Introduction: Mixed neuroendocrine and nonneuroendocrine neoplasms (MiNENs) are the rarest neuroendocrine appendiceal neoplasms, for which no standard treatment guidelines are available. Here, we present a case report of a pelvic MiNEN.
Case description:A 41-year-old woman underwent clinical evaluation to determine the causes of her infertility. Medical tests revealed a massive cystic mass in the left pelvis and a small fluid-filled nodule originating from the appendix. Fertility-sparing surgery evidenced an appendiceal MiNEN, with a low-grade neuroendocrine component in the context of a low-grade mucinous neoplasm (LAMN). Pathological stage was pT4 N0 M1 (according to the AJCC VIII ed.), as the tumour reached the visceral peritoneum, showing no metastatic lymph node involvement. A single metastatic nodule expressing neuroendocrine features was found in the omentum. Instrumental re-evaluation performed after surgery revealed two additional abdominal spots, suspicious for metastases. After multidisciplinary discussion, a second surgery was performed, followed by intraperitoneal intraoperative hyperthermia (HIPEC). Three months later, CT scan revealed a neoformation between the stomach and the pancreas tail, suspicious for disease progression. Surgical biopsy was deemed unfeasible. Since LAMN was the main component of the pelvic mass previously removed, the patient underwent oral fluoropyrimidine-based chemotherapy. Simultaneously, patient was treated with somatostatin analogues (SAs), after assessing a moderate 68Ga-PET scan positivity. The patient is currently on treatment, alive and progression-free after 28 months.
Conclusion:Our case highlights the importance of multidisciplinary approach and literature review to guarantee the most appropriate management of rare diseases such MiNENs, underlining the unmet need of common guidelines.