Primary Ovarian Carcinoid: Extensive Clinical Experience With an Underrecognized Uncommon Entity

Preda, Veronica; Chitoni, Marina; Talbot, Dennis; Reed, Nicholas; Grossman, Ashley

doi:10.1097/igc.0000000000001215

Cited by 16 publications

(36 citation statements)

References 26 publications

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“…Serotonin is produced in large amounts by NEN cells, most commonly in patients with NENs in the small intestine with multiple liver metastases, and less frequently in patients with lung NENs. More rarely, patients with direct tumour drainage into the central circulation such as ovarian carcinoids (11), or with widespread peritoneal disease, may also present with CS. Serotonin is a tryptophan-derived biogenic amine which is synthesised and stored mainly in enterochromaffin cells of the gastrointestinal tract and also in dense granules of platelets (storage only), and in the serotoninergic neurons of the central nervous system.…”

Section: Introductionmentioning

confidence: 99%

Measurement of urinary 5-HIAA: correlation between spot versus 24-h urine collection

Calanchini

Tadman

Krogh

et al. 2019

Endocrine Connections

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Background The 24-h urinary output of 5-hydroxyindoleacetic acid (5-HIAA) is used to monitor disease progression and treatment responses of neuroendocrine neoplasms (NENs). Several conditions are required for 5-HIAA assay, involving urine collection/preservation and food/drug restrictions. Aim To evaluate the correlation between 5-HIAA concentration in a spot urine sample and the output in a 24-h urine collection, and whether spot urine specimens can replace 24-h collection. Methods Patients with NENs or symptoms suggestive of NENs were asked to provide a separate spot urine at the end of the 24-h urine collection for 5-HIAA assessment. The upper reference limit for 24-h urinary 5-HIAA was 40 µmol/24 h. 5-HIAA measurements in spot urine samples were corrected for variation in urine flow rate by expressing results as a ratio to creatinine concentration. Results We included 136 paired urinary samples for 5-HIAA assessment from 111 patients (100 NENs). The correlation between 5-HIAA values measured in 24-h and spot urines was r = +0.863 (P < 0.001) and r = +0.840 (P < 0.001) including only NEN patients. Using the 24-h urinary 5-HIAA as reference method, the AUC on ROC analysis for spot urinary 5-HIAA was 0.948 (95% CI, 0.914–0.983; P < 0.001), attaining a sensitivity of 83% and specificity of 95% using 5.3 mol/mmol as cut-off for the spot urine. The AUC among NEN patients alone was 0.945 (95% CI, 0.904–0.987; P < 0.001). Conclusions The ratio of 5-HIAA to creatinine in a spot urine could replace the measurement of 5-HIAA output in a 24-h urine collection, especially for follow-up of patients with known elevated 5-HIAA levels.

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Section: Introductionmentioning

confidence: 99%

Measurement of urinary 5-HIAA: correlation between spot versus 24-h urine collection

Calanchini

Tadman

Krogh

et al. 2019

Endocrine Connections

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“…Multiple agents including streptozocin, doxorubicin, 5-fluorouracil (5-FU), dacarbazine, actinomycin-D, bleomycin, cisplatin, etoposide, temozolomide, capecitabine, carboplatin and paclitaxel have been utilized individually and in various combinations for advanced NETs with variable success (objective response rates: 0–70%) (7). In a recent case series of patients with primary ovarian NETs, five patients received treatment with chemotherapy (varying protocols including doxorubicin or capecitabine or capecitabine, etoposide, platinum and temozolomide or cisplatin, 5-fluorouracil, and streptozocin); however, individual outcome data for these patients were not reported (17). Given significant toxicity and modest benefit, chemotherapy is usually reserved for situations when the patient has already received or is not eligible for above mentioned targeted therapies and SSAs.…”

Section: Discussionmentioning

confidence: 99%

Clinical presentation and management of primary ovarian neuroendocrine tumor in multiple endocrine neoplasia type 1

Jhawar

Lakhotia

Suzuki

et al. 2019

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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Summary Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant condition characterized by parathyroid, anterior pituitary and enteropancreatic endocrine cell tumors. Neuroendocrine tumors occur in approximately in 5–15% of MEN1 patients. Very few cases of ovarian NETs have been reported in association with clinical MEN1 and without genetic testing confirmation. Thirty-three-year-old woman with MEN1 was found to have right adnexal mass on computed tomography (CT). Attempt at laparoscopic removal was unsuccessful, and mass was removed via a minilaparotomy in piecemeal fashion. Pathology showed ovarian NET arising from a teratoma. Four years later, patient presented with recurrence involving the pelvis and anterior abdominal wall. She was treated with debulking surgery and somatostatin analogs (SSAs). Targeted DNA sequencing analysis on the primary adnexal mass as well as the recurrent abdominal wall tumor confirmed loss of heterozygosity (LOH) at the MEN1 gene locus. This case represents to our knowledge, the first genetically confirmed case of ovarian NET arising by a MEN1 mechanism in a patient with MEN1. Extreme caution should be exercised during surgery as failure to remove an ovarian NET en masse can result in peritoneal seeding and recurrence. For patients with advanced ovarian NETs, systemic therapy options include SSAs, peptide receptor radioligand therapy (PRRT) and novel agents targeting mammalian target of rapamycin (mTOR) and vascular endothelial growth factor (VEGF). Learning points: Ovarian NET can arise from a MEN1 mechanism, and any adnexal mass in a MEN1 patient can be considered as a possible malignant NET. Given the rarity of this disease, limited data are available on prognostication and treatment. Management strategies are extrapolated from evidence available in NETs from primaries of other origins. Care should be exercised to remove ovarian NETs en bloc as failure to do so may result in peritoneal seeding and recurrence. Treatment options for advanced disease include debulking surgery, SSAs, TKIs, mTOR inhibitors, PRRT and chemotherapy.

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“…Los tumores neuroendocrinos del ovario suelen ser metástasis de un tumor de origen gastrointestinal. Los tumores carcinoides primarios de ovario fueron descritos por Stewart en 1939 (1) . Son tumores muy poco frecuentes, representan el 1% de los tumores carcinoides y < 0,1% de los carcinomas ováricos (2) .…”

Section: Introductionunclassified

“…Estos tumores se caracterizan por la liberación de sustancias vasoactivas como la serotonina, taquiquininas y prostaglandinas, que normalmente son inactivadas por el hígado (5)(6)(7) . El aumento de estas sustancias ocasiona el síndrome carcinoide, que se caracteriza por rubor cutáneo, diarrea, broncoespasmo e hipotensión (1,2,5) .…”

Section: Introductionunclassified

Tumor carcinoide primario de ovario que se presenta como enfermedad cardiaca carcinoide

Albareda

Meo

Merinero

et al. 2020

Rev peru ginecol obstet.

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El tumor carcinoide primario de ovario es una entidad poco frecuente que se caracteriza por la producción de sustancias vasoactivas que pasan directamente al torrente circulatorio y producen el llamado síndrome carcinoide. Estas sustancias también son las causantes de la formación de placas de fibrosis en las válvulas cardiacas, principalmente las derechas. Presentamos un caso de tumor carcinoide primario de ovario con afectación de la válvula tricúspide, que fue diagnosticado a partir de la lesión cardiaca. En la literatura están descritos casos clínicos aislados y revisiones de casos que nos pueden ayudar en el diagnóstico y en la toma de decisiones terapéuticas. Debemos conocer la existencia de estos tumores; la clínica debe alertarnos sobre su presencia. Es primordial un manejo multidisciplinar junto con endocrinólogos, cardiólogos, oncólogos, anatomo-patólogos, anestesistas, ginecólogos y cirujanos cardiacos, para ofrecer un tratamiento completo.

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Primary Ovarian Carcinoid: Extensive Clinical Experience With an Underrecognized Uncommon Entity

Abstract: Primary ovarian NET is rare and likely underrepresented in the literature. It can no longer be simply dismissed as a generally benign entity and may present with both the carcinoid syndrome and carcinoid heart disease. It requires careful evaluation and long-term review.

Cited by 16 publications

References 26 publications

Measurement of urinary 5-HIAA: correlation between spot versus 24-h urine collection

Measurement of urinary 5-HIAA: correlation between spot versus 24-h urine collection

Clinical presentation and management of primary ovarian neuroendocrine tumor in multiple endocrine neoplasia type 1

Tumor carcinoide primario de ovario que se presenta como enfermedad cardiaca carcinoide

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