Maria T. Castaldi, Assistant Professor of Surgery, Department of Surgery, Albert Einstein College of Medicine, Bronx, NY, Associate Director, North Central Bronx Hospital, Bronx, NY; Nicole B. White, Chief Resident, Department of Surgery, Albert Einstein College of Medicine, Bronx, NY; Laurie J. Kirstein, Resident, Department of Surgery, Albert Einstein College of Medicine, Bronx, NY; Lawrence S. Bizer, Chief of Surgery, Department of Surgery,
Maria T. Castaldi, MD
Assistant Professor of Surgery Department of Surgery Albert Einstein College of Medicine Bronx, NY Associate Director North Central Bronx Hospital Bronx, NY
Nicole B. White, MD
Chief Resident Department of Surgery Albert Einstein College of Medicine Bronx, NY
Laurie J. Kirstein, MD
Resident Department of Surgery Albert Einstein College of Medicine Bronx, NY
Lawrence S. Bizer, MD
Chief of Surgery Department of Surgery North Central Bronx Hospital Bronx, NY
Qi Wang, MD
Attending Pathologist Department of Pathology North Bronx Health Care Network Bronx, NY
Primary splenic angiosarcoma is al?most as rare as simultaneous, spontaneous rupture of the liver and spleen. The authors report the first case of presumed metastatic primary splenic angiosarcoma to the liver presenting as simultaneous rupture of the liver and spleen. The overall prognosis is poor for primary splenic angiosar-coma and is significantly worsened when it presents as an acute surgical emergency. Simultaneous, spontaneous rupture of the liver and spleen is rare. A review of the medical literature from the past 50 years reveals only three cases?two reported in association with systemic amyloidosis and one caused by an acute immunopathologic reaction of unknown origin.1,2 A few cases of iatrogenic, nontraumatic rupture of liver and spleen have been reported.3,4 These were in association with streptokinase infusion for treatment of deep venous thrombosis. Simultaneous traumatic rupture of the liver and spleen is well recognized. Causes include blunt trauma sustained during motor vehicle accidents and during cardiopulmonary resuscitation (with or without the use of thrombolytics).5 We report the first case, to our knowledge, of simultaneous, spontaneous rupture of the liver and spleen associated with primary angiosarcoma of the spleen in the ab?sence of trauma, iatrogenic injury, or other systemic disease.
A 43-year-old man in previously good health was brought by ambulance to North Central Bronx Hospital's emergency department because of severe epigastric pain. The patient reported a 2-month history of abdominal pain, mostly in the left upper quadrant and epigastrium, which was dull in character. The patient had been seen in three different emergency departments over the past 2 months with similar symptoms and was given a presumptive diagnosis of peptic ulcer disease.
One hour before admission, his pain suddenly increased in severity, becoming sharp and radiating to the right scapula. It was accompanied by an episode of nonbilious emesis. The patient's bowel function was normal, and he reported no fever or chills, associated symptoms, weight loss, or trauma. On arrival to the emergency department, he was diaphoretic and had a blood pressure of 80/0 mm Hg, heart rate of 132 beats per minute, and a respiratory rate of 40 breaths per minute. His abdomen was distended, quiet on auscultation, and diffuse tenderness was eli?cited on palpation. Rectal examination was normal. Fluid resuscitation was initiated and an emergency surgical consultation was obtained.
Laboratory values showed a white blood cell count of 13.5 x 103/?L with 71% granulocytes, a hemoglobin level of 5.9 g/dL, a hematocrit of 18%, and a platelet count of 60,000/mm3. Red blood cell indices demonstrated normochromic, micro?cytic anemia. Liver function tests, coagulation profile, and amylase and lipase levels were all within normal limits. Arterial blood gas analysis showed the following: pH, 7.26; PaCO2, 28 mm Hg; PaO2, 135 mm Hg; bicarbonate, 12 mmol/L; O2 saturation, 99%; and base ex?cess, ?13 mmol/L. An upright chest radio?graph showed no free air, and an abdominal radiograph was noncontributory. Focused abdominal ultrasonography for trauma revealed free fluid in the peritoneal cavity (Figure 1). A diagnostic peritoneal lavage was performed and there was return of gross blood. The patient was immediately taken to the operating room for an ex?ploratory celiotomy.
Operative findings?On entering the peritoneal cavity, 4 L of blood were evacuated. All quadrants were packed and the left upper quadrant was explored. The spleen was enlarged and actively bleeding. The entire splenic parenchyma was replaced by bleeding cystic masses. A splenectomy was performed. Next, attention was turned to the right upper quadrant. The liver was also enlarged, with multiple hemorrhagic cysts, and active-ly bleeding from a ruptured cyst in the left lobe. Bleeding vessels were suture-ligated. Hemostasis was achieved and the patient's hemodynamics normalized. On further exploration of the abdomen, the kidneys and pancreas were found to be free of cystic disease. The patient re?ceived 7 L of crystalloid, 10 units of packed red blood cells, 14 units of platelets, and 7 units of fresh frozen plasma. The estimated blood loss was 6 L. Urine output was adequate throughout the operation.
Postoperative course?The patient's postoperative recovery was uneventful. He was extubated on postoperative day 1 and resumed a regular diet and had normal bowel function by postoperative day 3. He was discharged to home on postoperative day 4, with follow-up in the surgical clinic. A postoperative computed tomography (CT) scan of the abdomen showed findings consistent with splenectomy, and multiple hypodense hepatic lesions, presumed to be metastases (Figure 2). The patient underwent single-agent chemotherapy with paclitaxel, 50 mg/m3 total, as decided by the department of oncology.
Pathologic findings?Grossly, the spleen was markedly enlarged. It weighed 545 g and measured 13.5 x 8 x 4.2 cm. On the cut surface, the parenchyma was completely replaced by the tumor, which was partially cystic and extremely hemorrhagic (Figure 3). Microscopically, the neoplasm showed different growth patterns, ranging from anastomosing vascular channels to solid areas (Figure 4). The endothelial nature of the tumor cells was documented by the immunoreactivity for CD34. The tumor cells were not particularly pleomorphic but the pattern of growth was very complex. These findings indicate splenic angiosarcoma (malignant hemangioendothelioma).
Primary splenic angiosarcoma is a rare neoplasm with an extremely poor prognosis. It was first reported in the literature by Langhans in 1879.6 Since then, fewer than 100 cases have been documented. The pathogenesis is unknown. The association of arsenic, thorium oxide (Thorotrast?), and vinyl chloride with hepatic angiosarcomas led to speculation of their involvement in splenic angiosarcoma; however, this hypothesis has not been supported by the literature.7 There is no true prototype for patients with splenic angiosarcoma. The average age at presentation is around 50 years, with an equal incidence in men and women.
Two studies showed left-sided abdominal pain and splenomegaly were present in 50% to 75% of patients with splenic angiosarcoma.8,9 Eighty percent of pa?tients had anemia, and for 10%, weight loss was the only symptom.7,9 Spon?tan?eous rupture of the spleen is a potentially fatal presentation. According to various reports, about 30% of primary splenic angiosarcomas present with rupture, and 50% of these are fatal.6,8,9 Metastases are found in 80% of patients and usually spread by the hematogenous route to the liver, lungs, lymph nodes, and bones.10 In a study of 28 cases, metastases developed in all of the patients during the course of their disease.9 Twenty-six patients died despite aggressive chemo?therapy. Only two were alive at 8- and 10-year follow-up.9 The prognosis of primary splenic angiosarcoma is extremely poor. Most patients have a fulminant clinical course with a mean survival rate of 10.3 to 14.4 months.11 The mean survival rate decreases to 4.4 months when there is spontaneous splenic rupture.7 Be?cause angiosarcomas are rare, no Phase II or III trials have been conducted exclusively for this disease, making it difficult to predict response rates to chemotherapy. Until studies are conducted, no conclusions can be drawn regarding the effect of chemotherapy on survival. Multiagent chemotherapy is the modality of choice and the use of ra-diation is not favored, unless it offers symptomatic palliation of painful bone metastases.
We chose paclitaxel as the che?mo?thera?peutic agent for our patient because of its use in the treatment of angiosarcoma of the scalp and face.12 The antiangiogenic and apoptotic properties of paclitaxel may contribute to its efficacy in angiosarcoma of other sites. Although there are no data for paclitaxel treatment of an?giosarcoma of the spleen and liver, data extrapolated from an?giosarcoma of other sites allow paclitaxel to be considered acceptable therapy with good palliative effects thus far.
Spontaneous, simultaneous rupture of the liver and spleen is rare, as is primary splenic angiosarcoma. Although splenic rupture associated with this neoplasm has been reported in 30% of cases, this is the first case report of spontaneous rupture of the liver and spleen caused by primary splenic angiosarcoma with presumed metastasis to the liver.
We thank Dr. J. Rosai at Impath Laboratories, New York City, with whom we consulted on this case.
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