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Systemic mastocytosis with involvement of the pelvis: a radiographic and clinicopathologic study--a case report.

The musculoskeletal manifestations of systemic mastocytosis have been described in the literature. We present a case of systemic mastocytosis involving the pelvis. Conventional radiographs and CT imaging may demonstrate diffuse sclerotic, or mixed lytic and sclerotic lesions. On MRI, the lesions, if sclerotic, may show low signal on T1- and T2-weighted images. However, if lytic, the lesions may demonstrate low signal on T1, and increased signal on T2. As there are numerous disease entities included within the differential diagnosis, the clinical and pathological findings are crucial for establishing the correct diagnosis.

Adult↗

Transitional cell carcinoma of the ureter and renal pelvis.

Transitional cell carcinoma (TCC) of ureter and renal pelvis is relatively uncommon. Smoking, occupational carcinogens, analgesic abuse, Balkan nephropathy are the risk factors. Cytogenetic studies revealed that the most frequent aberration is the partial or complete loss of chromosome 9. Approximately 20-50% of patients with upper urinary tract (UUT) TCC have bladder cancer at some point on their course, whereas the incidence of UUT TCC after primary bladder cancer is 0.7-4%. Excretory urography and retrograde pyelography are the conventional diagnostic tools; however, ureteropyeloscopy combined with cytology and biopsy is more accurate. Grade and stage of the disease have the most significant impact on survival. Nephroureterectomy with bladder cuff excision has been the mainstay of treatment. Local resection may be appropriate for distal ureteral lesions especially when the disease is low grade and stage. Advances in endourology have made it possible to treat many tumors conservatively. Ureteroscopic and to a certain extent percutaneous surgical approaches are widely used today especially in patients with low grade, low stage disease. Endoscopic close surveillance is mandatory for these patients. Adjuvant topical therapies appear to be safe but confirmation of any benefits awaits the results of further large studies. More recently, laparoscopic techniques have become a viable alternative to open surgery, but long term cancer control data are lacking. Aggressive surgical resection does not affect the outcome of patients with advanced disease. Adjuvant radiotherapy is ineffective, and systemic chemotherapy results in a low complete response rate for patients with metastases.

Carcinoma, Transitional Cell↗

Magnetic resonance imaging of the adolescent female pelvis.

MR imaging is well suited to evaluation of the pelvis. Ultrasonography remains the study of choice for the initial evaluation of a suspected gynecologic mass. MR imaging and computed tomography remain problem-solving tools that can help to characterize masses and stage gynecologic malignancies. MR imaging has the advantage of higher soft tissue contrast compared with computed tomography. This article reviews the MR findings of congenital anomalies and masses of the adnexa and uterus in adolescent girls.

Adolescent↗

Magnetic resonance angiography of the pediatric abdomen and pelvis.

Although abdominal and pelvic vascular disease is much less common in children than in adults, MR angiographic techniques widely used in adults can also be readily applied in the pediatric population with suspected vascular disease. MR techniques, equipment, and image analysis software are in a continual state of development and refinement. Given the advantages of MR angiography (i.e., its absence of ionizing radiation, capability to obtain images without iodinated contrast material, and limited invasiveness), the applications of MR angiography in the pediatric abdomen and pelvis are likely to continue to increase.

Abdomen↗

Epidermoid cyst in the renal pelvis.

Epidermoid cysts are benign cysts localizing rarely in solid organs with an unclear pathogenesis. A 55 years old woman having an epidermoid cyst in the renal pelvis was evaluated according to the literature.

Epidermal Cyst↗

Squamous cell carcinoma of the renal pelvis manifesting after percutaneous nephrolithotomy for long standing calculus.

Squamous cell carcinoma of the renal pelvis is a rare neoplasm and is usually associated with long standing stone disease. The disease is aggressive in nature and usually has a poor prognosis. We report a case of renal lithiasis who underwent successful percutaneous nephrolithotomy (PCNL) for renal pelvic calculus, and eight months later presented with a large invasive squamous cell carcinoma in the same location.

Carcinoma, Squamous Cell↗

Prognostic significance of cyclin E and p53 protein overexpression in carcinoma of the renal pelvis and ureter.

Cyclin E gene alteration in the cell cycle plays an important role in carcinogenesis, while p53 protein affects different phase checkpoint pathways by activating p21WAF1/CIP1 in the normal cell cycle. We immunohistochemically examined the expression of cyclin E and p53 proteins in 121 patients with transitional cell carcinoma (TCC) of the renal pelvis and ureter to determine their significance for tumour behaviour and patient prognosis. Cyclin E and p53 immunostaining of the nucleus was observed in 36 tumours (29.8%) and 35 tumours (28.9%) respectively. A significant percentage, 69.4% (25 out of 36 tumours), of the cyclin E-positive tumours exhibited simultaneous labelling for p53 (P < 0.05). Mirror-section technique was performed in five selected double-positive tumours to identify cancer cells that were nuclei positive for both cyclin E and p53. The prevalence of cases simultaneously exhibiting both cyclin E and p53 immunostaining was higher in the high-grade tumours (P < 0.01) than in the other types of tumours. Patients with TCCs coexpressing cyclin E and p53 had a significantly poorer prognosis than those expressing neither cyclin E nor p53 (P < 0.001). These in vivo findings provide evidence for cyclin E protein overexpression in TCCs intimately associated with p53 alteration and suggest that simultaneous overexpression of both cyclin E and p53 is related to tumour behaviour and poor prognosis.

Aged↗

Excitatory motor and electrical effects produced by tachykinins in the human and guinea-pig isolated ureter and guinea-pig renal pelvis.

1. In isolated tissue experiments, neurokinin A (NKA) produced concentration-dependent contraction of human and guinea-pig ureter (pD2 = 6.7 and 7.2, respectively); an effect greatly reduced (>80% inhibition) by the tachykinin NK2 receptor-selective antagonist MEN 11420 (0.1 microM). The tachykinin NK1 and NK3 receptor agonists septide and senktide, respectively, were ineffective. 2. Electrical field stimulation (EFS) of the guinea-pig isolated renal pelvis produced an inotropic response blocked by MEN 11420 (0.01-1 microM). In the same preparation MEN 11420 (0.1 microM) blocked (apparent pK(B) = 8.2) the potentiation of spontaneous motor activity produced by the NK2 receptor-selective agonist [betaAla8]NKA(4-10). 3. In sucrose-gap experiments, EFS evoked action potentials (APs) accompanied by phasic contractions of human and guinea-pig ureter, which were unaffected by tetrodotoxin or MEN 11420 (3 microM), but were blocked by nifedipine (1-10 microM). NKA (1-3 microM) produced a slow membrane depolarization with superimposed APs and a tonic contraction with superimposed phasic contractions. NKA prolonged the duration of EFS-evoked APs and potentiated the accompanying contractions. MEN 11420 completely prevented the responses to NKA in both the human and guinea-pig ureter. 4. Nifedipine (1-10 microM) suppressed the NKA-evoked APs and phasic contractions in both human and guinea-pig ureter, and slightly reduced the membrane depolarization induced by NKA. A tonic-type contraction of the human ureter in response to NKA persisted in the presence of nifedipine. 5. In conclusion, tachykinins produce smooth muscle excitation in both human and guinea-pig ureter by stimulating receptors of the NK2 type only. NK2 receptor activation depolarizes the membrane to trigger the firing of APs from latent pacemakers.

Aged↗

Extravasation of parenteral alimentation fluid into the renal pelvis--a complication of central venous catheter in a neonate.

Many complications of central venous catheters, which include perforation of the vessel walls and extravasation of the infusate into pericardial, pleural, and peritoneal cavities, have been reported. We report an infant with a central venous catheter in inferior vena cava who experienced extravasation of parenteral alimentation fluid into the right renal pelvis secondary to perforation of the renal vein. To our knowledge, this rare complication has not been reported earlier.

Catheterization, Central Venous↗

Fascial structures and autonomic nerves in the female pelvis: a study using macroscopic slices and their corresponding histology.

We investigated the topographical anatomy of the pelvic fasciae and autonomic nerves using macroscopic slices of five decalcified female pelves. The lateral aspect of the supravaginal cervix uteri and superior-most vagina issued abundant thick fiber bundles. These visceral fibrous tissues extended dorsolaterally, joined another fibrous tissue from the rectum (the actual lateral ligament of the rectum) and attached to the parietal fibrous tissues at and around the sciatic foramina (i.e. the sacrospinous ligament, thick fasciae of the coccygeus and piriformis and dorsal end of the covering fascia of the levator ani). The inferior or ventral vagina also issued thick fiber bundles communicating with the levator ani fascia. This connection between the vagina and levator fascia, when stretched, seemed to provide a macroscopic morphology called the arcus tendineus fasciae pelvis. The overall morphology of the visceroparietal fascial bridge exhibited a bilateral wing-like shape. The fascial bridge complex was adjacent but dorso-inferior to the internal iliac vascular sheath and located slightly ventral to the pelvic splanchnic nerve. However, the pelvic plexus and its peripheral branches were embedded in the fascial complex. The hypogastric nerve ran along and beneath the uterosacral peritoneal fold, which did not contain thick fibrous tissue. During surgery, in combination with the superficially located vascular sheath, the morphology of the visceroparietal fascial bridge and associated nerves seemed to be artificially changed and developed into the so-called cardinal, uterosacral, uterovesical and/or rectal lateral ligaments. The classical and original concepts of these pelvic fascial structures may need to be altered to adjust to these surgical observations.

Aged↗

Spontaneous rupture of the renal pelvis.

The object of the case report is to present a case of spontaneous rupture of the renal pelvis with no underlying pathological condition to account for the rupture, a very rare and unusual occurrence. The patient was a 46-year-old Korean who presented with severe left loin pain and vomiting. The diagnosis was suspected on intravenous urography and confirmed by CT. Treatment was by ureteral stenting. The patient made a complete recovery. The differential diagnosis of this condition is discussed and the literature is reviewed.

Diagnosis, Differential↗

Adenocarcinoma of the renal pelvis with vimentin-positive intracytoplasmic inclusions.

A case of mucus-producing papillary adenocarcinoma of the renal pelvis associated with multiple renal calculi in a 16-year-old male is reported. The majority of the tumor cells contained large, round or cone-shaped, vimentin-positive intracytoplasmic inclusions, which exhibited some morphological and immunohistochemical resemblance to inclusions found in malignant rhabdoid tumor of the kidney. An intracytoplasmic accumulation of mucus was also noted in some tumor cells. The patient is free from recurrence and metastasis after a nephrectomy.

Adenocarcinoma, Papillary↗

Embryonal sarcoma of adult and pediatric kidneys: report of a case with localized submucosal invasion of the renal pelvis and long-term survival.

A case of cystic embryonal sarcoma of the kidney (CESK) with a rapidly fatal outcome was recently reported.1 Here, we report another case of a 12-year-old boy with a localized but ill-defined submucosal lesion of CESK in the right renal pelvis. The tumor consisted principally of small mesenchymal cells with oval to spindle nuclei and scanty cytoplasm, infiltrating in dense arrangements. Two growth patterns were distinguished in the tumor cells: (i) a diffuse infiltrating pattern without an epithelial component; and (ii) a foliated pattern with an epithelial lining over the surface. Foci of the diffuse pattern predominated over those that were lobular, infiltrating superficial layers of renal sinuses and along pyramids, in both of which remarkable intravenous invasion was evident. Foci of the foliated pattern invaded deeper portions of a few sinuses and frequently penetrated into their veins, producing together with their epithelial lining a characteristic foliated structure. Lining epithelial cells around lobular foci often appeared hob-nailed or eosinophilic in the cytoplasm. Despite the remarkable intravenous encroachment, the patient has remained well without a recurrence for more than 26 years after a simple nephrectomy. The present case report expands our understanding of the biological nature of CESK.

Child↗

The natural history of pelvi-ureteric junction obstruction in children presenting clinically with the complaint.

OBJECTIVE: To determine the natural history of untreated pelvi-ureteric junction (PUJ) obstruction in children presenting clinically with the complaint. PATIENTS AND METHODS: The study comprised 42 children with anatomical PUJ obstruction (three with bilateral lesions) who were managed expectantly in the first instance, who had no immediately troublesome symptoms and differential function in the affected kidney(s) of > 40%. They were followed by serial ultrasonography and dynamic diuresis renography. Pyeloplasty was advised for those with persistent symptoms or where differential renal function fell below 40%. RESULTS: Only 15 children had presented with clearly relevant symptoms (loin pain or febrile urinary infection). Thirty-four kidneys showed obstructive renographic curves initially and 38 had moderate or severe hydronephrosis. During the follow-up (range 14-120 months, mean 56) patients remained asymptomatic except for four of the nine presenting with loin pain. Renographic curves were apt to change, with 'obstruction' increasing or decreasing with time, in the latter instance usually with lessening or resolution of hydronephrosis. Eleven patients underwent pyeloplasty, five for symptoms and six because of deteriorating renal function. Renal function did not decline in any patient with mild hydronephrosis and/or a non-obstructive renographic curve at presentation, but did so disproportionately in those with severe hydronephrosis or a Type IV renographic curve. CONCLUSIONS: Patients effectively asymptomatic at presentation are likely to remain so. Expectant management is appropriate for patients with mild hydronephrosis and/or non-obstructive renographic curves at the outset. Conversely, pyeloplasty may be advisable for those with severe hydronephrosis or Type IV renographic curves. Otherwise, for most the natural history of the complaint remains to be determined.

Adolescent↗

Functional results of pyeloplasty in patients with ante-natally diagnosed pelvi-ureteric junction obstruction.

OBJECTIVE: To assess the outcome after pyeloplasty in children with an ante-natal diagnosis of hydronephrosis, shown on post-natal renography to be due to pelvi-ureteric junction (PUJ) obstruction, and in particular to review the outcome of those who had initially been managed expectantly. PATIENTS AND METHODS: Between 1984 and 1995, 321 patients were diagnosed as having PUJ obstruction, after investigating ante-natal hydronephrosis. Of these, 47 had undergone pyeloplasty and also had a normal contralateral kidney; 26 patients had early pyeloplasty because of impaired function and 21 underwent surgery after a period of expectant management. Renal function was assessed renographically before and at least one year after surgery. RESULTS: Relative renal function was stabilized in those patients who underwent early pyeloplasty (mean differential function 28.1% before and 32.7% after surgery). In patients who underwent pyeloplasty after a period of expectant management, the mean relative function decreased from 44.8% initially to 30.5% before surgery; this recovered to 37.5% at the post-operative reassessment, representing a statistically significant loss of function (P < 0.01). CONCLUSION: In patients born with PUJ obstruction and impaired renal function, pyeloplasty failed to significantly improve function. Possibly because of cortical loss. In patients with ante-natal PUJ obstruction managed expectantly there is a small but significant risk of a modest loss of renal function.

Child↗

Retained surgical materials in the postoperative abdomen and pelvis.

The imaging appearances of surgical materials in the postoperative abdomen and pelvis can be confusing and difficult to interpret. With the increasing complexity of surgical procedures and more frequent use of postoperative imaging, the radiologist needs to be familiar with the imaging characteristics of a variety of intentionally and unintentionally placed surgical materials and devices. In addition, they must be differentiated from postoperative complications such as hematoma or abscess.

Abdomen↗

Misplaced devices in the chest, abdomen, and pelvis: Part I.

Numerous medical devices are used in the chest and fewer in the abdomen and pelvis. They are frequently seen on various radiological studies in daily practice. Knowing the specific name of the device is not important. However, knowing the proper positioning and function of the device is necessary. It is a duty of the reporting radiologist to recognize the malpositioning or breakage of a medical device and to inform the responsible physician promptly, since these complications can have undesirable consequences and sometimes a fatal outcome.

Equipment and Supplies↗