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Year : 2018  |  Volume : 61  |  Issue : 4  |  Page : 636-638
Primary pulmonary melanoma: An unexpected diagnosis

1 Departments of Medical Oncology, Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, India
2 Department of Research, Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, India

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Date of Web Publication10-Oct-2018

How to cite this article:
Agrawal CR, Talwar V, Tayal J, Babu Koyyala VP, Goyal P. Primary pulmonary melanoma: An unexpected diagnosis. Indian J Pathol Microbiol 2018;61:636-8

How to cite this URL:
Agrawal CR, Talwar V, Tayal J, Babu Koyyala VP, Goyal P. Primary pulmonary melanoma: An unexpected diagnosis. Indian J Pathol Microbiol [serial online] 2018 [cited 2022 Dec 8];61:636-8. Available from:


Malignant melanoma is a malignant tumor arising from melanosomes (pigment-producing cells) of deeper layers of the skin or the eye.[1] Most common variety is cutaneous melanoma which constitutes around 90% of total melanoma burden, while extracutaneous locations are rare.[2] However, extracutaneous origin of melanoma from different body sites such as the esophagus, gastric, eyes, nasal cavity, and cervix has been previously described in literature. Primary lung melanoma is a very rare entity with <45 cases reported in literature so far and most of them do not even fulfill the published diagnostic criteria. These cases suggest the origin of melanoma in pulmonary tissue from residual melanoblasts. Sign and symptoms of any pulmonary tumor depend on the location of tumor in the endobronchial tree and primary pulmonary melanomas (PPMs), by virtue of their frequent endobronchial location, most commonly present with symptoms of cough, hemoptysis, and lobar collapse.[3] Due to its rarity, the typical clinical presentation, investigations, and management options are poorly understood. Herein, we report a patient who presented with a lung mass with no evidence of metastatic disease elsewhere in the body and diagnosed to have primary lung melanoma on the basis of histopathology and immunohistochemical (IHC).

A 48-year-old female patient, with no prior history of any cutaneous lesion or excision or fulguration of cutaneous or ocular lesion, presented to our department in March 2015 for the evaluation of cough, hemoptysis, and gradually worsening breathlessness of onset 3 weeks before presentation. Chest radiography showed round opacity of the right lung. Computed tomography (CT) of the chest and abdomen was done which revealed a well-demarcated hypodense mass lesion of size 5 cm × 4.2 cm in the right upper lobe with cystic changes mostly due to necrosis and homogenous enhancement [Figure 1]. CT- guided fine-needle aspiration cytology and biopsy of lesion were done which on histopathological examination showed alveolar parenchyma with tumor composed of large polygonal cells arranged in sheets with acidophilic cytoplasm [[Figure 2] Panel A]. A diagnosis of poorly differentiated tumor was made and a battery of IHC markers was applied for confirmation. IHC revealed tumor cells exhibiting intense cytoplasmic positivity for Melan-A and HMB 45 [[Figure 2] Panel B] and immunonegativity for p40, thyroid transcription factor 1, cytokeratin, and leukocyte common antigen. On the basis of these findings, tumor was diagnosed as malignant melanoma of the lung. Detailed dermatological examination was done which did not reveal any abnormal finding suggestive of melanoma as the primary focus. Whole-body positron emission tomography scan was advised, but the patient was not willing due to financial constraints. Considering the nature of disease, the patient was offered surgical resection, for which they opted out. A further discussion in the multidisciplinary tumor board led to the decision of administering single-agent carboplatin in April 2015. The patient developed hematological toxicity post first cycle, for which she was managed in the Intensive Care Unit. Further on, the patient was discharged against medical advice on patients request and died at her native place in May 2015.
Figure 1: Noncontrast computed tomography chest showing well-defined round hypodense lesion in the right lung without any lymphadenopathy

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Figure 2: Panel A: Sections showing alveolar parenchyma infiltrated with tumor composed of large polygonal cells, arranged in sheets (H and E, ×40). Panel B: Immunohistochemistry: Tumor cells show intense cytoplasmic positivity for Melan-A (H and E, ×40)

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Melanoma usually originates from the skin, but other organs such as eyes, nasal cavity, stomach, liver, and cervix have been reported as primary sites. PPM forms <0.01% of all lung tumors and is usually characterized by an aggressive clinical course.[4] Lung usually lacks melanocytes and therefore not a likely organ for development of melanoma, however, some likely explanations for the presence of melanoma in the lung are – (1) migration of benign melanocytes during embryogenesis, (2) melanocytes and melanocytic proliferation have been identified in the larynx and esophagus, (3) the larynx, esophagus, and the lungs all share a common embryologic origin suggesting possible migration of melanocytes, and (4) melanogenic metaplasia in the submucosa.[5] Because of its rarity, it comes very lower down in sequence in differential diagnosis of pulmonary masses and lack of any specific radiological feature for this rare entity further complicates the diagnosis. An aggressive search over PubMed, in different languages, identified not more than 45 cases reported so far in the last 60 years. Due to paucity of data and reliability of criteria based merely on the small series and case reports, the precise presentation, management, and outcome remain speculative.

Clinical presentation of PPM varies. It may present with diversity of clinical manifestations and different pulmonary locations as reported previously in literature. Most common presenting symptoms in majority of cases are cough and hemoptysis with features of postobstructive pneumonia, lobar collapse, and atelectasis due to frequent endobronchial location of tumor.[3] However, most important in the exact diagnosis of PPM is the exclusion of other possible primary site of melanoma like skin. It has been observed and reported by Harpole et al. in their series of 7564 cutaneous melanoma cases that 12% of patients can develop lung metastasis leading to diagnostic dilemmas.[6] In fact, many previously published reports and series lack the required diagnostic criteria and also exclusion of extrapulmonary primary site. Jensen and Egedorf proposed four clinical criteria required for the diagnosis of PPM which included (1) solitary lung mass or nodule; (2) typical histopathology confirmed by IHC and/or electron microscopy; (3) no prior history of melanoma or excision/fulguration of a cutaneous, mucus membrane, or ocular lesion unless the pathological examination explicitly rule out a melanoma; and (4) no demonstrable melanoma outside the chest at the time of diagnosis.[7] Our patient satisfied all four clinical criteria and lacked any extrapulmonary possible site of primary on detailed dermatological examination and imaging as suggested by contrast-enhanced CT chest and abdomen.

Histological assessment is considered the gold standard for the diagnosis of pulmonary melanoma, but it is very difficult to distinguish it from metastatic melanoma because of microscopic similarities. Approximately 5%–10% of patients with metastatic melanoma have primary melanoma of unknown origin.[8] To solve this dilemma, histological criteria for pulmonary melanoma were proposed by Allen and Drash which required three criteria – (1) junctional change with nesting of melanoma cells just beneath the bronchial epithelium, (2) invasion of bronchial epithelium by malignant cells in an area without epithelial ulceration, and (3) an obvious melanoma beneath the above-described changes.[9] Our patient's biopsy satisfied all three above-mentioned criteria with strong positivity with Melan-A and HMB 45. Based on clinical and histological criteria, a diagnosis of PPM was made and the patient was planned for surgery (pneumonectomy/lobectomy). Surgical intervention appears to be appropriate in early stages of PPM and form the cornerstone of therapy based on prior reports. Existing evidences suggest that lobectomy or pneumonectomy represents the best chance of long-term survival irrespective of lymph nodal involvement.[10] The role of adjuvant chemotherapy or radiotherapy is still unclear due to lack of robust evidences. Our patient was planned for surgical management; however, the patient refused due to financial constraints. A telephonic follow-up with the family revealed that the patient died due to the disease in May 2015.

Consent and privacy

A general informed consent was taken from the patient regarding sharing of clinical data for research purpose. All the patients' information in the manuscript is anonymised and only de-identified data is used.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Frenkel S, Hendler K, Pe'er J. Uveal melanoma in Israel in the last two decades: Characterization, treatment and prognosis. Isr Med Assoc J 2009;11:280-5.  Back to cited text no. 1
Chang AE, Karnell LH, Menck HR. The national cancer data base report on cutaneous and noncutaneous melanoma: A summary of 84,836 cases from the past decade. The American college of surgeons commission on cancer and the American cancer society. Cancer 1998;83:1664-78.  Back to cited text no. 2
Abul Y, Eryüksel E, Celikel C, Tosuner Z, Yazici Z, Karakurt S. Endobronchial metastasis of malignant melanoma presenting with dyspnea: Case report and literature review. Turk Klinikleri J Med Sci 2011;31:468-70.  Back to cited text no. 3
Wilson RW, Moran CA. Primary melanoma of the lung: A clinicopathologic and immunohistochemical study of eight cases. Am J Surg Pathol 1997;21:1196-202.  Back to cited text no. 4
Jennings TA, Axiotis CA, Kress Y, Carter D. Primary malignant melanoma of the lower respiratory tract. Report of a case and literature review. Am J Clin Pathol 1990;94:649-55.  Back to cited text no. 5
Harpole DH Jr., Johnson CM, Wolfe WG, George SL, Seigler HF. Analysis of 945 cases of pulmonary metastatic melanoma. J Thorac Cardiovasc Surg 1992;103:743-8.  Back to cited text no. 6
Jensen OA, Egedorf J. Primary malignant melanoma of the lung. Scand J Respir Dis 1967;48:127-35.  Back to cited text no. 7
Serna MJ, Vázquez-Doval J, Sola MA, Ruiz de Erenchun F, Quintanilla E. Metastatic melanoma of unknown primary tumor. Cutis 1994;53:305-8.  Back to cited text no. 8
Allen MS Jr., Drash EC. Primary melanoma of the lung. Cancer 1968;21:154-9.  Back to cited text no. 9
Seitelman E, Donenfeld P, Kay K, Takabe K, Andaz S, Fox S, et al. Successful treatment of primary pulmonary melanoma. J Thorac Dis 2011;3:207-8.  Back to cited text no. 10

Correspondence Address:
Chaturbhuj Ramanand Agrawal
Consultant, Departments of Medical Oncology, Rajiv Gandhi Cancer Institute, Rohini, New Delhi - 110 085
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_490_17

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