Cystic lymphangioma of the spleen--a case report

Splenic cystic lymphangioma is a very rare condition. It occurs commonly in children in whom it is often an incidental imaging finding. In the absence of histologic confirmation, it usually mimics hydatid disease and other cystic vascular proliferations of the spleen. We present a case of cystic lymphangioma of the spleen in 15 years old female.     

脾脏淋巴管瘤的临床病理观察

目的探讨脾脏淋巴管瘤的临床病理特征和鉴别诊断特点。方法结合文献回顾,对18例脾脏淋巴管瘤进行临床资料分析、病理形态学观察及免疫表型检测。结果18例淋巴管瘤患者年龄9～72岁,中位年龄40岁;男13例,女5例;可无明显临床症状,也可表现以腹痛为主的消化道症状或脾功能亢进的相关症状。18例患者随访率72．2％,随访时间从5个月到15年不等,患者均无瘤生存,无复发及转移。脾脏多有肿大,病灶大体形态多样,包括囊性（8例）、实性（5例）和蜂窝状（5例）,可单发（5例）或多发（13例）;组织形态上分为海绵状淋巴管瘤（9例）、囊状淋巴管瘤（5例）及混合型（4例）。16例（100％）均表达CD9;7例（43．8％）表达D2-40。结论发生在脾脏的淋巴管瘤非常少见,应注意与脾脏血管瘤等鉴别。     

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.     

Enlarging cystic lymphangioma of the mediastinum in an adult: is this a neoplastic lesion related to the recently discovered PIK3CA mutation?

Cystic lymphangioma, a lymphatic system malformation, is usually observed in infants and children and is rarely found in adults. It most commonly occurs in the cervicofacial region, followed by the axilla. Mediastinal cystic lymphangioma is rare, accounting for 1.8% of all mediastinal cysts. Herein, we present an exceedingly rare adult case of mediastinal cystic lymphangioma that had increased in size over a 5-year period. Although fluid collection might be an alternative explanation for this increase in size, this lymphangioma might harbor a neoplastic nature related to the recently discovered PIK3CA mutation.     

Cystic lymphangioma as mesenteric tumor--a case report

Cystic lymphangioma is an uncommon mesenteric tumor usually reported in children. We report a case of 14 year old female who presented with dull aching abdominal pain. At laparotomy a cystic tumor was found in mesentery, which was attached to bowel loops. The histopathology examination showed features of cystic lymphangioma. Although pre-operative diagnosis is usually possible on CT or MRI, confirmatory diagnosis of this lesion requires laparotomy followed by histopathology.     

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.     

Solitary intrapulmonary cystic lymphangioma in an infant: a case report with literature review

Lymphangioma rarely presents as a solitary pulmonary lesion. We encountered a case of solitary cystic lymphangioma and present its clinicopathologic and immunohistochemical findings. A 2-month-old boy was referred to the hospital after developing a persistent cough. Chest X-ray showed a large cyst in the right lung. Under the preoperative diagnosis of bronchogenic cyst, he underwent right lower lobectomy at the age of 11 months. The resected specimen contained a 5.5-cm septate cystic lesion. Microscopically, the lesion consisted of a large cystic space and interconnected slit-like spaces surrounding bronchovascular islands. The cyst was lined by a monolayer of flat cells with focal multinucleated giant cells. Immunohistochemically, the cells lining the cystic lesion were positive for D2-40, Prox1, CD34, and CD31, and weakly positive for VEGFR-3, but were negative for AE1/3, HMB45, VEGF-A, VFGF-C, VEGFR-1. Differential diagnoses included lobar or interstitial emphysema, bronchogenic cyst, congenital pulmonary airway malformation and alveolar adenoma. D2-40 and Prox1 were useful in differentiation and in determining the extent of the lesion. A review of the literature found only 15 cases of solitary pulmonary lymphangioma. In younger patients, the lesions tend to occupy more of the lung. Focal giant cell reaction has not been described in the reported papers.     

Multilocular peritoneal inclusion cyst--a case report

Multilocular Peritoneal Inclusion Cyst (MPIC) is a rare mesothelial lesion. It is most commonly found in women of reproductive age group involving the abdominal and pelvic peritoneum. Previously, this lesion was often confused with cystic lymphangioma, but now its mesothelial origin has been confirmed. We report a case of a 26-year old female who underwent Caesarean section during which cysts which were multiloculated, thin-walled and filled with serous fluid, were incidentally discovered. Its histopathologic examination established the diagnosis     

Cholestasis caused by a choledochal botryoid rhabdomyosarcoma in a 22-month-old boy

Botrioid rhabdomyosarcoma of the extrahepatic bile ducts is a rare cause of jaundice in children. It has a very poor prognosis and is rarely diagnosed preoperatively. We report a choledochal botrioid rhabdomyosarcoma in a 22-month-old boy who developed an obstructive jaundice. Radiographic explorations suggested cystic lymphangioma. The gallbladder, the cystic duct, the common bile duct and the pancreatic head were resected. The diagnosis was made on pathological examination; adjuvant chemotherapy followed. The patient was disease free 20 months following treatment.     

Acquired Omental Cystic Lymphangioma after Subtotal Gastrectomy: A Case Report

We herein describe a case of cystic lymphangioma in the greater omentum of the remnant stomach, which is thought it to be related with subtotal gastrectomy 10 yr ago for early gastric cancer. A 76-yr-old man was admitted to our department with postprandial abdominal discomfort and bowel habit change. Intraabdominal multilocular cystic mass was detected by ultrasonography and computed tomography. We performed a complete En-bloc tumor resection including spleen and distal pancreas, and histological examination confirmed cystic lymphangioma originated from the greater omentum of the remnant stomach. Although the etiology of omental lymphangioma remains largely unclear, these findings suggested strongly that obstruction of the lymphatic vessels after gastric resection for gastric carcinoma might be the most plausible cause. The surgical extirpation with resection of organs involved appears to be a treatment of choice for such unusual case.     

Intrapulmonary cystic lymphangioma in a 2-month-old infant.

Lymphangioma is an abnormal collection of lymphatics that are developmentally isolated from the normal lymphatic system. Lymphangioma rarely presents as a solitary pulmonary lesion. We report a rare case of intrapulmonary cystic lymphangioma involving the upper lobe of the right lung, which presented with dyspnea in a 2-month-old infant. High-resolution computed tomography (HRCT) of the chest demonstrated a well-circumscribed, multiseptate, cystic lesion in the upper lobe of the right lung, mimicking the feature of type I congenital cystic adenomatoid mal-formation. The tumor was removed by bilobectomy of the upper and middle lobes of the right lung, and its pathologic examination confirmed the diagnosis of an intra-pulmonary cystic lymphangioma.     

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.     

Mesenteric cystic lymphangiomas--a report of five cases

Five cases of cystic lymphangiomas are described. There were three children and two adult patients. Age ranged from 4 to 38 years. All the patients presented with pain abdomen. On examination, four out of five patients were found to have an ill defined lump abdomen. One patient had no abdominal symptoms. Abdominal ultrasonography done in all the cases revealed the cystic nature of the tumour thereby making the pre operative diagnosis easier. A histological diagnosis of cystic mesenteric lymphangioma was made in each case. Although mesenteric lymphangiomas are rare, they should be considered as a possible cause of acute abdomen, both in children and adults.     

Cystic fibrosis with homozygous R553X mutation in a Taiwanese child

It has been reported that cystic fibrosis is very rare in Asians, and its clinical expression and genetic mutations are different from those found in Caucasians. We report the case of a boy who had chronic diarrhea with failure to thrive and frequent respiratory tract infections beginning at the age of 2 months. He developed bronchiectasis with chronic severe hypoxemia and pancreatic insufficiency by the age of 3 years and 5 months, which raised the consideration of cystic fibrosis. DNA analysis revealed a homozygous R553X mutation, and both his parents were subsequently proven to be R553X carriers. This case is the first report in a Taiwanese with cystic fibrosis attributable to a mutation commonly seen in Caucasians. However, the age of onset was much younger and the clinical course was more severe than those associated with Western patients. We reviewed the eight reported Taiwanese patients with cystic fibrosis, including the present case. We believe that the incidence of cystic fibrosis in Taiwan may be underestimated. Both genetic and environmental factors may play a role in the phenotypic disparity between Asians and Caucasians.     

Prenatal Ultrasound Evaluation and Outcome of Pregnancy with Fetal Cystic Hygromas and Lymphangiomas

Cystic hygroma is a type of lymphangioma, which is a vascular anomaly associated with lymphatic malformations and formed by fluid accumulation mainly located at the cervicofacial and axillary regions. Cystic hygroma is mostly located in the neck (75%), followed by axilla (20%), retroperitoneum and intra-abdominal organs (2%), limbs and bones (2%), and mediastinum (1%). It is often associated with chromosome aneuploidies, hydrops fetalis, and even intrauterine fetal demise. The prognostic factors of the fetal cystic hygroma or lymphangioma are chromosome abnormalities, hydrops fetalis, septations, or thickness of the cystic hygroma and are associated with other major malformations. Prenatal managements including ultrasound serial follow-up, magnetic resonance imaging, or even intrauterine injection of sclerosing agents are suggested. For fetus with the risk of airway obstruction at delivery, ex utero intrapartum treatment is also indicated. Detailed prenatal counseling is necessary for better neonatal outcome.     

Huge cystic lymphangioma of the adrenal gland. A case report and review of the 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. Preoperative diagnosis is difficult. Diagnosis is histological. We report a 22-year-old female presented with abdominal pain. Laboratory findings were normal. Radiological examination revealed a 35 cm left adrenal cyst. The cyst was resected and the diagnosis of cystic lymphangioma of the adrenal gland was histologically made. In presenting this case and reviewing the literature, we seek to make better known the epidemiologic, the clinicopathologic presentation and the prognosis of this rare entity.     

Comparative light and electron microscopic studies of cystic and papillary tumors of the peritoneum

Summary Using a cystic lymphangioma of the greater omentum and a benign cystic mesothelioma as examples, the distinguishing characteristics of cystic peritoneal tumors are shown, using light microscopic and ultrastructural findings. A benign papillary mesothelioma is used for comparison. The cellular structures and growth rate of the mesotheliomas are contrasted with the tissue reactions which are typical for irritated serosa. The diffusely growing papillary mesothelioma is a very rare tumor, the cells of which are similar to normal serosa cells, but also show characteristics of other benign or malignant mesothelial tumors and of reactive proliferated mesothelial cells. The diffusely growing benign cystic mesothelioma has only been described in isolated cases and is characterized by cysts grouped in acini with mainly flat, localized cuboidal cell lining. The histochemical properties and cytological findings correspond closely to those of the papillary tumor or normal peritoneal lining cells. In contrast, the cystic lymphangioma probably represents a congenital defect with a slow growth rate. The structure is characterized by a sponge-like arrangement of smooth-walled cysts, in the walls of which smooth muscle cells and lymph follicles are embedded. The endothelium is also flat and ultrastructurally resembles that of lymph vessels.     

Cystic renal cell carcinoma: Arose as a simple cyst, recurred repeatedly as a multicystic mass and finally presented as a solid mass

An unusual case of cystic renal cell carcinoma in a 70-year-old Japanese male is reported. He had had a simple renal cyst removed 1 year ago. On presentation the right kidney was surrounded by multiple translucent cysts, which varied in size from 1 to 50 mm. The cyst walls were lined by slngle-layered cuboidal epithelium. The differential diagnosis included cystic mesothelioma, cystic lymphangioma and multlcystic dysplastic kidney. An immunohistochemical and ultrastructural study and flow cytometric analysis of DNA ploldy were performed. The tumor was dlfferentiated to such an extent that it was difficult to diagnose as carcinoma; however, it recurred repeatedly. Three and a half years after initial presentation the tumor had invaded the ileum with the pathological change to be almost solid when viewed grossly and, microscopically, showed tubulo-papillary structures in addltlon to a cystic pattern. The DNA ploidy pattern revealed a near-diploid aneuplold in the early specimen and a polyploid aneuploid in the last specimen.     

Peritoneal cystic mesothelioma: an electron microscopic and immunohistochemical study of two male patients.

The clinical, pathological, and ultrastructural features of two cases of peritoneal cystic mesothelioma occurring in men were studied. The results of immunohistochemical staining for CAM 5.2, epithelial membrane antigen, carcinoembryonic antigen, and Factor VIII related antigen are reported for the first time and compared with the staining results of two peritoneal cystic lymphangiomas. Although resembling cystic lymphangioma by light microscopy, cystic mesothelioma may have a greater tendency for local recurrence. Staining for CAM 5.2 or epithelial membrane antigen may facilitate the differentiation of these two entities.