Journal of Clinical Oncology, Vol 19, Issue 23
(December), 2001: 4341-4343
© 2001 American Society for Clinical Oncology
Diagnostic Dilemmas in Oncology
Case 2. Dermatomyositis and Ovarian Cancer
Glen D. Raffel,
Ellen M. Gravallese,
Pascale Schwab,
Jeffery T. Joseph,
Stephen A. Cannistra
Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA
A 60-year-old woman was diagnosed in December 1998 with stage IIIC papillary serous ovarian cancer. She underwent optimal surgical debulking and was treated postoperatively with six cycles of carboplatin and paclitaxel through May 1999. At the end of first-line therapy, she was in clinical remission with a normal examination, normal computed tomography scan, and CA-125 level of 14. After 4 months she experienced marker-only relapse (CA-125 of 56) and was started on tamoxifen. Her CA-125 level continued to rise despite a 2-month trial of tamoxifen, during which time she developed a shawl-like, erythematous rash over her upper chest, back, and forehead (Fig 1). She was otherwise feeling well, with no complaints of muscle tenderness or weakness. Tamoxifen was discontinued in November 1999 due to progressive disease, and she was restarted on carboplatin and paclitaxel. After one cycle of treatment, the CA-125 level declined from 1,133 to 540, although the rash was unchanged. In December 1999, the patient developed a hypersensitivity reaction to carboplatin and was subsequently treated with paclitaxel as a single agent. Because of progression on paclitaxel, she was switched to gemcitabine and experienced several months of disease stability (although her rash persisted).
In October 2000, her CA-125 level began to rise, in association with exacerbation of her rash. In addition, she now complained of difficulty lifting heavy objects and climbing stairs, as well as generalized muscle soreness. Proximal muscle weakness was noted upon examination, and the serum creatine kinase level was found to be significantly elevated at 1,434 IU/L (normal range, 0 to 200 IU/L). The diagnosis of dermatomyositis was suspected, and a left deltoid muscle biopsy was obtained for confirmation. The muscle biopsy revealed a predominantly perimysial and perivascular lymphocytic infiltrate, with obliteration of several blood vessels (Fig 2 is a hematoxylin and eosin stain that shows an obliterated blood vessel in the center of the field, x400). Areas of acute muscle infarction were also found (Fig 3, x200), as evidenced by lack of reduced nicotinamide adenine dinucleotide–tetrazolium reductase staining in necrotic tissue (dark staining areas in Fig 3 represent viable muscle). These features were consistent with a histologic diagnosis of dermatomyositis. The patient was placed on prednisone, and over the following 4 weeks she experienced significant improvement in her muscle strength and normalization of her creatine kinase level. Nevertheless, her rash persisted after 3 months of corticosteroid therapy, and her tumor continued to progress despite treatment with liposomal doxorubicin.
Dermatomyositis is a rare paraneoplastic syndrome that may precede the development of malignancies such as lung, breast, or ovarian cancer by several months.1 A rash that occurs in a shawl-like distribution is characteristic of this disease, as is proximal muscle weakness and elevation of serum creatine kinase levels. The incidence of ovarian cancer developing in the setting of dermatomyositis was reported to be as high as 13.3% in one series.2 Although dermatomyositis often precedes the diagnosis of ovarian cancer, it may also present simultaneously with this malignancy.1-3 In contrast, the development of dermatomyositis after an established diagnosis of ovarian cancer, as demonstrated by our patient, seems to be less common. In a series of 12 patients with ovarian cancer and dermatomyositis, only one patient developed dermatomyositis after an initial diagnosis of ovarian cancer.3 Likewise, in a small series of seven patients with ovarian cancer and dermatomyositis, only two patients developed dermatomyositis after an initial diagnosis of ovarian cancer.2 Our patient demonstrates that dermatomyositis may develop in the setting of pre-existent ovarian cancer and that skin manifestations may precede clinically obvious muscle symptoms by several months. Such patients may experience improvement in muscle strength with corticosteroid therapy, despite persistence of skin rash and progression of their underlying neoplasm.
REFERENCES
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Sigurgeirsson B, Lindelof B, Edhag O, et al: Risk of cancer in patients with dermatomyositis or polymyositis. N Engl J Med 326: 363-367, 1992[Abstract]
2.
Cherin P, Piette J, Herson S, et al: Dermatomyositis and ovarian cancer: A report of 7 cases and literature review. J Rheumatol 20: 1897-1899, 1993[Medline]
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Davis MD, Ahmed I: Ovarian malignancy in patients with dermatomyositis and polymyositis: A retrospective analysis of fourteen cases. J Am Acad Dermatol 37: 730-733, 1997[Medline]
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