GnRH agonist for intravenous leiomyomatosis with cardiac extension. A case report.

BACKGROUND: Intravenous leiomyomatosis with cardiac extension is an extremely rare disease. CASE: We recently treated a case of intravenous leiomyomatosis with extension from the inferior vena cava into the right atrium. Three operations--exploratory laparotomy, debulking of the pelvic mass and resection of the intracardiac leiomyoma--were performed. Since cells of the resected leiomyomatosis were estrogen receptor positive, we postoperatively administered GnRH agonist (leuprorelin acetate) for six months to prevent regrowth of the residual mass in the pelvis. The residual mass began to enlarge immediately after cessation of leuprorelin acetate. The same medication was readministered, and regrowth of the residual mass was completely inhibited for 15 months, until this writing. CONCLUSION: Intravenous leiomyomatosis seems to be hormone dependent, as in the case of uterine leiomyomas. In the absence of total resection, functioning ovarian tissue may remain. Therefore, long-term treatment with GnRH agonist may be useful in preventing recurrence of this disease.     

Intravenous leiomyomatosis with cardiac extension

Intravenous leiomyomatosis with cardiac extension is an extremely rare uterine tumor. We report here a case of a patient with a uterine leiomyoma which extended into the right atrium through the left ovarian vein, progressing into the left renal vein along the inferior vena cava. Complete one-stage removal of the tumor was performed using cardiopulmonary bypass, and the patient has shown a favorable outcome. Successful therapy for intravenous leiomyomatosis is dependent on total surgical excision of the tumor, cessation of ovarian function and avoidance of postoperative estrogen replacement therapy.     

Intravenous leiomyomatosis: Case report and review of the literature

IntroductionWe retrospectively reviewed a case of a 42-year-old woman with intravenous leiomyomatosis.Clinical findingsThe present study describes a case of IVL extending into the right internal and common iliac veins.DiagnosisThe patient was diagnosed by definitive pathological examination after a hysterectomy and double salpingectomy. Hypovolaemic shock due to delayed intraperitoneal bleeding from the ovarian vessels pedicle was observed. An emergent laparotomy for haemostasis was performed. A follow-up MRI, 2 months later, showed a polylobulated mass extending inside the right internal and common iliac veins.Intervention and resultThe patient underwent a third laparotomic procedure with the removal of the right pelvic mass together with the involved veins. The postoperative course was uneventful, and the patient remains well at 3-month follow-up, with no signs of lower limb edema or venous disorders.ConclusionIntracardiac leiomyomatosis is mostly diagnosed in premenopausal women. The most severe manifestation could be a vascular thrombosis or a right atrial tumor in the case of intracardiac involvement. Early and appropriate diagnosis is essential for optimal treatment. Surgery is the best treatment.     

Uterine intravenous leiomyomatosis. A case report

Uterine intravenous leiomyomatosis is a rare benign tumor. We report the case of a 41-year-old woman, with no history, who presented an abdominopelvic mass arising from the uterus. Histological examination revealed uterine intravenous leiomyomatosis. The specificity of this tumor is hormonodependency and potential vascular extension.     

Intravenous uterine leiomyomatosis with diffuse dissemination in pelvic cavity. Case report and literature review

Diffuse uterine leiomyomatosis is a rare benign entity in gynecology, with only 30 worldwide reports. Its most frequent complications are dissemination into vena cava, cardiac obstruction (right cavities and valves), and diffuse pelvic invasion. We report a case of a 40 years old female with history of pelvic mass resection. During surgery it was found a large abdominal-pelvic mass diffusely invading uterine body, and round and infundibulum-pelvic ligaments. This mass extends itself out of pelvis until aorto-abdominal bifurcation, and has gummatous, elastic, and highly bleeding consistency. Histopathologic report was intravenous leiomyomatosis with benign smooth muscular fibers infiltrating adjacent structures. Its origin was uterine tissue, but it invades, adjacently, all reproductive annexes deforming them anatomy. Its infiltrative quality confers a technical difficulty to surgeon, so he or she will need a multidisciplinary team to establish its treatment.     

子宫静脉内平滑肌瘤病的诊治进展

子宫静脉内平滑肌瘤病（intravenous leiomyomatosis,IVL）是一种罕见的疾病,可沿血管生长,延伸到下腔静脉,甚至心脏。目前对其发病机制尚有争议,多数学者考虑其起源于子宫肌瘤直接侵入子宫肌层静脉并沿血管腔扩散所致。子宫IVL好发于40～50岁有生育史的女性,早期临床表现不典型,如累及下腔静脉或右心时,可出现腹水、肝脾肿大、呼吸困难、心力衰竭,严重者甚至猝死。手术是目前首选的治疗方法,但具体的手术方案尚无统一共识。该病复发率高,术后需长期严密随访。综述子宫IVL的发病机制及高危因素、临床及影像学表现、诊断与鉴别诊断、治疗、预后及随访,以期为该病的诊疗提供参考。     

Tricky Diagnosis and Robot-laparoscopic Surgical Approach to Disseminated Peritoneal Leiomyomatosis

Study ObjectiveTo show the challenging diagnosis of, and safe robotic surgical approach to, a rare case of disseminated peritoneal leiomyomatosis (DPL).DesignA clinical case shown by a sequential demonstration of investigation, diagnosis, and surgical approach, with narrated video footage.SettingDPL is a rare disease, with only a little more than 150 cases reported in the literature [1]. It is defined by subperitoneal proliferation of benign smooth muscle cell nodules, macroscopically mimicking peritoneal carcinomatosis [2]. The etiology remains unclear, but different hypotheses have been put forward, such as subperitoneal mesenchymal stem cell metaplasia and iatrogenic origin after myomectomy [3]. Despite its usual benign behavior, DPL can rarely present with malignant degeneration, and therefore a complete resection of multiple lesions is recommended [4]. This case involves a 45-year-old patient presenting with dysmenorrhea, abdominal pain, and major abnormal uterine bleeding, requiring previous blood transfusion and no response after 2 years of clinical treatment. She had a previous cesarean delivery, with no reproductive desire at present and no history of other pelvic surgeries. Pelvic examination showed an enlarged mobile uterus at the height of the pubic symphysis, and both ultrasonography and magnetic resonance imaging confirmed an enlarged uterus due to multiple myomas.Interventions(1) Diagnostic laparoscopy with implant biopsies and uterine curettage for investigation of DPL and its differential diagnoses, followed by robot-assisted laparoscopic approach, with key strategies for a safe performance. (2) Radical hysterectomy with bilateral salpingo-oophorectomy, omentectomy, and wide pelvic peritoneal resection were performed. (3) For this complex procedure, the identification and preservation of important landmarks and pelvic anatomy were mandatory, as well as removal of all surgical specimens in monobloc. Final pathology report: disseminated leiomyomatosis with no evidence of malignancy. The patient had no complications after surgery and was discharged on the second postoperative day with mild abdominal pain.ConclusionDPL diagnosis can be tricky owing to its macroscopic similarity to peritoneal carcinomatosis and the difficulty of identification in imaging examinations. Moreover, the robotic platform can be a helpful and safe tool for the surgical treatment of DPL and complete resection of all peritoneal lesions.     

Intravenous leiomyomatosis of the uterus

We present a case of intravenous leiomyomatosis (IVL) associated leiomyoma of the uterus in a 57-year-old woman. It is clinically and morphologically suspected to be leiomyoma. IVL is a rare benign neoplasm that commonly grows into the pelvic veins and the inferior vena cava and, rarely, the right side of the heart. Histologically benign smooth-muscle tumor arises from either a uterine myoma or the wall of a uterine vessel with extension into veins. The primary treatment of IVL is hysterectomy and the excision of any extrauterine tumor, when technically feasible. Antiestrogenic therapy has been suggested as potentially useful in controlling of the unresectable tumor.     

Intracardiac leiomyomatosis

We describe a case of intracardiac leiomyomatosis originating from the uterus, growing up in the inferior vena cava, and extending into the right ventricle. She also found to have lung metastasis. As she declined for further operative intervention, Tamoxifen was given for the control of disease. This case represented an unusual rapid recurrence of intravenous leiomyomatosis which was potentially preventable. To investigate for the appropriate management, a review of the literature concerning this rare condition was made. Received: 5 June 2000 / Accepted: 26 July 2000     

Rare tumours of myometrium--intravenous leiomyomatosis and benign metastatic leiomyoma

Leiomyomas are the most common benign uterine neoplasms of the myometrium. Rarely, can be observed unusual manifestations of these tumours. We present a case of intravenous leiomyomatosis in a 52-year-old woman and a case of benign metastasizing leiomyoma in a 45-year-old woman. Intravenous leiomyomatosis is an uncommon condition in which morphologically benign smooth muscle is present within the lumina of the veins. Benign metastasizing leiomyoma is a very rare phenomenon where histologically benign smooth muscle tumours are present at distant sites, particularly in the lungs, in women having histologically benign uterine leiomyoma. The diagnostic features, etiology, histology, therapeutic management and prognosis of these extremely rare conditions are discussed.     

Granulosa Cell Tumor of the Ovary Accompanying with Ollier’s Disease: First Case of Contralateral Presentations

Objective

Granulosa cell tumor (GCT) is a rare entity of ovarian malignancies. Juvenile GCT is considered a malignant tumor with an indolent course and tendency toward late recurrence. However, the association of this tumor and multiple enchondromas has been reported.

Case Presentation

A 17-year-old female with abnormal uterine bleeding was referred to our center. Ultrasonographic evaluation revealed a mass with origin in right ovary. Patient was worked up to undergo salpingo-oophorectomy, she felt a dull pain in her left lower limb. X-ray imaging was indicative for Ollier’s disease at the distal part of femur and proximal part of tibia. Postoperative pathological review was compatible with juvenile granulosa tumor of the right ovary.

Conclusion

This case was the first of its kind that ovarian tumor was contralateral to the side involved by enchondromatosis.

     

Pre-operative diagnosis of a primary uterine mature teratoma

ObjectiveMature cystic teratoma is a common benign ovarian tumor. But extragonadal teratomas are very rare. They mainly occur in the midline structure of the body. Uterine teratomas are extremely rare with only few reports. The diagnosis was mainly based on the operative findings. We report a case of uterine mature teratoma in a 37 year-old woman who was diagnosed before the operation. We also review the literature about this exceptional presentation.Case reportWe report a case of uterine teratoma that was initially diagnosed as a uterine tumor under ultrasound examination. But teratoma was highly suspected preoperatively by the abdominal CT scan. She underwent tumor excision via laparotomy. The operative finding and the histological examination confirms the diagnosis of primary uterine teratoma.ConclusionPreoperatively diagnosis of uterine teratoma was difficult. Although there are no gold standard to treat the uterine teratoma, the majority of the treatment choice is surgery. The prognosis of this unusual disease is relatively good in benign lesions.     

A case with diffuse uterine leiomyomatosis and review of the literature

Leiomyomas and diffuse uterine leiomyomatosis are smooth muscle tumors of the uterus. Diffuse uterine leiomyomatosis is a benign and extremely rare condition in which the uterus is symmetrically enlarged as a result of the almost complete replacement of the myometrium by innumerable poorly defined, confluent nodules. The etiology of these neoplasms is not completely understood. Initial symptoms of the diffuse uterine leiomyomatosis usually are abdominal pain and abnormal uterine bleeding. Similar to uterine leiomyomas, patients with leiomyomatosis present with menorrhagia, dysmenorrhea, abdominal pain, infertility, and pelvic pressure. Hormonal treatment usually fails to control the symptoms, anemia, or tumor growth after treatment is stopped. As a result, despite patients being in the third or fourth decades of life, hysterectomy has been the only permanent treatment option offered to patients for treatment of the symptoms related to uterine fibroids in diffuse leiomyomatosis. A case of a patient with a huge uterine mass (2,650 g in weight) who underwent hysterectomy due to diffuse uterine leiomyomatosis is presented together with a review of the literature.     

Juvenile Cystic Adenomyoma vs Blind Uterine Horn: Challenges in the Diagnosis and Surgical Management

BackgroundJuvenile cystic adenomyomas (JCAs) are rare uterine lesions. Differential diagnosis might be difficult. We present the case of an adolescent who was diagnosed with JCA and was managed with laparoscopic excision.CaseA 14-year-old patient with complaint of menarche with excruciating dysmenorrhea, was diagnosed using magnetic resonance imaging with a uterine anomaly consisting of a normal right hemiuterus, and a left cystic lesion with surrounding hypotense myometrium. She was managed with laparoscopic excision of the left side, and uterine reconstruction. Histology was suggestive of JCA, associated with diffuse adenomyosis. Dysmenorrhea improved considerably after surgery.Summary and ConclusionDifferential diagnosis between cystic uterine lesions relies on clinical, imaging, and perioperative clues that might assist in their formal classification. Doubt might still remain in some cases.     

Successful One-Stage Complete Removal of an Entire Intravenous Leiomyomatosis in the Heart, Vena Cava, and Uterus

The patient was a 61-year-old woman who had intravenous leiomyomatosis (IVL) extending into the right ventricle of the heart from the uterus. Tumors in the heart and the inferior vena cava were removed under cardiopulmonary bypass and then after terminating the cardiopulmonary bypass, tumors in the abdominal cavity including uterine leiomyoma were removed. This is the first case of extended IVL in which all tumors were successfully removed at one time. If a tumor is found in the right ventricle in a woman who had or currently has uterine leiomyoma, IVL should be suspected. Complete one-stage removal of tumors can be performed using cardiopulmonary bypass, and the present patient has shown a favorable outcome.     

子宫平滑肌瘤长入右心腔一例报道

子宫平滑肌瘤是女性生殖器官中最常见的良性肿瘤,进入静脉或淋巴管内称为脉管内平滑肌瘤病,局限于子宫可出现月经量增多、经期延长,肿瘤过大压迫可导致下肢肿胀、下腹部不适,累及髂静脉可引起腹痛,肿瘤向上生长累及下腔静脉、右心房、右心室、肺动脉等可出现呼吸困难、心悸、胸痛、胸闷、晕厥等,甚至可出现突发性大出血及休克。报道1例49岁女性患者因子宫平滑肌瘤长入右心腔导致下肢水肿3年,胸闷、胸痛2个月的病例,入院完善检查后予经腹全子宫+双侧附件+盆腔包块切除术,开胸行下腔静脉、右心房、右心室、肺动脉肿物清除术及房间隔缺损修补术,结合术中及病理结果,确诊为子宫平滑肌瘤、血管内平滑肌瘤。好转出院,随访8个月,肿瘤未复发。子宫平滑肌瘤侵入血管长入右心室达肺动脉者极少,病情严重,早发现、早诊断、早治疗尤为重要。     

Retroperitoneal angioleiomyomatosis

Retroperitoneal intravenous leiomyomatosis is a rare benign tumor that can spread through veins carrying significant morbidity. The challenge of its management lies within the complexity of completely excising the tumor, which if carried out improperly can result in neurological or vascular complications requiring complex reparative surgeries. Here we present the successful resection of a retroperitoneal angio-leiomyoma by combining laparoscopic route, micro-surgical techniques and modern endoscopic tools.Electronic supplementary materialThe online version of this article (10.1007/s13224-020-01404-7) contains supplementary material, which is available to authorized users.     

Leiomyomatosis peritonealis disseminata and ovarian Brenner tumor associated with tamoxifen use

Abstract. Bristow RE, Montz FJ. Leiomyomatosis peritonealis disseminata and ovarian Brenner tumor associated with tamoxifen use.
Tamoxifen is frequently administered as adjuvant therapy for breast carcinoma and produces weak estrogen agonist effects in estrogen sensitive tissues. In addition to producing a measurable increase in the risk of endometrial carcinoma, tamoxifen has also been associated with increasing size of uterine leiomyomata as well as the development of new leiomyomata. As the indications for tamoxifen therapy expand, surveillance for additional potential associated adverse outcomes is warranted.
A 44-year-old woman with a history of bilateral breast carcinoma presented with leiomyomatosis peritonealis disseminata and a right ovarian Brenner tumor 18 months after beginning adjuvant tamoxifen therapy. Although a causal link cannot be proven, this case is the second reported association between leiomyomatosis peritonealis disseminata, an ovarian Brenner tumor, and tamoxifen use for the treatment of breast carcinoma. Given the hormonal sensitivity of leiomyomatosis peritonealis disseminata, both mutagenic and mitogenic effects of tamoxifen on this rare entity must be considered. In the setting of continued hormonal treatment for breast carcinoma, the management of leiomyomatosis peritonealis disseminata presents unique clinical challenges.     

Pregnancy and Placenta Increta in a Noncommunicating Uterine Horn

BackgroundWe present a rare case of pregnancy and invasive placentation in a unruptured, noncommunicating rudimentary uterine horn at 20 weeks’ gestation.CaseThe patient was followed with ultrasound throughout early pregnancy and initial imaging for dating purposes showed a pregnancy within a communicating right horn of the uterus. At the 18-week anatomy ultrasound, the pregnancy was discovered to be within the noncommunicating, rudimentary left horn of the uterus. This was confirmed using pelvic magnetic resonance imaging. The patient opted for surgical management and subsequently underwent laparotomy and removal of the noncommunicating uterine horn and pregnancy. Placental tissue was adherent to the level of the serosa during surgery and pathologic diagnosis was significant for a placenta increta.Summary and ConclusionThe patient recovered well from surgery and subsequently went on to have a successful term pregnancy delivered via cesarean section for breech in the right horn 15 months later.     

Complete non-puerperal uterine inversion caused by uterine hemangioma: A case report

ObjectiveUterine inversion is a rare postpartum complication. Non-puerperal uterine inversion is extremely rare. It mostly occurs with uterine tumors, especially leiomyoma. In most instances, the inversion may not be noticed until the time of surgery. The preoperative diagnosis is difficult.Case reportWe report a case of non-puerperal complete uterine inversion that was initially diagnosed as cervical cancer. The uterine inversion was diagnosed preoperatively and she underwent total abdominal hysterectomy and bilateral salpingooophorectomy. The histological examination showed uterine hemangioma.ConclusionAccurate diagnosis of the non-puerperal uterine inversion is important. Surgical intervention is necessary and it provides good prognosis. Hemangioma may be one of the causes of non-puerperal uterine inversion.