Co-occurrence of two cases of progressive multifocal leukoencephalopathy in a natalizumab "infusion group".

We observed two cases of progressive multifocal leukoencephalopathy (PML) that occurred in the same "infusion group". The group consisted of four patients with relapsing-remitting multiple sclerosis (RRMS) who had been treated with natalizumab (NAT) in the same medical practice for more than four years at the same times and in the same room, raising concerns about viral transmission between members of the infusion group. DNA amplification and sequence comparison of the non-coding control region (NCCR) of JC virus (JCV) present in cerebrospinal fluid (CSF) samples from PML patients #1 and #2 revealed that the amplified JCV sequences differed from the JCV archetype. The NCRR of the viral DNA was unique to each patient, arguing against the possibility of viral transmission between patients. Statistical considerations predict that similar co-occurrences of PML are likely to happen in the future.

Significantly lower prevalence of Helicobacter pylori in uremic patients than in patients with normal renal function.

The objective of the study was to evaluate the prevalence of Helicobacter pylori in patients with different degrees of renal function. Two hundred and twenty consecutive patients requiring gastroscopy for upper intestinal symptoms were enrolled in the study: group I (normal renal function, n = 127), group II (chronic renal failure, creatinine clearance > 5 < 90 ml/min, n = 59), and group III (hemodialysis therapy, n = 34). On endoscopy, biopsy specimens were taken for analysis of H. pylori infection by urease test and histology. The prevalence of H. pylori in patients with renal dysfunction proved to be significantly lower than that in patients with normal renal function (22.6% vs 37%, P < 0.05). The incidence of ulcer disease in patients with normal renal function was higher than that in uremic patients (14.2% vs 10.8%, not significant). These findings indicate that uremic patients seem to be partly protected against H. pylori infection.

Disseminated lobular carcinoma - a predominantly pleomorphic lobular carcinoma of the whole breast.

Contrary to the usual distribution of lobular carcinoma in situ in a few lobules or terminal ducts, a case of disseminated lobular carcinoma is described occupying all lobules of the whole breast. The tumor developed in the left breast of a 61-year-old woman with the clinical symptoms of an increasing diffuse induration. The proliferating cells were predominantly pleomorphic with hyperchromatic nuclei and loss of cell cohesion. In 690 slides examined the tumor cells in lobules and ducts revealed various stages of proliferation with transition to invasive small cell carcinoma in 15 different areas. This disseminated lobular carcinoma appears to be an advanced form of primary cancer multicentric in one breast and originating from the basal cells of the lobules and terminal ducts due to a general carcinogenic stimulus.

"Diabetic mastopathy" in the male breast--a special type of gynecomastia. A comparative study of lymphocytic mastitis and gynecomastia.

Focal B-lymphocytic mastitis and focal fibrosis of the breast in young women have rarely been reported as a complication of longstanding insulin-dependent diabetes mellitus type I. We present two cases of "diabetic mastopathy" in male diabetics suffering from gynecomastia. Furthermore, these two cases were examined in comparison to a selected group of 6 patients showing gynecomastia with a marked inflammatory reaction, as well as to 24 non-selected cases of common gynecomastia. The lesion is interpreted as a diabetes-induced autoimmune reaction of the breast and may be regarded as a lympho-epithelial lesion. Its histopathological characteristics are a marked chronic periductal and perivascular mastitis with a predominance of B-lymphocytes, a focal homogenous fibrosis and so called "epithelioid stromal fibroblasts" within the fibrotic matrix. Our findings support the existance of "diabetic mastopathy" in the male and point out to the potentially misleading pattern of this benign tumor-like lesion simulating gynecomastia.

[Hamartoma of the breast. Comparative mammographic and histopathological studies]. / Das Hamartom der Mamma. Vergleichende mammographische und histopathologische Untersuchung.

Hamartomas of the breast are well circumscribed tumour-like lesions composed of fibroadipose and glandular tissue in variable degrees, depending on the patients' age. The nodules have a pseudocapsule and can easily be enucleated surgically. They may be recognised on mammography by their contours and density. Clinically they appear as painless areas of induration most frequently in the fourth and fifth decade of life. Pathogenetically it is assumed that they are an additional sprout of the breast, comparable to an "intra-glandular polymastia".

Papillary tumor of the temporal bone.

Papillary tumors of the middle and inner ear have been interpreted histogenetically in many ways. In 1989 Heffner proposed the endolymphatic sac epithelium as a possible origin. These rare tumors are clinically aggressive and can cause extensive temporal bone destruction. Because of this behavior, endolymphatic sac tumors (ELST) were classified as low-grade adenocarcinomas, although metastasis has not yet been documented. Two papillary neoplasms of the temporal bone are presented, which we believe are examples of adenomatous tumors arising from the epithelium of the endolymphatic sac. One was associated with a pituitary adenoma. A third case of a papillary middle ear neoplasm is described that shows histologic features similar to the other two, but it was located in the tympanum and had no connection to the endolymphatic sac. This report focuses on clinical, radiologic, and histologic findings of papillary tumors of the temporal bone with additional emphasis on modern concepts of histogenesis and aspects of differential diagnosis.

[Effect of low dose alcohol concentration on saccadic eye movements. Infrared reflection technique for recording oculomotor reactions with reference to dangerous traffic situations]. / Wirkung niedriger Alkoholkonzentrationen auf sakkadische Augenbewegungen. Infrarotreflexionstechnik zur Erfassung okulomotorischer Reaktionen bei Betrachtung gefährlicher Verkehrssituationen.

Saccadic eye movements were registered under different ethanol concentrations (0 g/kg, 0.5 g/kg, 1.0 g/kg) using an infrared reflexion method. On the basis of photographic pictures conflict situations of a car driver were simulated. 12 young car drivers were exposed for 10 sec to four pictures, all under different ethanol concentrations. The eye movements were registered and analyzed by a computer. The amplitude of saccades, their number, duration and velocity as well as the points of fixation and the scanning of the pictures were qualitatively and quantitatively analyzed. All persons demonstrated deficits in all parameters under 1 g/kg blood ethanol concentration. Nearly all results were reduced under 0.5 g/kg in comparison to 0 g/kg situation. In conclusion, low dose ethanol influence the saccadic eye movements. On the other hand the study could demonstrate a new technique (infrared reflexion technique), which allows the qualitative and quantitative recording of the influence of ethanol as well as peripheral as cortical structures of the brain. The exposition to photographic pictures demonstrating dangerous traffic situations simulate real traffic situations and allows to transfer the laboratory results to real situations.

Metal clip as a nidus for formation of common bile duct stone following laparascopic cholecystectomy.

A 83-year-old woman came to our hospital with fever up to 38.5 degrees C, abdominal pain and jaundice. She had undergone a cholecystectomy for symptomatic gallstones in 1992. The endoscopic retrograde cholangiography showed a dilated common bile duct with a free floating surgical clip with stone formation around this foreign body. After endoscopic sphincterotomy the extraction of the foreign body was successful. We found a two cm cholesterol stone the nidus of which was built by a Filshie-clip. The post endoscopic course was uneventful.

[Pathogenesis of endocarditis verrucosa simplex in the newborn (author's transl)]. / Zur Pathogenese der Endocarditis verrucosa simplex bei Neugeborenen.

Verrucous endocarditis in the newborn is not only a rare but also a problematic disease as concerns its etiology and pathogenesis. In the older literature septic infections of mother and child were said to be the causative factors. Recent experiences with the pathophysiology and pathomorphology of shock have also shown new aspects of the origin of this kind of endocarditis. Two cases from our own experience are compared with 7 cases from the literature. In the maternal history of gestation there was no infectious disease which could explain the endocarditis of the newborn. A short time after delivery the children showed a respiratory distress syndrome and died between 1.5 hours and 3 days after birth. At autopsy verrucous vegetations up to 8 X 5 X 4 mm in diameter were found on the tricuspid valve in 8 cases, once on the mitral valve and twice on both mitral and tricuspid valve. Malformations of the hearts were not noted. Histologically the fresh vegetations consist of clotted platelets and a fibrin network. At the base of the verruca the valvular leaflets show an edema, occasional fibroblastic changes and tiny lympho-histiocytic infiltrates in the subendothelial layer. Extracardial findings are hyaline membranes of the lungs and microthrombi in small vessels of lung, heart, liver and kidney due to shock and consumption-coagulopathy. According to Mittermayer et al. (1971a, b) who studied endocarditis verrucosa simplex in adults and animal-experiments endocarditis verrucosa simplex of the newborn is probably caused by intrauterine or perinatal shock.

[Clinic and pathogenesis of the medial cervical cleft. A contribution to the differential diagnosis of the cervical cleft (author's transl)]. / Klinik und Pathogenese der medianen Halsspalte. Ein Beitrag zur Differentialdiagnose der Halsfisteln.

The midline cervical cleft is a rare malformation related to the cleft of the lower lip and mandible. In this group it is the least pathological form. The clinical and histological findings could lead to misdiagnosing, those as caused by fistulas of the neck. The knowledge of the atypical signs facilitates the correct diagnosis. Therapy ist total excision applying the principles of plastic surgery not only because of aesthetic but also functional reasons. Suture lines in horizontal directions assure best results.

[The rare case--neurilemmoma of the vocal cord]. / Der seltene Fall--Neurilemmom der Stimmlippe.

We present the case of a neurilemmoma of the vocal cord, which is a very rare tumour at this site. The tumour was completely removed by microlaryngoscopy. The 78-year old female is free of recurrence for 1 year. She does not show any sign of neurofibromatosis. Microscopic features and the literature are reviewed.

[Histopathologic breast diagnosis in view of consultant studies of the Mamma-Regisstry Fulda]. / Histopathologische Mammadiagnostik im Spiegel konsiliarischer Untersuchungen des Mamma-Registers Fulda.

The Mamma-Registry Fulda, founded in 1976 by the senior co-author, is a personal service for pathologists having problems with histological diagnosis of breast diseases. From nearly 7000 cases filed up to now we selected 1112 consecutive consultations from 1996 to 1999 for this study. The aims were a critical analysis of a "submission-profile" and for each case a comparison of submitters' diagnoses with that of the register to crystallize special fields of problems in histopathological diagnosis and to make a statement about quality standards which was shown in a raster of results. The submitted cases came from pathologists in university institutes (13.9%), city hospitals (49.0%), group practices (24.2%), and single practices (11.6%). The material consisted of selected paraffin-blocs in about two thirds and of slides only in less than one third. The sendings were accompanied by letters with sufficient information on history, clinical background, and gross findings in 72%, and in an additional rate of 17.1% by copies of the histological reports already given by the submitters to their clinicians. The main reasons for consultations were a primarily uncertain diagnosis (45.8%) or the request to affirm a more or less definite diagnosis (40.7%) in cases of rare lesions or differing judgements in the submitting institution. Each diagnosis of the registry was coded in a special diagnostic key. In a raster of results the diagnosis of each case was listed as identical (55.0%) when there was complete agreement between submitter's and register's result, as included (23.9%) when one of the differential diagnoses named by the submitter fitted the register's diagnosis, and as different (6.7%) when there was only agreement about the dignity of differently classified lesions. False positive (2.3%) and false negative (4.5%) diagnoses of submitters were subclassified as clinically irrelevant (2.6%) and relevant (4.2%). The most often missed diagnostic entity in the latter group was tubular carcinoma. Overall the results of the study justify awarding a high standard of quality to histopathological diagnostics.

[Diagnosis and therapy of fibromatosis in head and neck region (author's transl)]. / Zur Diagnostik und Therapie der Fibromatosen im Kopf- und Halsbereich.

Fibromatosis is a heterogenous group of soft tissue neoplasias, only some forms of which occur in the head and neck region. In the pathological classification it takes place between the fibroma and fibrosarcoma. With nodular fasciitis, it's subspecies myositis proliferans and aggressive fibromatosis exist differential diagnostic difficulties, especially as it's difficult to exclude fibrosarcoma. The benign myositis proliferans gives an impression of a malignant process by it's rapid proliferation, cell-polymorphia and high mitotic activity. The monomorphous histological picture and the slower proliferation of the aggressive fibromatosis, however can simulate a benign tumor, although thought to be semimalignant. 3-case-reports point out these characteristics. Complete excision is the therapy advised for all fibromatosis. In aggressive fibromatosis a large enough healthy area is the best condition to prevent recidives.

[Malignant degeneration of juvenile laryngeal papillomatosis?]. / Maligne Entartung einer juvenilen Larynxpapillomatose?

BACKGROUND: Spontaneous malignant transformation of laryngeal papillomatosis was in the past mostly negated, or the discussion in literature was rather toned down and reserved. Therefore, from a biological and prognostic point of view, HPV infection of the larynx seems to carry a different weight than a viral infection in genital region. According to general consensus, secondary, malignant transformation in juvenile papillomatosis occurs in irradiated patients and leads to the conclusion that radiation therapy of this disease is presently contraindicated. Because there is as yet no causal and curative treatment, repeated and frequent removal of papillomatous tissue by microlaryngoscopy may often be necessary to keep the airway patent. PATIENT, METHOD AND RESULTS: We diagnosed and treated an advanced laryngeal squamous cell carcinoma with lymphatic metastasis in a 50-year old male. Juvenile papillomatosis had been diagnosed already at the age of five, and at the patient's last presentation 5 years ago (age 45), typical clinical and histological features of laryngeal papillomatosis had been observed. Furthermore, virus infection of the papillomatous tissue (HPV-6/11) was proved by using the technique of in-situ hybridisation. Risk factors for malignant transformation, such as smoking, alcohol or radiation, were denied by the patient. CONCLUSIONS: From these aspects, a spontaneous, malignant transformation of laryngeal papillomatosis must be considered with regard to six similar observations in the German and English literature. In the reported case, a tumoural origin in the flat laryngeal mucosa in close neighbourhood to the former site of papillomas, is less probable, albeit not ruled out completely, since continuous changes from benign squamous papilloma to atypical, invasive tumour and a HPV-infection in the carcinomatous tissue could not be proved by in-situ hybridisation.