Treatment of a mixed acinar-endocrine carcinoma with uptake on 68Gallium-DOTATOC positron emission tomography-computed tomography: A case report

De Both,Anneleen; De Man,Marc; Troisi,Roberto; Van Vlierberghe,Hans; Hoorens,Anne; Geboes,Karen

doi:10.3892/ol.2017.6242

Treatment of a mixed acinar-endocrine carcinoma with uptake on 68Gallium-DOTATOC positron emission tomography-computed tomography: A case report

Authors:
- Anneleen De Both
- Marc De Man
- Roberto Troisi
- Hans Van Vlierberghe
- Anne Hoorens
- Karen Geboes
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Affiliations: Department of Gastroenterology and Digestive Oncology, Ghent University Hospital, 9000 Ghent, Belgium, Department of General, Hepato‑Biliary and Liver Transplantation Surgery, Ghent University Hospital, 9000 Ghent, Belgium, Department of Gastroenterology and Hepatology, Ghent University Hospital, 9000 Ghent, Belgium, Department of Anatomopathology, Ghent University Hospital, 9000 Ghent, Belgium
Published online on: May 24, 2017 https://doi.org/10.3892/ol.2017.6242
Pages: 547-552
Copyright: © De Both et al. This is an open access article distributed under the terms of Creative Commons Attribution License.

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Abstract

The case of a 35-year old female patient with a diagnosis of metastatic mixed acinar-endocrine carcinoma (MAEC) is investigated in the present study. The patient was believed to have a well‑differentiated neuroendocrine tumor (NET) with a high Ki‑67 index and uptake on 68Gallium‑DOTATOC positron emission tomography‑computed tomography for 9 years, and was treated accordingly. The patient had long lasting disease control by treatment with sunitinib, and a response was observed in numerous lesions with peptide receptor radionuclide therapy (PRRT). Following treatment for metastatic disease for >4 years, liver transplantation was performed, as an exception to normal recommendations, at the time of progression of a centrally located liver lesion inducing obstructive jaundice. Following transplantation, the diagnosis of a Grade 3 NET, as defined by the WHO 2010 classification, was challenged and changed to MAEC. MAEC is a rare type of tumor of the pancreas, exhibiting endocrine and acinar differentiation. It is difficult to diagnose, often being misidentified as acinar cell carcinoma or predominantly as neuroendocrine neoplasms. Immunohistochemical labelling provides the only evidence for the dual differentiation of neuroendocrine (synaptophysin and chromogranin) and acinar (lipase, trypsin and chymotrypsin) cell markers. Studies investigating MAECs with a clear histopathological diagnosis are scarce, in addition to evidence of disease behaviour and treatment options. It is generally agreed that surgery is the primary treatment in patients with resectable tumors. The responses to sunitinib and PRRT suggested that treatments considered or developed for NETs may be beneficial in MAEC cases.

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