Rare Cystic Tumor Research Articles

Mucinous Cystic Neoplasm of the Liver- Three Rare Cases with Different Clinical Impressions

Mucinous Cystic Neoplasm of the Liver (MCN-L) is a rare cystic epithelial tumour that does not communicate with bile ducts and comprising ovarian-type mesenchymal stroma and mucinous epithelium. Though it is a benign tumour it may be associated with invasive carcinoma. Therefore, complete surgical excision and extensive sampling is important to prevent recurrence and detect associated malignancy. Its rarity precludes its recognition as it can mimic other cystic liver lesions. Awareness of this rare entity is essential as it lacks well-defined characteristics and poses a considerable diagnostic challenge preoperatively. Here we present three cases of MCN-L with different clinical impressions preoperatively. All three were women who presented with abdominal pain or discomfort and were found to have cystic lesions in the liver which subsequently underwent excision. The preoperative diagnoses were hydaticd cyst, hepatic abscess and simple liver cyst in case 1 ,2 and 3 respectively. Histopathology of all three cysts were MCN-L with no high grade dysplasia or invasive carcinoma.

Multilocular Cystic Nephroma. Kidney Disease Difficult To Diagnose Before Surgery: A Case Report

Cystic nephroma is a benign renal lesion. It is a benign and rare cystic tumor of the kidney whose diagnosis needs renal specimen after nephrectomy. We report an observation of 62-year-old woman, obese with a history of type 2 diabetes who consults for left lumbar pain without hematuria. The examination found no lumbar contact or signs of urinary tract infection. The first exploration by an abdominal echotomographic shows the existence of an inferior medieval and polar lesion of 84mm of the large axis, heterogeneous with multiple anechoic zones within it of small sizes. The interpretation made by the radiologist retained 3 possible diagnoses, namely: a renal mass suspected of malignancy, a heterogeneous multicystic lesion and a hydatid cyst stage III of Gerbi. The computed tomography found a heterodyne left medieval tumor process and lower polar poorly limited with several areas of necrosis of 80mm of large diameter taking the contrast and interpreted as a malignant lesion, without hilar lymphadenopathies or retroperitoneal visible. The rest of the extension balance sheet was free of anomalies. Hydatid serology was performed and returned negative. The patient was operated on with the diagnosis of kidney cancer. The intraoperative exploration showed the existence of a left kidney increased in volume in its compartment, the accidental break-in of the latter allowed even the multilocular cystic appearance of small infracentimetric sizes of this lesion. An enlarged total nephrectomy was realized. Macroscopic examination of the part reveals a kidney carrying multiple cystic lesions arranged one against the other without nephrogenic tissue in the septic letting out a clear liquid. The pathology study showed the appearance of a multilocular cystic nephroma. Our patient is alive at 48months and in well health.

Mucinous Cystadenoma of Liver with Mesenchymal Stroma

Hepatobiliary cystadenoma is a rare benign cystic tumor of the liver. These are predominantly located in right hepatic lobe. A 45 year old female presented with abdominal pain, epigastric discomfort, fever and vomiting. On radiological evaluation USG showed large multiloculated cystic mass in right lobe of liver. Contrast Enhanced CT examination exhibited exophytically growing multiloculated cytic lesion with differential content and peripheral capsular and central enhancement with imaging diagnosis of hydatid cyst was given. The surgical excision of hepatic cyst was done. On histopathology reported as mucinous cystadenoma of liver with mesenchymal stroma. We are presenting this case for its rarity, clinical, radiological and histopathological findings.

Recurrent Giant Intrahepatic Biliary Cystadenoma: Case Report and Review of Literature

AbstractBiliary cystadenoma is a very rare cystic neoplasm of the liver that usually occurs almost exclusively in middle-aged women. These are slowly progressive tumours and cannot be safely differentiated from cystadenocarcinomas on preoperative imaging and hence should always be considered for resection. Historically these cystic tumors have been treated by a variety of techniques including aspiration, fenestration, internal drainage, partial resection resulting in high rates of recurrence. In addition biliary cystadenoma is a premalignant lesion and only surgical excision can differentiate it from its malignant counterpart, biliary cystadenocarcinoma.

A rare cause of intra-abdominal cysts: pancreatic cystic lymphangiomas.

Echenique et al. described a lymphangioma as a rare cystic neoplasm of the pancreas. We present a similar intra-abdominal lesion diagnosed by endoscopic ultrasound (EUS)-fine-needle aspiration (FNA) cytology.

Rare Cystic Tumor Occluding the External Auditory Canal : Eccrine Hydrocystoma

Rare Cystic Tumor Occluding the External Auditory Canal : Eccrine Hydrocystoma

Mature Cystic Teratoma of the Pancreas: A Rare Cystic Neoplasm.

Mature cystic teratoma of the pancreas is an extremely rare benign neoplasm. Only 51 cases have been reported in the literature. Its cystic nature often appears to have malignant potential in preoperative image studies. Moreover, no characteristic features could be shown on image studies, such as abdominal CT scan or pancreas MRI. The accurate diagnosis is generally obtained after surgical resection. We present a rare case of a 53-year-old male with mature cystic teratoma of the pancreas, which was confirmed on pathology after laparoscopic distal pancreatectomy.

Spontaneous tension pneumocephalus and pneumoventricle in ecchordosis physaliphora: case report of a rare presentation and review of the literature

We describe the case of a 65-year-old lady who presented with mutism and a right hemiparesis. Imaging showed a severe spontaneous tension pneumocephalus. The cause was diagnosed as Ecchordosis physaliphora (EP). EP is a rare cystic congenital hamartomatous benign notochordal tumor (BNCT) arising from an ectopic notochordal remnant. To the authors’ knowledge, this is the first case of EP to be described in the literature which presented with a life-threatening but treatable condition of severe tension pneumocephalus.

Solid pseudopapillary neoplasm of the pancreas: a single institution experience

Background: Pancreatic solid pseudopapillary neoplasm (SPN) is a rare cystic tumor. It is thought to have low malignant potential, however due to its scarcity, little is known about its long term sequelae and pathogenesis. Curative surgical resection is recommended as the best treatment for long term survival. Methods: Using our IRB approved pancreatic database, we performed an analysis of pancreatic resections from 2011 to 2017. We identified a total of five patients diagnosed with pancreatic solid pseudopapillary neoplasm. Of these patients, four underwent resection at our institute. All demographic and outcome data statistics were analyzed using SPSS v 17.0 software. Results: All five patients were female with a mean age at diagnosis of thirty-two. All patients presented with a complaint of vague abdominal pain as the most common presenting symptom. The average BMI for all patients was 29.4. Racial make-up of this population included African American (2, 40%), Hispanic (2, 40%), and White (1, 20%). There was no history of alcohol or tobacco use in any of the patients. Three patients (60%) were uninsured while two patients (40%) had private insurance. CT was the most common initial imaging used to identify the pancreatic mass. None of the five patients presented with metastatic disease. Tumor size varied greatly; mean size of 7.7cm (with a range 1 cm-17 cm). SPN was most commonly located at the head of the pancreas (60%). Four (80%) patients underwent definitive resection at our institute with R0 resection. The fifth patient was lost to follow-up. The estimated blood loss for each operation was less than 200 ml. The mean length of hospitalization was eight days. There were no major perioperative complications and no evidence of recurrence during follow-up. Conclusion: SPN is a rare pancreatic mass that predominately occurs in young women. We describe five cases at our institute during a six year period. Regardless of the size of tumor, best practice includes R0 resection with clinical follow up to monitor recurrence for excellent prognosis.

Advances in diagnosis and treatment of intrahepatic biliary cystadenoma

Intrahepatic biliary cystadenoma (IBC) is a rare benign cystic neoplasm of liver, with malignant potential to transform into intrahepatic biliary cystadenocarcinoma. IBC predominantly occurs in women up to 85%, showing no special clinical symptoms and a polycystic lesion inside the liver on image examination. It is difficult to differentially diagnose from other cystic lesions of the liver such as simple liver cyst, intraductal papillary neoplasm of the bile duct, etc. The missed diagnosis and misdiagnosis rate were rather high in IBC, which is accurately diagnosed by pathological examination. Complete surgical removal of the tumor is the best way to cure it, and also can bring a satisfactory outcome to patients. Key words: Intrahepatic biliary cystadenoma; Intrahepatic biliary cystadenocarcinoma; Liver mucinous cystic neoplasms; Etiology; Pathogenesis; Clinical manifestation; Therapy

Biliary cystadenoma – A rare cystic neoplasm of liver

Biliary cystadenoma – A rare cystic neoplasm of liver

Trans-abdominal ultrasound features of the newly named intraductal papillary neoplasm of the bile duct

Background: Intraductal papillary neoplasm of the bile duct (IPMN-B) is a rare cystic neoplasm of the liver. This study retrospectively reviewed a relatively large sample of IPMN-B cases for diagnostic clinical or trans-abdominal ultrasound features.

Trans-abdominal ultrasound features of the newly named intraductal papillary neoplasm of the bile duct

Background: Intraductal papillary neoplasm of the bile duct (IPMN-B) is a rare cystic neoplasm of the liver. This study retrospectively reviewed a relatively large sample of IPMN-B cases for diagnostic clinical or trans-abdominal ultrasound features. Methods: The medical records of 20 pathologically confirmed IPMN-B cases were reviewed. Serum concentrations of carbohydrate antigen 19-9 (CA19-9), carcinoembryonic antigen (CEA), and alphafetoprotein (AFP) were evaluated, and images of conventional trans-abdominal ultrasound. According to the relationship between the hepatic mass and the dilated bile duct, we classified cases as type I–IV: I, non-tumor; II, asymmetrical; III, symmetrical; and IV, aneurismal dilation. The clinical and trans-abdominal ultrasound characteristics of these types were compared. Results: The clinical features were non-specific; only malignant cases showed CA19-9 or CEA elevation. Three, 9, 3, and 5 cases were types I, II, III, and IV, respectively, and the diameters of the dilated common bile ducts were 2.27±1.08, 1.82±0.79, 0.57±0.15, and 0.68±0.08 cm. The dilations of the common bile duct of types I and II were significantly larger than that of III and IV. Pathologically, 15, 3, and 2 cases were adenocarcinoma, benign, and two border adenomas. The pathological distribution among the four types differed significantly for all of the benign cases and 50% of the border adenomas were type I or II; types III and IV more aggressively progressed to adenocarcinoma. Conclusions: In patients with IPMN-B, the elevation of serum tumor markers may indicate malignant potential. The typical trans-abdominal ultrasound feature of IPMN-B was significant diffuse dilation of the intrahepatic and extrahepatic bile ducts, especially for benign cases. Some malignant cases only showed serious local bile duct dilation with IPMN. After exclusion of extrahepatic bile duct obstructive disease, IPMN-B should be suspected in patients with significant dilation of the bile duct.

A Systematic Review of Solid Pseudopapillary Neoplasms: An Update Since 2012

Introduction: Solid-pseudopapillary neoplasms (SPNs) were first described by Frantz in 1959 and defined by the World Health Organization in 1996. SPNs are rare cystic tumors of the pancreas which usually present as large, solitary, well-circumscribed lesions. While the majority of patients have localized disease, there has been a reported incidence of malignant transformation of 9-15%. An initial literature review identified 718 cases of SPN that had been reported in the English language up to 2003. A follow up systematic review was then performed which identified 2,744 cases of SPN published in the English literature up to 2012. The aim of this study was to determine if there had been any change in the number of SPN cases detected and their evaluation or management over time since 2012. Methods: A systematic review of SPNs was performed of all articles published in English in PubMed and SCOPUS. Predetermined search strings that included the terms “pancreas” and “pseudopapillary” were used. A review of the bibliography of all studies was performed as part of the systematic review. Results: 1,170 patients with SPN were identifiedin 49 studies published between September 19, 2012 (end date of the previous systematic review) to May 1, 2017. 1,103 (94.3%) patients were female and the mean age was 33 years (S.D. ± 10.7). The most common symptom was abdominal pain in 52.7% with incidental detection in 28.1%. There were 1,114 patients who underwent pancreatic resection. The mean tumor size was 8 cm (S.D. ± 3.7). Follow up was reported for 870 patients, with mean follow-up of 44.7 months (S.D. ± 48.9). Disease-free survival was documented in 1,069 (91.4%) patients with recurrence in 101 (8.6%). The mean time to recurrence was 35.1 months (S.D. ± 30.7). Chemotherapy for treatment of recurrence was administered in seven cases, while transarterial chemoembolization (TACE) was used to treat liver metastases in two cases. Conclusion: The number of SPNs reported in the literature has increased by just over 40% in nearly five years. These tumors continue to be found primarily in young women and are frequently found with nonspecific symptoms, such as abdominal pain, or are found incidentally. Surgery remains the mainstay of treatment with an excellent long term prognosis.

Rare Cystic Neoplasm of the Pancreas With Isolated Metastases to Vertebrae

Accounting for 2-5% of exocrine pancreatic tumors, mucinous cystic neoplasms (MCN) are considered a rare manifestation with a female predominance. These neoplasms of the pancreas are biologically less aggressive than de novo pancreatic adenocarcinoma, and their propensity for metastasis is low. We report an case of an isolated vertebral metastases from a pancreatic adenocarcinoma arising within a cystic neoplasm of the pancreas. This case highlights that slow growing MCN tumors can have unusual patterns of metastases and bolsters the need for careful evaluation of staging tests, including a complete evaluation of the vertebrae. A 63 year old female presented to the E.D. complaining of diffuse abdominal pain & bloating over 4 months. Computed tomography (CT) revealed a cystic abdominal lesion originating from the body and tail of the pancreas. There was no associated pancreatic duct dilatation or solid component visualized. CA199 and CEA levels were elevated with no other lab abnormalities. An endoscopic ultrasound was performed which confirmed a cystic lesion at the body/tail of the pancreas with a mural nodule both of which were aspirated and biopsied, respectively. The biopsies confirmed adenocarcinoma of the pancreas. On review of CT and a subsequent magnetic resonance imaging (MRI) a single hyper dense lesion at L1 was noted and was confirmed to be metastatic pancreatic adenocarcinoma by biopsy. Surgery was no longer warranted and the patient was offered palliative chemotherapy. To the best of our knowledge, this is the first report of an isolated vertebral metastasis from pancreatic cancer originating from an MCN. Pancreatic cystic lesions (PCL) are classified as Non-neoplastic cysts [serous cystadenomas, pseudocysts, epithelial cysts]; and Cystic Neoplasms [intraductal papillary mucinous neoplasm (IPMN) and mucinous cystic neoplasm (MCN)]. Overall, cystic neoplasms of the pancreas have a low risk of malignancy; the estimated risk of malignancy is 0.01-0.21 % depending on cyst size. A Japanese multicenter study in 2004 found that the 5-year survival rate of MCN patients was 100% in adenoma to minimally invasive carcinoma cases and 37.5% in invasive carcinoma cases, yet no cases to date have ever reported isolated distant metastasis to the vertebrae. This case reinforces that even slow growing cystic neoplasms of the pancreas can have unusual patterns of spread; and vigilance to this finding is paramount to making effective management decisions.Figure: Endoscopic Ultrasound: Mucinous Pancreatic Mass with Mural Nodule.Figure: MCN of the Pancreas and Vertebral Metastasis with Corresponding Pathology.

The management of a cystic hepatic lesion ruptured in the bile ducts: a case report

BackgroundHepatic cystadenoma is a rare benign cystic tumor; it tends to recur after incomplete surgical resection and has malignant potential. We report the case of a patient with a ruptured biliary cystadenoma in the common bile duct that caused diagnostic and therapeutic problems.Case presentationA 34-year-old North African woman, admitted for angiocholitis, was operated 2 months before for a hepatic cystic lesion taken for a hydatid cyst compressing her common bile duct. The clinical and the complementary examinations converged toward recurrence of the hydatid cyst for which a surgical resection was decided. Intraoperative findings as well as the histological study of the “membranes” extracted from her common bile duct indicated a hepatic cystadenoma.ConclusionsThe rarity of hepatic cystadenoma and the non-specificity of clinical and imaging signs make diagnosis of hepatic cystadenoma difficult, especially when it is complicated by rupture in the bile ducts; this contributes to a delay in diagnosis and an inadequate therapeutic approach.

Multilocular cystic renal cell carcinoma: A rare case report with review of literature

Multilocular cystic renal cell carcinoma (MCRCC) is a rare adult cystic tumor of the kidney and is considered as a distinct type of clear cell renal cell carcinoma. Most patients are asymptomatic and they are detected incidentally. MCRCC is consistently low nuclear grade and has an excellent prognosis. Concern still persists regarding its true malignant potential. Although surgical resection is curative, lymph node metastasis has been reported recently. There are very few reports on large series of cases and limited available follow-up. We report a case of MCRCC in a 60-year-old male patient who presented with abdominal distention. Careful follow-up is essential in these patients for better prognosis.

Solid (Type 3) pleuropulmonary blastoma

Introduction: Pleuropulmonary blastoma is a rare solid and/or cystic progressive and aggressive malignant intra-thoracic tumor of childhood which belongs to a group of dysontogenetic neoplasms such as Wilms tumor, neuroblastoma and hepatoblastoma. The incidence of pleuropulmonary blastoma ranges between 0.25 and 0.5% of primary lung tumors making this entity a rare primary malignant lung tumor. Case Report: A two-month-old male neonate was referred from a regional hospital with a three-day history of cough, fever and shortness of breath. No previous medical history reported. The patient was delivered at term through a normal vaginal delivery which was uneventful. Birth weight 3 kg, length 48 cm, Apgar score 10/10 and the head circumference was 35 cm. The patient's retro-viral disease status was negative. On percussion there was dullness and bilateral crepitations. There was bilateral decreased air-entry. No signs of cardiac failure. The rest of the systems were within normal limits. The arterial blood gases showed respiratory acidosis with hypoxia. Chest X-ray showed a white out of the right hemithorax and CT scan of the chest showed a large soft tissue mass in the right hemithorax associated with free fluid in the right hemithorax and the biopsy was performed which confirmed the diagnosis of a pleuropulmonary blastoma. The pleuropulmonary blastoma was a solid type (Type 3). Conclusion: Pleuropulmonary blastoma poses a diagnostic challenge because it does not have specific clinical or radiological features. The late diagnosis and delayed treatment of pleuropulmonary blastoma are associated with a deadly prognosis.

Mucinous cystadenoma of the appendix causing chronic intermittent right iliac fossa pain: case report

Mucinous cystadenoma of appendix (MCA) is a rare clinical entity with chronic intermittent right iliac fossa pain in the literature (1). MCA is a rare cystic neoplasm of the appendix characterized by villous adenomatous changes of the appendiceal epithelium associated with distension of the appendiceal lumen with mucin. MCA presentation is right iliac fossa pain, similar to an acute appendicitis; but, about 25% of patients are asymptomatic and diagnosed incidentally on imaging or at the time of surgery(2)

Intraductal papillary neoplasms of intrahepatic bile duct: a clinicopathologic study of 25 cases

To study the clinicopathologic and immunohistochemical features of intraductal papillary neoplasms of the bile duct (IPNB) of the intrahepatic bile duct. The clinical findings, morphologic features and immunophenotype of 25 cases of IPNB were investigated. Of the 25 cases of IPNB, 18 were in the left lobe, 6 were in the hilar bile duct, and 1 was in the right lobe. The average age was 60.6 (26-73) years. The presenting symptom was abdominal pain in 15 cases. Imaging results were variable, including multilobular cysts, mass lesions within the dilated ducts, cholangiolithiasis with dilated bile duct, and solid mass. Gross examinations were concordant with imaging findings. Microscopically, all the IPNB could be divided into four papillary patterns, including 12 gastric type, 8 intestinal type, 4 pancreatobiliary type and 1 oncocytic type. Five IPNB showed mild to moderate atypia, 10 showed high-grade atypia and 10 IPNB were associated with carcinomas.The invasive IPNB were mainly bile duct adenocarcinomas(9 cases), and rarely colloid carcinoma (1 case). Immunohistochemically, IPNB expressed MUC5AC (96.0%, 24/25), MUC2 (32.0%, 8/25), MUC6 (40.0%, 10/25) and MUC1 (12.0%, 3/25). The different MUC expression was commonly associated with specific histological subtype. IPNBs are rare cystic and premalignant neoplasms of the liver. The imaging features vary, but show a common feature of dilated bile duct. The pathologic types and classifications are similar to intraductal papillary mucinous neoplasm of the pancreas. The MUC stains are helpful in diagnosis and histological subtyping.