Benign notochordal cell tumors: A comparative histological study of benign notochordal cell tumors, classic chordomas, and notochordal vestiges of fetal intervertebral discs.

Department of Pathology, Koshigaya Hospital, Dokkyo University School of Medicine, Saitama, Japan.
American Journal of Surgical Pathology (Impact Factor: 4.59). 07/2004; 28(6):756-61. DOI: 10.1097/01.pas.0000126058.18669.5d
Source: PubMed

ABSTRACT Intraosseous benign notochordal cell tumors are recently recognized conditions that may undergo malignant transformation to classic chordomas. This study attempts to define the morphologic and immunohistochemical characteristics of 34 benign notochordal cell tumors by contrasting them with classic chordomas and the notochordal vestiges in fetal intervertebral discs. Benign notochordal cell tumors were characterized by well-demarcated though unencapsulated sheets of adipocyte-like vacuolated and less vacuolated eosinophilic cells within axial bones. The round nuclei were mildly polymorphic but bland. The tumor cells often contained cytoplasmic eosinophilic hyaline globules and lack any intercellular myxoid matrix or necrosis. The involved bone trabeculae were often sclerotic without evidence of bone destruction. The histologic features were different from those of both notochordal vestiges in fetal intervertebral discs and classic chordomas. There was overlap in immunohistochemical reactivity of benign notochordal cell tumors and chordomas, but notochordal vestiges failed to demonstrate cytokeratin 18 positivity. A more appropriate term for the lesions is "benign notochordal cell tumor" rather than "notochordal rest" or "notochordal hamartoma" as they are not rests and do not fulfill the definition of hamartoma. Benign notochordal cell tumors do not need any surgical procedure and must be adequately recognized to prevent unnecessary operations.

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    ABSTRACT: Much discussion about benign notochordal cell tissue in vertebrae has centered on the nature of its relationship, if any, to chordoma. Often referred to as benign notochordal cell tumors (BNCTs), these lesions have unique morphological features, however, differentiating between notochordal cells in discs, BNCT, and chordoma can be difficult. They are described as radiologically distinct from chordoma, with lysis, contrast enhancement, and a soft tissue mass indicating chordoma. All chordomas diagnosed at our institution, the Istituto Ortopedico Rizzoli (Bologna, Italy), prior to 2008 were reviewed, yielding 174 cases. Five were limited to bone; one was a recurrent chordoma without original data available. The remaining four were re-evaluated in detail. There were three women and one man, aged 33-57 years (mean, 48 years). Two were BNCTs and two were mixed lesions containing BNCT and chordoma. On computed tomography, all were radiopaque with areas of lysis. One BNCT was heterogeneous on magnetic resonance imaging, enhancing after contrast. Microscopically, one BNCT had a well-defined cystic area with a sclerotic border. The other had a minute atypical area; it recurred as chordoma. The mixed lesions had areas of definitive BNCT, definitive chordoma, and atypical areas that did not meet the criteria for either. The atypical areas in all three cases 'blended' with areas of chordoma or BNCT. These cases illustrate the ongoing challenges in differentiating between BNCT and chordoma. All had unique imaging features; three had atypical microscopic areas blending with BNCT or chordoma, strengthening the argument for a relationship between the two entities and supporting the idea that some BNCTs may progress to chordoma. Our study dispels the notion that any single radiologic criterion used to distinguish between chordoma and BNCT is reliable, opening the discussion as to whether or how to monitor BNCTs.
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    ABSTRACT: Background A retroclival lesion can represent a notochordal remnant-derived mass. The differential diagnoses includes benign lesions such as ecchordosis physaliphora (EP) and neuroenteric cyst or malignant ones such as chordomas. In the case of EP and chordoma, although both types arise from remnants of fetal notochord tissues, they represent two separate entities with different radiographic and biologic behaviors. Case Description We present a case of an incidental finding of a retroclival lesion. The magnetic resonance imaging (MRI) characteristics of the lesion match the neuroimaging profile of a benign lesion and are suggestive of an EP. There was no enhancement noted with the addition of gadolinium. Nonetheless, pathology determined the lesion to be a malignant chordoma. Conclusion The differential diagnosis of a retroclival lesion includes benign and malignant notochordal lesions. Here we present a case of a patient with an incidental finding of a retroclival lesion. Radiographic findings were suggestive of a benign lesion, possibly EP, yet the pathology revealed a chordoma. This report suggests that despite benign imaging, chordoma cannot be excluded and the implications for treatment can be significant. It is important to achieve the correct diagnosis because the prognostic and therapeutic implications are different.
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