Research Article

Somatostatin Treatment for Ectopic ACTH Syndrome due to Pancreatic Neuroendocrine Tumors: Review of the Literature

Table 1

Clinical characteristics of 13 patients with EAS-p-NETs.

YearFirst authorAge at diagnosis (year)SexMorning ACTH (pg/ml)Morning cortisol (ug/dl)24 h UFC (ug/d)Pancreatic tumor size (cm)Tumor metastasisPercentage of Ki-67Treatment procedureEffect of controlling ACTH and cortisol after SSA treatmentEffect of tumor growth after SSA treatmentDuration of follow-up (months)Prognosis

2020Our case23F13924.1653.641.1 × 0.8Lymph nodes and multiple liver metastases10–15%Octreotide LAR and operationControlledSD28Survival
2017do Amor Divino et al. [14]58M1904865195.0Multiple liver metastases6%Operation, octreotide LAR and chemotherapyUncontrolledPD42N/A
2016Tadokoro et al. [15]61F205.915.286.16.0Lymph nodes metastasis11.50%Octreotide LAR and chemotherapyUncontrolledPD36Died
2014Sauer et al. [16]46M2471198382001.2Liver metastasis<1%Octreotide and operationN/AN/AN/AN/A
2014Rajeev et al. [17]34M22772.6N/AN/ALymph nodes and liver metastasis20%Operation, lanreotide, and sunitinibUncontrolledPD27Survival
2013Patel et al. [18]44FN/AN/AN/ALargeMultiple liver metastasesN/AOperation and octreotide LARControlledN/A74N/A
2010Kondo et al. [19]64F34026.98932.5 × 2.5Multiple liver metastases10%Octreotide LAR and operationControlledPR20Survival
2003Doi et al. [20]21F735145N/A5.0 × 3.5 × 2.0Liver and lymph nodes metastasisN/AOctreotide and operationControlledPD61Died
1999Gill et al. [21]31F17778.22102N/ALiver, bone, and pelvic metastasisN/AOctreotide and operationControlledPD18Died
1993Woodhouse et al. [22]33F436181.2N/AN/AExtensive liver metastasisN/AOperation and octreotideControlledPR18N/A
1989Bertagna et al. [23]37F21826550N/ADiffuse metastasisN/AChemotherapy and octreotideControlledPD4Died
1988Lamberts et al. [24]37M68.13210805.0 × 3.0Lymph nodes metastasisN/ASMS 201-995ControlledPR6N/A
1988Ruszniewski et al. [25]49F13816.5N/AN/AMultiple liver metastasesN/AOperation, chemotherapy, and SMS 201-995ControlledSD80N/A

EAS: ectopic ACTH syndrome; p-NETs: pancreatic neuroendocrine tumors; EAS-p-NETs: EAS due to p-NETs; ACTH: adrenocorticotropic hormone; UFC: urinary free cortisol; SSA: somatostatin analog; LAR: long-acting release; SD: stable disease; PD: progressive disease; PR: partial response; N/A: not available.