|Year : 2022 | Volume
| Issue : 3 | Page : 130-133
Burkitt’s lymphoma at nose and nasopharynx of a 10-year-old boy—A case report
Santosh K Swain
Department of Otorhinolaryngology and Head and Neck Surgery, IMS and SUM Hospital, Siksha “O” Anusandhan University, Bhubaneswar, Odisha, India
|Date of Submission||12-Mar-2022|
|Date of Decision||28-May-2022|
|Date of Acceptance||06-Jun-2022|
|Date of Web Publication||21-Feb-2023|
Santosh K Swain
Department of Otorhinolaryngology and Head and Neck Surgery, IMS and SUM Hospital, Siksha “O” Anusandhan University, K8, Kalinga Nagar, Bhubaneswar 751003, Odisha
Source of Support: None, Conflict of Interest: None
Burkitt’s lymphoma (BL) is an aggressive and rapid neoplasm that is curable and highly sensitive to chemotherapy. It can affect any organ or part of the body, leading to several clinical presentations. Early diagnosis and treatment of BL are very important before landing life-threatening complications such as airway compromise and compression of vital structures such as the spinal cord. This is a case of a 10-year-old boy, who developed BL at the posterior part of the nasal cavity and nasopharynx. This case report aims to describe the rarity of the location, course of the pathology, clinical presentations, investigations, and treatment. As BL is an aggressive neoplasm and is considered a serious health problem, it needs timely diagnosis for prompt and appropriate treatment.
Keywords: Burkitt’s lymphoma, nasopharynx, non-Hodgkin’s lymphoma
|How to cite this article:|
Swain SK. Burkitt’s lymphoma at nose and nasopharynx of a 10-year-old boy—A case report. D Y Patil J Health Sci 2022;10:130-3
| Introduction|| |
Burkitt’s lymphoma (BL) is a highly aggressive lymphoma documented by Denis Burkitt in Africa, in a region of endemic malaria. BL is a subtype of non-Hodgkin’s lymphoma (NHL) and is described as a malignant neoplasm of mature, low-frequency B-cell lymphocytes. It accounts for 1%–3% of the NHL and is characterized by the aggressive nature of the neoplasm. The etiology of the BL is due to chromosomal translocation t (8; 14) (q24; q32) and deregulation of the c-Myc oncogene. However, there are some other associated factors such as Epstein-Barr virus (EBV) infection and malaria seen in BL. There are three types of BL identified such as endemic, sporadic, and immunodeficiency associated. BL is more commonly seen in males rather than females with a ratio of 2:1. Timely and accurate diagnosis of the BL is challenging but crucial. Here, we report a case of BL at the posterior part of the nasal cavity and nasopharynx in a young boy who was managed successfully by chemotherapy.
| Case Report|| |
A 10-year-old boy attended the Outpatient Department of Otorhinolaryngology for complaints of the nasal block for 3 weeks and facial pain for 10 days. Anterior rhinoscopy showed a friable mass in the posterior part of the right side nasal cavity extending into the nasopharynx. The diagnostic nasal endoscopy revealed the same friable mass with an irregular surface in the posterior part of the right side nasal cavity, which arises from the nasopharynx [Figure 1]. The visual acuity and extraocular movements were within the normal limit. Neck examinations showed no palpable lymph nodes. On systemic examination, no other significant findings were present. Routine blood examinations showed normal reports except mild anemia. The computed tomography (CT) scan showed a soft-tissue mass at the right side nasopharynx without any evidence of bony erosion [Figure 2]. All the paranasal sinuses were within the normal limit. The biopsy was planned under general anesthesia. Tissue from the nasopharynx was sent for histopathological examination. The histopathological report revealed typical features of BL such as diffuse sheets of monotonous and medium-sized B lymphocytes with starry sky patterns. The tumor cells showed square-off borders with single-round nuclei possessing finely clumped chromatin and several small nucleoli with a high mitotic rate [Figure 3]. Immunohistological study showed tumor cells are immunoreactive for CD20 [Figure 4]a and Ki67 [Figure 4]b markers. The patient was sent to the Department of Hemato-oncology for treatment, where six cycles of chemotherapy such as prednisone, cyclophosphamide, allopurinol, cytarabine, methotrexate, and dexamethasone were given. The child responded well to this chemotherapy regimen. The endoscopy of the nasopharynx after 3 months of treatment revealed a normal appearance without any evidence of the lesion. The patient was recovered satisfactorily and is doing well after 6 months.
|Figure 1: Diagnostic nasal endoscopy showing friable mass in the right nasal cavity covered with slough, which extends into the nasopharynx|
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|Figure 2: CT scan of the nose and paranasal sinus showing radio-opaque mass in the nasal cavity and nasopharynx|
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|Figure 4: Immunohistological examinations show a strong positive to (a) CD20 (400×) and (b) Ki67|
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| Discussion|| |
Lymphomas are broadly classified into Hodgkin’s lymphoma or NHL. NHL is again classified into B-cell lymphoma, T-cell lymphoma, or natural killer cell lymphoma. NHL is the commonest lymphoma in the head and neck region, and most often these are B-cell lymphomas. NHL is often high grade in the pediatric age group. BL accounts for about 30% of NHL cases and is usually aggressive. The common primary sites for BL are lymph nodes, particularly within the head and neck region or abdomen. Other locations can be skin, bone, or central nervous system. BL was thought to occur in tropical Africa other than at high altitudes or in the region where the climate was relatively cool. The occurrence of the BL was more in areas with greater rainfall. These climatic and geographical associations suggested a relation between BL and falciparum malaria.
BL is of three variants such as endemic (African) form, which is the most common worldwide, followed by the sporadic (American), which is commonly seen in North America, and then the immunodeficiency-associated type. Sporadic-type and immunodeficiency-type are commonly found in the abdominal region and bone marrow and rarely seen in the head and neck region. But the African type of BL commonly manifests nasofacial bones and nearby soft tissues and is known to be associated with EBV. Around 58% of the head and neck cases of BL are found in the mandible or maxilla. However, it is less common in the nose and nasopharynx, although very few have been reported in the nasal cavity and nasopharynx. The head and neck area, jaw, and other facial bones are commonly affected by the endemic type, whereas Waldeyer’s ring (palatine and lingual tonsils, adenoids) and nodal involvement are often seen in the sporadic type. Other than abdominopelvic and head and neck region involvement, BL may rarely be located primarily at the breast, pleura, soft tissues, pancreas, thyroid, skin, lung, or spinal extradural area. In the disseminated form of the BL, these tissues may be affected. BL can be found in HIV-positive patients as AIDS is often related to the development of NHL. BL is a rare type and prevalent in patients with AIDS. There is around 10%–34% of the NHLs seen in extranodal sites. Similarly, BL in the head and neck area often originates as cervical adenopathy, although it can be found in the mandible, maxilla, nasopharynx, and tonsils. The clinical presentations of this aggressive neoplasm are related to the site of the expansion such as jaw swelling in mandibular involvement, facial swelling, proptosis, and loss of teeth. In our case, the child was presenting with nose block, mouth breathing, and otalgia, which was confusing with adenoid hypertrophy.
CT scan is an important radiological investigation used to evaluate the spread and severity of the disease and tumor staging. CT is the best medical investigation to localize the lesion. Presently, positron emission tomography/CT scans are used for functional and anatomical assessment during the staging of the tumor and follow-up of the after treatment. Incisional biopsy and in situ hybridization are other tests that help get the correct diagnosis. Histopathological report shows the proliferation of large polymorphic B cells with irregular nuclei, suggesting diffuse large B-cell NHL. Histologically, the tissue shows a hypercellular area admixed with small-to-medium slightly atypical lymphocytes with several tangible body macrophages showing a classical starry sky appearance. On the other hand, immunohistochemistry is a valuable technique to find out specific tumor cells such as anti-CD20 positive and anti-CD3 negative; immune-staining tests are helpful to corroborate the B-lymphocyte origin of the neoplasm and to rule out the tumor origin as T lymphocytes. Based on the pathological findings, the differential diagnosis includes other verities of high-grade B-cell lymphoma, lymphoblastic lymphoma, leukemia, and blastoid mantle cell lymphoma. Other high-grade nonlymphomatous childhood malignancies are also considered as differential diagnoses such as embryonal rhabdomyosarcoma, Ewing’s sarcoma, metastatic neuroblastoma, and primitive neuroectodermal tumor.,
The definitive treatment of BL is often individualized according to the patient’s age and the localization of the tumor. There are different treatment options for BL: surgery, radiotherapy, chemotherapy, and radioimmuno therapy. The role of surgery in BL is still controversial. Surgery is often indicated in case of organic obstruction of the airway or in a situation such as a decompression of the optic nerve when the orbit is involved and in the case of diagnostic biopsy. BL dramatically responds to chemotherapies inducing regressions of the tumor and often leads to long-term remission. Presently 5-year survival for advanced BL in pediatric patient and young adults have increased by 2–3 times in the last 3 decades, from 85% to 90% with less than 6 months of intensive chemotherapy. This is due to a better understanding of the disease biology and the advancement of chemotherapeutic drugs.
| Conclusion|| |
BL at the nose and nasopharynx is an extremely rare clinical entity, and the diagnosis of it is confirmed by histopathological examinations and immunohistochemistry. It is rarely found in the nose and nasopharynx and presents with nonspecific insidious symptoms from nasal obstruction to mouth breathing. With the diversity of the clinical presentations and nature of the disease, a high degree of clinical suspicion from physicians is warranted to find out this pathology in its polymorphism. BL is an aggressive neoplasm and is often considered as a serious health condition, so prompt and appropriate treatment of the disease is always required.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]