Biomedical subjects
Publications and source records attributed to F Fetissoff.
Solitary fibrous tumour is a particular kind of mesenchymal tumour, classically arising in the pleura. We report the first case arising in the larynx, associated with a metastasizing adenocarcinoma. The diagnosis is mainly histopathological, especially when strong immunoreactivity for the CD34 antibody is present.
Hodgkin's disease (HD) has seldom been reported after transplantation. Epstein-Barr virus (EBV) is present in about 50% of Reed-Sternberg cells in HD developing in immunocompetent individuals, but is more frequently found in HD of acquired immune deficiency syndrome patients. We report 7 cases of HD that occurred in transplant recipients. Clinical and pathological data and studies of EBV reveal specific features of HD after transplantation. Six patients received kidney transplants and 1 patient received combined kidney and pancreas transplantation. Immunosuppressive therapy consisted of cyclosporine, steroids, azathioprine, and antilymphocyte globulins. One patient received, in addition, anti-CD3 mAb therapy and an EBV+ B cell lymphoma developed. Retrospective EBV serological data from patients were collected. Tumors were classified according to pathology. EBV studies were conducted by immunohistochemical methods with monoclonal antibodies to EBV-latent membrane protein (LMP) or EBV-nuclear antigen 2 (EBNA2), and by in situ hybridization for latent nuclear EBV-early RNAs (EBERs). The mean lapse of time between transplantation and HD was 49 months. Six patients presented with enlarged lymph nodes and 1 patient presented with liver involvement. HD was classified as IA in 2 patients, IIA in 3 patients, IIIB in 1 patient, and IVB in 1 patient. Four patients had primary EBV infection after graft, before HD, and the others reactivated latent EBV infection. Histological subtypes were mixed cellularity in 6 cases and lymphocytic depletion in 1 case. Latent EBV infection was detected with EBERs in all tumors. Reed-Sternberg cells expressed LMP, and were negative for EBNA2 expression. Six patients were treated: 2 patients at stage I received radiotherapy, and relapsed within 1 year with a more advanced stage of HD; chemotherapy was indicated as primary therapy in 5 patients, and as salvage therapy in 2 patients; it was associated with radiotherapy in 4 patients. Immunosuppressive therapy was reduced in all patients. Four patients were alive and in complete remission 18, 25, 31, and 67 months after chemotherapy, with a functioning graft in 3 patients. Two patients died of infection. Mixed cellularity is the most frequent histological subtype observed in HD occurring in transplant patients. EBV is present in all Reed-Sternberg cells. Posttransplant HD shows similarities with human immunodeficiency virus-associated HD. These facts argue for a role of EBV infection and immunosuppression in the progression of HD after transplantation.
Local recurrence after conservative treatment of breast cancer is associated with a significant risk for metastasis. In order to identify criteria predictive of metastasis in this subset of women, we analyzed a series of 35 patients with local relapse among 512 consecutive patients treated with tumorectomy and radiotherapy. When relapse occurred within 2 years of initial treatment, overall 2-year survival from the time of local relapse was 39.5%. When local relapse occurred more than 2 years from initial therapy, 2-year survival was 80.5% (p < 0.001). Pathological slides of both initial and recurrent tumors were reviewed and compared. In 17 patients, local relapse and initial tumor had the same morphological features, with an in-situ component either absent or present in the same proportion. Metastasis occurred in two of these patients. In contrast, 9 of 12 patients in whom the proportion of non-invasive carcinoma had decreased at the time of local recurrence developed metastasis. Overall 2-year survival from the time of relapse was significantly better in the former group of patients (93.3% versus 52.5%, p < 0.05). We concluded that early relapses have a poor prognostic significance and that disappearance of the in-situ component or increase of the invasive component at the time of relapse is a feature predictive of tumor-related death and that more intensive therapy might benefit to this subset of women.
PAF-acether (PAF; formerly platelet-activating factor), a potent lipid mediator of inflammation, is involved in multiple cellular functions. To evaluate the role of PAF in human cancer, we obtained specimens from patients with localized breast carcinoma and assayed them for PAF. PAF was found in almost all carcinoma, although it was not detected in most of the matched, nontumor breast tissue samples. Intratumor PAF level was elevated when axillary lymph node involvement was low. Greater axillary extension (two or more positive lymph nodes) was associated with a decreased intratumor PAF level. These findings, along with the independence of other prognostic factors, indicate that PAF is a tumor marker of axillary lymph node involvement.
Endometrial sarcomas (sarcomas of the endometrial stroma and mixed mullerian tumors) are rare tumors with a bad prognosis. Because of our experience in treating these tumors we have been able to make an analysis of epidemiology and the prognostic factors. We have treated 22 patients. The mean age was 61 years. There were 15 stage I, 3 stage II, 2 stage III, and 2 stage IV. 21/22 patients had a surgical resection of the primary tumor, 19/22 had pelvic irradiation after surgery with vaginal radiotherapy given for 14 patients. The actuarial survival rates at 3 and 5 years were 51 and 34%. 5/22 patients had pelvic recurrence, 6/22 had distant metastasis. In our study the prognostic factors were the stage and the histological subtype. Pelvic radiotherapy seems to improve the rates for survival. The main points for discussion are the need to have a definite histological diagnosis done after initial surgery. A CT scan of the lungs and a lymphangiogram must be performed before treatment. After surgery it appears necessary to give pelvic and vaginal radiotherapy. At the present time chemotherapy did not appear to improve survival.
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Hereditary osteodystrophy of Albright's is a set of hereditary dystrophies associated or not with renal and bony resistance to the parathyroid hormone. Two observations of a true brotherhood are reported. These two patients had in common: short stature, obesity (especially facio-troncular), round face, flat and saddled nose, short neck, early cataract and mental deficiency. One of them showed fourth metacarpals. In these two cases there were cutaneous ossifications, markedly profuse on one of them than the other. These ossifications are a frequent manifestation of the osteodystrophy of Albright's. They appear as cutaneous nodules on any part of the body and are visible, palpable and present on X-ray examination. These ossifications share other phenotypic expressions of the disease and do not seem to be related to the resistance against parathyroid hormone.
A case of Paget's disease of the vulva associated with Paget's disease of the nipple is reported. When the patient was 74-year-old, Paget's disease of the vulva was diagnosed; four years later, Paget's disease of the nipple was detected. Paget's disease of the vulva, although recurrent, is devoid of underlying infiltrating carcinoma. Eccrine sweats glands show different type of lesions consistent with pagetoid extension, in situ carcinoma and epithelial papillary proliferation. Ultrastructural study demonstrates dense granules within Paget's cells. A positive Thiery's cytochemical reaction indicates that these granules contain glycoprotein. Paget's disease of the nipple is associated with an intraductal carcinoma (cribriform type). Some feature of intralobular carcinoma is also observed. No foci of infiltration are found. Oestrogen receptors are detected by the histofluorescence technique of Sin Hang Lee. Few receptors are seen within the intraductal neoplastic cells and within the Paget's cells whereas no receptor at all, are observed within the keratinocytes. Several reports emphasized the relationship between vulvar Paget's disease and infiltrating breast carcinoma. Associated vulvar and nipple Paget's disease is rather rare and was just observed in two cases. These two observations were associated with a mammary infiltrating carcinoma whereas in our case, it remains an in situ carcinoma.
It is rare to find associated Paget's disease of the vulva and Paget's disease of the nipple (fewer than 100 cases have been published). A case history with this association in a woman of 72 years of age is reported here. Although the diagnosis of Paget's disease was not made at the same time in the two sites, the treatment was carried out at the same time. This case is, as far as we know, a unique case because the vulval Paget's disease was not accompanied by an infiltrating underlying carcinoma, whereas the nipple Paget condition was associated with a non-invasive cancer of the breast. After reviewing the literature the commentators have developed a clinical and anatomo-pathological plan. A very exact anatomo-pathological study was carried out with a search for oestradiol receptors and these contribute to the establishment of the link between these two pathological states and a way of understanding their pathogenicity better.
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For animal experimentation, the 95m and 96 technetium isotopes offer many advantages over technetium-99m. Their long physical half-lives and the emission of extranuclear electrons of low penetrating power make it possible to obtain autoradiograms of a great precision. The uptake of technetium stannous pyrophosphate by the epiphyseal plate was studied using liquid-emulsion microautoradiography, 3 hr after i.v. injection into 10-week-old rabbits. Microautoradiograms showed a well-defined and rather specific pattern of localization with intense uptake beneath the epiphyseal disk on the extremities of the vascular buds and a lack of accumulation in the cartilage, whether calcified or uncalcified. In the metaphysis, the label was located where new bone was being laid down and also over the cytoplasm of osteoclasts. We deduce from these results that in normal bone the general distribution of this tracer reflects mainly the arrangement of the blood supply, but the specific sites of accumulation are the bone-forming surfaces and the active resorbing osteoclasts.
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