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)

Eleven patients suffering from neuroblastoma and Wilms' tumor were examined with MR imaging (25 examinations). The results were compared with those obtained with other diagnostic methods--e.g. CT--to verify MR reliability in locating and staging the lesions. Signal patterns were studied, as recorded on T1- and T2-weighted sequences using various repetition and echo times. An attempt was made to verify the presence of pathognomonic findings and to differentiate tumors from post-therapy fibroses. All findings were subsequently confirmed at biopsy or, in the patients who underwent surgery, by surgical findings. To carry out all the procedures, the patients had to be sedated or to be administered a general anesthetic. When possible, a brain or surface coil was used to obtain a clear, well-defined image in thin-section (7-8 mm) sequences. MR imaging allowed all lesions to be detected and located in the examined population. A large thrombus in the vena cava and local adenopathy were observed in a patients suffering from Wilms' tumor. Spread into the spinal canal was present in 2 cases of neuroblastoma. One of the main advantages of MR imaging is its multiplanarity which usually allows lesion extent to be demonstrated, together with the involved organs. The additional advantages of MR imaging over CT should not be underestimated. MR allows images of the abdomen to be obtained devoid of respiratory artifacts and provides a sharp contrast between pathological and healthy tissue without contrast medium administration. In all the patients who underwent it, MR imaging proved superior to CT in the evaluation of the intraspinal spread of paraspinal lesions. MR also allowed serial follow-up to be carried out, with no risk of subsequent proteximetric problems, thus helping distinguish tumor masses from fibrous tissue in all patients.
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PMID:[Magnetic resonance in the study of Wilm's tumor and neuroblastoma]. 166 19

The majority of lesions in the neck of children will prove to be benign and of congenital origin arising from such structures as the thyroglossal duct and the branchial clefts in addition to hemangiomas and lymphangiomas. However, it is essential that lymphadenopathy be proven infectious and any asymptomatic lymph node enlargement must be considered Hodgkin's disease until proven otherwise. The occasional neuroblastoma or rhabdomyosarcoma presenting in the neck can usually be identified as solid by sonography, leading to early investigation and biopsy. Because of the abundance of important structures that course through the neck, surgery should be conducted in an operating suite with sophisticated, modern anesthetic techniques and with a surgeon experienced in dealing with the full array of lesions that occur.
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PMID:Common lumps and bumps of the head and neck in infants and children. 266 78

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

The sonograms and medical records of 49 patients were reviewed to determine if there is a characteristic sonographic appearance for certain neck masses. Neck masses included: inflammatory masses (12), noninflammatory masses (23), and thyroid masses (14). Four entities including thyroglossal duct cyst, fibromatosis colli, cystic hygroma, and multiple lymphadenopathy showed characteristic sonographic appearance. Thyroglossal duct cysts were seen as midline or slightly off midline cystic masses. A sinus tract extending superiorly was nicely demonstrated in one patient. The mass in fibromatosis colli (neonatal torticollis) appeared as a well defined mass clearly within the sternocleidomastoid muscle, uniformly echogenic, but less echogenic than the normal surrounding muscle, without good through-transmission. A cystic or primarily cystic mass with linear septations was the characteristic finding of the cystic hygroma. Multiple lymphadenopathy demonstrated multiple discrete, oval, relatively hypoechoic masses along the cervical lymphatic chain. The sonographic appearance of inflammatory masses was variable, being either inhomogeneously echogenic or of mixed echogenicity. Hemangiomas were either echogenic with cystic vascular spaces or linear septations or relatively homogeneously echogenic. Intrinsic thyroid masses could be distinguished from extrinsic masses in most cases. The demonstration of calcification in a mass was useful in narrowing the differential diagnosis. Although it is not a specific finding, the presence of calcification highly suggests a neoplastic lesion, particularly neuroblastoma or teratoma. Not only can the location, extent, and internal characteristics of a mass be determined, but in certain entities, the sonographic appearance is characteristic and an accurate diagnosis can be made.
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PMID:Sonography of neck masses in children. 351 42

We reviewed the Tumor Registry for 1981 at the Children's Hospital of Philadelphia to identify all the children with newly diagnosed cancer who were seen initially in the emergency department (ED). Of the 220 new patients listed, 16 (7.3%) sought initial care in the ED (1 per 4,500 ED visits). Seven had leukemia, five had non-CNS solid tumors (2 lymphoreticular, 1 Wilms', 1 neuroblastoma, and 1 ovarian), and four had CNS tumors. Among the children with leukemia, pallor (6) and decreased activity (4) were the most common complaints. Duration of symptoms ranged from 4 days to 3 weeks. Physical examination showed pallor (5), splenomegaly (4), fever (3), hepatomegaly (3), lymphadenopathy (3), and ecchymoses or petechiae (2). The complete blood count and peripheral smears were all abnormal. The five patients with non-CNS solid tumors had symptoms related to the location of their neoplasms. The patients with Wilms' tumor, neuroblastoma, and ovarian dysgerminoma had abdominal masses; the patient with lymphoma had a large, painful inguinal node; and the patient with histiocytosis X had an infiltrative rash, gingivitis, and pneumonitis. Of the four children with CNS tumors, three had headache, and one had an incidentally detected scotoma following head trauma. All four eventually had abnormal neurologic exams and computer tomographic scans, but two were discharged initially with psychiatric diagnoses. We conclude that cancer, although rare in children, occurs with greater relative frequency in the referral hospital ED than that predicted by published cancer rates from the referring hospital's ED.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Detection of cancer in the pediatric emergency department. 384 22

The catecholic amino acids, dopa and 5-S-cysteinyldopa, and the dopamine metabolite, homovanillic acid, were determined in 8 neuroblastomas. 5-S-Cysteinyldopa and/or dopa were detected in all cases and homovanillic acid was present in 5. Dopa was also found in two tumours in which no definite histological diagnosis could be made. Neuroblastoma cells were cultured from 7 patients. The ageing of human sympathoblasts in culture was accompanied by modifications in the ability to synthetize dopa, which is a precursor of catecholamines as well as of melanins, and the metabolite homovanillic acid. An increase in the levels of 5-S-cysteinyldopa, a metabolite of the melanocytes, has been observed. Concurrent modifications of ultrastructural morphology with the disappearance of granular vesicles and appearance of melanosomes were noticed. This modulation of the original phenotypic expression commonly resulted in cell death, but in one case of metastatic adenopathy of a neuroblastoma we have been able to establish a permanent pigmented cell line.
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PMID:Melanogenesis in cultured human neuroblastomas. 678 5

Fifty-five patients (38 and 17), whose age ranged 14 to 153 days, with a clinical suspicion of sternocleidomastoid mass, were examined with US. Neck movements were impaired in all patients and 6 of them exhibited no clinically palpable mass. Sternocleidomastoid masses were detected in 50 patients. Other neck masses (lymphadenopathy, neuroblastoma, lymphangioma) were demonstrated in the remaining 5 patients. US sensitivity was confirmed to be higher than that of clinical palpation in detecting the masses. Our results show that males (69%) and the right side (80%) were mostly affected. The sternal head (92%) and the lower third of the muscle (88%) were most commonly involved. Forty-four masses (88%) had homogeneous echostructure and their echogenicity relative to normal muscle was mostly isoechoic in 33 patients (66%) and hyperechoic in 11 patients (22%). Six masses (12%) had a patchy echotexture and mixed echogenicity. No cases of mostly hypoechoic echotexture or intralesional calcifications were observed. The mass to muscle ratio in the largest transverse sections ranged 25-175% (mean: 95%). Sternocleidomastoid masses disappeared in all 50 patients within their 6th month of age. US is confirmed as a valuable tool to diagnose and follow-up sternocleidomastoid masses.
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PMID:[Ultrasonic diagnosis of "hematoma" of the sternocleidomastoid muscle]. 764 25

A case of a parotid mass in a 2-year-old boy, postoperatively diagnosed as neuroblastoma, a rare tumour not previously reported in the parotid gland is presented. The neoplasm developed within the parotid gland as a painless mass without regional lymphadenopathy. Histopathologically, the tumour showed primitive nerve cells-neuroblasts-with round or oval dark basophilic nuclei and scanty cytoplasm. The cells were arranged in circular rosettes around an eosinophilic mass consisting of very fine filaments originating in the tumour cells or papillary configuration and sometimes scattered in the poorly developed stroma. Immunohistochemical evaluation of the tumour showed a positive immunoreactivity for vimentin, alpha and beta subunits of S-100 protein, neurone-specific enolase (NSE), substance P, met-enkephalin and chromogranin but cytokeratins, desmin, actin, myosin, glial fibrillary acidic protein (GFAP) and calcitonin gene related peptide (CGRP) were negative. The histopathological and immunohistochemical findings conclude a diagnosis of neuroblastoma of the parotid gland.
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PMID:Neuroblastoma of parotid gland: report of a case and immunohistochemical characteristics. 770 7

To assess the utility of the current imaging modalities, a six-year retrospective study was conducted from May 1985 through April 1991 on 11 patients with histopathologically confirmed neuroblastoma. There were four males and seven females. Median age at presentation was six years. All patients underwent intravenous urography (IVU), abdominal ultrasonography (US), skeletal surveys and bone marrow aspiration. Computed tomographic (CT) scanning was performed in nine patients and trephine bone biopsy in five. Suprarenal gland was the primary site in six patients, including one bilateral neuroblastoma. Two presented with primary paraspinal mass, one with posterior mediastinal mass, one had a retroperitoneal tumour and one presented as primary cerebral neuroblastoma. IVU showed indirect evidence of suprarenal tumours in five patients. US demonstrated suprarenal mass in six and calcification within the mass in five patients. US also documented retroperitoneal lymphadenopathy in five patients. US-guided fine needle aspiration biopsy was performed in seven patients and it gave correct histological diagnosis in all. CT accurately depicted all the primary tumours and their involvement of the surrounding structures and metastases in the brain and orbits. US proved to be quite accurate in diagnosing primary abdominal tumours despite its limitations but in other sites, CT was of greater help. Solid appearance of a suprarenal mass on US made the diagnosis of neuroblastoma very likely and calcification within the mass further supported the diagnosis.
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PMID:Neuroblastoma and diagnostic imaging. 826 49

Most neuroblastoma identified by mass screening are clearly different from the clinically diagnosed tumors with respect to biology, epidemiology, and outcome. Because the neuroblastomas detected by mass screening have favorable biological features (97% survival rate at 5 years), aggressive surgery may not be appropriate for such tumors. Laparoscopic adrenalectomy was performed on a 9-month-old boy and two 8-month-old girls who had suitable lesions. The tumors were small (< 20 mm in diameter). With the patients under general anesthesia, five trocars were placed in the abdomen. The abdominal wall traction method was used in addition to pneumoperitoneum. Intraabdominal pressure was maintained at below 4 mm Hg. The adrenal tumors were well encapsulated and completely excised, placed into a plastic bag, and removed through one of the 10-mm trocar sites. No lymphadenopathy was observed. The postoperative course was uneventful. The tumors were of favorable Shimada histology and had no N-myc gene amplification. The patients have survived, with no evidence of recurrence or metastasis, through the follow-up period of 17 to 22 months.
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PMID:Laparoscopic surgery for neuroblastoma identified by mass screening. 870 8


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