Difference between pages "SMARCA4-deficient non-small cell carcinoma of the lung" and "Thoracic SMARCA4-deficient undifferentiated tumour"

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'''SMARCA4-deficient non-small cell carcinoma of the lung''' is an emerging subtype of [[lung cancer]].<ref name=pmid28555282>{{Cite journal | last1 = Agaimy | first1 = A. | last2 = Fuchs | first2 = F. | last3 = Moskalev | first3 = EA. | last4 = Sirbu | first4 = H. | last5 = Hartmann | first5 = A. | last6 = Haller | first6 = F. | title = SMARCA4-deficient pulmonary adenocarcinoma: clinicopathological, immunohistochemical, and molecular characteristics of a novel aggressive neoplasm with a consistent TTF1 | journal = Virchows Arch | volume = 471 | issue = 5 | pages = 599-609 | month = Nov | year = 2017 | doi = 10.1007/s00428-017-2148-5 | PMID = 28555282 }}</ref>
'''Thoracic SMARCA4-deficient undifferentiated tumour''' is poorly differentiated malignancy of the thorax.<ref name=pmid38117955>{{cite journal |authors=Shinno Y, Ohe Y |title=Thoracic SMARCA4-deficient undifferentiated tumor: current knowledge and future perspectives |journal=Jpn J Clin Oncol |volume=54 |issue=3 |pages=265–270 |date=March 2024 |pmid=38117955 |doi=10.1093/jjco/hyad175 |url=}}</ref>


==General==
==General==
*~5.1% of non-small cell carcinoma is SMARCA4 deficient in a series of 316 cases.<ref name=pmid28038711>{{Cite journal | last1 = Herpel | first1 = E. | last2 = Rieker | first2 = RJ. | last3 = Dienemann | first3 = H. | last4 = Muley | first4 = T. | last5 = Meister | first5 = M. | last6 = Hartmann | first6 = A. | last7 = Warth | first7 = A. | last8 = Agaimy | first8 = A. | title = SMARCA4 and SMARCA2 deficiency in non-small cell lung cancer: immunohistochemical survey of 316 consecutive specimens. | journal = Ann Diagn Pathol | volume = 26 | issue = | pages = 47-51 | month = Feb | year = 2017 | doi = 10.1016/j.anndiagpath.2016.10.006 | PMID = 28038711 }}</ref>
*It was debated whether it is a poorly differentiated [[carcinoma]] or a [[sarcoma]].<ref name=pmid31751681>{{cite journal |authors=Rekhtman N, Montecalvo J, Chang JC, Alex D, Ptashkin RN, Ai N, Sauter JL, Kezlarian B, Jungbluth A, Desmeules P, Beras A, Bishop JA, Plodkowski AJ, Gounder MM, Schoenfeld AJ, Namakydoust A, Li BT, Rudin CM, Riely GJ, Jones DR, Ladanyi M, Travis WD |title=SMARCA4-Deficient Thoracic Sarcomatoid Tumors Represent Primarily Smoking-Related Undifferentiated Carcinomas Rather Than Primary Thoracic Sarcomas |journal=J Thorac Oncol |volume=15 |issue=2 |pages=231–247 |date=February 2020 |pmid=31751681 |pmc=7556987 |doi=10.1016/j.jtho.2019.10.023 |url=}}</ref>
*May represent ''or'' overlap with what is known as ''hepatoid adenocarcinoma of the lung''.{{fact}}
*WHO recognized entity as of 2021.<ref name=pmid38306886>{{cite journal |vauthors=Zhou P, Fu Y, Tang Y, Jiang L, Wang W |title=Thoracic SMARCA4-deficient undifferentiated tumor: A clinicopathological and prognostic analysis of 35 cases and immunotherapy efficacy |journal=Lung Cancer |volume=189 |issue= |pages=107471 |date=March 2024 |pmid=38306886 |doi=10.1016/j.lungcan.2024.107471 |url=}}</ref>
*The main differential diagnosis is ''metastatic adenocarcinoma''.
 
==Microscopic==
Features:
*Poorly differentiated malignancy.
*+/-[[Rhabdoid morphology]].


==IHC==
==IHC==
Features:<ref name=pmid28555282/>
*SMARCA4 (BRG1) loss (~75% of cases) ''or'' strongly reduced (~25% of cases) - '''definitional'''.
*CK7 +ve.
*AE1/AE3 -ve or focal.
*[[TTF-1]] -ve.
*Synaptophysin +ve.
*HepPar-1 +ve (15/20 +ve, 2/20 isolated +ve cells, 3/20 -ve).<ref name=pmid28555282/>
*Chromogranin -ve.
*SMARCA4 -ve.
*Napsin A -ve (1/28 +ve).<ref>{{cite journal |authors=Sayeda S, Naqvi A, Begum H, Finley C, Coschi C, Juergens R, Bonert M. |title=34th European Congress of Pathology - Abstracts (''Prevalence of TTF-1 negative lung adenocarcinoma on lung core biopsy with EGFR, ALK and PD-L1 status'') |journal=Virchows Arch |volume=481 |issue=Suppl 1 |pages=1–364 |date=August 2022 |pmid=35972647 |pmc=9379246 |doi=10.1007/s00428-022-03379-4 |url=}}</ref>
 
Note:
*Hepatoid [[adenocarcinoma of lung]] shows cytoplasmic [[TTF-1]] staining.<ref name=pmid24030743>{{Cite journal  | last1 = Haninger | first1 = DM. | last2 = Kloecker | first2 = GH. | last3 = Bousamra Ii | first3 = M. | last4 = Nowacki | first4 = MR. | last5 = Slone | first5 = SP. | title = Hepatoid adenocarcinoma of the lung: report of five cases and review of the literature. | journal = Mod Pathol | volume = 27 | issue = 4 | pages = 535-42 | month = Apr | year = 2014 | doi = 10.1038/modpathol.2013.170 | PMID = 24030743 }}</ref>
 
==Sign out==
<pre>
The tumour stains as follows:
POSITIVE: CK7 (diffuse, strong), HepPar-1 (strong, diffuse).
NEGATIVE: TTF-1, napsin A, p63, CK5/6.
 
The staining pattern for this tumour is not typical for lung adenocarcinoma; please correlate with the clinical findings.
 
HepPar-1 POSITIVITY with CK7 POSITIVITY and TTF-1 NEGATIVITY may be seen in SMARCA4-deficient adenocarcinoma of the lung.[1]
 
1. Virchows Arch 471 (5): 599-609. doi:10.1007/s00428-017-2148-5. PMID 28555282.
</pre>


==See also==
==See also==
*[[Lung adenocarcinoma]].
*[[SMARCA4]].
*[[SMARCB1]] (INI-1).
*[[SMARCA4-deficient non-small cell carcinoma of the lung]].


==References==
==References==
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[[Category:Diagnosis]]
[[Category:Diagnosis]]
[[Category:Lung tumours]]

Revision as of 20:12, 21 August 2025

Thoracic SMARCA4-deficient undifferentiated tumour is poorly differentiated malignancy of the thorax.[1]

General

  • It was debated whether it is a poorly differentiated carcinoma or a sarcoma.[2]
  • WHO recognized entity as of 2021.[3]

Microscopic

Features:

IHC

  • SMARCA4 (BRG1) loss (~75% of cases) or strongly reduced (~25% of cases) - definitional.
  • AE1/AE3 -ve or focal.
  • Synaptophysin +ve.
  • Chromogranin -ve.

See also

References

  1. ↑ Shinno Y, Ohe Y (March 2024). "Thoracic SMARCA4-deficient undifferentiated tumor: current knowledge and future perspectives". Jpn J Clin Oncol 54 (3): 265–270. doi:10.1093/jjco/hyad175. PMID 38117955.
  2. ↑ Rekhtman N, Montecalvo J, Chang JC, Alex D, Ptashkin RN, Ai N, Sauter JL, Kezlarian B, Jungbluth A, Desmeules P, Beras A, Bishop JA, Plodkowski AJ, Gounder MM, Schoenfeld AJ, Namakydoust A, Li BT, Rudin CM, Riely GJ, Jones DR, Ladanyi M, Travis WD (February 2020). "SMARCA4-Deficient Thoracic Sarcomatoid Tumors Represent Primarily Smoking-Related Undifferentiated Carcinomas Rather Than Primary Thoracic Sarcomas". J Thorac Oncol 15 (2): 231–247. doi:10.1016/j.jtho.2019.10.023. PMC 7556987. PMID 31751681. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7556987/.
  3. ↑ "Thoracic SMARCA4-deficient undifferentiated tumor: A clinicopathological and prognostic analysis of 35 cases and immunotherapy efficacy". Lung Cancer 189: 107471. March 2024. doi:10.1016/j.lungcan.2024.107471. PMID 38306886.
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