Dodion P, De Valeriola D
Service de médecine, Institut Jules-Bordet, Université Libre de Bruxelles, Belgique.
Bull Cancer. 1989;76(9):939-46.
Cancers of unknown origin represent approximately 5% of all cancers and are therefore as frequent as some solid tumors such as gastric or pancreatic cancers. The diagnosis of cancer of unknown origin should be based on a detailed pathological examination including immunohistochemical techniques and electron microscopy; hormonal receptors should also be measured. Besides detailed medical history and physical examination, only a few additional tests should be carried out: routine chemistry including the assay of HCG, alphafoetoprotein and specific antigen of the prostate, chest X-ray, thyroid scan, mammography and abdominal CT scan. Other tests are generally not of sufficient specificity and sensitivity. Unknown primary tumors arising in the cervical area are frequently squamous cell carcinomas corresponding to occult primary tumors of the upper aerodigestive mucosae and are efficiently treated by cervicofacial radiotherapy or lymph node dissection. Women presenting with axillary lymph nodes with no obvious primary tumor should be treated according to the guidelines used for breast cancer. The patients with inguinal lymph nodes of unknown origin are usually treated with radiation therapy. The syndrome of germinal tumors of extragonadic origin corresponds to cases of undifferentiated or poorly differentiated carcinomas in patients under 50 years of age and with one of the following characteristics: involvement of the median organs, lung involvement, lymph node involvement or increase of alphafoetoprotein or HCG. The therapeutic approach recommended for these patients consists of the chemotherapeutic combination used for testicular cancer. For all other patients, the prognosis remains poor. Patients with local symptoms may be treated by radiation therapy; others may receive a combination of fluorouracil, doxorubicin and mitomycin.
原发灶不明的癌症约占所有癌症的5%,因此其发病率与某些实体肿瘤(如胃癌或胰腺癌)相当。原发灶不明癌症的诊断应基于详细的病理检查,包括免疫组化技术和电子显微镜检查;还应检测激素受体。除详细的病史和体格检查外,只需进行几项额外检查:常规化学检查,包括人绒毛膜促性腺激素、甲胎蛋白和前列腺特异性抗原检测、胸部X线检查、甲状腺扫描、乳房X线摄影和腹部CT扫描。其他检查通常特异性和敏感性不足。发生在宫颈区域的原发灶不明肿瘤通常为鳞状细胞癌,对应于上呼吸消化道黏膜的隐匿性原发肿瘤,可通过颈面部放疗或淋巴结清扫有效治疗。出现腋窝淋巴结且无明显原发肿瘤的女性应按照乳腺癌的治疗指南进行治疗。原发灶不明的腹股沟淋巴结患者通常采用放射治疗。非生殖腺来源的生殖细胞肿瘤综合征对应于50岁以下、具有以下特征之一的未分化或低分化癌患者:中位器官受累、肺部受累、淋巴结受累或甲胎蛋白或人绒毛膜促性腺激素升高。推荐给这些患者的治疗方法包括用于睾丸癌的化疗联合方案。对于所有其他患者,预后仍然很差。有局部症状的患者可采用放射治疗;其他患者可接受氟尿嘧啶、阿霉素和丝裂霉素联合治疗。