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Trabecular carcinoid tumour composed of solid parts and showing a trabecular growth pattern (H&E × 20) (left up). Carcinoid cells with oblong nuclei and columnar cytoplasm presented with long, wavy, parallel ribbon-like arrangement (H&E × 40) (right up). Insular carcinoid tumour composed of solid nests and small acini (H&E x 20) (left down); H&E x 40 (right down)
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BACKGROUND: Ovarian strumal carcinoid is a germ cell tumour characterised by a mixture of thyroid tissue and carcinoid. Ovarian struma is a very rare occurrence with 0.3-1% incidence of all ovarian tumours and 3% of mature teratomas. Primary carcinoid ovarian tumours are still uncommon as a part of mature teratoma or mucinous cystadenoma. There are...
Citations
... Ovarian stromal carcinoid is a rare monodermal germ cell tumor composed of thyroid tissue and carcinoid components. This tumor accounts for less than 1 % of all ovarian cancers and 3 % of mature teratomas [1]. It is often unilateral and occurs most commonly in perimenopausal and postmenopausal women [2]. ...
... Strumal carcinoid tumor of the ovary is a rare and unique teratoma of the ovary, which consists of thyroid tissue mixed with neuroendocrine neoplasm [6]. It has a very rare occurrence with 0.3-1 % incidence of all ovarian tumors and 3 % of mature teratomas [1]. Li and al in a retrospective study of 119 patients published in 2022 reported that perimenopausal and postmenopausal women are typically affected. ...
Introduction
Ovarian strumal carcinoid is a rare type of germ cell tumor. It usually affects perimenopausal and postmenopausal women. Very few cases of stromal carcinoid have been reported in the literature in women of childbearing age, particularly during pregnancy. The clinical presentation of the tumor, and in particular its non-specific clinical and radiological appearance and rarity, explain the difficulties in diagnosis and management.
Presentation of case
Herein, we describe a rare case of a 36-year-old patient who was followed-up in our outpatient clinic for organic cyst of the ovary. The ultrasound revealed a multilocular regular cystic mass with a modestly thickened wall and fine septations. The MRI indicated a right ovarian cyst with solid tissue. The levels of tumor markers were normal. The patient was lost to follow-up and did not return until six months later. She was admitted in our Department with acute ovarian torsion and underwent emergency surgery at 17 weeks' gestation. A laparoscopic cystectomy of the right ovary was provisionally performed. Pathology revealed an ovarian strumal carcinoid tumor.
Discussion
Patients with ovarian stromal carcinoid have an excellent prognosis. Ovarian strumal carcinoid ‘s primary therapy method is operation. The majority of original ovarian carcinoid tumors progress slowly, and practically all thyroid carcinoid tumors are clinical stage I with a positive prognosis.
Conclusion
In the absence of standardized treatment, the association of carcinoid strumal tumor with pregnancy, underlines the need for early diagnosis and appropriate multidisciplinary management, taking into account both the maternal and fetal prognosis.
... Strumal carcinoid of the ovary is also an extraordinary form of primary germ cell tumor, and consists of an intimate mixture of thyroid tissue and carcinoid tissue. 3,4) PTC and strumal carcinoid coexistence in patients with mature teratoma are very rare. Here, we present a rare case of combined PTC and strumal carcinoid arising from a mature cystic teratoma (MCT) in a 32-year-old pregnant woman. ...
... The mucinous variant is made out of glands lined by goblet cells, floating inside mucin pools [79]. This subtype is usually pure, only rarely associating a strumal component, and is regarded as a more aggressive subtype [58]. ...
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.
... Strumal carcinoids can be pure and sometimes in association with mature teratomas, granulosa tumors, and mucinous cystadenomas of the ovary [1,2]. These are reported mainly in pre-and post-menopausal women with a peak in the fifth decade of life [1,[3][4][5][6][7][8]. ...
... The histogenesis of its origin is being debated as it is mostly considered as a component of the teratoma as a germ cell origin. The carcinoid component is native to the ovary and less likely to be a metastatic component from the gastrointestinal tract [4][5][6][7][8]. There is also a theory about hybrid cells differentiating into thyroid follicular cells and hindgut neuroendocrine cells [9,10], and the carcinoid arises from neuroendocrine cells of the genital tract [3]. ...
... They can also appear as thickened lining of cyst wall and also as a component of other neoplasms [1,[3][4][5]. According to WHO, carcinoids are of four histological types which include insular, trabecular, strumal, and mucinous [1,3,4,8,12]. Insular is the most common type followed by strumal [7,10]. A well-differentiated neuroendocrine tumor is seen. ...
Strumal carcinoid is an unusual rare ovarian teratoma characterized by the presence of thyroid tissue with a carcinoid tumor. We report a case of a 60-year-old nulliparous woman, who presented with complaints of a decrease in appetite, urinary frequency, and left lower extremity edema. By ultrasound of the abdomen, a large multiloculated cystic lesion occupying almost the entire pelvis and measuring 24 x 14 x 20 cm with internal debris concerning either uterine or ovarian cystic carcinoma was seen. By MRI, it was confirmed to be an ovarian lesion. Labs revealed elevated cancer antigen 125 (CA125) of 105 U/ml and carcinoembryonic antigen (CEA) of 6.4 ng/ml. The patient underwent surgery and the intraoperative consultation confirmed teratoma with a neuroendocrine component. Grossly, it was a multicystic ovarian mass and on sectioning, it had partial solid and cystic areas with clear to mucoid fluid. Histopathology showed foci of ectopic thyroid tissue admixed with foci of well-differentiated neuroendocrine tumor, grade 1 (carcinoid) displaying insular and trabecular patterns consistent with the diagnosis of strumal carcinoid (monodermal teratoma). Thyroid transcription factor-1 (TTF-1) and thyroglobulin immunostains highlighted ectopic thyroid tissue and synaptophysin highlighted neuroendocrine component. Strumal carcinoids are almost invariably benign and pathologic staging is not warranted. Treatment of strumal carcinoid is salpingo-oophorectomy.
... Strumal Carcinoid of the Ovary (SCTO) is a rare monodermal germ cell tumor, compose of the components teratoma histomorphologic ally characterized by an innimate mixture of thyroid tissue contains less than 50% and admixtures neuroendocrine tumor (carcinoid) tissue [11][12][13]. ...
... Insular carcinoid is the most common type about 50%. One third of cases present clinical as related to carcinoid syndrome [12][13][14]. ...
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.
... When the thyroid tissue composition exceeds 50%, it can be diagnosed as an ovarian goiter [1]. It is most commonly found in the fifth and sixth decades of life and accounts for 0.3% -1% of all ovarian tumors and 3% of mature teratomas [2] [3]. Herein, we report two interesting cases of patients with primary strumal carcinoid of the ovary who had been admitted to our hospital's gynecological department in 2018. ...
... They are usually seen in perimenopausal or early postmenopausal females [5]. More than 50% of ovarian carcinoids reported as a component of MCT [6]. These patients may present with an enlarging abdominal or pelvic mass [1][2][3][4][5][6]. ...
... More than 50% of ovarian carcinoids reported as a component of MCT [6]. These patients may present with an enlarging abdominal or pelvic mass [1][2][3][4][5][6]. The other uncommon symptoms include constipation, urinary frequency, abdominal pain, hirsutism may also be present [1][2][3][4][5][6]. ...
... These patients may present with an enlarging abdominal or pelvic mass [1][2][3][4][5][6]. The other uncommon symptoms include constipation, urinary frequency, abdominal pain, hirsutism may also be present [1][2][3][4][5][6]. The symptoms like cutaneous or facial flushing, bronchospasm, abdominal cramps, diarrhea, edema, carcinoid heart disease, etc., are also reported in these patients [5,6]. ...
Mature cystic teratoma (MCT) is the most common benign germ cell tumor of the ovary and contains the different tissues that originate from the endoderm, mesoderm, and ectoderm. The monodermal teratoma has a component of only the germ layer. Ovarian carcinoid is rare and considered as a monodermal teratoma. We report a case of carcinoid tumor arising in MCT in a 60-year-old postmenopausal woman.
https://www.cureus.com/articles/46692-ovarian-carcinoid-misinterpreted-as-endometrioid-adenocarcinoma-in-mature-cystic-teratoma
... 8 Most primary ovarian carcinoids occur in a wide age range with most arising in peri-or postmenopausal females. 2,9 The majority develop within mature cystic teratomas, and most are unilateral tumours that are small and are microscopically detected within teratomas. 6 They may, however, be identifiable as yellow foci at the time of macroscopic dissection. ...
Primary ovarian neuroendocrine tumours are very uncommon tumours. Herein, we describe a case of 27-year-old female who presented with abdominal pain and an ovarian mass, for which she underwent a right salpingo-oophorectomy. Histopathological evaluation confirmed a right-sided primary ovarian neuroendocrine tumour occurring in a benign mature cystic teratoma. The patient has not undergone any additional therapy and is currently well. This case illustrates the need for thorough clinicopathological correlation together with adequate sampling to ensure accurate diagnosis and timeous management of the patient.
... Laparotomy, including TAH, BSO, omentectomy, pelvic lymphadenectomy, and appendectomy, was performed [11] 52 -CHD No TAH-BSO 2009 Gungor et al [12] 47 -No No TAH-BSO +staging+appendectomy 2010 Chen et al [13] -Strumal Constipation No Laparoscopic oophorectomy 2010 Bai et al [14] 55 Trabecular No No TAH-BSO 2010 Aggeli et al [15] 60 -CHD No -2011 Alexander et al [16] --No Endometrial cancer+gliomatosis peritonii TAH-BSO 2011 Roberts et al [17] 53 -CHD liver mets -2011 Djurovic et al [18] 49 -Yes No TAH-BSO 2012 Hinshaw et al [19] 74 Strumal No Adenocarcinoma+strumal thyroid cancer Robotic LaparoscopicTAH-BSO+staging 2012 Buda et al [20] 78 Insular CHD -TAH-BSO +staging 2012 Takatori et al [21] 48 Strumal Constipation No USO 2012 Yamaguchi et al [22] 24 Strumal Constipation Mucinous cystadenoma Oophrectomy 2013 Amano et al [23] 67 -CHD Nodal recurrence Excision 2013 Bassi et al [24] 45 -No Gall bladder carcinoid TAH-BSO +staging+Radical cholecystectomy 2013 Petousis et al [25] 28 Trabecular No No Laparoscopic Excision 2014 Ting et al [26] -Insular No No BSO 2014 Horikawa et al [27] 57 Trabecular ---2014 Huang et al [28] 46 -Cushing No BSO 2014 Spaulding et al [29] 51 Trabecular No Ovarian ependymoma+MEN1 Robotic TAH-BSO 2014 Sharma et al [30] 50 Trabecular No No TAH-BSO 2015 Muller et al [31] 34 Trabecular Constipation No -2015 Dessauvagie et al [32] 69 -CHD No -2015 Quiñonez et al [33] -Mixed No Pseudomyxoma peritonii -2015 Agarwal et al [34] 75 -CHD No USO 2015 Tarcoveanu et al [35] 55 Trabecular No Colonic lymphangioma Laparoscopic USO 2016 Kolouch et al [9] 77 Insular CHD No TAH-BSO 2016 Kim et al [36] 39 Mixed No No USO 2017 Tadokoro et al [37] 73 -CHD, Diarrhea -TAH+USO 2017 Saraf et al [38] 75 Insular CHD No TAH-BSO 2018 Van et al [39] 55 Mucinous stomach pain, weight loss, fatigue, backache spine, liver, breasts, subcutis, and lungs -2018 Antovska et al [40] 59 Strumal perimenopausal uterine bleeding No TAH-BSO+staging biopsy 2019 Ishida et al [41] 46 and 52 Strumal Case 1, enlarged both ovaries Case 2, enlarged right ovary No Case 1, TAH-BSO Case 2, Laparoscopic oophorectomy 2019 Hsu et al [42] 33 Atypical Recurrence, Hydronephrosis, Dead -USO+ILND+staging+chemotherapy -= unmentioned, CHD = carcinoid heart disease, ILND = ipsilateral lymph node dissection, TAH-BSO = total abdominal hysterectomy and bilateral salpingo-oophrectomy, USO = unilateral salpingooophrectomy. ...
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.
... Carcinoid tumors of the ovary are rare and account for less than 1% of all malignant neoplasm of the ovary [1]. They are subclassified into 4 types: insular, mucinous, trabecular, and strumal. ...
... In addition, an origin of C-cell was also postulated given immunohistochemical calcitonin expression in some cases [8]. The vast majority of strumal carcinoids are biologically indolent and curable by oophorectomy [1]. Only very few cases with metastatic disease were described [9,10]. ...
... Only one tumorassociated death has been published [11]. Due to the rarity of strumal carcinoids, knowledge about this tumor entity has been mostly generated from case reports [1,12,13]. In addition, a large series of 50 cases was published in 1980 addressed to the description of macroscopic and microscopic architectural features as well as the clinical outcome [11]. ...
Strumal carcinoid is an extraordinary rare tumor of the ovary consisting of thyroid tissue intermixed with neuroendocrine tumor component. The cellular origin of strumal carcinoids has been an area of debate. There is also little data on detailed immunohistochemical and molecular characteristics of these neoplasms. For this reason, this series investigated the characteristics of a series of 13 strumal carcinoids using immunohistochemical markers and a 47-gene next-generation sequencing (NGS) solid tumor panel analysis. Both cellular components showed thyroglobulin expression in all tumors. TTF-1 expression was noted in both cellular components of 11 cases. Chromogranin A was positive in both components of most tumors (n = 12, 92.3% in the neuroendocrine component and n = 10, 76.9% in the thyroid follicular component). Synaptophysin stained the neuroendocrine component of all cases, and it was also identified in the follicular thyroid component of a single case. All tumors were negative for CDX2 and calcitonin. ISLET1 was positive in the neuroendocrine component of 8 cases (6.5%). With the exception of one case, all tumors were positive for SSTR2a. The tumors were associated with a low Ki67 labeling index. All cases were microsatellite stable and no pathogenic mutations were identified using a 47-gene NGS solid tumor analysis. This series underscored that strumal carcinoids are distinct neuroendocrine tumors. The synchronous expression for thyroid follicular epithelial and neuroendocrine differentiation biomarkers may suggest a precursor cell origin displaying mixed-amphicrine differentiation. While strumal carcinoids can be diagnosed by their typical morphology and immunohistochemical profile, frequent SSTR expression may serve as a potential theranostic biomarker in the management of affected patients. In addition, the absence of common driver mutations in the NGS solid tumor panel may suggest that these neoplasms seem to be genetically unrelated to follicular epithelial–derived thyroid tumors and potentially different than other commonly identified well-differentiated neuroendocrine neoplasms. Therefore, further studies focusing on molecular characteristics of this entity are still needed.
