Brain metastasis from extramammary Paget's disease.

Herein, we present a case of extramammary Paget's disease with brain metastasis that was diagnosed pathologically for the first time in Japan. Moreover, invasive extramammary Paget's disease (with distant metastasis) highly resistant to treatment. Only for brain metastasis, we may control the tumor by surgical resection and stereotactic radiosurgery (SRT) for the treatment of intracranial metastases was assessed. An 76-year-old man was diagnosed with extramammary Paget's disease of the vulva at nearby hospital. Surgical resection and sentinel lymph node dissection were then performed, and the patient received chemotherapy because multiple lymph node metastases were suspected. The patient's response to chemotherapy was poor, and he was in the state of Progressive Disease. He complained of dyslexia and was referred to another hospital when he was 81 years old. Plain magnetic resonance imaging (MRI) was conducted, and two brain tumors in the vicinity of the left cerebellar tent were suspected. In our hospital, gadolinium contrast-enhanced MRI was performed and showed a tumor in the cerebellum (left posterior temporal lobe) and another tumor under the tent (left cerebellar hemisphere). Significant edema was also noted. Based on these findings, the intracranial lesion was diagnosed as metastatic brain tumor. The pathological diagnosis was brain metastasis from extramammary Paget's disease. Postoperative intracranial residual disease was treated with stereotactic radiosurgery. MRI showed that the size of the cerebellar lesions decreased, and no recurrence of cerebral lesions was observed. SRT for extracranial lymph node metastases was performed. Mass reduction and SRT may be the best way to treat brain metastasis from extramammary Paget's disease.

Spermatic cord dedifferentiated liposarcoma presenting as a recurrent inguinal hernia.

Paratesticular sarcomas are a rare entity and provide a unique clinical challenge due to their slow growing, often painless natural course. Adding to this challenge is the complex anatomy of the scrotum that allows these masses to mimic other conditions, including inguinal hernia, cysts, or fluid collections. We report such a case and our approach to an 83-year-old male with dedifferentiated liposarcoma of the spermatic cord with a history of inguinal hernia. In doing so, we highlight the need for thorough evaluation of scrotal masses and the management of these rare, though well-described, tumors.

Long-term outcomes and patient satisfaction of continent catheterizable limb and augmentation cystoplasty simultaneous with salvage prostatectomy.

AIMS: We evaluated the long-term outcomes of augmentation cystoplasty and continent catheterizable limb formation in patients who underwent simultaneous salvage prostatectomy. METHODS: Twelve men who underwent salvage prostatectomy with augmentation cystoplasty and either an appendicovesicostomy or Monti ileovesicostomy between October 2000 and February 2003 were assessed for long-term surgical complications, reoperations, continence rates, and patient satisfaction using the Incontinence Symptom Index (ISI) questionnaire and a self-designed catheterization questionnaire. RESULTS: In addition to bladder augmentation, six patients underwent appendicovesicostomy, four had a Monti ileovesicostomy and two a spiral Monti ileovesicostomy. There were no intraoperative complications or surgical-related deaths. Four patients required revision surgery (33%) at a mean of 39 months. Minor revisions were performed for stomal stenosis in two patients with stomal relocation in two patients. Ten of 12 patients were dry (83.3%) with a catheterization frequency of between 3 and 8 hr. At a mean follow-up of 61 months (SD = 20.51), the mean ISI severity score was 1.86 (SD = 3.54) and the mean ISI bother score was 1 (SD 0.74). The majority (86%) would choose to undergo the surgery again. CONCLUSIONS: Although surgically challenging and associated with significant morbidity, salvage prostatectomy with concomitant bladder augmentation and continent catheterizable reconstruction is a feasible and effective means of obtaining adequate long-term urinary continence, while preserving the native bladder. In comparison to similar historical patients with catheterizable limbs only, fewer of the augmented patients needed antimuscarinic medication and delayed augmentation was not necessary. Complications do not increase over time and the continence rates are stable.

Phenotypic heterogeneity of mutations in androgen receptor gene.

Androgen receptor (AR) gene has been extensively studied in diverse clinical conditions. In addition to the point mutations, trinucleotide repeat (CAG and GGN) length polymorphisms have been an additional subject of interest and controversy among geneticists. The polymorphic variations in triplet repeats have been associated with a number of disorders, but at the same time contradictory findings have also been reported. Further, studies on the same disorder in different populations have generated different results. Therefore, combined analysis or review of the published studies has been of much value to extract information on the significance of variations in the gene in various clinical conditions. AR genetics has been reviewed extensively but until now review articles have focused on individual clinical categories such as androgen insensitivity, male infertility, prostate cancer, and so on. We have made the first effort to review most the aspects of AR genetics. The impact of androgens in various disorders and polymorphic variations in the AR gene is the main focus of this review. Additionally, the correlations observed in various studies have been discussed in the light of in vitro evidences available for the effect of AR gene variations on the action of androgens.

Sexual health issues in men with cancer.

While the cancer patient may be affected by sexual dysfunction throughout the entire course of the disease, sexual health is largely underevaluated and undertreated. Sexual problems should be anticipated and patients should be actively screened as they are unlikely to initiate discussion on sexual issues. Cancer-related sexual dysfunction may involve several components, and an understanding of the underlying etiologies is essential to tailoring the appropriate treatment to the individual patient. This article reviews the numerous factors potentially involved in male sexual dysfunction associated with a variety of cancers.

Multiple spreading epidermolytic acanthomas of the genital and perigenital skin.

Epidermolytic acanthoma is an uncommon benign tumour mainly characterized histologically by a prominent epidermolytic degeneration of the keratinocytes of the upper layers of the stratum spinosum and of the stratum granulosum. The absence of desmosome involvement allows to differentiate this condition from others such as acantholytic acanthoma. We report the first case, to our knowledge, of a 54-year-old male patient exhibiting disseminated scrotal, gluteal, inguinal and perineal epidermolytic acanthomas.

Growth hormone-releasing hormone and pituitary adenylate cyclase-activating polypeptide in the reproductive system.

Growth hormone-releasing hormone (GHRH) and pituitary adenylate cyclase-activating polypeptide (PACAP) are both members of the glucagon superfamily that, with gonadotropins, act at central and peripheral levels as paracrine and autocrine coregulators of reproductive function. GHRH and PACAP are ancient peptides. Their original forms (both 27 amino acids long) were encoded by a single ancestral gene, several duplications of which led to the genes that encode the neuropeptides of the glucagon superfamily. In the male and female reproductive tracts, GHRH and PACAP interact with a subset of G protein-coupled receptors that are structurally similar to the PACAP receptor and variants of the vasoactive intestinal peptide receptor, and share several biological actions. These are related mainly to the modulation of cAMP-dependent and other signal transduction pathways in several cells of the pituitary-gonadal axis. The recent discovery that antagonists of GHRH and PACAP suppress the growth of human cancer cell lines that are derived from reproductive tissues indicates the potential importance of these peptides as local regulators of cell division, cell cycle arrest, differentiation and cell death.

Odynorgasmia.

Painful ejaculation ("odynorgasmia") is not well recognized. When it occurs it may indicate the precise site of pathology.

A role for hepatocyte growth factor/scatter factor in regulating normal and neoplastic cells of reproductive tissues.

OBJECTIVE: To review data regarding the expression, function, and signal transduction pathways associated with hepatocyte growth factor/scatter factor (HGF/SF) as related to normal and neoplastic cells of reproductive tissues. METHODS: Studies involving HGF/SF were reviewed and the data were summarized. RESULTS: Hepatocyte growth factor/scatter factor is generally expressed within the stromal components of the testis, prostate, placenta, mammary gland, uterine endometrium, and ovary. The receptor for HGF/SF, c-met, is expressed in the epithelial cells of these tissues. Ligand activation of c-met results in its autotyrosine phosphorylation and the subsequent stimulation of numerous signal transduction pathways. Which pathways are activated determine in part the effect that HGF/SF has on a specific tissue. Hepatocyte growth factor/scatter factor can promote a variety of biologic responses including mitosis, motogenesis (i.e., cell movement), differentiation, and apoptosis. In addition, both HGF/SF and c-met continue to be expressed during the neoplastic transformation of cells from these reproductive tissues. CONCLUSION: Hepatocyte growth factor/scatter factor plays an important role in regulating the physiologic function of several reproductive tissues and may also be involved in the oncogenesis of cells within these tissues.

Midline teratomas, mullerianosis, the multifariousness of gynaecological neoplasias, and the scrotum. Are they manifestations of a germ-soma barrier?

Empirical evidence and theoretical considerations suggest that there are mechanisms protecting the germ line from untoward somatic influences. In the intraorganismal competition between cell lineages, evolution will give priority for protection to the germ line, which carriers the heritable genes. In embryogenesis, germ cells migrate along the midline as this is an area where developmental influences are lower; exposure to somatic factors may cause inception of teratomas. In order not to hinder the germ line, the female genital tract has a reduced level of cell determination, which results in the multifariousness of gynaecological proliferations, including mullerianosis. The external location of testes reduces somatic constraints on spermatogenesis.

Rabdomiosarcoma embrionario: presentación de un caso y revisión de la literatura / Embrionic rhabdomyosarcoma: a case presentation and literature revision

Se trata de un caso de 14 años, quien consultó por dolor en región inguinoescrotal izquierda y aumento de volumen en ese hemiescroto, de 5 días de evolución, que se presentó posteriormente a la realización de un esfuerzo; concomitantemente náuseas, vómitos y ausencia de evacuaciones por 2 días

[Expression of cytokeratins during embryogenesis and in pathologic epithelia]. / Expression des cytokératines au cours de l'embryogénèse et dans les épithéliums pathologiques.

Epithelial cell intermediate filaments, or cytokeratins, are excellent markers for cell differentiation. During embryogenesis, cytokeratins specific of a stage of differentiation step always become detectable before corresponding morphologic changes: for instance, cytokeratins 5 and 14 are found around the eight week, shortly before stratification of the epithelium occurs, and cytokeratins 1 and 10 are produced before morphologic evidence of keratinization becomes detectable. Among potential diagnostic applications, analysis of cytokeratin patterns of epidermal cells desquamated in the amniotic fluid may provide earlier and less invasive diagnosis than fetoscopic biopsies. Similarly, a review of cytokeratins expressed in a variety of epithelial diseases (involving the epidermis, digestive tract, respiratory tract, urogenital tract, or breast) demonstrated persistence of the original tissue pattern in some instances (this was the case for the majority of simple epithelia) but not in others (complex epithelia). This suggests that cytokeratins may prove valuable as markers for specific tumor stages or types and may provide earlier information than morphologic studies.

Natural history and epidemiological features of genital HPV infection.

The spectrum of genital HPV infections comprises clinical, subclinical, and latent disease in addition to HPV-associated neoplasia. The definition of subclinical and latent HPV infection is still incomplete and awaits clarification by highly sensitive HPV detection systems that preserve the morphology of the tissue. Genital HPVs infect the human body mainly by sexual transmission, but other pathways of HPV transmission may be possible as suggested by (a) high prevalences of antibody reactivity in children; (b) lack of association of HPV seropositivity with sexual activity; (c) presence of HPV DNA in oral cavity scrapings of children and adults; and (d) development of recurrent respiratory papillomatosis among children exposed to HPV 6 or 11 during birth. Successful infection depends on the infection site and the immunological state of the host: susceptibility to genital HPVs seems highest for the squamous epithelium of the lower genital tract. HPV DNA is also present in extragenital sites but this is rarely accompanied by clinical or subclinical lesions. The molecular basis for this specific tropism is unknown. The immune response in HPV-infected tissues is characterized by depletion of T helper/inducer cells or Langerhans cells and an impaired immunological function of natural killer cells or the infected keratinocyte. Epidemiological studies indicate that individuals with cell-mediated immunodeficiencies are at increased risk for genital HPV infections. Data about the biological course of genital HPV infections are just beginning to emerge. Regression or persistence of subclinical and latent genital HPV infections as analysed in longitudinal investigations show a constant come-and-go of HPV presence. In an infected individual, complete clearing of the virus seems rather exceptional. With respect to progression, the biological potential of cervical HPV infections is characterized by an increased risk for development of HPV-associated neoplasia, especially in lesions infected with high-risk HPV types (e.g., HPV 16 and 18). Demographic data of genital HPV infections are very variable due to differences in the HPV detection assays used and in the populations examined; the prevalence of subclinical and latent genital HPV infections appears to be at least three times higher compared with clinical HPV infections. This rate increases by a further 3-5-fold when patients are examined several times. Seroreactivity against genital HPV types may be due to an active infection or the result of contact with HPV earlier in life.(ABSTRACT TRUNCATED AT 400 WORDS)

Sexuality and fertility in urologic cancer patients.

With the advent of effective treatment for urologic cancer, the preservation of sexual function and fertility has become an important goal. Some cancer treatments damage the physiological systems involved in reproduction. All have a psychological impact on sexuality. For men with prostate cancer, current issues in sexual rehabilitation include the debate on nerve-sparing radical prostatectomy, the role of vascular damage in causing erectile dysfunction after radiotherapy, and the need for a better understanding of hormonal effects on central and peripheral mechanisms of sexual function. In the treatment of men and women with bladder cancer, the sexual function morbidity of radical cystectomy is described in data from prospective interview studies. Sexual desire and orgasm remain normal after surgery despite disruption of the genital vasocongestion accompanying sexual arousal. Long-term follow-up studies of testicular cancer patients suggest that some increase in sexual dysfunction does occur. Infertility remains a concern for a subgroup of younger, childless men. Attempts to modify or eliminate retroperitoneal lymphadenectomy are discussed, as is recovery of spermatogenesis after chemotherapy and radiotherapy. Sexual function in patients with penile, urethral, or renal cell carcinoma is briefly reviewed.

Condyloma acuminatum in Rochester, Minn., 1950-1978. I. Epidemiology and clinical features.

From 1950 through 1978, condyloma acuminatum was diagnosed in 746 residents of Rochester, Minn (246 male and 500 female subjects). With the number of cases increasing almost every year until 1975, the mean annual incidence rate reached 106.5 per 100,000 population in the period 1975 through 1978, apparently the highest reported to date. Among age groups, the rate was highest in the 20- through 24-year-old group. Female patients contracted the infection at a higher rate and at a younger age than male patients. Anal condyloma occurred in one third of the male subjects studied and recurrent condyloma in 30% of the male subjects. More female than male patients had lesions at multiple locations.

Giant scrotal hemangioma with azoospermia.

We report a case of giant scrotal hemangioma with azoospermia, which was 37 cm in maximum circumference and 20 cm in length along the raphe. The biopsy specimens of the testes were examined histologically and cytologically. The testicular damage was believed to be due to the heat generated by hemangioma.