Upper aerodigestive tract liposarcoma: report on four cases and literature review.

OBJECTIVE: To report on the clinical behavior, histopathology, treatment, and prognosis of laryngeal, hypopharyngeal, and cervical esophageal liposarcomas. STUDY DESIGN: Retrospective reviews of pathology files and hospital records at a tertiary care hospital and a retrospective search of the English-language literature. METHODS: Cases of upper aerodigestive tract (UADT) liposarcoma with adequate histopathologic documentation and clinical information were included for review. RESULTS: Four cases of UADT liposarcomas were identified. The literature review revealed 26 cases of laryngeal liposarcomas, 7 cases of hypopharyngeal liposarcomas, and 6 cases of esophageal liposarcomas: the mean age at presentation was 55.8 years, the male:female ratio was 5:1, and 60% of the patients presented with dysphagia. Eighty-six percent of tumors had low-grade histologic findings. The recurrence rate after primary resection was 50%. Recurrence correlated with surgical procedure rather than with histologic subtype; 94.7% of recurrences happened after simple excision. Distant metastases occurred in three patients; two of them died of the disease. CONCLUSIONS: The literature supports that UADT liposarcomas are rare and usually of low-grade histologic type. The rate of metastatic disease and tumor-related mortality is low. However, high recurrence rates have been noted, particularly when less radical surgery is employed.     

Laryngeal angiosarcoma: a clinicopathologic study of five cases with a review of the literature

OBJECTIVE: Primary laryngeal angiosarcoma (LA) is rare without a reported series evaluating these tumors. STUDY DESIGN/METHODS: Five patients with LA were retrospectively retrieved from the Otorhinolaryngic Registry of the Armed Forces Institute of Pathology. RESULTS: Three men and 2 women, aged 29 to 71 years, presented with hoarseness (n = 4) and hemoptysis (n = 1). Two patients had previous neck radiation. The tumors involved the supraglottis (n = 4) with a mean size of 3.1 cm. Histologically, all tumors had anastomosing vascular channels lined by remarkably atypical endothelial cells protruding into the lumen, frequent atypical mitotic figures, and hemorrhage. All cases tested (n = 4) demonstrated immunoreactivity with antibodies to Factor VIII-RA and CD34. All patients had surgery followed by postoperative radiation (n = 3 patients). Three patients died with disease (mean, 17 mo), whereas one patient is alive with no evidence of disease at 18 years. CONCLUSIONS: LA is a rare tumor, frequently associated with previous radiation, usually involving the supraglottis with characteristic histomorphologic and immunophenotypic features. LA has a poor prognosis, making appropriate separation from other conditions important.     

Multiple synchronous and metachronous cancers of the upper aerodigestive tract: a nine-year study

This retrospective study was based on 9,089 patients and includes 855 cases (9.4%) of multiple primary cancers treated between 1975 and 1983. Three hundred fifty (42.1%) of these multiple cancers were considered synchronous, while 480 (57.9%) were classified as metachronous. Fifty percent of the metachronous cancers occurred within 31 months after the first primary; the mean time between the first primary and the second primary was 45 months. Approximately 22% of second primaries were found more than 5 years after the initial tumor. Five-year survival rates were higher in metachronous cancers (55%) than in synchronous cancers (18%). Survival rates varied according to the treatment. In 49.4% of synchronous tumors the treatment had to be modified because of the presence of two cancers. In these patients, 5-year survival rates were as low as 8%. In synchronous cancers that required no modification of the treatment, the prognosis was better and the survival rate was 28%. The preferential anatomical location of the second synchronous and metachronous cancers is discussed for first primaries situated in the oral cavity (103 cases), the oropharynx (120 cases), the hypopharynx (92 cases), and the larynx (87 cases). Second primaries in the esophagus were mostly synchronous cancers while, in the lung, second cancers were more frequently metachronous tumors.     

Nocardiosis of the upper aerodigestive tract

Nocardia asteroides is the major cause of nocardiosis in the respiratory tract. The primary site of infection is the lung, with the upper aerodigestive tract being a rare region for localized disease to occur. We describe a case of a nocardial vallecular cyst and discuss the diagnosis and management of this unusual infection.     

Amyloidosis of the upper aerodigestive tract

OBJECTIVES/HYPOTHESIS: To delineate the clinical and pathologic characteristics of upper aerodigestive tract amyloidosis with particular attention to laryngeal amyloidosis. STUDY DESIGN: Retrospective chart review of patients with amyloidosis of Thomas Jefferson University and its affiliated hospitals. MATERIAL AND METHODS: The charts of 16 patients with upper aerodigestive tract amyloidosis identified from the databases of the Thomas Jefferson University pathology department were reviewed and included in the study. RESULTS: Sixteen patients (9 male and 7 female, with an average age of 49.8 years) with upper aerodigestive tract amyloidosis were identified. The most common site of amyloid involvement was the larynx. Consequently, patients most commonly presented with hoarseness (14 of 16). All patients underwent surgical removal of the amyloid deposits. Fourteen patients had primary localized amyloidosis. Two experienced systemic involvement. Seven of the 16 patients developed recurrences requiring further treatment. CONCLUSIONS: Amyloidosis of the upper aerodigestive tract generally behaves as a benign, localized condition treatable by surgical resection. Regular follow-up with laryngoscopy is indicated for early diagnosis of recurrence, and multiple surgical procedures may be required to control symptoms.     

Rapid sequence treatment of advanced squamous cell carcinoma of the upper aerodigestive tract: A pilot study

A review of the literature suggested that prolonged treatment time may lessen the probability of cure for patients with advanced squamous cell carcinoma of the upper aerodigestive tract. To shorten treatment time, rapid sequence treatment (RST) was devised in which chemotherapy, surgery, and irrada-tion were administered in a total treatment time of 8 weeks. Twelve patients were treated and followed 3 years or longer. Medical complications were minor. Osteonecrosis occurred in each of the first five patients and was the only major complication of the protocol. Surgical techniques were modified, and no additional patient developed osteonecrosis. No patient developed local or regional recurrence. Two patients developed distant metastases and three other patients developed second primaries. Absolute survival was 50%. Rapid sequence treatment is an aggressive and potentially hazardous protocol that yielded encouraging results in this pilot study.     

Topical use of MMC in the upper aerodigestive tract: a review on the side effects

Not much is known about the side effects of mitomycin C (MMC), an anti-fibrogenetic agent, in the upper aerodigestive tract. However, its use in ophthalmology is widely known and without quantitatively important side effects. A literature review was performed for side effects of MMC in the upper aerodigestive tract. Forty-six articles, describing the use of MMC to prevent scarring, were retracted from PubMed. Thirty-two are human studies. MMC is used in different concentrations (0.1–10 mg/ml) with different application times (2–5 min) and frequencies (up to 4 times). Five hundred and thirty-eight patients were included in those publications, of whom 19 developed side effects (3.53%). No side effects developed in studies, where post-application irrigation with saline was reported. The longest mean follow-up period is 75.5 months. Direct relations between the reported side effects and MMC seem absent in most studies. Serious complications seem to occur when MMC is used in high concentrations. Unfortunately, sometimes crucial information is lacking. One patient was described who supposedly developed laryngeal carcinoma after repeated treatment of hyperkeratosis and anterior commissure webbing. Animal studies show that excessive fibrin production can lead to acute airway obstruction. In conclusion, topical application of MMC on a wound with consecutive irrigation with saline can be performed safely to prevent scar formation in circular structures of the upper aerodigestive tract. Long-term yearly control of the application site seems advisable.     

Flow cytometry: DNA analysis of squamous cell carcinoma of the upper aerodigestive tract

The flow cytometer is capable of analyzing a large number of cells or cell nuclei. The flow cytometer has been used to analyze the DNA content of tumor cells from squamous cell carcinomas of the upper aerodigestive tract using a modification of the Vindelov staining procedure. The main difficulty in analyzing solid tumors using flow cytometry technology is the disaggregation of the cells and the quantification of the diploid point (2N point). A set of internal standards was developed for establishing the 2N point, as well as a technique for disaggregation, which is described. A histogram characteristic of a more aggressive squamous cell carcinoma which tends to recur more quickly and more frequently than the other squamous cell carcinoma histogram types was identified. The flow cytometer proved to be a practical method of analysis allowing for quantitative measurement of DNA content from a large number of cells originating from a squamous cell carcinoma. The trend between recurrence and histogram type will require further study.     

Condylomatous lesions of the upper aerodigestive tract

Condyloma acuminatum is one of four types of common human verrucous lesions that are of viral etiology. Also known as "moist wart," condyloma acuminatum is most often seen on the mucosal surfaces of the anogenital area. However, occurrences in the mucosal lined areas of the head and neck region are quite rare. Since 1901, 30 cases of condylomatous lesions have been reported in the upper aerodigestive tract, occurring mainly in the various regions of the oral cavity. Eighteen of the cases were confirmed by histopathologic documentation, while the remainder were anecdotal. We have recently encountered six new cases of condyloma acuminatum, verified by histologic examination. One occurred on the tongue, another in the tonsillar fossa, one in the hypopharynx and three on the vocal cords. We present these cases and review the previously reported cases. In addition, we will discuss the differential diagnosis of these lesions, and their importance to the practicing otolaryngologist.     

Human papillomavirus DNA in squamous cell carcinoma of the upper aerodigestive tract

Nineteen samples of DNA from squamous cell carcinoma of the upper aerodigestive tract were analyzed by Southern blot hybridization for the presence of human papillomavirus (HPV) DNA. Two samples of DNA contained HPV 16 DNA or its homologous sequence. In one maxillary carcinoma, the sequences homologous to HPV 16 were detected. In one tonsillar carcinoma, HPV 16 sequence was also shown to be present. The patients positive for HPV DNA were female and had neither smoking nor drinking habits. These results indicate that HPV infections may play a role in the development of some types of squamous cell carcinomas of the upper aerodigestive tract.     

侵犯上消化呼吸道复发性甲状旁腺癌的再手术治疗

目的 探讨侵犯上消化呼吸道复发性甲状旁腺癌的术后复发原因及再手术疗效.方法 回顾总结6例伴上消化呼吸道侵犯的甲状旁腺癌患者首次手术方法、术后复发部位、复发间隔时间,本次手术方法及其血钙和甲状旁腺激素的水平的动态改变.6例患者之前的手术次数分别为4次者2例,2次、3次、5次和7次者各1例,上次手术后血钙及PTH无改善者3例.本组患者本术前的甲状旁腺激素值为860～2830 ng/L.行Ⅱ、Ⅲ、Ⅳ及Ⅵ区淋巴结清扫3例,Ⅱ～Ⅵ区、Ⅱ～Ⅶ区和Ⅲ～Ⅶ区清扫各1例,对术后缺损器官行一期修复.5例患者行预防性气管切开.结果 病理结果显示6例患者均有上消化呼吸道侵犯,其中单纯气管食管侵犯者4例,气管、食管、喉和下咽均受侵者1例,单纯喉内侵犯1例.2例伴Ⅳ及Ⅶ区淋巴结转移.本次手术后所有甲状旁腺激素均于术后2h内恢复正常,高钙血症均于术后24h内消失.4例患者随诊11 ～40个月PTH和血钙均在正常范围,另2例分别于术后8个月和11个月再次局部复发.术后并发食管瘘和淋巴管瘘各1例,均于保守治疗后愈合.6例患者出院时均拔管.1例患者并发胸骨愈合不良.结论 局部复发并侵犯上消化呼吸道的甲状旁腺癌可获得较好的手术效果,术前准确定位和术中甲状旁腺激素监测是保证手术成功的关键.     

Extraesophageal reflux and upper aerodigestive tract diseases

Reflux disease can cause multiple disorders not only of the esophagus but also of the upper aerodigestive tract. In the last decade, an association between reflux disease and multiple head and neck diseases was described, including those of the larynx and pharynx as well as those of the nose, the sinuses and the middle ear. The present article summarizes these different diseases and possible pathomechanisms are explained. In particular, there is a need to differentiate between the classical gastroesophageal reflux disease (GERD) and the extraesophageal manifestation of the reflux disease, called extraesophageal reflux (EER). Both diseases are characterized by different symptoms and can cause different disorders. To differentiate between GERD and EER, double-probe pH monitoring is mandatory. At last, some aspects of reflux therapy are explained.     

Liposarcomas of the larynx and hypopharynx: A clinicopathologic study of eight new cases and a review of the literature

Laryngeal and hypopharyngeal liposarcomas were studied in seven men and one woman. Patient age ranged from 25 to 81 years, with a median of 64 years. Symptoms included dysphagia, airway obstruction, and the sensation of a foreign body in the back of the throat. Histologically, seven of the tumors were of the biologically favorable types, either representing well-differentiated (lipoma-like) liposarcomas or myxoid liposarcomas. One tumor was a pleomorphic liposarcoma. Six of the eight patients had one or more episodes of recurrent tumor. Surgery is the treatment of choice and can include conservative (organ-sparing) procedures. However, to eradicate the tumor completely and thereby prevent recurrent disease, open surgical approaches (i.e., lateral pharyngotomy), rather than endoscopic techniques should be employed. The morbidity rate for laryngeal and hypopharyngeal liposarcomas is high because these tumors tend to recur over extended periods of time. In this study, however, there were no instances of metastatic disease, and no deaths were attributed to liposarcoma. Prospectively, laryngeal and hypopharyngeal well-differentiated (lipoma-like) liposarcoma is a difficult clinical and histopathologic diagnosis to establish. Often, this diagnosis is made only after one or more episodes of recurrent disease.     

Low incidence of point mutation at codon 12 of K-ras proto-oncogene in squamous cell carcinoma of the upper aerodigestive tract

Forty-two cases of squamous cell carcinoma arising in the upper aerodigestive tract were examined to determine the incidence and type of point mutation in codon 12 of the c-K-ras gene by using the polymerase chain reaction and oligonucleotide hybridization techniques on DNA extracted from paraffin blocks. DNA sequencing, in addition, was performed in 4 cases. No point mutation was detected in codon 12 of c-K-ras in the 42 squamous cell carcinomas we examined. According to the results of DNA sequencing of 4 cases, codon 13 also revealed no point mutation. Thus, point mutational activation of codon 12 of c-K-ras oncogene is an uncommon event in human upper aerodigestive tract squamous cell carcinoma.