Annals of African Medicine
Home About AAM Editorial board Ahead of print Current Issue Archives Instructions Subscribe Contact us Search Login 


 
RESEARCH ARTICLE
Year : 2007  |  Volume : 6  |  Issue : 4  |  Page : 190-193 Table of Contents     

Adult laryngeal rhabdomyosarcoma: Report of a case and literature review


1 Department of Otolaryngology and Head and Neck Surgery, Hull Royal Infirmary, Hull HU3 2JZ, United Kingdom
2 Department of Pathology, Hull Royal Infirmary, Hull HU3 2JZ, United Kingdom

Date of Web Publication29-Sep-2009

Correspondence Address:
A Shayah
58 Whinmoor Court, Leeds LS14 1NX
United Kingdom
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1596-3519.55698

Rights and Permissions
   Abstract 

Rhabdomyosarcoma is relatively seen in the pediatric age group with the head and neck region as the commonest site. To the best of our knowledge, few cases of laryngeal involvement in adult have been described in the literature. Biologically, rhabdomyosarcoma is different from squamous cell carcinoma, which is the commonest tumor of the larynx. A previously healthy non-smoker 77-year-old lady presented to the ENT outpatient with a six weeks history of intermittent alteration of voice quality. She had no history of sore throat, or any symptoms suggesting laryngo-pharyngeal reflux. Examination showed asymmetry of the left arytenoid cartilage and aryepiglottic fold. She subsequently had a direct laryngoscopy and biopsy. Histology and immunohistochemistry examination suggested the diagnosis of mesenchymal neoplasm. Following discussion at MDT she subsequently had a total laryngectomy. Histology confirmed a completely excised laryngeal rhabdomyosarcoma. Rhabdomyosarcoma of larynx in adult is a rare disease. Surgical treatment with or without adjuvant radiotherapy is currently the treatment of choice for this disease.

   Abstract in French 

Le Rhabdomyosarcome est relativement connu dans le groupe d'âge pédiatrique et les régions les plus touchées sont la tête et le cou. A notre connaissance, peu de cas de l'implication du larynx chez des adultes ont été cités dans la littérature. Biologiquement, rhabdomyosarcome est différent de la cellule squameuse carcinome, qui est la tumeur de larynx la plus ordinaire. Une femme non-fumeuse, âgée de 77 ans d'une très bonne santé au départ, s'est présentée à l'ORL ambulatoire avec une période de six semaines histoires de l'altération répétée de la qualité de la voix. Elle n'avait pas d'antécédente de maux de gorge ou aucun symptômes suggestifs le reflux laryngo-pharyngien. L'examen a montré l'asymétrie de la gauche aryténoïde cartilage aryepiglottique pli. Elle a par la suite eu une laryngoscopie directe et la biopsie. L'examen histologique et immunohistochimie a suggéré le diagnostique des tumeurs mesenchymteuses, néoplasme. A la suite d'une discussion au MDT elle a ensuite eu une laryngectomie totale. L'histologie a confirmé une excisée larynx chez des adultes qui est une maladie rare. Le traitement chirurgical avec ou sans radiothérapie adjuvante est actuellement le traitement du choix pour cette maladie.

Keywords: Rhabdomyosarcoma, larynx cancer


How to cite this article:
Shayah A, Agada F O, Karsai L, Stafford N. Adult laryngeal rhabdomyosarcoma: Report of a case and literature review. Ann Afr Med 2007;6:190-3

How to cite this URL:
Shayah A, Agada F O, Karsai L, Stafford N. Adult laryngeal rhabdomyosarcoma: Report of a case and literature review. Ann Afr Med [serial online] 2007 [cited 2018 May 27];6:190-3. Available from: http://www.annalsafrmed.org/text.asp?2007/6/4/190/55698


   Introduction Top


Laryngeal cancer is an important cause of morbidity and mortality accounting to 25% of all head and neck cancers.1[] The vast majority of laryngeal cancers are squamous cell carcinoma. Rhabdomyosarcoma is a malignant tumor of striated muscle rarely occurs in the larynx. In adults, rhabdomyosarcomas are extremely rare tumors, with few cases of these tumors have been adequately described in world literature in the adult population.[2],[3],[4]

There is very scanty literature on the presentation and behavior of laryngeal rhabdomyosarcoma. However because it is the most common malignant mesenchymal tumor in children,[5] pediatric literature indicates that the most common site of presentation is in the head and neck region (35%). Lungs, bone marrow, bones, liver and brain are among the most common sites of metastases.[6]

Five years survival ranges from 30% in patients with metastasis at presentation to 80% in those where the disease is localized and complete resection is performed.[4] It is unclear if rhabdomyosarcoma in larynx behaves similarly to those histologically different tumors occurring in other sites. Biologically, rhabdomyosarcoma is quite different from the more common cancer in larynx, which is squamous cell carcinoma. The diagnostic difficulties, immunohistochemistry and long term outcome of the various treatment modalities for adult primary laryngeal rhabdomyosarcoma are discussed.

Case report

A previously healthy non-smoker 77-year-old lady presented to the ENT outpatient with a six weeks history of intermittent alteration of voice quality. She had no history of sore throat or feeling of lump in throat, and had no symptoms suggesting laryngo-pharyngeal reflux.

Initial examination with a flexible laryngoscope showed asymmetry of the left arytenoid cartilage and aryepiglottic fold. She subsequently had a direct laryngoscopy were an irregular swelling of the left arytenoid cartilage and left aryepiglottic fold was found. The tumor extended into the region of posterior comissure and adjacent cricoid cartilage. However the piriform fossa, vocal cords, epiglottis, valleculla, appeared normal. Biopsy was taken from the left arytenoids and aryepiglottic fold.

CT scan of the larynx shows a bulky tumor involving the left arytenoid and post-cricoid cartilage. There was no nodal involvement and no evidence of metastasis on chest scan.

The diagnosis of a mesenchymal neoplasm was made and following discussion at multidisciplinary team meeting (MDT), she subsequently had a total laryngectomy.[Figure 1] and [Figure 2] On immunohistochemistry the cells, demonstrated very bizarre staining for desmin and were strongly positive for vimentin, myoglobin and MyoD1.[Figure 3],[Figure 4] and [Figure 5] However they were negative for cytokeratin, EMA, CEA, S-100 protein and actin.There was no invasion of the underlying cartilage and the tumor was completely excised with a thin margin of about 0.5 mm. A diagnosis of completely excised laryngeal pleomorphic rhabdomyosarcoma was confirmed. She is been regularly followed up at the MDT Clinic, and she had no signs of local or regional recurrence over a year follow up.


   Discussion Top


Rhabdomyosarcomas are mesenchymal tumors usually seen in children.[4] Though in children it is reported to occur commonly in the head and neck region, the larynx is a very rare site even in this group.[6] The literature on laryngeal Rhabdomyosarcomas indicates that in the larynx the tumor tends to originate in the vocal cord region and appear as polypoid mass, which may extend above and below the vocal cord.[7]

Presentation can vary from alteration in voice to a feeling of strange lump sensation in throat, or on rare occasions it may be life threatening because of upper airway obstruction.[7] It is important to note that it's presentation does not differ from the common presentations of squamous cell carcinoma, however they tend to grow rapidly and present with a short history of onset of symptoms.[8] The challenge is to differentiate clinically benign from malignant lesions, when they present as a polypoid tumor. [7] It is not clear if smoking plays any role in rhadomyosarcoma of the larynx. Our patient was a non- smoker. To the best of our knowledge, there is no association between smoking and rhabdomyosarcoma. Diagnosis is made from biopsy on direct laryngoscopy. CT scan is important in determining the extent of tumor.

There are four main types of rhabdomyosarcoma. These are embryonal, alveolar, pleomorphic and botryoid. It is important to note that the pleomorphic type is uncommon in children and is rare, even in adults.[9]

Cross-striations are usually visible with ordinary hematoxylin-eosin staining accentuated by phosphotungstic acid- hematoxylin (PTAH) staining. Histologically tumor composed of sheets of anaplastic cells, several of the cells shows striking nuclear pleomorphism, with very bizarre nuclear features. Many of the cells are multinucleated with prominent eosinophilic cytoplasm. Tadpole-shaped and strap-- shaped cells are commonly present[Figure 4].[9]

It is important to note that immunohistochemistry plays a key role in diagnosis. The tumor cells are strongly positive for desmin, vimentin, myoglobin and MyoD1, but negative for cytokeratine, epithelia membrane antigen, CEA, S-100 protein, and actin. MyoD1 RNA protein is highly sensitive and specific for rhabdomyosarcoma. [10],[11],[12] Electron microscopic examination may be useful especially in uncertain cases.[7]

Treatment and prognosis is based on results of the Intergroup Rhabdomyosarcoma study group (IRS).[13],[14] Surgical treatment with adjuvant radiotherapy is currently the treatment of choice for laryngeal disease.[2] However the use of adjuvant combination Chemotherapy with radiotherapy is gaining ground and reducing the need for mutilating surgery in head and neck rhabdomyosarcoma[15],[16] ; however it is not clear if this modality of treatment has any significant effect on outcome in adult laryngeal disease considering that only few cases has been reported. Improvement in outcome has been shown to occur with multimodality treatment regimens for head and neck rhabdomyosarcoma.[17]

Conclusion

Rhabdomyosarcoma should be considered in any patient with a laryngeal polypoidal lesion and presenting with a very short history.



 
   References Top

1.Shaha AR, Patel S, Shaha D, Harrison LB. Head and neck cancer. In: Lenhard RE, Osteen RT, Gansler T (Eds). Clinical oncology. American Cancer Society Inc, Atlanta, 2001;297-327.   Back to cited text no. 1      
2.Ruske DR, Glassford N, Costello S, Stewart IA. Laryngeal rhabdomyosarcoma in adults.J Laryngol Otol. 1998;112:670-672.   Back to cited text no. 2      
3.Libera DD, Falconieri G, Zanella M. Embryonal "Botryoid" rhabdomyosarcoma of the larynx: a clinicopathologic and immunohistochemical study of two cases.Ann Diagn Pathol. 1999;3:341-349.   Back to cited text no. 3      
4.Abali H, Aksoy S, Sungur A, Yalçin A. Laryngeal involvement of rhabdomyosarcoma in an adult.World J Surg Oncol. 2003;1:17.   Back to cited text no. 4      
5.Barista I, Tekuzman G, Yalcin S, et al. Treatment of advanced soft tissue sarcomas with ifosfamide and doxorubicin combination chemotherapy.J Surg Oncol. 2000;73:12-16.   Back to cited text no. 5      
6.Marcus KC. Pediatric solid tumors. In: In: Lenhard RE, Osteen RT, Gansler T (Eds). Clinical oncology. American Cancer Society Inc, Atlanta, 2001; 577-609.   Back to cited text no. 6      
7.Robin PE, Olofsson J. Tumours of the larynx. In: Hibbert J (Ed). Scott-Brown's otolargngology. Butterworth-Heinemann, Edinburgh, 1997;11/7.   Back to cited text no. 7      
8.Newman AN, Rice DH. Rhabdomyosarcoma of the head and neck.Laryngoscope. 1984;94:234-276.   Back to cited text no. 8      
9.Kodet R, Newton WAJr, Hamoudi AB, Asmar L, Jacobs DL, Maurer HM. Childhood rhabdomyosarcoma with anaplastic (pleomorphic) features. A report of the Intergroup Rhabdomyosarcoma Study.Am J Surg Pathol. 1993;17:443-453.   Back to cited text no. 9      
10.Dias P, Parham DM, Shapiro DN, Webber BL, Houghton PJ. Myogenic regulatory protein (MyoD1) expression in childhood solid tumors: diagnostic utility in rhabdomyosarcoma.Am J Pathol. 1990;137:1283-1291.   Back to cited text no. 10      
11.Cessna MH, Zhou H, Perkins SL, et al. Are myogenin and myoD1 expression specific for rhabdomyosarcoma? A study of 150 cases, with emphasis on spindle cell mimics.Am J Surg Pathol. 2001;25:1150-1157.   Back to cited text no. 11      
12.Gaffney EF, Dervan PA, Fletcher CD. Pleomorphic rhabdomyosarcoma in adulthood. Analysis of 11 cases with definition of diagnosticcriteria.Am J Surg Pathol. 1993;17:601-609.   Back to cited text no. 12      
13.Wolden SL, Anderson JR, Crist WM et al. Indications for radiotherapy and chemotherapy after complete resection in rhabdomyosarcoma: A report from the Intergroup Rhabdomyosarcoma Studies I to III.J Clin Oncol. 1999;17:3468-3475.   Back to cited text no. 13      
14.Breneman JC, Wiener ES. Issues in the local control of rhabdomyosarcoma.Med Pediatr Oncol. 2000;35:104-109.   Back to cited text no. 14      
15.Raney RB, Asmar L, Vassilopoulou-Sellin R, et al. Late complications of therapy in 213 children with localized, nonorbital soft-tissue sarcoma of the head and neck: A descriptive report from the Intergroup Rhabdomyosarcoma Studies (IRS)-II and - III. IRS Group of the Children's Cancer Group and the Pediatric Oncology Group.Med Pediatr Oncol. 1999;33:362.   Back to cited text no. 15      
16.Ruymann FB, Grovas AC. Progress in the diagnosis and treatment of rhabdomyosarcoma and related soft tissue sarcomas.Cancer Invest. 2000;18:223-241.   Back to cited text no. 16      
17.Simon JH, PaulinoAC, Smith RB, Buatti JM. Prognostic factors in head and neck rhabdomyosarcoma.Head Neck. 2002;24:468-473.  Back to cited text no. 17      


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


This article has been cited by
1 Pleomorphic Rhabdomyosarcoma Arising from True Vocal Fold of Larynx: Report of a Rare Case and Literature Review
Sevdegül Mungan,Selçuk Arslan,Eda Küçüktülü,Safak Ersöz,Bengü Çobanoglu
Case Reports in Otolaryngology. 2016; 2016: 1
[Pubmed] | [DOI]
2 Laryngeal embryonal rhabdomyosarcoma in an adult - A case presentation in the eyes of geneticists and clinicians
Wojciech Kukwa, Piotr Wojtowicz, Beata Jagielska, Grzegorz Sobczyk, Andrzej Kukwa, Anna M Czarnecka
BMC Cancer. 2011; 11(1): 166
[VIEW] | [DOI]
3 Nowotwory złośliwe krtani pochodzenia mezenchymalnego w materiale własnym
Żyłka Stanisław, Zawadzki Marcin, Bień Stanisław, Okła Sławomir
Otolaryngologia Polska. 2011; 65(2): 97
[VIEW] | [DOI]
4 Low-grade myofibroblastic sarcoma of the larynx
Covello, R. and Licci, S. and Pichi, B. and Spriano, G. and Vidiri, A. and Morelli, L. and Rosenberg, A.E.
International Journal of Surgical Pathology. 2011; 19(6): 822-826
[Pubmed]
5 A rhabdomyosarcoma arising in the larynx of a dog
Yamate, J. and Murai, F. and Izawa, T. and Akiyoshi, H. and Shimizu, J. and Ohashi, F. and Kuwamura, M.
Journal of Toxicologic Pathology. 2011; 24(3): 179-182
[Pubmed]
6 The presentation of laryngeal malignant tumors of mesenchymal origin [Nowotwory złośliwe krtani pochodzenia mezenchymalnego w materiale własnym]
Stanisław, Z. and Marcin, Z. and Stanisław, B. and Sławomir, O.
Otolaryngologia Polska. 2011; 65(2): 97-102
[Pubmed]
7 An unusual cause of hoarseness: Rhabdomyosarcoma of the larynx
Vira, D., Nguyen, C., Mowry, S.
Laryngoscope. 2011; 121(sup ): 4-182
[Pubmed]
8 Pathology quiz case 1
Hsia, J.C., Schmitt, N.C., Hoch, B., Moe, K.S.
Archives of Otolaryngology - Head and Neck Surgery. 2011; 137(6): 635-638
[Pubmed]
9 Rhabdomyosarcoma: A rare laryngeal neoplastic entity [Il rabdomiosarcoma: Una rara entità neoplastica laringea]
Pittore, B. and Fancello, G. and Rocca, P.C. and Ledda, G.P. and Tore, G.
Acta Otorhinolaryngologica Italica. 2010; 30(1): 52-57
[Pubmed]
10 Rhabdomyosarcoma: A rare laryngeal neoplastic entity | [Il rabdomiosarcoma: Una rara entità neoplastica laringea]
Pittore, B., Fancello, G., Rocca, P.C., Ledda, G.P., Tore, G.
Acta Otorhinolaryngologica Italica. 2010; 3(1): 52-57
[Pubmed]
11 Laryngeal alveolar rhabdomyosarcoma involving the true vocal fold in an adult: Case report
Leventhal, D.D., Spiegel, J., Keane, W.
Ear, Nose and Throat Journal. 2010; 89(12): E8
[Pubmed]
12 Nonsquamous carcinomas of the larynx -15 years experience in the single institution [Neepidermoidni tumori larinksa - 15-godišnje iskustvo u našoj ustanovi]
Bilić, M. and Prgomet, D. and Kovač, L. and Topić, I. and Katić, V.
Lijecnicki Vjesnik. 2009; 131(7-8): 196-198
[Pubmed]



 

Top
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
    Abstract
    Introduction
    Discussion
    References
    Article Figures

 Article Access Statistics
    Viewed2399    
    Printed143    
    Emailed1    
    PDF Downloaded105    
    Comments [Add]    
    Cited by others 12    

Recommend this journal