Background

SH is a well-circumscribed lung parenchymal lesion. Interest in the cell of origin for the tumor has been around since it was first described by Liebow in 1956. The round cells, thought to be the neoplastic cell, have been proposed to originate from several cell types including endothelial, mesothelial, mesenchymal, and pulmonary epithelial cells of alveolar and bronchiolar type. With modern immunohistochemical techniques employed by pathologists, it is clear that this is a tumor of epithelial origin and not endothelial or vascular, as the name suggests.

LABORATORY/RADIOLOGIC/ OTHER TESTS	CHARACTERIZATION
Radiographs
CT scan and MRI
Sclerosing hemangiomas of the lung and interlobar fissures: CT findings. Im JG, Kim WH, Han MC, Han YM, Chung JW, Ahn JM, Do YS. Department of Radiology, Seoul National University Hospital, Korea.	J Comput Assist Tomogr 1994 Jan-Feb;18(1):34-8 OBJECTIVE: Sclerosing hemangioma is a rare pulmonary tumor. This article describes CT features of the tumor on the basis of CT-pathology correlation. MATERIALS AND METHODS: The CT features of eight patients with sclerosing hemangioma of the lung were studied retrospectively and were correlated with the pathologic findings of the resected specimens. All eight patients had an incidental lung mass found on chest radiographs. Seven patients were female. Contrast enhancement of the masses was assessed visually by two radiologists independently, and the density was measured before and after contrast enhancement by using a standard cursor in three patients. RESULTS: On CT, sclerosing hemangiomas appeared as well defined juxtapleural masses in all cases. The tumors enhanced by visual criteria in all but one patient with the smallest mass. The CT density of the enhancing mass ranged from 96 to 157 HU. Three patients had calcification and two had well defined areas of low attenuation. On CT-pathology correlation, the higher, iso-, and lower attenuation areas corresponded with angiomatous, solid and sclerotic, and cystic areas, respectively. CONCLUSION: A well defined juxtapleural mass with marked contrast enhancement coupled in select cases with foci of sharply marginated areas of low attenuation and calcification are characteristic CT findings of sclerosing hemangioma and should suggest this diagnosis especially in women with these findings.
Pulmonary sclerosing hemangioma: report of a case with emphasis on dynamic MR imaging findings. Nakanishi K, Kohzaki S, Fujimoto S, Horita Y, Hayashi K. Department of Radiology, Nagasaki Municipal Hospital, Japan.	Radiat Med 1997 Mar-Apr;15(2):117-9 Abstract quote We report a case of pulmonary sclerosing hemangioma, a rare benign neoplasm. Marked homogeneous enhancement was noted on postcontrast CT. On the dynamic magnetic resonance (MR) imaging study, peak enhancement occurred 2 minutes after the administration of gadolinium-diethylenetriaminepentaacetic acid (Gd-DTPA).

 

HISTOLOGICAL TYPES	CHARACTERIZATION
General	Circumscribed tumor composed of round cells with bland nuclei, intermixed with papillary and tubular structures lined by cuboidal cells The cuboidal cells are thought to be entrapped alveolar pneumocytes and bronchiolar epithelium and the round cells have been regarded as the neoplastic cells
VARIANTS

 

SPECIAL STAINS/IMMUNOPEROXIDASE/ OTHER	CHARACTERIZATION
Special stains
Immunoperoxidase
Pulmonary sclerosing hemangioma of the lung. A type II pneumocytoma by immunohistochemical and immunoelectron microscopic studies. Satoh Y, Tsuchiya E, Weng SY, Kitagawa T, Matsubara T, Nakagawa K, Kinoshita I, Sugano H. Department of Pathology, Cancer Institute, Tokyo, Japan.	Cancer 1989 Sep 15;64(6):1310-7 Abstract quote Three cases of pulmonary sclerosing hemangioma were studied by immunohistochemical and immunoelectron microscopic methods using a panel of antibodies. S ix cases of adenocarcinoma of the lung, three cases of normal mesothelium, and three cases of mesothelioma were used as controls. The cytoplasm of some of the sclerosing hemangioma tumor cells was positive for the anti-lung surfactant apoprotein monoclonal antibody (PE-10). These cells were the pale cells of the solid areas, the cells covering the papillary projections, and the cells lining the cleft-like spaces. These cells also were positive for conventional epithelial cell markers. Some cells also were positive for vimentin. Electron microscopic study showed that the predominant cell was a poorly differentiated pneumocyte. Immunoelectron microscopic study also demonstrated that PE-10 existed in the rough endoplasmic reticulum of some of the cells in the solid areas, in the same way as normal type II pneumocytes. We concluded that the sclerosing hemangioma is an epithelial tumor with differentiation towards type II pneumocytes.
Pulmonary sclerosing hemangioma consistently expresses thyroid transcription factor-1 (TTF-1): a new clue to its histogenesis. Chan AC, Chan JK. Department of Pathology, Queen Elizabeth Hospital, Hong Kong.	Am J Surg Pathol 2000 Nov;24(11):1531-6 The histogenesis of pulmonary sclerosing hemangioma has remained controversial despite extensive studies by many investigators. The availability of an antibody to thyroid transcription factor-1 (TTF-1), which is expressed in type II pneumocytes and Clara cells, has prompted us to readdress this issue. Sixteen cases were immunostained with a panel of antibodies including TTF-1. The patients were predominantly women with an age range of 30 to 73 years (mean, 52 yrs). All tumors were solitary. The single male patient showed regional lymph node metastases, an unusual occurrence reported only once in the literature. All cases exhibited the classic histologic features, with variegated patterns. TTF-1 expression was observed in both the surface lining cells and the pale polygonal cells. The surface lining cells were epithelial membrane antigen (EMA)+ cytokeratin+ surfactant apoprotein A+, whereas the polygonal cells were EMA+ cytokeratin- surfactant apoprotein A-. The neuroendocrine markers synaptophysin and chromogranin were both negative. The metastatic deposits in the lymph nodes comprised only polygonal cells and exhibited an EMA+ cytokeratin- surfactant apoprotein A- TTF- 1+ immunophenotype. These results suggest that pulmonary sclerosing hemangioma is an epithelial neoplasm derived from primitive respiratory epithelium or incompletely differentiated type II pneumocyte or Clara cell.
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 Mojgan Devouassoux–Shisheboran, M.D.; Tomayoshi Hayashi, M.D.; R. Ilona Linnoila, M.D.; Michael N. Koss, M.D.; William D. Travis, M.D. From the Department of Pulmonary Pathology (M.D.-S., M.N.K., W.D.T.), Armed Forces Institute of Pathology, Washington, DC; the Department of Pathology (M.D.-S.), Hospices Civils de Lyon, Hôpital de la Croix Rousse, Lyon, France; the Department of Pathology (T.H.), Nagasaki University Hospital, Japan; and the Cell and Cancer Biology Department (R.I.L.), Medicine Branch, National Cancer Institute, National Institutes of Health, Rockville, Maryland, U.S.A.	Am J Surg Pathol 2000;24:906-916 Abstract quote Pulmonary sclerosing hemangioma (SH) is a lung neoplasm of uncertain histogenesis that is composed of two major cell types: surface and round cells. The authors studied 100 cases of pulmonary SH that presented as a peripheral (95%), solitary (96%) mass of less than 3 cm in diameter (74%) in asymptomatic patients who were mostly women (83%) with a mean age of 46.2 years. Immunohistochemistry of multiple epithelial, mesothelial, pneumocyte, neuroendocrine, and mesenchymal markers was performed on 47 cases to investigate the histogenesis of this neoplasm. Both surface and round cells stained with epithelial membrane antigen (EMA) and thyroid transcription factor-1 (TTF-1) in more than 90% of cases; however, the round cells were uniformly negative for pancytokeratin and positive for cytokeratin-7 and CAM5.2 in only 31% and 17% of cases, respectively. Surfactant proteins A and B as well as Clara cell antigen were positive in varying numbers of surface cells but they were negative in the round cells. Neuroendocrine cells either as isolated scattered cells or as a tumorlet within the center of SH were detected (chromogranin, Leu-7, synaptophysin positive) in three cases. The expression of TTF-1 in the absence of surfactant proteins A and B and Clara cell antigens in the round cells of SH suggests that they are derived from primitive respiratory epithelium. The alveolar pneumocytes and neuroendocrine cells may either represent phenotypic differentiation of a primitive respiratory epithelial component or they may correspond to non-neoplastic entrapped or hyperplastic elements. The concomitant positivity of both cell types in SH for TTF-1 and EMA, and the negativity of round cells for pancytokeratin and neuroendocrine markers, provide useful clues not only for histogenesis but also for the diagnosis of this lung neoplasm.
Expression of thyroid transcription factor-1 and other markers in sclerosing hemangioma of the lung. Illei PB, Rosai J, Klimstra DS. Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY (Drs Illei and Klimstra); and the Dipartimento di Patologia, Istituto Nazionale Tumori, Via G. Venezian 1, 20133 Milano, Italy (Dr Rosai).	Arch Pathol Lab Med 2001 Oct;125(10):1335-9 Context.-Sclerosing hemangioma of the lung is well characterized histologically, but the line of differentiation expressed by the tumor cells has been unclear. Despite the implication by its name of a vascular neoplasm, sclerosing hemangioma is considered by most authorities to be an epithelial tumor, possibly related to the pulmonary epithelium. Objectives.-To determine the line of differentiation of the tumor cells with immunohistochemistry and to review the related literature. Design.-Nine cases of histologically typical pulmonary sclerosing hemangioma were studied with pan-epithelial (epithelial membrane antigen [EMA] and CAM 5.2), endothelial (CD31), neuroendocrine (chromogranin A), and pulmonary epithelial markers (thyroid transcription factor-1 and PE10). Staining intensity was separately evaluated in the pale cells of the solid areas and the cells lining the papillary structures. Results.-Both cell types were positive for thyroid transcription factor-1 and EMA in all cases (100%). Thyroid transcription factor-1 showed diffuse strong staining, and EMA staining varied from focal weak to diffuse strong. The pale cells showed focal staining for keratin (CAM 5.2) in 2 (28%) of 7 cases, and for PE10 in 5 (62%) of 8 cases. The papillary lining cells were at least focally positive with CAM 5.2 and PE10 in all cases (100%). Reactions for chromogranin and CD31 were negative in both cell types in every case. The number of PE10- or CAM 5.2-positive papillary lining cells was less than the number of EMA-positive papillary lining cells. Conclusion.-The uniform positivity for EMA is consistent with the notion that the tumor cells of sclerosing hemangioma are epithelial, and the strong thyroid transcription factor-1 positivity suggests differentiation toward pulmonary epithelium. The papillary lining cells expressing EMA as well as PE10 or CAM 5.2 likely represent entrapped metaplastic alveolar epithelium, whereas the papillary lining cells expressing only EMA more likely constitute true neoplastic cells similar to those in the solid areas.

 

PROGNOSIS AND TREATMENT	CHARACTERIZATION
Treatment	Surgical excision

Liebow AA, Hubbell DS. Sclerosing hemangioma (histiocytoma, xanthoma) of the lung. Cancer 1956;9:53–75.
Sternberg S. Diagnostic Surgical Pathology. Third Edition. Lipincott Williams and Wilkins 1999.

Lungs

Last Updated 8/15/2001

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