Header Emsos

EMSOS studies planned/ ongoing

Periprosthetic Joint Infection in Megaprostheses following Bone Sarcomas

Infection rate in end-prosthetic reconstruction ranges from 3 to 30%, with even 60% for revision arthroplasty. Due do use of neoadjuvant chemotherapy, bone sarcoma patients are at particularly high risk to develop periprosthetic joint infections (PJI). This EMSOS study aims at systematically collecting data from patients treated for PJI of megaprostheses following reconstruction for bone sarcoma surgery.

Contact
Dr. Andrea Sambri
Email: andra.sambri@aosp.bo.it

Management of Skeletal Metastases

The current retrospective study aims at obtaining data related to anatomical location and extent, radiological presentation and choice of surgical treatment policy of metastatic bone disease located at the shoulder girdle, upper and lower extremities, and pelvic girdle.

Based on the data ascertained, the clinical and radiological presentation as well as treatment strategy of metastatic bone disease with regards to histological type and medical history will be analysed.

Contact
Prof. Jacob Bickels
Email: MBDemsos@gmail.com

Prof. Domenico Andrea Campanacci

A retrospective study of local relapse after high grade osteosarcoma

Aim of this study is to analyse the modalities of diagnosis of LR in high-grade osteosarcoma, pattern of recurrence, treatment of LR and factors influencing post-LR survival (PLRS) focusing on the role of second line chemotherapy, margins and type of surgery

Contact
Emanuela Palmerini, Bologna
Email: emanuela.palmerini@ior.it

EMSOS + EPOS bony cyst retrospective cohort collaborative study

Unicameral bone cysts (UBC), also called simple bone cysts, Aneurysmal bone cysts (ABC) (and Fibrous Dysplasia (FD) lesions) can present as benign fluid filled cavities that can enlarge over time, leading to thinning and with that weakening of the bone. Both bone cysts and FD predominantly occur in children and adolescents, with a peak age between 3 and 14 years [1-3]. The annual prevalence and incidence are both estimated at ±0.3 per 100.000 people for UBC [4, 5]. For ABC, the annual prevalence is estimated at ±0.3, with an annual incidence ranging from 0.14 to 0.54 per 100.000 people [1, 4-6]. However, the true prevalence and incidence is difficult to estimate due to clinically silent cases and spontaneous regression. For the same reason the incidence and prevalence of FD is unknown and no estimation is given in literature.

Contact
Michiel van de Sande (Leiden)
M.A.J.van_de_Sande@lumc.nl

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.

Contact
Dr. Marko Bergovec
marko.bergovec@medunigraz.at

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’.

Contact
University Department of Experimental Pathology, Vrije Universiteit Brussel, Belgium.
Prof. dr. Ramses Forsyth, director (ramses.forsyth@vub.be).

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.

Contact

Frank Traub MD, PhD | Frank.Traub@med.uni-tuebingen.de
Dimosthenis Andreou, M.D. | Dimosthenis.Andreou@ukmuenster.de

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.

Contact
Doz. Dimosthenis Andreou, Münster, Germany
Dimosthenis.Andreou@ukmuenster.de

Mazabraud syndrome

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.

If you need any assistance please contact Bas Majoor,  b.c.j.majoor@lumc.nl

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.

Contact
Stefano Ferrari MD
SSD Chemioterapia dei Tumori Muscoloscheletrici
Istituto Ortopedico Rizzoli – WWW.ior.it
Via Pupilli 1, 40136 Bologna. Italy
Stefano.ferrari@ior.it

European Relapsed Osteosarcoma Registry "EURELOS"

Eurelos European Relapsed Osteosarcoma Registry