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7. Varying presentation
• Presentation
1) most common presentation is huge
abdominal lump with compressive
symptoms.
2) Asymptomatic: diagnosis is accidental
3) presentation is usually late: because
i) tumors are slow growing & painless:
pain occurs in benign pathologies like
hemangioma, schwannoma, fibroma,
hematoma, abscess etc.
ii)tumors displaces the adjacent
structures. Infitration ocurrs in late
stages. T2-weighted MRI
9. Signs of Retroperitoneal Mass
1) Due to retroperitoneal mass:
i)No clinical findings unless the swelling is very large
on examination:
• Consistency:Firm to hard mass
• surface :Smooth , but in lymphoma it
is nodular
• Margins: can not be traced properly because of deep
position of the swellings
• Not moving with respiration.
• Non mobile.
• Non tender
• Does not fall forward
(confirmed by knee-elbow position).
10. ii) Deep seated dull ache abdominal pain.
iii) Flank pain : most commonly in carcinoma of kidney
2) Due to compression on adjacent organs:
i) Back Pain- Severe back pain often following pressure by
tumor mass, hematoma, and abscess over muscles, facet joint and
vertebral column.
Radicular Pain- Radiating type of pain along the nerve root due to
its compression.
ii) Obstruction of Viscera and Tubular Organs – usually
of duodenum , colon , ureter , pancreas, kidney
resulting in
• Nausea and Vomiting-
• Colicky Pain-
• Constipation/ obstipation
• Urinary Retention-
11. iii) Compression of Aorta
• Hypertension-
• Renal Insufficiency-
• Mesenteric Ischemia-
• Intermittent Claudication-
iv) Compression of Vena Cava
– Edema of Feet
– Low Blood Pressure
v)Nerve Lesions
– Tingling and Numbness in Lower limbs
– Weakness of the Lower limbs
13. Investigation
1) Routine blood investigations: to know about
i) hemoglobin: anemia
ii) blood and serum creatinine- raised on compression of kidney and
ureter
iii) liver function test
iv) Effect of paraneoplastic syndrome
Hypoglycemia:- due to increased insulin like hormone
Hypercalcemia:- due to increased ectopic PTH hormone
Catecholamines:- paraganglioma
v) Tumor markers:-AFP(alfa feto protein), beta-HCG- Will be raised in
primary germ cell tumor
2) Chest X ray PA view:- lung metastasis
3) Plane X ray abdomen:- signs of intestinal obstruction, obliterated psoas
shadow, calcification of tumor mass.
14. 5)Usg abdomen : nature of mass(solid/cystic)
and relation to the adjacent structures.
6)CT abdomen and pelvis
7) CT chest
8) CT/usg guided/laproscopic core biopsy
9)PET-CT
10)FNAC has got limited role as the representative
tissue may not be obtained .
11) IVU ;- Can show obstruction and displacement of
kidney and ureter, distortion of renal pelvis and
bladder compression.
12) Confirmation of diagnosis is only by tissue biopsy.
16. Indications of preoperative biopsy
• An unusual appearing mass
• Unresectable tumor
• Distant mets
• Patient being considered for
neoadjuvant chemotherapy
• An unusual appearing mass
• Unresectable tumor
• Distant mets
• Patient being considered for
neoadjuvant chemotherapy
18. Medical management :
Retroperitoneal Lymphoma : Chemotherapy is principal treatment
Non Hodgkins’s Lymphoma treatment regime – R-CHOP
( Rituximab, Cyclophosphamide, Oncovin, Predinisolone)
Hodgkin’s lymphoma treatment regime – ABVD
( Adriyamycin, Bleomycin, Vinblastin,Dacarbazin )
Retroperitoneal fibrosis :
In early cases : Empirical therapy includes
corticosteroids : predinisolone for one year
tamoxifen : 10 mg day per for 6 month to 3 year
azathioprine
indicated only when associated with raised ESR , Leucocytosis , Anti-Nuclear
Antibody Positive cases
Patients with hydronephrosis and uremia- Emergency decompression
(by Per-cutaneous Nephrostomy or Indwelling DJ stent.)
21. Surgical Treatment :
Surgery is Primary treatment & potentially curative
Treatment of retroperitoneal fibrosis :
Bilateral ureterolysis, even if single ureter invloved.
Omental wrap: In extensive retroperitoneal fibrosis cases,
surround the ureters with omentum and reposition them within the peritoneal
cavity.
Treatment of retroperitoneal benign swelling & cyst
Simple ENUCLEATION is sufficient
Treatment of retroperitoneal Metastatic lymph nodes
Retroperitoneal lymph node dissection (RPLND) –
Nerve Sparing Technique is preferable
Approach : Open /Laproscopy
Indication: Stage IIC/ III seminomatous tumor
Stage I /II nonseminomatous tumor
22. En bloc resection with complete clearance of margin is
standard treatment for sarcomas (malignancy)
• 40 to 60% are amenable to complete surgical resection.
• Nephrectomy (42%) followed by colectomy (30%) resection of intestine are most
common adjunctive surgery .
• Positive surgical margins are associated with high local recurrence.( 50% in 5 YRS )
• Radical lymphadenectomy generally not indicated.
Indications:
Localized resectable tumor
Resectable tumor with isolated liver or pulmonary mets
Palliative debulking in Symptonmatic unresectable tuor
Treatment of retroperitoneal sarcoma
23. • Approach : Open/Lap/Robotics
• Access : Inrtaperitoneal/ Retroperitoneal
open intraperitoneal is most favoured
Robotics approach has shown to decrease morbidity and
mortality in retroperitoneal tumor of size less than 3 cm.
Incision : midline, rooftop (cheveron) ,
thoracoabdominal, Subcoastal
Cattell maneuver approach to exposure of retroperitoneal
structures from Right-sided
Mattox maneuver to expose retroperitoneum from left side
24. Contraindications of surgery :
Tumor invading major vascular structure
Multiple Distant metastasis
Gross peritoneal invasion / peritoneal disease
Patient not fit for major surgery
• Complications of surgery :
hemorrhage
Damage to adjacent bowel , kidney , ureter, nerve
Chylous ascitis
retrograde ejaculation
Application of metallic clips at
surgical fields is essential. It acts as
a guide for planning post op
radiotherapy
25. Radiation : improves local control
Preoperative radiotherapy followed by wide ( 50 Gy in 25
fractions ).
Postoperative radiotherapy( 60 Gy in 30 Fractions )
Primary radiotherapy
IORT ( INTRA OPERATIVE RADIOTHERAPY )
EBRT / IMRT
– GI and neurotoxicities limits delivery of sufficient doses
28. Overveiw of management of
Retoperitoneal sarcoma
• Stage I – surgery
• Stage II - pre-op radiation + surgery + post op
radiation
• Stage III - Neoadjuvant chemo-radiotherapy +
Surgery
• Stage IV – Palliative CTRT
29. Treatment of recurrent retroperitoneal
tumors
• Surgery is Primary treatment
• Palliative Chemoradiation : Recommended
only for patients with unresectable or
progressive disease
30. Points to be noted
• Lymphoma is most common retroperitoneal tumor
• Liposarcoma is most common primary reroperitoneal
tuomr
• Retroperitoneal sarcoma has got worse prognosis among
all soft tissue tumor
• Liver followed by peritoneum is most common site of
distant metasatsisof retroperitoneal tumor .
• FNAC has got no role is retroperitoneal sarcoma .
• CECT is investigation of choice for the retroperitoneal
lesion.
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