Difference between revisions of "ALK-rearranged renal cell carcinoma"

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| Tx        = may respond to ALK inhibitors
| Tx        = may respond to ALK inhibitors
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'''ALK translocation renal cell carcinoma''', also '''ALK-rearranged renal cell carcinoma''' and '''ALK-positive renal cell carcinoma''', is a type of [[renal cell carcinoma]] recognized by the WHO.
'''ALK-rearranged renal cell carcinoma''', also '''ALK translocation renal cell carcinoma''' and '''ALK-positive renal cell carcinoma''', is a type of [[renal cell carcinoma]] recognized by the WHO.


==General==
It may be abbreviated ''ALK-RCC''.
*Extremely rare.
**One case was identified in 829 consecutively screened Korean patients.<ref name=pmid24255633>{{Cite journal  | last1 = Lee | first1 = C. | last2 = Park | first2 = JW. | last3 = Suh | first3 = JH. | last4 = Nam | first4 = KH. | last5 = Moon | first5 = KC. | title = ALK-Positive Renal Cell Carcinoma in a Large Series of Consecutively Resected Korean Renal Cell Carcinoma Patients. | journal = Korean J Pathol | volume = 47 | issue = 5 | pages = 452-7 | month = Oct | year = 2013 | doi = 10.4132/KoreanJPathol.2013.47.5.452 | PMID = 24255633 }}</ref>
**A second series described 8 in 276 cases on a microarray.<ref name=pmid38039739>{{cite journal |authors=Doğan K, Onder E |title=ALK-rearranged renal cell carcinoma (ALK-RCC): Evaluation of histomorphological and immunohistochemical features by analysis of 276 renal cell carcinoma cases in Turkey |journal=Pathol Res Pract |volume=253 |issue= |pages=154951 |date=January 2024 |pmid=38039739 |doi=10.1016/j.prp.2023.154951 |url=}}</ref>
**In 2016, less than 17 cases reported.<ref name=pmid27554841>{{Cite journal  | last1 = Jeanneau | first1 = M. | last2 = Gregoire | first2 = V. | last3 = Desplechain | first3 = C. | last4 = Escande | first4 = F. | last5 = Tica | first5 = DP. | last6 = Aubert | first6 = S. | last7 = Leroy | first7 = X. | title = ALK rearrangements-associated renal cell carcinoma (RCC) with unique pathological features in an adult. | journal = Pathol Res Pract | volume = 212 | issue = 11 | pages = 1064-1066 | month = Nov | year = 2016 | doi = 10.1016/j.prp.2016.07.015 | PMID = 27554841 }}</ref>
*May respond to [[ALK inhibitors]] such as alectinib<ref name=pmid29685646>{{Cite journal  | last1 = Pal | first1 = SK. | last2 = Bergerot | first2 = P. | last3 = Dizman | first3 = N. | last4 = Bergerot | first4 = C. | last5 = Adashek | first5 = J. | last6 = Madison | first6 = R. | last7 = Chung | first7 = JH. | last8 = Ali | first8 = SM. | last9 = Jones | first9 = JO. | title = Responses to Alectinib in ALK-rearranged Papillary Renal Cell Carcinoma. | journal = Eur Urol | volume = 74 | issue = 1 | pages = 124-128 | month = 07 | year = 2018 | doi = 10.1016/j.eururo.2018.03.032 | PMID = 29685646 }}</ref> or crizotinib.<ref name=pmid27554841>{{Cite journal  | last1 = Jeanneau | first1 = M. | last2 = Gregoire | first2 = V. | last3 = Desplechain | first3 = C. | last4 = Escande | first4 = F. | last5 = Tica | first5 = DP. | last6 = Aubert | first6 = S. | last7 = Leroy | first7 = X. | title = ALK rearrangements-associated renal cell carcinoma (RCC) with unique pathological features in an adult. | journal = Pathol Res Pract | volume = 212 | issue = 11 | pages = 1064-1066 | month = Nov | year = 2016 | doi = 10.1016/j.prp.2016.07.015 | PMID = 27554841 }}</ref>
 
==Microscopic==
Features:
*Mix of morphologies - including:
**[[Papillary renal cell carcinoma]].<ref name=pmid24255633/>
**[[Renal cell carcinoma with rhabdoid morphology|Rhabdoid change]].<ref name=pmid26017874>{{Cite journal  | last1 = Kuroda | first1 = N. | last2 = Karashima | first2 = T. | last3 = Inoue | first3 = K. | last4 = Kasajima | first4 = A. | last5 = Ohe | first5 = C. | last6 = Kawakami | first6 = F. | last7 = Mikami | first7 = S. | last8 = Matsuura | first8 = K. | last9 = Moriyama | first9 = M. | title = Review of renal cell carcinoma with rhabdoid features with focus on clinical and pathobiological aspects. | journal = Pol J Pathol | volume = 66 | issue = 1 | pages = 3-8 | month = Mar | year = 2015 | doi =  | PMID = 26017874 }}</ref>
**[[Renal cell carcinoma with sarcomatoid differentiation|Sarcomatoid change]].
**"[[Unclassified renal cell carcinoma]]".
 
DDx:
*[[Papillary renal cell carcinoma]] - especially what used to be known as "type 2".<ref name=pmid24255633/>
*[[Unclassified renal cell carcinoma]]
*[[Renal medullary carcinoma]].
*[[Clear cell renal cell carcinoma]].<ref name=pmid38039739/>
*[[Renal mucinous tubular and spindle cell carcinoma]].<ref>{{cite journal |authors=Kai K, Tobu S, Kido S, Mikami S, Takeuchi K, Dobashi A, Togashi Y, Noguchi M, Aishima S |title=ALK rearrangement-associated renal cell carcinoma morphologically mimicking mucinous tubular and spindle cell carcinoma: a case report |journal=Diagn Pathol |volume=17 |issue=1 |pages=52 |date=June 2022 |pmid=35718773 |pmc=9206751 |doi=10.1186/s13000-022-01238-z |url=}}</ref>
 
==IHC==
Features:<ref>{{Cite journal  | last1 = Jeanneau | first1 = M. | last2 = Gregoire | first2 = V. | last3 = Desplechain | first3 = C. | last4 = Escande | first4 = F. | last5 = Tica | first5 = DP. | last6 = Aubert | first6 = S. | last7 = Leroy | first7 = X. | title = ALK rearrangements-associated renal cell carcinoma (RCC) with unique pathological features in an adult. | journal = Pathol Res Pract | volume = 212 | issue = 11 | pages = 1064-1066 | month = Nov | year = 2016 | doi = 10.1016/j.prp.2016.07.015 | PMID = 27554841 }}</ref>
*L-ALK +ve. ‡
*PAX8 +ve.
*Vimentin +ve (diffuse).
*CK7 +ve (focal).
*[[SMARCB1]] normal nuclear staining.{{fact}}
 
‡ There are different clones for ALK; they may be called ''ALK1'' (used in lymphoma) and ''L-ALK'' (used in lung cancer).  In the context of RCC, the ALK clone for lung cancer (e.g. D5F3) should be used.<ref>{{cite journal |authors=Kai K, Tobu S, Kido S, Mikami S, Takeuchi K, Dobashi A, Togashi Y, Noguchi M, Aishima S |title=ALK rearrangement-associated renal cell carcinoma morphologically mimicking mucinous tubular and spindle cell carcinoma: a case report |journal=Diagn Pathol |volume=17 |issue=1 |pages=52 |date=June 2022 |pmid=35718773 |pmc=9206751 |doi=10.1186/s13000-022-01238-z |url=}}</ref>
 
==Molecular==
*ALK rearrangement.
 
==See also==
*[[Vancouver classification]].
*[[Kidney tumours]].
*[[Lung carcinoma with ALK rearrangement]].
 
==References==
{{Reflist|2}}
 
[[Category:Kidney tumours]]
[[Category:Diagnosis]]
 
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{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      =
| Width      =
| Caption    =
| Synonyms  =
| Micro      = mixed morphology, +/-papillae, +/-myxoid change
| Subtypes  =
| LMDDx      = [[papillary renal cell carcinoma]], [[unclassified renal cell carcinoma]], [[clear cell renal cell carcinoma]], [[SMARCB1-deficient renal medullary carcinoma]], [[renal mucinous tubular and spindle cell carcinoma]]
| Stains    =
| IHC        = ALK +ve
| EM        =
| Molecular  = ALK rearranagement
| IF        =
| Gross      =
| Grossing  = [[total nephrectomy for tumour grossing]], [[partial nephrectomy grossing]]
| Staging    = [[kidney cancer staging]]
| Site      = [[kidney]] - see ''[[kidney tumours]]''
| Assdx      =
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence = very rare
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  =
| Other      =
| ClinDDx    =
| Tx        = may respond to ALK inhibitors
}}
'''ALK-rearranged renal cell carcinoma''', also '''ALK translocation renal cell carcinoma''' and '''ALK-positive renal cell carcinoma''', is a type of [[renal cell carcinoma]] recognized by the World Health Organization (WHO).


==General==
==General==

Latest revision as of 21:50, 26 September 2025

ALK-rearranged renal cell carcinoma
Diagnosis in short
LM mixed morphology, +/-papillae, +/-myxoid change
LM DDx papillary renal cell carcinoma, unclassified renal cell carcinoma, clear cell renal cell carcinoma, SMARCB1-deficient renal medullary carcinoma, renal mucinous tubular and spindle cell carcinoma
IHC ALK +ve
Molecular ALK rearranagement
Grossing notes total nephrectomy for tumour grossing, partial nephrectomy grossing
Staging kidney cancer staging
Site kidney - see kidney tumours
Prevalence very rare
Treatment may respond to ALK inhibitors

ALK-rearranged renal cell carcinoma, also ALK translocation renal cell carcinoma and ALK-positive renal cell carcinoma, is a type of renal cell carcinoma recognized by the WHO.

It may be abbreviated ALK-RCC.

General

  • Extremely rare.
    • One case was identified in 829 consecutively screened Korean patients.[1]
    • A second series described 8 in 276 cases on a microarray.[2]
    • In 2016, less than 17 cases reported.[3]
  • May respond to ALK inhibitors such as alectinib[4] or crizotinib.[3]

Microscopic

Features:

DDx:

IHC

Features:[7]

  • L-ALK +ve. ‡
  • PAX8 +ve.
  • Vimentin +ve (diffuse).
  • CK7 +ve (focal).
  • SMARCB1 normal nuclear staining.[citation needed]

‡ There are different clones for ALK; they may be called ALK1 (used in lymphoma) and L-ALK (used in lung cancer). In the context of RCC, the ALK clone for lung cancer (e.g. D5F3) should be used.[8]

Molecular

  • ALK rearrangement.

See also

References

  1. ↑ 1.0 1.1 1.2 Lee, C.; Park, JW.; Suh, JH.; Nam, KH.; Moon, KC. (Oct 2013). "ALK-Positive Renal Cell Carcinoma in a Large Series of Consecutively Resected Korean Renal Cell Carcinoma Patients.". Korean J Pathol 47 (5): 452-7. doi:10.4132/KoreanJPathol.2013.47.5.452. PMID 24255633.
  2. ↑ 2.0 2.1 Doğan K, Onder E (January 2024). "ALK-rearranged renal cell carcinoma (ALK-RCC): Evaluation of histomorphological and immunohistochemical features by analysis of 276 renal cell carcinoma cases in Turkey". Pathol Res Pract 253: 154951. doi:10.1016/j.prp.2023.154951. PMID 38039739.
  3. ↑ 3.0 3.1 Jeanneau, M.; Gregoire, V.; Desplechain, C.; Escande, F.; Tica, DP.; Aubert, S.; Leroy, X. (Nov 2016). "ALK rearrangements-associated renal cell carcinoma (RCC) with unique pathological features in an adult.". Pathol Res Pract 212 (11): 1064-1066. doi:10.1016/j.prp.2016.07.015. PMID 27554841.
  4. ↑ Pal, SK.; Bergerot, P.; Dizman, N.; Bergerot, C.; Adashek, J.; Madison, R.; Chung, JH.; Ali, SM. et al. (07 2018). "Responses to Alectinib in ALK-rearranged Papillary Renal Cell Carcinoma.". Eur Urol 74 (1): 124-128. doi:10.1016/j.eururo.2018.03.032. PMID 29685646.
  5. ↑ Kuroda, N.; Karashima, T.; Inoue, K.; Kasajima, A.; Ohe, C.; Kawakami, F.; Mikami, S.; Matsuura, K. et al. (Mar 2015). "Review of renal cell carcinoma with rhabdoid features with focus on clinical and pathobiological aspects.". Pol J Pathol 66 (1): 3-8. PMID 26017874.
  6. ↑ Kai K, Tobu S, Kido S, Mikami S, Takeuchi K, Dobashi A, Togashi Y, Noguchi M, Aishima S (June 2022). "ALK rearrangement-associated renal cell carcinoma morphologically mimicking mucinous tubular and spindle cell carcinoma: a case report". Diagn Pathol 17 (1): 52. doi:10.1186/s13000-022-01238-z. PMC 9206751. PMID 35718773. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9206751/.
  7. ↑ Jeanneau, M.; Gregoire, V.; Desplechain, C.; Escande, F.; Tica, DP.; Aubert, S.; Leroy, X. (Nov 2016). "ALK rearrangements-associated renal cell carcinoma (RCC) with unique pathological features in an adult.". Pathol Res Pract 212 (11): 1064-1066. doi:10.1016/j.prp.2016.07.015. PMID 27554841.
  8. ↑ Kai K, Tobu S, Kido S, Mikami S, Takeuchi K, Dobashi A, Togashi Y, Noguchi M, Aishima S (June 2022). "ALK rearrangement-associated renal cell carcinoma morphologically mimicking mucinous tubular and spindle cell carcinoma: a case report". Diagn Pathol 17 (1): 52. doi:10.1186/s13000-022-01238-z. PMC 9206751. PMID 35718773. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9206751/.
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