Eyelid metastasis from mediastinal teratoma with malignant transformation

A 29-year-old Hispanic male with an unremarkable past medical history, was diagnosed with a mediastinal germ cell tumor (GCT) of mixed histology including choriocarcinoma, embryonal carcinoma and teratomatous components. At the time of diagnosis the human chorionic gonadotropin (HCG) was 287 IU/l [normal range 52], alphafetoprotein (AFP) 421 mgr/l [normal range 55] and LDH 215 IU/l [normal range 5190]. No testicular primary or other metastasic sites were observed. The patient received treatment with a combination of bleomycin, etoposide and cisplatin [BEP] for four cycles achieving a normalization of the tumoral markers but only a mild reduction of the tumor volume. Shortly afterwards due to radiological progression, an R0 resection of the mediastinal mass was performed. Pathologic examination revealed a mixed histology with mature teratoma, gastrointestinal adenocarcinoma and sarcoma components together with extensive areas of fibrosis and necrosis. Two months later, the patient relapsed presenting with multiple pulmonary nodules and one suspicious liver lesion. A hepatic biopsy confirmed the diagnosis of teratoma with malignant transformation in keeping with sarcoma. The patient was then treated with a combination of vinblastine, ifosfamide and cisplatin (VeIP) with no response after two cycles. During treatment course he presented with a painless small nodule on his left inferior eyelid measuring around 8 mm (Figure 1a and b). A biopsy of this nodule (Figure 2) revealed areas of normal conjunctiva (white arrow) along with sarcomatoid components (black arrow) in keeping with metastatic sarcoma. Further systemic treatment options were discussed with the patient who rejected any active approach. To date, four months after diagnosis of this metastasis, the patient remains clinically stable with no symptoms. Though GCTs account for only 1 2% of all human malignancies they are the most common tumors diagnosed in men ages 15 to 34 years [1]. Only 10% of GCTs present as extragonadal malignancies arising in the mediastinum, retroperitoneum and central nervous system in order of frequency. The biology of GCTs is unique having the capacity to display totipotential differentiation ranging from embryonal carcinoma to extraembryonic cell types (i.e. yolk sac tumor or choriocarcinoma) or to somatic cell types (i.e. teratoma) [2]. Teratomas are tumors that display somatic elements with diverse differentiation stages (i.e. mature and immature teratomas). On rare occasions the teratomatous component in a GCT may undergo malignant transformation leading to histology indistinguishable from a somatic malignancy. Examples of histologic transformed cell types include rhabdomyosarcoma,