True Adrenal Mesothelial Cyst in a Patient with Flank Pain and Hematuria

True mesothelial (epithelial) cysts in the adrenal gland are rare lesions. They represent 9% of adrenal cysts and are much less common than vascular adrenal cysts. We report a case of a true adrenal mesothelial cyst in a patient with flank pain and hematuria that was diagnosed on imaging as a renal cyst. Immunohistochemical studies were performed to investigate the nature of the cyst lining. The positive immunostains for calretinin and WT-1 lend support to the postulate of Medeiros et al nearly 20 years ago of a mesothelial origin for these cysts. The clinical presentation and salient radiologic and pathologic features are described.     

Mesothelial cyst of the adrenal gland

We report a case of a mesothelial adrenal cyst which was incidentally discovered in a 38-year old woman. The diagnosis was confirmed by an immunohistochemistry study. Adrenal cysts are rare. They occur more commonly in female patients during the 4th and 5th decades and are usually asymptomatic. Adrenal cysts are divided into four groups : parasitic, epithelial, endothelial cysts and pseudocysts. The management is still discussed : follow-up, surgical resection or fine-needle aspiration.     

Epithelial-lined (true) cyst of the adrenal gland: a case report

A case of an epithelial-lined (true) adrenal cyst is reported. Although over 300 adrenal cysts have been reported in the literature, true cysts are rare. In this case, a 4.0 cm cyst lined by cuboidal to flattened cells with bland cytologic features was incidentally found at autopsy. Immunologic studies performed on formalin-fixed, paraffin-embedded sections demonstrated that the cells expressed keratins (AE1/AE3+, CAM 5.2+, and MAK-6+) and were negative for epithelial membrane antigen, vimentin, factor VIII, and desmin. Normal adrenal cortical and medullary cells did not express keratins, suggesting that the cyst lining was not derived from either adrenal cortex or medulla. A mesothelial origin, with a pathogenesis analogous to the formation of primary cysts of the spleen, is proposed.     

Cystic lymphangiomatous lesions of the adrenal gland: A clinicopathological study of 37 cases including previously unreported cysts with papillary endothelial proliferation

Published data regarding lymphangiomatous cysts of the adrenal glands (also known as adrenal cystic lymphangiomas) are limited to case reports and a few small case series. We analyzed the clinicopathologic features and histomorphologic spectrum of 37 cases of adrenal cystic lymphangiomatous lesions. There were 26 females and 11 males ranging from 12 to 67 years old (median, 34 years). Twenty two lesions (59.5%) were diagnosed incidentally on imaging studies for unrelated causes, while 15 cases (40.5%) were symptomatic: 8 patients presented with abdominal or flank pain and 7 patients presented with arterial hypertension. Clinically, 4 lesions (10.8%) were reported to have concurrent hormonal hypersecretion. Follow-up data were available for 23 patients (62.2%), ranging from 6 to 156 months (median, 52 mo). One of the 22 patients showed local recurrence at 12 months after partial adrenalectomy. The median size of the adrenal lymphangiomatous cysts was 4.5 cm (range, 1.5 to 10 cm). Based on the histopathologic findings these lesions were grouped into three, morphologically distinct types: typical multicystic lymphatic malformation (n = 16), typical unilocular lymphangiomatous cyst (n = 14) and lymphangiomatous cyst with papillary endothelial proliferation (n = 7). The median patient age of the first group was significantly higher than that of the other groups and calcifications in these cysts were more common than in the other two groups. The unilocular lymphangiomatous cysts were more frequently associated with a history of previous intra-abdominal surgical procedures and/or inflammatory processes than the other two groups. Cysts with papillary endothelial proliferation were significantly larger than other cysts and shared some microscopic features with a vascular neoplasm known as papillary intralymphatic angioendothelioma (PILA). In conclusion, adrenal lymphangiomatous cysts are usually asymptomatic, incidentally diagnosed lesions with a female predominance. They may imitate other adrenal tumors, both radiologically and clinically. Despite being non-functioning lesions, they should be considered as a possible cause of pseudopheochromocytoma. Although most adrenal lymphangiomatous cysts seem to be non-neoplastic, vascular abnormalities (malformations or lymphangiectasias), those with papillary endothelial proliferations may represent true neoplastic lesions.     

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.     

Adrenal epithelial cyst: a case report

Epithelial-lined or true cysts of the adrenal gland are exceedingly rare accounting for only 9% of all adrenal cysts. A 55-year-old woman with a medical history for cholecystectomy and liver hydatidosis presented with an 8-month history of abdominal pain in the upper left quadrant. Physical examination was unremarkable and laboratory tests were within normal range. Computed tomography displayed a homogeneous and finely calcified cystic mass of the left adrenal gland measuring 12 x 10 cm. A hydatid cyst of the adrenal gland was suspected preoperatively and the patient underwent uneventful excision of the cyst. Histological examination of the surgical specimen revealed that the cyst wall was lined by cuboidal to flattened cells with bland cytologic features. Immuohistochemically, the cyst lining expressed intensely cytokeratin but was negative for CD 34 and calretinin. The final pathological diagnosis was epithelial adrenal cyst.     

Retroperitoneale Zysten, die Nebennierenzysten imitieren können

Adrenal cysts are uncommon lesions and most of them are found incidentally during abdominal imaging. We report on two benign extraadrenal lesions mimicking adrenal tumors in abdominal imaging. The histopathological investigation of the lesions revealed a foregut duplication cyst of the lesser gastric curvature and an epithelial inclusion cyst (epidermoid cyst) in an intrapancreatic accessory spleen respectively.     

Multilocular bronchogenic cyst of the bilateral adrenal: report of a rare case and review of literature

Purpose: Bronchogenic cysts are rare benign congenital anomalies, originating from the embryonic foregut ventral segment. Adrenal bronchogenic cyst is a rare form of this anomaly. One extremely rare case of bilateral adrenal multilocular bronchogenic cyst in our hospital was reported and the relevant literatures were reviewed. Significant findings: A 51-year-old man suffered from an intermittent vague headache, fatigue and hypertension history for 2 years, which were gradually worsened in a week. Imaging tests showed bilateral suprarenal mass and left renal cysts. After underwent two retroperitoneal laparoscopic adrenal gland tumor separately, they were all proved to be both the multilocular bronchogenic cyst located in bilateral adrenal gland by histopathological examination. Conclusions: This report confirms the bronchogenic cyst that can be involved bilateral joint in the adrenal gland. And we demonstrated retroperitoneoscopic surgical management is effective in the treatment of the disease.     

Adrenal Cystic Lesions: A Clinicopathological Analysis of 25 Cases with Proposed Histogenesis and Review of the Literature

Adrenal cystic lesions are uncommon and we analyzed clinical and pathologic features of 25 such cases from a single institute over 23 years. There were 16 pseudocysts, eight endothelial cysts, and one epithelial cyst. Seven of eight endothelial cysts were confirmed to be lymphangiomatous by D2-40 immunostaining. We suggest that pseudocysts and endothelial cysts may have different histogenesis. The proposed mesothelial origin of adrenal epithelial cyst cannot be confirmed in our example. Seven adrenal pseudocysts were associated with tumor, including two pheochromocytomas, one neuroblastoma, one adrenal cortical carcinoma, one adrenal cortical adenoma, one myelolipoma, and one schwannoma. The distinction of true cystic lesion from cystic neoplasm is important and requires thorough sampling of the specimens.     

Mesothelial cyst of the adrenal gland

A case of mesothelium-lined cyst of the adrenal gland is reported. Although more than 300 adrenal cysts have been reported in the literature, epithelial cell-lined cysts are rare and comprise only 9% of the cases. An adrenal cyst was found, the lining cells of which had features consistent with mesothelial cells. The inner surtace of the cyst was lined by a single layer of cuboidal cells, which showed alcian blue positive cellular outline. Immunohistochemically, the lining cells were positively stained for keratin, epithelial membrane antigen and CA-125. Electron microscopic examination revealed many long complex microvilli and desmosomes in the lining cells. There was a basal lamina beneath the cell layer. These morphological and immunohistochemical findings indicated that the lining cell is derived from mesothelium.     

Cystic lesions of the retrorectal space

Cysts of the retrorectal space comprise a heterogeneous group of rare lesions. Most develop from embryological remnants and include tailgut cysts, dermoid cysts, rectal duplication cysts, anal canal duplication cysts, sacrococcygeal teratomas and anterior meningocoele. Tailgut cyst is the most common cyst of developmental origin, usually presenting as a multilocular cystic mass with mucoid content and lined by multiple epithelial types. Compared with tailgut cysts, rectal duplication cysts display all layers of the large bowel wall including a well-defined muscularis propria. Retrorectal cysts of non-developmental origin are far less common and represent lesions that either infrequently involve the retrorectal space or undergo extensive cystic change. This review provides an overview of the various histological types of cystic lesions of the retrorectal space, divided into cysts of developmental origin and those of non-developmental origin. A practical pathological and multidisciplinary approach to diagnosing these lesions is presented.     

Retroperitoneal bronchogenic cyst presenting as an adrenal mass

Subdiaphragmatic bronchogenic cysts are rare, and those located retroperitoneally are exceptional. A review of the English-language literature revealed only three reported cases. We describe an additional case of a retroperitoneal bronchogenic cyst that presented uniquely as a symptomatic adrenal mass and discuss the cases of subdiaphragmatic bronchogenic cysts reported in the English-language literature.     

肾上腺囊肿的误诊分析

目的提高对肾上腺囊肿的诊断水平。方法回顾性分析9例经手术和病理证实被误诊的肾上腺囊肿的临床资料,复习文献并讨论肾上腺囊肿的误诊原因。结果误诊原因主要有四点：①对肾上腺囊肿的认识和重视程度不足;②B超作为初步诊断对术前诊断的影响较大;③B超和CT本身的局限性,对病灶来源判断不清;④部分肾上腺囊肿CT值较高,使得对病灶性质造成误判。结论提高对肾上腺囊肿的认识和重视程度对减少误诊率非常重要,合理地联合应用B超、CT、MRI能显著提高肾上腺囊肿的诊断正确率。     

Mǔllerian cyst of the mesentery: A case report of an unusual location

Mǔllerian cysts or paramesonephric cysts arise from the fused embryonic ducts, which typically regress in the uterus. These cysts are usually located paravertebrally. We present an unusual case of a Mǔllerian cyst developing within the mesentery of the ileocecum that was successfully resected. The patient presented to our surgical unit with abdominal pain and swelling. She underwent all the necessary tests to rule out other pathologies before she underwent right hemicolectomy. The patient was discharged without complications. Histopathology confirmed the presence of female reproductive tract epithelium, which was conclusive of a Mǔllerian cyst or paramesonephric cyst. Mǔllerian cysts are rarely malignant, and they are usually treated surgically. The incidence of Mǔllerian cysts is one in 105,000, with almost equal sex distribution. Their unusual intraperitoneal location further demonstrates their uncommon presentation. Clin. Anat. 23:866–869, 2010. © 2010 Wiley‐Liss, Inc.     

Neuroendoscopic removal of large choroid plexus cyst: a case report

Choroid plexus cysts (CPCs) are the most common neuroepithelial cysts, occurring in more than 50% of some autopsy series. They are typically small and asymptomatic and are discovered incidentally in older patients, usually in the trigone of the lateral ventricle. Symptomatic CPCs (usually exceptionally large, 2-8 cm) are rare. The authors report a case of large symptomatic choroid plexus cyst, located in the trigone of the right lateral ventricle in a 26-yr-old man who presented with headache and vomiting. The patient underwent endoscopic removal through a burr hole placed 3 cm from the midline and just behind the hair line. The histological examination of the cyst wall was consistent with choroid epithelium. Despite of postoperative intraventricular hemorrhage and catheter infection, he discharged home without neurologic deficits. The endoscopic fenestration rather than excision should be considered as the first surgical procedure because the goal of treatment is shrinkage of the cyst until normal cerebrospinal fluid flow is restored.     

Retroperitoneal multilocular bronchogenic cyst adjacent to adrenal gland

Bronchogenic cysts are generally found in the mediastinum, particularly posterior to the carina, but they rarely occur in such unusual sites as the skin, subcutaneous tissue, pericardium, and even the retroperitoneum. A 30-year-old Korean man underwent surgery to remove a cystic adrenal mass incidentally discovered during routine physical checkup. At surgery, it proved to be a multilocular cyst located in the retroperitoneum adjacent to the left adrenal gland. Microscopically, the cyst was lined by respiratory epithelium over connective tissue with submucous glands, cartilage and smooth muscle, thereby histologically confirming bronchogenic cyst. This is the first reported case of retroperitoneal bronchogenic cyst in an adult without other congenital anomalies in Korea.     

A retroperitoneal bronchogenic cyst: a rare cause of a mass in the adrenal region

This report documents a bronchogenic cyst presenting as an adrenal tumour in a 51 year old man with persistent epigastric pain. The cyst is regarded as a developmental abnormality of the primitive foregut, which typically occurs in the chest. Subdiaphragmatic, and retroperitoneal locations in particular, are unusual. The differential diagnosis of a bronchogenic cyst in the retroperitoneum includes cystic teratoma, bronchopulmonary sequestration, cysts of urothelial and mullerian origin, and other foregut cysts.     

Primary papillary carcinoma of the thyroglossal duct cyst--a case report

Thyroglossal duct cyst is the most common developmental anomaly of the thyroid gland, presenting as a mid line neck swelling. Malignancy arising in them is a rare event, with papillary thyroid carcinoma being the commonest. Thyroglossal duct cyst carcinomas are usually asymptomatic and are not suspected preoperatively in most instances, hence the need for surgical excision and careful pathological examination of these cysts. We present a case of a 57 year old lady who underwent a Sistrunk procedure for the removal of thyroglossal duct cyst, which was responsible for a progressive mid line neck swelling of 2 months duration. The thyroid gland was normal. Histological examination of the excised cyst showed a papillary thyroid carcinoma arising in the thyroglossal duct cyst. Long term survival of patients with thyroglossal duct cyst carcinoma is excellent.     

Urogenital mesenteric cyst with fallopian tubal features

Urogenital cysts are retroperitoneal or mesenteric cysts that are derived from vestigial remnants of the embryonic urogenital apparatus. Although their precise embryonic origins are usually obscure, occasionally they display histologic features indicative of a mesonephric or metanephric origin. This report describes an unusual mesenteric müllerian cyst consisting of an endosalpingial lining and two-layered smooth muscle walls, probably representing a müllerian duct duplication cyst.     

Intestinal obstruction due to a mesenteric cyst

Mesenteric cysts are rarely thought of, may be difficult to diagnose, and are usually asymptomatic except when complicated. Intestinal obstruction is a rarely reported complication of these cysts. A case of mesenteric cyst that was causing obstruction of the large bowel is presented, along with a review of the literature.