Thymoma associated with aregenerative and aplastic anemia in a five-year-old child

✍ Scribed by Aleksander Talerman; Alvaro Amigo

• Publisher: John Wiley and Sons
• Year: 1968
• Tongue: English
• Weight: 787 KB
• Volume: 21
• Category: Article
• ISSN: 0008-543X
• DOI: 10.1002/1097-0142(196806)21:6<1212::aid-cncr2820210624>3.0.co;2-9

✦ Synopsis

A case of benign thymoma presenting with aregenerative anemia in a 5-year-old boy is reported. T h e tumor was successfully excised from the anterior mediastinum. The histology showed that it was a thymoma of mainly epithelial type with a number of spindle cell areas. Four weeks after thymectomy the child developed a severe aplastic anemia with pancytopenia a n d i n spite of treatment with blood transfusions, prednisone and testosterone the patient died of generalized bleeding tendency. Thymomas in children younger than 11 years are rare a n d the association of thymoma and aregenerative or aplastic anemia in the first decade has not been reported in the literature.

N RECENT YEARS SEVERAL REPORTS OF THYMO-I mas have been published.1, 2, 5-8, l 5 All these studies have shown that thymoma is only occasionally observed in patients younger than 20 years and appears to be even less common in the first decade. This is supported by other investigators.3~ 9916 T h e association of thymoma with myasthenia gravis and occasionally with Cushing's syndrome has been noted for some time.2912

T h e association between a thymoma and aregenerative or aplastic anemia is now quite well established.431~ 13 T h e first description of this syndrome has been ascribed to Polayes and LedererlO though doubt has been expressed as to whether the tumor mentioned by them was in fact a thymoma. 4,13 In 1964 Schmid et al.11 reviewed the literature and found 42 cases of thymoma associated with red cell agenesis or aplastic anemia; they added four new cases.

Recently in a comprehensive review of the world literature Hirst and Robertson4 found 56 cases which were considered acceptable using the following criteria: