Angiosarcoma of small intestine: case report and literature review / Angiossarcoma de intestino delgado: relato de caso e revisão da literatura

ABSTRACT Introduction: Angiosarcomas are rare pathologies, and their appearance in the small intestine is extremely unusual. Case report: Female patient presenting vomiting, colic, abdominal distension and peritoneal irritation. An exploratory laparotomy and an enterectomy were performed. Histopathology revealed vascular neoplasm; immunohistochemistry, markers CD31, p53 and cell proliferation antigen Ki-67. The patient developed diffuse sarcomatosis and died two months after the onset of symptoms. Conclusion: This case demonstrates the difficulty in early diagnosing this pathology, due to its nonspecific clinical presentation. The disease aggressiveness, its unestablished predisposing factors and the controversy around the adequate treatment engender the poor prognosis.

RESUMO Introdução: Angiossarcomas são patologias raras, e o aparecimento deles no intestino delgado é extremamente incomum. Relato de caso: Paciente do sexo feminino apresentando vômitos, cólica, distensão abdominal e irritação peritoneal. Foram realizadas laparotomia exploratória e enterectomia, evidenciando-se neoplasia vascular ao anatomopatológico e marcadores CD31, p53 e antígeno de proliferação celular Ki-67 à imuno-histoquímica. A paciente evoluiu com sarcomatose difusa e faleceu dois meses após o início dos sintomas. Conclusão: Demonstra-se a dificuldade em diagnosticar precocemente essa patologia devido à sua clínica inespecífica. A agressividade da doença, seus fatores predisponentes indeterminados e a controvérsia referente ao tratamento adequado suscitam seu péssimo prognóstico.

Sister chromatid exchange studies in oral cancer patients.

Sister chromatid exchange (SCE) was studied in PHA-stimulated peripheral blood lymphocytes from 81 untreated patients with squamous cell carcinoma of the oral cavity and 50 age and sex matched controls. The SCE frequencies were found to be 10.80 +/- 1.38 and 4.15 +/- 1.15 in cancer cases and controls respectively. The SCE values of cancer cases deviate significantly from that of controls. The SCE frequency in patients who were addicted to the single habit of betel with tobacco chewing or bidi/cigarette smoking or combined habits of chewing and smoking was 9.38 +/- 1.28, 12.28 +/- 1.68 and 13.12 +/- 2.13 respectively. The SCE frequency in patients who were habituated to alcohol and tobacco usage was 13.43 +/- 2.16. These values were significantly higher as compared with the SCE values observed in normal controls. Single habit of bidi, cigarette and combined habit of bidi and cigarette smokers had a mean SCE per cell of 12.98 +/- 2.11, 10.18 +/- 1.25 and 13.48 +/- 1.32 respectively, which were significantly higher than the mean value of 4.15 +/- 1.15 found in controls. Higher frequencies of SCE were also observed in individuals who smoked more than 10 bidis or cigarettes per day compared with people who smoked less than 10 bidis or cigarettes per day. Individuals who smoked bidis or cigarettes for more than 10 years also showed an increased frequency of SCE as compared with those who smoked bidis or cigarettes for less than 10 years. Among chewers a significant difference was observed with regard to duration and frequency of chewing when compared to controls. An increase in the mean frequency of SCE for each cancer patient group is directly proportional to the clinical stage of the disease. Interchromosomal distribution of SCE revealed an increased frequency of SCE in almost all the groups of chromosomes in cancer patients when compared to controls.

Chediak-Higashi syndrome--a case report with ultrastructural and cytogenetic study.

We report the ultrastructural abnormalities of the leukocyte granules and the cytogenetic findings in a patient of Chediak-Higashi syndrome (CHS), who presented with cutaneous melanosis as the only clinical feature. The diagnosis of CHS was established by peripheral smear and bone marrow examination. Chediak-Higashi syndrome, a rare autosomal recessive disorder is characterized by enlarged abnormal organelles in leukocytes and other cells. An interesting aspect of our patient was the absence of recurrent infections or any other clinical stigmata. Ultrastructurally, the leukocytes and their precursors in the bone marrow showed characteristic homogenous and heterogenous giant inclusions of variable sizes and shapes. These represent the primary granules which enlarge to attain the giant abnormal size by fusion with other primary or secondary granules. Cytogenic study of the bone marrow cells showed monosomy of chromosomes 8 and 17 in 20 percent of the metaphases. Neither the gene nor the chromosomal abnormalities specific for CHS have been identified as yet and thus the significance of our cytogenetic finding is presently not clear.

Cytogenetic and viral studies in nasopharyngeal carcinomas in patients of Indian origin.

Four cases of undifferentiated nasopharyngeal carcinomas (NPC) (grade III-IV) in patients of Indian origin were investigated for specific chromosome markers and evidence of Epstein-Barr virus (EBV) positivity. Abnormalities involving chromosome #3, like del (3) (p24-pter) and 3q+(q27-qter) were found in these patients, similar to earlier reports in patients of Chinese and Kenyan origin2,4,13 who however were EBV positive, unlike the patients in this study who were EBV negative. Implications of the cytogenetic and serological data in Indian patients with NPC, available for the first time, may throw some light on the etiology of the disease in this ethnic group where nasopharyngeal carcinoma is also endemic.

Chromosome analysis in effusions as a diagnostic adjunct to cytology.

Cytogenetic analysis using G-banding techniques for the diagnosis of malignancy in pleural and peritoneal effusions is compared with the results of traditional cytomorphology. The studies indicate that the yield of mitotic cells in malignant effusions is quite high when compared to fluids with no cytological evidence for malignancy. Chromosome criteria for malignancy are of special value in the differential diagnosis of reactive cells versus malignant cells, which pose problems when cytological evaluation alone is considered. Cytogenetic analysis can be performed rapidly and used side by side with conventional cytological procedures to obtain higher sensitivity for cancer diagnosis.