Synchronous mucinous adenocarcinoma of the rectosigmoid seeding onto a pre-existing anal fistula

Carcinoma within a long-standing fistula-in-ano is rare and may be defined by specific neoplastic involvement of the fistulous track in the absence of rectal mucosal carcinoma. The presence of a carcinoma of mucinous histology occurring synchronously in the perianal region and the colon is exceptionally rare. We present a case with a review of the literature concerning its aetiopathogenesis and treatment. A 72-year-old man with a 2 months history of dark red rectal bleeding and mucus per rectum with alternating constipation and diarrhoea, was observed. Clinical examination and a barium enema showed a perianal fistula and an annular stenosing lesion of the rectosigmoid. Preoperative CT scan confirmed the colonic lesion. Colonic resection and wide fistula excision were performed. Histology showed an adenocarcinoma with a clear resection margins. The fistula also showed a similar histology. Chemoradiation (5-Fluorouracil (425 mg/m(2)) and Leucovorin (20 mg/m(2)) with 4500 cGy external beam radiotherapy was utilized. Subsequent clinical follow-up and CT examination of the patient has not revealed recurrent disease at 14 months

Extended abdominoperineal resection in women: the barbadian experience

BACKGROUND AND OBJECTIVES: We report our results of a selective approach to primary direct appositional vaginal repair versus transverse rectus abdominis flap repair (TRAM) in patients with extensive rectal/anal cancer or in cases with primary cancer of cervix, vagina or vulva involving the anal canal and anal sphincters. METHODS: Eighteen female patients (mean age: 62.9 years; range: 44–81 years) with a median follow-up of 14 months (range: 2–36 months) undergoing extended abdominoperineal reconstruction with total mesorectal excision between May 2002 and September 2005, were studied. RESULTS: Twelve patients underwent an extended abdominoperineal resection with hysterectomy and vaginectomy, with 6 patients undergoing primary TRAM flap reconstruction following pelvic exenteration. Exenterative procedures were performed in 2 cases of primary vaginal cancer, following Wertheim hysterectomy for carcinoma of the cervix with recurrence after radiation and in 2 further cases of anal cancer with extensive pelvic recurrence after primary chemoradiation. Fifteen cases are alive on follow-up with no evidence of disease; 2 patients who had recurrent carcinoma of the cervix and who underwent TRAM flap reconstruction, have recurrent disease after 5 and 6 months of follow-up, respectively. DISCUSSION: Our experience shows that careful primary closure of an extended abdominoperineal resection wound is effective and safe. Our one case of wound breakdown after primary repair underwent external beam and intracavitary irradiation primarily with wound breakdown of a primary repair followed by a delayed pedicled graciloplasty. TRAM flap reconstruction has been reserved in our unit for patients undergoing total pelvic extenteration. In general, we would recommend the use of TRAM flap reconstruction in younger sexually active patients where there has been external irradiation combined with brachytherapy

Prognostic factors affecting survival after surgical resection of gastrointestinal stromal tumours: a two-unit experience over 10 years

BACKGROUND: Gastrointestinal stromal tumours (GISTs) are the most common mesenchymal neoplasm of the gastrointestinal (GI) tract which has only been recently described based on their specific immunohistochemistry and the presence of particular KIT-related mutations which potentially make them targets for tyrosine kinase inhibition. METHODS: Sixty-one patients (29 M; 32 F, median age 60 years; range: 23–86 years) between June 1994 and March 2005, were analyzed from two allied institutions. Patient, tumour, and treatment variables were analyzed to identify factors affecting survival. RESULTS: Of the 61 patients, 55 (90%) underwent complete surgical resection of macroscopic disease. The 5-year overall survival (OS) rate in the 61 patients was 88% and the 5-year disease-free survival (DFS) in the 55 cases completely resected was 75%. Univariate analysis revealed that R0 resection was strongly associated with a better OSrate (p < 0.0001). Likewise, univariate analysis also showed high mitotic count of > 10 mitoses/per 50 HPF was a significant variable in worse prognosis for OS (≤ 10 mitoses/per 50 HPF 95% 5-year OS vs. > 10 mitoses/per 50 HPF 74% 5-year OS, respectively; p = 0.013). On subsequent multivariate analysis, only high mitotic count remained as a significant negative prognostic variable for OS (p = 0.029). Among patients resected for cure, there were 8 recurrences during follow-up. The mean time to recurrence was 21 ± 10 months (range: 4–36 months). Univariate analysis revealed that mitotic count of > 10 mitoses per 50 high power fields, intratumoural necrosis, and pathological tumour size (> 10 cm in maximal diameter) significantly correlated with DFS (p = 0.006, 0.002 and 0.02, respectively), with tumour necrosis and high mitotic count remaining as independent predictive variables affecting prognosis on subsequent multivariate analysis. CONCLUSION: Most GISTs are resectable with survival principally dependent upon mitotic count and completeness of resection. Future metabolic and genetic analyses will define the role of and resistance to induction or postoperative adjuvant targeted kinase inhibition therapy

Development of synchronous VHL syndrome tumors reveals contingencies and constraints to tumor evolution

This is an Open Access article distributed under the terms of the Creative Commons Attribution License.-- et al.[Background]: Genomic analysis of multi-focal renal cell carcinomas from an individual with a germline VHL mutation offers a unique opportunity to study tumor evolution. [Results]: We perform whole exome sequencing on four clear cell renal cell carcinomas removed from both kidneys of a patient with a germline VHL mutation. We report that tumors arising in this context are clonally independent and harbour distinct secondary events exemplified by loss of chromosome 3p, despite an identical genetic background and tissue microenvironment. We propose that divergent mutational and copy number anomalies are contingent upon the nature of 3p loss of heterozygosity occurring early in tumorigenesis. However, despite distinct 3p events, genomic, proteomic and immunohistochemical analyses reveal evidence for convergence upon the PI3K-AKT-mTOR signaling pathway. Four germline tumors in this young patient, and in a second, older patient with VHL syndrome demonstrate minimal intra-tumor heterogeneity and mutational burden, and evaluable tumors appear to follow a linear evolutionary route, compared to tumors from patients with sporadic clear cell renal cell carcinoma. [Conclusions]: In tumors developing from a germline VHL mutation, the evolutionary principles of contingency and convergence in tumor development are complementary. In this small set of patients with early stage VHL-associated tumors, there is reduced mutation burden and limited evidence of intra-tumor heterogeneity.RF and JL received funding from EU FP7 (PREDICT project), EB is a Rosetrees Trust fellow, NM received funding from the Rosetrees Trust, MG is funded by the UK Medical Research Council, IV is funded by Spanish Ministerio de Economía y Competitividad subprograma Ramón y Cajal, and CS is a senior Cancer Research UK clinical research fellow and is funded by Cancer Research UK, the Rosetrees Trust, EU FP7 (projects PREDICT and RESPONSIFY, ID:259303), the Prostate Cancer Foundation, and the Breast Cancer Research Foundation. This study was supported by researchers at the National Institute for Health Research Biomedical Research Centres at University College London Hospitals and at the Royal Marsden Hospital.Peer Reviewe

The role of impedance planimetry in anorectal assessment (letter)

The advantage of the impedance planimetric technique is to more readily define reproducible values for dynamic rectal compliance and elasticity, where conventional proctometrographic (pressure/volume) curves have proven less accurate. The real-time ultrasonographic measurement of rectal cross-sectional areas (CSA) during distension (and deflation) is less reliant on the intrinsic compliance characteristics of the balloon assembly or on inherent rectal geometry. In this sense, impedance planimetry provides a better reflection of rectal wall stiffness and altered viscoelastic properties during inflation/deflation cycles

Reinterventions after complicated or failed STARR procedure

Background/aims: The stapled transanal rectal resection (STARR) procedure has been suggested as a simple surgical option for patients presenting with evacuatory difficulty in the clinical presence of a rectocele. Most of these patients have a multiplicity of pelvic floor pathology unaddressed by the performance of one procedure. The aim of the study was to assess an unselected group of patients referred to a tertiary coloproctological unit following performance of the STARR procedure for obstructed defecation (OD) where the procedure was complicated or had failed. Materials and methods: Anorectal, urogynecological, and psychological examination with objective constipation/incontinence scoring, anal–vaginal-perineal ultrasound, manometry, and defecography were selectively performed utilizing the Iceberg Diagram to detect occult pelvic floor pathology. Results: Twenty patients were referred with 13 cases (female, 10; median age, 65 years; range, 40–72) operated upon. Post-STARR surgery was performed for three complications and ten failures including recurrent OD, severe proctalgia, and fecal incontinence. Overall, 11 patients underwent biofeedback therapy and psychotherapy. Of the operated group, 11 patients had a median of four associated disorders. Seven patients had a significant psychological overlay with severe depression or anxiety and four heterogeneous anal sphincter defects. Operative procedures were tailored to the clinical findings using enterocele repair, staple removal, fistulectomy, rectosigmoid resection, and levatorplasty where appropriate. Twelve patients were evaluated after a median follow-up of 18 months. Of these, six (all with psychoneurosis remained unchanged. Three patients with no psychological overlay were asymptomatic with a further two improved. Conclusion: The STARR procedure, when complicated or failed, has a poor outcome following surgical reintervention. It requires careful patient selection to determine the associated pelvic floor pathology and pre-existent psychopathology

Magnetic resonance imaging of anatmoic defects in faecal incontinence

Endoanal probe technology provides high-resolution soft-tissue imaging for periluminal anal and rectal disease, most notably in complex perirectal sepsis and in patients presenting with fecal incontinence and sphincter damage. The trend is a movement away from direct sphincter repair toward sacral neuromodulation in external anal sphincter (EAS) injury. However, endoanal magnetic resonance imaging (EAMRI) has shown clear accuracy in the delineation of both EAS defects suitable for surgical treatment and in the definition of internal anal sphincter (IAS) damage potentially suitable for bioimplant deployment. Moreover, endoanal MR images have shown a correlation with histopathologically defined sphincter atrophy, which in turn has been predictive of relatively poor postsphincteroplasty outcomes. The role of EAMRI in the hierarchy of imaging modalities for use in an incontinence algorithm is somewhat unclear in the absence of comparative randomized clinical trials. However, it has a definitive place in defining sphincter atrophy in equivocal cases that are perhaps best treated nonoperatively or initially by temporary neuromodulatory stimulation

Subphrenic abscess secondary to Actinomycosis meyeri and Klebsiella ozaenae following laparoscopic cholecystectomy

A case is reported of a subphrenic abscess 12 months post-aparoscopic cholecystectomy in a 72-year-old male with identification of Actinomyces meyeri and the oropharyngeal commensal Klebsiella ozaenae. The first organism is exceptionally rare following laparoscopic cholecystectomy and is presumed to be a result of inadvertent gallstone spillage. The second organism has not previously been reported in a subphrenic abscess. The etiopathogenesis and management of this condition are presented