1/60. neuroblastoma metastatic to the liver in infants.Four infants are described who presented with rapid enlargement of the liver. This was found to be due to neuroblastoma which had metastasized to the liver; the condition was associated with high levels of urinary vanillylmandelic acid (VMA). In 3 infants the primary tumour was in the adrenal gland and in one it was not identified. One infant died after laparotomy and 2 infants survive disease free with normal VMA levels, one after adrenalectomy and hepatic irradiation and one after a short course of chemotherapy. The fourth patient responded initially to hepatic irradiation and chemotherapy, but relapsed 2 years later with recurrent disease; at the same time the VMA level which had been normal, again rose. The importance of screening for an abnormal VMA level in any infant with a rapidly enlarging liver in order to obtain an early diagnosis is stressed. Careful follow-up, with serial VMA estimations, is essential to detect recurrent disease. The prognosis for some infants with this distribution of neuroblastoma which has metastasized to the liver, but not to the bones or oribt, is good.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
2/60. Congenital bilateral cystic neuroblastoma with liver metastases and massive intracystic haemorrhage.A case of bilateral cystic neuroblastoma with liver metastases in a newborn is reported. CT showed a 10 cm right suprarenal multicystic mass and numerous hepatic cystic masses with intracystic fluid-fluid levels. Multiple smaller cystic lesions were also present in the left adrenal gland. To our knowledge, the CT findings of neonatal bilateral cystic neuroblastoma with liver metastasis and massive acute intracystic haemorrhage has not been previously documented.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
3/60. Metastatic neuroblastoma of the mandible mimicking osteogenic sarcoma radiologically. Case report.This paper presents a case of a neuroblastoma of the adrenal gland metastasizing to the mandible of a 21-month-old infant, which presented radiographically as the so-called 'sun-ray' appearance, characteristic of osteogenic sarcoma.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
4/60. 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 = 1keywords = gland (Clic here for more details about this article) |
5/60. 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 = 1keywords = gland (Clic here for more details about this article) |
6/60. 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 = 5keywords = gland (Clic here for more details about this article) |
7/60. 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 = 1keywords = gland (Clic here for more details about this article) |
8/60. Ewing's sarcoma family of tumor arising in the adrenal gland--possible diagnostic pitfall in pediatric pathology: histologic, immunohistochemical, ultrastructural, and molecular study.We present an adrenal Ewing's sarcoma family of tumor (ESFT) arising in an 11-year-old Japanese boy. Although intensive chemoradiotherapy and radical surgery were performed, the patient died of obstinate disease 1 year and 3 months after the initial presentation. The primary site (adrenal gland) with radiologic findings (with foci of calcification), high titer of serum neuron specific enolase, and sheets of monotonous primitive rounded cells on histology mostly favored neuroblastoma. However, a diagnosis of ESFT was confirmed by immunohistochemical profile, including MIC2-positivity and molecular study disclosing EWS-FLI1 chimera gene verified by direct sequencing. Recognition of adrenal ESFT and use of newly developed diagnostic techniques are required for differential diagnosis of undifferentiated small round cell tumor of the adrenal gland.- - - - - - - - - - ranking = 6keywords = gland (Clic here for more details about this article) |
9/60. osteosarcoma as a second malignancy after treatment for neuroblastoma.A 4-month old girl was diagnosed as having stage IV neuroblastoma of the right adrenal gland. Preoperative chemotherapy was given, followed by local surgical excision. Postoperatively, irradiation of the tumor bed and adjuvant chemotherapy was given for 11 months. Nine years after cessation of chemotherapy, the patient developed left hip-joint pain. biopsy of the ischium showed chondroblastic osteosarcoma. limb salvage surgery was performed after preoperative chemotherapy. Postoperatively, adjuvant chemotherapy was given for 14 months. Twenty-two months after treatment for the secondary osteosarcoma, the patient has been remained in disease-free condition without any evidence of relapse. A second osteosarcoma occurring outside the radiation field after treatment for neuroblastoma is quite rare. This unusual case emphasized the need for close monitoring for development of second malignant neoplasms in survivors of neuroblastoma even in the absence of a known predisposing factor, such as radiation therapy.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
10/60. Severe oral mucositis after therapeutic administration of [131I]MIBG in a child with neuroblastoma.OBJECTIVE: The purpose of this report is to document a newly encountered oral side effect of targeted radiotherapy with iodine 131-metaiodobenzylguanidine ([(131)I]MIBG) in the treatment of neuroblastoma. STUDY DESIGN: A 14-month-old girl was diagnosed with stage 4 neuroblastoma. After completion of chemotherapy, the tumor showed no signs of regression; treatment with 3700 MBq [(131)I]MIBG was therefore decided on, 8 months after diagnosis. RESULTS: Fourteen days after infusion of MIBG, severe oral mucositis was diagnosed, with a generalized erythema involving the mucous membranes of the hard and soft palate, buccal mucosa, and upper and lower lips. The gingiva exhibited a general linear erythema. CONCLUSIONS: Visualization of the salivary glands on [(123)I]MIBG images suggests that accumulation of radiolabeled MIBG in the salivary glands may be related to sympathetic innervation.- - - - - - - - - - ranking = 2keywords = gland (Clic here for more details about this article) |
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