A Rare Case Report of Invasive Ocular Surface Squamous Neoplasm of Conjunctiva

Ocular surface neoplasm(OSSN) represent a rare but broad spectrum of disease, including mild dysplasia on one end of the spectrum and invasive Squamous cell carcinoma(SCC) on the other. Thus, the diagnosis completely depends on histopathological examination of the lesion. The incidence of ocular surface neoplasia is strongly associated with factors like solar ultraviolet radiation, HIV and human papilloma virus(HPV) infections. Most lesions occur at the limbus within the interpalpebral fissure particularly the nasal side. Their importance lies in the fact that they mimic benign lesions like pterygium or even chronic conjunctivitis and thus can be misinterpreted and inadequately treated. Red eye and ocular irritation are the most common presenting symptoms. No tumor related deaths or metastasis are generally seen. Surgery with intra-operative control of surgical margins and adjunctive chemotherapy, immunotherapy and cryotherapy result in good tumor control rates, with promising results in aggressive, recurrent and large tumors.

Upfront Gamma Knife radiosurgery for Cushing’s disease and acromegaly: a multicenter, international study

OBJECTIVEGamma Knife radiosurgery (GKS) is typically used after failed resection in patients with Cushing’s disease (CD) and acromegaly. Little is known about the upfront role of GKS for patients with CD and acromegaly. In this study, the authors examine the outcome of upfront GKS for patients with these functioning adenomas.METHODSAn international group of 7 Gamma Knife centers sent pooled data from 46 patients (21 with CD and 25 with acromegaly) undergoing upfront GKS to the coordinating center of the study for analysis. Diagnosis was established on the basis of clinical, endocrine, and radiological studies. All patients were treated on a common radiosurgical platform and longitudinally followed for tumor control, endocrine remission, and hypopituitarism. Patients received a tumor median margin dose of 25 Gy (range 12–40.0 Gy) at a median isodose of 50%.RESULTSThe median endocrine follow-up was 69.5 months (range 9–246 months). Endocrine remission was achieved in 51% of the entire cohort, with 28% remission in acromegaly and 81% remission for those with CD at the 5-year interval. Patients with CD achieved remission earlier as compared to those with acromegaly (p = 0.0005). In patients post-GKS, the pituitary adenoma remained stable (39%) or reduced (61%) in size. Hypopituitarism occurred in 9 patients (19.6%), and 1 (2.2%) developed third cranial nerve (CN III) palsy. Eight patients needed further intervention, including repeat GKS in 6 and transsphenoidal surgery in 2.CONCLUSIONSUpfront GKS resulted in good tumor control as well as a low rate of adverse radiation effects in the whole group. Patients with CD achieved a faster and far better remission rate after upfront GKS in comparison to patients with acromegaly. GKS can be considered as an upfront treatment in carefully selected patients with CD who are unwilling or unable to undergo resection, but it has a more limited role in acromegaly.

Gamma Knife surgery for patients with jugular foramen schwannomas: a multiinstitutional retrospective study in Japan

OBJECTIVE This study aimed to explore the efficacy and safety of stereotactic radiosurgery in patients with jugular foramen schwannomas (JFSs). METHODS This study was a multiinstitutional retrospective analysis of 117 patients with JFSs who were treated with Gamma Knife surgery (GKS) at 18 medical centers of the Japan Leksell Gamma Knife Society. The median age of the patients was 53 years. Fifty-six patients underwent GKS as their initial treatment, while 61 patients had previously undergone resection. At the time of GKS, 46 patients (39%) had hoarseness, 45 (38%) had hearing disturbances, and 43 (36%) had swallowing disturbances. Eighty-five tumors (73%) were solid, and 32 (27%) had cystic components. The median tumor volume was 4.9 cm3, and the median prescription dose administered to the tumor margin was 12 Gy. Five patients were treated with fractionated GKS and maximum and marginal doses of 42 and 21 Gy, respectively, using a 3-fraction schedule. RESULTS The median follow-up period was 52 months. The last follow-up images showed partial remission in 62 patients (53%), stable tumors in 42 patients (36%), and tumor progression in 13 patients (11%). The actuarial 3- and 5-year progression-free survival (PFS) rates were 91% and 89%, respectively. The multivariate analysis showed that pre-GKS brainstem edema and dumbbell-shaped tumors significantly affected PFS. During the follow-up period, 20 patients (17%) developed some degree of symptomatic deterioration. This condition was transient in 12 (10%) of these patients and persistent in 8 patients (7%). The cause of the persistent deterioration was tumor progression in 4 patients (3%) and adverse radiation effects in 4 patients (3%), including 2 patients with hearing deterioration, 1 patient with swallowing disturbance, and 1 patient with hearing deterioration and hypoglossal nerve palsy. However, the preexisting hoarseness and swallowing disturbances improved in 66% and 63% of the patients, respectively. CONCLUSIONS GKS resulted in good tumor control in patients with either primary or residual JFSs. Although some patients experienced some degree of symptomatic deterioration after treatment, persistent adverse radiation effects were seen in only 3% of the entire series at the last follow-up. Lower cranial nerve deficits were extremely rare adverse radiation effects, and preexisting hoarseness and swallowing disturbances improved in two-thirds of patients. These results indicated that GKS was a safe and reasonable alternative to surgical resection in selected patients with JFSs.

Effectiveness of a 1-day aspiration plus Gamma Knife surgery procedure for metastatic brain tumor with a cystic component

Object Gamma Knife surgery (GKS) has gained increasing relevance in the treatment of metastatic brain tumors, but many metastatic tumors contain a large cystic component and often exceed the size limit for GKS. For such lesions, the authors adopted a procedure in which stereotactic aspiration is first performed and followed immediately by GKS on the same day. In this paper, the authors describe this 1-day combined procedure and evaluate its efficacy. Methods Between 2005 and 2010, 25 cystic metastases in 25 patients were treated at Dokkyo Medical University. The patients first underwent MRI and stereotactic aspiration of the cyst while stationary in a Leksell stereotactic frame; immediately afterward, the patients underwent a second MR imaging session and Gamma Knife treatment. Tumor volume reduction, tumor control rate, and overall survival were examined. Results Tumor volume, including the cystic component, decreased from 8.0–64.2 cm3 (mean 20.3 cm3) to 3.0–36.2 cm3 (mean 10.3 cm3) following aspiration, and the volume of 24 of 25 lesions decreased to less than 16.6 cm3, which is equivalent to the volume of a 3.16-cm sphere. At least 20 Gy was delivered to the entire lesion in 24 of 25 cases. Good tumor control was obtained in 16 of 21 cases that could be evaluated during a median follow-up period of 11 months (range 1–27 months); however, reaccumulation of cyst contents was observed in 2 patients who required Ommaya reservoir placement. Conclusions The 1-day aspiration plus GKS procedure is an effective and time-efficient treatment for large cystic brain metastases.

Long-Term Outcome of AIDS-Related Lymphoma Treated with Abbreviated Cycles of EPOCH-RR: A Prospective Study of 40 Patients

We hypothesized that the addition of rituximab to EPOCH chemotherapy could improve tumor kill, allowing delivery of fewer cycles of treatment and therefore reducing toxicity. Patients received EPOCH-RR (in mg/m2/d – etoposide 50, vincristine 0.4 and doxorubicin 10 all CIV d 1–5; cyclophosphamide 750 mg IV d 5; prednisone 60 po days 1–5 and rituximab 375 IV d 1,5 and G-CSF sc d 6–15) every 21 days. Prophylactic IT methotrexate was administered and HAART was suspended during therapy. Cyclophosphamide was adjusted based on absolute neutrophil count (ANC) nadir. Response was assessed by CT and FDG-PET scan and patients received 1 cycle beyond CR for a minimum of 3 cycles. Characteristics of 40 enrolled patients are: median (range) age 42 (9–60) years; IPI 3 (0–4); ECOG PS 1 (1–4), CD4 count 222 (0–835) cells/mm3; HIV viral load 34,766 (0–6,080000) RNA copies/mL; male sex 35 (88%); LDH > N 27 (68%); stage IV 27 (68%) and histology diffuse large B-cell lymphoma (DLBCL) 32 (80%) and Burkitt lymphoma (BL) 8 (20%). Of 38 evaluable patients (2NE), median (range) number of cycles given is 3 (3–5) with CR/CRu in 35 (92%) and PR in 1 (3%) patients. At 4 years median follow-up, PFS and OS are 86% and 70%, respectively. Eight patients with BL are in continuous CR. For patients with CD4 > and < 100 cells/mm3, PFS is 96% and 69%, respectively. IPI did not impact OS and PFS. Early PET scanning (after cycle 2) had a high negative predictive value (100%) but low positive predictive value (20%). Fever/neutropenia occurred on 30%, ANC < 500/mm3 on 40%, and platelets < 50,000/mm3 on 23% of cycles. EPOCH-RR was associated with less CD4 loss - median 128 cells/mm3 (range +154 to −639) compared to historical data with EPOCH alone (median 189 cells/mm3 (range +19 to −973). Patients with CD4 < 100/mm3 had good tumor control, but OS was only 31% due to late deaths from advanced AIDS. Patients with CD4 counts > 100/mm3 had an extremely good PFS and OS. The addition of rituximab did not appear to cause serious infection related complications or deaths. However, one treatment-related death occurred from complications of mycobacterium avium intercellulare. Abbreviated EPOCH-RR is highly effective and tolerable in ARL and enables the administration of fewer treatment cycles (median 3 versus 6). PET scanning has a very high negative but low positive predictive value for subsequent relapse, possibly due to HIV associated PET changes. Accrual continues.

Trigeminal schwannomas: results of Gamma Knife surgery in 37 cases

Object Information on outcomes of Gamma Knife surgery (GKS) for patients harboring trigeminal schwannomas is limited because these tumors are rare. The authors evaluated tumor control and functional outcomes in patients who underwent GKS for trigeminal schwannomas to clarify the efficacy of this treatment. Methods Forty-two patients with trigeminal schwannomas but no evidence of neurofibromatosis Type 2 were treated with GKS at Komaki City Hospital between November 1991 and December 2003. Of these, 37 patients were assessed. The mean tumor volume in these patients was 10 cm3. The mean maximum radiation dose directed to the tumor was 27.9 Gy and the mean dose directed to the tumor margin was 14.2 Gy. The mean follow-up period was 54 months. In four patients (11%) there was complete tumor remission; in 20 (54%) there was partial tumor remission; in eight (22%) the disease remained stable; and in five (14%) the tumor enlarged or uncontrollable facial pain developed with radiation-induced edema requiring resection. The actuarial 5- and 10-year tumor control rates were both 84%. With respect to functional outcomes, 40% of patients noted an improvement in their symptoms, and one patient experienced new symptoms despite good tumor control. Conclusions Gamma Knife surgery was a safe and effective treatment for a select group of patients harboring trigeminal schwannomas. Large tumors that compress the brainstem and cause deviation of the fourth ventricle should first be removed surgically and any remnant should be treated by GKS.