Papillary Tumors of Pineal Region: A Single-Center Experience in Management of 11 Cases.

BACKGROUND: Papillary tumors of pineal region (PTPR) comprise a very rare subset of pineal region tumors that have been recently described. Literature on the management and outcome of PTPR is scarce owing to the rarity of these tumors. To address this lacuna, we analyzed our experience in management of PTPR. METHODS: We retrospectively analyzed the outcome of 11 patients with histopathologically proven PTPR who underwent surgical excision at our center. RESULTS: Mean patient age was 33.3 years (range, 12-45 years), and male-to-female ratio was 1.75:1. Headache was the most common presentation followed by visual disturbances, altered sensorium, Perinaud syndrome, and seizures. Cerebrospinal fluid diversion was required in 6 patients. Krause approach was the most common approach used for tumor excision (9/11 cases). There was no perioperative mortality. Two patients were lost to follow-up. In the remaining 9 patients, the average follow-up period was 45 months (range, 12-79 months). On first postoperative magnetic resonance imaging, 8 patients showed no evidence of residual tumor (gross total resection), while 1 patient had small residual tumor (near-total resection) that remained stable during follow-up. Four patients underwent adjuvant chemoradiotherapy. None of the patients developed recurrence during follow-up. CONCLUSIONS: PTPR are a rare subgroup of pineal region tumors with distinct cells of origin but presentation similar to other pineal region tumors. Surgical resection constitutes the mainstay of management, and the extent of resection appears to be the most important determinant of prognosis. The role of adjuvant therapy still needs to be determined.

Retroperitoneal and mediastinal follicular dendritic cell sarcoma: report of 3 cases with review of literature.

Follicular dendritic cell sarcoma (FDCS) is a rare malignant histiocytic proliferation of antigen presenting follicular dendritic cell. It is an uncommon primary malignancy first described by Monda et al. in 1986. Most commonly reported cases are lymph nodal. Occasional cases occur in extra nodal sites. Here, we describe the clinicopathological features, histomorphology and outcome of three patients with extranodal FDCS along with a concise review of literature on the topic. All three patients were adult females. Two patients were in third decade, and one had age of 50 years. Among the three cases, two cases are presented as retroperitoneal mass and one as mediastinal mass. CT scans revealed heterogeneously enhancing masses. All the cases showed ovoid to spindle neoplastic cells arranged predominantly in whorling, fascicular and storiform patterns with inflammatory infiltrate. Immunohistochemically, the tumor cells are positive for CD21, CD23, CD35 and Clustrin. In view of rarity and variable clinical presentation in FDCS, accurate diagnosis is necessary. Copyright © 2015 John Wiley & Sons, Ltd.

Nonrhabdomyosarcomatous abdominopelvic sarcomas: Analysis of prognostic factors.

BACKGROUND: Data concerning treatment outcome and prognostic factors in sarcomas of abdomen and pelvis are sparse in literature. METHODS AND RESULTS: Of 696 patients with nonrhabdomyosarcomatous soft tissue sarcoma registered at our center between June 2003 and December 2012, 112 (16%) patients of sarcomas arising from abdomen and pelvis were identified, of which 88 patients were analyzed for treatment outcome and prognostic factors. The median age was 40 years (range: 1-78 years) with a male: female ratio of 0.7:1. Twenty-one (24%) patients were metastatic at baseline. The most common tumor sites were retroperitoneum in 70% patients and abdominal wall in 18% patients. Leiomyosarcoma was the most common histological subtype in 36% patients followed by liposarcoma in 17% patients. Thirty-five (40%) patients had Grade III tumors. Forty-six (52%) patients underwent surgical resection. At a median follow-up of 43 months (range: 2-94 months), the 5-year event-free survival (EFS) and overall survival (OS) were 35% and 42%, with a median of 22 months and 43 months, respectively. Multivariate analysis identified male gender (P - 0.03, hazard ratio [HR] - 0.46, 95% confidence interval [CI] - 0.23-0.92), baseline metastatic disease (P - 0.01, HR - 2.98, 95% CI - 1.27-6.98) and Grade III tumors (P - 0.02, HR - 1.84, 95% CI - 1.08-3.13) as factors associated with poor EFS, whereas baseline metastatic disease (P < 0.001, HR - 5.45, 95% CI - 2.31-12.87) and unresectability (P - 0.01, HR - 2.72, 95% CI - 1.27-5.83) were associated with poor OS. CONCLUSION: This is a single-institutional study of patients with abdominopelvic sarcomas where gender was identified as a new factor affecting survival apart from baseline presentation, histologic grade, and surgical resection.

Multimodality management of a giant skull base hemangioendothelioma of the sphenopetroclival region.

A 20-year-old man presented with proptosis, nasal obstruction, vision loss and cavernous sinus syndrome, ongoing for 6 years. Imaging and biopsy confirmed a middle skull base epithelioid hemangioendothelioma arising from the left sphenopetroclival region with infratemporal fossa and intracranial-intradural extension into the left temporal lobe. Preoperative embolization of the left internal maxillary artery followed by a combined neurosurgical (front-temporal orbito-zygomatic craniotomy) and otorhinolaryngology (maxillary swing) approach was performed for tumor debulking. Postoperative radiotherapy and maintenance interferon chemotherapy was given to achieve a favorable outcome at 6 months follow-up. We describe the pertinent clinical, genetic, radiological and histopathological features, along with the available therapeutic modalities for a primary giant skull base hemangioendothelioma.

Role of bone marrow derived pluripotent stem cells in peripheral nerve repair in adult rats: A morphometric evaluation.

OBJECTIVES: Semi-quantitative and quantitative assessment of the effect of bone marrow-derived mononuclear cells (BM-MNC) on early and late phase of nerve regeneration in rat sciatic nerve model. MATERIALS AND METHODS: Sciatic nerve transection and repair was performed in 50 inbred female Wistar albino rats divided equally in two groups. In the test group the gap was filled with BM-MNCs obtained from the two male rats and fibrin sealant, while in the control group only fibrin sealant was used. Sciatic nerve was harvested at 15 days and at 60 days interval. Parameters of regeneration were assessed at anastomosis (G), intermediate distal (C), and distal site (A). Semi-quantitative (histopathological) and quantitative (morphometric) parameters were analyzed. RESULTS: At 15 days there was a statistically significant difference found in mean axon diameter, mean nerve thickness and myelin thickness at the repair site (P < 0.05). However, in the distal areas, the axons were sparse and myelin rings were very thin in both the groups. At 60 days, the difference in above-mentioned parameters was statistically significant at the distal most sites. FISH assay confirmed the presence of Y chromosome, confirming the presence of BM-MNCs from the male rats. CONCLUSIONS: Transplanting BM-MNCS at the site of peripheral nerve injury leads to significantly better recovery. These differences were evident at the repair site and at the intermediate distal site at 15 days and at the distal most sites at 60 days. With practically no ethical issue regarding their isolation and application, they can be easily used for clinical trials.

Posterior urethral valves: Persistent renin angiotensin system activation after valve ablation and role of pre-emptive therapy with angiotensin converting enzyme-inhibitors on renal recovery.

AIM: To study renin angiotensin system (RAS) activity after posterior urethral valve ablation and the role of early induction of angiotensin converting enzyme-inhibitors (ACE-I) on the outcome of renal function. MATERIALS AND METHODS: Thirty four children underwent valve ablation in which therapy with ACE-I was started 40.5 ± 4.1 (range 32-47 months) formed the study group. Post-ACE-I data were collected after mean duration of 18.2 ± 4.0 (12-28 months). Plasma renin activity (PRA), urinary micro albumin, glomerular filtration rate (GFR), and serum creatinine, before and after therapy were monitored. RESULTS: Therapy with ACE-I resulted in a fall in micro albuminuria by 45.7% and 42.0% in patients without and with vesico ureteral reflux, respectively, and improvement in split renal function by 6.6% and 5.9% GFR respectively. A similar response was noted in patients without and with renal scars. CONCLUSION: The decline in renal function after valve ablation is accompanied by activation of RAS reflected in a gradual rise in PRA. Therapy with ACE-I stabilizes and then improves renal function, thereby, retarding the pace of renal damage.

Clival giant cell tumor presenting with isolated trigeminal nerve involvement.

Giant cell tumour (GCT) constitutes about 5 % of all skeletal tumors. They rarely occur in the skull. When involved, they preferentially involve the sphenoid or temporal bones. Skull-base GCTs generally present with multiple cranial nerves involvement, most commonly sixth followed by the third cranial nerve. We describe a case of clival GCT presenting with an isolated trigeminal nerve involvement in a 19-year-old man which was managed by surgery and adjuvant radiation. At 18 months of follow-up, the patient is clinically asymptomatic. Clival GCT should also be considered in the differential diagnosis of any isolated trigeminal nerve palsy. Adjuvant radiation has an important role to play in managing this tumour.

Dose-dependent facilitation of peripheral nerve regeneration by bone marrow-derived mononuclear cells: a randomized controlled study: laboratory investigation.

OBJECT: Bone marrow-derived stem cells enhance the rate of regeneration of neuronal cells leading to clinical improvement in nerve injury, spinal cord injury, and brain infarction. Recent experiments in the local application of bone marrow-derived mononuclear cells (BM-MNCs) in models of sciatic nerve transection in rats have suggested their beneficial role in nerve regeneration, although the effects of variable doses of stem cells on peripheral nerve regeneration have never been specifically evaluated in the literature. In this paper, the authors evaluated the dose-dependent role of BM-MNCs in peripheral nerve regeneration in a model of sciatic nerve transection in rats. METHODS: The right sciatic nerve of 60 adult female Wistar rats (randomized into 2 test groups and 1 control group, 20 rats in each group) underwent transection under an operating microscope. The cut ends of the nerve were approximated using 2 epineural microsutures. The gap was filled with low-dose (5 million BM-MNCs/100 µl phosphate-buffered saline [PBS]) rat BM-MNCs in one group, high-dose (10 million BM-MNCs/100 µl PBS) rat BM-MNCs in another group, and only PBS in the control group, and the approximated nerve ends were sealed using fibrin glue. Histological assessment was performed after 30 days by using semiquantitative and morphometric analyses and was done to assess axonal regeneration, percentage of myelinated fibers, axonal diameter, fiber diameter, and myelin thickness at distal-most sites (10 mm from site of repair), intermediate distal sites (5 mm distal to the repair site), and site of repair. RESULTS: The recovery of nerve cell architecture after nerve anastomosis was far better in the high-dose BM-MNC group than in the low-dose BM-MNC and control groups, and it was most evident (p < 0.02 in the majority of the parameters [3 of 4]) at the distal-most site. Overall, the improvement in myelin thickness was most significant with incremental dosage of BM-MNCs, and was evident at the repair, intermediate distal, and distal-most sites (p = 0.001). CONCLUSIONS: This study emphasizes the role of BM-MNCs, which can be isolated easily from bone marrow aspirates, in peripheral nerve injury and highlights their dose-dependent facilitation of nerve regeneration.

Unilateral ureteric obstruction: Role of renin angiotensin system blockade on renal recovery: An experimental study.

AIMS: To study and compare the effects of angiotensin II antagonist (Losartan) and angiotensin converting enzyme (ACE) inhibitor (Enalapril) on renal recovery following reversal of iatrogenic unilateral upper ureteric obstruction. MATERIALS AND METHODS: Unilateral upper ureteric obstruction was created in 96 adult Wistar rats that were reversed after predetermined intervals. Losartan and Enalapril were given to different subgroups of rats following relief of obstruction. Rats were sacrificed and kidneys were subjected to planimetric and histopathological analysis. RESULTS: Dorsal lumbotomy approach provided a rapid and safe approach to kidneys in rats. The planimetric and histopathological changes were most severe in the group of rats in whom obstruction was not relieved before sacrifice. Addition of Enalapril and Losartan significantly hastened the reversal of renal changes following relief of obstruction as compared with the group in which no treatment was given following reversal of blockade. CONCLUSIONS: Renin angiotensin system (RAS) is the major pathway responsible for renal damage following outflow obstruction. However, this damage can be reversed with the use of drugs acting on the RAS.

Post operative pituitary apoplexy: preoperative considerations toward preventing nightmare.

INTRODUCTION: Post operative pituitary apoplexy after partial resection of a giant pituitary adenoma is mostly fatal, despite early and best management. Pathophysiology, clinical presentation and preoperative consideration toward prevention of apoplexy are discussed. MATERIAL AND METHODS: Patients with post operative pituitary apoplexy were critically reviewed for clinical presentation, endocrine status, preoperative imaging and post operative course with outcome. Operative findings and histopathology were correlated. RESULTS: Thirteen patients over 11 years with a mean age of 36 years were reviewed. All patients had giant pituitary adenomas. Four patients had functional adenomas. All patients were optimized for endocrine status before surgery. Twelve patients underwent transsphenoidal excision of the tumor. Only partial excision could be achieved in all cases. Deterioration of consciousness (9), visual deterioration (3), delayed reversal and excessive bleeding (1) were the primary indicators toward apoplexy. Ten patients were reexplored within 24 h of first surgery. All except one were explored transcranially a second time. Twelve patients died with variable post operative course. Hypothalamic dysfunction and dyselectolytemia (9) were leading causes of death, followed by meningitis and raised intracranial pressure. CONCLUSIONS: Post operative pituitary apoplexy is associated with high mortality, despite early and best management. Partial resection of the giant pituitary adenoma is directly responsible for post operative apoplexy. Maximum possible resection of the tumor by suitable exposure should be the optimal goal of surgery. Surgical exposure, either transcranial or transsphenoidal, should be dictated by tumor configuration on preoperative imaging. Endocrine status, histology of the tumor and clinical presentation do not appear to contribute to post operative pituitary apoplexy.

Her-2/neu, p-53, and their coexpression in osteosarcoma.

BACKGROUND: There is controversy regarding the patterns and prognostic significance of Her-2/neu and p-53 expression in osteosarcoma. Further, their combined expression has not been studied. PROCEDURE: Prechemotherapy biopsy specimens of 63 osteosarcoma patients from June 2004 to August 2006 were analyzed for Her-2/neu and p-53 using immunohistochemistry and compared with grade, stage, and morphologic subtype of tumor. RESULTS: There were 59 high-grade tumors; 32 of 63 had metastases. Histopathologic types included 36 of 63 osteoblastic and 18 of 63 chondroblastic subtypes. Male sex (P=0.045) and chondroblastic type (P=0.004) were associated with metastatic disease. Her-2/neu cytoplasmic staining was seen in 30 of 63 (47.1%) cases, 4 of 30 had additional membranous staining, 17 of 30 were metastatic, and 29 of 30 were high-grade tumors. Staining grade was 3+ in 10 of 30 samples whereas staining intensity 3+ was observed in 26 of 30 samples. Her-2/neu 3+ staining grade was significantly associated with chondroblastic subtype (6/18, P=0.026). P-53 staining was seen in 20 of 63 (31.74%) cases (all high grade), of which 11 were metastatic. Staining grades 3+ and 4+ were seen in 16 of 20 samples whereas staining intensity 3+ in 13 of 20 samples. P-53 expression was higher in chondroblastic (7/18, P value was not significant) and fibroblastic (3/3, P<0.05) subtypes. Coexpression of Her-2/neu and p-53 was seen in 10 cases (15.87%); 3 of 10 cases showed Her-2/neu membranous staining (P=0.01). CONCLUSIONS: Her-2/neu (47.1%), p-53 (31.74%), and their combined expression (15.87%) were not related to grade or stage of tumor. Chondroblastic subtype was associated with staining grade 3+ of Her-2/neu staining whereas coexpression of Her-2/neu and p-53 was significantly associated with membranous Her-2/neu staining.

Sacral myxopapillary ependymoma with extensive osteolysis.

Myxopapillary ependymoma rarely presents as a primary intra-sacral lesion and extensive bony destruction is unusual. Radiological features do not help in distinction from other commoner sacral tumors, like chordoma. Hence, histopathology serves as the best diagnostic modality for this differentiation. We report the case of a 42-year-old man with a primary intra-sacral myxopapillary ependymoma causing extensive osteolysis.

Oncocytic carcinoid tumor of the lung: a case report of diagnostic pitfall in filter membrane preparation of bronchial washings.

BACKGROUND: Oncocytic carcinoid tumor of the lung is a rare variant of pulmonary carcinoid. This report describes the morphologic appearance of this rare tumor on filter membrane preparation along with potential pitfalls. CASE: A 49-year-old woman presented with cough and expectoration. On chest radiograph a mass lesion was seen in the upper zone of the right lung. Bronchial washings were sent for evaluation. On filter membrane (Millipore) preparation of bronchial washing the possibility of a non-small cell carcinoma, possibly squamous, was suggested. Right upper lobectomy was subsequently performed and a histologic diagnosis of oncocytic carcinoid given. The cytomorphologic features of this tumor on the Millipore preparation were reviewed. CONCLUSION: Differential diagnosis of oncocytic carcinoid should be kept in mind while assessing cytologic material when tumor cells show abundant granular cytoplasm and prominent nucleoli. Oncocytic carcinoid also must be differentiated from oncocytoma and granular cell tumor. Immunocytochemistry and electron microscopy are useful in confirming the diagnosis.