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Primary retroperitoneal tumours, in the material of our department.

After giving a brief survey of the literature, the authors discuss the symptomatology, diagnostics and management of retroperitoneal tumours. Four of their own cases treated between 1979 and 1985 summarized in tables are presented. All of them were operated and three of them received irradiation therapy. The fate of the first patient is unknown. The second patient survived for 24 months following the first operation, with additional three operations and irradiation on two occasions for recurrence of tumour. The third patient survived for 5 months. In the fourth, it could be revealed by ultrasound and angiography that the retroperitoneal tumour was independent of the kidney. Thus, the intact left kidney was preserved during operation. The patient died three months postoperatively at the department of pulmonology. The histological finding of the tumours of all patients was a malignant tumour of mesenchymal origin, a type of sarcoma. Based on their experiences, the authors stress the importance of the early recognition and removal of the tumour, since the patients have a poor chance of surviving these extremely malignant sarcomas in spite of the surgical and irradiation therapies.

Adult↗

Myxoid sarcoma arising from the pulmonary trunk.

We reported a case of a myxoid sarcoma of the pulmonary trunk in a 40-year-old women. On light and immunohistochemical studies, spindle-shaped tumor cells revealed the nature of smooth muscle origin.

Adult↗

Cytogenetic analysis of 46 pleomorphic soft tissue sarcomas and correlation with morphologic and clinical features: a report of the CHAMP Study Group. Chromosomes and MorPhology.

With the aim of identifying objective cytogenetic-morphologic correlations, we evaluated 46 pleomorphic soft tissue sarcomas (mainly diagnosed originally as malignant fibrous histiocytomas) with clonal chromosome aberrations both cytogenetically and morphologically as part of an international collaborative study. By detailed histopathologic examination, most cases could be categorized into specific tumor types. Eight sarcomas were diagnosed as lipogenic (4 pleomorphic, 1 combined pleomorphic and myxoid/round cell, and 3 dedifferentiated liposarcomas), 19 as myogenic [11 leiomyosarcomas, 1 rhabdomyosarcoma, 4 myosarcomas not otherwise specified (NOS), and 3 probable myosarcomas NOS], 8 as myxofibrosarcomas, 1 as a malignant peripheral nerve sheath tumor, 1 as malignant mesenchymoma, 1 as extraskeletal osteosarcoma, I as sarcoma resembling proliferative fasciitis, and 7 as pleomorphic sarcomas NOS. In a three-grade system, 10 tumors were grade 2 and 36 were grade 3. The majority had highly complex karyotypes. A total of 24 recurrent abnormalities (defined by their presence in at least five cases) were detected: ring chromosomes, homogeneously staining regions (hsr) and/or double minute chromosomes (dmin), and structural rearrangement of 22 different chromosome bands or regions. The frequency and distribution of the recurrent karyotypic features were uneven. Grade 3 tumors displayed, on average, more aberrations per case than did grade 2 tumors. Nine of the selected abnormalities, including hsr/dmin and rearrangements of 19p13 and 19q13, were found only among the high-grade tumors. When the tumors were subdivided according to lineage of differentiation, the highest frequency of aberrations was seen in pleomorphic sarcomas NOS, followed by myxofibrosarcomas, myogenic sarcomas, and lipogenic sarcomas. None of the selected rearrangements was, however, specific for any of these subgroups. The sole consistent cytogenetic-morphologic association was that all three dedifferentiated liposarcomas had multiple abnormal clones, at least one of which contained supernumerary ring chromosomes. Due mainly to karyotype complexity, it therefore seems unlikely that cytogenetic analysis can assist in the differential diagnostic subclassification of pleomorphic sarcomas, nor was there any clear-cut indication that the karyotypic picture could be used to predict clinical outcome. Although the mean number of recurrent chromosome aberrations was almost twice as high in sarcomas that gave rise to metastases as among those that did not, no particular aberration was restricted to either of the two subgroups.

Adult↗

Primary cardiac tumors: experience at the University of Minnesota.

A review of all primary cardiac tumors seen at the University of Minnesota or entered in the Registry of Cardiovascular Disease, affiliated with the University of Minnesota, between 1959 and 1989. Of a total of 124 primary cardiac tumors, 103 (83%) were benign and 21 (17%) were malignant. Atrial myxomas (51 cases) accounted for 42% of all tumors and 50% of benign neoplasias. The remaining 51 benign tumors were, in order of frequency: rhabdomyomas, 14 (13%), papillomas, 12 (11%), fibromas, 9 (9%), hamartomas, (4%), teratomas (2%), lipomas (2%), mesotheliomas (2%), fibroelastomas, hemangiomas, glomangiomas, and a few others. The 21 malignant tumors, all sarcomas, were, in order of frequency: rhabdomyosarcomas, 6 (29%), angiosarcomas, 4 (19%), myxosarcomas, 3 (14%), fibrosarcomas, 2 (10%), and one each leiomyosarcoma, reticulum cell sarcoma, and liposarcoma. There were 3 cases of spindle cell sarcoma (14%) originating in the great vessels: 1 in the pulmonary artery and 2 in the aorta. The prognosis of the resected benign tumors was good, with no complications of recurrences for up to 15 years of follow-up. Tumors that could not be excised included mostly rhabdomyomas, in newborns, and cavernous hemangiomas involving most of the heart, with poor prognosis. Malignant tumors of the heart had a very poor prognosis, even when operated on promptly after diagnosis. The mean survival time of these patients was 5 months after resection, even when followed with chemotherapy and/or radiation; 26% of the tumors had already metastasized at the time of operation. Extensive resections in malignant tumors did not render satisfactory results.

Combined Modality Therapy↗