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Year : 2022  |  Volume : 24  |  Issue : 4  |  Page : 188-191

Primary Burkitt's lymphoma at the nasopharynx and skull base with unusual clinical presentations in a 13-year-old boy

Department of Otorhinolaryngology and Head and Neck Surgery, IMS and SUM Hospital, Siksha “O” Anusandhan University, Bhubaneswar, Odisha, India

Date of Submission03-Sep-2022
Date of Decision06-Nov-2022
Date of Acceptance07-Nov-2022
Date of Web Publication30-Dec-2022

Correspondence Address:
Prof. Santosh Kumar Swain
Department of Otorhinolaryngology and Head and Neck Surgery, IMS and SUM Hospital, Siksha “O” Anusandhan University, K8, Kalinga Nagar, Bhubaneswar - 751 003, Odisha
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/sjoh.sjoh_40_22

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Burkitt's lymphoma (BL) is a rare and aggressive neoplasm an uncommon at the nasopharynx and skull base area. BL is rapidly growing B-cell non-Hodgkin's lymphoma. It commonly affects the gastrointestinal tract, whereas the primary involvement of the anterior skull base and nasopharynx is uncommon. Histopathological and immunohistochemical examinations confirm the diagnosis. Here, we are presenting a case of a 13-year-old boy presenting with BL at the nasopharynx and anterior skull base with intracranial invasion. The objective for reporting of this case report is to describe the rarity of location, unusual clinical presentations, and management of the BL. As BL is an aggressive tumor and considered a serious clinical condition, so requires timely diagnosis for prompt and appropriate treatment.

Keywords: Burkitt's lymphoma, nasopharynx, recurrent epistaxis

How to cite this article:
Swain SK. Primary Burkitt's lymphoma at the nasopharynx and skull base with unusual clinical presentations in a 13-year-old boy. Saudi J Otorhinolaryngol Head Neck Surg 2022;24:188-91

How to cite this URL:
Swain SK. Primary Burkitt's lymphoma at the nasopharynx and skull base with unusual clinical presentations in a 13-year-old boy. Saudi J Otorhinolaryngol Head Neck Surg [serial online] 2022 [cited 2023 Jan 30];24:188-91. Available from: https://www.sjohns.org/text.asp?2022/24/4/188/366332

  Introduction Top

Burkitt's lymphoma (BL) is an aggressive lymphoma first described by Denis Burkitt in Africa, in region of endemic malaria.[1] BL is a subtype of non-Hodgkin's lymphoma (NHL) and described as a malignant neoplasm of mature, low-frequency B-cell lymphocytes. It accounts for 1%–3% of the NHL and characterized by aggressive nature of the neoplasm.[2] The common sites of the BL are the mandible, facial bones, gastrointestinal tract, kidneys, ovaries, breast, and extranodal sites. 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 infection and malaria seen in BL.[3] There are three types of BL identified such as endemic, sporadic, and immunodeficiency associated.[3] BL is more commonly seen in males rather than females with ratio of 2:1.[4] Accurate and early diagnosis of the BL is often 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. Here, we are presenting a case of BL in a 13-year-old boy with unusual clinical presentations of recurrent epistaxis and bilateral nasal block, mimicking with juvenile nasopharyngeal angiofibroma.

  Case Report Top

A 13-year-old boy attended the outpatient department of otorhinolaryngology with complaints of recurrent nasal bleeding from the right nostril for 3 months. He was also presented with both side nasal blocks for 2 months. He was treated with topical nasal decongestants, antibiotics, and antihistamines by local pediatricians but failed from symptomatic relief. The clinical examinations of the oral cavity, oropharynx, and neck were within normal limits. Fiberoptic nasopharyngolaryngoscopy showed a pinkish mass which completely filling the nasopharynx and blocking both choana. Then, computed tomography (CT) scan of the head-and-neck area with contrast was done which revealed a large nasopharyngeal tumor with intracranial extension. All the paranasal sinuses were within normal limits in the CT scan. Magnetic resonance imaging (MRI) was done to find out the soft tissue invasion and intracranial extension [Figure 1]. It was reported as juvenile nasopharyngeal angiofibroma which originated from the ascending pharyngeal artery with intracranial extension through the foramen ovale. Then, the child was planned for endoscopic excision of the tumor and biopsy. Endoscopic picture showed the vascular appearance of the anterior part of the tumor and the rest was friable which bleeds on touch. The tumor was debulked from the nasopharynx with help of the coblation and both the choana were made patent. The mass 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 [Figure 2]. The cells of the tumor showed square-off borders with single round nuclei possessing finely clumped chromatic and several small nucleoli with high mitotic rate. Immunohistological study showed positive for CD20 and Ki67 markers [Figure 3]. The serology for human immunodeficiency virus (HIV) was negative. The patient was referred to the hemato-oncology department 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. Endoscopy of the nasopharynx after 3 months of treatment revealed a normal appearance without any evidence of the lesion. The patient recovered satisfactorily and is doing well after 6 months of treatment.
Figure 1: MRI of the head-and-neck area shows a large nasopharyngeal homogenously enhancing mass with intracranial extension. MRI: Magnetic resonance imaging

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Figure 2: Histopathological picture showing diffuse large B-cell non-Hodgkin's lymphoma

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Figure 3: (a and b) Immunohistological examinations shows strong positive to CD20 (×400) and Ki67

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  Discussion Top

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.[5] NHL is the most common 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 usually aggressive in nature.[6] 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 the central nervous system.[6] BL was thought to found in tropical Africa other than at higher altitudes or in the region of a relatively cool climate. BL occurs more in the area of greater rainfall.[6] The association of climatic and geographical components suggests a relation between BL and falciparum malaria.[6]

BL is of three variants such as endemic (African) form which is the most common globally followed by sporadic (American) which is commonly seen in North America and then the immunodeficiency-associated type.[7] Sporadic type and immunodeficiency type are commonly found in the abdomen and bone marrow and uncommon in the head-and-neck area.[8] However, the African type of BL is often affected nasofacial bones and nearby soft tissues and associated with the Epstein-Barr virus.[9] Around 58% of the head-and-neck cases of BL found in mandible or maxilla.[10] However, it is less common in the nose and nasopharynx, although very few have been reported in the nasal cavity and nasopharynx. In the head-and-neck area, jaw, and other facial bones are commonly affected in 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 located primarily at the breast, pleura, soft tissues, pancreas, thyroid, skin, and lung or spinal extradural area. In disseminated form of the BL may these tissues may be affected. BL can be found in HIV-positive patients as acquired immunodeficiency syndrome (AIDS) is often related to the development for NHL. BL is a rare type and prevalent in patients with AIDS. There is around 10%–34% of the NHLs seen in extranodal sites.[11] Similarly, BL in the head-and-neck area often originates as a cervical adenopathy, although it can be found in the mandible, maxilla, nasopharynx, and tonsils. The clinical features of this aggressive neoplasm are related to the site of the expansion such as jaw swelling in mandibular involvement, facial swelling, proptosis, and loosening of teeth. In our case, the child was presenting with nose block, mouth breathing, and otalgia which was confusing with adenoid hypertrophy. In this case, the patient was presenting with recurrent nasal bleeding and bilateral nasal block which confused with the diagnosis of juvenile nasopharyngeal angiofibroma.

CT scan is an important radiological investigation used to evaluate the spread and severity of the disease and staging of the tumor. CT is the best medical investigation to localize the lesion. At present, positron emission tomography/CT scans are done for functional and anatomical assessments during the staging of the tumor and after treatment follow-up.[12] MRI is helpful to rule out the soft tissue and intracranial invasion of the tumor. In this case, MRI showed a large mass at the nasopharynx with intracranial invasion through foramen ovale. Incisional biopsy and in situ hybridization are other tests helpful to get the correct diagnosis. Histopathological report shows 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.[13] In this case, immunohistochemical examinations showed strong positive to CD20 and Ki67. On the basis of the pathological findings, the differential diagnosis includes other verities of high-grade B-cell lymphoma, lymphoblastic lymphoma, leukemia, and blastoid mantle cell lymphoma.[14] Other high-grade non-lymphomatous childhood malignancies are also considered differential diagnosis such as embryonal rhabdomyosarcoma, Ewing's sarcoma, metastatic neuroblastoma, and primitive neuroectodermal tumor.[15],[16]

The definitive treatment of BL is often based on the patient's age and the location 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. Surgical treatment is often required in organic obstruction of airway or in conditions like optic nerve decompression if the orbit is affected or in the case of a diagnostic biopsy. BL dramatically responds to chemotherapies inducing regressions of the tumor and often leads to long-term remission.[11] In this case, six cycles of chemotherapy such as prednisone, cyclophosphamide, allopurinol, cytarabine, methotrexate, and dexamethasone were given. At present, 5-year survival for advanced BL in pediatric patients and young adults have increased of 2–3 times in the past three decades, from 85% to 90% with <6 months of intensive chemotherapy.[17] This is due to a better understanding of the disease biology and advancement of chemotherapeutic drugs.

  Conclusion Top

BL at the nasopharynx and skull base region is extremely rare clinical entity and the diagnosis of it is confirmed histopathological examinations and immunohistochemistry. The recurrent epistaxis with nasal obstruction in a male adolescent boy as in our case often confuses the clinician as a diagnosis of juvenile nasopharyngeal angiofibroma. The pediatrician or pediatric otolaryngologists should keep in mind about the diversity of the clinical presentations of the BL in the pediatric age group. BL is an aggressive neoplasm and often considered a serious health condition, so prompt and appropriate treatment of the disease is always required.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed

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Conflicts of interest

There are no conflicts of interest.

  References Top

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  [Figure 1], [Figure 2], [Figure 3]


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