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2022-RA-1501-ESGO Neuroendocrine tumors of the female genital tract; a rare entity
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  1. Dimitrios Giannoulopoulos1,
  2. Sofia Lekka1,
  3. Kalliopi Kokkali1,
  4. Victoria Psomiadou1,
  5. Gabriela Stanc2,
  6. Dimitrios Korfias1,
  7. Panagiotis Giannakas1,
  8. Christos Iavazzo1 and
  9. George Vorgias1
  1. 1Gynaecological Oncology Department, Metaxa Cancer Hospital, Piraeus, Greece
  2. 2Pathology Department, Metaxa Cancer Hospital, Piraeus, Greece

Abstract

Introduction/Background Neuroendocrine tumors (NETs) of the female genital tract are a very unusual clinical entity, with most known cases involving the uterine cervix. Those tumors include small and large-cell neuroendocrine carcinomas and carcinoid tumors. Interestingly small cell cervical cancer is a non-pulmonary variation of small-cell lung cancer. Moreover, metastatic pancreatic NETs to the ovaries are exceedingly scarce, with only three other cases to be found in literature. We present five gynaecologic NET cases.

Methodology The first patient presented with advanced disease; biopsy revealed small-cell cervical NET. She received primary chemoradiation, systemic chemotherapy and immunotherapy. The second patient was diagnosed with cervical NET via biopsy, without having completed the prescribed imaging studies. The third patient was diagnosed with large-cell cervical NET, stage IVb. The fourth patient presented with an adnexal mass, ascites and diarrhea; biopsy of the ovary revealed metastatic VIPoma without visible pancreatic pathology in the imaging studies. The fifth patient, presented with frozen pelvis and distant metastases. Cervical biopsy yielded the diagnosis of cervical NET with both small-cell and large-cell component.

Results The first patient has had a progression free survival of 24 months despite metastatic disease at diagnosis. The second patient eventually sought for medical care elsewhere due to long distance/personal reasons. The third patient was referred to palliative care, however she was lost to the follow up. The fourth patient was offered a left adnexectomy; nevertheless she did not follow through as she passed out due to cardiac problems. The fifth patient received chemotherapy, but succumbed to the disease 10 months after diagnosis.

Conclusion Diagnosis of a cervical NET at an early stage is of paramount importance, because of the worse prognosis of the disease compared to squamous cell cervical cancer. Moreover, VIPomas can metastasise to the ovaries presenting as an extremely rare diagnosis.

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