Anaplastic lymphoma kinase-positive large cell lymphoma of the anterior skull base: Report of an unusual case and review of the literature

Clarkc Chen,Huan-You Wang,Jamesy Chen,Pouya Jamshidi

doi:10.4103/2152-7806.110656

Abstract

Background:Anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma (ALCL) is a rare peripheral T-cell lymphoma, accounting for approximately 3% of adult non-Hodgkin lymphomas (NHL). In this report we describe an unusual case of an ALK(+) ALCL, which presented as an aggressive mass involving upper nasal cavity and anterior skull base. The pathogenesis, histopathology with radiologic correlations, and management of this case are reviewed.Case Description:A 28-year-old Asian female presented with a 3-month history of nasal congestion culminating in epistaxis. Physical examination was notable for a tissue mass obstructing nasal cavity and the sphenoid sinus. Computed tomography (CT) and magnetic resonance (MR) imaging revealed a lesion primarily involving the upper nasal cavity extending intracranially through the cribriform plates into the anterior cranial fossa. Histologic and immunohistochemical analysis of the specimen obtained through a transnasal biopsy revealed an ALK(+) ALCL. The patient underwent two cycles of chemotherapy and focal radiation therapy, achieving minimal residual disease. The patient remained neurologically unchanged with stable minimal residual disease at the 1-year follow-up.Conclusions:To the best of our knowledge, this is the first case of an ALK(+) ALCL that presented as an aggressive upper nasal cavity and anterior skull base lesion. This case report highlights the importance of multi-modality approaches including preoperative imaging and tissue biopsy for definitive diagnosis.

Highlights

Anaplastic lymphoma kinase (ALK)‐positive anaplastic large cell lymphoma (ALCL) is a rare peripheral T‐cell lymphoma, accounting for approximately 3% of adult non-Hodgkin lymphomas (NHL)
Lymphoma exclusively involving the nasal sinus or the anterior cranial fossa is rare in Western populations
We describe an unusual case where an ALK(+) ALCL presents as an aggressive lesion of upper nasal cavity and anterior skull base

Summary

Conclusions

To the best of our knowledge, this is the first case of an ALK(+) ALCL that presented as an aggressive upper nasal cavity and anterior skull base lesion. This case report highlights the importance of multi‐modality approaches including preoperative imaging and tissue biopsy for definitive diagnosis. We describe an unusual case where an ALK(+) ALCL presents as an aggressive lesion of upper nasal cavity and anterior skull base. MR imaging of the orbital and nasal region demonstrated a homogenous and well‐circumscribed mass, with mild homogenous enhancement [Figure 1] and mild restricted diffusion [Figure 2a] The lesion expands both nasal cavities and extends intracranially through the cribriform plates and through the clivus into the prepontine cistern. Chromosomal analysis of the rearrangements suggest that the positive ALK expression was most likely due to the translocation of ALK located on chromosome 2p23 and nucleophosmin located on 5q35 namely t(2;5)(p23;q35), based on the both nuclear and cytoplasmic granular staining pattern [Figure 3f]

Findings

DISCUSSION

Result