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PubMed2004

Autosomal malignant osteopetrosis. From diagnosis to therapy.

Osteopetrosis is a heterogeneous family of rare human genetic disorders due to markedly decreased bone resorption. It is one among disorders causing osteosclerosis of the trabecular bone and/or hyperostosis of the cortical bone. Four types of human osteopetrosis have been clearly defined, but patients with atypical symptoms are frequent, suggesting that there are additional forms. The most severe expression of this condition in its malignant form is inherited as an autosomal recessive disorder and it is usually fatal before school age. It presents with failure to thrive, severe hepatosplenomegaly, pancytopenia and nerve compression leading to blindness and deafness during infancy. The case of a 2-month-old female child with severe hepatosplenomegaly, failure to thrive, nystagmus, pancytopenia, gengival hyperplasia, optic atrophy, absent evoked visual potential and increased bone density within the total skeleton, is reported. Diagnosis of autosomal recessive malignant osteopetrosis was established by transiliac bone biopsy. She underwent bone marrow transplantation, but died soon afterwards. This rare and mortal disorder of bone formation requires early diagnosis and immediate pharmacological treatment, consisting in administration of vitamin D, in order to enhance bone resorption and of prednisone to improve hematological indexes and, if possible, bone marrow transplantation in order to ameliorate quality of life and survival.

Anti-Infective Agents↗
PubMed2004

[Erdheim-Chester disease: a non-Langerhans cell histiocytosis. A clinical-case and review of the literature].

We make a retrospective evaluation of clinical and radiologic features, treatment, and outcome of Erdheim-Chester disease, a rare non-Langerhans cell histiocytosis. We report a case of Erdheim-Chester disease and review 60 cases from the literature. These cases are consider to have Erdheim-Chester disease when they have either typical bone radiographs (symmetrical long bones osteosclerosis) and/or histologic criteria disclosing histiocytic infiltration with distinctive immunohistochemical phenotype of the non-Langerhans cell histiocytes with positive staining for CD68 and negative staining for S-100 protein and CD1a. Our patient undergoes chemiotherapy according to the LCH-II stratification and therapy plan (Vinblastine, Etoposide and Prednisone) and thereafter receives Carboplatin and Etoposide, and Somatostatin. She is alive and clinically well 33 months after onset of symptoms and the lesions don't appear to progress at imaging examinations. In conclusion, Erdheim-Chester disease may be confused with Langerhans cell histiocytosis as it sometimes shares the same clinical (exophthalmos, diabetes insipidus) or radiologic (osteolytic lesions) findings. However, the characteristics radiological pattern of Erdheim-Chester disease together the immunohistochemical phenotype of hystiocytic infiltration supports the theory that Erdheim-Chester disease is a unique disease entity distinct.

Adolescent↗
PubMed1992

[Osteoid osteoma of the elbow].

The osteoid osteoma is a rare, benign neoplasm of bone, most frequently located in the lower extremity (head of the tibia). Because of its rarity and its mostly rather unspecific clinical symptoms, diagnosis of the tumor is frequently problematic. Ideally, osteosclerosis surrounding a central focus (nidus) is revealed by radiology. Radiological imaging seems to be the most effective tool in reaching the correct diagnosis. Operative resection of the nidus is the only known curative therapy for osteoid osteoma.

Adult↗
PubMed2003

Skeletal implications of prostate cancer.

This manuscript reviews the theories behind the propensity of prostate cancer to cause bone metastases and skeletal implications of the prostate cancer biology and treatment modalities. The escape of tumor cells from the primary tumor in the prostate to secondary tumor sites in the axial skeleton probably occurs before the primary tumor is detected. Several theories offer explanations for the observed proclivity of prostate tumors to selectively colonize the axial skeleton. The interaction between the tumor cells and cells that populate bone marrow, in particular osteoblasts and osteoclasts, is important for creating a 'fertile' environment where tumor cells can establish and grow. Prostate cancer cells are capable of producing growth factors that can affect both osteoblasts, resulting in osteoblastic bone formation, and osteoclasts, resulting in excessive bone resorption. In addition to the capability to progress from testosterone-dependent to testosterone-independent phenotype, the hallmark of metastatic prostate cancer is osteosclerosis similar to one induced experimentally in nude rats using CWR22 human prostate cancer cell line. Metastatic bone disease caused by excessive bone formation and bone resorption is the major cause of morbidity in patients with prostate cancer. The most common symptoms include pain, pathological fractures, spinal cord compression, cranial nerve palsies, bone marrow suppression and hypercalcemia. The introduction of prostate-specific antigen in clinical practice created a shift to where more prostate cancer patients with early disease receive androgen ablation treatment, which in return causes more bone loss and cancer-associated osteoporosis. Introduction of third generation bisphosphonates to treat skeletal consequences of malignancy further stressed the important interaction between the bone marrow stroma and cancer cells. Nevertheless, animal models and human prostate tumor cell lines that mimic all aspects of skeletal conditions in prostate cancer patients including osteoblastic bone response are needed to develop and screen for novel therapeutic and diagnostic modalities.

Journal Article↗
PubMed2003

Intra-tibial injection of human prostate cancer cell line CWR22 elicits osteoblastic response in immunodeficient rats.

We investigated the utility of CWR22 human prostate cancer cells for modeling human metastatic prostate cancer, specifically their ability to induce bone formation following intra-tibial injections in the nude rat. Prostate cancer is unique in regard to its tropism for bone and ability to induce new bone formation. In contrast to humans, other mammalian species rarely develop prostatic cancer spontaneously upon aging and do not have the propensity for bone metastasis that is the hallmark of cancer malignancy in men. We chose human prostate cancer cell line CWR22 based on its properties, which closely resemble all of the features that characterize the early stages of prostatic cancer in human patients including slow growth rate, hormone dependence/independence and secretion of prostate-specific antigen. When CWR22 cells were injected directly into the proximal tibia of immunodeficient male rats, both osteoblastic and osteolytic features became evident after 4 to 6 weeks, with elevated levels of serum prostate-specific antigen. However, osteosclerosis dominates the skeletal response to tumor burden. Radiological and histological evidence revealed osteosclerotic lesions with trabeculae of newly formed bone lined by active osteoblasts and surrounded by tumor cells. Toward the end of the 7-week study, osteolytic bone lesions become more evident on X-rays. Paraffin and immunohistochemical evaluations revealed mature bone matrix resorption as evidenced by the presence of many tartrate resistant acid phosphatase positive multinucleated osteoclasts. We conclude that the CWR22 human prostate cell line used in an intra-tibial nude rat model provides a useful system to study mechanisms involved in osteoblastic and osteolytic bony metastases. This type of in vivo model that closely mimics all major features of metastatic disease in humans may provide a critical tool for drug development efforts focused on developing integrated systemic therapy targeting the tumor in its specific primary or/and metastatic microenvironments. In addition to targeting bone marrow stroma, this strategy will help to overcome classical drug resistance seen at the sites of prostate cancer metastasis to bones.

Journal Article↗
PubMed2005

[Osteopetrosis with carbonic anhydrase II deficiency: report of 24 cases].

Twenty four patients suffering from osteopetrosis caused by carbonic anhydrase II deficiency are colliged. This pathology seems to be frequent in Tunisia. Mental retardation is present in 52%, 85% of patients have short stature and 25% have optic atrophy. All affected subjects show craniofacial disproportion and dental anomalies. Twenty patients have at least one bone fracture. Metabolic acidosis is constant: it is profound during the first life decade. A severe selective reduction of carbonic anhydrase II in erythrocyte is confirmed in 18 cases. Osteosclerosis and defective skeletal modelling are constant, cerebral calcification can be seen at the scanner approximately at the age of two years and six months. All patients are homozygous for a splice junction mutation in intron 2 of the carbonic anhydrase II gene, this mutation does not seem to protect patients from bone fractures nor induce a severe metabolic acidosis.

Adolescent↗
PubMed1991

Renal osteodystrophy.

The term "renal osteodystrophy" is used to include skeletal disorders of patients with chronic renal failure: osteitis fibrosa, osteomalacia, osteosclerosis, osteoporosis and the frequently associated extraskeletal calcifications. It is the chronic glomerular disease with phosphate retention and resultant hyperphosphatemia on one hand and deficient 1,25 (OH)2 D3 and resultant hypocalcemia on the other to induce secondary hyperparathyroidism. The three most common causes of chronic renal failure in our patients are chronic glomerulonephritis, diabetic nephropathy, hypertensive nephropathy in decreasing frequency, polycystic renal disease occurs in five patients. Other miscellaneous causes include nephrotic syndrome, chronic pyelonephritis, systemic lupus erythematosus, periarteritis nodosa, interstitial nephritis and renal stones. The bone changes are similar in primary and secondary hyperparathyroidism and the incidence of brown tumor is about 3% in the former and 1.5 to 1.7% in the latter. We present one among the 94 dialyzed patients who has long-standing severe chronic renal failure from polycystic kidney disease and develops brown tumor in the mid ulna after 7 years on maintenance hemodialysis. The incidence of brown tumor in our series is about 1.1%. Because of increased longevity of the dialyzed patients, brown tumor from secondary hyperparathyroidism is now more commonly observed. Hyperphosphatemia with serum calcium-phosphate products exceeding plasma solubility of 60 to 75 mg/dl may induce soft tissue and vascular calcification. This explains the much higher incidence of soft tissue calcification in secondary than primary hyperparathyroidism; two of our patients with generalized Monckeberg's type arterial calcification and multiple periarticular calcifications in five patients have been observed.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed2006

[Radiological diagnosis of primary malignant fibrous histiocytoma of bone].

OBJECTIVE: To explore the radiological diagnosis of primary malignant fibrous histiocytoma (MFH) of bone. METHODS: Sixteen patients with biopsy-or surgery-confirmed MFH received both plain X-ray and CT examinations, among whom six patients simultaneously received MRI. The imaging features were analyzed and the differential diagnoses were assessed. RESULTS: (1) Plain X-ray findings: All these lesions showed irregularly osteolytic, accompanied by cortical destruction. Five patients had varied degrees of cortical expansion, 12 had large soft tissue masses adjacent to the lesions, and only 2 had periosteal reaction. (2) CT findings: All lesions were osteolytic areas but had no evidences that its internal architecture had been replaced by soft tissue mass, and the cortical adjacent to the lesions were permeative osteolysis. Four patients had internal or marginal crest within the lesions and marginal inconsecutive osteosclerosis. Twelve had large soft tissue masses but without any calcification and residual architecture adjacent to the lesions, among which 3 patients had solitary or multiple cystic attenuation areas within the masses. No clear periosteal reaction was observed on CT. (3) MRI findings: All of lesions in 6 patients who received MRI showed inhomogeneous long T1 and long T2 abnormal signal intensity with soft tissue masses adjacent to the osteo-destructions. CONCLUSIONS: The imaging manifestations of MFH were specific to some extent. Combined utilization of plain X-ray, CT, and MRI is helpful for the diagnosis and differential diagnosis of MFH.

Adult↗
PubMed1991

[Surgical treatment of total forms of chronic osteomyelitis of the femur in children].

In the article is described a new method of treatment of chronic hematogenic osteomyelitis of femur in children in case of its total affection. The essence of the method consists in making a longitudinal groove along the whole length of a bone by means of chisels and milling cutters till the medullar canal is reached and removal of pus, sequesters, pathologic granulations and zones of excessive osteosclerosis. The cavity after bathing is subjected to ultrasound cavitation; fibrinous film and gauze plastrons, impregnated with hemostatic sponge, are placed in it with the proximal and distal ends connection to the tubular drainage in order to ensure permanent lavage of cavity with antiseptic solution and active vacuum aspiration of its content with the subsequent application of sutures on the wound, tight bandaging of a limb and its immobilization. 6 patients have been subjected to treatment by this method with good outcomes in all cases. The results have been followed through 2.5-8 years.

Child↗
PubMed1991

Dacryocystorhinostomy in osteopetrosis.

Osteopetrosis is a rare congenital disorder of bone metabolism characterized by generalized sclerotic bone resulting from osteoclast dysfunction. There are several ocular manifestations, one of which is nasolacrimal duct obstruction from osteosclerosis of the nasolacrimal foramen. The surgeon must anticipate and prepare for the difficulties of working with marbleized, ultra-dense bone, alternating technique accordingly. In nasolacrimal duct obstruction, surgical management produces seemingly stable long-term results. However, if ossification recurs at the operative site, reoperation may become necessary for either optic nerve decompression or dacryocystorhinostomy.

Adult↗
PubMed1991

Skeletal and dental fluorosis: two case reports.

Two case reports from a high fluoride (10 ppm) rural community. They presented with severe degrees of dental fluorosis, hyper-sensitivity of teeth and skeletal fluorosis all arising from the ingestion of high amount of fluoride in water over a long period of time. Both cases had deformities of the upper and lower limbs. However, the deformities were more pronounced in the lower limbs than in the upper limbs, resulting in knock knee. Radiological finding showed osteosclerosis of the axial bones while the appendicular bones exhibited osteoporosis. There was marked change of bone structure observed as osteomalacia, and course trabecular bone pattern. Osteoporosis was also associated with cortical thinning. Periosteal bone apposition was observed in the bones: and genu valgum of the limbs. Biochemical tests revealed normal values for serum calcium and inorganic phosphate. However, the serum alkaline phosphatase was elevated. This may be an indication of a pathological condition where there are possible compensatory mechanisms to maintain normal levels of serum calcium and inorganic phosphate. One case which had undergone corrective surgical intervention of the lower limbs four years earlier, had continued to live in the same environment using drinking water with 10 ppmF after corrective surgery, and showed no improvement.

Adolescent↗
PubMed1990

[Juxta-articular osteoid osteoma--Image morphology and diagnosis].

Juxta-articular osteoid osteomas as a cause of persistent pain of the shoulder, elbow and knee are presented basing on three own observations and a review of the literature. The sequence of the imaging examinations is equivalent to the osteoid osteomas in other localisations. CT permits a clear presentation of the nidus in the complex juxta-articular anatomy. Juxta-articular osteoid osteomas often show an absent or small perifocal osteosclerosis, whereas a laminar periosteal reaction was seen in all own cases.

Adolescent↗
PubMed1990

[Environment and genetics of endocrine polyoncoses. An aging bull model].

An old bull, it is said by those who know, can have his troubles. Included among these are vertebral osteosclerosis and ankylosing spondylosis--this stiffening up limite, rather than accentuates, the value and reproduction potential of a stud bull past prime. But associated with these abnormalities--and not seen in age-matched cows of comparable breeds--are fascinating endocrine neoplasms that might suggest a pattern that could be productive as a model of human hereditary endocrine abnormalities. Adjacent to the thyroid gland in other vertebrates are ultimobranchial bodies, that are incorporated into the lateral thyroid lobes in primates as the parafollicular "C-cells" of the thyroid. These are the cells in man that give rise to medullary thyroid cancer and are associated with calcitonin secretion, useful as a tumor marker. In aging bulls of whatever breed, nearly half exhibit abnormality of these ultimobranchial bodies: 20% show hyperplasia, and 30% have frank neoplasia. These ultimobranchial tumors appear in bulls passing 6 1/2 years in age, and are absent in young bulls and all cows of any age. Calcitonin can be demonstrated in the ultimobranchial tumors from bulls, and secretion is stimulated by calcium infusion, though serum calcium remains normal. The ultimobranchial tumors themselves can range from hyperplasia through adenoma to metastasizing carcinoma--in fact, representing one of the commoner cattle cancers. Parathyroid glands taken from bulls with these ultimobranchial tumors initially show evidence of inhibited secretory activity and morphologic atrophy, but later go on to develop hyperplasia and, eventually, autonomy.(ABSTRACT TRUNCATED AT 250 WORDS)

Aging↗
PubMed1991

The radiographic spectrum of renal osteodystrophy.

Chronic renal failure often results in bone changes, collectively known as renal osteodystrophy. These changes include osteitis fibrosa, osteosclerosis, soft tissue calcifications, osteomalacia (in adults) and rickets (in children). Early recognition of renal osteodystrophy allows more aggressive clinical control of serum calcium and phosphate levels. Intervention may spare the patient late complications such as fractures, pain and loss of skeletal function.

Aluminum Hydroxide↗
PubMed1991

[The early changes in bone mineral metabolism due to radiation--measurement of bone mineral density in lumbar vertebra by quantitative computed tomography].

Osteosclerosis, osteonecrosis and compression fracture are commonly observed several years after radiation. Since lumbago usually occurs several months after radiation, the possibility that bone mineral metabolism is disturbed during and immediately after radiation cannot be ruled out. However, there have been no reports concerning early changes in bone mineral metabolism due to radiation. The bone mineral density was measured by QCT (Quantitative Computed Tomography) in 30 normal non-radiated cases and 14 radiated cases to investigate the changes in bone mineral metabolism due to radiation. The bone mineral density (QCT-Value: QCT-V) in the 3rd lumbar vertebra (L3) of normal non-radiated subjects decreased linearly with age (Y = 291.114447-3.01473X). The QCT-V of the 5th lumbar vertebra (L5) of normal cases also decreased linearly with age (Y = 309.641397-3.03986X), resembling that of L3. The ratio of the QCT-V of L5 to L3 (L5/L3, expressed as a percentage) definitely increased with age (Y = 86.5657441 + 0.58885064X). In radiated cases, the QCT-V of L3 in the non-radiated field did not change appreciably. The QCT-V of L5 in the radiated field was decreased from 20GY and reached 53.08 +/- 17.37% of the pre-radiation value after 50GY. The L5/L3 ratio was also decreased from 20GY and reached 55.47 +/- 15.32% of the pre-radiation value after 50GY. It becomes apparent that the QCT-V of the radiated lumbar vertebra is decreased during radiation. It is suggested that bone mineral metabolism may be disturbed in the early phase of radiation.

Bone Density↗
PubMed1990

[POEMS syndrome: report of a case and review of the literature].

A case of POEMS Syndrome of six years of evolution is reported. This syndrome is characterized by Raynaud phenomenon, polyneuropathy, edema, anasarca, papilledema, osteosclerosis and lymphadenopathy with the histopathology of Castleman's disease, hypothyroidism, hypogonadism, cutaneous sclerosis, hyperpigmentation, axillary alopecia and the presence of urinary lambda light chains. A bone marrow biopsy did not show plasmocytic infiltration and there was no evidence of extramedullary plasmocytoma. Methylprednisone was given at the dose of 1 mg/kg/day and subjective and objective improvement was observed. The edema and anasarca disappeared as well as the lymphadenopathies; muscle strength improved and the patient was able to walk without aid. Papilledema persisted. The pathogenesis of this syndrome remains unknown; some of the symptoms have been attributed to paraprotein deposits in peripheral nerves, high capillary permeability due to vascular alterations, accelerated conversion of androgen to estrogen, or to the production by plasma cells of a toxic substance. Mortality is related to complications of the polyneuropathy. Some patients in whom POEMS syndrome was associated, or not, with myeloma were treated with chemotherapy and/or radiotherapy with different responses; in others, corticosteroids were of short lived benefit. Our patients remains well after 42 months treatment with 20 mg methylprednisone every other day.

Edema↗
PubMed1990

[Hyperostosis cranialis interna; a new syndrome with autosomal dominant inheritance].

A family is described which currently comprises nine individuals, spanning three generations, who are affected with a bone disorder which is confined to the skull and is accompanied by impaired function of the cranial nerves. Radiological examination showed intracranial hyperostosis and osteosclerosis of the calvaria and the base of the skull, without involvement of the mandible or other skeletal bones. Invariably, the main presenting symptom was recurrent facial nerve paralysis from late childhood onwards, but concurrent and variable involvement of the olfactory, optic, vestibular and acoustic nerves was seen; this could be attributed to nerve compression by the bony encroachment into the cranial foramina. Morphological investigations revealed increased formation of bone tissue with a normal structure. The pedigree suggests an autosomal dominant mode of heredity. A review of the literature did not disclose any previous reports on this disorder.

Adolescent↗
PubMed1990

[Radiologic changes in the pelvis of patients with chronic renal insufficiency undergoing periodic dialysis].

Bone alterations in the patients undergoing periodic dialysis represent a frequent and invalidating complication and cause the pattern called uremic osteodystrophy. In this study we have examined 173 pelvic X-rays of 73 patients affected with chronic renal insufficiency and undergoing periodic dialysis. The results indicate the presence of all the lesions characterizing uremic osteodystrophy. Arterial calcifications and osteoporosis are the most frequent patterns; with various incidence, osteomalacia, osteitis fibrosa, osteosclerosis and brown tumors are associated. In this group of patients, who were followed for many years, a non-univocal behavior was observed: next to patterns of progressive aggravation, such as vascular calcifications, phases of stabilization/improvement were observed, e.g., in case of brown tumors and osteitis fibrosa.

Adolescent↗