A true hermaphrodite with some unusual features

A fifteen-month-old boy with ambiguous external genitalia was found to have a 46XX karyotype, an ovotestis in the right labioscrotal fold, and an absent left gonad. He also had a rudimentary uterus and fallopian tubes and a blind-ending vagina lined with squamous epithelium. These features are compared with those more commonly found in true hermaphroditism.     

Small-cell neuroendocrine carcinoma of the ampullary region. A clinicopathologic, immunohistochemical, and ultrastructural study of three cases.

We report the clinicopathologic, immunohistochemical, and ultrastructural features of three small-cell neuroendocrine carcinomas of the ampullary region of the duodenum. All patients were men; their ages were 51, 62, and 66 years. The therapy consisted of pancreatoduodenectomy. All patients died of the disease; median survival was 10 months from the diagnosis. The histological appearance was identical to pulmonary and extrapulmonary small-cell carcinoma. The neuroendocrine differentiation was demonstrated ultrastructurally by the presence of dense-core granules, and by the positive immunoreaction for neuron-specific enolase and Leu-7 in each case. One case expressed a focal positivity for chromogranin A (PHE-5) and argyrophilic granules. The same case showed the presence of neurofilaments on frozen material. Neurofilament proteins could not be demonstrated in any case in paraffin sections. Neoplastic cells exhibited cytoplasmic immunostaining for cytokeratins (CAM 5.2) in all cases. In one case, a large number of neoplastic cells (60-70%) exhibited nuclear Ki-67 positivity. We postulate that the disease's histogenesis was from epithelial stem cell expressing both epithelial and neuroendocrine characteristics. The clinical behavior of small-cell neuroendocrine carcinomas of the ampullary region appears to be extremely aggressive, with early metastases and fatal outcome.     

Giant intradiploic epidermoid tumor of the occipital bone: case report

The authors describe a case of a giant intradiploic epidermoid cyst of the occipital bone with an intracranial extension in the posterior fossa and no signs of neurological involvement. The lesion started as a painless lump under the scalp. Roentgenographic and computed tomographic findings led to a correct diagnosis, and the complete removal of the cyst was accomplished, despite its large size. The total removal of these cysts is associated with a good prognosis.     

Gadolinium-enhanced magnetic resonance imaging in the staging of urinary bladder neoplasms

Magnetic resonance imaging (MRI) proved to be an important diagnostic tool in the correct staging of bladder neoplasms. The advantage of multiplane imaging and high soft-tissue contrast may be extended by the use of MRI contrast media such as the gadoliniumdiethylene-triaminepentaacetic acid complex. In 60 patients with suspected or proved bladder tumors, the results of preoperative gadolinium-enhanced MRI were correlated with the histopathologic findings. The staging accuracy of infiltrating tumors was 83% and sensitivity and specificity 86% and 83%, respectively. Three tumors could only be localized after administration of gadolinium. All active tumors demonstrated significant contrast enhancement after intravenously injected gadolinium. Small papillary tumors or carcinoma in situ remain problematic to preoperative staging procedures. The advantages of gadolinium-enhanced MRI will best be employed in the exact pretherapeutic staging of infiltrative bladder neoplasms or in restaging procedures after chemotherapy and radiotherapy.