Smooth Muscle Tumor of Uncertain Malignant Potential Arising from Renal Vein

Smooth muscle tumors (SMTs) typically fall under either benign leiomyoma or malignant leiomyosarcomas based on histologic characteristics. SMTs that cannot be diagnosed as benign or malignant are then labeled as smooth muscle tumors of uncertain malignant potential (STUMP). A 31-year-old male presented with the right abdominal pain for 6 months. Imaging showed an enhancing soft-tissue mass arising from the right renal vein. A pre-operative biopsy confirmed STUMP of renal vein, and the patient underwent surgical resection of the mass with partial nephrectomy. Imaging and pathologic findings play a crucial role in an accurate pre-procedural diagnosis of unusual SMTs.

Thrombocytopenia and Deep Vein Thrombosis on the Top of an Iceberg

Systemic Lupus Erythematous (SLE) is a chronic autoimmune disorder with a relapsingremitting course. Besides, SLE most commonly occurs in child-bearing-age women. Due to protean manifestations, the diagnosis may be challenging; however, a high index of suspicion, i.e. achieved by experience and perceptivity is the key to a correct decision. Here, we present an SLE patient; her initial symptoms resembled a malignant process, but important elements guided us to the underlying autoimmune process. A 34-year-old woman presented with a uterine mass, deep vein thrombosis, and significant thrombocytopenia. Our first impression was malignant processes; however, considering her age with thrombosis and thrombocytopenia as the key components of her presentation, we suspected antiphospholipid syndrome and SLE. Her abdominal mass was a benign leiomyoma. We treated her with glucocorticoids and anticoagulants. Accordingly, after improving cytopenia, we discharged her and the follow-up examination result was satisfactory.

Developing a Preoperative Algorithm for the Diagnosis of Uterine Leiomyosarcoma

Early diagnosis of the rare and life-threatening uterine leiomyosarcoma (LMS) is essential for prompt treatment, to improve survival. Preoperative distinction of LMS from benign leiomyoma remains a challenge, and thus LMS is often diagnosed post-operatively. This retrospective observational study evaluated the predictive diagnostic utility of 32 preoperative variables in 190 women who underwent a hysterectomy, with a postoperative diagnosis of leiomyoma (n = 159) or LMS (n = 31), at the Liverpool Women’s National Health Service (NHS) Foundation Trust, between 2010 and 2019. A total of 7 preoperative variables were associated with increased odds of LMS, including postmenopausal status (p < 0.001, OR 3.08), symptoms of pressure (p = 0.002, OR 2.7), postmenopausal bleeding (p = 0.001, OR 5.01), neutrophil count ≥7.5 × 109/L (p < 0.001, OR 5.72), haemoglobin level <118 g/L (p = 0.037, OR 2.22), endometrial biopsy results of cellular atypia or neoplasia (p = 0.001, OR 9.6), and a mass size of ≥10 cm on radiological imaging (p < 0.0001, OR 8.52). This study has identified readily available and easily identifiable preoperative clinical variables that can be implemented into clinical practice to discern those with high risk of LMS, for further specialist investigations in women presenting with symptoms of leiomyoma.

Successful pregnancy after complete resection of leiomyomatosis peritonealis disseminate without recurrence:A case report with next generation sequencing analysis and literature review

Background: Peritoneal leiomyomatosis disseminate (LPD) is a rare disease characterized by widespread dissemination of leiomyomas nodules throughout the peritoneal and omental surfaces. Reports of pregnancy with LPD are even rarer. Therefore, there is no clear consensus on the treatment of LPD on pregnancy, and the pathogenesis is still unclear. Case presentation：We reported a case of LPD patient who developed during pregnancy. The patient underwent cesarean section at 32 weeks of gestation while removing all visible tumors, and no LPD lesions were seen in the subsequent cesarean section at full-term. NGS of LPD lesions detected 4 mutations with focal high-level amplifications of CDK4 (Cyclin-dependent kinases 4), NBN (Nibrin), DAXX (Death domain associated protein) and MYC (Myelocytomatosis oncogene). Immunohistochemistry staining analysis among benign leiomyoma, LPD and leiomyosarcoma verified that LPD was an unusual intermediate between benign and malignant uterine smooth muscle tumors. Besides, LPD is a hormonal-dependent leiomyoma. After detailed literature search, we summarized the detailed clinical features and follow-up information of patients with LPD during pregnancy.Conclusions: This is the first reported LPD case of successful term pregnancy without recurrence, following resection of all visible lesions in prior pregnancy. LPD is an unusual intermediate between benign and malignant uterine smooth muscle tumors.

Spontaneous Rupture of Leiomyoma Diagnosed Preoperatively with Noncontrast Computed Tomography

Background. Spontaneous rupture of benign uterine fibroids is extremely rare and has been associated with fibroid degeneration. It can cause acute intraperitoneal bleeding requiring immediate surgical intervention. Case. A previously healthy 50-year-old, Caucasian, nullipara presented with syncope, hemodynamic instability, and an acute abdomen. Noncontrast computed tomography images showed a positive sentinel clot sign in the pelvis as well as a large uterine fibroid with internal hyperdense clot suggesting acute rupture. Urgent laparotomy and hysterectomy confirmed a ruptured, actively bleeding, uterine fibroid with final pathological diagnosis of a benign leiomyoma. Conclusion. Prompt diagnosis and emergency surgical intervention were necessary to control acute hemorrhage from a ruptured uterine fibroid. Noncontrast computed tomography is an important adjunct to contrast-enhanced computed tomography and was vital for diagnosis in this case.

Successful pregnancy after complete resection of leiomyomatosis peritonealis disseminate without recurrence: A case report with next generation sequencing analysis and literature review

Background: Peritoneal leiomyomatosis disseminate (LPD) is a rare disease characterized by widespread dissemination of leiomyomas nodules throughout the peritoneal and omental surfaces. Reports of pregnancy with LPD are even rarer. Therefore, there is no clear consensus on the treatment of LPD on pregnancy, and the pathogenesis is still unclear. Case presentation：We reported a case of LPD patient who developed during pregnancy. The patient underwent cesarean section at 32 weeks of gestation while removing all visible tumors, and no LPD lesions were seen in the subsequently cesarean section at full-term. NGS of LPD lesions detected 4 mutations with focal high-level amplifications of CDK4 (Cyclin-dependent kinases 4), NBN (Nibrin), DAXX (Death domain associated protein) and MYC (Myelocytomatosis oncogene). Immunohistochemistry staining analysis among benign leiomyoma, LPD and leiomyosarcoma verified that LPD was an unusual intermediate between benign and malignant uterine smooth muscle tumors. Besides, LPD is a hormonal-dependent leiomyoma. After detailed literature search, we summarized the detailed clinical features and follow-up information of patients with LPD during pregnancy. Conclusions: This is the first reported LPD case of successful term pregnancy without recurrence, following resection of all visible lesions in prior pregnancy. LPD is an unusual intermediate between benign and malignant uterine smooth muscle tumors.