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Ganglioneuroma Mimicking Wilms' Tumor

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Ganglioneuroma Mimicking Wilms' Tumor Journal of Scientific Research and Studies Vol. 3(6), pp. 115-118, June, 2016 ISSN 2375-8791 Copyright © 2016 Author(s) retain the copyright of this article http://www.modernrespub.org/jsrs/index.htm MRRRPPP Case Report Ganglioneuroma mimicking Wilms’ tumor: Pediatric case report Qazi Adil INAM 1* and Abdul MANAN 2 1Department of Urology, Nawaz Sharif Medical College, University of Gujrat, Pakistan. 2Department of Urology and Renal Transplant, Services Hospital/Services Institute of Medical sciences, Lahore, Pakistan. *Corresponding author. E-mail: [email protected] Accepted 22 June, 2016 Ganglioneuroma is the tumor of sympathetic nerve fibers arising from neural crest cells. It may be present anywhere in the body along the autonomic nerve cells. This is usually a non-cancerous tumor. Its age incidence is between the 10-40 years of age. Usually tumor is asymptomatic; symptoms depend upon the location of tumor. In chest and pelvis, it may give symptoms by compression effect. Also, in the abdomen, mass may be palpable; this may be an incidental finding by radiologist. Radiological investigations may not differentiate this condition from other solid masses; misdiagnosis is common. Furthermore, the diagnosis is made on the basis of histopathology. Literature also shows that ganglioneuroma is misdiagnosed mostly. Key words: Retroperitoneal mass, neural crest cells, autonomic nerve cells, incidentalomas. INTRODUCTION Ganglioneuroma is a rare entity which arises from and females with enlarged clitoris. The most important sympathetic nerve fibers. The commonest sites are differential diagnosis is with neuroblastoma in which mediastinum, retroperitoneum and adrenal medulla but it urinary adrenaline, dopamine and VMA are raised may arise anywhere in the body usually along autonomic whereas, ganglioneuroma is usually hormonally silent nerve cells (Erem et al., 2008). It is a benign tumor and (Sucandy et al., 2011). malignant transformation is extremely rare. Age incidence Misdiagnosis and maltreatment is not uncommon. The is between 10-40 years but is more common in best diagnostic tool to identify these tumors is computed adolescents (Srinivasan et al., 2007). It is usually tomography or MRI of chest, abdomen and pelvis. discovered during evaluation or treatment of another Treatment involves surgical removal if symptomatic (Qing condition and the diagnosis is only histological (Oderda et et al., 2010). The drastic complications include al., 2011). The radiology mainly helps in its detection, as irreversible compression effect on spinal cord which may an incidentaloma (Carrión López et al., 2012). not resolve even after removal of tumor. Usually this condition is asymptomtomatic (Erem et al., 2009) and the symptoms depend upon its location. In chest it may cause breathing difficulty, chest pain or CASE REPORT tracheal compression, and in retroperitoneal space, it can cause abdominal pain, mass or bloating. The pressure A 12 year old Pakistani boy was presented with history of effects on spinal cord may range from pain to motor or palpable mass in left flank. The mass gradually increased sensory loss (Alimoglu et al., 2012). Rarely, in a size over a period of twelve weeks. Initially, the boy ganglioneuroma may produce certain chemicals or was seen and evaluated by a general practitioner. hormones and patients may be present with Abdominal ultrasound (USG) revealed heterogenous hypertension, increased body hair, sweating, diarrhea solid mass involving left kidney measuring 13.2x9.6 cm. 116 J. Sci. Res. Stud. Figure 1. Axial postcontrast CT image shows the well-circumscribed mass in the retroperitonium on the left side. The mass is seen anterior to the abdominal aorta and inferior vena cava. Left kidney not visualized. Figure 2. Per operative photo showing huge solid mass (Black arrow) adjacent to left kidney (Red Arrow). There was no stone or hydronephrosis and impression unremarkable. On abdominal examination, the mass was was that of left renal mass with suspicion of Wilms’ visible upon inspection with normal overlying skin and tumor. there were no prominent vessels. It was bimanually He was admitted at the Urology Department, Services palpable, firm in consistency and partially mobile. Also, Hospital Lahore, Pakistan which is a tertiary care overlying skin temperature was normal, examining fingers hospital. Nothing was remarkable in general physical could insinuate below costal margins since there was no examination. However, the systemic examination of bruit on auscultation. The investigations complete blood respiratory, cardiovascular and central nerve system was count, renal and liver functions were within normal limits. Inam and Manan 117 Figure 3. Well encapsulated Specimen after resection. (a) (b) Figure 4. (a) Low power view of; ( b) High power view of benign neoplastic lesion showing wavy fibers of schwan cells and fibrous tissue. Ganglion cells are seen in between. Computed tomography (CT) abdomen was carried out and suprarenal gland was identified and removed and according to CT abdomen report, fairly well defined (Figures 2 and 3). The recovery was uneventful and soft tissue density encapsulated minimally enhancing patient was discharged in good condition. The biopsy hypo-dense mass was seen arising from upper and mid report came out to be “Ganglioneuroma” (Figure 4a and pole of left kidney; left adrenal gland was not separately b). The follow up USG at 3 months showed normal visible. Medially mass was projecting towards midline kidneys and there was no local recurrence. close to lateral margin of abdominal aorta, not encircling or infiltrating the vessels (Figure 1). Radiological diagnosis was in favour of the Wilms’ tumor. In addition, CONCLUSION Patient was referred to oncologist for neo-adjuvant chemotherapy but he demanded tissue diagnosis. Tru-cut Ganglioneuroma is usually a rare condition which can be biopsy was performed which was inconclusive. easily confused with other pathologies. It provides a great Explanation was made to the Parents and the boy was diagnostic challenge. Computed tomography may not planned for exploration. The mass was approached delineate the retroperitoneal mass from kidney. High through transperitoneal left Chevron incision. A solid index of suspicion is required to reach the proper 14x10 cm retroperitoneal mass separate from left kidney diagnosis. Also, misdiagnosis can lead to misdirected 118 J. Sci. Res. Stud. management. Erem C, Kocak M, Cinel A, Erso HO, Reis A (2008). Dopamine- secreting adrenal ganglioneuroma presenting with paroxysmal hypertension attacks. Saudi Med. J. 29(1):122-5. Erem C, Ucuncu O, Nuhoglu I, Cinel A, Cobanoglu U, Demirel A, Koc E, Consent Kocak M, Guvendi GF (2009). Adrenal ganglioneuroma: report of a new case. Endocrine 35(3):293-6. Written informed consent was obtained from patient’s Oderda M, Cattaneo E, Soria F, Barreca A, Chiusa L, Morelli B, Zitella A, Gontero P (2011). Adrenal Ganglioneuroma with Multifocal parents for publication of this case report and any Retroperitoneal Extension: A Challenging Diagnosis. Sci. World J. accompanying images. 11:1548-1553. Qing Y, Bin X, Jian W, Li G, Linhui W, Bing L, Huiqing W, Yinghao S (2010). Adrenal ganglioneuromas: a 10-year experience in a Chinese population. Surgery 147(6):854-60. REFERENCES Srinivasan R, Koliyadan KS, Krishnand G, Bhat SS (2007). Retroperitoneal ganglioneuroma with lymphnode metastasis: a case Alimoglu O, Caliskan M, Acar A, Hasbahceci M, Canbak T, Bas G report. Indian J. Pathol. Microbiol. 50(1):32-5. (2012). Laparoscopic Excision of a Retroperitoneal Ganglioneuroma. Sucandy I, Akmal YM, Sheldon DG (2011). Ganglioneuroma of the JSLS 16(4):668-670. adrenal gland and retroperitoneum: A case report. North Am. J. Med. Carrión López P, Martínez Ruiz J, Martínez Sanchiz C, Perán Teruel M, Sci. 3:336-338. Atienzar Tobarra M, Donate Moreno MJ, Virseda Rodriguez JA (2012). Adrenal ganglioneuroma. Arch. Esp. Urol. 65(8):773-6. .
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