Surgical resection of pulmonary and extrapulmonary recurrences of uterine leiomyosarcoma

OBJECTIVE: The objective was to determine long-term survival and predictors of outcome in a retrospective cohort of patients who underwent surgical resection of recurrent uterine leiomyosarcoma (LMS). METHODS: Between January 1991 and March 2001, 41 patients who underwent surgical resection for recurrent uterine leiomyosarcoma were identified. The records of these patients were reviewed and abstracted data included patient age, date of initial diagnosis, tumor histology and grade, residual tumor after all operations, the use of adjuvant therapy, dates and sites of all recurrences, and disease status at last follow-up. Survival was determined from the time of first recurrence to last follow-up. Survival curves were estimated using the Kaplan-Meier method and P values were generated using the likelihood ratio test from the Cox proportional hazards model and chi(2) analysis. RESULTS: Forty-one patients with recurrent uterine LMS (17 local pelvic, 18 distant, 6 both) underwent surgical resection at time of first recurrence. A thoracic procedure alone was performed in 13 cases. Information on residual disease was available for 37 patients. The disease-specific 2-year survival for all 41 patients was 71.2% (95% CI: 58.1, 87.3). In univariate analysis, time to first recurrence and optimal resection were significantly associated with longer overall survival. CONCLUSION: Optimal surgical resection for recurrent uterine leiomyosarcoma may provide an opportunity for long-term survival in a select patient population. Time to first recurrence and optimal surgical resection were predictors of improved outcome in this study.     

Uterine leiomyosarcoma metastatic to the sphenoid sinus: a case report and review of the literature

BACKGROUND: Leiomyosarcoma (LMS) of the uterus is a rare neoplasm with an aggressive growth pattern. Although the majority of uterine LMS is diagnosed with disease confined to the uterus, the rate of recurrent disease is high. The most common sites of recurrent disease are lung, liver, and peritoneal cavity. The rate of lymph node involvement with disease confined to the uterus is less than 3%. Metastases to brain and skull are rare. CASE: A 39-year-old woman underwent a total abdominal hysterectomy, bilateral salpingo-oophorectomy, pelvic and paraaortic lymph node biopsies, omentectomy, and appendectomy for Grade 2 FIGO Stage 1 uterine leiomyosarcoma. She remained disease-free for 2 years until she presented with metastases to the sphenoid sinus. She underwent incomplete resection of the recurrence and was treated postoperatively with adjuvant MAID chemotherapy with poor response. She then underwent a second resection at an outside institution. Five months later, she was treated with radiation therapy to the base of the skull with no response. She expired from the disease 13 months after the diagnosis of the recurrence. CONCLUSION: The prognosis for patients who recur is dismal. The treatment options are limited. Surgical management should be considered as uterine LMS has a low response to chemotherapy and surgical resection of LMS to sites such as lung and abdomen has been suggested to offer a benefit. Radiation therapy may provide palliative benefit in the setting of metastatic disease.     

Conservative management of pneumatosis intestinalis

BACKGROUND: Pneumatosis intestinalis is a rare condition characterized by subserosal and submucosal gas-filled cysts in the gastrointestinal tract; it may be associated with bowel ischemia, perforation, and a high mortality rate. As a result, many authorities advocate an aggressive surgical approach in patients with pneumatosis intestinalis. CASE: A 53-year-old female with recurrent, metastatic uterine leiomyosarcoma underwent resection of the pelvic recurrence, low anterior rectal resection with primary anastomosis, and partial hepatectomy for liver metastasis. Her postoperative course was notable for a small bowel obstruction and the finding of pneumatosis intestinalis on radiologic studies. The patient developed mild abdominal pain. She did not develop tenderness or fevers. She was managed with bowel rest, nasogastric tube decompression, total parenteral nutrition, and broad-spectrum antibiotics. The finding of pneumatosis intestinalis resolved over the ensuing 6 days. Her diet was slowly advanced, and she was discharged home in stable condition without further surgical intervention or recurrence of the obstruction or pneumatosis. Currently, her only evidence of disease is pulmonary metastases. CONCLUSIONS: In select patients, the outcome of a conservative approach to the management of pneumatosis intestinalis is not much different than surgical re-exploration for highly selected patients. The clinical condition of the patient, not solely the finding of pneumatosis intestinalis, should drive management in these cases.     

Primary vaginal leiomyosarcoma in pregnancy

BACKGROUND: Primary vaginal sarcoma constitutes about 2% of all malignant vaginal lesions, with leiomyosarcoma being the most common in adult women. To our knowledge, this is the first case of vaginal leiomyosarcoma occurring during pregnancy. CASE: A 21-year-old woman, at 39 weeks of gestation, presented with a pedunculated vaginal mass. Local excision of the mass was performed immediately. Histopathologic evaluation revealed a grade 2 leiomyosarcoma. The patient delivered a healthy infant 2 weeks after resection. She has had no evidence of recurrence in 28 months of follow-up. CONCLUSION: Previous cases of vaginal leiomyosarcoma and sarcomas of the female genital tract occurring during pregnancy are reviewed. The histopathologic criteria for vaginal smooth muscle tumors are well established. Primary management of vaginal leiomyosarcoma is surgical.     

Leiomyosarcoma of the vagina

Vaginal leiomyoma is uncommon and borderline leiomyoma of the vagina is an extreme rarity. Leiomyoma of the vagina may undergo malignant change to become borderline malignancy or leiomyosarcoma. A 38-year-old woman, complaining of foul vaginal discharge and dyspareunia with 2 months duration, underwent total excision of tumor. The removed tumor was diagnosed as borderline case of leiomyoma histologically. Unfortunately, she had tumor recurrence within 4 months. Thus, it was more likely that the primary diagnosis was incorrect and that the diagnosis leiomyosarcom has been missed. She underwent total abdominal hysterectomy, bilateral salphingo-oophorectomy and total vaginectomy. The final diagnosis was leiomyosarcoma. Three months later tumor recurred again and re-excision followed by chemotherapy was performed. However, she died of her disease on her 5th month following three courses of chemotherapy.     

Primary malignant vaginal melanoma treated with adriamycin and ifosfamide: a case report and literature review

BACKGROUND: Vaginal melanoma is a very rare but highly malignant gynecological disease, usually diagnosed in postmenopausal woman. The prognosis tends to be poor and it is associated with high rate of recurrence and short survival rates. CASE: The following paper describes a case report regarding a 72-year-old woman with a locally advanced malignant melanoma. The previous erroneous histopathological diagnosis was leiomyosarcoma. She underwent chemotherapy with 3 courses of doxorubicin and ifosfamide. The diagnosis of malignant melanoma was obtained after a repeated biopsy and further pathological investigations. She later underwent radical surgery and 2 additional cycles of the same chemotherapy. At present, 7 months after the last cycle, the patient was locally disease-free, but developed brain metastases, requiring chemotherapy treatment. CONCLUSION: In view of poor survival, this chemotherapy regimen may be an interesting alternative to the traditional treatment of vaginal melanoma.     

Primary leiomyosarcoma of the vagina. A case report and literature review

Abstract. Ciaravino G, Kapp DS, Vela M, Fulton RS, Lum BL, Teng NNH, Roberts JA. Primary leiomyosarcoma of the vagina. A case report and literature review.
Primary vaginal leiomyosarcoma is a rare tumor. We report a unique case of a 27-year-old woman with stage I, high-grade primary leiomyosarcoma of the vagina treated with surgical resection and adjuvant radiation therapy. She returned within 6 months with an abdominal-pelvic recurrence and lung metastases. The patient died of disease 9 months after diagnosis. A comprehensive review of primary vaginal leiomyosarcoma was performed and factors affecting survival were analyzed.
A Medline search of the English-language literature revealed 66 previously reported cases. Forty-eight of these had follow-up data. Survival probabilities were calculated using the Kaplan-Meier method, and the effects of age, stage, grade, tumor location, and treatment modality were analyzed. Stage III and IV data were combined.
The overall 5-year survival rate was 43%. Patients more than 50 years of age had a 5-year survival rate of 26% compared with 51% for those less than 40 years. Five-year survival for stage I and II tumors was 55% and 44%, respectively. Patients with stage III/IV disease had 25% survival at 18 months. No patient treated primarily with chemotherapy or radiation therapy survived beyond 36 months. In contrast, patients treated primarily with surgery had a 5-year survival rate of 57%. Only stage remained an independent predictor of survival on Cox regression analysis.
We continue to recommend surgical resection as primary treatment. Exenteration may be an option for select patients, but ultimately management should continue on a case-by-case basis.     

High dose rate intraoperative radiotherapy for recurrent cervical cancer and nodal disease

BACKGROUND: Patients with pelvic sidewall recurrences of cervical cancer have a dismal prognosis. Intraoperative radiation therapy (IORT) has demonstrated encouraging results. Patients have traditionally been excluded from IORT if they had distant metastases. CASE: A patient underwent radical tumor resection and high dose rate (HDR) IORT for a pelvic sidewall recurrence of cervical cancer. She also had metastatic disease in a para-aortic node. The patient has been followed for >20 months with no evidence of disease recurrence. CONCLUSIONS: HDR-IORT may be offered to select patients with recurrent cervical cancer and isolated metastatic para-aortic lymph nodes.     

Endometrial carcinoma with laparotomy wound recurrence: complete remission following surgery and chemotherapy consisting of paclitaxel and carboplatin

We present a patient with surgical stage I endometrial cancer who experienced laparotomy wound recurrence 4 years after primary treatment. She was treated successfully by complete surgical resection of recurrent tumors and chemotherapy. A 62-year-old white female with laparotomy wound recurrence of endometrial carcinoma with small-bowel involvement and concomitant subcutaneous metastasis in the abdominal wall underwent complete surgical resection of metastatic tumors followed by six cycles of chemotherapy consisting of paclitaxel (175 mg/m2) and carboplatin (area under the curve 5). Since 24 months after resection of recurrence, she has no evidence of disease recurrence. Endometrial carcinoma with laparotomy wound recurrences, especially those with concomitant metastases, can be successfully treated by complete surgical resection followed by chemotherapy consisting of paclitaxel and carboplatin.     

Fetus-in-Fetu: A Case of Ovarian Involvement and Residual Regrowth in a Teenager

Background: Fetus-in-fetu (FIF) is a rare, congenital soft tissue mass typically occurring retroperitoneally in neonates younger than 18 months. We present a 13-year-old girl with an ovarian FIF occurrence and subsequent residual regrowth after resection.Case: A 13-year-old girl presented with abdominal pain and was found to have a 19-cm, complex, right adnexal mass. Preoperative tumor markers were normal and risk assessment favored a benign process. She underwent open ovarian cystectomy with pathology showing FIF. Nine months later, she had a recurrence of her ovarian mass and underwent right oophorectomy, with FIF on pathology.Summary and Conclusion: In patients in whom FIF is discovered within the ovary, consider postoperative surveillance, because of the risk of recurrence or residual disease.     

Myxoid leiomyosarcoma of the uterus with subsequent pregnancy and delivery

BACKGROUND: Myxoid leiomyosarcoma of the uterus is an extremely rare neoplasm. In young women with this disease, fertility should be considered in the treatment protocol. CASE: A 20-year-old woman presented with a huge pelvic mass originating from the uterine wall and showing a histopathology of myxoid leiomyosarcoma. Five and one-half years after laparotomy, the recurrent tumor was found and removed. Two years after the second operation, she became pregnant, and delivered a healthy baby by cesarean section. Multiple recurrent masses were evident and removed at cesarean section. She is alive and well without evidence of recurrence 1 year after delivery. CONCLUSION: The best treatment for this tumor is not established. In young women with a desire to bear children, the management is more controversial.     

Leiomyosarcoma of the broad ligament: a case report and literature review

Primary leiomyosarcoma of the broad ligament is a very rare, rapidly progressive and highly malignant gynaecological tumor. There are only 11 cases reported so far in the literature. An 87-year-old Caucasian lady with leiomyosarcoma of the broad ligament is presented. Treatment consisted of resection and total abdominal hysterectomy with bilateral salpingo-oopphorectomy. Unusually, she developed large recurrent pelvic disease in the absence of disseminated metastasis following surgery. She died within 8 weeks of primary surgery. This highlights the unknown behavior of this tumor and the need for more research into management.     

Pulmonary artery leiomyosarcoma.

Pulmonary artery leiomyosarcoma is a rare but highly lethal disease, and can be mistaken for pulmonary thromboembolism. We report a case of pulmonary artery leiomyosarcoma managed with surgical resection, chemotherapy, and radiotherapy. A 57-year-old woman was admitted with complaints of aggravated dyspnea. She was initially treated with oxygen therapy and heparinization for a suspected pulmonary embolism. Echocardiography revealed a dilated right atrium and ventricle and severe tricuspid regurgitation, with an estimated systolic right ventricular pressure of 95 mm Hg; a shadow of a mass in the main pulmonary artery was also noted. Right ventriculography revealed a filling defect, and to-and-fro motion of the mass in the main pulmonary artery. The left pulmonary artery was almost totally occluded by the mass. The patient's condition improved dramatically after palliative excision of the mass and patch reconstruction of the outflow tract of the right ventricle with a bicuspid xenograft. Pathologic examination of the mass revealed leiomyosarcoma. Chemotherapy and radiotherapy were subsequently administered and follow-up imaging studies 3 months postoperatively revealed no recurrence of the tumor. The patient remains well, more than 1 year after treatment. This report emphasises that pulmonary artery sarcoma should be considered in the differential diagnosis in cases of suspected pulmonary thromboembolism.     

Radiotherapy for the Treatment of Metastatic Granulosa Cell Tumor in the Mediastinum: A Case Report

OBJECTIVE: We report a case of metastatic ovarian granulosa cell tumor in the mediastinum with a 2-year disease-free interval after treatment with radiotherapy and review the literature regarding the use of radiotherapy in recurrent and metastatic granulosa cell tumor. METHODS: The patient's medical records, histological slides, and radiological films were reviewed. The pertinent references were obtained using a Medline search and cross-references. RESULTS: A patient with Stage 1A granulosa cell tumor developed a recurrence in the retroperitoneum 10 years after initial surgery. She was treated with chemotherapy followed by surgical resection. She subsequently developed metastatic tumor in the mediastinum which responded completely to radiotherapy. She has remained disease free for 2 years since the completion of radiotherapy. CONCLUSION: Radiotherapy is a treatment option that should be considered in localized recurrent or metastatic granulosa cell tumor that is not amenable to surgery as it can potentially control the disease for several years.     

Leiomyosarcoma of the cervix

BACKGROUND: Epithelioid leiomyosarcoma arising from the uterine cervix is extremely rare, with only three cases reported in the world literature. We present the case report of a 14-cm epithelioid leiomyosarcoma arising from the uterine cervix. CASE: A 47-year-old female presented with a 1-year history of worsening menorrhagia and was found to have a large pelvic mass extending into and filling the upper vagina. She underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy, revealing a 14 x 10 x 9 cm cervical epithelioid leiomyosarcoma. CONCLUSION: Although exceedingly rare, leiomyosarcoma is able to arise primarily from the uterine cervix. Given its extreme rarity, management of cervical LMS must be extrapolated from the currently accepted management for uterine LMS.     

Uterine Adenosarcoma with Sarcomatous Overgrowth: A Case Report of Aggressive Disease in a 16-Year-Old Girl and a Literature Review

Background

Uterine adenosarcoma with sarcomatous overgrowth (ASSO) is a rare and aggressive disease. A case of a 16-year-old girl with uterine ASSO is reported herein. The patient received surgical resection and chemotherapy and remained alive without disease 11 months after the surgery.

Thymoma in pregnancy

The natural history of slow, localized progression usually seen with thymomas seems to be transformed by pregnancy into one of rapid growth and distant metastatic spread. Five of the six previously reported cases had died of the malignancy within 6 months postpartum. The one previous long-term survivor died of treatment-induced complications. Two important questions remain unresolved: What is the risk of recurrence in the patient who has no residual disease at the time of conception? Approximately 50 per cent of female patients can anticipate having complete resection of their thymoma, and their recurrence risk is about 2 per cent. Potentially, there are many patients who have conceived and delivered after surgical therapy. Documentation of these case histories is needed for an accurate prediction of the true risk. Two of the cases within the present review fall within this category. The clinical course in these two patients is consistent with the natural history of the disease. One exhibited local recurrence and progression. The other patient died from sepsis, probably related to immunosuppression. Whether there is a cause and effect relationship between pregnancy and their recurrences is unknown. What is the risk to the patient who is first diagnosed while pregnant? The only survivor in this category was presented by Goldman. This patient underwent immediate therapeutic abortion following diagnosis. She had radiation therapy for invasive unresectable disease and subsequently delivered two term pregnancies, but died of postcesarean cardiac arrest. She had no evidence of recurrence.(ABSTRACT TRUNCATED AT 250 WORDS)     

Hormonal therapy for the management of grade 1 endometrial adenocarcinoma: a literature review

OBJECTIVE: We reviewed reported cases of grade 1 endometrial adenocarcinoma that were conservatively managed with hormonal therapy in an effort to identify the most effective treatment regimen. METHODS: We searched MEDLINE and other databases for English-language articles describing patients with grade 1 endometrial adenocarcinoma who were treated with hormonal therapy. The search included articles published between January 1966 and December 2003. The following key words were used: endometrial cancer, uterine cancer, adenocarcinoma, hormones, progesterone, medroxyprogesterone acetate, megestrol acetate, conservative therapy, fertility, and female. A total of 79 articles were found. Studies were excluded for the following reasons: advanced stage, metastatic or recurrent disease, progestin use after radiation, chemotherapy, or surgery, concurrent with radiation therapy or chemotherapy, administration of progestin other than orally or intramuscularly, tumor confined to a polyp, grade 2 or 3 disease, undocumented grade, nonendometrioid histology, progestin use in conjunction with ovarian wedge resection or other hormones, and hyperplasia. Our study ultimately included 81 patients in 27 articles. RESULTS: Sixty-two patients (76%) responded to treatment. The median time to response was 12 weeks (range, 4-60 weeks). Fifteen patients (24%) who initially responded to treatment recurred. The median time to recurrence was 19 months (range, 6-44 months). Ten (67%) of the patients with recurrence ultimately underwent total abdominal hysterectomy. Residual endometrial carcinoma was found in six patients (60%). Nineteen patients never responded. Twenty patients were able to become pregnant at least once after completing treatment. The median follow-up was 36 weeks (range, 0 weeks-30 years). No patients died of their disease. CONCLUSION: The majority of patients reported with well-differentiated endometrial adenocarcinoma who undergo conservative treatment with a progestational agent respond to treatment. When an initial response is not achieved or when disease recurs, carcinoma extending beyond the uterus is rare.     

Phase II Trial of Paclitaxel in Leiomyosarcoma of the Uterus: A Gynecologic Oncology Group Study

OBJECTIVE: The aim of this study was to determine the activity of paclitaxel in chemotherapy-naive patients with advanced or recurrent uterine leiomyosarcoma. METHODS: Patients received 175 mg/m(2) of paclitaxel iv over 3 h. Courses were repeated every 3 weeks until disease progression or adverse side effects supervened. RESULTS: Thirty-four women were entered, but 1 patient was ineligible because of wrong cell type. Median age was 55 years (range: 35-84 years). GOG performance status was 2 in 2 instances, 1 in 9 cases, and 0 in 22 others. Eight patients (23.4%) had received radiotherapy. A median of 2.5 courses was given (range: 1-18). Eleven patients (33.3%) experienced grade 3 or 4 neutropenia, 1 (2.9%) had grade 4 thrombocytopenia, and 1 had grade 3 anemia. There were no cases of grade 3 or 4 gastrointestinal or dermatologic toxicity. One patient each developed deep venous thrombosis and a grade 3 allergic reaction. Eight patients (24.2%) had stable disease for at least 2 courses of therapy. Three complete responses were reported (9.1%). CONCLUSION: With the dose and schedule tested, paclitaxel has limited activity in patients with uterine leiomyosarcoma. Modest toxicity suggests that a higher dose of paclitaxel might be evaluated.     

Trimetrexate in the treatment of recurrent or advanced leiomyosarcoma of the uterus: a phase II study of the Gynecologic Oncology Group.

OBJECTIVE: This study was conducted to determine the objective response of trimetrexate in patients with advanced or recurrent leiomyosarcoma of the uterus. METHODS: Eligibility was restricted to patients with measurable disease who had received no more than one prior chemotherapy regimen, who had adequate bone marrow, renal, and hepatic function, and who had recovered from previous therapy. Trimetrexate was begun at 5 mg/m2/day orally for 5 days every other week, with dose modifications specified by study design. RESULTS: Of 28 patients entered into the study, 27 were evaluable for toxicity and 23 for response. Prior therapy included radiation (7 patients) and/or chemotherapy (10 patients). Measurable disease was extrapelvic in 20 cases and confined to the pelvis in 3. The overall response rate was 4.3%; there were no complete responses and 1 partial response. Toxicities were mild to moderate with no treatment-related deaths. Hematological toxicity was most common, consisting of leukopenia (grade 1 to 2, 8 patients; grade 3 or 4, 2 patients), thrombocytopenia (grade 1 to 2, 10 patients; grade 3 or 4, 1 patient), and anemia (grade 1 to 2, 6 patients; grade 3 or 4, 4 patients. Severe (grade 3 or 4) nonhematologic toxicity was uncommon: nausea/vomiting/gastrointestinal (3 patients) and neurological (1 patient). Progression-free and overall survival, in months, was 2.2 (range: 0.9-13.4) and 7.2+ (range: 1.0-13.4+), respectively. CONCLUSION: Although toxicity is acceptable, trimetrexate at this dose and schedule is ineffective therapy for patients with recurrent leiomyosarcoma. Further development of this specific regimen for this indication is unwarranted.