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

Testicular and even more paratesticular tumours in children are rare. The aim of the study is to characterise the spectrum of these lesions with focus on the feasibility and effectiveness of testis sparing surgery. Twenty-four boys treated between 1980 and 2004 at the University Leipzig Medical Centre were evaluated. At presentation patients were between 5 months and 18 years old (median 23 months). Generally a high rate of malignant or potentially malignant tumours was observed. The majority of these tumours occurred in the first three years of age. The spectrum of testicular tumours comprised 13 germ cell tumours (6 yolk sac tumours, 6 teratomas, 1 embryonal carcinoma) and 4 sex cord stromal tumours (2 Leydig's cell, Sertoli's cell, granulosa cell). Both Leydig's cell tumours were endocrine active. Further on, we observed 3 boys with paratesticular rhabdomyosarcoma (RMS), and three with testicular and paratesticular metastases (Wilms' tumour, neuroblastoma, leukaemia). Serum alpha1-fetoprotein (AFP) was clearly elevated in 5 of 6 yolk sac tumours but remained within normal limits concerning the other entities. Human chorionic gonadotrophin was normal in all cases tested. During the observation period high inguinal orchidectomy was the surgical standard method. Dependent on tumour histology, stage and the recommended treatment schedule postoperative chemotherapy was added. Testis sparing surgery was performed in 3 boys with primary testicular tumours (2 Leydig's cell, mature cystic teratoma). Local relapses were not observed. Systemic relapses occurred in 3 cases (2 RMS, leukaemia). During a median follow up of 5 years all patients with primary testicular tumours survived event free. Meta-analysis of the recent literature revealed that testis sparing surgery is feasible and save in prepubertal boys after exclusion of a malignant tumour. If a testis sparing approach is planned, the following criteria are essential: 1. The presence of a well defined circumscribed nodule confirmed by imaging. 2. Normal levels of serum AFP and hCG. 3. The presence of sufficient healthy testicular parenchyma. However, the high rate of malignant or potentially malignant tumours suggests that high inguinal orchidectomy should remain the surgical standard of therapy.
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PMID:Surgery in infants and children with testicular and paratesticular tumours: a single centre experience over a 25-year-period. 1752 8

Neuroblastoma (NB) is the most common malignant solid tumor in childhood. There are well-recognized prognostic factors in NB such as age at diagnosis, organ of origin, stages, MYCN gene amplification, and expression of H-ras, trkA and survivin. Moreover, we investigated the expression of vascular endothelial growth factor (VEGF), tyrosine hydroxylase (TH), p53, stem cell factor (SCF) and c-kit of its receptor with quantitative real-time polymerase chain reaction (PCR) in 22 NBs and 4 other tumors (one malignant lymphoma, one malignant teratoma, and 2 rhabdomyosarcomas) samples. The correlation between patients' prognoses and the expression of TH or c-kit was newly recognized, particularly the good prognosis in patients in whom c-kit highly expressed and the poor prognosis contrarily associated with low or no expression, although the SCF of its ligand had no relationship with patient prognosis. It is possible that tumors without c-kit expression can not react with SCF (via the autocrine or paracrine system) and remain immature. It may be that this is a new critical clinical event in NB patients.
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PMID:Analyses of novel prognostic factors in neuroblastoma patients. 1805 15

Fetuses with tumors associated with hydrops have a high mortality rate. Relatively few survivors have this potentially fatal combination. This study examined the clinical and pathological findings, pathogenesis, and outcomes of fetuses with tumors and hydrops. One hundred and fifty-eight study cases were collected from the literature and from personal files. Only patients where adequate clinical and pathological data were given and the outcome of pregnancy was described were included in the study. Cardiac tumors were the majority found in association with fetal hydrops. Leukemia and extracardiac teratomas were next in frequency followed by hepatic tumors, neuroblastoma, placental, soft tissue, and renal tumors. The main presenting findings along with hydrops were hydramnios, a tumor mass, placentomegaly, and stillbirth. Most tumors were detected in the third trimester of pregnancy. No fetus with the diagnosis of cardiac rhabdomyoma, neuroblastoma, brain tumor, rhabdoid tumor, or histiocytosis associated with hydrops survived. Those patients with placental chorangioma, pericardial teratoma, and hepatic hemangioma had the best outcome. The overall survival rate was low: 30 of 158 (19%).
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PMID:Fetal hydrops associated with tumors. 1807 61

Craniofacial resection is commonly performed in the surgical resection of sinonasal tumours involving anterior skull base. It entails a bicoronal scalp flap with lateral rhinotomy or an extended lateral rhinotomy to expose the anterior skull base. Transfacial approach is necessary in the resection of the nasal part of the tumour. The choice of surgical approach is based heavily on the surgeon's experience and training. The results of endoscopic-assisted craniofacial resection for sinonasal tumours performed in our center in eight patients from 1998 to 2005 were reviewed. There were seven males and one female with age ranging from 18 to 62 years (mean 42.4 years). There was each a case of mature teratoma, poorly differentiated squamous cell carcinoma, undifferentiated squamous cell carcinoma, olfactory neuroblastoma, fibrous dysplasia, inverted papilloma and two cases of sinonasal neuroendocrine carcinoma. The mean follow up duration for these eight patients post surgery was 21.4 months. Out of eight patients, five underwent surgery with no adverse complications. The complications encountered were a cerebrospinal leak and a postoperative transient V and VI cranial nerve palsy. One patient with sinonasal undifferentiated carcinoma died of lung metastasis at 11 months post-surgery. The endoscopic-assisted craniofacial resection is a highly useful surgical technique to avoid the unsightly facial scar of the lateral rhinotomy or the Weber-Ferguson incision, postoperative paranasal sinuses infection and avoidance of tracheostomy in selected cases. We found that this approach has lower morbidity rate in selected cases.
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PMID:Endoscopic-assisted craniofacial resection: a case series and post-operative outcome. 1824 14

A 6-week-old girl presented with an abdominal mass and spinal cord compression. Clinical and radiological features indicated a diagnosis of congenital neuroblastoma. Histology revealed a diagnosis of germ cell tumour after therapy for neuroblastoma had been commenced. This is, to the authors' knowledge, the first reported case of paediatric dumbbell retroperitoneal teratoma.
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PMID:Pelvic teratoma with extensive spinal involvement in a neonate: an important differential diagnosis. 1856 32

This study reviewed different types of primary cardiac and mediastinal tumors in infants and children as well as their clinical presentation and management. Altogether, 34 consecutive patients followed from 1976 through December 2005 were analyzed. Of these 34 patients, 14 (41%) underwent surgery and 20 (59%) with rhabdomyomas were managed conservatively. Histologic examination of the surgically resected tumors showed teratoma in four patients, rhabdomyoma in four patients, and hemangioma in two patients. Myxoma, myofibroma, neuroblastoma, and malignant fibrous histiocytoma were encountered in one patient each. Follow-up evaluation was completed for 97% of the patients and extended up to 30 years. Half of the nonsurgical patients with rhabdomyoma showed partial or complete spontaneous regression. One patient died after resection of a malignant histiocytoma, and one patient required a tumor-related reoperation. Freedom from tumor-related reoperation after 10 years was 91% +/- 8.7%. Of the survivors, 85% were New York Heart Association (NYHA) class 1, and 100% had sinus rhythm. Spontaneous tumor regression is common in rhabdomyoma and surgery, and is indicated only for symptomatic patients with hemodynamically significant intracardiac obstruction. For all other benign primary cardiac tumors, complete resection usually can be accomplished with good results. Patients with giant tumor masses compressing or infiltrating the heart frequently cannot undergo complete resection. For these patients, restoration/preservation of sufficient heart function is the primary goal. Malignant tumors are extremely rare in pediatric patients and have a very poor prognosis.
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PMID:Treatment strategies for pediatric patients with primary cardiac and pericardial tumors: a 30-year review. 1860 Mar 70

Immature ovarian teratoma (IOT) is a rare and aggressive malignant neoplasm characterized by immature neural tissue. The cytomorphologic features have only rarely been described. We herein describe an additional case and review the literature regarding this entity. To the best of our knowledge, this is the first reported case with imprint cytology. A 35-year-old woman presented with a pelvic mass which was resected and sent for frozen section evaluation. Imprint smears and frozen section of the mass were diagnostic of IOT. IOT has diagnostic cytologic features which show complete concordance with histology. Differential diagnoses include other small round cell neoplasms such as ovarian neuroblastoma, small cell carcinoma of hypercalcemic type, primitive neuroectodermal tumor, Wilm's tumor, desmoplastic small round cell tumor, and Non-Hodgkin lymphoma. Distinguishing IOT from these tumors can be challenging however if diligent morphologic study and/or ancillary studies are performed accurate diagnosis is possible.
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PMID:Imprint cytology of high-grade immature ovarian teratoma: a case report, literature review, and distinction from other ovarian small round cell tumors. 1861 28

Cystic neuroblastoma (CN) is an extremely rare entity, although neuroblastoma is the most common solid tumor in infants. The radiologic diagnosis of CN is very difficult because of both the rarity and minimum solid component of the lesion. We describe herein the case of a 2-month-old girl presenting with dysuria because of a large presacral mass. Imaging studies including ultrasonography, computed tomography, and magnetic resonance imaging demonstrated a large septated cystic tumor mimicking a cystic sacrococcygeal teratoma, which commonly occurs in the presacral region. The tumor was finally diagnosed as CN after surgical resection. This is the second case report of presacral CN in the English literature. Cystic neuroblastoma should be considered in the differential diagnosis of presacral cystic tumors in infants.
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PMID:A rare case of presacral cystic neuroblastoma in an infant. 1863 1

We report a case of retroperitoneal teratoma diagnosed prenatally by serial sonographic examinations in the third trimester. A 29-year-old woman was referred for sonographic evaluation at 33 weeks' gestation because of a fetal intra-abdominal mass. Our initial sonographic image suggested a neuroblastoma. Repeat ultrasound images demonstrated an increase in size of the tumor, while the content of the tumor became predominantly solid with areas of calcification. Teratoma should be considered on detection of any cystic or mixed semisolid mass, especially when calcification is present. The fetus was prenatally diagnosed with retroperitoneal teratoma. After birth at 39 weeks, the tumor was removed and histological analysis revealed an immature retroperitoneal teratoma. Intensive monitoring of the changes in ultrasound images of the tumor should provide ground for a precise antenatal diagnosis.
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PMID:Prenatal diagnosis of retroperitoneal teratoma: a case report and review of the literature. 1921 6

A prenatally detected suprarenal cystic mass measuring 2 cm was found to have enlarged upon postnatal ultrasonography at 6 weeks of age. Magnetic resonance imaging showed a 57 x 50 mm mass in the left adrenal region displacing the kidney inferiorly. The infant underwent an adrenalectomy with total resection of the tumor, which proved on histologic examination to be a mature teratoma. Prenatally detected suprarenal masses are likely to be neuroblastoma or adrenal hemorrhage, but may be rare benign lesions such as extralobar pulmonary sequestration, bronchogenic cyst, or renal dysplasia. Although teratoma in the adrenal region is extremely rare, it should be included in the clinical and radiologic differential diagnosis of prenatally detected suprarenal masses. Total excision of the mass for histologic diagnosis is indicated.
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PMID:Prenatal diagnosis of a mass in the adrenal region that proved to be a teratoma. 1941 17


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