Rhabdomyosarcoma: Nose Presenting As Second Primary with Carcinoma Tongue

Document Type : Case Report

Authors

1 Department of Otolaryngology and Head and Neck Surgery, Lady Hardinge Medical College, New Delhi (India)-110001.

2 Department of Pathology, Lady Hardinge Medical College, New Delhi (India)-110001.

Abstract

Introduction
Presence of two primary malignancies is rare and occurs in 3-5% of the cancer patients. As per our extensive internet research, this is the only reported case of a synchronous sino-nasal embryonal rhabdomyosarcoma with squamous cell carcinoma-tongue. The case report is important because of the rare diagnosis and the challenge we faced in the diagnosis and treatment of the patient because of the paucity of literature available on management adult rhabdomyosarcoma.
 
Case Report:
We present a very rare case of an adult male with a sino-nasal mass diagnosed to be an embryonal type rhabdomyosarcoma. The patient also had a moderately differentiated squamous cell carcinoma-tongue for the past 8 months. Radiological investigations were done to see the extent of the sino-nasal mass and the extent of tongue lesion, which was seen to be involving the base of the tongue. The patient was referred for chemoradiotherapy but succumbed to the disease after 2 weeks of treatment.
 
Conclusion
Occurrence of rhabdomyosarcoma in synchronous malignancies is extremely rare as the most common first as well as second primary malignancy in a diagnosed case of head and neck cancer is squamous cell carcinoma. A multidisciplinary approach to the treatment of adult rhabdomyosarcoma has been recommended. The combined use of chemoradiotherapy and surgery has improved treatment in the recent past but RMS in adults is still a rare head and neck tumour that carries a poor prognosis despite aggressive therapy.

Keywords


  1. Heroiu Cataloiu AD, Danciu CE, Popescu CR. Multiple cancers of the head and neck. Maedica (Buchar). 2013;8(1):80–5.
  2. Morris LG, Sikora AG, Patel SG, Hayes RB, Ganly I. Second primary cancers after an index head and neck cancer: subsite-specific trends in the era of human papillomavirus-associated oropharyngeal cancer. J Clin Oncol. 2010;29(6):739–46.
  3. Gallo O, Sardi I, Pepe G, Franchi AAttanasio MGiusti B, et al. Multiple Primary Tumors of the Upper Aero-digestive Tract: Is There a Role for Constitutional Mutations in the p53 Gene? Int J Cancer 1999; 82: 180-6.
  4. Gale N, Zidar N. Benign and Potentially Malignant Lesions of the Squamous Epithelium and Squamous Cell Carcinoma, In. Cardesa A, Slootweg PJ. Pathology of the Head and Neck, Springer, Germany, 2006: 1-38.
  5. Chang S, Ha P – Biology of Head and Neck, In: Cummings Otolaryngology, Head and Neck Surgery, Fifth Edition, Volume One, Mosby Elsevier, 2010:1015-29.
  6. Ganly I, Ibrahimpasic T, Patel SG, Shah JP. Tumors of the Oral Cavity, In: Montgomery PQ, Rhys Evans PH, Gullane PJ. Principles and Practice of Head and Neck Surgery and Oncology, Second Edition, United Kingdom: Informa Healthcare, 2009:160-91.
  7. Barnes L, Eveson JW, Reichart P, Sidransky D. Pathology and Genetics of Head and Neck Tumors, Lyon: IARC Press, 2005.
  8. Antanova NA, Dormak VV. Rhabdomyosarcoma and squamous cell carcinoma of the larynx. Vestn Otorinolaringol. 1978; 6:69-71.
  9. Goldman RL, Weidner N. Pure squamous cell carcinoma of the larynx with cervical nodal metastasis showing rhabdomyosarcomatous differentiation. The American Journal of Surgical Pathology. 1993; 17(4):415-21.
  10. Wurm J, Constantinidis J, Grabenbauer GG, Iro H. Rhabdomyosarcomas of the nose and paranasal sinuses: treatment results in 15 cases. 
    Otolaryngol Head Neck Surg.2005 Jul; 133(1):
    42-50.
  11. Thompson CF, Kim BJ, Lai C, Grogan T, Elashoff D, St John MA, Wang MB. Sinonasal rhabdomyosarcoma: prognostic factors and treatment outcomes. Int Forum Allergy Rhinol. 2013 Aug; 3(8):678-83.
  12. Hawkins WG, Hoos A, Antonescu CR, Urist M J, Leung DH, Gold JS. et al. Clinicopathologic analysis of patients with adult rhabdomyosarcoma. Cancer, 91(4), 794–803.