Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2021 May 28:8:666389.
doi: 10.3389/fsurg.2021.666389. eCollection 2021.

Posterior Mediastinal Epithelioid Angiosarcoma Arising in Schwannoma: A Case Report and Review of the Literature

Affiliations
Case Reports

Posterior Mediastinal Epithelioid Angiosarcoma Arising in Schwannoma: A Case Report and Review of the Literature

Yingming Xiang et al. Front Surg. .

Abstract

Epithelioid angiosarcoma arising in schwannoma is an extremely rare mesenchymal tumor that accounts for only 1 to 2% of all sarcomas. This type of tumor occurs in all parts of the body, most often in the skin and soft tissues and rarely in the mediastinum. The present study describes the case of an asymptomatic, 58-year-old male who presented with epithelioid angiosarcoma in the posterior mediastinum during a physical examination. Enhanced computed tomography of the chest revealed a 3.5 × 3.1-cm mass in the posterior mediastinum. Thoracoscopic mediastinal mass resection was performed under general anesthesia due to the possibility that the tumor was malignant. Pathological examination revealed the presence of angiosarcoma and schwannoma components. Immunohistochemical staining for cluster of differentiation (CD) 31, CD34, early growth response (EGR), vimentin, Sry-related HMG box (SOX)-10 and S-100 was strongly positive. The patient recovered and was discharged on postoperative day 5. Two months postsurgery, the patient returned for evaluation, and no evidence of tumor recurrence was observed.

Keywords: angiosarcoma; mediastinum; schwannoma; surgery; thoracoscopic.

PubMed Disclaimer

Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Computed tomography (A: axial, C: coronal, D: sagittal) of the chest showed a mass in the posterior mediastinum (red arrow). The enhancement scan (B) was uneven and slightly enhanced.
Figure 2
Figure 2
(A) Hematoxylin-eosin staining showed that epithelioid angiosarcoma had abundant eosinophilic cytoplasm and vesicular nuclei, with prominent nucleoli (red arrow) (200×). (B) Hematoxylin-eosin staining showed that the schwannoma cells were spindle, with abundant cytoplasm and insignificant nucleoli, which were arranged in a palisade shape (red arrow) (40×). (C) Epithelioid tumor cells stained positive for CD-31 (100×). (D) Spindled schwannian cells were diffusely positive for SOX-10 (200×).
Figure 3
Figure 3
Two months postsurgery, the patient was followed up by chest CT and had no evidence of tumor recurrence.
Figure 4
Figure 4
Timeline of symptoms, investigation, and treatment.

References

    1. Trassard M, Le DV, Bui BN, Coindre JM. Angiosarcoma arising in a solitary schwannoma (neurilemoma) of the sciatic nerve. Am J Surg Pathol. (1996) 20:1412–7. 10.1097/00000478-199611000-00014 - DOI - PubMed
    1. Demiröz SM, Findik G, Aydogdu K, Hekimoglu B, Incekara F, Demirag F, et al. . Mediastinal epithelioid angiosarcoma arising in schwannoma: the first case in the literature. Turk Gogus Kalp Damar Cerrahisi Derg. (2018) 26:305–8. 10.5606/tgkdc.dergisi.2018.14795 - DOI - PMC - PubMed
    1. Mentzel T, Katenkampl D. Intraneural angiosarcoma and angiosarcoma arising in benign and malignant peripheral nerve sheath tumours: clinicopathological and immunohistochemical analysis of four cases. Histopathology. (1999) 35:114–20. 10.1046/j.1365-2559.1999.00714.x - DOI - PubMed
    1. McMenamin ME, Fletcher CD. Expanding the spectrum of malignant change in schwannomas: epithelioid malignant change, epithelioid malignant peripheral nerve sheath tumor, and epithelioid angiosarcoma: a study of 17 cases. Am J Surg Pathol. (2001) 25:13–25. 10.1097/00000478-200101000-00002 - DOI - PubMed
    1. Ito T, Tsutsumi T, Ohno K, Kitamura K. Intracranial angiosarcoma arising from a schwannoma. J Laryngol Otol. (2007) 121:68–71. 10.1017/S0022215106003070 - DOI - PubMed

Publication types