Treatment of leiomyomatosis peritonealis disseminata with goserelin acetate: A case report and review of the literature

BACKGROUND Leiomyomatosis peritonealis disseminata(LPD)is a rare condition characterized by multiple pelvic and abdominal nodules,which are composed of smoothmuscle cells.To date,no more than 200 cases have been reported.The diagnosis of LPD is difficult and there are no guidelines on the treatment of LPD.Currently,surgical excision is the mainstay.However,hormone blockade therapy can be an alternative choice.CASE SUMMARY A 33-year-old female patient with abdominal discomfort and palpable abdominal masses was admitted to our hospital.She had undergone four surgeries related to uterine leiomyoma in the past 8 years.Computed tomography revealed multiple nodules scattered within the abdominal wall and peritoneal cavity.Her symptoms and the result of the core-needle biopsy were consistent with LPD.The patient refused surgery and was then treated with tamoxifen,ulipristal acetate(a selective progesterone receptor modulator),and goserelin acetate(a gonadotropin-releasing hormone agonist).Both tamoxifen and ulipristal acetate were not effective in controlling the disease progression.However,the patient achieved an excellent response when goserelin acetate was attempted with relieved syndromes and obvious shrinkage of nodules.The largest nodule showed a 25%decrease in the sum of the longest diameters from pretreatment to posttreatment.Up to now,2 years have elapsed and the patient remains asymptomatic and there is no development of further nodules.CONCLUSION Goserelin acetate is effective for the management of LPD.The long-term use of goserelin acetate is thought to be safe and effective.Hormone blockade therapy can replace repeated surgical excision in recurrent patients.

Intravenous Leiomyomatosis with Right Heart Involvement—A Report of 4 Cases and Literature Review

Intravenous leiomyomatosis（IVL） is a rare benign neoplasm which originates from the smooth muscle cells and is usually confined to the pelvic venous system.Rarely,intracaval and intracardiac extension has been described.Death can occur as a result of intracardiac involvement.We reported 4 cases of IVL with right heart involvement（intracardiac leiomyomatosis,ICL）.Three of them suffered recurrent sudden syncope,and the other one was totally asymptomatic.All of them were successfully treated through one-stage operation under extracorporeal circulation.

Uterine intravenous leiomyomatosis with cardiac extension: Imaging characteristics and literature review

Intravenous leiomyomatosis(IVL),showing unusual growth patterns of uterine leiomyoma,is a rare neoplasm characterized by intravascular proliferation of a histologically benign-looking smooth muscle cell tumor mass,but not invading the tissue.To date,less than 300 cases have been reported and fewer than 100 cases with cardiac involvement.Imaging characteristics of IVL are still not clear so it is usually misdiagnosed before surgery.A 36-year-old woman,who had undergone hysterectomy due to hysteromyoma,presented with shortness of breath after activities.Imaging showed IVL with mass involvement of the left ovarian vein,left renal vein,left external and common iliac vein,as well as within the inferior vena cava(IVC),extending into the right atrium.The operation demonstrated that the mass had no stalk and had welldemarcated borders with the wall of the right atrium and IVC.The patient underwent a one-stage combined multidisciplinary thoraco-abdominal operation under general anesthetic.Subsequently,the pathology report confirmed IVL.IVL should be considered in a female patient presenting with an extensive mass in the right side of the heart.Imaging technology,such as echocardiogram,contrast-enhanced computed tomography and magnetic resonance imaging,can provide important information to reveal the mass,the range and path of the lesion,and relates to the surgical plan decision.Consequently,perfect and exact image examination is very necessary pre-operation.

Intravenous leiomyomatosis with different surgical approaches:Three case reports

BACKGROUND Intravenous leiomyomatosis(IVL) is a rare and complicated disease, which requires surgery by a multidisciplinary team. However, the optimal surgical approach has not been determined.CASE SUMMARY Here we report three cases of IVL treated with different surgical approaches. All patients presented with circulation symptoms. Two patients had lower extremity edema and the other had cardiopalmus. The diagnosis of IVL was confirmed based on the imagining examinations and pathological findings. All patients underwent surgical treatment and were discharged without any complications.CONCLUSION Preoperative examination is crucial for surgical planning and surgical approach is dependent on the patient's condition and tumor involvement.

Diagnosis and surgical treatment of intraveneous leiomyomatosis extending into the heart:two cases report and review of the literature

Objective： To investigate the clinical characteristics, diagnosis and surgical treatment of intravenous leiomyomatosis （IVL）, and outline the differences between Chinese and overseas cases. Methods： Clinical data of two IVL cases, treated in our hospital, were analyzed retrospectively and the related literature was also reviewed. The data of preoperative diagnostic rate, surgical procedures, and postoperative recurrence between patients in China and other countries were compared. Results： The first stage operation was performed successfully in 2 patients. However, they refused subsequent therapies, including a second stage operation to excise the remnants Of the tumor, uterus, bilateral oviducts and ovaries, and anti-estrogen therapy. Both suffered from IVL recurrence, one at 6-month and the other at 9-month, and died at 16-month and 12-month respectively after the first stage surgery. Worldwide reports of 110 IVL cases were reviewed, which included 28 cases in China and 82 cases in other countries. In the majority of the Chinese patients, tumors only extended into the fight atrium rather than the right ventricle （RA 22 cases vs RV 4 cases）. However, among the overseas patients, the rate of extension into the right atrium was similar to that of extension into the right ventricle （RA 41 cases vs RV 38 cases）. The rate of hysterectomies was not significantly different between Chinese and overseas patients （ 67.86% vs 55.9%, P=0.278）. The rate of correct preoperative diagnosis in the Chinese patients was significantly lower than that in the overseas patients （32.14% vs 80%, P=0.000002.）, as well asthe rate of complete excision of the tumor （22.7@0 vs 75.5%, P=0.000001）. The proportion of patients who undergoing a single-stage or a two-stage operation was similar in Chinese and overseas patients. The recurrence rate was significantly higher in the Chinese patients than in the overseas patients （36.8% vs 9.1%, P=0.0055）, and the patients with tumor recurrence were partial tumor excision patients. Conclusion： The possibility of IVL should be considered if echocardiography in female patients demonstrates a tumor in the right heart and a mass in the inferior vena cava （IVC）. Further imaging should be performed to confirm the diagnosis. The correct diagnosis and accurate preoperative delineation of tumor extension are essential for an optimal surgical outcome. The key point in IVL treatment is the complete excision of tumors （single-stage or two-stage surgical procedure）.

Disseminated peritoneal leiomyomatosis with malignant transformation involving right ureter:A case report

BACKGROUND Disseminated peritoneal leiomyomatosis(DPL)with myxoid leiomyosarcoma is a rare variant of leiomysosarcoma,and hematuria as a presenting symptom has never been reported.Through this case report,we emphasize the investigation of the etiology,clinical presentation,diagnosis,treatment,and prognosis of DPL with malignant changes mimicking metastatic urinary tract cancer and to help develop further clinical management.CASE SUMMARY We describe a case of DPL with malignant transformation involving the right ureter after laparoscopic hysterectomy.An exploratory laparotomy was performed and all visible nodules were surgically removed.DPL with focal malignant transformation to myxoid leiomyosarcoma was confirmed based on pathology results.CONCLUSION Professionals who preoperatively diagnose DPL with malignant change to myxoid leiomyosarcoma involving the genitourinary tract should consider symptoms of abdominal pain,hematuria,and imaging of disseminated pelvic tumors in women,especially those with prior history of laparoscopic hysterectomy.Early complete removal of all tumors is the cornerstone to prevent DPL from malignant changes.

Diffuse uterine leiomyomatosis: A case report and review of literature

BACKGROUND Diffuse uterine leiomyomatosis(DUL)is a benign uterine smooth muscle neoplasm with unknown etiology.Since DUL is rarely reported,knowledge regarding it is limited.The rate of early diagnosis is low,and DUL is often misdiagnosed as common multiple uterine leiomyomas before surgery.CASE SUMMARY A 27-year-old patient with no sexual activity presented to the Emergency Department of our hospital complaining of heavy vaginal bleeding.She had a history of uterine fibroids and menorrhagia.Pelvic examination showed a regularly enlarged uterus,similar in size to that associated with a 4-mo pregnancy.Pelvic magnetic resonance imaging(MRI)revealed numerous multiple uterine fibroids,and a transabdominal myomectomy(TM)was performed.Intraoperative exploration revealed that the myometrium was full of myoma nodules of variable sizes.Over 50 leiomyomas were removed.The pathology report confirmed leiomyoma.The patient was discharged and received a gonadotropin-releasing hormone analog(3.75 mg)for 6 mo.Ten months after surgery,the patient presented to the hospital again for abnormal uterine bleeding.MRI showed an irregular mass with a diameter of 5.2 cm without sharp demarcation in the uterine cavity.Submucosal leiomyoma was considered first,and the patient underwent a hysteroscopic myomectomy plus hymen repair.Intraoperative exploration showed that there were several leiomyomatosis masses in the cavity.Postoperative pathological exam-ination confirmed submucosal leiomyoma and necrotic and generative tissue.Although the menstrual cycle was still irregular,the patient did not have symptoms of menorrhagia for a period of 28 mo after the second surgery.CONCLUSION Individuals with DUL are easily misdiagnosed due to the lack of specific manifestations of this disease.MRI is helpful for early identification and preoperative evaluation.There is currently no unified method of diagnosis.For women who want to preserve fertility,conservative surgery should be made an option.When TM is chosen,a modified new myomectomy should be considered to avoid the drawbacks of traditional TM.

Evolution of a Disseminated Peritoneal Leiomyomatosis: About a Case

Disseminated peritoneal leiomyomatosis (DPL) is a benign tumor of smooth muscle tissue. It is rare and is characterized by the development of multiple peritoneal nodules simulating peritoneal carcinosis. Less than 200 cases have been reported in the literature so far. We are reporting a case of DPL detected during a Caesarean section 6 years ago in a 41-year-old female patient, G5P2. The patient underwent an elective iterative caesarean section at 38 weeks of amenorrhea for a fetus in breech presentation. During laparotomy, there was a marked regression of the peritoneal nodules varying in size from 0.1 to 0.5 cm. Histological examination showed a proliferation of smooth muscle fibers without mitosis, atypia or necrosis. DPL is a benign, confusing condition that simulates peritoneal carcinomatosis, which must be recognized as such in order to avoid a dilapidated and unnecessary surgical procedure.

Diagnosis of intravenous leiomyomatosis extending to heart with emphasis on magnetic resonance imaging

Background Intravenous leiomyomatosis is a rare neoplasm, and its cardiac extension is often overlooked or misdiagnosed. The purpose of this study was to explore the imaging features of intravenous leiomyomatosis with cardiac extension, especially the value of magnetic resonance imaging in differential diagnosis. Methods Between July 2005 and August 2008, 4 cases of intravenous leiomyomatosis with cardiac extension were resected in Cangzhou Central Hospital. Three cases had echocardiography performed, two had post contrast scans of CT performed, and two had MRI performed. Between July 2005 and May 2010, 25 cases of histopathologically proven other kinds of tumors involving the inferior vena cava and right atrium were compared for discussion of differential diagnosis. Results Intravenous leiomyomatosis with cardiac extension demonstrated a hyperechoic elongated mobile mass extending from the inferior vena cava to the right atrium with or without evidence of protruding into the right ventricle on echocardiography. The lesion was enhanced heterogeneously on post contrast scans of CT and was of relatively lower density compared to the enhanced blood in the inferior vena cava and right atrium, with common iliac vein and the ipsilateral internal iliac and ovarian veins involved in some cases. The untreated uterus myoma demonstrated enlargement of the uterus with heterogeneous contrast enhancement. On MRI, the lesion looked like a luffa vegetable sponge on FIESTA coronal images and a sieve pore on T2-weighted axial images. All four tumors were removed successfully, and follow up of one to four years revealed no recurrence. The 25 cases of histopathologically proven other kinds of tumors involving inferior vena cava and right atrium had their own imaging features different from those seen on intravenous leiomyomatosis with cardiac extension. With reference to their medical history, differential diagnosis can often be made. Conclusion The imaging appearance of intravenous leiomyomatosis has some unique features, and the luffa vegetable sponge and sieve pore like appearance on MRI may be helpful for differential diagnosis.

An unusual case of intravenous leiomyomatosis involving the right atrium

To the Editor:A 48-year-old Chinese woman complained the chest distress,shortness of breath and amaurosis fugax for 3 months.The above clinical symptoms worsened gradually 1 week ago.The diastolic rumbling murmur was heard on the third and fourth intercostal space of the left sternum during a physical examination,when the patient was sitting down.The patient's vital signs were stable.A laboratory test revealed normal.

Leiomyomatosis peritonealis disseminata associated with endocervicosis

Leiomyomatosis peritonealis disseminata （LPD） is a rare but well-documented disorder mainly found in young women. Occasionally, LPD coexists with endometriosis and/or multi-cystic mesothelioma. Endocervicosis is another uncommon condition, which was recognized as a distinct entity by Clement and Young in 1992 and has been documented involving the lymph node, pelvic peritoneum, bladder and small intestine. We described a case with a unique combination of LPD and endocervicosis.

Renal cell carcinoma: Evolving and emerging subtypes

Our knowledge of renal cell carcinoma(RCC) is rapidly expanding. For those who diagnose and treat RCC, it is important to understand the new developments. In recent years, many new renal tumors have been described and defined, and our understanding of the biology and clinical correlates of these tumors is changing. Evolving concepts in Xp11 translocation carcinoma, mucinous tubular and spindle cell carcinoma, multilocular cystic clear cell RCC, and carcinoma associated with neuroblastoma are addressed within this review. Tubulocystic carcinoma, thyroid-like follicular carcinoma of kidney, acquired cystic disease-associated RCC, and clear cell papillary RCC are also described. Finally, candidate entities, including RCC with t(6;11) translocation, hybrid oncocytoma/chromophobe RCC, hereditary leiomyomatosis and RCC syndrome, and renal angiomyoadenomatous tumor are reviewed. Knowledge of these new entities is important for diagnosis, treatment and subsequent prognosis. This review provides a targeted summary of new developments in RCC.

Uterine Fibromyoma and Intravascular Thrombosis—Eight Cases

Objectives: We present eight cases of vascular thrombosis of the pelvic vessels and deep venous thrombosis (DVT) of the lower limb, secondary to compression of the pelvic vessels by the uterine fibroid, managed over a span of twelve years from 2001 to 2013. We discuss the mean size of the leiomyoma, the prediliction for DVT of the left lower limb, the role of oral contraceptives when used in women with large fibroids, to increase the risk of DVT and present a brief literature review.?Results:?Age,?the mean age of our patients was—41.12 yrs, range—18 yrs to 50 yrs.?Parity and Menopausal Status: Seven were parous women. One was unmarried, nulligravid.?Seven were premenopausal and one was postmenopausal.?Vascular Thrombosis:?In one it was arterial thrombosis and in seven it was venous thrombosis.?DVT was on the left side in -6/8?-?75%. Clinical Complaints:?The presenting complaints were heavy menstrual bleeding in three,?severe dysmenorrhoea and heavy bleeding in one,?mass per abdomen in one, heavy menses and abdominal mass in one, no complaints apart from DVT in one.?Uterine Enlargement:?The size of the uterus was between 12 to 26 weeks. The mean size was 20 weeks.?It was a single fibromyoma in six and multiple, two in two.?Use of Oral Contraceptives:?OC pills were used to control heavy menstrual bleeding in three cases—3/8, 37.5%.?Discussion:?The DVT was on the left side in six of our cases,?75% were on the left side in our series. Menstrual problems like heavy bleeding can be secondary to fibromyoma. Three of the eight—37.5%,?women used OC pills to control menorrhagia. One developed DVT after one cycle of use of OC pills;?the second developed arterial thrombosis of the pelvic vessels after four cycles of OC pill use;?the third used OC pills for a longer period, on and off. The woman who developed arterial thrombosis was using OC pills with 50 mcgs of oestrogen and 0.5 mg norgestrel. Conclusions: Uterine leiomyoma can cause vascular thrombosis secondary to compression of the pelvic vessels. The mean size of the uterine enlargement by the fibromyoma was 20 weeks and in 6/8, it was a solitary fibromyoma. DVT was on the left side in 75% of our cases. Use of OC pills in women with an enlarged uterus with leiomyoma can increase the risk of DVT.