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Sarcoma of the larynx in a newborn.

A newborn male presented with respiratory distress and laryngeal stridor at the time of birth. Laryngoscopy revealed a circumscribed mass in the right vocal cord which was diagnosed as an undifferentiated malignant neoplasm on frozen section. Further light microscopic studies, special stains and electron microscopy disclosed features consistent with a special subtype of sarcoma adopted by the Intergroup Rhabdomyosarcoma Committee. Several authors have also demonstrated similarities between this type of tumor and soft tissue Ewing sarcomas. Nonepithelial malignancies of the larynx are rare in children and are only anecdotally reported in newborns. Histopathologically, the tumors predominantly include rhabdomyosarcomas among other rarer less well documented sarcomas and lymphomas. Although modern chemotherapy and radiotherapy have improved the otherwise grim prognosis of soft tissue Ewing sarcoma, this young patient was only treated with total laryngectomy at ten days of age and is alive and well two years later.

Cytoplasm↗

Metastatic cancer to the larynx. Diagnosis and management.

A case of metastatic tumor in the larynx following successful treatment of a breast carcinoma and a colon carcinoma is presented. The lesion was visible by indirect laryngoscopy, and computed tomography of the larynx assisted in delineating the extent of the disease. The separation of primary adenocarcinoma of the larynx from metastases is discussed, as is the identification of the origin of a metastasis where two separate primaries have existed. Other reports of cancer metastatsizing to the larynx are reviewed. Tumor spread to the larynx may be asymptomatic or may result in hoarseness, stridor and/or airway obstruction.

Adenocarcinoma↗

Inflammatory myofibroblastic tumor of the larynx. A clinicopathologic study of eight cases simulating a malignant spindle cell neoplasm.

BACKGROUND: Inflammatory myofibroblastic tumors of the larynx are uncommon lesions that easily may be misinterpreted as malignant epithelial or mesenchymal spindle cell neoplasms. METHODS: Eight cases of laryngeal inflammatory myofibroblastic tumors were identified from the files of the Otolaryngic Tumor Registry--Armed Forces Institute of Pathology. Clinical records and follow-up were available in all cases. The light microscopic features (hematoxylin and eosin and special histochemical stains) were evaluated in all cases; immunohistochemical analysis was performed in the seven cases with available paraffin blocks; in four cases ultrastructural analysis was done. RESULTS: The patients included five males and three females ranging in age from 19-69 years (median, 59 years). Presenting symptoms included hoarseness, dysphonia, or rapidly progressive stridor with the duration of symptoms ranging from 10 days to 4 months. The most common site of involvement was the true vocal cord. The lesions appeared as polypoid or pedunculated masses. Histologically, the cellularity of the lesions varied, consisting of spindle-shaped to stellate cells with no consistently discernible growth pattern, in a fibromyxoid stroma that included a mixed inflammatory cell infiltrate. Features suggesting a malignant cellular infiltrate were not present. The spindle-shaped cells had consistent immunoreactivity with vimentin, muscle specific actin, and smooth muscle actin. Ultrastructurally, intracytoplasmic microfilaments were identified. In seven of the patients, conservative but complete excision of the lesion was curative; these patients have been free of disease over periods ranging from 12 to 36 months. In one patient, the lesion recurred twice over a 2-year period and ultimately required a total laryngectomy. This patient died of unrelated causes. CONCLUSIONS: Inflammatory myofibroblastic tumors of the larynx are unusual benign proliferative lesions. Conservative surgical management is advocated and is curative. Recurrence is rare, but metastases disease or death attributable to these lesions is not.

Actin Cytoskeleton↗

Patterns of care for patients with primary differentiated carcinoma of the thyroid gland treated in Germany during 1996. U.S. and German Thyroid Cancer Group.

BACKGROUND: To determine current patterns of care and disease characteristics for patients with thyroid carcinoma, a Patient Care Evaluation Study was initiated in 1996 in the U.S. and Germany. This project addresses ongoing concerns with respect to the diagnostic evaluation and treatment of patients diagnosed with thyroid carcinoma and raises questions concerning how physicians are interpreting current standards and acting on the basis of these recommendations. METHODS: Patients with primary thyroid carcinoma were entered into a prospective multicenter observational study with free choice of treatment (no control group) between January 1, 1996 and December 31, 1996 in Germany. This resulted in a total of 2537 cases under observation and analysis; 1685 patients had papillary carcinoma (66.4%), 691 had follicular carcinoma (27.2%), 70 had medullary carcinoma (2.8%), and 91 had anaplastic carcinoma (3.6%). The 2376 patients with carcinoma of either papillary or follicular histology were included in the current analysis. RESULTS: The major symptoms reported for patients with papillary and follicular thyroid carcinoma was neck mass (reported in 76% and 79%, respectively) followed by dysphagia (reported in 25% and 27%, respectively), stridor (reported in 9% and 14%, respectively), and neck pain (reported in 7% and 8%, respectively). Greater than 50% of the patients with papillary thyroid carcinoma were reported to have American Joint Committee on Cancer/International Union Against Cancer Stage I disease. Between 37-39% of the follicular carcinoma patients had Stage I and Stage II disease. Only slight differences in the diagnostic approach to patients with papillary or follicular carcinoma were noted. The majority of patients underwent an ultrasound of the thyroid region (78.1%), which was suggestive of carcinoma in only 39% of the cases. A thyroid scan was performed on 76.6% of patients, and the results were suggestive of carcinoma in 44.8% of the individuals. In contrast, fine-needle aspiration biopsy of the thyroid is highly recommended in the current Clinical Practice Guidelines (CPG) but results were obtained in only 27.4% of the patients. Total thyroidectomy without lymph node dissection was the most commonly used surgical procedure in the treatment of patients with papillary and follicular thyroid carcinoma. Only approximately 2% of patients at low risk in the group with Stage I disease were treated with a lobectomy. In 80% of the patients with Stage I papillary thyroid carcinoma and approximately 90% of those patients diagnosed with Stage II, III, and IV disease treating physicians chose to utilize radioiodine as adjuvant treatment after disease-directed surgery. External beam radiation was added to the treatment regimen for many patients diagnosed with Stage III and IV disease (30% in patients with papillary thyroid carcinoma and 33% in patients with follicular thyroid carcinoma). CONCLUSIONS: To the authors' knowledge no single effective diagnostic test for thyroid carcinoma currently is available and in the majority of cases a combination of ultrasound, thyroid scan, or fine-needle aspiration biopsy together with the clinical findings (e.g., thyroid mass) led to a diagnosis of carcinoma. The authors suspect that the high prevalence of concomitant pathologic findings such as goiter, even in the healthy population in Germany, reduces the accuracy of all diagnostic test methods and may account for the frequent use of imaging techniques. The majority of patients underwent a total or near-total thyroidectomy. Total thyroidectomy with radical lymph node dissection was used very frequently in those patients with papillary thyroid carcinoma (22%). German physicians tend to surgically treat early stage thyroid carcinoma somewhat more radically than recommended in the CPG. With respect to other treatment options employed as part of the first course of treatment, radioiodine appears to play the most important role. [See commentary o

Adenocarcinoma, Follicular↗

Recurrent nasopharyngeal carcinoma presenting as diffuse dermal lymphatic infiltration in the neck: three case reports.

BACKGROUND: Any malignancy has the propensity to metastasize to skin. The frequency of skin metastases vary in different tumors and occur in about 0.7% to 10% of all patients diagnosed with cancer. It is rare in nasopharyngeal carcinoma. METHOD: Three cases of relapsed nasopharyngeal carcinoma with diffuse dermal involvement were described. Their clinical presentation, results of investigations, and response to treatment were reviewed. Literature review of similar forms of presentation was done by means of a MEDLINE search. RESULTS: At the time of dermal relapse, all three patients had a uniform clinical picture of facial, periorbital, and lip swelling associated with stridor and dysphagia. Histologic findings showed dermal infiltrates of malignant cells, and CT scan showed diffuse infiltration of the subcutaneous tissue. Despite chemotherapy, the clinical course was relentless. CONCLUSION: This report describes a presentation of disease that is underdiagnosed and heightens awareness of oncologists to this form of recurrence in nasopharyngeal carcinoma.

Adult↗

Humidified air inhalation for treating croup.

BACKGROUND: Croup (laryngotracheobronchitis) is a common cause of upper airway obstruction in children with a peak incidence of 60 per 1000 child years in those aged between one and two years. It is characterised by hoarseness, a barking cough, and inspiratory stridor. These symptoms are thought to occur as a result of oedema of the larynx and trachea, which have been triggered by a recent viral infection. Para influenza virus type 1 is the agent most commonly identified in cases of croup. Severe cases are admitted to hospital and steroid treatment is established to reduce disease severity. Treatment with humidified air was previously widely used and is still commonly recommended as home treatment. OBJECTIVES: To assess the efficacy of humidified air in the treatment of croup. SEARCH STRATEGY: We searched the Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library Issue 4, 2005), MEDLINE (1966 to January 2006) and EMBASE (1990 to January 2006). SELECTION CRITERIA: Randomised controlled trials (RCTs) involving children suffering from croup treated with humidified air. DATA COLLECTION AND ANALYSIS: Two authors independently identified potentially relevant abstracts identified from the search and then assessed the full papers for inclusion and methodological quality. Outcome measures included mortality, ventilation, admission to hospital, re-contact with medical services, number of days off school and relief of symptoms; these were separately analysed for the week following treatment. Data extraction was performed by the two authors then entered by one and checked by the second author. Missing data were obtained from trails authors where possible. Data were analysed using Review Manager version 4.2. Sensitivity and sub-group analysis were not possible due to the paucity of trials. MAIN RESULTS: Three studies in emergency settings provided data on 135 patients with moderate croup for the main outcome (croup score). The combined results from 20 to 60 minutes in the three studies marginally favoured the treatment group with a weighted standardised mean difference of -0.14 (95% confidence interval (CI) -0.75 to 0.47). No other outcomes were significantly different between the groups. AUTHORS' CONCLUSIONS: The croup score of children managed in an emergency setting with mild to moderate croup probably does not improve greatly with inhalation of humidified air. Further research is needed in primary care settings, using a wider range of more sensitive outcome measures.

Air↗

Phenotypic overlap of the BBB and G syndromes.

Three males with similar malformations including hypertelorism, telecanthus, cleft lip and palate, and hypospadias, have been evaluated. One also had a laryngotracheoesophageal cleft and therefore was considered to have the G syndrome. The other two had no stridor, aspiration, or difficulty swallowing, and were considered to have the BBB syndrome. Both disorders are associated with multiple malformations and can be most readily distinguished by the presence of laryngoesophageal abnormalities in the G syndrome and differences in facial features evident later in childhood. The BBB syndrome appears to be inherited as an X-linked disorder with the affected female showing only telecanthus and hypertelorism. The G syndrome exhibits autosomal dominant inheritance with males more severely affected, although a few few females have had serious malformations in addition to telecanthus and hypertelorism. In the family with the G syndrome evaluated for this report, the mother of the affected infant had telecanthus, hypertelorism, and anosmia, the latter a feature not previously noted in this disorder.

Abnormalities, Multiple↗

Laryngeal cleft: report of eight patients and a review of the literature.

Eight cases of laryngeal cleft are presented and the literature on this topic is reviewed. Patients most frequently present with stridor, respiratory distress, or a history of choking while feeding. A number of cases are found at autopsy or surgery. Laryngeal cleft frequently is found associated nonspecifically with tracheoesophageal fistula, cleft lip and cleft palate, and congenital heart defects. Laryngeal cleft may be a component manifestation of several syndromes, eg, the G syndrome, and the Pallister-Hall syndrome of congenital hypothalamic hamartoblastoma, hypopituitarism, imperforate anus, and postaxial polydactyly. Surgical treatment is successful in more than 50% of the reported cases, depending on the extent of the cleft. Cleft larynx is most likely a developmental field defect, occurring coincidentally with separation of larynx and esophagus and closure of the larynx.

Abnormalities, Multiple↗

Sudden death in childhood in a case of the G syndrome.

We report on the unexpected death at almost 8 years of a boy with the G syndrome who had successfully survived many prior life-threatening complications of the condition. The patient had a characteristic facial appearance, hypertelorism, second-degree hypospadias, stridor and cough on feeding with aspiration of barium, and uncoordinated esophageal swallowing mechanism, but no obvious laryngotracheobranchial defect on endoscopy (or coroner's autopsy). He had a prior cardiac arrest and had outgrown need for a tracheostomy. Aspiration is presumed to be the cause of death.

Abnormalities, Multiple↗

Tracheomalacia in Hallermann-Streiff syndrome.

We report on a white boy with Hallermann-Streiff syndrome (HSS) who also had tracheomalacia. Chronic respiratory insufficiency led to biventricular failure and death at age 6 months. There have been no previously reported cases of Hallermann-Streiff syndrome with documented tracheomalacia. However, there may be cases in which tracheomalacia may have been present, but not diagnosed. The literature contains 6 HSS cases with severe respiratory symptoms. Tracheomalacia should be considered in a patient with HSS who presents with an unusual cry, stridor, choking, or apnea. With the availability of surgery and supportive treatment, early diagnosis of tracheomalacia in these patients may prevent death and secondary neurologic insult from acute hypoxia.

Cartilage Diseases↗

Autosomal dominant congenital laryngomalacia.

A family is presented in which congenital stridor due to laryngomalacia was evident in 9 individuals through 3 generations. This report confirms the autosomal dominant transmission of at least one type of laryngomalacia.

Adult↗

Phase II trial of ifosfamide and mesna in previously treated patients with non-Hodgkin's lymphoma: Cancer and Leukemia Group B study 8552.

Ifosfamide (1.25 g/m2 intravenously/day x 5) with mesna (20 per cent of the ifosfamide dose x six doses on each day of ifosfamide therapy) was administered to 46 previously treated patients with non-Hodgkin's lymphoma of which 31 were eligible and evaluable. A 29 per cent response rate (9/31) was observed (two CR and seven PR) with a median duration of response of 2.5 months. Myelosuppression was dose-limiting. Hemorrhagic cystitis was observed in three patients (10 per cent). Nausea and vomiting was generally mild or moderate. One patient developed transient neurotoxic symptoms with confusion and disorientation. An additional patient developed an anaphylactic-type reaction with shortness of breath and respiratory stridor during the fourth course of therapy. Ifosfamide, as a single agent, produces remissions of limited duration in non-Hodgkin's lymphoma in patients in second or third relapse.

Adolescent↗

Laryngotracheal invasion by well-differentiated thyroid cancer: diagnosis and management.

Ten cases of laryngotracheal invasion by well-differentiated thyroid cancer are described. All the patients were over 40 years of age, with a male preponderance of 4:1. A mass in the neck, stridor, and hemoptysis was the common symptom. Bronchoscopy done in six of ten patients revealed an intraluminal tumour in all. Radiographs of the neck showed invasion of the trachea in six patients. Surgery consisted of tracheostomy alone (three of ten), total thyroidectomy with laryngectomy (one of ten), circumferential resection of trachea (one of ten), and total thyroidectomy with tracheotomy and "shaving" of the intraluminal disease in five of ten patients. Adjuvant external radiation, radioiodine, or laser therapy was given when indicated. Of the ten patients, three died and seven survived. Five of seven were alive without disease for 2.5 years or more, and one had skull metastasis 2 years later but no local disease.

Adenocarcinoma↗

REM sleep behavior disorder and vocal cord paralysis in Machado-Joseph disease.

We evaluated the occurrence of REM sleep behaviour disorder (RBD) and vocal cord abductor paralysis (VCAP) in a group of 9 Machado-Joseph disease (MJD) patients. RBD was diagnosed by clinical history plus audiovisual polysomnography in 4 men and 1 woman (55%). While dreaming, 4 fell out of the bed and the other injured his arms. Laryngoscopy detected bilateral VCAP in 1 patient with stridor who required emergency tracheotomy, and partial vocal cord abductor restriction in 2. RBD and VCAP are two potentially injurious conditions that should be considered part of the clinical spectrum of MJD.

Adult↗

Brainstem respiratory control: substrates of respiratory failure of multiple system atrophy.

Multiple system atrophy may manifest with severe respiratory disorders, including sleep apnea and laryngeal stridor, which reflect a failure of automatic control of respiration. This function depends on a pontomedullary network of interconnected neurons located in the parabrachial/Kölliker Fuse nucleus in the pons, nucleus of the solitary tract, and ventrolateral medulla. Neurons in the preBötzinger complex expressing neurokinin-1 receptors are critically involved in respiratory rhythmogenesis, whereas serotonergic neurons in the medullary raphe and glutamatergic neurons located close to the ventral medullary surface are involved in central chemosensitivity to hypercapnia, hypoxia, or both. Pathological studies using selective neurochemical markers indicate that these neuronal groups are affected in multiple system atrophy. This finding may provide potential anatomical substrates for the respiratory manifestations of the disease.

Apnea↗

Life-threatening cranial dystonia following trihexyphenidyl withdrawal.

A 64-year-old woman with blepharospasm, sustained clenching of the jaw, antecollis, and a strained, high-pitched phonation continued chronic trihexyphenidyl therapy despite the lack of any obvious benefit. Abrupt, accidental withdrawal of trihexyphenidyl triggered severe exacerbation of the cranial dystonia associated with inspiratory stridor and acute respiratory difficulties, prompting emergency admission. On indirect laryngoscopy, hyperadduction of the vocal folds was not the cause of the upper airway obstruction. A more likely cause of the inspiratory obstruction appeared to be forward bending of the neck combined with mouth-clenching spasms. Reinstitution of intravenous anticholinergic medication provided relatively prompt relief. We caution against abrupt interruption of anticholinergics in patients with severe segmental cranial dystonia, even in those cases in which no benefit is apparent to observers.

Aged↗