Radiotherapy of T4 squamous cell carcinoma of the skin of nasal pyramid.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to E Bertani.
Explore the source record for details and available documents.
Cytogenetic analysis performed on 73 sporadic basal cell carcinomas (BCCs) and three squamous cell carcinomas (SCCs) showed different findings in direct preparations (24 hours) and in short-term cell cultures. Except for loss of the Y chromosome, not one of the other clonal (+6, +16, add(2)(q37), del(3)(q13), add(1)(p31), and near triploidy) or sporadic changes found in direct preparations was found in cell cultures and vice versa. Clonal trisomy 6 found in two BCC direct preparations and demonstrated by interphase fluorescence in situ hybridization in 8 other cases seems to be a nonrandom change in basal cell carcinoma. Immunohistochemistry showed that the cell type investigated was different in the two methods of analysis used: epithelial in direct preparations and fibroblastic in cell cultures. Thus, the results obtained in direct preparations indicate the BCC or SCC epithelial karyotype, whereas the aberrations found in cell cultures indicate the presence of chromosome instability in the fibroblastic stroma. The apparent lack of correspondence between direct and indirect preparations and the presence of clonal chromosome changes in both epithelial and stromal cells suggest tumor cell heterogeneity of BCC. The fibroblastic stroma seems to be implicated in the neoplastic process. This is not evident in SCC, in which clonal changes are present only in direct preparations. The chromosomal distribution of the breakpoints involved in structural changes in direct and cell culture preparations is random; together with those reported in the literature, the breakpoints found in BCC cultures show, however, a cluster to 1p36, 3q13, 9q22, 14p11, 15p11, and Xp11 bands. We did not find any significant correlations between BCC cytogenetic results and the clinical data (site, age, sex, recurrence). The incidence of cases of BCC (38%) and of SCC (100%) showing clonal chromosome changes agree with their benign and malignant nature, respectively. Finally, a significantly high incidence of constitutional inv(9) and dup(9)(q11q21) was found in the group of patients with BCC.
BACKGROUND: Cutaneous T-cell lymphoma (CTCL) includes several lymphoproliferative disorders involving mature T-lymphocyte proliferation initially confined to the cutis. These affections, after variable periods, may progress to the blood, limph nodes and visceral organs. Mycosis fungoides (MF) is the most frequent form of CTCL and has an indolent clinical course. The therapy of CTCL depends on the stage of the disease and the patient's general conditions. For advanced cases it includes chemotherapy, retinoids, and interferon-alpha. Since 1987 extracorporeal photochemotherapy (ECP), a novel immunomodulatory approach based on apheresis and photoirradiation of leukocytes, has been successfully introduced for the treatment of advanced CTCL. It can prolong survival of patients with erythrodermic CTCL without significant side effects. OBJECTIVE: To review our five-year experience with ECP in CTCL. METHODS: Since June 1994, 33 CTCL patients have been recruited for ECP, using two different regimens: two procedures on two consecutive days at four-week intervals for six months, or at two-week intervals for three months with progressive tapering in the second three-month period for the more severe forms. Six patients received ECP with IFN-alpha. ECP was done using the photopheresis UVAR system and UVAR XTS (Therakos, West Chester, Pa) and always with 8-MOP liquid formulation injected directly into the buffy coat bag. Lymphocytes in peripheral blood were immunophenotypically characterized for each patient and every ECP session. RESULTS: All patients tolerated ECP well, without significant side effects. Thirty patients are clinically evaluable (at least three ECP cycles). A favourable clinical response was obtained in 80.9% (16/21) of MF patients (complete response 33%, partial response 47.6%) and in 66% (6/9) of patients in the Sézary's syndrome phase (complete response 33.3%, partial response 33.3%). Five of the six patients given IFN-alpha as adjunctive therapy had a PR and one a CR. Four patients are in CR without therapy at follow-ups of 46, 20, 10 and 8 months. There have been no changes in the peripheral lymphocyte immunophenotype during the follow-up. In 19/30 patients the CD95 antigen, correlated with cellular apoptosis, was expressed and was frequently associated with a good clinical response. CONCLUSIONS: In our experience ECP achieved favourable clinical responses in 73% of patients, in monotherapy or in combination with IFN-alpha, without significant side effects.
Sarcoidosis is a granulomatous disorder of unknown etiology with a multi-disciplinary interest. However, a multiple organ involvement is not always clearly presented. A case of a 62-year-old woman with cutaneous, pulmonary, ocular, cardiac, bone and articular involvement is described.
The Authors report a case of eosinophilic cellulitis (Wells' syndrome). The patient was a 61 year old woman, diabetic, with a cardio-respiratory insufficiency and a maniaco-depressive psycosis. She presented, on the upper arms and trunk, a cutaneous eruption of erythematous-urticarial plaques, that histopathologically were characterized by a dermic leukocyte population, with a prevalence of eosinophils, distributed in the perivascular site. Laboratory tests revealed eosinophilia and circulating immune complexes. The etiopathogenesis of the disease is discussed as is the possible role of immune complexes in eosinophilic cellulitis.
A 35 year female with a previous history of asthma came to our observation about 10 months ago. She was dyspneic, showed small nodules (2 to 5 mm) in arms, hands and legs and had radiological evidence of pulmonary granulomatosis and pleural effusion. Diagnosis was made by means of cutaneous biopsy showing a necrotic granulomatous vasculitic lesions with eosinophilic infiltration and giant cells, consistent with allergic granulomatosis of Churg-Strauss syndrome. Steroid therapy alone (prednisone 1 mg/kg/die) led to a rapid and complete clinical and humoral remission. The patient is still doing an alternate day prednisone therapy (15 mg) and is well after a 10 months follow-up.
Explore the source record for details and available documents.
Acquired tufted angioma is a benign, slowly progressive angioma with a typical histologic pattern that was first described by Wilson Jones. The clinical, histopathologic and transmission electron microscopic features of a case of this rare condition are presented. Angioblastoma of Nagakawa is clinically and histopathologically the same as acquired tufted angioma.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
The case of an 81-year-old woman suffering for about 1 year from a typical form of Letterer-Siwe disease (LSD) is reported. The only important systemic involvement was hepatosplenomegaly. The patient was treated with steroids and vinblastine and then with thymopoietin pentapeptide. This last drug proved useful for decreasing the dose of steroid and thus for checking the appearance of new skin lesions. The ultrastructural investigations, carried out before and after vinblastine, showed the presence in some histiocytosis X cells (HXC) of coated Birbeck granules (BG). Immunoelectron microscopy showed about 90% of HXC to be OKT6+ and the OKT6 labeling of these cells to be similar to that of the Langerhans cells of normal skin. Five percent of OKT6+ cells apparently contained no Langerhans granules.
To our knowledge, these are the first cases of almost complete Netherton's syndrome in two adult brothers born of consanguineous parents. The aromatic retinoid, etretinate, although initially worsened the eczematous manifestations, proved to be capable of reducing the primary skin lesions in one patient.
The authors describe two brothers showing leukonychia totalis in association with deafness, hyperkeratosis palmaris and plantaris, scalp modifications and dental lesions. The onychopathia began during early childhood and was also present in four other members of the same family. All nails of both hands and feet were affected and appeared white, dull and with inconspicuous lunulae. The hair was rough, dark, bristly, looking like steel wire. In particular, the changes of the hair shaft were studied under scanning electron microscope. Hair dysplasia, accompanied by structural changes such as complete and incomplete twistings, longitudinal grooves and modified imbrication, was found. After briefly reviewing the literature on the subject, the authors point out the suggestive hypothesis that all these manifestations might possibly we ascribed to a single genetic defect, in spite of their different clinical features in the various patients.
Explore the source record for details and available documents.
AIMS AND BACKGROUND: The clinical value of the UICC TNM classification and the Izumi TNM modification regarding prognosis after hepatic resection was evaluated. METHODS: Between January 1993 and December 1998, survival and disease-free survival were analyzed in 53 cirrhotic patients (40 males, 13 females; mean age, 65 years; range, 43-81) who underwent hepatic resection for HCC. RESULTS: The 1-, 3-, and 5-year overall survivals were: 89%, 54%, and 50%, with disease-free survivals of 70%, 38%, and 28%, respectively. The difference between stages 1 and 2 or stages 3 and 4A using the UICC TNM classification was not significant with respect to survival or disease-free survival. Conversely, the Izumi TNM modification showed a significant difference between each stage with respect to survival and disease-free survival. In a multivariate analysis the lack of micro/macro vascular invasion was predictive of long-term outcome. CONCLUSIONS: Our results show that the UICC TNM classification for hepatocellular carcinoma is inadequate. The Izumi modified TNM staging system is superior in assessing prognosis for surgical HCC patients.
BACKGROUND/AIMS: From a consecutive series of 51 patients surgically treated from January 1993 to August 1997 for hepatocellular carcinoma (HCC) complicating cirrhosis, 6 subjects (12%) presented with acute hemoperitoneum due to spontaneous rupture of the tumor: 3 patients were suffering from chronic hepatitis C, 2 were affected by alcoholic cirrhosis, and one by chronic hepatitis B. The present paper reports experience of the treatment of ruptured HCC complicating cirrhosis in 6 patients undergoing emergency hepatectomy. METHODOLOGY: Hemoperitoneum was successfully diagnosed pre-operatively with the combination of abdominal ultrasound (US) and paracentesis. All subjects had a known history of chronic liver disease, but undiagnosed HCC. Child-Pugh classification assessed the hepatic functional reserve to predict operative risk. Surgical indication was based on hemodynamic instability and/or persistent bleeding. Time from admission to operation was recorded as well as tumor site, size and number, the site of bleeding, and the duration of surgery and hepatic devascularization. Tumor location was defined according to segmental anatomy. All patients underwent one-stage liver resection (segmentectomy VII-VIII in one patient; non-anatomical wedge resections in 5). Operative mortality was defined as death within 30 days of surgery. RESULTS: No intra-operative death occurred. In 4 patients the post-operative course was uneventful. Two patients died 2 weeks after surgery from liver failure (one patient) eventually complicated by renal failure (one patient). Three patients are alive and 2 of them disease-free at 24 months after surgery, whilst one patient has died from liver failure 21 months after surgery in the presence of intrahepatic recurrence of HCC. CONCLUSIONS: Present experience, combined with a literature review on 755 ruptured HCC cases, indicates that emergency liver resection is feasible in patients with limited tumor and preserved liver function (Child-Pugh A or B grade); surgical resection is the only procedure possibly associated with long-term survival, as shown by 4/6 patients of ours surviving more than 12 months, with 2 subjects disease-free at 24 months. Conservative management, such as surgical/radiological devascularization, packing or plication, can be conducted on high risk patients, though long-term survivors have not been reported.
In hepatic adenomatosis, multiple liver cell adenomas (usually > or = 10) generally affects patients with no prior history of oral contraceptive use, androgenic steroid use, or glycogen storage disease. We report a rare case of a 44 year-old female who underwent emergency surgery for hemoperitoneum due to spontaneous rupture of a liver cell adenoma in hepatic adenomatosis, after prolonged use of two different contraceptives (Gestodene and Ethinylestradiol).
Between January 1993 and June 1999, 54 patients with hepatocellular carcinoma (HCC) complicating cirrhosis underwent hepatic resection. Forty-five (83%) minor hepatic resections were performed. Four (7%) peroperative deaths (within 30 days following surgery) were recorded. During follow-up it was noted that hepatocellular carcinoma recurred in 24 patients (44%) and local recurrence was the most frequent event. Mean disease free interval was 19 months after surgery. By multivariate analysis only vascular invasion proved to be statistically significant to HCC recurrence and survival. The recurrence rate of HCC in patients undergoing liver resection is high and long-term survival is long. An accurate analysis of risk factors for HCC recurrence after liver resection is of the utmost importance and further treatment alternative to and/or in association with surgery requires consideration.