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Weekly Chest CasesArchive of Old Cases

Case No : 1088 Date 2018-09-03

  • Courtesy of Joosung Sun, Kyung Joo Park MD. / Ajou University Medical Center
  • Age/Sex 36 / F
  • Chief ComplaintReferred due to incidentally identified multiple pulmonary nodules
  • Figure 1
  • Figure 2
  • Figure 3

Diagnosis With Brief Discussion

Diagnosis
Pulmonary epithelioid hemangioendothelioma
Radiologic Findings
Fig 1. Chest PA shows multiple pulmonary nodules and mild elevation of right hemidiaphragm.
Fig 2 Lung and mediastinal window (1.0-mm section thickness) images show multiple small well-defined nodules in both lungs. Some of the nodules show internal calcifications.
Fig 3. PET/CT images depict scanty FDG uptake in bilateral lung nodules.
Brief Review
Pulmonary epithelioid hemangioendothelioma (PEH) is a rare, low- to intermediate-grade tumor of endothelial origin with features of epithelioid and histiocytoid cells. There are three distinctive CT patterns:
1) Multiple pulmonary nodules (mimicking hematogenous metastases), with calcifications occasionally seen within the nodules
2) Multiple pulmonary reticulonodular opacities (mimicking hematolymphangitic metastases), which could be associated with interlobular septal thickening and ground-glass opacities and may show poor prognosis
3) Diffuse infiltrative pleural thickening (mimicking malignant pleural mesothelioma), which is seen less frequently and predominantly in older men and shows the most aggressive clinical course
The treatment of PEH is not standardized. Surgery alone is indicated in the presence of a single pulmonary nodule or unilateral multiple nodules. Lung transplantation should be considered in patients with vascular infiltration. Various chemotherapies have been reported for unrespectable or metastatic PEH, with variable effectiveness.
References
1. Kim EY, Kim TS, Han J, Choi JY, Kwon OJ, Kim J. Thoracic epithelioid hemangioendothelioma: imaging and pathologic features Acta Radiol. 2011 Mar 1;52(2):161-6. doi: 10.1258/ar.2010.100292.
2. 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(e4348)
3. Riccardo Cazzuffi, Nunzio Calia, Franco Ravenna, et al., “Primary Pulmonary Epithelioid Hemangioendothelioma: A Rare Cause of PET-Negative Pulmonary Nodules,” Case Reports in Medicine, vol. 2011, Article ID 262674, 6 pages, 2011. doi:10.1155/2011/262674
Keywords
Lung, Neoplasm,

No. of Applicants : 92

▶ Correct Answer : 15/92,  16.3%
  • - Higashi-Ohmi General Medical Center , Japan AKITOSHI INOUE
  • - Chonbuk National University Medical School , Korea (South) HYEJIN YANG
  • - Chonbuk National University Hospital , Korea (South) MINGI SHIN
  • - Other , Korea (South) SEONGSU KANG
  • - The Jikei university , Japan TAKU GOMI
  • - Onomichi municipal hospital , Japan Hirofumi Mifune
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - Medicheck health care , Korea (South) Chae hun Lim
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey MERIC TUZUN
  • - Chonbuk National University Hospital , Korea (South) KUM JU CHAE
  • - Teikyo University Hospital , Japan KAORU SUMIDA
  • - Kyoto Prefectural University of Medicine , Japan TADASHI TANAKA
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - Hamamatsu University Hospital , Japan YUKI HAYASHI
  • - Seoul Veterans Hospital , Korea (South) HYUN JUNG YOON
▶ Correct Answer as Differential Diagnosis : 6/92,  6.5%
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - Narayana Multispeciality Hospital Jaipur Rajasthan , India JAINENDRA JAIN
  • - University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
  • - Multimagem Diagn泥˜sticos , Brazil PEDRO PAULO TEIXEIRA E SILVA TORRES
  • - , Japan YUMI MAEHARA
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
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