ALK-rearranged renal cell carcinoma: Difference between revisions
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{{ Infobox diagnosis | |||
| Name = {{PAGENAME}} | |||
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| 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 = | |||
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| 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 WHO. | |||
It may be abbreviated ''ALK-RCC''. | |||
==General== | |||
*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]] | |||