Satge D, Philippe E, Ruppe M, Levy J M, Lutz P, Walter P
Institut d'anatomie pathologique, Strasbourg, France.
Bull Cancer. 1988;75(4):373-84.
A cortical adrenal carcinoma arising during the neonatal period and associated with virilization recurred after an initial excision and is in complete remission after a follow-up period of 10 years. The authors have performed a review of the medical literature which has yielded 56 neonatal carcinomas including this one. More than half of these cases concerned the thyroid gland (15 cases) and the adrenal cortex (14 cases). These were followed by malignant melanomas (12 cases), carcinomas of the parotid gland (6 cases), oropharyngeal region (3 cases), adrenal medulla (2 cases) and stomach, liver, breast and cutaneous sweat gland (one case each). Six tumors were associated with congenital malformations which included Beckwith-Wiedeman's syndrome; 4 tumors arose from preexisting tissue lesions; in one case, oral contraceptives were taken during pregnancy. The main treatment of neonatal carcinomas is surgery. Local recurrences (7 cases) do not markedly affect the outcome, whereas metastases (12 cases) have a very unfavorable prognosis. Superficial tumors are diagnosed earlier and more frequently cured. Out of 44 cases for which the outcome is known, only 18 children survived.
一例新生儿期发生的伴有男性化的肾上腺皮质癌，在初次切除后复发，经过10年随访期后完全缓解。作者对医学文献进行了回顾，共发现56例新生儿期癌症，包括本病例。其中一半以上的病例涉及甲状腺（15例）和肾上腺皮质（14例）。其次是恶性黑色素瘤（12例）、腮腺癌（6例）、口咽区域癌（3例）、肾上腺髓质癌（2例）以及胃癌、肝癌、乳腺癌和皮肤汗腺癌（各1例）。6例肿瘤与先天性畸形有关，其中包括贝克威思-维德曼综合征；4例肿瘤起源于先前存在的组织病变；1例在孕期服用了口服避孕药。新生儿期癌症的主要治疗方法是手术。局部复发（7例）对预后影响不明显，而转移（12例）预后非常差。浅表肿瘤诊断较早，治愈的频率更高。在已知预后的44例病例中，只有18名儿童存活。
