technetium-tc-99m-medronate and Fibrous-Dysplasia--Polyostotic

McCune-Albright syndrome is a rare disorder caused by a somatic, constitutively activating mutation in the gene (GNAS1) encoding the subunit of the signal transducing guanine nucleotide binding protein (G protein). The condition is characterized by polyostotic fibrous dysplasia, cafe-au-lait pigmentation and multiple endocrine hyperfunction, most commonly gonadotropin-independent precocious puberty in girls. Our patient, a 16-year-old male, with radiologically confirmed polyostotic fibrous dysplasia in cranium, thoracic and pelvic girdles, spine and extremities was studied using planar 99mTc-hydroxymethyldiphosphonate bone scintigraphy and single photon emission computed tomography. Using bone scintigraphy, an unusually extensive and asymmetric fibrous dysplasia was observed in the cranium, face, ribs, femur, humerus, ulna, tibia and the vertebral column, all on the left side. The whole body scan revealed only a few foci on the right side. Single photon emission computed tomography demonstrated extensive unilateral involvement in the base of the skull, facial bones, maxilla and mandible. All the lesions reached only the midline. These findings formed the basis of further treatment, eg. reconstructive surgery of facial asymmetry.. McCune-Albright syndrome should be considered in the differential diagnosis when interpreting extensive unilateral predominance in paediatric bone scans.

Topics: Adolescent; Bone and Bones; Cafe-au-Lait Spots; Fibrous Dysplasia, Polyostotic; Humans; Male; Radiopharmaceuticals; Technetium Tc 99m Medronate; Tomography, Emission-Computed, Single-Photon

Mazabraud's syndrome, though uncommon, is reported increasingly frequently. It represents an entity readily recognisable radiologically on MR imaging. Awareness of the syndrome, particularly when the myxoma is solitary, can prevent misdiagnosis of intramuscular myxomas (especially when large) as malignant mesenchymal tumors containing myxoid tissue. We review the 34 cases previously reported in the literature and include a recent case from our center.

Topics: Adult; Diagnosis, Differential; Femur; Fibrous Dysplasia, Polyostotic; Follow-Up Studies; Humans; Magnetic Resonance Imaging; Male; Muscle Neoplasms; Myxoma; Radiopharmaceuticals; Ribs; Sarcoma; Syndrome; Technetium Tc 99m Medronate; Thigh; Tomography, X-Ray Computed

Topics: Adult; Biopsy; Bone Cysts, Aneurysmal; Diagnosis, Differential; Elbow Joint; Female; Fibrous Dysplasia, Polyostotic; Humans; Magnetic Resonance Imaging; Radiography; Radionuclide Imaging; Technetium Tc 99m Medronate

Topics: Fibrous Dysplasia, Polyostotic; Humans; Technetium Tc 99m Medronate

Topics: Adult; Alkaline Phosphatase; Bone and Bones; Female; Femur; Fibrous Dysplasia, Polyostotic; Humans; Radiography; Radionuclide Imaging; Radiopharmaceuticals; Technetium Tc 99m Medronate

Fibrous dysplasia commonly involves the skull in both its monostotic and polyostotic variants. We present two cases of fibrous dysplasia involving the sphenoid wing, which were strikingly similar in their bone scan appearance. Both patients demonstrated intense increased uptake of Tc-99m MDP in a pattern reminding us of a "pirate wearing an eyepatch." We propose that this characteristic appearance of fibrous dysplasia of the sphenoid wing be called the "pirate sign." A review of the literature revealed several other pathologic conditions that have been reported to involve the sphenoid bone and should be considered in the differential diagnosis of abnormal bone tracer uptake in this region.

Topics: Adult; Female; Fibrous Dysplasia, Polyostotic; Humans; Male; Radionuclide Imaging; Radiopharmaceuticals; Sphenoid Bone; Technetium Tc 99m Medronate; Tissue Distribution

Topics: Acromegaly; Adult; Fibrous Dysplasia, Polyostotic; Humans; Male; Radionuclide Imaging; Radiopharmaceuticals; Technetium Tc 99m Medronate

Lesions of fibrous dysplasia involving the spine and scoliosis are thought to be uncommon entities in patients with polyostotic fibrous dysplasia and McCune-Albright syndrome. By examining bone scans of a relatively large cohort of patients with these disorders, we sought to determine the prevalence of spinal involvement and any association with scoliosis.. Sixty-two patients with polyostotic fibrous dysplasia were studied. There were twenty-three male and thirty-nine female patients, and they had a mean age of twenty-five years (range, four to eighty years). Technetium-99m-methylene diphosphonate (MDP) bone scans of the patients were evaluated for evidence of increased uptake in the spine. The presence or absence of scoliosis or a level pelvis and the distribution of other lesions in the skeleton were noted.. Thirty-nine (63%) of sixty-two patients were found to have seventy-six lesions of fibrous dysplasia in the spine. Fifty-four lesions (71%) demonstrated increased uptake in the posterior aspects of the spine. Most lesions were located in the lumbar spine (thirty-two lesions) and the thoracic spine (twenty-seven), with less frequent involvement in the sacrum (ten) and cervical spine (six). Twenty-five (40%) of the sixty-two patients had scoliosis; seventeen had a thoracolumbar curve; six, a lumbar curve; and two, a thoracic curve. Seven patients had curves that could not be accurately measured by bone scanning and, therefore, could not be classified. Thirty patients (48%) had no evidence of scoliosis. Thus, the prevalence of scoliosis in patients with polyostotic fibrous dysplasia was between 40% and 52%. There was a strong correlation between spinal lesions and scoliosis (p < 0.001) and pelvic asymmetry (p < 0.05). Back pain was an uncommon symptom. Two patients had a neurologic abnormality; neither abnormality was related to the location of the lesions or the curve.. Spinal lesions and scoliosis may be more common in patients with polyostotic fibrous dysplasia than has been previously reported. Since there is a strong correlation between a spinal lesion and scoliosis, these patients should be screened clinically for scoliosis.. Prognostic study, Level II-1 (retrospective study). See Instructions to Authors for a complete description of levels of evidence.

Topics: Adolescent; Adult; Age Distribution; Aged; Aged, 80 and over; Analysis of Variance; Anthropometry; Back Pain; Cervical Vertebrae; Child; Child, Preschool; Disease Progression; Female; Fibrous Dysplasia, Polyostotic; Humans; Lumbar Vertebrae; Magnetic Resonance Imaging; Male; Middle Aged; Prevalence; Radiopharmaceuticals; Sacrum; Scoliosis; Technetium Tc 99m Medronate; Thoracic Vertebrae

Fibrous dysplasia of bone is a congenital, sporadic developmental disorder characterized by immature fibrous connective tissue and bone deformities. Hepatic Tc-99m-MDP uptake is a rare, serendipitous finding during bone scanning studies. The present patient was a 25-year-old male who had severe polyostotic fibrous dysplasia. On Tc-99m-MDP (methylene diphosphonate) bone scintigraphy, increased activity accumulations were seen on multiple ribs, vertebrae and base of the cranium. In addition, diffuse increased pathologic uptake of Tc-99m-MDP in the liver was shown. Intravenous pamidronate was administered monthly for two months. In the third week of the last administration Tc-99m-MDP bone scintigraphy was performed again, but despite sustained bone involvement, pathologic hepatic uptake was not seen on the scan. We thought that pathologic hepatic Tc-99m-MDP accumulation, may be related to the formation and aggregation of calcium oxalate and phosphate crystals which improved with pamidromat treatment.

Topics: Adult; Chest Pain; Fibrous Dysplasia, Polyostotic; Humans; Incidental Findings; Liver; Male; Radionuclide Imaging; Radiopharmaceuticals; Technetium Tc 99m Medronate

Topics: Adult; Diagnosis, Differential; Fibrous Dysplasia of Bone; Fibrous Dysplasia, Polyostotic; Humans; Hyperparathyroidism; Male; Radionuclide Imaging; Radiopharmaceuticals; Technetium Tc 99m Medronate; Technetium Tc 99m Sestamibi; Whole-Body Counting

Topics: Adult; Bone and Bones; Diagnosis, Differential; Female; Fibrous Dysplasia, Polyostotic; Humans; Radionuclide Imaging; Radiopharmaceuticals; Technetium Tc 99m Medronate; Whole-Body Counting

A 38-year-old woman sought treatment with cranio-facial dysplasia involving the cranium, maxilla and the mandible. Her chief complaint was a mandibular swelling, which had appeared about 2 years previously, had gradually enlarged, and was associated with spontaneous pain. X-ray film examination revealed a ground-glass opacity with blurred demarcation and a 99 Tc medronate bone scan disclosed an increase in tracer uptake in the cranium, maxilla and mandible. The rest of the skeleton was not affected. Histological examination of the lesions revealed solid proliferation of spindle-shaped cells associated with islands of osteid and bone trabecullae with Chinese letter pattern and numerous multinucleated giant cells consistent with fibrous dysplasia. The continued osteoblastic activity of involved bones, coupled with the medical condition of the patient, restricted the management of the patient to largely supportive and palliative care.

Topics: Adult; Diagnosis, Differential; Facial Bones; Female; Fibrous Dysplasia, Polyostotic; Humans; Jaw Diseases; Skull; Technetium Tc 99m Medronate

A 66-year-old man affected by polyostotic form of fibrous dysplasia in consequence of worsening of lower extremity bone pain aggravated by walking and concomitant increase of serum alkaline phosphatase and osteocalcin, was subjected first to a radionuclide study. Bone scan demonstrated a pathological uptake of the radiotracer in the craniofacial bones, right scapula, left and right posterior ribs, right hemipelvis and lower extremities confirming the diagnosis but establishing especially the extent of bone involvement, greater than expected on the basis of symptoms and X-ray findings, underlying the importance of nuclear medicine imaging in the assessment and follow-up of this rare disease.

Topics: Aged; Bone and Bones; Diagnosis, Differential; Fibrous Dysplasia, Polyostotic; Humans; Male; Radiography; Radionuclide Imaging; Radiopharmaceuticals; Technetium Tc 99m Medronate

Fibrous dysplasia is a benign bone disorder. It is diagnosed by distinctive X-ray radiography, CT, and MRI findings. Although bone scintigraphy helps to identify the tumor origin according to accelerated bone turnover, the glucose metabolism in fibrous dysplasia has not yet been investigated. We reported a case of fibrous dysplasia in craniofacial bone which showed signs of the acceleration of bone mineral turnover without elevated glucose utilization by Technetium-99m-HMDP SPECT and Fluorine-18-FDG PET. We concluded that the growth of fibrous dysplasia needed the acceleration of bone mineral turnover without an increase in glucose metabolism.

Topics: Bone Density; Facial Bones; Female; Fibrous Dysplasia, Polyostotic; Fluorodeoxyglucose F18; Glucose; Humans; Hyperplasia; Middle Aged; Radiopharmaceuticals; Sclerosis; Skull; Technetium Tc 99m Medronate; Tomography, Emission-Computed, Single-Photon; Tomography, X-Ray Computed

Topics: Adult; Bone and Bones; Female; Femur; Fibrous Dysplasia, Polyostotic; Humans; Pelvic Bones; Radionuclide Imaging; Radiopharmaceuticals; Technetium Tc 99m Medronate; Tibia; Whole-Body Counting

Topics: Adult; Female; Fibrous Dysplasia, Polyostotic; Humans; Hyperparathyroidism; Kidney Failure, Chronic; Mandible; Maxilla; Parathyroid Glands; Radionuclide Imaging; Renal Dialysis; Technetium Tc 99m Medronate; Tomography, X-Ray Computed

There have been few reports in the literature concerning the long-term prognosis of fibrous dysplasia. We reported here a boy with craniofacial fibrous dysplasia, which showed marked involution at the end of his puberty.

Topics: Child; Fibrous Dysplasia, Polyostotic; Humans; Male; Maxillary Diseases; Orbital Diseases; Postoperative Period; Puberty; Technetium Tc 99m Medronate; Tomography, X-Ray Computed

Polyostotic fibrous dysplasia, a major osseous change in McCune-Albright syndrome, is seen in the cranium, facial bones, bones of the extremities, and ribs, but rarely in the spine. Spinal X-rays revealed no abnormalities in an 8-year-old girl with this syndrome, but 99mTc-methylene diphosphonate bone scintigraphy disclosed high-density areas in the thoracic and lumbar vertebrae. Multiple well-circumscribed areas of low signal intensity were seen on T1-weighted magnetic resonance imaging (MRI) of the spine. Although MRI spine scans in this disease have never been reported, our findings in this case proved interesting for evaluating osseous lesions. MRI made it possible to differentiate between fibrous lesions (low signal intensity on T1- and T2-weighted MRI) and cartilaginous lesions (low signal intensity on T1-weighted MRI and high signal intensity on T2-weighted MRI).

Topics: Child; Female; Femur; Fibrous Dysplasia, Polyostotic; Humans; Lumbar Vertebrae; Magnetic Resonance Imaging; Radionuclide Imaging; Technetium Tc 99m Medronate; Thoracic Vertebrae; Tomography, X-Ray Computed

Topics: Adult; Bone Neoplasms; Fibrous Dysplasia of Bone; Fibrous Dysplasia, Polyostotic; Humans; Male; Radionuclide Imaging; Technetium Tc 99m Medronate

Bone scintigraphy is a sensitive imaging modality for detecting early lesions and polyostotic involvement in fibrous dysplasia. Common findings include multiple areas of focal uptake that are often unilateral and typically involve the ribs, tibia, femur, and craniofacial bones. A knowledge of various skeletal manifestations of fibrous dysplasia is helpful to distinguish it from Paget's disease, osteoblastic metastasis, and fractures. Several recent cases representing varying involvement of fibrous dysplasia are presented and the literature is reviewed.

Topics: Adult; Bone and Bones; Diagnosis, Differential; Female; Fibrous Dysplasia of Bone; Fibrous Dysplasia, Polyostotic; Humans; Male; Middle Aged; Radionuclide Imaging; Technetium Tc 99m Medronate

Scintigraphic manifestations of fibrous dysplasia were analyzed in 59 lesions of 26 patients (12 monostotic, 14 polystotic). Bone imaging with Tc-99m MDP revealed a high percentage of increased uptake of radioisotope in the lesions of fibrous dysplasia. Four (14%) of 29 cystic lesions and two (7%) of 30 lesions with the appearance of ground glass showed no increase in radioisotope uptake, although roentgenograms showed marked changes. Therefore, care must be taken in the diagnosis of fibrous dysplasia with bone imaging alone. Nuclear methods, however, are indispensable in evaluating the dynamic aspects of bone mineral behavior and in demonstrating disease where none was suspected, or in visualizing polyostotic involvement in those cases where only monostotic disease was suspected clinically. It is concluded that both scintigrams and roentgenograms are complementary procedures in the diagnosis of fibrous dysplasia.

Topics: Adolescent; Adult; Child; Child, Preschool; Female; Fibrous Dysplasia of Bone; Fibrous Dysplasia, Monostotic; Fibrous Dysplasia, Polyostotic; Humans; Male; Middle Aged; Radionuclide Imaging; Technetium Tc 99m Medronate

Fibrous dysplasia, a bone dysplasia of unknown pathogenesis, may be either monostotic or polyostotic. Not only is the femur involved in nearly all cases of the polyostotic form, but a distinct unilateral predilection is often noted. The following case illustrates the classic polyostotic changes.

Topics: Adult; Diphosphonates; Fibrous Dysplasia of Bone; Fibrous Dysplasia, Polyostotic; Humans; Leg; Male; Radiography; Radionuclide Imaging; Skull; Technetium; Technetium Tc 99m Medronate