Crohn's disease associated to chordoma: a case report

Crohn's disease is an inflammatory bowel disease characterized by remissions and exacerbations. Immunosuppressants are frequently used to induce and maintain remission in these patients. The use of the immunomodulator azathioprine has been associated to malignancies. Chordomas are rare, locally aggressive tumors arising from remnants of the notochord. A specific trigger for this tumor has not been identified and association to any medication has not been reported. The purpose of this report is to present the first case reported in the literature of Crohn's disease associated to a chordoma. The patient to be presented was on azathioprine therapy, among other medications. A review of literature revealed that Crohn's disease and chordoma have abnormalities in chromosomes 1 and 10. Inflammatory bowel disease and chordoma also have abnormalities in chromosomal regions 1p, 3p, and 7q. Despite these findings, a direct genetic relationship between these diseases is speculative.

Epstein-Barr virus in biopsies from patients with Hodgkin and non-Hodgkin lymphoma at the University of Puerto Rico immunohistochemistry laboratory

OBJECTIVES: We aimed to determine the Epstein-Barr Virus (EBV) presence rate in our laboratory's lymphoma tissue biopsies for comparison with that reported in literature. BACKGROUND: The presence of EBV has been established in Hodgkin lymphoma (HL), endemic Burkitt Lymphoma and some non-Hodgkin lymphomas (NHL). It has been linked to geographic, ethnic and socioeconomic factors, with a lower rate in developed countries. METHODS: We used the immunoperoxidase technique to determine the rate of the EBV LMP-1 in eighty-seven biopsies diagnosed as lymphoma. Tissue slides were stained using the Ventana Automated Slide Stainer with the DAKO EBV LMP-1 primary antibody and the results were analyzed with the SYSTAT program. RESULTS: We found an LMP-1 positive rate of 50 per cent for 22 cases of HL and 35 per cent for 63 cases of NHL. Among HL, 5 were children and 16 were adults, with LMP-1 positive rates of 60 per cent and 50 per cent respectively. Among NHL, 3 were children and 59 were adults, with equal LMP-1 positive rates of 33 per cent. The sex LMP-1 positive rates for HL were 42 per cent for 12 males and 60 per cent for 10 females. Among NHL, the sex LMP-1 positive rates were 39 per cent for 38 males and 28 per cent for 27 females. NHL was further subdivided into subtypes and LMP-1 primary antibody positive rates were reported. CONCLUSIONS: We found a similar presence rate of EBV in the HL biopsies to that of developed countries, but a similar presence rate of EBV in NHL biopsies to that of developing countries.

Prevalence of steroid receptors and HER 2/neu in breast cancer biopsies of women living in Puerto Rico

OBJECTIVE: The purpose of this study is to determine the prevalence rate of estrogen and progesterone receptors and HER 2/neu in the breast cancer biopsies analyzed in the Laboratory of Immunohistochemistry of the University of Puerto Rico School of Medicine in the year 2000. This data may serve as a reference point for future studies of the epidemiological aspects of breast cancer among women living in Puerto Rico. BACKGROUND: Determination of estrogen receptor (ER) and progesterone receptor (PR) on biopsy specimens of breast carcinoma prior to treatment is standard practice in the management of breast carcinoma. ER and PR are used to identify patients who are likely to respond to endocrine therapy. The prevalence of ER, PR and Her2/neu among USA women is 77 per cent, 55 per cent and 10-34 per cent, respectively. One of the major clinical roles for testing HER 2/neu expression is to determine eligibility for treatment with Trastuzumab. METHODS: Retrospective analysis of 309 breast cancer biopsies was done. Paraffin embedded blocks of breast cancer tissue biopsies were received from different hospitals and Pathology Laboratories located throughout the island specifically for routine analysis of steroid receptor (ER/PR) and/or HER 2/neu expression. Immunostaining was performed in a Ventana Medical Systems automated instrument. RESULTS: Positive nuclear staining for ER and PR were seen in 65.9 per cent (203/308) and 51.8 per cent (159/307), respectively. In the HER2/neu test, 27.8 per cent (46/165) gave a strong and complete membranous staining (score 3+). CONCLUSIONS: There is a lower prevalence of estrogen receptor in the breast cancer biopsies of women living in Puerto Rico than their USA counterparts, but similar prevalence of progesterone receptorstatus and HER 2/neu protein over expression.

The contribution of immunohistochemistry in the diagnosis of pediatric neoplastic lesions in the University of Puerto Rico, School of Medicine.

Immunohistochemistry has revolutionized the field of diagnostic pathology in the past fifteen years. Since then, it has been increasingly used as an adjunct to morphological diagnosis. The purpose of this retrospective analysis is to examine the value of this technique in the diagnosis of pediatric neoplasias in our laboratory. Forty eight pediatric cases, collected from January 1998 until May 1999, were reviewed, and classified in one of four categories: confirmed the morphological diagnosis, provided the definite diagnosis from a list of probable diagnoses, contributed by excluding other entities, and non-contributory. Immunohistochemistry confirmed the morphological diagnosis in 29 cases (60.4%), provided the definite diagnosis from a list of probable diagnoses in 13 cases (27.1%), was contributory by exclusion of other entities in 2 cases (4.2%), and was non-contributory in 4 cases (8.3%). In this preliminary study, we conclude that immunohistochemistry is being used in our laboratory mostly as a confirmatory tool for the definitive diagnosis of the lesions and once more exalts the utility of this technology in the field of diagnostic pathology.

Chronic hepatitis C: treatment comparison between 3 and 5 million units of interferon alpha-2b.

Interferon (IFN) is the only drug that has been approved by the FDA for therapy of chronic hepatitis C. However, optimal dose and duration of therapy are still controversial. This study compares the effectiveness of treatment of chronic hepatitis C patients with 3 vs. 5 million units (MU) of recombinant alpha-interferon 2-b three times per week. We also evaluated the relapse rate with a shorter 12 week-course of therapy in those patients who had normalization of aminotransferases by week 12. Seventy-five patients were randomized to receive either 3 vs. 5 MU of IFN; seventy-two completed the study. A complete response was seen in 11/35 (31%) of those treated with 5 MU vs. 13/37 (35%) in the 3 MU dose (p = 0.74). Patients were followed after IFN was withdrawn and only 2 had persistently normal aminotransferases. Analysis of multiple variables was done to predict response to IFN and only elevations of GGT, ferritin and alkaline phosphatase were found to be predictors of a poor response. Therefore, we recommend initial therapy with 3 MU of IFN for a longer period than 12 weeks in patients who show a response.

Sustained response to interferon in a patient with common variable immunodeficiency and hepatitis C.

Hepatitis C infection progresses faster in patients with hypogammaglobulinemia, often leading to death from liver failure within 10 yr of exposure. Response to interferon treatment has been poor in these patients. We report the case of a patient with common variable immunodeficiency and hepatitis C in whom a sustained biochemical and viral remission was obtained after treatment with interferon.

Acute fatty liver of pregnancy. Case report and review of the literature.

A case of acute fatty liver of pregnancy is presented. This case is characterized by many of the reported diagnostic features of AFLP, including the abrupt onset of symptoms with progressive liver failure, coagulopathy and encephalopathy, during the third trimester of pregnancy. Autopsy findings confirmed the diagnosis. The literature is reviewed, emphasizing the clinical and laboratory features helpful in the diagnosis of this entity.

Budd Chiari syndrome in a post partum female with adrenal cortical carcinoma. Case report and review of the literature.

We report the case of a post partum female whose first manifestation of her adrenocortical carcinoma was an acute Budd Chiari syndrome. We also review previously reported cases of adrenocortical carcinomas with associated Budd Chiari syndrome.