[Uncommon case of paraganglioma syndrome in combination with malignant paraganglioma]. / Seltener fall eines paragangliom-syndroms mit auftreten eines malignen paraganglioms.

BACKGROUND: Combination of paraganglioma syndrome and malignant paraganglioma is an uncommon disease worldwide. PATIENT: We report the case of a 69-year old man with a jugular-tympanal paraganglioma, who underwent surgery. Histopathological examination revealed a malignant paraganglioma. An other contralateral carotid-body and a tumor in the thyroid gland had been discovered during staging. The molecular results confirmed the existence of a paraganglioma syndrome. DISCUSSION: A malignant paraganglioma based on a hereditary paraganglioma syndrome is a rare described case in literature. On the background of the literature we discuss the pathology, pathogenesis, diagnostic and therapy of this disease.

[Unclear per anum bleeding during pregnancy]. / Unklare peranale Blutung in der Schwangerschaft.

A 30-year-old, 7-months pregnant woman presented with per anum bleeding. Rectoscopy showed the cause to be a polyp the size of a fingertip 9 cm from the anus. Since the bleeding stopped spontaneously and therefore the need for radical intervention, and due to the advanced pregnancy stage, no biopsy was done. About 1 year later, the same patient experienced sharp pains and anal bleeding during defecation. A poorly defined neuroendocrine carcinoma was diagnosed. Seeking a prognosis optimal for the newborn child, the interdisciplinary decision was made for primary surgery with adjuvant chemotherapy. Other options for this malignancy during and after pregnancy are also discussed.

Hepatoid thymic carcinoma: report of a case.

We describe the clinicopathologic findings in a so far unrecognized thymic tumor. The tumor occurred in a 70-year-old woman with respiratory distress but neither myasthenia gravis nor other symptoms. Metastases or another primary tumor were absent. The well-circumscribed neoplasm was located in the thymic region, measured 18 x 12 x 8 cm, and showed a homogeneous, tan-colored, soft cut surface. By histology, the tumor lacked a true capsule and a lobular growth pattern, was almost devoid of stroma, and infiltrated among remnant thymus lobules. The polygonal tumor cells formed solid sheets, trabeculae, or occurred as single cells that resembled hepatocytes. Proliferative activity was low. Portal structures, sinuses, and bile were absent as were areas of conventional thymoma, adenocarcinoma, or germ cell tumor. The tumor expressed cytokeratins 7 and 19, alpha1-antitrypsin, alpha1-antichymotrypsin, and hep-Par-1. Alpha-fetoprotein (AFP), human beta-chorionic gonadotropin (beta-HCG), placental alkaline phosphatase, CD5, CD30, CD31, CD34, CD45, CD68, CD99, S-100, HMB45, desmin, actin, or neuroendocrine markers were not expressed, and intratumorous CD1a+ or TdT+ immature T cells were absent. AFP was repeatedly undetectable in the blood. Mediastinal tumor recurrence was detected 6 months after surgery. Following radiochemotherapy, the patient has remained free of disease for 26 months. We conclude that this tumor is a thymic carcinoma (WHO type C thymoma). A diagnosis of hepatoid yolk sack tumor appears unlikely considering absence of a bona fide germ cell component, lack of AFP expression, and the patient's female gender. Because of its morphologic and immunohistochemical features, we propose the term "hepatoid thymic carcinoma" for this new type of thymic carcinoma.

[Synovial sarcoma of the pericardium]. / Synovialsarkom des Perikards.

A precordial tumor of the pericardium was radiologically diagnosed as the cause of an untypical clinical picture of heart disease in a 41-year-old soldier. As the patient had an increased asbestos exposure due to his profession, he was admitted to operation under the tentative diagnosis of a pericardial mesothelioma and the question of an occupational disease (BK 4105). Microscopic and immunohistochemical findings are compatible with the diagnosis of a synovial sarcoma of the pericardium. The present immunohistochemical marker spectrum allowed a reliable differentiation between synovial sarcoma and pericardial mesothelioma, which is more frequent than synovial sarcoma. The epithelioid component was determined using the following antibodies: MNF 116, CK 19, CK 7, EMA and Ber EP-4 were positive while Factor VIII, Calretinin, S100, Vimentin, CEA, CD 31, bcl-2 and HBA-71 were negative. The sarcomatous component was determined with antibodies to Vimentin, bcl-2 and HBA-71 which were positive, and to MNF 116, CK 19, CK 7, Factor VIII, Calretinin, S100, EMA, CEA, Ber EP-4 and CD 31 which were negative. Synovial sarcomas of the pericardium in the lower anterior mediastinum or the myocardium are exceedingly rare. A causal relationship between tumor formation and an increased asbestos exposure--similar to the epidemiologically based experiences with pericardial mesothelioma--is not likely. Primary extrapericardial synovial sarcoma could be excluded.

[Thoracic actinomycosis]. / Die thorakale Actinomykose.

Thoracic actinomycosis is a rare disease without characteristic clinical signs. Approximately 90% of patients suffering from thoracic actinomycosis have undergone diagnostic and therapeutic procedures based on a wrong diagnostic hypothesis (malignancies 35-44%, other pulmonary disorders 33-35%). The opportunities for a timely and adequate diagnosis by the use of clinical examination, laboratory studies, microbiology studies, radiologic imaging or invasive measures are limited. In 85%, thoracic actinomycosis has not been identified prior to thoracotomy, open biopsy and histological examination. Based on a wrong diagnostic hypothesis, resective thoracic surgery according to the principles of oncologic surgery can hardly be avoided. We report on a 43-year-old male suffering from actinomycosis of the left hemithorax. Clinical signs, differential diagnosis, treatment and clinical course are described. The role of surgery in the treatment protocol of thoracic actinomycosis is discussed. In pulmonary and pleural disorders of unknown origin, differential diagnosis should include thoracic actinomycosis as early as possible. Due to the considerably high mortality rate of untreated disease, the outcome of thoracic actinomycosis can only be improved by a timely and combined employment of surgical and antibiotic therapy.

[The conjunctival epithelium of dogs. Light and electron microscopic investigations]. / Das Konjunktivalepithel des Hundes. Licht- und elektronenmikroskopische Untersuchungen.

The conjunctiva of the dog has a smooth surface on the inside of the upper and lower lid and of the bulbus oculi. In these areas the conjunctiva is covered by a stratified squamous epithelium, the polygonal surface cells of which have microvilli and microplicae which are different in number and show a great variety of shapes. In the passage towards the fornix conjunctivae a stratified cuboidal epithelium is to be discerned. In the fornix itself, the conjunctiva is arranged in small folds which are protruded by goblet cells and the openings of lacrimal glands. Here a stratified cuboidal or columnar epithelium can be observed which at the electron microscopic level shows in all epithelial layers--mostly however in the stratum superficiale--morphological signs of enhanced metabolic activities. The bulbar surface of the third eyelid which is covered by a stratified squamous epithelium has a three-dimensional moulded pattern of pads and grooves. The structure of the conjunctival epithelium to some extend is age dependent.

Contribution of thallium-201-SPECT to the grading of tumorous alterations of the brain.

Single photon emission computed tomography (SPECT) with thallium-201-chloride (201Tl) was used in 22 patients to assess the grade of malignancy of brain tumors. Low- and high-grade malignant gliomas could be well differentiated by calculating the Grade Index (GI), i.e., 201Tl uptake in the tumor area relative to a contralateral brain region. Low-grade gliomas (WHO-grade I-II) usually showed a GI of < 1.5. Tumors classified histologically as high-grade malignant (WHO-grade III-IV) had GI values greater than 1.42 and a mean value of 1.89. Until labelled amino-acid tracers for gamma-cameras become commercially available, thallium-201 brain-SPECT can provide an independent and complementary method to CT/MRI for the differential diagnosis of grading of brain tumors. This simple technique can help to reduce sampling errors during needle biopsies of brain tumors, particularly of high-grade lesions incorrectly graded as low-grade tumors due to inadequate biopsy material. In addition, pre- and post-therapy studies can influence the strategy of therapy itself and allow an early detection of recurrences.

[Quality control by autopsy. How often do the postmortem examination findings correct the clinical diagnosis?]. / Qualitätskontrolle durch Autopsie. Wie häufig korrigiert der Obduktionsbefund die klinische Diagnose?

To ascertain whether autopsies still provide important information in the face of modern diagnostic procedures, the findings in 780 autopsies (543 men and 237 women: mean age 58 [1-94] years), performed between 1977 and 1990, were compared with the clinical diagnoses. Autopsy confirmed the clinical diagnosis in 74.9% of cases, a clinically not diagnosed basic disease was demonstrated in 13.6%, and a not recognized final complication in 11.5%. In 9.7% of autopsies previously unrecognized but clinically important additional diseases were found. There was no decrease in the proportion of unrecognized basic diseases in the course of the observation period. It was unrelated to the duration of hospital stay, but increased with increasing age of the patients. These observations indicate that even nowadays, in the age of highly technical medicine, autopsies point to any limitations of diagnosis and are thus of importance for clinical quality control.

[Asymptomatic paraganglioma of the bladder wall with unusual extensive submucous vascular proliferation]. / Asymptomatisches Paragangliom der Harnblasenwand mit ungewöhnlich ausgeprägter submuköser Gefässproliferation.

Paraganglioma of the urinary bladder is a very rare tumor, which is usually diagnosed on the basis of its endocrine symptoms because of the excretion of vasoactive substances. One case of asymptomatic paraganglioma detected by chance is described, it was misdiagnosed as hemangioma because of its marked suburothelial vessel proliferation.

Experimental reovirus myocarditis in newborn mice. Electron microscopic observations.

Reovirus is a double-stranded RNA-virus which induces myocarditis in newborn mice. Due to the large diameter of the viral particles (70-75 nm) it can be detected by electron microscopy. Subcutaneous inoculation of 0.05 ml reovirus type 3 (TCID50-titer: 10(8.5)/ml) into newborn NMRI-mice (12-18 h after birth) caused a grey-yellow mottling on the ventricular surface first seen on the 5th day after birth. At the same time muscle fiber necrosis was observed which increased with time. Electron microscopic investigations of the diseased heart muscle disclosed a marked interstitial oedema, swelling of the tubular system and sarcoplasmic reticulum, and degenerative changes in the mitochondria of individual myocardiocytes as early as the 2nd post-inoculation day. Simultaneously, an enlarged Golgi-apparatus and an increasing number of lysosomes, partially exhibiting acid phosphatase activity, was detected in the perinuclear region of ventricular myocardiocytes. On the 5th day after infection, viruses were detected either within single membrane vesicles, dispersed in cytoplasm or as aggregated clusters in the perinuclear region. These in vivo electron microscopic findings correspond with observations of virus propagation in cell-culture systems.

Comparative effects of trypsin, collagenase and mechanical harvesting on cell membrane lipids studied in monolayer-cultured endothelial cells and a green monkey kidney cell line.

Experiments are presented in which membrane lipids of endothelial cells in monolayer culture were labelled with [14C]linoleic acid. Approx. 90% of the radioactive label were incorporated into phospholipids. A comparison of various harvesting methods showed that during the disruption of the labelled endothelial cell monolayer, 0.25% trypsin and 0.125% trypsin (+0.01% EDTA) released 650 and 470% more radioactivity, respectively, than did 0.01% collagenase (+0.01% EDTA). Parallel studies were performed on a green monkey kidney cell line. In this case, 0.25% trypsin released 520% more radioactivity than did 0.1% collagenase (+0.01% EDTA), although 0.125% trypsin in the presence of EDTA (0.01%) was much less traumatic than trypsin alone, the released radioactivity being of the same order of magnitude as that for collagenase. Morphological studies on endothelial cell cultures failed to reveal any distinctive differences in surface morphology following the various enzyme treatments. The results suggest that collagenase treatment of endothelial cell monolayers is the least traumatic harvesting or subculturing method as far as the integrity of the lipids in the cell membrane is concerned.