Intraabdominal desmopl small round cell tumor is a rare neoplasm predominant in adolescent or young adult male. The tumor is characterized by widespread growht along the peritoneal surfaces of the abdomen and pelvis,typical histologic features, and a specific immunohistochemical staining pattern. We report the clinical,radiologic, and pathologc findings of intraabdominal desmoplastic small round cell tumor.
Abdomen ; Adolescent ; Desmoplastic Small Round Cell Tumor* ; Humans ; Male ; Young Adult

No abstract available.
Bowen's Disease* ; Carcinoma, Merkel Cell*

Chromosomally derived sterility has long been recognized. A review of the literature of somatic chromosome investigations in infertile males has shown that 13.7% of azoospermic males and 4.6% of oligozoospermic males have an abnormal karyotype. In the first group, sex chromosome abnormalities predominate (mainly 47,XXY), whereas in the latter, autosome anomalies (i.e. Robertsonian and reciprocal translocations) are the most frequent. A similar review on meiotic studies revealed that meiotic chromosome anomalies can explain male infertility in 4.3-40.4% of patients. Recently, fluorescent in-situ hybridization studies on spermatozoa from infertile men were published; it was suggested that both X-Y pairing and pairing of the autosomes were impaired, resulting in spermatogenic disruption. It is estimated that there are 2,000 genes that regulate spermatogenesis, most of these being present on the autosomes, but there are approximately 30 genes on the Y chromosome. In general, autosomal genes that regulate spermatogenesis are concerned with regulation of metabolic processes in other cells in the body as well as in the cells of spermatogenesis, whereas Y genes are not essential for vital functions related to reproduction. To be able to provide proper counseling for those couples whose male infertility can now be treated by intracytoplasmic sperm injection, it is suggested that clinical investigations should include mitotic and meiotic studies, an analysis of the chromosome content of individual spermatozoa and a DNA analysis of blood and spermatozoa to detect partially deleted Y chromosome material. We have experienced a case of azoospermia associated with inversion of chromosome 1. So we report this case with a brief review of literatures.
Abnormal Karyotype ; Azoospermia* ; Chromosomes, Human, Pair 1* ; Counseling ; DNA ; Family Characteristics ; Humans ; Infertility ; Infertility, Male ; Male ; Metabolism ; Reproduction ; Sex Chromosome Aberrations ; Sperm Injections, Intracytoplasmic ; Spermatogenesis ; Spermatozoa ; Y Chromosome

A balanced translocation in a parent may produce unbalanced gametes leading to abortions or defective liveborn children, or interval infertility. It also may give rise to a balanced gamete resulting in a balanced carrier, or it may produce a cytogenetically normal gamete. The incidence of balanced chromosomal translocations in couples with multiple abortions was reported as 0% to 31%. This wide variation is related to the heterogeneous criteria used for patient selection. Because parents with balanced chromosomal rearrangements and history of only repeated abortions have a significant chance with each pregnancy of having a child with normal or balanced karyotype, the usual criteria for investigation include at least two abortions or reproductive losses. There is no evidence from several reported series that increasing the number of losses to three or more leads to any change in the yield of chromosomal rearrangements detected.
Abortion, Spontaneous* ; Child ; Family Characteristics ; Female ; Germ Cells ; Humans ; Incidence ; Infertility ; Karyotype ; Parents ; Patient Selection ; Pregnancy ; Translocation, Genetic

Pulsatile secretion of GnRH from the hypothalamus is a prerequisite for both the initiation and maintenance of the reproductive axis in humans. Failure of this episodic GnRH secretion results in the clinical syndrome of hypogonadotropic hypogonadism. Deficient GnRH secretion may occur in isolation (idiopathic hypogonadotropic hypogonadism: IHH), in association with anosmia (Kallmann syndrome), or as a result of a variety of structural and functional lesions of the hypothalamic pituitary axis. The familial occurrence of hypogonadotropic hypogonadism associated with anosmia, color blindness, synkinesia, and mental defect is the classic Kallmann syndrome. Affected individuals respond readily to pulsatile administration of exogenous GnRH, and clearly this is the most physiologic approach to ovulation induction. For women not seeking pregnancy, replacement therapy with exogenous estrogen and progestin is indicated. We have experienced a case of Kallmann syndrome which was conceived by administration of gonadotropin. So we report this case with a brief review of literatures.
Axis, Cervical Vertebra ; Color Vision Defects ; Estrogens ; Female ; Gonadotropin-Releasing Hormone ; Gonadotropins* ; Humans ; Hypogonadism ; Hypothalamus ; Kallmann Syndrome* ; Olfaction Disorders ; Ovulation Induction ; Pregnancy

Recent advances in the understanding of the biology of Hodgkin lymphoma have led to a new classification. Hodgkin lymphoma is now recognized as a B-cell disorder of germinal center or post-germinal center origin. In the WHO classification, Hodgkin lymphoma consists of two categories, namely, nodular lymphocyte predominant Hodgkin lymphoma and classical Hodgkin lymphoma. Classical Hodgkin lymphoma encompasses not only nodular sclerosis, mixed cellularity, and lymphocyte depletion subtypes, but also lymphocyte-rich subtype, among which, nodular sclerosis stands out as a distinct entity. A borderline neoplasm with features intermediate between Hodgkin lymphoma and diffuse large B-cell lymphoma has also been recognized.
B-Lymphocytes ; Biology ; Diagnosis, Differential ; Germinal Center ; Hodgkin Disease ; Lymphocyte Depletion ; Lymphocytes ; Lymphoma, B-Cell ; Sclerosis

No abstract available.
Thanatophoric Dysplasia*

Background: Implant-based breast reconstruction is a technique frequently used for breast reconstruction. Infection and inflammation are considered to be the most troublesome complications of implant-based breast reconstruction and can lead to capsular contracture or implant failure. To date, however, only a few methods have been proposed to prevent these complications. Therefore, the authors introduce a simple irrigation system using indwelling drain catheters to decrease postoperative inflammation. Methods: Continuous saline irrigation was performed once per day for 3 days immediately after prosthesis-based breast reconstruction. Normal saline (500 mL) was inserted into the implant pocket through a superomedial-oriented drain catheter and drained through an inferolateral-oriented drain catheter using a suction device. Inflammatory indicators, including C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), white blood cell count (WBC), and postoperative complications were compared between the non-irrigation and irrigation groups. Results: This study included 37 patients divided into two groups (20 non-irrigation and 17 irrigation). An analysis of inflammatory indicators revealed that the peak CRP level in the irrigation group was significantly lower than that in the non-irrigation group, while no statistically significant differences were found for the other mediators (ESR and WBC). In the immediate postoperative period, continuous irrigation effectively washed out tissue debris and blood clots within the implant pocket, which helped maintain the function of the drain catheter and decrease pro-inflammatory mediators. Conclusions This irrigation method was simple and cost-effective for decreasing inflammation within the breast pocket. Furthermore, it can also be applied to infected breast pockets in combination with antibiotics.

Primary actinomycosis of the urachal remnants is rare disease, and was not reported in Korean literature. Wereport the fistulography and CT findings of a cases of actinomycosis in the urachal sinus in a 31 year-old man.
Actinomycosis* ; Adult ; Humans ; Rare Diseases ; Urachus

We describe here a 67-year-old man who was diagnosed with a rare case of intraductal papillary mucinous tumors that occurred simultaneously in the liver and pancreas. Abdominal computed tomography showed a tubular and cystic dilatation of the pancreatic duct in the pancreas tail, which suggested an intraductal papillary mucinous tumor (IPMT), and multiple intrahepatic duct stones. The patient underwent a distal pancreatectomy with splenectomy and a lateral segmentectomy of the liver. Microscopic examination showed an intraductal papillary mucinous neoplasms of borderline malignancy in the pancreas and a non-invasive intraductal papillary mucinous tumor with moderate dysplasia of the bile duct. Although several cases of intraductal papillary mucinous neoplasm of the liver (IPNL) without any pancreatic association have been described, the simultaneous presentation of both IPMT of the pancreas and IPNL is very rare. The patient has been doing well for 10 months postoperatively.
Aged ; Bile Ducts ; Dilatation ; Humans ; Liver ; Mastectomy, Segmental ; Mucins ; Pancreas ; Pancreatectomy ; Pancreatic Ducts ; Splenectomy