Skip to main content

Spontaneous rupture of primary splenic angiosarcoma: a case report and literature review

Abstract

Background

Primary angiosarcoma of the spleen is a rare mesenchymal malignant tumor of vascular origin often with a poor prognosis, due to its high metastatic potential. This disease often presents with atraumatic rupture and lethal hemorrhage.

Case presentation

We report a case of a 65-year-old man who presented with abdominal pain, anemia, thrombocytopenia, and palpable abdominal mass with unstable blood pressure. Laparotomy revealed a huge actively bleeding spleen, thus splenectomy was performed. Some liver metastasis foci were also found during the procedure. Histopathology diagnosis of the removed spleen was primary splenic angiosarcoma. The patient was discharged on the 10th day post operation with no complication.

Conclusions

Splenic angiosarcoma should be considered one of the differential diagnoses in patients with spleen parenchymal lesions. Definitive diagnosis requires laparotomy followed by splenectomy. In the majority of the patients with spleen angiosarcoma, metastatic diseases have already occurred at the time of laparotomy, so splenectomy is an approach more for diagnostic purpose rather than curative purpose.

Background

Primary angiosarcoma of the spleen is an extremely rare malignancy, the pathogenesis of which is unknown, with high metastatic potential and a very poor prognosis. This aggressive disease usually presents in adults in their sixth to seventh decade. The reported median survival rates range from 4.4 to 14 months[1, 2]. These mesenchymal tumors can easily be neglected and splenic rupture is the most frequently manifestation at the time of diagnosis. We report a case of spontaneous splenic rupture due to angiosarcoma in a 65-year-old man.

Case presentation

A 65-year-old man was admitted with diffuse abdominal pain and distention. The pain was constant and dull, and started 2 hours prior to admission with no obvious inducible factor. The patient’s past medical history was unremarkable except mild weight loss during the recent 5 months. Physical examination revealed a palpable abdominal mass in the left upper abdominal quadrant with tenderness, and the patient’s hemodynamic condition was unstable with systolic arterial blood pressure only 85 mmHg and heart rate 110 beats/minute. After initial fast fluid resuscitation (1,000 ml hetastarch 130/0.4 NaCl), the hemodynamic status of the patient became stable. Peripheral blood count revealed anemia (hemoglobin 10.0 g/dl) and thrombocytopenia (platelets 90 × 109/l), but it did not reach disseminated intravascular coagulopathy. Results of other laboratory examinations had not been reported to us before the operation due to the emergency. Abdominal ultrasonography showed an enlarged spleen filled with irregular nodules, multiple hepatic solid lesions and moderate accumulation of peritoneal fluid. Abdominal paracentesis was performed with blood fluid aspirated from the peritoneal cavity. These findings were further confirmed by an abdominal computed tomography scan (Figure1), demonstrating a heterogeneous, low-density signal within the splenic parenchyma, with variable degrees of contrast enhancement as well as intra-abdominal hemorrhage originating most possibly from the spleen. Meanwhile, there were multiple low-density lesions with enhanced rim in the liver.

Figure 1
figure 1

Enlarged spleen completely replaced by low-density tumor tissue and multiple hypervascular metastases in liver. Contrast-enhanced image in the portal venous phase computed tomography scan showing the enlarged spleen completely replaced by tumor tissue with low density and multiple hypervascular metastases in the liver with sizes varying from 5 mm to 2 cm. Some free fluid around the liver and spleen was also found, indicating intraperitoneal hemorrhage.

Laparotomy revealed a huge spleen actively bleeding and an abnormal liver with several metastatic foci. We did observe a fissure of the capsule of the spleen with a size of 6 cm (length) × 2.5 cm (depth) in the lower pole of spleen. Two liters of blood had been collected from the peritoneal cavity. Splenectomy was performed. During the procedure, the patient received 4 units of concentrated red blood cells and 2 units of fresh frozen plasma.

The spleen was 1,650 g in weight, 19 cm × 16 cm × 11 cm in size, with nodular appearance and bleeding. The pathology diagnosis of the excised spleen was angiosarcoma originating from the spleen (Figure2). Immunohistochemical staining was positive for vimentin (Figure3), CD31 (Figure4), CD34 (Figure5), factor VIII (Figure6), a1ACT (Figure7) and SMA (Figure8), and was negative for lysozyme (Figure9). The Ki-67 proliferation index was less than 10% (Figure10).

Figure 2
figure 2

Histopathologic findings of the spleen angiosarcoma. Spindle tumor cells have replaced the normal red and white pulp in the spleen, whereas ecstatic vascular spaces lined with hypertrophied endothelial cells are apparent.

Figure 3
figure 3

Specimen stained positive for vimentin.

Figure 4
figure 4

Specimen stained positive for CD31.

Figure 5
figure 5

Specimen stained positive for CD34.

Figure 6
figure 6

Specimen stained positive for factor VIII.

Figure 7
figure 7

Specimen stained positive for a1ACT.

Figure 8
figure 8

Specimen stained positive for SMA.

Figure 9
figure 9

Specimen stained negative for lysozyme.

Figure 10
figure 10

Ki-67 proliferation index less than 10%.

Discussion

Angiosarcoma originated from the spleen is an extremely rare malignant tumor, which affects 0.14 to 0.25 per million people[3]. People at any age may develop this disease. The presentation age is between 14 months and 89 years. This neoplasm shows a slight male predominance[3–5]. We reviewed related literatures and summarized the data (Table 1).

Table 1 Data of the published literatures regarding splenic rupture secondary to splenic angiosarcoma

Although the pathogenesis of this tumor is not clear, ionizing radiation, arsenic, vinyl chloride and chemotherapy for lymphoma have been implicated as causative factors[18, 19], and it has been reported that the angiosarcoma requires the existence of a benign pathology such as hemangioma or hemangioendothelioma[18, 20]. However, there was no evidence of any of these factors involved in our case.

Clinical presentation of splenic angiosarcoma is variable. Left upper abdominal pain is the most common symptom[5]. Other complaints include fatigue, weight loss, and anorexia. Splenomegaly is the most common physical examination finding[5]. Hepatomegaly and left upper quadrant mass are other two common findings. The most serious manifestation is splenic rupture, which often leads to fatal hemorrhage[21].

Laboratory examination can find cytopenia, leukocytosis, thrombocytosis and elevated erythrocyte sedimentation rate. Anemia and thrombocytopenia are presented in more than 50% cases[3, 11].

Imaging examinations are helpful for the differential diagnosis, although lacking accuracy. The most common ultrasonography findings are splenomegaly and ill-defined solid and cystic masses in the spleen with heterogeneous echotexture[22, 23]. The areas of hemorrhage and necrosis within the tumors are frequently shown as cystic areas. Increased blood flow may be detected in the solid part of the masses on color Doppler images. Computed tomography may reveal an enlarged spleen with hypo-attenuating or hyper-attenuating areas with punctate or massive calcification on plain computed tomography scan (without contrast agent infused)[24, 25]. Areas with hyper-attenuation are likely to reflect acute hemorrhage. On contrast-enhanced computed tomography scans, the tumors may exhibit peripheral or heterogeneous enhancement similar to that of hepatic cavernous hemangiomas[26, 27]. On magnetic resonance imaging, ill-defined nodular lesions with low or high signal intensity may be seen on both T1-weighted and T2-weighted images. High signal intensity on both T1-weighted and T2-weighted images is related to subacute hemorrhage or tumor necrosis, and low signal intensity is related to chronic hemorrhage or fibrosis within the tumor[28].

Biopsy is contraindicated in splenic angiosarcoma because of high risk of rupture. Histologic studies can therefore only be made after splenectomy. Microscopic examinations reveal freely anastomosing papillary and classical characteristic vascular channels lined by masses of endothelial cells[4]. Immunohistochemically, at least two vascular proliferation markers (CD31, CD34, and factor VII) plus at least one histiocytic differentiation marker (lysozyme and/or CD68) are required to makes the diagnosis[5].

This tumor has a high incidence of early metastasis, as reflected in the reported rates of between 69% and 100%, with the most common sites being in the liver, lungs, bones or bone marrow, lymph nodes, gastrointestinal tract, brain and adrenal glands[3, 5, 29]. Our patient, however, had metastases in the liver.

Our case died 6 months after the surgery. Primary splenic angiosarcomas are very aggressive neoplasms, with a median survival of 5 months irrespective of treatment. Neuhauser and colleagues reported that 93% of patients died with disseminated tumor within 29 months[5]. Early diagnosis with splenectomy can result in a much more favorable survival rate[3, 21], but it is very difficult.

Conclusions

Splenic angiosarcoma is very rare but has a poor prognosis. It may cause secondary spontaneous rupture, resulting in fatal hemorrhage, which should be paid sufficient attention. Immediate surgery is necessary but the outcome is still unsatisfactory. Attempts should be made to improve the outcome by developing new techniques.

Consent

Written informed consent was obtained from the patient for publication of this report and any accompanying images.

References

  1. Buckner JW, Porterfield G, Williams GR: Spontaneous splenic rupture secondary to angiosarcoma. J Okla State Med Assoc. 1990, 83: 211-213.

    PubMed  Google Scholar 

  2. Montemayor P, Caggiano V: Primary hemangiosarcoma of the spleen associated with leukocytosis and abnormal spleen scan. Int Surg. 1980, 65: 369-373.

    CAS  PubMed  Google Scholar 

  3. Falk S, Krishnan J, Meis JM: Primary angiosarcoma of the spleen. A clinicopathologic study of 40 cases. Am J Surg Pathol. 1993, 17: 959-970. 10.1097/00000478-199310000-00001.

    Article  CAS  PubMed  Google Scholar 

  4. Valbuena JR, Levenback C, Mansfield P, Liu J: Angiosarcoma of the spleen clinically presenting as metastatic ovarian cancer. A case report and review of the literature. Ann Diagn Pathol. 2005, 9: 289-292. 10.1016/j.anndiagpath.2005.03.007.

    Article  PubMed  Google Scholar 

  5. Neuhauser TS, Derringer GA, Thompson LD, Fanburg-Smith JC, Miettinen M, Saaristo A, Abbondanzo SL: Splenic angiosarcoma: a clinicopathologic and immunophenotypic study of 28 cases. Mod Pathol. 2000, 13: 978-987. 10.1038/modpathol.3880178.

    Article  CAS  PubMed  Google Scholar 

  6. Stutz FH, Tormey DC, Blom J: Hemangiosarcoma and pathologic rupture of the spleen. Cancer. 1973, 31: 1213-1215. 10.1002/1097-0142(197305)31:5<1213::AID-CNCR2820310526>3.0.CO;2-H.

    Article  CAS  PubMed  Google Scholar 

  7. Koutelidakis IM, Tsiaousis PZ, Papaziogas BT, Patsas AG, Atmatzidis SK, Atmatzidis KS: Spleen rupture due to primary angiosarcoma: a case report. J Gastrointest Cancer. 2007, 38: 74-77. 10.1007/s12029-008-9034-y.

    Article  PubMed  Google Scholar 

  8. Manouras A, Giannopoulos P, Toufektzian L, Markogiannakis H, Lagoudianakis EE, Papadima A, Papanikolaou D, Filis K, Kekis P: Splenic rupture as the presenting manifestation of primary splenic angiosarcoma in a teenage woman: a case report. J Med Case Reports. 2008, 2: 133-10.1186/1752-1947-2-133.

    Article  PubMed Central  Google Scholar 

  9. Miyata T, Fujimoto Y, Fukushima M, Torisu M, Tanaka M: Spontaneous rupture of splenic angiosarcoma: a case report of chemotherapeutic approach and review of the literature. Surg Today. 1993, 23: 370-374. 10.1007/BF00309058.

    Article  CAS  PubMed  Google Scholar 

  10. Mayir B, Colak T, Dinckan A: [Spontaneous spleen rupture due to primary splenic angiosarcoma: a case report]. Ulus Travma Acil Cerrahi Derg. 2007, 13: 313-315.

    PubMed  Google Scholar 

  11. Raffel S, Hildebrandt B, Grieser C, Pahl S, Sturm I: Thrombocytopenia as first manifestation of splenic angiosarcoma. Ann Hematol. 2010, 89: 109-110. 10.1007/s00277-009-0778-7.

    Article  PubMed  Google Scholar 

  12. Simansky DA, Schiby G, Dreznik Z, Jacob ET: Rapid progressive dissemination of hemangiosarcoma of the spleen following spontaneous rupture. World J Surg. 1986, 10: 142-145. 10.1007/BF01656109.

    Article  CAS  PubMed  Google Scholar 

  13. Maier A, Bataille F, Krenz D, Anthuber M: [Angiosarcoma as a rare differential diagnosis in spontaneous rupture of the spleen]. Chirurg. 2004, 75: 70-74. 10.1007/s00104-003-0733-4.

    Article  CAS  PubMed  Google Scholar 

  14. Villedieu Poignant S, Mermet L, Bousquet A, Dupont P: [A rare cause of spontaneous hemoperitoneum]. Rev Med Interne. 2000, 21: 809-811. 10.1016/S0248-8663(00)00231-9.

    Article  CAS  PubMed  Google Scholar 

  15. Linan-Padilla A, Suarez-Grau JM, Valera Sanchez Z, Vazquez-Medina A, Docobo-Durantez F: [Spontaneous hemoperitoneum due to hemangiosarcoma of the spleen]. Cir Esp. 2008, 84: 171-172. 10.1016/S0009-739X(08)72163-8.

    Article  PubMed  Google Scholar 

  16. Sivelli R, Piccolo D, Soliani P, Franzini C, Ziegler S, Sianesi M: Rupture of the spleen in angiosarcoma: a case report and review of the literature. Chir Ital. 2005, 57: 377-380.

    PubMed  Google Scholar 

  17. Winde G, Sprakel B, Bosse A, Reers B, Wendt M: Rupture of the spleen caused by primary angiosarcoma. Case report. Eur J Surg. 1991, 157: 215-217.

    CAS  PubMed  Google Scholar 

  18. Sordillo EM, Sordillo PP, Hajdu SI: Splenic angiosarcoma. Am J Surg Pathol. 1995, 19: 119-120.

    Article  CAS  PubMed  Google Scholar 

  19. Zwi LJ, Evans DJ, Wechsler AL, Catovsky D: Splenic angiosarcoma following chemotherapy for follicular lymphoma. Hum Pathol. 1986, 17: 528-530. 10.1016/S0046-8177(86)80044-2.

    Article  CAS  PubMed  Google Scholar 

  20. Alt B, Hafez GR, Trigg M, Shahidi NT, Gilbert EF: Angiosarcoma of the liver and spleen in an infant. Pediatr Pathol. 1985, 4: 331-339. 10.3109/15513818509026906.

    Article  CAS  PubMed  Google Scholar 

  21. Autry JR, Weitzner S: Hemangiosarcoma of spleen with spontaneous rupture. Cancer. 1975, 35: 534-539. 10.1002/1097-0142(197502)35:2<534::AID-CNCR2820350236>3.0.CO;2-4.

    Article  CAS  PubMed  Google Scholar 

  22. Nahman B, Cunningham JJ: Sonography of splenic angiosarcoma. J Clin Ultrasound. 1985, 13: 354-356. 10.1002/jcu.1870130513.

    Article  CAS  PubMed  Google Scholar 

  23. Vrachliotis TG, Bennett WF, Vaswani KK, Niemann TH, Bova JG: Primary angiosarcoma of the spleen – CT, MR, and sonographic characteristics: report of two cases. Abdom Imaging. 2000, 25: 283-285. 10.1007/s002610000034.

    Article  CAS  PubMed  Google Scholar 

  24. Reddy SC: Hemangiosarcoma of the spleen: helical computed tomography features. South Med J. 2000, 93: 825-827.

    Article  CAS  PubMed  Google Scholar 

  25. Kinoshita T, Ishii K, Yajima Y, Sakai N, Naganuma H: Splenic hemangiosarcoma with massive calcification. Abdom Imaging. 1999, 24: 185-187. 10.1007/s002619900472.

    Article  CAS  PubMed  Google Scholar 

  26. Ferrozzi F, Bova D, Draghi F, Garlaschi G: CT findings in primary vascular tumors of the spleen. AJR Am J Roentgenol. 1996, 166: 1097-1101. 10.2214/ajr.166.5.8615251.

    Article  CAS  PubMed  Google Scholar 

  27. Rabushka LS, Kawashima A, Fishman EK: Imaging of the spleen: CT with supplemental MR examination. Radiographics. 1994, 14: 307-332.

    Article  CAS  PubMed  Google Scholar 

  28. Thompson WM, Levy AD, Aguilera NS, Gorospe L, Abbott RM: Angiosarcoma of the spleen: imaging characteristics in 12 patients. Radiology. 2005, 235: 106-115. 10.1148/radiol.2351040308.

    Article  PubMed  Google Scholar 

  29. Chen KT, Bolles JC, Gilbert EF: Angiosarcoma of the spleen: a report of two cases and review of the literature. Arch Pathol Lab Med. 1979, 103: 122-124.

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Feng Zhu.

Additional information

Competing interests

The authors declare that they have no competing interests.

Authors’ contributions

Y-FD and FZ participated in the clinical management of the patient and wrote the manuscript. YJ and C-XW carried out the pathological examination. Y-FD and FZ were involved in the final editing. All authors approved the final manuscript.

Authors’ original submitted files for images

Rights and permissions

Open Access This article is published under license to BioMed Central Ltd. This is an Open Access article is distributed under the terms of the Creative Commons Attribution License ( https://creativecommons.org/licenses/by/2.0 ), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Reprints and permissions

About this article

Cite this article

Duan, YF., Jiang, Y., Wu, CX. et al. Spontaneous rupture of primary splenic angiosarcoma: a case report and literature review. World J Surg Onc 11, 53 (2013). https://doi.org/10.1186/1477-7819-11-53

Download citation

  • Received:

  • Accepted:

  • Published:

  • DOI: https://doi.org/10.1186/1477-7819-11-53

Keywords