Case report 782. Predominantly osteolytic Paget's disease of the calcaneus.
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Adult↗
Biomedical subjects
W Marsden
Publications and source records attributed to W Marsden.
Malignant transformation of osteoblastoma: study using image analysis microdensitometry.
AIM: To determine if the malignant transformation, as perceived histologically, in a case of osteoblastoma from the right femur, was also expressed as a quantitative change in nuclear DNA during tumour progression over five months. METHODS: Nuclear DNA microdensitometry by computer image analysis was used to acquire relative DNA distribution patterns. Tissue had been removed on four separate occasions from a lesion in the right femur of an 18 year old man. Retrospective DNA analysis was performed on formalin fixed, paraffin wax-embedded tissue. RESULTS: The DNA profile of the initial biopsy specimen, which was histologically diagnosed as osteoblastoma, was euploid with a near diploid (2c) modal DNA. The second biopsy specimen taken one month later also resembled osteoblastoma but showed an aneuploid DNA profile with a diploid modal DNA and some nuclei with ploidy greater than 5c. The third biopsy specimen taken four months later showed histological evidence of osteosarcoma and a near pentaploid (5c) modal DNA with large number of nuclei exceeding 5c. CONCLUSIONS: DNA microdensitometry confirmed the initial and final diagnosis. The technique also seems to be capable of detecting aneuploidy before malignancy is morphologically evident.
Adolescent↗
Fuhrmann syndrome of right-angle bowed femora, absence of fibulae and digital anomalies: two further cases.
We describe 2 patients with bilateral right-angle bowing of femora, absence of fibulae, and reduced, flattened and nail-less toes and symmetrical fingernail deficiency in a consanguineous family of ethnic South Vietnamese Baptists and a non-consanguineous Polish family. These cases are similar to those reported by Fuhrmann et al. (in "Skeletal Dysplasias," New York: Alan R. Liss, Inc., pp 519-524), indicating possible autosomal recessive inheritance of this rare non-lethal limb malformation syndrome.
Abnormalities, Multiple↗
Fibrosarcoma of metacarpal treated by combined therapy and immediate reconstruction with vascularized bone graft.
Fibrosarcomas of the bones of the hand are very rare entities. We present a case of low-grade fibrosarcoma of the left third metacarpal that was successfully treated by preoperative intraarterial chemotherapy, wide local excision, and immediate reconstruction with a vascularized bone graft from the ipsilateral radius. Excellent hand function has been preserved and the patient remains free of recurrence 5 years after presentation.
Adolescent↗
Regional chemotherapy with the use of cisplatin and doxorubicin as primary treatment for advanced sarcomas in shoulder, pelvis, and thigh.
Eight patients who had large sarcomas in the hip, thigh, or shoulder girdle have been described. Three had osteogenic sarcomas, and one each had Ewing's sarcoma, biphasic synovial sarcoma, pleomorphic liposarcoma, undifferentiated spindling sarcoma, and malignant fibrous histiocytoma. All eight tumors showed evidence of regression after intraarterial infusion of cisplatin and Adriamycin (doxorubicin) given over 48 hours at 3-week intervals, for a total of between three and seven courses. Tru-cut needle biopsy specimens of five of the lesions were normal after chemotherapy. However, after resection of the regressed fibrotic tumor in seven of the patients, four contained foci of probably viable malignant cells. These cell foci were intraosseous in three cases and in the wall of a cyst in one case. In the remaining case, tumor in the distribution of the infused artery regressed, but tumor in a region supplied by an artery that was not infused continued to enlarge. In one patient with osteogenic sarcoma in the pelvis, despite a good response to intraarterial chemotherapy that was followed by surgical resection and radiotherapy, tumor recurred in an adjacent area in tissues supplied by an artery not infused. A hindquarter amputation subsequently was required. With the exception of the two cases in which adequate tumor arterial infusion was not achieved, local primary tumor control was accomplished by intraarterial infusion chemotherapy followed by local resection or radiotherapy and local resection in all patients. Four patients are well without evidence of residual or metastatic sarcoma 3.5 years after presentation in the case of an osteogenic sarcoma of shoulder, 2.5 years after presentation in the case of a large pleomorphic liposarcoma of thigh and groin, 20 months after presentation in the case of lower-thigh malignant fibrous histiocytoma, and 1 year after presentation in a child with an osteogenic sarcoma of lower femur.
Adolescent↗