Long-course temozolomide in aggressive pituitary adenoma: real-life experience in two tertiary care centers and review of the literature

Pituitary - Aggressive pituitary adenomas (APAs) and pituitary carcinomas (PCs) are challenging for their invasive nature, resistance to treatment and recurrences. Temozolomide (TMZ) is...     

Temozolomide in patients with glioblastoma at second relapse after first line nitrosourea-procarbazine failure: a phase II study

OBJECTIVES: To investigate the efficacy of temozolomide (TMZ) in relationship to progression free survival at 6 months (PFS-6), median time to progression (TTP), response rate and toxicity, a phase II study was conducted in patients with recurrent glioblastoma multiforme (GBM) following surgery plus radiotherapy and a first-line regimen based on nitrosourea, procarbazine and vincristine. METHODS: Forty-two patients with GBM were administered TMZ at the dose of 150 mg/m(2)/daily for 5 days every 4 weeks. RESULTS: The PFS-6 and at 12 months (PFS-12) was 24% (95% Confidence Interval [CI] = 14-42%) and 8% (CI = 2-27%), respectively, with a median TTP of 11.7 weeks (CI = 9-22 weeks). The response was assessed in all 42 patients; we observed 2 complete responses (CR) (4.7%), 6 partial responses (PR) (14.3%), and 9 stable disease (SD) (21.4%), with CR+PR = 19% (CI = 7-31%). CONCLUSION: TMZ as a second line regimen is a valid option in patients with heavily pretreated GBM.     

Collecting duct carcinoma of the kidney: an immunohistochemical study of 11 cases

Background  

Collecting duct carcinoma (CDC) is a rare but very aggressive variant of kidney carcinoma that arises from the epithelium of Bellini's ducts, in the distal portion of the nephron. In order to gain an insight into the biology of this tumor we evaluated the expression of five genes involved in the development of renal cancer (FEZ1/LZTS1, FHIT, TP53, P27 ^kip1, and BCL2).     

Efficacy and feasibility of standard procarbazine, lomustine, and vincristine chemotherapy in anaplastic oligodendroglioma and oligoastrocytoma recurrent after radiotherapy. A Phase II study

BACKGROUND: To the authors' knowledge, there is a scarcity of accurate data regarding the feasibility of standard chemotherapy with procarbazine, lomustine, and vincristine (PCV) in a homogeneous series of patients with primary anaplastic oligodendroglioma (AO) and oligoastrocytoma (AOA) that was recurrent after surgery and standard radiotherapy. The aim of the current study was to evaluate the overall response rate, toxicity, and time to progression (TTP) with the use of standard PCV in this setting. METHODS: Between November 1994 and September 2000, 37 patients were enrolled in the current study. Of these, 23 had AO (62%) and 14 had AOA (38%). All patients received standard PCV comprised of lomustine (110 mg/m2) on Day 1, procarbazine (60 mg/m2) on Days 8-21, and vincristine (1.4 mg/m2, maximum total 2 mg) on Days 8 and 29. Cycles were repeated every 6 weeks. RESULTS: There were 11 complete responses (CR; 29.7%) and 11 partial responses (PR; 29.7%) reported and 8 patients had stable disease (SD; 21.6%). The response rate was higher in patients with AO compared with patients with AOA (77.2% vs. 22.7%; P = 0. 02). The median TTP, which was 12.3 months overall, was 30.3 months in patients who achieved a CR, 19.1 months in patients who achieved a PR, and 6.1 months in patients with SD. The median TTP was 18.6 months in AO patients and 6.14 in AOA patients. There were no cases of severe toxicity reported although in 16 patients (43%) who were free of disease progression, PCV was discontinued because of toxicity or inadequate recovery after 2 weeks of delay. CONCLUSIONS: PCV chemotherapy was reported to achieved a high response rate and TTP but incurred a high risk of persistent toxicity.     

Recurrent nephrogenic adenoma in bladder diverticulum: case report and literature review

Nephrogenic adenoma is an uncommon benign lesion of the urinary tract induced by chronic irritation of the vesical mucosa, due to infection, trauma, surgery, calculi, foreign bodies and chemical agents. A 68-year-old male was admitted to our linic for a periodical cystoscopic evaluation as part of a follow-up initiated due to a past transitional cell carcinoma. The scheduled cystoscopy revealed, within a bladder diverticulum, an unexpected and completely asymptomatic nephrogenic adenoma that we removed by transurethral resection. We followed up the patient at 24 months, then later we made the diagnosis of nephrogenic adenoma. During this time, the patient experienced three relapses within the same diverticulum, always involving a nephrogenic adenoma we persistently treated by transurethral resections. As the nephrogenic adenoma is considered a benign lesion without any direct evidence of a possible evolution to an overt cancer, we successful attempted a half-yearly cystoscopic follow-up to control the growth of a highly recurrent benign entity, interposing between controls a periodical imaging. This paper represents the second report of a nephrogenic adenoma within a bladder diverticulum, but the first case of a nephrogenic adenoma highly recurrent within the same diverticulum and managed conservatively by regular transurethral resection scheduled over the time.     

Postpartum maternal death associated with undiagnosed Hodgkin's lymphoma

The authors describe the case of a 28-year-old patient who died from an extensive intracerebral haemorrhage due to disseminated intravascular coagulation (DIC) which occurred a few days after delivery. Access to an emergency department of gynaecology on three separate occasions in the three days preceding hospitalization made judicial evaluation of the case necessary. The case was studied with a methodological approach based on the following steps: (1) examination of clinical records; (2) autopsy; (3) study of the placenta; (4) anatomo-histopathological observations concerning particular organs and structures potentially involved in postpartum maternal death; and (5) evaluation of physicians' behaviour, in the light of necroscopic findings and a review of the literature. The causes of death most frequently described in the postpartum period were excluded; a mediastinal nodular sclerosing Hodgkin's lymphoma with transdiaphragmatic diffusion, not diagnosed in life, was demonstrated. The cause of death was identified as intracerebral haemorrhage following DIC, Hodgkin's disease and recent delivery by caesarean section. Analysis of the physicians' conduct, together with a review of the literature, revealed a medical error. However, no causal relationship between the error and the death of the patient was considered to exist. The interest of the case lies in the unusual cause of DIC discussed in relation to a hypothesis of obstetric-gynaecological liability.     

Clinical and pathological characteristics of patients presenting with biochemical progression after radical retropubic prostatectomy for pathologically organ-confined prostate cancer

INTRODUCTION: To identify risk factors for biochemical failure after radical prostatectomy (RP) in men with pathologically organ-confined (OC) prostate cancer (PCa). MATERIALS AND METHODS: Clinical and pathological features of 350 consecutive patients with pathologically OC PCa treated only with RP and bilateral pelvic lymphadenectomy were analyzed, retrospectively, to identify predictor parameters of prostate-specific antigen (PSA) failure (PSA>or=0.4 ng/ml). The median follow-up was 58.6 months (range: 3.9-183 months). All pathological specimens were step sectioned at 4-mm intervals. Kaplan-Meier progression-free survival rates and chi2 test were adopted for statistical analyses. Multivariate Cox proportional hazard regression models were used to test the association between pathological Gleason score and surgical margin status. RESULTS: 67 patients (19.1%) failed at a median follow-up of 40.2 months (range 1.9-123.3). Age and preoperative PSA failed to reveal significance also in patients with serum PSA>or=20 ng/ml (p=0.46). Patients with T3 clinical stage had a higher progression rate compared to T1C and T2 (43.5 vs. 27.8 and 17.3%, respectively) even if no high statistical significance was pointed out. Presence of perineural infiltration (p=0.04) and prostatic apex infiltration (p=0.74) in the prostatectomy specimens failed to reveal significance. A high pathological Gleason score (>or=7; p=0.0003) and surgical margin status (p<0.0001) were shown to be the most powerful predictive parameters of biochemical progression. CONCLUSIONS: In patients with pathologically OC PCa the presence of a high pathological Gleason score and positive surgical margins appear to represent the most important factors for prediction of outcome following RP.     

Diffuse Leptomeningeal Glioneuronal Tumors: A New Entity?

The peculiar radiological and pathological findings of four pediatric cases admitted to the University Hospital of Padua between 1990 and 2007 are described. In all cases, the contrast‐enhanced head and spine magnetic resonance images revealed thickened and abnormally enhancing subarachnoid spaces particularly at the level of basal cisterns and interhemispheric fissure. Furthermore, small cystic lesions scattered throughout the brain and mainly in the cerebellum were also visible. All patients were missing a well‐defined intraparenchymal mass, although during the follow‐up a small intramedullary lesion appeared within the cervical spine in two and subsequently in the frontal horn of the left lateral ventricle in one of those. All patients presented an indolent long‐term follow‐up. Histologically, the tumors were composed by a monotonous population of cells arranged in straight lines or in small lobules. The cells were characterized by round to oval nuclei with finely granular dispersed chromatin, inconspicuous nucleoli with oligodendrocyte‐like features. The morphological and immunohistochemical findings suggested in all cases a “glioneuronal commitment” of the tumors. Because of the unique similar clinical and neuroradiological characteristics, we propose this small series of tumors as a new possible distinct pathological and clinical entity.     

A 14‐YEAR‐OLD GIRL WITH MULTIPLE TUMORS

Pleomorphic xanthoastrocytoma (PXA) is a rare astrocytic tumor that usually occurs in the superficial cerebral hemispheres of children and young adult and has a favorable prognosis. We report a case of a 14‐year‐old girl with a recent history of sciatica and ataxic gait. Pre‐ and post‐contrast brain and spinal MRI revealed the presence of multiple solid lesions with a cystic component in the cerebellum and the spinal cord with a concomitant massive leptomeningeal involvement Histological and immunohistochemical findings were concordant with a final diagnosis of WHO grade II PXA. Even the biological indolent PXAs'' behavior, this is the third report in the literature of such an unusual multicentric PXA with leptomeningeal dissemination.