Extraskeletal Ewing's sarcoma（EES） in the head and neck is extremely rare, with non-specific clinical manifestations, high malignancy, easy recurrence and metastasis, and poor prognosis. This paper reports a case of EES of the parapharyngeal skull base presenting with secretory otitis media as the initial symptom.The treatment consisted of surgery, chemotherapy and radiotherapy. No further metastasis or recurrence was observed during the two years and six months follow-up. Now we reviewed the relevant literatures and summarized the experience of diagnosis and treatment in EES.
Humans ; Otitis Media with Effusion/etiology* ; Sarcoma, Ewing/therapy* ; Skull Base ; Skull Base Neoplasms/therapy*

BACKGROUND: Craniofacial resection (CFR) has been regarded as a standard treatment for various tumors involving the anterior skull base. The purpose of this study was to evaluate the results of CFR for the patients with anterior skull base malignancies in our hospital. METHODS: We retrospectively analyzed 17 patients with anterior skull base malignancies treated with CFR between 2001 and 2012. Mean follow-up duration was 41 months (range, 2-103 months). RESULTS: Intracranial involvement was found in 11 patients (65%) and orbital extension in 6 patients (35%). Classical bifrontal craniotomy was combined with endoscopic endonasal approach in 14 patients and external approach in 3 patients. Vascularized flap was used for reconstruction of the anterior fossa floor in 16 patients (94%). The most common pathological type was squamous cell carcinoma (6 patients). Gross total resection was achieved in all cases. Postoperative complications developed in 4 patients (24%) and included local wound problem and brain abscess. One patient with liver cirrhosis died from unexpected varix bleeding after the operation. Although postoperative treatment, such as radiotherapy or chemotherapy, was performed in 14 patients, local recurrence was seen in 6 patients. The mean overall survival time after the operation was 69.0 months (95% confidence interval: 47.5-90.5 months) with a 1-, 2-, and 5-year survival rate of 82.3%, 76.5%, and 64.7%, respectively. Postoperative radiotherapy was found to be the powerful prognostic factor for favorable survival. CONCLUSION: Considering the higher local control rate and acceptable complication or mortality rate, CFR with adjuvant radiotherapy is a gold standard treatment option for malignant tumors involving anterior skull base, especially with extensive intracranial involvement.
Brain Abscess ; Carcinoma, Squamous Cell ; Cranial Fossa, Anterior ; Craniotomy ; Drug Therapy ; Follow-Up Studies ; Hemorrhage ; Humans ; Intraoperative Complications ; Liver Cirrhosis ; Mortality ; Orbit ; Paranasal Sinus Neoplasms ; Postoperative Complications ; Radiotherapy ; Radiotherapy, Adjuvant ; Recurrence ; Retrospective Studies ; Skull Base* ; Skull* ; Survival Rate ; Treatment Outcome ; Varicose Veins ; Wounds and Injuries

OBJECTIVETo analyze the clinical features and treatment of skull base osteosarcoma.

METHODSThe clinical data of 18 patients with skull base osteosarcoma, who were admitted to the CAMS Cancer Hospital from January 2005 to November 2013, were retrospectively analyzed. The patients were followed up by telephone, outpatient review and other means. Fifteen patients were followed up, 4 cases received surgery only, and 11 cases received surgery with adjuvant chemotherapy and/or radiotherapy. Kaplan-Meier survival curve analysis was used to analyze the clinical data and Log rank method was used for verification.

RESULTSNine patients died among the 15 patients who were followed up for 3-103 months (mean 25.0 months): seven patients died of local recurrence, and two patients died of distant metastasis, and six patients were still alive. Four patients received surgery only, with a median survival time of 25.0 months, and 11 patients received comprehensive treatment, with a median survival time of 47.0 months (P = 0.02). Five patients received sub-total resection, with a mean survival time of 47.0 months, and 10 patients received total resection, with a mean survival time of 45.0 months (P = 0.37). The 1- and 2-year recurrence rates were 46.6% and 68.9%, respectively. The overall 1-, 2-, 3- and 5-year survival rates were 82.4%, 61.8%, 36.0% and 36.0%, respectively, with a median survival time of 30.0 months.

CONCLUSIONSTo compare the long bone and head and neck osteosarcoma with skull base osteosarcoma, the skull base osteosarcoma has a lower total resection rate, a higher recurrence rate, and a poorer prognosis. Radical surgery and comprehensive treatment are appropriate for skull base osteosarcoma.

Bone Neoplasms ; diagnosis ; pathology ; therapy ; Chemotherapy, Adjuvant ; Combined Modality Therapy ; Humans ; Kaplan-Meier Estimate ; Neoplasm Recurrence, Local ; Osteosarcoma ; diagnosis ; pathology ; therapy ; Retrospective Studies ; Skull Base ; pathology ; Survival Rate

OBJECTIVETo analyze the factors affecting prognosis of the carcinoma arising from nasal and sinonasal inverted papilloma.

METHODSThe clinicopathological data of sixty-two patients treated in our hospital from January 1974 to February 2012 were retrospectively analyzed. Of the 62 cases, 10 were at stage I or II, 24 at stage III, and 28 at stage IV. Twenty-six patients were treated with surgery alone, and 36 with surgery combined with radiation therapy. Kaplan-Meier method and log-rank test were used for the survival analysis.

RESULTSThe overall 5-years survival rate was 39.6%. The 5-years survival rate was 67.5% in the stage I or II patients,51.3% in the stage III patients, and 18.3% in the stage IV patients (P<0.05). The 5-years survival rate was 65.7% in patients who had no surgical history, and 29.9% in patients who had surgery (P<0.05). The 5-year survival rate was 17.6% in the group who relapsed after the treatment, and 49.6% in the non-relapsed patients (P<0.05). The 5-year survival rate was 23.4% in the patients who had involvement of cranial base and (or) orbit, and 47.6% in the patients whose cranial base and (or) orbit were clean (P<0.05). Of the patients at the same stage (III-IV), the 5-year survival rate of patients treated with surgery alone was 32.4%, and those treated with combination therapy was 36.2%(P=0.89). The univariate analysis showed that clinical stage, surgical history before malignization, involvement of the cranial base and (or) orbit organs, and post-operative relapse are significantly correlated to prognosis of the patients (P<0.05 for all). Multivariate analysis showed that age, clinical stage, and previous history of surgery were independent factors affecting the prognosis of the patients. Distant metastasis was the major cause of death, mostly lung metastases.

CONCLUSIONSAge, clinical stage and surgical history are the main factors affecting the prognosis of the patients. The history of recurrence and involvement of cranial base or orbit also play an important role for the prognosis. Distant metastasis is the main cause of death in the patients with carcinoma arising from nasal and sinonasal inverted papilloma.

Carcinoma ; diagnosis ; therapy ; Combined Modality Therapy ; Head and Neck Neoplasms ; Humans ; Lung Neoplasms ; Multivariate Analysis ; Neoplasm Recurrence, Local ; Papilloma, Inverted ; diagnosis ; therapy ; Papillomavirus Infections ; Prognosis ; Retrospective Studies ; Skull Base ; Survival Rate

Nasopharyngeal cancer is a low differentiated squamous cell carcinoma, and the radiation therapy is the primary choice. It's 5 year survival rate may reach 64.4%, while 10.0% cases may suffer from the local recurrence. The salvage radiation or surgery is still the main choice for recurrent cases now. However the recurrent tumor become radiation insensitive and meanwhile, morbidity and mortality become higher. The experience of open salvage surgery on the recurrent radiation insensitive tumor had been proved safely, and the related morbidity and mortality are acceptable. Recently, the endoscopic salvage surgery has been developed, some preliminary experience has been obtained and the result looks promising. In this article, the pathobiological characteristics of the postradiation local recurrent nasopharyngeal cancer, the anatomy of nasopharyngus and related skull base area, especially the petroclival region and current situation of endonasal endoscopic salvage surgery were reviewed here. Basic principle of oncological surgery that endoscopic surgery should followed and possible endoscopic surgical approaches were proposed.
Carcinoma ; Carcinoma, Squamous Cell ; radiotherapy ; surgery ; Endoscopy ; Humans ; Nasopharyngeal Carcinoma ; Nasopharyngeal Neoplasms ; radiotherapy ; surgery ; Neoplasm Recurrence, Local ; radiotherapy ; surgery ; Radiation Tolerance ; Salvage Therapy ; methods ; Skull Base ; Survival Rate

OBJECTIVE: To explore medical and nursing management of postoperative complications of basicranial tumor resection through nasal endoscopy. METHOD: Retrospective analysis was carried out in 178 patients with skull base tumors who underwent nasal endoscopic resection of basicranial tumors under general anesthesia, and controlled hypotension was conducted during the surgery. Different surgical approaches were applied in terms of anatomical regions which the tumor involved. RESULT: Postoperative complications occurred in 87 of 178 patients, including cerebrospinal fluid rhinorrhea, intracranial infections, diabetes insipidus, etc. Eighty-five cases with complications recovered through proper medical and nursing care, except for 2 cases of death. CONCLUSION Because there is a higher risk for nasal endoscopic basicranial procedure, doctors and nurses are required to master multidisciplinary skills and experiences. Careful and rigorous nursing care of postoperative complications is quite important for successful management of basicranial operative patients.
Adolescent ; Adult ; Aged ; Child ; Endoscopy ; adverse effects ; Female ; Humans ; Male ; Middle Aged ; Postoperative Complications ; therapy ; Retrospective Studies ; Skull Base Neoplasms ; surgery

Adolescent ; Adult ; Aged ; Child ; Endoscopes ; Female ; Humans ; Laser Therapy ; methods ; Male ; Microsurgery ; methods ; Middle Aged ; Prognosis ; Skull Base Neoplasms ; mortality ; pathology ; surgery

OBJECTIVETo investigate the diagnostic value of F-18-fluoro-deoxyglucose positron emission tomography (FDG-PET) for the recurrent or residual nasopharyngeal carcinomas in the skull base area.

METHODSNine post-irradiation nasopharyngeal carcinoma patients did FDG-PET scanning, CT/MRI imaging and underwent nasopharynx and skull base-biopsy under endoscopy. The results of FDG-PET were evaluated and compared with CT/MRI studies and biopsies.

RESULTSIn 9 cases of post-irradiation nasopharyngeal carcinoma, CT/MRI detected 7 recurrent cases and 2 suspected recurrent cases in occipital bone and clivus. All 9 cases had accumulated FDG in nasopharynx and cranial base. A definite diagnosis was made by biopsy, 3 cases were confirmed recurrence, and others 6 cases were proved mucous chronic inflammation and (or) osteoradionecrosis. The accuracy of FDG-PET was 33.3% (3/9), and the false positive rate was 66.7% (6/9).

CONCLUSIONSDiagnosis of recurrent or residual nasopharyngeal carcinomas in the skull base area with FDG-PET had high false-positive rate, final diagnosis must depend on histopathologic examination under endoscopy.

Adult ; Aged ; False Positive Reactions ; Female ; Fluorodeoxyglucose F18 ; Humans ; Male ; Middle Aged ; Nasopharyngeal Neoplasms ; diagnostic imaging ; drug therapy ; radiotherapy ; Positron-Emission Tomography ; methods ; Skull Base ; diagnostic imaging

OBJECTIVETo investigate the clinical characteristics, treatment modalities and prognostic factors of chordoma in the skull base.

METHODSThe data of 37 patients with chordoma in the skull base treated from 1970 to 2002 were retrospectively reviewed. Twenty-eighty received surgery plus radiotherapy, 8 radiotherapy alone and 1 surgery alone. The total dose of irradiation ranged from 30 - 75 Gy with a median of 60 Gy.

RESULTSThe rates of symptom relief and remarkable tumor regression at the end of radiotherapy were 86.5% and 48.6%, respectively. The nerve and endocrine function recovery was observed in some patients within six months to two years after treatment. The overall 1-, 3-, 5- and 10-year survival were 97.3%, 87.3%, 71.5% and 41.0%, respectively. The overall 1-, 3-, 5- and 10-year survival were 100.0%, 92.9%, 85.7% and 50.8% in patients with palsies of cranial nerves II-VIII, but 100.0%, 75.0%, 45.0% and 0 in patients with dysfunction of cranial nerves IX-XII, respectively (P = 0.04).

CONCLUSIONSurgery is the first option for chordoma, but resection with a safe margin is difficult to achieve due to the special anatomical location of the skull base. Local failure is the main cause of death. Radiotherapy is an important adjuvant for local control. The total dose should not be less than 60 Gy for prophylactic purpose, and more than 70 Gy should be given to patients with recurrence, residual lesions after surgery or those not operated. The injury of cranial nerves IX-XII suggests a poor prognosis.

Adolescent ; Adult ; Aged ; Child ; Chordoma ; mortality ; radiotherapy ; surgery ; Combined Modality Therapy ; Female ; Humans ; Male ; Middle Aged ; Prognosis ; Radiotherapy Dosage ; Retrospective Studies ; Skull Base Neoplasms ; mortality ; radiotherapy ; surgery ; Survival Rate

PURPOSE: Germ cell tumors have variable clinical characteristics according to the age, sex, primary site, and pathologic diagnosis. To provide the therapeutic principle, the clinical characteristics, response to the treatment, and prognostic factors were analyzed. METHODS: Medical records of 57 children who were diagnosed as malignant germ cell tumor in Seoul National University Children's Hospital from January 1986 till June 1996 were reviewed. RESULTS: 1) Fifty seven cases (male : 22, female : 43, true hermaphrodite : 1) were analyzed. The age of diagnosis was between 2 months and 15 years and median age was 4 year 7 month. 2) The primary sites were sacrococcygeal area (28%), intracranial area (25%), ovary (18%), testis (11%), retroperitoneum (7%), and mediastinum (4%) in order of frequency. Another cases were in liver, spinal cord, skull base, tongue, and ovary & testis in hermaphrodite. 3) Pathologic diagnosis was possible in 53 cases. Mixed germ cell tumor (37%), endodermal sinus tumor (23%), germinoma (13%), immature teratoma (8%), embryonal carcinoma (4%), and choriocarcinoma (2%) were included in order of frequency. 4) The stage by CCG/POG classification revealed that 6% of stage I, 19% of stage II, 43% of stage III, and 32% of stage IV in 53 cases. 5) After the initial surgery or needle biopsy, radiation therapy was done in the intracranial tumor with EP induction or modified CCG 8891, etc. and cis-VAB, EP, VAB-3, VAC, or CCG 8891, etc. was done in the extracranial tumor with/without radiation therapy. 6) The response rate revealed that 71% of complete response, 22% of partial response, and 7% of progressive disease in 45 cases. 7) The response rate was related to the primary site but not pathology and stage. All the gonadal tumors had complete response. 8) The 5 year-survival rate of all malignant germ cell tumors was 73%. Stage 4 or sacrococcygeal tumor had poor survival rate. CONCLUSIONS: Stage 4 and sacrococcygeal area were thought as the poor prognostic factors affecting survival. Survival rate of tumors at sacrococcygeal area were poor owing to the relatively younger age of onset, and poor response rate. The 5 year-survival rate of tumors treated with cis-VAB were 74% but shown complications frequently. So BEP will be preferred.
Age of Onset ; Biopsy, Needle ; Carcinoma, Embryonal ; Child ; Choriocarcinoma ; Classification ; Diagnosis ; Drug Therapy ; Endodermal Sinus Tumor ; Female ; Germ Cells* ; Germinoma ; Gonads ; Humans ; Liver ; Mediastinum ; Medical Records ; Neoplasms, Germ Cell and Embryonal* ; Ovary ; Pathology ; Pregnancy ; Seoul ; Skull Base ; Spinal Cord ; Survival Rate ; Teratoma ; Testis ; Tongue