This entails discussion about primary retroperitoneal masses and few commmon other retroperitoneal mass

1. Etiology, investigations &Etiology, investigations &
Management ofManagement of
Retroperitoneal MassRetroperitoneal Mass
PROF. Dr A. B. SINGH UNITPROF. Dr A. B. SINGH UNIT
Department of SurgeryDepartment of Surgery
Patna Medical College & HospitalPatna Medical College & Hospital

2. Objective…
 Retroperitoneal anatomy
 Etiology of retroperitoneal mass
 Clinical features of retroperitoneal mass
 Investigations of retroperitoneal mass
Management of retroperitoneal mass

4. Retroperitoneum
• Boundary
Anteriorly : posterior parietal
peritoneum
Posteriorly : Vetebral column,
iliopsoas ,quadratus lumborum muscle
and tendinous part of transverse
abdominis
Superiorly : Diaphragm
Inferiorly : Levator Ani and Pelvic
Diaphragm

5. It is divided into three spaces by the peri renal fascia
i.e. fascia of Gerota
The Three spaces are:
 anterior pararenal space
Colon, Pancreas, Duodenum
 perirenal space
Kidneys, Adrenal glands
Upper portion of ureters
 posterior pararenal space
Fat , connective tissue,
nerves

6. Causes of Retroperitoneal
Swelling

7. Solid Neoplastic Retroperitoneal mass
Solid tumor from other site
lymphoma
Metastatic germ cell tumor

8. Solid Neoplastic Retroperitoneal mass
Fetus in Fetus Fetal skull
with hairs TERATOMA

9. Cystic Neoplastic Retroperitoneal mass

10. Non- Neoplastic Retroperitoneal mass
Solid Cystic
Retroperitoneal fibrosis
( ORMOND’S Disease)
Hematoma
urinoma
Pseudocyst

11. CliniCal
presentation and
investigations

12. 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

13. 4) Constitutional symptoms:
• Fatigue
• Weakness
• fever
• Loss of Appetite
• Loss of weight
• Back Pain

14. Symptoms and 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).

15. 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-

16. 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

17. Investigations of
retroperitoneal mass

18. 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.

19. 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
An unusual appearing mass
Unresectable tumor
Distant mets
Patient being considered for
neoadjuvant chemotherapy

20. CECT/ MRI- showing retroperitoneal
tumours
1) LIPOSARCOMA:
well differentiated liposarcoma
shwoing huge heterogenous mass
with predominantly fat
attenuation.
Fat attenuation
2) LIPOMA:
T1 weighted mri
Homogenous high signal
intensity mass
Kidney displaced upward

21. 3) leiomyosarcoma
Necrosis
CT angiogram: The aorta and IVC
anteriorly displaced, producing the
“floating aorta sign’’ in case of
retroperitoneal lymphoma .
4) Lymphoma
IVC
Aorta

22. CT Scan : showing Fetal skeleton CT Scan : showing Fetal skeleton
FETUS IN FETU is differentiated from teratoma by the presence of vertebral
organization with limb buds and organ systems.
FETUS IN FETU is differentiated from teratoma by the presence of vertebral
organization with limb buds and organ systems.

23. IVU shows medial deviation of
middle part of both ureters
Retroperitoneal fibrosis
T2- MRI: Mature teratoma with fat fluid
level and calcification
Retroperitoneal Teratoma

24. Histopathology of retroperitoneal
tumours
1)Histology of normal adipose tissue 2) Histology of retroperitoneal lipoma
3) Well differentiated liposarcoma 4) Dedifferentiated liposarcoma
Irregular cells with hyperchromatic nuclei
Mature white
cell with no
pleomorphism
lipoblast

25. 5)Malignant fibrous histiocytoma:
Storiform arrangement of
multinucleate cells and matrix
6b) leiomyosarcoma
Cells are arranged in wavy sheet with cigar shaped nuclei
6a)Histology of normal smooth
muscle

26. 7b) Rhabdomyosarcoma
eosinophilic granular cytoplasm
with either round or elongated
cells( tadpole)
7a)Histology of skeletal muscle
polygonal cells
rhabdomyoblasts

27. • Liposarcoma is the most common (33%)
primary retroperitoneal sarcoma
• four subtypes: well-differentiated, myxoid,
pleomorphic, and round cell subtypes.
• predominantly hypoattenuating lesion on CT
images because of its fat content.
• Calcification is seen in as many as 30% of cases
• The overall prognosis for patients with
retroperitoneal tumors is worse than that for
patients with extremity sarcomas.
• Occasionally, a portion of the well-differentiated
liposarcoma undergoes histologic
dedifferentiation and becomes more aggressive
and metastatic and then carries a worse
prognosis.

28. TNM STAGINGTNM STAGING
*Superficial tumor is located
exclusively above the superficial
fascia without
invasion of the fascia;
Deep tumor is located exclusively
beneath the
superficial fascia, superficial to
the fascia with invasion of or
through the
fascia, or both superficial yet
beneath the fascia.

29. HISTOLOGICAL GARDE AND TUMOR SIZE IS MOST
IMPORTANT PROGNOSTIC FACTORS FOR
RETROPERITONEAL SARCOMA
HISTOLOGICAL GARDE AND TUMOR SIZE IS MOST
IMPORTANT PROGNOSTIC FACTORS FOR
RETROPERITONEAL SARCOMA

30. Schwannoma.
• Benign tumor that arises from the
perineural sheath of Schwann cell.(neurilemma)
• 6% of retroperitoneal neoplasms
• more common in females (2:1)
• usually asymptomatic
• Microscopically, the tumor is composed of alternating
Antoni A and Antoni B areas.
• At CT, small schwannomas are round, well defined,
and homogeneous, but large schwannomas may be
heterogeneous in appearance.
• Calcification can be punctate, mottled, or curvilinear

31. Teratoma
• Germ cell tumor
• Less than 10% of teratomas are
found in the retroperitoneum.
• The third most common tumor in the retroperitoneum in
children, after neuroblastoma and Wilms tumor
• More common in females, with a bimodal age distribution (<6
months and early adulthood).
• Mature teratoma (dermoid cyst) contains well-differentiated
tissues from at least two germ cell layers.
• Mature teratomas are predominantly cystic.
• Calcification (toothlike or well defined) and fat can be seen in
56% and 93% of cases, respectively

33. FETUS IN FETU
♣ Most common site is retroperitoneum,
however it has been reported in other sites
as well from cranial cavity to the scrotal
sac
♣ It is differentiated from teratoma by the
presence of vertebral organization with
limb buds and organ systems.
♣ Unlike teratomas, it is a benign condition.

34. CT Scan : showing Fetal skeleton
CT Scan : showing Fetal skeleton
Resected specimen Fetal skull with hairs

35. Lymphoma
• Most common retroperitoneal malignancy,
accounting for 33%
• seen in the 40–70-year age group
• frequently manifests with extranodal disease in the liver,
spleen, or bowel, often at an advanced stage.
• History of fever , myalgia , night sweats , weight loss
• Paraaortic lymph nodes are involved in 25% of the
patients with Hodgkin lymphoma and 55% of the
patients with non-Hodgkin lymphoma.

36. • At CT, lymphoma is seen as a well-defined
homogeneous mass, with mild
homogeneous contrast enhancement.
• The aorta and IVC can be anteriorly
displaced, producing the “floating aorta” or
“CT angiogram” sign

37. Retroperitoneal hematoma
• Caused by trauma, blood dyscrasia,
anticoagulation therapy, rupture of an
abdominal aortic aneurysm, or
interventional or surgical procedures.
• low signal intensity on MR images
because of hemosiderin deposition.
• Occasionally, the heterogeneous
appearance on contrast-enhanced CT
images can be confused with a sarcoma,
but the well-defined margin, the
absence of contrast enhancement,
and the changing appearance with
time, with a progressive decrease in
size, distinguish retroperitoneal
hematoma from sarcoma

39. Urinoma
• A collection of extravasated urine that is
found secondary to trauma or iatrogenic
causes.
• A well-defined cystic lesion is seen in
the retroperitoneum, more commonly in
the perirenal space.
• CT shows a well-defined fluid collection
with progressively increasing
attenuation caused by contrast-
enhanced urine entering the urinoma

40. Retroperitoneal fibrosis
• A fibro-inflammatory mass envelops and
potentially obstructs retroperitoneal
structures, including either or both ureters.
• Fibrous, whitish plaque encases aorta, IVC
& their major branches, ureters, other
retroperitoneal structures, at times
intraperitoneal structures including GIT.
• Idiopathic-70%(Ormond’s disease):
Inciting etiology not defined.
• Definitive etiology in 30%.
• M.C.symptom(92%)-dull, poorly
localized, noncolicky pain in flank, back,
or lower abdomen. Unrelated to posture
IVU shows medial deviation of middle
part of both ureters

41. Treatment of
retroperitoneal mass

42. 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.)

43. Chemotherapy for retroperitoneal sarcoma :
Indications: neoadjuvant
unresectable tumor ( Palliative )
Distant metastasis
Treatment regimens :
AIM : Adriyamycin , Iphosphamide , Mesna
MAID: Mesna , Iphosphamide, Adriyamycin, Dacarbazine
Chemotherapy for retroperitoneal metastatic lymphnode :
BEP : Bleomycin , Etoposide , Cisplatin
Indication : in unresectable lymphnode ( Stage 3 germ cell tumor )

44. 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

45. 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

46. • 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

47. Mattox maneuver to expose
retroperitoneum from left side
Right-sided approach to exposure of retroperitoneal structures
using Cattell maneuver

48.  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
Application of metallic clips at
surgical fields is essential. It
acts as a guide for planning
post op radiotherapy

50. 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

51. ALGORITHM FOR MANAGEMENT OF
RETROPERITONEAL SARCOMAS
PRIMARY RESECTABLE
RETROPERITONEAL SARCOMA
BIOPSY & NEOADJUVANT CHEMORADIATION
FOLLOW CLINICALLY
RESECTION
< 5CM , LOCALIZED , NO
NECROSIS
> 5CM , LOCALIZED , NECROSIS &
CALCIFICATION
RESECTION

52. MANAGEMENT OF LOCALLY ADVANCED &
METASTATIC DISEASE
LOCALLY ADVANCED & METASTATIC
RETROPERITONEAL SARCOMA
ASYMPTOMATIC MECHANICAL SYMPTOMS
PALLIATIVE RESECTION
CHEMOTHERAPY
± RADIATION THERAPY
CLINICAL
OBSERVATION
CHEMOTHERAPY
+RADIATION THERAPY

53. 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

54. Treatment of recurrent retroperitoneal tumors
• Surgery is Primary treatment
• Palliative Chemoradiation : Recommended only for
patients with unresectable or progressive disease.

55. Key Facts :
• 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.