								Brookdale University Hospital & Medical Center

	Sarcomas are rare tumors that represent less than 1% of all newly diagnosed malignancies in the United States annually.

 These tumors may arise from mesenchymal tissue at any body site, including skeletal and extraskeletal connective tissues and the peripheral nervous system. Most of these tumors (approximately 75%) arise in soft tissue and the remainder in the bones. The majority of soft tissue sarcomas are found in the extremities, with only 13% occurring in the retroperitoneum.

 In a population-based series from the Surveillance, Epidemiology, and End Results (SEER) database, the average annual incidence of retroperitoneal sarcomas was 2.7 cases per million population.

 The majority of retroperitoneal tumors are liposarcomas and leiomyosarcomas. Most patients present with an asymptomatic abdominal mass, but symptoms may include early satiety, obstruction, and bleeding from pressure effects of the mass on neurovascular components.

	We report the case of a patient who presented with diffuse abdominal pain and right-sided distention that had been increasing for approximately 8 weeks. Radiographic studies showed a large, heterogeneous, right-sided abdominal mass that extended across the midline and displaced various organs into the left abdomen. Surgical excision yielded a 20.67-lb, high-grade, dedifferentiated retroperitoneal liposarcoma.

 A 53-year-old man with a 30-year undocumented medical history presented to the emergency department with abdominal pain and right-sided distention that had been increasing for approximately 8 weeks. The patient reported a dragging sensation in the right side of the upper abdomen and occasional epigastric pain, which he rated as a 7 out of 10 on a pain intensity scale. The pain was alleviated slightly by laying on his left side. He also reported early satiety and more frequent urination and bowel movements, with the need to empty his bowels forcefully. He also experienced increasing episodes of insomnia, with frequent awakenings throughout the night, and a weight loss of 10 lb over the previous year. He reported no associated chest pain, shortness of breath, fever, nausea, vomiting, diarrhea, or bloody stools. The patient had no surgical history.

	Upon physical examination, the upper and lower quadrants of the right side of the abdomen were found to be significantly distended. Bowel sounds with dullness to percussion were auscultated over the entire affected region. There was also notable epigastric tenderness and questionable hepatomegaly on palpation. No lower extremity edema or other positive physical findings were identified.

	Upon admission to the hospital, laboratory evaluations yielded the following results: hemoglobin, 8.8 g/dL (normal, 12.5—18 g/dL); white blood cell count, 8.7 x10

/L); sodium, 130 mEq/L (normal, 136—145 mEq/L); chloride, 92 mEq/L (normal, 101&mdash;111 mEq/L); glucose, 454 mg/dL (normal, 70&mdash;110 mg/dL); and hemoglobin A

, 14.1% (normal, < 6%). Based on these results, the initial working diagnosis included an (1) unidentifiable potentially malignant abdominal mass; (2) anemia that might result from an underlying malignancy; and (3) newly discovered diabetes mellitus.

	To identify the tumor better, a series of radiographic studies was undertaken. Abdominal ultrasonography showed an irregularly marginated mass within the right mid-abdomen, which caused obstruction of the right urinary collecting system, and hydronephrosis on the ipsilateral side. Abdominal radiographs showed no evidence of bowel obstruction, ileus, or free air in the abdomen. Stool was observed in the descending colon, but the tumor appeared only as a hazy density within the abdomen. Computed tomography (CT) scans of the chest, abdomen, and pelvis showed a large, heterogeneous,?right-sided abdominal mass that extended across the midline into the left abdomen (Figure 1). The mass was approximately 31.5 cm long, and its greatest axial dimension measured 23.5 x 17 cm. There was a deformity of the right renal contour at the mid- and lower poles, and the mass arose from?within the right kidney. There was mild-to-moderate hydronephrosis of the right kidney and likely obstruction of the ureter. The mass displaced the ascending and transverse colon anteriorly and pushed most of the bowel into the left abdomen, but there was no evidence of bowel obstruction. The right renal artery and vein were found to be patent; however, the superior mesenteric vein was displaced into the left upper quadrant, and the inferior vena cava was compressed against the aorta. There was no evidence of liver or lung metastases. Magnetic resonance imaging (MRI) of the abdomen and pelvis was suggestive of sarcoma (Figure 2).

	Exploratory laparotomy, right nephrectomy, and resection of the tumor and mesenteric lymph nodes were undertaken (Figure 3). Upon surgical excision, the mass was found to be a 9,395-g (20.67 lb) retroperitoneal tumor. It was circumscribed, well-encapsulated, and encased the kidney without directly infiltrating the parenchyma. Microscopic examination showed predominantly vascular myxoid stroma scattered with pleomorphic neoplastic cells and multinucleated giant cells with areas of increased cellularity, confirming a neoplasm (Figure 4). There were frequent atypical mitotic figures and extensive tumor necrosis. A diagnosis of high-grade dedifferentiated retroperitoneal liposarcoma was made.

	Postoperative radiation therapy was not undertaken due to the large affected surface area (tumor bed) and the relative intolerance of normal surrounding tissue to radiation. Radiographic studies at the patient’s 3-month follow-up did not show any previous or newly developed metastases.

 Soft tissue sarcomas are almost always thought to arise de novo and not from pre-existing benign lesions. They are classified according to their presumptive tissue of origin. Although most cases have no clearly defined etiology, an array of predisposing risk factors has been described, including genetic predisposition; chronic irritation; and previous exposure to radiation, chemotherapy, or chemical carcinogens. In addition to the potential for local growth and destruction, the risks of recurrence and distant metastasis are substantial. Liposarcomas pose many challenges with respect to early detection, clinical behavior, and treatment.

 Currently, liposarcomas are generally classified into four types based on their morphologic features and cytogenic aberrations; these are (1) well-differentiated, (2) dedifferentiated, (3) myxoid/round cell, and (4) pleomorphic.

 The extent of differentiation affects the clinical course of the disease and the patient’s prognosis after resection. Using the American Joint Committee on Cancer (AJCC) staging system, our patient had a stage III, malignant, soft tissue sarcoma, which was histologically classified as a dedifferentiated liposarcoma.

 To determine staging, the AJCC system considers histologic grade, tumor size, tumor depth, and the presence or absence of lymph node and distant metastasis.

 Stage III disease is characterized by the presence of a poorly differentiated primary tumor larger than 5 cm in its greatest diameter and no distant or histologically verified metastasis to regional lymph nodes. In stage IV disease, there is regional lymph node involvement or evidence of distant metastatic disease, regardless of the tumor’s histologic grade, size, or site.

	Dedifferentiated liposarcoma refers to the development of a high-grade nonlipogenic sarcoma juxtaposed with a well-differentiated liposarcoma.

 Dedifferentiated liposarcomas generally have a worse prognosis than other sarcoma variants, with an overall 5-year survival rate of approximately 20%.

 There is local recurrence in 40% to 83% of cases and distant metastasis in 15% to 30% of cases.

 When liposarcoma is suspected, a thorough history and physical examination should be undertaken to determine which major vessels, organs, nerves, or bones may be affected, and to assess regional lymph node status. CT scanning of the abdomen and pelvis are useful in evaluating the primary site and offer clues regarding intraperitoneal seeding, regional lymph node involvement, and whether there is distant metastasis to the liver. CT scanning of the chest should also be undertaken to check for possible metastasis to the lungs. While CT scanning cannot indicate the histologic grade of the tumor, it can show features that may be suggestive of a higher grade (such as necrosis), thereby offering some insights. The increased use of CT scanning has allowed liposarcomas to be detected earlier in both symptomatic and asymptomatic patients; however, despite the increased use of this modality, most retroperitoneal liposarcomas are detected as locally advanced tumors.

	The morphologic variability of liposarcomas may lead to nonspecific imaging features that reflect the coexistence of a nonlipomatous mass and predominantly fatty tumor. Although MRI is sensitive enough to detect minute fat deposits or immature fatty components, liposarcomas may contain various amounts of myxoid matrix composed of hyaluronic acid, which often has a similar signal intensity to that of fatty tissue, confounding the diagnosis.

	The collaborative use of CT scanning and MRI have revealed well-defined, nonlipomatous masses associated with fatty tumor, where the transition between the two components has been found to be characteristically abrupt in most cases.

Another characteristic imaging pattern of dedifferentiated liposarcoma is calcification or ossification, which may be suggestive of metaplasia within the tumor. The effect of metaplasia on prognosis is not fully understood.

 Complete surgical resection of the primary lesion at initial presentation is critical to achieving a cure or long-term survival.

 The histologic grade of the liposarcoma is more indicative of prognosis than histologic type. Traditionally, patients with retroperitoneal liposarcomas have been more likely to die from local recurrence than from distant metastasis.

 Because the risk of local recurrence is attributed to surgical margins, complete excision is imperative to avoid this complication. Complete resection of these tumors is challenging, because these lesions are usually large (> 10 cm) when diagnosed and the lack of distinct anatomic compartments within the retroperitoneum allows these tumors to extend and involve many adjacent organs and structures. Retroperitoneal liposarcomas also have a high degree of adipocyte differentiation, which makes it difficult to distinguish tumor from retroperitoneal fat; thus, the determination of negative microscopic margins is a difficult and imprecise task.

	The role of radiation therapy for retroperitoneal liposarcomas is unclear, because there is a paucity of data substantiating favorable impact with this therapy on recurrence rates or on overall patient survival.

 A randomized trial evaluating radiation therapy in the management of retroperitoneal sarcomas compared intraoperative and low-dose postoperative radiation with high-dose postoperative radiation.

 Patients in the intraoperative radiation therapy arm had significantly better control of local disease but showed no improvement in overall survival. Despite the lack of controlled trials confirming the efficacy of radiation therapy for retroperitoneal sarcomas, it has been hypothesized that adjuvant radiation still may play a role in controlling local disease, potentially enhancing long-term survival.

	An important consideration of postoperative radiation therapy is the relative intolerance of normal surrounding tissues to this therapy, including the loops of bowel, which may become fixed due to postoperative adhesions. Preoperative radiation may be a safer alternative since it allows for more accurate targeting of the volume around the tumor. Because the normal surrounding tissues and bowel are displaced by the tumor, a higher dose of radiation may be used directly at the site. A randomized trial that evaluated intraoperative radiotherapy as an adjuvant treatment showed improvement in local control but failed to show a significant increase in patient survival.

 The optimal treatment regimen for local tumor control may be a combination of surgical resection and radiation therapy, but further trials are necessary to determine this definitively. While the best outcomes are observed in patients with negative surgical margins, and the importance of obtaining clear surgical margins is accepted, the highly aggressive nature of sarcomas and their propensity for local recurrence and distant metastasis create a fundamental need for enhanced adjuvant therapy.

 The histopathologic spectrum of sarcomas is extensive, presumably because they are composed of embryonic mesenchymal cells, which have the ability to differentiate. We reported a case of a dedifferentiated liposarcoma, which has a significantly worse prognosis than other variants of sarcoma. Adequate surgical resection with negative microscopic margins remains the highest priority for long-term survival. Neoadjuvant radiation and chemotherapy have been used for limb salvage in unresectable extremity sarcomas, but the role of these therapies for retroperitoneal liposarcomas requires further evaluation. The highly aggressive nature of these tumors supports the need for more effective adjuvant therapies.

 1. Jemal A, Siegel R, Ward E, et al. Cancer statistics, 2006. 

	2. Lawrence W Jr, Donegan WL, Natarajan N, et al. Adult soft tissue sarcomas. A pattern of care survey of the American College of Surgeons. 

	3. Porter GA, Baxter NN, Pisters PW. Retroperitoneal sarcoma: a population-based analysis of epidemiology, surgery, and radiotherapy. 

	4. Fletcher CD, Akerman M, Dal Cin P, et al. Correlation between clinicopathological features and karyotype in lipomatous tumors. A report of 178 cases from the Chromosomes and Morphology (CHAMP) Collaborative Study Group. 

Pathology and Genetics of Tumors of Soft Tissue and Bone.

	5. Fletcher CD, Unni KK, Mertens F, eds. 

American Joint Committee on Cancer (AJCC) Cancer Staging Manual

	6. Greene FL, Page DL, Fleming ID, eds. 

. 6th ed. New York, NY: Springer-Verlag NY; 2002:193.

	7. Nascimento AG. Dedifferentiated liposarcoma. 

	8. Singer S, Antonescu CR, Riedel E, et al. Histologic subtype and margin of resection predict pattern of recurrence and survival for retroperitoneal liposarcoma. 

	9. Tateishi U, Hasegawa T, Beppu Y, et al. Primary dedifferentiated liposarcoma of the retroperitoneum. Prognostic significance of computed tomography and magnetic resonance imaging features. 

	10. Hassan I, Park SZ, Donohue JH, et al. Operative management of primary retroperitoneal sarcomas: a reappraisal of an institutional experience. 

	11. Lewis JJ, Leung D, Woodruff JM, et al. Retroperitoneal soft-tissue sarcoma: analysis of 500 patients treated and followed at a single institution. 

	12. Sindelar WF, Kinsella TJ, Chen PW, et al. Intraoperative radiotherapy in retroperitoneal sarcomas. Final results of a prospective, randomized, clinical trial. Arch Surg. 1993;128(4):402-410.

Articles

Surgical Rounds

Vahe S. Tateosian, Medical Student IV, Department of Surgery, Flushing Hospital Medical Center, Flushing, NY; Lourdes Castanon, Senior Surgical Resident, Department of Surgery, Brookdale University Hospital & Medical Center, Brooklyn, NY; Charles C. Conte, Surgical Oncology Chairman, Department of Surgery, Flushing Hospital Medical Center, Flushing, NY

Giant dedifferentiated liposarcoma of the retroperitoneum

	As a former NYH-Cornell student, intern, and resident, I wanted to point out that in the Libby Zion case, the house staff took the fall. Mr. Zion, a prominent and wealthy New Yorker who wrote for the New York Times, had a society internist for his family and for Libby. She came to the emergancy department in the middle of the night with a probable drug overdose, and the house staff could not get the internist to come in and see her; he stayed in bed. The house staff was relatively green, and when Libby was oversedated and died, they took the rap while the attending walked away scot-free. It was the internist who was sleepy, not the intern and first-year resident. That this became a mandated law for residency is ironic and sad, because to the adult attending responsible goes all the blame for being lazy, uncaring, and, frankly, negligent.

	It may be that I am old enough to remember and long for different times in medicine, but as I read your editorials month-to-month, I find myself saying, “Right on,” with each issue. Over the years, I have been associated with a residency program. I have found a direct correlation between the new time restriction rules and the lack of patient commitment by the residents. This, of course, has been just one of many steps that over the years has and will drive a wedge between physicians and patients. Without “commitment,” we really do become an assembly of mercenaries that is looking for an easy way to make a living.

Editorial: Mired in detail 

								University of California San Francisco

								University of Connecticut Health Center

								The Ludwig J. Pyrtek, MD, Chair in Surgery

								Director of Surgery and Chief, Division of General Surgery

								University of Connecticut School of Medicine

					Laparoscopic marsupialization of a splenic cyst is a rewarding procedure, because it alleviates symptoms of pain, eliminates or minimizes many operative complications (such as bleeding or rupture), decreases morbidity, allows shorter hospital stays, and preserves splenic tissue. The authors provide a good review of the various classification systems used to categorize splenic cysts. Of these systems, I prefer Morgenstern’s classification. The authors also provide key technical details regarding laparoscopic marsupialization of a giant splenic cyst. Gradual aspiration of the cyst is important. I prefer to remove the free wall of the cyst with an ultrasonic dissector, as described by the authors.

	Splenic cysts are rare lesions that can be classified as primary (“true”) cysts or secondary (“false”) pseudocysts based on the presence or absence of an epithelial lining.

 The etiology of primary cysts can be parasitic or nonparasitic (congenital). Worldwide, 

 infestation accounts for over 50% of all splenic cysts.

 In North America, secondary splenic cysts compose the majority of splenic cysts (75%) and are usually a late complication of previous abdominal trauma.

	Many treatment modalities have been described for nonparasitic splenic cysts to prevent or treat the rare but life-threatening complications of rupture, hemorrhage, and infection.

 Recently, minimally invasive procedures have been employed to preserve splenic parenchyma and decrease morbidity. We report a case of a giant nonparasitic splenic cyst that was managed successfully using laparoscopic marsupialization. We also discuss the etiology, classification, diagnosis, and management options for this uncommon disorder of the spleen.

	A 27-year-old African American man came to our hospital because of mild abdominal discomfort and progressive abdominal fullness, which had started 3 weeks earlier. Approximately 3 years before his current presentation, he had experienced similar symptoms, and a large splenic cyst was identified and percutaneously drained. He had no other significant medical or surgical history and, despite being an avid soccer player, he reported no history of abdominal trauma. On physical examination, a large abdominal mass was easily palpable in the left upper quadrant, from which no tenderness was elicited.

	An abdominal computed tomography (CT) scan confirmed a recurrent giant splenic cyst (Figure 1A). It was unilocular and measured 20 cm at its largest diameter. The remaining splenic parenchyma was displaced, forming the “beak sign.” The left kidney was shifted inferior and toward the midline (Figure 1B). Serologic testing for 

 were negative. Surgery was recommended because of the patient’s symptoms and the size and recurrent nature of the cyst. Two weeks before the operation, the patient received pneumococcal, 

	The patient was placed supine on the operating room table. After induction of general anesthesia, a roll was put under the left abdomen for anterior displacement of the spleen, and a Foley catheter was placed. Pneumoperitoneum was established after placing a 12-mm Hasson trocar in the supraumbilical position. A 30-degree, 10-mm laparoscope was inserted, and the abdomen was explored. A giant splenic cyst occupied the left upper quadrant and was moderately scarred to the anterior parietal peritoneum, presumably from the previous percutaneous drainage. A loop of small intestine was adhesed to the inferior border of the cyst, and the transverse colon was identified inferior to the mass.

	A second trocar (10 mm) was placed in the left lower quadrant in the anterior axillary line, and a third trocar (5 mm) was placed 7 cm below the xiphoid process in the midline. The adhesions attaching the anterior portion of the cyst to the anterior parietal peritoneum were lysed using an ultrasonic dissector. The cyst was then decompressed by puncturing the anterior cyst wall with the dissector and removing the fluid in a controlled manner using a suction device. Over 3 L of dark brown fluid was drained from the cyst. Cytologic examination of the cyst fluid showed no malignant cells and no ova or parasites.

	The cyst was widely unroofed by removing the anterior portion of the cyst wall with the ultrasonic dissector (Figure 2). Caution was taken to avoid the area of the cyst that was adherent to the loop of small intestine. The diameter of the cyst wall varied from a few millimeters to over 1 cm, and bleeding from the cyst wall was well controlled with the ultrasonic dissector. The excised cyst wall was then placed into a specimen bag and removed through the supraumbilical wound. The entire surgical procedure was completed in 1 hour and 10 minutes, and blood loss was minimal.

	Final pathologic analysis of the cyst wall was consistent with a benign pseudocyst. The cyst wall did not have an epithelial lining and showed evidence of old hemorrhage (Figure 3). The cyst fluid specimen contained red blood cells, rare mesothelial elements, and cholesterol-like clefts consistent with hemorrhage or tissue infarction. The patient had an unremarkable postoperative course and was discharged home on postoperative day 2. He was back to his baseline activity level after 2 weeks. At 6-month follow-up, there were no signs of recurrence.

	There are two standard classification systems for splenic cysts, which are based predominantly on the characteristics of the cyst lining. Fowler described primary and secondary splenic cysts in a comprehensive review, while Martin provided a simplified clinical classification (Table).

 In 2002, Morgenstern questioned the validity of the two standard classification systems and provided a third method for categorizing nonparasitic splenic cysts (Table).

 His classification system focused on specific features that define the cyst as congenital, neoplastic, traumatic, or degenerative, rather than the presence or absence of an epithelial lining. Morgenstern postulated that most nonparasitic splenic cysts are congenital in origin and the history of antecedent trauma usually is incidental.

	Most patients with splenic cysts experience minor, nonspecific symptoms related to the mass effect of the cyst.

 The diagnosis is made by taking a thorough patient history, conducting a physical examination, and evaluating ultrasonography and CT scan findings. Rupture, hemorrhage, and infection, which may be life-threatening, have been reported.

 Splenic cysts may become very large and are considered giant when they grow larger than 15 cm.

	Few surgeons in North America ever encounter nonparasitic splenic cysts, and the overwhelming majority of such cysts are thought to form as a result of blunt abdominal trauma. Many management strategies have been described. The primary goal of treatment is to resolve symptoms and prevent complications. The secondary goal is to preserve splenic parenchyma.

	Nonoperative measures, such as observation, have been recommended for asymptomatic cysts smaller than 5 cm.

 The natural history of these small cysts is largely unknown, but if the imaging characteristics reveal regularity of the cyst wall, absence of a solid component, and a typical round shape, there is no indication for cyst removal. Spontaneous resolution of traumatic pseudocysts can occur. Surgical treatment usually is recommended for symptomatic patients or for those with cysts larger than 5 cm.

	Percutaneous aspiration of the cyst has been described as a definitive treatment, but this option often leads to recurrence.

 Chemical agents, such as alcohol or tetracycline, have been percutaneously injected into the cyst cavity after aspiration in an attempt to collapse the cyst wall, promote fibrosis, and prevent reaccumulation; however, a case by Moir and colleagues showed that despite repeated attempts in the same patient, fluid reaccumulated and the cyst recurred.

 Wu and Kortbeek reported on four patients who underwent percutaneous drainage.

 Of these patients, three had a cyst recurrence and one died after accidental puncture of the colon. Therefore, percutaneous aspiration with or without sclerosis should not be considered a definitive treatment and may complicate subsequent surgical management.

	Total splenectomy used to be the treatment of choice for nonparasitic splenic cysts. In 1867, the French surgeon Jules Pean performed a total splenectomy for a splenic cyst that he initially attempted to excise.

 However, due to the increasing awareness of the immunologic function of the spleen, organ-preserving techniques were developed to avoid the rare but life-threatening risk of overwhelming postsplenectomy sepsis. These salvage procedures ranged from cyst excision with partial splenectomy to cyst marsupialization with partial cyst wall excision.

 Splenic cyst marsupialization is a simpler procedure with less risk of major blood loss; however, this technique leaves a portion of the cyst wall contiguous with the preserved splenic parenchyma and there is a theoretical risk of recurrence. Wu and Kortbeek performed fenestration procedures in two patients, and both patients experienced recurrences.

 In contrast, Touloukian and colleagues performed six partial decapsulations of splenic cysts over a 10-year period without any recurrences.

	With the advent of advanced laparoscopy, previously standard open operations for the treatment of nonparasitic splenic cysts have been undertaken using a minimally invasive approach. This has resulted in effective treatment with less morbidity and postoperative pain, shorter hospital stays, better cosmesis, and a faster return to normal activities.

	In 1985, Salky and associates reported the first laparoscopic treatment of a splenic cyst by creating a “cyst-peritoneal window.”

 The cyst was decompressed with an aspiration needle, and a 5-mm scissors instrument with cautery was used to make a 3-cm window in the cyst wall. Over the past 15 years, a variety of laparoscopic techniques have been successful and have resulted in minimal risk of recurrence; these include laparoscopic total splenectomy, laparoscopic partial splenectomy, and laparoscopic marsupialization.

 Despite such successes, it is important to note that laparoscopic splenectomy still incurs a risk of postsplenectomy sepsis and that laparoscopic partial splenectomy is a technically challenging operation with a longer operative time and the potential for greater blood loss.

	Many reports of laparoscopic marsupialization of nonparasitic splenic cysts have been reported. The most common technique includes removing a large portion of the cyst wall without dividing splenic parenchyma. A variety of instruments can be used to unroof the cyst, such as scissors with electrocautery, staplers, and other bipolar devices. Our instrument of choice is the ultrasonic dissector. Omentum can be used to pack the cyst cavity. Unless the cyst is infected, a closed drain is unnecessary if the cyst is unroofed adequately.

	Despite our patient having no history of splenic injury, the pathology suggests that the etiology of this secondary cyst was previous trauma. Laparoscopic cyst marsupialization proved to be a safe and effective treatment for our patient and provided the benefits of a minimally invasive approach; however, longer follow-up is needed to assess the long-term success of this procedure.

	A wide range of treatment modalities have been described for symptomatic or very large nonparasitic cysts of the spleen. Laparoscopic marsupialization is a safe and effective management strategy that has the advantages of a minimally invasive approach, including minimal blood loss, reduced postoperative pain, shorter hospitalization, improved cosmesis, and quicker return to normal activities. It also eliminates the risk of overwhelming postsplenectomy sepsis.

	1. Martin JW. Congenital splenic cysts. 

	2. Narasimharao KL, Venkateswarlu K, Mitra SK, et al. Hydatid disease of spleen treated by cyst enucleation and splenic salvage. 

	3. Pachter HL, Hofstetter SR, Elkowitz A, et al. Traumatic cysts of the spleen—the role of cystectomy and splenic preservation: experience with seven consecutive patients. 

	4. Moir C, Guttman F, Jequier S, et al. Splenic cysts: aspiration, sclerosis, or resection. 

	5. Fowler RH. Nonparasitic benign cystic tumors of the spleen. 

	6. Morgenstern L. Nonparasitic splenic cysts: pathogenesis, classification, and treatment. 

	7. Qureshi MA, Hafner CD. Clinical manifestations of splenic cysts: study of 75 cases. 

	8. Goldfinger M, Cohen MM, Steinhardt MI, et al. Sonography and percutaneous aspiration of splenic epidermoid cyst. 

	9. Wu HM, Kortbeek JB. Management of splenic pseudocysts following trauma: a retrospective case series. 

	10. Pean MJ. Operation de splenectomie. 

	11. Uranus S, Kronberger L, Kraft-Kine J. Partial splenic resection using the TA-stapler. 

	12. Brown MF, Ross AJ 3rd, Bishop HC, et al. Partial splenectomy: the preferred alternative for the treatment of splenic cysts. 

	13. Millar JS. Partial excision and drainage of post-traumatic splenic cysts. 

Mark Takata, Fellow in Laparoscopic Surgery, Department of Surgery, University of California San Francisco,            San Francisco, CA; Christine Rader, Resident, Department of Surgery, University of Connecticut Health Center, Farmington, CT; Orlando Kirton, The Ludwig J. Pyrtek, MD, Chair in Surgery, Director of Surgery and Chief, Division of General 

Laparoscopic marsupialization of a giant nonparasitic splenic cyst

	These questions are similar to those asked on the American Board of Surgery Recertification Examination. You can use them to prepare for the examination, fulfill learning requirements for various maintenance of competence programs, or simply improve your surgical knowledge.

 Are best diagnosed by computed tomography (CT) scanning

 Cause pain in the lateral thigh due to nerve compression

 Occur at the junction of the pubic and iliac bones

2. Which of the following conditions does not cause elevation of the basal serum gastrin level? 

3. In the treatment of Grave’s disease, thyroidectomy is contraindicated 

 If the patient has not been rendered euthyroid

 In patients with an inadequate re&shy;sponse to radioactive iodine ablation

4. The small bowel tumor with the greatest propensity for bleeding is 

6. Clinical indications for intubation after a severe burn include all of the following, 

 Have an abnormality of procollagen type III

 Have abnormal bleeding despite normal coagulation studies

8. The most common anomaly of the biliary ductal system is 

 A separate right anterior hepatic duct, which enters the common hepatic duct

 A separate right posterior hepatic duct, which enters the common hepatic duct

 Cystic duct crossing the common bile duct and entering on the left side

9. In surgical patients, adrenal insufficiency 

 Presents with unexplained perioperative hypotension

 Can be successfully treated with prednisone alone

 Develops when the inferior mesenteric artery becomes occluded

 Should be treated by revascularization procedures

11. Appropriate modalities to diagnose renovascular hypertension include all of the following, 

 Magnetic resonance imaging (MRI) with gadolinium enhancement

 Spiral computed tomography (CT) scanning

12. The most common cause of death in a patient in whom traumatic lateral compression (T-boning) caused a pelvic fracture is 

 The same as after anterior-posterior compression (head-on) injury

American Board of Surgery Recertification Examination

								Emory University School of Medicine

Adrenal pseudocysts are rare cystic masses that arise within the adrenal gland and are usually asymptomatic. They consist of a fibrous wall devoid of epithelial or endothelial lining. Adrenal 

pseudocysts generally result from hemorrhage within a normal adrenal gland. The authors report the case of a 30-cm, left-sided cystic suprarenal mass in a patient who presented with anorexia, hypertension, increased abdominal girth, worsening back pain, and shortness of breath over a 5-year period. They also review the literature and discuss the etiology, diagnosis, and management of these rare lesions. 

	Adrenal pseudocysts are rare lesions, especially those larger than 10 cm. They are usually nonfunctioning and asymptomatic.

 The first report of an adrenal pseudocyst was in a postmortem description provided by Greiselius in 1670.

 He described the mass as large enough to fill the left side of the abdomen, displacing the diaphragm and spleen superiorly and extending inferiorly to the psoas muscle. Autopsy revealed the suprarenal mass to have a "rupture big enough to admit the fist," and the peritoneal cavity was found to contain 12 lb of serosanguineous fluid and 2 lb of purulent clot. Greiselius concluded that the patient's death was due to traumatic rupture of the pseudocyst. Since his account 337 years ago, approximately 300 cases of adrenal cysts have been reported in the literature, of which fewer than 100 were pseudocysts.

	The frequency of adrenal pseudocyst has been estimated to be between 0.35% and 4.4% on computed tomography (CT) scanning and 1.4% to 5.7% on autopsy series.

 Although the incidental discovery of suprarenal masses has increased with the advent of radiological technology, the etiology of these lesions is still unclear. Three mechanisms have been proposed to account for the occurrence of adrenal pseudocysts. These include cystic degeneration of a primary adrenal neoplasm, degeneration of a vascular neoplasm, and malformation and hemorrhage of adrenal veins into the adrenal gland. There have been no reports in the literature of an adrenal pseudocyst as large as the one we describe, which measured 30 cm. The largest previously documented pseudocyst measured 26 x 16 x 14 cm.

	A 54-year-old, obese, African American woman presented to the surgical oncology service because of a large, palpable left upper quadrant abdominal mass. The patient gave a history of increasing abdominal girth and worsening shortness of breath for the past 5 years. She also reported pitting edema, a weight loss of 33 lb, nausea, and early satiety, but no abdominal pain. Her medical history was significant for poorly controlled hypertension, chronic back pain, arthritis, palpitations, and anxiety disorder. She had undergone diagnostic fine needle aspiration of the mass approximately 8 months earlier at another hospital. Pathology examination at that time revealed bloody proteinaceous fluid with hypocellularity and no malignancy.

	On physical examination, the patient had normal vital signs, with a blood pressure of 115/64 mm Hg. Her abdomen was soft and distended with an obvious left upper quadrant mass, and no tenderness was elicited on palpation. Laboratory evaluations revealed a hemoglobin of 8.7 g/dL (normal, 11.5?15.5 g/dL), white blood cell count of 12,000/?L (normal, 4,000?8,500/?L), and amylase of 71 U/L (normal, 25?85 U/L). Serum chemistries and liver function tests were within normal limits.

	Abdominal ultrasonography revealed multiple hypoechoic areas within the mass. Abdominal and pelvic CT scans showed a 30 x 25 x 15-cm cystic mass with a few septations and mild calcium deposition (Figure 1). The mass appeared to arise from the retroperitoneum, displacing the left kidney across the midline, the pancreas posteriorly, the spleen laterally, and the stomach anteriorly. The adrenal gland also appeared to be displaced. The liver contained a 7-cm lesion, which subsequently was determined to be a hemangioma on tagged red blood cell scan. The right ovary was enlarged, measuring 7.5 cm, and heterogeneous.

	Celiotomy was performed via a midline incision, which immediately revealed a large, smooth, opaque cystic mass arising from the retroperitoneum. The mass was bluntly dissected and excised in its entirety from the surrounding tissues and from the closely opposing left kidney (Figure 2). The enlarged, multicystic right ovary also was removed. Pathologic examination revealed an encapsulated cystic mass with the adrenal gland embedded in its wall (Figure 3). It contained areas of secondary hemorrhage, microcalcifications, and 6 L of hemorrhagic fluid. There was no epithelial lining. The patient had an uneventful recovery and was discharged home on postoperative day 5.

 Adrenal cysts are rare, with only 300 cases reported in the literature as of 2003. Adrenal pseudocysts are cystic lesions thought to arise within the cortex or medulla. They have a fibrous wall, and no recognizable epithelial or endothelial lining encloses these cystic structures. Adrenal pseudocysts are one of the more common non-neoplastic cysts of adrenal origin, accounting for 32% of all adrenal cysts.

 They have been noted to occur from birth to 80 years of age, with a peak incidence in the third to sixth decades of life; our patient falls in this peak age range. Adrenal cysts occur more frequently in women, with a 2:1 female-to-male ratio.

 Approximately 8% to 10% of these lesions are bilateral.

 Although most adrenal pseudocysts are asymptomatic, size may play a significant role in symptomology. the most common symptom is abdominal pain, which may or may not radiate.

 The five pathologic entities of adrenal cysts that have been described are parasitic, epithelial, endothelial, neoplasm, and pseudocyst.

—Seven percent of adrenal cysts are attributed to infection or parasitic infestation, of which 

 is the most common cause. Diagnosis is made when a hydatid cyst or scolices are identified along with leukocytosis, eosinophilia, or both.

 The origin and nature of the cyst should be examined carefully, and excision is the treatment of choice. Complete and prompt removal of the gland is imperative. Complications may include exophytic growth, perforation into hollow viscera, peritoneal seeding, abdominal wall invasion, and possible hematogenous dissemination.

—Nine percent of adrenal cysts are characterized by a columnar epithelial lining, with or without cilia. Most of these cysts are thought to originate from remnants of the embryonic urogenital sinus. These cysts are normally small, thin-walled, and sometimes multifocal.

 Surgical treatment is not recommended for cysts smaller than 5 cm, and it is acceptable to monitor patients with such cysts closely.

—Endothelial cysts are the most common variety and account for approximately 45% of all adrenal cysts. The presence of dilated vascular channels in the surrounding fibrous wall and in the adjacent adrenal tissue suggests that these cysts arise as a result of lymphangiectasia or abnormal development of vascular channels.

 Two types exist: lymphatic and vascular. Lymphatic cysts are more common and usually contain proteinaceous lymphatic fluid. Vascular cysts arise from blood vessels, contain blood, and are usually multiloculated.

	Adrenal cysts smaller than 5 cm may be monitored closely, whereas surgical intervention may be necessary when a suspected benign cyst is larger than 5 cm; the diagnosis is uncertain; compression of surrounding structures is evident; or the cyst is functional. Surgical intervention may include simple enucleation, marsupialization, percutaneous aspiration and drainage, or adrenalectomy.

—Malignant neoplasms account for 7% of all adrenal cysts and are usually caused by hemorrhage or necrosis within a preexisting tumor. Neoplasms may be cystic and are metastatic tumors in 95% of cases, pheochromocytomas in 3%, and adrenocortical carcinomas in 2%. The walls of these cystic structures tend to be thickened, fibrous, and irregular.

 Treatment involves margin-free tumor resection, and local recurrence after resection is uncommon.

—Adrenal pseudocysts account for 32% of all adrenal cysts.

 In neonates, adrenal cysts are rare and are thought to be hemorrhagic pseudocysts if they resolve spontaneously.

 Other predisposing factors for adrenal pseudocysts include trauma, infection, anoxia in infants, hemorrhagic diathesis, vascular aneurysm, or embolism with subsequent infarction.

 The etiology of our patient’s pseudocyst was unclear.

 The most valuable technique for examining the adrenal gland is CT scanning, which can identify tumors as small as 1 cm with 100% sensitivity.

 Calcifications may be seen in adrenal cysts. In addition, the lack of enhancement of solid parts of a mass may help in the differential diagnosis of pseudocyst versus adrenal neoplasm.

 Other modalities that have been used to evaluate cysts include ultrasonography and magnetic resonance imaging (MRI). Abdominal ultrasonography can detect adrenal masses larger than 2 cm in diameter and are inexpensive and low risk; however, they cannot accurately define the size of an adrenal mass or its morphologic characteristics. In contrast, MRI provides anatomical details and allows tissue characterization.

 Evaluation by CT scanning or MRI is integral in determining whether an adrenal mass is malignant or a benign cyst. Features on CT scanning that suggest malignancy include a thick, well-enhanced border, extension into surrounding structures, indistinct margins, and heterogeneous attenuation.

 The choice of treatment for adrenal pseudocyst depends on several factors, including endocrine function, symptoms, size, and correctly differentiating it from an adrenal cyst. Hyperfunction is not typically present in adrenal pseudocysts. Surgery is indicated for masses larger than 5 cm or when there is suspicion of malignancy. Fine needle aspiration should only be implemented before surgery if extra-adrenal malignancy is suspected.

 Surgical excision may be achieved through an abdominal incision (as was done in our case study), groin incision, or by a thoracoabdominal approach. The prognosis for a benign cyst is excellent. This was evident in our patient's case, and her recovery and follow-up continue to be uncomplicated.

	There is an association between hypertension and nonfunctioning adrenal cysts. Resolution of hypertension after excision has been reported; however, this was not the case with our patient. Suggested causes of hypertension in patients with large adrenal masses include compression of the renal artery or parenchyma, leading to release of renin; increase in catecholamines secondary to compression of the renal medulla; and compression of the cortex, leading to adrenal regeneration with hypertension.

 Size and functionality dictate the treatment approach. Small benign adrenal cysts (3.0 to 4.5 cm) are closely monitored for 18 months. If the size and functionality of the cyst remain stable, follow-up should be extended to include a much longer surveillance time. Symptoms usually appear when the cyst is large enough to cause nonspecific abdominal or flank pain due to compression of surrounding organs. Once this happens, surgical intervention is generally required.

 The most important characteristics of adrenal neoplasms, which determine the correct therapeutic approach, are size and functionality. Neoplasms larger than 5 cm have at least a 10% to 15% increased risk of malignancy compared with neoplasms smaller than 3 cm, which carry low risk. Those tumors that are 3 to 5 cm in size have equivocal risks, and one may have to consider comorbidities, rapidity of growth changes, and symptomatology to determine whether adrenalectomy is appropriate. Functional tumors usually lead to unabating symptoms, and surgical extirpation is recommended. CT scanning and MRI are essential to defining adrenal cystic neoplasms. Most pseudocysts are nonfunctional, and surgical decisions usually depend on their size or symptoms related to their size.

 1. Karayiannakis AJ, Polychronidis A, Simopoulos C. Giant adrenal pseudocyst presenting with gastric outlet obstruction and hypertension. 

	2. Doran AHG. Cystic tumour of the suprarenal body successfully removed by operation. 

	3. Medeiros LJ, Lewandrowski KB, Vickery AL Jr. Adrenal pseudocyst: a clinical and pathologic study of eight cases. 

	4. Barzon L, Boscaro M. Diagnosis and management of adrenal incidentalomas. 

	5. Scheibel W, Coel M, Siemers PT, et al. Percutaneous aspiration of adrenal pseudocysts. 

	6. Mohan H, Aggarwal R, Tahlan A, et al. Giant adrenal pseudocyst mimicking a malignant lesion. 

	7. Ghandur-Mnaymneh L, Slim M, Muakassa K. Adrenal cysts: pathogenesis and histological identification with a report of 6 cases. 

	8. Khoda J, Hertzanu Y, Sebbag G, et al. Adrenal cysts: diagnosis and therapeutic approach. 

	9. Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 15-1992. A 23-year-old woman with a painful mass in the right adrenal region. 

	10. Patti G, Fiocca G, Latini T, et al. Prenatal diagnosis of bilateral adrenal cysts. 

	11. Wang LJ, Wong YC, Chen CJ, et al. Imaging spectrum of adrenal pseudocysts on CT. 

	12. Arnold DT, Reed JB, Burt K. Evaluation and management of the incidental adrenal mass. 

 Choose the best answer for each of the following questions.

1. The following mechanism(s) are proposed etiologies for adrenal pseudocysts:

 Degeneration of primary adrenal neoplasms

 Vascular malformation with hemorrhage into the adrenal gland

2. The most common symptom of an adrenal pseudocyst is:

3. Surgery is not indicated for adrenal cysts that are:

 Less than 3 cm and fluid-filled with no septations

Adrenal pseudocysts are rare cystic masses that arise within the adrenal gland and are usually asymptomatic.

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