malignant melanoma ( mm ) is a skin malignant tumor which derives from melanocytes and can originate in any part of the body that contains these cells .
primary extracutaneous mm is rare and can present in ocular , rectal , mucosal , under the nail , conjunctival , vaginal , urogenital , esophageal , and meningeal locations . in the respiratory system most cases are metastases .
treatment strategies of mm include surgery , radiotherapy , chemotherapy and the latest management option is biological treatment . surgical excision of the primary lesion can be curative only for patients with localized disease . in patients who present with disseminated disease , the treatment aims to extend survival and improve quality of life . in this article we present 3 cases of the respiratory system
malignant melanomas , two primary lung melanomas and one metastatic melanoma originated from vaginal mucosa .
a 69-year - old female was admitted to the department of pulmonology with a history of persistent cough and exertional dyspnea .
chest x - ray showed atelectasis of the right upper lobe and solid tumor in the same area . computed tomography ( ct ) revealed a large solid and cystic lesion in the upper right lobe expanding from the apex along the anterior chest wall .
additionally paratracheal , inferior tracheobronchial , sub- and supraclavicular lymph nodes were enlarged ( fig . 1 ) .
positron emission tomography ( pet ) scan showed no other malignancy except for findings in ct .
bronchoscopy exposed a darkly pigmented , endobronchial mass obstructing the right upper lobe bronchus , visually mimicking thrombus .
cytological examination revealed non - small cell cancer , but full histopathological examination resulted in the diagnosis of malignant melanoma .
immunohistochemistry of tissue biopsy tested positive for s100 protein , hmb45 , melanin a and negative for panck , ttf1 , ck7 , p63 and ck5/6 ( fig .
2 ) . the patient was qualified to adjuvant chemotherapy , unfortunately progression of the disease was observed and the patient died 6 months after diagnosis .
solid cystic mass in the upper pole of the right hilum a ) he stain : bronchial mucosa infiltrated by epithelioid tumor cells .
a 63-year - old male with a medical history of hypertension and diabetes mellitus presented in the emergency department with right - sided hemiparesis lasting for 2 days . computed
3 ) , chest x - ray revealed a round shadow in the middle zone of the left lung . for further assessment the patient was admitted to the department of internal medicine .
physical examination was unremarkable except for a right - sided hemiparesis , no skin lesions suggestive of skin cancer were found .
computed tomography of the chest showed solid tumor in the upper lobe of the left lung and one similar smaller lesion in the same lung ( fig .
due to disseminated lesions in the central nervous system and the right lung the patient was disqualified from surgery . at that moment , because of hemorrhagic changes in the brain radiotherapy was not indicated either .
treatment with systemic corticosteroids was introduced and improvement of hemiparesis was observed although the general condition of the patient gradually deteriorated . due to no available effective management further examinations were not performed . with the diagnosis of stage iv malignant melanoma in the stable clinical condition
lesion in the left parietal lobe with hyperdense fluid - fluid level , surrounded with white matter edema that could be suspicious for the brain metastasis with the hemorrhage male , 63-year - old , right - sided hemiparesis . computed tomography examination of the chest after i.v .
large rounded mass in the 3 segment of the left lung surrounded with ground - glass opacity .
a 50-year - old female after surgically treated mm of vaginal mucosa 7 years before and reoperation for local recurrence 4 years later presented with persistent cough . computed
tomography of the chest revealed 3 circular solid tumors ( 0.6 cm in diameter ) in the right lung and 2 similar lesions in the left lung ( fig . 5 ) .
positron emission tomography scan showed increased glucose metabolism in the same locations and additionally in the iliac lymph nodes .
in poland , mm is responsible for 1.7% cancer morbidity in men and 1.9% in women but these numbers have been still growing for the past three decades . at the time of diagnosis in approximately 80% of patients melanoma
is a localized skin lesion , 15% of patients present with regionally advanced disease and 5% with disseminated cancer [ 3 , 4 ] .
no data regarding occurrence of primary extracutaneous mm in our country are available . in the literature only 4 - 5% of all primary melanomas
most frequently they originate from the mucous membranes lining the respiratory , digestive , and genitourinary tracts or in the eyes as well as in the cerebral meninges . among lung tumors ,
frequently cited criteria for diagnosing primary melanoma of the lung proposed by allen and darsh and others in the 1960s include pathological ( 1  junctional change with a  dropping off or  nesting  of malignant cells containing melanin just beneath the bronchial epithelium ; 2  invasion of the bronchial epithelium by the melanoma cells in an area where the bronchial epithelium is not ulcerated ; 3  an obvious melanoma beneath the above described changes ) as well as clinical features ( 1  no history of a cutaneous , mucous membrane or ocular melanoma ; 2  solitary lung tumor ; 3  absence of any other tumor at the time of diagnosis ) [ 2 , 4 , 5 ] . presented case 1 meets the above criteria .
the patient had no history of neoplasm , pet scan excluded other malignancy locations except for the respiratory system , the lesion presented as solitary , dark pigmented tumor obstructing bronchus , histopathological staining was typical for melanoma cells .
case 2 can not be so unfailingly considered as primary lung melanoma because of lack of examinations certainly excluding other origin of mm , however solitary lung tumor with no pigmented skin lesions suggests that it could be primary disease .
a complete endoscopic or imaging diagnostic procedure in this case was not performed due to severe and gradually worsening general condition of the patient .
we included this case study in this article because of primary origin in vaginal mucosa which is quite rare .
it occurs in less than 1% of all malignant melanomas and less than 3% of all primary malignant tumors of the vagina .
contemporary treatment of malignant melanoma includes surgery , radiotherapy and systemic therapy with chemo- and immunotherapy . as there are no specific guidelines for primary lung mm recommendations for cutaneous melanoma
the first line treatment is surgical excision of the primary lesion with an oncologically adequate margin .
radiotherapy can be beneficial in particular locations ( head and neck ) as palliative management in order to prevent or delay hemorrhagic complications and neurologic symptoms . in presented case 2 ,
the patient already has hemorrhagic changes in central nervous system which disqualified him from this type of treatment .
adjuvant therapy with interferon -2b should be considered on a case - by - case basis .
although data show prolonged median survival [ 3 , 7 , 8 ] these drugs are still investigated . according to polish guidelines in 2013 patients with advanced melanoma
unfortunately most of these clinical trials exclude patients with brain metastases which require steroids , as in our case 2 .
results of treatment of disseminated mm also remain unsatisfactory , median survival is about 6 - 10 months .
primary lung melanoma should be considered in cases with no extrapulmonary disease which presents as a solitary lesion especially when bronchoscopy reveals dark pigmented and endobronchial mass .
 extracutaneous locations of primary malignant melanoma are rare . in the respiratory system 
 most melanomas present as metastatic tumors . for the diagnosis of primary lung melanoma 
 , strict histopathological and clinical criteria should be met . in this paper 
 we present three cases of malignant melanoma which showed in the respiratory system . 
 the first 2 case studies present primary lung melanomas , while the last one shows late lung metastasis of tumor originated from vaginal mucosa . 
 the treatment of choice for localized disease as well as single metastasis is surgical excision . 
Introduction
Case 1
Case 2
Case 3
Discussion
Conclusions
Disclosure