Sex cord-stromal tumor with annular tubules of the ovary – a case report

Sex cord-stromal tumors with annular tubules (SCTAT) of the ovary are rare. They have two clinicalpresentation forms: the syndromic form which is associated with Peutz-Jeghers syndrome and thenon-syndromic form which is frequently seen in the second or third decades. We describe a 25-year-old patient who underwent exploratory laparotomy. Macroscopically large ovarian mass was24.5×24×8 cm in diameter, encapsulated, congested, and lobulated. On the cut section, it wasgreyish with small cystic and hemorrhagic areas. Microscopically, the tumor mass is composed ofmany simple and complex tubular structures that have eosinophilic PAS-positive hyaline globules inthe center and are surrounded by peripheral palisading of the cells. Finally, the tumor was diagnosedas non-syndromic ovarian SCTAT.

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Ovarian Steroid Cell Tumor (Not Otherwise Specified): A Case Report of Ovarian Hyperandrogenism

Case Reports in Oncological Medicine ◽

10.1155/2020/6970823 ◽

2020 ◽

Vol 2020 ◽

pp. 1-6

Author(s):

Hadjkacem Faten ◽

Ghorbel Dorra ◽

Charfi Slim ◽

Safi Wajdi ◽

Charfi Nadia ◽

...

Keyword(s):

Transvaginal Ultrasound ◽

Cell Tumor ◽

Ovarian Mass ◽

Stromal Tumors ◽

Not Otherwise Specified ◽

Fertility Sparing Surgery ◽

Fertility Sparing ◽

Steroid Cell Tumor ◽

Ovarian Hyperandrogenism ◽

Sex Cord Stromal Tumors

Steroid cell tumors (SCTs) (not otherwise specified (NOS)) are rare sex cord-stromal tumors of the ovary. These are associated with hormonal disturbances resulting in menstrual bleeding patterns and androgenic effects. We report the case of a 36-year-old female presented with hirsutism, signs of virilization, and elevated androgen levels. Transvaginal ultrasound showed a solid-appearing right ovarian mass. She underwent fertility-sparing surgery with a laparoscopic left oophorectomy. Histological examination showed a benign steroid cell tumor, NOS. These tumors often small can then present a problem of positive diagnosis responsible for a delay in the diagnosis.

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Retiform Sertoli-Leydig Cell Tumor in a 38-Year-Old Woman: A Case Report, Retrospective Review, and Review of Current Literature

Case Reports in Pathology ◽

10.1155/2017/3421832 ◽

2017 ◽

Vol 2017 ◽

pp. 1-8 ◽

Cited By ~ 1

Author(s):

Laura C. Nwogu ◽

Josh A. Showalter ◽

Suvra Roy ◽

Michael T. Deavers ◽

Bihong Zhao

Keyword(s):

Leydig Cell ◽

Stromal Cells ◽

Ovarian Neoplasms ◽

Leydig Cell Tumor ◽

Cell Tumor ◽

Ovarian Mass ◽

Immunohistochemical Examination ◽

Stromal Tumors ◽

Sex Cord Stromal Tumors ◽

Leydig Cell Tumors

Ovarian sex cord-stromal tumors arise from the stromal cells that surround and support the oocytes. Sertoli-Leydig cell tumors belong to this category of ovarian neoplasms. We present the case of a 38-year-old woman who was found to have a right ovarian mass. The mass was resected and diagnosed as Stage I Sertoli-Leydig cell tumor, retiform variant, following histopathologic and immunohistochemical examination. This case is unusual given the rarity of the retiform variant of Sertoli-Leydig cell tumor and the atypically older age of 38 years at presentation.

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Preoperative Differentiation of Benign and Malignant Non-epithelial Ovarian Tumors: Clinical Features and Tumor Markers

Revista Brasileira de Ginecologia e Obstetrícia / RBGO Gynecology and Obstetrics ◽

10.1055/s-0040-1712993 ◽

2020 ◽

Vol 42 (09) ◽

pp. 555-561

Author(s):

Tiago Augusto Gomes ◽

Elizabeth Aparecida Campos ◽

Adriana Yoshida ◽

Luís Otavio Sarian ◽

Liliana Aparecida Lucci de Angelo Andrade ◽

...

Keyword(s):

Germ Cell ◽

Clinical Features ◽

Germ Cell Tumors ◽

Serum Levels ◽

Cell Tumor ◽

Ovarian Tumors ◽

Stromal Tumors ◽

Fertility Sparing ◽

Sex Cord Stromal Tumors ◽

Sex Cord Stromal Tumor

Abstract Objective To evaluate the role of clinical features and preoperative measurement of cancer antigen 125 (CA125), human epididymis protein (HE4), and carcinoembryonic antigen (CEA) serum levels in women with benign and malignant non-epithelial ovarian tumors. Methods One hundred and nineteen consecutive women with germ cell, sex cord-stromal, and ovarian leiomyomas were included in this study. The preoperative levels of biomarkers were measured, and then surgery and histopathological analysis were performed. Information about the treatment and disease recurrence were obtained from the medical files of patients. Results Our sample included 71 women with germ cell tumors (64 benign and 7 malignant), 46 with sex cord-stromal tumors (32 benign and 14 malignant), and 2 with ovarian leiomyomas. Among benign germ cell tumors, 63 were mature teratomas, and, among malignant, four were immature teratomas. The most common tumors in the sex cord-stromal group were fibromas (benign) and granulosa cell tumor (malignant). The biomarker serum levels were not different among benign and malignant non-epithelial ovarian tumors. Fertility-sparing surgeries were performed in 5 (71.4%) women with malignant germ cell tumor. Eleven (78.6%) patients with malignant sex cord-stromal tumors were treated with fertility-sparing surgeries. Five women (71.4%) with germ cell tumors and only 1 (7.1%) with sex cord-stromal tumor were treated with chemotherapy. One woman with germ cell tumor recurred and died of the disease and one woman with sex cord-stromal tumor recurred. Conclusion Non-epithelial ovarian tumors were benign in the majority of cases, and the malignant cases were diagnosed at initial stages with good prognosis. The measurements of CA125, HE4, and CEA serum levels were not useful in the preoperative diagnosis of these tumors.

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Genetics and Mutational Landscape of Ovarian Sex Cord-Stromal Tumors

10.5772/intechopen.97540 ◽

2021 ◽

Author(s):

Trang Nguyen ◽

Trang Minh Tran ◽

Yee Shen Choo ◽

Maria Alexiadis ◽

Peter J. Fuller ◽

...

Keyword(s):

Chromosomal Abnormalities ◽

Stromal Tumors ◽

Activating Mutations ◽

Molecular Events ◽

Gsp Oncogene ◽

Genomic Studies ◽

Peutz Jeghers Syndrome ◽

Dicer1 Syndrome ◽

Human Telomerase ◽

Sex Cord Stromal Tumors

Ovarian sex cord-stromal tumors (SCST) are uncommon tumors accounting for approximately 8% of all ovarian malignancies. By far, the most common are granulosa cell tumors (GCT) which represent approximately 90% of SCST. SCST are also found in the hereditary syndromes: Peutz-Jeghers syndrome, Ollier disease and Maffucci syndrome, and DICER1 syndrome. Key genomic and genetic events contributing to their pathogenesis have been the focus of recent studies. Most of the genomic studies have been limited to GCT which have identified a number of recurring chromosomal abnormalities (monosomy and trisomy), although their contribution to pathogenesis remains unclear. Recurrent DICER1 mutations are reported in non-hereditary cases of Sertoli cell and Sertoli–Leydig cell tumors (SLCT), while recurrent somatic mutations in both the juvenile (jGCT) and adult forms of GCT (aGCT) have also been reported. Approximately 30% of jGCT contain a somatic mutation in the gsp oncogene, while a further 60% have activating mutations or duplications in the AKT gene. For aGCT, a well characterized mutation in the FOXL2 transcription factor (FOXL2 C134W) is found in the majority of tumors (primary and recurrent), arguably defining the disease. A further mutation in the human telomerase promoter appears to be an important driver for recurrent disease in aGCT. However, despite several studies involving next generation sequencing, the molecular events that determine the stage, behavior and prognosis of aGCT still remain to be determined. Further, there is a need for these studies to be expanded to other SCST in order to identify potential targets for personalized medicine.

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MIXED GERM CELL-SEX CORD-STROMAL TUMORS

JURNAL BIOMEDIK (JBM) ◽

10.35790/jbm.4.3.2012.1207 ◽

2013 ◽

Vol 4 (3) ◽

Author(s):

Poppy M. Lintong

Keyword(s):

Germ Cell ◽

Germ Cells ◽

Stromal Tumor ◽

Stromal Tumors ◽

Unusual Phenomenon ◽

Microscopic Features ◽

Germinal Cells ◽

Histopathologic Feature ◽

Sex Cord Stromal Tumors ◽

Sex Cord Stromal Tumor

Abstract: The term mixed germ cell sex-cord-stromal tumors is used for a mixed tumor containing mixed germ cells and sex-cord-stromal tumors. In pathology, this is an unusual phenomenon because although the tumor has several components, the histopathologic feature is still as a single unit. The most frequent form of mixed germ cell and sex-cord-stromal tumors is gonadoblastoma. This tumor usuallly occurs in sexual development abnormality, and can be found either in ovarium or testis. The basic microscopic features of this tumor are a mixture of dysgerminoma-like primitive germinal cells, and granulosa and immature Sertoli like cells of the sex-cord-stromal tumor. Besides gonadoblastoma, there is an unclassified tumor consisting of mixed germ cells and sex-cord-stromal tumor which cover all tumors containing a mixture of germinal cells and sex-cords but its cellular characteristics differ from gonadoblastoma. Keywords: mixed germ cell, sex cord-stromal tumors, gonadoblastoma. Abstrak: Istilah mixed germ cell - sex cord-stromal tumors sering digunakan untuk tumor campuran mixed germ cell dan sex cord-stromal tumors. Dalam bidang patologi hal ini merupakan suatu fenomena luar biasa karena meskipun terdapat beberapa komponen tumor namun tetap memberikan gambaran histopatologik sebagai suatu kesatuan tunggal. Bentuk paling sering dari mixed germ cell - sex cord-stromal tumors ialah gonadoblastoma. Gonadoblastoma sering ditemukan pada individu dengan perilaku seksual tidak normal. Selain pada ovarium, tumor ini juga dapat ditemukan pada testis. Gambaran mikroskopik yang mendasar dari tumor ini ialah campuran dari sel-sel germinal primitif menyerupai disgerminoma dan sel stroma sex-cord menyerupai sel-sel granulosa dan sertoli imatur. Selain gonadoblastoma, juga terdapat mixed germ cell - sex cord-stromal tumors unclassified yang mencakup semua tumor yang mengandung campuran bermacam-macam sel germinal dan sex-cord namun karakteristik selulernya tidak serupa dengan gonadoblastoma. Kata kunci: mixed germ cell, sex cord-stromal tumors, gonadoblastomsa.

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Sex cord-stromal tumors of the ovary: road map for progress

International Journal of Gynecological Cancer ◽

10.1136/ijgc-2020-002329 ◽

2021 ◽

Vol 31 (2) ◽

pp. 169-170

Author(s):

R. Tyler Hillman ◽

David Gershenson

Keyword(s):

Stromal Tumors ◽

Road Map ◽

Sex Cord Stromal Tumors

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Ovarian sex cord-stromal tumors: an update on clinical features, molecular changes, and management

International Journal of Gynecological Cancer ◽

10.1136/ijgc-2020-002018 ◽

2021 ◽

pp. ijgc-2020-002018

Author(s):

Rehab Al Harbi ◽

Iain A McNeish ◽

Mona El-Bahrawy

Keyword(s):

Hormonal Therapy ◽

Clinical Presentation ◽

Therapeutic Modality ◽

Biological Behavior ◽

Molecular Changes ◽

Histological Features ◽

Stromal Tumors ◽

Tumor Subtypes ◽

Tumor Types ◽

Sex Cord Stromal Tumors

Sex cord stromal-tumors are rare tumors of the ovary that include numerous tumor subtypes of variable histological features and biological behavior. Surgery is the main therapeutic modality for the management of these tumors, while chemotherapy and hormonal therapy may be used in some patients with progressive and recurrent tumors. Several studies investigated molecular changes in the different tumor types. Understanding molecular changes underlying the development and progression of sex cord-stromal tumors provides valuable information for diagnostic and prognostic biomarkers and potential therapeutic targets for these tumors. In this review, we provide an update on the clinical presentation, molecular changes, and management of sex cord-stromal tumors.

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