Cystadenoma of the Rete Testis in a Patient With von Hippel-Lindau Disease.

von Hippel-Lindau disease is a rare, inherited multicystic disorder that is characterized by several benign and malignant neoplasms (Odrzywolski, 2010). Classically, the disease manifests itself in a broad spectrum, including renal cell carcinomas, intracranial and spinal hemangioblastomas, endolymphatic sac tumors, renal and pancreatic cysts, and pheochromocytomas. Another important, but commonly forgotten manifestation is the cystadenoma of the rete testis.

[Miniinvasive interventions for focal liquid accumulations in region of pancreas and surrounding tissues].

The results of treatment of 264 patients, suffering an acute and chronic pancreatitis, are analyzed. In 30% of observations the liquid peripancreatic accumulations observed have had regressed under the influence of complex conservative therapy and in other--there were performed transcutaneous punctures under ultrasonographic guidance, the cysts drainage, using laparoscopic method. The recurrence free postoperative period had lasted not less than 3 months.

Laparoscopic heminephrectomy and resection of ectopic ureter in an adult.

We report a unique case of a duplex kidney and ectopic ureter presenting as a cystic tumor of the seminal vesicle with consecutive dysuria in an adult. The medical history of the patient, the diagnostic pathway and the laparoscopic treatment as well as the key points in laparoscopic surgery are described.

[Von Hippel-Lindau disease associated with renal cell carcinoma and bilateral cystadenoma of the epididymis: a case report].

We present a case report of von Hippel-Lindau disease associated with renal cell carcinoma and bilateral cystadenoma of the epididymis. A 26-year-old man appeared with painless tumors of the bilateral scrotal contents. Ultrasonography and other radiographic examinations including computed tomographic scan and dripinfusion pyelography showed multiocular tumors in the bilateral epididymis and a right renal tumor 3 cm in diameter. The tumors of the bilateral epididymis were surgically resected and of the right renal tumor enucleated. Histopathological examination revealed cystadenoma of the epididymis and renal cell carcinoma (clear cell carcinoma, G1, pT1a). He has not received adjuvant therapy, and is doing well with no evidence of metastatic disease 2 years after surgery.

Tsc2(+/-) mice develop tumors in multiple sites that express gelsolin and are influenced by genetic background.

Tuberous sclerosis (TSC) is an autosomal dominant genetic disorder in which benign hamartomas develop in multiple organs, caused by mutations in either TSC1 or TSC2. We developed a murine model of Tsc2 disease using a gene targeting approach. Tsc2-null embryos die at embryonic days 9.5-12.5 from hepatic hypoplasia. Tsc2 heterozygotes display 100% incidence of multiple bilateral renal cystadenomas, 50% incidence of liver hemangiomas, and 32% incidence of lung adenomas by 15 months of age. Progression to renal carcinoma, fatal bleeding from the liver hemangiomas, and extremity angiosarcomas all occur at a rate of less than 10%. The renal cystadenomas develop from intercalated cells of the cortical collecting duct and uniformly express gelsolin at high levels, enabling detection of early neoplastic lesions. The tumor expression pattern of the mice is influenced by genetic background, with fewer large renal cystadenomas in the outbred Black Swiss background and more angiosarcomas in 129/SvJae chimeric mice. The slow growth of the tumors in the heterozygote mice matches the limited growth potential of the great majority of TSC hamartomas, and the influence of genetic background on phenotype correlates with the marked variability in expression of TSC seen in patients.

No evidence for microsatellite instability from allelotype analysis of benign and low malignant potential ovarian neoplasms.

UNLABELLED: The genetic events that lead to the development of benign and low malignant potential (LMP) tumors from normal ovarian surface epithelium are not well understood. In contrast to invasive ovarian neoplasms, loss of heterozygosity (LOH) is not common in these tumors except on the X chromosome, but one report has suggested that an alternative genetic mechanism, microsatellite instability (MSI), might be an important pathogenic mechanism for LMP ovarian tumors. OBJECTIVE: To determine the frequency of MSI in LMP tumors and to detect novel regions of LOH in benign and LMP ovarian tumors. METHODS: Sixty-nine microsatellite markers were analyzed in 23 benign and 31 LMP ovarian tumors. RESULTS: No evidence of MSI was found in any of the tumors studied, nor were any novel regions of LOH identified. CONCLUSIONS: This suggests that new approaches may be necessary to understand the genetic basis of benign and LMP ovarian neoplasms since neither LOH nor MSI appears to play a major role.

[Developing cancer from a probable misdiagnosed pancreatic cystadenoma]. / Cancérisation d'un probable cystadénome pancréatique méconnu.

This paper reports the malignant transformation of a benign pancreatic cyst treated by internal drainage in a 30-year-old woman. The initial lesion was diagnosed on surgical biopsy of the cyst wall. The diagnosis of pancreatic carcinoma was made after one year of satisfactory course, by fine needle cytologic aspiration of the fibrous retraction of the cyst; at that time the patient had severe pancreatic pain with multinodular liver on ultrasonography. The conclusion is the recommendation of total excision of pancreatic cysts whenever the diagnosis is incomplete or imprecise.

[Hemoperitoneum from post-traumatic ovarian cystoma. Videolaparoscopic treatment]. / Emoperitoneo da cistoma ovarico post-traumatico. Trattamento videolaparoscopico.

Personal experience in a case of ovarian cystoma (misunderstood to the patient herself) broken consequently to a car trauma and cause of an intracystic angiorrhagy and hemoperitoneum is presented. The advantages of videolaparoscopic technique both for the diagnosis formulation and for the surgical treatment are underlined and the use of this new technique is suggested due to its advantages such as quickness, mini-invasiveness and post-operation comfort.

The spectrum of serous cystadenoma of the pancreas. Clinical, pathologic, and surgical aspects.

Serous cystadenoma of the pancreas is a rare lesion thought to be almost invariably benign. Since 1978, 211 cases have been reported in the literature. Some have been recognized by computed tomography (CT) when small and asymptomatic. The authors have reviewed their experience with 40 patients (median follow-up of 1.9 years, maximum of 22.2 years) from 1936 to 1991. One third (13) were asymptomatic, of whom eight (20%) were discovered intraoperatively. Of those 20 who had CT, an unequivocal preoperative diagnosis was reached in none. Needle biopsy proved accurate in two patients. Endoscopic retrograde cholangiopancreatography (ERCP) and biopsy were performed with diagnostic success on one occasion. Three patients presented acutely. The tumor was resected in 90%, with an operative mortality rate of 10%. Enucleation of the tumor without formal anatomic pancreatectomy necessitated reoperation for complications in four of eight patients. Survival after successful resection paralleled expected survival. Serous cystadenoma may be associated with von Hippel-Lindau syndrome. The current role for conservative management remains questionable because of our current inability to reliably differentiate many of these benign neoplasms from malignant cystic neoplasms of the pancreas.

Virus-induced pancreatic cancer in guinea fowl. An electron-microscopical study.

Pancreatic tumors were induced in guinea fowls inoculated with virus strain Pts-56. Sequential high-resolution light microscopic and ultrastructural studies revealed consecutive occurrence of alterations in the pancreas of the infected birds. From the second month p.i., there were nonobligatory, unspecific focal degenerative changes in acinar units that were replaced by tubular complexes, lined with centroacinar-like cells. From the third month, proliferation of ductule structures with mucin-producing or mucin-nonproducing epithelium occurred, giving rise to cystic and papillary adenomas. From the fourth to sixth months, pancreatic adenocarcinomas and poorly differentiated carcinomas arose. The cells of the serous adenomas ultrastructurally resembled the normal pancreatic centroacinar and ductular cells with their regular glandular arrangement on basal lamina, elongated nuclei with finely dispersed heterochromatin, scanty cytoplasmic organelles, microvilli, and occasional cilium. The cells of the mucinous tumors showed similarities to ductal cells with their darker cytoplasmic matrix, larger number of small mitochondria, microfilaments, vesicles, mucin granules, and extensive interdigitations. The cells of the pancreatic carcinomas revealed irregularities in glandular formation, nuclear polymorphism, low cytodifferentiation, and ultrastructural abnormalities, but in most cases retained basic fine structural similarities to the epithelium of the pancreatic ductal system. The present results indicate that the centroacinar cell is the cell of origin of the broad spectrum of pancreatic neoplasms with various differentiation and malignancy induced in guinea fowl by virus strain Pts-56.

[Histogenetic considerations on mucinous cystomas of the ovary based on histochemical and immunohistochemical findings]. / Considerazioni istogenetiche sui cistomi mucinosi dell'ovaio in base a rilievi istochimici ed immunoistochimici.

Mucinous cystomas of the ovary, according to a new proposed classification (I.A.P., Dublin 1988), are classified in three types: endocervical, intestinal and mixed. Their histogenesis is still controversial, thus requiring further investigations. There are two main theories on this matter: a teratomatous theory based on the assumption that the mucinous cystoma is allegedly a teratoma having a monophyletic development where only the endodermal gastrointestinal component remains. The second theory, currently the most widely accepted one, maintains that mucinous cystomas derive from Muller duct residues or, more generally, from introflections of the coelomic epithelial lining through a Muller-type metaplastic process. Some authors also accept both theories. A group of 117 mucinous cystomas were investigated by histochemical methods (PB/KOH/PAS; PAT/KOH/Bh/PAS), to demonstrate the presence of O-acetylated sialomucin variants in goblet cells of intestinal type component. Endocervical type mucinous cystomas have always presented as PB/KOH/PAS negative, whereas mixed type mucinous cystomas presented as positive according to the following percentage: benign forms, 31%; borderline, 67%; malignant, 50%. These data should confirm the hypothesis that intestinal type cystomas may derive from the surface coelomic epithelium of the ovary, through a gastrointestinal metaplastic process. This hypothesis is further supported by the data obtained from the observation on two cases of intestinal metaplasia of endocervical glands, kindly supplied by Dr. Trowell. In one of them, a weak O-acetylated sialomucin secretion was identified, in addition to the presence of argentaffin cells. Furthermore, out of 38 adenocarcinomas of the endometrium and 15 adenocarcinomas of the endocervix, one case of endocervical adenocarcinoma was found, characterized by a mucous secretion rich in O-acetylated sialomucins. Moreover, immunohistochemically, by means of anti-chromogranin A monoclonal antibodies, endocrine cells were found in benign, borderline and malignant mucinous cystomas of mixed type. These data do not seem to confirm the assumed correlation between neuroendocrine cell presence and biologic behaviour of the neoplasm nor do they clarify tumor histogenesis. Another immunohistochemical study with BD5 monoclonal antibody demonstrated that this marker was present in the intestinal type epithelium of mixed mucinous cystomas. The histogenetic teratomatous hypothesis of ovarian mucinous cystomas was confirmed by reviewing 100 ovarian teratomas, in which O-acetylated sialomucins were found in the epithelial component of one mucinous carcinoid and in the intestinal type epithelium of 9 mature cystic teratomas.(ABSTRACT TRUNCATED AT 400 WORDS)

[Appendicular mucocele. Review of the literature and presentation of 8 cases]. / "Mucocele apendicular". Revisión de la literatura y aportación de ocho casos.

We reviewed the term mucocele, which some authors think should be abolished, although we do not share this opinion. We think it should be understood as no more than a descriptive term indicating dilation of the vermiform appendix by intraluminal accumulation of mucoid material, with no regard for its diverse etiology, which is the origin of the variety of terms and classifications of this condition encountered in the literature. We describe the clinical variety of this entity, emphasizing the exceptionality of its preoperative diagnosis before the introduction of modern techniques (US and CAT), which have permitted a growing number of exact diagnoses. The most appropriate technique for surgical treatment is discussed. The cases recently published in Spain are reviewed and we contribute 8 cases of our own.

Adenoma and stone formation of the biliary tract in puppies that had choledochopancreatic anastomosis.

A canine model of anomalous choledochopancreatic ductal junction (APCDJ) was produced by choledochopancreatic end to side ductal anastomosis performed in 77 mongrel puppies weighing between 1.5 and 5 kg. Histopathological studies of the biliary tract showed that as the post-operative period became lengthened metaplasia and hyperplasia increased. In nine dogs that lived for over 5 years after the operation, papillomatous adenoma occurred in four (44%), and biliary stones in seven (78%). The histological changes seen in the biliary epithelium of our experimental canine model suggest that bile duct carcinoma may be due to the persistent return of pancreatic juice into the bile duct in patients with APCDJ.

Papillary adenoma of the bladder in a patient with intermittent self-catheterization.

We report a case of papillary adenoma of the bladder. The patient had had intermittent self-catheterization for the previous 3 years. The tumors developed multiply and were identified on the trigone and posterior wall of the bladder. Dysplasia of the urothelium was associated in nontumorous areas. Transurethral resection of the tumors was performed. The tumor recurred 3 times for 3 years, but no malignant changes were identified.