medicina Case Report Sarcomas of the Larynx: One Institution’s Experience and Treatment Protocol Analyses Jaromir Astl 1,2,3, Richard Holy 1,2,* , Inna Tuckova 4, Tomas Belsan 5, Miloslav Pala 6,7 and Jan Rotnagl 1,2 1 Department of Otorhinolaryngology and Maxillofacial Surgery, Military University Hospital, 16902 Prague, Czech Republic; [email protected] (J.A.); [email protected] (J.R.) 2 Third Faculty of Medicine, Charles University, 10000 Prague, Czech Republic 3 Department of Otolaryngology, Institute of Postgradual Medical Education, 10005, Prague, Czech Republic 4 Department of Pathology, Military University Hospital, 16902 Prague, Czech Republic; [email protected] 5 Department of Radiology, Military University Hospital, 16902 Prague, Czech Republic; [email protected] 6 Institute of Radiation Oncology, Bulovka University Hospital, 18081 Prague, Czech Republic; [email protected] 7 First Faculty of Medicine, Charles University, 12108 Prague, Czech Republic * Correspondence: [email protected] Abstract: Soft tissue sarcomas in the head and neck are rare malignancies. They occur in this area in less than 1% of all malignant tumors. Some authors have described the development of sarcoma from the mesenchymal tissue in the larynx. The histological diagnosis of a sarcoma depends on the immunohistochemical investigation. In particularly difficult diagnoses, electron microscopy has to be used. The treatment recommendation depends on the histological type of sarcoma. We analysed and summarized data on the diagnostic criteria and therapy for sarcoma of the larynx presented in the literature. We present three new cases of laryngeal sarcoma and describe the analyses of the published diagnostic and treatment schedules of laryngeal sarcomas. We developed a treatment Citation: Astl, J.; Holy, R.; Tuckova, protocol recommendation for laryngeal sarcoma based on an analysis of literature data and case I.; Belsan, T.; Pala, M.; Rotnagl, J. reports. This recommendation is based on histological type, staging, grading, size, and survival data. Sarcomas of the Larynx: One Institution’s Experience and Keywords: larynx; laryngoscopy; sarcoma; surgery; laryngectomy; treatment; surviving Treatment Protocol Analyses. Medicina 2021, 57, 192. https:// doi.org/10.3390/medicina57030192 1. Introduction Academic Editor: Eugenia Allegra Laryngeal carcinoma accounts for 95–98% of laryngeal malignant tumors. Primary sarcoma of the larynx is a rare disease (occurrence below 1% of all malignant tumors). [1,2]. Received: 19 January 2021 More than 50% sarcomas of larynx are fibrosarcomas, and chondrosarcomas are the next Accepted: 19 February 2021 Published: 25 February 2021 most frequent sarcomas in the larynx. Osteosarcomas, liposarcomas, undifferentiated pleomorphic sarcomas, synovial sarcomas, and rhabdomyosarcomas are very rare [2]. Publisher’s Note: MDPI stays neutral Laryngeal leiomyosarcoma is extremely rare [3]. Laryngeal osteosarcoma is certainly rarer with regard to jurisdictional claims in than leiomyosarcoma in the same localization [4]. A rare case of moderately differentiated published maps and institutional affil- squamous cell carcinoma (SCC) larynx associated with osteocartilaginous metaplasia of iations. the adjacent stroma similar to osteosarcoma is described in the literature [5]. The first two documented examples of laryngeal myofibroblastic sarcoma (low-grade form) in the world literature were described by MacGregor et al. in 2002 as a rare new subtype in the laryngeal area [6]. Low-grade myofibroblastic sarcoma (LGMS) is an atypical myofibroblastic tumor with Copyright: © 2021 by the authors. Licensee MDPI, Basel, Switzerland. fibromatosis-like features with preference predominantly in the head and neck area. LGMS This article is an open access article generally was described mostly as a malignant sarcoma (low-grade type) with a consider- distributed under the terms and able tendency to recur with low metastatic potential [7,8]. Only isolated cases of laryngeal conditions of the Creative Commons low-grade myofibroblastic sarcoma have been described in the world literature [9–11]. Attribution (CC BY) license (https:// Another very rare subtype of laryngeal sarcoma is undifferentiated epithelial sarcoma. This creativecommons.org/licenses/by/ rare type of sarcoma was described in the sphenoid sinus, maxillary sinuses, and once on 4.0/). the vocal fold. We have only seen some case reports about this laryngeal sarcoma. Medicina 2021, 57, 192. https://doi.org/10.3390/medicina57030192 https://www.mdpi.com/journal/medicina Medicina 2021, 57, 192 2 of 12 Myofibroblastic sarcomas (MFS) or myofibrosarcomas are very rare. They are identi- fied by the structure of the cells and are characterized by special immunohistochemical markers: desmin, vimentin, alpha-smooth muscle actin. The challenging histological diag- nosis of sarcoma depends on immunohistochemical investigation. In controversial cases, laryngeal sarcomas diagnosis has to be substantiated by electron microscopy [4]. There is a minimum of information in the literature regarding recommended thera- peutic protocols for laryngeal sarcoma. The new paper by de Juan Ferré et al. (2021) on clinical guideline of management of soft-tissue sarcoma [12] is certainly worth mentioning. In the present work, the diagnostic-therapeutic procedures were analyzed in a review of published cases of malignant sarcoma of the larynx. 2. Case Reports 2.1. Case 1 A 63-year-old man, smoker, presented to our clinic with complaints of shortness of breath and dysphonia that had been ongoing for 3 months. This patient was referred to the Otorhinolaryngology (ENT) outpatient department with edema of the right vocal cord with a history of hoarseness. He denied any alcohol consumption. A laryngeal examination revealed an exophytic mass in the right vocal cord. There was fixation of the right vocal cord and a tumor (size 12 mm × 15 mm × 12 mm) arising from the anterior commissure and ventral part of vocal cord. Laryngoscopy showed the tumor of the vocal cord, the mobility of the right vocal fold had reduced with excursion of the vocal cord by the tumor mass, but the mobility was Medicina 2021, 57, x FOR PEER REVIEW 3 of 13 2 mm more in the lateromedial direction. The vocal cord on the left side was completely functional. See Figure1. Figure 1. MyofibroblasticMyofibroblastic sarcoma MRI axial scan (a) and coronar scan of the larynx (b) T2 imaging with depression of fat signal: The tumor mass ( (T)T) on the right part of the larynx and anterior commissure without spread into thyroid cartilidge. WeDirect performed microlaryngoscopy a partial laryngectomy—extended was performed under generalchordectomy anesthesia with partial with a represen-resection oftative the laryngeal biopsy taken ventricle from (Morgagni’s the lesion. Thesinus), result partial of the resection histopathological of the perichondral examination layer wasof themyofibroblastic thyroid cartilage, sarcoma and ofa theresection larynxof (MFS). the anterior commissure 3 mm on the left vocal cord.We The performed resection was a partial done laryngectomy—extendedwith a CO2rLase (8 W), and chordectomy the section with of the partial margins resection of the resectionof the laryngeal was histologically ventricle (Morgagni’s tumor free sinus), (frozen partial section resection and full of investigation). the perichondral layer of Radical surgery was sufficient for the treatment. We followed‐up the patient, who has been without signs of recurrence or persistence of the disease for 36 months. 2.2. Case 2 A 67‐year‐old man presented to our ENT department with complaints of shortness of breath and dysphonia that had been ongoing for 2 months. This patient was referred to the ENT outpatient department with a growing tumor on the right side of the larynx. Histology of a biopsy sample verified a benign tumor. Because the biological behavior of the tumor was described as aggressive growth, a new biopsy was performed. The sample was analyzed, and histology showed a suspicious fibrosarcoma. He had not been smoking and denied alcohol consumption. Respiration was difficult. A laryngeal examination revealed an exophytic mass in the right vocal cord and an‐ terior commissure. The mobility of the right vocal cord was maintained, and there was a tumor of approximately 12 mm × 15 mm × 17 mm arising from the commissure and ventral part of the vocal cord. Direct microlaryngoscopy was performed under general anesthesia with a repre‐ sentative biopsy taken from the lesion. The result of the histopathological examination was myofibroblastic sarcoma of the larynx. The laryngoscopy showed the tumor of the vocal cord, the mobility of the right vocal fold was with reduced excursion of the vocal cord by the tumor mass, but they mobilit was more than 2 mm in the lateromedial direc‐ tion. The vocal cord on the left had complete function. Given the respiratory distress, an urgent tracheostomy with general anesthesia was performed. CT scans showed the transglottic location of the tumor mass being 40 mm × 22 mm × 16o mm int the base of the epiglottic cartilage. No infiltration to the cartilage was evident. Medicina 2021, 57, 192 3 of 12 the thyroid cartilage, and a resection of the anterior commissure 3 mm on the left vocal cord. The resection was done with a CO2 Laser (8 W), and the section of the margins of the resection was histologically tumor free (frozen section and full investigation). Radical surgery was sufficient for the treatment. We followed-up the patient, who has been without