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PubMed2004

Bone morphogenetic proteins are expressed by both bone-forming and non-bone-forming lesions.

CONTEXT: Bone morphogenetic proteins (BMPs) are thought to be responsible for bone formation; they cause bone to form in soft tissues and are clinically used in helping fracture union or tumor reconstructions. Skeletal metastases from epithelial tumors may be either bone-forming (blastic) or non-bone-forming (lytic). OBJECTIVE: We studied the expression of BMPs in a variety of primary and secondary lesions of bone (both bone-forming and non-bone-forming) to determine if there was a consistent relationship between bone formation and BMP expression. DESIGN: We compared a bone-forming lesion (fibrous dysplasia) with a non-bone-forming lesion (desmoid tumor), using reverse transcription-polymerase chain reaction, Northern blot analysis, and immunohistochemistry to detect BMPs. We also studied a number of non-bone-forming secondary lesions (carcinomas that formed lytic metastases to the skeleton) and found BMP production in most of these tumors. RESULTS: We found that BMPs were expressed in both bone-forming and non-bone-forming benign musculoskeletal lesions. In the first part of the study, BMPs were found in both fibrous dysplasia and desmoid tumors. Bone morphogenetic proteins were also expressed by several tumors. In the next part of the study (paraffin-embedded tissue), BMPs were expressed by a variety of tumors, irrespective of the radiological nature (blastic or lytic) of their metastases. CONCLUSIONS: We conclude that BMP production alone cannot explain bone formation, and other factors either alone or in combination may be responsible for blastic metastases to the skeleton and for bone formation by primary bone lesions, such as fibrous dysplasia.

Bone Morphogenetic Protein 2↗
PubMed2003

[Repair of bone defect due to tumour resection with self-setting calcium phosphate cement in children].

OBJECTIVE: To study the effect of self-setting calcium phosphate cement (CPC) on the repair of local bone defects after resection of cyst in children. METHODS: From December 1998 to May 2002, 22 patients with bone defects were repaired with CPC. Their ages ranged from 4 to 10 years with an average of 8.3 years. There were 11 cases of non-ossifying fibroma, 7 cases of osteoid osteoma, 2 cases of bone cyst and 2 cases of fibrous dysplasia. The bone defects are located as the following: femur in 14 cases, tibia in 6 cases and humerus in 2 cases. CPC spongiosa granules were filled in 11 cases, injectable CPC were filled in 2 bone cyst cases. The patients were followed up for 5-48 months, averaged 23.5 months. RESULTS: Bone matrix grew well and no recurrence was found. CONCLUSION: The method with simple CPC in repairing bone defects is safe, non-toxic, economic and convenient in children.

Biocompatible Materials↗
PubMed1987

Short-term effects of testolactone compared to other treatment modalities on longitudinal growth and ovarian activity in a girl with McCune-Albright syndrome.

In a 6 1/2-month-old girl with McCune-Albright syndrome, gonadotropin-independent isosexual precocity and recurrent ovarian cysts, the short-term effects of surgical therapy, cyproterone acetate (120 mg/m2/d), combined medroxyprogesterone acetate (10 mg/d), and spironolactone (50-75 mg/d) treatment, and testolactone (40 mg/kg/d) were evaluated sequentially. No significant reduction of cyst frequency was achieved with any of the medical treatments. The rate of bone maturation (delta BA/delta CA) was increased and the height standard deviation score (SDS) for bone age as a potential indicator of final height was decreased with surgical treatment alone and combined medroxyprogesterone acetate and spironolactone. Both parameters normalized with cyproterone acetate and testolactone. Height velocity SDS, however, was higher with testolactone (0.97 vs. 0.45).

Androgen Antagonists↗
PubMed2003

Oral bisphosphonates in polyostotic fibrous dysplasia.

The bisphosphonates inhibit osteoclastic bone resorption and have been used as an intravenous infusion in fibrous dysplasia of the bone. Oral bisphosphonates are cheaper and also easy to administer. We report a case of polyostotic fibrous dysplasia in a three-year-old child who showed significant improvement in bone mineral density after treatment with oral alendronate.

Administration, Oral↗
PubMed2004

Bone Morphogenetic Proteins Are Expressed by Both Bone-Forming and Non-Bone-Forming Lesions.

Context.-Bone morphogenetic proteins (BMPs) are thought to be responsible for bone formation; they cause bone to form in soft tissues and are clinically used in helping fracture union or tumor reconstructions. Skeletal metastases from epithelial tumors may be either bone-forming (blastic) or non-bone-forming (lytic).Objective.-We studied the expression of BMPs in a variety of primary and secondary lesions of bone (both bone-forming and non-bone-forming) to determine if there was a consistent relationship between bone formation and BMP expression.Design.-We compared a bone-forming lesion (fibrous dysplasia) with a non-bone-forming lesion (desmoid tumor), using reverse transcription-polymerase chain reaction, Northern blot analysis, and immunohistochemistry to detect BMPs. We also studied a number of non-bone-forming secondary lesions (carcinomas that formed lytic metastases to the skeleton) and found BMP production in most of these tumors.Results.-We found that BMPs were expressed in both bone-forming and non-bone-forming benign musculoskeletal lesions. In the first part of the study, BMPs were found in both fibrous dysplasia and desmoid tumors. Bone morphogenetic proteins were also expressed by several tumors. In the next part of the study (paraffin-embedded tissue), BMPs were expressed by a variety of tumors, irrespective of the radiological nature (blastic or lytic) of their metastases.Conclusions.-We conclude that BMP production alone cannot explain bone formation, and other factors either alone or in combination may be responsible for blastic metastases to the skeleton and for bone formation by primary bone lesions, such as fibrous dysplasia.

Journal Article↗
PubMed2006

G(s)alpha mutations in fibrous dysplasia and McCune-Albright syndrome.

Fibrous dysplasia (FD) is a focal bone lesion composed of immature mesenchymal osteoblastic precursor cells. Some FD patients also have hyperpigmented skin lesions (café-au-lait spots), gonadotropin-independent sexual precocity, and/or other endocrine and nonendocrine manifestations (McCune-Albright syndrome [MAS]). MAS results from somatic mutations occurring during early development, resulting in a widespread mosaic of normal and mutant-bearing cells, which predicts that the clinical presentation of each patient is determined by the extent and distribution of abnormal cells. These mutations encode constitutively active forms of G(s)alpha, the ubiquitously expressed G protein alpha-subunit that couples hormone receptors to intracellular cAMP generation. These mutations lead to substitution of amino acid residues that are critical for the intrinsic GTPase activity that is normally required to deactivate the G protein. This leads to prolonged activation of G(s)alpha and its downstream effectors even with minimal receptor activation. This explains why MAS patients have stimulation of multiple peripheral endocrine glands in the absence of circulating stimulatory pituitary hormones and increased skin pigment, which is normally induced by melanocyte-stimulating hormone through G(s)alpha/cAMP. Similar mutations are also present in 40% of pituitary tumors in acromegaly patients and less commonly in other endocrine tumors. FD results from increased cAMP in bone marrow stromal cells, leading to increased proliferation and abnormal differentiation. Parental origin of the mutated allele may also affect the clinical presentation, because G(s)alpha is imprinted and expressed only from the maternal allele in some tissues (e.g., pituitary somatotrophs).

Cyclic AMP↗
PubMed1998

[Psammomatoid ossifying fibroma].

OBJECTIVE: To clarify the histopathological, clinical behavior biological characterics and the relationship among the psammomatoid ossifying fibroma (POF), ossifying fibroma and fibrous dysplasia of bone. METHODS: Thirty-one cases of POF in the ear and sinonasal region were studied retrospectively. The sections, clinicopathological records from June, 1956 to November, 1996 were reviewed. The reticulum fiber and Masson's stain and alkaline phosphatase immunostain were done in 10 cases, Vimentin, Desmin, EMA and smooth actin immunohistochemical stain were done in 5 cases. One case was studied by transmission electron microscope. RESULTS: The specimens were broken tissue with the sand-like felling, eleven specimens with intact thin shell consisted of bone tissue were used to study the changes in the area between tumor and normal bone. Histopathologically they were rich in mesenchymal tumor cells and a lot of calcifying psammomatoid-like osteoid and a few of calcified bodies similar to those described by Shafer, and the bony trabecula was not found in the center of the tumor. In addition, three zones were noted at the junction of normal bone and tumor, namely, the normal bone-reactive proliferative bone-tumor. In some cases, the reactive bony proliferation may be not marked. The normal bone adjacent to the tumor may be absorbed and disappeared, and were then replaced by the tumor, and finally led to tumor tissue invasion into the soft tissue, but the infiltration appearance is not present. CONCLUSION: POF is the slowly growing benign tumor and is different from ossifying fibroma and fibrous dysplasia of bone.

Adolescent↗