Tumoral Calcium Pyrophosphate Dihydrate Crystal Deposition DiseaseA Clinicopathologic Analysis of Five Cases

Abstract

We describe five cases of tumoral calcium pyrophosphate dihydrate crystal deposition disease (CPPDCD) and discuss the clinical, radiological and pathological features. Patients included 4 males and 1 female, ranging in age from 49 to 70 years (median, 63 yrs). The wrist was involved in two patients. The thumb, palmar aspect of the proximal phalanx of the middle finger and dorsum of the carpal bone of the hand were involved in one patient each. In one patient, a preoperative diagnosis of chondrosarcoma had been made. Macroscopically, the lesion was a circumscribed whitish-gray mass with a more or less chalky appearance, measuring between 1.0 to 6.2 cm (median, 2.5 cm). Histologically, all five lesions contained areas of calcification with crystal deposits and chondroid metaplasia. The majority of crystals were rhomboid in shape, characteristic of CPPD, but some needle-shaped crystals were also identified, which resembled urate crystals. A review of the 54 reported cases of tumoral CPPDCD including our series indicated that they could be divided into two categories based on anatomic location: central (head and neck) type (n = 33) and distal (extremity) type (n = 21). Patients of these two groups were not different with respect to age and gender, but those with the central type often presented with a painful mass (15 patients, 46%), or neurological disturbances (11 patients, 33%). Patients with the distal type presented with a painless mass or swelling (12 patients, 57%), but none had neurological signs, although 8 (38.1%) presented with acute attack similar to tophaceous gout. Tumoral CP-PDCD should be differentiated from tophaceous gout, tumoral calcinosis, and malignant or benign tumors.

Calcium pyrophosphate dihydrate deposition disease of the filum terminale

“…Calcium pyrophosphate dihydrate crystal deposition disease (CPPDD) is also known as ''pseudogout'', for the first description of gout-like symptoms without sodium urate crystals and as ''chondrocalcinosis'' for the characteristic multiple foci of calcification in hyaline and fibrocartilage of the joints and intervertebral discs [2].…”

“…Although diffuse or non-tumoral deposition of CPPD involving large articular joints and para-articular tissues represents the most frequent clinical form, focal, massive and tumoral CPPDD have been also described in these tissues [2,4,7,8].…”

“…Clinically, it has a wide range of manifestations, from asymptomatic to severely destructive arthropathy or conditions simulating a neoplasm depending on the site of the focal localization. Tumoral CPPDD occurs in patients with a mean age of 60 years and mostly in females [2].…”

“…b The arachnoid was then opened, revealing the presence of the first calcified mass. c, d The two calcified lesions appeared deeply hidden among the nerve roots of the cauda equine and attached to the filum terminale by a vascular pedicle (arrow), which was coagulated and cut (e) hydroxyapatite [2]. The crystals of CPPD are 2-40 lm with rhomboid shape, but small-long rods and even squares may be seen; they appear birefringent with polarized light.…”

“…Calcium pyrophosphate dihydrate crystal deposition disease (CPPDD) is a rare benign inflammatory joint disorder characterized by the presence of calcium pyrophosphate dihydrate crystal in the interarticular and periarticular tissue [1,2]. Patients affected by CPPDD often present a mono or oligoarthropathy, generally involving knees, wrists, metacarpophalangeal joints, pubic symphysis and hip; tophaceous pseudogout (tumoral or massive CPPDD) is one of its rarest clinical forms characterized by focal deposition of CPPD [1,[3][4][5].…”