Early development of a gonadal tumor in a patient with mixed gonadal dysgenesis

SUMMARY A gonadal tumor was diagnosed in the first months of life in a patient with genital ambiguity, a 45,X/46,XY karyotype, and mixed gonadal dysgenesis. Gonadal biopsies at the age of 3 months revealed dysgenetic testes and a gonadoblastoma on the right testis. Even though gonadal tumors are rare in childhood, this case indicates that prophylactic removal of dysgenetic gonads should be performed as early as possible, especially when the female sex is assigned to a patient with a Y-chromosome sequence.

Linfoma no Hodgkin primario testicular / Non hodgkin primary testicular limphoma

Los linfomas constituyen un grupo diverso de neoplasias malignas, que se originan del sistema linfático(1). Aproximadamente el 85% se agrupan dentro de los denominados Linfomas No Hodgkin (LNH). Los LNH constituyen un numeroso grupo de linfomas derivados de la transformación neoplásica de los linfocitos B, T y Células NK, provenientes de diversas zonas que comprometen los folículos linfáticos en los nódulos linfáticos o en el sistema linfoide extranodal(2). Las localizaciones extra ganglionares primarias representan más de 10% de los casos y los sitios más frecuentes comprenden estómago, intestino delgado, orofaringe, piel, hígado y cerebro; además de otras bastante raras como ano, corazón, músculos y encías. Los linfomas testiculares primarios son muy raros, representan aproximadamente el 5% de todas las neoplasias testiculares malignas, menos del 1% de los LNH y el 4% de los LNH extra nodales(3). Esta neoplasia constituye el tumor maligno testicular más frecuente en sujetos ancianos. La presentación clínica típica es una masa testicular unilateral acompañada en ocasiones de hidrocele y dolor agudo escrotal. A continuación presentamos un caso que requirió de la participación de múltiples especialidades de nuestra institución para un manejo conjunto y finalmente el paciente falleció(AU)

The lymphomas constitute a diverse group of malignant neoplasms, which originate from the lymphatic system(1). Approximately 85% are grouped into the so-called Non-Hodgkin's Lymphomas (NHL). NHL are a large group of lymphomas derived from the neoplastic transformation of B, T and NK cells of different areas with involvement of lymphatic follicles in the lymph nodes or the extranodal lymphoid system(2). The primary extraganglionic sites represent more than 10% of the cases and the most frequent sites include stomach, small intestine, oropharynx, skin, liver and brain; others, quite rare, are anus, heart, muscles and gums.Primary testicular lymphomas are extremely rare, accounting for approximately 5% of all malignant testicular neoplasms, less than 1% of NHL and 4% of extranodal NHL(3). This neoplasm constitutes the most frequent testicular malignant tumor in elderly subjects. The typical clinical presentation is a unilateral testicular mass, sometimes accompanied by hydrocele and acute scrotal pain. We present a case which required the participation of multiple specialties of our institution for management but the patient finally died(AU)

Serum Levels of Trace Elements in Patients with Testicular Cancers

Introduction: Trace elements are primary components of biological structures; however, they can be toxic when their concentrations are higher than those needed for biological functions. Materials and Methods: In the present study serum levels of trace elements were measured in 30 patients (mean age was 26.9±11.2 years) newly diagnosed with germ cell testicular cancer and 32 healthy volunteers (mean age: 27.4±10.8) by using furnace atomic absorption spectrophotometer. Serum samples were stored at-20°C until assays. Results: In patients with germ cell testicular cancer, the diagnosis was seminoma in 15, mix germ cell tumor in 7, embryonal carcinoma in 4, yolk sac tumor in 2 and teratoma in 2 patients. There was stage I testicular tumor in 19 patients (63.3%) while stage II in 6 patients (20.0%), stage IIIA in 4 patients (13.3%) and stage IIIC in one patient (3.4%). It was found that serum Co, Cu, Mg and Pb levels were increased (p<0.05), whereas Fe, Mn, and Zn levels were decreased in patients with testicular cancer (p<0.05). Conclusions: These alterations may be important in the pathogenesis of testicular cancers; however, further prospective studies are needed to identify the relationship between testicular cancer and trace elements.

Splenogonadal fusion and testicular cancer: case report and review of the literature / Fusão esplenogonadal e câncer testicular: relato de caso e revisão da literatura

A 36 year-old man after tests for assessing male infertility was diagnosed with primary infertility, bilateral cryptorchidism, nonobstructive azoospermia and discontinuous splenogonadal fusion. Carcinoma in situ was found in his left testicle, which was intraabdominal and associated with splenogonadal fusion. To our knowledge, this is the fourth case of splenogonadal fusion associated with testicular cancer reported. One should always bear in mind the possibility of this association for the left cryptorchid testicle.

Um homem de 36 anos, depois de ser submetido a exames para avaliação de infertilidade masculina, foi diagnosticado com infertilidade masculina primária, criptorquidia bilateral, azoospermia não obstrutiva e fusão esplenogonadal descontínua. Carcinoma in situ estava presente no testículo esquerdo, que tinha localização intra-abdominal e estava associado à fusão esplenogonadal. Esse é o quarto caso de fusão esplenogonadal associada a câncer testicular, segundo nossa avaliação. Deve-se sempre ter em mente a possibilidade dessa associação em testículos criptorquídicos à esquerda.

Familial germ cell tumor.

Familial testicular germ cell tumors are well known in literature. Only few cases are reported where both brother and sister of the same family suffered from germ cell malignancies. We present a family where the proband is a survivor of ovarian dysgerminoma stage IA. Her elder male sibling became acutely ill and was detected to have disseminated testicular malignancy with grossly elevated markers and vegetations in the mitral valve leaflets. Despite all measures he could not be saved. Presence of germ cell malignancies in the siblings of different sex in the same family points toward a genetic susceptibility. Literature review revealed only six similar cases. A discussion regarding the rare occurrence of familial germ cell malignancies with the affected family members may be worthwhile.

Tumor testicular por restos suprarrenales en un adolescente con hiperplasia suprarrenal congénita clásica: revisión de su fiopatología e implicancias en fertilidad / Testicular adrenal rest tumors in a teenager with classical congenital adrenal hyperplasia: report of one case

Testicular Adrenal Rest Tumors (TART) may play a role in fertility disturbances of patients with Classical Congenital Adrenal Hyperplasia (CAH). We report a 17 years old male with classical CAH, diagnosed in the newborn period due to a salt wasting crisis with dehydration and severe hyponatremia. He was treated with cortisol and fludrocortisone with a low adherence to therapy. He had a precocious puberty and accelerated bone age, and was treated with a luteinizing hormone releasing hormone (LHRH) analog during two years. At the age of 14 years, bilateral testicular masses were detected during the physical examination. Testicular color Doppler ultrasound showed the presence of TART. A new ultrasound at 17 years of age showed the persistence of adrenal rests and an abnormal testicular growth.

Primary testicular non-Hodgkin's lymphoma: a review article

Primary testicular non-Hodgkin's lymphoma was first described as a clinical entity in 1866. It is a rare disease and accounts for 1 percent of all non-Hodgkin's lymphoma, 2 percent of all extranodal lymphomas and 5 percent of all testicular neoplasms. It is the most common testicular tumor in males between sixty and eighty years of age. Testicular non-Hodgkin's lymphoma is unique in its high incidence of bilateral involvement (8-38 percent), and it is also the most common bilateral testicular tumor. Testicular non-Hodgkin's lymphoma has a predilection for spreading to non-contiguous extranodal sites, especially the central nervous system. Advanced-stage disease is usually managed with doxorubicin-based chemotherapy. For early-stage disease, opinion is divided regarding systemic chemotherapy following orchidectomy. The high incidence of spreading, especially to the central nervous system, leads to advocacy of the use of central nervous system prophylaxis with intrathecal chemotherapy. Prospective multicenter trials incorporating a large number of patients may lead to better guidelines for optimal management of this subtype of non-Hodgkin's lymphoma.

O linfoma primário do testículo (LPT) foi descrito como uma entidade clínica pela primeira vez em 1866. É uma doença rara e corresponde a 1 por cento de todos os linfomas não-Hodgkin, 2 por cento de todos os linfomas extranodais e 5 por cento de todos as neoplasias testiculares. É o tumor testicular mais comum em homens entre 60 e 80 anos de idade. LPT é único em sua elevada incidência de envolvimento bilateral (8-38 por cento), sendo o tumor testicular bilateral mais comum. Tem uma predileção por disseminação para regiões extranodais não-contíguas, especialmente para o sistema nervoso central (SNC). Estágios avançados da doença são usualmente tratados com quimioterapia à base de doxorubicina. Para os estágios mais precoces, as opiniões são divergentes quanto à quimioterapia associada à orquiectomia. A alta prevalência de disseminação, especialmente para o SNC, sugere o uso de quimioterapia intratecal como profilaxia. Estudos prospectivos multicêntricos incluindo um grande número de pacientes poderiam resolver a questão com relação ao manejo deste subtipo de linfoma não-Hodgkin.

Gonadal Tumors Developed from Consecutively Transplanted Spleens Bearing Gonad Implants

This study was conducted by consecutively transplanting spleens, which had gonads implanted previously. A total of 84 cases for infantile testicles and 106 cases for ovarian follicles were performed. In the case of ovarian implants, the results were determined by the total number of follicle implants. A modified spleen transplantation technique called double implantation of ovarian follicles was applied to increase the amount of the implants. In this technique, an extra spleen is implanted into the potential donor so that the ovarian follicles can be implanted to two different spleens, doubling the amount of implants. Through consecutive spleen transplantation, we observed the results beyond a typical rat's life span. In many of these cases, we found more aggressive forms of malignant tumor, seminoma and dysgerminoma. We present the results and discuss possible pathogenic mechanisms of tumor formation.

Cryptorchidism and testicular neoplasia

Traditionally there is a concern about the possibility of developing testicular tumors as a consequence of undescended testis. This study was conducted to investigate the correlation between testicular tumors and undescended testis. Medical records of all patients with testicular germ cell cancer were seen and examined at the Princess Basma Teaching Hospital, Jordan University of Science and Technology, Irbid, Jordan, between 1990 and 2000 [group 1] for any evidence of undescended testis. All males with undescended testis attending the infertility clinic during the period 1999-2001 [group 2] were re-evaluated for any evidence of testicular tumors. The operative records of all patients who had surgery for undescended testis during the past 30 years [group 3] were reviewed and the patients were contacted to see if any of them developed testicular tumor as expected. Forty-four patients with testicular germinal cell tumors; mean age at diagnosis were 32.6 years [range 20-50] were identified. All patients had unilateral involvement and none of them were found to have tumors in undescended testis. The tumor was in the scrotum in all patients. None of the 117 infertile patients with undescended testis had clinical or ultrasonic evidence of testicular tumors. Two thousand nine hundred and nineteen patients had an operation for undescended testicle in the past 30 years, average of 97.3 operations annually. Median age at surgery for undescended testis was 7.7 years [range 2-29 years]. None of the 2071 patients who were traceable developed testicular tumor. We found no evidence to support that patients with undescended testis are at a higher risk than the general population to develop testicular cancer

Testículo no descendido / Cryptorchidism

Testículo no descendido es la falta de descenso testicular en el saco escrotal. Si esto ocurre en cualquier punto del trayecto normal de descenso recibe el nombre de criptorquidia y si ésta se ubica por fuera se denomina testículo ectópico. El testículo retráctil o en ascensor es aquel testículo no descendido que desciende fácil a nivel escrotal y permanece en éste durante el examen, constituyendo una condición normal frecuente durante la infancia, especialmente entre los 2 y 7 años, por un acentuado reflejo cremasteriano

Testicular and paratesticular tumours at the University Hospital of the West Indies

A review of tumours of the testis and paratesticular region diagnosed at the University Hospital of the West Indies over a 30-year period revealed 14 of the former and 22 of the latter. The testicular tumours were all malignant, with 50 per cent of them being gerem-cell neoplasms. Seventeen of the 22 paratesticular tumours, (77.3%) were benign. The histological types, racial incidence and possible aetiological factors are discussed and compared with the findings of other series