Apollo Medicine

CASE REPORT
Year
: 2018  |  Volume : 15  |  Issue : 1  |  Page : 32--34

Dermoid cyst of pancreas: A rare entity


Apurva S Shah, Shravan Bohra, Lakshman Khiria 
 Department of Gastroenterology, Apollo Hospitals International Limited, Ahmedabad, Gujarat, India

Correspondence Address:
Apurva S Shah
Apollo Hospitals International Limited, Ahmedabad - 382 428, Gujarat
India

Abstract

Dermoid cysts or mature cystic teratomas are mesenchymal neoplasms most commonly found in the ovaries, but which may occur in any location along the pathways of ectodermal cell migration. Pancreatic dermoid cysts are rare entity and preoperative diagnosis is difficult. A 27-year-old female presented with upper abdominal pain and vomiting of 1 week duration without jaundice, diarrhea, and fever. Ultrasound abdomen showed a 4 cm × 3 cm cyst in the head of pancreas with smooth walls. Magnetic resonance cholangiopancreatography showed large multilocular cystic lesion in pancreatic head region which displaces pancreas anteriorly and mildly superiorly. Endoscopic ultrasound-guided cyst fluid aspiration was done which revealed high cyst fluid amylase and carcinoembryonic antigen. At surgery, a pylorus-preserving pancreatoduodenectomy was done. Histopathologic examination revealed a rare diagnosis of dermoid cyst of pancreas. This case report highlights difficulty in preoperative diagnosis of cystic lesions of pancreas by imaging. Therefore, we want to highlight the literature in this rare entity.



How to cite this article:
Shah AS, Bohra S, Khiria L. Dermoid cyst of pancreas: A rare entity.Apollo Med 2018;15:32-34


How to cite this URL:
Shah AS, Bohra S, Khiria L. Dermoid cyst of pancreas: A rare entity. Apollo Med [serial online] 2018 [cited 2022 May 21 ];15:32-34
Available from: https://www.apollomedicine.org/text.asp?2018/15/1/32/229064


Full Text

 Introduction



Dermoid cysts or mature cystic teratomas are mesenchymal neoplasms most commonly found in the ovaries, but which may occur in any location along the pathways of ectodermal cell migration. Pancreatic dermoid cysts are rare entity with less than 40 cases described in world literature. Dermoid cysts, in general, occur in all ages, have no sex preference. The clinical picture is non-specific and the preoperative diagnosis is difficult. The objective of this report is to present a case of dermoid cyst of the pancreas in young lady highlighting difficulty in preoperative diagnosis of cystic lesions of pancreas and to review epidemiological, clinical, diagnostic and histopathological aspects of this rare neoplasm.

 Case Report



A 27-year-old female admitted to our hospital with upper abdominal vague pain and vomiting of 1-week duration without diarrhea, fever, and jaundice. The patient had no significant medical or surgical history in the past. A physical examination was unremarkable without any tenderness or palpable mass in the abdomen. Initial laboratory tests were unremarkable except high C-reactive protein levels of 69 mg/L (reference range <6 mg/L). An ultrasound abdomen showed 4 cm × 3 cm cyst in head of pancreas. Further, laboratory studies revealed normal carcinoembryonic antigen (CEA) level of 1.55 ng/mL (reference range: 0–4 ng/mL), but elevated CA 19-9 of 1789 IU/mL (reference range: 0–39 IU/mL).

Magnetic resonance cholangiopancreatography showed large multilocular cystic lesion of 5.5 cm × 3.1 cm × 5 cm in pancreatic head region which displaces pancreas anteriorly and mildly superiorly [Figure 1]. A contrast-enhanced computed tomography abdomen revealed multiple rim enhancing necrotic lesions conglomerated with each other showing calcification in their wall, posterosuperior to pancreas displacing it upward and anteriorly, with mild prominence of suprapancreatic common bile duct (CBD) [Figure 2]. Largest one is 3.9 cm × 2.7 cm. Pancreas, CBD and pancreatic duct were normal. At endoscopic ultrasound (EUS), two cystic lesions of 36 mm × 25 mm and 23 mm × 18 mm size with calcifications at periphery of lesions, seen in peripancreatic head region with large hypoechoic nodal lesion seen in retroperitoneum. The pancreatic tail, head, and uncinate process were all unremarkable, as well as the CBD, gallbladder and proximal pancreatic duct. EUS-fine needle aspiration (FNA) from cystic lesion and nodal lesion was done. Cystic fluid amylase and CEA were 13189 U/L and 279 ng/ml, respectively. GeneXpert of nodal lesion was negative for mycobacterium tuberculosis. Due to the persistent symptom of pain even on intravenous analgesics and the uncertain nature of the neoplasm, the decision was made to resect the mass.{Figure 1}{Figure 2}

At surgery, pylorus-preserving pancreatoduodenectomy was done with resection of retroperitoneal lymph nodes. Macroscopically, cut surface of pancreas revealed cystic lesion of 5 cm × 2.8 cm which was multiloculated with whitish and yellowish areas. Focally firm to hard areas also identified. Microscopically, cyst was benign, and the cyst wall was lined by mature stratified squamous epithelium and surrounded by lymphoid tissue containing germinal centers and sebaceous glands. There was no evidence of malignancy within the cyst lining or the surrounding pancreatic tissue. The morphological features were consistent with a benign dermoid cyst of the pancreas [Figure 3].{Figure 3}

Postoperative recovery was unremarkable, and the patient had a favorable evolution, with no complications, being discharged 2 weeks after surgery. She remains without complaints.

 Discussion



Teratomas are tumors that are composed of tissue derived from all three-germ layers. Cystic mature teratomas are known as dermoid cyst. Pancreatic dermoid cysts are extremely rare entity.[1],[2]

To the best of our knowledge, the world literature reports 36 cases of pancreatic dermoid cysts with complete data.[3] Pancreatic dermoid cysts usually develop at a young age, with a mean age at diagnosis of 34.7 years (range 2–74 years). There is also a slight male preponderance in reported cases (59% men, 41% women).[2],[4] Although teratomas have been shown to develop anywhere in the pancreas, most lesions are located in the body or the head (47% and 41%, respectively), while the tail of the pancreas is less frequently involved (12%).[4],[5] In our patient, the lesion was situated on the superior portion of the pancreatic head.

Clinical presentation is nonspecific. Patients may complain of nausea, vomiting, abdominal pain, back pain, weight loss, anorexia, fatigue, and fever;[2],[4],[5] however in some cases, patients are asymptomatic and it may be incidental finding in diagnostic imaging.[6],[7] Laboratory tests are usually unremarkable unless the lesion hinders the flow of biliary or pancreatic fluids. Serum levels of CEA and CA19-9 are considerably lower in dermoid cysts, although this was not the case in our patient.[1],[2] The most common location of the mass was in the head and/or body, with only five cases identified as being wholly, or even partially within the tail.[8],[9]

The preoperative diagnosis, due to rarity of lesion, is difficult. The radiologic appearance is variable, depending on the proportions of the diverse tissues of which they are composed. The presence of hypo-attenuated fat areas within the cyst is considered highly suggestive of cystic teratoma, particularly if calcifications are detected in the cyst wall. The presence of fat/fluid or hair/fluid levels is considered pathognomonic of dermoid in other locations, but their presence occurs in only a minority of cases.[4] When cross-sectional imaging does not allow definitive diagnosis, EUS-FNA may provide additional useful information.[1] To further confound the issue, on EUS our case was predominantly hypoechoic, which differed from the description of dermoid that contain a predominant fatty component as being echogenic.[2],[4]

Dermoid cysts are true cysts; thus, their wall consists of stratified squamous epithelium and underlying connective tissue.[4] This characteristic is important to distinguish them from pancreatic pseudocysts, which correspond to 90% of cystic lesions of the pancreas and are collections of pancreatic secretions enclosed in a fibrous wall with no epithelium lining.

The treatment is based on surgical removal through either open surgery (distal pancreatectomy, enucleation, cystectomy, and external drainage) or laparoscopy.[2] Despite being benign in nature, ambiguity regarding a diagnosis often leads to surgical resection. Not only would excision of the lesion provide definitive diagnosis, it is also expected to lessen symptoms.[5],[10] Simple excision (enucleation or cystectomy) of the lesion, preceded by intraoperative frozen section, usually suffices as recurrence or evidence of malignant degeneration, has not been reported.[4],[5] Intraoperative frozen examination is a useful tool that may help prevent overtreatment of pancreatic masses in young individuals. Histology of the cyst in our case revealed squamous epithelium and sebaceous glands but lacked significant components of adipose tissue or hair.

Pancreatic dermoid cysts are benign neoplasms with good prognosis and have been little reported in literature. They must be remembered during preoperative investigation of cystic pancreatic lesions, to enable a more conservative treatment, like the laparoscopic enucleation, which may be used as a good alternative to prevent overtreatment in young individuals.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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