Case presentation
A 42-year-old African origin male patient who had no comorbidities or previous surgeries or trauma history and no family history of cancer, Familial adenomatous polyposis syndrome, Gardner syndrome or cancer. His childhood was unremarkable, fully vaccinated, and never been hospitalized. Presented with complaints of recurrent left upper abdominal pain for more than 10 years and transferred to our hospital for suspicious large splenic mass for investigation. The pain was disturbing his daily activities as well as sleeping for associated with nausea and vomiting and constipation as well as loss of Appetit. However, there was no history of fever and other systemic review are unremarkable.
By physical examination, the patient looks underweights but conscious, oriented with stable vital signs, not pale or jaundiced and his cardio-respiratory system was unremarkable. There was no lymphadenopathy or scares. There was large abdomen mass mildly-tender at the left hypochondrial area extend to left hypochondrium the umbilicus with rigid and smooth surface approximately 25 X18 cm in size extending from left upper quadrant of the abdomen till the hypochondrial region. By palpation, the mass was not mobile, hard consistency, round borders, and smooth surface. No lower limbs varicose vein, edam, or ischemic changes. All laboratory blood investigations revealed within normal range values for the blood count, liver and renal function tests, furthermore, all tumour markers were within normal ranges e.g. CA19-9 equal to 7.10 U/ml. Contrast CT (figure 1) scan showed normal liver and spleen size and density and revealed large heterogenous mass of pancreatic origin, from the body and tail measuring about 23 cm by 15 cm by11 cm. Displacing and compressing the stomach medially and spleen posteriorly and left kidney as well. Small bowel loops were also displaced to the Rt side. No lymph nodes were seen. Biopsy taken trans gastric under Endoscopic ultrasound guidance showed epithelial cells clusters a long with sheets of cells characterize by oval to round to spindly shaped nuclei in Hg background, however, no malignant cells demonstrated.
The had radical resection of the tumour thorough midline laparotomy. Extensive adhesion and infiltration to adjacent organ noticed encountered. the pancreatic mass was invading nearby organ but assessed to be resectable safely, therefore, radical en mass resection with extended left pancreatectomy, transverse colectomy. Splenectomy, adrenalectomy Patient had smooth recovery and no blood transfusion needed. Blood loss was estimated to be 450 ml; therefore, no blood transfused intraoperatively. Postoperatively, our patient progressed smoothly. The accurate gross size was 26 x 17 x 9 cm and the weight found to be 3.6 Kg (figure 2).
The Histopathology demonstrated Intera-abdominal Fibromatosis by immunohistochemistry Positivity for B-catenin and C-Kit (CD117) and as important, negativity for S-100, Desmin, CD34, Bc12, CD99, ALK-1, Pan-Ck. All surgical resection margins were free of tumour no metastasis to adjacent organs and eleven lymph nodes examined from the specimen showed no malignant cell (figure 2).
Because the resection was curative and based on the histopathological nature of the tumour and the negative lymph node and no distant metastasis no chemo- or radiotherapy prescribed for the patient in tumour-board decision. The three-month follow-up CT scan and annual ultrasound for five years did not demonstrate any recurrence; therefore, curative management is achieved. Furthermore, the patient had excellent satisfaction and improvement of quality of life as he stated and confirmed by the oncologist too.