Primary malignant melanoma of the epiglottis: a rare presentation.Abstract Primary malignant melanoma of the epiglottis epiglottis (ĕp'əglŏt`ĭs): see larynx. is extremely rare. Until now, only 4 cases have been reported in the world literature. We describe a new case of epiglottic epiglottic pertaining to or emanating from the epiglottis. epiglottic cartilage attached to the thyroid cartilage of the larynx by the thyroepiglottic ligament; it is the structural basis of the epiglottis. primary malignant melanoma in a 74-year-old man who presented with hoarseness and a foreign-body sensation. Clinical examination revealed the presence of a small, whitish, polypoid tumor on the laryngeal surface of the epiglottis; no other primary melanoma was detected. Wide excision of the lesion was performed, and microscopy revealed that it contained melanin-pigmented tumor cells in both the mucosa and submucosa submucosa /sub·mu·co·sa/ (sub?mu-ko´sah) areolar tissue situated beneath a mucous membrane. sub·mu·co·sa n. A layer of loose connective tissue beneath a mucous membrane. . Immunostaining was positive for S-100 protein. The patient was treated with radiotherapy, and he remained well 1 year after the diagnosis with no evidence of recurrence. Introduction Malignant melanoma is a neoplasm neoplasm or tumor, tissue composed of cells that grow in an abnormal way. Normal tissue is growth-limited, i.e., cell reproduction is equal to cell death. that arises from melanocytes Melanocytes Skin cells derived from the neural crest that produce the protein pigment melanin. Mentioned in: Malignant Melanoma, Skin Pigmentation Disorders melanocytes . Melanocytes are derived from the neural crest and are widely distributed throughout all cutaneous and mucosal surfaces. They are found in the basal layer of the epidermis near the dermal-epidermal junction; they have been reported in the larynx, nasal cavity, and mouth. (1) Although melanoma usually arises from the skin, in rare cases it can affect the eye, meninges meninges (mĭnĭn`jēz), three membranous layers of connective tissue that envelop the brain and spinal cord (see nervous system). The outermost layer, or dura mater, is extremely tough and is fused with the membranous lining of the skull. , and the mucous membranes of the digestive and upper respiratory tract. Several varieties of epiglottic neoplasms have been described in the literature. Of these, malignant melanoma is rare. When malignant melanoma of the epiglottis does occur, it can be either primary or secondary. Until now, only 4 cases of primary malignant melanoma of the epiglottis have been reported in the world literature. (2-5) None of them were associated with basal cell carcinoma basal cell carcinoma n. A slow-growing, locally invasive, but rarely metastasizing neoplasm of the skin derived from basal cells of the epidermis or hair follicles. Also called basal cell epithelioma. . A case of a secondary amelanotic melanoma of the epiglottis was reported by Ikeda et al in 1991. (6) In this article, we report a new case of primary malignant melanoma of the epiglottis, and we briefly review the relevant literature. Case report A 74-year-old man presented to the Department of Laryngology laryngology /lar·yn·gol·o·gy/ (-gol´ah-je) the branch of medicine dealing with the throat, pharynx, larynx, nasopharynx, and tracheobronchial tree. lar·yn·gol·o·gy n. and Otology otology /otol·o·gy/ (o-tol´ah-je) the branch of medicine dealing with the ear, its anatomy, physiology, and pathology.otolog´ic o·tol·o·gy n. The branch of medicine that deals with the ear. at James Paget Hospital Located at Gorleston, Norfolk, on the A12 road, the James Paget Healthcare NHS Trust serves a population of around 220,000 people in the Great Yarmouth, Lowestoft and Waveney areas. It was established as a third wave NHS Trust from 1 April 1993. with a 3-month history of hoarseness and an unusual sensation in his throat. His medical history was significant for several recurrent basal cell carcinomas of the head and neck (which had been treated with cryotherapy Cryotherapy Definition Cryotherapy is a technique that uses an extremely cold liquid or instrument to freeze and destroy abnormal skin cells that require removal. and excision) and for tuberculous tuberculous /tu·ber·cu·lous/ (too-ber´ku-lus) pertaining to or affected with tuberculosis; caused by Mycobacterium tuberculosis. tu·ber·cu·lous adj. 1. cervical lymphadenitis Lymphadenitis Definition Lymphadenitis is the inflammation of a lymph node. It is often a complication of a bacterial infection of a wound, although it can also be caused by viruses or other disease agents. (for which he had received a full course of standard antituberculosis chemotherapy). He was a smoker and had been so for more than 40 years. Clinical examination did not reveal any cutaneous melanomas or palpable cervical lymph nodes Cervical lymph nodes are lymph nodes found in the neck. Anterior cervical nodes The anterior cervical nodes are a group of nodes found on the anterior part of the neck. . Fiberoptic laryngoscopy detected a small, white, polypoid lesion on the posterior aspect of the epiglottis (figure 1). Under general anesthesia, the patient underwent direct laryngoscopy, and the lesion was excised and submitted for histopathologic examination. A paraffin section stained with hematoxylin and eosin (H&E) showed tumor cells with clear nuclei exhibiting pleomorphism pleomorphism /pleo·mor·phism/ (-mor´fizm) the occurrence of various distinct forms by a single organism or within a species.pleomor´phicpleomor´phous ple·o·mor·phism n. 1. and melanin melanin (mĕl`ənĭn), water-insoluble polymer of various compounds derived from the amino acid tyrosine. It is one of two pigments found in human skin and hair and adds brown to skin color; the other pigment is carotene, which contributes pigmentation in the cytoplasm (figure 2). These cells had hyperchromatic nuclei, and they invaded both the mucosa and submucosa. On immunohistochemistry for S-100 protein, the tumor cells demonstrated a strong positive staining in both the nuclei and cytoplasm (figure 3). The lesion was diagnosed as a primary malignant melanoma because all clinical, panendoscopic, and ophthalmologic examinations conducted to identify a possible primary lesion elsewhere were negative. Following surgical excision, the patient opted to undergo radiotherapy. One year after his diagnosis, he was well and showed no evidence of recurrence. Discussion No case of malignant melanoma of the epiglottis has been published since 1991. (6) Only 0.6 to 9.3% of patients with cutaneous malignant melanoma will experience a metastasis to the mucosa of the upper aerodigestive tract. (7) Mucosal melanomas are usually of the acral lentiginous len·ti·go n. pl. len·tig·i·nes A small, flat, pigmented spot on the skin. [Latin lent type, (8) and they occur most often in the nasal cavity and sinuses (table 1). (9) Among laryngeal melanomas, the supraglottic region is the most common site of involvement. (10) Cases of metastatic melanoma from an unknown primary may be attributable to (1) spontaneous regression of the primary, (2) the location of a primary in a visceral organ, or (3) an inability to detect a primary in the skin. The clinical features of laryngeal melanomas include hoarseness, hemoptysis Hemoptysis Definition Hemoptysis is the coughing up of blood or bloody sputum from the lungs or airway. It may be either self-limiting or recurrent. Massive hemoptysis is defined as 200-600 mL of blood coughed up within a period of 24 hours or less. , dysphagia, airway obstruction, and stridor Stridor Definition Stridor is a term used to describe noisy breathing in general, and to refer specifically to a high-pitched crowing sound associated with croup, respiratory infection, and airway obstruction. . (11-13) Enlarged cervical lymph nodes may be present. The tumor can spread to the brain, lungs, and spine. (12) Our patient presented with hoarseness and a foreign-body sensation. His hoarseness may have been attributable to chronic laryngitis secondary to his smoking. The foreign-body sensation disappeared after the tumor was excised. A diagnosis of melanoma must be confirmed by histologic examination of an excisional biopsy specimen; obtaining a specimen might require direct laryngoscopy under anesthesia. Under light microscopy, the tumor will exhibit a pleomorphic pleomorphic adjective Referring to a variable appearance or morphology epithelioid cell population of malignant cells. Malignant spindle-shaped ceils may also be identified. Cells often contain dark-brown cytoplasmic and nuclear melanin. (12,14) The presence of melanin is identifiable on H&E staining, Fontana staining, and immunohistochemistry, which will show immunoreactivity with S-100 protein and HMB-45. (12) Electron microscopy may identify the presence of melanosomes or premelanosomes. Although the tumor in our patient appeared to be amelanotic on clinical examination, melanin pigmentation was seen in the cytoplasm on microscopy, and immunohistology was positive for S-100 protein. Three conditions usually exist that help identify a tumor as a primary malignant melanoma: (1) the tumor should be the dominant lesion, (2) there may be local and regional metastasis, and (3) the patient should have no history of a primary cutaneous or ocular melanoma or nevus nevus /ne·vus/ (ne´vus) pl. ne´vi [L.] 1. any congenital skin lesion; a birthmark. 2. a type of hamartoma representing a circumscribed stable malformation of the skin and occasionally of the oral mucosa, that regressed spontaneously. (8) The hallmark of a primary malignant melanoma is the presence of junctional activity in the overlying overlying suffocation of piglets by the sow. The piglets may be weak from illness or malnutrition, the sow may be clumsy or ill, the pen may be inadequate in size or poorly designed so that piglets cannot escape. or adjacent mucosa. (8) Malignant cells must be identified in the surface epithelium to establish a diagnosis of a primary malignant melanoma. (14,15) In our patient, malignant cells were clearly seen in the surface epithelium (figure 2). Our patient had no other identifiable malignant lesion, and histology of his previous basal cell carcinomas did not provide any evidence of pigmentation. Therefore, his tumor was diagnosed as a primary. Positron-emission tomography (PET) and magnetic resonance imaging magnetic resonance imaging (MRI), noninvasive diagnostic technique that uses nuclear magnetic resonance to produce cross-sectional images of organs and other internal body structures. (MRI) may be used for staging malignant melanomas. In a retrospective study of fluorodeoxyglucose (FDG) PET in 18 patients with mucosal melanoma, Goerres et al reported that all tumors were visible at staging, but the FDG uptake was dependent on the size and anatomic site of the lesion. (16) Big nodular lesions were more visible than the more superficial spreading lesions. Lesions in the anterior part of the nose were more difficult to detect than those located elsewhere. Yoshioka et al analyzed MRI findings in 6 patients with mucosal melanoma and found that on T1-weighted imaging, 5 melanomas were hyperintense and 1 was isointense. (17) On T2-weighted imaging, 5 were of mixed intensity and 1 was isointense. The mean signal intensity ratios for the primary melanoma to muscle on T1-weighted imaging were 1.51 with gadolinium gadolinium (gădəlĭn`ēəm), metallic chemical element; symbol Gd; at. no. 64; at. wt. 157.25; m.p. 1,312°C;; b.p. 3,233°C;; sp. gr. 7.898 at 25°C;; valence +3. contrast and 1.39 without it; the difference was not statistically significant. This finding suggests that hyperintensity on T1-weighted imaging is a common characteristic of mucosal melanoma but not a universal finding. The only curative treatment is radical local excision (total laryngectomy and neck dissection for laryngeal melanoma). (11) Local recurrences are common, even when surgical margins are wide. A simple local excision is occasionally used. There is 1 report in the literature of a case of melanoma of the right vocal fold that was managed with a right cordectomy and adjuvant radiotherapy. (18) That patient remained well without any evidence of local recurrence or metastasis for more than 3 years. In the case described by Ikeda et al, a patient with cutaneous amelanotic melanoma developed a metastatic tumor that involved the epiglottis; the tumor was successfully excised via an intraoral approach and KTP/532 laser surgery. (6) Nonsurgical treatments include radiotherapy, chemotherapy, immunotherapy, and C[O.sub.2] laser ablation. (19) Several reports published during the past decade on radiotherapy alone have documented complete response rates of 50 to 75% and enduring long-term control in one-half to two-thirds of the complete responders. (18,20) These data, taken in conjunction with the high rate of local failure after surgery alone, suggest that radiotherapy would have a useful role as a surgical adjuvant in a combined-modality approach, as well as being of value in the primary management of unresectable disease. (18,20) Currently, chemotherapy is principally used for the treatment of disseminated disease and for palliation pal·li·ate tr.v. pal·li·at·ed, pal·li·at·ing, pal·li·ates 1. To make (an offense or crime) seem less serious; extenuate. 2. . (21) Immunotherapeutic options include OK-432, interleukin 2, lymphokine-activated killer cells lymphokine-activated killer cells LAK cells, see there , and cyclophosphamide cyclophosphamide /cy·clo·phos·pha·mide/ (-fos´fah-mid) a cytotoxic alkylating agent of the nitrogen mustard group; used as an antineoplastic, as an immunosuppressant to prevent transplant rejection, and to treat some diseases . (22) The American Joint Committee on Cancer The American Joint Committee on Cancer (AJCC) is an organization best known for defining and popularizing cancer staging standards. External links
Several reasons explain the poor prognosis associated with mucosal melanoma: (1) the nonspecific nature of its symptoms leads to delays in diagnosis, (2) the tumor affects many older people whose immune function is inefficient, (3) the rich vascular and lymphatic efferents of the mucosa favor early metastasis, and (4) mucosal melanoma is histologically aggressive. (1,25-27) Even so, overall mortality for early head and neck melanoma has actually declined because a higher percentage of patients are seeking treatment when the tumor is at an early stage (table 2). (28) Acknowledgment The authors thank D.A. Harrison, BSc, FRCPath, of the Department of Pathology at James Paget Hospital in Great Yarmouth, U.K., for his advice on the pathology section. References (1.) Xavier R. Paiva A, Ribeiro da Silva P, Gameiro dos Santos A. Primary malignant melanoma of the palatine tonsil: A case report. J Laryngol Otol 1996; 110:163-6. (2.) Shanon E, Covo J, Loeventhal M. Melanoma of the epiglottis. A case treated by supraglottic laryngectomy Laryngectomy Definition Laryngectomy is the partial or complete surgical removal of the larynx, usually as a treatment for cancer of the larynx. Purpose Normally a laryngectomy is performed to remove tumors or cancerous tissue. . Arch Otolaryngol 1970;91:304-5. (3.) Welsh LW, Welsh JJ. Malignant melanoma of the larynx. Laryngoscope 1961:71:185-91. (4.) Pantazopoulos PE. Primary malignant melanoma of the larynx. Laryngoscope 1964;74:95-102. (5.) Carlin car·line or car·lin n. Scots A woman, especially an old one. [Middle English kerling, from Old Norse, from karl, man.] D. Cutaneous melanoma of the head and neck. Am J Surg 1966;112:512-21. (6.) Ikeda M, Takahashi H, Karaho T, et al. Amelanotic melanoma metastatic to the epiglottis. J Laryngol Otol 1991;105:776-9. (7.) Billings KR, Wang MB, Sercarz JA, Fu YS. Clinical and pathologic distinction between primary and metastatic mucosal melanoma of the head and neck. 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Pau H, De S, Spencer MG, Steele PR. Metastatic malignant melanoma of the larynx. J Laryngol Otol 2001;115:925-7. (14.) Amin HH, Petruzzelli GJ, Husain AN, Nickoloff BJ. Primary malignant melanoma of the larynx. Arch Pathol Lab Med 2001;125: 271-3. (15.) Allen AC, Spitz S. Malignant melanoma; a clinicopathological analysis of the criteria for diagnosis and prognosis. Cancer 1953;6: 1-45. (16.) Goerres GW, Stoeckli SJ, von Schulthess GK, Steinert HC. FDG PET for mucosal malignant melanoma of the head and neck. Laryngoscope 2002; 112:381-5. (17.) Yoshioka H, Kamada T, Kandatsu S, et al. MRI of mucosal malignant melanoma of the head and neck. J Comput Assist Tomogr 1998;22:492-7. (18.) Asare-Owusu L, Shotton JC, Schofield JB. Adjuvant radiotherapy for primary mucosal malignant melanoma of the larynx. J Laryngol Otol 1999;113:932-4. (19.) Hussain SS, Whitehead E. Malignant melanoma of the larynx. J Laryngol Otol 1989;103:533-6. (20.) Trotti A, Peters LJ. Role of radiotherapy in the primary management of mucosal melanoma of the head and neck. Semin Surg Oncol 1993;9:246-50. (21.) Medina JE, Ferlito A, Pellitteri PK, et al. Current management of mucosal melanoma of the head and neck. J Surg Oncol 2003;83: 116-22. (22.) Mochimatsu I, Tsukuda M, Kurihara M, et al. [Case reports of mucosal melanoma of the head and neck]. Nippon Jibiinkoka Gakkai Kaiho 1996;99:552-7. (23.) Agarwala SS, Neuberg D, Park Y, Kirkwood JM. Mature results of a phase III randomized trial of bacillus Calmette-Guerin (BCG) versus observation and BCG plus dacarbazine versus BCG in the adjuvant therapy of American Joint Committee on Cancer Stage I-III melanoma (E1673): A trial of the Eastern Oncology Group. Cancer 2004; 100:1692-8. (24.) Pfahlberg A, Kolmel KF, Grange JM, et al. Inverse association between melanoma and previous vaccinations against tuberculosis and smallpox: Results of the FEBIM study. J Invest Dermatol 2002; 119:570-5. (25.) Pandey M, Mathew A, Iype EM, et al. Primary malignant mucosal melanoma of the head and neck region: Pooled analysis of 60 published cases from India and review of literature. Eur J Cancer Prev 2002; 11:3-10. (26.) Pandey M, Abraham EK, Mathew A, Ahamed IM. Primary malignant melanoma of the upper aero-digestive tract. Int J Oral Maxillofac Surg 1999;28:45-9. (27.) Pandey M, Mathew A, Abraham EK, et al. Primary malignant melanoma of the mucous membranes. Eur J Surg Oncol 1998;24: 303-7. (28.) Cowan A, Pou AM. Melanoma of the head and neck. In: Quinn FB Jr., Ryan MW, eds. Dr. Quinn's Online Textbook of Otolaryngology. Galveston, Tex.: University of Texas Medical Branch "UTMB" redirects here. For other system schools, see University of Texas System. The University of Texas Medical Branch (UTMB) is a component of the University of Texas System located in Galveston, Texas, about 50 miles (80 km) southeast of downtown Houston. ; 2004. www.utmb.edu/otoref/grnds/Melanoma-2004-0310/Melanoma-20040310.htm (accessed Feb. 20, 2006) (29.) Prasad ML, Patel SG, Huvos AG, et al. Primary mucosal melanoma of the head and neck: A proposal for microstaging localized, Stage I (lymph node-negative) tumors. Cancer 2004; 100:1657-64. Rajaraman Durai, MS, MRCS MRCS Member of Royal College of Surgeons. MRCS abbr. Member of the Royal College of Surgeons ; Syed Hashmi, FRCS FRCS Fellow of the Royal College of Surgeons. FRCS abbr. Fellow of the Royal College of Surgeons From the University Department of Surgery, Royal Free Hospital, London (Dr. Dural dural /du·ral/ (dur´'l) pertaining to the dura mater. dural pertaining to the dura mater. dural ossification see dural ossification. ), and the Department of Laryngology and Otology, James Paget Hospital, Great Yarmouth, U.K. (Dr. Hashmi). Reprint requests: Rajaraman Durai, MS, University Department of Surgery, Royal Free Hospital, Pond St., London NW 2QG, UK. Phone: 44-775-126-5993; fax: 44-207-377-7684; e-mail: dr_durai@yahoo.com Table 1. Location of mucosal melanomas of the head and neck in 259 patients in Merseyside, U.K. (9) Site % Nasal cavities and sinuses 69 Oral cavity 22 Pharynx, larynx, and upper esophagus 9 Table 2. Prognosis of stage I primary mucosal mela-nomas of the head and neck's Level Invasion Survival I In situ 11 yr 6 mo II Lamina propria only 5 yr 9 mo III Deep tissue 1 yr 5 mo |
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