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.

[Papillary cystadenoma: a rare differential diagnosis of a paratesticular tumour]. / Papilläres Zystadenom als seltene Differenzialdiagnose einer paratestikulären Raumforderung.

Testicular and paratesticular cystadenomas arise from an oviduct-like structure, which, morphologically, is almost identical with the ovarian surface epithelium. These are very rare benign tumours of adults. They present as asymptomatic cystic lesions. Bilateral paratesticular cystadenomas are associated with the Von-Hippel-Lindau syndrome and may be associated with infertility. Most cystadenomas are benign, but a few cases of malignant transformation of embryonic remnants have been reported in the appendix testis, including cases of adenocarcinoma, cystadenocarcinoma, and a Müllerian-type epithelial tumour with a low malignant potential. We report the case of a 74-year-old man with a rare paratesticular cystadenoma of the male adnexa.

Autoantibodies against HSF1 and CCDC155 as Biomarkers of Early-Stage, High-Grade Serous Ovarian Cancer.

Background: Tumor-directed circulating autoantibodies (AAb) are a well-established feature of many solid tumor types, and are often observed prior to clinical disease manifestation. As such, they may provide a good indicator of early disease development. We have conducted a pilot study to identify novel AAbs as markers of early-stage HGSOCs.Methods: A rare cohort of patients with early (FIGO stage Ia-c) HGSOCs for IgG, IgA, and IgM-mediated AAb reactivity using high-content protein arrays (containing 9,184 individual proteins). AAb reactivity against selected antigens was validated by ELISA in a second, independent cohort of individual patients.Results: A total of 184 antigens were differentially detected in early-stage HGSOC patients compared with all other patient groups assessed. Among the six most highly detected "early-stage" antigens, anti-IgA AAbs against HSF1 and anti-IgG AAbs CCDC155 (KASH5; nesprin 5) were significantly elevated in patients with early-stage malignancy. Receiver operating characteristic (ROC) analysis suggested that AAbs against HSF1 provided better detection of early-stage malignancy than CA125 alone. Combined measurement of anti-HSF1, anti-CCDC155, and CA125 also improved efficacy at higher sensitivity.Conclusions: The combined measurement of anti-HSF1, anti-CCDC155, and CA125 may be useful for early-stage HGSOC detection.Impact: This is the first study to specifically identify AAbs associated with early-stage HGSOC. The presence and high frequency of specific AAbs in early-stage cancer patients warrants a larger scale examination to define their value for early disease detection at primary diagnosis and/or recurrence. Cancer Epidemiol Biomarkers Prev; 27(2); 183-92. ©2017 AACR.

Adolescent Hydrocele Carrying a Surprise: A Case of Papillary Cystadenoma of the Epididymis.

Papillary cystadenoma of the epididymis (PCE) is a rare benign epithelial tumor remarkable for its association with von Hippel-Lindau disease. A 12-year-old boy consulted for a progressive enlargement of the left testicle. At time of surgery, the whole epididymis was enlarged. Pathologic diagnosis was PCE with a focus on borderline malignancy. Scrotal left epididymectomy was performed. von Hippel-Lindau disease screening was negative. No relapse has been detected 2 years later. In case of atypical clinical examination of a hydrocele, unusual presentations such as PCE should be considered. The main differential diagnoses were adenomatoid tumor, nonpapillary cystadenoma, and metastatic clear cell renal carcinoma.

Expression of Membrane-Bound Mucins and p63 in Distinguishing Mucoepidermoid Carcinoma from Papillary Cystadenoma.

The aim of this study was to compare the immunoexpression of epithelial mucins (MUCs) in salivary duct cysts, papillary cystadenomas, and mucoepidermoid carcinomas and to evaluate if any of these markers could be useful for differentiating between mucoepidermoid carcinoma and papillary cystadenoma. We also sought to validate the p63 expression pattern found to differentiate between mucoepidermoid carcinoma and papillary cystadenoma. Immunoexpression of MUC1, MUC2, MUC4, MUC7, and p63 was studied and quantified in 22 mucoepidermoid carcinomas, 12 papillary cystadenomas, and 3 salivary duct cysts. The immunohistochemical evaluation was collectively performed by 3 oral pathologists. Scores and trends in proportions were assessed using the nonparametric Wilcoxon-Mann-Whitney rank sum test. Mucoepidermoid carcinomas, papillary cystadenomas, and salivary duct cysts demonstrated variable MUC expression patterns. All tumors were positive for p63 immunoexpression with p63 labeling in salivary duct cysts and papillary cystadenomas (15/15) limited to the basal layers of the cystic spaces, whereas in mucoepidermoid carcinomas (22/22) the p63 labeling extended throughout the suprabasal layers (p < 0.001). This study adds more confirmatory data to validate that the reactivity pattern of p63 protein can be used in distinguishing between papillary cystadenoma and low-grade mucoepidermoid carcinoma. Although positive reactivity in a tumor with MUC1 and MUC4 was inconclusive, negative reactivity suggests the diagnosis of a benign PC or SDC.

Mesonephric (Wolffian) Pseudoendometrioid Carcinoma of the Broad Ligament, Arising From a Papillary Cystadenoma.

This article describes the case of a 70-year-old woman with an adnexal cystadenocarcinoma located in the right broad ligament and displaying a striking resemblance to a well-differentiated endometrioid adenocarcinoma. The uniqueness of this pseudoendometrioid carcinoma lies in the fact that its mesonephric nature is revealed by the origin from a papillary cystadenoma of the broad ligament, where remnants of the mesonephric duct are seated, and the immunohistochemical profile, particularly a uniform negativity for estrogen and progesterone receptors, apical-luminal positivity for CD10, and strong nuclear positivity for GATA3.

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.

Serous papillary cystadenofibroma of the fallopian tube: A case report and short review of literature.

Serous papillary cystadenofibromas (SPCAFs) of the fallopian tube are very rare benign tumors of the female genital tract. They are usually asymptomatic and are found incidentally. Until now, only 18 cases of this tumor have been reported in the world literature. We report a case of SPCAF of the left fallopian tube in a 30-year-old female who presented with a large abdominal mass and pain. On computed tomography, a diagnosis of ovarian neoplasm was given. However, during surgery the tumor was found to arise from the fallopian tube and was treated with tubal cystectomy with sparing of the ovary. We present this unique case on account of its rarity, unusual presentation, and huge size along with a short review of literature.

Squamous cell carcinoma of the larynx arising in multifocal pharyngolaryngeal oncocytic papillary cystadenoma: a case report and review of the literature.

We report on a rare case of a laryngeal carcinoma arising in a multifocal pharyngolaryngeal oncocytic papillary cystadenoma (OPC). The disease of a 63-year-old man is well documented by computed and positron emission tomography, histology, and electron microscopy. We could show that an OPC can even develop in the pharynx. The coexistence of both tumors makes this a challenging diagnosis for pathologists. Treated by surgery and radiotherapy, both lesions dissolved. Based on the literature available, we discuss the theory that the laryngeal carcinoma might be the result of a true metaplasia facilitated by chronic irritation and recommend a regular follow-up for OPC too. As in benign oncocytic lesions, we could show that the detection of numerous mitochondria is a diagnostic indicator for malignant variants as well.

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.

Papillary cystadenoma of the lower lip exhibiting ciliated pseudostratified columnar epithelium: report of a bizarre case and review of the literature.

BACKGROUND: Salivary gland tumors are uncommon and constitute 2-6.5 % of all head and neck neoplasms. Tumors of minor salivary gland origin account for less than 25 % of all salivary gland neoplasms. Papillary cystadenoma of salivary glands is a rare benign epithelial neoplasm characterized by multicystic growth in which the epithelium exhibits adenomatous proliferation. Papillary cystadenoma of minor salivary glands most frequently involves the lip, buccal mucosa, and palate. This tumor typically presents as a slow-growing, painless mass, usually with diameter of less than 1 cm and clinical resemblance to a mucocele. Although most papillary cystadenomas are predominantly of one cell type, a regional variability may be present. CASE REPORT: We present a case of papillary cystadenoma of the minor salivary glands in a 58-year-old patient exhibiting an upper respiratory tract epithelium, a profoundly atypical benign tumor. DISCUSSION: This type of minor salivary gland tumor epithelium in the lower lip may be the result of a metaplastic process or simply another neoplastic manifestation of papillary cystadenoma. As far as the differential diagnosis of this entity is concerned, it is important to distinguish it from papillary cystadenoma lymphomatosum (Warthin's tumor), low-grade mucoepidermoid carcinoma, the papillary-cystic variant of acinic cell carcinoma, and cystadenocarcinoma Recognition of this lesion is important for the clinician since the differential diagnosis includes lesions with similar clinical appearance and infiltrative behavior.

Solid pseudopapillary tumor of the pancreas: a single-institution 20-year series of pediatric patients.

PURPOSE: Solid pseudopapillary tumor (SPT) of the pancreas is a rare neoplasm. The objective of this study was to review our institution's experience and provide an update on current management in the pediatric population. METHODS: Our pathology database identified all patients with SPT for a 20-year period (1991-2011). Demographics, clinical characteristics, operative details, pathology, and outcomes data were retrospectively reviewed. RESULTS: Eleven patients with SPT were identified. Most were female and Hispanic. Median age at resection was 14 years (9-17 years). Most patients presented with abdominal pain. Diagnostic imaging was most commonly an ultrasound or computed tomography. All tumors were resected en bloc. Median greatest tumor diameter was 5 cm (3.5-12 cm). Median length of stay was 8 days (5-19 days). Complications included pancreatic leak, chyle leak, delayed gastric emptying, fat malabsorption, and incisional keloid. Recurrence developed after 2.5 years in 1 patient with positive surgical margins. There were no metastases or deaths. Median follow-up was 1.4 years (0.6-5.9 years). CONCLUSION: This pediatric series of SPT from a single institution corroborates previous reports in the literature. In our experience, SPT behaves like a low-grade malignancy and has an excellent prognosis. Surgical resection is dictated by tumor location and remains the treatment of choice.

[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.

Proper management and follow-up strategy of branch duct intraductal papillary mucinous neoplasms of the pancreas.

BACKGROUND AND AIM: It has been reported that main duct intraductal papillary mucinous neoplasms are more invasive and have a worse prognosis than branch duct intraductal papillary mucinous neoplasms. Therefore, an aggressive surgical approach has mainly been recommended for all MD-IPMNs. However, the surgical management of BD-IPMNs has been controversial and the consensus guidelines are not specific for an indicator of malignancy in BD-IPMNs. The objective of this study was to determine the proper management and follow-up strategy of BD-IPMNs. METHODS: We monitored and analysed patients with presumed BD-IPMNs between March 1995 and March 2010. RESULT: The mean value of the initial cyst size in all patients with BD-IPMNs was 2.19 cm. Amongst 194 patients with BD-IPMNs, 34 underwent immediate surgical resection, 152 were followed conservatively. Amongst the 152 conservatively managed patients, 18 (11.8%) underwent surgical resection after a median follow-up of 12.7 months (range, 3-48 months). In 132 patients who were managed conservatively without surgery, the mean incremental rate of cyst size growth was 0.0038 cm/month during a median of 30.7 months of follow-up and there were no IPMN-related deaths. CONCLUSION: Amongst patients with BD-IPMNs, about 10% have surgery within approximately 1 year from the time of diagnosis because of the occurrence of new malignant stigmata. Therefore, a conservative approach without surgery and careful follow-up every 3 months or 6 months during the first year after diagnosis can be safely advocated in patients with BD-IPMNs larger than 10mm in size who have no risk factors for malignant IPMNs.

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.

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.