Estrogen and progesterone receptor determination in the papillary cystic neoplasm of the pancreas. With immunohistochemical and ultrastructural observations.

Two cases of papillary cystic neoplasm (PCN) of the pancreas occurring in 18- and 34-year-old women are described. In both, the clinicopathologic features were typical. Immunohistochemical staining for neuron-specific enolase (NSE), S100 protein (S100), alpha-fetoprotein (AFP), carcinoembryonic antigen (CEA), and several peptide hormones was negative. Alpha-1-antitrypsin immunoreactivity was prominent, and its significance is discussed. Ultrastructural features were most compatible with partial acinar differentiation. Of the well-described cases of PCN, approximately 95% have occurred in women between the ages of 12 and 35, suggesting a role for hormonal factors in the pathogenesis of this tumor. Consequently, tumor tissue from one patient was assayed for estrogen and progesterone receptors: significant levels of high-affinity receptors were demonstrated for both hormones. Assays for the same receptors in five normal pancreases were negative. This constitutes the first reported measurement of these receptors in the PCN; the results indicate that the PCN may be another hormone-sensitive tumor.

Mitomycin-C nephrotoxicity: a clinico-pathologic study of 17 cases.

The potential nephrotoxicity of Mitomycin-C (MMC) was studied in 17 autopsies from patients treated with this drug, usually in association with other anti-neoplastic agents. Renal functional deterioration was present in seven cases while urinalysis showed an overt proteinuria and hematuria in six and four instances, respectively. Four of these patients also developed a severe microangiopathic hemolytic anemia (MAHA) and thrombopenia. Histological examination did not reveal residual tumor in three of them. However renal lesions similar to those observed in the hemolytic-uremic syndrome (HUS) were associated with glomerular mesangiolytic changes and nuclear atypias in glomerular and tubular cells. Immunofluorescence and ultrastructural studies confirmed these findings. In other patients, variable degrees of cellular atypias and of mesangiolysis were observed. The severity of the glomerular, vascular, and tubular lesions correlated with the total dosage of MMC received. The nephrotoxic potential of MMC seems to be delayed in onset and dose related.

Microangiopathic hemolytic anemia, renal failure, and noncardiogenic pulmonary edema: a chemotherapy-induced syndrome.

Following gastrectomy for locally advanced adenocarcinomas, three patients developed microangiopathic hemolytic anemia and renal failure shortly after completing courses of adjuvant chemotherapy with mitomycin and 5-FU. These complications progressed despite cessation of chemotherapy, and all three patients died of noncardiogenic pulmonary edema precipitated in two cases by blood transfusions. At autopsy, two patients had no residual carcinoma and all had a diffuse microangiopathy involving mainly the kidneys and lungs. There was intimal hyperplasia of many arterioles sometimes associated with complete occlusion of the lumen, prominent nuclear atypia in many capillary cells, and numerous capillary fibrin thrombi. Direct immunofluorescence studies revealed extensive fibrinogen-fibrin deposits in the vascular lesions. Chemotherapy-induced microangiopathic hemolytic anemia and renal failure may predispose patients to fatal episodes of noncardiogenic pulmonary edema that can be triggered by blood transfusions.

Inferior vena cava stenosis and Budd--Chiari syndrome in a woman taking oral contraceptives.

A young white woman who had been taking oral contraceptives for 8 yr presented with the Budd--Chiari syndrome. Stenosis of the hepatic segment of her inferior vena cava was observed and surgical correction was attempted, but she died of intravascular coagulation during the procedure. Postmortem examination showed myxoid intimal proliferation involving the hepatic, portal and femoral veins, and the inferior vena cava at the level of the stenosis. The literature describing inferior vena caval lesions causing the Budd--Chiari syndrome is reviewed, and the possible role of contraceptive steroids in the present patient is discussed.

False aneurysm of the ascending aorta presenting as an acute myocardial infarction: a late complication of aortocoronary bypass.

After coronary bypass surgery, a 40-year-old man developed disruption of the site of cannulation of the ascending aorta. The false aneurysm which ensued presented as recurrent episodes of spontaneous angina and myocardial infarction ending in coronary death 48 days after surgery. the aortic origin of all three permeable coronary bypasses were strangulated by the hematoma. Awareness of this unusual potential hazard is essential, since an early suspicion would lead to proper diagnostic interventions and reoperation.

Acro-renal-mandibular syndrome.

We report two female sibs, with severe split-hand/split-foot malformation associated with renal and genital anomalies. The patients also have severe mandibular hypoplasia and some other, minor anomalies. The relationship to the acro-renal "syndrome" in particular and to other phenotypes with similar malformations in general is discussed. Because of consanguinity in the parents, autosomal recessive inheritance seems likely. However, the presence of a septate uterus in the mother and a double collecting system in the only living sib could also suggest possible dominant inheritance with variable expressivity.

[A case of doubly differentiated epithelioma of the cervix (a mixed carcinoma) (author's transl)]. / A propos d'un cas d'épithélioma à double différenciation du col utérin.

Following a clinical case of doubly differentiated epithelioma of the cervix, we have reviewed the histological, epidemiological and clinico-pathological characteristics of this epithelioma. This lesion, which is particularly found in young patients, is often associated with pregnancy, and has a strong predilection for lymphatic spread. Finally, this tumour is very aggressive and seems to respond neither to radiotherapy nor to surgery.

Replacement therapy for inherited enzyme deficiency: liver orthotopic transplantation in Niemann-Pick disease type A.

Liver homotransplantation was attempted as replacement therapy in a 2-year-old patient with near total absence of sphingomyelinase activity of Niemann-Pick disease type A. Satisfactory function of the graft was observed until the death of the recipient from respiratory complication 2 years after transplantation. The clinical stigmata of the disease became less severe during the first 6 months after transplantation, with no further improvement thereafter. Sphingomyelinase activity was restored to near normal levels in serum, was present in cerebrospinal fluid and was maintained in the graft at normal or supranormal levels. No accumulation of sphingomyelin was observed in the transplanted organ as evaluated by histopathological and chromatographic studies. These findings support the interest of organ transplantation for long-term enzyme replacement in Niemann-Pick disease type A and similar lysosomal deficiencies.

[Anaplastic epithelioma of the thyroid gland 6 years after cervical irradiation for malignant lymphoma]. / Epithélioma anaplasique de la thyroïde 6 ans après irradiation cervicale pour lymphome malin

Carcinoma of the thyroid gland may develop after irradiation of the cervical region. It is well documented in patients who received radiation therapy to the neck in childhood but rarely when the therapy was given in adulthood. A case of an undifferentiated carcinoma of the thyroid gland occurring in an adult 6 years after irradiation of the cervical region for a malignant lymphoma is reported. The role of irradiation and the factors related to the development of carcinoma of the thyroid gland are reviewed.