Pneumocytoma

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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.

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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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