Gastric epithelioid hemangioendothelioma: A rare cause of spontaneous hemoperitoneum

Dhanabir Thangjam; Rosemary Vumkhoching; Ng Javan; Ningombam Jitendra

doi:10.4103/0972-4958.135238

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CASE REPORT

Year : 2014 | Volume : 28 | Issue : 1 | Page : 54-56

Gastric epithelioid hemangioendothelioma: A rare cause of spontaneous hemoperitoneum

Dhanabir Thangjam, Rosemary Vumkhoching, Ng Javan, Ningombam Jitendra
Department of Surgery, Jawaharlal Nehru Institute of Medical Sciences (JNIMS), Imphal, Manipur, India

Date of Web Publication

24-Jun-2014

Correspondence Address:
Dr. Dhanabir Thangjam
Department of Surgery, Jawaharlal Nehru Institute of Medical Sciences,(JNIMS) Porompat, Imphal - 795 005, Manipur
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/0972-4958.135238

Abstract

Spontaneous hemoperitoneum (SHP) also known as abdominal apoplexy is a rare medical condition but often life threatening. The most important treatment strategy for abdominal apoplexy is surgery where identification and ligation of the bleeding points is the main goal. Gastric hemangioendothelioma is an intermediate-grade vascular neoplasm. Vascular tumors of the stomach are rare, 0.9-3.3% of all gastric neoplasms hemangioendothelioma. It may develop in any organ, but more common in lungs and liver. We present an unusual case report of a 42 year-old gentleman admitted as a case of acute cholecystitis, who was intra operatively found to have spontaneous hemoperitoneum due to a ruptured serosal gastric tumor. Histopathological examination of the tumor reported as gastric hemangioendothelioma-epithelioid type. Although the treatment of choice is surgery, radical surgery can be avoided if there is no evidence of any malignant potential.

Keywords: Abdominal apoplexy, Gastric epithelioid hemangioendothelioma, Spontaneous hemoperitoneum(SPH)

How to cite this article:
Thangjam D, Vumkhoching R, Javan N, Jitendra N. Gastric epithelioid hemangioendothelioma: A rare cause of spontaneous hemoperitoneum. J Med Soc 2014;28:54-6

How to cite this URL:
Thangjam D, Vumkhoching R, Javan N, Jitendra N. Gastric epithelioid hemangioendothelioma: A rare cause of spontaneous hemoperitoneum. J Med Soc [serial online] 2014 [cited 2023 Apr 1];28:54-6. Available from: https://www.jmedsoc.org/text.asp?2014/28/1/54/135238

Case Report

A 42-years-old gentleman was admitted in male surgical ward unit I, JNIMS, with complaints of pain in upper abdomen and burning epigastrium of 1-day duration. History of past illness was not contributory. On general physical examination, his vitals were stable. There was no pallor and icterus. Per abdominal examination revealed tenderness over the epigastric and right hypochondrium regions. Blood investigations showed normal hemogram and coagulation profile. The total bilirubin level was 2.6 gm./dl with indirect 2.30 gm./dl and the liver enzymes were within normal limit. USG whole abdomen report showed cholelithiasis with cholecystitis. Repeat LFT after 1 week became normal. Patient underwent open cholecystectomy. On entering the abdominal cavity there was repeated collection of hemorrhagic fluid at the operative field along with thickened gall bladder wall. Gallbladder contained multiple calculi. After completing cholecystectomy, and excluding bleeding from the operative site, a midline exploratory laparotomy was done to locate the source of bleeding. Dark hemorrhagic fluid with clots around 500 ml in the perihepatic, perisplenic, perigastric regions and the pelvis was found. On further exploration, there was a ruptured serosal tumor measuring 5 × 3 cm arising from the anterior surface of the body of the stomach [Figure 1]. Wide excision of the tumor and repair of the serosal wall was done in two layers. Other visceras, omentum, and mesentery were normal.

Figure 1: Intraoperative photograph showing the ruptured gastric serosal tumor (arrow)

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After a thorough peritoneal lavage, abdominal closure was done with an abdominal drain in the pelvis. Postoperatively repeat blood investigations showed hemoglobin level of 6.9 gm%, PCV 22.1 gm%. The other blood parameters were normal. Patient received two units of compatible blood. His recovery was uneventful and discharged on the eighth postoperative day. Histopathological examination reported the gall bladder specimen as chronic cholecystitis. Microscopic examination [Figure 2]. of the gastric serosal tumor specimen showed the presence of numerous proliferating blood vessels lined by plump endothelium with foci of epithelioid cells and numerous dilated congested blood-filled cavernous spaces. However, there was no evidence of abnormal mitoses or areas of necrosis. So features were suggestive of hemangioendothelioma ( benign epithelioid type) He is doing well in the first month of follow up. He has been recommended for upper GI endoscopy in the next month follow up and subsequent regular follow up.

Figure 2: Photomicrograph showing the dilated blood-filled cavernous spaces lined by plump endothelium (white arrow) with background of epithelioid cells (black arrow). (H and E stain, 40×)

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Discussion

Spontaneous hemoperitoneum (SPH) was first reported by Barber in 1909 and was later termed 'abdominal apoplexy' by Green and Powers in 1931. It is a rare medical condition associated with high mortality; however, its true incidence is unknown. ^[1] It is an uncommon cause of acute abdominal pain and is defined as the presence of blood within the peritoneal cavity which is unrelated to trauma. ^[2] It can also be idiopathic or related to spontaneous rupture of either known or unknown pathology. ^[1] It may be intraperitoneal or retroperitoneal and is frequently found in conjunction with hypertension (33-50%) and atherosclerosis (80-87%). ^[1] There is a male predominance within the affected people and the majority of cases present in the fifth or sixth decade of life. ^[3] The presentation and clinical progression of abdominal apoplexy frequently follows a rather predictable course. Before rupture, there may be a history of vague abdominal pain. The symptoms are usually nonspecific.

Physical examination before or soon after rupture is likely to be relatively normal although no finding is pathognomonic. Symptoms of acute hemoperitoneum can probably be divided into three phases: an initial phase of mild to severe abdominal pain, a latent phase lacking any symptoms, which can last for hours to days, and a final phase of rapid progression to severe acute hemoperitoneum. ^[2]

We did not appreciate the presence of these three phases in our patient as his acute abdominal signs were considered to be due to acute calculus cholecystitis which was reported on USG abdomen and vitals were stable with normal hemoglobin levels and mildly raised total bilirubin on the day of his admission.

The diagnosis of spontaneous hemoperitoneum is generally made on exploratory laparotomy for hemodynamically unstable patient. In less urgent cases or stable, FAST (focused assessment by sonography in trauma) may be useful to detect intra-abdominal hemorrhage. However, CT scan represents the most important imaging technique in most patients for preoperative diagnosis. Abdominal CT angiography is also a useful technique to elucidate sites of active intra-abdominal bleeding. ^[1] The most important step of diagnosis of this rare condition is clinical suspicion of hemoperitoneum. Treatment of spontaneous hemoperitoneum is resuscitation and restoration of circulating volume traditionally followed by surgical correction which consists of resection of the tumor/aneurysm, ligation of the feeding vessels or some forms of arterial reconstruction. Radiological intervention with embolization of the feeding vessel in splanchnic aneurysmal bleed is also an option. Nonoperative treatment for spontaneous hemoperitoneum has almost 100% mortality and nontherapeutic exploration results in about 42% postoperative mortality rate. ^[4]

Hemangoiendothelioma is a vascular tumor of intermediate malignancy that may occur in a variety of sites, including soft tissues, bone, lung, liver, pleura and peritoneum, skin, lymph nodes, stomach and brain. ^[5],[6] It is broadly described in four types they are:

Epithelioid hemangioendothelioma,
Spindle cell type,
Kaposi form type, and
Endovascular papillary type (Dabska). ^[7]

The etiology and pathogenic factors remains unknown. ^[7] Vascular tumors of the stomach including gastric hemangioendothelioma are rare 0.9-3.3% of all gastric neoplasms. ^[8] Due to its nonspecific symptoms early diagnosis is difficult as in this present case. It can present as an abdominal mass or GOO or upper GI bleeding. ^[6] Its preoperative diagnosis is difficult as biopsies are inadequate because of its submucosal or serosal location. These lesions may even go undetected in the imaging studies. ^[7] Histopathology and immunohistochemistry (IHC) are crucial for the proper diagnosis. Histologically this neoplasia is characterized by the presence of endothelial cells with a large amount of eosinophilic cytoplasm similar to histiocytes, contains cords and blocks of epithelioid endothelial cells, sometimes intumescent and located inside a fibromyxoid stroma. ^[9] IHC of hemangioendothelioma shows positive for CD34, CD 31, and factor VIII and negative for markers of neuronal or muscular differentiation. ^[10] Surgery represents the treatment of choice of which wide excision seems to be adequate and radical gastric surgery can be avoided if there is no histological evidence of any malignant potential. ^[7],[11] The neoplasm also carries good prognosis in the absence of histological markers of aggressiveness. But careful regular follow up is recommended due to the borderline biological behavior of these tumors. ^[11]

In this case, the diagnoses of spontaneous hemoperitoneum and gastric hemangioendothelioma were made intraoperatively and postoperatively, respectively, as the case was operated for acute calculus cholecystitis as there was no clinical clues for the above two rare conditions preoperatively. This case report aims to highlight the rarity of this disease and the difficulty of preoperative diagnosis, which is not possible in many cases. After a review of the literature in PubMed relative to period from 1980 to 2013, we found that there are only four cases reported in English publication for gastric epithelioid hemangioendothelioma and one case of malignant hemangioendothelioma of stomach.

References

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