ISOLATED CENTRAL NERVOUS SYTEM BURKITT'S LYMPHOMA IN ADVANCED AGE: A CASE STUDY

Abstract

Objective

Burkitt lymphoma is an aggressive type of B-cell lymphoma that is usually seen in the pediatric and young adult population and characteised with myc gene translocation. This disease manifests itself with rapidly growing abdominal mass and systemic sign and symptoms. However, atypical involvements, such as isolated cranial involvements, face both diagnostic and therapeutic challenges, especially in older age groups. Although isolated cranial involvement of Burkitt's lymphoma is rare in older patients, this case report emphasizes the challenges in clinical practice.

Case report

A 67-year-old female patient was taken with complaints of headache and vomiting in June 2022. An MRI scan revealed a mass measuring 3.3 × 2.8 × 1.5 cm in the left temporal region. Upon this finding, the patient was referred to the neurosurgery department and the mass was surgically removed. As a result of the pathological examination resulting from the operation, CD10, CD20 were diffusely positive; BCL2 negative; BCL6 positive; C-MYC 70% positive; Kİ67 is 100% positive and confirms Burkitt's Lymphoma. In the PET-CT scan performed for the staging of the patient, reticular dense growths and irregular growth FDG uptakes in ground glass density areas were observed in the medial posterobasal segment of the lower lobe of two lungs and in the anterior segment of the upper lobe of the left lung. In the mediastinal area, increased degrees of FDG uptake were detected in bilateral lower paratracheal and subcarinal lymph nodes. These findings were evaluated as a potential infectious event. While there were no findings in hemogram and biochemical pathological tests, HbsAg positivity was detected but no active disease was found.Prophylactic intrathecal(IT) treatmentwas also recommended for the disease, which started to systemic chemotherapy, but IT chemotherapy was rejected.. In subsequent MRI examinations, the defect formed after craniotomy in the left temporofrontoparietal region and fluid collection in the calvarium were observed, while no residue or recurrence was observed in the operation area.However, a lesion measuring 2 × 3 cm in size was detected in the left parietal at the vertex level, which was primarily considered a fibroma and showed marked hypointenses and heterogeneous contrast enhancement in all sections.

In the evaluation PET-CT performed after four cycles of the R-HYPERCVAD regimen, a mild increase in metabolic activity was observed in the mediastinal lymph nodes, but this was consistent with inflammatory processes, and no signs of recurrence or metastasis were found in other parts of the body. Despite these findings, which were accepted as a response to treatment, the planned OKIT treatment was not accepted by the patient and their relatives.

After completing the seventh course of treatment, the patient presented to the emergency room with altered consciousness and recurrent headaches. Antieodema treatment was applied to the patient who was diagnosed with brain edema, but the recommended advanced chemotherapy and full cranial radiotherapy were rejected. In December 2023, the patient was re-admitted with symptoms of brain edema and shingles zoster infection was observed, and the patient died after his condition worsened despite symptomatic treatment.

Conclusion

This case report highlights the rarity of advanced age Burkitt lymphoma with isolated cranial involvement and the diagnostic and therapeutic difficulties of this condition. Our patient exhibited atypical involvement of an aggressive B-cell lymphoma that usually occurs in childhood and young adults and is characterized by myc gene translocation. The disease, which usually manifests itself with an abdominal mass and systemic symptoms, is rare to show isolated cranial involvement, and this requires us to re-evaluate the diagnosis and treatment strategies in our clinical practice. In this case, although the patient's symptoms and radiological findings initially suggested a typical brain tumor, pathological examination confirmed the diagnosis of Burkitt's lymphoma. During the patient's treatment process, the importance of systemic chemotherapy and prophylactic intrathecal treatment became evident. However, rejection of various treatment options by patients and their relatives may negatively affect the effectiveness of the treatment and patient survival. This case highlights the rarity of Burkitt lymphoma with isolated cranial involvement in older age patients and the challenges and important lessons encountered in the diagnosis and treatment of these atypical presentations.