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a tumor composed of immature undifferentiated cells (同)blastocytoma, embryonal carcinosarcoma

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/04/17 18:42:20」(JST)

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Pleuropulmonary blastoma (PPB) is a rare cancer originating in the lung or pleural cavity. It occurs most often in infants and young children^[1] but also has been reported in adults.^[2] In a retrospective review of 204 children with lung tumors, pleuropulmonary blastoma and carcinoid tumor were the most common primary tumors (83% of the 204 children had secondary tumors spread from cancers elsewhere in the body).^[1] Pleuropulmonary blastoma is regarded as malignant.

Signs and symptoms

Symptoms may include coughing, an upper respiratory tract infection, shortness of breath, and chest pain. These symptoms are very non-specific, and can be caused by other types of tumor in the lung or mediastinum more generally, and by other conditions. Imaging (X-ray, CT, MRI) may be used to determine the presence and precise location of a tumor, but not a specific diagnosis of PPB or other tumor.^[3] Doctors are unable to tell if a child has PPB right away, and not upper respiratory tract infection, until more test are taken and they show that there is no infection. Another symptom is pneumothorax.

Types

Pleuropulmonary blastoma is classified into 3 types:

Type I is multicystic
Type II shows thickening areas (nodules) within this cystic lesion
Type III shows solid masses.

Type I PPB is made up of mostly cysts, and may be hard to distinguish from benign lung cysts, and there is some evidence that not all type I PPB will progress to types II and III.^[4] Types II and III are aggressive, and cerebral metastasis is more frequent in PPB than in other childhood sarcomas.^[5]

Diagnosis

The most common way to test someone for PPB is to take a biopsy. Other tests like x-rays, CAT scans, and MRI's can suggest that cancer is present, but only an examination of a piece of the tumor can make a definite diagnosis.

A number of PPBs have shown trisomy 8 (17 out of 23 cases studied per the PPB registry). Trisomy 2 and p53 mutations/deletions have also been described.

Treatment

Treating PPB depends on the size and location of the tumor, whether the cancer has spread, and the child's overall health. Surgery is the main treatment for PPB. The main goal of surgery is to remove the tumor. If the tumor is too large to be completely removed, or if it's not possible to completely remove the tumor, surgery may be performed after chemotherapy. Because PPB can return after treatment, regular screening for possible recurrence should continue for 48 to 60 months, after diagnosis.

History

Pleuropulmonary blastoma was first described in 1988.^[6]

An international registry has been established.^[4]

To find out more information PPB Registry has the biggest information section on Pleuropulmonary Blastoma in the world.

References

^ ^a ^b Dishop MK, Kuruvilla S (July 2008). "Primary and metastatic lung tumors in the pediatric population: a review and 25-year experience at a large children's hospital". Arch. Pathol. Lab. Med. 132 (7): 1079–103. doi:10.1043/1543-2165(2008)132[1079:PAMLTI]2.0.CO;2. PMID 18605764.
^ Indolfi P, Casale F, Carli M et al. (September 2000). "Pleuropulmonary blastoma: management and prognosis of 11 cases". Cancer 89 (6): 1396–401. doi:10.1002/1097-0142(20000915)89:6<1396::AID-CNCR25>3.0.CO;2-2. PMID 11002236.
^ Cakir O, Topal U, Bayram AS, Tolunay S (March 2005). "Sarcomas: rare primary malignant tumors of the thorax". Diagn Interv Radiol 11 (1): 23–7. PMID 15795839.
^ ^a ^b Hill DA, Jarzembowski JA, Priest JR, Williams G, Schoettler P, Dehner LP (February 2008). "Type I pleuropulmonary blastoma: pathology and biology study of 51 cases from the international pleuropulmonary blastoma registry". Am. J. Surg. Pathol. 32 (2): 282–95. doi:10.1097/PAS.0b013e3181484165. PMID 18223332.
^ Priest JR, Magnuson J, Williams GM, Abromowitch M, Byrd R, Sprinz P, Finkelstein M, Moertel CL, Hill DA (September 2007). "Cerebral metastasis and other central nervous system complications of pleuropulmonary blastoma". Pediatr Blood Cancer 49 (3): 266–73. doi:10.1002/pbc.20937. PMID 16807914.
^ Manivel JC, Priest JR, Watterson J et al. (October 1988). "Pleuropulmonary blastoma. The so-called pulmonary blastoma of childhood". Cancer 62 (8): 1516–26. doi:10.1002/1097-0142(19881015)62:8<1516::AID-CNCR2820620812>3.0.CO;2-3. PMID 3048630.

External links

Pleuropulmonary Blastoma Bulletin Board
Pleuropulmonary Blastoma Family Support Group
Pleuropulmonary blastoma (images at humpath.com)

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English Journal

Adult biphasic pulmonary blastoma.

Jethava A, Dasanu CA.SourceDepartment of Hospital Medicine, Saint Francis Hospital and Medical Center, Hartford, USA.
Connecticut medicine.Conn Med.2013 Jan;77(1):19-22.
Pulmonary blastoma is a rare malignant tumor, histologically resembling the fetal lung. Since its first description in 1945, only about 200 cases have been reported worldwide. This tumor predominantly affects children, but has also been reported in adults with a peak incidence in the fourth decade o
PMID 23427368

[Pulmonary sarcomatoid carcinoma].

Vieira T, Duruisseaux M, Ruppert AM, Cadranel J, Antoine M, Wislez M.SourceHôpital Tenon, service de pneumologie et de réanimation, Paris, France.
Bulletin du cancer.Bull Cancer.2012 Oct;99(10):995-1001. doi: 10.1684/bdc.2012.1619.
Pulmonary sarcomatoid carcinomas are a rare group of tumors accounting for about 1 % of non-small cell lung carcinoma (NSCLC). In 2004, World Health Organization classification united under this name all the carcinomas with sarcomatous or sarcomatous-like component with spindle cell or giant cell a
PMID 22889810

Japanese Journal

Congenital Pulmonary Airway Malformation(CPAM)との鑑別が困難であったI型胸膜肺芽腫の1例

鈴木孝明,漆原直人,松岡尚則,福本弘二,福澤宏明,川島章子,渡邉健太郎,長谷川史郎,三間屋純一,堀越泰雄,高嶋能文,野村明孝,高場恵美,小山雅司
小児がん : 小児悪性腫瘍研究会記録 47(2), 293-297, 2010-05-25
胸膜肺芽腫は嚢胞性肺疾患との鑑別診断に苦慮する場合がある.今回われわれは,CPAMの術前診断で手術し,術後本症と診断された1歳2か月男児例を経験した.手術所見では肺外へ発育する巨大な多房性嚢胞を認め,嚢胞を含む肺葉部分切除を行った.病理組織検査でI型胸膜肺芽腫と診断され,化学療法を開始した.年少児の嚢胞性肺病変の診療に際しては,本症も念頭においた慎重な対応が必要である.
NAID 110007671765