Laryngeal pleomorphic rhabdomyosarcoma

Adult rhabdomyosarcoma of the larynx is a rare disease. Two cases of laryngeal rhabdomyosarcoma of the pleomorphic subtype are presented. One case was treated with surgery followed by chemotherapy and radiotherapy, the other by surgery alone. At present 20 months after treatment the patients are without local reccurrence or metastases. The pathology of the tumor, diagnosis, and treatment are discussed.     

Rhabdomyosarcoma of the larynx.

A case of rhabdomyosarcoma of the larynx in a 54-year-old man is reported. Patient presented dramatically with a large tumour mass obstructing his laryngeal inlet. The patient remains alive and well sixteen months after a total laryngectomy. Only seven previous reports of a rhabdomyosarcoma of the larynx could be found in the literature and these are compared with the case reported. It would seem that a rhabdomyosarcoma of the larynx has a better prognosis than elsewhere in the body, probably because of its earlier recognition and accessibility to radical surgery.     

Rhabdomyosarcoma of the ear and temporal bone

Rhabdomyosarcoma, the most common soft tissue sarcoma of childhood, involves the temporal bone in approximately 7% of reported cases. Until recently, the outcome of this disease was always fatal. The recent Intergroup Rhabdomyosarcoma Study Group (IRS-I) reported on the efficacy of multimodality therapy consisting of multiagent chemotherapy, radiation, and surgical resection when indicated. Twelve patients with rhabdomyosarcoma involving the temporal bone were treated between 1966 and 1988. Three patients were treated according to the IRS-I protocols and the remaining nine patients received various combinations of treatment modalities. Ten patients succumbed to their disease, most with distant metastases or intracranial extension. Two patients are alive; one at 5 1/2 years and one at 19 years. It is apparent that, although survival for rhabdomyosarcoma in general has improved with the use of IRS-I protocols, prognosis remains poor for disease involving the temporal bone and other parameningeal sites.     

Laryngeal pleomorphic rhabdomyosarcoma

A case of an extremely unusual tumor of the larynx, pleomorphic rhabdomyosarcoma, is presented with a review of literature. This is the fifth case of this malignancy described in the larynx in the English language literature. A histopathological diagnosis was made with immunohistochemistry and electron microscopy. In contrast to other reported cases, the tumor in the present case had a very aggressive behavior. Despite radical surgery involving total laryngectomy and neck dissection followed by radiation therapy, the patient died of disease 8 months following treatment. Received: 14 March 1997 / Accepted: 23 January 1998     

Laryngeal alveolar rhabdomyosarcoma involving the true vocal fold in an adult: Case report

Rhabdomyosarcoma of the larynx is extremely rare in adults, as only 17 well-documented cases have been previously reported in the English-language literature. Of these, only 2 cases (both male) involved the alveolar subtype of rhabdomyosarcoma, and neither involved the true vocal folds. We report a case of alveolar rhabdomyosarcoma of the true vocal fold in 54-year-old woman. She was successfully treated with conservative surgery, radiation, and chemotherapy. Management of head and neck rhabdomyosarcoma has evolved from radical surgery to less morbid procedures supplemented with radiation and chemotherapy. However, because this tumor is so rare in the adult population, no adult-specific treatment regimen has emerged. Nevertheless, the success of treatment in the pediatric population supports its use in adults.     

Primary rhabdomyosarcoma of the larynx

Sarcomas constitute fewer than 1% of the head and neck cancers. They represent less than 1% of laryngeal cancers. Primary rhabdomyosarcoma of the larynx is an extremely rare malignancy. The available literature on this medical oddity is in the form of isolated case reports only. The purpose of this article is to add another case of primary rhabdomyosarcoma of a rare site, the larynx, of which only 36 cases have so far been reported in the world literature. The present patient, an eighteen-year-old boy is only the third case being reported from India among all reported cases of rhabdomyosarcoma of the larynx in the world literature.     

Childhood rhabdomyosarcomas of the head and neck

The most common soft tissue sarcoma occurring in infants and children is rhabdomyosarcoma, the head and neck being a frequently involved site. Treatment philosophy underwent an evolution in 1968 from radical surgery with or without adjunctive radiation therapy to a planned protocol incorporating surgery and irradiation with combination cyclic chemotherapy involving vincristine sulfate, dactinomycin, and cyclophosphamide. This study represents an analysis of 35 infants and children treated at Columbus Children's Hospital from 1955 to 1976. An evaluation of factors is discussed with respect to effect on survival. The fact that the study includes the full spectrum of treatment philosophies ranging from the previous program of aggressive surgical resection to the current multimodality approach permits a unique opportunity to analyze the success of the recent therapeutic program.     

Primary small cell (oat cell) carcinoma of the larynx associated with an IgD multiple myeloma

Primary small cell (oat cell) carcinoma of the larynx is a rare condition. We report a case of primary oat cell carcinoma of the subglottic larynx associated with a synchronous IgD multiple myeloma (an unreported association). An increased incidence of carcinoma associated with plasma cell disorders has been reported, and the theories of this association are discussed. In a review of the reported cases, the most successful management of oat cell carcinoma of the larynx appears to incorporate a combination of radiotherapy and chemotherapy. Our case was treated with a combination of protocols used for oat cell carcinoma of the larynx and multiple myeloma. At 24 months after diagnosis, the patient is free of oat cell carcinoma, and the multiple myeloma is under control.     

Alveolar rhabdomyosarcoma of the larynx: Case report and literature review

We report the 11th well-documented case of laryngeal rhabdomyosarcoma and only the second of laryngeal alveolar rhabdomyosarcoma. The optimum treatment of head and neck rhabdomyosarcoma has not yet been defined, but it appears that the ideal should consist of an aggressive multimodality approach. This includes surgical extirpation if it can be done without major morbidity, postoperative radiotherapy with a margin around known and suspected disease, and multiagent chemotherapy. Unlike most sarcomas, rhabdomyosarcoma (especially alveolar rhabdomyosarcoma) spreads by way of the lymphatic system as well as the blood stream. Inclusion of the draining lymphatics in the radiation field must be given strong consideration.     

Alveolar rhabdomyosarcoma of the ethmoid sinus.

A case of the alveolar rhabdomyosarcoma originating in the left ethmoid sinus was reported. Despite treatment by radical excision of the tumor, irradiation and chemotherapy, the patient died of generalized metastases of the tumor six months after surgery; however, no local recurrence of the tumor was found at an autopsy. The tumor was further studied by light and electron microscopic procedures.     

True carcinosarcoma of the larynx: a case report

A case of true carcinosarcoma of the larynx occurring in a 55-year-old male is reported. The two components of this very rare tumour are pleomorphic rhabdomyosarcoma and squamous cell carcinoma. A review of the literature has revealed only two authentic cases of true carcinosarcomas.     

Laryngeal adenocarcinoma, not otherwise specified, treated with carbon dioxide laser excision and postoperative radiotherapy.

Glandular carcinomas of the larynx are aggressive neoplasms that comprise less than 1% of all laryngeal malignancies. Adenocarcinoma, not otherwise specified, is the most common histologic type. The rarity of these lesions has prohibited clarification of definitive therapy. Traditionally, radical surgery is performed because of the usually high stage at presentation and the relative insensitivity to radiotherapy. A case of glottic T1 adenocarcinoma, not otherwise specified, is reported. The tumor presented clinically as a vocal fold granuloma. The treatment modalities included endoscopic excision with the carbon dioxide laser and postoperative radiotherapy. The patient has been followed up for 39 months without evidence of recurrence or metastases. The current literature is reviewed in regard to treatment of laryngeal glandular carcinomas.     

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.     

Papillomas of the larynx and pharynx: Two case reports

Two unusual cases of papillomas are presented: the first is that of a young boy with extensive papillomas of the larynx who was treated successfully without tracheotomy using endolaryngeal surgery and steroids at the time of each procedure. This case adds to the clinical evidence that the most probable cause of juvenile papillomas is a virus, since the mother had condyloma acuminata during pregnancy and at term. The second case is that of a young girl with rapidly recurring papillomas of the mouth and pharynx who was treated successfully with an autogenous vaccine and cryosurgery after other measures failed. This case demonstrates the effectiveness of cryosurgery in the treatment of papillomas and the possible efficacy of the autogenous vaccine suggests a viral etiology for papillomas of the pharynx and larynx.     

Paranasal sinus mucormycosis in an immunologically competent host

A case of paranasal sinus mucormycosis in an immunocompetent patient is reported. After an extensive evaluation, no evidence of either diabetes mellitus or underlying immunologic abnormality was found. The combination of excisional surgery and amphotericin B therapy resulted in an excellent response. High-resolution axial and coronal computed tomography was useful in both planning treatment and monitoring the response to therapy.     

Carcinoid tumour of the larynx (a case report and review of the literature)

Carcinoid tumours of the larynx are extremely rare, only 13 cases having previously been reported in the world literature. A case is reported here with the unusual complication of skin metastases. The true diagnosis is seldom made on the original biopsy, which is commonly reported as poorly differentiated carcinoma. A review of the literature is made with a discussion of the histological diagnosis and treatment modalities.     

Current trends in initial management of laryngeal cancer: the declining use of open surgery

The role of open surgery for management of laryngeal cancer has been greatly diminished during the past decade. The development of transoral endoscopic laser microsurgery (TLS), improvements in delivery of radiation therapy (RT) and the advent of multimodality protocols, particularly concomitant chemoradiotherapy (CCRT) have supplanted the previously standard techniques of open partial laryngectomy for early cancer and total laryngectomy followed by adjuvant RT for advanced cancer. A review of the recent literature revealed virtually no new reports of conventional conservation surgery as initial treatment for early stage glottic and supraglottic cancer. TLS and RT, with or without laser surgery or CCRT, have become the standard initial treatments for T1, T2 and selected T3 laryngeal cancer. Photodynamic therapy (PDT) may have an emerging role in the treatment of early laryngeal cancer. Anterior commissure involvement presents particular difficulties in application of TLS, although no definitive conclusions have been reached with regard to optimal treatment of these lesions. Results of TLS are equivalent to those obtained by conventional conservation surgery, with considerably less morbidity, less hospital time and better postoperative function. Oncologic results of TLS and RT are equivalent for glottic cancer, but with better voice results for RT in patients who require more extensive cordectomy. The preferred treatment for early supraglottic cancer, particularly for bulkier or T3 lesions is TLS, with or without postoperative RT. The Veterans Administration Study published in 1991 established the fact that the response to neoadjuvant CT predicts the response of a tumor to RT. Patients with advanced tumors that responded either partially or completely to CT were treated with RT, and total laryngectomy was reserved for non-responders. This resulted in the ability to preserve the larynx in a significant number of patients with locally advanced laryngeal cancer, while achieving local control and overall survival results equivalent to those achieved with initial total laryngectomy. Following this report, similar “organ preservation” protocols were employed in many centers. By 2003, results of the RTOG 93-11 trial, utilizing CCRT as initial treatment, were published, demonstrating a higher rate of laryngeal preservation with this protocol. Surgery was reserved for treatment failures. This concept changed the paradigm for management of advanced laryngeal cancer, greatly reducing the number of laryngectomies performed. While supracricoid laryngectomy has been employed for selected patients, total laryngectomy is the usual procedure for salvage of failure after non-surgical treatment.     

Lower-extremity liposarcoma metastatic to the larynx: case report

Distant metastases to the larynx are rare. We describe the case of a 46-year-old man who was referred to our head and neck surgery clinic with a 6-week history of sore throat and otalgia. He was found to have a laryngeal lesion that was consistent with a primary myxoid liposarcoma that had been extirpated from a lower extremity earlier To the best of our knowledge, no case of myxoid liposarcoma metastatic to the larynx has been previously reported in the English-language literature.     

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.     

Metastases to the larynx (author's transl)

Four cases of metastases to the larynx are reported and 43 similarly reported in the literature are reviewed. Most were metastases from hypernephromas or melanomas, producing supraglottic soft tissue tumours. Metastases from bronchial and mammary carcinomas in most cases are "bone metastases" within the laryngeal skeleton. Metastases in the larynx from all other malignancies are extremely rare. The clinical picture is variable and can cause considerable diagnostic difficulty especially in laryngeal skeletal metastases. Solitary metastases can be successfully treated by surgery and irradiation.