Figure 1 - uploaded by Cecilia Schweblin
Content may be subject to copyright.
and 2: Intermixed ganglioneuroblastoma in a 10-year-old boy: MRI performed to complete the assessment of an adrenal incidentaloma discovered on ultrasound.
Source publication
Neuroblastomas are common tumors in children. However, in the same tumor spectrum, intermixed ganglioneuroblastomas are much rarer. In our case report, we present a 10-year-old boy with an asymptomatic adrenal intermixed ganglioneuroblastoma discovered incidentally. He was treated with total adrenalectomy and had no recurrence of the disease.
Context in source publication
Citations
... There are two approaches to establishing the diagnosis of neuroblastic tumors: either by histological examination of the main tumor or by finding both tumor cells during a puncture biopsy of the bone marrow and a rise in catecholamines or urine catecholaminergic metabolites. 7 Due to their wide range of differentiation in terms of morphology, small biopsy samples brought on by the rising use of core needle biopsies (often image-guided), or FNACs, and morphological overlap with other mesenchymal tumors, neuroblastic tumors present significant diagnostic challenges. 8 The IPNC defines two subcategories of GNB: the intermixed subtype (GNBi), which is Schwannian stroma rich, and the nodular subtype (GNBn), which could be composite, Schwannian stroma rich, or Schwannian stroma poor. ...
... It is used to exclude non-sarcomatous entities and determine which line of mesenchymal differentiation (if any) the tumor cells display to diagnose small round cell tumors. 1,7,8,11 In neuroblastic tumors, immunohistochemical staining with antibodies such as neurofilament protein (NFP), synaptophysin, chromogranin, and S-100 is typically positive. Compared to neuroblastic tumors, alveolar rhabdomyosarcomas have more nuclear pleomorphism, a greater amount of cytoplasm, and are positive for muscle-specific markers such as desmin, myogenin, and MyoD. ...
This case report highlights the Importance of recognising and accurately diagnosing ganglioneuroblastoma, an uncommon variant of neuroblastic tumours 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 utilise radiological examinations to promptly Identify and treat these tumours.
... According to He et al., most GNBI tumors are localized and present no MYCN amplification, which correlates with higher survival rates [37]. The assessment of tumor progression requires the use of imaging modalities and laboratory tests [42]. Since NTs frequently cause an irregularity in the production, secretion, or catabolism of catecholamines, laboratory testing includes a measurement of catecholamines and their metabolites in blood and urine. ...
... Often, lymphadenopathy is seen [45]. The primary tumor assessment is conducted using CT and/or magnetic resonance imaging (MRI) [42]. On CECT scans, the characteristics of GNBs display diversity, spanning from well-defined, oblong paravertebral masses with homogeneous enhancement to irregular, cystic, hemorrhagic, or locally invasive masses [33]. ...
Nonspecific gastrointestinal symptoms remain a problem for pediatricians because, out of a thousand trivial cases, there are rare diseases that require in-depth diagnostics and extensive knowledge to identify them. These complaints may be caused by a neoplastic process. We present the case of a 5-year-old boy whose diagnostic pathway lasted about 3 months. He was admitted to hospital due to severe abdominal pain. Physical examination revealed a bloated, hard, and painful abdomen. In the standing X-ray, the features of intestinal obstruction were visualized. An ultrasound examination showed a possible malignant lesion in the location of the left adrenal gland. After the surgical removal of the pathological mass and histopathological examination, the diagnosis of ganglioneuroblastoma intermixed was made. This tumor, along with neuroblastoma, ganglioneuroma, and ganglioneuroblastoma nodular, belongs to neuroblastic tumors (NTs), which originate from primitive cells of the sympathetic nervous system. NTs are quite rare, but they are still the majority of extracranial solid tumors in children, and their symptoms often appear relatively late when the neoplastic process is already advanced. The purpose of this review is to present current information about ganglioneuroblastoma, with a special emphasis on nonspecific gastrointestinal symptoms as first sign of this tumor and its diagnostics.
