Küttner's tumor: Unusual presentation with bilateral involvement of the lacrimal and submandibular glands

Oral cavity tumors may develop occult metastases to the cervical lymph nodes. Current imaging techniques and routine histopathologic methods may fail to detect lymph node micrometastases, but the surgeon has to electively dissect a neck at risk of developing clinical disease. Supraomohyoid neck dissection has been the elective surgery for treating a clinically negative neck in patients with oral cavity primaries. A literature review revealed that level IV nodes can be significantly affected by occult disease with and without metastases in level I-III lymph nodes. This means that level IV nodes have to be included in the supraomohyoid neck dissection, resulting in a more extensive surgical procedure to ensure a margin of oncological safety.     

Küttner's tumor: unusual presentation with bilateral involvement of the lacrimal and submandibular glands

Chronic sclerosing sialadenitis, known as Küttner's tumor (KT), is a chronic inflammatory disease of the salivary gland characterized by progressive periductal fibrosis, dilated ducts with a dense lymphocyte infiltration and lymphoid follicle formation, and acinar atropy. We report a unique case of KT in a 30-year-old female who presented with time-serial involvement of the bilateral lacrimal and submandibular glands with reactive follicular hyperplasia of bilateral upper cervical lymph nodes. All tissue samples of the submandibular and lacrimal glands were typical of KT on pathologic examination. There was no evidence of lymphoepithelial lesions or intraepithelial lymphocytes. This is the first case of KT involving both lacrimal and submandibular glands, suggesting that KT is closely related to an active local immune process.     

Küttner's tumor (chronic sclerosing sialadenitis) - a rare cause of submandibular gland enlargement

Küttner's tumor is a benign tumour-like lesion of the salivary glands. Predominantly affects the submandibular gland. It is also known as chronic sclerosing sialoadenitis or cirrhosis of submandibular gland. This is an underrecognized entity in the surgical pathology and cytology literature. Most patients experience recurrent pain, discharge and swelling that is often associated with eating, but others only have asymptomatic hard swelling of the submandibular gland. Histologic examination of the excised submandibular glands revealed preserved lobular architecture, thickening of interlobular septa by sclerotic tissue, dense lymphoplasmacytic infiltrate, preservation of ducts with periductal fibrosis, and variable loss of acini. The morphologic appearance, in conjunction with the elevated IgG4 expression, distinguishes chronic sclerosing sialadenitis from other inflammatory diseases of the salivary glands. Chronic sclerosing sialadenitis belongs to the spectrum of IgG4-related diseases. We present a case of Küttner's tumor in a 62-year-old female treated by surgery. Although this disease was first described by Küttner in 1896, this clinical entity which masquerades as carcinoma is underdiagnosed by many surgeons. There is not enough evidence to support any diagnostic means that could help in the differential diagnosis of this benign condition. Given the high rate of malignancy in firm, painless lesions of the submandibular gland, surgical excision is often advocated and Küttner's tumor is usually diagnosed by the histopathologist.     

Werner Kümmel

Ohne Zusammenfassung     

Case report: Meniere's disease and otosclerosis-Different outcomes of the same disease?

The etiologies of Meniere's disease and otosclerosis are largely unknown. An association between these two diseases has been proposed on both a clinical and histopathologic basis but the causal relationship is controversial. In this paper we report two families in which both otosclerosis and Meniere's disease are inherited as independent phenotypes suggesting that the two diseases represent different outcomes of the same mutation. Thus the Meniere's disease occasionally seen in otosclerotic patients might not be caused by otosclerosis, but rather by a molecular defect leading to endolymphatic hydrops and/or clinical otosclerosis.     

Castleman’s disease of the head and neck

Castlemans disease is an uncommon benign disease that causes progressive lymph node enlargement. We report 12 cases of Castlemans disease in the head and neck region in a retrospective review of the medical records of all patients with the pathological diagnosis of Castlemans disease during the period of 1993 through 2002. In the 12 patients, the neck was the most commonly involved site with 9 (75%) cases. Level III was the most common subsite (five cases). The most common sign in our study was an asymptomatic neck mass. No patient had any past histories that required medical attention. Preoperative work up such as fine-needle aspiration and radiographic study was not helpful for diagnostic confirmation. The histopathologic evaluation was the only way to make a definitive diagnosis. The histopathologic subtype of our study was hyaline-vascular type. Excision was curative for all cases. There was no evidence of recurrence with a minimum follow-up duration of 24 months.     

Multisystem Langerhans’ cell histiocytosis (Hand-Schüller-Christian disease) in an adult: a case report and review of the literature

Langerhans cell histiocytosis (LCH) is a rare and enigmatic clonal disorder that affects mainly children. It is characterized by single or multiple granulomatous mass lesions composed of cells with the Langerhans cell phenotype. Clinical presentation and behavior are heterogeneous and can range from a solitary lytic bone lesion (i.e., eosinophilic granuloma) with a favorable course to a fatal disseminated leukaemia-like form, with a wide spectrum of intermediate clinical presentations between these two extremes. Although LCH typically involves the bone, lesions can be found in almost all organs. We are reporting the case of a multisystem LCH in a 47-year-old patient who presented with a panhypopituitarism and diabetes insipidus, and who, 5 years later, developed mandibular, mastoid and femoral lesions. The final diagnosis of LCH was made on mandibular biopsy.     

Does the tonsillar surface flora differ in children with and without tonsillar disease?

OBJECTIVE: To investigate whether the tonsillar flora differ in children with and without adenotonsillar disease. MATERIAL AND METHODS: Tonsil surface swabs were taken from 218 children indicated for adenotonsillectomy because of moderate symptoms of recurrent tonsillopharyngitis or adenotonsillar hypertrophy (T+Ads group). Control swabs were taken from 100 children without symptoms of adenotonsillar disease who visited the ophthalmology clinic. Potential respiratory pathogens were identified. RESULTS: Potential respiratory pathogens were found in 54% of the T+Ads group, compared to 41% of the control group (p = 0.04). Haemophilus influenzae was the commonest pathogen in both groups, being found in 41% of the T+Ads group and 34% of the control group. Moraxella catarrhalis was found more often in the T+Ads group compared to the control group: 7% vs 0% (p = 0.004). H. influenzae was found in 32% of the children with recurrent tonsillitis, compared to 48% of the children with symptoms of tonsillar hypertrophy (p = 0.03). CONCLUSIONS: The prevalence of potential respiratory pathogens on the tonsillar surface of children with moderate symptoms of recurrent tonsillopharyngitis and/or tonsillar hypertrophy differs only slightly from that in children without symptoms of adenotonsillar disease. Variations in the microbial flora do not seem to play an essential role in the predisposition of these children to tonsillar disease.     

Survival and prognosis in Hürthle cell carcinoma of the thyroid gland

OBJECTIVE: To determine factors that affect survival in patients with Hürthle cell carcinoma of the thyroid gland. METHODS: Data for all cases of Hürthle cell carcinoma that occurred between January 1, 1988, and December 31, 1998, were extracted from the Surveillance, Epidemiology, and End Results database. Clinical data regarding age, sex, tumor size, primary site extension, nodal involvement, and vital status were tabulated. Patients with distant metastases were excluded, and Kaplan-Meier survival analysis was conducted. Survival data for patients with Hürthle cell carcinoma were compared with data for a control group of patients with follicular cell carcinoma matched for age, sex, tumor size, and local disease extension. Cox multivariate regression analysis was conducted to determine the effect of predictor variables on overall survival in Hürthle cell carcinoma. RESULTS: We identified 555 cases of nonmetastatic Hürthle cell carcinoma (mean age at diagnosis, 55.9 years; women, 67.9%). The primary tumor was intrathyroidal in 83.8% of patients, whereas 11.2% had minor local extension. Mean tumor size was 3.5 cm. Mean, 5-year, and 10-year survival for Hürthle cell carcinoma was 109 months, 85.1%, and 71.1%, respectively. Mean survival for 411 matched patients with follicular cell carcinoma was 113 months, which was not statistically different from that of patients with Hürthle cell carcinoma (P =.47, log-rank test). On multivariate analysis, increasing age at diagnosis, male sex, and increasing tumor size were statistically significant predictors of poor survival; degree of primary site extension did not affect survival. CONCLUSIONS: Overall survival for Hürthle cell carcinoma is similar to that of comparably staged follicular cell carcinoma. Increasing age, male sex, and increasing tumor size substantially diminish survival in patients with Hürthle cell carcinoma.     

Comparative study between the caloric vestibular and the video-head impulse tests in unilateral Menière’s disease

Objective: Compare the caloric vestibular test (CVT) and the video head impulse test (vHIT) in the evaluation of Meniere’s disease (MD) and to analyze their diagnostic accuracy.

Materials and methods: Retrospective review of CVT and vHIT performed in MD patients at a tertiary care referral center in Spain.

Results: A total of 88 patients were reviewed. The CVT was abnormal in 67% (n?=?59) of patients, while the vHIT was abnormal in 66% (n?=?58) of them. Agreement between both tests was poor, regardless of whether the horizontal semicircular canal (SCC) or all SCC were analyzed (kappa?=?.21). Anterior SCC pathology was found in 30% (n?=?27) of the subjects while 51% (n?=?45) had altered gains in the posterior SCC.

Conclusions: Our study shows that patients with MD can have functional deficit involving the vertical semicircular canals, which cannot be detected by the CVT alone. Therefore, both tests should be used in a complementary fashion.     

Obstructing lesions of the endolymphatic sac and duct mimicking Ménière's disease

In this retrospective case series and literature review, we demonstrate that temporal bone lesions that obstruct the endolymphatic sac or duct can cause symptoms of Ménière's disease. This finding is likely attributable to endolymphatic hydrops; initially, such cases typically masquerade as Ménière's disease. Between July 1995 and April 2002, a total of 379 patients were treated for an initial diagnosis of Ménière's disease at our institution. Among this group, 3 patients were found to have an obstructing lesion of the endolymphatic sac or duct that we felt was causally related to their Ménière's-like symptomatology. We reviewed these cases and noted the similarities in each patient's presentation, including a common pathophysiology. On imaging studies, each patient had a different pathologic lesion that involved the endolymphatic sac or duct: patient 1 had a jugular megabulb, and she was ultimately treated with vestibular nerve section; patient 2 had a cholesterol granuloma, which was treated with surgical excision; patient 3 had an endolymphatic sac tumor that was treated with surgical excision. As has been suggested in previous reports, not all cases of Ménière's disease are idiopathic. We conclude that obstruction of the endolymphatic sac or vestibular aqueduct by a mass lesion or vascular anomaly can lead to vestibulocochlear pathology that mimics Ménière's disease.     

Periodic fever and pharyngitis in young children: a new disease for the otolaryngologist?

OBJECTIVE: A clinical entity consisting of periodic fever associated with aphthous stomatitis, pharyngitis and cervical adenitis termed "PFAPA syndrome" in young children (<5 years old) may be unfamiliar to otolaryngologists. We present our 5-year experience of PFAPA syndrome. DESIGN: Case series. SETTING: Tertiary academic. PATIENTS: A 5-year retrospective chart review for children (<5 years old) who have undergone tonsillectomies with and without adenoidectomies was conducted. Medical records from subjects who underwent the procedures for recurrent pharyngitis were reviewed with reference to a history of periodic fever and stomatitis associated with pharyngitis. INTERVENTIONS: Tonsillectomy with and without adenoidectomy. MAIN OUTCOME MEASURE: The objective measure was a comparison of the number of visits to the primary care physician for pharyngitis associated with fever in a 3-month period before and after the surgical intervention. The subjective measure was a telephone interview evaluating preoperative and postoperative symptoms. RESULTS: Of the 117 patients identified, 22 (19%) underwent surgery for recurrent pharyngitis. Five subjects (average age, 2.5 years) were identified as having PFAPA syndrome. The average number of preoperative PFAPA-related complaints was 11.6 compared with 0.2 for the number of postoperative PFAPA-related complaints (P=.03). CONCLUSIONS: Our experience suggests that PFAPA syndrome is an uncommon disease. Most of these children have undergone workup(s) for sepsis performed by their pediatricians because of the associated high fever. The clinical history of this cohort was quite distinctive. This small sample suggests a significant decrease if not cessation of pharyngitis following surgical intervention.     

Summating potential-action potential waveform amplitude and width in the diagnosis of Menière's disease

OBJECTIVES: It has been suggested that analyzing the width and amplitude of the summating potential-action potential (SP-AP) waveforms can increase the sensitivity of electrocochleography. The objective of this study was to evaluate the ratio of SP to AP amplitude (SP/AP) and SP-AP waveform width, as well as the AP latency difference to condensation and rarefaction clicks, for the diagnosis of Menière's disease. STUDY DESIGN: This was a prospective, controlled study. METHODS: We used transtympanic electrocochleography to evaluate 21 patients with definite Menière's disease and 19 normal-hearing patients with other cochleovestibular disorders, comparing SP/AP, AP latency difference, and SP-AP waveform widths as well as calculating the diagnostic sensitivity of these parameters. RESULTS: Mean SP-AP waveform width was 1.89 ms in the study group and 1.58 ms in the control group. Mean SP/AP was 0.37 in the study group and 0.22 in the control group. The mean product of SP-AP waveform width and SP/AP was 75.26 ms% in the study group and 34.60 ms% in the control group. Mean AP latency difference was 0.13 ms in the study group and 0.07 ms in the control group. For the parameters evaluated, the differences between the groups were statistically significant. In the study group, the sensitivities for the width of the SP-AP waveform, the SP/AP, and the AP latency difference were 33.3%, 52.4%, and 23.8%, respectively. CONCLUSIONS: The use of the parameters evaluated did not increase the sensitivity of the electrocochleography, whether used in isolation or in conjunction with the SP/AP. Determining SP/AP presented the greatest sensitivity.     

Comparing the sensitivity and specificity of cervical vestibular-evoked myogenic potentials and electrocochleography in the diagnosis of Ménière’s disease

Abstract

Objective: To compare the sensitivity and specificity of objective cervical vestibular-evoked myogenic potential (cVEMP) tuning curves and electrocochleography (ECochG) for the diagnosis of Ménière’s disease (MD).

Design: Sensitivity and specificity were calculated from 95% normative ranges of 500?Hz cVEMP threshold and ECochG SP/AP amplitude ratios.

Measures: Extra-tympanic ECochG testing to 90?dB nHL clicks and cVEMP threshold tuning curves (250–1000?Hz).

Study sample: We tested 15 patients (30 ears) diagnosed with definite bilateral MD based on the clinical criteria proposed by the American Academy of Otolaryngology Head and Neck surgery, 1995 (assumed gold standard) and 20 controls.

Results: 500?Hz cVEMP threshold was the most promising parameter to differentiate MD ears from controls. cVEMP and ECochG showed high specificity (83.3 and 100%, respectively) and low to moderate sensitivity (22.2 and 71.4%) for long term MD. ECochG sensitivity increased to 89% during a symptomatic period, compared to 33% for cVEMP. However, ECochG can be difficult to schedule during symptomatic periods. Sensitivity of cVEMP for the diagnosis of MD appears limited.

Conclusions: ECochG has higher sensitivity than cVEMP in the diagnosis of Ménière’s patients, but the ECochG SP/AP amplitude ratio measure is not perfect for the diagnosis of MD.     

Relationship of vestibular aqueduct and inner ear pressure in Ménière's disease and the normal population

OBJECTIVE: Etiopathogenesis of Ménière's disease has not been resolved. The principal histopathologic finding in this disease is endolymphatic hydrops. The majority of radiologic and histopathologic studies demonstrated a narrow vestibular aqueduct in Ménière's disease. There is no study in the literature investigating the relationship between inner ear pressure and vestibular aqueduct dimensions. Static acoustic compliance is a noninvasive procedure that is thought to measure perilymphatic pressure at the footplate. An increase in mechanical fluid pressure in the inner ear is transmitted to the footplate of the stapes. This causes a reduction in the compliance at the drum. The aim of this study is to investigate the relationship between vestibular aqueduct dimensions and static acoustic compliance in Ménière's disease and the normal population. STUDY DESIGN: Prospective study. METHODS: Forty patients with Ménière's disease and 40 healthy individuals with no otolaryngologic disorders were the subjects of this study. Each group was further divided into two according to static compliance value (normal and low static compliance). In these four groups dimensions of vestibular aqueduct were determined radiologically by high-resolution computerized tomography and correlated with normal and low static acoustic compliance values (normal and high perilymphatic pressure). RESULTS: The results demonstrated that vestibular aqueduct is narrower in patients with Ménière's disease than the normal population. However, there is no relationship between vestibular aqueduct dimensions and inner ear pressure obtained by static acoustic compliance measurements.