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Multiple variants of classic bladder exstrophy have been described, all of them infrequent. Superior vesical fissure is a mild variant of this pathology in which genital involvement is scarce or absent. To date, there are only isolated reports of this entity. We report a full-term female patient of Arabian descent with a clinical and radiological diagnosis of superior vesical fissure that was surgically corrected in our center with a favorable evolution. Histological study, supported by immunohistochemical techniques, showed squamous and transitional epithelium and discrete chronic inflammation. Our literature review identified 26 reports of superior vesical fissure (including ours), with high heterogeneity in terms of clinical characterization and associated malformations and with only two histological reports. The clinical evolution of the patients reported in the literature was favorable, with lower morbidity and mortality than in classical forms of bladder exstrophy., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Rectosigmoid solitary juvenile polyps are benign lesions, relatively frequent in childhood. The clinical debut of a pediatric polyp with bleeding is relatively frequent, but there are very few reports of rectal prolapse of polyps. We present the case of a 7-year-old female patient with no previous history who presented with rectal prolapse of a polyp with acute bleeding. An urgent endoscopic examination was performed and 2 rectosigmoid polypoid lesions were found and resected. The anatomopathological study showed that these were 2 hamartomatous polyps with mild dysplasia. The patient is asymptomatic and is being followed up. The literature concerning rectal prolapse of polyps in the pediatric population is scarce. In a pediatric patient with a rectal prolapse, this entity should be considered in the differential diagnosis., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Congenital prepubic sinus (PS) is an extremely infrequent malformation consisting of a prepubic fistulous tract that classically does not communicate with the genitourinary system. Previous studies centered on its immunohistochemical characterization have shown inconsistent results, and the etiology has not been clarified. We present the case of a 2-year-old male who presented since birth with a fistulous orifice on the dorsum of the penis. He had no associated symptoms. Under general anesthesia, the fistulous tract was explored, and methylene blue was instilled through it. After cystoscopically verifying the absence of communication with the urethra, a complete resection of the lesion was performed. The immunohistochemical study showed positivity for low and high molecular weight keratins and a transitional pattern for keratin 7 and GATA3, with positivity at cul de sac level and negativity at proximal level. These findings suggest that this lesion is an incomplete dorsal duplication variant.

Superficial angiomyxoma is characterized as a benign, slow-growing vascular cutaneous myxoma. A 6-year-old Arab girl with no medical history presented with a vulvar tumor located on the left labia majora. The lesion was present since birth, but it had significantly increased over the last 6 months. She did not have any associated symptoms. Physical examination revealed an exophytic tumor of the left labia majora, which measured 5 cm in its major axis. Doppler ultrasound study showed a mass with abundant arterial and venous vascularization, and magnetic resonance imaging showed a highly vascular contrast-enhanced mass with well-delimited margins, which depended on the labia majora. A macroscopically complete resection was performed, achieving a tension-free primary closure. Histologically, the lesion was characterized as a well-demarcated superficial tumor with thin-walled vessels and myxoid stroma, S100 (-), CD34 (+), vimentin (+), and actin (+). The final histopathological diagnosis was superficial angiomyxoma. The literature review of this entity in the pediatric population shows a predominance of this lesion in the vulvar location. Local recurrence has been described. Loss of PRKAR1A expression may be involved in the pathogenesis of superficial angiomyxoma.

Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Peutz-Jeghers syndrome is an autosomal dominant condition characterized by the association of hamartomatous polyps in the digestive tract, mucocutaneous pigmentation, family history, and infrequently tumors of the female genital tract with one of the most characteristic being the gastric-type endocervical adenocarcinoma. We present the case of a 75-year-old woman with a history of gastrointestinal polyps and cancer of the pancreas and breast, diagnosed with Peutz-Jeghers syndrome, who clinically debuted with a primary adnexal tumor. However, on histologic examination it was found to be a gastric-phenotype primary mucinous carcinoma tubal in origin, associated to tubal mucinous metaplasia and secondary ovarian involvement. One of her daughters had a confirmed genetic diagnosis of Peutz-Jeghers syndrome and presented with mucinous metaplasia of the tubal mucosa in the pathological study of a prophylactic hysterectomy specimen. Another of her daughters died from an ovarian juvenile granulosa cell tumor, she did not have a genetic diagnosis of Peutz-Jeghers syndrome. This case intends to highlight the rarity of gastrointestinal-type mucinous carcinomas of the ovary and fallopian tube (similar to gastric-type endocervical adenocarcinoma) in Peutz-Jeghers syndrome and emphasize the importance of genetic counseling of these patients as well as the adequate sampling of surgical specimens for early detection and treatment.

Several authors have studied the potential of sentinel lymph node (SLN) tumor burden as prognostic factor but the microscopic classifications used in different study groups were variable. We examined the prognostic role of tumor burden in SLN on melanoma specific-survival and competing causes of death. We also analysed clinical and histological factors as predictors of disease relapses and additional non sentinel lymph node (NSLN) metastases. We included all patients with cutaneous melanoma that underwent SLN biopsy between 2002 and 2012 at Complejo Hospitalario de Navarra (Spain). The study end-points were death due to melanoma, melanoma relapse and involvement of NSLN. We used Fine-Gray test for competing risk analysis. A logistic regression model was performed to predict the risk of involvement of NSLN. Between 2002 and 2012, there were 348 patients who underwent SLN biopsy in our centre (308 were eligible for the study). 26.9% patients positive SLN. 88 patients died during the follow-up period and 66 (75%) died from melanoma. The 5-year cumulative incidence of melanoma death was 15.33% (95 % CI 15.25-15.42). The cumulative probability of death from melanoma was associated with gender, histological subtype, Breslow thickness, mitotic rate, ulceration and SLN tumor burden. In multivariable analysis, Breslow thickness and SLN tumor burden remained as independent prognostic factors. SLN tumor burden appears to be an important prognostic factor. It is very important reporting these characteristics in pathological reports. More prospective studies would be necessary to analyze these variables and to be able to make recommendations in management of melanoma patients., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: For patients with metastatic renal cell cancer (mRCC), treatment choice is mainly based on clinical parameters. With many treatments available and the limited response to treatment and associated toxicities, there is much interest in identifying better biomarkers for personalized treatment. EuroTARGET aims to identify and characterize host- and tumor-related biomarkers for prediction of response to tyrosine kinase inhibitor therapy in mRCC. Here, we describe the EuroTARGET mRCC patient cohort., Methods and Materials: EuroTARGET is a European collaborative project designed as an observational study for which patients with mRCC were recruited prospectively in 62 centers. In addition, 462 patients with mRCC from previous studies were included. Detailed clinical information (baseline and follow-up) from all patients was entered in web-based case record forms. Blood was collected for germline DNA and pharmacokinetic/pharmacodynamic analyses and, where available, fresh-frozen tumor material was collected to perform tumor DNA, RNA, kinome, and methylome analyses., Results: In total, 1,210 patients with mRCC were included. Of these, 920 received a tyrosine kinase inhibitor as first-line targeted treatment (sunitinib [N = 713, 78%], sorafenib [N = 41, 4%], or pazopanib [N = 166, 18%]) and had at least 6 months of outcome assessment (median follow-up 15.3 months [interquartile range: 8.5-30.2 months]). Germline DNA samples were available from 824 of these patients, fresh-frozen tumor material from 142 patients, fresh-frozen normal kidney tissue from 95 patients, and tissue microarrays created from formalin-fixed paraffin-embedded tumor material from 247 patients. Of the 920 patients, germline DNA variant chip data were successfully generated for 811 patients (Illumina HumanOmniExpress BeadChip). For 80 patients, next-generation exome sequencing of germline and tumor DNA was performed, tumor RNA sequencing was performed for 124 patients, kinome activity measured and processed for 121 patients (PamChip), and methylome data (Illumina Infinium HumanMethylation450 BeadChip) were created for 116 RCC tissues (and 23 normal kidney tissues). For 73 out of the 920 patients, all platform data types were generated. In addition, 40 patients were included in a pharmacokinetic/pharmacodynamic phase IV substudy., Conclusions: Analysis of EuroTARGET cohort data will contribute to personalization of therapy for patients with mRCC. The extensive clinical data and multiplatform EuroTARGET data will be freely available., (Copyright © 2017 The Authors. Published by Elsevier Inc. All rights reserved.)

We report the case of a 47 years old man previously diagnosed of left hidrocele. After having a recent mild left testicular pain, an ultrasonografic study revealed a solid hipoecoic testicular lesion rounded by a big hidrocele, suggesting a testicular neoplasm. Radical inguinal orchiectomy was made and pathologic study showed segmental testicular infarction. No malignancy was found. We review the literature of the topic.

Micropapillary carcinoma is an uncommon pathologic variant of bladder carcinoma with aggressive behavior. Its usual presentation is like a high grade and high stage carcinoma and associated with other histologic types in different proportion. It doesn't differ clinically from normal transitional cell carcinoma of the bladder. Studies of molecular markers are still contradictories. Treatment should be early and aggresive, based on surgical therapy as radiotherapy and chemotherapy have shown limited results. We report a 72 year old man suffering from low urinary tract symptoms for years and recently presented gross hematuria. He was diagnosed as high stage micropapillary carcinoma. One year after radical cystectomy and subsequent chemotherapy based on carboplatin and gemcitabine, progression of the disease was shown on CT and the patient died 14 months after the diagnosis.

Objectives: To report one case of collecting duct (Bellini) carcinoma and to review the national bibliography. To analyze the clinical, diagnostic, and therapeutic features of this malignant renal tumor., Methods/results: A 36-year-old male presenting with monosymptomatic hematuria and clot retention was diagnosed of left renal mass suggestive of renal carcinoma. Radical nephrectomy with interaortocaval lymphadenectomy through an "L" abdominal incision (inverted Makuuchi) and postoperative radiotherapy were carried out. The pathologic report showed a collecting duct (Bellini) carcinoma. The patient died 34 months after surgery, having been treated with two courses of chemotherapy and palliative vertebral radiotherapy., Conclusions: The collecting duct (Bellini) carcinoma is an infrequent malignant renal tumor. It does not differ from the clear cell adenocarcinoma either clinically or epidemiologically. However, it presents its own embryological, pathologic, immunohistochemical, and cytogenetic characteristics. Radical nephrectomy is the treatment of choice. Prognosis is ominous because of the aggressive behaviour and diagnosis in advanced stages.

A wide spectrum of glandular epitheliales metaplastic changes may be seen in the bladder. Extensive replacement of the urotelium with an epithelium resembling intestinal mucosa is recognised as a premalignant condition. However, the natural history of intestinal metaplasia of the urinary bladder and long-term outcome are unknown. We report a case of glandular cystitis with intestinal metaplasia in a young patient. Cystoscopy revealed a tumour well circumscribed measuring 4 cm on the trigone. Microscopic examination showed numerous glands lined by intestinal type epithelium without atipia, conforming to the appearance of the intestinal variant of cystitis glandularis. This case had prominent foci of basophilic mucin in the stroma. Rounded aggregates of mucin were occasionally surrounded by compressed connective tissue cells, simulating mucinous cyst. This case illustrates the extent to which cystitis glandularis may mimic a neoplasm on gross evaluation and the propensity of mucin extravasation to cause diagnostic difficulty, a finding documented only rarely previously, and we discuss its biologic significance.

Objective: To report a case of extrinsic ureteral obstruction arising from mucocele of the appendix and review the clinical, diagnostic and therapeutic aspects of this appendiceal pathology of interest in urology., Methods: A 71-year-old female patient that had previously undergone radical gynecological surgery and postoperative radiotherapy presented with right lumbar pain. Patient evaluation by ultrasound, IVP and CT showed a pelvic mass causing right extrinsic ureteral obstruction. Release and reinsertion of the right ureter and appendectomy were performed., Results: The anatomopathological study showed an appendiceal mucinous cystadenoma. The patient is asymptomatic after surgery., Conclusions: Appendiceal mucocele is a dilatation of the vermiform appendix due to collection of mucoid material that is frequently produced by a cystadenoma. The presenting feature is usually that of acute appendicitis. The definitive diagnosis is based on the anatomopathological findings. Treatment is by appendectomy for the benign tumors and by right hemicolectomy in a second stage procedure for malignant tumors. This appendiceal pathology should be taken into account in the differential diagnosis of right extrinsic ureteral obstruction.

Introduction: The postoperative spindle cell nodule of the urinary tract is a being proliferative lesion similar to sarcoma in the microscopic exam. We present a case of modulein renal pelvis location., Case: A woman presenting a stag horn lithiasis in left kidney was treated by LOC and percutaneous nephroscopy. Because of the large size of the stone a second intervention was required four weeks later. Then we discovered a 2 cm exophytic lesion in renal pelvis and removed it easily with a forceps., Discussion: This kind lesion belong to the group of iatrogenic inflammatory pseudotumours, which are secondary to an injury that leads to a tissular repairing response. Differential diagnostic includes sarcomas., Conclusion: It is important that Urology and pathology specialist think of this tumour in patients having a recent surgical intervention. A mistake in the diagnosis would lead us to an unnecessary radical surgery.

Objective: To report a case of retroperitoneal bronchogenic cyst, an anomaly during the development of the primitive anterior intestine from which the bronchi and lungs are developed., Methods/results: A 38-year-old male presented with left lumbar pain. Patient evaluation disclosed a left adrenal polycystic mass which was removed by surgery. Pathological analysis of the surgical specimen demonstrated a bronchogenic cyst. This case is compared with some of the few cases reported in the literature., Conclusions: Retroperitoneal bronchogenic cyst is a cause of retroperitoneal tumors in the splenic or hepato-renal region. Although it is uncommon, it should be taken into account when making the differential diagnosis.

The inverted papilloma is a rare urothelial tumor, and its localization at the prosthatic urethra is also exceptional. We present a case of inverted papilloma of the prostatic urethra in a 72 years-old male, with symptoms of urinary flow obstruction. The diagnose is obtained after urethrocistoscopy and transurethral resection at the same time. We discuss about the etiology, clinical presentation, diagnose and treatment of this rare tumor, making special attention to its malignancy ability.

Objectives: To review our series of nephrogenic adenoma and compare our results with those reported in the literature., Method: The records of our Department of Pathology were reviewed to determine the cases of nephrogenic adenoma diagnosed at our center. The clinical features, diagnostic aspects, pathological findings, treatment and outcome are presented., Results/conclusions: Nephrogenic adenoma is a rare lesion, probably arising from nephrogenic metaplasia caused by injury to the urothelial mucosa. The site of presentation is frequently the bladder, followed by the urethra. The diagnosis is based on the histopathological findings, which can be occasionally equivocal. The cases described in the present series were benign. To date there is no established therapeutic approach. We performed TUR in two patients, antibiotic therapy was administered in one patient and 4 patients were managed conservatively. Two of our patients had no control evaluation following biopsy of the lesion. In 2 of the 4 patients that were managed conservatively, the lesion remitted spontaneously.

Small cell carcinoma of the urinary bladder is an uncommon tumor. We report a case in a 80 year-old man. The patient had gross hematuria. Urographic and echographic studies revealed a sold tumor on the right wall of bladder. Pathologic study revealed a deeply invasive tumor composed of small, uniform, round and spindled shaped cells with hyperchromatic nuclei and numerous mitosis. The immunohistochemical reaction was positive for Neuron Especific Enolase and Keratin. No metastasis were found at clinical stating (T2 No Mo). We discuss the differencial diagnosis, prognosis and histogenesis of this tumor.

A 12-year-old boy had a history of pain in the right knee for 2 months, mainly pain in extension. He could not recall any trauma to the knee. The radiograph suggested osteochondritis dissecans in the anterior spine. An arthroscopy was performed and a prominence in the tibial insertion of the anterior cruciate ligament in the anterointernal area was observed, and the fragment resected. The diagnosis of osteochondritis dissecans was confirmed by pathological study. As far as we are aware, this is the first case describing this new location.