PROJECT: Tumors at the elbow –
clinical and oncological results
after resection and endoprosthesis replacement
Invitation to participate the study
Dear colleagues, hereby we invite you to participate in a multicentric retrospective study “Tumors at the elbow –
clinical and oncological results after resection and endoprosthesis replacement”.
Bone and soft tissue tumors involving the elbow are very rare. There is a limited knowledge about
clinical and oncological results of distal humerus and/or proximal ulna resection and endoprosthetic elbow
reconstruction: in the last two decades only a few publications analyzed this problem, including per study
at average about 20 patients. These studies include numerous various parameters (different implants,
various diagnosis…), making the task of summarizing the results not easy.
Dr. Marko Bergovec
Elbow TM-invitation.pdf141.66 K
EMSOS study “Sarcoma during Pregnancy”
Malignancy during pregnancy is a very rare entity with an estimated incidence of 1/1000 pregnancies.
Individual therapeutic customization is the only choice of treatment with respect to the health of the
mother, and her child.
The occurrence of sarcoma during pregnancy is even more exceptional, with about 140 reported cases
in the accessible literature. 60% is reported as primary soft tissue sarcomas, 40% as primary bone
sarcomas. No true systematic study has been undertaken to investigate the whole diagnostic,
therapeutic and prognostic concept of these sarcomas during pregnancy as an ‘entity’.
University Department of Experimental Pathology, Vrije Universiteit Brussel, Belgium.
EMSOS study Sarcoma during Pregnancy.pdf136.25 KB
Expandable distal femoral megaprostheses
Over the last 20 years, expandable prostheses have made a significant impact on the reconstruction methods we routinely use in children with bone tumors.[1-4] Although these implants are expensive and associated with a high complication rate [5-9], even in experienced hands, they are considered the main reconstructive option for children in a certain age group and for selected bone segments.
Musculoskeletal Oncology Department
Istituto Ortopedico Rizzoli, Bologna, Italy
EMSOS study on expandable distal femoral megaprostheses.pdf190.02 KB
Desmoplastic Fibroma of bone
Fibroma of bone is a rare, locally aggressive bone tumor. Once it was called the osseous counterpart of extra-abdominal desmoid tumor, because of the histological aspects. In 2013, the WHO described the microscopic appearance of desmoplastic fibroma as being composed of slender, spindle to stellate cells with minimal cytological atypia and abundant collagenous matrix.
Desmoplastic Fibroma of bone.pdf135.88 KB
Desmoplastic Fibroma EMSOS study datasheet.xls48.00 KB
EMSOS Extra-abdominal Fibromatosis Multicenter Study
Desmoid-type fibromatosis is an intermediate soft tissue tumour (WHO) with clonal fibroblastic proliferation originating in the deep soft tissues. It is characterised by local infiltration without metastatic potential. In the past decades resectable masses have been mainly treated surgically with a high incidence of local recurrence in up to one third of the patients independently from resection margins. Local recurrence negative prognostic factors after surgery have been identified in younger age, female gender, distal limb location and recurrent disease.
Alternative management including radioterapy or medical treatment with NSAIDS, hormone therapy, chemotherapy and tyrosine kinase inhibitors led to high recurrence rate as well.
As a consequence in recent years the wait and see approach, with strict and close clinical and instrumental monitoring of the involved site has gained popularity as first line management for silent masses not involving neurovascular bundles.
Clear cell chondrosarcoma
Clear cell chondrosarcoma is a rare variant of chondrosarcoma, behaving as a low grade malignant bone tumor and characterized by a proliferation of tumor cells with clear cytoplasm.
Clear cell chondrosarcomas account for around 2% of all chondrosarcomas. They have been described in most bones of the body but usually affect the proximal epiphysis of the humerus or femur.
Doz. Dimosthenis Andreou, Münster, Germany
Mazabraud Syndrome is a very rare combination of fibrous dysplasia (FD) and intramusculary myxomas. Both entities are caused by activating GNAS-mutations and while FD is often diagnosed at a younger age, myxomas tend to arise later, often in the fourth or fifth decade of life, and are localized adjacent to FD lesions in the majority of the patients. The myxomas can lead to mechanical problems and symptoms of pain. Excision of the myxomas has been proposed, although outcome of surgical interventions and whether there are predictive factors for recurrence of the myxomas remains eluded.
EMSOS Study "Angiosarcoma"
Angiosarcoma is the malignant end of the spectrum of vascular tumors, spanning diagnosis including hemangiomas, hemangioendothelioma, well-differentiated and poorly-differentiated angiosarcoma.
Angiosarcoma of bone is rare, accounting for <1% of all primary bone sarcomas and it is associated with a poor prognosis. Although any age can be affected, the incidence is highest between 50 and 70 years of age.
Angiosarcoma of bone may present as unifocal or multifocal disease. The most common locations of unifocal tumor are the long and short tubular bones, followed by the pelvis, and trunk.
Histologically, angiosarcomas of bone are composed of anastomosing vascular channels lined by atypical endothelial cells with enlarged nuclei, prominent nucleoli, and increased mitoses. Inflammatory cells, mostly eosinophils, may be present.
Given the rarity of angiosarcoma of bone, the literature is limited regarding treatment and outcome of patients with this tumor.
The role of chemotherapy and prognostic factors for these patients remain unclear.
Under the auspices of the EMSOS, a retrospective study has been launched with the aim of improving our knowledge on the natural history of the tumor and on prognostic and predictive factors of survival.
Stefano Ferrari MD
SSD Chemioterapia dei Tumori Muscoloscheletrici
Istituto Ortopedico Rizzoli – WWW.ior.it
Via Pupilli 1, 40136 Bologna. Italy
DGOOC / EMSOS study "Clear cell sarcoma"
Osteosarcoma after retinoblastoma
European Relapsed Osteosarcoma Registry "EURELOS"