Saudi Journal of Gastroenterology
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CASE REPORT Table of Contents   
Year : 2007  |  Volume : 13  |  Issue : 3  |  Page : 136-137
Spontaneous rupture of the spleen: A case report and review of the literature

Department of Surgery, Guilan University of Medical Sciences, Razi Hospital, Rasht, Iran

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Date of Submission19-Feb-2007
Date of Acceptance16-Mar-2007


Atraumatic rupture of the spleen is an uncommon but important clinical entity. The diagnosis is often missed due to the absence of any history of trauma. Various aspects of atraumatic rupture of the spleen including those of pathologic and spontaneous rupture have been reviewed. Primary angiosarcoma of the spleen is a very rare neoplasm with disastrous prognosis. Increased awareness of this condition would enhance early diagnosis and effective treatment.

Keywords: Angiosarcoma, splenic rupture

How to cite this article:
Safapor F, Aghajanzade M, Kohsari MR, Hoda S, Safarpor D. Spontaneous rupture of the spleen: A case report and review of the literature. Saudi J Gastroenterol 2007;13:136-7

How to cite this URL:
Safapor F, Aghajanzade M, Kohsari MR, Hoda S, Safarpor D. Spontaneous rupture of the spleen: A case report and review of the literature. Saudi J Gastroenterol [serial online] 2007 [cited 2021 Dec 8];13:136-7. Available from:

Atraumatic rupture of the spleen has been described in the medical literature as a clinical oddity with grave consequences if unrecognized and untreated. Primary angiosarcoma of the spleen is very rare and only 143 cases have been reported so far. [1],[2] Clinical symptoms usually are diffuse abdominal pain, left upper quadrant mass and mild form of hypovolemic shock. [3] Associated clinical findings are anemia, thrombocytopenia and coagulopathy. [2] In the absence of trauma, a clinical diagnosis of perforated peptic ulcer is usually made at presentation. [1]

The purpose of this review is to identify the important features of atraumatic rupture of the spleen in adults and to enhance our awareness of this interesting and unusual condition.

   Case Report Top

A 75 year old woman known to have chronic obstructive pulmonary disease (COPD) was admitted to our emergency department. She complained of left upper abdominal pain and left shoulder pain, nausea and vomiting. The pain was constant and dull. The pain started two days prior to admission and was not related to meals. She had no change in her bowel habits. There was no history of recent trauma. On examination, the fever was present (37.5șC) with a blood pressure of 80/40 mm/Hg and a pulse rate of 110 / minute. She had tenderness in the left upper quadrant of her abdomen. Bowel sounds were absent. Rectal examination was normal.

Laboratory evaluation showed leukocytosis of 19 (10 9 /L), haematocrit 31%, haemoglobin 10 mg/dL and the platelet count was 87 (10 9 /L). Abdominal plain film demonstrated no significant bowel gas pattern but abdominal sonography showed a hyperechogenic mass (140 x 79 mm) and perisplenic fluid. Another mass (40 x 60 x 45 mm) containing internal echogenicities was seen in the perisplenic area and a moderate amount of free fluid was present in the pelvic area. Abdominal paracentesis was done and frank blood was aspirated from the peritoneal cavity. Laparotomy revealed a huge hematoma containing splenic tissue. Subsequent histological findings were consistent with angiosarcoma of the spleen [Figure - 1],[Figure - 2].

   Discussion Top

Spleen rupture has been reported to be due to trauma or to the spontaneous rupture of a diseased or a normal spleen. [4] Primary angiosarcoma of the spleen is a very rare neoplasm with disastrous prognosis and is associated with widespread metastasis or splenic rupture. Some authors have suggested that the atraumatic or so-called spontaneous rupture of a diseased spleen be termed 'pathologic rupture' which would be analogous to the accepted condition of the pathologic fracture of a diseased bone. Spontaneous rupture of the spleen has also been described as occult rupture, therefore "spontaneous / atraumatic rupture of the spleen" should be reserved for the healthy spleen that has ruptured without overt trauma and is also known as idiopathic rupture. [4]

Although angiosarcoma is rare, it is now regarded as the most frequent malignant tumor of the spleen. The average weight of the spleen in one series of studies was found to be 1700 grams. Most patients are middle-aged and other symptoms are related to mass effect. In addition, patients may present with weight loss, cachexia, ascites and plural effusion. The lesions grow rapidly and metastasize to regional lymph nodes, marrow, liver and lung. Splenectomy in emergent and elective cases is rarely curative. Angiosarcoma tends to recur locally, spreads widely and has a high rate of lymph node and systemic metastasis. Primary non-hematopoietic malignant tumors of the spleen are rare and depending on cell origin. They are classified into endotheliomas and angiosarcomas arising from the sinus epithelium, and fibrosarcomas which arise from connective tissue elements. Among these rare malignant tumors, angiosarcomas are more common and are of greater clinical significance.

Immunohistochemical and ultrastructural studies are necessary to verify the diagnosis as the histopathological picture of the tumor is variable due to the small number of reported cases. There are no guidelines for adjuvant or palliative therapy, and chemotherapy has not improved the prognosis. However, several investigators have reported a better prognosis in cases in which splenectomy was done. Awareness of this rare splenic malignancy and use of imaging especially spiral CT may help in making a diagnosis and improve survival. [5]

Spontaneous splenic rupture, also a rare entity, is usually associated with infectious, neoplastic or hematologic diseases. Unlike traumatic splenic rupture, spontaneous rupture of the spleen is not often considered in the differential diagnosis of abdominal pain and can be easily confused with other abdominal pathology. [6] Failure to consider splenic rupture can be catastrophic. In conclusion, we treated a case of atraumatic splenic rupture in a patient with no underlying disease pathology. The case illustrates the importance of maintaining a broad differential diagnosis in patients presenting with acute abdominal pain where the diagnosis of spontaneous non-traumatic splenic rupture should be entertained.

   References Top

1.Debmath D, Valerio D. Atraumatic rupture of the spleen in adults. J R Coll Surg Edinb 2002;47:437-45.  Back to cited text no. 1    
2.Reddy SC, Reddy SC. Hemangiosarcoma of the spleen: helical computed tomography features. South Med J 2000;93:825-7.  Back to cited text no. 2  [PUBMED]  
3.Falk S, Krishnan J, Meis JM. Primary angiosarcoma of the spleen. A clinicopathologic study of 40 cases. Am J Surg Pathol 1993;17:959-70.  Back to cited text no. 3    
4.Giagounidis AA, Burk M, Meckenstock G, Koch AJ, Schneider W. Pathologic rupture of the spleen in hematologic malignancies: Two additional cases. Ann Hematol 1996;73:297-302.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.Urban BA, Fishman EK. Helical CT of the spleen. AJR Am J Roentgenol 1998;170:997-1003.  Back to cited text no. 5  [PUBMED]  
6.Skadberg JE, Sondenaa K, Soreide JA, Andersen E. Non traumatic (spontaneous) splenic rupture. Tidsskr Nor Laegeforen 1993;113:1354-5.  Back to cited text no. 6    

Correspondence Address:
Faizollah Safapor
Guilan University of Medical Sciences, Razi Hospital, Rasht - 41448
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1319-3767.33466

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  [Figure - 1], [Figure - 2]

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