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Primary cutaneous anaplastic large cell lymphoma

MedGen UID:
266255
•Concept ID:
C1301362
•
Neoplastic Process
Synonyms: Lymphoma, Primary Cutaneous Anaplastic Large Cell; Primary Cutaneous Anaplastic Large Cell Lymphoma; Primary Cutaneous CD30 positive Large T Cell Lymphoma; Primary Cutaneous CD30-positive Large T-Cell Lymphoma
SNOMED CT: Primary cutaneous anaplastic large cell lymphoma (397352006); Primary cutaneous CD30+ large T-cell lymphoma (128875000); Anaplastic large cell lymphoma, T/Null cell, primary cutaneous type (397352006); Cutaneous T-cell lymphoma, large cell, CD30-positive (397352006); Primary cutaneous anaplastic large T-cell lymphoma, CD30-positive (397352006); Primary cutaneous CD30 antigen positive large T-cell lymphoma (128875000); Primary cutaneous anaplastic large cell lymphoma (773995001); pcALCL - primary cutaneous anaplastic large cell lymphoma (773995001)
 
Monarch Initiative: MONDO:0017598
Orphanet: ORPHA300865

Definition

Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is a rare T-cell non-Hodgkin lymphoma that affects the skin and generally shows no extracutaneous involvement at presentation. It belongs to the spectrum of primary cutaneous CD30+ lymphoproliferative disorders along with lymphomatoid papulosis (see this term) with which it shares overlapping clinical and histopathologic features. [from ORDO]

Professional guidelines

PubMed

Diagnosis and management of cutaneous lymphomas and lymphoid proliferations in children, adolescents and young adults (CAYA).
Stokke J, Tann A, El-Mallawany NK, Oschlies I
Best Pract Res Clin Haematol 2023 Mar;36(1):101448. Epub 2023 Feb 10 doi: 10.1016/j.beha.2023.101448. PMID: 36907638
Primary Cutaneous Lymphomas in Thailand: A 10-Year Retrospective Study.
Pruksaeakanan C, Teyateeti P, Patthamalai P, Thumrongtharadol J, Chairatchaneeboon M
Biomed Res Int 2021;2021:4057661. Epub 2021 Jun 11 doi: 10.1155/2021/4057661. PMID: 34235215Free PMC Article
Cutaneous CD30-positive T-cell lymphoproliferative disorders-clinical and histopathologic features, differential diagnosis, and treatment.
Kempf W, Kerl K, Mitteldorf C
Semin Cutan Med Surg 2018 Mar;37(1):24-29. doi: 10.12788/j.sder.2018.001. PMID: 29719017

Recent clinical studies

Etiology

Primary cutaneous T-cell lymphoma: a review of the most common entities with focus on recent updates.
Saleh JS, Subtil A, Hristov AC
Hum Pathol 2023 Oct;140:75-100. Epub 2023 Oct 4 doi: 10.1016/j.humpath.2023.09.009. PMID: 37802757
Primary cutaneous T-cell lymphomas other than mycosis fungoides and Sézary syndrome. Part I: Clinical and histologic features and diagnosis.
Stoll JR, Willner J, Oh Y, Pulitzer M, Moskowitz A, Horwitz S, Myskowski P, Noor SJ
J Am Acad Dermatol 2021 Nov;85(5):1073-1090. Epub 2021 Apr 30 doi: 10.1016/j.jaad.2021.04.080. PMID: 33940098
CD30+ T-cell lymphoproliferative disorders.
Martin JM, Wu H, Barta SK
Chin Clin Oncol 2019 Feb;8(1):4. Epub 2018 Oct 11 doi: 10.21037/cco.2018.09.06. PMID: 30525751
Brentuximab vedotin or physician's choice in CD30-positive cutaneous T-cell lymphoma (ALCANZA): an international, open-label, randomised, phase 3, multicentre trial.
Prince HM, Kim YH, Horwitz SM, Dummer R, Scarisbrick J, Quaglino P, Zinzani PL, Wolter P, Sanches JA, Ortiz-Romero PL, Akilov OE, Geskin L, Trotman J, Taylor K, Dalle S, Weichenthal M, Walewski J, Fisher D, Dréno B, Stadler R, Feldman T, Kuzel TM, Wang Y, Palanca-Wessels MC, Zagadailov E, Trepicchio WL, Zhang W, Lin HM, Liu Y, Huebner D, Little M, Whittaker S, Duvic M; ALCANZA study group
Lancet 2017 Aug 5;390(10094):555-566. Epub 2017 Jun 7 doi: 10.1016/S0140-6736(17)31266-7. PMID: 28600132
Primary cutaneous anaplastic large cell lymphoma.
Brown RA, Fernandez-Pol S, Kim J
J Cutan Pathol 2017 Jun;44(6):570-577. Epub 2017 Apr 25 doi: 10.1111/cup.12937. PMID: 28342276

Diagnosis

Analysis and therapeutic targeting of the IL-1R pathway in anaplastic large cell lymphoma.
Song Z, Wu W, Wei W, Xiao W, Lei M, Cai KQ, Huang DW, Jeong S, Zhang JP, Wang H, Kadin ME, Waldmann TA, Staudt LM, Nakagawa M, Yang Y
Blood 2023 Oct 12;142(15):1297-1311. doi: 10.1182/blood.2022019166. PMID: 37339580Free PMC Article
Primary cutaneous T-cell lymphomas other than mycosis fungoides and Sézary syndrome. Part I: Clinical and histologic features and diagnosis.
Stoll JR, Willner J, Oh Y, Pulitzer M, Moskowitz A, Horwitz S, Myskowski P, Noor SJ
J Am Acad Dermatol 2021 Nov;85(5):1073-1090. Epub 2021 Apr 30 doi: 10.1016/j.jaad.2021.04.080. PMID: 33940098
CD30-Positive Lymphoproliferative Disorders.
Nikolaenko L, Zain J, Rosen ST, Querfeld C
Cancer Treat Res 2019;176:249-268. doi: 10.1007/978-3-319-99716-2_12. PMID: 30596222
Primary cutaneous anaplastic large cell lymphoma.
Brown RA, Fernandez-Pol S, Kim J
J Cutan Pathol 2017 Jun;44(6):570-577. Epub 2017 Apr 25 doi: 10.1111/cup.12937. PMID: 28342276
Lymphoma of the eyelid.
Svendsen FH, Heegaard S
Surv Ophthalmol 2017 May-Jun;62(3):312-331. Epub 2016 Nov 26 doi: 10.1016/j.survophthal.2016.11.009. PMID: 27894880

Therapy

Non-Hodgkin Lymphoma Developed Shortly after mRNA COVID-19 Vaccination: Report of a Case and Review of the Literature.
Cavanna L, Grassi SO, Ruffini L, Michieletti E, Carella E, Palli D, Zangrandi A, Inzerilli N, Bernuzzi P, Di Nunzio C, Citterio C
Medicina (Kaunas) 2023 Jan 12;59(1) doi: 10.3390/medicina59010157. PMID: 36676781Free PMC Article
Randomized phase 3 ALCANZA study of brentuximab vedotin vs physician's choice in cutaneous T-cell lymphoma: final data.
Horwitz SM, Scarisbrick JJ, Dummer R, Whittaker S, Duvic M, Kim YH, Quaglino P, Zinzani PL, Bechter O, Eradat H, Pinter-Brown L, Akilov OE, Geskin L, Sanches JA, Ortiz-Romero PL, Weichenthal M, Fisher DC, Walewski J, Trotman J, Taylor K, Dalle S, Stadler R, Lisano J, Bunn V, Little M, Prince HM
Blood Adv 2021 Dec 14;5(23):5098-5106. doi: 10.1182/bloodadvances.2021004710. PMID: 34507350Free PMC Article
Existing and Emerging Therapies for Cutaneous T-Cell Lymphoma.
Van-de-Velde V, Zhou Y
J Cutan Med Surg 2019 May/Jun;23(3):319-327. Epub 2019 Apr 3 doi: 10.1177/1203475419840629. PMID: 30943788
Antibody-Based Therapies for Cutaneous T-Cell Lymphoma.
Welborn M, Duvic M
Am J Clin Dermatol 2019 Feb;20(1):115-122. doi: 10.1007/s40257-018-0402-5. PMID: 30430444
Brentuximab vedotin or physician's choice in CD30-positive cutaneous T-cell lymphoma (ALCANZA): an international, open-label, randomised, phase 3, multicentre trial.
Prince HM, Kim YH, Horwitz SM, Dummer R, Scarisbrick J, Quaglino P, Zinzani PL, Wolter P, Sanches JA, Ortiz-Romero PL, Akilov OE, Geskin L, Trotman J, Taylor K, Dalle S, Weichenthal M, Walewski J, Fisher D, Dréno B, Stadler R, Feldman T, Kuzel TM, Wang Y, Palanca-Wessels MC, Zagadailov E, Trepicchio WL, Zhang W, Lin HM, Liu Y, Huebner D, Little M, Whittaker S, Duvic M; ALCANZA study group
Lancet 2017 Aug 5;390(10094):555-566. Epub 2017 Jun 7 doi: 10.1016/S0140-6736(17)31266-7. PMID: 28600132

Prognosis

Analysis and therapeutic targeting of the IL-1R pathway in anaplastic large cell lymphoma.
Song Z, Wu W, Wei W, Xiao W, Lei M, Cai KQ, Huang DW, Jeong S, Zhang JP, Wang H, Kadin ME, Waldmann TA, Staudt LM, Nakagawa M, Yang Y
Blood 2023 Oct 12;142(15):1297-1311. doi: 10.1182/blood.2022019166. PMID: 37339580Free PMC Article
Primary cutaneous T-cell lymphomas other than mycosis fungoides and Sézary syndrome. Part I: Clinical and histologic features and diagnosis.
Stoll JR, Willner J, Oh Y, Pulitzer M, Moskowitz A, Horwitz S, Myskowski P, Noor SJ
J Am Acad Dermatol 2021 Nov;85(5):1073-1090. Epub 2021 Apr 30 doi: 10.1016/j.jaad.2021.04.080. PMID: 33940098
CD30-Positive Lymphoproliferative Disorders.
Nikolaenko L, Zain J, Rosen ST, Querfeld C
Cancer Treat Res 2019;176:249-268. doi: 10.1007/978-3-319-99716-2_12. PMID: 30596222
Primary cutaneous anaplastic large cell lymphoma.
Brown RA, Fernandez-Pol S, Kim J
J Cutan Pathol 2017 Jun;44(6):570-577. Epub 2017 Apr 25 doi: 10.1111/cup.12937. PMID: 28342276
Lymphoma of the eyelid.
Svendsen FH, Heegaard S
Surv Ophthalmol 2017 May-Jun;62(3):312-331. Epub 2016 Nov 26 doi: 10.1016/j.survophthal.2016.11.009. PMID: 27894880

Clinical prediction guides

Primary cutaneous anaplastic large-cell lymphoma with DUSP22-IRF4 rearrangement following insect bites.
Zhang Y, Chen M, Yu Y, Liu X, Liu W, Jiang L, Zhang W
J Cutan Pathol 2022 Feb;49(2):187-190. Epub 2021 Oct 20 doi: 10.1111/cup.14147. PMID: 34622970Free PMC Article
Primary Cutaneous CD30+ Lymphoproliferative Disorders: a Comprehensive Review.
Di Raimondo C, Parekh V, Song JY, Rosen ST, Querfeld C, Zain J, Martinez XU, Abdulla FR
Curr Hematol Malig Rep 2020 Aug;15(4):333-342. doi: 10.1007/s11899-020-00583-4. PMID: 32435988
Radiotherapy of primary cutaneous anaplastic large cell lymphoma: our experience in 30 cases.
Piccinno R, Damiani G, Rossi LC, Berti E
Int J Dermatol 2020 Apr;59(4):469-473. Epub 2020 Jan 9 doi: 10.1111/ijd.14754. PMID: 31916593
Lymphomatoid Papulosis and Other Lymphoma-Like Diseases.
Moy A, Sun J, Ma S, Seminario-Vidal L
Dermatol Clin 2019 Oct;37(4):471-482. Epub 2019 Aug 6 doi: 10.1016/j.det.2019.05.005. PMID: 31466587
CD30+ T-cell lymphoproliferative disorders.
Martin JM, Wu H, Barta SK
Chin Clin Oncol 2019 Feb;8(1):4. Epub 2018 Oct 11 doi: 10.21037/cco.2018.09.06. PMID: 30525751

Recent systematic reviews

Lymphomatoid Papulosis in Children and Adolescents: A Systematic Review.
Wieser I, Wohlmuth C, Nunez CA, Duvic M
Am J Clin Dermatol 2016 Aug;17(4):319-27. doi: 10.1007/s40257-016-0192-6. PMID: 27138554
EORTC, ISCL, and USCLC consensus recommendations for the treatment of primary cutaneous CD30-positive lymphoproliferative disorders: lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma.
Kempf W, Pfaltz K, Vermeer MH, Cozzio A, Ortiz-Romero PL, Bagot M, Olsen E, Kim YH, Dummer R, Pimpinelli N, Whittaker S, Hodak E, Cerroni L, Berti E, Horwitz S, Prince HM, Guitart J, Estrach T, Sanches JA, Duvic M, Ranki A, Dreno B, Ostheeren-Michaelis S, Knobler R, Wood G, Willemze R
Blood 2011 Oct 13;118(15):4024-35. Epub 2011 Aug 12 doi: 10.1182/blood-2011-05-351346. PMID: 21841159Free PMC Article

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