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Laryngeal Involvement of Rhabdomyosarcoma in an Adult.

Abali H, Aksoy S, Sungur A, Yalçin S - World J Surg Oncol (2003)

Bottom Line: BACKGROUND: 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.CASE REPORT: A case of rhabdomyosarcoma in the right thigh, which involved laryngeal structures three years after the diagnosis, is presented.CONCLUSIONS: The laryngeal involvement by primary and metastatic rhabdosarcomas is very rare, however when it occurs it can result in life treating upper airway obstruction.

View Article: PubMed Central - HTML - PubMed

Affiliation: Hacettepe University Oncology Institute, Department of Medical Oncology, Ankara, Turkey. saksoy07@yahoo.com

ABSTRACT
BACKGROUND: 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. CASE REPORT: A case of rhabdomyosarcoma in the right thigh, which involved laryngeal structures three years after the diagnosis, is presented. CONCLUSIONS: 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.

No MeSH data available.


Related in: MedlinePlus

Photomicrograph showing sheets of round and spindled cells (H&E ×230)
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Figure 1: Photomicrograph showing sheets of round and spindled cells (H&E ×230)

Mentions: A 45 year-old man presented with a complaint of a lump in the inner side of the right thigh for the past 2 months. Excision biopsy was performed. Histologically the tumor was composed of sheets of malignant round and spindled cells in a variably myxoid stroma. Some cells had abundant eosinophilic cytoplasm. Atypical mitotic figures were seen (Figure 1). Immunohistochemical stains revealed that the tumor cells were positive for vimentin, desmin and myod1. With a diagnosis of rhabdomyosarcoma combination chemotherapy with ifosfamide (2500 mg/m2 for 3 days, together with mesna at the same doses) and adriamycine (60 mg/m2 on day 1) was given for six cycles as adjuvant treatment. Five months following completion of chemotherapy, a hemipelvectomy was performed upon local recurrence of the tumor in the previous location. The pathological examination was consistent with rhabdomyosarcoma. The DICE (dexamethasone, ifosfamide, carboplatin and etoposide) combination adjuvant chemotherapy was started. However, it had to be stopped after the 3rd cycle due to elevation of aminotransferases as high as 5 times the upper limit of normal ranges. After almost a year of uneventful period, a 3 cm metastatic nodule in the superior segment of left lower lobe was discovered and metastatectomy was performed. The histopathological examination was consistent with malignant mesenchymal tumor. He had received a total 4 courses of ifosfamide at the dose of a total of 14 g/m2 in 7 days with hematopoietic growth factor support every month. Four months after the last course he began to experience hoarseness and mild dyspnea. Physical examination showed multiple polypoid masses in the sub-glottic region. Magnetic resonance images of head and neck were showed the laryngeal masses (Figure 2). Biopsy revealed rhabdomyosarcoma. As the curative surgery was not feasible, palliative radiotherapy was given to relieve his symptoms. He experienced only a mild throat pain during radiotherapy. However, 2 months later he noticed a mass of an almond size in his mouth. On inspection of oral cavity, a 3 cm mass just between left tonsil and tongue, covered with a necrotic pseudomembrane, was discovered. Excisional biopsy showed rhabdomyosarcoma. CT of head and region disclosed several other metastatic lesions. He was re-admitted to hospital 7 days after following his last discharge with acute severe dyspnea and wheezing. His complaints responded well to dexamethasone 16 mg/day. Palliative chemotherapy with VAC (vincristine, actinomycine, cyclophosphamide) every week was started. After the 1st cycle, the mass in the floor of mouth disappeared.


Laryngeal Involvement of Rhabdomyosarcoma in an Adult.

Abali H, Aksoy S, Sungur A, Yalçin S - World J Surg Oncol (2003)

Photomicrograph showing sheets of round and spindled cells (H&E ×230)
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC222905&req=5

Figure 1: Photomicrograph showing sheets of round and spindled cells (H&E ×230)
Mentions: A 45 year-old man presented with a complaint of a lump in the inner side of the right thigh for the past 2 months. Excision biopsy was performed. Histologically the tumor was composed of sheets of malignant round and spindled cells in a variably myxoid stroma. Some cells had abundant eosinophilic cytoplasm. Atypical mitotic figures were seen (Figure 1). Immunohistochemical stains revealed that the tumor cells were positive for vimentin, desmin and myod1. With a diagnosis of rhabdomyosarcoma combination chemotherapy with ifosfamide (2500 mg/m2 for 3 days, together with mesna at the same doses) and adriamycine (60 mg/m2 on day 1) was given for six cycles as adjuvant treatment. Five months following completion of chemotherapy, a hemipelvectomy was performed upon local recurrence of the tumor in the previous location. The pathological examination was consistent with rhabdomyosarcoma. The DICE (dexamethasone, ifosfamide, carboplatin and etoposide) combination adjuvant chemotherapy was started. However, it had to be stopped after the 3rd cycle due to elevation of aminotransferases as high as 5 times the upper limit of normal ranges. After almost a year of uneventful period, a 3 cm metastatic nodule in the superior segment of left lower lobe was discovered and metastatectomy was performed. The histopathological examination was consistent with malignant mesenchymal tumor. He had received a total 4 courses of ifosfamide at the dose of a total of 14 g/m2 in 7 days with hematopoietic growth factor support every month. Four months after the last course he began to experience hoarseness and mild dyspnea. Physical examination showed multiple polypoid masses in the sub-glottic region. Magnetic resonance images of head and neck were showed the laryngeal masses (Figure 2). Biopsy revealed rhabdomyosarcoma. As the curative surgery was not feasible, palliative radiotherapy was given to relieve his symptoms. He experienced only a mild throat pain during radiotherapy. However, 2 months later he noticed a mass of an almond size in his mouth. On inspection of oral cavity, a 3 cm mass just between left tonsil and tongue, covered with a necrotic pseudomembrane, was discovered. Excisional biopsy showed rhabdomyosarcoma. CT of head and region disclosed several other metastatic lesions. He was re-admitted to hospital 7 days after following his last discharge with acute severe dyspnea and wheezing. His complaints responded well to dexamethasone 16 mg/day. Palliative chemotherapy with VAC (vincristine, actinomycine, cyclophosphamide) every week was started. After the 1st cycle, the mass in the floor of mouth disappeared.

Bottom Line: BACKGROUND: 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.CASE REPORT: A case of rhabdomyosarcoma in the right thigh, which involved laryngeal structures three years after the diagnosis, is presented.CONCLUSIONS: The laryngeal involvement by primary and metastatic rhabdosarcomas is very rare, however when it occurs it can result in life treating upper airway obstruction.

View Article: PubMed Central - HTML - PubMed

Affiliation: Hacettepe University Oncology Institute, Department of Medical Oncology, Ankara, Turkey. saksoy07@yahoo.com

ABSTRACT
BACKGROUND: 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. CASE REPORT: A case of rhabdomyosarcoma in the right thigh, which involved laryngeal structures three years after the diagnosis, is presented. CONCLUSIONS: 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.

No MeSH data available.


Related in: MedlinePlus