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BONE and SOFT TISSUE TUMORS SOFT TISSUE TUMORS Disorder Inheritance Locus Gene Tumor

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BONE and SOFT TISSUE TUMORS SOFT TISSUE TUMORS Disorder Inheritance Locus Gene Tumor CLASSIFICATION BONE AND SOFT TISSUE z Purpose of classification is to link similar tumors in order TUMORS to understand their behavior, determine the most appropriate treatment, and investigate their biology. z However, purpose of a classification system is simplicity and reproducibility z Therefore tumors are classified according to the cell type they resemble. z Refinements are coming from cytogenetics, molecular, and gene expression studies. z The majority arise from -or show differentiation toward- mesenchymal cells, but some show other differentiation (neuroectodermal, histiocytic). z A small subset is of unknown histogenesis. Fabrizio Remotti MD DEFINITION z Soft tissue pathology deals CLASSIFICATION with tumors of the connective tissues. z Many tumors resemble z The concept of soft tissue is tissues present in the understood broadly to region of origin. include non-osseous tumors z These tumors may be derived from stem cells of extremities, trunk wall, Vascular leiomyosarcoma retroperitoneum and that belong to local, mediastinum, and head & organ-specific pools. neck. z Other involved stem cells may be bone marrow z Excluded (with a few derived. exceptions) are organ specific tumors. Lipoma Alveolar soft part sarcoma DEFINITION CLASSIFICATION z Bone pathology deals with tumors of the z Some tumors have no skeletal system. resemblance to normal tissue in the region z Included are subsets of (metaplastic foci within a tumors from extra- ttftumor, or tumors of osseous sites that show different histogenesis from osseous and cartilaginous the normal cells of the region) differentiation. z Some sarcomas have no normal cell counterparts, probably reflecting an unique genetic makeup. Epithelioid sarcoma, proximal type 1 HISTOGENESIS EPIDEMIOLOGY All tumors are derived from stem cells that are programmed to differentiate into various mature cell types. CANCER CELL : SEPTEMBER 2002 · VOL. 2, 175- 178 EPIDEMIOLOGY EPIDEMIOLOGY z Soft tissue (ST) sarcomas are z There is a slight male rare tumors compared to other predominance (with some malignancies: 8,700 new subtypes more common in sarcomas in 2001, with 4,400 women). deaths. z The majority of soft tissue z The incidence of ST sarcomas tumors affect older adults in the USA is approximately (some sub-groups occur 3.3 cases per 100,000 people. predominantly or exclusively Extra-renal malignant rhabdoid tumor z This is roughly 5% of each of in children). some of the most common z Incidence of benign soft tissue carcinomas (prostate, breast tumors not known, but and lung), half of all brain probably outnumber tumors, and approximately malignant tumors 100:1. equal to AML. SOFT TISSUE TUMORS EPIDEMIOLOGY z The knowledge of epidemiologic data may help in diagnosis. z What you don’t know may hurt you! Nowhere in the picture….. 2 BONE TUMORSTUMORS-- BONE TUMORSTUMORS-- EPIDEMIOLOGY EPIDEMIOLOGY • The majority of tumors involving bone are z Primary bone tumors are rare. secondary (or z Bone sarcomas account for 0.2% of all metastatic): neoplasms (SEER Cancer Statistics Review , - secondary 1973-1996). (metastases) (95%) z Soft tissue sarcomas are approximately 10 - primary (5%) times more common than primary bone sarcomas. BREAST CANCER TO HIP Secondary Tumors of Bone BONE TUMORSTUMORS-- EPIDEMIOLOGY •The carcinomas most frequently involved with bone metastasis originate from: • Lung • Breast • Prostate • G.I • Kidney • Thyroid MELANOMA TO PROXIMAL HUMERUS BONE TUMORS EPIDEMIOLOGY •Bone sarcomas as a group have a bimodal distribution. •The first peak is in the second decade. Bone and soft tissue sarcomas •The second peak occurs in patients older than sixty. 30 80 25 70 /100,000 s 20 60 OS Series2 15 50 CS Series1 persons) 40 ES 10 30 CH 5 20 MFH Incidence(case rate 0 10 1 210 3 420 5 630 7 8 40 9 10 50 11 12 60 13 14 70 15 16 80 17 18 0 Age at diagnosis Soft tissue sarcomas 0 to 4 Bone sarcomas 10 to14 20 to24 30 to34 40 to44 50 to54 60 to64 70 to74 80 to85 3 ETIOLOGY ETIOLOGY z The etiology of sarcomas is poorly understood, and what is known apply only z Herbicides (“agent orange”) and to a small fraction of the ggproup. ppperipheral soft tissue sarcomas z Retained metal objects z The known etiologic agents are ionizing (shrapnel, surgical devices) and radiation, oncogenic viruses, and chemicals. OS, AS and MFH z Vinyl chloride, inorganic z These agents are able to cause genetic arsenic, Thorotrast, anabolic alterations that can lead to tumorigenesis. steroids linked to AS and MFH. ETIOLOGY ETIOLOGY z Host factors may also z Radiation induced sarcomas play a role in the develop in 1% of patients who development of soft have undergone therapeutic tissue sarcomas. irradiation. – Immunosuppression, besides Kaposi’s z The interval between irradiation sarcoma, may be and diagnosis of sarcoma varies associated with between 5 and 10 years. sarcomas. – Lymphedema, z The majority of radiation- congenital or acquired induced sarcomas are high grade (post-mastectomy) is a and poorly differentiated (MFH, rare cause of FS, OS, and AS). extremity-based AS. AS in lymphedema ETIOLOGY ETIOLOGY z Oncogenic viruses introduce new genomic material in the cell, which encode for oncogenic proteins that disrupt the regulation of cellular proliferation. z Two DNA viruses have been linked to soft tissue sarcomas: – Human herpes virus 8 (HHV8) linked to Kaposi’s sarcoma – Epstein-Barr virus (EBV) linked to subtypes of leiomyosarcoma z In both instances the connection between viral infection and sarcoma is more common in immunosuppressed hosts. 4 CONGENITAL SYNDROMES ASSOCIATED WITH BONE AND SOFT TISSUE TUMORS SOFT TISSUE TUMORS Disorder Inheritance Locus Gene Tumor Albright hereditary osteodystrophy AD 20q13 GNAS1 Soft tissue calcifications and osteomas Bannayan -Riley- Ruvalcaba AD 10q23 PTEN Lipomas, hemangiomas syndrome Beckwith- Wiedemann syndrome Sp/AD 11p15 Complex Embryonal RMS, myxomas, fibromas, hamartomas Bloom syndrome AR 15q26 BLM Osteosarcoma Carney complex AD 17q23-24 PRKAR1AK Myxomas and pigmented schwannomas (Familial myxoma syndrome) 2p16 Familial chordoma AD 7q33 - Chordomas Costello syndrome Sporadic - - Rhabdomyosarcomas Fibroma of tendon sheath, finger Low-grade fibromyxoid sarcoma, neck 19F Cowden disease AD 10q23 PTEN Lipomas, Hemangiomas (Multiple hamartoma syndrome) Diaphyseal medullary stenosis AD 9p21-22 - MFH Familial adenomatous polyposis AD 5q21 APC Craniofacial osteomas, desmoid tumors Familial expansile osteolysis AD 18q21 TNFRSF11A Osteosarcomas Familial infiltrative fibromatosis AD 5q21 APC Desmoid tumors Langer- Giedion syndrome Sporadic 8q24 EXT1 Osteochondromas, chondrosarcomas Li-Fraumeni syndrome AD 17p13 TP53 Osteosarcomas, RMS, other sarcomas 22q11 CHEK2 Familial multiple lipomas AD - - Lipomas IM myxoma, thigh 65M Myxoid MFH, thigh, 60M Symmetrical lipomatosis Sporadic - - Lipomas, lipomatosis of head and neck CONGENITAL SYNDROMES ASSOCIATED WITH BONE AND SOFT TISSUE TUMORS SOFT TISSUE TUMORS Disorder Inheritance Locus Gene Tumor Maffucci syndrome Sporadic - - Enchondromas, CS, hemangiomas, AS Mazabraud syndrome Sporadic 20q13 GNAS1 Fibrous dysplasia, OS, IM myxomas McCune –Albright syndrome Sporadic 20q13 GNAS1 Fibrous dysplasia, osteosarcomas Multiple osteochondromas, AD 8q24 EXT1 Osteochondromas, chondrosarcomas non- syndromic 11p11-12 EXT2 Myofibromatosis AR - - Myofibromas Neurofibromatosis type 1 AD 17q11 NF1 Neurofibromas, MPNST Neurofibromatosis type 2 AD 22q12 NF2 Schwannomas Rhabd omyoma, hear t, new born F Ollier disease Sporadic 3p21-22 PTHR1 Enchondromas, chondrosarcomas Embryonal RMS, thigh, 5M Paget disease of bone, familial AD 18q21 Osteosarcomas 5q31 5q35 Proteus syndrome Sporadic - - Lipomas Retinoblastoma AD 13q14 RB1 Osteosarcomas, soft tissue sarcomas Rhabdoid predisposition syndrome AD 22q11 SMARCB1 Malignant rhabdoid tumors Rothmund- Thompson syndrome AR 8q24 RECQL4 Osteosarcomas Rubinstein- Taybi syndrome AD 16p13 CREBBP Rhabdomyosarcomas Venous malf. With glomus cells AD 1p21-22 - Glomus tumors Scwannoma, leg, 35F MPNST, arm, 45M with NF Werner syndrome AR 8p11-12 WRN Bone and soft tissue sarcomas Cartilage tumors Osteochondroma SOFT TISSUE TUMORS WHO CLASSIFICATION OF Chondroma Enchondroma BONE TUMORS Periosteal chondroma CLASSIFICATION Mult. chondromatosis Chondroblastoma Chondromyxoid fibroma Chondrosarcoma Central MAJOR TYPES OF SOFT TISSUE TUMORS Peripheral Cell type Benign tumor Malignant tumor Dedifferentiated Mesenchymal (Myo)fibroblast Fibroma, myxoma Fibrosarcoma, MFH Clear cell Adipocyte Lipoma Liposarcoma Osteogenic tumors Osteoid osteoma Smooth muscle cell Leiomyoma Leiomyosarcoma OtOsteo blas toma Osteosarcoma Conventional Skeletal muscle cell Rhabdomyoma Rhabdomyosarcoma Telangiectatic Endothelial cell Hemangioma Angiosarcoma Small cell Low grade central Schwann cell Schwannoma, neurofibroma MPNST Secondary Cartilage cell Chondroma Chondrosarcoma Parosteal Periosteal Interstitial cell GIST GIST High grade surface Histiocyte JXG, GCTTS, RDD True histiocytic sarcoma Fibrogenic tumors Desmoplastic fibroma Unknown No benign counterparts ES, SS, ES, ASPS Fibrosarcoma Osteosarcoma Fibrohistiocytic tumors Desmoplastic fibroma Fibrosarcoma 5 Ewing/PNET Ewing sarcoma WHO CLASSIFICATION OF BONE Hematopoietic tumors Plasma cell myeloma OSTEOSARCOMA Malignant lymphoma TUMORS z Largely a disease of Giant cell tumor Giant cell tumor the young (60% <25 Malignant giant cell tumor years) Notochordal tumors Chordoma z 30 % >40 years. Vascular tumors Hemangioma z In older people rule Angiosarcoma out predisposing Smooth muscle tumors Leiomyoma
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