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| CASE REPORT |
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| Year : 2021 | Volume
: 12
| Issue : 3 | Page : 131-133 |
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Hormone-secreting ganglioneuroma: A rare entity
Aastha Shah, Darshan Thummar, Maitrik Mehta, Dishu Shah, U Suryanarayan, Dinesh Anand
Department of Radiation Oncology, GCRI, Ahmedabad, Gujarat, India
| Date of Submission | 28-Dec-2020 |
| Date of Acceptance | 03-Feb-2021 |
| Date of Web Publication | 04-May-2021 |
Correspondence Address:
Dr. Aastha Shah
Department of Radiation Oncology, GCRI, Ahmedabad, Gujarat
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/jrcr.jrcr_73_20
Ganglioneuromas are slow-growing benign counterparts of neuroblastoma groups along with ganglioneuroblastomas. Herein, we report a rare case of hormone-secreting benign ganglioneuroma in a 4-year-old kid. A 4-year-old male child was referred to our outdoor patient department from the primary health center to evaluate for the persistent complaint of increase in weight and raised blood pressure. On computed tomography (CT) of the abdomen and pelvis, an 6.4 cm × 6.5 cm mass lesion was seen over the right adrenal gland which was compressing segments VI and VII of the liver, and the right hilar region showed a 1.6 cm × 1.2 cm × 0.9 cm enlarged lymph node. The rest of the scan was clear. The patient was therefore started on cyclophosphamide and vincristine, received two cycles, and 4 weeks following it, a diagnostic CT scan of the abdomen, pelvis, and thorax was undertaken which showed a 5.1 cm × 4.5 cm × 6 cm lesion over the right suprarenal region abutting inferior vena cava, inferior surface of the liver, and superior renal surface. Surgical laparotomy followed by right adrenalectomy was done which on pathological evaluation to a surprise showed features suggestive of maturing ganglioneuroma with no evidence of any blastemal components. However, the posterior resection margin turned out to be positive. Radiation was omitted despite the margins being positive. Very few cases of hormone-secreting pediatric ganglioneuroma have been reported in literature and their management requires a meticulous approach.
Keywords: Benign, ganglioneuroma, hormone, radiation, surgery
How to cite this article:
Shah A, Thummar D, Mehta M, Shah D, Suryanarayan U, Anand D. Hormone-secreting ganglioneuroma: A rare entity. J Radiat Cancer Res 2021;12:131-3 |
| Introduction | |  |
Ganglioneuromas are slow-growing benign counterparts of neuroblastoma groups along with ganglioneuroblastoma. Ganglioneuromas comprise the mature spectrum of the neuroblastoma group and thus do not cause significant symptoms till they grow large enough to cause local mass compressing symptoms, and therefore, the outcomes are excellent with surgery alone.[1] The incidence of hormone secretory ganglioneuromas as per literature is very less and as the novel modalities of imaging have come up, they are being increasingly recognized. Herein, we report a rare case of hormone-secreting benign ganglioneuroma in a 4-year-old kid and its management.
| Case Report | | |
A 4-year-old male child was referred to our outdoor patient department from the primary health center to evaluate for the persistent complaint of increase in weight and raised blood pressure. The patient was started on antihypertensive. The patient's family history was unremarkable. Routine blood investigations were unremarkable. A hormonal profile was done which showed raised serum cortisol value. The rest of the other hormones including prolactin, testosterone, growth hormone, luteinizing hormone, follicle-stimulating hormone, and thyroid-stimulating hormone were within normal limits. Magnetic resonance imaging (MRI) of the brain was also done which showed no evidence of any abnormality. On computed tomography (CT) of the abdomen and pelvis, an 6.4 cm × 6.5 cm mass lesion was seen over the right adrenal gland which was compressing segments VI and VII of the liver and the right hilar region showed a 1.6 cm × 1.2 cm × 0.9 cm enlarged lymph node, as shown in the [Figure 1] and [Figure 2] showing the coronal and the axial view of CT scan. The rest of the scan was clear. On ultrasonographic-guided biopsy of the right suprarenal lesion, histopathologic report showed well-differentiated, stromal-rich neuroblastoma with the presence of Schwann cells. Bone scan and bone marrow biopsy were negative. A multidisciplinary tumor board discussion was done, wherein it was decided to commence with cytotoxic neoadjuvant chemotherapy followed by response evaluation. The patient was therefore started on cyclophosphamide and vincristine, received two cycles, and 4 weeks following it, a diagnostic CT scan of the abdomen, pelvis, and thorax was undertaken which showed 5.1 cm × 4.5 cm × 6 cm lesion over right suprarenal region abutting inferior vena cava, inferior surface of the liver, and superior renal surface. Surgical laparotomy followed by right adrenalectomy was done which on pathological evaluation to a surprise showed features suggestive of maturing ganglioneuroma with no evidence of any blastemal components. However, the posterior resection margin turned out to be positive. Immunohistochemical analysis showed markers such as leukocyte common antigen positive and chromogranin negative. Post surgery, hormonal profile was repeated which returned to baseline and the hypertension also got controlled. On reviewing literature intensely, a decision was taken to keep the patient on follow-up. Post surgery, CT scan of the abdomen, pelvis, and thorax was done which showed only postoperative changes, as shown in [Figure 3]. Meta-iodobenzyl guanidine scan was also done which showed no evidence of any disease.[2] Radiation was omitted despite the margins being positive. At present, the patient is on monthly follow-up with clinical examination and ultrasonography of the abdomen and pelvis is done as a part of imaging. | Figure 1: Coronal computed tomography image showing right suprarenal mass
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 | Figure 3: Coronal computed tomography image showing postoperative tumor bed
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| Discussion | | |
Ganglioneuromas form a part of benign tumors in the neuroblastoma group.[3] Neuroblastoma family of tumors has the quirky ability to differentiate and mature, which delineates a family composed of neuroblastoma, ganglioneuroblastoma, and ganglioneuroma. Ganglioneuroma is considered the most benign form, consisting of gangliocytic and mature stromal cells.[4] They arise from sympathetic ganglion cells and complete surgical resection alone is considered to be curative. They are commonly seen in the mediastinum, retroperitoneum, and adrenal gland.[5] Among these, the posterior mediastinum is the most common location. Ganglioneuromas are commonly asymptomatic, but patients can present with symptoms due to local mass effect. MRI is considered the radiological investigation of choice.[6] CT scan shows dot-like calcifications in lesions of ganglioneuroma, but it has not been observed in our case. Ganglioneuromas have a tendency to remain silent for a long time and are often associated with a long-term disease-free survival. A series of cases of presacral ganglioneuromas have also been reported in the literature,[6] but hormone-secreting adrenal ganglioneuromas are extremely rare.[7] It is difficult to identify it on imaging alone so a histological diagnosis is necessary. Considering the benign nature of ganglioneuroma and their spontaneous regressing nature and keeping in mind the detrimental effects of radiation, ganglioneuroma post surgery can be kept on observation despite being secretory in nature. Malignant transformation of these tumors has been rarely reported in literature.[8]
| Conclusion | | |
Very few cases of hormone-secreting pediatric ganglioneuroma have been reported in literature and their management requires a meticulous approach.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Decarolis B, Simon T, Krug B, Leuschner I, Vokuhl C, Kaatsch P, et al. Treatment and outcome of ganglioneuroma and ganglioneuroblastoma intermixed. BMC Cancer 2016;16:542.  |
| 2. | Pirson AS, Krug B, Tuerlinckx D, Lacrosse M, Luyx D, Borght TV. Additional value of I-123 MIBG SPECT in neuroblastoma. Clin Nucl Med 2005;30:100-1. |
| 3. | Leeson MC, Hite M. Ganglioneuroma of the sacrum. Clin Orthop Relat Res 1989:102-5. PMID: 2766597. |
| 4. | Hsiao CC, Huang CC, Sheen JM, Tai MH, Chen CM, Huang LL, et al. Differential expression of delta-like gene and protein in neuroblastoma, ganglioneuroblastoma and ganglioneuroma. Mod Pathol 2005;18:656-62. |
| 5. | Hayes FA, Green AA, Rao BN. Clinical manifestations of ganglioneuroma. Cancer 1989;63:1211-4. |
| 6. | Mounasamy V, Thacker MM, Humble S, Azouz ME, Pitcher JD, Scully SP, et al. Ganglioneuromas of the sacrum-a report of two cases with radiologic-pathologic correlation. Skeletal Radiol 2006;35:117-21. |
| 7. | Erem C, Fidan M, Civan N, Cobanoglu U, Kangul F, Nuhoglu I, Alhan E. Hormone-secreting large adrenal ganglioneuroma in an adult patient: A case report and review of literature. Blood Press 2014;23:64-9. |
| 8. | Califano L, Zupi A, Mangone GM, Long F. Cervical ganglioneuroma: Report of a case. Otolaryngol Head Neck Surg 2001;124:115-6. |
[Figure 1], [Figure 2], [Figure 3]
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