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Testicular sex cord-stromal tumors are clonal neoplasms, with the majority being of Leydig cell followed by Sertoli cell origins. In Leydig cell tumors, adipocytic differentiation has been previously reported as a possible distinguishing feature, which has not been reported in other sex cord-stromal tumors. Herein, we report a case of a 48-year-old man who presented with an incidentally discovered 1.1 cm testicular mass, for which he underwent partial orchiectomy. Microscopically, the tumor showed features consistent with sex cord-stromal tumor with strong and diffuse nuclear and cytoplasmic reaction for B-catenin immunohistochemistry, supporting the diagnosis of Sertoli cell tumor. A novel adipocytic differentiation, reported previously in Leydig cell tumors, was present in this tumor., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

A 45-year-old man was referred to our hospital with a complaint of right scrotal discomfort. With a diagnosis of testicular tumor, right orchiectomy was performed. The tumor was histologically diagnosed as malignant Sertoli cell tumor pT1N0M0. A pulmonary nodule appeared, 53 months after the operation, and increased in size there after. Thoracoscopic left upper lobectomy was performed 64 months after the operation, and the pathological diagnosis was metastasis of malignant Sertoli cell tumor. No recurrence has been observed for 94 months after the resection of the metastatic lesion.

Characteristic ultrasound features of large cell calcifying Sertoli cell tumor (LCCSCT), including hypoechoic masses with amorphous coarse calcifications can aid in differentiating this tumor from other entities. Bilateral multiple LCCSCTs almost always show a benign course; therefore, defining the diagnosis with sonographic findings is crucial to avoid unnecessary orchiectomy., (© 2023 Wiley Periodicals LLC.)

Background: Testicular Sertoli cell tumor (SCT) is very rare sex cord-gonadal stromal tumor, and sclerosing SCT (SSCT) is even rarer. So far, no more than 50 cases of SSCT have been reported. 80% of SSCTs are less than 2 cm in diameter, large volume mass is pretty unusual. SSCT is usually benign with very low malignant potential. However, it is easily misdiagnosed as a malignant tumor resulting in the removal of the entire testicle., Case Presentation: A 55-year-old Chinese male patient presented with a six months' history of right testis progressively enlargement and negative tumor markers. The physical examination was nothing special except for swelling in the right testicle. Imaging identified a large mass in right testicle with rich blood. A right radical orchiectomy was performed on suspicion of malignancy. However, the tumor was postoperatively diagnosed as SSCT, which pathologically consisted of a tubular pattern with regular nuclei and embedded in a densely collagenous stroma, as well as diffusely positive for vimentin, β-catenin and synaptophysin. After 7 months of follow up, no evidence of local recurrence and metastasis has been observed., Conclusion: This rare case is helpful to expand the knowledge of the testicular tumor and alert us fully understand the rare variant of SCTs in order to choose the optimal management when they encounter SSCT., (© 2023. The Author(s).)

The Sertoli cell tumor of the ovary is a rare ovarian tumor with non-specific symptoms. According to the literature, endocrine manifestations occur in two-thirds of patients, but testosterone production is extremely rare. Typically, it is a unilateral benign tumor of the ovary that most commonly presents in adolescents and young women of childbearing potential. We report a 29-year-old patient, previously diagnosed to have polycystic ovarian syndrome, who presented with complaints of amenorrhea for the past three years. A transvaginal ultrasound scan revealed polycystic structure ovaries and a solid cystic formation of 32 × 31 mm size with strong blood flow in the left ovary. The laboratory tests reported an elevated testosterone level. During laparoscopic surgery, a solid, yellowish tumor was removed and the left ovary was resected. Histological examination revealed a left ovary Sertoli cell tumor with an immature prepubertal-like Sertoli cell component. Following surgery, the serum testosterone levels returned to normal and the menstrual cycle became regular. Due to the substantially low incidence of ovarian Sertoli cell tumors, information on their clinical behavior, morphologic spectrum, optimal management, and prognosis is limited. They are characterized by a wide variety of clinical manifestations, treated surgically, and, if diagnosed at an early stage, have good prognosis. We emphasize the extraordinarily rare clinical presentation of this case report.

Sertoli cell tumours are one of the most common canine testicular neoplasia. These tumours are significantly more likely to arise in cryptorchid dogs and are often functional, oestrogen-secreting tumours which can lead to fatal myelotoxicity. The goal of this study was to describe the outcome of dogs with oestrogen-induced bone marrow suppression secondary to Sertoli cell tumours in seven client-owned dogs. Medical records from April 1, 2011 through April 1, 2021 were reviewed to identify dogs that underwent surgical management of a Sertoli cell tumour with documented bone marrow suppression. Overall, 5/7 dogs required transfusion of blood products peri-operatively. Cases 1 and 6 received a transfusion of packed red blood cells (RBC) prior to surgery and case 5 required a transfusion of whole blood. Case 1 also required a transfusion of platelets before surgery. Post-operatively, cases 1 and 2 received packed RBC's and case 6 received two transfusions of whole blood. Case 3 required transfusions of both fresh frozen plasma and platelets post-operatively. All dogs survived to discharge and 6/7 dogs had documented improvement in haematopoietic values. Two dogs remained chronically thrombocytopenic. The median hospital stay was 4 days. One dog died within 4 weeks of surgery from worsening pancytopenia. Survival for greater than 1 year was documented in 4/7 dogs, and one dog was lost to follow-up 4 months post-operatively. One dog remained severely pancytopenic 4 weeks post-operatively and received oral lithium treatment. Improvements in all blood cell lines were observed within the 4 weeks and resolution of pancytopenia within 6 weeks. Historically, the prognosis for dogs with bone marrow suppression secondary to Sertoli cell tumours was guarded to poor. This report documented improved outcomes for dogs that underwent surgery, including one dog that received lithium chloride as treatment for Sertoli cell tumour-induced bone marrow suppression., (© 2021 John Wiley & Sons Ltd.)

We present a series of 18 (8 clinically benign, 8 clinically ambiguous [ie, lacking sufficient follow-up to determine behavior], and 2 clinically malignant) large cell calcifying Sertoli cell tumors (LCCSCT) of the testis. The median patient age and size were 15.5 years and 1.9 cm for the benign tumors; 19 years and 1.6 cm for the ambiguous tumors; and 28.5 years and 2.3 cm for the malignant tumors. The most common presentation was a mass (n=12/18, 67%). Two patients (11%) had the Carney complex, and 2 had neurofibromatosis type 1. All tumors showed nodular growth with frequent lymphoid aggregates at the periphery. Within the nodules, there were nests and trabeculae of pale to eosinophilic epithelioid tumor cells with frequent cytoplasmic vacuolization interspersed with hypocellular, often myxoid stroma with conspicuous neutrophils. Spindled tumor cells were a minor component (<5%) in the clinically benign, ambiguous, and malignant tumors, except in 1 malignant tumor where they comprised 50% to 60% of the cellularity. Calcifications were noted in all but 2 benign tumors that were otherwise of typical appearance. Six tumors (3 in the clinically benign, 1 in the clinically ambiguous, and 2 in the malignant groups) were considered potentially malignant based on the presence of ≥1 adverse pathologic features previously recognized (see reference 1)-that is, size>4 cm, extratesticular growth, necrosis, significant atypia, vascular invasion, and >3 mitotic figures/10 HPFs. Of these, 3 tumors had ≥2 adverse features. One in a 7-year-old was clinically benign despite 5 "malignant" features; the remaining 2 in 27- and 30-year-olds, were clinically malignant, with both fulfilling previously suggested criteria for pathologically malignant tumors (age above 25 y and ≥2 adverse pathologic features). No clinically benign or ambiguous tumor met those same criteria. Of the adverse features, each of the 2 clinically malignant tumors showed tumor necrosis and lymphovascular invasion. All patients, except 1 with a clinically malignant tumor, were alive at a median follow-up of 33 months. In addition, in our literature review of 97 additional LCCSCTs, we identified 2 clinically malignant tumors in 42- and 45-year-old men that lacked any documented adverse pathologic criterion and 2 clinically malignant cases in patients with either the Carney complex or Peutz-Jeghers syndrome. In summary, our study and literature review support that all LCCSCTs in patients above 25 years old should be considered potentially malignant, and those in this age group with ≥2 adverse pathologic features warrant aggressive clinical management; furthermore, syndrome-associated cases are not uniformly benign. Tumor necrosis and lymphovascular invasion likely should receive greater adverse prognostic weight. LCCSCTs in young children may show benign outcomes despite several adverse pathologic features., Competing Interests: Conflicts of Interest and Source of Funding: The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Rare tumors of the testis not originating from germinal epithelium are a diagnostic and therapeutic challenge., Objectives: To present current approaches in rare tumors of the testis using the examples of Sertoli cell tumor (SCT) and malignant mesothelioma of the tunica vaginal testis (MMTVT)., Methods: A literature search in PubMed and the abstract databases of ASCO and ESMO was performed. Articles and book chapters were selected based on relevance to everyday treatment., Results: The low incidence of testicular tumors not originating from the germinal epithelium makes a standardized approach difficult. Diagnosis and treatment depend on the underlying diagnosis. While most SCT are benign, malignant subtypes require extensive resection including metastatic surgery if complete resection is possible. In MMTVT, multimodality treatment concepts are followed, according to the malignant mesotheliomas of the pleura., Conclusion: Systematic registration of rare testicular tumors and comprehensive molecular pathological analysis are urgently needed to improve the understanding of tumor biology and to develop new therapeutic strategies., (© 2021. Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

An 11-year-old miniature poodle dog was presented with bilateral flank alopecia, gynecomastia, severe thrombocytopenia, and preputial edema. Based on characteristic clinical and hematological findings of hyperestrogenism and the presence of a caudal abdominal mass, a Sertoli cell tumor (SCT) was diagnosed. After a platelet concentrate transfusion, the SCT was surgically removed in addition to an atrophied contralateral testicle containing a mixed germ cell-stromal cell tumor. Recovery was uneventful. This combination of different neoplasms in separate testicles has yet to be documented. Key clinical message: This case of a SCT/mixed germ cell-stromal cell tumor combination in a bilaterally abdominal cryptorchid dog highlights common clinical signs associated with hyperestrogenism and the management of estrogen-induced myelotoxicity causing severe thrombocytopenia., (Copyright and/or publishing rights held by the Canadian Veterinary Medical Association.)

Introduction: There are less than 100 cases of Large-cell calcifying Sertoli cell tumour (LCCSCT) reported in English literature. Most of them are benign, bilateral and affect paediatric population. Malignant cases occur in older patients. LCCSCT is often associated with Carney complex or Peutz-Jaghers syndrome. We present the clinicopathological features of a young adult, with unilateral "stone-like" LCCSCT, without changes in hormonal status and no clinical characteristics of noted genetic disorders., Case Report: A 24-year-old male presented with painless hardening of the right testis. There was no gynaecomastia, and serum levels of human chorionic gonadotropin and α-fetoprotein were normal. Ultrasound depicted hyperechogenic, clearly demarcated intratesticular lesion. Partial orchiectomy was performed. Macroscopically, tumour appeared as almost entirely calcified round mass, measuring 10 mm. Histopathological evaluation showed well-circumscribed, unencapsulated tumour composed of massive calcified geographic formations, surrounded with tumour cells. Neoplastic cells were large, polygonal, with abundant eosinophilic cytoplasm, and formed irregular cords, pseudo tubular structures, and nests in a fibrous and myxoid stroma, surrounded with lymphocytes. Other forms of calcification were also present: Needle-like deposits and lamellar, mulberry-like structures. There was no necrosis, mitotic activity and nuclear pleomorphism. Immunohistochemical study was positive for inhibin α and negative for Melan A, EMA, synaptophysin, chromogranin and AFP., Discussion: LCCSCT needs to be differentiated from other, more frequent, sex cord stromal tumours. Clinical and genetical evaluation of these patients had to be performed, due to connection of LCCSCT with genetic abnormalities. In evidently benign cases, organ-sparing surgery should be considered for younger patients, followed by long term follow-up.

Background: Patients with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome are infertile secondary to hypoplasia or complete agenesis of the uterus, yet they remain at risk of primary neoplasms of the ovaries because embryologically the uterus and ovaries develop via separate mechanisms., Case: A 72-year-old nulliparous woman with a history of primary amenorrhea underwent an exploratory laparotomy for a suspected uterine fibroid. In addition to the pelvic mass, the patient was found to have findings consistent with MRKH syndrome. Postoperative pathological examination demonstrated bilateral ovarian Sertoli cell tumors., Conclusions: The case presented is unique in that 2 rare pathologies, bilateral Sertoli cell tumors of the ovary and MRKH syndrome, developed concomitantly in the same patient.

Purpose: Small benign testicular masses are often misinterpreted as germ cell tumors and immediate inguinal orchiectomy is performed. We analyzed the diagnostic and therapeutic workup of testicular masses to improve preoperative stratification algorithms., Materials and Methods: We performed a retrospective, single center analysis of the records of 522 patients diagnosed with primary testicular masses of unknown malignant potential., Results: A total of 28 patients (5%) showed a primary benign tumor after resection, including Leydig cell tumors in 9 (32%), epidermoid cysts in 9 (32%), adenomatoid tumors in 8 (29%) and Sertoli cell tumors in 2 (7%). The median volume of benign tumors was significantly less than that of malignant tumors (0.75 cm 3 , range 0.1 to 2.1 vs 15, range 4.5-39.9, p ≤0.001). At a cutoff of 2.8 cm 3 tumor volume most accurately differentiated between benign and malignant disease, and it was a predictor of malignancy with 83% sensitivity and 89% specificity (OR 1.389, 95% CI 1.035-1.864, p = 0.029). Symptom duration in patients with benign tumors was significantly longer (365 days, range 25.5 to 365 vs 20, range 7 to 42, p ≤0.001). Also, tumor markers were unaltered in benign lesions. In patients with benign tumors significantly more fertility disorders or cryptorchidism were found (p ≤0.001) as well as a tendency toward lower testosterone (3.9 μg/l, range 0.9 to 4.9 vs 5.3, range 3.5 to 6.8, p = 0.084). Testis sparing surgery was performed in 22 of all patients (79%) with benign tumors. There was no case of relapse during followup., Conclusions: Nongerm cell tumors should be considered when small testicular masses have a volume of less than 2.8 cm 3 and there are hormone disorders or normal tumor markers. Immediate orchiectomy should be avoided, favoring testis sparing surgery., (Copyright © 2018 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

An otherwise healthy 7-year-old boy was diagnosed with malignant large cell calcifying Sertoli cell tumor (LCCSCT) of the testis. He underwent attempted partial orchiectomy with conversion to radical orchiectomy due to suspected malignancy on intraoperative frozen section. There was no lymph node or visceral metastases. To our knowledge, this is the first report of malignant LCCSCT in the prepubertal population. LCCSCT of the testis is an extremely rare neoplasm, with low malignant potential. Malignant cases are exclusively reported previously in the adult population. We report the first case of malignant LCCSCT in a pediatric patient. We review the literatures and discuss the clinical, pathologic features and treatments of malignant LCCSCT., (Copyright © 2018 Elsevier Inc. All rights reserved.)

A 4-year-old, unilateral cryptorchid golden retriever dog was presented to the Ontario Veterinary College Health Sciences Centre with gynecomastia, dribbling urine, lethargy, neutropenia, and thrombocytopenia. A Sertoli cell tumor was diagnosed in a cryptorchid testicle with estrogen-induced myelotoxicity. The tumor was removed and bone marrow regenerated within 4 months.

Large-cell calcifying Sertoli cell tumor (LCCSCT) of the testis is an exceptionally rare lesion, found sparsely in any medical literature. There is a correlation between this entity and Peutz-Jeghers syndrome and Carney complex (40% of tumors). The remaining 60% of tumors are sporadic. A 13-year-old male patient underwent a left partial orchiectomy. Intraoperative frozen section was used, and pathology revealed a benign LCCSCT with negative margins. To our knowledge, there is not a case in the literature of an LCCSCT being treated with partial orchiectomy in a pubertal male with a normal contralateral testis., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Carney complex is a rare genetic disease characterised by a complex of myxomas, spotty pigmentation and endocrine overactivity. At diagnosis, about one-third of male patients presents with testicular tumours, namely large cell calcifying Sertoli cell tumours, which are often multicentric and/or bilateral and have a low malignant potential. Although radical orchiectomy is the gold standard for the treatment of testicular neoplasms, a conservative approach with partial orchiectomy or tumourectomy may be the best treatment option for these patients, allowing the preservation of endocrine function, fertility and body image. We present a case of a 19-year-old man with a known history of Carney complex with early identification of a small testicular tumour treated with organ-sparing surgery., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Carney Complex (CNC) is a rare autosomal dominant condition with characteristic clinical presentation, tumor development, and unique genetic mutation. We present a unique case and literature review of CNC in which two neoplasms characteristic of this complex were initially diagnosed through cytological fine needle aspirate specimens, leading to the identification of CNC, with subsequent surgical and cytogenetic confirmation. Diagn. Cytopathol. 2017;45:634-639. © 2017 Wiley Periodicals, Inc., (© 2017 Wiley Periodicals, Inc.)

Sertoli cell nodule consists of clusters of seminiferous tubules composed of immature Sertoli cells and, in some cases, spermatogonia. We report a case of macroscopic Sertoli cell nodule that occurred in the descended left testis of a 26-year-old man. The nodule was centrally located and measured 1.7cm in greatest dimension, the largest such lesion reported to date. Spermatogonia were prominent, a feature that is particularly common in the larger nodules. Macroscopic Sertoli cell nodules (greater than 0.5cm) typically occur in a descended testis. The age range of patients in cases reported since the year 2000 is 19-36 years. Unlike incidental Sertoli cell nodules, macroscopic Sertoli cell nodules containing numerous germ cells can be mistaken for several other Sertoli cell and germ cell lesions and neoplasms., (Copyright © 2016 Elsevier GmbH. All rights reserved.)

We describe the surgical and post-operative management of a large, invasive, and metastatic functional Sertoli cell tumor in a 9-year-old cryptorchid male Labrador retriever dog. Despite residual disease after surgery, bone marrow recovery occurred without administration of bone marrow stimulants and serum estradiol accurately predicted tumor recurrence.

A 64-year-old man had complained of a left scrotal mass and gynecomastia since June 2012. A left testicular tumor was suspected and the patient was referred to our department in December 2013. He presented with bilateral gynecomastia and a painless left scrotal mass that was firm, smooth surfaced, and the size of large hen's egg. Levels of markers of testicular germ cell tumors were all within normal range. Endocrinological examination revealed a marked elevation in serum estradiol (E2) level. The patient underwent high inguinal orchiectomy in December 2013.The pathological diagnosis was a Sertoli cell tumor of the left testis. Immunohistochemistry revealed the expression of aromatase synthesis; we speculated that this E2 production by the tumor caused the gynecomastia.Serum E2 level normalized after the orchiectomy. Owing to the diagnosis of malignancy, retroperitoneal lymph node dissection was performed in January 2014. No lymph node metastasis was found in the specimen. The gynecomastia improved gradually, and the patient has been free of disease since the surgery.

Sertoli cell tumors are a rare malignancy which account for approximately 1.5 % of all testicular tumors. Although malignant Sertoli cell tumors are uncommon, they are associated with a poor prognosis. So far 36 cases of malignant courses of disease have been described. We present a patient with a lymphogenic metastasized Sertoli cell tumor, who 24 months after orchiectomy and extended retroperitoneal lymphadenectomy is relapse-free.

Introduction: The incidence of testicular nodules discovered during infertility evaluation is increasing. These nodules are suspicious of malignancy. There is no paraclinical examination which allows histological orientation to these nodules. The recommendations propose priority treatment by total orchidectomy., Patients and Methods: Through a retrospective cohort study of infertile patients, our goal is to study the enhancement of testicular nodules after injection of ultrasound contrast. The secondary objective is to determine whether CEUS may argue in favor of conservative treatment. From june 2010 to march 2013, 24 patients had underwent ultrasound contrast study of abnormal testicular parenchyma detected prior to infertility evaluation carried ultrasound. The characteristics of ultrasound enhancement were correlated with the pathological findings of surgical patients and proposed treatments (surgery or surveillance)., Results: Fifteen patients were followed up, 9 were operated (7 partial orchidectomies, 2 total orchidectomies). Histological analysis found four Leydig cell tumors, 2 Sertoli cell tumors and 3 seminomas. No adverse changes were noted during the follow-up. This study showed a typical semiology of early, intense and homogeneous enhancement with a phenomenon of wash in 100% of Leydig cell tumors. All Leydig cell tumors have been treated by partial orchidectomy. Seminomas have intense enhancement in 100% of cases. There was a phenomenon of wash in 2 out of 3 cases. When a wash in was described, it was always described as heterogeneous. All seminomas were finally treated by total orchidectomy. The sensitivity and positive predictive value of ultrasound intense enhancement for the diagnosis of testicular cancer was 89% (Se) and 80% (PPV)., Conclusion: There is a semiology of ultrasound enhancement of testicular nodules with features that can guide in favor of a malignant tumor, seminoma or Leydig cell tumor. If a prospective study was undertaken, these arrangements could guide us to treatments promoting preservation of the testicular parenchyma., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Peutz-Jeghers syndrome (PJS) is a rare autosomal dominant disorder that typically displays familial inheritance. Gastrointestinal polyposis and cutaneous pigmentation is a classic presentation of this syndrome. The reported lifetime cumulative cancer risk in PJS patients is >76% when compared with the general public with females affected more often than males. The prepubertal testicular tumor registry found Sertoli cell tumors (SCTs) to compose approximately 1% of all pediatric solid tumors. Prepubertal testicular masses are relatively rare. Only a small number of SCT cases have been reported in the first decade of life. The concurrence of PJS and feminizing SCTs of the testes is an increasingly recognized cause of prepubertal gynecomastia. The testicular lesions observed in patients with PJS primarily represent multifocal intratubular large cell hyalinizing SCTs with a distinct morphology that differs from large cell calcifying SCTs and sex cord tumors with annular tubules. Here, we describe the diagnosis and treatment course of a 4-year-old male with a SCT of the testes and diagnosis of PJS.

Objective: To investigate the safety and feasibility of testis-sparing surgery (TSS) in the treatment of testicular tumor., Methods: We retrospectively analyzed the clinical data of 8 cases of benign testicular tumor treated by TSS in our hospital from October 2005 to March 2012., Results: The 8 patients, aged 18-67 (mean 45) years, were preoperatively diagnosed with benign testicular tumor and all underwent partial testis resection. Rapid intraoperative pathology showed the incisal margins to be negative. Postoperative pathological examination confirmed Sertoli cell tumor in 3 cases, adenomatoid tumor in another 3, and mature teratoma in the other 2. The patients were followed up for 6 months to 7 years (mean 4 years), which revealed no relapse and metastasis, nor significant differences from the baseline in the testosterone level, IIEF score, and routine semen parameters., Conclusion: Testis-sparing surgery is one of the effective options for the management of benign testicular tumor, which can maximally preserve the testis tissue and protect the patient's sexual function.

Purpose: To assess the validity of frozen section examination (FSE) on testis nodules., Materials and Methods: A series of 86 preselected patients with testicular nodules were recruited in this study. Nodules smaller than 2 cm had been surgically removed and biopsies of the margins performed. Larger nodules were just biopsied. Orchiectomy was the treatment of choice for malignant lesions and stromal tumors. Conservative surgery was performed on 2 previously monorchid patients with Leydig cell tumor because of the presence of just one testis. Conservative surgery was the treatment of choice for benign lesions in 32 cases., Results: At FSE we observed that nodules were malignant germinal tumors in 47% of the cases, stromal tumors in 7% of the cases, benign lesions in 45% of the cases and doubtful for lymphoproliferative lesion in 1 case. The diagnosis made by FSE were confirmed in the definitive ones in all of them, we reported just 2 cases of Leydig cell tumor and benign fibrosis lesion. In these 2 cases, definitive histology of the collected specimens revealed areas of Leydig cell hyperplasia and seminomatous foci, respectively., Conclusion: Our data suggest that FSE is a valid tool to discriminate between benign and malignant neoplastic lesions, particularly when an adequate sample is available.

Complete androgen insensitivity syndrome is an extremely infrequent disease. The patients exhibit female phenotype because of insensitivity to the androgen receptor and may develop tumors, especially in their undescended gonads. We report a case of bilateral Sertoli cell adenoma in gonads with unilateral serous cystadenoma, in an elderly phenotypic woman with primary amenorrhea. We also provide radiological and pathological studies.

A 19-year-old male Caucasian, without prior medical history, noticed a painless right testicular mass. Physical examination revealed neither gynecomastia nor abnormal skin pigmentation. Serum alpha-fetoprotein, β-HCG and testosterone levels were normal. Sonography depicted an intratesticular diffusely hyperechoic lesion with acoustic shadowing. The patient underwent right orchiectomy. Histology revealed a benign large cell calcifying Sertoli cell tumour. This tumour is rare and may be associated with genetic abnormalities., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

We present a case of sclerosing Sertoli cell tumor of the testis that was examined preoperatively by magnetic resonance imaging. A 28-year-old male presented to the urology department of our hospital complaining of a painless mass in the right testicle that had been present for the past 2 years. Levels of the tumor markers AFP, hCG, and LDH were all within the standard ranges. T2-weighted MR images showed a well-demarcated lobular hypointensity 15 mm in size in the right testis. Contrast enhancement of this mass after gadolinium administration was homogeneous, with slightly stronger enhancement than the normal testis tissue. Tumor enucleation was performed, and the tumor was diagnosed as a sclerosing Sertoli cell tumor.

Sertoliform cystadenoma of the rete testis represents an uncommon benign tumour. They appear in patients from 26 to 62 years of age. We describe a case of a 66-year-old man with a tumour in the area of the epididymal head. The tumour markers were not increased. Under the assumption of a malignant testicular tumour an inguinal orchiectomy was performed. The cut surface of this tumour was of grey/white color and showed small cysts. The tumour consisted of two compartments. The epithelial like tumour cells showed a sertoliform growth pattern and cystic dilatations. In between the tumour cells repeatedly actin expressing sclerotic areas could be recognized as the second tumour component. Proliferative activity was not increased. Immunohistochemically the tumour cells were positiv for inhibin, S-100, and CD 99. Alpha feto protein (AFP), human chorionic gonadotropin (ß-HCG) and placental alkaline phosphatase (PLAP) as well as synaptophysin, epithelial membrane antigene (EMA), and BCL-2 were not expressed. As far as we know this is the sixth reported case of this tumour. Because of the benign nature of this tumour the correct diagnosis is important for the intra- and postoperative management. Here we present a case of this rare tumour and discuss potential differential diagnosis., Virtual Slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1956026143857335.

Background: Complete androgen insensitivity syndrome (CAIS) is a rare and usually unexpected cause of primary amenorrhoea that results from receptor resistance to androgens, producing a female phenotype in genetically male patients., Case: A 16-year-old girl was diagnosed with CAIS after investigations for primary amenorrhoea. Her left inguinal gonad and the right intra-abdominal gonad were resected and histopathology revealed the presence of dysgenetic multinodular testes with absence of germ cells, significant hyperplasia of Sertoli cells (Sertoli cell adenoma) and presence of paratesticular leiomyomas., Conclusion: Although the risk of gonadal tumour development is considered to be low, a variety of tumours have been described in association with CAIS, but this is the first report of development of bilateral paratesticular leiomyomas developing concurrently with Sertoli cell adenomas., (Copyright © 2012 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

To investigate the role of frozen section assessment in sparing unnecessary orchiectomy for suspected lesions, we retrospectively reviewed intraoperative testicular and paratesticular frozen section assessments performed at our institution between the years 1993 and 2010. Frozen section assessments were performed on 45 testicular lesions (age, 5-60 [mean, 32.2] years; lesion size, 0.5-9.7 [mean, 2.1] cm) and 20 paratesticular lesions (age, 26-76 [mean, 43.5] years; lesion size, 0.4-11.0 [mean, 2.8] cm) before the decision to complete radical orchiectomy. Benign/malignant frozen section assessment diagnoses were reported in 26/19 testicular cases and 17/3 paratesticular cases, respectively. Of the 26 benign testicular frozen section assessments, 5 cases resulted in orchiectomy, where permanent diagnoses included epidermoid cyst, large cell calcifying Sertoli cell tumor, fibrous pseudotumor, abscesses, and sarcoidosis, caused by a concern for potential malignancy or questionable viability of the testicles. Of the 19 malignant testicular frozen section assessments, orchiectomy was performed in 16 cases with germ cell tumor, but not in the remaining 3 cases with lymphoma. Of the 17 benign paratesticular frozen section assessments, 2 cases, both fibrous pseudotumors, resulted in orchiectomy. There were statistically significant differences in the size of the testicular (P < .001) or paratesticular (P < .001) lesions between benign and malignant frozen section assessments. Thus, in 36 (83.7%) of 43 cases with benign frozen section assessments, in addition to all 3 cases of lymphoma, orchiectomy was successfully avoided. These results suggest that frozen section assessment is useful for permitting testicular preservation, especially in men with small, nonpalpable, incidentally found masses as well as other benign lesions where a clinical diagnosis of malignancy is in doubt., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Large cell calcifying sertoli cell tumor (LCCSCT) is an exceptionally rare neoplasm originating from sperm cord cells. The tumors have relatively low malignant potential and unlikely proceed to metastasis formation. The lesions may occur in an isolated form or in ca. 40% of cases may be associated with genetic abnormalities, by and large Peutz-Jeghers syndrome and Carney complex. At presentation, 20% of LCCSCT cases are bilateral and/or multifocal. Owning to characteristic skin lesions and particular hyperechoic ultrasound image of the tumor, preliminary diagnosis of the syndromic LCCSCT is possible in the preoperative period. Consequently, testicle organ-sparing procedure can be attempted, which is especially justified in bilateral lesions. Here, we report a case of a bilateral LCCSCT in a 20-year-old man with atypical Peutz-Jeghers syndrome due to amplification of the exon 1 of STK11 gene who was successfully treated with bilateral testicle-sparing tumorectomies.

Objective: Frasier syndrome (FS) phenotype in 46,XY patients usually consists of female external genitalia, gonadal dysgenesis, high risk of gonadoblastoma and the development of end stage renal failure usually in the second decade of life. FS is caused by heterozygous de novo intronic splice site mutations of the Wilms' tumor suppressor gene 1 (WT1), although a few cases with typical exonic WT1 Denys-Drash mutations that resemble an FS phenotype have been described. The aim of this study was to present further data on the spectrum of FS phenotypes through the evaluation of a 29-year-old patient with a predominantly male phenotype and coexistence of Sertoli cell tumor and gonadoblastoma., Results: Genetic analysis using standard methods for DNA sequencing confirmed FS due to a WT1 gene mutation, IVS9+4C>T., Conclusions: This very rare case illustrates the natural course of FS over many years due to the neglect by the patient to address his need for follow-up, while adding further data on the spectrum of FS phenotypes associated with IVS9+4 C>T mutations. The coexistence of the rare Sertoli cell tumor and gonadoblastoma emphasizes that early clinical recognition and molecular identification facilitates appropriate patient management, especially with respect to the high risk of gonadal malignancy.

The association of cryptorchidism, functional Sertoli cell tumors, and spermatic cord torsion has been rarely reported in the literature. Two dogs were admitted for bilateral skin alopecia and weight loss. Both animals were cryptorchid and displayed a pendulous preputial sheath, prostate hypertrophy, and increased levels of circulating oestrogen. Transabdominal palpation and ultrasonography revealed the presence of neoplastic retained gonads. During surgery, spermatic cord torsion was also detected in the enlarged neoplastic testes of both dogs. Histologic examination confirmed the presence of Sertoli cell tumors that were primarily responsible for the feminizing syndrome. Complete remission of all symptoms occurred within 3 months after orchiectomy.

Complete androgen insensitivity syndrome (AIS) is an X-linked disorder of sex development. Surgical management entails timely gonadectomy given the risk of malignant transformation. Our patient presented at age 15 years with primary amenorrhea. Initial laboratory testing showed elevated testosterone, luteinizing hormone, anti-Müllerian hormone levels, and 46,XY karyotype. Imaging studies showed no uterus, ovaries, and identified two candidate gonads. She underwent bilateral gonadectomy. Pathology reports revealed Sertoli cell and intratubular germ cell tumors located in separate gonads. Our case is the first report of the youngest patient with AIS with bilateral gonadal tumors derived from different histological origins. We also review literature for reports of AIS patients with gonadal tumors. Currently, there is no consensus for the timing of gonadectomy in AIS patients. However, given the varying potential for malignant transformation of gonads in AIS patients with different phenotypes, development of a standardized treatment guideline is indicated.

Introduction: Testicular parenchyma abnormalities and testis cancers are more frequent in infertile men, hence the guidelines recommending a systematic scrotal ultrasound., Methods: A retrospective review of all patients treated with total or partial orchidectomy, from January, 2000 to July, 2010, for a testicular lesion discovered during an infertility evaluation work-up. Physical, examination data, type of surgery and pathological results were reported., Results: Forty-five patients were treated. The majority of tumors (80%) were non palpable, and incidentally discovered with scrotal ultrasonography. Eight cases were partial orchidectomies, and 37 cases were radical orchidectomies. A frozen section examination was performed in 13 cases, and led to two radical orchidectomies. Standard histological examination revealed 33 (73.3%) benign lesions (11 Leydig cell hyperplasias, 17 Leydig cell tumors, five Sertoli cell tumors) and 10 (22.2%) malignant lesions (nine seminomas and one teratoma). Ten patients had a Klinefelter syndrome, for whom all the lesions were benign., Conclusion: The majority of non-palpable testicular lesions, discovered by ultrasonography in a population of infertile men were benign tumors. Conservative management in this context appears to be an option, to preserve the endocrine function and the fertility of these patients, while being ontologically safe., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Two distinct nodules developed in a cryptorchid testis of an 8-year-old male West Highland White Terrier. One nodule was a Sertoli cell tumor. The other was a spermatocytic seminoma with focal primitive neuroectodermal differentiation: formation of Homer-Wright rosettes and perivascular pseudorosettes, with immunoreactivity for S-100 protein, neuron-specific enolase, synaptophysin, neurofilament-68 kDa, microtubule-associated protein 2, and vimentin. The dog was alive and healthy 2 years after castration.

The 1st pediatric case of sertoliform cystadenoma with unique features is described herein. The patient is a 6-year-old boy who presented with gynecomastia and a left testicular cystic mass. Histopathologically the tumor was found to originate from the rete channels, filling and distending them with areas of mural Sertoli cell proliferations reminiscent of large cell Sertoli cell tumor (noncalcifying form) and showing widespread intratubular Sertoli cell proliferation islands in the vicinity. Histopathologic and immunohistochemical features are described in light of the relevant literature.

Sertoli cell nodules are almost always incidental microscopic lesions found in both cryptorchid and normally descended testes. Sertoli cell nodules, when present as masses or ultrasonographic lesions, may create diagnostic confusion. Herein, we report 6 cases of macroscopic Sertoli cell nodules that were received in consultation. The referral diagnoses included Sertoli cell tumor (2 cases), sex cord tumor with annular tubules (1 case), and gonadoblastoma (1 case). The patients were 19 to 36 years old: 3 patients presented with palpable testicular masses and 3 with lesions that were worrisome for neoplasms in ultrasonographic examinations conducted for pain (2 cases) or infertility (1 case). All were phenotypically normal male patients who lacked endocrine symptoms. The Sertoli cell nodules ranged from 6 to 10 mm in diameter and on microscopic examination consisted of circumscribed proliferations of immature Sertoli cells, globules and trabeculae of basement membrane, and spermatogonia in varying proportions. In 2 cases the lesion was distinctly intratubular, consisting of closely packed tubules containing various components; in the other cases there was confluent growth of the tubules. Immunostains for α-inhibin highlighted the Sertoli cells (5 of 5 cases), with the germ cells appearing in negative relief. An antibody for testis-specific protein, Y-encoded (TSPY), stained the spermatogonia (2 of 2 cases), whereas OCT 3/4 was negative in all the cases (5 of 5 cases). We conclude that Sertoli cell nodules may present clinically as mass lesions, and that it is important to distinguish them from true neoplasms to avoid unnecessary procedures.

Objective: We report two new cases of Sertoli cell testicular tumors, and a Cochrane and Medline search of cases published worldwide., Methods: We reviewed our series of testicular tumors, the stromal tumor incidence, clinical presentation, treatment and prognosis, and the experience reflected in the literature., Results: The prevalence of testicular tumors in our health area is of 0.09%, and 2.3% of them are Sertoli cell neoplasms. This figure is slightly higher than the found in other series in which Sertoli tumors range from 0.4% to 1.5% of testicular malignancies in adults and reach 4% in children., Conclusions: Sertoli cell tumor has an incidence not exceeding 4%. The most common symptom is a painless mass; in cases with endocrine manifestation, up to 10% are malignant depending on the age of the patient.