Aditya Kumar Gupta1, Abdul Wajid M1, Jagdish P Meena1, Sreedharan T ArunRaj2, Asit Mridha3, Priyanka Naranje4, Rakesh Kumar2, Rachna Seth1. 1. Division of Pediatric Oncology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India. 2. Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, India. 3. Department of Pathology, All India Institute of Medical Sciences, New Delhi, India. 4. Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi, India.
Abstract
BACKGROUND: Erdheim Chester disease (ECD) is very rare in pediatrics with no standard treatment guidelines. Here we present the case of a pediatric ECD patient who was cured with a Langerhan cell histiocytosis (LCH) directed chemotherapy protocol. AIM: The aim of the report was to publish this rare presentation of ECD in pediatrics and highlight the complete response obtained to treatment. METHODS: The details of the patient were extracted by a retrospective review of her clinical records. RESULTS (CASE): An 11 years old girl presented with fever and bone pain. On investigating she had multiple lytic bony lesions scattered throughout her skeleton. A biopsy from one of the bone lesions confirmed the diagnosis to be ECD. ECD is very rare in pediatrics and this case adds to the existing list of 11 previously reported ones. Also, worth mention is the fact that the child presented with isolated skeletal involvement in form of multiple osteolytic lesions. The child was started on the LCH-III protocol on which she achieved a cure. CONCLUSION: Lytic bone lesions in a child may be present in ECD. A subset of ECD may have good response to LCH like chemotherapy.
BACKGROUND:Erdheim Chester disease (ECD) is very rare in pediatrics with no standard treatment guidelines. Here we present the case of a pediatric ECDpatient who was cured with a Langerhan cell histiocytosis (LCH) directed chemotherapy protocol. AIM: The aim of the report was to publish this rare presentation of ECD in pediatrics and highlight the complete response obtained to treatment. METHODS: The details of the patient were extracted by a retrospective review of her clinical records. RESULTS (CASE): An 11 years old girl presented with fever and bone pain. On investigating she had multiple lytic bony lesions scattered throughout her skeleton. A biopsy from one of the bone lesions confirmed the diagnosis to be ECD. ECD is very rare in pediatrics and this case adds to the existing list of 11 previously reported ones. Also, worth mention is the fact that the child presented with isolated skeletal involvement in form of multiple osteolytic lesions. The child was started on the LCH-III protocol on which she achieved a cure. CONCLUSION:Lytic bone lesions in a child may be present in ECD. A subset of ECD may have good response to LCH like chemotherapy.
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Authors: Aditya Kumar Gupta; Abdul Wajid M; Jagdish P Meena; Sreedharan T ArunRaj; Asit Mridha; Priyanka Naranje; Rakesh Kumar; Rachna Seth Journal: Cancer Rep (Hoboken) Date: 2020-10-06
Authors: Aditya Kumar Gupta; Abdul Wajid M; Jagdish P Meena; Sreedharan T ArunRaj; Asit Mridha; Priyanka Naranje; Rakesh Kumar; Rachna Seth Journal: Cancer Rep (Hoboken) Date: 2020-10-06