primary pleural angiosarcoma a case report with literature
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Dr. T.S. WONG, Dr. F. HIOE
Department of Clinical Pathology, Pamela Youde Nethersole Eastern Hospital, Hong Kong
Primary pleural angiosarcoma –A case report with literature review
Clinical history
The patient was a 68-year-old Chinese male who presented with blood-stained sputum, dyspnea, and
pleuritic chest pain. Chest X-ray showed significant left pleural effusion. Computer tomography (CT)
scan showed pleural effusion with smooth pleural thickening (Figure 1). Thoracoscopy showed diffuse
hemorrhagic surface with irregular growth. Repeated fine needle aspiration of pleural effusion showed
negative results. Pleural biopsies were taken and only scanty materials were sampled. Serial CT scans
showed similar pleural thickening and new vertebral and splenic lesions (Figure 2). Clinical diagnosis
was malignant mesothelioma with disseminated disease. He soon passed away and was referred to
coroner for possibility of occupational lung disease.
Introduction
Angiosarcoma is a rare malignant neoplasm of endothelial origin. It accounts for around 1% of all soft
tissue malignancy and commonly involves skin, soft tissue, breast, bone and viscera [1]. Primary pleural
angiosarcoma is an exceedingly rare malignant mesenchymal tumor of pleura. It was first described in
1943 [3]. As of 2010, there are less than 50 reported cases documented in literature [2]. We report a case
of primary pleural angiosarcoma of left pleura in a 68-year-old Chinese male, together with a brief
literature review on this entity.
Microscopic examination
Tumor growth along the left pleural surface showed atypical spindle cells with enlarged hyperchromatic
nuclei. Rudimentary vascular channels were seen in the tumor. These vascular channels were lined by
atypical cells (Figure 6). Atypical epithelioid cells forming sheets were also identified. These epithelioid
cells possessed abundant eosinophilic cytoplasm with enlarged oval nuclei and distinct nucleoli.
Cytoplasmic vacuoles containing red cells were focally seen (Figure 7). Both atypical spindle cells and
epithelioid cells were positive for CD31 and ERG (Figure 8). They also show cytoplasmic staining for
WT1 (Figure 8). The epithelioid cells were also positive for AE1/AE3 (Figure 8). They were all negative
for calretinin and CK5/6 (Figure 8). Features were consistent with angiosarcoma. Tumor showed
infiltration into lung parenchyma and intercostal soft tissue (Figure 9-10). There was no evidence of
asbestosis on extensive sampling.
Microscopic examination also confirmed tumor deposits in spleen and vertebra (Figure 11-12).Conclusion
Primary pleural angiosarcoma is an extremely rare and aggressive disease. Patients usually have a
dismal prognosis. Moreover, its clinical, radiological and histological features can mimic malignant
mesothelioma and the use of immunohistochemical studies is necessary for a correct diagnosis.
Discussion
Primary pleural angiosarcoma is an extremely rare malignant mesenchymal tumor of pleura. It was
first described in 1943 [3]. As of 2010, less than 50 cases of primary pleural angiosarcoma were
reported in literature [1,11]. The mean age of diagnosis is 58 years (22 - 80 years) with a male
predominant ratio of 6:1 [2, 4, 5].
Due to rarity of the tumor, definite pathogenesis and etiology are not yet defined. However, several risk
factors are reported, including exposure to asbestos, tuberculosis, chronic pyothorax, radiation therapy
or parasitic infestation [6-12]. In small case series of five cases, it was postulated that patients with
history of chronic pyothorax had 3600-fold increased risk of developing pleural angiosarcoma [11].
Clinical presentation includes chest pain, shortness of breath, hemoptysis, coughing and constitutional
symptoms [1]. On imagining studies, pleural effusion, usually bloody and pleural thickening are always
encountered [1, 2, 5]. These simulate the clinical presentation and radiological finding of malignant
mesothelioma. Diagnosis is usually made on decortication or resection specimens and examination of
cytology or small biopsy specimens usually do not give a definitive diagnosis [1, 13]. It is advised that in
cases of refractory bloody pleural effusion of uncertain etiology and pleural thickening, surgical biopsy
including debulking excision, pleurectomy and decortication should be performed for definitive
diagnosis [13]. In patients with late-stage disease, the disease is also diagnosed in autopsy setting [1].
Histological features of primary pleural angiosarcoma include malignant cells with epithelioid or
spindle appearance with vasoformative nature. Occasionally, cytoplasmic vacuoles containing red cells
can be seen. Common differential diagnoses include mesothelioma and sarcomatoid carcinoma.
Immunohistochemical study is needed to distinguish the lineage of malignant cells. Expression of
endothelial markers such as CD31, CD34, factor VIII, FLI-1 or ERG is required [1, 13]. Sarcomatoid
carcinoma and mesothelioma are usually negative for these markers. Positive cytokeratin staining
(CAM5.2 and CK7) was reported in a proportion of primary pleural angiosarcoma, approximately 60%
of cases, usually in the areas with epithelioid appearance [1, 13]. Mesothelioma and sarcomatoid
carcinoma can also show positivity to cytokeratin, especially pan-cytokeratin. They will be less useful in
confirming the diagnosis. Markers of mesothelial cells such as calretinin, CK5/6, D2-40 and WT1 is
useful to differentiating tumor of vascular origin from mesothelioma. Interestingly, nuclear expression
of WT1 is seen in mesothelial cells while strong cytoplasmic expression, as in our case, is reported to be
found in tumor of endothelial lineage [2].
Treatment modalities include surgery, radiotherapy and chemotherapy. Surgery can be useful for
localized tumor. Vascular embolization is reported to be helpful in shrinking the tumor for subsequent
surgical resection. Radiotherapy can be used in adjuvant setting [1].
Clinical course of primary pleural angiosarcoma is usually rapidly fatal. The mean survival is around 1
year [1]. Most patients died of the disease within 7 months [13]. Sites of metastasis include regional
lymph node, adrenal gland, bone, brain, oral cavity, liver, skin, spleen, gastrointestinal tract, kidney and
spinal cord [13].
Gross examination
Reference
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Figure 3.
Figure 1. Figure 2.
Figure 4. Figure 5.
Autopsy was performed. On gross examination,
there were bilateral blood-stained pleural effusion.
Diffuse tumor growth along the left pleural surface
encasing the left upper lobe was identified while the
left lower lobe was collapsed (Figure 3). Tumor
growth showed invasion into intercostal spaces.
Congestion and focal consolidation were identified
in right lung.
Spleen weighed 88 gm and there were multiple
tumor deposits measuring 0.5 cm to 2 cm across the
greatest dimension (Figure 4). There was also
involvement of multiple levels of vertebra (Figure
5). The rest of the internal organs were
unremarkable.
Figure 11.
Figure 7.
Figure 9.
Figure 10.
Figure 6.
Figure 12.
CD31
ERG
WT1
AE1/AE3
Calretinin
CK5/6
Figure 8.
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