64: Pancreatic Pseudocysts

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Pancreatic Pseudocysts

RachaelPalumbo andAditiKapil

A 47-year-old male presents to the emergency room with a 3-week history of dull epigastric abdominal pain. She reports a recent history of pancreatitis secondary to alcohol use approximately 1 month ago. She has since recovered and returned to normal activities except for ongoing abdominal fullness, early satiety, and nausea. Denies fevers, weight loss, changes in bowel habits, and skin changes. Since her episode of pancreatitis, she has abstained from alcohol. Her past medical/surgical history includes cholecystectomy and hypertension. Exam is signicant for mildly distended but soft abdomen with mild tenderness in epigastric and left upper quadrant; otherwise, normal. Vitals and labs are within normal limits. How would you manage this patient?

• Given her recent history of pancreatitis and chronic abdominal pain and fullness, peripancreatic uid collection is high on the differential. Other diagnoses to consider include gastric etiologies such as peptic ulcer disease, GERD, malignancy and other general abdominal pathologies such as partial small bowel obstruction.

• To evaluate further, contrast-enhanced CT abdomen/pelvis is recommended (Colvin et al. 2020). While an abdominal ultrasound can be considered, CT scans have a higher sensitivity for diagnosis of pseudocyst and are not operator dependent. Moreover, this will evaluate for other abdominal pathologies in the setting of diagnostic uncertainty.

– MRI and MRCP are also reasonable imaging choices

as they are the most sensitive and accurate diagnostic modalities for pancreatic pseudocyst (Tan etal. 2021). However, in the emergency room setting, the rst diagnostic tool will be CT imaging.

A CT abdomen/pelvis (compared to imaging performed

4weeks prior) was performed which revealed a new 7cm cystic lesion adjacent to the pancreas extending into the ret-

R. Palumbo · A. Kapil (*) Department of Surgery, Yale School of Medicine, New Haven, CT, USA e-mail: aditi.kapil@yale.edu

roperitoneum posterior to the stomach. The lesion is noted to be small walled and symmetric with Hounseld Units <15. How would you manage this patient and discuss his imaging ndings?

• This patient has evidence of a large peripancreatic uid collection, likely having developed after his recent episode of pancreatitis. Given that the uid collection developed from his pancreatitis 4weeks prior, the lesion would be termed a pseudocyst (Aghdassi etal. 2006).

• It is important to explain to the patient that development of a pseudocyst is a common complication after an episode of pancreatitis. The majority of pseudocysts resolve spontaneously within 6weeks.

• At this time, no intervention would be offered to the patient. Rather, symptom management and close follow up in the next 2–4weeks would be recommended. He is at risk of needing intervention due to the symptomatic nature of the lesion and large size (Tan etal. 2021).

The patient follows up with you in the clinic 4weeks after

evaluation in the emergency room. He indicates that his symptoms have marginally improved, and he still complains of signicant early satiety, abdominal fullness, and nausea. He continues to have abstained from alcohol, and he has no other changes to his medical or surgical history. CT imaging was performed the day prior to follow up which showed a persistent 8 cm cystic lesion extending from the pancreas posterior to the stomach into the retroperitoneum. There is no evidence of pancreatic duct compression or venous occlusive disease. How would you manage this patient?

• It is now 8weeks since onset of the index episode of pancreatitis, and the patient continues to complain of a large, symptomatic pancreatic pseudocyst. It would be reasonable to pursue intervention at this time.

• Percutaneous drainage is not recommended due to the high complication risk and overall good surgical candidate.

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R. Palumbo and A. Kapil

• Technique options include endoscopic and surgical intervention. The anatomic location and concurrent complications of previous episodes of pancreatitis should be considered when recommending a treatment technique. For example, simple pseudocysts abutting the stomach may be amenable to an endoscopic cystogastrostomy where interventional GI is available. However, in the setting of complex pathology such as location in the pancreatic head or tail, concurrent main pancreatic duct stricture caused by mass effect of the pseudocyst, failed endoscopic management, multiple lesions, and venous occlusive disease. Outcomes for endoscopic and surgical management are comparable (Tan etal. 2021).

• In this scenario, it would be reasonable to recommend GI evaluation for an endoscopic cystogastrostomy or surgical cystogastrostomy (laparoscopic or open).

The interventional GI clinic is unable to accommodate

the patient for 8weeks due to scheduling conicts. Instead, you decide to surgically drain the lesion. Describe the procedure.

• General steps include:

– Midline laparotomy – Anterior gastrotomy – Large bore needle to aspirate the cyst through the pos-

terior wall of the stomach. An ultrasound may be employed to ensure absence of large vessels prior to performing a posterior gastrotomy

– Posterior gastrotomy with creation of suture marsupi-

alization of the cyst to the posterior gastric wall to create cystogastrostomy

– Closure of anterior gastrotomy

Bonus Points

• A recent episode of pancreatitis may be described by the examiner rather than stating a recent history of pancreatitis. Further workup with cross sectional imaging at the time of presentation should help delineate abdominal pathologies.

• Doing an MRCP prior to undergoing surgery is a reasonable consideration, especially if there is pancreatic ductal compression. This study will further dene the surrounding anatomy.

• Evaluate any available imaging closely prior to performing or recommending an intervention. Other complications of pancreatitis include pseudoaneurysms, necrotic uid collections, venous occlusive disease, etc. It is important to anticipate major vascular collateralization or rupture that may be encountered during a procedure.

Words ofWisdom

Early management of acute peripancreatic uid collections and pseudocysts (<6 weeks) is supportive care. Persistent symptoms, mass effect of the pseudocyst, or large size (>6 cm) are indications for intervention of a pseudocyst, especially after 6weeks from the index episode of pancreatitis. Intervention can be endoscopic or surgical by creating a stula between the uid collection and an enteric organ (i.e., cystogastrostomy and cystojejunostomy), depending on the anatomy of the lesion, existing complications of previous pancreatitis, and institutional resources.

Bibliography

Clean Kills

Intervention prior to 6 weeks since index episode of pancreatitis.

Aghdassi AA, Mayerle J, Kraft M, Sielenkämper AW, Heidecke CD,

Lerch MM.Pancreatic pseudocysts--when and how to treat? HPB (Oxford). 2006;8(6):432–41.

Colvin SD, Smith EN, Morgan DE, Porter KK.Acute pancreatitis: an

update on the revised Atlanta classication. Abdom Radiol (NY). 2020;45(5):1222–31.

Tan JH, Chin W, Shaikh AL, Zheng S.Pancreatic pseudocyst: dilemma

of its recent management (review). Exp Ther Med. 2021;21(2):159.

Part VIII

Abdominal Endocrine

Carcinoid Tumors

AliaAbdulla andCristaE.Horton

Introduction

• Histology – Microscopically composed of sheets of small round

cells called enterochromafn cells (Kulchitsky cells) with uniform nuclei and cytoplasm, containing neurosecretory granules, similar to synaptic vesicles in neurons, producing amines, proteins, and prostaglandins.

– Distinguishing factors between benign versus malig-

nant include range of cellular atypia, mitotic activity, or presence or abscess of necrosis.

• Incidence and distribution – Originate in the foregut, midgut, or hindgut – Most frequent sites of origin are the appendix (38%),

followed by ileum, rectum, and bronchus (23%, 13%, and 11.5%, respectively).

– Carcinoid tumors are associated with increased risk of

synchronous colorectal and small bowel tumors, as well as lung, prostate, and urinary tract neoplasms.

– Colonic carcinoids occur most commonly in the sev-

enth to eighth decade of life and are more common in women than men.

– Gastric carcinoids—three types.

Type I—most common (70–80%), associated with achlorhydria and atrophic gastritis or prolonged PPI use, small and benign, with favorable prognosis. Type II—associated with hypergastrinemia with gastrinomas and Zollinger–Ellison syndrome, with multiple small tumors. Prognosis is good, but there is a higher risk of metastasis. Type III—sporadic lesions without hypergastrinemia or other associated conditions. More aggressive and high rates of metastasis with low 5-year survival of 20%.

A. Abdulla (*) · C. E. Horton Department of Surgery, Broward Health Medical Center, Fort Lauderdale, FL, USA e-mail: chorton@browardhealth.org

• Clinical presentation – Half diagnosed following appendectomy for suspected

appendicitis.

– Colonic carcinoids present as a polyp or mass indistin-

guishable from a carcinoma both grossly and radiographically.

– Most patients are either asymptomatic, have symptoms

from another condition that leads to diagnosis, or have symptoms similar to carcinoma (bleeding, pain, change in bowel habits).

– When in the colon, carcinoids are most commonly

found in the cecum (48%), followed by sigmoid colon (20%), then ascending colon (16%), then descending colon (11%), then transverse colon (6%).

– Rectal carcinoids present either as asymptomatic or

with rectal bleeding or change in bowel habits. Most are found during colonoscopy for cancer screening. Incidence is approximately 0.05%.

– Malignancy with rectal carcinoids are usually >2cm,

with ulceration and rectal bleeding and invasion through the muscularis propria.

– Metastasis occurs more frequently with carcinoids of

the small bowel (50%) and colon, followed by foregut tumors, than with hindgut tumors.

• Carcinoid syndrome – Systemic symptoms produced by carcinoid tumors. – Occur in 10–18% of patients with carcinoids, and 50%

of patients with advanced disease.

– Symptoms include facial ushing (80% of patients),

watery diarrhea (80% of patients), abdominal pain, tearing, salivation, or bronchopulmonary spasm and wheezing.

– Kallikrein secretion is thought to be the cause of facial

ushing, and serotonin (5-HIAA) is likely the cause of watery diarrhea and abdominal cramping.

– Patients may develop right-sided heart failure due to

myobroblasts causing broplasia, increased vascular tone, bronchoconstriction, and platelet aggregation due to serotonin release.

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A. Abdulla and C. E. Horton

– Lungs inactivate these substances, so left-sided heart

failure is not seen.

– Liver metabolizes and inactivates these substances, so

development of carcinoid syndrome only develops after the tumor has already metastasized to the liver, or in the case of a midgut tumor due to their ability to secrete higher serotonin levels. Rectal carcinoids almost never produce carcinoid syndrome due to inability to produce serotonin and foregut tumors lack ability to convert its precursor to serotonin.

• Diagnosis – Colonoscopy – Endoscopy to evaluate for synchronous malignancies. – Endoscopic ultrasonography. – Biochemical testing—24-h urine 5-HIAA assay, nor-

mal range 2–8mg/24h.

– Staging with CT scan of the chest, abdomen, pelvis to

evaluate for distant metastatic disease.

– Somatostatin receptor scintigraphy (SRS) to identify

occult metastases and determine if a patient would respond to octreotide. This is a highly sensitive test in detecting carcinoids except in 10% of the tumors not expressing the somatostatin receptor.

– PET imaging—identify primary tumors and lymph

nodes. Well-differentiated neuroendocrine tumors are usually not PET avid, so a better imaging modality would be 68Ga-DOTATATE PET/CT which is a radiolabeled analog of somatostatin.

• Treatment

Surgical resection

1. Type depends on location of the tumor, likelihood of

residual disease, and lymphatic or metastatic disease.

2. Local or endoscopic resection

3. Full resection of primary tumor (colectomy)

4. Bypass of primary tumor

5. Lymph node resection

6. Debulking – Small bowel

Multicentric lesions in up to one-third of tumors, leading to obstruction, intussusception, brosis of the mesentery. 50% have regional lymph node metastases, and 20–30% of tumors <1cm as well. Size of the tumor is a poor indicator of metastasis.

– Colon

Often larger in size. Resection similar to adenocarcinoma recommended, with similar survival outcomes.

– Rectum

May be amenable to local resection via transanal or endoscopic excision for most tumors <1 cm in diameter. Formal transanal excision includes full thickness of the rectal wall, with rare recurrence even with positive margins.

T2 disease, invasion of the muscularis propria, is associated with lymph node metastases in up to 47% of patients. Size of the tumor is associated with muscular invasion. Size >2 cm—treat with proctectomy with total mesorectal excision. Tumors between 1 and 2cm—treatment is individualized to the patient based on tumor features.

– Liver

Hepatic resection, radiofrequency ablation, cryosurgery, and chemoembolization. Somatostatin analogs (octreotide).

– Gastric

Endoscopic removal for small pedunculated lesions not extending beyond submucosa. Larger >1cm lesions may require wedge resection or partial gastrectomy. Some surgeons perform an antrectomy to remove the source of gastrin secretion, or total gastrectomy with multiple gastric tumors.

Systemic treatment

1. Efcacy is limited; however, various systemic chemotherapy agents have been used.

2. Somatostatin derivatives.

A 61-year-old woman presents to the emergency room with abdominal pain, nausea, and vomiting. She has been having symptoms of diarrhea and facial ushing and tearing for the past 4months. Workup includes a CT scan demonstrating a 3cm hyper-enhancing mass in the terminal ileum with surrounding brosis of the mesentery. She also has a lesion in the right lobe of the liver.

• What are your next steps?

Obtain relevant history to evaluate for other symptoms such as weight loss, changes in bowel habits, blood in the stool, or cardiopulmonary symptoms of ushing. Conduct a physical exam including abdominal exam and thorough lymphatic exam. Order tests including a urinalysis with 24-h 5-HIAA levels. Other studies include CBC, BMP, and chromogranin A (CgA), and tumor markers such as CEA or AFP, and stool cultures and studies. Ensure to replete the patient’s electrolytes and resuscitate with IV uids. If signs of dilated bowel or bowel obstruction secondary to an infectious process or mechanical obstruction then placement of a nasogastric tube and keeping the patient NPO would be warranted. If the patient was short of breath or demonstrating signs of heart failure, other tests would include chest X-ray, EKG, troponins, BNP, echocardiogram, and pulmonary function testing.

Twenty-four-hour 5-HIAA levels returned elevated.

• What is the next step in workup and management of this patient?

65 Carcinoid Tumors

203

This nding would be consistent with a diagnosis of a carcinoid tumor in the terminal ileum that metastasized to the liver, producing carcinoid syndrome. Since carcinoid tumors have a propensity to be multifocal, a thorough search for other tumors via colonoscopy and upper endoscopy is warranted, as they may not be large enough to detect on CT scan. Control of the patient’s symptoms of carcinoid syndrome include starting the patient on octreotide for symptom control while surgical planning is underway. Obtaining full staging workup including a CT of the chest is warranted to evaluate for metastases to the lung. A somatostatin receptor scintigraphy may be benecial to identify occult lesions and determine if the patient’s symptoms will respond to octreotide.

Of note, certain foods such as cheese and wine, and medications such as monoamine oxidase inhibitors and acetaminophen can falsely elevate the urinary 5-HIAA level. CgA can be falsely elevated by the usage of proton pump inhibitors, which ideally should be stopped prior to testing. CgA is >95% sensitive for well-differentiated metastatic neuroendocrine tumors. It can be used for screening and monitoring recurrence.

A 48-year-old woman presents to the clinic after an

uncomplicated laparoscopic appendectomy 2 weeks ago. The nal pathology demonstrated a 2.3cm carcinoid tumor at the appendiceal tip.

• What is the next step in management? The next step in management would be staging workup

including a triple-phase CT of the liver or dedicated contrast- enhanced MRI of the liver to determine further treatment.

Given that this carcinoid tumor is >2cm, further stag-

ing workup is indicated to determine the presence of metastasis or need for neoadjuvant treatment. Then, the surgical treatment plan is with an oncologic right hemicolectomy. Indications for a right hemicolectomy with pathology of a carcinoid of the appendix includes a tumor >2cm located anywhere in the appendix, or between 1 and 2cm with location at the base of the appendix, positive margins or serosal, mesoappendiceal, or lymphovascular invasions, with high mitotic activity. If the lesion had been <2 cm at the tip of the appendix, the laparoscopic appendectomy would have been adequate. A colonoscopy and endoscopy prior to surgical treatment would also be valid, as many neuroendocrine tumors present with synchronous lesions.

A 58-year-old man presents at the ofce complaining of rectal pain with defecation. He has a small mass palpable on the digital rectal exam. On colonoscopy, a 0.6 cm mass is excised endoscopically and sent for pathology. The results demonstrate a well-differentiated rectal neuroendocrine tumor invading the submucosa with negative margins.

• What is the next step in management? Since carcinoid tumors <1cm in size and conned to

the mucosa/submucosa (T1) with negative margins can be treated with endoscopic or transanal excision alone, there is no further surgical intervention indicated for this patient.

• When would a low anterior resection or abdominoperi-

neal resection be necessary for rectal neuroendocrine tumors?

Management of T1 tumors 1–2cm in size is contro-

versial. Treatment should be individualized based on presence of lymphovascular invasion and mitotic rate. Any tumors >2cm or those that invade beyond the muscularis propria (T2) or with lymph node metastasis require a low anterior resection or abdominoperineal resection.