[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.

Melanotic Neuroectodermal Tumor of Infancy at Skull: Rare and Rapid-Growing Tumor but Histologically Benign.

INTRODUCTION: Melanotic neuroectodermal tumor of infancy (MNTI) is a rare and rapid-growing tumor. However, a neurosurgeon should not overlook this entity when differential diagnosing rapid-growing skull tumor because its histology nature is just benign, and the prognosis is much better than other malignant tumors. CASE PRESENTATION: We reported the case of a 5-month old male presenting with progressive rapid-growing skull tumor which became 10 cm in diameter in only 5 months compared to the normal head circumference at birth. At first, we thought of malignant skull tumor and performed only biopsy to establish diagnosis. But, when the pathology revealed benign MNTI, we performed preoperative tumor embolization and then radical surgery. Good result was observed. DISCUSSION: Skull MNTI is the second most common location after the maxilla. Even advanced imaging nowadays cannot distinguish MNTI from other malignant tumors definitely. Urgent biopsy is recommended to establish diagnosis of this benign tumor first. Preoperative angiography with tumor embolization is recommended when feasible, followed by craniotomy with radical resection.

Gorham-Stout disease with parietal bone osteolysis: a case series and review of literature.

BACKGROUND: Gorham-Stout disease (GSD) is a rare and idiopathic bone disorder, characterized by massive osteolysis. To date, there is no established treatment strategy for GSD. We empirically treated two patients, who had presented to us with cranial lesions of GSD. Here, we propose a novel algorithm for the management of Gorham's disease based on our experience and review the literature published to date. METHODS: We reviewed all existing literature on GSD describing the pathophysiology and suggested treatment methods, up to 2018. RESULTS: We found 13 papers with 14 reported cases; an inclusion of our two cases brings the total count up to just 16 recorded cases of GSD involving the skull. Of these, the base of the skull was affected in eight cases, while the remaining eight cases showed cranial involvement. The patients with skull-base involvement were managed conservatively, using medications or radiotherapy. The patients with cranial osteolysis were managed surgically, with an excision of the osteolytic portion, followed by cranioplasty. Of the latter group, the pericranium was not removed in one patient, in whom a very slight progression of the osteolytic process was later observed. CONCLUSIONS: The pathogenesis of GSD remains poorly understood. Further study is required to determine an optimum management strategy. A long-term follow-up will also be necessary to establish the effectiveness of the treatment process. The untreated patients show a progressive resorption of the affected bones of the skull. A painful, vanishing skull deformity is an alarming sign of GSD. Early diagnosis and treatment are necessary to arrest disease progression and to prevent complications.

[A Case of Ascending Colon Cancer with Skull Metastasis].

A 55-year-old woman became aware of a tumor on the left side of the head in July, 2020 and was referred to our hospital in September because of its rapid growth. A head CT showed a neoplastic lesion of the skull. A CT from the neck to the pelvis revealed an ascending colon tumor and multiple lesions in the liver, which was suspected as metastasis. A colonoscopy also showed a type 2 like lesion in the ascending colon, and a biopsy showed adenocarcinoma. A pedunculated polyp had been pointed out in the ascending colon at another hospital four years previously, and the pathological result was an adenoma, but endoscopic mucosal resection was not performed. It is considered that the adenoma became advanced colon cancer with metastasis through the mechanism of multistage carcinogenesis. Metastatic lesions of the ascending colon cancer was suspected with regard to the skull lesion. In addition to the rapid growth, surgical removal was desirable from the viewpoint of cosmetology, and surgery was performed in November. The postoperative pathological diagnosis was a metastatic skull tumor derived from ascending colon cancer. The diagnosis was Stage IVb according to the Japanese Classification of Colorectal, Appendiceal, and Anal Carcinoma (9th Edition). Although chemotherapy was started after surgery, the metastatic liver cancer increased rapidly and the patient passed away in April, 2021.

Case report: cranial angiosarcoma with multiple hemorrhagic brain metastasis in a child.

BACKGROUND AND IMPORTANCE: Angiosarcoma is a rare malignant tumor with an aggressive course and poor prognosis. It is typically seen in adults but very rarely seen in children. Angiosarcoma of the skull with brain metastasis is exceptionally rare. Due to the rare nature of these tumors, much is left unknown about clinical progression and treatment guidelines are not well established. CLINICAL PRESENTATION AND COURSE: A 14-year-old male patient presented with an enlarging mass on the parietal region of the head. Further investigations revealed a mass lesion involving scalp and skull tissue. Biopsy result showed angiosarcoma and the patient underwent multiple surgical interventions including scalp excision, craniectomy on tumor site, and excision of brain metastases. He also received chemotherapy and radiation therapy. Despite aggressive treatment, disease progression could not be controlled. CONCLUSION: Here we report a pediatric patient with intracranially invasive angiosarcoma of the scalp and skull, with recurrent hemorrhagic metastases to the brain. This is a very rare case in pediatric age group with very poor prognosis. Our patient had impressively longer survival than those reported in the literature despite multiple hemorrhagic brain metastasis and this is most likely related to our aggressive treatment strategy that includes multiple craniotomies for metastatic tumor resection in addition to neoadjuvant chemotherapy and radiation therapy. We believe optimal treatment of skull angiosarcoma in children should aim gross total resection of the skull tumor including involving scalp and dura as well as neoadjuvant chemotherapy and radiation therapy, and the patient should be followed closely with repeated brain MRI studies to pursue additional surgeries to remove brain metastasis if feasible.

Treatment and survival of osteosarcoma and Ewing sarcoma of the skull: a SEER database analysis.

BACKGROUND: Common primary bone tumors include osteosarcomas (OSC) and Ewing sarcomas (EWS). The skull is a rare site, and literature about their treatment and survival is scarce. Using the Surveillance, Epidemiology, and End Results (SEER) database, this study aims to assess the treatment and survival of skull OSC and skull EWS, as well as predictors for survival. METHODS: Skull OSC and EWS cases were obtained from the SEER database. Patient and tumor characteristics, treatment modalities, and survival were extracted. Overall survival (OS) was assessed using multivariable Cox proportional hazard regression stratified by tumor histology. Kaplan-Meier curves were constructed for OS comparing OSC and EWS, as well as histological subtypes in OSC. RESULTS: A total of 321 skull OSC and 80 skull EWS patients were registered from 1973 to 2013. EWS was more common in younger patients (p < 0.001). Resection was the predominant treatment strategy (80.1%), frequently in combination with adjuvant radiotherapy (30.4%). The 5-year survival rate varied significantly between OSC and EWS (51.0% versus 68.5%, p = 0.02). Kaplan-Meier curves show that EWS had a significantly better survival compared to OSC. Comparing histological subtypes of skull OSC, chondroblastic OSC had the best OS, Paget OSC the worst. Older age, tumor advancement, no surgical treatment, and the use of radiotherapy were identified as independent predictors of decreased OS in skull OSC. CONCLUSION: Overall prognosis is better for EWS compared to OSC. Chondroblastic OSC have the best overall survival, while OSC associated with Paget's disease of the bone has the poorest overall survival.

Growing skull hemangioma: first and unique description in a patient with Klippel-Trénaunay-Weber syndrome.

We present the first and unique case of a rapid-growing skull hemangioma in a patient with Klippel-Trénaunay-Weber syndrome. This case report provides evidence that not all rapid-growing, osteolytic skull lesions need to have a malignant character but certainly need a histopathological verification. This material offers insight into the list of rare pathological diagnoses in an infrequent syndrome.

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.

Primary intraosseous xanthoma of the frontal bone in a child: illustrative case.

BACKGROUND: Skull lesions are a common finding in children, with dermoid cysts and eosinophilic granulomas observed most frequently. However, primary intraosseous xanthomas of the calvaria, which are lytic, expansile lesions that develop without underlying hyperlipidemic disease, are rare in children, with only one prior case reported. OBSERVATIONS: The authors describe the case of a healthy 6-year-old male who presented with a 2-month history of an enlarging midline skull mass that developed after a recent minor trauma. Imaging showed a full-thickness, lytic frontal bone lesion with an aggressive appearance and heterogeneous contrast enhancement. The patient underwent gross-total resection of the lesion with placement of a mesh cranioplasty. Histopathology revealed a primary intraosseous xanthoma. The patient was discharged on postoperative day 2 and required no further treatment at the 1-month follow-up. LESSONS: This is the first reported case of a primary intraosseous xanthoma in the frontal bone of a pediatric patient. It emphasizes the need to include primary xanthomas in the differential diagnosis for pediatric skull lesions, particularly when the lesion has an aggressive radiographic appearance or the patient has a history of focal trauma. Furthermore, our findings indicate that resection, together with subsequent monitoring for lesion reccurrence, is an adequate first-line treatment.

Giant cranial angiolipoma with arteriovenous fistula: A case report.

Background: Angiolipomas are benign mesenchymal tumors comprising mature adipocytes and abnormal blood vessels, commonly found in the subcutaneous tissue of the trunk and rarely in the skull. Furthermore, sporadic cases of angiolipoma with arteriovenous fistula (AVF) have been reported. Case Description: We reported the case of a 72-year-old woman who presented with head swelling, seizures, and cognitive dysfunction. Computed tomography and magnetic resonance imaging revealed a right frontal bone tumor exceeding a sagittal suture of up to 10.7 cm. Angiography revealed AVF and varices formation. Endovascular embolization was performed to treat the AVF and reduce blood loss during surgical resection. Two days after the embolization, a craniotomy was performed; however, uncontrollable bleeding was observed at the time of tumor resection. Postoperatively, the patient was symptom-free and has been stable for 2 years without recurrence. Conclusion: Despite careful preoperative evaluation and treatment planning, the patient in this case report was difficult to treat. Such cases require adequate preparation.

Surgical management of giant cell tumor invading the occipital bone: A case report and literature review.

Background: Giant cell tumor of bone (GCTB) rarely originates in the skull, particularly in the occipital bone. Although benign, it can severely destroy the surrounding tissue and undergo an unpredictable clinical course. We report the successful resection of a GCTB invading the occipital bone in a Hispanic adult woman and present a comprehensive review of the literature on this rare pathology by focusing on the occipital area. Case Description: A 40-year-old Hispanic woman presented with a 3-month history of neck pain and a bulging lesion on the retromastoid area. Brain magnetic resonance imaging (MRI) revealed an extradural, expansive, and contrast-enhancing lesion in the right occipital bone with multiple thin septa and evidence of bleeding. The patient underwent an uneventful gross total resection (GTR) of the lesion. The histopathological examination findings included numerous scattered osteoclast-type giant multinucleated cells. At a 10-month follow-up, the patient has not developed any neurological deficits, impairment of life functioning, or signs of recurrence in MRI. Conclusion: GCTB rarely originates in the skull, being the occipital bone the most infrequent site of presentation. When feasible, total surgical resection effectively reduces the risk of recurrence. Nonetheless, radiation and adjuvant therapies have been employed when GTR could not be achieved. A close follow-up with a brain MRI is advised to control recurrence.

Adult Langerhans Cell Histiocytosis Diagnosed by Biopsy of the Skull Tumor Generated after Craniotomy.

Langerhans cell histiocytosis (LCH) is a disease characterized by the proliferation of Langerhans cells. Most cases of LCH occur in children, although it can be seen in adults as well. We encountered an adult case of LCH. A 44-year-old woman who was diagnosed as diabetes insipidus underwent a magnetic resonance imaging (MRI) of the head which revealed sellar and suprasellar gadolinium-enhanced mass. Prolactin level was high and cabergoline was prescribed. The size of this mass had reduced, so we supposed the tumor was prolactinoma. However, after 4 years of observation, it had increased once again. The biopsy of pituitary stalk lesion was performed via transcranial approach. The histological diagnosis was initially gangliocytoma. The patient complained of back pain after surgery. Three months after the biopsy, a computed tomography (CT) scan revealed multiple osteolytic lesions throughout the entire body. One of the osteolytic lesions of the skull was removed to determine the diagnosis. The pathological examination of the skull led to a diagnosis of LCH. We concluded retrospectively that the lesion of the pituitary stalk was LCH mimicking gangliocytoma though classical pathological findings were not obtained. In conclusion, LCH should be considered as a differential diagnosis in adult cases of diabetes insipidus with hypothalamic-pituitary lesion.

Custom-Made Porous Hydroxyapatite Cranioplasty in Patients with Tumor Versus Traumatic Brain Injury: A Single-Center Case Series.

BACKGROUND: Cranioplasty is a common neurosurgical procedure with the goal of restoring skull integrity. Custom-made porous hydroxyapatite prostheses have long been used for cranial reconstruction in patients with traumatic brain injury. We present a large consecutive series of 2 groups of patients undergoing cranioplasty with hydroxyapatite custom bone and compare the adverse events (AEs) between the 2 groups. METHODS: We examined a series of consecutive patients who underwent cranioplasty using custom-made porous hydroxyapatite implants following tumor resection and traumatic brain injury at a single center between March 2003 and May 2018. The implants were designed and produced according to the surgeon's specifications and based on the patient's computed tomography scan data obtained through a standardized protocol. AEs were recorded. RESULTS: Information on 38 patients with tumor and 39 patients with traumatic brain injury was collected and analyzed. A significant difference in the timing of surgery was found between the 2 groups; single-stage surgery was performed in 84% of patients in the tumor versus 8% of those in the traumatic brain injury group (P < 0.0001). The rate of AEs was not significantly different between the 2 groups (P = 0.4309) and was not related to the timing of surgery. CONCLUSIONS: Custom-made hydroxyapatite cranioplasty is a solution for cranial reconstruction in patients with cranial tumors. The low incidence of AEs in a consecutive series of patients with either trauma or tumors demonstrates that these prostheses represent a safe solution independent of the characteristics of cases.

Epithelioid Osteoblastoma of the Temporal Bone: A Case Report.

BACKGROUND: Epithelioid osteoblastoma of the cranium is extremely rare and can mimic other etiologies on radiographic imaging, pathology, and symptomatology. CASE DESCRIPTION: An 18-year-old male patient had a 3-week history of a palpable left temporal mass. Magnetic resonance imaging revealed a large, extra-axial, hypervascular mass in the left temporal bone, with bony erosion and intracranial extension. The patient underwent surgical near gross-total resection of the mass. Initial frozen microscopic examination of the tumor was inconclusive. The postoperative course was uneventful, and the patient was discharged a few days later. Final pathology confirmed the diagnosis of epithelioid osteoblastoma. CONCLUSIONS: Epithelioid osteoblastoma of the skull base is exceedingly rare but should be included in the differential diagnoses of all extra axial tumors. Preoperative radiographic clues are limited, and final diagnosis relies solely on accurate pathologic examination. A diagnosis of epithelioid osteoblastoma should be considered for all cranial bone-based tumors, as an incorrect diagnosis of another radiographic and histologic mimic could lead to the patient receiving unnecessary and harmful neoadjuvant/adjuvant chemotherapy or radiotherapy.

Intraosseous Orbital Cavernous Hemangioma with Frontal Extension and Dural Involvement.

Primary intraosseous cavernous hemangiomas are rare skull lesions that are not typically known to involve the orbital bones or the dura. We describe a rare case of a fronto-orbital bone cavernous hemangioma with extension into the dura. A 68-year-old female presented with a one-year history of diplopia with discomfort around her left orbit. Magnetic resonance images demonstrated a mass in the left frontal skull extending into the orbital rim. The patient underwent a craniotomy for tumor resection. Dural invasion was found intraoperatively. Gross total resection and reconstruction were achieved. On the postoperative follow-up, the patient was asymptomatic.  Primary calvarial intraosseous cavernous hemangiomas are most commonly located in the frontal and parietal bones. These lesions typically involve only the outer table of the skull. In lesions involving the orbit and dura, excision with cranioplasty can provide symptomatic relief with good cosmetic outcomes.

Dedifferentiated parosteal osteosarcoma of the calvaria.

Dedifferentiated parosteal osteosarcoma is a rare tumor and is even rarer when involving the skull bones. We present a case of a 57-year-old man with a partially ossified progressive enlarging left skull mass in the left temporoparietal region, with erosion of the outer table. Radiological diagnosis of dedifferentiated parosteal osteosarcoma was suggested, and histopathology confirmed the diagnosis.

Direct puncture Onyx embolization of a large calvarial metastasis with intracranial extension: Case report.

We report a case of renal cell carcinoma (RCC) metastasis to the calvarium and describe a strategy for percutaneous embolization of hypervascular calvarial tumors with intracranial extension. An elderly patient with history of RCC presented with left-sided weakness. Imaging studies showed a large right frontoparietal calvarial mass with intra- and extracranial extension. The tumor was devascularized by direct puncture tumor embolization using Onyx 18, allowing subsequent operative resection without significant blood loss or the need for flap reconstruction of the scalp. Compared to more common endovascular approaches, direct-needle puncture embolization of transcalvarial masses may offer lower risk of injury to scalp vessels and underlying brain parenchyma.

Intensity-modulated radiotherapy for paranasal sinuses and base of skull tumors.

Paranasal sinus and skull base tumors are rare aggressive head and neck cancers, and typically present in the locally advanced stages. As a result, achieving wide surgical resection with clear margins is a challenge for these tumors, and radiotherapy is thus usually indicated as an adjuvant modality following surgery to optimize local control. Given the integral role of radiotherapy in the management of this subgroup of head and neck tumors, the advent of intensity-modulated radiotherapy (IMRT) has led to substantial improvement of clinical outcomes for these patients. This is primarily driven by the improvement in radiation dosimetry with IMRT compared to conventional two dimensional (2D)- and 3D-techniques, in terms of ensuring dose intensity to the tumor target coupled with minimizing dose exposure to critical organs. Consequently, the evident clinical benefits of IMRT have been in reduction of normal tissue toxicities, ranging from critical neurological symptoms to less debilitating but bothersome symptoms of eye infections and radiation-induced skin changes. Another domain where IMRT has potential clinical utility is in the management of a subset of non-resectable T4 paranasal sinus and skull base tumors. For these inoperable lesions, the steep dose-gradient between tumor and normal tissue is even more advantageous, given the crucial need to maintain dose intensity to the tumor. Innovative strategies in this space also include the use of induction chemotherapy for patient selection. In this review, we summarized the data for the aforementioned topics, including specific discussions on the different histologic subtypes of paranasal sinus and skull base tumors.

Skull Meningioma Associated with Intradural Cyst: A Case Report.

We present the first report of intraosseous meningioma accompanied by intradural cyst formation. A 76-year-old woman had previously undergone breast cancer treatment, so the preoperative diagnosis was metastatic breast cancer. This case reminds us that the possibility of meningioma should be kept in mind in patients with breast cancer, irrespective of neuroimaging findings.

Osteolytic calvarial lesions as initial presentation of latent neurosyphilis.

We report a patient with human immunodeficiency virus infection whose initial presentation of neurosyphilis was painful calvarial lesions. A literature search reveals seven previous cases of calvarial involvement in early acquired syphilis but none in patients diagnosed with neurosyphilis. This patient emphasizes the need to consider atypical infections when encountering skull lesions, especially in the setting of human immunodeficiency virus co-infection.