J Gastrointest Canc (2007) 38:74–77 DOI 10.1007/s12029-008-9034-y
Spleen Rupture Due to Primary Angiosarcoma: A Case Report Ioannis M. Koutelidakis & Panagiotis Z. Tsiaousis & Basilios Th. Papaziogas & Aristeides G. Patsas & Stefanos K. Atmatzidis & Konstantinos S. Atmatzidis
Published online: 19 November 2008 # Humana Press Inc. 2008
Abstract A case of a 79-year-old female with rupture of the spleen due to primary angiosarcoma is presented. Symptoms were non-specific. Diagnosis was based on histology postoperatively. Primary angiosarcoma of the spleen is a very rare and aggressive neoplasm with a high metastatic rate and almost uniformly fatal. Due to small number of reported cases, there are no guidelines concerning adjuvant or palliative treatment or any beneficial protocols of chemotherapy or radiotherapy up to date. Splenectomy prior to rupture seems to have a positive impact on long-term survival. Keywords primary spleen angiosarcoma . spleen tumors . spleen rupture
Introduction Primary splenic angiosarcoma is the most common malignant primary non-lymphoid tumor, although its occurrence is rare. It is characterized by a high metastatic rate and a dismal prognosis. Langhans first identified this disease in 1879 [1] and from then on few studies have investigated it. To our knowledge approximately 240 cases have been reported worldwide with only a handful of small-volume I. M. Koutelidakis : P. Z. Tsiaousis : B. T. Papaziogas : A. G. Patsas : S. K. Atmatzidis : K. S. Atmatzidis 2nd Department of Surgery, Aristoteles University of Thessaloniki, “G. Gennimatas” General Hospital, Thessaloniki, Greece P. Z. Tsiaousis (*) 32, Katsimidi Str., 54638 Thessaloniki, Greece e-mail:
[email protected]
case series of 30 to 50 patients and even less cases of splenic ruptures secondary to primary angiosarcoma [2–14].
Clinical Presentation A 79-year-old female was admitted in the internal medicine department with anemia and symptoms and laboratory findings of digitalis intoxication. The admission laboratory data were: hematocrit 22.6% (MCV, MCH, and MCHC indexes within normal values), WBC 11,300/mm3 (79% polymorphs), platelets 188,000/mm3. Urinalysis, serum electrolytes, hepatic enzymes, and chest X-ray were not remarkable. Digoxin serum levels were five times above normal. Personal history included heart failure, hypertension, and osteoporosis. Operative history included appendectomy in childhood. Neither localizing symptoms nor abnormal findings on examination were reported. The abdomen was soft and no masses were palpated. Melena was not reported although a Mayer exam was positive. Upper GI endoscopy did not detect any source of bleeding. The patient underwent blood transfusion of 5 U of red packed cells within 48 h without remarkable response (Hct ranged from 22.6% to 25.1% maximum back down to 21.1%). An ultrasonography of the abdomen revealed the presence of fluid around the liver, the spleen, and in the area of Douglass. Moreover, a multimodal mass of the spleen was detected, 6.5 cm in diameter, with an increased echogenic rim around it. Doppler results showed this rim to be hypervascular. Cholelithiasis was also discovered. Abdominal computed tomography identified an enlarged spleen containing multiple heterogeneous complex masses that replaced the spleen parenchyma almost completely. Large quantities of fluid with density similar to blood
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Fig. 3 Sections of the resected spleen in the area of rupture showing its prominent replacement by irregular and cystic variably blood-filled vascular spaces and infarcts Fig. 1 Abdominal contrast-enhanced CT scan demonstrates multiple heterogeneous low-density masses in the enlarged spleen and large hepatic and perisplenic mass of varying densities secondary to hemorrhage
around the spleen, liver, and lower abdomen suggested hemoperitoneum (Fig. 1). The patient underwent urgent laparotomy that revealed hemoperitoneum from a spleen rupture presented in the upper pole (Fig. 2). After blood drainage, an urgent splenectomy was performed. Macroscopically, there were no other abnormal findings in the rest of the abdominal organs. The postoperative course was complicated with
Fig. 2 Resected spleen. The rupture can be seen in the upper pole (arrow)
severe atelectasia of the left lung. Intensive respiratory physiotherapy and early mobilization confronted this problem adequately. The patient was discharged on the tenth postoperative day. The pathology findings were consistent with primary angiosarcoma of the spleen. At gross examination and sectioning, the resected spleen weighed 376 g, measured 11×7×6 cm, and showed multiple well-demarcated nodules consisting of grey hemorrhagic tissue. Its parenchyma was in most places replaced by various-sized, irregular, occasionally blood-filled spongelike communicating spaces with fibrosis and necrosis (Figs. 3 and 4). Microscopically, newly formatted vascular spaces were lined by pleomorphic endothelial cells with irregular hyperchromatic nuclei and abundant eosinophilic cytoplasm (Fig. 5).
Fig. 4 Resected specimen after sectioning shows the tumor grossly enlarged, replacing the spleen almost completely. Areas of fibrosis and necrosis are present
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Fig. 5 Microphotography shows presence of sinusoidal vascular spaces lined by large pleomorphic tumor cells with irregular hyperchromatic nuclei and abundant eosinophilic cytoplasm. (Hematoxylin and eosin stain, original magnification ×20)
Discussion The term angiosarcoma currently encompasses cases of angiosarcomas and lymphangiosarcomas. Angiosarcomas of soft tissue are uncommon, and those of the spleen are rare [3]. Most primary splenic angiosarcomas arise from the endothelial lining of splenic blood vessels and are the most common non-hematolymphoid malignant tumors of the spleen [3]. It is among the rarest types of neoplasm, occurring at an annual incidence of 0.14–0.25 cases per million persons. Manifestation of symptoms occurs mainly in the sixth decade of life, although pediatric cases have been reported [4, 13]. Gender preference is not certain, although some studies present a slight predominance in men [2–4]. Angiosarcoma predominantly affects the skin and soft tissues (57%) and less frequently liver, breast, bone marrow, and spleen (4–6% of total) [2]. Unlike the primary hepatic form, there is no known association between the splenic form and occupational exposure to chemicals such as vinyl chloride, arsenic, and Thorotrast [15]. In the current case report, no history of such occupational exposure to chemicals could be sustained. Most common clinical findings include left upper quadrant pain and splenomegaly with occasional fever, weight loss, and anemia [3, 4]. Splenic rupture presenting with acute abdominal pain has been reported in 13–32% of patients, a manifestation that accelerates the disease and causes immediate life-threatening danger due to hemodynamic instability [2–14]. No fever was recorded in our patient preoperatively, but physical deterioration and anemia signs were profound.
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Metastases occur early in the natural history of the disease and are present in about 70–100% of cases. They spread hematogenously and most frequently involve the liver (70–80%), but may also involve the lungs, lymph nodes, bone, omentum, peritoneum, and GI tract [13]. There was no evidence of metastases in our patient at initial presentation. Imaging findings of splenic angiosarcoma vary. Several CT appearances have been described as splenic angiosarcomas. These include a low-density mass with peripheral enhancement, homogeneous low-density mass with no contrast enhancement, high-density mass with minimal enhancement, and multiple varying-sized heterogeneously enhancing masses in an enlarged spleen [16], as in our case. Cystic or necrotic areas may be seen within the mass. Thompson et al. report that MR imaging features include nodular hypointense masses on both T1- and T2-weighted images; large masses with increased signal intensity on both T1- and T2-weighted images that are likely related to subacute hemorrhage, as well as tumor necrosis; and areas of decreased signal intensity within the tumor, owing to chronic hemorrhage with hemosiderin deposition [17]. Ultrasound and angiography may be complementary and yield additional information [17]. Diagnosis of splenic angiosarcoma by fine-needle aspiration biopsy (FNAB) of the primary lesion has been reported by some authors [18, 19] but others suggest that it should be probably avoided due to the increased risk of bleeding complications, especially among patients with refractory thrombocytopenia and vascular splenic neoplasms [17]. Profoundly, FNAB cannot be considered in the emergency setting of a splenic rupture, as in our case. Definitive diagnosis requires histologic analysis following splenectomy with liver biopsy in the presence of metastatic disease. The microscopic appearance is quite heterogeneous, although a vasoformative component can be identified. Neuhauser et al. suggest that immunohistochemical studies with markers of vascular and histiocytic differentiation may be helpful in confirming the diagnosis [3]. No signs of metastatic liver disease were present in our patient at the time of the splenic rupture, while the microscopic appearance of the excised enlarged spleen was characterized by multiple fibrotic and necrotic vasoformative components. Splenic angiosarcoma is known to be a highly lethal disease with a mean survival rate of 10.3 to 14.4 months [20]. The survival is only 4.4 months after the development of splenic rupture [5]. Early diagnosis with prophylactic splenectomy has a favorable survival rate in comparison with splenectomy after rupture as Montemayor et al. report (14.4 months vs. 4.4 months, respectively) [21]. Due to the rarity of splenic angiosarcomas, no phase II or III trials have been conducted exclusively for this
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disease, making it difficult to predict response rates to adjuvant chemotherapy thus far, as Castaldi et al. point out [12]. Until studies are conducted, no conclusion can be drawn regarding the effect of chemotherapy on survival. Multiagent chemotherapy is the modality of choice, while the use of radiation is not favored, unless it offers symptomatic palliation of painful bone metastases [12]. Our patient denied the neo-adjuvant chemotherapy that was suggested to her.
Conclusion We report a case of a very rare primary neoplasm of the spleen, an angiosarcoma, the manifestation of which was life threatening. The patient was admitted with anemia and no signs of acute abdomen. The subsequent deterioration of the patient’s clinical status and the US and CT findings of hemoperitoneum led the patient to the operating room for an emergency laparotomy and splenectomy. Splenic angiosarcoma is an aggressive neoplasm with dismal prognosis. Clinical diagnosis demands a high index of suspicion and should be considered in patients with splenomegaly and microangiopathic anemia but without evidence of lymphoma or leukemia. One third of cases are presented by splenic rupture and subsequent hemorrhagic ascites. Early diagnosis following splenectomy raises survival rates substantially. Due to small number of reported cases, there are no guidelines concerning adjuvant or palliative treatment or any beneficial protocols of chemotherapy or radiotherapy up to date.
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