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Retroperitoneal
Sarcoma
SHAY DAJI
10/10/20
HPI
 CC: “Fever and chills”
 40 y/o M w/ PMH of RLE Myxoid Liposarcoma (T2N0) s/p neoadjuvant RT presented with
imaging findings concerning for oligometastatic recurrence in RLL juxtapleural and L suprarenal
space
PMH: Asthma, R-popliteal fossa Myxoid Liposarcoma, GERD
FH: Unremarkable; Neg for malignancy
SH: Denies tobacco, alcohol abuse, and recreational drug use. Divorced w/ 2 young children.
Systems engineer at CMU. Strong support system.
Meds: Ondansetron, Loratadine, Miralax
Oncological History
Initially dx after palpated a mass at the back of his right knee. MRI in 09/2017 showed possible
sarcoma, measuring 7.7 x 4.6 x 11.4 cm heterogeneous mass.
Ultrasound-guided bx on 11/14/17 noted myxoid liposarcoma, no round cells identified. FISH
+CHOP.
Neoadjuvant RT completed 1/30/18, 50 Gy in 25 fractions
Surgical resection of the mass on 3/12/18. Pathology noted Myxoid Liposarcoma, measuring 9.5-
cm, necrosis 30%, treatment effect present, viable tumor % was <10%, Mitotic rate 0/10 hpf,
margins were negative for tumor. Stage T2N0. Grade could not be assessed (s/p neoadj tx).
No adjuvant chemotherapy recommended.
June 2018 showed a fluid collection in the surgical bed. Sent for cytology which was negative for
malignancy.
Physical Exam / ROS / Labs
General: No acute distress, lying comfortably in bed. Calm, answers questions appropriately,
Respiratory: Lungs CTBA, Respirations are non-labored, Breath sounds are equal, Symmetrical
chest wall expansion, No chest wall tenderness.
Cardiovascular: Normal rate, Regular rhythm. No m/r/g.
Gastrointestinal: Soft, Non-tender to palpation, Non-distended. Normoactive BS. No rebound
tenderness.
Eye, HENT, GU, MSK, Neuro, Psych exam all unremarkable.
 14 point ROS neg except for fevers/chills
Vitals: Afebrile. HDS.
Labs:
April 2020 CT showed new posteromedial RLL pleural-based mass as well as left suprarenal
lesion (in hindsight had grown since March 2019 CT from 1.2-cm).
CT-guided bx of the juxtapleural mass and left suprarenal mass on 4/27/20. Pathology noted
metastatic myxoid liposarcoma at both locations
Radical Resection of 8cm L Retroperitoneal Mass
Post-op
Transferred to the PACU in stable condition and then transferred to floor.
Able to ambulate and void. Denied CP, SoB, and n/v
Pain well controlled with Lidocaine paravertebral catheter, Dilaudid.
Tachycardic to 110s on 10/4, did not respond to fluid bolus, obtained CT AP on 10/5 CT AP showing
partially loculated fluid collection containing gas locules, measuring 9 x 5.6 x 11 cm (AP x transverse x
craniocaudal) in the surgical bed, c/f pancreatic leak s/p IR drain placement on 10/5.
Fluid cx showed no growth. Fluid Amylase 488 (10/5) -> 22 (10/7).
Continued to improve, vital signs remained stable, patient remained afebrile, tolerating PO,
ambulating without assistance, surgical wounds healed without signs of infection or complications ->
discharged to home on 10/7/20.
Scheduled for resection of juxtapleural metastasis in November
Sent specimen to Pathology for margins  consider RT if not completely resected
Post-op
Transferred to the PACU in stable condition and then transferred to floor.
Able to ambulate and void. Denied CP, SoB, and n/v
Pain well controlled with Lidocaine paravertebral catheter, Dilaudid.
Tachycardic to 110s on 10/4, did not respond to fluid bolus, obtained CT AP on 10/5 CT AP showing
partially loculated fluid collection containing gas locules, measuring 9 x 5.6 x 11 cm (AP x transverse x
craniocaudal) in the surgical bed, c/f pancreatic leak s/p IR drain placement on 10/5.
Fluid cx showed no growth. Fluid Amylase 488 (10/5) -> 22 (10/7).
Continued to improve, vital signs remained stable, patient remained afebrile, tolerating PO,
ambulating without assistance, surgical wounds healed without signs of infection or complications ->
discharged to home on 10/7/20.
Scheduled for resection of juxtapleural metastasis in November
Sent specimen to Pathology for margins
Pathology
Histologic sections demonstrated a conventional myxoid liposarcoma. There are focal areas with
modestly increased cellularity “transitional areas”; however, high-grade transformation (round cell
change) is not identified.
Morphology supports the diagnosis of Myxoid Liposarcoma.
Retroperitoneal Sarcomas
• Sarcomas = malignant tumors arising from connective tissue
• Most commonly osteosarcoma
• Retroperitoneal sarcomas constitute 10-15% all sarcomas
• 80% malignant
• Majority of patients presenting with retroperitoneal, extravisceral, solitary
soft tissue mass found to have a sarcoma
• Typically asymptomatic until mass effect
• Median 15 cm at time of presentation
• Distant mets at presentation in 10% pts; spread hematogenously most
commonly to the lungs
Retroperitoneal Sarcomas
Preferred diagnostic workup -> A/P CT scan
Chest CT to rule out metastatic disease to the lungs
Low threshold for percutaneous core needle biopsy -> low risk of complications
Surgical resection has traditionally been the only potentially curative approach.
Resection with microscopically negative margins is often not achieved, and locoregional
recurrence is common (especially low-grade tumors)
Role of adjunctive therapy is unclear -> no consensus as to the best approach for all
patients
The main prognostic factors for long-term outcome are resectability, histologic grade of
differentiation, and histologic subtype.

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Retroperitoneal Sarcoma Case.pptx

  • 2. HPI  CC: “Fever and chills”  40 y/o M w/ PMH of RLE Myxoid Liposarcoma (T2N0) s/p neoadjuvant RT presented with imaging findings concerning for oligometastatic recurrence in RLL juxtapleural and L suprarenal space PMH: Asthma, R-popliteal fossa Myxoid Liposarcoma, GERD FH: Unremarkable; Neg for malignancy SH: Denies tobacco, alcohol abuse, and recreational drug use. Divorced w/ 2 young children. Systems engineer at CMU. Strong support system. Meds: Ondansetron, Loratadine, Miralax
  • 3. Oncological History Initially dx after palpated a mass at the back of his right knee. MRI in 09/2017 showed possible sarcoma, measuring 7.7 x 4.6 x 11.4 cm heterogeneous mass. Ultrasound-guided bx on 11/14/17 noted myxoid liposarcoma, no round cells identified. FISH +CHOP. Neoadjuvant RT completed 1/30/18, 50 Gy in 25 fractions Surgical resection of the mass on 3/12/18. Pathology noted Myxoid Liposarcoma, measuring 9.5- cm, necrosis 30%, treatment effect present, viable tumor % was <10%, Mitotic rate 0/10 hpf, margins were negative for tumor. Stage T2N0. Grade could not be assessed (s/p neoadj tx). No adjuvant chemotherapy recommended. June 2018 showed a fluid collection in the surgical bed. Sent for cytology which was negative for malignancy.
  • 4. Physical Exam / ROS / Labs General: No acute distress, lying comfortably in bed. Calm, answers questions appropriately, Respiratory: Lungs CTBA, Respirations are non-labored, Breath sounds are equal, Symmetrical chest wall expansion, No chest wall tenderness. Cardiovascular: Normal rate, Regular rhythm. No m/r/g. Gastrointestinal: Soft, Non-tender to palpation, Non-distended. Normoactive BS. No rebound tenderness. Eye, HENT, GU, MSK, Neuro, Psych exam all unremarkable.  14 point ROS neg except for fevers/chills Vitals: Afebrile. HDS. Labs:
  • 5. April 2020 CT showed new posteromedial RLL pleural-based mass as well as left suprarenal lesion (in hindsight had grown since March 2019 CT from 1.2-cm).
  • 6. CT-guided bx of the juxtapleural mass and left suprarenal mass on 4/27/20. Pathology noted metastatic myxoid liposarcoma at both locations
  • 7. Radical Resection of 8cm L Retroperitoneal Mass
  • 8.
  • 9. Post-op Transferred to the PACU in stable condition and then transferred to floor. Able to ambulate and void. Denied CP, SoB, and n/v Pain well controlled with Lidocaine paravertebral catheter, Dilaudid. Tachycardic to 110s on 10/4, did not respond to fluid bolus, obtained CT AP on 10/5 CT AP showing partially loculated fluid collection containing gas locules, measuring 9 x 5.6 x 11 cm (AP x transverse x craniocaudal) in the surgical bed, c/f pancreatic leak s/p IR drain placement on 10/5. Fluid cx showed no growth. Fluid Amylase 488 (10/5) -> 22 (10/7). Continued to improve, vital signs remained stable, patient remained afebrile, tolerating PO, ambulating without assistance, surgical wounds healed without signs of infection or complications -> discharged to home on 10/7/20. Scheduled for resection of juxtapleural metastasis in November Sent specimen to Pathology for margins  consider RT if not completely resected
  • 10.
  • 11. Post-op Transferred to the PACU in stable condition and then transferred to floor. Able to ambulate and void. Denied CP, SoB, and n/v Pain well controlled with Lidocaine paravertebral catheter, Dilaudid. Tachycardic to 110s on 10/4, did not respond to fluid bolus, obtained CT AP on 10/5 CT AP showing partially loculated fluid collection containing gas locules, measuring 9 x 5.6 x 11 cm (AP x transverse x craniocaudal) in the surgical bed, c/f pancreatic leak s/p IR drain placement on 10/5. Fluid cx showed no growth. Fluid Amylase 488 (10/5) -> 22 (10/7). Continued to improve, vital signs remained stable, patient remained afebrile, tolerating PO, ambulating without assistance, surgical wounds healed without signs of infection or complications -> discharged to home on 10/7/20. Scheduled for resection of juxtapleural metastasis in November Sent specimen to Pathology for margins
  • 12. Pathology Histologic sections demonstrated a conventional myxoid liposarcoma. There are focal areas with modestly increased cellularity “transitional areas”; however, high-grade transformation (round cell change) is not identified. Morphology supports the diagnosis of Myxoid Liposarcoma.
  • 13. Retroperitoneal Sarcomas • Sarcomas = malignant tumors arising from connective tissue • Most commonly osteosarcoma • Retroperitoneal sarcomas constitute 10-15% all sarcomas • 80% malignant • Majority of patients presenting with retroperitoneal, extravisceral, solitary soft tissue mass found to have a sarcoma • Typically asymptomatic until mass effect • Median 15 cm at time of presentation • Distant mets at presentation in 10% pts; spread hematogenously most commonly to the lungs
  • 14. Retroperitoneal Sarcomas Preferred diagnostic workup -> A/P CT scan Chest CT to rule out metastatic disease to the lungs Low threshold for percutaneous core needle biopsy -> low risk of complications Surgical resection has traditionally been the only potentially curative approach. Resection with microscopically negative margins is often not achieved, and locoregional recurrence is common (especially low-grade tumors) Role of adjunctive therapy is unclear -> no consensus as to the best approach for all patients The main prognostic factors for long-term outcome are resectability, histologic grade of differentiation, and histologic subtype.

Editor's Notes

  1. Initially thought to be a Bakers cyst
  2. Gy = gray Chop – fusion protein which confirmed the diagnosis.
  3. Left subcostal incision. Mobilized the omentum off the transverse colon to enter the lesser sac. Mobilized the splenic flexure and moved it and the left colon medially. Then retracted the inferior edge of the pancreas superiorly allowing us to visualized the kidney. completely removed all of the fat in the left retroperitoneum up to the diaphragm as well as a portion of the left adrenal gland.
  4. Preferred diagnostic workup -> A/P CT scan Chest CT to rule out metastatic disease to the lungs  Low threshold for percutaneous core needle biopsy -> low risk of complications and the unsubstantiated fear of tumor seeding of the biopsy tract are far outweighed by the information obtained from the biopsy Surgical resection has traditionally been the only potentially curative approach. Because of the typically large size and anatomic complexity of these tumors at diagnosis, a resection with microscopically negative margins (R0 resection) is often not achieved, and locoregional recurrence is common, especially for low-grade tumors. Yet, the role of adjunctive therapy (RT, chemotherapy; either given preoperatively or postoperatively) continues to be debated, and there is no consensus as to the best approach for all patients. The main prognostic factors for long-term outcome are resectability, histologic grade of differentiation, and histologic subtype.