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Images in paediatrics
Periorbital chloroma mimicking raccoon eyes in a child with acute myeloid leukaemia A 6-year-old boy presented with fever, purpuric rashes and a 2-week history of reddish periorbital discolouration bilaterally. On physical examination he had periorbital swelling which was palpable and surrounded by ecchymosis (raccoon eyes) (figure 1A), sternal tenderness, and hepatosplenomegaly. Blood tests revealed a haemoglobin level of 7 g/dL, platelets 23 000 cells/mm3, and a white cell count of 17 000 cells/mm3 with 70% myeloblasts with Auer rods. Flow cytometry showed blasts positive for myeloid markers CD13, CD33, CD117 and cMPO, suggesting acute myeloid leukaemia (AML) FAB-M2. Karyotyping showed t(8,21) translocation. The patient was treated with four cycles of chemotherapy with cytarabine, daunorubicin and etoposide, and remains in remission (figure 1B). AML is a heterogeneous group of leukaemias that arise from the precursors of myeloid, monocytic, megakaryocytic and erythroid series. The clinical presentation reflects leukaemic infiltration of the bone marrow and extramedullary sites. Chloroma is a rare extramedullary presentation. It consists of immature cells of the granulocytic series. In 1811, Allen Burns first reported this as a green tumour involving the orbit.1 The characteristic green colour is derived from the enzyme myeloperoxidase (MPO). In 1966, Rappaport noted that 30% of chloromas were not green2 and renamed these tumours granulocytic sarcoma. Chloromas can manifest either at diagnosis, during treatment or during relapse, and mainly affect soft tissues, bone and the orbit. Initially, chloroma can be misdiagnosed as periorbital cellulitis, eosinophilic sarcoma, child abuse or extramedullary haematopoiesis.3 Chloromas are usually isolated and can be differentiated from extramedullary haematopoiesis, which presents with ‘blueberry muffin’ lesions, which are characteristic bluish macules or firm papules that are often generalised but may predominately affect the trunk, head and neck.4 Raccoon eyes are more commonly seen with neuroblastoma and very infrequently in acute lymphoblastic lymphoma.3 5
In AML, chloromas are observed in only 2–8% of presentations. Although they may occur with M3, M4, M5 and M7 morphology, chloromas are primarily associated with M2 morphology. The t (8, 21) translocation is the most common cytogenetic abnormality linked to chloroma, and carries a favourable prognosis. Isolated chloroma without evidence of either peripheral smear or bone marrow involvement is rare.6 One study noted that 2.5% of children with AML (24/937) presented with isolated chloroma.7 Radiotherapy can be considered for inadequate response to chemotherapy, but carries considerable toxicity. Epidural chloroma can present with spinal cord compression and requires immediate radiotherapy or surgery to preserve neurological function. However, chemotherapy alone may lead to a rapid response and obviate the need for local surgery or radiotherapy. Each case should be treated on its merits. Balaganesh Karmegaraj,1 Sneha Latha M,2 Ravindran Manipriya,2 Sowmya Vijayakumar,1 Aruna Rajendran,2 Venkatadesikalu Maljetty,1 Ramesh Samikannu,1 Julius Xavier Scott2 1
Division of Pediatrics, Rajah Muthiah Medical College and Hospital, Annamalai University, Chidambaram, Tamil Nadu, India 2 Division of Pediatric Hemato-Oncology, Sri Ramachandra University, Chennai, Tamil Nadu, India Correspondence to Dr Balaganesh Karmegaraj, Department of Pediatrics, Rajah Muthiah Medical College and Hospital, Annamalai Nagar, Chidambaram 608002, Tamil Nadu, India; [email protected] Contributors BK: prepared the manuscript; SLM: corrected the manuscript; RM: helped with article submission; SV: took the clinical pictures; AR: helped review the literature; VM: diagnosed the disease with peripheral smear; RS: suggested improvements to the manuscript; JXS: managed the case and helped review the literature. Competing interests None. Parental/guardian consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed.
To cite Karmegaraj B, Latha M S, Manipriya R, et al. Arch Dis Child 2014;99:1025. Accepted 8 May 2014 Published Online First 26 May 2014 Arch Dis Child 2014;99:1025. doi:10.1136/archdischild-2014-306149
REFERENCES 1 2
3 4 5 6
Figure 1 (A) Chloroma of both periorbital regions mimicking raccoon eyes. (B) Appearance after chemotherapy.
7
Burns A. Observation on the surgical anatomy of the head and neck. 4th edn. Baltimore, USA: Granville Sharp Pattison, 1823. Rappaport H. Tumours of the hematopoietic system. In: Sec III, Fascicle 8, Atlas of tumour pathology. Editor: Lukes and Collins. Washington, DC: Armed Forces Institute of Pathology, 1966:241–63. Timmerman R. Images in clinical medicine. Raccoon eyes and neuroblastoma. N Engl J Med 2003;349:e4. Mehta V, Balachandran C, Lonikar V. Blueberry muffin baby: a pictoral differential diagnosis. Dermatol Online J 2008;14:8. Cheng FW, Ho AC, Li CK. Raccoon eyes as presentation of lymphoblastic lymphoma in a child. Br J Haematol 2013;162:2. Isik P, Tavil B, Tunç B, et al. Extra-medullary orbital granulocytic sarcoma without bone marrow involvement: a report of two cases. Pediatr Hematol Oncol 2011;28:65–70. Reinhardt D, Creutzig U. Isolated myelosarcoma in children—update and review. Leuk Lymphoma 2002;43:565–74.
Karmegaraj B, et al. Arch Dis Child November 2014 Vol 99 No 11
1025
Downloaded from http://adc.bmj.com/ on May 24, 2015 - Published by group.bmj.com
Periorbital chloroma mimicking raccoon eyes in a child with acute myeloid leukaemia Balaganesh Karmegaraj, Sneha Latha M, Ravindran Manipriya, Sowmya Vijayakumar, Aruna Rajendran, Venkatadesikalu Maljetty, Ramesh Samikannu and Julius Xavier Scott Arch Dis Child 2014 99: 1025 originally published online May 26, 2014
doi: 10.1136/archdischild-2014-306149 Updated information and services can be found at: http://adc.bmj.com/content/99/11/1025
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Articles on similar topics can be found in the following collections Oncology (707) Immunology (including allergy) (1864) Child abuse (260) Competing interests (ethics) (155) Abuse (child, partner, elder) (103) Clinical diagnostic tests (1041) Injury (405) Journalology (207) Radiology (898) Rheumatology (461) Trauma (401)
Notes
To request permissions go to: http://group.bmj.com/group/rights-licensing/permissions To order reprints go to: http://journals.bmj.com/cgi/reprintform To subscribe to BMJ go to: http://group.bmj.com/subscribe/
Images in paediatrics
Periorbital chloroma mimicking raccoon eyes in a child with acute myeloid leukaemia A 6-year-old boy presented with fever, purpuric rashes and a 2-week history of reddish periorbital discolouration bilaterally. On physical examination he had periorbital swelling which was palpable and surrounded by ecchymosis (raccoon eyes) (figure 1A), sternal tenderness, and hepatosplenomegaly. Blood tests revealed a haemoglobin level of 7 g/dL, platelets 23 000 cells/mm3, and a white cell count of 17 000 cells/mm3 with 70% myeloblasts with Auer rods. Flow cytometry showed blasts positive for myeloid markers CD13, CD33, CD117 and cMPO, suggesting acute myeloid leukaemia (AML) FAB-M2. Karyotyping showed t(8,21) translocation. The patient was treated with four cycles of chemotherapy with cytarabine, daunorubicin and etoposide, and remains in remission (figure 1B). AML is a heterogeneous group of leukaemias that arise from the precursors of myeloid, monocytic, megakaryocytic and erythroid series. The clinical presentation reflects leukaemic infiltration of the bone marrow and extramedullary sites. Chloroma is a rare extramedullary presentation. It consists of immature cells of the granulocytic series. In 1811, Allen Burns first reported this as a green tumour involving the orbit.1 The characteristic green colour is derived from the enzyme myeloperoxidase (MPO). In 1966, Rappaport noted that 30% of chloromas were not green2 and renamed these tumours granulocytic sarcoma. Chloromas can manifest either at diagnosis, during treatment or during relapse, and mainly affect soft tissues, bone and the orbit. Initially, chloroma can be misdiagnosed as periorbital cellulitis, eosinophilic sarcoma, child abuse or extramedullary haematopoiesis.3 Chloromas are usually isolated and can be differentiated from extramedullary haematopoiesis, which presents with ‘blueberry muffin’ lesions, which are characteristic bluish macules or firm papules that are often generalised but may predominately affect the trunk, head and neck.4 Raccoon eyes are more commonly seen with neuroblastoma and very infrequently in acute lymphoblastic lymphoma.3 5
In AML, chloromas are observed in only 2–8% of presentations. Although they may occur with M3, M4, M5 and M7 morphology, chloromas are primarily associated with M2 morphology. The t (8, 21) translocation is the most common cytogenetic abnormality linked to chloroma, and carries a favourable prognosis. Isolated chloroma without evidence of either peripheral smear or bone marrow involvement is rare.6 One study noted that 2.5% of children with AML (24/937) presented with isolated chloroma.7 Radiotherapy can be considered for inadequate response to chemotherapy, but carries considerable toxicity. Epidural chloroma can present with spinal cord compression and requires immediate radiotherapy or surgery to preserve neurological function. However, chemotherapy alone may lead to a rapid response and obviate the need for local surgery or radiotherapy. Each case should be treated on its merits. Balaganesh Karmegaraj,1 Sneha Latha M,2 Ravindran Manipriya,2 Sowmya Vijayakumar,1 Aruna Rajendran,2 Venkatadesikalu Maljetty,1 Ramesh Samikannu,1 Julius Xavier Scott2 1
Division of Pediatrics, Rajah Muthiah Medical College and Hospital, Annamalai University, Chidambaram, Tamil Nadu, India 2 Division of Pediatric Hemato-Oncology, Sri Ramachandra University, Chennai, Tamil Nadu, India Correspondence to Dr Balaganesh Karmegaraj, Department of Pediatrics, Rajah Muthiah Medical College and Hospital, Annamalai Nagar, Chidambaram 608002, Tamil Nadu, India; [email protected] Contributors BK: prepared the manuscript; SLM: corrected the manuscript; RM: helped with article submission; SV: took the clinical pictures; AR: helped review the literature; VM: diagnosed the disease with peripheral smear; RS: suggested improvements to the manuscript; JXS: managed the case and helped review the literature. Competing interests None. Parental/guardian consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed.
To cite Karmegaraj B, Latha M S, Manipriya R, et al. Arch Dis Child 2014;99:1025. Accepted 8 May 2014 Published Online First 26 May 2014 Arch Dis Child 2014;99:1025. doi:10.1136/archdischild-2014-306149
REFERENCES 1 2
3 4 5 6
Figure 1 (A) Chloroma of both periorbital regions mimicking raccoon eyes. (B) Appearance after chemotherapy.
7
Burns A. Observation on the surgical anatomy of the head and neck. 4th edn. Baltimore, USA: Granville Sharp Pattison, 1823. Rappaport H. Tumours of the hematopoietic system. In: Sec III, Fascicle 8, Atlas of tumour pathology. Editor: Lukes and Collins. Washington, DC: Armed Forces Institute of Pathology, 1966:241–63. Timmerman R. Images in clinical medicine. Raccoon eyes and neuroblastoma. N Engl J Med 2003;349:e4. Mehta V, Balachandran C, Lonikar V. Blueberry muffin baby: a pictoral differential diagnosis. Dermatol Online J 2008;14:8. Cheng FW, Ho AC, Li CK. Raccoon eyes as presentation of lymphoblastic lymphoma in a child. Br J Haematol 2013;162:2. Isik P, Tavil B, Tunç B, et al. Extra-medullary orbital granulocytic sarcoma without bone marrow involvement: a report of two cases. Pediatr Hematol Oncol 2011;28:65–70. Reinhardt D, Creutzig U. Isolated myelosarcoma in children—update and review. Leuk Lymphoma 2002;43:565–74.
Karmegaraj B, et al. Arch Dis Child November 2014 Vol 99 No 11
1025
Downloaded from http://adc.bmj.com/ on May 24, 2015 - Published by group.bmj.com
Periorbital chloroma mimicking raccoon eyes in a child with acute myeloid leukaemia Balaganesh Karmegaraj, Sneha Latha M, Ravindran Manipriya, Sowmya Vijayakumar, Aruna Rajendran, Venkatadesikalu Maljetty, Ramesh Samikannu and Julius Xavier Scott Arch Dis Child 2014 99: 1025 originally published online May 26, 2014
doi: 10.1136/archdischild-2014-306149 Updated information and services can be found at: http://adc.bmj.com/content/99/11/1025
These include:
References Email alerting service
Topic Collections
This article cites 5 articles, 0 of which you can access for free at: http://adc.bmj.com/content/99/11/1025#BIBL Receive free email alerts when new articles cite this article. Sign up in the box at the top right corner of the online article.
Articles on similar topics can be found in the following collections Oncology (707) Immunology (including allergy) (1864) Child abuse (260) Competing interests (ethics) (155) Abuse (child, partner, elder) (103) Clinical diagnostic tests (1041) Injury (405) Journalology (207) Radiology (898) Rheumatology (461) Trauma (401)
Notes
To request permissions go to: http://group.bmj.com/group/rights-licensing/permissions To order reprints go to: http://journals.bmj.com/cgi/reprintform To subscribe to BMJ go to: http://group.bmj.com/subscribe/
