Alpha-fetoprotein producing endometrioid carcinoma arising in an adenomyoma of the uterus.

We report a case of alpha-fetoprotein-producing endometrioid carcinoma (AFP-EC) that originated within an adenomyoma of the uterine corpus. A 76-year-old Japanese woman was incidentally discovered to have a uterine tumor along with multiple lung nodules. Upon surgical removal of the uterus, it was revealed that the tumor was situated within the adenomyoma. The tumor exhibited microfollicular structures and solid growth patterns, with hyaline globules, clear cell glands, and primitive tumor cells. Immunohistochemical analysis indicated the presence of germ cell markers, including AFP, SALL4, and glypican3, leading to final diagnosis of AFP-EC. Histopathologically, AFP-ECs exhibit characteristics similar to those of AFP-producing neoplasms in other organs. Furthermore, a nomenclature issue arises when distinguishing AFP-ECs from yolk sac tumors of the endometrium in older patients due to their shared features. The concept of retrodifferentiation or neometaplasia suggests that "endometrioid carcinoma with yolk sac tumor differentiation" or "endometrioid carcinoma with a primitive phenotype" may serve as more fitting terms for the diverse spectrum of AFP-producing neoplasms in the endometrium. In conclusion, this case underscores the diagnostic challenges posed by AFP-ECs arising from adenomyomas and emphasizes the need for refining the nomenclature and classification of AFP-producing neoplasms within the endometrium.

Clinical analysis of 44 cases of atypical polypoid adenomyoma of the uterus.

BACKGROUND: Atypical polypoid adenomyoma (APA) is a rare intrauterine polypoid lesion that occurs predominantly in premenopausal women. Although APA was previously considered a benign lesion and treated conservatively, an increasing number of cases show that APA has a high rate of recurrence or residual disease and that it precedes the development of carcinoma. The clinical management of APA remains to be established. The aim of this study was to analyse the clinicopathological features of APA and discuss its diagnosis and prognosis. METHODS: Forty-four patients with APA were admitted to Beijing Obstetrics and Gynecology Hospital from 2005 to 2019, and their clinical and histopathologic features were evaluated. B-ultrasound was performed, and all the patients (n = 44) underwent hysteroscopy. Endometrium excision was performed by means of the four-step diagnosis and treatment method. Hysteroscopic transcervical resection (TCR) was performed in 5 patients with APA-H and 11 with APA-L. Except for one patient who underwent transcervical endometrial resection, all the patients underwent hysterectomy and salpingectomy or salpingo-oophorectomy. Data from a median follow-up of 42 months (ranging from 3 to 174 months) were available for these patients. RESULTS: Pathological diagnosis were made according to the degree of abnormality of the APA surface glands, resulting in APA-L in 36 patients and APA-H in 8 patients. Among these patients, 28 (25 APA-L and 3 APA-H) were treated conservatively. The effect of the four-step diagnosis and treatment method as an APA therapy was excellent. During the follow-up, no evidence of recurrence was found. CONCLUSIONS: For patients with intracavitary lesions > 1 cm, the hysteroscopic four-step diagnosis and treatment method and pathological diagnosis are the basis of clinical treatment. More than 30% of APA surface glands have complex structures characterized by branching and budding or other high-risk factors, such as endometrial hyperplasia, which are indications for hysterectomy. For patients who desire to become pregnant or to preserve the uterus, hysteroscopy with complete excision of the lesions should be the preferred treatment method. The patients should be treated and followed up closely with regular hysteroscopy and endometrial biopsy.

MRI and Transvaginal Ultrasound Findings of Atypical Polypoid Adenomyoma: A Case Report.

Atypical polypoid adenomyoma (APA) is an uncommon type of polypoid characterized by fibroid stroma and endometrial glands. It occurs mostly in premenopausal women and rarely in postmenopausal women with irregular vaginal bleeding. In our current case, a 76-year-old woman presented with irregular vaginal bleeding. The final pathological diagnosis of the mass was APA. APA is not easy to diagnose before surgery. On the one hand, there was no obvious particularity in imaging features and clinical features, especially for uncomfortably identifying endometrial cancer. On the other hand, APA has a pedicle, attaching to any part of the uterine cavity, which can cause pseudocoel between the mass with the uterine cavity wall. So, when it comes to getting the pathological tissue in the absence of hysteroscopy, it is easy to access to the pseudocoel and obtain endometrial tissue rather than the pathological tissue of the mass. Therefore, preoperative imaging examination is of great significance diagnosis way of thinking to clinicians for APA. In the meantime, pathological tissue of APA can be obtained by hysteroscopy in visual conditions.

Patterns of SATB2 and p16 reactivity aid in the distinction of atypical polypoid adenomyoma from myoinvasive endometrioid carcinoma and benign adenomyomatous polyp on endometrial sampling.

AIMS: Atypical polypoid adenomyoma (APAM) is an uncommon uterine lesion composed of complex endometrioid glands with frequent squamous morular metaplasia and fibromuscular stroma. On endometrial curettage, biopsy or polypectomy specimens, the admixture of endometrioid glands and smooth muscle raises the differential diagnosis of myoinvasive endometrioid carcinoma. Reproductive-age APAM patients may opt for fertility preservation, whereas myoinvasive carcinoma is treated surgically. One previous study reported an incidental finding that the stroma of APAM, in contrast to that of other polypoid lesions, was SATB2-positive. APAM has also been reported to show increased stromal p16 staining. We aimed to assess whether SATB2 and p16 are useful stains for the distinction of APAM from myoinvasive carcinoma and benign adenomyomatous polyps. METHODS AND RESULTS: Cases of 'atypical polypoid adenomyoma' (n = 32), 'adenomyomatous polyp' (n = 39) and 'myoinvasive endometrioid carcinoma' (n = 30) were identified. Morphological features were assessed, along with the intensity and extent of SATB2 and p16 staining in the stromal component of each lesion. SATB2 expression was seen in the stromal components of 30 of 32 (94%) APAMs, versus none of 39 (0%) benign adenomyomatous polyps and five of 30 (17%) myoinvasive endometrioid carcinomas. Stromal p16 expression was seen in 31 of 31 (100%) APAMs, versus 20 of 39 (51%) benign adenomyomatous polyps and 12 of 30 (40%) myoinvasive endometrioid carcinomas. CONCLUSIONS: Patchy to diffuse SATB2 and block-type p16 staining of fibromuscular stroma separating atypical endometrioid glands is more consistent with APAM than with myoinvasive endometrioid carcinoma. These stains are potentially useful adjuncts to careful morphological evaluation of endometrial biopsies/curettings.

Imaging in gynecological disease (21): clinical and ultrasound characteristics of accessory cavitated uterine malformations.

OBJECTIVE: To describe the clinical and ultrasound characteristics of accessory cavitated uterine malformations (ACUMs). METHODS: This was a single-center observational study of consecutive patients diagnosed with an ACUM, who had undergone an ultrasound examination by an experienced ultrasound examiner between January 2013 and May 2019, identified retrospectively from medical records. ACUM was diagnosed when a cavitated lesion with a myometrial mantle and echogenic contents was seen within the anterolateral wall of the myometrium beneath the insertion of the round ligament. In all women, presenting symptoms and clinical history were recorded along with detailed descriptions of the lesions and any concomitant pelvic abnormalities. RESULTS: Twenty patients diagnosed with an ACUM were identified. Median age was 29.2 (interquartile range, 25.0-35.8) years. None of the women was premenarchal or postmenopausal. All of the women reported painful periods or pelvic pain and none of them reported subfertility. Twelve of the ACUMs were in the right anterolateral myometrium and eight were in the left anterolateral myometrium. Both a myometrial mantle and a fluid-filled cavity were considered to be defining features on ultrasound. The fluid contained within the cavity was either echogenic with a ground-glass appearance or hyperechoic. All of the lesions were spherical in shape. The Doppler flow seen in the outer rim was not markedly different from that of the surrounding myometrium, and the content of the cavity was avascular on Doppler examination. The mean outer cavity diameter of the ACUMs was 22.8 (95% CI, 20.9-24.8) mm and the mean internal cavity diameter was 14.1 (95% CI, 12.2-16.1) mm. Four women opted for transvaginal ultrasound-guided alcohol sclerotherapy. Surgical excision was carried out in eight cases, and the diagnosis was confirmed on histopathological examination in all of them. CONCLUSIONS: ACUMs are a uterine abnormality with a distinct ultrasound appearance, which are associated with dysmenorrhea and chronic pelvic pain. Knowledge of their typical appearance on ultrasound could facilitate early detection and treatment. There are several treatment options for ACUM, ranging from simple analgesia to complete excision. Further prospective and longitudinal studies are required to study the prevalence and natural history of this condition. © 2020 International Society of Ultrasound in Obstetrics and Gynecology.

Late presentation of extrauterine adenomyomas after laparoscopic morcellation at hysterectomy: a case report.

BACKGROUND: An adenomyoma is a well circumscribed form of adenomyosis and can be located within the myometrium, in the endometrium as a polyp, or extrauterine with the last being the rarest presentation amongst the three. With the ongoing advancement in gynecological surgery, the use of electromechanical morcellators have made the removal of large and dense specimens possible with minimally invasive techniques. However, it has also caused an increase in complications which were previously rare. Whilst the tissue is being grinded within the abdominal cavity, residual tissue can spread and remain inside, allowing for implantation to occur and thereby giving rise to recurrence of uterine tissue as a new late postoperative complication. Case presentation A 45-year-old woman presented with worsening constipation and right iliac fossa pain. Her past surgical history consists of laparoscopic supra-cervical hysterectomy that was indicated due to uterine fibroids. Computerized tomography and magnetic resonance imaging were done, which showed an irregular lobulated heterogeneous mass seen in the presacral space to the right, located on the right lateral aspect of the recto-sigmoid, measuring 4.5 × 4.3 × 4.3 cm in size. A transvaginal ultrasound revealed a cyst in the left ovary. The patient had a treatment course over several months that included Dienogest (progestin) and Goserelin (GnRH analogue) with add-back therapy. In line with the declining response to medications, the patient was advised for a laparoscopic ovarian cystectomy. During the surgery, an additional lesion was found as a suspected fibroid and the left ovarian cyst was identified as pockets of peritoneal fluid which was sent for cytology. The surgical pathology report confirmed adenomyosis in both specimens, namely the right mass and the initially suspected fibroid. CONCLUSION: In this case report, we showcase a rare occurrence of an extrauterine adenomyoma presenting two years post laparoscopic morcellation at hysterectomy. This poses questions regarding the benefits versus risks of power morcellation in laparoscopic hysterectomy.

Morphological and immunophenotypical analysis of the spindle cell component in adenomyomatous hyperplasia of the gallbladder.

BACKGROUND: Adenomyomatous hyperplasia (AMH) of the gallbladder, reported in 1-8.7% of cholecystectomies, consists of cystically dilated sinuses/glands with a surrounding spindle cell proliferation which is thought to be composed of smooth muscle cells. Myofibroblasts are contractile cells that secrete a variety of biochemical modulators causing a "field-effect". Myofibroblasts can be immunohistochemically distinguished from smooth muscle cells by their desmin negativity. METHODS: Eighteen cases of AMH and five cases each of chronic follicular cholecystitis, chronic cholecystitis, gallbladder carcinoma and 10 colonic diverticular disease were stained with actin and desmin. The percentage of myofibroblasts was estimated by the difference between actin and desmin staining in the same field. Statistical anlysis was performed using SPSS 22.0. RESULTS: The percentage of actin staining was significantly higher in AMH and gallbladder carcinoma compared to chronic follicular and chronic cholecystitis (p = 0.04). The percentage of desmin staining did not show any significant difference between the four groups. The estimated myofibroblastic population was significantly higher in AMH when compared to chronic follicular and chronic cholecystitis (p = 0.005). CONCLUSION: The spindle cell proliferation around cystically dilated glands in AMH is composed predominantly of myofibroblasts and of smooth muscle cells as previously described. This finding suggest a derangement in epithelial-stromal interactions as the underlying pathophysiology in AMH.

Lesion-targeted cytology to improve cytological sampling for atypical polypoid adenomyomas of the uterus: A case series and review of the literature.

OBJECTIVE: Atypical polypoid adenomyomas (APAs) are uncommon tumours consisting of atypical endometrioid glands and fibromyomatous stroma. Identifying the biphasic nature of atypical glandular components and spindle mesenchymal components without atypia is crucial for the cytological diagnosis of APA. We investigated the utility of lesion-targeted cytology (LTC) to directly collect firm spindle components. METHODS: We recruited seven consecutive surgical patients who underwent cytological examinations before surgery and were diagnosed with APA on postoperative histological examinations. Cytological smears were obtained by routine sampling in five cases and by targeted sampling using transvaginal ultrasonography, that is, LTC, in two cases. We retrospectively analysed the cytological findings from our cases and compared them to those of APA cases previously reported in the English literature. RESULTS: Among 5/7 cases that involved routine cytological sampling, normal cytological findings were found in 2 and atypical glandular cells were found in 3, but spindle cells from mesenchymal components were not detected. In contrast, among 2/7 cases in which sampling involved LTC, spindle cells without atypia, in addition to atypical glandular cells were found. CONCLUSIONS: Lesion-targeted cytology is useful to assess mesenchymal components of APAs and may improve the cytological diagnosis of APA.

Atypical polypoid adenomyoma of the endometrium: diagnosis and treatment. A case report.

Atypical polypoid adenomyoma (APA) is a rare uterine lesion that commonly recurs after local excision and is occasionally associated with or anticipates the development of atypical hyperplasia or endometrioid adenocarcinoma. We report a case of a 45-year-old woman affected by APA treated with local resection.

Gastric Adenomyosis: A Rare Cause of Pyloric Mass in Children.

Adenomyoma of the stomach is a benign tumor with a very low incidence. Clinical presentation and imaging modalities are usually nonspecific and variable. A rare case of gastric adenomyoma in a 12-year-old child is being reported who presented with gastric outlet obstruction. The diagnosis could only be established after an excision biopsy performed after multiple diagnostic modalities failed to clinch the diagnosis. The case is being reported in view of the rarity of this entity in the pediatric age group as a cause of gastric outlet obstruction.

Uncommon Mixed Endometrial-Myometrial Benign Tumors of the Reproductive Tract: Literature Review.

BACKGROUND: We reviewed published cases of uterus-like mass (U-LM), endomyometriosis, polypoid adenomyoma (PA), adenomyomatous polyp, atypical PA (APA), and adenomyoma. SEARCH METHODS: PubMed, Medline, and Scopus searches of all cases published in the databases till November 26, 2018. RESULTS: We identified 45 case reports of U-LM in the pelvis, 10 cases of endomyometriosis, 44 cases of adenomyomatous polyp, 466 cases of APA and case series of adenomyoma and PA. Most case reports focused on histological description of removed lesions with no or very limited clinical correlates. Histological descriptions were often used interchangeably, which creates considerable confusion. It is unclear if endomyometriosis warrants inclusions as a distinct entity, since the distinction is blurred between adenomyomatous polyp and PA. The glandular epithelium in atypical polypoid adenomyoma exhibits atypia and the lesions have a tendency to recur with a risk of malignant transformation. Smooth muscle metaplasia and Müllerian fusion defects have been proposed as etiology, but it is possible that the lesions do not share a common origin. CONCLUSION: There is need for more detailed and structured description of reported cases including clinical presentation and associated pathology.

Successful laparotomy tumor resection and levonorgestrel-releasing intrauterine system for atypical polypoid adenomyoma.

Hysteroscopic transcervical resection (TCR) is often performed as fertility sparing treatment for atypical polypoid adenomyoma (APA) patients. However, TCR has the risk of uterine wall perforation, especially when the tumor extends deeply into the uterine muscle layer. We report an APA patient in whom it was impossible to completely resect the tumor by TCR, but laparotomy tumor resection followed by levonorgestrel-releasing intrauterine system (LNG-IUS) was successful. The patient was a 35-year-old nulligravida woman. We performed laparotomy tumor resection and inserted the LNG-IUS into uterine cavity just after surgery. Microscopic residual tumor was suspected based on histopathological findings. However, the patient has not relapsed for 26 months, even though the LNG-IUS was removed after 6 months. Laparotomy tumor resection may be one fertility sparing treatment option for APA patients. Furthermore, it may be effective to use the LNG-IUS after surgery for two purposes that are adhesion prevention and tumor disappearance.

Sonographic diagnosis of an ileal adenomyoma in a neonate.

An ileal adenomyoma leading to intussusception is very rare. We describe the case of a 4-month-old boy who presented with hematochezia. He was diagnosed with ileal adenomyoma by sonography, which was confirmed by histopathology. This case confirms that sonography is the preferred modality to diagnose an ileal adenomyoma.

[Feasibility and safety of MRgFUS ablation for uterine fibroids and adenomyosis: a preliminary study].

Objective: To investigate the feasibility and safety of magnetic resonance guided focused ultrasound surgery (MRgFUS) ablation treatment for uterine fibroids and adenomyosis. Methods: From February 2017 to July 2018, a total of 61 women in Foshan Hospital of Traditional Chinese Medicine with uterine fibroids or adenomyosis (36 cases of fibroids and 25 cases of adenomyosis) were included for treatment of MRgFUS, mean age was 27-48 (39±5) years. The treatment status, treatment effect and complications were recorded, and the differences between myoma and adenomyosis were compared. Results: Thirty-two (88.9%) patients of fibroid group and twenty-one (84.0%) patients of adenomyosis group were completed MRgFUS treatment respectively (P>0.05). The spot energy of adenomyosis group was 1 039-5 698(2 852±991) J, which was higher than 600-6 466(2 485±1 137) J of fibroid group (P<0.01). There was no significant statistical difference in mean temperature of spot and ablation time between the two groups (P>0.05).The non-perfusion volume ratio (NPVR) of the fibroid and adenomyosis group was 54%-99%(84%±15%) and 60%-98%(82%±12%) and there was no significant statistical difference (P>0.05), but the ablation efficiencies of adenomyosis group was less than fibroid group (0.8-4.3(2.1±0.9) cm(3)/min vs 1.3-7.8(3.6±1.5)cm(3)/min, P<0.01).The incidence of complications of adenomyosis group was 47.6%(10/21), it was higher than fibroid group 18.8%(6/32) (P<0.05). Conclusions: MRgFUS is a non-invasive, safe and effective treatment for both uterine fibroids and adenomyosis. Compared with uterine fibroids, MRgFUS treatment of adenomyosis has some disadvantages such as higher energy, lower ablation efficiency and more adverse reactions, and further optimization is needed.

Adenomyomas of the Uterine Cervix in the First-Trimester of Pregnancy: A Case Report.

Cervical adenomyomas of endocervical type (endocervical adenomyomas) are very rare benign lesions. Here we report the case of a 33-year-old woman who referred to the Perinatology Clinic of Ommolbanin Hospital (Mashhad, Iran) in September 2017. The patient was 8 weeks pregnant and complained of spotting and feeling a mass protruding from her vagina for 2 months. Physical examination revealed the presence of three masses of approximately 10 cm in the vagina, which were treated surgically. Histopathological examination of the excised specimen showed the presence of glands lined by a single layer of endocervical-type mucinous epithelium with smooth muscle fibers. Clinicians should be aware of such lesions in order to differentiate them from other malignancies and to individualize treatment.

CT diagnosis of gallbladder adenomyomatosis: importance of enhancing mucosal epithelium, the "cotton ball sign".

OBJECTIVES: To determine the diagnostic value of the cotton ball sign and other CT features in patients with gallbladder (GB) wall thickenings (WTs). METHODS: Three blinded readers reviewed the preoperative CT and MR images of 101 patients with pathologically confirmed GB adenomyomatosis (GA) (n = 34) and other benign (n = 29), malignant (n = 41), and premalignant (n = 2) GBWTs. Three readers analysed the morphological features of GBWT and presence of the "cotton ball sign", defined as fuzzy grey dots in GBWT or a dotted outer border of the inner enhancing layer on contrast-enhanced (CE) CT. In addition, the "pearl necklace sign" on MR was analysed. RESULTS: In the GA group (n = 34), prevalence of the cotton ball sign and pearl necklace sign was 74% (25/34) and 44% (15/34), respectively. Presence of the cotton ball sign, smooth contour of the mucosa, double-layering enhancement, and enhancement degree weaker than the renal cortex on CT images were significant predictors of benign GBWT (p < 0.01). When differentiating GA from GB malignancy or premalignancy, accuracy of the cotton ball sign and pearl necklace sign was 81% (62/77) and 74% (57/77), respectively. CONCLUSION: The cotton ball sign on CE-CT showed higher sensitivity and comparable specificity to those of the pearl necklace sign in differentiating GA from malignancy. KEY POINTS: • Prevalence of the cotton ball sign on CT was 74% in gallbladder adenomyomatosis. • The cotton ball sign was useful in differentiating gallbladder adenomyomatosis from gallbladder cancer. • The cotton ball sign was more sensitive than the pearl necklace sign for adenomyomatosis diagnosis.

[Atypical polypoid adenomyoma of the uterus: a clinicopathological review of 27 cases].

Objective: To investigate the clinical and pathological characteristics of atypical polypoid adenomyoma (APA) for improvement of the diagnosis, different diagnosis and treatment of the disease. Methods: The clinical data, pathological characteristics, and the follow-up information were retrospectively analyzed in 27 cases of APA admitted in Peking Univeristy People's Hospital from 2007 to 2016. Results: The median age was 42.6 years old (range 25-60 years old). Fifteen patients were nullipara, 2 patients were postmenopausal. The most common presenting symptom was abnormal uterine bleeding (81%, 22/27) . Leisions were obtained by using hysteroscopy in 23 cases, hysterectomy 3 cases and dilatation and curettage 1 case. Fertility preserving treatments were performed in 10 patients who had strong desire for fertility, among which 1 case progressed into endometrial carcinoma. Among 15 patients underwent hysterectomy and (or) bilateral salpingo-oophorectomy, 9 cases of them had endometrial atypical hyperplasia. Endometrial carcinoma along with APA were found in three patients, 2 cases of them underwent hysterectomy and bilateral salpingo-oophorectomy and pelvic lymphadenectomy, the other one received medication for fertility preservation. Follow up information were available in 24 cases (89%, 24/27) with a median follow up of 46 months (range 4-108 months), 1 case recurred and 1 case progressed into endometrial carcinoma. One case died of other malignancy, while the other patients were alive. Conclusions: APA is a rare uterine neoplasm mixed with epithelial and mesenchymal component. It occurs mostly in childbearing-age women and its diagnosis is dependent on pathology. Although it's clinical course is benign, there is risk of co-existance of endometrial carcinoma and endometrial atypical hyperplasia. For those who has desire of fertility, the treatment strategy is completely removed the lesion and closely followed up. For those who do not desire to preserve fertility, hysterectomy may be an option.

Endocervical Atypical Polypoid Adenomyoma.

Atypical polypoid adenomyomas (APAMs) are rare uterine tumors that occur predominantly in premenopausal women, with less than 250 cases reported so far, worldwide. They may recur after treatment, and they may coexist with, or precede development of an endometrial adenocarcinoma. For this reason cases managed with conservative surgery or medical therapies require long-term follow-up. We report the case of a 41 years old nulliparous patient who during a diagnostic hysteroscopy was found with an endocervical atypical polypoid adenomyoma (APAM). The patient was desirous of a pregnancy, reported menometrorrhagia, and had a coexistent 5 cm, grade 2, submucous myoma, 3 endometrial polyps, and diffuse adenomyosis. She was treated with hysteroscopic resection of the APAM and polyps, plus laparoscopic myomectomy and wedge resection of adenomyosis. She is on an IVF list and after 4 months she is symptoms-free.

Systematic review shows that pathological lead points are important and frequent in intussusception and are not limited to infants.

Intussusception is the most clinically relevant cause of bowel obstruction in infancy and can be idiopathic or occur as a result of pathological lead points. The incidence of these pathological lead points varies from 0.3 to 20%, and they can be mucosal, intramural or extrinsic structures. A systematic literature review was performed from 1998 to 2016 to evaluate the incidence and types of pathological lead points in paediatric intussusception, and this identified 31 epidemiological and retrospective case cohort studies, reviews and case reports. CONCLUSION: Pathological lead points were frequent in intussusceptions and not limited to infants.