Congenital orbital respiratory epithelial cyst with anomalous maxillary antrum in the absence of bony defect.

A 23-year-old female symptomatic with unilateral proptosis with superior globe displacement and hypertropia was found to have a multiloculated cystic lesion with bony lining arising from the inferomedial orbit. The adjacent maxillary sinus was markedly smaller on the affected side, with no defect of the wall on radiographic or intraoperative examination. Surgical excision and histological analysis of the lesion demonstrated an orbital respiratory epithelial cyst. A literature review of congenital orbital respiratory epithelial cysts is presented. To our knowledge, this is the first published case of choristomatous orbital respiratory epithelial cyst associated with ossification, and a primary or secondary anomaly of the adjacent sinus without bony defect.

Adrenal cysts of lymphatic origin: A clinical and pathological study of six cases and systematic literature review.

Adrenal cysts are rare, benign, and usually asymptomatic, being detected as an incidental finding on imaging methods. Adrenal Cysts of Lymphatic Origin (ACLO) and Adrenal Lymphangiomas (AL) are types of endothelial cyst and are the most prevalent subtype in this series. This study aims to present a single institutional experience of these rare cysts and compare their features with those found in the review of existing literature on ACLO and AL. Overall, thirteen cases of adrenal cysts were diagnosed and surgically excised during the study period, onto which we performed immunohistochemistry using a panel of antibodies (CD31, CD34, Pan Cytokeratin AE-1/AE-3, Factor VII, D2-40, and ERG). Four cases of ACLO and two AL were found. The lesions predominantly affected right adrenal, and the majority of patients were middle-age females, of Caucasian ethnicity, and asymptomatic. In our literature review, we found 108 cases of ACLO/AL from 57 articles with similar sex and age distribution. The diagnosis and subclassification of adrenal cysts are challenging, and there is a significant overlapping between the definition of ACLO and AL.

Epithelial cyst arising in an intrapancreatic accessory spleen: a case report of robotic surgery and review of minimally invasive treatment.

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.

Aberrant endocytosis leads to the loss of normal mitotic spindle orientation during epithelial glandular morphogenesis.

Epithelial cells form tissues with many functions, including secretion and environmental separation and protection. Glandular epithelial tissues comprise cysts and tubules that are formed from a polarized, single-epithelial cell layer surrounding a central, fluid-filled lumen. The pathways regulating key processes in epithelial tissue morphogenesis such as mitotic spindle formation are incompletely understood, but are important to investigate, as their dysregulation is a signature of epithelial tumors. Here, we describe a signaling axis that manifests in a defect in mitotic spindle orientation during epithelial growth and cystogenesis. We found that activation of the small GTPase ADP-ribosylation factor 6 (ARF6) results in the sustained internalization of cell-surface components such as the cMet receptor and the cell-adhesion molecule E-cadherin. The spindle orientation defect arising from elevated levels of ARF6-GTP required an increase in cMet endocytosis, but was independent of E-cadherin internalization or elevated extracellular signal-regulated kinase (ERK) activity resulting from internalized receptor signaling on endosomes. Misorientation of the mitotic spindle resulted in the development of epithelial cysts with structural abnormalities, the most conspicuous of which was the presence of multiple intercellular lumens. Abnormal mitotic spindle orientation was necessary but insufficient to disrupt glandular development, as blocking the strong prosurvival signal resulting from ERK hyperactivation yielded structurally normal cysts despite continued manifestation of spindle orientation defects. Our findings highlight a previously unknown link between ARF6 activation, cMet receptor internalization, and mitotic spindle orientation during epithelial glandular morphogenesis.

Histological and immunohistochemical analyses of splenic epidermoid cysts.

Splenic epidermoid cyst (SEC) is a rare condition. We aimed to evaluate the immunohistochemical profiles of the epithelial lining of SECs. A total of 7 SEC cases were analyzed: 2 cases involved a monolayered epithelial lining and 5 cases involved a multilayered epithelial lining. Among the multilayered SECs, the superficial/luminal layer showed mucin 4 (MUC4), cytokeratin 5/6 (CK5/6), and CK7 expression in 5 cases (100%); MUC1, carcinoembryonic antigen (CEA), CA19-9, and thrombomodulin expression in 4 cases (80%); Wilms' tumor-1 (WT-1) and Hector Battifora mesothelial-1 (HBME-1) expression in 2 cases (40%), but it did not express p63 or D2-40. The basal layer expressed MUC1, CK5/6, p63, and thrombomodulin in 5 cases (100%); CK7 and WT-1 in 4 cases (80%); D2-40 in 3 cases (60%); CA19-9 and HBME-1 in 2 cases (40%) and MUC4 in 1 case (20%) but it did not express CEA. The analysis showed that all cases of multilayered SECs were negative for MUC2, MUC5AC, MUC6, CK20, calretinin, uroplakin-II, and uroplakin-III. Both cases of monolayered SECs expressed CK5/6, CK7, HBME-1, WT-1, and thrombomodulin but not MUC2, MUC4, MUC5AC, MUC6, p63, CEA, CK20, CA19-9, D2-40, uroplakin-II, or uroplakin-III. One case of monolayered SEC expressed MUC1 and calretinin. Our findings indicate that monolayered SECs have mesothelial-like characteristics, whereas multilayered SECs have glandular and squamous-like characteristics besides mesothelial-like characteristics. Furthermore, monolayered SECs may develop from mesothelial inclusion and monolayered SECs develop squamous and glandular metaplasia, which results in multilayered SECs.

α5ß1 integrin mediates pulmonary epithelial cyst formation.

BACKGROUND: Formation of the epithelial cyst involves the establishment of apical-basolateral polarity through a series of cellular interactions that are in part mediated by the extracellular matrix (ECM). We report that in a three-dimensional multi-cellular self-assembly model of lung development, α5 integrin regulates epithelial cyst formation through organization of soluble fibronectin matrix into insoluble fibrils through a process called fibrillogenesis. RESULTS: Dissociated murine embryonic lung cells self-assemble into three-dimensional pulmonary bodies that are dependent on α5ß1 integrin mediated fibrillogenesis for cell-cell mediated self-assembly: compaction and epithelial cyst formation. Knockdown of α5 integrin resulted in a significant increase in another mediator of fibrillogenesis, αV integrin. Compensatory increased expression of another mediator of fibrillogenesis, αV integrin, was not sufficient to normalize epithelial cyst formation. Loss of α5 integrin-mediated fibrillogenesis perturbed the ability of clustered epithelial cells to establish clear polarity, loss of epithelial cell pyramidal shape, and disrupted apical F-actin-rich deposition. Lack of rich central epithelial localization of F-actin cytoskeleton and Podocalyxin suggests that loss of α5 integrin-mediated fibrillogenesis interferes with the normal cytoskeleton organization that facilitates epithelial cysts polarization. CONCLUSIONS: We conclude that lung epithelial cyst formation in development is mediated in part by α5ß1 integrin dependent fibrillogenesis. Developmental Dynamics 246:475-484, 2016. © 2017 Wiley Periodicals, Inc.

Portable OCT-assisted surgical treatment of intracorneal pre-Descemet epithelial cyst: a case report.

BACKGROUND: Intracorneal epithelial cysts are a rare clinical condition that can occur anywhere in the corneal tissue; however, they appear most commonly in the stroma. They are sometimes challenging to treat because of their location, depth, and visual outcomes. Herein, we report a pre-Descemet epithelial cyst that was successfully treated surgically, with guidance from Fourier-domain optical coherence tomography (FD-OCT). CASE PRESENTATION: This interventional case report presents a patient with gradually decreasing vision caused by a pre-Descemet epithelial cyst. A 4-year-old girl with no history of trauma or ocular surgery showed a deep-seated intracorneal cyst in her left eye (8 o'clock corneoscleral area, dissecting into the pre-Descemet cornea). The cyst was threatening the visual axis. An epithelial cyst was diagnosed after drainage on the basis of the cyst contents. We irrigated inside the cyst using 10% trichloroacetic acid (TCA), distilled water, and 1% 5-fluorouracil (5-FU) solutions for chemical cyto-destruction of the lining epithelial cells of the cystic wall. We used a portable FD-OCT during operation to guide this procedure, without perforating the Descemet's membrane and endothelial layer. Recurrence could be prevented after removal of the cystic tissue located in the sclera area outside of the limbus. No recurrence was noted during the 4-year follow-up. CONCLUSION: When treating centrally deep-seated intracorneal epithelial cysts, clinicians must consider recurrence, endothelial damage, and visual outcome. Herein we report the case of a deep-seated, intracorneoscleral epithelial cyst that was completely resolved with chemical cyto-destruction and removal of the intrascleral cystic tissue under the guidance with FD-OCT; thus, endothelial damage could be minimized.

Non-metastatic squamous cell carcinoma within a Rathke's cleft cyst.

INTRODUCTION: Primary intracranial and sellar squamous cell carcinoma is an extremely rare entity, usually caused by malignant transformation of epidermoid cysts, or very rarely other non-malignant epithelial cysts. Malignant transformation of a Rathke's cleft cyst has never been described. CASE DESCRIPTION: We present a 49-year-old male patient who presented with a 3-month history of progressive frontotemporal headaches. Imaging revealed a 1.2 cm cystic pituitary mass consistent with a hemorrhagic Rathke's cleft cyst. The patient underwent trans-sphenoidal resection of the pituitary cyst, and pathologic analysis revealed a squamous cell carcinoma lining a Rathke's cleft cyst. Extensive imaging and otorhinolaryngologic evaluation revealed no primary source for metastasis. CONCLUSIONS: We feel this represents the first case of a patient with a pituitary lesion in which presentation and MRI imaging were consistent with Rathke's cleft cyst, yet histology revealed squamous cell carcinoma in situ.

Epithelial cyst of the superior oblique muscle trochlea.

Most of the congenital orbital cysts are choristomas such as dermoid or epidermoid and only in a few cases they are epithelial. Clinically, they manifest as cystic movable formations mostly localized in the upper temporal quadrant of the orbit. We describe here the case of a 49-year-old man with an orbital cyst localized in the upper-nasal quadrant of the orbit and which was showing signs of a gradual enlargement and progression over the past weeks. Computed tomography revealed a cyst of 1.9 × 1.6 cm in size and located within the trochlea of the upper oblique muscle. The cyst was completely extirpated after orbitotomy performed by superciliary approach. Histopathology revealed a cyst with nonkeratinized cuboidal epithelium. Postoperative course was uneventful, without inflammation signs, and after 5 weeks excellent functional and aesthetic effects were achieved with no iatrogenic alteration of the ocular motility.

Angiomyolipoma With Epithelial Cysts: A Rare but Distinct Variant of Angiomyolipoma.

Renal angiomyolipomas, common benign tumors, can exhibit slow growth in sporadic cases or have aggressive tendencies when linked to genetic conditions like tuberous sclerosis. This case report focuses on the exceptionally rare angiomyolipoma with epithelial cysts (AMLEC) variant, particularly challenging to diagnose due to its scarcity. Describing a 41-year-old woman's case, initially suspected to be renal cell carcinoma during an infertility evaluation, subsequent partial nephrectomy revealed a tumor comprising smooth muscle, blood vessels, and fat, with cystic regions featuring cuboidal linings and a layer devoid of abnormal cell activity. Immunohistochemistry confirmed specific markers within different tumor components, highlighting the diagnostic complexities of AMLEC and emphasizing the crucial role of histopathological examinations in accurate characterizations.

Atypical Presentation of Pilar Cyst Over the Volar Aspect of the Thumb: A Case Report and Review of Literature.

Pilar cysts are derived from the outer layer of the root sheath of hair follicles. They were conventionally thought to arise from hair-bearing skin like the scalp. However, this notion has been refuted recently. Pilar cysts of the hand are extremely rare, with only a few case reports in the literature. We report the case of a 40-year-old male patient, with no known medical co-morbidities, who presented with a swelling over his left thumb. It was occasionally painful, and caused difficulty in grasping objects. Physical examination revealed a 2.5 x 1.5 cm swelling over the volar aspect of the thumb, at the level of the proximal phalanx. MRI revealed the presence of a well-defined cystic lesion superficial to the flexor tendons. The possibility of an epidermal cyst was considered, and the patient was advised surgery in view of his symptoms and progression in the size of the swelling. He underwent excision of the lesion along with a segment of adherent skin. Histopathological examination of the lesion revealed the presence of a pilar cyst. The patient did not have recurrence of symptoms following surgery, and was found to be doing well at the three-year follow-up. This case report urges a re-thinking of the possible origins of pilar cysts from atypical locations.

Fluctuating hypermetropia due to intraocular lens displacement caused by iris pigment epithelial cyst.

Purpose: To report a case of hyperopic shift following lens replacement surgery due to an enlarging iris pigment epithelial (IPE) cyst. Observations: A gentleman presented with reduced visual acuity (Snellen unaided 20/25) 12 months followed lens replacement surgery. Examination revealed a retro-pupillary iris lesion that appeared to be displacing the posterior chamber intraocular lens (IOL) and was causing a hyperopic shift (refraction +2.00). Anterior segment optical coherence tomography imaging confirmed this to be an IPE cyst with a posteriorly displaced IOL body. After observation over 30 months, the IPE cyst spontaneously reduced in size and the IOL returned to a more physiological position. Unaided visual acuity improved to Snellen 20/16 and refraction improved to +0.50. Conclusions and Importance: To our knowledge, an IPE cyst that shows growth following intraocular surgery has not previously been reported. This growth resulted in a hyperopic shift due to posterior displacement of the IOL. This case demonstrates spontaneous regression of the cyst, and suggests that over time these cysts can change in size.

Anterior segment swept-source optical coherence tomography and ultrasound biomicroscopy in iris and ciliary body lesions.

OBJECTIVES: Differentiation of iris and ciliary body lesions as benign or malignant and cystic or solid is important. The aim of this study was to compare anterior segment swept-source optical coherence tomography (AS SS-OCT) and ultrasound biomicroscopy (UBM) findings in iris and ciliary body tumors. RESEARCH DESIGN AND METHODS: Forty-two eyes of 38 cases with iris and ciliary body tumors imaged with UBM and AS SS-OCT between September 2018 and September 2023 were evaluated retrospectively. RESULTS: Of 42 eyes, 14 had melanoma, 14 iris pigment epithelial (IPE) cysts, 7 nevi, 3 Lisch nodules, 2 iris stromal cysts, 1 pars plana cysts, and 1 iris mammillations. An equivalent (100%) visualization of the anterior tumor margin was obtained with both techniques. Compared to AS SS-OCT, UBM was superior for posterior margin visualization in melanocytic tumors and IPE cysts. Bland-Altman plots demonstrated good agreement between UBM and AS SS-OCT for melanocytic tumors < 2.5 mm in base diameter and < 2 mm in thickness. CONCLUSIONS: Although, UBM is the gold standard for ciliary body and iridociliary tumors. AS SS-OCT should be considered as an excellent alternative to UBM, especially in minimally elevated iris lesions.

Renal Angiomylipoma with Epithelial Cyst (AMLEC): A Rare Cystic Variant of Angiomyolipoma.

Teaching Point: AMLEC is a rare subtype of an angiomyolipoma (AML) and not a primary cystic lesion.

A Case Report of a Large Splenic Cyst in a Pediatric Patient.

Splenic cysts in the pediatric population are rare but can present with a range of clinical manifestations. Acute abdominal pain, although uncommon, is a significant presentation that requires prompt evaluation and management. We present the case of an 11-year-old female who presented to the emergency department with severe left upper quadrant abdominal pain of 24 hours' duration. Physical examination revealed tenderness and guarding in the left upper quadrant with a palpable, firm mass. Elevated serum amylase and lipase levels initially raised suspicion of a pancreatic etiology, but further investigations confirmed the presence of a large cystic lesion in the spleen. The patient underwent laparoscopic splenectomy, and the resected specimen confirmed a benign splenic cyst. Postoperatively, she recovered uneventfully and was discharged with appropriate follow-up plans. This case report underscores the importance of early recognition and prompt surgical intervention in managing splenic cysts in pediatric patients. The diverse etiologies and pathophysiological mechanisms of splenic cysts necessitate a comprehensive diagnostic approach.

A rare case of a branchial cyst with calcification: A case report.

Branchial cysts are relatively rare lesions with lymphoid tissue in the underlying epithelium of the cyst wall. The present study describes the case of a branchial cyst with keratinization and calcification that occurred in the right submandibular region, along with a review of the literature. A 49-year-old female patient presented with a complaint of swelling in the right submandibular region. Computed tomography revealed a well-defined, cystic lesion located anterior to the sternocleidomastoid muscle, outside the hyoid bone, and in front of the submandibular gland. The cystic cavity presented an opaque image suggestive of calcification. Magnetic resonance imaging showed high-intensity lesions on both T2-weighted and short-τ inversion recovery images on the anterior margin of the right sternocleidomastoid muscle, just below the platysma muscle, with a clear demarcation from the surrounding tissue, and posterior compression and flattening of the submandibular gland. Cystectomy was performed under general anesthesia, and histopathological examination confirmed the diagnosis of branchial cyst with keratinized and calcified substances. The patient recovered well and had no complications or recurrence at ~2-year follow-up. This case highlights the rare occurrence of a branchial cyst containing calcification in the cystic cavity and provides a literature review of the factors contributing to the calcification.

Unusual cause of chronic abdominal pain in a 17-year-old female: a case report of an epithelial adrenal cyst.

Adrenal cysts are rare lesions that make up 4% of all adrenal masses. They are often found incidentally during imaging or surgery and can vary in symptoms depending on their size and location; with cysts < 10 cm often being asymptomatic. These cysts are more common in adults in their 30s or 60s and can be divided into four histological subtypes, with epithelial cysts being less common. We present a case of a 17-year-old female who experienced recurrent right flank pain for a year. Ultrasonography revealed a cystic mass on the right kidney, and computerized tomography showed a well-defined suprarenal cyst measuring ~8 cm misdiagnosed as mesenteric cyst. However, during laparoscopy, an adrenal cyst was discovered, which was excised along with the right adrenal gland. Histopathology confirmed the diagnosis of an epithelial adrenal cyst. This case highlights the occurrence of adrenal cysts in younger individuals and the uncertainty of preoperative diagnosis.

Case Report: Adrenal Epithelial Cyst in an 11-Year-Old Leptailurus Serval.

Case Description: A serval (Leptailurus serval) presented for progressive enlargement of the right adrenal gland, which had been found incidentally on abdominal ultrasound 2 years previously and upon subsequent ultrasound examinations enlarged progressively from 1.26 to 1.43 cm. Clinical Findings: Clinical signs had not been recorded by the zookeeper, however, progressive weight gain and lethargy were reported. Computed tomography (CT) confirmed the presence of a right caudal pole adrenal mass measuring 1.8 cm. Treatment and Outcome: The right adrenal with associated mass was surgically resected via a ventral midline laparotomy that included the resection of the right phrenicoabdominal vein in association with the mass. Histopathological examination identified the mass as an epithelial cyst. Chromogranin A staining for a possible pheochromocytoma was performed but was negative. The serval recovered at the zoo without complication and has been on display for 18 months. Clinical Relevance: Epithelial adrenal cysts have not been previously reported in felids. An adrenal cyst should be included on the differential list for any animal with an enlarged, slowly growing adrenal gland mass with non-specific clinical signs.