Neuroblastoma arises from primitive cells of the sympathetic nervous system and consists of three histologic subtypes: neuroblastoma, ganglioneuroblastoma (GNB), and ganglioneuroma. [1,2]These three tumors vary in malignancy, with neuroblastoma being the most malignant of the three and the most common extracranial solid tumor in childhood. [3,4]Ganglion neuromas are classified as benign tumors, whereas GNBs show a moderate malignant potential. [3]Neuroblastic tumors can occur anywhere in the sympathetic nervous system, but most occur in the adrenal glands [1]Cases in adolescents and adults are very rare, but have been reported in the literature. [1,5-7].
GNBs contain both mature ganglion cells and immature neuroblasts and are composed of two subgroups: nodular GNBs (poor stroma) and mixed GNBs (rich stroma). [1]The majority of GNB cases occur by age 10 years, the median diagnosis is 22 months, and fewer than 50 adult GNB cases have been reported in the literature. [8,9]To the authors’ knowledge, only 21 cases of adrenal GNB in adults have been reported. Here, we present a literature review examining symptoms, type of treatment, adult age and sex, and the presence of tumor metastases and calcifications from these 21 cases of her.
Inclusion criteria for our study consisted of all cases in the literature reporting adrenal GNB in adults (18 years and older). A search of this type of article on PubMed and Google Scholar found 21 cases.A literature review was conducted examining patient presentation, type of treatment, age and sex, and the presence of tumor metastases and calcifications from these 21 cases (Table 1). 1).
Of the 21 cases in the literature review, 12 were male and 9 were female. The age group most affected was He 20 to He 29, with 11 patients within this range. He had only two patients over the age of 60, one male and his other female. The average age of onset was approximately 37.2 years, and excluding cases of unknown age, the average age was 37.5 years for males and 36.7 years for females. The patient was asymptomatic in his 6 cases, and the tumor was discovered incidentally on imaging studies. Abdominal pain/discomfort, flank pain, epigastric pain, low back pain, low back pain, hematuria, fatigue, dysuria, diarrhea, polyuria, recurrent headache, vomiting, hypertension, and nausea were all reported symptoms. Abdominal pain/discomfort was the most common symptom reported. Almost one-third of patients were asymptomatic, so the prevalence of adrenal GNB in adults may be higher in the general population than reported in the literature. Hypertension was reported in two cases, and tumor resection corrected hypertension in both cases. [10,16]Surgical removal of GNB was performed in 19 of 21 cases and is therefore the most common therapeutic approach. In addition, of his two cases who did not receive curative treatment, one did not receive chemotherapy, and he died 3 months after the first imaging study in which an adrenal mass was identified. [22]Two reported surgical removal combined with radiotherapy and chemotherapy, and one reported using OK-432 and irradiation of the surgical site plus systemic vincristine and cyclophosphamide therapy. [25]Metastasis was present in 9 cases (6 males, 6 females) and was undetermined in 1 case, with a prevalence at diagnosis of 42.9%. Five of nine patients with metastasis are in the age group of 20 to 29 years, and the prevalence of metastasis in the age group of 20 to 29 years is 45.5%, which is higher than the overall prevalence in this population. Study bigger than. Locations of metastasis included liver, lymph nodes, bone/bone marrow, and para-aortic nodules. Lymph node metastasis was the most common, occurring in 4 of 9 cases with metastasis. Calcification was present in 6 cases and unknown in 13 cases. Most of the cases with 5 of 6 calcifications were seen in the population aged 20-29 years and only 1 case with metastases, especially the presence of lymph nodes. No calcifications were seen in patients aged 35 years or older, otherwise there were no comments.
The results of the literature review, including patient age, type of treatment, and presentation, are compatible with conclusions drawn from previous literature reviews conducted on adult-onset adrenal GNB cases. [1,9,12]However, these literature reviews concluded that adult-onset adrenal GNB was found in men and was greater than our findings. In the current review, tumors occurred in 43% of women and 57% of men, but 24% of women and 76% of men. [1]36.4% of women and 63.6% of men [9]37.5% of women and 62.5% of men [12] In three previous literature reviews. We believe these differences are due to errors by the authors of previous literature reviews. We recently showed that inaccurate information was presented in a literature review conducted by Bolzacchini et al. [1]leading to misleading conclusions about the gender in which tumors are most common [29]Errors were also found in Table 3 of the literature review conducted by Benedini et al. [12] When they referred to the adult-onset adrenal GNB case by Fujiwara et al. Benedini et al.List patients for this case [10] As men when they were women. In a literature review conducted by Vassallo et al. [9], they noted that 22 cases of adult-onset adrenal GNB were reported in the literature, 14 occurring in men. However, we did not cite all 22 cases. As a result, we were unable to verify our findings. Because this tumor type is so rare, these minor errors made in at least two previous literature reviews have impacted the statistics for this tumor, and therefore the gender distribution of tumors compared to the current review. A slightly different conclusion was drawn. Thus, our results suggest that while adult-onset adrenal GNB may affect men at slightly higher rates than women, the magnitude at which this occurs may be due to errors made in previous literature. , probably not as high as the current literature concludes. A literature review that resulted in misleading conclusions, which may not even be statistically significant due to the small number of cases reported in the literature.
Because GNB can be symptomatic or asymptomatic, imaging techniques such as computed tomography (CT), ultrasound, and magnetic resonance imaging (MRI) are critical when attempting to diagnose GNB. important toThese imaging modalities help characterize the mass and aid in pretreatment risk stratification [9,30]CT findings of GNB vary from a relatively solid mass to a cyst-like mass containing a few thin strips of solid tissue. [9]On MRI, GNB is usually heterogeneous and contains low signal intensity on T1-weighted images, whereas T2-weighted images usually contain higher signal intensity. [9]Although these imaging techniques play a fundamental role in helping to locate and visualize GNBs that may be present, the three neuroblastomas show striking similarities in imaging. Therefore, a definitive diagnosis cannot be made until histopathological analysis is performed. According to the International Neuroblastoma Pathology Committee, GNB is divided into two groups. GNB mixed (rich stroma) and GNB nodular (poor stroma). [2]GNB admixture is characterized by the presence of a mixture of neuroblasts and ganglion cells, whereas GNB nodules are usually hemorrhagic/necrotic and coexist with stromal-rich or stromal-dominant components. It expresses a stroma-poor/neuroblastic nodule component. [9].
Regardless of age or tumor location, it is important to continue to examine patients regularly after treatment for GNB. This is because most of the chances of recurrence occur within 2 years after surgery. [9,31]Good practice has been reported to see patients every 3 months for the first 2 years and every 6 months after 2 years. [1,9,31]A thorough examination includes blood count, urinary catecholamine synthesis analysis, and imaging of the site where the tumor was originally diagnosed. [1]High levels of urinary catecholamine synthesis have been shown to correlate with relapse [32]hence why it is recommended to assess this marker during a patient’s physical examination.
GNB is a rare subtype of neoplastic tumor that contains both mature ganglion cells and immature neuroblasts. Fewer than 50 cases of adult GNB exist in the literature and only 21 reported cases of her GNB arising in the adrenal glands in adults. A literature review was conducted examining patient presentations, type of treatment, age, and sex from her 21 cases of adult-onset adrenal GNB in the literature. From this review, we conclude that adult-onset adrenal GNB affects men at slightly higher rates than women, but not as much as reported in the current literature by previous literature reviews on this topic, and more at 20 years of age. It was concluded that many will be diagnosed. The -29-year-old patient population, more than any other adult population, may present with varying degrees of symptoms, most treated by surgical excision of the tumor, and may also exhibit metastatic features and calcification. I have.
