Sertoli-Leydig cell tumor with heterologous element: a case report and a review of the literature

The patient was a 19-year-old female who presented with a chief complaint of progressive pelvic pain. Preoperative ultrasound of the right ovary revealed an ovarian torsion as the cause of the patient’s progressive pain. Laparoscopy confirmed the torsion and revealed a right ovary measuring 10 cm in greatest diameter. Intraoperative incision into the ovary revealed a simple ovarian cystic mass measuring 3.0 x 1.5 x 0.8 cm. A solid component within the cyst was identified. Histological sections of the cystic mass demonstrated mononuclear and hyperchromatic Sertoli cells with a trabecular growth pattern. Clusters of medium-sized epithelioid cells with abundant eosinophilic cytoplasm consistent with Leydig cells were also identified between the trabeculae of Sertoli cells. In addition, focal areas of intestinal type mucinous epithelium were identified embedded within the trabeculae of Sertoli cells. Immunohistochemical studies revealed that the Sertoli cells were positive for calretinin (bright) while the Leydig cells were positive for calretinin (dim), inhibin, CAM5.2 and AE1&3. CEA showed positivity mainly of the intraluminal contents of the mucinous type intestinal epithelium. The patient had an uneventful post-operative course and was disease-free for 3 years.     

Sertoli-Leydig Cell Tumor of the Ovary with Heterologous Elements and Carcinoid: An Immunohistochemical and Ultrastructural Study

Our thanks are due to Dr Michael Cullen, St. James's Hospital, Dublin, for clinical data in this case and to Dr Sean O'Briain, Central Pathology Laboratory, St. James's Hospital, and Dr Fred Bosman, Rijksuniversiteit, Leiden, who supplied the various antisera. James Dunne and Derek Cullen provided expert technical assistance in electron microscopy and staining techniques.

A Sertoli-Leydig cell tumor of the ovary is described in which there were heterologous elements consisting of enteric cysts and an evolving carcinoid. Endocrine ceils in the cysts and carcinoid were found to contain gastrin and other polypeptide hormones. Aspects of the organization of the enteric epithelium, its histogenesis, and the possible relationship of this tumor to other cystic ovarian neoplasms are discussed.     

Sertoli-Leydig Cell Tumor of the Ovary with Heterologous Elements and Carcinoid: An Immunohistochemical and Ultrastructural Study

Our thanks are due to Dr Michael Cullen, St. James's Hospital, Dublin, for clinical data in this case and to Dr Sean O'Briain, Central Pathology Laboratory, St. James's Hospital, and Dr Fred Bosman, Rijksuniversiteit, Leiden, who supplied the various antisera. James Dunne and Derek Cullen provided expert technical assistance in electron microscopy and staining techniques.

A Sertoli-Leydig cell tumor of the ovary is described in which there were heterologous elements consisting of enteric cysts and an evolving carcinoid. Endocrine ceils in the cysts and carcinoid were found to contain gastrin and other polypeptide hormones. Aspects of the organization of the enteric epithelium, its histogenesis, and the possible relationship of this tumor to other cystic ovarian neoplasms are discussed.     

Detection of the DICER1 hotspot mutation alongside immunohistochemical analysis may provide a better diagnostic measure for ovarian Sertoli-Leydig cell tumors

Background

To evaluate the clinicopathological and histopathological characteristics of ovarian Sertoli-Leydig cell tumors (SLCTs) in relation to differential diagnosis, and patient prognosis.

Cytopathologic differential diagnosis of small cell carcinoma and poorly differentiated non‐small cell carcinoma in bronchial lavage specimens using a regression analysis

Cakir E, Demirag F, Aydin M. Cytopathologic differential diagnosis of small cell carcinoma and poorly differentiated non‐small cell carcinoma in bronchial lavage specimens using a regression analysis. APMIS 2010; 118: 150–55. The aim of this study was to determine the most significant cytologic features to differentiate small cell carcinoma (SCC) from poorly differentiated non‐small cell carcinoma (NSCC) in bronchial lavage specimens. Bronchial lavage specimens from 35 SCC cases and 63 poorly differentiated NSCC cases were examined and the cytologic parameters reviewed retrospectively. Thirty‐five cytologic features considered useful in differential diagnosis were assessed. Statistical analysis indicated that salt and pepper chromatin, small cell size and nuclear molding have more than 90% sensitivity and 70% specificity for SCC cases. Logistic regression analysis demonstrated that the most effective criteria to differentiate SCC from poorly differentiated NSCC are small cell size, salt and pepper chromatin, prominent nucleolus and papilla formation. When these selected variables were used, sensitivity for predicting SCC was 94.3% and specificity 96.8%, and sensitivity for predicting NSCC was 96.8% and specificity 94.3%. There are several cytologic features, which are highly sensitive and specific for distinguishing SCC from NSCC. Nuclear features such as chromatin pattern, and size of the nucleoli and nuclei are more valuable than cytoplasmic features to distinguish between the two.     

Immunohistochemical characterization of extracellular matrix components of granulosa cell tumor of ovary

In order to clarify the characteristics of granulosa cell tumors of the ovary, extracellular matrix components were investigated by immunohistochemical techniques. Twenty-three granulosa cell tumors (GCT; eight juvenile and 15 adult type) were studied in comparison with non-neoplastic granulosa cells of human ovaries. In all 23 cases of GCT, chondroitin 6-sulfate proteoglycan revealed with antibody 3B3 was characteristically observed in the extracellular matrix in the solid nest, as well as in microfollicles. In the juvenile cases, the extracellular matrix also contained large proteoglycan (PG) revealed with antibody 2B1. Macrofollicles as well as micro-follicles contained PG chondroitin 6-sulfate side chains with a significant amount of chondroitin 4-sulfate. By biochemical analysis using high pressure liquid chromatography, it was also found that disaccharide composition of glycosaminog-lycan fractions extracted from granulosa cell tumor tissues consisted mainly of 2-acetamide-2-deoxyl-3-0-(β-D-gluco-4-enepyranosyluronic acid)-6-O-sulfo-D-galactose (δ Di-6S). The characteristic feature of granulosa cell tumors is the accumulation of chondroitin sulfate PG, especially chondroitin 6-sulfate PG, which may be synthesized by the tumor cells themselves. Immunohistochemical characterization of the extracellular matrix components (collagen, laminin, heparan sulfate PG, chondroitin 4-sulfate PG) was also studied in relation to chondroitin 6-sulfate PG localization.     

DICER1 mutations in a patient with an ovarian Sertoli-Leydig tumor,well-differentiated fetal adenocarcinoma of the lung,and familial multinodular goiter

DICER1 syndrome, a recently described tumor-predisposition syndrome, often involves multiple organs and is characterized by pleuropulmonary blastoma (PPB), cystic nephroma, ovarian Sertoli-Leydig tumors, familial multinodular goiter, etc. Germline DICER1 mutations have been identified in individuals with a variety of malignant conditions. However, in a review of the reported DICER1 syndrome cases that feature an unusual array of neoplastic and hyperplastic phenotypes, no mentions are made of these patients also presenting well-differentiated fetal adenocarcinoma of the lung.Here, we present a 16-year-old Chinese adolescent suffering from an ovarian Sertoli-Leydig cell tumor, well-differentiated fetal adenocarcinoma of the lung, and familial multinodular goiter with a nonsense mutation (c.3540C > A; p.Tyr1180*) in exon 21 of DICER1. This report presents the first case in which the clinical features of DICER1 syndrome appear in combination with well-differentiated fetal adenocarcinoma of the lung. We hypothesize that this case may suggest that well-differentiated fetal adenocarcinoma of the lung falls within the wide spectrum of manifestations of the DICER1 syndrome. Remarkably, this mutation is reported in a patient from The International PPB Registry.     

Composite mucinous and granulosa cell tumor of the ovary

A composite mucinous and granulosa cell tumor of the ovary in a 76-year-old woman is herein reported. At laparotomy this tumor proved to be a solid and cystic mass measuring 10 cm in greatest diameter. Many of the cysts were lined with a benign mucinous epithelium of the endocervical type, and solid areas contained a proliferation of granulosa cells. These two disparate components were intimately mixed. A theca cell component was also present in areas adjacent to the mucinous epithelium. The coexistence of mucinous and granulosa cell tumor is extremely rare and only four such cases have previously been reported in the literature, and the histogenesis of this tumor has not yet been elucidated. In the present case it is suggested that the granulosa cell element commenced as a reactive stromal hyperplasia in the wall of the pre-existing mucinous neoplasm and thereafter progressed to the point of producing a tumor-like mass or neoplastic changes.     

Malignant granular cell tumor. A clinico-pathologic and ultrastructural study of a case

The clinical and light- and electron-microscopic features of a malignant granular cell tumor in a 64-year-old man are reported. The ultrastructural appearance, with abundance of cytoplasmic "phagosomes", may help in the differential diagnosis. The light-microscopic findings and some ultrastructural observations support the suggestion that the tumor originated from peripheral nerves, and that a close relationship exists between malignant granular cell tumors and malignant Schwannoma.     

Primary carcinoid tumor of the testis: Immunohistochemical, ultrastructural and FISH analysis with review of the literature

Primary carcinoid tumor of the testis only accounts for 0.2% of all carcinoid tumors in Japan. The clinicopathological features, including differences between Japanese and Europe/United States (Eur/US) series', which are known in ovarian carcinoids, have not yet been sufficiently recognized. In the present study, five testicular carcinoids from various clinicopathological viewpoints were analyzed, with a review of the literature. All 15 cases in Japan, including 10 previously reported, were insular carcinoids. Three of the 15 cases (20%) were associated with a teratoma. Serotonin production was confirmed in at least 10 cases (five cases in the present study and five cases in previous studies), but carcinoid syndrome was present in only two cases. Three of the 15 cases (20%) showed distant metastases, and the tumors that metastasized were larger than those that did not. These features were almost similar to those observed in the Eur/US series. Three pure carcinoids were also studied for the number of sex chromosomes by fluorescence in situ hybridization (FISH). They did not show a significant numerical abnormality of the X chromosome, which is common in testicular germ cell tumors. Therefore, the genetic background of pure carcinoids might be different from that of common germ cell tumors.     

恶性颗粒细胞瘤临床病理、免疫组化和超微结构观察

目的：探讨恶性颗粒细胞瘤的病理学诊断和鉴别诊断要点及组织学起源。方法：对３例恶性颗粒细胞瘤进行临床病理、免疫组化及超微结构观察研究。结果：男性２例，女性１例，平均年龄为４９岁。部位分别为颈部１例，右大腿２例。其中２例分别于术后２年半及７年复发，并伴有区域淋巴结转移。组织学上３例与良性颗粒细胞瘤十分相似，局部区域出现梭形瘤细胞，空泡状核及明显的核仁，其中１例在肿瘤的周边部可见到瘤细胞与外周神经束支之间有直接移行关系。免疫酶标结果显示瘤细胞强阳性表达Ｓ－１００蛋白和神经特异性烯醇化酶（ＮＳＥ），１例电镜检测显示胞浆内充满膜包被复合性溶酶体。结论：对临床明显恶性而组织学上却极似良性的恶性颗粒细胞瘤，以下几点能提示恶性诊断：（１）肿瘤超过４ｃｍ；（２）核分裂象超过２个／１０ＨＰＦ；（３）核呈空泡状并有明显的核仁；（４）出现梭形瘤细胞；（５）有肿瘤性坏死。此外，免疫组化标记及超微结构观察有助于鉴别诊断及揭示组织学起源。     

Ovarian mixed germ cell tumor composed of dysgerminoma, endodermal sinus tumor, choriocarcinoma and mature teratoma in a 44-year-old woman: Case report and literature review

A case of ovarian mixed germ cell tumor In a 44-year-old woman was examined. The tumor was well circumscribed, measured 15 times 11 times 10cm and appeared solid and partly cystic on the cut surface. Light microscopic examinations revealed that the tumor was composed of four different neoplastic germ cell elements, Intermingled with each other. They are: (i) choriocarcinoma, immunohistochemically positive for human placental lactogen (hPL) and human chorionic gonadotropin (hCG); (ii) dysgerminoma, positive for placental alkaline phosphatase; (iii) endodermal sinus tumor positive for α-fetoproteln (AFP); and (lv) mature teratoma. Among these histological types, dysgermlnoma occupied more than 50% of the neoplasm. The patient was diagnosed as a stage la ovarian mixed germ cell tumor and was subsequently treated with chemotherapy. A second-look laparotomy after completion of chemotherapy revealed no residual tumors in the abdomen and the patient Is alive and well 15 months after operation. This Is the fourth reported case of ovarian mixed germ cell tumor arising In patients over 40 years old.     

146例套细胞淋巴瘤免疫组织化学结果分析

目的：讨论套细胞淋巴瘤的免疫组织化学特征。方法：回顾性分析146例套细胞淋巴瘤的免疫组化结果,并用FISH方法检测1例Cyclin D1阴性病例是否存在t(11;14)易位。结果：套细胞淋巴瘤免疫组化阳性率为CD20：98.6%(144/146);CD79a：100%(146/146);CD5：88.4%(129/146);CyclinD1：99.3%(145/146);PAX-5：100%(122/122);CD43：84%(79/94);Ki67指数：5%~90%,中位数为20%。少部分病例异常表达CD10、Bcl6、CD23、CD56、CD3、CD45RO。FISH检测1例Cyclin D1阴性病例结果为检测到t(11;14)易位形成的IgH/CCND1融合基因。结论：套细胞淋巴瘤存在较为特征性的免疫组化表达模式,并存在异常表达现象。     

Large-cell calcifying Sertoli cell tumor of the testis: a case report with ultrastructural study

Large-cell calcifying Sertoli cell tumor (LCCSCT) is a rare histologic variant of Sertoli cell tumor. Recently we observed a case of LCCSCT of the testis with no associated endocrine abnormality. Our ultrastructural findings of rows of tight junctions, numerous intracytoplasmic filaments, and abundant rough endoplasmic reticulum in whorled formations support the Sertoli cell origin of this neoplasm.     

骨的恶性巨细胞瘤临床病理观察

目的 探讨骨的恶性巨细胞瘤临床病理学特征及其诊断和鉴别诊断.方法 对13例恶性巨细胞瘤的临床及病理学资料进行回顾性分析.结果 13例恶性巨细胞瘤中原发性恶性巨细胞瘤6例,发病年龄21-71岁,平均年龄39.5岁.复发后继发性恶性巨细胞瘤7例,发病年龄27-52岁,平均年龄36.7岁.6例原发性恶性巨细胞瘤除可见到骨巨细胞瘤区域外,还可见到高度恶性的梭形细胞肉瘤区域,7例继发性恶性巨细胞瘤其原发性肿瘤均为骨巨细胞瘤,而复发性肿瘤则呈恶性纤维组织细胞瘤/未分化肉瘤形态.结论 诊断恶性巨细胞瘤时需将临床、影像及病理结合,并除外其他肉瘤如富含巨细胞的骨肉瘤、富含巨细胞的恶性纤维组织细胞瘤等.     

卵巢幼年型粒层细胞瘤8例临床病理分析

目的 探讨卵巢幼年型粒层细胞瘤(juvenile granulosa cell tumor, JGCT)的临床病理特点、诊断及鉴别诊断.方法 回顾本院诊治的8例JGCT的临床、病理特征及免疫表型特点,并进行随访获知其预后情况.结果 8例JGCT患者发病年龄6～21岁,平均15.1岁.临床主要表现为腹部包块、腹水及女性假性性早熟.巨检表现为囊实性肿块.光镜下肿瘤细胞呈实性巢状,片状弥漫性排列,部分可排列成多个圆形或椭圆形大小不等的滤泡,少数可形成巨滤泡结构,有的滤泡腔内还可见均质红染物质.瘤细胞呈圆形、多边形,中等大小或较大,胞质丰富,空淡或微嗜酸性,核圆形,染色质均质状,无明显核沟,有一定异型性,可见核分裂象.免疫表型:瘤细胞均表达inhibin-α、CD99、vimentin,部分病例Melan-A、calretinin、S-100阳性,瘤细胞不表达CKpan、EMA、PLAP、Syn和CgA.结论 JGCT非常少见,属于低度恶性肿瘤,预后较好.确诊依赖于临床特点、组织形态学及免疫组化标记.病理诊断时要与卵巢的成人型粒层细胞瘤、高钙血症型小细胞癌、类癌、无性细胞瘤等肿瘤相鉴别.