Difference between revisions of "Pneumocytoma"

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'''Pneumocytoma''' is a rare [[lung tumour]] that is typically benign. It is also known as '''benign sclerosing pneumocytoma'''.<ref name=pmid6291188>{{Cite journal  | last1 = Chan | first1 = KW. | last2 = Gibbs | first2 = AR. | last3 = Lo | first3 = WS. | last4 = Newman | first4 = GR. | title = Benign sclerosing pneumocytoma of lung (sclerosing haemangioma). | journal = Thorax | volume = 37 | issue = 6 | pages = 404-12 | month = Jun | year = 1982 | doi =  | PMID = 6291188 }}</ref>
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Pneumocytoma - alt -- intermed mag.jpg
| Width      =
| Caption    = Pneumocytoma. [[H&E stain]]. (WC/Nephron)
| Synonyms  = sclerosing hemangioma, benign pneumocytoma
| Micro      = mixed cell population, variable architecture (papillary, sclerotic, solid, hemorrhagic), +/-granulomas
| Subtypes  =
| LMDDx      = [[carcinoid tumour]], papillary pattern [[lung adenocarcinoma]], metastatic [[papillary thyroid carcinoma]]
| Stains    =
| IHC        = TTF-1 +ve, Ki-67 +ve membranous pattern, PR +ve, CD56 -ve, CD34 -ve
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Staging    =
| Site      = lung - see ''[[lung tumours]]''
| Assdx      =
| Syndromes  =
| Clinicalhx = typical patient - female 40s
| Signs      =
| Symptoms  =
| Prevalence = rare <= 1% of lung tumours
| Bloodwork  =
| Rads      = slow growth/no growth, typically peripheral location
| Endoscopy  =
| Prognosis  = benign, case reports of mets
| Other      =
| ClinDDx    = other [[lung tumours]]
| Tx        = usually excision, may be followed
}}
'''Pneumocytoma''' is a rare [[lung tumour]] that is typically benign. It is also known as '''sclerosing pneumocytoma'''.<ref name=pmid6291188>{{Cite journal  | last1 = Chan | first1 = KW. | last2 = Gibbs | first2 = AR. | last3 = Lo | first3 = WS. | last4 = Newman | first4 = GR. | title = Benign sclerosing pneumocytoma of lung (sclerosing haemangioma). | journal = Thorax | volume = 37 | issue = 6 | pages = 404-12 | month = Jun | year = 1982 | doi =  | PMID = 6291188 }}</ref><ref name=pmid23380035/>


It was previously known as '''sclerosing hemangioma'''.
It was previously known as '''sclerosing hemangioma'''.<ref name=pmid23380035>{{Cite journal  | last1 = Ruiz de la Cuesta | first1 = D. | last2 = Lafont Rufat | first2 = M. | last3 = Ruiz de la Cuesta Martín | first3 = E. | title = Pneumocytoma (formerly known as sclerosing hemangioma of the lung): a rare cause of chest pain. | journal = Arch Bronconeumol | volume = 49 | issue = 6 | pages = 276-7 | month = Jun | year = 2013 | doi = 10.1016/j.arbres.2012.10.004 | PMID = 23380035 }}</ref>
==General==
==General==
*Derived from type 2 pneumocyte.<ref name=pmid15138814>{{Cite journal  | last1 = Yamazaki | first1 = K. | title = 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. | journal = Virchows Arch | volume = 445 | issue = 1 | pages = 45-53 | month = Jul | year = 2004 | doi = 10.1007/s00428-004-1023-3 | PMID = 15138814 }}</ref>
*Derived from type 2 pneumocyte.<ref name=pmid15138814>{{Cite journal  | last1 = Yamazaki | first1 = K. | title = 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. | journal = Virchows Arch | volume = 445 | issue = 1 | pages = 45-53 | month = Jul | year = 2004 | doi = 10.1007/s00428-004-1023-3 | PMID = 15138814 }}</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>
*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>
**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:
*Surgical excision preferred, may be followed.<ref name=pmid23838116/>


===Epidemiology===
===Epidemiology===
*Female in 40s.<ref name=pmid19415961>{{Cite journal  | last1 = Keylock | first1 = JB. | last2 = Galvin | first2 = JR. | last3 = Franks | first3 = TJ. | title = Sclerosing hemangioma of the lung. | journal = Arch Pathol Lab Med | volume = 133 | issue = 5 | pages = 820-5 | month = May | year = 2009 | doi =  | PMID = 19415961 }}</ref>
*Female in 40s.<ref name=pmid19415961>{{Cite journal  | last1 = Keylock | first1 = JB. | last2 = Galvin | first2 = JR. | last3 = Franks | first3 = TJ. | title = Sclerosing hemangioma of the lung. | journal = Arch Pathol Lab Med | volume = 133 | issue = 5 | pages = 820-5 | month = May | year = 2009 | doi =  | PMID = 19415961 }}</ref>
*Considered benign; excision is curative.
*Considered benign; excision is curative.
**Rare case reports of [[metastases]].
**Rare case reports of [[metastases]].<ref name=pmid27346414>{{Cite journal  | last1 = Pokharel | first1 = S. | last2 = Dhillon | first2 = SS. | last3 = Ylagan | first3 = L. | last4 = George | first4 = S. | last5 = Yendamuri | first5 = S. | title = Sclerosing Pneumocytoma with Lymph Node Metastasis. | journal = J Thorac Oncol | volume = 11 | issue = 10 | pages = 1802-4 | month = Oct | year = 2016 | doi = 10.1016/j.jtho.2016.06.005 | PMID = 27346414 }}</ref><ref name=pmid3009921>{{Cite journal  | last1 = Tanaka | first1 = I. | last2 = Inoue | first2 = M. | last3 = Matsui | first3 = Y. | last4 = Oritsu | first4 = S. | last5 = Akiyama | first5 = O. | last6 = Takemura | first6 = T. | last7 = Fujiwara | first7 = M. | last8 = Kodama | first8 = T. | last9 = Shimosato | first9 = Y. | title = A case of pneumocytoma (so-called sclerosing hemangioma) with lymph node metastasis. | journal = Jpn J Clin Oncol | volume = 16 | issue = 1 | pages = 77-86 | month = Mar | year = 1986 | doi =  | PMID = 3009921 }}</ref>


==Gross==
==Gross==
*Peripheral, solitary.
*Peripheral, solitary.
*Well-circumscribed.
*Well-circumscribed.
*Classically hemorrhagic.


==Microscopic==
==Microscopic==
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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===
-->
<gallery>
Image: Pneumocytoma -- low mag.jpg | Pneumocytoma - low mag.
Image: Pneumocytoma -- intermed mag.jpg | Pneumocytoma - intermed. mag.
Image: Pneumocytoma -- high mag.jpg | Pneumocytoma - high mag.
Image: Pneumocytoma -- very high mag.jpg | Pneumocytoma - very high mag.
 
Image: Pneumocytoma - alt -- low mag.jpg | Pneumocytoma - low mag.
Image: Pneumocytoma - alt -- intermed mag.jpg | Pneumocytoma - intermed. mag.
</gallery>


==IHC==
==IHC==
Features:<ref name=pmid10716159>{{Cite journal  | last1 = Rodriguez-Soto | first1 = J. | last2 = Colby | first2 = TV. | last3 = Rouse | first3 = RV. | title = A critical examination of the immunophenotype of pulmonary sclerosing hemangioma. | journal = Am J Surg Pathol | volume = 24 | issue = 3 | pages = 442-50 | month = Mar | year = 2000 | doi =  | PMID = 10716159 }}</ref>  
Features:<ref name=pmid10716159>{{Cite journal  | last1 = Rodriguez-Soto | first1 = J. | last2 = Colby | first2 = TV. | last3 = Rouse | first3 = RV. | title = A critical examination of the immunophenotype of pulmonary sclerosing hemangioma. | journal = Am J Surg Pathol | volume = 24 | issue = 3 | pages = 442-50 | month = Mar | year = 2000 | doi =  | PMID = 10716159 }}</ref>  
*EMA +ve.
*[[EMA]] +ve.
*PR +ve.<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>
*PR +ve.<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>
*Ki-67 membranous pattern.<ref name=pmid23030396>{{Cite journal  | last1 = Kim | first1 = BH. | last2 = Bae | first2 = YS. | last3 = Kim | first3 = SH. | last4 = Jeong | first4 = HJ. | last5 = Hong | first5 = SW. | last6 = Yoon | first6 = SO. | title = Usefulness of Ki-67 (MIB-1) immunostaining in the diagnosis of pulmonary sclerosing hemangiomas. | journal = APMIS | volume = 121 | issue = 2 | pages = 105-10 | month = Feb | year = 2013 | doi = 10.1111/j.1600-0463.2012.02945.x | PMID = 23030396 }}</ref>
*[[Ki-67]] membranous pattern.<ref name=pmid23030396>{{Cite journal  | last1 = Kim | first1 = BH. | last2 = Bae | first2 = YS. | last3 = Kim | first3 = SH. | last4 = Jeong | first4 = HJ. | last5 = Hong | first5 = SW. | last6 = Yoon | first6 = SO. | title = Usefulness of Ki-67 (MIB-1) immunostaining in the diagnosis of pulmonary sclerosing hemangiomas. | journal = APMIS | volume = 121 | issue = 2 | pages = 105-10 | month = Feb | year = 2013 | doi = 10.1111/j.1600-0463.2012.02945.x | PMID = 23030396 }}</ref>


Negative stains:<ref name=pmid10716159/>
Negative stains:<ref name=pmid10716159/>
Line 49: Line 98:
*HNF-3 alpha +ve.
*HNF-3 alpha +ve.
*HNF-3 beta +ve.
*HNF-3 beta +ve.
===Images===
<gallery>
Image: Pneumocytoma - TTF1 -- intermed mag.jpg | Pneumocytoma - TTF-1 - intermed. mag.
Image: Pneumocytoma - TTF1 -- high mag.jpg | Pneumocytoma - TTF-1 - high mag.
Image: Pneumocytoma - Ki67 -- intermed mag.jpg | Pneumocytoma - Ki-67 (membranous) - intermed. mag.
Image: Pneumocytoma - Ki67 -- high mag.jpg | Pneumocytoma - Ki-67 (membranous) - high mag.
</gallery>


==Sign out==
==Sign out==
Line 58: Line 115:
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===
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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