Large B-cell Lymphoma with IRF4 Rearrangement in the Nasolacrimal Duct: A Clinicopathological Study of One Case and Literature Review.

Wang-Xing Chen, Jun Wu, Jian-Guo He
Author Information
  1. Wang-Xing Chen: Department of Otolaryngology, The Fourth Affiliated Hospital, Zhejiang University School of Medicine, Yiwu, 322000, Zhejiang, China.
  2. Jun Wu: Department of Otolaryngology, The Fourth Affiliated Hospital, Zhejiang University School of Medicine, Yiwu, 322000, Zhejiang, China.
  3. Jian-Guo He: Department of Otolaryngology, The Fourth Affiliated Hospital, Zhejiang University School of Medicine, Yiwu, 322000, Zhejiang, China.

Abstract

BACKGROUND: Large B-cell lymphoma (LBCL) with interferon regulatory factor 4 (IRF4) rearrangement (LBCL-IRF4) is a rare subtype of LBCL, with a high prevalence in Waldeyer's ring as well as the neck, head and gastrointestinal lymph nodes.
MATERIALS AND METHODS: A patient with 2-month clinical symptoms of nasal obstruction and facial swelling was reported in this short review. A nasal endoscopy examination revealed a neoplasm in the inferior nasal meatus. Both CT and enhanced MRI showed that a soft tissue occupied the nasolacrimal duct, with bone destruction, and extended into the left nasal cavity and left lacrimal gland area. Then, a biopsy of the neoplasm in the inferior nasal meatus was performed.
RESULTS: HE staining results showed that neoplastic cells presented diffuse growth patterns, abundant cytoplasm, vacuole shape, lightly stained nuclei, and irregular nuclear membrane. Immunohistochemistry staining results revealed MUM1(+), Bcl- 6(+), CD20(+), CD79α(+), and CD10(+). FISH analyses detected positive IRF4 rearrangement. LBCL-IRF4 was diagnosed in the patient. The patient received treatment with four cycles of R-CHOP and two times of rituximab, followed up for 2 years, and finally got complete remission.
CONCLUSION: For the first time, we summarize the imaging and pathological features, drug treatment, and curative effect of LBCL-IRF4 in the nasolacrimal duct.

Keywords

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Grants

  1. Y202146976/Foundation of Zhejiang Provincial Education Department and Science
  2. 2021-4-273/Technology program of Jinhua Science and Technology Bureau

MeSH Term

Humans
Antineoplastic Combined Chemotherapy Protocols
Cyclophosphamide
Gene Rearrangement
Interferon Regulatory Factors
Lymphoma, Large B-Cell, Diffuse
Nasolacrimal Duct
Rituximab
Vincristine

Chemicals

Cyclophosphamide
interferon regulatory factor-4
Interferon Regulatory Factors
Rituximab
Vincristine

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