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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe 11 cases (8 females, 3 males) of papillary thyroid carcinoma in children treated at St. Jude Children's Research Hospital over a 33-year period, and review the literature. Ages ranged from 7-25 years (median, 16 years). Six patients had primary papillary thyroid carcinoma. Five patients had secondary papillary thyroid carcinoma after treatment of Hodgkin's disease (n = 2), acute lymphoblastic leukemia (n = 2), and neuroblastoma (n = 1) with chemotherapy and cervical radiation. The typical presentation was either cervical lymphadenopathy or a thyroid mass of short duration. Treatment consisted of thyroidectomy, cervical lymph node dissection, and postoperative thyroid hormone replacement (n = 1), parathyroid reimplantation (n = 1), 131I ablation (n = 4), external-beam irradiation (n = 1), and chemotherapy with doxorubicin (n = 1) or carboplatin and topotecan (n = 1). Nine patients are alive without evidence of disease 3.0-22.4 years from diagnosis. One patient has persistent but stable disease 17.3 years after diagnosis. One patient relapsed with metastatic lung disease 0.3 years after the initial diagnosis. He continues to do well after a brief but unsustained complete radiographic remission of disease to combination chemotherapy with carboplatin and topotecan. Our review supports excellent long-term outcome for primary or secondary papillary thyroid carcinoma in pediatric patients although complications may require close follow-up in a multidisciplinary setting.
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PMID:Papillary thyroid carcinoma: demographics, treatment, and outcome in eleven pediatric patients treated at a single institution. 914 89

Fine-needle aspiration cytology is a valuable technique in the work-up of nodules and masses arising within the head and neck. Squamous cell carcinoma is present most often, and because of this relative frequency, the primary utility of needle-aspiration cytology is in the confirmation or exclusion of this diagnosis. FNA is particularly helpful in the work-up of cervical masses and nodules because biopsy of cervical adenopathy should be avoided unless all other diagnostic modalities have failed to establish a diagnosis. As such, needle-aspiration cytology represents an accurate, inexpensive, and rapid technique for elucidation of the etiology of cervical adenopathy. The majority of aspirates from cervical lymph nodes will disclose either reactive lymphadenopathy or metastatic squamous cell carcinoma. Occasional nodules will be due to lymphoma. While primary diagnosis of lymphoma by needle-aspiration cytology is generally not considered definitive, it is helpful in clarifying the nature of the process and the direction additional diagnostic tests should take. Similarly, establishing the presence of carotid body tumors, brachial cleft cysts or epidermal inclusion cysts excludes metastatic carcinoma and negates the need for open biopsy as well as allaying concerns on the part of both clinician and patient. Fine-needle aspiration of lesions within the mouth, oral pharynx, nasopharynx, and nasal sinuses has similar diagnostic goals, in that eliminating squamous cell carcinoma is its paramount objective. Fine-needle aspiration cytology can also establish a specific diagnosis for many lesions within this area. This technique can make specific diagnoses of angiofibroma, primary adenocarcinoma of the nasal sinuses, rhabdomyoma, granular cell tumor, and rhabdomyosarcoma. Each of these represents an important clinical entity with a specific therapy. Utilizing electron microscopy and immunohistochemical techniques, along with flow cytometry, can greatly broaden the diagnostic range and specificity of needle-aspiration cytology. Flow cytometry and immunohistochemistry are particularly useful in the establishment of monoclonality in lymphoproliferative processes and, hence, aid in the separation of reactive from lymphomatous lymphadenopathy. Immunohistochemistry can establish the precise nature of lesions as variable as rhabdomyosarcoma, olfactory neuroblastoma, and granular cell tumor. The prudent use of these techniques can be cost-effective and negate the need for more invasive diagnostic procedures. Needle-aspiration cytology represents a cost-effective and rapid technique for the assessment of nodules and masses within the head and neck area. Limitations in accuracy exist. In particular, the separation of reactive atypia in benign squamous epithelium from well-differentiated squamous cell carcinoma may be exceedingly difficult, if not impossible. Nonetheless, the technique has a high degree of accuracy for the diagnosis of both primary and metastatic disease.
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PMID:Fine-needle aspiration of the head and neck. 923 65

Lymphoma, mediastinal cysts, and neurogenic neoplasms are the most common primary middle and posterior mediastinal tumors. Lymphoma may involve the anterior, middle and/or posterior mediastinum, frequently as lymphadenopathy or as a discrete mass. Foregut cysts are common congenital mediastinal cysts and frequently arise in the middle mediastinum. Pericardial cysts are rare. Schwannoma and neurofibroma are benign peripheral nerve neoplasms, represent the most common mediastinal neurogenic tumors, and rarely degenerate into malignant tumors of nerve sheath origin. Sympathetic ganglia tumors include benign ganglioneuroma and malignant ganglioneuroblastoma and neuroblastoma. Lateral thoracic meningocele is a rare cause of a posterior mediastinal mass.
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PMID:Primary mediastinal tumors: part II. Tumors of the middle and posterior mediastinum. 1008 19

Nineteen children with neuroblastoma (aged 2 w.-7 y.o.) were studied to evaluate the optimal scan conditions for Iodine-123-Metaiodobenzylguanidine (MIBG) scintigraphy for accurate staging at the time of diagnosis. Six and 24 hours after an injection of 123I-MIBG, whole body image and truncal spot and SPECT images were obtained. Compared with other studies (CT or MRI and bone scintigraphy), each 123I-MIBG image was evaluated visually to investigate which image can demonstrate the extent of neuroblastoma most exactly. MIBG images demonstrated primary tumors in all patients, and metastatic lymphadenopathy in 8 of 9 patients. Twenty-four hour SPECT images gave us the most detailed information about the extent of abnormal accumulation. As to bone and bone marrow lesions, 6 hour images were superior to 24 hour images in detectability. Moreover, MIBG showed many more lesions and more extended accumulation than the bone scan. 123I-MIBG scintigraphy was very useful in detecting neuroblastomas. In order to get the most valuable information, both delayed SPECT and early whole body planar images should be obtained.
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PMID:[123I-metaiodobenzylguanidine (MIBG) scintigraphy for the staging of neuroblastoma]. 991 99

Cervicothoracic lesions are not uncommon in children. All cervicothoracic lesions except superficial lesions extend from the neck to the thorax through the thoracic inlet. Evaluation of this area involves multiple imaging modalities: plain radiography, ultrasonography, nuclear medicine, computed tomography, and magnetic resonance (MR) imaging. However, MR imaging is the method of choice for assessing the full extents of cervicothoracic lesions and their relationships to neurovascular structures. Cervicothoracic lesions can be classified as congenital lesions, inflammatory lesions, benign tumors, malignant tumors, and traumatic lesions. Lymphangioma is the most common cervicothoracic mass in children; other congenital lesions include hemangioma, thymic cyst, and vascular anomalies. Inflammatory adenopathy reactive to tuberculosis, mononucleosis, tularemia, cat-scratch fever, infection with human immunodeficiency virus, or other upper respiratory tract infections can manifest as cervicothoracic lesions; tuberculous abscesses and abscesses of other origins can also be seen. Lipoma, lipoblastoma, aggressive fibromatosis, and nerve sheath tumors (either isolated lesions or those associated with neurofibromatosis) can also occur as cervicothoracic masses. Malignant cervicothoracic tumors include lymphoma, thyroid carcinoma, neuroblastoma, and chest wall tumors (rhabdomyosarcoma, Ewing sarcoma, and neuroectodermal tumor). Traumatic cervicothoracic lesions include pneumomediastinum of traumatic origin, traumatic pharyngeal pseudodiverticulum, esophageal foreign-body granuloma, and cervicothoracic hematoma.
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PMID:Cervicothoracic lesions in infants and children. 1033 90

A case of congenital thyroid teratoma with nodal spread is reported. Primary surgery was performed on a female infant on the 6th day of life. The thyroid mass was removed in toto, and an adjacent 1.2 cm lymph node was also removed. Histology showed solid and cystic teratoma with a variety of elements including prominent neurological tissue that was neuroblastoma-like in places. Residual compressed non-neoplastic thyroid tissue was identified in the subcapsular plane. The lymph node was largely replaced by neuroglial tissue that was cellular in some areas and showed intrasinusoidal growth and some mitotic activity. Recurrent cervical lymphadenopathy gradually developed, commencing a few months after surgery. Excision of cervical nodes was undertaken at 9 months of age. About 13 nodes up to 2 cm in diameter were excised. Most of the specimens consisted of reactive lymph nodes, but in three of the smaller nodes, there were subcapsular and sinusoidal masses of focally cellular neuroglial tissue, again with occasional mitoses. This tissue stained strongly for glial fibrillary acidic protein, in addition to expressing neural markers. The lymph node "deposits" were interpreted as "displaced" lesional tissue rather than metastases in the usual, aggressive sense. The girl remains well at 5 years of age.
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PMID:Congenital thyroid teratoma: a case with persistent neuroglial involvement of cervical lymph nodes. 1046 96

Although cancer has an annual incidence of only about 150 new cases per 1 million U.S. children, it is the second leading cause of childhood deaths. Early detection and prompt therapy have the potential to reduce mortality. Leukemias, lymphomas and central nervous system tumors account for more than one half of new cancer cases in children. Early in the disease, leukemia may cause nonspecific symptoms similar to those of a viral infection. Leukemia should be suspected if persistent vague symptoms are accompanied by evidence of abnormal bleeding, bone pain, lymphadenopathy or hepatosplenomegaly. The presenting symptoms of a brain tumor may include elevated intracranial pressure, nerve abnormalities and seizures. A spinal tumor often presents with signs and symptoms of spinal cord compression. In children, lymphoma may present as one or more painless masses, often in the neck, accompanied by signs and symptoms resulting from local compression, as well as signs and symptoms of systemic disturbances, such as fever and weight loss. A neuroblastoma may arise from sympathetic nervous tissue anywhere in the body, but this tumor most often develops in the abdomen. The presentation depends on the local effects of the solid tumor and any metastases. An abdominal mass in a child may also be due to Wilms' tumor. This neoplasm may present with renal signs and symptoms, such as hypertension, hematuria and abdominal pain. A tumor of the musculoskeletal system is often first detected when trauma appears to cause pain and dysfunction out of proportion to the injury. Primary care physicians should be alert for possible presenting signs and symptoms of childhood malignancy, particularly in patients with Down syndrome or other congenital and familial conditions associated with an increased risk of cancer.
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PMID:Recognition of common childhood malignancies. 1077 55

Embryonal rhabdomyosarcoma (ERMS) is one of the commonest childhood tumors that usually presents as a small round cell tumor (SRCT). We report a case of an ERMS expressing frequent and prominent intranuclear cytoplasmic inclusions (INCIs). Our patient was a 3-year-old female child who presented with proptosis of the left eye with left sided cervical lymphadenopathy. Fine-needle aspiration (FNA) smears from both the orbital lesion and the cervical lymph nodes were cellular with features of a small round cell tumor (SRCT). The most striking cytologic feature observed was the presence of frequent and highly prominent INCIs. Despite the diagnostic dilemma caused by this unusual finding, a differential diagnosis of peripheral neurectodermal tumor (PNET) /Ewing's sarcoma, neuroblastoma, and ERMS was considered. Immunocytochemistry (ICC), using an appropriate panel of markers showed it to be a case of ERMS. Interestingly, though, not as frequent as on cytologic smears; INCIs could be appreciated even on biopsy material. INCIs in an ERMS are highly unusual and, to our knowledge, have not been documented so far. As a result of lack of any such cases in the literature, at present, the significance of this unique finding is not clear, and therefore needs to be investigated, as and when such cases are encountered in the future.
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PMID:Intranuclear cytoplasmic inclusions--an extremely unusual finding in embryonal rhabdomyosarcoma: Report of a case diagnosed by fine-needle aspiration biopsy. 1953 98

The authors describe 10 cases of myelofibrosis diagnosed and managed at their center over 16 years. There were 2 and 8 cases, respectively, of primary and secondary myelofibrosis. All patients presented with fever, pallor, hepatosplenomegaly, and/or lymphadenopathy. Hodgkin's lymphoma (n = 4), neuroblastoma (n = 1), thrombasthenic thrombopathy (n = 1), and retroperitoneal-mass (n = 1) were causal in 7 patients, whereas the diagnosis could not be established in a sole case of secondary myelofibrosis. Patients were managed with chemotherapy and appropriate care. However, outcome was poor. The authors emphasize variable clinical-laboratory spectrum of myelofibrosis, highlight management concerns, and demonstrate that prognosis/outcome depends upon appropriate management of the underlying condition.
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PMID:Myelofibrosis in children: experience at a single tertiary care center in India. 2067 Jan 65

Mesoblastic nephroma is by far the most frequent intrarenal fetal tumor. To the best of our knowledge, we report the first case of a newborn with an intrarenal neuroblastoma that was discovered prenatally. An intrarenal echogenic and homogenous mass was observed on routine prenatal ultrasonography, corroborated by magnetic resonance imaging, in a 30-week gestation fetus. A male weighing 3280 g was born with elevated blood pressure and cardiac failure. Postnatal ultrasound confirmed a left intrarenal tumor with microcalcifications and perirenal adenopathy. An open total left nephrectomy by laparotomy was performed. The pathologic study reported that the mass was an intrarenal neuroblastoma with local and regional invasion. Immediate postoperative urine analysis revealed a high level of vanillylmandelic acid, and blood samples showed high levels of normetanephrine. The purpose of this report is to demonstrate that prenatal intrarenal neuroblastoma can clinically and radiologically mimick a mesoblastic nephroma. High blood pressure, calcifications, and lymphadenopathy on ultrasound should raise the index of suspicion for a possible malignant process. Preoperative measurement of urinary vanillylmandelic acid (VMA) and metanephrines should be performed if the diagnosis is in doubt.
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PMID:Prenatal intrarenal neuroblastoma mimicking a mesoblastic nephroma: a case report. 2290 38


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