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

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Revision as of 21:46, 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 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.

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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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 World Health Organization (WHO).

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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