This article reports a patient who suffered from Wolffian adnexal tumor.We also briefly elucidate the pathogenesis,clinicopathological features,diagnosis,differentiation,and treatment of Wolffian adnexal tumor,with an attempt to increase the awareness of the disease and reduce misdiagnosis.
Adenoma ; Adnexal Diseases ; Female ; Humans ; Immunohistochemistry ; Wolffian Ducts

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare, congenital genitourinary anomaly involving the Müllerian and Wolffian structures, and is characterized by the triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. It usually presents in adolescent girls in whom hematometrocolpos produces a pronounced mass effect and pain on the side of the obstructed hemivagina. Accurate diagnosis and surgical treatment can be delayed for several months or even years. Here, we report a case of a 12-year-old girl who presented to the emergency department with lower abdominal pain and mass that had lasted for 2 weeks. After the confirmation of HWW syndrome with magnetic resonance imaging, hysteroscopic septostomy was carried out as a definitive treatment. When we evaluate adolescent girls with lower abdominal pain and mass, we should consider the possibility of HWW syndrome.
Abdominal Pain ; Adolescent ; Child* ; Diagnosis ; Emergency Service, Hospital ; Female* ; Hematocolpos ; Humans ; Magnetic Resonance Imaging ; Mullerian Ducts ; Wolffian Ducts

Female adnexal tumor of probable Wolffian origin (FATWO) is a rare disease entity that arises from the mesonephric duct system. FATWO is different than other gynecological cancers in terms of embryology. Here, we describe the case of a 52-year-old woman with malignant FATWO. The patient underwent explorative laparotomy and surgical staging after a frozen section revealed malignancy. Detailed examination of the pathologic findings were consistent with FATWO. Counseling and further testing were provided to the patient to assess the risk of germline mutation and epigenetic change. An O-6-methylguanine-DNA methyltransferase gene methylation test was positive, and all other tests were normal. This is the first study to report a case of O-6-methylguanine-DNA methyltransferase methylation with FATWO in Korea.
Counseling ; Embryology ; Epigenesis, Genetic ; Epigenomics ; Female* ; Frozen Sections ; Germ-Line Mutation ; Humans ; Korea ; Laparotomy ; Methylation* ; Middle Aged ; Rare Diseases ; Wolffian Ducts

Because the ureter arises from the mesonephric or Wolffian duct (WD), the WD opening should migrate inferiorly along the urogenital sinus or future urethra. However, this process of descent has not been evaluated morphometrically in previous studies and we know little about intermediate morphologies for the descent. In the present work, serial sagittal sections of 15 specimens at gestational age 6–12 weeks and serial horizontal sections of 20 specimens at 6–10 weeks were analyzed. Monitoring of horizontal sections showed that, until 9 weeks, a heart-, lozenge- or oval-shape of the initial urogenital sinus remained in the bladder and urethra. Thus, the future bladder and urethra could not be distinguished by the transverse section or plane. The maximum width of the urogenital sinus or bladder at 6–10 weeks was 0.8 mm, although its supero-inferior length reached 5 mm at 10 weeks. During earlier stages, however, the medial shift of the WD was rather evident. Depending on the extent of upward growth of the bladder smooth muscle, the descent of the vas deferens became evident at 10–12 weeks. Development of the urethral rhabdosphincter likely resulted in the differentiation of urogenital sinus into the urethra and bladder before formation of the bladder neck with 3-layered smooth muscles. Development of the prostate followed these morphological changes, later accelerating the further descent of the WD opening. Because of their close topographical relationships, slight anomalies or accidents of the umbilical cord at 10–12 weeks may have a significant effect on normal anatomy.
Embryonic Structures* ; Fetus* ; Gestational Age ; Human Development ; Humans* ; Muscle, Smooth ; Neck ; Prostate ; Umbilical Cord ; Ureter ; Urethra ; Urinary Bladder ; Vas Deferens* ; Wolffian Ducts*

Carcinosarcomas of the uterine cervix are extremely rare. Cervical carcinosarcoma can be characterized by having two different origins: the Mullerian ducts and the mesonephric duct remnants. A 53-year-old Korean woman was admitted to the hospital because of pelvic mass detected on computed tomography scan done at private clinic. A Radical hysterectomy with bilateral salpingooophorectomy and pelvic lymphadenectomy was carried out upon a diagnosis of stage IB2 cervical sarcoma. Immunohistochemically, the epithelial component was positive for pancytokeratin and estrogen receptor, but negative for CD 10 and carletinin. The mesenchymal component was positive for vimentin. The histopathologic diagnosis was a carcinosarcoma of the uterine cervix arising from Mullerian ducts. She underwent chemotherapy. She developed systemic recurrence seven months after operation and died of disease. The origin of cervical carcinosarcoma needs to be verified and immunohistochemical studies using mesonephric marker (CD 10, carletinin, and estrogen receptor) is helpful.
Breast Neoplasms ; Carcinosarcoma* ; Cervix Uteri* ; Diagnosis ; Drug Therapy ; Estrogens ; Female ; Humans ; Hysterectomy ; Lymph Node Excision ; Middle Aged ; Mullerian Ducts* ; Recurrence ; Sarcoma ; Vimentin ; Wolffian Ducts

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

OBJECTIVETo report a rare case of ectopic mesonephric duct cyst with ectopic testicular malignancy and improve the diagnosis and treatment of the disease.

METHODSWe retrospectively analyzed the clinical data of a case of ectopic mesonephric duct cyst with ectopic testicular malignancy, reviewed relevant literature at home and abroad, and investigated the pathogenesis, diagnosis and treatment of the disease.

RESULTSA large cyst and the right ectopic malignant testis were removed via abdominal incision, and the left undescended testis was lowered into the scrotum. Pathological examination confirmed the lesion to be right ectopic mesonephric duct cyst with right ectopic testicular seminoma. No metastasis was found during a year of follow-up.

CONCLUSIONEctopic mesonephric duct cyst with ectopic testicular malignancy was a rare disease. Imaging examination contributes to its diagnosis, but it has to be confirmed by pathology. Surgical removal should be performed as early as possible and follow-up treatment depends on the pathologic type and stage of ectopic testicular malignancy.

Cryptorchidism ; therapy ; Cysts ; pathology ; Humans ; Male ; Neoplasms, Germ Cell and Embryonal ; surgery ; Seminoma ; surgery ; Testicular Neoplasms ; surgery ; Testis ; surgery ; Wolffian Ducts ; pathology

We report two cases of ectopic epididymal ducts and efferent ductules in the testicular appendices (TAs) of adult men with normally descended testes. In both cases, a sessile TA was incidentally found at the upper pole of the right testis during the scrotal hydrocelectomy. Microscopically, a few closely arranged tubules were detected within the TA. In the first case, the tubules were lined with a pseudostratified columnar epithelium with numerous, long microvilli, and were surrounded by a smooth muscle coat. In contrast, in the second case, the tubules had a wavy luminal surface, because ciliated columnar cells alternated with groups of cuboidal cells. In both cases, strong CD10 immunoreactivity was observed in the luminal border of the lining epithelium. Surgical pathologists should be aware of the presence of both ectopic epididymal ducts and efferent ductules that can occur in TAs, in order to avoid misinterpretation as transected, functional reproductive structures.
Adult ; Choristoma ; Epididymis ; Epithelium ; Female ; Humans ; Male ; Microvilli ; Muscle, Smooth ; Parovarian Cyst ; Phenobarbital ; Testis ; Wolffian Ducts

We report a rare case of a left ejaculatory duct that allotropically protrudes towards or invades the left vesicle triangular area with its dead end. The patient simultaneously exhibited multiple congenital malformations of the homolateral urogenital system, such as absence of the left kidney, dysplasia and allotopia of the left seminal vesicle, absence of the left ureterostoma, separation between the left testis and the epididymis tail, and maldevelopment of the left testis. According to all clinical and laboratory evidence, the case represented a new syndrome, which we named Wuyang's syndrome. It involved a rare phenomenon in embryonic development; the dysplastic proximal vas precursor, having intruded into a common mesonephric duct and accidentally encroaching on the ureteric bud position, resulted in the absence or dysplasia of the homolateral urinary tract and ectopic invasion of the bladder by the homolateral seminal tract.
Abnormalities, Multiple ; pathology ; surgery ; Adult ; Ejaculatory Ducts ; abnormalities ; pathology ; surgery ; Humans ; Magnetic Resonance Imaging ; Male ; Syndrome ; Ureter ; abnormalities ; Urinary Bladder ; abnormalities ; pathology ; surgery ; Wolffian Ducts ; abnormalities

Vitamin D-dependent rickets(VDDR) is a rare autosomal disorder, characterized by hypocalcemia, hypophosphatemia, increased alkaline phosphatase, secondary hyperparathyroidism and many other clinical features. Type I VDDR is due to congenital defects of renal 1alpha-hydroxylase, the enzyme responsible for the conversion of 25-(OH)D3 to 1,25-(OH)2D3. Type II VDDR arise from target organ resistance to 1,25-(OH)2D3. Unilateral renal aplasia is generally thought to result from a lack of induction of the metanephric blastema from the ureteral bud, which may be secondary to ureteral bud maldevelopment and/or to a problem with the formation of the mesonephric duct. The incidence of unilateral renal aplasia is approximately 1/500-3,200. Type 1 VDDR associated with unilateral renal aplasia has not been reported yet. Thus we report a case of a 3 month old female infant diagnosed as type 1 VDDR with unilateral aplasia of kidney.
Alkaline Phosphatase ; Congenital Abnormalities ; Female ; Humans ; Hyperparathyroidism, Secondary ; Hypocalcemia ; Hypophosphatemia ; Incidence ; Infant ; Kidney ; Rickets ; Ureter ; Urogenital Abnormalities ; Vitamins ; Wolffian Ducts