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This study deals with the pattern of estrogen receptors in 52 cases of invasive breast carcinoma by comparing the ligand-binding assays with dextran-coated charcoal (DCC) to an immunoperoxidase assay (IPE) technique in paraffin-embedded material from the same tumor. A modification of the IPE by adding cobalt chloride in the final reaction and counter-staining with eosin instead of hematoxylin was introduced. A significantly high correlation was found between the two compared methods (80.8% positivity by the DCC method and 80% by the IPE method). The correlation was very significant in cases where the immunohistochemical grading was higher than 4 (according to our semi-quantitative evaluation scale of 0-8), and for these cases we suggest the IPE method as highly specific and precise.

The histological findings in the first diagnostic biopsy of 50 patients with Kaposi's sarcoma (KS) were classified and were related to the clinical course. Forty-one patients had the "classic KS," of whom 31 had a benign course and 10 an aggressive disease. Nine patients had iatrogenically induced KS. Histologically the following subtypes were found: mixed type in 18 patients, spindle type in 16, early type in 14, and hemangiomatous and lymphangiomatous types in one each. We did not find any statistical relationship between the histological subtypes, degree of nuclear atypia, number of mitoses, and density of the inflammatory cell infiltrates in the KS lesions, and the clinical types and course of the disease. The results of this study do not support the possibility of a prognostic importance regarding the histological features of the early lesions in patients with classic or iatrogenic Kaposi's sarcoma.

Amelanotic malignant melanoma can present a diagnostic problem in histopathology, especially when it presents in an extracutaneous location. In such cases electron microscopy and/or immunohistochemistry are invaluable for diagnosis. A 63-year-old women with rectal bleeding was found to have an amelanotic malignant melanoma of the anal canal, with compatible clinical and gross pathology and light and electron microscopic findings. Tumor cells showed positive staining with antibodies to vimentin, S 100 protein, and HMB-45 (melanoma-specific antibody), but also with polyclonal antibody to carcinoembryonic antigen (CEA). Tumor cells failed to stain with monoclonal antibody to CEA. The nature and significance of CEA reactivity in malignant melanoma are discussed.

Dermatofibrosarcoma protuberans is an uncommon skin tumor with a high potential for local recurrence if not adequately excised. There are only two reports in the literature describing dermatofibrosarcoma protuberans that enlarged considerably during pregnancy. On the other hand, the new development or enlargement of dermatofibromas in pregnancy is well documented. We present 2 additional patients in whom a dermatofibrosarcoma protuberans appeared and grew rapidly during pregnancy. Immunohistochemical studies were negative for estrogen and progesterone receptors. The possible pathophysiology and surgical management are presented and discussed.

To emphasize the importance of early diagnosis in cases of müllerian adenosarcoma that appeared as benign-looking cervical polyps.We examined seven cases of müllerian adenosarcoma of the uterus in patients 14-63 years of age (median 39 years). Tissue protruding from the external os and an initial diagnosis of a cervical polyp were common findings for all patients. On repeated examination, all lesions were interpreted as müllerian adenosarcomas.Histologic examination demonstrated benign glands with a sarcomatous stroma, which typically formed periglandular cuffs of increased cellularity. The sarcomatous stroma was homologous in four cases and contained heterologous elements such as striated muscle, lipoblast, and cartilage in three cases; one patient had a sarcomatous overgrowth of stromal elements. The question of a müllerian adenofibroma versus adenosarcoma was raised in three cases with the general appearance of slit-like glands surrounded by a stroma with fibrosis and a low mitotic rate. Using the criteria of stromal cellularity--marked stromal atypia and a mitotic index of two figures per ten high-power fields--the cases were classified as adenosarcomas. The sarcomatous overgrowth, the presence of heterologous elements, and a high mitotic rate seem to be important prognostically.Gynecologists and pathologists should be aware of the difficulties and delay in the diagnosis of müllerian adenosarcoma when the tumor presents as a benign-looking cervical polyp.

Fibrous hamartoma of infancy is an uncommon lesion of uncertain histogenesis. Three cases were studied by light microscopy, electron microscopy, and immunohistochemistry. Two histologic variants are presented. Ultrastructurally, fibroblasts and myofibroblasts were found in the fascicular-fibroblastic areas while primitive mesenchymal cells were found in the immature-appearing regions. Vimentin positivity was noted in both areas, whereas desmin and actin positivity was found mainly in the fascicular-fibroblastic regions. The lesion appears to fulfill the criteria for its characterization as “hamartoma”.

Thirty-three children with idiopathic nephrotic syndrome who underwent kidney needle biopsy were reevaluated. The male to female ratio was 2:1, and a preponderance of North-African Jewish and Arab origin over Ashkenazi Jewish origin was noted. There was a positive correlation between the severity of glomerular changes and prognosis among the 10 cases with minimal change disease (MCD) and the 23 with focal segmental glomerulosclerosis (FSGS). On long-term follow-up (mean over 11 years) chronic renal failure developed in none of 10 MCD patients, 1 of 12 FSGS patients with mild glomerular sclerosis, 1 of 7 FSGS patients with moderate glomerular sclerosis and 3 of 4 FSGS patients with severe glomerular sclerosis. Prognosis of patients with mild glomerular sclerotic lesion on light microscopy was substantially not worse than the prognosis of patients with mild glomerular alterations only on the electron microscopic study (MCD-B). Thus, both pathologically and prognostically, there was a continuous spectrum from 'pure' MCD (MCD-A) to FSGS with severe glomerular sclerosis. Glomerular changes confined to the origin of the proximal tubule ('tip' changes) were seen only in 4 patients and did not have a distinct prognostic significance. No case of peripheral location of the sclerotic segment within the glomerulus was found in our series of FSGS, and therefore no correlation between location of segmental sclerosis and prognosis was feasible.

Fechtner syndrome, a variant of Alport syndrome, was first reported by Peterson et al. [1985]. It is characterized by nephritis, hearing loss, eye abnormalities, macrothrombocytopenia, and leucocyte inclusions, present in varying combinations in several members of the same family. This is the second family reported; 16 relatives are affected. The clinical manifestations of the syndrome are delineated. The pattern of inheritance is autosomal dominant. The hematologic abnormalities are similar to those detected in May Hegglin anomaly. They are present in every affected relative and may be present at birth. The feasibility of prenatal diagnosis is discussed.

Summary The combination of systemic lupus erythematosus, Sjögren's disease and severe diffuse glomerular nephritis has only rarely been reported. A 14-year-old girl is described with lupus nephritis in whom co-existent clinical and histological features of Sjögren's syndrome were found. These include bilateral parotid enlargement, xerostomia, increased serum amylase, reduced salivary secretion and lymphocyte infiltration of both salivary glands and kidneys. The co-existence of systemic lupus erythematosus with Sjögren's syndrome is discussed together with a consideration of pathogenesis.

Four children from two related families are described with a clinical picture characterized by the onset of asymptomatic proteinuria with subsequent nephrotic syndrome in infancy, and progression to renal failure and death before the age of three years. The clinical picture and the renal pathological studies were consistent with the entity described by Habib as infantile mesangial sclerosis. We propose that this entity possibly represents a genetic disorder which is transmitted as an autosomal recessive trait.

A series of 120 breast biopsies and mastectomy specimens originally diagnosed as carcinoma was reviewed in order to emphasize the differences between lobular carcinoma in situ and cancerization of lobules. Criteria for differential diagnosis between the two types of lobular lesion are proposed. In the reviewed material 15 (12.5%) cases of lobular carcinoma in situ and 64 (53.3%) of lobular cancerization were found. The carcinoma in situ usually co-existed with other types of breast carcinoma, such as intraductal carcinoma, cancerization and invasive carcinoma of different types.

SUMMARY The mechanism of skin hyperreactivity (pathergy) in Behcet's disease is unknown. It has been suggested that the response is due to an Arthus-like reaction. In 18 patients with active Behcet's disease, pathergy was induced and the skin biopsied after 24 h. The histological reaction was that of mild to intense round cell infiltration, perivascular in type. Direct immunofluorescence failed in all 18 patients to demonstrate the local presence of immunoglobulins and complement at the site of lesion. A marked increase in the number of mast cells was observed at the site of reaction and scattered throughout the dermis, the role of which is to be further investigated. The study failed to demonstrate a humoral mechanism in the production of the cutaneous hyperreactivity.

10 of 16 (62.5%) patients with granuloma annulare presented microangiopathic changes in the skin. The incidence in control groups was 100% in diabetic patients, 40% in old-age patients not known as diabetics and 0 in young victims of road accidents. The frequency of the vascular findings does not seem to be shared equally by the two types of granuloma annulare, the generalized and the localized type. In the first, as in the diabetic group, microangiopathy was observed in all patients examined. Whereas, in the second it was detected in only 2 of the 8 patients. It is concluded, therefore, that from the point of view of their relation to a diabetic state, the two forms of the disorder differ from each other.

Summary A case of medullary cystic disease and chronic renal failure with severe sodium wastage is described. Urinary sodium and calcium concentrations were fixed at a level approximating plasma ultrafiltrate. Renal salt loss was flow dependent. It is suggested that the main cause of the severe salt loss was a tubular and medullary defect of sodium transport; hyperfiltration and increased osmotic load per nephron played a contributory role.In the patient described, calcium was excreted in the urine in quantities that were unusually large for chronic renal failure but were commensurate with the renal salt loss.

A case of a newborn child with extensive congenital absence of epidermis is presented. Following our review of the literature, this appears to be the most extensive case described. Some possible etiological causes of this rare disease are briefly discussed.

Postoperative jaundice is an occasional complication of major surgery. Four illustrative cases are described and a mechanism of development is suggested. The jaundice may seem to be biochemically of the obstructive type. Most of the bilirubin is of the conjugated type. The disturbance of liver function can be caused by several factors, including massive transfusion, hematoma in tissues, shock, heart failure, anoxia, infection and perhaps medications. Postoperative jaundice by itself is not of cardinal importance in deciding the prognosis of the patient.

Four initially misdiagnosed cases of thyroid cancer or purported thyroideal malignancy are described. In each, aspirative cytology was instrumental in establishing the true nature of the lesion.

While immigrants from Morocco and Romania make up 7 and 6%, respectively, of the Israeli population, one-third of all postnecrotic cirrhosis patients come from Morocco and another third from Romania. The rate of hepatitis B virus (HBV) infection, as expressed by serum hepatitis B surface antigen, anti-HBs, and anti-HBc, was compared in immigrant patients with postnecrotic cirrhosis and in age and sex matched controls from the same countries. Among 19 Moroccan immigrants with postnecrotic cirrhosis 74% showed evidence of HBV infection. This was not significantly different from the prevalence of HBV infection (64%) in a matched control group of 61 Moroccan individuals. In the 26 Romanian cirrhotics, however, a significantly higher prevalance of HBV infection than in the 60 matched controls was observed (54 and 23%, respectively, p less than 0.01). The difference was detected only when anti-HBc was looked for in addition to hepatitis B surface antigen and anti-HBs. These results indicate that: 1) HBV infection and postnecrotic cirrhosis are associated in Romanian immigrants to Israel. 2) Association between HBV infection and cirrhosis in Moroccan immigrants cannot be shown because of the high infection rate in the control population. 3) Anti-HBc is often the only marker of HBV infection in cirrhotic patients, and should be examined in addition to hepatitis B surface antigen and anti-HBs in order to get meaningful results.

Light microscopy findings in early Wilson's disease (hepatolenticular degeneration) may be normal even when special cytochemical stains are used. We present a case of Wilson's disease in which light microscopy was negative, while electron microscopy showed the characteristic changes in the hepatocytes. Since low serum ceruloplasmin levels and high urinary copper excretion are not by themselves definite proof of Wilson's disease, and since copper content of the liver is not universally measured, electron microscopy examination of liver tissue appears to be a worthwhile additional tool for the early diagnosis of Wilson's disease.

Distant bone metastases in cases of intracranial meningiomas are uncommon. Two patients with meningiomas showing malignant histological features are described, in both of whom bone metastases were observed many years after cranial surgery.

The clinical picture and histopathological findings in 11 infants with infantile nephrotic syndrome are presented. Three infants, who presented in the perinatal period, had congenital nephrotic syndrome (Finnish type); six of eight infants who presented in the 1st yr of life had mesangial sclerosis nephrotic syndrome, while the remaining two had minimal change nephrotic syndrome. Four of the six infants with mesangial sclerosis nephrotic syndrome were from one family. All patients died within 2 mo to 2 yr after presentation.

Two patients in whom various immunological phenomena obscured and delayed the diagnosis of subacute bacterial endocarditis (SBE) are described. Immunological abnormalities usually correlate well with the chronicity and severity of the disease, as is demonstrated here. The mechanisms underlying the various immunological phenomena are discussed. The importance of alertness to the possibility of SBE in cases of disease manifesting itself as an immunological disorder, thus achieving early diagnosis and early antimicrobial treatment, is stressed.

Ascites induced by hybridoma cells following their injection into pristane-primed peritoneal cavities of BALB/c mice is often associated with the formation of intra-abdominal tumors related to the transplanted cells. These tumors and adjacent abdominal and extra-abdominal organs were excised and examined by electron microscopy. Clusters of viral particles were seen in nearly all cells composing the hybridoma tumors. The viral particles were usually present in rough ER cisternae of the transplanted tumor cells and at times were observed budding from the cisternal membranes. Individual viral units were doughnut shaped, 80-85 nm in diameter, with two concentric shells of which the outer one was more electron dense. No exogenous viral particles were detected in the peritoneally growing hybridoma cells. All non-tumor tissues examined such as liver, spleen, lung, brain and circulating WBC were free of virus. These findings provide further evidence of viral contamination of hybridomas and demonstrate the mode of persistence of such contamination when cells are placed in-vivo.

Inbred X/Gf mice are reported to have a very low incidence of spontaneous and induced tumors. Experiments were performed to determine whether the cells of the X/Gf mouse are themselves resistant to in vitro oncogenesis or the organism as a whole is surveyed by a homeostatic mechanism that prevents the expression of potential neoplasia. Cultures of X/Gf or C57BL/6J embryonic fibroblasts were exposed to the chemical carcinogens 7,12-dimethylbenz[a]anthracene and benzo[a]pyrene to test whether the cells of the X/Gf mouse are themselves resistant to oncogenesis. The chemical carcinogens caused transformation of both X/Gf and C57BL/6 fibroblasts into cells that demonstrated altered growth patterns in liquid culture and grew in a clonal fashion in soft agar. Cloned transformed cells were shown to be malignant and resulted in the growth of sarcomas in vivo. X/Gf mice were decidedly more resistant than were C57BL/6 mice to their respective clones of transformed cells. The resistance to neoplastic growth demonstrated by the X/Gf mice was inherited by (C57BL/6J X X/Gf)F1 mice that were resistant to the growth of X/Gf-transformed and C57BL/6-transformed cells at cell doses at which the parental X/Gf mice had survived but at which the C57BL/6 had succumbed to progressively growing tumors. It has thus been established that the tumor resistance of the X/Gf mice is not based on the resistance of isolated cells from these animals to malignant transformation, but rather is attributable to an in vivo homeostatic mechanism that prevents tumor growth.

An unusual case of a müllerian adenosarcoma of the peritoneal mesothelium in a 32-year-old pregnant woman is described. On emergency laparotomy, a tumor was found in the pelvis connected with the right broad ligament only, and was removed. Light and electron microscopic examination showed a rare example of an extrauterine müllerian adenosarcoma composed of a stromal sarcoma, muscle tissue resembling primitive myoblasts, and sex-cord-like tubular lumens. The patient was followed, and after 22 months the primary surgery was reexplored. On laparotomy, no evidence of disease was found, and the ovaries were tumor-free. Histologic examination of the pelvic peritoneum demonstrated small areas of adenosarcoma resembling elements of the previous tumor. Müllerian adenosarcoma is a rare tumor of müllerian origin, and its less aggressive behavior as compared with mixed müllerian sarcoma is warranted. The better prognostic outlook of müllerian adenosarcoma is probably related to the histologic structure, but depends in addition on the size of the tumor and the site of origin. No case of peritoneal origin of müllerian adenosarcoma has been previously reported; we discuss this unusual location.

VIF3 is a hybridoma-derived mouse IgG monoclonal antibody (MoAb) generated in a fusion with the use of a malignant fibrous histiocytoma as the immunizing agent and shown to recognize a 70-kilodalton antigen expressed within connective tissues. Of 55 human tissue culture cell lines tested by indirect immunofluorescence, VIF3 was shown to bind to 20 of 35 (57%) sarcomas, 4 of 9 (44%) normal fibroblasts, and none of 11 carcinomas and other neoplasm-derived cell lines. A panel of over 259 human frozen tissue sections obtained from surgical pathology specimens, postmortem studies, and elective abortions was used to further determine the histopathologic specificity of VIF3. MoAb VIF3 was found to bind to 15 of 19 (79%) human sarcoma tissue sections tested. It was also shown to recognize an antigen expressed on a subset of fibroblasts dispersed within the stroma of carcinomas obtained from all 46 patients tested, as well as a subset of cells within 3 of 10 benign hyperplastic tissues (30%). VIF3-positive cells were detected in all 60 fetal tissues tested including amniotic membranes, placentas, and umbilical cords. In contrast, fibroblasts of normal adult tissues tested stained infrequently (22/97 or 23%) with this reagent. The results confirm that this MoAb is directed against a human connective tissue-specific marker. VIF3 detects a marker appearing on fetal fibroblasts that is typically not present in normal adult tissues, but reappears in association with neoplastic diseases. MoAb VIF3 therefore defines a fibroblast-oncofetal antigen and as such may serve as a probe for immunopathologic studies.

Neonatal intnaabdominal calcification has been reported in the radiologic literature [1-9]. It is usually caused by intraluminal or intrapenitoneal meconium and is often associated with bowel obstruction by atnesia on meconium ileus. This report describes a case of extensive intramural intestinal calcifications associated with multiple bowel atnesia. This type of calcification is rare, and its radiologic appearance has not yet been reported in detail.

A case of chronic uveitis with shiny spots in the iris is described. Histologically the shiny spots proved to be Russel bodies. The nature of the Russel bodies is discussed.