Giant stromal ovarian carcinoid with distant metastasis to the right atrium: a case report

ObjectiveTo investigate the clinicopathological features, diagnosis and differential diagnosis of patients with primary ovarian carcinoid tumours arising in mature cystic teratomas.MethodsThis retrospective case series analysed the data from patients with primary ovarian carcinoid tumours arising in mature cystic teratomas.ResultsThe study enrolled four patients. Histopathological analysis of the tumours identified the following subtypes: insular (n = 1), trabecular (n = 1) and strumal (n = 2). All four primary ovarian carcinoid tumours originated from a mature teratoma. The morphology of the primary ovarian carcinoids was similar to other neuroendocrine tumours. Strumal carcinoids were composed of different proportions of thyroid tissue intimately admixed with carcinoid tumour. Tumour tissue was arranged in insular and/or trabecular patterns. The nucleus of tumour cells displayed exquisite chromatin without obvious mitotic figures. Tumour tissues were positively stained for neuroendocrine markers chromogranin A, synaptophysin and CD56 to varying degrees. Strumal carcinoid tumours were cytokeratin 19 positive and thyroid transcription factor 1 negative. No recurrence or metastasis occurred during follow-up (12–71 months).ConclusionPrimary ovarian carcinoid tumours arising in mature cystic teratomas are rare. Diagnosis and differential diagnosis should be confirmed by clinical features, histopathological characteristics and specific immunophenotyping.

E-cadherin nuclear staining is useful for the diagnosis of ovarian adult granulosa cell tumor

Objective: To describe the clinicopathologic features, diagnosis and differential diagnosis of ovarian carcinoid tumors. Methods: A retrospective chart review was performed of all patients diagnosed with primary ovarian carcinoid tumors at Fudan University Shanghai Cancer Centre from 2007 to 2017. Results: The histologic analysis of these carcinoid tumors revealed 3 were insular, 1 was trabecular, 1 was mucinous, and 10 were strumal. Histologic features of insular and trabecular carcinoid were similar to other parts of the neuroendocrine tumor. Strumal carcinoid was composed of thyroid tissue intimately admixed with carcinoid tumor, showing trabecular pattern. Mucinous carcinoid was resembles Krukenberg tumor. Most ovarian carcinoid tomours were diffusely positive with at least one neuroendocrine marker, especially synaptophysin (14/14) and CD56(9/10). The median follow-up time was 53 months, 1 patient with squamous-cell carcinoma of cervixrecur rence in vaginal after 37 months, and only 1 patient died of disease. The remaining patients were disease-free survival. Conclusions: Primary carcinoid of the ovary is a very rare low grade malignant monodermal teratomas and somatic-type tumours arising from a dermoid. The diagnosis and differential diagnosis mainly relies on the histopathologic characteristics and the immuno-phenotype. Primary ovarian carcinoid almost always exhibit a benign clinical behavious except mucinous carcinoid.

Carcinoid tumors of the ovary are rare neoplasms that may be primary or metastatic. Clinicopathologic features such as unilaterality and early stage favor a primary ovarian neoplasm but in the absence of other teratomatous elements it may be difficult or impossible to determine whether an ovarian carcinoid is primary or metastatic. CDX-2 is a marker of intestinal differentiation that has been proposed as a marker of midgut origin for metastatic carcinoids. Its expression has not been tested in ovarian carcinoids. Additional markers of potential help in defining the origin of a carcinoid include cytokeratin (CK) 20, CK7, and thyroid transcription factor (TTF-1), none of which have been studied in ovarian carcinoids. We evaluated the diagnostic utility of CDX-2, CK20, CK7, and TTF-1 as well as conventional clinicopathologic features in determining the site of origin in 26 ovarian carcinoids (16 primary and 10 metastatic from midgut). Non-neoplastic premenopausal ovaries (n=10) served as controls. All primary ovarian carcinoids were unilateral whereas only 3/10 metastatic carcinoids were unilateral. Multinodular growth occurred in 6/10 metastatic carcinoids but not in any primary carcinoid. The average size of primary ovarian carcinoids was 3.4 cm (range: 0.2-13.5 cm) versus 10.2 cm for metastatic carcinoids (range: 4-32 cm). Of the primary ovarian carcinoids, 12/16 were 3 cm or smaller whereas all metastatic carcinoids were 4 cm or larger. Teratomatous elements were present in association with 10/16 primary ovarian carcinoids, whereas none were present in any metastatic carcinoid. The primary ovarian carcinoid types were insular (n=6), trabecular (n=3), strumal (n=6, of which 5 were trabecular pattern and 1 was insular pattern) or mucinous (n=1). CDX-2 was not expressed in any cells in normal ovaries. Among primary ovarian neoplasms, there was diffuse nuclear CDX-2 expression in 4/6 insular, 0/3 trabecular, 1/6 strumal (1/1 insular pattern and 0/5 trabecular pattern strumal carcinoids), and 1/1 mucinous carcinoids. All metastatic carcinoids, except for two of mucinous type, were insular. CDX-2 was diffusely and strongly expressed in all 8 metastatic insular carcinoids and in both metastatic mucinous carcinoids. None of the metastases was trabecular in type but 12 primary hindgut or foregut trabecular carcinoids were evaluated and all were negative for CDX-2. None of the ovarian carcinoids expressed TTF-1, CK7, or CK20, except for the primary and metastatic mucinous carcinoids, all of which were CK20-positive. These results demonstrate that CDX-2 cannot be used to determine if a carcinoid is primary in the ovary or metastatic from the intestine as insular and mucinous types of either origin express this marker. Trabecular carcinoids of either origin lack CDX-2 expression. CK20, CK7, or TTF-1 do not have diagnostic utility in this context. Conventional clinicopathologic features (unilaterality, lack of multinodular growth, early stage, presence of teratomatous elements, and size 3 cm or smaller) are the most helpful findings in suggesting a primary origin for an ovarian carcinoid tumor.

Objective To investigate the clinicopathological features of ovarian malignant tumor with thyroid components. Methods The clinical and pathological data of ovarian malignant tumor with thyroid component were collected from May 2006 to March 2018, including four cases of stroumal carcinoid and one case of metastatic thyroid follicular carcinoma, and the related literature was also reviewed. Results Ages of 5 cases ranged from 25 to 65 years old, and pelvic mass or abdominal discomfort was presented in physical examination. Tumors were unilateral, and mainly on the left side. The diameter of tumors was 5 to 13 cm with cyst or solid mainly. The strumal and carcinoid components were seen in strumal carcinoid. The insular and trabecular carcinoid were the common types of carcinoid. The synaptophysin (Syn) and chromogranin (CgA) were positive by immunohistochemistry. The patient with metastatic thyroid follicular carcinoma of ovary had a history of thyroid nodules for 13 years and the tumor grew rapidly in a short time. Under the microscope, dense thyroid follicles were arranged in papillary or diffuse distribution. Tumor cells were rich in eosinophilic cytoplasm and accompanied by follicular cell tumor-like hyperplasia. Conclusions Papillary tumor and strumal carcinoid are the most common primary malignant tumors of ovary with thyroid component, which both presented low malignancy and good prognosis. Ovarian metastasis from thyroid cancer is extremely rare and needs to be differentiated from primary ovarian thyroid cancer. Key words: Ovarian neoplasms; Thyroid gland; Carcinoma, papillary; Retrospective studies

Carcinoid tumors are neuroendocrine neoplasms that secrete vasoactive substances, primarily serotonin, and have an incidence of approximately 2 in 100,000 people. Up to 60% of carcinoid tumors result in carcinoid heart disease (CHD), and unlike other carcinoid tumors, ovarian carcinoid tumors can involve the heart without liver metastasis. By bypassing the liver, ovarian carcinoid tumors can result in CHD in the absence of liver metastasis. We present a rare case of a 40-year-old female who presented in cardiogenic shock requiring inotropic support with echocardiographic evidence of preserved left ventricular systolic function (LVEF 50-55%) and multiple retracted leaflets that exhibited poor coaptation resulting in severe mitral regurgitation, torrential tricuspid regurgitation, and moderate-to-severe aortic regurgitation. Her left heart catheterization revealed non-obstructive coronary artery disease. A non-contrast CT torso revealed an isolated large septated left ovarian cystic mass (7.1x5.2 x7.1cm) without any other mass or lesion. In the setting of an elevated CA-125 (536units/mL, normal 0-35units/mL) and 24-hour urinary 5-HIAA (12.1mg, normal 2-9mg) with a normal CEA, CHD was suspected as the most likely diagnosis given the isolated ovarian mass and severe valvular disease. She underwent triple valve surgery in which valvular pathology revealed myxoid degeneration. Her post-operative course was complicated by multi-organ failure requiring multiple inotropes and V-A ECMO shortly thereafter, but the patient unfortunately passed away. While there are a few documented cases of CHD from ovarian carcinoid (less than 0.5% of cases) and left-sided CHD (less than 10% of cases), this is the first documented case, to our knowledge, describing severe right and left-sided CHD associated with a suspected ovarian carcinoid tumor.

Introduction/BackgroundOvarian carcinoid tumors are rare neoplasms that account for 0.8–1.2% of all ovarian cancer. In 15% of cases there is a mature teratoma on the contralateral ovary. Suspicion prior to...

Ovarian carcinoid tumors are uncommon and account for 1% of all carcinoid tumors. The insular type of ovarian carcinoid tumor is common in western countries; in contrast, the strumal and trabecular types seem to be common in Asian countries. Strumal and trabecular types are associated with peptide YY (PYY) production, which may cause constipation. Here, we report the case of a 70-yr-old Japanese woman with chronic constipation who was referred to Kumamoto University Hospital because of a right adnexal mass. Imaging tests suggested that the solid mass might be malignant; therefore, abdominal total hysterectomy, bilateral salpingo-oophorectomy, and omentectomy were performed. A subsequent histopathologic examination confirmed an insular carcinoid tumor with a trabecular component in the right ovary. Both components were positive for PYY but not for serotonin. The patient complained of diarrhea instead of constipation soon after the surgery. Because PYY-positive insular carcinoid tumor in the ovary has not been previously reported, we reviewed 19 reported cases of patients with PYY-positive ovarian carcinoid tumors. The origins, common histologic types and symptoms caused by specific peptides secreted in ovarian carcinoid tumors differ between western and Asian countries.

Carcinoid tumor of the ovary is a rare neoplasm that may present as a solid mass or combined with mucinous tumors or teratomas. Primary ovarian carcinoid represents <0.1% of ovarian malignancies. These tumors are often unilateral presenting as a solid mass and vary from microscopic to very large tumors. Metastatic ovarian carcinoid tumors are seen to be bilateral in most cases. Involvement of the ovary from gastrointestinal carcinoid is rare with no hepatic or peritoneal seedlings. Ovarian carcinoid tumors commonly occur in perimenopausal and postmenopausal women. Surgical removal of the tumor is the standard treatment modality. Tumor size and the presence of metastasis are necessary to plan the treatment modality. We herein report a case of carcinoid tumor of the ovary in a 55-year-old female, which we thought was a dermoid cyst of the ovary and turned out to be carcinoid after detailed immunohistochemical analysis.

Ovarian carcinoid tumors are very rare entities that often mimicother ovarian neoplasms. A case of primary ovarian carcinoid in a 44-year-old woman is presented with emphasis on the magnetic resonance imaging (MRI) features of the tumor and pathologic correlation. Ovarian carcinoid tumors can be variable in their MRI appearance, presumably due to different tumor subtypes and tumor components, thus requiring pathologic diagnosis. It is imperative to accurately diagnose primary ovarian carcinoid tumors, as their prognosis is usually more favorable compared to other malignant ovarian neoplasms.

To describe the clinical features, treatment, clinical course and survival rates of women diagnosed with ovarian carcinoid tumours. A retrospective chart review was performed of all patients diagnosed with primary ovarian carcinoid tumours who were managed by the Queensland Centre for Gynaecological Cancer from 1982 to 2015. Eighteen patients were identified with ovarian carcinoid tumours over the 32 years of the study period. Of the 18 patients, 14 were diagnosed with stage 1 disease, two were diagnosed with stage 3 disease and two were diagnosed with stage 4 disease. Carcinoid syndrome was present in two patients. All patients underwent surgical management. Follow-up strategies varied for early stage disease, but no patient with early stage disease received any adjuvant treatment and no patient developed recurrent disease. Patients with advanced stage disease were treated with cytoreductive surgery and chemotherapy. The five year survival was 100% for stage 1 disease, and 25% for stages 3 and 4 disease. The vast majority of carcinoid tumours are diagnosed as an incidental finding. Prognosis for early stage disease is excellent, whether conservative or more extensive surgery with staging was performed, and intensive follow up did not influence survival. Optimal treatment for advanced disease remains unknown and requires further study.

BackgroundThe exact characteristics of primary ovarian carcinoid tumors remain largely unknown because of the rarity of the cases. This study aimed to investigate the clinical features, pathological characteristics, and therapeutic outcomes of patients with primary ovarian carcinoid tumors.MethodsThis retrospective case series included patients with primary ovarian carcinoid tumors diagnosed between January 2009 and December 2023 at the First Affiliated Hospital of Wenzhou Medical University.ResultsFifteen patients were included. They were 45.8 ± 2.7 years at diagnosis. Eight tumors were located in the left ovary, while seven were in the right. All patients were stage I. Microscopically, nine tumors were classified as strumal carcinoid, two as insular carcinoid, three as trabecular carcinoid, and one as mixed. Synaptophysin (Syn) was positive in 14 cases, chromogranin A (CgA) in 10, CD56 in eight, thyroid transcription factor 1 (TTF-1) in five, and thyroglobulin (TG) in six. Twelve patients had a Ki67 index ≤ 7%. All 15 patients underwent surgery, with eight retaining fertility. Among them, one patient underwent comprehensive staging surgery, four underwent lateral adnexectomy, and three underwent cyst stripping. Seven patients underwent total hysterectomy and bilateral adnexectomy, including two patients undergoing comprehensive staging surgery. Three patients received intravenous chemotherapy. One patient was lost to follow-up. The remaining patients were followed up for 48–148 months; they were without recurrences and alive at the last follow-up.ConclusionsPrimary ovarian carcinoid tumors present with atypical symptoms and signs. Surgical intervention may be an optimal choice for treatment, leading to favorable prognostic outcomes.

Case report: Radiolabelled octreotide scanning as a guide to the management of an occult carcinoid tumour

Primary ovarian carcinoid tumours are uncommon neoplasias. There are distinct histological types with different behaviours: insular, trabecular, mucinous and mixed. The trabecular subtype is very rare and unlike other carcinoid subtypes, it is characterised by the absence of a clinical carcinoid syndrome and has been related with a better prognosis than the others. No distant metastases have yet been reported. We present a case of a 76-year-old woman diagnosed with a left ovarian tumour. She underwent a radical hysterectomy with bilateral salpingo-oophorectomy and regional lymph node clearance. Histology revealed an ovarian trabecular carcinoid tumour and no adjuvant treatments were performed. The patient is alive and free of disease 70 months following diagnosis. Immunohistochemical staining for p53 protein, and a comparative study with other subtypes of ovarian carcinoid tumours (insular and mucinous) is discussed in this report.

Primary carcinoid tumors are rare neoplasms of the ovary. Of the 4 histologic subtypes, ovarian carcinoid tumors with insular patterns produce carcinoid syndrome in approximately one third of cases, versus strumal and trabecular carcinoids which very rarely cause typical carcinoid syndrome. A unique presentation of ovarian carcinoid tumors with concurrent severe constipation has been reported, which is thought to represent a new carcinoid syndrome. The proposed mechanism is the production of peptide YY by the tumor, a gastrointestinal hormone responsible for decreasing gut motility. We report a case of a 34-yr-old white woman who presented with constipation and weight loss for 1 yr, and was found to have a unilateral ovarian strumal carcinoid, which produced peptide YY as demonstrated by immunohistochemistry. The 13 previous case reports of ovarian carcinoids with constipation are reviewed and the clinicopathologic features are discussed. This report and literature review further solidifies this entity as a new type of carcinoid syndrome.