bromochloroacetic-acid and Spinal-Neoplasms

Chordomas are uncommon, bone, axial, or (rarely) extra-axial tumors that are malignant and frequently recur but less commonly metastasize. They usually affect adults, with a very small proportion being pediatric tumors. For children, such tumors present a different biology, since they are more common as scull rather than sacral tumors, with aggressive histological features, including a loss of SMARCB1/INI1 and a dismal prognosis. Histologically, chordomas, believed to derive from notochordal tissue, characteristically show physaliphorous cells in a myxoid or chondroid matrix. Dedifferentiated and poorly differentiated forms can be observed. Moreover, a grading scale for chordomas has been proposed. Cytokeratin, EMA, S100, and brachyury are expressed by most chordomas. These are chemo-resistant tumors, for which surgical resection and/or radiotherapy are the treatments of choice. In this review, the histological, immunohistochemical, molecular, and clinical data of chordomas are discussed.

Topics: Chordoma; Fetal Proteins; Humans; Keratins; Neoplasm Recurrence, Local; Pathology, Molecular; Spinal Neoplasms; T-Box Domain Proteins

Topics: Cell Dedifferentiation; Chordoma; Diagnosis, Differential; Female; Fibrosarcoma; Humans; Immunohistochemistry; Keratins; Male; Middle Aged; Mucin-1; Sacrococcygeal Region; Sarcoma; Spinal Neoplasms; Vimentin

Chordomas are relatively rare tumors that arise from the neuraxis. Most often, chordomas are single lesions that metastasize late. There have been very few cases of chordomas arising from multiple foci along the neuraxis. Here, we present a case of a multicentric chordoma.. The patient presented with pain in her right neck and soreness in her right shoulder that she had experienced for about 2.5 years that she attributed to a muscle strain. She experienced worsening of her symptoms, which prompted her to seek medical care. The patient underwent an occiput-to-C6 posterolateral fusion with autograft and an occiput-to-C6 posterior segmental instrumentation, along with decompression of the spinal cord. One month after the initial surgery, the patient underwent a second surgery. The C2 and C3 vertebral bodies were completely resected, and a C1-C4 anterior fusion was then carried out. A C5 vertebrectomy and C4-C6 fusion were also performed at this time. The patient then received proton beam radiation to the entire affected area.. Recent studies have suggested that chordomas arise from benign notochordal tumors. We suggest that our patient suffered from multicentric chordomas with possible benign notochordal tumors. Although benign notochordal tumors do not require surgical resection, the possibility of transformation to a malignant lesion requires close follow-up.

Topics: Adult; Cervical Vertebrae; Chordoma; Female; Fetal Proteins; Follow-Up Studies; Humans; Keratins; Magnetic Resonance Imaging; S100 Proteins; Spinal Neoplasms; T-Box Domain Proteins; Vimentin

Case report.. We present a giant cervical chordoma without typical vertebral bony destruction in an 11-year-old girl.. Chordomas are rare malignant bone tumors that arise at both the cranial and the caudal ends of the axial skeleton, characteristically destroying the bone. Cervical chordomas comprise only 3% to 7% of all chordomas. To our knowledge, there is no case of cervical chordoma in a child, presenting without vertebral body involvement, in English literature.. Discussion on the patient's clinical, radiologic history, and histopathologic diagnosis of the resected tumor, with a review of the relevant background literature.. We report the first case of cervical chordoma in a child without typical vertebral bony destruction, the diagnosis of which was difficult to confirm before and after operation.. Giant notochordal rest and benign notochordal cell tumors (BNCTs) need to be recognized for differential diagnosis of this atypical chordoma. A long-term follow-up might be necessary for the diagnosis of this nontypical patient.

Topics: Cervical Vertebrae; Child; Chordoma; Female; Humans; Keratins; Radiography; S100 Proteins; Spinal Neoplasms; Treatment Outcome

Malignant fibrous histiocytoma (MFH), arising in combination with a sacral chordoma in a 70-year-old men, is described. Intermediate spindle-shaped cells demonstrating keratin positivity, showed a gradual transition between the areas of conventional chordoma, and the spindle cell areas, lending credence to the theory of a multipotential neoplasm. We chose the descriptive term "chordoma with malignant spindle cell component" in the sense that high malignant sarcomatous components exists in conjunction with chordomas in the primary tumor and the local recurrence. A review of literature is undertaken chronicling the documented associations of chordoma and sarcoma, followed by a discussion of the various causes proposed to explain this phenomenon.

Topics: Aged; Biomarkers, Tumor; Cell Division; Cell Transformation, Neoplastic; Chordoma; Coccyx; Diagnosis, Differential; Histiocytoma, Benign Fibrous; Humans; Keratins; Male; Neoplasms, Multiple Primary; Sacrum; Spinal Neoplasms

A case of paravertebral malignant rhabdoid tumor in a 59-year-old man is described. The location of the tumor and age of the patient appear to be unique. The clinical evolution was aggressive and resulted in the death of the patient. The tumor exhibited the light microscopic features of a typical rhabdoid tumor. It was composed of poorly differentiated round or polygonal cells with acidophilic glassy inclusions and vesicular nuclei exhibiting prominent nucleoli. Immunohistochemistry revealed the coexpression of cytokeratin and vimentin in neoplastic cells.

Topics: Cell Nucleus; Humans; Hyalin; Immunoenzyme Techniques; Keratins; Male; Middle Aged; Soft Tissue Neoplasms; Spinal Neoplasms; Vimentin

Chordomas are slow-growing tumors derived from notochord remnants. Despite margin-negative excision and postoperative radiation therapy, spinal chordomas (SCs) often progress. The potential of immunohistochemical (IHC) markers, such as epithelial membrane antigen (EMA), combined with machine learning algorithms to predict long-term (≥ 12 months) postoperative tumor progression, has been understudied. The authors aimed to identify IHC markers using trained tree-based algorithms to predict long-term (≥ 12 months) postoperative tumor progression.. The authors reviewed the records of patients who underwent resection of SCs between January 2017 and June 2021 across the Mayo Clinic enterprise. Demographics, type of treatment, histopathology, and other relevant clinical factors were abstracted from each patient's record. Low tumor progression was defined as more than a 94.3-mm3 decrease in the tumor size at the latest radiographic follow-up. Decision trees and random forest classifiers were trained and tested to predict the long-term volumetric progression after an 80/20 data split.. Sixty-two patients diagnosed with and surgically treated for SC were identified, of whom 31 were found to have a more advanced tumor progression based on the tumor volume change cutoff of 94.3 mm3. The mean age was 54.3 ± 13.8 years, and most patients were male (62.9%) and White (98.4%). The most common treatment modality was subtotal resection with radiation therapy (35.5%), with proton beam therapy being the most common (71%). Most SCs were sacrococcygeal (41.9%), followed by cervical (32.3%). EMA-positive SCs had a postoperative progression risk of 67%. Pancytokeratin-positive SCs had a progression rate of 67%; however, patients with S100 protein-positive SCs had a 54% risk of progression. The accuracy of this model in predicting the progression of unseen test data was 66%. Pancytokeratin (mean minimal depth = 1.57), EMA (mean minimal depth = 1.58), cytokeratin A1/A3 (mean minimal depth = 1.59), and S100 protein (mean minimal depth = 1.6) predicted the long-term volumetric progression. Multiway variable importance plots show the relative importance of the top 10 variables based on three measures of varying significance and their predictive role.. These IHC variables with tree-based machine learning tools successfully demonstrate a high capacity to identify a patient's tumor progression pattern with an accuracy of 66%. Pancytokeratin, EMA, cytokeratin A1/A3, and S100 protein were the IHC drivers of a low tumor progression. This shows the power of machine learning algorithms in analyzing and predicting outcomes of rare conditions in a small sample size.

Topics: Adult; Aged; Chordoma; Humans; Keratins; Middle Aged; Neoplasm Recurrence, Local; S100 Proteins; Spinal Neoplasms

Assess the significance of chordoma as a neurosurgical pathology, taking into account the latest edition of the WHO classification of soft tissues and bone tumors (2020).. An analysis of 28 chordomas was carried out. All chordomas were histologically verified, including using immunohistochemical markers of notochordal differentiation (S100, EMA, keratin, brachiuria protein).. Patients with chordomas accounted for 0.25% of the total number of neurosurgical patients. The vast majority (27) of chordomas had a cranio-vertebral localization. Sacral localization (S. Chordoma, due to its axial localization, naturally involves adjacent structures of the nervous system, has clinically significant neuropathological manifestations, and often provides direct indications for a special neurosurgical approach. This requires its consideration not only as a bone, but also as a neurosurgical oncological pathology, along with other non-meningothelial (mesenchymal) tumors of the CNS.. Оценить значение хордомы как нейрохирургической патологии с учетом последней редакции классификации ВОЗ опухолей мягких тканей и костей (2020 г.).. Проведен анализ 28 хордом у больных, прооперированных в РНХИ им. проф. А.Л. Поленова. Все хордомы верифицированы гистологически, в том числе с использованием иммуногистохимических маркеров нотохордальной дифференцировки (S100, EMA, кератина, белка брахиурии).. Пациенты с хордомами составили 0,25% от общего числа нейрохирургических пациентов. Подавляющее большинство (27) хордом имели кранио-вертебральную локализацию. Сакральная локализация (S. Хордома в силу своей осевой локализации закономерно вовлекает прилежащие структуры нервной системы, имеет клинически значимые нейропатологические проявления и часто предъявляет прямые показания для специального нейрохирургического пособия. Это требует ее рассмотрения не только как костной, но и нейрохирургической онкологической патологии, наряду с другими неменинготелиальными (мезенхимальными) опухолями ЦНС.

Topics: Chordoma; Humans; Keratins; Spinal Neoplasms

Topics: Humans; Keratins; Lumbar Vertebrae; Male; Middle Aged; Paraganglioma; Spinal Neoplasms

Paragangliomas are derived from neurosecretory cells believed to be of neural crest origin. A spinal location of paraganglioma is rare and usually presents as an intradural mass.. A primary intraosseous paraganglioma of sacrum is extremely unusual, and only 6 cases were reported. In this study, we report a rare case of a 44-year-old man with the complaint of low back pain and lower extremity weakness. Imaging workup, including computerized tomography (CT), and magnetic resonance imaging (MRI) presented an intraosseous sacral lesion with invasion of sacrum in the S1-S3 vertebrae, and extension to L4-L5 spinal canal. The patient underwent subtotal tumor resection, followed by radiation therapy.. The morphological and immunohistochemical studies revealed a composite tumor of paraganglioma and ganglioneuroma components, with immunopositivity for cytokeratin.. To the best of our knowledge, this is the first report in the literature demonstrating an intraosseous sacral paraganglioma with these 2 pathological features.

Topics: Adult; Bone Cysts; Ganglioneuroma; Humans; Keratins; Male; Paraganglioma; Sacrum; Spinal Neoplasms

Spinal chordomas are more often located on the midline and are associated with marked destruction of the vertebral bodies. We report a rare case of large cervical (C2-C3) right lateral paravertebral chordoma extending into the spinal canal through a very enlarged intervertebral foramen. The tumor was initially diagnosed as a mucous adenocarcinoma on a percutaneous needle biopsy. However, the neuroradiological features, including the well-defined tumor margins, the regular and sclerosing lytic bone changes with regular enlargement of the intervertebral C2-C3 foramen, were in favor of a more slowly growing lesion, such as schwannoma or neurofibroma. At surgery a well-demarcated capsulated tumor involving the nerve root was partially resected. Histology was in favor of a low-grade chordoma (Ki-67/MIB-1<1%). Postoperative proton beam therapy was also performed. The differential neuroradiological diagnosis is discussed.

Topics: Adenocarcinoma; Aged; Cervical Vertebrae; Chordoma; Cone-Beam Computed Tomography; Female; Humans; Keratins; Magnetic Resonance Imaging; Spinal Neoplasms

In this study, we report 2 pediatric cases of nuclear protein of the testis (NUT) midline carcinoma (NMC) suggestive of pulmonary origin: case 1 was a 14-year-old Japanese boy and case 2 was a 7-year-old Japanese girl. Initial symptoms of both cases were prolonged cough and chest pain, and the case 2 patient also complained of lumbago and lumbar mass due to bone metastases. Imaging studies revealed that pulmonary tumors from both patients were located at the hilar region of the lower lobe. Biopsies of the tumors showed undifferentiated carcinoma in case 1 and combined undifferentiated and squamous cell carcinoma in case 2. Despite intensive treatment with chemotherapy and radiation, progression of neither tumor was controlled, and both patients died of the tumors at 1 year (case 1) and 4 months (case 2) after onset of disease. Both tumors were diffusely positive for p63 and NUT expression and were partially positive for various cytokeratins. Reverse transcription polymerase chain reaction analysis and subsequent direct sequencing revealed that the bromodomain-containing protein 4-NUT chimeric gene was present in tumor tissue of both patients, leading to a diagnosis of NMC. The tumor cells of case 1 were also positive for thyroid transcription factor-1 expression, but those of case 2 were negative. Histologic examination of the surgically removed lung tumor of case 1 indicated that the origin of the tumor was basal cells of the bronchiolar epithelia.

Topics: Adolescent; Biomarkers, Tumor; Biopsy; Carcinoma, Squamous Cell; Cell Differentiation; Child; Disease Progression; Fatal Outcome; Female; Humans; Immunohistochemistry; Keratins; Lung Neoplasms; Male; Neoplasm Proteins; Nuclear Proteins; Oncogene Proteins; Oncogene Proteins, Fusion; Reverse Transcriptase Polymerase Chain Reaction; Spinal Neoplasms; Thyroid Nuclear Factor 1; Time Factors; Tomography, X-Ray Computed; Transcription Factors; Treatment Outcome; Tumor Suppressor Proteins

Chordomas are malignant tumours that occur along the spine and are thought to derive from notochordal remnants. There is significant morphological variability between and within chordomas, with some showing prominent areas of chondroid differentiation. Our microarray data from a broad range of connective tissue neoplasms indicate that, at the transcriptional level, chordomas resemble cartilaginous neoplasms. Here we show that chordomas express many genes known to be involved in cartilage development, but they also uniquely express genes distinguishing them from chondroid neoplasms. The brachyury transcription factor, known to be involved in notochordal development, is only expressed by chordomas. Using a polyclonal antibody, we show that brachyury is expressed in the embryonic notochord and in all 53 chordomas analysed, labelling both chondroid and chordoid areas of these tumours. In contrast, the protein was not detected in over 300 neoplasms, including 163 chondroid tumours. Brachyury was not detected in the nucleus pulposus, arguing against the hypothesis that this tissue derives directly from the notochord. These data provide compelling evidence that chordomas derive from notochord and demonstrate that brachyury is a specific marker for the notochord and notochord-derived tumours.

Topics: Biomarkers, Tumor; Cartilage Diseases; Chondrosarcoma; Chordoma; Fetal Proteins; Gene Expression Regulation, Neoplastic; Humans; Immunohistochemistry; Keratins; Notochord; Reverse Transcriptase Polymerase Chain Reaction; Spinal Neoplasms; T-Box Domain Proteins; Tissue Distribution

We report a case of monostotic low-grade stromal sarcoma (ESS) with sex cord-like elements metastatic to the thoracic spines, which to the best of our knowledge has not previously been documented. A 48-year-old female who had undergone total abdominal hysterectomy for low-grade endometrial stromal sarcoma 7 years previously presented with insidious onset of severe back pain of 2 months' duration. Magnetic resonance image (MRI) showed involvement of the eleventh and twelfth thoracic vertebral bodies. Decompression at the level of T10-12 was performed. Histologically, the laminae of thoracic vertebrae 11 and 12 were replaced by sheets of ovoid cells with plump nuclei intermixed with anastomosing trabeculae, cords and small nests, reminiscent of a sex-cord stromal tumor pattern. The tumor cells showed diffuse nuclear immunostaining for estrogen receptors (ER) and progesterone receptors (PR), as well as membranous immunostaining for CD10. The immunostaining for smooth muscle actin was focal and sparse. These findings confirmed the diagnosis of metastatic low-grade ESS with sex cord-like differentiation. Low-grade ESS with sex cord-like differentiation is an uncommon tumor which rarely metastasizes to the bone, and use of a panel of ER, PR, CD10, actin, cytokeratin and inhibin immunostains is essential to establish the diagnosis.

Topics: Actins; Endometrial Neoplasms; Female; Humans; Hysterectomy; Keratins; Magnetic Resonance Imaging; Middle Aged; Neprilysin; Receptors, Estrogen; Receptors, Progesterone; Sarcoma, Endometrial Stromal; Sex Cord-Gonadal Stromal Tumors; Spinal Neoplasms; Thoracic Vertebrae

The incidence of occult nodal metastases associated with head and neck squamous cell carcinoma (HNSCC) and the clinical significance of nodal micrometastases by cytokeratin immunohistochemical analysis are examined.. In all, 1012 lymph nodes from 50 patients treated between 1992 and 2001 at the University of Colorado Health Sciences Center (Denver, CO) were evaluated retrospectively for micrometastases.. Serial sectioning in 5-to 6-microm interval specimens stained either with hematoxylin and eosin (H&E) or immunostaining for cytokeratins using the monoclonal antibody cocktail AE1/AE3 was performed in 21 N0, 11 N1, and 14 N2 patient cases. Cases that showed scattered cells with suspect staining qualities but without morphological features consistent with HNSCC were further evaluated by epithelial membrane antigen (EMA) immunohistochemical analysis.. H&E-stained and cytokeratin-stained sections revealed occult nodal micrometastases in 3.8% of N0 and 5% of N1 cases. Overall, 26 micrometastases were identified in N0 and N1 patients, causing 29% of N0 patients and 45% of N1 patients to be upstaged. Cytokeratin immunostaining detected micrometastases in eight cases that were negative on H&E serial sectioning. Serial sectioning by H&E alone identified three additional micrometastases. Negative EMA immunostaining confirmed the absence of malignant cells in lymph node sections that were equivocal on cytokeratin staining.. The use of serial sectioning with H&E and cytokeratin immunohistochemical analysis increases the detection of micrometastases that are often elusive by routine processing in patients with HNSCC. Improved methods of detecting micrometastases may provide a basis for improved planning of postoperative therapy for patients already at risk for tumor recurrence.

Topics: Antibodies, Monoclonal; Carcinoma, Squamous Cell; Culture Techniques; Head and Neck Neoplasms; Humans; Immunohistochemistry; Keratins; Mucin-1; Neoplasm Metastasis; Neoplasm Staging; Spinal Neoplasms

Cytokeratin (CK)7 and CK20, the low molecular weight cytokeratins, have been found to have a benefit in the differential diagnosis of some epithelial neoplasms. In the present study, the actual role of these markers in the search of primary tumors in 32 patients with craniospinal metastasis of an unknown primary site at presentation, is evaluated. A series of 36 patients with a known primary tumor were presented for comparison. In the first group, two CK7 and CK20 expression profiles were observed; 87% of metastatic tumors were CK7+/CK20- and 13% CK7-/CK20-. The lung was the major source (82%) of CK7+/CK20- metastatic tumors, whereas it represented only 38% of primary tumor in the second group of a known primary site (P=0.006). Given the fact that metastatic tumors to the craniospinal axis of an unknown primary site are frequently CK7+/CK20-, and they have commonly metastasized from the lung, it is doubtful that immunohistochemistry is really helpful. However, CT scan and MRI of the chest still play an important role. Many patients in the present study had to undertake these imaging studies, regardless of the CK7/CK20 result. The immunostains may be useful in cases with other expression profiles, but such examples constituted only a minority in the present study.

Topics: Biomarkers, Tumor; Brain Neoplasms; Female; Humans; Immunohistochemistry; Intermediate Filament Proteins; Keratin-20; Keratin-7; Keratins; Male; Neoplasms, Unknown Primary; Spinal Neoplasms

Sarcomatous transformation in 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 arising from the skull base region of an 11-year-old boy, with tumor recurrence within one year. This tumor showed features of pleomorphic cell sarcoma with areas more typical of chordoma. Most of tumor cells expressed cytokeratin, epithelial membrane antigen, vimentin and S-100 protein, thus confirming the diagnosis of dedifferentiated chordoma.

Topics: Biomarkers, Tumor; Cell Differentiation; Cervical Atlas; Child; Chordoma; Combined Modality Therapy; Cranial Irradiation; Disease Progression; Fatal Outcome; Humans; Keratins; Male; Mucin-1; Neoplasm Proteins; Neoplasm Recurrence, Local; Osteolysis; Prognosis; S100 Proteins; Sarcoma; Skull Base Neoplasms; Spinal Neoplasms; Torticollis; Vimentin

A 70-year-old man was admitted to hospital because of oral and cervical masses. Computed tomographic scanning revealed a lobulated mass lesion in the retropharyngeal region, with a protruding extension in the oral cavity and with destruction of the second cervical vertebra. A biopsy was performed under the diagnosis of a retropharyngeal tumor. Histologically, this lesion was composed of vacuolated tumor cells in a solid or cord-like arrangement, with an abundant myxoid matrix. Immunohistochemically, the tumor cells were positive for pancytokeratin, epithelial membrane antigen and S-100 protein. The tumor was diagnosed as chordoma. Chordoma presenting as an intra-oral mass lesion is very rare.

Topics: Aged; Cervical Vertebrae; Chordoma; Diagnosis, Differential; Humans; Immunohistochemistry; Keratins; Male; Mouth Neoplasms; Mucin-1; S100 Proteins; Spinal Neoplasms

We report on the crush preparation findings of a case of "sarcomatoid" chordoma occurring in the sacral region of a 78-yr-old Chinese male. The smears showed clumps and small cords of polygonal tumor cells containing bubbly cytoplasm and round to oval nuclei. Focally, there were also aggregates of long filamentous spindle cells and stellate bizarre cells with marked nuclear pleomorphism. Occasional tumor cells were seen in association with dense amorphous material. Histologic examination of the excised specimen showed features of the so-called "sarcomatoid" chordoma which consisted of prominent foci of mitotically inactive spindle and pleomorphic cells, in addition to the conventional chordoma areas. An osteosarcoma-like pattern of probably metaplastic nature was also seen within the tumor. Immunohistochemical study showed that most tumor cells expressed cytokeratins. Ultrastructural examination revealed the characteristic rough endoplasmic reticulum-mitochondria complexes. While there are many spindle and pleomorphic cells seen in crush preparations, the distinction from other true high-grade malignancies is important. Recognition of these "pseudoanaplastic" cytologic features also helps to expand the morphologic spectrum of chordoma.

Topics: Aged; Biomarkers, Tumor; Chordoma; Cytodiagnosis; Histological Techniques; Humans; Immunohistochemistry; Keratins; Male; Microscopy, Electron; Organelles; Sacrum; Sarcoma; Spinal Neoplasms

This case report illustrates a patient presenting with sciatica and diagnosed with epithelioid sarcoma involving the spine.. The treatment of this patient involved multiple mass resections and decompressions of the spinal canal. Radio- and chemotherapy were applied once a clear diagnosis was obtained.. Epithelioid sarcoma is a rare tumor mainly arising in the extremities. Confusion with a benign inflammatory process are possible. Treatment after histologic diagnosis involves wide resection. To our knowledge, this report represents the first case of epithelioid sarcoma involving the spine.. After initial discectomy, recurrent scarring and mass formation required multiple decompression procedures and fusion. The initial pathology revealed inflammatory reaction on fibrosis. In a later procedure, the histologic diagnosis of epithelioid sarcoma was made. Radiotherapy and chemotherapy were begun immediately.. Despite aggressive resections, radiotherapy, and chemotherapy, the patient died 3 months after the last surgical procedure.. Spinal epithelioid sarcoma can be mistaken for a benign inflammatory process. After a histologic diagnosis, aggressive wide resection is necessary. Multiple recurrences are documented with this tumor in other sites. Prognosis in trunk involvement is less favorable than involvement of the extremities. The role of adjuvant radio- and chemotherapy is unclear for spinal involvement.

Topics: Adult; Electromyography; Humans; Keratins; Magnetic Resonance Imaging; Male; Reoperation; Sacrum; Sarcoma; Sciatica; Spinal Neoplasms; Tomography, X-Ray Computed; White People

In order to investigate the clinicopathological and immunohistochemical features of chordomas, 34 chordomas, with 5 chondrosarcomas for comparison, were studied by clinicopathological and immunohistochemical methods.. Based on the presence or absence of cartilaginous areas, chordomas are classified into two subtypes: chondroid chordoma (14 cases) and classic chordoma (20 cases). Chondroid chordoma occurred in a younger age group (mean age 40.9 years) than classic chordoma (mean age 51.1 years). 7/14 (50%) of chondroid chordomas occurred in the sacrococcygeal region, 4/ 14 (28.6%) occurred in the spheno-occipital region. Immunohistochemical staining showed that all chordomas were positive for cytokeratin, and 16 (47.1%) chordomas were also positive for EMA. In contrast, 5 chondrosarcomas were immunonegative for both cytokeratin and EMA. Vimentin and S-100 protein were positive in the majority of chordomas (29 & 24 respectively) and in the 5 chondrosarcomas. The present study confirms the dual features of chordoma-epithelial and mesenchymal, and also the utility of immunohistochemical staining in the differential diagnosis of chordoma and chondrosarcoma. The pathologic diagnosis of chondroid chordoma and other issues were also discussed in the study.

Topics: Adolescent; Adult; Child; Child, Preschool; Chondrosarcoma; Chordoma; Female; Humans; Immunohistochemistry; Infant; Keratins; Male; Middle Aged; Mucin-1; Sacrococcygeal Region; Skull Base Neoplasms; Spinal Neoplasms

A chordoma metastatic to the skin of the nose is reported. The patient (a 40-year-old man) had undergone excision of a sacral chordoma 16 months previously. In patients whose clinical histories are unknown, cutaneous metastases of chordoma can be confused with mixed tumors of sweat glands. Cytological features, including the presence of physaliphorous cells, and immunohistochemical coexpression of low molecular weight keratins and S-100 protein are helpful features that lead to a correct diagnosis.

Topics: Adult; Chordoma; Diagnosis, Differential; Humans; Immunohistochemistry; Keratins; Male; Nose Neoplasms; S100 Proteins; Sacrum; Skin Neoplasms; Spinal Neoplasms; Sweat Gland Neoplasms; Vimentin

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.

Topics: Adolescent; Adult; Antigens, Neoplasm; Carcinoembryonic Antigen; Chondrosarcoma; Chordoma; Female; Humans; Immunoenzyme Techniques; Keratins; Male; Membrane Glycoproteins; Middle Aged; Mucin-1; S100 Proteins; Skull Neoplasms; Spinal Neoplasms; Vimentin

Topics: Aged; Biopsy, Needle; Carcinoma, Neuroendocrine; Female; Humans; Keratins; Magnetic Resonance Imaging; Neoplasms, Unknown Primary; Phosphopyruvate Hydratase; Spinal Neoplasms; Thoracic Vertebrae

We report a case of osteosarcoma with coexpression of epithelial and mesenchymal markers immunohistochemically of the second lumbar vertebra in a 49-year-old man. Histologically, the tumor largely showed typical patterns of osteosarcoma with lacelike osteoid deposition and chondroblastic and fibroblastic areas. Immunoreactivity for osteocalcin (human bone gamma-carboxyglutamic acid-containing protein) was demonstrated in most of these tumor cells. Both primary and recurrent tumors contained areas that were composed of epithelioid cells with an alveolar pattern and a trabecular arrangement that resembled carcinoma. Some of the epithelioid cells gave positive reactions for cytokeratin and epithelial membrane antigen. On extensive examination, no other site of malignancy was detected. There have been a few reports of osteosarcomas with epithelioid cells, but to our knowledge, the immunohistochemical properties of this type of tumor have not been reported previously. This is an unusual case of osteosarcoma with epithelioid features.

Topics: Humans; Immunohistochemistry; Keratins; Male; Membrane Glycoproteins; Middle Aged; Mucin-1; Osteocalcin; Osteosarcoma; Spinal Neoplasms; Vimentin

Topics: Animals; Cervical Vertebrae; Chordoma; Ferrets; Keratins; Male; Spinal Neoplasms; Vimentin

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.

Topics: Adult; Chordoma; Female; Humans; Immunohistochemistry; Intermediate Filaments; Keratins; Male; Middle Aged; Notochord; Sacrum; Spinal Neoplasms; Vimentin

All definite cases of oncogenic osteomalacia have, until now, been classified as mesenchymal tumours. We report here a case of oncogenic osteomalacia caused by a spinal tumour. Microscopically, it resembled the mixed connective tissue variant of previously described phosphaturic tumours. Immunohistochemical studies, however, showed the tumour cells to be positive for low molecular weight cytokeratin (CAM 5.2), S100 protein, PGP 9.5, and synaptophysin. Electron microscopy demonstrated neurosecretory granules. The histopathological findings strongly suggest that this is a neuroendocrine tumour.

Topics: Adult; Cytoplasmic Granules; Endocrine System Diseases; Female; Humans; Immunohistochemistry; Keratins; Neuropeptides; Neurosecretory Systems; Osteomalacia; S100 Proteins; Spinal Neoplasms; Synaptophysin; Ubiquitin Thiolesterase

Altogether 20 chordomas were reported. The site distribution included 9 cases at the sacrococcygeal region, another 9 cases at the spheno-occipital region, 1 at the cervical vertebra, and another 1 at the lumbar vertebra. Histologic examination revealed that characteristic "physaliphorous cells" were easily identified in all the 19 cases. Tissue for immunohistochemistry study was available in 18 cases. Among them, tumor cells were found strongly positive to EMA, but negative for CEA. 16/18 cases also showed positive for keratin and S-100 protein. Totally, 2 cases were studied ultrastructurally and there were abundant RER and microfilaments seen in the cytoplasm of the tumor cells but only few surface microvilli detected. The epithelial nature of chordoma is strongly supported by the ultrastructural and immunohistochemical findings of these 20 cases.

Topics: Adult; Chordoma; Humans; Immunohistochemistry; Keratins; S100 Proteins; Sacrum; Sella Turcica; Skull Neoplasms; Spinal Neoplasms

The cytologic findings in a series of 17 patients with chordoma (one clivus, three cervical, one lumbar, and 12 sacral tumors), all of whom underwent fine-needle aspiration biopsy (FNAB) in the preoperative investigation, were studied. Cytologically, three main cell types were recognized: large, mononucleated or binucleated physaliferous cells with a vacuolated "bubbly" cytoplasm; small, rounded, uniform cells; and short spindle-shaped cells. The May-Grünwald-Giemsa staining was found superior to Papanicolaou staining in demonstrating the mucoid matrix and the vacuolated cytoplasm of the physaliferous tumor cells. Ultra-structurally, the tumor cells contained prominent bundles of filament of the intermediate type, as well as large vacuoles and lumina often bordered by microvillous projections; the cells were connected to one another by well-developed desmosomes. The resin embedding technique for the light and electron-microscopic examination of FNAB material (eight cases), the histochemical demonstration of sulphated glucosaminoglycans in the matrix (four cases), and the immunocytochemical analysis (four cases) with positivity for cytokeratin, epithelial membrane antigen, vimentin, and S-100 protein and negativity for carcinoembryonic antigen were found to be of value for the cytologic diagnosis of chordoma, and helped in distinguishing it from other chondrogenic tumors and metastatic mucous-producing carcinoma. From this study we conclude that a preoperative diagnosis of chordoma can be reached by FNAB, provided the findings are carefully evaluated in relation to the clinical and roentgenographic findings. Adjunctive histochemical, immunocytochemical, and ultrastructural techniques applied on the FNA material may be helpful in reaching a conclusive diagnosis when differential diagnostic problems occur.

Topics: Adult; Aged; Biopsy, Needle; Carcinoembryonic Antigen; Chordoma; Female; Glycosaminoglycans; Histocytochemistry; Humans; Immunohistochemistry; Keratins; Male; Membrane Glycoproteins; Middle Aged; Mucin-1; S100 Proteins; Skull Neoplasms; Spinal Neoplasms

A detailed immunohistochemical characterization of different cytokeratin subclasses was performed on frozen tumour tissue from three classical chordomas. Simple epithelium cytokeratins Nos 8, 18, and 19 were detected in all tumour cells while cytokeratin No. 7 was not found. Cytokeratins characteristic of squamous differentiation, including keratinization, were generally lacking, with the exception of the varying expression of cytokeratin No. 4. Vimentin was found in all the tumours, while they lacked desmin immunoreactivity. The present study indicates the co-expression of vimentin and cytokeratins, predominantly of the simple epithelium type. In addition, chordoma cells have the ability to express cytokeratins characteristic of squamous differentiation. This finding corresponds well to the electron microscopic findings of tonofilament bundles ending in well-developed desmosomes.

Topics: Antibodies, Monoclonal; Chordoma; Fluorescent Antibody Technique; Humans; Keratins; Spinal Neoplasms; Vimentin

Nineteen epithelial cysts in the central nervous system including six colloid cysts of the third ventricle, seven Rathke's cleft cysts in the sella, two enterogenous cysts in the posterior fossa, two epithelial cysts in the spinal canal and two neuroectodermal cysts in the cerebrum were examined immunohistochemically for expression of intermediate filament proteins-simple type, stratified type and skin type cytokeratins and GFAP. Colloid cysts of the third ventricle, Rathke's cleft cysts in the sella and epithelial cysts in the spinal canal expressed complex type cytokeratins while enterogenous cysts and neuro-ectodermal cysts showed only simple type cytokeratins. In addition, Rathke's cleft cysts expressed GFAP in occasional lining cells. The characteristic composition and distribution of cytokeratins in various kinds of epithelial cysts in the central nervous system are demonstrated and discussed with regard to their origins.

Topics: Adult; Aged; Aged, 80 and over; Brain; Brain Diseases; Brain Neoplasms; Child; Child, Preschool; Choroid Plexus; Craniopharyngioma; Cysts; Ependyma; Epithelium; Female; Humans; Keratins; Male; Middle Aged; Pituitary Gland; Pituitary Neoplasms; Spinal Diseases; Spinal Neoplasms; Spine

Chordomas are rare slow-growing but locally invasive tumors. They are thought to develop from residues of the notochord. Three chordomas and ten chondroid tumors were investigated by an indirect immunoperoxidase method for expression of the epithelial markers: MAM-6, keratin, tissue polypeptide antigen (TPA) and for carcinoembryonic antigen (CEA). In all the chordomas investigated the antigens MAM-6, keratin and TPA were detected using monoclonal antibodies and conventional antisera, respectively. These data provide further evidence of the epithelial nature and the ectodermal origin of chordomas. Moreover, the findings suggest that immunohistochemical methods may be useful for the differential diagnosis between chordomas and morphologically similar chondroid tumors.

Topics: Antibodies, Monoclonal; Bone Neoplasms; Carcinoembryonic Antigen; Chondrosarcoma; Chordoma; Coccyx; Diagnosis, Differential; Humans; Immune Sera; Immunoenzyme Techniques; Keratins; Lumbar Vertebrae; Membrane Proteins; Mucin-1; Osteosarcoma; Peptides; Sphenoid Bone; Spinal Neoplasms; Tissue Polypeptide Antigen

Five cases of chordoma, diagnosed by fine-needle aspiration (FNA) biopsy, are presented. Four cases were histologically confirmed, and in one, immunocytochemical and ultrastructural studies were performed on both the aspirate and tissue specimen. Four cases presented as sacral masses, while in the fifth case, a destructive lesion of the clivus extended into the soft tissues of the lateral neck. A spectrum of cytomorphologic features was encountered including the presence of abundant microtissue fragments and cells in a dissociate pattern, often with abundant metachromatic extracellular matrix. Stellate and cuboidal cells often contained intracytoplasmic vacuoles of varying sizes. Intranuclear inclusions, mitotic figures, and anisonucleosis were prominent features of several cases. Immunoperoxidase studies on a single case demonstrated cytoplasmic staining for low- and high-molecular-weight cytokeratins, vimentin, and epithelial membrane antigen, while glial fibrillary acidic protein and carcinoembryonic antigen were negative. Ultrastructural features included the presence of mitochondrial endoplasmic reticulum complexes, occasional desmosome-like junctions, and abundant extracellular matrix adherent to the tumor cells. We believe the cytomorphologic findings are characteristic and, when taken in concert with immunocytochemical and ultrastructural studies, allow differentiation of chordoma from other primary or metastatic neoplasms occurring in bone. As demonstrated in our series, chordoma is often an unsuspected diagnosis. We believe that FNA biopsy of these lesions can lead to a correct preoperative diagnosis and may also be utilized to document recurrence and thus facilitate the evaluation and management of patients with these lesions.

Topics: Aged; Biopsy, Needle; Bone Neoplasms; Cervical Vertebrae; Chordoma; Female; Histocytochemistry; Humans; Immunoenzyme Techniques; Keratins; Male; Microscopy, Electron; Middle Aged; Sacrum; Spinal Neoplasms