Surface bone sarcomas: an update on current clinicopathological diagnosis and treatment

Abstract

Surface bone sarcomas are rare malignant bone tumours. Osseous and cartilaginous surface bone sarcomas are the most common, with parosteal and periosteal osteosarcomas, periosteal chondrosarcomas and secondary peripheral chondrosarcomas being the most frequent. Their clinical symptoms are non-specific and include pain for several months, swelling and limited range of motion of the adjacent joints. Prompt diagnosis is important, as biological behaviour, imaging and histopathologic characteristics, treatment and prognosis differ considerably from their conventional intramedullary counterparts. Moreover, their imaging characteristics are not infrequently non-characteristic and may be misinterpreted as juxtacortical benign lesions leading to incorrect diagnosis and treatment, with life-threatening repercussions. Molecular studies and histopathological sampling are essential for accurate diagnosis. There are still numerous issues regarding the biology, pathophysiology and treatment options of these entities due to their rarity. Cite this article: EFORT Open Rev 2021;6:905-917. DOI: 10.1302/2058-5241.6.210064

Caso clínico: Sarcoma de Ewing periostal en húmero proximal

“…10 Its main clinical presentation is localized pain and a sensation of a palpable mass. 2,5 The presence of fever or pathological bone fracture is common in advanced disease or metastasis. 2 Histologically, small uniform round cells are evident that are analogous to medullary and extra-skeletal forms, containing a round nucleus with chromatin and eosinophilic cytoplasm, with cytoplasmic membranes.…”

“…9 Currently, there is limited information about PES in the literature; It is known that its prognosis is more favorable than central ES, it is more prevalent in men, it usually does not present metastases and it has less aggressive clinical and radiological characteristics, frequently affecting the proximal aspect of the extremities. 1,[3][4][5]9,11,13,15 There is a retrospective study that evaluated the proportion of medullary, periosteal and extra-skeletal ES in 126 patients, finding that 88.9% of cases corresponded to the medullary variant while only 4.8% of cases were periosteal. Furthermore, a prognostic and therapeutic impact was distinguished according to the topographic categories, reaffirming better survival and prognosis rates in PES.…”

“…2,3 Other less frequent forms of presentation are extra-skeletal ES, where there is only soft tissue involvement without affecting bone tissue, and periosteal Ewing's sarcoma (PES), which originates from the periosteum, respecting the spinal canal, with an incidence of 12% and 3% respectively. 4,5 In 2020, the book of classifications of bone and soft tissue tumors of the World Health Organization (WHO) in its 5th edition, updates some definitions, identifying ES as a small round cell sarcoma that involves the fusion of a limb of the FET gene family (usually EWSR1) and a member of the ETS gene family. 2 One of the big changes in this new edition is that it identifies and separates as different entities all small round cell sarcomas that histologically resemble SE but lack the fusion between EWSR and ETS, 2,6-8 highlighting the role of molecular pathology in the diagnosis of these clinical entities.…”

“…2 3 Otras formas de presentación menos frecuentes son el SE extra-esquelético, en donde sólo hay compromiso de partes blandas sin afectar tejido óseo y el SE periostal (SEP), que se origina a partir del periostio, respetando el canal medular, con una incidencia del 12% y 3% respectivamente. 4 5…”

Caffeic Acid Inhibits Tumour Mass Formation in MG-63 Cells-induced Nude Mice

“…A formation of tumour mass, especially in the region of long bones, such as femur, tibia and humerus, is one of the symptoms of osteosarcoma development. (1,2) Osteosarcoma is a highly malignant bone tumour that typically composed of spindle cells producing disordered and immature osteoid. (3) An osteosarcoma grows in a radial manner and forms a ball-like mass.…”