Detecting a rare composite small bowel lymphoma by Magnetic Resonance Imaging coincidentally: A case report with radiological, surgical and histopathological features.

INTRODUCTION: Diagnosing lymphoma continues to prove challenging in the clinical practice. Composite lymphoma (CL) is defined by the coexistence of different lymphoma subtypes in the same anatomical location. This condition has seldom been witnessed in the gastrointestinal (GI) tract. We weren't able to find previous cases in the literature about small bowel CL with follicular lymphoma (FL) and classical Hodgkin lymphoma (CHL). Surgery is the treatment of choice to obtain accurate histology, to manage and prevent acute complications. We state that this work has been reported in line with the SCARE criteria. CASE PRESENTATION: We describe an extremely rare case of small bowel CL, presenting as an intestinal bulky mass with circumferential infiltration of bowel loops. The small bowel tumor was incidentally detected by abdominal Magnetic Resonance Imaging (MRI) in a 64-year-old man who suffered from rectal discomfort and non-specific clinical symptoms. After this radiological finding, the patient underwent multiphase contrast computed tomography (MDCT) for initial staging and to study vascular involvement. Surgery was recommended to obtain an accurate diagnosis both due to initial symptoms of the intestinal obstruction and to avoid small bowel complications. The histopathological examination revealed a small bowel CL composed mainly of B cells FL with also CHL components. CONCLUSION: It is important to note that involvement of the proximal ileal loops is very rare in small bowel lymphoma. MRI represents a precious diagnostic tool to evaluate the intra and extramural extent of the tumor.

Determinants of percent mammographic density in women with premature ovarian insufficiency.

OBJECTIVE: To evaluate the determinants of breast density in women with premature ovarian insufficiency (POI). METHODS: In a cross-sectional study of 163 women with POI undergoing mammography, percent mammographic density (PMD) was evaluated by digitizing the image. PMD was correlated with age, age at menarche, age at POI, time since POI, body mass index (BMI), gestational history and hormone therapy (HT) use (duration, dose, regimen). RESULTS: POI was diagnosed at a mean age of 32.3 ± 5.9 years. The mean age of the women at mammography was 41.3 ± 5.4 years; mean BMI was 27.4 ± 5.4 kg/m2 and mean PMD was 24.3 ± 18.5. Mean PMD did not differ between the different age groups evaluated (29-39, 40-49 and 50-55 years) or between users and non-users of HT. Mean duration of HT use was 5.6 ± 4.7 years. PMD was higher in nulligravidas compared to women who had been pregnant (p = 0.0016); however, POI occurred earlier in nulligravidas (p < 0.0001). PMD correlated negatively with BMI (r = -0.27; p = 0.0005). CONCLUSION: In women with POI, HT use had no effect on PMD, irrespective of the duration of use, dose or regimen. Pregnancy and BMI were consistently associated with PMD, with density being greater in nulligravidas and in women with lower BMI.

Allogeneic cellular gene therapy in hemoglobinopathies--evaluation of hematopoietic SCT in sickle cell anemia.

Many patients with thalassemia have been cured with BMT since the first successful transplant in 1981. Allogeneic stem cell gene therapy is the only treatment option for patients with sickle cell anemia (SCA). A total of 11 patients with a median age of 12 years (range, 2-16), affected by SCA, received hematopoietic SCT from HLA-identical, related donors following a myeloablative-conditioning regimen. Indications for transplantation were vaso-occlusive crisis, acute chest syndrome, avascular bone necrosis, chronic RBC transfusions, or hemorrhagic stroke. All patients had sustained engraftment. One patient became a stable mixed chimera with 25% of donor cells at 4 years after transplantation. One patient died at 1 year after transplantation. The probability of survival, SCA-free survival and TRM at 5 years after transplant were 90, 90 and 10%, respectively. All 10 surviving patients remained free of any SCA-related events after transplantation. In conclusion, these data confirm SCT from a suitable HLA-matched, related donor should become the primary option for curing children with SCA. There is an excellent survival rate and a return to normal life, free of SCA-related events.

A phase II trial of short course fludarabine, mitoxantrone, rituximab followed by 9°Y-ibritumomab tiuxetan in untreated intermediate/high-risk follicular lymphoma.

BACKGROUND: A prospective, single-arm, open-label, multicenter, nonrandomised phase II trial to evaluate efficacy and safety of short fludarabine, mitoxantrone, and rituximab (FMR) induction followed by radioimmunotherapy, in untreated, intermediate/high-risk follicular non-Hodgkin's lymphoma (NHL) patients. PATIENTS AND METHODS: Fifty-five patients were treated using a sequential treatment schedule of four induction cycles of FMR chemoimmunotherapy, and a subsequent consolidating single administration of (90)Y-ibritumomab tiuxetan ((90)Y-IT), 8-14 weeks later. Patients were eligible for radioimmunotherapy if at least in partial response (PR) after induction, with normal platelet and granulocyte counts and a bone marrow infiltration ≤ 25%. Primary study end points were response rate and hematologic toxic effects; secondary end points were overall survival (OS) and progression-free survival (PFS). RESULTS: All the 55 patients received four induction cycles with an overall response rate of 96% (38 complete responses [CR] and 15 PR). Fifty-one patients (38 in CR and 13 in PR) received (90)Y-IT. By the end of the treatment, 49/55 patients achieved a CR. With a median follow-up of 21 months, the estimated 3-year PFS was 81% and the 3-year OS 100%. CONCLUSIONS: This study has established feasibility, tolerability, and efficacy of a regimen composed by short FMR induction with (90)Y-IT consolidation in untreated intermediate/high-risk follicular NHL patients.

Sleep disorders in adult-onset myotonic dystrophy type 1: a controlled polysomnographic study.

BACKGROUND: Sleep disturbances and excessive daytime somnolence are common and disabling features in adult-onset myotonic dystrophy type 1 (DM1). METHODS: Our study used questionnaires, ambulatory polysomnography and the multiple sleep latency test to evaluate sleep-wake cycle and daytime sleepiness in unselected adult-onset DM1 patients. We recruited 18 patients affected by adult-onset DM1 and 18 matched controls. RESULTS: Sleep efficiency was <90% in 16/18 patients, and it was significantly reduced when compared with controls. Reduced sleep efficiency was associated with abnormal respiratory events (5/18 patients) and/or periodic limb movements (11/18 patients). The Periodic Limb Movement Index was significantly increased in DM1 versus controls. A significantly lower mean MSLT sleep latency was detected in DM1 versus controls, but it did not reach pathological levels. CONCLUSIONS: Our controlled study demonstrated sleep alterations in unselected consecutive DM1 patients. Periodic limb movements in sleep are commonly associated with sleep disturbance in adult-onset DM1, and it may represent a marker of CNS neurodegenerative processes in DM1.

ICTAL aphasia as manifestation of partial status epilepticus in a long-lasting misdiagnosed symptomatic epilepsy: an emblematic case.

Magnetic Resonance Imaging (MRI) represents the procedure of choice for detection of anatomical lesions in epilepsy. Vascular malformations in central nervous system, such as cavernoma, can cause symptomatic epilepsy. We describe a case of ictal aphasia as manifestation of a partial status epilepticus probably due to a mutual interaction between a recent bleeding cavernoma and a concomitant reduction of antiepileptic treatment in a long-lasting misdiagnosed symptomatic epilepsy. We conclude that MRI is a mandatory diagnostic method to identify structural abnormalities underlying epilepsy in all patients affected by recurrent focal seizures independent of the duration of epilepsy.

Pregabalin as add-on therapy induces REM sleep enhancement in partial epilepsy: a polysomnographic study.

BACKGROUND AND PURPOSE: To evaluate the effects of pregabalin (PGB) adjunctive therapy on sleepwake cycle and daytime somnolence in adult patients affected by partial epilepsy. METHODS: Twelve patients affected by partial epilepsy underwent a 24-h ambulatory polysomnography and a subjective evaluation of daytime somnolence by means of the Epworth Sleepiness Scale (ESS), before and after 3 months treatment with PGB. RESULTS: Pregabalin therapy reduced seizures by >50% in 8 out of 12 patients. It induced a significant increase of REM sleep and a decrease of stage 2 NREM sleep (S2). A significant increase of the ESS score was observed without reaching the pathological cut-off value (mean ESS score <10). No statistical correlation between REM sleep and seizure frequency was observed. DISCUSSION: Pregabalin seems to be effective and safe in partial epilepsy. The increase of REM sleep may be indicative of an improvement of nocturnal sleep quality considering the involvement of REM sleep in learning and memory processes. REM sleep enhancement may be the result of both a direct effect of PGB on sleep generators and an indirect effect due to its clinical efficacy. The increase of ESS score within normal range suggests that daytime somnolence is a minor adverse effect of PGB.

Cognitive and psychiatric effects of topiramate monotherapy in migraine treatment: an open study.

Few data are available on cognitive and psychiatric effects of topiramate (TPM) monotherapy in migraine. Twenty patients affected by migraine were treated with TPM monotherapy. At the same time, twenty control subjects were selected. A comprehensive neuropsychological and behavioural battery of tests were performed at baseline (T0), at titration (T1) and in maintenance period (T2). Topiramate serum levels were also investigated at T1 and T2. On comparison with the control group, no cognitive and psychiatric differences were detected at baseline. A significant reduction of word fluency score (P < 0.05) was evident after TPM treatment, both at T1 and T2. No patient developed psychiatric adverse events. TPM induced an impairment of verbal fluency and no psychiatric adverse events, demonstrating selective negative cognitive profile in migraine therapy. Slow titration, low doses, lack of previous psychiatric disorders and/or familial history may explain our data.

Effects of motor cortex rTMS on lower urinary tract dysfunction in multiple sclerosis.

We tested the effects of 5-Hz rTMS over the motor cortex in multiple sclerosis (MS) subjects complaining of lower urinary tract symptoms either in the filling or voiding phase. Our data show that motor cortex stimulation for five consecutive days over two weeks ameliorates the voiding phase of the micturition cycle, suggesting that enhancing corticospinal tract excitability might be useful to ameliorate detrusor contraction and/or urethral sphincter relaxation in MS patients with bladder dysfunction.

Repetitive transcranial magnetic stimulation of the motor cortex ameliorates spasticity in multiple sclerosis.

OBJECTIVE: To investigate whether repetitive transcranial magnetic stimulation (rTMS) can modify spasticity. METHODS: We used high-frequency (5 Hz) and low-frequency (1 Hz) rTMS protocols in 19 remitting patients with relapsing-remitting multiple sclerosis and lower limb spasticity. RESULTS: A single session of 1 Hz rTMS over the leg primary motor cortex increased H/M amplitude ratio of the soleus H reflex, a reliable neurophysiologic measure of stretch reflex. Five hertz rTMS decreased H/M amplitude ratio of the soleus H reflex and increased corticospinal excitability. Single sessions did not induce any effect on spasticity. A significant improvement of lower limb spasticity was observed when rTMS applications were repeated during a 2-week period. Clinical improvement was long-lasting (at least 7 days after the end of treatment) when the patients underwent 5 Hz rTMS treatment during a 2-week protocol. No effect was obtained after a 2-week sham stimulation. CONCLUSIONS: Repetitive transcranial magnetic stimulation may improve spasticity in multiple sclerosis.

The effect of esophageal myotomy and myectomy on the lower esophageal sphincter pressure profile: intraoperative computerized manometry study.

SUMMARY: The surgical treatment of achalasia, based on Heller's myotomy is the procedure of choice to reduce the sphincterial high pressure zone, either by laparotomy or, most recently, by laparoscopy. What is the right length of the myotomy? Many authors have reported 10-15% postoperative residual dysphagia, due to the incomplete gastric myotomy and not to esophageal pouring. The aim of this study is to experimentally determine the modifications induced by Heller's myotomy and myectomy of the esophago-gastric junction on lower esophageal sphincter (LES) pressure profile, using a computerized manometric system. Myotomy of the esophageal portion of the LES (i.e. without dissection of the gastric fibers) has not modified the parameters considered, while the dissection of gastric fibers for at least 2-3 cm on the anterior gastric wall has created a significant modification of the LES pressure profile. Our observations seem to confirm and more clearly demonstrate the important role played by gastric fibers in sustaining the sphincteric HPZ. Moreover, analysis of our data, showed the need to always perform a complete myotomy. This was objectively shown during the intervention by means of intraoperative manometry, in order to significantly reduce the possibility of a dysphagic relapse, caused by inadequate treatment.

[Total gastrectomy for gastric cancer: can the type of lymphadenectomy condition the long-term results?]. / Gastrectomia totale per cancro gastrico: può il tipo di linfadenectomia condizionare i risultati a distanza?

INTRODUCTION: Gastric cancer is the second tumor for frequency in the world. Surgery is still the only curative treatment. Good results in terms of long distance survival, postoperative morbidity and mortality have been achieved in the last years. The extension of lymphadenectomy is an important and discussed matter and it is not clear if lymphadenectomy may contribute to improve the surgical results. The Japanese surgeons were the first ones, in the 60's, to introduce a D2-D3 extended lymphadenectomy, but the real benefits of this technique are still being discussed. Indeed lymphonodal metastasis seem to be one of the most important prognostic factors in the gastric cancer and the level and the number of metastatic nodes are useful to predict the patients' survival. The aim of this study is to value the D2 lymphadenectomy in the patients who were treated with total gastrectomy for gastric adenocarcinoma, comparing the results both with the D1 lymphadenectomy and the D3-D4, paying attention to the survival rates related with the lymphonodal dissection. PATIENTS AND METHOD: From 1998 to 2004, we studied 87 patients with gastric cancer. Out of 78 patients treated surgically, 9 were judged unresectable. Out of 69 patients treated surgically, one died before surgery and so he was put away by this study. All the patients were treated with total gastrectomy and a GI tract reconstruction by Roux's Y termino-lateral esophageal-jejunal anastomosis. In 20 patients we also made a splenectomy. We followed the Japanese Research Society for Gastric Cancer guidelines, according to which nodes are gathered into 16 levels and divided in 4 groups (N1-N4) depending on the cancer localization. The extension of the lymphadenectomy has been classified according to the level of the removed nods. The patients were divided into 3 groups. First group: patients undergone a total gastrectomy with D1 lymphadenectomy. Second group: patients undergone D2 lymphadenectomy. Third group: patients undergone D3 and D4 lymphadenectomy. The data we obtained let us value the survival rate. RESULTS: Out of the 78 patients treated, 69 were resected with a 88.5% resection rate (69/78). Perioperatory mortality was 1.4% (1/69). Global survival was 53.8% (44/68). The 5 years survival for the Ia stage was 82.6%, 89.3% for the Ib stage, 67.8% for the II stage, 56.6% for the IIIa, 16.8% for the IIIb and 0% for the IV stage. In addition the 5 years survival in the patients without lymphnodal metastasis was 79%, much higher than the 30.6% obtained in the patients with lymphonodal metastasis (p <0.0001). In the patients who underwent D1 lymphadenectomy, survival was 73.4%, while we obtained a result of 70.4% and 13.8% respectively in the D2 and D3-D4 (p <0.05). In the advanced stages (IIIa, IIIb, IV), the survival rate in the patients with lymphadenectomy D2 vs D1 vs D3-D4 was 38.9% vs 0% vs 36.3% (p <0.0001). The survival rate based on the extension of the lymphadenectomy in the patients with lymphonodal metastasis resulted to be much higher in those patients who underwent a D2 lymphadenectomy (43.5% in 5 years) compared both to D1 (0% in 6 months) and (29.5% in 5 years) D3-D4 lymphadenectomy. CONCLUSIONS: The relation between long distance survival and extension of the lymphadenectomy in the patients with gastric adenocarcinoma is still being discussed. Different studies show the importance of a careful lymphadenectomy as the main mean for a better long distance survival in the patients with gastric cancer. Other studies showed a link between gastric cancer prognosis and number of positive nodes. If more than 7-8 nodes are affected with metastasis, prognosis is usually poor. Therefore the presence of nod metastasis has a negative influence in the prognosis of this tumor; for this reason D2 lymphadenectomy is the technique to be used for the treatment of the gastric adenocarcinoma, both for a good staging and a better long distance survival, but particularly in the advanced cancers which are, at the moment, the highest number of cases that reach the surgeon's attention.

Gastric cancer in the elderly patient.

The incidence of gastric cancer is increasing in elderly patients, unlike what it's being observed in the younger patients. The aim of this study is to evaluate the impact of the age on the resecability, resection type (R0-R1-2), morbidity, mortality and survival rate. The higher mortality observed in the elderly patients, the discussed role of an extended lymphadenectomy and the poor survival rate, would lead to a surgical approach which mostly tends to the palliation of the symptoms than the obtainment of a curative resection. But, on the other hand, the impossibility of making chemotherapy in most of the over 75 year old patients, lead us to a R0 surgery treatment.

[Antithrombotic prophylaxis in day surgery: proposal of a clinical protocol]. / La profilassi antitrombotica in day surgery. Proposta di un protocollo clinico.

The considerable diffusion of the day surgery has new problems such as the use of antithrombotic prophylaxis, now carried out systematically in major surgery. An the basis of the poor data present in the literature, a protocol of study is proposed to evaluate the real effectiveness of this type of prophylaxis in DS, dividing the patients into 3 groups, according to the patient's risk factors to the intervention and to the disease.

[Surgical treatment of esophageal achalasia by Heller+Nissen laparoscopic procedure. A 24-hour ambulatory esophageal manometry study]. / Il trattamento chirurgico dell'acalasia esofagea mediante Heller + Nissen laparoscopica. Studio mediante manometria ambulatoriale di 24 ore.

BACKGROUND: The aim of this work was to evaluate, by means of a 24-h esophageal manometry, the motor activity of esophageal body in achalasic patients before and after Heller's myotomy and Nissen's fundoplication. METHODS: Twenty-five achalasic patients underwent a 24-h esophageal manometry. After surgical treatment they had a further 24-h esophageal manometry during follow-up. RESULTS: A statistically significant increase of amplitude of contraction and an increase, but not statistically significant, of frequency and duration of contractions were observed. The study of peristaltic activity showed an increase of peristaltic activity in total and complete sequences and decrease of dropped and interrupted peristaltic sequences. CONCLUSIONS: These data surprisingly showed the presence of a peristaltic activity (31.2%), in achalasic esophageal body, and complete sequences in 20%. The improvement of peristaltic activity observed after surgical abolition of the functional sphincteral rub, proposes again the question about the fall of the peristaltic activity of the esophageal body, which could be due to the hard transit through the LES. This preliminary data seem to confirm, in qualitative and quantitative manner, the positive effect of Heller's myotomy and the null effect of Nissen's fundoplication on the esophageal transit.

Neoadiuvant treatment with intravesical interleukin-2 for recurrent superficial transitional bladder carcinoma Ta-T1/G1-2.

The aim of this study was to evaluate the direct action of IL-2 on recurrent superficial transitional bladder carcinoma and the effect on recurrence rate. 27 patients were submitted to neoadjuvant treatment by intra-vesical instillation of recombinant IL-2 and to transurethral resection. We did not observe any effect on neoplasms but the recurrence rate was less than the expected one. It is possible that treatment of bladder carcinoma with intra-vesical instillation of IL-2 may promote immuno-prophilaxis.

High-dose epirubicin in the prophylactic treatment of T1G2 superficial bladder tumors.

OBJECTIVES: To evaluate the activity and safety of intravesical instillations of 80 mg epirubicin in a selected patient population with T1G2 primary and multiple superficial bladder tumors. To assess the completeness of the transurethral resection (TUR) at 4 weeks (second look) and to compare the histology of local and review pathology. METHODS: One hundred and sixty-nine patients have been histologically assessed both locally and extramurally for T1G2 superficial bladder tumors. Epirubicin (80 mg/instillation) started within 20 days after TUR was administered weekly during the first month and then monthly for another 11 months. Assessments for relapse were carried out according to the standard methods. RESULTS: Histological consistency for T1G2 between local and extramural assessments was found in 85.2% of cases. At the median follow-up time of 38 months, the overall relapse rate was 43.3%. Treatment was very well tolerated: no systemic adverse events were reported and local adverse events were confined to chemical cystitis which in 3% required treatment discontinuation. CONCLUSIONS: Epirubicin (80 mg/instillation) appeared effective in the prophylaxis of relapse in primary and multiple T1G2 superficial bladder tumors. A second TUR at 3-4 weeks is necessary in T1 tumors. Excellent concordance between local and review pathology was found.