extraskeletal of the face

INTRODUCTION: Chondrosarcomas are rare malignancies of the cartilage and myxoid chondrosarcoma is its variant which commonly occurs in soft tissue of extremities. Extraskeletal chondrosarcoma is a rare malignant neoplasm of bone or soft tissue origin and is characterized by the presence of spindle cells admixed with well differentiated cartilage or chondroid stroma. They are mostly radioresistant tumours and surgical resections with adequate margins is considered as the ideal treatment modality with adjuvant radiotherapy in high grade tumours and add on chemotherapy, in case of presence of poor prognostic factors...

Extraskeletal osteosarcoma (EOS) is a malignant tumor producing bone matrix and/or chondroid material, without direct attachment to bone or periosteum. In humans and dogs, EOS is highly infiltrating, rapidly growing, often characterized by osteoid deposition and variable ossification, similar to primary skeletal osteosarcoma (SOS). In dogs, EOS arises from visceral and soft tissue locations, occasionally in trauma or foreign body sites, or in granulomas. Few data are currently available on the phenotype of these tumors...

AIMS: Extraskeletal myxoid chondrosarcoma (EMC) is a rare form of adult sarcoma with distinct histology and NR4A3 gene fusion. Immunohistochemically, EMCs are variably positive for S100 protein and neuroendocrine markers. Unlike histologically similar soft-tissue myoepithelial tumours, keratin expression is rare. Prompted by two recent EMC cases with diffuse keratin expression, we investigated the expression of epithelial markers in a molecularly confirmed cohort of EMC and identified two additional similar cases...

Osteoma is a benign, slow growing lesion that consists of compact or cancellous bone. Three types of osteomas could be classified: the central osteoma arising from the endosteum, the peripheral osteoma from the periosteum, and the extraskeletal soft tissue osteoma. in the craniofacial region, peripheral osteomas of the zygoma are quite rare. A literature review identified 7 cases of zygomatic arch and 3 cases of zygomatic body. This is the first report of zygomatic osteoma that was endoscopically removed. This report presents a rare case of osteoma of the zygoma and its endoscopic approach...

AIM: Cutaneous bone formation is an uncommon lesion of the skin. It may be primary or secondary. Secondary lesions are mostly associated with melanocytic nevi. Although many different theories have been proposed to explain the etiology, extraskeletal bone formation is complex and poorly understood phenomenon.Here the authors report a series of melanocytic nevi with cutaneous bone formation and the authors described morphologic and clinicopathologic features such as age, sex, location, focus number and size of the lesion...

Osteoma of the skull is a benign slow-growing osteogenic lesion typically composed of well-differentiated mature bone tissue. It is characterized by the proliferation of compact or cancellous bone and is found almost exclusively in the head and neck region. Central, peripheral, and extraskeletal are the major variants of craniofacial osteomas. Trauma, inflammation, developmental disorders, and genetic defects are considered their etiologic factors. Paranasal sinuses, especially frontal and ethmoidal sinuses, are the favorite locations of peripheral craniofacial osteomas...

Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis of unknown etiology, characterized by multi-organ involvement. ECD is usually diagnosed on the basis of characteristic radiologic and histopathological findings. Lesions may be skeletal and/or extraskeletal in location, and may include the skin, lung, heart, and central nervous system. We describe here a 68-year-old man with multiple yellowish plaques and a pinkish nodule on his face and scalp. He had been previously diagnosed with diabetes insipidus, and recently complained of coughing and dyspnea...

We report, to our knowledge, the first case of a congenital, widespread, aggressive high-grade sarcoma, presented as multiple skin nodules and respiratory distress in a neonate that had a t(9;22)(q22;q11-12) cytogenetic abnormality suggestive of a more indolent extraskeletal myxoid chondrosarcoma (EMC). EMC is generally thought of as a slow-growing tumor that presents between the fourth and sixth decades of life. Our patient was a 45,XY, t(13;14) newborn who presented at birth with subcutaneous nodules involving the face, scalp, back and extremities, as well as multiple intrathoracic, intraabdominal and intracranial masses...

Besides bringing problems, aging can let the mind's eye see more clearly than before, and it can let us express ourselves better. As age, experience and common sense examine today's skeletal medicine and surgery two questions keep popping up: A) How did we fail?; B) How to make it better? The Utah paradigm of skeletal physiology and the seminal ISMNI offer some answers, but exploiting them faces problems. Problem #1: By 1960 all clinicians and physiologists 'knew' (as the ancients 'knew' this world is flat) that effector cells controlled solely by nonmechanical agents explain all skeletal physiology and disorders ('effector cells' include osteoblasts, osteoclasts, chondroblasts and fibro-blasts)...

Secondary hyperparathyroidism (HPT) is a common complication of chronic kidney disease (CKD) that can lead to clinically significant bone disease. Additional consequences of secondary HPT, such as soft-tissue and vascular calcification, cardiovascular disease, and calcific uremic arteriolopathy, may contribute to the increased risk of cardiovascular morbidity and mortality among CKD patients. Secondary HPT arises from disturbances in calcium, phosphorus, vitamin D and parathyroid hormone metabolism, which develop early in the course of CKD and become more prominent as kidney function declines...

Schinzel phocomelia syndrome is characterized by limb/pelvis hypoplasia/aplasia: specifically, intercalary limb deficiencies and absent or hypoplastic pelvic bones. The phenotype is similar to that described in a related multiple malformation syndrome known as Al-Awadi/Raas-Rothschild syndrome. The additional important feature of large parietooccipital skull defects without meningocele, encephalocele, or other brain malformation has thus far been reported only in children with Schinzel phocomelia syndrome. We recently evaluated a boy affected with Schinzel phocomelia born to nonconsanguineous healthy parents of Mexican origin...

Osteosarcoma is the most common primary malignant tumor of bone in adolescents and young adults. It accounts for approximately 15% of all primary bone tumors confirmed at biopsy. There are numerous types of primary osteosarcoma, including intramedullary (high grade, telangiectatic, low grade, small cell, osteosarcomatosis, and gnathic), surface (intracortical, parosteal, periosteal, and high-grade surface), and extraskeletal. Osteosarcoma may also occur as a secondary lesion in association with underlying benign conditions...

This is the first reported case of congenital extraskeletal Ewing's sarcoma (EES) in the head and neck region. The tumor arose from the medial aspect of the right lower eyelid and rapidly increased in size despite surgery and chemotherapy. Accurate histologic diagnosis is emphasized, and the differential diagnosis, pathology, and treatment are discussed.

Based on a review of 752 cases coded as soft tissue sarcomas, histologic features were analyzed for a differential diagnosis, dividing the sarcomas in the first diagnostic step into purely descriptive groups, regardless of biologic behavior: spindle cell, small cell, pleomorphic and myxoid varieties. The tumor categories mainly discussed in order were fibrosarcoma, leiomyosarcoma, malignant schwannoma, synovial sarcoma, malignant fibrous histiocytoma, embryonal and alveolar rhabdomyosarcoma, malignant neuroepithelioma , extraskeletal Ewing's sarcoma, and myxoid and other types of liposarcoma...

This study evaluated the morphogenetic properties of the bone matrix as a biological delivery system in a composite allogeneic bone/alloplastic implant placed in extraskeletal recipient sites in rats. Seventy-two allogeneic diaphyseal bone cylinders were chemosterilized to obtain autolyzed antigen-extracted allogeneic (AAA) bone and implanted in twenty 140- to 160-day-old rats. In 30 implants, porous hydroxyapatite biomatrix (HAB) was inserted into the AAA bone cylinders creating the composite implants. Serial sections from the specimens harvested at 23, 30, 45, 53, and 63 days showed that 84% of the AAA bone cylinders induced the differentiation of variable amounts of endochondral bone and that in 61% bone formed in the interior of the cylinders; 74% of the composite implants had no bone-inductive effect, recruiting instead macrophages and multinucleated giant cells at the HAB/mesenchymal interface...