bony manifestations of the langerhans cell histiocytosis: a ......bony manifestations of the...
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CONCLUSION
Histiocytic disorders have a wide spectrum of radiologic appearances. Although many imaging appearances are nonspecific, familiarity with and knowledge of these disorders will facilitate their inclusion in the differential diagnosis. Imaging findings that are more specific include
lytic skull lesions with beveled edges, vertebra plana and patchy medullary osteosclerosis. Radiology plays a large role in the diagnosis and follow-up of patients with histiocytic diseases. Familiarity with the typical imaging appearances of histiocytic disorders is crucial for
appropriate patient management. Sixty percent of our patients had a solitary bone abnormality, and 40% of the patients had multiple lesions. Thus this data is comparable to that reported in the literature.
Bony manifestations of the Langerhans Cell Histiocytosis: A pictorial ReviewHafsa Shahwaiz Babar*, Anis ur Rehman*, Imran Khalid Niazi* *Shaukat Khanum Memorial Cancer Hospital and Research Centre, Radiology, Lahore, Pakistan
LEARNING OBJECTIVES
To discuss and illustrate the bony
manifestations of Langerhans cell
histiocytosis, as observed in different
imaging modalities.
To educate about wide spectrum of
radiologic appearances of histiocytic
disorders.
The study will also compare the frequency
of the various manifestations found in our
patients with those reported in the medical
literature.
BACKGROUND
Langerhans cell histiocytosis (LCH) describes
a group of syndromes that share the common
pathologic feature of infiltration of involved
tissues by Langerhans cells. Bony
manifestations of Langerhans cell
histiocytosis (LCH) are underestimated in
frequency and diversity.
Patients with apparently restricted LCH
require careful staging to rule out a more
extensive process. The clinical course is
generally benign, and sponta- neous
remissions are common. Single bone lesions
tend to resolve spontaneously over months on
years. Biopsy, performed for diagnosis, may
initiate healing with or without curettage.
When there is only lymph node involvement,
the prognosis is favorable and most patients
recover without therapy.
IMAGING FINDINGS
Bone lesions are found in most
patients with LCH, and painful
swelling is the most common initial
sign. The skull is most often
affected, followed in frequency by
the long bones of the upper
extremities and then the flat bones
(rib, pelvis, and vertebrae). There
may be adjacent soft-tissue
swelling. Older children and young
adults are more often affected.
0
10
20
30
40
50
60
skull pelvis femur ribs humerus mandible spine
TABLE 1. SITE DISTRIBUTION
Site %age
SKULL 50
PELVIS 23
FEMUR 17
RIBS 8
HUMERUS 7
MANDIBLE 7
SPINE 3
Fig 1: Huge lytic lesion with extra-osseous soft tissue component in the right iliac bone with uptake on PET Scan.
Fig 3 Lytic lesion
in the left pedicle
with uptake on
PET scan.
Fig 2. AP and lateral views of the right
femur shows a displaced fracture with an
underlying lesion.
Fig 4. Multiple lucenciies
involving the distal right
femoral diaphysis.
Fig 5. Axial and Coronal views of CT scan showing a
lytic lesion with irregular margins in the mandible on
right.
Fig 1. Axial images through MRI show a soft
tissue mass occupying left mastoid with
peripheral enhancement on contrast-enhanced
sequences.