Adenomyomas of the Gallbladder: An Analysis of Frequency, Clinicopathologic Associations, and Relationship to Carcinoma of a Malformative Lesion.

CONTEXT.­: The nature and associations of gallbladder (GB) "adenomyoma" (AM) remain controversial. Some studies have attributed up to 26% of GB carcinoma to AMs. OBJECTIVE.­: To examine the true frequency, clinicopathologic characteristics, and neoplastic changes in GB AM. DESIGN.­: Cholecystectomy cohorts analyzed were 1953 consecutive cases, prospectively with specific attention to AM; 2347 consecutive archival cases; 203 totally embedded GBs; 207 GBs with carcinoma; and archival search of institutions for all cases diagnosed as AM. RESULTS.­: Frequency of AM was 9.3% (19 of 203) in totally submitted cases but 3.3% (77 of 2347) in routinely sampled archival tissue. A total of 283 AMs were identified, with a female to male ratio = 1.9 (177:94) and mean size = 1.3 cm (range, 0.3-5.9). Most (96%, 203 of 210) were fundic, with formed nodular trabeculated submucosal thickening, and were difficult to appreciate from the mucosal surface. Four of 257 were multifocal (1.6%), and 3 of 257 (1.2%) were extensive ("adenomyomatosis"). Dilated glands (up to 14 mm), often radially converging to a point in the mucosa, were typical. Muscle was often minimal, confined to the upper segment. Nine of 225 (4%) revealed features of a duplication. No specific associations with inflammation, cholesterolosis, intestinal metaplasia, or thickening of the uninvolved GB wall were identified. Neoplastic change arising in AM was seen in 9.9% (28 of 283). Sixteen of 283 (5.6%) had mural intracholecystic neoplasm; 7 of 283 (2.5%) had flat-type high-grade dysplasia/carcinoma in situ. Thirteen of 283 cases had both AM and invasive carcinoma (4.6%), but in only 5 of 283 (1.8%), carcinoma arose from AM (invasion was confined to AM, and dysplasia was predominantly in AM). CONCLUSIONS.­: AMs have all the features of a malformative developmental lesion, and may not show a significant muscle component (ie, the name "adeno-myoma" is partly a misnomer). While most are innocuous, some pathologies may arise in AMs, including intracholecystic neoplasms, flat-type high-grade dysplasia or carcinoma in situ, and invasive carcinoma (1.8%, 5 of 283). It is recommended that gross examination of GBs include serial slicing of the fundus for AM detection and total submission if one is found.

Gastric type III heterotopic pancreas presenting as adenomyoma in the antrum of the stomach: a case report.

Although heterotopic pancreas usually occurs in the stomach and rarely presents as a submucosal tumor, an accurate preoperative diagnosis is often difficult because of the variety of clinical symptoms and findings depending on the size and location of the lesion. We experienced a case of gastric type III heterotopic pancreas presenting as a gastric adenomyoma in the antrum of the stomach. A 62-year-old woman visited a local hospital for epigastric discomfort. An esophagogastroduodenoscopy study indicated a submucosal tumor in the greater curvature of the gastric antrum. The patient underwent surgical resection of the tumor because it was enlarged. The histological sections of the resected specimen showed that the tumor was composed of ductular structures lined by tall columnar epithelia and a prominent smooth muscle stroma with no atypical cells. The tumor was compatible with Heinrich's type III heterotopic pancreas, which presented as an adenomyoma of the stomach. These findings provide useful histological features and some insight into a better understanding of the embryonic origin and development of adenomyoma and heterotopic pancreas in the antrum of the stomach.

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.

Adenomyoma of the small intestine in children: a 12-year experience in a tertiary referral center.

BACKGROUND: Adenomyoma of the small intestine is rare in children and the clinical characteristics is not clear. The study was to document the clinical characteristics and treatment of children with adenomyoma of the small intestine. METHODS: A retrospective study was conducted in children with intestinal adenomyoma from 2010 to 2022. We recorded age, gender, symptoms, location, tumour size and treatment options. RESULTS: Thirteen patients with adenomyoma of the small bowel were included. The median age was 20 months with a male-to-female ratio of 10:3 and more than half of the patients were younger than 2 years old. The mean tumour size was 2.0 cm. The lesion was found accidentally in one patient, and the others presented with symptoms of intussusception. A pathological lead point was found on ultrasound in seven patients. All tumours were located in the ileum, ranging from 24 to 260 cm proximal to the ileocecal valve. The tumour was found in an antimesenteric site in eight patients. Three patients suffered intestinal necrosis, and segmental resection of the ileum was performed. Three patients without intestinal necrosis underwent tumour rection, while intestinal resection and anastomosis were performed in the remaining seven. All patients recovered well except one, who developed intussusception 7 days after surgery; that patient underwent surgery and recovered uneventfully. CONCLUSIONS: Adenomyoma of the small intestine has a male predominance in children and intussusception is a common presentation. The ultrasound feature is a mass of mixed echogenicity containing several small cystic areas. Surgery is the primary treatment option and the procedure should be chosen based on intraoperative findings.

Surgical treatment of a rare case of atypical polypoid adenomyoma of the uterus in a menopausal patient: a case report.

Atypical polypoid adenomyoma is a rare benign tumor of the uterus that usually affects women of reproductive age and has an increased risk of progression into endometrial cancer. The pathogenetic mechanism has not been completely clarified. Due to the rarity of the tumor, current experience regarding the diagnostic and therapeutic approach is limited. For menopausal patients, hysterectomy seems to be the main treatment option. Our case concerns an asymptomatic menopausal patient with a vaginal delivery in her obstetric history and no hereditary history of gynecological cancer who came to the outpatient clinic for a gynecological examination. Transvaginal ultrasound revealed the presence of a large, round solid mass with increased vascularity within the endometrial cavity. A diagnostic dilation and curettage of the endometrium was performed. Histological examination of the endometrial biopsy showed an atypical polypoid adenomyoma, and it was decided to perform a total abdominal hysterectomy with bilateral adnexectomy. Histological examination of the surgical specimen of the uterus revealed no residual disease, no coexisting foci of atypical endometrial hyperplasia or endometrial cancer. The postoperative course was uneventful. The patient remains to this day under regular follow-up. The present case report highlights the significant difficulties involved in the preoperative diagnosis of atypical polypoid adenomyoma of the uterus and the difficult differential diagnosis from atypical endometrial hyperplasia and endometrial cancer, particularly in menopausal patients. At the same time, it is pointed out that despite its rarity, the early diagnosis of atypical polypoid adenomyoma, especially in young women, must be the main concern of the modern gynaecologist, in order to design the optimal treatment aimed at preserving fertility while avoiding the risk of recurrence of damage or malignant progression into endometrial cancer.

Extrauterine adenomyoma of the lesser omentum: A case report and review of the literature.

RATIONALE: The extrauterine adenomyoma is rare and it is extremely rare outside the pelvic cavity. Herein, we reported the first case of a single extrauterine adenomyoma occurring in the lesser omentum. PATIENTS CONCERNS: This case involved a 55-year-old woman who had undergone subtotal gastrectomy and omentectomy for gastric carcinoma. During postoperational pathological examination, 1 lymph node-like mass was coincidentally found in the lesser omentum. The patient had a history of hysterectomy for uterine leiomyoma 8 years ago. DIAGNOSES: The resected 17 "lymph nodes" from the lesser omentum were routinely checked for possible metastasis of gastric carcinoma. One of lymph node-like mass was microscopically showed that it was composed of benign smooth muscle components, endometrial glands and stroma by HE staining. Therefore, adenomyoma was initially considered. INTERVENTIONS: The lymph node-like mass was removed together with the lesser omentum during the subtotal gastrectomy and omentectomy for gastric carcinoma. No special intervention was performed for the adenomyoma. OUTCOMES: Immunohistochemical staining confirmed that smooth muscle tissue was diffusely and strongly positive for Desmin, smooth muscle actin, estrogen receptor, and progesterone receptor, and negative for CD117, Dog-1, S100, and CD34. Endometrial glands and stroma were positive for estrogen receptor and progesterone receptor, and the endometrium interstitium was also positive for CD10. The final diagnosis of extrauterine adenomyoma occurring in the lesser omentum was established. LESSONS: So far, to the best of our knowledge, total 53 cases of extrauterine adenomyoma have been reported in 45 English reports. The most common location for a single mass was pelvic cavity (37 cases), but rarely outside the pelvic cavity. This is the first case of a single extrauterine adenomyoma occurring in the lesser omentum.

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.

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.

[Endometrioide-type cervical adenomyoma]. / Adenomioma cervical de tipo endometrioide.

Adenomyoma of the cervix is a rare, benign lesion. It is a localized form of adenomyosis, which is surrounded by a benign muscle proliferation forming a tumor similar to leiomyoma. Of the three histological variants of adenomyomas, the most frequent is the endocervical type. We present a case of a 34-year-old woman with a tumor in the lower uterine segment which enlarged during pregnancy, replacing the entire cervix and resulting in the occlusion of the endocervical canal. Ultrasound and tomography showed a mixed tumor pushing aside the uterus and bladder. Speculoscopy revealed that the tumor protruded towards the vaginal canal. Histopathological established the diagnosis of a cervical adenomyoma of the endometrioid type.

MRI and Transvaginal Ultrasound Findings of Atypical Polypoid Adenomyoma: A Case Report / 中国医学科学杂志(英文版)

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.

Adenomiosis apendicular: reporte de dos casos / Appendicular adenomiosis: report of two cases

Resumen Los adenomiomas son tumoraciones benignas constituidas por un agregado nodular de músculo liso, glándulas endometriales y estroma endometrial. La presencia de adenomiomas fuera del útero es un hallazgo infrecuente. Presentamos dos casos de adenomiomas extrapélvicos localizados en el apéndice. El estudio histológico resultó esencial para el diagnóstico.

Abstract Adenomyomas are a benign tumor compound of smooth muscle nodular aggregate, endometrial glands and endometrial stroma. Adenomyomas presenting outside uterus are a rare finding. Here we report two extrapelvic adenomyomas of the appendix. Histological examination was essential for diagnosis.

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.

Detection of MED12 mutations in mesenchymal components of uterine adenomyomas.

Adenomyoma of the uterus is a biphasic nodular lesion composed of a mesenchymal component with smooth muscle differentiation and a glandular epithelium. The neoplastic nature of uterine adenomyomas has been controversial because some are considered to be nodular adenomyosis. MED12 mutations are involved in the pathogenesis of uterine smooth muscle tumors (leiomyomas and leiomyosarcomas) and biphasic tumors of the breast (fibroadenomas and phyllodes tumor). To investigate the histogenesis of uterine adenomyomas, we performed pathological and genetic analyses, including Sanger sequencing of MED12. In total, 15 cases of uterine adenomyomas were retrieved and assessed for clinicopathological factors. Immunohistochemistry for smooth muscle actin, desmin, and CD10 was performed. Exon 2 of MED12 was Sanger sequenced using DNA obtained by macrodissection of the adenomyomas. For cases that were positive for somatic MED12 mutations, we next performed microdissection of the mesenchymal and epithelial components. The DNA extracted from each component was further analyzed for MED12 mutations. MED12 mutations were detected in two adenomyomas (2/15, 13%), all in a known hot spot (codon 44). In both lesions, MED12 mutations were detected in multiple spots of the mesenchymal component. The epithelial component did not harbor MED12 mutations. The relatively low frequency of MED12 mutations suggests that not all adenomyomas are leiomyomas with entrapped glands. However, the results of our study suggest that a subset of uterine adenomyomas are true mesenchymal neoplasms.

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.

Mural Intracholecystic Neoplasms Arising in Adenomyomatous Nodules of the Gallbladder: An Analysis of 19 Examples of a Clinicopathologically Distinct Entity.

Intracholecystic neoplasms (ICNs) (pyloric gland adenomas and intracholecystic papillary neoplasms, collectively also called intracholecystic papillary/tubular neoplasms) form multifocal, extensive proliferations on the gallbladder mucosa and have a high propensity for invasion (>50%). In this study, 19 examples of a poorly characterized phenomenon, mural papillary mucinous lesions that arise in adenomyomatous nodules and form localized ICNs, were analyzed. Two of these were identified in 1750 consecutive cholecystectomies reviewed specifically for this purpose, placing its incidence at 0.1%. Median age was 68 years. Unlike other gallbladder lesions, these were slightly more common in men (female/male=0.8), and 55% had documented cholelithiasis. All were characterized by a compact multilocular, demarcated, cystic lesion with papillary proliferations and mucinous epithelial lining. The lesions' architecture, distribution, location, and typical size were suggestive of evolution from an underlying adenomyomatous nodule. All had gastric/endocervical-like mucinous epithelium, but 5 also had a focal intestinal-like epithelium. Cytologic atypia was graded as 1 to 3 and defined as 1A: mucinous, without cytoarchitectural atypia (n=3), 1B: mild (n=7), 2: moderate (n=2), and 3: severe atypia (n=7, 3 of which also had invasive carcinoma, 16%). Background gallbladder mucosal involvement was absent in all but 2 cases, both of which had multifocal papillary mucosal nodules. In conclusion, these cases highlight a distinct clinicopathologic entity, that is, mural ICNs arising in adenomyomatous nodules, which, by essentially sparing the "main" mucosa, not displaying "field-effect/defect" phenomenon, and only rarely (16%) showing carcinomatous transformation, are analogous to pancreatic branch duct intraductal papillary mucinous neoplasms.

Significance of stromal markers in atypical polypoid adenomyoma.

Atypical polypoid adenomyoma (APA) may progress to endometrioid carcinoma and may mimic myoinvasive carcinoma on biopsy specimens. Here, we present a case of an APA of the uterine cervix hysteroscopically treated, which recurred two years after and progressed to endometrioid carcinoma. In all biopsy specimens and in the hysterectomy specimen, the benign APA component showed an unusual immunohistochemical stromal pattern (periglandular fringe-like CD10 pattern, diffuse h-caldesmon positivity, p16 negativity), which is typical of myoinvasive carcinoma. Interestingly, the other three cases of cancerized APA assessed for h-caldesmon in the literature showed diffuse stromal positivity also in the benign APA component. Our case shows that the stromal markers used for differentiating between APA and myoinvasive carcinoma may be misleading even when their pattern seems unequivocal. Furthermore, our case suggests that h-caldesmon positivity might be a prognostic marker for progression of APA to carcinoma. Further studies are encouraged in this regard.

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.