Extramedullary Mass Research Articles

Hemangioma of spinal nerve root.

Hemangiomas of the spinal nerve roots are rare. Only 12 cases have been reported in the literature, all since 1965. These lesions occurred in adults, primarily in males, and were located in the cauda equina. Of the 12 lesions, eight were cavernous hemangiomas. The authors report here the clinicopathological features of 10 cases of hemangioma of the spinal nerve root, nine of which were of the capillary type. The group included six men and four women who ranged in age from 40 to 62 years. The majority of patients presented with pain and weakness. One patient also experienced worsening of pain during menses. In all cases, gadolinium-enhanced magnetic resonance imaging or computerized tomography myelography demonstrated a discrete intraspinal extramedullary mass. Administration of contrast agent resulted in uniform, intense enhancement. A gross-total resection was achieved in all but one case; in most cases, it was necessary to sacrifice the parent nerve root. Preoperative symptoms and signs improved in nine cases. On histological examination, all but one tumor, a spindle-cell hemangioendothelioma, were shown to be ordinary capillary hemangiomas. Two lesions exhibited a cavernous component, and five showed a partly solid growth pattern resembling juvenile capillary hemangioma. In all cases, the relationship with the nerve root was histologically confirmed. The tumor-nerve relation varied. The intact nerve was displaced by the epineurial mass in three cases. The fascicles appeared separated in six cases, and individual nerve fibers were seen throughout one lesion with endoneurial involvement. . Hemangiomas of spinal nerve roots pose a challenging diagnostic problem. Knowledge of their existence is relevant in that they may mimic tumors and are amenable to surgical treatment.

Intrathecal gadolinium-enhanced MR myelography and cisternography: a pilot study in human patients.

This study was designed to evaluate the safety, MR imaging characteristics, and clinical response to intrathecal gadopentetate dimeglumine (gadolinium) administration in human patients. Eleven adult patients were included in this prospective study. Via lumbar puncture, a single dose of either 0.2 ml, 0.5 ml, or 1.0 ml of gadolinium (500 mmol/l) mixed with 5 ml of previously removed CSF was slowly injected into the lumbar subarachnoid space. Immediate and delayed MR imaging were subsequently carried out using a 1.0-T magnet. No patient manifested gross behavioral changes, neurologic alterations, or seizure activity. The intrathecal gadolinium-enhanced MR myelography revealed disk herniation (n = 4), posttraumatic spinal stenosis (n = 3), postsurgical noncommunicating cyst (n = 1), myelitis (n = 1), intradural extramedullary mass formation (n = 1), and intradural vascular malformation (n = 1). This pilot study shows the relative safety and feasibility of low-dose intrathecal gadolinium administration. The potential clinical applications include the evaluation of obstructions and communications of the subarachnoid space, spontaneous or traumatic CSF leaks, and CSF dynamics. Additional animal and human studies must be performed to further evaluate the long-term safety and to prove the clinical applications of this procedure in a larger number of subjects.

Magnetic resonance imaging of the cervical spine in 27 dogs.

The cervical spine of 27 dogs with cervical pain or cervical myelopathy was evaluated using magnetic resonance imaging (MRI). Spin echo T1, T2, and post-contrast T1 weighted imaging sequences were obtained with a 0.5 Tesla magnet in 5 dogs and a 1.5 Tesla magnet in the remaining 22 dogs. MRI provided for visualization of the entire cervical spine including the vertebral bodies, intervertebral discs, vertebral canal, and spinal cord. Disorders noted included intervertebral disc degeneration and/or protrusion (12 dogs), intradural extramedullary mass lesions (3 dogs), intradural and extradural nerve root tumors (3 dogs), hydromyelia/syringomyelia (1 dog), intramedullary ring enhancing lesions (1 dog), extradural synovial cysts (1 dog), and extradural compressive lesions (3 dogs). The MRI findings were consistent with surgical findings in 18 dogs that underwent surgery. Magnetic resonance imaging provided a safe, useful non-invasive method of evaluating the cervical spinal cord.

Extramedullary paraspinal hematopoiesis in thalassemia: CT and MRI evaluation

We present a comparative CT and MRI study of the paraspinal extramedullary hematopoiesis in 32 thalassemic patients. The patients were classified into four groups according to the MRI and CT imaging findings. Active recent extramedullary paraspinal hematopoietic masses show soft tissue behavior in both CT and MRI. Older inactive masses reveal iron deposition or fatty replacement. Combined imaging findings of paraspinal extramedullary hematopoiesis revealed the phase of its evolution and the correct diagnosis.

Sinus histiocytosis (Destombes-Rosai-Dorfman disease) revealed by paraplegia

Sinus histiocytosis with massive cervical lymphadenopathy (Rosai-Dorfman disease) is a non-neoplastic lymphoproliferative disorder. Extranodal involvement, especially of the nervous system, is unusual. We report a case revealed by neurological symptoms. A 10-year-old girl presented with paraparesis due to a dural extramedullary mass on magnetic resonance imaging. Massive cervical lymphadenopathy appeared secondarily. Radiological investigations showed mediastinal, paranasal sinus and lower eyelid involvement. The diagnosis of Rosai-Dorfman disease was established histologically and by immunohistochemical studies of nodal lesions by the demonstration of characteristic sinus histiocytosis with sheets of S-100 protein and CD-68 positive large histiocytes displaying lymphocyte phagocytosis. A dramatic response occurred with complete resolution of all clinical findings after treatment with corticosteroids and etoposide, although neurological lesions were unchanged on magnetic resonance imaging. Despite its rarity, this case underlines the unknown pathogenesis of this disease (immune dysfunction?) and the difficulties of treatment (choice of chemotherapeutic agents, duration).

Imaging of spinal intradural arachnoid cysts: MRI, myelography and CT.

Imaging studies in eight patients with surgically-confirmed spinal arachnoid cysts were analyzed retrospectively. All patients had preoperative MRI of the spine and seven preoperative myelography with postmyelographic CT. In all cases the correct diagnosis could be made preoperatively on the basis solely of MRI. The diagnosis could also be established from myelography and postmyelographic CT in six of the seven cases. In one case myelography and CT simply demonstrated an intradural extramedullary mass.

Intradural Spinal Hibernoma: Case Report

Background Hibernoma is a rare, benign tumor arising from vestigial remnants of brown adipose tissue. It usually affects muscle and subcutaneous tissue and is asymptomatic and slow growing. Methods A review of the worldwide literature contains about 110 cases of hibernomas. However, only two cases (including our case) of hibernoma located in the intradural spine have been reported. We present an extremely rare case of intradural extramedullary spinal hibernoma and summarize the magnetic resonance imaging (MRI) findings of the lesion. Case Description A 35-year-old woman suffered from motor weakness and numbness of the left upper extremity. Cervical spinal MRI revealed an intradural extramedullary mass at C7. She underwent surgery via the posterior approach and the lesion was completely removed. Histopathologic examination showed multivacuolated cells with centrally placed nuclei, and the diagnosis of hibernoma was made. Conclusions Because the CT and MRI findings of hibernoma are almost same as those of other lipomatous tumors, a surgical specimen of this tumor is necessary to establish the correct diagnosis. Early diagnosis and total resection are necessary as some cases showed malignant features or rapid growth. Etiologically, the tumor may have developed by the ectopic growth or migration of adipose tissue.

Prognostic value of cytogenetics in multiple myeloma.

Karyotypic studies of bone marrow were conducted in 79 previously untreated patients with multiple myeloma who received a standard programme of chemotherapy. An abnormal karyotype was observed in 46% of patients, virtually all showing multiple abnormalities consistent with a long period of preclinical clonal evolution. Patients with an abnormal pattern showed various aberrations with hyperdiploidy in 64%, pseudodiploidy in 5% and hypodiploidy in 31%. The number of chromosomes affected ranged from two to 19 (median 10), with at least one trisomy in 83%, one monosomy in 75%, and one translocation in 42% of patients. Lymphoma-like karyotypes were present in 17% of patients with an abnormality but were not associated with atypical clinical features, such as an extramedullary mass, leukaemia, or increased serum lactate dehydrogenase. Monosomy or deletion of chromosome 13 was present in 47% of patients with an abnormal pattern, who lived for a shorter duration (median 10 months) than patients with other abnormalities (median 34 months) or with diploidy (median 35 months). The cause of the short survival of patients with monosomy or deletion of chromosome 13 was not clear, but further studies on the relationship with specific oncogenes are indicated.

The effect of intrauterine growth restriction upon fetal and postnatal hepatic glucose transporter and glucokinase proteins.

Employing immunohistochemical and Western blot analyses, we investigated the cellular localization (22-d fetal and 14-d postnatal animals) and concentrations (22-d fetal to 21-d postnatal animals) of rat hepatic glucose transporters (Glut 1 and Glut 2) and glucokinase in response to development and uteroplacental insufficiency with IUGR. Glut 1, the predominant fetal hematopoietic cellular isoform, persisted in postnatal hematopoietic islands and was noted minimally in fetal hepatic cellular membranes. A approximately 40% extrauterine decline in Glut 1 levels paralleled the decline in hematopoietic cells. IUGR increased the fetal hepatic Glut 1 levels in parallel with an expanded hematopoietic cell mass (p < 0.05). In contrast, IUGR failed to alter the 2-fold increase in extrauterine Glut 2 concentrations (1-7-d postnatal animals), the isoform found in fetal and postnatal hepatocytic cell membranes. Glucokinase, the nuclear enzyme, increased 25% postnatally. IUGR caused a 16% increase in fetal glucokinase levels and a approximately 25% decline at postnatal d 1 (p < 0.05) without a comparable change in the hepatocytic cell number (92 +/- 6 versus 86 +/- 4). We conclude that hepatic Glut 1 concentrations reflect the extramedullary hematopoietic cellular mass, whereas extrauterine Glut 2 changes herald the need for enhanced flexibility in hepatocytic glucose transport with the initiation of food ingestion. The age-related alteration along with the IUGR-induced compensatory changes in the nuclear-mitochondrial glucokinase levels attributes a critical role for this enzyme in perinatal hepatocytic glucose homeostasis.

Clinical and Hematological Response to Hydroxyurea in a Patient with Hb Leporbp-Thalassemia

The possibility of increasing Hb F in vivo using drugs like 5-azacy-tidine, hydroxyurea, and butyrate has been established. However, in many cases this does not entail an increase in total hemoglobin. We report on a patient with Hb Lepore/β-thalassemia being treated with hydroxyurea (30 mg/Kg/day) because of the presence of erythroid extra-medullary masses with severe neurological abnormalities. During therapy the patient showed a remarkable improvement in neurological signs due to the reduction in extra-medullary masses, a significant increase in both total hemoglobin (from 5.8 to 9.7 g/dl) and Hb F (from 4.9 g/dl to 9.1 g/dl). The marked improvement in hemoglobin level in our patient with Hb Lepore/β-thalassemia suggests γ-globin gene activation due to the DNA structure determined by the crossover event.

Granulocytic Sarcomas: Clinical, Diagnostic and Therapeutical Aspects

Granulocytic sarcomas (GS) are extramedullary tumor masses of immature myeloid cells most frequently associated with acute myeloblastic leukemia. We report our diagnostic, clinic and therapeutic approaches in the treatment of 6 patients affected by GS who presented with different localizations and symptoms: mediastinal mass with chest pain, rectal tumor with bowel occlusion, bladder mass with acute kidney failure, quadriceps tumor with pain, vertebral localization with pain and bowel mass with pain, respectively. The correct diagnosis of GS by bone biopsy, the immunohistological evaluation of the tumor masses, the prompt use of active drugs in the first line therapy schedule as for acute myeloblastic leukemia are the parameters for the achievement of the long-term remission.

Retroperitoneal Chloroma Presenting with Uremia, Bilateral Ureteral Obstruction and Encasement of the Great Vessels

Chloroma or granulocytic sarcoma is an unusual tumor commonly associated with myelogenous and rarely with lymphocytic types of leukemia. I t is named for a greenish hue on gross cut section. Chloromas are soft tissue extramedullary masses of leukemic progenitor cells, which may predate detectable involvement of bone marrow by leukemia. Granulocytic sarcoma has been reported a t almost every anatomical site, including skin, brain, head and neck, thorax, abdomen and testicles. To our knowledge only 1 previous case of bilateral obstruction of the ureters by chloroma has been reported, presenting as uremia. We report on a man who presented with flank pain and uremia 18 months after bone marrow transplantation as a result of a chloromatous retroperitoneal mass encroaching on the ureters.

Are "early" and "late" T-acute lymphoblastic leukemias different diseases? A single center study of 34 patients.

Clinical and biological parameters were retrospectively reviewed in 34 cases of T-lineage acute lymphoblastic leukemia (T-ALL), classified as "early" (20 cases) or "late" (14 cases) subgroups, according to the degree of blast cell differentiation, assessed by immunophenotyping. In "early" T-ALL, age, co-expression of "immature" (CD34 and HLA-Dr) or myeloid (My+) antigens, proliferative activity (as evaluated by Ki67 monoclonal antibody), and expression of the "multidrug-resistance" (MDR) phenotype (as determined by C-219 monoclonal antibody) were significantly higher, while WBC count and expression of CDl0 were significantly lower, than in "late" T-ALL. Furthermore, although no statistically significant difference was found between the two groups, "late" T-ALL more frequently displayed a greater extramedullary tumor mass ("lymphoma-like" syndrome), LI FAB morphology and a normal karyotype. A single patient, with "late" T-ALL, also showed positivity for TCR gamma/delta chains, specific monoclonal antibodies. On the whole, 30 patients (88.2%) achieved complete remission: 16(80%) were "early" and 14(100%) "late" T-ALL. No statistical difference was found between the two groups with respect to disease free survival (42% vs 54% at six years), whereas median overall survival was significantly shorter in "early" T-ALL (23 months vs median not yet reached at six years for "late" T-ALL, p < 0.05). We conclude that "early" and "late" T-ALL show clinical and biological differences, that could perhaps justify differential therapeutic approaches.

Symptomatic Intradural Adrenal Adenoma of the Spinal Nerve Root

The second and third cases of an adrenal adenoma involving the intraspinal space, a rare location for a lesion that occurs rather commonly at a variety of extraneural sites, are reported. The first patient, a 16-year-old girl with a 3-month history of radicular pain, was found to have an intradural, extramedullary mass arising on a spinal nerve root. Its resection resulted in cure. The second patient, a 63-year-old woman with a 10-month history of low back pain with radiation to both lower extremities, had a similar mass arising from one of the nerve roots of the cauda equina. In both cases, ultrastructural examination of these demarcated, oncocytic, lipid-rich lesions demonstrated abundant smooth endoplasmic reticulum and mitochondria with tubular cristae. The presence of steroidogenic enzymes was noted on immunohistochemical examination. The literature is reviewed, as is the differential diagnosis of these rare lesions.

Retrocrural masses of extramedullary hemopoiesis in beta-thalassemia

A case of extramedullary hemopoiesis in an 18-yr-old girl suffering from beta-thalassemia is reported. Extramedullary hemopoietic masses were localized apart from the paraspinal region in the retrocrural areas as well as the presacral region associated with massive destruction of the sacrum. The importance of recognition of hemopoietic masses in these regions in conditions other than hereditary hemolytic anaemias is briefly discussed.

Intraoperative Spinal Sonography

The uses of intraoperative spinal sonography are reviewed, including localization of lesions and monitoring of surgical correction. The sonographic appearance of intramedullary and extramedullary masses and cysts are reviewed and illustrated. The use of sonography in the surgical treatment of spinal fractures, herniated disks, and spinal stenosis is also discussed.

Exophytic syrinx, an extreme form of syringomyelia: CT, myelographic, and MR imaging features.

Magnetic resonance (MR) imaging and computed tomography (CT) were used to study severe syringomyelia, in which the syrinx was so eccentric relative to the center of the spinal cord that at initial examination it appeared to be an exophytic extramedullary mass, in five of six patients with type 2 Arnold-Chiari malformation and one patient with an intramedullary tumor. Sagittal and axial images were routinely obtained at 1.5 T; CT was performed after intrathecal injection of contrast material. On sagittal MR images, the exophytic component of the syrinx typically displaced the spinal cord peripherally and mimicked an extramedullary intradural lesion such as an arachnoid cyst or meningioma. On axial MR images, the gradual appearance and disappearance of this component could be traced as it compressed the spinal cord, which had a signet-ring appearance at the equator of the syrinx. It is concluded that both MR imaging and postmyelographic CT can reveal the characteristics of this lesion on axial images, but MR imaging is superior because it enables direct sagittal imaging.

Spinal neurenteric cyst in association with syringomyelia: Case report

A case of spinal neurenteric cyst in association with Klippel-Feil deformity, spinal lipoma, and syringomyelia is presented. Neuroradiological evaluation was performed by means of magnetic resonance imaging of the spine and a computed tomography myelograph. The diagnosis of neurenteric cyst was established by histological and immunohistochemical studies. The pathogenesis of syringomyelia associated with an extramedullary mass lesion is discussed.

Thoracic intradural arachnoid cyst: possible pitfalls with myelo-CT and MR

A thoracic intradural arachnoid cyst presenting as an intradural extramedullary mass highly suggestive of psammoma on myelogram and myelo-CT is reported in a 34-year-old female. High densities of the cyst were related to collection of contrast media within the cyst. However MR examination of the thoracic spinal cord including sagittal T1 (without and with contrast) and T2 studies failed to demonstrate the mass. Lack of MR changes were related on one hand to the small size of the cyst and to the absence of mass effect on the spinal cord, and on the other hand to a CSF-like signal of the contents of the cyst. Only combination of myelography, myelo-CT and MR allows precise diagnosis of small intradural arachnoid cyst; however MR is the method of choice for evaluation of large intradural subarachnoid cysts.

Craniospinal Enterogenous Cyst

An enterogenous cyst of the craniocervical region producing anterior medullary compression is reported. The patient presented with quadriparesis with greater involvement of the upper limbs. Computed tomography showed a high attenuation intradural extramedullary mass located anteriorly. Magnetic resonance imaging demonstrated a high intensity mass extending from the pontomedullary junction to the second cervical vertebra, flattening the medulla and upper cervical cord. Complete recovery ensued following total excision of the cyst.