Heart transplantation for restrictive cardiomyopathy: development of cardiac amyloidosis in preexisting monoclonal gammopathy.

A 54-year-old woman died 6 months after heart transplantation for treatment of restrictive cardiomyopathy. A monoclonal gammopathy without other signs of malignant disease was present preoperatively, and up to 6 weeks before transplantation no evidence of amyloidosis was established in the rectal, bone marrow, and cardiac specimens. At autopsy there was amyloidosis type AL in the kidneys, bone marrow, liver, spleen, recipient atrium, and donor heart. Retrospectively, the explanted heart also revealed amyloidosis. We conclude that in patients undergoing heart transplantation for treatment of restrictive cardiomyopathy with a preexisting monoclonal gammopathy, a thorough evaluation, including multiorgan biopsy for amyloidosis with electron microscopic workup, should be performed.

Evaluation of a porcine internal mammary artery (No-React II) as a small-diameter conduit.

BACKGROUND: The patency of biologic small-diameter vascular grafts in the aortocoronary position is still unsatisfactory. Most of the studies suggest that xenografts are to be avoided as an aortocoronary bypass. METHODS: The porcine internal mammary artery treated by the No-React II procedure was developed for use as an alternative coronary artery bypass conduit. The attempt of this study was to evaluate the patency and histologic changes of the porcine internal mammary artery in animals. Five calves underwent coronary artery bypass grafting with a porcine internal mammary artery graft to the right coronary artery. After euthanasia of the animals 103 days later, the samples of these grafts were studied morphologically for patency, structural changes, calcifications, and inflammatory and immunologic response. RESULTS: One animal died during the procedure as result of acute thrombosis of the porcine internal mammary artery graft. In the other 4 animals all grafts became occluded. In the histologic sections of the grafts we noted multiple calcifications and a host-graft immunologic reaction (severe chronic rejection). CONCLUSIONS: The present study demonstrates a very poor experience with the porcine internal mammary artery (No-React II) conduit. We do not recommend this prosthesis for clinical use in humans.

Feasibility study of postoperative adjuvant chemotherapy and radiotherapy for soft-tissue sarcoma.

In a nonrandomized trial, postoperative, adjuvant, combined chemotherapy and radiotherapy were given to 17 patients with high-grade soft-tissue sarcomas. All patients had undergone conservative limb-sparing surgery. Soft-tissue sarcomas were localized in the extremities (13 patients), superficial trunk (3), and neck (1). In all, 13 patients received 50 mg/m2 doxorubicin and 5 g/m2 ifosfamide with mesna uroprotection for a total of 6 cycles and 4 patients received CYVADIC (cyclophosphamide/vincristine/doxorubicin/dacarbazine). Chemotherapy was started immediately after wound healing. Irradiation using the shrinking-field technique was commenced 3-7 days following chemotherapy; a total dose of 65 Gy was applied. The major side effects of chemotherapy were nausea and vomiting [17 of 17 patients, 5 experiencing World Health Organization (WHO) grade 3 toxicity and 1, WHO grade 4], leukopenia of <3.0 x 10(9)/l (17 patients), and leukopenia of <1.0 x 10(9)/l (7 patients). The median leukocyte nadir was reached on day 11 (range, days 7-16). The duration of critical leukopenia did not exceed 1 week. Reversible alopecia occurred in all patients. Temporary cardiomyopathy was recorded in 1 patient. Following radiotherapy, 11 episodes of epitheliolysis and 1 case of moderate lymphedema were documented. There was no life-threatening condition. After a follow-up of 58 months, the outcome was as follows: disease-free survival, 9 patients; distant metastases, 7; local recurrence, 1. Excluding 3 patients who entered the study after undergoing surgery for local relapse, the rate of distant metastases was 36%. In summary, the postoperative use of chemotherapy/radiotherapy is feasible, producing relevant but manageable toxicity. This combination results in effective local tumor control with good functional results following limb-sparing surgery. The incidence of distant metastases, however, is high.

Limits of coronary artery surgery. Clinical and postmortem findings.

In the first series of 90 continuous patients with coronary artery disease 9 patients died after aorto-coronary bypass procedures (10%). There were 7 operative deaths, and 2 postoperative deaths (respiratory failure after bronchopneumonia and bleeding duodenal ulcer; acute necrosis of the liver following hepatitis). The study of the deceased patients made evident that postoperative impairment of left ventricular function is caused by ventricular aneurysms. This fact can be shown by the poor ventricular function with an elevated left ventricular enddiastolic pressure (LVEDP) of more than 18 mm Hg. The results in patients with congestive heart failure could not be improved by multiple bypass-grafts. Probably the prolonged surgical intervention may cause additional stress to the predamaged myocardium. So, in our group the indication for using multiple grafts in cases with ventricular aneurysm is confirmed with great caution.

Detection of surface antigen 17-1A in breast and colorectal cancer.

Substantial progress has been made in detecting cell surface or intracytoplasmatic antigens to identify spread tumor cells with monoclonal antibodies (MAbs). The 17-1A antigen is already used as a target for specific immunotherapy in colorectal cancer. The purpose of this study was to compare the expression of 17-1A antigen in colorectal tumors versus breast cancers. MAb against the epithelial-specific antigen (ESA) and a routine staining technique were used to detect the 17-1A antigen in 100 cases of colorectal and 111 cases of breast cancer. The antigen expression of each tumor entity was examined by light microscopy on paraffin sections. Thirty six of the formalin-fixed paraffin sections of breast cancer were compared with their corresponding frozen sections. Evaluation was realized by a histological score (grade 0-9) considering the distribution and the staining intensity. We found an antigen expression of 17-1A in colorectal cancer quantified at 7.1+/-1.8 and at 4.5+/-2.5 for breast cancer in our score. Comparing paraffin sections and frozen sections in the 36 cases of breast cancer, the score was 5.5+/-2.3 in the paraffin and 8.1+/-1.9 in the frozen section group. Our results confirmed the high expression of 17-1A cases of in colorectal carcinoma. Furthermore, 17-1A is expressed in the majority of breast carcinomas, revealing a high difference between paraffin and frozen sections. As a result, a specific immunotherapy with MAbs against 17-1A antigen in minimal residual stages of breast cancer might be considered.

[Morphology of coronary heart disease (author's transl)]. / Morphologische Grundlagen der coronaren Herzerkrankung

Atherosclerosis and insufficiency of the coronary arteries and their sequelae are summarized in the term "coronary heart disease". For the evaluation of the coronary arteries the knowledge of malformations, variants and supply areas is of importance. Extension and severity of atherosclerosis of the coronary arteries and their insufficiency is being influenced by hyperlipidemia, hypertension and diabetes mellitus. The process of atherosclerosis as a cause of the proliferation of vascular smooth muscle cells in complicated by ulceration, parietal and obliterative thrombosis as well by intramural hemorrhages. Relative ischemia leeds to disseminated cell necrosis; total ischemia causes large myocardial tissue necrosis, called infarction. Localization and extension of infarction and the later scars correspond to the caliber of the obliterated coronary artery and to the significance of the collaterals. Postmortem coronary angiography can detect cause and extension of the damaged cardiac area. Functional significance of chronic coronary heart disease is related to the "critical connective tissue content" of the heart. After surgical treatment qualitative and quantitative morphology may help to explain postoperative cardiac failure.

[Contribution to the morphometry of coronary arteriosclerosis (author's transl)]. / Beitrag zur Morphometrie der Coronarsklerose

In 94 cases with and without coronary arteriosclerosis or various cardiac diseases the coronary arteries were pressure fixed, filled with a mixture of barium sulfate and gelatine for coronary angiography and dissected in 1 cm long segments for morphometry. On consecutive cross sections of the three main branches of the coronary arteries the absolute area of lumen, intima and media as well as of the thickness of the intima and media were measured. From the numerous data of the coronary arteries different indices on coronary arteriosclerosis and by correlation to the heart weight also on coronary insufficiency had been calculated. Using these quantitative data age dependent intimal changes could be distinguished convincingly from cases with uncomplicated coronary arteriosclerosis and cases with hypertension or infarcts. Thus in forensic pathology comparative evaluations can be made in regard to the extent, severity and significance of acute and chronic coronary insufficiency.

[Pathogenesis of acquired coronary anomalies. Case report]. / Kasuistischer Beitrag zur Pathogenese erworbener Koronaranomalien

The heart of a 44 years old man who died of a severe calcifying endocarditis of the aortic valves and the aortic root demonstrated a rare anomaly of the coronary arteries. The ostium and the stem of the left coronary artery were missing. In the left aortic sinus there was a small dimple covered by calcified tissue. Using histological serial sections a small vessel was detected that was completely obliterated by organized thrombotic tissue running between the small dimple in the aortic sinus and the main branches of the left coronary artery. This vessel was supposed to be the remaining stem of the left coronary artery. The regular branches of the left coronary artery were supplied by an anastomosis between the proximal part of the right coronary artery and the descending branch of the left coronary artery running within the upper interventricular septum. It has been suggested that this rare anomaly was caused by a gradual obliteration of the ostium and the stem of the left coronary artery during the course of the calcifying rheumatic endocarditis of the aortic valves.

[Comparative enzyme-histochemical studies on normal myocardium, in cases of coronary insufficiency and myocardial infarction (author's transl)]. / Vergleichende fermenthistochemische Untersuchungen am normalen Herzmuskel, bei Koronarinsuffizienz und Herzinfarkt

Comparative histochemical studies with Tetrazolium salt solutions were performed on 25 human and 3 canine hearts. After perfusion of whole hearts with Tetrazolium solutions extensive unstained areas were observed which proved to be artefacts, since they reacted positively after incubation of the heart-slices. The perfusion method of Lichtig, Feldman, Glagov and Wissler (1973) which was proposed for macroscopic and microscopic diagnosis of ischemic cardiac lesions was not found to be suitable for the identification of microcirculatory disturbances. The usual incubation method, however, proved to be useful for the macroscopic localization of recent myocardial infarcts.

Karyological and electron-microscopic studies in myocardial cells of primates after experimentally induced cardiac hypertrophy.

Precocious polyploidization in hypertrophic hearts of infants with congenital malformation can be reproduced banding the ascending aorta of young rhesus monkeys. Electron micrographs show hypoxic changes, signs of DNA, RNA, and protein synthesis as well as degenerative changes comparable to human hearts. Enforced polyploidization of cor pulmonale is found in rhesus exposed to coal quartz dust for 48 months. The pig heart, containing disturbed mitoses, serves as a model for incomplete mitoses. During evolution they became the rule for the myocardial cells of primates.

[Basal cell adenocarcinoma of the parotid gland: a rare tumor entity. Case report and review of the literature]. / Basalzelladenokarzinom der Glandula parotis: eine seltene Tumorentität. Fallbericht und Literaturübersicht.

Basal cell adenocarcinoma is a rare entity that was first defined as a malignant salivary gland tumor in 1991. We present another case report and discuss pathology, pathogenesis, differential diagnosis, therapy and prognosis on the basis of currently available literature. Although histomorphologic features of the tumors are similar to basal cell adenomas, proof of an infiltrative and destructive growth is essential for diagnosis. Adenoid cystic carcinoma and basaloid squamous carcinoma must also be considered in any differential diagnosis. Tumor development within a pre-existing basal cell adenoma and de novo development are discussed. Most of the tumors appear to be benign clinically. Facial pain is rare and facial nerve palsy was noted in only one case. Metastases have occurred in less than 10% of patients, with only one involving the lung. Due to their biologic behavior and prognosis, basal cell adenocarcinomas should be classified as low-grade carcinomas. The therapy of choice is parotidectomy with preservation of the facial nerve. Neck dissection has to be added in cases with cervical metastases. Radiation is advisable in patients with recurrent disease. Since there is a nearly 30% local recurrence rate, intensive follow-up is necessary.

Hypertrophic non-obstructive cardiomyopathy caused by disorder of the myofiber texture.

A case of hypertrophic non-obstructive cardiomyopathy caused by a disorder of the myofiber texture was observed in a 10 year old boy. The heart weighed 390 g and showed concentric hypertrophy of the left and right ventricles as well as of the ventricular septum. Additional findings consisted in an abnormal septum membranaceum and a accessory tricuspid valve leaflet. Severe cardiac hypertrophy was associated with prominent interstitial and subendocardial fibrosis, and pronounced intimal fibrosis of the intramural arteries. Electron microscopy revealed various degrees and stages of hypertrophy of the myocardial cells combined with severe degenerative changes. Additional changes of the sinus node and conduction system were responsible for a tachycardia-bradycardia syndrome, complete left bundle branch block and final total AV-block.

[Primary calcification in Hodgkin's disease]. / Primär verkalkte Lymphogranulomatose

A chance radiological observation of a calcified mediastinal tumour in 23-year-old woman led to its operative removal. Histologically it proved to be a Hodgkin's sarcoma in a nodular sclerosing form of Hodgkin's disease. There was no other clinical evidence of Hodgkin's foci and, after radiotherapy, the patient has been without recurrence for over one year.

[Doppler ultrasonic measurement for diagnosing carotid occlusions and stenoses (author's transl)]. / Befunde und Verlauf bei sonographisch ermittelter Strömungsbehinderung in der Arteria carotis interna.

Doppler ultrasonic measurements were performed in 74 patients suffering from cerebrovascular disorders. Flow direction was determined in the supratrochlear artery by using a directional Doppler sonographic instrument according to the method of Keller et al. 15 patients presented, unilateral or bilateral, with a flow reversal (i.e. direction of flow into the orbita via supratrochlear artery). In 8 of these patients carotis or aoitic arch angiographies were performed and in 4 cases necropsy reports were available. In all instances where flow reversal occurred severe carotid stenoses or occlusions were demonstrable. Thus 9 internal carotid occlusions, 1 common carotid occlusion, and 3 severe internal carotid stenoses were proved. One internal carotid occlusion and 3 internal carotid stenoses were operated on. Check-up sonograms after endarterectomy of carotid occlusions or stenoses showed a normal antegrade flow thus demonstrating patency of the carotid vessels. Obviously, supratrochlear flow reversal as measured by ultrasonic technique signals ipsilateral carotid vessel occlusion or stenoses. Furthermore, the method seems to be a reliable means for controlling carotid endarterectomy.