S2690 You Cannot Ignore Abdominal Dropsy: A Rare and a Fateful Presentation

Abstract

INTRODUCTION: Angiosarcoma (AS) is a rare soft tissue sarcoma known to be one of the aggressive malignancies which carry a poor prognosis with 5- year overall survival reported at <43%. AS mainly originates from the breast (35%) and skin (20%), followed by soft tissue (13%) involvement. Hepatic AS accounts for only 0.5%–2% of primary hepatic malignancies. CASE DESCRIPTION/METHODS: A 56-year-old male presents with abdominal pain and 20-pound weight loss for the past two months. His abdomen appeared tense and distended. Results showed a hemoglobin 6.1 gm/dL, platelet count 63 × 103 /mm3, total bilirubin of 3.2 mg/dL, ALP 315 IU/L, AST 305 IU/L, ALT 209 IU/L, and albumin of 2.6 gm/dL. A CT of the abdomen showed multiple hypodense lesions in the liver, and spleen with the peripheral enhancement of the contrast [Figure 1A] suggestive of metastatic lesions. Colonoscopy was normal and EGD demonstrated multiple erythematous nodular lesions in the gastric body [Figure 1B] and duodenum [Figure 1C], biopsies of which were inconclusive. A CT guided liver biopsy showed small aggregates of anastomosing vascular channels with highly atypical cells suggestive of AS [Figure 2A]. Tumour cells were positive for CD-34 [Figure 2B] and focally positive for Factor VIII [Figure 2C]. The patient deteriorated very rapidly and died 2 weeks later due to cardiac arrest. DISCUSSION: Hepatic AS is more commonly seen in males (3:1) with a median age of the presentation around the 5th- 6th decade. Use of anabolic steroids, oral contraceptives, exposure to radiation, and chemicals like arsenic, vinyl chloride, and thorium dioxide are certain risk factors for the development of hepatic angiosarcoma. Often patients report non-specific symptoms such as abdominal pain, anemia, weight loss, fever, fatigue, however, can present with jaundice and ascites in case of advanced disease. Rare but spontaneous rupture of the liver and intraabdominal bleeding can also occur in patients with hepatic AS. Tumor size, visceral tissue involvement, the presence of necrosis, and metastatic disease at the initial presentation are some of the poor prognostic factors. CT angiography is the preferred imaging modality. Pathological diagnosis is often required. Median survival is approximately 6 months without any treatment. Surgery can be considered if they are localized to a lobe. Chemotherapy can be offered for palliation in cases of advanced and multifocal disease. Because of a high recurrence rate, liver transplant is generally not recommended for hepatic AS.Figure 1.: A. (From top to bottom) CT of the abdomen showed multiple hypodense lesions in the liver. B. EGD demonstrated multiple erythematous nodular lesions in the gastric body. C. EGD demonstrated multiple erythematous nodular lesions in theduodenum.Figure 2.: A: Liver biopsy showed small aggregates of anastomosing vascular channels with highly atypical cells suggestive of AS. B: Tumour cells positive for CD-34. C: Focally positive for Factor VIII.