IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 7, July 201 3. ISSN – [0976-31 98]

IJCRI 201 3;4(7):396–398.www.ijcasereportsandimages.com

Pulmonary epithelioid hemangioendothelioma identifiedby computed tomography scanYoshiro Nakahara, Tatsuru Okamura, Kan Kato, Tsunekazu Hishima

CASE REPORTComputed tomography (CT) scan showed bilateral,nodular, lung shadows in an asymptomatic 38­year­oldfemale (Figure 1). Five nodules up to 10 mm each wereidentified in the right and left lungs. She was followed­up for four months, during which the size and density ofthese shadows remained unaltered. The patientrequested a definitive diagnosis and underwent a video­assisted thoracoscopic lung biopsy of two lesions in theright lower lobe (S6c, S9a). A histopathologicalexamination showed vacuolated tumor cells,immunohistochemical staining of which was positive forthe endothelial marker CD34. Electron microscopyidentified Weibel–Palade bodies. These findingssupported a diagnosis of pulmonary epithelioidhemangioendothelioma (PEH) (Figure 2). All otherorgans were free of abnormalities and the patient wasremained under observation for two years withouttreatment or disease progression. DISCUSSION

The grade of malignancy of rare, vascular pulmonaryepithelioid hemangioendothelioma (PEH) is low tointermediate. These tumors can simultaneously orsequentially arise from the lungs, liver, bone and softtissue, which renders multicentric tumor growthdifficult to distinguish from primary lesions withmetastasis to other tissues. Most patients withpulmonary epithelioid hemangioendothelioma (PEH)are female (61–80%) and the median age is 36–50years, which is relatively younger than those with lungcancer. No specific symptoms are associated with PEH,and 50–76% of patients are asymptomatic at the timethe condition is detected by chest radiography. Ourpatient was also asymptomatic. Multiple pulmonarynodules, multiple pulmonary reticulonodular opacities,and diffuse infiltrative pleural thickening have beenidentified by computed tomography (CT) scan [1]. Themost common feature of PEH on chest CT scan is thepresence of multiple small discrete pulmonary nodulesof up to 2 cm with well­defined margins in both lungs.However, most nodules are less than 1 cm in diameter.Dail et al. reviewed 20 patients and found that 20, 65%

CLINICAL IMAGE OPEN ACCESS

Yoshiro Nakahara1 , Tatsuru Okamura1 , Kan Kato2,Tsunekazu Hishima3

Affi l iations: 1Department of Thoracic Oncology andRespiratory Medicine, Tokyo Metropolitan Cancer andInfectious Diseases Center Komagome Hospital, Tokyo,Japan; 2Department of Respiratory Medicine, TokyoKensei Hospital, Tokyo, Japan; 3Department of PathologyTokyo Metropolitan Cancer and Infectious, DiseasesCenter, Komagome Hospital, Tokyo, Japan.Corresponding Author: Yoshiro Nakahara, MD,Department of Thoracic Oncology and RespiratoryMedicine, Tokyo Metropolitan Cancer and InfectiousDiseases Center Komagome Hospital, 3-1 8-22,Honkomagome, Bunkyo-ku, Tokyo 11 3-8677, Japan; Ph:+81 - 3-3823-21 01 ; Fax: +81 - 3-3823-5433; E-mail :md1 00062@cick. jp

Received: 1 7 January 201 3Accepted: 25 Apri l 201 3Published: 01 July 201 3

Nakahara et al. 396

Figure 1: Computed tomography scan of chest. Bilateral,multiple, nodular, lung shadows range in size up to 10 mm.

IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 7, July 201 3. ISSN – [0976-31 98]

IJCRI 201 3;4(7):396–398.www.ijcasereportsandimages.com Nakahara et al. 397

and 25% of them had < 10, 10–20 and > 20 nodules,respctively [2]. On the other hand, Kitaichi et al.reported the CT scan of 18 patients, among whom fourand 14 had unilateral and bilateral opacities,respectively. Two of the four with unilateral opacitieshad a single nodular opacity and one patient had pleuraleffusion without nodular opacities. All 14 patients withbilateral opacities had plural nodules, and one patienthad pleural effusion. None of them hadlymphadenopathy [3]. Surgery is an option forunilateral nodules, but a single effective treatment hasbeen suggested for multiple or bilateral involvement [4].Although PEH usually grows very slowly, it canmetastasize to other organs. The 5­year survival rate isaround 60%. Respiratory failure (41.9%) and metastasis(38.7%) are common causes of death [4]. Hemorrhagicsymptoms such as hemoptysis and pleural hemorrhagiceffusion might indicate a poor prognosis [4].

CONCLUSIONSince multiple small lung nodules are likely bediscovered more frequently with the increasingpopularity of computed tomography (CT) screening,pulmonary epithelioid hemangioendothelioma (PEH)should be considered as a differential diagnosis.

*********Nakahara Y, Okamura T, Kato K, Hishima T. Pulmonaryepithelioid hemangioendothelioma identified bycomputed tomography. International Journal of CaseReports and Images 2013;4(7):396–398.

*********doi:10.5348/ijcri­2013­07­340­14

*********Author ContributionsYoshiro Nakahara – Substantial contributions toconception and design, Acquisition of data, Analysis andinterpretation of data, Drafting the article, Revising itcritically for important intellectual content, Finalapproval of the version to be publishedTatsuru Okamura – Substantial contributions toconception and design, Acquisition of data, Analysis andinterpretation of data, Drafting the article, Revising itcritically for important intellectual content, Finalapproval of the version to be publishedKan Kato – Substantial contributions to conception anddesign, Acquisition of data, Analysis and interpretationof data, Drafting the article, Revising it critically forimportant intellectual content, Final approval of theversion to be publishedTsunekazu Hishima – Substantial contributions toconception and design, Acquisition of data, Analysis andinterpretation of data, Drafting the article, Revising itcritically for important intellectual content, Finalapproval of the version to be publishedGuarantorThe corresponding author is the guarantor ofsubmission.Conflict of InterestAuthors declare no conflict of interest.Copyright© Yoshiro Nakahara et al. 2013; This article isdistributed under the terms of Creative Commonsattribution 3.0 License which permits unrestricted use,distribution and reproduction in any means providedthe original authors and original publisher are properlycredited. (Please see www.ijcasereportsandimages.com/copyright­policy.php for more information.)

Figure 2: (A) Macroscopic appearance of the resected tumor(right lower lobe:S6c) showing whitish solid material.Vacuolated tumor cells were observed by Hematoxylin­eosinstain, (B) Immunohistochemical staining of tumor cells. Cellsare positive for CD34, an endothelial marker. Electronmicroscopy shows Weibel–Palade bodies.

IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 7, July 201 3. ISSN – [0976-31 98]

IJCRI 201 3;4(7):396–398.www.ijcasereportsandimages.com Nakahara et al. 398

REFERENCES1. Kim EY, Kim TS, Han J, Choi JY, Kwon OJ, Kim J.Thoracic epithelioid hemangioendothelioma:imaging and pathologic features. Acta Radiol2011;52(2):161–6.2. Dail DH, Liebow AA, Gmelich JT, et al. Intravascularbronchiolar, and alveolar tumor of thelung(IVBAT).An analysis of twenty cases of apeculiar sclerosing endothelial tumor. Cancer1983;51(3):452–64.3. Kitaichi M, Nagai S, Nishimura K, et al. Pulmonaryepithelioid hemangioendothelioma in 21 patients,including three with partial spontaneous regression.Eur Respir J 1998;12(1):89–6.4. Bagan P, Hassan M, Le Pimpec Barthes F, et al.Prognostic factors and surgical indications ofpulmonary epithelioid hemangioendothelioma: areview of the literature. Ann Thorac Surg2006;82(6):2010–3.

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