Elderly onset intramedullary epidermoid cyst in the conus medullaris: a case report

Introduction

Epidermoid cysts are known as embryonic or acquired ectopic aberrations of the ectoderm. To the best of our knowledge, there are only a few reports of elderly onset intramedullary epidermoid cysts. We report a case of elderly onset intramedullary epidermoid cyst at the conus medullaris.

Case presentation

A 63-year-old Japanese woman working as a farmer presented with slowly progressive gait disturbance and voiding dysfunction. A magnetic resonance imaging scan revealed an intramedullary mass lesion at L1 to L3. We diagnosed the lesion as an intramedullary spinal cord tumor. A laminectomy was performed at the level of Th12 to L3. Upon spinal cord dissection, a yellowish milky exudation erupted from the cystic lesion. We resected white cartilage-like pieces from the cystic cavity. Because the wall of the cystic lesion tightly adhered to the spinal cord parenchyma, we abandoned complete resection of the cyst wall. The pathological diagnosis was an epidermoid cyst.

Conclusions

We propose that evacuation of the cyst contents is preferable, especially in cases with elderly onset and congenital origin.

Electronic supplementary material

The online version of this article (doi:10.1186/1752-1947-9-7) contains supplementary material, which is available to authorized users.     

An epidermal cyst of the penis after distal hypospadias surgery: a case report

Background

Epidermoid cyst is a benign tumor that can occur anywhere in the body but is rarely seen in the penis. Congenital and previous penile surgeries have been reported to be involved in the etiology of the disease, which is usually asymptomatic. Here we describe a case of a patient with a penile epidermoid cyst, which occurred in the circumcision line on the left side of his penis, and urethral dehiscence following hypospadias surgery.

Case summary

A 3-year-old white boy who underwent primary distal hypospadias surgery 1.5?years ago presented with a slowly growing mass in the left ventrolateral portion of the penile circumcision line and urethral dehiscence. The histology of the excised mass revealed an epidermal inclusion cyst. Since then, he has remained healthy.

Conclusions

Epidermal inclusion cyst as a complication of hypospadias surgery is a very rare situation. The diagnosis is made histologically and surgical excision is sufficient for treatment.

     

Specific Glycoforms of MUC5AC and Endorepellin Accurately Distinguish Mucinous from Nonmucinous Pancreatic Cysts

Specific protein glycoforms may be uniquely informative about the pathological state of a cyst and may serve as accurate biomarkers. Here we tested that hypothesis using antibody-lectin sandwich arrays in broad screens of protein glycoforms and in targeted studies of candidate markers. We profiled 16 different glycoforms of proteins captured by 72 different antibodies in cyst fluid from mucinous and nonmucinous cysts (n = 22), and we then tested a three-marker panel in 22 addition samples and 22 blinded samples. Glycan alterations were not widespread among the proteins and were mainly confined to MUC5AC and endorepellin. Specific glycoforms of these proteins, defined by reactivity with wheat germ agglutinin and a blood group H antibody, were significantly elevated in mucinous cysts, whereas the core protein levels were not significantly elevated. A three-marker panel based on these glycoforms distinguished mucinous from nonmucinous cysts with 93% accuracy (89% sensitivity, 100% specificity) in a prevalidation sample set (n = 44) and with 91% accuracy (87% sensitivity, 100% specificity) in independent, blinded samples (n = 22). Targeted lectin measurements and mass spectrometry analyses indicated that the higher wheat germ agglutinin and blood group H reactivity was due to oligosaccharides terminating in GlcNAc or N-acetyl-lactosamine with occasional α1,2-linked fucose. The results show that MUC5AC and endorepellin glycoforms may be highly specific and sensitive biomarkers for the differentiation of mucinous from nonmucinous pancreatic cysts.Cysts in the pancreas can be a clinical challenge to patients and physicians, because some are precancerous and may progress to invasive cancer, whereas others remain indolent (1). The first step in diagnosis is to determine the type of cyst. Two types of pancreatic cysts—intraductal papillary mucinous neoplasms (IPMNs)¹ and mucinous cystic neoplasms (MCNs), together termed “mucinous cysts”—have malignant potential, and other types of cysts, such as serous cystadenomas (SCs) and pseudocysts (PCs), are essentially benign. To date, there are difficulties in correctly determining the cyst type. The methods commonly used to assess pancreatic cysts are endoscopic ultrasound and cyst fluid aspiration followed by the carcinoembryonic antigen (CEA) assay and cytology. The most useful is CEA, which distinguishes mucinous cysts from nonmucinous cysts with 70% to 80% accuracy (2–4). Cytology (microscopic analysis of the cells in the fluid) has limited value because of the lack of consistency in obtaining reliable cellular material (2). Other biomarkers, including DNA analysis, have yet to achieve widespread use (5).Because the current tests are not conclusive for many patients, new, more effective molecular markers are needed to provide more accurate diagnoses and to help guide management. Researchers have investigated molecular features such as DNA mutations (6, 7), specific protein levels (8), microRNAs (9), inflammatory cytokines (10), and the presence of mucin (11, 12). Broad searches utilizing genomic, proteomic, and glycomic profiling (13–15) have uncovered other candidate biomarkers that require further study and validation.We previously investigated whether specific protein glycoforms are present exclusively in the fluid from mucinous cysts (16). The reason for investigating specific glycoforms, rather than simply the protein levels, is that the glycoforms may be more closely associated with disease. Cells can rework the glycosylation machinery as they become neoplastic and progress to invasive cancer. For example, cancer cells and dysplastic cells can enhance N-glycan complexity through increased branching (17), produce truncated O-glycans through the lost activity of an enzyme critical for O-glycan extension (18), or increase fucosylation or the production of Lewis structures (19). Such glycan restructuring can affect cellular adhesiveness, migration, cytokine signaling, receptor recycling, or immune cell interactions (20), all potentially involved in cancer progression. Therefore, we potentially can get more information about the differentiation state of a cell by detecting protein glycosylation in addition to protein abundance.The protein MUC5AC in cyst fluid demonstrated such potential in a previous study by our group (16). We examined both protein levels and specific glycoform levels of MUC5AC in fluid from mucinous cysts relative to nonmucinous cysts. A glycoform of MUC5AC, one that binds the lectin wheat germ agglutinin (WGA), was almost exclusively found in the mucinous cysts and was a better biomarker than the MUC5AC protein measured over all glycoforms. Furthermore, the glycosylation patterns in pancreatic cysts were different from those we found in pancreatic ductal adenocarcinomas (21, 22).In the current study, we performed a broad search of addition glycoforms of MUC5AC and other proteins that may be up-regulated in mucinous cysts and tested the hypothesis that the detection of specific glycoforms can serve to accurately discriminate mucinous from nonmucinous cysts. We confirmed the importance of the WGA-reactive glycoform of MUC5AC and found a second glycoform of MUC5AC, displaying the blood group H (BGH) antigen, up-regulated in mucinous cysts. We found a new marker, endorepellin, that also displayed elevated WGA-reactive and BGH-reactive glycoforms in mucinous cysts. A panel of three markers, comprising two glycoforms of MUC5AC and one of endorepellin, classified the samples with high sensitivity and specificity, providing the potential for more accurate diagnoses of pancreatic cysts. We further characterized the diagnostic glycoforms using mass spectrometry and targeted lectin analyses, which provided information about the molecular features of cystic precursors of cancer and routes for the further development of biomarker assays.     

Traumatismes fermés des bourses: stratégie de prise en charge

Introduction

Blunt scrotal trauma is increasingly frequent, but surgical exploration of these cases of trauma remains controversial. The objectives of this study were to assess the diagnostic value of clinical examination and ultrasound in testicular trauma and to analyse the complications of the various treatments proposed (surgical and medical treatments), in order to more clearly define the place of medical or surgical treatment in this form of trauma in young adults.

Patients and Methods

130 cases of blunt scrotal trauma were managed between 1993 and 2004. In the absence of clinical and ultrasound criteria of severity (haematocele, very large intratesticular haematoma, rupture of the tunica albuginea), medical treatment consisting of rest, anti-inflammatory drugs, and testicular support was instituted. Surgical exploration was performed when serious lesions of the testis were suspected. Scrotal ultrasound was performed in 68 patients and 46 of them underwent scrotal exploration. The sensitivity and specificity of scrotal ultrasound were determined by comparing radiological findings with definitive intraoperative findings. The immediate morbidity and long-term sequelae were analysed.

Results

The mean age of the patients was 24 years (range: 4 to 73 years). The clinical features were dominated by pain (73.8%) and scrotal swelling (89.2%). The sensitivity of testicular ultrasound was 34.7% for testicular rupture, 100% for testicular contusions, 33.3% for intratesticular hematoma and 76.9% for haematocele. Medical and surgical treatments were instituted in 29.2% and 70.7% of cases, respectively. With a mean follow-up of 8 months, chronic pain and testicular atrophy were observed in 18% and 5.5% of cases, respectively.

Conclusion

In the absence of signs of severity, medical treatment with regular surveillance remains justified. However, in the presence of doubtful clinical or ultrasound findings, surgical exploration must be performed as soon as possible     

Sorafenib-induced Scrotal Eczema

Sorafenib is an orally active, small-molecule multikinase inhibitor that blocks tumor cell proliferation and angiogenesis. Studies have shown that it is a highly potent, selective inhibitor of vascular endothelial growth factor receptors 2 and 3, platelet-derived growth factor-β, RAF, FLT-3, and c-Kit. This drug was recently approved for the treatment of metastatic renal cell carcinoma and hepatocellular carcinoma. We report a case of a patient treated with sorafenib for metastatic renal cell carcinoma who developed scrotal eczema.Key words: Sorafenib, Scrotal eczema, Hand-foot skin reactionSorafenib is approved by the US Food and Drug Administration for use in metastatic renal cell carcinoma.¹ It is a multikinase inhibitor that blocks both tumor cell proliferation and angiogenesis. ² Up to 90% of patients receiving sorafenib have reported dermatologic symptoms, including handfoot skin reaction, facial erythema, subungual hemorrhage, alopecia, pruritus, and xerosis.³ Here we report on a patient on sorafenib who presents with the uncommon adverse effect of scrotal eczema.     

Formation of Diapause Cyst Shell in Brine Shrimp, Artemia parthenogenetica, and Its Resistance Role in Environmental Stresses

Artemia has attracted much attention for its ability to produce encysted embryos wrapped in a protective shell when subject to extremely harsh environmental conditions. However, what the cyst shell is synthesized from and how the formative process is performed remains, as yet, largely unknown. Over 20 oviparous specifically expressed genes were identified through screening the subtracted cDNA library enriched between oviparous and ovoviviparous Artemia ovisacs. Among them, a shell gland-specifically expressed gene (SGEG) has been found to be involved in the cyst shell formation. Lacking SGEG protein (by RNA interference) caused the cyst shell to become translucent and the chorion layer of the shell to become less compact and pultaceous and to show a marked decrease of iron composition within the shell. The RNA interference induced defective diapause cysts with a totally compromised resistibility to UV irradiation, extremely large temperature differences, osmotic pressure, dryness, and organic solvent stresses. In contrast, the natural cyst would provide adequate protection from all such factors. SGEG contains a 345-bp open reading frame, and its consequentially translated peptide consists of a 33-amino acid residue putative signal peptide and an 81-amino acid residue mature peptide. The results of Northern blotting and in situ hybridization indicate that the gene is specifically expressed in the cells of shell glands during the period of diapause cyst formation of oviparous Artemia. This investigation adds strong insight into the mechanism of cyst shell formation of Artemia and may be applicable to other areas of research in extremophile biology.Salt lakes on plateaus, are widely known as “seas of death,” because they represent one of the most hostile environments on the earth in terms of extreme salinity, high pH, anoxia, large temperature differences, and intermittent dry conditions. Hardly any animal can survive such extremes. However, one notable exception lies in the shape of a small crustacean, Artemia.Artemia, also called the brine shrimp, is an ancient species that first appeared ∼400 million years ago (1). To cope with harsh and complex habitats such as salt lakes, Artemia are able, when the circumstances become adverse, to release their offspring into a dormant, encysted state, rather than simply releasing swimming nauplius, to ensure survival. Such adverse conditions include environments where the Artemia may experience high salinity, low oxygen levels, short days, or conditions of extreme temperature variation (2, 3). These dormant cysts will keep diapause until the state is terminated by activation (triggered by factors such as desiccation, dehydration, cold or chemical treatment), at which point they resume development when appropriate and stable environmental conditions have arisen (4–7).The diapause cysts, with their greatly reduced metabolic activity, contain embryos existing as late gastrulae and are composed of ∼4000 cells that are arrested at the G₂/M phase with a complete turning off of RNA and protein synthesis (8, 9). Previous studies indicate that the resistance and resumption ability of Artemia cysts have several causes. In addition to the arrested cell cycle, it has been noted that large amounts of two molecular chaperone proteins, namely p26 and artemin, are synthesized (10–12), and a high concentration of trehalose is also accumulated (13–15). Moreover, a complicated enzyme system is also involved in the diapause and resumption mechanism, including AMP-activated protein kinase (16) and p90 ribosomal S6 kinase regulatory pathway (17–19).In addition to falling into diapause, Artemia themselves secrete a rigid noncellular shell to cope with the extreme environmental stresses before they release the diapause cysts. The complex noncellular cyst shell consists of two main regions; the outer region, secreted by the shell gland, is of hypochlorite-soluble chorion, whereas the hypochlorite-resistant inner region is formed by blastoderm cells and comprises the embryonic cuticle (5, 20, 21). The shell glands, which are composed of clusters of secretory cells, are situated at the ovisac and open into the uterus. There are many dark brown secretory granules, which probably contain chorion material and pigments such as hematin formed in the cells of the shell glands at the point where the oocytes emerge in the ovaries during the reproductive period. These are secreted out at the second day after the oocytes enter the uterus. Therefore the shell glands vary from dark brown to white, even to colorless, as reproductive cycles differ (22, 23).Microphotographs shot by Sugumar and Munuswamy (24) reveal that both the chorion and the embryonic cuticle have an exquisite structure (21). Chorion consists of two distinct layers. First, a compact outer covering is over the cyst with many radially aligned aeropyles penetrating through. This is known as the cortical layer. Second, in a cavernous region below the cortical layer is the alveolar layer, which may act as a float for the newly laid cysts. A thin supra cortical layer, probably consisting of cuticulin, covers the outer surface of the cortical layer. The embryonic cuticle, which is impermeable to nonvolatile solutes, is otherwise composed of a broad multilamellar region as a fibrous layer sandwiched between the outer and inner cuticular membranes and constructed as a tripartite structure. This forms an area of relative independence from the external environment and serves to maintain the homeostasis of inorganic ions (2). The molecular formulation of the cyst shell is complex, and details remain unclear, although it is known that the cyst shell does contain chitin, lipoprotein, hematin, and some metal elements (25–27).Besides preventing mechanical damage (28), the cyst shell also plays an important role in protecting the embryo within from other lethal environmental stresses. Previous experimental data have confirmed the protective capabilities of the cyst shell. Tanguay et al. (29) indicated that the hatching rate of intact cysts is significantly higher than the decapsulated ones after ultraviolet irradiation treatment. Hematin, the hemopigment of the cyst shell, is also demonstrated to have a light-screening function (27). Clegg (30) indicated that the cyst shell plays a critical role in desiccation tolerance, because the rate of dehydration of decapsulated cysts is much higher than intact ones in the dehydration study, and rapid water loss significantly reduces the hatching level of dehydrated cysts. Liu et al. (31) also found that intact cysts have better thermotolerance than decapsulated ones in both dry and water heating studies.In our experiments, through the in vivo gene knockdown by RNA interference, a shell gland-specifically expressed gene (SGEG) has been found to be involved in the cyst shell formation. The formed cyst shell has been demonstrated to play an important role in resistance to UV irradiation, large temperature differences, osmotic pressure, dryness, and organic solution stresses.     

Rare Case of Monozygotic Twins Diagnosed With Klinefelter Syndrome During Evaluation for Infertility

Although neither Klinefelter syndrome nor monozygotic twins are particularly rare (1/667 male births and 3–4/1000 live births, respectively), the occurrence of both in the same pregnancy (ie, identical twins with Klinefelter syndrome) is exceedingly rare and has only been reported three times previously in the literature. This report describes the fourth ever reported case of monozygotic twins with Klinefelter syndrome (who presented to our male fertility clinic with failure to conceive) and sheds interesting light on the reproductive concordance observed with this rare clinical entity. To our knowledge, this is the first reported case of monozygotic twins with Klinefelter syndrome that describes the infertility workup and outcomes of microsurgical testicular sperm extraction.Key words: Klinefelter syndrome, Microsurgical testicular sperm extraction, Azoospermia, Sertoli only syndrome, Germ cell aplasiaKlinefelter syndrome, the most common sex chromosome disorder in men, is the clinical result of an additional X chromosome in human males. This syndrome, which affects an estimated 1 in 667 live male births, most commonly manifests as 47,XXY, but may also take the form of 46,XY/47,XXY (Klinefelter mosaicism), 48,XXXY, or 49,XXXXY.¹ Typical clinical manifestations of the syndrome include primary infertility, atrophic testes, hypergonadotropic hypogonadism, gynecomastia, eunuchoidism, and decreased facial and body hair.¹ This condition often goes undiagnosed in prepubertal boys, and even in adult men, despite the physical hallmarks of the syndrome; many cases come to light only during the evaluation of primary male factor infertility. The andrologist, therefore, plays a central role in the diagnosis, work-up, and management of men with Klinefelter syndrome.With an incidence approximately twice that of Klinefelter (3–4 per 1000 live births worldwide),² monozygotic (identical) twinning occurs when one fertilized egg splits and divides into two embryos. Monozygotic twins have been observed with various karyotypic abnormalities, including trisomy 21,³ trisomy 18,⁴ and trisomy 13.⁵ However, the presentation of monozygotic twins with Klinefelter syndrome (a fertilized egg with a 47, XXY karyotype splitting to produce identical embryos with Klinefelter syndrome) is exceedingly rare. In this report, we discuss one case of identical twin brothers diagnosed with Klinefelter syndrome at our fertility clinic (Glickman Urological & Kidney Institute, Cleveland, OH) as part of a work-up for inability to conceive.     

Calyceal Diverticula: A Comprehensive Review

Calyceal diverticula are rare outpouchings of the upper collecting system that likely have a congenital origin. Stones can be found in up to 50% of calyceal diverticula, although, over the combined reported series, 96% of patients presented with stones. Diagnosis is best made by intravenous urography or computed tomography urogram. Shock wave lithotripsy (SWL) is an option for first-line therapy in patients with stone-bearing diverticula that have radiologically patent necks in mid- to upper-pole diverticula and small stone burdens. Stone-free rates are the lowest with SWL, although patients report being asymptomatic following therapy in up to 75% of cases with extended follow-up. Ureteroscopy (URS) is best suited for management of anteriorly located mid- to upperpole diverticular stones. Drawbacks to URS include difficulty in identifying the ostium and low rate of obliteration. Percutaneous management is best used in posteriorly located mid- to lower-pole stones, and offers the ability to directly ablate the diverticulum. Percutaneous nephrolithotomy remains effective in the management of upperpole diverticula, but carries the risk of pulmonary complications unless subcostal access strategies such as triangulation or renal displacement are used. Laparoscopic surgery provides definitive management, but should be reserved for cases with large stones in anteriorly located diverticula with thin overlying parenchyma, and cases that are refractory to other treatment. This article reviews the current theories on the pathogenesis of calyceal diverticula. The current classification is examined in addition to the current diagnostic methods. Here we summarize an extensive review of the literature on the outcomes of the different treatment approaches.Key words: Calyceal diverticula, Percutaneous nephrostolithotomy, Laparoscopic surgery, Shock wave lithotripsy, UreterorenoscopyCalyceal diverticula are eventrations of the upper collecting system lying within the renal parenchyma.¹ These nonsecretory outpouchings are lined by transitional cell epithelium and communicate with the main collecting system via a narrow channel, allowing for passive filling with urine. They were first described in 1841 by Rayer in “Traitements des maladies des reins.”² Thought to be either cysts or localized hydronephrosis, he used the term kyste urinaire to describe his finding of intrarenal urine-containing cavities that communicate with calyces. Other investigators reported similar findings and—depending on location and postulated etiology—described them as pelvic cysts,³ peripelvic cysts,⁴ pyelorenal cysts,⁵ pyelosynaptic cysts,⁶ pyelogenous cysts,⁷ hydrocalicosis,⁸ cystic dilatations of the calyx,⁹ congenital cortical cysts,¹⁰ congenital cystic dysplasia,^4,11 calyceal pseudocysts,¹² juxta-calyceal cysts,¹³ pelvic diverticula, ¹⁴ congenital diverticula of the calyx,¹⁵ and finally, calyceal diverticula.^16–18 Prather is credited with coining the term and the definition of calyceal diverticulum that we use today.     

Traumatisme du testicule chez l’enfant

Introduction

The testicular trauma is uncommon in children and the literature about this topic is very poor. The aim was to investigate the testicular trauma in children from a retrospective study.

Materials and methods

All the French pediatric surgical departments were requested to send their cases of testicular trauma reported between 2000 and 2009. The analysis was performed from the data and the operative report. Isolated scrotal wounds, testicular torsions revealed by trauma, and incomplete files were excluded.

Results

Fifteen departments sent their data and 2 had no cases. From 60 cases, 15 were excluded and 45 selected. The mean age was 12.3 years (2 days–18 years). The circumstances of the trauma were 23 knocks (foot, knee, and fist), 13 falls, 4 motorcycle accidents, 4 unknown traumas, and 1 obstetrical trauma. There were 4 wounds and 41 without wound. A color Doppler sonography was performed in 34 cases (75.5%) with an accurate diagnosis in 30 cases; 2 testicular fractures and 1 intratesticular hematoma were not confirmed at surgery, and 1 epididymal hematoma was not seen on sonography. The lesions were testicular fractures (15), intratesticular hematomas (8), benign testicular blunt traumas (6), spermatic cord hematomas (6), epididymal hematomas (6), isolated hematoceles (3), and avulsion (1). Surgical treatment was done in 33 children and 12 had nonoperative treatment. Among the 15 testicular fractures, 13 were operated (8 sutures and 5 partial orchidectomies) and 2 were only observed. For the intratesticular hematomas, a surgical treatment was performed in 5 children and a nonoperative treatment in 3, with a good evolution. Only 20 children were controlled beyond 4 months, especially the testicular fractures and no secondary testicular atrophy was seen.

Conclusion

The testicular trauma in children is rare and the prognosis is rather good. The color Doppler sonography now allows a good diagnosis in most cases and the surgery can be often avoided in benign blunt trauma. In this situation, it is important to repeat sonography 24 or 48 hours after trauma to ensure there is no fracture or other lesion necessitating a surgical treatment.     

Effectiveness of Antegrade Access in Bladder Tumors With Inaccessible Urethra

Inaccessible urethra with no retrograde endoscopic access due to multiple/diffuse strictures or multiple urethrocutaneous fistulas with acute urinary retention due to posturethral instrumentation (transurethral resection of bladder tumor [TURBT], or TURBT with transurethral resection of the prostate [TURP]), is a rare entity. Management of such a case with a bladder tumor for TURBT/surveillance cystoscopy poses a great challenge. The authors present 12 cases of bladder tumor with inaccessible urethra, 10 cases due to multiple strictures (post-TURBT and/or TURP), and 2 cases due to urethrocutaneous fistulas (post-TURBT), who presented to our emergency department with acute urinary retention. Emergent suprapubic catheterization was used as a temporary treatment method.Key words: Suprapubic cystostomy, Inaccessible urethra, Bladder tumors, Tract seedlingBladder tumors are the most common neoplasm of the lower urinary tract, comprising 6% of all malignancies in men and 2% of those in women.¹ A majority of patients present with gross painless hematuria, usually as the sole presenting symptom.² Bladder carcinoma is unique among human neoplasms in that many of its etiologic factors are known; the urologist should be aware of the possible occupational exposures to urothelial carcinogens.³ Initial symptoms of urothelial carcinoma of the bladder (UCB) include microhematuria, painless macrohematuria, and/or irritative voiding symptoms, and require further investigation. Carcinoma in situ of the bladder causes irritative lower urinary tract symptoms (LUTS) more often than does papillary UCB. Histopathologic evaluation is necessary to assess stage and grade with sufficient certainty after the appearance of bladder tumors.⁴ Bladder tumors spread by implantation in abdominal wounds, denuded epithelium, resected prostatic fossa, or traumatized urethra⁵; implantation occurs most often with high-grade tumors.     

Aspects diagnostiques et thérapeutiques de la torsion du cordon spermatique au CHU Aristide-Le-Dantec de Dakar

Objective

To study the diagnostic and therapeutic features of testicular torsion in our daily practice, and to compare our results with that of the existing literature.

Patients and methods

A retrospective study was conducted from January 2002 to December 2009 on all patients who presented in emergency with suspicion of testicular torsion.

Results

Testicular torsion was confirmed in 58 patients after scrotal exploration. The average age was 20 years (range, 1–44 years), and 48 patients (83%) were more than 15 years old. The average duration from time of onset of pain to arrival at the emergency department was 102 hours; 47 patients (81%) were received after the sixth hour and 19 (33%) were referred from peripheral health facilities. Torsion was supravaginal in 5 patients, all more than 15 years old; orchidectomy was performed in 30 patients (52%).

Conclusion

In our study, we have a high proportion of orchidectomy. To reduce this, it will be important to sensibilize population to go to the hospital when they have cases of testicular pain and edema.     

Occult Renal Cell Carcinoma Manifesting as Nasal Mass and Epistaxis

Metastasis of renal cell carcinoma (RCC) to the nasal cavity and paranasal sinuses is rare, with fewer than 50 cases described in the literature. Nasal metastasis as the initial presentation of RCC is even rarer. Metastases to the nasal cavity usually represent advanced disease with poor outcome. The authors report a case of metastatic RCC presenting with right nasal cavity mass and epistaxis, followed by a brief review of the relevant literature.Key words: Renal cell carcinoma, Nasal metastasis, EpistaxisRenal cell carcinoma (RCC) accounts for approximately 85% of primary renal tumors, and represents approximately 3% of all adult malignancies.¹ Usual sites of metastasis include lungs (75%), regional lymph nodes (65%), bone (40%), liver (40%), and brain (5%).² Metastasis to the nasal cavity is an extremely rare occurrence, with fewer than 50 cases reported,^3,4 although RCC is the most common infraclavicular primary tumor that metastasizes to the nasal cavity and paranasal sinuses.⁵ We describe a case of occult clear-cell RCC that presented with epistaxis due to nasal cavity metastasis.     

Immunoglobulin G4-related Retroperitoneal Fibrosis of the Pelvis

Retroperitoneal fibrosis (RPF) is a rare disease characterized by the replacement of normal tissue with fibrosis and/or inflammation. In this case, a 68-year-old man presented with RPF in the pelvis, a rare location for this disease. Biopsies were performed, which showed elevated levels of C-reactive protein, erythrocyte sedimentation rate, and, most importantly, immunoglobulin G4 (IgG4). It has been postulated that IgG4-related sclerosing disease is a systemic disease. Treatment has been successful with systemic corticosteroids.Key words: Retroperitoneal fibrosis, Serum immunoglobulin G4Retroperitoneal fibrosis (RPF) is characterized by the replacement of the normal retroperitoneal tissue with fibrosis and/or chronic nonspecific inflammation.¹ Pelvic retroperitoneal fibrosis is a rare location for the primary presentation of this disease, with only a few documented cases in the literature.² Identification of immunoglobulin G4 (IgG4) autoimmune activity among these cases has begun to improve our understanding of the inflammatory nature of this rare disease process.³ This case demonstrates the involvement of immune-mediated IgG4 within a case of pelvic retroperitoneal fibrosis.     

Contribution of the scrotum,testes, and testicular artery to scrotal/testicular thermoregulation in bulls at two ambient temperatures

The objective of this study was to determine the contribution of the scrotum, testes, and the testicular artery to scrotal/testicular thermoregulation in bulls at two ambient temperatures. Crossbred beef bulls, 1.5 years of age, were placed in controlled environment chambers at ambient temperatures of 15°C (n = 5) or 25°C (n = 6). The distal lateral aspects and entire ventral part of the scrotum was incised under caudal epidural anaesthesia (xylazine, 0.07 mg kg⁻¹). Both testes were withdrawn from the scrotum and then replaced and maintained by clamping the scrotal incisions with towel clamps. One testis was randomly chosen to be the exposed testis and was withdrawn prior to temperature measurements. Surface and internal temperatures were measured with infrared thermography and needle thermocouples, respectively. Temperature gradients (°C; difference in temperature from top to bottom at 15 and at 25°C) were: scrotal surface (with testis), 1.5 and 1.3; scrotal surface (without testis), 2.1 and 1.6; surface of exposed testis, −0.6 and 0.0; sub-tunic of exposed testis, −2.2 and −0.6; intratesticular (covered testis), 0.0 and 0.4; and intratesticular (exposed testis), −1.3 and 0.4. The scrotum markedly affects testicular temperature but the testes have limited influence on scrotal surface temperature. The bovine scrotum and testes have opposing temperature gradients that complement one another, resulting in a relatively uniform intratesticular temperature. These temperature gradients are attributed in part to the testicular artery, which goes from the top of the testis to the bottom, divides into several branches and ramifies dorsally and laterally before entering the testicular parenchyma. Intra-arterial temperatures (measured with needle thermocouples) were lower (P < 0.05) where the artery entered the testis than at both the bottom and top of the testis for both the covered (31.7, 33.4 and 34.3°C) and exposed testis (29.6, 32.0 and 32.5°C) at an ambient temperature of 15°C. Temperature differences were similar, but less pronounced, at 25°C (covered testis, 34.8, 36.3 and 36.5°C; exposed testis, 32.4, 33.5, 33.9°C). Results supported the hypothesis that blood within the testicular artery has a similar temperature at the top of the testis (just ventral to the testicular vascular cone) compared with the bottom, but subsequently cools before entering the testicular parenchyma.     

Les torsions du cordon spermatique chez l’adulte au CHU Yalgado Ouédraogo de Ouagadougou

Objective

To evaluate the epidemiologic, diagnostic and therapeutic features of testicular torsion in adults aged 15 years and older.

Materials and methods

A retrospective study was conducted from January 2004 to June 2010 in the general surgery emergency unit and urology department of the CHU Yalgado-Ouedraogo of Ouagadougou (Burkina Faso). Medical records of 51 patients who were suspected of torsion of spermatic cord were included in this study.

Results

Torsion of spermatic cord was confirmed in 40 patients (78.4%) after scrotal exploration. The average age was 26 years (range 16–55 years). The average duration from the time of onset of pain to arrival at the emergency department was 24.6 hours, and 84.3% of the patients arrived after 6 hours. Hemi-scrotal tumefaction and ascended testicle were the main clinical findings. Orchidectomy was performed in 22 patients (55%). Post-operative findings were good for all patients, and the average hospital stay was 4.3 days (range 2–7 days).

Conclusion

In our study, a high proportion of patients underwent orchidectomy. We suggest that actions must be taken to educate men about testicular pain and to receive timely treatment in case of any testicular pain.     

Adenocarcinoma of the Urethra With Mucinous Features

Primary adenocarcinoma of the female urethra is a rare malignancy. Previous studies hypothesize multiple origins, including periurethral glands or intestinal metaplasia. We report a case of a 60-year-old white woman with adenocarcinoma of the urethra who initially presented with obstructive voiding complaints secondary to a urethral mass. Wide local excision revealed invasive adenocarcinoma of the urethra with mucinous features. There was intestinal metaplasia adjacent to the tumor, as well as separate identification of intestinal metaplasia along the urethra. Ultimately, the patient underwent radical cystectomy with ileal conduit urinary diversion with no evidence of recurrence, indicating the role of early identification and surgical intervention for such cases.Key words: Urethral adenocarcinoma, Urethra, Skene gland, Genital tract tumor, Radical cystectomyCarcinoma of the urethra in women is an uncommon malignancy, accounting for approximately 0.02% of cancers in women.^1,2 Of the urethral cancers, 70% are squamous cell, 20% are transitional cell, and 10% are adenocarcinoma.¹ Primary urethral adenocarcinoma in women is a rare malignancy of unclear origin. It has been divided into two primary histologic subtypes: clear cell and columnar/mucinous (“intestinal”).³ The histologic appearance of columnar/mucinous-type adenocarcinoma is similar to colonic and endocervical malignancies.⁴ However, clear-cell adenocarcinoma is rare and may histologically resemble genital tract tumors in women.^2,4 Previous studies have alluded to the origin of urethral adenocarcinoma in the periurethral Skene glands, as these tumors were known to stain positive for prostate-specific antigen (PSA).^5,6 Other studies have proposed a different pathway after noting that chronic irritation of the urethral mucosa can lead to metaplasia into intestinal tissue, or urethritis glandularis.⁶ Although a PSA-negative adenocarcinoma does not necessarily rule out a periurethral Skene gland origin, tumors arising in this urethritis glandularis pathway are typically PSA negative.^7,8 Clear-cell carcinoma has been postulated to originate from a third pathway based on a different morphology and staining pattern. With regard to location, proximal tumors are typically the adenocarcinoma type and have a poorer prognosis than distal squamous cell tumors.⁴ Urethral adenocarcinoma typically presents with vague symptoms leading to discovery of more advanced tumors at the time of presentation. Delayed presentation has made standardization of treatment difficult to determine in the majority of patients.²     

Metastatic Urethral Melanoma: Case Report and Review of the Literature

Melanoma is a cancer that originates from melanocytes, is predominant in adults with white skin, represents 4% of skin cancers, and has high possibility of forming metastasis. This review reports on the case of a young man, age 36 years, previously diagnosed with melanoma. The patient complained of obstructive urinary symptoms and, while he was undergoing a cystoscopy, it was discovered that he had a lesion corresponding with metastatic melanoma of the prostatic urethra, which occluded almost the entire urethra and resulted in blocked urinary flow. He underwent a transurethral resection of the prostate, followed by resection of the lesion. After the procedure, he had good urinary flow and is currently on follow-up.Key words: Melanoma, Urethra, Urinary obstruction, Metastasis, Urethral melanomaPrimary malignant melanoma of the urethra is rare, representing < 1% of all melanomas^1,2; it is often misdiagnosed, which leads to delays in treatment.² The lethality is high, but its incidence is low. Prognosis is considered good if it is detected in its early stages.¹ In recent years, there have been great improvements in patient survival rates. In developed countries, the average estimated 5-year survival is 73%, whereas in developing countries, the average survival is 56%. The estimated world average is 69%.¹ Risk factors in order of importance are sensitivity to the sun, light skin, excessive sun exposure, history of skin cancer, family history of melanoma, congenital nevi, maturity, xeroderma pigmentosum, and dysplastic nevi.¹Individual management according to the clinical presentation is based on extrapolation of evidence for other melanoma treatments.² Due to low occurrence rates of urethral melanoma, the optimal therapy has not yet been established, and surgery remains the mainstay of primary therapy; adjuvant locoregional and systemic therapies are needed.² This article reports on the case of a young patient with metastatic melanoma in the urethra which led to urinary obstruction and urinary symptoms.     

Quantitative Proteomics Reveals the Essential Roles of Stromal Interaction Molecule 1 (STIM1) in the Testicular Cord Formation in Mouse Testis

Testicular cord formation in male gonadogenesis involves assembly of several cell types, the precise molecular mechanism is still not well known. With the high-throughput quantitative proteomics technology, a comparative proteomic profile of mouse embryonic male gonads were analyzed at three time points (11.5, 12.5, and 13.5 days post coitum), corresponding to critical stages of testicular cord formation in gonadal development. 4070 proteins were identified, and 338 were differentially expressed, of which the Sertoli cell specific genes were significant enrichment, with mainly increased expression across testis cord development. Additionally, we found overrepresentation of proteins related to oxidative stress in these Sertoli cell specific genes. Of these differentially expressed oxidative stress-associated Sertoli cell specific protein, stromal interaction molecule 1, was found to have discrepant mRNA and protein regulations, with increased protein expression but decreased mRNA levels during testis cord development. Knockdown of Stim1 in Sertoli cells caused extensive defects in gonadal development, including testicular cord disruption, loss of interstitium, and failed angiogenesis, together with increased levels of reactive oxygen species. And suppressing the aberrant elevation of reactive oxygen species could partly rescue the defects of testicular cord development. Taken together, our results suggest that reactive oxygen species regulation in Sertoli cells is important for gonadogenesis, and the quantitative proteomic data could be a rich resource to the elucidation of regulation of testicular cord development.Male gonadogenesis is a complex process that requires the formation and assembly of several cell types that come together to form a functional organ. These cell lineages coordinate to maintain testicular cord development but do not differentiate independently (1, 2). Shortly after the activation of Sox9, when the genital ridges are still long and very thin, pre-Sertoli cells start to aggregate around germ cell clusters and form cords; they are then referred to as Sertoli cells. Partitioning of this mass of cells into cord-forming units coincides with endothelial cell invasion and expansion of interstitial space (3, 4). In mice, organization of the testicular cords begins with aggregate of germ cell and Sertoli progenitors in the gonad. Previous studies using confocal analysis and three-dimensional modeling have reported that testicular cord formation involves three basic steps (5, 6): pre-Sertoli cells and germ cells coalesce between 10.5 and 12.5 days post coitum (dpc)¹; cords partition at 12.5 dpc with a clear basal lamina surrounding the cords, and all cords are characterized as “external” cords, defined as a single transverse loop located just under the celomic epithelium that surrounds the gonad at this stage; and refinement of cords continues at 13.5 dpc. Although Sertoli cells acting as a organizing center in testicular cord formation has been well known (3) and studies in knockout mouse models have revealed several genes associated with testicular cord formation (7–10), how these cell types assemble into a functional organ remains to be explored (2, 11).Proteomics technology has been widely used in postnatal testis development and function research in mice (12–16). Two proteomics studies have been carried out in the fetal gonads in mice, and identified more than 1000 proteins expressed in gonads (17, 18), however, the temporal proteome changes have not been elucidated during gonadogenesis. Additionally, mRNA abundance may not always predict the quantity of the corresponding functional protein, and proteomic approach can provide a systemic view of protein level regulation in a large scale (18). Therefore, this study aimed to obtain a better understanding of male gonadogenesis by establishing a first temporal proteomic profile during the initiation of gonad development in male mice. After confirming the specific time point by immunofluorescence (IF) staining, we performed a comparative proteomic analysis of samples of male mouse gonads obtained at 11.5, 12.5, and 13.5 dpc. Bioinformatics analysis and functional studies demonstrate that reactive oxygen species (ROS) regulation in Sertoli cells may be important for testicular cord formation, and functional characterizing the of stromal interaction molecule 1 (stim1), a Sertoli cell specific protein, supported this hypothesis. Our categorized protein lists can serve as a useful resource for further exploring the molecular mechanisms involved in gonadal development.     

Retroperitoneal Ancient Schwannoma: A Case Report

Schwannomas are extremely rare tumors that are composed of Schwann cells. Retroperitoneal localization comprises 0.7% to 2.6% of all schwannomas. Patients usually present with nonspecific symptoms. There are no pathognomonic features on radiologic evaluation. Preoperative biopsy is not recommended because of complication risks; however, surgery is necessary for diagnosis and treatment. Although most schwannomas are benign tumors, those that are associated with von Recklinghausen disease are malignant. Schwannomas exhibit regions of high and low cellularity, termed Antoni A and Antoni B areas, with a diffuse positivity of S100 protein on pathologic evaluation. If there are degenerative changes, such as cyst formation, hemorrhage, calcification, and hyalinization, these tumors are termed ancient schwannomas. We present a case of retroperitoneal ancient schwannoma.Key words: Retroperitoneal schwannoma, Neurilemmoma, Schwannoma, Retroperitoneal tumor, RetroperitoneumA schwannoma is a tumor of the Schwann cell or nerve cell sheath.¹ This tumor may occur anywhere there is a nerve with Schwann cells— predominantly in the head and neck region, or flexor surfaces of the extremities. Most schwannomas are benign tumors²; however, malignant schwannomas may be associated with von Recklinghausen disease.³ Retroperitoneal schwannomas are usually larger than those in other sites, and have a greater tendency to undergo spontaneous degeneration and hemorrhage when compared with head, neck, and extremity locations.⁴ We present a case of a retroperitoneal schwannoma that was reported to be an ancient schwannoma on pathologic evaluation.     

Solitary Candida albicans Infection Causing Fournier Gangrene and Review of Fungal Etiologies

Polymicrobial bacterial infections are commonly found in cases of Fournier gangrene (FG), although fungal growth may occur occasionally. Solitary fungal organisms causing FG have rarely been reported. The authors describe a case of an elderly man with a history of diabetes who presented with a necrotizing scrotal and perineal soft tissue infection. He underwent emergent surgical debridement with findings of diffuse urethral stricture disease and urinary extravasation requiring suprapubic tube placement. Candida albicans was found to be the single causative organism on culture, and the patient recovered well following antifungal treatment. Fungal infections should be considered as rare causes of necrotizing fasciitis and antifungal treatment considered in at-risk immunodeficient individuals.Key words: Fournier gangrene, Fournier’s Gangrene Severity Index, Candida albicansFournier gangrene (FG) is a rare, rapidly progressive, necrotizing infection of the perineum and genital area that was first described in 1883 by Jean Alfred Fournier in five young male patients.¹ The infectious flora causing necrotizing fasciitis are typically polymicrobial, involving aerobic and anaerobic bacteria derived from gastrointestinal, genitourinary, and cutaneous sources.^2,3 Certain predisposing conditions increase the risk of developing FG, including diabetes, chronic kidney disease, immunosuppression, local trauma, urethral stricture, or genitourinary infections.^4–6It is essential to diagnose FG early and treat it emergently because the infection can quickly progress, with mortality rates of 7.5% to 50% cited in various series.^7,8 Aggressive management involves hemodynamic stabilization, broad spectrum antibiotics to empirically cover all potential organisms, and wide surgical debridement.^3–5 Early surgical debridement with excision of all nonviable tissue is the most important component of treatment. Multiple surgical debridements are often required, as the areas of cutaneous involvement may not indicate the full extent of subcutaneous disease.⁵Rapid initiation of broad spectrum antibiotic coverage is also necessary to stabilize the presenting patient with FG before and after surgical management. The infection is generally caused by three or more microorganisms, most commonly Escherichia coli, Proteus, Enterococcus, and anaerobes.⁴ Fungal etiologies of necrotizing infections are rare but have been increasingly reported in the literature.^9–12 Candida species are commonly part of the normal flora in the gastrointestinal and genitourinary tracts of humans but may cause acute disease in the setting of compromised host immunity. This report describes a case of primary C albicans necrotizing fasciitis of the genitalia and reviews the literature regarding fungal FG to determine possible predisposing factors.