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28 Cards in this Set

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You do a diagnostic lap on a patient for chronic right lower quadrant pain and you come across peritoneal implants and evidence of old ruptured appendicitis. How will you further evaluate their disease while in the operating room?

Calculate the Peritoneal Cancer Index (Prognostic)


Divides abdomen into 9 regions and divides the jejunum/ileum into 4 segments (proximal/distal)


1 point for tumor <0.5 cm


2 points for tumors 0.5-5cm


3 points for tumors>5 cm


So, scale of 0-39

You do a diagnostic lap on a patient for chronic right lower quadrant pain and you come across peritoneal implants and evidence of old ruptured appendicitis. What is the prognostic value of the PCI?

The cutoff value is 20 points


DPAM >20 60% 20-year survival


DPAM <20 90% 20-year survival

You do a diagnostic lap on a patient for chronic right lower quadrant pain and you come across peritoneal implants and evidence of old ruptured appendicitis. You take out the appendix and close. What are you going to want to know from the pathologist about the peritoneal met?

Whether it is Disseminated Peritoneal Adenomucinosis (DPAM, doesn't invade, better prognosis)


or Peritoneal Mucinous Carcinoma (PMCA)

You do a diagnostic lap on a patient for chronic right lower quadrant pain and you come across peritoneal implants that come back as Peritoneal mucinous carcinoma. You're managing the patient's care. What is going to be your treatment strategy?

Refer to Med-onc: Pre-op chemotherapy (oxaliplatin and 5-FU based)


Schedule Cytoreductive Surgery to get all implants >2.5 mm, possible visceral resections and peritonectomy


HIPEC: Doxorubicin and Mytomycin C based

You see a patient in clinic who was recently diagnosed with Peritoneal mucinous carcinoma with a few large mets. How do you explain the importance of the ability to perform complete resection of the tumors?

Completeness of Cytoreduction (CC)Score is 0 or 1 if all tumor is removed or all tumor greater than 2.5 mm


Complete Resection 10 year survival


DPAM—75%


PMCA—27%


Incomplete Resection 10 year survival


DPAM—25%


PMCA—6%

You take a patient to the OR for sigmoidectomy for colorectal cancer and see peritoneal metastases. How do you proceed?

Complete the Sigmoidectomy


Sample Peritoneal Mets for pathology, if you can remove them all safely, do so.


Calculate PCI


Close and plan for HIPEC

You are seeing a patient in clinic after a sigmoidectomy in which you found peritoneal-based disease. What will you council him on his survival with chemotherapy vs. going forward with cytoreductive surgery and HIPEC?

Median Survival with chemotherapy alone is 12-15 months


HIPEC has median survival >30 months


Also quite morbid, 30% grade 3/4

You are going to see a patient in clinic after a sigmoidectomy in which you found peritoneal-based disease. What are the indications for HIPEC for these patients?

Good performance status


PCI≤20 without other mets


No rapid progression during systemic chemo

You take a patient back to the OR after sigmoidectomy, intending to do CRS and HIPEC for peritoneal metastasis. You realize you cannot safely completely resect one of the large metastases. How do you proceed?

Abort. Anything less than CC1 resection has the same survival as simple palliative chemotherapy. Need to have <1mm residual tumor for HIPEC to penetrate

You are seeing a patient in clinic that you resected a Colon Cancer from with some small peritoneal metastases that were easily resected at time of surgery. How will you proceed managing this patient?

Referral to Medical Oncology--chemotherapy


Second Look operation in 1 year with CCRS and HIPEC(even if no macroscopic disease found)

You Resect a Colon Cancer and some small peritoneal metastases that are easily resected at time of surgery. What 3 situations put someone at high-risk for recurrent disease requiring a second look in 1 year and possible CCRS and HIPEC?

Ovarian metastases at resection, peritoneal metastases at time of resection, perforation of colon at time of resection

Patient presents with weight loss, abdominal distension, persistent nausea, diarrhea, cachectic appearing, CT shows thickened plaques on inner abdominal wall with some masses interspersed in the abdomen. What's on your ddx? How will you diagnose?

Diffuse Malignant Peritoneal Mesothelioma, desmoid disease, Metastatic disease from elsewhere.


If DMPM, diagnostic lap, get tissue


Diagnosis relies on specific immunohisotchemical markers: Thrombomodulin, calretenin, keratin 5/6, D2-40, podoplanin, mesothelin, Wilms’tumor 1 protein

Patient presents after diagnostic laparoscopy/biopsy shows mesothelioma. How do we further differentiate and stage? Which subtype has best prognosis?

Benign-borderline disease: multi-cystic, well-differentiated papillary


DMPM:Sarcomatoid,Epithelial (best prognosis),Biphasic


Staging is based on Peritoneal Cancer Index with every 10 points upstaging



Patient presents after diagnostic lap/biopsy shows mesothelioma. How do you want to treat? You know it's pathology is Diffuse Malignant Peritoneal Mesothelioma. How does that affect your pre-op planning?

Complete Cytoreductive therapy and HIPEC.


DMPM will likely require more visceral resection

You get called into the Gyn OR when they found peritoneal metastasis from a suspected epithelial ovarian cancer. What are her treatment options?

HIPEC likely warranted to improve survival. RCTs pending

50 y/o male with Biopsy-confirmed gastric adenocarcinoma. Has undergone Neoadjuvant therapy. On entrance into abdomen for resection, find peritoneal metastasis. When is HIPEC indicated for gastric adenocarcinoma?

Indicated for Small mets without diffuse disease in which you can get good cytoreductive surgery. May actually be indicated for T3/T4 cancers without macroscopic evidence of metastasis. Studies show improved survival, not adopted yet in western medicine

35 year-old woman presents with chronic abdominal pain, intermittent obstructive symptoms, and early satiety. CT shows a poorly defined, circular mass, in the mesentery of her distal small bowel. What's on your differential?

Desmoid Tumor


Metastasis

35 year-old woman in diagnostic lap where frozen sectional biopsy shows monoclonal fibroblast proliferation and spindle cells. diagnosis?

Desmoid Tumor

35 year-old woman presents with chronic abdominal pain and early satiety. CT shows a poorly defined, circular mass, in the mesentery of her distal small bowel. Frozen sectional biopsy shows monoclonal fibroblast proliferation and spindle cells. Management?

Attempt complete resection since it's symptomatic.


Plan for possible chemotherapy depending on pathology.


Refer to radiation oncologist if positive margins to go over risks/benefits (some evidence it may help.


Refer to Medical Oncologist: Some reports of Anti-inflammatory drugs, hormonal drugs, Tyrosine Kinase inhibitors, cytotoxic chemotherapy, and interferon having some efficacy

What is the typical mutation pattern in Desmoid Tumors?

Somewhere in the ß-catenin pathway. 85-90% are ß-catenin gene itself, but can be associated with APC (FAP). You see, APC sets up ß-catenin for phosphorylation by GSK3ß and eventual ubiquitination and degradation. Most tumors have an abundance of ß-catenin which is both a cell adhesion molecule promotor of cell proliferation

What do desmoid tumors look like microscopically?

A sea of fibroblasts (in spindle formation) surrounded by collagen matrix. No pseudo capsule. Often radially extending and infiltrating. Stain + for ß-catenin

What are the known predisposing factors for Desmoid tumors?

FAP/gardner syndrome, pregnancy, and trauma/abdominal surgery

When is an FAP patient most likely to develop desmoid tumors?

After surgery.


Most commonly, colon taken out prophylactically


Interestingly, desmoids have a prediliction for prior surgery sites


~50% will develop desmoids in a prior surgery site according to some studies

You get a complete resection of a desmoid tumor in a patient with negative margins. What prognostic factors are most associated with recurrence of desmoid tumors?

Age<37


Tumor >7 cm


peripheral tumor location all have poor prognosis

What is your general strategy for treatment of desmoid tumors?

No true advantage seen with observation vs. surgery vs. medical therapy.


Start with observation. Refer to medical oncology. Treat tumors that are causing morbidity and risking mortality.

What are your surgery goals for desmoid tumors?

Really reserved for functional impairment due to tumors


Difficult to get wide negative margins due to propensity to invade neuromuscular structures


NCCN recommends surgical resection with either positive or negative margins being acceptable in these circumstances

What's the story on radiation therapy for desmoid tumors?

Conflicting reports, no true consensus. Strongly consider not using in young patient who could have life-long radiation induced problems

You find a large, 5 cm desmoid tumor incidentally on a CT confirming diverticulitis. How do you proceed with this finding of the desmoid tumor?

Counsel patient on observation vs. surgery vs. chemotherapy probably having equal efficacy, especially in asymptomatic tumors. R


efer to Medical Oncologist: Some reports of Anti-inflammatory drugs, hormonal drugs, Tyrosine Kinase inhibitors, cytotoxic chemotherapy, and interferon having some efficacy