Japanese Journal of Clinical Oncology 33:360-363 (2003)
© 2003 Foundation for Promotion of Cancer Research
Sclerosing Hemangioma of the Lung with Cystic Appearance
1 Department of Internal Medicine, Kanto-Chuo Hospital, Tokyo, Departments of 2 Human Pathology, 3 Radiology and 4 Thoracic Surgery, University of Tokyo, Tokyo, Japan
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ABSTRACT |
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 TOP ABSTRACT INTRODUCTIONCASE REPORTPATHOLOGICAL FINDINGSDISCUSSIONREFERENCES |
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A case of cystic sclerosing hemangioma of the lung is reported. A chest X-ray of a 55-year-old woman who had been suffering from a cough with sputum for several months revealed an abnormal nodular shadow. A chest CT scan revealed a solitary tumor with cystic appearance occupying S7 of the right lung and the inferior pulmonary ligament. Radiological differential diagnosis for the lesion included bronchogenic cyst, cystic Schwannoma, pulmonary necrotic carcinoid, and lung carcinoma. Right lower lobectomy was carried out and the lesion was pathologically diagnosed as sclerosing hemangioma of the lung with cystic features, expanding into the pulmonary ligament. Differential diagnosis of the cystic lesion of the lung should include cystic sclerosing hemangioma as observed in this case.
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INTRODUCTION |
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TOPABSTRACTINTRODUCTIONCASE REPORTPATHOLOGICAL FINDINGSDISCUSSIONREFERENCES |
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Sclerosing hemangioma of the lung is an intrapulmonary tumor with variegated histological appearances, but characteristic clinico-radiological features. Sclerosing hemangioma of the lung was observed in a middle-aged woman as a solitary, well-circumscribed, and solid tumor, with radiological images occasionally showing calcification (1–3). However, when the tumor exhibits unusual radiographical presentation, there are several pitfalls in the histological diagnosis of sclerosing hemangioma of the lung. Here, we report such a case where the tumor expanded into the pulmonary ligament, and had a cystic appearance. We will discuss the potential pitfalls in histological diagnoses and how to avoid them by using immunohistochemistry.
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CASE REPORT |
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TOPABSTRACTINTRODUCTIONCASE REPORTPATHOLOGICAL FINDINGSDISCUSSIONREFERENCES |
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A health check screening radiography of a 55-year-old woman who had been suffering from a cough with whitish non-bloody sputum for several months, revealed a coin lesion at the right lower lung near the hilum (Fig. 1). She had been smoking 15 cigarettes daily for 30 years, but had no history of medication or operation. She had not experienced any weight loss. She was admitted for examination and treatment. There were no abnormal findings in the physical examination. Laboratory examinations, including blood gas analysis and respiratory function tests, were within normal ranges. Chest computed tomography (CT) images revealed a well-defined oval lesion located in S7 of the right lung and the inferior pulmonary ligament, bordering the right inferior pulmonary vein and left atrium of the heart without retracting surrounding tissues. The tumor was 2.5 cm in diameter. Contrast enhanced CT revealed slight enhancement only in the margin of the lesion, while the inside of the lesion was heterogeneously hypoattenuate, suggesting its cystic nature (Fig. 2a). Images from T1-weighted magnetic resonance imaging (MRI) with fat suppression, revealed the content of the lesion to be hyperintense (Fig. 2b) and on T2-weighted images it was as hypointense as the surrounding air. These radiological findings suggested a cystic lesion with proteinous content in the S7 of the right lung and inferior pulmonary ligament. With respect to tumor markers, SCC and NSE levels were high (27.4 ng/ml and 12.8 ng/ml respectively) and CEA, CA19-9, and CYFRA were within normal ranges. Differential diagnoses included mediastinal bronchogenic cyst or cystic Schwannoma, and pulmonary necrotic carcinoid or carcinoma. A thoracotomy was performed for diagnosis and treatment. The intraoperative frozen section consisted of atypical cuboidal cells with papillary growth. The possibility of adenocarcinoma could not be ruled out, since there had been no cases where sclerosing hemangioma presented as a cystic tumor. Right lower lobectomy was then performed. The postoperative course was uneventful. The patient received no further treatment and is still disease-free, 27 months after the operation.
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) at the right lower lung near the hilum.
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PATHOLOGICAL FINDINGS |
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TOPABSTRACTINTRODUCTIONCASE REPORTPATHOLOGICAL FINDINGSDISCUSSIONREFERENCES |
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Macroscopically, the tumor measured 2.5x2.2 cm and was located in S7 of the right lung and the inferior pulmonary ligament. The tumor was cystic, contained dark red fluid, and had a thin cyst wall, the inner side of which was covered by a tan and coarse substance.
Microscopically, the lesion was composed of a cyst wall, tumor cells proliferating with various histological patterns, and red blood cells accumulated within the cavity. The cyst wall was fibrous and partly hyalinized, and the tumor cells exhibited various histological patterns, such as papillary, solid, and sclerotic patterns inside the wall (Fig. 3). Epithelioid tumor cells covering the surface of the papillae had a hobnail or cuboidal appearance like Type 2 pneumocytes, and polygonal or round tumor cells were observed inside the papillary structure or in the solid part (Fig. 4). Most of these tumor cells had a bland appearance, but some cells showed cellular atypia with prominent nucleoli. The margin of the lesion was surrounded by the lung tissue, and a part of the tumor cells spread out into the surrounding pulmonary parenchyma. Other associated histological features, such as collection of foamy macrophages, multinucleated giant cells, cholesterol clefts, and lymphoplasmacytic infiltration with scattered lymph follicles were also observed. Tumor invasion into blood or lymph vessels was not observed. Another remarkable histological finding was the destruction of the bronchial wall within the lesion (Fig. 5).
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Immunohistochemical examination with various antibodies was performed on the resected specimens, and nearly all the tumor cells tested positive for TTF-1. Tumor cells covering the surface of the papillae tested positive for cytokeratin (CAM5.2) and surfactant apoprotein A (PE10). On the other hand, polygonal or round tumor cells inside the papillary structure or in the solid part tested negative for cytokeratin and surfactant apoprotein, but positive for vimentin (Fig. 6). The results of the immunohistochemical study are shown in Table 1.
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The pathological specimen was diagnosed as sclerosing hemangioma with cystic features.
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DISCUSSION |
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TOPABSTRACTINTRODUCTIONCASE REPORTPATHOLOGICAL FINDINGSDISCUSSIONREFERENCES |
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Cystic change is rarely observed in sclerosing hemangioma of the lung, although it is accompanied by some amount of hemorrhage in one-third of the cases (1–3). The location of the sclerosing hemangioma on the CT scan in the present case, and the equal involvement of both lung parenchyma and pulmonary ligament, was also uncommon (4). Thus, both unusual findings challenged the radiological and clinical diagnosis of the present tumor. Differential diagnoses included nodular and cystic lesions of both mediastinum and lungs, such as mediastinal bronchogenic cyst or cystic Schwannoma, and pulmonary necrotic carcinoid or carcinoma (5,6). As a large part of the tumor was occupied by cystic fluids, the tumor showed few radiological features that would indicate sclerosing hemangioma of the lung (1,7).
The histological diagnosis of sclerosing hemangioma, especially in the frozen section, is potentially difficult in an unusual setting like this one. Cytological atypia observed in the epithelial lining as well as the papillary pattern in sclerosing hemangioma might lead to a diagnosis of papillary adenocarcinoma (8,9). Unless cautious scanning at low-power view identifies the most characteristic pattern of SHL, namely two-cell components in the solid and cellular trabecle, i.e., epithelial lining and internal round cells (3), the frozen section procedure cannot rule out lung adenocarcinoma as in this case. A combination of the frozen section technique and imprint cytology might be the best method to make an intraoperative diagnosis, as the morphological features of each tumor cell are more obvious in imprint cytology than frozen section. Additionally, imprint cytology can accurately differentiate between sclerosing hemangioma and adenocarcinoma (10). Moreover, recognition of an unusual presentation of sclerosing hemangioma is indispensable for differential diagnosis. Along these lines, it is interesting to point out a recent case report of sclerosing hemangioma arising in the mediastinum (4).
In accordance with the recent immunohistochemical findings, the surface lining cells were cytokeratin (+) SAA (surfactant apoprotein A) (+) vimentin (–), while round cells were cytokeratin (–) SAA (–) vimentin (+)(11,12). An association with pleura and the location of interlobar fissures has been observed in some tumors of sclerosing hemangioma, which once led to the erroneous view that sclerosing hemangioma was derived from mesothelial or submesothelial cells (13). Recent immunohistochemical studies have demonstrated that most of the constituent cells of sclerosing hemangioma express thyroid transcription factor-1 (TTF-1), as confirmed in the present case (12,14). TTF-1 is expressed in Type I and II pneumocytes and Clara cells, but not in mesothelial cells (12,14,15). Thus, the current hypothesis is that sclerosing hemangioma originates from primitive respiratory epithelium with differentiation to Type I and II pneumocytes. Therefore, slow growth of the primitive respiratory epithelium beneath the pleural region might have caused considerable protrusion of the intrapulmonary tumor into the pulmonary ligament, which then mimicked a mediastinal tumor.
The prognosis of patients with sclerosing hemangioma is excellent. In the present case, the patient has been disease-free for 27 months after the operation. However, a small bronchus in the tumor appeared to have been obliterated by the neoplastic cells, the lumen of which contained cholesterol-cleft. The non-neoplastic lung tissue appeared to be integrated within the tumor at the border. These features should not be regarded as local aggressiveness. Both findings, local obliteration of the normal components, and irregular border within the lesion, are also observed in inflammatory processes, such as sclerosing adenosis of the breast (16). It is well known that lymph node metastasis has been extremely rare in sclerosing hemangioma (17).
The cyst formation of the tumor might be caused by excessive hemorrhage or gradual pooling of hemorrhagic contents in the tumor. Sclerosing hemangioma is usually accompanied by hemorrhage, and in the present case, bronchus in the lesion was obstructed and the hemorrhagic contents of the tumor might not have been drained off (2,3).
Here, we have reported a case of SHL expanding into the pulmonary ligament and exhibiting a cystic appearance. When the tumor appears clinically and radiographically unusual, several pitfalls emerge, even in the histological diagnosis of sclerosing hemangioma. A combination of the frozen section and imprint cytology for intraoperative diagnosis, and application of immunohistochemistry (cytokeratin, vimentin, TTF-1) can overcome these potential pitfalls.
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FOOTNOTES |
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+ For reprints and all correspondence: Akiteru Goto, Department of Human Pathology, Graduate School of Medicine, University of Tokyo, 7–3–1, Hongo, Bunkyo-ku, Tokyo 113-0033, Japan. E-mail: akiteru{at}m.u-tokyo.ac.jp
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Received April 30, 2003; accepted June 23, 2003
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