Unresectable Metastatic Testicular Germ Cell Tumor With Low-grade Neuroglial Neoplasm, Stable After Radiation and Chemotherapy.
Unresectable Metastatic Testicular Germ Cell Tumor With Low-grade Neuroglial Neoplasm, Stable After Radiation and Chemotherapy.
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不可切除的转移性睾丸生殖细胞肿瘤，伴有低度神经胶质肿瘤，放疗和化疗后稳定。
DOI：
10.1097/pai.0000000000001081
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发表时间：
2023
期刊：
影响因子：
--
通讯作者：
Matoso,Andres
中科院分区：
文献类型：
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作者：
Tabibi,Seena;Kamanda,Sonia;Llosa,Nicolas;Pratilas,ChristineA;Duncan,BrynnB;Esteves,RodrigoSalgado;Matoso,Andres
我们介绍了一名 18 岁男性患者，其表现为 16 厘米的睾丸肿瘤，同时肺部多处病变，右侧骨盆累及坐骨/耻骨，影像学检查显示骨盆淋巴结肿大，怀疑有转移性疾病。组织学和免疫组织化学检查显示胚胎型神经外胚层肿瘤（体细胞型恶性肿瘤，10%）起源于由畸胎瘤（50%）、胚胎癌（10%）和卵黄囊肿瘤（30%）组成的恶性混合生殖细胞肿瘤。经过化疗和放疗后，重复成像显示右侧骨盆侧壁肿块的大小从初次诊断后 11 个月时的 40 毫米减小到初次诊断后 22 个月时的 18 毫米。具有溶骨成分的右骨盆内侧大腿壁肿块显示其尺寸略有增加，从最初诊断后 11 个月时的 151 毫米增加到诊断后 22 个月时的 154 毫米。在初次诊断后 3、10 和 26 个月进行的活检中，这种溶解性病变始终显示出由低级神经胶质细胞分化组成的肿瘤。这是医学文献中的第一例，其中由低度神经胶质肿瘤组成的残留恶性生殖细胞肿瘤位于不适合切除且没有显着发病率的部位。肿瘤最初通过传统的一线放化疗方案消退，但通过第二次化疗方案重新生长并稳定下来。该病例的临床病程要求考虑对具有类似特征的病例采取主动监测方法。 背景我们之前发表了一个病例系列，其中包括 13 例睾丸生殖细胞肿瘤合并神经胶质细胞肿瘤患者，包括低级别星形细胞瘤、双细胞星形细胞瘤、间变性星形细胞瘤、神经节神经瘤、胶质母细胞瘤和胶质肉瘤。 1 该系列的大多数肿瘤都是腹膜后淋巴结切除术，转移灶被认为是化疗后复发或持续存在的疾病。在原发性肿瘤中检测到较小比例的神经胶质肿瘤，总是与其他恶性生殖细胞肿瘤（最常见的是畸胎瘤）相关。 1, 2 中枢神经系统中出现的神经胶质肿瘤的治疗方法是切除，然后进行放疗和化疗，主要是因为无法在不引起显着发病率的情况下完全切除它们。化疗或放疗对睾丸生殖细胞肿瘤中产生的神经胶质肿瘤的影响尚不清楚。截至我们之前发表文章时，没有患者出现需要辅助治疗的不可切除的神经胶质肿瘤。在这里，我们介绍了第一例位于不可切除部位的转移性睾丸生殖细胞肿瘤中的神经胶质肿瘤，并描述了其在多模式治疗后的演变。
We present an 18-year-old male patient who presented with a 16 cm testicular tumor alongside multiple lesions in the lungs, right pelvis with involvement of the ischio/pubic bone, and enlarged pelvic lymph nodes on imaging, suspicious for metastatic disease. Histologic and immunohistochemical examinations revealed an embryonic type neuroectodermal tumor (somatic-type malignancy, 10%) arising in a malignant mixed germ cell tumor composed of teratoma (50%), embryonal carcinoma (10%) and yolk sac tumor (30%). After treatment with chemotherapy and radiation, repeat imaging demonstrated a right pelvic sidewall mass that decreased in size from 40 mm at 11 months after the initial diagnosis to 18 mm at 22 months after the initial diagnosis. A right pelvis medial thigh wall mass that had a lytic bone component showed a slight increase in size from 151 mm at 11 months after the initial diagnosis to 154 mm at 22 months after the diagnosis. On biopsies performed at 3, 10, and 26 months after the initial diagnosis, this lytic lesion consistently demonstrated a neoplasm composed of low-grade neuroglial differentiation. This is the first case in the medical literature where a residual malignant germ cell tumor consisting of low-grade neuroglial neoplasm is in a site that is not amenable to resection without significant morbidity. The tumor initially regressed with the traditional first-line chemo-radiotherapy regimen but regrew and stabilized with a second regimen of chemotherapy. The clinical course of this case invites consideration for an active surveillance approach in cases with similar characteristics.BACKGROUNDWe previously published a case series of 13 patients with testicular germ cell tumors with neuroglial neoplasms, including low-grade astrocytomas, gemistocytic astrocytoma, anaplastic astrocytomas, ganglioneuroma, glioblastomas, and gliosarcoma. 1 Most of the tumors of that series were resections of retroperitoneal lymph nodes with metastases that were considered recurrences or persistent disease after chemotherapy. A smaller proportion of neuroglial neoplasms were detected in primary tumors, always associated with other malignant germ cell tumors, most frequently teratomas. 1, 2 Neuroglial neoplasms arising in the central nervous system are treated with resection followed by radiation and chemotherapy, mainly due to the inability to remove them completely without causing significant morbidity. The effect of chemotherapy or radiation therapy in neuroglial neoplasms arising in testicular germ cell tumors is unknown. At the time of our previous publication, none of the patients had presented unresectable neuroglial neoplasms requiring adjuvant therapy. Here, we present the first case of neuroglial neoplasm in a metastatic testicular germ cell tumor in an unresectable site and describe its evolution after multimodal therapy.