High risk Ewing's sarcomas. Effectiveness and toxicity of a regimen designed for children and used in adults

Introduction

Ewing's sarcoma is a highly malignant small round-cell tumor arising primarily from bone. Adults with this neoplasm have a poorer survival than that of children. The Spanish Society of Paediatric Oncology (SEOP) recommends that high-risk patients be treated with a combination of 5 different drugs (EVAIA schedule). The objective of this study is to evaluate response, overall survival, dose intensity and toxicity of the SSOP protocol in adult patients with high-risk Ewing's sarcoma.

Material and methods

Between 1997 and 2001, 16 adults with a histological diagnosis of high-risk Ewing's sarcoma were treated according to SEOP recommendations. Median age was 23 years (range: 15–42). Enneking staging system indicated 7 patients (44%) were II-B and 9 patients (56%) at stage III.

Results

The responses were: 12 complete responses (75%), 1 partial response (6%), 2 patients with stable disease and 1 disease progression. At the time of the present analysis, 6 patients (38%) were alive and free of disease, 1 (6%) was alive with disease and 9 patients had died (8 of them due to disease progression and 1 secondary to a second line chemotherapy administered after EVAIA). Median survival was 21 months (range: 9–67) and median time to progression was 14 months (range: 6–23).

Conclusions

EVAIA protocol is an aggressive regimen in which the most frequent toxicity is granulocytopenia and anaemia. In our experience no deaths were due to this protocol. The use of EVAIA in first-line therapy achieved 81% response rate with a high rate of long-term survival in this patient population with poor prognosis.     

Expectant management of severe preeclampsia and preeclampsia superimposed on chronic hypertension between 24 and 34 weeks' gestation

BACKGROUND: Timing of delivery is difficult to judge in preeclampsia. OBJECTIVE: To compare the differences of maternal and perinatal outcome of patients with severe preeclampsia and essential hypertension with superimposed preeclampsia, with expectant management at 24-34 weeks' gestation. STUDY DESIGN: A retrospective review of a conservative regime using intravenous magnesium sulfate, glucocorticoids and antihypertensive drugs, monitored by serial liver function tests, full blood count, coagulation profile, and renal function tests. Fetal status was assessed by daily non-stress test and ultrasound twice by week. RESULTS: A total number of 100 women had severe preeclampsia and 29 superimposed preeclampsia. The average pregnancy prolongation was 8.4 and 8.5 days, respectively. Oliguria, abruption placentae and HELLP syndrome were frequent complications similar in each group. There were no cases of eclampsia or disseminated coagulopathy in either group. The total neonatal survival rate was 93% in both groups. CONCLUSION: Expectant management is equally safe in both superimposed preeclampsia and severe preeclampsia of early onset.     

Lipomatous variant of nasopharyngeal angiofibroma: a case report

Nasopharyngeal angiofibroma is an uncommon benign vascular tumor that occurs in adolescent boys and young men. We describe an unusual histological variant of this entity characterized by a prominent adipose tissue component. The patient was a 35-year-old man with a 20-year history of nasal obstruction. Results of physical examination and angiography demonstrated a nasal mass consistent with a nasopharyngeal angiofibroma. We discuss the differential diagnoses pathologically for this lesion.     

The use of magnetic resonance in the study of sensorineural hearing loss in children

Although newborn screening of congenital hearing loss through otoacustic emissions allow prompt recognition, imaging techniques, such as CT and MRI are needed to get a morphological diagnosis. Furthermore they can be very useful in unilateral cases, whose clinical presentation is belated and more insidious. Our aim is to show the utility of MRI in the study of inner ear congenital anomalies, whose presentation is belated. Thus from a series of 88 consecutive patients in which a MRI was performed as screening of assymetric sensorineural hearing loss, we selected 6 cases aged between 6 and 20. Four of them showed an inner ear anomaly on MRI. We present these anomalies commenting the findings on CT and MRI. Imaging techniques are required to start hearing rehabilitation programs early on patients with bilateral inner ear anomalies. But also they are very useful in the evaluation of unilateral assymetric sensorineural hearing loss, in young patients, even if only some frequencies are damned, to determine the nature of hearing loss.