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A 51-year-old male presented with metastasis of a small cell carcinoma of unknown origin in a right inguinal lymph node. Clinical and laboratory studies failed to locate the primary tumour. After three years, a swelling appeared in the right testis, which was removed. Histological examination revealed a proliferation of small tumour cells forming irregular masses or nests that occupied most of the testicular parenchyma. At the periphery of the testicular parenchyma a few seminiferous tubules could be observed, showing a low and incomplete seminiferous epithelium and numerous tumour cells in the lumen. Most of the tumour cells showed a euchromatic nucleus with small nucleoli and scanty cytoplasm. Among these cells, larger binucleate or trinucleate cells as well as small cells with pyknotic nuclei were also observed. Mitoses were numerous. Electron microscopy revealed some tumour cells with 80 to 100 nm vesicles containing electron-dense granules. Some cells displayed dendrite-like prolongations with numerous intermediate filaments and electron-dense vesicles. This tumour is compatible with a primary neuroectodermal tumour of the testis.

The testes and epididymes collected at autopsy from 21 newborns showed apparent Leydig cell hyperplasia which was studied by light and electron microscopy. Twelve newborns were the sons of diabetic mothers, two had undergone rhesus isoimmunization, two were twins of a non-diabetic mother, three had Beckwitz-Widemann's syndrome, and two had leprechaunism. In the first two groups the placentas were also collected and studied. All the testes showed normal seminiferous tubules and diffuse Leydig cell hyperplasia in the testicular interstitium. In addition one son of a diabetic mother and another with Beckwitz-Widemann's syndrome presented multiple Leydig cell nodules in the mediastinum testis and epididymis. The number of Leydig cells per unit area of the testis was calculated on histological sections stained with the peroxidase-anti-peroxidase method for the detection of testosterone. These numbers varied from 1.4 to 3.2 times those found in age-matched controls, except for the two testes with nodular hyperplasia in which the increase in Leydig cells was even greater. The differential diagnosis between Leydig cell hyperplasia, ectopic adrenal cells and leydig cell tumour is discussed. It is proposed that the cause of congenital Leydig cell hyperplasia might be related to placental secretion of human chorionic gonadotrophin.

One case of bilateral "in situ" gonadoblastoma associated with aniridia and Smith-Lemli-Opitz syndrom is reported. This observation confirms the incidence of gonadoblastoma in intersex patients. Coincidence of this tumor and aniridia is considered by the authors as previously unreported.

Normal (infant and adult) and pathological testes were examined by electron microscopy in order to study testicular innervation. Nerves composed of non-myelinated fibres were abundant in the tunica vasculosa of the tunica albuginea. These nerves penetrated into the testicular septa reaching the interstitial tissue. This showed numerous non-myelinated nerve fibres running among the Leydig cells and blood vessels. Single axons or small groups of them, partially surrounded by Schwann cells, approached: 1) the Leydig cells, 2) the interstitial blood vessels, and 3) the seminiferous tubules. Single naked axons were also observed primarily in the proximity of the seminiferous tubules. These axons showed varicosities containing both small and large "synaptic" vesicles. The latter were less numerous and contained a central dense core. Small vesicles were agranular. Some varicose axons ran across the myofibroblast layer of the tunica propria reaching the basal lamina of the seminiferous tubules at the level of the Sertoli cells but not at the level of the spermatogonia. The intercellular space between Sertoli cell and axon membrane was about 150-200 nm.

A retroperitoneal tumor was removed from a fifteen-day-old infant. Light microscopy revealed a teratoma consisting mainly of immature nervous tissue. Three months later the patient had recurrence and numerous peritoneal metastases showing a histologic pattern similar to that of the primary tumor. Twelve months later there was enlargement of the left testis due to metastases from teratoma infiltrating the tunica vaginalis of the left testis, the epididymis, and the spermatic cord. These metastases consisted of mature neurons and glial cells. The early dissemination of the tumor suggests an intracavitary spread pattern. The tumor maturation in paratesticular structures suggests that mesothelial cells are involved in the differentiation of tumoral germ cells.

Two cases of nemaline myopathy, a boy and a girl, are presented. Both children show a general and facial appearance peculiar to this myopathy of which, about 38 cases have been described till now in medical literature. The diagnosis is made, by microscopic or ultramicroscopic study, which shows the characteristic rods of the disease. However, the clinical symptomatology of this syndrome is peculiar, with little muscular mass, a long face with an open mouth from which the spittle runs easily, muscular hypotony, myotatic areflexia of hyporeflexia, normal serum enzymes and E.M.G. myopathic of normal. This myopathy sometimes appears spontaneously and sometimes it is hereditary, this latter factor can be recessive or dominant with littre penetrability of the pathogenous gene.

Report of a case of multicentric reticulohistiocytosis with clinical, histopathologic, histochemical and immunohistochemical study. Neutral mucins and lipids were demonstrated in the cytoplasm of dermal histiocytes. The monocyte-histiocytic nature of the cells was shown by means of acid phosphatase, ATP-ase, muramidase and alpha-1-antitrypsin. Due to the immunohistochemical results, the relationship with Langerhans cells was rejected. No secretory activity of immunoglobulins was shown and the proliferation qualified as benign. The cells among the histiocytic population were typified as mature T lymphocytes.

A 3-year-old girl had a 4-month history of a tumor in her right hand. The tumor was located in the subcutaneous and soft tissues of the palm and the long, ring, and small fingers. Histologic studies showed a malignant fibrous histiocytoma that was confirmed by the ultrastructural study as having a fibroblastic and histiocytic origin. The long, ring, and small fingers were amputated. The postoperative course was normal, and 18 months later no recurrence or metastases were observed.

Ultrastructural cytochemical study of the nuage in the human adult testis revealed that this structure was a cytoplasmic fine fibrillar electron-dense mass, similar to the chromatoid body of spermatids, in all spermatogonial types and spermatocytes. The nuage was often observed in relation with the nucleus or mitochondria. Cytochemical techniques showed staining affinity of the nuage for both ethanolic phosphotungstic acid and ethylene diamine tetra-acetic acid. The intermitochondrial bars were also stained with the two procedures. The results suggest that the nuage originates from the nucleus and migrates to the cytoplasm through nuclear pores, giving rise to the intermitochondrial bars.

The histological study of epididymides obtained from autopsies (41 children, 7 young adults and 8 elderly men) and surgical specimens (10 young adults and 3 elderly men) revealed the presence of multiple diverticula in the cauda epididymis in all elderly men and in only 2 young adults (32 and 33 years old). The diverticula appeared as evaginations of the epithelial lining of the duct, disrupting the muscular coat and expanding into the surrounding connective tissue. The formation of epididymal diverticula seems to be an age-related process that might be caused by repeated increases in the intraluminal pressure in the cauda epididymis.

The ultrastructural study of testicular biopsies from 87 adult men revealed mitosis in two mature Leydig cells, each from a different man. The men showed normal hormone levels and had received no previous chemotherapy or hormone treatment, nor had they been exposed to known toxic agents. The presence of mitotic Leydig cells suggests that differentiated Leydig cells may divide and contribute either to the increase in the number of Leydig cells or to the formation of multinucleate Leydig cells.

Mediastinal teratomas are reported in three children. Two cases: an eighteen-month-old female and a twelve-years-old female, were diagnosed of mature cystic teratoma. Third patient was a twelve-year-old male with an immature mediastinal teratoma. All tumors were located in anterior mediastinum. Mediastinal mature cystic teratomas were completely excised and their evolution were favourable. However, immature mediastinal teratoma infiltrated lung parenchyma and great blood vessels; in this case only a biopsy was performed. This patient died a month after surgical procedure. Clinical, radiological and pathological findings of mediastinal teratomas in childhood are commented. Prognostic factors and evolution of different histological types of mediastinal teratomas are evaluated.

Intracanalicular or intra-abdominal undescended testicles often present a difficult surgical problem, due to a vascular pedicle shortening that prohibits scrotal placement with a 1 stage orchiopexy. The division of spermatic vessels to properly place the testis in the scrotum solves the problem of the shortening of the vessels, but raises the question of whether the endocrine function and the potential for fertility are preserved. In the present study transection of spermatic vessels before arterial branch-off was done intra-abdominally in rats. Angiographic, patho-anatomic and hormonal evaluations have been made. Revascularization of the testicular artery through the vasal artery has occurred in all cases. The anastomotic circuit, however, does not always have the same functional value. The spermatogenic alterations suggest that fertility can be impaired in a significant percentage of cases. Spermatogenesis was quantified taking into account the mean tubular diameter and the situation of seminiferous epithelium, expressed as the percentage of tubules containing well-developed spermatogenesis. Plasma testosterone levels are in a low range. However, Leydig cell functional response to human chorionic gonadotropin stimulation is maintained in every case and so are plasma and pituitary luteinizing hormone levels, suggesting that plasma testosterone levels are high enough to maintain an adequate hormonal activity.

Juvenile granulosa cell tumor occurred in a newborn. The tumor presented with testicular torsion, and no malformations were observed. The karyotype was normal. The occurrence of initial tumoral lesions in the seminiferous tubules located in the vicinity of the tumor suggests that the tumor originated from immature Sertoli's cells. To our knowledge, this is the tenth case reported in a newborn and the second associated with testicular torsion.

The numbers of Ap and Ad spermatogonia per unit section of the testis were calculated in autopsy specimens from young adults and elderly men without testicular pathology. The number of Ap spermatogonia decreased from the 6th decade of life, whereas that of Ad spermatogonia began to decrease in the 8th decade. Although it has been reported that Ad spermatogonia are more sensitive to noxious agents than Ap spermatogonia, the involution of Ap spermatogonia precedes that of Ad spermatogonia. These findings provide new information on concepts relating to the spermatogonia precedes that of Ad spermatogonia. These findings provide new information on concepts relating to the spermatogonial stem cell in man.

Human testicular specimens were obtained from biopsies and autopsies covering the period from birth to adulthood. The number of testosterone-containing Leydig cells was determined using the peroxidase-anti-peroxidase method. This number decreased markedly from 3-6 months of age to the end of the first year of life and, up to 6 years of age, only a small number of testosterone-containing cells was found. From 6 years onwards the number of Leydig cells progressively increased. Ultrastructural examination revealed four types of Leydig cells: fetal-type Leydig cells (from birth to 1 year of age) with round nuclei, abundant smooth endoplasmic reticulum and mitochondria with tubular cristae; infantile-type Leydig cells (from birth to 8-10 years of age), showing a multilobated nucleus, moderately abundant smooth endoplasmic reticulum, some lipid droplets and mitochondria with parallel cristae; prepubertal, partially differentiated Leydig cells (from 6 years of age onwards) with regularly-outlined round nuclei, abundant smooth endoplasmic reticulum, mitochondria with tubular cristae, and some lipid droplets and lipofuscin granules; and mature adult Leydig cells (from 8-10 years of age onwards). The ultrastructure of the infantile-type Leydig cells and the lack of delay between the disappearance of the fetal-type Leydig cells and the appearance of infantile-type Leydig cells suggest that fetal-type Leydig cells give rise to the infantile-type Leydig cells. Before puberty, myofibroblast-like precursor cells differentiate into the prepubertal, partially differentiated Leydig cells, which complete their differentiation into the adult Leydig cells.