A rare intramedullary spinal cord metastasis from uterine leiomyosarcoma

Leiomyosarcoma is a rare smooth-muscle-derived malignancy with a significant malignant potential. Systemic metastases are a common late complication of leiomyosarcoma typically to lungs, liver, brain and bones. We report a 44-year-old woman with a prior history of uterine leiomyosarcoma who presented to us with a cervicothoracic intramedullary lesion and recent onset of neurological deficits. She underwent surgery with histological confirmation of a diagnosis of metastatic leiomyosarcoma, which was followed by adjuvant radiation and chemotherapy. To our knowledge there is no prior report of intramedullary spinal cord metastases (ISCM) from a leiomyosarcoma in the English literature. We report the present patient in view of the rarity of ISCM and its clinical significance. Even though ISCM are unusual, they should be suspected in any patient with primary malignancy irrespective of the histology. The overall prognosis remains grim irrespective of the treatment modality chosen and recognition of the same is important in preoperative counseling and overall treatment approach.     

Skull metastasis from uterine leiomyosarcoma: a case report

Uterine leiomyosarcoma is a rare malignancy whose presenting symptoms usually are not specific for the disease. The clinical presentations include vaginal bleeding, pelvic pain or pressure, and awareness of an abdominal-pelvic mass. The diagnosis should be considered if rapid uterine enlargement occurs, especially in a post-menopausal woman. Local spreading of the tumor could involve the myometrium, pelvic blood vessels and lymphatics, contiguous pelvic structures, and then the abdominal organs, whereas distant metastasis most often involve the lungs. A 63-year-old female presented a rare uterine leiomyosarcoma metastasis to the skull with the manifestions of a bulging mass over her left occipito-parietal region. On admission, neurological examination revealed right hemiplegia. She underwent total resection of the tumor with the reconstruction of the dura, the skull plate and the scalp. Her post-operative course was smooth and the muscle power of her right limbs was improved from grade 1 to grade 3 after the procedure. The histological diagnosis is leiomyosarcoma. Aggressive management of the metastatic skull tumor is recommended in selected patients at least for a betterquality of life.     

Intracranial dural cyst

Intracranial dural cyst is a very rare congenital malformation. The presence of such a cyst was confirmed by means of computed tomography and magnetic resonance findings in a child with an enlargement of the head and developmental retardation and confirmed histologically in surgically obtained specimens of cyst wall. Received: 12 August 1995 Revised: 20 September 1996     

Temporalis muscle metastasis of the uterine leiomyosarcoma: a case report

Leiomyosarcomas are malignant tumors of smooth muscle origin. These tumors are very rare in the head and neck region. The majority of leiomyosarcomas of the head and neck arise in the paranasal sinuses, oral cavity, jaws and superficial soft tissues like the scalp. A mass was observed in the right temporoparietal region of the scalp of a 76-year-old female. Two years before presentation, the patient was operated for primary uterine leiomyosarcoma. Over the ensuing years, the mass substantially increased in size. Radiologic findings revealed a mixed density mass of the right temporalis muscle. Histologic and immunohistochemical examination of the tumor showed a malignant mesenchymal neoplasm consisting of spindle-shaped atypical mesenchymal cells with marked pleomorphism and central cigar shaped nuclei arranged in fascicles. We report a rare case of uterine leiomyosarcoma metastatic to the temporalis muscle, proven by histopathology.     

Intracranial dural based chondroma

Intracranial chondromas are benign, slow-growing, cartilaginous tumors, which comprise only about 0.2% of all intracranial tumors. The majority of these lesions occur at the base of the skull, where they are thought to arise from residual embryonic chondrogenic cells along the basal synchondrosis. Very rarely, they may also originate from the convexity dura, falx cerebri, or the brain parenchyma. We present a patient with a dural based chondroma to highlight the technical considerations of surgical resection. The recent literature on intracranial chondromas regarding incidence, pathophysiologic origin, clinical symptoms, imaging, histopathology and prognosis is reviewed.     

Intracranial dural arteriovenous fistula with spinal venous drainage]

Two cases of intracranial dural arteriovenous fistulae draining into the medullary veins are reported. One patient was a 68-year old man who experienced brief and repeated episodes of paraplegia, followed by a permanent spinal cord syndrome. The other patient was a 74-year-old woman who rapidly developed a syndrome of the medulla and spinal cord. In both cases spinal cord angiography failed to show vascular malformations, but myelography revealed venous imprints and magnetic resonance imaging of the cervical spinal cord disclosed a pre-medullary signal. Cerebral angiography showed an intracranial arteriovenous fistula fed by the external carotid artery and draining into the veins of the posterior fossa and the spinal venous network. Complete occlusion of the fistula was achieved by embolization with microparticles. Intracranial dural arteriovenous fistulae draining into the medullary veins are rare: to our knowledge, only 4 cases have been reported. Such lesions must be considered whenever a spinal cord syndrome has no detectable cause.     

Intracranial haemorrhage resulting from unsuspected choriocarcinoma metastasis

A retrospective analysis of clinicopathological data of 10 patients with clinically unsuspected cerebral metastatic choriocarcinoma was carried out. All patients were young adult females. History of preceding pregnancy/abortion was forthcoming in 5 cases but none had a prior history of abnormal gestation. Features of raised intracranial tension followed by hemiparesis were the commonest presenting symptoms. A clinicoradiologic diagnosis of intracerebral haemorrhagic mass, either primary or secondary to tumour bleed, cortico-venous thrombosis or arteriovenous malformation, was entertained in 8 out of 10 cases. In other two cases, ring enhancing lesions prompted the diagnosis of granulomatous masses. Eight patients were operated upon, of whom two died after short hospital stay, and were autopsied. All had haemorrhagic masses noted at surgery/autopsy. Accurate diagnosis of metastatic choriocarcinoma was established only by histologic examination of these haemorrhages. This report emphasizes the importance of considering metastatic choriocarcinoma as an important differential diagnosis of haemorrhagic intracerebral lesions in women of child bearing age group. Measurement of serum/CSF level of HCG in suspected cases helps to implement early therapy. The diagnostic value of histopathologic examination of surgically resected blood clots in determining aetiology of intracerebral haemorrhagic masses is highlighted.     

Giant cell tumour of the sphenoid bone with dural extension

A case of giant cell tumour of the sphenoid bone is reported. Besides the familiar presentation with headaches, ocular manifestations, and upper cranial nerve lesions, this case showed previously undescribed features such as grand mal seizures, hemiparesis with facial palsy, and dural extension. The reported high incidence in females is questioned. The tumour histologically showed some cartilage.     

Inferior division third nerve paresis from an orbital dural arteriovenous malformation.

Isolated inferior division third nerve palsies are rare. The authors report a patient with an isolated, pupil-involving inferior division third cranial nerve palsy from an intraorbital dural arteriovenous malformation. Despite spontaneous thrombosis of the malformation, the third nerve palsy persisted. To the authors' knowledge, this is the first case report of an inferior division third nerve palsy caused by an orbital dural arteriovenous malformation.     

Intracranial extension of meibomian gland carcinoma with pagetoid changes

A 41-year-old man presented with exophthalmos and loss of visual acuity. Neuroradiological studies showed a large mass extending from the intraorbital region to the frontal lobe. In addition, it also involved the middle cranial and infratemporal fossae with accompanying skull destruction. The tumor was almost totally removed. The histological diagnosis was sebaceous carcinoma with pagetoid changes. Despite surgery and local irradiation, intracranial metastases were recognized one year later, and the patient underwent total tumor removal and whole-brain irradiation. Although multiple lung metastases were detected one year after the second operation, three years post-surgery he remains free of intracranial tumor recurrence. Sebaceous carcinoma of meibomian gland origin with pagetoid changes is a distinct, highly aggressive clinical entity. Early diagnosis and appropriate treatment are essential to improve the prognosis of patients with meibomian gland carcinoma with intracranial extension.     

Multiple cerebral metastasis of a retroperitoneal leiomyosarcoma

A 61-year-old woman presented with dementia, 7 years after an operation for retroperitoneal leiomyosarcoma. Magnetic resonance imaging (MRI) revealed enhanced masses with perifocal edema in the bilateral frontal regions and a very small mass in the right medial frontal region. The tumors in the bilateral frontal regions were completely removed surgically, and γ-knife radiotherapy was administered for the very small tumor in the right medial frontal region. The histological diagnosis was metastatic leiomyosarcoma. Postoperatively, an MRI showed that the perifocal edemahad decreased, and the symptoms gradually improved. Cerebral metastasis from a retroperitoneal leiomyosarcoma is very uncommon. Ideally, the tumors should be surgically removed because radiotherapy and chemotherapy are apparently ineffective. A combination of complete surgical removal and γ-knife radiotherapy may be effective in prolonging patient survival.     

Intracranial dural venous anomalies in familial cervical cystic hygroma

This report describes a 3-year-old male with a familial cervical cystic hygroma and intracranial dural venous abnormalities consisting of a falcine sinus between the superior sagittal and the straight sinus with a rudimentary posterior part of the superior sagittal sinus; a prominent occipital sinus with narrowed transverse sinuses; and no intraparenchymal vascular abnormality. We hypothesize that a genetic factor that resulted in familial cervical cystic hygromas may also have caused the intracranial dural venous anomalies.     

Intracranial dural arteriovenous fistulas: analysis of 60 patients

OBJECTIVE: To analyze and update the clinical symptomatology, CT and MRI findings, angiographic features, and therapeutic outcomes of patients with dural arteriovenous fistulas (DAVFs). BACKGROUND: Studies of DAVFs in a large number of patients have seldom appeared in neurology literature. METHODS: The authors investigated 60 consecutive patients with DAVFs who were admitted between January 1991 and January 2001. The DAVFs were graded into five types according to the classification of Cognard et al. [Radiology 1995;194:671-680]. Presumable etiologies, clinical features, imaging findings and therapeutic outcomes were evaluated on the basis of the location and type of DAVFs. RESULTS: Sinus thrombosis, previous trauma, craniotomy, cerebral infarction and acupuncture were detected as possible etiologic factors of DAVFs. The cavernous sinus (57%) was the most common location of DAVFs. Although the neurological symptoms were closely related to the location of the DAVFs, in some patients, there were also symptoms that did not reflect the location. Although the women outnumbered the men, the men presented with aggressive neurological manifestations more often (p < 0.05). Ten out of 12 patients (83%) with DAVFs involving locations other than the large sinuses presented with aggressive neurological manifestations. 70% of brain CTs and 81% of brain MRIs showed abnormal findings suggestive of DAVFs. Of 33 patients who underwent only endovascular embolization, 29 patients (88%) were cured or improved. Radiosurgery and surgical excision done in some patients provided fair results. Patients with DAVFs involving large sinuses showed a better therapeutic outcome than those in whom locations other than the large sinuses were involved, while patients without venous ectasia had more a favorable outcome than those with it (each, p < 0.01). CONCLUSIONS: DAVFs result from various etiologic factors, show diverse manifestations usually reflecting the location and can be treated successfully in most patients. Factors related to poor clinical outcome include male sex, the presence of venous ectasia and involved locations other than the large sinuses.     

Mandibular ameloblastoma with intracranial extension and distant metastasis

A case of mandibular ameloblastoma which invaded intracranially through the skull base and developed distant metastasis to the femur is reported. The intracranial extension of the tumor was partially removed three times and the metastasis of the femur was curetted. The remaining tumor temporarily responded to chemotherapy, but the patient died of intracranial tumor regrowth. The period from the initial treatment of the mandibular tumor to death was 21 years.     

Intracranial dural granulomas secondary to operative use of oxidised cellulose

A case is presented of intracranial dural granulomata which appear to have developed as a reaction to surgical use of oxidised cellulose.