The bullet that hit a nerve: the history of Lucja Frey and her syndrome.
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Biomedical subjects
Publications and source records attributed to P Dulguerov.
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Paraganglioma is a rare benign tumor arising from the sympathetic nervous system. Here we describe an exceptional case of a paraganglioma located in the nasopharynx with an extension through the clivus up to the dura. Atypically, no contact with any major vessels was found. A radical resection of the mass was performed by an anterior transmaxillary approach through a Le Fort I osteotomy. One year follow up reveals no signs of local or distant recurrence. No cosmetic changes can be observed after the surgery and nasal and masticatory functions are unmodified. We review the clinical presentation, workup of paraganglioma, as well as the surgical approaches to the clivus.
CD9 is a transmembrane protein that has been implicated in cell adhesion, motility and proliferation, and numerous studies have demonstrated the prognostic value of its expression in different solid tumours. The purpose of this study is to determine the predictive value of CD9 in squamous cell carcinoma (SCC) of the head and neck. A total of 153 cases were examined for CD9 expression using immunohistochemistry applied on formalin-fixed, paraffin-embedded tissue. Cases were stratified in two categories depending on CD9 expression, as positive (>/=50% positive cells) or reduced (<50%). In all, 108 cases were positive for CD9 (85 cases with membranous, and 23 with both membranous and cytoplasmic staining) and 45 reduced expression. Reduced CD9 expression was significantly associated with high grade (P=0.0007) and lower disease-free survival (DFS) (P=0.017). The latter retained its significance in the multivariate analysis. When the 23 cases with both membranous and cytoplasmic patterns were studied as a separate subgroup, there were significant associations between CD9 expression and tumour grade (P=0.025) (95% CI 11-68), tumour stage (P=0.08) (95% CI 3.5-86) and the occurrence of any failure (P=0.083) (95% CI -1.7-57). Immunohistochemical CD9 expression proved to be an independent prognostic factor in SCC of the head and neck, and it may detect patients at a high risk of recurrence. In addition, the cytoplasmic pattern seems to have an even more significant value. However, this finding is limited to the small number of cases with this pattern.
The case of a 34-year-old otherwise healthy woman with retropharyngeal abscess due to a fishbone injury and presenting with neck stiffness and aphagia without visualization of a pharyngeal mucosal lesion is reported. The case illustrates that sore throat with symptoms out of proportion to oropharyngeal findings should prompt a search for pathologies other than simple pharyngotonsillitis. Other typical symptoms of a retropharyngeal abscess are high fever, dysphagia, hot potato voice and, less commonly, dyspnea and sepsis. Retropharyngeal abscess in adults occurs most often as a complication of a spread of infection from a pharyngeal focus, a foreign body injury, an iatrogenic trauma due to tracheal intubation or endoscopy, and blunt or perforating neck trauma. Contrary to children, a retropharyngeal abscess in adults without loco-regional infection or preceding trauma is very rare. This case illustrates how important imaging investigations (CT-scan) are in order to locate foreign bodies and to decide on surgical management. Aetiology, presenting signs, symptoms, methods of diagnosis, treatment and complications of a retropharyngeal abscess are briefly discussed.
BACKGROUND: The authors reviewed treatment results in patients with nasal and paranasal sinus carcinoma from a large retrospective cohort and conducted a systematic literature review. METHODS: Two hundred twenty patients who were treated between 1975 and 1994 with a minimum follow-up of 4 years were reviewed retrospectively. A systematic review of published articles on patients with malignancies of the nasal and paranasal sinuses during the preceding 40 years was performed. RESULTS: The 5-year survival rate was 40%, and the local control rate was 59%. The 5-year actuarial survival rate was 63%, and the local control rate was 57%. Factors that were associated statistically with a worse prognosis, with results expressed as 5-year actuarial specific survival rates, included the following: 1) histology, with rates of 79% for patients with glandular carcinoma, 78% for patients with adenocarcinoma, 60% for patients with squamous cell carcinoma, and 40% for patients with undifferentiated carcinoma; 2) T classification, with rates of 91%, 64%, 72%, and 49% for patients with T1, T2, T3, and T4 tumors, respectively; 3) localization, with rates of 77% for patients with tumors of the nasal cavity, 62% for patients with tumors of the maxillary sinus, and 48% for patients with tumors of the ethmoid sinus; 4) treatment, with rates of 79% for patients who underwent surgery alone, 66% for patients who were treated with a combination of surgery and radiation, and 57% for patients who were treated exclusively with radiotherapy. Local extension factors that were associated with a worse prognosis included extension to the pterygomaxillary fossa, extension to the frontal and sphenoid sinuses, the erosion of the cribriform plate, and invasion of the dura. In the presence of an intraorbital invasion, enucleation was associated with better survival. In multivariate analysis, tumor histology, extension to the pterygomaxillary fossa, and invasion of the dura remained significant. Systematic review data demonstrated a progressive improvement of results for patients with squamous cell and glandular carcinoma, maxillary and ethmoid sinus primary tumors, and most treatment modalities. CONCLUSIONS: Progress in outcome for patients with nasal and paranasal carcinoma has been made during the last 40 years. These data may be used to make baseline comparisons for evaluating newer treatment strategies.
Sialolithiasis is a relatively frequent occurrence; however, cases of sialolithiasis originating around a "foreign body" nidus are rare. We describe a patient with submandibular sialolithiasis organized around a vegetal nidus and discuss the etiology of the case.
PURPOSE OF THE STUDY: To assess whether early stage (pT1-2,pN0-1) oral cavity carcinoma is adequately treated by radical surgical resection alone. MATERIAL AND METHODS: Prospective multicenter study. Of 105 patients with cT1-2 cN0-1 oral carcinoma treated in conformity with the study design, 12 had to be excluded because of tumor-positive margins or pN stage > N1. The remaining 93 patients were monitored for at least 2 years. RESULTS: Seventeen patients had local or regional recurrence develop. In 12 of the 17 patients locoregional control was achieved by second treatment. Overall, the 4-year disease-specific survival probability was 94%. Patients treated initially without selective neck dissection had significantly higher recurrence rates than those with neck dissection, although the survival probability was not adversely affected. CONCLUSIONS: Early (pT1-2, pN0-1) squamous cell carcinoma of the oral cavity is adequately treated by surgery alone, provided the resection margins are tumor free. On the basis of the presented data, we would also advocate routine selective neck dissection.
Our objective was to review recent developments in diagnosis, staging, and treatment of esthesioneuroblastoma (ENB). A meta-analysis of publications between 1990 and 2000 was carried out, and studies were classified according to their main subject: origin/aetiology of ENB, histopathological diagnosis, and treatment. Data so far point to the basal progenitor cells of the olfactory epithelium as the origin of ENB. Histopathological diagnosis remains difficult and is based on results of antigen expression detected through a panel of antibodies by immunohistochemistry. RT-PCR of HASH expression could be a specific marker of ENB. Overall and disease-free survival at 5 years averaged 45% (SD 22) and 41% (SD 21) in the studies included in the meta-analysis. Survival in Hyams' grades I-II was 56% (SD 20) compared with 25% (SD 20) in grades III-IV (odds ratio 6.2). In patients with metastases in cervical lymph nodes (on average 5% of the total) survival was 29%, compared with 64% for patients with N0 disease (odds ratio 5.1). Survival according to treatment modalities was 65% for surgery plus radiotherapy, 51% for radiotherapy and chemotherapy, 48% for surgery, 47% for surgery plus radiotherapy and chemotherapy, and 37% for radiotherapy alone. The histopathological grading according to Hyams and the presence of cervical lymph-node metastases emerged as prognostic factors. A combination of surgery and radiotherapy seems to be the optimum approach to treatment. The exact role of chemotherapy in treatment protocols is still unclear. The role of elective neck dissection is unclear.
OBJECTIVE: To present our initial experience with sialendoscopy of the parotid duct. STUDY DESIGN METHODS: Diagnostic and interventional sialendoscopy procedures were performed in 79 and 55 cases, respectively. Diagnostic sialendoscopy was used to classify ductal lesions into sialolithiasis, stenosis, sialodochitis, and polyps. Interventional sialendoscopy was used to treat these disorders. The type of endoscope used, the type of sialolithiasis fragmentation and/or extraction device used, the total number of procedures, the type of anesthesia, and the number and size of the sialoliths removed were the dependent variables. The outcome variable was the endoscopic clearing of the ductal tree and resolution of symptoms. RESULTS: Diagnostic sialendoscopy was possible in all cases, with an average duration of 26+/-14 minutes and no complications. Interventional sialendoscopy was successful in 85% of cases, with an average duration of 73+/-43 minutes (+/- standard deviation). Multiple procedures were performed in 45% of cases, general anesthesia was used in 24%, and parotidectomy in 2%. Multiple sialoliths were found in 58% of ducts and associated with more procedures under general anesthesia and longer operations. The average size of sialoliths was 3.2+/-1.3 mm; larger stones were associated with more procedures under general anesthesia, longer and multiple procedures, use of fragmentation, and sialendoscopy failures. Sialolithiasis fragmentation was required in 10% of cases, with a success rate of 70%. Semirigid sialendoscopes performed better than flexible ones. Complications were mostly minor but were encountered in 12% of cases. CONCLUSIONS: Diagnostic sialendoscopy is a new technique for evaluating salivary duct disease, a technique which is associated with low morbidity. Interventional sialendoscopy allows the extraction of sialoliths in most patients, preventing open gland excision.
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OBJECTIVE: To evaluate the feasibility and efficacy of endoscopic neck dissection (END) in human cadavers. STUDY DESIGN: Experimental self-controlled study. METHODS: END on five human cadavers through three openings: one for the camera, one for the dissecting instrument, and one for a grasping one. The tissue specimens removed were divided into traditional neck groups (I to V). After the completion of END, open neck dissection was performed using standard surgical techniques and the remaining tissue within each neck group was retrieved. The important neck structures (carotid artery, internal jugular vein, cranial nerves X, XI, and XII, phrenic nerve) were evaluated for lesions. A pathologist evaluated each specimen, without knowing its exact origin in terms of neck group or side, and type of surgical technique used. For each specimen, the number of retrieved lymph nodes and their anatomic integrity was analyzed. RESULTS: Ten neck dissections were performed on 5 cadavers, without any major difficulty. An injury of the internal jugular vein occurred twice and once the phrenic nerve was cut. Little tissue was usually left for open surgical dissection. The average number of retrieved lymph nodes by endoscopy was 4.9 +/- 2.7 (mean +/- standard deviation). Completion open neck dissection retrieved an additional 0.5 +/- 0.5 lymph nodes. Efficacy of END was 92 +/- 10%. The majority of retrieved lymph nodes were intact but exhibited important postmortem autolysis artifacts. CONCLUSIONS: Endoscopic neck dissection is possible in human cadavers and is free of lesions to major structures. The majority of neck lymph nodes can be removed endoscopically.
We reviewed the clinical history of 48 consecutive patients who underwent submandibular gland removal for radiologically proven sialolithiasis. The specimens were examined by a pathologist blinded to the clinical data. A histopathologic classification into I of 3 grades was established by evaluating the degrees of atrophy, fibrosis, and inflammation. A correlation between the clinical and pathological variables was sought in order to define clinical variables that would predict abnormal submandibular glands that required extirpation. A significant percentage of the submandibular glands exhibited normal histologic findings. The patients with normal submandibular glands had a clinical evolution similar to that of other patients with severely damaged glands. The only clinical variable that correlated with increased histopathologic alteration was the patient's age. In view of the newly available diagnostic and therapeutic techniques for sialolithiasis, a conservative attitude to submandibular gland resection appears justified.
BACKGROUND: Accelerated radiotherapy (RT) represents a promising method with which to improve the treatment outcome in patients with head and neck carcinoma. However, its applicability to elderly patients has not been well established. This study assessed treatment toxicities, patient compliance, and oncologic results in patients age >/= 70 years who were treated with an accelerated concomitant boost RT schedule. METHODS: Between 1991 and 1997, 39 patients aged >/= 70 years (mean, 75 +/- 6 years) presenting with carcinomas of the oral cavity, pharynx, or larynx were treated radically with a modified concomitant boost RT schedule (planned dose of 69.9 grays [Gy] over 38 days). Based on American Joint Committee on Cancer staging, there were 14 patients with Stage I-II disease and 25 patients with Stage III-IV disease. Eighty-one patients age < 70 years who were treated with the same RT schedule served as a comparative group. The median follow-up for the surviving patients was 19 months (range, 3-65 months) and 23 months (range, 2-76 months), respectively, for the elderly and younger patient groups. RESULTS: The planned RT schedule was completed in all cases. Three patients (8%) in the elderly group and none in the younger group had an unplanned treatment interruption because of acute toxicity or lack of compliance (P = 0.03). The median tumor dose (69.9 Gy; range, 67-73 Gy) and the median overall treatment time (41 days; range, 36-60 days) were identical in both groups. According to the Radiation Therapy Oncology Group grading system, Grade 3-4 acute reactions were observed in 66% of elderly patients and in 71% of younger patients. Ten elderly patients (26%) and 19 younger patients (23%) required a nasogastric tube or a percutaneous gastrostomy for feeding, with a median weight loss of 4.1 kg and 4.4 kg, respectively, in the 2 groups. Grade 3-4 late complications were observed in 3% of the elderly patients and 10% of the younger patients (P = 0.43). Both elderly and younger patients had similar results with regard to 3-year actuarial overall survival (68% vs. 62%; P = 0.48) and locoregional control (73% vs. 68%; P = 0.31). CONCLUSIONS: The current study suggests that an accelerated concomitant boost RT schedule is feasible in elderly patients who are physically healthy enough to undergo curative treatment. The acute and late toxicities appear to be similar to those observed in younger patients, and treatment outcomes appear to be comparable.
OBJECTIVE: To evaluate the possibility, complications, and efficacy of endoscopic neck dissection (END) in a porcine model. DESIGN: Experimental self-controlled study. SUBJECTS: Minipigs. INTERVENTION: Endoscopic neck dissection was performed using general anesthesia with techniques adapted from laparoscopic surgery. The tissue specimens removed were divided according to porcine equivalents of human neck groups. After the completion of END, open-neck dissection was performed using standard surgical techniques, and the remaining tissue within each neck group was retrieved. A pathologist evaluated each specimen without knowing its exact origin in terms of neck group or side and the type of surgical technique used. For each specimen, the number of retrieved lymph nodes and their anatomical integrity were analyzed. RESULTS: Ten neck dissections were performed in 8 minipigs without any major complications. The number of retrieved lymph nodes by END was 18.4 +/- 7.4 (mean +/- SD). Completed open-neck dissection retrieved an additional 3.3 +/- 1.8 lymph nodes. The efficacy rate of END was 88% +/- 10% (+/ -SD). The majority of retrieved lymph nodes were intact, with less than 5% of nodes exhibiting crushing artifacts. CONCLUSIONS: Endoscopic neck dissection in a porcine model seems to be free of major complications and able to retrieve the majority of neck lymph nodes. A larger number of animals and their survival need to be studied before human studies can begin.
BACKGROUND: This study was conducted to evaluate quality of life (QOL) and functional outcome in patients with carcinomas of the larynx and hypopharynx treated with accelerated radiotherapy (RT). METHODS: Between January 1991 and September 1996, 21 patients treated with accelerated concomitant boost RT schedule (69.9 Gy in 5. 5 weeks) for laryngeal (n = 10) or hypopharyngeal (n = 11) carcinomas and who remained free of disease at 1-year minimum follow-up were evaluated. The functional outcome was assessed by the subjective Performance Status Scale for Head and Neck cancer (PSSHN) and general QOL by the European Organization for Research and Treatment of Cancer Core QOL questionnaire (EORTC QLQ-C30). The median length of follow-up was 37 months (range, 13 to 75). RESULTS: The PSSHN scores were 89, 84, and 86, respectively, for eating in public, understandability of speech and normalcy of diet (100 = normal function). Significantly lower scores for understandability of speech were observed in patients with advanced and laryngeal carcinomas. Normalcy of diet was affected negatively by the severity of xerostomia. All mean functional scale scores of the EORTC QLQ-C30 module were 20% to 25% below the higher score. Most of these scale scores were significantly affected by the severity of xerostomia. CONCLUSIONS: Patients treated with concomitant boost RT for laryngeal and hypopharyngeal carcinomas appear to have similar QOL and functional outcome to those reported for patients treated with conventional or hyperfractionated RT. As expected, many QOL scales were affected by the severity of xero- stomia.
The goal of this work is to present our results of the intradermic infiltration with botulinum toxin in patients with Frey syndrome. Sixteen hemifaces in 15 patients were studied. Gustatory stimulation was evoked by sucking on a slice of lemon while measurements were done on both hemifaces, with the normal side being used as a control. Skin temperature and color (erythema) were measured with a digital surface thermometer and a skin chromameter, respectively. Sweat quantity and surface were measured by using the previously described blotting paper and iodine-sublimated paper histogram methods, respectively. Testing was repeated 2 weeks after skin infiltration with botulinum toxin (dilution of 50 U/mL). The interinjection distances were 1 cm, and 0.1 mL (5 U) was infiltrated at each injection site. Frey syndrome complaints disappeared in all patients. Small residual amounts of sweat were measurable. The difference in sweat quantity before and after botulinum toxin infiltration was significant in every patient (P < 0.001). Skin temperature and color measurement gave inconclusive results. In conclusion, Frey syndrome treatment with botulinum toxin is an efficient and well-tolerated technique. Further work should address the optimal injection parameters.
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PURPOSE: To evaluate the accuracy of magnetic resonance (MR) sialography in detecting salivary glandular calculi and ductal stenoses. MATERIALS AND METHODS: In a prospective study, 64 salivary glands in 61 consecutive patients with acute or recurrent parotid or submandibular glandular swelling were examined by using three-dimensional (3D) extended-phase conjugate-symmetry rapid spin-echo (EXPRESS) MR imaging. Transverse and sagittal-oblique source images and maximum intensity projection images were obtained. All MR images were analyzed independently by two radiologists, without knowledge of the final diagnosis. The reference standard was conventional sialography, ultrasonography (US), and sialendoscopy with or without surgery in 31 glands and was conventional sialography and US in 33 glands. RESULTS: Final diagnoses included sialolithiasis (n = 23), sialolithiasis and stenosis (n = 9), stenosis without lithiasis (n = 11), early Sjögren syndrome without ductal stenosis (n = 2), ductal displacement (n = 3), and normal salivary glands (n = 16). The sensitivity, specificity, and positive and negative predictive values of MR sialography to detect calculi were 91%, 94%-97%, 93%-97%, and 91%, respectively. False-negative readings occurred due to calculi with a diameter of 2-3 mm in nondilated salivary ducts. Ductal stenosis was assessed, with a sensitivity of 100%, specificity of 93%-98%, positive predictive value of 87%-95%, and negative predictive value of 100%. Interobserver agreement was very good (kappa = 0.85-0.97). CONCLUSION: MR sialography with 3D EXPRESS imaging enables reliable prediction of salivary gland calculi and stenoses.