|Year : 2014 | Volume
| Issue : 3 | Page : 730-732
Paraneoplastic dermatomyositis associated with adenocarcinoma of the lung
Daliborka S Bursac, Danica S Sazdanic-Velikic, Aleksandar P Tepavac, Nevena M Secen
Department for Chemotherapy, Institute for Pulmonary Diseases of Vojvodina, Clinic for Pulmonary Oncology, Faculty of Medicine, University of Novi Sad, Put Doktora Goldmana 4, Sremska Kamenica, Serbia
|Date of Web Publication||14-Oct-2014|
Dr. Daliborka S Bursac
Department for Chemotherapy, Institute for Pulmonary Diseases of Vojvodina, Clinic for Pulmonary Oncology, Faculty of Medicine, University of Novi Sad, Put Doktora Goldmana 4, Sremska Kamenic
Source of Support: None, Conflict of Interest: None
The association of dermatomyositis and lung cancer has been recognized a long time ago. The case of a 57-year old patient with lung adenocarcinoma and dermatomyositis as the first sign of the illness is presented. The occurrence of dermatomyositis should be considered as a potential presentation of paraneoplastic syndromes, particularly in patients at risk for lung cancer.
Keywords: Dermatomyositis, lung cancer, paraneoplastic symptoms
|How to cite this article:|
Bursac DS, Sazdanic-Velikic DS, Tepavac AP, Secen NM. Paraneoplastic dermatomyositis associated with adenocarcinoma of the lung. J Can Res Ther 2014;10:730-2
| > Introduction|| |
Paraneoplastic syndrome is a group of signs and symptoms that are not related to the local spread of the primary tumor or its metastases and could be the first sign of a malignancy. ,
Dermatomyositis associated with a lung cancer has been described in many publications. It is believed to occur in 7-15% of all cancer patients. [2.3] The purpose of this paper is to the present a case of a patient with dermatomyositis as the first sign of adenocarcinoma of the lung.
| > Case Report|| |
A 57-year-old male patient was hospitalized with redness of the chest and upper extremities skin, weakness in the muscles of the upper, lower extremities, neck muscles and swallowing difficulties as confirmed by the physical examination of the patient [Figure 1]a and b.
Blood tests showed elevated transaminase, gamma glutamyl transferase and creatin kinase levels. The skin biopsy indicated the edematous papillary dermis, with minor changes, vacuolar degeneration of basal cells, subepidermal split and dilated blood vessels in the upper half of the dermis. The entire dermis is a perivascular mononuclear cell infiltrate as a sign of inflammation. The muscle biopsy material was obtained from the edematous interstitium and infiltrates of lymphocytes, suggesting polymyositis. The electromyography findings pointed to myopathic potentials and denervation in the muscle deltoideus activity.
The chest radiography obtained at the time of diagnosis revealed an infiltration in the region of the right hilum of about 5 cm in diameter. The chest computed tomography (CT) confirmed the presence of a tumor in the S6 segment on the right, sized 50 mm × 29 mm, with enlarged lymph nodes in the right hilum, about 14 mm in diameter [Figure 2]a and b.
|Figure 2: (a and b) Pre-operative chest X-ray and computed tomography scan shows an infiltratio in the right lung|
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Bronchoscopy demonstrated a normal endo bronchial anatomy with no endobronchial lesions at the level of the segmental bronchi. Transbronchial biopsies form Nelson's bronchi were non-diagnostic. Cytologic findings of the transbronchial aspiration from the right lobe carina confirmed non-small cell carcinoma. TNM evaluation was performed (brain CT, abdomen ultrasonography). Pulmonary function parameters pointed to a mild obstructive ventilation disorder with hyperinflation of the lung parenchyma and arterial blood hypoxemia.
Esophagogastroduodenoscopy showed cardiac insufficiency and chronic erythematosus gastroduodenitis elements. Corticosteroids (prednisone at a dose of 1 mg/1 kg/d) were administered as initial therapy for skin lesions and muscle weakness, but without a significant clinical improvement.
After an acceptable cardiac and pulmonary pre-operative risk assessment, the right pneumonectomy was performed via the standard right thoracotomy [Figure 3]a and b. The pathology revealed a tumor of the right lower lobe in the size of 6 cm × 4 cm × 4 cm with metastatic hilar and paratracheal lymph nodes.
|Figure 3: (a and b) Post-operative a complete withdrawal of changes in the chest and upper extremities skin|
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Post-operatively, a complete withdrawal of the skin changes was achieved with a reduction of the muscle fatigue feeling [Figure 4].
During 1 year follow-up, the patient remained in the good health condition, without symptoms of dermatomioyitis or local relapse of lung cancer.
| > Discussion|| |
The first report of dermatomyositis associated with stomach cancer, by Stertz, dates from 1916.  At the same time, Kankeleit reported dermatomioyitis associated with breast cancer.  There are controversial data on the connection between cancer and the occurrence of dermatomyositis. The association of dermatomyositis and cancer has been reported with variable frequency, but it is likely to be more common in elderly people.  Koh et al. reported that 17 of 75 (22%) patients with dermatomyositis had an associated malignancy.  Bohan et al. assessed 153 patients with dermatomyositis/polymyositis and found 8.5% of cancers, amounting to 19.2%. in men over 50 of age.  In a large scale review of the literature Andreev found that lung cancer is the most common malignancy associated with dermatomyositis. 
In their study, Fujita et al. investigated 24 patients who had lung cancer associated with dermatomyositis, and three patients had dermatomyositis/polymyositis. They found that dermatomyositis usually occurred with small cell and squamous cell carcinoma of the lung and that it was more common in smokers. The interval between the start of the two entities was usually less than 1 year. In this study dermatomioyitis occurred immediately before the diagnosis of lung cancer in the majority of cases. 
Pathophysiology of paraneoplastic syndromes is complex. A possible mechanism for the occurrence of dermatomyosisis in patients with malignancies has been hypothesized. Stone reports the changes in the extracellular matrix surrounding the tumor from which the various fractions of the depolymerized glycosaminoglycans are released into the circulation. The hypothesis is that these glycosaminoglycans may lead to changes in the extracellular matrix of non-tumoral tissue. 
When the tumor occurs, the body can produce antibodies with the aim of destroying tumor cells. Unfortunately, in some cases, these antibodies may cross-react with normal tissues and destroy them, which can lead to disorders that are manifested as a paraneoplastic syndrome. Otherwise, paraneoplastic syndromes may result from the production and release of physiologically active substances from the tumor, hormones, hormone precursors, various enzymes, or cytokines. ,
Diagnostic criteria for dermatomioyitis (Bohan and Peter)  are as follows:
- progressive symmetric muscle weakness of the inferior and superior extremity girdle with dysphagia (or not) or with respiratory muscle involvement
- muscle biopsy confirming myositis
- increase in the muscle enzyme serum levels
- Electromyogram abnormalities indicating a primary muscle damage
- characteristic skin lesions.
The presence of 3-5 symptoms confirms the diagnosis of dermatomyositis. 
If dermatomyositis appeared as a paraneoplastic syndrome, its prognosis depends on the prognosis of malignant diseases.
The combined corticosteroid therapy, considered to be the standard treatment for dermatomyositis, and resection of the primary tumor or the use of chemotherapy can improve the symptoms of lung cancer associated with dermatomyositis. ,,,,
In conclusion, paraneoplastic dermatomyositis may be the first sign of lung cancer (as it was in our case) or a relapse. After a surgical treatment of the primary tumor, there has been a reduction in dermatomyositis. It is recommended that a patient with dermatomyositis conducts a detailed diagnostic procedure for the detection of a malignancy and these patients should not be excluded from the treatment with a curative aim only because of the appearance of dermatomyositis.
| > References|| |
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Fujita J, Tokuda M, Bandoh S, Yang Y, Fukunaga Y, Hojo S, et al
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Koh ET, Seow A, Ong B, Ratnagopal P, Tjia H, Chng HH. Adult onset polymyositis/dermatomyositis: Clinical and laboratory features and treatment response in 75 patients. Ann Rheum Dis 1993;52:857-61.
Bohan A, Peter JB, Bowman RL, Pearson CM. Computer-assisted analysis of 153 patients with polymyositis and dermatomyositis. Medicine (Baltimore) 1977;56:255-86.
Andreev VC. Skin manifestations in visceral cancer. Curr Probl Dermatol 1978;8:1-168.
Stone OJ. Dermatomyositis/polymyositis associated with internal malignancy: A consequence of how neoplasms alter generalized extracellular matrix in the host. Med Hypotheses 1993;41:48-51.
Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med 1975;292:344-7.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]