Aggressive neuroendocrine tumor of the ovary with multiple metastases treated with everolimus: A case report

Kaiho-Sakuma, Michiko; Toyoshima, Masafumi; Watanabe, Mika; Toki, Asami; Kameda, Satomi; Minato, Takahiro; Niikura, Hitoshi; Yaegashi, Nobuo

doi:10.1016/j.gore.2018.01.002

Cited by 12 publications

(20 citation statements)

References 20 publications

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“…Chemotherapy could be used in late stage OC. [56] In our study, Case 1 and Case 2 were classified as stage I and stage III, respectively, without and with chemotherapy conducted postoperatively. Satisfactory results were achieved in both patients during follow-up visit.…”

Section: Discussionmentioning

confidence: 98%

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Primary ovarian carcinoid

Zhai¹,

Zhang²,

Zheng³

et al. 2020

Medicine

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Introduction: Carcinoid tumor is one of the most frequent neuroendocrine tumors, and the majority of which are usually observed in the lungs and gastrointestinal tract. The prevalence of ovarian carcinoids is merely 0.1% in ovarian neoplasms and 1% in carcinoid tumors. We described 2 rare cases in our hospital of primary ovarian carcinoid (POC), causing carcinoid syndrome (CS) of the diarrhea, constipation, and carcinoid heart disease. Besides, we also reviewed related literatures about its origin, variant, clinical manifestation, diagnosis methods, pathological features, treatment strategies and prognosis from 2009 to 2019. Patient concerns: Case 1 was a 61-year-old postmenopausal woman and presented with diarrhea, abdominal pain, enlargement, bloating and dizziness. Case 2 was a 49-year-old patient who complained of constipation, abdominal pain, bloating, and headache. Diagnosis: Both patients were diagnosed as primary ovarian carcinoid, insular type. Interventions: Total abdominal hysterectomy (TAH), bilateral salpingo-oophorectomy (BSO), omentectomy, pelvic lymphadenectomy, and appendectomy without chemotherapy were performed in case 1. Cervix resection, right salpingo-oophorectomy, appendectomy, and pelvic lesion resection with chemotherapy was conducted in case 2. Outcomes: Both patients achieved satisfactory treatment effects. The follow-up period was 18 and 17 months in case 1 and case 2, respectively. Case 1 encountered carcinoid heart disease and received percutaneous transluminal coronary angioplasty (PTCA) postoperatively. Case 2 suffered multiple metastases postoperatively. However, after effective treatment, both patients were in good condition during follow-up duration. Conclusion: POC is an extraordinarily rare disease, and commonly with a satisfactory outcome. TAH+BSO with or without postoperative chemotherapy has been considered as an acceptable treatment strategy for POC patients.

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Section: Discussionmentioning

confidence: 98%

“…Recently, some molecular medications were reported to help survival in gastrointestinal and pancreatic carcinoids, and is also proved to be useful in OC. [ 3 56 ]…”

Section: Discussionmentioning

confidence: 99%

Primary ovarian carcinoid

Zhai¹,

Zhang²,

Zheng³

et al. 2020

Medicine

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“…A highly positive Ki-67 is indicative of a poor prognosis. These tumors are negative for epithelial membrane antigen (EMA), estrogen, and progesterone receptors and sex-cord stromal markers such as inhibin and calretinin [ 1 ]. Immunohistochemistry has become an essential examination for the identification and differential diagnosis of neuroendocrine carcinomas with other primary or metastatic tumors.…”

Section: Discussionmentioning

confidence: 99%

“…Ovarian NETs (neuroendocrine tumors) accounting for only 0.5% of all NETs and 0.1% of all ovarian cancers [ 1 ] are associated with a conventional surface epithelial tumor or a teratoma. Pure ovarian NETs (47%) are usually larger in size and present higher rates of metastases and carcinoid syndrome [ 2 – 4 ].…”

Section: Introductionmentioning

confidence: 99%

Mixed Large Cell Neuroendocrine Carcinoma of the Ovary: Report of a Rare Case

Galanis

Floros

Bompoula

et al. 2020

Case Reports in Obstetrics and Gynecology

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LCNC (large cell neuroendocrine carcinomas) of the ovary is a rare aggressive tumor entity of the genital tract. Its pathogenesis, origin, and prognosis have not been fully elucidated, since there are a limited number of cases reported in literature. We report a case of an 80-year-old patient, who presented with a growing abdominal mass, which turned out to be a mixed LCNC/epithelial neoplasm. Although this type of tumor is rare, the continuous development of pathologoanatomy and immunohistochemistry contributes to our better knowledge and comprehension of these neoplasms.

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“…A summary of clinical studies with targeted agents in the treatment of advanced NETs is provided in Table 1. In a recently published case report, treatment with everolimus, an mTOR inhibitor, of a patient with right ovarian intermediate grade NET with liver metastases resulted in stable disease for more than 7 months, after the patient previously progressed on bleomycin, etoposide and cisplatin chemotherapy (16).…”

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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Aggressive neuroendocrine tumor of the ovary with multiple metastases treated with everolimus: A case report

Cited by 12 publications

References 20 publications

Primary ovarian carcinoid

Primary ovarian carcinoid

Mixed Large Cell Neuroendocrine Carcinoma of the Ovary: Report of a Rare Case

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

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