1/9. Bizarre parosteal osteochondromatous proliferation of bone (BPOP): an unusual foot mass in a child.This report describes an unusual case of bizarre parosteal osteochondromatous proliferation of bone (BPOP) which developed in the foot of an eight year-old child. Also described as Nora's lesion, BPOP is a rare benign bone tumor found most commonly in the hands and feet with a radiographic appearance occasionally confused with malignancy. Resembling osteochondromas at first glance, these lesions have a distinct radiographic and histologic appearance that permits differentiation from other benign lesions or low-grade malignant tumors. Treatment of BPOP consists of simple excision, although there is high rate of recurrence. Despite this high recurrence rate, there have been no reported metastases and local excision is still recommended.- - - - - - - - - - ranking = 1keywords = osteochondromas (Clic here for more details about this article) |
2/9. Multiple occurrence of osteochondromas in dysplasia epiphysealis hemimelica.Dysplasia epiphysealis hemimelica was defined by Trevor (1950) as a rare congenital growth disorder of the tarsus and of the epiphysis of the long bone. In this report, a rare case of dysplasia epiphysealis hemimelica associated with multiple extraskeletal osteochondromas is presented. Although different modes of expression of the same pathologic process have been suggested for dysplasia epiphysealis hemimelica and osteochondroma, the biological feature of cartilaginous overgrowth in the skeletal system still seems unclear.- - - - - - - - - - ranking = 5keywords = osteochondromas (Clic here for more details about this article) |
3/9. Acetabular dysplasia associated with hereditary multiple exostoses. A case report.Hereditary multiple exostoses is an autosomal dominant disorder characterised by multiple osteochondromata, most commonly affecting the forearm, knee and ankle. Osteochondromata of the proximal femur have been reported to occur in 30% to 90% of affected patients with coxa valga in 25%. Acetabular dysplasia is rare but has been described. This is the first report of a patient requiring surgical intervention. A girl was seen at the age of nine with hereditary multiple exostoses and when 12 developed bilateral pain in the groin. Radiographs showed severely dysplastic acetabula with less than 50% coverage of the femoral heads and widening of the medial joint space. Large sessile osteochondromata were present along the medial side of the femoral neck proximal to the lesser trochanter, with associated coxa valga. The case illustrates the importance of obtaining initial skeletal surveys in children with hereditary multiple exostoses to identify potential problems such as acetabular dysplasia and subluxation of the hip.- - - - - - - - - - ranking = 10.552121554842keywords = hereditary multiple (Clic here for more details about this article) |
4/9. Uncommon clinical course of multiple osteochondromatosis in a patient with a long-term history of Cushing's disease.Cushing's disease (CD), the chronic endogenous hypercortisolism derived from an acth-secreting pituitary adenoma, and multiple osteochondromatosis (MO), a congenital mesoderm dyschondroplasia, represent two distinct rare neoplastic diseases. Clinical appearance of MO usually occurs during the first-second decade of life. In fact, the growth of osteochondromas parallels the patient's growth, then becoming quiescent after the closure of the epiphyses and the achievement of final stature. Here we describe an uncommon case of a patient with a long-term history of childhood-onset CD, who surprisingly developed MO during the third decade of life, after the remission of CD. Indeed, a female patient had been followed for CD from the age of 12 to the age of 24 years, when CD definitively remitted. At the age of 26 the patient complained progressively worsening backache and pain at level of hips and feet. Standard radiography of skeleton showed multiple bone dysmorphisms at level of the four limbs, spine and pelvis consistent with multiple osteochondromas and exostoses. A diagnosis of MO was performed. Total body bone scintigraphy with 99mTc-MDP revealed an increased uptake of the radioligand, suggesting an increased metabolic turnover in correspondence of the majority of the osteochondromas. However, the negativity of the majority of the lesions at 99mTc-DMSA scintigraphy and the histological diagnosis of benign osteochondroma of the only positive lesion at 99mTc-DMSA evidenced that the high metabolic activity of the osteochondromas was not due to malignant transformation. However, the activity of the lesions was highly surprising considering that they usually become quiescent after the achievement of the final stature. In last analysis, the uncommon characteristics of MO and, particularly, its occurrence after stable remission of hypercortisolism, suggests a possible role of glucocorticoids in influencing the clinical course of the skeletal disease. The inhibitory effect of hypercortisolism on bone growth and maturation could explain the block in the proliferation of skeletal lesions during the developmental age, where CD was in the active phase, and the opposite effect of stimulation of the ostochondromas growth during stable normalization of cortisol secretion, after CD remission.- - - - - - - - - - ranking = 4keywords = osteochondromas (Clic here for more details about this article) |
5/9. Tenosynovial osteochondromatosis of the tarsal tunnel.A case of tenosynovial osteochondromatosis in the tarsal tunnel in a 23-year-old man is presented. The lesion was treated surgically, and multiple osteochondromas were excised, which had no continuity with any tarsal bone or joint cavity but did with the sheaths of the flexor digitorum longus and flexor hallucis longus. Histologic examination of the lesion showed a fibrous capsule, hyaline cartilage and extensive areas of cancellous bone. necrosis and mitosis were absent in the hyaline cartilage and there were no synovial nodules indicative of synovial metaplasia. The macroscopic findings showed "end-stage" tenosynovial osteochondromatosis. There was no evidence of recurrence 5 years after operation, and the patient remains free of symptoms.- - - - - - - - - - ranking = 1keywords = osteochondromas (Clic here for more details about this article) |
6/9. Dominant carpotarsal osteochondromatosis.Dominant carpotarsal osteochondromatosis is a particular disorder of the wrist and tibiotalar joints with abnormal bone proliferation and osteochondromas. Two patients, a mother and son, are described here; a similar condition has previously been described in seven affected members of a family. The upper and the lower limbs are affected in the same patient and the lesion can be bilateral. Autosomal dominant inheritance is a further criterion allowing the diagnosis of dysplasia epiphysealis hemimelica.- - - - - - - - - - ranking = 1keywords = osteochondromas (Clic here for more details about this article) |
7/9. Osteochondroma of the thoracic spine: an unusual cause of spinal cord compression.A 24-year-old man with hereditary multiple exostoses had numbness of the lower extremities and difficulty walking. CT displayed a calcified extradural mass lesion within the spinal canal at T-8 causing cord compression. MR imaging showed it to be contiguous with the upper endplate of T-8, suggesting the diagnosis of osteochondroma, a rare cause of cord compression, and distinguishing the lesion from a calcified disk fragment.- - - - - - - - - - ranking = 1.758686925807keywords = hereditary multiple (Clic here for more details about this article) |
8/9. Hereditary spinal osteochondromas in diaphyseal aclasia.We present cases of cervical cord compression in father and son with diaphyseal aclasia. Both patients were investigated with plain radiography, CT and MRI. We believe this to be the first report of spinal cord compression in first-degree relatives with diaphyseal aclasia.- - - - - - - - - - ranking = 4keywords = osteochondromas (Clic here for more details about this article) |
9/9. Cervical laminar exostosis in multiple hereditary osteochondromatosis: anterior stabilization and fusion technique for preventing instability.Multiple hereditary osteochondromatosis is a genetically transmitted disorder consisting of multiple projections of bone capped by cartilage, which are called exostoses. spinal cord compression due to expansion of a laminar osteochondroma is rare but well recognized. Surgical decompression usually improves the patient's neurological status but, in cervical exostosis, post-laminectomy kyphosis and instability problems, especially in the high-risk adolescent group, form the most significant potential difficulties in the postoperative period. We report a case of cervical laminar exostosis that was treated by anterior stabilization and fusion and discuss the benefits of this technique.- - - - - - - - - - ranking = 1.5813525511784keywords = exostosis (Clic here for more details about this article) |