Bilateral adrenal neuroblastoma.
Zaizen Y,Suita S,Yamanaka K,Nagoshi M,Tsuda T
European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift fur Kinderchirurgie
Neuroblastoma has been recognized as the most common solid tumor of infancy and childhood. The occurrence of bilateral adrenal neuroblastoma, however, is extremely rare and only a small number of cases have been previously reported. The authors herein report the clinical, histopathological and molecular biological features of two bilateral adrenal cases out of 125 neuroblastoma patients treated at Kyushu University Hospital over a 35-year period. The clinical and histopathological data of the two cases of bilateral adrenal neuroblastoma were reviewed. In Case 1, which had multiple liver metastases, a post-mortem examination revealed bilateral adrenal involvement. In Case 2, which had been detected by mass screening, CT showed masses in both adrenal glands. No big differences in size were recognized between the tumors in either of the cases. A histopathological examination revealed rosette fibrillary type neuroblastomas in both cases. The DNA of these tumor samples stored at -80 degrees C was extracted and the number of copies of the N-myc gene was determined by a Southern blot analysis. Fourteen copies of the gene were detected in Case 1, whereas neither of the tumors in Case 2 showed any amplification. The clinical outcome, histopathological findings and N-myc gene analysis of two cases might support the variety of biological features of this rare group of neuroblastoma.
10.1055/s-2008-1071179
Lineage-Independent Tumors in Bilateral Neuroblastoma.
The New England journal of medicine
Childhood tumors that occur synchronously in different anatomical sites usually represent metastatic disease. However, such tumors can be independent neoplasms. We investigated whether cases of bilateral neuroblastoma represented independent tumors in two children with pathogenic germline mutations by genotyping somatic mutations shared between tumors and blood. Our results suggested that in both children, the lineages that had given rise to the tumors had segregated within the first cell divisions of the zygote, without being preceded by a common premalignant clone. In one patient, the tumors had parallel evolution, including distinct second hits in , a putative predisposition gene for neuroblastoma. These findings portray cases of bilateral neuroblastoma as having independent lesions mediated by a germline predisposition. (Funded by Children with Cancer UK and Wellcome.).
10.1056/NEJMoa2000962
Bilateral adrenal neuroblastoma.
Pagès Perle Maliszewicz,Dufour Christelle,Fasola Sylvie,Michon Jean,Boutard Patrick,Gentet Jean-Claude,Hartmann Olivier
Pediatric blood & cancer
BACKGROUND:Bilateral adrenal neuroblastoma is extremely rare. To date, 45 cases have been reported in the literature. PROCEDURES:We retrospectively identified and reviewed 15 cases of bilateral adrenal neuroblastoma, treated between 1988 and 2004, by the French Society of Pediatric Oncology. We then compared our cohort to the 45 cases reported in literature. RESULTS:Median age at diagnosis was 4 months in our cohort whereas it was 3 months in the literature. The same percentage of infants was found in both series (86.6%). Disease had generally been detected due to metastasis-related symptoms. Ten out of 15 patients in our cohort and 18/39 cases in the literature were classified as stage 4S according to the International Neuroblastoma Staging System (INSS). The incidence of stage 4S was significantly higher than that described in other neuroblastoma cohorts (P = 10(-4)). Five-year overall survival was 90% for stage 4S. In our series, bilateral neuroblastoma was neither associated with familial cases nor with any risk factors. CONCLUSIONS:The majority of bilateral neuroblastoma carry a favorable prognosis. Exceptional cases exhibiting risk factors, such as amplified MYCN, are comparable to high-risk unilateral neuroblastoma cases with the same poor prognostic features. The therapeutic strategy could be similar to that used against unilateral neuroblastoma, except for surgery. However, the low incidence of relapse and the risk of adrenal failure if radical surgery is performed, argue against an aggressive surgical approach.
10.1002/pbc.21765
Magnetic Resonance Imaging Findings of Bilateral Cystic Neuroblastoma: Case Report of a Very Rare Entity.
Özgül Esra
Cureus
Neuroblastoma (NB) is the most common solid tumor seen in children under two years old. It has both solid and cystic forms. It generally involves the adrenal gland unilaterally. Only 10% of the NBs are as seen bilateral. Both bilateral and cystic forms of NB are a very rare entity. Magnetic resonance imaging (MRI) is a suitable imaging modality for evaluating lesions radiologically to avoid ionizing radiation exposure in children. Herein, we present the MRI findings of a bilateral cystic NB case.
10.7759/cureus.7073
[4S neuroblastoma with bilateral adrenal tumors].
Kerdudo C,Corradini N,Michon J,Leverger G
Archives de pediatrie : organe officiel de la Societe francaise de pediatrie
UNLABELLED:4S neuroblastoma with bilateral adrenal involvement is defined by small primitive tumors (stage 1 or 2) with disseminated disease restricted to the liver, skin, and/or bone marrow. Children are less than one year old. These tumors are rare and of multicentric origin. PATIENTS AND METHODS:Our multicentric study analyzed four children less than four months old at diagnosis. RESULTS:All had a favourable histology, with normal MYC-N copy number, and one case had a diploid tumor. The four patients had first supportive care at the beginning, but three cases received chemotherapy because of progressive disease, with liver radiotherapy in two cases because of massive hepatomegaly; three cases had surgery (unilateral adrenal resection in two cases and bilateral in one case) and one had only a biopsy. Surgery was the only treatment in one case. One patient relapsed 17 months after initial treatment and was treated with intensive chemotherapy and stem cell rescue. The outcome is favorable for the four patients, without evidence of recurrent disease. CONCLUSION:Children with 4S neuroblastoma with bilateral adrenal tumors have a good prognosis. Treatment should be the less aggressive as possible. The group with favorable prognostic parameters should have supportive care if spontaneous regression occurs. But we have to treat with chemotherapy neonates with massive hepatomegaly and children with one or more unfavorable prognostic factors (unfavorable histology, high MYC-N copy number).
10.1016/j.arcped.2004.09.020
Bilateral cystic neuroblastoma: imaging features and differential diagnoses.
Cassady C,Winters W D
Pediatric radiology
Neuroblastoma is one of the most common malignant tumors of childhood, with 40 % arising in the adrenal glands. Bilateral adrenal involvement from synchronous development or metastatic spread of the tumor is seen in less than 10 % of children with neuroblastoma [1]. Neuroblastoma rarely presents as a cystic suprarenal mass that is difficult to differentiate from adrenal hemorrhage, extralobar sequestration, or dilated upper-pole renal calyces. To our knowledge, bilateral cystic neuroblastoma has not been previously reported. We present a case of bilateral cystic adrenal neuroblastoma to demonstrate the imaging features of this unusual entity, and to expand the differential diagnosis of bilateral cystic suprarenal masses in an infant.
10.1007/s002470050220
[A case of bilateral adrenal neuroblastomas detected by bone scintigraphy].
Suga K,Shimizu K,Nishigauchi K,Kume N,Kawamura T,Matsunaga N
Kaku igaku. The Japanese journal of nuclear medicine
A 7-month-old girl, who had been resected the left adrenal neuroblastoma 20 days ago, underwent bone scintigraphy with 90mTc-HMDP to survey bone metastases. The bone scan demonstrated avid uptake in the right adrenal gland, in which 123I-MIBG also accumulated. However, the degree of 123-I-MIBG uptake in the right adrenal gland was not necessary higher compared to that in the normal adrenal glands in 7 patients after resection of hemilateral neuroblastomas. Reviewing the preoperative abdominal CT, a swelling of the right adrenal gland had been overlooked because of the small size of 10 x 12 mm in diameter. The follow-up CT after surgery revealed a more growth of the right adrenal gland, accompanied by reelevation of urine vanilmandelic acid. This patient was thus diagnosed as bilateral neuroblastomas. Because bone scintigraphy frequently shows abnormal uptake in primary neuroblastomas, it has potentially diagnostic value for early detection of bilateral adrenal neuroblastomas.
The feasibility of adrenal-sparing surgery in bilateral adrenal neuroblastoma.
Chui Chan Hon,Chang Kenneth Tou En
Journal of pediatric surgery
PURPOSE:Loss of adrenal function is a major concern in the treatment of children with bilateral adrenal neuroblastoma (BAN). We aimed to evaluate the feasibility of adrenal-sparing surgery in this unique subgroup. METHOD:Retrospective review of our center's neuroblastoma database was conducted. Patients with synchronous BAN confirmed at surgery were included. Their demographic data and clinical charts were analyzed. RESULTS:Five patients were confirmed with BAN. Mean age at diagnosis was 0.89 (0.39-1.32) years; male:female ratio was 4:1. They were stratified as stages M (n = 2), MS (n = 1), L1 (n = 1) and L2 (n = 1). MYCN amplification was present in 1 patient (stage M) and was stratified as high-risk group. All patients except one received preoperative chemotherapy. Among the 10 adrenal gland tumors, 2 underwent tumor adrenalectomy (TA) and 8 had adrenal-sparing tumorectomy (AST). After chemotherapy, 3 patients underwent single-stage bilateral AST and 1 patient underwent 2-stage TA-AST. One patient underwent upfront single-stage TA-AST, where lack of preoperative chemotherapy rendered the adrenal gland indistinguishable. The high-risk patient received autologous hematopoietic cell transplantation for consolidation. No patients required adrenal replacement therapy. All patients were alive without evidence of disease with mean follow-up 5.5 (2.6-8.5) years. CONCLUSION:Adrenal-sparing surgery is feasible in patients with BAN. LEVEL OF EVIDENCE:Level IV (case series with no comparison group).
10.1016/j.jpedsurg.2020.06.038