Clinical Group

Clinical presentation

Clinical presentation varies with the site of origin. Orbital tumors produce chemosis, a mass on the conjunctiva or eyelid or proptosis. Progression to blindness and ophthalmoplegia may result. Tumors originating in the nasopharynx, paranasal sinuses, middle ear and mastoid (parameningeal tumors) present with pain, discharge, epistaxis, cranial nerve palsies, voice changes and airway obstruction. Tumors arising from the soft tissues of the head and neck produce an asymptomatic firm mass. Bloody vaginal discharge or a red polypoid friable lesion at the introitus is highly suggestive of RMS of the vagina. Bladder tumors may mimic an infection with urinary frequency or other difficulties with urination. Prostatic lesions block urinary outflow, necessitating catheterization. Since prostatic lesion bulges into the rectum, digital rectal examination is diagnostic. Trunk or extremity lesions are visible as firm, fixed subcutaneous masses.

Diagnosis.Ultrasound, CT and MRI can be used to better define the anatomic relationships of the primary tumor. Metastatic evaluation includes chest radiographs, abdominal and chest CT scan, bone scan, skeletal survey, bone marrow aspirate and biopsy.

Depending upon the site and size of the tumor, diagnosis is made by incisional or excisional biopsy. It is imperative that enough tissue be obtained to run the special tests including analysis of fresh tissue for cytogenetic and molecular characteristics. It is also very important to make the incision after giving thought to the possibility of incorporating it in the ultimate excision.

Note!When an entire lump or suspicious area is removed, the procedure is called an excisional biopsy. When only a sample of tissue is removed with preservation of the histological architecture of the tissue’s cells, the procedure is called an incisional biopsy or core biopsy. When a sample of tissue or fluid is removed with a needle in such a way that cells are removed without preserving the histological architecture of the tissue cells, the procedure is called a needle aspiration biopsy.

The extent of residual disease after resection is one of the most important prognostic factors in RMS. For this reason, a clinical grouping system was developed in 1972 to stratify patients into groups that would more accurately reflect their prognosis and treatment options. Currently, patients are assigned to a clinical group based on the completeness of tumor excision and the evidence of tumor metastasis to the lymph nodes or distant organs after pathologic examination of surgical specimens (Table 9.3). This system differs from TNM staging in that determination of each patient's clinical group is based on the extent of the surgical resection instead of tumor size and site.

Table 9.3

Clinical grouping for RMS patients