+ All Categories
Home > Documents > Continuation of ORTHOPAEDIC. RADIOLOGY. · PDF filethelasma (eye-lid), xanthoma (nose bridge),...

Continuation of ORTHOPAEDIC. RADIOLOGY. · PDF filethelasma (eye-lid), xanthoma (nose bridge),...

Date post: 19-Feb-2018
Category:
Upload: vanlien
View: 216 times
Download: 0 times
Share this document with a friend
1
140 Hong Kong Journal of Orthopaedic Surgery 2002;6(2):140-144. PATHOLOGY FINDINGS This patient presented with multiple skin and soft tis- sue swellings over a period of 9 years. According to their locations, the swellings were separately diagnosed as xanthofibroma (tendinous-fascia at elbow), xan- thelasma (eye-lid), xanthoma (nose bridge), and giant cell tumour of tendon sheath (finger tendon). Review of all the biopsy material showed the same disease morphology as xanthogranulomatosis (Fig. 10). There were nodular sheets of lipid-laden foamy macrophages admixed with chronic inflammatory cells and Touton- type, multinucleated, giant cells. There were varying degrees of fibrosis in the background, and eosinophils were notably absent. In the elbow mass, there were sheets of histiocytes with kidney-shaped nuclei. They stained positively on immunostudies for Langerhans cell markers, including S100 protein and CD1a (Fig. 11). This raised the possibility of Langerhans cell his- tiocytosis (LCH). As Langerhans cells were not seen in the other biopsy material, and given the fact that eosi- nophils (important accompanying cells in LCH) were absent, the possibility of LCH was excluded in this case. On the other hand, the multifocal occurrence did pin- point a systemic infiltrative disease rather than a coin- cidental occurrence of a localised disease at different sites. Xanthomatous lesions over tendons and eyelids necessitated an investigation into familial or acquired hypercholesterolaemia. This possibility was subse- quently excluded by blood tests, which revealed nor- mal cholesterol levels and lipid profiles. Without other clues, the cause of this systemic xanthogranulomatosis remained enigmatic. RADIOLOGY FINDINGS The initial radiograph of the left middle finger showed nonspecific soft tissue swelling around the proximal in- terphalangeal joint and middle phalanx. No bony ero- sion was noted. Computed tomography and MRI of the facial region were performed that showed soft tissue masses in the periorbital region and over the right na- sal bridge (Fig. 12). No retro-orbital mass was detected. Separate MRI studies of the right knee and both ankles were subsequently performed. Apart from the prepa- Continuation of ORTHOPAEDIC. RADIOLOGY. PATHOLOGY CONFERENCE from page 114 Figure 10 Xanthogranulomatous tissue containing lipid-laden foamy macrophages, chronic inflammatory cells, and Touton giant cells. Figure 11 Sheets of CD1a-positive Langerhans cells in the elbow mass. tellar mass detected on physical examination, there were actually multiple soft tissue masses around the right knee. They were of similar signal intensity, being isointense on T1-weighted images (Fig. 13) and het- erogeneously slightly hyperintense on T2-weighted images (Fig. 14). Some intralesional cystic areas were present. They were infiltrative and encased the right patellar tendon, right biceps femoris (Fig. 15), and both Achilles tendons (Fig. 16). There were extensive irregular hypointense lesions in
Transcript
Page 1: Continuation of ORTHOPAEDIC. RADIOLOGY. · PDF filethelasma (eye-lid), xanthoma (nose bridge), and giant cell tumour of tendon sheath (finger tendon). Review ... Continuation of ORTHOPAEDIC.

140

HKJOS Wong TC, Cheung FMF, Siu TH, et alHong Kong Journal of Orthopaedic Surgery2002;6(2):140-144.

PATHOLOGY FINDINGS

This patient presented with multiple skin and soft tis-sue swellings over a period of 9 years. According totheir locations, the swellings were separately diagnosedas xanthofibroma (tendinous-fascia at elbow), xan-thelasma (eye-lid), xanthoma (nose bridge), and giantcell tumour of tendon sheath (finger tendon). Reviewof all the biopsy material showed the same diseasemorphology as xanthogranulomatosis (Fig. 10). Therewere nodular sheets of lipid-laden foamy macrophagesadmixed with chronic inflammatory cells and Touton-type, multinucleated, giant cells. There were varyingdegrees of fibrosis in the background, and eosinophilswere notably absent. In the elbow mass, there weresheets of histiocytes with kidney-shaped nuclei. Theystained positively on immunostudies for Langerhanscell markers, including S100 protein and CD1a (Fig.11). This raised the possibility of Langerhans cell his-tiocytosis (LCH). As Langerhans cells were not seenin the other biopsy material, and given the fact that eosi-nophils (important accompanying cells in LCH) wereabsent, the possibility of LCH was excluded in this case.On the other hand, the multifocal occurrence did pin-point a systemic infiltrative disease rather than a coin-cidental occurrence of a localised disease at differentsites. Xanthomatous lesions over tendons and eyelidsnecessitated an investigation into familial or acquiredhypercholesterolaemia. This possibility was subse-quently excluded by blood tests, which revealed nor-mal cholesterol levels and lipid profiles. Without otherclues, the cause of this systemic xanthogranulomatosisremained enigmatic.

RADIOLOGY FINDINGS

The initial radiograph of the left middle finger showednonspecific soft tissue swelling around the proximal in-terphalangeal joint and middle phalanx. No bony ero-sion was noted. Computed tomography and MRI of thefacial region were performed that showed soft tissuemasses in the periorbital region and over the right na-sal bridge (Fig. 12). No retro-orbital mass was detected.

Separate MRI studies of the right knee and both ankleswere subsequently performed. Apart from the prepa-

Continuation of ORTHOPAEDIC. RADIOLOGY. PATHOLOGY

CONFERENCE from page 114

Figure 10 Xanthogranulomatous tissue containing lipid-ladenfoamy macrophages, chronic inflammatory cells, and Touton giantcells.

Figure 11 Sheets of CD1a-positive Langerhans cells in the elbowmass.

tellar mass detected on physical examination, therewere actually multiple soft tissue masses around theright knee. They were of similar signal intensity, beingisointense on T1-weighted images (Fig. 13) and het-erogeneously slightly hyperintense on T2-weightedimages (Fig. 14). Some intralesional cystic areas werepresent. They were infiltrative and encased the rightpatellar tendon, right biceps femoris (Fig. 15), and bothAchilles tendons (Fig. 16).

There were extensive irregular hypointense lesions in

Recommended