Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature. Non-Orbital Rhabdomyosarcoma of the Head and Neck Although not limited to the pediatric population, rhabdomyosarcomas are predominantly found in children. In fact, they are the most common soft tissue sarcomas in the pediatric population and the second most common solid tumor in childhood. Population incidence figures vary from about 1.3 per million in blacks to 4.6 per million in whites. The peak age of incidence is five years, with 75% of these lesions diagnosed by age 12. Males are more frequently affected than females. Of all areas in the body, the head and neck region is the most frequent site of presentation for these tumors. Orbital rhabdomyosarcomas (RMS) are more prevalent than other head and neck sites with soft tissue head and neck tumors second and pharyngeal rhabdomyosarcomas third. Many authors consider these malignancies to have a genetic etiology. Several autopsy cases have documented coexistent developmental anomalies in patients with RMS (approximately 30%) and family members of affected patients have a higher than normal prevalence of other malignancies. Furthermore, many patients with RMS have been found to have a chromosomal translocation involving the second and thirteenth chromosomes which suggests a causative genetic abnormality leading to the development of this lesion. Their most common mode of presentation is that of a painless mass that usually causes anatomic obstruction of the involved area. Patients may complain of common symptoms such as rhinorrhea, nasal obstruction, hoarseness, pain or facial swelling. Patients suspected of having RMS should be biopsied after a thorough head and neck exam. Radiologic imaging consisting of computed tomography and magnetic resonance imaging (MRI) studies are very helpful in elucidating the extent of disease. As experience with MRI grows, it is emerging as an important tool for following patients who have undergone treatment. Metastatic workup consists of chest C.T., bone scan, bone marrow biopsy and (when indicated) lumbar puncture for parameningeal tumor sites. Prior to 1950, rhabdomyosarcomas were diagnosed only when a classical pattern was observed under the light microscope. Striated tumor cells with a spindled appearance were pathogenic of the disease whereas those tumors with less distinct histology were classified as mesenchymal tumors of uncertain histogenesis. During the 1950's, rhabdomyosarcomas were recognized when a malignant tumor had the appearance of embryonal (i.e. developing) skeletal muscle and several patterns were named. The most common of these, embryonal RMS, make up about 75% of all head and neck RMS. The next most common pattern, alveolar RMS, comprises 20% of head and neck rhabdomyosarcomas. More recent technologic developments - immunohistochemistry and electron microscopy - have helped confirm many "indeterminate" lesions as RMS and now represent the standard of histopathologic diagnosis. These tumors are staged according to a combined clinical and surgical system that takes into account the site, resectability, and spread of the lesion. It is therefore different for the accepted TMN staging system used in most other head and neck malignancies. Optimal treatment of RMS involves a multimodal approach - surgical, radiotherapeutic and chemotherapeutic measures may all play a role depending on the site and size of the tumor. Radiation therapy and chemotherapy are necessary in nearly all cases, whereas surgical management has remained controversial. Most recently, there has been renewed interest in surgical resection of the tumor mass in an effort to improve local control of disease. Craniofacial techniques and base of skull approaches are growing in importance as potential methods of disease management. Radiotherapy is administered to a total dose of 4000-6000 cGy depending on tumor size, patient age and site ("parameningeal" sites such as the temporal bone, nasopharynx and paranasal sinuses are treated with whole brain radiation). The most commonly employed chemotherapeutic agents are vincristine, actinomycin-D and cytoxan given in a "pulse" schedule of administration. Newer antineoplastic agents are being developed and tested at the present time. Prognosis depends on stage, site, and several other factors. Stage I and II lesions have an 80-90% two year survival rate. Stage III tumors carry a 50-60% two year survival rate (orbital tumors are best with an 80-90% rate). Patients with Stage IV lesions have a 10-20% two year survival rate. The Stage III and Stage IV figures are worse for non-orbital head and neck sites than for other body sites. Other negative prognostic factors include extensive bone destruction, parameningeal involvement, metastatic disease at presentation, unfavorable histology (alveolar or undifferentiated) and older patient age. Patients must be followed for a long period of time not only to detect recurrent or persistent disease but also to monitor for the late effects of radiation and chemotherapy given during childhood. Case Presentation A six and one-half year old girl was first seen in consultation with the Otolaryngology Service eight months ago. At that time, her mother stated that she had increasing right-sided ear pain and nasal discharge, despite having just undergone adenoidectomy and bilateral pressure equalization tube placement at an outlying hospital. She had not been eating well and had lost approximately five pounds over the previous six months. Sinus radiographs were taken which showed opacification of both maxillary antra. She was initially treated for sinusitis with antibiotics and Proetz irrigation, but failed to respond. She was taken to the operating room for antral irrigation whereupon a nasopharyngeal mass was discovered and biopsied. The pathologic diagnosis on the tissue specimen was interpreted as embryonal rhabdomyosarcoma. A Computed Tomographic scan of the head and base of skull region demonstrated a large soft tissue mass extending from the right side of the nasopharynx into the right middle cranial fossa with bone erosion in the area of the right pterygoid plates and clivus. A metastatic workup consisting of chest C.T., lumbar puncture, bone scan and bone marrow biopsy was completed - all of which were negative for metastatic disease. She was enrolled in the Intergroup Rhabdomyosarcoma Study (IRS) protocol and subsequently treated with external beam radiotherapy to the tumor and cranial cavity as well as pulse chemotherapy. Although her treatment course has been complicated by intermittent aural drainage and profound anorexia requiring parenteral nutrition, her tumor response has been significant. She has continued her pulse chemotherapy up to the present time. Bibliography Anderson GJ, Lawrence WCT, Womer RB, et al: Rhabdomyosarcoma of the head and neck in children. Arch Otolaryngol Head Neck Surg 116:428-431, 1990. Batsakis JG: Neoplastic and non-neoplastic tumors of skeletal muscle, in Batsakis JG (ed): Tumors of the head and neck. Philadelphia, Williams and Wilkins, 1979, p 280-290. Batsakis JG, Regezi JA, Rice DH: The pathology of head and neck tumors: Fibroadipose tissue and skeletal muscle, part 8. Head Neck Surg 3:145-168, 1980. Bonilla JA, Healy GB: Management of malignant head and neck tumors in children. Pediatr Clin North Am 36:1443-1450, 1989. Cotton RT, Ballard ET, Going JA, et al: Tumors of the head and neck in children, in Thawley WE, Panje WR (eds): Comprehensive Management of Head and Neck Tumors. N.Y., W.B. Saunders, 1987, pp 1770-1779. Crist WM, Garsney L, Beltangady MS, et al: Prognosis in children with rhabdomyosarcoma: A report of the Intergroup Rhabdomyosarcoma Studies I and II. J Clin Oncol 8:443-452, 1990. Cunningham MJ, Myers EN, Bluestone CD: Malignant tumors of the head and neck in children: A twenty-year review. Int J Pediatr Otorhinolaryngol 13:279-292, 1987. Ehrlich FE, Haas JE, Kiesewetter WB: Rhabdomyosarcoma in infants and children: Factors affecting long-term survival. J Pediatr Surg 6:571-577, 1971. Flamant F, Hill C: The improvement in survival associated with combined chemotherapy in childhood rhabdomyosarcoma: A historical comparison of 345 patients in the same center. Cancer 53:2417-2421, 1984. Flamant F, Rodary C, Voute PA, et al: Primary chemotherapy in the treatment of rhabdomyosarcoma in children: Trial of the International Society of Pediatric Oncology (SIOP) preliminary results. Radiother Oncol 3:227-238, 1985. Goepfert H, Cangir A, Lindberg R, Ayala A: Rhabdomyosarcoma of the temporal bone. Arch Otolaryngol 105:310-313, 1979. Grosfeld JL, Weber TR. Weetman RM, et al: Rhabdomyosarcoma in childhood: analysis of survival in 98 cases. J Pediatr Surg 18:141-146, 1983. Healy GB: Malignant tumors of the head and neck in children: Diagnosis and treatment. Otolaryngol Clin North Am 13:483-488, 1980. Houghton PJ, Shapiro DN, Houghton JA: Rhabdomyosarcoma: From the laboratory to the clinic. Otolaryngol Clin North Am 38:349-364, 1991. Larson DL, Kroll S, Jaffe N, Serure A, Goepfert H: Long-term effects of radiotherapy in childhood and adolescence. Am J Surg 160:348-351, 1990. Lawrence W, Gehan EA, Hays DM et al: Prognostic significance of staging factors of the UICC staging system in childhood rhabdomyosarcoma: A report from the Intergroup Rhabdomyosarcoma Study (IRS-II). J Clin Oncol 5:46-54, 1987. Lawrence W, Hays DM, Heyn R, et al: Surgical lessons from the Intergroup Rhabdomyosarcoma Study (IRS) pertaining to extremity tumors. World J Surg 12:676-684, 1988. Mandell LR, Massey V, Ghavimi F: The influence of extensive bone erosion on local control in non-orbital rhabdomyosarcoma of the head and neck. Int J Radiat Oncol Biol Phys 17:649-653, 1989. McGill T: Rhabdomyosarcoma of the head and neck: An update. Otolaryngol Clin North Am 22:631-636, 1989. Miller RW, Dalager NA: Fatal rhabdomyosarcoma among children in the United States, 1960-69. Cancer 34:1897-1900, 1974. Mitchell CD, Ventris JA, Warr TJ, et al: Molecular definition on a somatic cell hybrid of a specific 2:13 translocation breakpoint in childhood rhabdomyosarcoma. Oncogene 6:89-92, 1991. Mulligan LM, Matlashewski GJ, Scrable HJ, et al: Mechanisms of p53 loss in human sarcomas. Proc Natl Acad Sci USA 87:5863-5867, 1990. Parham DM, Webber B, Holt H, et al: Immunohistochemical study of childhood rhabdomyosarcomas and related neoplasms: Results of an Intergroup Rhabdomyosarcoma Study Project. Cancer 67:3072-3080, 1991. Pedrick TJ, Donaldson SS, Cox RS: Rhabdomyosarcoma: The Stanford experience using a TNM staging system. J Clin Oncol 4:370-378, 1986. Raney RB, Tefft M, Maurer HM, et al: Disease patterns and survival rate in children with metastatic soft-tissue sarcoma: A report from the Intergroup Rhabdomyosarcoma Study (IRS)-1. Cancer 62:1257-1266, 1988. Raney RB, Tefft M, Newton WA, et al: Improved prognosis with intensive treatment of children with cranial soft tissue sarcomas arising in nonorbital parameningeal sites: A report from the Intergroup Rhabdomyosarcoma Study. Cancer 59:147-155, 1987. Robinson LD, Smith RJH, Rightmire J, Torpy JM, Fernbach DJ: Head and neck malignances in children: An age-incidence study. Laryngoscope 98:11-13, 1988. Rodary C, Gehan EA, Flamant F, et al: Prognostic factors in 951 nonmetastic rhabdomyosarcoma in children: A report from the International Rhabdomyosarcoma Workshop. Med Pediatr Oncol 19:89-95,1991. Ruymann FB, Maddux HR, Ragab A, et al: Congenital anomalies associated with rhabdomyosarcoma: An autopsy study of 115 cases. A report from the Intergroup Rhabdomyosarcoma Study Committee. Med Pediatr Oncol 16:33-39, 1988. Schuller DE, Lawrence TL, Newton WA: Childhood rhabdomyosarcomas of the head and neck. Arch Otolaryngol 105:689-694,1979. Sutow WW, Lindberg RD, Gehan EA, et al: Three-year relapse-free survival rates in childhood rhabdomyosarcoma of the head and neck. Cancer 49:2217-2221, 1982. Sutow WW, Sullivan MP, Ried HL, et al: Prognosis in childhood rhabdomyosarcoma. Cancer 25:1384-1390, 1970. Wharam MD, Beltandady MS, Heyn RM, et al: Pediatric orofacial and laryngeal rhabdomyosarcoma: An Intergroup Rhabdomyosarcoma Study report. Arch Otolaryngol Head Neck Surg 113:1225-1227, 1987. Wharam MD, Foulkes MA, Lawrence W, et al: Soft tissue sarcoma of the head and neck in childhood: Nonorbital and nonparameningeal sites. Cancer 53:1016-1019, 1984. Wiatrak BJ, Pensak ML: Rhabdomyosarcoma of the ear and temporal bone. Laryngoscope 99:1188-1192, 1989. Yousem DM, Lexa FJ, Bilaniuk LT, et al: Rhabdomyosarcomas in the head and neck: MR imaging evaluation. Radiology 177:683-686, 1990. Grand Rounds Archive | Department Home page BCM Public | BCM Intranet | Privacy Notices | Contact BCM | BCM Site Map | ©2001-2006 Baylor College of Medicine
|