Imre Antal

Good prognosis of localized osteosarcoma in young patients treated with limb-salvage surgery and chemotherapy

The objective of this report was to estimate long‐term outcome and prognostic factors in children and adolescents with osteosarcoma. A large group of osteosarcoma patients were analyzed at our national oncology center.

Calcitonin therapy of aneurysmal bone cysts.

SummarySeven aneurysmal bone cysts (ABC) were treated with the hormone calcitonin. Six of the cysts, which were hypovascular responded well to the calcitonin administered directly into the cyst. Ossification and rebuilding of the ABC occurred after some months in every case. One hypervascularized ABC, however, failed to respond either to embolotherapy or to the calcitonin hormone treatment. The authors recommend calcitonin administration as a useful non-invasive method for the treatment of hypovascular ABC.

Fibrous dysplasia associated with intramuscular myxoma (Mazabraud's syndrome): a long-term follow-up of three cases

Abstract The association between of fibrous dysplasia and intramuscular myxomas is extremely rare. The authors summarize the characteristics of Mazabrauds syndrome, as well as its clinical course on the basis of 24 cases reported in the literature and 3 cases of their own. The syndrome is quite uniform, and mostly occurs in women. Usually the presentation of poliostotic fibrous dysplasia bilaterally in the lower limbs and pelvic bones is followed by the appearance of multifocal intramuscular myxomas in the adjacent muscles, mostly decades later. These hamartomas tend to recur locally or symmetrically in the ipsilateral muscle groups and may reach enormous size without treatment. Malignant transformation (osteosarcoma, fibrosarcoma) on the basis of fibrous dysplasia in Mazabrauds syndrome has been reported in the literature. In our cases, however, in spite of the many recurrences, and the enormous size of the tumours, no malignant transformation was noted in either the fibrous dysplasias or the intramuscular myxomas during the long follow-up time (31 years for fibrous dysplasia and 16 years for myxomas). Early wide surgical excision and a carful long-term follow-up for the often very late recurrences of the myxomas is suggested in Mazabrauds syndrome.

The prognostic significance of DNA cytophotometry and proliferation index (Ki-67) in giant cell tumors of bone

Abstract We studied DNA ploidy by smear cytophotometry and proliferation activity by Ki-67 MIB immunohistochemistry in 69 primary and recurrent giant cell tumors (GCT) from 50 randomly selected patients. The obtained results were evaluated with comparisons made to the available clinical data. From the 46 primary tumors 63% showed diploidy and 37% aneuploidy. A significantly (P=0.026) higher recurrence rate (64%) was observed in aneuploid than in diploid tumors (31%). In the course of the recurrences, both the ratio of aneuploid tumors as well as the proliferation index of the tumors increased, though the degree of the latter was non-significant. Aneuploidy did not mean an unambiguous tendency towards malignant transformation; however, a close follow-up of recurrent aneuploid tumors, and wide excision of the recurrence instead of intralesional curettage are the recommended procedures. The DNA cytophotometry and proliferation index of GCTs – as compared to other histologic examinations – are of prognostic value in the evaluation of the recurrence potential of the GCTs.Résumé Les auteurs ont examiné 1’anomalie chromosomique de DNA á 1’aide de cytophotometrie de frottis; et 1’activité de proliferation par le moyen de 1’immuno- histochimie Ki-67 MIB dans 69 tumeurs de cellules géantes de 50 patients choisis accidentellement. lls ont évalué les résultats en les comparant aux données cliniques disponibles. Parmi les 46 tumeurs primaires 63% ont manifesté un caractère diploide et 37% un caractère aneuploide. On a observé une proportion de recidive significativement plus élevée (P=0.026) dans les tumeurs aneuploides (64%) que dans les tumeurs diploides (31%). Au cours des récidives on a remarquè une augmentation tant dans la proportion des tumeurs aneuploides que dans 1’indice de proliferation mais en ce dernier 1’augmentation n’était pas significative. Le caractère aneuploide ne signifie pas forcément une tendance univoque á la transformation maligne cependant dans les tumeurs aneuploides récidivantes il est nécessaire de suivre la maladie avec beaucoup de soin et comme intervention chirurgicale on conseille une excision large au lieu du curettage intralésionel.

Multicentric extraabdominal desmoid tumour: A case report

The very rare condition of multicentric desmoid tumours involving two distant and apparently independent sites is reported in a 17-year-old man. The tumours grew simultaneously and reached approximately equal size. No evidence of familiar polyposis or any other feature of Gardners syndrome were noted. The proximal desmoid tumour developed from the left hip region and extended into the femoral bone, whereas the distal mass was attached to the left popliteal fossa infiltrating the flexor muscles, the nerves and vessels. On the basis of the good results published recently in the literature and our own earlier experiences, the intralesional resection of the desmoid tumours was completed with postoperative fractionated radiotherapy.

Her-2 Oncogene Amplification, Chromosome 17 and DNA Ploidy Status in Synovial Sarcoma

The treatment options for synovial sarcoma (SS) are very limited, though this type of sarcoma seems to be more heterogeneous than it has been traditionally considered. The present study investigates the Her-2 oncogene status of 20 cases of SS, to determine whether Her-2 amplification can be considered as a prognostic factor. Her-2 oncogene amplification was determined on smears (frozen material was used from our tumor bank in each case), using fluorescence in situ hybridization technique (dual color FISH with centromeric probe for chromosome 17 and specific probe for Her-2 oncogene). Moreover, protein expression was assessed by immunohistochemistry, and DNA ploidy status was measured using image analysis. We had 5 biphasic and 15 monophasic SSs, patients’ age ranged from 13 to 68 years (mean, 39.8 years). Tumor size was larger than 5 cm in each case. Followup time ranged from 6 to 78 months (mean, 38.5 months). For statistical analysis the chi-square test was used. Her-2 oncogene amplification was found in three cases (15.0%) of 20 SSs. These cases proved to be 2+ positive by immunohistochemistry, but massive amplification, characteristic of a subset of breast carcinomas, was not observed. Her-2 oncogene amplification was significantly associated with a lower risk of developing metastasis (P<0.05) (none of the 3 amplified cases had metastases), while no association was found with recurrence. Six cases proved to be aneuploid and 14 were diploid, but no association was found between Her-2 amplification status and ploidy, and between ploidy status and metastasis or recurrence. Our results emphasize and confirm that Her-2 oncogene amplification is a rare event in SS, but the small subset of SS with Her-2 amplification has a better overall prognosis. Furthermore, this may open a theoretically new treatment possibility with Trastuzumab for Her-2-amplified cases of SS.

Functional outcome and life quality after endoprosthetic reconstruction following malignant tumours around the knee.

Between 1993 and 2002, we treated 43 patients with malignant musculoskeletal tumours of the knee region. All patients had partial resection of the femur or tibia together with endoprosthetic replacement. We were able to follow-up 23 patients with an average follow-up of 30 (12–97) months. Complications occurred in ten cases, of which one was a case of local recurrence. Most of the patients were completely satisfied with their condition, with a decreased walking distance as the only notable restriction. There was no correlation between the functional outcome and life quality assessment and the type of the implanted prosthesis, length of resected bone and type of resection. However, patients with tumours in the distal femur had significantly better functional and life quality outcome than those with a proximal tibial tumour.RésuméEntre 1993 et 2002 nous avons traité 43 malades avec une tumeur musculo-squelettique maligne de la région du genou. Tous les malades avaient une résection partielle du fémur ou du tibia avec remplacement prothétique. Nous avons suivi 23 malades avec un délai moyen de 30 (12–97) mois. Des complications se sont produites dans 10 cas dont une récidive locale. La plupart des malades ont été satisfaits du résultat avec un périmètre de marche diminué comme seule restriction notable. Il n’y avait aucune corrélation entre, d’une part les résultats fonctionnels et la qualité de vie et, d’autre part, le type de résection, la longueur de résection osseuse et le type de prothèse implantée. Cependant, les malades avec une tumeur du fémur distal avaient de meilleurs résultats pour la fonction et la qualité de vie que ceux atteints d’une tumeur du tibia proximal

Diagnostic algorithm, prognostic factors and surgical treatment of metastatic cancer diseases of the long bones and spine

Oncological management of skeletal metastases has changed dramatically in the last few decades. A significant number of patients survive for many years with their metastases. Surgeons are more active and the technical repertoire is broader, from plates to intramedullary devices to (tumour) endoprostheses. The philosophy of treatment should be different in the case of a trauma-related fracture and a pathological fracture. A proper algorithm for establishing a diagnosis and evaluation of prognostic factors helps in planning the surgical intervention. The aim of palliative surgery is usually to eliminate pain and to allow the patient to regain his/her mobility as well as to improve the quality of life through minimally invasive techniques using life-long durable devices. In a selected group of patients with an oncologically controlled primary tumour site and a solitary bone metastasis with positive prognostic factors, which meet the criteria for radical excision (approximately 10% to 15% of the cases), a promising three to five years of survival may be achieved, especially in cases of metastases from breast and kidney cancer. Spinal metastases require meticulous evaluation because decisions on treatment mostly depend on the tumour type, segmental stability, the patient’s symptoms and general state of health. Advanced radiotherapy combined with minimally invasive surgical techniques (minimally invasive stabilisation and separation surgery) provides durable local control with a low complication rate in a number of patients. Cite this article: EFORT Open Rev 2017;2:372-381.

Midterm outcome after rotator cuff reconstruction

UNLABELLED Rotator cuff tear is a common degenerative shoulder disorder that often requires surgical treatment. However, the correlation between the size of the tear and the functional results is somewhat controversial, which generates inconsistency among orthopaedic surgeons about the indications for and methods of rotator cuff reconstruction. AIMS The aim of the authors was to evaluate the midterm functional outcome after rotator cuff reconstruction and the possible connection between the results and the surgical technique or the postoperative ultrasound examination. In addition, recently published corresponding studies are also reviewed by the authors. METHODS Twenty-seven patients with full thickness rotator cuff tear were enrolled into the study who were treated either by arthroscopic (14 patients) or by open repair (13 patients) technique. Functional results were assessed using clinical tests. Ultrasound examination was also performed. RESULTS The average postoperative Constant Score was 73, the average DASH (Disabilities of the arm, shoulder and hand score) was 14. The Constant scores averaged 80 for the arthroscopic and 70 for the open group. Ultrasound examination showed partial or full thickness retear of the cuff in 40% of the cases. CONCLUSION More than 70% of the patients had excellent or good results two years after the reconstruction. The change in the acromiohumeral distance or partial retear failed to affect the results significantly, but full thickness tear had an effect on them.

Die maligne Entartung eines Riesenzelltumors am distalen Radiusende

ZusammenfassungDer Riesenzelltumor ist ein primärer Knochentumor von intermediärer Dignität. Die Vorhersage des biologischen Verhaltens und des Krankheitsverlaufs ist nach konventionellen histologischen Diagnosemaßnahmen kaum möglich. Anhand einer Fallbeschreibung einer malignen Entartung eines Riesenzelltumors am distalen Radius wird die Möglichkeit und die Bedeutung der DNS-Zytophotometrie dargestellt. Mit Hilfe dieses Untersuchungsverfahrens kann das biologische Verhalten der Riesenzelltumoren besser vorhergesagt werden.AbstractThe GCT is a primary bone tumor of intermedier dignity, whereby the forecast of the biological behaviour and outcome is impossible on the base of the conventional histological diagnosis. The authors observed in one of their GCT-patients a malignant transformation of the formerly benign tumor. Through announcement of their case the authors want to call the attention to the importance of the DNA-Cytophotometry and to their possible future additional role in the diagnostics of the GCT and in the prediction of their biological behaviour.