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PubMed1994

Immunohistochemical and electron microscopic studies on intracranial chordomas: difference between typical chordomas and chondroid chordomas.

Immunohistochemical and electron microscopic studies were carried out on 9 cases of intracranial chordomas. Three of them were typical chordomas and 6 were chondroid chordomas. Immunohistochemically, both typical chordomas and chondroid chordomas were positively stained for cytokeratin and epithelial membrane antigen. Chondroid chordomas were stained for vimentin with moderate intensity whereas typical chordomas were only slightly stained. In comparison to typical chordomas, the chondroid chordomas had relatively few desmosomes and intermediate filaments. These findings suggest that intracranial chordomas are of mixed epithelial-mesenchymal nature, and that chondroid chordomas have a predominant mesenchymal character as compared to typical chordomas.

Adult↗

PubMed1984

[Three cases of intracranial chordoma. Typical chordoma and chondroid chordoma].

Three cases of intracranial chordoma: 2 cases of typical chordoma and a case of chondroid chordoma, were reported and the clinico-pathological difference between these two types of chordoma was discussed. It is well known that typical chordoma is composed of physaliferous cells containing various quantities of intracytoplasmic mucin histologically, whereas chondroid chordoma as Heffelfinger reported in 1973 is composed of cellular area with scanty myxoid matrix and Chondroid matrix. Clinical course of the typical chordoma shows recurrence of the tumor within 2 years after surgical removal and radiation therapy in the majority of the cases, and the average survival range is said to be about 4.1 years, while chondroid chordoma takes benign course with average survival of 24.1 years after surgery and radiation therapy. One of our two patients with typical chordoma died from the recurrence two years after surgery and radiation therapy. However, the patients with chondroid chordoma showed no sign of recurrence over 2 years after subtotal removal and radiation. Surgical approach for the removal of the tumor was discussed as well. When the tumor is rather small and located at the upper clival region, transsphenoidal approach may be preferable, otherwise other approach should be chosen. When tumor has forward extension over the clivus, subtemporal approach will be most favorable with wider operative field. Identification of vessels and nerves around the tumor will be readily obtained by this approach.

Adult↗

PubMed1995

Chondroid chordoma. A hyalinized chordoma without cartilaginous differentiation.

"Chondroid chordoma" is a controversial and confusing entity that was originally described by Heffelfinger and colleagues as a biphasic malignant neoplasm possessing elements of both chordoma and cartilaginous tissue. Because the premise for this distinction was based strictly on histomorphologic criteria, the light microscopic, immunohistochemical, and electron microscopic features of the chondroid and chordoid areas of five chondroid chordomas of the skull base were evaluated separately, and compared to five typical chordomas and six low grade chondrosarcomas. Using light microscopy, chondroid chordoma revealed areas that resembled typical chordoma (chordoid areas) and areas that resembled low grade chondrosarcoma (chondroid areas). However, both the chordoid and chondroid areas had an epithelial phenotype and stained strongly for cytokeratin and EMA as well as S-100. 5'-nucleotidase, an enzyme that has been described in chordoma but not in chondrosarcoma, was found in both the chordoid and chondroid areas of one chondroid chordoma. Electron microscopic studies of both the chordoid and chondroid areas in four of the tumors demonstrated both tonofibrils and desmosomes. Chordoma demonstrated immunohistochemical and electron microscopic features that were nearly identical to chondroid chordoma. Chordoma was cytokeratin, EMA, S-100, and 5'-nucleotidase positive. Ultrastructurally, chordoma exhibited variably-sized vacuoles, abundant rough endoplasmic reticulum (RER), and desmosomes with tonofilaments. In contrast to chondroid chordoma, chondrosarcoma consistently stained for only S-100 protein and was cytokeratin, EMA and 5'-nucleotidase negative. Ultrastructurally, chondrosarcoma demonstrated a flocculogranular matrix, glycogen, abundant RER, and scalloped cellular outlines, but lacked desmosomes with tonofilaments. These findings indicate that "chondroid chordoma" is a variant of chordoma with histologic features that may mimic chondrosarcoma. Despite the resemblance of these hyalinized areas to cartilaginous tissue, these tumors retain their epithelial phenotype. Biphasic differentiation is not present. These findings undermine the original premise for distinguishing "chondroid chordoma" from typical chordoma. The authors propose that these tumors be classified as "hyalinized chordomas," rather than "chondroid chordoma," to clarify their histogenesis and avoid confusion with chondrosarcomas of the base of the skull.

Adult↗

PubMed1994

Chondroid chordoma--a variant of chordoma. A morphologic and immunohistochemical study.

In 1973, Heffelfinger and coworkers described a variant of chordoma that contained cartilaginous areas indistinguishable from hyaline type chondrosarcoma. They designated these tumors chondroid chordomas and found that they had a better prognosis than classic (nonchondroid) chordomas. Since that time, there has been an ongoing debate over whether chondroid chordoma is best considered a distinct clinicopathologic entity separable from chondrosarcoma or a misdiagnosed chondrosarcoma whose concept developed from the erroneous interpretation of morphology. In an attempt to clarify the issue, the authors used light microscopy and immunohistochemistry to study 12 chondroid chordomas, 38 classic chordomas, and 28 chondrosarcomas that arose in the base of the skull or spine. As a reference, they also analyzed the immunohistochemical profile of fetal notochord, ecchordosis physaliphora, and fetal hyaline cartilage. They found that all chondroid and nonchondroid chordomas were positive for cytokeratin, and the majority were also positive for epithelial membrane antigen (EMA) and carcinoembryonic antigen (CEA). In contrast, none of the chondrosarcomas stained for cytokeratin, EMA or CEA. Vimentin and S-100 were positive in more than 95% of both classic and chondroid chordomas and chondrosarcomas. The immunohistochemical profile of these tumors was similar to the pattern of immunoreactivity of their nonneoplastic counterparts. The authors conclude that chondroid chordomas is a variant of chordoma and should not be confused with chondrosarcoma. Because chondroid chordomas have been reported to have a better prognosis, they felt that this nosologic term should be preserved and that chondroid chordoma should continue to be a focus of clinical and pathologic study.

Adolescent↗

PubMed2005

Incipient chordoma: a report of two cases of early-stage chordoma arising from benign notochordal cell tumors.

Chordomas are rare malignant bone tumors primarily involving both ends of the axial skeleton that present as destructive bone lesions with a large soft tissue mass. Chordomas were previously believed to arise from notochordal remnants. However, recent studies suggest the possibility that chordomas arise from benign notochordal cell tumors. We present two cases of coccygeal incipient chordoma that strengthen the new hypothesis. The first case was an 83-year-old man who died of prostatic adenocarcinoma. The second case was a 79-year-old man who died of hepatocellular carcinoma. The coccygeal tumors were composed of intraosseous and extraosseous infiltrative lesions. The intraosseous lesions consisted of both benign notochordal cell tumor and incipient chordoma. The extraosseous lesions were consistent with incipient chordoma. In addition, two other small benign notochordal cell tumors were found at a different level in case 1. It is conceivable that pre-existing intraosseous benign notochordal cell tumors transform into incipient chordoma and then extend through the cortex into the surrounding soft tissue. The incidence of incipient chordoma appears much higher than expected because chordomas are rare tumors with an incidence of one case per 1 000 000 persons per year. We suspect that unknown factors transform incipient chordoma into classic chordoma.

Aged↗

PubMed2001

Genome-wide analysis of sixteen chordomas by comparative genomic hybridization and cytogenetics of the first human chordoma cell line, U-CH1.

Cytogenetic information on chordomas is rudimentary and restricted to GTG-banding analysis of 26 cases worldwide. In this study, we present the chromosomal imbalances detected in a series of 16 chordomas (10 sacrococcyeal, five sphenooccipital, and one spinal) from 13 patients using comparative genomic hybridization (CGH) and fluorescence in situ hybridization (FISH). On average, 3.2 losses and 4.2 gains were detected per tumor. The most common DNA copy number alterations were losses on chromosomal arms 3p (50%) and 1p (44%). Losses of 3p were detected in five of seven primary chordomas. Therefore, the loss of 3p might be an early event in chordoma genesis. The most common gains involved 7q (69%), 20 (50%), 5q (38%), and 12q (38%). Additionally, we raised the first human chordoma cell line, U-CH1, from a recurrence of a sacral chordoma. U-CH1 and its parent tumor had almost the same CGH profile. According to GTG-banding and multicolor FISH, U-CH1 has the following clonal chromosomal abnormalities: der(1)t(1;22), del(4), +del(5), +del(6), +7, del(9), del(10), +der(20)t(10;20), +21. Thus, the novel permanent human chordoma cell line U-CH1 has chordoma-typical cytogenetic aberrations. Our data suggest that tumor suppressor genes or mismatch repair genes (located at 1p31 and 3p14) and oncogenes (located in 7q36) might be involved in chordoma genesis.

Adult↗

PubMed1998

Keratin subsets and monoclonal antibody HBME-1 in chordoma: immunohistochemical differential diagnosis between tumors simulating chordoma.

Thirty-five chordomas and more than 100 other tumors that have to be considered in the differential diagnosis, were immunohistochemically analyzed using a panel of antibodies including those to subsets of keratins (K), HBME-1, a monoclonal antibody recognizing an unknown antigen on mesothelial cells, and neuroendocrine markers. The patterns of immunoreactivities in chordoma were compared with those in renal cell carcinoma, colorectal mucinous adenocarcinoma, pituitary adenoma, skeletal chondrosarcoma, and extraskeletal myxoid chondrosarcoma (ESMC). Chordomas were consistently positive for keratin cocktail AE1/AE3, and for the individual keratins K8 and K19, and nearly always positive for K5, but they showed negative or only sporadic reactivity for K7 and K20. The keratin K8 and K19 reactivity was retained in those chordomas showing solid sheets of epithelioid, spindle cells, or cartilaginous metaplasia, and in one of two cases showing overtly sarcomatous transformation. In comparison, keratins were never present in skeletal chondrosarcoma, although K8 and to a lesser extent K19 were seen in occasional cases of ESMC with chordoid features. HBME-1 reacted strongly with chordoma and skeletal chondrosarcoma but was almost never positive in renal or colorectal carcinoma. These carcinomas lacked K5-reactivity, in contrast to chordoma. Chordomas were also consistently positive for neuron-specific enolase and occasionally focally for synaptophysin, but never for chromogranin. In contrast, pituitary adenomas regularly expressed the full spectrum of neuroendocrine markers and differed from chordoma by having a narrower repertoire of keratins, often showing negative or focal keratin 8- or AE1/AE3 reactivity and being almost always K19-negative. These findings indicate that chordoma can be immunohistochemically separated from tumors that can resemble it. Immunohistochemistry is especially useful in the diagnosis of small biopsy specimens that offer limited material for morphological observation.

Adenocarcinoma, Mucinous↗

PubMed1993

Chordoma cutis: a report of nineteen patients with cutaneous involvement of chordoma.

BACKGROUND: Chordoma, an uncommon tumor originating from remnants of the notochord, with cutaneous involvement has rarely been reported. OBJECTIVE: Our purpose was to document clinical manifestations, histopathologic features, immunohistochemical findings, treatment, and course of chordoma with cutaneous involvement. METHODS: Pathologically proven cases of chordoma were reviewed retrospectively for cutaneous involvement. Detailed clinical data and histopathologic changes were studied. Skin biopsy specimens were stained for immunohistochemical phenotyping. RESULTS: Of 207 cases of chordoma, 19 had skin involvement: as local recurrences or metastasis in 12, as direct extension of primary tumor in 6, and as a result of distant metastasis from sacrococcygeal chordoma in 1. Local recurrences were frequent, but distant metastasis to various organs, including skin, occurred. CONCLUSION: We propose the term chordoma cutis to describe this condition. In seven patients, cutaneous lesions were detected when the diagnosis of primary chordoma was made.

Adult↗

PubMed1994

Malignant fibrous histiocytoma in chordoma--immunohistochemical evidence of transformation from chordoma to malignant fibrous histiocytoma.

Sarcomatous transformation in chordoma (dedifferentiated chordoma) is a very rare condition and has been emphasized as a distinct entity because of its more aggressive clinical course. Here we describe a case of dedifferentiated chordoma (chordoma associated with malignant fibrous histiocytoma) arising from the sacrococcygeal region of a 55-year-old woman. The results of immunohistochemical stain in the chordoma area were strong positive for cytokeratin, epithelial membrane antigen and S-100 protein. The spindle and giant cells in the transitional areas of chordoma and malignant fibrous histiocytoma were positive for cytokeratin and epithelial membrane antigen in addition to vimentin and alpha-1-antichymotrypsin. The spindle and giant cells in the central area of malignant fibrous histiocytoma were negative for cytokeratin and epithelial membrane antigen, but positive for vimentin and alpha-1-antichymotrypsin. This supports the pathogenesis of sarcomatous transformation from chordoma.

Chordoma↗

PubMed2000

Sarcomatoid chordoma: chordoma with a massive malignant spindle-cell component.

We report a case of chordoma containing a spindle cell sarcomatoid component with a gradual transition from conventional chordoma. Immunohistochemically, many tumor cells in both conventional chordoma and sarcomatoid components were positive for cytokeratins (AE1/AE3, CAM5.2) and epithelial membrane antigen as well as vimentin. This report provides a rare example of sarcomatoid chordoma. Familiarity with this type of bone tumor should help to avoid confusion with dedifferentiated chordoma and other spindle cell sarcomas or carcinomas.

Aged↗

PubMed1993

Myasthenia gravis in a man with a history of chordoma: Observations of muscle-like antigens in chordoma

A 67-year-old white man with a remote history of a chordoma of the clivus presented with myasthenia gravis. We investigated the possibility that these conditions were related immunologically. Tissue sections of various chordoma specimens were reacted with dilutions of patient serum and control serum by an indirect immunoperoxidase method. In addition, sections were reacted with antibodies to muscle antigens. Of six chordomas, five reacted positively to patient serum. None reacted to control serum. One chordoma reacted positively to desmin, and all six reacted positively to myoglobin. We propose that the patient may have produced antibody to muscle-like antigens of the chordoma that subsequently cross-reacted with acetylcholine receptor and led to clinical myasthenia gravis.

Actins↗

PubMed1985

A comparative ultrastructural study of chondrosarcoma, chordoid sarcoma, chordoma and chordoma periphericum.

The present study is based on electron microscopical examinations of 15 conventional chondrosarcomas, 1 clear cell chondrosarcoma, 3 mesenchymal chondrosarcomas, 2 so-called chordoid sarcomas (extraskeletal myxoid chondrosarcoma), 4 sacrococcygeal chordomas, 2 ecchordoses and 1 neoplasm of tibia with features of a true peripheral chordoma (parachordoma). The neoplastic cells from various types of chondrosarcoma shared a number of features with nonneoplastic chondrocytes as e.g. a well-developed rough endoplasmic reticulum and microvillous cytoplasmic processes. In clear-cell chondrosarcoma, glycogen accumulation in the tumour cells was a prominent feature. The cells of mesenchymal chondrosarcoma usually showed the characteristics of immature mesenchymal cells. In contrast, chordomas commonly contained physaliferous cells with two types of vacuoles in their cytoplasm. The first type can be most adequately characterized as intracytoplasmic pseudoinclusions of intercellular substance, whereas the other type, glycogen-containing, single membrane-bound vacuoles most probably correspond to autophagosomes (cytolysosomes). Only vacuoles of the first type were recorded in the so-called chordoid sarcoma. They were also seen in chondrosarcomas. In contrast, both types of vacuoles were identified in the above-mentioned tibial tumour which, in addition, showed even other cytological characteristics of chordoma. The findings presented here have demonstrated distinct structural relationships between chordoid sarcoma and chondrogenic tumours. On the other hand, our observation of the uncommon tibial neoplasm indicates the possibility that tumours identical with chordoma may occur at sites other than the axial skeleton.

Bone Neoplasms↗

PubMed1992

[Coexpression of intermediate filaments in notochords and chordomas and its significance in the histogenesis and diagnosis of chordoma].

Six chordomas, ten fetal notochords and eight adult notochords were stained for keratin, vimentin, GFAP, desmin, CEA, EMA and s-100 protein. Coexpression of four kinds of intermediate filaments was common in fetal notochords and chordomas. According to the histologic and immunohistochemical characteristics, chordoma may originate from the ectopic remnants of undegenerated notochord during the embryonic stage. The chordoma consists of two cell types; nonvacuolated and physaliphorous cells, and exhibits the potentiality of multidirectional differentiation. The coexpression of different types of intermediate filaments could explain the morphological transformation of chordoma.

Adult↗

PubMedDate not supplied

Dedifferentiated chordoma arising in irradiated sacral chordoma.

A patient who received radiation therapy for a sacral lesion 10 years ago and eventually developed dedifferentiated chordoma at the primary site is reported. Initially, the patient received 54 Gy with Co-60 to the sacral tumor, and 6 years later an additional 50 Gy with 10 MV X-rays to the local recurrence. Four years later, an evident sacral tumor was noticed. When readmitted for re-confirmation of histology, a large rapidly growing lung metastasis was observed. The patient eventually died of progressive disease with incidental aspiration pneumonia. At autopsy, 10 years after the initial treatment, the recurrent sacral tumor proved to be dedifferentiated chordoma accompanied by typical chordoma. Although this case suggests the possible malignant transformation of chordoma rather than collision, the factors inducing this change remain unknown.

Chordoma↗

PubMed1996

Proliferative activities in conventional chordoma: a clinicopathologic, DNA flow cytometric, and immunohistochemical analysis of 17 specimens with special reference to anaplastic chordoma showing a diffuse proliferation and nuclear atypia.

Chordoma shows various degrees of atypia histologically, however, the relationship between the histological features and the biological behavior still remains controversial. The authors subclassified 17 specimens with chordoma into two groups (ie, trabecular type showing a trabecular patterns and solid type mainly consisting of a diffuse proliferation of tumor cells). The histological grading was performed according to the degree of nuclear atypia on a scale of 1 to 3. Using DNA flow cytometric and immunohistochemical techniques, both the proliferative index (% S + G2 + M phase) and the MIB-1 labeling index (LI) of the tumor cells were estimated regarding their proliferative activities. In addition, p53 overexpression was also investigated using immunohistochemical techniques. There were eight (47.1%) specimens of trabecular type and nine (52.9%) of solid type. In nine specimens of solid type, those with higher nuclear atypia (grade 2 or 3) were significantly more frequent (five specimens, 55.6%) than in trabecular type in which all of the eight specimens were grade 1 (P = 0.44). The proliferative index was significantly higher in grade 2 or 3 lesions than in grade 1 lesions (P = .014), and the MIB-1 LI tended to be higher in solid type than in trabecular (P = .088). p53 overexpression was detected in two specimens of solid type, and the MIB-1 LI in these two specimens was significantly higher (P = .037) than that in the specimens without p53 overexpression. It was considered that the preceding anaplastic histological features, including either diffuse proliferation or high grade nuclear atypia, together with p53 overexpression, were thus closely related to the proliferative activities in chordomas.

Adolescent↗

PubMed1993

[Intracranial chordoma. Case report of a destructively growing chondroid chordoma in the area of the sella turcica].

Evaluating the literature a short survey is given about chordomas of intracranial localization. A case of a chondroid chordoma growing destructively in the base of the skull is described. Because of the localisation in the region of the sella turcica the first diagnosis was an adenoma of the hypophysis or craniopharyngioma. Although it is a chondroid chordoma 14 months after operation a recidive tumour was seen.

Chordoma↗

PubMed1988

Local control of recurrent clival and sacral chordoma after interstitial irradiation with iodine-125: new techniques for treatment of recurrent or unresectable chordomas.

Using new 125I brachytherapy techniques, we were able to deliver safely a tumor volume dose of 16,000 rads to a previous irradiated, large, recurrent sacral chordoma by means of the intraoperative interstitial implantation of 229 low activity 125I seeds and 40,000 rads to a previously irradiated, small, recurrent clival chordoma by means of the transnasal needle implantation of two high activity 125I seeds. Iodine-125 brachytherapy was followed by regression of tumor, lessening of symptoms, and bony recalcification in both cases.

Adult↗

PubMed1978

[Chordomas. Excision of a lumbar chordoma: 2 1/2 years survival without signs of recurrence and without functional complications].

In connection with surgical removal of a chordoma in the lumbar region by large excision, followed by 31-month survival of the patient without complications, personal experience of 8 chordomas operated on over the last 20 years is reported. The authors believe their observations show the value of active surgical treatment combined with radiotherapy, which has a palliative effect on the pain and slows the evolution of the tumor. The effectiveness of surgical treatment and radiotherapy leads the authors to challenge the negative attitude generally taken towards the treatment of this condition and to adopt a position of moderate optimism.

Abdominal Muscles↗