Primary Malignant Tumors of the Cervical Spine



Primary Malignant Tumors of the Cervical Spine


Stefano Boriani

Stefano Bandiera

Luca Boriani

James N. Weinstein



A low rate of occurrence, nonspecific symptoms, and sometimes slow evolution (1,2) frequently result in late diagnosis of primary malignant tumors of the cervical spine. Computed tomography (CT) and magnetic resonance imaging (MRI) can allow early detection of lowgrade malignancies (3), but it is uncommon to order these expensive diagnostic studies in the face of neck pain unaccompanied by neurologic deficit. Therefore, these conditions are mostly discovered when pain becomes intractable or when neurologic or anatomically related symptoms (like dysphagia) occur (1). The high-grade malignancies, such as osteosarcoma, Ewing’s sarcoma, and malignant fibrous histiocytoma, are typically detected earlier because of their more rapidly evolving clinical picture: they are expansive and involve more anatomic “compartments” at first detection (4). Excluding metastases and plasmacytomas, most bone tumors of the cervical spine are benign (1,2). Malignant hemoglobinopathies are more common than primary malignant bone tumors in the cervical spine, but the role of surgery in these conditions remains controversial.

Surgical treatment of primary malignant bone tumors of the cervical spine, when indicated, is difficult and frequently requires multiple approaches. The peculiarities of the surgical anatomy of the neck—characterized by close proximity of the spine to vital neurovascular and soft tissue structures—make it difficult to plan oncologically appropriate surgical treatment (5). Only the most recently published literature contains reports of en bloc surgical resection of neoplasms in the cervical spine (6, 7, 8, 9, 10 and 11). Intralesional surgery, combined with modern techniques of radiation therapy, or radiation alone (when high doses can be delivered without undue radiation effects on the spinal cord and other radiosensitive structures), is more frequently an option (12).

Advances in anesthesiology have made it possible to perform these often time-consuming and bloody procedures more safely. One example is performing a dorsal approach to the cervical spine in the sitting position (13). Unlike benign lesions of the cervical spine, malignant processes such as chordomas are seen more often in elderly patients, adding further anesthesiologic risk and associated morbidity.

Often, the rapid evolution or the late discovery of such lesions makes it difficult to identify the site of origin within the vertebra of primary malignant tumors of the cervical spine. These primary malignancies can involve the entire vertebra and the surrounding tissues at the time of discovery. In this chapter, we discuss the role of imaging techniques, oncologic surgical planning, and the expected outcome of different histologic tumor types by using the published literature and our institutional experience.

A word of caution is in order. These tumors in and of themselves provide even the most skilled clinician with a diagnostic and therapeutic challenge. A multidisciplinary approach is needed to provide the best opportunity for surgical success. Institutions without significant experience in the diagnosis, staging, and treatment options in these cases should refer them to a center where such experience exists. As with most tumors, the coordinated effort of an experienced intensive care unit is important to the management of such cases. Finally, it is often difficult to make definitive treatment recommendations because each tumor in every patient is different, and generalities or algorithmic approaches to such cases do not have evidence-based support. Cancer, in and of itself, is difficult for patients and their families to deal with, but “shared decision making” with patients in such cases is essential. All options must be considered and the patient’s values entered into the treatment equation.


POPULATION

From the archives of C.A. Pizzardi and Rizzoli Institutes in Bologna (Italy), 1,109 cases of bone tumors of the spine treated between 1990 and 2008 were selected for review. This resulted in 178 cases of primary malignant, 335 benign lesions, 434 spinal metastases, and 162 myelomas. Out of 513 cases of primary bone tumors of the spine, 115 cases (20%) occurred in the cervical spine; 39 of these were primary malignant in origin (Table 57.1) and 76 were benign. Males were more frequently affected by malignancies than females (25:14); 43.5% of the cases occurred in patients
aged over 50 years; and 23% were in patients over 60 years of age. Fourteen cases arose from C2, four from C3, three from C4, four from C5, three from C6, and ten from C7. It is of some relevance to remark that in the series of 76 benign tumors (48 males and 28 females), 25 (33%) arose in the first two decades and 50% before the age of 30. Only two cases arose over the age of 50, one of which was over 60. Sixty metastases arose in the cervical spine out of 434 cases, and 26 cervical spine myelomas were noted out of 162 cases of patients with spinal involvement.








TABLE 57.1 Histologic Diagnoses of 39 Cases of Primary Malignant Tumors of the Cervical Spine Treated at C.A. Pizzardi and Rizzoli Institute, Bologna, Italy

































DIAGNOSIS


No. of Cases


Chordoma


18


Chondrosarcoma


4


Ewing’s sarcoma


5


Osteosarcoma


5


Malignant fibrous histiocytoma


1


Rhabdomyosarcoma


1


Spindle cell sarcoma


1


Non-Hodgkin lymphoma


4


TOTAL


39


Most chordomas occurred at either C2 or C3. The osteosarcomas, malignant fibrous histiocytomas, and chondrosarcomas were located at the lower cervical segments. At initial presentation, multisegmental involvement was seen in six cases (chordoma, chondrosarcoma, and Ewing’s sarcoma). Although all malignant histologic types, except for chondrosarcomas, originated from the vertebral body, extension to include the whole vertebra was often the case at the time of diagnosis.




STAGING


ONCOLOGIC STAGING

Oncologic and surgical staging is of the utmost importance in order to provide appropriate treatment. The
oncologic staging (5) is based on the histology and the local aggressiveness of the tumor, thereby defining its biologic behavior. The WBB (Weinstein, Boriani, Biagini) surgical staging system (5) describes the extension of the tumor and provides a tool for planning surgery, exchanging information, and evaluating the relationship between treatment and outcome. The Enneking staging system divides benign tumors into three stages and localized primary malignant tumors into four stages (IA-B and IIA-B). This system is based on clinical features, radiographic patterns, CT scan, MRI data, and histologic findings. It was formerly described for long bone tumors and later applied to spinal tumors (5). Histologically low-grade malignant tumors are included in stage I and are further subdivided into IA (the tumor remains inside the vertebra) and IB (the tumor invades perivertebral compartments). No capsule is found, but a thick pseudocapsule of reactive tissue is permeated by small microscopic islands of neoplastic tissue. High-grade malignancies are defined as IIA and IIB. The neoplastic growth is so rapid that the host has no time to form a continuous reactive tissue layer. These rapidly growing tumors are known to seed locally with satellite lesions or to have skip metastases at a distance from the main tumor mass. These malignancies are usually seen on plain radiographs as radiolucent and destructive processes and may be associated with a pathologic fracture. The CT scan and MRI define the transverse and longitudinal extent of tumor and may confirm the absence of a reactive tissue layer.

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Jun 29, 2016 | Posted by in NEUROLOGY | Comments Off on Primary Malignant Tumors of the Cervical Spine

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