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. Melanoma of the Head and Neck Between 2 and 3 billion melanocytes in the human body provide pigmentation to the skin and hair by producing melanin. Melanocytes develop from neuroectodermal elements in the early stages of gestation. Melanomas develop from the malignant neoplastic transformation of these cells. The reported incidence in the U.S. population is 4 cases per 100,000, while the incidence in Australia is as high as 40 cases per 100,000. Ethnicity and sun exposure appear to play a major role in the development of cutaneous melanoma. Ultraviolet B light is thought to be the most important factor in sun exposure. According to epidemiologic data, those with blond hair, blue eyes, and those that tend to burn or freckle easily after sun exposure are at the highest risk. There is also a reported slight male preponderance of 1.5 : 1 and a peak incidence in the 4th to 7th decades. Mucosal melanoma, on the other hand, has no association with sun exposure. The peak age is about the same as that for cutaneous melanoma. Interestingly, mucosal melanoma is much more common in Japan where it represents 22% to 32% of all melanomas, arising predominantly in the oral cavity. Head and neck melanomas account for 17% to 25% of all cutaneous melanomas, although representing only 9% of the total skin surface area. Lesions are found predominantly on the face, scalp, neck, and ear, in that order. Mucosal melanomas constitute only 2% to 3% of all melanomas, but 8.5% of all head and neck melanomas. The majority appear in the nose and paranasal sinuses, followed by the oral cavity. The diagnosis of cutaneous melanoma must be confirmed by excisional or punch biopsy of the thickest portion of the lesion so that histologic microstaging can be performed. Shave biopsy or curettage is contraindicated as thickness cannot be ascertained. Histologic staging systems have been developed by Clark (1969) and by Breslow (1970). Clark staged by level of invasion: Level I: atypical melanocytic hyperplasia Breslow proposed staging by tumor thickness: < 0.75mm Both staging systems provide independent prediction of prognosis; however, tumor thickness appears to be more reliable. Studies have shown that stage, tumor thickness, and location are the only independent predictors of survival. In the head and neck, scalp tumors appear to have the worst prognosis. In stage I disease, location, thickness, Clark's level, and ulceration were all independent predictors of survival. There is no histologic system for staging mucosal melanoma. Thickness and invasion do not appear to have any prognostic value: the prognosis is uniformly poor. Distant metastasis at the time of diagnosis is the only factor that appears to have prognostic significance. The surgical management of melanoma has been much debated. Currently, 1 cm to 2 cm margins are considered adequate for the excision of thin melanomas less than 1.5 mm thick. Two to three centimeter margins are indicated for thicker lesions. Regional elective node dissection in the clinically negative neck is very controversial as the literature is full of conflicting reports. However, it is clear that the risk of metastasis varies directly with tumor thickness and with unfavorable prognostic factors. Thus, lesions less than 0.75 mm thick have less than a 2% chance of micrometastasis and node dissection is not necessary. Lesions 0.76 mm to 1.49 mm have a 5% to 20% chance of micrometastasis and require node dissection if they are ulcerated, nodular, or have a deep Clark's level, or are located in an unfavorable location. Lesions more than 1.5 mm thick have a high incidence of micrometastasis, varying from 20% to 60%, and elective node dissection is recommended. In the clinically positive neck, many authors recommend that a therapeutic neck dissection be performed. Despite this procedure the prognosis remains poor. Seventy to eighty percent of patients with nodal disease will die of disseminated melanoma. The pattern of cervical lymph node metastasis is so unpredictable that a radical neck dissection is usually necessary. The surgical management of mucosal melanoma is usually unrewarding. The overall prognosis is usually poor. By nature of the location of these lesions, resection may be very debilitating and not alter the prognosis significantly. Palliative resection may be indicated in some cases. Survival statistics for cutaneous melanoma vary directly with stage. Five-year survival of stage I disease is 79%; for stage II, 68%; for stage III, 28%; and for stage IV, 15%. In contrast, mucosal melanoma has a uniformly poor prognosis with a five-year survival of 8% to 15%, although a few isolated studies have shown longer survival. In conclusion, it is important for the otolaryngologist to be aware of the clinical presentation and treatment of melanoma. The incidence of melanoma is increasing along with the increase in the rate of the other skin malignancies associated with sun exposure. In addition, melanomas appear to be more common in the head and neck than other parts of the body. One should also be aware that mucosal melanoma has different behavior, treatment, and prognosis than its cutaneous counterpart. Case Presentation A 57-year-old white female presented with a two-month history of rhinorrhea, nasal obstruction, and headache. Symptoms were predominantly on her left. She also stated that she had experienced epistaxis one year ago which had resolved spontaneously. Her surgical history was significant for a hysterectomy and a right thyroid lobectomy for thyroid cancer 15 years ago. She denied any other illness. Physical examination revealed for a pale pink polypoid mass in her left nasal cavity. CT scanning demonstrated a mass confined to the left nasal cavity. The patient was brought to the operating room for a nasal biopsy and possible medial maxillectomy. Operative findings revealed a large pigmented lesion involving the superior one-third of the nasal septum with satellite lesions along the floor of the nose. Lesions were also present on the left middle turbinate and the ethmoids bilaterally. Pathologic diagnosis revealed malignant melanoma with a high degree of anaplasia and a high rate of mitotic activity. The tumor cells were positive for vimentin, S-100, and HMB-45. No further procedure was performed and her nose was packed. Upon removal of her nasal packs, the patient developed intermittent rhinorrhea. The serosanguinous discharge had elevated glucose, a positive ring sign, and was associated with a headache. This was felt to represent CSF rhinorrhea. She was treated conservatively with bedrest and head elevation. The rhinorrhea resolved spontaneously. Oncologic workup included a CT scan of the head, MRI of the neck, chest x-ray, and a bone scan, all of which were negative. CT and MRI of the abdomen revealed a right adrenal mass which was biopsied under CT guidance. This was negative for malignancy. 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