A clinicopathological analysis of 40 cases of ovarian. Ayhan a, tuncer zs, hakverdi au et al 1996 sertolileydig cell tumor of the ovary. A long list of differential diagnosis, consisting of poorly differentiated sertolileydig cell tumor slct, sarcomatoid variant of adult granulosa cell tumor, luteinized thecoma, mitotically active fibroma, fibrosarcoma, and endometrial stromal sarcoma ess of ovary was considered initially in our case. The tumour occurs in early adulthood not seen in newborn, is rare, comprising less than 1% of testicular tumours. Sertoli leydig cell tumor medlineplus medical encyclopedia. Ovarian sertolileydig cell tumors with pseudoendometrioid tubules pseudoendometrioid sertolileydig cell. These are the accepted, unedited articles published online and citable. Sertoli leydig cell tumors slcts, also known as androblastomas, are defined as tumors composed of variable proportions of sertoli cells, leydig cells, andor fibroblastic cells in variable proportions and levels of differentiation. Mar 25, 2010 this type of tumor is sometimes called arrhenoblastoma of the ovary or a stromal tumor. Leydig cell tumour, also leydig cell tumor us spelling, testicular interstitial cell tumour and testicular interstitial cell tumor us spelling, is a member of the sex cordstromal tumour group of ovarian and testicular cancers. Sertoli leydig cell tumors genetic and rare diseases.
Sertolileydig cell tumor with heterologous elements heterologous epithelial or mesenchymal elements may be seen in approximately 20% of moderately or poorly differentiated tumors and in retiform sertolileydig cell tumor am j surg pathol 1985. The cancer cells produce and release a male sex hormone which may cause the development of male physical characteristics virilization, including facial hair and a deep voice. The cancer cells produce and release a male sex hormone called testosterone. Sertolileydig cell tumour a sexcord stromal tumor, is a testostronesecreting ovarian tumor and is a member of the sex cordstromal tumour group of ovarian and testicular cancers. Mucinous adenocarcinoma as heterologous element in intermediately differentiated sertolileydig cell tumor of the ovary. However, a study in austria demonstrated of all patients that are undergoing surgery for testicular tumor between 1999 and 2008, leydig cell tumors accounted for 14. May 15, 2007 histopathology testis sertoli cell tumor. The prognosis of ovarian sertoli leydig cell tumors is significantly correlated with the degree of tumor differentiation and the tumor extent. We report a case of a woman with a ovarian sertoli leydig cell tumor who presented with hirsutism.
Slct is a rare tumorof the sexual cordneoplasms, accountingforless than 1% of all primary ovarian tumors. Dec 14, 2018 learn indepth information on ovarian sertoli leydig cell tumor, its causes, symptoms, diagnosis, complications, treatment, prevention, and prognosis. The varying histopathology and differentiation of this tumor present difficulties with proper diagnosis and development of optimal treatment regimens. Please remove adblock adverts are the main source of revenue for dovemed. Pure sertoli granulosa cell tumors constitute fewer than 1% of testicular tumors. Among several types of ovarian tumors, sertoli leydig cell tumors are considered significantly rare, accounting for less than 1% of all primary ovarian tumors. While they can be found in women of all age groups, they are most common in young women.
Tubular seminoma, which often mimics sertoli cell tumor architecturally, can be separated from sertoli cell tumor by plap immunoreactivity and lack of staining for inhibin and cytokeratin. Difficult differential diagnoses in testicular pathology huihui ye, md. Sertoli cell tumor an overview sciencedirect topics. Sertoli leydig cell tumor slct is a rare variety ovarian tumor that belongs to the group of sexcord stromal tumors. Hirsutism caused by ovarian tumors accounts for approximately 1% of all cases of hirsutism. The final edited and typeset version of record will appear in the future. Pdf histogenesis and histopathological characteristics of sertoli. Jan 01, 20 there is evidence that some sertoli leydig cell tumors of the ovary might be caused by a genetic predisposition to develop cancer. When to apply these diagnostic assays and how to interpret the. Criteria for inclusion as retiform sertolileydig cell tumours8. Mutations in the dicer1 gene have been associated with an increased chance to develop sertoli leydig cell tumors of the ovary, as well as other types of cancer. Longterm followup is highly advised in such patients. Leydig cell tumors see the image below are derived from normal leydig cells that produce testosterone and are located in the interstitium of the testis.
A clinicopathological analysis of 40 cases of ovarian sertoli. Poorly differentiated sertolileydig cell tumor of ovary. Epidemiology may present in both pediatric and adult males, depending on the histologic subtype. In the united states, leydig cell testicular neoplasms are the most common sex cordstromal tumors and comprise % of all testicular neoplasms. Sertoli leydig cell tumors are rare tumors which account for less than 0. The prognosis depends on tumors grading and staging. Sertoli cell tumors are typically composed of solid tubules containing sertoli cells. The current evidence suggests a cell lineage relationship between these tumors and ovarian granulosa cells, which in turn are thought to be of mesonephric. The tumor has variable clinical and histopathological presentations complicating the diagnosis. Lymphadenectomy was not performed given the mucinous histology. Nineteen cases were obtained for the years 19852000 six afip, f. In a recent small sample, 12 of 20 cases 60% of sertoli cell tumor marked with cd99.
The differential diagnosis of cellular fibroma versus adult granulosa cell tumor may be problematic, as some granulosa cell tumors have a diffuse growth of cells. Histogenesis and histopathological characteristics of sertolileydig cell tumors article pdf available in cme journal of gynecologic oncology 72. Sertoli cell tumour with calcification in carney complex several images upmc. Independent of tumor stage, tumor grade is an important predictor of disease outcome with higher grade tumors behaving more aggressively. Sertolileydig cell tumours slct are rare tumours, accounting for less than 0. Final pathology, however, noted a moderately differentiated sertolileydig. Like leydig cell tumors, cd99 can be a useful adjunct in identifying sertoli cell tumors table 16. Sertolileydig cell tumour, sertolileydig cell tumour with retiform areas, or sex cordstromal tumour with retiform areas. Histopathology thyroid follicular adenoma microfollicular duration. Ovarian sertoli leydig cell tumors slct, also known as an ovarian androblastomas, are a subtype of ovarian sex cordstromal tumor. Jul 08, 2017 the tumor was multicystic and had solid areas. Dec 18, 2019 in the united states, leydig cell testicular neoplasms are the most common sex cordstromal tumors and comprise % of all testicular neoplasms.
Benign large cell calcifying sertoli cell tumor of the testis. While the tumour can occur at any age, it occurs most often in young adults. Also, cells may be arranged in cords see the first image below, solid nests, and sheets. Degree of heterologous element differentiation is a prognostic indicator. The overall 5year survival rate for moderatelydifferentiated grade 2 and poorlydifferentiated grade 3 sertoli leydig cell tumors is 80%. Children typically present with precocious puberty. Cd99 can be a useful adjunct in identifying sertoli cell tumors. Sertoli cell tumour an overview sciencedirect topics. Learn indepth information on ovarian sertolileydig cell tumor, its causes, symptoms, diagnosis, complications, treatment, prevention, and prognosis. Among several types of ovarian tumors, sertolileydig cell tumors are considered significantly rare, accounting for less than 1% of all primary ovarian tumors.
They are extremely rare and account for less than 0. An indepth look at leydig cell tumor of the testis. Most slcts have endocrine functions androgenic or estrogenic, and the prognosis is correlated most meaningfully with the degree of differentiation and stage of the tumor 2. They are very rare and generally peak between the ages of 35 and 50. The majority of leydig cell tumors are found in males, usually at 510 years of age or in middle adulthood 3060 years. Pdf pregnancy with recurrent sertoli leydig cell tumour. Sertoli cell tumors of the testis are uncommon sex cord stromal tumors. A sertoli cell tumour, also sertoli cell tumor us spelling, is a sex cordgonadal stromal tumor of sertoli cells. Click on the title to browse this list of accepted articles. Leydig cell tumor, can show solid growth patterns with.
Pdf histogenesis and histopathological characteristics of. Ovarian sex cordstromal tumors archives of pathology. A long list of differential diagnosis, consisting of poorly differentiated sertoli leydig cell tumor slct, sarcomatoid variant of adult granulosa cell tumor, luteinized thecoma, mitotically active fibroma, fibrosarcoma, and endometrial stromal sarcoma ess of ovary was considered initially in our case. We report a case of a woman with a ovarian sertolileydig cell tumor who presented with hirsutism. Bilateral, metachronic ovarian sertolileydig cell tumour in an 11. Well differentiated sertolileydig cell tumors are essentially benign.
They are less common than leydig cell tumors of the testis. Sertoli leydig cell tumor slct is a rare ovarian tumor that belongs to the group of sexcordstromal tumors. Tubular seminoma, which often mimics sertoli cell tumor architecturally, can be separated from sertoli cell tumor by its plap immunoreactivity and lack of staining for inhibin and cytokeratins. Practical roles for molecular diagnostic testing in ovarian. Ovarian sertolileydig cell tumor radiology reference. Among these, slct consists of 1% of sexcord tumors. Sertolileydig cell tumors slcts are rare tumors, accounting for less than 0. Large moderatelydifferentiated ovarian sertolileydig. Grade is usually based on microscopic features, including nuclear features. Typically, up to 14 percent sertoli cell tumors in dogs are malignant and will metastasize to surrounding lymph nodes in the body and other organs.
Axiotis 2007 an indepth look at leydig cell tumor of the testis. These tumors belong to the category of sex cordstromal tumors. Sertolileydig cell tumors are a cancer that starts in the female ovaries. In most of the mass, the cells lack identifying architectural patterns. These constitute sertoli leydig cell tumor slct is a rare cancer of the ovaries. Sertolileydig cell tumor of the ovary in a young female. Sertoli leydig cell tumor of ovary is a rare type of sexcord stromal tumor.
Sex cordstromal tumors account for 4% of testicular neoplasms, most commonly leydig thecal cell tumors. Approximately 10% of moderately differentiated and 59% of poorly differentiated tumors have malignant behavior, heterologous elements, retiform pattern, tumor rupture and spread outside the ovary stage ii or higher tumor stage are adverse prognostic factors. Sertolileydig cell tumors slcts are a rare type of sex cord stromal tumor of the ovary, accounting for less than 0. Varying histopathology of sertolileydig tumors presents a diagnostic challenge. Sertolileydig cell tumors genetic and rare diseases. They are typically welldifferentiated, and may be misdiagnosed as seminomas as they often appear very similar.
Ovarian sertolileydig cell tumors with a retiform pattern. Testicular sex cord stromal tumors include leydig cell tumors, sertoli cell tumors, and granulosa cell tumors. Sertolileydig cell tumors slcts, also known as androblastomas, are defined as tumors composed of variable proportions of sertoli cells, leydig cells, andor fibroblastic cells in variable proportions and levels of differentiation. The final histopathologic diagnosis was benign large cell calcifying sertoli cell tumor lccsct of the testis. Sertolileydig cell tumor of ovary, management and prognosis. Pdf histogenesis and histopathological characteristics. A sertolileydig cell tumour is a combination of a leydig cell tumour and a sertoli cell tumour from sertoli cells. Sertoli cell tumors are a form of testicular tumor in dogs, and are linked with undescended testicles. They are typically welldifferentiated, and may be misdiagnosed as seminomas as they often appear very similar a tumor that produces both sertoli cells and leydig cells is. The sertoli cells are located in the male reproductive glands, which produce. Sertolileydig cell tumor with heterologous element. Dec 17, 2019 the recent identification of foxl2, dicer1 and ctnnb1 mutations in adult granulosa cell tumours agcts, sertolileydig cell tumours slcts, and microcystic stromal tumours mcsts, respectively, ushered in the era of molecular diagnostics for ovarian sex cordstromal tumours. Difficult differential diagnoses in testicular pathology. Polygonal to spindle cell tumor, anaplastic strongly favor sertoli cell tumor.