History:
- Personal or familial history of malignancy, lymphoma
- Hereditary syndromes – neurofibromatosis, MEN, Lynch, TSC, retinoblastoma, Li Fraumeni
- Symptoms – abdominal, gastrointestinal, urinary, pain, endocrinopathies (paraganglioma)
- Fertility / family planning
- Medical co-morbidities – anticoagulation, immunosuppression, threats to future renal function, fitness for GA
- Surgical history and prior procedures
- Previous radiation therapy
Exam:
- BMI, performance status, frailty
- Blood pressure
- Abdominal mass, scars, flank mass
- Scrotal exam / pelvic exam / DRE
- Lymphadenopathy
- Stigmata of familial syndromes (neurofibromas)
Investigations:
- Bloods
- PSA, testis tumour markers, ovarian tumour markers
- Plasma metanephrines
- FBC / UEC / LFT / coags
- Imaging
- CT C/A/P with contrast gold standard
- FDG PET / MRI in select cases
- Scrotal ultrasound
CT findings in retroperitoneal tumours:
- Fat presence (liposarcoma, lipoma, myelolipoma, AML)
- Cystic or solid components
- Other lymphadenopathy
- Organ of origin
- Other metastases
- Visceral involvement or invasion
- Vascular involvement or invasion
- Feasibility of resection with margins
Biopsy?
- If imaging is classic or suggestive of sarcoma, may proceed straight to surgical resection without biopsy
- If MDT consensus for histology – retroperitoneal percutaneous CT guided biopsy with multiple passes in co-axial technique, aiming for solid viable components
Potential considerations for retroperitoneal mass surgery (and auxiliary surgery)
Right side
- Duodenum
- Liver – requiring mobilisation or resection, HPB involvement
- Cava – requiring resection, graft, hepatic mobilisation
- Adrenal vein
Left side
- Spleen and tail of pancreas
- Aorta
General masses:
- Diaphragm involvement
- Ureteric involvement – needing nephrectomy or ureteric stenting
- Lumbar vessels
- If inferior retroperitoneum – sympathetic chain, fertility or ejaculatory concerns
Retroperitoneal sarcoma
12 – 15 % of all sarcomas
Sarcomas arise from tissues of mesodermal origin.
One third of retroperitoneal masses
Typically incidental finding on imaging – can grow to large sizes without detection – mean 15 – 20 cm at diagnosis
Complete resection is only curative option – first operation is the best operation.
- Aim is always for R0 resection – may require extensive resection of adjacent organs
- MDT management mandatory – heterogenous group of diseases with different responses to radiation and chemotherapy
- Even with complete R0 resection, often recurs.
Retroperitoneal tumour, particularly fat containing, if not clearly arising from kidney or adrenal, is a retroperitoneal sarcoma until proven otherwise.
Liposarcoma – may be well differentiated or dedifferentiated
Leiomyosarcoma – more common in younger patients
Other rarer types – solitary fibrous tumour, synovial sarcoma, Ewing’s sarcoma, malignant nerve sheath tumour
Neoadjuvant chemotherapy given for Ewing’s sarcoma.
Neoadjuvant and adjuvant radiation is case-by-case and being studied.
Sarcoma can also occur in spermatic cord, bladder, kidney.