Malignant melanoma of the mucous membranes of the head and neck: three case reports.

Abstract

Primary malignant melanoma of the oronasal region is rare. When it does occur, it is difficult to manage, and the prognosis is not good. Early diagnosis and radical surgical management appear to offer the best hope of curing the disease. We report 3 new cases of malignant melanoma of the mucous membranes of the head and neck. Despite the generally poor outlook, 2 of these patients underwent excision of the tumor and remained disease-free 2 years later; the other patient refused surgery and was lost to follow-up.

Introduction

Malignant melanoma of the head and neck is a rare and threatening disease. Approximately 20% of melanomas occur in the head and neck region, with 6.3% occurring in the mucous membranes. (1) The incidence of oronasal malignant melanoma compared with the incidence of this tumor over the entire body is low. In a group of 1,225 cases of malignant melanoma reviewed by Pack et al, only 31 (2.5%) occurred in the mucosa of the oronasal region. (2) In this article, we present 3 new cases that were diagnosed and treated by us.

Case reports

Patient 1. A 40-year-old woman was admitted with a 6-month history of pain in the right cheek, blackening of the right side of the hard palate, loosening of the teeth, and oral bleeding on brushing. On examination, a blackish, ulcerated growth was seen on the right side of the hard palate (figure 1, A). Medially, the growth had reached the midline; laterally, it involved the right gingivobuccal sulcus; anteriorly, it involved the alveolus up to the central incisors; posteriorly, it extended just anterior to the retromolar trigone but did not involve it. The mass was soft and tender, and it bled when manipulated. The right upper incisors, upper canines, and upper premolars were loose. No cervical lymphadenopathy was found on general examination, and the systemic examination revealed no abnormality. Analysis of a biopsy specimen obtained with the patient under local anesthesia revealed features of a malignant melanoma. An orthopantograph was normal, as was computed tomography (CT) of the paranasal sinuses.

A subtotal maxillectomy was performed with general anesthesia via a lateral rhinotomy approach with lip splitting. The growth was excised in toto and sent for histopathologic examination. The tumor had well-defined borders and was made up of a biphasic population of cell masses, bundles of spindle-shaped cells, and round pleomorphic cells (figure 1, B). The tumor cells featured abundant melanin pigment, moderate pleomorphism, and abnormal mitotic figures. Masses of tumor cells had permeated the stratified squamous mucosa in a pagetoid pattern. Junctional activity was seen in a few places, along with moderate lymphocytic infiltration. These findings were consistent with a malignant melanoma. Resection margins were free of disease.

Postoperatively, the patient had an uneventful recovery, and she was given an obturator for her defect. She was called for follow-up every 4 months. Two years postoperatively, she remained free of any recurrence.

Patient 2. An 80-year-old man presented with epistaxis and an 8-month history of gradually increasing nasal obstruction on the right side. On examination, a single blackish mass was seen in the right nasal cavity. The lesion was soft and not sensitive, and it bled when manipulated. The left side of the nasal cavity was normal. Posterior rhinoscopy showed the mass in the nasopharynx. No cervical lymphadenopathy was evident, and systemic examination revealed no abnormality. A biopsy was obtained under local anesthesia, and histopathology detected malignant-melanoma-like features. Findings on routine blood and urine testing were normal. A skiagram of the chest was normal. CT of the paranasal sinuses showed that the growth extended from the nasal cavity into the nasopharynx with pansinusitis.

A right medial maxillectomy was performed with general anesthesia via a lateral rhinotomy approach, and the growth was excised in toto. Intraoperatively, the growth was found to have arisen from the middle turbinate. The specimen was sent for histopathology, and the tumor was identified as a malignant melanoma (figure 2).

Postoperatively, the patient's recovery was uneventful. He was called for follow-up every 4 months, and he exhibited no evidence of recurrence at 2 years.

Patient 3. A 65-year-old man presented for evaluation of a blackening of the palate. He reported that he had experienced occasional bleeding from the mouth during meals over the previous year.

On examination, a blackish, ulcerated growth was noted to involve the hard palate, soft palate, right anterior tonsillar pillar, and right upper alveolus (figure 3). The growth was soft and bled when touched. No cervical lymphadenopathy was detected on general examination, and the systemic examination revealed no abnormality. A biopsy was obtained under local anesthesia, and histopathology identified features consistent with malignant melanoma.

The patient was advised to undergo surgery, but he refused. He was then referred for radiotherapy but lost to follow-up.

Discussion

The behavior of malignant melanoma of the oronasal region is unpredictable, and the prognosis is generally poor. The origin of a melanoma in a mucous membrane is quite similar to that of a melanoma on the skin, as the melanocytes undergo malignant degeneration. The changes in the junctional layer are the dominant feature in the histogenesis of melanoma of the mucous membrane.

No predilection for either sex has been noted with this disease. (3) It seems to occur most often during the fifth through eighth decades of life, and it appears to be considerably less common in blacks than in whites. (3) Conley and Pack reported that previous radiation exposure is a predisposing factor. (4) Holdcraft and Gallagher found that patients often report a history of multiple polypectomies. (5) Conley and Pack reviewed 52 cases and found that the most common sites of origin were the oral cavity (50.0%), primarily the palate and alveolus; the nasal cavity (34.6%), primarily the septum and lateral wall; and the pharynx and larynx (15.4%). (4) The tumor is spread by local extension or via the lymphatic system or the circulatory system.

The most common presenting symptoms of malignant melanoma of the oronasal region are nasal blockade and epistaxis. Other symptoms include pain, swelling of the nose, a feeling of compression around the nose, and swelling of the palate. (5)

Aggressive radical surgery is the treatment of choice. (6) Radiation is not effective against melanoma in most instances, although some radiotherapeutic cures have been reported. (7) Radiotherapy reduces the likelihood of local failure, but it probably does not improve survival. The primary negative influences on survival are an advanced T stage and the presence of regional metastasis. (8) Chemotherapy and electrodesiccation have been tried, but their role is limited. Intralesional bacille Calmette-Guerin vaccine has been tried for metastatic disease. (9) Immunotherapy has been reported to be successful, but prospective trials are needed to confirm this. (10)

In view of the absence of any treatment modality that may substantially increase long-term survival, we suggest early diagnosis via biopsy of suspicious lesions and resection surgery with wide margins. (11)

References

(1.) Conley JJ, Pack GT. Melanoma ofthe head and neck. Surg Gynecol Obstet 1963;116:15-28.

(2.) Pack GT, Lenson N, Gerber DM. Regional distribution of moles and melanomas. AMA Arch Surg 1952;65:862-70.

(3.) Freedman HM, DeSanto LW, Devine KD, Weiland LH. Malignant melanoma of the nasal cavity and paranasal sinuses. Arch Otolaryngol 1973;97:322-5.

(4.) Conley J, Pack GT. Melanoma of the mucous membranes of the head and neck. Arch Otolaryngol 1974;99:315-19.

(5.) Holdcraft J, Gallagher JC. Malignant melanomas of the nasal and paranasal sinus mucosa. Ann Otol Rhinol Laryngol 1969;78:5-20.

(6.) Carlin D. Mucosal melanomas of the head and neck. Am J Roentgenol Radium Ther Nucl Med 1967;99: 809-16.

(7.) Rubenfeld S. Melanoma of the nasal air passages. Jew Mem Hosp Bull 1962;6-7:119-25.

(8.) Mendenhall WM, Amdur R J, Hinerman RW, et al. Head and neck mucosal melanoma. Am J Clin Oncol 2005;28:626-30.

(9.) Mastrangelo MJ, Bellet RE, Berkelhammer J, Clark WH Jr. Regression of pulmonary metastatic disease associated with intralesional BCG therapy of intracutaneous melanoma metastases. Cancer 1975:36:1305-8.

(10.) Shaw PM, Sivanandham M, Bernik SF, et al. Adjuvant immunotherapy for patients with melanoma: Are patients with melanoma of the head and neck candidates for this therapy? Head Neck 1997;19:595-603.

(11.) Gonzalez-Garcia R, Naval-Gias L, Martos PL, et al. Melanoma of the oral mucosa. Clinical cases and review of the literature. Med Oral Patol Oral Cir Bucal 2005; 10:264-71.

Arjun Dass, MS; Ramandeep S. Virk, MS; Harbir Hundal, MS; Harsh Mohan, MD

From the Department of Otolaryngology-Head and Neck Surgery, Government Medical College and Hospital, Chandigarh, India.

Reprint requests: Dr. Ramandeep S. Virk, House No. 2168, Sector 15C, Chandigarh 160015, India. Phone: [phone]; fax: [phone]; e-mail: [e-mail]