Clear Cell Papillary Cystadenoma of the Ovary Masquerading as Metastatic Clear Cell Renal Cell Carcinoma: A Case Report and Review of the Literature.

Clear cell papillary cystadenoma of the epididymis is an uncommon benign neoplasm, usually seen in patients with von Hippel-Lindau disease. Morphologic and immunohistochemical examination aid in distinguishing clear cell papillary cystadenoma from malignant histologic mimics including low-grade mesothelial proliferations and metastatic clear cell renal cell carcinomas. Analogous lesions have been described in the female genital tract, often posing diagnostic challenges due to their low incidence. Here, we present the difficult diagnostic aspects of the first case of clear cell papillary cystadenoma involving the ovary, including the salient immunohistochemical, ultrastructural, and molecular characteristics.

Oncocytic papillary cystadenoma of the larynx: comparative study of ten cases and review of the literature.

OBJECTIVE: Oncocytic papillary cystadenomas (OPCs) of the larynx are rare benign cystic lesions that usually present as supraglottic masses arising from the laryngeal ventricles. OPCs are found in patients older than 60 years, with a female predominance. Symptoms vary from asymptomatic to hoarseness, dyspnea, and dysphagia; often, they mimic a laryngocele. The treatment is surgical. Diagnosis is based on histopathologic examination. MATERIALS AND METHODS: Surgical records for laryngeal masses diagnosed between 2005 and 2020 were searched retrospectively. RESULTS: Ten patients were identified and included in the study. OPCs predominantly occurred in women (9/10), and the mean age at presentation was 73 years. Most patients (8/10) presented with hoarseness and were smokers. OPCs were localized in the ventricle in eight out of ten patients. Surgical treatment was performed in all cases, mostly using transoral endolaryngeal approach (9/10). Histopathologic examination revealed oncocytic cyst or oncocytic papillary cystadenoma (the former term being the older synonym for OPC). CONCLUSION: OPCs present a separate clinicopathologic entity, distinct from other cystic laryngeal lesions. They have a characteristic location, age and sex group, microscopic appearance, and potential for local recurrence.

Linear syringocystadenoma papilliferum on the retroauricular area associated with nevus sebaceus.

Syringocystadenoma papilliferum is a rare cutaneous adnexal tumor that usually arises in the head and neck region. It may develop de novo or within a nevus sebaceus. Linear syringocystadenoma papilliferum is an uncommon variant of this benign tumor. We report a child with linear retroauricular distribution of syringocystadenoma papilliferum. A background nevus sebaceus was shown histologically. Total excision was curative with no recurrence. An association between the linear variant of syringocystadenoma papilliferum and nevus sebaceus has not been reported previously.

[Papillary cystadenoma of the epididymis: a report of 2 cases and review of the literature].

OBJECTIVE: To study the clinicopathological characteristics of papillary cystadenoma of the epididymis. METHODS: Using routine pathology and immunohistochemistry, we observed the surgically obtained samples from 2 cases of papillary cystadenoma of the epididymis, analyzed their pathological features and clinical presentations, and reviewed the related literature. RESULTS: The 2 patients were both adult males. The tumors typically manifested as painless swelling in the epididymis, with occasionally dull pain and tenesmus in 1 of the cases. Pathologically, the lesions exhibited three morphological features, i. e., dilated ducts and small cysts surrounded by fibrous connective tissue, adenoid papillary hyperplasia into the cysts embraced by fibrovascular stroma, and acidophil substance present in the cysts. Immunohistochemistry showed that the tumors were strongly positive for CK8/18, CK7, and EMA, but negative for CK20, CEA, MC, Calretenin, P53, P63, SMA, VHL, and CD10, with the positive rate of Ki-67 <1%. Follow-up visits revealed good prognosis in both cases. CONCLUSION: Papillary cystadenoma of the epididymis is a rare benign tumor in the male urogenital system, which may be accompanied by the VHL syndrome. Surgery is the first choice for its treatment.

CT and MR imaging of multilocular acinar cell cystadenoma: comparison with branch duct intraductal papillary mucinous neoplasia (IPMNs).

OBJECTIVES: To describe CT and MR imaging findings of acinar cell cystadenoma (ACC) of the pancreas and to compare them with those of branch duct intraductal papillary mucinous neoplasia (BD-IPMN) to identify distinctive elements. METHODS: Five patients with ACC and the 20 consecutive patients with histologically proven BD-IPMN were retrospectively included. Clinical and biological information was collected and histological data reviewed. CT and MR findings were analysed blinded to pathological diagnosis in order to identify imaging diagnostic criteria of ACC. RESULTS: Patients with ACC were symptomatic in all but one case and were younger than those with BD-IPMN (p = 0.006). Four radiological criteria allowed for differentiating ACC from IPMN: five or more cysts, clustered peripheral small cysts, presence of cyst calcifications and absence of communication with the main pancreatic duct (p < 0.05). Presence of at least two or three of these imaging criteria had a strong diagnostic value for ACC with a sensitivity of 100% and 80% and a specificity of 85% and 100%, respectively. CONCLUSIONS: Preoperative differential diagnosis between ACC and BD-IPMN can be achieved using a combination of four CT and/or MR imaging criteria. Recognition of ACC patients could change patient management and lead to more conservative treatment. KEY POINTS: Four imaging findings are associated with acinar cell cystadenoma (ACC). Imaging could achieve differential diagnosis between ACC and BD-IPMN. Diagnosis on imaging would change patient management and avoid surgical resection.

Oncocytic papillary cystadenoma of the larynx: a case report.

BACKGROUND: Cystadenoma of the salivary glands is a rare benign clinical condition affecting both major and minor salivary glands equally. It constitutes approximately 2% of total neoplasms and 4.2-4.7% of benign formations in minor salivary glands. Typically presenting as a slow-growing, painless neoplasm, it can be distinguished from Cystadenolymphoma (Whartin's Tumor) by the absence of lymphoid elements in histological examination. While mostly located in the oral cavity and oropharynx, it can also be found in sinonasal mucosa, and rare cases have been identified in the larynx. CASE PRESENTATION: A 75-year-old Caucasian woman presented to the ear, nose, and throat department with complaints of dysphonia and headaches persisting for several months. Dysphonia had developed months after an unspecified vocal cord surgery elsewhere. Flexible laryngoscopy identified a left-sided cystic swelling affecting the supraglottic space, leading to respiratory obstruction and dysphonia. Head and neck computed tomography confirmed a 1.9 × 1.7 cm bilobed cystic mass originating from the left Morgagni ventricle. Microlaryngoscopy with CO2 laser excision and biopsy revealed a histopathological diagnosis of oncocytic papillary cystadenoma. Post-surgery, the patient fully recovered from dysphonia, with no significant complications noted. Long-term clinical surveillance was advised to detect potential recurrences promptly. CONCLUSION: Ectopic minor salivary gland tumors, both benign and malignant, should be taken into consideration as potential differential diagnosis for any swelling arising within the upper digestive tract mucosa. Ears, nose, and throat clinical examination completed by videolaryngoscopy can easily point out the location of the mass. Imaging is mandatory for differential diagnosis and for surgical planning. Surgical excision can provide both diagnosis and definitive cure.

[Ultrasound-guided biopsy of the testis: indications and results]. / Biopsie testicolari sotto guida ecografica: indicazioni e risultati.

Ultrasound examination of the testis is the imaging modality of choice for the evaluation of intratesticular focal lesions. In spite of its high sensibility, eco-Doppler-elastography is lacking of specificity in discrimination between benign and malign lesions, not always allowing us to make a definitive diagnosis of malignancy. When a diagnostic doubt persists, for such lesions that are indeterminate at clinical and radiological evaluation, it is possible to recur to ultrasound-guided testicular needle biopsy. This paper describes the main application scenarios of testicular fine-needle aspiration under ultrasound guidance and the experience in our institute.

Papillary cystadenoma of the major salivary gland: A case report and review of literature.

Papillary cystadenoma (PC) of the salivary gland is an uncommon benign epithelial neoplasm that shows predominantly multicystic growth pattern with intraluminal papillary proliferation and areas of oncocytic differentiation. We report a case of papillary cystadenoma of the parotid gland in a 44-years-old female. The patient presented with painful nodular swelling in the right parotid region for two months. Ultrasonography revealed a well marginated oval lesion with altered signal intensity involving the superficial lobe. The excision specimen showed a neoplasm with multicystic spaces having papillary projections lined by benign low-grade epithelium and supported by fibrovascular cores. No significant cytological atypia or mitosis was observed. The cells were immunoreactive for Keratin, Keratin 7, and were negative for Keratin 20, AR, HeR2/neu, TTF1, CDX2, and GATA3. p63 and Keratin 5/6 highlighted the myoepithelial cell layer lining the cystic spaces as well as the papillary projections. The Ki-67 proliferation index was 6%. The patient is on close clinical and imaging follow-up for the last 1year and 8 months without any evidence of disease recurrence or metastasis. Rarity of the lesion and distinct histomorphology warrants appropriate knowledge and discussion of the subject.

An analysis of five clear cell papillary cystadenomas of mesosalpinx and broad ligament: four associated with von Hippel-Lindau disease and one aggressive sporadic type.

AIMS: Clear cell papillary cystadenoma (CCPC) is associated with von Hippel-Lindau disease (VHLD), but rarely involves mesosalpinx and broad ligament (M/BL). This study provides new data about its behaviour and immunophenotype. METHODS AND RESULTS: We performed an analysis of four benign cases of CCPC of M/BL with either characteristic clinical features or genetic markers [loss of heterozygosity (LOH)] of VHLD in patients ranging from 24 to 36 years and a sporadic case in a 52-year-old presenting with peritoneal metastases. All CCPCs were papillary but had solid and tubular areas. Haemorrhage, thrombosis and scarring were constant features and related to an unusual pattern of sub-epithelial vascularity. All clear or oxyphilic cells co-expressed cytokeratin 7 (CK7), CAM5.2 and vimentin, with strong apical CD10 and nuclear paired box gene 2 (PAX2) immunoreactivity. Three cases also showed positivity for VHL40, epithelial membrane antigen (EMA), Wilms' tumour suppressor gene (WT-1) and cancer antigen 125 (CA125) but only one expressed renal cell carcinoma (RCC) antigen. Vascular plexus overexpressed nuclear and cytoplasmic WT-1. CONCLUSION: The VHLD-associated cases appeared to be benign, but the sporadic case exhibited a low malignant potential. CCPCs show histological and immunophenotypical similarities with the recently reported clear cell papillary RCC, although the previously unreported apical CD10 and nuclear PAX2 expression may be related to their mesonephric origin. CCPC has a distinctive sub-epithelial vascular pattern that is consistent with its pathogenesis.

A detailed immunohistochemical analysis of 2 cases of papillary cystadenoma of the broad ligament: an extremely rare neoplasm characteristic of patients with von hippel-lindau disease.

We report 2 cases of papillary cystadenoma, a rare neoplasm characteristic of patients with Von Hippel-Lindau (VHL) disease, involving the pelvic soft tissues of women and probably arising within the broad ligament. In only one of the women was there a history of VHL disease. The other woman was investigated for VHL disease after the diagnosis of papillary cystadenoma and all tests were negative. There has been debate as to whether papillary cystadenomas in women are of mesonephric (Wolffian) or of Mullerian origin and to investigate this we undertook a detailed immunohistochemical analysis. Both tumors were positive with AE1/3, Ber EP4, epithelial membrane antigen, CK7, CD10, CA125, CA19.9, calretinin, and vimentin. One exhibited focal nuclear staining with WT1 and PAX8. The tumors were negative with estrogen receptor, progesterone receptor, androgen receptor, CK20, CEA, TTF1, inhibin, RCC marker, and hepatocyte nuclear factor 1ß. Although favoring a mesonephric origin, the immunohistochemical findings are essentially inconclusive and not definitive for either a mesonephric or a Mullerian origin. We believe that patients found to have papillary cystadenoma should be investigated for VHL disease if there is no history of this. This is the second reported example of papillary cystadenoma in a woman not known to have VHL disease and the first in which investigations have excluded this disease.

Role of laparoscopic distal pancreatectomy for solid pseudopapillary tumor.

BACKGROUND: Since the first case report regarding laparoscopic distal pancreatectomy (DP) for solid pseudopapillary tumor (SPT), few additional articles have been published. The objective of this study was to evaluate the feasibility, safety, and long-term outcome of the laparoscopic DP based on a series of adult SPT patients. METHODS: In a single-center study, we screened all adult patients undergoing a laparoscopic DP for SPT. Preoperative, operative, and postoperative data were retrospectively analysed and compared to the results of open DP for SPT published in the medical literature. RESULTS: From April 2000 to June 2010, 5 adult female patients (median age 34 y) underwent a laparoscopic DP for an SPT. No conversion to open surgery was required. The median size of the tumor was 45 mm. The postoperative mortality rate was 0%, and serious complications (Dindo IV) occurred in 2 patients. The postoperative quality of life was not significantly altered by the laparoscopic procedure. At a median follow-up of 60 mo, all patients were alive and without evidence of local recurrence, distant metastasis, diabetes, or exocrine insufficiency. CONCLUSION: Laparoscopy may offer an alternative to open surgery in the treatment of SPT of the distal pancreas in adult female patients. The laparoscopic procedure impacts neither the oncologic outcome nor the quality of life. However, due to the risk of postoperative complications, this procedure should be reserved for specialized centers.

[A case of papillary cystadenoma of the epididymis mimicking a testicular tumor].

A 68-year-old male presented with painless left scrotal enlargement of one year duration. Ultrasound, computed tomography (CT) and magnetic resonance imaging showed a multilocular cystic mass, 10×7.5× 8.5 cm in size, in the left scrotum. The intracystic fluid was partially hemorrhagic. A solid part of the tumor, seen at the base of the scrotum, was partially calcified and was enhanced by contrast medium. The left testis could not be identified by imaging studies. Although CT imaging showed a simple cyst in the right kidney, no other lesions in the kidneys, adrenal glands, pancreas or the central nervous system were detected. Serum tumor marker values for testicular cancer were within the normal range. Under the pre-operative diagnosis of a left testicular tumor, left high orchiectomy was performed. Grossly the specimen consisted of a multilocular cystic tumor, 12.5×8.5×8.5 cm in size, with a 2.7 cm tan-colored solid component within the wall of the cyst. The left testis was atrophic, 1.3 cm in size, and demonstated no continuity with the solid part of the tumor. Histologically, the solid component of the tumor showed tubular and papillary growth of cuboidal and columnar tumor cells with clear cytoplasm. Histopathological diagnosis of papillary cystadenoma of the epididymis (PCE) was made. Von Hippel-Lidau disease was ruled out by subsequent genetic analysis. After follow up for 18 months, there was no sign of recurrence. To our knowledge, this is the 33rd and the largest case of PCE reported in Japan.

Laparoscopic pancreatectomy for solid pseudo-papillary tumors of the pancreas is a suitable technique; our experience with long-term follow-up and review of the literature.

BACKGROUND: Solid pseudopapillary tumors (SPTs) are rare pancreatic neoplasms of low malignant potential that occur mainly in young women. Only 17 cases of SPT treated laparoscopically have been published in the literature and long-term follow-up data are still lacking. METHODS: Retrospective analysis of ten patients (8 women, 2 men; mean age, 25.4 years) (DS: 12.1; minimum 11, maximum 51) who underwent laparoscopic distal pancreatectomy with a definitive histological diagnosis of SPT. Long-term follow-up data were collected. RESULTS: The average tumor size was 43.8 mm (minimum 20, maximum 65 mm). The mean operative time was 177.5 minutes (DS: 53.7; minimum 120, maximum 255). In all, five patients underwent distal splenopancreatectomy; five patients underwent spleen-preserving distal pancreatectomy of whom three with splenic vessel preservation and two with the Warshaw technique. The conversion rate was nil and no case of perioperative mortality was recorded. The mean hospital stay was 7 days (DS: 2.7; minimum 4, maximum 12). Six patients had an uneventful postoperative course and four had postoperative complications. Two of them underwent reoperation, and the other two had nonsurgical complications. After a median follow-up of 47 (range, 5-98) months, all patients were alive and disease-free. CONCLUSIONS: Laparoscopic pancreatic resection is a safe and feasible procedure that could become the treatment of choice for patients affected by pancreatic SPT. Distal pancreatectomy should be performed, if possible, with spleen-preserving technique, especially in young patients. To avoid metastatic spread, laparoscopic or laparotomic biopsy should not be performed in patients affected by SPT.

Intraductal papillary neoplasm arising from peribiliary glands connecting with the inferior branch of the bile duct of the anterior segment of the liver.

Intraductal papillary neoplasms of the bile duct are generally thought to arise from neoplastic papillary proliferation of epithelial cells lining the bile duct. We herein report a case with findings that strongly suggested that the biliary cystic tumor might have derived from a peribiliary gland. A 69-year-old female was found to have a cystic lesion with intracystic protrusions at the anterior segment of the right hepatic lobe and underwent hepatic anterior segment resection. Fluoroscopy of the resected specimen injected with contrast medium into the cyst revealed a connection between the cystic lesion and the bile ducts. The cyst was multilocular in appearance. On microscopic examination, the cyst was located within the portal tract of the inferior branch of the anterior segment and connected with the inferior branch of the bile duct. The wall of the hepatic cyst lacked an ovarian-like stroma. The tumor was composed of papillary and glandular components, and the tumor cells were similar to gastric foveolar and pyloric gland epithelia and regarded as adenoma. These tumor cells were positive for MUC 5AC, MUC6, and HIK1083. The tumor was finally diagnosed as an intraductal papillary neoplasm of the bile duct (adenoma, gastric type) arising from a peribiliary gland.

[Solid pseudopapillary pancreatic neoplasm: report of five cases]. / Neoplasia sólida seudopapilar del páncreas: comunicación de 5 casos.

Solid pseudopapillary pancreatic neoplasms (SPPN) are rare entities mainly diagnosed in young women. Prognosis is generally relatively favorable. Histological evaluation usually reveals papillary as well as solid areas of low malignant potential, as the name implies. A retrospective review of five cases, diagnosed and treated in our institution, is presented with the corresponding clinical and pathological data and follow-up. All SPPN occurred in women, with an average age of 19 years (13 to 27 years), who consulted for abdominal pain. In all patients, the tumors were > 4cm, distributed in several pancreatic segments. The histopathologic report confirmed papillary as well solid areas and low mitotic count. In two patients, neural and angioinvasion were found.

Pancreatic true cysts--diagnosis and treatment difficulties.

Pancreatic true cysts represent a rare, heterogeneous group of pancreatic tumors; therapeutic strategy is based on patient's general status, cyst topography, and especially the estimated risk of malignancy. This paper aim is to present 7 cases of pancreatic true cysts, operated on a six years period (January 2004-January 2010) in our surgical clinic: 2 men and 5 women, aged between 24-61 years old; cyst diameter varies between 3.5-15 cm, tumor location being pancreatic head in two cases and the distal pancreas in 5 cases. Surgical treatment consisted in cyst enucleation (two cases), splenopancreatectomy (three cases), duodenopancreatectomy (one case), and subtotal splenopancreatectomy (one case). Histology was represented by serous cystadenoma (one case), mucinous cystadenoma (2 cases), intraductal papillary mucinous cystadenoma (one case), and papillary cystadenocarcinoma (3 cases).Postoperative results were good in all cases, with 3 postoperative pancreatic external fistulas, resolved conservatory; no case of post-pancreatectomy diabetes mellitus was registered. In conclusion, surgical removal of the pancreatic cystic tumors is necessary, especially due to the risk of malignancy, at least in the absence of rigorous histological proofs of benignancy. Postoperative results are favorable in terms of postoperative morbidity and mortality.

Effect of cigarette smoking on epithelial ovarian cancer survival.

OBJECTIVE: To determine associations between smoking and survival in patients with ovarian cancer. STUDY DESIGN: We performed a retrospective review of patients undergoing surgery for ovarian cancer. Abstracted patient data included age, smoking history, stage, grade, extent of cytoreduction, and survival. Data were examined with Fisher's exact test, Kaplan-Meier survival analysis, and Cox regression analysis. RESULTS: A total of 130 patients met criteria for review. Seventeen (13%) smoked cigarettes at the time of initial laparotomy. Twenty-one (16%) were left with > 1 cm residual disease. Smoking did not correlate with the incidence of suboptimal cytoreduction. There were no statistical differences in incidence of hypertension, obesity, or coronary artery disease in smokers compared to nonsmokers. Smoking was found to negatively influence length of progression-free survival. Similarly, smokers were found to have decreased disease-specific overall survival compared to nonsmokers. Cigarette smoking retained independent significance as poor prognostic factors, after controlling for age, stage, and grade. CONCLUSION: These findings identify a negative correlation with cigarette smoking and survival in women with ovarian cancer. Further studies are proposed to elucidate the molecular mechanisms underlying these clinical observations.

Bilateral papillary cystadenoma of the mesosalpinx: a rare manifestation of Von Hippel-Lindau disease.

We report a rare case of a woman with bilateral papillary cystadenomata of the broad ligament with von Hippel-Lindau disease (VHL) (other manifestations: capillary hemangioblastomas of the spinal cord). Patient surveillance is important, because in the course of VHL-associated tumors malignant lesions may arise that are relevant for the prognosis.

Synchronous Oncocytic Papillary Cystadenoma and Warthin Tumor of the Parotid Gland.

Warthin tumor (WT) is the second most common benign salivary gland neoplasm. It is also the most frequent salivary gland tumor to occur synchronously or metachronously with another salivary gland neoplasm, in the same gland or on the other side. Oncocytic papillary cystadenomas (OPCs) are rare neoplasms that are more common in minor salivary gland locations and in women. We describe in detail the case of a 73-year-old male smoker with synchronous OPC and WT of the parotid gland. On microscopy, both tumors resembled each other considerably, with the only difference being that the OPC lacked the tumor-associated lymphoid proliferation characteristic of WT. These findings highlight that OPC bears considerable similarity to WT. While this morphological similarity may lead to misdiagnosis on rare occasions, it does not affect patient management, as clinical behavior of both these tumors is similar.