Asian Cardiovasc Thorac Ann 2003;11:113-115
© 2003 Asia Publishing EXchange Ltd
Pulmonary Sclerosing Hemangiomas: New Approach in Patients With Low Cancer Risk
Balakrishnan Mahesh, FRCS1,
Edward Sheffield, FRCPath2,
Christopher Forrester-Wood, FRCS1,
Khalid Amer, FRCS(CTh)1
1 Department of Thoracic Surgery
2 Department of Pathology, Bristol Royal Infirmary, Bristol, England, UK
For reprint information contact: Khalid Amer, FRCS(CTh) Tel: 44 117 9284210 Fax: 44 117 9283522 email: khalid.amer{at}btinternet.com Department of Thoracic Surgery, Bristol Royal Infirmary, Bristol BS2 8HW, England, UK.
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ABSTRACT
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Three cases of sclerosing hemangioma managed differently are described. Thoracic surgeons should raise their awareness of this benign slow-growing tumor in the differential diagnosis of solitary pulmonary nodule. Radiographic evidence of expansion of such a lesion is not conclusive for malignancy, and should not affect its biological behavior. Intraoperative frozen-section histology may prevent unnecessary resection of lung tissue. Extracapsular enucleation is the treatment of choice for sclerosing hemangiomas.
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INTRODUCTION
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Liebow and Hubbell1 first described pulmonary sclerosing hemangioma (PSH) in 1956. Such tumors had been reported under several other names in the past, including xanthoma, fibroxanthoma, plasma cell granuloma, histiocytoma, sclerosing pneumocytoma, and alveolar angioblastoma.2 They have often been treated by lobectomy on the mistaken diagnosis of adenocarcinoma on fine-needle aspiration cytology, with which it can easily be confused. 3 We report 3 cases of PSH, including one where resection of lung tissue was avoided because of intraoperative frozen-section diagnosis.
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CASE 1
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A 38-year-old asymptomatic woman undergoing chest radiography for immigration purposes was discovered to have a solitary pulmonary nodule (SPN) in the left upper zone. She had no history of smoking or exposure to asbestos, radiation, or tuberculosis. Computed tomography of the chest confirmed the presence of a 3.5-cm lesion in the left upper lobe, and the absence of mediastinal lymphadenopathy. Hematologic and biochemical profiles were normal. Spirometry revealed a forced expiratory volume in the first second (FEV1) of 2.2 L (90% of predicted) and a forced vital capacity (FVC) of 2.8 L (92% of predicted). The patient was admitted for a left thoracotomy and frozen-section histology. At operation, a 3-cm mass was encountered in the apicoposterior segment of the left upper lobe. As the patient was at low risk of malignancy, the lesion was enucleated by diathermy through a plane 1–2 mm peripheral to the tumor capsule; only a thin sheet of normal parenchyma was removed with the tumor (Figure 1
). Frozen-section histology indicated a benign hemangioma with an epithelial element highly suggestive of PSH. No further action was taken and the thoracotomy was closed. The chest was re-explored in the immediate postoperative period for excessive bleeding. A small bleeder was found in the inferior pulmonary ligament and easily controlled by diathermy. The patient subsequently made an uneventful recovery and was discharged on the 10th postoperative day. Histology of the paraffin-embedded specimen revealed blood-filled spaces separated by septa lined by small regular polyhedral cells in cohesive groups. The lining cells showed epithelial characteristics on immunochemistry, and they were positive for the lung epithelial marker for thyroid transcription factor-1. The features were those of PSH. The patient remained well with a satisfactory chest radiograph 10 months after her operation.
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Figure 1. Photograph of pulmonary sclerosing hemangioma resected by extracapsular enucleation in case 1.
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CASE 2
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A 54-year-old woman presented with a single episode of hemoptysis. Chest radiography revealed SPN in the right mid zone. Significant medical history included total gastrectomy, distal pancreatectomy, and splenectomy for carcinoma of the stomach 3 years previously. This nodule had been present on a chest radiograph taken at the time of the abdominal operation (Figure 2A), and it was managed conservatively because of its benign features. The lesion had subsequently increased in size (Figure 2B). Hematologic and biochemical profiles were normal. Spirometry revealed FEV1 of 2 L (90% of predicted) and FVC of 2.75 L (92% of predicted). At thoracotomy, a 4-cm tumor was found occupying the apical segment of the lower lobe, with a collection of reactive-looking interlobar lymph nodes. The tumor was removed by lobectomy because it was not anatomically possible to perform a smaller lung-sparing procedure. Histology of the specimen revealed PSH with the same histological and immunocytochemical features as the lesions in cases 1 and 3. All interlobar lymph nodes showed reactive changes only. Postoperative recovery was complicated by a small air-leak which settled on low suction and water-seal drainage for 8 days. The patient was discharged on the 13th postoperative day. After 18 months of follow-up, she remained asymptomatic with a clear chest radiograph.
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Figure 2. Chest radiographs of case 2, showing the increase in size of a solitary pulmonary nodule, from 3 years before thoracotomy (A) to one day before thoracotomy (B).
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CASE 3
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A 72-year-old woman presented with a dry cough that had persisted for 3 months. Chest radiography showed SPN in the right mid zone. Computed tomography confirmed the lesion to be in the middle lobe, with no mediastinal lymphadenopathy. The patient had undergone total hip replacement 10 years earlier, which was complicated by pulmonary embolism. Her hematologic and biochemical profiles were normal. Spirometry revealed FEV1 of 1.85 L (93% of predicted) and FVC of 2.55 L (91% of predicted). At operation, a 4-cm mass was found in the superior segment of the middle lobe. It was removed by a wedge excision with a 2-cm clearance margin, together with a benign-looking 1.5-cm hilar lymph node. Postoperative histology revealed PSH with the same histological and immunocytochemical features described earlier, while the lymph node showed reactive changes only. The patient made an uneventful recovery and was discharged on the 6th postoperative day. She remained well and asymptomatic with a satisfactory chest radiograph 20 months after the operation.
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DISCUSSION
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Pulmonary sclerosing hemangiomas are rare lung tumors that most often present as SPN on a routine chest radiograph, and rarely with hemoptysis. They are usually peripheral (95%) and solitary (96%) masses, seen mostly in women (83%) with a mean age of 46 years. 2 Multiple lesions have also been described. 3 Spencer and Nambu 4 reviewed several series of PSH and proposed a classification based on microscopic features: papillary, solid, mixed papillary and solid, and angiomatous subtypes. These are slow-growing tumors as evidenced by serial radiographs. They are thought to originate from type-2 pneumocytes, based on the detection of surfactant material, muramidase, epithelial membrane antigen, thyroid transcription factor-1, and 
-1 antitrypsin.2 Despite the misnomer "hemangioma", these lesions are mainly epithelial in structure, derived from primitive respiratory epithelium, and generally thought to be hamartomas.5 We do not share this view and regard PSH as slowly growing benign tumors that can arise de novo, as in our second patient. It is generally perceived that an increase in SPN size is an ominous sign, yet this should not be construed as proof of malignancy. Intralesional hemorrhage is the usual cause of the increase in size, and it gives the appearance of hemangioma. Unlike malignant lesions, we believe that the increase in size should not adversely affect the biological behavior of this tumor.
We cannot overemphasize the importance of intraoperative frozen-section histology in resolving the nature of SPN prior to contemplating surgical resection. Confident recognition of PSH should be possible on macroscopic and frozen-section examinations, particularly in a pathology department with regular experience of pulmonary specimens. Adenocarcinoma, especially the bronchioloalveolar type, may be confused with PSH on percutaneous fine-needle aspiration biopsy.6 However, some authors are of the opinion that careful attention to the cytological patterns and features of the tumor cells, when combined with appropriate clinical correlation, should enable a confident diagnosis of PSH to be made on percutaneous fine-needle aspiration biopsy alone.7
In patients with a high risk of malignancy, the absence of a definitive preoperative histological diagnosis makes lobectomy a feasible option. It is sometimes acceptable for patients with compromised lung function and poor performance status to undergo wedge resection with adequate clearance margins, rather than lobectomy. Yet the controversy continues as lobectomy is the gold standard for the treatment of malignant lung conditions.8 Our second patient was at high risk of malignancy, yet she lost a lobe because of a benign lesion. This may influence her future candidacy for lung resection to remove secondaries. These 3 cases of PSH were managed differently because of their different risks of malignancy. Thoracic surgeons should raise their awareness of this benign slow-growing tumor in the differential diagnosis of SPN, especially in patients with low risks of malignancy. We advocate routine intraoperative frozen-section histology before resection of SPN, especially in such low-risk cases. Extracapsular enucleation is the treatment of choice for sclerosing hemangiomas.
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Acknowledgments
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We are grateful to Mrs. Janet Watkins for her efforts in collecting some of the data required for this article.
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REFERENCES
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- Liebow AA, Hubbell DS. Sclerosing hemangioma (histiocytoma, xanthoma) of the lung. Cancer (Philadelphia)
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- 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
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- Singh G, Katyal SL, Ordonez NG, Dail DH, Negishi Y, Weedn VW, et al. Type II pneumocytes in pulmonary tumors. Implications for histogenesis. Arch Pathol Lab Med
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- Martini N, Beattie EJ Jr. Results of surgical treatment in stage I lung cancer. J Thorac Cardiovasc Surg
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ABSTRACT
INTRODUCTION
