• Investigative Magnetic Resonance Imaging
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• v.19 no.3
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• pp.196-199
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• 2015

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is an uncommon congenital abnormality of the female urogenital tract characterized by the triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. A 13-year-old female presented with acute lower abdominal pain. Magnetic resonance imaging (MRI) revealed uterine didelphys, hematometrocolpos, obstructed hemivagina, and right ipsilateral agenesis, consistent with OHVIRA syndrome. Also, a well-defined mass with fluid signal intensity, mimicking adnexal neoplasm was seen in the right lower pelvic cavity adjacent to the posterior wall of the bladder. Vaginal septotomy and drainage of hematometrocolpos were done initially, but unilateral hysterectomy was later performed to relieve the patient's symptoms. The cystic mass in the right lower pelvic cavity was also excised and confirmed as a blind megaureter.

• Childhood Kidney Diseases
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• v.23 no.2
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• pp.124-127
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• 2019

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital anomaly of the genitourinary tract comprising uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Patients with HWW syndrome usually present symptoms such as dysmenorrhea, abdominal pain, pelvic mass, and purulent vaginal discharge. If not treated at an appropriate time, complications such as infertility, endometriosis, pyosalpinx, and subsequent pelvic adhesions may occur. Here, we report a case of HWW syndrome in a 7-year-old-girl who was also diagnosed as having central precocious puberty. She was brought to the pediatric department with chief complaints of lump in her breast and vaginal discharge. When she was around 2 months old, she was confirmed to have a single kidney on ultrasonography. We checked her past medical history and diagnosed her as having HWW syndrome based on the results of imaging studies, including abdominal ultrasonography and pelvic magnetic resonance imaging. She underwent treatment with gonadotropin-releasing hormone analogue for 2 years. During 24 months of follow-up, she showed no serious problems or complications. If renal anomalies are identified immediately after birth or in infancy, further screening tests should be conducted prior to menstruation for determining congenital abnormalities of the reproductive tract and vice versa.

• Investigative Magnetic Resonance Imaging
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• v.21 no.2
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• pp.119-124
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• 2017

Chromophobe renal cell carcinoma (RCC) is an uncommon subtype of RCC having a better prognosis than clear cell RCC. Although there are several reports of seeding metastasis of RCC after biopsy, seeding metastasis of chromophobe RCC after surgical resection has seldom been reported. Here, we describe a case of multiple seeding metastases in the abdomen and pelvis 78 months after robot-assisted laparoscopic partial nephrectomy, without prior history of biopsy for chromophobe RCC in the right kidney. As magnetic resonance imaging (MRI) of the pelvic mass showed a similar appearance to the primary renal mass and displayed separate margins with the rectum and prostate gland, we were able to make a diagnosis before pathologic confirmation.

• Advances in pediatric surgery
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• v.10 no.1
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• pp.52-55
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• 2004

A newborn male baby was transferred to our hospital with a left inguinal mass. The mass was huge measuring $10{\times}10cm$, engorged, and dark-blue colored as a result of internal hemorrhage. Unstable vital signs were combined with DIC and acute renal failure. Emergency operation was performed because of the suspicion of bowel perforation. The peritoneal cavity was full of ascitis and the distal jejunum had a 0.5 cm perforation. Segmental resection of the jejunum and incisional biopsy of the inguinal mass were performed. On pelvic and thigh MRI, the mass protruded into pelvic cavity and encircled large vessels and nerves of the thigh. Pathologic diagnosis was congenital infantile fibrosarcoma. Fifteen days after operation, primary tumor excision was undertaken. The second look operation, performed after 6 times VAC chemotherapy, revealed no remained malignant cell on microscopic section. The baby has been followed closely for the last eight months.

• Archives of Craniofacial Surgery
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• v.20 no.6
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• pp.392-396
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• 2019

Renal cell carcinoma (RCC) represents 2% to 3% of human cancers and is aggressive, with metastatic capability. The frequent metastatic sites are lung, bone, and liver. Reports of RCC metastatic to skin, and especially scalp are rare. Here we present an 83-year-old woman who was diagnosed with RCC 19 years prior and had a metastatic scalp lesion. An 83-year-old woman presented with a red-to-purple, protruding lesion at the right parietotemporal area. Twenty-three years ago, a right renal mass was incidentally discovered on ultrasound through a routine medical examination. She underwent right nephrectomy for RCC 4 years later. Five months after nephrectomy, new lung nodules were observed. Fifteen years after nephrectomy, metastatic lesions were found in the pelvic bone. She visited dermatology department for evaluation of the new scalp lesion, a year before she first visited our department. Despite chemotherapy, the mass was gradually enlarged. She consulted the plastic surgery department for management of the metastatic RCC was successfully treated with total excision including a 1-cm safety margin, local flap, and STSG coverage. Complete healing was observed, without evidence of recurrence during a 7-month followup. Metastases to the skin are rare, but must be kept in mind because of its high metastatic ability and poor prognosis.

• Childhood Kidney Diseases
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• v.22 no.1
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• pp.12-16
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• 2018

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital malformation syndrome that is characterized by a triad of uterine didelphys, blind hemivagina, and ipsilateral renal agenesis. There is a wide variety of phenotypic presentation which is recognized as a spectrum of disease rather than a separate entity. The exact incidence and pathogenesis of HWW syndrome are yet to be investigated. While this disease typically involves adolescent girls who present with abdominal pain or a pelvic mass that is secondary to hematocolpos, nowadays, a majority of potential patients with HWW are being prenatally screened for renal anomalies. Therefore, it is recommended to search for uterovaginal anomalies whenever a multicystic dysplastic kidney or the absence of a kidney is noted in a newborn female, and the role of pediatric nephrologists has become ever more important for early recognition of the disease.

• Childhood Kidney Diseases
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• v.11 no.2
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• pp.299-305
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• 2007

Herlyn-Werner-Wunderlich syndrome(HWWs) is a rare variant of Mullerian ductal anomalies characterized by the presence of a hemivaginal septum, a didelphic uterus, and ipsilateral renal agenesis. It usually presents after menarche with progressive pelvic pain, and palpable mass due to hemihematocolpos. If a cystic mass is detected behind the urinary bladder in children, in association with the absence of a kidney, the diagnosis of uterus didelphys with imperforate vagina and hydrocolpos should be considered. When renal agenesis is found in asymptomatic children, the small size and the tubular shape of the uterus makes it almost impossible to evaluate uterine anomalies, so follow-up should be performed until the end of puberty. Appropriate preoperative diagnosis and treatment will prevent unnecessary procedures and offer relief of symptoms. We report one case of didelphic uterus with blind hemivagina and ipsilateral renal agenesis with biopsy- proven thin glomerular basement membrane disease which is not related to the above syndrome.

• Journal of Medicine and Life Science
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• v.20 no.2
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• pp.94-98
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• 2023

This report presents the case of 75-year-old men with spindle cell neoplasm. The patient underwent percutaneous nephrolithotomy and transurethral resection of the prostate (TURP) for renal stones and benign prostatic hyperplasia. One month postoperatively, the patient was able to void without any difficulty. Five months later, the patient experienced difficulty voiding and presented to the emergency room with severe pelvic pain. Computed tomography (CT) showed regrowth of the prostate mass into the posterior bladder and penile root. The prostate-specific antigen level remained constant at 1.14 ng/mL during the pre-and postoperative periods. Five months before the TURP operation, the patient's CT scan showed a soft and mildly enlarged prostate with no protrusion into the bladder. Biopsy of the prostate, however, showed a protruding mass, indicative of a spindle cell neoplasm. The patient was subsequently treated with the chemotherapeutic drug adriamycin. Unfortunately, treatment was unsuccessful, and the patient died 18 months later.

• Journal of Medicine and Life Science
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• v.19 no.3
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• pp.116-120
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• 2022

Objective: To estimate the efficacy of sequential treatment of bladder endometriosis (BE) of the vesicoureteric junction using transurethral resection (TUR) and hormonal therapy. Design: Case report. Setting: Private multispecialty hospital. Patient: A multiparous woman presented with perimenstrual lower urinary tract symptoms, cyclic chronic pelvic pain, and left loin pain. Intervention[s]: Ultrasonography revealed marked left renal dilatation. Computed tomography confirmed the presence of a bladder mass. A diagnostic cystoscopy revealed compression of the left vesicoureteral junction. Complete TUR BE with release of chocolate material during resection, followed by ureteric double J stent insertion for 3 months, was performed. Histopathology confirmed the diagnosis of BE, followed by adjuvant hormonal therapy (dienogest) for 3 months. Follow-up for about 2 years revealed complete relief of the symptoms without any recurrence. Main Outcome Measure[s]. Success and recurrence rates of sequential TUR and hormonal therapy of BE of the vesicoureteric junction. Result[s]. TUR BE followed by adjuvant hormonal therapy was very effective in eradicating BE of the vesicoureteric junction in a safe manner without recurrence on follow-up for 2 years. Conclusion[s]. BE of the vesicoureteric junction can be properly treated by sequential TUR and hormonal therapy without recurrence over a 2-year follow-up.