|Year : 2018 | Volume
| Issue : 3 | Page : 210-212
Watery diarrhea: An unusual presentation of adrenal ganglioneuroma in a child
Pramila Sharma1, Rahul Gupta1, Arvind K Shukla1, Ranjana Solanki2, Aarti Mittal2
1 Department of Pediatric Surgery, SMS Medical College, Jaipur, Rajasthan, India
2 Department of Pathology, SMS Medical College, Jaipur, Rajasthan, India
|Date of Web Publication||17-Sep-2018|
Dr. Rahul Gupta
Department of Pediatric Surgery, SMS Medical College, Jaipur, Rajasthan
Source of Support: None, Conflict of Interest: None
A 2-year-old male child presented with upper respiratory tract infection along with history of recurrent watery diarrhea for the last 9 months, which was being treated as lactose intolerance. Computed tomography revealed a 5 cm right adrenal mass; complete excision was performed. Histopathology revealed a mature adrenal ganglioneuroma. The patient is no longer suffering from frequent diarrhea and is doing fine on 1-year follow-up. In any pediatric patient with adrenal tumor presenting with watery diarrhea, vasoactive intestinal polypeptide-producing ganglioneuroma must be kept as differential diagnosis.
Keywords: Adrenal, ganglioneuroma, pediatric, vasoactive intestinal polypeptide, watery diarrhea
|How to cite this article:|
Sharma P, Gupta R, Shukla AK, Solanki R, Mittal A. Watery diarrhea: An unusual presentation of adrenal ganglioneuroma in a child. J NTR Univ Health Sci 2018;7:210-2
|How to cite this URL:|
Sharma P, Gupta R, Shukla AK, Solanki R, Mittal A. Watery diarrhea: An unusual presentation of adrenal ganglioneuroma in a child. J NTR Univ Health Sci [serial online] 2018 [cited 2019 May 20];7:210-2. Available from: http://www.jdrntruhs.org/text.asp?2018/7/3/210/241290
| Introduction|| |
Ganglioneuromas are uncommon benign tumors arising from the neural crest cells. Most of them are posterior mediastinal or retroperitoneal in origin, while adrenal ganglioneuroma is rare. Ganglioneuromas are usually asymptomatic and detected incidentally during routine evaluation of unrelated illnesses. We report an unusual case of a 2-year-old child with mature right adrenal ganglioneuroma presenting with recurrent watery diarrhea.
| Case Report|| |
A 2-year-old male child presented with fever and cough for 1 week along with history of recurrent watery diarrhea for the last 9 months. The patient was being treated as lactose intolerance (milk allergy); celiac disease was ruled out. There was no history of crying episodes, sweating, breathlessness, or weight changes. On examination, child was hemodynamically stable, mildly dehydrated, and febrile (99.5°F); abdomen was soft and mildly distended. Routine abdominal ultrasonography revealed 4.9 × 3.6 cm mixed echogenic retroperitoneal mass adjacent to right kidney. Contrast-enhanced computed tomography (CT) showed 4.9 × 3.4 × 3.6 cm right adrenal mass [Figure 1]. Laboratory values revealed leukocytosis (total leukocyte count: 12,700/mm 3) and raised platelet counts (768,000/mm 3). Renal and liver functions, serum electrolytes, and coagulation profile were normal. Thyroid profile was normal (thyroid stimulating hormone: 1.2 mIU/L, T4: 7.5 μg/dL). Lactate dehydrogenase levels were raised (647 U/L). Serum alfa-fetoprotein, serum B-hCG levels, 24 h total urinary catecholamine levels (35 μg/24 h), free cortisol excretion levels (25 μg/24 h), serum testosterone, and mineralocorticoids were normal.
|Figure 1: Contrast-enhanced computed tomography abdomen coronal and transverse images showing 4.9 × 3.4 × 3.6 cm mass (G) with intratumoral calcification (white arrow) present in the right suprarenal region closely abutting the adjacent right lobe of liver (L), anterior margin of right kidney (K), and inferior vena cava. Computed tomography urography image showing normally functioning kidneys; right adrenal not separately visualized [GB = gall bladder, G = ganglioneuroma]|
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The diagnoses of adrenal tumor incidentaloma and neuroblastoma were considered. Preoperative optimization was performed; symptoms of fever and cough improved, but watery diarrhea did not respond. Exploration was done with right subcostal incision and unilateral right adrenalectomy was performed without complications [Figure 2]. Intraoperatively, the blood pressure remained stable. Histopathological evaluation revealed a mature adrenal ganglioneuroma [Figure 3]. In serial sections, there were no evidence of composite tumor or neuroblastoma components; surgical margins were negative for tumor. The patient is no longer suffering from frequent watery diarrhea and is doing well on 1-year follow-up.
|Figure 2: Clinical photographs showing right kidney (black arrow) with tumor bed (white arrow) in the right suprarenal region after excision of the tumor (a), gross specimen of an encapsulated, white, firm, grossly homogenous lobular mass (b), cut section with homogenous brownish appearance of tumor (c)|
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|Figure 3: Hematoxylin and eosin stained sections of the adrenal mass (×40 magnifification) showing (a) mature ganglion cells (inside the white circle) in Schwannian cell-dominant stroma and adrenocortical cells (green arrow) with absence of distinct hemorrhagic nodules. Inset images (×100) showing mature ganglion cells (yellow arrow, b) and adrenocortical cells (green arrow, c); capsule of the adrenal gland (×40, d)|
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| Discussion|| |
Neuroblastoma is the most common adrenal tumor in children <4 years. Also adrenal gland is the most common site (35%) for neuroblastoma, but it is a rare site (21%) for ganglioneuroma with approximately 500 cases reported in the literature till date.,
Ganglioneuromas arise from the sympathetic chains extending from the base of the skull to the neck, mediastinum, retroperitoneum, and adrenal glands. They can arise de novo or from maturation of ganglioneuroblastoma, and sometimes spontaneous regression from neuroblastoma (chemotherapy induced). They comprise about 0–6% of incidentalomas and rarely seen in pediatric patients., It has a slow growth pattern; present with nonspecific symptoms due to compression effects on the adjacent viscera. It has propensity to encase vessels., Less than one-third of patients of ganglioneuromas are hormonally active with elevated plasma and urinary catecholamine levels but rarely symptoms of catecholamine excess. This is in contrast to neuroblastomas which secrete catecholamines in approximately 90% cases, mainly homovanillic acid and dopamine and present with specific signs and symptoms. Symptoms of pain abdomen, vomiting, hypertension, and virilization have been reported in hormonally active tumors. Presence of recurrent watery diarrhea can be explained by a functioning ganglioneuroma in our case.,, This association was first described by Hawfield and Daisley. Vasoactive intestinal peptide (VIP) is commonly expressed in ganglion cells., Raised plasma concentrations of VIP associated with secretory diarrhea has been described in ganglioneuroblastoma (55.6%), ganglioneuromas (31.7%), and neuroblastomas (7.9%). VIP-producing tumors result in a syndrome of nontreatable watery diarrhea, hypokalemia, and achlorhydria. VIP acts by causing splanchnic vasodilatation, stimulation of adenylate cyclase activity, and secretion of intestinal mucosa. VIP level was not done in our patient. However, presentation with recurrent watery diarrhea for 9 months and its abrupt cessation after tumor resection is highly suggestive of a VIP-producing ganglioneuroma, in absence of gastrointestinal infections and malabsorption states. Adrenal adenoma, which is the most common adrenal incidentaloma, was not considered in our case, as it is associated with high levels of glucocorticoid and aldosterone.
Features suggestive of ganglioneuroma on CT are presence of calcification and absence of vascular involvement. Magnetic resonance imaging is the most useful and sensitive imaging modality for the diagnosis of adrenal mass (not performed in our case due to resource limitations).131 I-metaiodobenzylguanidine scan cannot rule out ganglioneuromas. Adrenal ganglioneuroma is difficult to differentiate from malignant type using radiological armamentarium preoperatively.
Adrenal tumors >3 cm in diameter are likely functional, while >5 cm with heterogeneity and calcification suggest malignancy. In children, lesions >3 cm and suspicious for malignancy should be excised. Surgical excision with meticulous histopathological evaluation is the only investigation to differentiate ganglioneuroma from other entities. Prognosis is excellent with complete surgical excision. Immunohistochemical analysis of the tumor specimen for VIP should ideally be performed for confirmation. Finally, any patient with adrenal tumor presenting with watery diarrhea, VIP-producing ganglioneuroma must be kept as differential diagnosis.
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.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Koch CA, Brouwers FM, Rosenblatt K, Burman KD, Davis MM, Vortmeyer AO, et al
. Adrenal ganglioneuroma in a patient presenting with severe hypertension and diarrhea. Endocr Relat Cancer 2003;10:99-107.
Celik V, Unal G, Ozgultekin R, Goksel S, Unal H, Cercel A. Adrenal ganglioneuroma. Br J Surg 1996;83:263.
Geoerger B, Hero B, Harms D, Grebe J, Scheidhauer K, Berthold F. Metabolic activity and clinical features of primary ganglioneuromas. Cancer 2001;91:1905-13.
Alqoaer KI, Albalawi M, Alnoaiji MS, Khan A. Mediastinal ganglioneuroma presented as chronic diarrhea in a young Saudi girl. Int J Clin Med 2014;5:420-4.
Zhang WQ, Liu JF, Zhao J, Zhao SY, Xue Y. Tumor with watery diarrhoea, hypokalaemia in a 3-year-old girl. Eur J Pediatr 2009;168:859-62.
Adas M, Koc B, Adas G, Ozulker F, Aydin T. Ganglioneuroma presenting as an adrenal incidentaloma: A case report. J Med Case Rep 2014;8:131.
Staren ED. Prinz RA. Selection of patients with adrenal incidentaloma for operation. Surg Clin North Am 1995;75:499-509.
[Figure 1], [Figure 2], [Figure 3]