Epithelioid sarcoma is an uncommon mesenchymal malignancy which represents less than 1% of all sarcomas. Rarer still are reports of this tumor initially presenting in the vulva. We report a case of vulvar proximal-type epithelioid sarcoma.

A 52-year-old had a 5-month history of slowly growing papule on the right labia majora. Excision of the mass revealed a tumor composed of large polygonal cells with abundant eosinophilic cytoplasm. An immunohistochemistry panel revealed cytokeratin AE1/AE3 positivity only. She underwent radical vulvectomy with bilateral groin node dissection. The specimen revealed a cream tan, firm, fairly defined mass at the right vulva. Microscopic examination showed a sheet-like growth pattern of large pleomorphic epithelioid cells with large vesicular nuclei and prominent nucleoli. The tumor showed loss of INI1 nuclear expression and absence of CD34 staining. EMA was positive. The case was signed out as proximal-type epithelioid sarcoma of the right vulva. Two months post-operatively, the patient was given concurrent chemotherapy with 5 cycles of cisplatin 40 mg/m2 and 6600 centigray vulvar intensity-modulated radiotherapy. She had no evidence of disease for five months until repeat workup showed tumor recurrence in the perineum. She was subsequently given 6 cycles of gemcitabine 900 mg/m2 and gemcitabine 900 mg/m2 with docetaxel 100 mg/m2. Two months after, repeat workup showed persistent progressive disease in the vulva. She was subsequently given 4 cycles of doxorubicin 60 mg/m2 and is for repeat workup.

The immunohistomorphologic features of this tumor, in addition to its unusual location, present a diagnostic challenge. Clues to the diagnosis include an initial presentation as a soft tissue mass and microscopic features showing the presence of epithelioid to spindle cytomorphology with an infiltrative growth pattern. Immunohistochemistry studies revealing the loss of INI1 nuclear expression and expression of epithelial markers would ultimately establish the diagnosis of this rare clinical entity.

Human ; Female ; Middle Aged: 45-64 Yrs Old ; Female Urogenital Diseases ; Vulvar Neoplasms ; Epithelioid Sarcoma

: Epithelioid sarcoma is an uncommon mesenchymal malignancy which represents less than 1% of all sarcomas. Rarer still are reports of this tumor initially presenting in the vulva. We report a case of vulvar proximal-type epithelioid sarcoma. : A 52-year-old had a 5-month history of slowly growing papule on the right labia majora. Excision of the mass revealed a tumor composed of large polygonal cells with abundant eosinophilic cytoplasm. An immunohistochemistry panel revealed cytokeratin AE1/AE3 positivity only. She underwent radical vulvectomy with bilateral groin node dissection. The specimen revealed a cream tan, firm, fairly defined mass at the right vulva. Microscopic examination showed a sheet-like growth pattern of large pleomorphic epithelioid cells with large vesicular nuclei and prominent nucleoli. The tumor showed loss of INI1 nuclear expression and absence of CD34 staining. EMA was positive. The case was signed out as proximal-type epithelioid sarcoma of the right vulva. Two months post-operatively, the patient was given concurrent chemotherapy with 5 cycles of cisplatin 40 mg/m2 and 6600 centigray vulvar intensity-modulated radiotherapy. She had no evidence of disease for five months until repeat workup showed tumor recurrence in the perineum. She was subsequently given 6 cycles of gemcitabine 900 mg/m2 and gemcitabine 900 mg/m2 with docetaxel 100 mg/m2. Two months after, repeat workup showed persistent progressive disease in the vulva. She was subsequently given 4 cycles of doxorubicin 60 mg/m2 and is for repeat workup. The immunohistomorphologic features of this tumor, in addition to its unusual location, present a diagnostic challenge. Clues to the diagnosis include an initial presentation as a soft tissue mass and microscopic features showing the presence of epithelioid to spindle cytomorphology with an infiltrative growth pattern. Immunohistochemistry studies revealing the loss of INI1 nuclear expression and expression of epithelial markers would ultimately establish the diagnosis of this rare clinical entity.
epithelioid sarcoma ; vulvar neoplasms ; female urogenital diseases

Population analyses and demographic data have clearly documented the rapid growth of the aging population in recent years. Although the population over 65 currently accounts for approximately 13.2% of the total population in Korea, it is estimated that this number will reach at least 40% by 2060. Aging is associated with a general decline in almost every physiological function of biological systems, including the lower urinary tract. Aging is also known to put individuals at a high risk for urinary tract infections. Geriatric hospitals in Korea have undergone a large expansion in terms of the number of beds and institutions. Many elderly patients in geriatric hospitals suffer from various genitourinary disorders. In particular, voiding dysfunction and urinary tract infections are prevalent comorbidities of other chronic geriatric diseases in patients in geriatric hospitals. Careful monitoring and management can help to reduce those genitourinary problems in elderly patients in geriatric hospitals.
Aged* ; Aging ; Comorbidity ; Female Urogenital Diseases ; Humans ; Korea ; Male Urogenital Diseases ; Urinary Tract ; Urinary Tract Infections

OBJECTIVETo summarize the features and treatment of male infertility induced by autosomal dominant polycystic kidney disease (ADPKD), and compare the outcomes of intracytoplasmic sperm injection (ICSI) for infertile men with ADPKD and those with congenital bilateral absence of vas deferens (CBAVD).

METHODSWe retrospectively analyzed 21 cases of ADPKD-induced infertility, 15 treated by ICSI (group A), and another 164 cases of strictly matched CBAVD-induced infertility (group B). We compared the two groups in the couples' age, the number of ICSI oocytes, and the rates of fertilization, transferrable embryos, good embryos, embryos implanted, clinical pregnancy, biochemical pregnancy, early abortion, singleton and twins in the first cycle.

RESULTSAfter 28 cycles of ICSI, 10 of the 15 ADPKD-induced infertility patients achieved clinical pregnancy, including 7 cases of live birth, 1 case of spontaneous abortion, and 2 cases of pregnancy maintenance. No significant differences were observed between groups A and B in the couples' age, the wives' BMI, or the numbers of ICSI oocytes and embryos transplanted (P >0.05), nor in the rates of ICSI fertilization (72.64% vs 76.17%), transferrable embryos (51.28% vs 63.24%), quality embryos (38.46% vs 49.83%), embryo implantation (17.64% vs 38.50%), abortion (0 vs 9.23%), singleton (50% vs 81.54%) and twins (50% vs 18.46%). However, the rates of clinical pregnancy (13.33% vs 42.68%, P = 0.023 <0.05) and biochemical pregnancy (13.33% vs 39.63%, P = 0.032 <0.05) were significantly lower in group A than in B.

CONCLUSIONICSI is effective in the treatment of male infertility induced by either ADPKD or CBAVD, but the ADPKD cases have a lower success rate than the CBAVD cases in an individual cycle. The affected couples should be informed of the necessity of prenatal genetic diagnosis before embryo implantation and the inevitable vertical transmission of genetic problems to the offspring.

Abortion, Spontaneous ; Embryo Implantation ; Embryo Transfer ; Female ; Humans ; Infertility, Male ; therapy ; Male ; Male Urogenital Diseases ; therapy ; Oocytes ; Polycystic Kidney, Autosomal Dominant ; complications ; Pregnancy ; Retrospective Studies ; Sperm Injections, Intracytoplasmic ; Vas Deferens ; abnormalities

Congenital absence of the uterine cervix and vagina in the presence of a functional endometrium is an extremely rare congenital anomaly. Women born with this anomaly present with collection of blood in the uterine cavity or hematometra, disabling pelvic pain and progressively worsening endometriosis. Presented is a case of a 16 year-old girl with severe pelvic endometriosis and hematometra complicated by cervicovaginal agenesis. She was managed by total abdominal hysterectomy with bilateral salpingectomy, left oophorocystectomyandadhesiolysis. Surgical management of congenital cervicovaginal agenesis remains controversial. The decision to do a conservative surgical procedure or a hysterectomy depends on the clinical profile of the patient, the expertise of the surgeon, the extent of the malformation and its association with other mullerian anomalies.

Human ; Female ; Adolescent ; abnormalities ; Endometriosis ; Female Urogenital Diseases and Pregnancy Complications ; Hematometra

OBJECTIVETo investigate the influence of Ureaplasma urealyticum (Uu) infection in the male reproductive tract on the outcomes of IVF and the clinical significance of preoperative Uu test by analyzing the correlation between the results of Uu culture before IVF-ET and the outcomes of IVF-ET.

METHODSAmong 1,059 couples undergoing IVF-ET, we selected 973 after excluding genetic factors and divided them into a Uu negative and a Uu positive group according to the results of culture of Uu in the semen of the males. We compared the rates of IVF fertilization, oocyte cleavage, clinical pregnancy and abortion between the two groups, and analyzed the influence of Uu infection on IVF outcomes.

RESULTSAmong the 973 selected subjects, 836 were Uu negative (group A) and 137 Uu positive (group B), and of the latter, 130 were restored to Uu negative after treatment (group B1) and the other 7 remained unchanged (group B2). No significant differences were found between groups A and B in the rates of IVF fertilization (81.6% vs 79.8%, P = 0.13), abnormal fertilization (11.8% vs 12.4%, P = 0.58) and oocyte cleavage (92.0% vs 92.1%, P = 0.94), nor between groups A and B2 (81.6% vs 89.8%, P = 0.10; 11.8% vs 13.2%, P = 0.75; 92.0% vs 92.5%, P = 0.10). Totally, 747 of the patients underwent embryo transfer, including 643 in group A and 104 in group B. There were no significant differences between groups A and B in the rates of clinical pregnancy (38.6% vs 34.7%, P = 0.44) and abortion (16.5% vs 22.2%, P = 0.39), nor between groups A and B2 (38.6% vs 33.3%, P = 0.79; 16.5% vs 0, P = 0.53).

CONCLUSIONUu infection in the male reproductive tract does not significantly affect the rates of IVF fertilization, oocyte cleavage, clinical pregnancy and abortion. However, more investigations with larger sample sizes of the cases restored from Uu positive to Uu negative are needed to lend further support to our findings.

Adult ; Embryo Transfer ; Female ; Fertilization in Vitro ; Genitalia, Male ; microbiology ; Humans ; Infertility, Male ; microbiology ; therapy ; Male ; Male Urogenital Diseases ; epidemiology ; microbiology ; Middle Aged ; Pregnancy ; Pregnancy Rate ; Ureaplasma Infections ; epidemiology ; Ureaplasma urealyticum

Herlyn-Werner-Wunderlich syndrome (HWWS) is a very rare congenital anomaly of the urogenital tract involving Mullerian ducts and Wolffian ducts, and is characterized by the triad of uterine didelphys, unilateral obstructed hemivagina and ipsilateral renal agenesis. Generally it is diagnosed at puberty after menarche due to recurrent pelvic pain or abdominal mass. We report 2 cases of female newborns whose fetal ultrasonography (USG) showed unilateral renal agenesis and were diagnosed with HWWS by postnatal evaluation. Both cases were female newborns who were born at term by vaginal delivery. They had no perinatal problems except suspicious findings of unilateral renal agenesis from fetal USG. Abdominal ultrasonography and pelvic MRI were performed after birth, and they were diagnosed with HWWS. The potential complications of this syndrome such as pyosalpinx, pelvic adhesions and increased risk of abortion or infertility can occur, but without complication, the prognosis is very good with simple surgical treatment. If renal agenesis is detected in a fetus or a newborn, possible anomalies of urogenital tract such as HWWS should be considered; and postnatal evaluation should be performed, as a simple surgical treatment before menarche can prevent unnecessary complications of disease.
Congenital Abnormalities ; Female ; Fetus ; Humans ; Infant, Newborn ; Infertility ; Kidney ; Kidney Diseases ; Menarche ; Mullerian Ducts ; Parturition ; Pelvic Pain ; Prognosis ; Puberty ; Ultrasonography, Prenatal ; Urogenital Abnormalities ; Wolffian Ducts

Human ; Female ; Adult ; Female urogenital diseases ; radiography ; tomography scanners ; signs and symptoms ; diagnostic imaging

Introduction. Recurrent pregnancy loss is a devastating reproductive problem that affects 5% of couples trying to conceive. Majority of the cases are due to cytogenetic errors. This study determines the prevalence of chromosomal structural abnormalities in Filipino couples who presented with 2 or more pregnancy losses. Methods. Results from chromosomal analysis of couples referred for 2 or more miscarriages done at the Institute of Human Genetics-National Institutes of Health-University of the Philippines, Manila on peripheral blood samples from 1991 to 2010 were restrospectively reviewed. Results. There were 356 couples with a history of 2 or more miscarriages sent for chromosomal analysis from 1991-2010 included in this study. Among these 356 couples, 17 couples (4.8%) were found to be carriers of different chromosomal abnormalies. From a total of 18 cases, there were 13(3.6%) translocations, 1(0.3%) insertion, 2(0.6%) with marker chromosomes, 1(0.3%) pericentric inversion and 1(0.3%) deletion. Conclusion. The overall frequency of chromosomal structural abnormalities among patients with RPL in this study is 4.8% with translocations being the most common type detected. The results of this study are similar to that of previous large-scale studies which have demostrated that parental chromosomal abnormalities are associated with RPL.
Male ; Female ; RECURRENCE ; PREGNANCY ; CHROMOSOME ABERRATIONS ; ABORTION, SPONTANEOUS ; FEMALE UROGENITAL DISEASES AND PREGNANCY COMPLICATIONS ; PREGNANCY COMPLICATIONS ;

Gemella morbillorum is a normal flora of the oral cavity, upper respiratory tract, gastrointestinal tract, or genitourinary system. Human infection cause by G. morbillorum is very rare. Known predisposing conditions are intravenous drug abuse, alcoholism, cardiovascular disease, chronic obstructive pulmonary disease, diabetes, renal disease and poor oral hygiene. Most reported cases are infective endocarditis and vasculitis. We experienced a case of liver abscess by G. morbillorum in a healthy young woman, who was treated successfully with antibiotics and drainage. We report this case along with a literature review.
Abscess ; Alcoholism ; Anti-Bacterial Agents ; Cardiovascular Diseases ; Drainage ; Endocarditis ; Female ; Gastrointestinal Tract ; Gemella ; Humans ; Liver ; Liver Abscess ; Mouth ; Oral Hygiene ; Pulmonary Disease, Chronic Obstructive ; Respiratory System ; Substance Abuse, Intravenous ; Urogenital System ; Vasculitis