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Important prognostic factors in patients with skull base erosion from nasopharyngeal carcinoma after radiotherapy.

PURPOSE: To evaluate the long-term outcome and prognostic factors in patients with skull base erosion from nasopharyngeal carcinoma after initial radiotherapy (RT). METHODS AND MATERIALS: From January 1985 to December 1986, 100 patients (71 males, 29 females) with a diagnosis of nasopharyngeal carcinoma were found on computed tomography (CT) to have skull base erosion. The mean age was 41 years (range 16-66). Ninety-six patients had World Health Organization type III undifferentiated carcinoma, and 4 had type I. The metastatic workup, including chest radiography, liver ultrasound scanning, and liver function test was negative. All patients underwent external beam RT (EBRT) alone to 66-80 Gy during 6-8 weeks. A daily fraction size of 2 Gy was delivered using 60Co or a linear accelerator. No patient received chemotherapy. All patients were followed at regular intervals after irradiation. The median follow-up was 22.3 months (range 2-174). Survival of the cohort was computed by the Kaplan-Meier method. The potential prognostic factors of survival were examined. Multivariate analyses were performed using the Cox regression model. RESULTS: The 1, 2, 5, and 10-year overall survival rate for the cohort was 79%, 41%, 27%, and 13%, respectively. However, the subgroup of patients with both anterior cranial nerve (I-VIII) and posterior cranial nerve (IX-XII) involvement had a 5-year survival of only 7.7%. A difference in the time course of local recurrence and distant metastasis was observed. Both local recurrence and distant metastasis often occurred within the first 2 years after RT. However, local relapse continued to occur after 5 years. In contrast, no additional distant metastases were found after 5 years. The causes of death included local recurrence (n = 59), distant metastasis (n = 21), both local recurrence and distant metastasis (n = 1), and unrelated causes (n = 5). After multivariate analysis, complete recovery of cranial nerve involvement, cranial nerve palsy, and headache after irradiation were found to be independent prognostic factors in this cohort. CONCLUSIONS: We present one of the longest follow-ups of patients with nasopharyngeal carcinoma invading the skull base. Our results demonstrate the importance of cranial nerve involvement, recovery of headache, and cranial nerve palsy. These factors should be carefully evaluated from the history, physical examination, and imaging studies. A subgroup of patients with skull base involvement had long-term survival after RT alone. The findings of this study are important as a yardstick against which more aggressive strategies, such as combined radiochemotherapy and altered fractionation RT can be compared.

Adolescent↗

Clinical results of proton beam therapy for skull base chordoma.

PURPOSE: To evaluate clinical results of proton beam therapy for patients with skull base chordoma. METHODS AND MATERIALS: Thirteen patients with skull base chordoma who were treated with proton beams with or without X-rays at the University of Tsukuba between 1989 and 2000 were retrospectively reviewed. A median total tumor dose of 72.0 Gy (range, 63.0-95.0 Gy) was delivered. The patients were followed for a median period of 69.3 months (range, 14.6-123.4 months). RESULTS: The 5-year local control rate was 46.0%. Cause-specific, overall, and disease-free survival rates at 5 years were 72.2%, 66.7%, and 42.2%, respectively. The local control rate was higher, without statistical significance, for those with preoperative tumors <30 mL. Partial or subtotal tumor removal did not yield better local control rates than for patients who underwent biopsy only as the latest surgery. CONCLUSION: Proton beam therapy is effective for patients with skull base chordoma, especially for those with small tumors. For a patient with a tumor of <30 mL with no prior treatment, biopsy without tumor removal seems to be appropriate before proton beam therapy.

Adolescent↗

Treatment of skull base tumors.

The rich multidisciplinary environment in which skull base surgery has developed has allowed for significant progress in the field and has improved patient outcomes. More precise and detailed anatomic dissection in the laboratories has provided surgeons with the benefit of undertaking the serious technical challenges encountered in the skull base. Significant work has been done and continues to be developed in the field of adjuvant treatments including radiotherapy, chemotherapy, and hormonal therapy. Using biologic end points and specific genetic targets in the tumors may further expand our management options in the future.

Antineoplastic Combined Chemotherapy Protocols↗

Intraoperative radiotherapy of skull base cancer.

As the head and neck surgeon expands the boundaries of resectability into the skull base, the margin of tumor clearance diminishes. Intraoperative radiotherapy (IORT) can be used as an adjunct to skull base surgery and external beam radiation to enhance local control in areas with close margins or remaining microscopic disease. During the period from May 1982 to May 1988, 25 patients underwent IORT of the skull base following resection and prior to closure to treat microscopic disease in 9 patients, sterilize close margins in 14 patients, and attempt to eliminate remaining gross disease in 2 patients. The types of tumors treated were 13 squamous cell carcinomas, 4 adenoid cystic carcinomas, 3 high-grade mucoepidermoid carcinomas, 3 sarcomas, 1 malignant mixed tumor, and an oncocytic adenocarcinoma. The majority of the tumors either originated in the sinuses or were skull base extensions of oral cavity or oropharyngeal cancers. In 22 patients with 1-year follow-up, IORT prevented local recurrence in 14 (64%) patients. This may represent improved control of local disease in patients who historically have a very dismal prognosis. The indications, techniques, and complications of this exciting therapeutic modality are also reviewed.

Adolescent↗

Skull base osteomyelitis interpreted as malignancy.

Lesions in the skull base may present difficulties in diagnosis primarily because the access needs to be invasive and one has to rely on imaging that may still be misleading. The case presented here illustrates this example in that the patient had abnormalities on computed tomography (CT) and magnetic resonance image (MRI) scans quite convincing of malignancy but which with time proved, essentially through clinical judgment, to be skull base osteomyelitis secondary to malignant external otitis (OME).

Aged↗

Review of skull base chordomas: prognostic factors and long-term results of proton-beam radiotherapy.

Fractionated proton beam radiotherapy has been used for skull base tumors in the United States since the mid-70s, and more than 300 patients in whom diagnosis of chordoma of the skull base has been made have been treated. The ability to achieve high degrees of radiation dose conformity by using protons has resulted in higher radiation doses than can be delivered with conventional radiotherapy in the base of skull. High target volume doses have led to improved tumor control and patient survival. Side effects such as severe toxicity are acceptable considering the alternatives of uncontrolled tumor growth. The authors of various analyses have identified prognostic factors that can be used to predict a patient's chance of treatment success. On the horizon are important technical developments that will further increase dose conformity and increase target doses. In this paper the author reviews long-term outcome data and prognostic predictors for survival of patients with skull base chordomas based on the largest worldwide patient series.

Age Factors↗

Reconstruction of lateral skull base oncological defects: the role of free tissue transfer.

Surgical ablation for oncological disease of the lateral skull base can result in extensive defects, with exposed bone and dura. Inadequate coverage can result in meningitis, osteomyelitis, or delay in adjuvant therapy. Successful reconstruction requires well-vascularized soft tissue and often a large cutaneous component. This study evaluates the role of free tissue transfer in reconstruction of lateral skull base defects. This study is a retrospective review of all patients undergoing lateral skull base resection for oncological disease and immediate reconstruction from 1993 through 1997. There were 18 patients with a mean age of 57 years. The temporal bone was resected in 50% of patients. All defects were reconstructed with free tissue transfers from the following donor sites: rectus abdominis (N = 14), latissimus dorsi (N = 2), anterolateral thigh (N = 1), and lateral arm (N = 1). A cutaneous skin island was employed in all patients. Free flap survival was 100%. Flap-related complications occurred in 33% of patients but did not delay the onset of adjuvant therapy. Vein grafts were not required to lengthen the vascular pedicle. Two patients required split-thickness skin grafts because of inadequate size of the skin island. Four patients underwent flap revision for contour deformity a mean of 4 months postoperatively. Free tissue transfer is a highly reliable method of reconstructing lateral skull base defects in a single stage. Careful flap selection and design can minimize the need for skin and vein grafts. The rectus abdominis donor site is preferred because of its location, large skin island, and excellent vascular pedicle.

Adolescent↗

Gamma knife radiosurgery of skull base meningiomas.

Meningiomas are the most frequent benign tumors treated by gamma knife radiosurgery and the majority of them are located on the skull base. Between 1992 and 1999, 197 skull base-located meningiomas in 192 patients were treated by gamma knife in Prague. Contact with the chiasma or optic tract was not regarded as a contraindication for gamma knife radiosurgery and such contact was observed in 32% of the skull base meningiomas treated. 176 patients were monitored during a median of 36 months, of whom 73% showed a decrease in tumor volume; no change was observed in 25% and continued growth was observed in 2%. Neurodeficit improved in 63% of patients, temporary morbidity occurred in 11% and persistent morbidity remained in 4.5%. Radiosurgery induced edema in 11%. Significantly lower edema occurrence was observed after radiosurgery in patients with no history of edema prior to radiosurgery, where the tumor was located in the posterior skull base and where the dosage to the tumor margin was lower than or equal to 14 Gy. Radiosurgery of skull base meningiomas has been proven to be safe and efficient. We consider gamma knife treatment for skull base meningiomas to be the method of choice whenever tumors are within the volume limits and there is no need for an urgent decompressive effect from the open operation.

Adult↗

[Selection of operative approaches for rhinosinusal malignant tumor involving in the anterior skull base and exploration for reconstruction].

OBJECTIVE: To investigate operative approaches and techniques of reconstruction for rhinosinusal malignant tumors involving in the anterior skull base. METHOD: In a retrospective study, clinical data of 22 patients treated surgically in our department from 1988 to 2002 were analysed. Lateral rhinotomy was carried out in 8 patients and ampliative rhinotomy in 4 patients; in 10 patients craniofacial operation was performed. Multiple layer fascia flaps were used to repair lager defect in the anterior skull base. RESULT: All tumors were totally resected. In lateral rhinotomy, local locoeled meningitis happened in 1 case, and 4 patients in craniofacial operation. None complication with cerebrospinal fluid leaks, encephalomeningocele and intracranial air. All patients were followed up from 6 months to 11 years after surgery. 7 patients died from intracranial infection or metastasis of tumor. 3-year survival rates was 15/19(78.9%); 5-year survival rates was 13/19(68.4%). The tumor invaded the orbital fascia in 9 patients. Vs 4/9 (44.4%) and 2/9(22.2%) for these 3 and 5-year survival rates. CONCLUSION: Selection of operative approaches depends on the size of defects in the anterior skull base, extent of intracranial invasion and location of rhinosinusal malignant tumors; multiple layer fascia flaps can be used to reconstruct bigger defects of the skull base. It is safe, technically easy, effective procedure and worthy to be recommended.

Adolescent↗

Successful chemoradiation therapy for high-grade skull base chondrosarcoma in a child.

High-grade chondrosarcoma in the skull base has been known to be extremely refractory to adjuvant therapy. We report successful chemoradiation therapy for skull base chondrosarcoma in a child. The patient was a 6-year-old boy with an invasive skull base tumor. In spite of gross total removal of the tumor, it recurred 1 month after surgery. Following an intra-arterial injection of adriamycin (10 mg), the second gross total removal was carried out. At the end of the operation, cisplatin (20 mg) was locally injected into the surgical cavity. The patient was further given a total of six courses of systemic chemotherapy in combination with adriamycin (30 mg/m2) and cisplatin (100 mg/m2) and 55 Gy focal irradiation. One year after the most recent surgery, the patient is in complete remission. The efficacy of adjuvant therapy of this rare tumor is discussed.

Antineoplastic Combined Chemotherapy Protocols↗

Fibrous dysplasia involving the skull base and temporal bone.

OBJECTIVE: To gain a broader appreciation of the clinical presentation, operative treatment, and outcome of patients with fibrous dysplasia involving the skull base. DESIGN: Retrospective review of a clinical case series. SETTING: A single tertiary academic medical center. PATIENTS: Twenty-one patients with histopathologically confirmed fibrous dysplasia involving the skull base cared for over a 15-year-period (1983-1998). MAIN OUTCOME MEASURES: Clinical and radiographic location of the fibrous dysplasia lesions within the skull base, clinical presentation, surgical intervention, and clinical outcome were tabulated for each patient. RESULTS: The ethmoids were most commonly involved (71%), followed by the sphenoid (43%), frontal (33%), maxilla (29%), temporal (24%), parietal (14%), and occipital (5%) bones. The most common presenting features included atypical facial pain and headache, complaints referable to the sinuses, proptosis and diplopia, hearing loss, and facial numbness. Surgical treatment, guided by clinical presentation, ranged from simple biopsy with conservative follow-up to craniofacial resection. CONCLUSIONS: Fibrous dysplasia can present in myriad ways within the skull base. Modern imaging modalities and histopathologic analysis have made diagnosis relatively straightforward. Surgery, particularly in such a challenging region as the skull base, should be reserved for patients with functional impairment or a cosmetic deformity. Because of the benign nature of the condition, the surgery itself should be relatively conservative, with the primary goal being preservation of existing function.

Adolescent↗

Three-layer reconstruction with fascia lata and vascularized pericranium for anterior skull base defects.

BACKGROUND: We report an assessment of the efficiacy of a triple layer graft composed of fascia lata and vascularized pericranium for anterior skull base reconstruction. This technique is based on the concept that vascularized tissue over a free flap may promote vascularization and rapid wound healing. METHOD: A large fascial graft is prepared from the fascia lata and divided in two pieces and trimmed to a size larger than the bone and dural defect. Vascularized pericranium is harvested after bicoronal incision and elevating the bifrontal scalp flap down to the supraorbital rims. First is dural repair, which is performed with fascia lata placed between the brain and remaining dura. Second, fascia lata is placed over the skull base defect and secured with mini titanium screws over the cranial surface of the orbital ridges. Third, vascularized pericranium is laid between the two layers of fascia lata. FINDINGS: We studied 17 patients of whom 2 had malignancy, 6 had olfactory groove meningioma, 6 had skull base fracture and rhinorrhea, 1 case had orbital meningioma, 1 had invasive pituitary adenoma and 1 had basal encephalocele. The transbasal approach was used as a single procedure in 13 cases. The extended transbasal approach combined with a transfacial approach was used in 3 cases and with a pterional approach in 1 case. In each patient, reconstruction of the cranial base was performed with triple layer graft of fascia lata and vascularized pericranium. The patients were followed-up 2 months to 5 years. None of the patients experienced postoperative cerebrospinal fluid leakage, meningitis, abscess, brain herniation and tension pneumocephalus. INTERPRETATION: Fascia lata with vascularized pericranium is highly reliable, tensile and well suited for reconstruction of the anterior skull base.

Adolescent↗

Skull base reconstruction after anterior subcranial tumor resection.

OBJECT: The goal of this study was to demonstrate the efficacy of a simple and reliable technique for anterior skull base and craniofacial reconstruction in patients who have undergone excision of tumors via the subcranial approach. METHODS: There were 63 patients who had undergone 71 anterior skull base resections of tumors via the aforementioned approach. Twenty-nine cases (41%) involved malignant tumors and 42 (59%) involved benign tumors. Reconstruction of the anterior skull base was performed by a single team who used double-layer fascial graft. Limited dural defects were reconstructed using the temporalis fascia, whereas large anterior skull base defects were reconstructed using a fascia lata sheath. Reconstruction was achieved without the support of bone graft or titanium mesh and without pericranial, galeal, or free flaps. Pericranial flap wrapping of the frontonasoorbital segment was performed to prevent osteoradionecrosis if postoperative radiotherapy was planned. The incidence of cerebrospinal fluid (CSF) leakage, intracranial infection, and tension pneumocephalus was 5.6%. Histopathological examination of fascia lata grafts obtained in patients who had undergone a second procedure demonstrated integration of vascularized fibrous tissue to the graft, as well as local proliferation of a newly formed vascular layer embedding the fascial sheath. CONCLUSIONS: The use of a double-layer fascial graft alone was adequate for prevention of CSF leakage, meningitis, tension pneumocephalus, and brain herniation. The double-layer fascial flap provided a simple and reliable means for anterior skull base reconstruction after en bloc resection of both malignant and benign tumors.

Adolescent↗

Computed tomography findings of bony regeneration after radiotherapy for nasopharyngeal carcinoma with skull base destruction: implications for local control.

PURPOSE: To evaluate the response of bony destruction (BD) of the skull base following radiotherapy in nasopharyngeal carcinoma (NPC) and investigate the implications of bony regeneration (BR) on local control and its related factors. METHODS AND MATERIALS: Ninety patients with NPC with skull base destruction clearly demonstrated on computed tomography (CT) were reviewed. These patients have completed the prescribed treatment and received regular CT follow-up. A total of 338 sets of CT images of the head and neck were reviewed. The tumor response and the appearance of BR in the previous destructive part of the skull base were recorded and analyzed. The tumor response was divided into complete, partial, or no response. BR was defined as recalcification or sclerotic change with partial or complete healing in the previous osteolytic bony defect. Local failure was confirmed either by pathological or merely by imaging studies showing progression of tumor in consecutive radiological pictures. RESULTS: The distribution of specific sites of bony destruction (BD) in these patients included the sphenoid bone (68%), paracavernous sinus area (48%), petrous apex (47%), clivus (44%), pterygoid plates (20%), and others (7%). The CT showed 57 patients (63%) had BR. All were observed within 1 year after treatment. Sixty-two patients (69%) had complete tumor response after treatment. Analyzed by logistic regression method, tumor response after treatment was found to have a statistically significant correlation with BR (p = 0.0004). Most BR (55/57) was demonstrated in patients with complete tumor response. The 3-year actuarial local control rate was 54 % in these patients. The local control was quite different in the comparison of patients with BR versus those with persistent BD (77% vs. 21%, p < 0.0001). Multivariate analysis showed that patients with complete tumor response or with BR on imaging had statistically better local control than those without either of the two findings (p < 0.05). CONCLUSION: Appearance of BR at previous destructive skull base following radiotherapy for NPC patients could be clearly demonstrated on CT. Bony regeneration significantly correlated with treatment response and local control. Although the underlying significance of BR was unknown, to predict the outcome after treatment, the appearance of BR shown on CT may imply the complete eradication of tumor in this area.

Adolescent↗

Combined approach for lesions involving the cerebellopontine angle and skull base: experience with 20 cases--preliminary report.

Skull base tumors involving the clivus, petrous bone, and adjacent areas can be formidable lesions to successfully remove without causing significant neurologic deficits. At our institution in the last 5 years, twenty patients out of 103 patients with skull base tumors have undergone the supratentorial-infratentorial combined approach for removal of a neoplasm (nine schwannomas, six meningiomas, two epidermoids, one extensive basal cell carcinoma, one pontine cavernous malformation, and one basilar artery aneurysm). The average patient age was 43 years. The combined approaches in conjunction with the subtemporal exposure were retrosigmoid-retrolabyrinthine, retrosigmoid-translabyrinthine, or retrosigmoid-transcochlear. The choice depended upon the type and location of the lesion and the deficits noted preoperatively. Basically, the approach allowed communication of the middle fossa and posterior fossa by totally dividing the tentorium as much as necessary for effective surgical manipulations. Both sigmoid and superior petrosal sinuses are divided. There were no deaths. Postoperative neurologic deficits included temporary seventh nerve paralysis, sixth nerve weakness, fifth nerve sensory deficits, cerebrospinal fluid leaks, and hydrocephalus requiring a shunt. Overall, the results were very gratifying, considering the difficulties encountered in the surgical removal of these lesions.

Adult↗

Two-stage skull base surgery for tumours extending to the sub- and epidural spaces.

The surgical management of extensive skull base neoplasms, which often extend to both the sub- and epidural spaces, is still a great challenge with considerable risk. The authors report 12 cases in which a two-stage operation was performed for such nonmalignant tumours. The series consisted of four cavernous sinus meningiomas, one sphenoid-ridge meningioma, one cerebello-pontine angle meningioma, three pituitary adenomas, two chordomas, and one fibroma. Our operative strategy involved removal of the epidural part of the tumour and extensive skull base reforming during the first stage. After approximately one month, the second stage operation was performed by removing the residual subdural parts and the affected dura, which were less vascular, with dural plasty and subsequent spinal drainage. No complications such as cerebrospinal fluid (CSF) leakage or infection were observed. During the long-term follow-up (1.4 to 4.6 years, with a mean of 2.7 years), tumour recurrence was observed in a single case. In conclusion, the major advantages of this procedure were as follows: [1] improvement of the total removal rate, [2] prevention of postoperative CSF leak and infection, [3] residual tumours were avascular, necrotic, and dwindling, and also shifted outwards resulting in less adhesions to the brain. Although it may counter the trend toward less invasive procedures, the two-staged skull base surgery warrants serious consideration as an option for the management of patients with such extensive cranial base tumours.

Adenoma↗

[Substantiation of application of the anterior trans-pyramidal approach in skull base tumors].

The microtopographic-anatomic substantiation of anterior transpyramid approaches to the skull base tumors was conducted. The transpyramid approaches introduction in clinical practice had permitted to improve surgical treatment results in patients with the difficult of access skull base tumors. Indications and choice of approach depending on the kind, localization and tumor spread in the skull base in the temporal bone pyramid part were substantiated.

Adult↗

[Adenoid cystic carcinomas invading the skull base. Apropos of 4 cases and review of the literature].

Head and neck adenoid cystic carcinomas may invade the adjacent skull base by bone lysis and/or by perinervous and perivascular spread within the skull base foramina. Neurosurgical decision making is not well defined regarding the extent of intracranial tumor component removal, as neurosurgical expertise is limited for this peculiar type of tumors. The issue is to decide whether a radical supposedly locally curative surgery should be attempted, or if a large non disfigurating surgery is mandatory, keeping in mind the frequency of local recurrences and of distant metastases. Over a 13-year period, four adenoid cystic carcinomas invading the skull base were operated on at our institution: two tumors originated in the parotid gland, one in the sphenoid sinus, and one in the ethmoid sinus. Surgical removal was total in one case, subtotal in three cases. Post-operative irradiation was delivered in the four patients (two neutron irradiation, two conventional). One patient with advanced metastatic disease was submitted to chemotherapy. Three patients died from local tumor progression and distant metastases within three years after the intracranial tumor extension has been diagnosed. The patient with an ethmoid tumor is still alive seven years after surgery without any evidence of local tumor progression nor distant metastases. Surgery remains the gold standard treatment for adenoid cystic carcinomas invading the skull base. However, in our opinion a large tumor removal, without or with bone osteotomies, but without sacrifice of cranial nerves, cavernous sinus, internal carotid artery, and of the orbit allows patient survival with an acceptable comfort and absence of psychological distress due to disfigurating surgery nor surgically induced neurological functional deficit. Post-operative irradiation may sometimes stabilize locally the lesions. The place of chemotherapy has, yet, to be determined.

Adult↗