Well-Differentiated Laryngeal/Hypopharyngeal Liposarcoma in the MDM2 Era Report of Three Cases and Literature Review

Abstract

Laryngeal/hypopharyngeal liposarcomas are very rare, fewer than 40 cases have been reported. These tumors are polypoid, with a male predisposition, and usually cause hoarseness and difficulty breathing. Their clinical course is characterized by multiple local recurrences. No distant metastasis has been reported, and dedifferentiation is extremely rare. In sum, the prognosis of these tumors is excellent; the 5-year survival rate is essentially 100 %. Pathologic diagnosis of these well-differentiated liposarcomas can be challenging. Many of them were initially diagnosed as benign lipoma, fibrovascular polyp, or "inflammatory polyp". The correct diagnosis is usually made after multiple recurrences. On the other hand, the literature bears out that these incorrect diagnoses do not impact disease-specific survival. Here, we report three patients with laryngeal/hypopharyngeal well-differentiated liposarcomas; this is the first documentation of MDM2 amplification in liposarcomas at this site.

Prevalence of MDM2 amplification and coalterations in 523 advanced cancer patients in the MD Anderson phase 1 clinic

“…In contrast, Lyle et al [ 45 ] found no MDM2 amplification in their series of ten tests among 38 patients with malignant phyllodes tumor of the breast. Zhu et al [ 46 ] reported the first documented cases of MDM2 amplification in laryngeal and hypopharyngeal liposarcoma. In a separate study by Kato et al [ 47 ], MDM2 has been recognized as a marker of pseudo-progression in patients treated with single-agent checkpoint (PD-1/PDL-1) inhibitors.…”

Lipossarcoma de laringe: relato de caso

“…Deve ser realizada quando não é possível preservar a função do órgão. Já o uso de tratamento adjuvante não é consenso na literatura 4,5 .…”

“…O lipossarcoma é o sarcoma mais comum da vida adulta, representando 20% dos diagnósticos de sarcoma, acometendo principalmente membros inferiores e retroperitônio 3,4 . Desses, apenas 5% são encontrados na região de cabeça e pescoço e apenas uma pequena parcela é encontrada na laringe 5,6 .…”

“…O lipossarcoma de laringe atinge principalmente o sexo masculino, acometendo qualquer faixa etária, principalmente na quinta década de vida com idade média de apresentação de 46 anos 7 . Trata-se de uma neoplasia extremamente rara -existindo aproximadamente 40 casos descritos na literatura, desses nenhum em língua portuguesa 2,4 .…”

Das Liposarkom im Kopf-Hals-Bereich – Ein seltener Fall

“…[5]  Im Larynx und Hypopharynx gibt es in der Weltliteratur bisher weniger als 40 dokumentierte Fälle. [1,3,4] Ein…”