DrSreelekshmy , DrShilpa Radhakrishnan, DrNaveen Viswanath , Dr C Jayakumar Aims Kochi
6 year old female child,was admitted due to high grade intermittent fever and wet cough of 8 days. Vitals were stable and PICCLE was normal . Clinically child had 3cm soft liver below the right costal margin and just palpable spleen. CBC and peripheral smear were within normal limits except for neutropenia.
Differentials considered were IMN,Typhoid,
IgM EBV, Widal were negative. Chest xray done as child had cough was normal. As the child continued to have fever spikes, she was evaluated further with USG Abdomen which revealed well circumscribed solid lesion in the retroperitoneum in the left paraaortic region at the level of renal hilum measuring 25x15x45mm with focal calcific speck seen within, which is suggestive of ?neurogenic origin such as neuroblastoma or ganglioneuroma. CT Abdomen showed left para aortic lesion overlying psoas muscle with no features of local invasion, possible differentials of ganglioneuroma and ganglioneuroblastoma.
Blood metanephrine and normetanephrines was normal. Neuron specific enolase ,was 4518pg/ml (normal :313-1652pg/ml) ,LDH 358 U/L (0-314 U/L) and Ferritin 330.2ng/ml (7-140ng/ml) done were also elevated. Child was discharged as the fever subsided completely with the antibiotics given for ALRI child was and kept under follow up of pediatrics and pediatric surgery. Laproscopic surgery was done and solid mass was removed in toto and sample sent for histopathological examination. HPE revealed cellular neoplasm composed of spindle cells arranged in fascicles admixed with both immature and mature ganglion cells in small clusters and singly scattered and the spindle cells have elongated hyperchromatic nuclei with wavy cytoplasm, with no neuroblastic component or neutrophil seen which is highly suggestive of a Ganglioneuroma, maturing type. Child is now under follow up of pediatrics , Medical Oncology and Hematology.
Discussion: Ganglioneuroma is a benign neurogenic tumor, originating from the neuroepithelium along sympathetic ganglia. The posterior mediastinum is the main localization followed the retroperitoneum and cervical region.They may arise denovo or other malignant neuroblastic tumors may show spontaneous regression to form mature benign ganglioneuroma. Metastasis is rare. Surgical excision warrants complete cure in almost all patients. Ganglioneuromas should be thoroughly examined to exclude any foci of neuroblastoma.
Carry home message : Good sonologist will pick up what we could not do with our hands. It may be worth to do abdominal ultrasonogram at least in precious children as nowadays most couple practise one child norm.