Cases reported "Osteochondromatosis"

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1/9. Synovial osteochondromatosis. A case report and review of the literature.

    Synovial osteochondromatosis is a benign disease of unknown etiology that involves the articular and periarticular structures. The disorder primarily affects men, and trauma is the most common predisposing factor. The authors review the literature, clinical findings, proposed etiology, classification, ancillary tests, histopathology, differential diagnosis, treatment options, and prognosis. A case report of this rare entity is also presented.
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2/9. 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.
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keywords = osteochondromatosis
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3/9. 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.
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ranking = 1.2116009520284
keywords = osteochondromatosis, synovial osteochondromatosis
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4/9. Secondary osteochondromatosis in the subacromial bursa: a report of two cases and review of the literature.

    osteochondromatosis is classified into primary and secondary lesions; primary osteochondromatosis is a tumor-like lesion, whereas secondary lesions are due to such joint or bursal disorders as osteoarthritis and osteochondral fractures. There is no clinical distinction between these two conditions. Only the pathological findings of loose bodies and synovium can clearly distinguish between them. In this report, we present two patients with secondary osteochondromatosis in the subacromial bursa. Both had shoulder pain and were treated surgically. We discuss methods of differentiating between primary and secondary lesions and elucidate the pathogenesis of osteochondromatosis in the subacromial bursa by analyzing findings for 17 shoulders with this condition reported in the literature. We also present two more cases. We reviewed the 12 cases of osteochondromatosis in the literature for histopathological findings of loose bodies and the presence or absence of acromial osteophytes. Our findings suggest that osteochondromatosis of the subacromial bursa is secondary in most cases, and that osteocartilaginous fragments from acromial osteophytes can be shed into the subacromial bursa and grow into loose bodies.
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ranking = 1.8
keywords = osteochondromatosis
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5/9. Periosteal chondrosarcoma in a 9-year-old girl with osteochondromatosis.

    A 9-year-old girl with multiple osteochondromatosis presented with a 1 year history of a gradually enlarging surface lesion originating from the midsection of the right humerus, distal to an osteochondroma. Radiographically and histologically this lesion proved to be a periosteal chondrosarcoma adjacent to an osteochondroma.
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keywords = osteochondromatosis
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6/9. Synovial chondromatosis simulating neoplastic degeneration of osteochondroma: findings on MRI and CT.

    A case is presented of synovial chondromatosis within a bursal sac overlying an osteochondroma in a patient with osteochondromatosis. This condition presented with a symptomatic soft tissue mass containing calcified bodies. It can be mistaken clinically and radiographically for malignant degeneration of an osteochondroma with development of chondrosarcoma. Magnetic resonance findings have not previously been described in this entity and proved helpful in the preoperative diagnosis. magnetic resonance imaging was also helpful in defining the extent of the lesion. Ultrasound and other imaging modalities are also discussed, including the pathologic basis for the radiographic findings.
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keywords = osteochondromatosis
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7/9. 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.
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keywords = osteochondromatosis
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8/9. Dedifferentiated chondrosarcoma in patients with multiple osteochondromatosis: report of a case and review of the literature.

    Multiple osteochondromatosis (MOS) is a familial disorder of autosomal dominant transmission characterized by the development of multiple exostoses and often derangements of epiphyseal cartilage, sometimes resulting in long bone growth retardation. patients with the disorder appear to be at increased risk for developing secondary chondrosarcomas. Rarely, dedifferentiated chondrosarcomas may also occur. We report a single case of a 27-year-old man with multiple osteochondromatosis who developed a fatal dedifferentiated chondrosarcoma. Radiographically, the neoplasm arose from the pelvis completely destroying the left pubic ramus. Subsequently, the patient underwent preoperative chemotherapy followed by a left external hemipelvectomy. On pathologic examination, the tumor was characterized by high-grade pleomorphic sarcoma sharply juxtaposed to a low-grade chondrosarcoma. The patient ultimately died of widespread metastatic sarcoma.
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ranking = 1.2
keywords = osteochondromatosis
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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.
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keywords = osteochondromatosis
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