Long-term histological and hemodynamic findings of repairing penile tunica albuginea defects with collagen fleece in dogs.

Clinically, collagen fleece patching of the penile tunica albuginea (TA) has been successful. However, the histopathological and hemodynamic outcomes are not known. We studied in vivo TachoSil® patching in two beagle dogs weighing 16.8 (16.7-16.9) Kg. Bilateral intracavernous pressures (ICP) response to 10 mg papaverine hydrochloride were measured. A full-thickness defect was created on the left side in TA 1 × 0.5 cm, and four transverse incisions 1 cm long were made on the right side, placed 0.5 cm apart, and covered with TachoSil®. Six months later, ICP measurements were repeated, and the penis was excised for histopathology. Grossly, the graft site was indistinguishable. The mean baseline ICP was 19.3 ± 2.98 mmHg and increased after papaverine injection to a mean peak ICP of 122 ± 26.1 mmHg. The ICP measurement before and after grafting did not show a significant difference in the baseline (p = 0.068) or the peak pressure (p = 0.465). Histologically, minimal foreign body reaction was seen, and the TA was completely regenerated. The underlying cavernous tissue did not show inflammation or necrosis. The study is the first to show the long-term histopathologic regeneration of TA after collagen fleece patching while maintaining the hemodynamic response to papaverine.

Using machine learning models to predict synchronous genitourinary cancers among gastrointestinal stromal tumor patients.

Objectives: Gastrointestinal stromal tumors (GISTs) can occur synchronously with other neoplasms, including the genitourinary (GU) system. Machine learning (ML) may be a valuable tool in predicting synchronous GU tumors in GIST patients, and thus improving prognosis. This study aims to evaluate the use of ML algorithms to predict synchronous GU tumors among GIST patients in a specialist research center in Saudi Arabia. Materials and Methods: We analyzed data from all patients with histopathologically confirmed GIST at our facility from 2003 to 2020. Patient files were reviewed for the presence of renal cell carcinoma, adrenal tumors, or other GU cancers. Three supervised ML algorithms were used: logistic regression, XGBoost Regressor, and random forests (RFs). A set of variables, including independent attributes, was entered into the models. Results: A total of 170 patients were included in the study, with 58.8% (n = 100) being male. The median age was 57 (range: 9-91) years. The majority of GISTs were gastric (60%, n = 102) with a spindle cell histology. The most common stage at diagnosis was T2 (27.6%, n = 47) and N0 (20%, n = 34). Six patients (3.5%) had synchronous GU tumors. The RF model achieved the highest accuracy with 97.1%. Conclusion: Our study suggests that the RF model is an effective tool for predicting synchronous GU tumors in GIST patients. Larger multicenter studies, utilizing more powerful algorithms such as deep learning and other artificial intelligence subsets, are necessary to further refine and improve these predictions.

A study of the histopathology of collagen fleece (TachoSil) patching of tunica albuginea in the rat penis and a literature review of penile graft materials in experimental animals.

Background: A penile tunica defect may arise during surgery in patients with Peyronie's disease. Collagen fleece (TachoSil) has recently gained popularity in penile surgery to cover the tunica albuginea (TA) defect associated with clinical success. However, it is not known what the histological outcomes of these grafts are in the penis. We aimed to study the histopathology of the TachoSil graft in an experimental animal model for the regeneration of TA, inflammation, fibrosis, and the underlying cavernous tissue. Methods: Six adult male Sprague Dawley rats were used. The penis was degloved through a circumferential subcoronal incision. A longitudinal 1 mm × 10 mm defect was created at the base of the lateral aspect of the penis. A TachoSil patch (Takeda, Japan) was applied to the defect. The penile skin covering was then restored. At 2 months, the rat penis was excised and examined with hematoxylin, eosin, and trichrome stains. We conducted a literature review of penile grafts in animals for comparison. Results: Rats weighed 369.2 gm (standard deviation: 31.5). At 2 months, all rats showed normal-looking penis with complete healing, no scaring, tethering, or gross inflammatory features. Histopathology of the patch site showed fibrosis, chronic inflammation, and foreign body giant cell reaction. There was no generation of a new TA, or new vascularity. No inflammatory or pathological reaction affected the underlying corpus cavernous tissue. One rat died on the 6th postoperative day. Postmortem showed massive multiorgan hemorrhage consistent with disseminated intravascular coagulopathy (DIC). Unlike some other reported grafts, there is no TA regeneration. Conclusions: TachoSil patching of penile TA defect forms a distinctive barrier against inflammation, protecting the underlying corpus cavernosum. However, no regeneration of the tunica defect is observed at 2 months. DIC is a potential complication of systemic absorption of TachoSil.

A retrospective cohort study on the use of machine learning to predict stone-free status following percutaneous nephrolithotomy: An experience from Saudi Arabia.

Background: Machine learning techniques have been used extensively in the field of clinical medicine, especially when used for the construction of prediction models. The aim of the study was to use machine learning to predict the stone-free status after percutaneous nephrolithotomy (PCNL). Materials and methods: This is a retrospective cohort study of 137 patients. Data from adult patients who underwent PCNL at our institute were used for the purpose of this study. Three supervised machine learning algorithms were employed: Logistic Regression, XGBoost Regressor, and Random Forests. A set of variables comprising independent attributes including age, gender, body mass index (BMI), chronic kidney disease (CKD), hypertension (HTN), diabetes mellitus, gout, renal and stone factors (previous surgery, stone location, size, and staghorn status), and pre-operative surgical factors (infections, stent, hemoglobin, creatinine, and bacteriuria) were entered. Results: 137 patients were identified. The majority were males (65.4%; n = 89), aged 50 years and above (41.9%; n = 57). The stone-free status (SFS) rate was 86% (n = 118). An inverse relation was detected between SFS, and CKD and HTN. The accuracies were 71.4%, 74.5% and 75% using Logistic Regression, XGBoost, and Random Forest algorithms, respectively. Stone size, pre-operative hemoglobin, pre-operative creatinine, and stone type were the most important factors in predicting the SFS following PCNL. Conclusion: The Random Forest model showed the highest efficacy in predicting SFS. We developed an effective machine learning model to assist physicians and other healthcare professionals in selecting patients with renal stones who are most likely to have successful PCNL treatment based on their demographics and stone characteristics. Larger multicenter studies are needed to develop more powerful algorithms, such as deep learning and other AI subsets.

A Staghorn Calcium Phosphate Stone in a Child With Sanjad-Sakati Syndrome: An Iatrogenic Manifestation?

Sanjad-Sakati syndrome (SSS) is an autosomal recessive genetic condition, with the first report discussing this condition presented in Saudi Arabia. This case report describes an iatrogenic stone as a result of hypocalcemia overtreatment, along with its subsequent management procedure. The current literature concerning the iatrogenic stone occurrence and the operative outcome of percutaneous nephrolithotomy in individuals with SS is scarce, warranting further investigation.

A Primary Urothelial Carcinoma Presenting as a Mid-Bulbar Urethral Stricture in a 30-Year-Old Male.

The bulbar urethra is the most common site of anterior urethral strictures. In this case report, we present a 30-year-old male who was referred to us as a case of mid-bulbar urethral stricture. Urethroplasty was booked and a papillary lesion was found on routine diagnostic cystoscopy. An open biopsy was taken which showed invasive high-grade papillary urothelial carcinoma with squamous differentiation. This case is rare in terms of a young age of incidence, a lack of risk factors, an absence of suspicious symptoms, and a short duration of signs and symptoms. Urologists should consider workup for malignancy even in young patients who present with an idiopathic urethral stricture and a short duration of symptoms.

The grasper-integrated disposable flexible cystoscope is comparable to the reusable, flexible cystoscope for the detection of bladder cancer.

Flexible cystoscopy under local anaesthesia is standard for the surveillance of bladder cancer. Frequently, several reusable cystoscopes fail to reprocess. With the new grasper incorporated single-use cystoscope for retrieval of ureteric stents, we explored the feasibility of using it off-label for diagnosis and the detection of bladder cancer. Consecutive diagnostic flexible cystoscopies between Mar 2016 and Nov 2018 were reviewed comparing the reusable versus the disposable cystoscopes. A total of 390 patients underwent 1211 cystoscopies. Median age was 61.5 years (SD 14.2, 18.8-91.4), males 331 (84.9%) and females 59 (15.1%). Indication for cystoscopy was prior malignancy in 1183 procedures (97.7%), haematuria 19 (1.6%) or bladder mass 7 (0.6%). There were 608 reusable and 603 disposable cystoscopies. There was no significant difference between groups at baseline in age, sex, BMI, smoking status, or prior tumor risk category. There was no significant difference in positive findings (123/608, 20.2% vs 111/603, 18.4%, p = 0.425) or cancer detection rates (95/608, 15.6% vs 88/603, 14.4%, p 0.574) among the two groups, respectively. We conclude that the disposable grasper integrated cystoscope is comparable to reusable cystoscope in the detection of bladder cancer.

Operative outcomes of robotic partial nephrectomy. A report of the first 101 cases from a single center in Saudi Arabia.

OBJECTIVES: To report robotic partial nephrectomy (RPN) outcomes from a single tertiary hospital in Saudi Arabia. Methods: We retrospectively reviewed consecutive cases of patients undergoing RPN at King Faisal Specialist Hospital and Research Center, Riyadh, Kingdom of  Saudi Arabia, between January 2008 and January 2018. The study reports patient's demographics, tumor characteristics, operative details, and perioperative outcomes, using descriptive statistics of median and range values. Results: One hundred and one patients underwent RPN during the study period. Average tumor size was 3 (1.3-6.4) cm and average radius exophytic nearness anterior/posterior location (RENAL) score was 6 (4-10). Perioperative parameters were blood loss 200 (5-1500) ml and warm ischemia time 17 (8-40) minutes, excluding off-clamp surgery in 12 (11.9%); operative time was 166 (66-381) minutes. Conversion to open partial nephrectomy occurred in 9 (8.9%) patients, major complications in 3 (3%) patients, positive surgical margins in 5 (5%) patients, and the hospital stay was 4 (2-14) days. A total of 73 (73%) patients achieved a trifecta of freedom from any complication, negative surgical margins, and ischemia time ≤25 minutes. Study limitations included the retrospective design and small cohort size. Conclusions: The initial experience of robotic partial nephrectomy was associated with a surgical outcome comparable to that reported by higher-volume centers.

The Risks of Renal Angiomyolipoma: Reviewing the Evidence.

Renal angiomyolipoma (RAML), though a rare benign tumor, may impose a significant morbidity or even mortality due to its unique characteristics and the complications subsequent to its treatment. The classic tumor variant is composed of smooth muscular, vascular, and fatty components. The most straightforward diagnosis is when the fat component is abundant and gives a characteristic appearance on different imaging studies. In fat-poor lesions, however, the diagnosis is difficult and presumed a renal cell carcinoma. Yet, some variants of RAML, though rare, express an aggressive behavior leading to metastasis and mortality. The challenge lies in the early detection of benign variants and identifying aggressive lesions for proper management. Another challenge is when the vascular tissue component predominates and poses a risk of hemorrhage that may extend to the retroperitoneum in a massive life-threatening condition. The predicament here is to identify the characteristics of tumors at risk of bleeding and provide a prophylactic treatment. According to the clinical presentation, different treatment modalities, prophylactic or therapeutic, are available that span the spectrum of observation, embolization, or surgery. Renal impairment may result from extensive tumor burden or as a complication of the management itself. Improvement of diagnostic techniques, super-selective embolization, nephron-sparing surgery, and late treatment with the mammalian target of rapamycin inhibitors have provided more effective and safe management strategies. In this review, we examine the evidence pertaining to the risks imposed by RAML to the patients and identify merits and hazards associated with different treatment modalities.

Hypospadias repair during adulthood: Case series.

PURPOSE: We report our complications and success rate in adult hypospadias repair. PATIENTS AND METHODS: This was a retrospective study of patients aged ≥17 years who underwent hypospadias repair during 2006-2014. We analyzed two groups, one with primary repair and the other that had secondary repair after failed childhood hypospadias surgery. Outcome was compared between the two groups and among different surgical procedures. Descriptive statistics and Fisher's exact test were used and significance level was set at P < 0.05. RESULTS: Forty patients were included, of which 26 presented for a secondary repair and 14 for primary repair. The meatus was distal in 15 patients, mid-penile in 16, and penoscrotal in 9. The median age of patients at the time of surgery was 21 years (standard deviation [SD] =4, range 17-30). The median follow-up period was 37 months (SD = 8, range 5-75). In the primary repair group, the success rate was 71% (10/14), whereas in the secondary repair group, the success rate was 55% (14/26). The overall complication rate was 60%. Following a subsequent repair, the overall success rate reached 95% (38/40). There was no significant difference in success or complications between patients who presented with primary or secondary hypospadias or between methods of repair. CONCLUSION: Delayed hypospadias repair in adults is associated with a high success rate of 95% with no difference between primary and secondary repair. Secondary repair however may require more than one procedure most of the time.

Serum testosterone levels and other determinants of sperm retrieval in microdissection testicular sperm extraction.

BACKGROUND: Microdissection testicular sperm extraction (microTESE) has become the standard of care for sperm retrieval in non-obstructive azoospermia (NOA) patients. Understanding the significant determinants of microTESE outcomes may result in improvements in sperm retrieval rates and provide better-informed clinical decisions. METHODS: This is a clinical retrospective study conducted through chart review of 421 NOA patients who underwent microTESE between August 2009 and July 2015 in a tertiary-care referral hospital. Clinical, biochemical and histopathological characteristics were collected. Normal serum testosterone level was defined as testosterone >9.9 nmol/L. Multiple logistic regression was used to identify determinants of microTESE in the studied population. A P<0.05 was considered significant. RESULTS: Sperms were successfully retrieved in 39.4% of cases. The average testosterone level was 11.51±7.40 and 11.67±6.42 in patients with successful and unsuccessful microTESE, respectively (P=0.820). No significant association was found between serum testosterone level and sperm motility and amount. Of all variables, histological subtype remained to be the most significant determinant of microTESE outcomes in the examined population, with hypospermatogenesis having over a 3-fold higher odd of successful microTESE than sertoli-cell only. CONCLUSIONS: Serum testosterone level appears to have no significant association with microTESE outcomes in NOA. The underlying histological pattern is a significant determinant of the procedure's success.

Penile autotransplantation in rats: An animal model.

CONTEXT: Penile allotransplantation might be a viable option for patients who need penile reconstruction. AIMS: A successful autotransplantation rat model is the first step toward proceeding for allotransplantation. We herein evaluate autotransplantation following transaction of the rat penis just distal to the urethral bulb. SETTINGS AND DESIGN: Experimental animal study. MATERIALS AND METHODS: Five Sprague-Dawely rats weighing 520 g (SD 19) were used. Utilizing a magnification of 6-40, transection and immediate anastomosis of the tunica albuginea, urethra, dorsal vein and nerves were carried out. Vesicostomy was made to divert urine. The glandular skin was sutured to the perineum and the abdominal wall was closed in layers. STATISTICAL ANALYSIS USED: Descriptive statistics. RESULTS: Average surgery time was 8 h. The first two rats had no vesicostomy and died in the first postoperative day from retention. Three rats tolerated well the procedure and survived to the end point. One rat was sacrificed at day 10 and histopathology showed 30-50% necrosis of the implanted penis. Another rat was sacrificed at day 20 and showed normal cavernous tissue. The fifth rat was sacrificed 3 months postoperatively and showed evidence of moderate corporal fibrosis. Urethral fistula and necrosis of corpus spongiosum, dorsal nerve necrosis and dorsal vein occurred in all animals. CONCLUSIONS: Penile autotransplantation in rats is feasible and provides the basis for evaluation of the corpora cavernosa in an allotransplantation model. Long-term urethral continuity and dorsal neurovascular bundle survival in this model is difficult to establish.

Long-term outcome of genital reconstruction of Middle Eastern women with congenital adrenal hyperplasia.

OBJECTIVES: There is a paucity of data on the long-term outcome of genital reconstruction of female children with congenital adrenal hyperplasia (CAH) as they become adult women. We report on the surgical outcome general condition and marriage status. MATERIALS AND METHODS: We reviewed the medical records of women 20 years or older with CAH who had genital reconstruction. We interviewed married patients utilizing the female sexual function index (FSFI-6) questionnaire and compared them to age-matched controls. RESULTS: We identified 43 women with CAH with a median age of 24.2 ± 3.9 years and a median follow-up of 23.4 ± 4.6 years. Salt wasting and the severity of virilization affected most patients, parents were commonly cousins, children were reared as boys for a protracted period and surgical reconstruction was usually complex. Only five women had married. Compared with single women, married women had significantly more frequent normal menses, emergency hospital admissions and number of repeated reconstructive surgery. There was no significant difference in FSFI score between patients and controls. Four women conceived and three gave birth to one healthy child. There was no significant difference in the number of children between patients and controls. CONCLUSIONS: CAH has a significant impact on adult women in our region. Most of the patients remain single. Few women get married and these are able to lead a nearly normal sexual life and give birth to healthy children.

Isolated renal hydatid disease in a non-endemic country: a single centre experience.

OBJECTIVE: Isolated renal hydatid disease (HD) is rare in non-endemic countries. Clinical and radiological suspicion warrants appropriate serological tests, preoperative treatment and intra-operative precautions. We present a tertiary care centre experience of isolated renal HD in a non-endemic country. METHODS: We reviewed the medical records of patients with HD treated in the past 20 years. We identified patients with the definitive diagnosis of isolated renal HD and described their management. RESULTS: Of the 119 cases with HD, 6 were found to have isolated renal involvement (5%). Their median age was 46.5 (28-70) years. Five patients presented with flank pain and 1 had an incidentally discovered renal mass. Radiologic investigations raised the suspicion of possible HD in 4 cases, while 2 cases were diagnosed as renal tumours. Computerized tomography showed complex renal cyst in 4, solid renal mass with heterogonous enhancement in 2 and calcification in 5. Eosinophilia and indirect hemagglutination test (IHA) were positive in 3 of the 4 suspected cases. Three cases were treated as renal tumours, while 3 were managed as HD. Four cases had total nephrectomy and 2 had partial nephrectomy. Histopathology revealed that all cases had renal HD. Patients were followed for a median of 7.3 (0.4-11.3) years with no evidence of recurrence. CONCLUSIONS: Isolated renal HD is a challenging preoperative diagnosis in non-endemic countries. The definitive diagnosis is only possible by histopathology. Retrospectively, HD mimicked renal tumours in half the cases and should be considered in the differential diagnosis of renal space occupying lesions.

Changing trends in presentation, diagnosis and management of renal angiomyolipoma: comparison of sporadic and tuberous sclerosis complex-associated forms.

OBJECTIVES: To evaluate the changing management of sporadic renal angiomyolipoma and renal angiomyolipoma associated with the tuberous sclerosis complex (TSC) during the past 16 years. METHODS: We retrospectively reviewed the charts of 60 patients with angiomyolipoma seen at our institutions. RESULTS: The median age at presentation was 45 years (range 7-78). The presentation was pain in 30 patients and hematuria in 13; it was incidentally discovered in 17 patients. Of the 60 patients, 43 were females. TSC was present in 14 patients. The median tumor size was 4 cm (range 0.3-40, mean 6.5 +/- 1.1). Of the 60 patients, 31 were followed up expectantly. Surgery or intervention was needed for 29 patients to control hemorrhage or relieve pain or because of the suspicion of malignancy. Of these 29 patients, 12 underwent nephrectomy, 11 partial nephrectomy, and 6 embolization. The patients treated for hemorrhage had a median tumor diameter of 11 cm (range 2-21). Patients were followed up for a mean of 39.3 +/- 5.4 months. The lesions grew an average of 4.7 +/- 3.4 cm for TSC tumors and 0.6 +/- 0.2 cm for sporadic angiomyolipoma tumors. None of the patients developed renal impairment. Patients with TSC presented at a younger age, had larger and bilateral lesions, and were more symptomatic during follow-up. In the past 6 years, a significant trend was seen toward finding tumors in asymptomatic patients and toward the use of conservative or interventional (embolization) treatment. CONCLUSIONS: Renal angiomyolipoma has a slow growth rate. The preservation of renal function was noted in all our patients. A recent shift was noted toward finding smaller tumors in asymptomatic patients and the use of conservative and interventional treatment.

Pheochromocytoma in children and adolescents: a clinical spectrum.

PURPOSE: The purpose of the study was to identify the spectrum of disease characteristics of pheochromocytoma in children. METHODS: Records of 21 consecutive children diagnosed with pheochromocytoma were reviewed. Patients' age, sex, presentation, associated conditions, diagnostic and imaging modalities used, preoperative preparation, operative details, outcome, and follow-up were recorded. RESULTS: The study included 21 children. Patients were diagnosed clinically and confirmed by biochemical tests. Tumors were localized by imaging studies and all were confirmed pathologically. Patients included 17 with adrenal and 4 with extra-adrenal tumors including 1 in the urinary bladder. Seventeen had sporadic and 4 had familial pheochromocytoma. Associated manifestations were the predominant features in 1 of the 4 patients with familial pheochromocytoma. The patient with bladder pheochromocytoma presented with gross hematuria. Hypertension and visual disturbances were prominent findings in the other patients with sporadic form. Two patients (1 sporadic and 1 familial) had malignant pheochromocytoma. One patient with benign pheochromocytoma had multiple recurrences in chromaffin-containing sites. All patients were treated surgically. Seventeen patients were treated preoperatively with alpha-adrenergic blockade. Two patients continued to have significant visual disturbances. One patient with malignant pheochromocytoma died of the disease, and 1 with recurrent pheochromocytoma had neurologic consequences. CONCLUSIONS: Pheochromocytoma in children has unique characteristics. To our knowledge, this series is one of the largest reports of adrenal pheochromocytoma in children. It also reflects the spectrum of pheochromocytoma in this age group.

Familial pheochromocytoma.

OBJECTIVES: To identify disease characteristics, operative outcome, and prognosis in patients with familial pheochromocytoma. METHODS: Records of 29 patients with familial pheochromocytoma were retrospectively analyzed. Patients' age, gender, presenting symptoms and clinical signs, chemical and radiologic findings, and associated conditions were recorded. RESULTS: Of the 29 patients, 25 were adults and 4 were children. Age ranged from 18 to 52 years (mean age 30.8 years). Twenty patients were females. Of the 29 patients, 26 had adrenal tumors, 2 had extra-adrenal tumors, and 1 had a combined adrenal and extra-adrenal tumor. The patients included 17 with multiple endocrine neoplasia (MEN), 8 with von Hippel Lindeau disease (vHLD), and 4 with Von Recklinghausen disease (vRD). Two patients had malignant pheochromocytoma. All except one patient with MEN had thyroid carcinoma. The two patients with malignant pheochromocytoma had MEN II. All patients were managed by either open or laparoscopic adrenalectomy or excision of extra-adrenal tumors. There was no perioperative mortality. CONCLUSIONS: Associated conditions in patients with familial varieties are often the dominant or initial presentation. Presence of other manifestations of familial pheochromocytoma significantly influenced the clinical course and prognosis. However, it had no bearing on operative outcome of these patients.

Robotic augmentation enterocystoplasty.

Augmentation enterocystoplasty is an established procedure performed to increase bladder capacity and reduce intravesical pressure in patients with neurogenic bladder. Although the open surgical procedure remains the most widely accepted technique, laparoscopic enterocystoplasty has been described. As an extension of the minimally invasive approach, we describe a technique for robotic augmentation enterocystoplasty with a completely intracorporeal method. To our knowledge, this is the first report of such a technique.

The clinical spectrum of phaeochromocytoma: analysis of 115 patients.

OBJECTIVE: To analyse the presentation, manifestations and outcome in consecutive patients with phaeochromocytoma, as this disease has a wide range of pathological and clinical expressions. PATIENTS AND METHODS: The records of 115 patients with phaeochromocytoma were analysed retrospectively, recording the patients' age, sex, presenting symptoms and clinical signs, chemical, radiological and pathological findings and associated conditions. RESULTS: Of the 115 patients, 90 had adrenal tumours, 18 extra-adrenal and seven combined adrenal and extra-adrenal tumours. Ten patients had malignant and 105 had benign phaeochromocytoma. Eighty-six patients had sporadic and 29 had familial phaeochromocytoma, comprising eight with von Hippel-Lindau (VHL) disease, 17 with multiple endocrine neoplasia type II (MEN II) and four with von Recklinghausen disease. Two patients with sporadic phaeochromocytoma had Grave's disease. Ten patients (8.7%) had malignant phaeochromocytoma, of whom two had MEN II. A pregnant woman required prolonged intensive-care management before adrenalectomy and lost a fetus. CONCLUSION: Phaeochromocytoma is an interesting clinical entity with a wide spectrum of pathological and clinical manifestations. The diagnosis of phaeochromocytoma is confirmed by chemical methods, and located using imaging techniques, with computed tomography, magnetic resonance imaging and (131)I-meta-iodobenzyl guanidine radioisotope scanning being the most common. This series reflects the pathological and clinical spectrum of phaeochromocytoma. The presence of other manifestations of familial phaeochromocytoma influenced the presentation and prognosis of these patients.