Primary spinal tumors Assessment prognostic factors and management
- Slides: 42
Primary spinal tumors Assessment, prognostic factors, and management Presenter’s name Arial 24 pt Meeting Arial 24 pt Presenter’s title Arial 20 pt City, Month, Year Arial 20 pt
Learning outcomes • Recognize the signs and symptoms of primary malignant disease of the spine • Initiate and interpret appropriate radiographic, laboratory tests, biopsy, and input from professional colleagues to determine the patient’s prognosis • Determine the appropriate course of action based on presenting symptoms, radiographic features, pathology, and prognosis • Recognize and manage common complications relating to primary malignant disease of the spine
Outline • Incidence • Presentation • Assessment and investigation • Histopathology • Prognostic factors and staging • Indications for and goals of surgical intervention
Incidence • Primary tumors • Less than 5% of spinal neoplastic disease • Affect 2. 5– 8. 5 per 100, 000 persons per year • Children < 6 years of age most spinal tumors are malignant • Neuroblastoma • Astrocytoma • Sarcoma • Adults over 35 years • Multiple myeloma • Osteosarcoma
Presentation • Most common presenting symptom is pain • Generally localized and often more troublesome at night • May be relieved by simple analgesics • Usually no constitutional upset • Neurological involvement is unusual at presentation • In general, posterior arch lesions tend to be benign • Primary spine tumors affecting the vertebral body are more likely to be malignant
Assessment Clinical examination • Lumps and bumps may be evident
Assessment Deformity may result from a lesion such as an osteoid osteoma
Assessment Deformity may result from a lesion such as an osteoid osteoma
Investigation • Plain radiographs may reveal the lesion • Bone scan useful and will show increased activity in the majority of primary bone tumors • CT and MRI useful to assess the extent of disease and soft tissue extension • Biopsy should be undertaken if diagnosis not evident from imaging • Biopsy tract should be marked and located to enable excision with tumor if malignant lesion suspected
Histopathology • • • Plasmacytoma (multiple myeloma) Aneurysmal bone cysts Osteoid osteoma/osteoblastoma Hemangiomas Giant Cell Tumors Chordoma Chondroma/chondrosarcoma Ewing’s Sarcoma Osteosarcoma
Multiple myeloma Plasmacytoma or multiple myeloma
Multiple myeloma • Considered a primary tumor of bone, but arises from plasma cells in the bone marrow • Plasma cells produce monoclonal paraproteins that are evident in blood and urine • May present with anemia, bleeding, skin lesions, infection, renal failure and bone pain or pathological fractures • Generally produce lytic lesions in bone • Usually treated by chemotherapy and/or radiation unless instability, pathological fracture or cord compression • 5 -year survival around 35%
Aneurysmal bone cysts
Aneurysmal bone cysts • A benign blood-filled cyst in bone that tends to expand • Occurs in the vertebral column as well as the extremities • Usually present in patients under the age of 20 years • The second most common bone tumor in children • Locally aggressive and may lead to pathological fracture or compress adjacent structures • Etiology is uncertain
Aneurysmal bone cysts • Treatment is intralesional curettage and grafting • About 20% recur after treatment, but recurrence in the spine is rare • Associated with a chondroblastoma or giant cell tumor in 20– 30% of cases
Osteoid osteoma
Osteoid osteoma • A benign tumor of bone with around 10– 20% occurring in the spine • Usually presents between the age of 10 and 30 years • Presentation is usually with pain, often worse at night and relieved by nonsteroidal antiinflammatories • Men are twice as likely to be affected than women • Usually resolve on their own, but takes years and patients often request treatment to relieve symptoms • Treatment is excision/curettage or radiofrequency ablation
Osteoblastoma • A rare, benign bone tumor • Usually develop in the lower vertebra of the spine or in long bones of the leg • Aggressive forms may mimic an osteosarcoma • May occur in conjunction with an aneurysmal bone cyst • Closely related to an osteoid osteoma histologically • Lesions <1. 5 cm are generally considered an osteoid osteoma, >1. 5 cm an osteoblastoma • Surgical excision is recommended for this tumor
Haemangiomas
Haemangiomas
Giant cell tumors
Giant cell tumors • A relatively rare, but locally aggressive bone tumor • Most commonly seen around the knee but may occur in the spine • Clinical behavior is not predictable based on clinical, radiographic, or histologic features • Usually presents in adults 20– 40 years of age • Histology characterized by presence of multiple multinucleated giant cells • Potential to recur locally after excision • Distant metastases develop in 2– 3% of cases • Rarely undergoes true malignant transformation
Giant cell tumors • Radiation treatment may be effective, but results in malignant transformation in up 15% of cases, therefore this treatment is generally reserved for recurrent and inoperable cases • Simple curettage and grafting leads to recurrence rates of up to 45% • Complete excision is the preferred treatment
Chordoma
Chordoma • A rare malignant primary tumor of bone • Occurs along the spine, most often in the sacrum or base of the skull • Occurs at any age, but generally 40– 60 years • Although slow growing are difficult to treat and may be life threatening • Develop from small remnants of the notochord, that embryologically develop into the spinal cord • The aim of treatment is complete excision, but this may be difficult due to the location in many cases • May undergo malignant change
Chondroma and chondrosarcoma
Chondroma • Chondroma’s are benign tumors of bone that occur most commonly on the peripheral skeleton on tubular bones • Rarely affect the bones of the trunk but may occur on vertebra where chondrosarcomas are common • Chondroma’s develop from nests of growth plate cartilage • Usually diagnosed between the age of 10 and 30 years • Treatment may be just observation or excision if they result in symptoms
Chondrosarcoma • Typically develop in the pelvis, legs or shoulders of adults over the age of 40 years • Increased risk in patients with • • • Ollier’s disease Maffucci syndrome Multiple Hereditary Exostoses Wilms’ Tumor Paget’s disease Diseases of children leading to chemo or radiation therapy • Complete surgical excision with a wide margin is the preferred treatment • Chemo or radiation therapy is generally limited to those with recurrent disease
Ewing’s Sarcoma
Ewing’s Sarcoma • • Affects mainly children and adolescents Can occur at any age, rarely in adults over 30 years May present with a fever of unknown origin If diagnosed early, before it metastasizes, can be treated successfully in 50% to 75% of cases • Thought to originate in a certain kind of primitive bone cell, but can also occur in extra-osseous sites • Treatment consists of chemotherapy to shrink the tumor then surgery and/or radiation
Osteosarcoma
Osteosarcoma • The most common primary bone cancer • Generally occurs around the ends of long bones, particularly around the knee but can occur anywhere • Usually diagnosed under the age of 25 years • More common in men than in women • Treatment generally consists of a course of chemotherapy over several months, then surgery • Surgical goal is complete excision with clear margins • Survival rates are around 70% for lesions that localized and can be completely excised
Prognostic factors and staging • The single most important variable is the histological type and activity • The presence of metastatic disease at presentation • The location of the tumor, and ability to perform total excision with clear margins • Staging • Localized versus disseminated or metastatic • Contained (intracapsular or extracapsular extension)
Staging Enneking classification of musculoskeletal tumors • Grade: Histology with aid of radiographic findings and clinical correlation • G 0 – Benign • G 1 – Low grade – uniform cell type, few mitoses • G 2 – High grade – atypical nuclei, many mitoses • Site: Compartmental extension • T 1 – Intra compartmental – within limits of periosteum • T 2 – Extra compartmental – breach of compartment • Metastasis: • M 0 – No metastasis • M 1 – Skip lesions or distant metastasis
Staging Enneking classification of musculoskeletal tumors Benign lesions: 1 Latent 2 Active 3 Aggressive G 0 T 0 G 0 T 1 -2 M 0 M 0 -1
Staging Enneking classification of musculoskeletal tumors Malignant lesions: Ia Low grade, intra compartmental G 1 M 0 Ib Low grade, extra compartmental G 1 M 0 IIa High grade, intra compartmental G 2 M 0 IIb High grade, extra compartmental G 2 M 0 IIIa Low or high grade intra compartmental with metastasis G 1 -2 T 1 M 1 IIIb Low or high grade extra compartmental with metastasis G 1 -2 T 2 M 1 T 2 T 1 T 2
Weinstein-Boriani-Biagini staging for spinal tumors
Indications for and goals of surgical intervention Benign lesions • Symptomatic relief, to achieve stability or decompress neural elements • Excision, curettage and grafting is usually effective • Lesions may respond to intralesional excision radiofrequency ablation or injection
Indications for and goals of surgical intervention Malignant lesions • The primary goal is cure or long-term survival • If not cure: • Local control of disease • Achieve or maintain stability • Decompress and protect neural structures • Restoration of function • The preferred form and sequence of treatment varies • Surgical excision, preferably “en bloc” with adjuvant chemotherapy, often prior to surgery • Radiation therapy may be indicated in certain cases
Take-home messages • The most common presenting feature is pain • Need to obtain a tissue diagnosis before planning intervention • CT-guided biopsy is a safe and effective way to obtain a tissue diagnosis, but ensure biopsy tract can be resected if a primary malignant tumor is suspected • Histology and the extent of disease are the most important determinants of prognosis
References 1. Fuchs B, Boos N (2008) Primary tumors of the Spine. Spinal Disorders. Aebi M, Boos N (eds). Springer Verlag. 951– 974. 2. Enneking WF, Spanier SS, Goodman MA (1980) A system for the surgical staging of musculoskeletal sarcoma. Clin Orthop Relat Res; 106– 120. 3. Chan P, Boriani S, Fourney DR, et al (2009) An assessment of the reliability of the Enneking and Weisstein-Boriani-Biagini classifications for staging of primary spinal tumors by the Spinal Oncology Study Group. Spine; 384– 391. 4. Hay MC, Paterson D, Taylor TK (1978) Aneurysmal bone cysts of the spine. J Bone Joint Surg Br; 60 -B(3); 406– 411. 5. Kurugoglu S, Adaletli I, Mihmanli I, et al (2008) Lumbosacral osseous tumors in children. Eur J Radiol; 65(2): 257– 269. 6. Zileli M, Cagli S, Basdemir G, et al (2003) Osteoid osteomas and osteoblastomas of the spine. Neurosurg Focus; 15(5): E 5. 7. Sundaresan N, Boriani S, Rothman R (2004) Tumours of the Spine. J. Neurooncology 69: 273– 290.
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