Primitive neuroectodermal tumor of the kidney associated with Budd-Chiari syndrome in a 17-year-old girl.

Renal primitive neuroectodermal tumor (PNET) is a rare and highly malignant neoplasm of the kidney. We report the case of a 17-year-old girl with renal PNET that was complicated by Budd-Chiari syndrome. She was admitted due to abrupt left flank pain and gross hematuria. Abdominal sonography and computerized tomography (CT) disclosed a large hemorrhagic left renal mass and thrombus in the inferior vena cava (IVC). Left radical nephrectomy was performed and renal PNET with tumor rupture and tumor invasion into the IVC was diagnosed based on operative findings and histologic features. Tumor cells were positive for neuronspecific enolase, chromogranin-A, and vimentin but negative for cytokeratin, leukocyte common antigen, CD3, and CD20. The thrombus in the IVC extended into the right atrium and caused obstruction of the right and middle hepatic venous outflow, which was evident on follow-up CT scan 5 months later. The patient died due to hepatic failure and progressive cardiovascular compromise 6 months after surgery. This case demonstrates that renal PNET can be life threatening when the tumor thrombus extends into the IVC and causes hepatic outflow obstruction.     

Aggressive angiomyxoma of the vulva: a case report and review of literature

Background Aggressive angiomyxoma (AA) is a rare vulvovaginal mesenchymal neoplasm with a marked tendency to local recurrence but which usually does not metastasize. Case report We describe a case of AA in the left labium majus pudendi in a 47-year-old woman who underwent incomplete surgical excision. Follow-up 2 years later revealed no recurrence. Conclusion In the past, most authors advocated wide excision even if genitourinary and digestive tract resection were necessary. These days, a less radical surgery is recommended, but the significance of hormonal treatment and/or radiation therapy is not clear yet. Further investigation is necessary.     

Laparoscopic management of ureteral endometriosis: our experience

STUDY OBJECTIVE: Ureteral endometriosis is rare, accounting for less than 0.3% of all endometriotic lesions. The aim of our study is to evaluate the prevalence of extrinsic ureteral endometriosis in women undergoing laparoscopic surgery for severe endometriosis and to suggest that laparoscopic ureterolysis represents a mandatory measure in all cases to avoid ureteral injury. METHODS: A retrospective analysis was performed of all cases of patients who underwent laparoscopic surgery for severe endometriosis at the departments of obstetrics and gynecology at CMCO-SIHCUS and Hautepierre Hospital, Strasbourg, from November 2004 through January 2006. MEASUREMENTS AND MAIN RESULTS: We recorded 54 patients with a mean age of 31 years and a mean body mass index of 21.9. Reported symptoms were dysmenorrhea (88%), severe dyspareunia (88%), severe pelvic pain (38.8%), and infertility (74%). Five women presented with dysuria, frequency, recurrent urinary tract infections, and pain in the renal angle, and 2 patients had hydronephrosis. We observed 3 patients (5.6%) with ureteral stenosis, 35 (64.8%) with adenomyotic tissue surrounding the ureter without stenosis, and 16 (29.6%) with adenomyotic tissue adjacent to the ureter. It was on the left side in 47.4% of cases, on the right side in 31.6% cases, and bilaterally in 21% of cases. In 9 patients, ureteral involvement was associated with bladder endometriosis (16.7%). In all patients, ureterolysis was performed. There was 1 case of ureteral injury during the procedure, 2 of transitory urinary retention, and 1 of uretero-vaginal fistula after surgery. During the first year of follow-up, the disease recurred in 4 patients, with no evidence of the disease in the urinary tract. CONCLUSION: Conservative laparoscopic surgery to relieve ureteral obstruction and remove pathologic tissue is the management of choice. Resection of part of the ureter should be performed only in exceptional cases. Ureterolysis should be performed in all patients before endometriotic nodule resection to recognize and prevent any ureteral damage.     

Scar recurrence of uterine sarcoma: a case report

BACKGROUND: Uterine leiomyosarcoma is an aggressive tumor that has a propensity for recurrence. Most of the recurrences occur at either pelvic or distant sites, such as lung or liver. Recurrences in the laparotomy scar are extremely rare. CASE: A 52-year-old woman underwent hysterectomy for stage I, grade 2 uterine leiomyosarcoma. She did not receive any adjuvant treatment. She presented 2 years later with wound recurrence. She had no evidence of intraabdominal disease. She underwent radical resection with mesh repair. Pathology revealed high grade spindle cell sarcoma very similar to the previous cancer. After completion of 18 months of follow-up, she was disease free. CONCLUSION: Radical resection of isolated metastases may be of benefit for these patients.     

Primary synovial sarcoma of the kidney.

Primary synovial sarcoma arising from the kidney is extremely rare. We report two cases with primary renal synovial sarcoma. Both were initially diagnosed as renal cell carcinoma. The first case was a 30-year-old woman who presented with right flank soreness. Ultrasonography disclosed a multiloculated cystic tumor measuring 9 x 7 cm. She underwent hand-assisted laparoscopic radical nephrectomy; there was no recurrence during 15 months of follow-up. The second case was a 49-year-old woman who presented with a palpable mass in the left upper quadrant of the abdomen of 1 month's duration. Computed tomography showed a heterogeneously enhanced tumor measuring 13 x 11 cm at the left retroperitoneum with displacement of the pancreas and the left kidney. Hand-assisted retroperitoneoscopic radical nephrectomy was performed. She had no evidence of recurrence after 27 months of follow-up. Pathology of the two cases showed histologic and immunochemical features of synovial sarcoma with coexisting spindle and epithelial cells. Physicians should be aware of the possibility of malignancy in cystic renal masses and that synovial sarcoma is one of the possibilities.     

Hemivaginal Septum Resection in a Patient With a Rare Variant of Herlyn-Werner-Wunderlich Syndrome

Herlyn-Werner-Wunderlich syndrome is characterized by a triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. The syndrome should be suspected in any young woman with abdominal pain or cyclic dysmenorrhea, and radiologic evidence of müllerian duct and renal anomalies. Herein is presented the case of a 25-year-old woman with a rare variant of Herlyn-Werner-Wunderlich syndrome, characterized by an anomalous connection between the 2 endocervical canals, who underwent hemivaginal septum resection to relieve progressively worsening dysmenorrhea. The right hemivaginal septum was resected medially from the left cervix and laterally off the right vaginal wall. Hydrodissection between the hemivaginal septum and right cervix facilitated surgical resection. After resection of the hemivaginal septum there was complete resolution of dysmenorrhea and no recurrence of hematometra or hematocolpos.     

Necrotizing fasciitis caused by Serratia marcescens in two patients receiving corticosteroid therapy.

Necrotizing fasciitis (NF), a devastating soft tissue infection, is rarely attributed to Serratia marcescens. We here report two patients with S. marcescens NF, both of whom had underlying renal disease and had been receiving corticosteroid therapy. The first patient, a 40-year-old man with systemic lupus erythematosus and uremia on prednisolone therapy, developed fulminant cellulitis and septic shock 1 month after a skin biopsy for cutaneous vasculitis of the left foot. The cellulitis evolved to NF, and blood and necrotic tissue cultures both grew S. marcescens. The patient completely recovered after debridement and ceftazidime therapy. The second patient, a 73-year-old man receiving prednisolone therapy for nephrotic syndrome, developed right leg cellulitis that evolved to NF. Blood and necrotic tissue cultures both grew S. marcescens. After aggressive debridement and ciprofloaxcin therapy, the NF improved. However, the patient died of aspiration pneumonia and massive gastrointestinal bleeding 1 month later. These findings illustrate that S. marcescens should be considered as a potential pathogen causing NF in susceptible hosts.     

Renal cell carcinoma with inferior vena cava tumor thrombi

Renal cell carcinoma is a unique neoplasm because of its common propensity to propagate into the renal vein and inferior vena cava (IVC) as tumor thrombus. Historically, the surgical difficulties encountered in removal of these cancers limited the ability of a single institution to obtain experience with large numbers of instances. Between January 1956 and July 1987, 68 patients with renal cell carcinoma extending into the IVC or right atrium underwent radical nephrectomy with vena cava thrombus extraction at the Cleveland Clinic. Twenty-five patients had partial resection of the IVC with reconstruction. Fifteen patients had partial resection and reconstruction of the IVC; however, because of narrowing of the infrarenal IVC, persisting bland thrombus in the proximal IVC or iliac veins or concern regarding postoperative pulmonary emboli, the infrarenal IVC was either ligated or clipped. Seven patients underwent cavectomy with division of the IVC. A right atriotomy was performed upon 14 patients and cardiopulmonary bypass was used in 20 patients, with 17 also having deep hypothermic circulatory arrest. The tumor thrombus was removed intact in 64 per cent of the patients and in multiple small fragments ("piecemeal") in 36 per cent of the patients. The mortality rate was 7 per cent. Survival was examined relative to extent of vena caval thrombus. Patients with extension into the atrium had a significantly worse prognosis than those with other levels of vena caval involvement. Other factors, such as lymph node status, perinephric fat involvement, resection of IVC and intact or "piecemeal" extraction, did not influence the survival rate. Patients with pre-existing metastases preoperatively had an extremely poor survival rate. The techniques now available for surgical resection of all levels of tumor thrombus of the IVC make resection feasible in most patients. In our opinion, the addition of deep hypothermic circulatory arrest has been a significant advance.     

Lower urinary tract resection as part of cytoreductive surgery for ovarian cancer

Twenty-four patients underwent lower urinary tract resection as part of cytoreductive surgery for ovarian epithelial malignancy from 1960 to 1979. Eight patients had partial cystectomies, two of which had reimplantation of the ureters, and 16 had partial ureteral resection. Optimal cytoreduction was associated with a longer mean survival. Patients who presented without ureteral obstruction also survived significantly longer than those with obstruction. Morbidity from surgery involving the urinary tract was not excessive in this series. Partial lower urinary tract resection appears justified as part of a primary cytoreductive effort.     

Renal artery thrombosis following secondary cytoreduction in a patient with ovarian cancer

BACKGROUND: In order to successfully perform aggressive cytoreductive surgery for patients with recurrent epithelial ovarian cancer, resection of retroperitoneal disease in close proximity to major vessels is often required. CASE: We describe a case of a 44-year-old female patient with a history of Stage IV carcinoma of the ovary, who underwent a successful secondary debulking procedure. To remove the left para-aortic tumor implant she required complete mobilization of the left kidney, with skeletonization of the left renal artery and vein. Postoperatively, the patient developed left renal artery thrombosis necessitating a unilateral nephrectomy. CONCLUSION: This is, to our knowledge, the first reported case of renal artery thrombosis following a debulking procedure. Gynecologic oncologists should be aware of this possibility and be familiar with the diagnosis and management of this condition.     

Cyst-like chylous coagulum in the urinary bladder of a patient with recurrent chyluria.

We report a rare case of recurrent chyluria in a 64-year-old man, who had undergone sclerosing therapy for chyluria with complete remission 40 years previously. The chyluria recurred 4 months before the present hospital admission. The patient presented with acute urine retention and weight loss of 6 kg during the past 6 months. Urine biochemistry studies showed elevated concentrations of albumin (0.7 g/dL), globulin (0.6 g/dL), cholesterol (0.59 mmol/L), and triglyceride (0.89 mmol/L), confirming the diagnosis of chyluria. Intravenous urography showed a substantial filling defect in the urinary bladder. Cystoscopy revealed milky urine flowing out of the right ureteral orifice, and a large chylous coagulum presenting as a cyst-like mass in the urinary bladder. The chyluria was successfully treated with sclerosing therapy with injection of 76% Urografin (Shering AG, Berlin, Germany) into the right upper urinary tract, and the chylous coagulum was evacuated endoscopically. No evidence of chyluria recurrence has been noted during a year of follow-up, and the patient has regained 6 kg of body weight. This particular presentation of chylous coagulum in a chyluria patient has not been previously reported in the literature.     

Cervical primitive neuroectodermal tumor

BACKGROUND: Primitive neuroectodermal tumors (PNETs) are rare and potentially aggressive malignancies. CASE: A 24-year-old woman in her eighth week of pregnancy presented with a cervical mass. Tissue biopsy demonstrated poorly differentiated carcinosarcoma with neuroendocrine features. Immunohistochemical studies confirmed the diagnosis of PNET. Treatment included alternating courses of cyclophosphamide, adriamycin, vincristine (CAV) and ifosfamide, etoposide (IE). A radical hysterectomy with bilateral ovarian transposition and periaortic lymphadenectomy was performed with postoperative chemotherapy and radiotherapy. The patient remains disease free 2 years from therapy. CONCLUSION: This is a rare case of cervical PNET occurring in a pregnant patient. A review of the literature indicates that cervical PNET is distinguishable from uterine PNET. This tumor affects younger women and may have a different histogenesis. Pregnancy should not delay diagnosis of this potentially aggressive tumor.     

Intentional ureteral ligation in advanced pelvic malignant disease.

It is now widely accepted that debulking operative resections can provide effective palliation for some patients with advanced pelvic malignancy, particularly with those malignancies originating in the ovary. Some of these radical operations require the resection of long segments of the ureter and portions of the bladder. Although there is a variety of techniques that can be used in this situation to restore the continuity of the urinary tract, in some instances a simple ureteral ligation may represent the best method of managing the "short ureter." In our experience with 23 patients who underwent intentional ureteral ligation, the procedure was found to be safe, expeditious, and beneficial in managing some of the complex problems associated with advanced pelvic malignant disease.     

Primary adenoid cystic carcinoma of sweat glands in vulva: report of an unusual case and review of the literature

Adenoid cystic carcinoma arising from the vulvar sweat glands is a rare malignancy of the female genital tract. We report a case of adenoid cystic carcinoma of sweat glands occurring in the left labia majora of a 52-year-old female patient. The patient underwent radical hemivulvectomy and left inguinal lymph node dissection with negative surgical margins and negative inguinal lymph node metastasis. Then, four episodes of combined chemotherapy without further radiotherapy were given. However, the tumor recurred after 3?months. Currently, the patient has been followed up for 2?years with no distant metastasis. According to our experience, although the tumor has a high tendency of local recurrence after resection, an acceptable survival time of the patient can be achieved with primary surgery.     

Late recurrence of ovarian granulosa cell tumor at the retroperitoneal and renal hilum level in a single-kidney patient--case report

BACKGROUND: Ovarian granulosa cell tumors are rare tumors characterized by a long natural history and a tendency to late recurrence. Surgical resection, radiotherapy, chemotherapy and hormone therapy are possible options to treat recurrent disease. The choice will depend on the patient's condition and the site of recurrence. CASE: We describe the case of a 72-year-old patient with a single left kidney who presented retroperitoneal recurrence of ovarian granulosa cell tumor at the left renal hilum ten years after primary treatment. CONCLUSION: This case illustrates an example of very late recurrence and emphasizes the importance of extended follow-up for these patients.     

"Can laparoscopy really complete full surgical staging?" A case of early recurrence and malignant transformation of borderline ovarian tumor

Ovarian borderline tumor (BOT) with noninvasive implants traditionally is considered to be non-aggressive. Recurrences are delayed and transformations to high-grade carcinoma are rarely documented. We report on a patient with BOT with early recurrence and high-grade carcinoma transformation in a short interval after complete laparoscopic staging. A 27-year-old unmarried woman presented with a 26 cm in size ruptured left ovarian mass. Laparoscopic left salpingo-oophorectomy with right ovarian biopsies, multiple peritoneal biopsies, omental biopsy and washing cytology were performed. FIGO Stage I ovarian serous borderline tumor with microinvasion was confirmed. About ten months later, a 15 cm in size left BOT recurred and was resected by laparoscopic cystectomy including staging surgery. Seven months after the second surgery, we found a pelvic mass by sonogram and elevated CA125. A third diagnostic laparoscopy revealed invasive serous carcinoma with multiple peritoneal implants. In spite of radical surgery and adjuvant chemotherapy, the patient died of a progressive metastatic liver tumor. A case of early recurrence with malignant transformation of BOT is presented together with a brief review.     

Vulvar epithelioid sarcoma in pregnancy

BACKGROUND: Epithelioid sarcoma is a soft tissue tumor rarely found centrally and even less commonly on the vulva. Vulvar sarcoma in pregnancy is also exceedingly rare with only five cases reported to date, none of which have been an epithelioid sarcoma. CASE: We report a case of a 29-year-old woman presenting with a vulvar epithelioid sarcoma at 36 weeks of gestation. The patient underwent a radical resection 6 weeks postpartum followed by chemotherapy. Despite a radical hemivulvectomy and doxorubicin and ifosfamide chemotherapy, she developed pulmonary metastasis and died of tumor-related pulmonary failure secondary to her disease 612 months after diagnosis. To our knowledge this is the first case of a vulvar epithelioid sarcoma presenting during pregnancy. The English literature is reviewed and a total of 18 previous cases of vulvar epithelioid sarcoma have been reported outside of pregnancy. Insight into the biological behavior and therapeutic management of this disease is discussed. CONCLUSION: The optimal management of vulvar epithelioid sarcoma remains to be determined. However, it would seem that early and aggressive surgical resection provides the best possibility for cure. The role of radiation and/or chemotherapy remains to be determined.     

Intestinal surgery and surgery of urinary tract in treatment of recurrent cervical cancer--case report

A case of a 31-year-old patient admitted to the Institute with a diagnosis of recurrent cervical cancer after radical hysterectomy and radiation therapy 12 months before. The patient had intestinal and urinary obstruction and also the tumor compressed the iliac blood vessels superficially. She underwent clinical examination, pelvic and abdominal ultrasound and multislice CT scan. A recurring tumor with a diameter of 7 cm was diagnosed. It was localized in the left parailiac and obturator region and infiltrated the left ureter, left bladder side wall, sigmoid colon and iliac blood vessels superficially. The patient had left pelvic sidewall relapse, so she underwent a palliative surgical procedure. We evacuated the complete tumor together with the infiltrated parts of the left ureter, sigmoid colon and bladder. At the end of operation left ureterocystoneostomy was performed as well as the Hartmann procedure with anus praeter insertion. There were no significant postoperative complications. After the surgical treatment of the recurrence, we suggested that the patient continue treatment of her disease with chemotherapy.     

A case of uterine cervical carcinosarcoma recurrence who obtained a clinically complete response by ifosfamide, doxorubicin and cisplatin

Cervical carcinosarcoma (CS) is a rare gynecologic tumor. The histogenesis, clinical features, and optimal treatment remain unclear. We report a case of cervical CS recurrence to the right lung, which had complete response by treating with ifosfamide, doxorubicin and cisplatin (IAP). A 61-year-old woman underwent semi-radical hysterectomy, bilateral salpingo-oophorectomy, and pelvic lymphadenectomy for CS of the uterine cervix. Eleven months later, the patient presented with left pulmonary metastasis. She refused debulking surgery and had chemotherapy with IAP. After four cycles of chemotherapy, the metastatic tumor completely disappeared. Unfortunately, a re-recurrent tumor was seen in the same lung area six months after IAP. Eventually, she died 39 months after surgery.