Asian Cardiovasc Thorac Ann 2008;16:57-58
© 2008 Asia Publishing EXchange Ltd
Endobronchial Sclerosing Hemangioma: A Rare Presentation of a Parenchymal Tumor
Mohamed-Sadok Boudaya, MD,
Pierre-Emmanuel Falcoz, MD,
Marco Alifano, MD,
Sophie Camilleri-Broet, MD,
Jean-François Régnard, MD
Departments of Thoracic Surgery and Pathology, Hôtel-Dieu Hospital, Paris V University Paris, France
For reprint information contact: Mohamed-Sadok Boudaya, MD, Tel: 33 62 944 7565, Fax: 33 14 234 8885, Email: mohamedsadok.boudaya{at}gmail.com, Department of Thoracic Surgery, Hôtel-Dieu University Hospital, 1, place du Parvis Notre Dame, 75181 Paris, Cedex 04, France.
 |
ABSTRACT
|
|---|
Endobronchial localization of sclerosing hemangioma is extremely rare. Exact localization of lesion at preoperative work-up allows parenchymal-sparing procedures. We report a case of endobronchial sclerosing hemangioma diagnosed preoperatively and treated by lingular-sparing upper lobectomy with nodal dissection.
|
INTRODUCTION
|
|---|
Sclerosing hemangioma is a rare pulmonary neoplasm occurring generally in women in their forties or fifties.1 It almost always involves lung parenchyma though endobronchial location has also been reported.2,3 This topography engenders particular diagnostic and therapeutic approaches.
|
CASE REPORT
|
|---|
A solitary lung nodule was incidentally discovered in a 56-year-old woman during a work-up for sleep apnea syndrome. She was morbidly obese (BMI = 45) and had arterial hypertension.
Computed tomographic (CT) scans revealed a 21 x 16 mm lesion of the ventral segment of the left upper lobe. The nodule was central and was punctuated by several calcifications of about 1–2 mm (Figure 1
). There was no enlarged hilar or mediastinal lymph node. The positron emission tomography scan did not show any abnormal accumulation of tracer.
View larger version (160K):
[in this window]
[in a new window]
|
Figure 1. Chest computed tomographic scan demonstrating a culminal tumor invading the ventral bronchus.
|
|
Bronchial endoscopy showed a smooth and soft lesion in the ventral bronchus of the left upper lobe. Pathologic examination of biopsies was consistent with sclerosing hemangioma. At thoracotomy, a 2.5 cm solid tumor was found inside the upper division of the left upper lobe. This lesion respected the visceral pleura and there were neither enlarged nodes nor other lung lesions. A lingular-sparing upper lobectomy was performed, allowing a security bronchial margin of 1 cm. A complete nodal dissection was also carried out.
On pathology, a proliferation of regular round cells containing eosinophilic small nucleoli and cytoplasm in mainly a solid type arrangement was observed. The lesion involved all the bronchus and was responsible for destruction of bronchial architecture. It infiltrated the adjacent pulmonary tissue and formed some pseudo-glandular structures lined by cubic or flattened epithelium, sometimes with sclerotic pattern. The tumor was unencapsulated and its limits with the surrounding parenchyma were not distinctive. Some collagenous fibrous rearrangements were calcified.
On immunohistochemical studies, the ovoid tumor cells were positive for epithelial membrane antigen (EMA), thyroid transcription factor-1 (TTF-1) and negative for cytokeratins (KL 1). The pseudo-glandular structures were positive for KL 1. Bronchial and parenchymal resection margins were free. Lymph node examination showed no tumoral invasion. Postoperative course was uneventful. No recurrence was observed at 1-year follow-up.
|
DISCUSSION
|
|---|
Pulmonary sclerosing hemangioma is rare, with about 150 cases reported.2 Devouassoux–Shisheboran et al reported a single localization in pulmonary parenchyma in a majority of cases. However, exceptional presentations were observed: multifocal lesions 4%, pleural localization 4%, mediastinal localization 1% or endobronchial localization 1%.2
The presence of sclerosing hemangioma inside the bronchial lumen has been described in only three patients so far. In one patient,2 intra bronchial localization was highlighted in a peripheral segmental bronchus at definitive pathologic examination of resection specimen. In the two other cases, the tumor was seen at fiberoptic bronchoscopy in the left lower bronchus and pathologic diagnosis could be established preoperatively.3
Histogenesis of pulmonary sclerosing hemangioma has been a matter of debate since its first description by Liebow and Hubbell who suggested an endothelial origin and this theory was supported by others.4 More recently, a possible mesothelial origin was advocated.5 However, based on ultra structural and recent immunohistochemical analyses, several authors consider that this tumor originates from a proliferation of epithelial cells.1 In fact, it was demonstrated that the two main cell types forming sclerosing hemangioma (round and surface cells) share similar characteristics with pneumocytes and primitive foetal respiratory epithelium cells. The neoplasm would thus develop from an undifferentiated primitive respiratory epithelium.2,6,7
Presence of sclerosing hemangioma inside a bronchial wall (as in our case) would be in contradiction with this assertion. However, we think that this particular presentation of sclerosing hemangioma may not be a proliferation originating in the bronchus itself but a proliferation from peri-bronchial lung parenchyma invading adjacent bronchus. Alternatively, cells of the undifferentiated primitive respiratory epithelium could be entrapped in the bronchial wall during development and subsequently undergo tumoral transformation.
We report a new case of the endobronchial variant of pulmonary sclerosing hemangioma in the ventral bronchus of the left upper lobe diagnosed by endoscopic biopsies. Clinically, our patient was asymptomatic, whereas Devouassoux-Shisheboran et al3 reported in both patients clinical signs related to an intra bronchial lesion (cough, sputum production, recurring bronchitis or pneumonias). In spite of the endobronchial location and the important size of the lesion in these three cases, hemoptysis was absent. This symptom was not infrequent in patients having peripheral pulmonary sclerosing hemangioma.1–3
Recommended treatment of sclerosing hemangioma is tumor wedge resection with security margin of parenchyma when it is located in periphery.1 In the extremely rare case of intra bronchial localization, anatomical lung resection is mandatory. If diagnosis is made preoperatively, planned surgery can be limited to a sublobar resection, allowing conservation of maximum of pulmonary parenchyma. To our knowledge, ours is the first case where preoperative diagnosis permitted limited anatomical lung resection. Hilar and mediastinal lymph node dissection must be associated with the resection, in fact, disease spread was described when the tumor size exceeds 3.5 cm.8
|
REFERENCES
|
|---|
- Sugio K, Yokoyama H, Kaneko S, Ishida T, Sugimachi K. Sclerosing hemangioma of the lung: radiographic and pathological study. Ann Thorac Surg 1992;53:295–300.[Abstract]
- 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.[Medline]
- Devouassoux-Shisheboran M, de la Fouchardière A, Thivolet-Béjui F, Sourisseau-Millan ML, Guerin JC, Travis WD. Endobronchial variant of sclerosing hemangioma of the lung: histological and cytological features on endobronchial material. Mod Pathol 2004;17:252–7.[Medline]
- Kay S, Still W, Borochowitz D. Sclerosing hemangioma of the lung: an endothelial or epithelial neoplasm? Hum Pathol 1977;8:468–74.[Medline]
- Katzenstein AA, Weise DL, Fulling K, Battifora H. So-called sclerosing hemangioma of the lung. Evidence of mesothelial origin. Am J Surg Pathol 1983;7:3–14.[Medline]
- Satoh Y, Tsuchiya E, Weng SY, Kitagawa T, Matsubara T, Nakagawa K, et al. Pulmonary sclerosing hemangioma of the lung. A type II pneumocytoma by immunohistochemical and immunoelectron microscopic studies. Cancer 1989;64:1310–7.[Medline]
- Yoo SH, Jung KC, Kim JH, Sung SW, Chung JH, Shim YS, et al. Expression patterns of markers for type II pneumocytes in pulmonary sclerosing hemangiomas and fetal lung tissues. Arch Pathol Lab Med 2005;129:915–9.[Medline]
- Yano M, Yamakawa Y, Kiriyama M, Hara M, Murase T. Sclerosing hemangioma with metastases to multiple nodal stations. Ann Thorac Surg 2002;73:981–3.[Abstract/Free Full Text]
TOP
ABSTRACT
INTRODUCTION
