Long-Term Results after Intraocular Surgery in Treated Retinoblastoma Eyes.

Introduction: The aim of the study was to analyze the results of intraocular surgery in treated retinoblastoma eyes and to assess the long-term results with a priority on local recurrences, secondary enucleation, and metastases. Methods: Retrospective noncomparative case series. Results: From March 1964 to January 2020, 42 eyes of 40 retinoblastoma patients underwent intraocular surgery. Time interval between the last therapy and surgery was 9.5 years (mean: 114 months; median: 54.5 months). 31 eyes were treated for radiogenic cataract formation with a gain in visual acuity of 61.3%. One child developed an upper eyelid metastasis, 3 showed second primary malignancies (SPM), one a late recurrence, and 2 eyes were enucleated. Retinal surgery was performed in 17 eyes; 6 eyes were done as a combined procedure. Indications were radiogenic complications in the sense of a vitreous hemorrhage in 11 eyes and a rhegmatogenous retinal detachment in 6 eyes. 41.2% of the treated eyes had a postoperative gain in visual acuity, whereas 9.5% of the eyes could not be preserved in the long term. Regarding systemic involvement 2 patients developed late recurrences and one a SPM. Conclusion: Surgical therapy in treated retinoblastoma is necessary in isolated cases. In our series, cataract surgery was a safe procedure with a good option of a significant increase in visual acuity. As expected, vitreoretinal treated eyes showed a limited gain in visual acuity, a higher risk of late recurrences, and a lower globe retention rate. Therefore, a careful indication and individual risk-benefit analysis are mandatory.

Feasibility of Proton Beam Therapy as a Rescue Therapy in Heavily Pre-Treated Retinoblastoma Eyes.

Despite the increased risk of subsequent primary tumors (SPTs) external beam radiation (EBRT) may be the only therapeutic option to preserve a retinoblastoma eye. Due to their physical properties, proton beam therapy (PBT) offers the possibility to use the effectiveness of EBRT in tumor treatment and to decisively reduce the treatment-related morbidity. We report our experiences of PBT as rescue therapy in a retrospectively studied cohort of 15 advanced retinoblastoma eyes as final option for eye-preserving therapy. The average age at the initiation of PBT was 35 (14-97) months, mean follow-up was 22 (2-46) months. Prior to PBT, all eyes were treated with systemic chemotherapy and a mean number of 7.1 additional treatments. Indication for PBT was non-feasibility of intra-arterial chemotherapy (IAC) in 10 eyes, tumor recurrence after IAC in another 3 eyes and diffuse infiltrating retinoblastoma in 2 eyes. Six eyes (40%) were enucleated after a mean time interval of 4.8 (1-8) months. Cataract formation was the most common complication affecting 44.4% of the preserved eyes, yet 77.8% achieved a visual acuity of >20/200. Two of the 15 children treated developed metastatic disease during follow-up, resulting in a 13.3% metastasis rate. PBT is a useful treatment modality as a rescue therapy in retinoblastoma eyes with an eye-preserving rate of 60%. As patients are at lifetime risk of SPTs consistent monitoring is mandatory.

Differentiating Benign from Malignant Adrenocortical Tumors by a Single Morphological Parameter-a Clinicopathological Study on 837 Adrenocortical Neoplasias.

The morphological differentiation between benign and malignant adrenocortical tumors is an ongoing problem in diagnostic pathology. In recent decades the complex scoring systems have been widely used to calculate the probability of malignancy in adrenocortical tumors on the basis of a variety of histomorphological parameters. We herewith present a substantially simplified method to diagnose adrenocortical carcinoma by a single histomorphological parameter on a consecutive series of more than 800 adrenocortical tumors. Between January 2000 and May 2019, altogether 2305 adrenalectomies for of all types of diseases were removed, approximately 98% by minimally invasive approaches. After exclusion of pheochromocytomas, adrenal ganglioneuromas, adrenal metastases, Cushing's disease related specimens, and Conn's adenomas, the present series finally consisted of 837 adrenocortical tumors. All tumors were analyzed by experienced pathologists of a single institution using standard histopathological methods (Hematoxylin-Eosin and Ki67 stained sections). Clinical and histopathologic data were prospectively collected and retrospectively analyzed. Clinically, 385 patients had 420 functioning tumors (FT), and 417 had non-functioning adrenal tumors (NFT). The mean size of FT was 3.8 ± 1.4 cm (range 0.5-16 cm) and for NFT 4.5 ± 1.6 cm (range 1.5-18 cm). Histomorphologically, 32 adrenal tumors were classified as adrenocortical carcinoma (ACC; 3.8%). In all 32 cases (tumor size 9.1 ± 4.0 cm, range 3-18 cm), confluenting tumor necrosis could be demonstrated. The remaining 805 tumors (control group) completely lacked this highly reproducible single morphological feature. Ki67 levels above 10% were found in 31 of 32 ACCs and never in adrenocortical adenomas (ACA). With a mean follow-up of 8.2 years, 24 out of 32 patients primarily diagnosed as ACC developed distant metastases (75.0%), whereas all patients in the control group remained free of local or distant recurrence. We conclude that a single morphological parameter (confluenting tumor necrosis) is sufficient to predict a poor clinical course in adrenocortical tumors. The histomorphological diagnosis of this parameter is straightforward and highly reproducible.

Long-Term Clinical Results and Management following Vitrectomy in Undetected Retinoblastoma Eyes.

INTRODUCTION: Given the rarity of retinoblastoma and the consequences of accidental vitrectomy in the event of misdiagnosis, reporting on clinical experience in this area is important. OBJECTIVE: The aim of this study was to analyse the management and complications with a focus on local orbital recurrence and metastatic disease in 10 children vitrectomized in an undetected retinoblastoma eye. METHODS: This is a retrospective descriptive case series conducted in a single-centre referral university hospital. RESULTS: From October 1991 to June 2019, 10 patients with a vitrectomy in an unsuspected retinoblastoma eye were included in this study. The main preoperative diagnoses were unilateral inflammation with a suspected lymphoma, uveitis or toxocariasis in 5 cases, vitreous haemorrhage after trauma in 2 cases, and the last 3 were misdiagnosed with Coats disease, rhegmatogenous retinal detachment and congenital cataract. Mean age at surgery was 3 years, ranging from 14 months to 6 and a half years. Nine patients were suffering from unilateral retinoblastoma; these were enucleated and treated with 4-6 cycles of chemotherapy and/or radiation therapy. The sclerotomy sites were infiltrated with tumour cells in 3 cases. In 1 patient, the differential diagnosis of a malignant medulloepithelioma could not be excluded. One patient had bone marrow infiltration on initial presentation; all other patients are healthy without any signs of orbital recurrence or metastatic disease with a mean follow-up of 5.4 years. CONCLUSION: In children, intraocular tumours, including retinoblastoma and medulloepithelioma, should be ruled out before pars plana vitrectomy is performed. If no doubtless preoperative diagnosis can be established, preoperative magnetic resonance imaging is mandatory. If a vitrectomy in a retinoblastoma eye has been performed, immediate enucleation of the eye with subsequent chemotherapy and orbital radiation is effective to avoid local recurrence and systemic metastases.

Histopathology of retinoblastoma eyes enucleated after intra-arterial chemotherapy.

BACKGROUND: To demonstrate histopathological findings in retinoblastoma eyes enucleated after intra-arterial chemotherapy (IAC) with special emphasis on vascular toxicity and local tumour control. METHODS: Retrospective study with a consecutive series of 23 retinoblastoma eyes enucleated after IAC where histopathological work-up was available. RESULTS: From November 2010 to June 2019 23 eyes were enucleated after the attempt of eye salvaging therapy with IAC using melphalan. IAC was the first line treatment in nine and salvage treatment in 14 eyes. Doses of melphalan ranged from 3 to 7.5 mg, whereby a strict protocol with age-appropriate dosage was not used until 2015. The mean number of treatment cycles was 1.8. The main indications for enucleation were poor treatment response or tumour progression in 14 eyes, severe vascular complications in five eyes and a total exudative retinal detachment with amaurosis in the remaining four eyes. We found active disease in 15 eyes with an indication for adjuvant chemotherapy due to high risk factors for metastases in four eyes. To date none of these patients developed metastatic disease. Concerning vascular toxicity, we detected a central retinal artery occlusion in three eyes, severe vasculitis in another three, ischaemic outer retina atrophy and choroidal ischaemia in seven eyes with one eye developing a severe proliferative retinopathy. CONCLUSION: IAC is a highly effective treatment option for advanced retinoblastoma, but the described potential risks should be kept in mind. These include severe vascular complications, as well as the possibility of persisting vital tumour cells fulfilling high-risk criteria for adjuvant chemotherapy.

Suspected vitreous seeding of uveal melanoma: relevance of diagnostic vitrectomy.

AIM: To review all cases of suspected vitreous seeding of treated or untreated uveal melanoma at our clinic and to compare clinical, cytological and histological findings with patients' survival. METHODS: Retrospective non-randomised study of 23 patients with consecutive uveal melanoma who underwent diagnostic vitrectomy in our clinic between January 2000 and November 2013. Reason for vitrectomy was suspected dissemination of tumour cells inside the eye. Treated as well as treatment-naïve primary uveal melanomas were included in this study. Follow-up data of all patients were collected. RESULTS: The study included 23 patients with a mean age of 66 years. Four patients presented pigmented vitreous debris at initial presentation prior to treatment of the uveal melanoma. All but one of these four patients has been enucleated as a consequence of cytology-proven vitreous spreading of vital melanoma cells. The remaining 19 patients presented pigmented vitreous debris at a mean of 60 months following local tumour treatment. Thirteen of these patients had been treated with a ruthenium plaque (mean scleral dose 1295 Gy, mean apex dose 152 Gy), three with binuclid plaque (mean scleral dose 1005 Gy, mean apex dose 70 Gy) and three with proton beam radiation. Of the 19 patients, 10 showed only melanophages in the vitreous specimen, while the remaining 9 patients had vital tumour cells in vitreous cytology. Four out of these nine patients have been enucleated in the course of follow-up. During follow-up of our cohort of 23 patients, 4 patients died, but only 1 of them due to metastatic disease. CONCLUSION: The outcome of this small cohort study shows that obtaining a vitreous specimen helps to distinguish melanophages from vital tumour cells. We could not observe an increased risk of metastasis in patients who showed melanoma cell dissemination inside the eye, compared with those patients only showing melanophages. We therefore suggest to carefully re-evaluate the necessity of enucleation in every patient.

High-Resolution Magnetic Resonance Imaging Can Reliably Detect Orbital Tumor Recurrence after Enucleation in Children with Retinoblastoma.

PURPOSE: Orbital tumor recurrence is a rare but serious complication in children with retinoblastoma, leading to a high risk of metastasis and death. Therefore, we assume that these recurrences have to be detected and treated as early as possible. Preliminary studies used magnetic resonance imaging (MRI) to evaluate postsurgical findings in the orbit. In this study, we assessed the diagnostic accuracy of high-resolution MRI to detect orbital tumor recurrence in children with retinoblastoma in a large study cohort. DESIGN: Consecutive retrospective study (2007-2013) assessing MRI findings after enucleation. PARTICIPANTS: A total of 103 MRI examinations of 55 orbits (50 children, 27 male/23 female, mean age 16.3±12.4 months) with a median time of 8 months (range, 0-93) after enucleation for retinoblastoma. METHODS: High-resolution MRI using orbital surface coils was performed on 1.5 Tesla MRI systems to assess abnormal orbital findings. MAIN OUTCOME MEASURES: Five European experts in retinoblastoma imaging evaluated the MRI examinations regarding the presence of abnormal orbital gadolinium enhancement and judged them as "definitive tumor," "suspicious of tumor," "postsurgical condition/scar formation," or "without pathologic findings." The findings were correlated to histopathology (if available), MRI, and clinical follow-up. RESULTS: Abnormal orbital enhancement was a common finding after enucleation (100% in the first 3 months after enucleation, 64.3% >3 years after enucleation). All histopathologically confirmed tumor recurrences (3 of 55 orbits, 5.5%) were correctly judged as "definitive tumor" in MRI. Two orbits from 2 children rated as "suspicious of tumor" received intravenous chemotherapy without histopathologic confirmation; further follow-up (67 and 47 months) revealed no sign of tumor recurrence. In 90.2%, no tumor was suspected on MRI, which was clinically confirmed during follow-up (median follow-up after enucleation, 45 months; range, 8-126). CONCLUSIONS: High-resolution MRI with orbital surface coils may reliably distinguish between common postsurgical contrast enhancement and orbital tumor recurrence, and therefore may be a useful tool to evaluate orbital tumor recurrence after enucleation in children with retinoblastoma. We recommend high-resolution MRI as a potential screening tool for the orbit in children with retinoblastoma to exclude tumor recurrence, especially in high-risk patients within the critical first 2 years after enucleation.

Hereditary Diffuse Infiltrating Retinoblastoma.

Retinoblastoma is one of the most common childhood cancers. The diffuse infiltrating retinoblastoma is a rare subtype of this neoplasm. The majority of cases of diffuse infiltrating retinoblastoma are unilateral and occur sporadically. Herein we report on a family with three children affected by retinoblastoma, among them one girl with diffuse infiltrating retinoblastoma. This girl was diagnosed at the age of 8 years with a unilateral diffuse infiltrating retinoblastoma. By contrast, the two brothers became clinically apparent in the first 2 years of life with bilateral retinoblastoma. The parents were clinically unremarkable. Genetic analysis of RB1 gene was performed. The girl with diffuse infiltrating RB was found to be heterozygous for an oncogenic mutation in the RB1 gene that was also carried by both brothers and the father of the family. These results show that diffuse infiltrating retinoblastoma can develop on the background of a hereditary predisposition to retinoblastoma.

Diagnostic Accuracy of Intraocular Tumor Size Measured with MR Imaging in the Prediction of Postlaminar Optic Nerve Invasion and Massive Choroidal Invasion of Retinoblastoma.

Purpose To assess the correlation of intraocular retinoblastoma tumor size measured with magnetic resonance (MR) imaging in the prediction of histopathologically determined metastatic risk factors (postlaminar optic nerve invasion and massive choroidal invasion). Materials and Methods The ethics committee approved this retrospective multicenter study with a waiver of informed consent. The study population included 370 consecutive patients with retinoblastoma (375 eyes) who underwent baseline MR imaging, followed by primary enucleation from 1993 through 2014. Tumor sizes (maximum diameter and volume) were measured independently by two observers and correlated with histopathologic risk factors. Receiver operating characteristic curves were used to analyze the diagnostic accuracy of tumor size, and areas under the curve were calculated. Logistic regression analysis was performed to evaluate potential confounders. Results Receiver operating characteristic analysis of volume and diameter, respectively, yielded areas under the curve of 0.77 (95% confidence interval [CI]: 0.70, 0.85; P < .0001) and 0.78 (95% CI: 0.71, 0.85; P < .0001) for postlaminar optic nerve invasion (n = 375) and 0.67 (95% CI: 0.57, 0.77; P = .0020) and 0.70 (95% CI: 0.59, 0.80; P = .0004) for massive choroidal tumor invasion (n = 219). For the detection of co-occurring massive choroidal invasion and postlaminar optic nerve invasion (n = 219), volume and diameter showed areas under the curve of 0.81 (95% CI: 0.70, 0.91; P = .0032) and 0.83 (95% CI: 0.73, 0.93; P = .0016), respectively. Conclusion Intraocular tumor size shows a strong association with postlaminar optic nerve invasion and a moderate association with massive choroidal invasion. These findings provide diagnostic accuracy measures at different size cutoff levels, which could potentially be useful in a clinical setting, especially within the scope of the increasing use of eye-salvage treatment strategies. (©) RSNA, 2015 Online supplemental material is available for this article.

High-resolution MRI using orbit surface coils for the evaluation of metastatic risk factors in 143 children with retinoblastoma: Part 1: MRI vs. histopathology.

INTRODUCTION: A reliable detection of metastatic risk factors is important for children with retinoblastoma to choose the right therapeutic regimen. First studies using high-resolution magnetic resonance imaging (MRI) with orbit surface coils were promising. The aim of this study was therefore to evaluate the ability of high-resolution MRI to detect metastatic and especially advanced metastatic risk factors in a large group of children with retinoblastoma. METHODS: One hundred forty-three consecutive children with retinoblastoma (148 enucleated eyes, 64 girls, 79 boys, mean age 19.7 ± 15.3) who received pretherapeutical high-resolution MRI with orbit surface coils on 1.5 T MR scanner systems between 2007 and 2012 and subsequent primary enucleation within 14 days were included in this retrospective study. Image analysis was performed by two neuroradiologists experienced in ocular imaging in consensus. Histopathology served as gold standard. RESULTS: Sensitivity/specificity for the detection of metastatic risk factors using high-resolution MRI with orbit surface coils were 60 %/88.7 % for postlaminar optic nerve infiltration, 65.5 %/95.6 % for choroidal invasion, 100 %/99.3 % for scleral invasion, and 100 %/100 % for peribulbar fat invasion, respectively. The results increased for the detection of advanced metastatic risk factors, 81.8 %/89.1 % for deep postlaminar optic nerve infiltration, 70.6 %/97.6 % for massive choroidal invasion. CONCLUSIONS: High-resolution MRI is clinically valuable for the detection of metastatic, especially of advanced metastatic risk factors in children with retinoblastoma.

High-resolution MRI using orbit surface coils for the evaluation of metastatic risk factors in 143 children with retinoblastoma: Part 2: new vs. old imaging concept.

INTRODUCTION: High-resolution magnetic resonance imaging (MRI) is recommended for the evaluation of metastatic risk factors in children with retinoblastoma according to recent guidelines. The aim of this study was to compare diagnostic accuracy of a new imaging concept with two orbit surface coils to that of an old imaging concept with one orbit surface coil. METHODS: One hundred forty-three patients (148 eyes, 64 girls, 79 boys) underwent high-resolution MRI on 1.5 T scanners using orbit surface coils. The old imaging concept (one orbit surface coil focusing on the (most) effected eye additionally to the standard head coil) was used in 100 patients/103 eye; the new imaging concept (two orbit surface coils (each focusing on one eye) additionally to the standard head coil) in 43 patients/45 eyes. Image analysis was performed by two neuroradiologists in consensus. Histopathology served as gold standard. RESULTS: Detection rate for choroidal invasion was higher for the new compared to that for the old imaging concept (sensitivity/specificity 87.5/94.6 % vs. 57.1/96.1 % for choroidal invasion and 100/97.5 % vs. 58.3/97.7 % for massive choroidal invasion, respectively). Sensitivity and specificity for the detection of postlaminar optic nerve infiltration, peribulbar fat, and scleral invasion were comparable in both imaging concepts; however positive predictive value was higher in the new imaging concept (new vs. old imaging concept: 60 vs. 31.6 % for postlaminar and deep postlaminar optic nerve infiltration, respectively, and 100 vs. 66.7 % for scleral invasion). CONCLUSION: The new imaging concept shows a trend towards improving the accuracy of detecting metastatic risk factors in children with retinoblastoma and is therefore recommended for pretherapeutic imaging and follow-up.

Diagnostic image quality of gadolinium-enhanced T1-weighted MRI with and without fat saturation in children with retinoblastoma.

BACKGROUND: Gadolinium-enhanced T1-weighted MRI without fat saturation has been recommended for assessment of retinoblastoma. OBJECTIVE: The purpose of this study was to compare diagnostic image quality without and with fat saturation following gadolinium administration. MATERIALS AND METHODS: High-resolution gadolinium-enhanced T1-weighted sequences with and without fat saturation performed in children with subsequently histopathologically confirmed retinoblastoma were included. Image analysis (image quality [1 = poor, 2 = moderate, 3 = good], anatomical detail depiction, tumour extension) was performed by two neuroradiologists in consensus. Enhancement was scored and measured. Signal- and contrast-to-noise ratios were calculated. Image-assessed tumour invasiveness was compared to histopathological findings. Paired sample t-test was used for statistical analysis. RESULTS: Thirty-six children (mean age, 19.0 ± 16.8 [SD] months) were included. Image quality and anatomical detail depiction were significantly better without fat saturation (P < 0.001). Tumour enhancement was rated higher with fat saturation (P < 0.001). Fat saturation improved detection of (post-)laminar optic nerve infiltration. Detection of choroidal invasion was improved without fat saturation. Combining both sequences was best in the assessment of tumour extension (sensitivity/specificity for (post-)laminar optic nerve infiltration, 75.0%/100.0%, and for choroidal invasion, 87.5%/85.7%). CONCLUSION: Combined T1-weighted spin-echo imaging with and without fat saturation improved the image quality for assessment of invasiveness of retinoblastoma.

Retroperitoneoscopic adrenalectomy in Conn's syndrome caused by adrenal adenomas or nodular hyperplasia.

BACKGROUND: In patients with primary hyperaldosteronism, solitary adrenal adenomas are an indication for surgical intervention. In contrast, adrenal hyperplasia is almost exclusively treated by drugs. PATIENTS AND METHODS: In a prospective clinical study 183 patients (81 men, 102 women; age 49.6+/-12.8 years) with Conn's syndrome were operated on using the posterior retroperitoneoscopic approach. Tumor size ranged from 0.2 to 5.0 cm (mean 1.5+/-0.8 cm). Final histology described a solitary adenoma in 127 patients and adrenal hyperplasia in 56 patients. Partial adrenalectomies were performed in 47 operations. RESULTS: The perioperative complication rate was 4%, mortality zero. In none of the cases was conversion to open surgery necessary. The mean operating time was 58+/-32 minutes (range 20-230 minutes) and was associated with sex (p<0.001) but not with the extent of resection (partial vs. total, p=0.51) or with tumor size (1.5 cm; p=0.43) or tumor site (p=0.77). Median blood loss was 15 ml. Median duration of postoperative hospitalization was 4 days. After a mean follow-up of nearly 5 years, 96% of patients are normokalemic, 30% of patients are cured (normotensive without medication), and 87% showed an improvement of hypertension (normotensive without or with reduced medication). Cure of hypertension depended on the patient's age (p<0.001) and sex (p<0.001), duration of hypertension (p<0.05), and histomorphology (p<0.001). Improvement of hypertension was not associated with any of these factors. CONCLUSIONS: Retroperitoneoscopic removal of adrenal glands in patients with Conn's syndrome is a safe, rapidly performed surgical procedure and can thus be considered as first choice option for treatment of both solitary adrenal adenomas and hyperplasia presenting with a clinically predominating nodule.

Diagnostic yield of computed tomography-guided coaxial core biopsy of undetermined masses in the free retroperitoneal space: single-center experience.

The purpose of this study was to evaluate the diagnostic yield of core biopsy in coaxial technique under guidance of computed tomography (CT) for retroperitoneal masses. We performed a retrospective analysis of CT-guided coaxial core biopsies of undetermined masses in the non-organ-bound retroperitoneal space in 49 patients. In 37 cases a 15-G guidance needle with a 16-G semiautomated core biopsy system, and in 12 cases a 16-G guidance needle with an 18-G biopsy system, was used. All biopsies were technically successful. A small hematoma was seen in one case, but no relevant complication occurred. With the coaxial technique, up to 4 specimens were obtained from each lesion (mean, 2.8). Diagnostic accuracy in differentiation between malignant and benign diseases was 95.9%. A specific histological diagnosis could be established in 39 of 42 malignant lesions (92.9%). Correct subtyping of malignant lymphoma according to the WHO classification was possible in 87.0%. Benign lesions were correctly identified in seven cases, although a specific diagnosis could only be made in conjunction with clinical and radiological information. In conclusion, CT-guided coaxial core biopsy provides safe and accurate diagnosis of retroperitoneal masses. A specific histological diagnosis, which is essential for choosing the appropriate therapy, could be established in most cases of malignancy.

Unusual aggressive course of a giant cell tumor of soft tissue during immunosuppressive therapy.

Giant cell tumor of soft tissue with low malignant potential (GCT-ST) is a low-grade, primary soft tissue sarcoma with histological and clinical features similar to giant cell tumor of the bone. The main tumor localizations are the extremities, but it may also occur in the head and neck region. GCT-ST shows a recurrence rate of approximately 15%, but it very rarely metastasizes. The risk of cancer development, especially of the skin, is up to fivefold increased in immunosuppressed patients after organ transplantation. The association of sarcomas and immunosuppressive therapy is best known for Kaposi sarcomas, whereas other types of sarcomas are rarely found. We report of a GCT-ST of low malignant potential, which developed under long-term immunosuppression in a patient 12 years after heart transplantation. The tumor presented with an unusual aggressive course and metastatic site: the parotid gland. Therefore, we suggest that in patients with immunosuppression, even low malignant cancerous lesions should be carefully observed, as their local behavior may be aggressive with development of metastasis.

Partial versus total adrenalectomy by the posterior retroperitoneoscopic approach: early and long-term results of 325 consecutive procedures in primary adrenal neoplasias.

The retroperitoneoscopic approach is a standardized operative procedure for primary adrenal gland tumors. It allows direct access with a detailed view of the adrenal gland. Thereby, a clear differentiation between normal and neoplastic adrenal tissue is often possible, which permits a planned partial resection of the gland in selected cases. Between July 1994 and November 2003 325 posterior retroperitoneoscopic adrenalectomies were performed for primary benign adrenal gland tumors (106 Conn's adenomas, 83 pheochromocytomas, 76 Cushing's adenomas, 60 nonfunctioning tumors; size: 2.8 +/- 1.5 cm; site: 160 right, 165 left) in 318 patients (122 M, 196 F, age: 49.0 +/- 14.3 years). In 96 patients 100 tumors were removed by partial adrenalectomy (30 Conn's adenomas, 33 pheochromocytomas, 20 Cushing's adenomas, 17 nonfunctioning tumors; site: 61 right, 59 left) maintaining tumor-free parts of the adrenal gland. Of this group, 15 patients suffered from bilateral adrenal neoplastic diseases. During the same period, 225 total adrenalectomies (76 Conn's adenomas, 50 pheochromocytomas, 56 Cushing's adenomas, 34 nonfunctioning tumors; site: 109 right, 116 left) were performed in 224 patients. There was no mortality. Major complications were seen in 1.8%, minor complications in 14.5%. Three conversions were necessary to an open or a laparoscopic approach (2 patients and 1 patient, respectively). There are no differences between the two groups (total versus partial adrenalectomy) with regard to tumor size (2.8 +/- 1.6 cm versus 2.8 +/- 1.5 cm), operating time (80 +/- 44 minutes versus 79 +/- 42 minutes), and blood loss (33 +/- 71 ml versus 29 +/- 31 ml). In all patients with partial adrenalectomy, biochemical healing was proven. Fourteen of 15 patients with bilateral diseases had preservation of adrenocortical function. After a mean follow up of 51 months (range: 7-120 months) local recurrence or relapse of the initial diseases was noticed in 6 patients after total adrenalectomy: in 4 patients with Conn's syndrome and bilateral hyperplasia, and in 2 patients with malignant pheochromocytoma and adrenocortical carcinoma, respectively. Our data demonstrate that partial adrenalectomy is a safe procedure not only perioperatively but also in the long-term follow-up.

Testicular, other genital, and breast cancers in first-degree relatives of testicular cancer patients and controls.

Previous studies showed an increased prevalence of testicular cancer among fathers and brothers of testicular cancer patients. We examined whether testicular, other genital, and breast cancers aggregate in parents and siblings of testicular cancer patients in a population-based case-control study, including males, ages 15 to 69 years at diagnosis, with primary malignant tumors of the testes or extragonadal germ cell tumors. Controls were ascertained through the mandatory registries of residents and frequency matched to the cases by age and region of residence. In a face-to-face interview, 269 cases and 797 controls provided health-related information on parents and siblings. We calculated odds ratios (OR) and corresponding 95% confidence intervals (95% CI) based on the generalized estimating equations technique, adjusting for the matching variables and relatives' age. Three (1.1%) fathers and eight (3.2%) brothers of cases were affected with testicular cancer compared with four (0.5%) fathers and two (0.2%) brothers of controls. The OR (95% CI) of familial testicular cancer was 6.6 (2.35-18.77). Only nonseminoma patients had fathers with testicular cancer, whereas the affected brothers were all related to seminoma patients. Overall, we found an increased risk for genital other than testicular cancers (OR 2.5, 95% CI 1.43-4.43). For breast cancer, we detected an increased risk in sisters (OR 9.5, 95% CI 2.01-45.16, adjusted for age of study participant and age of sister) but not in mothers. Our findings support the hypothesis that testicular and other genital cancers have a common familial component that may be due to genetic and shared exogenous factors such as estrogen exposure during fetal development.

Hemangioendothelioma of the liver associated with a solitary extrahepatic lesion.

Epithelioid hemangioendothelioma of the liver is very uncommon and is quite difficult to distinguish from a cholangiocarcinoma or a metastatic liver disease by imaging diagnostics. However, the histological features are characteristic for this soft-tissue tumor. Although liver tumor is usually multifocal and a liver resection is not possible in most cases, we report a case of a multifocal epithelioid hemangioendothelioma of the liver that was completely resected. The tumor was associated with a solitary extrahepatic lesion, which was first discovered six months after the liver resection and which was also resected.

Intraoperative radiation therapy in liver tissue in a pig model: monitoring with dual-modality PET/CT.

PURPOSE: To assess, in a pig model, the value of dual-modality positron emission tomography (PET)/computed tomography (CT) for monitoring radiation therapy. MATERIALS AND METHODS: Central bile duct resection followed by creation of a biliodigestive anastomosis was performed in nine pigs. Six of these pigs were also treated with intraoperative radiation therapy (IORT) (20 Gy) in the area of the anastomosis. Two, 4, and 8 weeks postoperatively, contrast material-enhanced fluorine 18 fluorodeoxyglucose (FDG) PET/CT of the liver was performed in all of the animals. The radioactive tracer concentration in the region of the anastomosis was quantified, and the values were compared intraindividually with the values at the liver periphery. Histologic evaluation of the liver was performed 8 weeks postoperatively. The PET/CT images were assessed for changes in liver volume and bile duct diameter over time. RESULTS: In all nine pigs, the region of the anastomosis could be clearly defined on the fused PET/CT images. PET/CT revealed a decreased concentration of FDG in the irradiated field 2 and 4 weeks after IORT. At 8 weeks, however, the distribution of the tracer in the irradiated pigs did not differ from that in the nonirradiated pigs. Homogeneous tracer uptake in all liver regions was observed in the nonirradiated animals. The CT images showed an increase in liver volume in all pigs and bile duct dilatation that increased over time in the irradiated pigs. CONCLUSION: The morphologic and functional changes due to IORT in liver tissue can be accurately monitored with dual-modality PET/CT. By enabling the integration of functional and morphologic data, PET/CT may have an important role in monitoring radiation treatment.