|Year : 2001 | Volume
| Issue : 2 | Page : 159-161
Functioning ganglioneuroma of the adrenal gland - case report and review of literature
Anand Kumar Mishra, Amit Agrawal, Dilip K Kar, KK Prasad, Gaurav Agrawal, Priti Dabadgaonkar, Lily Pal, SK Mishra
Departments of Endocrine Surgery, Endocrinology and Pathology, Sanjay Gandhi Institute of Medical Sciences, Lucknow, India
Department of Endocrine Surgery, Sanjay Gandhi Institute of Medical Sciences, Raihareli Road, Lucknow - 226 014
Source of Support: None, Conflict of Interest: None
| Abstract|| |
A ganglioneuroma of adrenal gland in a 34-year-old female presenting as adrenal incidentaloma is described. The significance of this finding is discussed, and literature in English is reviewed to highlight the importance of hormonal evaluation of an incidentaloma.
Keywords: Adrenal Gland; Ganglioneuroma: Incidentaloma.
|How to cite this article:|
Mishra AK, Agrawal A, Kar DK, Prasad K K, Agrawal G, Dabadgaonkar P, Pal L, Mishra S K. Functioning ganglioneuroma of the adrenal gland - case report and review of literature. Indian J Urol 2001;17:159-61
|How to cite this URL:|
Mishra AK, Agrawal A, Kar DK, Prasad K K, Agrawal G, Dabadgaonkar P, Pal L, Mishra S K. Functioning ganglioneuroma of the adrenal gland - case report and review of literature. Indian J Urol [serial online] 2001 [cited 2022 Jan 21];17:159-61. Available from: https://www.indianjurol.com/text.asp?2001/17/2/159/21048
| Introduction|| |
Adrenal gland tumors are rare. However, since the development of the CT-scanner adrenal tumors are much more commonly identified. Many of these lesions are asymptomatic and picked up during investigation of an unrelated problem. They occur with a reported prevalence of 0.3-5% on all CT scans.  Ganglioneuromas are a rare cause of adrenal incidentaloma. They are, however, benign, differentiated tumors originating from primordial neural crest cells and situated at one end of spectrum that includes neuroblastoma and ganglioneuroblastoma.' - The presence of these tumors in the adrenal medulla is less common but presents diagnostic difficulty.  As with neuroblastoma and ganglioneuroblastoma, ganglioneuroma may also secrete catecholamines, and some patients have diarrhea, hypertension or Cushing's syndrome. , The dilemma in the work up of incidentaloma is how far to investigate regarding the functional status of the mass.
| Case Report|| |
A 34-year-old married woman presented to her gynecologist with symptoms of oligomenorrhea. She was nonhypertensive and her physical examination was unremarkable. Subsequent ultrasound imaging revealed a 7.5 x 4.7 cm right supra-renal mass with multiple calcification. The patient was subsequently referred to our department for evaluation and management. Clinical examination revealed no evidence of a likely primary tumor elsewhere. In particular, both breast and axilla were unremarkable.
Hemogram was within normal limits. Serum K+, overnight dexamethasone suppression test were within normal limits. The 24-hour urinary metanephrine was raised and twice the normal value (n = 2.03 mg / 24 hours; laboratory normal < 0.50mg / 24 hours). CT scan confirmed a 7.5 x 6.5 cm right adrenal mass [Figure - 1]. Diagnosis of pheochromocytoma presenting as adrenal incidentaloma was made. Preoperatively alpha blockade was achieved by 8 mg/24 hours of prazopress. Patient also needed 20 mg/24 hours of beta-blocker for maintenance of heart rate.
Using a right subcostal transperitoneal approach the right adrenal gland was mobilized off the cava, diaphragm and kidney and excised. Intraoperatively the patient was hemodynamically stable and did not require any anti-hypertensive. Postoperative period was uneventful. She was discharged on the 9 th postoperative day.
Histology revealed an encapsulated mass compressing the cortical tissue at periphery. Tumor tissue comprised of interlacing bundles of spindloid cells with elongated, bipolar spiral nuclei and fibrillary cytoplasm in the background of rich stroma. Interspread among these cells are seen clusters of mature ganglion cells having abundant cytoplasm and round to oval nuclei with prominent nucleoli [Figure - 2]. The finding was consistent with ganglioneuroma of the right adrenal medulla.
| Discussion|| |
Prior to 1974, 178 non-hypersecretory adrenal cortical tumors had been reported in the world literature.  The number of such masses has increased substantially because of serendipitous detection of much smaller lesions by the new generation CT systems. The reported prevalence is 0.35 to 4.36% of patients imaged with CT for reasons other than suspected adrenal pathology.  If patients with concurrent extra-adrenal malignancy, adrenal hemorrhage and known hypersecretion are excluded, the prevalence of true adrenal incidentalomas varies from 0.6 to 1.3% of all abdominal CT scan reviewed.  Benign adrenal adenomas are the most common cause and tumors metastatic to the adrenal the second most common cause.  The most common tumors metastatic to the adrenal are from the lung, breast, colon, stomach, kidney and melanoma and lymphoma.  The incidence of ganglioneuromas is unknown but they are frequently associated with Von Recklinghausen's disease. ,
Establishing the nature of incidentally discovered adrenal masses has been a major concern because, once they are discovered, their nature must be defined in order to exclude a metastatic lesion and, to a lesser degree, a pheochromocytoma or a primary adrenal carcinoma. CT and MRI can definitely characterize only if simple cysts, myelolipoma, obvious malignant invasion is present. Biochemical screening for hormonal excess is essential regardless of a nonsuggestive complete history and physical examination. Published reports indicate that 80% or more of patients with adrenal incidentaloma that are `nonhypersecretory' may in fact demonstrate subtle evidence of excess hormone production when evaluated by more sensitive biochemical testing procedures.  Recommended biochemical screening tests for incidentally discovered adrenal masses in all patients unless specified includes serum catecholamines or 24-hour urinary catecholamines or 24-hour urinary metanephrines, over night dexamethasone suppression test (ONDST) and serum potassium. Besides these, serum dehydroepiandrosterone sulfate in virilized females and boys with precocious sexual development and serum estradiol in feminized men are recommended.  Percutaneous adrenal biopsy plays only a limited role in evaluation of adrenal lesions. Only in presence of a suspected metastasis is percutaneous biopsy warranted. If fine needle aspiration is considered it is imperative to exclude the presence of a pheochromocytoma. Fine needle aspiration and biopsy of pheochromocytoma has been reported to precipitate a hypertensive crisis, retroperitoneal hemorrhage and death.  An adrenal incidentaloma found to be functioning on screening should undergo surgery.  For non-functional tumors, attempts have been made to classify them into either benign or malignant lesion as per radiological criteria. Currently the most effective determination of a lesion's risk of malignancy is its size. Tumors < 3 cm in diameter carry only a small risk of malignancy and can be observed.  In contrast, 90% of all adreno-cortical carcinomas reported in six series were >6 cm in diameter.' Current recommendation is to remove all adrenal lesions >6 cm in diameter to avoid unnecessarily observing a malignancy. The management of non-hypersecretory adrenal lesions ranging from 3 to 6 cm is controversial. Lesions >3 cm in diameter and suspicious for malignancy, particularly in younger patients, should be excised. ,
Ganglioneuromas of the adrenal are rare tumors arising from nerve cells present within the adrenal medulla.  The majority of ganglioneuromas are identified within the sympathetic nervous plexus. Only 15-30% of all reported ganglioneuromas arise in the adrenal gland. , Most ganglioneuromas are located in the posterior mediastinum and retroperitoneum. Ganglioneuromas can occur at any age although 75% occur before the age of 20.  Such patients often present with paraneoplastic symptoms and such as diarrhea and hypertension. , Histologically they are composed of ganglion cells and spindle cells with a variable composition of collagen. Other type of neural adrenal tumors include neuroblastomas and ganglioneuroblastomas. Neuroblastomas arise from embryonic sympathetic neuroblasts and are always encountered in children < 4 years of age. Similarly, ganglioneuroblastomas are most commonly reported in children < 10 years of age. Prognosis is stage-dependent. Clinically ganglioneuromas can expand locally and can cause symptoms from focal pressure.  Most authors believe that ganglioneuromas are clinically benign. , However, malignant transformation of adrenal ganglioneuromas and tumors of mixed ganglioneuroma and pheochromocytoma have also been reported. Furthermore, malignant transformation into malignant peripheral nerve sheath tumors instead of neuroblastomas has been reported.  In view of above. their surgical removal will help in avoiding these two potential complications. In our patient, multiple sections were studied and no areas of neuroblastoma or other malignant tumor were observed; the ganglioneuroma appear to have occurred de novo.
This case highlights the potentially unusual nature of adrenal lesions. Ganglioneuroma can develop spontaneously and asymptornatically and are diagnosed incidentally. There is therefore a need for hormonal evaluation consisting serum K+, ONDST and 24-hour urinary/serum catecholamines or metanephrines.
| Acknowledgement|| |
We are thankful to Dr. Aneesh Srivastava, Assistant Professor, Department of Urology, SGPGIMS for critical review of the manuscript.
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[Figure - 1], [Figure - 2]