Multiple gastrointestinal stromal tumors and bilateral pheochromocytoma in neurofibromatosis

Table 1 Review of the literature. Six cases with the coincidence of NET and GIST in Neurofibromatosis type 1 (NF1)

Patientage, sex	GISTLocation /Diameter	NETLocation + Histology/Diameter	Surgery	Follow up /outcome	Reference [no]/year of publication
36, m	Jejunum 3, 5 cm	Papilla of vater n.i. 1, 2 cm	Duodeno-pancreatectomy	Two years/ well, no recurrence	Karatzas et al[10] 2000
60, m	Duodenojejunal n.i.	Pheochromocytoma (bilateral) n.i.	n.i. (Article in Italian)	n.i.	Rizzo et al[9] 2001
64, f	Duodenum, Jejeunum, Stomach Numerous: 0.5-2 cm	Papilla of vater Somatostatinoma n.i.	Local resection	n.i.	Usui et al[11] 2002
43, f	Jejunum 3, 5 cm	Duodenum neuroendocrine carcinoma 2, 7 cm	Duodeno-pancreatectomy	54 mo/ well	Kramer et al[12] 2005
60, m	Small bowel numerous: cm	Pheochromocytoma (bilateral)	n.i.	n.i.	Lisewski et al[13] 2006
63, f	Ileum 6, 5 cm Jejunum (prerenal) 3 cm	Pheochromocytoma (metachron, bilateral) 5 cm, left adrenal gland right adrenal gland 3 cm	Adrenalectomy intestinal segment resection tumor resection jejunal resection	48 mo/ well, no recurrence	as presented here

n.i.: no information.

Table 2 Time follow up of the presented case

Time	Tumor (size)		Localization	Treatment
0	PCC (3 cm) GIST (6.5 cm)		Left adrenal gland ileo-terminal	Adrenalectomy segment resection
9 m	PCC (3 cm) GIST (3 cm) 7 x GIST (2 mm)		Right adrenal gland proximo-jejunal	Tumor-resection segment resection tumor resection
45 m	No tumor			Corticoid substitution

PCC: pheochromocytoma; GIST: gastrointestinal stromal tumor; m: month; cm: centimetre; mm: millimetre.