CASE REPORTS   

Chirurgia 2008 October;21(5):303-5

Copyright © 2008 EDIZIONI MINERVA MEDICA

language: English

Incidentally discovered huge retroperitoneal ganglioneuroma in a severely obese girl

Sartorio A. ^{1, 2}, Boniello S. ², Moro D. ¹, Contessini-Avesani E. ³, Poggi L. ³, Carsana L. ⁴, Del Piano S. ⁵

¹ Divisione di Auxologia, Istituto Auxologico Italiano, IRCCS, Verbania e Milano 2 Laboratorio Sperimentale Ricerche Auxo-endocrinologiche, Istituto Auxologico Italiano, IRCCS, Verbania e Milano 3 Department of Surgery, University of Milan Maggiore Hospital, IRCCS, Milan, Italy 4 Division of Pathology, Department of Medicine Surgery and Dental Sciences, University of Milan A.O. S.Paolo and Fondazione Ospedale Maggiore Policlinico, Mangiagalli, Regina Elena, Milan 5 Istituto Auxologico Italiano, IRCCS, Servizio di Radiologia, Milano

We report a case of a silent huge ganglioneuroma growing in retroperitoneum, incidentally discovered in a severely 16-year-old girl, hospitalized for a body-weight reduction program for severe obesity (weight: 86.7 kg; height: 162 cm; BMI: 33.0 kg/m2, >97th centile for age and sex). Abdominal ultrasonography demonstrated a inhomogeneous hypoechoic mass, 14x18 cm in diameter, with multiple hyperechoic calcifications, which compressed the right hepatic lobe and displaced the right kidney. Plain CT showed a huge, well-circumscribed retroperitoneal mass, occupying extensively the right side of the abdomen, which was slightly and heterogeneously enhanced on contrast-enhanced CT. Therefore, we performed a medial laparotomy with excision of the mass and a right adrenalectomy. The tumour weighed 1840 g and had a maximum diameter of 18.5 cm and consisted of a smooth-contoured, solid, ovoid mass, covered by a thin incomplete fibrous capsule. The mass was firm, grey to tan, translucent and homogeneous, with only patchy hemorrhagic areas. Histological examination was ganglioneuroma, mitotic index being lower than 1 mitosis per 50 high power field; neither neuroblasts nor areas of necrosis were found through a wide sampling. In conclusion, the lack of clinical signs, symptoms and/or hormonal alterations and the presence of severe abdominal obesity contributed to mask the earlier diagnosis of this huge abdominal mass during regular physical examinations by the pediatricians. Despite the huge dimension of the mass and the concomitant right adrenalectomy, the post-operative clinical course was satisfactory and the 3-month follow-up showed normal adrenal function and routine laboratory investigations.