Epidemiological Profile of 96 Intracranial Tumors Treated in a Single Reference Center

Objectives The present study aims to categorize the prevalence of intracranial tumors surgically treated at the neurosurgery service of Hospital Universitário Evangélico Mackenzie (HUEM) between 2016 and 2018. Material and Methods This survey included patients surgically treated due to primary or metastatic intracranial neoplasia between 2016 and 2018 at a referral center in the city of Curitiba. These patients were analyzed for epidemiological, histopathological, and topographic data, and they underwent an assessment of the outcome at the time of hospital discharge. Results Atotal of 96patientsmet the inclusion criteria. Themost prevalent tumorwas the glioma, with 39.6% of the sample, with glioblastoma being themost prevalent histological type. Brainmetastases andmeningiomas represented, respectively, 21.9%and 18.8%of the total. There was a predominance of supratentorial and intra-axial tumors in our sample. Conclusion Glioma was the most commonly found tumor, directly associated with high morbidity and mortality. The development of new and more effective drugs with action directed at themolecular level of intracranial tumorsmay be the path to a longer survival and improvement in the quality of life of these patients.

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.

Low-Grade Papillary Schneiderian Carcinoma of the Sinonasal Cavity and Temporal Bone.

OBJECTIVES:: The aim of this study was to further characterize a newly described neoplasm, low-grade papillary Schneiderian carcinoma, occurring simultaneously in the sinonasal cavity and mastoid. Additionally, the authors review the only 2 similar cases within the literature and describe the common clinical features, radiographic findings, and pathologic characteristics of this exceptionally rare disease process. METHODS:: Chart review for single patient, review of literature. RESULTS:: The patient presented with bilateral nasal obstruction. Computed tomography revealed a left sinonasal mass with skull base hyperostosis, and follow-up magnetic resonance imaging showed a concomitant olfactory groove meningioma. Examination showed a bilateral, completely obstructing sinonasal mass with skip areas, and biopsy confirmed inverted papilloma (human papilloma virus strains 16 and 18 indeterminate). The patient underwent bilateral endoscopic sinus surgery, left medial maxillectomy, and left partial nasopharyngectomy. Given her multifocal disease, she was advised that she would require additional excision, but was lost to follow up. One year later she developed acute left facial paralysis. Magnetic resonance imaging demonstrated an enhancing mass in the left mastoid with enhancement along the Eustachian tube in addition to her known recurrent sinonasal disease. Simultaneous endoscopic sinus surgery and mastoidectomy were performed. Polypoid tissue was removed from the nasopharynx, mesotympanum, epitympanum, and retrofacial air cells. Immunohistochemistry showed that cells stained positive for p63 and dermCK and negative for synaptophysin. Morphologically, cells were bland, without classic stromal invasion, retaining their smooth, cystic, and papillary features, despite their increased depth within the tissue. Upon further review and consultation with an outside pathologist, a diagnosis of low-grade papillary Schneiderian carcinoma was made. The patient was referred for radiation therapy and is disease free at 3-month follow-up, with return of her facial function. CONCLUSIONS:: This case represents the first report of concurrent low-grade papillary Schneiderian carcinoma of both the nasal cavity and mastoid. It emphasizes the importance of recognizing this new entity through pathologic analysis and suspecting it when the clinical course does not follow an expected pattern.

Rare Case of Convexity Meningioma Growing into Arachnoid Cyst.

BACKGROUND: Meningioma growing into an arachnoid cyst is an extremely rare event. Only 3 cases are reported in the literature. In 2 of them, an operative procedure in or near the arachnoid cyst preceded tumor growth. CASE DESCRIPTION: We report a case of a patient requiring marsupialization of an arachnoid cyst of the middle cranial fossa. On follow-up, 3 years postoperatively he showed no signs of recurrence or tumor growth. One year later, the fourth year after surgery on the cyst, he presented with large tumor growth into the former cyst's cavity. Pathologic workup after resection revealed an atypical meningioma (World Health Organization grade II). CONCLUSIONS: We discuss the possible pathogenesis in light of the scarce published literature, as well as the differential diagnosis of this rapidly growing tumor.

Sellar solitary plasmacytoma progressing to multiple myeloma: a case report and literature review.

Sellar plasmacytoma is a rare cause of sellar lesions. Preoperative diagnosis remains a challenge. We present a 34-year-old Chinese woman with a 25-day history of headache and diplopia. A physical examination revealed incomplete left abducens nerve palsy. The initial diagnosis was invasive pituitary adenoma. The patient's condition deteriorated suddenly the day before the arranged operating date, with the hemoglobin level declining from 113 to 70 g/L. The operation was cancelled and further studies confirmed the diagnosis of sellar solitary plasmacytoma that progressed to multiple myeloma. After undergoing radiotherapy, high-dose chemotherapy, and autologous peripheral blood stem cell transplantation, complete remission was achieved on 4 years follow-up. We reviewed the pertinent literature and reached the following conclusions: sellar plasmacytomas with development of multiple myeloma on follow-up more likely happened in men than in women; and if the sellar plasmacytoma does not compress the cranial nerve, transsphenoidal resection should be cautious because the systemic treatment with radiotherapy, chemotherapy, and autologous peripheral blood stem cell transplantation may be more effective with little invasion.

Primary intraosseous cavernous hemangioma of the zygoma: a case report and literature review.

Intraosseous hemangiomas are rare. We report the case of a 47-year-old man who presented with a gradually enlarging left zygomatic mass that had caused pain, deformity, and superficial soft-tissue swelling. Computed tomography revealed a well-circumscribed 2.0 × 2.5-cm mass with a ground-glass matrix in the left zygoma. Following surgical excision, the patient's symptoms resolved. Findings on pathologic examination of the excised tissue were consistent with an intraosseous cavernous hemangioma. We describe the features of this rare case, we discuss the pertinent radiologic features and pathophysiology of intraosseous hemangiomas, and we review the available literature.

Melanotic neuroectodermal tumor of infancy (MNT1) arising in the skull. Short review of two cases.

INTRODUCTION: Melanotic neuroectodermal tumor of infancy (MNT1) is a rare congenital pigmented neoplasm of neural crest origin, locally aggressive, and rapidly growing that develops during the first year of life. It most commonly arises from the maxilla, the cranial vault, and the mandible. Early diagnosis and radical surgery are critical for a long-term outcome. METHODS: A literature search through PUBMED revealed 43 cases of MNT1 arising in the skull. We reviewed the available literature and studied the presenting symptoms, diagnostic procedures, treatment, rates of recurrences, malignancy, and data of follow-up. We report two further cases of infants aged 4 and 10 months, respectively, with MNT1 arising from the cranial vault who underwent radical excision of the lesion. CONCLUSION: Melanotic neuroectodermal tumor of infancy should be included in the differential diagnosis of skull lesions in infants. Radical surgery must be considered as the treatment of choice and close follow-up for at least 2 years is necessary.

Nasoseptal flap reconstruction of high flow intraoperative cerebral spinal fluid leaks during endoscopic skull base surgery.

BACKGROUND: Over the past 10 years, significant anatomic, technical, and instrumentation advances have facilitated the exposure and resection of intradural lesions via a fully endoscopic expanded endonasal approach (EEA). The vascularized nasoseptal flap (based on the posterior nasoseptal artery) has become our primary endoscopic reconstructive technique. The goals of this study are to prospectively evaluate the nasoseptal flap and high-risk cerebral spinal fluid (CSF) leak variables. METHODS: Prospective evaluation was performed of EEA patients with intraoperative high-flow leaks (either a cistern or ventricle open to nasal cavity during tumor dissection) who underwent nasoseptal flap reconstruction. RESULTS: Seventy consecutive nasoseptal flaps for high-flow intraoperative leaks were evaluated prospectively by the primary author. Twelve risk factors were then graded at the time of the operations and correlated to CSF leak outcomes. The overall postoperative CSF leak rate was 5.7% (4/70). All four postoperative leaks were successfully managed with endoscopic repair and CSF diversion. A multivariate analysis of all 12 risk factors is detailed. Pediatric patients, large dural defects, and radiation therapy were noted to be factors in reconstructive failure. One flap death occurred in a patient with prior surgery and proton therapy, this leak was managed with a temporoparietal flap and endonasal repair. CONCLUSION: The nasoseptal flap is an excellent anterior skull base reconstructive technique. Patients with high-flow intraoperative CSF leaks had a 94% successful reconstruction rate. Patients with skull base proton radiation therapy are at higher risk for flap failure and preparation for nonradiated tissue reconstruction should be discussed with the patient.

Temporal fossa hemangiopericytoma: a case series.

OBJECTIVE: Review clinical experience with temporal fossa hemangiopericytomas (HPCs). STUDY DESIGN: Retrospective case series review. SETTING: Tertiary referral center. PATIENTS: Intracranial HPCs within the temporal fossa. INTERVENTIONS: Craniotomy for either subtotal or gross total tumor excision. MAIN OUTCOME MEASURES: Determination of clinical outcome (alive with no evidence of disease, alive with disease, and died of disease). RESULTS: Five cases of HPC involving the temporal fossa were treated at our tertiary referral center for the period from 1995 to 2008. All but 1 patient were men. The age of presentation ranged from 31 to 62 years, and duration of follow-up ranged from 8 to 153 months. Clinical presentation was protean; headache was the most common symptom. Gross total tumor excision was achieved in 2 patients, whereas subtotal tumor excision was achieved in 3 patients. Reasons for subtotal resection included excessive intraoperative blood loss and inextricable tumor. Histologically, all tumors were composed of tightly packed, randomly oriented (jumbled-up) tumor cells with little intervening collagen. CD34 staining mostly highlighted the vascular background. One patient died of disease, 2 patients were alive with disease, and 2 patients had no evidence of disease. CONCLUSION: Management of temporal fossa HPC is challenging because clinical presentation is often late, and extent of tumor excision is constrained by vital structures in the cranial base and intracranial contents. A multidisciplinary approach with neurosurgery and neurotology undertaken to achieve the most complete tumor resection possible, whereas minimizing morbidity are likely to confer a longer period of symptom-free survival and improves curability of these difficult lesions.

Benign fibrous histiocytoma of the skull with increased intracranial pressure caused by cerebral venous sinus occlusion.

The authors present a very rare case of benign fibrous histiocytoma of the skull with increased intracranial pressure caused by sinus occlusion. A 33-year-old woman was referred for investigation of a right occipital protrusion with tenderness and double vision. She had only mild divergence insufficiency and bilateral papilledema neurologically. Imaging findings showed that the skull tumor was located at the right occipital bone with bone disruption and a compressed right sigmoid sinus. When planning the resection, caution was required to spare the collateral flow so as to manage the intracranial pressure. Immunohistochemical analysis showed that the tumor was positive for CD68, alpha1-antichymotrypsin, and alpha1-antitrypsin. From these findings, the tumor was diagnosed as a primary benign fibrous histiocytoma of the skull.

Occipital condyle syndrome as the first sign of metastatic cancer.

BACKGROUND: Occipital condyle syndrome is characterized by severe, unilateral, occipital headache and ipsilateral twelfth-nerve palsy. It is associated with skull-base metastasis. CASES: We identified two patients with sub-acute onset of severe, unilateral, occipital headache and ipsilateral tongue paralysis. The first patient was a 58-year-old woman with a history of limited stage small-cell lung cancer in clinical remission. The second patient was an otherwise healthy 36-year-old man. Neither patient had any other findings on general medical or neurological examination. One patient had only equivocal findings on initial magnetic resonance imaging (MRI), and the other patient's MRI was normal. Although initial work-up for metastatic disease was normal, the first patient developed severe bone pain over the next few months, and follow-up investigations demonstrated metastases to her spine, tibia, skull base and brain. The second patient improved initially, but was admitted to hospital three months later with constitutional symptoms and pancytopenia. Bone marrow and lymph node biopsies were consistent with Stage IVB Hodgkin's lymphoma. CONCLUSION: Occipital condyle syndrome can be the first presentation of disseminated malignancy. Initial imaging of the brain and skull base may be normal, and recognition of this syndrome warrants thorough investigation and close follow-up.

Audiovestibular evolution in a patient undergoing surgical resection of a temporal bone myxoma.

Primary myxoma in the head and neck region occurs mostly in the maxilla and mandible, and rarely in the temporal bone. A 32-year-old female patient with temporal bone myxoma manifested as acute vertigo, headache, and tinnitus on the right ear. Audiometry and auditory brainstem response revealed normal responses, bilaterally. Vestibular function test displayed spontaneous nystagmus beating toward the left side. Absent ice water caloric response was disclosed on the right ear, whereas vestibular evoked myogenic potential test showed normal responses, bilaterally. MRI scan demonstrated a well-enhanced mass at the anterior middle portion of the right temporal bone with intracranial extension. Tumor excision via craniotomy was performed, and the histopathological study confirmed as myxoma. One year after operation, follow-up audiovestibular function tests revealed normal responses, except for 23 dB conductive hearing loss on the right ear.

Management of the large cranial osteoma: experience with 13 adult patients.

BACKGROUND: Large osteomas are benign, slow-growing and rare neoplasms of the skull, which are usually asymptomatic but may need surgical resection. PATIENT AND METHODS: We reported a series of 13 adult patients who had large cranial osteomas and who underwent surgical treatment over a period of 5 years. All of the patients were male and the mean age was 21.8 years. FINDINGS: Craniectomy associated with cranioplasty was performed in 10 patients and drilling of the bone tumor was performed in 3 patients. Tumour regrowth was not observed in any patient. CONCLUSION: Although most of the cranial osteomas are asymptomatic, surgical treatment is indicated for large ones. Each patient must be individualized and the selection of the type of surgery depends on the shape and growth pattern of the osteoma.

Skull metastasis from ampulla of Vater adenocarcinoma: case report.

We report a rare case of skull metastasis from ampulla of Vater adenocarcinoma in a patient with controlled primary neoplasm. This 65-year-old Korean man presented with headache and painful parietal scalp swelling, but he did not show any neurological deficits. Computed tomography revealed an osteolytic mass that invaded and expanded through the skull tables in full-thickness. On magnetic resonance images following Gadolinium injection, the mass appeared slightly hypointense signal on all sequences and intense heterogeneous enhancement. He underwent surgical excision with artificial grafting of calvaria and dura. Postoperative radiation therapy was ensued for metastatic adenocarcinoma, and he was well for the follow-up period of 3 months. To the best of our knowledge, this is the first such case that involved the skull.

Button osteoma: its etiology and pathophysiology.

The present study investigates a circumscribed bony overgrowth on the cranial vault, known as button osteoma (BtO) and referred to here as button lesion (BtL). We discuss its anthropological implications. Data on its histology, location, and population distribution (by age, race, and gender) are provided. Microscopically, BtL is composed of well-organized dense lamellated bone which is poorly vascularized and with very few osteocytes. It forms a dome-shaped roof over an underlying diploeized area which includes the ectocranial table. The frequency of BtL is similar in modern (37.6%) and archaeological (41.1%) populations, in blacks, whites, males, and females, and correlates with age. It is rare in nonhuman primates. Fifty-five percent of the human skulls studied by us had BtL only on the parietal, 23.6% on the frontal, and 3.6% on the occipital bones. Fifteen percent had BtL on both the frontal and parietal bones. No lateral preference was found. Most skulls with BtL (64.1%) had only one lesion, 20.4% had two BtL, and 15.4% demonstrated multiple BtL. The average number of button osteomas on an affected skull was 1.97. The frequency of large osteomas (0.5-1.0 cm) was similar in young and old age groups. The demographic characteristics of BtL, mainly its high frequency among ancient and modern populations, its independence of sex and race, its scarcity in other primates, and the fact that its macro- and microstruture are indicative of an hamartoma (and not an osteoma or exostosis) suggest an evolutionary background to the phenomenon.