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Rarity of Sertoli cell tumours contributes to a low index of suspicion and therefore a thorough knowledge of the clinicopathological and immunological characteristics of such tumours is essential to diagnosis and proper management of the treatment and follow-up. The current narrative review of literature was planned to focus on ovarian Sertoli cell tumours that arise from the sex cords cells, which are typically benign unilateral neoplasia incidentally detected, or associated with hormonal hyperactivity, in women of reproductive age.

A priory unpublished case of a year old female is also introduced as the base of discussion Abdominal massrelated syndrome and vaginal bleeding anomalies have been reported. The tumour displays a microscopic tubular pattern and rarely displays cords or trabecular, retiform, spindles, diffuse or areolar structures. Although immunohistochemistry can be helpful in establishing the diagnosis, the are sometimes inconclusive and the current require new research to establish a specific immunological panel.

Introduction Ovarian Sertoli cell tumours OSCTs are rare neoplasia associating a relatively large of morphological aspects in addition to heterogeneous endocrine behaviour. Typically benign, they are frequently seen in women of reproductive age with ranges between first years of life to the seventh decade. Therefore, neoplasia is usually detected by a gynaecologist or an endocrinologist. The underlying histological reports include these tumours in the vast family of sex cord-stromal tumours, 2,3 and some have a genetic background, as seen in Peutz-Jeghers syndrome or DICER mutation the gene encoding endoribonuclease Dicerthe latter more frequently associated with Sertoli- Leydig cell tumours.

The patient ed the informed written consent to anonymously use her medical data referring to the pathological report as well as medical history and hormonal profile. The research of literature Women seeking sex Cord focused on clinical and histological aspects of OSCT, as a topic of gynaecological endocrinology.

Most studies were found while searching on PubMed. Main characteristics The onset age is between 2 and 79 years, with an average of about 30 years. Classically, they are considered tumours of reproductive age and, therefore, the possibility of detection during periodical gynaecological control. Postmenopausal vaginal bleeding in association with high cancer antigen CA levels mimicking an ovarian cancer has been described. Figure 1 Pelvic computed tomography CT confirmed a solid, well-shaped left ovarian tumour of 3.

The most common clinical presentation when occurring in children is isosexual precocity. Fine needle aspiration FNA is cited as useful in one large series of cases in order to appreciate the malignant potential as alternative of surgery. Therefore, the primary treatment is surgical unilateral oophorectomy and the prognosis is generally favourable. Histological and immunohistochemistr y feature Pathology and immunocytochemistry are basic tools for the diagnosis of OSCT, having Women seeking sex Cord a prognostic value.

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Macroscopically, the tumour is yellow or brown, usually solid, with potential mix Women seeking sex Cord solid-cyst. The diameter varies from 7,8,23 Their morphological spectrum are not clearly established. Many other neoplasms of diverse types can closely simulate Sertoli cell tumours. The neoplasia with Sertoli cells of gon, ovary and testes, have common by the lobular aspect, but the lobulation is less evident in ovaries than the testis.

Other features have been identified in ovarian Sertoli cell tumours like cord or trabecular, retiform, spindles, diffuse or alveolar areas. Several pattern scan coexist in the same tumour. Thestroma devoid of Leydig cells can be abundant with marked hyalinisation.

It can be noticed round or elongated tubules, hollow or solid, lined by columnar or cuboidal Sertoli-like cells, with moderate to abundant amounts of slightly eosinophilic cytoplasm, occasionally vacuolated. The nuclei of the tumoural cells usually lack atypical features or mitotic activity. Ultrastructurally, abundant trough endoplasmic reticulum, some cystically dilated smooth endoplasmic reticulum profile and mitochondria with tubular crystals visible. These, together with lipid droplets, suggest a potential or steroidhormone synthesis which is not always expressed clinically. The free border of cell show occasional cilia or microvili, considered to be a manifestation of focal metaplasia of the neoplastic Sertoli cell.

Laterally, the cells show tight junc tions and desmosomes. The immunohistochemical IHC panel can be helpful in establishing the diagnosis. Some reports revealed calretinin-positive reaction in more than half of the cases, as well as vimentin, and inhibin. IHC report seems to be more useful in cases where classical tubular pattern is not clearly identified.

In our case, tumour cells were positive for vimentin and CK7, but negative for inhibin, with a low Ki67 index of proliferation which Women seeking sex Cord usually correlated with tumour progression. Pathological report after left laparoscopic ovarectomy on a year-old asymptomatic female with ovarian incidentaloma showed a benign sex cord tumour with Sertoli cells Figure 3A. Positive actin was found in stroma but not into the tumour cells.

Rarity of Sertoli cell tumours cases reported to date contributes to a low index of suspicion and therefore at thorough knowledge of the clinicopathological and immunological characteristics of such tumours is essential for diagnosis and proper management right up to treatment and patient follow-up. As current limits of the concept related to OSCT, we consider five aspects: the insufficient description of risk factors and genetic predisposition, especially in non-Peutz Jeghers syndrome cases, and the exact role of identifying DICER1 mutation s in selected cases; which is the driven force of endocrineactivity in relationship to a particular histological expression; lack of high specificity markers regardingIHC features; the poor prognosis markers are still a matter of debate, an aspect that cannot be sustained based on small sample size studies; and, finally, which is the clear definition and multi-disciplinary protocol for ovarian.

Sertoli cell tumour of the ovary is a rare neoplasia of low malignancy risk that typically occurs in women of reproductive age, and it has a good prognosis in association with the usual tubular pattern.

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IHC may be useful but current require new research to establish a specific immunological panel. The detection of the tumour and its follow-up after removal may be done either by a gynaecologist or an endocrinologist. Ovarian Sex Cord-Stromal Tumours. J Oncol Pract ; Menopausal androgen excess-associated cardio-metabolic risk: clues for ovarian Leydig cell tumour case report and mini-review of literature. Virilising Sertoli-Leydig cell tumour associated with thyroid papillary carcinoma: case report and general considerations. Gynecol Endocrinol ; Int J Surg Pathol ; DM, et Women seeking sex Cord.

Gynecol Oncol ; Familial multi nodular goiter and sertoli-Leydig cell tumours associated with a large intragenic in-frame DICER1 deletion. Eur J Endocrinol ; KK Sertoli cell tumours of the ovary: a clinicopathologic and immunohistochemical study of 54 cases. Am J Surg Pathol ; Pure Sertoli cell tumour. A case report and review of the literature. Eur J Gynecol Oncol ; Young RH. Ovarian tumours and tumour-like lesions in the first three decades.

Semin Diagn Pathol ; Hum Pathol ; Ovarian tumors secreting androgens: an infrequent cause of hyperandrogenism. Minerva Ginecol ; Clinical syndromes associated with ovarian neoplasms: a comprehensive review. Radiographics ; A year-old woman with abdominal distention, vaginal bleeding, and elevated CA level. Pure Sertoli cell tumour of the ovary with differentiation varying from well-differentiated tubules, to intermediate foci, to sarcomatoid spindle cell areas. Arch Pathol Lab Med ; e Incidentaloma: from general practice to specific endocrine frame.

J Pak Med Assoc ; Solnik MJ, Alexander C. Ovarian incidentaloma. A pure Sertoli cell tumour of the ovary in ayear-old female. J Pediatr Adolesc Gynecol ; Surg Pathol Clin ; 9: FOXL 2 mutation is absent in uterine tumours resembling ovarian sexcord tumours. Pure Sertoli cell tumour of the ovary with Meig's syndrome. Pathology Women seeking sex Cord Clinicopathological spectrum of ovarian sexcord-stromal tumours.

J Ovarian Res ; 6: Ovarian sex cord-stromal tumours and their mimics. Perspectives on testicular sex cord-stromal tumors and those composed of both germ cells and sex cord-stromal derivatives with a comparison to corresponding ovarian neoplasms.

Ovarian sex-cord stromal tumours and small cell tumours: Pathological, genetic and management aspects. Crit Rev Oncol Hematol ; Sertoli-stromal cell tumour of the ovary: immunohistochemical, ultrastructural, and genetic studies. Scully, MD. Arch Pathol Lab Med ; Lim D, Oliva E.

Ovarian sex cord-stromal tumours: an update in recent molecular advances. Comparative immunohistochemical analysis of granulose and sertoli components in ovarian sexcord-stromal tumours with mixed differentiation: potential implications for derivation of sertoli differentiation in ovarian tumours.

Int J Gynecol Pathol ; Diagnostic utility of WT1 immuno staining in ovarian sertoli cell tumour. Comparative analysis of alternative and traditional immunehistochemical markers for the distinction of ovarian sertoli cell tumour from endometrioid tumours and carcinoid tumour: A study of cases.

Contribution of lymph node staging method and prognostic factors in malignant ovarian sex cord-stromal tumors: A world wide database analysis. Eur J Surg Oncol ; Genetics and genomics of ovarian sex cordstromal tumours. Clin Genet ; Journal of the Pakistan Medical Association has agreed to receive and publish manuscripts in accordance with the principles of the following committees:.

About Us Disclaimer. By Author. By Article. Advanced Search. In all Journals JanuaryVolume 70, Issue 1 Narrative Review. Ovarian Sertoli cell tumours: practical points.

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