RESPONSES TO SEQUENTIAL SURGERY AND INTERFERON THERAPY OF THE NEUROENDOCRINE PANCREATIC CANCER METASTASES IN A FEMALE PATIENT WITH VON HIPPEL-LINDAU DISEASE
Authors: DEMIDOV ALEXEY A. ,   Astrakhan State Medical University, SLUVKO LEON V. , DYAKOVA OLGA N. , VOROBYOVA ANNA A. , FEDOTOVA ANNA D. , GORBUNOVA OKSANA E. , PANOVA TAMARA N. ,   Astrakhan State Medical University,   Astrakhan State Medical University,   Astrakhan State Medical University,   Russian Scientific Center for Radiology, Moscow,   Astrakhan State Medical University,   Astrakhan State Medical University
Published: July 2024

Abstract. Introduction. The article provides a literature review on the epidemiology, diagnosis, and treatment of neuroendocrine tumors. The morpho-functional classification by WHO 2022 is presented with the division of neuroendocrine tumors into three groups. Justifications are given for the choice of instrumental methods of topical diagnostics and the need for determining universal and specific biochemical markers in blood serum or plasma. The principles are indicated for choosing the advanced methods of treating neuroendocrine tumors. Aim. To review the scientific literature on neuroendocrine tumors, providing our own clinical observations. Materials and Methods. Russian- and English-language literature sources on this disease were searched for in the databases, such as PubMed, Medscape, Cochrane Library, and eLibrary. Clinical case of our female patient V., 36 years old, with von Hippel-Lindau disease (heterozygous mutation R167W of the VHL gene). Results and Discussion. As an example, we describe a clinical case of surgical treatment followed by long-term use of interferons in a female patient with neuroendocrine pancreatic cancer with metastases to the liver and retroperitoneal lymph nodes. Histological examination of the surgical material revealed highly differentiated neuroendocrine cancer with low malignant potential. A screening examination of the genetic syndrome components revealed a heterozygous mutation R167W of the VHL gene. Her survival under treatment exceeded 9.5 years, including more than 4.5 years from the start of interferon therapy. This pathology occurred in a young woman with one of the rare genetic multiorgan hereditary forms of neuroendocrine tumors, Von Hippel-Lindau disease that manifested itself as a combination of benign (retinal angiomatosis of both eyes and adenomas of both adrenal glands) and malignant diseases (pancreatic cancer). Conclusions. The given clinical example demonstrates that in a patient with highly differentiated metastatic pancreatic cancer, after distal subtotal resection of the pancreas, splenectomy, retroperitoneal lymphadenectomy, followed by treatment with interferons, life expectancy was more than 9.5 years, including more than 4.5 years from the start of therapy with interferons only. Despite the numerous side effects of interferons described in the literature, the patient tolerated the treatment well. At the same time, a connection between the hypothyroidism detected in the patient and treatment with interferons cannot be ruled out, since dysfunction of the thyroid gland is one of its side effects.