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.