Atypical extragonadal germ cell tumors

Abstract

Aim:To review the experience with the diagnosis and management of extragonadal germ cell tumors (GCT) with a subset analysis of those with atypical features.Materials and Methods:A retrospective chart review of patients of extragonadal germ cell tumors between 2000 and 2010 was carried out.Results:Fifteen children aged 7 days to 15 years (median, 1.5 years) were included. Three had an antenatal diagnosis (one sacrococcygeal, one retrobulbar, one retroperitoneal tumor) and were operated in the neonatal period. The locations were distributed between the retrobulbar area (1), anterior neck-thyroid gland (1), mediastinum (4), abdominothoracic extending through the esophageal hiatus (1), retroperitoneal (4) and sacrococcygeal (4). On histological examination, five harbored immature elements while two were malignant; the latter children received postexcision adjuvant chemotherapy. There was no mortality. At a median follow-up of 4.5 years (6 months to 8 years), 14/15 have had an event-free survival. One immature mediastinal teratoma that recurred locally 7.5 years after the initial operation was excised and adjuvant chemotherapy instituted.Conclusions:Extragonadal GCTs in children are uncommon and occasionally present with atypical clinical, radiological and histological features resulting in diagnostic and therapeutic dilemmas.

Retroperitoneal yolk sac tumour encroaching the liver and adrenal gland with tumour thrombus in cavo-atrial region and hepatic veins

“…Kazi et al reported a case of successful management of extensive cavo-atrial and hepatic venous tumour thrombus in a retroperitoneal paediatric germ cell tumour by radical surgical resection with hepatectomy, cavotomy, retrohepatic caval resection and right atrial thrombectomy under cardiac bypass 2. Other than this paediatric case report, all the other reports are in adult age group 4–7.…”

“…Primary extragonadal RPYSTs are extremely rare and usually present late only when they reach a considerable size 1 2. A high alpha fetoprotein (AFP) value with a large retroperitoneal mass should always raise suspicion of such a tumour 1 2.…”

“…Primary extragonadal RPYSTs are extremely rare and usually present late only when they reach a considerable size 1 2. A high alpha fetoprotein (AFP) value with a large retroperitoneal mass should always raise suspicion of such a tumour 1 2. Paediatric retroperitoneal germ cell tumours with tumour thrombus extending to the atrio-caval or hepatic veins are rare.…”

Germ Cell Tumor Located in Gastrointestinal System: A Report of Two Cases

“…The treatment requires a multimodality approach. The treatment of localized disease is surgery, the treatment of disseminated disease is combined chemoradiotherapy [ 9 ].…”

Giant adrenal germ cell tumour in a 59-year-old woman

“…4 In rare cases, extragonadal GCTs have been associated with Klinefelter syndrome. 5,6 Their anatomic distribution varies widely and includes the mediastinum, 7 sacrococcygeal region, 8 neck, retrobulbar, 9 retroperitoneum, 10 and other rare sites. 11,12 GCT rarely involves the adrenal gland.…”