More than 10 years of follow-up of two patients after total femur replacement for malignant bone tumor

One patient with osteosarcoma and one with Ewing’s sarcoma of the femur were in 1987 and 1988 treated with prosthetic replacement of the femur and chemotherapy. There has been no loosening of the prostheses and no recurrence of the tumor. The patients have maintained 60% and 63% limb function scores evaluated by ISOLS criteria.

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Résumé  Deux patients, l’un avec un ostéosarcome, l’autre avec un sarcome d’EWING, furent opérés en 1987–1988 avec remplacement prothétique du fémur, associéà une chimiothérapie. Ces patients ont été suivis sans qu’il soit noté de récidive. Il n’y a pas de descellement, ni de luxation de la prothèse. Un score fonctionnel évalué selon les critères de l’ISOLS, montre une conservation de 60% et 63% de la fonction du membre inférieur.

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Accepted: 17 March 2000     

Angiomyolipoma arising in the thigh

A 41-year-old man presented with an asymptomatic mass in the right medial thigh. Magnetic resonance imaging (MRI) revealed a well-demarcated, 10-cm mass in the right adductor muscles. The margins of the mass exhibited high signal intensity and the rest showed low or iso signal intensity on T1-weighted MR images. However, the high signal intensity was decreased on T2-weighted images with fat suppression. The central part of the tumor was of inhomogeneous high signal intensity on T2-weighted images; after Gd-DTPA injection it enhanced inhomogeneously on T1-weighted images with fat suppression. On dynamic computed tomography (CT) in the arterial phase, there were strongly enhancing spotty areas in the tumor. At surgery, a yellow-whitish tumor was resected and a pathological diagnosis of angiomyolipoma (AML) in the thigh was made. Received: 21 June 1999 Revision requested: 28 July 1999 Revision received: 13 December 1999 Accepted: 15 December 1999     

Multiple bone lesions after allogeneic bone marrow transplantation in a patient with relapsed adult acute lymphoblastic leukemia: minimal residual disease analysis may predict extramedullary relapse

We describe a patient with acute lymphoblastic leukemia (ALL, L2) who relapsed with multiple bone lesions after allogeneic bone marrow transplantation (allo-BMT). Allo-BMT was performed from an HLA-identical sibling during the first hematological complete remission (CR). Minimal residual disease (MRD) assessed by polymerase chain reaction (PCR) with primers for T cell receptor delta (TCRdelta) gene became positive in the bone marrow sample on day 46 after allo-BMT. On day 113, the patient complained of a painful tumor at the right clavicle. The examination of biopsy specimen revealed infiltration of leukemic cells. After partial response was achieved by local radiotherapy, disseminated bone lesions were demonstrated by 99mTC scintigraphy scan, followed by bone marrow relapse on day 137. The patient died of cardiac tamponade on day 236 after Allo-BMT. MRD assessed by PCR assay for TCRdelta gene in the bone marrow is useful for the prediction of extramedullary as well as medullary relapse after BMT.     

Adriamycin binding assay: a valuable chemosensitivity test in human osteosarcoma

Summary The reliability of a simple method evaluating the pattern of subcellular binding of Adriamycin (Adriamycin binding assay, ABA) as an index of sensitivity was demonstrated in different primary cultures and in sensitive and resistant cell lines of human osteosarcoma. After exposure to Adriamycin (10 g/ml for 30 min at 37°C), living sensitive cells showed selective intranuclear uptake of the drug, whereas in resistant cells no distinct subcellular distribution was observed. The binding pattern of Adriamycin in sensitive and in highly resistant cells was inversely related to the expression of P-glycoprotein. However, low levels of resistance in vitro, not detectable by increased levels of expression of P-glycoprotein, were revealed by ABA. The use of ABA in combination with the estimate of P-glycoprotein expression is recommended in clinical practice as an accurate means for predicting the sensitivity of osteosarcoma to Adriamycin.This study was supported by a grant from the Associazione Italiana per la Ricerca sul Cancro (A.I.R.C.).     

Effect of radioactivity outside the field of view on image quality of dedicated breast positron emission tomography: preliminary phantom and clinical studies

Annals of Nuclear Medicine - Semi-quantitative positron emission tomography (PET) values, such as the maximum standardized uptake value (SUVmax), are widely used to identify malignant lesions and...     

Asymptomatic disseminated carcinomatosis of bone marrow presenting as hyperphosphatasia: report of a case

Metastatic involvement of the musculoskeletal system is one of the most significant clinical issues facing orthopaedic oncologists. The number of patients with metastasis to the skeletal system from a carcinoma is 15 times greater than the number of patients with primary bone tumours of all types. However, progression patterns like disseminated carcinomatosis of bone marrow are comparatively rare. The pathophysiology for disseminated carcinomatosis of bone marrow, with a prognosis reported to be very poor, is still unknown. We describe a patient who had no symptoms with hyperphosphatasia. Bone scintigraphy showed a so-called super bone scan and a needle biopsy from the ileum showed adenocarcinoma cells. Additional endoscopic investigation was performed and signet cell gastric cancer was found. From the bone scan and biopsy, we established the diagnosis of disseminated carcinomatosis of the bone marrow. From the experience of this case, we believe that intensive stomach investigation should be considered in cases with hyperphosphatasia, even when the patient has no symptoms.     

Extraskeletal osteosarcoma arising in myositis ossificans

A 53-year-old woman had extraskeletal osteosarcoma that developed from a soft tissue bony mass present on the volar aspect of the left wrist for 4 years. Initially, the bony mass was soft and movable, but during the first year it became hard and fixed. The patient had no history of trauma. Because the lesion did not grow or cause any symptoms, the patient did not come to the hospital until 4 years after she first noticed the lesion. Radiologically, the bony mass had features characteristic of mature myositis ossificans, showing ”eggshell” ossification. A nonmineralized soft tissue mass occurred between the surface of the radius and the bony shell. Histologically, a high-grade osteosarcoma was present between the surface of the radius and the well-differentiated bone tissue, which included fatty and hematopoietic marrow. All the findings indicated that our patient had an extremely rare case of malignant transformation of myositis ossificans. Received: 22 June 2000 Revision requested: 2 August 2000 Revision received: 14 September 2000 Accepted: 25 September 2000     

Osteoblastic differentiation and P-glycoprotein multidrug resistance in a murine osteosarcoma model

A recent study of multidrug resistance (MDR) 1 gene transfected osteosarcoma cells found a cause-effect relationship between increased expression of P-glycoprotein (P-gp) and a low aggressive phenotype. However, several experimental and clinical studies have observed contradictory findings in that P-gp expression has been associated with tumour progression. In the present study, we characterized P-gp-positive and P-gp-negative single-cell clones of a murine osteosarcoma, to further investigate the relationship between P-gp expression and changes in cell phenotype. Although these clones were all selected by doxorubicin (DOX) exposure, they were heterogeneous with respect to MDR1 gene expression. The P-gp-positive clones revealed MDR phenotype, whereas the P-gp-negative clones showed no resistance to drugs. Morphological and functional analysis showed that both the P-gp-positive and P-gp-negative clones were more differentiated than the parent cells in terms of enhanced activity of cellular alkaline phosphatase, an increase in well-organized actin stress fibres and enhanced osteogenic activity. Moreover, these subclones all displayed a decrease in malignant potential such as oncogenic activity, tumour growth rate and metastatic ability, regardless of their P-gp status. These results indicate that the observed osteoblastic differentiation and less aggressive phenotype in DOX-selected osteosarcoma cells may not only be explained by the direct effect of P-gp, and accordingly, consideration of the effect of DOX, as well as P-gp, appears to be important.     

Relationship between binuclear and multinuclear cells in giant cell tumor of bone

Giant cell tumor of bone (GCT) consists of stromal and multinuclear type tumor cells. Although most people believe that the stromal cells are mononuclear, we recently found the existence of many binuclear cells among stromal cells using DNA cytofluorometric examination. This study, using 18 tumors of GCT was conducted to elucidate the cell biological significance of the binuclear cell, especially its relationship to multinuclear cell formation or tumor cell proliferation. The investigation was carried out by means of DNA-RNA cytofluorometry with acridine orange (AO) and histological method. Using fluorescence microscopic observation, we counted the numbers of both mononuclear and binuclear cells and calculated the index of % BNC, which expresses the frequency (percentage) of binuclear cells in a population of mononuclear and binuclear cells. The index of % S-G2 obtained by DNA-RNA cytofluorometry showed the frequency (percentage) of mononuclear cells in the S and G2 phases of the cell cycle. In the histological study, we counted the numbers of multinuclear giant cells with more than 3 nuclei in the cytoplasm and stromal cells including mononuclear and binuclear cells and calculated MNS/SC, which showed the percentage of multinuclear cells in the stromal cells in the microscopic field. Eight tumors showed a value of % BNC greater than 10% and 2 had a value of 40%. The index of % BNC significantly correlated with the average value of MNC/SC in all tumors. There was no significant correlation between % BNC and the average value of % S-G2, in 18 tumors although 4 tumors having a % BNC value greater than 20% showed a % S-G2 value greater than 12% in 18 tumors. These results revealed the presence of many binuclear cells among stromal cells of GCT and suggested that these binuclear cells might be formed in association with the active proliferation of mononuclear cells and closely relate to the formation of multinuclear giant cells.