1/1895. Complete paraplegia due to multiple intracerebral and spinal cavernomas. We report on a 29-year-old male patient with multiple intracerebral and spinal cavernomas. Bleeding in the thoracic cord at admission and additional bleeding which occurred 12 days later in the cervical cord resulted in complete paraplegia below thoracic level 4 (Th4). Four years earlier multiple cerebral cavernomas had been diagnosed by magnetic resonance imaging (MRI). Based upon reported cases in the literature multiple intracerebral and spinal cavernomas are exceptional. Additionally, the clinical presentation in our case is uncommon. ( info) |
2/1895. Cavernous angioma of the conus medullaris as a cause of paraplegia. Cavernous angiomas of the conus medullaris are unusual lesions, representing about 3% of all intramedullary cavernomas. Most are asymptomatic. magnetic resonance imaging (MRI) is the best diagnostic tool for the detection. We report a case of a 74-year-old man who initially developed low back pain and numbness of the right leg and subsequently paraplegia, asia impairment scale 'c'. MRI revealed a cavernous angioma of the conus medullaris with perilesional oedema and signs of acute bleeding. Clinical improvement was associated with changes in the MRI. ( info) |
3/1895. Conus medullaris hematomyelia associated with an intradural-extramedullary cavernous angioma. A unique case of a 50-year-old woman with a conus medullaris hematomyelia associated with a low thoracic intradural-extramedullary cavernous angioma localized 2 cm above is reported. The patient had a 2-month history of progressive paraparesis, hypoesthesia of legs, and bowel and bladder disturbances. The symptoms worsened acutely during the last days before admission. A thoraco-lumbar MRI showed a space-occupying lesion at T10-T11 (vertebral interspace associated with a hematomyelia localized about 2 cm below. A T10-L1 laminectomy was performed and complete removal of both lesions was obtained with microsurgical technique. A non-traumatic hematomyelia should always prompt the suspicion of a spinal AVM or, more rarely, of a cavernous angioma. The possible anatomical and clinical correlations of this unusual association are discussed. ( info) |
| Two young men with severe hypercalcemia in association with renal failure (one acute and one chronic) are reported in whom usual diagnostic tests failed to reveal an etiology, and the final diagnoses were given by bone marrow examinations. Early bone marrow examinations in specific patients with hypercalcemia of undetermined origin sometimes are vital as shown by our two patients. ( info) |
5/1895. An unusual case of the complete Currarino triad: case report, discussion of the literature and the embryogenic implications. OBJECTIVE AND IMPORTANCE: We present and illustrate an unusual case of the complete familial Currarino triad (an association between a bony sacral defect, a presacral mass, and an anorectal malformation) in which the teratoma arose from the conus medullaris and contained mature neurons, glia, and branching ependymal canals that were in communication with a terminal syrinx. The embryogenic implications are discussed. CLINICAL PRESENTATION: The patient was a term neonate when discovered to have imperforate anus. Further workup revealed lumbosacral dysraphism with a presacral mass, a rectovaginal fistula, and a single pelvic kidney. The family pedigree revealed a familial transmission pattern; the patient had a second cousin with anal atresia and a first cousin with similar sacral anomalies. The motor level was L4 with trace L5, and there was absent sensation in the sacral dermatomes. INTERVENTION: A diverting colostomy was performed on Day 14, and the infant returned at 3 months of age to undergo near-total resection through the previous abdominal approach. Only a subtotal resection was possible because the mass arose from the low-lying conus and was firmly adherent to the sacral nerve roots and iliac vessel. Follow-up magnetic resonance imaging performed 18 months after surgery revealed that the residual tumor had not progressed. CONCLUSION: Complete Currarino triad is rare and is familial in half of the cases. The special features of the tumor in our case were the presence of mature neurons with ependymal canals and its origin from the conus. The possible embryogenesis may provide evidence that the caudal notochord is important for organized secondary neurulation. ( info) |
6/1895. Meningeal chondrosarcomas, a review of 31 patients. We reviewed the literature to study the clinical features, the management and the outcome of meningeal chondrosarcomas. We included 31 patients in this review: 22 were mesenchymal and nine were non-mesenchymal. The mean age was 27 years and 64% arose from the cranial meninges. The treatment was mainly total surgical excision. Adjuvant therapy was given to 36% of patients. Spinal meningeal chondrosarcomas had a better prognosis (81% 1-year survival and 45% 3-year survival). There were no pathognomonic clinical or radiological features. We concluded that the best management of meningeal chondrosarcomas is total surgical excision whenever possible, followed by combined course of radiotherapy and chemotherapy as soon as possible. ( info) |
7/1895. Synovial sarcoma in the parapharyngeal space: case report and review of the literature. We encountered a rare case of synovial sarcoma in the parapharynx of a 47-year-old Japanese man. This patient presented with an enlarging tumor in the right side of his neck that had grown progressively over a 4-week period. Radiological examinations revealed that the tumor arose from the parapharyngeal space. The tumor could not be completely removed at surgery. Metastasis to the lumbar vertebra was detected postoperatively. The patient underwent three courses of chemotherapy and the delivery of palliative radiation to the lumbar vertebra without success. The patient died of lung metastasis 7 months after surgery. ( info) |
| A 74-year-old male patient presented with anal and sacral pain 18 months after abdomino-perineal resection for rectal cancer. Computerized tomography (CT) of the pelvis demonstrated a well defined mass anterior to the lower sacrum, posteriorly infiltrating and destroying the fourth and fifth sacral nerves and invading the right gluteal fossa. A 7.5 x 15 x 2 cm encapsulated mass was demonstrated during the operation using a posterior approach and the lower sacral segments together with the tumour were removed by amputation at S3 level. Histopathology revealed chordoma. This case is unique because of the rarity of chordoma in association with rectal tumour at the sacrococcygeal region. ( info) |
9/1895. Pelvic and lumbar metastasis detected by bone scintigraphy in malignant pleural mesothelioma. A case of a 43-year-old man suffering from pleural mesothelioma with distant bone metastasis is reported. The results of bone scintigraphy and NMR findings allowed the diagnosis. The current case describes a hematogenous metastasis to the pelvis and vertebral column from a malignant pleural mesothelioma that was detected initially by bone scintigraphy. ( info) |
10/1895. bone marrow metastases in disseminated alveolar rhabdomyosarcoma: case report with ultrastructural study and review. A case of desseminated alveolar rhabdomyosarcoma in an 18-year-old male with leuco-erythroblastic anaemia is described. Numerous bizarre malignant cells, including frequent multinucleated giant cells, were seen in bone marrow aspirates, and osteolytic lesions appeared late in the clinical course. The primary site of the neoplasm remained undertermined during life and also at necropsy, which revealed minute pulmonary metastases and extensive lymph nodal, pleural and skeletal deposits. The diagnosis was confirmed on necropsy tissue by ultrastructural examination which demonstrated numerous thin (5 nm) and thick (15 nm) intracytoplasmic filaments in tumour cells, sometimes organized in bundles; scattered dense Z-band-like bodies, and rod-shaped structures were also seen. The fine structure of the rhabdomyosarcoma in the present case is compared with previous ultrastructural studies. Elongated, thick intracytoplasmic filaments whose diameter corresponds to that of myosin myofilaments are strong evidence for rhabdomyoblastic differentiation and are considered to be the sine qua non of a positive electron microscopic diagnosis of rhabdomyosarcoma. Orgaized bundles of filaments and Z-band-like dense bodies are usually present, and rod-shaped structures are found infrequently, but none of these are necessary for the ultrastructural diagnosis. ( info) |