Neck Vsh Investigation

Rhabdomyosarcoma is classified into three cell types: embryonal, alveolar (which has botryoid and spindle cell variants), and pleomorphic. The embryonal subtype accounts for 60% to 70% of childhood cases and is the most common subtype. It is also the most common subtype presenting in the head and neck (20-22).

Rhabdomyosarcomas are malignant tumors of skeletal muscle. They are part of the larger group of soft-tissue sarcomas. Sarcomas of bony origin are classified separately. Like most malignant tumors, the etiology is probably multifactorial. Studies have suggested that maternal exposure to ionizing radiation during pregnancy increases the risk of sarcoma development. There is also evidence that socioeconomic status and maternal use of recreational drugs during pregnancy play a role in soft-tissue sarcoma development. Chromosomal abnormalities probably play a role in sarcoma development (20), especially translocation of chromosomes 2,13 and 1,13. The associated chromosome abnormalities can be used for diagnostic confirmation and have a role in residual tumor diagnosis and monitoring. An example is the use of chromosomal confirmation to assess a residual mass for viable tumors cells. Several syndromes have been associated with the development of rhabdomyosarcoma. Among them are Li-Fraumeni, neurofibromatosis type I, and Beckwith-Wiedemann syndrome (22).


Patient presentation depends on the tumor location and size. Head and neck presentations mimic other head and neck neoplasms and depend on the site and extent of the mass. These tumors occur along the anterior skull base, parameningeal surfaces, temporal bone, and within the orbit.

Temporal bone and middle ear tumors often present with unilateral otorrhea and conductive hearing loss. As the tumor progresses, facial nerve paralysis and other cranial nerve neuropathies occur. Other head and neck manifestations include nasal obstruction, facial swelling, oropharyngeal mass, and unilateral proptosis. Metastatic rhabdomyosar-coma is part of the differential diagnosis for pediatric neck masses (23).

Rhabdomyosarcomas may occur in multiple other locations. Common areas include the kidney, biliary tract, extremities, and spinal cord. The symptoms will depend on the site and severity of the disease.


Diagnosis is made by biopsy (fine needle or open biopsy). If rhabdomyosarcoma is strongly suspected, comprehensive imaging should be done prior to open biopsy to accurately delineate tumor extent. Once a diagnosis is established, a multidisciplinary approach is required for all patients. CT scan of the chest and bone marrow aspirate should always be performed. Further imaging is based upon tumor location, i.e., magnetic resonance imaging (MRI) scans of the skull base for parameningeal disease and a CT scan of the abdomen and pelvis for GU and lower extremity primaries (21,22,24).

TABLE 2 Grouping of Rhabdomyosarcoma




I: Localized disease or


Ia: Tumor limited to muscle of origin

completely resected (low risk)


Ib: Local spread beyond muscle of origin

II: Gross total resection


IIa: Residual microscopic tumor


IIb: Microscopic tumor cells in local lymph nodes

III: Incomplete resection


IIIa: Biopsy only

(intermediate risk)


IIIb: 50% resection

IV: (High risk)


Distant metastatic disease present at onset

TABLE 3 Tumor, Node, and Metastasis Staging



Tumor <5cm


Tumor >5cm



Negative nodes


Any positive nodes





Any metastasis

Stage I


Favorable sites, any size, any nodal status [orbit, GU (not bladder or prostate), head and neck]

Stage II

Unfavorable sites, but small and negative lymph nodes (extremities, bladder, prostate, and parameningeal)

Stage III


Unfavorable sites, small or large and positive lymph nodes

Stage IV

Distant metastatic disease

Abbreviations: GU, genitourinary.

Abbreviations: GU, genitourinary.

The Intergroup Rhabdomyosarcoma Study Group has established prognostic indicators for rhabdomyosarcoma. This group, along with the National Wilms Tumor Study Group and the Children's Oncology Group, produced a staging system based on tumor size, nodal involvement, and presence or absence of metastases (Tables 2 and 3). These staging systems help determine the level of risk of individual patients and can help tailor treatment options (Fig. 2A and B) (24).


The management of previously untreated childhood rhabdomyosarcoma has been well studied. All patients are treated with multimodality therapy and are considered to have micrometastases. Therefore, all patients receive chemotherapy (22). The principles of

FIGURE 2 (A) Nasal rhabdomyosarcoma before treatment, staged as group III (biopsy only), T2N0M0 (Stage II—orbit involvement). (B) MRI of tumor after treatment with course of chemotherapy. Patient underwent medical maxillectomy and additional radiation therapy. Abbreviation: MRI, magnetic resonance imaging.

FIGURE 2 (A) Nasal rhabdomyosarcoma before treatment, staged as group III (biopsy only), T2N0M0 (Stage II—orbit involvement). (B) MRI of tumor after treatment with course of chemotherapy. Patient underwent medical maxillectomy and additional radiation therapy. Abbreviation: MRI, magnetic resonance imaging.

surgical treatment are to preserve all vital cosmetic and functional structures to accept close margins, and to reserve radical surgery for recurrence following radiotherapy and chemotherapy.

Orbital tumors require biopsy for diagnosis, followed by chemotherapy and radiation therapy. If a tumor persists after primary therapy, orbital exenteration should be considered (22).

Extraorbital head and neck tumors are treated with wide local excision and neck lymph node sampling of clinically involved lymph nodes. Close margins (< 1 mm) are reasonable if anatomic restrictions are present. Anterior skull base tumors can be resected in cases of children with recurrent locoregional disease and residual disease following chemotherapy and radiation therapy. Tumors that are considered unresectable should undergo radiation and chemotherapy, with surgery to remove any residual masses, if feasible.

As previously mentioned, chemotherapy is used in the treatment of all patients with rhabdomyosarcoma (22). The regimen depends on the risk status of the patient (Table 2). "Low-risk" patients receive vincristine and dactinomycin. "Intermediate-risk" patients receive additional doses of cyclophosphamide. "High-risk" patients have poor response rates to current chemotherapeutic options. High-dose chemotherapy with stem cell rescue is under investigation. In general, patients in this group receive the standard therapy plus additional drugs such as ifosfamide and doxorubicin.

Radiation therapy is tailored to the specific disease. Patients who do not have residual disease after primary treatment do well without radiation. Patients with gross residual disease receive a standard dose of 5000 cGy. Radiation is given after chemotherapy (20,22).

Patients with middle-ear rhabdomyosarcoma deserve special mention due to the potential role the otolaryngologist plays in early diagnosis and intervention. As mentioned, these patients can present with bloody otorrhea not responsive to conservative management. Middle-ear rhabdomyosarcoma tends to present in a parameningeal location and often is unresectable (Groups III and IV) (25). However, most tumors are small (<5 cm) and do not involve lymph nodes. These patients are usually treated with chemotherapy and radiation. Five-year survival rates are 67% for all patients and have improved over time (25).


Recurrent rhabdomyosarcoma presents the most favorable prognosis if it is local or regional. Most children, however, present with advanced disease (distant metastasis or unresectable disease) and have a poor prognosis. These patients are generally treated with combinations of chemotherapeutics that have not been given to them previously.

The prognosis of low-risk patients is good, with greater than 95% five-year survival rates with standard therapies. Intermediate-risk patients have between 50% and 70% survival rates. High-risk patients have poor outcomes, with survival rates from 22% to 34% (22,26,27).


In general, pediatric rhabdomyosarcoma is a soft-tissue malignancy commonly seen in the head and neck. Its presentation depends on the site and extent of the disease. Surgical management is an important part of treatment. Complete resection can place a patient in the low-risk group with a more favorable prognosis. A multidisciplinary approach is imperative in the management of this disease. As with most malignant tumors, early diagnosis and treatment can make a significant difference in outcomes.


1. Coppes-Zantinga A, Egeler RM. The langerhans cell histiocytosis X files revealed. Br J Haematol 2002; 116(1):3-9.

2. Lipton JM, Arceci RJ. Dendritic cell disorders. In: Hoffman R, ed. Hematology: Basic Principles and Practice. 4th ed. Philadelphia, PA: Churchill Livingstone (Elsevier), 2005:857-868.

3. Aster JC. Diseases of white blood cells, lymph nodes, spleen, and thymus. In: Kumar V, Abbas AK, Fausto N, eds. Robbins & Cotran Pathologic Basis of Disease. 7th ed. Philadelphia, PA: Elsevier Saunders, 2004:661-710.

4. Azouz EM, Saigal G, Rodriguez MM, et al. Langerhans' cell histiocytosis: pathology, imaging and treatment of skeletal involvement. Pediatr Radiol 2005; 35(2):103-115.

5. Irving RM, Broadbent V, Jones NS. Langerhans' cell histiocytosis in childhood: management ofhead and neck manifestations. Laryngoscope 1994; 104(1 Pt 1):64-70.

6. Vassallo R, Ryu JH. Pulmonary langerhans' cell histiocytosis. Clin Chest Med 2004; 25(3): 561-571, vii.

7. Davis SE, Rice DH. Langerhans' cell histiocytosis: current trends and the role of the head and neck surgeon. Ear Nose Throat J 2004; 83(5):340, 342, 344 Passim.

8. Merchant S, Nadol JB Jr. Otologic manifestations of systemic disease. In: Cummings CW, ed. Cummings Otolaryngology-Head and Neck Surgery. 4th ed. Philadelphia, PA: Elsevier, 2005: 2881-2905.

9. Lahey ME. Prognostic factors in histiocytosis X. Am J Pediatr Hematol Oncol 1981; 3(1): 57-60.

10. Uddin DE, Dickson LG, Brodine CE. Screening of military recruits for hemoglobin variants. JAMA 1974; 227(12):1405-1407.

11. Stuart MJ, Nagel RL. Sickle cell disease. Lancet 2004; 364(9442):1343-1360.

12. Firth PG, Head CA. Sickle cell disease and anesthesia. Anesthesiology 2004; 101(3): 766-785.

13. Stuart MJ, Setty BN. Sickle cell acute chest syndrome: pathogenesis and rationale for treatment. Blood 1999; 94(5):1555-1560.

14. Ohene-Frempong K, Weiner SJ, Sleeper LA, et al. Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood 1998; 91(1):288-294.

15. Adams RJ, McKie VC, Carl EM, et al. Long-term stroke risk in children with sickle cell disease screened with transcranial Doppler. Ann Neurol 1997; 42(5):699-704.

16. Ballas SK. Sickle cell anaemia: progress in pathogenesis and treatment. Drugs 2002; 62(8): 143-1172.

17. Charache S, Terrin ML, Moore RD, et al. Effect ofhydroxyurea on the frequency ofpainful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia. N Engl J Med 1995; 332(20):1317-1322.

18. Koshy M, Weiner SJ, Miller ST, et al. Surgery and anesthesia in sickle cell disease. Cooperative Study of Sickle Cell Diseases. Blood 1995; 86(10):3676-3684.

19. Davies SC. Blood transfusion in sickle cell disease. Curr Opin Hematol 1996; 3(6):485-491.

20. Gurney JG, Young JL Jr, Roffers SD, et al. Soft tissue sarcomas. In: Ries LA, Smith MA, Gurney JG, et al., eds. Seer Pediatric Monograph. Bethesda, MD: National Cancer Institute, Seer Program, 1999:111-123.

21. Rosenberg AE. Bone, joints, and soft tissue tumors. In: Kumar V, Abbas AK, Fausto N, eds. Robbins & Cotran Pathologic Basis of Disease. 7th ed. Philadelphia, PA: Elsevier Saunders, 2005:1321-1322.

22. National Cancer Institutes PDQ Cancer Information Database. Childhood Rhabdomyosarcoma. http:// (Accessed October 2005).

23. Van De Graaf RL, Cass SP. Tumors of the ear and temporal bone. In: Bluestone CD, ed. Pediatric Otolaryngology. 4th ed. Philadelphia, PA: Saunders, 2004:849-859.

24. Breitfeld PP, Meyer WH. Rhabdomyosarcoma: new windows of opportunity. Oncologist 2005; 10 (7):518-527.

25. Hawkins DS, Anderson JR, Paidas CN, et al. Improved outcome for patients with middle ear rhabdomyosarcoma: a children's oncology group study. J Clin Oncol 2001; 19(12):3073-3079.

26. Gurney JG, Severson RK, Davis S, et al. Incidence of cancer in children in the United States. Sex-, race-, and 1-year age-specific rates by histologic type. Cancer 1995; 75(8):2186-2195.

27. Crist W, Gehan EA, Ragab AH, et al. The third intergroup rhabdomyosarcoma study. J Clin Oncol 1995; 13(3):610-630.

Part D ■ Other Systemic Disorders

Was this article helpful?

0 0
10 Ways To Fight Off Cancer

10 Ways To Fight Off Cancer

Learning About 10 Ways Fight Off Cancer Can Have Amazing Benefits For Your Life The Best Tips On How To Keep This Killer At Bay Discovering that you or a loved one has cancer can be utterly terrifying. All the same, once you comprehend the causes of cancer and learn how to reverse those causes, you or your loved one may have more than a fighting chance of beating out cancer.

Get My Free Ebook

Post a comment