[Primary intraosseous cavernous hemangioma of the cranium: a case report]. / Pervichnaya vnutrikostnaya kavernoznaya gemangioma kostei cherepa: obzor literatury i opisanie klinicheskogo nablyudeniya khirurgicheskogo lecheniya.

Primary intraosseous cavernous hemangioma (PICH) is a rare benign vascular tumor. This neoplasm is common in the spine and less common in skull. Toynbee J. first described this tumor in 1845. PICH of the cranium does not always have typical X-ray features and should be always differentiated with other more common skull lesions. Surgical resection is preferable since total resection is followed by favorable prognosis. We present a 65-year-old patient with asymptomatic tumor of the right parietal bone. CT revealed osteolytic lesion that required total resection and skull repair. Histopathological analysis revealed intraosseous cavernous hemangioma.

A unique case of multiple calvarial hemangiomas with one large symplastic hemangioma.

BACKGROUND: Symplastic hemangioma is a benign superficial abnormal buildup of blood vessels, with morphological features which can mimic a pseudo malignancy. A few cases have been reported in the literature. We report here, a unique case of calvarial symplastic hemangioma, which is the first case in the calvarial region. CASE PRESENTATION: A 29-year-old male patient, with a left occipital calvarial mass since childhood, that gradually increased in size with age, was associated with recurrent epileptic fits controlled by Levetiracetam (Keppra), with no history of trauma. He presented to the emergency room with a recent headache, vomiting, frequent epileptic fits and a decrease in the level of consciousness 1 day prior to admission. A CT scan showed three diploic, expansile, variable sized lytic lesions with a sunburst appearance; two that were biparietal, and one that was left occipital, which were all suggestive of calvarial hemangiomas. However, the large intracranial soft tissue content, within the hemorrhage of the occipital lesion was concerning. The patient had refused surgery over the years; however, after the last severe presentation, he finally agreed to treatment. The two adjacent, left parietal and occipital lesions were treated satisfactorily using preoperative embolization, surgical resection, and cranioplasty. Histopathology revealed cavernous hemangiomas, in addition to symplastic hemangioma (pseudo malignancy features) on top at the occipital lesion. The right parietal lesion was not within the surgical field; therefore, it was left untouched for follow-up. CONCLUSIONS: Histopathology and radiology examinations confirmed the diagnosis as symplastic hemangioma, on top of a pre-existing cavernous hemangioma. To the best of our knowledge, this is the first case of a calvarial symplastic hemangioma, which we report here.

Multiple myeloma complicated by skull plasmacytoma discovered after head injury.

Plasmacytoma is a malignant tumor originating from the plasma cells of the bone marrow. Those discovered after a head injury is rare. We report a case of a 48-year-old female who complained of scalp mass without other symptoms after head injury. Meningioma was considered preoperatively based on imaging findings, and surgical resection was performed. Postoperatively, multiple myeloma complicated by skull plasmacytoma was diagnosed by histopathology and systematic examinations in succession. When evaluating a head mass that appeared after a head injury, plasmacytoma should be considered at times. Osteolytic changes and biconvex form on imaging are beneficial to differentiation.

Intraosseous cavernous malformations of the skull: clinical characteristics and long-term surgical outcomes.

Intraosseous cavernous malformations (ICMs) of the skull are relatively rare, benign, and slow-growing tumors. Knowledge of these lesions is poor. The goals of this study were to describe the clinical manifestations, radiological features, and long-term surgical outcomes of this disease. We performed a retrospective analysis of a series of 16 cranial ICM patients who underwent surgical treatment in our hospital between 2003 and 2016. The incidence of cranial ICM was 1.15% among the entire series of intracranial and intraspinal CMs. Our cohort included 6 male and 10 female patients; their mean age at operation was 38.7 years (range, 1.9 to 63 years). Slowly growing swelling was the commonest clinical manifestation of this disease. Complete lesion resection was achieved in all but one patient, and cranioplasty was performed using titanium mesh in 11 cases. Postoperative complication was found in one patient who developed diplopia, and this symptom resolved spontaneously before discharge. The mean follow-up period after operation was 76.2 months (range, 19 to 119 months). Only the patient with petroclival ICM had some occasional headaches (mRS = 1); others were all in stable neurological status (mRS = 0). No lesion recurrence was found during the follow-up period. Although cranial ICM is rare, it should always be considered as a differential diagnosis in the case of firm, gradually enlarging skull lesions. Surgical resection should be the treatment of choice for the symptomatic patients and their long-term outcomes were excellent after gross total removal.

Diffuse cavernous hemangioma of the skull misdiagnosed as skull metastasis in breast cancer patient: one case report and literature review.

BACKGROUND: Primary intraosseous cavernous hemangiomas (PICHs) of the skull are extremely rare. To date, diffuse cranial hemangioma of skull has not been reported. In cancer patients, it is often misdiagnosed as metastasis. CASE PRESENTATION: Here, we presented a case of a 50-year-old female patient suffering from slightly headache who received breast cancer modified radical mastectomy in 2004, computed tomography and magnetic resonance imaging findings revealed abnormal lesions of diffuse skull which were misdiagnosed as skull metastasis, and the relevant literatures were also reviewed. CONCLUSIONS: Diffuse cavernous hemangioma of the skull is exceedingly rare, and imaging data are not typical. The condition is often misdiagnosed, and pathological evaluation is necessary and important. In cases where the mass cannot be completely removed by surgery, radiotherapy could be beneficial.

Primary intraosseous osteolytic meningioma: a case report and review of the literature.

BACKGROUND: Primary intraosseous meningioma is a subset of extradural meningioma that arises in the bone, and only a few cases have been reported to date. CASE PRESENTATION: An 80-year-old man presented with decreased hearing on the right side accompanied by a disturbance of balance 10 months prior to admission. Magnetic resonance imaging revealed an 8 × 7 cm osteolytic mass in the right posterior fossa related to the petrous bone, with extension to the cervical region. During surgery, the tumor was found to be located extradurally, with no invasion of the dura. The tumor was removed entirely, apart from a small portion around the jugular foramen to avoid lower cranial nerve injury. CONCLUSION: The final diagnosis was primary intraosseous osteolytic meningioma with atypical pathology. Here, we report a rare case of an osteolytic skull lesion in the skull base not invading the dura and with extensive bone destruction.

Co-existence of Langerhans cell histiocytosis and reticulohistiocytosis with initial presentation of skull lesions: A case report.

Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder resulting from dysregulated clonal proliferation of Langerhans cells. Reticulohistiocytosis (RH) is another rare histiocytosis caused by the proliferation of histiocytes other than Langerhans cells. Co-existence of LCH and RH in different organs and in the same skin area has not been reported. We present the case of a 20-year-old woman who initially had co-existing bone LCH and cutaneous RH. After 1 year of chemotherapy with cytarabine, bone LCH significantly improved but cutaneous LCH developed in the same area where cutaneous RH was, resulting in hybrid LCH and RH of the skin. This unique history provides some evidence to support the theory that LCH and RH originate from the same stem cells and subsequently develop into hybrid LCH and RH of the skin in a cytokine environment influenced by chemotherapy. Repeat skin biopsies may be considered for adjusting treatment regimens in LCH patients whenever pre-existing skin lesions progress.

Myeloid sarcoma involving the greater wing of the sphenoid bone and additional skeletal sites presenting with unilateral proptosis and fevers.

We report a case of myeloid sarcoma with multifocal skeletal involvement, including the greater wing of the sphenoid bone. A 23-month-old boy presented with left-sided proptosis and fevers, and was found to have an infiltrative mass involving the left sphenoid bone on orbital imaging. Full body imaging further demonstrated multiple bony lesions in the pelvis, thoracic and lumbar vertebrae, bilateral femura, and left humerus, and biopsies of the humerus were consistent with myeloid sarcoma. The patient was started on a standard chemotherapy regimen and is responding well. Myeloid sarcoma presenting with proptosis due to sphenoid bone involvement with simultaneous multifocal skeletal involvement is very uncommon and highlights the importance of biopsy for establishing a definitive diagnosis.

Pediatric cranial intraosseous hemangiomas: a review.

Hemangiomas are benign, slow-growing tumors composed of sinusoidal blood vessels. Skeletal hemangiomas are uncommon and are mostly vertebral, followed by cranial in location. Cranial hemangiomas are very rarely encountered in children. Authors report a 12-year girl who presented with a painless enlarging mass over the parietal scalp for 3 months. Imaging revealed a left parietal intraosseous lytic mass with a sunburst appearance. Enbloc removal and cranioplasty was performed, and histopathology was suggestive of hemangioma. We reviewed the literature on pediatric cranial intraosseous hemangiomas (PCIH) (age ≤18 years) by searching online database. Including ours, a total of 24 cases were analyzed. Mean age was 10.2 years (range 4 months-17 years). Eight were in the first decade and 16 were in the second decade. Male:female ratio was 12:12 (1:1). A painless palpable mass was the commonest presenting feature. Parietal and frontal bones were most commonly involved. Intracranial extension was noted in cases. Mean size of the lesion was 5 cm (range 1-12 cm). Twenty-two underwent primary surgical removal while two had additional pre-operative embolization. Surgical procedures were craniectomy alone (n-3), craniectomy + cranioplasty (n-6), tumor excision + remodeling (n-3), and tumor debulking (n-2). Histopathology was cavernous type in majority of cases. Mean follow-up duration was 11.8 months (range 2-38 months). There were no recurrences. One patient died due to systemic infection. Ours is the first review exclusively on PCIH. Although rare, they need to be considered in the differential diagnosis of lytic skull lesions in children. Enbloc removal with cranioplasty is the preferred treatment in vault hemangiomas, while embolization followed by debulking would suffice in large cranial base lesions.

Melanotic neuroectodermal tumor of infancy: A rare pediatric head and neck lesion.

Melanotic neuroectodermal tumors of infancy are rare tumors arising from the neural crest and typically present during the first 12 months of life. The majority involve the facial bones, although melanotic neuroectodermal tumors of infancy of the skull and extremities have been observed with less frequency, as in the present case. This entity may initially be presented to the dermatologist as a scalp mass and should be considered in the differential diagnosis of infants with rapidly growing head and neck lesions.

Congenital Calvarial Hemangioma.

OBJECTIVES: The authors describe a case of congenital calvarial hemangioma successfully managed using propranolol therapy. Presenting symptoms, radiological and pathological features, differential diagnosis, and management of this rare congenital mass are described. CASE PRESENTATION: A 2-year-old boy presented with a 1-year history of a growing right parietal skull mass. No obvious etiology was apparent. No focal neurological deficits or associated craniofacial anomalies were identified. Plain film imaging demonstrated focal thickening of the right parietal bone with internal trabeculations in a sunburst appearance. Computed tomography (CT) scan showed bone thickening with coarsening of the bony trabeculae, minor irregularity of the outer table, unaffected inner table, and no evidence of aggressive features. A diagnostic biopsy of the lesion was performed in the operating room. Microscopic examination was consistent with hemangioma. Based on histological and radiological features of the lesion, it was identified as a cavernous hemangioma. Medical treatment utilizing propranolol was initiated for over 3 years with interval reduction in the lesion size. MRI head following treatment with propranolol demonstrated reduction of the mass compared to preoperative imaging. CONCLUSIONS: Although a rare entity, it is important to consider congenital calvarial hemangioma in the differential diagnosis of slow growing skull lesions due to the possibility of complications as a result of the hemangioma's intracranial extension, and the potential for treatment. En bloc resection has classically been described as a treatment for such lesions, although our case demonstrates that medical treatment with propranolol therapy may be appropriate in certain situations.

Extraskeletal presentation of Ewing's Sarcoma.

The case of a 27-year-old Hispanic female who presented with an occipito-parietal tumor after suffering trauma to the area. A physical examination revealed no tenderness to palpation and with evidence of healing ulcerations. The biopsy was consistent with a synovial sarcoma. A wide excision of the mass (15cm x 14cm x 6cm) followed by a pericranial flap was performed. A follow-up CT showed recurrence involving the parietal sagittal sinus. After a second biopsy the mass was determined to be a small-cell sarcoma, consistent with Ewing's sarcoma. Chemotherapy included 8 cycles of doxorubicin, vincristine, and cyclophosphamide, with alternating cycles of etoposide and ifosfamide. A year later, a second wide excision of the mass was performed, followed by bilaminate skin substitute and skin graft placement for reconstruction of the soft-tissue defect. After chemotherapy, a follow-up PET scan showed no signs of re-uptake in any soft tissue or skeletal structures. After 2 years, the patient remains in complete remission.

Clinical implications, diagnosis, and treatment of a giant frontoethmoid osteoma.

Osteomas are benign, asymptomatic fibro-osseous tumors that are most commonly observed in the paranasal sinuses and sometimes are found in imaging examinations that were taken for other reasons. Giant osteomas are rarely found in the frontal and ethmoid sinuses but, when they are, they may cause intracranial and orbital complications. The aims of this case report are to describe a frontoethmoid osteoma in a 40-year-old woman, discuss the characteristics of this lesion through cone beam computed tomography (CBCT) imaging, and review the options for treatment. A CBCT examination performed for the purpose of orthodontic diagnosis revealed the presence of a large, well-defined, lobular, hyperdense mass that occupied a sizeable area of the frontal sinus and extended to the ethmoid sinus. The patient did not report any pain. Based on the physical and imaging characteristics of the mass, the location and size of the lesion, and the patient's age, the diagnosis was a giant frontoethmoid osteoma. Due to its enhanced field of view, CBCT may enable earlier diagnosis of lesions that affect the maxillofacial region.

Unusual skull tumors with psammomatoid bodies: a diagnostic challenge.

AIM: We describe a series of three diagnostically challenging, histologically similar fibro-osseous skull masses. METHODS: The cases were identified in our archives among 50,000 neuropathology specimens. A comprehensive review of the histological, immunohistochemical, ultrastructural, and imaging features as well as the clinical outcome was performed. RESULTS: The routine histology was similar in all 3 cases and showed spindle cell proliferations with frequent calcospheres or psammomatoid bodies. There was no evidence of an underlying subdural component. Immunohistochemistry for the meningioma markers EMA and SSTR2A raised the possibility of intraosseous meningioma, as all 3 lesions were convincingly positive for epithelial membrane antigen (EMA) and 1 lesion was convincingly positive for the somatostatin receptor subtype 2A (SSTR2A); weak, questionable positivity for SSTR2 was present in the remaining 2 cases. In addition, electron microscopy was available in 1 case and showed features consistent with meningioma. CONCLUSIONS: Overall, the findings were most consistent with intraosseous meningioma. Primary intraosseous meningiomas are rare lesions that may present a diagnostic challenge. It is important to consider meningiomas in the differential diagnosis, as extradural meningiomas are associated with an increased risk of recurrence and may occasionally undergo malignant transformation.
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Sphenoid Wing Meningioma With Extraocular Muscle Involvement Mimicking Idiopathic Orbital Inflammation.

Meningiomas are slow growing, typically benign, tumors originating from arachnoid cap cells. Specifically, sphenoid wing or spheno-orbital meningiomas infiltrate the lesser wing of the sphenoid, lateral orbital wall, and orbital roof with occasional extension to the superior orbital fissure, optic canal, anterior clinoid process, and middle cranial fossa where neurologic and ophthalmologic functions are impaired by compressive injury. The extraocular muscles are rarely involved. The authors present a rare case of an spheno-orbital meningioma causing extraocular muscle enlargement mimicking idiopathic orbital inflammation and highlight important diagnostic clues to meningioma.

Differential Diagnosis of Intramuscular Schwannoma of the Craniocervical Region.

Schwannomas in the head and neck are a relatively common condition. However, intramuscular schwannomas in the craniocervical region are extremely rare. The preoperative consideration of schwannomas is very important to preserve adjacent circulation and nerve function during the surgery in this area. Moreover, the treatment and preoperative evaluation of this condition is not firmly established in the past literatures. The authors successfully managed the intramuscular schwannoma in the craniocervical region, and provide the differential diagnoses with appropriate treatments.

Primary Ectopic Ethmoidal Craniopharyngioma.

Craniopharyngiomas are benign but aggressive epithelial tumors usually originating in the anterior lobe of the pituitary gland from squamous remnants of an incompletely involuted craniopharingeal duct developing from the Rathke pouch. To the authors' knowledge only 1 patient of a primary isolated ethmoidal craniopharyngioma has been reported in the literature.The authors report the case of a 17-year-old boy with a primary extracranial ethmoidal craniopharyngioma. An endoscopic endonasal approach was employed to resect the tumor. After 2 years of clinical and radiological follow-up no recurrence of disease was observed.Primary ethmoidal craniopharyngiomas are rare entities and biopsy is necessary for diagnosis. However, a preoperative assessment by means of nasal endoscopy, computed tomography scan, and enhanced magnetic resonance imaging is mandatory to better evaluate the extension and characteristics of the tumor. The endoscopic endonasal technique is a safe and effective approach for the treatment of these lesions.

Benign fibrous histiocytoma arising at the temporal bone of an infant--case report and review of the literature.

PURPOSE: We report a very rare case of a fast-growing benign fibrous histiocytoma at the temporal bone. METHODS: A 1-year-old girl was referred for investigation of a right temporal mass that increased during 2-week observation. Imaging studies showed a lytic, loculated skull tumor at the left temporal bone. On magnetic resonance imaging scans, the tumor was isointense on T1- and relatively high-intense on T2-weighted images. The tumor was mostly homogeneously enhanced by gadolinium. On diffusion-weighted images, it was iso- to relatively low-intense; perfusion-weighted images revealed low perfusion. RESULTS: Complete macroscopic resection of the tumor was performed. Immunohistochemical analysis showed that the tumor was positive for CD68 and α-smooth muscle actin and negative for CD1a and CD34; the MIB-1 labeling index was 4 %. A diagnosis of primary benign fibrous histiocytoma of the skull was made. At 6-month follow-up, there were no clinical or radiological signs of tumor recurrence and/or metastasis. CONCLUSIONS: We review the clinical, radiological, and immunohistochemical characteristics of benign fibrous histiocytoma at the skull.

Characteristic of optic canal invasion in 31 consecutive cases with tuberculum sellae meningioma.

Optic canal invasion by tuberculum sellae meningiomas (TSMs) has been reported, but the characteristics of invasion remain unclear. This study was performed to clarify the incidence and characteristics of optic canal invasion by TSM and to determine whether optic canal invasion could be predicted preoperatively by magnetic resonance imaging (MRI). Between February 2002 and August 2014, 31 patients with TSM underwent tumor resection in our institute. In all cases, the optic canal was explored to identify any tumor invasion. We classified the characteristics of optic canal invasion from intraoperative findings. Invasion was classified into four types: type 1: no invasion; type 2: secondary invasion; type 3: partial wall invasion (two subtypes); and type 4: invasion into the supero-medial-inferior walls of the optic canal. Thirty of 31 cases showed optic canal invasion. Of these 30 cases, 9 (30 %) showed bilateral optic canal invasion. The most common finding was type 1 (23 sides). Among cases with optic canal invasion (39 sides), type 4 was the most common pattern (17 sides), followed by type 3-infero-medial (13 sides), type 2 (5 sides), and type 3-supero-medial (4 sides). Blinded prediction of tumor invasion was accurate in 61 % of cases, but characteristics of tumor invasion were undeterminable from preoperative MRI. In conclusion, optic canal invasion was frequently seen in our consecutive series of TSM, characteristics of which were unpredictable preoperatively. Neurosurgeons should be aware of the high incidence and variety of optic canal invasion in planning strategies for TSM treatment.

A Rare Presentation of a Dermoid Cyst with Draining Sinus in a Child: Case Report and Literature Review.

Orbitofacial dermoid cysts in children are treated using surgical excision. Dermoid cysts of the frontotemporal region usually present as superficial, slow-growing masses without deep extension. We report a rare case wherein a frontotemporal dermoid in a 21-month-old girl presented with a draining sinus tract to the skin and extended intracranially through the sphenoid bone. It was removed surgically and a dermoid cyst was removed from the frontotemporal region, extending superficially from the skin sinus tract through the sphenoid bone and attaching to the dura of the anterior temporal lobe. In addition to our case presentation, a literature review was performed to identify the few reported similar cases in the published literature, the combination of which suggests that frontotemporal dermoid cysts with associated draining sinus tracts may require preoperative imaging with computed tomography or magnetic resonance imaging before surgical intervention because of the high likelihood of intracranial extension.