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Presented by Eric Burks, MD and prepared by Jeffrey T. Schowinsky, M.D.
Case 2: The patient is a 24-year-old woman with a retroperitoneal mass.
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1. Question
Week 308: Case 2
The patient is a 24-year-old woman with a retroperitoneal mass./images/041607EB2a.jpg
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/images/041607EB2e.jpgCorrect
Answer: Anaplastic large cell lymphoma
Histology: The biopsy is notable for large lymphoid cells which infiltrate in a cohessive pattern with a predilection for the nodal sinuses. Cytologically the nuclei are enlarged, often multinucleated, and have prominent nucleoli, which tend to be smaller and less eosinophilic than those observe in Hodgkin lymphoma. Some cells have nuclei which are eccentric and indented by a cytoplasmic light zone (hoff), and are thus defined as “hallmark cells”.
Discussion: The morphologic findings are highly suggestive of ALCL. Immunohistochemical confirmation is made by intense and diffuse membranous and golgi staining with CD30 in the absence of B-cell markers. In this context, ALK positivity confirms the diagnosis but is not positive in all cases and in fact becomes less frequent as a function of patient age. In ALK negative cases, staining with CD4, CD2 and/or TIA-1 can be confirmatory in conjunction with appropriate morphology, however there appears to be no uniform criteria for separating ALK negative ALCL from PTCL NOS. Never-the-less, the distinction is relevant prognostically as the survival decreases from ALCL ALK+ (best), ALCL ALK- (intermediate), to PTCL NOS (worst). Isolated cutaneous ALCL has the best prognosis of all.
Incorrect
Answer: Anaplastic large cell lymphoma
Histology: The biopsy is notable for large lymphoid cells which infiltrate in a cohessive pattern with a predilection for the nodal sinuses. Cytologically the nuclei are enlarged, often multinucleated, and have prominent nucleoli, which tend to be smaller and less eosinophilic than those observe in Hodgkin lymphoma. Some cells have nuclei which are eccentric and indented by a cytoplasmic light zone (hoff), and are thus defined as “hallmark cells”.
Discussion: The morphologic findings are highly suggestive of ALCL. Immunohistochemical confirmation is made by intense and diffuse membranous and golgi staining with CD30 in the absence of B-cell markers. In this context, ALK positivity confirms the diagnosis but is not positive in all cases and in fact becomes less frequent as a function of patient age. In ALK negative cases, staining with CD4, CD2 and/or TIA-1 can be confirmatory in conjunction with appropriate morphology, however there appears to be no uniform criteria for separating ALK negative ALCL from PTCL NOS. Never-the-less, the distinction is relevant prognostically as the survival decreases from ALCL ALK+ (best), ALCL ALK- (intermediate), to PTCL NOS (worst). Isolated cutaneous ALCL has the best prognosis of all.
