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.
Case Report ; Chordoma/diagnosis/*pathology ; Female ; Histiocytoma, Fibrous/diagnosis/*pathology ; Human ; Immunohistochemistry ; Middle Age ; Neoplasms, Multiple Primary/*pathology ; Pelvic Neoplasms/diagnosis/*pathology

OBJECTIVETo report 2 rare cases of benign notochordal cell tumor (BNCT), according to WHO classification of tumors of soft tissue and bone (4th edition). Their radiologic and clincopathologic features and differential diagnosis were investigated.

METHODSTwo cases of BNCT were studied by retrospective review of the clinical, radiologic, pathologic and immunophenotypical findings. Related literatures were reviewed at the same time.

RESULTSCase 1 was a 53-year-old man, and case 2 was a 61-year-old woman. Radiographically, both patients presented with abnormal imaging findings in the fifth cervical vertebral body with the lesions located within the bone but without extra osseous mass. Histopathologically, the lesions lacked lobular architecture and extracellular myxoid matrix. The tumor cells were vacuolated and had centrally or peripherally placed round or oval nuclei with small nucleoli, mimicking mature adipocytes. No cytological atypia or mitotic figures were seen. The affected bone trabeculae were sclerotic and islands of bone marrow were often entrapped within the tumor.

CONCLUSIONSAlthough sharing similar anatomic distribution and immunophenotype to those of chordoma, BNCT has distinct radiologic and pathologic features and different treatment and prognosis. The differential diagnosis between BNCT and chordoma requires detailed clinical, radiologic and histopathologic evaluations.

Cervical Vertebrae ; diagnostic imaging ; pathology ; Chordoma ; pathology ; Diagnosis, Differential ; Diagnostic Imaging ; Female ; Humans ; Male ; Middle Aged ; Notochord ; diagnostic imaging ; pathology ; Radiography ; Retrospective Studies ; Spinal Neoplasms ; diagnostic imaging ; pathology

BACKGROUNDChordomas of the upper cervical spine are rare and present unique surgical challenge. This study aimed to describe the clinical characteristics and surgical management of patients with chordomas of the upper cervical spine.

METHODSTwenty-one patients with chordomas of the upper cervical spine who were treated in Peking University Third Hospital from January 1999 to October 2012 were retrospectively analyzed. Survival was calculated by the Kaplan-Meier method and was compared between groups using the log-rank test.

RESULTSThe postoperative diagnosis was classical chordoma in 20 cases and chondroid chordoma in one case. The mean operative time was 9.5 hours (range 6-17 hours), and the mean blood loss was 2 812 ml (range 700-4 800 ml). There were two postoperative deaths. Unilateral vertebral artery ligation was performed in six patients, cervical nerve roots were cut in six patients, and the external branch of the superior laryngeal nerve was repaired after being cut in one case. Two patients developed postoperative velopharyngeal incompetence, and loosening of the occipitocervical screws was observed in one patient. The recurrence rate was 66.7% (10/15) after a mean follow-up period of 46.8 months (range 14-150 months). The 5- and 10- year overall survival rates were (39.8±13.1)% and (31.9±12.7)%, respectively. There was a significant difference in survival rate between patients who underwent surgery and those who did not.

CONCLUSIONIn spite of the high rates of recurrence and complications after surgical treatment of chordomas of the upper cervical spine, intralesional resection combined with adjuvant radiotherapy remains the optimal treatment to prolong survival.

Adult ; Aged ; Cervical Vertebrae ; pathology ; surgery ; Chordoma ; diagnosis ; surgery ; Female ; Humans ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; diagnosis ; surgery ; Retrospective Studies ; Spinal Neoplasms ; pathology ; surgery ; Treatment Outcome

Chordoma ; metabolism ; pathology ; Diagnosis, Differential ; Elbow ; Humans ; Immunohistochemistry ; Male ; Mucin-1 ; metabolism ; Myoepithelioma ; metabolism ; pathology ; surgery ; Soft Tissue Neoplasms ; metabolism ; pathology ; surgery ; Vimentin ; metabolism ; Young Adult

OBJECTIVETo discuss the early diagnosis and the treatment of chordoma in the jugular foramen area.

METHODSThree cases of chordoma in the jugular foramen area were diagnosis and reviewed retrospectively. The initial symptom was facial paralysis. Surgical removal was performed through the infratemporal fossa approach in 1 case, through petro-occipital approach in 2 cases. For repairing facial nerve defect, great auricular nerve was used in 1 case and facial-hypoglossal anastomosis was used in another 2 cases.

RESULTSThe tumor were totally removed in all 3 cases. The patients were follow-up for 6 months to 1 year and no recurrence was found. The function of facial nerve was II grade (House-Brackmann) in one patient and IV grade in another 2 patients. No complications occurred in 3 cases.

CONCLUSIONSAlthough chordoma originating from the jugular foramen area were extremely rare, it was possible to make early diagnosis through finding clinical feature and imaging methods. The final diagnosis depended on pathology. Generally, the different surgical approaches were used according to the size and position of the tumor. The classic infratemporal fossa approach for tumor removal and facial nerve reconstruction should be considered in the patients with chordoma around jugular foramen.

Adult ; Chordoma ; diagnosis ; surgery ; Female ; Glomus Jugulare ; pathology ; Humans ; Male ; Middle Aged ; Otologic Surgical Procedures ; Retrospective Studies ; Skull Base Neoplasms ; diagnosis ; surgery ; Young Adult

Arrested pneumatization of the sphenoid sinus is a developmental variant that is not always well recognized and is often confused with other pathologies associated with the skull base. This report describes the case of a patient referred for cone-beam computed tomography (CBCT) imaging for dental implant therapy. CBCT demonstrated a well-defined incidental lesion in the left sphenoid sinus with soft tissue-like density and sclerotic borders with internal curvilinear opacifications. The differential diagnoses included intraosseous lipoma, arrested pneumatization of the sphenoid sinus, chondrosarcoma, chondroid chordoma, and ossifying fibroma. The radiographic diagnosis of arrested pneumatization was based on the location of the lesion, its well-defined nature, the presence of internal opacifications, and lack of expansion. Gray-scale CBCT imaging of the area demonstrated values similar to fatty tissue. This case highlighted the fact that benign developmental variants associated with the skull base share similar radiographic features with more serious pathological entities.
Adipose Tissue ; Chondrosarcoma ; Chordoma ; Cone-Beam Computed Tomography ; Dental Implants ; Diagnosis ; Diagnosis, Differential ; Fibroma, Ossifying ; Humans ; Lipoma ; Pathology ; Skull Base* ; Sphenoid Sinus*

OBJECTIVETo study the morphologic characteristics, immunophenotype and differential diagnosis of a case of microcystic/reticular schwannoma occurring in cervical spine.

METHODSThe pathologic features and immunophenotypic profile of a case of microcystic/reticular schwannoma were studied. Immunohistochemistry was performed using EnVision two-step method.

RESULTSThe patient was a 35-year-old male and presented with a bump over the fifth cervical spine on radiologic check up. Grossly, the bump was gray-white in color, soft, well-circumscribed but non-encapsulated. The tumor measured 3.5 cm × 3.0 cm × 1.8 cm in size. Histologically, it was composed of two distinctive components. One component resembled the conventional schwannoma but showed focally nuclear pleomorphism, reminiscent of changes in degenerating schwannoma. The other component consisted of epithelial-like cells arranged in a reticular or lace-like pattern, amongst a myxoid matrix. Immunohistochemical study showed that the tumor cells were strongly positive for vimentin, S-100 protein, glial fibrillary acidic protein and neuron-specific enolase, focally positive for CD68, CD10 and Ki-67, and negative for pan-cytokeratin, epithelial membrane antigen, neurofilament, carcinoembryonic antigen, smooth muscle actin, estrogen receptor, progesterone receptor and p53.

CONCLUSIONSMicrocystic/reticular schwannoma is a novel variant of schwannoma, arising mainly in internal viscera but seldom in bone. Awareness of this entity is helpful in distinction from chordoma, other mucoid tumors or sarcomas.

Adult ; Cervical Vertebrae ; Chondrosarcoma ; metabolism ; pathology ; Chordoma ; metabolism ; pathology ; Diagnosis, Differential ; Glial Fibrillary Acidic Protein ; metabolism ; Humans ; Male ; Neurilemmoma ; metabolism ; pathology ; surgery ; Phosphopyruvate Hydratase ; metabolism ; S100 Proteins ; metabolism ; Sarcoma ; metabolism ; pathology ; Spinal Neoplasms ; metabolism ; pathology ; surgery ; Vimentin ; metabolism

A giant vertebral notochordal rest is a newly described, benign entity that is easily confused with a vertebral chordoma. As microscopic notochordal rests are rarely found in adult autopsies, the finding of a macroscopic vertebral lesion is a new entity with only seven previously presented cases. We report here radiological findings, including diffusion weighted images, of a patient with a giant notochordal remnant confined to the L5 vertebra, with an emphasis on its distinction from a chordoma.
Chordoma/*diagnosis ; Diagnosis, Differential ; Diffusion Magnetic Resonance Imaging/methods ; Female ; Humans ; Low Back Pain/etiology ; Lumbar Vertebrae/pathology/radiography ; Magnetic Resonance Imaging/*methods ; Middle Aged ; Notochord/*pathology/radiography ; Physical Therapy Modalities ; Spinal Neoplasms/*diagnosis/therapy ; Tomography, X-Ray Computed

The term "chordoid meningioma" means meningioma, which is pathologically similar to chordoma, and previously reported that rarely associated with microcytic anemia and/or dysgammaglobulinemia especially in pediatric population. We present a case of this rare variant, which comprises less than 0.5% of all meningiomas. A 33-yr-old man visited our hospital, complaining visual field defect worsening over 7 yr. Neurological examination showed left homonymous hemianopsia. The brain magnetic resonance imaging revealed well enhancing right temporo-occipital mass with cystic portion. Histopathologic findings of resected tumor were compatible with chordoid meningioma which included trabeculae of eosinophilic, vacuolated cells in a myxoid matrix with prominent lymphoplasmacellular infiltration. The neoplastic cells were positive for vimentin and epithelial membrane antigen and negative for glial fibrillary acidic protein and cytokeratin. This is an adult case of chordoid meningioma without anemia or dysgammaglobulinemia.
Adult ; Antigens, CD20/biosynthesis ; Antigens, CD3/biosynthesis ; B-Lymphocytes/pathology ; Brain/pathology ; Brain Neoplasms/*diagnosis/*pathology ; CA-15-3 Antigen/biosynthesis ; Chordoma/*diagnosis/*pathology ; Human ; Magnetic Resonance Imaging ; Male ; Meningeal Neoplasms/*diagnosis ; Meningioma/*diagnosis ; T-Lymphocytes/pathology ; Vimentin/biosynthesis ; Visual Fields

Adenocarcinoma, Mucinous ; pathology ; Adult ; Aged ; Chondrosarcoma ; pathology ; Chordoma ; pathology ; radiotherapy ; surgery ; Combined Modality Therapy ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Nasopharyngeal Neoplasms ; pathology ; radiotherapy ; surgery ; Neoplasm Recurrence, Local ; Paranasal Sinus Neoplasms ; pathology ; radiotherapy ; surgery ; Retrospective Studies ; Sphenoid Sinus