Three Danish cases of malignant childhood lymphoma ("Burkitt").
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A seven-year-old neutered female golden retriever was presented for an acute onset of cervical pain and ataxia. Intervertebral disc disease or neoplasia was suspected. There was no evidence of a soft tissue or bony mass on physical examination or survey radiographs. Cerebrospinal fluid analysis did not show any evidence of a neoplastic process. Myelographic evaluation identified an extradural mass lesion extending from inside the calvarium to the mid-body of the first cervical vertebra. No additional diagnostic tests were performed as the owners elected for euthanasia because of the high possibility of a neoplastic process with an accompanying poor prognosis. Multilobular tumour of bone was diagnosed on postmortem examination.
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The embryonic transcription factor TBXT (brachyury) drives chordoma, a spinal neoplasm without effective drug therapies. TBXT's regulatory network is poorly understood, and strategies to disrupt its activity for therapeutic purposes are lacking. We developed designed ankyrin repeat proteins that block TBXT-DNA binding (T-DARPins). In chordoma cells, T-DARPins reduced cell cycle progression, spheroid formation, and tumor growth in mice and induced signs of senescence and differentiation. Transcriptomic and proteomic analyses identified gene networks involved in cell cycle regulation, embryonic cell identity, and interferon response and revealed features of regulome components, such as susceptibility to pharmacologic inhibition and the fine-tuning of TBXT downstream effectors through IGFBP3. Finally, we found high interferon signaling in chordoma cell lines and patient tumors, which was promoted by TBXT and associated with sensitivity to JAK2 inhibitors. These findings demonstrate the potential of DARPins for probing nuclear proteins to understand the regulatory networks of transcription factor-driven cancers, including entry points for therapies that warrant testing in patients.
Sclerosing epithelioid fibrosarcoma is a recently described sarcoma in which ultrastructural evidence of fibroblastic differentiation forms part of the diagnostic criteria. This report describes a further case of this tumour, which showed evidence of both fibroblastic and perineurial differentiation by immunohistochemistry and electron microscopy, and which had areas of high grade morphology. The tumour metastasised and the patient died of disease 12 months after presentation. The relevance of these findings to diagnosis and differentiation in these tumours is discussed.
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A technique for securely stabilising the spine affected by malignancy has been developed. It provides excellent pain relief and some improvement of neurological deficit, and allows of laminectomy and radiotherapy. Although the technique is relatively simple, and the materials used are commonplace and inexpensive, it needs to be learnt which may mean that surgery for spinal malignancy will need the service of a trained surgeon, rather than that of an initiate.
Lesions of the central afferent and efferent pathways cause onset-delays of long-latency EMG responses from anterior tibial muscle after passive dorsiflection of the ankle joint in standing subjects. In 23 patients with spinal tumour or cervical stenosis and clinical signs of a medullary lesion, short-, medium- and long-latency EMG responses from distal leg muscles after ankle dorsiflection were recorded prior to and after surgical intervention. Fifteen of the patients were re-examined between 1 and 2 years after surgery. The results of the follow-up study support the hypothesis of a supraspinal pathway for long-latency EMG responses in distal leg muscles and show their significance as a quantitative measure of sensorimotor functions.
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One hundred cervical and thoracic myelographies were performed with the non-ionic, water-soluble contrast medium metrizamide. Good myelograms were obtained in almost all cases, adverse effects were minor, no serious complications occurred, and no convulsions were seen. Metrizamide is better than gas or isophendylate for demonstrating intraspinal expansive lesions of affections of the root sleeves.
Extra-axial posterior fossa masses can be diagnosed reliably by computed tomography (CT) in most cases. Acoustic and trigeminal neurinomas, meningiomas, cholesteatomas, and other extra-axial masses can usually be distinguished from intra-axial masses by asymmetric widening of the basal subarachnoid spaces, bone destruction, continuity of the tumor mass with the tentorium or bone, and more sharply defined margins. Multiple-cut study of the posterior fossa improved visualization of the fourth ventricle and basal cisterns. Interpretation of cisternal changes in association with changes in the fourth ventricle and abnormal attenuation coefficients permits accurate diagnosis of posterior fossa masses.
Studies indicate that computed tomography (CT) has several important clinical applications in the evaluation of tumors of the musculoskeletal system. These include (a) diagnosis of lesions involving difficult areas such as the sacrum and ilium, (b) demonstration of soft-tissue components of bone tumors or primary soft-tissue masses, (c) assessment of bone-marrow involvement, and (d) follow-up after irradiation or chemotherapy. Several selected pediatric cases are described in detail.
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Two cases of children with paravertebral soft-tissue masses that histologically resembled Ewing sarcoma of bone are reported. In both cases the mass was located immediately above the diaphragm. It depressed the posterior segment of the diaphragm and indirectly caused displacement of the ipsilateral kidney, thus simulating an adrenal mass on excretory urography. The anatomic reasons for this phenomenon and the main features of the tumor are discussed.
The detection and differential diagnosis of extruded (free) disk fragments by CT has not been described in detail. We reviewed the lumbar CT scans of 57 patients with extruded disk fragments and of 31 patients in whom the CT appearance of disk fragments was simulated by other processes. An extruded disk fragment commonly appears on CT scans as an epidural mass that must be distinguished from either an epidural tumor or an anomalous root sheath. A normal posterior disk margin does not exclude a disk herniation when the nuclear fragment is extruded. Free disk fragments can be differentiated from root-sheath anomalies and tumors in most cases by measuring tissue densities and analyzing adjacent bone.
Twenty histologically verified intracranial and upper cervical chordomas were retrospectively studied with both magnetic resonance (MR) imaging and computed tomography (CT), and the advantages of each modality were compared with regard to three criteria: detection of tumor, delineation of extent of tumor, and characterization of tumor. MR imaging and CT were equivalent in permitting the detection of chordomas. MR imaging was considerably better in delineating the full extent of the tumor, which would influence establishment of treatment. MR imaging also provided a degree of histologic specificity that would be useful in prognosis.