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Clinical Approach Involving a Case of Pediatric Atypical Histiocytic Lesion

June 9, 2022

Clinicians from the UPMC Children’s Hospital of Pittsburgh Division of Pediatric Hematology/Oncology published a new case report in the journal Pediatric Hematology Oncology that explores the multidisciplinary diagnostic, treatment, and management approach brought to bear on a recent case involving a chronic upper eyelid lesion in a child that was ultimately found to be an atypical histiocytic lesion.

The paper’s lead author was Archana Ramgopal, DO, a third-year pediatric hematology/oncology fellow in the Division.

Dr. Ramgopal and colleagues' report chronicles the case of a male 13-year-old patient who had developed an upper eyelid growth with a five-month history that was subsequently surgically excised in the pediatric ophthalmology clinic at UPMC Children’s.

Pathological findings of the excised growth pointed toward an initial diagnosis of an atypical histiocytic neoplasm that appeared to have the characteristics of a histiocytic sarcoma.

Further laboratory testing and molecular sequencing uncovered the true nature of the lesion, a very rare form of juvenile xanthogranuloma.

Due to the rarity of these types of lesions, standardized methods of care have not been established, nor is there consensus on the identification of low-grade versus high-grade lesions that would necessitate a more aggressive treatment approach.

Because of the patient’s laboratory findings and the nonaggressive nature of the lesion as characterized by molecular sequencing and a lack of progression or recurrence, the treatment approach taken was conservative, consisting of CT surveillance at three-month intervals in addition to the original excision of the lesion.

At the one-year follow-up, the patient remained free from any kind of recurrence or secondary malignancy.

Read more about Dr. Ramgopal and colleagues’ multidisciplinary diagnostic and treatment approach in this rare case of a juvenile xanthogranuloma using the link below.


Ramgopal A, Segal J, Mukhtar S, Yu J, Picarsic J, Tersak JM, Allen SW. A Chronic Eyelid Lesion in a Child: Multidisciplinary Approach to Diagnosis, Treatment and Management of a Highly Atypical Histiocytic Lesion. Pediatr Hematol Oncol. 2022 Mar; 39(2): 180-186.