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Year : 2023  |  Volume : 38  |  Issue : 1  |  Page : 84-86  

Demonstration of multiple metastatic sites by positron emission tomography/computed tomography in a rare case of epithelioid angiosarcoma of the scalp

Department of Nuclear Medicine and Molecular Imaging, Tata Memorial Hospital, Mumbai, Maharashtra, India

Date of Submission27-May-2022
Date of Decision12-Aug-2022
Date of Acceptance22-Sep-2022
Date of Web Publication24-Feb-2023

Correspondence Address:
Dr. Nilendu Purandare
Department of Nuclear Medicine and Molecular Imaging, Tata Memorial Hospital, E. Borges Road, Parel, Mumbai - 400 012, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijnm.ijnm_92_22

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Epithelioid angiosarcoma is a rare subtype of angiosarcoma, with metastases occurring in more than 50% of cases and the lung is the most organ which is involved. Whole-body fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) has demonstrated its clinical utility in the early detection of metastases in angiosarcoma. It is helpful to differentiate between benign lesions with low FDG uptake as compared to malignancies with high FDG avidity. Here, we present a rare case of a young man with epithelioid angiosarcoma, in which FDG PET/CT has demonstrated metastatic sites (especially lung metastases).

Keywords: Epithelioid angiosarcoma, fluorodeoxyglucose positron emission tomography/computed tomography, lung metastases

How to cite this article:
Kale S, Purandare N, Shah S, Puranik A, Agrawal A, Rangarajan V. Demonstration of multiple metastatic sites by positron emission tomography/computed tomography in a rare case of epithelioid angiosarcoma of the scalp. Indian J Nucl Med 2023;38:84-6

How to cite this URL:
Kale S, Purandare N, Shah S, Puranik A, Agrawal A, Rangarajan V. Demonstration of multiple metastatic sites by positron emission tomography/computed tomography in a rare case of epithelioid angiosarcoma of the scalp. Indian J Nucl Med [serial online] 2023 [cited 2023 Mar 31];38:84-6. Available from:

A 24-year-old man presented with multiple painless scalp swellings for the past 4 months. These were smooth and nonmobile. The patient complained of pain over right occipital swelling. No palpable cervical or axillary lymph nodes were found on examination. Magnetic resonance imaging (MRI) brain was performed to assess the swelling and revealed well-defined, encapsulated, iso-intense on T1, and intermediate-to-hyperintense on T2 lesions in the scalp over the right occipital region. Another ring-enhancing lesion with perilesional edema was seen in the left parieto-occipital region [Figure 1]a and [Figure 1]b. The patient underwent fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) for detection of the primary and overall assessment of the disease, which showed [Figure 2]a, [Figure 2]b, [Figure 2]c, [Figure 2]d, [Figure 2]e metabolically active right parieto-occipital scalp lesion, ground-glass lung opacities with low-grade metabolism, and ring enhancing brain lesion in the left occipital lobe. Another FDG avid soft-tissue lesion was noted in the left upper back. Multiple bilateral lung nodules with surrounding ground glassing opacities were noted (CT halo sign), which is a classic finding for hemorrhagic lung metastases. Since the ground-glass opacities raised suspicion of hemorrhagic metastases, a biopsy was performed to confirm the same. The histopathological report turned out to be an epithelioid subtype of angiosarcoma. On immunohistochemical examination, the tumor cells were positive for CD 31, AE1/AE3, and EMA (epithelial membrane antigen) and negative for CD 34, S100P, SMA, and desmin. INI1/SMARCB1 is retained in tumor cells. The patient was started on metronomic chemotherapy.
Figure 1: (a) A well-defined enhancing lesion seen in the scalp in the right occipital region on post contrast T1 FSPGR sequence (arrow). Another enhancing lesion was seen in the left parieto-occipital region of the brain (arrowhead). (b) T2W images show a heterogeneous appearing scalp lesion (arrow). Parieto-occipital lobe brain lesion appears hyperintense with surrounding edema (arrowhead). FSPGR - fast spoiled gradient-echo, T2W: T2 weighted

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Figure 2: MIP (a) (black arrow), fused (b), and axial (c) (arrows) images shows FDG avid scalp lesion in the right occipital region. Axial fused PET/CT images (d) and CT image (e) show bilateral ground glass opacities surrounding the soft-tissue nodule which is classic CT sign (Halo sign) for hemorrhagic lung metastases (arrows). Sagittal fused PET/CT (f) and sagittal CT (g) (arrows) images showed left parieto-occipital brain lesion, PET/CT: Positron emission tomography/computed tomography, FDG: Fluorodeoxyglucose, MIP: Maximum Intensity Projectiion

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   Discussion Top

Angiosarcoma is an aggressive, malignant endothelial cell tumor of lymphatic or vascular origin and represents <1% of all soft-tissue sarcoma.[1] A unique and rare morphologic subtype of angiosarcoma, in which the malignant cells have a predominantly epithelioid appearance is called epithelioid angiosarcoma. It has a male predilection with the highest incidence in the seventh decade of life. These tumors have aggressive nature with more than 50% of cases have <5-year survival.[2] There are reported cases of epithelioid angiosarcoma involving the thyroid,[3] ovary,[4] bone,[5] and deep soft tissue.[6] Metastases occur in more than 50% of patients with epithelioid angiosarcoma and the lung is the primary organ involved. Patients with lung metastases often present with respiratory-associated symptoms such as hemoptysis, cough, and dyspnea.[7]

MRI features of tumor include intermediate T1 signal intensity with possible regions of hyperintensity suggestive of hemorrhage with high T2 signal intensity. The most important finding is the presence of high-flow serpentine vessels (low-signal intensity on both T1- and T2-weighted images) in an otherwise solid nonspecific soft-tissue mass. Low flow vessels, however, may show hyperintensity on T2-weighted images. Angiosarcoma demonstrates enhancement after intravenous contrast administration and may show nonenhancing areas reflecting tumor necrosis. Although these imaging features are relatively nonspecific, they indicate malignancy and should prompt biopsy for further characterization.[8],[9] FDG-PET/CT provides important details that can be used in the diagnosis, staging, restaging, treatment response monitoring, and prognostication of STSs (soft-tissue sarcomas). FDG PET/CT has provided some reliability in the early detection of distant metastases, staging, and its prognostication of angiosarcoma.[10] Benign vascular lesions, such as hemangiomas and hemangioendothelioma, demonstrate significantly lower FDG avidity compared with angiosarcoma, allowing distinguishability.[11]

There are reported cases of the demonstration of metastases in cardiac angiosarcoma by FDG PET.[12] In this case, whole-body PET/CT scan has been helpful to demonstrate hemorrhagic lung metastases and other sites of disease involvement, thus providing the complete assessment of disease in this rare malignancy.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed

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Conflicts of interest

There are no conflicts of interest.

   References Top

Requena C, Sendra E, Llombart B, Sanmartín O, Guillén C, Lavernia J, et al. Cutaneous angiosarcoma: Clinical and pathology study of 16 cases. Actas Dermosifiliogr 2017;108:457-65.  Back to cited text no. 1
Hart J, Mandavilli S. Epithelioid angiosarcoma: A brief diagnostic review and differential diagnosis. Arch Pathol Lab Med 2011;135:268-72.  Back to cited text no. 2
Huang SH, Wu SC. Primary angiosarcoma of the thyroid in an Asian woman: A case report with review of the literature. Case Rep Pathol 2020;2020:9068506.  Back to cited text no. 3
Peng X, Duan Z, Yin H, Dai F, Liu H. Ovarian epithelioid angiosarcoma complicating pregnancy: A case report and review of the literature. J Int Med Res 2021;49:3000605211019641.  Back to cited text no. 4
Lang J, Chen L, Chen B, Chen K, Liu A, Li J, et al. Epithelioid angiosarcoma of the spine: A case report of a rare bone tumor. Oncol Lett 2014;7:2170-4.  Back to cited text no. 5
Fletcher CD, Beham A, Bekir S, Clarke AM, Marley NJ. Epithelioid angiosarcoma of deep soft tissue: A distinctive tumor readily mistaken for an epithelial neoplasm. Am J Surg Pathol 1991;15:915-24.  Back to cited text no. 6
Wang H, Shi J, Liu H, Chen Y, Wang Y, Wang W, et al. Clinical and diagnostic features of angiosarcoma with pulmonary metastases: A retrospective observational study. Medicine (Baltimore) 2017;96:e8033.  Back to cited text no. 7
Walker EA, Salesky JS, Fenton ME, Murphey MD. Magnetic resonance imaging of malignant soft tissue neoplasms in the adult. Radiol Clin North Am 2011;49:1219-34, vi.  Back to cited text no. 8
Watanabe S, Yano F, Kita T, Soga S, Shinmoto H, Kosuda S, et al. 18F-FDG-PET/CT as an indicator for resection of pulmonary epithelioid hemangioendothelioma. Ann Nucl Med 2008;22:521-4.  Back to cited text no. 9
Tokmak E, Ozkan E, Yağcı S, Kır KM. F18-FDG PET/CT Scanning in angiosarcoma: Report of two cases. Mol Imaging Radionucl Ther 2011;20:63-6.  Back to cited text no. 10
Lee WW, So Y, Kang SY, So MK, Kim H, Chung HW, et al. F-18 fluorodeoxyglucose positron emission tomography for differential diagnosis and prognosis prediction of vascular tumors. Oncol Lett 2017;14:665-72.  Back to cited text no. 11
Dhull VS, Sharma P, Mukherjee A, Jana M, Bal C, Kumar R. 18F-FDG PET-CT for evaluation of cardiac angiosarcoma: A case report and review of literature. Mol Imaging Radionucl Ther 2015;24:32-6.  Back to cited text no. 12


  [Figure 1], [Figure 2]


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