|Year : 2020 | Volume
| Issue : 1 | Page : 32-34
Sporadic form of isolated orbital Burkitt lymphoma presenting with fulminant proptosis and dystopia
Department of Ophthalmology, College of Medicine, Qassim University, Buraidah, Qassim, Saudi Arabia
|Date of Submission||28-Dec-2018|
|Date of Decision||15-Jul-2019|
|Date of Acceptance||16-Oct-2019|
|Date of Web Publication||6-Mar-2020|
Department of Ophthalmology, College of Medicine, Qassim University, P.O. Box 6655, Buraidah 51452, Qassim
Source of Support: None, Conflict of Interest: None
This is a rare case report of fulminant proptosis with dystopia caused by isolated orbital involvement of nonendemic Burkitt lymphoma. We present the case of a 6-year-old Egyptian child who suffered from progressive, painful proptosis and dystopia in his left eye for 10 days. We discovered a noncompressible, soft-tissue mass at the superotemporal part of the orbit. The presence of an orbital inflammatory pseudotumor was suspected when the patient was administered with systemic corticosteroids and antibiotics. Histopathological examination of the obtained specimen revealed the “starry-sky” appearance of histiocytes, which is characteristic of Burkitt lymphoma. The patient underwent chemotherapy with remarkable improvement of his dystopia and proptosis. Although orbital Burkitt lymphoma is a rare tumor, ophthalmologists should be aware of its differential diagnosis of rapidly progressive proptosis with dystopia in children, which may initially masquerade as orbital cellulitis or a pseudotumor.
Keywords: Burkitt lymphoma, dystopia, proptosis
|How to cite this article:|
Al-Mohaimeed M. Sporadic form of isolated orbital Burkitt lymphoma presenting with fulminant proptosis and dystopia. J Clin Ophthalmol Res 2020;8:32-4
|How to cite this URL:|
Al-Mohaimeed M. Sporadic form of isolated orbital Burkitt lymphoma presenting with fulminant proptosis and dystopia. J Clin Ophthalmol Res [serial online] 2020 [cited 2020 Mar 29];8:32-4. Available from: http://www.jcor.in/text.asp?2020/8/1/32/280219
Burkitt lymphoma is a rapidly growing, aggressive malignancy. It is characterized as a high-grade non-Hodgkin's lymphoma of B-cell origin. As a mandibular malignancy, Burkitt lymphoma was initially described in African children, in 1958. It is the first human tumor described to be caused by a virus, the Epstein–Barr virus (EBV). The overexpression of the c-MYC oncogene occurs due to translocation, which in turn disrupts its usual function in controlling cell growth and proliferation. The endemic form is the most common form with a geographic distribution like endemic falciparum malaria. The disease mostly occurs in low, warm, and humid lands and almost always found to be associated with EBV infection. It mostly affects the jaw and other facial bones, distal ileum, cecum, kidney, ovaries, or breast. The incidence of immunodeficiency-associated form is similar to that of nonendemic form but is considered clinically more aggressive as it is frequently associated with the invasion of the central nervous system. Among human neoplasms, Burkitt's lymphoma has the fastest cell doubling time, thus creating special challenges for diagnosis and treatment. In this case report, we present a rare case of fulminant proptosis with dystopia caused by isolated orbital involvement of nonendemic Burkitt lymphoma.
| Case Report|| |
A 6-year-old Egyptian child was reported with a chief complaint of rapidly progressive, painful proptosis and dystopia in his left eye for 10 days [Figure 1]a. The patient was not immunologically challenged. During the examination, a noncompressible soft-tissue mass was felt at the superotemporal part of the orbit without pulsation and thrill. A down-and-in displacement (3 mm) of the globe was detected along with a down-and-in proptosis (2 mm). The pupil was reactive to direct and consensual light, and the fundus was normal in both eyes. Visual acuity was 6/6 in both eyes, and extraocular muscle motility was full in all directions.
|Figure 1: (a) (Up) A 6-year-old child presenting with rapidly progressive, painful proptosis and dystopia in his left eye. (b) (Down) Improvement of dystopia, proptosis, and ocular motility following combination chemotherapy|
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A computed tomography scan revealed a homogeneous lump in the upper-lateral part of the left orbit, with the lesion inseparable from the lacrimal gland. In addition, a bilateral maxillary sinusitis with no bone destruction was detected [Figure 2]a and [Figure 2]b, suggesting the presence of an orbital inflammatory pseudotumor. No other focus of the same lymphoma in other parts of the body was observed. Thus, systemic corticosteroids and antibiotics were prescribed for the patient.
|Figure 2: Orbital computed tomography scan showing extensive homogenous soft-tissue mass seen in the upper-lateral part of the left orbit. The lesion is inseparable from the lacrimal gland with no bone destruction in addition to bilateral maxillary sinusitis (arrows). (a) (Up) The coronal plane. (b) (Down) The axial plane|
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After 5 days of such treatment, the patient's condition remarkably deteriorated. Visual acuity became 6/24 in the left eye with the limitation of ocular motility, especially on attempted abduction. Displacement and proptosis in the left eye increased to 7 mm and 5 mm, respectively. Left upper eyelid ecchymosis appeared, but no mitosis was observed.
Tumor debulking with excision biopsy was performed through transcutaneous anterior orbitotomy. Histopathological examination of the obtained specimen revealed “starry sky” appearance, with abundant clear cytoplasm and the presence of multiple, monotonous, medium-sized, highly mitotic lymphoid cells with vesicular basophilic nuclei and prominent nucleoli, which is characteristic of Burkitt's lymphoma [Figure 3]. Immunohistochemical staining for CD20 (a marker of B-cell differentiation) was positive for the tumor cells, thus confirming the diagnosis. His chemotherapy regimen included two cycles of cyclophosphamide, vincristine, prednisolone, and doxorubicin, resulting in remarkable improvement of his dystopia, proptosis, and ocular motility [Figure 1]b. No regional or distant lymphadenopathies were observed. Visual acuity stayed stable at 6/24 due to exposure keratitis. The child remained in remission for >1.5 years of follow-up.
|Figure 3: Histopathology of the debulking excision biopsy revealing the “starry sky” appearance of Burkitt lymphoma (H and E, ×200) (arrow)|
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| Discussion|| |
The nonendemic form of Burkitt's lymphoma is a rare aggressive malignancy, which is less frequently associated with EBV infection, compared to the endemic form, and is typically presented as a painful abdominal mass in children. The mean age of the onset of Burkitt lymphoma is about 12 years, and it is very rare in adults, but the incidence increases at the second and third decades of life. The male-to-female ratio (3.5:1) varies depending on the geographical areas. A few previous case reports have described nonendemic Burkitt lymphoma as an initially masquerading condition that can resemble several different conditions, including conjunctival mass, Tolosa–Hunt syndrome, an orbital pseudotumor, and advanced retinoblastoma.,, Shields et al. reviewed 1264 patients with orbital masses, out of which, only one case of Burkitt lymphoma was discovered. In our case report, we have described a case of the sporadic form of isolated orbital Burkitt lymphoma with a fulminant presentation involving painful proptosis and dystopia in a 6-year-old child, which was initially masqueraded as an orbital pseudotumor. Because ocular involvement is rare with Burkitt lymphoma, misdiagnosis typically occurs. As a consequence, patients with Burkitt lymphoma are often mistakenly suspected of having infectious and inflammatory diseases of the orbit and are initially administered antibiotics and/or steroids. However, this type of treatment leads to transient improvement or none. The progressive growth over a 2-week period that was observed in the present study was highly suggestive of Burkitt lymphoma – one of the fastest growing malignancies in humans.
However, this lymphoma is curable in many patients who receive good supportive care, which may involve undergoing aggressive forms of chemotherapy (with drugs such as cyclophosphamide, doxorubicin, vincristine, methotrexate, ifosfamide, cytarabine, and etoposide), surgical resection, monoclonal antibody-targeted therapy (with rituximab), radiotherapy, and bone marrow or stem cell transplantation. According to the extremely high mitotic index of Burkitt lymphoma (cell doubling time of 24–26 h), it is necessary to keep the time period between diagnosis and identification of stage and the initial treatment as short as possible. A cure can be achieved in about 80%–90% of those with localized disease and in more than half of the children with a disseminated disease. Other treatment options, such as immunotherapy, radiation therapy, and surgical debulking of a tumor, may help improve the prognosis, especially if combined with the recently developed aggressive combination chemotherapy.
In conclusion, despite the rare occurrence of the nonendemic form of orbital Burkitt lymphoma, ophthalmologists should be aware of its differential diagnosis of rapidly progressive proptosis with dystopia in children, which may initially masquerade as orbital cellulitis or a pseudotumor.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]