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Factor VII deficiency is an autosomal recessive hereditary disorder characterized by a normal partial thromboplastin time and a prolonged prothrombin time. Definite diagnosis of this condition requires a specific Factor VII assay. Its quite uncommon occurrence in the newborn was first described by Alexander and associates in 1951. A one-day-old male, full-term newborn was presented here with skin pallor, respiratory distress and bitemporal bulging masses noted immediately after birth. Computed tomography scan of the brain revealed a subgaleal hematoma and subarachnoid hemorrhage. Laboratory studies revealed prolonged prothrombin time and a Factor VII level less than 1%. Literature on the clinical manifestation and management of Factor VII deficiency is reviewed briefly.

A 1 year and 7 months old girl had suffered from chronic diarrhea for 6 weeks. A huge abdominal mass was found. She received subtotal resection of the tumor and the histological picture showed ganglioneuroblastoma. Then, 12 courses of chemotherapy (including cyclophosphamide, doxorubicin cisplatin and etoposide) were given for 8 months, but the residual tumor mass over the previous surgical area was still noted by CT scan. A second excision of the tumor was performed and histology showed a picture of ganglioneuroma incidentally. Chemotherapy was discontinued. The patient is still alive and well for more than one year.

Congenital brain tumor is a rare disease in the neonatal period. According to the literatures, they comprise only about 1% of childhood brain tumors. Among the congenital brain tumors, 10%-25% are astrocytomas. Anaplastic astrocytoma is one of the malignant glioma. The prognosis is usually not good in the childhood or adult stage. We report one case of congenital anaplastic astrocytoma who received combination chemotherapy, including vinblastine, cisplatin and etoposide following subtotal resection of tumor. After chemotherapy, he got a favorable outcome. And now, he is still no evidence of tumor recurrence for two years.

Superior vena cava syndrome (SVCS) is rare in children. In Veterans General Hospital-Taipei, a total of 364 cases of SVC syndrome were diagnosed in the past 12 years. Of them only seven cases were younger than 18 years of age, ranging from 6 to 17 years, and they were all caused by mediastinal tumor. The underlying malignancy included malignant lymphoma in 4, teratocarcinoma in one and unknown in 2. The most common initial symptom was cough, followed in order of frequency by chest discomfort or neck mass. Dyspnea, orthopnea, swelling of head and neck, and venous engorgement might develop gradually within one to three weeks. Of the reported seven cases, two cases received immediate resuscitation upon arrival but expired in 1-2 hours. The other five cases received treatment with intravenous steroid as well as chemotherapy, and three cases also received committent emergent radiotherapy. Two of them expired 4 months and 2 years after treatment, respectively. Of the two surviving cases, one has received a complete course of chemotherapy and the other is still under regular chemotherapy in our hospital. Both of them are stable till now.

From January 1981 to January 1986, 34 children between 2 and 14 years of age were diagnosed as non-Hodgkin's lymphoma by the Rapport's classification at the Department of Pediatrics of Veterans General Hospital, Taipei, R.O.C. These patients were retrospectively analysed. There was male predominance with a male to female ratio of 2.4:1. The most common symptom was the palpable mass (29 cases, 85.3%) involved the neck and abdomen equally. All the cases were diffuse patterns, DUL (diffuse undifferentiated lymphoma) and DPDL (diffuse poorly differentiated lymphoma) both were the common subtypes of histological patterns. Twenty-eight cases (82%) were in the advanced stage (stage III and IV). The incidence of BM and CNS involvement was 56% and 29.4%, respectively. CNS involvement demonstrated a higher mortality of 100%. Surgery, radiation, and chemotherapy were mainly used for stage I-II and chemotherapy for stage III-IV. The 3-year survival rate of the whole series was 26.5%, and stage I, II, III, and IV was 100%, 50%, 33%, 9%, respectively. The causes of death were CNS involvement (10 cases, 40%), extensive involvement (7 cases, 28.0%), and sepsis (3 cases, 12.0%). This study shows that NHL in children are usually diffuse rather than follicular in histological patterns, often present a palpable mass involved the cervical or abdominal area, and commonly are widespread and tend to disseminate early.