The accuracy of preoperative imaging in measuring the length of the ileocolic segment affected by Crohn's disease: a prospective cohort study.

AIM: The study aimed to evaluate the accuracy of imaging for measurement of the length of the ileocolic segment affected by Crohn's disease. METHOD: Fifty-four consecutive patients who underwent resection between 2011 and 2014 for ileocolic Crohn's disease were prospectively studied. All had preoperative MR or CT enterography. Two independent radiologists measured the length of the diseased intestinal segment. The measurements were compared with the length of disease assessed on pathology of the non-fixed surgical specimen. RESULTS: The median preoperative length of the Crohn's disease segment on imaging was 20.5 (2-73) cm and 20 (3-90) cm, as measured by the two radiologists. Interobserver agreement was substantial (κ = 0.69) with a correlation coefficient (r) of 0.82 (P < 0.001). The median length of the Crohn's disease segment on pathological examination was 16.5 (2-75) cm and was closely correlated with the radiological measurement (r = 0.76, P < 0.001). The length of the Crohn's disease segment on imaging was correct to within 5 cm of the value on pathology. It was correct in 30 (55%) patients and was underestimated and overestimated in 6 (11.1%) and 18 (33.3%). A length of disease of less than 20 cm found on imaging in 26 patients was confirmed in 25 (96%) on pathology, whereas a length of more than 20 cm found on imaging in 28 patients was confirmed in 18 (64%) on pathology. The sensitivity, specificity, positive predictive value, negative predictive value and overall accuracy of imaging for predicting a length of less than 20 cm were 71%, 95%, 96%, 64% and 79%. CONCLUSION: Imaging accurately identifies the length of the ileocolic segment of Crohn's disease when it is 20 cm or less on pathological examination. In patients with more extensive disease, imaging tends to overestimate the length and should be interpreted with caution.

Outcome of emergency surgery for severe neuroleptic-induced colitis: results of a prospective cohort.

AIM: The study evaluated the outcome of severe acute antipsychotic (neuroleptic) drug related colitis requiring emergency surgery. METHOD: From 2009 to 2014, 20 patients underwent emergency surgery for acute and severe neuroleptic-related ischaemic colitis. Neuroleptic-induced colitis was defined as another cause besides inflammatory, infectious or ischaemic colitis with a relationship to treatment by antipsychotic drugs. RESULTS: The main drugs involved were cyamemazine (n = 9, 45%), loxapine (n = 5, 25%), haloperidol (n = 4, 20%) and alimemazine (n = 4, 20%). Most (n = 14, 70%) patients presented with haemodynamic instability requiring massive resuscitation and vasopressive drugs. CT signs of digestive impairment were found in 13 (65%) patients having emergency surgery. The lesions were pancolonic in 40%; transparietal necrosis was found in 45% and 15% had colonic perforation. Twelve (60%) patients had total or subtotal colectomy and eight (40%) a segmental colectomy with colostomy or ileostomy in all cases. The postoperative mortality was 15% and morbidity was 70%, necessitating surgical reintervention in two (10%) patients. Of the 17 surviving patients, 11 (64.7%) had restoration of intestinal continuity after a median delay of 103 days, with a postoperative morbidity rate of 36.3%. In the intent-to-treat population, the permanent stoma rate was 30%. CONCLUSION: The morbidity and mortality of surgery for neuroleptic-drug-induced colitis is higher than for colitis due to other causes. A better knowledge of this condition should lead to early diagnosis.

Pathological response of locally advanced rectal cancer to preoperative chemotherapy without pelvic irradiation.

AIM: Pathological response to chemotherapy without pelvic irradiation is not well defined in rectal cancer. This study aimed to evaluate the objective pathological response to preoperative chemotherapy without pelvic irradiation in middle or low locally advanced rectal cancer (LARC). METHODS: Between 2008 and 2013, 22 patients with middle or low LARC (T3/4 and/or N+ and circumferential resection margin < 2 mm) and synchronous metastatic disease or a contraindication to pelvic irradiation underwent rectal resection after preoperative chemotherapy. The pathological response of rectal tumour was analysed according to the Rödel tumour regression grading (TRG) system. Predictive factors of objective pathological response (TRG 2-4) were analysed. RESULTS: All patients underwent rectal surgery after a median of six cycles of preoperative chemotherapy. Of these, 20 (91%) had sphincter saving surgery and an R0 resection. Twelve (55%) patients had an objective pathological response (TRG 2-4), including one complete response. Poor response (TRG 0-1) to chemotherapy was noted in 10 (45%) patients. In univariate analyses, none of the factors examined was found to be predictive of an objective pathological response to chemotherapy. At a median follow-up of 37.2 months, none of the 22 patients experienced local recurrence. Of the 19 patients with Stage IV rectal cancer, 15 (79%) had liver surgery with curative intent. CONCLUSION: Preoperative chemotherapy without pelvic irradiation is associated with objective pathological response and adequate local control in selected patients with LARC. Further prospective controlled studies will address the question of whether it can be used as a valuable alternative to radiochemotherapy in LARC.

Clinical and molecular characterization of diffuse large B-cell lymphomas with 13q14.3 deletion.

BACKGROUND: Deletions at 13q14.3 are common in chronic lymphocytic leukemia and are also present in diffuse large B-cell lymphomas (DLBCL) but never in immunodeficiency-related DLBCL. To characterize DLBCL with 13q14.3 deletions, we combined genome-wide DNA profiling, gene expression and clinical data in a large DLBCL series treated with rituximab, cyclophosphamide, doxorubicine, vincristine and prednisone repeated every 21 days (R-CHOP21). PATIENTS AND METHODS: Affymetrix GeneChip Human Mapping 250K NspI and U133 plus 2.0 gene were used. MicroRNA (miRNA) expression was studied were by real-time PCR. Median follow-up of patients was 4.9 years. RESULTS: Deletions at 13q14.3, comprising DLEU2/MIR15A/MIR16, occurred in 22/166 (13%) cases. The deletion was wider, including also RB1, in 19/22 cases. Samples with del(13q14.3) had concomitant specific aberrations. No reduced MIR15A/MIR16 expression was observed, but 172 transcripts were significantly differential expressed. Among the deregulated genes, there were RB1 and FAS, both commonly deleted at genomic level. No differences in outcome were observed in patients treated with R-CHOP21. CONCLUSIONS: Cases with 13q14.3 deletions appear as group of DLBCL characterized by common genetic and biologic features. Deletions at 13q14.3 might contribute to DLBCL pathogenesis by two mechanisms: deregulating the cell cycle control mainly due RB1 loss and contributing to immune escape, due to FAS down-regulation.

Multifocal deficits due to leukemic meningoradiculitis in chronic lymphocytic leukemia.

Symptomatic nervous system leukemic infiltration is rarely observed in CLL. Various clinical manifestations including headache, confusion, cranial nerve palsies, focal central deficits and peripheral neuropathies have been seldom reported, occurring in less than 1% of patients. We report herein 2 CLL patients with unusual clinical presentations of nervous system invasion. They presented multiple progressive peripheral deficits due to meningoradiculitis. In both, CSF immunophenotyping analysis identified a majority of T cells (>90%), and less than 10% of B-CLL cells expressing CD5, CD19 and CD20. Our analyses revealed the transformation of CLL into an aggressive B-cell lymphoma in one case (Richter's syndrome). A post mortem study showed massive infiltration of cranial nerves and spinal roots by large B lymphomatous cells. In the other case, CNS oriented chemotherapy led to remission and total neurological recovery. In practice, the etiological diagnosis of neurological deficits in CLL patients is difficult. CSF analysis may be useful, requiring viral PCR, repeated cytological studies and immunophenotyping analysis. Although rare, leptomeningeal leukemic localization has to be discussed, even in the absence of overt Richter syndrome, and may require an early therapeutic test.

[Poorly differentiated large cell endocrine carcinoma of the extrahepatic bile ducts]. / Carcinome endocrine peu différencié à grandes cellules des voies biliaires extrahépatiques.

We report a case of a poorly differentiated endocrine large cell carcinoma of the extrahepatic bile ducts in a 73-year-old man, revealed by abdominal pain, jaundice and weight loss. Computed tomography and endoscopic retrograde cholangiography found tumoral stenosis of the main bile duct. Brush cytology detected tumor cells. Pathological examination of the resected bile duct disclosed a high-grade large cell carcinoma with morphological endocrine features and positivity for chromogranin A. This tumor was associated with a minor component of adenocarcinomatous cells. Despite polychemotherapy, the patient had widely metastatic disease a few months later. We discuss here the histogenesis of this tumor as well as its nosological position among the endocrine and mixed tumors of bile ducts.

[Autoimmune myelofibrosis with dermatomyositis]. / Myélofibrose auto-immune secondaire à une dermatomyosite.

We report a second observation of autoimmune myelofibrosis associated with an inflammatory myositis in a 30-year-old female. The links between myelofibrosis and autoimmunity are discussed.

National multicentric evaluation of quality of pathology reports for rectal cancer in France in 2016.

The quality of pathologic assessment of rectal cancer specimens is crucial for treatment efficiency and survival. The Royal College of Pathologists (RCP) recommends evaluating the quality of the pathology report in routine practice using three quality indicators (QIs): the number of lymph nodes (LNs) analyzed (≥ 12), the rate of venous invasion (VI ≥ 30%), and peritoneal involvement (pT4a ≥ 10%). In this study, we evaluated the three QIs of the French national pathology reports and compared them with British guidelines and assessed the influence of neoadjuvant radiochemotherapy on QIs. From January 1 to December 31, 2016, all pathology reports for rectal adenocarcinoma were collected from French departments. Neoadjuvant radiochemotherapy included long-course radiotherapy with concomitant 5-FU-based chemotherapy. A total of 983 rectal cancer pathology reports were evaluated. A median of 15 LNs were analyzed and 81% of centers had ≥ 12 LNs. The rate of VI was 30% and 41% of centers had ≥ 30% VI. The rate of pT4a was 4% and 18% of centers reported ≥ 10% pT4a. None of the centers reached the threshold for the three QIs. All three QIs were lower after radiochemotherapy compared to surgery alone. In conclusion, in French routine practice, the values of two of the three QIs (LNs analyzed and VI) were globally in line with RCP guidelines. However, the rate of pT4a was very low, particularly after radiochemotherapy, suggesting its low value in rectal cancer.

[Risk factors for incident cervical intraepithelial neoplasia (CIN) among HIV-infected women: a prospective study]. / Facteurs de risque d'acquisition de lésions du col utérin dans une population de femmes infectées par le VIH: étude prospective.

OBJECTIVES: To study risk factors for incident cervical intraepithelial neoplasia (CIN) among HIV-infected women. PATIENTS AND METHODS: Prospective study of a population of 97 HIV-infected women with normal Pap smear at inclusion. RESULTS: Fourteen CIN (diagnosed by colposcopy and confirmed with biopsy) were observed within a median follow-up of 38 months (13 CIN 1, one CIN 2). The incidence of cervical lesions was estimated to be 2%, 7% and 10% respectively at one year, two and three years after inclusion, The time to occurrence was very variable (ranging from 7 months to 6 years) among our patients. No known risk factors, in particular neither the CD4 cell count nor antiretroviral treatment, were identified to be associated with occurrence of CIN in our study population. CONCLUSION: Regardless of their immune status and HIV treatments, extensive and prolonged gynaecological follow up of HIV-infected women remains necessary.

[Primary uterine and vulvar Ewing's sarcoma/peripheral neuroectodermal tumors in children: two unusual locations]. / Sarcomes d'Ewing/tumeurs neuroectodermiques primitives périphériques de l'utérus et de la vulve chez l'enfant: deux localisations exceptionnelles.

Two genital cases of Ewing's sarcoma/peripheral neuroectodermal tumor in 14 and 15 year old teenagers are reported. They arose in uteri and vulva, as small round cell tumors with some rosette-like formations and intense membranous immunoreactivity for CD99. The EWS/FLI-1 transcript was present in the vulvar tumor. Under chemotherapy, uterine tumor outcome was fatal within 9 months. The vulvar lesion was treated by surgery and chemotherapy, without relapse after 7 months. Likewise to these 2 cases, the literature seems to indicate different prognosis for uterine and vulvar tumors.

[Stomatococcus mucilaginosus infection. A rare cause of cutaneous histiocytic infiltrate]. / Infection à Stomatococcus mucilaginosus. Une cause rare d'infiltrat histiocytaire cutané.

We report the case of a systemic infection due to Stomatococcus mucilaginosus with cutaneous localisations occurring in a neutropenic child. The histological features of this cutaneous involvement are very unusual and the diagnosis can be suggested before bacteriological results.

[Gastric epithelioid angiosarcoma, a biopsy diagnostic pitfall]. / Angiosarcome épithélioïde gastrique, un piège diagnostique sur biopsie.

We report the case of a 70-year-old man who presented a bulky gastric mass. The diagnosis of poorly differenciated adenocarcinoma was made on the biopsy. On surgical specimen, the final diagnosis of gastric angiosarcoma with secondary intestinal involvement was established. The patient did not respond to chemotherapy and died 8 months later. The diagnosis was difficult because of epithelioid areas which expressed both cytokeratin and vascular antigens. Human herpesvirus 8 previously detected in some soft tissue angiosarcoma was not found by polymerase chain reaction.