Dieckmann Klaus-Peter, Anheuser Petra, Gehrckens Ralf, Wilczak Waldemar, Sauter Guido, Höflmayer Doris
Department of Urology, Albertinen-Krankenhaus Hamburg, Süntelstrasse 11a, 22457 Hamburg, Germany.
Department of Diagnostic Radiology, Albertinen-Krankenhaus Hamburg, Süntelstrasse 11a, 22457 Hamburg, Germany.
Case Rep Oncol Med. 2017;2017:2457023. doi: 10.1155/2017/2457023. Epub 2017 Mar 7.
Somatic type malignancy (STM) occurs in 2% of all germ cell tumours (GCTs). The prognosis is unfavourable and the origin is poorly understood. Pathogenetic hypotheses involve direct transformation of teratoma, origin from totipotent cancer cells, or derivation from yolk sac tumour elements. A 31-year-old patient was cured from testicular seminoma clinical stage IIc by orchiectomy and cisplatin-based chemotherapy. Nine years later, he experienced a late relapse with a mass sized 5 × 6 cm located at the former metastatic site. As no remission occurred after chemotherapy with three cycles of cisplatin, ifosfamide and etoposide, the mass was surgically resected. Histologically, the specimen consisted of neurofibroma with areas of malignant peripheral nerve sheath tumour and spots with mature bone formation. FISH analysis disclosed isochromosome 12p in the majority of evaluated cells suggesting somatic type malignancy (STM) of GCT. The patient is well 1 year after surgery. The pathogenesis of this STM remains enigmatic. The origin from GCT was evidenced by documentation of isochromosome 12p. Unrecognized teratomatous elements in the primary and totipotent cancer cells surviving the first chemotherapy could be hypothesized to represent the origin. STM developing from seminoma cells would be another novel hypothesis.
体细胞型恶性肿瘤（STM）发生于所有生殖细胞肿瘤（GCT）的2%中。其预后不佳，起源也知之甚少。发病机制假说包括畸胎瘤的直接转化、全能癌细胞的起源或卵黄囊瘤成分的衍生。一名31岁患者因睾丸精原细胞瘤临床IIc期接受睾丸切除术和顺铂化疗后治愈。九年后，他在原转移部位出现了一个大小为5×6厘米的肿块，出现晚期复发。在用顺铂、异环磷酰胺和依托泊苷进行三个周期化疗后未出现缓解，遂对该肿块进行了手术切除。组织学检查显示，标本由神经纤维瘤组成，伴有恶性外周神经鞘瘤区域和成熟骨形成的斑点。荧光原位杂交（FISH）分析显示，在大多数评估细胞中存在12号染色体短臂等臂染色体，提示为生殖细胞肿瘤的体细胞型恶性肿瘤（STM）。患者术后1年情况良好。该STM的发病机制仍然成谜。12号染色体短臂等臂染色体的存在证明了其起源于生殖细胞肿瘤。可以推测，原发肿瘤中未被识别的畸胎瘤成分以及首次化疗后存活的全能癌细胞可能是其起源。由精原细胞瘤细胞发展而来的STM将是另一个新的假说。
