Extraskeletal osteosarcoma of the larynx: an extremely unusual tumour

Abstract

Osteosarcoma of the larynx is probably the rarest mesenchymal tumour of the larynx, with only 16 cases reported so far. The majority of them occur in males between the sixth and eighth decades of life. Patients usually present with non-specific symptoms such as dysphonia and upper airway compromise. The most common site of distant metastasis is the lung. Clinically, the tumour follows an aggressive course and is associated with high mortality. The case we present is unusual as it occurred at a young age (38 years) as compared with the norm and it did not arise from the endolarynx, unlike many of the other cases. This was the only known case where a Pearson near-total laryngectomy was performed whereby the patient's natural speech mechanism was preserved. This surgery was possible because the contralateral half of the larynx was clearly disease free and the interarytenoid region was uninvolved. The patient underwent postoperative adjuvant external beam radiotherapy beginning 4 weeks after surgery. The patient is doing well after 15 months of follow-up and shows no signs of recurrence.

Characteristics of Laryngeal Osteosarcoma: A Critical Review

“…Odynophagia NED at 20 months Neck mass High-grade osteoblastic OS No metastases. Partial LE Baroda, Gujarat, India Bhatt et al [ 19 ] 2014 38/M. Smoker.…”

Extraskeletal osteosarcoma in right neck subcutaneous tissue: A case report of an extremely rare tumour

“…There are several treatment methods for ESOS, including surgery, radiation therapy, and chemotherapy ( 2 – 4 , 10 , 11 ), surgical resection is dominated, 85 to 98% ( 10 , 11 ), even all patients with localized disease were managed with surgical resection of the primary tumor ( 4 ), radical resections are effective for local control and have the best chance of cure for ESOS ( 8 ), 5-year OS and 5-year disease-free survival (DFS) was 51.4 and 43% respectively ( 10 ), but the proportion of patients treated alone with surgery was low (21.8%) ( 10 ), and its effect on distant metastasis is not so clear, multiagent chemotherapy may be help to reduce distant metastasis, a trend towards increased length of survival was found in patients who received chemotherapy compared to those who did not (16.4 months vs. 9.3 months) ( 2 ), the gemcitabine-docetaxel chemotherapy regimen was considered as well-tolerated and induced a long lasting partial response for ~14 months in the treatment of ESOS ( 17 ). Higher survival was observed in patients who received perioperative chemotherapy with a trend in favour of multiagent osteosarcoma-type regimen which included doxorubicin, ifosfamide and cisplatin ( 10 ), and postoperative adjuvant external beam radiotherapy being considered to improve local control rate and preserve organ function ( 18 , 19 ), and especially the patients who with tumour >5 cm and R0 margins seems to benefit more from RT ( 10 ), and there is a tendency to extend 5-y DFS in patients who underwent postoperative adjuvant RT compared with surgery alone (66 vs. 42%, P=0.38) ( 11 ), though RT was not associated with a lower disease-related mortality rate or a longer event-free survival ( 4 ). However, the available data are contradictory with regard to the use of chemotherapy and radiotherapy regimens in the management of ESOS, radiographic response rates and pathologic complete response rates to doxorubicin-based systemic therapy are low ( 3 ), no significant association of disease-specific or event-free survival was found with the addition of radiation, chemotherapy, or both to surgery, radiation and chemotherapeutic treatment were not associated with a lower incidence of death due to disease or a longer event-free survival ( 4 ).…”

Primary osteosarcoma of the parotid gland: A case report and literature review

“…[1] Extraskeletal osteosarcoma of gall bladder, esophagus, colon-rectum, larynx, pleura, kidney, small intestine, liver, heart, urinary bladder and breast are also have been reported in the literature. [2,[6][7][8][9][10][11] However, primary extraskeletal osteosarcoma in the parotid gland is extremely rare. Only five cases have been reported.…”

“…[1] Visceral involvement also have been reported. [2,[6][7][8][9][10][11] Since 1980s (see Table 1), [12,13] only five cases of primary osteosarcomas in the parotid gland were reported in the literature.…”