Diagnostic challenge: Sclerosing Hemangioma of the Lung. Department of Medicine, Division of Pulmonary and Critical Care, Lincoln Medical and

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1 Diagnostic challenge: Sclerosing Hemangioma of the Lung. S. Arias M.D, R. Loganathan M.D, FCCP Department of Medicine, Division of Pulmonary and Critical Care, Lincoln Medical and Mental Health Center/Weill Medical College of Cornell University, New York Case Report A 26 years old non-smoker Hispanic female with medical history of bronchial asthma was found to have a 1 cm solitary pulmonary nodule on routine chest radiograph. She had mild persistent asthma with occasional productive cough; there was no dyspnea, chest pain, fever, chills, sweats, or weight loss. Subsequent radiographs and CT scan of the chest two years later confirmed the presence of a 1 cm soft tissue non-calcified nodule in the basilar segment of the left lower lobe. Given the stability in size and expectant follow-up, serial CT scans were recommended after discussion with the patient. Surveillance CT scan after one year showed a stable, smooth walled nodule with no significant change in size. The diameter of the nodule increased in size to 2 cm on a subsequent CT scan done 6 years after initial presentation, there was no thoracic adenopathy or any other abnormality. A transthoracic biopsy of the nodule was done but was non-diagnostic. The patient was referred to surgical biopsy. Frozen sections exam of the wedge resection suggested adenocarcinoma and left lower lobectomy was performed. As the pattern was not fully conclusive for adenocarcinoma, further histological analysis at the Memorial Sloan Kettering Cancer Center was performed. It showed several patterns sclerotic papillary, solid and hemorrhagic patterns of growth, epithelial

2 membrane antigen (EMA) and antibodies to thyroid transcription factor-1 (TTF-1) were positive. These findings confirmed the diagnosis of sclerosing hemangioma of the lung. As this disease can metastasize, a positron emission tomography (PET) with 18 [F]- fluorodeoxyglucose (FDG-PET) was also performed. There was no evidence of metastases or multiorgan involvement. Radiologic Findings Figure 1. Chest xr+ct Figure 2 Chest radiograph shows left lower lobe nodule. Chest CT 6 years after diagnosis. A B C SH show papillary (A), solid/sclerotic (B),hemorrhagic (C) patterns.

3 Discussion Pulmonary sclerosing hemangioma is a relative rare neoplasm of benign clinical course. It presents most frequently in middle aged females.[1, 2] Typically SH is discovered incidentally on chest radiographs as an asymptomatic lesion. Pathologic diagnosis of SH can be a diagnostic challenge, as publish by Katzenstein et al in a series of 33 cases only 42% were correctly diagnosed.[3] Because of the rarity of this condition, there is not clear diagnostic approach. There are few reports in the literature of diagnostic cytology of SH using fine needle aspiration. The most typical characteristic feature of the SH on roetgen examination and Chest computed tomography is the presence of a single spherical or oval coin size well circumscribed homogeneous mass sometimes with punctate calcifications that consist of a non-specific benign solitary lesion.[4] The MRI findings of high signal intensity areas on T1 seem to correspond to solid sclerotic parts. Further evaluation is necessary to confirm MRI diagnostic usefulness. There are few reports on the evaluation of sclerosing hemangioma using FDG-PET and its diagnostic significance still undetermined, more studies and the use of different agents are warranted. On pathology, this uncommon tumor, originally considered vascular in origin, is now generally classified as an epithelial tumor derived from type II pneumocytes.[5] The tumor usually consists of different pathologic patters, including solid cellular areas, papillary structures, sclerotic regions, and dilated blood-filled spaces resembling hemangioma.[6-8] Histologically the lesion characterized by two different cells types, surface cuboidal and stromal polygonal cells. Surface cuboid epithelial cells expresses

4 epithelial membrane antigen (EMA), keratin, CD15, Ber-EP4 whereas polygonal stromal are immunoreactive to the thyroid transcription factor-1 (TTF-1) and EMA.[9] When a papillary pattern predominates, samples of the tumor obtained by needle biopsy or frozen section can be confused with an epithelioid hemangioendothelioma, carcinoid tumor, or well-differentiated papillary adenocarcinoma.[10, 11] Conclusion In summary, we describe a case of pulmonary sclerosing hemangioma diagnosed by open lung biopsy. Diagnostic evaluation of this lesion is a diagnostic challenge due to the nonspecific radiological findings, potential intraoperative dilemmas like difficult frozen section interpretation secondary to atypical cell or artifacts. Deferral of extensive surgical procedures is justified in case of diagnostic doubt. More studies are needed to evaluate extension of this disease, despite metastasis does not seem to change the excellent prognosis. ACKNOWLEDGMENT We thank Dr. Travis, Department of Pathology, Memorial Sloan Kettering Cancer Center, New York for assistance with the pathologic interpretation. References 1. Liebow, A.A. and D.S. Hubbell, Sclerosing hemangioma (histiocytoma, xanthoma) of the lung. Cancer, (1): p Lange, C. and S.B. Vries Robles, Über Lungenangiome bei einem Säugling. European Journal of Pediatrics, (5): p

5 3. Katzenstein, A.L., J.T. Gmelich, and C.B. Carrington, Sclerosing hemangioma of the lung: a clinicopathologic study of 51 cases. Am J Surg Pathol, (4): p Im, J.G., et al., Sclerosing hemangiomas of the lung and interlobar fissures: CT findings. J Comput Assist Tomogr, (1): p Yousem, S.A., et al., So-called sclerosing hemangiomas of lung. An immunohistochemical study supporting a respiratory epithelial origin. Am J Surg Pathol, (8): p Dail, D.H. and S.P. Hammar, Pulmonary pathology. 1994, New York: Springer- Verlag. 7. Thurlbeck, W.M. and A. Churg, Pathology of the Lung. 1995: Thieme. 8. Sugio, K., et al., Sclerosing hemangioma of the lung: radiographic and pathological study. Ann Thorac Surg, (2): p Yatabe, Y., T. Mitsudomi, and T. Takahashi, TTF-1 expression in pulmonary adenocarcinomas. Am J Surg Pathol, (6): p Iyoda, A., et al., Imprint cytologic features of pulmonary sclerosing hemangioma: comparison with well-differentiated papillary adenocarcinoma. Cancer, (3): p Chan, A.C.L. and J.K.C. Chan, Can pulmonary sclerosing haemangioma be accurately diagnosed by intra-operative frozen section? Histopathology, (5): p