Endocrine manifestations of Langerhans cell histiocytosis diagnosed in adults.

Langerhans cell histiocytosis (LCH) is a rare granulomatous disease of unknown etiology. We retrospectively reviewed data from four patients (3 males and 1 female), mean age 33.5 years old (range: 21-40), with histopathological diagnosis of LCH. All of them presented with symptoms suggestive of endocrine involvement. The main complaint was goiter in two patients and polyuria and polydipsia in three. Before the LCH diagnosis, two patients had unevaluated symptoms of diabetes insipidus (DI) and hypogonadism. The mean time from symptoms onset to diagnosis was 6.25 years (range: 2-13). Histopathological diagnosis was established by total thyroidectomy (TT) biopsy in two patients, skin lesion biopsy in one, and pituitary stalk biopsy in the other. In the two-first patients, surgery was indicated after the fine-needle aspiration biopsy (FNAB) showed a false positive result of differentiated thyroid carcinoma and immunohistochemistry was used for diagnosis confirmation. Three cases were treated with chemotherapy; one of them had already received radiation therapy on the hypothalamic-pituitary region, developing post-radiation hypopituitarism.

Epidemiology and survival of endometrial cancer. Valuation of data of National Cancer Institute in Naples 1987-1992.

Cancer of the endometrium is a neoplasm which essentially strikes postmenopausal women and in which the predominant risk factors are recognized as an excessive imbalance of estrogen and obesity. The survival rate is good in the first stage and is acceptable in the second, while in the third and especially the fourth stage is unfavorable. To verify and evaluate such facts we have carried out a study of survival rates on patients admitted in our Institute surgery division from 1987 to 1992. The results confirm the importance of the stage and histologic grading of the determining variables, while there appears to be more controversy regarding the role of myometrial invasion. In conclusion, our data show that intervention by pelvic-aortic lymphadenectomy contributes to an improvement in survival rates independently of stage.

Computed tomography and second-look surgery in ovarian cancer patients. Correlation, actual role and limitations of CT scan.

Computed tomography (CT scan) was performed on 58 clinically disease-free ovarian cancer patients. The scans were correlated with the results obtained at a subsequent second-look laparotomy. The sensitivity was 0.47, the specificity 0.87, diagnostic accuracy 0.63, positive predictive value 0.84 and negative 0.53. Undetected microscopic disease was classified as a false-negative result. Sensitivity was poor for omental, mesenteric and peritoneal implants and for bowel infiltration, good for lymphnodal involvement and abdominal mass and decisively good for intrahepatic and plenic metastases of ovarian cancer. Due to a still high false-negative rate a normal CT scan does not provide sufficiently accurate diagnostic information to replace a second-look laparotomy. But on the other hand, due to a high specificity, the usefulness of CT can be limited to approximately 27% of patients, with true-positive findings, who might have been saved surgical reexploration. Adjunct studies such as immunoscintigraphy with radiolabelled monoclonal antibodies and measurement of tumor markers further increase its diagnostic accuracy.

Intraperitoneal chemotherapy with carboplatin and recombinant interferon alpha in ovarian cancer.

Twenty-five patients with Stage III ovarian cancer were entered into a trial with intraperitoneal combinations of carboplatin (400 mg/cqm) and recombinant interferon alfa (50 MU). All patients had received prior intravenous platinum-based chemotherapy and underwent 2nd look laparotomy at study entry. Our study indicates that this combination chemotherapy is safely administered by the intraperitoneal route. Myelotoxicity was frequent, but rarely of grade 3. No major local toxicity was recorded by accessing the peritoneal cavity with a temporary catheter. The response to treatment was promising in the group of patients with less then 2 cm residual disease at study entry (15 patients); in this group, all patients had no clinical evidence of disease at the completion of the therapy. In 2 cases reexploration was performed and pCR was recorded. Only one patient of this group relapsed during a mean follow-up of 21 months. Two pCRS were also recorded in the group of patients with more than 2 cm at 2nd look (9 patients), although relapse occurred after 9 and 15 months respectively. In the remaining patients of this group, persistence of disease was observed after intraperitoneal chemotherapy.