Angiocentric lymphoma of the head and neck: patterns of systemic failure after radiation treatment.

PURPOSE: To investigate the patterns of systemic failure and the clinical outcome in patients with angiocentric lymphoma of the head and neck who were treated with radiation alone, and to discuss the optimal mode of treatment for these patients. PATIENTS AND METHODS: We reviewed the records of 92 patients with stage I or II angiocentric lymphoma who were treated at Yonsei Cancer Center between 1976 and 1994. All patients were treated with involved-field irradiation. Radiation doses ranged from 40 to 60 Gy (median dose, 50.4 Gy). Treatment response, patterns of treatment failure including systemic failure, and clinical outcome after radiation treatment were analyzed. RESULTS: The most frequently involved site was the nasal cavity, either alone or in conjunction with other sites. In 16 patients (17.4%), angiocentric lymphoma was accompanied by cervical lymphadenopathy. Disease was classified as stage I in 62 patients (67.4%) and stage II in 30 patients (32.6%). After completion of radiation treatment, 61 patients (66.3%) achieved a complete response and 16 (17.4%) a partial response. Half of the patients (50.0%) ultimately experienced local recurrence with or without other components of failure, whereas regional failure was relatively uncommon (10.9%). Systemic failure occurred in 25.0% of patients during follow-up. Six patients had histologic findings identical to those at the time of the original disease (group I), whereas four patients exhibited morphologic features of frank lymphomas (group II). The majority of patients with systemic relapse had the predilection sites for widespread extranodal involvement, such as the skin, brain, lung, gastrointestinal tract, or testes. In addition, seven patients died from various medical illnesses or immunologic disorders, including hemophagocytic syndrome and second primary cancers (group III). After a median follow-up of 56 months, the overall survival and disease-free survival rates for all patients were 40.1% and 37.8%, respectively. All patients except one with systemic failure died within 1 year. CONCLUSION: Treatment with radiation alone had suboptimal results, partly because of the occurrence of a variety of systemic failure with diverse clinicopathologic features. Given the frequent occurrence of systemic failure after radiation treatment, we believe that the multimodality treatment approach containing more effective chemotherapeutic agents should be incorporated in the treatment of angiocentric lymphoma confined to the head and neck.

Laryngeal pacemaker using a temperature sensor in the canine.

With the use of a temperature sensor that would detect temperature changes during the respiratory cycle in the pharynx, electrical stimulation of the posterior cricoarytenoid muscle was achieved in dogs whose recurrent laryngeal nerves were artificially paralyzed. Due to the temperature difference between inspiratory and expiratory air flow, a trigger pulse was generated at the beginning of inspiration. The stimulation pulse following the trigger pulse stimulated the electrodes inserted into the posterior cricoarytenoid muscles. In all five canines, obvious abduction of the vocal folds synchronized with inspiration was observed during electrical pacing.

Superior laryngeal nerve brain stem evoked response in the cat.

The generator sources of each wave of the laryngeal brain stem evoked response (LBR) have yet to be precisely demonstrated, although this has been studied in several animal species. This study was carried out to record the near-field brain stem activity as well as the far-field brain stem activity in the cat under the same experimental setup, and to search for the generator sources of the waves. Under general anesthesia, the LBR tracings were recorded adjacent to and within the brain stem following direct electrical stimulation of the internal branch of the superior laryngeal nerve. Reproducible positive and negative waves were detected by the far- and near-field techniques. Mean latencies, configurations, and reproducibility of each wave were demonstrated. From these results, we speculate on the generator sources of each wave.

Age dependence of laryngeal chemoreflex in puppies.

Previously collected data have indicated that the laryngeal chemoreflex (LCR) response is exaggerated during a critical period of postnatal development in several experimental animals. These animals had fewer anatomic and physiological similarities to humans than do puppies. This investigation of the LCR in 14 anesthetized puppies was undertaken to determine age-related differences in the response to stimulation of the supraglottic laryngeal mucosa by saline solution, distilled water, cow's milk, and acid at pH 1.0. The dogs were divided into 3 age groups: group 1 consisted of 4 dogs that were 2 weeks old, and in groups 2 and 3 there were 5 puppies each, of 4 and 6 weeks of age, respectively. The LCR response (laryngospasm, apnea, respiratory depression, and bradycardia) was found in the puppies only after stimulation of the laryngeal mucosa with acid at pH 1.0, and it was more easily achieved in the 4- and 6-week age groups than in the 2-week group. These findings suggest that the LCR is an age-dependent response that appears in dogs only after 2 weeks of age. The important implication of this finding is that postnatal neural maturation may influence the laryngeal reflex in humans to some extent.

Primary carcinoid tumor of the larynx.

A case of laryngeal carcinoid in a 54-year-old Korean female is reported and discussed. This tumor is extremely rare in the larynx and there have been just over twenty cases reported in the literature to date. The case showed an initial histologic finding of epithelial dysplasia and was finally confirmed to be a carcinoid tumor. Laryngeal carcinoids are often atypical histologically and may be misdiagnosed as undifferentiated carcinoma. An electron microscopic study revealed neurosecretory-type granules. Although the patient underwent a total laryngectomy and radical neck dissection, multiple hepatic metastasis was noted postoperatively.

Choroidal tuberculoma with membranous glomerulonephritis.

We report treatment of a 24-year-old man with membranous glomerulonephritis (MGN) who developed a solitary choroidal tuberculoma in association with miliary tuberculosis during steroid therapy. In June 1995, the patient had developed nephrotic syndrome. He had refused renal biopsy at that time. So we treated him with corticosteroids having assumed a diagnosis of minimal change nephrotic syndrome. After initial corticosteroids and diuretics therapy for 5 months, his generalized edema resolved but proteinuria (3 positive) continued, suggesting the presence of other forms of glomerulonephritis. Renal biopsy performed in January 1996. The patient was diagnosed as having MGN. The patient was closely observed over a period of 34 months and remained stable without steroid therapy. However at 34 months, generalized edema was again noted and steroid therapy at high dosage was initiated. After 5 months of steroid therapy, he developed miliary tuberculosis and a solitary choroidal mass. An antituberculosis chemotherapeutic regimen was started and after a further 5 months, all clinical symptoms and signs of the pulmonary lesion were resolved and a measurable shrinking of the choroidal mass was recorded.

Coxiella burnetii in polymorphic lymphocytes in tissue and blood of patients with polymorphic reticulosis.

Coxiella burnetii is a well-known causative agent of granulomatous inflammation and an inducer of morphological changes and transformation of human B lymphocytes in vitro. An association of the organism with polymorphic reticulosis (PMR), a malignant granulomatous inflammation characterized by polymorphic lymphocytes, was examined. The infection of C. burnetii was demonstrated in all cases tested, especially in polymorphic lymphocytes. Also the presence of morphologically transformed peripheral blood lymphocytes (PBLs) infected with C. burnetii was demonstrated. In cultures of blood lymphocytes, C. burnetii-infected polymorphic cells identified as B cells became immortalized in vitro. These findings implicate the role of C. burnetii in the process of PMR.

Role of definitive radiation therapy for larynx preservation in patients with advanced laryngeal cancer.

OBJECTIVE: Recently, neoadjuvant chemotherapy (CT) and radiation therapy (RT) have been advocated as a standard treatment for laryngeal preservation in patients with locally advanced laryngeal cancer. However, it is still being debated whether adding neoadjuvant CT to conventional RT makes an effective contribution to laryngeal preservation. The current study was designed to resolve this controversy. DESIGN: Retrospective clinical study. SETTING: The Severance Hospital, Yonsei Cancer Center, Yonsei University, Seoul, Korea. METHOD: Eighty patients (stages III, IV) with squamous cell carcinoma of the larynx were divided into two groups according to treatment modalities, which consisted of RT alone (N = 40, Group 1) and neoadjuvant CT plus RT (N = 40, Group 2). Comparative analysis was undertaken to investigate the differences in the organ preservation rate and treatment results between the two groups. RESULTS: There was no significant difference in the response rate and patterns of treatment failure between the two groups. The 5-year survival rate was similar between Group 1 (24%) and Group 2 (31%) (p = .1556). In addition, the larynx was almost equally preserved in Group 1 (62%) versus Group 2 (63%). CONCLUSIONS: Radiation therapy without neoadjuvant CT seems to be a valid alternative treatment for the purpose of laryngeal preservation in locally advanced laryngeal cancer.

Fanconi anemia screening by diepoxybutane and mitomicin C tests in Korean children with bone marrow failure syndromes.

Fanconi anemia (FA) is an autosomal recessive disorder of progressive bone marrow failure in patients with congenital malformations. FA is different from acquired aplastic anemia (AA) in terms of the natural course and treatment options. As the frequency of FA is unknown in Korea, we conducted screening tests using DNA clastogenic agents, diepoxybutane (DEB) and mitomicin C (MMC) in southwestern Korea. Forty-three children with AA or other bone marrow failure syndromes and siblings of known FA were evaluated. Six patients with AA (6/24=25.0%) and a 2-month-old patient with myelodysplastic syndrome were found to have increased chromosomal breakage to both DEB and MMC, confirming the diagnosis of FA. No overlap in chromosomal breakage to both agents was found between the FA group and non-FA group. The frequency of FA in this study, much higher than those of previous studies in Korea which did not incorporate the above tests, was similar to that of other countries. DEB and MMC tests were readily feasible and useful in screening FA in patients with AA as well as other bone marrow failure syndromes. A nation-wide screening and registry for FA should be initiated since FA requires different therapeutic and management options from idiopathic AA.

Adenoid cystic carcinoma of the maxillary antrum.

PURPOSE: To investigate characteristic clinical features and outcome for patients with adenoid cystic carcinoma (ACC) of the maxillary antrum. PATIENTS AND METHODS: Twenty-two patients with ACC of the maxillary antrum were initially treated with surgery alone (3 patients), radiation alone (9 patients), or a combination of surgery and radiation (10 patients). Salvage treatment for initial failure was individualized. Patterns of failure, survival, and prognostic factors were retrospectively analyzed. RESULTS: The most frequent site of failure was local recurrence at the primary site (72.7%). All patients treated with either surgery alone or radiation alone experienced one or more local recurrences, whereas patients who received planned combined surgery and radiation had a much lower local recurrence rate (40%). Neck node failure (4.6%) was an uncommon event, whereas distant metastases were clinically documented in seven patients (32%). Most of the treatment failures appeared within 5 years, but treatment failures after 5 years were not uncommon. The overall survival and disease-free survival rates at 10 years were 37.6% and 13.6%, respectively. Clinicopathological factors, such as location of primary tumor, tumor stage, and histological grade were of no value in predicting a favorable survival. The significant prognostic factors influencing 10-year survival were the pathological finding of perineural invasion and the initial mode of treatment. CONCLUSION: ACC of the maxillary antrum represented a unique natural history characterized by a more aggressive tumor behavior and an unfavorable prognosis. Combined surgery and radiotherapy is recommended for optimal local control and survival.