia neoplastica, pulsatile nodules, Sister Mary Joseph's nodules, morpheaform, and cellulitis-like lesions. Biopsying these lesions reveals adenocarcinoma, squamous cell carcinoma, or anaplastic carcinoma. The type of histologic pattern seen can be a clue to the organ of origin giving rise to the cutaneous metastasis. Skin that is damaged allows for circulating malignant cells, often of epithelial or leukemic origin, to lodge and proliferate locally (inflammatory oncotaxis). The commonest form of leukemia to affect the skin of elderly males is chronic lymphocytic leukemia. However, when leukemia involves the mucous membranes, acute myeloid leukemia (acute monocytic and acute myelomonocytic leukemia) is the most likely diagnosis. When papules, nodules, or plaques develop on the head, neck, or torso in a middle-aged male accompanied by lymphadenopathy, there must be a high index of suspicion that these lesions are metastatic lymphomatous deposits. Definitive histologic diagnosis on a skin biopsy specimen is difficult. In this situation, it is best to rely on histologic patterns seen in lymphoid tissue along with cellular marker studies. An elderly patient having bone pain, anemia, elevated blood calcium level, and renal failure along with purplish or skin-colored nodules and plaques on the trunk has a good chance of having multiple myeloma. Biopsying these lesions is most certain to reveal atypical plasma cells, and blood immunoelectrophoresis will demonstrate characteristic monoclonal gammopathy. There are two malignancies seen in children under 3 years of age that often times affect the skin in a characteristic fashion. Letterer-Siwe disease, which is distinguished from other histocytic disorders by its cell of origin, the Langerhans cell, clinically shows maculopapular and erosive lesions distributed in a seborrheic pattern. Neuroblastoma derived from cells of the neural crest demonstrates clinically widespread bluish papulonodules. Kaposi's sarcoma, a multifocal vascular malignancy, has a wide spectrum of clinical expression. Those patients who are immunocompromised secondary to concomitant disease or immunosuppressive therapy are more susceptible to a disseminated fulminant course accompanied by opportunistic infection. In conclusion, although specific signs of internal malignancy are less common than nonspecific ones, they are just as important; if the clinician managing the cancer patient is familiar with these clues to internal disease, proper patient management will ensue.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Specific cutaneous manifestations of internal malignancy. 307 47

Allogeneic bone marrow transplantation is limited by the availability of suitable marrow donors and risk of graft-versus-host disease (GVHD) and opportunistic infection. In an attempt to ameliorate these limitations, umbilical cord blood has been postulated as an alternative source of allogeneic haemopoietic stem cells for transplantation. From September, 1994, umbilical cord blood from sibling donors has been used to reconstitute haemapoiesis in 44 children with acquired or congenital lympho-haemapoietic disorders, neuroblastoma, or metabolic diseases. Patients who had HLA-identical and HLA-1 antigen disparate grafts, had a probability of engraftment at 50 days after transplantation of 85%. No patient had late graft failure. The probability of grade II-IV GVHD at 100 days was 3% and the probability of chronic GVHD at one year was 6%. With a median follow-up of 1.6 years, the probability of survival for recipients of HLA-identical or HLA-1 antigen disparate grafts is 72%. We conclude that umbilical cord blood is a sufficient source of transplantable haemopoietic stem cells for children with HLA-identical or HLA-1 antigen disparate sibling donors with very low risk of acute or extensive chronic GVHD. The feasibility of umbilical-cord-blood transplantation with HLA-2 and HLA-3 antigen disparate sibling donors remains to be determined.
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PMID:Allogeneic sibling umbilical-cord-blood transplantation in children with malignant and non-malignant disease. 747 21

Myoclonic encephalopathy of infancy (MEI) is a unique cause of acute ataxia in infants and is a rare presentation of neuroblastoma. Five cases presenting to a tertiary referral children's hospital during a 10 year period are reviewed. Two cases were associated with a neuroblastoma. All children were treated with intramuscular injections of adrenocorticotropic hormone, with symptomatic improvement. One child died from an opportunistic infection following chemotherapy for neuroblastoma. The four survivors have mild to moderate clinical and intellectual deficits. Investigation and continuing observation for occult neural crest tumours is emphasized for all cases of MEI, though no underlying cause was found in 60% of children in this study.
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PMID:Myoclonic encephalopathy of infancy: a 10 year review. 786 61

From 1991 to 1995, we reviewed the medical records of 200 pediatric patients with systemic malignancies to study the occurrence of neurologic complications and their treatment. A total of 25 patients with neurologic complications were found. Complications included intracranial metastasis (one patient), intraspinal metastasis (one), spinal epidural compressions (three), leptomeningeal metastases (six), metabolic encephalopathy (10), opportunistic infection (one), cerebrovascular disorders (three), treatment complications (six) and paraneoplastic syndromes (two). Ten patients had seizures. One patient with acute lymphoblastic leukemia (ALL) had the unusual complication of cytomegalovirus retinitis and glaucoma. Seven patients had neurologic features at presentation. ALL was the most common malignancy (56%) and neuroblastoma (20%) was the second. Neurologic deficits are frequently seen in pediatric patients with systemic malignancies and can, in fact, be the presenting signs. Early diagnosis and treatment is important to prevent further neurologic disability.
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PMID:Neurologic complications of pediatric systemic malignancies. 885 52

Improving survival rates following pediatric bone marrow transplantation (BMT) will likely result in greater numbers of children progressing to end-stage renal disease (ESRD) because of prior chemotherapy, irradiation, sepsis, and exposure to nephrotoxic agents. Renal transplantation remains the treatment of choice for ESRD; however, the safety of renal transplantation in this unique population is not well established. We report our experience with living related renal transplantation in three pediatric patients with ESRD following prior BMT. Two patients with neuroblastoma and ESRD because of BMT nephropathy, and one patient with Schimke immuno-osseous dysplasia and ESRD because of immune complex mediated glomerulonephritis and nephrotic syndrome. Age at time of BMT ranged from 2 to 7 yr. All patients had stable bone marrow function prior to renal transplantation. Age at renal transplant ranged from 8 to 14 yr. All three patients have been managed with conventional immunosuppression, as no patient received a kidney and BMT from the same donor source. These patients are currently 7 months to 6 yr status post-living related transplant. All have functioning bone marrow and kidney transplants, with serum creatinine levels ranging 0.6-1.2 mg/dL. There have been no episodes of rejection. One patient with a history of grade III skin and grade IV gastrointestinal-graft-vs.-host disease (GI-GVHD) prior to transplantation, had a mild flare of GI-GVHD (grade I) post-renal transplant and is currently asymptomatic. The incidence of opportunistic infection has been comparable with our pediatric renal transplant population without prior BMT. One patient was treated for basal cell carcinoma via wide local excision. Renal transplantation is an excellent option for the treatment of pediatric patients with ESRD following BMT. Short-term results in this small population show promising patient and graft survival, however long-term follow-up is needed. Pre-existing immune system impairment and bone marrow function should be taken into consideration when weighing different immunosuppressive agents for renal transplantation. Patients who have undergone renal transplantation following BMT are at high risk for opportunistic infections and malignancy, and need life-long medical surveillance.
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PMID:Successful renal transplantation following prior bone marrow transplantation in pediatric patients. 1536 89

Mucormycosis is a rare but potentially mortal opportunistic infection caused by Mucorales. We present a case of rhinoorbital mucormycosis in an 11-year old male with neuroblastoma successfully treated with aggressive surgical debridement and antifungal combination of liposomal amphotericin B and posaconazole. Our patient developed signs of paranasal sinus infection and culture of fine needle biopsy grew Rhizopus arrhizus. Prompt treatment and drastic surgical resection led to complete clinical and radiological recovery without evidence of mucormycosis relapse.
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PMID:Successful treatment of rhinoorbital mucormycosis due to Rhizopus arrhizus with liposomal amphotericin B, posaconazole and surgical debridement in a child with neuroblastoma. 3124 70