Table 2. Clinicopathological and molecular relevant data of the patients with PETs harboring TERT promoter mutations.

Case number	Gender	Age1	Location	PET type	Size2	Microadenomas	Functional status	Germline mutations	pT3	pT4	Lymph node metastasis	Distant metastasis	Follow-up5	Status at last follow-up
1	F	39	body	NET G1	44	yes	insulinoma	MEN1 p.Q453X	3	3	N1	M0	107	AWD
2	F	55	body	NET G2	30	no	non functional	-6	2	2	N1	M0	9	DOD
3	M	51	tail	NET G1	30	yes	insulinoma	MEN1 p.A572V	2	3	Nx	M0	124	DOC
4	F	32	tail	NET G2	94	yes	non functional	VHL p.S65W	3	3	Nx	M1 (liver)	46	DOD

¹years;

²mm;

³according to ENETS classification;

⁴according to UICC/AJCC classification; ⁵months; ⁶No MEN1 or VHL mutations were detected.

AWD – alive without disease; DOD – death of disease; DOC – death of other causes. Clinical presentations: Case 1: Primary hyperparathyroidism and insulinoma. Known family history, both the father and a sister with pancreatic tumour, a pituitary adenoma with prolactin production and primary hyperparathyroidism; Case 3: Recurrent episodes of hypoglycaemia, associated with insulinoma. No other crises following surgery. Posterior history of recurrent upper gastrointestinal haemorrhage associated with gastric ulcers. The presence of gastrinoma has never been confirmed. In both MEN1 cases there was no clinical or laboratorial evidence of other functioning-type NET. Case 4: bilateral retinal angiomatosis, cervical spinal hemangioblastoma, endolymphatic sac tumour, hepatic haemangioma and multiple renal cysts.