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© 2003 American Society for Clinical Oncology
Some Unusual Paraneoplastic SyndromesCASE 2. DIGITAL ULCERATION AS A PARANEOPLASTIC SYNDROME IN OVARIAN CANCERAcademic Medical Center, Amsterdam; Flevo Hospital, Almere; and St Anthonius Hospital, Nieuwegein, the Netherlands A 62-year-old woman presented in early November 1999 with a 12-week history of painful, pale fingers and toes of both hands and feet, worsening with exposure to cold, and cutaneous lesions on both hands. She also had an 8-week history of malaise, nausea, dizziness, diarrhea, unspecified weight loss, fatigue, and a periodically distended abdomen. There was no history of Raynaud’s phenomenon, vascular or connective tissue disease, or hyperlipidemia. Previous history was unremarkable. Physical examination revealed a pale, tired-looking afebrile woman with paronychia on digits two and four of both hands and feet and some bruising. Both the fingers and toes were pale, cool, and painful to touch. Peripheral pulses were present and symmetric. Livedo reticularis was visible on the ventral side of both legs. The abdomen was not distended or painful, and there was no shifting dullness. The physical examination was otherwise unremarkable. Laboratory results at that time were normal, except for mild thrombocytosis. In December 1999, while the analysis of dermal symptoms was still ongoing, she was diagnosed with pulmonary embolism arising from the deep venous thrombosis of the right femoral vein. Additional investigation, focused on malignancy, revealed a poorly differentiated adenocarcinoma of the right ovary, International Federation of Gynecology and Obstetrics classification stage IIIC, grade 3, which was subsequently treated with debulking surgery and six courses of chemotherapy. Evaluation of the skin lesions by immunofluorescence for immunoglobulin (Ig) G, IgA, IgM, and complement C1q and C3c were negative, as were tests for autoimmune disease, cryoglobulins, and paraprotein. Magnetic resonance imaging of the hands did not show osseous abnormalities. Biopsy of the dermis revealed no epidermal abnormalities or signs of inflammation, and there were no signs of vasculitis or tumor emboli. By exclusion of other possible diagnoses, we concluded that the skin condition was most likely a paraneoplastic syndrome.
Treatment with nifedipine did not stop progression of the cutaneous symptoms, which deteriorated even though a clinical remission of the ovarian cancer was demonstrated after surgery and the first courses of chemotherapy in March 2000 (Fig 1
Paraneoplastic dermal manifestations in ovarian cancer are uncommon.1,2 The physiopathology of most paraneoplastic syndromes remains elusive, and to date, no standard treatment has been defined. The distinct cutaneous paraneoplastic syndrome in our patient is extremely rare, and to our knowledge, only three similar cases have been reported.3–5 No specific diagnostic tools are available; therefore, the diagnosis of the dermatosis was confirmed by the clinical morphology of the lesions, by the temporal relationship with malignant disease, and by exclusion of other diagnoses.6 Unfortunately, biopsy is inconclusive,5 as was the case in our patient. Corticosteroids are mostly ineffective in these lesions,5 and therefore, in this patient, a symptom-directed policy was chosen with continuous intravenous infusion of the prostacyclin analog iloprost. Iloprost has vasodilating and platelet-inhibitory effects, and it is known to be effective in patients with peripheral arterial occlusive disease or with Raynaud’s phenomenon.7 Moreover, iloprost can produce significant healing of digital ulcers.8 Treatment of a cutaneous paraneoplastic syndrome with iloprost has not been described before. It is of interest to note that, although treatment of the malignancy was not sufficient to induce a remission of the dermal manifestations in our patients, iloprost administration could not achieve improvement without treatment of the tumor. EDITOR’S NOTE See also Iamandi C, Dietemann A, Grosshans E, et al: Unusual presentations of lung cancer. Case 3: Paraneoplastic digital necrosis in a patient with small-cell lung cancer. J Clin Oncol 20:4600–4601, 2002
REFERENCES
1. Ashour AA, Verschraegen CF, Kudelka AP, et al: Paraneoplastic syndromes of gynecologic neoplasms. J Clin Oncol 15:1272–1282, 1997 2. Sabir S, James WD, Schuchter LM: Cutaneous manifestations of cancer. Curr Opin Oncol 11:139–144, 1999[CrossRef][Medline]
3. Hawley PR, Johnston AW, Rankin JT: Association between digital ischaemia and malignant disease. BMJ 3:208–212, 1967
4. Freundlich B, Makover D, Maul GG: A novel antinuclear antibody associated with a lupus-like paraneoplastic syndrome. Ann Intern Med 109:295–297, 1988 5. Chow SF, McKenna CH: Ovarian cancer and gangrene of the digits: Case report and review of the literature. Mayo Clin Proc 71:253–258, 1996[Abstract] 6. Cohen PR, Kurzrock R: Mucocutaneous paraneoplastic syndromes. Semin Oncol 24:334–359, 1997[Medline]
7. Black CM, Halkier-Sorensen L, Belch JJF, et al: Oral iloprost in Raynaud’s phenomenon secondary to systemic sclerosis: A multicenter, placebo-controlled dose-comparison study. Br J Rheumatol 37:952–960, 1998 8. Wigley FM, Seibold JR, Wise RA, et al: Intravenous iloprost in the treatment of Raynaud’s phenomenon and ischaemic ulcers secondary to systemic sclerosis. J Rheumatol 19:1407–1414, 1992[Medline]
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Copyright © 2003 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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