Head and Neck Rhabdomyosarcoma in Adults

Abstract

Head and neck RMS in adults is a rare malignancy with a poor outcome, which is more likely to have CLN involvement compared with other soft tissue sarcomas of the head and neck. Standard treatment for AHNRMS should comprise surgery and chemotherapy.

A clinicopathologic study of head and neck rhabdomyosarcomas showing FOXO1 fusion-positive alveolar and MYOD1 -mutant sclerosing are associated with unfavorable outcome

“…The clinical presentation of HNRMS is divided into 3 sub-sites based on its anatomic location and local relapse: parameningeal (PM), including the paranasal sinuses, nasopharynx, nasal cavity, middle ear, mastoid, parapharyngeal region, pterygopalatine and infratemporal fossa; orbital; and non-PM / non-orbital site, encompassing the neck, face, oral cavity, cheek, external ear, scalp and larynx [7]. PM subsite is the most common presentation and is associated with the least favorable outcome compared to other locations [4-6, 8]. …”

“…ERMS is the most common variant, being associated with the most favorable outcome compared to other variants [6, 8]. ERMS occurs in younger patients and shows a morphologic resemblance to fetal skeletal muscle.…”

“…Although no genetic abnormality prevails, small subsets of ERMS harbor LOH at 11p15, as well as FGFR4 , P53 , BCOR , ARID1A and RAS mutations, as shown in recent large genomic studies [10-13]. ARMS is the second most common variant, being associated with a poor prognosis [6, 8]. ARMS has a predilection for older children and young adults, having a histologic appearance of undifferentiated small blue round cells, arranged in a variable alveolar or solid pattern.…”

“…The 5-year overall survival rate of childhood RMS has risen to 62% and the 5-year relative survival rate of HNRMS is estimated at 63% [6, 42]. The prognostic factors in HNRMS have been multifactorial, such as age, tumor site, overall stage, distant metastases, histologic variant, nodal status and status of primary surgical site [6, 8, 43-46]. The aim of this study focused on a detailed clinicopathologic analysis, assessing the oncologic outcome and prognostic factors of HNRMS patients managed at our institution.…”

Age-based disparities in treatment and outcomes of retroperitoneal rhabdomyosarcoma

“…Furthermore, it is certainly impossible to fully comprehend the complexities of individual treatment decisions when looking at patients in aggregate. However, given that 46 % of our elderly cohort were deemed healthy enough to receive local therapy (surgery and/or radiation), as well as the substantial potential for improved survival given the poor prognosis of undertreated RMS, and the fact that multiple studies have shown chemotherapy to be a mainstay of treatment for RMS, it is likely that chemotherapy is under-utilized in the EP group [5,[9][10][11]. This is also particularly disturbing when >71 % of YPs received chemotherapy.…”