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Ein 87-jahriger Patient berichtete uber eine knotige, grosenprogrediente Struktur des anterioren Nasenseptums mit partieller Verlegung der Naseneingange. In der Bildgebung sowie intraoperativ zeigte sich die Raumforderung lokal begrenzt, ohne Anzeichen der Infiltration von Subkutis, Knochen sowie der angrenzenden Sinus paranasales. Histologisch zeigte sich ein chondroider Tumor mit lobularem Wachstum sowie physaliphorem Zytoplasma. Durch die immunhistochemische Untersuchung konnte bei Positivitat fur Brachyury und Negativitat fur ein EWSR1(Ewing-Sarcoma-Breakpoint-Region)-Rearrangement die Diagnose eines chondroiden Chordoms gestellt werden. Der vorliegende Fall belegt die differenzialdiagnostischen Uberlegungen zu diesem Tumor, der per se selten ist, aber auch an untypischen extraaxialen Stellen lokalisiert sein kann. Nach Literaturrecherche wurde eine Ubersicht aller von uns identifizierten 34 primaren extraossaren Chordome der Nase, des Nasopharynx und der paranasalen Sinus erstellt und der vorgestellte Fall in diese Ubersicht eingeordnet.

Chordomas are rare, malignant and locally aggressive tumors that are derived from the remnants of primitive notochord, out of which chondroid chordomas are even rarer. Very few case reports have described this variant, which is difficult to pick up on cytopathology alone and has a number of other differentials too. We report here a case of chondroid chordoma at the sacrococcygeal region that was diagnosed on FNAC in an elderly male patient emphasizing on its cytomorphology and how to differentiate from its cytological mimickers. Keywords: FNAC, notochordal tumors, Cartilaginous, Physaliphorous.

Parapharyngeal space (PPS) tumors are a rare entity and account for less than 1% of all head and neck tumors. Although they exhibit a benign behavior, malignant neoplasms may arise from any of the structure contained in the space. Differential diagnosis of a PPS mass can be determined based on its origin from the prestyloid or poststyloid space. Furthermore, the choice of surgical approach is dictated by the size of the tumor, its location, its relationship to the great vessels, and suspicion of malignancy. Chordomas are rare, slow-growing tumors arising from remnants of the notochord. They are locally aggressive malignant neoplasms. The majority of head and neck chordomas arise in the skull base with a small minority arising along the cervical spine. In this report, we describe the first case of a patient with a chondroid chordoma of the PPS with no direct involvement of the axial skeleton.

A unique case of eyelid metastasis from nasopharyngeal chondroid chordoma in a 63-year-old woman was reported. Chordomas are rare tumors of the bone deriving from remnants of the embryonic notochord. Histologically, the tumor showed lobulated structure and concludes two types of cells: liquid drop cell and small round/cubic cell. Immunohistochemically, AE1/AE3, epithelial membrane antigene (EMA), and S100 showed a uniform and strong positivity. It has a great capacity for recurrence and malignant transformation, despite their slow-growing nature. The most common sites of metastases are liver, lungs, and bones. The eyelid metastasis from chordoma is an extremely rare finding, which may suggest a poor prognosis for the patient. Its significant clinicopathological characteristic could prompt us to take it into consideration when assessing the patient's prognosis.

Chordomas are rare, slow-growing, and locally aggressive malignant neoplasms derived from primitive notochord remnants. The chondroid variety represents 14% of all chordomas mainly developing in the spheno-occipital region and presenting between the third and fifth decades of life. When developing intracranially, symptoms can range from headaches and neck pain to cranial nerve neuropathies and facial numbness. We illustrate a case of an adolescent woman who presented with excruciating facial pain, otalgia, decreased visual acuity, quadriparesis, headache, nausea, and dysphagia. Radiological studies revealed a large heterogeneous mass in the spheno-occipital region with clivus destruction. Biopsy and histopathology confirmed the diagnosis. Proper identification with prompt surgical resection and adjuvant radiotherapy remains critical to prevent complications.

Chordomas are rare tumors of the bone with an average incidence of 0.1 per 100,000 per year.1 Chordomas derive from remnants of the embryonic notochord, which extends throughout the axial skeleton. The incidence of distant metastases varies, ranging between 3% and 50% among the different series.2, 3, 4 The most common sites of metastases are liver, lungs, and bones. Although skin may be often involved because of direct extension from the primary tumor or local recurrences, distant skin metastasis from chordoma is an extremely rare finding, with less than 20 cases reported in the literature. We present here the unusual case of a 45-year-old woman with skin metastasis from a sacral chondroid chordoma.

Collision tumors are exceptional, associating two independent tumoral contingents. We report a case of an association of two rare tumors: sinonasal adenocarcinoma and chondroid chordoma. Initially, only adenocarcinoma was diagnosed. The treatment consisted of endoscopic endonasal surgery followed by conventional radiotherapy. After 18 months, a local recurrence was diagnosed after a facial trauma, but the true histology was difficult to assess. The tumor was dual, associating adenocarcinoma and chondroid chordoma, with atypical localization in the ethmoid. Further evolution was particularly aggressive. We discuss the key points of this observation.

Chondroid chordoma" is a controversial and confusing entity, a biphasic malignant neoplasm possessing elements of both chordoma and cartilaginous tissue. The annual incidence of chordoma is approximately one in one million. Chondroid chordoma is a subtype of chordoma possessing elements of both chordoma and cartilaginous tissue with better prognosis than classic (non chondroid) chordomas. We have come across a case of Chondroid Chordoma in a 55 year old male. Our report features particularly a rare entity, chondroid chordoma, with an exceptional localization to the nasal septum. Chondroid Chordomas are likely to recur and hence diagnostically important to institute effective treatment. MICROSCOPY: Showed a tumor tissue with a lobulated appearance, with the fibrous septae and mucoid intercellular tissue separating the lobules. Some of the tumor cells (physaliferous) were extremely large, with vacuolated cytoplasm and prominent vesicular nucleus; other tumor cells were small, with inconspicuous nuclei and no visible nucleoli. Mitotic figures were scanty or absent. Areas of cartilaginous differentiation were present. (Fig. 2) Immunohistochemical staining for S-100 protein, Cytokeratin and EMA were done. The tumor showed positivity for CK and EMA. It was negative for S-100 protein. (Fig. 3, 4). So the diagnosis was based on the Immunohistochemistry along with the histomorphological features.

Chordoma is a tumor of the axial skeleton that is distinctly uncommon in children and adolescents. Previous observations have suggested that chordomas in pediatric patients differ from adult chordomas in presentation, morphology, and behavior. This study examines the clinical and histologic features of chordomas in young patients (≤25 years old). All cases from the Mayo Clinic files were graphed according to age and a bimodal distribution was observed. The 35 cases representing the youngest population were selected for review. Histopathology ranged from low cellularity tumors with lobulated architecture and abundant myxoid matrix (conventional chordoma), to those with varying amounts of chondroid matrix (chondroid chordoma), to more cellular tumors (atypical chordoma), and finally to neoplasms with high-grade spindle-cell differentiation (dedifferentiated chordoma). Over an average follow-up period of 129 months (range 1 to 501 months), there were 13 deaths (37%) and 3 patients with metastasis. This survival rate was slightly better than the reported mortality rate for adults with chordoma, but there was a subset of young patients (those with atypical chordoma) that had a significantly worse survival rate, suggesting that histologic subtyping may be predictive of prognosis.

Cranial chordomas are rare tumors that have been difficult to study given their low prevalence. Individual case series with decades of data collection provide some insight into the pathobiology of this tumor and its responses to treatment. This meta-analysis is an attempt to aggregate the sum experiences and present a comprehensive review of their findings. We performed a comprehensive review of studies published in English language literature and found a total of over 2,000 patients treated for cranial chordoma. Patient information was then extracted from each paper and aggregated into a comprehensive database. The tumor recurrences in these patients were then stratified according to age (21 vs.21 years), histological findings (chondroid vs. typical) and treatment (surgery and radiation vs. surgery only). Data was analyzed via Pearson chi-square and t-test. A total of 464 non-duplicated patients from 121 articles treated for cranial chordoma met the inclusion criteria. The recurrence rate among all patients was 68% (314 patients) with an average disease-free interval of 45 months (median, 23 months). The mean follow-up time was 39 months (median, 27 months). The patients in younger group, patients with chordoma with chondroid histologic type, and patients who received surgery and adjuvant radiotherapy had significantly lower recurrence rate than their respective counterparts. The results of our systematic analysis provide useful data for practitioners in objectively summarizing the tumor recurrence in patients with cranial chordomas. Our data suggests that younger patients with chondroid type cranial chordoma treated with both surgery and radiation may have improved rates of tumor recurrence in the treatment of these tumors.

Chordoma is an uncommon malignant neoplasm derived from remnants of the embryonal notochord. The tumor arises in the sacrococcygeal region in most cases. Cytogenetic information on clival chordomas is scarce due to the low incidence of these tumors. In this study, we applied the G-banding and spectral karyotyping techniques to compare the karyotypes of three variants of clival chordoma: conventional, chondroid, and dedifferentiated. We describe a normal karyotype of a chondroid chordoma and a complex karyotype of a conventional chordoma involving chromosomes 1, 2, 3, 5, 8, 9, 11, 15, 19, 20, and X. The cytogenetic analysis of the dedifferentiated chordoma showed a polyploid complex karyotype of 71-123 chromosomes with double minutes that originated from chromosome 17.

Chordomas are known by primary malign tumors that have formed from primitive notochord remains and exhibit different epithelial properties. Morphologically these cells have distinct borders; the nucleus displays a monotony and blandness. It was found few cases of pleomorphism, atypia, anaplasia and hipercromatic nuclei. Vacuoles were seen in the cytoplasm. This study was realized during January 1995 until June 2005, with 22 surgeries of chordomas and intraoperative crush. The background was dirty with myxoid appearance, myxoid matrix growing in sheets or cord and had vacuoles showed more evidence of physaliferous cells. Chondroid chordoma showed vacuolated cells as well as other cells with eosinophilic cytoplasm and myxoid background. Single cells were observed only in two cases and high cellularity was observed in 8(42%) cases. Two cases were frank errors, only one had a partial correlation. The percentage of mistakes was 30% and our diagnosis accuracy was 70%. The correlation with clinical details and radiological findings were helpful in improving the accuracy rate. There was no differentiation between cytomorphological features smear of intracranial tumours and the sacrococcygeal location. Intraoperative analysis of chordomas could be helpful to have a good diagnosis and to have a better surgical resection.

背景:頭蓋内原発の軟骨肉腫はまれな腫瘍で, 多くは頭蓋底に発生する. 組織像が通常の脊索腫や軟骨腫様脊索腫に類似し, 鑑別診断の困難な場合があるが, 両腫瘍の予後は大きく異なり, その鑑別は臨床上重要である. 頭蓋底原発軟骨肉腫の1例を, 過去に経験した脊索腫9例, 軟骨腫様脊索腫1例との比較を通して, その細胞像と鑑別点について報告する.症例:症例は26歳男性, 近医にてトルコ鞍背側～右側頭骨内側部にかけて腫瘍を指摘され, 当院脳神経外科にて腫瘍切除術を受けた. 術中細胞診では, 粘液様物質を背景に腫瘍細胞が散在性に出現し, 核は小型で円形から卵円形, 核の腫大や二核も認めた. 軟骨への分化を示唆する1acunar structureを認めたが, 軟骨腫様脊索腫にみられる上皮様細胞接着や典型的な担空胞細胞 (physaliphorous cells) は認められず, 軟骨肉腫と診断した. 術後の組織所見, 免疫染色所見 (S-100蛋白+, Vimentin+, EMA-, Cytokeratins-) もこれを支持した.結論:粘液様背景や軟骨への分化を正確に捉え, さらに細胞間接着性を詳細に観察すれば, 脊索腫, 軟骨腫様脊索腫との細胞診による鑑別も可能と考えた.

In August 2007, a 4-year-old, intact, female Domestic Shorthair cat was examined for a mass on the tip of the tail. Histological examination performed after apical caudectomy revealed a neoplasm affecting the distal part of the last coccygeal vertebra. The neoplasm consisted of lobules of physaliferous cells surrounding cartilaginous tissue and a central core of trabecular bone. A diagnosis of chondroid chordoma was made based on histomorphological features and immunohistochemical results. Chondroid chordoma has been previously reported in humans, rats, ferrets, and mink. To the authors' knowledge, chondroid chordoma has not been reported in cats. Neither recurrence nor metastasis was reported 7 months after surgery.

Resumen Presentarnos el caso de una mujer de 68 anos con un cordoma condroide del penasco derecho que se extendia extracranealmente por el agujero rasgado posterior. Inmunohistoquimicamente mostraba un perfil peculiar con positividad para vimentina, proteina S100, queratinas (AEl-AE3) y CEA, siendo negativo para EMA, fenotipo no descrito hasta ahora. El cordoma condroide no esta reconocido corno una entidad definida por todos los autores. Tiene rasgos morfologicos de cordoma y condrosarcoma, lo que se corrobora por su inmunofenotipia inmunohistoquimica, que es la de esos dos tipos de tumor. Ocurre mayormente en la base del craneo, pero tambien en la region sacrocoxigea. Parece tener una mejor evolucion que los cordomas convencionales, aunque el numero de casos recogido en la literatura es todavia insuficiente para asegurarlo.

Chondroid chordomaは通常の脊索腫に軟骨様部分を伴う腫瘍であるが, 軟骨肉腫との異同を含め論議の多い疾患である. 頭頸部のchondroid chordomaの4例について, 特に軟骨様部分を中心に, 細胞像を含め病理組織学的, 免疫組織学的, 電顕的に観察した. その結果, 軟骨様部分は通常の脊索腫同様の上皮様性格を示し, 硝子様基質を伴うchordomaと考えられた. 少量の生検材料や細胞診では軟骨肉腫や軟骨様部分の多い多形腺腫との鑑別がしばしば問題となるが, cytokeratinやEMAの上皮系マーカーの証明が重要と考えられる. また, 再発を繰り返す例では他のものよりMIB-1陽性率が高く, p53蛋白陽性であり, 再発傾向や悪性度との関連が注目された.

Chordomas are rare tumours that arise from remnants of the primitive notochord. Chondroid chordoma is believed to be a variant of chordoma. We present a patient with a chondroid chordoma presenting with an oculomotor nerve palsy. Computed tomography (CT) demonstrated a mildly enhancing mass in the parasellar region and a markedly enhanced mass in the prepontine cistern. Magnetic resonance imaging also demonstrated a moderately enhanced, dumb-bell shaped mass. The parasellar mass was removed and the oculomotor nerve palsy disappeared three months after operation. Histology of the resected specimen revealed chondroid chordoma. One year after surgery she developed a left hemiparesis. CT demonstrated haemorrhage in the prepontine tumour. The encapsulated tumour with haematoma was removed and histology revealed that the tumour was composed of only chordoma tissue without cartilaginous tissue. The tumours located in the parasellar region and prepontine cistern enhanced differently on CT.

Clinical, radiological, and pathologic features of an intracranial chondroid chordoma in a 9-year-old boy are described. This is the first reported case of a chordoma, the center of which was laterally situated in the cranial base, lying in or near jugular foramen and carotid canal, but without midline involvement. Although cranial chordomas in childhood are extremely rare, and all previously reported cases appeared to have arisen in the clivus, this location should not be considered ectopic. Forking at the rostral end of the notochord has been demonstrated in embryos, and would be the presumed embryological source for this tumor. This is also the first reported case of a chondroid chordoma in a child with immunohistochemical documentation distinguishing it from a chondrosarcoma. This chondroid chordoma contained two populations of cells: neoplastic cartilage and chordoid tissue in a myxoid stroma. The distinction between chordoma and chondrosarcoma and the implications on treatment will be discussed.

Chondroid chordoma is an extremely rare tumour with an annual incidence of around 0.1 cases per 100 000 population. Involvement of the thoracic vertebrae may be present in 2%–5% of cases. Definitive diagnosis usually requires a suitable distinction between this and other mesenchymal tumours such as chondrosarcomas, so immunohistochemical analysis is virtually mandatory. In spite of its slow-growing nature, chondroid chordoma tends to relapse, and it may eventually become malignant, often jeopardising the patient's prognosis. Although surgery remains the main therapeutic approach, research into the molecular and genetic aspects of this tumour is ongoing. These new advances are likely to improve future oncology therapies by complementing surgery and radiotherapy, changing the currently poor prognosis. We report the case of a patient with a chondroid chordoma involving the thoracic vertebrae and pleural cavity, and the treatment performed.

The classification of cartilaginous tumors of the skull base, including chondroid chordoma and chondrosarcoma remains the subject of controversy. Critical review of the literature and our own experience of chordomas and cartilaginous tumors of the skull base led to the following conclusions: 1) Chondrosarcoma of the skull base is a distinct clinicopathological entity. The immunohistochemical staining pattern (cytokeratin negative, epithelial membrane antigen (EMA) negative) can be helpful in distinguishing it from chordoma with chondroid differentiation (cytokeratin positive, EMA positive). 2) The chondroid chordomas originally described by Heffelfinger et al. may have included some true chondrosarcomas with focal areas of myxoid chordomalike appearance. 3) Focal chondroid differentiation in chordoma is not such a rare phenomenon. Further study is needed to define whether chordoma with chondroid foci should be separated out from conventional chordoma as a distinct entity with a better prognosis.

Chordoma is an uncommon tumor of bone that is believed to develop from remnants of the notochord. The chondroid variant of chordomas contains elements possessing both chordoma and cartilaginous tissues. Chondroid chordomas occur almost exclusively in the sphenooccipital region of the skull base. Primary treatment with surgical excision and radiotherapy for residual or recurrent tumor is recommended. Here, we describe a patient with chondroid chordoma with skull base involvement who underwent an expanded endonasal endoscopic approach for complete resection. In conclusion, endonasal endoscopic approach should be kept in mind for the resection of carefully selected chondroid chordomas with skull base involvement because of its minimally invasive characteristics.

Made available in DSpace on 2015-12-07T15:30:03Z (GMT). No. of bitstreams: 0 Previous issue date: 2011. Added 1 bitstream(s) on 2015-12-07T15:53:53Z : No. of bitstreams: 1 PMC3317097.pdf: 3948159 bytes, checksum: 5bea21a9aea672cfa74aae5c8c14e4a0 (MD5) Chordomas are tumors derived from cells that are remnants of the notochord, particularly from its proximal and distal extremes, they are mainly midline and represent approximately 1% of all malignant bone tumors and 0.1 to 0.2% of intracranial neoplasms. Chordomas involving the sellar region are rare. Herein, we describe a 57-year-old male patient presenting with a history of retro-orbital headache, progressive loss of vision, and clinical features of hypopituitarism, for over 2 months. During evaluation, the CT scan revealed a large contrast-enhancing intrasellar tumor with a 3.6-cm largest diameter. The patient underwent transsphenoidal partial resection of the tumor, and histological examination was consistent with the diagnosis of chondroid chordoma. Although chordomas are rare, they may be considered to constitute a differential diagnostic of pituitary adenomas, especially if a calcified intrasellar tumor with bone erosion is diagnosed. Division of Endocrinology and Metabolism, Department of Internal Medicine, Faculty of Medicine of Botucatu, UNESP, 18618-970 Botucatu, SP, Brazil. Division of Endocrinology and Metabolism, Department of Internal Medicine, Faculty of Medicine of Botucatu, UNESP, 18618-970 Botucatu, SP, Brazil.

Background. Chordomas are rare neoplasms that show a proclivity for the spheno-occiput and sacral regions. A “chondroid” variant involving the spheno-occiput has been associated with improved survival. “Classic” or nonchondroid chordomas are uniformly immunoreactive for keratins. Chondroid chordomas are said to be immunonegative for epithelial markers, a feature used to support the concept that they represent chondrosarcomas. Methods. The authors performed immunohistochemical studies on 25 patients with chondroid chordoma (mean age, 40.0 years) and on 16 patients with classic chordomas (mean age, 44.2 years) to establish tumor subsets based upon immunophenotype, specifically reactivity for epithelial markers. Kaplan–Meier survival curves were then constructed for each group with age as an added variable. Results. All classic chordomas reacted for keratins as did 8 (32%) of the 25 chondroid chordomas. Forty-four percent of classic and 85% of chondroid chordomas were positive for S-100 protein. At 5 years, all patients younger than 40 years of age were alive in both the classic and chondroid groups. In contrast, of patients older than 40 years of age, only 22% with classic chordomas and 38% with chondroid chordomas were alive. Conclusions. Regardless of tumor subtype, age is the single most important variable in determining survival; patients younger than 40 years of age do better than older patients. There are no significant survival differences between patients with cartilage-containing tumors that are keratin immunopositive (“true” chondroid chordoma) or negative (chondrosarcoma). Immunostaining for keratins is of no prognostic value in assessing chondroid lesions of the spheno-occiput.

We report herein a case of lower clival chondroid chordoma, focusing on the surgical procedure of endoscopic endonasal surgery. A 36-year-old woman presented with progressive headache, right shoulder pain, and right hypoglossal nerve palsy. Computed tomography (CT) and magnetic resonance (MR) imaging revealed an extradural tumor located in the lower clivus, including the anterior aspect of the foramen magnum, deeply compressing the medulla and upper cervical spinal cord. Endoscopic endonasal surgery was performed via two nostrils. Since the basiocciput was destroyed by the tumor, removal of the tumor allowed identification of the middle clivus superiorly, the anterior arch of the atlas inferiorly, and anteromedial parts of occipital condyles bilaterally without drilling the basiocciput. The tumor was removed except for laterally and inferiorly extended lesions. Pathological diagnosis was chondroid chordoma. Postoperative course was uneventful, and the patient was discharged without further neurological deterioration. Endonasal endoscopic surgery provided safe and reliable tumor resection for a lower clival lesion. We believe that this minimally invasive procedure should be considered as an alternative to traditional surgical treatment.