DOI:10.2214/AJR.05.0827
AJR 2007; 189:W26-W28
© American Roentgen Ray Society
Pulmonary Sclerosing Hemangioma Manifesting as a Nodule with Irregular Air Clefts on High-Resolution CT
Hiroshi Takatani1,
Kazuto Ashizawa2,
Kiyoko Kawai3 and
Shigeru Kohno4
1 Department of Internal Medicine, Nagasaki Municipal Hospital, Nagasaki
852-8555, Japan.
2 Department of Radiology and Radiation Oncology, Nagasaki University Graduate
School of Biomedical Science and Nagasaki University Hospital, 1-7-1 Sakamoto,
Nagasaki 852-8501, Japan.
3 Department of Pathology, Nagasaki Municipal Hospital, Nagasaki, Japan.
4 Department of Internal Medicine, Nagasaki University Hospital, Nagasaki,
Japan.
Received May 16, 2005;
accepted after revision July 12, 2005.
Address correspondence to K. Ashizawa
(ashi{at}net.nagasaki-u.ac.jp).
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Keywords: CT • high-resolution CT • lung disease • lung nodules • pulmonary sclerosing hemangioma
Introduction
Sclerosing hemangioma of the lung is a rare benign neoplasm
[1,
2]. Most affected patients are
asymptomatic, but some present with hemoptysis, cough, chest pain, dyspnea,
and pleurisy. Because there are few findings on radiologic studies that are
characteristic of sclerosing hemangioma, it is difficult to diagnose on the
basis of imaging. In a few case reports, investigators have mentioned the
presence of an air space around a sclerosing hemangioma on chest CT
[3–9].
We report a case of pulmonary sclerosing hemangioma with irregular air spaces
on high-resolution CT (HRCT) that had been growing like a ground cleft for 6
years.
Case Report
A 42-year-old woman with hemoptysis was admitted to our hospital for
further workup and management of a pulmonary mass in July 2003. The pulmonary
mass was first detected on chest radiography when she underwent surgery for
carcinoma of the uterine cervix 6 years earlier. The pulmonary mass had been
followed up on chest radiography and CT for 6 years. She had smoked 10
cigarettes per day for 16 years but was no longer smoking cigarettes. On
admission, physical and laboratory findings were unremarkable. Sputum studies
for acid-fast bacilli, Aspergillus organisms, other pathogenic
organisms, and malignant cells were negative.
The initial chest radiograph in 1997 revealed a solitary pulmonary mass in
the left upper lung field (Fig.
1A). Chest CT performed on the same day showed a well-defined
2.8-cm mass with irregular air spaces in the left upper lobe.
Contrast-enhanced CT was not performed, and CT attenuation value was not
calculated. An emphysematous area and ground-glass opacity (GGO) in the
surrounding lung parenchyma (Fig.
1B) were noted.
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Fig. 1A —42-year-old woman with pulmonary sclerosing hemangioma over
6-year follow-up period. Magnified view of initial chest radiograph in 1997
reveals solitary pulmonary mass in left upper lung field.
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Fig. 1B —42-year-old woman with pulmonary sclerosing hemangioma over
6-year follow-up period. Initial chest CT image in 1997 shows well-defined
mass with irregular air spaces (arrowheads) in left upper lobe.
Emphysematous change (black arrows) and ground-glass opacity (GGO)
(white arrows) are also seen in surrounding lung parenchyma.
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Follow-up studies performed in 2000 because the patient was experiencing
hemoptysis showed that the mass had become slightly larger than it had been at
the initial examination and that the air spaces within the mass had spread
like a ground cleft (Fig. 1C).
HRCT showed that the area of GGO around the mass had expanded. In 2001, a year
after the first follow-up studies, the patient was asymptomatic and HRCT
showed that the area of GGO had contracted.
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Fig. 1C —42-year-old woman with pulmonary sclerosing hemangioma over
6-year follow-up period. High-resolution CT (HRCT) image obtained in 2000 when
patient experienced hemoptysis shows increase in area of GGO (arrows)
around mass.
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On admission in 2003 when the patient was again experiencing hemoptysis,
chest radiography revealed slight enlargement of the pulmonary mass and an
increase in the size of the areas showing emphysematous change and GGO in the
surrounding lung parenchyma. HRCT performed on the same day showed that GGO
extended to the pleural surface and that emphysematous change around the mass
was more prominent (Fig. 1D).
Contrast-enhanced CT was not performed during the follow-up period.
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Fig. 1D —42-year-old woman with pulmonary sclerosing hemangioma over
6-year follow-up period. HRCT image obtained at admission in 2003 when patient
was again experiencing hemoptysis shows air spaces (arrowheads)
within mass have spread like ground cleft, area of GGO (white arrows)
extends to pleural surface, and emphysematous change (black arrows)
around mass is more marked.
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Fiberoptic bronchoscopy and transbronchial biopsy were performed, but
findings for a definitive diagnosis were not obtained. The presumed diagnoses
before surgery were aspergilloma and lung cancer.
The patient underwent partial segmentectomy of the left upper lobe.
Pathologic examination revealed a gray-red solitary tumor, measuring 3.3
x 3.4 cm, that was located in the lung parenchyma. The tumor was well
circumscribed, but it was not encapsulated. A semicircular rim of air space
was noted between the periphery of the tumor and the lung parenchyma
surrounding the tumor.
Microscopically, the histopathologic specimen showed hemorrhagic and solid
patterns with hyalinization. Irregularly enlarged cystic air spaces that were
lined by cuboidal epithelial cells were present in the solid proliferation of
epithelioid tumor cells (Fig.
1E). Hemosiderin-laden macrophages and emphysematous change with
fibrosis were seen in the lung parenchyma surrounding the tumor. These
findings confirmed the diagnosis of sclerosing hemangioma.
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Fig. 1E —42-year-old woman with pulmonary sclerosing hemangioma over
6-year follow-up period. Histopathologic specimen shows hemorrhagic and solid
pattern with hyalinization. Irregularly enlarged cystic air spaces lined by
cuboidal epithelial cells are present in solid proliferation of epithelioid
tumor cells. (H and E, x100)
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Discussion
Pulmonary sclerosing hemangioma is a rare benign tumor. It was first
described by Liebow and Hubbell in 1956
[1]. It occurs predominantly in
young or middle-aged women. Pathologically, it is composed of four major
histologic components—namely, solid, papillary, sclerotic, and
hemangiomatous components. On the basis of ultrastructural and
immunohistochemical findings, it is considered to be a proliferation of type
II pneumocytes [2].
Radiologically, the tumor usually presents as a well-defined homogeneous
mass on chest radiography and CT and frequently manifests as a markedly
contrast-enhancing juxtapleural mass with or without areas of low attenuation
and calcification on contrast-enhanced CT
[3]. Because there are few
characteristic features of pulmonary sclerosing hemangioma on radiologic
studies, it is difficult to diagnose preoperatively. The "air
meniscus" sign, or the presence of an air-trapping zone, is one of the
characteristics of this tumor.
In a few case reports, researchers have described interesting CT findings
[4–6].
In these reports, investigators proposed possible mechanisms of air-space
development. These include, first, proliferation and hyalinization of
undifferentiated alveolar mesenchymal cells that wrap around the bronchus and
lead to distention of the distal air space
[4]; second, contraction of the
capsule and tumor, particularly when the tumor is highly cellular and
hemorrhagic [4]; third,
peritumoral hemorrhage followed by communication with the airway
[5,
7]; and, fourth, peritumoral
hemorrhage and tumor contraction
[8].
The distinguishing characteristics of pulmonary sclerosing hemangioma in
our patient are air spaces within the tumor and emphysematous change and GGO
around the tumor. Over a 6-year follow-up period, the air spaces had slowly
spread like a ground cleft. The area of GGO increased when the patient
experienced hemoptysis and decreased when she was asymptomatic. The size of
the area showing emphysematous change had also gradually increased. Sakamoto
et al. [9] reported that a
"check-valve" effect is an important mechanism in the formation of
peritumoral emphysematous change.
Although we did not recognize the communication between the tumor and
bronchus in our patient, we hypothesize that repeated hemorrhage from the
tumor in surrounding lung parenchyma resulted in the formation of irregular
air spaces. The presence of many hemosiderin-laden macrophages in the air
spaces suggests that peritumoral bleeding had occurred. We also hypothesize
that fibrous change due to peritumoral hemorrhage made a check-valve mechanism
that led to an increase in the size of the area showing emphysematous
change.
The differential diagnosis for the air meniscus sign includes infectious
diseases, such as aspergilloma, tuberculoma, echinococcal cyst, and lung
abscess, and neoplastic diseases, such as lung cancer and hamartoma. A
tuberculous cavity with a Rasmussen aneurysm is also considered. In our
patient, however, the so-called "air meniscus sign" and air spaces
within the tumor were seen on HRCT. Emphysematous change and GGO around the
tumor were also seen. Although these findings are not common, knowledge of
them is useful to correctly diagnose pulmonary sclerosing hemangioma.
Acknowledgments
We thank Keiji Inoue, Kazunori Minami, Yuji Ishimatsu, and Yasumasa Doutsu
for their useful work and discussions, and we thank Rashid Hashmi for his
assistance in the preparation of this manuscript.
References
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Introduction
Case Report
