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Weekly Chest CasesArchive of Old Cases

Case No : 1094 Date 2018-10-15

  • Courtesy of Sang A Lee, Dong Jin Im, Hye-Jeong Lee / Severance Hospital
  • Age/Sex 59 / F
  • Chief ComplaintIncidental lung nodules detected on CT.
  • Figure 1
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Diagnosis With Brief Discussion

Diagnosis
Epithelioid hemangioendothelioma
Radiologic Findings
Non-enhanced chest CT images show innumerable and variable-sized (less than 1 cm) nodules in bilateral lungs. These nodules are well-defined, round, and non-calcified. There are no enlarged lymph nodes, pleural effusion, or other relevant pulmonary changes.
Enhanced abdominal CT images show multiple low-attenuating lesions in both hemilivers.
Upon 18F-FDG PET/CT examination, these pulmonary nodules show no remarkable accumulation of the tracer; however, a false negative result cannot be entirely excluded, due to the small size of the nodules. There is no lesion with abnormal increased FDG uptake in the scanned body.
The patient underwent VATS biopsy at the right lower lobe, and pulmonary nodules were confirmed as epithelioid hemangioendotheliomas.
Brief Review
Epithelioid hemangioendothelioma (EH) is a rare vascular tumor, originating from vascular endothelial or pre-endothelial cells. This low- to intermediate-grade sarcoma may involve the liver alone (21%), liver and lung (18%), lung alone (12%), or bone alone (14%), and can be aggressive, even resulting in systemic metastasis. Pulmonary EH are four times more common in women than in men and are often detected incidentally. The prognosis is variable, and the 5-year survival probability is 60% (range: 47-71%). There is no established standard treatment of choice; Surgery and/or chemoradiation therapy could be considered, depending on intrathoracic tumor spread and systemic metastases.
Multiple discrete pulmonary nodules are the most common CT finding of pulmonary EH. The nodules range in size up to 2 cm, but most are less than 1 cm in diameter. On serial CT examinations, these lesions show little or no growth. In this form of pulmonary EH, differential diagnosis includes pulmonary metastases, miliary granulomatous infection, sarcoidosis, silicosis, and primary lung malignancy. Other findings of pulmonary EH include multiple reticulonodular opacities mimicking hematolymphangitic metastasis and diffuse infiltrative pleural thickening mimicking malignant pleural mesothelioma or diffuse pleural carcinomatosis.
PET/CT findings can demonstrate increased FDG-uptake but usually are not a pivotal tool for the diagnosis, and a negative PET cannot exclude pulmonary EH. This false negative result is thought to be caused by the small size of the nodules, little FDG activity in the neoplasm, or low cellular density.
In our case, a close follow-up with chest CT was planned. The patient still has no symptoms and has survived for 33 months following the initial diagnosis. On the last chest CT, previous nodules are similar or have slightly increased in size without measurable change.
References
1. Angela Sardaro, Lilia Bardoscia, Maria Fonte Petruzzelli, Maurizio Portaluri : Epithelioid hemangioendothelioma : an overview and update on a rare vascular tumor. Oncology Review 2014; 8:82-91
2. Kenneth Lau, Malek Massad, Cynthia Pollak, Charles Rubin, Joannie Yeh, Jing Wang et al. Clinical patterns and outcome in epithelioid hemangioendothelioma with or without pulmonary involvement: insights from an internet registry in the study of a rare cancer. Chest 2011; 140:1312–1318
3. Eun Young Kim, Tae Sung Kim, Joungho Han, Joon Young Choi, O Jung Kwon and Jhingook Kim : Thoracic epithelioid hemangioendothelioma. Acta Radiologica 2011; 52: 161-166
4. Kyung Soo Lee, Joungho Han, Man Pyo Chung, Yeon Joo Jeong. Radiology Illustrated: Chest Radiology, Verlag Berlin Heidelberg: Springer, 2014:190
5. Jung Han Han, Tae Jung Kim, Kyung Soo Lee, Tae Sung Kim, Byung-Tae Kim : Epithelioid hemangioendothelioma in the thorax: Clinicopathologic, CT, PET, and prognostic features. Medicine 2016; 95: 30
Please refer to
Case 1088 Case 932 Case 772 Case 762 Case 691 Case 649
Keywords
Lung, Neoplasm,

No. of Applicants : 77

▶ Correct Answer : 37/77,  48.1%
  • - The University of Kyoto Hospital , Japan TOMOAKI OTANI
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
  • - Showa University Fujigaoka Hospital , Japan KYOKO NAGAI
  • - Higashi-Ohmi General Medical Center , Japan AKITOSHI INOUE
  • - The University of Tokyo Hospital , Japan Akifumi Hagiwara
  • - Vita Hospital , Brazil DIOGO LAGO PINHEIRO
  • - Other , Korea (South) SEONGSU KANG
  • - Oita University, Faculty of Medicine , Japan Fumito Okada
  • - Beaulieu clinic Geneva , Switzerland GILLES GENIN
  • - Onomichi municipal hospital , Japan Hirofumi Mifune
  • - Ajou University Hospital , Korea (South) Taeyang Ha
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - Asan Medical Center , Korea (South) JOOAE CHOE
  • - Gifu University Hospital , Japan Yo Kaneko
  • - The University of Tokyo Hospital , Japan RYO KUROKAWA
  • - Ajou University Hospital , Korea (South) DAYOUNG KIM
  • - , United States MAANSI P
  • - Mallinckrodt Institute of Radiology , United States Naganathan BS Mani
  • - Dae Jin Medical Center , Korea (South) JIYOUNG CHOI
  • - University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
  • - Avrasya Hospital/ISTANBUL , Turkey MURAT ULUSOY
  • - Ajou University Hospital , Korea (South) HYERIN KIM
  • - Azienda Ospedaliera di Cremona , Italy Pietro Sergio
  • - Korea University Guro Hospital , Korea (South) KYU-CHONG LEE
  • - Fortis hospital , Mohali , India SHALEEN RANA
  • - The First Affiliated Hospital of Nanjing Medical University , China TENG ZHANG
  • - Kizawa Memorial Hospital , Japan Shoji Okuda
  • - University of Tsukuba Hospital , Japan SODAI HOSHIAI
  • - Teikyo University Hospital , Japan KAORU SUMIDA
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - , Japan YUMI MAEHARA
  • - Hamamatsu University Hospital , Japan YUKI HAYASHI
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
  • - Seoul Veterans Hospital , Korea (South) HYUN JUNG YOON
  • - Ajou University Hospital , Korea (South) YOO YOUNGJIN
  • - Jiangsu province hospital , China WANGJIAN ZHA
▶ Correct Answer as Differential Diagnosis : 5/77,  6.5%
  • - Chonbuk National University Hospital , Korea (South) MINGI SHIN
  • - Chonbuk National University Hospital , Korea (South) EUN HA JUNG
  • - The Jikei university , Japan TAKU GOMI
  • - Kyoto Prefectural University of Medicine , Japan YOSHIAKI OTA
  • - Chonbuk National University Hospital , Korea (South) KUM JU CHAE
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Current Editor : Sang Young Oh, M.D., Ph.D Email : sangyoung.oh@gmail.com

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