Neoadjuvant Peptide Receptor Radionuclide Therapy in a Rare Case of Pediatric Primary Hepatic Gastrinoma.

ABSTRACT: Gastrinomas with predilection for the adult male population are located in the gastrinoma triangle (>90%). Primary hepatic gastrinoma especially in pediatric population is very rare. Peptide receptor radionuclide therapy has shown benefit in metastatic gastroenteropancreatic neuroendocrine tumors (NETs) with an increasing interest in expanding its role as neoadjuvant treatment modality to improve the surgical candidature in inoperable NETs. There is currently no literature supporting its role in the pediatric NET patients. We present a rare case of a young boy with primary hepatic gastrinoma where 177Lu-based peptide receptor radionuclide therapy in the neoadjuvant setting contributed to his final disease-free status.

Safety of hepatic resection in metastatic disease to the liver after yttrium-90 therapy.

BACKGROUND: Unresectable hepatic metastases from aerodigestive cancers are common and in most cases herald a poor prognosis. A small percentage of patients maybe amenable to surgical resection or ablation once the biology of the disease and the burden of hepatic disease are better understood. The use of hepatic arterial resin microspheres containing the ß emitter, yttrium-90, has been reported in the treatment of unresectable hepatic metastases. The goal of this review was to evaluate the use of yttrium-90 hepatic arterial therapy in the management of hepatic metastases and surgical downstaging. METHODS: We reviewed our prospective hepatic arterial therapy registry and found 44 patients who had received Sir Sphere treatment for unresectable hepatic malignancies from 11/06 to 7/08. Response was assessed by using CT-imaging and characterized using modified response evaluation criteria in solid tumors (RECIST). All patients were managed in a multidisciplinary tertiary referral center specializing in hepatic malignancies. RESULTS: A total of 44 patients, 34 men and 10 women, with a median age of 60 y (range 44-8), received 67 treatments. The disease types treated were one adenosquamous tongue, one adrenal, nine carcinoid, three cholangiocarcinoma, four esophageal, one gastric, one gastrinoma, one GIST, four HCC, 15 colorectal, one melanoma, one non-small-cell lung, one occular, and one sarcoma. Four patients treated proceeded to resection because of downstaging of disease or no evidence of extrahepatic progression. The median age in these patients was 61 y (range 49-62). All of the patients had less than 25% tumor burden in the liver. Surgical therapy consisted of two patients undergoing right hepatic lobectomy, one patient who also underwent two wedge resections of segment 3, and one patient who had a left lateral hepatectomy with right lobe microwave ablation. The median length of postoperative stay was 7 d. There was no evidence of liver dysfunction following resection in any of the patients. None of the patients show evidence of recurrence in the liver following resection. One patient has had progression of disease in the lungs following resection, histologically confirmed as metastatic rectal carcinoma. All of the patients are currently alive with a median survival of 2 y. CONCLUSION: Hepatic directed yttrium-90 is a minimally invasive, highly effective therapy that can be utilized to downstage the hepatic burden and/or assess the biology of the disease to allow for appropriate treatment. The use of yttrium-90 microspheres for radio-embolization of metastases in the liver can successfully downstage the lesions to allow for surgical resection in patients with amenable predictors, and can provide a significantly better prognosis in these patients. This form of therapy for the purposes of downstaging tumors for resection merits more extensive study in order to provide the best possible outcomes for patients with metastatic liver disease.

Gastrinoma de localización anatómica "normal" / Gastrinoma of "normal" anatomic location

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Considerations concerning a tailored, individualized therapeutic management of patients with (neuro)endocrine tumours of the gastrointestinal tract and pancreas.

Endocrine tumours of the gastrointestinal tract and pancreas may present at different disease stages with either hormonal or hormone-related symptoms/syndromes, or without hormonal symptoms. They may occur either sporadically or as part of hereditary syndromes. In the therapeutic approach to a patient with these tumours, excessive hormonal secretion and/or its effects should always be controlled first. Tumour-related deficiencies or disorders should also be corrected. Subsequently, control should be aimed at the tumour growth. Surgery is generally considered as first-line therapy for patients with localized disease, as it can be curative. However, in patients with metastatic disease the role of first-line surgery is not clearly established and other therapies should be considered, such as non-surgical cytoreductive therapies, biotherapy (with somatostatin analogues or interferon-alpha), embolization and chemoembolization of liver metastases, chemotherapy (with single or multiple dose regimens) and peptide receptor-targeted radiotherapy. The delicate balance of the use of the different therapeutical options in patients with endocrine tumours of the gastrointestinal tract and pancreas emphasizes the importance of team approach and team expertise.

[Bone metastasis from gastrinoma without hepatic involvement. Utility of the scintigraphy with 111 In-pentetreotide]. / Metástasis ósea de gastrinoma sin afectación hepática.Utilidad de la gammagrafía con 111-In-pentetreótide.

The case of a 54 year old patient suffering from gastrinoma (within a type I multiple endocrine neoplasm) is presented. In 1990, she underwent a surgical resection. Eight years later, she suffered from a pain in the left thigh. The 99mTc-MDP bone scintigraphy showed bone metastasis. The CT scan did not show any hepatic involvement and the scintigraphy with 111In-Pentetreotide verified bone metastasis of a neuroendocrine tumor as a single expression of the recidive. In this case, the utility of the scintigraphy with octreotide to facilitate the localization and extent of the neuroendocrine tumors is demonstrated.

Toxicity of high-activity 111In-Octreotide therapy in patients with disseminated neuroendocrine tumours.

Disseminated neuroendocrine tumours are difficult to treat and are generally not responsive to radiotherapy or chemotherapy. Nuclear medicine techniques using a radiolabelled somatostatin analogue, 111In-Octreotide, have been used for the diagnosis of neuroendocrine tumours. It has been suggested that high activities of such an agent may have a therapeutic effect. The aims of this study were to assess toxicity and to determine if there had been evidence of efficacy. Eight patients with known disseminated neuroendocrine tumours were enrolled in the study; six had carcinoid tumours, one had a medullary cell carcinoma of the thyroid and one patient had a malignant gastrinoma. Between 1.3 and 4.6 GBq of 111In-Octreotide were administered to each patient for up to five administrations over 12 months. A total of 23 administrations were given. Tests of vital signs, renal, liver and endocrine function as well as haematological markers were taken before and after treatment. The treatment was well tolerated with only one patient suffering from a sensation of flushing during the infusion but no changes in vital sings. There was a transient (up to 48 h) drop in circulating lymphocytes in four patients and platelets in two patients; no supportive therapy was needed. One patient with severe renal impairment had a slight reduction in glomerular filtration rate. We conclude that high-activity 111In-Octreotide is well tolerated with low toxicity and can be considered for use in patients with disseminated neuroendocrine tumours. Further work is now being performed to assess efficacy.

Response to treatment with yttrium 90-DOTA-lanreotide of a patient with metastatic gastrinoma.

1,4,7,10-tetraazacyclododecane-N,N',N",N'''-tetraacetic acid (DOTA)-lanreotide is a universal somatostatin (SST) receptor subtype ligand that binds to a large variety of human tumors. We report the case of a patient with metastatic gastrinoma who was treated with 90Y-DOTA-lanreotide. Before treatment, dosimetry with 111In-DOTA-lanreotide (150 MBq, 10 nmol) indicated a dose of 5.8 mGy/MBq for the recurrent abdominal gastrinoma, and a mean dose of approximately 1.0 mGy/MBq for liver metastases (i.e., 56 and approximately 10 mGy/MBq for 90Y-DOTA-lanreotide, respectively). After four infusions of 90Y-DOTA-lanreotide (each 1 GBq, approximately 30 nmol) over a 6-mo period, the 111In-DOTA-lanreotide scintigraphy of the liver had returned to a nearly normal condition and a remarkably decreased uptake by the recurrent gastrinoma was calculated (approximately 5 mGy/MBq for 90Y-DOTA-lanreotide). The imaging results were well-correlated with a 25% regression of the liver metastases as indicated by CT. Blood, urine and whole-body clearances of 111In-DOTA-lanreotide and 90Y-DOTA-lanreotide were very similar. The DOTA-lanreotide promises to be useful for functional tumor diagnosis (111In-DOTA-lanreotide) and receptor-mediated tumor radiotherapy (90Y-DOTA-lanreotide).