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Vaginal adenosarcoma arising from endometriosis.

OBJECTIVE: Malignant transformation of endometriosis has been well documented. Endometrioid adenocarcinoma is the most common malignancy to occur in this setting, although other carcinomas and rarely stromal tumors can be seen. We present the first case in the literature of adenosarcoma, a rare mixed mullerian or mesodermal tumor, arising in extrauterine vaginal endometriosis. CASE: A 42-year-old woman underwent multiple medical therapies and surgeries for aggressive endometriosis. A pelvic exenteration was abandoned secondary to severe fibrosis, and low-dose radiotherapy was used to control bleeding from vaginal endometriosis. The pathologic diagnosis of recurrent endometriosis was confirmed multiple times over her 4-year course. Excision of a recurrent vaginal mass revealed adenosarcoma with heterologous elements. CONCLUSION: It is important to biopsy or excise recurrent endometriosis, as malignant transformation can occur, giving rise to epithelial, stromal, or mixed epithelial-mesenchymal tumors.

Adenosarcoma↗

Procedures required to accomplish complete cytoreduction of ovarian cancer: is there a correlation with "biological aggressiveness" and survival?

OBJECTIVE: The aim of this study was to determine if the necessity of using specific procedures to attain complete cytoreduction in ovarian cancer correlates with innate biologic aggressiveness and independently influences survival. METHODS: Between 1990 and 2000, 213 patients with Stage IIIC epithelial ovarian cancer underwent complete cytoreduction before initiation of systemic platinum-based combination chemotherapy. Survival was stratified and analyzed (log rank and Cox regression) on the basis of whether extrapelvic bowel resection, diaphragm stripping, full-thickness diaphragm resection, modified posterior pelvic exenteration, peritoneal implant ablation and/or aspiration, and excision of grossly involved retroperitoneal lymph nodes were necessary to attain a visibly disease-free cytoreductive outcome. RESULTS: The median and estimated 5-year survival for the cohort were 75.8 months and 54%, respectively. Survival was influenced (log rank) by the requirement of diaphragm stripping (required, median 42 months vs not required, median 79 months; P = 0.03) and the extent of mesenteric and serosal implants that required removal (none, median not reached, vs 1-50 implants, median not reached, vs >50 implants, median 40 months; P = 0.002). Survival was independently influenced (Cox regression) only by the extent of peritoneal metastatic implants that required removal (P = 0.01). The other investigated procedures and type of chemotherapy used did not influence survival. CONCLUSIONS: The need to remove a large number of peritoneal implants correlates with biological aggressiveness and diminished survival, but not significantly enough to preclude long-term survival or justify abbreviation of the operative effort. The need to use the other investigated procedures had minimal or no observed influence on survival.

Adult↗

Primary invasive vaginal cancer in the setting of the Mayer-Rokitansky-Kuster-Hauser syndrome.

BACKGROUND: The Mayer-Rokitansky-Kuster-Hauser syndrome occurs in 1 in 4000 to 5000 female births. Primary vaginal cancer constitutes less than 2% of all malignancies of the female genital tract. A report of the first case of the unlikely occurrence of both of these developments in the same patient is presented. CASE: A 34-year-old nulligravid Philippine woman with a history of Mayer-Rokitansky-Kuster-Hauser syndrome presented with a 5-month history of bleeding from a blind vaginal pouch. Vaginal biopsy identified a moderately differentiated endometrioid adenocarcinoma. Exploratory laparotomy, bilateral salpingo-oophorectomy, pelvic and iliac lymph node samplings, and excision of a mullerian remnant were performed with no evidence of disease. A FIGO Stage I vaginal cancer was assigned and pelvic irradiation was given. Disease recurred 4 months later and the patient underwent total pelvic exenteration. More than 1 year since the exenteration procedure, she is without evidence of disease. CONCLUSION: This is the first reported case of a primary vaginal cancer in a patient with Mayer-Rokitansky-Kuster-Hauser syndrome. It is a reminder that routine gynecologic examinations are still warranted as these patients are at risk for malignant changes in residual mullerian tissues.

Abnormalities, Multiple↗

Reduction of blood loss during extensive pelvic procedures by aortic clamping--a preliminary report.

OBJECTIVE: The aim was to determine the effect of intraoperative aortic clamping during extensive pelvic procedures on blood loss, operative time, and morbidity. METHODS AND MATERIALS: Thirteen women with ovarian cancer, 1 with cervical cancer, and 1 with an extensive pelvic sarcoma had their aortas completely occluded with a vascular clamp before the pelvic phases of their operations. Heparin and protamine reversal were used. RESULTS: Patients requiring en bloc excision of the internal reproductive organs, pelvic peritoneum, and recto-sigmoid colon in the context of a cytoreductive operation had a median estimated total blood loss of 650 ml (range 200 to 3500), a median of 2 units (range 0 to 8) of blood transfused, and a median total operative time of 155 min (range 90 to 280). There were no complications due to the aortic clamping. CONCLUSION: Most procedures were completed with a less than anticipated blood loss and operative time. Clamping of the aorta may potentially diminish blood loss, operative time, and the incidence of transfusion-related morbidity associated with extensive pelvic operations. Intraoperative aortic clamping merits further investigation.

Adult↗

The unusual presentation of mucinous carcinoma in the colon making preoperative diagnosis difficult: report of a case.

A 51-year-old man was admitted to our hospital for investigation of fever, lower abdominal pain, and pain on micturition. Barium enema and endoscopic findings revealed an extracanal large cavity of the rectosigmoid colon, which suggested an abscess caused by penetration. Histological examination of the endoscopic biopsy specimen demonstrated no malignancy. A preoperative diagnosis of malignant lymphoma was strongly suspected from the imaging findings; however, intraoperative inspection revealed a large tumor invading the urinary bladder. An intraoperative incisional biopsy was performed and histological examination demonstrated mucinous carcinoma. The patient subsequently underwent pelvic exenteration about 1 week later followed by adjuvant chemotherapy and radiotherapy.

Adenocarcinoma, Mucinous↗

Leiomyosarcoma of the rectum and anus. A series of 22 cases.

Twenty-two patients with leiomyosarcoma of the rectum (n = 19) or the anus (n = 3) were treated surgically at the Mayo Clinic from 1950 through 1985. The majority of tumors occurred in men (1.4:1.0) during the sixth and seventh decades of life. Fifty-nine percent of the patients had symptoms including, most commonly, change in bowel habit, bleeding, and pain. Wide local excision was performed in 10 patients, whereas a more radical surgical procedure, including abdominoperineal resection (n = 8), pelvic exenteration (n = 2), and low anterior resection (n = 1), were performed in 11 patients. One tumor was unresectable. The overall survival until death from disease was 90% at 1 year, 74% at 5 years, and 51% at 10 years postoperatively. The percentage of patients free of disease at 1, 5, and 10 years postoperatively was 85, 62, and 40, respectively. Wide local resection was not superior to a more radical surgical approach in preventing tumor recurrence or improving survival. Lesions less than 2.5 cm and confined to the bowel wall can be treated by wide local excision, whereas larger or more extensive tumors should be treated by a more radical surgical approach.

Actuarial Analysis↗

Neoadjuvant external beam radiation and proctectomy for adenocarcinoma of the rectum.

PURPOSE: The aim of this study was to determine the survival rate, local failure, and perioperative morbidity in patients with adenocarcinoma of the rectum undergoing curative proctectomy who were felt to have transmural disease on preoperative assessment. Eighty-nine percent of these patients were treated with preoperative external beam radiotherapy. METHODS: The records of 191 consecutive patients undergoing abdominal surgical procedures for primary treatment of rectal cancer were reviewed. The product-limit method (Kaplan-Meier) was used to analyze survival rate and tumor recurrence. RESULTS: One patient was excluded from survival analysis because of incomplete record of tumor stage. The study population comprised 109 males and 81 females, median age 64 (range, 33-91) years. Curative resection was performed in 152 of these 190 patients (80 percent), including low anterior resection with coloproctostomy or coloanal anastomosis (n = 103), abdominoperineal resection (n = 44), Hartmann's procedure (n = 4), and pelvic exenteration (n = 1). Mean follow-up of patients undergoing curative resection was 96 +/- 48 months. Palliative procedures were performed in 38 of 190 patients (20 percent). Perioperative mortality was 0.5 percent (1/190). Complications occurred in 64 patients (34 percent). The anastomotic leak rate was 4 percent (5/128). Disease-free five-year survival rate by pathologic stage was as follows: Stage I, 90 percent; Stage II, 85 percent; Stage III, 54 percent; Stage IV, 0 percent; and no residual tumor, 90 percent. Of the 152 patients treated with curative resection, disease-free survival rate was 80 percent at five years. Preoperative external beam radiation was administered to 135 of these 152 patients (89 percent). Tumor recurred in 32 of 152 patients (21 percent) treated with curative resection. The predominant pattern of recurrence was distant failure only. Kaplan-Meier overall local recurrence (local and local plus distant) at five years was 6.6 percent. The local recurrence rate paralleled tumor stage: Stage I, 0 percent; Stage II, 6 percent; Stage III, 20 percent; and no residual tumor, 0 percent. CONCLUSION: Preoperative external beam radiotherapy and attention to mesorectal dissection can achieve low local recurrence and excellent long-term survival rate in patients with adenocarcinoma of the rectum. Moreover, these goals can be obtained with low morbidity and mortality.

Adenocarcinoma↗

Extended follow-up of patients treated with cytotoxic chemotherapy for intra-abdominal desmoid tumors.

BACKGROUND: Cytotoxic chemotherapy can achieve a good initial response in inoperable desmoid tumors that have caused progressive obstruction of the gastrointestinal and urinary tracts and have caused unrelenting pain. METHODS: We have reviewed 8 patients (3 male) with desmoid tumors and familial adenomatous polyposis who underwent cytotoxic chemotherapy for inoperable gastrointestinal obstruction and/or uncontrolled pain. They were treated with doxorubicin and dacarbazine followed by carboplatin and dacarbazine. RESULTS: Follow-up after cytotoxic chemotherapy in the 7 patients for whom it was available was a mean of 42 (range 24-54) months. Two patients achieved complete remission after therapy. Four patients achieved a partial remission after completing all or some of the chemotherapy regimen; of these, three remained in stable remission, whereas the other was lost to follow-up. There were two recurrences that required further therapy; one of these patients was treated with further chemotherapy, which induced a second remission, and the other was treated with pelvic exenteration and has subsequently died. CONCLUSIONS: Most patients had a substantial response to cytotoxic chemotherapy; however, two patients required additional therapy 24 and 30 months after cytotoxic chemotherapy, respectively. Cytotoxic chemotherapy is effective in producing short-term and long-term remission in these difficult patients.

Adenomatous Polyposis Coli↗

p53 protein overexpression and response to induction chemoradiation therapy in patients with locally advanced rectal adenocarcinoma.

BACKGROUND: The association between mutations in the p53 gene and prognosis in colorectal cancer remains controversial. This report evaluates the role of p53 protein to predict the response of neoadjuvant chemoradiation therapy in patients with primary locally advanced rectal adenocarcinoma. METHODS: Between January 1993 and December 1994, 26 patients were seen with locally advanced primary rectal adenocarcinoma, located between 0 and 10 cm from the anal verge, demonstrated clinically and by CT scan. Each received 45 Gy of preoperative radiation therapy (RT) concomitantly with bolus infusion of 5-fluorouracil (5-Fu) (450/mg/m2 on days 1 to 5 and 28 to 33 of RT). Surgery was performed between 4 and 8 weeks later. All the primary tumors were mapped and sliced. The response rate was divided according to the percentage of malignant cells in the rectal wall and perirectal fat. Lymph nodes were studied with the manual or modified clearing technique. p53 mutant status was assessed immunohistochemically from sections of the formalin-fixed, paraffin-embedded pretreatment biopsy and the resected specimen. RESULTS: There were 14 females and 12 males, with a mean age of 54 years. All received the scheduled treatment. An abdominoperineal resection (n = 10), low anterior resection (n = 10), and pelvic exenteration (n = 6) were performed. The stages of tumors were as follows: no residual tumor (n = 4); T2 (n = 6); T3-4 (N = 9); and T3-4, N1,2 (n = 7). Fourteen specimens (54%) had mutated p53, and 10 (71%) had >50% of residual tumor, whereas only two (17%) of the specimens with normal p53 had >50% of residual tumor (P = .018). Eight of the 10 low anterior resections were performed in patients whose specimens expressed normal p53. CONCLUSION: Our results suggest that the determination of p53 is a factor in predicting tumor response in patients who undergo preoperative chemoradiation therapy for rectal adenocarcinoma.

Adenocarcinoma↗

Surgical treatment and late results in 1226 cases of colorectal cancer.

One thousand two hundred twenty-six cases of colorectal cancer were treated surgically between 1956 and 1978. Seven hundred ninety-eight cases (65.08 per cent) were rectal, 74.3 per cent of which were located extraperitoneally. Dukes' C2 cases and cases with distant metastasis constituted 39.15 per cent, and Dukes' A cases, 9.22 per cent. Resectability rate was 75.1 per cent (77.6 per cent for rectal cancer). Two hundred and four cases (32.96 per cent) of rectal excision were restorative resections. Of the combined excisions for extraperitoneal lesions in females, 84.8 per cent were posterior pelvic exenterations. The overall operative mortality rate was 2.93 per cent. The mortality rate for 921 cases of resection was 1.73 per cent, for rectal resection, 0.8 per cent, and for curative rectal resection, 0.63 per cent. The follow-up rate was 94.13 per cent. The five-and ten-year survival rates for rectal resection were 53.08 +/- 2.29 per cent and 47.65 +/- 2.44 per cent; for curative rectal resection. 66.91 +/- 2.54 per cent and 60.27 +/- 3.03 per cent; and for Dukes' A cases, 98.05 +/- 1.35 per cent and 96.39 +/- 2.13 per cent. The five-and ten-year survival rates for colonic resection were 59.79 +/- 2.04 per cent and 52.18 +/- 3.49 per cent; for curative colonic resection, 72.79 +/- 3.39 per cent and 62.06 +/- 4.17 per cent; and for Dukes' A cases, both 100 per cent. Besides the extent of spread and degree of malignancy of a lesion, the local immunologic reaction of the host is also important in prognosis. The more lymphocytic infiltration in and around the cancer, the more follicular hyperplasia and sinus histiocytosis in regional lymph nodes, the better is the prognosis. The problem of anal preservation in radical resection of rectal cancer and the problem of improvement of results in the treatment of extraperitoneal rectal cancer are discussed in detail.

Adult↗

[Mesonephric carcinoma of the urethra. A case report].

Mesonephric carcinoma is a tumor entity that rarely develops in the neck of the uterus and vaginal wall and is extremely unusual in the urinary bladder or paraurethral tissue. The tumors arise in the remnants of the wolffian or mesonephric duct. The malignant potential is considered to be lower than in tumors of the urethra with different histologic findings. Anterior pelvic exenteration is recommended in the literature as the treatment of choice. In the case described here, the tumor presented as a space-occupying lesion in a urethral diverticulum exhibiting striking clinical features of macrohematuria and urinary retention with subvesical obstruction of the urethra. In accordance with reports in the literature, cystectomy was performed and a Kock pouch constructed for urinary diversion. Tumors occurring in a diverticulum-and in this case manifesting as the rare tumor form of mesonephric carcinoma-should always be included in the differential diagnosis of macrohematuria in women.

Aged↗

Urinary retention caused by adenocarcinoma arising in recurrent urethral diverticulum.

We present a case of adenocarcinoma arising in a urethral diverticulum. A 71-year-old woman presented with urinary retention and a mass in the anterior vaginal wall. A biopsy was performed and revealed adenocarcinoma. Anterior pelvic exenteration with continent urinary diversion was performed followed by adjuvant radiation therapy as the tumor was large and poorly differentiated. She developed stomal stenosis and underwent stomal revision. The patient has done well since, with no evidence of recurrence at 1-year follow-up at the time of this report.

Adenocarcinoma↗

Virtual reality of the lower urinary tract in women.

Advances in computerized and imaging technology permit both students and doctors to depict the anatomy of the human pelvis more realistically than with previous methods. Further refinements outline fine pelvic structures, such as the nerve plexus, which may as a result be spared during major pelvic surgery, thus preserving the function of the bladder neck and urethra. Dynamic computerized tomography or magnetic resonance imaging, coupled with three-dimensional depiction of the lower urinary tract and its adjacent structures, enable visualization of the whole lower urinary tract and the pelvic floor musculature in both continent and incontinent women. In patients with a reconstructed lower urinary tract computer-assisted image processing shows the postoperatively altered topographical anatomy. This may be clinically useful for interpretation of unexpected findings with conventional imaging modalities, postoperative morbidity, and surgical planning of a lower abdominal reoperation. Examples of our own work regarding the innervation of female pelvic organs, dynamic depiction of the bladder and pelvic floor musculature during straining in normal and incontinent women, and the situation of female patients after undergoing an anterior pelvic exenteration with subsequent orthotopic neobladder procedure, are given. In addition, the data of these patients have been compiled for virtual reality endoscopy, which is useful for patient consent and for teaching residents, students and nurses.

Female↗

Total cystectomies in the surgical treatment of rectal cancer with prior chemoradiation: analysis of postoperative morbidity and survival.

BACKGROUND AND AIMS: Curative surgery for rectal cancer seldom requires urinary tract resections. The study investigated morbidity and survival following resection of rectum with total cystectomy following chemoradiation for primary rectal cancer. PATIENTS AND METHODS: 19 consecutive patients with primary nonresectable rectal cancer undergoing preoperative chemoradiation and operated on by a multidisciplinary team of surgeons. RESULTS: Morbidity was moderately low, and only five cases required surgical reintervention. No postoperative deaths were observed. Long-term survival in this group of patients compares well with the survival of patients with primarily nonresectable rectal cancer without the involvement of urinary bladder. CONCLUSION: Extended pelvic exenteration due to rectal cancer is relatively safe and in selected patients offers long-term survival and a chance of a cure. Involvement of the urinary bladder does not adversely affect outcome of rectal cancer treatment.

Adult↗

Laparoscopic repair of postoperative perineal hernia.

Perineal hernia is an uncommon complication of abdominoperineal resection and pelvic exenteration. We present an alternative not previously described for the surgical repair of this type of hernia: laparoscopic repair of postoperative perineal hernia.

Herniorrhaphy↗

Early postoperative oral feeding after colectomy: an analysis of factors that may predict failure.

BACKGROUND: Previous studies have shown that early postoperative oral feeding is feasible. Traditionally patients were fed when flatus or defecation documented the return of bowel function. This study was undertaken to determine factors that may preclude early feeding. METHODS: One hundred four successive patients underwent colorectal surgery from October 1999 to January 2001. Eighty-nine patients started an oral diet either on postoperative day 1 or 2. Their clinical outcomes were recorded prospectively. Fifteen of the 104 patients were excluded for small-bowel resection (5 patients), perioperative complications (5 patients), prior radiation (3 patients), and small-bowel obstruction (2 patients). A failure in postoperative feeding consisted of nausea, vomiting, or readmission. RESULTS: The mean age of our cohort was 65 years (range, 28-87 years). There were 45 male and 44 female patients. The mean postoperative hospital stay was 6 days (range, 3-13 days). The median American Society of Anesthesiology score was II (range, I-IV). The types of resection performed were right colectomy (27 patients), low anterior resection (26 patients), sigmoid resection (11 patients), abdominoperineal resection (8 patients), formation or closure of colostomy (7 patients), posterior pelvic exenteration (4 patients), total colectomy (3 patients), left colectomy (2 patients), and transverse colectomy (1 patient). Sixty-five patients (73%) tolerated early oral feeding. Of the 24 patients that did not, 16 had nausea or emesis, and 8 required readmission for postoperative complications (small-bowel obstruction [4 patients], wound dehiscence [1 patient], abdominal pain [1 patient], and anastomotic leak [2 patients]). Univariate analysis revealed that the use of volume expanders contributed to intolerance of early feeding. On multivariate analysis, blood loss during the operation was the only factor contributing to failure of early postoperative oral feeding. CONCLUSIONS: Early oral feeding is safe and feasible for postcolectomy patients with a history of colorectal neoplasms.

Adult↗

Surgical management of gynecologic rhabdomyosarcoma.

The multidisciplinary treatment strategy for rhabdomyosarcoma (RMS) of the female gynecologic system is evidence of the progress made in the management of many childhood cancers. Previously, initial radical surgery (eg, complete vaginectomy, hysterectomy, and pelvic exenteration) and less effective single-agent chemotherapeutic agents were used with suboptimal results. Over the past 30 years, there has been an increase in overall survival with gynecologic RMS and a significant decrease in the hysterectomy and vaginectomy rates. The usual recommended treatment strategy for gynecologic RMS is initial biopsy to establish the diagnosis, followed by neoadjuvant chemotherapy. The specific chemotherapy regimen is selected based on the risk stratification of the particular patient, and this will often allow a more conservative surgical approach to be used, with the goal of complete tumor resection. Radiation therapy is considered before definitive surgical resection with minimal response to chemotherapy or after definitive surgical resection with positive margins. Recurrent or persistent RMS, including RMS of the female gynecologic system, represents a very aggressive disease with poor outcome. In these cases, aggressive management, including experimental chemotherapy agents and organ-sacrificing surgery, is often indicated to afford any chance of long-term survival. Continued enrollment of a high proportion of these patients into ongoing prospective clinical trials should allow the outcomes to continue to improve.

Antineoplastic Combined Chemotherapy Protocols↗