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M. Triantafyllou
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P3.08 - Poster Session/ Thymoma, Mesothelioma and Other Thoracic Malignancies (ID 226)
- Event: WCLC 2015
- Type: Poster
- Track: Thymoma, Mesothelioma and Other Thoracic Malignancies
- Presentations: 1
- Moderators:
- Coordinates: 9/09/2015, 09:30 - 17:00, Exhibit Hall (Hall B+C)
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P3.08-027 - Teratoid Wilms' Tumor of the Mediastinum in an Adult (ID 111)
09:30 - 09:30 | Author(s): M. Triantafyllou
- Abstract
Background:
Teratoid Wilms’ tumor is an uncommon histologic variant of nephroblastoma, which is mainly characterized by a predominance of heterologous components within the tumor mass, and typically located in the kidney. Extrarenal forms of this neoplasm are extremely rare and have been previously described almost exclusively in pediatric patients.
Methods:
We herein describe the clinical, imaging, histological and immunohistochemical features of a mediastinal teratoid Wilms’ tumor in a 63-year old female. Diagnosis was established following surgical resection and histopathological evaluation of the tumor specimen, along with documentation of complete absence of a primary renal lesion.
Results:
Microscopic examination of representative tumor sections showed the typical WT architecture consisting of blastemal, mesenchymal and epithelial components (fig.1). More than 50% of the tumor’s volume was occupied by a variety of heterologous elements. Immunohistochemistry showed positive staining of the epithelial tumor cells for pankeratin (fig.2a), while the blastematous component stained positive for CD56 (fig.2b), CD99 (fig.2c) and WT1 (fig.2d). The patient received one preoperative course of carboplatin etoposide, as well as eight and four postoperative courses of carboplatin-etoposide and cyclophosphamide-doxorubicin, respectively. Disease progression was noted 15 months following administration of the first chemotherapy cycle, and the patient died 23 months after her initial presentation. Figure 1 Figure 2
Conclusion:
Identification of the typical triphasic WT histology along with a predominant heterologous differentiation –but without evidence of unequivocal heterotopic organogenesis- as well as documentation of complete absence of a primary renal neoplasm, are all required to establish the diagnosis of this extremely rare tumor.
