Intracranially Extended Sinonasal Undifferentiated Carcinoma: A Case Report and Literature Review.

BACKGROUND Sinonasal undifferentiated carcinomas (SNUC) are highly malignant and rare lesions. Therapeutic efforts often provide frustrating results. Their course is characterized by indolent progression, until it culminates in extensive local infiltration of adjacent anatomical structures or cervical lymphadenopathy in approximately one-third of patients upon admission. It most frequently affects males, with a sex ratio of 3: 1. The age at manifestation tends to be about 40-50 years. CASE REPORT We report the case of a 41-year-old man with intracranial expansion of SNUC. Two previous sinus surgeries were performed endoscopically because the lesion at that moment was exclusively located endonasally. Within the last few months, he had been having persistent headaches. Magnetic resonance imaging (MRI) revealed an anterior cranial fossa lesion. Therefore, he underwent a bifrontal craniotomy and excision of the space-occupying lesion (SOL). The osseous defect of the skull base was covered with a titanium mesh. Finally, we performed a duraplasty using a pericranial flap and fat tissue taken from his abdomen. Postoperatively, his wound was dehisced. We proceeded then to a frontal craniectomy with surgical debridement, subgaleal empyem and epidural abscess removal, and copious irrigation with oxygen peroxide. Enterococcus spp. were isolated from pus cultures. Despite receiving bacteria-focused antibiotics, he unfortunately developed sepsis and died. The histopathologic findings revealed a SNUC, which is the criterion standard for diagnosis. CONCLUSIONS Multimodal treatment offers the best prognosis to patients with SNUC. Combined operations by otolaryngologists and neurosurgeons provide the necessary radicality. There is high risk of wound healing disorders, especially when local irradiation had been administered.

Patient-Assessed Outcomes following Temporal Bone Fractures.

The long-term impact of neurotological symptoms after a temporal bone fracture (TBF), including facial nerve palsy (FP), hearing loss, tinnitus, and dizziness on the quality of life of patients is often underevaluated. Thus, we retrospectively assessed 30 patients with TBF (26 men and 4 women) in our university tertiary referral center. They participated from injury onset to the final follow-up, over an 18-month period. Quality of life was estimated using validated questionnaires, such as the Facial Disability Index (FDI: physical and social), Hearing Handicap Inventory (HHI), Tinnitus Handicap Inventory (THI), and Dizziness Handicap Inventory (DHI). The FDI score was significantly worse in patients with severe initial (for physical FDI) and final facial palsy (for both physical and social FDI), mainly with immediate onset. The HHI score was statistically worse in patients with mixed hearing loss compared to those with conductive or sensorineural hearing loss and in those with profound hearing loss vs. normal hearing. The mixed TBF and the severity of hearing loss (especially profound hearing loss) were correlated with HHI, THI and DHI score values. In the long-term period after a TBF, moderate or severe facial palsy, mainly with immediate onset, may cause psychological distress, more easily resulting in social disability than functional impairment. Mixed TBF and mixed or profound hearing loss may also negatively influence quality of life.

Intraorbital Expansion of an Intradiploic Frontal Epidermoid Cyst: A Case Report and Short Review of the Literature.

BACKGROUND Calvarial epidermoid cysts (EC) are encased remnants of ectoderm at the third week of gestation. There are also reports which consider them sequelae of head trauma. They are benign lesions. As they develop, they exert a mass effect to adjacent anatomical structures. CASE REPORT We report the case of a 58-year-old male patient with left-sided exophthalmos. His radiologic examinations depicted an oval cystic lesion (7×5×5.5 cm) arising from the left frontal bone and abutting the ipsilateral orbital roof. Our patient underwent a total extirpation of the lesion through a frontal craniectomy. Cranioplasty was then performed with a Porex® graft. The pearl-hued lamellae of the lesion macroscopically resembled keratin tissue. Histopathological findings supported the diagnosis of an epidermoid cyst. Postoperatively, our patient had no neurologic deficits and a computed tomography scan showed no residual effects. CONCLUSIONS Large calvarial EC with intraorbital expansion in adults are rare clinical entities. Gross total resection with the infiltrated bone and cranioplasty is the treatment of choice, which also establishes the diagnosis.

Massive subgaleal hematoma in a 62-year-old man treated with apixaban as a consequence of mild head trauma.

Subgaleal hematoma, accumulation of blood in the loose areolar tissue of the subgaleal space of the skull, is considered the most catastrophic complication of instrumental delivery. It is a rare finding in older ages, usually associated with coagulation disorders, severe head trauma leading to skull base fractures and accidental or abusive hair pulling. Complications include periorbital necrotising fasciitis, permanent blindness, infections and, in extreme rare cases, airway obstruction. Most cases of subgaleal hematoma resolve spontaneously, without the need of aspiration or drainage.We present here the case of a 62-year-old male on anticoagulant therapy with apixaban for chronic atrial fibrillation, who came to the emergency department after a car accident suffering from mild head trauma. The patient was complaining of a diffuse headache and physical examination showed a large ecchymosis and edema on the frontal area of the head. His neurological examination was unremarkable. Full-body computed tomography (CT) revealed a fracture of the third right rib. Twelve hours after admission, due to an excessive decrease of hematocrit, a second CT was performed. Although the images didn't show intracranial hemorrhage or skull base fractures, a large and diffuse hematoma of the subaponeurotic space was observed and the diagnosis of subgaleal hematoma was confirmed.Massive subgaleal hematoma after mild head trauma is rather infrequent. Early diagnosis improves outcomes and can avert serious complications. Therapeutic strategy should be based on the severity of each case. In our case, conservative treatment appeared to be a valid alternative to surgery, as hematoma resolved spontaneously within 10 days. It is noteworthy that the use of anticoagulation is the only evident factor that could have been the precipitating factor for the development of the hematoma in our patient.

Gross total resection of intracisternal accessory nerve schwannomas using a familiar midline suboccipital subtonsilar approach.

BACKGROUND: Intracisternal Accessory Nerve Schwannomas (ANS) constitute a rare clinical entity with only a few cases reported so far. Their symptoms are usually due to brainstem compression and not manifested until they render of great size. Secondary neuropathy and muscle atrophy are other late signs. The hitherto reported literature advocates a suboccipital craniotomy as a suitable approach. In most of the cases the spinal root was the location tumor arose from. Gross total resection was possible to be carried out in most cases with only sporadic tolerable postoperative deficits reported. CASE DESCRIPTION: We hereby present three cases of patients with intracisternal ANS treated in the same fashion. Two patients were male and one female. Two patients complained of mainly chronic headaches and neck pain, whereas in the third patient the lesion was found incidentally. Only one patient suffered post-operatively cerebrospinal fluid leakage and wound healing complication, which was treated with revision surgery and administration of antibiotics. None of the patients had postoperative neurological deficits. Furthermore, we conducted a review of the relevant literature where we noted that there is no consensus yet with regards to the appropriate surgical approach. CONCLUSIONS: Based on relevant anatomical studies, we advocate that suboccipital subtonsilar approach provides a wide corridor to the area of lesion allowing complete and safe resection of intracisternal ANS. We thus support that, in most cases, the neurosurgeon should consider using this familiar approach for treating this rare lesion.

Does proximity to the subventricular zone and expression of CXCR-4 and nestin affect prognosis in high-grade glioma?

BACKGROUND: It is suspected that infiltration of stem cell areas with high-grade glioma (HGG) generates a population that compromises treatment results and survival. In this prospective study we set to assess the prognostic value of the proximity of the contrast-enhancing lesion (CEL) on MRI to the subventricular zone (SVZ) and the expression of CXCR4 and nestin as potential factors in the stem cell migration pathway. METHOD: All patients diagnosed with high-grade glioma over a three-year period from a single institution were enrolled in this prospective study. Based on MRI preoperative findings, the patients were classified into 4 Groups (I-IV) according to the proximity of the CEL on MRI to the SVZ. Histological samples were assessed with immunohistochemistry for nestin and CXCR4. Classification into groups and the presence of nestin and CXCR4 were evaluated as predictive factors for overall (OS) and progression free survival (PFS). RESULTS: Fourty patients were included in the study. In multivariate analysis, Groups II, III and IV predicted longer OS in comparison to group I (p = 0.01; p < 0.01; p < 0.01 respectively) and group III and IV predicted longer OS in comparison to group II (p < 0.01; p = 0.04 respectively). Group III predicted longer PFS than group I and II (p = 0.01; p < 0.01 respectively). The expression rates of CXCR-4 and nestin could not predict OS or PFS. CONCLUSIONS: In our study the classification according to the proximity of the contrast enhancing part of the lesion and the SVZ proved to be prognostically significant for both OS and PFS. Presence of CXCR4 or nestin was not predictive for OS or PFS.

Pilomyxoid astrocytoma of the cervical spinal cord in a child with rapid progression into glioblastoma: case report and literature review.

INTRODUCTION: Pilomyxoid astrocytoma (PMA) is a recently described glial tumor with similarities to pilocytic astrocytomas, yet with distinct histopathological characteristics and a more aggressive behavior. It occurs predominantly in the hypothalamic/chiasmatic region. Only four patients with spinal cord PMA have been reported in the pediatric population. The 2007 WHO Working Group recognized PMA as a new variant and recommended an assignment to WHO grade II. OBJECTIVE: The purpose of this paper was to report a rare location, address the aggressive behavior and rapid progression, and based on the specific patient, to review the literature and discuss current treatment strategies. CASE PRESENTATION: A 12-year-old girl presented with motor and sensory deficits of the left side as well as gait disturbance. Imaging revealed an intramedullary tumor extending from C2 to C7. The patient improved impressively after surgical resection. Histopathological findings were consistent with PMA. Three months later, the patient presented with rapid neurological deterioration. Histopathology after the second operation was consistent with glioblastoma. The outcome was fatal 12 months after initial diagnosis, despite adjuvant therapy. CONCLUSIONS: This is the fifth pediatric spinal cord PMA in literature. Furthermore, it is the only documented patient with rapid recurrence and progression within 3 months into a glioblastoma. The question of a sampling error affecting initial pathology is raised. Based on contemporary literature data, we discuss the further treatment options, as there are no guidelines yet. Efforts towards registries should be encouraged, as the documentation of PMA might lead to more evidence based treatment strategies.

Biopsy versus resection in the management of malignant gliomas: a systematic review and meta-analysis.

OBJECT: The aim of this study was to answer the question whether quality of life and progression-free and overall survival are increased in adults with supratentorial malignant glioma who are treated with cytoreductive resection as compared with those who only undergo biopsy. METHODS: A literature search of the electronic databases MEDLINE, EMBASE, and CENTRAL was performed to identify relevant studies published before May 2008. Hand-searching of reference lists of the identified studies and relevant review articles was also performed. A study was considered eligible, regardless of study design (prospective or retrospective), if: 1) quality of life and/or progression-free and/or overall survival was compared among adult patients undergoing biopsy or resection, and 2) patient age and Karnofsky Performance Scale scores were not significantly different among the 2 groups compared. RESULTS: One randomized controlled trial and 4 retrospective studies (involving a total of 1111 patients) were found eligible for this systematic review. A meta-analysis of the eligible studies demonstrated a significant increase in overall survival in the patients treated with resection instead of biopsy (hazard ratio 0.61, 95% CI 0.52-0.71, p < 0.0001, fixed-effect model). Although statistical pooling was not feasible, the available data suggest that quality of life was increased in patients treated with resection rather than biopsy, while there did not seem to be any significant difference in progression-free survival between the 2 groups. CONCLUSIONS: Based on the best available evidence, it appears that cytoreductive resection in adults with supratentorial malignant glioma is associated with improved overall survival as compared with biopsy. However, well-designed prospective studies are needed for more solid conclusions to be drawn.

Spontaneous spinal cord herniation: recurrence after 10 years.

BACKGROUND CONTEXT: Transdural spontaneous spinal cord herniation is a very rare nosological entity, which despite recent reports in the medical literature remains often misdiagnosed preoperatively. Usually it affects the thoracic segment, protrudes ventrally, presents clinically as a progressive Brown-Séquard syndrome and carries a favorable surgical outcome. PURPOSE: To describe a rare case of delayed recurrence of spontaneous spinal cord herniation despite excellent outcome for 10 years, and discussion of management and observation issues. STUDY DESIGN/SETTING: Case report/University Hospital. METHODS: We describe the management of a patient with spontaneous spinal cord herniation that presented initially with a Brown-Séquard syndrome and has been treated successfully with surgery. After 10 years free of symptoms, the patient developed progressive paraparesis and urinary incontinence because of recurrence of the herniation at the same level. RESULTS: Despite technical challenges, during the second operation the enlarged ventral dural defect was meticulously closed and the patient's neurological condition presents 9-month postsurgery considerable improvement. To our best knowledge, recurrences of spontaneous spinal cord herniation are extremely rare. CONCLUSIONS: The reported case underlines the necessity to be aware of this rare but treatable spinal disease, notify the possibility of late recurrences and the need of long-term follow-up even if initial outcome is favorable.

Delayed persistent hyperthermia after resection of a craniopharyngioma.

OBJECTIVE AND IMPORTANCE: Disorders of thermoregulation are occasionally noticed after operations in the region of the third ventricle. Various factors are usually implicated, but the actual contribution of each of them is rather vague. Apart from the presumed derangement in the functional connections of the hypothalamic region, mechanical reasons of compression should be thoroughly considered. CLINICAL PRESENTATION: An 8.5-year-old patient was subjected to a radical excision of a craniopharyngioma compressing the third ventricle. Three months after the operation, he presented with a febrile syndrome of unknown origin. All usual investigations proved negative. INTERVENTION: A chronic subdural hygroma was evacuated, an encapsulated CSF cyst of the suprachiasmatic cistern was drained and the lamina terminalis incised resulting in a moderate control of pyrexia. The administration of chlorpromazine contributed to the final resolution of hyperthermia. CONCLUSION: Postoperative hyperthermia may result following resection of tumors of the hypothalamic floor. It should not be blindly attributed to hypothalamic dysfunction as surgical causes could be implicated as well. Chlorpromazine could be a useful adjunct to the correction of the disorder.