Rare "Inverted" Verrucous Carcinoma (Carcinoma Cuniculatum) of the Sacrogluteal Region: Case Report and Literature Review.

A 49-year-old woman with a past history of several infundibular cysts in the head and neck region presented with a 6-month history of a nonhealing perianal abscess on a background of 13 years of chronic sacral-buttock abscesses and discharging sinuses as part of hidradenitis suppurativa. After 2 surgical procedures and inadequate healing, a wide local excision was performed. The surgical specimen revealed an extensive, infiltrating, cystic and deeply penetrating, dermal and subcutaneous neoplasm with multiple fistulous tracts extending to the skin surface. The histopathology was consistent with carcinoma cuniculatum, a rare, slow growing, verucciform variant of squamous cell carcinoma. An entirely subcutaneous verrucous carcinoma of the sacrogluteal region is exceptionally rare, and this represents the first documentation of such a case in a female.

Obstetric and cryptoglandular rectovaginal fistulas: long-term surgical outcome; quality of life; and sexual function.

PURPOSE: Rectovaginal fistula (RVF) repair can be challenging. Additionally, women may experience sexual dysfunction and psychosocial ramifications even after a successful repair. The aim of this study was to investigate variables looking for predictors of healing/failure and examine long-term quality-of-life (QOL) and sexual function in women with low RVF from obstetrical or cryptoglandular etiology METHODS: From June 1997-2009, 268 women underwent RVF repair. Of those, 100 with obstetric or cryptoglandular etiology agreed to participate in this study. Healing, type of procedure, use of seton or stoma, number of previous procedures, smoking, age, body mass index (BMI), dyspareunia, QOL using SF-12, FIQL, IBS-QOL, and female sexual function index was obtained from our prospective database and telephone contact. Fisher's exact test, chi-square test, and multivariable-logistic-regression model were used to identify the variables associated with healing/failure. RESULTS: Mean follow-up was 45.8 ± 39.2 months; mean age 42.8 ± 10.5 years; and BMI was 28.8 ± 7.6. Sixty (60%) fistulas were obstetric and 40 (40%) cryptoglandular and 68/100 patients (68%) healed. On multivariate analysis, treatment failure was related to a heavier BMI (p = 0.001) and number of repairs (p = 0.02). Looking at each type of repair, episioproctotomy had significant healing compared to the other choices (but was not significant on multivariate analysis). Forty-seven women were sexually active at follow-up and 12/47 (25.5%) reported dyspareunia. Fecal incontinence was reported preoperatively in 42 women, more often in those with obstetric-related RVF (76% vs. 24% p < 0.05). Healing was not affected by age, smoking, co-morbidities, preoperative seton, or stoma use. Fecal and sexual function and QOL were comparable between women with healed and unhealed RVF. CONCLUSION: Patients with higher BMI and more repairs had a decreased healing rate following RVF repair. Despite surgical outcome, QOL and sexual function were surprisingly similar regardless of fistula healing.

Analysis of function and predictors of failure in women undergoing repair of Crohn's related rectovaginal fistula.

PURPOSE: Crohn's-related rectovaginal fistulae have significant impact on quality of life including sexual function. The aim of this study was to obtain long-term follow-up of Crohn's related rectovaginal fistulae to assess variables that influence surgical success and determine its effects on quality of life and sexual function. METHODS: All women with Crohn's-related rectovaginal fistulas who underwent surgical repair from 1997 to 2007 were contacted for long-term follow-up. Variables assessed were age, body mass index, smoking, presence of active Crohn's disease, type of surgical procedure performed, use of perioperative seton or stoma, number of previous procedures, time interval between last repair and current repair, use of immunomodulators, and steroids. SF-12, Fecal Incontinence Quality-of-Life Scale, and Female Sexual Function Index were used to assess quality of life and sexual function. Multivariable logistic regression model was used to identify variables associated with surgical failure. RESULTS: Sixty-five women were identified at median follow-up of 44.6 months (interquartiles, 13.1-79.1) of which 30 patients (46.2%) were successfully healed. Methods of repair included advancement flap (n = 47), episioproctotomy (n = 8), colo-anal anastomosis (n = 7), and fibrin glue or plug (n = 3). Twenty-eight women (43.1%) were sexually active at follow-up, and of those, nine complained of dyspareunia, all within the unhealed group of patients. On multivariate analysis, only immunomodulators were associated with successful healing (p = 0.009). Smoking and steroids were associated with failure (p = 0.04). Sexual function and quality-of-life scores were comparable between healed and unhealed groups. CONCLUSIONS: Crohn's-related rectovaginal fistulae are difficult to treat. Healing increased with use of immunomodulators; however, smoking and steroids were predictors of failure. Dyspareunia was higher in unhealed women.

Downstaging after chemoradiotherapy for locally advanced rectal cancer: is there more (tumor) than meets the eye?

PURPOSE: Preoperative chemoradiotherapy can lead to pathologic complete response of rectal cancer. This study was designed to determine the relationship between postchemoradiotherapy pathologic T stage (ypT stage) and nodal metastases and to evaluate whether pathologic complete response of the primary tumor results in sterilization of mesorectal lymph nodes. METHODS: Clinicopathological data from 1997 to 2007 of a prospectively maintained colorectal cancer database were examined. Inclusion criteria were patients with extraperitoneal rectal cancer who underwent preoperative chemoradiotherapy and subsequent radical resection. Statistical analysis was performed by use of Kruskall-Wallis and Wilcoxon rank-sum tests. RESULTS: Two hundred forty-two patients were identified (73.1% male, median age, 57 y (range, 36-85 y)). Data regarding preoperative chemoradiotherapy were available for 177 patients (73.1%). The median dose of radiotherapy was 5040 cGy (3060-6100 cGy). The mean preoperative radiotherapy dose and interval between chemoradiotherapy and surgery are similar when stratified by ypT stage (P = .55 and P = .72, respectively). Low anterior resection was performed in 174 patients (71.6%), and the remainder underwent abdominoperineal resection. A mural pathologic complete response was achieved in 62 patients (25.6%). In this pathologic complete-response group, positive lymph nodes were found in 2 patients (3.2%). The rate of metastatic lymph nodes increased as ypT stage increased (ypT1 = 11.1%, ypT2 = 29.2%, ypT3 = 37.3%). CONCLUSION: Patients with a mural pathologic complete response have a low rate of positive lymph nodes. These findings may have implications for the management strategies of these patients, including the use of local resection or a watch-and-wait policy. When the response to chemoradiotherapy is not complete, radical surgery should remain the treatment based on high rates of lymph node involvement.

Ureteric obstruction in familial adenomatous polyposis-associated desmoid disease.

PURPOSE: Intra-abdominal desmoid disease is the second leading cause of death in familial adenomatous polyposis patients. The aim of this study was to identify the incidence, management, and outcomes for familial adenomatous polyposis associated intra-abdominal desmoids causing ureteric obstruction. METHODS: Clinical data were abstracted from an institutional review board-approved, prospectively maintained familial polyposis registry. RESULTS: Of 107 patients identified with familial adenomatous polyposis related desmoid disease, 30 (28%) had documented CT scan evidence of ureteric obstruction. There was a 1:2.3 female predominance. Preceding surgery was the most prominent risk factor for development of desmoid disease (28 of 30 patients); 2 patients were diagnosed with desmoids before abdominal surgery. Overall, 11 patients had ureteric obstruction at the time of diagnosis. In the other 19 patients, median time from desmoid diagnosis to ureteric obstruction was 2 years. Pharmacologic management alone was effective in 8 patients. Eighteen patients (60%) underwent retrograde ureteric stent insertion. Five patients (17%) required percutaneous nephrostomy tubes. Three patients (10%) underwent autotransplant of 4 kidneys, and 4 patients (13%) required nephrectomy. One patient underwent ureterolysis, and another underwent ureteric resection with reimplantation. One-third of patients required more than one urologic procedure, and 63% had extensive small-bowel involvement with desmoid. CONCLUSIONS: The majority of patients with familial adenomatous polyposis associated desmoid disease who develop hydronephrosis require stenting. Complete obstruction may necessitate a nephrostomy. Renal autotransplant is an option for persistent symptomatic obstruction. Physicians treating patients with familial adenomatous polyposis and desmoid disease must be aware of the potential for development of ureteric obstruction and available treatment options.

Fatal haemorrhage from a caput medusae: a differential to a stabbing.

Fatal haemorrhage from caput medusae is reported once previously in the literature. We report the case of a 48-year-old man who presented to the ED in hypovolaemic shock, with a suspected stab wound to the abdomen. He was subsequently found to have exsanguinated from a cutaneous varix secondary to chronic liver disease.