Intimal angiosarcoma masquerading as retroperitoneal fibrosis.

We present a rare case of intimal angiosarcoma arising from the iliac artery with unusual symptoms and signs mimicking retroperitoneal fibrosis (RPF). This 84-year-old male presented with constitutional symptoms, abdominal pain, increased acute-phase reactant levels, impaired renal function and a CT-documented left-sided parailiac soft-tissue mass with unilateral extrinsic ureteric obstruction. Whole-body 18F-fluorodeoxyglucose positron emission tomography scan showed highly increased FDG-uptake in a horseshoe-like pattern surrounding the left common iliac artery, but no pathologic activity elsewhere. Further diagnostic workup revealed no signs of malignancy. Because of its location, CT-guided biopsy of the mass was precluded. A tentative diagnosis of RPF was made and treatment with Tamoxifen 20 b.i.d. was started. However, his condition gradually deteriorated, eventually succumbing to severe pneumosepsis. Autopsy revealed extensive iliac intimal angiosarcoma with infiltrative expansion to the left ureter and tumor emboli in both lungs. The present case suggests that intimal angiosarcoma should be included in the differential diagnosis of suspected RPF.

Efficacy and complications of urinary drainage procedures in idiopathic retroperitoneal fibrosis complicated by extrinsic ureteral obstruction.

OBJECTIVE: To investigate the efficacy and complications of urinary drainage procedures in patients with idiopathic retroperitoneal fibrosis complicated by ureteral obstruction. METHODS: A retrospective study of 30 idiopathic retroperitoneal fibrosis patients involving 44 obstructed urinary units who underwent urinary drainage from January 2002 through April 2010 was carried out. Data of all diagnostic procedures, blood and urine cultures, and hospital admissions were collected and analyzed. RESULTS: In 12 of 44 (27%) cases, percutaneous nephrostomy was carried out at the first step. Attempted ureteral stenting at the first step was successful in 25 of 32 (79%) cases, of which 20 (80%) cases could be managed successfully by ureteral stenting alone throughout the study period. Successful prolonged urinary drainage with percutaneous nephrostomy alone was accomplished in 10 cases, three at the first step and seven at the second step after failed intraureteral stent insertion or after unsuccessful maintenance of urinary drainage with an intraureteral stent. A total of 21 urinary tract infection episodes occurred in 11 patients. The incidence and accumulated incidence of acute pyelonephritis was 0.062 episodes/100 person-days and 30%, respectively. The incidence and accumulated incidence of urosepsis was 0.015 episodes/100 person-days and 6.6%, respectively. The overall number of complications did not differ between external and internal urinary drainage procedures (percutaneous nephrostomy, 21% vs intraureteral stent, 17.9%; P = 0.79). CONCLUSION: Both intraureteral stent and percutaneous nephrostomy placement are a safe way to relieve urinary obstruction in idiopathic retroperitoneal fibrosis patients, and they have comparable complication rates. Over time, the need for using both management options in the same patient might be required. Hence, these techniques should be regarded as complementary.

Value of acute-phase reactants in monitoring disease activity and treatment response in idiopathic retroperitoneal fibrosis.

BACKGROUND: Prospective evaluation of the value of erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels in monitoring disease activity and treatment response in patients with idiopathic retroperitoneal fibrosis (RPF). METHODS: This study included 57 patients with idiopathic RPF receiving tamoxifen monotherapy with at least 8 months follow-up. Clinical, laboratory and radiological investigation was performed at presentation and at repeated follow-up. Remission was defined as significant clinical improvement within 6 weeks of treatment together with stable or decreasing mass size on follow-up computed tomography (CT) scanning at 4 months and definitive decrease in mass size on follow-up CT scanning at 8 months. RESULTS: ESR and CRP levels at presentation and their respective decreases over time correlated strongly with each other (P<0.001). Baseline ESR and CRP levels correlated with visual analogue scale (VAS) score for pain (ESR, P<0.01; CRP, P<0.001); baseline ESR levels also correlated with VAS score for discomfort (P<0.001). Short-term decreases in ESR or CRP levels at 6 weeks follow-up did not correlate with subsequent mass regression but decrease in ESR at 4 months and decrease in CRP at 4 and 8 months follow-up correlated with mass regression. Kaplan-Meier analysis showed no difference in remission rate between patients with normal or elevated baseline ESR or CRP (log-rank P=0.22/P=0.88) or between patients with or without (near-)normalization of ESR or CRP in first 6 weeks of treatment (log-rank P=0.12/P=0.32). CONCLUSIONS: Patients with idiopathic RPF who have elevated acute-phase reactant levels are more symptomatic. Neither acute-phase reactant levels or their initial changes can be taken as a major predictor for treatment success.

Retroperitoneal Fibrosis After Spinal Surgery: A Report of 2 Cases.

CASE: Retroperitoneal fibrosis (RPF) is a rare chronic fibroinflammatory disorder with typically unknown etiology (i.e., idiopathic). However, several causes have been identified, including retroperitoneal injury or inflammation. In this study, we describe 2 patients who developed RPF complicated by hydroureteronephrosis after (combined) anterior lower interbody fusion (ALIF) and posterior spine fusion. We also reviewed 6 additional cases from the literature of suspected RPF development after spinal surgery. In these cumulative 8 cases, ALIF was the common denominator. CONCLUSION: RPF may develop after ALIF and should be considered a potential longer term complication of this procedure.

Long-term renal and patient outcome in idiopathic retroperitoneal fibrosis treated with prednisone.

BACKGROUND: Primary medical treatment of idiopathic retroperitoneal fibrosis (RPF) increasingly is accepted. However, the optimum treatment strategy is still unclear. STUDY DESIGN: Case series. SETTING & PARTICIPANTS: Single tertiary care referral center. 24 patients with idiopathic RPF treated with prednisone for 1 year, if needed, with (urgent) renal drainage from June 1991 through October 2006. OUTCOME & MEASUREMENTS: Clinical improvement, laboratory parameters, repeated computed tomographic (CT) scanning. Treatment was considered successful if the following criteria were met at the end of the 1-year treatment period: significant subjective clinical improvement, (near-)normalization of acute-phase reactants, improvement in renal function with disappearance of ureteral obstruction, and CT-documented mass regression. Recurrence is defined as need for retreatment because of return of signs and symptoms after the 1-year treatment period in patients with initial treatment success. RESULTS: 22 patients reported significant to complete resolution of symptoms after median treatment duration of 2.0 weeks (0.7 to 3.0). Follow-up showed decreases in erythrocyte sedimentation rate, C-reactive protein level (both P < 0.0001), and serum creatinine level (P = 0.0230) at 6 weeks, which persisted during the treatment period. Repeated CT scanning showed mass regression in 19 patients during the treatment period. Six patients were considered treatment failures, and there were 23 recurrences 10 months (7 to 14) after prednisone withdrawal in 13 of 18 patients with initial treatment success. At the end of follow-up (median, 55 months), 7 patients had impaired renal function; 1 patient reached end-stage renal disease. The mortality rate was 8%. LIMITATIONS: There was no comparison with other treatments. CONCLUSION: One-year treatment with prednisone is associated with a high rate of initial success, but a high recurrence rate. Despite frequent disease relapse, long-term renal and patient outcome was good.

Brief communication: tamoxifen therapy for nonmalignant retroperitoneal fibrosis.

BACKGROUND: Anecdotal case reports suggest tamoxifen as a possible treatment for retroperitoneal fibrosis, but a systematic assessment of its effect is not available. OBJECTIVE: To describe the course and outcomes of patients with nonmalignant retroperitoneal fibrosis treated with tamoxifen. DESIGN: Prospective, consecutive series. SETTING: Single tertiary care referral center. PATIENTS: 19 patients with nonmalignant retroperitoneal fibrosis treated with tamoxifen from April 1998 through April 2005. INTERVENTION: Tamoxifen, 20 mg orally twice daily. MEASUREMENTS: Clinical improvement, laboratory variables, and follow-up computed tomography (CT) and gallium scan findings. RESULTS: Fifteen patients reported substantial resolution of symptoms after a median treatment duration of 2.5 weeks. Erythrocyte sedimentation rate and C-reactive protein also improved. Gallium scanning at follow-up showed incomplete disappearance of pathologic gallium-67 activity. Repeated CT scanning showed slow but steady mass regression in 14 of 15 clinical responders. Five patients failed treatment, including 1 patient who improved clinically. Disease recurred in 1 patient who responded to reintroduction of tamoxifen. One patient developed reversible hepatitis. LIMITATIONS: This small observational study did not have a control group. CONCLUSION: Tamoxifen may be a viable therapeutic option in the treatment of retroperitoneal fibrosis.

[Localised swelling of a child's leg is not always harmless]. / Lokale zwelling in kinderbeen niet altijd onschuldig.

BACKGROUND: Soft tissue swelling of the leg is a relatively uncommon occurrence in children, and is often benign. In adults, 1% of malignancies originate in soft tissue, whereas for children the figure is 8%. Based on medical history and physical examination alone, it is difficult to differentiate between benign and malignant soft tissue tumours. CASE DESCRIPTION: A 15-year-old girl presented to her GP with a swelling in the left thigh, without alarming symptoms. The GP suspected the lesion to be benign and she was asked to return if the swelling increased in size. Five months later, when the lesion was excised, it proved to be a granular cell tumour. The second case concerns a 12-year-old boy who presented to his GP with a swelling of the lateral malleolus, which had developed after impact against a door five years ago. The boy was referred to the surgical department where after excision it was shown to be a dermatofibrosarcoma protuberans. CONCLUSION: In paediatric cases when diagnosis is inconclusive, including cases involving possible growth of the swelling, suspicions raised during physical examination, or unusually lengthy recovery after trauma, awareness is important. Imaging diagnostics or even tissue excision with histopathology may be indicated to allow the correct diagnosis to be made.

Outcome in patients with idiopathic retroperitoneal fibrosis treated with corticosteroid or tamoxifen monotherapy.

BACKGROUND: Although corticosteroids (CS) are used primarily in idiopathic retroperitoneal fibrosis (iRPF), tamoxifen (TMX) may be a suitable alternative. We compared outcome with CS or TMX monotherapy for first presentation in a large group of patients with iRPF disease. METHODS: Of all patients with iRPF disease who were seen at our tertiary care referral centre from February 1999 to December 2011, 118 patients were eligible for this retrospective study. Treatment success was defined as the composite of (i) amelioration of symptoms, (ii) computed tomography (CT)-documented mass regression and, if applicable, (iii) definitive removal of ureteral stent or nephrostomy tube. Recurrence was defined as recurrence of signs and symptoms and/or CT-documented mass increase after initial treatment success with primary treatment. RESULTS: Presenting signs and symptoms did not differ between patients treated with CS (n = 50) or TMX (n = 68). Time to amelioration of symptoms after treatment initiation was shorter in CS-treated patients [CS, 2.0 (0.8-3.8) weeks versus TMX, 4.0 (2.0-6.0) weeks; P < 0.01]. Short-term percentual decrease in acute-phase reactant levels (P < 0.001 for both erythrocyte sedimentation rate and C-reactive protein) and serum creatinine level (P < 0.01) following treatment initiation was greater in CS-treated patients compared with that in TMX-treated patients. Mass regression at first follow-up CT scan was observed more frequently in CS-treated patients (CS, 84.0% versus TMX, 68.3%; P = 0.05) with no difference in time interval from treatment initiation to first follow-up CT between groups [CS, 5 (2-7) months versus TMX, 4 (4-5) months; P = 0.34]. Definite treatment success was non-significantly higher in CS-treated patients (CS, 72.7% versus TMX, 58.3%; P = 0.15). In patients with initial treatment success with primary treatment, recurrence rate was lower in TMX-treated patients (CS, 62.5% versus TMX, 21.4%; P < 0.01). CONCLUSIONS: CS are superior to TMX in treating iRPF disease. However, in patients with initial treatment success with primary treatment, recurrence rate was lower in TMX-treated patients.

[A man with an abnormal PET-CT scan]. / Een man met een afwijkende PET-CT-scan.

A 65-year-old patient with rectal carcinoma underwent an 18FDG PET-CT scan. The scan showed nodular foci of 18FDG uptake against the ventral abdominal wall, possibly carcinomatous peritonitis. These abnormalities were caused by a foreign body reaction to a mesh that was placed five months earlier because of an umbilical hernia repair.

[A woman with an abnormality in the lung]. / Een vrouw met een afwijking in de long.

A 54-year-old woman was referred to the radiology department for an X-ray of her shoulder because of shoulder ache. Coincidentally, the X-ray showed an abnormality in the right lung. The abnormality was confirmed on chest X-ray, and a CT-scan showed a branching tubular structure with distal air trapping. This finding is pathognomonic for congenital bronchial atresia, a rare congenital abnormality. Usually this is a coincidental finding, because it is not associated with any clinical symptoms or signs. Treatment or follow-up is not necessary.