Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0851184 (thinning)
 11,252 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Enchondroma is a benign growth of cartilage arising in the bone metaphysis as a solitary or multiple primary lesions. The form of multiple enchondromatosis with unilateral predominance is termed Ollier's disease. We have recently treated a case of Ollier's disease with the chief complaint of deformity of the left hand. The patient was an 11-year-old boy. Radiographic examination showed honeycombed clear spaces in the metaphyses of the middle and proximal phalanges of the left ring and little fingers as well as of the fourth and fifth metacarpals, and thinning of the cortex of these bones, but with no evidence of pathological fracture. The tumors of the left fourth and fifth metacarpal bones and of the phalanges of the left ring and little fingers were removed, and the metacarpophalangeal joint of the little finger was capsulotomized. The patient was free from recurrence 19 months after surgery. With regard to the prognosis of Ollier's disease, malignant transformation into chondrosarcoma or osteosarcoma has been reported of the chondroma. Since Ollier's disease is self-limited in that it usually stops spontaneously as the patient grows, and since the cartilaginous lesions in occasional cases may regress or even disappear, any cartilaginous lesions that are still active or painful after termination of the growth period should be examined thoroughly under suspicion of undergoing malignant transformation.
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PMID:A case of Ollier's disease of the hand. 901 45

We described a case of Werner's syndrome associated with osteosarcoma. A 37-year-old Japanese man was diagnosed as having Werner's syndrome by the presence of juvenile cataracts, skin sclerosis and hyperpigmentation of the feet, high-pitched voice, characteristic bird-like appearance of the face with beak-shaped nose, thinning of the entire skin and hyperkeratoses on soles, hyperlipemia, hyperuricemia, diabetes melitus, and the mutated responsible gene (WRN). He had a 3-month history of a tumor on his left forearm. Histologically, the tumor included four histological patterns; a malignant fibrous histiocytoma-like, a desmoid-like, a dermatofibrosarcoma protuberans-like, and a chondrosarcoma-like pattern. Tumoral osteoid formation was also found in the tumor. Therefore, the tumor was diagnosed as osteosarcoma.
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PMID:A case of Werner's syndrome associated with osteosarcoma. 1055 36

In contrast to solitary enchondroma,patients with multiple enchondromatosis of bone (Ollier's disease) get affected in childhood by bone swelling and growth retardation. In adults, the main problem is the risk of malign transformation up to 40%.Pain, increasing local tumor and thinning of the corticalis are the typical clinical and radiological signs of transformation to a low grade chondrosarcoma. There is no need for a regular radiological screening. If a malign transformation occurs,marginal or wide resection of the lesion should be performed.
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PMID:[Multiple enchondromatosis: Ollier's disease]. 1248 82

We present the case of a man with dyspnea due to a mass in the cricoid cartilage that turns out to be an extramedullary plasmocytoma. Although the patient has a history of multiple myeloma, the disease only rarely affects the cricoid cartilage. Other subglottic lesions possibly involving the cricoid cartilage are squamous cell carcinoma, chondroma, chondrosarcoma and metastasis. The imaging characteristics suggesting extramedullary plasmocytoma arising from the cricoid consist of thinning and expansion of the cartilage laminae without mucosal lesions nor soft tissue mass adjacent to the cricoid cartilage. The patient was successfully treated with radiation therapy and peroral steroids.
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PMID:Multiple myeloma involving the cricoid cartilage. 2384 29

Fibrous dysplasia (FD) is a monostotic or polyostotic benign bone lesion with spindle-cell proliferation in woven bone and stroma. Rarely, cartilaginous differentiation can be seen in the lesions of FD. FD with massive cartilaginous differentiation is called fibrocartilaginous dysplasia (FCD) and is considered a rare variant of FD. Although pathological findings of FD show irregular immature bone formation without osteoblastic rimming in fibrous tissue, and rarely show very small amounts of cartilage, histological images of FCD are said to show that cartilage with a relatively high cell density is present in the majority and that FD-like findings are seen in parts of it. The most characteristic feature of FCD on images is calcification in the lesions reflecting cartilaginous tissue. On the other hand, typical radiographic findings of FD include shadows with a ground-glass appearance and thinning and bulging of the cortical bone, the observation if calcification is not usual. Therefore, in the diagnosis of FCD, differentiation from multiple enchondromatosis, Ollier disease, chondrosarcoma, and chondrosarcoma secondary to FD is necessary, and it seems important to make a careful diagnosis based not only on the pathological findings but also on imaging and clinical findings. Herein, we report on a case of FD of the proximal femur associated with intralesional extensive carti laginous differentiation in which a pathological fracture occurred during follow-up, with a review of the literature.
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PMID:Fibrous Dysplasia with Massive Cartilaginous Differentiation (Fibrocartilaginous Dysplasia) in the Proximal Femur: A Case Report and Review of the Literature. 2729 99