A Rare Tumor in the Cervical Sympathetic Trunk: Ganglioneuroblastoma

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Abstract

Ganglioneuroblastoma is a rare tumor with moderate malignancy, which is composed of mature ganglion cells and seen in sympathetic ganglia and adrenal medulla. The diagnosis is possible after cytological and immunohistochemical studies following a needle biopsy or surgical excision. There is no consensus regarding the need for chemo- or radiotherapy after surgery. In this case report, clinical behavior and diagnosis and treatment of the rare tumor cervical ganglioneuroblastoma were discussed.

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The International Neuroblastoma Risk Group (INRG) classification system: an INRG Task Force report.

Susan L. Cohn, Andrew D.J. Pearson, Wendy B. London … (2009)

Because current approaches to risk classification and treatment stratification for children with neuroblastoma (NB) vary greatly throughout the world, it is difficult to directly compare risk-based clinical trials. The International Neuroblastoma Risk Group (INRG) classification system was developed to establish a consensus approach for pretreatment risk stratification. The statistical and clinical significance of 13 potential prognostic factors were analyzed in a cohort of 8,800 children diagnosed with NB between 1990 and 2002 from North America and Australia (Children's Oncology Group), Europe (International Society of Pediatric Oncology Europe Neuroblastoma Group and German Pediatric Oncology and Hematology Group), and Japan. Survival tree regression analyses using event-free survival (EFS) as the primary end point were performed to test the prognostic significance of the 13 factors. Stage, age, histologic category, grade of tumor differentiation, the status of the MYCN oncogene, chromosome 11q status, and DNA ploidy were the most highly statistically significant and clinically relevant factors. A new staging system (INRG Staging System) based on clinical criteria and tumor imaging was developed for the INRG Classification System. The optimal age cutoff was determined to be between 15 and 19 months, and 18 months was selected for the classification system. Sixteen pretreatment groups were defined on the basis of clinical criteria and statistically significantly different EFS of the cohort stratified by the INRG criteria. Patients with 5-year EFS more than 85%, more than 75% to or = 50% to < or = 75%, or less than 50% were classified as very low risk, low risk, intermediate risk, or high risk, respectively. By defining homogenous pretreatment patient cohorts, the INRG classification system will greatly facilitate the comparison of risk-based clinical trials conducted in different regions of the world and the development of international collaborative studies.

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Terminology and morphologic criteria of neuroblastic tumors: recommendations by the International Neuroblastoma Pathology Committee.

H Shimada, I Ambros, L Dehner … (1999)

As part of the international cooperative effort to develop a complete set of International Neuroblastoma Risk Groups, the International Neuroblastoma Pathology Committee (INPC) initiated activities in 1994 to devise a morphologic classification of neuroblastic tumors (NTs; neuroblastoma, ganglioneuroblastoma, and ganglioneuroma). Six member pathologists (H.S., I.M.A., L.P.D., J.H., V.V.J., and B.R.) discussed and defined morphologically based classifications (Shimada classification; risk group and modified risk group proposed by Joshi et al.) on the basis of a review of 227 cases, using various pathologic characteristics of the NTs. The classification-grading system was evaluated for prognostic significance and biologic relevance. The INPC has adopted a prognostic system modeled on one proposed by Shimada et al. It is an age-linked classification dependent on the differentiation grade of the neuroblasts, their cellular turnover index, and the presence or absence of Schwannian stromal development. Based on morphologic criteria defined in this article, NTs were classified into four categories and their subtypes: 1) neuroblastoma (Schwannian stroma-poor), undifferentiated, poorly differentiated, and differentiating; 2) ganglioneuroblastoma, intermixed (Schwannian stroma-rich); 3) ganglioneuroma (Schwannian stroma-dominant), maturing and mature; and 4) ganglioneuroblastoma, nodular (composite Schwannian stroma-richlstroma-dominant and stroma-poor). Specific features, such as the mitosis-karyorrhexis index, the mitotic rate, and calcification, were also included to allow the prognostic significance of the classification to be tested. Recommendations are made regarding the surgical materials to use for an optimal pathobiologic assessment and the practical handling of samples. The current article covers the essentials and important points regarding the histopathologic evaluation of NTs. Using the morphologic criteria described herein, the INPC is proposing the International Neuroblastoma Pathology Classification. It is reported in a companion article in this issue (Cancer 1999;86:363-71).

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Primitive neuroectodermal tumours: anatomic location, extent of surgery, and outcome.

C D Kimber, L Spitz, T Michalski … (1997)

Primitive neuroectodermal tumours (PNET) are rare tumours sharing similar histology, immunohistochemistry, and cytogenetics to Ewing's sarcomas. The aim of the study was to document the effect of site of origin and the completeness of surgical excision on the outcome in infants and children with PNET. All patients (n = 26) with PNET treated in our hospital during the last 6 years were included in the study. The diagnosis was based on the histopathologic findings and a positive MIC2 antibody test. The tumours were classified according to the Intergroup Rhabdomyosarcoma Study III, and were treated according to the UKEESG protocol for Ewing's Sarcoma. Complete remission (CR) was achieved in 52% of the patients completing their chemotherapy. Overall, survival was 42% (11 of 26). Tumours from the paraspinal and scapula areas responded relatively well (CR, 83%), whereas abdominal PNET did not respond to treatment. Tumours arising from the head, neck, or chest had an intermediate prognosis (37% survival). Three of the 10 patients who had a complete excision of the tumour died of progressive disease. Complete surgical excision reduced the risk of local recurrence but did not prevent metastatic spread. These data indicate that PNET have a worse prognosis and different favourable sites than other sarcomas. New treatment strategies are necessary for these rare tumours.

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Journal

Journal ID (nlm-ta): Case Rep Otolaryngol

Journal ID (iso-abbrev): Case Rep Otolaryngol

Journal ID (publisher-id): CRIOT

Title: Case Reports in Otolaryngology

Publisher: Hindawi Publishing Corporation

ISSN (Print): 2090-6765

ISSN (Electronic): 2090-6773

Publication date (Print): 2016

Publication date (Electronic): 14 November 2016

Volume: 2016

Electronic Location Identifier: 1454932

Affiliations

Department of Otorhinolaryngology, Baskent University Faculty of Medicine, 06500 Ankara, Turkey

Author notes

*Ozan Erol: ozzy.erol@ 123456gmail.com

Academic Editor: Abrão Rapoport

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Ozan Erol http://orcid.org/0000-0002-4209-9403

Article

DOI: 10.1155/2016/1454932

PMC ID: 5124665

PubMed ID: 27965907

SO-VID: 2fe13946-e341-4fcc-8f6c-85817e64629e

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This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

A Rare Tumor in the Cervical Sympathetic Trunk: Ganglioneuroblastoma

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Most cited references 10

The International Neuroblastoma Risk Group (INRG) classification system: an INRG Task Force report.

Terminology and morphologic criteria of neuroblastic tumors: recommendations by the International Neuroblastoma Pathology Committee.

Primitive neuroectodermal tumours: anatomic location, extent of surgery, and outcome.

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