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Primary Malignant Melanoma of the Larynx.

Compared with cutaneous melanomas of the head and neck, primary mucosal melanoma of the upper airways and digestive tract is associated with a very poor prognosis. Such a lesion is known to be insidious and remains dormant for most of its course. In general, mucosal melanoma of the head and neck is an uncommon lesion and comprises only 0.5% to 3% of all cases of malignant melanomas.[1] Laryngeal malignant melanoma constitutes only 3.8% to 7.4% of these cases.[2, 3] To date, only 53 cases of primary malignant melanoma of the larynx have been reported in the medical literature. Therefore, clinicopathologic features, treatment protocols, and prognostic factors are not clearly established in primary malignant melanoma of the larynx. Furthermore, pathologic and histologic criteria to discriminate primary from secondary lesions are not well defined. In this report, we describe a case of primary malignant melanoma of the larynx.


A 53-year-old white man presented to the outpatient clinic at Loyola University Medical Center, Maywood, Ill, with a primary complaint of diffuse swelling and redness extending from the right mid thigh to the right lower leg, which was diagnosed as deep venous thrombosis. Additionally, the patient revealed that he had suffered recurrent episodes of hoarseness and hemoptysis once or twice a week during the past 4 months. He had no other complaints or symptoms. The patient admitted smoking 20 to 40 cigarettes a day for more than 30 years. He was also an occasional drinker. All vital signs and laboratory tests were within normal range. Because of the upper respiratory symptoms, a computed tomographic scan and laryngoscopy of the larynx were undertaken; these tests demonstrated the presence of an exophytic mass arising from the left true vocal cord with no evidence of cartilage destruction or cervical lymphadenopathy (Figures 1 and 2). The mass was then totally excised by carbon dioxide laser and submitted for histopathologic evaluation.



On gross examination, a slate-gray to red piece of soft mucosal tissue measuring 1.3 cm in greatest dimension was identified. Histologic examination revealed infiltration of the subepithelium by malignant melanocytes containing cytoplasmic and nuclear brown pigment (Figure 3). Atypical mitotic figures were also present. Despite extensive ulceration, focally dispersed neoplastic melanocytes were identified within the epithelium. The tumor cells demonstrated pleomorphic nuclei and prominent nucleoli. Some areas of the tumor consisted of elongated, spindle-shaped cells, while others showed polygonal to epithelioid cells. Immunohistochemical stains were strongly positive for S100 protein, HMB-45, and vimentin. On the other hand, cytokeratin and iron stains were negative.


All clinical and radiologic studies evaluating the presence of a possible primary melanocytic lesion were negative. Therefore, a diagnosis of primary malignant melanoma of the larynx was established. Following the surgical excision, the patient refused all additional therapeutic modalities.


Primary mucosal malignant melanoma of the larynx is a rare neoplasm. Few articles have addressed the subject and published case reports are scarce. Therefore, every reported case is useful in defining the diagnostic, prognostic, therapeutic, and natural history criteria of the disease. In a recent article, Wenig[1] cited only 46 cases. He also included 4 cases from the files of the Armed Forces Institute of Pathology. Thereafter, 3 more cases were reported.[4-6] A primary origin of the tumor in a fourth case could not be established owing to the extensive ulceration of the lesion.[7] The authors in this particular report adhered strictly to the criteria established in 1953 by Allen and Spitz[8] for the diagnosis of primary malignant melanoma. According to Allen and Spitz, malignant melanocytes must be identified in the dermoepidermal junction or in the surface epithelium (ie, in situ component) to establish a diagnosis of primary malignant melanoma.[8] Several authors have firmly adhered to these criteria for the histopathologic classification of malignant melanoma of the larynx into primary and secondary types.[4, 5] However, some of the previously published literature either ignored these criteria or failed to document the junctional or in situ component due to extensive surface ulceration, which is frequently encountered in laryngeal malignant melanoma.[9, 10] In these reports, designation of the primary origin was based solely on clinical examination and radiographic findings, which showed no evidence of other coexisting malignant melanocytic lesions. The consideration of only clinical and radiographic findings for the diagnosis of primary malignant melanoma is refuted by some investigators owing to the fact that cutaneous malignant melanoma is capable of spontaneous regression after having metastasized.[11] On the other hand, the identification of melanocytes in the submucosal compartment and mucoserous glands of the larynx[12] led to the assumption that primary malignant melanoma could arise in the larynx not only from melanocytes present in the surface epithelium, but also from those present in the submucosa. Hence, the presence of a dermoepidermal junctional or in situ component is not a cardinal feature in establishing the diagnosis of a primary lesion.[1] In the present case, however, an in situ component was identified despite the extensive ulceration and the sparse areas of intact epithelium. Moreover, clinical and radiographic examinations failed to identify another malignant melanocytic lesion. Therefore, a diagnosis of primary malignant melanoma of the larynx was confirmed based on histopathologic, radiographic, and clinical criteria.

The diagnosis of laryngeal malignant melanoma is essentially dependent on histopathologic examination. Clinical and endoscopic evaluations are of very limited value. Slate gray, brown, or black pigmentation of the lesion could be a clue in some cases. Diligent microscopic examination of tissue sections stained with hematoxylin-eosin remains the mainstay for the diagnosis of malignant melanoma. However, some lesions are amelanotic and others demonstrate features similar to other malignant neoplasms of ectodermal, mesodermal, or hemopoietic origin. Therefore, immunohistochemical staining must be performed to establish and confirm the diagnosis of malignant melanoma.

The patient presented in this report refused further therapeutic interventions. The treatment of choice for mucosal melanomas of head and neck, including laryngeal lesions, is complete surgical excision. The incidence of regional lymph node metastases is relatively low[13]; therefore, elective neck dissection is not considered a routine practice. Postoperative radiation therapy could be useful in cases of mucosal malignant melanoma of the head and neck, but chemotherapy has not been found to be effective. Passive and active immunotherapy may be a promising adjuvant therapeutic modality for mucosal malignant melanoma in the near future.[14]

Despite all therapeutic efforts to control mucosal malignant melanoma of the larynx, the overall 5-year survival rate is very poor. In most cases, patients die of metastatic disease within 3 to 4 years. In fact, 80% of the patients with mucosal malignant melanoma of the larynx have metastatic lesions at presentation. Although several histopathologic features, for example, anaplasia, pleomorphism, degree of mitotic activity, and depth of invasion, are known to affect the outcome of cutaneous malignant melanoma, similar correlation has not been established in mucosal malignant melanoma of the head and neck owing to the rarity and unpredictability of these lesions.


[1.] Wenig BM. Laryngeal mucosal malignant melanoma: a clinicopathologic, immunohistochemical, and ultrastructural study of four patients and a review of the literature. Cancer. 1995;75:1568-1577.

[2.] Conley J, Hamaker RC. Melanoma of the head and neck. Laryngoscope. 1976;87:760-764.

[3.] Snow GB, van der Esch EP, van Slooten EA. Mucosal melanomas of the head and neck. Head Neck Surg. 1978;1:24-30.

[4.] Mattavelli F, Di Palma S, Guzzo M. Primary mucosal malignant melanoma of the larynx: case report and review of the literature. Tumori. 1995;81:460-463.

[5.] Duwel V, Michielssen P. Primary malignant melanoma of the larynx: a case report. Acta Otorhinolaryngol Belg. 1996;50:47-49.

[6.] Folz BJ, Niemann AM, Lippert BM, Hauschild A, Werner JA. Mucous membrane melanomas of the upper aerodigestive tract: an analysis of 34 cases [in German J. Laryngorhinootologie. 1997;76:289-294.

[7.] Szudrowicz Z, Dymek A. Malignant melanoma of the larynx. Pol J Pathol. 1995;46:255-256.

[8.] Allen AC, Spitz S. Malignant melanoma: a clinicopathological analysis of the criteria for diagnosis and prognosis. Cancer. 1953;6:1-45.

[9.] Vuori EEJ, Hormia M. Primary malignant melanoma of the larynx and pharynx. J Laryngol Otol. 1969;83:281-287.

[10.] Hussain SSM, Whitehead E. Malignant melanoma of the larynx. J Laryngol Otol. 1989;103:533-536.

[11.] Clark WH, From L, Bernardino EA, Mihm MC. The histogenesis and biologic behavior of primary human malignant melanoma of the skin. Cancer Res. 1969;29:705-727.

[12.] Goldman JL, Lawson W, Zak FG, et al. The presence of melanocytes in the human larynx. Laryngoscope. 1972;82:824-835.

[13.] Panje WR, Moran WJ. Melanoma of the upper aerodigestive tract: a review of 21 cases. Head Neck Surg. 1986;8:309-312.

[14.] Shaw PM, Sivanandham M, Bernik SF, Ditaranto K, Wallack MK. 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.

Accepted for publication June 28, 2000.

From the Departments of Pathology (Drs Amin, Husain, Nickoloff), and Otolaryngology (Dr Petruzzelli), Loyola University Medical Center, Maywood, Ill.

Reprints: Hesham M. Amin, MD, MSc, Department of Pathology, Loyola University Medical Center, 2160 S First Ave, Maywood, IL 60153.
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Author:Amin, Hesham M.; Petruzzelli, Guy J.; Husain, Aliya N.; Nickoloff, Brian J.
Publication:Archives of Pathology & Laboratory Medicine
Date:Feb 1, 2001
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