Pathology and diagnosis of follicular lymphoma and related entities

“…Adult patients with PCFCL typically present with one or more localized, erythematous papules, plaques, and/or tumors on the head or trunk 11 . The 5‐year survival rate in adults with PCFCL has been estimated to be over 95% 19 . The prognosis of PCFCL in pediatric and adolescent patients is believed to be similar.…”

Section: Discussionmentioning

confidence: 99%

Primary cutaneous follicle center lymphoma in a 16‐year‐old girl

et al. 2021

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In the pediatric and adolescent age group, primary cutaneous lymphomas are rare, especially cutaneous B‐cell lymphomas. According to the World Health Organization, the three main subtypes of primary cutaneous B‐cell lymphomas are primary cutaneous marginal zone B‐cell lymphoma (PCMZL), primary cutaneous follicle center lymphoma (PCFCL), and primary cutaneous diffuse large B‐cell lymphoma, leg type. We present an extraordinary case of PCFCL arising in a 16‐year‐old female, only the sixth case of PCFCL to be reported in the literature in a patient less than 20 years old. Although PCMZL was considered in this case, the finding of lambda light chain restriction in the BCL‐6 and CD10 positive population of lymphocytes established the diagnosis of primary cutaneous follicle center lymphoma. Not many data currently exist on the prognosis of PCFCL in young individuals, but adult PCFCL has a good prognosis with an indolent course and 5‐year survival rates over 95%. Because of its uncommon manifestation in young patients, the diagnosis of PCFCL is often delayed or missed. This case is presented to raise awareness of PCFCL in the pediatric/ adolescent population and to contribute to the ongoing research of this condition.

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“…All small B‐cell lymphomas are thought to be arrested at a particular stage of development; PCFCL is arrested at the germinal center stage, whereas MZLs have arrested after germinal center selection. The maturation arrest of follicle center lymphomas is likely multifactorial; however, expression of CD10, BCL2, and BCL6 may be involved 11,25,26 . PD in follicle center B‐cell lymphomas has been divided into two morphologic types with one type having predominately interfollicular PC, usually with BCL2 rearrangement, and the second type having predominately intrafollicular/perifollicular PC, usually without BCL2 rearrangement in either component 10,17 .…”

Section: Discussionmentioning

confidence: 99%

Primary cutaneous follicle center lymphoma with extensive plasmacytic differentiation and t(14;18) in both the lymphoid and plasma cell components

et al. 2021

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Primary cutaneous follicle center lymphoma (PCFCL) is the most common cutaneous B‐cell lymphoma. The typical immunophenotype includes expression of both CD20 and BCL6, with the majority of cases lacking expression of CD10, BCL2, and the characteristic t(14;18)/IGH‐BCL2 rearrangement seen in systemic follicular lymphoma (FL). Plasmacytic differentiation (PD) is an uncommon finding in both systemic and cutaneous FLs and presents a diagnostic challenge when present, leading to the potential for misdiagnosis as marginal zone lymphoma (MZL). Limited reports have described light chain restriction in the plasma cell component of FLs with PD, and rare cases of PCFCL with PD have been described. While the IGH‐BCL2 translocation has been identified in a subset of FLs with PD, the presence of the BCL2 translocation in monotypic plasma cells of PCFCL has not been previously described to our knowledge. Here, we report a case of PCFCL with extensive PD in a 77‐year‐old woman that was favored to represent primary cutaneous MZL on an initial punch biopsy. Excisional biopsy, however, revealed that the atypical lymphocytes expressed CD10, BCL6, and BCL2, while the plasma cell component demonstrated light‐chain lambda restriction. FISH studies showed the presence of an IGH‐BCL2 translocation in both the lymphocytic and plasmacytic components.

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Pathology and diagnosis of follicular lymphoma and related entities

Cited by 19 publications

References 79 publications

Diffuse large B-cell lymphoma variants: an update

Diffuse large B-cell lymphoma variants: an update

Primary cutaneous follicle center lymphoma in a 16‐year‐old girl

Primary cutaneous follicle center lymphoma with extensive plasmacytic differentiation and t(14;18) in both the lymphoid and plasma cell components

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