A serotonin and βHCG‐producing islet cell carcinoma associated with focal nodular hyperplasia of the liver

Abstract

A 48-year-old woman is described with a pancreatic islet cell carcinoma that had no clinical evidence of hormone production. This neoplasm was removed by radical distal pancreatectomy. Immunohistochemical and extraction studies performed on the tumor tissue revealed that it was producing serotonin and beta HCG. Circulating levels of these hormones were elevated in preoperative serum samples that had been frozen. Beta HCG, a known tumor marker, has remained normal following operation, and this mirrors her clinical course since she has no evidence of disease 1.5 yr after operation. This patient also had focal nodular hyperplasia of the liver. It is possible that the trophoblastic hormone, beta HCG, which was being produced in excess by the islet tumor, may have directly or indirectly caused or facilitated the development of focal nodular hyperplasia.

Primary carcinoid tumor of the pancreas

“…Primary EC cell carcinoid tumors are only rarely found in the pancreas. Review of the English literature revealed only 13 published cases of carcinoid tumor of the pancreas [2][3][4][5][6][7][8][9][10][11][12][13][14]. The endocrine-secreting activity and clinical symptoms varied in all these patients.…”

“…The endocrine-secreting activity and clinical symptoms varied in all these patients. All but one [9] presented with some degree of carcinoid syndrome. In two cases [12,14], pancreatitis resulted from obstruction of the pancreatic duct by the tumoral mass.…”

Surgical treatment of non‐functioning pancreatic islet cell tumors

“…Non-functioning PETs can also secrete a variety of peptide hormones, proteins and glycoproteins, including PP [55][56][57][58], CgA [59][60][61], NSE [62,63], and the alpha (a-) or beta (b-) subunits of hCG [59][60][61][62][63][64][65]. CgA is considered the best tumor marker currently available for the evaluation and follow-up of patients with non-functioning PETs, as these tumors do not reliably produce any other suitable marker [59].…”

Pancreatic Carcinoid: A Rare Cause of Diarrheogenic Syndrome

“…7,8 Carcinoid tumors are immunohistochemically positive for the pan-endocrine markers, neuron-specific enolase and chromogranin A, and also for antiserotonin antibodies, which are considered specific markers for such tumors. 3,4,8,9 When immunohistochemical techniques are positive for serotonin in the tumor cells, the tumor can be classified as a carcinoid tumor. 3,7 In the present case, the immunohistochemical study of the neoplastic tissue exhibited reactivity to serotonin, but also to glucagon and somatostatin, among others.…”

“…The clinical features of VIPoma are a severe but intermittent diarrhea, often of a watery nature. A true carcinoid tumor of the pancreas accounts for less than 1% of gastrointestinal carcinoids [2][3][4][5][6][7][8][9] and generally has a poor prognosis. [8][9][10][11] The most frequent symptoms found in patients with pancreatic carcinoids are abdominal pain and diarrhea.…”