Cystic Teratoma of the Pancreas: Presentation, Evaluation and - PDF document

JOP. J Pancreas (Online) 2006; 7(6):643-646. CASE REPORT Cystic Teratoma of the Pancreas: Presentation, Evaluation and Management Kevin J Koomalsingh, Rafael Fazylov, Mitchell I Chorost, Joel Horovitz Department of Surgery, Maimonides Medical Center. Brooklyn, NY, USA ABSTRACT CASE REPORT Context Congenital cystic lesions of the A fifty-two-year-old male with past medical pancreas are rare findings. Furthermore, a history of non-insulin dependent diabetes dermoid cyst of the pancreas is exceptionally mellitus and hypertension, presented with uncommon. A review of the world literature nonspecific epigastric pain for several weeks. shows 18 documented cases. The pre- Review of systems was otherwise negative. operative evaluation of this lesion is rather Physical exam revealed mild epigastric questionable, with definitive diagnosis taking tenderness. Laboratory studies were place intra-operatively. essentially normal (Table 1), likewise an Case report A 52-year-old male with a abdominal X-ray. Further evaluation was symptomatic, 3-cm cystic-type mass in the undertaken. Computed tomography (CT) pancreas. imaging of the abdomen demonstrated a 3.5x3.0 cm soft tissue mass arising from the Conclusions From our case presentation and distal portion of the pancreas with possible review of the world literature, we hope to extension to the posterior stomach (Figure 1). establish an increased awareness in the Its exact origin though could not be clearly diagnostic evaluation of these patients. defined. Esophagogastroduodenoscopy demonstrated mild gastritis and endoscopic ultrasound confirmed a cystic lesion arising INTRODUCTION from the pancreatic tail measuring 2.2x3.2 Teratomas can be divided into two subtypes, cm. No septations, vascular invasion or solid mature and immature. The mature type can be components were identified but the lesion did further subdivided into a solid type and a exhibit a non-homogenous appearance cystic type, hence dermoid cyst. A review of (Figure 2). Again, the margins could not be the English literature has identified about clearly identified. Our patient consented to a twenty cases limited to the pancreas, the distal pancreatectomy, with possible rarest site. Derived from totipotent stem cells, splenectomy. Intraoperatively, a cystic lesion they possess the ability to generate tissues was palpated arising from the tail of the from all three germ layers: endodermal, pancreas without any infiltration into the mesodermal and ectodermal. We present our surrounding tissues. The lesion was case and review the literature, in an attempt to intrapancreatic. Its borders were well defined increase awareness for the preoperative and the mass contained predominantly detection of these rare tumors. sebaceous material. Frozen section identified JOP. Journal of the Pancreas - http://www.joplink.net - Vol. 7, No. 6 - November 2006. [ISSN 1590-8577] 643

JOP. J Pancreas (Online) 2006; 7(6):643-646. parenchyma. Pathological evaluation revealed Table 1. Laboratory values at admission. Value Reference a benign teratoma (dermoid cyst). range Histologically, the mass contained stratified White blood cells (x1,000/µL) 8.1 3.8-10.8 squamous epithelium and skin appendages, Hematocrit (%) 43.2 41-50 surrounded by a wall composed of abundant Platelets (x1,000/µL) 260 130-400 lymphoid tissue. Our patient had an Glucose (mg/dL) 128 70-125 uneventful postoperative stay and was Total bilirubin (mg/dL) 0.5 0-1.3 discharged home on post-op day three. Direct bilirubin (mg/dL) 0.1 0-0.4 Laboratory values prior to discharge were Alkaline phosphatase (IU/L) 42 20-125 again normal. At 16-month follow-up, our AST (IU/L) 32 0-48 patient has been asymptomatic, without any ALT (IU/L) 34 0-42 evidence of recurrence. Amylase (IU/L) 72 30-170 Total cholesterol (mg/dL) 141 0-200 DISCUSSION the specimen as a cystic teratoma. A simple cystectomy was performed, without any Dermoid cysts are thought to arise from the compromise to the surrounding pancreatic embryonic inclusion of skin, at the time of neural groove closure [1], therefore, typically found lying along the midline. Most commonly located in the ovaries, they have Figure 1. Computed tomography scan. A homogenous mass is seen arising from the tail of the pancreas, (large Figure2. EUS. A cystic lesion with soft tissue arrows) in close proximity to the posterior stomach attenuation arising from the tail of the pancreas (dashed (arrowhead). The mass appears non-homogenous (not lines). The lesion measures 2.2x3.2 cm. No infiltration depicted). into the vessels is seen. JOP. Journal of the Pancreas - http://www.joplink.net - Vol. 7, No. 6 - November 2006. [ISSN 1590-8577] 644

JOP. J Pancreas (Online) 2006; 7(6):643-646. been found at several extragonadal sites, the areas of calcifications and fat and characterize pancreas being most rare. the fluid as sebum, serous or complex [3]. Surrounding the lesion is the cyst wall, which Magnetic resonance imaging (MRI) can also may contain adnexal tissue, sebaceous glands, be performed for further characterization. We lymphoid tissue and even inflammatory cells. did not proceed with MRI. But expected Beneath this surface, lies a single layer of findings may include: low signal intensity on keratinizing squamous epithelium [1]. The T1-weighted images [3], areas of fat-fluid inner compartment, often filled with thick, level, if present, and distinct margins. pasty, doughy sebaceous secretions, contains At this point an excisional biopsy is usually fully differentiated tissue(s) from one or more performed with the possibility of a more germ cell layers, most commonly ectodermal. extended resection, if warranted. However, Amongst the list of differentials includes the prospect of cytologic diagnosis should not pseudocyst and neoplastic cysts, including be overlooked. In 1991, Markovsky et al . [4] both benign and malignant lesions. As stated described the findings of the first reported by Brugge et al ., the clinical challenge rests in preoperatively diagnosed cystic teratoma by distinguishing the more common benign fine needle aspiration. Cytological findings cystic lesions from their rarer, malignant included mature benign squamous cells, counterparts [2]. Our clinical concern, keratin debris and inflammatory cells (the therefore, was ruling out a malignant process. three predominant cell types, also found in Though benign, dermoid cysts prove ovarian dermoid cyst). Furthermore, clinically and radiologically challenging in Markovsky et al . illustrated that such differentiation from the more concerning histological findings are inconsistent with lesions, but once identified can be treated other pancreatic disorders as pseudocyst, appropriately. pancreatitis and degenerated carcinomas for Akin to most pancreatic cystic lesions, their their lack of specific histological elements. clinical presentation is nonspecific. The Despite our failure to perform an FNA, we do symptomatology has ranged from an believe in its selective utility in asymptomatic asymptomatic palpable mass to obstructive patients and patients considered high-risk jaundice with liver failure. Most patients surgical candidates. If a differential diagnosis though, present with varying severity of for a cystic lesion in the pancreas has been abdominal pain, back pain, vomiting or assimilated and radiologic evidence is jaundice. inconclusive but consistent with the features Laboratory values will likely be normal illustrated above, a fine needle aspiration for unless an obstructive pattern to the normal cytologic analysis can confirm the diagnosis drainage of biliary or pancreatic secretions pre-operatively. exists. Unlike dermoid cysts elsewhere in the Treatment has been surgical. Observation has body, little radiographic evidence is available not been reported. Can these lesions be regarding their pancreatic location. However, observed? With a high enough pre-test extrapolating the documented findings to the probability (based on cross sectional imaging pancreas, it appears equally so that the studies and cytology findings consistent with radiologic appearance of these lesions dermoid cyst) these lesions can be safely depends on the proportions of the various observed. For the eighteen reported cases tissues of which they are composed [3]. though, surgical procedures included simple Ultrasound will initially define the mass as cystectomy (9 patients, 50%), external cystic, without septations and with distinct drainage procedures (5 patients, 20%), distal margins. The fatty component would be pancreatectomy (1 patient, 6%), distal expected to appear hyperechoic with focal pancreatectomy plus splenectomy (1 patient, areas of high-intensity signals plus acoustic 6%), cystogastrostomy (1 patient, 6%), and shadowing, secondary to the presence of one case unreported (6%). The trend over the calcified tissues [3]. CT will confirm these years has steered away from external drainage JOP. Journal of the Pancreas - http://www.joplink.net - Vol. 7, No. 6 - November 2006. [ISSN 1590-8577] 645