[Alveolar proteinosis. Report of 2 cases and diagnostic-therapeutic considerations].
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Biomedical subjects
Publications and source records attributed to E Grandi.
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A family with "arrhythmogenic right ventricular dysplasia (ARVD)" is described. ARVD is pathologically characterised by a partial or total degeneration of the right ventricular myocardium, replaced by fatty and fibrous tissue. This causes dangerous ventricular arrhythmias or congestive heart failure in infancy. About the three described patients, a sixteen-year-old subject died suddenly, and his anatomical and histopathological reports have been presented. Familiarity, rarely treated in literature, and the presence, in the died subject, of a damage of the left ventricle too, are the peculiar characteristic of these "ARVD" cases. The importance about instrumental non-invasive studies has been underlined in order to know early "ARVD", which can be deceptive clinically. The authors suppose there is surely, at the beginning of "dysplasia", a genetic alteration of the right cardiac musculature, but acquired noxae could insert afterwards, on this genetic basis.
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Authors have passed from a combined clinical and radioisotopic analysis to an integrated polyinstrumental approach by adding thermography and above all echography. In order to achieve the purpose Authors have confronted in retrospective a "clinico-radioisotopic group" of 103 cases, from 1965 to 1975, and an "integrated polyinstrumental group" of 53 cases, since 1976. Comparing the "clinico-radioisotopic method" with "polyinstrumental approach" the main results could be summarized as follows: the diagnostic specificity improved in benign lesions from 77% to 96%, however in malignant lesions it remained about the same (63%). Considering these results the Authors have subdivided thyroid nodules, mainly based on echography, in two distinct categories with the following orientation: I. Solitary Thyroid Nodule: a) definitely cystic -- demonstrated by echography; b) "cold" -- by conventional scintygraphy; c) "negative" -- by thermography; d) non uptake of tumor seeking radiopharmaceutical. The control, therapy and surveilance of these cases should be limited to needle aspiration of the cystic cavity followed, of course, by citologic examination. This aspirations is both diagnostic and possibly therapeutic. II. Solitary Thyroid Nodule: a) definite solid -- echography; b) "cold" -- convential scintygraphy; c) "positive" --thermography (possibly "negative"); d) non uptake of tumor seeking radiopharmaceutical. Here the therapeutic orientation is clearly surgical.
A study of membrane proteins in pernicious anaemia and other haemolitic and anhaemolytic erythropathies is reported. Cellulose acetate and acrylamide gel electrophoresis was carried out and acid, neutral and basic amino acid contents were determined. Relatively specific alterations were noted. Chromatographic analysis of the amino acids suggested that in pernicious anaemia, as in other conditions, structural changes take place in the red cell membrane, though these may be confined to certain sectors. A constant alteration related to the concomitance or otherwise of erythrocyte hyperhaemolysis, however, could not be discerned.
The Authors describe a case of mature cystic teratoma found in a little girl 9 + 8/12 years old and removed in laparoscopic way. Various specialists' contribution shows up for the resolution of the case.
We present a case of biphasic pulmonary blastoma, a rare primary malignant lung neoplasm, in a 48 year old man. Despite its resection followed by postoperative chemotherapy, the neoplasm recurred and the patient survived only 41 months after the diagnosis.
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Adipose tissue tumors in ten years of diagnostic activity (1979-1988). We have reconsidered our material on tumors of adipose tissue, which were observed for 10 years, from 1979 to 1988, at Istituto di Anatomia Patologica dell'Università degli Studi - Arcispedale S. Anna di Ferrara. The whole collection consists of 772 tumors, 742 lipomas and 30 liposarcomas. We have intended to examine the development of our diagnostic experience through time and compare our data with those of literature. Simple lipomas and fibrolipomas are the most common histological types (78.8%), followed by angiolipomas (3.6%) and intramuscular lipomas (2.5%). The other types have a lower percentage. In every cases the morphologic and clinicopathologic features are shown, as well as compared with those of literature, and discordances that may result are interpreted. We are firmly convinced that routine diagnosis is not always compatible with rigid classifications and that it is not easy to use morphologic criteria which vary in quality and quantity. We have consequently aimed at reducing any possible distortion in diagnosis due to subjectivity, by sticking rigorously to consolidated morphology. Although our findings mostly agree with those of literature, some discordances still exist; the most significant of them regard age incidence. Among spindle cell lipomas there are two cases diagnosed in early childhood; the number of angiolipomas as well seems to be very high in this period of life. Particularly important from the clinical point of view has been the decision of diagnosing a thigh tumor as lipoblastoma in an eighteen-year-old boy. Other discordances seem to us less significant, as, for example, the absence of intramuscular lipomas in the thigh, which is to be considered, in our opinion, a chance event that we could not explain otherwise. Liposarcomas represent the 4% of the cases. Only one cases has been diagnosed in a patient less than 40 years old. The location sites agree with those recorded in literature. Our material, on the other hand, does not confirm that the average age of the patients with retroperitoneum liposarcomas is higher than that of the patients with thigh tumors. We has well have identified a greater incidence of retroperitoneum tumors on women. Gross and microscopic findings correspond to those known. Myxoid and pleomorphic liposarcomas are the most common histological types. As regards myxoid liposarcomas, we point out a tumor in the breast in a forty-nine-year-old women. Three of the well differentiated liposarcomas are fibrosing liposarcomas of the spermatic cord. The liposarcomas recurring during the time considered have been seven.(ABSTRACT TRUNCATED AT 400 WORDS)
The purpose of the present work was to define some aspects (i.e. the influence of histological grading on prognosis and the possibility of an eventual hormone dependency) of adenoidocystic carcinoma, a highly controversial form of carcinoma still open to debate. A study is thus reported of 33 cases of adenoidocystic carcinoma of the head and neck seen from 1965-1985 at the E.N.T. Clinic of Ferrara. The individual histological samples were seen over a period of time by a single histopathologist who was not informed as to the patients' clinical progression. In this manner the diagnostic criteria was kept constant. Tumoral grading was then performed on the basis of the Marsh and Allen classification. The overall actuarial survival curves are presented according to site and grading. Besides confirming the already known data regarding the decrease in survival rate well beyond the fifth year, local recurrences and the frequency of lymph node and secondary metastases, the results indicate a direct relationship between grading and prognosis. In fact, overall survival decreases for the less differentiated forms while the frequency of local recurrences increases as does that of lymph node and systemic metastases. Furthermore, in 63.3% of the female patients a genital hormone dependent pathology was noted prior to the appearance of a salivary gland tumor. These observations would suggest that hormones may play a role in the genesis of adenoidocystic carcinoma.
Hibernoma. Case report. Although well defined in its morphological features, hibernoma still remains an interesting type of tumor, due to its rareness. The case here presented shows the morphological characteristics of a lesion which could be classified as intermediate between simple lipoma and typical hibernoma, with lipoblastic-like cells similar to those we find in lipoblastomas and lipoblastomatosis. The pictures suggest the idea that this lesion may be the result of a maturing process toward well differentiated tumors, as pure lipomas and typical hibernomas.
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