An unusual case of desmoid tumour presenting as haemorrhagic shock

Abstract

We report the case of a 22-year-old patient who presented to the emergency department with epigastric pain and vomiting. Haematological studies showed a rapid decrease in haemoglobin levels from 13.6g/dl to 4.9g/dl. Abdominal ultrasonography revealed the presence of fluid around the spleen and the patient was immediately referred for surgery. An intra-abdominal desmoid tumour presenting as a hemorrhagic shock has not previously been described. Given the relatively benign course of the disease and the young age at presentation, this clinical entity should not be overlooked as it has the potential to invade vessels and therefore be fatal. KEywORdSDesmoid -Aggressive fibromatosis -Hemorrhagic shock Case historyA 22-year-old woman presented to the emergency department with epigastric pain of 12 hours' duration and vomiting. On examination, the patient was afebrile with mild epigastric tenderness. Upper and lower abdominal ultrasounography was unremarkable. Initial haematological and biochemical parameters were normal except for leucocytosis (white blood count: 14,000 cells/mm 3 ). At the time of admission, the patient looked acutely ill, was pale and pulseless with severe epigastric and left upper abdominal quadrant tenderness. Serial haematological studies showed a rapid decrease in haemoglobin level from 13.6g/dl at presentation to 4.9g/dl one hour later. New abdominal ultrasonography was performed, which revealed the presence of fluid around the spleen. The patient was referred for surgery immediately. An area of bleeding behind the body of the pancreas was located. A splenectomy was performed as a lifesaving manoeuvre to maximise access to the bleeding area. Palpation revealed the presence of a 1.5cm x 0.5cm tumour strangulating the splenic artery and branches of the artery to the pancreas. Resection of the mass en bloc with a portion of the splenic artery was undertaken. Nine units of packed red blood cells and seven units of fresh frozen plasma were administered.The patient was discharged a few days later completely well. Follow-up magnetic resonance imaging at three months did not show any recurrence. It is noteworthy that the patient had no history of familial adenomatous polyposis syndrome.Histological examination revealed proliferation of myofibroblasts with a fasciculate and storiform growth pattern in a background of a mainly collagenous and focally myxoid intercellular matrix. The benign tumour entrapped part of the pancreas and infiltrated medium-sized arteries, replacing their smooth muscle cells (Fig 1). Recent haemorrhage between myofibroblasts was found with absence of haemosiderin laden macrophages, suggestive of a chronic haemorrhage. No pleomorphic, atypical or hyperchromatic nuclei were seen. Immunohistochemical staining showed granular cytoplasmic positivity and focal nuclear positivity for β-catenin. Immunohistochemistry for desmin, smooth muscle actin, CD117 and CD34 was negative. The proliferation marker Ki-67 stained approximately 2% of cells. These features were suggestive of dee...

Ruptured desmoid tumor imitating acute appendicitis – a rare reason for an emergency surgery

“…Usually, this complication results from direct invasion of major vessels and subsequent rupture. We found only one such case reported by C. Georgiades et al (2012), demonstrating a 1.5 cm × 0.5 cm retroperitoneal tumor strangulating the splenic artery and branches of the artery to the pancreas [13]. We could not find any case presenting with intraabdominal hemorrhage due to mesenteric or small bowel fibromatosis, as in our case.…”

“…Again, our case was different because there was no perforation of a hollow organ but there was a rupture of the tumor pseudo-capsule with subsequent hemoperitoneum. Clinical signs such as aortic rupture, intra-abdominal abscess, or hepatic pneumatosis have also been reported [11][12][13][14].…”

Not all abdominal masses after colorectal cancer surgery are malignant: intra‐abdominal fibromatosis masquerading as recurrence

“…Several case reports have been published whereby fibromatosis presents as an incidental or symptomatic abdominal mass [12,[16][17][18][19][20][21][22][23]. Some present as surgical emergencies with bleeding requiring emergency laparotomy [24,25]. Fibromatosis has also been reported after abdominal surgery for benign tumours, such as uterine myoma [26], or mimicking recurrent GIST either in the mesentery or the retroperitoneum [27,28].…”

Desmoid-Type Fibromatosis of the Mesentery: Report of a Sporadic Case with Emphasis on Differential Diagnostic Problems

“…Its pathogenesis is not completely understood, even if some cases have been associated with antecedent abdominal trauma, including previous surgery (25%) [ 2 , 6 , 9 , 11 ], or hyperestrogenic states. Hormonal estrogenic disorders may be involved in the pathogenesis, and this could explain the high incidence of this disease in females (female-male ratio 3 : 1), frequently in young pregnant or postpartum women with an age ranging from 25 to 35 years [ 2 , 9 , 13 ].…”