Malignant teratoid medulloepithelioma: a case report

We report the histopathological findings of the anterior cornea from a patient with skin biopsy-proven epidermolysis bullosa simplex. As seen with light microscopy, the cornea had a fibrocellular pannus deep to a thickened epithelial basement membrane. Transmission electron microscopy of the anterior cornea demonstrated a nonhomogeneous basal epithelial layer with abnormal attachment complexes to an irregular, multilaminar basement membrane. The findings of transmission electron microscopy of the bulbar conjunctiva were normal.     

Congenital urethrocutaneous fistula: a case report and literature review, with a nomenclature proposal

The authors report the case of a 4-year-old boy who had a congenital urethrocutaneous fistula (CUF) on the penile urethra. The previous reports of patients with fistula from the urethra to the skin are evaluated, and the spectrum of CUF is discussed.     

Congenital tracheoesophageal fistula with symptoms commencing at the onset of trauma: report of a case

Congenital tracheoesophageal fistula without atresia can persist and remain undetected without symptoms until adulthood. We have recently encountered such a rare case and also conducted a review of the literature. Symptoms are commonly present during infancy, but occasionally patients do not develop symptoms until adulthood. In this case, it is suggested that the symptoms commenced after the redundant mucosal flap or membrane was broken due to a trauma to the chest.     

Congenital coronary artery fistula in an Ethiopian child: case report

Dual photon absorptiometers (DPA) are currently being replaced by dual energy X-ray absorptiometers (DXA) for measurements of bone mineral density (BMD). In order to evaluate how to use the previously obtained BMD results by DPA (BMDo) in the follow-up of patients, the following study was performed. Ninety-five women who had had BMDo during the last 12 months were selected. L2-L4 BMD was measured twice on the same day using both DPA (BMDp) and by DXA (BMDx). BMDp was highly correlated to BMDx (R = 0.95; P < 0.001) but a wide variation of the ratio BMDp:BMDx was observed which ranged between 0.65 and 1.09 (mean 0.90, S.D. 0.05). There was no significant relationship between the ratio BDMp:BMDx and age or height but there was a significant relationship between this ratio and the weight of the patient (R = 0.31; P < 0.001). The results also indicated that the correlation between DXA and DPA was lower than that observed between the two DPA measurements (R = 0.98; P < 0.001) which, moreover, were not performed on the same day. These observations contrast with the relatively high precision of both instruments and have to be attributed to relative inaccuracy of one or probably both techniques.     

Malignant rhabdoid tumor of the small intestine in an adult: a case report with immunohistochemical and ultrastructural findings

The expression of platelet-derived endothelial cell growth factor (PD-ECGF) was determined immunohistochemically in 143 non-small cell lung carcinomas. Staining was observed in 48% of the cases. A relationship between histology, stage, erbB-1, erbB-2, ras and PD-ECGF expression was not found. A relationship of borderline significance was observed between PD-ECGF and p53 expression. There was also no relationship between PD-ECGF expression and proliferative activity (G1 phases, S phases, cyclin A). In contrast, a correlation between PD-ECGF- and VEGF-expression was detectable (p=0.009). Furthermore, PD-ECGF expression was related to the response of lung carcinomas to doxorubicin (p=0.0004). Of 35 sensitive tumors, 26 carcinomas were PD-ECGF-positive (74%) while of 108 resistant carcinomas only 43 tumors (40%) exhibited PD-ECGF expression.     

Congenital H-type anourethral fistula with severe urethral hypoplasia: case report and review of the literature

The annual number of cadaveric organ donors increased from 13.1 per million inhabitants in the period 1989-92 to 15.8 in the period 1993-96. Multiple organ harvesting was performed in 68% of the donors. There are significant differences in donation rate between health regions. An increase to 20 organ donors per year per million inhabitants is required to meet the anticipated need for organs. Strategies to increase organ donation are discussed.     

Hemihypertrophy with a liver cyst: a case report

An 8-month-old girl with a right-sided hemihypertrophy was found to have a large abdominal mass. At exploration, the mass, arising from the antero-inferior edge of the right lobe of the liver, was pedunculated and measured 17 cm x 12.5 cm x 12.5 cm. This was excised and histology confirmed it to be a benign single cyst of the liver.     

Chondrosarcoma of the urinary bladder: report of a case with immunohistochemical and ultrastructural findings and review of the literature

A primary chondrosarcoma arising in the urinary bladder is described in a 73-year-old female, together with a review of the 2 previously reported cases in the literature. The clinical symptoms are similar to transitional cell carcinoma, but chondrosarcoma usually presents at an advanced stage, and the outcome is rather poor. This very rare and commonly poorly differentiated tumor should not be confused with poorly differentiated transitional cell carcinoma. The differential diagnosis from other lesions with chondroid features, such as chondroid metaplasia, osteosarcoma and carcinosarcoma, is discussed. The value of immunohistochemistry and electron microscopy in the differential diagnosis is demonstrated.     

Perineurioma with ossification: a case report with immunohistochemical and ultrastructural studies

We evaluated the elbow flexion test in 216 elbows without compression of the ulnar nerve at the cubital tunnel and without other neuropathies. We used Rayan's four positions as our test. The percentage of positive tests was only 3.6% at one minute, whereas evaluating the responses at three minutes we saw positive results in 16.2%. Therefore we find that if the test is performed for one minute it may be useful to help in diagnosing ulnar nerve compression at the cubital tunnel.     

Syringomyelia associated with posterior fossa cyst: a case report

We report a case of a 47-year-old woman with a posterior fossa cyst associated with syringomyelia and hydrocephalus. Her birth was traumatic and she had suffered a fractured skull. About 10 years prior to coming to our department she had occipitalgia and a pain had developed over a month from her right shoulder to hand. Shortly before presenting occasional electric-like shocks were felt in her right hand associated with coughing and hiccuping. Magnetic resonance imaging (MRI) revealed a large midline posterior fossa cyst, hydrocephalus and syrinx (C1-Th11). Cisternography could not demonstrate communication between the cyst and the subarachnoid space and the 4th ventricle was present but without communication with the cyst. The cyst was tentatively diagnosed as an arachnoid cyst. One week after placement of a cyst-peritoneal shunt, CT scans showed a decrease in cyst and ventricle size and 2 months later a follow-up MRI revealed resolution of the syringomyelia. We suspected that cyst-peritoneal shunt was effective for this patient who had syringomyelia associated with incommunicated posterior fossa cyst.     

Vesico-vaginal fistula with a giant vesico-vaginal stone: a case report

Our purposes were to develop an improved linkage map for rat Chromosome 3 and to develop new markers polymorphic between Dahl salt-sensitive (S) and Dahl salt-resistant (R) rats. The linkage mapping panel consisted of three F2 populations totaling 359 rats. Twenty-five new markers were developed and placed on the linkage map. About half of these markers (13) were polymorphic between S and R rats. The final map spans 124.7 centiMorgans (cM) and includes 64 markers. The average distance between adjacent markers is 1.9 cM, and the largest separation is 10.5 cM.     

Solitary bone cyst with epiphyseal involvement: a case report

A large solitary bone cyst involving the epiphysis produced 5.0 cm shortening of the proximal humerus in a 15-year-old girl. Biopsy revealed the cyst was filled with thin yellow fluid under pressure and lined by a fibrous membrane. X-rays 9 months after biopsy demonstrated progressive cyst obliteration. A review of literature revealed no similar case. It is postulated that solitary cysts rarely may involve the physeal and epiphyseal structures and thereby retard longitudinal bone growth.     

Duplication cyst of the antrum: a case report

A four generation family (UoM1) was ascertained with Waardenburg syndrome type 1 (WS1). The proband exhibited both WS1 and septo-optic dysplasia. A G to C transversion was identified in PAX3 exon 7 in four subjects affected with WS1 in this family including the proband. This glutamine to histidine missense mutation at position 391 may also affect splicing. There are over 50 mutations characterised in PAX3 in WS1 patients; however, this is the first example of a WS1 mutation in exon 7 of PAX3.     

Epidermoid cyst of the scrotum: a case report

A 52-year-old male visited our department for a painless swelling from the right side of the anus to the right scrotum that he first noticed 5 years earlier. Palpation revealed a small fist-sized elastic solid mass with a smooth surface from the central area to the inferior pole of the right scrotum. This mass was clearly demarcated from the contents of the scrotum and not adherent to the scrotal skin. A diagnosis of a tumorous lesion in the scrotum was made, and the tumor was resected. Histopathological examination demonstrated a scrotal epidermoid cyst. To our knowledge, this is the 17th case reported in Japan.     

Congenital thymic cyst: prenatal sonographic and postnatal magnetic resonance findings

The link between human Papillomavirus (HPV) and cervical carcinomas has become increasingly convincing recently, and the detection of infection with certain HPV types may become an important element in screening for cervical pre-cancer. We applied the polymerase chain reaction (PCR) technique to detect and type HPV-DNA in biopsies from the uterine cervix with a histological diagnosis of CIN and condylomatosis. Forty-eight consecutive cervical biopsies were analysed for HPV-DNA by PCR, using two different sets of consensus-primers. Typing was performed with HPV type-specific primers. Histological reevaluation revealed that 42 biopsies (87%) had condylomatous features. HPV was demonstrated in 46/48 (95.8%). HPV type 6/11 was found in seven biopsies, while HPV 16 was present in 23 samples, including two cases of double-infection. HPV type 18 was not found at all, and in 19 cases the HPV type present could not be determined. Apparently, HPV-DNA was absent in two cases. The results indicate that HPV-DNA screening is a valuable supplement to histopathological screening for cervical neoplasia. The value of different primer pairs is discussed.     

Uretero-external iliac artery fistula: a case report

We report a case of uretero-external iliac artery fistula. A 60-year-old female was referred to our hospital complaining of intermittent gross macrohematuria. She had undergone radical hysterectomy, radiation therapy and chemotherapy for advanced cervical cancer 2 years ago. The patient had a 7 Fr ureteral double-J stent for left hydronephrosis. Retrograde urography showed a filling defect (8 mm in diameter) of the left ureter. A contrast-enhanced computed tomographic scan showed left hydronephrosis and hydroureter but no evidence of fistula formation or extravasation. A pelvic arteriography revealed a pseudoaneurysm of the left external iliac artery at the crosspoint between the left ureter and the iliac artery. Surgical repair of the left uretero-external arterial fistula was successfully performed as well as left nephroureterectomy. The possibility of fistula formation between ureter and artery should be kept in mind in patients with long-term indwelling ureteral stents and history of radiation therapy.     

Congenital mediastinal bronchogenic cyst with malignant transformation: an autopsy report

A rare autopsy case of mediastinal bronchogenic cyst with malignant transformation is presented. The cyst had been located in the anterior mediastinum for at least 28 years in a 52 year old male. Chest X-ray findings showing rapid enlargement of the cyst and biopsy of the spine for lumbago made a clinical diagnosis as suspicious mediastinal cystic teratoma with malignant transformation metastasizing to the spine. Postmortem examination revealed that the cyst was located in the anterior mediastinum extending to the left pulmonary hilum and had no connection with the tracheobronchial tree. The cyst wall consisted of bronchus-like tissue including ciliated epithelium, hyaline cartilage, smooth muscle and mucoserous glands. There were no teratomatous components in the wall. Malignant tumor predominantly consisting of round cells occurred in the thickened cyst wall and grew into the cyst cavity with direct invasion of the lung and metastases to the liver, adrenal glands, bone marrow of the lumbar spine and lymph nodes. An immunohistochemical study showed that the tumor cells frequently expressed cytokeratin, epithelial membrane antigen and carcino-embryonic antigen, occasionally CA19-9, vimentin and neuron-specific enolase. From these findings, the tumor was diagnosed as undifferentiated carcinoma arising in the mediastinal bronchogenic cyst.