Publication: Diffuse large B cell lymphoma presenting as Horner’s syndrome in a patient diagnosed with neurofibromatosis type 1: a case report and review of the literature
Issued Date
2012
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eng
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Mahidol University
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BioMed Central
Bibliographic Citation
Journal of Medical Case Reports. Vol. 6, (2012), 8
Suggested Citation
Saoraya Lueangarun, Chirayu U Auewarakul Diffuse large B cell lymphoma presenting as Horner’s syndrome in a patient diagnosed with neurofibromatosis type 1: a case report and review of the literature. Journal of Medical Case Reports. Vol. 6, (2012), 8. doi:10.1186/1752-1947-6-8 Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/2642
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Diffuse large B cell lymphoma presenting as Horner’s syndrome in a patient diagnosed with neurofibromatosis type 1: a case report and review of the literature
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Abstract
Introduction: Horner’s syndrome has a variety of etiologies ranging from benign to serious life-threatening
conditions and has been infrequently reported as a presenting symptom of patients with lymphoid neoplasms.
Only one case of Burkitt’s lymphoma presenting with toothache, paresthesia, and Horner’s syndrome has been
described and no case reports of diffuse large B-cell lymphoma as the etiology of Horner’s syndrome currently
exist in the literature. In addition, lymphoid neoplasms have rarely been reported to occur in patients with
neurofibromatosis type 1 despite an increased risk of many types of cancer in such cases.
Case presentation: A 28-year-old Thai man presented with a progressively enlarged left supraclavicular mass
together with a significant weight loss and night sweating for four months. He also noticed hoarseness and ptosis
of his left eye associated with double vision for two months. Physical examination revealed large supraclavicular
lymphadenopathy and Horner’s syndrome (ptosis, miosis, and anhydrosis) on the left side of his face. A large
mediastinal mass was clearly detected by chest X-ray and computed tomography and subsequent lymph node
biopsy provided a diagnosis of diffuse large B-cell lymphoma. Interestingly, the patient was also definitely
diagnosed with neurofibromatosis type 1 from multiple café au lait macules, axillary freckles, three neurofibromas,
multiple Lisch nodules, and a history of affected family members. He subsequently received chemotherapy with a
good response. Twenty-seven cases of various types of lymphoid neoplasms previously reported to occur in
neurofibromatosis type 1 patients were also extracted from the literature. All cases were non-Hodgkin lymphoma
and the major subtype was T-cell. Only nine cases were B-cell lymphoma. The majority of cases were young with a
median age at lymphoma diagnosis of 9.4 years (range 1.1 to 77 years). Two-thirds of the cases were boys or men.
Other concomitant malignancies were brain tumor, colorectal cancer, pheochromocytoma, and acute
lymphoblastic leukemia.
Conclusions: We describe for the first time a case of diffuse large B-cell lymphoma that occurred in a
neurofibromatosis type 1 patient with Horner’s syndrome. Horner’s syndrome can be an initial manifestation of
diffuse large B-cell lymphoma. Patients who present with a classical triad of Horner’s syndrome should always be
fully investigated for lymphomatous involvement, especially in the thorax. The exact molecular mechanism for
diffuse large B-cell lymphoma development in neurofibromatosis type 1 cases remains to be elucidated.