S Aslam Shah, M A Zahid, Shahid Ahmad Khan, Syed Liaqat Shah, Ghulam Murtaza Dar, Syed Shabbir Akhtar.
Primary Retroperitoneal Liposarcoma.
J Coll Physicians Surg Pak Jan ;11(11):719-20.

A case of huge primary retroperitoneal liposarcoma, weighing 16.5 kg, is being reported. This is the second largest tumor reported in literature so far. A forty-eight years old male initially being treated for tropical splenomegaly for several months was admitted to the medical unit of Pakistan Institute of Medical Sciences, Islamabad for PUO and mass abdomen. A tumor was suspected on ultrasound and CT scan. Complete excision was successfully performed and histopathology confirmed the diagnosis of well-differentiated liposarcoma.

Case Report : A 48-year-old male was admitted in Medical Unit of Pakistan Institute of Medical Sciences, Islamabad with the complaint of high grade fever and rigors for the last one and half-year. Four to five weeks after the initial attack of fever and rigors, he noticed progressive abdominal distension associated with epigastric discomfort and occasional pain in abdomen, vomiting and constipation off and on. He also experienced weight loss and weakness. There was no history of melena, hematemesis or urinary symptoms. He consulted many physicians and the diagnosis of tropical splenomegaly was finally made for which he had received many courses of different antimalarial drugs. On physical examination, his vital signs were normal and he was extremely pale. His abdominal examination revealed distension with a huge firm mass with irregular surface occupying almost whole of the abdomen. There was no free fluid in the abdomen. Rectal examination was insignificant. The patient was provisionally suspected as a case of splenomegaly, visceral lieshmaniasis and hairy cell leukemia at the medical unit. On investigation his Hb was 6.4 gm%, renal function tests, LFTs and bleeding profile were normal. Ultrasound abdomen revealed multiple, large rounded, echomixed masses, seen in whole of the abdomen and an impression of retroperitoneal lymphadenopathy was arrived at. The surgical unit was called and on their advice ultrasound-guided FNAC of the mass and CT scan of the abdomen were carried out. The FNAC report was inconclusive. C.T scan abdomen reported a large soft tissue loculated mass lesion in the mid abdomen, extending upward to the head of the pancreas and downwards to the pelvis and an impression of retroperitoneal mass with a possibility of sarcomatous change was made.

After pre-operative preparation of the patient, an exploratory laparotomy was done through a midline abdominal incision. The operative finding was a huge retroperitoneal mass occupying whole of the abdomen, pushing the small gut to the right. Transverse mesocolon was adherent to the mass. The tumor was resected enbloc without any damage to the adjacent structures. The postoperative hospital stay was uneventful. On gross examination the tumor weighed 16.5 kilogram, measuring 40 cm x 40 cm x 30 cm. On cut section, there were necrotic and hemorrhagic areas at places. The microscopic picture was consistent with liposarcoma (welldifferentiated - grade-I). Patient was discharged from the hospital on the 10th postoperative day. On follow-up till date for two years, with regular ultrasound abdomen there is no evidence of recurrence of the disease and he has gained more than 20 kg of weight till the last checkup.

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