Long-Term Survival after Primary Ewing's Sarcoma of the Skull with Intracranial Extension.

BACKGROUND AND OBJECTIVE: Primary Ewing's sarcoma of the skull is a very rare malignant neoplasm, predominantly occurring in children and adolescents. We describe here the clinical, neuroradiologic, and histopathologic features of a patient with primary Ewing's sarcoma of the skull and discuss the standards of therapy for this type of tumor. CLINICAL PRESENTATION: This 18-year-old male patient presented with a primary Ewing's sarcoma of the skull, involving the dura of the frontal and parietal lobes of the left cerebral hemisphere. He was treated with gross total surgical excision of tumor, skull reconstruction, chemotherapy, and irradiation. Twelve years after the surgery, the patient has no evidence of local recurrence or distant metastases. Radical surgical excision of the primary tumor with safety margins is thought to play a role in the favorable clinical course. CONCLUSION: The presented case is the longest surviving patient after treatment of primary Ewing's sarcoma of the skull bone. This rare type of tumor may allow better survival rates under adequate management than sarcoma elsewhere in the body.

Lesiones de cráneo en el mieloma múltiple / Skull Lesions in Multiple Myeloma

Fig A) Radiografía de cráneo en proyección lateral: se observan múltiples lesiones radiolúcidas distribuidas en todo el cráneo. B) Acercamiento donde se evidencian múltiples lesiones en sacabocado, compatibles con mieloma múltiple(AU)

Endoscopic Approach for Resection of Frontal Forehead Osteoma: Technical Case Report Instruction.

Forehead osteomas are benign bone tumors. They are frequently associated with exophytic growth in the outer table of the skull, causing cosmetic disfigurement of the face.1-9 The objective of this study was to present the efficacy and feasibility of the endoscopic treatment of forehead osteomas by presenting a case report with details of the surgical technique. A 40-year-old female patient presented with aesthetic complains of a progressing bulge in the forehead. A computed tomography scan with 3-dimensional reconstruction showed bone lesions on the right side of the forehead. The patient underwent surgery under general anesthesia with no noticeable incision, which was planned 2 cm behind the hairline in the midline because the osteoma was close to the midline plane on the forehead (Video 1). A retractor coupled with a 4-mm channel for endoscopy and a 30-degree optic was used to dissect, elevate the pericranium, and locate the 2 bone lesions in the forehead. The lesions were removed using a chisel, endoscopic facelifting raspatory, and a 3-mm burr drill. The tumors were resected completely, resulting in good cosmetic outcomes. The endoscopic approach for treating forehead osteomas is less invasive and facilitates complete removal of tumors, which results in good cosmetic outcomes. Neurosurgeons should consider and add this feasible approach to enhance their surgical armamentarium.

Combined endoscopic endonasal & transoral approach to transpatial lesion involving the infratemporal fossa and masticator space: A case study and literature review / Abordaje endoscópico combinado endonasal y transoral a una lesión transespacial involucrando la fosa infratemporal y el espacio masticador: Reporte de caso y revisión de la literatura

Transpatial skull base lesions involving the infratemporal fossa (ITF) are challenging due to the complex neurovascular structures of the region. Open approaches have traditionally been utilized to access these spaces. We present a 55-year-old woman presented with a mesenchymal mass involving the left ITF and masticator space. A combined endoscopic endonasal transpterygoid approach was performed followed by an endoscopic transoral-transmandibular corridor to access and resect the tumor. The post-operative course was unremarkable with no recurrence during her follow-up. Combined endoscopic approaches for transpatial tumor resection offered sufficient exposure to access safely each space (AU)

Las lesiones de la base craneal con una distribución transespacial e involucrando la fosa infratemporal (ITF) representan un desafío debido a las complejas estructuras neurovasculares de dicha región. Los abordajes abiertos han sido empleados tradicionalmente para acceder a estas regiones. Presentamos el caso de una mujer de 55 años que consultó por una lesión mesenquimal localizada en la ITF izquierda y en el espacio masticatorio. Se realizó un abordaje combinado endoscópico transpterigoideo endonasal seguido de un abordaje endoscópico transoral-transmandibular para acceder y resecar el tumor. El curso postoperatorio fue anodino, y no ha presentado recidiva con 21 meses de seguimiento. Los abordajes endoscópicos combinados para la resección de lesiones tumorales, con una distribución transespacial involucrando la ITF, ofrecen una exposición suficiente para acceder de forma segura a cada espacio (AU)

Extradural hematoma from trauma to midline skull tumour.

We present a case of extradural hematoma resulting from a relatively minor closed injury over the vertex where a plasma cell tumour had invaded the superior sagittal sinus. The patient underwent an emergency craniotomy and evacuation of the hematoma. Hemostasis and prevention of recollection of the hematoma were hampered by the erosion of the sagittal sinus making its direct repair impossible. This was achieved by hitching up the dura lateral to the sinus to become its lateral wall reinforced by hemostatic agents. The patient made a full recovery. Malignant tumours invading the dural venous sinuses and eroding the skull can cause life-threatening intracranial bleeding after relatively minor trauma.

Giant Skull Tumor: A Benign Meningioma.

ABSTRACT: A 37-year-old male patient was complaining from painless forehead swelling, which started 5 years ago. Brain computed tomography scan and magnetic resonance imaging showed a large extradural mass compressing the both frontal lobes with skull bone infiltration, hyperostosis and enlargement. The patient was operated in 2 stages. In the first stage, the authors achieved separation for the tumor from the scalp and skull. In the second stage after 1 week, the authors continued with circumferential dural opening around the tumor and separation of the tumor from brain tissue. Duroplasty was performed by autologous fascia latta and skin flap was closed primarily. The specimen was received grossly as huge mass weighing 1530 g and measuring 39 × 16 × 12 cm. Histopathologic examination showed grade I meningioma extending beyond the skull to the surrounding soft tissue.

Pediatric Cases of Recurrent Skull Giant Osteoma Misdiagnosed as Fibrous Dysplasia.

ABSTRACT: Osteomas are benign mature bone tumors that typically arise in the skull. Osteomas larger than 3 cm in diameter are considered giant osteomas. Giant osteomas of the skull vault are very rare, especially in children; therefore, only a few cases have been reported in the literature. Although osteomas are usually asymptomatic, a large skull mass can cause headache, as well as esthetic disfigurement of the forehead. it can be misdiagnosed as other conditions, such as fibrous dysplasia, ossifying cephalhematoma, or other malignant bone tumors. Herein, the authors report 2 rare pediatric cases of giant osteomas mimicking fibrous dysplasia and their successful surgical excision. These cases showed good results without recurrence or complications on long-term follow-up after complete excision.

Primary Intraosseous Cavernous Hemangioma of the Cranium: A Systematic Review of the Literature.

Primary intraosseous cavernous hemangioma (PICH) is a rare, benign tumor of vascular origin, typically arising in the vertebral body. Its presence in the skull is exceedingly rare, with only a few cases being reported worldwide. We carried out the first systematic review of the literature, covering the epidemiology, clinical and imaging features, management, and prognosis of cranial PICH. The literature search revealed 51 studies with 77 patients; the mean age of the patients was 32.7 years with a female predominance of 1.4:1. The majority of cranial PICHs were located in the calvarium, primarily in the frontal and parietal regions, with only a few located in the skull base. The most common initial clinical manifestation was local growth or swelling, followed by a headache. Radiographically, PICHs represented osteolytic, intradiploic masses, which in many cases displayed trabeculations, leading to the so-called "honeycomb" or "starburst" pattern. After contrast administration, PICHs typically enhance. Tumor removal, with craniectomy or en bloc resection and subsequent skull reconstruction, was selected for calvarial PICHs, whereas a transsphenoidal approach, with only partial resection, was applied for clival/sella PICHs. Preoperative embolization, aiming to minimize intraoperative blood loss, was performed in the case of large tumors. At a mean follow-up of 39 months, no patient experienced tumor recurrence, even after subtotal resection. Owing to the benign nature of the tumor, maximal safe resection is recommended as the treatment of choice for patients with cranial PICH.

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)

Two-step treatment of a giant skull vault hemangioma: A rare case report and literature review.

Skull 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.

Endolymphatic Sac Tumour: A Case Report and Review of the Literature

Introduction Endolymphatic sac tumor (ELST) is a slow-growing, low-grade, locallyinfiltrative tumor arising from the endolymphatic sac/duct, which is located in the posterior part of the petrous temporal bone. It may be sporadic in origin, or may be associated with Von-Hippel Lindau (VHL) syndrome. Case description A 40-year-old female patient with an ELST without VHL syndrome who was treated successfully by microsurgical extirpation of the tumor. Discussion We discuss the radiological features and the histopathology of this rare tumor and review the relevant literature. Conclusion The case herein reported adds to the previously-reported cases of this rare tumor.

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.

Surgical Approaches to Tumors of the Occipito-Cervical, Subaxial Cervical, and Cervicothoracic Spine: An Algorithm for Standard versus Extended Anterior Cervical Access.

OBJECTIVE: To propose a surgical approach algorithm for the tumors of the cervicothoracic spine. METHODS: All patients operated for vertebral column tumors involving the occipito-cervicothoracic spine were reviewed. Oncologic characteristics and surgical approach were gathered. Approach was classified by the use of staging and trajectory (posterior, transnasal, transoral, transmandibular, transcervical, transsternal). Angle of attack was defined for the occipitocervical junction tumor as the angle inscribed by the inferior mandibular plane and line connecting the superior tumor pole and mandibular angle. For lesions extending below the thoracic inlet, angle of attack was that inscribed by the plane of the thoracic inlet and the line connecting the jugular notch and inferior tumor pole. RESULTS: In total, 115 patients were included (mean age 56.7 years, 64 [56%] male, average size 26.5 cm3, 39 [34%] primary tumors). Sixty-nine (60%) of patients had single-stage procedures (57 [49.6%] posterior-only, 12 [10.4%] anterior-only), 35 (30.4%) had 2-stage procedures, and 11 (9.6%) had 3- or 4-stage approaches. Lesions requiring a combined transmandibular-transcervical approach all involved the C2 and C3 levels and had a significantly steeper angle of attack (42.5 ± 9.5 vs. 6.1 ± 13.3°; P = 0.01) and greater superior tumor extent above the inferior plane of the mandible (3.69 ± 2.18 vs. 0.33 ± 0.78; P = 0.002). Lateral tumor extent, tumor size, nor inferior angle of attack differed significantly between approach groups. CONCLUSIONS: Here, we present a preliminary decision-making algorithm for the management of vertebral column tumors of the cervicothoracic spine. Based on this single-center experience, we suggest which patients, assessed via a combination of tumor histology and regional anatomy, may benefit from extended anterior surgical access.

Recurrent endometrial carcinoma metastasis to the temporal bone and skull base.

A 62-year old woman presented with a 1-month history of left otalgia, facial palsy and hearing loss. She had a background of non-insulin-dependent diabetes mellitus and stage 2 endometrial adenocarcinoma, treated 18 months ago. Computed tomography scan showed erosion of the skull base and temporal bone. She was referred to the otolaryngology team with a diagnosis of necrotising otitis externa. On clinical examination, there was an exophytic, necrotic lesion in the ear canal arising from the posterior canal wall. A subsequent magnetic resonance imaging scan showed a lesion located in the left jugular foramen extending into the middle ear, with characteristics consistent with a glomus jugulo-tympanicum. Interestingly, histology of the lesion showed malignant cells with immunohistochemical staining suggestive of an adenocarcinoma. This is the first reported case of metastatic endometrial carcinoma involving the jugular foramen and temporal bone. Although a diagnosis is rare, it is important to consider it when other differential diagnoses are not fitting. Imaging should always be interpreted with caution, correlating to the clinical findings.

Middle Ear and Temporal Bone Papilloma: A Clinicopathologic Study and Comprehensive Literature Review of 57 Cases.

Sinonasal papilloma (SP), formerly Schneiderian papilloma, represents a rare group of benign epithelial neoplasms, most commonly identified in the sinonasal tract, while less frequently identified in the pharynx, lacrimal sac, and middle ear. Within temporal bone sinonasal-type papilloma (TBSP), there seems to be a much higher recurrence and malignant transformation risk than those identified in the sinonasal tract. Based on this clinical report and a review of the cases reported in the English literature, 49% of the 57 cases developed in the setting of concurrent or antecedent sinonasal or nasopharyngeal SP. There is an equal sex distribution (26 females and 31 males), with a broad age range (19-81 years) at presentation (median 56 years; average 54 years). Three patients had bilateral disease. Symptoms include a mass lesion with hearing loss, otitis media, otorrhea, otalgia, and tinnitus, among others. Inverted SP was identified in 42 patients, oncocytic SP in six, and exophytic SP in four (undefined in the remainder). Recurrence was identified in 38 of 49 patients with follow-up (78%), often with multiple recurrences over time, with carcinoma developing in the temporal bone in 19 patients (33%), with males developing carcinoma by a 1.7:1 ratio over females. Surgery was the treatment of choice (radical mastoidectomy) with 6 patients (10%) dead of disease (median 30 months, mean 38 months), while 47 patients were alive at last follow-up: 31 without disease (mean 33 months); 7 with locally recurrent disease (mean 20 months); 9 patients alive but with unknown disease status; and 4 patients without follow-up. In conclusion, TBSP is frequently identified in the setting of concurrent sinonasal tract disease, showing similar histologic features to sinonasal tract counterparts. There is no sex predilection, with patients most commonly presenting in the sixth decade of life. Recurrences are common, with carcinoma developing much more frequently than in sinonasal tract papilloma (33%), but recognizing that carcinoma may be documented in either or both anatomic sites. Overall outcome is excellent, with long term clinical follow-up warranted to manage recurrence or malignant transformation.

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.

[Proposal for the delineation of postoperative primary clinical target volumes in ethmoid cancers]. / Proposition de délinéation des volumes cibles anatomocliniques postopératoires de la tumeur primitive des cancers de l'ethmoïde.

It is proposed to delineate the anatomo-clinical target volumes of primary tumor (CTV-P) in ethmoid cancers treated with post-operative radiotherapy. This concept is based on the use of radioanatomy and the natural history of cancer. It is supported by the repositioning of the planning scanner with preoperative imaging for the replacement of the initial GTV and the creation of margins around it extended to the microscopic risk zones according to the anatomical concept. This article does not discuss the indications of external radiotherapy but specifies the volumes to be delineated if radiotherapy is considered.

Primary osteosarcoma of the parietal bone.

Osteosarcoma is the most common primary bone tumor and usually involves the long bones. Osteosarcoma of the skull, on the other hand, is relatively rare. Here, we present a 29-year-old man with a growing mass in the skull he first noticed after a fall while skateboarding. The initial clinical diagnosis was hematoma. While undergoing an evacuation surgery for a hematoma, a suspicious mass was detected which was biopsied. Histopathological evaluation showed high-grade osteosarcoma. The patient was referred to our hospital where he underwent definitive resection followed by adjuvant chemotherapy. His course was complicated by wound infection. Even though osteosarcoma of the skull is a rare finding, it should be suspected in a patient with a skull mass, and the history of prior head trauma does not exclude the diagnosis.