Cystic Lymphangioma of Rectum-A Case Report and Review of Literature.

Colorectal cystic lymphangiomas are rare benign lesions. They are characterized by the presence of either single or multi-cystic spaces lined by endothelium. Though there are multiple case reports of right and transverse colonic lymphangioma; only around 10 cases of lymphangioma of the rectum have been reported. We present a case report of rectal lymphangioma with the relevant literature review.

Retroperitoneal cystic lymphangioma in a cirrhotic patient: A case report and review of the literature.

Retroperitoneal cystic lymphangiomas (RCL) are rare benign tumors of the lymphatic system. They account for less than 1% of all lymphangiomas. Surgical resection is the recommended treatment option; however, obtaining a pre-operative diagnosis is often difficult and, in most cases, the final diagnosis is only possible following histological assessment of the surgical specimen. This report describes a case of RCL in a 58-year-old female cirrhotic patient who presented to our center with dull aching abdominal pain and distension. To our knowledge, this is the first case of a RCL in a cirrhotic patient reported in the literature.

Peri-Hilar Cystic Lymphangioma Mimicking a Biliary Cystic Lesion Causing Biliary Obstruction: A Case Report and Literature Review.

BACKGROUND Lymphangiomas are rare and benign malformations of the lymphatic system. The presentation of intra-abdominal lymphangiomas, especially from within the hepatoduodenal ligament, is rare in the adult population. In this report, we examine a lymphangioma within the hepatoduodenal ligament resulting in biliary obstruction. CASE REPORT A 62-year-old man with surgical history of cholecystectomy presented to the hepatobiliary clinic for a peri-hilar cystic lesion identified on surveillance magnetic resonance imaging (MRI). The patient's MRI revealed a 5.5-cm cystic lesion at the peri-hilar region, likely arising from the biliary tree, which had been increasing in size and causing biliary dilatation. The patient underwent an endoscopic ultrasound, showing a 4.3×2.2 cm cystic structure likley arising from the cystic duct stump with internal septation. An endoscopic retrograde cholangiopancreatography (ERCP) was performed and demonstrated no communication between the biliary tree and the cystic lesion. Given the uncertain etiology of the lesion and its obstructive nature, the patient was moved to the operating room for a complete excision. A well-encapsulated cystic lesion was identified between the cystic duct and the common hepatic duct, which did not communicate with the biliary tree. Pathology confirmed the diagnosis of lymphangioma with features of vascular channel proliferation in the background of fibrotic stroma and lymphoid aggregates. The vascular channel proliferation demonstrated positive immunohistochemical staining for D2-40. At 3-year follow-up, there was no evidence of post-resection recurrence. CONCLUSIONS This case represents an acquired lymphangioma occurring as a sequela of cholecystectomy, likely caused by interruption of the lymphatic drainage system secondary to surgical manipulation.

Retroperitoneal lymphangioma as the final diagnosis of a middle-aged woman with abdominal pain: a case report.

BACKGROUND: Lymphangiomas are lesions attributed to congenital malformations of the lymphatic system, or acquired chronic obstruction of the lymphatic network due to trauma, radiation, surgical manipulation, inflammation, or infection. Overall, lymaphangiomas are rare, and particularly, retroperitoneal lymphangiomas are far more uncommon per reported cases. CASE PRESENTATION: A 49-year-old Iranian woman presented with a progressive abdominal pain since approximately 1 month before admission. She was found to have a retroperitoneal lymphangioma after a precise radiological and surgical workup. CONCLUSION: Retroperitoneal lymphangiomas are rare lesions, sometimes indistinguishable from malignant lesions originating from pancreas and adjacent organs. Complete surgical removal and histologic evaluation of the lesion is the gold standard of treatment and diagnosis.

Intra-abdominal Cystic Lymphangiomas: The Vanderbilt Experience.

INTRODUCTION: Lymphangiomas are rare, cystic tumors that represent congenital malformation of the lymphatic vessels. We reviewed our institution's experience treating abdominal lymphangiomas with the purpose of describing the clinical features, management, and outcomes of this rare pathology. METHODS: This is a single-institution, institutional review board-approved retrospective review of abdominal lymphangiomas presenting between January 2010 and February 2021. The diagnosis of lymphangioma was made on histopathology from either endoscopic or excisional biopsy of the lesion. Demographics, diagnostic imaging, histopathologic characteristics, and outcomes were analyzed. RESULTS: We identified 48 patients, of whom 29 (60%) were female, >18 y (38; 79%), with a mean age of 43 y at the time of diagnosis (range, 4 d-87 y). Tumors ranged in size from <1 cm to 30 cm. Only 1/3 were symptomatic, most commonly with abdominal pain (9; 19%) On preoperative imaging, mural nodules or thickened walls were present in one case, in which pathology was consistent with benign lymphangioma. The majority of lymphangiomas were associated with the small bowel or its mesentery (31; 65%), followed by the colon/omentum (7; 15%). Most patients underwent surgical excision (29; 60%) with incomplete excision in one patient due to extensive local invasion, and three (10%) patients required multivisceral resection. The median duration of the follow-up was 13 mo (range, 1-105 mo), during which time, none of the patients developed malignancy. CONCLUSIONS: Most abdominal lymphangiomas arise from the small bowel and are found incidentally and have a favorable prognosis. Resection should be reserved for symptomatic lesions or when there is a diagnostic uncertainty.

A case of cystic lymphangioma arising from the parauterine tissue.

A 57-year-old woman, gravida 3, para 3, with no complaints visited our hospital for right-sided adnexal tumor found incidentally in cancer screening. She had no medical history, surgical history, or gynecological disease. Imaging studies showed a 5-cm lobular cystic tumor on the right side of uterus. We suspected right hydrosalpinx and decided to perform diagnostic laparoscopy. During laparoscopy, the right adnexa was found to be atrophic, and the tumor was located in the broad ligament. The tumor was observed to be a multilocular cyst containing yellow fluid that developed from the right parauterine tissue. The tumor was resected from the surrounding tissue. Histological examination revealed that the multilocular cyst contained a vascular component surrounding the lymphatic endothelium and was decided to be a cystic lymphangioma. The patient was followed up and there was no evidence of recurrence at postoperative 7 months. We experienced a very rare case of lymphangioma arising from the parauterine tissue. The laparoscopic approach can assist with both diagnosis and treatment.

A large pericardial cystic lymphangioma presenting as acute-onset respiratory distress in a child: a case report.

BACKGROUND: Lymphangiomas are rare benign malformations of the lymphatics that occur due to blockage of the lymphatic system during fetal development. They commonly occur in the neck and axilla, while involvement of the pericardium is rare. We report herein the case of a 16-month-old Sri Lankan child with a large pericardial cystic lymphangioma presenting with sudden-onset shortness of breath. CASE PRESENTATION: A 16-month-old Sri Lankan boy presented with sudden-onset dyspnea for 1-day duration following a febrile illness that lasted 2 days. On examination, he was afebrile and had subcostal, intercostal, and suprasternal recessions, with a respiratory rate of 50 breaths per minute. He had a loud expiratory grunt. The chest expansion was reduced on the right side, which was dull to percussion. Auscultation revealed a marked reduction of air entry over the right lower and mid zones. Chest X-ray showed a well-demarcated opacity involving the lower and mid zones of the right hemithorax associated with a tracheal shift to the opposite side. Ultrasound scan of the chest revealed fluid-filled right hemithorax suggesting a septate pleural effusion. A contrast-enhanced computed tomography scan of the thorax showed a large multiloculated extrapulmonary cystic lesion involving the right hemithorax with a mediastinal shift towards the left side associated with displacement of the right-side mediastinal structures. He underwent mini-thoracotomy and surgical excision of the cyst. A large cyst originating from the pericardium was observed and excised during surgery. Histological examination revealed a lesion composed of cysts devoid of a lining epithelium but separated by connective tissue, mature adipose tissue, and lymphoid aggregates. The child showed complete recovery postoperatively with full expansion of the ipsilateral lung. CONCLUSION: We report the case of a patient with cystic lymphangioma who was perfectly well and asymptomatic until 16 months of age. This case report presents the very rare occurrence of a large cystic lymphangioma originating from the pericardium. It highlights the importance of considering rare possibilities and performing prompt imaging in situations of diagnostic uncertainty to arrive at an accurate diagnosis that can be lifesaving.

First Description of Fetal Cystic Hygroma Associated With Early Equine Pregnancy Loss.

Cystic hygroma (hygroma cysticum) is a malformation that has not yet been described as a cause of early pregnancy loss in equines. The condition is a congenital anomaly occurring during embryogenesis due to a failure in which the primitive lymphatic sac does not reach the venous system at the jugular vein, resulting in a lymphatic stasis that starts in the neck region and continues to the rest of the body. From 2015 to 2020, a total of 5,730 ultrasound examinations were performed in mares from 43 different horse farms and embryo transfer farms when sexing pregnancies. In 12 pregnant mares, a suspected fetal cystic hygroma was diagnosed via transrectal ultrasound performed from day 52 to 75 of pregnancy. Six fetuses were collected and fixed to conduct histopathological and karyotyping. Macroscopic and microscopic analysis supported the suggested diagnosis being the first description of cystic hygroma in equine fetuses and concluded as a cause of pregnancy loss around 65 days of gestation.

Giant retroperitoneal lymphangioma in a 70-year-old male: a case report.

Lymphangiomas are relatively rare benign congenital tumors of the lymphatic system. They are usually discovered during childhood and typically occur in the neck and axillary regions. Retroperitoneal lymphangiomas are a rare occurrence and represent 1% of all cases. Here, we presented a 70-year-old male who presented with abdominal discomfort and chronic constipation in the last three years. A computerized tomography scan showed a giant abdominal hypodense cystic mass measuring 195 x 145 mm, which laminates the abdominal aorta, the left iliac vessels, and the left ureter. The patient underwent surgical exploration. Due to the giant cystic volume, it was carefully opened, aspirated, and removed. The histopathological examination showed a retroperitoneal lymphangioma. In conclusion, giant retroperitoneal lymphangioma in an adult is a rare occurrence. The primary treatment is complete surgical excision. Histopathological examination is essential for diagnosis confirmation.

A Case Report of Huge Lymphangioma over the Chest Wall: A Rare Presentation of a Newborn.

BACKGROUND: Lymphangioma is a rare benign tumor of lymphatic system that is often diagnosed in the first few years of life. The presentation and complications depend on the site and the size of the lesion. CLINICAL DESCRIPTION: This was a term male newborn weighing 3230g born to a 38 year old para IIV mother. Delivery was spontaneous and uneventful. The neonate was active, had no gross dysmorphic feature except the huge, 20cm by 28cm cystic, non-tender mass over the left lateral chest area. DIAGNOSIS: Lymphangioma was diagnosed based on chest ultrasound, there was a large multiloculated cystic lesion over left lateral chest, and the cyst had no communication with spinal canal, and had no solid component. THERAPY: The patient was observed for complications, otherwise not needing intervention in the first few days. OUTCOMES: He developed superinfection of the mass, for which intravenous antibiotics administered, infection was controlled and surgery was postponed until a few months. However, the patient was presented with severe malnutrition at the age of three months and subsequently lost to follow up. CONCLUSION: Huge lymphangiomas at neonatal age are likely to get superinfected; a close observation for signs of complications is needed. Though surgical intervention could be postponed until the baby grows to avoid the complications of surgery, adequate counseling is needed to reassure the parents about the benign and treatable nature of the disease. And individualized decision on earlier surgical intervention has to be considered with adequate postoperative care whenever follow up is not guaranteed.

Complex rearrangements of Y chromosome suggest RPS4Y1 as lymphedema candidate gene.

OBJECTIVE: Cystic hygromas are frequently encountered in fetus with Turner syndrome (TS). Nevertheless, identification of genetic loci responsible for the cystic hygroma has been problematic. Here, we tried to elucidate the candidate gene for cystic hygroma through a rare case of complex Y chromosomal rearrangements involving duplication of partial Yq and monosomy of partial Yp. CASE REPORT: A 30-year-old woman, gravida 1 para 0, was diagnosed with fetal cystic hygroma at 12 weeks of gestation. The genetic analysis of the product of conception revealed complex rearrangement of Y chromosome: microdeletion in Yp11.2p11.31 and microduplicatin in Yq11.223q11.23. The deleted region spans about 6.25 Mb and includes 76 genes, including SRY. The duplicated region spans about 4.76 Mb and includes 145 genes. CONCLUSION: From this rare case with non-mosaic complex Y-chromosome rearrangements, we could narrow down Turner stigmata critical region to Yp11.2～p11.3. We also propose RPS4Y1 as lymphedema candidate gene.

Current approach to branchial remnants in the neck.

Congenital branchial fistulas and cysts are an interesting subject in cervical pathology. There are congenital malformations with late expression in young adults that require correct diagnosis and appropriate treatment. We review essential notions of cervical embryology to understand the mechanism of occurrence of these malformations and their clinical expression. The most common cases present vestiges from the second branchial arch, with the appearance of a cystic tumor or a fistulous orifice on the anterior edge of the sternocleidomastoid muscle, at the level of the hyoid bone. Performant imagery is mandatory for appropriate diagnosis, so we recommend a cervical computed tomography (CT) scan or cervical magnetic resonance imaging (MRI) to evaluate the relations with great vessels of the neck or other lesions. The treatment implies complete surgical excision because otherwise there is a high risk of recurrence of the lesion. The differential diagnosis includes cystic lymphangioma, dermoid cyst, tuberculous adenopathy, cystic hygroma, lateral cervical cystic metastases. Histological examination is mandatory for a definite diagnosis. Also, there is a small percentage of malignancy of these malformations, but it is very important to check that all the histological diagnostic criteria for a primary branchiogenic carcinoma are accomplished. Therefore, although it is a benign cystic cervical pathology, the diagnosis and treatment must be made very accurately for a complete cure, and this review aims to summarize the current approach to branchial remnants of the neck.

[A Case of Giant Mesenteric Lymphangioma in an Adult].

A 26-year-old man with left inguinal pain and frequent urination was examined. An abdominal ultrasound revealed a cystic lesion. In further examinations, CT and MRI showed a large cystic lesion of about 20 cm in size, connected to mesenteric- derived blood vessels. We suspected a huge mesenteric lymphangioma and decided to perform a laparotomy. A tumor was seen in the mesentery of the jejunum and adhered to the duodenum widely. The tumor could be removed safely without resection of the duodenum by first sucking the contents and shrinking the tumor. The final pathological diagnosis was mesenteric lymphangioma. Adult mesenteric lymphangiomas measuring larger than 20 cm are relatively rare. We review the case in the context of the relevant literature.

Laparoscopic-assisted excision of a huge polycystic omental lymphangioma in a 3 year old patient presenting with acute abdomen: case report and review.

Lymphangioma is a rare benign neoplasm affecting mainly children. In this report we present a complicated case of polycystic omental lymphangioma in a 3 year old female presenting with acute abdomen. The patient underwent a laparoscopic-assisted excision of the lesion and had an excellent postoperative course. We discuss the effectiveness and advantages of this laparoscopic surgical approach in children and elaborate on the current literature.