Safety of bevacizumab in patients younger than 4 years of age.

PURPOSE: Limited data exist regarding the safety and efficacy of bevacizumab in pediatric patients under the age of 4 years. Here, we report a large cohort of pediatric patients under 4 years of age treated with bevacizumab. METHODS: The primary objective was to document adverse events with a possible relationship to bevacizumab. Patients (n = 16) were identified through retrospective chart review and harbored a variety of conditions (44% central nervous system (CNS) tumors, 31% vascular anomalies, 13% neuroblastoma, 12% other). RESULTS: The median age was 34.3 months (range 4.9-47.3), including five patients <2 years of age. Patients received bevacizumab for a median duration of 6.2 months, alone or with chemotherapy, and a median dose of 9.25 mg/kg (range 7.0-11.8). Partial responses were seen in 19% of patients, and clinical improvements were seen in 69%. Adverse events known to be associated with bevacizumab occurred in 37%. Outcomes observed in this population resemble those reported for bevacizumab in older pediatric patients. The overall pattern and frequency of adverse events observed was similar to those seen in reports of older pediatric patients with a variety of conditions. The highest level of efficacy observed was seen among patients with vascular malformations or with low-grade CNS tumors. CONCLUSIONS: Our results suggest that the use of bevacizumab is safe for the youngest children.

Utilidad de la tinción con laminina-332 para diferenciar queratoacantoma, queratoacantoma con áreas de carcinoma epidermoide y carcinoma epidermoide crateriforme / The Value of Laminin-322 Staining in Distinguishing Between Keratoacanthoma, Keratoacanthoma With Areas of Squamous Cell Carcinoma, and Crateriform Squamous Cell Carcinoma

Introducción: El queratoacantoma (QA) es un tumor cutáneo crateriforme, de crecimiento rápido; aproximadamente el 25% de los QA presentan transformación maligna (QAm), observándose áreas de carcinoma epidermoide (CE). La laminina-332 se ha relacionado con progresión a fases invasoras en diversos CE. El objetivo de este estudio es evaluar si la tinción con laminina-332 es útil para distinguir QA, QAm y CE. Material y métodos: Seleccionamos 74 casos del archivo de Anatomía Patológica. Se analizaron 4 grupos: 20 QA sin CE, 20 QAm con áreas evidentes de CE, 20 CE invasores sin relación con QA (8 con morfología crateriforme) y 14 casos «problema» (QA con «dudosas» áreas de CE). Posteriormente se realizó tinción inmunohistoquímica para laminina-332 a todas estas lesiones. Resultados: En las áreas de CE asociado a QAm y en los CE invasores, la tinción con laminina fue positiva de forma intensa, habitualmente en el frente invasor del CE, a diferencia de los QA, en que la tinción fue positiva solo de forma débil y focal, en células aisladas o en pequeños «grupos» celulares. Los casos «problema» se reexaminaron tras valorar la tinción con laminina-332 (8 se diagnosticaron de QA con CE incipiente, 6 de QA sin CE). Conclusiones: La tinción con laminina-332 es diferente en los QA respecto a los CE, por lo que ayudaría a diferenciar los QA de los CE invasores y de las áreas de CE en QAm, así como en el diagnóstico de QA con «dudosas» áreas de CE y QA con CE incipientes (AU)

Introduction: Keratoacanthoma is a fast-growing crateriform skin tumor. Approximately 25% of such tumors undergo malignant transformation and develop areas of squamous cell carcinoma (SCC). The presence of laminin-322 has been associated with progression to invasive forms of SCC. The aim of this study was to determine whether or not immunohistochemical staining for laminin-322 would be of value in distinguishing between keratoacanthomas, keratoacanthomas with areas of squamous cell carcinoma, and SCCs. Material and methods: Seventy-four lesions were selected from the pathology archives of our hospital and divided into 4 groups: 20 keratoacanthomas without SCC, 20 keratoacanthomas with areas of squamous cell carcinoma, 20 invasive SCCs (8 with crateriform morphology) unrelated to keratoacanthoma, and 14 problem lesions (keratoacanthomas with areas suggestive of SCC). All 74 lesions were stained for laminin-322. Results: Laminin-322 staining was strongly positive both in areas of SCC in keratoacanthomas with malignant transformation and in invasive SCCs (mostly at the invasive front of the SCC). However, in benign keratoacanthomas, it was only weakly positive and furthermore it was confined to isolated cells or small groups of cells. The 14 problem lesions were reexamined after laminin-322 staining and 8 were diagnosed as keratoacanthomas with incipient SCC and 6 as keratoacanthomas without SCC. Conclusions: Laminin-322 staining is different in keratoacanthomas and SCCs and would thus be a useful test for differentiating keratoacanthomas from both invasive SCCs and keratoacanthomas with areas of squamous cell carcinoma. It would also be of value in diagnosing keratoacanthomas with areas suggestive of SCC or with incipient SCC (AU)

[The value of laminin-322 staining in distinguishing between keratoacanthoma, keratoacanthoma with areas of squamous cell carcinoma, and crateriform squamous cell carcinoma]. / Utilidad de la tinción con laminina-332 para diferenciar queratoacantoma, queratoacantoma con áreas de carcinoma epidermoide y carcinoma epidermoide crateriforme.

INTRODUCTION: Keratoacanthoma is a fast-growing crateriform skin tumor. Approximately 25% of such tumors undergo malignant transformation and develop areas of squamous cell carcinoma (SCC). The presence of laminin-322 has been associated with progression to invasive forms of SCC. The aim of this study was to determine whether or not immunohistochemical staining for laminin-322 would be of value in distinguishing between keratoacanthomas, keratoacanthomas with areas of squamous cell carcinoma, and SCCs. MATERIAL AND METHODS: Seventy-four lesions were selected from the pathology archives of our hospital and divided into 4 groups: 20 keratoacanthomas without SCC, 20 keratoacanthomas with areas of squamous cell carcinoma, 20 invasive SCCs (8 with crateriform morphology) unrelated to keratoacanthoma, and 14 problem lesions (keratoacanthomas with areas suggestive of SCC). All 74 lesions were stained for laminin-322. RESULTS: Laminin-322 staining was strongly positive both in areas of SCC in keratoacanthomas with malignant transformation and in invasive SCCs (mostly at the invasive front of the SCC). However, in benign keratoacanthomas, it was only weakly positive and furthermore it was confined to isolated cells or small groups of cells. The 14 problem lesions were reexamined after laminin-322 staining and 8 were diagnosed as keratoacanthomas with incipient SCC and 6 as keratoacanthomas without SCC. CONCLUSIONS: Laminin-322 staining is different in keratoacanthomas and SCCs and would thus be a useful test for differentiating keratoacanthomas from both invasive SCCs and keratoacanthomas with areas of squamous cell carcinoma. It would also be of value in diagnosing keratoacanthomas with areas suggestive of SCC or with incipient SCC.

Hemorrhagic cholecystitis and hemobilia: two infrequent complications of systemic lupus erythematosus.

INTRODUCTION: the patients affected by systemic lupus erythematosus (SLE) often suffer gastrointestinal symptoms. The differential diagnosis should contemplate pathology of the gall bladder. We present the case of a patient with hemorrhagic lithiasic cholecystitis and hemobilia. CASE REPORT: 24 year old female diagnosed with SLE under treatment with Sintrom®, Dacortin® and Dolquine® that presented acute lithiasic cholecystitis and hemobilia with a distal calculus. Cholecystectomy and aperture of the ductus choledochus were performed allowing to confirm the hemobilia and to extract the calculus. DISCUSSION: The treatment of cholecystitis in the patients with SLE is controversial due to the fact that most reviewed cases have been solved with cholecystectomy, or in other cases with conservative treatment with corticosteroids. We believe that the presence of cholelithiasis in a patient with SLE with pain on the right hypochondrium and ultrasound confirming the suspicion of cholecystitis demands a surgical treatment since the cause may be vascular, lithiasic or combined. Besides, the possible complications will not respond to pharmaceutical treatment.

Hepatic angiosarcoma. Presentation of two cases.

Hepatic angiosarcoma is a rare primary tumor of the liver with a mesenchymal origin. Diagnosis is difficult because clinical manifestations and imaging studies are inconclusive. In many cases a diagnosis is obtained during necropsy, not being apparent during the course of disease. It is associated with several risk factors, but these contribute to explaining only a few of all reported cases. When clinical manifestations begin progression is often fast, and possibilities for curative treatment are limited.We report two cases of hepatic angiosarcoma. In the first one, our patient had an insidious initial course, and then suddenly presented with hepatic failure followed by acute respiratory distress. A diagnosis was reached during necropsy. In the second case, we initiated the study of a chronic liver disease using fine-needle aspiration biopsy, which showed findings suggestive of hepatic angiosarcoma. In the following weeks the patient started on a torpid clinic course, and died from multiple organ failure.

Assessing the short- and long-term prognosis of patients with cirrhosis and acute variceal bleeding.

OBJECTIVE: to evaluate the efficacy of various indicators in predicting short- and long-term survival in patients with cirrhosis and acute variceal bleeding. MATERIAL AND METHODS: prognostic indicators were calculated for a cohort of 201 cirrhotic patients with acute variceal bleeding hospitalized in our center, a third-level teaching hospital. The studied variables were: age, sex, etiology of cirrhosis, endoscopic findings, previous variceal bleeding episodes, human immunodeficiency virus (HIV) infection, hepatocellular carcinoma (HCC), infection during episode, and Child-Turcotte-Pugh (CTP) and Model for End-stage Liver Disease (MELD) scores within 24 hours of bleeding onset. Patients were followed up for at least 6 months until death, liver transplantation, or end of observation. RESULTS: median follow-up was 66.85 weeks (range 0-432.4). The 6-week, 3-month, 12-month and 36-month mortality rates were 22.9, 24.9, 34.3, and 39.8%, respectively. Age >= 65 years, presence of HCC, CTP score >=10, and MELD score >= 18 were the variables associated with mortality in the multivariate analysis. The accuracy of MELD scores as predictors of 6-week, 3-month, 12-month, and 36-month mortality was better than that of CTP scores (c-statistics: 6 week MELD 0.804, CTP 0.762; 3-month MELD 0.794, CTP 0.760; 12-month MELD 0.766, CTP 0.741; 36 month MELD 0.737, CTP 0.717). CONCLUSION: MELD and CTP scores together with age and a diagnosis of hepatocellular carcinoma are useful indicators to assess the short- and long-term prognosis of patients with acute variceal bleeding.

[Primary linitis plastica of the rectum]. / Linitis plástica de recto primaria.

Linitis plastica of the rectum is an uncommon entity that is difficult to diagnose due to the lack of mucosal lesions on endoscopy, the low diagnostic yield of biopsy and non-specific findings of barium radiology and computerized tomography. Rectal endoscopic ultrasonography has had a radical impact on the differential diagnosis of stenosing lesions of the rectum, among them linitis plastica, allowing diagnosis of this lesion even in patients with negative results of biopsy.

Outcome of silent lupus nephritis.

To analyze the long-term outcome in patients with silent lupus nephritis, we retrospectively studied 20 patients with systemic lupus erythematosus without clinical renal involvement who had renal biopsies in our unit between 1978 and 1986 and reviewed 193 cases reported between 1957 and 1995. Two patients of the current series were lost to follow-up. Mean follow-up in the other 18 was 13 +/- 3 years (range, 2 to 17). On kidney biopsy, nine had class I, six class II, one class IV, and two class V disease (WHO classification). Three patients with prior normal renal function died of nonrenal causes. During the study, the remaining 15 patients had normal renal function and urinalysis. Most patients from the literature had "mild" histologic lesions, but 30 had diffuse proliferative glomerulonephritis. Over an average of 46 months of follow-up from biopsy, renal survival rate and patient survival rate were 98% and 91%, respectively. Three patients died of end-stage renal failure. In conclusion, end-stage renal failure in patients with silent lupus nephritis is rare regardless of the histopathological renal lesions. Patients survival depends on nonrenal causes.

Cutaneous infection with rapidly growing mycobacteria in patients with systemic rheumatic disease.

We report two patients with systemic rheumatic disease being treated with steroids whose cases were complicated by subcutaneous nodules. In both, clinical and histological features suggested cutaneous infection and M. chelonae was isolated from skin specimens. Antibiotic therapy in both and surgery in one led to healing after a prolonged course. A review of the literature and our experience with these two patients suggest that rheumatic patients on steroid therapy are at risk of infection with these unusual pathogens. Knowledge of the risk factors and the distinctive picture of cutaneous mycobacteriosis should improve its diagnosis and therapy.