Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.4.22.36 (caspase-1)
 6,285 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Ewing's sarcoma is the most common malignant bone tumour occurring in children and adolescents and exists in two different clinicopathological entities: osseous Ewing's sarcoma (OES) and extraosseous Ewing's sarcoma (EES). Five cases of primary epidural EES are described, which presented with non-specific symptoms leading to a long diagnostic delay. The median age at diagnosis was 22 years (range 13-36 years). The median diagnostic delay was 3 months. All patients had one or more neurological deficits. All underwent surgical exploration with a laminectomy and partial resection followed by adjuvant radiotherapy to a dose of 46-50 Gy and chemotherapy with VAC (vincristine, adriamycin and cyclophosphamide) alternating with ICE (ifosphamide, cisplatin and etoposide) for at least six cycles. The mean follow-up period is 21.2 months (range 11-32 months). Four of the five patients achieved a complete remission and are disease free at the time of writing this report. Two patients have a residual neurological deficit--both having presented with long history of neurological deficit. Primary spinal epidural EES should be suspected whenever young patients present with back pain and/or radicular pain, have abnormal neurology and an extradural mass is demonstrated on MRI. Surgical excision followed by adjuvant radiotherapy (50 Gy) and combination chemotherapy (VAC alternating with ICE) achieved local and systemic control in these patients. A greater number of patients and longer follow up are required to evolve a generally accepted treatment policy for this aggressive but potentially curable malignancy.
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PMID:Primary spinal epidural extraosseous Ewing's sarcoma: report of five cases and literature review. 1153 70

A 32-year-old Chinese man without significant medical history presented with a 2-month history of worsening lumbosacral back pain radiating to the groin and left lower extremity. He also described a shooting pain in the sciatic nerve distribution that began with and was exacerbated by alcohol consumption, a rare but known symptom of Hodgkin lymphoma (HL). On exam, an anterior chest wall mass was noted. Radiographic evaluation of an anterior mediastinal mass showed osseous erosion into the manubrium. MRI of the lumbar spine showed diffuse osseous disease of the lower thoracic and lumbar spine with extension into the right sacroiliac joint and S2 neural foramen without vertebral body collapse or stenosis. Tissue biopsy revealed nodular sclerosis HL, stage IVB IPS2. The patient was primary refractory to ABVD (adriamycin, bleomycin, vinblastine, dacarbazine) therapy. Salvage ICE (ifosfamide, carboplatin, etoposide) chemotherapy was used and then followed by non-myeloablative haploidentical bone marrow transplant was performed on 5 March 2019.
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PMID:Hodgkin lymphoma presenting as alcohol-induced back pain. 3178 Jun 9