Journal of Surgical Oncology 29:28-30 (1985)

Systemic Scleroderma and Small Cell Carcinoma of the Lung

DEBA P. SARMA, MD, AND THOMAS G. WEILBAECHER, MD

From the Department of Pathology, VA Medical Center and Louisiana State University Medical School, New Orleans

A 64-year-old man diagnosed to have systemic scleroderma for 1 year developed small-cell carcinoma of the lung. Six additional cases obtained from the literature describing small-cell carcinoma occurring in patients with scleroderma are reviewed.

KEY WORDS: scleroderma and cancer, small-cell carcinoma, scleroderma, scleroderma and lung cancer

INTRODUCTION

Whereas the association of malignancy with dermato- myositis is well known, the association of cancer with systemic scleroderma remains doubtful. Most common type of lung cancer reported among patients with sys- temic scleroderma is alveolar-cell carcinoma or adeno- carcinoma [Talbott and Barrocas, 19801. Small-cell carcinoma of the lung occurring in a patient with sys- temic scleroderma appears to be very uncommon. We describe such a case and review six other cases reported in the literature.

CASE REPORT A 64-year-old white man came to the hospital with a

1 -month history of shortness of breath and dysphagia . He had lost 40 pounds of weight over the previous year. One year prior to admission, the patient was diagnosed to have systemic scleroderma when he presented with tight- ness of skin and reduced range of movement of the fingers of both hands.

Physical examination revealed an emaciated man with tight, shiny, atrophic skin over the face, hands, fingers, and feet. Both hands (Fig. 1) showed flexion contractures of the fingers without ulceration, arthralgias, joint swell- ing, or soft-tissue calcification. A left hilar mass was noted on chest roentgenogram (Fig. 2). A biopsy of the left upper lobe bronchus revealed small-cell undifferen- tiated carcinoma (Fig. 3). A workup including various scans, radiographs, and bone marrow examination yielded no evidence of metastasis. Laboratory tests in- cluded an elevated antinuclear antibody (ANA) with a

titer of 1 : 1,200, showing a speckled immunofluorescent pattern, suggestive of scleroderma. Rheumatoid factor was absent. Results of other routine hematologic and chemical tests were not significant. Skin biopsy of the finger revealed marked dermal sclerosis consistent with scleroderma.

The patient was treated with multiple courses of com- bination chemotherapeutic agents and radiotherapy to the left hilar mass. He died 9 months after the diagnosis of lung cancer. There was no autopsy study.

DISCUSSION Table I summarizes the essential features of six cases

found in the literature and of our case from the present communication. Patients of both sexes were equally af- fected. Age varied from 30 to 77 years. Lung cancer was diagnosed at least 1 year after the onset of scleroderma, although some cancers were diagnosed as many as 20- 30 years later. In most of the cases the small-cell carci- noma was present in the right lung.

About 15 % of patients with dermatomyositis may de- velop a malignant tumor [Barnes, 19761, whereas the cancer rate is aobut 3 4 % among patients with sclero- derma [Tuffanelli and Winkelmann, 1961 ; Duncan and Winkelmann, 19791. A few authors [Zatuchni et al, 1953; Monti, 19731 believe that scleroderma predisposes a pa- tient to cancer. However, other authors are not convinced

Accepted for publication May 8, 1984. Address reprint requests to D. Sarma, MD, 1601 Perdido Street. New Orleans, LA 70146.

0 1985 Alan R. Liss, Inc.

Scleroderma and Lung Cancer 29

Fig. 1 . Dorsal view of both hands showing somewhat flexed taper- ing fingers.

Fig. 3 . Left bronchial biopsy showing small-cell carcinoma in the submucosa. H&E X 80.

of the association between scleroderma and cancer [Rich- ards and Milne, 1958; Tuffanelli and Winkelmann, 19611. In an exhaustive review of the topic of lung cancer in systemic sclerosis, Talbott and Barrocas [ 19801 state that development of lung cancer in patients with systemic scleroderma may be related to the high incidence of pulmonary fibrosis in such patients. They noted that 77 % of the reported lung cancers among the patients with systemic scleroderma were adenocarcinoma or bron- chiolo-alveolar carcinoma. This type of carcinoma is well known to occur in fibrotic or scarred lung. As for small-cell carcinoma of lung occurring in patients with scleroderma, the evidence or explanation of an intimate association is lacking. Haggani and Holti [1973] have speculated on such an association in their case report. From our review of the reported cases, it appears that most of the small-cell carcinomas of the lung have oc- curred in elderly patients and that the coexistence of scleroderma has been a coincidence.

Fig. 2. Chest radiograph showing left hilar mass.

Patient’s AuthorlYear agelsex

Hale et al 61, male

Richards et al 65, male

Ashba et al 30, female

119443

[1958]

[ 19651

30 Sarma and Weilbaecher

TABLE I. Scleroderma and Small-Cell Carcinoma of Lung

Interval between onset

-

of scleroderma and discovery of cancer

(years)

1

1

1

Haggani et al 55, female [I9731

Monti 77, female

Trotta et a1 55, female [ 19731

[I9821

Sarma et al 64, male

30

14

20

1

Comment

Small, asymptomatic small-cell cancer of right lower lobe found at autopsy

Small-cell cancer of right lung and mediastinum found at autopsy

Oat-cell carcinoma involving right lung and hilum with metastases to liver and thyroid found at autopsy: right hilar mass was noted 2 months before on x-ray

Right lower lobe of lung had the peripheral oat-cell carcina with metastases to hilar lymph nodes liver, spleen, bones, adrenal glands, and ovary

Small-cell cancer of right lung with regional lymph node metastasis

Small-cell cancer of right upper lobe with metastases to mediastinal lymph nodes, liver, bone

Small-cell cancer of left upper lobe with [ 19841 hilar involvement

ACKNOWLEDGMENTS Thanks to Ms. Karen Dunn for excellent secretarial

assistance. REFERENCES

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Hale CH, Schatzki R: The roentgenological appearance of the gas- trointestinal tract in scleroderma. Am J Roentgen01 Radium Ther

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