Bone and Cartilage Tumors PDF

Bone, Cartilage, Soft Tissue, Vascular tumors:
Benign and Malignant Lesions DR. TO SI N ALADE Classif ication of Bone and Cartilage Tumors Histologic type Benign Malignant Osteogenic/bone forming Osteoma Osteosarcoma O...

Bone, Cartilage, Soft Tissue, Vascular tumors:
Benign and Malignant Lesions DR. TO SI N ALADE Classif ication of Bone and Cartilage Tumors Histologic type Benign Malignant Osteogenic/bone forming Osteoma Osteosarcoma Osteoid osteoma (primary/secondary) Osteoblastoma Chondrogenic/cartilaginous Osteochondroma Chondrosarcoma Chondroma Dedifferentiated Chondroblastoma chondrosarcoma Hematopoietic Myeloma Malignant lymphoma Unknown origin Giant-cell tumor Unicameral cyst Aneurysmal bone cyst Benign tumors, Bone OSTEOMA: involves the skull and facial bones, with extremely slow growth rate (HYPEROSTOSIS FRONTALIS) it may extends into the orbit or sinuses(Gardner´s syndrome) Peak incidence: 40-50 years of age OSTEOID OSTEOMA: benign, painful growth of the diaphysis of a long bone (often the tibia or femur) - Age : 5-25 years, mostly males Symptoms: Pain is worse at night and is relieved with aspirin -X rays: central radiolucency surrounded by a sclerotic rim. -Micro: small (< 2 cm) lesion of the cortex with central nidus of osteoid surrounded by dense sclerotic rim of reactive cortical bone. OSTEOBLASTOMA: Similar to an osteoid osteoma but larger than 2 cm in size and often involving vertebrae. Benign tumors: Cartilage A OSTEOCHONDROMA (exostosis) -Benign bone metaphyseal growths capped with cartilage that originates from epiphyseal growth plate. -It may affects adolescent males as a firm, solitary growth at the ends of long bones. -It may be asymptomatic or cause pain, producing deformity, and can undergo with malignant transformation ( rarely) OSTEOCHONDROMATOSIS ( Multiple hereditary exostosis) -Characterized with multiple, often symmetric, osteochondromas. ENCHONDROMA: benign cartilaginous growth within the medullary cavity of bone, usually involving the hands and feet. -Is a typical solitary lesion often asymptomatic and require no treatment. MULTIPLE ENCHONDROMAS (Enchondromatosis) OLLIER DISEASE: a non hereditary syndrome, with multiple enchondromas in hands and feet. It may presents with pain It may undergo malignant transformation to chondrosarcoma. MAFUCCI SYNDROME Multiple enchondromas Soft tissue hemangiomas Increased risk of malignant transformation, ovarian Ca. and brain gliomas. Malignant Tumors of Bone. OSTEOSARCOMA ( Osteogenic sarcoma) - Osteosarcoma is a highly malignant bone tumor characterized by formation of bone matrix or osteoid (unmineralized bone) by malignant tumor cells. - Most common primary malignant tumor of bone -Males> females 2:1 - Age group - Osteosarcoma has a bimodal age distribution. Most occur in ( ages 10-25) then in elderly after 40 years. -Patients with familial retinoblastoma have a high risk -Clinical features: localized pain and swelling Pathogenesis:
Based on the pathogenesis, OS is divided into primary and secondary Primary Osteosarcomas In this type, the underlying bone is unremarkable. About 70% of osteosarcomas have acquired genetic abnormalities such as ploidy changes and chromosomal aberrations. - Mutations in tumor suppressor gene: a. RB, the retinoblastoma gene (a cell cycle regulator) mutations is associated with 1000 -fold increased risk of osteosarcoma and its mutation is found in about two-thirds of patients. b. Patients with Li-Fraumeni syndrome (germline p53 mutations) have a greater incidence of osteosarcoma c. Abnormalities in INK4a, which codes p16 (a cell cycle regulator) and p14 have also been found in osteosarcoma. Secondary Osteosarcoma Osteosarcomas in older persons almost always develop in association with pre-existing bone disorders. These include: A, Paget disease of bone. B, Radiation exposure: C, Chemotherapy: Children treated with alkylating agents D, Pre-existing benign bone lesions: e.g. fibrous dysplasia and osteomyelitis. MORPHOLOGY The tumors usually arise in the metaphyseal region of the long bones of the extremities, and almost 50% occur about the knee. Several subtypes of osteosarcoma are recognized and are grouped according to Site of origin (intramedullary, intracortical, or surface) Degree of differentiation Multicentricity (synchronous, metachronous) Primary (underlying bone is unremarkable) or secondary to preexisting disorders such as benign tumors, Paget disease, bone infarcts, previous irradiation Histologic features (osteoblastic, chondroblastic, fibroblastic, telangiectatic, small cell, and giant cell). The most common subtype arises in the metaphysis of long bones and is primary, solitary, intramedullary, and poorly differentiated. Grossly- osteosarcomas are big bulky tumors that are gritty, gray-white, and often contain areas of hemorrhage and cystic degeneration. Microscopy Malignant tumor cells: – Tumor cells vary in size and shape – Nucleus: Usually show large hyperchromatic nuclei and often shows mitotic figures – Bizarre tumor giant cells are common. Matrix component: Production of osteoid (unmineralized/noncalcified) or bone (calcified osteoid) by malignant tumor cells is the single diagnostic feature of conventional OS. ◆ Osteoid: It appears as a dense, uniform, eosinophilic glassy intercellular material. ◆ Neoplastic bone: It usually has a coarse, lace-like architecture. Histological subtype: Most tumors contain a mixture of cells with varying amounts matrices mentioned below. Depending on the one predominant matrix, conventional OS is divided into: – Osteoblastic: Large amount of osteoid and bony trabeculae. – Chondroblastic: Abundant malignant cartilage. – Fibroblastic/fibrohistiocytic: They appear similar to malignant fibrous histiocytoma. – Telangiectatic: Large cavernous dilated vascular channels and it is more aggressive. – Small cell: The tumor cells are small in size, uniform and simulate the appearance of Ewing’s sarcoma and malignant lymphoma. – Giant cell: This subtype contains numerous giant cells. Classic X ray findings: -Codman’s triangle ( periosteal elevation) -Sunburst pattern -Bone destruction Tx: surgery/ chemotherapy Prognosis: poor (hematogenous metastasis to the lungs is a common complication) CHONDROSARCOMA
Malignant tumor of cartilage Chondrosarcoma is subclassified according to site as central (intramedullary) and peripheral (juxtacortical and surface) -Males > females age 30-60 -Tumor may arise primarily or secondary from a preexisting enchondroma, exostosis or Paget’s disease. -Clinical presentation: progressively enlarging mass with pain and swelling, that typically involves the pelvic bones, spine, and shoulder girdle. -Micro: composed of atypical chondrocytes and chondroblasts, often with multiple nuclei in a lacunar structure Histologically, they include conventional (hyaline and/or myxoid), clear cell, dedifferentiated, and mesenchymal variants. Conventional central tumors constitute about 90% of chondrosarcomas GIANT CELL BONE TUMOR
(Osteoclastoma) Uncommon malignant neoplasm containing multinucleated giant cells admixed with stromal cells. Females>males, with ages between 20-50 years Cell of origin: Primitive stromal cells. Clinical features: bulky mass with pain. X rays: expanding lytic lesion surrounded by a thin rim of bone. It may have also a soap bubble appearance Gross: often involves the epiphyses of long bones, usually around the knee ( distal femur and proximal tibia) seen a red brown mass with cystic degeneration. Site: Giant-cell tumors in adults involve both the epiphyses and the metaphyses of a long bone, but in adolescents, they are limited to the metaphysis. GIANT CELL TUMOR(cont.)
Bones involved: – Knee area: Distal femur and proximal tibia. – Lower end of radius, humerus and fibula. Micro: multiple osteoclast-like giant cells that are distributed within a background of mononuclear stromal cells. Tx: surgery/ curettage or block resection Prognosis: locally aggressive with a high rate of recurrence. EWING SARCOMA Malignant neoplasm of undifferentiated cells arising within the marrow cavity Males are affected slightly more often than females, most occur in teenagers ( 5-20) It arises from the long bones like femur, tibia, humerus and fibula. Can be found in the pelvic bone ribs and vertebrae. Clinical features: pain , swelling and tenderness Classic translocation t11;22 which produces the EWS- FL11 fusion protein X-ray: concentric onion skin layering of new periosteal bone. Gross: often affects the diaphysis of long bones with most common sites like femur, pelvis and tibia seen a white tan mass with necrosis and hemorrhage. Microscopy: sheets of undifferentiated small round blue cells resembling lymphocytes. Characteristic Homer- Wright pseudorosettes Frequently the tumoral cells erode cortex and periosteum and invade surrounding tissues. Treatment.: chemotherapy, surgery and/ or radiation Prognosis: 5 year survival rate of 75% ANEURYSMAL BONE CYST  Benign tumor of bone characterized by multiloculated blood-filled cystic spaces that may present as a rapidly growing expansile tumor  First 2 decades of life and has no sex predilection  Metaphyses of long bones and the posterior elements of vertebral bodies  Most common signs and symptoms are pain and swelling  Histology: Aneurysmal bone cyst with blood-filled cystic space surrounded by wall containing proliferating fibroblasts, reactive woven bone, and osteoclast-type giant cells Metastatic Tumors to Bone  Pathways of spread – Direct extension – Lymphatic or hematogenous dissemination  – intraspinal seeding (via the Batson plexus of veins)  In adults more than 75% of metastases originate from cancers of the prostate, breast, kidney, and lung.  In children, metastases to bone originate from neuroblastoma, Wilms tumor, and rhabdomyosarcoma SOFT TISSUE TUMORS Soft-tissue tumors constitute a large and heterogeneous group of neoplasms. Traditionally, tumors have been classified according to histogenetic features. (Fibrosarcoma, for example, is categorized as a tumor arising from fibroblasts.) However, histomorphologic, immunohistochemical, and experimental data suggest that most, if not all, sarcomas arise from primitive, multipotential mesenchymal cells, which in the course of neoplastic transformation differentiate along one or more lines Etiology Various cytogenetic abnormalities. Benign schwannoma , Monosomy 22; Lipoblastoma, Rearrangement of 8q; Uterine leiomyoma, t(12;14)(q15;q24) Radiation, Environmental carcinogens, Infection, Trauma, Chronic lymphoedema TUMORS OF ADIPOSE TISSUE Lipomas Liposarcoma TUMORS AND TUMOR-LIKE LESIONS OF FIBROUS TISSUE Nodular fasciitis Fibromatoses Superficial fibromatoses Deep fibromatoses Fibrosarcoma TUMORS OF SKELETAL MUSCLE Rhabdomyoma Rhabdomyosarcoma TUMORS OF SMOOTH MUSCLE Leiomyoma Leiomyosarcoma PERIPHERAL NERVE TUMORS Neurofibroma Schwannoma Granular cell tumor Malignant peripheral nerve sheath tumors FIBROHISTIOCYTIC TUMORS Fibrous histiocytoma Dermatofibrosarcoma protuberans Malignant fibrous histiocytoma TUMORS AND TUMOR-LIKE LESIONS OF FIBROUS TISSUE Nodular fasciitis Fibromatoses Superficial fibromatoses Deep fibromatoses Fibrosarcoma TUMORS OF UNCERTAIN HISTOGENESIS Synovial sarcoma Alveolar soft-part sarcoma Epithelioid sarcoma Tumors of Adipose Tissue


LIPOMAS Benign tumors of fat, known as lipomas, are the most common soft-tissue tumor of adulthood. They are subclassified according to particular morphologic features as conventional lipoma, fibrolipoma, angiolipoma, spindle cell lipoma, myelolipoma, and pleomorphic lipoma Some of the variants have characteristic chromosomal abnormalities; for example, conventional lipomas often show rearrangements of 12q14–q15, 6p, and 13q, Morphology: The conventional lipoma, the most common subtype, is a well- encapsulated mass of mature adipocytes that varies considerably in size. It arises in the subcutis of the proximal extremities and trunk, most frequently during middle adulthood Lipomas are soft, mobile, slipping sign and painless (except angiolipoma) and are usually cured by simple excision Tumors of Skeletal Muscle  Skeletal muscle neoplasms, in contrast to other groups of tumors, are almost all malignant. The benign variant, rhabdomyoma, is distinctly rare.  RHABDOMYOSARCOMA: Rhabdomyosarcoma, the most common soft-tissue sarcoma of childhood and adolescence, usually appears before age 20 It may arise in any anatomic location, but most occur in the head and neck or genitourinary tract, where there is little if any skeletal muscle as a normal constituent. Only in the extremities do they appear in relation to skeletal muscle. Morphology  Rhabdomyosarcoma is histologically subclassified into embryonal, alveolar, and pleomorphic variants  The rhabdomyoblast—the diagnostic cell in all types—contains eccentric eosinophilic granular cytoplasm rich in thick and thin filaments  Rhabdomyoblasts may be round or elongate; the latter are known as tadpole or strap cells, and may contain cross-striations visible by light microscopy Tumors of Smooth Muscle


LEIOMYOMAS  Leiomyomas, the benign smooth muscle tumors, often arise in the uterus; in fact, uterine leiomyomas are the most common neoplasm in women  They develop in 77% of women and, depending on their number, size, and location, may cause a variety of symptoms including infertility  Leiomyomas may also arise from the errector pili muscles found in the skin, nipples, scrotum, and labia and less frequently develop in the deep soft tissues and the wall of the gut.  Morphology: Leiomyomas are usually not larger than 1 to 2 cm in greatest dimension and are composed of fascicles of spindle cells that tend to intersect each other at right angles. The tumor cells have blunt-ended, elongated nuclei and show minimal atypia and few mitotic figures LEIOMYOSARCOMA Leiomyosarcomas account for 10% to 20% of soft-tissue sarcomas  They occur in adults and afflict women more frequently than men.  Most develop in the skin and deep soft tissues of the extremities and retroperitoneum.  Morphology: Retroperitoneal tumors may be large and bulky and cause abdominal symptoms. Histologically they consist of malignant spindle cells with cigar-shaped nuclei arranged in interweaving fascicles VASCULAR TUMORS Hemangiomas are the most common tumors of the head and neck in infancy and childhood, comprising approximately 7% of all benign soft tissue tumors The hemangioma is a true vascular tumor that results from a overgrowth of normal vascular tissue The majority of the hemangiomas in infants are noted by the parent within the first month of life. Clinical Presentation Hemangiomas are found in the superficial tissue, the deep tissue, or both and may affect organ systems such as the liver, lung, spleen, and gastrointestinal tract The predilection for females is approximately a 3 :1 ratio Lymphangiomas Lymphangiomas are malformations of the lymphatic system, which is the network of vessels responsible for returning to the venous system excess fluid from tissues. These malformations can occur at any age and may involve any part of the body, but 90% occur in children less than 2 years of age and involve the head and neck. -Capillary lymphangiomas Capillary lymphangiomas are composed of small, capillary-sized lymphatic vessels and are characteristically located in the epidermis. -Cavernous lymphangiomas Composed of dilated lymphatic channels, cavernous lymphangiomas characteristically invade surrounding tissues. -Cystic hygromas are large, macrocystic lymphangiomas filled with straw-colored, protein-rich fluid. -Lymphangiomas may also be classified into microcystic, macrocystic, and mixed subtypes, according to the size of their cyst Glomus tumor A glomus tumor (also known as a "solitary glomus tumor,“ solid glomus tumor or glomangioma) is a rare benign neoplasm arising from the glomus body and mainly found under the nail, on the fingertip or in the foot. Histologically, glomus tumors are made up of an afferent arteriole, anastomotic vessel, and collecting venule. These lesions should not be confused with paragangliomas, which were formerly also called glomus tumors in now-antiquated clinical usage. Vascular Malformations Vascular malformations are present at birth and unlike hemangiomas, do not go through a a“ rapid proliferative phase” and they do not “involute ”. They grow commensurately with the patient. Vascular malformations may be capillary, venous, arterial, or combinations of these. Approximately 31% of these malformations are found in the head and neck region Bacillary angiomatosis Is a form of angiomatosis associated with bacteria of the Bartonella genus BA is characterised by the proliferation of blood vessels, resulting in them forming tumour-like masses in the skin and other organs Kaposi Sarcoma Kaposi's sarcoma is a tumor caused by human herpesvirus 8 (HHV8, also known as Kaposi's sarcoma-associated herpesvirus, KSHV). It became more widely known as one of the AIDS defining illnesses in the 1980. The viral cause for this cancer was discovered in 1994. Microscopy - Abundant small branching blood vessels. Spindle cells. Intracellular hyaline globs extravasated RBC Angiosarcoma Angiosarcoma is a malignant neoplasm of endothelial-type cells that line vessel walls. This may be in reference to blood (hemangiosarcoma) or lymphatic vessels (lymphangiosarcoma) Hemangiopericytoma A hemangeopericytoma (HPC) is a type of soft tissue sarcoma that originates in the pericytes in the walls of capillaries. THANK YOU KINDLY READ CAVERNOUS HEMANGIOMA