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To investigate the epidemiology and clinical characteristics of infants presenting with conjunctival, palpebral, or orbital congenital choristomas (dermolipomas, epidermoids, and dermoid cysts) and children undergoing surgery for congenital choristomas in the ocular adnexa.We reviewed the medical files of congenital choristomas in children seen in The Capital Region of Denmark during a 5-year period (2014-2018). Children ( 18 years) were divided into two groups: those referred 1 year of age (Group I) and those undergoing surgery to remove the lesion (Group II). Group I was used to calculate a population-based incidence of congenital choristomas by comparing our data to birth statistics from the Danish Medical Birth Registry.A total of 97 children were included, 43 in Group I and 70 in Group II (including 16 patients from Group I). The total incidence of congenital choristomas was 1 in 2537 live born children. Most lesions were palpebral choristomas (27/43, 63%) located in the superotemporal region (17/27, 63%), followed by the superonasal region (7/27, 26%). The main reasons for surgical removal of a congenital choristoma were growth (28/70, 40%) or cosmesis (25/70, 36%).The total incidence of congenital choristomas in the ocular adnexa of infants 1 year of age, including both conjunctival and palpebral congenital choristomas, is about 1 in 2537 live born children in The Capital Region of Denmark. Hence, congenital choristomas are common masses found in the ocular adnexa.

The term 'choristoma' refers to normal appearing tissue in an abnormal location. We describe a case of choristoma with gastric epithelium of the dorsal tongue in a pediatric patient. Lingual choristomas are uncommon cystic or solid lesions which may demonstrate different types of tissue (e.g. gastric epithelium, respiratory epithelium, osseous and neuroglial tissue) histologically. Choristomas with gastric epithelium, also known as heterotropic gastric mucosa or foregut duplication cysts, are thought to arise from pluripotential cells of the embryonic foregut. They most frequently involve the anterior two-thirds of the tongue. Most patients are asymptomatic, but larger lesions may lead to feeding and breathing difficulties. Pathologic evaluation and surgical excision remain the mainstay of diagnosis and treatment, respectively. The pathologic characteristics of other congenital tongue lesions are also discussed.

Background Ectopic intrathyroidal thymic tissue is a rare diagnosis, specifically in adults. Such ectopic tissue is usually misdiagnosed as benign or malignant thyroid lesions and is mainly investigated by ultrasonography and pathologic examination. Case presentation We present the case of an adult 31-year-old Persian female patient with a cervical mass and no other significant medical history. The lesion had hypo- to isoechoic features on sonographic imaging, and needle aspiration examination revealed lymphoid cells suspicious of lymphoid malignancies. However, pathologic examination after resection of the lesion showed normal thymic tissue. Conclusions The rare entity of ectopic thymic tissue within the thyroid gland in adult patients requires meticulous examination by clinicians, radiologists, and pathologists to diagnose the condition with high accuracy and plan appropriate management.

Background Neuromuscular choristomas (NMCs), are extremely rare developmental lesions that, have been previously established associated with recurrent fibromatosis after surgery, leading to several operations or even amputation. However, reports on the ultrasound imaging features and clinical conditions of NMCs are rare. The purpose of this study is to describe the ultrasound features and clinical analysis of NMCs to provide suggestions to identify the optimal management strategy. Methods From September 2020 to September 2021, 7 patients with a confirmed diagnosis of NMC who underwent ultrasound examination in our department were enrolled in our study. Physical examinations were performed to detect motor deficits, sensory deficits, neuropathic pain, limb undergrowth, muscular atrophy, cavus foot and bone dysplasia. Ultrasound imaging was performed and investigated both in affected nerves and neuromuscular choristomas associated desmoid-type fibromatosis (NMC-DTF). All patients had a definite history and regular follow-up. The clinical course, physical examinations, ultrasound features and pathologic results of NMC patients were analyzed. Results Seven patients with an average age of 7.0 ± 7.2 years (range: 2–22 years) were enrolled in our study. The affected nerves included the sciatic nerve (6 cases) and the brachial plexus (1 case). Six patients (85.7%) presented with limb undergrowth, 6 (85.7%) with muscular atrophy, and 5 (71.4%) with cavus foot deformity. Based on ultrasound findings, all the visibly affected nerve segments presented with hypoechoic and fusiform enlargement with intraneural skeletal muscle elements. Five patients (71.4%) had NMC-DTFs at the site of the affected nerve. All NMC-DTFs were shown as hypoechoic solid lesions adjacent to the nerve and were well circumscribed. In the subset of the surgery group, all 5 patients presented with progression to NMC-DTFs at the site of the NMCs. No fibromatosis was detected in the other two nonsurgical patients. Conclusions Understanding the typical ultrasound features and clinically associated conditions would support the early diagnosis of this rare disease. When a potential diagnosis is determined, an invasive procedure such as biopsy or resection might not be a good choice given the frequent occurrence of complications such as aggressive recurrence.

Ectopic pancreas often occurs in the gastrointestinal tract. However, the identification of a mediastinal ectopic pancreas is extremely rare. Herein, we report a case of ectopic pancreas of the anterior mediastinum, which was incidentally detected during treatment for pericarditis with widespread ST elevation on electrocardiogram. A 40-year-old woman complaining of chest pain was admitted with suspected pericarditis. A closer examination revealed a cystic mass in the anterior mediastinum, which was diagnosed as a pancreatic cyst of the anterior mediastinum on thoracoscopic tumor resection.

Background Neuromuscular choristoma (NMC) is a rare peripheral nerve lesion that is composed of ectopic mature muscle fibers and nerve fascicles, typically involving major nerve roots or trunks, such as the cranial nerves, brachial plexus, and sciatic nerves. The onset of NMC frequently occurs in the first decade of life. Here, we present the first documented case of a case of esophageal NMC in an adult patient. Case presentation A 46-year-old male patient presented in 2018 with a submucosal tumor of the esophagus. Upon presentation, the tumor was approximately 10 mm in diameter, covered by normal mucosa, and located in the left posterior wall of the esophagus in a position that was 30 cm from the incisor. The tumor was discovered incidentally during gastroscopic examination. In March 2021, endoscopic re-examination revealed no significant changes in the tumor. Endoscopic ultrasound revealed an oval hypoechoic mass with a homogeneous internal echo that originated from the muscularis propria with a maximum cross section of 13 mm × 6 mm. Resection was performed under gastroscopy. The resection specimen was 12 mm × 5 mm in size and was a well-demarcated, elastic, hard, and tough with a gray section. Histologically, the specimen consisted of an abundance of smooth muscle fiber bundles intercalated among nerve fibers, but without malignancy. Immunohistochemical examinations revealed positivity for S-100 protein, caldesmon, NSE and desmin, but negativity for CD117, DOG-1, HMB45, and Melan A. There was also aberrant nuclear localization of beta-catenin. Collectively, these findings led to a diagnosis of esophageal NMC. Conclusions NMC is extremely rare, especially esophageal NMC, and is very challenging to accurately diagnose prior to resection. It is important that we can differentiate NMC from other types of tumors.

Intrathyroid thymic carcinoma (ITTC) is a rare malignant neoplasm considered to be a eutopic thymic carcinoma (TC) arising ectopically in the thyroid. Histopathologically, ITTC resembles squamous cell carcinoma of the thymus with positive TC markers such as CD5 and c-KIT. Despite these similar histological findings, ITTC is clinically less aggressive than TC. In this study, we compared clinical, histological, and genetic characteristics of ITTCs and TCs. We collected 9 ITTCs and 8 TCs with their clinicopathological profiles. Immunohistochemistry for CD5, p63, CD117/c-KIT, Ki-67, p53, TTF-1, thyroglobulin, PAX8, EGFR, and PD-L1/CD274 plus in situ hybridization for EBER was performed. We further investigated mutation status of KIT, EGFR, BRAF, and TERT promoter using Sanger sequencing. In our study, ITTCs affected significantly younger patients than TCs. After a mean follow-up of 86 months, all patients with ITTC were alive, while two patients with TC had died. Immunohistochemistry showed ITTCs and TCs had a similar immunophenotype except for EGFR and p53. Genetic analysis did not identify KIT or BRAF mutations in any ITTCs or TCs. EGFR mutations were positive in 11% (1/9) of ITTCs and 25% (2/8) of TCs. Notably, TERT promoter C228T mutation was identified in 22% (2/9) of ITTCs but none of the TCs. There were no significant differences in age, tumor size, or sex between TERT-mutated and TERT-wild-type ITTCs. Collectively, ITTC and TC have similar histopathologic and immunophenotypic features but different clinical outcomes. Recurrent TERT promoter mutation may be a key event related to cancer progression in ITTCs and warrants further investigation.

Introduction: Isolated heterotopic pancreas (HP) as the primary cause of bowel intussusception is extremely rare. We report a case of a 33-year-old female patient with spina bifida admitted to the Emergency Surgical Department for ileal intussusception due to the presence of heterotopic pancreas associated with endometriosis. Areas Covered: Symptomatic ileal intussusception for ectopic pancreas is usually associated with overt gastrointestinal blood loss (predominantly melena), abdominal pain, vomiting, and weight loss. Treatment is universally surgical. Expert Commentary: Isolated heterotopic pancreas is a rare condition; it should be considered in the differential diagnosis of bowel intussusception.

Acromegaly is usually due to growth hormone (GH)-secreting pituitary adenomas, but it may be exceptionally caused by GH-secreting ectopic pituitary adenomas (EPA). EPA are defined as extra-sellar pituitary tumours, extra- or intra-cranially sited, entirely separated from the pituitary stalk and gland. The aim of the study is to address the challenges in the management of clival GHEPA. We reported a case of a 53-year-old acromegalic patient with a primary clival GHEPA and reviewed systematically the relevant English literature between 1975 and 2019, in keeping with the PRISMA guidelines. Four cases of primary clival GHEPA have been described in literature apart from ours. All patients presented with acromegalic features, elevated circulating GH and/or insulin-like growth factor-1 levels. Hyperprolactinemia and empty sella were described in two cases, respectively. These tumours show the typical imaging characteristics of pituitary adenomas, but their neuroradiological diagnosis may be challenging due to their sizes and the difficulty in defining the absence of connections with the pituitary fossa. Although primary clival GHEPA are exceedingly rare, even if likely under-reported in literature, they should be considered in the differential diagnosis of clival tumours because of their specific management. Surgery represents the first-line treatment option, while medical and radiation therapies can be adopted as neo-adjuvant, adjuvant or primary treatments according to tumour and patient characteristics.

We describe a case of ectopic cervical thyroid tissue which was involved by fibrosing Hashimoto’s thyroiditis and which mimicked metastatic papillary thyroid carcinoma both on fine needle aspiration cytology and biopsy. The patient underwent total thyroidectomy which revealed fibrosing Hashimoto’s thyroiditis, but no carcinoma. The entire thyroidectomy specimen was submitted for histopathological assessment. Even in the resected thyroidectomy specimen, there were cytological changes that were strongly reminiscent of papillary thyroid carcinoma. However, interpreted in the correct clinico-pathological context, these cytological alterations were deemed to be reactive secondary to the fibro-inflammatory process.

To present the sonographic follow-up of intrathyroidal ectopic thymus (IET) in children and adolescent patients. Out of the 507 children referred to FNAB between 2006 and 2018, 30 (5.9%) pediatric patients (10 females), mean age 5.7 years (1.2–13.8, median 4.9 years) were diagnosed with IET. A retrospective analysis of medical files of patients diagnosed with IET between 2006 and 2018. Assessed data included ultrasound characterisation, elastographic strain ratio (SR) results and hormonal evaluation. Analysis of thyroid US scans revealed that the mean age at the first thyroid ultrasound was 5.7 (1.2–13.8, median 4.9) years, and at the last US 10.7 (3.7–18, median 10.5) years. The mean time of the IET observation was 59.6 (2–148, median 53.5) months. On US, IET was hypoechoic with multiple linear and punctate echoes, hypovascular, fusiform on longitudinal plane and round or polygonal on an axial plane, more common in the right thyroid lobe (66.7%) and located in the posterior part of the lobes (54.5%), bilateral in two patients and multifocal in one patient. SR of IET was similar to the surrounding normal thyroid tissue. Complete regression of IET was observed in 12/30 patients after a mean time of 81.7 months (median 76.5), at the mean age of 13.7 (9.2–18, median 13.9) years. FNAB was performed in 10/30 and a hemithyroidectomy in 1/30 IET patients. In the FNAB (+) group, patients were younger (5.08 vs 6.08 years) and lesions were larger (0.12 ml vs 0.05 ml) than in the FNAB (−) group. All patients with IET were euthyroid with negative TPOAb and TgAb levels. The reproducibility of unique ultrasound features of IETs allows for safe long-term follow-up of these benign lesions in the majority of pediatric patients: not only monitoring the regression of IET but also screening towards the rare occurrence of a tumor arising from the IET.

BACKGROUND: Primary hyperparathyroidism (PHPT) in children and adolescents is uncommon. Data-driven guidelines for management in pediatric patients are limited. METHODS: We performed a retrospective cohort analysis of all patients (1997-2017) with PHPT ≤21 years of age who underwent parathyroidectomy at three institutions. Clinical and demographic variables were analyzed. Primary operative outcome was cure (normocalcemia >6 months after surgery); secondary outcome was operative success (intraoperative parathyroid hormone (IOPTH) decrease of ≥50%). RESULTS: We identified 86 patients with a median age of 17 years (IQR:14,19); 64% (n=55) were female. The mean preoperative serum calcium was 11.7 mg/dL, median parathyroid hormone (PTH) was 110 pg/mL, and median urine calcium was 4.1 mg/kg/24 hours. Preoperatively, sestamibi scan localized in 41/71 patients (58%); neck ultrasound localized in 19/44 (43%). The most common pathology at surgery was a single eutopic parathyroid adenoma in 71% (n=61). A high incidence of ectopic adenomas (25%, n=22) was observed, most commonly intrathymic (n=13), followed by tracheoesophageal groove (n=5), carotid sheath (n=2) and intrathyroidal (n=2). Of 56 patients with retrievable data >6 months postoperatively, cure was achieved in 55 of 56 patients (98%). One patient who presented to us with parathyromatosis requiring subsequent reoperation. CONCLUSION: In this multi-institutional series of PHPT in children and adolescents, the majority were sporadic PHPT and were due to a single adenoma. We observed a high incidence of ectopic parathyroid adenomas, most commonly intrathymic. Given the high risk for ectopic adenoma in pediatric patients, parathyroid surgery in children and adolescents should be performed by experienced surgeons.

Desmoid-type fibromatosis (DTF) frequently arises in patients with neuromuscular choristoma (NMC). We hypothesize that NMC-associated DTF occurs in soft tissues innervated by the NMC-affected nerve, and arises from CTNNB1-mutated (myo) fibroblasts within or directly adjacent to the NMC. A retrospective review of patients treated at our institution was performed for patients with biopsy-confirmed diagnosis of NMC-DTF. Clinical presentation, physical examination, electrodiagnostic findings and radiological features (MR and FDG PET/CT images for each NMC-DTF) and pathologic re-review of available materials were analyzed. A literature review was also performed. Eight patients from our institution met the inclusion criteria. All patients presented with neuropathic symptoms and soft tissue or bone changes in the nerve territory innervated by the NMC. All MR images (N=8 cases) showed the characteristic features of NMC, and also showed direct contact between unifocal (N=5) or multifocal (N=3) DTF(s) and the NMC-affected nerve NMC. FDG PET/CT (N=2 cases) showed diffuse, increased FDG uptake along the entire affected nerve segment, contiguous with the FDG-avid DTF. In all cases, the DTFs arose in the soft tissues of the NMC-affected nerve’s territory. No patient developed DTF at any other anatomic site. These data demonstrate that NMC-DTF arises solely within the NMC-affected nerve territory, and has direct contact with the NMC itself. Based on all these findings and the multifocality of NMC in several cases, we recommend imaging and surveillance of the entire NMC-affected nerve (from spine to distal extremity) to identify clinically-occult DTF in patients with NMC.

The diagnosis of Chiari 1 malformation is based on the extent of tonsillar ectopia.To examine the relationship between the extent of tonsillar ectopia and the intra-operative findings and clinical outcome following Chiari decompression surgery.Patients were divided into four groups depending on the position of the cerebellar tonsil (T): group 1: 0 T 3; group 2: 3 ≤ T ≤ 5; group 3: 5 T ≤ 10; and group 4: T 10. Intra-operative observations were recorded with regard to compression of the brain stem by posterior inferior cerebellar artery (pica), neuroma formation along the first cervical (C1), and accessory spinal nerves (XI), and pallor of the cerebellar tonsils. Brain stem auditory evoked potentials, (BAEP), were monitored in each case. One hundred sixty-eight patients accrued between 2009 and 2013 agreed to participate in an outcome study to determine the effectiveness of foramen magnum decompression. Findings across the four groups were compared using one-way ANOVA. Observed differences were further subjected to paired analysis. Intra-group comparisons were made using the paired t test. A P value less than 0.05 was considered statistically significant.There were 98 patients in group 1, 147 patients in group 2, 180 patients in group 3, and 63 patients in group 4. The mean extent of tonsillar ectopia was 0.4, 4.0, 7.1, and 14.3 mm in the four groups respectively. The prevalence of tonsillar pallor was greatest in group 4. Otherwise, there was no difference observed in the operative findings. A reduction of 0.1 msec in the wave III-wave V latency of the BAEP was noted in all four groups with equal frequency. One hundred ten patients complied with at least 6 months follow-up. There was no difference in the prevalence of symptoms between the four groups at the time of initial evaluation and at 6 weeks and 6 months following surgery. There was a statistically significant reduction in the intensity of individual symptoms 6 months following surgery regardless of the extent of tonsil ectopia.Other than the finding of tonsillar pallor, there was no relationship between the extent of tonsillar ectopia and the intraoperative anatomical and physiological observations, nor was there any relationship to the likelihood of symptomatic improvement following surgery. These observations call into question the focus on the extent of tonsillar of ectopia in assessing the patient who presents with symptoms of the Chiari malformation.

Accessory spleen (AS) is a benign condition, where ectopic spleen tissue can be found elsewhere in the abdomen and pelvis, with approximately 20% of cases located at or in the tail of the pancreas. When discovered on imaging, it can be mistaken for conditions that do require surgical removal, including neuroendocrine tumor, and so accurate diagnosis of AS can prevent unnecessary surgery. Endoscopic ultrasound (EUS)-guided fine-needle aspiration (FNA) is a commonly performed diagnostic modality that can lead to a confirmatory diagnosis of AS. We present a case of AS diagnosed at our institution by EUS-FNA and review the literature for all reported cases of AS that have been confirmed by EUS-FNA.

To evaluate the diagnostic performance of superparamagnetic iron-oxide (SPIO)-enhanced diffusion-weighted image (DWI) for distinguishing an intrapancreatic accessory spleen from pancreatic tumors. Twenty-six cases of intrapancreatic accessory spleen and nine cases of pancreatic tail tumors [neuroendocrine tumor (n = 8) and pancreatic adenocarcinoma (n = 1)] were analyzed. Two blind reviewers retrospectively reviewed the SPIO-enhanced magnetic resonance imaging (MRI) scans. The lesion visibility grades were compared and the diagnostic performance of SPIO-enhanced DWI was compared to those of SPIO-enhanced T2WI and T2*WI with the use of a receiver operating characteristic (ROC) analysis. The grade of lesion visibility was the highest on DWI [mean ± standard deviation (SD): 2.8 ± 0.3] followed by T2WI (2.3 ± 0.7, p

In cases of subepithelial tumors (SETs) located in the cardiac area, a preoperative histologic diagnosis might be helpful in determining the requirement of surgery. To investigate the efficacy of endoscopic ultrasound-guided fine-needle biopsy (EUS-FNB) in gastric SETs located in the cardia. The data of 107 patients who underwent EUS-FNB, from 2012 to 2017, for tissue sampling of gastric SETs located in the cardia were collected. The clinicopathological data, EUS-related parameters, and surgical outcomes were retrospectively reviewed. The EUS-FNB results were diagnostic in 86.9% (93/107) and nondiagnostic in 13.1% (14/107) of the patients. Immunostaining of the FNB specimens led to the diagnosis of gastrointestinal stromal tumor (GIST) in 25 SETs (23.4%), leiomyoma in 62 SETs (57.9%), heterotopic pancreas in 3 SETs (2.8%), and schwannoma in 2 SETs (1.9%). In the multivariate analysis, patients with GISTs showed significantly more inhomogeneous echogenicity [odds ratio (OR), 8.867], more cystic foci (OR, 26.98), and older age (OR, 1.087). In 26 patients who underwent surgical resection, the agreement between EUS-FNB and surgical pathological findings was 100% with respect to the diagnosis of GISTs (n = 20) and leiomyoma (n = 7). Among these cases, the proportion of high-risk GISTs was 20.0% (4/20), and no leiomyosarcoma was detected. Although a majority of the subepithelial lesions in the cardia of the stomach are benign, 20% of the cases diagnosed with GIST have a high malignant potential. Preoperative EUS-FNB might be a useful tool for decision-making regarding the ultimate management and outcomes of these lesions.

Small bowel obstruction is an uncommon disease in pregnant women and rare when it is caused by intussusception. Intussusception is a rare cause of intestinal obstruction in adult. When it occurs, it is almost always secondary to an underlying pathology acting as a lead point for invagination, such as polyps, hemartomas, lipomas, leiomyomas, Meckel's diverticulum, adenomas, strictures, and malignancies. Ectopic pancreatic tissue is a very rare pathology acting as the lead point. In this case, we present a very rare case of intussusception in a young pregnant woman, caused by an intramural ectopic pancreatic tissue in the ileum.

This exclusively surgical series on pediatric non-variceal gastrointestinal bleed (NVGIB) defines three levels of bleed site and describes etiology, bleed severity, diagnostic algorithm, and surgical management for each bleed site. Management challenges are detailed. Patients aged ≤ 18 years treated surgically for NVGIB were analysed. Bleed site (n = 87) was classified as: upper gastrointestinal bleed (UGIB; n = 11); small bowel bleed (SBB: n = 52); and lower GIB (n = 24). Four etiology-based groups were identified: lesions with ectopic gastric mucosa (EGM; n = 33), tumours (n = 23), ulcers (n = 21), and vascular pathology (n = 8). Bleed severity spectrum was: acute severe bleed (n = 12); subacute overt bleed (n = 59); and occult GIB (n = 16). Preoperative diagnosis was obtained in all UGIB and LGIB lesions. Eighty-two percent of surgical SB lesions were diagnosed preoperatively on Tc99m pertechnetate scan, computed tomography enterography–angiography, and capsule endoscopy; remaining 18% were diagnosed at laparotomy with intra-operative enteroscopy (IOE). Surgical management was tailored to bleed site, severity, and etiology. Indications of IOE and approach to management challenges are detailed. The commonest site-specific bleed etiologies were duodenal ulcers for UGIB, EGM lesions for SBB, and tumours for LGIB. SBB presented diagnostic challenge. Diagnostic algorithm was tailored to bleed site, age-specific etiology, bleed severity, and associated abdominal/systemic symptoms. Management challenges were acute severe bleed, occult GIB, SBB, obscure GIB, and rare etiologies. IOE has a useful role in SBB management.

A 73-year-old man with prior history of duodenal ulcer has been undergoing periodic upper gastrointestinal endoscopy since 1999. In 2017, a 25-mm submucosal tumor-like protrusion was detected in the lesser curvature of the upper stomach; histological examination of the lesion revealed gastric adenocarcinoma of fundic gland type. En bloc resection was achieved using endoscopic submucosal dissection. The patient was histopathologically diagnosed with gastric adenocarcinoma of fundic gland type arising from heterotopic gastric glands. Immunohistochemical staining was positive for MUC5AC, MUC6, pepsinogen I, and proton pump but negative for MUC2 and CD10. Moreover, the patient’s Ki-67 labeling index score was extremely low. The presence of MUC5AC indicated that the tumor differentiated to the foveolar epithelium and fundic glands. Gastric adenocarcinoma of fundic gland type that differentiates to several directions has a higher malignant potential than the disease that differentiates to chief cells. A retrospective review of the patient’s previous endoscopic examination revealed that the submucosal tumor-like protrusion existed since 2000; tumor size increased from 8 mm in 2000 to 25 mm in 2017. The present case is rare in that the carcinoma arose from heterotopic gastric glands. Moreover, the 19-year follow-up revealed that the tumor differentiated to the foveolar epithelium, considered as having high-grade malignancy.

During gastrulation, physical forces reshape the simple embryonic tissue to form the complex body plans of multicellular organisms1. These forces often cause large-scale asymmetric movements of the embryonic tissue2,3. In many embryos, the gastrulating tissue is surrounded by a rigid protective shell4. Although it is well-recognized that gastrulation movements depend on forces that are generated by tissue-intrinsic contractility5,6, it is not known whether interactions between the tissue and the protective shell provide additional forces that affect gastrulation. Here we show that a particular part of the blastoderm tissue of the red flour beetle (Tribolium castaneum) tightly adheres in a temporally coordinated manner to the vitelline envelope that surrounds the embryo. This attachment generates an additional force that counteracts tissue-intrinsic contractile forces to create asymmetric tissue movements. This localized attachment depends on an αPS2 integrin (inflated), and the knockdown of this integrin leads to a gastrulation phenotype that is consistent with complete loss of attachment. Furthermore, analysis of another integrin (the αPS3 integrin, scab) in the fruit fly (Drosophila melanogaster) suggests that gastrulation in this organism also relies on adhesion between the blastoderm and the vitelline envelope. Our findings reveal a conserved mechanism through which the spatiotemporal pattern of tissue adhesion to the vitelline envelope provides controllable, counteracting forces that shape gastrulation movements in insects.

Choristomas and hamartomas within the oral cavity are relatively uncommon lesions and may present with diverse clinical and histopathological appearances. In this report, we describe two infant patients with hamartoma with ectopic meningothelial elements involving tongue and maxillary alveolar ridge. To the best of our knowledge, these are the first two cases in which a meningothelial proliferation has been identified in the oral cavity. Hamartoma with ectopic meningothelial elements is a rare condition that has been classically described occurring in the scalp. These lesions are characterized by bland round to spindle-shape cells that interdigitate through collagen bundles and express progesterone receptor and epithelial membrane antigen by immunohistochemistry supporting a meningothelial origin.

The receptor tyrosine kinase FLT3 is expressed in myeloid and lymphoid progenitor cells. Activating mutations in FLT3 occur in 25-30% of acute myeloid leukaemia (AML) patients. Most common are internal tandem duplications of sequence (ITD) leading to constitutive FLT3-ITD kinase activity with an altered signalling quality promoting leukaemic cell transformation. Here, we observed the attenuating role of the receptor-like protein tyrosine phosphatase (RPTP) CD45/Ptprc in FLT3 signalling in vivo. Low level expression of this abundant RPTP correlates with a poor prognosis of FLT3-ITD-positive AML patients. To get a further insight into the regulatory role of Ptprc in FLT3-ITD activity in vivo, Ptprc knock-out mice were bred with FLT3-ITD knock-in mice. Inactivation of the Ptprc gene in FLT3-ITD mice resulted in a drastically shortened life span and development of severe monocytosis, a block in B-cell development and anaemia. The myeloproliferative phenotype was associated with extramedullary haematopoiesis, splenohepatomegaly and severe alterations of organ structures. The phenotypic alterations were associated with increased transforming signalling of FLT3-ITD, including activation of its downstream target STAT5. These data reveal the capacity of Ptprc for the regulation of FLT3-ITD signalling activity in vivo. In addition, histopathology and computed tomography (CT) revealed an unexpected bone phenotype; the FLT3-ITD Ptprc-/- mice, but none of the controls, showed pronounced alterations in bone morphology and, in part, apparent features of osteoporosis. In the spleen, ectopic bone formation was observed. The observed bone phenotypes suggest a previously unappreciated capacity of FLT3-ITD (and presumably FLT3) to regulate bone development/remodelling, which is under negative control of CD45/Ptprc.

Heterotopic tissue in the bile duct is a very rare condition. There are a few case reports of heterotopic tissue including gastric and pancreatic cells. However, we could not find any data regarding heterotopic chondroid tissue obstructing the common bile duct in the literature. A 56-year-old woman was admitted to our hospital with complaints of progressive jaundice and pruritus. Laboratory test results were within the normal limits except bilirubin and alkaline phosphatase which were 10.8 mg/dL and 256 IU/L, respectively. Endoscopic retrograde cholangiography (ERCP) and computed tomography (CT) revealed a biliary stricture confined to the upper part of the common bile duct. The patient was operated as soon as the bilirubin level dropped below 5 mg/dL by percutaneous transhepatic cholangiography. Despite the lack of a precise confirmation, the patient was prepared for operation considering the possibility of a malignant biliary tumor since the radiographic mass findings together with hyperbilirubinemia were highly suggestive of malignancy. The patient underwent total extrahepatic bile duct resection and Roux-en-Y hepaticojejunostomy, and was discharged without any postoperative complications. Histologic examination of the bile duct and the nodule revealed the presence of a well-demarcated chondroid tissue within the subepithelial connective tissue. Herein, we presented the first case, to our knowledge, of heterotopic chondroid tissue of the common bile duct in the literature. It is a benign condition that should be considered in the differential diagnosis of stricture and mass-forming lesions of the bile duct.

Malignant transformation of ectopic pancreas tissue is a diagnostic challenge as clinical symptoms and radiographic features of these tumors are non-specific. Given the rarity of these lesions, it is usually neither suspected nor included in the diagnostic workup of different tumors. We conducted a comprehensive literature review regarding malignancy arising from ectopic pancreas for a better understanding of its frequency, clinicopathological features, and prognosis. A literature search was performed in three major databases: PubMed, Cochrane, and Web of Science. Fifty-four well-documented cases of malignant ectopic pancreas were identified in the published literature. Our analysis provided the following observations: (1) there was a slight predominance of males over females; (2) most patients with malignant transformation of ectopic pancreas were middle-aged; (3) most commonly, the tumor was located in the stomach; (4) most tumors were adenocarcinomas; (5) most frequently, the malignancy arose within a type I heterotopia according to Heinrich classification; (6) macroscopically, a subepithelial-like appearance was most frequently observed; and (7) improved prognosis for ectopic pancreatic malignancies in comparison with reported survival data for orthotopic pancreatic cancer. Even if the majority of cases of ectopic pancreas are incidental findings and malignant transformation is a rare event, pancreatic heterotopy should be considered as a source of potentially malignant lesions.

Background An epithelial cyst in an intrapancreatic accessory spleen (ECIPAS) is rare. We report a case of ECIPAS that was treated with robot-assisted distal pancreatectomy with splenectomy. Case presentation The case was a 59-year-old woman who was referred to our hospital after a pancreatic tail tumor was found on computed tomography prior to surgery for small bowel obstruction at another hospital. A cystic lesion in the pancreatic tail was discovered and evaluated by magnetic resonance imaging and endoscopic ultrasonography. Based on clinical and radiological features, mucinous cystic neoplasm was included in the differential diagnosis. The patient underwent robot-assisted distal pancreatectomy with splenectomy. The postoperative course was uneventful. Pathological evaluation revealed a 20-mm ECIPAS in the pancreatic tail. Conclusions If a pancreatic tail tumor is present, ECIPAS should be included in the differential diagnosis. However, preoperative diagnosis is difficult, and a definitive diagnosis is often not obtained until after surgery. Surgery should be minimally invasive. Laparoscopic distal pancreatectomy has become a standard surgical procedure because it is minimally invasive. Robot-assisted surgery is not only minimally invasive, but also advantageous, because it has a stereoscopic magnifying effect and allows the forceps to move smoothly. Robot-assisted distal pancreatectomy may be a good option, when performing surgery for a pancreatic tail tumor.

Endometriosis is a common disorder of unknown etiology, and non-surgical therapies are still a challenge. To understand the pathogenesis and preclinical testing of drugs for endometriosis, animal models are highly desirous. Herein, we carried out longitudinal characterization of a mouse model for endometriosis where uterine tissue was transplanted onto the intestinal mesentery. During the course of lesion development from day 15 to 60 post-induction, the ectopic endometrium became pale, fluid-filled and the animals developed peritoneal adhesions. Most lesions resembled a well-differentiated type of endometriosis and ~13% of animals had mixed type of lesions. There was extensive stromal compaction in the ectopic tissue. During the progression of endometriosis, there was increased proliferation of epithelial and stromal cells as evident by PCNA staining. Cyp19a1 (aromatase) mRNA was detected in the ectopic lesions on day 15 and 30 post-induction of endometriosis, by day 60 the expression was reduced. As compared to the control endometrium, the mRNA levels of Esr1 progressively reduced while the levels of inflammation associated genes (Esr2, Ifng, Tnf and Il1b) increased in the ectopic lesions. Infiltration of macrophages and polymorphonuclear leucocytes was also observed in the ectopic lesions indicative of inflammation. As compared to control, there was no change in levels of Cytokeratin and E-cadherin in the epithelial cells of ectopic endometrium. We did not observe excessive collagen deposition or α -SMA positive myofibroblasts in the stroma of the ectopic endometrium. Thus, epithelial-to-mesenchymal transition and fibrosis are not detected in the mouse model of endometriosis. Our results show that the mouse model of endometriosis mimics some but not all the features of human endometriosis.

Background Hepatic adrenal ectopia is a common clinical diagnosis, whereas adrenal tumors developed from hepatic adrenal ectopia are rare. Hepatic adrenal tumors are easily misdiagnosed as hepatic carcinoma and frequently treated by unnecessary operations. Case presentation A 50-year-old female patient was hospitalized due to B-ultrasonic detection of “right focal liver lesions.” After hospitalization, enhanced CT examination was performed. A 2.2 cm × 1.8 cm tumor was found in the seventh section of the right liver, as indicated by obvious enhancement of the arterial phase and low density during the portal vein and delay stages. Enhanced MRI examination detected a 2.0 cm × 1.8 cm tumor on the right liver, which was considered a “primary hepatic carcinoma”. The patient was treated by open hepatectomy and recovered well after the operation. The postoperative pathological diagnosis was hepatic adrenal adenoma. No relapse was observed through the 1-year follow-up visit. Conclusions According to imaging manifestations, pathological immunohistochemical treatment, alpha fetoprotein (AFP) and clinical features, hepatic adrenal tumors should be considered in the diagnosis of hepatic carcinoma to prevent misdiagnosis. Hepatic adrenal tumors should be ruled out during the diagnosis to avoid unnecessary operation.

Ektopien primar weiblicher Genitalgewebe sind kein seltenes Ereignis im diagnostischen Alltag. Sie lassen sich uberwiegend aus wahrend der Embryonalentwicklung versprengten oder rudimentaren Zellen des Paranephros (Muller-Gangsystem) ableiten, aber auch gonadale, mesonephrische oder dem Mesothel abstammende Gewebe konnen an ortsuntypischen Stellen des weiblichen Genitales und in extragenitalen Lokalisationen vorkommen. Sie konnen Ursache gut- und bosartiger tumoroser Lasionen sein. Die vorliegende Ubersicht soll einen Uberblick moglicher Gewebeektopien geben und zum besseren entwicklungsgeschichtlichen Verstandnis tumoroser Erkrankungen des weiblichen Genitaltraktes beitragen. Auch die Ektopien primar extragenitaler Gewebe im weiblichen Genitaltrakt werden dargestellt.

Background Epibulbar complex choristoma, a rare congenital epibulbar tumor, has many diverse forms. Reviewing the literature, it can present clinically as either a circumferential or isolated epibulbar mass, limbal tumor, lateral canthal mass, aggregate of ectopic cilia in the upper eyelid, eyelid mass mimicking chalazion, or lacrimal caruncle mass. The management depends on the extent of involvement, the risk of amblyopia, and cosmetic concerns. Here, we report an atypical presentation of epibulbar complex choristoma with simultaneous eyelid involvement. Case presentation A 1-month-old full-term boy was brought to our clinic with congenital epibulbar mass of the right eye with simultaneous eyelid involvement. Dilated fundus examination was unremarkable. Survey for linear nevus sebaceous Jadassohn was negative. Due to concerns of possible amblyopia and cosmetics, lamellar keratectomy, sclerotomy, and conjunctivoplasty were performed to remove the epibulbar lesion. The eyelid defect was reconstructed with 6–0 Vicryl sutures. Histopathological examination reported complex choristoma. Upon three-year follow-up, low astigmatism and favorable cosmetics results were achieved. Conclusions Congenital complex choristoma can present clinically as an epibulbar mass with eyelid involvement. The management depends on the extent of involvement, the risk of amblyopia, and cosmetic concerns. The method of eyelid reconstruction should be tailored according to the residual eyelid defect.

We carried out a comparative study of the features of osteogenesis from the progenitor osteogenic periosteal cells in rabbit and human. At the initial stages, high osteogenic potential of both human and rabbit periosteal cells was observed. However, at the later stages, the cell response favors resorption of the new bone tissue formed from periosteal cells in rabbits, but does not affect the bone tissue formed from human progenitor osteogenic periosteal cells. These functional characteristics of rabbit periosteal cells should be considered when planning the experiment.

BACKGROUND: Essential tremor (ET) is among the most common neurological diseases. Postmortem studies have noted a series of pathological changes in the ET cerebellum. Heterotopic Purkinje cells (PCs) are those whose cell body is mis-localized in the molecular layer. In neurodegenerative settings, these are viewed as a marker of the progression of neuronal degeneration. OBJECTIVE: We (1) quantify heterotopias in ET cases vs. controls, (2) compare ET cases to other cerebellar degenerative conditions (Spinocerebellar ataxia [SCA] 1, 2, 3, and 6), (3) compare these SCAs to one another, and (4) assess heterotopia within the context of associated PC loss in each disease. METHODS: Heterotopic PCs were quantified using a standard LH&E stained section of neocerebellum. Counts were normalized to PC layer length (n-heterotopia count). It is also valuable to consider PC counts when assessing heterotopia, as loss of PCs extends both to normally located as well as heterotopic PCs. Therefore, we divided n-heterotopias by PC counts. RESULTS: There were 96 brains (43 ET, 31 SCA [12 SCA1, 7 SCA2, 7 SCA3, 5 SCA6] and 22 controls). The median number of n-heterotopias in ET cases was two times higher than that of controls (2.6 vs. 1.2, p

Accessory spleens (ASs), ectopic splenic tissue at intrapancreatic and extrapancreatic sites, rarely contain epidermoid cysts. Our aim was to analyze the incidence of epidermoid cysts in ASs and perform an immunohistochemical analysis of its epithelial lining. We included in the study 148 ASs from 135 patients, for which pathological data were available. Eleven were intrapancreatic ASs (IPASs) and 137 were extrapancreatic ASs (EPASs). Six of the eleven (55%) IPASs contained epidermoid cysts, but they were not detected in EPASs. Immunohistochemical analysis showed that both the superficial/luminal and basal layer of the epithelial lining of epidermoid cysts in IPASs are negative for MUC2, MUC5AC, MUC6, WT-1, calretinin, thrombomodulin, uroplakin-II, and uroplakin-III. The superficial/luminal layer was positive for MUC4, CK7, and CA19-9 in all cases (100%), for CEA and HBME-1 in three cases (50%), and for MUC1, CK5/6, and CK20 in two cases (33%). The superficial/luminal layer was negative for p63 and D2-40 in all cases. The basal layer was positive for MUC1, CK5/6, p63, and HBME-1 in all six cases (100%), for CK7 and D2-40 in two cases (33%), and for CEA in one case (17%). The basal layer was negative for MUC4, CK20, and CA19-9 in all cases. Epidermoid cysts are a characteristic feature of IPASs but not of EPASs. Immunohistochemical analysis showed that the epithelial lining of epidermoid cysts in IPASs has a mixed character of glandular, squamous, mesothelial, and urothelial epithelium.

Endometriosis is a common gynaecological disorder of unknown aetiology. Among the several factors, estrogen has been implicated as a causative factor in endometriosis. In the present study using mouse model, we assessed the role of estrogen in the initial implantation and growth of endometrium in ectopic locations. Uterine tissues from green fluorescent protein (GFP) mice were transplanted in to the peritoneum of wild type mice in presence and absence of estrogen. As compared to untreated controls, the implantation of uterine tissue at ectopic locations was higher when estrogen was administered to both host and donor animals. However, this effect was not sustained as lesions regressed within 14 days of treatment. Irrespective of the treatment, peritoneal adipose was the most preferred site of lesion establishment. The lesions did not have typical features of the endometriosis (presence of glands and stroma) even after estrogen treatment and the ectopic tissue underwent regression by apoptosis irrespective of treatment. Since estrogen promotes implantation of endometrial tissue to ectopic locations but failure of these ectopic lesions to grow and sustain even in high estrogenic environment we propose that estrogen is necessary but not sufficient to sustain endometriosis.

Perforation of jejunal diverticulum is a rare complication. Here, we report a case of jejunal diverticulum penetration with surrounding ectopic pancreas. An 83-year-old female patient was admitted to our department with acute onset of severe abdominal pain lasting for half a day. Abdominal computed tomography showed outpouching of the small intestine that contained air/fluid, with multiple surrounding air bubbles in the mesentery of the small intestine. She was diagnosed with penetration of the small intestine, and an emergency laparotomy was indicated. The penetrated jejunal diverticulum was identified ~20-cm distal to the ligament of Treitz. Partial resection of the jejunum was performed, and her postoperative course was uneventful. The pathological findings confirmed diverticulum penetration into the mesentery and severe inflammation at the site, with surrounding ectopic pancreas. Furthermore, the pancreatic ducts were opened through the penetrated diverticulum. This rare case shows that the ectopic pancreas might have caused penetration of jejunal diverticulum owing to the pancreatic duct opening through the diverticulum.

Heterotopic gastrointestinal cyst of the oral cavity is a rare congenital lesion that may arise from ectopic undifferentiated endodermal cells. Imaging, particularly MRI, is useful for surgical planning. On MRI, the cysts typically demonstrate high signal on T2-weighted sequences and variable signal on T1-weighted sequences, which can resemble other conditions, such as dermoids. On histology, the appearance of these lesions can be variable, and may include stratified squamous, simple and ciliated columnar, as well as foveolar and intestinal-type epithelia, often surrounding by smooth muscle. Complete surgical excision is the treatment of choice.

Ectopic pancreas also known as heterotopic pancreas (HP) is a rare congenital anomaly, mainly found as incidental finding during autopsy or abdominal exploration for an other condition. Incidence rate is probably underestimated as patients are mostly asymptomatic; otherwise, it is capable of producing symptoms depending on its location, size, often appearing in the 4th-to-6th decades. Complications such as inflammation, obstruction, bleeding, and malignancy degeneration must be considered. Pediatrics cases are very rare, generally concerning HP within Meckel's diverticulum, manifesting by gastrointestinal bleeding and intussusception. We report a rare case of jejunum bleeding, due to an isolated HP in a 15-year-old adolescent. Endoscopic and computed tomographic scan were normal, in particular did not found Meckel's diverticulum. Diagnosis and treatment have been apprehended performing a laparoscopic exploration. It is a singular location for HP, predominantly found in upper gastrointestinal tractus. So far, there have been no case reports of jejunal bleeding from ectopic pancreas without Meckel's diverticulum in children.

Background Ectopic pancreas is an infrequent submucosal tumor in the gastrointestinal tract defined as a pancreatic tissue lacking vascular or anatomic continuity with the main body of the pancreas. Ectopic pancreas in the ileum is a rare and often an incidental finding. We report a case of ectopic pancreas in the ileum causing obscure gastrointestinal bleeding and episodes of abdominal pain. Case presentation 59-year-old man with 3 months history of intermittent melena, accompanied by the episodes of abdominal pain in the left upper quadrant and generalized fatigue was admitted to our department. The investigations showed that the patient had a low hemoglobin level, i.e. 10.9 g/dL with hypochromic microcytic anemia pattern seen in complete blood count (MCV 70.2 fl, MCH 21.4 pg). Esophagogastroduodenoscopy and colonoscopy did not reveal any abnormalities. Magnetic resonance enterography revealed a large (2.5 × 2.3 cm) pedunculated polyp in the ileum. Examination by single-balloon enteroscopy revealed a polyp with long pedicle located approximately 1.5 m distal to terminal ileum. Polypectomy was performed. Histopathologic examination stated, that the specimen contained ectopic pancreatic tissue which was involving muscular layer of the ileum. Ectopic pancreatic tissue included acinar cells and cystically dilated secretory ducts without islets of Langerhans. Conclusion Our case report reveals a very rare cause of obscure gastrointestinal bleeding accompanied by the episodes of abdominal pain – an ectopic pancreas located in the ileum.

Invagination of peritoneal or retroperitoneal structures into the intervertebral disc space of the lumbar spine is extremely rare. In this article the imaging features and clinical findings are demonstrated in four patients with intervertebral invagination of intra-abdominal structures. Plain radiographs, CT scans, and MR images showed disruption of the anterior ligamentous complex (ALC) and invagination of various structures into the disc space, including the vena cava, iliac vessels, intestine, the torn redundant anterior longitudinal ligament, retroperitoneal fat, and the psoas muscle. The invaginations occurred at the level of L3-L4 and L4-L5 in one case each and at L5-S1 in two cases. Follow-up imaging in three cases (1, 5, and 8 years respectively) showed the progression of the invagination. These cases demonstrate that a close evaluation of this condition is necessary, particularly for older patients who show disruption or laxity of the ALC, degenerative spondylosis with chronic segmental instability, and osteoporosis of the lower lumbar spine.

Parathyroid gland mediastinal ectopia is an unusual but challenging condition in surgical management of hyperparathyroidism. Posterior mediastinum parathyroid ectopia is rare, and glands need to be removed either with a broad open cervical or thoracic approach. In recent years, several minimally invasive approaches to mediastinal parathyroid glands have been described, but for posterior mediastinum adenomas, proposed techniques are transthoracic. The aim of this paper is to describe, to our best knowledge for the first time, a standardized pure endoscopic cervical technique to approach posterior mediastinal parathyroid adenomas which we have used in three patients. The technique was applied in three patients which excellent surgical, postoperative, and cosmetic results. Endoscopic prevertebral approach is a feasible, sure and inexpensive standardized pure endoscopic cervical approach to posterior mediastinal parathyroid adenomas, which may result in a less aggressive surgical option when compared with thoracic approaches.

In salamanders, grafting of a left limb blastema onto a right limb stump yields regeneration of three limbs, the normal limb and two 'supernumerary' limbs. This experiment and other research have shown that the juxtaposition of anterior and posterior limb tissue plus innervation are necessary and sufficient to induce complete limb regeneration in salamanders. However, the cellular and molecular basis of the requirement for anterior-posterior tissue interactions were unknown. Here we have clarified the molecular basis of the requirement for both anterior and posterior tissue during limb regeneration and supernumerary limb formation in axolotls (Ambystoma mexicanum). We show that the two tissues provide complementary cross-inductive signals that are required for limb outgrowth. A blastema composed solely of anterior tissue normally regresses rather than forming a limb, but activation of hedgehog (HH) signalling was sufficient to drive regeneration of an anterior blastema to completion owing to its ability to maintain fibroblast growth factor (FGF) expression, the key signalling activity responsible for blastema outgrowth. In blastemas composed solely of posterior tissue, HH signalling was not sufficient to drive regeneration; however, ectopic expression of FGF8 together with endogenous HH signalling was sufficient. In axolotls, FGF8 is expressed only in the anterior mesenchyme and maintenance of its expression depends on sonic hedgehog (SHH) signalling from posterior tissue. Together, our findings identify key anteriorly and posteriorly localized signals that promote limb regeneration and show that these single factors are sufficient to drive non-regenerating blastemas to complete regeneration with full elaboration of skeletal elements.

Two cases where aberrant tissue was attached to the lower lip mimicking the inferior labial frenum were reported. The frenum-like tissue extended from the gingival margin between the lower left deciduous central and lateral incisors in case 1 and between the lower right deciduous central and lateral incisors in case 2, to the dry lower lip. Histologically, the resected specimen was regarded as normal oral mucosa covered with stratified squamous epithelium, without a clear amniotic band. The frenum-like tissue of the lower lip found in both our patients was diagnosed as a category of oral synechiae, of unknown origin.

Cervical chondrocutaneous branchial remnants are rare congenital choristomas. These lesions contain a cartilage core surrounded by skin with adnexal structures and subcutaneous fat. Correspondingly, on ultrasound there is a tubular hypoechoic core surrounded by hyperechoic, while on CT there is central intermediate attenuation surrounded by fat attenuation tissues. These features are exemplified in this sine qua non radiology-pathology correlation article. Management includes complete surgical resection and evaluating for potential associated anomalies, such as other branchial apparatus anomalies, as well as cardiac anomalies.

Esophageal adenocarcinoma arising from ectopic gastric mucosa (EGM) is extremely rare. We describe here two Japanese patients with adenocarcinoma of the cervical esophagus arising from EGM. Case 1 is a 62-year-old man who had slightly red EGM in the cervical esophagus on upper gastrointestinal endoscopy (UGE). Because the biopsy showed atypical glands that were suspicious for adenocarcinoma, endoscopic submucosal dissection was performed. Histopathological examination revealed that the lesion was a well-differentiated adenocarcinoma (pT1a MM). Lymphovascular invasion was absent, and the margins were free from carcinoma. Case 2 is a 57-year-old man who had an elevated lesion with a bleeding tendency in an area of EGM in the cervical esophagus on UGE. Adenocarcinoma was diagnosed in the biopsy. Because of the presence of enlarged lymph nodes (#106recL), preoperative chemoradiotherapy was performed to reduce the size of the adenocarcinoma and lymph nodes prior to resection of the cervical esophagus and reconstruction with free jejunal grafts. Histopathological examination revealed moderately differentiated adenocarcinoma (0-I, pT2N1M0, pStage II). In both cases, adenocarcinoma was surrounded by EGM, which led to the diagnosis of EGM-derived esophageal adenocarcinoma. Here, we report its immunohistochemical characteristics in the present cases and discuss the histogenesis.

Contains fulltext : 172796.pdf (Publisher’s version ) (Closed access) The clinical significance of papillary or follicular thyroid tissue incidentally discovered in cervical lymph nodes during pathological assessment of neck dissections for non-thyroid cancers of the upper aero-digestive tract is critically reviewed. Special emphasis is given to controversies over normal-looking, nodal, thyroid follicles. Arguments for and against the benign nature of these follicles are considered together with processes that could be involved in their formation. The admittedly limited evidence suggests that benign, thyroid follicular inclusions rarely occur in cervical lymph nodes. Histological criteria that could be helpful in recognizing the inclusions, which include assessing their extent in conjunction with the size of the node, are discussed. Finally, an algorithm based on collaboration between specialists, correlating histological findings with imaging and loco-regional control of the upper aero-digestive tract cancer, is suggested for the management of patients with incidentally discovered, nodal thyroid tissue.