A CASE HISTORY OF DEDIFFERENTIATED

LIPOSARCOMA

PRESENTED BY: DR EFFIOM, VICTOR EDEMHOUSE OFFICER, GENERAL SURGERY III UNIT

UCTH, CALABAR.

BIODATAName : B.A.MAge: 24yrsSex: maleOccupation: Undergraduate Student (final Year) Of

Uniuyo, Electrical Engineering)Marital statue: SingleReligion : Redeem Christian church of GodTribe : Ibibio (Ibiono-Ibom LGA)

Admitted Via MOPD with C/o abdominal distension x 4/12Difficulty with breathing x 4/12Weight Loss 2/52

HPC: Patient was in a stable state of health until about 4/12 months prior to presentation in MOPD when he noticed that his abdomen was getting progressively distended. Abdominal swelling was insidious in onset, painless, started centrally and has progressively worsened to cover the whole anterior abdomen. Started as indigestion and he was told to drink plenty of water based on the initial attribution to the fact that he had over fed.

Swelling did not regress at any time, and there was associated progressive body weakness. With the progressive abdominal distension, he developed difficulty with breathing which was made worse on lying supine and on walking for some distance. Sitting upright or inclining to an angle alleviates dyspnea

There was associative loss of appetite and early satiety, but no history of anorexia, nausea/vomiting, or constipation. However, his fecal matter became reduced in Caliber (mostly pellet form), markedly reduced in quantity but no history of melena/haematochezia

Associated swelling of both feet up to mid-leg and reduction in urine output with voiding of dark coloured urine (? Coke colored). No change in stool color. No cough, but Associated low grade fever and night sweats. No history of contact with a patient with chronic cough. No breathlessness, palpitations, orthopnea or PND

No yellowness of eyes, no LUTS, no facial puffiness, no sore throat. No hx of significant alcohol consumption or tobacco use. No history of sharing of sharps, blood transfusion or IV drug use.

No previous history of surgery & no trauma to the abdomen preceding the onset of the above symptoms.

On account of the above, patient presented to UCTH at MOPD, from where he was subsequently admitted into the MMW ward with a working diagnosis of abdominal TB to R/o intra-abdominal malignancy. He was commenced on Anti-Koch trial for 1-2 weeks. (RIPE) despite work Up for TB being Non-conclusive. (AFB-negative)

The unit was invited to review the patient 23 days post admission into the MMW following the abdominal CT scan result, which was suggestive of mesenteric Fibromatosis.

Our initial impression was that of an Intrabdominal tumor ? Cause.

PMHx/SHX : No previous surgery/ hospital admission. No inter-current illnesses. Not a known HEADS.

Family/SHX: Patient comes from a polygamous setting. He is the 6th of 7 children from his mother (4 males, 3 females) All siblings alive and well. Father deceased (unknown cause).No similar illness in any of his relatives. No alcoholic intake history, No tobacco history.

O/E: young man, anxious, chronically ill looking, (markedly wasted, as evidenced by bony protuberances and sagging skin folds) moderately pale, mildly febrile to touch, anicteric, acyanosed, bilateral leg swellings (pitting, up to the knee), no significant peripheral lymphadenopathy.

Vital signs: PR:116bpm, BP:110/80mmHg T:36.7 RR:22Cpm

Abdominal examination: Tensed and grossly distended with visible distended superficial veins over abdomen. Moves with respiration. Sausage shaped mass measuring 22x18cm extending from the right hypochondrium all the way to the RT. iliac fossa and transversely close to the midline with a bosselated edge. Firm, non-tender, smooth and not mobile. Not attached to overlying skin. superior margins enclosed in bony rib cage, but can’t go above it. Inferior and lateral borders ill defined.

Intrabdominal organs are not accessible. Bowel sounds are markedly reduced.

ABDOMINAL GIRTH MEASUREMENT: 22CM FROM XIPHISTERNUM (from MMW)

DATE ABDOMINAL GIRTH WEIGHT

25TH APRIL 2015 106CM 67KG

27TH APRIL 2015 107CM 67KG

28TH APRIL 2015 107CM 66KG

29TH APRIL 2015 107CM 68KG

30TH APRIL 2015 107CM 68KG

DRE: NormalChest: Vesicular breath sounds. Trachea central,

equal chest expansion, decreased air entry bi-basally and in mid lung zone. 

Impression/pre-op diagnosis: Intra-abdominal mass ? cause

Plan: Work-up patient for exploratory laparotomy 

PRE-OP INVESTIGATIONS/ WORK-UP:

FBC +ESR, E/U/Cr, UrinalysisLFT , Serum protein; HBSAgAbdominal X – RAYAbdominal CT- ScanBlood grouping & cross match (2units of whole

blood)Obtain informed consent for exploratory laparotomy.Withhold morning dose of anti-Koch’sNPO from 12 midnight prior to day of surgery

LFT- (Total Bilirubin: 10.2umol/L (2-17umol/L), Conjugated bilirubin 8.9umol/L (0-5umol/L), AST 66iu/L, (up to 40)ALT 20iu/L, (up to 40)ALP 38iu/L (22-140)

Serum Protein: Total 54g/l,(62-83) Albumin 33g/l,(33-52) And globulin 21g/l (18-36)

CXR: blunting of costophrenic and cardiophrenic angles. Elevation of Right Hemi-diaphragm. Imp: Basal Pneumonitis.

E/U/Cr result showed acidosis HCO3: 18mmol/LOthers: urea3.9mmol/l (2.5-6.7)Sodium 138mmol/l (132-145)Potassium 3.8mmo;/l (3.2-5.0)Cl- 107mmol/l (96-108)Creatinine 113.1mmol/l (88.6-177)

FBC: PCV 23.2% (41-53)RBC:2.84 X 109/L Hg:7.5g/dl (13.5-17.5)WBC: 4 X 109/L (4.3-5.9 million/mm3)ESR: 90mm/hr. (Westergren Method)

HBsAg, HIV serology: Negative Urinalysis: results reveal yellow and cloudy urine

with acidic PH of 6. All other findings are Norm

Result of Abdominal CT Scan: suggestive of a giant Mesenteric Fibromatosis.

PRE-OP DIAGNOSIS: - Intra-abdominal Tumor ?cause

 

Operation: Exploratory Laparotomy + resection of intra-abdominal tumor +

resection of greater curvature of stomachIntra-Op Management.Patient supine under GA + ETT.Skin prep. With spirit and Providone iodine and draping.Prophylactic antibiotics (Ceftriaxone) administered.NG-tubeUrethral Catheterization.Via a midline supra and Infra umbilical incision, tissue

planes dissected into abdominal cavity.Mass dissected via blunt and sharp dissections following

a plane.

Part of greater curvature infiltrated by mass, resected, alongside with the mass.

Mass completely resected; weighing ~ 20kgSome of the locules spontaneously ruptured

discharging hemorrhagic fluid, which was suctioned.Minimal oozing noted around splenic region; drain

insertedMass closure of wound one with nylon 2. Skin with

nylon 2/0.Patient received about 3L (4 units of blood)of whole

blood intra-Op. Also had 2 liters of Isoplasma and 4 liters of crystalloid.

EBL >4L.Mass sent for Histology

Intra-Op findings:Huge intrabdominal mass occupying whole

abdomen enclosed by greater OmentumPart of mass attached to greater curvature of the

stomach.Variable consistency - Mass cystic in parts

(containing hemorrhagic fluid), firm in parts and lobulated and multiloculated.

Hemorrhagic areas and grossly dilated vessels over the mass

No ascites, normal bowel loops, liver.Weight of mass tissue: 20kg

POST OP DIAGNOSIS: Omental Tumor ? cause

 Immediate Post Op Management.Admission into ICU x 2 days (could not take over

respiration spontaneously as mass splinted the diaphragm)

NPO till reviewed.Monitor v/s hourly till stable.5th unit of blood given in ICU (Anesthetist

Assessment)

(Post-Op Day 1)

Post-Op PCV 19%E/U/Cr: Urea 6.3mmol/l (2.5-6.7)Sodium 149 mmol/l (132-145)Potassium 4.3mmol/l (3.2-5.0)Chloride 116mmol/l (96-108)Bicarbonate 21 mmol/l (22-28)Creatinine 113.5umol/L (88-177).

(Day 2 Post Op)

Opened bowel to flatusBowel sounds normoactive (3 in 1 minute)NG Tube removedIV Astymin 200ml daily + IV B complex 10mls to

each fluid.Discharged to ward from ICU after establishing

spontaneous respiration and achieving satisfactory SPO2 at room air. (>98%)

WARD POST-OP MANAGEMENT(D3PO)

Post Op day 3 PCV 15%NG-Tube & Urethral catheter removed on D3POFirst dose of SC Erythropoietin given (4000iunits

alternate days x 3 doses) Plan: transfuse 1 unit of blood

(D4PO)Surgical drain removed. Graded oral intake commenced (D10PO)complaints of dizziness even while lying down + poor

appetite.Open bowel to dark tarry stool. Dyspnea on tachypnea.On High protein diet. Sutures removed.Plan: Transfuse 2 more units of blood (Making a total of 8);

last unit on morning of D11PO.Continue SC erythropoietin and other medications. (Had 4

out of 6 recommended doses of SC erythropoietin owing to financial constraints)

(D15PO)

Consult sent to hematology team to review in view of the persistent anemia post transfusion.

Hematology Review: Anemia ? cause r/o MyeloinfiltrationPlan: do FBC + Platelet, peripheral blood film,

reticulocyte Count, BMA. Peripheral Blood film: Aniscocytosis ++, Macrocytosis

and Microcytosis ++, Poikilocytosis ++, Polychromasia +, hypochromasia ++,

PCV now 23% after 7th and 8 unit of blood was transfused.

WBC 3.1 X 109/LNeutrophil 72%Lymphocyte 28%

Final Diagnosis: Histopathological report made a diagnosis of Intra-abdominal Mass-Dedifferentiated Liposarcoma.

Discharged home on D17PO for follow up in SOPD in 1/52

First appointment=19/06/15 (SOPD). Repeat FBC + ESR (22/06/15): Hb 11g/dl,PCV 33%, ESR 34 mm/hr. (Westergren Method), WBC 7.8 X 109/L, Neutrophil 62%, Eosinophil 2%,

Lymph 36%, Aniscocytosis +, hypochromasia +, microcytosis +.Urinalysis: NAD

Patient is doing well!