Case Report
 
A rare case of primary pulmonary angiosarcoma
Joel Alcid1, Wayne Pinover2
1Resident, Internal Medicine, Drexel, Philadelphia, PA, USA
2Attending, Radiation Oncology, Abington-Jefferson Health, Abington, PA, USA

Article ID: 100047Z10JA2018
doi: 10.5348/Z10-2018-47-CR

Corresponding Author:
Joel Alcid,
Hahnemann University Hospital / Drexel Medicine,
239 N Broad St, Philadelphia PA,
USA

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How to cite this article
Alcid J, Pinover W. A rare case of primary pulmonary angiosarcoma. J Case Rep Images Oncology 2018;4:100047Z10JA2018.


ABSTRACT
Introduction: Angiosarcoma is a malignant epithelial cell neoplasm of vascular or lymphatic origin. The key features of behavior of these tumors include insidious growth, extensive local invasion and hematogenous metastasis. Involvement of the lung usually indicates metastatic disease. Very few cases of primary pulmonary angiosarcoma have been reported. We present a case of a 77-year-old female with primary pulmonary angiosarcoma.
Case Report: A 77-year-old female, with a 30-pack year smoking history and current half pack per day smoker, presented to the emergency department with progressive shortness of breath and hemoptysis. Patient had seen her primary care provider one week prior to arrival and was advised to have a computed tomography scan of the chest, which showed 8x4.6x10.4 cm cystic/necrotic septated mass in the left posterior mediastinum. She was started on apixaban for this questionable tumor extension versus bland clot. Hemoptysis had become significantly heavier since starting apixaban, so she came to the emergency department for further evaluation. A fine needle aspiration (FNA) with endobronchial ultrasound (EBUS) was performed, histological examination of the biopsy specimen showed a high grade spindled and epithelioid malignant cells with associated blood vessels and extensive necrosis. Immunohistochemical staining of the tumor revealed it was strongly positive for CD31, vimentin, and cytokeratin, suggestive of epithelioid angiosarcoma. After five doses of radiation, patient was discharged to short term rehab facility. Based on the progression and aggressiveness of the tumor, the patient decided to go on hospice. Shortly after, the patient died from respiratory failure.
Conclusion: Primary pulmonary angiosarcoma is a rare malignant vascular tumor which usually represents metastasis from another primary site. Only a few cases of primary pulmonary angiosarcoma have been reported in literature. Prognosis is poor, with mortality within the first few months of presentation approaching 100%.

Keywords: Oncology, Primary, Pulmonary angiosarcoma, Radiation



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Author Contributions
Joel Alcid – Substantial contributions to conception and design, Acquisition of data, Drafting the article, Revising it critically for important intellectual content, Final approval of version to be published
Wayne Pinover – Substantial contributions to conception and design, Acquisition of data, Drafting the article, Revising it critically for important intellectual content, Final approval of version to be published
Guarantor of Submission
The corresponding author is the guarantor of submission.
Source of Support
None
Conflict of Interest
Authors declare no conflict of interest.
Copyright
© 2018 Joel Alcid et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.