Struma ovarii presenting as hyperemesis gravidarum in pregnancy.
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Ovarian germ cell tumors are reviewed with emphasis on recent developments. In the primitive germ cell tumor group the recently recognized subtypes of yolk sac tumor, the hepatoid and glandular variants are described. Hepatoid tumors, which resemble hepatocellular carcinomas, are usually admixed with other patterns of yolk sac neoplasia but when the hepatoid component predominates, distinction from metastatic hepatocellular carcinoma may be difficult. Glandular yolk sac tumors include those with a cribriform pattern as well as tumors that may be confused with endometrioid carcinoma. Within the teratoma category, a recent paper exploring the occasional relation between immature teratomas and dermoid cysts is reviewed. Problems caused by some of the monodermal teratomas are also highlighted. Struma ovarii may be the source of many diagnostic problems. It may be cystic and microscopic examination may reveal inconspicuous numbers of clearly recognizable thyroid follicles, resulting in confusion with other cystic ovarian tumors. In noncystic strumas many patterns, such as solid or pseudotubular, may be seen and the presence of cells with abundant clear or eosinophilic cytoplasm may add to the diagnostic difficulty, particularly if other teratomatous elements are lacking. Immunohistochemical staining for thyroglobulin may be crucial in establishing the diagnosis of a struma in these situations. The problem of identifying reliable criteria for malignancy of struma ovarii is explored on the basis of recent information. The two most recently recognized subtypes of carcinoid tumor, strumal and mucinous, are reviewed as are primary neuroectodermal and sebaceous tumors of the ovary. Unusual clinical features of germ cell tumors are discussed under three main categories: endocrine syndromes, paraendocrine and paraneoplastic syndromes, and miscellaneous. Included in these categories are disorders such as sexual precocity in association with germ cell tumors containing syncytiotrophoblast cells, endocrine abnormalities associated with the recently described functioning ovarian tumors with peripheral steroid cell proliferation, hyperthyroidism with struma ovarii, the carcinoid syndrome, autoimmune hemolytic anemia associated with dermoid cysts, complications of dermoid cyst rupture, which may simulate disseminated carcinomatosis, and ascites and Meigs' syndrome associated with struma ovarii.
A case of ovarian strumal carcinoid was studied by histochemistry, electron microscopy, and immunocytochemistry. The thyroid component of the tumor was micro-macrofollicular, whereas the carcinoid areas had a trabecular growth pattern. Carcinoid cells contained argyrophilic, dense-core secretory granules and were immunopositive for serotonin but not for calcitonin, somatostatin, or thyroglobulin. Follicular thyroid cells were positive for thyroglobulin, and were negative for calcitonin and somatostatin. Thyroglobulin and serotonin-containing cells were present in microfollicles adjacent to areas of trabecular carcinoid and struma ovarii. The data suggest that strumal carcinoids develop in close association with struma ovarii and represent a subset of ovarian teratomas. Current hypotheses on the cell of origin of strumal carcinoids are reviewed.
Malignant struma ovarii is a very rare tumour, with considerable controversy concerning the necessary histologic features for malignancy. Still more infrequent is the condition termed 'metastatic ovarian strumosis' or simply 'benign strumosis or strumatosis' and characterized by the presence of peritoneal implants of mature thyroid tissue occurring in struma ovarii. 'Strumosis' should not be confused with malignancy. Presented is a case of 'metastatic ovarian strumosis' in a 36-year-old woman with primary infertility who underwent three in-vitro fertilization (IVF) cycles with ovarian stimulation. She received hormonal treatment for 6 months after her last IVF because of 'persistent enlarged ovarian follicles' which were in fact 'thyroid follicles'.
Twenty-four cases of an unusual type of ovarian tumor with functioning stroma in which bands of steroid-hormone-producing cells were located adjacent to the tumor are presented. The cells were of stromal lutein cell type in 14 cases, stromal Leydig cell type in two cases, and hilus cell type in eight cases. Of the tumors with peripheral lutein cells, 11 were monodermal teratomas, including eight examples of struma ovarii, two strumal carcinoids, and one trabecular carcinoid; the other three tumors in this group were a dermoid cyst, a dysgerminoma containing syncytiotrophoblast cells, and a metastatic carcinoid. The tumors with peripheral stromal Leydig cells were a struma ovarii and a strumal carcinoid. Three of the tumors with peripheral hilus cells were mucinous cystadenomas, four were cysts of probable rete origin, and the eight was a dermoid cyst. The patients' ages ranged from 13-82 (average 58) years. Eighty percent of the patients were postmenopausal; one was pregnant. Forty-two percent of the patients had androgenic manifestations, 29% had evidence of hyperestrinism, and one (4%) had evidence of progesterone excess. In 29% of the cases there was no clinical evidence of hormonal imbalance. Those tumors with peripheral stromal Leydig cells or hilus cells were accompanied by androgenic manifestations in over one-half the cases, and those tumors with peripheral lutein cells had a slightly higher association with estrogenic rather than androgenic changes. Human chorionic gonadotropin and its beta subunit were demonstrated by the immunoperoxidase technique in some neoplastic cells in 4 of the 17 cases in which it was performed. Ovarian tumors with peripheral steroid-hormone-producing cells should be distinguished from the typical ovarian tumor with functioning stroma, which contains lutein cells scattered throughout its stroma.
Ovarian teratomas include mature cystic teratomas (dermoid cysts), immature teratomas, and monodermal teratomas (eg, struma ovarii, carcinoid tumors, neural tumors). Most mature cystic teratomas can be diagnosed at ultrasonography (US) but may have a variety of appearances, characterized by echogenic sebaceous material and calcification. At computed tomography (CT), fat attenuation within a cyst is diagnostic. At magnetic resonance (MR) imaging, the sebaceous component is specifically identified with fat-saturation techniques. The US appearances of immature teratoma are nonspecific, although the tumors are typically heterogeneous, partially solid lesions, usually with scattered calcifications. At CT and MR imaging, immature teratomas characteristically have a large, irregular solid component containing coarse calcifications. Small foci of fat help identify these tumors. The US features of struma ovarii are also nonspecific, but a heterogeneous, predominantly solid mass may be seen. On T1- and T2-weighted images, the cystic spaces demonstrate both high and low signal intensity. Familiarity with the US, CT, and MR imaging features of ovarian teratomas can aid in differentiation and diagnosis.
OBJECTIVES: To determine which extrauterine pelvic masses are difficult to correctly classify as benign or malignant on the basis of ultrasound findings, and to determine if the use of logistic regression models for calculation of individual risk of malignancy would improve the diagnostic accuracy in difficult tumors. METHODS: In a prospective, international, European multicenter study involving nine centers, 1066 women with a pelvic mass judged to be of extrauterine origin underwent transvaginal ultrasound examination by an experienced ultrasound examiner before surgery. A standardized examination technique and predefined definitions of ultrasound characteristics were used. On the basis of subjective evaluation of ultrasound findings, the examiner classified each mass as being certainly benign, probably benign, unclassifiable, probably malignant or certainly malignant. Even when the examiner found the mass unclassifiable (i.e. difficult mass) he or she was obliged to state whether the mass was more likely to be benign or malignant. Borderline tumors were classified as malignant. RESULTS: There were 90 (8%) unclassifiable masses. Multiple logistic regression analysis showed papillary projections, >10 locules in a cyst without solid components, low-level echogenicity of cyst fluid, and moderate vascularization as assessed subjectively at color Doppler examination to be ultrasound variables independently associated with unclassifiable mass. Borderline malignant tumors (n = 55) proved to be most difficult to assess with only 47% being correctly classified (i.e. classified as malignant), 29% being incorrectly classified (i.e. classified as benign) and 24% being unclassifiable vs. 90% of non-borderline tumors being correctly classified, 3% being incorrectly classified and 8% being unclassifiable (P < 0.0001). Papillary cystadeno(fibro)mas, myomas and cases of struma ovarii were also more common among the unclassifiable masses than among the classifiable ones (5.6% vs. 1.1%, P = 0.008; 4.4% vs. 0.9%, P = 0.02; 4.4% vs. 0.2%, P = 0.0006). No ultrasound variable or clinical variable (including CA 125) entered a logistic regression model to predict malignancy in difficult masses. A model could be constructed for difficult masses containing papillary projections but this model performed no better than subjective evaluation of the ultrasound image. Sensitivity and specificity of subjective evaluation with regard to malignancy in the group of unclassifiable masses were 56% (14/25) and 77% (50/65) vs. 91% (220/241) and 97% (712/735) in the classifiable masses. CONCLUSIONS: Borderline tumors cause great diagnostic difficulties, but so do papillary cystadeno(fibro)mas, struma ovarii and some myomas. Logistic regression models do not solve the diagnostic problem in difficult pelvic masses.
A malignant strumal carcinoid with widespread metastases resembling a well-differentiated thyroid follicular carcinoma is described. Strumal carcinoid is a rare ovarian tumor which usually behaves in a benign manner. Only one malignant case has been reported in the literature and in that case the carcinoid element metastasized. The clinical, histological, and immunohistochemical findings of the current case together with the relationship between strumal carcinoids and struma ovarii are discussed.
A case of low thyroid radioactive iodine uptake (RAIU) thyrotoxicosis due to a large struma ovarii comprising pure thyroid tissue is presented, including a detailed diagnostic evaluation, histopathology, and demonstration of rapid recovery of native thyroid function after surgical excision. In addition, the first comprehensive analysis of thyroglobulin obtained from an ovarian struma is reported.
Strumal carcinoid of the ovary is a type of germ-cell tumor characterized by an intimate mixture of thyroid tissue and carcinoid. Fifty patients with this type of tumor ranged in age from 21 to 77 years. The tumors measured up to 26 cm in diameter and were always unilateral. In 10% of the cases, the contralateral ovary contained another type of neoplasm, usually a dermoid cyst. Three-fifths of the strumal carcinoids arose in dermoid cysts or in mature solid teratomas. Birefringent calcium oxalate monohydrate crystals were commonly identified in the thyroid colloid, and argentaffin granules, in the carcinoid cells, thus substantiating the identity of the neoplastic components. Although 31% of the tumors were accompanied by focal stromal luteinization, only 8% of the cases exhibited clinical signs of steroid hormone production (endometrial hyperplasia, hirsutism, or virilism). In 8% of the cases, there was evidence suggesting functioning of the thyroid component, but no patient had the carcinoid syndrome. Only one woman died of tumour; five others died of unrelated causes. The remaining patients remained alive and well; 23, for at least five years, and 15, for at least ten years postoperatively. Although the carcinoid component of the strumal carcinoid has been considered a malignant transformation of struma ovarii, it is almost always benign, and treatment with a simple oophorectomy or salpingo-oophorectomy is effective.
A struma ovarii was diagnosed and excised during a Caesarean section. This seems to be the fifth case of such a tumor reported during pregnancy. The necessity of a routine inspection of the adnexe during Caesarean section is stressed.
Ovarian teratomas are known for their ability to form a wide variety of tissues. One unusual example of this potential is the strumal carcinoid in which the thyroid tissue is found in intimate association with carcinoid tumor. Although considered as a malignant transformation of struma ovarii, it is almost always benign. We report a rare case of strumal carcionid in a 44 year old lady who had metastasis in the contralateral ovary, myometrium and lungs.
A case of associated ovarian and cervical thyroid struma in a 24-year old female, is reported. The patient was affected by a right thyroid cold nodule which at fine needle aspiration biopsy showed the cytological pattern of benign follicular adenoma; as the nodule was increasing after one year of suppressive therapy, the patient was selected for surgery. At admission during routine physical examination a previously undetected left pelvic mass was discovered: at laparotomy it proved to be a cystic tumor consisting of benign thyroid tissue at the level of left ovary. Histology confirmed the diagnosis of follicular adenoma of the right thyroid nodule after right lobectomy. Albeit cases of thyroid tissue in the ovary have been reported, true struma ovarii is a rare occurrence. A concomitant hyperthyroidism is seen in only 1/4 of cases and the patients can be divided into three groups: hyperthyroid, ascitic and asymptomatic: our patient was in the asymptomatic group.
The association of ascites, hydrothorax and struma ovarii has been previously reported in the literature. This case is added to the literature describing a variant of Meigs' syndrome, or pseudo Meigs' syndrome. A discussion of the origin of ascitic fluid illustrates how little is known about these conditions as well as the importance of their careful differential diagnosis.
Benign lymph node inclusions are commonly encountered during surgery for gynecologic neoplasms and are potential mimics of metastatic disease. A 52-year-old woman presented with ascites, a complex adnexal mass, and a CA-125 of 1891 units/mL. A staging laparotomy was performed, diagnosing struma ovarii. Pathologic evaluation of pelvic lymph nodes demonstrated mesothelial inclusions in nodal sinuses suspicious for metastatic disease. Immunocytochemical evaluation revealed benign mesothelial inclusions rather than metastatic thyroid carcinoma. Benign mesothelial lymph node inclusions in nodal sinuses are potential mimics of metastatic carcinoma. Their presence in pelvic lymph nodes has not previously been reported. Given the potential difficulty in determining the origin of these inclusions, immunocytochemical evaluation is useful in reaching the correct diagnosis.
OBJECTIVE: To describe the clinical characteristics of benign ovarian tumor/mass which manifest as pelvic mass, ascites and high serum levels of CA(125). METHOD: Clinical data of 3 cases of such disease in Peking Union Medical College Hospital were analyzed and relevant literatures were reviewed. RESULTS: The patients all were misdiagnosed as ovarian carcinoma, and operations designed for malignant ovarian tumor were performed on all of them. There was no definite proof of malignant tumor, such as positive findings of pathology and/or cytology, in any patients before operation. Among these diseases, pelvic tuberculosis, ovarian endometrioid cyst and ovarian fibroma are the three most common ones, others are: fibrothecoma, thecoma, struma ovarii, adenofibroma, benign Brenner tumor, mature theratoma, ovarian edema and fibromatosis. CONCLUSIONS: Pelvic mass, ascites and high serum level of CA(125) can't always result of a diagnosis of ovarian carcinoma. Positive findings of pathology and/or cytology is the only definite proof of a diagnosis of ovarian malignant tumor. On the susceptible case, laparoscopy is a useful and reliable method.