Adrenal extramedullary hematopoiesis: report on a pediatric case and update of the literature.

The authors report on a rare pediatric case of adrenal extramedullary hematopoiesis in a patient with beta-thalassemia disease. The lesion was clinically discovered as incidentaloma of the right adrenal gland and treated by surgery. Adrenal extramedullary hematopoiesis may clinically be detected as incidentaloma. Adrenal incidentalomas presenting with hematologic disorders, such as agnogenic myeloid aplasia and beta-thalassemia, need careful imaging as well as adrenal hormonal investigation in order to exclude malignancy and subclinical hypersecretory syndromes. Ultrasound or CT-FNA of the lesion are effective in finding out the disease.

Adrenal incidentalomas: surgical treatment in 28 patients and update of the literature.

INTRODUCTION: Adrenal masses discovered by imaging techniques for reasons unrelated to adrenal diseases are called adrenal incidentalomas (Al). The aim of this study was to find out the clinical outcome of 28 patients operated for incidentally discovered adrenal mass and to update the literature concerning this topic. PATIENTS AND METHODS: From September 1976 to December 1999 we operated on 28 patients for adrenal incidentaloma. Adrenal masses were unilateral in 25 cases and bilateral in 5. Average age was 57 years (range 10-73). Hormonal study was performed in all patients. All patients underwent adrenalectomy by the transabdominal subcostal approach. RESULTS: Histopathology assessed the adrenal masses as primary in 19 patients and secondary in 9. 24-hour urinary vanillylmandelic acid (VMA) excretion was elevated in 2 patients. Adrenal insufficiency was detected in 1 case. Average tumor diameter resulted 5.8 cm (range 2-17). Histopathologic features of primary adrenal masses included pheochromocytoma in 5 cases, cysts in 4, myelolipomas in 3. nodular hyperplasia in 2, tuberculous mass in 1, cortical adenoma in 1, extra-bone marrow hemopoiesis in 1, cortical carcinoma in 1 and neuroendocrine tumor of the adrenal medulla in 1. The 9 adrenal metastasis resulted by renal cell carcinoma in 7 patients, urothelial carcinoma of the upper urinary tract in I and primary renal lymphoma in 1. Average follow-up was 68 months (range 6-246). Patients alive were 18 (64%), deal 10 (36%). Of the 19 patients with primary adrenal tumors 16 (84%) were alive and disease free and 3 (16%) died (I for disease and 2 for reasons unrelated to the primary tumor). Of the 9 patients with adrenal metastasis 2 (22%) were alive (I disease free and I with progression of the disease) and 7 (78%) died for disease. Replacement therapy for adrenocortical hormones was given 5 patients. CONCLUSIONS: Management of Al need CT or MRI and hormonal investigation in order to detect malignancy and subclinical hypersecretory syndromes. Subclinical functional adrenal masses, single adrenal metastasis and primary nonhypersecretory adrenal tumors sized 4 cm are treated by surgery. A close morpho-functional follow-up is indicated for primary adrenal incidentalomas when nonhypersecretory and smaller than 4 cm.

[Polyorchidism: 2 case reports]. / Poliorchidismo: descrizione di due casi.

Polyorchidism is a rare anomaly with approximately 70 cases reported in literature. The exact explanation for the production of polyorchidism is not known, although several theories have been proposed, including anomalous appropriation of cells, initial longitudinal duplication of the genital ridge and transverse division of the genital ridge, either through some local accident of development of peritoneal bands. A functional classification based upon the embryogenic development is provided. Type I: the supernumerary testis lacks an epididymis and vas. The split-off part of the primordial gonad does not communicate with the mesonephric tubules from which the epididymis develops. Type II: the supernumerary testis is linked to the regular testis by a common epididymis and shares a common vas with it. The division of the genital ridge occurs in the region where the primordial gonads are attached to the mesonephric ducts, although the latter are not divided (incomplete division). Type III: the supernumerary testis has its own epididymis but shares the vas with the regular testis. This variant results from a complete transverse division of the genital ridge. In the majority of the reported cases, the patients are asymptomatic and have painless groin or testicular masses. Approximately 50% occur as maldescent or cryptorchidism, and about 30% are associated with indirect hernia. The remaining 20% are discovered variously in relation to torsion, or are associated with hydrocele, epididymitis, varicocele or infertility. Moreover, since there is a 20 to 40 fold increase in testicular malignancy in patients with cryptorchidism compared with the normal testis, tumours of the supernumerary testicles are not unusual. We reported two cases of polyorchidism: the first patient is probably a longitudinal division of the genital ridge and the second is a completely duplication of the primordial gonads. The patients described vague, intermittent, testicular pain. Physical examination and the scrotal sonography and magnetic resonance revealed in the first patient a supernumerary testis in the right scrotal space and in the second a bilateral double testis. In conclusion we think that in the absence of any concomitant disorder and if testicular tumor can be ruled out by ultrasonography and magnetic resonance imaging, surgical exploration with biopsy is unnecessary.

[Fournier's gangrene: report of 2 cases and review of the literature]. / Gangrena di Fournier: osservazione di due casi e revisione della letteratura.

Fournier's gangrene is an extensive fulminant infection of the genitals, perineum or the abdominal wall. Since the first description by Jean Alfred Fournier in 1883 about 700 cases have been reported in the literature. The main aetiological factors are: perianal, perirectal or periurethral infections, diabetes mellitus and chronic alcoholism. Many aerobic and anaerobic organisms may be involved. Mortality rates range from 30-50%. The dramatic course of Fournier's gangrene requires early recognition, surgical drainage, extensive surgical debridment, antimicrobial therapy, hyperbaric oxygen therapy, as well as intensive care treatment in order to prevent irreversible endotoxic shock. We report two cases of Fournier's gangrene. In both cases symptoms were fever, pain and extensive cutaneous necrosis in the scroto-perineal region.

[Histopathologic risk factors in patients with non-seminomatous germ tumors of the testis in clinical stage 1. Retrospective study of 75 patients]. / Fattori istopatologici di rischio in pazienti con tumore germinale non seminomatoso del testicolo in stadio clinico 1. Uno studio retrospettivo su 75 pazienti.

OBJECTIVES: This retrospective study was performed to evaluate histopathologic prognostic risk factors in 75 patients on clinical stage 1 nonseminomatous germ cell cancer of the testis (NSGCTT). METHODS: From September 1976 to February 2000 we operated on 75 patients for NSGCTT on clinical stage 1 disease. Average age was 29.5 years (range 16-71). After orchiectomy, therapeutic options included retroperitoneal lymph node dissection (RLND) for 44 patients (58.6%), surveillance for 26 (34.6%) and neoadjuvant chemotherapy for 5 (6.6%). Testis primary tumor samples were assessed for studying prognostic risk factors that included vascular and/or lymphatic invasion (IV/IL+), percentage of embryonal carcinoma (%EC) and absence of yolk sac tumor (YS-). RESULTS: All patients were alive and disease-free. The average age follow-up was 84.5 months (range 1-254). Relapses occurred in 11 (14.6%) patients after an average follow-up of 9.09 months (range 3-24). Prognostic risk factors were detected as follows: IV/IL+ in 17 cases (22.7%), (50-80%) EC in 23 (30.6%), CE% > 80 in 23 (30.6%), YS- in 55 (72%). In 8 (10.6%) patients there was not any prognostic risk factor. Disease relapse related to prognostic risk factors was detected as follows: 18.1% for VI/LI, 90.9% for EC% > 50 (27.2% for 50-80% EC and 63.6% for CE% > 80) and 90.9% for YS-. Relapsing rates between patients with EC% > 80 and 50-80% EC resulted statistically significant (p = 0.02, odds ratio = 12.25). Relapsing rates between patients on surveillance and those who underwent RLND was next to be significant (p = 0.05, odds ratio 3.68). CONCLUSIONS: EC% > 80 is a prognostic risk factor for disease relapse in patients with clinical stage 1 NSGCT who are selected in a high risk group requiring RPLND or neoadjuvant chemotherapy as therapeutical option.

[Transitory postoperative anuria after transvesical prostatic adenomectomy: review of the literature, description of new clinical cases and physiopathological considerations]. / Anuria post-operatoria transitoria dopo adenomectomia prostatica transvescicale: revisione della letteratura, descrizione di nuovi casi clinici e considerazioni fisiopatologiche.

Anuria is a rare complication after prostate adenomectomy. Three cases of complete bilateral anuria and one case of monolateral anuria are reported following transvesical prostate adenomectomy, characterized by immediate onset without any apparent cause and by spontaneous resolution within 48 hours. After a thorough review of the literature, the authors analyze the pathophysiological and clinical aspects of post-prostate-adenomectomy anuria and postulate an "obstructive" aetiology for the anuria cases observed in their series.

[The complications of lumbotomies]. / Le complicazioni delle lombotomie.

After having described the surgical anatomy of the lumbar region, with particular regard to the relationships with pleura and peritoneum, as well as to the course of the nervous structures of the region (12th intercostal, ileohypogastric and ileoinguinal), the authors describe the possible complications related with the surgical approaches to the lumbar region. Pathogenesis of post-operatory pains and parietal hypotonies is especially evaluated; these complications may be caused by section, ligature or compression of one or more nervous trunks. The authors conclude that the respect of the anatomic structures located in the lumbar region represents the best way in order to prevent most complications related to lumbotomies.

[Transperineal TRUS-guided prostate biopsy]. / La biopsia prostatica transperineale TRUS guidata

Prostate biopsy can be performed through different approaches, with several differences in patient preparation, procedural technique and post-biopsy patient management. On the basis of our personal experience, and comparing it with literature data, this paper presents our data reviews regarding enema and antibiotic prophylaxis administration, biopsy technique, prostate sampling, core pathological management and post-operative management. We also provide a possible standardization of these procedures in patients undergoing transperineal TRUS-guided prostate biopsy. We accordingly classify antibiotic prophylaxis and bowel preparation as optional/advisable, ultrasound prostate examination before biopsy and local anaesthesia as recommended. Prostate sampling should be performed with at least 8 cores, to be increased proportionally to prostate volume. Each sample should be sent to the pathologist in single containers, according to the pre-embedding sandwich method. Finally, the patient should be evaluated for early complications before discharging, and for delayed complications within one month after the procedure.

[Conservative surgical treatment of bilateral renal neoplasms]. / Il trattamento chirurgico conservativo delle neoplasie renali bilaterali.

Surgery seems to be the only effective treatment for bilateral kidney neoplasms as shown by the better survival rate of operated patients compared with those otherwise treated or untreated at all. The best conservative treatments, regarding radicality and security required by these neoplasms, are the enucleation and the partial in situ nephrectomy. Our study is based on 11 patients affected by bilateral kidney neoplasms; 8 of them, in which there were no signs of metastatization, have undergone surgical treatment. In 2 patients (18%) a bilateral enucleation was performed and in 6 (55%) monolateral enucleation together with a controlateral radical nephrectomy. All tumors developed simultaneously have been operated in the same session. No postoperative complications were observed except in 2 patients which developed a transient renal insufficiency. The follow up was from 2 to 66 months. All patients were examined with echotomography or CT scan at least once a year and are, up today, in good health condition and tumor free.

High-energy microwave thermotherapy in the treatment of benign prostatic hyperplasia.

INTRODUCTION: Transurethral microwave thermotherapy is an anesthesia-free, outpatient method of treating lower urinary tract symptoms due to benign prostatic hyperplasia (BPH). Our results with the use of this technique in 25 patients are reported. MATERIALS AND METHODS: Twenty-five patients with BPH, 8 of whom with complete urinary retention, were treated with high-energy transurethral microwave thermotherapy (HE-TUMT) (Prostatron system). Preoperative investigations included digital rectal examination, urinary free flow rate, PSA, urinalysis, urine culture, transrectal ultrasonography, urodynamic evaluation, International Prostatic Symptom Score (IPSS) and quality of life (QoL). Main selection criteria included: large prostate, high surgical risk, reluctance to undergo surgery. All patients were obstructed according to the Abrams-Griffith's nomogram. For the statistical analysis a repeated-measures, one-way ANOVA was performed on previously non-catheterized patients. RESULTS: Six of the 8 patients with catheter before treatment were able to urinate spontaneously with no significant post-voiding residual urine. In the 17 remaining patients, IPSS decreased from a mean of 18.5 at baseline to 7.30 and QoL from a mean of 3.9 to 1.2. Mean maximum flow rates during uroflowmetry increased from 8.5 to 16.9 ml/s. P(det) Q(max) decreased from a mean of 83.0 cm H(2)O at baseline to 50.7 cm H(2)O and Q(max) increased from a mean of 6.8 ml/s at baseline to 15.1 ml/s during the pressure-flow study. After TUMT, 13 patients were unobstructed and 4 equivocal according to the Abrams-Griffith's nomogram. CONCLUSION: Our study performed in a selected population of patients with BPH documents the efficiency and safety of HE-TUMT. This technique appears to be a valid therapeutic option, particularly in patients with high surgical risk.

Renal vein and vena cava involvement does not affect prognosis in patients with renal cell carcinoma.

OBJECTIVES: The prognostic value of tumor extension into the renal vein or vena cava is still a controversial issue. The aim of this study is to report our experience with radical surgery in patients with renal cell carcinoma (RCC) extending into the renal vein or subdiaphragmatic vena cava. METHODS: We evaluated 142 patients with RCC involving the renal vein or inferior subdiaphragmatic vena cava. RCC had extended into the renal vein in 118 patients and into the inferior vena cava in the remaining 24. Radical nephrectomy was performed in all cases with renal vein invasion. Radical nephrectomy with cavotomy and tumor thrombus removal was carried out in all cases with inferior subdiaphragmatic vena caval invasion. Cause-specific survival was calculated by means of the Kaplan-Meier method. The log rank test was used for survival comparisons and univariate analysis. RESULTS: The 5- and 10-year cause-specific survival rates were 51.5 and 39%, respectively, in the group of patients with tumor extension into the renal vein and 33.4% in those with inferior vena caval involvement. In 52 patients (44%), RCC extended only into the renal vein. In the remaining 66 patients, renal vein invasion was associated with other adverse prognostic factors. Life expectancy was lower for patients with other concurrent adverse prognostic factors than for those affected by renal vein involvement alone (p < 0.0001). In the latter group, survival expectancy was similar to those with stage T2N0M0 tumor. In 7 cases (29%), inferior vena caval invasion was not associated with other adverse prognostic factors. In the remaining 15 patients (71%), vena caval involvement was associated with other adverse prognostic factors. Concurrence of other adverse prognostic factors with vena caval invasion significantly decreased the disease-specific survival expectancy in comparison with the patients in whom vena caval involvement was the main prognostic factor (p = 0.008). In these patients, disease-specific survival was similar to those with stage T2N0M0 tumor. CONCLUSION: Renal vein or inferior subdiaphragmatic vena caval involvement does not significantly affect prognosis in patients with RCC.

Primary lymphoma of the epididymis: case report and review of the literature.

OBJECTIVE: To report an extremely rare clinical pathological observation of a case of primary lymphoma of the epididymis, without testicular or systemic involvement, and to update the relevant literature. MATERIALS AND METHODS: A 25-year-old white male patient complaining of right scrotal pain was referred to our department. Clinical examination detected a hard painful mass at the right epididymal head. Epididymitis was diagnosed and conservative therapy with antibiotics and anti-inflammatory drugs was given. After 2 months of therapy the patient was admitted to our department because a tumor was suspected. Tumor markers were normal. Right scrotal exploration was performed through a standard inguinal incision. The epididymal head was completely replaced by a hard white mass. Fresh frozen sections indicated a malignant tumor. Right radical orchiectomy was performed. RESULTS: High-grade primary epididymal non-Hodgkin's lymphoma with diffuse large cells (group G according to the Working Formulation) was diagnosed. Clinical pathological staging detected stage IE (extranodal) primary epididymal lymphoma. The patient was referred to the Hematologic Unit for combined chemotherapy, according to the VACOP-B protocol. After an 18-month follow-up the patient is well and disease free. CONCLUSIONS: When an epididymal mass does not benefit from medical treatment, scrotal exploration and fresh frozen sections of the lesion should be done. The possible bilateral involvement by primary epididymal lymphoma has to be kept in mind. Radical orchiectomy is the treatment of choice for primary lymphoma of the epididymis. Adjuvant chemotherapy is indicated in high-grade malignant lymphoma. Prognostic parameters of the disease may be the grade of malignancy and the size of the tumor.