The Morphologic Spectrum of Sertoliform Cystadenoma of the Rete Testis: A Series of 15 Cases.

Sertoliform cystadenoma of the rete testis (SCRT) is rare with only 9 cases reported to date in the literature, none with follow-up. Four large genitourinary pathology consult services were searched. We identified 15 cases of SCRT. Men were 21 to 84 years old (mean, 46 y) and had testicular discomfort or mass. Other findings were seminoma (n=1), spermatocele (n=2), hydrocele (n=1), varicocele (n=1), and scrotal hematoma (n=1). Eight had preoperative serum tumor markers, which were normal. Tumors ranged from 0.3 to 4 cm (mean, 1.5 cm). All of them were well circumscribed with solid and cystic features and occupied on average, 73% of the rete (20% to 100%). The tumors were mostly confined within dilated channels of the rete testis and showed classic features consisting of: (1) tubules with well-formed lumina in 87% of cases; (2) well-formed tubules with no lumina in 87% of cases; and (3) cords/nests in hyalinized or myxoid stroma in 73% of cases. Other patterns included: (1) solid/sheet growth in 26% of cases; (2) individual cells in 13% of cases; (3) festoons in 13% of cases; (4) branching tubules in 7% of cases; and (5) papillary in 7% of cases. Cells were cuboidal with round to oval nuclei with small nucleoli, except at the periphery where projections into rete tubules had a more columnar appearance. In the festooning pattern, nuclei were pseudostratified and columnar with prominent nucleoli and nuclear grooves. In 4 cases, tumor extended into adjacent seminiferous tubules surrounded by dense peritubular fibrosis, with in some cases small cysts lined by flattened epithelium containing pale lightly granular material. All cases lacked necrosis and significant atypia. Mitoses ranged from 0 to 2 per 10 high-power field. Follow-up ranged from 4 to 170 months with mean of 97 months. For the 13 cases with information, all patients were alive, except for 3 who died of either unrelated causes (9.2 and 10 y) or of unknown cause (4.8 y at age 89 y). We performed immunohistochemistry for steroidogenic factor 1 and inhibin in 4 of our cases, where 3 (75%) were positive for both markers. We also describe 2 additional cases which morphologically resembled SCRT but had more atypical features. This study highlights that SCRT has variable morphology. We also verify the benign nature of the lesion and its lack of association with any syndromes.

Intratubular large cell hyalinizing Sertoli cell tumor of the testis presenting with prepubertal gynecomastia: a case report.

Intratubular large cell hyalinizing Sertoli cell neoplasia (ITLCHSCN) resulting from Sertoli cells of the testis are mainly reported in young adults and these are rarely seen in childhood. The most common presenting symptoms of the patients diagnosed with ITLCHSCN are gynecomastia, enlargement in the testicles, increase in growth velocity, and advanced bone age. Symptoms are basically resulting from increased aromatase enzyme activity in Sertoli cells. In this case report, an eight-and-a-half-year-old case presenting with complaint of bilateral gynecomastia since two years, showing no endocrine abnormality in laboratory during two years of follow-up, determined to have progression in bilateral gynecomastia, increase in testicular volumes, advanced bone age, increase in growth velocity in the clinical follow-up, and diagnosed with ITLCHSCN after testis biopsy was presented.

Management of an invasive and metastatic Sertoli cell tumor with associated myelotoxicosis in a dog.

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.

Gestion d'une tumeur à cellules de Sertoli invasive et métastatique avec une myélotoxicose secondaire chez un chien. Nous décrivons la gestion chirurgicale et postopératoire d'une tumeur à cellules de Sertoli fonctionnelles, de grande taille, invasive et métastatique chez un chien Labrador retriever cryptorchide âgé de 9 ans. Malgré une maladie résiduelle après la chirurgie, le rétablissement de la moelle osseuse s'est produit sans l'administration de stimulants de la moelle osseuse et l'œstradiol sérique a fidèlement prédit la récurrence de la tumeur.(Traduit par Isabelle Vallières).

Testicular sex cord stromal tumors: analysis of patients from the MAKEI study.

BACKGROUND: In children and adolescents, testicular sex cord stromal tumors (TSCSTs) are rare. There is only limited information available regarding their clinical presentation, biology, and prognosis. METHODS: Between 1993 and 2009, 42 patients were prospectively reported to the cooperative MAHO and MAKEI studies on childhood germ cell tumors. Based on standardized documentation, data on epidemiology, clinical presentation, diagnostic features, histopathological differentiation, therapy, and follow-up were evaluated. RESULTS: During the study period, a gradual increase of the documentation of these rare tumors was observed. Palpable, indolent testicular swelling was the most common clinical finding. In three patients, retention of the testis was observed. Two patients showed sexual precocity, and one patient showed a 45X/46XY mosaic. Juvenile granulosa cell tumors (n = 16) and Sertoli cell tumor (n = 15) were the leading histopathological subtypes. The first were commonly diagnosed during the first weeks of life (median age: 6(0-162) days, the latter during infancy (median 7(0-14) months, P < 0.05). Other histological diagnoses included Leydig cell and Large Cell Calcifying Sertoli cell tumors (both n = 3) and not-otherwise-specified TSCSTs (n = 5), which were diagnosed during childhood and adolescence. All tumors were limited to the testis; there were no metastases. Treatment was surgical, only. After a median follow-up of 3.8 years, no relapse was observed. CONCLUSIONS: Diagnosis and therapy of testicular tumors should be planned in accordance with the recommendations of the respective childhood germ cell tumor protocols. High inguinal orchiectomy is safe and constitutes definitive therapy. Diagnostic work-up and follow-up should also consider potentially associated tumor predisposition syndromes.

[Case report of malignant sertoli cell tumor].

Malignant sertoli cell tumor is a rare disease and only a few cases have been described previously. We report a terminal case of malignant sertoli cell tumor. A 38-year-old male visited a hospital with a complaint of swelling his left testis. He underwent high left orchiectomy. His pathologic diagnosis was suspected seminoma, and all tumor markers (LDH, HCG, AFP) were negative, and CT imaging confirmed clinical stage 1 (pT1N0M0S0). One year later, a CT scan showed a small retroperitoneum lymph node swelling. Four months later, these lesions increased to 55 x 45 x 70 mm in diameter. He received 3 courses of chemotherapy with BEP (bleomycine, etoposide, cisplatin), but, lymph node size did not change. After he underwent a CT guided lymph node biopsy, his pathologic diagnosis was viable embryonal carcinoma. He then came to our hospital. We selected CPT-11 and nedaplatin for his salvage chemotherapy, but lymph node lesions did not change. After he received 3 courses of chemotherapy, we performed retroperitoneal lymphadenectomy. His pathologic diagnosis was viable sertoli cell tumor, malignant type. After 30 days, he had multiple liver metastases ane died 27 months after orchiectomy. All tumor markers were negative in his all clinical courses.

Neoplasias testiculares en caninos: un caso de tumor de células de sertoli / Testicular neoplasms in dogs: a case of sertoli cell tumor

Las neoplasias del testículo no son muy frecuentes en los animales domésticos Los caninos son los que las manifiestan con mayor frecuencia; estando entre los 0.7 y 4.6% de todos los tumores que sufren. Algunos factores se han asociado como predisponentes: entre ellos la edad madura y la presencia del criptorquidia uni o principalmente bilateral. Las neoplasias del testículo más frecuente son el tumor de células de Sertoli, el tumor de células de Leydig y los seminomas, que en general presentan la conducta benigna. El tratamiento recomendado previa evaluación clínica, es la orquiectomía (uni o bilateral), incluidos en los casos de criptorquidismo el testículo alojado en el escroto. En el presente manuscrito se realiza una revisión del tema en su primera parte; en la segunda, se describe el caso de un tumor de células del Sertoli en un canino Cocker que presentó al examen clínico un testículo retenido y síndrome feminizante. En este se caso se efectuó el diagnóstico histopatológico después de la extracción quirúrgica de la masa abdominal y también se realizó la extirpación del testículo alojado en el escroto.

Conservative management of bilateral Sertoli cell tumors of the testicle in association with the Carney complex: a case report.

Large cell calcifying Sertoli cell tumor of the testicle is a rare, hormonally active sex cord-stromal tumor seen in patients with Carney complex. When such tumors occur bilaterally, treatment options for preserving fertility and addressing the secondary effects of excess hormone production must be considered. The availability of specific antiestrogen drugs means that bilateral orchiectomy for this benign tumor may no longer be warranted. Testicular-sparing surgery and advances in reproductive technology may also improve the overall prognosis for fertility. Gynecomastia in prepubescent boys can be emotionally very distressing. Approximately two thirds of teenaged boys will develop some degree of breast enlargement that spontaneously regresses as testosterone levels rise (Ill Med J 1938;73:113). In all cases, a thorough history and physical examination are required to exclude nonphysiologic causes such as drugs, pulmonary disease, chronic liver disease, exogenous estrogens, and estrogen-producing tumors (Seashore J. Disorders of the breast. In: Rowe MI, O'Neill JA, Grosfeld JL et al, editors. Pediatric surgery, 5th ed. St Louis (MO): Mosby Year Book, 1998). We report on a child who presented with a 2-year history of gynecomastia with associated bilateral testicular swellings and discuss a novel treatment strategy for managing bilateral testicular tumors in the context of the Carney complex.

Sertoliform endometrioid carcinoma of the ovary: a potential diagnostic pitfall.

Sertoliform endometrioid carcinoma of the ovary (SEC) is an uncommon variant that bears histologic similarity to Sertoli and Sertoli-Leydig cell tumors (SLTs). Clinically, SEC affects an older population (60-70 years), while patients with SLT have an average age of 25 years and may exhibit endocrine manifestations. A number of histologic features can be used to distinguish the 2 entities, the most important ones being (1) the presence of areas with the usual pattern of endometrioid carcinoma, and (2) the presence of mucin at the apical borders of the tumor cells. Cytokeratin stains positively, while inhibin and calretinin stain negatively in SEC; the converse is true for SLTs. Based on the clinicopathologic behavior of this entity, SEC should be considered a well-differentiated carcinoma with relatively good prognosis if limited to the ovary.

Testicular sertoli cell tumours and relative sub-types. Analysis of clinical and prognostic features.

INTRODUCTION: Sertoli cell tumours have a rare (0.4-1.5% of all testicular neoplasms) and heterogeneous pathology. The aim of this paper is to analyse the histological classification of Sertoli cell tumours, in order to assess if the three different histotypes--classic type, large cell calcifying Sertoli cell tumour (LCCSCT) and sclerosing Sertoli cell tumour (SSCT)--really present distinctive clinical and prognostic features. MATERIALS AND METHODS: The current literature was reviewed; Sertoli cell tumour clinical series and single case reports were searched and analysed. Hence, more than 200 classic Sertoli cell tumours, 48 LCCSCTs and only 12 SSCTs were found. The thirteenth SSCT has been found by us in a 34-year-old man. RESULTS: Every single sub-type presents clinical specific characteristics regarding age of onset, bilaterality, focality, abnormal hormone production, correlated systemic symptoms. Ultrasonographic findings, size and--above all--malignant potential. CONCLUSIONS: The precise classification of these tumours is not important only histologically: the currently recognised variants really differ in clinical presentation and course. Moreover, LCCSCTs can be further divided in two subgroups with very different clinical behaviour, those in older patients and those associated with well-known syndromes. These clinical and prognostic variables are of great importance when deciding on the therapeutical approach.

Clinical behavior and a contemporary management algorithm for prepubertal testis tumors: a summary of the Prepubertal Testis Tumor Registry.

PURPOSE: The Prepubertal Testis Tumor Registry was established by the Urologic Section of the American Academy of Pediatrics in 1980 to record data on a large number of prepubertal testis tumors regarding presentation, treatment and outcome to define appropriate management better. We reviewed the registry data in the context of other modern studies to elucidate the appropriate management of these rare tumors. MATERIALS AND METHODS: Relevant data in the prepubertal testis tumor registry were tabulated and analyzed. RESULTS: There were 395 prepubertal patients who had a primary testis tumor. Generally benign tumors accounted for 38% of cases. A significant proportion of tumors were benign regardless of patient age. alpha-Fetoprotein levels for patients with benign and malignant tumors overlapped in children younger than 6 months. Of the patients with yolk sac tumor 80% presented with stage 1 disease and overall survival was excellent. There were no metastases or deaths among the patients with teratoma. Of all patients with stromal tumors metastases developed in only 1. CONCLUSIONS: We recommend initial excisional biopsy for all amenable prepubertal testis tumors, except those with an alpha-fetoprotein level that is clearly increased for patient age. Patients with benign tumors may be released from oncological followup. Patients with stage I yolk sac tumor should be monitored closely, and those with recurrent or metastatic yolk sac tumor should be treated with chemotherapy. Retroperitoneal lymph node dissection is reserved for patients with recurrent retroperitoneal masses following chemotherapy. Aggressive treatment of metastatic Sertoli cell or undifferentiated stromal tumors is warranted.

Sertoli cell tumor in androgen insensitivity syndrome--a case report.

A 26-year-old individual with androgen insensitivity syndrome was operated on for a 3200-g Sertoli cell tumor of the left gonad with retroperitoneal metastases. Six courses of bleomycin, etoposide, and cisplatin chemotherapy followed surgical treatment. Eighteen months after the initial surgery the patient is free of disease and in good health. The association of Sertoli cell tumor with androgen insensitivity syndrome is discussed and the relevant literature is briefly reviewed.

Treatment of aggressive testicular tumors in four dogs.

In this report, the authors describe four dogs referred for diagnosis and treatment of unusual and aggressive testicular tumors. For the vast majority of dogs with testicular tumors, orchiectomy is curative. All dogs in this report had surgical resection, and three of four dogs were treated with cisplatin chemotherapy. Cisplatin is widely recognized as the most active agent in testicular cancer in human medicine.

Androblastoma en preescolar de 4 años: a propósito de un caso / Androblastoma in preschool of the four years: purpose of a case

Los Androblastomas son neoplasias muy raras típicas de la edad joven, con una incidencia entre los 20 y los 30 años. Los tumores de células de Sertoli, pertenecen al grupo de los androblastomas. Los tumores de ovario en la infancia son raros, y corresponden a menos del 1 por ciento de todos los tumores malignos. El tumor de Sertoli tiene una incidencia de 2.5 por millón de niñas al año. Nosotros presentamos el caso de la primera paciente del Hospital General del Oeste

Metastatic Sertoli cell carcinoma of the testis.

Metastatic Sertoli cell tumor is a very rare and deadly disease accounting for approximately 1% of all testicular carcinomas. With fewer than 30 cases reported in the literature, there has not been a uniform treatment regimen with good results. Retroperitoneal lymph node dissection, chemotherapy, and radiation therapy in combination appear to offer the best outcome. This report describes the occurrence of this rare tumor in a 38-year-old man 2 years after left orchiectomy.

Gonadal stromal tumors: a report of the Prepubertal Testicular Tumor Registry.

A total of 15 gonadal stromal tumors in prepubertal boys has been reported to the Prepubertal Testicular Tumor Registry of the Section on Urology of the American Academy of Pediatrics. Leydig cell tumors invariably are benign and patients usually present with precocious puberty when they are 5 to 9 years old. Other gonadal stromal tumors either present in infancy as a scrotal mass and exhibit a benign behavior or occur later in childhood and may be malignant.

Ovarian Sertoli-Leydig cell tumour with raised serum alpha fetoprotein. A case report.

A case of ovarian Sertoli-Leydig cell tumour with a raised serum alpha fetoprotein is reported. The patient first presented at the age of 27 years with a history of 6 years' amenorrhoea followed by 3 months irregular vaginal bleeding. A ovarian tumour was found and excised and shown microscopically to be a spindle cell malignant tumour. The patient was treated with chemotherapy and had a complete response. Thirty months after first presentation there was a recurrence in the pelvis which microscopically showed the typical features of a Sertoli-Leydig cell tumour. Six months later a second recurrence had the microscopic appearance of a lipid cell tumour. A raised serum alpha fetoprotein was found at the time of the second recurrence and immunohistochemistry showed this protein in the Leydig and luteinized cells of the recurrent tumours but not in the spindle cells of the original ovarian neoplasm.