Case Presentation: We present a case of a 63 year old man who came in with a left sided neck mass and blue discoloration of his left upper extremity. Patient had left side limb discoloration for a month, he woke up with a large left sided neck mass that prompted him to come to the hospital. Exam, blood pressure was 135/84 mmHg, Heart rate 87 bpm and saturation was 98% on room air. He had left sided supraclavicular mass approximately 4×5 cm and axillary mass 1×1 cm; both the masses were non tender, soft and mobile. The Patient’s left limb was dusky in appearance with palpable peripheral pulses, muscle strength was 5/5 and sensations were intact. Initial laboratory workup revealed hemoglobin of 14.8 g/dl, white blood cell of 9.6 K/uL and platelets of 315 k/uL. D-dimer of 3.81 ug/mL, ESR of 74 mm/hr and CRP of 277.81 mg/L. On imaging his CT neck, upper extremity, chest and abdomen with contrast revealed underlying cervical nodal masses throughout the spinal accessory chain and at the neck base.The largest lymph node at the neck base measuring 3 cm. CT also revealed extensive thrombosis of the left internal jugular, brachiocephalic, left subclavian vein that extends into the left axillary vein. Innumerable enlarged retroperitoneal, para-aortic and pelvic adenopathy largest measuring upto 7.5 cm x 1.8 cm with an enlarged prostate.Further workup revealed Negative CMV IGM ab, Toxoplasma, TB quantiferon, Bartonella Henselae Ab, EBV IGM, cryptococcal serum antigen. elevated antithrombin III 125%. Normal protein C function 110%, protein S function 80%, anticardiolipin antibody negative, beta-2-glycoprotein. PSA >1000 ng/ml. Retroperitoneal lymph node biopsy confirmed metastatic prostatic adenocarcinoma. Patient was started on bicalutamide with transfer to oncology services.

Discussion: Unilateral upper or lower extremity discoloration is an uncommon presentation of any cancer. Cancer is a risk factor for thromboembolic disease, and patients with cancer are estimated to be around four times more likely to develop a thrombosis than a similar individual without cancer. Paraneoplastic vascular syndromes may present in various forms, such as digital gangrene, Raynaud phenomenon, and acrocyanosis. A thromboembolic mechanism could also explain the phenomenon, possibly seen in this case. In cases of paraneoplastic acrocyanosis, small vessel thrombosis due to increased blood viscosity and proliferation of cellular elements may be responsible for the skin changes. Although commonly seen in fingers, our case can have similar pathogenesis since it was painless with thrombosis of the entire left internal jugular, brachiocephalic, subclavian and axillary venous supply. Phlegmasia cerulea dolens has similar presentation as our case but presentation is painful whereas our case was painless.

Conclusions: Paraneoplastic vascular syndrome presenting as single painless limb discoloration is rarely seen. Vascular syndromes may be encountered in various clinical conditions, such as connective tissue diseases, thromboembolic conditions, idiopathy or in paraneoplastic conditions. Patients presenting with limb discoloration and lymphadenopathy should alert a physician to make appropriate investigations to exclude possible underlying malignancy in a timely fashion. Also, further studies need to conducted for patients presenting with painless discoloration of entire limb.

IMAGE 1: Figure 1: Limb discoloration; Left upper limb blue in color as compared to Right (depicted by white arrow)

IMAGE 2: Figure 2: CT Abdomen and Pelvis with multiple retroperitoneal lymph nodes posterior to the right renal artery displacing the IVC anteriorly- chain of lymph nodes measuring up to 7.5 cm x 1.8 cm.