Difference between revisions of "Multifocal micronodular pneumocyte hyperplasia associated with tuberous sclerosis"

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{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Multifocal micronodular pneumocyte hyperplasia - tuberous sclerosis - a3 -- high mag.jpg
| Width      =
| Caption    = Micronodule of pneumocyte hyperplasia in multifocal micronodular pneumocyte hyperplasia associated with tuberous sclerosis. [[H&E stain]].
| Synonyms  =
| Micro      =
| Subtypes  =
| LMDDx      = [[atypical adenomatous hyperplasia of the lung]]
| Stains    =
| IHC        =
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Staging    =
| Site      = [[lung]]
| Assdx      = [[lymphangioleiomyomatosis]] - also assoc. with tuberous sclerosis
| Syndromes  = [[tuberous sclerosis]]
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence = rare
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = benign
| Other      =
| ClinDDx    =
| Tx        =
}}
'''Multifocal micronodular pneumocyte hyperplasia associated with tuberous sclerosis''', also '''multifocal micronodular pneumocyte hyperplasia in tuberous sclerosis''', is the presence of rare [[hamartoma]]tous lesions in the [[lung]] seen in [[tuberous sclerosis]].<ref name=pmid18234663 >{{Cite journal  | last1 = Nagar | first1 = AM. | last2 = Teh | first2 = HS. | last3 = Khoo | first3 = RN. | last4 = Morani | first4 = AC. | last5 = Vrishni | first5 = K. | last6 = Raghuram | first6 = J. | title = Multifocal pneumocyte hyperplasia in tuberous sclerosis. | journal = Thorax | volume = 63 | issue = 2 | pages = 186 | month = Feb | year = 2008 | doi = 10.1136/thx.2006.076604 | PMID = 18234663 }}</ref>
'''Multifocal micronodular pneumocyte hyperplasia associated with tuberous sclerosis''', also '''multifocal micronodular pneumocyte hyperplasia in tuberous sclerosis''', is the presence of rare [[hamartoma]]tous lesions in the [[lung]] seen in [[tuberous sclerosis]].<ref name=pmid18234663 >{{Cite journal  | last1 = Nagar | first1 = AM. | last2 = Teh | first2 = HS. | last3 = Khoo | first3 = RN. | last4 = Morani | first4 = AC. | last5 = Vrishni | first5 = K. | last6 = Raghuram | first6 = J. | title = Multifocal pneumocyte hyperplasia in tuberous sclerosis. | journal = Thorax | volume = 63 | issue = 2 | pages = 186 | month = Feb | year = 2008 | doi = 10.1136/thx.2006.076604 | PMID = 18234663 }}</ref>


Revision as of 02:42, 8 March 2016

Multifocal micronodular pneumocyte hyperplasia associated with tuberous sclerosis
Diagnosis in short

Micronodule of pneumocyte hyperplasia in multifocal micronodular pneumocyte hyperplasia associated with tuberous sclerosis. H&E stain.
LM DDx atypical adenomatous hyperplasia of the lung
Site lung
Associated Dx lymphangioleiomyomatosis - also assoc. with tuberous sclerosis
Syndromes tuberous sclerosis
Prevalence rare
Prognosis benign

Multifocal micronodular pneumocyte hyperplasia associated with tuberous sclerosis, also multifocal micronodular pneumocyte hyperplasia in tuberous sclerosis, is the presence of rare hamartomatous lesions in the lung seen in tuberous sclerosis.[1]

General

  • Rare.

Gross

Features:[2]

  • Multiple small lung nodules - apparently random distribution.

Microscopic

Features:

  • Macrophages within the air spaces.
  • Enlarged alveolar lining cells with:
    • Hobnail morphology - free (luminal) surface area > attached/basal surface area.
    • Round or oval nuclei.

DDx:

Images

  • MMPH - intermed. mag.

  • MMPH - high mag.

See also

References

  1. ↑ Nagar, AM.; Teh, HS.; Khoo, RN.; Morani, AC.; Vrishni, K.; Raghuram, J. (Feb 2008). "Multifocal pneumocyte hyperplasia in tuberous sclerosis.". Thorax 63 (2): 186. doi:10.1136/thx.2006.076604. PMID 18234663.
  2. ↑ Kobashi, Y.; Sugiu, T.; Mouri, K.; Irei, T.; Nakata, M.; Oka, M. (Jun 2008). "Multifocal micronodular pneumocyte hyperplasia associated with tuberous sclerosis: differentiation from multiple atypical adenomatous hyperplasia.". Jpn J Clin Oncol 38 (6): 451-4. doi:10.1093/jjco/hyn042. PMID 18535095.
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