Thoracic Cancer ISSN 1759-7706

CASE REPORT

Pulmonary sclerosing pneumocytoma mimicking lung cancer: Case report and review of the literature Jung-Hwan Lim1*, Nuri Lee2*, Dae-Woong Choi2, Hyung-Joo Oh1, Ha Young Park1, Ki-Hyun Kim1, Tae-Ok Kim1,3, Cheol-Kyu Park1,3, Hong-Jun Shin1, Yoo-Duk Choi1,4, Ju-Sik Yun3,5, Sang-Yun Song3,5 & In-Jae Oh1,3 1 2 3 4 5

Department of Internal Medicine, Chonnam National University Medical School, Gwanju, Korea Chonnam National University Medical School, Gwangju, Korea Lung and Esophageal Cancer Clinic, Chonnam National University Hwasun Hospital, Jeonnam, Korea Department of Pathology, Chonnam National University Medical School, Gwangu, Korea Department of Thoracic and Cardiovascular Surgery, Chonnam National University Hwasun Hospital, Jeonnam, Korea

Keywords Positron emission tomography; pulmonary sclerosing hemangioma; solitary pulmonary nodule. Correspondence In-Jae Oh, Lung and Esophageal Cancer Clinic, Chonnam National University Hwasun Hospital, 322 Seoyang-ro, Hwasun, Jeonnam 519-763, Korea. Tel: +82 61 379 7617 Fax: +82 61 379 7619 Email: [email protected]

Abstract Pulmonary sclerosing pneumocytoma (PSP) is a rare benign neoplasm that predominantly affects middle-aged Asian women. PSP is often asymptomatic and demonstrates a solitary pulmonary nodule on radiologic examination. We report a case of PSP initially misdiagnosed as lung cancer because of strong 18Ffluorodeoxyglucose (FDG) uptake revealed by 18F-FDG positron emission tomography-computed tomography scan. After surgery, pathology revealed that the tumor cells were immunopositive for epithelial membrane antigen and thyroid transcription factor-1. The patient has been followed up without complication or recurrence.

*Jung-Hwan Lim and Nuri Lee contributed equally to this work. Received: 10 January 2016; Accepted: 19 January 2016. doi: 10.1111/1759-7714.12341 Thoracic Cancer 7 ••(2016) (2016)508–511 ••–••

Introduction Pulmonary sclerosing pneumocytoma (PSP) is a rare benign neoplasm that was first described over 50 years ago as pulmonary sclerosing hemangioma.1 PSP predominantly affects middle-aged adults over 50 years of age, with a female to male ratio of 5:1.2,3 The nomenclature of this disease came from a concept that sclerosing hemangioma has an endothelial and vascular origin. However, several immunohistochemical studies have revealed that sclerosing hemangioma originates from primitive respiratory epithelium, most likely type II alveolar pneumocytes.2 The key feature of PSP is the presence of two cell types – cuboidal surface cells and stromal round cells – both of which are considered to be neoplastic.4 Pulmonary sclerosing pneumocytoma is usually a solitary, well-circumscribed nodule or mass. Although some features 508 Thoracic Cancer •• Cancer (2016) ••–•• Thoracic 7 (2016) 508–511

have been determined, there is still no definitive diagnostic radiologic finding. Because 18F-fluorodeoxyglucose (FDG) positron emission tomography-computed tomography (PET-CT) is widely used to evaluate cancer staging or screening, the chance of false positive interpretation has increased. We report a case of surgically diagnosed PSP demonstrating a positive result for lung malignancy on the preoperative 18FFDG PET/CT scan.

Case report In January 2015, a 36-year-old woman visited our hospital for evaluation of a solitary pulmonary nodule in her left lung. She had no respiratory symptoms or a history of smoking or previous pulmonary tuberculosis. The nodule had been detected in December 2013 in another hospital (Fig 1a). At

© The 2016Authors. The Authors. Thoracic published by China Lung Oncology andWiley John Wiley Sons Australia, © 2016 Thoracic CancerCancer published by China Lung Oncology GroupGroup and John & Sons&Australia, Ltd Ltd1 This isis an an open access article under the terms of the Creative Commons Attribution-NonCommercial License, which which permits permits use, use, distribution distribution and and reproduction reproduction in in any any medium, medium, This Attribution-NonCommercial License, provided the the original original work work isis properly properly cited cited and and isis not not used used for for commercial commercial purposes. purposes. provided

J.-H. Lim et al. Pulmonary sclerosing pneumocytoma

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Figure 1 (a) Computed tomography (CT) obtained at another hospital during percutaneous needle biopsy shows a 1.5 cm round nodule in the left lower lobe, adjacent to the left cardiac border. (b) CT performed six months later revealed a slightly increased nodule diameter of 2 cm. (c) The nodule was solitary, well-circumscribed, and round-shaped in the anteromediobasal segment of the left lower lobe. (d) 18F- fluorodeoxyglucose (FDG) positron emission tomography-CT revealed high FDG uptake (maximum standardized uptake value = 3.9) at the nodule without regional or distant metastasis.

that time, a percutaneous needle biopsy revealed no evidence of malignancy. High-resolution CT scans were taken every six months; a scan taken in December 2014 revealed an increase in nodule diameter to 3 cm (Fig 1c). The mass was a solitary, well-circumscribed, heterogeneously enhanced feature located in the anteromediobasal segment of the left lower lobe. There was no evidence of enlarged lymph node or pleural effusion. At the time of the patient’s visit to our hospital, serum carcinoembryonic antigen (CEA) and cancer antigen (CA) 19-9 levels were within normal range (0.78 ng/mL and 6.2 U/ mL, respectively). 18F-FDG PET-CT demonstrated high uptake of FDG at the nodule (maximum standardized uptake value [SUVmax] = 3.9; Fig 1d). Malignancy was suspected, with no metabolic evidence of regional or distant metastasis. Bronchoscopy showed no gross specific finding, except for mild edematous mucosa. A wedge resection followed by video-assisted thoracic surgery was planned to make a definitive diagnosis and determine treatment for preoperative clinical stage IIB (T2aN0M0) lung cancer. No malignant cells were evident in the frozen sections of tissue retrieved during the operation. This finding did not correspond with hamartoma, which is the most common ThoracicThoracic Cancer Cancer 7 (2016)••508–511 2 (2016) ••–••

benign tumor of the lung. The surgery ended with only a wedge resection of the anteromedial basal segment of the left lung. A permanent section biopsy revealed that the tumor was composed of monotonous, round tumor cells covered by hyperchromatic spindle cells (Figs 2, 3). Immunohistochemical stains were positive for cytokeratin7 (CK7), epithelial

Figure 2 After surgical resection, a solid, yellow mass about 2.5 cm in size was separated by normal lung parenchyma.

© 2016 The Authors. published by China Lung Oncology Group and John and Wiley & Sons Australia, Ltd © 2016 The Thoracic Authors. Cancer Thoracic Cancer published by China Lung Oncology Group John Wiley & Sons Australia, Ltd 509

J.-H. Lim et al. Pulmonary sclerosing pneumocytoma

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Figure 3 (a) A well-demarcated solid mass was easily separated by normal lung parenchyma (hematoxylin and eosin [H&E], × 20). (b) The hemorrhagic mass is situated in the sub-bronchial area (H&E, × 40). (c) Focal sclerotic change was shown within the tumor (H&E, × 100). (d) The tumor was composed of monotonous, round tumor cells covered by hyperchromatic spindle cells (H&E, × 400). (e) Immunohistochemical staining detected epithelial membrane antigen in the surface of the tumor cells, but not in the round cells (× 100). (f) Thyroid transcription factor-1 was detected in both the surface tumor cells and the round cells (× 100).

membrane antigen (EMA), thyroid transcription factor 1 (TTF-1), progesterone receptor (PR), and negative for estrogen receptor (ER), CD56, and synaptophysin. After PSP was confirmed, no adjuvant therapy was needed because the tumor was benign. The patient was discharged without any complication. Follow-up high-resolution CT scans have not detected recurrence.

Discussion Pulmonary sclerosing hemangioma is not a vascular tumor. The tumor is derived from primitive respiratory epithelial cells that express TTF-1.4 PSP is a tumor of pneumocytic origin with a dual population of surface cells resembling type II pneumocytes and round cells, with slightly different histogenetic profiles. Therefore, sclerosing hemangioma is 510 Thoracic Cancer •• Cancer (2016) ••–•• Thoracic 7 (2016) 508–511

classified as sclerosing pneumocytoma, moved from “miscellaneous tumors” in the 1999 and 2004 World Health Organization Classification to “adenomas” in the 2015 World Health Organization Classification.4 Pulmonary sclerosing pneumocytoma is a benign tumor with low prevalence. Patients are usually women with a mean age of 46 years. The most common presenting form is an asymptomatic solitary pulmonary nodule that can grow up to 7 cm in diameter, although 73% of lesions are smaller than 3 cm.2,5 PSP typically presents on chest radiograph as a peripheral, solitary, well-defined, homogenous nodule or mass without predilection for a particular lobe. Calcification or cavitation is hard to see in PSP.6 Enhanced CT of the chest demonstrates a round to oval shaped lesion with smooth margins, but these findings are not specific.7,8 A recent study reported that most patients had a single lesion (92.1%),

© 2016 Authors. Thoracic CancerCancer published by China Lung Oncology GroupGroup and John Sons&Australia, Ltd Ltd 3 © The 2016 The Authors. Thoracic published by China Lung Oncology andWiley John & Wiley Sons Australia,

J.-H. Lim et al. Pulmonary sclerosing pneumocytoma

smooth boundary (65.8%), and oval shape (65.8%), with a mean diameter of 2.27 cm.9 CT signs included marginal pseudocapsule (50%), overlying vessel (26.3%), air gap (2.6%), and halo sign (17.1%).9 Generally, 18F-FDG PET/CT scan results are interpreted as positive for malignancy when the FDG uptake of a lung nodule is greater than the background mediastinal blood pool activity on qualitative assessment, or when the SUVmax values exceed 2.5.10,11 In the present case, 18F-FDG PET/CT showed a false positive result, indicating malignancy. The majority of sclerosing hemangiomas show increased FDG uptake, with SUVmax values significantly correlated with tumor size. Sclerosing hemangioma ≥ 2 cm can frequently be falsely interpreted as malignant by FDG-PET-CT.12 Pulmonary sclerosing pneumocytoma can be very challenging to diagnose in frozen sections, small biopsies, and cytology where they can easily be mistaken for adenocarcinoma or carcinoid tumors.4 These tumors typically have a benign clinical course, with metastasis being exceedingly rare.2 Treatment of PSP involves surgical resection and the prognosis is favorable; recurrence after surgery or death as a result of PSP has not been reported.13 This case of PSP highlights the difficulty in diagnosing this rare tumor. Clinicians should be aware of the possibility of a false positive result for slow-growing, solitary pulmonary nodules with a diameter exceeding 2 cm, even if a 18F-FDG PET-CT scan indicates hypermetabolic malignancy.

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Disclosure No authors report any conflict of interest.

References

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1 Liebow AA, Hubbell DS. Sclerosing hemangioma (histiocytoma, xanthoma) of the lung. Cancer 1956; 9: 53–75. 2 Devouassoux-Shisheboran M, Hayashi T, Linnoila RI, Koss MN, Travis WD. A clinicopathologic study of 100 cases of pulmonary sclerosing hemangioma with

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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. Illei PB, Rosai J, Klimstra DS. Expression of thyroid transcription factor-1 and other markers in sclerosing hemangioma of the lung. Arch Pathol Lab Med 2001; 125: 1335–9. Travis WD, Brambilla E, Nicholson AG et al. The 2015 World Health Organization Classification of Lung Tumors: Impact of genetic, clinical and radiologic advances since the 2004 classification. J Thorac Oncol 2015; 10: 1243–60. Cardemil G, Fernández E, Riffo P et al. [Sclerosing hemangioma presenting as a solitary lung nodule. Report of one case.] Rev Med Chil 2004; 132: 853–6. (In Spanish.) Kim KH, Sul HJ, Kang DY. Sclerosing hemangioma with lymph node metastasis. Yonsei Med J 2003; 44: 150–4. Chung MJ, Lee KS, Han J, Sung YM, Chong S, Kwon OJ. Pulmonary sclerosing hemangioma presenting as solitary pulmonary nodule: Dynamic CT findings and histopathologic comparisons. AJR Am J Roentgenol 2006; 187: 430–7. Cheung YC, Ng SH, Chang JW, Tan CF, Huang SF, Yu CT. Histopathological and CT features of pulmonary sclerosing haemangiomas. Clin Radiol 2003; 58: 630–5. Shin SY, Kim MY, Oh SY et al. Pulmonary sclerosing pneumocytoma of the lung: CT characteristics in a large series of a tertiary referral center. Medicine (Baltimore) 2015; 94: e498. Yap CS, Schiepers C, Fishbein MC, Phelps ME, Czernin J. FDG-PET imaging in lung cancer: How sensitive is it for bronchioloalveolar carcinoma? Eur J Nucl Med Mol Imaging 2002; 29: 1166–73. Lowe VJ, Fletcher JW, Gobar L et al. Prospective investigation of positron emission tomography in lung nodules. J Clin Oncol 1998; 16: 1075–84. Lee E, Park CM, Kang KW et al. 18F-FDG PET/CT features of pulmonary sclerosing hemangioma. Acta Radiol 2013; 54: 24–9. Kalhor N, Staerkel GA, Moran CA. So-called sclerosing hemangioma of lung: Current concept. Ann Diagn Pathol 2010; 14: 60–7.

© 2016 The Authors. published by China Lung Oncology Group and John and Wiley & Sons Australia, Ltd © 2016 The Thoracic Authors. Cancer Thoracic Cancer published by China Lung Oncology Group John Wiley & Sons Australia, Ltd 511

Pulmonary sclerosing pneumocytoma mimicking lung cancer: Case report and review of the literature.

Pulmonary sclerosing pneumocytoma (PSP) is a rare benign neoplasm that predominantly affects middle-aged Asian women. PSP is often asymptomatic and de...
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