Teratoma with malignant transformation: Diverse malignant histologies arising in men with germ cell tumors
Teratoma with malignant transformation: Diverse malignant histologies arising in men with germ cell tumors
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DOI：
10.1016/s0022-5347(01)64035-7
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发表时间：
1998-01-01
影响因子：
6.6
通讯作者：
Reuter, VE
中科院分区：
文献类型：
--
作者：
Motzer, RJ;Amsterdam, A;Reuter, VE
目的：畸胎瘤恶性转化是指一种生殖细胞肿瘤，其中体细胞畸胎瘤成分在形态上变得恶性并发展为侵袭性生长。我们评估了频谱的组织学，染色体异常和恶性transformation.Materials和方法的畸胎瘤患者的临床结局：我们确定了46例生殖细胞肿瘤恶性转化的形态标准，组织学，疾病的程度和治疗与生存相关。通过常规细胞遗传学或分子遗传学技术对12例患者的肿瘤进行了研究，以确定等染色体12 p [i（12 p）]，这是生殖细胞肿瘤的标志物，以及其他染色体异常。21例（44%）首次发现恶变部位在原发灶，20例（43%）转移部位，5例（10%）两个部位均存在。肉瘤是最常见的组织学类型，在29例患者（63%）中发现横纹肌肉瘤是最常见的亚型。17例肿瘤（37%）包含肉瘤以外的实体瘤组织学，腺癌和原始神经外胚层肿瘤是最常见的组织学。4例纵隔生殖细胞瘤肉瘤患者同时患有血液系统恶性肿瘤，包括纵隔原发肿瘤的非霍奇金淋巴瘤病灶（1例）和脾脏或骨髓的非淋巴细胞白血病（3例）。畸胎瘤恶变成分局限于睾丸或腹膜后完全切除的患者比远处转移或肿瘤未完全切除的患者生存时间长（p = 0.003）。12例肿瘤中有11例（包括腺癌、原始神经外胚层瘤、肉瘤和白血病）发现了与生殖细胞肿瘤相关的染色体异常（i [12p]）。除了i（12 p），在4个肿瘤中检测到转化组织学特征的染色体重排。结论：生殖细胞肿瘤可发生多种非生殖细胞肿瘤，包括肉瘤、腺癌、原始神经外胚层瘤和白血病，这些肿瘤中的染色体异常包括i（12 p），反映生殖细胞肿瘤的克隆性，以及与组织学转化相关的染色体异常。这些肿瘤不像生殖细胞肿瘤那样对含顺铂的化疗方案有反应。治疗应根据转化组织学的标准管理中使用的方法进行调整，手术切除是治疗的主要手段。
Purpose: Teratoma with malignant transformation refers to a form of germ cell tumor in which a somatic teratomatous component becomes morphologically malignant and develops aggressive growth. We evaluated the spectrum of histologies, chromosomal abnormalities and clinical outcome in patients with teratoma with malignant transformation.Materials and Methods: We identified 46 patients with germ cell tumor meeting morphologic criteria for malignant transformation, Histology, disease extent and treatment were correlated with survival. Tumors in 12 patients were studied by conventional cytogenetics or molecular genetic techniques for the isochromosome 12p [i(12p)], a marker for germ cell tumor, as well as other chromosomal abnormalities.Results: The site of first detection of malignant transformation occurred in the primary tumor of 21 cases (44%), at a metastatic site in 20 (43%) and in both sites in 5 (10%). Sarcoma was the most frequent histology, identified in 29 patients (63%) with rhabdomyosarcoma the most common subtype. Seventeen tumors (37%) contained a solid tumor histology other than sarcoma, with adenocarcinoma and primitive neuroectodermal tumor as the most common histologies. Four patients with mediastinal germ cell tumor containing sarcoma also had hematological malignancies, including a focus of nonHodgkin's lymphoma in the mediastinal primary tumor (1) and nonlymphocytic leukemia in spleen or bone marrow (3).Patients who had teratoma with malignant transformation components confined to the testis or retroperitoneum completely resected experienced a longer survival than those with distant metastases or incompletely resected tumors (p = 0.003). Chromosomal abnormalities associated with germ cell tumor (i [12p]) were identified in 11 of 12 tumors containing adenocarcinoma, primitive neuroectodermal tumor, sarcoma and leukemia. In addition to i (12p), chromosomal rearrangements characteristic of the transformed histology were detected in 4 tumors. Conclusions: A variety of nongerm cell histologies, including sarcoma, adenocarcinoma, primitive neuroectodermal tumor and leukemia, may occur in association with germ cell tumor.Chromosomal abnormalities in these tumors include i (12p), reflecting germ cell tumor clonality, as well as chromosomal abnormalities associated with the transformed histology. These tumors do not respond like germ cell tumor to cisplatin-containing chemotherapy regimens. Treatment should be tailored according to that used in standard management of the transformed histology, and surgical resection is the mainstay of therapy.