Ileocolic Intussusception in Infancy is not Always Idiopathic - Adenomyoma as the Leading Point.

BACKGROUND Intussusception is a frequent abdominal emergency in infancy, requiring immediate diagnosis and therapeutic intervention. In approximately 90% of cases, intussusception seems to be idiopathic. There has been a reported association of intussusception with lymphoid hyperplasia of Peyer patches in the terminal ileum, possibly acting as the triggering factor. Clinical presentation varies substantially, while the etiology seems idiopathic in most reported cases. CASE REPORT This case describes a previously healthy 2-month-old girl who presented with an episode of non-bilious vomiting and deterioration during the 12 hours preceding the visit. Abdominal ultrasonography revealed the typical target sign in the right iliac fossa, without visible peristalsis, confirming the diagnosis of intussusception. Failure of non-surgical reduction led to emergency laparotomy with the working diagnosis of intussusception due to Meckel's diverticulum. However, laparotomy instead revealed an adenomyoma of the small intestine, a rare benign tumor-like lesion, as the pathological lead point. CONCLUSIONS This case is interesting for 2 distinct reasons. Not only does it underline the need to maintain a high index of suspicion for triggering factors, even in patients within atypically affected age groups, but it also adds to the remarkably limited selection of reported adenomyomas of the small intestine acting as the pathological lead point for intussusception. In this case report, we aspire to emphasize that especially in patients outside the most affected age group, pediatric surgeons should remain aware of the possibility of adenomyoma as a pathological lead point.

Ultrasound diagnosis of the small intestinal adenomyoma in children.

AIM: Adenomyoma is an exceptionally rare hamartoma in the small intestine. Few data have been reported on the features of this rare disease. The aim of this study was to describe the ultrasound (US) characteristics of small intestinal adenomyomas. Material and methods: This retrospective study analyzed the clinical features and US data of 15 pediatric patients diagnosed as small intestinal adenomyomas in the age range between 1 day to 12 years in our hospital during 2014-2021. RESULTS: The clinical manifestations of all the small intestinal adenomyomas were abdominal pain, vomiting or/and hemafecia. The small intestinal adenomyoma usually acted as the lead point of secondary intussusception. They were identified in the ileum (n=11), jejunum (n=2), and Meckel's diverticulum (n=2). The diagnostic accuracy (the concordance rate between US diagnosis and pathological diagnosis) of small intestinal adenomyoma was 73.3%. The small intestinal adenomyoma had approximately 1.0-3.0 cm, were typically located in the submucosal region, had the basal part wide and without a pedicle, and its boundaries were clear. The mass protruded into the intestinal cavity, and showed oval hypoechoic polycystic echo nodules, containing multiple small quasi-circular or irregular cysts of different sizes surrounded by solid hypoechoic mosaic areas. The color Doppler US showed in the solid hypoechoic areas of the mass abundant or sparse blood flow signals.Conclusions The US findings of small intestinal adenomyomas in children are characteristic, and US is valuable in the identification of intestinal adenomyomas in children.

Laparoscopic intervention for solid pelvic tumours in Mayer-Rokitansky-Küster-Hauser syndrome: a case of bilateral uterine adenomyomas of the rudimentary uterus.

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, a condition caused by Müllerian anomalies, is characterised by congenital vaginal aplasia and a rudimentary uterus. Case reports concerning uterine fibroids associated with MRKH syndrome are limited, and differentiating between uterine fibroids and ovarian solid tumours prior to surgical intervention is often challenging. Here, we present the case of a patient with MRKH syndrome and asymptomatic bilateral pelvic solid tumours located close to both ovaries. Based on intraoperative and histopathological findings, the tumours were diagnosed as adenomyomas of the rudimentary uterus. This is the first reported case of a uterine adenomyoma associated with MRKH syndrome. Moreover, our report highlights the fact that diagnostic laparoscopy is a valuable method to evaluate pelvic tumours in MRKH syndrome.

Uterine Adenosarcoma Originating in Adenomyosis: Report of an Extremely Rare Phenomenon and Review of Published Literature.

Müllerian adenosarcoma is an uncommon biphasic malignant tumor most often occurring in the uterine corpus and derived from native surface endometrium. We report a case of intramural uterine adenosarcoma arising in association with adenomyosis, in the absence of tumor involving the surface endometrium. This is an extremely rare phenomenon, with only 8 other published cases of uterine corpus adenosarcoma in the absence of surface endometrial involvement, 5 originating in adenomyosis and 3 in adenomyomas. We review these cases. The current FIGO staging system for uterine adenosarcoma assumes origin from the surface endometrium and does not address the rare occurrence of intramural tumors without a surface endometrial component. Such tumors are problematic to stage and could potentially be overtreated, particularly if there is deep myometrial involvement.

Identification of Juvenile Cystic Adenomyoma Using High-Resolution Imaging.

BACKGROUND: Juvenile cystic adenomyoma is a rare condition that is often misdiagnosed as a noncommunicating uterine horn or adnexal mass during adolescence. CASES: We describe two patients who presented with dysmenorrhea unresponsive to standard management with oral contraceptives. Both patients were initially misdiagnosed as having endometriotic cysts. Juvenile cystic adenomyoma was suspected on standard pelvic ultrasound scan and subsequent high-resolution three-dimensional ultrasonography. The diagnosis was subsequently confirmed and the lesions successfully treated laparoscopically. CONCLUSION: Gynecologists should be aware of the possibility of juvenile cystic adenomyoma in adolescents with dysmenorrhea refractory to medical management. Three-dimensional ultrasonography may provide the resolution necessary to distinguish this rare condition.

Immunohistochemical results and case report of an incidental finding of uterine polypoid adenomyoma after long-time therapy for metrorrhagia.

We present a 25-year-old female woman with a 9-year history of metrorrhagia, in whom a uterine polypoid adenomyoma (UPA) was incidentally detected. Intense nuclear staining in the uterine adenomyoma tissue showed an immunoreaction with BAF250a/ARID1A, Arginase-2 as well as 1LRH-2E1/NR5A2, suggesting a role of these proteins and transcriptional activity of their genes in uterine polypoid adenomyoma development. Neither Nidogen-2 nor SF-1/NR5A1 were expressed in UPA.

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.

Clinical presentation and management of atypical polypoid adenomyomas: Systematic review of the literature.

The aim of this study is to investigate clinical behavior of Atypical Polypoid Adenomyomas (APAs) and to describe the rates of (i) recurrences, (ii) their association with endometrial hyperplasia and (iii) with endometrial cancer. All studies that reported the outcome of the clinical management of patients with histologically proven APAs were included. A review of the English literature since 1970 was systematically performed (PROSPERO No CRD42018080003). A quality assessment tool was used to assess the scientific value of all the studies. Main contribution of this review is the proposal of new definitions regarding the clinical behaviour of APAs: Cure, Residual or persistent APA, Recurrent APA, Synchronous endometrial hypeprasia, Subsequent endometrial hyperplasia, Synchronous endometrial cancer, and Subsequent endometrial cancer, are terms elucidated in the context of this review. Their rates after initial diagnosis and treatment of APAs are presented as the main outcome measures. 63 studies and 350 patients were included in the systematic review. Fifteen studies that reported 208 patients who did not have hysterectomy as initial treatment were included for further quantitative assessment. The cure rate of APAs in cases where uterus was preserved was 51.0% (106/208), the residual rate of APA was 20.2% (42/208), the recurrence rate of APA was 35.1% (73/208), the concurrent endometrial hyperplasia rate was 7.2% (15/208), the concurrent endometrial cancer rate was 4.8% (10/208), the subsequent endometrial hyperplasia rate of was 6.7% (14/208), and the subsequent endometrial cancer rate of was 10.1% (21/208). Moreover, 56.4% of the patients with APA who opted for uterine sparing treatment and wished to conceive they had a viable pregnancy. The use of hysteroscopic techniques at the initial management of APAs is related with significantly decreased residual rate, and signicantly increased cure rates. In this review, the recurrence rate and the association of APAs with cancer appeared to be higher compared to the previously reported in the literature rates. APAs comprise an intriguing clinical entity that needs individualized treatment, considering the increased association to serious gynecological diseases. Hysterectomy is the appropriate treatment, however uterus-sparing surgery can be offered to selected patients.

Parasitic Myomas and an Adenomyoma Obstructing the Ureter After Power Morcellation of Myomas and Endometriotic Nodule Resection.

BACKGROUND: The use of power morcellation (PM) in abdominal and pelvic surgery has been discouraged and even banned in some institutions because of the risk of spreading malignant cells, although some authorities maintain that PM can be an appropriate tool for selected patients deemed to be at low risk of malignancy. CASE: A 42-year-old woman developed parasitic myomas and an adenomyoma obstructing the right ureter after laparoscopic excision of multiple myomas and deep infiltrating endometriosis using PM. Laparoscopic excision of the parasitic myomas and removal of the adenomyoma relieved the obstruction of the ureter. CONCLUSION: Although there is reasonable concern about the use of PM spreading malignant disease, benign disease can also be spread by PM and can cause significant complications. Use of PM should be restricted as much as possible.

Neoplasia in Turner syndrome. The importance of clinical and screening practices during follow-up.

AIM OF THE STUDY: Turmer syndrome (TS) patients show increased morbidity due to metabolic, autoimmune and cardiovascular disorders. A risk of neoplasia is also reported. Here, we review the prevalence of neoplasia in a cohort of Turner patients. METHODS: We retrospectively evaluated 87 TS women. Follow-up included periodic ultrasound of the neck, abdominal and pelvic organs, dermatologic evaluation and fecal occult blood test. Karyotype was 45,X in 46 patients. During follow-up, 63 girls were treated with growth hormone, 65 with estro-progestin replacement therapy and 20 with L-thyroxine. Autoimmune diseases were present in 29 TS. RESULTS: A total of 17 neoplasms in 14 out of 87 patients were found. Six skin neoplasia, 3 central nervous system tumors, 3 gonadal neoplasia, 2 breast tumors, 1 hepatocarcinoma, 1 carcinoma of the pancreas and 1 follicular thyroid cancer were detected. Age at tumor diagnosis was higher in 45,X pts than in those with other karyotypes (p = 0.003). Adenomioma gallbladdder (AG) was detected in 15.3% of the patients, with a lower age in girls at diagnosis with an associated neoplasia in comparison with TS without tumors (p = 0.017). No correlation between genetic make up, treatment, associated autoimmune diseases and neoplastia was found. CONCLUSION: In our TS population an increased neoplasia prevalence was reported. A high prevalence of AG was also noted and it might be indicative of a predisposition to neoplasia. Further studies are needed to define the overall risk for neoplasia, and to determine the role of the loss of the X-chromosome and hormonal therapies.

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.

Magnetic resonance imaging findings in atypical polypoid adenomyoma.

OBJECTIVES: This article describes magnetic resonance imaging (MRI) findings in 3 cases of atypical polypoid adenomyoma (APAM). METHODS: Clinical and MRI manifestations of 3 patients with APAM were evaluated. High b value diffusion-weighted and dynamic contrast-enhanced images were performed. The size, shape, site of origin, and signal intensity (SI) of MRI findings were evaluated. RESULTS: All patients (age range, 37-47 years; mean age, 40 years) had a chief complaint of atypical genital bleeding with no history of pregnancy. In 2 cases, cytology of the endometrium was positive, and pathological analysis of curettage specimens indicated endometrioid adenocarcinoma. The MRI revealed an endometrial polypoid mass arising from the upper corpus (50%) or lower uterine segment (50%). Except for 1 tumor that seemed to invade the myometrium of the uterine wall due to its irregular margin, all tumors were well circumscribed. On T2-weighted MRI scans, the SI of the tumor was homogenous and marginally hyperintense, and contained markedly hyperintense cystic foci. On T1-weighted images, all the tumors were isointense relative to the myometrium, and in 1 case, a number of cystic foci showed high SIs. In a dynamic contrast-enhanced study, solid portions other than the cystic foci showed contrast enhancement in the arterial phase and demonstrated a washout pattern or plateau pattern in the late phase. All tumors demonstrated high SIs on diffusion-weighted images. CONCLUSIONS: Differentiating APAM from other malignant polypoid uterine endometrial tumors may still pose difficulties, especially when the tumor seems to invade the myometrium on MRI. Comprehensive clinical information about the patient including age, pathological assessment of the endometrium, and MRI findings should be essential to provide indication for a diagnosis of APAM.

[Clinicopathologic analysis of 14 cases of atypical polypoid adenomyoma of endometrium with the emphasis on its cancerous transformation].

OBJECTIVE: To analyze the clinicopathologic characteristics of atypical polypoid adenomyoma (APA) of endometrium, and investigate the special characteristics of cancerous transformation from APA. METHODS: Fourteen cases of APA were collected in General Hospital of People's Liberation Army from January 2007 to March 2013. The clinical data, morphologic features, immunohistochemistry and the related literature were reviewed. RESULTS: The median age of the 14 patients was 38 years (ranged from 23 to 72 years), only 1 patient was postmenopausal. The most common symptom was irregular vaginal bleeding (4/14), and 4 patients were identified during routine physical examination for infertility. Among 14 cases, 4 cases were diagnosed as well differentiated endometrioid adenocarcinoma originating from APA, and their median age was 35 years (ranged from 28 to 41 years); color Doppler flow imaging (CDFI) of ultrasound showed rich blood flow signal. The tumors with cancerous components were obviously larger than the usual APA (mean diameter: 4.7 versus 1.8 cm). Histologically, irregular and branched glands were embedded in fibromuscular stroma and the glandular epithelium were atypical hyperplasia in varying degrees. While carcinoma developed in the APA, the sieve, solid and papillary structures were noticeable, and necrosis were common. CONCLUSIONS: APA is a rare lesion of the uterus. Although the clinical behavior is benign in most cases, there may be possible for some cases developing carcinomas. If the APA mass is more than 4 cm in diameter, and microscopically demonstrates prominent sieve, solid, papillary structures and necrosis, the diagnosis of carcinoma developed from APA can be made. Thorough analysis should be done before the most proper therapeutic regimen is drawn up.