PubMed · 6123087
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F Schwarz. 1982-03-13. [Multiple endocrinopathies].. https://pubmed.ncbi.nlm.nih.gov/6123087/
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A rare case of Frantz's tumor with liver metastasis is reported. An 11-year-old girl had local resection of a Frantz's tumor in the head of the pancreas. Ten years later, she had a recurrence in the pancreatic head, and two liver metastases were also disclosed at reoperation. Pancreaticoduodenectomy and enucleation of metastatic lesions in the liver were performed. Six years after the second operation, she is well and free of disease. A review of 174 cases of Frantz's tumor disclosed that metastatic disease in the pediatric age group is extremely rare and yet the prognosis is favorable. On the other hand, the outcome in older patients, especially those over 40 years old, is very poor.
The plasma levels of chromogranin A (CGA) in patients with islet cell tumor and plasma CGA responses to administration of a somatostatin analogue (Octreotide) in two of these patients were examined in comparison with plasma pancreastatin (PST) levels. There was a significant correlation between the fasting plasma levels of CGA and PST (r = 0.6, P < 0.001). Administration of the somatostatin analogue reduced the plasma concentrations of PST and CGA within 1 h, but the responses of CGA and PST to the analogue were not parallel in either patient. Thus, the suppressive effects of the analogue on the secretions of PST and CGA may be different. The results suggest the value of the PST and CGA assays used in this study.
Adrenocorticotropic hormone (ACTH) secreting islet cell tumors of the pancreas are extremely rare. A case of an ACTH producing islet cell tumor with multiple liver metastases is reported and the magnetic resonance imaging (MRI) findings are described.