Paraneoplastic vasculitis with digital necrosis: a rare presentation of advanced ovarian cancer

Abstract

We report an interesting case of a rare presentation of ovarian cancer. A 64-year-old woman attended the Accident and Emergency department with a complaint of sudden onset of ‘‘painful, cold and blue fingers’’ (see Fig. 1). On further questioning, she revealed symptoms of myalgia and malaise. Several nail fold infarcts were also noticed. She was found to have a tense distended abdomen; however, no obvious masses were felt. She was referred to a Rheumatologist who carried out a battery of tests for autoimmune vasculitis, which were negative. A pelvic ultrasound revealed moderate ascites and a right sided complex adnexal cyst measuring 4.7 9 5.2 cm. Tumour marker serum CA-125 was raised at over 800 U/ mL and ascitic fluid sent for cytology showed cells suspicious of adenocarcinoma of the ovary. Subsequently, CT scan was carried out which revealed the adnexal mass with ascites and extensive omental as well as peritoneal seedlings. During the course of her admission, her vasculitis became more prominent. She was commenced on a short course of oral prednisolone therapy which resulted in symptomatic relief but did not resolve the vasculitis. The clinical presentation with sudden onset of ‘‘painful, cold and blue fingers’’, negative immunological tests and advanced ovarian malignancy led us to the diagnosis of digital vasculitis affecting the fingers secondary to a paraneoplastic phenomenon. She received six cycles of chemotherapy with carboplatin which resulted in good biochemical and radiological response, with a drop in CA125 to 55 U/mL. The symptoms of digital vasculitis also showed improvement and appeared to resolve completely by the time of third cycle of chemotherapy, further raising the suspicion that the paraneoplastic syndrome is caused by an ovarian tumour. The patient underwent debulking surgery and the vasculitis remained stable during the subsequent course of her treatment without relapse. The first report on the association of digital ischaemia and carcinoma was by O’Connor in 1884. He observed a correlation between breast cancer and gangrenous fingertips [1]. The prevalence of paraneoplastic syndromes associated with ovarian cancer is estimated in the order of only one in 1,000 [2]. With regard to the pathogenesis, induction of vasculitis by antibodies to tumour antigens is proposed as a likely method of paraneoplastic phenomenon [3]. The other pathogenesis could be digital vasospasm caused by sympathetic hyperactivity and digital artery obstruction due to increased blood coagulation associated with malignancy, hyperviscosity and arteritis [4]. Some paraneoplastic syndromes are also idiopathic. While investigating a patient with paraneoplastic vasculitis, apart from the haematological tests, immunological and serological investigations are of the utmost importance. Although the imaging studies required will depend on the individual presentation, the following tests may be of value: chest and sinus radiographs, echocardiograph, upper limb arteriography, ultrasound of abdomen and pelvis, erect abdominal X-ray, abdominal and pelvis CT. Biopsy of the digit or biopsy of the underlying malignancy should be considered [1, 5]. The aim of the treatment is to alleviate symptoms of ischaemia, prevent progression and to treat underlying cause. Although the symptoms of digital ischaemia may improve with medical therapies, definite benefits have been S. Robati K. Razvi K. Madhavan K. Gajjar (&) Department of Obstetrics and Gynaecology, Southend University Hospital NHS Foundation Trust, Westcliff on Sea, Essex, UK e-mail: gajjarkb@yahoo.co.uk