Difference between revisions of "Pneumocytoma"

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| Micro      = mixed cell population, variable architecture (papillary, sclerotic, solid, hemorrhagic), +/-granulomas
| Micro      = mixed cell population, variable architecture (papillary, sclerotic, solid, hemorrhagic), +/-granulomas
| Subtypes  =
| Subtypes  =
| LMDDx      = [[carcinoid tumour]]
| LMDDx      = [[carcinoid tumour]], papillary pattern [[lung adenocarcinoma]], metastatic [[papillary thyroid carcinoma]]
| Stains    =
| Stains    =
| IHC        = TTF-1 +ve, Ki-67 +ve membranous pattern, PR +ve, CD56 -ve, CD34 -ve
| IHC        = TTF-1 +ve, Ki-67 +ve membranous pattern, PR +ve, CD56 -ve, CD34 -ve
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*Progesterone-receptor positive stromal cells.<ref name=pmid15731902>{{Cite journal | last1 = Einsfelder | first1 = BM. | last2 = Müller | first2 = KM. | title = ["Pneumocytoma" or "sclerosing hemangioma": histogenetic aspects of a rare tumor of the lung] | journal = Pathologe | volume = 26 | issue = 5 | pages = 367-77 | month = Sep | year = 2005 | doi = 10.1007/s00292-005-0751-8 | PMID = 15731902 }}</ref>
*Progesterone-receptor positive stromal cells.<ref name=pmid15731902>{{Cite journal | last1 = Einsfelder | first1 = BM. | last2 = Müller | first2 = KM. | title = ["Pneumocytoma" or "sclerosing hemangioma": histogenetic aspects of a rare tumor of the lung] | journal = Pathologe | volume = 26 | issue = 5 | pages = 367-77 | month = Sep | year = 2005 | doi = 10.1007/s00292-005-0751-8 | PMID = 15731902 }}</ref>
*Rare - 0.2% to 1% of lung tumours.<ref name=pmid23838116>{{Cite journal  | last1 = Salemis | first1 = NS. | last2 = Seretis | first2 = C. | last3 = Nakos | first3 = G. | last4 = Kantounakis | first4 = I. | last5 = Stoumpos | first5 = C. | last6 = Spiliopoulos | first6 = K. | title = Synchronous occurrence of breast cancer and pulmonary sclerosing hemangioma: management and review of the literature. | journal = Breast Dis | volume = 34 | issue = 2 | pages = 61-5 | month = Jan | year = 2013 | doi = 10.3233/BD-130352 | PMID = 23838116 }}</ref>
*Rare - 0.2% to 1% of lung tumours.<ref name=pmid23838116>{{Cite journal  | last1 = Salemis | first1 = NS. | last2 = Seretis | first2 = C. | last3 = Nakos | first3 = G. | last4 = Kantounakis | first4 = I. | last5 = Stoumpos | first5 = C. | last6 = Spiliopoulos | first6 = K. | title = Synchronous occurrence of breast cancer and pulmonary sclerosing hemangioma: management and review of the literature. | journal = Breast Dis | volume = 34 | issue = 2 | pages = 61-5 | month = Jan | year = 2013 | doi = 10.3233/BD-130352 | PMID = 23838116 }}</ref>
**One large series had 100 cases.<ref name=pmid10895813>{{Cite journal  | last1 = Devouassoux-Shisheboran | first1 = M. | last2 = Hayashi | first2 = T. | last3 = Linnoila | first3 = RI. | last4 = Koss | first4 = MN. | last5 = Travis | first5 = WD. | title = A clinicopathologic study of 100 cases of pulmonary sclerosing hemangioma with immunohistochemical studies: TTF-1 is expressed in both round and surface cells, suggesting an origin from primitive respiratory epithelium. | journal = Am J Surg Pathol | volume = 24 | issue = 7 | pages = 906-16 | month = Jul | year = 2000 | doi =  | PMID = 10895813 }}</ref>


Management:  
Management:  
Line 50: Line 51:
*Peripheral, solitary.
*Peripheral, solitary.
*Well-circumscribed.
*Well-circumscribed.
*Classically hemorrhagic.


==Microscopic==
==Microscopic==
Line 62: Line 64:


DDx:<ref>URL: [http://www.med.muni.cz/biomedjournal/pdf/2004/01/37_42.pdf http://www.med.muni.cz/biomedjournal/pdf/2004/01/37_42.pdf]. Accessed on: 17 June 2010.</ref>
DDx:<ref>URL: [http://www.med.muni.cz/biomedjournal/pdf/2004/01/37_42.pdf http://www.med.muni.cz/biomedjournal/pdf/2004/01/37_42.pdf]. Accessed on: 17 June 2010.</ref>
*Papillary adenoma.
*Papillary pattern:
*[[Neuroendocrine tumour]] (carcinoid).
**Papillary pattern [[lung adenocarcinoma]].
**Metastatic [[papillary thyroid carcinoma]].
*Solid pattern:
**[[Neuroendocrine tumour]] (carcinoid).


===Images===
===Images===
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The lesion stains as follows:
The lesion stains as follows:
POSITIVE: Ki-67 (membranous pattern), TTF-1, PR.
POSITIVE: Ki-67 (membranous pattern), TTF-1, PR.
NEGATIVE: CD56, p53.
NEGATIVE: CD56, p53, CD34.
</pre>
</pre>


===Micro===
===Micro===
The sections show lung with thickened alveolar walls containing bland appearing nuclei without conspicuous nucleoli. Necrosis is absent.
The sections show lung with thickened alveolar walls containing bland appearing cells with round/oval nuclei without conspicuous nucleoli. Necrosis is absent.


==See also==
==See also==

Latest revision as of 23:23, 17 March 2019

Pneumocytoma
Diagnosis in short

Pneumocytoma. H&E stain. (WC/Nephron)
Synonyms sclerosing hemangioma, benign pneumocytoma
LM mixed cell population, variable architecture (papillary, sclerotic, solid, hemorrhagic), +/-granulomas
LM DDx carcinoid tumour, papillary pattern lung adenocarcinoma, metastatic papillary thyroid carcinoma
IHC TTF-1 +ve, Ki-67 +ve membranous pattern, PR +ve, CD56 -ve, CD34 -ve
Site lung - see lung tumours
Clinical history typical patient - female 40s
Prevalence rare <= 1% of lung tumours
Radiology slow growth/no growth, typically peripheral location
Prognosis benign, case reports of mets
Clin. DDx other lung tumours
Treatment usually excision, may be followed

Pneumocytoma is a rare lung tumour that is typically benign. It is also known as sclerosing pneumocytoma.[1][2]

It was previously known as sclerosing hemangioma.[2]

General

  • Derived from type 2 pneumocyte.[3]
  • Progesterone-receptor positive stromal cells.[4]
  • Rare - 0.2% to 1% of lung tumours.[5]
    • One large series had 100 cases.[6]

Management:

  • Surgical excision preferred, may be followed.[5]

Epidemiology

  • Female in 40s.[7]
  • Considered benign; excision is curative.

Gross

  • Peripheral, solitary.
  • Well-circumscribed.
  • Classically hemorrhagic.

Microscopic

Features:[7]

  • Mixed cell population.
  • Variable architecture:
    • Papillary.
    • Sclerotic.
    • Solid.
    • Hemorrhagic.
  • +/-Granulomas.

DDx:[10]

Images

  • Pneumocytoma - low mag.

  • Pneumocytoma - intermed. mag.

  • Pneumocytoma - high mag.

  • Pneumocytoma - very high mag.

  • Pneumocytoma - low mag.

  • Pneumocytoma - intermed. mag.

IHC

Features:[11]

Negative stains:[11]

  • SMA -ve.
  • CEA -ve.
  • CD34 -ve.
  • S100 -ve.
  • Chromogranin A -ve.

Others:[3]

  • TTF-1 +ve.
  • HNF-3 alpha +ve.
  • HNF-3 beta +ve.

Images

  • Pneumocytoma - TTF-1 - intermed. mag.

  • Pneumocytoma - TTF-1 - high mag.

  • Pneumocytoma - Ki-67 (membranous) - intermed. mag.

  • Pneumocytoma - Ki-67 (membranous) - high mag.

Sign out

Lung, Left Lower Lobe, Core Biopsy:
     - Sclerosing pneumocytoma (sclerosing hemangioma).

Comment:
The lesion stains as follows:
POSITIVE: Ki-67 (membranous pattern), TTF-1, PR.
NEGATIVE: CD56, p53, CD34.

Micro

The sections show lung with thickened alveolar walls containing bland appearing cells with round/oval nuclei without conspicuous nucleoli. Necrosis is absent.

See also

References

  1. ↑ Chan, KW.; Gibbs, AR.; Lo, WS.; Newman, GR. (Jun 1982). "Benign sclerosing pneumocytoma of lung (sclerosing haemangioma).". Thorax 37 (6): 404-12. PMID 6291188.
  2. ↑ 2.0 2.1 Ruiz de la Cuesta, D.; Lafont Rufat, M.; Ruiz de la Cuesta Martín, E. (Jun 2013). "Pneumocytoma (formerly known as sclerosing hemangioma of the lung): a rare cause of chest pain.". Arch Bronconeumol 49 (6): 276-7. doi:10.1016/j.arbres.2012.10.004. PMID 23380035.
  3. ↑ 3.0 3.1 Yamazaki, K. (Jul 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. doi:10.1007/s00428-004-1023-3. PMID 15138814.
  4. ↑ 4.0 4.1 Einsfelder, BM.; Müller, KM. (Sep 2005). "["Pneumocytoma" or "sclerosing hemangioma": histogenetic aspects of a rare tumor of the lung]". Pathologe 26 (5): 367-77. doi:10.1007/s00292-005-0751-8. PMID 15731902.
  5. ↑ 5.0 5.1 Salemis, NS.; Seretis, C.; Nakos, G.; Kantounakis, I.; Stoumpos, C.; Spiliopoulos, K. (Jan 2013). "Synchronous occurrence of breast cancer and pulmonary sclerosing hemangioma: management and review of the literature.". Breast Dis 34 (2): 61-5. doi:10.3233/BD-130352. PMID 23838116.
  6. ↑ Devouassoux-Shisheboran, M.; Hayashi, T.; Linnoila, RI.; Koss, MN.; Travis, WD. (Jul 2000). "A clinicopathologic study of 100 cases of pulmonary sclerosing hemangioma with immunohistochemical studies: TTF-1 is expressed in both round and surface cells, suggesting an origin from primitive respiratory epithelium.". Am J Surg Pathol 24 (7): 906-16. PMID 10895813.
  7. ↑ 7.0 7.1 Keylock, JB.; Galvin, JR.; Franks, TJ. (May 2009). "Sclerosing hemangioma of the lung.". Arch Pathol Lab Med 133 (5): 820-5. PMID 19415961.
  8. ↑ Pokharel, S.; Dhillon, SS.; Ylagan, L.; George, S.; Yendamuri, S. (Oct 2016). "Sclerosing Pneumocytoma with Lymph Node Metastasis.". J Thorac Oncol 11 (10): 1802-4. doi:10.1016/j.jtho.2016.06.005. PMID 27346414.
  9. ↑ Tanaka, I.; Inoue, M.; Matsui, Y.; Oritsu, S.; Akiyama, O.; Takemura, T.; Fujiwara, M.; Kodama, T. et al. (Mar 1986). "A case of pneumocytoma (so-called sclerosing hemangioma) with lymph node metastasis.". Jpn J Clin Oncol 16 (1): 77-86. PMID 3009921.
  10. ↑ URL: http://www.med.muni.cz/biomedjournal/pdf/2004/01/37_42.pdf. Accessed on: 17 June 2010.
  11. ↑ 11.0 11.1 Rodriguez-Soto, J.; Colby, TV.; Rouse, RV. (Mar 2000). "A critical examination of the immunophenotype of pulmonary sclerosing hemangioma.". Am J Surg Pathol 24 (3): 442-50. PMID 10716159.
  12. ↑ Kim, BH.; Bae, YS.; Kim, SH.; Jeong, HJ.; Hong, SW.; Yoon, SO. (Feb 2013). "Usefulness of Ki-67 (MIB-1) immunostaining in the diagnosis of pulmonary sclerosing hemangiomas.". APMIS 121 (2): 105-10. doi:10.1111/j.1600-0463.2012.02945.x. PMID 23030396.
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