Tumor de Células de Sertoli del ovario: Reporte de un caso / Sertoli cell tumor of the ovary. A case report

RESUMEN Introducción: Los tumores de Células de Sertoli son una entidad rara, con una frecuencia de 0,2-0,5 % del cáncer de ovario. El diagnóstico inicial de este tipo de tumores es difícil, debido a las variedades histológicas que presenta y a su diferenciación. Objetivo: Demostrar el valor del completamiento del proceso diagnóstico con técnicas de inmunohistoquímica en tumores ováricos para identificar una variante rara como el tumor de Células de Sertoli. Presentación del caso: Se presenta un caso de un tumor de ovario primitivo de Células de Sertoli en una paciente de 43 años de edad, con antecedentes personales de hipotiroidismo, intervenida quirúrgicamente por un tumor sólido del ovario derecho en el Hospital General Docente "Dr. Agostino Neto". En el intraoperatorio, se encontró un tumor gigante de aspecto maligno. El resultado anatomopatológico informó un tumor carcinoide típico. La paciente fue remitida al Instituto Nacional de Oncología y Radiobiología, donde la inmunohistoquímica de la biopsia informó un tumor de Células de Sertoli. Actualmente la paciente se encuentra asintomática. Conclusiones: El diagnóstico anatomopatológico preciso y la inmunohistoquímica correcta son muy importantes para el manejo y tratamiento de este tipo de tumor.

ABSTRACT Introduction: Sertoli cell tumors are a rare entity with an incidence of ovarian cancer of 0,2-0,5 %. The initial diagnosis of this type of tumors is difficult to make due to the histological varieties and its differentiation. Objective: To demonstrate the value of immunochemical techniques in the completion of the diagnostic process of ovarian tumors aimed at identifying a rare variant such as Sertoli cell tumor. Case presentation: A 43-year old woman with a primitive Sertoli cell tumor of the ovary is presented. The patient had personal history of hypothyroidism. She underwent surgery at "Dr. Agostinho Neto" General Teaching Hospital because she had been diagnosed with a solid tumor of the right ovary. During the intraoperative period, a giant tumor with a malignant appearance was found. The pathological result reported a typical carcinoid tumor. The patient was referred to the National Institute of Oncology and Radiobiology where the immunohistochemistry of the biopsy reported a Sertoli cell tumor. Currently, the patient is asymptomatic. Conclusions: The accurate pathological diagnosis and the correct immunohistochemistry are very important for the management and treatment of this type of tumor.

Malignant Sertoli cell tumor of the testis masquerading as seminoma with bone metastasis

Sex cord–stromal tumors of the testes are rare malignancies as compared to germ cell tumors. Pure Sertoli cell tumors are still rare representing <1% of testicular cancers and the malignant forms are too rare. Furthermore, the occurrence of metastasis in such cases is extremely rare with <30 cases reported in literature so far to the best of our knowledge. We present herein a case of malignant Sertoli cell tumor in a 48-year-old male who was initially misdiagnosed as seminoma based on histology and clinical presentation. Four months later, he presented with symptoms due to bony metastasis and found to have widespread metastatic disease which is a very rare presentation of Sertoli cell tumors. Diagnosis of sex cord–stromal tumor requires high index of suspicion as these tumors are most of the times misdiagnosed as germ cell tumors due to their rare incidence and atypical presentation leading to mismanagement. Timely diagnosis at an early stage can provide therapeutic benefit due to lack of well-defined treatment options at advanced stages.

Tumor de células de sertoli esclerosante. Reporte de un caso / Sclerosing sertoli cell tumor. Case report

RESUMEN El tumor esclerosante de células de Sertoli del testículo es una entidad patológica infrecuente, con todo, que solo se han descrito 22 casos en la literatura científica. Son descritos como tumores unilaterales que en su mayoría se presentan entre la tercera y sexta década de vida, siendo carentes de malignidad, sin embargo, en ninguno de los casos reportados se han realizado seguimientos por periodos prolongados. Se presenta un caso clínico cuyo objetivo es socializar el primer caso de tumor esclerosante de células de Sertoli (SSCT), diagnosticado por el departamento de patología de la Universidad industrial de Santander, que se registra en Colombia. Después de tres años de seguimiento clínico el paciente no presenta recidiva, así como tampoco otras lesiones tumorales. Se considera la orquiectomia el tratamiento curativo.(AU)

SUMMARY Sclerosing tumor of Sertoli cells of the testis is an infrequent pathological entity, only 22 cases have been described in the scientific literature. They are described as unilateral tumors that mostly occur between the third and sixth decade of life, being devoid of malignancy, however, in none of the cases have been followed for prolonged periods. We present a clinical case whose objective is to socialize the first case of Sertoli cell sclerosing tumor (SSCT), diagnosed by the pathology department of the industrial university of Santander, which is registered in Colombia. After three years of clinical follow-up, the patient does not present recurrence, as well as other tumor lesions. Orchiectomy is considered the curative treatment.(AU)

Sertoli cell tumor arising in a cryptorchid testis presenting as a content of inguinal hernial sac.

Sertoli cell tumors (SCTs) are rare tumors accounting for <1% of all testicular tumors. Here, we report a rare case of SCT in a 60‑year‑old man presenting as a painless swelling in the right groin since childhood. Clinically, he presented with right‑sided inguinal hernia with absence of the right testis. He had normal left testis and had no gynecomastia or infertility. The specimen of hernial sac showed testis with a 1.6 cm × 1.5 cm nodular mass having gray tan‑cut surface. Histopathologically, the testis showed atrophy and the nodular portion showed tumor cells arranged in tubular and microcystic pattern, with no solid pattern or necrosis. The diagnosis of SCT was confirmed with immunohistochemical staining for inhibin which showed fine granular cytoplasmic positivity. Cryptorchid testis having SCT and presenting as a content of inguinal hernia is a rare occurrence.

Complexo de Carney esporádico com tumor testicular e mixoma atrial: relato de caso / Sporadic Carney complex with testicular tumor and atrial myxoma: case report

O complexo de Carney é uma rara forma de neoplasia endócrina múltipla familial autossômica dominante. Está associado à alteração de pigmentação cutânea e mucosa, doença nodular adrenal pigmentosa primária, mixomas cardíacos e cutâneos, adenomas hipofisários funcionantes, neoplasia testicular, adenoma ou carcinoma de tireoide, além de cistos ovarianos. Aproximadamente 70% dos indivíduos diagnosticados com complexo de Carney têm pais afetados, e 30% apresentam forma esporádica. O objetivo deste estudo foi relatar um caso de complexo de Carney esporádico por mixoma cardíaco e tumor testicular. Ressalta-se a importância do caso por sua raridade e sua forma curiosa de apresentação. Homem, 33 anos, manifestou dois quadros de acidentes vasculares cerebrais em 4 meses. Na investigação apresentou pressão arterial elevada com sopro sistólico discreto e fraqueza muscular (força grau 4 em membro superior direito e grau 3 em membro inferior direito). História mórbida de tumor testicular de células de Sertoli há 7 anos com orquiectomia bilateral. História familiar sem particularidades. Na investigação, evidenciaram-se sobrecarga atrial esquerda ao eletrocardiograma e massa tumoral pedunculada compatível com mixoma atrial esquerdo ao ecocardiograma transesofágico. Foi configurada síndrome de Carney pela presença de dois critérios maiores, e o paciente foi submetido à atriotomia esquerda, com ressecção da massa tumoral e confirmação anatomopatológica. A curiosa apresentação do caso recorda que, diante de um caso de acidente vascular cerebral em paciente jovem, a suspeita clínica seja direcionada a causas mais raras. O complexo de Carney esporádico é raro, dificultando ainda mais a elucidação.

Carney complex is a rare form of autosomal dominant multiple endocrine neoplasia familial. Changing skin pigmentation and mucos, primary pigmented nodular adrenal disease, cardiac and cutaneous myxomas, functioning pituitary adenomas, testicular cancer, thyroid adenoma or carcinoma is associated, and ovarian cysts. Approximately 70% of individuals diagnosed with Carney complex have affected parents and 30% have sporadically. The aim of this study was to report a case of sporadic Carney complex due to cardiac myxoma and testicular tumor. We emphasized the importance of the case for its rarity and curious form of presentation. Man, 33, showed two episodes of strokes in 4 months. In research presented high blood pressure with mild systolic murmur and muscle weakness (grade 4 strengthin the right arm and grade 3 in the right lower limb). Morbid history of testicular Sertoli cell tumor 7 years ago with bilateral orchiectomy. No special family history. On investigation, left atrial enlargement and was evident on the electrocardiogram, and transesophageal echocardiogram revealed the presence of pedunculated tumor mass setting a left atrial myxoma. Carney's syndrome was characterized by the existence of two major criteria and patient underwent left atriotomy with resection of the tumor mass and anatomic-pathologic confirmation. The curious case presentation reminded us that before a case of stroke in a young patient should direct the clinical suspicion for rarer causes. The Carney complex sporadic is rare, yet difficult to elucidate.

Sertolioma unilateral como causa de morte súbita por hemorragia interna em ganso Anser cygnoides / Unilateral Sertolioma as a cause of sudden death by internal bleeding in a Anser cygnoides goose

Um ganso adulto macho (Anser cygnoides), da família Anseriformes, de idade desconhecida, proveniente de uma criação da Universidade Luterana do Brasil, foi encontrado morto, sem apresentar histórico clínico, e foi submetido à investigação post mortem no Setor de Patologia Veterinária do Hospital Veterinário. Com base nos achados de necropsia e no exame histopatológico, definiu-se como causa da morte do animal hemorragia interna em razão da ruptura de vasos sanguíneos em uma neoplasia no testículo direito (sertolioma), com metástase no fígado...

An adult male goose (Anser cygnoides) of unknown age, raised at the Lutheran University of Brazil, was found dead without showing clinical history and was submitted for post mortem investigation in the Department of Pathology of the Veterinary Hospital. From the necropsy and histopathological findings, the cause of death was defined as exsanguination due to intestinal hemorrhage from ruptured vessels in a tumor in the right testis, which also presented hepatic metastasis...

Large Cell Calcifying Sertoli Cell Tumor of the Testis: A Case Study and Review of the Literature

A 24-year-old man was admitted due to an incidentally detected mass in his left testis, which showed radiopaque calcification on plain X-ray film. Left orchiectomy was performed, and the resected testis contained a well-demarcated, hard mass measuring 1.1 cm. Histological analysis revealed that the tumor was composed of neoplastic cells, fibrotic stroma, and laminated or irregularly shaped calcific bodies. The individual cells had abundant eosinophilic or clear cytoplasm with round nuclei, each of which contained one or two conspicuous nucleoli. They were arranged in cords, trabeculae, clusters, and diffuse sheets. There were several foci of intra-tubular growth patterns, with thickening of the basal lamina. Immunohistochemically, the neoplastic cells were positive for S-100 protein and vimentin, focally positive for inhibin alpha, and negative for cytokeratin, CD10, and Melan-A. In addition to reporting this rare case, we also review the relevant literature regarding large cell calcifying Sertoli cell tumors.

Umbilical KeyPort bilateral laparoscopic orchiectomy in patient with complete androgen insensitivity syndrome

MAIN FINDINGS: A 22-year-old woman with complete androgen insensitivity syndrome (CAIS) presenting with primary amenorrhea and normal female external genitalia was referred for laparoscopic gonadectomy. She had been diagnosed several years earlier but was reluctant to undergo surgery. CASE HYPOTHESIS: Diagnosis of this X-linked recessive inherited syndrome characterizes by disturbance of virilization in males with an AR mutation, XY karyotipe, female genitalia and severely undescended testis with risk of malignization. The optimal time to orchidectomy is not settled; neither the real risk of malignancy in these patients. Early surgery impacts development of a complete female phenotype, with enlargement of the breasts. Based on modern diagnostic imaging using DCE-MRI and surgical technology with single port laparoscopic access we hypothesize that the optimum time for gonadectomy is not at the time of diagnosis, but once feminization has completed. PROMISING FUTURE IMPLICATIONS: An umbilical laparoendoscopic single-site access for bilateral gonadectomy appears to be the first choice approach as leaves no visible incision and diminishes the psychological impact of surgery in a patient with CAIS absolutely reassured as female. KeyPort, a single port access with duo-rotate instruments developed by Richard Wolf facilitates this surgery and allows excellent cosmetic results.

Sertoli cell tumors associated with feminizing syndrome and spermatic cord torsion in two cryptorchid dogs

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.

Tumor de Sertoli variante célula grande calcificante asociado a síndrome de peutz-jeghers / Large cell calcifying sertoli cell tumor associated with peutz-jeghers syndrome

El tumor de células de Sertoli calcificante de células grandes (TCSCCG) es una neoplasia testicular muy rara, con 60 casos descritos en todo el mundo; en Latinoamérica y Colombia son escasos los casos reportados en la literatura. Esta neoplasia se origina en el estroma gonadal/cordones sexuales, y, en general, tiende a mostrar un comportamiento biológico benigno. Se piensa que estos tumores tienen características de presentación diferente, dependiendo de si se presentan en el contexto de un síndrome congénito complejo, en cuyo caso tienden a ser bilaterales y multifocales, en contraposición a los no asociados a síndromes, que tienden a ser unilaterales y focales. El estudio de inmunohistoquímica es fundamental para el diagnóstico; en especial, para diferenciar estos tumores de neoplasias germinales. Se reportan en esta investigación tres casos de esta neoplasia, uno de ellos asociado al síndrome de Peutz-Jeghers, y haciendo énfasis en los criterios histológicos para definir malignidad.

The large cell calcifying Sertoli cell tumor (LCCSCT) is a very rare testicular tumor with 60 cases reported worldwide; in Latin America and Colombia, few cases are reported in the literature. This neoplasm originates in the gonadal stromal/sex cord, and, in general, tends to display benign biological behavior. It is thought that these tumors may display differing characteristics: depending upon whether they occur in the context of a complex congenital syndrome, in which case they tend to be bilateral and multifocal; as opposed to those not associated with syndromes, which tend to be unilateral and focal. Immunohistochemical study is essential for diagnosis, in particular, to differentiate these tumors from germ cell tumors. This study reports on three cases of this tumor--one associated with Peutz-Jeghers Syndrome—and emphasis is given to histological criteria to define malignancy.

Spontaneous Sertoli Cell Tumor with Cryptorchism in a Beagle Dog / 한국실험동물학회지

A male one year-old beagle dog with unilateral cryptorchism was presented for investigation of reduced appetite. Abdominal sonography and radiography demonstrated abnormal enlargement of the left testicle in the abdominal cavity. Both the retroperitoneal cryptorchid testicle and the other contralateral testicle were removed surgically. The retroperitoneal cryptorchid testicle was an enlarged, firm and bulging sphere mass. The cut surface revealed a homogeneous white color. The contralateral testicle in the scrotum showed an almost normal appearance. Histopathologically, the retroperitoneal cryptorchid testicle was diagnosed as a Sertoli cell tumor. This report describes a case of Sertoli cell tumor with cryptorchism in a beagle dog.

Malignant Sertoli cell tumor in Shih Tzu dog / 대한수의학회지

Malignant Sertoli cell tumor was diagnosed in a 5-year-old male Shih Tzu dog. Clinical features of the dog were anorexia, urinary incontinence, constipation, anemia, alopecia, and epistaxis. The dog also had unilateral cryptorchid testis in the abdomen. Several abdominal and thoracic masses were identified on radiography. Grossly, the cryptorchid testicular mass was markedly enlarged to 8 cm in diameter. On cut surface, firm and well demarcated milk-white neoplastic areas were irregularly separated by white fibrous bands. Histologically, the testicular mass was diagnosed as tubular pattern Sertoli cell tumor. In addition, abdominal and mediastinal lymph nodes metastasis were found. Immunohistochemically, the tumor cells were strongly positive for vimentin and neuron specific enolase, but negative for S-100 and cytokeratin.

Tumores testiculares na infância: [revisão] / Testicular tumors in childhood: [review]

Testicular and paratesticular prepuberal tumors are rare. They represent around 1 percent of the total of tumors of infancy. They subdivide in 2 groups: germ cells tumors and non germ cells tumors, being able to occur in all the ages, and about 75 percent are malignant, and about 19 percent of these they present metastasis. The tumors of germ cells tumors represent 60 75 percent of the tumors testiculars in infancy, having as main example the yolk sac tumor (65 percent of the neoplasms), followed for teratomas (14 percent); although some works to exist where teratoma, if presents as most common .The non germ cells tumors include the Leydig cell tumor and Sertoli cell tumor. The Leydig cell tumor, are most frequent between the non germ cells tumors testicular. This review article on epidemiology, diagnosis and treatment of to testicular and to paratesticular tumors in child.

Testicular Sertoli Cell Tumor in an Adult

Sertoli cell tumors of the testis are very rare and are usually benign. Here we report a case of a Sertoli cell tumor of the testis in a 46-year-old man. His chief complaint was a painless, palpable testicular mass that he had for 4 years. Serum levels of tumor markers were within normal limits. Testicular ultrasonography showed a 1.5 cm sized well-demarcated nonhomogeneous echogenic mass in the left testis. Chest x-ray and abdominopelvic CT showed no metastasis. Radical orchiectomy was performed. Histopathology showed a Sertoli cell tumor with no evidence of malignancy.

Comparative immunohistochemical characterization of canine seminomas and Sertoli cell tumors

Primary testicular tumors are the most common causes of cancer in male dogs. Overall, the majority of canine patients should be cured by testicular surgery. However, tumor markers are not well-known in veterinary medicine. We sought to determine using immunohistochemistry whether the combined human testicular tumor markers (placental alkaline phosphatase, OCT3/4, CD30, alpha-fetoprotein, inhibin-alpha, vimentin, c-KIT, and desmin) are expressed in canine seminomas and Sertoli cell tumors (SCTs). We examined 35 canine testicular tumors, 20 seminomas and 15 SCTs. c-KIT was expressed markedly in canine seminomas. Both inhibin-alpha and vimentin were expressed significantly in canine SCTs. The results of this study demonstrate differences and similarities between tumor marker expression of testicular tumors in dogs and humans. All the main markers in current routine use are discussed as well as potential useful markers for benign and malignant tumors, and tumor progression.

Malignant Sertoli Cell Tumor of Testis / 대한비뇨기과학회지

A testicular Sertoli cell tumor is a very rare, usually benign, testicular neoplasm. Of the total number of reported Sertoli cell tumors, only 30 cases were malignant. Usually, these patients have a history of a slowly growing scrotal mass and gynecomastia. These neoplasms vary greatly in size and in their microscopic appearances. A radical inguinal orchiectomy is the initial treatment, with the efficacy of chemotherapy or radiotherapy being controversial. They have an aggressive metastatic behavior and a poor prognosis. Herein, the case of 67-year-old man with a malignant Sertoli cell tumor is reported.

Sertoli Cell Tumor of the Testis in a Young Child / 대한비뇨기과학회지

Prepubertal Sertoli cell tumor of testis is very rare and most of them are benign. The choice of treatment is radical orchiectomy but careful follow-up for possible retro peritoneal spread is appropriate. We report a rare case of Sertoli cell tumor of testis in a 8-year-old boy. His chief complaint was a painless left testicular swelling from birth. Serum levels of tumor markers were within normal limits. Radical orchiectomy was performed, and cut surface of testis was yellowish and was completely displaced by lobulated tumor mass. Postoperative CT and chest x-ray showed no evidence of metastasis. The patient is alive without evidence of disease for 8 months postopera tively.