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'''Pneumocytoma''' is a rare | {{ 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 | *Papillary pattern: | ||
*[[Neuroendocrine tumour]] (carcinoid). | **Papillary pattern [[lung adenocarcinoma]]. | ||
**Metastatic [[papillary thyroid carcinoma]]. | |||
*Solid pattern: | |||
**[[Neuroendocrine tumour]] (carcinoid). | |||
Image: | ===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> | |||
Negative stains:<ref name=pmid10716159/> | Negative stains:<ref name=pmid10716159/> | ||
| Line 48: | 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== | |||
<pre> | |||
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. | |||
</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== | ||