Two cases of primary osteolytic intraosseous meningioma of the skull metastasizing to whole skull and the spine

We report here two cases of primary intraosseous meningioma with aggressive behavior. A 68-year-old man presented with a one year history of a soft, enlarging mass in the right parietal region. Magnetic resonance image (MRI) revealed a 6 cm sized, heterogeneously-enhancing, bony expansile mass in the right parietal bone, and computed tomograph (CT) showed a bony, destructive lesion. The tumor, including the surrounding normal bone, was totally resected. Dural invasion was not apparent. Diagnosis was atypical meningioma, which extensively metastasized within the skull one year later. A 74-year-old woman presented with a 5-month history of a soft mass on the left frontal area. MRI revealed a 4 cm sized, multilobulated, strongly-enhancing lesion on the left frontal bone, and CT showed a destructive lesion. The mass was adhered tightly to the scalp and dura mater. The lesion was totally removed. Biopsy showed a papillary meningioma. The patient refused adjuvant radiation therapy and later underwent two reoperations for recurred lesions, at 19 and at 45 months postoperative. The patient experienced back pain 5 years later, and MRI showed an osteolytic lesion on the 11th thoracic vertebra. After her operation, a metastatic papillary meningioma was diagnosed. These osteolytic intraosseous meningiomas had atypical/malignant pathologies, which metastasized to whole skull and the spine.     

Primary Osteolytic Intraosseous Atypical Meningioma with Soft Tissue and Dural Invasion: Report of a Case and Review of Literatures

Primary intraosseous meningioma is a rare tumor, and atypical pathologic components both osteolytic lesion and dura and soft tissue invasion is extremely rare. A 65-year-old woman presented with a 5-month history of a soft mass on the right frontal area. MR imaging revealed a 4 cm sized, multilobulated, strongly-enhancing lesion on the right frontal bone, and CT showed a destructive skull lesion. The mass was adhered tightly to the scalp and dura mater, and it extended to some part of the outer and inner dural layers without brain invasion. The extradural mass and soft tissue mass were totally removed simultaneously and we reconstructed the calvarial defect with artificial bone material. The pathological study revealed an atypical meningioma as World Health Organization grade II. Six months after the operation, brain MR imaging showed that not found recurrence in both cranial and spinal lesion. Here, we report a case of primary osteolytic intraosseous atypical meningioma with soft tissue and dural invasion.     

Destructive Radiologic Development of Intravascular Papillary Endothelial Hyperplasia on Skull Bone

Intravascular papillary endothelial hyperplasia (IPEH) is a rare vascular benign lesion that rarely involves the central nervous system with or without skull invasion. We report a rare case of IPEH on the skull bone, which displayed destructive radiologic development associated with hemorrhage. A 14-year-old male presented with an incidentally detected a small enhancing, left frontal osteolytic lesion. Previously, he underwent operation and received adjuvant chemoradiation therapy for cerebellar medulloblastoma. Follow-up magnetic resonance imaging revealed a left frontal bone lesion, which expanded to an approximately 2 cm-sized well-circumscribed osteolytic lesion associated with hemorrhage for 20 months. Frontal craniectomy and cranioplasty were performed. Destructive change was detected on the inner table and diploic space of the skull. The mass had a cystic feature with hemorrhagic content without dural attachment. Pathologic examination showed the capsule consisted of parallel collagen lamellae representing a vascular wall, vascular lumen, which was pathognomonic for IPEH. Immunohistochemical staining revealed that the capsule was positive for CD34 and factor VIII, which favor the final diagnosis of IPEH. This was the first case of intracalvarial IPEH.     

A case of multiple myeloma showing intracranial hypertension due to large cranial mass lesions

It is well known that the case of multiple myeloma shows punched-out lesions of the cranium without intracranial hypertension. In this paper a case of multiple myeloma is reported showing intracranial hypertension due to a large tumor that developed in the left parietal bone. There are only 13 case reports about cranial mass lesion of multiple myeloma since 1928. A 52 year-old female was admitted to Iwate Prefectural Isawa Hospital suffering from headache, nausea and vomiting. She had been already diagnosed as multiple myeloma and treated with chemotherapy using Cyclophosphamide, Melphalan and Prednisolone for 2 years. On admission, a large subcutaneous mass was presented on the left parietal region. Craniogram revealed large osteolytic lesion of the left parietal bone and 3 punched-out lesions of the frontal bone. CT scan revealed a large mass lesion in the left epidural space, diploe and subcutaneous space. Angiography showed avascular area. Brain scintigram showed diffuse hot area. Other skeletal bones showed no abnormality. Laboratory examination revealed high concentration of gamma-globulin and high erythrocyte sedimentation rate. Electrophoresis showed high value of immunoglobulin G; immunoglobulin assay was as follows: IgG-6000 mg/dl, IgA-150 mg/dl, IgM-410 mg/dl, IgE-0 mg/dl. Serum electrolytes were within normal limits. Urine didn't include Bence-Jones protein. The patient was diagnosed as multiple myeloma suffering from intracranial hypertension caused by large tumor which developed in the left parietal bone. On the operation, large tumor was existed in the epidural and subcutaneous space invading into the diploe but without infiltration into the dura mater or cerebral cortex.(ABSTRACT TRUNCATED AT 250 WORDS)     

Intravascular Papillary Endothelial Hyperplasia (Masson tumor) of the Skull : Case Report and Literature Review

A 10-year-old female patient presented with a rapidly growing nodular mass lesion on her right frontal area. On skull radiography and computed tomography (CT) imaging, this mass had a well-demarcated punch-out lesion with a transdiploic, exophytic soft tissue mass nodule on the frontal scalp. Magnetic resonance (MR) imaging revealed the presence of a 1.5×1.2×1 cm sized calvarial lesion. This lesion was hypointense on T1 and heterogenous hyperintense on T2 weighted MR images, and exhibited heterogeneous enhancement of the soft tissue filling the punch-out lesion after intravenous administration of gadolinium. En block removal of the tumor with resection of the rim of the normal bone was performed. The pathological diagnosis was intravascular papillary endothelial hyperplasia (IPEH). After surgery, no recurrence was found for 8 months. IPEH is a rare and benign reactive lesion usually found in thrombosed subcutaneous blood vessels. Involvement of skull bone is rare. In this article, we present a case of IPEH involving the calvarium, in a 10-year-old woman.     

Chondromyxoid fibroma of frontal bone: a case report and review of the literature

Chondromyxoid fibroma is an unusual benign tumor of cartilaginous tissues that may be confused with other some malign tumors. It is rarely seen in the skull. A 45-year-old female was admitted with painless bony swelling in the forehead. Computerized tomography demonstrated a well-defined expansive lesion with a sclerotic margin measuring approximately 3 cm in diameter in the right frontal bone. On MRI, T1-weighted images revealed a well circumscribed, lobulated and strongly enhancing lesion. On the T2-weighted images, the lesion showed high heterogeneous signal intensity. The patient underwent tumor excision with craniectomy, then acrylic cranioplasty. The histopathological diagnosis was chondromyxoid fibroma. There was no recurrence in a period of 22-months. In conclusion, chondromyxoid fibroma is a benign primary bone tumor that is located extremely rarely in the frontal bone. Accurate initial diagnosis of such tumors are important for appropriate treatment. En block surgical resection of the tumor is the cornerstone of treatment.     

A huge occipital osteoblastoma accompanied with aneurysmal bone cyst in the posterior cranial fossa

Osteoblastoma is an infrequent benign tumor and skull involvement is extremely rare. The occipital bone is much less frequently involved. We report an unusual case of a huge occipital osteoblastoma with aneurysmal bone cyst in the posterior cranial fossa of a 20-year-old young man. MRI scan and CT three-dimensional reconstruction revealed that the tumor (approximately 8.5 cm x 6 cm x 5 cm) occupied nearly half volume of the posterior cranial fossa. The cerebellum, the forth ventricle and brain stem were compressed remarkably and hydrocephalus was noted. The tumor mass was multiloculated cystic and highly vascular. Excessive bleeding occurred during operation and total removal was achieved. The patient was symptom-free and MRI scan revealed no evidence of recrudescence after a follow-up period of 18 months. Pathological examination revealed that the lesion was a benign osteoblastoma with secondary aneurysmal bone cyst (ABC). ABC constitutes a highly vascular lesion while complete surgical resection is often impeded by excessive intraoperative bleeding. Preoperative embolization may reduce the risk. Despite the benign nature of osteoblastoma, local recurrence after incomplete resection is not rare and malignant transformation during recurrence may occur even after adequate surgery. Our case confirms the findings of previous investigators that if osteoblastoma is removed completely, no recurrence is expected even without adjuvant irradiation and chemotherapy.     

Intraosseous meningioma mimicking osteosarcoma in an adolescent: a case report

Intaosseous meningiomas (IM) are the one of the less frequent benign tumors of the skull. The etiology of IM has not been cleared yet. The frontoparietal and orbital regions are the most common locations for IM. The average age for IM diagnosis is 50.5. A 16-year-old girl with a right frontoparietal mass was referred to our outpatient clinic. Cranial CT revealed a mass lesion which resulted in expansion in the right parietal and posterior frontal bone, having lytic and sclerotic regions inside with accompanying irregular cortex in inner and outer tables of the calvarium. Prediagnosis was osteosarcoma according to the imaging studies and after the performed biopsy and consecutive surgery, the lesion was diagnosed as IM. Though CT with bone windows is often useful, it is not always diagnostic. Biopsy plays an important role in calvarial vault lesions for planning the treatment. In our case, malignant criteria in radiology did not match the benign histology revealed. Radiological preoperative misdiagnosis of meningioma is possible.     

visual bone marrow mesenchymal stem cell transplantation in the repair of spinal cord injury

An important factor in improving functional recovery from spinal cord injury using stem cells is maximizing the number of transplanted cells at the lesion site. Here, we established a contusion model of spinal cord injury by dropping a weight onto the spinal cord at T7–8. Superparamagnetic iron oxide-labeled bone marrow mesenchymal stem cells were transplanted into the injured spinal cord via the subarachnoid space. An outer magnetic field was used to successfully guide the labeled cells to the lesion site. Prussian blue staining showed that more bone marrow mesenchymal stem cells reached the lesion site in these rats than in those without magnetic guidance or superparamagnetic iron oxide labeling, and immunofluorescence revealed a greater number of complete axons at the lesion site. Moreover, the Basso, Beattie and Bresnahan(BBB) locomotor rating scale scores were the highest in rats with superparamagnetic labeling and magnetic guidance. Our data confirm that superparamagnetic iron oxide nanoparticles effectively label bone marrow mesenchymal stem cells and impart sufficient magnetism to respond to the external magnetic field guides. More importantly, superparamagnetic iron oxide-labeled bone marrow mesenchymal stem cells can be dynamically and non-invasively tracked in vivo using magnetic resonance imaging. Superparamagnetic iron oxide labeling of bone marrow mesenchymal stem cells coupled with magnetic guidance offers a promising avenue for the clinical treatment of spinal cord injury.     

Pediatric giant cell granuloma of the temporal bone: a case report and brief review of the literature.

Giant cell granuloma of the skull base is a distinct rare clinicopathologic lesion, which progressively destroys the involved bone. It causes increased intracranial pressure and mass effect on the brain. Histologically it may mimic an aneurysmal bone cyst, giant cell tumour and the brown tumour of hyperparathyroidism. Although cited by some authors, the role of trauma in its aetiology is still considered controversial. Authors present an interesting case of a 12 year old Omani boy who sustained a cricket bat injury to his right temporal region from which he initially recovered but later on progressively developed vertigo, tinnitus, right hearing loss and a mild right facial weakness. Computed tomography (CT scan) and Magnetic resonance imaging (MR scan) revealed a large destructive lesion of the temporal bone. Microsurgical excision was curative. Interesting clinicoradiological findings are presented with a brief review of the literature.     

Massive osteolysis of the skull--case report and review of the literature

The authors describe a rare case of massive osteolysis of the skull, along with a brief review of the literature. A 11 year-old female was admitted with a complaint of a painless depression in the right parietal region, which had been slowly enlarging for several years. Skull x-ray films revealed a radiolucent area with irregular and non-sclerotic margin in the right parietal bone. Plain CT showed an osteolytic lesion involving the outer table and diplo? with absence of new bone formation. On 99mTc scintigrams, there were no abnormal accumulation in the whole skeletal system including the skull. Blood examinations including serum calcium, phosphate, and alkaline phosphatase were entirely normal. In the differential diagnosis of lytic skull lesion, the long, inactive course without any corroborative local or systemic evidence of illness, and the absence of new bone formation on radiological examinations clinch the diagnosis of massive osteolysis.     

A therapeutic window for intravenous administration of autologous bone marrow after cerebral ischemia in adult rats

The primary objective of this study was to test the hypothesis that intravenous administration of autologous bone marrow cells could improve functional recovery after middle cerebral artery occlusion (MCAO) for 45 min in the rat and to determine specific time windows for efficacy. Mononuclear cells from autologous bone marrow were transfected with the LacZ reporter gene, and injected intravenously into rats at 3-72 h after induction of MCAO. Histological analysis of the ischemic lesion at 14 days after transplantation revealed reduced ischemic lesion volume. Lesion volume was 250+/-45 mm(3) (n=6) after MCAO without cell transplantation. Lesions were minimally detected by absence of 2,3,5-triphenyltetrazolium chloride (TTC) staining when bone marrow cells were infused 3 h after lesion induction. Lesions were clearly detected beginning with the 6-h postlesion group and became progressively larger at 12, 24 and 72 h (80+/-25, 140+/-18, and 180+/-22 mm(3), respectively; n=6 for each group). Transplanted LacZ(+) bone marrow cells accumulated extensively in and around the ischemic lesions, and immunohistochemistry suggests some neuronal and glial lineage differentiation. Behavioral testing (Morris water maze and Treadmill stress test) indicated greater functional recovery in the treated group. These findings suggest that early intervention with intravenous administration of autologous mononuclear cells from bone marrow can reduce lesion size in the MCAO model in the rat, and improve functional outcome.     

Comparative analysis of remyelinating potential of focal and intravenous administration of autologous bone marrow cells into the rat demyelinated spinal cord

The remyelinating potential of autologous bone marrow cells was studied after direct injection and following intravenous injection into rats with a demyelinated lesion in the spinal cord. Both focal and intravenous injections of acutely isolated mononuclear bone marrow cell fractions resulted in varying degrees of remyelination. Suspensions of bone marrow cells collected from the same rat were delivered at varied concentrations (10(2) to 10(5) for direct injection and 10(4) to 10(7) for i.v. injections). The lesions were examined histologically 3 weeks after transplantation. Light microscopic examination revealed remyelination in the dorsal funiculus with both injection protocols, but the extent of remyelination was proportional to the number of injected cells. To attain the same relative density of remyelination achieved by direct injection, intravenous administration of cells required delivery of substantially more cells (two orders of magnitude). However, the availability of autologous bone marrow cells in large number and the potential for systemically delivering cells to target lesion areas without neurosurgical intervention suggest the potential utility of intravenous cell delivery as a prospective therapeutic approach in demyelinating disease.     

Eosinophilic granuloma of the occipital bone presenting as intracranial venous hypertension.

Intracranial venous sinus thrombosis is an uncommon cause of pseudotumor cerebri. The diagnosis is often not confirmed on the rationale that treatment will not be altered. We report a case presenting a pseudotumor cerebri where the underlying pathology disclosed dural sinus thrombosis resulting from compression by an eosinophilic granuloma of the occipital bone. Routine CT of the head and Technetium-99m brain scan initially demonstrated neither tumor nor thrombosis. Plain skull x-rays subsequently revealed a lytic lesion of the occiput. When reinvestigated with CT using bone density windows the tumor was revealed. Excision of the tumor and a short course of cobalt therapy was curative. Special techniques in nuclear scanning, CT and MRI designed to improve the sensitivity for diagnosing venous sinus thrombosis are described. This case illustrates the importance of establishing a definitive diagnosis and shows the importance of pre-test consultation between clinicians and radiologists to ensure that specific investigative techniques are properly utilized.     

Sclerosing Leucoencephalopathy with Membranocystic Lesions of Adipose Tissue: A Contribution to the Pathology of Nasu Disease

A case of sclerosing leucoencephalopathy with membranocystic lesions of adipose tissue was reported. A 38-year-old Japanese housewife, whose parents were consanguineous, developed gradually neuropsychiatric symptoms characterized by euphoria, gait disturbance and urinary incontinence, followed by spastic tetraplegia with epileptic convulsions and died eight years later. Neuropathologically a form of sudanophilic leucodystrophy with a prominent fibrillary gliosis (dissociation glio-myelinique) associated with axonal spheroids and calcospherite depositions was revealed. A peculiar membranocystic lesion of adipose tissue was recognized not only in the bone marrow but also in other areas. However, no apparent bone pathology was observed except for a ring-like pleated lamellar structure. This case was thought to be classified as heterogeneous phenotype of membranous lipodystrophy (Nasu).     

Solitary costal plasmacytoma: a rare cause of subacute demyelinating polyradiculoneuropathy

INTRODUCTION: The solitary plasmacytoma (SP) is a non-frequent, localized variant of multiple myeloma with a single bone lesion and earlier appearance. Polyradiculoneuropathy is the most frequent neurological manifestation. We report the case of a young male who developed a subacute demyelinating polyradiculoneuropathy as the initial symptomatology of a costal SP, which constitutes an extremely infrequent association, given age and site of the lesion. CASE REPORT: A 32-year old, previously healthy, man presented with a one month history of progressive symmetrical paraparesis and paresthesias in feet. The neurological examination revealed 4/5 paraparesis, global arreflexia and hypopalesthesia in legs. Cerebrospinal fluid examination revealed elevated proteins (83 mg/dl) with normal cell count and glucose content. Investigations showed high levels of beta(2)-microglobulin (3 mg/l), and a monoclonal IgG lambda gammapathy. The chest X-ray and thoracic CT revealed an osteolytic lesion in the left third rib. Nerve conduction studies showed sensitive and motor polyneuropathy with secondary axonal degeneration. Bone marrow biopsy was normal. Second and third left ribs were excised, revealing a pathologic diagnosis of plasmacytoma. The patient became asymptomatic after corticosteriod administration lesion excision and local radiotherapy. Conclusions. Subacute demyelinating polyradiculoneuropathy can be due to multiple causes. SP can be one of its etiologies and occur at infrequent ages and localizations.     

Chondromyxoid fibroma of the anterior skull base invading the orbit in a pediatric patient: case report and review of the literature

Chondromyxoid fibroma (CMF) is a rare bone tumor of cartilaginous derivation representing less than 1% of all benign bone neoplasms. It is a slow-growing, locally destructive tumor that usually affects the metaphyseal region of long bones of patients in their second or third decade of life. Involvement of the skull base and orbit is extremely rare. We present the first case of histopathology-proven CMF originating in the greater sphenoid wing and extending into the orbit in a 14-year-old boy. He complained of pain and swelling of his left lateral orbit for 4 months and had 3-mm nonaxial proptosis. Magnetic resonance imaging of the orbit revealed a well-defined lesion in the lateral wall of the left orbit, hypointense on T1-weighted images, and heterogenous hyperintense on T2-weighted images and enhanced after contrast media injection. Through a left semicoronal skin incision, the mass was removed totally and the lateral orbital wall was reconstructed. The clinical manifestations, imaging findings, and surgical treatment of the lesion are discussed with a review of the literature.     

POEMS syndrome with plasmocytoma lytic bone lesion

INTRODUCTION: Crow-Fukase or Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, and Skin changes syndrome (POEMS) is a rare multisystemic affection with incompletely elucidated etiopathogenesis. CASE REPORT: We report a case of POEMS syndrome in a 48-year-old adult revealed four months before admission by areflexic flask tetraparesis prevalent on the lower limbs in connection with demyelinating and axonal CIDP "like" sensoriomotor neuropathy of the four limbs electroneuromyographically. The patient presented elevated protein level in the CSF with monoclonal standard IgG gammapathy associated with a narrow band lambda, suggesting POEMS syndrome. Further explorations revealed skin lesions with glomeruloid angiomas, edematous vasomotor disorders as well as erythrocyanose, hypogonadism, papillar edema and a lytic bone lesion of the left scapula. Radiotherapy was associated with corticosteroids and plasma exchanges. Outcome was good with resolution of the symptoms and stabilization of the neuropathy. DISCUSSION: POEMS syndrome is rare; the diagnosis is based on necessary criteria, the presence of a demyelinating and axonal polyneuropathy associated with an IgA or IgG monoclonal gammapathy, the light chain being almost entirely lambda, associated to other characteristic elements, in particular glomeruloid angiomas, endocrinopathy, sclerosing plasmocytoma which must be carefully required. Treatment is based on surgical cure or radiotherapy for bone lesion and non specific treatments such as corticosteroid therapy, plasma exchanges and IVIG.     

Primary diffuse leptomeningeal gliomatosis: an autopsy case and review of the literature

We report a case of primary diffuse leptomeningeal gliomatosis (PDLG) in a 76-year-old male presenting with confusion, dysarthria, diplopia, lumbal pain and headaches of recent onset. Neurological examination revealed nuchal rigidity and bilateral sixth cranial nerve palsy. The cerebrospinal fluid showed a marked hyperproteinorachia (4711 mg/L) and mild cytorachia (5-10 leucocytes/mm3) with a few atypical lymphoid cells. On admission, brain CT scan and MRI demonstrated diffuse and nodular leptomeningeal contrast enhancement predominant at the skull base and several osteolytic lesions in the right parietal bone. Extensive serological studies for infectious, autoimmune or neoplastic diseases were negative. The work-up diagnosis was neurosarcoidosis or multiple meningeal and osseous metastases of an unknown primary cancer. Surgical biopsy of the right parietal bone lesion showed only fibrous tissue with no evidence of tumour or inflammation. The patient was treated with high dose corticosteroids but its neurological status progressively worsened and he died of aspiration pneumonia 35 days after admission. Post-mortem examination revealed a PDLG, a rare fatal tumour with about 60 cases reported. PDGL is characterized by the diffusion of neoplastic glial cells throughout the leptomeninges without evidence of a primary intra-parenchymal lesion. Recognition of this rare brain tumour is important as recent reports suggest that radiotherapy and chemotherapy can improve patient survival.     

Rapidly enlarging chordoid meningioma with abundant mucin production

This 77‐year‐old woman with a rapidly enlarging chordoid meningioma first noticed a growing, non‐pulsatile, non‐painful soft mass in the left temporal region after a head trauma 2 years earlier. Neuroimaging showed a homogeneously enhanced osteolytic mass lesion in the left temporal bone. Surgery revealed an extradural tumor without significant adhesions. Histopathologically it was a meningioma with features reminiscent of chordoma. Most of the tumor cells contained mucin‐rich chordoid elements. Immunohistochemically, the lesion was positive for vimentin and epithelial membranous antigen; it was negative for cytokeratin and S‐100 protein. Based on these findings a diagnosis of chordoid meningioma was made. We posit that the rapid enlargement of the tumor over a relatively short period was attributable to its high mucin‐producing activity.