Table 5.

Suggestions for the clinical management of patients with strumal carcinoids.

Pre-operative diagnosis • Hormone work-up: YY evaluation in case of ovarian mass and severe constipation or specific hormone assessment such as insulin, glucagon, thyroid hormones, androgens, serum chromogranin A and urinary 5-hydroxy-indolil acetate according to concurrent specific syndromes. • An appropriate staging should be carried out in presence of lesions of the ovary: transvaginal ultrasound/abdominal ultrasound should be the first step, followed by CT scan of the thorax-abdomen and/or abdominal MRI. • In case of symptoms compatible with ovarian carcinoid confirmed by the hormonal assessment 68Ga PET could be useful for further diagnostic confirmation.

Primary tumor management • Surgery is the mainstay of treatment. If the diagnosis of ovarian carcinoid is suspected by the specific pre-operative symptoms, a conservative intervention should be planned particularly in the case of a pre-menopausal patient. • Other treatment strategies (radiotherapy, chemotherapy) are not advisable both in neoadjuvant and adjuvant setting.

Pathologic diagnosis • The criteria for the diagnosis of thyroid and neuroendocrine tumors should be adopted to define the strumal and carcinoid components.

Follow-up, long-term implications and survivorship • Since few patients may experience disease relapse after surgery, follow-up with periodic clinical visits and ultrasound should be implemented. • Follow-up could include blood chemistry tests such as thyroglobulin and anti-thyroid antibodies or tests concerning the neuroendocrine component, such as YY peptide, glucagon, insulin, 5-hydroxy indolyl acetate if they were elevated in the pre-operative assessment. • CT and MRI should be prescribed in case of suspected disease progression based on clinical and laboratory findings and/or ultrasound data. • the duration of the follow-up cannot be estimated, it is reasonable that it can be at least 5 years.

Management of advanced/recurrent disease • In case of local recurrence, salvage surgery should always be considered. • If disease recurrence is not amenable to surgery, it should be hormonally and/or histologically studied to determine which compound (carcinoid or thyroid) drive the disease. • As regard as the systemic therapy, specific international guidelines for NETs and thyroid tumors should be followed in case of recurrence of the carcinoid or thyroid component, respectively.