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Rare Brain Cancer: ALK-Positive Lymphoma Case

Rare Pediatric Lymphoma Case: Presentation and Outcome

Bucharest — may 9, 2024 — A recent case study explores a rare instance of pediatric lymphoma, specifically anaplastic large cell lymphoma (ALCL), in a 12-year-old boy. The study describes the unusual presentation as an intradural extramedullary mass. The patient experienced specific symptoms, including pain, weakness, and hypertension, which led to intensive inquiry. The case also details the treatment protocol that was followed, and the successful outcome of the treatment, offering insights for medical professionals, and highlighting effective management going forward.

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Rare Pediatric Lymphoma Case: A Unique Presentation and Successful Outcome

A recent case study highlights the unusual presentation and successful treatment of anaplastic large cell lymphoma (ALCL) in a 12-year-old boy. ALCL, a rapidly growing hematological malignancy, typically manifests with systemic symptoms. However, this case presented as an isolated intradural extramedullary mass, a rare occurence in pediatric patients.

The Unusual Presentation

The patient initially presented with a two-week history of left iliac fossa and presacral pain radiating to his lower limbs. This was accompanied by emesis and constipation. Subsequently, he developed poorly controlled hypertension and progressive lower limb weakness. These symptoms led to an intensive diagnostic investigation.

Did you know? ALCL accounts for 10% to 15% of pediatric lymphomas, but primary central nervous system (CNS) involvement is rare, occurring in less than 5% of cases.

Imaging revealed an intradural extramedullary mass at the L1 level. Pathological analysis confirmed large, atypical cells consistent with ALK-positive ALCL.The location and isolated nature of the mass made this case particularly unique.

Diagnostic Challenges

the atypical presentation posed significant diagnostic challenges. According to the case study, the constellation of symptoms initially led to more common differential diagnoses, such as infectious processes or benign spinal tumors. This underscores the importance of considering rare conditions like primary CNS lymphomas, even in the absence of typical systemic involvement.

Treatment Protocol and Outcome

The patient’s oncological management followed the Berlin-Frankfurt-Munich ALCL-99 cooperative group protocol.This protocol includes high-dose systemic methotrexate and intrathecal chemotherapy. the results were promising.

Eighteen months of posttreatment follow-up revealed complete remission without evidence of tumor recurrence, highlighting the success of this protocol-based management.
Case Study Report

Pro Tip: Early diagnosis and adherence to standardized treatment protocols are crucial for favorable outcomes in ALCL cases with CNS involvement.

Incidental Findings and Diagnostic Accuracy

An incidental finding of a pelvic ectopic kidney,initially misinterpreted as a nodal conglomerate,further complicated the diagnostic process. this highlights the necessity of meticulous imaging evaluation to avoid diagnostic errors that could affect treatment and prognosis.

The Rarity of the Case

The authors emphasize the rarity of this presentation in the existing medical literature.

A review of the literature reveals that this type of presentation is extremely rare, making this case a valuable contribution to the existing knowledge of ALCL.
Case Study Report

Conclusion

This case underscores the importance of considering primary CNS lymphomas in pediatric patients presenting with intradural masses, even without nodal or systemic involvement. The successful outcome highlights the effectiveness of standardized treatment protocols in managing this rare condition.

Frequently Asked Questions (FAQ)

What is Anaplastic Large Cell Lymphoma (ALCL)?
ALCL is a rare and aggressive type of non-Hodgkin lymphoma.
How common is ALCL in children?
ALCL accounts for 10% to 15% of pediatric lymphomas.
What are the typical symptoms of ALCL?
Typical symptoms include painless masses, fever, weight loss, and night sweats.
What is the survival rate for ALK-positive ALCL?
Patients with ALK-positive subtypes have a 5-year survival rate of 70% to 80%.
What is the Berlin-Frankfurt-Munich ALCL-99 protocol?
It is indeed a treatment protocol that includes high-dose systemic methotrexate and intrathecal chemotherapy.

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