[Neonatal neuroblastoma complicated with a threatening tumoral hepatomegaly treated by irradiation: No sequel 20 years later]. / Neuroblastome néonatal, compliqué d'une hépatomégalie tumorale menaçante, traité par irradiation : absence de séquelle 20ans après.

In this article, we report the case of a newborn who presented a life-threatening hepatomegaly with respiratory distress at 12 days of life, complicating a metastatic neuroblastoma. Low-dose liver radiotherapy was performed in emergency in order to decompress. Chemotherapy has also been delivered due to a tumoral relapse 1 month after radiotherapy. After a follow-up of 20 years, this young woman is still in complete remission, with no long-term sequelae.

Repeated and preemptive palliative radiotherapy of symptomatic hepatomegaly in a patient with advanced myelofibrosis.

BACKGROUND: Patients with advanced myelofibrosis often suffer from symptomatic extramedullary hematopoiesis in spleen and/or liver. In case of drug-refractory disease splenomegaly is treated surgically, whereas hepatomegaly is palliated by radiotherapy (RT). CASE REPORT: A 56-year-old man with advanced and drug-refractory myelofibrosis suffered from extensive hepatomegaly with severe upper abdominal pain, satiety, weight loss, and fatigue 1.5 years after splenectomy. The patient was treated periodically with fractionated RT to the liver in order to obtain symptom control and to prevent severe symptom recurrence. RESULTS: After 2 Gy fractionated RT to a treatment field encompassing nearly the whole liver, symptoms improved and liver size decreased without severe side effects. This treatment regimen was successfully conducted 3 times in trimonthly intervals. Because symptoms recurred periodically, we then continued RT on a preemptive basis in monthly intervals and with single-dose irradiation. The patient responded well to 1 Gy preemptive single-dose RT to the liver, but not to 0.5 Gy single-dose partial liver irradiation. CONCLUSION: RT is effective in palliation of hepatomegaly in advanced myelofibrosis. Even preemptive RT can benefit selected patients with advanced disease and periodical recurrence of symptoms.

Radiation therapy for symptomatic hepatomegaly in myelofibrosis with myeloid metaplasia.

OBJECTIVE: To describe the experience with liver irradiation in advanced cases of myelofibrosis with myeloid metaplasia (MMM). METHODS: Over a 20-yr period, 14 patients with MMM were treated with a total of 25 courses of liver, abdominal, or abdominal and pelvic irradiation for symptomatic hepatomegaly with (5 patients) or without (9 patients) ascites. All 14 patients had advanced disease and 11 (79%) had previous splenectomy. The median radiation therapy (RT) dose per course was 150 cGy (range 50-1000) administered at a median of six fractions. Four patients received two to six courses. RESULTS: Twelve of the 14 patients (86%) had a transient (median 3 months) subjective response from RT. However, in only 35% of these was there a transient (median 3 months) decrease in palpable liver size. Four of the five patients with ascites experienced a short-term response from RT. Eight of the 13 patients suitable for evaluation (62%) had treatment-associated cytopenia, often in the form of anemia and/or thrombocytopenia. At last follow-up, 10 patients (71%) had died after a median of 7 months (range 0.1-23) and 4 were alive at 3, 20, 33, and 57 months after RT. CONCLUSIONS: Low-dose abdominal RT for symptomatic hepatomegaly or ascites associated with advanced-stage MMM is myelosuppressive and provides only temporary and mainly subjective and short-lived relief.

Treatment by splenic irradiation in 22 chronic lymphocytic leukemia patients.

Twenty-two patients with chronic lymphocytic leukemia, score 2 according to Rai et al. (10), who received only a course of splenic irradiation are reviewed. Splenic doses ranged from 420 to 1080 rad. Response to splenic irradiation was rated by evaluating peripheral lymphocytosis, hepatosplenomegaly, adenomegaly and disease-related symptoms. Following splenic irradiation, 8 patients showed a significant reduction in splenomegaly; 7 patients showed a significant reduction in peripheral lymphocytosis (less than 10,000/mm3), which has lasted from 15-42 months without any other treatment. In 14 patients, response to splenic irradiation was partial, and it has successively been necessary to treat 12 patients with chemotherapy. Methods of splenic irradiation, survival, clinical and hematologic behavior are discussed in detail.