
==== Front
Radiol Case Rep
Radiol Case Rep
Radiology Case Reports
1930-0433
Elsevier

S1930-0433(24)00803-3
10.1016/j.radcr.2024.08.023
Case Report
Bilateral pneumothorax unveiling cystic pulmonary metastases from facial angiosarcoma: A case report
Orgi Anas moussaouisalma@gmail.com
⁎
Moussaoui Salma
Merzem Aicha
Belgadir Hasna
Amriss Omar
Moussali Nadia
Benna Naima El
Radiology Department, University Hospital Center Ibn Rochd, Casablanca 20100, Morocco
⁎ Corresponding author. moussaouisalma@gmail.com
03 9 2024
11 2024
03 9 2024
19 11 54715473
19 5 2024
4 8 2024
5 8 2024
© 2024 The Authors. Published by Elsevier Inc. on behalf of University of Washington.
2024

https://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Cutaneous angiosarcoma is a rare malignant vascular tumor that most often affects the scalp and face of elderly males. It is most frequently associated with lung metastases, with the appearance of thin-walled cyst. We report the case of a 78-year-old male who presented with acute chest pain and dyspnea, subsequently revealing bilateral pneumothorax caused by cystic pulmonary metastases from a neglected facial angiosarcoma. The diagnosis was confirmed through comprehensive radiological evaluations, emphasizing the critical role of CT imaging. This case underscores the critical need to not underestimate pneumothorax complicating cystic lung lesions and to consider angiosarcoma metastases, particularly in elderly men, as a potential diagnosis.

Keywords

Pneumothorax
Cystic pulmonary metastases
Angiosarcoma
Case report
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pmcIntroduction

Angiosarcoma is a rare malignant tumor originating from endothelial cells, accounting for less than 1% of adult soft tissue sarcomas. It frequently arises in the scalp and facial regions of elderly patients and is known for its aggressive nature and tendency to metastasize to the lungs, liver, bone, and lymph nodes. Pulmonary metastases can present as solid nodules, thin-walled cysts, or cavitary lesions, which can lead to complications such as spontaneous pneumothorax [1,2].

Case report

A 78-year-old male presented to the emergency department with sudden onset of acute chest pain and dyspnea lasting 2 days. The patient had a history of neglected, progressively enlarging facial lesion over the preceding 8 weeks. Physical examination revealed a 6 × 5 cm nontender, violet, dome-shaped lesion on the right mandibular region (figure not available). Breath sounds were diminished bilaterally, with scattered crackles noted in the lower zones.

Faced with this clinical picture, a high-resolution CT scan was performed revealing multiple cystic lesions, patchy ground-glass opacities, and nodules of varying sizes, indicative of metastatic involvement. Bilateral pneumothorax and pleural effusion was also observed (Fig. 1, Fig. 2).• Cystic Lesions: Multiple thin-walled cysts were distributed throughout both lungs.

• Ground-Glass Opacities: Patchy areas consistent with inflammatory or hemorrhagic changes surrounding the cysts.

• Nodular Lesions: Scattered nodules of varying sizes were present, likely representing different stages of metastatic progression.

• Pneumothorax: Bilateral pneumothorax was evident, a direct result of the rupture of subpleural metastatic cysts.

Fig. 1 (A-D) : Chest computed tomography with lung parenchyma window, showing multiple cystic lesions bilaterally (white star), patchy areas of ground glass opacities (black arrow) and nodules (black arrowhead) with bilateral pneumothorax.

Fig 1

Fig. 2 Chest computed tomography with mediastinal window, showing bilateral pleural effusion, more marked on the right.

Fig 2

The patient underwent drainage of his pneumothorax and was started on broad-spectrum antibiotics. Given the prognosis of his disease, he refused any invasive procedure and died 2 weeks after due to fulminant hemoptysis and pneumothorax.

Discussion

Angiosarcoma is a rare, aggressive malignancy originating from endothelial cells, predominantly affecting the scalp and facial skin in elderly men [1,2]. Pulmonary metastases can present as solid nodules, thin-walled cysts, or cavitary lesions, which may lead to spontaneous pneumothorax [3,4].

CT Imaging plays a central role in identifying and characterizing the extent of pulmonary metastases. In this case, high-resolution CT imaging was crucial in detecting the cystic lesions and associated pneumothorax. The rarity and varied presentation of metastatic angiosarcoma can lead to misinterpretation of imaging findings. Radiologists must maintain a high index of suspicion when encountering unexplained cystic lung lesions, especially in elderly males [5,6].

Pathophysiology and mechanism of cyst formation:Necrosis of tumor nodules: One of the primary mechanisms for cyst formation is the necrosis of tumor nodules. As the tumor cells proliferate, they can outgrow their blood supply, leading to central necrosis and subsequent cavitation.

Invasion of pre-existing lung cysts: Tumor cells can invade preexisting lung cysts, causing further cystic transformation and enlargement.

Obstruction of small airways: Tumor cells can obstruct small airways, leading to air trapping and overdistension of the alveoli, resulting in cyst formation. This ball-valve effect can exacerbate the cystic changes and increase the risk of pneumothorax [6].

The management of metastatic angiosarcoma is challenging due to its aggressive nature and poor prognosis. Treatment options typically include surgical excision, radiotherapy, and chemotherapy. In this case, despite the identification and symptomatic management of the bilateral pneumothorax, the patient's condition deteriorated rapidly, leading to respiratory failure and death. This highlights the need for early recognition and aggressive management of metastatic angiosarcoma to improve patient outcomes [2,4].

This case underscores the critical role of radiologists in the early detection and accurate characterization of metastatic angiosarcoma. High-resolution CT scans provide detailed insights into the extent and nature of pulmonary involvement, which are crucial for guiding treatment decisions and managing complications. Radiologists must be vigilant and consider metastatic angiosarcoma in elderly patients presenting with spontaneous pneumothorax and characteristic radiological findings, even in the absence of a known primary tumor [5].

Conclusion

Bilateral spontaneous pneumothorax can be an initial manifestation of metastatic angiosarcoma, often leading to significant respiratory compromise. Advanced imaging techniques, particularly high-resolution CT, are essential for accurate diagnosis and management. This case underscores the importance of considering metastatic angiosarcoma in elderly patients presenting with spontaneous pneumothorax and characteristic radiological findings.

Declaration of generative AI and AI-assisted technologies in the writing process

During the preparation of this work the author used Open AI tools in order to enhance language. After using this Open AI, the author reviewed and edited the content as needed and takes full responsibility for the content of the publication.

Patient consent

We, the authors, by the present declare that we have obtained written informed consent from the patient for the publication of this case report and any accompanying images. The patient has been informed that their information will be de-identified to protect their privacy.

Competing Interests: The authors declare that there are no competing interests. We confirm that the manuscript has been read and approved by all named authors, and there are no other persons who satisfied the criteria for authorship but are not listed. We also confirm that the order of authors listed in the manuscript has been approved by all of us.
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