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The Oncologist, Vol. 13, No. 1, 79-81, January 2008; doi:10.1634/theoncologist.2007-0172
© 2008 AlphaMed Press

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Lung Cancer

Case Report: Dramatic Recovery of Lung Adenocarcinoma–Associated Dermatomyositis with Targeted Lung Cancer Therapy Alone

Yuan-Sheng Zanga, Qing-Yu Xiua, Zheng Fanga, Bing Lia, Tian-Bao Xiab

aDepartment of Respiratory Medicine, Changzheng Hospital, Second Military Medical University, Shanghai, China; bDepartment of Dermatology, No. 306 Hospital of the People's Liberation Army, Beijing, China

Key Words. Dermatomyositis • Corticosteroids • Lung cancer • Targeted therapy

Correspondence: Qing-Yu Xiu, 415 Fengyang Road, Huangpu District, Shanghai 200003, China. Telephone: 86-13816584620; Fax: 86-21-63610109(-73231); e-mail: doctorxiuqy{at}163.com

Received September 20, 2007; accepted for publication November 11, 2007.

Disclosure: No potential conflicts of interest were reported by the authors, planners, reviewers, or staff managers of this article.


   ABSTRACT
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 Footnotes
 Abstract
Case
 Discussion
 References
 
This case report details the sudden onset of severe dermatomyositis (DM) symptoms followed by rapid progression of adenocarcinoma of the lung and an obvious diminution of the primary tumor with the administration of lung cancer targeted drug therapy alone, followed by nearly complete disappearance of the DM symptoms, with no conspicuous improvement in the DM symptoms when using corticosteroids.


   CASE
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 Case
Discussion
 References
 
A 54-year-old nonsmoking woman presented with mauve edematous rash on the nose and knuckles beginning in June 2005. Eight months later, the rash, accompanied by mild itching and twinge, diffused to her whole face, trunk, and limbs (Fig. 1) in a symmetric distribution. Simultaneously, she experienced debilitating weakness, especially in her ability to grip with her hands, to lift her arms above her head, to walk, and to swallow. The Manual Muscle Test (MMT) revealed a Lovett 2 (poor) degree of strength in her limbs. An abnormal electromyogram of the limbs was also observed. After a biopsy of skin and muscle, dermatomyositis (DM) was diagnosed in May of 2006, and hence 30 mg/day prednisone was administered. Her weakness improved slightly during the first month, with no additional change in the subsequent days and no other change during the entire prednisone treatment duration. In June of 2006, she experienced cough, expectoration, and breathlessness. The patient discontinued the corticosteroid medication because she was told by a doctor of the local hospital that the corticosteroids might have induced infection.


Figure 1
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  		Figure 1. Pretreatment and post-treatment chest computed tomography (CT) images show a conspicuous reduction in the size of the tumor shadow (arrow) and an obvious regression of the bilateral pleural effusions, with corresponding obvious fading of the skin rashes. (A): Chest CT scan before gefitinib administration. (B): Chest CT scan after 1 month of gefitinib administration. (C): Image of the skin on the right upper arm before gefitinib administration. (D): Image of the skin on the right upper arm after 1 month of gefitinib administration.

 
After admission to our hospital in August of 2006, a chest computed tomography (CT) scan showed a tumor shadow in the lower lobe of the left lung accompanied by pulmonary closure of the lingual lobe, obstructive pneumonia in the lower lobe of the left lung, embolism in the right pulmonary artery trunk, pneumonia in the lower lobe of the right lung, lymphadenectasis of the mediastinum, pericardial effusion, and bilateral pleural effusions (Fig. 1). Cranial magnetic resonance imaging showed metastasis in the left frontal lobe. Radionuclide bones scan showed widespread metastasis. Cytological testing of the pleural fluid revealed adenocarcinoma. Elevated creatine kinase (CK, 400 U/l; normal value, 25–200 U/l), lactate dehydrogenase (LDH, 783 U/l; normal value, 230–460 U/l), and carcinoembryonic antigen (CEA, 936.6 ng/ml; normal value, 0–8 ng/ml) in blood were detected. Therefore, a clinical diagnosis of lung cancer complicated by DM was made and the lung adenocarcinoma was determined to be stage IV (T4N3M1). She was treated with 250 mg/day gefitinib starting in August of 2006. Five days later, the itching and twinge of the rash began to improve slightly. One month later, a chest CT scan showed a conspicuous reduction in the size of the tumor shadow and an obvious regression of the pleural effusions (Fig. 1), and also an obvious regression of the mediastinal lymph nodes. The radiological response was evaluated as a partial response. Decreased CK (309 U/l), LDH (659 U/l), and CEA (752.1 ng/ml) levels were detected. Meanwhile, a conspicuous improvement in the symptoms of itching and twinge of the rash and an obvious fading of the rash (Fig. 1) were observed. An MMT revealed a Lovett 3 (fair) degree of strength in both the upper and lower extremities. Three months later, a chest CT scan showed no further reduction in the size of the tumor shadow. The rash nearly disappeared completely and even lower CK (251 U/l), LDH (537 U/l), and CEA (112.2 ng/ml) levels were detected. An MMT revealed a Lovett 4 (good) degree of strength in the lower extremities and a Lovett 3 (fair) degree of strength in the upper extremities. The patient was still alive without relapse of the DM symptoms at the time that this article was submitted.


   DISCUSSION
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 Case
 Discussion
References
 
Lung cancer-associated DM has occasionally been described [1–5]. A combination of the administration of corticosteroids, which is considered to be the standard treatment of DM despite a lack of randomized studies [1, 6], and resection of the primary tumor or chemotherapy can improve the symptoms of lung cancer–associated DM [1–5]. However, no literature until now has mentioned the therapeutic efficacy of lung cancer–targeted therapy without the administration of corticosteroids, which may be the most important part of treating lung cancer–associated DM and even some other paraneoplastic syndromes.

Using the criteria set forth by Bohan and Peter in 1975 [7], a diagnosis of DM was made for our patient. Although lung cancer is occasionally associated with DM, small cell lung cancer and squamous cell lung carcinoma occur more frequently than other lung cancer histological types [8]. DM-associated adenocarcinoma of the lung is a very rare entity: only two cases were found by searching the MEDLINE database between the years of 1947 and 2006 [8–9].

As a histological type that is often late stage when diagnosed and resistant to most of the traditional chemotherapy drugs, adenocarcinoma of the lung usually exerts a heavy tumor load that is hard to reduce in the patient. Nevertheless, adenocarcinoma of the lung, especially in Asians, women, and nonsmokers, was recently found to be fairly responsive to a new lung cancer–targeted therapy drug, gefitinib. Gefitinib was therefore administered to the patient under discussion, who possessed all the features mentioned above. As a result, an obvious diminution in the primary tumor and a significant decrease in the CEA level, implying a remarkable reduction in the patient's tumor burden, were observed. In all the cancer-associated DM cases reported prior to this, corticosteroids were administered as the standard treatment for DM [1], whereas in the case under discussion, the administration of corticosteroids was accidentally discontinued by the patient herself before effective therapy targeted to the cancer itself started. A dramatic improvement in the cutaneous symptoms, significant decrease in the CK and LDH levels, and stepwise improvement in the MMT results were observed closely following the remarkable reduction in the patient's tumor burden. These facts may suggest the following points. (a) Reducing the patient's tumor burden, that is, controlling the cancer itself, may serve as the trigger to cure cancer-associated DM. Unfortunately, however, not all types of cancer patients can have their tumor burden reduced so easily. (b) In treating cancer-associated DM, if good control of the cancer itself is possible, corticosteroids could be, at least temporarily, removed from the therapeutic regimen, because the "standard treatment" of DM with corticosteroids lacks evidence from randomized studies and corticosteroids can sometimes induce infection, especially in cancer patients. But the question of whether or not the targeted therapy drug, gefitinib (which is an epidermal growth factor receptor inhibitor), has any effect on DM itself is yet to be answered, particularly if one considers that DM can be improved solely by drugs other than corticosteroids [10] and even without drugs [11].


   FOOTNOTES
 
Conception/design: Yuan-Sheng Zang, Qing-Yu Xiu, Zheng Fang, Tian-Bao Xia

Administrative support: Qing-Yu Xiu, Zheng Fang, Bing Li

Provision of study materials or patients: Yuan-Sheng Zang, Qing-Yu Xiu, Zheng Fang

Collection/assembly of data: Yuan-Sheng Zang

Data analysis and interpretation: Yuan-Sheng Zang, Qing-Yu Xiu, Zheng Fang, Bing Li, Tian-Bao Xia

Manuscript writing: Yuan-Sheng Zang

Final approval of manuscript: Qing-Yu Xiu


   REFERENCES
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 Footnotes
 Abstract
 Case
 Discussion
 References
 

  1. Gabrilovich M, Raza M, Dolan S et al. Paraneoplastic polymyositis associated with squamous cell carcinoma of the lung. Chest 2006;129:1721–1723.[CrossRef][Medline]
  2. Mori H, Habe K, Hakamada A et al. Relapse of dermatomyositis after 10 years in remission following curative surgical treatment of lung cancer. J Dermatol 2005;32:290–294.[CrossRef][Medline]
  3. Callen JP, Wortmann RL. Dermatomyositis. Clin Dermatol 2006;24:363–373.[CrossRef][Medline]
  4. Tsang KW, Lam WK, Ip M. Response of dermatomyositis co-existing with non-small cell lung cancer to chemotherapy. Respir Med 1998;92:788–790.[CrossRef][Medline]
  5. Fujita J, Tokuda M, Bandoh S et al. Primary lung cancer associated with polymyositis/dermatomyositis, with a review of the literature. Rheumatol Int 2001;20:81–84.[CrossRef][Medline]
  6. de Beukelaar JW, Sillevis Smitt PA. Managing paraneoplastic neurological disorders. The Oncologist 2006;11:292–305.[Abstract/Free Full Text]
  7. Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med 1975;292:344–347.[Medline]
  8. Weismann K. [Skin disorders as markers of internal disease. Paraneoplastic dermatoses.]. Ugeskr Laeger 2000;162:6834–6839.[Medline]
  9. Schulman P, Kerr LD, Spiera H. A reexamination of the relationship between myositis and malignancy. J Rheumatol 1991;18:1689–1692.[Medline]
  10. Chung L, Genovese MC, Fiorentino DF. A pilot trial of rituximab in the treatment of patients with dermatomyositis. Arch Dermatol 2007;143:763–767.[Abstract/Free Full Text]
  11. Jordan JR, Paruthi S, Pichardo RO et al. Spontaneously remitting dermatomyositis. J Eur Acad Dermatol Venereol 2004;18:495–498.[CrossRef][Medline]



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The Changing Face of Case Reports
Steven M Sorscher
The Oncologist Online, 30 May 2008  [Full text] 
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