Three distinct hematological malignancies from a single germ cell tumor: a case report
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CASE REPORT
Three distinct hematological malignancies from a single germ cell tumor: a case report M. Spencer Chapman1,2* , P. C. May3, E. Olavarria2 and E. Nadal Melsio4
Abstract Background: The association between non seminomatous germ cell tumors (GCTs) and hematological malignancies of rare lineage has been described in the literature. In some of these cases there is evidence that the leukemia derives from a pluripotent primitive clone present in the original germ cell tumor. Case presentation: We present a highly unusual case of a 23-year-old man of South Asian origin with a history of Klinefelter’s syndrome who initially developed mediastinal non seminomatous GCT. Following treatment with surgery and standard chemotherapy he went on to develop three different hematological malignancies of distinct lineages in sequential fashion over a short time period. Despite treatment with multiple intensive chemotherapy regimens and a matched unrelated donor allogeneic stem cell transplant, he died 41 months after initial diagnosis of his GCT and 10 months after the first diagnosis of hematological malignancy. Conclusions: This is an extreme case that highlights the pluripotency and aggressiveness of these GCT-derived hematological malignancies, and the need for novel therapeutic approaches. Keywords: Germ cell tumor, Teratoma, Mediastinal non seminomatous germ cell tumor, Acute myeloid leukemia, AML, Burkitt’s lymphoma, Klinefelter’s syndrome, Malignant transformation, Clonal evolution Background The majority of male germ cell tumors (GCTs) are located within the testis, however extra-gonadal GCTs represent 1 to 5% of all GCTs and have varied morphology and anatomic location including the anterior mediastinum and retroperitoneum [1]. As a malignancy of primitive cells, GCTs display pluripotentiality for extraembryonal and embryonal (somatic) differentiation [2, 3]. Among all GCTs, teratomas show the most vivid and varied patterns of somatic differentiation with multiple mature and immature elements [4]. Rarely, teratomas undergo further “malignant transformation” whereby aggressive proliferation of a differentiated non-germ cell tissue develops [5]. This includes neurogenic, epithelial, mesenchymal and hematopoietic malignancies which are *Correspondence: [email protected] 1 Cancer, Ageing & Somatic Mutation Programme, Wellcome Sanger Institute, Wellcome Genome Campus, Hinxton, Cambridge, UK Full list of author information is available at the end of the article
often indistinguishable from their spontaneous counterparts, their GCT origin shown only by genetic markers [6, 7]. Where hematopoietic malignancies develop, they are frequently unusual histologic subtypes (e.g. megakaryoblastic leukemia, histiocytic malignancies) and follow an aggressive clinical course [8]. However, they generally remain of one histologic subtype over time. We report an unusual case of a 23-year-old man with Klinefelter’s syndrome and previously treated mediastinal teratoma who went on to develop three different hematologica
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