<scp>ALK</scp>+ large B cell lymphoma presenting as multiple bowel‐obstructing, cytokeratin‐positive tumours

Petronilho, Sara; Gournay, Viviane; Tauziède‐Espariat, Arnault; Pina, Héloïse; Pécriaux, Adrien; Drieux, Fanny; Poullot, Elsa; Brière, Josette; Lechapt, Emmanuèle; Gaulard, P.

doi:10.1111/his.14635

Cited by 1 publication

(2 citation statements)

References 5 publications

(13 reference statements)

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“…Though it is usually positive for EMA (93% to 96%), the CK expression in ALK-positive LBCL is a relatively rare finding and has been reported in around 10 cases which is usually weak, focal, and described as perinuclear dot-like pattern 1,2,4 . In the literature, only 2 reported cases of ALK-positive LBCL with diffuse CK AE1/AE3 staining were found 10,11 . The current case is the first to be reported with diffuse expression of multiple epithelial-associated markers (AE1/AE3 and CK8/18) and focal EMA.…”

Section: Discussionmentioning

confidence: 66%

“…1,2,4 In the literature, only 2 reported cases of ALK-positive LBCL with diffuse CK AE1/AE3 staining were found. 10,11 The current case is the first to be reported with diffuse expression of multiple epithelial-associated markers (AE1/ AE3 and CK8/18) and focal EMA. In addition, focal GATA3 expression may misleadingly suggest neoplasms of urothelial or breast origin, although it should be noted that expression of EMA and GATA3 is not unique to epithelial tumors and they have also been reported to be expressed in lymphoid malignancies.…”

Section: Discussionmentioning

confidence: 74%

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ALK-positive Large B-Cell Lymphoma With Multiple Epithelial Antigen Expression and PABPC1::ALK Fusion

Seth

Ali

et al. 2023

American Journal of Surgical Pathology

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Anaplastic lymphoma kinase (ALK)-positive large B-cell lymphoma (LBCL) is a very rare type of LBCL with an aggressive clinical course and poor prognosis. This diagnosis can be challenging given the varied morphology (immunoblastic, plasmablastic, or anaplastic), frequent lack of B-cell antigens, and especially in cases with expression of epithelial antigens. Here, we report a case of ALK-positive LBCL with unusual expression of 4 epithelial-associated markers (AE1/AE3, CK8/18, EMA, and GATA3) and novel poly(A) binding protein cytoplasmic 1 (PABPC1)::ALK fusion which has not been previously reported in this entity. This case also emphasizes the use of comprehensive immunophenotyping that includes multiple lineage-specific antibodies when faced with a malignancy without a clear differentiation to avoid misdiagnosis. This case only achieved partial response to combination chemotherapy, radiation, and ALK inhibitor regimens, and furthers our understanding of this uncommon lymphoma.

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Section: Discussionmentioning

confidence: 66%