Multidisciplinary approach including receptor radionuclide therapy with 90Y-DOTATOC ([90Y-DOTA0, Tyr3]-octreotide) and 177Lu-DOTATATE ([177Lu-DOTA0, Tyr3]-octreotate) in ectopic cushing syndrome from a metastatic gastrinoma: a promising proposal.
OBJECTIVE
To present a case of a young woman with Cushing syndrome caused by ectopic production of adrenocorticotropic hormone from a metastatic pancreatic gastrin-secreting endocrine carcinoma, who had a good response to combination peptide receptor radionuclide therapy.


METHODS
We review the history, physical examination, laboratory investigations, and radiographic findings in this unusual patient. Moreover, the multimodal interventions are described and discussed.


RESULTS
In a 38-year-old woman with typical signs of cortisol excess, laboratory studies revealed diabetes mellitus, hypokalemia, and high levels of adrenocorticotropic hormone, plasma cortisol, and urinary cortisol. Abdominal computed tomography showed a 4-cm pancreatic mass and multiple metastatic lesions in the liver, and ectopic Cushing syndrome was diagnosed. Treatment consisted of surgical debulking of the tumor, ketoconazole, somatostatin analogues, chemoembolization of the liver metastatic lesions, and peptide receptor radionuclide therapy with the radiolabeled somatostatin analogues 90Y-DOTATOC ([90Y-DOTA0, Tyr3]-octreotide) and 177Lu-DOTATATE ([177Lu-DOTA0, Tyr3]-octreotate). The 5 1/2-year follow-up showed positive results, which included complete regression of all clinical and hormonal evidence of the tumor and substantial decrease in the size and number of hepatic metastatic lesions. The patient achieved and still maintains an optimal quality of life.


CONCLUSIONS
To the best of our knowledge, this is the first report of a multidisciplinary approach including peptide receptor radionuclide therapy with 90Y-DOTATOC and 177Lu-DOTATATE, which proved to be effective in improving clinical outcome in a case of metastatic endocrine carcinoma of the pancreas in conjunction with ectopic Cushing syndrome. In this unusual case, the patient has one of the longest durations of survival in this setting described in the literature.
Davì
Maria Vittoria
MV
The Department of Internal Medicine, University of Verona, Verona, Italy.
Bodei
Lisa
L
Ferdeghini
Marco
M
Falconi
Massimo
M
Testoni
Marco
M
Paganelli
Giovanni
G
Oliani
Cristina
C
Lo Cascio
Vincenzo
V
Francia
Giuseppe
G
D000324:Adrenocorticotropic Hormone / Q000378:metabolism; D000328:Adult; D018931:Antineoplastic Agents, Hormonal / Q000627:therapeutic use; D003131:Combined Modality Therapy; D003480:Cushing Syndrome / Q000209:etiology / Q000378:metabolism / Q000473:pathology*; D005260:Female; D015408:Gastrinoma / Q000150:complications / Q000473:pathology / Q000628:therapy*; D006801:Humans; D009362:Neoplasm Metastasis; D015282:Octreotide / Q000031:analogs & derivatives* / Q000627:therapeutic use; D009942:Organometallic Compounds / Q000627:therapeutic use*; D010190:Pancreatic Neoplasms / Q000150:complications / Q000473:pathology / Q000628:therapy*; D015021:Yttrium Radioisotopes / Q000627:therapeutic use
D018931:Antineoplastic Agents, Hormonal; D009942:Organometallic Compounds; D015021:Yttrium Radioisotopes; D000324:Adrenocorticotropic Hormone; C496730:90Y-octreotide, DOTA-Tyr(3)-; C447941:lutetium Lu 177 dotatate; D015282:Octreotide