Diffuse calvarial meningioma: a case report

A rare case of a diffuse calvarial meningioma in a sixty-three year-old female is reported. The patient presented with headache, painful proptosis and chemosis of the left eye. Imaging showed that the frontoparietal calvarium on both sides and the left orbital roof were thickened. The thickened bone showed patchy rarefaction. In relationship to the thickened bone, there was an enhancing en-plaque meningioma. The tumour extended on to the superolateral aspect of the left orbit and resulted in proptosis. Excision of the left frontoparietal calvarium and the orbital tumour was performed. The management of diffuse calvarial meningioma is discussed and the relevant literature is briefly reviewed.     

Heterotopic ossification in psammomatous spinal meningioma: a diagnostic controversy

Heterotopic ossification (HO), a synonym for osseous metaplasia, is a pathological phenomenon, characterized by abnormal bone formation outside the skeletal system observed commonly in various neoplastic and non-neoplastic diseases. HO occurring in meningioma is exceptionally rare. We reportherein an unusual case of spinal meningioma containing numerous calcified psammoma bodies and extensive HO in a 75-year-old woman, who presented with progressive worsening bilateral lower limb weakness and numbness. The presence of remarkable bone formation within a meningioma is controversial among pathologists; while some regard them as psammomatous meningioma as the primary diagnosis, others prefer osteoblastic meningioma, a form of metaplastic meningioma. There is compelling molecular data to advocate that HO is an active disease process involving metaplastic (osseous) differentiation of meningioma stroma mesenchymal stem-like cells, but not the meningothelial-derived tumor cells. Henceforth, the term “metaplastic meningioma” may not be appropriate in this context. A plausible designation as “psammomatous meningioma with osseous metaplasia” defines this entity more accurately. This paper highlights the need for a unifying nomenclature to reduce diagnostic controversy caused by conflicting terms in the literature. The possible pathogenesis of this intriguing phenomenon is discussed.     

A rare case of periosteal osteoblastoma located in the frontal cranial bone

Periosteal osteoblastoma is an extremely rare bone-forming neoplasm located on the surface of cortical bone. Of the fewer than 30 cases of periosteal osteoblastomas found in the literature, 2 have been reported to be located in cranial bone, and these have not been documented in detail with clinical history, radiographic findings, macroscopic features, and microscopic findings. Although the differential diagnoses of periosteal lesions include parosteal and periosteal osteosarcoma, periosteal chondroma and chondrosarcoma, osteochondroma, osteoid osteoma, periostitis ossificans, and myositis ossificans, an important differential diagnosis both radiologically and pathologically of such a lesion in the cranium is meningioma. We report an unusual case of periosteal osteoblastoma located in the frontal cranial bone that was radiologically consistent with a meningioma. The differential diagnosis of metaplastic meningioma with differentiation toward bone is discussed.     

Anaplastic secretory meningioma: a case report

Secretory meningiomas are rare histological subtypes of meningiomas with benign biological behaviour. In this study, the authors describe the first case of secretory meningioma with many mitotic figures and brain invasion, and discuss the clinicopathologic features including immunohistochemical staining profile and ultrastructural appearance of this tumour. A case of a 54-year-old man diagnosed with an intracranial tumour located in the left frontal lobe is presented. On pre-contrast CT scans, the tumour was hypodense and the contrast enhancement was marked in the pseudo membrane. The tumour was partially removed. The histological diagnosis was secretory meningioma with many mitotic figures, a high MIB-1 labeling index and a brain invasion.     

37-year-old woman with multiple intracranial masses

Extramedullary hematopoiesis (EMH), defined as the presence of hematopoiesis outside bone marrow and peripheral blood, occurs asa compensatory phenomenon in several hematologic disorders and bone marrow dysfunction. EMH predominantly affects reticuloendothelial system including the spleen, liver and lymph nodes. Here,we report a rare case of multiple intracranial meningeal EMH. A37-year-old woman was anemic with gradually worsening vision for 8 months. Multiple extra-axial masses were found on imaging and the patient underwent the biopsy for the left frontotemporal lesion.Final diagnosis was multiple intracranial meningeal EMH. Treatment of fractionated external beam radiotherapy resulted in marked symptomatic improvement. This case indicates that although the diagnosis of meningeal EMH is difficult, there is a need to consider EMHin the differential diagnosis of anemic patients with tumor-like mass lesions in extramedullary sites.     

Periosteal osteoblastoma of the calvaria mimicking a meningioma

While osteoblastoma of the cranial vault is rare, the periosteal form of the tumor is highly unusual, with only one case reported in the English literature. We report on a 24-year-old woman presenting with headache and tinnitus. Magnetic resonance imaging of the brain showed an extra-axial temporal mass with findings that were suggestive of a meningioma. The mass was excised completely, and histological examination revealed a periosteal osteoblastoma arising from the inner surface of the temporal bone and adhering to the dura. The tumor was strongly positive for epithelial membrane antigen, a feature not previously described in osteoblastoma, and one that could lead to a mistaken diagnosis of metaplastic meningioma in a limited sample. A detailed literature review of 40 other reported cases of calvarial osteoblastoma is presented. Apart from being slightly more common in females, calvarial osteoblastoma is similar in all other respects to that arising at conventional skeletal sites. Accurate histological diagnosis of a calvarial osteoblastoma requires adequate sampling of the tumor, including its interface with adjacent structures. Correlation with the radiological findings is crucial for the diagnosis in most cases; however, it is not helpful in differentiating the rare intracranial periosteal variant from a meningioma.     

Bronchial extramedullary hematopoiesis preceding chronic myelogenous leukemia

Extramedullary hematopoiesis of the bronchus is rare. The case of a 72-year-old man in whom the right lower lobe bronchus was obstructed by extramedullary hematopoiesis is presented. Ten months after the initial presentation, Philadelphia chromosome-negative chronic myelogenous leukemia was diagnosed. Such findings have not been reported previously. The various anatomic locations of extramedullary hematopoiesis are reviewed, with an emphasis on intrathoracic and pulmonary presentations. The clinical and pathologic features and the differential diagnosis in the present case are discussed.     

Janus kinase 2 V617F mutation is detectable in spleen of patients with chronic myeloproliferative diseases suggesting a malignant nature of splenic extramedullary hematopoiesis

Extramedullary hematopoiesis occurs in patients with a variety of hematologic diseases, and the spleen is a common site. Extramedullary hematopoiesis is very common in chronic myeloproliferative diseases and myeloproliferative/myelodysplastic diseases. The pathogenesis of extramedullary hematopoiesis is unknown. Using JAK2 V617F mutation as a molecular marker, we assessed paired spleen and bone marrow samples of 15 patients with various types of chronic myeloproliferative diseases and myeloproliferative/myelodysplastic diseases. The diagnosis was chronic idiopathic myelofibrosis (n=8), polycythemia vera (n=3), and chronic myelomonocytic leukemia (n=4). DNA was extracted from fixed, paraffin-embedded tissue and assessed for JAK2 V617F by real-time polymerase chain reaction assay followed by melting curve analysis. Concordant JAK2 mutation was detected in the paired samples in 7 patients. A discordant result with JAK2 V617F found in the spleen but not bone marrow was noted in 1 patient. These results indicate that extramedullary hematopoiesis in patients with chronic myeloproliferative diseases and myeloproliferative/myelodysplastic diseases is a clonal process and lend support to the theory that the cells of extramedullary hematopoiesis are carried from the bone marrow.     

Temporal bone secretory meningioma presenting as a middle ear mass

A 44-year-old woman presented with a history of increasing left hypoacusis and sporadic vertigo. CT scan revealed a tumor occupying the mastoid, middle ear, and external auditory canal. After surgical removal, a typical secretory meningioma was diagnosed. The histological hallmark and the immunohistochemical profile of secretory meningiomas are reviewed. The differential diagnosis of this tumor in this location is also commented on. As far as we know, primary temporal bone meningiomas with secretory histology have not been previously reported in the medical literature.     

Chronic abdominal pain caused by heterotopic ossification with functioning bone marrow: a case report and review of the literature

Heterotopic ossification is rarely seen after midline abdominal surgery. The etiology of heterotopic ossification is unknown. Although it is well recognized that heterotopic ossification may contain osteogenic cells and/or hematopoietic cells, to our knowledge, no case has ever been reported to have histologic evidence of hematopoiesis. We report the occurrence of heterotopic bone with bone marrow showing normal trilineage hematopoiesis in the incision scar of a woman who underwent gastric reduction surgery for the treatment of obesity. The literature regarding heterotopic ossification and extramedullary hematopoiesis is reviewed in this report, and discussion focuses on the mechanism of this pathophysiologic process.     

Cytomorphology of Recurrent Osseous Extracranial Meningioma of Right Pubic Ramus:

Meningiomas are well‐recognized neoplasms of the central nervous system. Primary extracranial meningiomas (ECMs) are extremely rare and arise in various anatomic sites. We present a 56‐year‐old female with 13‐year history of primary grade I meningothelial meningioma of right pubic symphysis, orthotopic heart transplant, and right total hip arthroplasty, who presented with progressive right hip pain for 3 weeks. Primary intracranial, intraspinal and other tumors were excluded. Imaging revealed a destructive lytic lesion at right superior and inferior pubic rami and body, associated with extensive bone destruction and soft tissue mass. Touch imprint (TI) cytology of computed tomography (CT)‐guided core biopsy from the right pubic ramus (PRA) lesion showed a spindle cell neoplasm, with classical syncytial, lobular, and whorling cellular arrangement, composed of spindle, oval or round nuclei with occasional pseudoinclusions, consistent with known history of osseous meningioma. Tumor was further characterized by histopathology as grade 1 meningioma with meningothelial features. To our knowledge, primary osseous ECM arising specifically at the PRA has not been reported previously. ECM at this site may pose a diagnostic challenge for cytologists as its features may resemble other more commonly observed lesions. Accurate diagnosis requires awareness of occurrence of ECM at PRA and recognition of its characteristic cytomorphology. TI cytological features of our case are presented and previously described cytology of ECMs and diagnostic pitfalls are reviewed. Diagn. Cytopathol. 2016;44:618–622. © 2016 Wiley Periodicals, Inc.     

ELASTOSIS IN MENINGIOMA. A Case Report and Electron Microscopic Study

A 55-year-old man underwent left frontoparietal craniotomy for total removal of a parasagittal meningioma. The tumor showed a predominant pattern of meningotheliomatous meningioma and a partial pattern of angiomatous, transitional and fibrous meningioma. A striking feature was the appearance of unusually numerous elastinophilic material in the intercellular stroma. We observed a process in which the progress of elastosis changed the appearance of meningotheliomatous meningioma to a hypocellular, and subsequently acellular flbroelastotic node. Three years after the first craniotomy, meningioma recurred at the same site. The recurrent tumor also showed the histological pattern of meningotheliomatous meningioma with stromal elastosis, as previously observed. This is the first report of a case of meningioma with stromal elastosis. The present case represents the first unequivocal demonstration of elastogenesis in meningioma. ACTA PATHOL JPN 38: 1019∼1029, 1988.     

Sclerosing extramedullary hematopoietic tumor involving lesser omentum and ligamentumteres in liver explant

A 47-year-old patient who presented with a 2-year history of hepatitis B virus-associated liver cirrhosis underwent a liver transplantation. The explanted liver was grossly enlarged and diffusely cirrhotic. The lesser omentum and ligamentum teres revealed a 4 cm ill-defined mass that, on sectioning, showed a gray-white appearance. Extramedullary hematopoiesis on a fibrosclerotic background constituted this mass, but there was also extramedullary hematopoiesis within the liver cords and possibly within the omentum. A bone marrow study also showed chronic idiopathic myelofibrosis. Acute rejection ensued despite immunosuppression and the patient continued to deteriorate and expired. Sclerotic extramedullary hematopoietic tumor is rare, has an unfavorable prognosis, and may mark the end stage of chronic myeloproliferative disorder.     

Malignant melanoma metastatic to a meningioma.

The case presented is that of a 63 year old man with a metastasis to an intracranial meningioma from a malignant melanoma. Although the phenomenon of tumor to tumor metastasis to a meningioma has been previously reported, this is the first case in the literature to date, in which the primary tumor is a malignant melanoma. The criteria for the diagnosis of tumor-to-tumor metastasis and possible reasons for the frequency of metastasis to meningiomas are briefly reviewed.     

Lateral ventricular meningioma presenting with intraventricular hemorrhage: a case report and literature review

Lateral ventricular meningiomas presenting with primary intraventricular hemorrhage are extremely uncommon. We report here a case of primary intraventricular hemorrhage attributable to a lateral ventricular meningioma. This case concerns a 46-year-old female patient who presented with sudden onset of headache. Computed tomography (CT), computed tomography angiography (CTA) and magnetic resonance imaging (MRI) examinations showed hemorrhage from a ruptured tumor mass, which was pathologically confirmed as a transitional meningioma. The patient underwent surgical treatment and had a good prognosis. A retrospective review of eight previous cases of hemorrhage from ruptured lateral ventricular meningiomas revealed that hemorrhage of lateral ventricular meningiomas and hemorrhage of meningiomas at other intracranial sites have similar causes. The clinical and pathological features of ruptured lateral ventricular meningiomas are consistent with those of unruptured lateral ventricular meningiomas. As this clinical entity is extremely rare, attention is called for while performing differential diagnosis.     

Extramedullary hematopoiesis in a bronchial carcinoid tumor. An unusual complication of agnogenic myeloid metaplasia

A case of bronchial carcinoid tumor with foci of extramedullary hematopoiesis is presented. The patient had a 6-year history of agnogenic myeloid metaplasia and hepatosplenomegaly. All three hematopoietic cell lines were represented in different areas of the bronchial tumor. To our knowledge, this is the first case of extramedullary hematopoiesis described in a bronchial carcinoid and only the fourth report of hematopoietic cells in a neoplasm of otherwise unrelated origin. The pathogenesis of this uncommon finding is discussed and the literature is reviewed.     

Rhabdoides Meningeom

Of the numerous morphological variants of meningiomas only few, and among these the rhabdoid meningioma, have prognostic importance. Rhabdoid meningiomas were described for the first time in 1998 as an unusual variant with increased proliferative activity. In 2000 they have been included in the revised WHO classification of tumours of the CNS as a subtype of meningiomas with increased risk of recurrence and more aggressive growth, corresponding to WHO grade III. We report the case of a rhabdoid meningioma in a 21-year-old woman presenting as a intracerebral tumour mimicking an oligodendroglioma. The tumour showed features of a meningioma and a rhabdoid morphology with angiomatous components and was considered to be a rhabdoid meningioma. After surgery a small residual tumour remained. The patient received postoperative radiotherapy resulting in regression of the residual tumour in control examinations after 4 and 8 months. Using the presented case we discuss the differential diagnosis and prognostic significance of recognition of a rhabdoid meningioma.     

Extracranial meningioma in the parapharyngeal space

A case of extracranial meningioma in the parapharyngeal space is reported. A 24 year old woman presented with swelling and tenderness of the parapharyngeal region. A tumor was palpable in this region, and the tumor was surgically removed. Macroscopically the tumor occurred from the portion between the axis and atlas. Histologically the tumor cells, which had oval nuclei and a slightly eosinophilic cytoplasm, proliferated in fibrous connective tissues to form small nests. As the cell borders were not clear, the tumor structure appeared to be syncytium-like. Immunohisto-chemically the tumor cells were positive for anti-vimentin antibodies, anti-S-100 protein antibodies and anti-epithelial membrane antigen (EMA) antibodies in part. Electron microscopically the tumor cells had complex interdigitations of their adjacent plasma membranes. These were studded with many desmosomes. Bundles of intermediate filaments were visible in the cytoplasm. On the basis of the clinical, histological, immunohistochemical and electron microscopical features, the tumor was diagnosed as extracranial meningotheliomatous meningioma. The parapharyngeal space is an extremely rare location for extracranial meningioma, and our case is the first in Japan as far as we know.     

Diabetes insipidus caused by extramedullary hematopoiesis

Myeloid metaplasia involving the central nervous system is a rare occurrence most frequently found as an incidental finding at autopsy. A case is presented of a 56-year-old man with myeloid metaplasia and myelofibrosis, who developed diabetes insipidus. Postmortem examination revealed posterior pituitary involvement by extramedullary hematopoiesis with fibrosis and gliosis. Diabetes insipidus caused by extramedullary hematopoiesis is a rare event and is thus noteworthy.     

Cerebral aspergilloma presenting as atypical meningioma in an immunologically competent patient: a case report

A 36-year-old man presented with history suggestive of intracranial space occupying lesion. Computed tomography of brain revealed a large lobulated, extra axial, hyperdense lesion in the right fronto-temporal region extending up to to the right frontal and ethmoidal sinuses, eroding the bone, enhancing homogeneously with contrast, which was suggestive of atypical meningioma. He had no predisposing factors that could lead to the suspicion of opportunistic infection. Craniotomy and total excision of the lesion was done. Histopathological study revealed aspergilloma.