11/152. Multifocal neuroblastoma: biologic behavior and surgical aspects.BACKGROUND: Although multifocal neuroblastoma is rare, its incidence has increased because of recent improvements in diagnostic tools and the introduction of mass screening. Among the 106 neuroblastoma cases treated at the authors' hospital between 1984 and 1998, 8 were multifocal neuroblastoma. methods: The authors examined clinicopathologic findings and biologic features, including MYCN amplification, NTRK1 and Ha-ras p21 expression, cellular dna content, and telomerase activity in these 8 multifocal neuroblastoma cases. Moreover, clinicopathologic findings were investigated with a review of 53 published cases of multiple neuroblastoma in the literature published in English between 1966 and 1999. RESULTS: Among these eight cases, five were detected by mass screening and three were incidental neuroblastomas. Histologically, all tumors were classified as ganglioneuroma or favorable neuroblastoma except one advanced case. All tumors lacked the MYCN gene amplification and expressed NTRAK1 mRNA and Ha-ras p21 protein. Cellular dna content showed that half of these tumors were near-triploid, and the proliferative index (%S-phase) of all tumors was less than 25%. High telomerase activity was detected in none of these cases. Four patients underwent multistage operation and five patients with bilateral adrenal neuroblastomas underwent tumor enucleation to preserve adrenal function. Currently, all patients are disease free and none have required corticosteroid replacement therapy. Among the previously reported 53 cases with multifocal neuroblastoma, 25 were incidentally detected, 18 had familiar history, and most patients without other major complications also had extremely good prognoses. CONCLUSIONS: These findings suggested that most multifocal neuroblastomas have favorable biologic features. Clinically, surgical approaches should be attempted to preserve organ function, especially adrenal function, and minimal invasive surgery should be performed. In cases of thoracoabdominal neuroblastoma, multistage surgery is effective and safe.- - - - - - - - - - ranking = 1keywords = adrenal (Clic here for more details about this article) |
12/152. Laparoscopic resection of an adrenal neuroblastoma detected by mass screening that grew in size during the observation period.Neuroblastomas (NB) identified by mass screening tests are characterized by benign features. Recently, laparoscopic resection has been applied to the treatment of patients with small adrenal NB (<2-3 cm). However, an increasing number of cases of small NB are followed without any treatment in japan because many cases regress spontaneously. We describe a case of right adrenal NB detected by mass screening that increased in size during an observation period of 8 months. In this case, laparoscopic resection was performed successfully. The size of the tumor was 27 x 20 x 18 mm at diagnosis and 51 x 42 x 35 mm when it was excised. Small adrenal NB that do not regress during the observation period may require laparoscopic resection before they reach 5 cm in maximum diameter.- - - - - - - - - - ranking = 2.3333333333333keywords = adrenal (Clic here for more details about this article) |
13/152. hemoperitoneum and disseminated intravascular coagulation in two neonates with congenital bilateral neuroblastoma.We report two neonates with anemia, hemoperitoneum, and bilateral palpable abdominal masses. Both developed bleeding: a hemoperitoneum in one and visceral hemorrhages secondary to disseminated intravascular coagulation in the other. Each child was thought to have an unusual complication of adrenal hemorrhage, as the masses were of mixed echogenicity. However, histological evaluation revealed neuroblastoma. These cases point out the diagnostic dilemma of a mixed echogenicity suprarenal mass in the neonatal period and emphasize that local and disseminated bleeding can occur as a major manifestation of neuroblastoma.- - - - - - - - - - ranking = 0.33333333333333keywords = adrenal (Clic here for more details about this article) |
14/152. Pitfalls in the interpretation of MIBG scans in cervical neuroblastoma.Cervical neuroblastoma (CNB) is relatively rare, accounting for less than 5% of these tumors. Because it arises from the cervical sympathetic chain, complete resection will leave the child with Horner's syndrome in a high proportion of cases. Advances in technology have allowed for the development of diagnostic and imaging modalities more specific to the disease. One of these has been the advent of radiolabeled meta-iodobenzylguanidine (MIBG) to assess the primary tumor and focal metastatic involvement. This nuclide is also taken up by normal salivary-gland tissue; this may be altered, however, in the presence of sympathetic denervation. We present a case of a primary CNB associated with Horner's syndrome, which led to confusion in interpretation of the subsequent MIBG scan. We alert the reader to potential pitfalls in the use of this examination in this disease entity.- - - - - - - - - - ranking = 0.0060480360231563keywords = gland (Clic here for more details about this article) |
15/152. 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.- - - - - - - - - - ranking = 1.6787258809811keywords = adrenal gland, adrenal, gland (Clic here for more details about this article) |
16/152. Sarcoid reaction in primary neuroblastoma: case report.We present a unique case of a 9-month-old infant with a left adrenal neuroblastoma with sarcoid reaction, detected by mass screening. There was no clinical evidence indicating systemic sarcoidosis or pulmonary mycobacterial infection. Histological examination of the resected adrenal tumor revealed many noncaseating epithelioid granulomas with lymphocytic infiltrate, composed of epithelioid cells and few giant cells, arising in tumor parenchyma and fibrovascular stroma. Most of the lymphocytes in the granulomas were CD3- or CD45RO-positive T cells, with fewer being CD20-positive B cells. The lymphocytes in the epithelioid granulomas expressed CD4 or CD8, but not CD56 and CD57. CD4-positive cells were observed more within the granulomas (internal area) than in the surrounding area (external area) of the same granulomas, while most of the CD8-positive cells were seen consistently at the outer margin of the granulomas (marginal zone). CD45RA-positive T cells were observed predominantly in the external area. The results of immunostaining demonstrated that lymphocytes in granulomas of this case showed the same distribution pattern as that seen in systemic sarcoidosis. Although the sarcoid reaction is a phenomenon known to be associated with the region of cancer, granuloma within the primary neuroblastoma is extremely rare. The sarcoid reaction in the present case of neuroblastoma may be associated with a delayed-type hypersensitivity reaction, and its significance and relevance still remain obscure.- - - - - - - - - - ranking = 0.66666666666667keywords = adrenal (Clic here for more details about this article) |
17/152. Prenatal sonographic diagnosis of adrenal neuroblastoma.neuroblastoma is the most frequent extracranial solid tumor in childhood, but it is seldom diagnosed prenatally. We report a case of adrenal neuroblastoma identified at 39 weeks' menstrual age and successfully treated by postnatal surgery and chemotherapy. Sonography revealed a hyperechoic mass in the right upper quadrant of the fetal abdomen associated with hydramnios and hydronephrosis. Two days after delivery, the tumor was resected. No metastasis was identified. The infant received 6 cycles of chemotherapy, and at 2 years of age she was well, with no signs of recurrence.- - - - - - - - - - ranking = 1.6666666666667keywords = adrenal (Clic here for more details about this article) |
18/152. Case report: Calcifying fibrous pseudotumour of the adrenal gland.Calcifying fibrous pseudotumour is a distinct pathological entity usually occurring in the soft tissue of the extremities, trunk, axilla, pleura, mediastinum and peritoneum. This report describes the hitherto unreported occurrence of this tumour of the adrenal gland in a 10-year-old girl whose imaging findings closely resembled a neuroblastoma. This entity is a potential pitfall in diagnosing adrenal neuroblastoma.- - - - - - - - - - ranking = 7.0602960715722keywords = adrenal gland, adrenal, gland (Clic here for more details about this article) |
19/152. Conformal proton radiation treatment for retroperitoneal neuroblastoma: introduction of a novel technique.BACKGROUND: Postoperative irradiation for locoregionally advanced neuroblastoma requires coverage of the paraspinal retroperitoneum. The proximity of both kidneys and the liver, and a more complex target configuration, can pose a dosimetric challenge for conventional X-ray treatment and intraoperative irradiation. We utilized proton radiation therapy (PRT) to reduce dose to uninvolved kidneys, liver, intestine, and spinal cord. PROCEDURE: A 4-year-old male underwent PRT for neuroblastoma of the right adrenal gland, following chemotherapy and delayed surgical resection. Clinical target volume (CTV), boost volume, and normal structures were outlined on the 3D treatment planning CT scan. The patient received 25.2 CGE (cobalt Gray equivalent) to the CTV and 34.2 CGE to the boost region, using 1.8 CGE per fraction, five treatments per week. Dose-volume histograms (DVHs) were obtained for target and nontarget structures. RESULTS: The 95% isodose volume enclosed CTV and boost volumes. The dose to 50% of the ipsilateral kidney, with tumor involvement of the medial renal surface, was < or = 16 CGE (47% of prescribed total dose). Doses to 50% and 20% of the contralateral kidney in close proximity to deep left-side, paraspinal soft tissue involvement were restricted to 1 CGE and 10 CGE, respectively. Eighty percent of the liver received 27 CGE (80% of prescribed dose). Using a patch technique, unique to charged particle therapy, the spinal cord was almost completely spared during boost volume irradiation. CONCLUSIONS: PRT can achieve excellent dose conformity for advanced retroperitoneal, paraspinal lesions, while respecting normal tissue tolerance levels.- - - - - - - - - - ranking = 1.3453925476478keywords = adrenal gland, adrenal, gland (Clic here for more details about this article) |
20/152. Congenital neuroblastoma mimicking early onset sepsis.A newborn girl presented with symptoms of severe early onset sepsis but also with systemic hypertension (SH) at age 3 h. plasma catecholamine (CAT) levels were extremely elevated, reflecting increased release of CAT from a congenital neuroblastoma (NB). Clinical symptoms at time of admission were: prolonged capillary refill (5 s), tachycardia, tachydyspnoea, metabolic acidosis (pH 7.17, lactate 11.8 mmol/l), fever (38.4 degrees C) and SH: 90/50/65 mmHg (systolic/diastolic/mean). The infant experienced organ failure (lung, heart, liver). A retroperitoneal dumbbell tumour was detected. plasma CAT levels at age 15 h were: noradrenaline 219 nmol/l; adrenaline 13 nmol/l; and dopamine 65.3 nmol/l. SH responded to intermittent alpha-adrenergic blockage. CAT-related symptoms ceased within 1 week. The intraspinal NB was surgically removed when cord compression became symptomatic. The neurological and developmental state is normal at age 17 months. The abdominal NB regressed spontaneously. CONCLUSION: A neuroblastoma should be considered in newborn infants presenting with a shock-like condition together with systemic hypertension.- - - - - - - - - - ranking = 0.66666666666667keywords = adrenal (Clic here for more details about this article) |
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