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Neonatal systemic juvenile xanthogranuloma with an ominous presentation and successful treatment.

Fan R, Sun J - Clin Med Insights Oncol (2011)

Bottom Line: The patient presented with blue muffin rush, cervical mass, bone destruction, lung nodule, hepatosplenomegaly, and coagulopathy and was successfully treated with Langerhans cell histiocytosis (LCH) based chemotherapy treatment.Similar cases in literature were reviewed and it seems that JXG, a relatively benign entity, when presented in its systemic form with liver involvement, could have an aggressive course and portend quite poor prognosis.Challenges and special consideration of the diagnosis, treatment, and future case observation are discussed.

View Article: PubMed Central - PubMed

Affiliation: Riley Hospital for Children at Indiana University Health Department of Pathology 702 Barnhill Drive, Room 2536 Indianapolis, Indiana, USA.

ABSTRACT
This case report originated from a case of neonatal multisystemic juvenile xanthogranuloma (JXG). The patient presented with blue muffin rush, cervical mass, bone destruction, lung nodule, hepatosplenomegaly, and coagulopathy and was successfully treated with Langerhans cell histiocytosis (LCH) based chemotherapy treatment. Similar cases in literature were reviewed and it seems that JXG, a relatively benign entity, when presented in its systemic form with liver involvement, could have an aggressive course and portend quite poor prognosis. Challenges and special consideration of the diagnosis, treatment, and future case observation are discussed.

No MeSH data available.


Related in: MedlinePlus

The liver ultrasound image showed multiple hypoechoic lesions.
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Related In: Results  -  Collection


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f5-cmo-1-2011-157: The liver ultrasound image showed multiple hypoechoic lesions.

Mentions: Diagnostic workup with abdominal ultrasound initially showed hepatomegaly with multiple hypoechoic lesions in the liver along with splenomegaly and ascites (Figure 5). Several CT scans confirmed these findings and found no other masses in the abdomen. Evaluation of the chest revealed a single right middle lobe nodule. Evaluation of the lymph node showed a mass adjacent to the left posterior cervical musculature and there was suggestion of destruction of the adjacent occipital bone and possible intracranial extension. MRI of the brain showed destruction of the left occiput with likely lesion of the left sigmoid sinus and abutment of the left internal carotid artery with encroachment of the spinal foramen without loss of flow in the artery or extension into the spinal cord. The bone marrow biopsy revealed a normocellular marrow with a decreased left shift. Other tests performed for these masses, including HVA and VMA, were all normal. The TORCH workup was negative. The differential for these various lesions, including the blueberry muffin spots, which were thought to be either in situ extramedullary hematopoiesis or superficial tumor metastases, included neuroblastoma and congenital leukemia.


Neonatal systemic juvenile xanthogranuloma with an ominous presentation and successful treatment.

Fan R, Sun J - Clin Med Insights Oncol (2011)

The liver ultrasound image showed multiple hypoechoic lesions.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC3117631&req=5

f5-cmo-1-2011-157: The liver ultrasound image showed multiple hypoechoic lesions.
Mentions: Diagnostic workup with abdominal ultrasound initially showed hepatomegaly with multiple hypoechoic lesions in the liver along with splenomegaly and ascites (Figure 5). Several CT scans confirmed these findings and found no other masses in the abdomen. Evaluation of the chest revealed a single right middle lobe nodule. Evaluation of the lymph node showed a mass adjacent to the left posterior cervical musculature and there was suggestion of destruction of the adjacent occipital bone and possible intracranial extension. MRI of the brain showed destruction of the left occiput with likely lesion of the left sigmoid sinus and abutment of the left internal carotid artery with encroachment of the spinal foramen without loss of flow in the artery or extension into the spinal cord. The bone marrow biopsy revealed a normocellular marrow with a decreased left shift. Other tests performed for these masses, including HVA and VMA, were all normal. The TORCH workup was negative. The differential for these various lesions, including the blueberry muffin spots, which were thought to be either in situ extramedullary hematopoiesis or superficial tumor metastases, included neuroblastoma and congenital leukemia.

Bottom Line: The patient presented with blue muffin rush, cervical mass, bone destruction, lung nodule, hepatosplenomegaly, and coagulopathy and was successfully treated with Langerhans cell histiocytosis (LCH) based chemotherapy treatment.Similar cases in literature were reviewed and it seems that JXG, a relatively benign entity, when presented in its systemic form with liver involvement, could have an aggressive course and portend quite poor prognosis.Challenges and special consideration of the diagnosis, treatment, and future case observation are discussed.

View Article: PubMed Central - PubMed

Affiliation: Riley Hospital for Children at Indiana University Health Department of Pathology 702 Barnhill Drive, Room 2536 Indianapolis, Indiana, USA.

ABSTRACT
This case report originated from a case of neonatal multisystemic juvenile xanthogranuloma (JXG). The patient presented with blue muffin rush, cervical mass, bone destruction, lung nodule, hepatosplenomegaly, and coagulopathy and was successfully treated with Langerhans cell histiocytosis (LCH) based chemotherapy treatment. Similar cases in literature were reviewed and it seems that JXG, a relatively benign entity, when presented in its systemic form with liver involvement, could have an aggressive course and portend quite poor prognosis. Challenges and special consideration of the diagnosis, treatment, and future case observation are discussed.

No MeSH data available.


Related in: MedlinePlus