Laryngeal Involvement of Rhabdomyosarcoma in an Adult
© Abali et al; licensee BioMed Central Ltd. 2003
Received: 12 July 2003
Accepted: 17 September 2003
Published: 17 September 2003
Although initial presentation of sarcomas in the head and neck region is relatively common, involvements of the larynx by rhabdomyosarcoma either primary or metastatic have been reported rarely.
A case of rhabdomyosarcoma in the right thigh, which involved laryngeal structures three years after the diagnosis, is presented.
The laryngeal involvement by primary and metastatic rhabdosarcomas is very rare, however when it occurs it can result in life treating upper airway obstruction. After emergency intervention, radiotherapy and / or chemotherapy must be undertaken for long-term benefit and surgery may be helpful only in selected cases.
KeywordsRhabdomyosarcoma larynx primary metastatic
Laryngeal cancer is an important cause of morbidity and mortality in smokers accounting for 25% of the head and neck cancers . Over 95 percent of the cancers of the larynx are of squamous histology. Non-squamous histology including sarcomas and salivary gland tumors are quite rare. Primary involvement of the larynx by mesenchymal tumors has been reported as case reports [2, 3]. Metastasis to the larynx from a neoplasia elsewhere is even rare, making 0.09 % to 0.4 % of total . Until present, about 150 metastatic cancers of larynx of various histology have been reported. Here, we report a case of rhabdomyosarcoma in the right thigh, which involved laryngeal structures three years after the diagnosis.
Rhabdomyosarcoma is a tumor of striated muscle. It has mainly two subtypes: Embryonel and alveolar. While the loss of heterozygosity of chromosome 11p15 identifies the first one, latter is characterized by the translocation t(2;13) (q35;q14) . It is the most common malignant mesenchymal tumor in children. Therefore most of the information is inspired from pediatric studies. It is quiet rare in adults. The most common site of presentation is head and neck region (35 %). Lungs, bone marrow, bones, liver and brain are among the most common sites of metastases . Ifosfamide, adriamycin, etoposide, vincristine and actinomycin are among effective agents in the treatment of soft tissue sarcomas [6–9]. Survival ranges from 30% in patients with metastatic presentation to 80% in those where the disease is localized and complete resection is performed .
Upper airway obstruction, irrespective of the cause, is an emergency. Therefore it is a must to intervene rapidly. Corticosteroids may be beneficial by relieving accompanying edema. However immediate tracheotomy can be required and when necessary should be performed undoubtedly. After emergency intervention, palliative measures like radiotherapy and / or chemotherapy must be undertaken for long-term benefit. However surgery may be helpful only in selected cases.
Although it is difficult to make the distinction between metastatic or primary for sure, we supposed our case was a metastatic invasion rather than primary in an adult owing to previous diagnosis of rhabdomyosarcoma, similar histology and multiplicity of the tumor and rarity of primary involvement of larynx in adults .
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