Treatment of acute lymphoblastic leukemia. 30 years' experience at St. Jude Children's Research Hospital

BACKGROUND: Therapy for childhood lymphoblastic leukemia has evolved during the past three decades, but key questions about what are the least toxic, most effective forms of treatment remain unanswered because of the lack of comprehensive follow-up information. METHODS: To assess long-term outcome in the series of clinical trials conducted at St. Jude Hospital, we compared the results of treatment typical of four eras: exploratory combination chemotherapy (era 1, 1962 to 1966; 91 patients), regimens for the control of meningeal leukemia (era 2, 1967 to 1979; 825 patients), limited intensification of therapy (era 3, 1979 to 1983; 428 patients), and extended intensification of therapy (era 4, 1984 to 1988; 358 patients). ("Intensification" refers to strategies of systemic chemotherapy that are more aggressive than conventional ones.) The major end points were survival and event-free survival; we also calculated the relative risk of treatment failure and the rate of relapse or death after treatment ended (post-treatment failure rate). RESULTS: The probability of event-free survival improved significantly in each successive era (P < 0.001 by the log-rank test), reaching 71 percent in era 4. There was a decrease of approximately 50 percent in the risk of treatment failure from one era to the next in each subgroup of patients defined according to different combinations of the leukocyte count, race, age, and sex. Leukemia appeared to be eradicated in patients who remained in complete remission for three years or more after treatment in era 4. The incidence of death due to nonleukemic causes remained 4 to 6 percent despite the trend toward more intensive treatment. An estimated 765 patients (45 percent) are long-term survivors; most of them (80 percent) have no health problems related to leukemia or its treatment. CONCLUSIONS: The development and successful application of preventive therapy for meningeal leukemia, followed by the intensification of systemic chemotherapy, has progressively improved the rate of cure of childhood lymphoblastic leukemia, with relatively few adverse sequelae.     

Treatment of the sequelae of enucleation by septal chondro-mucosal composite graft. Report of 21 cases]

Enucleation frequently and progressively causes an enophtalmus and atrophia of the inferior eyelid, thereby leading to a height deficiency. Buccal mucous grafts give rise to phenomena such as secondary retraction. This may have complex and painful post-operative outcomes. However, when a septal chondromucous graft is performed, the height in the inferior palpebral part becomes more aesthetic, more retentive and quickly allows the wearing of a more voluminous prosthesis. Thus, the notinable enophtalmus can be corrected and the aesthetic quality of the looking is substantially restored. The authors report this surgical procedure and the results obtained with 21 patients which appear to be particularly promising.     

Digestive cancers and choroid metastasis. Diagnostic circumstances and prognostic value apropos of 2 cases]

We report two observations of malignant gastric and pancreatic tumors with choroid metastasis. Clinical course was rapidly and spontaneously unfavorable.     

The immediate reconstruction of the breast after a mastectomy for carcinoma. Our experience

The authors surgically treated 281 consecutive women with breast cancer by breast conservation techniques (45.2%) or by mastectomy (38.8%), during a four-year period. Forty-five of these 281 patients (16%) were treated in the same period of time by mastectomy and immediate reconstruction (IR) of the breast. IR of the breast was performed by inserting a submuscular tissue expander at the same time of mastectomy (first stage of reconstruction). A second operation allowed the replacement of the expander with a prosthesis (second stage of reconstruction) and the simultaneous symmetrization of the contralateral breast (not always performed, however). In some cases nipple-areola complex was eventually reconstructed with a delayed surgical procedure (third stage of reconstruction). In the present paper the authors analyze the results and outline the advantages of IR. This easy and safe technique slightly increased the average operative time of a mastectomy, did not interfere with routine oncological follow-up, did definitely reduce patient's psychological trauma following mastectomy. The authors conclude that IR of the breast undoubtedly plays a major role in the so called "onco-plastic" surgical management of breast cancer.     

Ureteral metastases from prostatic cancers. 2 cases. Review of the literature

The authors report two new observations of ureteral metastases from prostatic cancers and review the 17 already published cases. The relative rarity might well be only apparent, in relation with the delay in the exploration of the first observations. The diagnostic criteria are less clinical and radiological than anatomo-pathological, the metastases contrasting themselves with ureteral invasion by direct propagation. The anatomical status shows no predominance between tumoral or infiltrating forms, nor of any particular site on the ureter, while noting a relative frequence of bilateral involvement. The therapeutic approach is dependant upon these factors. The prognostic is reserved, at the cost of a segmental ureteral resection or a nephro-ureterectomy.     

Treatment of differentiated thyroid cancers in children. Study and follow-up of a series of 130 cases at the Gustave-Roussy Institute

A 60 year old woman affected by Hashimoto's thyroiditis presented with a history of recurring episodes of urticaria and angio-oedema. Clinical and laboratory evaluation of the patient excluded allergy to external agents, hereditary angio-oedema, and occult infections. A pathogenic relation between Hashimoto's thyroiditis and chronic urticaria/angio-oedema was suspected. However, treatment with L-thyroxine had no influence on the frequency and severity of the cutaneous and mucosal manifestations, which occurred almost daily and required repeated administration of steroids. The patient therefore underwent total thyroidectomy. Cytometric analysis of intrathyroidal lymphocyte subsets showed unusual abnormalities. Urticaria and angio-oedema completely remitted after surgery; 18 months postoperatively the patient was still asymptomatic.     

Historical stages in the development of maxillofacial surgery and stomatology at the N. N. Burdenko Main Military Clinical Hospital

Effect of level of dietary calcium on body adaptation to radionuclides and lead exposure was studied in experiments with rats. Effectivity of adaptation was evaluated by analysis of accumulation and elimination of xenobiotics and antioxidant activity of liver tissue and erythrocytes. Low and normal levels of calcium in diet of rats do not provide for long-term adaptation to exposure of Ce-137 and Pb. The diet enriched by calcium caused a significant protective-adaptation effect on stages of accumulation and biotransformation of xenobiotics.     

Treatment of chondrosarcoma. A retrospective study at the Norwegian Radium Hospital

The authors present a retrospective analysis of 59 chondrosarcoma patients treated at the Norwegian Radium Hospital during the period 1981 to 1993. 31 patients were admitted with untouched tumour, seven after fine needle cytology and 20 after open biopsy or partial excision. One patient had recurrent local disease. Only 20% of the tumours were of high grade malignancy. 51 patients were treated by surgery. Reconstructions were performed in 16 patients, using allografts or endoprostheses. Amputations were performed in six cases and wide excision in 12 cases. In these 18 patients local recurrence appeared in one case, and two developed lung metastases. Only one of the 18 patients operated by amputation or wide excision has since died from chondrosarcoma. Marginal excisions were performed in 26 cases. Nine of these patients developed a local recurrence, five developed metastases and three have died. Six patients had partial excisions. Postoperative radiotherapy was given to one patient only. Five of the six are alive. In one case, the quality of the margins could not be evaluated. A total of 45 of the 51 patients treated for the primary tumour by surgery are alive. The median observation time is four years. Treatment of nonmetastatic chondrosarcoma should be surgical. Chondrosarcoma patients show wider variations in age, localization of tumour and tumour growth rate than patients with other bone sarcomas. Although wide excisions provide the best local control of any grade of malignancy, the mutilation or risk involved may be so great that some patients may benefit from marginal or even partial excision.     

Intra-oral cancer at the Massachusetts General Hospital. Squamous cell carcinoma of the floor of the mouth

A retrospective review of 163 consecutive patients with biopsy-proven, invasive squamous cell carcinoma of the floor of the mouth who underwent inpatient treatment at the Massachusetts General Hospital during the 15-year period from January 1962 through December 1976 is presented. The stage at first presentation, clinical features of the disease, incidence of second primary tumors, analysis of therapeutic modalities, and survival statistics are compared with reports from other large centers. Floor of mouth tumors comprised 28%, (163/592) of oral squamous cell carcinomas seen at the Massachusetts General Hospital during that time period. Seventy-one per cent of floor of mouth tumors were in men and 29% in women; women tended to present earlier in the course of their disease. Thirty-seven patients (23%) developed a secondary primary malignancy, and four of these 37 patients developed two second primaries. Distant metastatic disease appeared in 6% of patients with Stage I, II, or III disease and 26% of patients with Stage IV disease. Radiation therapy alone and surgery alone resulted in equivalent long-term survival rates for early stage disease. In more advanced stages (III and IV), a combined approach utilizing surgery and radiation therapy obtained superior results for short-term survival than either modality alone. The importance of early diagnosis and treatment and suggestions for development of cooperative protocols in an attempt to improve salvage of patients with this disease is discussed.     

Kidney tumors in Africa. Incidence and anatomo-clinical aspects. Apropos of 123 observed cases at the Central Hospital and at the University Hospital Center in Cameroon

123 renal tumours, among which 4 benign, were observed in 13 years. Renal tumours have a low incidence in Africa and are mostly seen in young patients. Nephroblastoma is the most frequent of these tumours. The clinical and paraclinical aspects of these tumours have nothing particular but the diagnosis is usually late.     

Urethral diseases in children at surgical departments of the Cotonou Hospital. Apropos of 77 cases

Over a 6-year period in the surgical departments of Cotonou hospital, urethral diseases in children represented 20% of all infantile urological diseases and essentially consisted of urethral strictures (26 cases), hypospadias (22 cases), posterior urethral valves (12 cases), prolapsed urethral mucosa, the only disease observed in girls (9 cases). These diseases generally do not raise any diagnostic problems, but the real problem concerns follow-up of these children, who are usually lost to follow-up after discharge.     

Malignant lesions of the liver identified on T1- but not T2-weighted MR images at 1.5 T

The authors reviewed their 2 1/2-year experience with a magnetic resonance (MR) imaging protocol for a 1.5-T MR imager that included T2-weighted fat-suppressed spin-echo, T1-weighted breath-hold gradient-echo, and serial dynamic gadolinium-enhanced T1-weighted gradient-echo imaging to identify histologic types of malignant liver lesions more apparent on T1- than on T2-weighted images. MR images of 212 consecutive patients with malignant liver lesions were reviewed. T2-weighted, T1-weighted, and dynamic contrast-enhanced T1-weighted images were examined separately in a blinded fashion. Seven patients demonstrated liver lesions (lymphoma [two patients] and carcinoid, hepatocellular carcinoma, colon adenocarcinoma, transitional cell carcinoma, and melanoma [one patient each]) on T1-weighted images that were inconspicuous on T2-weighted images. In all cases, the lesions were most conspicuous on T1-weighted images obtained immediately after administration of contrast agent. Histologic confirmation was present for all seven patients. The consistent feature among these lesions was that they were hypovascular, due either to a fibrous stroma or to dense monoclonal cellularity. These results suggest that in some patients with hypovascular primary neoplasms, the lesions may be identified only on T1-weighted images, and that immediate postcontrast T1-weighted images are of particular value in demonstrating lesions.     

Treatment of early stages of ovarian carcinoma. Evaluation of experience at the Institute of Radiation Oncology over a 10-year period (1983-1993)

The granulocyte colony-stimulating factor (G-CSF) regulates neutrophil differentiation and function. Serum levels of G-CSF increase during acute infectious processes. The levels of G-CSF were measured in 59 surgical intensive care unit (ICU) patients. In general, G-CSF was only elevated during the first 2 days after admission to the ICU. Higher G-CSF levels were more frequently observed in patients without infectious complications and in patients who survived. Later on, G-CSF levels were below 100 pg/ml in almost all patients studied. The highest G-CSF level (20,000 pg/ml) was observed in one patient with septic shock 36 h after leukopenia. The patient recovered from septic shock and multiple organ failure and was discharged. It is proposed that surgical ICU patients with low or undetectable G-CSF serum levels may benefit from exogenous G-CSF substitution protocols.