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Granular cell tumor of the uvula.

We describe the first case of granular cell tumor (GCT) of the uvula, which occurred in an 18-year-old woman. GCT is a relatively rare tumor, and is almost always benign. The most frequent locations are the skin and the oral cavity. These tumors usually present clinically as small, slowly growing tumors of characteristic histological appearance. Although its histogenesis is uncertain, our study as well as recent reports support a currently held theory of a neurogenic derivation. The recommended treatment for the granular cell tumor is local excision whenever possible.

Adolescent↗

[Malignant melanoma of the uvula and soft palate].

Malignant melanoma of the uvula and soft palate is very rare. We describe a case demonstrating the typical aggressive behavior of mucosal melanoma in contrast to cutaneous melanoma. Regional lymph node and distant metastases often develop rapidly in this type of melanoma. Despite aggressive surgical approaches, other treatment options are often limited and usually palliative. Therefore, early diagnosis is critical in patients with mucosal melanoma.

Adult↗

Definitive radiation treatment for early stage carcinoma of the soft palate and uvula: the indications for iridium 192 implantation.

From 1971 to 1984 59 T1 and T2 carcinomas of the soft palate and uvula were treated definitively by irradiation at the Henri Mondor hospital. Included are ten patients previously irradiated to the oropharyngeal area for either a carcinoma of the soft palate or another malignancy. Sixteen patients were treated by external irradiation alone, 14 by Iridium 192 implantation, and 29 by a combination of the two. Two techniques of implantation were used: the guide gutter technique (33 patients) and the plastic tube technique (10 patients). Clinically negative neck nodes (51/59) either received prophylactic telecobalt therapy (39/51) or were surveilled (12/51). Clinically involved nodes (8/59) were managed either by external irradiation alone (4/8) or combined with neck dissection (4/8). Local failure was 25% (4/16) after exclusive telecobalt therapy, 18% (5/19) after combined telecobalt therapy and implantation, and 0% (0/14) after Iridium 192 implantation alone. No local failures were seen with the plastic tube technique (0/10) as compared to 15% (5/33) for guide gutters. Only two nodal failures were observed (2/59: 3%). Crude 5-year disease-free survival was 33%. Severe complications were limited to one osteonecrosis, one soft tissue necrosis, and one partial palatal incompetence. Salivary impairment was reduced when implantation was used for part or all of the treatment. We recommend 45 Gy external radiation followed by 30 Gy from Iridium 192 implantation using the plastic tube method unless there has been prior oropharyngeal irradiation, in which case we give 60 Gy from implantation alone. For clinically negative neck nodes, we recommend 45 Gy prophylactic external neck irradiation. For clinically positive lymph nodes, this should be followed by either a 25 to 30 Gy boost to the involved nodes or a neck dissection.

Brachytherapy↗

Velopharyngeal insufficiency due to hypoplasia of the musculus uvulae and occult submucous cleft palate.

With the help of nasopharyngoscopy, it was possible to delineate specific morphologic changes in the palates of patients with velopharyngeal insufficiency, without an overt cleft and without the triad of symptoms of submucous cleft palate, visible through the oral cavity. Such malformations are part of the broad spectrum of the faulty midline mesodermal fusion of the palate. This anomaly is aptly called occult submucous cleft palate, because it can only be detected by viewing the functioning palate from the nasal surface. The musculus uvulae is either absent or deficient and is frequently associated with some degree of muscular diastasis that does not involve the oral surface. Like the cleft of the secondary palate, the submucous cleft palate often occurs as part of a generalized syndrome of multiple malformations.

Abnormalities, Multiple↗

Squamous carcinoma of the uvula.

From a study of the clinical courses of nine patients with squamous carcinoma of the uvula and a review of the clinical literature involving such lesions, we have concluded that these tumors have a very poor prognosis when metastases occur, despite seemingly adequate initial therapy to the primary tumor. Consequently, we believe that more aggressive treatment (which may include irradiation of even subclinically involved neck nodes) should be considered.

Carcinoma, Squamous Cell↗

A histopathological comparison of the uvula between snorers and non-snorers.

Snoring is a very common problem but there are few publications on the histological findings of the soft palate/uvula and these lack consistency. The relative proportions of tissue types in the base of the uvula removed from 17 adults who underwent uvulopalatopharyngoplasty for heavy snoring were therefore compared with 14 cadaveric specimens. The mean percentage of muscle from the snoring group was 12.1% compared with 7.2% in the control group (P < 0.05). The percentage of fibrous tissue was greater in the cadavers (52.8% vs 45.5% in the snorers, P < 0.05). The percentage of muscle was inversely related to the percentage of fibrous tissue in the snoring group (P < 0.02). We hypothesize that these changes are a consequence of the repetitive forces on the soft palate during snoring rather than being related to the pathogenesis of snoring.

Adult↗

Glycogen-rich clear cell carcinoma arising from minor salivary glands of the uvula. A case report.

Glycogen-rich clear cell carcinoma arising from minor salivary glands of the uvula in a 35-year-old woman is reported. This tumor was composed of nests and cords of clear cells containing cytoplasmic glycogen but no mucin. The epithelial nature of this lesion was obvious because of positive immunoreactivity for cytokeratin and epithelial membrane antigen, and the presence of immature lumina and intercellular spaces lined by many microvilli, associated with desmosomal junctions and basal lamina, as revealed by ultrastructural study. However, no myoepithelial cells could be detected. From these findings, it may be concluded that this tumor corresponds to glycogen-rich clear cell carcinoma (a variant of clear cell tumor), revealing glandular differentiation.

Adenocarcinoma↗

Paroxysmal alternating skew deviation and nystagmus after partial destruction of the uvula.

A patient with suspected brain stem glioma involving the area of the left vestibular nuclei and cerebellar peduncle, developed paroxysmal alternating skew deviation and direction changing nystagmus after biopsy of the inferior cerebellar vermis resulting in destruction of the uvula. Between attacks she had right over left skew deviation with asymptomatic right beating horizontal nystagmus. Slow phases of the resting nystagmus showed increasing velocity, similar to congenital nystagmus. At intervals of 40-50 seconds, paroxysmal reversal of her skew deviation occurred, accompanied by violent left beating horizontal torsional nystagmus lasting 10-12 seconds and causing severe oscillopsia. It is proposed that this complex paroxysmal eye movement disorder results from (1) a lesion in the left vestibular nuclei causing right over left skew and right beating resting nystagmus and (2) a disruption of cerebellar inhibition of vestibular nuclei, causing alternating activity in the vestibular system with intermittent reversal of the skew deviation and paroxysmal nystagmus towards the side of the lesion.

Adult↗

Long uvula: an unusual cause of chronic cough.

Chronic cough is a distressing symptom for patients and parents in paediatric practice. Investigations of the child with persistent cough can be simple or complex depending on the underlying cause. There are some unusual reasons for chronic cough, which need to be kept in mind when investigating the cause. A case of a long, floppy uvula causing chronic cough through mechanical irritation of upper airway is reported.

Chronic Disease↗

Carcinomas of the anterior tonsillar pillar and the soft palate--uvula: treatment by radiation therapy.

Sixty-six patients with carcinomas of the anterior tonsillar pillar and/or soft palate-uvula underwent radiation therapy. Three methods were used: (a) conventional fractionation, (b) a combination of conventional fractionation and radon seed implant, and (c) split course therapy in which two one-week courses were separated by two weeks rest. On time, dose, and fractionation (TDF) factor analysis, no significant difference in the success of tumor control was found between the methods. The determinate disease-free survival at two years was 82.4% for T1, 62.5% for T2, and 56.3% for T3--4 lesions; for the whole group, the two- and five-year determinate values were 67.3% and 59.4%, respectively.

Adult↗

Characteristics of the genioglossus and musculus uvulae in sleep apnea hypopnea syndrome and in snorers.

Genioglossus (GG) activity has been extensively studied by electromyographic recordings in the investigation of the pathophysiology of sleep apnea-hypopnea syndrome (SAHS). However, the effective force developed by this upper airway (UA) dilator muscle depends on its metabolic and histochemical characteristics. The aim of this study was to compare the metabolic and fiber type characteristics of two UA dilator muscles, musculus uvulae (MU) and GG, in 17 patients with SAHS and in 11 nonapneic snorers. MU and GG samples were obtained during uvulopalatopharyngoplasty. Anthropomorphic characteristics were similar in snorers and patients with SAHS, who differed only in the presence of sleep-related breathing abnormalities. MU glycolytic, glycogenolytic, and anaerobic enzyme activities were significantly greater in patients with SAHS than in snorers. These differences were not observed for GG. MU and GG enzyme activities differed only in snorers. The proportion of type I muscle fiber was greater in GG than in MU, but it was similar in patients with SAHS and snorers for each muscle. Type IIA and IIB muscle fibers were, respectively, in greater and smaller proportions in patients with SAHS than in snorers. We conclude that (1) the differences in metabolic characteristics between patients with SAHS and snorers are not observed in all UA muscles, and (2) similar histochemical differences are observed in GG and MU between these two groups, thus suggesting that these differences may be implicated in the pathophysiology of SAHS.

Adult↗

Influence of weight and sleep apnea status on immunologic and structural features of the uvula.

We investigated the influence of weight and obstructive sleep apnea status on inflammatory and histologic features of the uvula. Tissue samples resected during uvulopalatopharyngoplasty in 11 snorers without obstructive sleep apnea, 11 subjects with obstructive sleep apnea and of similar body mass index and age, and 8 additional obese subjects with obstructive sleep apnea were examined by immunohistochemistry and histologic staining techniques. The frequency and distribution of immune cells, the amount of collagen, and the integrity of the elastin fiber network were evaluated in proximal and distal uvular sections. T cell (CD4+, CD8+) and macrophage counts were higher in the more obese apneic subjects than in the other two groups. In all patients, T cell counts correlated with body mass index, but there was no relationship with the apnea-hypopnea index. A positive correlation was found between elastin fiber network disorganization score and apnea-hypopnea index. We conclude that (1) the amount of inflammatory markers is linked to obesity rather than to sleep-related breathing disorders, and (2) obstructive sleep apnea is associated with a structural alteration of the extracellular matrix of upper airway tissue.

Biopsy, Needle↗

Physiologic, metabolic, and muscle fiber type characteristics of musculus uvulae in sleep apnea hypopnea syndrome and in snorers.

Upper airway dilator muscles play an important role in the pathophysiology of sleep apnea hypopnea syndrome (SAHS). The mechanical and structural characteristics of these muscles remain unknown. The aim of this study was to compare the physiologic, metabolic, and fiber type characteristics of one upper airway dilator muscle (musculus uvulae, MU) in 11 SAHS and in seven nonapneic snorers. The different analyses were done on MU obtained during uvulo-palato-pharyngoplasty. Snorers and SAHS differed only in their apnea + hypopnea indices (11.5 +/- 5.9 and 34.2 +/- 14.6/h, respectively, mean +/- SD). Absolute twitch and tetanic tension production of MU was significantly greater in SAHS than in snorers while the fatigability index was similar in the two groups. Protein content and anaerobic enzyme activities of MU were significantly greater in SAHS than in snorers; no difference was observed for aerobic enzyme activities. The total muscle fiber cross-sectional area of MU was significantly higher in SAHS (2.2 +/- 0.9 mm2) than in snorers (1.1 +/- 0.7 mm2). The surface occupied by type IIA muscle fibers of MU was larger in SAHS (2.00 +/- 0.96) than in snorers (0.84 +/- 0.63 mm2). We conclude that the capacity for tension production and the anaerobic metabolic activity of MU are greater in SAHS than in snorers.

Adult↗

Isolated epithelial cysts of the uvula in children: a report of three cases.

Epidermoid cysts are unusual benign cysts that occur in the oral cavity less than 2% of the time. Epidermoid cysts isolated to the uvula are rare. A review of the literature has revealed only three confirmed cases of isolated uvular epidermoid cysts. We present a series of three such cases, two of which presented with significant symptomatology. The diagnosis and management of these unusual lesions will be discussed, as well as the differential diagnosis.

Cysts↗

Squamous cell carcinoma of the soft palate, uvula, and anterior faucial pillar.

This retrospective study concerns 188 patients with squamous cell carcinoma of the soft palate, uvula, and anterior faucial pillar treated for cure between 1970 and 1983. Men predominated in the group (1.9:1) and 55% of the patients were between 60 and 70 years old. Mean duration of followup was 56.7 months. TNM stage distribution was 29, 67, 37, and 49 patients for stages I, II, III, and IV respectively; six patients were unstaged because of previous excisional biopsy. Treatment to the primary site consisted of radiotherapy for 150 patients, surgery alone for 28 patients, and combined therapy for 10 patients. Primary control for T stages 1 through 4 was: 91% (31 of 34), 77% (71 of 92), 77% (30 of 39), and 35% (6 of 17), respectively. One hundred twenty-eight patients were N0 at presentation, as compared to 60 patients with regional nodal metastasis. Regional control was obtained in 87.5% of patients with N0 necks and in 76.7% of those with nodal involvement. In patients with primary control, these figures were 89% and 81%. Overall determinant survival was 80% at 2 years, but fell to 67% at 5 years. In addition to advanced tumor stage, the survival rate was reduced by regional lymph node metastasis. Tumor extension to the tongue base diminished survival. Survival was poorer among patients with midline tumors or tumors that extended across the palatine arch (37 patients) than for those with unilateral primary tumors (151 patients) (p less than 0.05). Despite similar T-stage distribution, the incidence of regional nodal metastasis was 49% in the former group, compared with 28% in the latter.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[A bifid uvula in a patient with schizophrenia as a sign of 22q11 deletion syndrome].

A bifid uvula and nasal speech were observed in a 25-year-old woman who was referred because of psychotic complaints. Fluorescence in situ hybridisation (FISH) research for the 22q11 deletion was carried out and the deletion was found. The 22q11-deletion syndrome (22q11DS) is characterised by somatic abnormalities including cardiovascular defects, velopharyngeal anomalies and typical facial characteristics. There is an increasing interest in the cognitive and psychiatric consequences of 22q11DS. There is a high prevalence of learning disabilities and the delayed development of language and speech. Mild mental retardation or borderline intellectual functioning is often reported. A broad range of psychiatric symptoms have been reported; a consistent finding is the development of a psychosis in a considerable proportion of 22q11DS patients from early adulthood onwards. It is important to consider the possibility of 22q11DS in psychiatric patients, as then early intervention strategies for later psychopathological abnormalities are possible, as well as the provision of genetic counselling.

Abnormalities, Multiple↗

[Palmoplantar epidermal atrophy with hypokeratosis, dys- and hypotrichosis, hypodontia, enamel and dentin hypoplasia, isolated cleft palate with cleft uvula, strabismus cryptorchism and other anomalies--an undescribed ecto-mesodermal dysplasia?].

A 15-year-old boy had suffered burning of both palms and all fingers during his 2nd year of life. Epitheliazation of the lesions did not occur. In his 6th year, erythema, desquamation and erosion-like alterations appeared spontaneously on the soles of both feet and on the plantar sides of the toes. These alterations were similar to those on the hands. Histologically the lesion on the right sole was covered with a very thin horny layer and thin epidermis. The cells of the epidermis had clear cytoplasm and probably derived from the ductal epithelium of the sweat glands. Under the atrophic epidermis, granulation tissue consisting of lymphocytes, plasma cells, histiocytes, and a few polynuclear cells and eosinophils was seen. The following symptoms were still present in the boy: dys- and hypotrichosis, oligodontia, enamel and dentin hypoplasia, cleft palate and uvula, concomitants strabismus (left eye), and cryptorchidism. Transplantation of the patient's skin from the right thigh to the lesion on the right sole was tried. The authors postulate a previously unreported form of ecto-mesodermal dysplasia in this case.

Abnormalities, Multiple↗

[Embryonal rhabdomyosarcoma of the uvula in a 5-year-old child].

A case of rhabdomyosarcoma embrionale uvulae in 5 years old girl was described. The child was treated at the beginning surgically, then chemotherapy was given according to schema IRS-III. After 2 years observation the child is in a good condition.

Antineoplastic Combined Chemotherapy Protocols↗