Disorders of the Mediastinum: Student/Resident Case Report Poster - Disorders of the Mediastinum |

Angiosarcoma After Treatment of Primary Mediastinal Yolk Sac Tumor FREE TO VIEW

Huy Do, MD; Janine Vintch, MD; Yvonne Carter, MD; James Yeh, MD; Justin Darrah, MD
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Harbor UCLA Medical Center, Torrance, CA

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):547A. doi:10.1016/j.chest.2016.08.635
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SESSION TITLE: Student/Resident Case Report Poster - Disorders of the Mediastinum

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Germ cell tumors are often composed of several different cell types. Rarely, these tumors can include sarcomatous components, such as rhabdomyosarcoma or angiosarcoma, which may be realized after failure of chemotherapy to reduce tumor size. Currently, given the poor response rate of systemic therapy for angiosarcoma, surgical resection is considered the most effective therapy. Monitoring response to therapy with tumor markers and imaging is important, as it may identify chemo-refractory malignancies.

CASE PRESENTATION: A 54-year-old male without prior medical history presented with dyspnea and 10-pound weight loss. His initial imaging identified a 5x13x8 cm anterior mediastinal mass; labs were notable for an elevated alpha fetoprotein (AFP). Biopsy of the mass revealed a yolk sac tumor. He was received four cycles of combination chemotherapy for non-seminomatous germ cell tumor with etoposide, ifosfamide, and cisplatin. Despite normalization of the AFP level, a repeat CT scan revealed that the mediastinal mass had increased in size. A repeat biopsy of the anterior mediastinum showed angiosarcoma; biopsy of a sacral lesion detected on SPECT scan also showed angiosarcoma, consistent with metastatic disease. The mediastinal tumor was partially resected, and the patient was subsequently initiated on paclitaxel.

DISCUSSION: This case demonstrates the importance of monitoring for progression of germ cell tumors with serial imaging, in addition to tumor markers. Similar to other case reports, this patient experienced normalization of his tumor markers, but demonstrated increasing tumor size on imaging. Detecting the expanding tumor prompted a concern for the presence tumor cells that were unaffected by treatment, which, in this case, were revealed to be those of angiosarcoma. Because angiosarcomas are very unlikely to respond to chemotherapy, the prognosis is poor. Novel therapies are under investigation.

CONCLUSIONS: Imaging is necessary in addition to tumor markers to identify unexpected outcomes in the course of treatment of germ cell tumors.

Reference #1: Mediastinal Germ Cell Tumors with an Angiosarcomatous Component: A Report of 12 Cases. Alejandro Luina Contreras, MD et al. Hum Pathol. 2010 June; 41 (6): 832-837

Reference #2: Poor prognosis of mediastinal germ cell cancers containing sarcomatous components. Gonzalez-Vela et al. Cancer. 1990 Sep 15;66(6): 1114-6.

Reference #3: Germ cell tumors with sarcomatous components: a clinicopathologic and immunohistochemical study of 46 cases. Malagon HD, Valdez AM, Moran CA, Suster S. Am J Surg Pathol. 2007 Sep;31(9):1356-62

DISCLOSURE: The following authors have nothing to disclose: Huy Do, Janine Vintch, Yvonne Carter, James Yeh, Justin Darrah

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