Baylor University Medical Center Proceedings July 2017 - 313

proton density and T1 and T2 relaxation properties of the mass
(7). This technique has been well validated in diﬀerentiating a
thrombus from a tumor compared to reference standards of both
pathology and clinical outcomes (8). The T1- and T2-weighted
signal characteristics of a thrombus vary depending on its age.
Chronic organized thrombus becomes water depleted and cellular debris containing methemoglobin is replaced by ﬁbrous
tissue. This greater ﬁbrin content leads to a lower signal intensity on both T1- and T2-weighted images. Contrast-enhanced
CMRI allows diﬀerentiation between thrombus and surrounding myocardium because thrombus is avascular and hence is
characterized by an absence of contrast uptake (9).
Angiosarcomas present macroscopically as large grossly
hemorrhagic inﬁltrative masses with areas of necrosis. These
attributes are reﬂected on CMRI, which typically shows a large
mass with heterogeneous appearance on T1-weighted images
with areas of intermediate, low, and high signal intensity reﬂecting tumor tissue, necrosis, and the presence of methemoglobin.
These tumors have a heterogeneous, predominantly hyperintense appearance on T2-weighted images and a heterogeneous
enhancement pattern after administration of gadolinium contrast material with marked surface enhancement; a sun ray
appearance has also been described (10, 11).
Surgical resection is the mainstay of treatment. When appropriate, surgical intervention should not be delayed, as the tumor
is aggressive and fast growing and there have been suggestions of
improved patient outcomes from earlier diagnosis and resection
(12). The value of radiation therapy and/or chemotherapy is still
equivocal (13, 14). Despite surgical removal of the tumor, prognosis is very poor. In the case series by Srivali et al, eight of the
nine patients studied were diagnosed while alive (5). Of the eight,
ﬁve died within a mean duration of 2.1 years following diagnosis.
Two of the remaining three had recurrences and metastases at 23
and 27 months following diagnosis. Only one remained alive and
disease-free. Another report of three cases of pulmonary artery
sarcoma by Zhu et al recorded deaths of these patients within an
18-month period following surgical resection (15).

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6.

7.
8.

10.

11.

12.

13.
14.

15.

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Spindle-cell sarcoma involving the major pulmonary arteries

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