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.

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.

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.

[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.

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.

Papillary oncocytic cystadenoma of the parotid glands: a report of 2 cases with varied cytologic features.

BACKGROUND: Papillary cystadenoma is a rare salivary gland neoplasm, and oncocytic change can be focal or marked. Papillary oncocytic cystadenoma has been reported mainly in the minor salivary glands and occasionally in the parotid glands. The cytologic features vary. CASES: A 26-year-old female presented with a 2.4-cm, cystic right parotid gland mass present for 4 months. Fine needle aspiration (FNA) showed cellular smears with sheets of oncocytic cells and some with micropapillary architecture. The diagnosis ofa neoplasm was rendered and excisional biopsy recommended. Oncocytic neoplasm was diagnosed during intraoperative consultation, and final surgical histology showed a unilocular, cystic, oncocytic neoplasm with variable papillary projections. A 52-year-old male presented with a 1.5-cm, cystic left parotid mass enlarging for the past few months. FNA showed less cellular smears with extensive necrotic debris and a few large sheets of epithelial cells with vague papillary architecture. Oncocytic neoplasm was diagnosed during intraoperative consultation, and final surgical histology showed a unilocular cystic lesion with multiple papillary fronds lined with oncocytic cells and focal metaplastic squamous cells. CONCLUSION: Papillary cystadenoma is rare in the parotid glands, and cytologic features may vary. Warthin's tumor, oncocytoma, intraductal papilloma and acinic cell carcinoma may arise in the differential diagnosis. In cases with extensive necrotic debris and metaplastic squamous cells, branchial cyst and cystic metastatic squamous carcinoma may also need to be considered.

A diagnostic pitfall of fine needle aspiration cytology in testicular papillary serous cyst adenoma: a case report.

BACKGROUND: Testicular neoplasmns resembling ovarian serous tumors are rarely reported. We describe the first fine needle aspiration biopsy (FNAB) finding of this type of tumor. CASE: A 35-year-old man presented with an enlarged right testis. Based on clinical observation, we hypothesized that it was a malignant tumor. FNA cytology findings revealed numerous papillary clusters, glandular-like structures and many isolated round cells. Metastatic papillary adenocarcinoma was suspected based on FNAB cytology. A right radical orchiectomy was performed, and histologic tissue examination was used to diagnose the tumor as a benign papillary serous cystadenoma. CONCLUSION: Papillary serous cystadenoma of the testis is a rare tumor. The cytologic findings of this tumor are confusing and difficult to diagnose. Clusters of epithelial cells with a high nuclear to cytoplasm ratio are mistaken for features of a malignant tumor.

Is laparoscopic management suitable for solid pseudo-papillary tumors of the pancreas?

PURPOSE: Solid pseudo-papillary tumors (SPT) are rare pancreatic neoplasms of low-malignant potential occurring mainly in young women. The purpose of this report is to describe our experience with laparoscopic management of these tumors with 4-year follow-up. METHODS: Three children with SPT were admitted to two hospitals in Paris, France, between February 2000 and December 2006. Diagnosis or treatment was carried out using laparoscopic techniques (biopsy and resection in one case and biopsy only in two). Long-term follow-up data were collected. RESULTS: All three patients presented recurrences within 3 years after resection, i.e., disseminated peritoneal recurrence in two patients and local recurrence in one. The two patients with peritoneal recurrences were treated by surgical resection and chemotherapy. The patient with local recurrence could not be treated due to contraindicating local factors. All three patients were alive at the time of this writing. CONCLUSION: This is the first report describing long-term follow-up after laparoscopic management of SPT. All three patients developed recurrences. These poor results contrast sharply with the low risk of local or disseminated recurrence after open laparotomy without chemotherapy that has been considered as the treatment of choice up to now. Recurrences after laparoscopic management may have been due to diffusion of tumor cells caused by gas insufflation especially during biopsy. Laparoscopic biopsy should not be performed in patients presenting SPT.