대한흉부영상의학회 Korean Society of Thoracic Radiology

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Weekly Chest CasesCases by Disease Category

Case No : 1303 Date 2022-10-11

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  • Courtesy of Sohee Park, Eun Young Kim, Jooae Choe, Kyung-Hyun Do / Asan medical center
  • Age/Sex 59 / F
  • Chief ComplaintAbnormal findings detected on chest radiograph for a regular check-up. History: none
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5
  • Figure 6

Diagnosis With Brief Discussion

Diagnosis
Pleuroparenchymal blastoma
Radiologic Findings
Fig 1. Chest PA shows a large thin-walled cyst in left lower lung zone.
Fig 2-5. Axial and coronal CT scans reveal a 5.9-cm air-filled cyst with a well-defined thin wall surrounded by normal lung parenchyma and an engorged pulmonary vein is observed along the cyst wall.
Fig 6. Compared with the CT scans performed 1 year ago, the cystic lesion shows interval growth.
The patient underwent wedge resection and the pathologic examination confirmed the presence of pleuroparenchymal blastoma, type 1, regressed type.
Brief Review
Pleuropulmonary blastomas (PPB) are a rare, variably aggressive, childhood primary intrathoracic malignancy. In up to 25% of cases, the mass can be extrapulmonary with attachment to the parietal pleura. They are classified under sarcomatoid carcinomas of the lungs. Pleuropulmonary blastoma is encountered in childhood, mostly in the first years of life (90% in those between 0-2 years old). Pleuropulmonary blastoma is considered a form of pulmonary blastoma. It comprises both mesenchymal and epithelial components resembling fetal lung. Bilateral occurrence is extremely rare.

This classification is a continuum from the less malignant to the most malignant lesion:

type 1: lesions manifest as a single cyst or a multicystic lesion (often air-filled)
type 2: lesions show air- or fluid-filled cavities with possible air-fluid levels along with solid internal nodules
type 3: neoplasms are solid lesions that show low attenuation at CT and homogeneous or heterogeneous enhancement

In 2008, those nonprogressed/regressed cystic cases were described as type I regressed (PPB Ir) which were purely cystic tumors that lack a primitive cell component and signifies regression or nonprogression. It is characterized by the same cystic structure of type I, but the septa completely lack primitive cells and/or rhabdomyoblasts likely secondary to regressive changes. The age range for type Ir at diagnosis is broad ranging from infancy to adulthood (median diagnosis ages, 7
References
1. Naffaa L & Donnelly L. Imaging Findings in Pleuropulmonary Blastoma. Pediatr Radiol. 2005;35(4):387-91. doi:10.1007/s00247-004-1367-5
2. Walker R, Suvarna K, Matthews S. Case Report: Pulmonary Blastoma: Presentation of Two Atypical Cases and Review of the Literature. Br J Radiol. 2005;78(929):437-40. doi:10.1259/bjr/45172814
3. Dobard G, Hedlund G, Johnson W, Kelly D, Howard T. Pleuropulmonary Blastoma with Vascular Invasion. AJR Am J Roentgenol. 1996;166(2):308. doi:10.2214/ajr.166.2.8553935
4. Messinger YH, Stewart DR, Priest JR, Williams GM, Harris AK, Schultz KA, Yang J, Doros L, Rosenberg PS, Hill DA, Dehner LP. Pleuropulmonary blastoma: a report on 350 central pathology-confirmed pleuropulmonary blastoma cases by the International Pleuropulmonary Blastoma Registry. Cancer. 2015;121(2):276-85. doi: 10.1002/cncr.29032.
Keywords
Pleuroparenchymal blastoma,

No. of Applicants : 90

▶ Semi-Correct Answer : 30/90,  33.3%
  • - Jichi Medical University Hospital , Japan JUN KANZAWA
  • - ZIGONG TCM HOSPITAL OF CHINA , China CAO CUN YOU
  • - Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
  • - Ajou University Hospital , Korea (South) SUNG HYUN AN
  • - Affilitated Hospital of Jining Medical college , China JIANG SHENG HUA
  • - National Center of Neurology and Psychiatry , Japan MOTO NAKAYA
  • - , Italy PAOLO BALDASSARI
  • - Other , Korea (South) SEONGSU KANG
  • - Osaka University , Japan AKINORI HATA
  • - Ajou University Hospital , Korea (South) HAEIN LEE
  • - Shimada General Medical Center , Japan HAYATO NOZAWA
  • - Other , Korea (South) HYEON IL CHOI
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - , Japan KENTARO KOTANI
  • - Kyoto University Hospital , Japan YASUHISA KURATA
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey MERIC TUZUN
  • - Avrasya Hospital/ISTANBUL , Turkey MURAT ULUSOY
  • - Osaka metropolitan university Hospital , Japan TATSUSHI OURA
  • - Other , Korea (South) SEONG WON JANG
  • - SRMC , India PRABHU RADHAN RADHAKRISHNAN
  • - Manipal hospital Hebbal ,Bengaluru , India PRAVIN KUMAR M
  • - , Korea (South) KIM HYE IN
  • - , Korea (South) SEUNG HOON OH
  • - Chonbuk National University Hospital , Korea (South) MINJEE KIM
  • - Toranomon Hospital , Japan KAORU SUMIDA
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - The University of Tokyo Hospital , Japan HIROSHI TAKUMIDA
  • - Shiga University of Medical Science , Japan RYO UEMURA
  • - , Japan YUKI HAYASHI
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
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