Neuroendocrine tumors adversely affect the pregnancy outcome, if remain undiagnosed or untreated. Management of such a patient is challenging, especially while weighing the advantages and disadvantages of surgical and medical treatment during early pregnancy.
The Case
We present the case of a pregnant woman who presented to us with hypertension, episodes of headache, sweating and palpitations. On investigation, she was found to have bilateral heterogenous adrenal masses, a hypervascular lesion inferior to the aortic bifurcation, multiple hypervascular masses with calcifications in the pancreas, and thrombosis of the splenic vein. Twenty-four hour urinary metanephrine measured 350 μg. Since she was in first trimester of pregnancy and waiting with such extensive disease was not favorable, she was aggressively treated along with urgent surgery in the form of bilateral adrenalectomy, distal pancreatectomy and splenectomy soon after the diagnosis. Histopathological examination of bilateral adrenal glands confirmed pheochromocytoma, tumour from aortic bifurcation showed paraganglioma, and pancreatic mass showed nodules having neuroendocrine morphology and positivity for chromogranin and synaptophysin. Biochemical studies revealed non- functioning nature of pancreatic tumour. She had a normal labor and delivery of a healthy baby. Genetic analysis revealed missense c.499C > T (p.ARG167TRP) mutation in von Hippel Lindau’s gene.
Principal conclusions
Any advantage of delaying the surgery in a pregnant woman diagnosed of neuroendocrine tumours in first trimester should be weighed against potential hazards of long term medical therapy and the disease itself. We emphasize the importance of timely treatment, particularly in multifocal and extensive neuroendocrine tumours disease, for a favorable pregnancy outcome.
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