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Unusual presentation of a malignant granular cell tumor of the pelvis: case report and literature review.

BACKGROUND: Malignant granular cell tumors are among the rarest of soft tissue cancers, currently understood to be of Schwann cell origin. As with their benign counterparts, malignant granular cell tumors (MGCTs) have a wide anatomic distribution and carry a poor prognosis, with recurrence and metastasis typically within 1 year of diagnosis. Only a handful of MGCTs have been described in the pelvis. CASE: We describe a case of malignant granular cell tumor that presented as a pararectal mass associated with severe rectal pain. The patient underwent pelvic exenteration and postoperative radiation therapy. She recurred with evidence of liver metastases on imaging studies 8 months following her exenteration. CONCLUSION: We discuss the diagnosis and prognosis of malignant granular cell tumors arising in the pelvis.

Combined Modality Therapy↗

Preoperative radiotherapy followed by radical vulvectomy with inguinal lymphadenectomy for advanced vulvar carcinomas.

A therapeutic alternative to exenteration for large locally advanced vulvar carcinoma involving the rectum, anus, or vagina is the use of preoperative radiation followed by radical surgery. Between 1980 and 1988, 13 patients with Stage III and 3 with Stage IV vulvar carcinoma involving the rectum/anus, urethra, or vagina were treated with 4000 rad to the vulva and 4500 rad to the inguinal and pelvic nodes followed by a radical vulvectomy and inguinal lymphadenectomy 4 weeks later. The overall 5 year cumulative survival was 45%. Twelve tumors regressed after radiation with 62.5% of the patients having visceral preservation while in 4 patients there was no major response to radiation and urinary or fecal diversion was required. Of the 6 recurrences 4 were central and 2 distant. Three patients with central recurrences had tumor within 1 cm of the vulvectomy margin. Complications included wet desquamation, inguinal wound separation, lymphedema, and urethral strictures. There were no operative deaths. It is concluded that the use of preoperative radiation followed by radical vulvectomy may be an alternative to pelvic exenteration in selected patients with advanced vulvar lesions.

Adult↗

Transvaginal parametrial needle biopsy for detection of postirradiation recurrent cancer of the cervix.

During the 27-year period from 1956 to 1982, transvaginal parametrial needle biopsies were performed on 76 patients clinically suspected of harboring postirradiation recurrent cervical cancer without mucosal lesions suitable for biopsy under direct vision. Needle biopsies indicated cancer in 41 patients, resulting in 20 abdominal explorations. Eleven of these patients underwent total pelvic exenteration. Three patients survived longer than 5 years. Among 35 patients whose biopsies did not suggest cancer, eight proved to be false-negative as demonstrated by later progression of local and systemic disease. The remaining 27 patients in whom biopsies showed negative results were cured by radiation therapy. Bleeding, easily controlled by vaginal packing, occurred in one patient; in another, a pelvic abscess was drained 7 days after the biopsy. The authors conclude that when judiciously performed, transvaginal parametrial needle biopsy has a high diagnostic yield (89%) with minimal morbidity (2.6%). Its application during the careful observation of patients suspected to harbor recurrence may increase the operability rate and may offer selected patients a chance for cure by radical pelvic surgery.

Adult↗

Contribution of surgery in patients with bulky residual disease after chemoradiation for advanced cervical carcinoma.

AIM: To report the outcome of 30 patients who underwent surgery after concomitant chemoradiation for locally advanced cervical cancer with residual disease > or = 2 cm. METHODS: From 1988 to 2004, 143 patients with FIGO stage IB2-IVA cervical cancer underwent surgery after concurrent chemoradiotherapy. Among them, 30 had a residual cervical tumour > or = 2 cm prior to surgery. Surgery consisted in a simple or radical hysterectomy (n=15) or in a pelvic exenteration (n=15). Endpoints were recurrence and distant metastasis rates, overall survival (OS) and disease-free survival (DFS) at 3 and 5 years. Analysis included FIGO stage, response to chemoradiation, para-aortic lymphatic status or type of surgery: palliative (remaining disease after surgery) or curative (no evidence of remaining disease after surgery). RESULTS: Surgery has been only palliative in 11 cases. Pelvic recurrences occurred in 8 patients after a median interval of 8.8 months. Distant metastases occurred in 8 patients after a median interval of 13 months. So far, 16 patients have died (53.3%). The 3-year and 5-year OS rates are 64.9% and 55.6%, respectively, for the 19 patients who had a curative surgery. The DFS rate is 50.8% at 3 and 5 years in this latter group. Overall 12 patients (40%) are alive and free of disease after a median follow-up of 32.5 months. CONCLUSIONS: Adjuvant surgery may improve the outcome of patients with bulky residual tumour after chemoradiation for locally advanced cervical cancer, allowing a 5-year OS of 55.6% after curative intervention.

Antineoplastic Combined Chemotherapy Protocols↗

[Clinical studies of ureteroileal junction stricture in ileal conduit].

Fifty eight patients who underwent ileal conduit formation in our hospital for the past 14 years were reviewed. Out of them, 11 patients developed hydronephrosis which should be treated. It occurred due to urolithiasis, recurrent tumor, contracted ileal loop in one patient respectively, and ureteroileal junction stricture in 8 patients (10 ureteral units). The 8 patients consisted of 6 males and 2 females. All of the males underwent total cystectomy because of bladder tumor (T.C.C.), and the females had anterior pelvic exenteration because of uterine cancer (adenocarcinoma). Out of 10 ureteral units, 7 were corrected by surgical intervention, 1 was dilated with endourological procedure, and 2 had no treatment. Six ureteral units including 1 ureteral unit treated by an endourological method resulted in improvement or disappearance of hydronephrosis. Histological examination of the stricture revealed fibrosis in 7 ureteral units treated surgically. According to our clinical studies, infection in the pelvic space seemed to be one of the causes which brought about ureteroileal junction stricture.

Adult↗

Pelvic and sacropelvic exenteration for locally advanced or recurrent anorectal cancer.

Postirradiation "fixed" anorectal tumors are often considered incurable. Since 1980, we have carried out 12 pelvic and seven sacropelvic exenterations for this problem (adenocarcinoma, 18; squamous cancer, one). Nine tumors were primary; ten were recurrent (five after an anterior resection and five after an abdominoperineal resection). Prior irradiation ranged from 3000 to 12,000 rad (30 to 120 Gy). Four patients had synchronous distant metastases; three died of disease (one with local recurrence), and the fourth patient has been living with disease (distant metastasis). Fifteen patients (four with B2 tumors and 11 with Astler-Coller C2 disease) had no extrapelvic disease. One patient died of postoperative complications; two others died free of disease. Three of the 15 patients died of disease (all with local recurrence), and one has been living with disease (local recurrence). Eight (53%) of 15 patients have been living free of disease 12+ to 53+ months. The results suggest that many patients with fixed postirradiation anorectal tumors may be salvaged by aggressive surgery.

Aged↗

Pain and quality of life after treatment in patients with locally recurrent rectal cancer.

PURPOSE: Because survival in patients with locally recurrent rectal cancer (LRRC) is limited, pain control and quality of life (QOL) are important parameters. The purpose of this study was to assess the prevalence of posttreatment pain and QOL of patients with LRRC treated with nonsurgical palliation or resection and identify predictors of poor outcome. PATIENTS AND METHODS: Posttreatment pain severity and QOL were prospectively assessed in 45 patients with LRRC using the Brief Pain Inventory and Functional Assessment of Cancer Therapy-Colorectal questionnaire. RESULTS: Fifteen patients received nonsurgical palliation, and 30 patients underwent resection of their pelvic tumors. There was a significant association between higher posttreatment pain scores and worse QOL (P <.001). Patients treated with nonsurgical palliation reported moderate to severe pain beyond the third month of treatment. Resected patients reported comparable levels of pain during the first 3 postoperative years, particularly after bony resections; long-term survivors (beyond 3 years), however, reported minimal pain and good QOL. Female sex, pelvic/sciatic pain at presentation, total pelvic exenteration, and bony resection were associated with higher rates of moderate to severe posttreatment pain (P =.04, P <.001, P =.04, and P =.02, respectively). Pain at presentation was an independent predictor of posttreatment pain (odds ratio, 7.4 [95% confidence interval, 1.8 to 30.3]; P =.006). CONCLUSION: Patients with LRRC treated with nonsurgical palliation or resection experience significant levels of pain after treatment. Close posttreatment pain monitoring is warranted in patients presenting with pelvic pain, and more aggressive pain management strategies may improve posttreatment QOL.

Activities of Daily Living↗

Preoperative chemoradiation therapy and anal sphincter preservation with locally advanced rectal adenocarcinoma.

Preoperative irradiation has been used to produce tumor regression and allow complete resection of rectal cancer with a sphincter-saving procedure. To evaluate the associated toxicity, the response in the primary tumor, and the postsurgical morbidity in a group of patients with locally advanced rectal cancer treated with preoperative chemoradiation therapy and low anterior resection, 120 patients were treated with 45 Gy of preoperative radiotherapy and a bolus infusion of 5-fluorouracil 450 mg/m2 on days 1 to 5 and 28 to 32 of radiotherapy. Four to six weeks later, 16 lesions were found unresectable; 36 patients underwent abdominoperineal resection or pelvic exenteration, and in the remaining 68 a low anterior resection was performed. For the purpose of this study only the latter group was included. There were 38 men and 30 women, with a mean age of 54.7 +/- 13.1 years. Gastrointestinal and hematologic acute toxicity grade 3 to 4 occurred in 12 and 7 patients, respectively. The mean distance of the tumor above the anal verge was 8.2 +/- 2.6 cm. In 10 patients the surgical resection included neighboring pelvic organs; 16 patients (23.5%) required a temporary diverting colostomy. The main causes of surgical morbidity were clinical anastomotic leakage in seven (10%), abdominal wall infection in five (7.4%), anastomotic stenosis in three (4.5%), and intraabdominal abscess in one (1.5%). No operative deaths occurred. The postsurgical stages were as follows: no tumor in the specimen, 17 (25%); T1, 4 (6%); T2, 12 (17%); T3, 17 (25%); T4, 5 (7%); any T with N+, 9 (13%); and any T, N with M+, 4 (6%). The median and mean follow-ups were 30.0 months and 37.4 +/- 25.0 months, respectively. The local recurrence rate was 2.9%, and the distant recurrence rate was 17%. The administration of preoperative chemoradiation therapy for locally advanced rectal cancer is associated with tolerable toxicity, a high rate of response in the primary tumor that allowed anal sphincter preservation, and a low rate of local recurrence.

Adenocarcinoma↗

Abdominosacral resection of recurrent rectal cancer in the sacrum.

PURPOSE: Resection of the sacrum is the only curative therapy of isolated sacral recurrence after primarily resected rectal cancer. The aim of the study was to assess whether there is a benefit in terms of overall survival, morbidity, and mortality when sacrum resection is performed more radically and in cooperation between colorectal and orthopedic surgeons. Possible prognostic factors were also assessed. METHODS: Twelve consecutive patients who underwent interdisciplinary partial sacral resection were included in a retrospective cohort study. Furthermore, overall survival rate and survival time were calculated. RESULTS: Histologic examination showed tumor-free resection margins in all cases. Extended resection was necessary in seven patients, including total pelvic exenteration in two. No perioperative death occurred and no patient required early reoperation. Complications were observed in 42 percent of patients, mainly caused by poor wound healing. All patients experienced relief from pain. One-year and three-year overall survival rates were 50 and 17 percent, respectively. The overall mean survival time was 21.7 months. Patients who died of recurrent disease within one year either underwent former resection for locoregional recurrence, had extensive local recurrent tumors affecting pelvic visceral structures, or retrospectively suffered from metastatic sacral tumor manifestation. CONCLUSION: The mortality and morbidity rates observed in the present study seem to justify partial sacral resection as a means to achieve palliation of perineosacral pain in spite of rare overall long-term survival.

Adenocarcinoma↗

Continent ileocolonic urinary reservoir (Miami pouch): the University of Miami experience over 15 years.

OBJECTIVE: A patient with a recurrent central pelvic malignancy after radiation will require urinary diversion as part of the reconstructive phase of the pelvic exenteration. The aim of our study was to assess the result of our 15-year experience with a continent ileocolonic urinary reservoir, which is known as the Miami pouch. STUDY DESIGN: Since 1988, all patients who received a continent ileocolonic urinary reservoir in the Division of Gynecologic Oncology, University of Miami School of Medicine, were included in the study. Parameters that were evaluated during the study period include functional outcomes, early and late perioperative complications, and their treatment. RESULTS: A total of 90 patients were identified from February 1988 to December 2002. Seventy-eight patients (87%) had a recurrent central pelvic malignancy, and 82 patients (91%) received radiation before the Miami pouch procedure. The non-reservoir-related morbidities were fever (76%), wound complication (30%), pelvic collection (12%), ileus/small bowel obstruction (12%), and postoperative death (11%). The most common reservoir-related complications were urinary infection (40%), ureteral stricture (20%), and difficulty with self-catheterization (18%). In our study, the overall complication rate that was related directly to the Miami pouch was 53%. Conservative treatment resolved>80% of these cases. The rate of urinary continence that was achieved in our patients was 93% during our 15-year experience with the Miami pouch. CONCLUSION: The Miami pouch is a good alternative for continent urinary diversion during exenteration or radiation-induced damage. The rate of major complications that require aggressive surgical intervention is acceptable. Most postoperative complications (80%) can be corrected with the use of conservative techniques that are associated with fewer deaths than reoperation and thus should be used first. The technique is simple and effective in women who are at high risk, who have undergone previous radiation therapy, and who have a high rate of functional success and is a profound advantage for a woman's psychosocial well-being.

Adult↗

Postoperative perineal hernia.

Eight patients (seven male and one female) with symptomatic postoperative perineal hernias treated at the Cleveland Clinic Foundation from 1975 to 1986 are reviewed. The patients ranged in age from 43 to 68 years (mean, 61 years). Seven had previous abdominoperineal resections of the rectum and one had a pelvic exenteration. The first two patients underwent repair via a perineal approach and both had recurrences of their hernias. These recurrences, as well as the presenting hernias in the six later patients, were repaired with mesh placed through an abdominal approach. Of these, there was one recurrence, which was repaired via an abdominal approach. There was no operative mortality. Based on experience, the authors believe that the best method of repair for this uncommon problem, involves placement of Marlex mesh across the pelvic inlet through an abdominal approach. This allows attachment of the mesh to the lateral musculature and posterior sacral periosteum under direct vision. The anterior borders of the mesh can be secured to the apex of the vaginal or prostatic tissue. The placement of metallic clips to the edges of the mesh allows for confirmation of the mesh position by radiographic examination during follow-up.

Aged↗

[Pelvic recurrence of rectal cancer: our experience].

Isolated recurrence of rectal carcinoma have been reported from 7% to 33% with a median of 15. Increasing recurrence is associated with increasing Dukes's stage. Patient who have recurrence after a low-anterior resection are more likely to present with non fixed, surgically correctable lesion versus recurrences after abdominoperineal resection. The most common symptom related to pelvic recurrence is pain, which may be perineal or radiate to the lower extremities. The diagnosis of a locally recurrent rectal cancer was obtained with CT; imaging is the first step to estimate the extent and location of the local tumor growth and the presence or absence of distant metastases. The most common location is at or around the anastomosis and the presacral region. Apart from distant metastases locoregional recurrence is the most important factor determining prognosis and survival. If an R0 resection can be performed, a 5-year survival rate of 20-30% can be achieved. Local or locoregional recurrence implies the reappearance of carcinoma after an intended complete removal of the tumor. For rectal cancer, the adjacent organs include the perineum, bladder and vagina, and LR failure includes perineal or pelvic lesions. Total pelvic exenteration is performed in patients with local recurrence of rectal cancer and a 5-year suvival rate of 30-40% was achieved. For patient with unresectable recurrence, chemotherapy and radiation contribute to a better quality of life and prolong survival. While radiotherapy may reduce recurrence, it is now apparent that total mesorectal excision is the most effective modality, with rates as low as 5%. The anastomotic recurrence that can be locally resected, the best approach for long-term survival is an extensive surgical procedure requiring en bloc removal of adjacent organs and pelvic structures so called composite resection. Intraoperative radiotherapy and brachytherapy, and/or preoperative chemoradiation may provide better results in future. While radioterapy remains the most common antineoplastic modality used for palliation of symptoms, surgical resection remains the mainstay of curative treatment for carcinoma of colon and rectum.

Aged↗

Concomitant chemoradiation prior to surgery in the treatment of advanced cervical carcinoma.

In patients with locally advanced cervical cancer, most of the treatment failures occur within the pelvis. In an attempt to improve local control, 40 patients with bulky tumors (stage IB > 5 cm, stage IIB with distal parametrial invasion, and stage III-IVA) were treated between 1988 and 1992 with concurrent chemoradiation (CCR). The whole pelvis received a midplane dose of 45 Gy over 33 days. Daily radiation dose was 1.8 Gy, with twice-daily fractionation in the last 20 patients. Chemotherapy was administered on the 1st and 21st days of radiation therapy (RT) consisting of cisplatin (60 mg/m2), followed by 5-fluorouracil (600 mg/m2/day continuous i.v. infusion) over 96 hr (and decreased to 40 and 400 mg/m2, respectively, in the last 23 patients). CCR was first followed by a single intracavitary application and then by a parametrial boost in stage IIB-III patients and in stage IVA patients with disease reaching the pelvis side wall. Then surgery (colpohysterectomy with lymphadenectomy or pelvic exenteration) was performed in 35 patients. Median follow-up time was 2.6 years (0.6-5.6 years). Acute toxicity (WHO grade 3-4 diarrhea) in 13 patients led to 6 RT interruptions and 4 incomplete RTs. One patient died of a septic episode without leukopenia after completion of CCR. Five postexenteration complications required a second surgical procedure, of which one patient died with tumor and small bowel fistula. One patient developed small bowel late complication and another patient developed urinary late complications. No postoperative or late complications were observed in patients treated with twice-daily fractionation. Pelvic control was achieved in 32 of 40 patients (81 and 74% in stage IB-IIB and stage III-IVA, respectively). Sites of failure were the pelvis (6 cases), metastases (7 cases), and both (2 cases). Two-year survival and DFS rates were 61 and 66%, respectively, in stage IB-IIB and 77 and 65% in stage III-IVA. High SCC-TA4 values significantly worsened DFS rates. In patients with stage III-IVA tumors, additional surgery could be an important component of this treatment strategy and may be compatible with CCR using twice-daily fractionation radiotherapy. However, these results must be confirmed by a large-scale prospective study.

Adult↗

Surgery for local pelvic recurrence after resection of rectal cancer.

This retrospective study evaluated outcome with regard to procedure, local control, and survival after curative surgical resection with and without preoperative radiotherapy for local pelvic recurrence. A total of 58 consecutive patients with local pelvic recurrence of rectal cancer after previous curative resection for primary tumors were reviewed. Of these, 36 underwent both initial resection and follow-up in our department; the remaining 22 had initial surgery and follow-up elsewhere. Of the 58 patients 27 underwent curative re-resection, 9 had palliative resection, and 22 were treated by conservative therapy. Among the 27 patients with curative resection 17 received preoperative radiotherapy (40 Gy) plus surgery and 10 surgery only. No patients were lost to follow-up; median follow-up time was 36.3 months. The overall rate of curative resection was 46.6%: 55.6% in our own follow-up group and 31.8% in the others. With regard to surgical procedure, abdominoperineal resection (APR) with or without sacral resection was standard following previous low anterior resection, and total pelvic exenteration (TPE) with or without sacral resection was common following APR. There was a high incidence of morbidity (71.4%) after TPE. Re-recurrence was observed in 12 (44.4%) after curative re-resection. There was local re-recurrence in 6 (22.2%). The local re-recurrence rate was 11.8% (n = 2) with radiotherapy plus surgery, and 40.0% (n = 4) with surgery alone. The estimated 5-year survival following curative re-resection was 45.6% (61.2% with radiotherapy plus surgery, 29.6% with surgery alone). Both survival and local control with radiotherapy plus surgery tended to be better than with surgery alone. Thus, in selected patients pelvic local recurrence of rectal cancer can be re-resected curably by APR or TPE (with or without sacral resection) combined with preoperative radiotherapy.

Adult↗

Postoperative perineal hernia.

PURPOSE: Perineal hernia is an uncommon complication following abdominoperineal resection. The aim of the study was to evaluate the predisposing factors and the optimum method of repair. METHODS: A retrospective review of patients with postoperative perineal hernia at the Massachusetts General Hospital between 1963 and 1995 was performed. RESULTS: Twenty-one patients with perineal hernias were found. The original perineal operations were as follows: abdominoperineal resection in 13 patients, pelvic exenteration in 5 patients, cystourethrectomy in 2 patients, and perineal resection of the rectal stump in 1 patient. The incidence of symptomatic perineal hernia following abdominoperineal resection was estimated to be 0.62 percent. A total of 69 percent of patients had the original perineal wound left partially open, and in 10 percent it was left completely open. The peritoneal defect was not closed in 53 percent of patients, and only 21 percent had closure of the levator defect. Of the 19 patients who had hernia repair, 13 were repaired transperineally and 3 transabdominally and 3 required a combined abdominoperineal approach. The repair methods were as follows: simple closure of the pelvic defect (10 patients), mesh closure (5 patients), gluteus flap (1 patient), and retroflexion of the uterus (2 patients) or bladder (1 patient). Four patients had postoperative complications (mostly wound infections), and the recurrence rate was 16 percent. There was no difference in length of hospitalization among transperineal, transabdominal, and combined approaches. CONCLUSIONS: Primary closure of the perineal wound, with careful avoidance of wound infection is the most important consideration for avoiding a perineal hernia. Repair via the perineum with simple closure of the defect or a mesh is successful in most cases.

Abdomen↗

Primary radiation, cisplatin, and 5-fluorouracil for advanced squamous carcinoma of the vulva.

OBJECTIVE: To evaluate a regimen of radiation and chemotherapy as an alternative for those patients in whom the location and extent of advanced vulvar carcinoma make pelvic exenteration the only surgical option. METHODS: Between December 1988 and March 1995, 14 patients with primary squamous carcinoma of the vulva who were not candidates for standard radical vulvectomy were treated with radiation therapy in combination with cisplatin and 5-fluorouracil (5-FU) chemotherapy at the Albany Medical Center. Patients ranged in age from 40 to 90 years, mean 68. Tumors were stage III in 9 patients and stage IV in 5 patients. Treatment included two cycles of chemotherapy with cisplatin (50 mg/m2) and 5-FU (1000 mg/m2/24 x 96 hr) in addition to radiation therapy. Total radiation doses to the vulva and groins ranged from 50 to 65 Gray (Gy), with pelvic doses of 45 to 50 Gy. Surgical excision of the primary site was not performed in patients who had complete clinical response. RESULTS: Acute complications included desquamation requiring treatment interruptions in 5 patients and deep venous thrombosis in 1 patient. Delayed complications were limited to small bowel obstruction and colonic stricture in one patient. There was a 92% response rate with complete responses in 9 patients (64%). Among patients with complete clinical response, there has been only one recurrence with follow-up of 7-81 months, mean 36.5. All patients with partial responses died, with survival of 8-25 months, mean 15.7. CONCLUSIONS: This combination of chemoradiation was found to be effective therapy for locally advanced vulvar carcinoma, with acceptable morbidity even in an elderly population. Surgical excision of the primary site is not necessary in patients with complete response.

Adult↗

Ultrasonography and computed tomography for diagnosis and follow-up of pelvic rhabdomyosarcomas in children.

The authors report 18 cases of pelvic rhabdomyosarcomas in children examined at presentation and followed on US and CT. There were 11 boys, 7 girls; tumor location was bladder, prostate or uterus in 12, perineum in 4, buttock in 2. US is good to define tumoral extension within the bladder CT is better to delineate the extension outside the bladder and regional spread to iliac nodes. CT is the only useful examination for perineal tumors. During chemotherapy US can be frequently repeated to appreciate tumoral regression and to give measurement of the mass. Preoperative CT should be performed when tumoral regression seems sufficient to permit complete tumoral excision without pelvic exenteration. Correlation between US and CT and surgical findings proved to be good. US and CT are also very useful in the follow-up when the child has finished treatment as it can demonstrate a local recurrence before clinical symptoms appear. However, post-surgical and post-radiation changes may be difficult to recognize. These aspects are discussed.

Adolescent↗