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Langerhans cell histiocytosis

Mitchell JM - MedPix (2015)

View Article: MedPix Image - MedPix Case

Affiliation: Unlisted Institution

ABSTRACT

Diagnosis: Langerhans cell histiocytosis

History: The patient is a 13 month old boy, with a history of prematurity at 25 weeks gestation, who presented to his primary care provider after his mother noticed a soft tissue mass over his left ear. The patient is not complaining of pain or tenderness. He has no other significant medical or surgical history.

Findings: -Head CT w IV contrast demonstrated 3 areas of bone destruction in the skull with associated enhancing soft tissue. The largest is in the right parietal bone above the squamosal suture. The additional areas of involvement include the greater wing of the sphenoid on the right and the left posterior parietal bone. -Skeletal survey revealed lytic skull lesions, L3 vertebral body completely collapsed and sclerosis, an erosive lytic lesion in the patient"s right 6 anterolateral rib along the anterior surface, and evidence of expansion of the patient"s left sixth anterolateral rib. -Bone scan revealed multiple foci of increased uptake within the skull, ribs bilaterally. There is heterogeneous uptake within the thoracic and lumbar spine which may represent small lesions as well. The area of vertebra plana at L4 is also demonstrated. -Chest AP plain film demonstrated prominent markings, at least partially due to low lung volumes. -Chest CT w IV contrast revealed multiple cystic lesions with nodules throughout both lungs and also scattered patchy areas of groundglass opacity bilaterally. There were also lytic lesions within several posterior lateral ribs bilaterally and there is vertebra plana at the level of T8.

Ddx: metastatic bone disease leukemia lymphoma multifocal osteomyelitis (less likely)

Dxhow: Biopsy and Histology

Exam: There is a soft tissue lesion superior to the left ear. Otherwise the patient is alert, active, and in no acute distress. The rest of the physical exam is within normal limits. Labs were significant for an elevated sedimentation rate and peripheral eosinophilia @ 7%. CBC, CMP, and Iron studies were within normal limits.

No MeSH data available.


The bones are well-mineralized. No radiographic evidence of rickets is seen. There are 2 lytic lesions in the calvaria (arrows). One on the superior portion of the left. Another on the lateral right with associated soft tissue edema, superior to the right auricle. No classic metaphyseal lesion is seen.
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MPX2465_synpic61283: The bones are well-mineralized. No radiographic evidence of rickets is seen. There are 2 lytic lesions in the calvaria (arrows). One on the superior portion of the left. Another on the lateral right with associated soft tissue edema, superior to the right auricle. No classic metaphyseal lesion is seen.


Langerhans cell histiocytosis

Mitchell JM - MedPix (2015)

The bones are well-mineralized. No radiographic evidence of rickets is seen. There are 2 lytic lesions in the calvaria (arrows). One on the superior portion of the left. Another on the lateral right with associated soft tissue edema, superior to the right auricle. No classic metaphyseal lesion is seen.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=MPX2465&req=5

MPX2465_synpic61283: The bones are well-mineralized. No radiographic evidence of rickets is seen. There are 2 lytic lesions in the calvaria (arrows). One on the superior portion of the left. Another on the lateral right with associated soft tissue edema, superior to the right auricle. No classic metaphyseal lesion is seen.

View Article: MedPix Image - MedPix Case

Affiliation: Unlisted Institution

ABSTRACT

Diagnosis: Langerhans cell histiocytosis

History: The patient is a 13 month old boy, with a history of prematurity at 25 weeks gestation, who presented to his primary care provider after his mother noticed a soft tissue mass over his left ear. The patient is not complaining of pain or tenderness. He has no other significant medical or surgical history.

Findings: -Head CT w IV contrast demonstrated 3 areas of bone destruction in the skull with associated enhancing soft tissue. The largest is in the right parietal bone above the squamosal suture. The additional areas of involvement include the greater wing of the sphenoid on the right and the left posterior parietal bone. -Skeletal survey revealed lytic skull lesions, L3 vertebral body completely collapsed and sclerosis, an erosive lytic lesion in the patient"s right 6 anterolateral rib along the anterior surface, and evidence of expansion of the patient"s left sixth anterolateral rib. -Bone scan revealed multiple foci of increased uptake within the skull, ribs bilaterally. There is heterogeneous uptake within the thoracic and lumbar spine which may represent small lesions as well. The area of vertebra plana at L4 is also demonstrated. -Chest AP plain film demonstrated prominent markings, at least partially due to low lung volumes. -Chest CT w IV contrast revealed multiple cystic lesions with nodules throughout both lungs and also scattered patchy areas of groundglass opacity bilaterally. There were also lytic lesions within several posterior lateral ribs bilaterally and there is vertebra plana at the level of T8.

Ddx: metastatic bone disease leukemia lymphoma multifocal osteomyelitis (less likely)

Dxhow: Biopsy and Histology

Exam: There is a soft tissue lesion superior to the left ear. Otherwise the patient is alert, active, and in no acute distress. The rest of the physical exam is within normal limits. Labs were significant for an elevated sedimentation rate and peripheral eosinophilia @ 7%. CBC, CMP, and Iron studies were within normal limits.

No MeSH data available.