The risk of second malignant tumors and its consequences for the overall survival of Hodgkin's disease patients and for the choice of their treatment at presentation: analysis of a series of 1524 cases consecutively treated at the Florence University Hospital.

PURPOSE: To quantify the incidence of second malignant tumors (SMT) as a whole and that of second "solid" tumors (SST) and leukemia (L) in a large series of 1524 Hodgkin's disease (HD) patients (pts) treated at the Florence University Hospital (UFH); to define the clinical and therapeutic features possibly related with SMT occurrence; to evaluate the consequences of SMT for the overall survival of the series studied and for the choice of the treatment of HD at presentation. METHODS AND MATERIALS: From 1960 to 1991, 1524 pts with HD, Clinical Stage (CS) I--IV have been treated at the UFH. Overall treatment consisted of radiation alone (RT, 36%), chemotherapy alone (CHT, 21%), or both (RT + CHT, 43%). The cumulative probability (CP) of SMT, SST, and L was calculated for the whole series and for the different clinical and therapeutic subgroups, and the results compared with uni- and multivariate analysis ("internal" comparison, IC). Standardized incidence ratios (SIR) for different SMT types (estimated on the basis of gender, age, period specific incidence rates of the general population) have been also calculated ("external" comparison, EC). The impact of the SMT-related mortality on the survival of the entire series has been estimated. RESULTS: A 14.9% 20-year CP of SMT was registered, along with a SIR of 2.04 (95% confidence interval [CI]: 1.2--2.5). Both IC and EC showed a statistically significant relationship between L incidence and treatment with CHT, alone or in combination with RT. A significant excess of breast cancers has been observed in RT-treated patients with longer follow-up (SIR, 2.9); an excess of other common SST (lung, non-Hodgkin's lymphomas) is evident in pts treated with either RT, RT + CHT, or CHT. The actuarial long-term survival of the series would have been better of about 3%, in absence of the SMT mortality possibly due to HD treatment, which is almost equally divided between patients treated with RT alone, CHT alone, and RT + CHT. CONCLUSIONS: SMT represent an important late event in HD long-term survivors. The relationship between L and treatment with CHT seems to be the most clearly defined. The effect of SMT on the survival of the entire series, although not negligible, does not seem to justify by itself substantial alterations in the current standards for the treatment of HD at presentation.

[Therapeutic implications of the peculiar clinical characteristics of Hodgkin's disease in clinical stages I-II with isolated neck presentation]. / Implicazioni terapeutiche delle peculiari caratteristiche cliniche del morbo di Hodgkin negli stadi clinici I-II con presentazione cervicale isolata.

Among 703 patients with supradiaphragmatic clinical stages I-II Hodgkin's disease consecutively treated 1960 through 1989 at the Florence Radiotherapy Department, we identified 98 cases presenting with cervical and supraclavicular nodes involvement only. The latter cases were retrospectively reviewed to assess: a) if they presented any peculiar clinicopathologic features and b) the possible prognostic and therapeutic implications (if any) of these features. The high incidence of lymphocytic predominance histology (33%) and Waldeyer's ring (WR) involvement (24%) and the very low risk of occult infradiaphragmatic involvement (observed in only 2 of 44 patients submitted to staging laparotomy with splenectomy, 4.5%) are among the distinctive features of the patients with supradiaphragmatic clinical stages I-II Hodgkin's disease. All the 98 patients were treated at presentation with irradiation alone. Actuarial cause-specific survival 20 years after the end of treatment was 87%; the corresponding value for relapse-free survival was 78%. The most frequent complications were irradiation pneumonia, most often asymptomatic, and xerostomia. Our data seem to suggest that, for the majority of these patients: a) staging laparotomy could be avoided and b) "mini mantle" irradiation is an adequate treatment. Moreover, WR irradiation could be avoided in the patients without clinical WR involvement.

[Late sequelae involving the breast after radiotherapy for lung metastasis of Wilms' tumor. Experience of the Florence Radiotherapy and analysis of the literature]. / Sequele mammarie tardive dopo radioterapia per metastasi polmonari di tumore di Wilms. Esperienza della Radioterapia di Firenze e analisi della letteratura.

A growing number of children treated for malignant tumors has been cured thanks to progress in the field of pediatric oncology, and therefore long-term treatment sequelae have been more frequently observed. Fourteen of 65 pediatric patients with Wilms' tumor treated in Florence 1957 through 1978, developed lung metastases and six survived at least five years after treatment. Four of these long-term survivors are girls. This paper reports on the long-term sequelae observed in the breast of these four patients: three of them presented breast hypoplasia and one breast cancer. We discuss our personal experience and the relative literature, describing the relationship between given dose and the incidence of breast hypoplasia. Some hypotheses on the etiology of secondary cancer are also discussed.

Second solid tumors and leukemia after treatment for Hodgkin's disease: an analysis of 1121 patients from a single institution.

PURPOSE: To define the risk of having a second malignant neoplasm (ST) in different subsets of Hodgkin's disease patients and possibly to identify potentially avoidable therapeutic behaviors, linked with an increased second tumor probability. METHODS AND MATERIALS: Cumulative probability of having a ST has been calculated for the different clinical and therapeutic subgroups of a population of 1121 patients consecutively treated (1960-1988) for Hodgkin's disease. Age groups at diagnosis were as follows: < 20 years, 18%; 20-40, 48%; 41-60, 26%; > 60, 8%. Initial treatment consisted of radiation alone (67%), combined modality treatment (24%), chemotherapy alone (9%). Treatment for relapse was also coded, and the occurrence of ST was related both to initial treatment (considering relapsed patients as censored at relapse) and to the "overall" treatment burden, without censoring at relapse. RESULTS: An increased ST risk has been observed in patients older at HD diagnosis. Second tumors cumulative probability rates were significantly higher in patients initially treated with chemotherapy, especially when associated with subtotal or total nodal irradiation (relative risks of 3.1 and 4.1, p = .03 and .005, respectively, when compared to involved field radiotherapy alone). The same trend was observed for second solid tumors. Acute leukemia was more frequent in patients initially given chemotherapy alone or associated with radiotherapy (p = .01), and in those treated with an increasing number of cycles (p = .004). "Salvage" chemotherapy after radiation alone at presentation does not seem to be linked with an increased risk of leukemia. CONCLUSION: The 15-year cumulative ST probability (11%) should be evaluated in the context of the very good cure rates achieved for Hodgkin's disease. The use of chemotherapy, particularly when associated with subtotal or total nodal irradiation, entails an increased risk of second malignancies and might be inappropriate in early stage Hodgkin's disease patients.

Chordoma-natural history, treatment and prognosis. The Florence Radiotherapy Department experience (1956-1990) and a critical review of the literature.

Fifteen cases of chordoma, seen between 1956 and 1990 at the Florence Radiotherapy Department are reported. Twelve of them were treated with radiotherapy and surgery, while one was left untreated. We analyzed the course of the disease in the treated cases, with particular emphasis on the problem of symptom control. The natural history of the disease seemed to be only marginally affected by the treatment and new therapeutic options are strongly needed. While actuarial survival rates at 5 and 10 years were 58% and 35% respectively (owing to the slow growth rate of this neoplasm), 10 years' symptomatic progression-free, symptom-free, and disease-free survival rates were only 25%, 17% and 8% respectively.

Merkel cell carcinoma and iodine-131 metaiodobenzylguanidine scan.

Two cases of Merkel cell carcinoma, a neuroendocrine neoplasia of the skin, investigated with iodine-131 metaiodobenzylguanidine (131I-mIBG) scintigraphy, are reported. Uptake in the tumor was evident only in 1 case. The possible diagnostic and therapeutic role of 131I-mIBG in patients with this rare neoplasm is discussed.