Treatment and outcome of Ganglioneuroma and Ganglioneuroblastoma intermixed

Decarolis, Boris; Simon, Thorsten; Krug, Barbara; Leuschner, Ivo; Vokuhl, Christian; Kaatsch, Peter; Schweinitz, Dietrich von; Klingebiel, Thomas; Mueller, Ingo; Schweigerer, Lothar; Berthold, Frank; Hero, Barbara

doi:10.1186/s12885-016-2513-9

Cited by 107 publications

(153 citation statements)

References 30 publications

(43 reference statements)

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“…In addition, DeCarolis et al. reported that only 5 of 55 patients with imaging‐detectable residual GN and GNB‐I tumors on GPOH NB97 and NB 2004 demonstrated evidence of local progression and all 5 went on to have SD following repeat partial resection (N = 3), biopsy (N = 1), or chemotherapy (N = 1) . Importantly, this study also supported a previous report demonstrating no role for cytotoxic therapy for GN and GNB‐I .…”

Section: Discussionsupporting

confidence: 89%

“…Several recent case series of patients with GN have advocated consideration of nonradical surgeries and/or observation approaches rather than complete surgical resection . To our knowledge, this report is the first to describe the natural history and growth over time for unresected GN and GNB‐I tumors.…”

Section: Discussionmentioning

confidence: 91%

“…Importantly, most of the changes involved revision of diagnosis to GNB‐I. In the German registry, there were no reported reclassifications to NB including resection and biopsy specimens from patients who underwent surgery due to progression . Of the 10 patients, 9 in our cohort with increases in growth (maximal diameter) of >10% did not go on to have resection, but were instead followed with surveillance with minimal further growth.…”

Section: Discussionmentioning

confidence: 99%

“…The majority of GNs are diagnosed de novo (primary) but secondary GN may also arise after treatment for NB or as a result of spontaneous differentiation of NB . Primary GN and GNB‐I present at older age (median 5–7 years) than other more aggressive subtypes of pNTs and are usually located in the thorax and abdomen . Patients may be symptomatic, most commonly with pain or respiratory distress, but frequently GN masses are diagnosed incidentally in evaluations for unrelated complaints .…”

Section: Introductionmentioning

confidence: 99%

“…The outcomes for GN and GNB‐I are excellent. Studies report 96–100% overall survival (OS), however, the morbidity from surgical resections is significant due to proximity to the spine, vessels, and organs. Complications, reported in 15–30% of cases, include hemorrhage, Horner's syndrome, intestinal obstruction, and death Often complete resection with negative margins is not possible, but tumor progression is rare in cases with residual disease …”

Section: Introductionmentioning

confidence: 99%

See 4 more Smart Citations

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

Alexander

Sullivan

Shaikh

et al. 2018

Pediatric Blood & Cancer

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Section: Discussionsupporting

confidence: 89%

Section: Discussionmentioning

confidence: 91%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

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

Alexander

Sullivan

Shaikh

et al. 2018

Pediatric Blood & Cancer

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Recurrent ganglioneuroma in PTPN11‐associated Noonan syndrome: A case report and literature review

Morales‐Rosado

Singh

Olson

et al. 2021

American J of Med Genetics Pt A

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Noonan syndrome (NS) is an autosomal dominant condition with variable expressivity most commonly due to a germline pathogenic variant in PTPN11, which encodes the protein tyrosine phosphatase SHP‐2. Gain‐of‐function variants in PTPN11 are known to promote oncogenic behavior in affected tissues. We report the clinical description of a young adult male presenting with relapsing ganglioneuromas, dysmorphic features, cardiac abnormalities, and multiple lentigines, strongly suspicious for NS. Solid tumor testing identified the recurrent pathogenic c.922G>A (p.Asn308Asp) in PTPN11. Proband and parental blood sampling testing confirmed c.922G>A as a de novo germline alteration. Comprehensive literature review of solid tumors specifically associated to PTPN11, indicates that this is the first documentation of ganglioneuroma and its clinical recurrence after resection in conjunction with a genetically confirmed NS diagnosis. The findings in our patient further extend the list of neuroblastic and neural crest‐derived neoplasms associated with this condition.

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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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Treatment and outcome of Ganglioneuroma and Ganglioneuroblastoma intermixed

Cited by 107 publications

References 30 publications

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

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

Recurrent ganglioneuroma in PTPN11‐associated Noonan syndrome: A case report 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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