Malignant paraganglioma of frontoethmoidal region

Nonchromaffin paragangliomas are unusual tumours arising from widely distributed paraganglionic tissues probably of neural crest origin. In the head and neck region they are usually seen as carotid body or jugulotympanic tumours. Other rarely reported sites in the head and neck region are the orbit, nose and larynx. This report deals with a case of sinonasal paraganglioma which was initially treated with surgery and radiotherapy. Twenty two years later the tumour recurred and showed a rapid growth due to malignant transformation which we believe is late effect of radiotherapy. The clinical features, histopathology and role of radiotherapy in sinonasal paragangliomas together with a review of the medical literature have been discussed.     

Actinomycosis of the post-cricoid space: an unusual cause of dysphagia.

Cervicofacial actinomycosis is known to affect many soft tissues and bony structures in the head and neck. However to the authors' knowledge, actinomycosis of the post-cricoid region has not been previously reported. A case of a 74-year-old male who developed actinomycosis of the post-cricoid region after radiotherapy for a laryngeal carcinoma is presented. Actinomycosis should be considered in the differential diagnosis of dysphagia following radiotherapy for squamous cell carcinoma of the larynx, as early treatment is likely to result in a favourable outcome.     

Carcinoma of the larynx in a child

Squamous cell carcinoma of the larynx in children is a rare entity, only 21 cases have been reported in patients 10-years-old or younger. A 9-year-old girl presented because of dyspnea and mild hoarseness of one week duration. Laryngoscopy and bronchoscopy showed a supraglottic mass, which proved to be poorly differentiated squamous cell carcinoma. No neck nodes or systemic metastasis were present. The tumor regressed with radiation therapy (6400 rads) but local recurrence and lung metastasis were evident 6 weeks later. Chemotherapy was refused by the parents and the patient died in one month. It is believed that squamous cell carcinoma of the larynx in children parallels that of adults in many respects including response to treatment. However, our attempt to save the larynx by avoiding surgery and using radiotherapy alone was not successful. Squamous cell carcinoma of the larynx in children may be more aggressive than that of adults. The scarcity of cases impedes establishment of treatment protocols in children. Initial aggressive management using surgery, radiotherapy, and possibly chemotherapy may be warranted in children.     

Basaloid squamous cell carcinoma of the larynx

Basaloid squamous cell carcinoma (BSCC) is a rare tumor with distinct morphological and biological features that differentiate it from the common form of squamous cell carcinoma (SCC) in the head and neck region. It is mostly seen in the supraglottic larynx, hypopharynx and a base of the tongue. We present two cases of BSCC of the larynx; both being transglottic tumors. Both of the patients underwent primary surgery including bilateral neck dissections. None of the patients had cervical metastases at histopathological examination. Both patients received radiotherapy after surgery. They were alive and free of disease at 24 and 27 months, respectively.     

Small cell anaplastic carcinoma of the larynx: Review of the literature and report of a case

The case study of a patient with small cell anaplastic carcinoma (oat cell) of the larynx is detailed and the literature is reviewed. Small cell anaplastic carcinoma of the larynx has been demonstrated to be histologically identical to small cell anaplastic carcinoma of bronchogenic origin. The aggressive biologic behavior of this tumor justifies managing small cell anaplastic carcinoma as a systemic disease. Because of early widespread dissemination of tumor, surgery or radiotherapy alone or in combination have not been successful in controlling the disease. The combination of radiotherapy with chemotherapy has been shown to be the most effective approach to the treatment of small cell anaplastic carcinoma of the lung. We believe that a similar regimen should be considered the treatment of choice for small cell anaplastic carcinoma of the larynx.     

Delayed radionecrosis of the larynx.

Radiation has been used to treat carcinoma of the larynx for more than 70 years. Radionecrosis is a well-known complication of this modality when treating head and neck neoplasms. It has been described in the temporal bone, midface, mandible, and larynx. Laryngeal radionecrosis is manifested clinically by dysphagia, odynophagia, respiratory obstruction, hoarseness, and recurrent aspiration. The vast majority of patients who develop laryngeal radionecrosis present with these symptoms within 1 year of treatment; however, delayed presentations have been reported up to 25 years after radiotherapy. We present, in a retrospective case analysis, an unusual case of laryngeal radionecrosis in a patient who presented more than 50 years after treatment with radiotherapy for carcinoma of the larynx. The cases of delayed laryngeal necrosis in the literature are presented. This represents the longest interval between treatment and presentation in the literature. The details of the presentation, clinical course, and diagnostic imaging are discussed. The pathogenesis, clinical features, and treatment options for this rare complication are reviewed. Early stage (Chandler I and II) laryngeal radionecrosis may be treated conservatively and often observed. Late stage (Chandler III and IV) cases are medical emergencies, occasionally resulting in significant morbidity or mortality. Aggressive diagnostic and treatment measures must be implemented in these cases to improve outcome. This case represents the longest interval between initial treatment and presentation of osteoradionecrosis in the literature. A structured diagnostic and therapeutic approach is essential in managing this difficult problem.     

Small cell neuroendocrine carcinoma in the petrous apex

With the exception of moderately differentiated neuroendocrine carcinoma of the larynx, neuroendocrine carcinomas are very rare head and neck malignancies. We report a case of a small cell neuroendocrine carcinoma in the petrous apex of the temporal bone. This unusual site for this tumour has not yet been reported in the literature. We confirmed the location and extent of the primary tumour by positron-emission tomography scan. The final histopathological diagnosis was of small cell carcinoma, and this was confirmed by immunohistochemistry. We treated this patient with surgery followed by radiotherapy and chemotherapy. After the treatment was completed there was subtotal remission of the tumour, with no distant metastases.     

Preoperative chemotherapy in advanced resectable head and neck cancer: final report of the Southwest Oncology Group

In 1980, the Southwest Oncology Group instituted a multi-institutional, prospective, randomized phase III trial to evaluate whether inductive chemotherapy improved survival in patients with advanced stage resectable squamous cell carcinoma of the head and neck. From a group of 158 eligible patients, 76 were randomized to conventional treatment (surgery and postoperative radiotherapy), and 82 were assigned to experimental treatment (induction chemotherapy, surgery, postoperative radiotherapy). Median follow-up for living patients was approximately 5 years. These analyses include chemotherapy responses and toxicities, surgical complications, radiotherapy toxicities, patient compliance, survival time, and patterns of treatment failure. Overall chemotherapy response was 0.70 (0.19 CR, 0.51 PR). The median survival time for conventional treatment was longer than the time for patients receiving preoperative chemotherapy, although the survival time differences were not statistically significant. This final analysis demonstrates no benefit in survival using preoperative chemotherapy for advanced stage, resectable head and neck squamous cell carcinoma.     

Treatment of cancer of the larynx. Analysis of success and failure

The treatment of patients with cancer of the larynx must consider the potential for multiple sites of origin and the possibility of regional metastasis. Treatment options must also address effects on the voice, deglutition, and the sphincteric function of the larynx which protects the lungs. Fortunately, our advanced techniques in surgery--reconstruction, radiotherapy, and endoscopic laser application--enable us to choose from and use these modalities in combination. Induction chemotherapy appears to be a promising, albeit unproved, adjuvant therapy. The oncologist dealing with cancer of the head and neck must select appropriate individualized therapy in a multidisciplinary environment.     

Outcome of locally advanced stage III and IV head and neck cancer treated by surgery and postoperative external beam radiotherapy

An uncontrolled retrospective analysis of 76 patients with locally advanced Stage III and Stage IV squamous cell carcinoma of the oral cavity, oropharynx, pyriform sinus, supraglottic larynx, glottic larynx, and hypopharynx, who were treated in a uniform manner by surgical resection and 6,600 rad postoperative external beam radiotherapy, revealed relatively high 2-year and 4-year adjusted survival rates of 76% and 68%, respectively. Complication rates were acceptable (8%). The advantages of this treatment approach for locally advanced head and neck cancers compared to treatment by surgery alone are discussed.     

The dilemma of treating hypopharyngeal carcinoma: more or less: Hayes Martin Lecture

The optimal therapy for hypopharyngeal carcinoma depends on its staging. For early-stage disease, radiotherapy and surgery achieve similar results. Radical surgery followed by radiotherapy is applicable in the management of patients with advanced-stage disease. Chemoradiation aiming to preserve the larynx can only be performed for selected patients and in well-equipped institutions. Thorough understanding of pathological behavior of hypopharyngeal carcinoma, its submucosal tumor extension, and its high propensity to metastasize to cervical lymph nodes allows head and neck surgeons to choose optimal surgical treatment. Lymph node status determines the type of neck dissection required while location and size of the primary tumor determine the extent of resection and choice of reconstruction procedure. Adequate tumor extirpation with less extensive and invasive procedures preserving unaffected normal tissue contribute to more tumor control and less morbidity.     

Laryngeal carcinoma in a non-smoker female patient with thyroid carcinoma: report of a case

Laryngeal carcinoma is usually encountered in smoker men, and thyroid carcinoma is sometimes discovered incidentally during treatment for these patients. However, this coexistence of malignancies could occur in non-smoker female. We report an unusual case of multiple primary malignancies in the larynx and the thyroid gland. The laryngeal carcinoma was suspected to be related to the malignant transformation of the papillomas. The case suggests the importance of meticulous examination in the head and neck region for treatment of cervical metastatic lymph nodes with negative cytology in non-smoker female.     

Paraneoplastic syndromes in patients with primary malignancies of the head and neck. Four cases and a review of the literature

Paraneoplastic syndromes rarely affect patients with head and neck cancer. Four patients with different histological types of head and neck cancer are presented in which the primary malignancy was preceded and/or accompanied by a paraneoplastic syndrome. In the first patient erythrodermia preceded the diagnosis of a nasopharyngeal carcinoma. The second patient presented with a B cell lymphoma of the nasopharynx in association with the syndrome of inappropriate secretion of arginine vasopressine (Schwartz-Bartter syndrome). In the third patient paraneoplastic polyarthritis had been diagnosed 5 months before a hypopharyngeal carcinoma was diagnosed. In the last patient the paraneoplastic anti-Hu positive encephalomyelitis was associated with a primary malignancy in the larynx with neck metastases. Diagnostic procedures, treatment and follow-up of these patients are reported and accompanied by a review of the literature.     

Metastatic endometrial carcinoma of the neck

We report a case of metastatic endometrial carcinoma of the neck. A patient with a past medical history of squamous cell carcinoma of the larynx, breast carcinoma and endometrial carcinoma presented with a neck mass. Fine needle aspiration cytology (FNAC) showed this to be a poorly differentiated carcinoma with squamoid features and thus a potentially curative neck dissection was performed. Histology of the mass showed a clear cell endometrial carcinoma. Metastatic gynacecological malignancies to the head and neck are rare and this is the first reported case of metastatic endometrial carcinoma in the neck.     

Three cases of cell granular tumor of the larynx

Cell Granular Tumor (CGT) are unusual tumors that can affect any site of the body, with most cases affecting the head and neck region. CGT of the larynx is an uncommon laryngeal tumor accounting for approximately 6-10% of the reported cases. In the larynx pseudoepitheliomatous hyperplasia may predispose to confuse the CGT with frothy (squamous) cell carcinoma. The origin of this tumor is a matter of debate but most authors believe it to be neural in origin.     

Clinical and histopathological considerations for the diagnosis and treatment of verrucous carcinoma of the larynx

Verrucous carcinoma of the larynx is quite distinct from well-differentiated squamous cell carcinoma has a characteristic morphology and specific clinical behavior. It accounts for approximately 1%-2% of all laryngeal carcinomas. However, difficulties are encountered clinically in separating verrucous carcinomas from keratoses, verruca vulgaris and squamous cell carcinomas with a verrucous appearance. Between 1962 and 1982, 1504 patients with squamous cell carcinomas of the larynx were diagnosed and treated by staff physicians in the Department of Otolaryngology--Head and Neck Surgery, Washington University in St. Louis. Fifteen of these patients (1%) had verrucous carcinomas. These cases were studied in order to clarify any diagnostic problems, trace the biologic behavior of the tumors and report the results of the treatment used. Of these 15 patients, we were able to follow up 8. The latter had been treated only with surgery and none of them had received radiotherapy. One death occurred, but the patient died of other causes and remained free of cancer. Reports in the literature describe a high rate of recurrence of verrucous carcinomas following radiotherapy with occasional anaplastic transformation of tumors. No patients in our series developed a metastasis in the neck, indicating that neck dissection is not justified for patients with these neoplasms even though clinically enlarged neck nodes may be present.     

Eight cases of neuroendcrine carcinoma of the head and neck

Small cell neuroendocrine carcinoma of the head and neck is rare, and diagnosis may be difficult. Malignancy is high, as is the incidence of distant metastasis. We reported eight cases of stage IV small cell neuroendocrine carcinoma of the head and neck, all in men with a mean onset age of 62 years (range: 45 to 80 years). Three cases arose from the maxillary sinus, two from the ethmoid sinus, one from the parotid gland, one from the tonsil, and one from the larynx. Histological analysis by hematoxylin-eosin staining tentatively revealed malignant lymphoma and undifferentiated carcinoma in two cases each, while immunohistological and/or electron microscopy analysis confirmed histological diagnosis. All were treated by chemotherapy (VP-16, CDDP) and seven cases with radiotherapy based on the schedule of small cell carcinoma of the lung and two cases with lesional resection. Chemotherapy and radiotherapy were effective locally. Five patients died of distant metastasis to the brain, bone, lung, liver, or skin within 12 months. One is alive with liver metastasis. Two have no evidence of disease--one for eight years and the other for one year. Long-term survival thus requires the effective treatment of distant metastasis.     

Merkel cell carcinoma of unknown primary site

Merkel cell carcinoma is an uncommon and aggressive primary neuroendocrine skin malignancy which mostly affects the extremities and the head and neck region of elderly patients. Merkel cell carcinoma occurs with increased frequency in sun-exposed areas, in individuals exposed to arsenic and in immunosuppressed patients. Many patients with Merkel cell carcinoma present with other malignancies, mainly skin cancers. Characteristic features are frequent recurrences and regional and distant metastases. Mortality rates range from 20 to 65 per cent. The mainstay of treatment is surgery, with wide local excision, and adjuvant radiotherapy is usually administered. Merkel cell carcinoma of unknown primary site is rare, and the majority of the few cases described have not been from head and neck areas. We present a case of Merkel cell carcinoma of unknown primary site, with upper neck and distant metastases.     

Immunohistochemical analysis of small cell carcinoma of the head and neck: a report of four patients and a review of sixteen patients in the literature with ectopic hormone production

Small cell carcinoma (SCC) occurs mostly in the lung, and in some patients is accompanied by production of ectopic hormones. Small cell carcinoma of the head and neck is very rare. We report 4 patients with SCC of the head and neck (larynx, tonsil, maxillary sinus, and parotid gland). The patient with SCC of the maxillary sinus demonstrated a high level of plasma serotonin and overexpression of parathyroid hormone; however, he did not show any related symptoms. The patient with SCC of the tonsil showed the syndrome of inappropriate secretion of antidiuretic hormone associated with antidiuretic hormone hyperproduction at the terminal stage. In the literature, 16 patients with SCC of the head and neck with ectopic hormone production have been reported. Antidiuretic hormone and adrenocorticotropic hormone were the hormones that caused clinical symptoms (paraneoplastic syndromes). We believe that the evaluation of hormonal syndromes is valuable for diagnosis and treatment.     

Photodynamic therapy and the treatment of neoplastic diseases of the larynx

Photodynamic therapy (PDT) is an innovative treatment involving the use of light-sensitive drugs to selectively identify and destroy diseased cells. Therefore, photodynamic therapy has the potential to treat and cure precancerous and early cancerous lesions (carcinoma in situ [CIS], T1 and T2) of the larynx while preserving normal tissue. Eleven patients with recurrent leukoplakia and carcinomas of the larynx were treated with PDT with follow-up to 27 months. One patient with a Tl verrucous carcinoma, 5 patients with T1 squamous cell carcinomas of the vocal cord failing radiotherapy, 1 patient with a T2 squamous cell carcinoma of the vocal cord failing radiotherapy, and 3 patients with CIS and severe atypia were treated with PDT and obtained a complete response and are disease free. One patient with a T3 carcinoma of the larynx was treated with PDT but died 4 weeks post-treatment of unrelated causes and could not be assessed. Photodynamic therapy is a promising therapy for treatment of precancerous and cancerous lesions of the larynx. This therapy may be particularly beneficial for the treatment of recurrent carcinomas of the larynx that have failed conventional radiotherapy, thereby preserving voice and eliminating the need for destructive laryngeal surgery.