Endocrine Abstracts

Background: Neuroendocrine tumors present in the MEN-1 syndrome are usually located in pancreatic islets and more than 10% appear de novo, affecting any age group. The debut of a carcinoid tumor is rare, with a prevalence of 2% of bronchial carcinoid tumors.

Materials and methods: We present the case of a 49-year-old woman referred from the Oncology clinic for the assessment of thyroid incidentalomas found in a follow-up CT scan of multifocal bronchial carcinoid tumor (typical carcinoid tumorlet), operated in 2015 with persistently increased chromogranin A (CrgA). Pathology results showed a non-infiltrating, low-grade neuroendocrine neoplasm (carcinoid tumor), within the surgical margins, and < 2 mitoses/mm². (2 in the mediastinal face of the left lobe and another in the right lobe)

Results: Thyroid ultrasound showed a multinodular goiter with a dominant 28-mm nodule classified as Bethesda category II. Laboratory tests revealed a pituitary profile, thyroid hormones, catecholamines, 5-HIA, and CEA within normal ranges, elevated ChromograninA levels and undetectable calcitonin. Increased levels of calcium and phosphorus are displayed in table 1. Octeoscan, bone densitometry and calciuria were requested, which didn’t show any abnormalities. Parathyroid scintigraphy was performed due to elevated calcaemia >1 gr/dl, with possible adenoma in the upper pole of the right thyroid lobe. Patient was referred for surgery in February 2020, although it was delayed due to the pandemic until June 2021. A total thyroidectomy and a parathyroidectomy were performed, and a parathyroid adenoma was found. Afterwards, the patient temporarily presented phosphocalcic metabolism values within a normal range, followed by a subsequent increase (Table 1). MEN-1 genetic study was requested. A pathogenic variant in the MEN-1 sequence was detected: c.1252G>A, P (Asp418Asn) in Heterozygosis (rs104894264).

Discussion: Following the suspicion of MEN-1, even in patients with an atypical condition, a genetic study should be requested early, prior to the indication of parathyroidectomy. This is due to the possible implication in the surgical technique, given the great recurrence of hyperparathyroidism in these cases and to avoid the appearance of early complications derived from it.