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Myxoid/round cell liposarcoma is a soft tissue sarcoma that is extremely rare in the brachial plexus. We report a case of a myxoid/round cell liposarcoma originating in the brachial plexus that was surgically resected and evolved well, with no deficit or recurrence after 2 years of follow-up. To date, there has been no other case of this sarcoma in the literature., (Copyright © 2014 Sociedad Española de Neurocirugía. Published by Elsevier España. All rights reserved.)

Objectives: To report the second case of solitary neurofibroma arising from the tunica albuginea in the literature and to show its imaging findings., Methods/results: We present a case of neurofibroma arising from the tunica albuginea in an adult patient not affected by neurofibromatosis. We describe the ultrasonographic and magnetic resonance imaging (MRI) features and the histopathological characteristics along with a brief bibliographic review., Conclusion: MRI may be useful to characterize paratesticular lesions. Neurofibroma should be included in the differential diagnosis when MRI depicts a well-circunscribed tumour with high-signal intensity on T2 and marked enhancement after gadolinium administration.

Primary choriocarcinoma of the lung is an extremely rare condition with a fatal outcome in most patients. We report the case of a 31-year-old woman with cough, chest pain, and hemoptysis, whose chest radiograph displayed nodules in the right upper lobe. After study and an unsuccessful attempt at histological characterization of the lesions, exploratory surgery was indicated. The patient underwent lobectomy and lymphadenectomy after diagnosis of a malignant process in the intraoperative biopsy. The definitive diagnosis was choriocarcinoma. Gonadal involvement was ruled out and the patient was referred to the oncology department for adjuvant therapy. She remains free of disease after 42 months. Although choriocarcinoma of the lung is extremely rare and it is hard to establish a standard treatment, in cases of localized disease, surgical resection followed by chemotherapy seems to be useful and can achieve long survivals.

Background: To report the cases of adenomatoid tumors seen at Hospital Universitario La Paz in the last 15 years., Methods: A clinical, pathological, and surgical study was conducted of males with testicular or paratesticular tumors with a histological report of adenomatoid tumor., Results: Among the nine cases studied, seven had paratesticular and two intratesticular adenomatoid tumors. Treatment of choice was mass removal for epididymal tumors and orchidectomy for intratesticular tumors., Conclusions: Adenomatoid tumors are uncommon benign neoplasms of a possible mesothelial origin. Because of their benign nature, the treatment of choice is local excision (conservative surgery), but orchidectomy was performed in two cases due to tumor location.

Objective: To create an animal model to stabilize the aneurysmal sac and prevent type II leaks in the endovascular treatment of aneurysms of the abdominal aorta (AAA) by placing an endoprosthesis (stent-graft) and filling the aneurysmal sac., Material and Methods: AAA were experimentally induced in pigs and sheep; AAA were subsequently treated with endovascular stent grafts and stabilized in the same procedure by introducing ethibloc and onyx through a vascular catheter placed within the aneurysmal sac. All animals underwent aortography, and MR angiography and abdominal CT were performed on randomly selected animals. Histological studies included both macroscopic and microscopic examination. Two sheep were analyzed one year after the procedure. We evaluated the characteristics of the materials employed, their diffusion in the collateral arteries, and their efficacy in stabilizing the aneurysmal sac., Results: The procedure was successfully carried out in all animals. Survival in the pigs was 100 %, whereas greater instability under anesthesia in the sheep led to 50 % perioperative mortality. Both ethibloc and onyx were considered easy to use and stable over time., Conclusion: The sac of an AAA treated with an endovascular stent graft and its collateral vessels can be treated in the same procedure with materials introduced through a catheter.

The gonadal biopsy provides essential information for the identification, classification and early detection of neoplasias in patients with disorders of sex development. Histopathological findings in these cases must be analysed together with clinical, hormonal, genetic and molecular information before deciding a therapeutic option. Sexual differentiation is the result of multiple and complex genetic and endocrinal mechanisms; therefore, we first present the events taking place during gonadal embryonic development, focusing on the genetic mechanisms involved in sexual determination and the differentiation of the testis and the urogenital tract. In second place, we describe the different gonads in the intersexual states -in testicular regression syndrome, fibrous streak, testicular dysgenesis, streak testes, ovotestes and microscopically normal testes and ovaries-, highlighting the histological features and the differential findings that allow the pathologist to distinguish between these entities with the aid of clinical, genetic, hormonal and molecular information that are characteristic for each situation. In third place, we studied the incidence of neoplasias in gonadal dysgenesis, male pseudohermaphroditism and true hermaphroditism. Finally, we discuss the limitations of gonadal biopsy to achieve a correct diagnosis in the disorders of sex development.

We describe the case of an 18-year-old female with Cowden syndrome in whom a simple x-ray detected a solitary pulmonary nodule that was identified as a sclerosing hemangioma. Pulmonary sclerosing hemangioma is an unusual lung neoplasm which typically presents as a solitary peripheral nodule in asymptomatic women. Although the histology of this entity is well defined, its origin and treatment is debated. One of the main diagnostic problems is to histologically differentiate a pulmonary sclerosing hemangioma from a papillary lung carcinoma.

Myofibroblastic inflammatory tumor is a controversial entity that shows great variability in clinical presentation, histological findings, evolution, and prognosis. It is a rare cause of primary lung tumor in adults; however, it is the most common cause of lung tumors in children. The diagnosis is fundamentally histological, although histological diagnosis is not easy because myofibroblastic inflammatory tumor is characterized by a polymorphic cellular infiltration of variable cellular composition that could be similar to other diseases such as lymphoma or low-grade sarcoma. We report the case of a 23-year-old woman in whom a solitary pulmonary nodule was discovered incidentally at plain-film chest x-ray.

An atypical case of primary ciliary dyskinesia is presented in which the inheritance, rather than the classical autosomal recessive, appears to be transmitted as an autosomal dominant trait through the maternal line. The case involves two brothers of 29 and 30 years of age, married without children, with a history of infertility, frequent episodes of sinusitis, and recurrent pulmonary infections. Their mother and sister have chronic bronchopathy of unknown etiology. Their father is healthy without pulmonary problems or sinusitis. At physical exam, both brothers, sister and mother presented with bronchial rhonchi at lung auscultation. Blood analysis and pulmonary function, liver and renal tests were all normal. The ultraestructual study of the sperm flagellum by electron microscopy revealed that both brothers have the same anomaly. Namely, in the majority of the cross-sections, both dynein arms are missing. The nexin filament was present, as well as the radial spokes and the central pair of microtubules. In some sperm, besides the absence of dynein arms, there was also absence of the central pair of microtubules. Neither anomalies of the fibrous sheath nor of the dense fibers were found. In approximately 50% of the spermatozoa, the midpiece had a decreased number of mitochondria and extra non-aligned mitochondria. Other findings included extra peripheral microtubules in the axoneme.

Macroorchidism is a rare condition in children and is usually associated with fragile X syndrome. Other possible etiologies of macroorchidism are long-standing primary hypothyroidism, adrenal remnants in congenital adrenal hyperplasia, follicle stimulating hormone (FSH)-secreting pituitary macroadenomas, local tumors, lymphomas, and aromatase deficiency. Bilateral macroorchidism can be a normal variant in adult men. We report the case of an 11.5-year-old boy who was referred to our hospital for evaluation of marked bilateral testicular enlargement in the previous few months. Physical examination revealed a testicular volume larger than 30 ml. Complementary investigations allowed us to rule out all hitherto reported etiologies of bilateral macroorchidism and therefore a diagnosis of idiopathic macroorchidism was established. The increased number of Sertoli cells found on biopsy, without elevated plasma FSH levels, prompted us to speculate about a hypothetical FSH receptor hyperfunction as a possible cause of the exaggerated testicular enlargement in this patient.

Objectives: To review current theories about etiology of varicocele and pathogenic mechanisms leading to a progressive disorder of spermatogenesis in relation to the subfertility or infertility these patients may present with. To evaluate its current anatomical knowledge of the normal venous drain of the testicle and its variations that may condition relapse or failure of the treatment of varicocele. To systematically review pathologic testicular lesions in patients with varicocele. To establish factors that may have prognostic significance on post-treatment fertility., Methods: We performed a systematic search in the Medline database for each of the proposed etiological and pathogenic theories on human varicocele. The evaluation of pathologic testicular lesions in patients with varicocele was obtained from the study of testicular biopsies performed at the Hospital La Paz in Madrid over the lost 30 years., Results: Regarding the anatomical theories of varicocele, congenital absence or incompetence of the internal spermatic vein valves, difficult venous drain, augmented hydrostatic pressure of the internal spermatic vein, disorder of the fascial-muscular pump mechanism, and compression of the venous drainage system are considered, among others, potential etiological factors. Regarding possible pathogenic theories of varicocele, we evaluate disorders of testicular thermoregulation, hypoxia, toxic effect of renal and adrenal metabolites, certain endocrine disorders, obstruction of the spermatic tract, disorders of blood flow and epididymal vasculature, oxidative stress, gonadotoxins, apoptosis, and lastly the effect of varicocele on the contralateral testicle., Conclusions: Available data support the idea that etiopathogenesis of varicocele is multifactorial. Many classic etiopathogenic factors related to anatomy, embryology, obstruction, and hyperthermia still prevail in addition to new factors related to oxygen reactive species and apoptosis. However, many pathogenic and physiopathologic aspects of varicocele need to be elucidated yet. As a matter of fact, neither of these data alone may clearly explain the variable effect varicocele has on spermatogenesis and male fertility. So, it is necessary to establish histological criteria with proved prognostic significance that allow us to detect possible progression of testicular lesions after treatment.

Bronchocentric granulomatosis is a non-specific necrotizing granulomatous reaction in the bronchi and bronchioles. In asthmatic patients, bronchocentric granulomatosis is considered a hypersensitivity reaction to intrabronchial fungi. However, in non-asthmatic patients the possible etiology has not been identified. We report the case of a 46-year-old woman with bronchocentric granulomatosis associated with pulmonary hydatidosis. The likely immuno-pathogenic mechanisms leading to bronchial lesions are briefly discussed.

Objective: Various tumors have been described in the scrotal area arising from skin and the underlying tissues: nevus, dermoid and epidermoid cysts, epidermoid carcinomas, lipomas, leiomyomas, angiokeratomas, lymphangiomas, granular cell tumors, granuloma, malignant tumors of the peripheral nerve tissue, and some 'pseudotumors' such as fibromatosis and nodular calcinosis. We describe for the first time a sweat gland tumor, which is also remarkable for its unusually large size., Methods/results: A 76-year-old patient consulted for a painful left scrotal tumor that he had noted for some time and that had slowly and gradually grown. Ultrasound assessment of the GU system confirmed the presence of a 4.2 cm left, solid paratesticular mass that was removed under local anesthesia., Conclusions: Chondroid syringoma is a tumor arising from the sweat gland that is usually localized to the head and neck. It has an excellent prognosis and recurrence has only been described in patients in whom the tumor had not been completely resected.

Objective: To report a case of locally invasive angiomyofibroblastoma of the scrotum. To our knowledge, this is the first case of invasive scrotal angiomyofibroblastoma reported in the literature., Methods: A case of invasive angiomyofibroblastoma of the scrotum is presented. The literature is reviewed with special reference to the etiopathogenesis, clinical features, diagnosis and treatment of this rare soft tissue tumor., Results/conclusions: The case described herein shows that angiomyofibroblastoma can be invasive.

Hyalinizing trabecular adenoma (HTA) is a follicle-derived tumor of the thyroid gland that has been described recently. The differential diagnosis includes medullar carcinoma, papillary carcinoma, and paraganglioma of the thyroid. We report a case of thyroid HTA in a 25-year-old woman. The tumor appeared as a <> of the left thyroid in radionuclide scans. Histologically, the tumor showed tumor cells arranged in trabeculae and a prominent hyaline stroma. The neoplastic cells were focally immunoreactive for thyroglobulin and negative for calcitonin, chromogranin, and S-100 protein. Three years after hemithyroidectomy, the patient is alive and free of disease.

Objective: To report a case of paraganglioma localized in the organ of Zuckerkandl and to discuss its diagnosis, treatment and outcome., Methods/results: A 37-year-old female presented with a periumbilical mass and pain that radiated to the lumbar region for the past three months. Abdominal US and CT disclosed a well vascularized mass lying adjacent to the aorta, 1 cm from its bifurcation. At laparotomy, an 8 x 10 cm tumor was discovered at the aortic bifurcation that displaced the left ureter and included the inferior mesenteric artery. Pathological analysis of the surgical specimen revealed a paraganglioma., Conclusion: Extra-adrenal paraganglioma should be taken into account in the differential diagnosis of retroperitoneal masses, particularly those adjoining the abdominal aorta.

Adrenocortical tumors are uncommon in childhood. The incidence of these tumors is about 0.3-0.4% of all the solid tumors in childhood. Because of its low incidence, there are no established protocols or uniform histological classification. Most of these endocrine tumors are hormone producing, causing virilization, Cushing's syndrome, feminization, hiperaldosteronism or hipoglicemy. Only a few of these do not produce hormones. The treatment is always surgical. The efficacy of another treatments (chemotherapy or radiotherapy) has not been demonstrated in infancy. It prognosis depends on the age and the resectability.

Coronary fibrinolysis through intravenous via is a usual technique in ICU worldwide, it is a procedure of controlled risk which provides great benefits to patients who undergo such technique. The role of nursing in the administration and control of the fibrinolytic treatment is essential. Despite that, the plans for fibrinolysis specific care are very scarce, if not inexistent, in the bibliography reviewed. The main objective of this work is identify the complications derived from the administration of fibrinolytic medicines through intravenous via. Using a chart of collected data, which goes from the admission of the patient to their discharge from ICU, 178 cases are studied. After the analysis of results, a standardization of intensive care units orientated to increase the assistance quality is suggested.

Macro-orchidism (MO) is the increase of the testicular volume, up to 25 ml in the adult male. It is frequently associated with mental retardation (MR) with fragile X-chromosome (FXC) (Martin-Bell syndrome). Sometimes it is of unknown origin and is called "benign idiopathic macro-orchidism" (BIMO). MO has also been described in association with bilateral testicular tumors, idiopathic precocious puberty, juvenile hypothyroidism and, more rarely, with congenital testicular cysts (cystic testicular dysplasia) and testicular microlithiasis in a patient treated with GH. The most common presentation is MR associated with MO, with positive or negative FXC. Among MO with MR and FXC-marfanoid habitus patients have been described and in the Atkin-Flaitz syndrome patients. Management of MO must be conservative in all cases and testicular biopsy must only be performed to diagnose leukemic infiltration or carcinoma in situ (CIS), or as the last fertility diagnostic test in BIMO. A wide range of primary testicular lesions may histopathologically be found: preserved spermatogenesis tubes, only Sertoli cell pattern and complete tubular sclerosis. Interstitial edema, frequently implicated in the MO pathogeny, does not explain by itself the important increase in testicular volume. In our laboratory, we have demonstrated that the increment of the testicular volume in MO is associated with a significant increase in the length of the seminiferous tubules. MO may pathogenically be intimately related to some hormonal regulation mechanism or to a higher seminiferous tubule sensitivity to FSH.

Three cases of Leydig cell tumor are described. Patient ages were 27, 44 and 55 years, respectively. The first two patients consulted for a testicular mass and the third patient presented with gynecomastia, an incidental finding during the ultrasound work up of a hydrocele. Hormonal evaluation revealed a marked serum testosterone level in one case, whereas in another case it fell within the lower limits of normal ranges. No evidence of dissemination was observed in all patients. Treatment was by radical inguinal orchiectomy in all patients. The pathological analysis of the surgical specimens revealed Leydig cell tumor with no histologic evidence of malignancy. A follow-up of 32, 14 and 10 months, respectively, revealed no evidence of tumor recurrence in all patients. The clinical and histologic features and treatment of this disease process are described.

Multiple series of undescended testes reported in the 1970's shown a significant decrease in spermatogonia content and tubular growth. After that most pediatric surgeons perform elective orchiopexy earlier. Pathologic studies in undescended testes shown significant complications. We studied 82 patients who were surgical treated between 1 to 13 years and we studied the tubular fertility index (TFI), mean of tubular diameter and Sertoli cells index (SCI). We found no correlation between age of patients and lesions. In our point of view the alterations of undescended testes is secondary to primary disease no to remain out of the scrotum. Current evidence suggests that the optimal time for performing orchiopexy is between four to seven years.

Authors present a case of a Leydig cell testicular tumor in a boy aged three years with a six-month follow-up. This type of tumor which is usually hormone-producing has in children a virilizing effect giving signs of pseudoprecocious puberty. Hormonal tests before and after surgery are described as well as the histological study. The various possibilities of differential diagnosis, treatment and prognosis in the presence of a unilateral testis enlargement in a boy are commented.

A case of arterial and venous thrombosis in a newborn is presented. The etiopathogenic aspects are discussed, and the possible relationship between arterial and venous thrombosis in comparison with clinical renal vein thrombosis are evaluated. Appropriated treatment for these patients is presented.

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.

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.

We performed vasectomy and microsurgical repair of the vas deferens in 20 rats utilizing absorbable and non-absorbable 10-0 sutures. At 40 days the results of the repair procedure were evaluated by seminal fluid and fertility analyses, deferentography, and histologic examination of the anastomoses. The procedure failed in one case that presented occlusion of the spermatic cord from a granuloma.

A case of renal vein and inferior cava thrombosis in a previously catheterized infant with cyanotic congenital heart disease is reported. The different origins of R.V.T. in newborns and infants are briefly discussed. The different factors that favour the beginning of thrombotic process (cyanotic heart disease diabetic mother, etc.) are also emphasized. Some considerations are made on the danger inherent to the use of intravascular catheters.

Two brothers have spermiograms with a similar pattern: absolute teratozoospermia with round-headed spermatozoa. The familial trait only has been previously one time in the literature. The spermatozoa show several abnormalities in the head. They lack acrosome and postacrosomal sheath. The nuclei are small and the chromatin is distributed in small groups. The nuclear envelope has abundant pores and is loosely attached to the chromatin. All the nuclei have brown inclusions, refringent when they are observed under the light microscope, vesicular and laminar under the electron microscope. Other abnormalities less frequently observed are: irregularities in the mitochondrial sheath, loss of parallelism of the dense fibres and duplication and angulation of the sperm tail. The hormonal assays and the biochemical tests are normal in both patients.

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.

This paper describes a case of Klinefelter's syndrome with 48,XXYY. Patient had mental retardation and dysmorfic face. Although mental retardation may be recognized early in life, it is difficult to establish a clinical diagnosis of Klinefelter's syndrome before puberty when small testes, gynecomastia and other phisical stigmata may become apparent.

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.

One case of renal venous thrombosis in a male foetus, born of a diabetic mother is reported. The intrauterine origin and the long age of the thrombosis were confirmed by pathologic studies. Some speculation about the etiopathogenic mechanisms of intrauterine renal venous thrombosis are made.

Six cases of renal venous thrombosis in infants of diabetic mothers, out of a series of 61 infants under two months old affected of R.V.T. (9.8%), are reported. The clinical, metabolic and histopathological features of these cases are discussed, as well as the different etiological theories that exist in the literature. An etiopathogenic hypothesis that maintains a similarity with the theory that explains the origin of R.V.T. in classical cases, is suggested. Prophilactic attitudes in order to prevent R.V.T. by a control of diabetes in the pregnant and close control of the newborn in the 10 first days of life, are pointed out.