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Rare syndromes


benign tumourlungchest x-rayBronchoscopythoracotomybiopsymillimetersCalcificationlymph nodespneumonocyte






Pulmonary sclerosing pneumocytoma
Pneumocytoma -- high mag.jpg
Micrograph showing a pneumocytoma( Sclerosing pneumocytoma)

Pulmonary sclerosing pneumocytoma is a rare benign tumour of the lung.




Contents





  • 1 Clinical


  • 2 Histology


  • 3 Diagnosis


  • 4 Treatment


  • 5 Epidemiology


  • 6 History


  • 7 References




Clinical


The most common presentation is as an incidental lesion noticed on a chest x-ray.[1] These lesions are rarely symptomatic.


Blood tests are normal. CT shows a solitary nodule with may have some calcium present. PET scans may be positive.[2][3]


Bronchoscopy is not usually useful as these lesions tend to lie in the perifery of the lung. Fine needle aspirates may be not be sufficient to make the diagnosis.[4][5]


Diagnosis is normally made by thoracotomy and biopsy.[6][7] The regional lymph nodes are normally removed as these may show evidence of spread.


If the diagnosis is made preoperatively radiation treatment may be an option.[8]


The prognosis is excellent with no evidence of recurrence. Long term follow up is recommended.



Histology


These are solitary lesions lying within the lung varying in size between 10 and 80 millimeters in diameter.


There are four elements in this tumour: solid, papillary, sclerotic and hemangiomatous. These are present in variable proportions depending on the lesion. Calcification occurs in 40%.


Although considered benign spread to regional lymph nodes occurs in <5%.[9][10]


The underlying causative cell type seems to be a type II pneumonocyte.[11]



Diagnosis



Treatment



Epidemiology


This tumour is rare - 1% cases of benign lung tumours. It is most common in Asian women and usually presents in the fifth decade of life. The male:female ratio is 1:5.



History


This lesion was first described in 1956 by Liebow and Hubbell.[12]



References




  1. ^ Baysak A, Oz AT, Moğulkoç N, Bishop PW, Ceylan KC (2013) A rare tumor of the lung: pulmonary sclerosing hemangioma (pneumocytoma). Respir Med 107(3):448-50. doi: 10.1016/j.rmed.2012.12.005


  2. ^ Hara K, Izumi N, Tsukioka T, Komatsu H, Okada S, Toda M, Ito R, Shibata T, Nishiyama N (2016) Multiple pulmonary sclerosing pneumocytoma with abnormal accumulation of fluorodeoxyglucose-positron emission tomography diagnosed by durgical treatment; Report of a case. Kyobu Geka 69(13):1123-1126


  3. ^ Shin SY, Kim MY, Oh SY, Lee HJ, Hong SA, Jang SJ, Kim SS (2015) Pulmonary sclerosing pneumocytoma of the lung: CT characteristics in a large series of a tertiary referral center. Medicine (Baltimore) 94(4):e498. doi: 10.1097/MD.0000000000000498.


  4. ^ Hissong E, Rao R (2017) Pneumocytoma (sclerosing hemangioma), a potential pitfall. Diagn Cytopathol 45(8):744-749. doi: 10.1002/dc.23720


  5. ^ Zeng J, Zhou F, Wei XJ, Kovacs S, Simsir A, Shi Y (2016) Sclerosing hemangioma: A diagnostic dilemma in fine needle aspiration cytology. Cytojournal 13:9. doi: 10.4103/1742-6413.180783.


  6. ^ Zhu J (2017) Analysis of the clinical differentiation of pulmonary sclerosing pneumocytoma and lung cancer. J Thorac Dis 9(9):2974-2981. doi: 10.21037/jtd.2017.08.07


  7. ^ Yang CH, Lee LY (2017) Pulmonary sclerosing pneumocytoma remains a diagnostic challenge using frozen sections: a clinicopathological analysis of 59 cases. Histopathology doi: 10.1111/his.13391


  8. ^ Fayers RW, Lim TS, Havlat MF (2016) Pulmonary sclerosing pneumocytoma (sclerosing haemangioma): Radical radiation therapy. J Med Imaging Radiat Oncol 60(5):693-695. doi: 10.1111/1754-9485.12477


  9. ^ Soo IX, Sittampalam K, Lim CH (2017) Pulmonary sclerosing pneumocytoma with mediastinal lymph node metastasis. Asian Cardiovasc Thorac Ann 25(7-8):547-549. doi: 10.1177/0218492317727668


  10. ^ Tanaka I, Inoue M, Matsui Y, Oritsu S, Akiyama O, Takemura T, Fujiwara M, Kodama T, Shimosato Y (1986) A case of pneumocytoma (so-called sclerosing hemangioma) with lymph node metastasis. Jpn J Clin Oncol 16(1):77-86


  11. ^ Yamazaki K (2004) Type-II pneumocyte differentiation in pulmonary sclerosing hemangioma: ultrastructural differentiation and immunohistochemical distribution of lineage-specific transcription factors (TTF-1, HNF-3 alpha, and HNF-3 beta) and surfactant proteins. Virchows Arch 445(1):45-53


  12. ^ Liebow AA, Hubbell DS (1956) Sclerosing hemangioma (histiocytoma, xanthoma) of the lung. Cancer 9 (1956) 53-75









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