Primary Ovarian Carcinoid Tumor Research Articles

Carcinoid Heart Disease Associated with Primary Ovarian Carcinoid Tumor: A Rare Presentation

Primary ovarian carcinoid tumors, an extremely rare subset of ovarian malignancies (<0.1% of cases), typically manifest with abdominal pain and bloating. The occurrence of carcinoid heart disease (CHD) without classic carcinoid syndrome features is exceptionally uncommon. We report a 54-year-old female presenting with dyspnea and edema over months, lacking typical carcinoid syndrome signs. Initial assessments diagnosed heart failure, supported by elevated NT-Pro BNP, echocardiographic right ventricular dilation, and pulmonary/tricuspid valve regurgitation (normal left ventricular function). Right heart catheterization showed normal pulmonary/right ventricular pressures. Subsequent investigations (computed tomography thorax abdomen pelvis, 5-hydroxyindole acetic acid levels) strongly suggested carcinoid disease, confirmed by an octreotide scan revealing an octreotide-secreting ovarian carcinoid tumor. Surgical intervention included tricuspid and pulmonary valve repair, total abdominal hysterectomy, and bilateral salpingo-oophorectomy. Primary ovarian carcinoid tumors, though rare, may lead to unexpected complications like CHD. Timely diagnosis and intervention are pivotal for optimizing patient outcomes in such cases.

Recovery of Tricuspid Valve Function After Resection of a Primary Ovarian Carcinoid Tumor.

Recovery of Tricuspid Valve Function After Resection of a Primary Ovarian Carcinoid Tumor.

Fortuitously detected primary ovarian carcinoid tumor: A case report.

Carcinoid tumors, derived from the cells of the disseminated neuroendocrine system, are rare, slow-growing neuroendocrine neoplasms that display a relatively indolent disease course. The majority of carcinoids are found within the gastrointestinal tract and bronchopulmonary system. Primary ovarian carcinoids are rare and account for merely 1% of all carcinoid tumors. We describe our experience of a rare case of primary ovarian carcinoid, presenting as chronic constipation, with no other carcinoid symptoms such as flushing, diarrhea, and wheezing. A 51-year-old postmenopausal woman with chronic constipation visited the clinic for routine check-up of her preexisting uterine fibroids. She had undergone hemorrhoidectomy 3 years ago. Physical examination revealed a soft abdomen without direct or rebound tenderness. Transvaginal ultrasonography revealed two subserosal fibroids, which had increased in size compared with previous ultrasonographic findings. A 3 cm hyperechoic mass was also detected in the right ovary. Her blood and urine tests were unremarkable, with no ascites in the pelvic cavity. She had a normal CA-125 level of 5.5 units/mL. The patient underwent a robot-assisted hysterectomy and right salpingo-oophorectomy because of enlarging fibroids and the right ovarian mass. Subsequently, based on the pathological and immunohistochemical findings, she was diagnosed with a primary ovarian carcinoid. The mass consisted of the insular and trabecular types of tumor cells. It was positive for pan-cytokeratin and synaptophysin, and the Ki-67 proliferation index was less than 1%. A follow-up positron emission tomography-computed tomography revealed no distant metastasis. Six months postoperatively, the patient was doing well without any signs of recurrence. Primary ovarian carcinoids without teratoma components are rare. It is crucial to make an accurate diagnosis based on the immunohistochemical staining results. Diagnosis in the early stages of the disease are associated with a favorable prognosis, but regular follow-up is mandatory.

Abstract 11155: Recovery of Tricuspid Valve Function After Resection of a Primary Ovarian Carcinoid Tumor

Case Presentation: A 51-year-old woman presented with a >1-year history of bilateral leg edema, diarrhea, and facial flushing. Examination revealed a large abdominal mass, which CT scan confirmed to be a 15x11x13 cm ovarian mass with solid and cystic components. She underwent hysterectomy and bilateral salpingo-oophorectomy. Histopathology revealed a 17 cm ovarian dermoid cyst containing an insular carcinoid tumor. Her post-operative urinary 5-hydroxyindoleacetate (HIAA) level was 23 umol/d (normal 10-40 umol/d) and chromogranin-A level was 95 ug/L (normal <94 ug/L), but positron emission tomography (PET) scan showed lymph node metastasis, prompting initiation of lanreotide, a somatostatin analogue. A perioperative echocardiogram revealed thickened, fixed, and retracted tricuspid and pulmonic valves with severe tricuspid and pulmonary regurgitation consistent with carcinoid heart disease. The right ventricle was dilated but showed preserved systolic function. Valve intervention was considered but deferred as right heart catheterization 15 weeks after surgery showed normal right-sided pressures. A follow-up echo 8 months post resection showed unchanged pulmonary regurgitation but significant improvement in tricuspid valve mobility with a corresponding reduction in tricuspid regurgitation to a mild-to-moderate level. Discussion: Thickened and fixed tricuspid valve leaflets with significant regurgitation represents a hallmark of carcinoid heart disease that can lead to progressive right heart failure, often necessitating surgical intervention. This is a rare case of carcinoid heart disease where there was marked improvement in tricuspid valve function after surgical resection of a primary carcinoid tumor and initiation of somatostatin analog therapy. It highlights the potential for timely treatment to reverse the deleterious effects of carcinoid tumors on cardiac valves, which may ultimately spare the need for cardiac surgery.

A 15-year comprehensive literature review of 99 primary ovarian carcinoid tumors

Objective: Primary ovarian carcinoids are neuroendocrine tumors, representing up to 1% of all ovarian tumors. In this paper, the authors aimed at analyzing the clinical and pathological aspects of all recently published ovarian carcinoid, providing new correlations regarding them. Mechanism: The authors have reviewed all cases of primary ovarian carcinoid reported in international journals since 2005 to date. A total of 99 cases published in 68 articles have been found and analyzed. Findings in brief: Our results up to 29.31% of patients presented with carcinoid heart disease, and 17.24% had an abdominal mass, which caused them to present to the physician. Patients presenting with metastases had more frequently the insular subtype (33.33%) and had similar median age as those without metastases. A teratoma component was noticed in 58.9% of cases and was also associated with the insular pattern of carcinoid. To our best knowledge, this paper includes the largest review of primary ovarian carcinoid to date. Conclusions: The incidence of carcinoid heart disease might have been underestimated up to this moment, thus, requiring further imagistic investigations of patients presenting with these symptoms. Additionally, the insular variant was most frequently associated with the presence of both teratoma (52.94%) and metastases (33.33%), although previous reports have noticed a higher incidence of teratoma in the mucinous variant. In our opinion, these apparently divergent results warrant further studies of this rare subtype of ovarian tumor.

Severe tricuspid regurgitation as first manifestation of a primary ovarian carcinoid tumor

A 52-year-old woman presented with a 1 year-history of reduced exercise tolerance and fatigue. Physical examination revealed a grade II/VI systolic murmur at the left sternal border. Transthoracic echocardiography showed thickened tricuspid leaflets with retraction and restricted movement, resulting in a severe tricuspid regurgitation. The characteristic pattern aroused the suspicion of a systemic or hormonal etiology. 24-Hour urine analysis revealed an increased level of 5hydroxyindoleacetic acid and abdominal computed tomography (CT) showed a large tumor in the pelvis; however, without the presence of liver metastasis. During uncomplicated trans-abdominal surgery a large mass in the ovary was removed, and pathology showed a primary ovarian carcinoid tumor. One year after the diagnosis, patient remains clinically stable without signs of right heart failure and an excellent exercise tolerance. This case report shows that isolated tricuspid valve pathology in the absence of left valvular disease may raise the suspicion for an atypical cause of valvular dysfunction, in which carcinoid tumor is included.

Primary strumal carcinoid tumor of the ovary arising from a heterochronous struma ovarii in young female patient

Primary ovarian carcinoid tumors are extremely rare. Ovarian strumal carcinoid is usually derived from mature cystic teratoma, an ovarian germ cell tumor composed of two distinctive components characteristic thyroid tissue intermixed with a carcinoid tumor. The incidence of stromal carcinoid tumor is accounting for 0.3-1% of all ovarian tumors and 3% of all mature teratomas. Herein, we report a 25-year-old female presented with severe abdominal pain. She had right struma ovarii after Laparoscopic-Assisted Ovarian Cystectomy (LAOC) procedure one year ago. The sonography of abdomen images study demonstrated a well capsulated cystic mass measured up to 11 cm in dimension. The mature cystic teratoma was the first diagnostic possibility. She underwent the laparoscopy-assisted left ovarian cystectomy. Histopathological and immunohistochemical examinations confirmed strumal Carcinoid Tumor of the Ovary (SCTO) arising from strum ovarii in the left ovary. She was recovered well and was still asymptomatic after two years follow-up. In conclusion, we first describe the primary SCTO arising from a heterochronous struma ovarii. The symptoms of SCTO are usually non-specific and misleading. Therefore, it is important to fully understand the characteristics, diagnosis and management of SCTO. Diagnosis should be confirmed by pathology and immunohistochemistry, and clinically metastatic carcinoid should be excluded.

Pure primary ovarian carcinoid tumor with carcinoid syndrome and cervical carcinoma: A rare concoction of dual primary malignancies

Carcinoid tumors are defined as rare slow-growing neuroendocrine tumors. A majority of primary ovarian carcinoids occur in association with mature cystic teratoma or are metastatic to the ovary. A 48-year-old post-menopausal woman presented with progressive facial puffiness, and intractable diarrhea. Radiological imaging suggested a 10 × 9 × 9.2 cm right ovarian mass. She underwent a total abdominal hysterectomy and bilateral salpingo-oophorectomy. Histopathological examination revealed primary pure carcinoid tumor of the ovary with the synchronous presence of early invasive squamous cell carcinoma of the cervix, along with the icthyosis uteri. The patient is doing well on 5 years of follow-up post-surgery without any recurrence or metastasis. In this report, we intend to highlight the rare association of cervical carcinoma and ichthyosis uteri with this tumor. In addition, we present a short review of the literature, over a decade of ovarian carcinoids associated with carcinoid heart disease.

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

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.

Tumor carcinoide primario de ovario. Reporte de caso

Los tumores neuroendocrinos primarios del ovario son raros y consisten en un grupo de neoplasias heterogéneas que expresan marcadores inmunohistoquímicos similares. Los tumores carcinoides son las neoplasias neuroendocrinas más comunes, la mayoría surgen en el tracto gastrointestinal y broncopulmonar. Los tumores carcinoides primarios del ovario son entidades raras que representan aproximadamente 0,3% de todos los tumores carcinoides y menos del 0,1% de todas las neoplasias ováricas, con buen pronóstico y generalmente limitados al parénquima ovárico. Estos tumores surgen del sistema de células neuroendocrinas del estroma, epitelio superficial y teratomas ováricos. Las manifestaciones clínicas, en la mayoría de los casos, están asociadas con liberación de sustancias vasoactivas que causan síntomas, como enrojecimiento cutáneo, diarrea y broncoespasmo. Para realizar el diagnóstico es necesario el uso de imágenes radiológicas multimodales y análisis bioquímicos de marcadores tumorales neuroendocrinos. El tratamiento de primera línea es la resección del tumor siempre que sea posible. El pronóstico es generalmente favorable, excepto en algunos casos con metástasis. Se presenta un caso de tumor carcinoide primario de ovario.

Strumal Carcinoid Focus in Mature Cystic Teratoma in a Patient with Breast Cancer and Desire for Fertility Preservation

Ovarian primary carcinoid tumors are very rare, accounting for 0.5-1.7% of all carcinoid tumors and 3% of ovarian teratomas. This type of tumor frequently arises from a mature cystic teratoma. The primary ovarian carcinoid tumors are divided into 4 major types, based on their histological characteristics: insular, trabecular or mucinous pattern. Strumal carcinoid refers to insular or trabecular pattern associated with struma ovarii. The strumal type is the most common histologic form, accounting for 40% of primary ovarian carcinoid tumors. Approximately 5% of carcinoid tumors of the non-mucinous type can be malignant. However, in the case of ovarian strumal carcinoid, the occurrence of metastasis is unusual. This article is based on a case of a 30-year-old nulliparous woman diagnosed with strumal carcinoid, who presented a right ovarian tumor suggestive of teratoma synchronous to a breast cancer. She was referred to our center for fertility preservation.

Primary ovarian carcinoid tumors: our experience and review of the literature

Primary ovarian carcinoid tumors: our experience and review of the literature

Carcinoid heart disease and a primary ovarian carcinoid tumor

Patients with primary ovarian carcinoid tumors may develop carcinoid syndrome and heart disease in the absence of metastasis. The release of bioactive amines by carcinoid tumors into the systemic circulation leads to right-sided valvular dysfunction and subsequent right ventricular failure. Surgical correction of carcinoid heart disease can reduce the symptoms of heart failure and lead to an improved prognosis. We report a case of a 55-year-old female who presented with carcinoid heart disease caused by a primary ovarian carcinoid tumor and underwent valve replacement surgery.

Carcinoid heart disease and a primary ovarian carcinoid tumor

Carcinoid heart disease and a primary ovarian carcinoid tumor

Primary Ovarian Carcinoid Tumor with a Mature Cystic Teratoma: A Case Report

Primary Ovarian Carcinoid Tumor with a Mature Cystic Teratoma: A Case Report

Clinicopathologic analysis of primary carcinoid of the ovary

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.

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

Primary ovarian neuroendocrine tumors (NETs) develop in pure form or in association with other tumors, mainly teratomas. The available data regarding this rare condition are limited. Much of the literature focuses on single case reports, previously dismissed as a totally benign disorder, and thus long-term considerations and evidence-based management guidelines are lacking. The objective of the current study was to describe the clinical and pathologic characteristics and the effect of various therapeutic modalities on patient morbidity and mortality from 2 major centers. A secondary objective was to highlight that carcinoid syndrome may be manifest in the absence of metastatic disease. The authors retrospectively studied a cohort of 34 consecutive patients with primary ovarian NETs who attended either the Oxford University Hospitals or Beatson Oncology Centre, Glasgow, between 1984 and 2014, and had their ovarian carcinoid data (eg, tumor growth, chemotherapy regimen) and mortality outcomes assessed. All patients were women, with an average age of 53 years (range, 23-87 years) at diagnosis. Of the 34 patients, 8 patients (23.5%) presented with carcinoid symptoms. Carcinoid heart disease was evident in 2 of the 34 patients (6%). All patients had a primary ovarian carcinoid tumor on histopathology, and 2 patients had bilateral ovarian carcinoids. Teratoma associations could be assessed in 29 tumors, with 20 (69%) of 29 showing a pathological association. The mean tumor size was 48 mm (range, 4-120 mm; SD, 40 mm). The mean follow-up was 4.5 years, ranging up to 17 years. The disease has been quiescent with no evidence of relapse in 22 of 34 patients; in 12 of 34 patients, it was metastatic. There were 5 patients who received treatment with somatostatin analogs or chemotherapy, whereas 8 patients (23.5%) died of disease. Metastatic disease was found up to 11 years from initial diagnosis. 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.

Ki67 Proliferative Index in Carcinoid Tumors Involving Ovary

Primary ovarian carcinoid tumors are rare neoplasms that constitute less than 0.1% of all ovarian carcinomas. However, carcinoid tumors metastatic to ovaries are more common. Cell proliferative rate is an important factor in the determination of neuroendocrine tumor prognosis. Limited data are available as regards Ki67 proliferation index in predicting the physiological features of carcinoid tumors involving the ovary. Pathology files of Mayo Clinic Rochester (1995–2014) were searched, and clinical information was collected from medical records. All cases were stained with an antibody against Ki67, and digital analysis was performed with digital imaging analysis. A total of 36 cases (median age 64 years, range 33–83 years), including 9 primary (median age 68 years, range 33–73 years) and 27 metastatic carcinoid cases (median age 64 years, range 36–83 years), were investigated in the current study. Seven out of nine (77.8%) primary ovarian carcinoids are associated with mature teratoma. Twenty two metastatic carcinoids (81.5%) were from the GI tract, four (14.8%) from the pancreas, and one (3.7%) from the posterior thorax location. There was significant difference of Ki67 index between primary (median 2.3%, range, 0.6–8.4%) and metastatic carcinoid tumors (median 9.7%, range, 1.3–46.7%) (p = 0.002). The survival time is much shorter among patients with metastatic carcinoid tumor (median survival 5.8 years) comparing to primary ovarian carcinoid tumor (median 14.2 years) (p = 0.0005). A strong association between Ki67 index and patient survival time was identified (Hazard ratio for 1-percentage point increase 1.11, p = 0.001). Comparing to primary ovarian carcinoid tumor, metastatic carcinoid usually exhibits a higher Ki67 index and a worse outcome.

Primary ovarian carcinoid tumor arising within a mature cystic teratoma in a 32-years-old patient.

Mature cystic teratomas (MCT) or dermoid cyst represent 10-20% of all ovarian neoplasm. Primary ovarian carcinoids are rare, accounting for 0,3% of all carcinoid tumors. We report a case of a 32-years-old woman diagnosed with primary carcinoid tumor of the left ovary arising in MCT. Vaginal ultrasonography showed left cystic adnexal image of 60 ? 40 mm, with fine echogenic dotting and a more refringent area in caudal pole. Doppler negative. Negative tumor markers. Pelvic MRI: Pelvic tumor of approximately 50 ? 61 mm of suprauterine location with a thin hypointense capsule with well-defined borders and fat-like signal intensity, which impresses of mature cystic teratoma as the first diagnostic possibility.Laparoscopy and left anexectomy was performed. The histopathological study reports, mature cystic teratoma of 5 cm with a single focus (0.6 cm) of carcinoid tumor. It is difficult to make a diagnosis of primary ovarian carcinoid tumor arising within a mature cystic teratoma of the ovary preoperatively. A thorough histopathological study of the tumor is very important and the surgical treatment adapted according to the characteristics of the patient.

Primary ovarian carcinoid tumor: a report of 4 cases

Ovarian carcinoid tumor is a rare neoplasm that account for 0.3% of all ovarian tumors. It is commonly seen in perimenopausal and postmenopausal women. The aim of this study is to investigate the clinical features and to evaluate the treatment of this rare tumor. The study reviewed retrospectively the clinical and pathological findings of 4 cases of primary OCT treated in Salah Azaiez Institute between 1994 and 2012. The median age was 50 years (ranging from 28 to 75 years). All the patients underwent radical surgery. Two patients had carcinoid tumors occurring in mature cystic teratoma. Three patients had stage I diseases, and one patient had stage III disease, this patient had adjuvant chemotherapy. The follow up median was 23 months. There was one case of bone and lung metastasis diagnosed in the patient who had stage III disease, fourteen months after the surgery but the patient died of her disease four month later. The other patients were alive without evidence of disease recurrence. Primary Ovarian carcinoid tumors are most of the time associated with good clinical results. Surgical excision of the tumor is usually sufficient treatment for most of the patients