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Introduction & History.
Introduction & History.
Retroperitoneal Tumours
Solid Neoplastic Retroperitoneal mass
Fetus in Fetus Fetal
skull with hairs TERATOMA
Cystic Neoplastic Retroperitoneal mass
Non- Neoplastic Retroperitoneal mass
Solid Cystic
Retroperitoneal fibrosis
( ORMOND’S Disease)
Hematoma
urinoma
Pseudocyst
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
4) Constitutional symptoms:
• Fatigue
• Weakness
• fever
• Loss of Appetite
• Loss of weight
• Back Pain
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).
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-
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
Investigation
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.
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.
Indications of preoperative biopsy
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
Medical management :
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.)
Chemotherapy for retroperitoneal
metastatic lymphnode
• BEP : Bleomycin , Etoposide , Cisplatin
• Indication : in unresectable
lymphnode ( Stage 3 germ cell tumor )
Indications: neoadjuvant
unresectable tumor ( Palliative )
Distant metastasis
Treatment regimens :
AIM : Adriyamycin , Iphosphamide , Mesna
MAID: Mesna , Iphosphamide, Adriyamycin, Dacarbazine
Chemotherapy for retroperitoneal sarcoma :
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
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
• 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
 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
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
ALGORITHM FOR MANAGEMENT OF
RETROPERITONEAL SARCOMAS
PRIMARY RESECTABLE
RETROPERITONEAL SARCOMA
BIOPSY & NEOADJUVANT CHEMOR
FOLLOW CLINICALLY
RESECTION
< 5CM , LOCALIZED ,
NO NECROSIS
> 5CM , LOCALIZED ,
NECROSIS &
CALCIFICATION
RESECTION
MANAGEMENT OF LOCALLY ADVANCED &
METASTATIC DISEASE
LOCALLY ADVANCED & METASTATIC
RETROPERITONEAL SARCOMA
ASYMPTOMATIC MECHANICAL SYMPTOMS
PALLIATIVE RESECTION
CHEMOTHERAPY
± RADIATION THERAPY
CLINICAL
OBSERVATION
CHEMOTHERAPY
+RADIATION THERAPY
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
Treatment of recurrent retroperitoneal
tumors
• Surgery is Primary treatment
• Palliative Chemoradiation : Recommended
only for patients with unresectable or
progressive disease
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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Retroperitoneal tumours.pptx

  • 1. Tips on using my ppt. 1. You can freely download, edit, modify and put your name etc. 2. Don’t be concerned about number of slides. Half the slides are blanks except for the title. 3. First show the blank slides (eg. Aetiology ) > Ask students what they already know about ethology of today's topic. > Then show next slide which enumerates aetiologies. 4. At the end rerun the show – show blank> ask questions > show next slide. 5. This will be an ACTIVE LEARNING SESSION x three revisions. 6. Good for self study also. Good for self study also. Display blank slide> Think what you already know about this > Read next slide.
  • 4. Solid Neoplastic Retroperitoneal mass Fetus in Fetus Fetal skull with hairs TERATOMA
  • 6. Non- Neoplastic Retroperitoneal mass Solid Cystic Retroperitoneal fibrosis ( ORMOND’S Disease) Hematoma urinoma Pseudocyst
  • 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
  • 8. 4) Constitutional symptoms: • Fatigue • Weakness • fever • Loss of Appetite • Loss of weight • Back Pain
  • 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.)
  • 19. Chemotherapy for retroperitoneal metastatic lymphnode • BEP : Bleomycin , Etoposide , Cisplatin • Indication : in unresectable lymphnode ( Stage 3 germ cell tumor )
  • 20. Indications: neoadjuvant unresectable tumor ( Palliative ) Distant metastasis Treatment regimens : AIM : Adriyamycin , Iphosphamide , Mesna MAID: Mesna , Iphosphamide, Adriyamycin, Dacarbazine Chemotherapy for retroperitoneal sarcoma :
  • 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
  • 26. ALGORITHM FOR MANAGEMENT OF RETROPERITONEAL SARCOMAS PRIMARY RESECTABLE RETROPERITONEAL SARCOMA BIOPSY & NEOADJUVANT CHEMOR FOLLOW CLINICALLY RESECTION < 5CM , LOCALIZED , NO NECROSIS > 5CM , LOCALIZED , NECROSIS & CALCIFICATION RESECTION
  • 27. MANAGEMENT OF LOCALLY ADVANCED & METASTATIC DISEASE LOCALLY ADVANCED & METASTATIC RETROPERITONEAL SARCOMA ASYMPTOMATIC MECHANICAL SYMPTOMS PALLIATIVE RESECTION CHEMOTHERAPY ± RADIATION THERAPY CLINICAL OBSERVATION CHEMOTHERAPY +RADIATION THERAPY
  • 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.
  • 31. Get this ppt in mobile 1. Download Microsoft PowerPoint from play store. 2. Open Google assistant 3. Open Google lens. 4. Scan qr code from next slide.
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  2. drpradeeppande@gmail.com 7697305442