A case of cystic lymphangioma of the pancreas

We report a case of a 58-year-old man presenting with a mild attack of upper abdominal pain and who was found to have a swollen pancreatic body and tail. Pathological study of the resected mass revealed cystic lymphangioma. Only 30 cases of lymphangioma of the pancreas, including our case, have been reported to date. Here we present this case in detail and discuss the differential diagnosis of cystic lesions in the pancreas.     

Adrenal lymphangioma masquerading as a pancreatic tail cyst

Cystic lymphangiomas of the adrenal gland are rare. A 79-year-old female presented in the emergency room with epigastric discomfort, and an immovable mass was palpated in her abdomen upon physical examination. Imaging studies revealed a large cystic lesion in the pancreatic tail. The radiologic impression ruled out the possibility of a mucinous cystic neoplasm, or a pseudocyst in the pancreas. The operative findings demonstrated that the cystic mass originated in the left adrenal gland. A laparoscopic excision of the cystic mass was performed, and immunohistochemistry confirmed that this mass was a lymphangioma of the adrenal gland. Several prior reports have suggested that lymphangioma can mimic renal or splenic cysts. However, lymphangioma cases mimicking pancreatic cysts are very rare.     

Cystic neoplasms of the pancreas: a series.

MATERIAL AND METHODS: a series of 23 cases with cystic pancreatic neoplasms studied and treated between December 1977 and December 2001 out of 297 cases of pancreatic neoplasms is presented. RESULTS: 16/23 were true pancreatic cystic neoplasms: 9 microcystic adenomas and 6 mucinous cystoadenoma type. There were also 2 hydatid cysts, 1 lymphangioma, 2 cases of tuberculosis involving de pancreas, 1 lymphoma and 1 papillary cystic tumor type. CONCLUSIONS: true cystic tumors of the pancreas accounts for 5,8% of the neoplasms studied in our hospital.     

Cystic lymphangioma of the pancreas

Lymphangioma of the pancreas is an extremely rare benign tumour of lymphatic origin, with fewer than 60 published cases. Histologically, it is polycystic, with the cysts separated by thin septa and lined with endothelial cells. Though congenital, it can affect all age groups, and occurs more frequently in females. Patients usually present with epigastric pain and an associated palpable mass. Complete excision is curative, even though, depending on the tumour location, surgery may be simple or involve extensive pancreatic resection and anastomoses. The authors present a 49-year-old woman in whom a polycystic septated mass, 35 mm× 35 mm in size, was discovered by ultrasonography （US） in the body of the pancreas during investigations for epigastric pain and nausea. At surgery, a well circumscribed polycystic tumor was completely excised, with preservation of the pancreatic duct. The postoperative recovery was uneventful. Histology confirmed a microcystic lymphangioma of the pancreas. Immunohistochemistry showed cystic endothelial cells reactivity to factor Ⅷ -RA （＋＋）, CD31 （＋＋＋） and CD34 （-）. Postoperatively, abdominal pain disappeared and the patient remained symptomfree for 12 mo until now. Although extremely rare, lymphangioma of the pancreas should be taken into consideration as a differential diagnosis of a pancreatic cystic lesion, especially in women.     

Pancreas cystic lymphangioma diagnosed with EUS-FNA

CONTEXT: Endoscopic ultrasound has proved to be an invaluable tool when obtaining high quality images of the pancreas. Furthermore, fine-needle aspiration of suspected lesions can be carried out simultaneously thus providing tissue samples for cytologic diagnosis. We present two cases of a rare pancreatic lesion that were diagnosed by endoscopic ultrasound with fine-needle aspiration. CASE #1: A 60-year-old asymptomatic gentleman was found to have an incidental pancreatic lesion on abdominal computed tomography scan during a cardiac workup. Patient had no personal or family medical history that would predispose him to pancreatic lesions. Endoscopic ultrasound was performed and patient was diagnosed with pancreatic cystic lymphangioma. CASE #2: A 40-year-old asymptomatic gentleman with history of heavy alcohol use was found to have an incidental pancreatic lesion on computed tomography scan during a work up of chest pain. Computed tomography guided fine-needle aspiration was negative for malignancy but no other studies were performed on the fluid sample at that time. Patient was then referred to our institution after repeat computed tomography scan showed a stable lesion. Endoscopic ultrasound did not show evidence of pancreatitis and fine-needle aspiration was consistent with pancreatic cystic lymphangioma. DISCUSSION: The universally available and escalating use of computed tomography scans has led to an increased detection of incidental cystic pancreatic lesions. Pancreatic cystic lymphangiomas are a rare lesion and account for less than one percent of all pancreatic cystic lesions. These lesions are easily and accurately diagnosed by the use of endoscopic ultrasound guided fine-needle aspiration.     

Mucinous cystic neoplasm of the pancreas during pregnancy: the importance of proper management

We describe a case of huge mucinous cystic tumor of the pancreas in a 26-year-old woman during pregnancy. Ultrasonography demonstrated a well-delimited cystic mass in the left upper abdominal quadrant, suggestive of benignity. Magnetic resonance imaging showed a large cystic mass resembling a mucinous cystic tumor of the pancreas. After this assessment the patient underwent surgical exploration and a huge cystic tumor of the pancreas was discovered. The tumor was enucleated and distal pancreatectomy was performed. The resected margin of the specimen was free of tumor. In this case report we discuss the management of mucinous pancreatic tumors during pregnancy and we briefly review the previously reported cases of mucinous pancreatic tumors in pregnant patients. We conclude that surgical resection of these tumors should be strongly considered in pregnancy. Removal of the tumor appears to be a safe procedure without harmful effects to the fetus.     

Splenic cystic lymphangioma in a young woman: case report and literature review

Splenic cystic lymphangioma is extremely rare, with very few cases reported until now. Here, we report a case of cystic lymphangioma of the spleen in a young woman who was admitted for evaluation of abdominal pain and a mass lasting for two years. We present this case with emphasis on the problem of differential diagnosis and the difficulties of diagnostic certainty in the absence of histologic features.     

Adrenal pseudocyst mimicking cystic neoplasm of pancreatic tail

Cystic neoplasms of the pancreatic tail are usually picked up as incidental findings during imaging studies. We report an unusual case of adrenal pseudocyst mimicking cystic tumor of pancreatic tail who presented with abdominal pain and weight loss. Ultrasonography and CT of the abdomen revealed a cystic mass in relation to the pancreatic tail. On exploration, a cystic mass was present in the tail of the pancreas, for which distal pancreatectomy was performed. The histopathology report surprisingly revealed an adrenal pseudocyst without a cellular lining. The normal adrenal gland was compressed in the wall. This case highlights that this rare possibility should be considered in the differential diagnosis of cystic lesions of the pancreatic tail.     

Retroperitoneal cystic lymphangioma in an adult: A case report and review of the literature

Lymphangiomas are rare benign cystic tumors of the lymphatic system. Retroperitoneal lymphangiomas account for 1% of all lymphangiomas, and approximately 186 cases have been reported. They may clinically present as a palpable abdominal mass and can cause diagnostic dilemmas with other retroperitoneal cystic tumors, including those arising from the liver, kidney and pancreas. This report describes the rare case of a cystic retroperitoneal lymphangioma in a 54-year-old male patient. The lymphangioma had progressed to the point of inducing clinical symptoms of abdominal distention, abdominal pain, anorexia, fever, nausea and diarrhea. Radiological imaging revealed a large multiloculated cystic abdominal mass with enhancing septations involving the upper retroperitoneum and extending into the pelvis. Surgical removal of the cyst was accomplished without incident. A benign cystic retroperitoneal lymphangioma was diagnosed on histology and confirmed with immunohistochemical stains.     

Minimally invasive excision of multiple cystic lymphangiomas of the mediastinum: a case report

Mediastinal lymphangioma is one of the rarest benign mediastinal masses. We report the successful minimally invasive surgical treatment of multiple cystic lymphangiomas in a middle-aged man with a familial history of lung cancer. This case report describes cystic lymphangioma as a rare differential diagnosis for a mediastinal mass in an adult.     

Lymphoepithelial cyst of the pancreas: report of a case

The aim of this report is to describe the clinical and pathological features of a lympoepithelial cyst of the pancreas and to recommend fine-needle aspiration biopsy for the differential diagnosis of this lesion. A 55-year-old man was incidentally diagnosed as having a pancreatic tumor by abdominal ultrasonography. A hypoechoic cystic lesion was detected on the surface of the pancreatic body. A computed tomography revealed a cystic lesion surrounded by a smooth, flat wall. Magnetic resonance imaging revealed that the lesion was a low-intensity mass on the T1-image and a slightly high-intensity mass on the T2-image. The lesion was diagnosed as a benign cystic tumor, and enucleation of the tumor was scheduled. The fine needle aspiration biopsy was performed after laparotomy, and cytology of the contents was negative for malignant cells. The postoperative pathological diagnosis was a lymphoepithelial cyst of the pancreas. This cyst is an unusual but benign mass that requires minimal surgery.     

Hemosuccus pancreaticus caused by a mucinous cystic neoplasm of the pancreas

Hemosuccus pancreaticus is a gastrointestinal hemorrhage through the main pancreatic duct. Here, we report a rare case of hemosuccus pancreaticus due to a mucinous cystic neoplasm of the pancreas. A 62-year-old woman who had been followed for a branch duct intraductal papillary mucinous neoplasm visited our emergency room due to severe abdominal pain and bloody discharge. Computed tomography revealed that the pancreatic cyst increased the tension of the wall and a high-density area indicative of bleeding into the cyst was observed. Endoscopy showed opening of and hemorrhaging from the papilla of Vater. The patient was diagnosed with hemosuccus pancreaticus caused by hemorrhaging into the cyst from the branch duct intraductal papillary mucinous neoplasm. Based on this diagnosis, elective distal pancreatectomy was performed. The histopathological diagnosis was a mucinous cystic neoplasm with intermediate-grade dysplasia based upon the pathological findings that fibrous ovarian-type stroma existed abundantly and the stroma cells were positive for progesterone receptor and inhibin. Hemosuccus pancreaticus caused by a mucinous cystic neoplasm is extremely rare and there has been only one case reported to date. In conclusion, it should be recognized that pancreatic cystic neoplasms including mucinous cystic neoplasms may cause hemosuccus pancreaticus.     

Macrocystic type of serous cystadenoma with a communication between the cyst and pancreatic duct

A 42-year-old woman with a cystic lesion in the head of the pancreas was evaluated by using abdominal ultrasonography, a computed tomographic scan, magnetic resonance imaging and endoscopic retrograde pancreatography. Multiple cystic lesions, 5 cm in diameter, which had papillary protrusion inside the cyst in the head of the pancreas and had the communication between the cysts and pancreatic duct, were determined. Pylorus-preserving pancreaticoduodenectomy was performed under the diagnosis of mucinous cystic neoplasm of the pancreas. Although the cut surface of the tumor showed a macrocystic tumor of 3 cm in diameter, part of the cyst wall was cavernous. A histopathological examination showed single-layered cuboidal cells, which lead to the diagnosis as being serous cystadenoma of the pancreas. Serous cystadenoma is a rare, almost benign pancreatic tumor. The macrocystic subtype of serous cystadenoma is even more rare. We describe a patient who had this macrocystic subtype of serous cystadenoma with a communication between the cyst and pancreatic duct. This case illustrates the difficulty in the diagnosis of cystic lesions in the pancreas, and might support the single category of cystic lesions of the pancreas.     

Rare case of adult pancreatic hemangioma and review of the literature

Pancreatic hemangiomas are a rare type of cystic tumor, with very few cases reported in the literature. Herein, we present the case of a 28-year-old woman who was admitted to our hospital for abdominal pain. A physical examination failed to reveal any abnormalities that could explain her symptoms. A contrast-enhanced computed tomography showed a multilocular cyst with moderately enhanced septa and fluid-fluid levels in the body and tail of the pancreas. A serous cystadenoma or pseudocyst of the pancreas was initially suspected, and the patient underwent a subtotal pancreatectomy and splenectomy. The pathologic diagnosis was a pancreatic hemangioma. This is the second case of pancreatic hemangioma with fluid-fluid levels reported in the literature. Upon imaging, the presentation of this tumor can resemble serous or mucinous cystadenomas, pseudocysts of the pancreas, and side-branch type intraductal papillary mucinous neoplasms. This report reviews the clinical symptoms, radiologic features, pathologic characteristics, differential diagnoses, and treatment of this rare lesion type.     

Serous cystadenoma of the pancreas associated with obstructive jaundice

We herein report a case of pancreatic serous cystadenoma in a patient who presented with jaundice, and we provide a review of the literature. A 53-year-old man was admitted with complaints of jaundice and weight loss. With a preoperative diagnosis of pancreatic serous cystadenoma with obstructive jaundice, he underwent pylorus-preserving pancreatoduodenectomy. A cystic tumor partially protruding into the bile duct was observed in the pancreatic head. Histology verified serous cystadenoma of the pancreas. Histologically, no atypia was proven in the epithelium. There have been only eight case reports dealing with serous cystadenoma of the pancreas with obstructive jaundice. Although serous cystadenoma of the pancreas has essentially a benign nature, pylorus-preserving pancreatoduodenectomy is the treatment of choice when available to avoid the recurrence of obstructive jaundice.     

Pancreatic tuberculosis masquerading as pancreatic serous cystadenoma

Solitary pancreatic involvement of tuberculosis is rare, especially in an immunocompetent individual, and it may be misdiagnosed as pancreatic cystic neoplasms. Pancreatic cystic neoplasms are being identified in increasing numbers, probably because of the frequent use of radiology and advances in endoscopic techniques. However, they are composed of a variety of neoplasms with a wide range of malignant potential, and it is often difficult to differentiate pancreatic tuberculosis mimicking cystic neoplasms from benign or malignant pancreatic cystic neoplasms. Non-surgical diagnosis of pancreatic tuberculosis is inconclusive and continues to be a challenge in many cases. If so, then laparotomy should be employed to establish the diagnosis. Therefore, pancreatic tuberculosis should be kept in mind during the differential diagnosis of solitary cystic masses in the pancreas. We report a patient who had solitary pancreatic tuberculosis masquerading as pancreatic serous cystadenoma.     

Cystic tumors of the pancreas

The discovery of a cystic lesion in the pancreas implies a challenge for the physician. Approximately 10% are cystic tumors, benign to highly malignant, or true cysts, showing all stages of cellular differentiation, from benign to highly malignant tumors. Malignant cystic tumors are rare and comprise only about 1% of all pancreatic malignancies, they are potentially curable. Therefore, correct diagnosis and treatment of these lesions are of great importance. It is usually not possible to separate a pseudocyst from a benign cyst or a cystic tumor, but there are some signs and findings that could be helpful in the clinical decision. The diagnosis of a cystic pancreatic tumor requires different imaging techniques, including ultrasonography, computerized tomography, magnetic resonance imaging, and magnetic resonance cholangiopancreatography, but to distinguish a pseudocyst or a benign cyst from a potentially malignant lesion can be very difficult. The usefulness of blood tests and investigations of cyst fluid can be questionable. Today, surgical treatment of cystic pancreatic tumors can be performed with low morbidity. Therefore, we conclude that an active strategy with resection of cystic tumors of the pancreas should be recommended.     

Cystic endocrine tumor of the pancreas.

A large cystic tumor in the pancreatic body was found incidentally in an 85-yr-old male. A distal pancreatectomy was performed after a diagnosis of cystadenocarcinoma. Microscopic examination of the resected specimen revealed a pancreatic cystic endocrine tumor; however, this tumor produced no symptoms. Immunohistochemical studies of the tumor cells showed positivity for gastrin, neuron-specific enolase, chromogranin A, and synaptophysin, and two cell types of neurosecretory granules were recognized in electron-microscopic studies. Although endocrine tumors of the pancreas are usually solid and cystic change occurs only rarely, such tumors should be considered in the differential diagnosis of patients who have a cystic lesion in the pancreas.     

Two cases of pancreatic ductal adenocarcinoma, manifested as solid pseudopapillary tumor and intraductal papillary mucinous neoplasm]

Compared with other types of cancers, pancreatic cancer is one of the most dreadful malignancies and is fifth leading cause of cancer-related death in Korea. It is difficult to expect early diagnosis or improvement in prognosis due to lack of specific early symptoms and effective diagnostic methods. Whereas cystic neoplasm of the pancreas is a rare type of pancreatic tumor, surgical resection provides good prognosis because of its low possibility of local invasion or distant metastasis. In case of pancreatic cystic tumor, radiologic differentiation between benign and malignant lesions is crucial for the selection of appropriate treatment and the prediction of prognosis. And ductal adenocarcinoma of pancreas presenting in cystic form is an uncommon type of cystic tumor, making it extremely rare among all pancreatic malignancies. We report two cases of atypical pancreatic ductal adenocarcinoma presenting as solid pseudopapillary tumor and intraductal papillary mucinous neoplasm, respectively.     

Cystic ductal adenocarcinoma of pancreas: an unusual variant.

Cystic lesions of the pancreas are usually pseudocysts (90%); only 10% of them are cystic tumors. These cystic tumors constitute less than 10% of all pancreatic neoplasms, making them an extremely uncommon type of pancreatic malignancy. What is more important is that these tumors are very easily misdiagnosed as pseudocysts because their characteristics are very similar to those of the benign pseudocysts. This gains importance as the cystic tumors have a high cure rate and good prognosis if diagnosed and treated on time. Of all the cystic tumors, the most common are the benign serous cystadenomas, mucinous cystic tumors, intraductal papillary mucinous neoplasms etc. Ductal adenocarcinoma of pancreas presenting in cystic form is an uncommon type of cystic tumor, making it extremely rare among all pancreatic malignancies (solid or cystic). We present the following case report. The review of literature concerning the diagnosis and management has also been discussed.