Rare intracranial plasmacytoma manifestations. Case reports and review of the literature in diffuse plasmocytoma, in primary solitary extramedullary plasmacytoma in in primary solitary osseous plasmacytoma

Plasmacytomas can be divided into multiple, solitary osseous and solitary extraosseous/extramedullary plasmacytomas. Intracranial plasmacytomas of the dura, leptomeninx and cerebrum are well known from the literature. They are manifestations of multiple myeloma, intracranial extramedullary plasmacytoma or metastatic disease of extramedullary plasmacytoma in distant locations. We describe a cerebellar manifestation of a solitary plasmacytoma of the bone, and a leptomeningeal carcinomatosis of a multiple plasmacytoma. A summary of the literature concerning intracranial plasmacytomas is given. Dural manifestations of plasmacytoma have the same features as meningiomas in CT or MRI. Cerebral or cerebellar manifestations cannot be differentiated from brain tumors by means of CT or MRI. In CT, plasmacytomas show high-density lesions. T2w-MRI reveals a low-intensity lesion. In T1w-MRI, intense homogeneous contrast enhancement can be demonstrated.     

Solitary plasmacytoma of the skull: a case report

Solitary plasmacytoma of the skull is very rare and only 35 cases have been reported in the English literature. It remains controversial whether solitary plasmacytoma of the skull is essentially identical with solitary plasmacytoma of bone or not. Solitary plasmacytoma of bone including solitary plasmacytoma of the skull is characterized by a radiologically solitary bone lesion, neoplastic plasma cells in the biopsy specimen, fewer than 5% plasma cells in bone marrow, less than 2.0 g/dl monoclonal protein in the serum when present and negative urine test for Bence Jones protein (monoclonal light chain). Solitary plasmacytoma of bone tends to disseminate or progress to multiple myeloma even as long as 7-23 years after presentation. We report the first case of solitary plasmacytoma of the skull in which both beta2-microglobulin for detection of early renal disturbance and neoplastic plasma cell labeling index for detection of DNA synthesis were examined in order to predict the clinical course of solitary plasmacytoma of the skull.     

Primary pineal melanoma with long-term survival: case report

A rare case of primary pineal melanoma is reported. The patient was a 53-year-old woman who complained of a severe headache. Computed tomography and magnetic resonance images revealed obstructive hydrocephalus caused by a mass lesion in the pineal region. A biopsy was performed through an occipital transtentorial approach. A black pigmented solitary tumor was seen without leptomeningeal dissemination. Histologic examination revealed melanoma. Chemotherapy consisting of dacarbazine, ACNU, vincristine, and interferon was used. Follow-up imaging studies showed dramatic reduction of the tumor without recurrence for 4 years. This report demonstrates that a solitary primary intracranial melanoma without leptomeningeal dissemination and with rare mitoses may yield a good result with chemotherapy.     

Extramedullary plasmocytoma of the larynx. Analysis of four cases

Four cases of solitary extramedullary plasmocytoma of the larynx (3 men, 1 women) treated with radiotherapy are presented. Laboratory and X-ray examinations showed no evidence of systemic plasmacytoma. One patient was treated with conventional X-ray therapy and 3 patients with telegammatherapy Co-60. All patients survived 10 years with no evidence of disease.     

Neurotrophic factor and neuronal apoptosis]

We document the occurrence of a solitary extramedullary plasmacytoma (SEP) in a cardiac transplant patient. The diagnosis of plasma cell malignancy was confirmed by histopathologic and immunohistochemical examination of a nodular skin lesion. A complete systemic evaluation showed no evidence of metastatic disease. The patient was treated locally with radiation therapy (RT), but disseminated multiple myeloma developed 4 months after diagnosis. A variety of tumors have been reported to develop in the cardiac or renal transplant recipient, although plasma cell malignancies are rare. To our knowledge, this is the first reported case of an SEP in an organ transplant recipient.     

Primary pulmonary plasmacytoma

BACKGROUND: A patient was diagnosed with an extramedullary plasmacytoma of the lung after complete resection of the mass at thoracotomy. Immunoperoxidase staining of the mass revealed monoclonal lambda chains. Screening for multiple myeloma identified a small amount of M-protein in the blood, but no other evidence of multiple myeloma was found. METHODS: A literature search was conducted to determine the prognosis and the best way to manage the patient. RESULTS: Nineteen cases of primary pulmonary plasmacytoma were found in the literature. The age range was 3-79 years. Most of these cases were diagnosed at thoracotomy and treated by surgical excision. Immunohistochemical evaluation of the lesion is essential for diagnosis but was done in only three cases. CONCLUSIONS: Surgery and radiation therapy seem to be equally effective forms of treatment. The role of adjuvant chemotherapy is unknown. Local recurrences are rare. Follow-up data were inadequate to determine disease-free survival, progression to multiple myeloma, and overall survival in primary pulmonary plasmacytoma. Close follow-up is needed to detect progression.     

Oral manifestations and differential diagnosis of isolated hypoglossal nerve palsy: report of two cases

Isolated hypoglossal nerve palsy is rare, but occasionally it appears as the initial or solitary sign of an intracranial or extracranial space-occupying lesion, a head and neck injury, or a vascular abnormality of the internal carotid artery. Therefore it should be considered in differential diagnosis. We report two cases of isolated unilateral hypoglossal nerve palsy. In Case 1 the cause of the palsy appeared to be hypoglossal nerve neurilemmoma within the hypoglossal canal, whereas in Case 2 the cause could not be identified. Neither patient complained of any disability other than slight dysarthria. The tongue deviated toward the healthy side at rest and toward the affected side on protrusion. Hemiatrophy of the tongue with fatty displacement was demonstrated by means of T1-weighted magnetic resonance imaging. Dentists who might at times see patients with isolated hypoglossal nerve palsy should be aware of the significance of its oral manifestation, and they should be able to perform differential diagnosis of patients with the condition who appear for treatment.     

Sphenoethmoidal mucoceles with intracranial extension--three case reports

Three unusual cases of sphenoethmoidal mucoceles with rare intracranial extension are reported. A 64-year-old female presented with a 7-month history of right visual disturbance. Computed tomography (CT) and magnetic resonance (MR) imaging demonstrated a huge mass in the right middle fossa. She underwent right frontotemporal craniotomy. Postoperatively, her proptosis and cranial nerve dysfunction had improved markedly. A 53-year-old female complained of headache, nausea, and dizziness. CT and MR imaging revealed a cystic mass filling the right sphenoid sinus. The cystic lesion was evacuated through the transnasal approach. She was doing well postoperatively and has been asymptomatic. A 39-year-old male complained of headache, vomiting, and right visual disturbance. CT and MR imaging demonstrated a homogeneous mass occupying the sphenoid sinus. Sphenoidotomy exposed the cyst extending superiorly into the anterior cranial fossa. He recovered from the visual disturbances and has been asymptomatic since. MR imaging provides confirmation of the diagnosis of sphenoethmoidal mucocele and is important for preoperative evaluation.     

Trochlear nerve enhancement on three-dimensional magnetic resonance imaging in Fisher syndrome

PURPOSE: To examine the lesion associated with external ophthalmoplegia in Fisher syndrome using three-dimensional magnetic resonance imaging (3-D MRI). METHOD: Case report. A 65-year-old woman with Fisher syndrome was investigated by gadolinium-enhanced 3-D MRI. RESULT: The extramedullary portion of the left trochlear nerve was enhanced. CONCLUSION: Contrast-enhanced 3-D MRI revealed that the lesion responsible for the external ophthalmoplegia in Fisher syndrome is located in the extramedullary portion of the trochlear nerve.     

Radiotherapy in the treatment of solitary plasmacytoma

Solitary plasmacytoma of bone (SPB) and extramedullary plasmacytoma (EMP) are rare. High local control rates are reported with radiotherapy, although the optimal dose and extent of radiotherapy portals remains controversial. Between 1983 and 1993, 30 patients with solitary plasmacytoma were seen at the Regional Cancer Centre, Trivandrum, India. 23 patients had SPB and seven EMP. The mean age was 52 years and the male to female ratio 3.2:1. Diagnosis of SPB was confirmed by biopsy in 16 patients and tumour excision in seven. 20 patients received megavoltage radiotherapy to the bone lesion with limited margins, and one received chemotherapy. Two patients who underwent complete tumour excision received no further treatment. All seven patients with EMP received megavoltage radiotherapy, four following biopsy and three after tumour excision. Local control was achieved in all patients with SPB. Nine progressed to multiple myeloma and one developed a solitary plasmacytoma in another bone. Six patients with EMP achieved local control. Three later progressed to multiple myeloma and one had local relapse. Median time to relapse was 28 months in SPB and 30 months in EMP. 5-year overall survival rates were 82% and 57% for patients with SPB and EMP, respectively. The corresponding progression free survival rates were 55% and 50%, respectively. Age, sex, site of tumour, serum M protein and haemoglobin levels did not significantly influence progression free survival. The extent of surgery, radiotherapy dose or time to relapse were not significant prognostic factors. Radiotherapy appears to be an effective modality of treatment of solitary plasmacytoma. No dose-response relationship is observed, and high local control rates are achieved with limited portals. Progression to multiple myeloma is the commonest pattern of failure, although no prognostic factors for progression are identified. The role of chemotherapy in preventing disease progression needs further evaluation.     

Colloid cysts of the third ventricle --report of one operated and one autopsied case (author's transl)

Colloid cysts of the third ventricle are considered as a relatively rate benign tumor, consituting approximately 0.5% of the whole brain tumors, but the surgical cases have rarely been reported in Japan. In this paper, two cases of colloid cyst are reported and the pathogenesis, the importance of the clinical diagnosis and dramatic results after complete extirpation was emphasized. In Case 1, a 29 year-old right handed male, who showed definite symptoms of increased intracranial pressure without lateralizing signs, was diagnosed as colloid cyst of the third ventricle, and operated on with dissecting microscope on April 20, 1973. The colloid cyst was totally removed and postoperatively V-A shunt was performed for adhesive arachnoiditis. Patient did well after surgery and has returned to the previous work. In Case 2, a 41 year-old male, who suffered from sudden onset of severe headache and bouts of frequent vomiting, was admitted to Tokyo Wome's Medical College Hospital on October 11, 1974. Right cerebral angiography revealed findings of increased intracranial pressure and third ventricle tumor was suspected. On October 13, progressively disturbed consciousness occurred. Immediate ventricular tap and continuous ventricular drainage could not regain consciousness and patient expired on October 30. In necropsy, colloid cyst to the third ventricle was found. As far as these are concerned, these cysts might be reasonably said as ventricular epithelial origin in view of the operative and histological findings. Some emphasis was also made in terms of dissecting microscope in removing this tumor, completely preserving the ventricular walls as well as the terminal veins under magnification.     

Magnetic resonance imaging of a mediastinal ependymoma

Ependymomas typically occur in the brain and spinal cord, and represent 5% and 16% of primary intracranial and spinal cord tumors respectively. Ependymomas arising outside the central nervous systems are uncommon and when they do occur are usually seen in the sacrococcygeal region. Other reported sites include the pelvis, spinal nerve roots, and lung. An extraspinal mediastinal ependymoma is a rare occurrence, with only two previously reported cases to our knowledge. In both instances, the lesion arose in the posterior mediastinum, and were imaged with either chest radiographs alone or chest radiographs and computed tomography. In our case, plain radiography was followed by magnetic resonance imaging. This report documents the clinical, pathological, and magnetic resonance imaging findings in a case of a posterior mediastinal ependymoma. Primary mediastinal ependymomas are rare lesions that may be considered in the differential diagnosis of a posterior mediastinal mass.     

Rosai-Dorfman disease presenting as a meningioma

This report documents an unusal case of extranodal sinus histiocytosis (Rosai-Dorfman disease) which produced a solitary intracranial mass lesion that was diagnosed clinically and radiologically as a meningioma. Morphological features have been described and literature extensively reviewed.     

Primary extramedullary esophageal plasmacytoma: First case report

Solitary extramedullary plasmacytomas are rare tumors, reported to occur most commonly in the upper respiratory passages, but which also are rarely seen in the middle and lower gastrointestinal system. This report documents the first primary plasmacytoma of the esophagus. A 67-year-old Caucasian man complained of dysphagia and weight loss. Preoperative diagnosis, based on functional inquiry, radiology, and biopsy, was undifferentiated carcinoma involving the lower third of esophagus. The patient was treated by esophagogastrectomy. The pathology specimen revealed the typical histology of plasmacytoma. The extramedullary nature of the tumor was established postoperatively on the basis of the following criteria: 1) Absence of Bence-Jones proteinurea; 2) normal serum electrophoresis; 3) normal bone marrow biopsy; and 4) absence of distant metastases on liver scan and bone survey.     

Insertion of a covered self-expandable esophageal metal stent for a malignant tracheoesophageal fistula in a patient with Zenker''s diverticulum

A 50-year-old woman presented with sensorimotor polyneuropathy in the lower limbs. Biopsy of the muscle revealed neurogenic changes, and electrophysiological study and pathology of the biopsied nerve indicated myelinoaxonal degeneration. Her symptoms responded to neither plasmapheresis nor prednisolone. Pelvic radiography disclosed an osteosclerotic lesion that was subsequently diagnosed as IgA-lambda plasmacytoma by biopsy. Radiation therapy for this solitary osteosclerotic myeloma improved the patient's polyneuropathy. In Japan, reports of cases of polyneuropathy associated with osteosclerotic myeloma without multiorgan disorders as in our case have not previously been described; thus, such cases may be misdiagnosed. Therefore, in patients with polyneuropathy of unknown etiology, a systemic bone survey and repeated searches for M-protein are important. Treatment of bone lesions in patients with solitary osteosclerotic myeloma may improve the associated polyneuropathy.     

High and low density water and resting, active and transformed cells

Melanoma is prone to spread to the brain and is the third most common source of intracranial metastasis. Patients usually present with signs and symptoms of increased intracranial pressure, a new focal neurologic deficit, or seizures. Contrasted magnetic resonance imaging (MRI) is the single most valuable imaging modality. Surgical therapy is the appropriate choice for single lesions that are accessible, especially if they are causing significant mass effect or are located in the posterior fossa. Patients with several intracranial metastases who undergo resection of all lesions may have a similar prognosis to those with single resected lesion. Stereotactic radiosurgery appears to provide good local control of small lesions. External beam radiotherapy may provide some benefit to patients, and is often used in conjunction with surgery or stereotactic radiosurgery. To date, chemotherapy has been limited because of chemo-resistance and drug delivery issues. Future directions for treatment may include local sustained delivery of either chemotherapy or immunoregulatory molecules.     

Multiple myeloma presenting as parasellar syndrome and cranial nerve palsies

Cranial and intracranial locations have been rarely reported in multiple myeloma. Their occurrence as a harbinger of multiple myeloma seems to have a particular significance. In this report, we discuss a case of multiple myeloma presenting as parasellar syndrome and cranial nerve palsies. A 75-year-old woman was admitted to the hospital in June, 1994, with a 3-month history of headache and a 3-week history of diplopia and photophobia. Physical examination revealed right third, fourth and sixth cranial nerve palsies. MRI scan demonstrated a homogeneous, voluminous mass, isointense in T1-weighted images with the cerebral parenchyma and hyperintense in T2-weighted images, occupying the sphenoid sinus and extending within the sella turcica and right cavernous sinus. Lying above the mass and apparently separated from it by a thin rim of hypointensity was a normal pituitary gland. X rays revealed destructive changes of the sella turcica. A minimal disturbance of endocrine function together with a radiologically abnormal pituitary fossa indicated that the primary lesion might lie outside the pituitary fossa. A diagnosis of IgG-kappa type multiple myeloma was made by pertinent laboratory studies. She received local radiation to the intracranial mass (50 Gy) and conventional chemotherapy. Sixteen months after the therapy she is in good health.     

Benign solitary fibrous tumour of the pre-sacral space: MRI findings

Benign solitary fibrous tumour, a rare mesenchymal tumour of adults, usually arises from the pleura. Only a few cases have been reported in the retroperitoneum and, to our knowledge, there has been no report of its imaging features. We describe the MRI features of benign solitary fibrous tumour arising from the pre-sacral space.     

A case of thoracic meningioma presenting paraplegia at 4.5 years after removal of a falx meningioma

A case of a thoracic meningioma presenting paraplegia 4.5 years after removal of a falx meningioma is reported. A 73-year-old woman, complaining of diplopia, was admitted to our department. Neurological examination revealed right abducens palsy. CT demonstrated a well-enhanced right frontal mass beneath the falx. The mass was totally removed under right frontal craniotomy. Its histology was transitional meningioma with rich fibroblasts. 4.5 years after craniotomy, she complained of progressing gait disturbance and nocturnal leg pain. Neurological examination revealed paraplegia, complete loss of leg sensation, loss of patellar and ankle reflex, bilateral positive Babinski reflex and urinary disturbance. Rectal function and anal reflex were preserved. Thoracic MRI demonstrated an intradural extramedullary mass which was well enhanced with Gd-DTPA at Th6-7. Under laminectomy, the mass was totally removed. Its histology was transitional meningioma with rich psammoma bodies and whirl formations. 4 months after removal, her palsy and sensory loss were almost completely recovered. We were able to find 15 cases of combined intracranial and spinal meningiomas in the literature. A young woman of neurofibromatosis suffered from tentorial, intraventricular and C1-2 meningiomas. Of 15 cases without neurofibromatosis including our case, 4 cases were of young boys and 11 cases were of women. Their initial symptoms originated from intracranial meningiomas in 8 cases. Multiple intracranial meningiomas were revealed in only 4 cases. In 9 cases, one case presented a combination of one intracranial meningioma and one spinal meningioma. Histology of intracranial meningioma was almost the same that of spinal meningioma in almost half of the 10 cases. These findings suggest the multi sentricity theory of multiple meningiomas originating in other neuroaxial compartments. Severe spinal dysfunction was recovered after removal in our case. Rectal function and anal reflex were preserved. These anorectal findings suggest that spinal dysfunction is either complete or incomplete. Motor evoked potentials are hopeful tools which can select reversible spinal motor dysfunctions.