|Year : 2013 | Volume
| Issue : 1 | Page : 35-37
Life threatening retropharyngeal Burkitt's lymphoma mimicking adenoid obstructive disease
Aminu Bakari1, Babagana M Ahmad2, Garba M Mohammed2, Tijjani S Abubakar2
1 Department of Surgery, Ahmadu Bello University, Zaria, Nigeria
2 Department of Clinical Services, National Ear Care Centre, Kaduna, Nigeria
|Date of Web Publication||18-Oct-2013|
Department of Surgery, Ahmadu Bello University, Zaria
Source of Support: None, Conflict of Interest: None
| Abstract|| |
The commonest sites of non-Hodgkin's lymphoma in the head and neck are the cervical nodes, Waldeyer's ring and extra lymphatic organs such as the sinuses, larynx, salivary glands and oral cavity etc. We present the case of a 10 year old with upper airway obstruction secondary to retropharyngeal Burkitt's lymphoma. The presenting features here are very similar to obstructive adenoid enlargement. Emergency tracheotomy, biopsy, and chemotherapy led to resolution and subsequent decanulation. This case is highlighted in relation to the increasing recognition of AIDS related lymphomas globally.
Keywords: Burkitt′s lymphoma, Non-Hodgkin′s lymphoma, retropharyngeal
|How to cite this article:|
Bakari A, Ahmad BM, Mohammed GM, Abubakar TS. Life threatening retropharyngeal Burkitt's lymphoma mimicking adenoid obstructive disease. Ann Nigerian Med 2013;7:35-7
|How to cite this URL:|
Bakari A, Ahmad BM, Mohammed GM, Abubakar TS. Life threatening retropharyngeal Burkitt's lymphoma mimicking adenoid obstructive disease. Ann Nigerian Med [serial online] 2013 [cited 2020 May 27];7:35-7. Available from: http://www.anmjournal.com/text.asp?2013/7/1/35/119986
| Introduction|| |
Lymphomas comprise of 33 to 47% of all pediatric cancers in Nigeria ,, and Burkitt's lymphoma predominates, with majority affecting males in age group between 6 to 10 years. 
The term Burkitt's lymphoma is applied for historical reasons to B-cell lymphoma arising in areas of the world where there is holoendemic malaria. First described by Dr. Denis Burkitt in Uganda, it was initially thought to be a sarcoma of the jaw, but shortly after became well known as a distinct form of non - Hodgkin's lymphoma.  The commonest site of extra nodal non - Hodgkin lymphoma in the head and neck is the Waldeyer's ring, salivary gland, paranasal sinuses, orbit, maxilla and the mandible.  The presenting features in Waldeyer's ring involvement are similar to non-neoplastic lesions like adenoids and retropharyngeal abscesses. Retropharyngeal Burkitt's lymphoma can mimic other common diseases mentioned above, thereby causing misdiagnosis and delay in accurate treatment as seen in this patient.
| Case Report|| |
A ten-year-old schoolboy was referred from a general hospital to our centre with a 4 months history of progressive neck swelling, intra oral swelling with nasal blockage resulting in mouth breathing. There was associated difficulty in breathing with daytime somnolence. There was marked weight loss, but with no history of fever.
On examination, he was found to be conscious with difficult and noisy breathing (mouth breathing). The nasal cavities were not patent but he had no obvious intranasal growth. Throat examination revealed a posterior pharyngeal wall swelling with healthy-looking palatine tonsils bilaterally. There were bilateral multiple non-tender cervical lymphadenopathy measuring 2 × 2 cm to 3 × 4 cm. His packed cell volume was 28%, which was corrected before surgery. The chest x-ray was within normal limits but the lateral soft tissue neck x-ray showed widening of the retropharyngeal space and straightening of the cervical spine [Figure 1]. A working diagnosis of adenoids enlargement to rule out retropharyngeal abscess was made. Abdominal ultrasound scans showed some para aortic lymph nodes with the liver, spleen, pancreas, gall bladder and both kidneys all within normal limits. Human immune deficiency (HIV) screening was negative.
|Figure 1: Lateral soft tissue neck x-ray showing widening of the retropharyngeal space and straightening of the cervical spine|
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He had an emergency tracheotomy and examination under anaesthesia to assess the nasopharynx and the oropharynx. The nasopharyngeal tonsil was not enlarged. The oropharyngeal swelling was firm with intact mucosa yielding a dry tap on aspiration. A biopsy was obtained via a 2 cm incision of the posterior wall of the pharyngeal mucosa to get to the retropharyngeal mass. The mass was yellowish and cheesy in some parts but not hemorrhagic. Specimens were obtained and embedded in 10% formalin and sent for histology. The posterior pharyngeal incision was sutured with 2/0 chromic catgut. Recovery from anaesthesia was uneventful and postoperative tracheotomy care guidelines were followed [Figure 2].
The histology report showed sections of malignant lymphoid cells with hyper chromatic non-cleaved nuclei and scanty cytoplasm. Interspersed among the malignant cells are histiocytes giving a starry sky appearance consistent with Burkitt's lymphoma [Figure 3]. The patient was referred to the hematologist for chemotherapy. Following two cycles of chemotherapy (cyclophosphamide, vincristine, methotrexate and prednisolone), there was complete tumor regression [Figure 4] and he was successfully decanulated and discharged to the hematologist to complete his therapy.
|Figure 3: Photomicrograph of the histology slide showing malignant lymphoid cells with hyper chromatic non-cleaved nuclei and scanty cytoplasm. Interspersed among the malignant cells are histiocytes giving a starry sky appearance consistent with Burkitt's lymphoma|
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| Discussion|| |
Childhood non-Hodgkin lymphomas are a heterogeneous group of malignancies that arise from T-cells, B-cells or natural killer (NK) lymphocytes.  Burkitt's lymphoma is a B cell lymphoma genetically characterized by a chromosomal translocation that results in de -regulation of the c - MYC oncogene, and is the most common neoplastic disease of childhood in Africa.  It is reported to account for 30 to 50% of all childhood cancers in equatorial Africa.  In the World Health Organization (WHO) classification, three clinical variants of Burkitt's lymphoma are described: endemic, sporadic, and immunodeficiency-associated types.  The endemic variety occurs in equatorial Africa between latitudes 10 degrees North and 10 degrees south, an area also referred to as the malaria belt or Burkitt's lymphoma belt.  It is invariably associated with Epstein Barr Virus infection. Malaria also appears an important co factor in on-cogenesis.  In contrast, the sporadic (western) variety is associated with Epstein Barr Virus in only 20% of cases and most often arises in the abdomen or Waldeyer's ring.  Sporadic Burkitt's lymphoma occurs worldwide, and it includes those cases occurring with no specific geographic or climatic association. It accounts for 1-2% of lymphomas in adults and up to 40% of lymphomas in children in the United States of America and Western Europe. The abdomen, especially the ileocecal area, is the most common site of involvement; the ovaries, kidneys, omentum, Waldeyer's ring, and other sites may also be involved.  Immunodeficiency-associated Burkitt's lymphoma occurs mainly in patients infected with HIV infection but also occurs in allograft recipients and individuals with congenital immunodeficiency. In the early years of the Acquired Immune Deficiency Syndrome (AIDS) epidemic, several cases of Burkitt's lymphoma were described in homosexual men. These were the first descriptions of non-Hodgkin's lymphoma arising in association with what was later recognized as HIV infection. Burkitt's lymphoma accounts for 30-40% of non-Hodgkin's lymphoma in HIV positive patients.  Our patient was HIV negative, had no features of immunosuppression and never received an allograft.
Endemic Burkitt's lymphoma presents as a rapidly growing solid tumor or tumors, which are predominantly extra nodal. The clinical history is short and very few patients have symptoms of greater than three months duration.  The site of presentation commonly involves the bones of the jaw and other facial bones, as well as the kidneys, gastrointestinal tract, ovaries, breast, and other extra nodal sites.  The presenting features in this patient are very similar to obstructive adenoid enlargement, especially with the airway obstruction, mouth breathing, and absence of fever. However, the radiographic finding of a homogenous widening of the retropharyngeal space down to C6 vertebra raised the possibility of a retropharyngeal abscess but for the absence of fever. A Computerised tomography Scan or Magnetic resonance Imaging of the pharyngeal lesion could have ruled out an abscess, but such a facility was not available at our centre. Endemic Burkitt's lymphoma as in this case has exquisite sensitivity to chemotherapy unlike sporadic and immunodeficiency-associated types. Short-duration, high-intensity chemotherapy yields excellent survival in children.  Four cycles of cyclophosphamide, vincristine, and methotrexate, with each cycle lasting three weeks resulted in complete resolution of disease in this patient.
Although the most important prognostic features have yet to be determined, some features that have been associated with adverse outcome in adults and children include older age, advanced stage, poor performance status, bulky disease, high lactate dehydrogenase, and central nervous system or marrow involvement. Among paediatric patients, a poorer prognosis is associated with age over 15 years. 
Burkitt's lymphoma of the retropharyngeal space is relatively uncommon but should remain an important differential diagnosis in patients presenting with retropharyngeal swellings, especially with the now increasing recognition of AIDS related Burkitt's lymphomas globally.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]