Characteristics and management of ganglioneuroma and ganglioneuroblastoma‐intermixed in children and adolescents

Alexander, Natasha; Sullivan, Katie; Shaikh, Furqan; Irwin, Meredith S.

doi:10.1002/pbc.26964

Cited by 31 publications

(35 citation statements)

References 33 publications

(71 reference statements)

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“…Adult GNB tumors >8 cm in diameter arising from the retrospective cavity tend to metastasize to other distant organs . The size of the lesion in the present case (13 cm) was large compared to those in other reports of mediastinal GNB, and therefore the risk of recurrence in our case may have been increased at the time of diagnosis. Therefore, cases of adolescent/adult GNB should be followed‐up carefully even after complete resection.…”

Section: Discussioncontrasting

confidence: 43%

“…Here, we present the clinical course of mediastinal GNB discovered in a 17‐year‐old male adolescent. As GNBs occur almost exclusively in the pediatric population, adult onset GNB is uncommon and the mediastinum as the primary site is a further rare clinical manifestation . These points are noteworthy in the present case.…”

Section: Discussionmentioning

confidence: 55%

“…Early and radical surgery is the best treatment available for mediastinal GNBs . The prognosis is generally favorable with a two‐ and five‐year survival rates of 92% and 88%, respectively, reported in a large series .…”

Section: Discussionmentioning

confidence: 99%

“…In addition, the most common sites of origin are the adrenal medulla (about 35%), extraadrenal retroperitoneum (30%–35%), and posterior mediastinum (20%) . Although cases of adolescent or adult onset GNB have been reported in the literature, they are extremely rare . With regard to mediastinal GNBs, less than 50 cases of adolescent or adult mediastinal GNB have been reported to date .…”

Section: Introductionmentioning

confidence: 99%

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Posterior mediastinal ganglioneuroblastoma in an adolescent: A case report and review

et al. 2019

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Ganglioneuroblastoma is an uncommon malignant tumor of the sympathetic nervous system, which is considered a disease of children with the majority of cases in patients less than four years old and it rarely occurs in adults. We encountered a very unusual case of a posterior mediastinal ganglioneuroblastoma that developed in a 17-year-old male adolescent who underwent successful excision of the mediastinal mass and remained stable postoperatively. However, he developed lumbago one year after the surgery. Radiographic findings revealed osteolytic lesions in the lumbar vertebra and histological analysis confirmed bone metastasis of ganglioneuroblastoma. Here, we report the clinical course and present a review of the literature regarding adolescent and adult onset mediastinal ganglioneuroblastoma.

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Section: Discussioncontrasting

confidence: 43%

Section: Discussionmentioning

confidence: 55%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Posterior mediastinal ganglioneuroblastoma in an adolescent: A case report and review

et al. 2019

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“…The pathogenesis of GN is still unclear. Most GNs are primary, but it has been reported that some GNs also arise after treatment for neuroblastoma or as a result of spontaneous neuroblastoma differentiation (10). GNs, just like PCCs, originate from neural crest cells in the neuroectoderm.…”

Section: Discussionmentioning

confidence: 99%

A Novel Phenotype of Germline Pathogenic Variants in MAX: Concurrence of Pheochromocytoma and Ganglioneuroma in a Chinese Family and Literature Review

Chang

et al. 2020

Front. Endocrinol.

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Background: MYC associated factor X (MAX) is a tumor suppressor gene and has been identified as one of the pathogenic genes of hereditary pheochromocytoma (PCC). To date, there have been no reports of ganglioneuroma (GN) with MAX variants. Case Presentation: The proband was a 45-years-old Chinese female with paroxysmal hypertension and palpitations who had undergone adrenalectomy for PCC 14 years ago. Her plasma free normetanephrine and 24-h urinary norepinephrine excretion were significantly increased, and abdominal computed tomography (CT) revealed an irregular mass in the left adrenal region, suggesting a recurrence of PCC. The mass was surgically removed and pathologically diagnosed as PCC with lymph node metastasis. The proband's son suffered from paroxysmal hypertension and palpitations. His plasma free metanephrine levels were normal. CT revealed a mass in the right adrenal. The tumor was surgically removed, and the pathological diagnosis was GN. Genetic testing of peripheral blood DNA revealed that the proband and her son had germline pathogenic MAX variant c.C97T, p.Arg33Ter, while proband's parents did not have MAX variants. Tumor DNA sequencing showed the same MAX variant (c.C97T, p.Arg33Ter) in PCC of the proband and GN of her son, both with retention of heterozygosity. Immunohistochemistry demonstrated loss of MAX protein expression in most tumor cells in PCC of the proband and some Schwannian cells in GN of the proband's son. Conclusion: We report a family with a new clinical phenotype of germline pathogenic variants in MAX who developed both PCC and GN. Germline pathogenic variants in MAX may contribute to the development of GN. Our findings suggest that it is not just paternally inherited MAX variants that can cause tumors.

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A needle in the haystack: An unusual case presentation of ganglioneuroblastoma at a tertiary care center in Coastal Karnataka

Radhakrishnan,

Rao,

Lashkari

et al. 2023

Clinical Case Reports

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Key Clinical MessageThis case report highlights the importance of recognizing and accurately diagnosing ganglioneuroblastoma, an uncommon variant of neuroblastic tumors in children. Ganglioneuroblastomas have diverse clinical and morphological presentations, and histopathological examination is paramount in guiding treatment decisions, especially in cases with ambiguous symptoms. Early detection is crucial, as the prognosis varies significantly based on the subtype and the presence of metastatic disease. Clinicians should maintain a high index of suspicion and utilize radiological examinations to promptly identify and treat these tumors.AbstractChildren are frequently affected by neuroblastic tumors, which grow from the sympathoadrenal lineage of the neural crest during its development. However, intermixed ganglioneuroblastomas are far less common within the same tumor spectrum, the diagnosis of which could become challenging amidst an unusual presentation. In our case report, we present a 4‐year‐old boy who had complaints of fever and difficulty in walking, with a supra‐renal mass on ultrasound, which was diagnosed as ganglioneuroblastoma‐intermixed type on histopathological examination. This report aims to contribute to the understanding of the diverse clinical and morphological spectrum of ganglioneuroblastomas and the importance of multidisciplinary collaboration and histopathological examination to enhance decision‐making in such ambiguous scenarios.

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Characteristics and management of ganglioneuroma and ganglioneuroblastoma‐intermixed in children and adolescents

Abstract: GN and GNB-I have a slow growth rate and resection can be associated with significant morbidity. Watch and wait approaches should be considered for some GN and GNB-I.

Cited by 31 publications

References 33 publications

Posterior mediastinal ganglioneuroblastoma in an adolescent: A case report and review

Posterior mediastinal ganglioneuroblastoma in an adolescent: A case report and review

A Novel Phenotype of Germline Pathogenic Variants in MAX: Concurrence of Pheochromocytoma and Ganglioneuroma in a Chinese Family and Literature Review

A needle in the haystack: An unusual case presentation of ganglioneuroblastoma at a tertiary care center in Coastal Karnataka

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