Primary spinal tumors Assessment prognostic factors and management

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Primary spinal tumors Assessment, prognostic factors, and management Presenter’s name Arial 24 pt Meeting

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

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

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

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

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 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

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

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

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 Plasmacytoma or multiple myeloma

Multiple myeloma • Considered a primary tumor of bone, but arises from plasma cells

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

Aneurysmal bone cysts • A benign blood-filled cyst in bone that tends to expand

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

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

Osteoid osteoma • A benign tumor of bone with around 10– 20% occurring in

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

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

Haemangiomas

Haemangiomas

Giant cell tumors

Giant cell tumors

Giant cell tumors • A relatively rare, but locally aggressive bone tumor • Most

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

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

Chordoma • A rare malignant primary tumor of bone • Occurs along the spine,

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 and chondrosarcoma

Chondroma • Chondroma’s are benign tumors of bone that occur most commonly on the

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

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

Ewing’s Sarcoma • • Affects mainly children and adolescents Can occur at any age,

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

Osteosarcoma • The most common primary bone cancer • Generally occurs around the ends

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

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

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

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

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

Weinstein-Boriani-Biagini staging for spinal tumors

Indications for and goals of surgical intervention Benign lesions • Symptomatic relief, to achieve

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

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

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.

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.

Excellence in Spine

Excellence in Spine