Progressive multifocal leukoencephalopathy in a patient with Alzheimer's disease.

An 80-year-old woman with slowly progressive dementia of at least 1 year's duration died with an abrupt change in the rate and character of mental deterioration. Pathologic examination of the brain showed Alzheimer's disease as well as progressive multifocal leukoencephalopathy (PML) in the parietal and cerebellar areas. JC virus (JCV) was detected by polymerase chain reaction (PCR) amplification of paraffin sections from both PML and non-PML areas of the cerebrum. Analysis by in situ hybridization and reverse transcriptase PCR in situ localized JC viral DNA and RNA to the nuclei of oligodendrocytes only in the areas of demyelination. This case is noteworthy in that PML occurred in the setting of Alzheimer's disease by presumed activation of latent JCV in the absence of usual causes of immunocompromise.

Squamous metaplasia of normal and carcinoma in situ of HPV 16-immortalized human endocervical cells.

The importance of cervical squamous metaplasia and human papillomavirus 16 (HPV 16) infection for cervical carcinoma has been well established. Nearly 87% of the intraepithelial neoplasia of the cervix occur in the transformation zone, which is composed of squamous metaplastic cells with unclear origin. HPV DNA, mostly HPV 16, has been found in 90% of cervical carcinomas, but only limited experimental data are available to discern the role of HPV 16 in this tissue specific oncogenesis. We have initiated in vivo studies of cultured endocervical cells as an experimental model system for development of cervical neoplasia. Using a modified in vivo implantation system, cultured normal endocervical epithelial cells formed epithelium resembling squamous metaplasia, whereas those immortalized by HPV 16 developed into lesions resembling carcinoma in situ. In contrast, their ectocervical counterparts formed well differentiated stratified squamous epithelium and a lesion with mild dysplastic change, respectively. The HPV 16-immortalized cells showed in vivo cytokeratin expression patterns similar to their respective normal counterparts, confirming their different origins. Thus, this study provides direct experimental evidence for the transformation of simple epithelial cells of endocervical origin into stratified squamous metaplasia and indicates the differential susceptibility of endo- and ectocervical epithelial cells for conversion to cancer by HPV 16.

Epithelioid hemangioendothelioma of liver. Clinicopathological correlation of 10 cases treated by orthotopic liver transplantation.

We present a review of the clinicopathologic features of a series of 10 patients with hepatic epithelioid hemangioendothelioma treated by orthotopic liver transplantation. This tumor has an unpredictable prognosis. Morphological features predictive of biological behavior were not identified in these patients. It appears that metastatic spread at the time of surgery is not a significant contraindication to surgery and has no correlation with survival. Nine patients are alive (5-134 months), five disease free and four with tumor. The etiology of this tumor is unknown. However, there is an association of oral contraceptive use with this tumor in four (66%) of the six female patients in this series and in 37% of such reported cases.

Disseminated granulomatous disease (BCGosis) following chemoimmunotherapy for ovarian carcinoma.

The use of BCG (bacille Calmette Guérin) in conjunction with cytotoxic chemotherapeutic agents has been advocated in patients with ovarian carcinoma. We describe a patient with stage III, grade I, endometrioid carcinoma of the ovary treated with cisplatin, doxorubicin, cyclophosphamide, and BCG. Following one course of therapy she presented with an elevated temperature, purpuric skin rash, abnormal liver function tests and hematological indices, and multiple organ failure resulting in sepsis and death. At autopsy, disseminated noncaseating granulomas were found in the lungs, hilar lymph nodes, liver, and spleen. Metastatic carcinoma was not present in these organs. This report describes the rapid onset of a disseminated BCG infection (BCGosis) in a patient with ovarian carcinoma receiving chemoimmunotherapy. Clinical recognition of BCGosis in immunocompromised patients is difficult but should be considered in the differential diagnosis of patients presenting with unexplained febrile illness, functional abnormalities in multiple organ systems, and a history of immunotherapy with BCG. Appropriate specimen collection is emphasized.

Multiple paragangliomas in neurofibromatosis: a new neuroendocrine neoplasia.

A pheochromocytoma, a glomus jugulare tumor, and multiple pulmonary paragangliomas were found in a patient with neurofibromatosis. We review the literature and add this case to the growing number of case reports of neurocutaneous disease in association with neuroendocrine tumors.