Presacral malakoplakia presenting as foot drop: a case report.

BACKGROUND: Malakoplakia is a rare condition characterized by inflammatory masses with specific histological characteristics. These soft tissue masses can mimic tumors and tend to develop in association with chronic or recurrent infections, typically of the urinary tract. A specific defect in innate immunity has been described. In the absence of randomized controlled trials, management is based on an understanding of the biology and on case reports. CASE PRESENTATION: Here we describe a case of presacral malakoplakia in a British Indian woman in her late 30s, presenting with complex unilateral foot drop. Four years earlier, she had suffered a protracted episode of intrapelvic sepsis following a caesarean delivery. Resection of her presacral soft tissue mass was not possible. She received empiric antibiotics, a cholinergic agonist, and ascorbic acid. She responded well to medical management both when first treated and following a recurrence of symptoms after completing an initial 8 months of therapy. Whole exome sequencing of the patient and her parents was undertaken but no clear causal variant was identified. CONCLUSIONS: Malakoplakia is uncommon but the diagnosis should be considered where soft tissue masses develop at the site of chronic or recurrent infections. Obtaining tissue for histological examination is key to making the diagnosis. This case suggests that surgical resection is not always needed to achieve a good clinical and radiological outcome.

Centrally infiltrating renal masses on CT: differentiating intrarenal transitional cell carcinoma from centrally located renal cell carcinoma.

OBJECTIVE: The objective of our study was to retrospectively determine the accuracy of CT for differentiating intrarenal transitional cell carcinoma (TCC) from centrally located renal cell carcinoma (RCC) and to define the most discriminating diagnostic CT features. MATERIALS AND METHODS: CT studies of 98 pathologically proven central renal tumors (64 centrally located RCCs and 34 intrarenal TCCs) seen over 5 years at three university hospitals were reviewed by five specialty-trained radiologists who were blinded to the final diagnosis. Multiple CT features and global impression were graded on a 4-point score. The sensitivity and specificity of each feature and of global assessment were calculated and compared using receiver operating characteristic (ROC) analysis. Interobserver agreement (kappa values) was also calculated for each parameter. RESULTS: All five readers recognized intrarenal TCCs with a high diagnostic accuracy (sensitivity, 90%; specificity, 90%; area under ROC curve [AUC], 0.80-0.95 for global assessment) with moderate-to-excellent interobserver agreement (κ = 0.72-1). Six CT features were most diagnostically specific for identifying intrarenal TCCs: tumor centered within the collecting system; focal filling defect in the pelvicalyceal system; preserved renal shape; absence of cystic or necrotic change; homogeneous tumor enhancement; and tumor extension toward the ureteropelvic junction (sensitivity, 68-82%; specificity, 79-89%; AUC, 0.75-0.84). There was moderate-to-good agreement among the readers over all these features (κ = 0.44-0.69). CONCLUSION: Intrarenal TCC can be recognized with a high accuracy on CT; global impression showed the best diagnostic performance. A solid, homogeneously enhancing mass that is centered on the collecting system and extends toward the ureteropelvic junction combined with a focal pelvicalyceal filling defect and preserved renal outline is more likely to be an intrarenal TCC than a centrally located RCC.

Lymphoscintigraphic Abnormalities Associated with Milroy Disease and Lymphedema-Distichiasis Syndrome.

Background: Primary lymphedema is genetically heterogeneous. Two of the most common forms of primary lymphedema are Milroy disease (MD) and lymphedema-distichiasis syndrome (LDS). This study aims to look further into the pathogenesis of the two conditions by analyzing the lymphoscintigram images from affected individuals to ascertain if it is a useful diagnostic tool. Methods and Results: The lymphoscintigrams of patients with MD and LDS were analyzed, comparing the images and transport parameters of the two genotypes against a control population. Lymphoscintigrams were available for 12 MD and 16 LDS patients (all genetically proven diagnoses). Eight of the 12 (67%) lymph scans performed on patients with MD demonstrated little or no uptake from the initial lymphatics and poor visualization of the inguinal lymph nodes. These changes were consistent with a "functional aplasia," that is, the lymphatic vessels were present but appeared to be ineffective in absorbing the interstitial fluid into the lymphatic system. In patients with LDS the lymphoscintigraphic appearances were different. In 12 of the 16 scans (75%), the lymph scans were highly suggestive of lymphatic collector reflux. Quantification revealed a significantly reduced uptake of tracer within the inguinal lymph nodes and a higher residual activity in the feet at 2 hours in MD compared with LDS and compared with controls. Conclusion: Lymphoscintigraphic imaging and quantification can be characteristic in specific genetic forms of primary lymphedema and may be useful as an additional tool for in-depth phenotyping, leading to a more accurate diagnosis and providing insight into the underlying mechanism of disease.

Uterine artery pseudoaneurysm requiring embolization in pregnancy: a case report and review of the literature.

BACKGROUND: Uterine Artery Pseudoaneurysm is a rare cause of pelvic pain and haemorrhage in pregnancy. It should be considered in the differential diagnosis of pregnant women presenting with abdominal pain and is readily diagnosed by colour Doppler ultrasound. If left untreated, they may bleed into the peritoneum causing severe pain and haemorrhagic shock and may progress to maternal and fetal death. CASE PRESENTATION: We describe a case of a woman presenting with severe right iliac fossa pain at 26 weeks gestation attributed to a right uterine artery pseudoaneurysm diagnosed on duplex ultrasound which was successfully treated by uterine artery embolization at 28 weeks gestation without complication to the fetus. CONCLUSION: Uterine artery embolization appears to be a safe and effective method to treat pseudoaneurysm during pregnancy without compromising uteroplacental perfusion.

A Case-Control Study of the Lymphatic Phenotype of Yellow Nail Syndrome.

BACKGROUND: Yellow nail syndrome (YNS) is a rare disease manifesting as a triad of yellow-green dystrophic nails, lymphedema, and chronic respiratory disease. The etiology of YNS is obscure and investigations are few. A single lymphatic pathogenesis has been proposed to account for all the associated features, and despite the lack of evidence for a unifying lymphatic mechanism, this hypothesis prevails. The objective was to explore the lymphatic phenotype in YNS and to establish whether lymphatic dysfunction could be a major contributing factor to the disease process. METHODS AND RESULTS: Four-limb lymphoscintigraphy was performed on patients with YNS and on healthy, age-matched controls. All 17 patients had lower limb swelling, and 14 (82%) had upper limb swelling also, including 5 (29%) with hand involvement. None of the YNS lymph scans was completely normal. Combined qualitative and quantitative assessment showed that 67% of YNS scans were clearly abnormal compared with 36% of healthy control scans. Mean axillary and ilio-inguinal nodal tracer uptakes were 41%-44% lower in the YNS group than in the controls (p < 0.0001). CONCLUSIONS: YNS is a lymphatic phenotype because lymphatic insufficiency was found to exist in all patients and the insufficiency was widespread (upper and lower limbs), with a common mechanistic fault of poor transport. The origin of the lymphatic fault is unclear. In healthy individuals, lymphatic abnormalities may be relatively common in the fifth decade of life onward.

Repeat planar white cell scanning to monitor short-term therapy of active inflammatory bowel disease: a methodological study and comparison with clinical scores and novel inflammatory markers.

OBJECTIVES: Radiolabelled white cell scans provide non-invasive quantification of inflammatory activity. Clinical activity scores measure severity of disease but are partly subjective. White cell scans may provide a suitable method of monitoring the treatment response of active inflammatory bowel disease. METHODS: Ten subjects with active ulcerative colitis and 13 subjects with active Crohn's disease were recruited. White cell scans were carried out before and 2 weeks after treatment. Prior to each scan, activity scores for ulcerative colitis or Crohn's disease were calculated and serum and faecal tumour necrosis factor-alpha and calprotectin measured. White cell scan activity at 1 h was calculated by using a validated visual grading system. RESULTS: Following anti-inflammatory treatment, 70% of white cell scans improved, 17% remained unchanged and 13% deteriorated. In the ulcerative colitis subgroup subjects there was modest agreement for change in scan score and activity scores. In the Crohn's disease subjects there was better agreement between change of white cell scan score and clinical scores. Planar white cell scans correlated with the van Hees activity index (r=0.68, P=0.002) and faecal calprotectin (r=0.58, P=0.0003). Changes in planar white cell scans correlated with changes in serum calprotectin (r=0.45, P=0.05). CONCLUSION: Non-invasive white cell scanning is a feasible and objective method to monitor the anti-inflammatory efficacy of treatments for active inflammatory bowel disease.

Evaluation of dynamic lymphoscintigraphy and sentinel lymph-node biopsy for detecting occult metastases in patients with penile squamous cell carcinoma.

OBJECTIVE: To evaluate the introduction of dynamic lymphoscintigraphy and sentinel lymph-node (SLN) biopsy (used to detect occult lymph node metastases in patients with penile cancer and clinically impalpable inguinal lymph nodes at presentation) at a UK tertiary referral centre for penile cancer. PATIENTS AND METHODS: In all, 75 patients with penile squamous cell carcinoma of stage T1, grade > or = 2, and unilateral or bilateral impalpable groin nodes, were prospectively enrolled over a 2-year period. Patients underwent lymphoscintigraphy with (99m)technetium-labelled nanocolloid which was injected intradermally around the tumour or into the distal penile shaft skin. Four hours later, the SLN(s) were identified during surgery using a hand-held gamma-probe and intradermal injections with blue dye. Completion lymph node dissection was subsequently used in patients with tumour-positive SLNs. RESULTS: In all, 255 SLNs were removed from 143 groins; all excised nodes had taken up the radioactive marker, and the blue dye was evident in 87%. Eighteen of 75 (24%) patients and 21 of 143 groins (15%) had a tumour-positive SLN. All but one patient went on to completion lymph node dissection. Three of these 18 (17%) had further disease in other than SLNs. Six of 143 (4%) groins developed minor complications. One false-negative result was reported at a median (range) follow-up of 11 (2-24) months. CONCLUSION: This technique is feasible for managing penile cancer in a UK tertiary referral centre. The initial results suggest that it can accurately identify the SLN(s), which can then be removed for pathological review with minimal morbidity.