Two-step treatment of a giant skull vault hemangioma: A rare case report and literature review / Tratamiento en dos pasos de un hemangioma de la bóveda craneal gigante: informe de un caso poco frecuente y revisión de la literatura

kull vault hemangiomas are benign vascular tumours of the calvaria that are usually asymptomatic or present as firm, painless lumps. We present a case of a 59-year-old female with a giant intraosseous calvarial hemangioma that was admitted in our department with a palpable mass over the left frontoparietal region, personality changes and impaired emotional and cognitive functions. The patient was treated with a two-step approach involving endovascular and surgical treatment, and suffered two rare, but recognized complications, a contrecoup intracerebral haemorrhage and valproate-induced stupor and parkinsonism. At the 6-month follow-up, the patient had complete recovery with a good neurological outcome (AU)

Los hemangiomas de la bóveda craneal son tumores vasculares benignos de la calota que suelen ser asintomáticos o aparecen como bultos firmes e indoloros. Presentamos el caso de una mujer de 59 años con un hemangioma de calota intraóseo gigante que ingresó en nuestra unidad con una masa palpable en la región frontoparietal izquierda, con cambios de personalidad y con la alteración de las funciones emocional y cognitiva. La paciente recibió tratamiento con un método de dos pasos que incluía terapia endovascular y quirúrgica, y experimentó dos complicaciones poco frecuentes, pero reconocidas: hemorragia cerebral por contragolpe, así como estupor y parkinsonismo inducidos por valproato. A los seis meses de seguimiento, la mujer se recuperó por completo con un buen desenlace neurológico (AU)

Basal Ganglia Germ Cell Tumors With or Without Sellar Involvement: A Long-Term Follow-Up in a Single Medical Center and a Systematic Literature Review.

Background: Basal ganglia germ cell tumors (BGGCTs) represent an extremely rare subset of tumors about which little is known. Some patients suffer from tumor dissemination, such as sellar involvement. This study aimed to evaluate the independent prognostic risk factors of patients with BGGCTs with or without sellar involvement. Methods: Sixteen patients were diagnosed with BGGCTs at Peking Union Medical College Hospital from January 2000 to December 2020. A literature review was performed on the online databases Medline and PubMed, and 76 cases in the 19 retrieved articles were identified at the same time. The data regarding biochemical tests, radiological examinations, and outcomes during follow-up were analyzed. Results: Of 92 patients in this study, seven patients were clinically diagnosed as germinomas, with the remaining 85 patients receiving surgery. Fifty-two patients suffered from multifocal lesions or tumor dissemination. The patients with BGGCTs demonstrated a significant male predilection. The patients with delayed diagnosis more likely had cognitive disturbance (p = 0.028), mental disturbance (p = 0.047), and diabetes insipidus (p = 0.02). Multivariate analysis demonstrated that the independent poor prognostic risk factors of patients with BGGCTs were delayed diagnosis [odd ratio (OR) 2.33; 95% CI 1.02-5.31], focal radiotherapy (OR 4.00; 95% CI 1.69-9.49), and non-pure germinoma (OR 4.64; 95% CI 1.76-12.22). Conclusions: The delayed diagnosis, focal radiotherapy, and non-pure germinoma were associated with a poorer prognosis for patients with BGGCTs with or without sellar involvement.

"Slipped capital femoral epiphysis in a 25-year-old hypogonadic man with a large cranial chondroma: causality or coincidence? ".

BACKGROUND: Slipped capital femoral epiphysis (SCFE) is a hip disorder frequently occurring in adolescence. In adults it is rare and so far very few cases have been documented. CASE PRESENTATION: This report presents a 25-year-old patient diagnosed with an anterior fossa giant chondroma, hypogonadotropic hypogonadism, and SCFE. The patient underwent surgical and hormonal therapy. His symptoms revealed, and he became a father. CONCLUSIONS: Every patient diagnosed with SCFE in adulthood should undergo endocrinological assessment based on physical examination and laboratory tests.

Primary yolk sac tumor of pterygopalatine fossa with loss of vision: A case report.

INTRODUCTION: Primary yolk sac tumor (YST) is an infrequently-diagnosed malignant extragonadal germ cell tumors. It is likely to recur locally and may present with widespread metastases once diagnosed. Primary YST of the head is uncommon but can cause severe complications, such as loss of vision once the tumor mass invades the optic nerve. PATIENT CONCERNS: A 20-month-old boy presented to the general clinic of the local children's hospital with a complaint of swelling of left face for 1 year and proptosis of the left eye for over 2 weeks as stated by his parents. Initially, he did have some vision, as he could walk by himself, but a special ophthalmologic examination was not performed. DIAGNOSES: Cranial computed tomography and magnetic resonance imaging revealed a large tumor accompanied by peripheral bone destruction in the left pterygopalatine fossa that extended to sphenoid, ethmoid, left maxillary sinuses, left nasoethmoid, and left orbit. The optic nerve was invaded on both sides. Chest and abdominal imaging were normal. A primary diagnosis of Langerhans cell hyperplasia was made. However, blood tests on the second day of hospitalization revealed significantly elevated serum alpha-fetoprotein levels. On the third day, the boy lost his eyesight, with loss of pupillary and no light sensation during flashlight stimulation on both sides. INTERVENTIONS: Nasal endoscopy was performed on the fourth day, the vast majority of soft tissue mass was resected for biopsy. Histopathological examination revealed features of endodermal sinus tumor. A final diagnosis of primary YST of pterygopalatine fossa was made. Because the mass could not be resected completely, he received combined chemotherapy with bleomycin, etoposide, and carboplatin for 6 cycles over six months. OUTCOMES: The patient recovered with significant tumor shrinkage and without secondary metastasis after 18 months but left permanently blind. CONCLUSION: The worst complication of loss of vision after Primary YST of pterygopalatine fossa alerts us that close physical examination during the initial investigation should be performed, which is especially important in young children who cannot express complaints well. Early detection and treatment with surgical resection and chemotherapy may contribute to satisfactory outcomes and avoidance of visual impairment.

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.

Benign leiomyoma with multiple metastases to vertebrae and calvarium: An index case with comprehensive review of endocrine targets.

"Benign" metastatic leiomyomas (BML) are indolently growing metastatic tumors which mostly associate with uterine leiomyomas in women in reproductive ages. The reason to define these lesions as "benign" despite metastasis is their pathological features with low mitotic counts, lack of or minimal nuclear atypia, pseudocyst formation, and coagulative necrosis unlike leiomyosarcomas. Despite lack of pathological malignant features, they may cause significant morbidity and even mortality. Here, we describe a BML case with metastases to vertebrae and skull bones. Vertebral and skull metastases of BMLs were very rarely reported. In treatment of these tumors, hysterectomy and GnRH modifier treatments are widely employed. GnRH agonists act by desensitization and downregulation of the GnRH receptors, while GnRH antagonists act via the canonical competitive blockage. These treatments reduce FSH and LH levels, thereby reducing the systemic levels of sex steroids which stimulate leiomyoma growth. However, leiomyomas inherently harbor aromatase activity and synthesize their own estrogen; hence, treatment with systemic estrogen antagonists may provide better tumor control. Another important factor in BML pathogenesis is progesterone, and both progesterone receptor antagonists and high-dose progesterone receptor agonists may reduce BML growth. Following surgical treatment of the calvarial mass and radiotherapy of the vertebral metastatic foci, our BML case was successfully managed with hysterectomy and anastrozole treatment. Higher awareness of BML cases and their molecular endocrinological features in the neurosurgical community may pave to develop better strategies for treatment of these tumors causing high morbidity.

Adjuvant electrochemotherapy after debulking in canine bone osteosarcoma infiltration.

Osteosarcoma is a bone cancer considered rare to humans, but common in dogs. Dogs and humans share genetic homology and environmental risk factors. Improving the treatment of osteosarcoma in dogs could also be relevant to improve procedures in humans. Traditional treatments of osteosarcoma involve surgery and chemotherapy. Such treatments are commonly aggressive and not possible for many patients. Electrochemotherapy emerges as a minimally invasive, effective, and safe treatment alternative. Electrochemotherapy combines applications of high-intensity electric fields during short periods with anti-cancer drugs to improve its medicine cytotoxicity. Analyzing the electric field distribution, as well as electric current density, are essential to electrochemotherapy success. This paper brings the first case of a canine osteosarcoma treatment performed with bleomycin and electrochemotherapy. We performed in silico studies with finite element method software to observe the electric field distribution. In silico experiments help to verify possibilities and limitations of treating bone destruction and macro or micro tumor infiltrations around the primary tumor mass. Results show that both needle or plate electrodes are feasible to remove the tumor even with invasion into the bone. Plate electrodes perform well in treating micro infiltrations when associated with conductive gel and direct contact between electrode and bone (without soft tissues). Needle electrodes are effective in treating tumor infiltration on external cortical bone. Multiple applications are needed to cover all cranium layers with sufficient electric field intensity. Electrochemotherapy protocol with needle or plate electrodes does not present sufficient electric current density capable of affecting brain tissue, even in cases of bone destruction.

Primary Advanced Squamous Cell Carcinoma of the Temporal Bone: A Single-Center Clinical Study.

OBJECTIVES/HYPOTHESIS: The extreme rarity of temporal bone squamous cell carcinoma (TB-SCC) has delayed the accumulation of high-quality clinical evidence. For the purposes of retrospective meta-analysis in the future, a large dataset with information from various institutions would be ideal. Our objective here was to retrospectively review cases of TB-SCC encountered at a single tertiary referral center and explore survival outcomes and prognostic factors. STUDY DESIGN: Retrospective chart review. METHODS: The medical records of all TB-SCC cases were retrospectively reviewed. The resulting dataset contained 71 cases of primary cancer eligible for initial definitive (curative) treatment. RESULTS: T4 status was associated with lower disease-specific 5-year survival than T1 to T3 staging (T1: 100%, T2: 92%, T3: 86%, T4: 51%). Survival was significantly higher in operable than in inoperable cases, even when restricted to advanced (T3/T4) cancers. The tumor extension to the middle ear cavity was observed in 13/17 of T3 cases, but it was not associated with poor survival. In addition, among operable cases, negative surgical margins were associated with significantly higher survival than positive margins. CONCLUSIONS: Definitive treatments can offer disease-specific 5-year survival of over 85% in T1 to T3 cases of TB-SCC. The tumor extension to the middle ear cavity is not associated with poor survival. T4 status, inoperability, nodal invasion, and positive surgical margin are identified as a predictor of poor prognosis. Still, the matter of how to deal with unresectable tumors remains an outstanding issue in the treatment of TB-SCC. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E583-E589, 2021.

Primary intraosseous meningioma of the calvarium: A systematic review.

BACKGROUND: Primary intraosseous meningiomas (PIM) of calvarial origin are a small subset of meningiomas that arise from and occur within the calvarial bone. Its definition is often confused with other forms of non-dural based intracranial meningiomas, which has made previously published retrospective reviews heterogenous, non-specific and sometimes inaccurate. We present a systematic review of calvarial PIM. METHODS: Using a systematic search protocol that included databases such as PubMed, Web of Science and Embase, we extracted all human studies on PIM published from inception to December 2017. This systematic review includes case reports and retrospective reviews that specifically described PIM. RESULTS: On review of 166 articles identified with the systematic search protocol, 69 articles were analyzed. These comprised of 64 case reports, 1 case series and 4 retrospective reviews. 111 patients with PIM of calvarial origin were analyzed, 58 % of which were females and 42 % males. The mean patient age was 51 years and the frontal bone was the most common tumor location, occurring in 26.1 % of the cases. Surgical resection was the predominant modality of treatment in 97.2 % of the cases, and gross total resection was achieved in 84 % of cases that reported extent of resection. There were no recurrences for grade I meningiomas. However, all grade III meningiomas recurred and 33.3 % of grade II meningiomas showed recurrence with a mean postoperative follow-up interval of 20 months. Statistical analysis using Fisher's exact test showed the recurrence rate to be strongly associated with WHO tumor grade (p-value <0.001). CONCLUSION: There is statistically significant increased recurrence rate for calvarial PIM of higher grades, and we recommend close follow-up in those cases. Surgical resection remains the overwhelming treatment of choice for calvarial PIM, and it has a high gross total resection rate and low risk of complications and mortality.

Temporal Bone Chondroblastoma: Systematic Review of Clinical Features and Outcomes.

OBJECTIVE: Chondroblastoma is an uncommon benign neoplasm of cartilaginous origin usually involving the long bones. The temporal bone is a rare location for this tumor. The clinical profile, optimal medical and surgical management, and outcomes of treatment for temporal bone chondroblastoma remain unknown. METHODS: We performed a systematic review of the SCOPUS, PubMed, and CENTRAL databases for case reports and case series on patients with histopathologically proven temporal bone chondroblastoma. Data on demographics, clinical manifestation, surgical management, adjuvant treatment, and outcome on last follow-up were collected. RESULTS: A total of 100 cases were reported in the literature, including one described in the current study. The mean age of patients was 42.3 years (range, 2-85 years), with a slight male predilection (1.3:1). The most common clinical manifestations were otologic symptoms (e.g., hearing loss [65%], tinnitus, and otalgia) and a palpable mass. Surgical excision was performed in all cases, with gross total excision achieved in 58%. Radiation therapy was performed in 18% of cases, mostly as adjuvant treatment after subtotal excision. There were no deaths at a median follow-up of 2 years. Among the patients with detailed status on follow-up, 58% had complete neurologic recovery, 38% had partial recovery, and 4% had progression of symptoms as a result of tumor recurrence. CONCLUSIONS: Temporal bone chondroblastoma has a distinct clinical profile from chondroblastoma of long bones. Surgery is the mainstay of treatment, and radiation therapy may be given after subtotal excision. Outcomes are generally favorable after treatment.

Epithelioid and spindle cell rhabdomyosarcoma with FUS-TFCP2 or EWSR1-TFCP2 fusion: report of two cases.

The WHO Classification of Tumors of Soft Tissue and Bone divides rhabdomyosarcoma (RMS) into alveolar, embryonal, pleomorphic, and spindle cell/sclerosing types. Advances in molecular diagnostics have allowed for further refinement of RMS classification including the identification of new subtypes. Very rare RMS with epithelioid and spindle cell morphology, female predominance, marked osseous predilection, ALK expression, EWSR1/FUS-TFCP2 gene fusions, and highly aggressive clinical behavior have recently been recognized with only 23 cases reported in the English language literature. Herein, we report two additional cases with detailed clinicopathologic description and molecular confirmation. In brief, two young women presented each with a primary bone tumor-one with a frontal bone tumor and another with an osseous pelvic tumor. Both tumors showed epithelioid to spindle cell morphology, ALK expression, and EWSR1/FUS-TFCP2 gene fusions. Both patients died of disease less than 17 months from diagnosis despite administration of multiple lines of aggressive treatment. In addition, we review the literature and discuss differential diagnostic and potential treatment considerations.

Treatment-related calvarial lesions in pediatric brain tumor survivors.

BACKGROUND: Despite improved survival, many pediatric brain tumor survivors receiving radiation therapy (RT) experience late effects. PROCEDURE: To study calvarial lesions in this population, we retrospectively reviewed records of patients undergoing neurosurgical evaluation for calvarial bone lesions detected in posttreatment follow-up imaging at St. Jude Children's Research Hospital. Primary tumor diagnosis, treatment, imaging, surgical intervention, and histopathology from patients with radiographic evidence of lesions followed for ≥2 years post-RT were studied. RESULTS: For 17 patients with 18 index lesions, median time to lesion manifestation was 2.34 years. Medulloblastoma patients developed lesions at a shorter interval from RT than ependymoma patients (P = .05). Twelve of 14 lesions requiring surgery were benign fibro-osseous or sclerotic. Two malignant lesions distinct from the primary tumor had genetic predisposition to malignancy. CONCLUSION: Most calvarial lesions arising post-RT are benign and fibro-osseous. Serial imaging is recommended, and high index of suspicion for malignant lesions is warranted for patients genetically predisposed to cancer.

Glomus Tumor of Sella Turcica With Synaptophysin Expression Mimicking Pituitary Adenoma.

Glomus tumor can rarely arise in the central nervous system as a sella turcica mass. In this article, we report a case of sellar glomus tumor in a female patient who presented at the age of 8 years with visual impairment. The tumor recurred at 4 years and 26 years after initial excision and gamma knife therapy. Histologic examination showed a monotonous population of oval cells accompanied by delicate blood vessels, features mimicking pituitary adenoma. The tumor showed histologic progression at the second recurrence. Synaptophysin staining was positive, but chromogranin and CD56 were negative. The tumor cells were negative for epithelial markers but expressed actin and SMA. Awareness of the rare occurrence of glomus tumor at this region, careful analysis of morphology, and appropriate immunohistochemical workup are essential to solve this diagnostic challenge. The clinicopathologic features of all previously reported cases are reviewed.

Intracranial Metastases from Prostate Carcinoma: Classification, Management, and Prognostication.

BACKGROUND: Prostate carcinomas rarely metastasize to the central nervous system but, when they do, dural localizations are as common as and far more aggressive than intraparenchymal ones. Those metastases can be further classified according to their extension toward the subdural or extradural space and can frequently simulate other pathologic conditions including chronic subdural hematomas, abscess, and primary bone tumors. Beside the challenges of the preoperative differential diagnostic and complexity of surgical planning and operative excision, subdural metastases seem to carry a much poorer prognosis. METHODS: A series of consecutive patients admitted during a 12-year period through our oncall pathway for subdural/extradural collections or intraparenchymal lesions found, on histologic analysis, to represent intracranial prostate cancer metastases was retrospectively reviewed. RESULTS: A total of 19 patients were included, but only 3 were diagnosed with small cell prostate carcinoma, while the majority had a primary prostate adenocarcinoma. Metastases could be classified as pure subdural space lesions, dural-based lesions, extradural/bony lesions, and pure intraparenchymal lesions. All patients with subdural metastases and 3 out of 5 patients with dural-based lesions required an emergency intervention due to rapidly deteriorating neurologic status. The mean follow-up in our series was 37 months; only subdural localizations had a remarkably unfavorable outcome. CONCLUSIONS: Supported by our experience and the review of the literature, we suggest that a low threshold for contrast-enhanced computed tomography/magnetic resonance imaging is advisable in case of suspicious subdural collection, even in an emergency setting, for patients with previous medical history of prostate cancer.

Primary osteosarcoma of the skull in teenager.

Osteosarcoma of the skull is a rare primary malignant bone tumour in children, representing 1-2% of all cranial tumours. We describe a case of a 17-year-old adolescent with chondroblastic osteosarcoma of the parietal-occipital bone and no distant metastases at presentation treated with neoadjuvant chemotherapy, surgery and, later, concurrent chemoradiotherapy. The patient suffered progressive disease and died 15 months after diagnosis. There are at least 15 paediatric cases of osteosarcoma of the skull described in the literature. Due to its rarity, there are no broad prospective studies on this entity, which has distinctive features when compared to other craniofacial tumours, carrying a worse prognosis. Complete surgical resection is needed for long-term survival, whereas chemotherapy and radiotherapy have still questionable indications.

Primary temporal bone chondrosarcoma: experience with 10 cases.

Background: Temporal bone chondrosarcoma (TBC) are uncommon primary temporal bone malignancies, and clinicians lack experience in its diagnosis and treatment. The optimal management of patients with tumor of TBC also remains a topic of debate and controversy. Objectives: This article summarizes the experience of diagnosis and treatment of TBC, in order to improve the quality of life of cancer patients in the future. Material and methods: We conducted a retrospective analysis of 10 patients who were referred to our hospital from June 2009 to June 2018 for the treatment of TBC. Results: There were 3 males and 7 females. The most common presenting symptoms were facial paresis (50%) and hearing loss (40%), whereas otalgia (10%), vertigo (10%) and headache (10%) were less common. All tumors originated from the temporal bone, and 80% involved the jugular foramen area. All patients survived without evidence of disease at a median time of follow up of 28.8 months. Conclusions and significance: TBC mostly originated in the middle ear mastoid area, and easily extended to the jugular foramen area. An individualized surgical procedure that removes tumors integrally with minimal nerve and blood vessel damage provides long-term cancer control and minimal morbidity in most cases.

Plasmacytoma of the skull mimicking an epidural hematoma: A case report.

RATIONALE: Plasmacytoma as the 1st presentation of skull tumors is a rare disorder. When it is combined with brain trauma or dramatic changes in intracranial pressure, patients are more prone to misdiagnosis. PATIENT CONCERNS: A 67-year-old woman complaining of a headache presented with a history of head trauma for the past 1 hour. Emergency head computed tomography initially suggested an epidural hematoma. DIAGNOSIS: Emergency surgery was performed to remove the intracranial hematoma, but a tumor-like mass was found during surgery, and pathologic assessment confirmed plasmacytoma. Surgery was difficult because of bleeding. The tumor was radically removed. INTERVENTIONS AND OUTCOMES: The patient underwent whole-brain radiotherapy and chemotherapy. She died 40 months after the surgery. LESSONS: Epidural lesions found after a head injury may be assumed to be an epidural hematoma, leading to unnecessary surgery. Diseases such as hematomas, meningiomas, eosinophilic granulomas, bone metastases, and osteosarcomas must be considered.