Esthesioneuroblastoma: the impact of treatment modality.

BACKGROUND: We evaluated the impact of treatment modality on esthesioneuroblastoma. METHODS: Between 1976 and 1996, 25 patients with esthesioneuroblastoma were treated at Mallinckrodt Institute of Radiology. There were 11 male and 14 female patients; their ages ranged from 16 to 73 years (median, 57 years). The tumors were Kadish stage A in 3, Stage B in 13, C in 8, and modified D in 1 (cervical nodal metastasis). Seventeen patients were treated with surgery and radiation therapy, six were treated with irradiation alone, and two were treated with surgery only. Eight patients received neoadjuvant chemotherapy. Median follow-up was 8 years (range, 2-24 years). RESULTS: The 5-year actuarial overall survival, disease-free survival, and local tumor control rates were 66.3%, 56.3%, and 73.0%, respectively. Kadish stage was not a significant prognosticator for local control or disease-free survival. Five-year local control rates were 87.4% for the combination of surgery and radiation therapy and 51.2% for irradiation alone. Two patients with Kadish stage A and B disease underwent surgical resection alone; both failed locally. In contrast, 33.3% of patients (three of nine) with Kadish stage A or B disease who received adjuvant radiation therapy had a local recurrence develop. With adjuvant radiation therapy, the surgical margin status did not influence local tumor control. Among the eight patients who received neoadjuvant chemotherapy, six patients showed no response, one had partial response, and one showed a complete response. CONCLUSIONS: Surgical resection plus adjuvant radiation therapy yielded the best treatment outcome. More effective chemotherapy agents with a reproducible effectiveness are needed for patients with locally advanced esthesioneuroblastoma.

Cancer of retromolar trigone: long-term radiation therapy outcome.

BACKGROUND: Cancer of the retromolar trigone is an uncommon head and neck cancer. In this retrospective study, we identified the prognostic factors and evaluated the therapeutic outcomes of patients treated with preoperative radiation therapy (RT), postoperative RT, and RT alone. METHODS: Between 1971 and 1994, 65 patients with histologically proven epidermoid carcinoma of the retromolar trigone were treated at the Mallinckrodt Institute of Radiology; 10 patients received preoperative RT (30-55.2 Gy), 39 received postoperative RT (46-66.6 Gy), and 15 were treated with RT alone (63-74 Gy). Surgery included 44 composite resections and 7 wide excisions. The minimum follow-up was 5 years. RESULTS: The 5-year disease-free survival rates were 90% with preoperative RT, 63% with postoperative RT, and 31% with RT alone. The 5-year disease-free survival rates were 76% for patients with T1 disease, 50% for T2, 72% for T3, and 54% for T4. The 5-year disease-free survival rates were 69% for patients with NO disease, 56% for N1, and 26% for N2. The locoregional recurrence rates were 10% (1 of 10) for preoperative RT, 23% (9 of 39) for postoperative RT, and 44% (7 of 16) for RT alone. On multivariate analysis, the significant factors for disease-free survival were treatment modality (p =.002) and N stage (p =.012); for locoregional control it was treatment modality (p =.046); and for distant metastasis it was N stage (p =.002). The incidence of bone necrosis, soft tissue necrosis, and severe trismus was 12% with postoperative RT, 11% with RT alone, and none with preoperative RT. CONCLUSIONS: Combination surgery with postoperative or preoperative RT offers better locoregional control and disease-free survival than RT alone for epidermoid carcinoma of the retromolar trigone. Lymph node status significantly influences the disease-free survival and distant metastasis rates.

Distant metastases from laryngeal and hypopharyngeal cancer.

A retrospective tumor registry analysis of patients with squamous cell carcinoma (SCC) of the larynx and hypopharynx who were treated with curative intent in the Department of Otolaryngology--Head and Neck Surgery at Washington University School of Medicine and Barnes Hospital between January 1971 and December 1991. In 2,550 patients, the mean age, sex and tumor differentiation did not affect the incidence of distant metastases. The overall incidence of distant metastases was 8.5% (217/2,550 patients) with the following distribution: glottis 4.4%, supraglottis 3.6%, subglottis 14%, aryepiglottic fold 16%, pyriform sinus 17% and posterior hypopharynx 17.6%. The overall 5-year disease-specific survival for distant metastases was 6.4%. Distant metastases were related to advanced local disease (T3 + T4), lymph node metastases at presentation (N+), tumor location (hypopharynx) and locoregional tumor recurrence (p < or = 0.028). A meta-analysis of variables which predispose to a higher incidence of distant metastases indicate that tumor location (hypopharynx > larynx), advanced primary disease (T3 + T4), regional disease (N+), locoregional recurrences, and advanced regional metastases (N2 + N3) are statistically significant.

Analysis of treatment results for floor-of-mouth cancer.

OBJECTIVE: This study reports the results of treating floor-of-mouth cancer with five different treatment modalities with long-term follow-up. STUDY DESIGN: Retrospective study of 280 patients with floor-of-mouth cancer treated in the Department of Otolaryngology-Head and Neck Surgery at Washington University Medical School (St. Louis, MO) from 1960 to 1994. METHODS: Patients with biopsy-proven squamous cell carcinoma of the floor of mouth who were previously untreated were treated with curative intent by one of five modalities and were all eligible for 5-year follow-up. The treatment modalities included local resection alone, composite resection alone (with neck dissection), radiation therapy alone, local resection with radiation therapy, and composite resection with radiation therapy. Multiple diagnostic, treatment, and follow-up parameters were studied using standard statistical analysis to determine statistical significance. RESULTS: The overall 5-year disease-specific survival (DSS) was 56% with death due to tumor in 44% of patients. The 5-year cumulative disease-specific survival (CDSS) was 0.61 (Kaplan-Meier probability) with a mean of 8.3 years and a median of 9.7 years. The DSS by treatment modality included local resection (76%), composite resection (63%), radiation therapy (43%), local resection with radiation therapy (61%), and composite resection with radiation therapy (55%). Overall, there was no significant difference in DSS by treatment modality. Recurrence at the primary site (41%) was the most common site of treatment failure. Nineteen percent of patients had recurrence in the neck. Eighty-eight percent of initial recurrences occurred within 60 months after the onset of treatment. Metastasis to a distant site occurred in 30% of patients. Twenty percent of these patients had second primary cancers, and 53% of these patients died of their second primary cancers. CONCLUSIONS: Significantly improved 5-year DSS was seen in the patients with clear margins, early clinical tumor stage, and negative nodes. Significantly decreased 5-year survival was seen in the patients with involved margins, advanced clinical tumor stage, positive nodes, and tumor recurrence. Patients with no clinically positive nodes (cNO) can be observed safely for regional nodal disease and subsequent positive nodes can be treated as they occur with no adverse affect on survival. Because of high recurrence rates at the primary site and neck, and an increased rate of both distant metastasis and the development of second primary cancers, patients should be monitored closely for a minimum of at least 5 years.

Carcinoma of the tonsillar fossa: prognostic factors and long-term therapy outcome.

PURPOSE: To identify prognostic parameters and evaluate the therapeutic outcomes for patients with carcinoma of the tonsillar fossa treated with three treatment modalities. METHODS AND MATERIALS: The results of therapy are reported in 384 patients with histologically proven epidermoid carcinoma of the tonsillar fossa; 154 were treated with irradiation alone (55-70 Gy), 144 with preoperative radiation therapy (20-40 Gy), and 86 with postoperative irradiation (50-60 Gy). The operation in all but four patients in the last two groups consisted of an en bloc radical tonsillectomy with ipsilateral lymph node dissection. RESULTS: Treatment modality and total irradiation doses had no impact on survival. Actuarial 10-year disease-free survival rates were 65% for patients with T1 tumors, 60% for T2, 60% for T3, and 30% for T4 disease. Patients with no cervical lymphadenopathy or with a small metastatic lymph node (N1) had better disease-free survival (60% and 70%, respectively) at 5 years than those with large or fixed lymph nodes (30%). Primary tumor recurrence (local, marginal) rates in the T1, T2, and T3 groups were 20-25% in patients treated with irradiation and surgery and 31% for those treated with irradiation alone (difference not statistically significant). In patients with T4 disease treated with surgery and postoperative irradiation, the local failure rate was 32% compared with 86% with low-dose preoperative irradiation and 47% with irradiation alone (p = 0.03). The overall recurrence rates in the neck were 10% for N0 patients, 25% for N1 and N2, and 35-40% for patients with N3 cervical lymph nodes, without significant differences among the various treatment groups. The incidence of contralateral neck recurrences was 8% with the various treatment modalities. On multivariate analysis the only significant factors for local tumor control and disease-free survival were T and N stage (p = 0.04-0.001). Fatal complications were noted in 7 of 144 (5%) patients treated with preoperative irradiation and surgery, 2 of 86 (2%) of those receiving postoperative irradiation, and 2 of 154 (1.3%) patients treated with radiation therapy alone. Other moderate or severe nonfatal sequelae were noted in 30% of the patients treated with preoperative irradiation and surgery, in 53% treated with postoperative irradiation, and in 19% receiving radiation therapy alone. CONCLUSION: Primary tumor and neck node stage are the only significant prognostic factors influencing locoregional tumor control and disease-free survival. Treatment modality had no significant impact on outcome. Radiation therapy remains the treatment of choice for patients with stage T1-T2 carcinoma of the tonsillar fossa. In patients with T3-T4 tumors and good general condition, combination surgery and postoperative irradiation offers better tumor control than single-modality and preoperative irradiation procedures, but with greater morbidity.

Giant intradiploic epidermoid cyst of greater sphenoid wing causing unilateral proptosis and optic nerve compression.

Epidermoids are cystic tumors that arise from aberrant epidermal cells. Intradiploic epidermoids are relatively rare tumors that occur in all bones of the calvarium, temporal and sphenoid bones, paranasal sinuses, and maxilla. We report the case of an intradiploic epidermoid of the sphenoid wing causing severe proptosis and visual loss. Theories of embryogenesis and pathophysiology are discussed.

Radiation therapy in the treatment of chemodectomas.

Twenty-six patients with chemodectomas were treated with radiation therapy from 1961 to 1983 and followed for at least 4 years. In 16 glomus tympanicum lesions treated primarily with radiation, long-term control was achieved in all (follow-up 4-24 years, mean 10.5). Fifteen of 16 had no clinical evidence of disease at time of death or last follow-up, and one patient had recurrent but stable disease at 10 years and died of an unrelated illness 19 years after treatment. Eight of the glomus tympanicum lesions were referred for radiation therapy with persistent disease after multiple surgical procedures. Two had extensive skull and intracranial involvement at the time of radiation. Six patients with glomus jugulare lesions were treated with radiation. Four achieved long-term control, and two died of their disease. In addition, two glomus vagal and one carotid body tumor were treated with palliative intent. One glomus tympanicum lesion was treated with preoperative radiation and resection. Chemodectoma was the cause of death in 4 of 25 patients--3 from intracranial extension and 1 from lung and mediastinal metastases. All four patients were referred for radiation with metastases or extensive bone involvement at the skull base. Radiation appears to be effective in achieving long-term clinical control of chemodectomas. Adequately treated volume should be determined with arteriography and/or contrast-enhanced computer tomography, allowing for geometric margins. Doses in the range of 4,500 to 5,000 cGy delivered in about 5 weeks are recommended.

Reirradiation of recurrent head and neck cancers.

Ninety-nine patients with recurrent cancers of the head and neck region were treated with surgery, radiation therapy, or combination therapy. The follow-up period ranged from 18 months to 18 years. An initial overall complete response rate of 67% and a partial response rate of 7% (overall response rate-74%) were achieved. The eventual tumor control rate was 15%. Although equal initial response rates were achieved in recurrences at the primary site and the cervical nodes, the eventual local control was better for the former (21% vs. 10%). Patients receiving less than 5,000 rad radiotherapy had a 44% complete response and an 11% eventual tumor control. Patients receiving over 5,000 rad had an 80% complete response and a 25% eventual tumor control.

Malignant tumors of the middle ear and external auditory canal: a 20-year review.

Twenty-four patients with malignant tumors of the external auditory canal and middle ear, originally seen between 1960 and 1980, were reviewed retrospectively. Seventeen patients had epidermoid carcinoma, one had adenocarcinoma, three had rhabdomyosarcoma, and one had osteosarcoma. At presentation, four had disease confined to the external auditory canal, three had superficial invasion of the bony canal, four had deeply invasive disease, and thirteen had disease that extended beyond the temporal bone. Treatment consisted of radiation, surgery, and combination therapy. Four patients with osteosarcoma or rhabdomyosarcoma received adjuvant chemotherapy. Five years after therapy, one of four patients with external auditory canal tumor died of disease, and two died of intercurrent disorders. One of three patients with superficial temporal bone invasion and two of four patients with deeply invasive tumor died of their disease; another died of intercurrent disorder. Twelve of 13 patients with tumor beyond the temporal bone died.

Adenoid cystic salivary gland carcinoma. A histopathologic review of treatment failure patterns.

Seventy-one cases of adenoid cystic salivary gland carcinoma were reviewed according to treatment modality and clinical course. Thirty-six patients (51%) were treated by combined surgery and radiation therapy. The tumors were classified by their histologic patterns into tubular, cribriform, and solid forms. Distant metastases, in 52%, were the most frequent and ominous sources of failure. In 35% of cases, distant metastases developed despite local control at the primary site. In this group, the disease had a more fulminant course with shorter survival. Histopathologically, the cribriform subtype was associated with multiple local recurrences, greater local aggressiveness, and a poorer salvage rate as compared with the tubular subtype. Late onset of local recurrences and distant metastases was especially associated with the cribriform subtype. Overall prognosis in terms of distant metastases and survival was worst for the solid subtype. Control of local disease is best achieved with combined surgery and radiation therapy. The high incidence of distant metastases may not be affected by this regimen. The ultimate outcome of therapy is poorly predicted. Survival appears to be based on the pattern in which distant metastases develop. Overly aggressive and mutilating surgical approaches for these tumors are not recommended in many instances. The need for the development of new, more effective forms of therapy is emphasized.

Fascial pulley: cross-commissure lip reanimation for inferior division facial nerve paralysis.

The risks of causing further harm to the facial nerve are greater in surgery for partial facial paralysis. We present a new active and passive reconstruction technique for paralysis of the buccal-mandibular divisions of the facial nerve. Our fascial pulley technique of reanimation and suspension of the oral commissure produces no further injury to the facial nerve and the eyelids. In addition, it adds a better technique to the armamentarium of the reconstructive surgeon dealing with partial lower facial nerve paralyses.

Laryngeal epidermoid carcinoma associated with juvenile laryngeal papillomatosis.

The speculation whether juvenile laryngeal papillomatosis may transform into or be associated with epidermoid carcinoma is explored. We document a case of invasive laryngeal carcinoma arising in preexisting juvenile laryngeal papillomatosis. After multiple childhood laryngoscopies and a tracheotomy, a 54-year-old, 30-pack per year smoker, who had never received radiation therapy, developed a florid exophytic transglottic squamous cell carcinoma. Histologically, the invasive epidermoid carcinoma was surrounded by a field of papillomata with varying degrees of atypical changes. After total laryngectomy, isolated papillomata were found in the lower trachea. There were no cervical lymph node metastases. No postoperative radiation therapy was given. Persistent squamous papillomata in the tracheostomy site, the lower trachea, and the posterior pharynx were treated with the CO2 laser. We emphasize the need to maintain a high index of suspicion for malignancy. In addition, we review the problem of benign papillomata in the aerodigestive tract following laryngectomy.

Selective facial neurectomy for spastic disorders of the facial nerve.

Selective facial neurectomy in combination with bilateral musculocutaneous resection, plication brow lift, upper lid blepharoplasty, and limited rhytidectomy was performed on 18 patients with essential blepharospasm, eight with hemifacial spasm, and two with CNS vascular compression malformations. Microscopy showed the nerve tissues to be normal. Initial results were excellent. At 3 months there was a slight, persistent spastic twitching of the affected muscles in five nerves (a 14% failure rate in correcting blepharospasm). After 13 months there were four additional failures resulting from nerve regrowth in three and from one patient not completing therapy. The overall blepharospasm failure rate was 26%. On repeat neurectomy those with nerve regrowth presented with a diffuse, fine meshwork of nerve fibers reinnervating the mimetic facial musculature. In six of seven patients operated on again, spasticity was eliminated. The initial surgical failure rate has been corrected by resecting the frontal branch and the superior division of the buccal branch of the facial nerve. Only repeat neurectomy can correct long-term failures resulting from facial nerve regrowth.

Developmental temporal bone anatomy and its clinical significance: variations on themes by H. F. Schuknecht.

Fifty-eight fetal and neonatal temporal bones were studied to evaluate the mechanisms of development of the hiatus of the facial canal, hypotympanic fissures, periotic duct, tympanomeningeal fissures, and fetal inner ear vascularity. These were correlated with the clinical pathologic entities of temporal bone trauma, glomus jugulare tumor extension within the temporal bone, CNS-temporal septic conduits, and inner ear vascularity. Temporal bone developmental anatomy and histopathology provide rich sources of information on which to base the scientific and clinical study of otology.

Auditory function in children with diabetes mellitus.

Fifty-one insulin-dependent diabetics and 13 nondiabetics between the ages of 8 and 21 years, without a history of exposure to noise, ototoxic drugs or ear disease, were evaluated to examine the relation between auditory function and diabetes, diabetic control and diabetic complications. Audiologic assessment included pure-tone audiometry, speech audiometry, impedance measures and brainstem evoked response audiometry. No statistically significant differences in auditory function were noted between insulin-dependent diabetics and normal controls, between the diabetics in good or poor control or between diabetics with or without neurologic or vascular complications.

Endolymphatic sac surgery for Menière's disease.

An endolymphatic-mastoid Silastic shunt procedure was performed in 122 cases of Menière's disease having a mean follow-up period of three years. In accordance with American Academy of Ophthalmology and Otolaryngology 1972 criteria, there were 43% class A, 20% class B, 21% class C, and 17% class D results. Analysis of 35 recent cases having a mean follow-up period of nine months revealed 57% class A, 25% class B, 9% class C, and 9% class D results. Sixteen percent of the patients who experienced classes A, B or C results complained of other fluctuating symptoms which were not relieved by surgery. Moreover, three new cases of otolithic crisis were found in the postoperative group. Seven of ten patients who experienced a class A or B result had either a recrudescence of their vertigo or a significant decrement in hearing in response to a postoperative salt-loading test. It is concluded that the surgical success rate decreases with time and that the procedure appears to alter the symptom complex but does not cure Menière's disease.