© Borgis - New Medicine 4/2014, s. 122-125
Teresa Ryczer, *Lidia Zawadzka-Głos
A rare case of Burkitt’s lymphoma in paranasal sinuses in a child
Department of Pediatric Otolaryngology, Medical University of Warsaw, Poland
Head of Department: Lidia Zawadzka-Głos, MD, PhD
Introduction. Burkitt’s lymphoma is a malignant tumor of lymphatic system that originates from B lymphocytes in germinal center of lymph nodes. There may be different clinical types of Burkitt’s lymphoma. Clinical manifestation of disease can involve different localization.
Aim. The aim of the study is to present a rare case of a 4.5-year-old patient With Burkitt’s lymphoma of craniofacial localization with paranasal sunus involvement.
Material and methods. The patient with Burkitt’s lymphoma was hospitalized in the Department of Pediatric Otolaryngology of Medical University of Warsaw in may 2013. Signs and symptoms, results of diagnostic examinations and treatment will be discussed. Available medical record of the patient and diagnostic images were used to prepare the case report.
Results. A 4.5-year-old male patient was admitted to the Pediatric ENT Department due to painless swelling and erythema of the right cheek that persisted for a week. ENT examination revealed edema and erythema of the right cheek, neck lymphadenopathy with no other abnormalities. CT of paranasal sinuses showed vast, soft-tissued mass in the right maxillary sinus, with no contrast enhancement, infiltrating orbita, optic nerve, pterygopalatine fossa. The result of the biopsy was Burkitt’s lymphoma.
Conclusions. Malignant tumors of head and neck in children are rare. First symptoms of Burkitt’s lymphoma may be localized in craniofacial area, thus, especially general practicioners and pediatricians should be aware of careful diagnostic procedures and treatment in these patients. Computed tomography with contrast should be performed in case of any non-specific anomaly in head area. Chemotherapy is treatment of choice in Burkitt’s lymphoma.
Burkitt’s lymphoma (BL) is recognized as a highly malignant B-cell non-Hodgkin’s lymphoma. Burkitt’s lymphoma can be divided into three main clinical types: endemic, sporadic and immunodeficiency-associated. The endemic variant, also known as African variant, occurs mainly in children living in malaria endemic regions of the world, e.g. equatorial Africa, Brazil, Papua New Guinea. In almost all patients with endemic variant of BL Epstein-Barr Virus is found. The disease involves jaw or other facial bones, ileum, cecum, ovaries, kidneys or mediastinum. The sporadic type („non-African”, or „American”) is the most common one in places where malaria is not endemic. In this type of Burkitt’s lymphoma EBV association is rare. The sporadic Burkitt’s lymphoma involves mainly ileocecal region, and rarely involves orbit or paranasal sinuses. Immunodeficiency-associated Burkitt lymphoma occurs usually in immunocompromised patients with HIV infection or after organ transplantation (1, 2). All types of BL are histologically comparable with the difference of epidemic presentation. Symptoms of Burkiit’s lymphoma may be various, thus diagnostic procedures depend on signs and symptoms presented by patient. Burkitt’s lymphoma treatment consist of chemotherapy. Other therapy may be immunotherapy, bone marrow transplants, stem cell transplant, surgery to remove tumor, and radiotherapy.
We would like to present a rare case of a 4.5-year-old patient with Burkitt’s lymphoma of craniofacial localization as first symptom that was hospitalized in the Department of Pediatric Otolaryngology of Medical University of Warsaw in may 2013. Signs and symptoms, results of diagnostic examinations, treatment and follow-up will be discussed.
A 4.5-year-old male patient was admitted to the Pediatric ENT Department due to painless swelling and erythema of the right cheek for a week. In ENT examination there was edema and erythema of the right cheek, neck lymphadenopathy, with no other abnormalities. The patient otherwise appeared normal, with no symptoms of proptosis, epistaxis, headache, fever, weight loss, or night sweats. There was no clinical improvement to applied antibiotic therapy which consisted of clindamycin and cefuroxim. Ultrasonography of the cheek showed hyperechogenic, hyperemic area within soft tissues of the cheek with irregular fluid spaces, and enlarged lymph nodes within the right parotid gland and in the neck. CT scan revealed vast, soft-tissued mass in the right maxillary sinus, with no specific contrast enhancement, infiltrating soft tissues of the orbita, optic nerve, pterygopalatine fossa, masticatory and temporal muscle, buccal space and showed destruction of bony elements of maxillar sinus, as well as of ala mayor of sphenoid sinus (fig. 1).
Fig. 1. CT scan of paranasal sinuses revealed vast, soft-tissued mass in the right maxillary sinus, with no specific contrast enhancement, infiltrating soft tissues of the orbita, optic nerve, pterygopalatine fossa, masticatory and temporal muscle, buccal space and showing destruction of bony elements of maxillar sinus, as well as of ala mayor of sphenoid sinus.
The biopsy of the lesion under general anesthesia was performed. First, the biopsy of the cheek lesion, and afterwards the biopsy of a mass in maxillary sinus through inferior antrostomy was done. At the same time, bone marrow biopsy was performed. The result of the surgical biopsy revealed Burkitt’s lymphoma. Bone marrow biopsy showed no abnormalities in myelogram. The patient was transferred to the Department of Hematology and Oncology for further diagnostic procedures and treatment. There were no signs of pancythopenia in blood test, LDH was normal. Examination of the cerebrospinal fluid did not show any atypical cells.
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