Introduction: Pheochromocytoma is a catecholamine-secreting adrenal tumor associated with major perioperative hemodynamic instability. When concurrent major pelvic pathology requires surgery, operative planning becomes particularly challenging. We describe the successful single-stage management of pheochromocytoma and a giant uterine fibroid, highlighting the importance of multidisciplinary coordination and perioperative optimization. Case: A 49-year-old female with symptomatic uterine fibroid was found to have proliferative endometrium on a pipelle biopsy. Computed tomography (CT) abdomen incidentally detected a right adrenal mass alongside a large posterior uterine fibroid (11.9 × 17.4 × 14.6 cm). CT adrenal protocol demonstrated a heterogeneously enhancing right adrenal mass (6.9 × 6.9 × 9.6 cm) with high unenhanced attenuation. Biochemical evaluation revealed markedly elevated 24-hour urinary metanephrine (4.7× upper limit) and normetanephrine (2.4× upper limit). Following multidisciplinary discussions, a single-stage surgical approach was planned after careful assessment of feasibility and perioperative risk in view of the uncertain malignant potential of the pelvic mass and to minimize repeated exposure to anesthesia. Preoperative optimization included transitioning from terazosin to phenoxybenzamine, with subsequent addition of bisoprolol for hemodynamic control. The operative strategy prioritized pheochromocytoma resection first, given its potential for significant hemodynamic instability. Progression to hysterectomy was contingent upon achieving adequate intraoperative hemodynamic stability following adrenalectomy, with continuous reassessment by the anesthetic and surgical teams. Right adrenalectomy was performed first, followed by total abdominal hysterectomy with bilateral salpingooophorectomy. Significant hemodynamic lability occurred during tumor manipulation, with hypertensive surges managed using sodium nitroprusside and remifentanil infusions. Following adrenal vein ligation and tumor removal, hypotension was managed with noradrenaline and additional adrenaline support as required. Total operative time was approximately 6 hours. Postoperatively, transient noradrenaline support was required but was rapidly weaned as hemodynamic stability was achieved. Conclusion Single-stage adrenalectomy and major pelvic surgery can be safely performed in selected patients with pheochromocytoma when guided by meticulous preoperative optimization, clear intraoperative sequencing, and close multidisciplinary coordination.
Pheochromocytoma ; Adrenalectomy ; Leiomyoma ; Hysterectomy

Introduction: Uterine fibroids are common in reproductive-age women and are associated with obstetric complications. Their potential contribution to maternal metabolic disturbances is poorly described. We report a case of maternal hypoglycemia in the setting of a large uterine fibroid and fetal overgrowth, suggesting a possible endocrine–metabolic interaction. Case: A 39-year-old multiparous female with no history of diabetes or endocrine disease had a large lower-segment uterine fibroid measuring 8 × 10 cm. Pregnancy was otherwise uncomplicated until 39 weeks, when she underwent classical Caesarean section for transverse lie and delivered a large-for-gestational-age infant. Postpartum, she developed recurrent symptomatic hypoglycemia despite normal hemoglobin A1c (5.0%) and preserved thyroid and adrenal function. There was no evidence of sepsis, liver disease, medication exposure, or insulinoma. Hypoglycemia resolved spontaneously following delivery. Postnatal imaging showed persistence of the fibroid (6 × 9 cm). She remains under follow-up, considering interval laparoscopic myomectomy with bilateral tubal ligation. The coexistence of maternal hypoglycemia and fetal overgrowth raises the possibility of altered maternal– fetal glucose dynamics. Large mesenchymal tumors may produce insulin-like growth factor 2 (IGF-2), causing non–islet cell tumor hypoglycemia. Fibroid-related uteroplacental hemodynamic changes or increased fetal glucose demand may also contribute. While causality cannot be established, the temporal resolution post-delivery suggests a contributory role of the fibroid in metabolic disturbance. Conclusion This case highlights a rare but clinically relevant association between a large uterine fibroid, maternal hypoglycemia, and fetal overgrowth. In pregnant patients with unexplained hypoglycemia, uterine fibroids may represent an overlooked factor. Multidisciplinary follow-up and further research into IGF signaling and placental–tumor interactions are warranted.
Diabetes, Gestational ; Fetal Macrosomia ; Leiomyoma ; Hypoglycemia

BACKGROUND

Hysteroscopic myomectomy is regarded as the gold standard in the management of intracavitary myomas. With its increased use, it is essential to explore its effectiveness for better therapeutic planning and patient selection.

This study aimed to determine the effectiveness of hysteroscopic myomectomy performed in a tertiary hospital in the Philippines.

A retrospective cross-sectional study of women who underwent hysteroscopic myomectomy in 5 years was performed.

A total of 167 women were included. Completeness of resection was achieved in 88.46% of the cases. In the final multivariate model, older age, pretreatment with gonadotropin-releasing hormone (GnRH) agonist, and lesser total fluid input were associated with increased odds of complete resection. Submucous myoma at least 3 cm in size had greater total fluid input, greater blood loss, the presence of complications, and a greater need for transfusion. Pretreatment with GnRH agonists had more International Federation of Gynecology and Obstetrics Grade 1 and 2 myoma, higher frequency of ≥3 myomas, lower frequency of complete resection, and increased operative time compared to those without pretreatment with GnRH agonists. Pretreatment with other hormonal therapy had a lesser need for transfusion compared to those without pretreatment with hormonal therapy.

Hysteroscopic myomectomy is a generally safe and effective procedure for the treatment of submucous myomas. The odds of complete resection are greater with older age and decreased with pretreatment with GnRH agonist and more distention fluid used. Larger submucous myoma was associated with greater total fluid input and blood loss, more complications, and greater need for transfusion. Pretreatment with GnRH agonist had no significant benefit and was associated with longer surgical time and lesser complete resection.

Objective: To investigate the clinicopathological features, diagnosis and differential diagnosis of pediatric myofibroma/myofibromatosis of the soft tissue and bone. Methods: All cases of pediatric myofibroma/myofibromatosis of the soft tissue and bone diagnosed between January 2011 and December 2018 were retrieved from the surgical pathology records in the Department of Pathology, Beijing Jishuitan Hospital, Beijing, China. Clinical and radiological data were collected. H&E and immunohistochemistry were used to examine histological and immunophenotypic features and to make the diagnosis and differential diagnosis. The relevant literature was also reviewed. Results: Twenty-eight cases of pediatric myofibroma/myofibromatosis of the soft tissue and bone were respectively collected. The patients' ages ranged from 2 months to 14 years, with a mean age of 7 years. There were 7 females and 21 males. There were 12 cases located in soft tissue, including the finger (n=9), upper arm (n=1) and foot (n=2). There were 14 cases located in the bone of limb, including the femur (n=8), tibia (n=4), clavicle (n=2), fibula (n=2) and radius (n=1). There were 2 cases of myofibromatosis involving multiple bones. Radiology showed lytic lesions in the bone. The proliferation of spindle-shaped myofibroblasts arranged in fascicles with indistinct eosinophilic cytoplasm and bland nuclei, with no pleomorphism and cytological atypia. The characteristic histologic structure was the biphasic nodular growth pattern with cellular and paucicellular regions. The tumors might arrange in a hemangiopericytoma-like pattern. The stroma varied between dense fibrosis and myxoid changes. The reactive new bone formation and inflammatory cell infiltration also existed. Immunohistochemical study showed that the SMA was positive. The surgical resections were performed. One of the patients had tumor recurrence as a result of 11-month follow-up. Conclusions: The pediatric myofibroma/myofibromatosis of the soft tissue and bone is a very rare benign tumor and has a good prognosis. It has a characteristic morphology and its differential diagnosis from other spindle cell tumors could be made with the immunohistochemical analysis.
Child ; Female ; Humans ; Infant ; Male ; Bone and Bones/pathology* ; Diagnosis, Differential ; Leiomyoma ; Myofibroma/diagnosis* ; Myofibromatosis/diagnosis* ; Child, Preschool ; Adolescent

Objective: To investigate the clinicopathologic and molecular characteristics of fumarate hydratase (FH) deficient uterine leiomyoma. Methods: Eighty cases of FH deficient uterine leiomyoma were diagnosed from April 2018 to September 2022 in Department of Pathology, Peking University Third Hospital. Sanger sequencing of FH gene exons (exon 1-10) were performed on tumor tissues and matched non-tumor tissues/peripheral blood for all cases. FH immunohistochemistry were performed in 74 cases; S-(2-succino)-cysteine (2SC) were also detected by immunohistochemistry in five cases. Results: Patients' age ranged from 18 to 54 (36.0±7.5) years, with more than 60% exhibiting clinical symptoms of multiple and large leiomyomas (the median diameter was 70 mm). More than four histologic features, including staghorn vasculature, alveolar-pattern edema, bizarre nuclei, oval nuclei arranged in chains, prominent eosinophilic nucleoli with perinucleolar haloes and eosinophilic intracytoplasmic globules were observed in 98.5% (67/68) patients. The immunohistochemical sensitivity of FH and 2SC were 97.3% and 100%, respectively. Based on the Sanger sequencing results, the cases were divided into germline variant group (31 cases), somatic variant group (29 cases) and no variant group (20 cases). Sixty-nine percent (20/29) of the patients with FH germline variation had clear family history. Conclusions: Clinical features, histological morphology, FH and 2SC immunohistochemistry and Sanger sequencing have their own significance and limitations in differential diagnosis of FH deficient uterine leiomyoma. In clinical practice, the above information should be fully integrated and studied for accurate pathologic diagnosis and selection of patients with FH germline variation.
Female ; Humans ; Adolescent ; Young Adult ; Adult ; Middle Aged ; Fumarate Hydratase/genetics* ; Uterine Neoplasms/pathology* ; Leiomyoma/pathology* ; Germ-Line Mutation ; Diagnosis, Differential ; Leiomyomatosis/pathology* ; Carcinoma, Renal Cell/diagnosis*

Objective: To investigate the clinicopathological features, diagnosis and differential diagnosis of breast myofibroblastoma. Methods: The clinicopathological data and prognostic information of 15 patients with breast myofibroblastoma diagnosed at the Department of Pathology of the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China from 2014 to 2022 were collected. Their clinical characteristics, histological subtypes, immunophenotypes and molecular characteristics were analyzed. Results: There were 12 female and 3 male patients, ranging in age from 18 to 78 years, with a median and average age of 52 years. There were 6 cases in the left breast and 9 cases in the right breast, including 12 cases in outer upper quadrant, 2 cases in inner upper quadrant and 1 case in outer lower quadrant. Most of the cases showed a well-defined nodule grossly, including pushing growth under the microscope in 13 cases, being completely separated from the surrounding breast tissue in 1 case, and infiltrating growth in 1 case. Among them, 12 cases were classic subtype and composed of occasional spindle cells with varying intervals of collagen fiber bundles; eight cases had a small amount of fat; one case had focal cartilage differentiation; one case was epithelioid subtype, in which epithelioid tumor cells were scattered in single filing or small clusters; one case was schwannoma-like subtype, and the tumor cells were arranged in a significant palisade shape, resembling schwannoma, and one case was invasive leiomyoma-like subtype, in which the tumor cells had eosinophilic cytoplasm and were arranged in bundles, and infiltrating into the surrounding mammary lobules like leiomyoma. Immunohistochemical studies showed that the tumor cells expressed desmin (14/15) and CD34 (14/15), as well as ER (15/15) and PR (15/15). Three cases with histologic subtypes of epithelioid subtype, schwannoma-like subtype and infiltrating leiomyoma-like subtype showed RB1 negative immunohistochemistry. Then FISH was performed to detect RB1/13q14 gene deletion, and identified RB1 gene deletion in all three cases. Fifteen cases were followed up for 2-100 months, and no recurrence was noted. Conclusions: Myofibroblastoma is a rare benign mesenchymal tumor of the breast. In addition to the classic type, there are many histological variants, among which the epithelioid subtype is easily confused with invasive lobular carcinoma. The schwannoma-like subtype is similar to schwannoma, while the invasive subtype is easily misdiagnosed as fibromatosis-like or spindle cell metaplastic carcinoma. Therefore, it is important to recognize the various histological subtypes and clinicopathological features of the tumor for making correct pathological diagnosis and rational clinical treatment.
Female ; Humans ; Male ; Antigens, CD34 ; Biomarkers, Tumor/analysis* ; Leiomyoma/pathology* ; Neoplasms, Muscle Tissue/pathology* ; Neurilemmoma ; Adolescent ; Young Adult ; Adult ; Middle Aged ; Aged

Benign mesenchymal tumors of the urinary bladder are extremely rare. This is a case of a 56- year old female presenting with an enlarging abdomen initially managed as a case of an ovarian new growth. Aside from the ruptured ovarian mass, a urinary bladder mass was also noted intraoperatively and a surgical dilemma was resolved by imaging review. The patient underwent exploratory laparotomy, enterolysis, total hysterectomy, bilateral salpingooophorectomy, and partial cystectomy. Final histopathology identified the bladder tumor to be a leiomyoma. The patient had an uneventful postoperative course. Awareness of this clinical entity and presentation will aid in diagnosis and management.
Leiomyoma

We report a case of giant hysteromyoma and complex pelvic adhesion treated by robotic assisted laparoscopic total hysterectomy and bilateral salpingectomy. The patient was diagnosed with uterine fibroids after physical examination in 1998 but did not receive any treatment, and regular examinations reported progressive growth of the fibroids. Ultrasound suggested multiple uterine fibroids, and pelvic MRI indicated large uterine fibroids with bleeding. Robot-assisted laparoscopic total hysterectomy and bilateral salpingectomy were performed after relevant examinations, and the operation was completed smoothly. The patient was discharged 4 days after surgery with good appearance of the abdominal wall and good recovery during the follow-up. With its unique advantages, robot-assisted laparoscopy provides a minimally invasive surgical approach for giant hysterectomy with complex pelvic adhesions.
Female ; Humans ; Hysterectomy ; Laparoscopy ; Leiomyoma/surgery* ; Robotics ; Uterus

To explore the value of quantitative perfusion histogram parameters of dynamic contrast-enhanced magnetic resonance imaging （DCE-MRI） in pathological classification of uterine leiomyoma and its correlation with Ki-67 protein expression. Thirty five patients with uterine leiomyoma confirmed by operation and pathology at Shaoxing People's Hospital from October 2015 to September 2017 were analyzed retrospectively，including 15 cases of ordinary type，8 cases of cellular type and 12 cases of degenerative type. All patients were examined by pelvic DCE-MRI before operation，and the histogram parameters （median，mean，skewness，kurtosis，energy，entropy） of various quantitative perfusion parameters，including volume transport constant （K），rate constant （K），extravascular extracellular space distribute volume per unit tissue volume （V），blood plasma volume per unit volume of tissue （V） were calculated，and the efficacy of different parameters in pathological classification of uterine leiomyoma was evaluated by ROC curve. The expression of Ki-67 protein in uterine leiomyoma was detected by immunohistochemical method，and the correlation between histogram parameters and Ki-67 protein expression was analyzed by Pearson and Spearman correlation analysis. The median and mean values of K，K，V and V in the cellular group were higher than those in the degenerative group and the ordinary group（<0.05 or <0.01），while the skewness of V，the skewness and kurtosis of K in the cellular group were lower than those in the ordinary group （all <0.05）. The entropy of K in the cellular group was higher than that in the degenerative group and the ordinary group （all < 0.05）. The entropy of V in the cellular group was higher than that in the ordinary group （<0.01）. The median，mean，skewness of K，median and mean of K，median and mean of V，median，mean，energy and entropy of V were correlated with Ki-67 expression（all <0.05）. The results of ROC curve analysis showed that the median threshold of K was 0.994/min，the sensitivity and specificity for the diagnosis of cellular uterine leiomyoma were 100.0% and 77.8% respectively，and the area under the ROC curve was 0.949. When the mean threshold of K was 1.170/min，the sensitivity and specificity for diagnosing cellular uterine leiomyoma were 100.0% and 77.8% respectively，and the area under the ROC curve was 0.958. The area under the ROC curve of K （entropy），K （median，mean），V （median，mean，entropy） in the diagnosis of cellular uterine leiomyoma were 0.755-0.907. :DCE-MRI quantitative perfusion histogram parameters have high diagnostic value in differentiating pathological types of uterine leiomyoma，especially for cellular uterine leiomyoma.
Contrast Media ; Humans ; Leiomyoma/diagnostic imaging* ; Magnetic Resonance Imaging ; Perfusion ; Retrospective Studies

Objective To explore the risk factors for incident endometrial injury and 3-month endometrial injury after magnetic resonance-guided focused ultrasound(MRgFUS)ablation of uterine fibroids(UF). Methods UF patients who were diagnosed in Peking Union Medical College Hospital and underwent MRgFUS ablation in Amcare Women's and Children's Hospital from August 2016 to October 2020 were retrospectively enrolled in this study.Clinical data of 66 UF patients were collected and compared between endometrial injury group and non-injury group.Stepwise regression was employed to determine the risk factors for the incident endometrial injury and 3-month endometrial injury.Multivariate logistic regression analysis was performed to explore the relationship of endometrial injury with age,pre-ablation tumor size,multiple UF,International Federation of Gynecology and Obstetrics(FIGO)classification,T2WI signal intensity,and treatment time.Results In terms of incident endometrial injury,the 66 patients included 41(62.1%)cases with no injury,4 cases(6.1%) with grade 1 injury,5 cases(7.6%)with grade 2 injury,and 16 cases(24.2%)with grade 3 injury.In terms of 3-month endometrial injury,the 66 patients included 49 cases(74.2%)with no injury,5 cases(7.6%)with grade 1 injury,2 cases(3.0%)with grade 2 injury,and 10 cases(15.2%)with grade 3 injury.Stepwise regression analysis indicated that FIGO classification was significantly associated with incident endometrial injury(
Child ; Female ; Humans ; Infant ; Leiomyoma ; Magnetic Resonance Imaging ; Magnetic Resonance Spectroscopy ; Retrospective Studies ; Treatment Outcome ; Uterine Neoplasms