Surg Today DOI 10.1007/s00595-014-0926-0


Surgical management of a patient with bilateral multiple pulmonary epithelioid hemangioendothelioma: report of a case Keisuke Eguchi • Makoto Sawafuji

Received: 9 December 2013 / Accepted: 1 April 2014 Ó Springer Japan 2014

Abstract Pulmonary epithelioid hemangioendothelioma (PEH) is a rare lung disease. This report describes the case of a 54-year-old female who underwent radical resection for bilateral multiple PEH nodules. Bilateral multiple nodular shadows were seen on the patient’s chest X-rays during an annual health check. PEH was diagnosed based on a video-assisted thoracoscopic biopsy specimen. Thirtytwo pulmonary nodules were resected through the bilateral transverse thoracosternotomy. The patient’s postoperative recovery was uneventful, and she remains free of PEH recurrence 11 years after the surgery. Keywords Lung wedge resection  Bilateral thoracotomy  VATS  Multiple lung nodules

Case report A 54-year-old female was referred to Kawasaki Municipal Hospital (Kawasaki City, Kanagawa, Japan) after abnormal findings were noted on a routine chest X-ray taken during an annual health check. She did not have any symptoms or clinical history of malignancy. High-resolution computed tomographic (HRCT) imaging revealed multiple tiny nodules (\1 cm in diameter) in the bilateral lungs (Fig. 1a) and a solitary hepatic nodule (2 cm in diameter). Most nodules were well defined and located in the peripheral lungs, while pleural effusion and mediastinal lymphadenopathy were not present. Tumor specimens acquired via video-assisted thoracoscopy showed pathological features K. Eguchi (&)  M. Sawafuji Department of General Thoracic Surgery, Kawasaki Municipal Hospital, 12-1 Shinkawa-tori, Kawasaki 210-0013, Japan e-mail: [email protected]

of epithelioid hemangioendothelioma (Fig. 2a). Immunohistochemical staining of the tumor was positive for CD31 (Fig. 2b) and factor VIII (Fig. 2c), and negative for cytokeratin. Radiological studies also showed a hepatic lesion that was suspected to have the same histology as the lung nodules. After obtaining informed consent from the patient, radical resection of the pulmonary and hepatic nodules was planned. Under general anesthesia, a clamshell incision was made on the anterior chest, and a bilateral transverse thoracosternotomy was performed in the fifth intercostal space. Most of the pulmonary nodules were visible through the surface of the visceral pleura, while other buried lesions could be manipulated. All palpable nodules with surrounding normal lung tissue were resected using a harmonic scalpel (Ethicon Endo-Surgery, Cincinnati, OH), and running sutures with absorbable thread were placed for each cut edge of the lung. Preoperative HRCT had revealed 12 nodules in the right lung and 10 nodules in the left lung; 21 nodules in the right lung and 11 nodules in the left lung were resected. The size of the nodules ranged 4–8 mm in diameter. After pulmonary resection, the hepatic nodule was enucleated. Although diameter of the hepatic nodule was 2 cm, it was located in the hepatic segment 8, which was under the diaphragmatic dome; therefore, a 10-cm oblique incision in the right upper abdomen was made for the hepatic surgery. The histological diagnosis of the hepatic nodule was hemangioma (Fig. 3). The patient’s postoperative course was uneventful, and she was discharged 10 days after surgery. Follow-up chest HRCT studies have been performed every year in the outpatient setting. The patient never complained of any remarkable respiratory deterioration after the pulmonary surgery. Although she suffered from


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Fig. 1 Computed tomography revealed multiple nodules in the bilateral lung fields (a). Peripheral nodules were seen in the right lung via transverse thoracotomy (b)

Fig. 2 H&E staining of the surgical specimen of the lung showed epithelioid hemangioendothelioma (a, 920). A pulmonary nodule had a central sclerotic hypocellular zone and a cellular peripheral zone. The nuclei of the neoplastic cells were round to oval. Immunohistochemical staining for CD31 (b, 940) and factor VIII (c, 940) showed that the neoplastic cells were positively stained

thyroid cancer and underwent thyroidectomy 10 years after the pulmonary surgery, no recurrence of pulmonary epithelioid hemangioendothelioma (PEH) has been observed during the 11-year follow-up period after the lung surgery.

Comment Pulmonary epithelioid hemangioendothelioma is a rare low-grade malignancy that can metastasize to distant organs [1]. It may also arise from several organs simultaneously or sequentially; therefore, it can be difficult to determine if the tumor is multicentric or is a primary lesion with metastases to other tissues. In this case, the patient also had a hepatic nodule. We presumed preoperatively that the hepatic tumor was of the same origin as the lung nodule; however, the pathological diagnosis of the hepatic tumor was hemangioma. The lung nodules in the present case were accidentally identified on an annual chest X-ray; however, the findings did not indicate aggressive growth of PEH. Compared with


Fig. 3 H&E staining of the surgical specimen of the liver showed cavernous hemangioma (910). The hepatic nodule was composed of cavernous vascular channels and thrombi

the findings on the X-ray taken the year before, no evident changes could be seen in the referral X-ray. Therefore, it was speculated that the tumor was indolent. The main reason of the referral was the patient’s fear of cancer.

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A wide opening through the bilateral transverse thoracosternotomy was appropriate for radical resection of multiple pulmonary nodules. Narrow manual palpation identified more nodules than were visualized on HRCT. However, it is possible that the use of newer HRCT technology would have detected these otherwise unrecognized nodules. The resection of numerous tumors was needed in this case; therefore, minimization of the lung parenchymal loss was also carefully considered. The harmonic scalpel was useful for minimizing the need for staples, and therefore minimized the amount of resected lung volume. Most of the PEHs were located beneath the visceral pleura in this case; therefore, it was easy to remove the tumors with minimal loss of the lung parenchyma. There was no major subjective respiratory difficulty post-surgery; however, it is obvious that surgery with mass reduction of the lung cannot be applied for PEH because it will greatly impact the patient’s outcome after surgery. A recent study showed the feasibility of combined chemotherapy [2], interferon [3] and molecular targeted therapy [4] for bilateral PEH. These therapies might hold promise for the treatment of multiple PEH; however, further clinical studies are needed. Bagan et al. [5] previously reviewed 80 PEH patients who underwent surgery. They found that surgery for PEH can be recommended for cases with unilateral single or multiple nodules, and that there is no single effective treatment for cases with bilateral multiple nodules. According to that review, surgery was not appropriate for our case with bilateral multiple lung nodules, but there was no other reliable treatment choice available at that time, and we therefore performed surgery after carefully considering all of the possible options. The natural history and clinical course of PEH remains poorly understood. The overall progression of this disease appears to be slow [6]. Some cases are associated with spontaneous regression, while others are more aggressive [7]. The major cause of death related to PEH is respiratory insufficiency due to an increase in size and number of pulmonary tumors [8]. According to previous reports, poor outcomes in patients with PEH are seen in those with respiratory symptoms, extensive lymphangitic spread or peripheral lymphadenopathy, pleural effusion, extensive intravascular, endobronchial, or interstitial tumor spread and hepatic metastases [2, 7, 8]; these findings were not present in this case of bilateral PEH. Based on the clinical findings, this case was categorized as benign PEH. It was

very difficult to predict when/if the benign PEH might show transformation; therefore, we considered surgery for this case. Miettinen et al. [9] described the case of a patient who survived for 24 years and underwent 11 different pulmonary resection procedures. In the present case, the patient’s course has been uneventful during the 11-year follow-up period. Thus, until more effective treatments become available, radical resection of pulmonary nodules might be indicated in some cases of PEH without infiltration, even in cases with bilateral involvement. Acknowledgments The authors thank Dr. Hitoshi Sugiura, the pathologist of Kawasaki Municipal Hospital, for providing us with the pathological data. Conflict of interest Keisuke Eguchi and Makoto Sawafuji have no conflicts of interest to declare in association with this study.

References 1. Endo T, Su CC, Numagami Y, Shirane R. Malignant intracranial epithelioid hemangioendothelioma presumably originating from the lung: case report. J Neurooncol. 2004;67:337–43. 2. Ye B, Li W, Feng J, Shi JX, Chen Y, Han BH. Treatment of pulmonary epithelioid hemangioendothelioma with combination chemotherapy: report of three cases and review of the literature. Oncol Lett. 2013;5:1491–6. 3. Roudier-Pujol C, Enjolras O, Lacronique J, et al. Multicentric epithelioid hemangioendothelioma in partial remission with interferon treatment. Ann Dermatol Venereol. 1994;121:898–904. 4. Chevreau C, Le Cesne A, Ray-Coquard I, Italiano A, Cioffi A, Isambert N, Robin YM, Fournier C, Clisant S, Chaigneau L, Bay JO, Bompas E, Gauthier E, Blay JY, Penel N. Sorafenib in patients with progressive epithelioid hemangioendothelioma: a phase 2 study by the French Sarcoma Group (GSF/GETO). Cancer. 2013;119(14):2639–44. 5. Bagan P, Hassan M, Barthes FLP, Peyrard S, Souilamas R, Danel C, Riquet M. Prognostic factors and surgical indications of pulmonary epithelioid hemangioendothelioma: a review of the literature. Ann Thorac Surg. 2006;82(6):2010–3. 6. Sweeney WB, Vesoulis Z, Blaum LC Jr. Intravascular bronchioloalveolar tumor: a distinctive surgical and pathological entity. Ann Thorac Surg. 1986;42:702–4. 7. Dail DH, Liebow AA, Gmelich JT, Friedman PJ, Miyai K, Myer W, et al. Intravascular, bronchiolar, and alveolar tumor of the lung (IVBAT): an analysis of twenty cases of a peculiar sclerosing endothelial tumor. Cancer. 1983;51:452–64. 8. Schattenberg T, Kam R, Klopp M, Herpel E, Schnabel PA, Mechtersheimer G, et al. Pulmonary epithelioid hemangioendothelioma: report of three cases. Surg Today. 2008;38:844–9. 9. Miettinen M, Collan Y, Halttunen P, Maamies T, Vilkko P. Intravascular bronchioloalveolar tumor. Cancer. 1987;60:2471–5.


Surgical management of a patient with bilateral multiple pulmonary epithelioid hemangioendothelioma: report of a case.

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare lung disease. This report describes the case of a 54-year-old female who underwent radical ...
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