Retroperitoneal sarcoma what is it

Careful monitoring and the involvement of an experienced surgical oncologist are important to the successful outcome for patients with RPS. If you are having surgery or a procedure, you will likely be scheduled for a visit to the Weiner Center for Preoperative Evaluation for pre-operative information and tests. The day of surgery, you will be cared for in the operating room by surgeons, anesthesiologists and nurses who specialize in surgery for patients with RPS.

After surgery, you will recover in the post-surgical care unit where you will receive comprehensive care by an experienced surgical and nursing staff. Learn more about your hospital stay and returning home. Our treatment team includes surgical oncologists, medical and radiation oncologists, plastic surgeons, nutritionists, pathologists and anesthesiologists.

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Stay Informed. In the retroperitoneum, the two most common subtypes are liposarcoma and leiomyosarcoma, cancers that originate from fat and smooth muscle, respectively.

These two can have very different behaviors. Even within the most common subtype, liposarcoma, patients can have tumors that range from slow growing, without the capability to spread, to those that are infiltrating, aggressive and rapidly spreading. Understanding these differences affects our approach to treatment, including, for example, how extensive to be with surgery or whether to give chemotherapy. Knowing the beast on the inside through research.

I also believe that we should focus on not just the bad cells cancer but also the good ones immune cells that actually make it into and reside in the tumor itself. Given the large tumor size, there is certainly more than enough material to study!

Working with others to fight the beast. Last year, I had the unique opportunity to do retroperitoneal sarcoma surgery with colleagues in Milan, Italy, at one of the largest specialty centers in the world for this rare cancer.

For me, this was a wonderful professional experience and continues to be rewarding to this day, as we continue to learn from each other, share experiences and discuss ways to potentially incorporate new research findings to optimize treatment.

En bloc resection of the tumor was performed with any adjacent involved structures such as diaphragm, kidney, and colon, including, as needed, on the left side the spleen, the tail of the pancreas, and a portion of the stomach, and on the right side, a portion of the right lobe of the liver.

For retroperitoneal sarcomas of the flank, the patient was also placed in a lateral position and a flank transperitoneal approach was used combined in a "T" extension with a midline incision. The tumor was mobilized first off all other attachments posterolateral and anterior , and then medial attachments to the aorta or inferior vena cava were dealt with. Retroperitoneal sarcomas of the midabdomen and midpelvis were approached through a midline incision with the patient in a supine position.

Exposure of the superior mesenteric vessels was carried out for tumors at the base of the mesentery to determine resectability and provide the widest and safest margin around the tumor. For midline pelvic sarcomas, a lower midline incision was extended to the pubic symphysis and thence transversely to the pubic tubercle on each side, transecting the rectus abdominis and the rectus sheath off the pubic crest. For sarcomas of the lower abdominal quadrants with fixation to the iliac fossa, the wall of the lesser pelvis, or the external iliac vessels, the abdominoinguinal incision 4 was used to provide exposure with the patient in a supine position.

Rarely, hemipelvectomy was required for pelvic sarcomas with extensive invasion to one side wall of the pelvis. There were no postoperative deaths. The improved survival after wide resection over that of local resection could not be explained on any bias resulting from a favorable grade distribution Table 3.

The survival rates according to grade also varied significantly within the subgroups of primary and locally recurrent tumors Table 2 Figure 3 and Figure 4. Retroperitoneal sarcomas usually attain a large size before they cause any symptoms for the patients, and they are often detected because of a palpable mass without any attendant symptoms.

In our series, only 3 patients had a tumor mass less than 5 cm in diameter. Often the wrong strategy is followed, ie, insistence on first separating the tumor mass from the major retroperitoneal vessels, eg, aorta and inferior vena cava, as an early determinant of resectability, which is a superficially logical policy but often induces the surgeon to abandon the procedure, as it carries a perceivable and actual risk of uncontrollable hemorrhage.

The correct strategy for a mass abutting the great retroperitoneal vessels is to mobilize it all the way around, from its anterior, lateral, and posterior attachments, and then as the last step to separate it from the aorta or inferior vena cava, as the case may be. These estimates approach those of extremity soft tissue sarcomas. The major problem of retroperitoneal sarcomas is that of local recurrence after an apparent complete resection because of the difficulties in procuring wide margins all the way around the tumor.

However, the rate of local recurrence continues to increase with the years of follow-up Figure 1. The difference in survival between wide and local excision could not be explained on the basis of any appreciable difference in the rate of the various grades encountered in each surgical group Table 3. It is generally accepted that wide resection results in a lower rate of local recurrence compared with local excision; however, the fact that it also leads to a significantly higher survival in retroperitoneal sarcomas has not been previously reported, to our knowledge, and, therefore, this point needs to be further examined in future preferably prospective studies.

After complete resection of the tumor, the most important biological factor to determine survival has been the grade of the retroperitoneal sarcoma.

It is therefore clear that in addition to a high initial resectability rate, a strategy of prompt reoperation for local recurrence is an important component of treatment for a high survival rate in patients with retroperitoneal sarcomas. Corresponding author: Constantine P. RPS comprises a multitude of histopathologic subtypes that are difficult to distinguish based on imaging alone. Similarly, laparotomy or laparoscopic open biopsies should be avoided. Of note, if at open or laparoscopic exploration for suspected adnexal mass, no abnormalities of the uterus, fallopian tubes, or ovaries are found but a retroperitoneal mass is detected, it is recommended that nothing further be done and that a coaxial core needle biopsy be performed after proper imaging.

It is important to realize that there can be more than 70 histologic subtypes of RPS and that, along with grade, they have prognostic significance and guide treatment planning, including the extent of surgical resection.

The most critical component of the treatment of RPS remains the surgical excision, and the best chance for cure is at the time of primary surgery. Surgery should achieve a macroscopically complete excision of the tumor R0 or R1 , minimizing marginality, ideally through an en-bloc resection of all potentially involved structures as determined by careful preoperative imaging in combination with intraoperative findings.

Operative planning also includes the functional assessment of critical organs-eg, the function of each kidney. Contraindications to primary resection are believed to be bilateral renal involvement; encasement of the superior mesenteric artery, celiac axis, and porta hepatis; and spinal cord involvement.

When planning for surgery, it is paramount to take into consideration the histology of the RPS as well as its predicted behavior pattern, as these differ widely. Indeed, the largest transatlantic multi-institutional series identified histologic subtype as a predictor of patterns of local and distant recurrence. In light of these data, clinicians should decide the extent of surgical resection in a multidisciplinary setting at a specialized center after review of imaging and pathology, given that the pattern of growth and prognostic risks vary broadly among the different histologic subtypes.

In addition, it is the one with the least clear separation from normal retroperitoneal fat, given that the well-differentiated component of liposarcoma is virtually undistinguishable from normal fat. As a consequence, the extent of surgery should be aimed at removing all ipsilateral retroperitoneal fat en bloc with the mass at the price of sacrificing at least the ipsilateral kidney and colon and part of or the entire psoas muscle.

Dissection of the inferior vena cava IVC if on the right side or aorta if on the left side, ligating ipsilateral renal vessels and other collaterals and dissection of the iliac vessels. Peritonectomy, resection of the psoas muscle in the pelvis plus rectal resection if on the left side after identification and liberation of the femoral nerve unless directly invaded and possibly of the femoral cutaneous branch, while the genitofemoralis and ilioinguinal nerves are usually resected, as these lie between the tumor and the psoas fascia.

Subcapsular liver dissection or partial hepatectomy are rarely needed for tumors located on the right side, whereas a complete liberation of the right liver lobe is usually of help. Similarly, sleeve gastrectomy or proximal gastrectomy is rarely required for tumors located on the left side.

Leiomyosarcoma and other rarer histologic subtypes such as solitary fibrous tumor are much more well-defined tumors. A wide resection is still required but not necessarily involving the adjacent organs if these are not clearly invaded Figure 2. Extended surgery may raise concern for added morbidity. A recent multi-institutional collaboration, however, found that a radical resection is safe and is associated with low day mortality 1.

Severe complications were associated with increased age, transfusion requirements, and organ resection score, with a more pronounced risk in patients undergoing splenectomy and pancreatectomy and Whipple procedure. Although major vascular resection MVR is associated with higher morbidity, vascular involvement does not preclude resection because it can be safely performed in specialized centers.

In essence, resection of RPS requires technical expertise in multiple sites throughout the abdominal and pelvic cavity, including the handling of large vessels. The ability to orchestrate a team of complementary surgical experts is critical to successful management of RPS patients.

To minimize the risk of intraoperative and perioperative morbidity, RPS resection should be undertaken by surgical teams with expertise in specific aspects of the anatomy of the retroperitoneal space-for example, expertise in retroperitoneal autonomic and somatic nerves, the lymphatic system, paravertebral vessels, and organs of the gastrointestinal tract. Required expertise also includes experience with additional procedures, such as full-thickness thoracoabdominal wall resection and reconstruction, diaphragmatic resection and reconstruction, major vascular resection and reconstruction, and bone resection.

Surgical teams with these abilities, which may accrue from prior participation in multidisciplinary surgical teams, can achieve macroscopically complete tumor resection in the majority of patients. Given the importance of local control in a majority of RPS subtypes, radiation therapy RT has long been an important tool in the multimodal treatment of this disease. There has been great debate, however, over the use of RT in either the adjuvant setting or the neoadjuvant setting, based on lessons learned from extremity sarcomas.

Analysis of retrospective series has shown mixed results. However, the STRASS trial NCT , an international randomized, controlled trial assessing oncologic outcomes in patients undergoing neoadjuvant RT and surgery compared with patients undergoing upfront surgery, has completed accrual and final results are expected to be released at the American Society of Clinical Oncology Annual Meeting.