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Presented by Edward McCarthy, M.D. and prepared by Lynette S. Nichols, M.D.
Case 6: A 40 year old man had a swelling over the distal portion of his thumb for many years.
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1. Question
Week 100: Case 6
A 40 year old man had a swelling over the distal portion of his thumb for many years. Radiographs demonstrate an extensive radiodense deposits in the soft tissue around the volar aspect of the distal phalanx and DIP joint.images/Lyn’s/Mc-7-22-02.6.a.jpg
images/Lyn’s/Mc-7-22-02.6.b.jpg
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images/Lyn’s/Mc-7-22-02.6.d.jpg
images/Lyn’s/Mc-7-22-02-6.e.jpgCorrect
Answer: Tumoral calcium pyrophosphate deposition disease
Histology: Histologically, lesions show a calcium pyrophosphate in the soft tissues. There is basophilic crystalline material which is refractile under poralized light. The crystals are small rhombdoid shapes. Frequently around deposits of these crystals, there is extensive chondroid metaplasia which may lead to the misinterpretation that this is a cartilage lesion.
Discussion: Tumoral calcium pyrophosphate deposition disease is a massive deposition of calcium pyrophosphate crystals in the soft tissues. Most commonly this develops in the fingers or around the temporal mandibular joint.
The lesion is not synovial chondromatosis because of the extensive deposition of calcium pyrophosphate crystals. Although synovial chondromatosis does occur in the tendons of the hand, calcium pyrophosphate deposition is not present.
The lesion is not a chondrosarcoma, a frequent misdiagnosis. Although there may be extensive chondroid metaplasia, the presence of the calcium pyrophosphate crystals rules out a diagnosis of chondrosarcoma.
The lesion is not tumoral calcinosis. Tumoral calcinosis is caused by deposition of calcium hydroxyapatite. This form of calcification is amorphus and non crystalline. The calcium deposition in the present case is clearly crystalline.
Incorrect
Answer: Tumoral calcium pyrophosphate deposition disease
Histology: Histologically, lesions show a calcium pyrophosphate in the soft tissues. There is basophilic crystalline material which is refractile under poralized light. The crystals are small rhombdoid shapes. Frequently around deposits of these crystals, there is extensive chondroid metaplasia which may lead to the misinterpretation that this is a cartilage lesion.
Discussion: Tumoral calcium pyrophosphate deposition disease is a massive deposition of calcium pyrophosphate crystals in the soft tissues. Most commonly this develops in the fingers or around the temporal mandibular joint.
The lesion is not synovial chondromatosis because of the extensive deposition of calcium pyrophosphate crystals. Although synovial chondromatosis does occur in the tendons of the hand, calcium pyrophosphate deposition is not present.
The lesion is not a chondrosarcoma, a frequent misdiagnosis. Although there may be extensive chondroid metaplasia, the presence of the calcium pyrophosphate crystals rules out a diagnosis of chondrosarcoma.
The lesion is not tumoral calcinosis. Tumoral calcinosis is caused by deposition of calcium hydroxyapatite. This form of calcification is amorphus and non crystalline. The calcium deposition in the present case is clearly crystalline.
