Soft tissue sarcomas of the head and neck are rare, constituting less than 10% of all forms of soft tissue sarcomas and less than 1% of all neoplasm infecting this particular region. Leiomyosarcoma is a malignant tumor originating from smooth muscle cells. This tumor accounts for only 4%of the head and neck sarcomas. Laryngeal leiomyosarcoma is very rare, accounting for less than 1% of all malignant laryngeal tumours. Only less than 50 cases of the laryngeal leomyosarcomas are reported in medical literature. In this paper, we are going to present two new cases with pure laryngeal Leiomyosarcoma. The Leiomyosarcoma of larynx is a rare malignant tumor. The diagnosis is purely histological. With the advenced immunohistochemical techniques now available, the diagnosis of leiomyosarcomas of the larynx can be picked out from the list of differential diagnosis of spindle cell tumors of the larynx. Treatment is not yet codified, however radical surgery remains the treatment of choice.
Soft tissue sarcomas of the head and neck are rare, constituting less than 10% of all forms of soft tissue sarcomas and less than 1% of all neoplasm infecting this particular region. Leiomyosarcoma is a malignant tumor originating from smooth muscle cells. This tumor accounts for only 4%of the head and neck sarcomas. Laryngeal leiomyosarcoma is very rare, accounting for less than 1% of all malignant laryngeal tumours. Only less than 50 cases of the laryngeal leomyosarcomas are reported in medical literature. In this paper, we are going to present two new cases with pure laryngeal Leiomyosarcoma.
A 23 year-old male, with history of long stay in the ICU following a highway accident in 2000, no history of smoking or alcohol was reported.
In 2009, he consults for acute laryngeal dyspnea requiring tracheostomy. Direct laryngoscopy under general anaesthesia showed a subglottic fibrous process. The patient underwent tracheal dilatation followed by weaning and successful decannulation
Five months after, he presented again with laryngeal dyspnea for which he had emergent tracheostomy. CT objectified extended process to a 3-storey larynx invading the arytenoid, and pharyngolaryngeal left wall, with fixity of the left vocal cord. A biopsy was performed, concluding the diagnosis of leiomyosarcoma. The staging was negative.
Macroscopic examination of the surgical specimen objectified an endoluminal laryngeal tumour of 10 cm, invading the left vocal cord and extending to the 3 levels of glottis, fixed to the posterior well.
Microscopically, we identified a normal squamous epithelium with an underlying spindle shaped tumour cells arranged in fascicles and a storiform pattern. There were eosinophilic spindle cells with moderate nuclear pleomorphism, with a variable mitotic rate 6 mitosis/10 HPF. There were areas for necrosis (less than 50%).
The surgical resection limits were free from tumour.There were no lymph node metastasis.
At immunohistochemistry, tumour cells stained for Hcaldesmone and SMA, but not for desmin and cytokeratin AE1/AE3. So the diagnosis of Leiomyosarcoma gade II FNCLCC was confirmed. The tumour was classified as pT4N0Mo.
An adjuvant radiotherapy was given to sterilize the tumour area.
A 55 year old patient, without particular medical history, was admitted to the Head and Neck department for dysphonia lasting for 6 months. Physical examination was unremarkable including absence of cervical lymphadenopathy. Indirect laryngoscopy reveals an exophytic tumor invading both the supra and subglottic levels of larynx. Computed tomography (CT) demonstrated laryngeal tumour of 4 cm extending to supra and subglottis with invasion of aryepiglottic fold and pre-epiglottic Ioge (Figure 1). Tumor biopsy was performed. It showed a partially necrotic spindle cell proliferation arranged in short bundles and storiform pattern.. It was made of cells with a cigar like fusiform nucleus. Nuclei were irregular, hyperchromatic, nucleolus showing many abnormal mitosis (8 mitoses/10 fields magnification) (Figure 2a-d). The cytoplasm was abundant and eosinophilic. Given these histological findings, immunohistochemistry was performed by the biotin streptavidin peroxidase method using the anti-actin smooth muscle antibodies, anti-desmin anti-PS100, anti-cytokeratin and anti-CD68. Tumor cells intensely expressed smooth muscle actin (Figure 2e) and focally desmin (Figure 2-f). Other antibodies were not expressed by tumor cells including the antibody anti-EMA . In view of these data, we used the diagnosis of laryngeal leiomyosarcoma. The evolution was marked by the occurrence of severe dyspnea requiring emergency tracheostomy. Our patient was referred to a specialized center where radiotherapy was conducted and completed by a total laryngectomy. The staging with chest radiography and abdominal ultrasound revealed no metastasis. The post-surgical outcome was good with a decline of 6 months.
Sarcomas of the head and neck region are rare. They represent less than 10% of soft tissue sarcoma and less than 1% of cancers in this region . The majority of leiomyosarcoma of the head and neck occur in the oral cavity, the hair cuff and sinus [5, 7]. The laryngeal Iocalisation of leiomyosarcoma was originally described by Frank in 1941 . Since then, less than 50 cases have been reported in the literature .
The clinical presentation of this tumour, involving the larynx, is similar to that of other mass lesions or tumors with airway compromise or interference of laryngeal function such as dysphonia and dyspnea [2-6]. The symptoms can sometimes be serious necessitating a tracheotomy as our two patients.
Direct laryngoscopy and CT locate the tumour and appreciate its extension into supra and subglottis and to the adjacent structures. Tumors are located in over than 50% of cases at right hemilarynx and instead of interested the glottis [3, 7, 8].
At histological examination,Leiomyosarcoma of the larynx is similar to leiomyosarcomas in soft tissues and demonstrate increased cellularity, nuclear and cellular pleomorphism, cytoplasmic vacuolization, necrosis, haemorrhage, and increased mitotic activity in addition to invasive growth . It shows as fascicles of spindle cells with elongated nuclei; they are distinguished from leiomyomas by characteristic malignant properties; nuclear mitotic activity and hyperchromasia and cellular pleomorphism [9-11] The differential diagnosis of this rare tumour arises with other spindle cells tumours, including fibrosarcoma, myofibrosarcome, rhabdomyosarcoma, melanoma, carcinosarcoma eliminated to the absence of an epithelial component .
During the last few years, specific immunohistochemical stains, in particular smooth muscle actin, desmin and h-caldesmon are positive in a great majority leiomyosarcomas. However, none of these is absolutely specific for smooth muscle, and positivity for two of these markers is more supportive of leiomyosarcoma than positivity for one alone [8, 11]. Other markers include EMA (epithelial membran antigen), myogenin, and PS 100 are usually negative thus removing the sarcomatoid carcinoma, rhabdomyosarcome and melanoma [1, 7, 8, 11].
Given the scarcity of cases reported in the literature, no therapeutic protocol is currently established. Most authors, however, advocate a total or partial surgical removal of the larynx. An association of postoperative radiotherapy, performed in some patients can also have a role in recurrence or in residual disease, but as a primary treatment modality, radiotherapy is less effective . Chemotherapy has also a limited role in leiomyosarcomas of the larynx .
Metastasis are not frequent. It spreads through blood to most often in the lungs, bones and brain [2, 6], which determines the prognosis. Literature search has shown that head and neck leiomyosarcomas generally have a survival of 35-50 % at 5 years  in absence of metastasis.
The Leiomyosarcoma of larynx is a rare malignant tumor. The diagnosis is purely histological. With the advenced immunohistochemical techniques now available, the diagnosis of leiomyosarcomas of the larynx can be picked out from the list of differential diagnosis of spindle cell tumors of the larynx. Treatment is not yet codified, however radical surgery remains the treatment of choice.
The authors declare that they have no competing interests.
All authors read and approved the final manuscript.
This article is edited for English by Mr Youssef Lakroudi.
- Jagveer Yadav, Jaimanti Bakshi, Head and Neck Leiomyosarcoma, Indian J Otolaryngol Head Neck Surg (July 2013) 65(Suppl 1):S1–S5. Available from: dx.doi.org/10.1007/s12070-011-0305-8
- W. AbID, y. yASSINE, S. MEZRI, LEOMYOSARCOME DU LARYNX; J. tun ORL - n° 26 JuIn-DÉCEMBRE 2011.
- Ashok Gupta Parvinderjit Singh Kohli Leiomyosarcoma of larynx A case report Indian J Otolaryngol Head Neck Surg October December 2007.
- Khadivi E, Taziky M, Jafarian A, Nasseri Sadr M. Laryngeal leiomyosarcoma, a case report and review of articles. Iran J Otorhinolaryngol. 2013;25:253-8 pubmed
- Rimmer J, Giddings C, Mady S. Leiomyosarcoma of the larynx. J Laryngol Otol. 2006;120:e3 pubmed
- Abbas A, Ikram M, Yaqoob N. Leiomyosarcoma of the larynx: A case report. Ear Nose Throat J. 2005;84:435-6, 440 pubmed
- Marioni G, Bertino G, Mariuzzi L, Bergamin-Bracale A, Lombardo M, Beltrami C. Laryngeal leiomyosarcoma. J Laryngol Otol. 2000;114:398-401 pubmed
- Marioni G, Staffieri C, Marino F, Staffieri A. Leiomyosarcoma of the larynx: critical analysis of the diagnostic role played by immunohistochemistry. Am J Otolaryngol. 2005;26:201-6 pubmed
- Roxana c, Helmberger, Leiomyosarcoma of the larynx presenting as a laryngopyocele, AJNR 17:1112–1114, Jun 1996 0195-6108/96/1706–1112 q American Society of Neuroradiology.
- Freedman A, Reiman H, Woods J. Soft-tissue sarcomas of the head and neck. Am J Surg. 1989;158:367-72 pubmed
- World Health Organization Classification of Tumours: Pathology and Genetics of Head and Neck Tumours.
- Cocks H, Quraishi M, Morgan D, Bradley P. Leiomyosarcoma of the larynx. Otolaryngol Head Neck Surg. 1999;121:643-6 pubmed