Extragenital müllerian adenosarcoma in the pouch of douglas.

BACKGROUND: Extragenital Müllerian adenosarcomas are extremely rare tumours characterised by a stromal component of low-grade malignancy and by a benign glandular epithelial component. CASE REPORT: A 26-year-old woman was admitted to our clinic because of lower abdominal distension and left lower quadrant pain. Clinical and radiological examinations suggested an ovarian malignancy. Laparotomy revealed a cystic mass in the pouch of Douglas, originating from the left sacrouterine ligament. A total excision of the tumour was performed and showed low-grade adenosarcoma without sarcomatous overgrowth. Follow-up at 24 months after the surgery showed no evidence of recurrence. CONCLUSION: Mullerian adenosarcoma located in the pouch of Douglas is rare. For treatment, success may be achieved with only excision of the tumour if there is no sarcomatous overgrowth or spread to adjacent tissues.

Left diaphragmatic eventration associated with ipsilateral pulmonary sequestration and intrathoracic kidney in a fetus: reviewing the prenatal diagnosis and etiopathogenesis.

The prenatal diagnosis of congenital diaphragmatic eventration, pulmonary extralobar sequestration and intrathoracic ectopic kidney in a single fetus is not reported yet according to the pubmed. Congenital diaphragmatic eventration is an abnormal elevation of the diaphragm. Differential diagnosis from hernia is essential for the perinatal management. Extralobar sequestrations are usually asymptomatic and detected incidentally. Intrathoracic kidney is an extremely rare congenital anomaly. Genitourinary and cardiac anomalies should be searched as common co-existing malformations. Besides prenatal ultrasound, fetal magnetic resonance imaging has a substantial support in counselling the family, planning the follow-up of the pregnancy and decision-making for the perinatal management.

Incidental detection of Sertoli-Leydig cell tumor by FDG PET/CT imaging in a patient with androgen insensitivity syndrome.

A 29-year-old female patient who was being followed up for differentiated papillary thyroid carcinoma was referred to us for exploration of any possible metastasis since her serum thyroglobulin levels were high. The patient underwent an F-18 fluorodeoxyglucose positron emission tomography study, and a pathologically increased FDG uptake at the left lower abdomen was detected corresponding to a solid, cystic lesion on CT images. The patient had a history of primary amenorrhea and, together with the magnetic resonance imaging findings of absent uterus, short and blind end vagina, a diagnosis of androgen insensitivity syndrome was made. The patient underwent laparoscopic left pelvic mass resection, and the histopathology of the lesion revealed Sertoli-Leydig cell tumor.

Prenatal ultrasonographical features of limb body wall complex: a review of etiopathogenesis and a new classification.

Limb body wall complex is a spectrum of multiple severe anomalies. The etiopathogenesis and clinical classification are still under discussion. In our article, while reviewing previous etiopathogenetical hypothesis, we propose a new clinical classification regarding embryological theories and pheneotypical features. According to the Van Allen diagnostic criteria, the findings of 6 affected fetuses are presented. Prenatal diagnosis was performed in 5 of 6 cases. Craniofacial malformations were present in only 1 case. Thoracic defect and abdominoschisis (either infraumbilical or supraumbilical) associated with visceral eventration, placental-umbilical cord anomalies, and limb defects were detected in the other 5 cases. Aberrant development of each of the 4 embryonic folds (cephalic, 2 lateral abdominal, and caudal) associated with faulty umbilical ring development and placental formation were considered responsible for development of various malformations. In previous clinical classifications, existence or absence of the craniofacial malformation was utilized as an unique discriminating criterion while multiple anomalies exist. In this report, we propose a new clinical classification concerning almost all anomalies caused by defective placental attachment and maldevelopment of the 4 folds.

Vulvar keratoacanthoma.

A keratoacanthoma of the vulva in a 33-year-old woman is described. The patient presented with a rapidly growing exophytic lesion of 1 cm in diameter over the clitoris that had been present for a couple of months. An excisional biopsy was performed. Pathologic examination of the exophytic nodule showed squamous proliferation with a characteristic central keratin- filled crater. There were pushing margins. High power microscopy showed squamous cells of cytologically bland appearance with glassy cytoplasm. There were few mitotic figures. Less than 10% Ki-67-positive staining nuclei were seen. There was no p53 oncoprotein overexpression. Keratoacanthoma of the vulva is rare with only five cases having been reviewed in the English literature since 1985. Keratoacanthomas are rapidly growing crateriform lesions on the sun-exposed skin of the elderly. Because of its rarity in the vulva, the lesion can be misinterpreted as a malignant lesion such as squamous cell carcinoma. Awareness of vulvar occurrence of keratoacanthoma and its characteristic histopathologic features will help avoid misdiagnosis of a squamous cell carcinoma leading to radical surgery.

The prenatal diagnosis of cloacal dysgenesis sequence in six cases: can the termination of pregnancy always be the first choice?

OBJECTIVE: Cloacal dysgenesis sequence is a lethal malformation, which usually requires termination. In this study, our aim was to evaluate the prenatal and postnatal diagnostic features of cloacal dysgenesis sequence and review the management of the patients. MATERIAL AND METHODS: The data of six cases of cloacal dysgenesis sequences were collected from the ultrasonography and neonatal records. The findings were evaluated in prenatal and postnatal periods. Chromosomal analysis was performed in all the cases. The evaluation of primary and secondary malformations was done. Coexisting anomalies were searched for by radiology and histopathology. RESULTS: Malformations in six cases (two females and four males) were described. The absence of anal, genital, and urinary openings with intact perineum covered by smooth skin were common findings. These features were considered as primary malformations for cloacal dysgenesis sequence. Secondary anomalies (urinary and gastrointestinal system malformations, pulmonary hypoplasia, and other coexisting anomalies) were evaluated. CONCLUSION: The prenatal differential diagnosis of cloacal dysgenesis sequence from other urinary obstructive diseases was essential regarding fetal prognosis, prenatal, and neonatal management. The bladder outlet obstruction and pulmonary hypoplasia due to reduced amniotic fluid and/or kidney disease were considered prognostic factors for neonatal death. Termination of pregnancy is almost always recommended instead of intrauterine shunt procedures; but if we take into consideration one of our cases and a few reported cases who survived in the neonatal period, the prenatal management of these pregnancies needs to be reevaluated.