British Journal of Radiology (2004) 77, 438-440
© 2004 British Institute of Radiology
doi: 10.1259/bjr/44163342
Concomitant pulmonary and mediastinal sclerosing haemangiomas
Y-Y Lu, MD1,
S-H Ng, MD1,
Y-C Cheung, MD1,
K-W Lui, MD1,
S-C Chan, MD1 and
H-C Lin, MD2
Departments of 1 Diagnostic Radiology and 2 Thoracic Medicine II, Chang Gung Memorial Hospital, 5 Fu-Hsing Street, Kwei Shan, Tao Yuan Hsien, Taiwan
Correspondence: Dr Yun-Chung Cheung
 |
Abstract
|
|---|
We report a case of thoracic sclerosing haemangioma with concomitant pulmonary and mediastinal involvement in a 19-year-old girl who presented with haemoptysis and dyspnoea. CT showed a large oval mass in the left lower lobe and another larger dumbbell-shaped mass in the posterior mediastinum. Both masses were well-defined and harboured punctate calcifications. They exhibited inhomogeneous contrast enhancement and contained some cystic areas. In addition, an air meniscus sign was present around the pulmonary lesion.
|
Introduction
|
|---|
Sclerosing haemangioma, also known as sclerosing pneumocytoma, is a rare benign tumour of the lung that occurs predominantly in middle-aged women. Radiologically, sclerosing haemangioma has a characteristic picture of a benign pulmonary mass. It appears as a well-defined round or oval mass on chest radiograph or a well-defined mass with good contrast enhancement on CT [1–3]. We report a case of sclerosing haemangiomas concomitantly involving both the left lower lobe and posterior mediastinum. Reviewing the literature, sclerosing haemangiomas may have unusual presentations, such as multiple nodules in a single pulmonary lobe [4, 5], an intrafissural mass [1, 4] or a mediastinal mass [4]. However, sclerosing haemangioma with concomitant pulmonary and mediastinal involvement has not been previously reported.
|
Case report
|
|---|
A 19-year-old girl had suffered from haemoptysis and had been found to have lung and mediastinal masses 1 year earlier prior to referral to our hospital. At our initial referral, she refused surgery, and instead undertook Chinese herb therapy. Subsequently after developing progressive dyspnoea and intermittent fever, she was admitted to our hospital for further evaluation and treatment. Physical examination revealed decreased breath sounds at the left lung base. Mild fever was noted at the time of admission and subsided a few days after antibiotic treatment. Bronchoscopic examination revealed external compression on the carina, partial obstruction of the right main bronchus and bleeding from the left lower lobe bronchus. The chest radiograph demonstrated a lung and a mediastinal mass. Chest CT showed a large, oval, well-defined mass of about 6 cm in its largest diameter in the left lower lobe and another larger, encapsulated mass, about 11 cm in its largest diameter, in the posterior mediastinum. Hilar and azygoesophageal recess extensions were evident. Both masses contained small cystic areas and tiny calcifications. They also exhibited inhomogeneous contrast enhancement after intravenous contrast administration (Figures 1 and 2
). In addition, subpleural consolidation, the "air meniscus" sign and reticular infiltration were also seen around the left lower lobe mass (Figure 3). CT-guided core needle biopsy of the left lower lobe mass was then performed suggesting a diagnosis of sclerosing haemangioma.
View larger version (90K):
[in this window]
[in a new window]
|
Figure 1. Enhanced chest CT shows a 6 cm well-defined inhomogeneous enhanced mass in the left lower lobe. Granular calcifications within the tumour and subpleural consolidation were also observed.
|
|
View larger version (90K):
[in this window]
[in a new window]
|
Figure 2. Enhanced chest CT shows a large dumbbell-shaped, encapsulated mass in the posterior mediastinum. The enhancement was inhomogeneous, similar to the left lower lung mass, with multiple cystic areas. Hilar and azygoesophageal recess extensions were evident. Calcification was also present.
|
|
View larger version (95K):
[in this window]
[in a new window]
|
Figure 3. Chest CT on lung window shows air meniscus sign (black arrow), reticular infiltration and subpleural consolidation around the sclerosing haemangioma in left lower lung.
|
|
Subsequently, she underwent left lower lobectomy and resection of the posterior mediastinal mass. Pathological diagnoses of both the pulmonary and mediastinal masses were of sclerosing haemangiomas. Microscopy showed the masses to be composed of various amounts of sclerotic, solid, papillary and haemangiomatous components with focal haemorrhagic areas. The patient remained well after 4 years follow-up.
|
Discussion
|
|---|
Pulmonary sclerosing haemangioma was first reported by Leibow and Hubbell in 1956 and is a rare, benign pulmonary lesion with a female predominance [2]. Sclerosing haemangioma has four major histological components: solid, papillary, sclerotic, and haemangiomatous in varying proportions.
Sclerosing haemangiomas were initially considered to be hamartomas [6, 7] or true neoplasms [8]. Ultrastructural and molecular studies suggested that sclerosing haemangiomas were neoplasms from the respiratory epithelium [9] and both the surface and round cells in sclerosing haemangiomas were neoplastic [10]. Sclerosing haemangioma was consequently removed from the category of tumour-like lesions to that of miscellaneous tumours in the 1999 classification of the World Health Organization/International Association for the Study of Lung Cancer [11]. In a recent immunohistochemical study of 100 cases of pulmonary sclerosing haemangioma, the expression of both thyroid transcription factor-1 (TTF-1) and epithelial membrane antigen (EMA) by the round cells but lack of pancytokeratin, surfactant and Clara cell markers indicate that sclerosing haemangioma originated from primitive undifferentiated respiratory epithelium [4]. Although sclerosing haemangioma is thought to be benign, a few reports of sclerosing haemangioma with solitary or multiple lymph node metastases have been published [12–14].
Radiologically, sclerosing haemangiomas have the characteristic features of solid masses with smooth marginated outline. Contrast enhancement is usually good and can be inhomogeneous or homogeneous, depending on the various amounts of different constituents. Due to the potent bleeding tendency of the haemangiomatous component, focal cystic spaces secondary to intratumoural haemorrhage are often present in the tumour. Calcification occasionally occurs with a reported incidence of 41% [15]. The air meniscus sign or air-trapping zone around sclerosing haemangioma has been reported to be a specific CT sign for sclerosing haemangioma. The postulated mechanism includes proliferation and hyalinization of undifferentiated alveolar mesenchymal cells, contraction of the capsule and tumour, and clearance of peritumoural haemorrhage through the airway [16, 17]. This sign may be helpful to differentiate sclerosing haemangioma from other benign tumours, but is seldom observed.
Sclerosing haemangioma typically presents as a solitary intrapulmonary mass, but unusual presentations have been reported [1, 4]. Cases of multiple sclerosing haemangiomas in a single pulmonary lobe have been described [4, 14]. Sclerosing haemangioma can also present as a solitary mass in a pulmonary fissure or mediastinum. Two cases of the eight sclerosing haemangiomas reported by Im [1], and six cases of the 100 sclerosing haemangiomas reported by Devouassoux-Shisheboran [4] presented as solitary intrafissural tumours. One of the 100 sclerosing haemangiomas reported by Devouassoux-Shisheboran appeared as a solitary mediastinal mass [4]. Although sclerosing haemangioma in the mediastinum is extremely rare, it may possibly occur due to the presence of primitive undifferentiated respiratory epithelium in the mediastinum during embryogenesis.
The mediastinal sclerosing haemangioma in our case, could be either a primary or a tumour metastasis from the lung lesions.
As the two masses were discovered simultaneously, we suspect that these may have been synchronous primary lesions but this cannot be confirmed. In either case the appropriate management was surgical resection with a view to cure.
Recognition of the CT features of this rare benign tumour can give important clues to the diagnosis and assist in planning definitive treatment.
Received for publication February 20, 2003.
Revision received June 16, 2003.
Accepted for publication August 20, 2003.
|
References
|
|---|
- Im JG, Kim WH, Han MC, Han YM, Chung JW, Ahn JM, et al. Sclerosing hemangiomas of the lung and interlobar fissures: CT findings. J Comput Assist Tomogr 1994;18:34–8.[Medline]
- Liebow AA, Hubbell DS. Sclerosing hemangioma (histiocytoma) of the lung. Cancer 1956;9:53–75.[CrossRef][Medline]
- Chan KW, Gibbs AR, Lo WS, Newman GR. Benign sclerosing pneumocytoma of lung (sclerosing hemangioma). Thorax 1982;37:404–12.[Medline]
- Devouassoux-Shisheboran M, Hayashi T, Linnoila RI, Koss MN, Travis WD. A clinicopathologic study of 100 cases of pulmonary sclerosing hemangioma with immunohistochemical studies: TTF-1 is expressed in both round and surface cells, suggesting an origin from primitive respiratory epithelium. Am J Surg Pathol 2000;24:906–16.[CrossRef][Medline]
- Papla B. Sclerosing hemangioma of the lung—benign sclerosing pneumocytoma. Pol J Pathol 1999;50:99–106.
- Williams LJ Jr, Greenberg SD, Mace ML Jr. Pulmonary sclerosing hemangioma: sometimes a form of pulmonary hamartoma. South Med J 1982;75:665–70.[Medline]
- Spencer H, Nambu S. Sclerosing hemangiomas of the lung. Histopathology 1986;10:477–87.[Medline]
- Katzenstein AL, Gmelich JT, Carrington CB. Sclerosing hemangioma of the lung: a clinicopathologic study of 51 cases. Am J Surg Pathol 1980;4:343–56.[Medline]
- Nagata N, Dairaku M, Sueishi K, Tanaka K. Sclerosing hemangioma of the lung. An epithelial tumor composed of immunohistochemically heterogenous cells. Am J Clin Pathol 1987;88:552–9.[Medline]
- Niho S, Suzuki K, Yokose T, Kodama T, Nishiwaki Y, Esumi H. Monoclonality of both pale cells and cuboidal cells of sclerosing hemangioma. Am J Pathol 1998;152:1065–9.[Abstract]
- Travis WD, Colby TV, Corrin B, et al. Histological typing of lung and pleural tumors. Berlin: Springer, 1999.
- Yano M, Yamakawa Y, Kiriyama M, Hara M, Murase M. Sclerosing hemangioma with metastases to multiple nodal stations. Ann Thorac Surg 2002;73:981–3.[Abstract/Free Full Text]
- Tanaka I, Inoue M, Matsui Y, et al. A case of pneumocytoma (so-called sclerosing hemangioma) with lymph node metastasis. Jpn J Clin Oncol 1986;16:77–86.[Abstract/Free Full Text]
- Dawson WB, Muller NL, Miller RR. Pulmonary sclerosing hemangioma: unusual cause of a solitary pulmonary nodule. Can Assoc Radiol J 1990;41:372–4.[Medline]
- Katzenstein A-LA, Gmelich JT, Carrington CB. Sclerosing hemangioma of the lung: a clinicopathologic study of 51 cases. Am J Surg Pathol 1980;4:343–56.
- Nam JE, Ryu YH, Cho SH, Lee YJ, Kim HJ, Lee DY, et al. Air-trapping zone surrounding sclerosing hemangioma of the lung. J Comput Assist Tomogr 2002;26:358–61.[CrossRef][Medline]
- Bahk YH, Shinn KS, Choi BS. The air meniscus sign in sclerosing hemangioma of the lung. Radiology 1978;128:27–9.[Abstract]
Top
Abstract
Introduction
