Spontaneous intraperitoneal hemorrhage as the initial presentation of a gastrointestinal stromal tumor: a case report
© Springer-Verlag London Ltd 2010
Received: 18 July 2009
Accepted: 4 November 2009
Published: 4 February 2010
Spontaneous hemoperitoneum is rare. The most common etiologies are gynecologic, splenic, and hepatic. Gastrointestinal stromal tumors (GISTs) are commonly associated with intraluminal bleeding, but rarely with spontaneous hemoperitoneum. We report a case of spontaneous hemoperitoneum caused by a gastric GIST.
A 54-year-old male presented with the acute onset of abdominal pain and a drop in hemoglobin. Subsequent evaluation, including a CT, MRI, and EUS, revealed a 1.2-cm mass along the greater curvature of the stomach and associated hemoperitoneum. The patient was taken electively to the operating room for laparoscopic removal of the mass. Pathology confirmed that it was a GIST.
GIST is a rare clinical entity that infrequently presents with spontaneous hemoperitoneum. Emergent treatment should be guided towards treating the spontaneous hemoperitoneum.
Differential diagnosis of spontaneous intraperitoneal hemorrhage
Ruptured ovarian cyst
Ovarian granulosa cell tumor
Ruptured corpus leuteum cyst
Spontaneous uterine rupture
Chronic myelomonocytic leukemia
Spontaneous splenic rupture
Spontaneous rupture of splenic vein
Torsion and rupture of wandering spleen
Primary splenic angiosarcoma
Primary hepatic angiosarcoma
Focal nodular hyperplasia
Transhepatic rupture of gallbladder
Ruptured cystic artery pseudoaneurysm
Ruptured splenic artery aneurysm
Segmental mediolytic arteriopathy
Mixed cavernous-capillary hemangioma
Ruptured benign solitary fibrous tumor
Important gynecologic causes of spontaneous hemoperitoneum include ruptured ectopic pregnancy, ruptured ovarian cyst, and endometriosis [1, 2]. Rare gynecologic causes of spontaneous hemoperitoneum include various benign and malignant neoplasms , hemorrhagic corpus luteum cyst torsion , and spontaneous uterine rupture in the first trimester of pregnancy .
Splenic causes of spontaneous hemoperitoneum include oncologic etiologies such as chronic myelomonocytic leukemia , as well as infectious etiologies, such as infectious mononucleosis , cytomegalovirus, Epstein-Barr virus, malaria, and rarely Bartonella henselae [1, 2]. In addition, spontaneous splenic rupture is commonly associated with an underlying mass, most commonly a hamartoma , or with infiltrative diseases such as amyloidosis and Gaucher’s disease .
Rupture of a previously unknown liver lesion, although rare, is a common cause of spontaneous hemoperitoneum [1, 2]. Hepatic adenomas, the most common benign liver lesion associated with spontaneous hemoperitoneum, are most frequently encountered in young females taking oral contraceptives or who are pregnant [1, 2]. Other benign liver lesions known to cause spontaneous hemoperitoneum include giant hemangiomas , focal nodular hyperplasia [1, 2], and hepatic adenomatosis, an uncommon condition resulting in multiple large hepatic adenomas not associated with oral contraceptive use . Hepatic amyloidosis rarely causes spontaneous hemoperitoneum [1, 2]. Hepatocellular carcinoma (HCC) is the most common malignant hepatic mass causing spontaneous hemoperitoneum, and is associated with a high mortality [1, 2]. Primary hepatic angiosarcoma is much less likely to bleed than carcinoma . Occasionally, metastatic cancer to the liver causes spontaneous hemoperitoneum [1, 2].
A 54-year-old male physician in good health developed the acute onset of upper abdominal pain over 15 min after playing basketball. The pain was moderate in intensity and did not radiate to the back. Over the next 12 h, the pain became significantly more intense, but the patient declined to seek medical advice. After the pain persisted at the same intensity for 3 days and became more diffuse, the patient went to an Emergency Department. The patient denied fever, anorexia, nausea, vomiting, diarrhea, constipation, or hemodynamic symptoms. Past medical history was noncontributory. He had no prior abdominal operations.
In the Emergency Department, the patient’s vital signs were normal, and he was in no apparent distress. The physical examination was normal except for moderate diffuse abdominal tenderness with rebound tenderness bilaterally at the upper quadrants. Bowel sounds were normoreactive. The stool was brown and guaiac negative.
At day 25, an MRI showed a resolving hemoperitoneum and a 1.2-cm lesion along the greater curvature of the stomach. At this point, the decision was made to perform a laparoscopic resection approximately 6 weeks later, when the hemoperitoneum would be mostly resolved. One week prior to surgery, gastroscopy with endoscopic ultrasound was performed. The ultrasound showed a blood vessel coursing through a submucosal mass, which was thought most likely to be a GIST.
At operation, the patient was found to have a <2 cm irregularly shaped mass in the mid-portion of the greater curvature of the stomach. There was no other possible etiology for the hemoperitoneum. This mass was resected using standard stapling techniques with clear margins. Pathology later revealed a C-kit-positive tumor consistent with a GIST. The tumor was 1.3 cm and had a mitotic index of up to 10 per high powered field. Because of concern about tumor rupture at presentation, the patient was started on adjuvant imatinib mesylate (Gleevac). He has remained free of macroscopic disease for 18 months.
Causes of spontaneous hemoperitoneum are varied, but initial Emergency Department treatment should be directed at emergent therapies. Beside the common causes discussed earlier, less common causes include ruptured cystic artery pseudoaneurysm , ruptured splenic artery aneurysm , and spontaneous variceal rupture due to portal hypertension [1, 2]. Anticoagulation, either due to medication or as a result of congenital blood dyscrasias, can lead to spontaneous hemoperitoneum [1, 2]. Segmental mediolytic arteriopathy, a rare condition of unknown etiology, can involve the colic, mid-jejunal, common hepatic, intrahepatic, and gastric arteries, causing intraperitoneal bleeding . A benign solitary fibrous tumor, which was once considered to be an exclusively thoracic lesion, can also cause hemoperitoneum . Ruptured cholangiocarcinomas can rarely present with spontaneous hemorrhage . A mixed cavernous-capillary hemangioma with central necrosis can occur on the greater curvature of the stomach, accompanied by bleeding and hemoperitoneum . Other rare conditions associated with hemoperitoneum include transhepatic rupture of the gall bladder, which has been described fewer than 20 times in the literature . Intraperitoneal hemorrhage can also be caused by inflammation of a non-perforated Meckel’s diverticulum . Pancreatic pseudocyst is an unusual entity of acute abdomen, usually occurring among alcoholics .
Gastrointestinal stromal tumors (GISTs) are rare, accounting for only 0.1–3% of all gastrointestinal malignancies [17–19]. The annual incidence of GIST in the US is 4,500 to 6,000 cases per year, or roughly 10 to 20 cases per million people per year [19, 20]. The median age at diagnosis of a GIST is 60 [19, 20]. Primary GISTs arise most commonly in the stomach (50–70%), followed by the small intestine (25–35%), colon and rectum (5–10%) and esophagus (<5%) . Gastric GISTs arise most commonly in the fundus. The clinical presentation of gastric GISTs is varied. Small GISTs <2 cm are generally asymptomatic and are often detected incidentally. Large GISTs can ulcerate and present with gastrointestinal bleeding [22–25]. Other common symptoms of GISTs include anorexia, weight loss, nausea, vomiting, early satiety, bloating, and abdominal pain.
Although it is common for GISTs to present with intraluminal bleeding [22–25], GISTs presenting with spontaneous hemoperitoneum are rare. We could only find one other case report in the literature of a GIST presenting with hemoperitoneum . Hepatic metastases of a GIST can spontaneously rupture, producing hemoperitoneum . Primary GISTs of the transverse mesocolon have been associated with hemorrhagic shock caused by rupture of the tumor .
The differential diagnosis of spontaneous intraperitoneal hemorrhage is broad. GIST is a rare possible etiology. Emergent management of a patient presenting with spontaneous hemoperitoneum involves surgical consultation and resuscitation as necessary. The patient presented in this case report did well with non-operative management followed by surgical resection.
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