Adenocarcinoid of the appendix presenting as a disseminated ovarian carcinoma: report of a case.

The occurrence of disseminated tumors of the appendix is a rare event. Usually appendix tumors are very small, located on the inside of the appendix, and can be pathologically diagnosed. Adenocarcinoid is an uncommon variant of carcinoid tumors that usually arises in the appendix. This report describes a case of a primary adenocarcinoid of the appendix in a patient who was preoperatively diagnosed to have uterus myomatosus but was intraoperatively found to instead have disseminated ovarian carcinoma. This case demonstrates that the clinical picture can be misleading, and that surgeons therefore always have to wait for the final pathological report before making a final diagnosis.

Prenatal diagnosis of a constitutional interstitial deletion of chromosome 5 (q15q31.1) presenting with features of congenital contractural arachnodactyly.

Prenatal diagnosis of a constitutional interstitial deletion of chromosome 5 (q15q31.1) in a 30-year-old woman is reported. At 21 weeks of pregnancy, routine fetal ultrasounds showed the presence of apparently isolated bilateral club feet. Fetal karyotyping documented an interstitial deletion of the long arm of chromosome 5: 46,XX,del(5) (q15q31) in all 50 analyzed metaphases. Because such deletion is associated with severe psychomotor retardation, the pregnancy was terminated. Postmortem karyotyping of skin fibroblasts confirmed the presence of this interstitial de novo deletion in all mitoses. The breakpoints on 5q were analyzed by fluorescent in situ hybridization and were localized at 5q15 and q31.1. This case illustrates the importance of fetal karyotyping in cases of isolated club feet. At autopsy, the fetus presented had minor anomalies and contractures of knee and hip joints. These clinical findings could fit the diagnosis of congenital contractural arachnodactyly (CCA) or Beals syndrome. CCA is caused by a defect in the fibrillin-2 (FBN2) gene. This gene was previously mapped on 5q23-31. Our molecular studies of both parents and the fetus, using an intragenic polymorphic GT repeat, showed that the FBN2 gene was deleted in the fetus and that the de novo interstitial deletion occurred on the paternally inherited chromosome 5. Thus, CCA may be caused by a loss of function of the FBN2 gene. Clinical findings in this fetus and those of other described cases with interstitial 5q deletions are reviewed, and similarities with CCA are stressed.

Development of endosalpingoblastosis and tuboperitoneal fistulas following tubal sterilization: relation with uterine adenomyosis.

A total of 25 consecutive patients who had undergone a tubal sterilization and who were referred for a hysterectomy, were examined by a peroperative methylene blue test of the tubal stumps, and extensive microscopic examination of the uterine wall, cornua and tubal stumps. Eighteen patients had been sterilized by electrocoagulation and 7 by mechanical methods (clips or rings). Tubo- or uteroperitoneal fistulas and endosalpingoblastosis were only observed in the group of patients sterilized by electrocoagulation. The development of tubo- or uteroperitoneal fistulas was correlated with the presence of endosalpingoblastosis and of uterine adenomyosis (P = 0.002 and P = 0.038, respectively). All patients with bilateral fistulas had bilateral endosalpingoblastosis and the only patient with a unilateral fistula had endosalpingoblastosis on the same side. The development of endosalpingoblastosis in patients sterilized by electrocoagulation was correlated with the presence of uterine adenomyosis (P = 0.008). In the same group of patients, a correlation between the length of the proximal tubal stump and the development of utero- or tuboperitoneal fistulas was observed (Wilcoxon test, P = 0.033). Two patients developed an ectopic pregnancy following sterilization. Both patients were sterilized by electrocoagulation, and had endosalpingoblastosis and bilateral fistulas. Our results suggest that the presence of uterine adenomyosis might predispose to the development of endosalpingoblastosis when performing tubal electrocoagulation close to the uterine cornum. We therefore suggest that when performing tubal coagulation, the intact proximal stump should be at least 2 cm.

Fryns syndrome without diaphragmatic hernia?

We report on a child with Fryns syndrome including lung hypoplasia, characteristic facial appearance, cleft palate, cardiac anomaly, distal limb abnormalities, absent nipples, bicornuate uterus and early death. In contrast to most patients with Fryns syndrome, diaphragmatic hernia was absent in our patient. However, the diaphragm was reduced to a fibrous web with reduced muscular component.

Perinatal listeriosis; more common than reported (2 case reports and revision of literature).

Listeriosis is again reported with an increasing frequency in recent literature. It is reported to be the third cause of neonatal sepsis [4,7]. Hereby we are presenting two cases of perinatal listeriosis together with a summary of literature.

Prostacyclin and thromboxane in benign and malignant breast tumours.

6-keto-PGF1 alpha and thromboxane B2 were determined by radioimmunoassay in 37 extracts of breast carcinomata, 8 fibroadenomata, 12 sclerocystic-disease specimens and 51 normal breast tissues. More prostanoids were extracted from carcinomata than from normal specimens, fibroadenomata or sclerocystic-disease tissues (P less than 0.05). The 6-keto-PGF1 alpha/TXB2 ratio was higher in carcinomata than in normal tissues and fibroadenomata (P less than 0.05) but was not significantly different from the ratio in sclerocystic disease. The prostaglandin levels and the 6-keto-PGF1 alpha/TXB2 ratios from carcinomata did not correlate significantly with age, tumour size, differentiation, lymph node status, nuclear-cytoplasmic ratio, host cell reaction, mast cells, necrosis, elastosis, fibrosis or blood vessel density. Lower nuclear density was associated with lower 6-keto-PGF1 alpha/TXB2 ratios (P = 0.01) whereas the latter value was higher when infiltration was lower (P = 0.03). There was a positive correlation between mitotic index and the 6-keto-PGF1 alpha/TXB2 ratio (P = 0.04). Cumulation of variables revealed lower prostanoid ratios in tumours greater than 2 cm without lymph node metastasis then tumours less than 2 cm with lymph node metastasis (P = 0.04). A first follow-up (14 months) showed a higher 6-keto-PGF1 alpha/TXB2 ratio in patients who developed metastasis (P = 0.04). Our study does not confirm the hypothesis that high prostacyclin levels are a good prognostic index in breast cancer.

Prostaglandin F2 alpha in benign and malignant breast tumours.

Prostaglandin F2 alpha (PGF2 alpha) was determined by radioimmunoassay in 57 breast carcinomata, 16 fibroadenomata, and 33 sclero-cystic-disease (SCD) specimens. In 41 cases of carcinoma and 10 cases of fibroadenoma, histologically non-malignant tissue was also obtained from the same breast. PGF2 alpha levels were significantly elevated in breast cancer when compared with the normal tissues and benign diseases (P less than 0.005 for each group). High PGF2 alpha levels were positively correlated with differentiation, positive oestrogen and progestagen receptor status, and low mitotic index. Tumours with good prognosis (less than 20 mm, negative lymph nodes, some degree of differentiation) showed significantly higher PGF2 alpha levels than tumours with a bad prognosis (greater than 20 mm, positive nodes and undifferentiated). A tendency for elevated PGF2 alpha levels was observed with negative lymphatic permeation, postmenopausal status, low grade of nuclear and cellular polymorphism and high degree of elastosis and fibrosis. No correlation was observed between PGF2 alpha levels and host-cell reaction. Plasma levels of 15-keto-13, 14-dihydro-PGF2 alpha were not elevated in cancer patients when compared with the SCD-group. The present study demonstrates that PGF2 alpha levels are high in tumours with good prognosis. However, since other authors have suggested that a high PGE2 production is a bad prognostic index, it is possible that conversion of PGE2 to PGF2 alpha by 9-keto-reductase explains this relationship. Nevertheless, the presented results question the unrestricted use of prostaglandin-synthesis-inhibitors in the treatment of breast cancer.