Ruptured Tentorium Originating Masson Tumor.

Intravascular papillary endothelial hyperplasia (IPEH) also known as Masson's tumor, is a benign, slow growing, vascular lesion which is seen very rarely and only a few cases have been reported intracranially in the literature. It has been reported at many sites, but the posterior fossa involvement is very rare. The preoperative diagnosis is very difficult, as there is no enough cases to achieve a clear understanding about the details of its radiological findings. Differential diagnosis have to be made especially from angiosarcoma and meningioma. It is curable by total surgical removal. In this article we presented the characteristic clinical, radiological, perioperative and pathological findings in a case of IPEH in an unusual location, origin and behavior. To best of our knowledge, we presented the first case of IPEH originating from tentorium.

Hepatocellular adenomas in the Turkish population: reclassification according to updated World Health Organization criteria.

AIMS: Hepatocellular adenoma (HCA) is an uncommon liver neoplasm, and studies of HCA subtypes have been primarily limited to France, the USA, and Japan. The aim of this study was to describe the clinicopathological features of HCA subtypes in Turkey. METHODS AND RESULTS: The resection specimens of 59 cases diagnosed as 'hepatocellular adenoma' collected from 15 institutions were reviewed to confirm the diagnosis and to classify them according to the current World Health Organization 2019 classification. Immunostaining for glutamine synthetase, liver fatty acid-binding protein, C-reactive protein, ß-catenin and reticulin was performed. Of the 59 cases, 48 (81%) were diagnosed as HCA. We identified 24 (50%) hepatocyte nuclear factor 1α (HNF1α)-inactivated HCAs, five (10%) inflammatory HCAs, 15 (32%) ß-catenin-activated HCAs, three (6%) ß-catenin-activated inflammatory HCAs, and one (2%) unclassified HCA. HCA patients were predominantly female (female/male ratio of 5:1); they had a median age of 34 years and a median tumour diameter of 60 mm. In the ß-catenin-activated HCA group, nine cases (19%) showed cytoarchitectural atypia, and were also referred to as atypical hepatocellular neoplasms. In the ß-catenin-activated HCA group, three cases (6%) showed focal areas supportive of transition to HCA. The original diagnosis of HCA was changed to well-differentiated hepatocellular carcinoma in nine cases and to focal nodular hyperplasia in two cases. CONCLUSION: In our series, the major HCA subtype was HNF1α-inactivated HCA. We found a low incidence of inflammatory-type HCA. Our data also showed that ß-catenin-activated hepatocellular neoplasms, including cases with atypical histology, constituted a relatively high proportion of the cases. These findings are in contrast to those of most other studies of HCA subtypes.

ATA Index: A novel score for predicting fibrosis stage in chronic viral hepatitis.

Background and Aim: This study was designed to predict the fibrosis stage with a clinical scoring system that may reduce the need for liver biopsy. Materials and Methods: The study cohort included the treatment of 430 chronic hepatitis B (CHB) and 170 chronic hepatitis C (CHC) of naive patients. The patients were divided into two groups as mild to moderate and severe fibrosis. After an index obtained in the study cohort, the index was tested in a validation cohort and compared with the FIB-4 Index. Results: The AUC of CHC index was found of 0.89 the sensitivity of 0.91 the specificity of 0.74, the positive predictive value (PPV) of 0.54 and the negative predictive value (NPV) of 0.96. The FIB-4 Index was applied to the CHC study cohort and the ATA Index Hepatitis C was found to be superior in terms of AUC (0.89-0.82), sensitivity (0.91-0.76) and NPV (0.96-0.86). The AUC of CHB Index was determined of 0.92, the sensitivity of 0.90, the specificity of 0.84, the PPV of 0.53 and the NPV of 0.98. Compared to the FIB-4 Index in CHB study cohort, the ATA Index Hepatitis B was predominant in terms of AUC (0.92-0.88), sensitivity (0.90-0.75), NPV (0.98-0.94) and PPV (0.53-0.49). Conclusion: ATA Indexes can predict the non-existence of severe fibrosis with an accuracy similar to FIB-4 Index and may reduce the need for liver biopsy.

The Contribution of Additional Sampling in Cholecystectomy Materials: A Multicenter Prospective Study.

OBJECTIVE: Cholecystectomy materials are frequently encountered in routine practice. The aim of this study was to determine the true frequency of gallbladder lesions, the diagnostic consistency, and standardization of reports after macroscopic sampling and microscopic evaluation based on previously defined criteria. MATERIAL AND METHOD: 14 institutions participated in the study within the Hepato-Pancreato-Biliary Pathology Study Group. Routinely examined cholecystectomies within the last year were included in the study in these institutions. Additional sampling was performed according to the indications and criteria. The number of blocks and samples taken in the first macroscopic examination and the number of blocks and samples taken in the additional sampling were determined and the rate of diagnostic contribution of the additional examination was determined. RESULTS: A total of 5,244 cholecystectomy materials from 14 institutions were included in the study. Additional sampling was found to be necessary in 576 cases (10.98%) from all institutions. In the first macroscopic sampling, the mean of the numbers of samples was approximately 4 and the number of blocks was 2. The mean of the numbers of additional samples and blocks was approximately 8 and 4, respectively. The diagnosis was changed in 144 of the 576 new sampled cases while the remaining 432 stayed unaltered. CONCLUSION: In this study, it was observed that new sampling after the first microscopic examination of cholecystectomy materials contributed to the diagnosis. It was also shown that the necessity of having standard criteria for macroscopic and microscopic examination plays an important role in making the correct diagnosis.

Effects of adipose derived stromal vascular fraction on diabetic neuropathy: an experimental study.

The stromal vascular fraction (SVF) obtained from inguinal adipose tissue was injected into the sciatic nerve region in diabetic rats. The effects of the SVF on the sciatic nerve and functional, electrophysiological and histopathological changes were examined in this study. Rats were divided into five groups; a non-diabetic control group, and four diabetic groups. In the first diabetic rat group, the SVF was obtained from inguinal adipose tissue. The remaining diabetic groups included a sham control group, a phosphate-buffered saline (PBS) injection group and an SVF injection group. Injections were made into the sciatic nerve region. Electromyography and walking track analyses were conducted on all groups at the beginning of the experiment. Diabetes was induced via a single dose of streptozotocin. Walking track analysis and electromyography measurements were repeated in week 8. SVF or PBS was injected into the right sciatic nerve region on week 8 of experiment group rats. Walking track analysis and electromyography were repeated in week 12 and all sciatic nerves were examined histopathologically. In the diabetic SVF group, the sciatic functional index calculated from walking track analysis in week 12 was better than week 8. Additionally, the myelin sheaths of the right sciatic nerve were thicker and more uniform and the nerve fibers were thicker than those of the left, untreated sciatic nerve. No statistical differences were detected in electromyographic measurements. The adipose-derived SVF may be beneficial for nerve regeneration in diabetic neuropathy.

The Prognostic Roles of the Ki-67 Proliferation Index, P53 Expression, Mitotic Index, and Radiological Tumor Invasion in Pituitary Adenomas.

The fourth edition of the World Health Organization (WHO) classification of pituitary tumors recommended evaluation of tumor proliferation and invasion to identify aggressiveness. We aimed to assess the prognostic roles of the Ki-67 proliferation index, mitotic index, P53 expression, and cavernous sinus invasion in pituitary adenomas (PAs). Among the 601 patients who underwent transnasal/transsphenoidal adenomectomy from 2001 to 2016, 101 patients (16.8%) who had tumors with a high (≥ 3%) Ki-67 index (group A) and a control group consisting of 43 patients with a low (< 3%) Ki 67 index who were matched for age, gender, and tumor type were included. Mitotic index and P53 expressions were evaluated. Patient characteristics, histopathology reports, pre/postoperative magnetic resonance imaging (MRI), and follow-up data were assessed retrospectively. The frequency of macroadenomas and mean tumor size were greater in group A when compared to group B (67.4 vs. 94.1%, p< 0.01 and 25 ± 10.6 vs. 18 ± 11 mm, p< 0.01, respectively). Invasion to cavernous sinus was found in 53 (36.8%) patients and was more frequent in group A (p<0.01). The mean number of surgery was higher in group A than group B (p< 0.05). The mean follow-up period was 46.6 ± 34 months. The postoperative MRIs and follow-up data for at least 24 months were available in 117 patients. Recurrence risk was higher in group A than group B (p = 0.03). Tumors with high Ki-67 proliferation index were grouped as 3-5, 6-10, 11-15, and > 15%. The risk of recurrence was not different between groups of high Ki-67 index. The optimal cutoff point of the Ki-67 proliferation index that predicted recurrence was 2.5% with 84.6% sensitivity and 47.4% specificity. The cavernous sinus invasion on MRI was associated with recurrence (p = 0.03). Tumor size and recurrence risk were not associated with P53 expression. High P53 expression was related with cavernous sinus invasion (p = 0.03). The mitotic index was not associated with recurrence risk and tumor invasion. Recurrence risk was higher in tumors with ≥ 2 histopathological atypia criteria (p = 0.01). High Ki-67 index with a 2.5% cutoff point and cavernous sinus invasion on MRI are reliable markers for predicting recurrence in PAs. Recurrence risk is also higher in tumors with two histopathological aggressiveness criteria. Strict follow-up and more aggressive treatment approaches may be necessary for invasive-proliferative PAs.

Lacrimal gland tumors in Turkey: types, frequency, and outcomes.

AIM: To evaluate the clinical, radiological, and treatment features of lacrimal gland tumors. METHODS: Retrospective review of 99 eyes of 92 patients with lacrimal gland tumors diagnosed and managed in a single institution between January 1999 and March 2017. Clinical and radiological features, histopathology, treatment methods, and prognosis were evaluated. RESULTS: The mean patient age was 40.3 (range: 7-80)y. The diagnosis was made histopathologically in 91 (91.9%) tumors and on a clinical and radiological basis in 8 (8.1%) tumors. Final diagnoses included idiopathic orbital inflammation (pseudotumor) in 46 (46.5%) lesions, pleomorphic adenoma in 14 (14.1%), adenoid cystic carcinoma in 12 (12.1%), granulomatous inflammation in 10 (10.1%), lymphoma in 5 (5.0%), benign reactive lymphoid hyperplasia in 3 (3.0%), dacryops in 3 (3.0%), carcinoma ex pleomorphic adenoma in 2 (2.0%), adenocarcinoma in 1 (1.0%), dermoid cyst in 1 (1.0%), cavernous hemangioma in 1 (1.0%), and leukemic infiltration in 1 (1.0%). Non-epithelial tumors comprised 64.6% (n=64) of all lacrimal gland tumors, epithelial tumors 32.3% (n=32), dermoid cyst 1% (n=1), cavernous hemangioma 1% (n=1), and leukemic infiltration 1% (n=1). There were in total 78 (78.8%) benign and 21 (21.2%) malignant tumors. CONCLUSION: Overall, 65% of lacrimal gland tumors were of non-epithelial origin and 32% of epithelial origin. By histopathology and clinical evaluation, 79% of lacrimal gland tumors were benign. The most common lacrimal gland tumors include idiopathic orbital inflammation (46.5%), epithelial (32.3%), and lymphoproliferative (8.1%) lesions.

Ectopic rhabdoid meningioma of the orbit in a child: case report and review of the literature.

In August 2016, an 11-year-old boy presented to the authors' institution with a right orbital tumor that was located superotemporally (superolaterally) and adherent to the sclera. The patient's past medical history revealed that he had undergone 2 previous craniotomies elsewhere in June 2008 and July 2010 for a superomedially located orbital lesion that had been histopathologically diagnosed as a neurothekeoma. After the second craniotomy, the patient underwent adjuvant intensity modulated radiotherapy (IMRT) to the right medial orbit. At the authors' institution, total excision of the orbital tumor was performed via an anterior conjunctival orbitotomy. Histopathological examination revealed a rhabdoid meningioma. Review of the histopathology obtained at the time of previous tumor excisions showed that the lesion was misdiagnosed as neurothekeoma and instead represented a meningioma from the beginning. The patient was started on a regimen of oral sunitinib and remained free of recurrence at 1.5 years of follow-up. Ectopic meningioma of the orbit is a rare entity. Rhabdoid meningioma is a rarely seen subtype of meningioma, accounting for 1%-3% of all intracranial meningiomas. To the best of the authors' knowledge, this is the first case of an ectopic orbital rhabdoid meningioma reported in the literature. They suspect that tumor seeding during the previous surgeries might have played a role in the occurrence of the tumor in an orbital location not targeted by IMRT.

Endoscopic removal of a suprasellar dermoid cyst in a pediatric patient: a case report and review of the literature.

INTRODUCTION: Dermoid cysts (DCs) are unusual benign congenital intracranial tumors that typicallyarise in the midline and form as a result of abnormal sequestration of ectodermal cells during neural tubeformation. In all age groups, endoscopic approaches are preferable for the removal of sellar lesions. A 6-year-old girl with recurrent meningitis underwent endoscopic endonasal surgery forsellar DC. CONCLUSION: To the best of our knowledge, we present the first case of a suprasellar DC in a pediatric patient that was removed endoscopically.

A Retrospective Evaluation of the Epithelial Changes/Lesions and Neoplasms of the Gallbladder in Turkey and a Review of the Existing Sampling Methods: A Multicentre Study.

OBJECTIVE: As there is continuing disagreement among the observers on the differential diagnosis between the epithelial changes/lesions and neoplasms of the gallbladder, this multicentre study was planned in order to assess the rate of the epithelial gallbladder lesions in Turkey and to propose microscopy and macroscopy protocols. MATERIAL AND METHOD: With the participation of 22 institutions around Turkey that were included in the Hepato-Pancreato-Biliary Study Group, 89,324 cholecystectomy specimens sampled from 2003 to 2016 were retrospectively evaluated. The numbers of adenocarcinomas, dysplasias, intracholecystic neoplasms/adenomas, intestinal metaplasias and reactive atypia were identified with the review of pathology reports and the regional and countrywide incidence rates were presented in percentages. RESULTS: Epithelial changes/lesions were reported in 6% of cholecystectomy materials. Of these epithelial lesions, 7% were reported as adenocarcinoma, 0.9% as high-grade dysplasia, 4% as low-grade dysplasia, 7.8% as reactive/regenerative atypia, 1.7% as neoplastic polyp, and 15.6% as intestinal metaplasia. The remaining lesions (63%) primarily included non-neoplastic polypoids/hyperplastic lesions and antral/pyloric metaplasia. There were also differences between pathology laboratories. CONCLUSION: The major causes of the difference in reporting these epithelial changes/lesions and neoplasms include the differences related to the institute's oncological surgery frequency, sampling protocols, geographical dissimilarities, and differences in the diagnoses/interpretations of the pathologists. It seems that the diagnosis may change if new sections are taken from the specimen when any epithelial abnormality is seen during microscopic examination of the cholecystectomy materials.

Do the Well Known Prognostic Parameters in Pancreatic Ductal Adenocarcinoma Really Reflect Survival?

OBJECTIVE: Pancreatic ductal adenocarcinoma is an aggressive tumor with short survival. In this study we aimed to investigate the effect of well-known prognostic parameters on survival in these tumors. MATERIAL AND METHOD: A total of 56 pancreatic ductal adenocarcinoma cases diagnosed between 2005 and 2014 were included in the study. Survival data were obtained and histopathological parameters were re-evaluated in each patient. RESULTS: Tumor size (p=0.029), mitotic count (p=0.030), lymph node metastasis (p=0.003), metastatic lymph node ratio (p < 0.001) and ampullary invasion (p=0.044) had a statistically significant relationship with survival. However, there was no relationship between survival and tumor grade, lymphovascular and perineural invasion, and peripancreatic soft tissue invasion. CONCLUSION: Our results showed that existent 2010 WHO pancreatic ductal adenocarcinoma grading parameters excluding mitotic count are subjective and not applicable. Considering that almost all of the tumors in our series were larger than 2 cm, we think that the 2 cm cut-off in tumor size is insufficient to make the tumor stage pT2. Peripancreatic soft tissue invasion, which is a common finding in pancreatic ductal adenocarcinoma, should also not be assessed like adjacent tissue invasion and make the tumor reach pT3 stage independent of tumor size. It is clear that the existent WHO tumor grading and pT staging parameters need to be revised and the mitotic count, which correlates with survival, should be presented in pathology reports.

Necrotic intraocular retinoblastoma associated with orbital cellulitis.

Orbital cellulitis associated with retinoblastoma is uncommon and is characterized by noninfectious inflammation of the periorbital structures. The underlying mechanism is thought to be necrosis of the intraocular tumor, leading to intraocular and periorbital inflammation. We report 2 retinoblastoma patients who presented with an orbital cellulitis-like picture and discuss clinical characteristics, histopathologic features, and treatment.

Coexistence of Mosaic Uniparental Isodisomy and a KCNJ11 Mutation Presenting as Diffuse Congenital Hyperinsulinism and Hemihypertrophy.

BACKGROUND: Isolated hyperinsulinaemic hypoglycaemia (HH) commonly results from recessively inherited mutations in the ABCC8 and KCNJ11 genes that are located on chromosome 11p15.1. More rarely, HH can feature in patients with Beckwith-Wiedemann syndrome (BWS), a congenital overgrowth disorder, resulting from defects at a differentially methylated region telomeric to the K-ATP channel genes at chromosome 11p15.5. SUBJECT: We undertook genetic testing in a patient with diazoxide-unresponsive HH diagnosed at birth. Physical examination later revealed hemihypertrophy of the right arm, a feature of BWS. RESULTS: We identified a novel mosaic, paternally-inherited KCNJ11 mutation(s) in the patient. Further analysis confirmed uniparental disomy (UPD) of chromosome 11, which extended across the KCNJ11 gene at 11p15.1 and the BWS locus at 11p15.5. CONCLUSION: These results highlight the importance of considering UPD as a mechanism of disease in patients with HH and a paternally inherited K-ATP channel mutation, especially when additional syndromic features are present.

Experimental Endoscopic Cordotomy in the Sheep Model.

AIM: Interventional pain therapies are usually based on destruction of the related pain-conducting pathways. Current procedures targeting pain have replaced conventional pain treatment modalities while being less invasive. In this study, we investigated the feasibility of the endoscopic percutaneous cordotomy process on the sheep cervical spinal cord. MATERIAL AND METHODS: Seven male sheep, Akkaraman® genus, were operated on in the study. The guide was introduced at C1 to C2 vertebrae. The interlaminar area was exposed by a dilator, the dura was identified, and then the working cannula was inserted into the subarachnoid space. The target point of cordotomy was defined by endoscopic visualization as the midpoint between the dentate ligament and ventral root entry zone. After determination of the target point, a carbon dioxide laser (CDL) probe was introduced through the cannula. Ablative lesioning was performed by CDL. Hindlimb withdrawal thresholds were measured using the "Sample Pain Scale". The lesion was demonstrated by magnetic resonance imaging and histopathological examination. RESULTS: Three sheep had ipsilateral hemiparesis and the response to firm pressure test was not performed on them. Among the remaining four sheep, the pain tolerance test showed that one sheep was at stage 0, two at stage 1, and the last one at stage 3. CONCLUSION: Cordotomy might be successfully performed with the endoscopic technique in the sheep model and this should encourage future studies regarding minimal invasive procedures for intractable pain.

Ectopic orbital meningioma: report of two cases and literature review.

Ectopic orbital meningioma is a rare tumor usually affecting the medial orbit. We present two cases that occurred in a 56-year-old woman and a 28-year-old man. The tumors in both patients were subtotally excised via orbitotomy surgery and were located in the superior quadrant in one of our patients and in the temporal quadrant in the other. Following histopathologic diagnosis, external beam radiotherapy (EBRT) was administered to one patient and intensity modulated radiotherapy to the other. We identified 12 other well-documented cases of ectopic orbital meningioma previously reported. Ectopic meningioma should be considered in the differential diagnosis of medial as well as lateral and superior orbital tumors. The tumor is usually well circumscribed but can be ill defined in imaging studies. There are intralesional calcifications and sclerosis of adjacent bone in some cases. Ectopic orbital meningioma can recur after incomplete excision. Based on the efficacy of EBRT in optic nerve sheath meningioma, we used this treatment to decrease the risk of recurrence in our two patients and found no tumor recurrence at follow-ups of 24 and 74 months, but one patient had severe vision loss from radiation retinopathy.

Breast hamartoma: a clinicopathologic analysis of 27 cases and a literature review.

OBJECTIVES: Breast hamartoma is an uncommon breast tumor that accounts for approximately 4.8% of all benign breast masses. The pathogenesis is still poorly understood and breast hamartoma is not a well-known disorder, so its diagnosis is underestimated by clinicians and pathologists. This study was designed to present our experience with breast hamartoma, along with a literature review. METHOD: We reviewed the demographic data, pathologic analyses and imaging and results of patients diagnosed with breast hamartoma between December 2003 and September 2013. RESULTS: In total, 27 cases of breast hamartoma operated in the Ankara University Medicine Faculty's Department of General Surgery were included in the study. All patients were female and the mean age was 41.8±10.8 years. The mean tumor size was 3.9±2.7 cm. Breast ultrasound was performed on all patients before surgery. The most common additional lesion was epithelial hyperplasia (22.2%). Furthermore, lobular carcinoma in situ was identified in one case and invasive ductal carcinoma was observed in another case. Immunohistochemical staining revealed myoid hamartoma in one case (3.7%). CONCLUSION: Breast hamartomas are rare benign lesions that may be underdiagnosed because of the categorization of hamartomas as fibroadenomas by pathologists. Pathologic examinations can show variability from one case to another. Thus, the true incidence may be higher than the literature indicates.

Breast hamartoma: a clinicopathologic analysis of 27 cases and a literature review

OBJECTIVES: Breast hamartoma is an uncommon breast tumor that accounts for approximately 4.8% of all benign breast masses. The pathogenesis is still poorly understood and breast hamartoma is not a well-known disorder, so its diagnosis is underestimated by clinicians and pathologists. This study was designed to present our experience with breast hamartoma, along with a literature review. METHOD: We reviewed the demographic data, pathologic analyses and imaging and results of patients diagnosed with breast hamartoma between December 2003 and September 2013. RESULTS: In total, 27 cases of breast hamartoma operated in the Ankara University Medicine Faculty's Department of General Surgery were included in the study. All patients were female and the mean age was 41.8±10.8 years. The mean tumor size was 3.9±2.7 cm. Breast ultrasound was performed on all patients before surgery. The most common additional lesion was epithelial hyperplasia (22.2%). Furthermore, lobular carcinoma in situ was identified in one case and invasive ductal carcinoma was observed in another case. Immunohistochemical staining revealed myoid hamartoma in one case (3.7%). CONCLUSION: Breast hamartomas are rare benign lesions that may be underdiagnosed because of the categorization of hamartomas as fibroadenomas by pathologists. Pathologic examinations can show variability from one case to another. Thus, the true incidence may be higher than the literature indicates. .

Vanishing bile duct syndrome after allogeneic bone marrow transplantation: is it the end of the road?

In this paper, we report the case of a 19-year-old male patient who presented with lymphoblastic phase of chronic myeloid leukemia and received an allogeneic bone marrow transplant from his cousin. The patient experienced severe, steroid-refractory acute graft versus-host disease of skin, gastrointestinal tract and liver that required further immunosuppression. However, hepatic graft-versus-host disease was complicated with vanishing bile duct syndrome, characterized by progressive destruction of small intrahepatic bile ducts, which was refractory to all available therapies and eventually led to end-stage liver disease. The pathogenesis and treatment of graft-versus-host disease after allogeneic hematopoietic cell transplantation is discussed with an emphasis on liver transplantation for intractable hepatic graft-versus-host disease.

Retinoblastoma in Turkey: results from a tertiary care center in Ankara.

PURPOSE: To evaluate the presentation patterns and results of management of retinoblastoma at a tertiary care center in Ankara, Turkey, with special emphasis on globe conservation rate in unilateral and bilateral intraocular retinoblastoma. METHODS: Patients were grouped according to the International Classification of Retinoblastoma. For intraocular retinoblastoma, group E and some group D eyes underwent primary enucleation. Secondary enucleation was performed after failure of chemoreduction, focal treatments, external beam radiotherapy (EBRT), and intra-arterial chemotherapy used in various combinations. For extraocular retinoblastoma cases, treatment consisted of enucleation/exenteration or orbital biopsy, high-dose chemotherapy, and EBRT to the orbit and metastatic sites. RESULTS: During the study period from October 1998 to May 2010, 165 of 192 (85.9%) patients had intraocular disease and 27 (14.1%) patients had extraocular disease. In total, primary or secondary enucleation was performed in 70 of 94 eyes with unilateral retinoblastoma (74.5%) and in 34 of 142 eyes with bilateral retinoblastoma (23.9%). The overall globe conservation rate was 69.6%. Only one patient in the intraocular retinoblastoma group died of metastatic retinoblastoma to the central nervous system. Twenty of 27 patients (74.1%) with extraocular retinoblastoma were found to have metastasis to the central nervous system, bone, bone marrow, and/or lymph nodes. At a mean follow-up of 28.0 months (median: 12 months; range: 1 to 120 months), survival was 33.3% despite intensive treatment. CONCLUSIONS: The overall risk of enucleation was 75% in eyes with unilateral retinoblastoma and 24% in eyes with bilateral retinoblastoma. Extraocular retinoblastoma carries a 75% risk of systemic metastasis and 67% risk of death.