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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

Juvenile xanthogranuloma. Foamy histiocytes aggregates with classic teuton giant cells embedded.
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f1-cmo-1-2011-157: Juvenile xanthogranuloma. Foamy histiocytes aggregates with classic teuton giant cells embedded.

Mentions: Biopsy of the neck mass revealed a histiocytic lesion; the lesional cells were mostly polygonal (some with quite visible vacuolation) with indistinct borders, plus abundant classic Touton giant cells. Scattered inflammatory cells, mostly lymphocytes, were observed as well. Focal areas showed significant hemosiderin deposits (Figure 1–3). Entrapped nerve bundles and large caliber blood vessels were noted. The staining pattern of lesion cells was as follows: S100 negative, CD1a negative; CD68 positive, cytoplasmic, alpha-1 antitrypsin positive, cytoplasmic, lysozyme positive (strong, patchy, and cytoplasmic). Additional stains included: CD34, CD35, CD21, and CD20 negative; CD4 positive membranous; factor XIIIa strong positive; fascin positive; and cytoplasmic (Figure 4). The diagnosis of juvenile xanthogranuloma was established.


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

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

Juvenile xanthogranuloma. Foamy histiocytes aggregates with classic teuton giant cells embedded.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

f1-cmo-1-2011-157: Juvenile xanthogranuloma. Foamy histiocytes aggregates with classic teuton giant cells embedded.
Mentions: Biopsy of the neck mass revealed a histiocytic lesion; the lesional cells were mostly polygonal (some with quite visible vacuolation) with indistinct borders, plus abundant classic Touton giant cells. Scattered inflammatory cells, mostly lymphocytes, were observed as well. Focal areas showed significant hemosiderin deposits (Figure 1–3). Entrapped nerve bundles and large caliber blood vessels were noted. The staining pattern of lesion cells was as follows: S100 negative, CD1a negative; CD68 positive, cytoplasmic, alpha-1 antitrypsin positive, cytoplasmic, lysozyme positive (strong, patchy, and cytoplasmic). Additional stains included: CD34, CD35, CD21, and CD20 negative; CD4 positive membranous; factor XIIIa strong positive; fascin positive; and cytoplasmic (Figure 4). The diagnosis of juvenile xanthogranuloma was established.

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