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| CASE REPORT |
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| Year : 2016 | Volume
: 4
| Issue : 1 | Page : 93-95 |
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De novo angiosarcoma of the pleura in a young male
Pallavi Periwal, Arjun Khanna, Rajat Saxena, Deepak Talwar
Metro Center for Respiratory Diseases, Metro Multispeciality Hospital, Noida, Uttar Pradesh, India
| Date of Web Publication | 2-Jun-2016 |
Correspondence Address:
Arjun Khanna
Metro Center for Respiratory Diseases, Metro Multispeciality Hospital, Sector 11, Noida, Uttar Pradesh
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/2321-4848.183370
Angiosarcomas are highly aggressive vascular tumors, which usually take origin from the endothelial cells of small blood vessels. They are extremely rare in the pleura and other serous surfaces such as the peritoneum and pericardium. These tumors are usually well localized, but may sometimes spread diffusely over the pleura and mimic mesotheliomas. Traditional histopathology alone, may not establish the diagnosis with certainty, and immunohistochemistry is essential to clinch the diagnosis. Outcome is usually grave and no formal guidelines exist for the exact therapy. We report this condition in a 22-year-old man who presented to us with a localized fluorodeoxyglucose-positron emissions negative pleural tumor, primarily suspected to be a solitary fibrous tumor, which was eventually diagnosed to be a primary pleural angiosarcoma. Keywords: De novo pleural angiosarcoma, pleural angiosarcoma, pleural tumor
How to cite this article:
Periwal P, Khanna A, Saxena R, Talwar D. De novo angiosarcoma of the pleura in a young male. Arch Med Health Sci 2016;4:93-5 |
| Introduction | |  |
Angiosarcomas account for less than 1% of all the soft tissue sarcomas.[1] It is an aggressive malignant tumor of the vascular endothelium. Primary pleural angiosarcoma is a rare form of this disease. Many of these tumors are highly mitotic and are therefore, who presented to us with a localized fluorodeoxyglucose-positron emissions negative pleural tumor (FDG-PET) positive.[2] The diagnosis is confirmed by combining histopathology with appropriate immunohistochemistry (IHC). The optimum treatment modality for these rare tumors has not yet been crystallized. Here, we describe a unique case where this disease entity was encountered in a young male where the diagnosis was uncertain after extensive radiological work up as the patient had a negative FDG-PET scan.
| Case Report | | |
A 22-year-old nonsmoker, previously healthy male was admitted with complaints of left sided chest pain since two and half months. He had no fever, cough, breathlessness or weight loss. His past medical history was insignificant. General clinical examination was normal. Examination of the respiratory system showed reduced air entry over the left lower hemithorax. Chest radiograph revealed a homogenous pleural-based opacity with an incomplete border sign on the left side. A contrast enhanced computed tomographic scan showed a lobulated mass lesion of size 3.4 cm × 5.6 cm with contrast enhancement and associated minimal pleural effusion [Figure 1]. There was associated scalloping of the sixth rib. FDG-PET scan showed no significant FDG uptake. His routine laboratory investigations were all within normal range. A video-assisted thoracoscopic excisional biopsy of the tumor was done, which revealed a single, fleshy tumor mass arising from the pleura [Figure 2]. The tumor mass was excised in toto and the tumor bed was cauterized. The excised tumor mass was subjected to histopathological examination, which revealed a neoplasm consisting of spindle cells and epithelioid cells in some areas and numerous blood vessels being formed by the tumor. In the epithelioid part of the tumor, intracytoplasmic red cells were seen. No significant mitotic figures or area of necrosis was seen. Immunohistochemical staining of the tumor tissue was positive for the anti-vimentin. CD 31 was focally positive and CD 34 was strongly positive suggesting the diagnosis of angiosarcoma (vascular markers) [Figure 3]. CD 99 and bcl-2 were negative ruling out the possibility of a solitary fibrous tumor. Staining with the anti-cytokeratin antibodies was negative, which excludes the diagnosis of a metastatic carcinoma or malignant mesothelioma. Final diagnosis of a low grade de novo pleural angiosarcoma was made. The patient is being followed up closely. | Figure 2: Video-assisted thoracoscopic surgery image, showing the plural tumor
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 | Figure 3: Histopathology and immunohistochemistry panel, establishing the diagnosis of angiosarcoma (CD 34 and 31 positivity)
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| Discussion | | |
De novo angiosarcoma of the pleura is an extremely rare malignancy. Most of the reported cases have been males and the mean age was around 57 years.[3] The genetic bases and the exact etiopathogenesis of these tumors are not known. Prior diseases, which have been implicated as risk factors for this condition include tuberculous pyothorax, therapeutic and accidental radiation, various acute and chronic pleural infections, and chronic inflammation secondary to foreign bodies and trauma.[4],[5] Our patient had none of the above-mentioned predisposing conditions.
The described clinical features are nonspecific, and may be encountered in a variety of pleural and pulmonary conditions. Aggressive and advanced tumors may be associated with systemic features and chest pain and hemoptysis. None of the radiological or nuclear modalities can establish the diagnosis of angiosarcomas with certainty. Computed tomography (CT) scans may reveal a lobulated pleural mass with irregular margins variable contrast enhancement. Positron emission tomographic (PET) scan may show diffuse and homogeneous FDG uptake indistinguishable from other pleural malignancies such as malignant mesothelioma.[2] However, in our case the FDG-PET showed no significant uptake.
Clinically and histologically these tumors can mimic mesotheliomas and other tumors with fibrous and epithelioid elements such as hemangioendothelioma and solitary fibrous tumor.
Appropriate IHC is essential to differentiate angiosarcomas from these differentials. Both mesotheliomas and adenocarcinomas stain strongly positive for cytokeratin. Negative cytokeratin and calretinin and positive vimentin reactivity generally rules out the diagnosis of mesothelioma and points toward a vascular tumor. The vascular lineage is then confirmed by testing for CD 31, CD 34 and VIII.[2],[3] Our case was positive for both CD 31 and CD 34.
In most patients, the prognosis of these tumors is poor and most, succumb to their illness, soon after diagnosis. A few reports of localized angiosarcomas with somewhat better prognosis, has been described in the literature.[6] The exact role of chemotherapy, radiotherapy and chemical ablation of these tumors has not been described yet, and no formal guidelines for management exist. Our patient had a solitary lesion with insignificant mitosis on histopathological examination, and the tumor bed was cauterized thoroughly at surgery. The patient continues to be under the supervision and repeat CT scan, a year after surgery is not suggestive of recurrence.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Patel AM, Ryu JH. Angiosarcoma in the lung. Chest 1993;103:1531-5.  |
| 2. | Del Frate C, Mortele K, Zanardi R, Hunsaker AR, Nikpoor N, Cibas ES, et al. Pseudomesotheliomatous angiosarcoma of the chest wall and pleura. J Thorac Imaging 2003;18:200-3. |
| 3. | Roh MS, Seo JY, Hong SH. Epithelioid angiosarcoma of the pleura: A case report. J Korean Med Sci 2001;16:792-5. |
| 4. | Maziak DE, Shamji FM, Peterson R, Perkins DG. Angiosarcoma of the chest wall. Ann Thorac Surg 1999;67:839-41. |
| 5. | Aozasa K, Naka N, Tomita Y, Ohsawa M, Kanno H, Uchida A, et al. Angiosarcoma developing from chronic pyothorax. Mod Pathol 1994;7:906-11. |
| 6. | Alexiou C, Clelland CA, Robinson D, Morgan WE. Primary angiosarcomas of the chest wall and pleura. Eur J Cardiothorac Surg 1998;14:523-6. |
[Figure 1], [Figure 2], [Figure 3]
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