Recurrence of a neuroendocrine tumor of adrenal origin: a case report with more than a decade follow-up

Fatemeh Rahmani,Maryam Dehghani,Maryam Tohidi,Farzad Hadaegh,Behrooz Broumand

doi:10.1186/s12902-020-00673-7

Abstract

BackgroundNeuroendocrine tumor (NET) with adrenocorticotropic hormone (ACTH) secretion are very rare. To our knowledge, no follow-up study is published for ACTH-secreting NET, regardless of the primary site, to show second occurrence of tumor after a long follow-up, following resection of primary tumor.Case presentationHere, we describe a 49-year-old-man with cushingoid feature, drowsiness and quadriparesis came to emergency department at December 2005. Laboratory tests revealed hyperglycemia, metabolic alkalosis, severe hypokalemia, and chemical evidence of an ACTH-dependent hypercortisolism as morning serum cortisol of 57 μg /dL without suppression after 8 mg dexamethasone suppression test, serum ACTH level of 256 pg/mL, and urine free cortisol of > 1000 μg /24 h. Imaging showed only bilateral adrenal hyperplasia, without evidence of pituitary adenoma or ectopic ACTH producing tumors. Importantly, other diagnostic tests for differentiating Cushing disease (CD) from ectopic ACTH producing tumor, such as inferior petrosal sinus sampling (IPSS), corticotropin releasing hormone (CRH) stimulation test, octreotide scan or fluorodeoxyglucose (FDG)-positron emission tomography (PET) scan were not available in our country at that time. Therefore, bilateral adrenalectomy was performed that led to clinical and biochemical remission of hypercortisolism and decreased ACTH level to < 50 pg/mL, findings suggestive of a primary focus of NET in adrenal glands. After 11 years uncomplicated follow up, the ACTH level elevated up to 341 pg/mL and re-evaluation showed a 2 cm nodule in the middle lobe of the right lung. Surgical excision of the pulmonary nodule yielded a carcinoid tumor with positive immunostaining for ACTH; leading to decrease in serum ACTH level to 98 pg/mL. Subsequently after 7 months, serum ACHT levels rose again. More investigation showed multiple lung nodules with metastatic bone lesions accompanied by high serum chromogranin level (2062 ng/mL), and the patient managed as a metastatic NET, with bisphosphonate and somatostatin receptor analogues.ConclusionThis case of surgically-treated NET showing a secondary focus of carcinoid tumor after one decade of disease-free follow-up emphasizes on the importance of long-term follow-up of ACTH-secreting adrenal NET.

Highlights

Neuroendocrine tumors (NETs) are a family of heterogeneous neoplasms of epithelial or neuronal/neuroectodermal origin, arising from any anatomical site
This case of surgically-treated NET showing a secondary focus of carcinoid tumor after one decade of disease-free follow-up emphasizes on the importance of long-term follow-up of adrenocorticotropic hormone (ACTH)-secreting adrenal NET
To the best of our knowledge, this is the first case of ACTH-secreting NET, with improvement of symptoms and signs of hypercortisolism and normalization of the serum ACTH level after bilateral adrenalectomy, with long term of remission, complicated by the recurrence of the tumor in the lung with multiple bone metastasis after 11 years

Summary

Conclusion

We reported a unique case of ACTH-secreting NET in adrenal with resolving hypercortisolism and normalization of ACTH level after bilateral adrenalectomy and recurrence of the second ACTH producing NET in the lung after more than a decade follow-up.

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