Cystic lymphangioma of adrenal gland: a clinicopathological study of 3 cases and review of literature

Cystic lymphangioma of the adrenal gland is a rare and benign lesion, most often found incidentally during abdominal imaging studies, abdominal surgery or at autopsy. We aimed to retrospectively review all adrenal lymphangioma cases at our hospital, further document their lymphatic origin by immunohistochemical staining and discuss the differential diagnosis with other cystic adrenal gland lesions. A total of 3 adrenal lymphangioma cases were identified. All three patients were men and adults at time of diagnosis aged 41 years, 43 years, and 66 years, respectively. All were incidentally identified during investigating for unrelated reasons, two of which were discovered by routine radiologic check-up while the last one was found during imaging detection of ureteral cancer. The average size of an adrenal lymphangioma lesion was 3.2 cm (range, 2.5-4.6 cm). Histologically, all three cases showed a typical multicystic architecture with dilated spaces lined by flattened, bland, simple lining. The cystic spaces occasionally contained proteinaceous material but lacked red blood cell content. On immunohistochemical stains, D2-40 cytoplasmic staining was positive in all three lesions, whereas AE1/AE3 was negative, thus, confirming their lymphatic nature.     

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.     

Adenomatoid tumor of the adrenal gland: a clinicopathologic study of 3 cases

Adenomatoid tumors are relatively uncommon benign neoplasms of mesothelial origin, usually occurring in the male and female genital tracts. Rare extragenital adenomatoid tumors have been identified in the adrenal glands, heart, mesentery, pleura, and lymph nodes. In the adrenal gland, adenomatoid tumors may pose a diagnostic challenge. The differential diagnosis includes adrenocortical carcinoma and metastatic carcinoma, especially signet ring cell carcinoma. Because of its glandular pattern, an adenomatoid tumor may be confused with an adenocarcinoma. We present 3 cases of adrenal adenomatoid tumors, including one with a concurrent large hemorrhagic vascular adrenal cyst. The adenomatoid tumors were unilateral, appeared solid and white, and varied from 1.7 to 4.2 cm in diameter. They occurred in 3 male patients aged 33, 33, and 46 years. One patient presented with abdominal pain due to the presence of a concurrent large adrenal cyst. The tumor was an incidental radiological finding in another case and was discovered during the course of a workup for hypertension in the third case. The light microscopic appearances were consistent with those of typical adenomatoid tumors. Immunohistochemical stains for calretinin and cytokeratin 5/6 were positive, confirming the tumors' mesothelial origin. Ultrastructural studies performed in 2 cases revealed microvilli and desmosomes. Follow-up showed no evidence of recurrence or metastasis. In our experience, the key to the diagnosis of this rare benign tumor is to consider adenomatoid tumor in the differential diagnosis of any glandular tumor occurring in the adrenal gland.     

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.     

Primary hydatid cyst of the adrenal gland: a case report and review of the literature

Hydatid cyst of adrenal gland is rare and usually incidentally found as a part of disseminated disease. Herein we report a rare case of primary adrenal hydatid cyst who presented with unusual symptom of arterial hypertension from an endemic country.     

Retroperitoneal bronchogenic cyst: Report of a case and literature review

A large cystic mass was found in the subdiaphragmatic region of a 46-year-old woman who had complained of continuous pain in the left flank . The cyst was located in the retroperitoneum just below the diaphragm and was adhered to the diaphragmatic skeletal muscle and abdominal aorta, but was separate from the spleen, pancreas, left adrenal gland and left kidney. The surgically resected cyst measured 8 x 8 x 7 cm and was filled with protein-rich fluid, which contained amylase and embryonal proteins such as carcinoembryonic antigen, CA125 and CA19-9. Histologically, the cyst wall was composed of a fibrovascular connective tissue containing thin smooth muscle layers and mucus-secreting glands and was lined by a ciliated pseudostratified or tall columnar epithelium without dysplastic changes. Thus, a diagnosis of bronchogenic cyst, which is usually discovered in the posterior part of the mediastinum, was made. A rare case of bronchogenic cyst and a literature review is presented.     

Adrenal lymphangioma: clinicopathologic and immunohistochemical characteristics of a rare lesion

Adrenal lymphangiomas, also known as cystic adrenal lymphangiomas, are rare, benign vascular lesions that usually remain asymptomatic throughout life. Although previously adrenal lymphangioma lesions were primarily found at autopsy, they are currently detected during imaging work-up for unrelated causes and are likely to imitate other adrenocortical or adrenal medullary neoplasms. We aimed to retrospectively review all adrenal lymphangioma cases at our hospital and further document their lymphatic origin by immunohistochemical staining. A search of surgical pathology records (1984-2008) was conducted. All hematoxylin and eosin sections were retrieved from archives and reviewed by 2 pathologists in the study. Clinical information was gathered from electronic medical records. Representative paraffin-embedded sections from each case were selected for immunohistochemical analysis using monoclonal antibodies D2-40 and AE1/AE3. A total of 9 adrenal lymphangioma cases were identified (6 women and 3 men). All 9 patients were adults at time of diagnosis with a mean age of 42 years (range, 28-56 years). There were 7 white patients, 1 African American patient, and 1 Asian patient. The average size of an adrenal lymphangioma lesion was 4.9 cm (range, 2.0-13.5 cm). Adrenal lymphangioma was twice more frequently located on the right side (6 right-sided and 3 left-sided). Clinically, 4 (44%) of the 9 lesions presented with abdominal, flank, or back pain. One lymphangioma was found during work-up for labile hypertension. The remaining 4 lesions (44%) were asymptomatic and incidentally found during imaging studies for unrelated causes. Surgical removal was achieved by total adrenalectomy in 8 of the 9 lesions and by partial adrenalectomy in the remaining case. No evidence of recurrence or development of a contralateral lesion was encountered in any of the patients. Histologically, our adrenal lymphangiomas showed a typical multicystic architecture with dilated spaces lined by flattened, bland, simple lining. The cystic channels/spaces occasionally contained proteinaceous material and lacked red blood cell content. On immunohistochemical stains, D2-40 cytoplasmic staining was positive in all 9 examined lesions, whereas AE1/AE3 was negative, thus, confirming their lymphatic nature. D2-40 staining was diffuse in 2 and focal in the 7 remaining lesions. Adrenal lymphangiomas are very rare, benign lymphatic neoplasms with a female, right-sided predominance in our current series. They may clinically present with abdominal pain or can be incidentally found during adulthood as a mass, necessitating surgical removal to rule out other types of adrenal neoplasms.     

Lymphangioma of the kidney: a pathologic entity distinct from solitary multilocular cyst.

A case of primary renal lymphangioma is reported. This represents the first documented example of such a lesion confirmed by immunocytochemical and ultrastructural studies. Whereas previously regarded as synonymous terms, the recognition of lymphangioma as a pathologic entity distinct from solitary multilocular cyst of the kidney appears warranted. Although rare, lymphangioma should be considered in the differential diagnosis of multicystic lesions of the kidney.     

Kyste hydatique primitif de la glande surrénale chez une patiente porteuse de situs inversus. À propos d’une observation

Primary hydatid cyst of the adrenal gland remains an exceptional localization. The adrenal gland is an uncommon site even in our country in which echinococcal disease is endemic. We report the case of a 38-year-old woman who consulted for left back pain. The CT scan showed a cystic mass in the left retroperitoneal area with a calcified wall. The patient underwent surgery which confirmed a diagnosis of hydatid cyst of the left adrenal gland. The surgical treatment consisted on a total resection of the cyst, without rupture of the cystic wall and preserving the gland. The diagnosis was confirmed on macroscopic and histological examination of the resected piece. The postoperative course was uneventful. No recurrence had occurred after 36 months of follow-up.     

Adrenal cyst

Adrenal cysts are rare lesions that usually present as incidental findings during surgery or at the time of autopsy. The cysts are usually small, seldom exceeding 10 cm in diameter, and are generally asymptomatic. In those few cases that are symptomatic, radiographic examination is the most important tool in establishing a correct preoperative diagnosis. Of the four main categories, endothelial cysts represent the most common type of adrenal cyst. Simple surgical enucleation of the cyst, with preservation of the remaining adrenal gland, is the treatment of choice.     

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.     

Fine-needle aspiration cytology of lymphangioma of the parotid gland in an adult

Lymphangioma or cystic hygroma is an uncommon benign congenital tumor of lymphatics that is seen in children and, rarely, adults. Lymphangioma primarily involving the parotid gland is an extremely uncommon occurrence in adults. We report on the cytologic findings of a parotid lymphangioma in a 34-yr-old man which showed 13 cc of yellow fluid with red blood cells, lymphocytes, and rare fragments of benign-appearing salivary gland epithelium. The differential diagnosis of cystic parotid gland lesions in adults may include Warthin's tumor, lymphoma, benign lymphoepithelial lesions, branchial cleft cysts, chronic sialadenitis, cystic low-grade mucoepidermoid carcinoma, and cystic pleomorphic adenoma. In this case, the fine-needle aspiration findings along with the magnetic resonance imaging (MRI) findings of a multiloculated cystic mass in the parotid gland allowed the diagnosis of lymphangioma.     

Adenomatoid tumor of the adrenal gland.

An adenomatoid tumor of the adrenal gland is presented. The tumor was discovered in a patient with hypertension and was clinically mistaken for a pheochromocytoma. This is the first reported case, to my knowledge, of an adenomatoid tumor within the adrenal gland, with the exception of one previous article in which the lesion was mistaken for a lymphangioma. This case illustrates that adenomatoid tumors can rarely occur in extragenital locations, even in regions presumably devoid of mesothelial cells.     

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.     

肾上腺少见病变的CT表现 (附12例报告)

目的探讨肾上腺少见病变的CT表现特点。方法2003年1月至2006年7月肾上腺少见占位性病变12例,其中男性7例,女性5例,年龄14～76岁,中位年龄42岁。回顾性分析12例经手术病理证实的肾上腺少见病变的CT资料。结果12例中,右肾上腺皮质癌2例,右肾上腺神经鞘瘤1例,左肾上腺节细胞神经瘤1例,多发内分泌肿瘤MEN-Ⅰ型1例,左肾上腺区巨淋巴结增生症1例,肾上腺髓样脂肪瘤4例5个病灶,肾上腺囊肿2例。囊肿及髓样脂肪瘤易于诊断;肾上腺皮质癌CT表现提示其为恶性;其他少见病变定性诊断困难。结论肾上腺少见病变均有一些CT表现特点;CT平扫加动态增强扫描对诊断有重要价值。     

Fine-needle aspiration cytology of primary granulosa cell tumor of the adrenal gland: a case report

Extraovarian granulosa cell tumors are extremely rare. We report on a primary granulosa cell tumor of the adrenal gland. A 69-yr-old African-American female presented with a 1-yr history of irregular uterine bleeding and a palpable right abdominal mass. CT scan showed a 9.0-cm suprarenal mass as well as an enlarged uterus. CT-guided fine-needle aspiration (FNA) cytology of the adrenal mass was interpreted as a malignant neoplasm. She underwent exploratory laparotomy, right nephrectomy, and hysterectomy with bilateral salpingo-oophorectomy. The gross, histologic, and immunohistochemical findings of the adrenal mass were characteristic of a granulosa cell tumor. The uterus contained multiple leiomyomas. The endometrium showed simple hyperplasia. Both fallopian tubes and ovaries showed no pathologic abnormality. There was no evidence of tumor elsewhere. Although rare, extraovarian granulosa cell tumor should be considered in the differential diagnosis of adrenal tumors in women showing the FNA features described herein, especially when there is evidence of excessive estrogen production. Diagn. Cytopathol. 2000;22:107-109.     

Bronchogenic cyst of the stomach masquerading as benign stromal tumor

Gastric bronchogenic cyst is an extremely rare lesion. A 62-year-old Korean woman presented with an incidentally found perigastric nodule. An abdominal ultrasonogram and computed tomography showed a 1.5 cm solid, homogeneous, low-density nodule in the hepatogastric ligament near the gastric lesser curvature. At the time of surgery, a soft 1.7 cm nodule was found in the lesser curvature side just below the gastroesophageal junction, and a gelatinous mass was protruded from the nodule during the resection. Microscopically, the gastric subserosa showed cystic structures lined by pseudostratified ciliated columnar epithelium, seromucinous gland, connective tissue and complete layers of smooth muscle bundles. Neither cartilage nor gastrointestinal epithelium was identified. A mucocele was found near the cyst and foamy cells and faintly bluish mucinous substances were found near the cystic structures. All the mucinous substances were stained by alcian blue at pH 2.5 with varying degrees of staining intensity. The present case is the smallest reported cyst among the gastric bronchogenic cysts, and these lesions are unusually located at the lesser curvature side of the stomach. Although these lesions are very rare, gastric bronchogenic cyst should be included in the differential diagnosis of gastric wall mass.     

Corticomedullary mixed tumor of the adrenal gland with concurrent adrenal myelolipoma

We report the case of a corticomedullary mixed tumor of the adrenal gland in a 55-year-old woman with a left adrenal mass who presented with mild symptoms of Cushing syndrome and an elevated urinary cortisol level. The patient underwent a left adrenalectomy. A well-circumscribed 2.5-cm mass, composed of an admixture of adrenal cortical cells and pheochromocytes, and an incidental 0.7-cm myelolipoma were present in the resected left adrenal gland. The diagnosis of adrenal corticomedullary mixed tumor was confirmed by both immunohistochemistry and electron microscopy. To our knowledge, this is the eighth well-documented report of this rare tumor.     

Angiosarcoma of the adrenal gland

We report the first case of primary angiosarcoma of the adrenal gland, to our knowledge. A 54-year-old man presented with chronic left upper quadrant abdominal pain, and a left adrenal mass was subsequently found on computed tomography of the abdomen. The tumor was surgically removed and a diagnosis of adrenal angiosarcoma, supported by findings of immunoperoxidase and ultrastructural studies, was made. Seven months later, recurrent tumor resulted in an en bloc resection of lateral gastric wall, tail of pancreas, left kidney, and spleen. One year after the initial surgery, the patient was free of tumor. Angiosarcoma should be added to the list of possible primary adrenal gland malignancies.     

Ganglioneuroma of the adrenal gland: a rare case

Ganglioneuromas, which are benign tumors of sympathetic nervous system, are rare to arise in adrenal gland. They are usually clinically silent and detected during work-up for other unrelated conditions. Currently, histopathology is the only tool to diagnose ganglioneuroma and to differentiate it from ganglioneuroblastoma and neuroblastoma. We discuss the case of a 40-year-old female found to have a right adrenal mass during investigation of dull ache in right upper abdomen. Clinico-radiological assessment was non-confirmatory on the nature of the mass, and excision was done. Histological examination showed the mass to be a ganglioneuroma arising in the adrenal gland. In conclusion, ganglioneuroma occurs rarely in adrenal gland and pre-operative diagnosis is difficult since the symptoms are usually non-specific. Histological examination is the mainstay of diagnosis and should be thorough to exclude neuroblastomatous foci, portending a worse outcome.