1/28. Congenital fascial dystrophy: abnormal composition of the fascia.BACKGROUND: A scleroderma-like genetic disease, congenital fascial dystrophy, probably a variant of stiff skin syndrome described by Esterly and McKusick, was found to be related to genetically determined fascial abnormalities. Our previous electronmicroscopic study disclosed as a main pathologic finding presence of giant amianthoid-like collagen fibrils in the affected fascia. OBJECTIVE: The aim of the present study was to disclose the collagen abnormalities in the affected and control fascias and in the patient's fibroblast cultures derived from the skin and fascia. methods: The study was performed by histologic, immunohistochemical, and electronmicroscopic techniques. Immunohistochemical studies were done with the use of monoclonal antibodies: anti-collagens I, III, IV, and VI, anti-laminin, anti-fibronectin, anti-desmin, anti-spectrin, anti-vimentin, anti-laminin, anti-heparan sulfate, and anti-alpha-actinin. Electronmicroscopic studies were performed on the fascia sections and on cultured fibroblasts. RESULTS: The main abnormality leading to giant collagen fibril formation was presence of myofibroblasts, absence of collagen III, and overproduction of spectrin and collagen type vi, mainly its filamentous form. CONCLUSION: Our findings suggest that the abnormal composition of the fascia could depend on modulation of fibroblasts into myofibroblasts capable of producing spectrin and long-spacing collagen.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
2/28. Giant-cell tumor of the synovial membrane: localized nodular synovitis in the knee joint.Giant-cell tumor of the synovia is a benign neoplasm characterized histologically by proliferating histiocytes bearing lipids and hemosiderin intermingled with a variable number of multinuclear giant cells. Areas of predilection are the hand, and in the case of synovial joints, the knee joint is particularly affected. Clinically, patients have signs of mechanical derangement and, with the knee joint, meniscal symptoms and locking are often present. Joint effusion without previous trauma is another typical finding. diagnosis is carried out by radiographic tools and has to be confirmed histologically. Giant-cell tumor of the synovia is treated by local excision either by arthroscopy or by arthrotomy. To our knowledge, the case we present is the largest giant-cell tumor of the synovia in the knee joint ever described in literature.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
3/28. Familial arthropathy with camptodactyly: reports of two families.Familial association of congenital camptodactyly and arthropathy without evidence of concurrent inflammation has an autosomal recessive pattern of inheritance. We describe four children born to consanguineous parents in two families with congenital camptodactyly and polyarthropathy which were misdiagnosed and treated as juvenile rheumatoid arthritis (JRA) for some time. The siblings in the second family also had fibrosing pleuritis. Histopathological examination of the synovial tissues of the children in the first family revealed synovial hypertrophy and presence of multinucleated giant cells with minimal inflammation and vasculitis. On the other hand, prominent fibrosis with no inflammation was present in the synovial tissue of the elder boy in the second family. Thus, while the children in the first family had the phenotypic characteristics of congenital familial hypertrophic synovitis, the latter siblings probably represent a form of the familial fibrosing serositis.- - - - - - - - - - ranking = 0.5keywords = giant (Clic here for more details about this article) |
4/28. Giant cell tumour of the patellar tendon sheath--an unusual cause of anterior knee pain: a case report.We present a case of a giant cell tumour of the patellar tendon sheath with anterior knee pain. The relevant MRI findings and differential diagnosis of such lesions is discussed here.- - - - - - - - - - ranking = 0.5keywords = giant (Clic here for more details about this article) |
5/28. Tumoral calcinosis of the hand: three unusual cases with painful swelling of small joints.Tumoral calcinosis is a rare ectopic calcification syndrome characterized by irregular soft tissue masses originally described as being found mainly in large joints. We report 3 cases of tumoral calcinosis that occurred in unusual locations, the metacarpophalageal and proximal interphalangeal joints of the hand. The patients were women who ranged in age from 26 to 44; all presented with complaints of painful swelling of the joints. Laboratory tests demonstrated normal serum phosphate and calcium levels. Radiologic examination disclosed para-articular calcified masses. On surgical excision, the deep-seated calcified masses were attached to collateral ligaments of the joints. Milky and chalklike fluid was released during surgery. The masses measured 1.5, 0.9, and 0.8 cm in length and had irregular surfaces. Microscopically, the masses had fibrous capsule and the inner small cystic spaces that contained granular, calcified material. These cysts contained proliferating capillaries, mononuclear lymph plasma cells, and giant cells. No recurrences were found in any of the patients during the follow-up periods.- - - - - - - - - - ranking = 0.5keywords = giant (Clic here for more details about this article) |
6/28. Pigmented villonodular synovitis of the hip mimicking soft-tissue sarcoma: a case report.Pigmented villonodular synovitis is a rare and benign but potentially locally aggressive disease that should be considered in younger patients who present with monoarticular joint symptoms and pathology. We present a 30-year-old Sudanese woman with a huge mass arising from the right hip joint. A multimodality radiological approach to investigation and diagnosis is demonstrated and discussed. Histopathological examination of the resected specimen confirmed the diagnosis of pigmented villonodular synovitis with the mass consisting of a proliferation of fibrohistiocytic cells, abundant haemosiderin, foamy histiocytes, and occasional giant cells. The patient made a good recovery, with mobility aided by arm crutches and a hip abduction brace.- - - - - - - - - - ranking = 0.5keywords = giant (Clic here for more details about this article) |
7/28. The acute carpal tunnel syndrome: nine case reports.Nine cases of acute carpal tunnel syndrome are reported. Etiologies include: bleeding secondary to chronic lymphatic leukemia; colles' fracture of the wrist (2 cases); Epiphyseal fracture (Salter II) of the distal radius; Bleeding secondary to giant cell tumor of the tendon sheath; Unstable distal radio-ulnar joint; Displaced intra-articular fracture of the distal radius; Rheumatoid synovitis and vasculitis; Trans-scaphoid, perilunar fracture dislocation of the wrist. Early recognition of median nerve compression in the carpal tunnel is vital. The signs of median nerve compression should be looked for in all cases of wrist trauma. In our opinion, immediate surgical decompression is frequently indicated.- - - - - - - - - - ranking = 0.5keywords = giant (Clic here for more details about this article) |
8/28. Giant cell fibroblastoma. A case report.A case of giant cell fibroblastoma occurring in the knee of a 16-month-old girl is reported. The ill-defined subcutaneous tumor measuring 2 x 2 cm was composed of diffusely proliferating spindle-shaped tumor cells with scattered, atypical multinucleated giant cells in a myxoid or collagenous background and irregularly branching sinusoid-like tissue spaces. In addition to floret-type giant cells, a few osteoclast-like giant cells were present in a cellular area where tumor cells were focally arranged in a storiform pattern. Immunohistochemically, the tumor cells gave positive reactions for only vimentin and actin. In spite of the high recurrence rate of this type of tumor, the course of the patient after excision of the tumor has been uneventful. It is important to distinguish this rare, peculiar fibrous tumor from other soft tissue tumors including some sarcomas.- - - - - - - - - - ranking = 2keywords = giant (Clic here for more details about this article) |
9/28. Malignant giant cell tumor of synovium and locally destructive pigmented villonodular synovitis: ultrastructural and immunohistochemical study and review of the literature.The first reported case of an intraarticular malignant giant cell tumor of synovium studied with electron microscopic and immunohistochemical examination is presented, together with a case of diffuse intraarticular pigmented villonodular synovitis with extensive bone destruction. The malignant case was dominated by uniform cells positive for histiocytic markers, the fine structure showing a gradual change from cells dominated by organelles serving a secretory function to cells with phagocytic activity. The reported cases of giant cell tumor of the tendon sheath indicate that the pertinent histologic changes regarding malignancy are an increase in cell polymorphism and in the number of mitoses, and a decrease in the number of multinucleated giant cells.- - - - - - - - - - ranking = 3.5keywords = giant (Clic here for more details about this article) |
10/28. Empty cavity and giant tears.A nine-year-old girl presented with a loss of vision in her left eye after minor head trauma one month prior to being seen. ophthalmoscopy disclosed a complete retinal detachment with a giant tear. She also had serious orthopedic problems. Her father and brother had retinal detachment and joint diseases. The differential diagnosis is discussed and the diagnosis of Stickler's syndrome is made.- - - - - - - - - - ranking = 2.5keywords = giant (Clic here for more details about this article) |
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