Paget’s disease of bone: Report of 11 cases

Table 1 Clinicopathological data of 11 patients with Paget’s disease of bone

	Sex	Age (yr)	Chief complaints	X-ray findings	Bone scintigraphy findings	Surgery	Pathology findings	ALP (IU/L)
1	Male	34	Painless mass on right mandible	An area of high-density bone sclerosis was seen on the right mandible measuring 2.5 cm × 2.5 cm × 2 cm	NA	Mandibular mass resection	Abnormal bone hyperplasia accompanied by fibrous hyperplasia	400
2	Male	46	Right hip joint pain and outward deformity of the right lower limb	Low density in the right femoral head was observed, with sparse patchy cystic areas. The articular surface was fuzzy with a narrowed joint space	NA	Lesion removal from the right hip joint	Mosaic appearance in bone trabeculae resulting from irregular cement lines	505
3	Male	60	Systematic bone pain with right humerus fracture	The diploic structure of the cranial bones was unclear. Bilateral femoral shafts were enlarged and deformed, with periosteal hyperplasia and ossification; the marrow cavity was irregular, and patchy, low-density areas were observed in cortical bone	Symmetrical, increased radionuclide uptake in the skull, bilateral ribs, clavicles, humeri, tibiae and femurs	NA	NA	1671
4	Male	65	Bone pain and deformity of the left lower limb	The middle part of the left femur was bent, the bone cortex was thickened, and there were patches of low-density areas in the bone marrow cavity	NA	NA	NA	277
5	Female	48	Left lower limb pain for 16 yr, with deformity for 14 yr	The cortex of the left femur was thickened, with the marrow cavity narrowed, and a sabre-like deformation was detected in the left femur	NA	NA	NA	248
6	Male	47	Constant pain in the right hip	Multiple cystic, low-density areas in the right ilium, ischia, and pubis	Abnormal, increased radionuclide uptake in the skull, spine and pelvis	CT-guided percutaneous biopsy of the right iliac bone	Obvious proliferation of localized matrix fibers in the bone paratrabecular area, with osteoclasts and osteoblasts aggregated	457
7	Female	66	Lumbar and lower limb pain with kyphosis	The pelvic bones and bilateral upper femur had multiple radiolucent spaces, with uneven bone density	Systemic osteolytic changes	Percutaneous biopsy of the left upper femur	Irregular trabeculae and hyperplasia of bone fibrous connective tissue	817
8	Male	42	Pain, deformity and limited mobility in the left knee	Diffuse bone lesions in the left femur, tibia, and fibula with thickened bone cortex and uneven density in the marrow cavity were observed	Diffuse, increased radionuclide uptake in the skull, left scapula, right fifth anterior rib, and right hemipelvis and limb bones	NA	NA	1212
9	Male	39	Pain and deformity of the left calf	The anterior arch of the left tibia was deformed and demonstrated disordered bone	NA	Percutaneous biopsy of the left tibia	Hyperplastic and disordered lamellar bone tissue of the left tibia and irregular broad trabeculae with fibrous vascular tissue	203
10	Male	37	Left hip pain for 16 yr, with claudication for 1 yr	Multiple uneven density bone lesions were observed in the middle and upper right humeri, left femur, and pelvis, showing multiple cystic radiolucent spaces, a thickened bone cortex, a narrowed marrow cavity, and a sabre-like deformation of the femur	Increased radionuclide uptake in the 11 thoracic vertebrae, pelvis, right humerus, and left femur	NA	NA	312
11	Female	52	Right maxillary mass	Expansion of the sphenoid bone, bilateral maxillae, cheekbones, temporal bones, and occipital bone was observed, and the diploic structure of the cranial bones was unclear	NA	Right maxillary mass resection	Mosaic appearance caused by randomly arranged cement lines and multinuclear osteoclasts	699

ALP: Alkaline phosphatase; NA: Not available; CT: Computed tomography.

Table 2 Medical treatment and follow-up for the 11 patients with Paget’s disease of bone

	Medical treatment	Follow-up	Symptoms	ALP (posttreatment IU/L)
1	NA	1 yr	No symptom	NA
2	Three cycles of etidronate sodium 200 mg administered twice a day orally for 2 wk and withdrawn for 11 wk	10 yr	Bone pain relieved; mobility of the affected limb improved after surgery	311
3	Pamidronate bisphosphonate 15 mg/week intravenous injection for 7 wk	3 mo	Bone pain relieved	1398
4	Three cycles of etidronate sodium 200 mg administered twice a day orally for 2 wk and withdrawn for 11 wk	19 yr	Bone pain relieved	212
5	Alendronate 10 mg once a day orally for 7 mo	15 yr	Bone pain relieved	158
6	Pamidronate bisphosphonate 15 mg/week intravenous injection for 7 wk	3 yr	Bone pain relieved	245
7	Alendronate 10 mg once a day orally for 7 mo	3 yr	Bone pain relieved	752
8	Zoledronate 5 mg per year intravenous injection for 3 yr	5 yr	Bone pain relieved	977
9	Zoledronate 5 mg per year intravenous injection for 3 yr	3 yr	Bone pain relieved	60
10	Zoledronate 5 mg per year intravenous injection for 2 yr	2 yr	Bone pain relieved	52
11	Pamidronate bisphosphonate 15 mg/week intravenous injection for 7 wk	1 yr	No symptom	405

ALP: Alkaline phosphatase; NA: Not available.