Long-term urological outcome of cloaca patients with multidisciplinary management.

PURPOSE: Urological management of Cloacal Malformation (CM) focuses on preserving renal function and continence. Study aim was to analyze urinary and intestinal outcomes in CM patients, considering the length of common channel (CC) and presence of occult spinal dysraphism (OSD). METHODS: Retrospective review of CM treated at our institution by a multidisciplinary team from 1999 to 2020. Patients with follow-up < 2.5 years were excluded. Length of CC, renal function, urinary and bowel outcomes, presence of associated anomalies (especially OSD) were evaluated. RESULTS: Twenty patients were included, median age at follow-up: 8 years (4-15). A long CC > 3 cm was described in 11 (55%). Chronic kidney disease was found in 3 patients. Urinary continence was achieved in 8/20 patients, dryness (with intermittent catheterization) in 9/20. Fecal continence was obtained in 3/20, cleanliness in 14 (under bowel regimen). OSD was present in 10 patients (higher prevalence in long-CC, 73%). Among OSD, 1 patient reached fecal continence, 7 were clean; 2 achieved urinary continence, while 6 were dry. CONCLUSIONS: Length of CC and OSD may affect urinary and fecal continence. An early counseling can improve outcome at long-term follow-up. Multidisciplinary management with patient centralization in high grade institutions is recommended to achieve better results.

Short versus mid-long-term outcome of transanal irrigation in children with spina bifida and anorectal malformations.

PURPOSE: We assessed short- and mid-long-term clinical efficacy of transanal irrigation (TAI) and its effect on the quality of life of children with spina bifida (SB) and anorectal malformations (ARM). METHODS: Seventy-four pediatric patients (age 6-17 years) with SB and ARM with neurogenic bowel dysfunction were enrolled for a prospective and multicentric study. Patients were evaluated before the beginning of TAI (T0), after 3 months (T1) and after at least 2 years (range 24-32 months) (T2) using a questionnaire assessing bowel function, the Bristol scale, and two validated questionnaires on quality of life: the CHQ-PF50 questionnaire for the parents of patients aged 6-11 years and the SF36 questionnaires for patients aged between 12 and 18 years. RESULTS: Seventy-two patients completed TAI program in T1, and 67 continued into T2. Bowel outcomes (constipation and fecal incontinence) improved in both the SB and the ARM groups in the short and mid-long term. In both groups at T1 and T2, parents and children reported an improvement in quality of life and there was a significant increase of stool form types 4 and 5 as described by the Bristol scale. Common adverse effects during the study were similar at T1 and T2 without serious complications. CONCLUSIONS: We observed a sustained improvement in bowel management and quality of life in SB and ARM children during the study, more significant in the short term than in mid-long term. To maintain success rates in the mid-long term and to reduce the dropout rate, we propose patient training and careful follow-ups.

Peristeen(®) transanal irrigation in paediatric patients with anorectal malformations and spinal cord lesions: a multicentre Italian study.

AIM: In paediatric and adult patients with neurogenic bowel, transanal irrigation (TAI) of the colon has gained popularity due to the introduction of a specifically designed device. The aim of this pilot study was to present the results of TAI using the Peristeen(®) TAI system in a group of paediatric patients with anorectal malformation (ARM) and congenital or acquired spinal cord lesions (SCLs). METHOD: Eight Italian paediatric surgery and spina bifida centres participated in the study. The inclusion criteria were age between 6 and 17 years, weight above 20 kg and unsatisfactory bowel management. Patients with chronic inflammatory bowel disease, mental disability and surgery within the previous 3 months were excluded. At the beginning of treatment (T0) and after 3 months (T1) the Bristol scale, a questionnaire assessing bowel function, and two questionnaires on quality of life (QoL) for patients aged 6-11 years (CHQ-pf50) and 12-17 years (SF36) were administered. RESULTS: Eighty-three patients were enrolled, and seventy-eight completed the study (41 ARMs, 37 SCLs). At T1, constipation was reduced in ARMs from 69% to 25.6% and in SCLs from 92.7% to 41.5%, faecal incontinence in ARMs from 50% to 18.6% and in SCLs from 39% to 9.8% and flatus incontinence in ARMs from 20.9% to 9.8% and in SCLs from 31.7% to 10%. At T0, the Bristol Stool Scale types were 1-2 in 45% of ARMs and 77.5% of SCL patients, whereas at T1 types 1-2 were recorded in only 2.5% of SCL patients. QoL improved in both groups. In the younger group, a significant improvement in QoL was recorded in ARM patients for eight of nine variables and in SCL patients for seven of nine variables. CONCLUSION: This study showed that Peristeen TAI resulted in a significant time reduction in colonic cleansing, increased independence from the carer and improved QoL in paediatric patients with ARMs and SCLs.

Epidydimo-orchitis and anorectal malformations: when and in whom?

PURPOSE: Epididymo-orchitis (EO) is infrequently reported in anorectal malformation (ARM) cases. Therefore, it is difficult to assess its risk factors. METHODS: A total of 110 male patients who were operated on for ARM at the same Institution over a period of 13 years were contacted. Association was assessed between EO and the following: spinal dysraphism (SD), symptomatic VUR (VUR), and bowel management (BM) requiring enemas. The data were analyzed with the Chi-square test. RESULTS: A total of 89 patients were contacted. Ten cases of EO were found, and all occurred in patients with recto-urethral (RU) fistula after reconstruction. The patients' age at first episode ranged between 4 and 11 years. RU fistula patients experiencing EO (Group A, 10 patients) were compared with those without EO (Group B, 33 patients). VUR occurred in 9/10 cases in Group A and in 13/33 cases in group B (Chi-square 7.8658, p = 0.005038). SD was present in 4/10 cases in group A and in 13/33 cases in Group B (Chi-square 0.0434, p = 0.83491). A total of 8/10 cases in Group A and 12/33 cases in Group B were on BM (Chi-square 5.87, p = 0.0015). CONCLUSIONS: EO occurs in approximately in 20 % of male cases with ARM, and recto-urinary communication and should be considered the primary diagnosis in the presence of testicular pain. This could avoid unnecessary surgical exploration, and the family should be counseled about this subject.

Gait analysis in bladder exstrophy patients with and without pelvic osteotomy: a controlled experimental study.

BACKGROUND: The role of bony pelvic anomalies in bladder exstrophy is long established and has generated many papers addressing walking problems. Biomechanical studies and kinematic gait analysis were performed on very young children. AIM: A direct kinetic gait evaluation has never been performed, nor has the effect of pelvis dimorphism on the upper body been studied. DESIGN: Controlled experimental study. SETTING: Outpatients were studied at the time of periodic follow up. POPULATION: Nineteen patients with bladder exstrophy, age 14±8 years, and twenty-five healthy control participants, age 15±8 years, were enrolled in the present gait analysis study. METHODS: Clinical evaluation and standard gait analysis were performed. RESULTS: Gait analysis deviations between exstrophy patients and controls and between patients that received pelvic osteotomy (OT--6 patients) and those that did not (no-OT--13 patients) were analyzed. Bladder exstrophy significantly affects kinematics and kinetics of trunk, spine, pelvis, knee and foot; in particular: in OT, trunk retroversion, pelvic retroversion and rotation, hip adduction angle and moment, knee flexion and its maximum power during loading response increased, whereas in no-OT, spine angle, pelvic posterior tilt, hip extension, and the external rotation of the foot progression angle increased. All the kinetics parameters analyzed in the study showed lower values in the patient group than in controls. CONCLUSION: . Walking in patients with bladder exstrophy is accomplished by retroversion of the pelvis and deviations mainly in the spine angle in no-OT and in knee flexion in OT. CLINICAL REHABILITATION IMPACT: Gait analysis was shown to be an effective tool for the detection of walking deviations that should be identified early, prompting rehabilitative treatment in order to prevent spine and knee diseases.

A 20-year study of persistence of lower urinary tract symptoms and urinary incontinence in young women treated in childhood.

OBJECTIVE: To determine whether urinary incontinence (UI) and lower urinary tract symptoms (LUTS) persist over years, patients treated for UI and LUTS in childhood were re-evaluated in adulthood. MATERIALS AND METHODS: Forty-seven women (cases) treated in childhood for daytime UI/LUTS (group A) and nocturnal enuresis (group B) self-completed (average age: 24.89 ± 3.5 years) the International Consultation on Incontinence Questionnaire for Female with LUTS (ICIQ-FLUTS). ICIQ-FLUTS was self-administered to 111 healthy women (average age: 23 ± 5.1 years) from a nursing school as a control group. Data obtained from ICIQ-FLUTS and quality of life (QoL) score (0-10) were compared (Fisher's exact test) between patients and controls, and between group A (n = 28) and group B (n = 19). RESULTS: Prevalence of LUTS was higher in patients than in controls. The difference between patients and controls was statistically significant (p = 0.0001) for UI (34% vs. 7%) and feeling of incomplete bladder emptying (49% vs. 28%). QoL score was >5 in 59% of patients and 1% of controls (p = 0.0001). No significant differences were found between groups A and B. CONCLUSIONS: UI and LUTS are confirmed in young women who suffered for the same condition in childhood. Longitudinal studies are needed to assess if these symptoms persist or are newly onset.

Consensus review of best practice of transanal irrigation in adults.

STUDY DESIGN: Review article. OBJECTIVES: To provide a consensus expert review of the treatment modality for transanal irrigation (TAI). METHODS: A consensus group of specialists from a range of nations and disciplines who have experience in prescribing and monitoring patients using TAI worked together assimilating both the emerging literature and rapidly accruing clinical expertise. Consensus was reached by a round table discussion process, with individual members leading the article write-up in the sections where they had particular expertise. RESULTS: Detailed trouble-shooting tips and an algorithm of care to assist professionals with patient selection, management and follow-up was developed. CONCLUSION: This expert review provides a practical adjunct to training for the emerging therapeutic area of TAI. Careful patient selection, directly supervised training and sustained follow-up are key to optimise outcomes with the technique. Adopting a tailored, stepped approach to care is important in the heterogeneous patient groups to whom TAI may be applied. SPONSORSHIP: The review was financially supported by Coloplast A/S.

Isolated fetal ascites secondary to persistent urogenital sinus.

Objective. To present a case of isolated ascites secondary due to urogenital abnormalities (urogenital sinus) without any other prenatal ultrasound marker. Method. A 36-year-old woman with prenatal isolated ascites delivered a female baby, weighing 2.285 g; ascites was drained at birth and the baby underwent several episodes of urinary retention prior to undergoing X-ray investigations. Results. A voiding cystourethrogram revealed a short urogenital sinus: a vesicostomy was performed. A vaginoscopy revealed double vagina with a large posterior vagina. A posterior sagittal anorectal pull-through with genitoplasty was performed at 2 years old with 1-year follow-up. Conclusions. Though rare, a urogenital abnormality is to be suspected in fetal ascites cases with negative viral tests and no cardiac anomalies. The most common ultrasound marker of such abnormalities (fluid filled cavity) may be missing because of complete drainage of urine through the tubes into peritoneum.

Acute oligohydramnios: antenatal expression of VURD syndrome?

OBJECTIVE: Oligohydramnios (OA) is nowadays regarded as one of the best markers of renal function (RF) impairment in bladder outlet obstruction (BOO) detected in utero. As such, its onset is usually early and progressive because of decline in fetal urine production. A series of acute OA complicating pregnancies with BOO has never been reported. METHODS: Over a 7-year period, 5 fetuses with in utero suspicion of BOO exhibited an abrupt decrease of amniotic fluid after the 30th week of gestation. RESULTS: All fetuses were delivered by cesarean section: diagnosis was posterior urethral valves in 3 cases, urethral atresia in 1, and prune-belly syndrome in 1. Urologic work-up demonstrated a unilateral vesicoureteral reflux dysplasia (VURD syndrome) in all 5 fetuses. RF at 1 year was normal in 4 fetuses and impaired in 1. CONCLUSIONS: Besides obstetrical reasons, OA may also have acute onset occurring in the presence of anomalies of the urinary tract; although diagnosis is almost always BOO, functional and anatomical characteristics of the urinary tract are those of VURD syndrome with a non-functioning, refluxing renal unit. The associated acute OA/VURD syndrome may represent a milder expression of a pop-off mechanism advocated in this syndrome with a more favorable prognosis than progressive OA detected early in pregnancy.

Treatment of neurogenic bowel dysfunction using transanal irrigation: a multicenter Italian study.

STUDY DESIGN: Thirty-six patients with unsatisfactory treatment of neurogenic bowel dysfunctions (NBD) were enrolled from Spinal Units and Rehabilitation Centers in Italy. Treatment was for 3 weeks using a newly developed integrated system with an enema continence catheter for transanal irrigation (Peristeen, Coloplast A/S Kokkedal Denmark). OBJECTIVES: To evaluate the effects of Peristeen Anal Irrigation on NBD and patient quality of life (QoL). SETTING: Italy. METHODS: Lesion level, ambulatory status and hand functionality were determined in all patients. NBD symptoms and QoL were evaluated before and after treatment, using a specific questionnaire. Statistical analysis was performed using McNemar Test and Sign Test. RESULTS: Thirty-six patients were enrolled, and 32 patients completed the study. At the end of the treatment, 28.6% of patients reduced or eliminated their use of pharmaceuticals. Twenty-four patients became less dependent on their caregiver. There was a significant increase in patients' opinion of their intestinal functionality (P=0.001), QoL score (P=0.001) and their answers regarding their degree of satisfaction (P=0.001). A successful outcome was recorded for 68% of patients with fecal incontinence, and for 63% of patients with constipation. CONCLUSION: Peristeen Anal Irrigation is a simple therapeutic method for managing NBD and improving QoL. It should be considered as the treatment of choice for NBD, playing a role in the neurogenic bowel analogous to that of intermittent clean catheterization in bladder treatment.

An unusual case of synovial cyst of the hip joint presenting as femoral vein compression and severe lower limb edema.

INTRODUCTION: Synovial cyst of the hip joint causing the compression of the femoral vein is a rare occurrence. We carefully reviewed the international literature collecting 26 additional cases. REPORT: A case of a patient affected with synovial cyst of the hip joint causing the compression of the femoral vein and severe lower limb edema is presented. DISCUSSION: The treatment of choice of synovial cyst compressing the femoral vein is surgical removal.

Pediatric spinal cord injury: approach for urological rehabilitation and treatment.

PURPOSE: As proposed in this report, early urological rehabilitative management of patients with spinal cord injuries (SCIs) is mandatory, in order to prevent a poorly compliant bladder with related upper urinary tract complications and secondary renal failure. Moreover, the approach to treating this traumatic condition in children must be as much rapid as appropriate. MATERIALS AND METHODS: We evaluated our experience of the last 5 years with 17 patients (12 males and five females), with a mean age of 9.6 years at injury (range 6 months-18 years), all affected by an SCI and with direct trauma involved in more than 50%. Mechanism of injury, lesional level, the mean interval between injury and bladder management onset, and the mean interval between bladder management onset and our last control were evaluated. A standardized diagnostic approach was instituted, and all patients received at least a video-urodynamic evaluation before and after the start of urological management. A continence score was established and evaluated before and at least 6 months after the application of rehabilitation treatments (catheterization, medication). Follow-up ranged from 12 to 60 months (average 29.6 months). RESULTS: Sixteen of the 17 patients showed, at first urodynamic evaluation, a neurogenic overactive bladder. Mean bladder maximum capacity was 287.7 ml+/-146.4 SD. Mean reflex volume and end filling pressure were 119.7 ml+/-76.4 SD and 44.6 cmH(2)O+/-25 SD, respectively. Detrusor sphincter dyssynergia was present in 16 out of 17 of the cases. All patients but one began self-catheterization and medication (anticholinergics). Urinary continence improved in all patients but one. An adjunctive endoscopic procedure for continence was carried out in five out of 17 cases. The upper urinary tract was involved in two out of 17 cases. CONCLUSIONS: A prompt and standardized urological approach to pediatric SCIs is mandatory. The aims of this initial management are the prevention of further secondary damage to the upper tract and the achievement of a socially acceptable degree of urinary continence as soon as possible after the traumatic event.

Voiding dysfunction in x-linked adrenoleukodystrophy: symptom score and urodynamic findings.

PURPOSE: Adrenoleukodystrophy is an x-linked genetic disorder characterized by the breakdown of the myelin sheath surrounding nerve cells in the brain and progressive dysfunction of the adrenal gland with 6 possible phenotypes. Lower urinary tract symptoms affect the quality of life of these patients but only sporadic cases have been previously studied by urodynamics. We report to our knowledge the first series in the literature looking at the prevalent pattern. MATERIALS AND METHODS: A total of 14 children and adults 8 to 53 years old (average age 30) with different phenotypes were referred for evaluation. Of the patients 6 had adrenomyeloneuropathy without cerebral involvement (C-) 3 had the adult cerebral form, 3 had the childhood cerebral form, 1 had cerebral involvement (C+) and 1 was asymptomatic. All patients were submitted to a screening protocol, consisting of the self-administered short form questionnaire International Conference on Incontinence Questionnaire-Short Form (ICIQ-SF) and a 3-day voiding diary. Patients were divided in group 1-no urinary symptoms, ICIQ-SF score 0 and no further investigation (3); group 2-frequency/urgency, ICIQ-SF score 1 to 5, uroflowmetry and post-voiding residual urine (4); and group 3-voiding symptoms and incontinence, ICIQ-SF score greater than 5, standard urodynamic examination (7). RESULTS: A neurogenic overactive bladder was the prevalent urodynamic finding (6 of 7 patients), while hypocontractility was found in 1 case. CONCLUSIONS: Vesicosphincteric dysfunction can be explained by spinal and/or cerebral involvement in the different neurological forms of adrenoleukodystrophy. Attempts at treatment with anticholinergics and neuromodulation in 4 of our patients provided temporary benefit with clear subjective amelioration of symptoms and improved quality of life.

Percutaneous tibial nerve neuromodulation is well tolerated in children and effective for treating refractory vesical dysfunction.

PURPOSE: We evaluated pain tolerability and the preliminary results of percutaneous tibial nerve stimulation (PTNS) in children with unresponsive lower urinary tract symptoms (LUTS). MATERIALS AND METHODS: A total of 23 children 4 to 17 years old with LUTS refractory to conventional treatment underwent PTNS at 12, 30-minute weekly sessions. Ten patients had idiopathic overactive bladder, 7 were in nonneurogenic urinary retention and 6 had neuropathic bladder. Ten children were carefully evaluated for pain during needle insertion and electrical stimulation using certain scoring systems, namely the faces pain rating scale, Children's Hospital of Eastern Ontario pain scale, visual analogue scale and Questionario Italiano del Dolore. Evaluation was done at the first, sixth and last sessions. An anxiety-depression test was administered. All 23 children underwent clinical and urodynamic evaluation before and after treatment. RESULTS: All except 1 patient completed treatment. An anxious-depressive trait was found in 7 of 10 children/parents on anxiety-depression testing. Regarding pain, the faces pain rating scale never showed the severe pain face, the Children's Hospital of Eastern Ontario scale showed signs of pain at the beginning of each stimulation but not at the end, and the visual analog scale generally showed a low score with a further decrease during the first (p = 0.05), sixth (p = 0.03) and twelfth (p = 0.02) sessions. The Questionario Italiano del Dolore score was significantly related to the affective component of pain (p = 0.002) and it decreased between the first and last sessions. The 10 children with overactive bladder had symptom improvement in 80%, incontinence was cured in 5 of 9 and urodynamics showed normalization of cystometric bladder capacity in 62.5% with no more unstable contractions in those who became continent. Symptoms improved in 71% of the children in urinary retention. One of 3 and 4 of 7 patients had incontinence and post-void residual urine cured, respectively. Urodynamics showed an improved detrusor pressure at maximum flow (p = 0.009) and flow rate (p = 0.005). Symptoms and urodynamics did not significantly change in the neuropathic bladder group. CONCLUSIONS: PTNS is safe, minimally painful and feasible in children. It seems helpful for treating refractive nonneurogenic LUTS.

Neurovesical dysfunction in children after treating pelvic neoplasms.

OBJECTIVE: To evaluate 10 years of experience, and thus define the occurrence and causes, of neurogenic lower urinary tract dysfunction in children with pelvic neoplasms treated by surgery. PATIENTS AND METHODS: From 1991 to 2000, 33 children were operated by the same surgeons for pelvic neoplasms; 11 were analysed, comprising four each with sacrococcygeal teratoma (ST) and ganglioneuroma, and one each with yolk sac tumour (YST), neuroblastoma and myofibroblastic bladder sarcoma (MBS). The other patients were not assessed because eight had died or were in severe progression, three were treated by bladder substitution and the others were lost to follow-up or refused a urological evaluation. All 11 children were evaluated at >/= 6 months after surgery with a questionnaire about bowel and voiding habits, a neurological and orthopaedic assessment, a noninvasive urodynamic study, renal ultrasonography and spinal and pelvic magnetic resonance imaging (MRI). All patients with signs of bladder dysfunction were evaluated by a pressure-flow study. The results were analysed for surgical approach and anatomical involvement, i.e. group A, extensive surgery for complete tumour excision in the sacral area (ST and YST); group B, surgery for tumour resection in the paraspinal ganglia area (neuroblastoma and ganglioneuroma); and group C, bladder tumour with partial bladder resection (MBS). RESULTS: Eight patients had signs or symptoms related to bladder sphincter dysfunction. One child refused the invasive urodynamic evaluation, leaving seven for analysis (two each ST and ganglioneuroma, one each YST, neuroblastoma and MBS). The urodynamic findings were normal in three children. On spinal and pelvic MRI a presacral lipoma with syringomyelia was discovered in one child with ST. Eight children had bladder dysfunction and two had no neurogenic damage (which was only in sacral tumours); in one child it was related to an upper motor neurone lesion from spinal dysraphism and in the other to a lower motor neurone lesion from surgical injury to the splanchnic nerves. Patients operated for paraspinal tumours had more bladder dysfunction but no signs of neurogenic damage, as did the patient with partial bladder resection. However in Group B, there may have been a transient or incomplete nerve injury in one patient. CONCLUSIONS: Deficits of parasympathetic, sympathetic and somatic innervation of the bladder and the urethra may occur in children after surgery for pelvic neoplasms, related to minor or major surgical trauma. In ST, a tethered cord may be associated with mixed neurogenic damage. Knowledge of bladder dysfunction in anorectal malformations, spinal dysraphism, etc. and the clinical protocol used in these patients also seemed to be useful for understanding the development of voiding dysfunction in patients with neoplasm.

[Ascariasis as a cause of acute abdomen: a case report]. / Ascaridiasi come possibile causa di addome acuto anche in Italia: presentazione di un caso clinico.

Ascaris Lumbricoides Infestation (ALI) is one of the most common helmintic disease of the gastrointestinal tract, and may cause severe surgical complications, especially in children. ALI is frequent in tropical and subtropical countries. We present our experience with a case of a 5-years old pakistan girl treated in Italy for acute abdomen in which ALI was detected during surgical exploration.

[Malignant pheochromocytoma. Personal experience, review of the literature]. / Considerazioni sul feocromocitoma maligno. Esperienza personale, revisione della letteratura.

Pheochromocytoma is a catecholamine-producing tumor of the sympathetic nervous system which in 4-14% of the cases may be malignant. The differentiation between benign and malignant phoechromocytomas is very difficult, the presence of metastatic lesions and gross invasion into surrounding tissues are the lonely accettable parameters to confirm malignancy. Since the patients with malignant pheochromocytomas have a very poor survival rate, care must be taken for an early detections and a careful follow up. Three cases of malignant pheochromocytoma are reported. In one of these cases the tumor was a functioning mass; the other two cases presented by a hemorrhagic complication. A careful review of the pertinent literature allows to outline the correct approach to this rare pathology as for the diagnosis and the treatment, also including the actual criteria to establish its malignancy.

Postmastectomy breast reconstruction: current techniques.

BACKGROUND: The techniques of breast reconstruction have evolved and matured over the past 25 years. Recent studies have proven the benefit of breast reconstruction for breast cancer patients. METHODS: The authors reviewed the recent literature on the techniques of breast reconstruction and the effects of reconstruction on patients following surgery for breast cancer. The findings in recent studies are correlated with the experience of the authors. RESULTS: A better understanding has been gained regarding surgical techniques of breast reconstruction as well as the proper indications for the various methods. The criteria of patient benefit have been defined by recent long-term studies. CONCLUSIONS: Breast reconstruction following mastectomy has been proven to be a safe and beneficial procedure.

Desmoplastic melanoma.

Desmoplastic melanoma is an uncommonly encountered variant of malignant melanoma. Three histological subtypes exist: desmoplastic, neurotropic, and neural transforming. Desmoplastic melanoma commonly presents in conjunction with existing melanocytic lesions or as an amelanotic firm nodule. Local recurrences are common. Thirty patients over a 6-year period were treated at our institution for desmoplastic melanoma. All lesions were treated with local excision. Local recurrence occurred in seven patients (23%) and was treated by aggressive re-excision in each instance. Clinical regional metastasis (lymph nodal basins) were detected in two patients (6%). Distant metastasis (lung) developed in two patients (6%). Twenty-three patients (76%) were found to have desmoplastic subtype, whereas five (17%) had neurotropic subtype. Six patients (20%) had associated pigmented melanotic lesions. Average length of follow-up has been 18 months. Overall survival is 96 per cent. Presentations and histologic diagnosis can sometimes be difficult and misleading. Treatment is aggressive local excision with follow-up necessary to detect resectable recurrent lesions.