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Hereditary leiomyomatosis and renal cell carcinoma (HLRCC) is an autosomal dominant syndrome wherein affected individuals are at risk for the development of cutaneous leiomyomas, early-onset multiple uterine leiomyomas, and an aggressive subtype of renal cell cancer. HLRCC is caused by germline mutations in the fumarate hydratase (FH) gene, which inactivates the enzyme and alters the function of the tricarboxylic acid/Krebs cycle. This article reviews the hitherto described morphologic features of HLRCC-associated renal cell carcinoma (RCC) and outlines the differential diagnosis and ancillary use of immunohistochemistry and molecular diagnostics for these tumors. The morphologic spectrum of HLRCC-associated RCC is wide and histologic features, including tumor cells with prominent nucleoli, perinucleolar halos, and multiple architectural patterns within the same tumor, which are suggestive of this diagnosis. FH immunohistochemistry in conjunction with genetic counseling and germline FH testing are the important parameters for detection of this entity. These kidney tumors warrant prompt treatment as even smaller sized lesions can demonstrate aggressive behavior and systemic oncologic treatment in metastatic disease should, if possible, be part of a clinical trial. Screening procedures in HLRCC families should preferably be evaluated in large cohorts., Competing Interests: None

Background:  Diffuse esophageal leiomyomatosis (DEL) is a rare disorder characterized by benign hypertrophy of esophageal smooth muscle cells. No rigorous summary of available evidence on how to best manage these patients exists., Objective:  To define the clinical features and outcomes of pediatric patients with DEL., Materials and Methods:  A systematic literature search of the PubMed and Cochrane databases was performed with respect to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement (end-of-search date: October 6, 2018). The algorithm: "esophageal leiomyomatosis AND (children OR pediatric*)" was implemented., Results:  Thirty-five studies including a total of 58 patients were analyzed. The female:male ratio was 1.45:1. Mean patient age was 8.54 ± 4.67 years. The most common disease manifestations were dysphagia and gastrointestinal symptoms (90.0%, 95% confidence interval [CI]: 78.2-96.1), followed by failure to thrive (57.9%, 95% CI: 36.2-76.9) and pulmonary symptoms (56.4%, 95% CI: 41.0-70.7). Alport syndrome (AS) was seen in 57.7% (95% CI: 44.2-70.1) of the patients. The most commonly implemented procedure was esophagectomy (85.2%; n  = 46/54; 95% CI: 73.1-92.6) with gastric transposition (37.8%; n  = 17/45; 95% CI: 25.1-52.4). Postoperative complications developed in 33.3% ( n  = 15/45; 95% CI: 21.3-48) of the patients. All-cause mortality was 7.0% (95% CI: 2.3-17.2) and disease-specific mortality was 3.5% (95% CI: 0.3-12.6)., Conclusion:  DEL is an uncommon condition that typically occurs in the setting of AS. Esophagectomy with gastric transposition is the mainstay of treatment. Although complications develop in one-third of the patients, mortality rates are low., Competing Interests: None declared., (Georg Thieme Verlag KG Stuttgart · New York.)

Rationale: Intravascular leiomyomatosis (IVL) is a rare nonmalignant tumor that can be fatal if untreated., Patient Concerns: A 49-year-old nulliparous Asian woman who underwent hysterectomy and left salpingo-oophorectomy for multiple uterine leiomyomas 18 months prior presented complaining of intermittent palpitation and chest tightness for approximately 1 month. Echocardiography revealed a large mobile tumor mass extending from the inferior vena cava (IVC) to the right atrium that partially obstructed IVC flow and tricuspid inflow. Thoracicabdominopelvic computed tomography revealed a left adnexal tumor (4.8 × 2.5 cm) causing intravascular obstruction extending from the left internal iliac vein to the IVC, right atrium, and right ventricle., Diagnosis: IVL with right heart involvement INTERVENTIONS:: Under cardiopulmonary bypass, a one-stage surgery combining sternotomy and laparotomy was performed. The tumor was approached and extracted via sternotomy, and tumor detachment and removal of residual tumors was accomplished via laparotomy., Outcomes: A firm, smooth, and regularly shape tumor 15.5 × 5.5 × 2.5 in size was completely removed and histopathologically confirmed as IVL. The patient tolerated the surgical procedure well and no postoperative complication was noted., Lessons: We describe a one-stage surgical approach to completely remove an IVL extending to the right ventricle.

Objectives: To determine the proportion and the characteristics of patients who did or did not respond after 3 months of ulipristal acetate (UPA) therapy., Study Design: In this retrospective cohort study conducted in the University Hospital of Bordeaux (France) and University Medical Center Ljubljana (Slovenia), symptomatic non-menopausal patients with fibroids that qualified for surgery were pretreated by 3 months of oral UPA 5 mg/day. Clinical success was defined by normalization of the bleeding score, and/or regression of pelvic pain, and/or abdominal distension. Imaging success was defined by reduction in fibroid volume ≥ 25%., Results: The clinical and imaging success rates were 54/66 (82%) and 39/66 (59%) respectively. The absence of previous pregnancy (p = 0.004) and the size of the dominant fibroid ≥ 80 mm (p = 0.004) were independent factors associated with clinical failure. Age <35 years (p = 0.02) was the only independent factor associated with imaging failure., Conclusion: Young women developing fibroids and/or women with large fibroids may be resistant to ulipristal acetate therapy., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Objectives: The aim of this study was to evaluate the pharmacoeconomic profile in Italy of preoperative treatment with ulipristal acetate at the dose of 5 mg/day for 13 weeks in comparison with placebo prior to surgical management of symptomatic uterine fibroids., Study Design: The pharmacoeconomic analysis was based on the calculation of incremental cost-effectiveness ratio (ICER). Effectiveness data were derived from the randomized-controlled trial PEARL-1, whilst costs data were retrieved from the published literature. A Markov model was employed to simulate the pattern of costs and two univariate sensitivity analyses tested the robustness of the results., Results: In comparison with placebo, ulipristal acetate 5 mg for presurgical therapy was estimated to be associated with an incremental cost of €351 per patient. Costs per patient were €3836 for ulipristal acetate vs €3485 for placebo. The incremental effectiveness was 0.01931 QALYs per patient (around 7 quality-adjusted days per patient). Hence, the cost effectiveness ratio was calculated to be €18,177 per QALY gained., Conclusions: Preoperative use of ulipristal acetate 5 mg in patients with uterine fibroids has a favourable pharmacoeconomic profile., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Competing Interests: Disclosure The authors report no conflicts of interest in this work.

Competing Interests: Disclosure The authors report no conflicts of interest in this work.

Competing Interests: Disclosure The authors report no conflicts of interest in this work.

Competing Interests: Disclosure The authors report no conflicts of interest in this work.

Competing Interests: Disclosure The authors report no conflicts of interest in this work.

Competing Interests: Conflict of Interest: The authors declare no conflict of interest.

Intravenous leiomyomatosis is a life-threatening leiomyoma that grows into the extrauterine venous system. A high recurrence rate has been reported in reproductive-age women who undergo only tumor excision to preserve fertility. Precise diagnosis of tumor extension is essential to achieve complete resection. A 24-year-old woman presented with hypermenorrhea. Contrast-enhanced MRI showed an intramural myoma with worm-like extension into the right parametrium. F-FES PET/MRI accurately depicted the extension with strong FES activity into the right uterine vein, whereas F-FDG PET/MRI excluded the possibility of malignancy. These modalities can be a novel strategy to manage such cases of intractable intravenous leiomyomatosis.

Intracardiac leiomyomatosis is a rare complication that occurs when a uterine leiomyoma (fibroid) undergoes vascular invasion and propagates within the inferior vena cava to reach the right atrium. This article describes a case of intracardiac leiomyomatosis in a middle-aged woman, exploring the presentation, diagnosis and surgical management of this condition. In this case the presenting complaints were syncope and atrial fibrillation, illustrating the importance of performing a transthoracic echocardiogram in patients presenting with their first episode of atrial fibrillation. Clinicians should consider intracardiac leiomyomatosis when evaluating women with right heart masses, especially those with a history of uterine leiomyomas., (Copyright © 2016 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). All rights reserved.)

Objectives: To characterize and describe treatment with Ulipristal acetate (UPA) in a pre-operative setting and to evaluate the safety, effectiveness, and Health Related Quality of Life (HRQoL) outcomes in a population treated according to standard clinical practice in the EU., Study Design: Multi-centre, prospective, non-interventional study (PREMYA) of patients diagnosed with moderate to severe symptoms of uterine fibroids and undergoing a pre-operative treatment with UPA (Esmya ® ) at 73 clinical practice sites within the EU. Patients were followed during UPA treatment and for 12 months after treatment discontinuation for a total of 15 months follow-up. Data was collected every 3 months in accordance with standard care visits., Results: A total of 1568 women were enrolled, of whom 1473 were found to be eligible for data analysis. Only 38.8% of patients underwent surgery, of which the majority were of a conservative/minimally invasive nature. Physicians' assessments of patients' overall symptomatic change, as measured on the Clinical Global Impression-Improvement (CGI-I) scale, indicated that 60% of patients were much improved or very much improved at 3 months. Pain and quality of life after treatment cessation remain lower than baseline during the entire period of follow-up CONCLUSIONS: The majority of patients do not undergo surgery immediately after treatment cessation. Quality of life and pain are highly improved by Esmya ® treatment., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

Aim: The aim of our study was to explore the effects on symptoms and female sexual function of the medical management with gonadotropin-releasing hormone agonist (GnRHa) in women of more than 45 years old compared to surgical management., Methods: Women with symptomatic uterine fibroids were enrolled to participate to the present open-label study. We offered two different treatment options: medical with GnRHa for 6 months (group A) or hysterectomy (group B). The patients were reviewed in follow-up for 24 months. The impact of medical or surgical therapy on sexual life was evaluated., Results: No significant differences were found in population characteristics between the two groups. GnRHa treatment was efficient in reducing symptoms in 88% of patients but 22% of patients needed adjunctive cycles of medical therapy. After 24 months, 16% of the patients did not complete the study. The failure percentage of the medical treatment was 12%. No severe side-effects were recorded, and eight patients had reached menopause. No significant differences were observed in the Female Sexual Function Index score in each domain between the medical and surgical groups, with total scores of 18.94 ± 10.16 and 22.00 ± 8.86, respectively (mean ± standard deviation), and the prevalence of dysfunction was 12% and 22%, respectively, similar to the general population of the same age., Conclusion: We found that medical therapy with GnRHa is a satisfactory alternative to surgery for fibroids in women of more than 45 years old., (© 2013 The Authors. Journal of Obstetrics and Gynaecology Research © 2013 Japan Society of Obstetrics and Gynecology.)

The myoma pseudocapsule (MP) is a fibro-vascular network rich of neurotransmitters, as a neurovascular bundle, surrounding fibroid and separating myoma from myometrium. We investigated the distribution of the opioid neuropeptides, as enkephalin (ENK) and oxytocin (OXT), in the nerve fibers within MP and their possible influence in human reproduction in 57 women. An histological and immunofluorescent staining of OXT and ENK was performed on nerve fibers of MP samples from the fundus, corpus and isthmian-cervical regions, with a successive morphometric quantification of OXT and ENK. None of the nerve fibers in the uterine fundus and corpus MPs contained ENK and the nerve fibers in the isthmian-cervical region demonstrated an ENK value of up to 94 ± 0.7 CU. A comparatively lower number of OXT-positive nerve fibers were found in the fundal MP (6.3 ± 0.8 CU). OXT-positive nerve fibers with OXT were marginally increased in corporal MP (15.0 ± 1.4 CU) and were substantially higher in the isthmian-cervical region MP (72.1 ± 5.1 CU) (p < 0.01). The distribution of OXY neurofibers showed a slight into the uterine corpus, while are highly present into the cervico-isthmic area, with influence on reproductive system and sexual disorders manifesting after surgical procedures on the cervix.

Uterine leiomyomas are the most common benign gynecologic condition. The prevalence is three times more common among women of African ethnicity. Disparity in this disease is evidenced by earlier age of onset, greater severity of symptoms, and different response to treatment. Although the pathogenesis of disease development is not completely known, growing evidence focuses on investigating the molecular mechanisms in disease development and the influence of ethnicity. Variation in the expression levels or function of estrogen and progesterone receptors, polymorphism of genes involved in estrogen synthesis and/or metabolism (COMT, CYP17), retinoic acid nuclear receptors (retinoid acid receptor-α, retinoid X receptor-α), and aberrant expression of micro-RNAs (miRNAs) are some of the molecular mechanisms that may be involved. Nutritional factors, such as vitamin D deficiency, might also contribute to the higher incidence in dark skinned populations who are also commonly suffer from hypovitaminosis D. Culture and environmental difference might have a role in disease development. Further analysis and better understanding of these mechanisms will provide insight into the molecular basis of racial disparities in leiomyoma formation and will help to develop new innovations in leiomyoma treatment., (Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.)

The uterine myoma pseudocapsule is a neurovascular bundle surrounding fibroid, containing neuropeptides, probably involved in uterine scar healing. We studied neurotensin (NT), neuropeptide tyrosine (NPY), and protein gene product 9.5 (PGP 9.5) nerve fibres in the pseudocapsule neurovascular bundle of intramural uterine fibroids on 67 no pregnant women by intracapsular myomectomy sparing the neurovascular bundle, sampling full thickness specimens of the pseudocapsule of uterine fibroids (PUF) and normal myometrium (NM) obtained from the fundus uteri (FU) and the uterine body (UB). The samples were sent for histological and immunofluorescent analyses and compared by morphometrical quantification. The Conventional Unit (C.U.) difference of NT, NPY, and PGP 9.5 nerve fibres was statistically analyzed. Our results showed that NT, NPY, and PGP 9.5 neurofibers are almost equally present in PUF as in NM of a no pregnant uterus. As all of these neuropeptides are present in the uterine muscle and can affect muscle contractility, uterine peristalsis and muscular healing. A myomectomy respecting the pseudocapsule neurofibers should facilitate smooth muscle scarring and promote restoration of normal uterine peristalsis with a possible positive influence on fertility.

Background: Our aim was to assess surgical complaints and reproductive outcomes of laparoscopic intracapsular myomectomies by a prospective observational study run in University affiliated hospitals., Methods: Between 2005 and 2010, 235 women underwent subserous and intramural laparoscopic myomectomy of fibroids (4-10 cm in diameter) for indications of pelvic pain, menstrual disorders, a large growing myoma or infertility. The main outcome measures were post-surgical parameters, including complications, the need for subsequent surgery or symptomatic relief, resumption of normal life and reproductive outcome., Results: Pelvic pain occurred in 27%, menorrhagia or metorrhagia in 21%, a large growing myoma in 10% and infertility in 42% of women. Single fibroids occurred in 51.9% of patients while 48.1% had multiple myomas. Of all patients, 58.2% had subserosal and 41.8% had intramural myomas. No laparoscopies were converted to laparotomy. In 3 years, 1.2% of patients had a second laparoscopic myomectomy for recurrent fibroids. The mean total operative laparoscopic time was 84 min (range 25-126 min), with mean blood loss of 118 ± 27.9 ml. By 48 h after surgery, 86.3% were discharged with no major post-operative complications. No late complications, such as bleeding, urinary tract infections or bowel lesions, occurred. Of the women who underwent myomectomy for infertility, 74% finally conceived. At term, 32.9% of patients underwent Caesarean section, 24.8% delivered by vacuum extractor and 42.2% had spontaneous deliveries. No case of uterine rupture occurred., Conclusions: Intracapsular subserous and intramural myomectomy saving the fibroid pseudocapsule showed few early and no late surgical complications, enhanced healing by preserving myometrial integrity and allowed a good fertility rate and delivery outcome. In young patients suffering fibroids, laparoscopic intracapsular myomectomy is a potential recommended surgical treatment.

Objective: To describe a case report of diffuse uterine leiomyomatosis who had successful pregnancy twice following conservative management., Design: Retrospective report., Setting: Private general hospital., Patient(s): A nulliparous woman 25 years of age presented with menorrhagia and infertility. She had innumerable small fibroids of 4-42 mm size throughout the myometrium. Size of the symmetrically enlarged uterus was 131×80×60 mm, clinically corresponding to that of 12 weeks of gestation., Intervention(s): She received a GnRH analogue (GnRHa; leuprolide acetate) 3.75 mg per month for 6 months., Main Outcome Measure(s): Reduction of uterus size, menstrual amount, conception, pregnancy outcome., Result(s): Enlarged uterus reduced to almost normal size after 3 doses of GnRHa. She did not experience heavy bleeding during menstruation. She conceived spontaneously in the first cycle after discontinuation of GnRHa. Antenatal course was uneventful. A healthy male baby of 2.5 kg was delivered by cesarean section at 39 weeks. The placenta weighed 330 g. There was no postpartum hemorrhage. She conceived spontaneously for the second time in the first cycle after resumption of menses. Antenatal, intranatal (cesarean section), and postnatal courses of the second pregnancy were uneventful. The second neonate weighed 3.0 kg and the placenta 400 g., Conclusion(s): Conservative treatment may help to achieve successful pregnancy in case of diffuse uterine leiomyomatosis., (Copyright © 2011 American Society for Reproductive Medicine. Published by Elsevier Inc. All rights reserved.)

X-linked Alport syndrome is associated in some families with diffuse leiomyomatosis. We describe herein, the pulmonary complications and outcome of three family members (mother, daughter, and son). The three underwent esophagectomy at different ages (22 years, three years, and 15 months respectively). Their current forced expiratory volume in the first second (FEV1) ranged from 33% in the mother to 60% in the daughter and 97% in the son. It is suggested that earlier intervention may lead to improved pulmonary function tests., (Copyright © 2011 Wiley-Liss, Inc.)

Objective: To report all pregnancies to date after magnetic resonance-guided focused ultrasound surgery (MRgFUS) for the conservative treatment of clinically significant uterine fibroids., Design: Prospective registry of all known pregnancies occurring after MRgFUS maintained by the device manufacturer and reported to the Food and Drug Administration., Setting: World experience of pregnancies after treatment with reports from 13 sites in seven countries., Patient(s): Fifty-one reproductive-age women with uterine leiomyomas., Intervention(s): Women underwent MRgFUS treatment for symptomatic uterine leiomyomas before this report., Main Outcome Measure(s): Pregnancy outcomes and complications., Result(s): Fifty-four pregnancies in 51 women have occurred after MRgFUS treatment of uterine leiomyomas. The mean time to conception was 8 months after treatment. Live births occurred in 41% of pregnancies, with a 28% spontaneous abortion rate, an 11% rate of elective pregnancy termination, and 11 (20%) ongoing pregnancies beyond 20 gestational weeks. The mean birth weight was 3.3 kg, and the vaginal delivery rate was 64%., Conclusion(s): Preliminary pregnancy experience after MRgFUS is encouraging, with a high rate of delivered and ongoing pregnancies., (Copyright 2010 American Society for Reproductive Medicine. Published by Elsevier Inc. All rights reserved.)

The purpose of this study was to evaluate the fibroid morphology in a cohort of women achieving pregnancy following treatment with uterine artery embolization (UAE) for symptomatic uterine fibroids. A retrospective review of magnetic resonance imaging (MRI) of the uterus was performed to assess pre-embolization fibroid morphology. Data were collected on fibroid size, type, and number and included analysis of follow-up imaging to assess response. There have been 67 pregnancies in 51 women, with 40 live births. Intramural fibroids were seen in 62.7% of the women (32/48). Of these the fibroids were multiple in 16. A further 12 women had submucosal fibroids, with equal numbers of types 1 and 2. Two of these women had coexistent intramural fibroids. In six women the fibroids could not be individually delineated and formed a complex mass. All subtypes of fibroid were represented in those subgroups of women achieving a live birth versus those who did not. These results demonstrate that the location of uterine fibroids did not adversely affect subsequent pregnancy in the patient population investigated. Although this is only a small qualitative study, it does suggest that all types of fibroids treated with UAE have the potential for future fertility.

Objective: To assess the effect of low-dose mifepristone on quality of life, pain, bleeding, and uterine size among women with symptomatic leiomyomata., Methods: Forty-two women with symptomatic uterine leiomyomata and uterine volume of 160 mL or more were randomized to mifepristone, 5 mg daily, or placebo for 26 weeks. Quality of life (Uterine Fibroid Symptoms Quality of Life Questionnaire and Medical Outcomes Study 36-Item Short Form survey) and uterine and leiomyoma size (ultrasonography) were assessed at baseline, and at 1 month, 3 months, and 6 months of treatment. Bleeding (daily logs and pictorial charts) and pain (McGill Pain Questionnaire) were assessed monthly. Endometrial pathology was assessed at baseline and 6 months., Results: Forty-two women were randomized; 37 women completed all 6 months. Women randomized to mifepristone showed an improvement in leiomyoma-specific quality of life. Forty-one percent became amenorrheic, rates of anemia improved, and adjusted uterine size was reduced by 47%. Compared with the placebo group, improvements in these outcomes in the treatment group were significantly greater (P<.05 to .001). There were no significant differences in adverse effects between the groups. No endometrial hyperplasia was noted in any participant., Conclusion: Low-dose mifepristone improves leiomyoma-specific quality of life and reduces leiomyoma size among women with symptomatic leiomyomata., Clinical Trial Registration: ClinicalTrials.gov www.clinicaltrials.gov NCT00133705, Level of Evidence: I.

Objective: To review the available information regarding the role of cytokines, growth factors, and the extracellular matrix in the pathophysiology of uterine leiomyomata and to integrate this information in a suggested model of disease at the cellular level., Design: A thorough literature and MEDLINE search was conducted to identify the relevant studies in the English literature published between January, 1966 and October, 2001. A model of disease at the cellular level was developed using the most likely cytokines to be involved in the pathogenesis of leiomyomata as determined by our assessment of the available literature., Result(s): A number of cytokines and growth factors, including transforming growth factor-beta (TGF-beta), epidermal growth factor, monocyte chemotactic protein-1, insulin-like growth factors 1 and 2, prolactin, parathyroid-hormone-related peptide, basic fibroblast growth factor, platelet-derived growth factor, interleukin-8, and endothelin, have been investigated in myometrium and leiomyoma. Among these cytokines, TGF-beta appears to be the only growth factor that has been shown to be overexpressed in leiomyoma vs. myometrium, be hormonally-regulated both in vivo and in vitro, and be both mitogenic and fibrogenic in these tissues. In addition to the cytokines, extracellular matrix components such as collagen, fibronectin, proteoglycans, matrix metalloproteinases, and tissue inhibitors of metalloproteinases seem to play pivotal roles in the pathogenesis of leiomyomata., Conclusion(s): We believe that, given the extent and depth of the current research on the cellular biology of leiomyomata, the cellular mechanisms responsible in the pathogenesis of leiomyomata will be identified clearly within the foreseeable future. This will enable researchers to develop therapy directed against the molecules and mechanisms at the cellular level.

Results of the right uterine artery dopplerometry at pregnancy 39-39 weeks and those of subsequent histotopographic and immunomorphologic study of the placental bed in the amputated uteri were compared in 50 pregnant women. Morphofunctional equivalent of a considerable reduction of the uterine-placental circulation depending on the myomatous tissue spread and particularly in rare observations of "the placenta on the node" were found. Pathogenesis of these disturbances is determined by deficiency of the second wave of interstitial cytotrophoblast invasion, by phenomenon of the uterine-placental circulation decrease due to arterial supply of myomatous nodes and local hormonal changes produced by alterations of cellular-tissular correlations in the uterine-placental bed.

Three cases of intravenous leiomyomatosis (IVL) of the uterus, a rare benign smooth-muscle tumor, are described. A preoperative diagnosis of IVL was not made in any of the patients, all of which presented with a pelvic mass with the presumptive diagnosis of leiomyoma. Surgical exploration confirmed the presence of uterine mass and two of the three cases showed extra-uterine extension into the ovarian or uterine veins. Histological examination demonstrated a fascicular pattern of bland spindle-shaped smooth-muscle cells, which extended to veins inside the myometrium or to extrauterine veins. This was confirmed by immunohistochemical stain for desmin and factor VIII. Despite their histological benignity, these lesions have a tendency to metastasize and are closely related to the conditions called "benign metastasizing leiomyoma" and "intracaval mass and cardiac extension". The primary treatment of IVL is hysterectomy and excision of any extrauterine tumor, when technically feasible. Anti-estrogenic therapy has been suggested as potentially useful in controlling of unresectable tumor. According to the literature, the follow-up must be long and periodic postoperative ultrasonic or magnetic nuclear resonance imaging studies may be useful in detecting growth of residual intravascular tumor.

The clinical and pathological features of four cases of the rare entity diffuse leiomyomatosis of the uterus are described. The patients, who ranged in age from 28 to 35 years, presented with a six month to ten year history of abnormal uterine bleeding, dysmenorrhoea and an enlarged uterus. Hysterectomy in each case revealed a symmetrically enlarged uterus containing numerous small ill-defined leiomyomatous nodules. On microscopic examination the nodules were composed of compact fascicles and interweaving bundles of plump uniform benign smooth muscle cells. The nodules blended with each other and merged imperceptibly with the surrounding less cellular normal myometrium. The four patients are alive and well without further treatment. The follow-up period ranged from six months to twelve years.