Retroperitoneal Lymphangiomas Research Articles

Retroperitoneal lymphangioma in a tertiary care hospital of North India

Less than 200 cases of retroperitoneal lymphangiomas (RL), a rare type of benign cystic tumour of the lymphatic system that makes about 1% of all lymphangiomas, have been documented to date. Their rarity makes preoperative diagnosis challenging. 95% of lymphangiomas are found in the head, neck, and axilla where they are most frequently found. We are presenting a 30 years old woman, who presented to outpatient department (OPD) of general surgery with symptoms of recurrent left lumbar pain, radiating towards inguinal region for one month. The contrast computed tomography (CT) scan of abdomen and pelvis defined a large thin walled homogenous non enhancing hypodense cystic lesion in left side of abdomen in retroperitoneal location from left lobe of liver till pelvis on left side craniocaudally. The patient was planned for exploratory laparotomy. Midline laparotomy was performed. A cystic lymph-filled tumour was detected in the abdominal cavity adjacent to the bowel loops. Histologically the mass contained variable-sized cystic spaces lined by flattened endothelium consistent with lymphatic vessels, which on immunostaining were positive for CD31 and negative for Pan CK. The patient was discharged satisfactorily. In conclusion, retroperitoneal lymphangioma presents vaguely as abdominal pain or mass and grown to large sizes without causing any significant symptom. Tumour markers for the excised tissue are important for diagnosis. Laparotomy or laparoscopy are the two surgical excision methods used to treat retroperitoneal cystic lymphangioma and it responds well to excision.

Retroperitoneal cystic lymphangioma in adult female

Abstract One percent of all lymphangiomas are retroperitoneal lymphangiomas, which are uncommon benign cystic lymphatic tumors. Lymphangioma mainly occurs in the age group of <2 years. This report describes retroperitoneal cystic lymphangioma in a 29-year-old female. She had a complaint of abdominal pain in the right lower region. The provisional diagnosis based on computed tomography scan was retroperitoneal mass. By en bloc, resection cyst was completely removed.

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

BackgroundLymphangiomas 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 presentationA 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.ConclusionRetroperitoneal 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.

Laparoscopic interventions in children with retroperitonial lymphatic malformations

Introduction. Lymphatic malformations (LM) is the developmental anomaly of lymphatic vessels which is formed at the stage of early embryogenesis. Intraabdominal and retroperitoneal LM are very rare and account for 4 % of all observed LM. Purpose. To clarify the effectiveness of minimally invasive surgical interventions in children with abdominal LM. Material and methods. Over a ten-year period, 15 children with retroperitoneal lymphangiomas , aged from 1 to 17, were operated on in our clinics. To clarify diagnosis, a comprehensive examination was performed: ultrasound, spiral computed tomography, magnetic resonance imaging of the abdominal cavity. Laparoscopic access was applied in all 15 cases for surgical intervention. Results. In all cases, lymphatic malformations were diagnosed before surgery which was later confirmed at laparoscopy. Malformations were excised laparoscopically. In 2 patients, lymphangiomatous tissue was partially left at the site of the greatest fusion with renal and iliac vessels due to a high risk of their damage. Conclusion. Endovideosurgical technologies in children with retroperitoneal LM may be used at any child’s age and at any size of retroperitoneal cystic formation since emptying the cystic cavity creates the necessary working space in the abdominal cavity. Such an approach minimizes surgical trauma, shortens surgical time and facilitates the postoperative period.

Huge Retroperitoneal Lymphangioma Presenting With Duodenal Obstruction: a case report.

lymphangiomas are benign tumors caused by the congenital proliferation of lymphatic tissue, causing an obstruction in the lymphatic ducts, producing fluid filled cysts (1). Histologically they are polycystic, with cysts divided by thin septas lined with endothelial cells. Most common presentation of cystic lymphangiomas are in the neck (75%) and axillary region (20%) and only 5% are intra-abdominal, while retroperitoneal lymphangiomas account for only 1% of cases (2). It is more common in children with males affected more than women, while women have shown to encounter pancreatic lymphangiomas more than men (3). Often retroperitoneal lypmhangiomas are asymptomatic but due to their large size they might cause a wide variety of symptoms from mild abdominal distention/discomfort and back pain to duodenal obstruction, sepsis and DIC (4). Diagnostic techniques are ultrasound, CT scan and MRI but often it is found accidentally and definitive diagnosis is made post-operatively. Treatment of choice is complete surgical resection with cystectomy

Retroperitoneal lymphangioma as the final diagnosis of a middle-aged woman with abdominal pain

Lymphangiomas are lesions attributed to the congenital malformations of 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 sparse per reported cases. In this study, we have introduced a middle-aged woman admitted to the gastroenterology unit with a progressive abdominal pain since approximately one month before her visit. She was found to have a retroperitoneal lymphangioma after a precise medical work up.

A rare case of a retroperitoneal cystic lymphangioma

Retroperitoneal Lymphangiomas are rare and account for only 1% of lymphangiomas. They usually present in infancy, rarely they may present symptomatically in adulthood. We present a case of a 19-year old female with a symptomatic retroperitoneal lymphangioma. It was treated with complete surgical excision. Retroperitoneal lymphangiomas are rare. Imaging alone cannot differentiate them from other retroperitoneal cystic masses. Surgical excision is the treatment of choice and required for final diagnosis.

Retroperitoneal lymphangioma in an adult: a case report of a rare clinical entity.

Background. Retroperitoneal lymphangioma is a rare, benign mesodermal tumor arising from the retroperitoneal lymphatics which usually presents in infancy and it is worthy to report a case when it has presented in an adult. After a thorough literature search in English we concluded that less than 200 cases of adult retroperitoneal lymphangioma have been reported so far. Apart from being a rare entity it also presents as a diagnostic dilemma and final diagnosis is often made on surgical exploration. Case Presentation. We report a case of retroperitoneal lymphangioma in a 55-year-old male who presented with abdominal distension and dull aching abdominal pain. Conclusion. Retroperitoneal lymphangiomas are rare tumors of infancy but may also present in adults where they are a diagnostic challenge. Differentiating cystic lymphangiomas from other cystic growths by imaging studies alone are often inconclusive and surgery is frequently required for definitive diagnosis and to ameliorate the symptoms.

Ossifying retroperitoneal cystic lymphangioma in a pregnant woman

Retroperitoneal cystic lymphangiomas are very rare lesions and may be misdiagnosed. Longstanding lymphangiomas may show secondary changes like inflammation, hemorrhage, fibrosis and rarely ossification. Treatment is complete surgical excision. We are reporting a rare case of ossifying retroperitoneal lymphangioma in a pregnant woman which was misdiagnosed clinically as ovarian tumor. Our diagnosis was confirmed by IHCCD- 31 and D2-40 positivity. Postoperative follow up for 3 years, patient is fine and she is full term pregnant now.

Giant Retroperitoneal Lymphangioma: a Rare Entity.

Retroperitoneal lymphangiomas are rare benign tumors, and the exact incidence of which is not well known. They usually arise from the developmental abnormalities of retroperitoneal lymphatics or their traumatic rupture. Confusion with other cystic tumors of the retroperitoneum including those arising from the kidney and pancreas is common. They usually lack specific symptoms and are mostly detected accidentally, while very rarely, some may present with complications such as rupture, torsion, hemorrhage, or infection. We report a case of a 22-year-old female in whom preoperative diagnosis of retroperitoneal lymphangioma was made on the basis of radiological investigations. On exploration, a large lymphangioma arising retroperitoneally was identified and excised. Postoperative recovery was uneventful.

Giant Cystic Retroperitoneal Lymphangioma in an Adult

Lymphangioma is an uncommon congenital malformation of the lymphatic system affecting usually the younger age group. These tumours frequently affect the neck and axillary region. Retroperitoneal lymphangiomas are rare and cause a clinical and pathological diagnostic challenge. Histopathological examination of the tumour is imperative for an accurate diagnosis. We report a case of a giant cystic retroperitoneal lymphangioma in an adult presenting as a mass in the left hypochondrium. Radiology revealed a large cystic mass in the retroperitoneum displacing the large bowel and compressing the left ureter. The mass was excised surgically and there is no evidence of recurrence on follow up.

F-18 FDG Uptake in a Retroperitoneal Cystic Lymphangioma Mimicking Malignancy

Retroperitoneal lymphangiomas are rare benign cystic tumors and occur because of failure of the developing lymphatic tissue to establish normal communication with the remainder of the lymphatic system. We present a case of retroperitoneal cystic lymphangioma that mimicked malignancy on FDG PET-CT. Inhomogeneous FDG accumulation was found in this multicystic tumor. Histopathologic findings suggested that lymphocytes in the fibrous septa may contribute to FDG uptake of this retroperitoneal cystic lymphangioma.

CystiC Pancreatic Lymphangioma

Lymphangioma of the pancreas is a rare benign tumor of lymphatic origin. Retroperitoneal lymphangiomas account for 1% of all lymphangiomas. Herein, we report a case of cystic pancreatic lymphangioma diagnosed in 34 year-old female patient who was hospitalized for a slight pain in the epigastrium and vomiting. Radiological imaging revealed a large multiloculated cystic abdominal mass with enhancing septations involving the upper retroperitoneum. During the laparoscopic surgery, a well circumscribed polycystic tumor was completely excised preserving the pancreatic duct. The patient made a complete recovery and is disease-free 12 months postoperatively.

Utility of EUS in Diagnosing a Retroperitoneal Lymphangioma Masquerading as a Pancreatic Cyst

Purpose: A 56 year old male veteran was referred to the gastroenterology clinic for evaluation of an incidental retroperitoneal mass on MRI performed for evaluation of back pain. The mass was described as cystic, 6 cm in cephalocaudal dimensions, anterior to the inferior vena cava at the level of renal veins. This mass was not present on a previous MRI scan performed three years ago. The veteran did not complain of abdominal pain, nausea, emesis, jaundice, weight loss or anorexia. No prior history of acute or chronic pancreatitis could be elicited. Physical exam was unremarkable. Basic laboratory tests revealed normal complete blood count, basic metabolic panel, liver function tests and serum lipase. A follow-up CT scan showed a cystic mass near the uncinate process of the pancreas, duodenum, right kidney and the inferior vena cava. Endoscopic ultrasonohraphy (EUS) showed a 6 cm nonseptated cyst posterior to the uncinate process of the pancreas. FNA of this cyst was performed under EUS guidance and yielded 8 ml of milky fluid. Amylase content of the fluid was 51 U/L. Carcinoembryonic antigen (CEA) level in the fluid was reported to be 1.0 ng/ml. Triglyceride level in the cystic fluid was extremely elevated at 2100 mg/dl. Having excluded pancreatic pseudocyst, serous and mucinous neoplasms of the pancreas, a diagnosis of retroperitoneal lymphangioma (chylous cyst) was made. The patient was reassured given the benign nature of the cyst. Since the patient was asymptomatic, surgical excision was not required. Lymphangiomas are rare cystic tumors of the lymphatic system. Retroperitoneal lymphangiomas constitute 1% of all lymphangiomas. Their development is believed to be due to an abnormal connection between the iliac and retroperitoneal lymphatic sacs and the venous system, leading to lymphatic fluid stasis in the sacs. They are mostly asymptomatic but may present with abdominal pain, nausea, vomiting and anorexia as size increases. Pre-operative diagnosis is challenging and is usually a diagnostic dilemma. The final diagnosis of lymphangioma is usually based on pathological examination of the surgical specimen. Prognosis is excellent since they are almost always benign. Through this report, we have highlighted the role of EUS-FNA in the diagnosis of this rare entity and prevented the surgical excision of this benign lesion.

A giant retroperitoneal lymphangioma in a patient with neurofibromatosis type 1

Neurofibromatosis type 1 (NF-1) is a genetically inherited disorder that may cause skin abnormalities and tumors that form on nerve tissues. These tumors can be small or large and can occur anywhere in the body, including the brain, spinal cord, or other peripheral nerves. Retroperitoneal lymphangiomas are very rare benign malformations of the lymphatic system. About 95% lymphangiomas occur in the skin and the subcutaneous tissues of the head, neck and axillary region and the remaining 5% appear in other parts of the body such as lungs, pleura, pericardium, liver, gallbladder, kidney, and the mesentery. Herein, we report the case of a giant retroperitoneal lymphangioma in a patient with NF-1 with a review of the literature.

Retroperitoneal cystic lymphangioma in an adult: A case report and review of the literature

Lymphangiomas are rare benign cystic tumors of the lymphatic system. Retroperitoneal lymphangiomas account for 1% of all lymphangiomas, and approximately 186 cases have been reported. They may clinically present as a palpable abdominal mass and can cause diagnostic dilemmas with other retroperitoneal cystic tumors, including those arising from the liver, kidney and pancreas. This report describes the rare case of a cystic retroperitoneal lymphangioma in a 54-year-old male patient. The lymphangioma had progressed to the point of inducing clinical symptoms of abdominal distention, abdominal pain, anorexia, fever, nausea and diarrhea. Radiological imaging revealed a large multiloculated cystic abdominal mass with enhancing septations involving the upper retroperitoneum and extending into the pelvis. Surgical removal of the cyst was accomplished without incident. A benign cystic retroperitoneal lymphangioma was diagnosed on histology and confirmed with immunohistochemical stains.

Single-Centre Results of Treatment of Retroperitoneal and Mesenteric Cystic Lymphangiomas

Background: Intra-abdominal cystic lymphangiomas are rare and usually present as benign large cystic masses. The treatment of choice of mesenteric and retroperitoneal cystic lymphangiomas is surgical resection. Methods: Seventeen adults, 11 male and 6 female, with a median age of 39 years were investigated. Presentation, treatment, and outcomes of the mesenteric and retroperitoneal cystic lymphangiomas were analyzed. Results: The most common symptom was abdominal pain. The median tumour size was 12.0 cm in diameter. Patients with retroperitoneal lymphangiomas were younger (p = 0.043). However, 4 out of 8 patients with the mesenteric type required bowel resection (p = 0.064). Magnetic resonance imaging allowed a good differentiation of cystic and septal structures. No postoperative complications occurred, but patients with mesenteric types had longer postoperative stays (7.0 vs. 13.5 days; p < 0.001). The long-term outcome of both groups was satisfactory. Conclusions: The surgical results were good with symptom relief. Bowel resection was common in mesenteric cystic lymphangiomas. Because of its higher resolution, magnetic resonance imaging is suggested.

Intraabdominal and Retroperitoneal Lymphangiomas in Adults: Report of Six Cases

This study retrospectively reviewed the clinical features of intraabdominal and retroperitoneal lymphangiomas. We also evaluated the preoperative diagnosis, radiological features, surgical treatment and outcome of these rare tumors. Between 2001 and 2006, six patients underwent surgical resection of abdominal lymphangioma. The clinicopathological data were collected and the patients medical records, operative notes and pathology reports were reviewed. The age at diagnosis ranged from 22 to 57 years. The main clinical symptoms were abdominal distension (n=2), flank pain (n=2) and tenesmus (n=1). One patient was asymptomatic and incidentally detected during the laparotomy. The male-to-female ratio was 1:1. The abdominal lymphangiomas occurred in the mesentery (n=2), retroperitoneum (n=2), jejunum (n=1) and spleen (n=1). All six patients underwent complete surgical resection with or without organ resection, and there was no recurrence at a mean follow-up of two years (ranged from two months to 4.5 years). Adult type abdominal lymphangiomas are benign and indolent. They develop during fetal life and have a chronic history, and thus may present later in adulthood. A complete resection is suggested after the diagnosis is established, and gives a favorable prognosis.

Total Laparoscopic Excision of Retroperitoneal Cystic Lymphangioma

Retroperitoneal cystic lymphangioma is a rare benign tumor of the retroperitoneal lymphatics that usually manifests in infancy. If surgical excision is used in treatment, it needs to be as complete as possible to reduce the risk of recurrence. Two pediatric patients, an 18-month-old girl and a 4-yearold boy, underwent laparoscopic excision of symptomatic retroperitoneal cystic lymphangiomas. Macroscopically, the resection was complete in both cases. The postoperative course in both cases was uneventful. Both children remained asymptomatic and no recurrence was observed at 18-month follow-up. Complete laparoscopic excision should be considered as a therapeutic option to treat retroperitoneal cystic lymphangioma.

Retroperitoneal Lymphangioma

Lymphangiomas are rare cystic tumours that may present as cystic masses in the retroperitoneum. Retroperitoneal lymphangiomas account for approximately 1% of all lymphangiomas. Confusion with other cystic tumours of the retroperitoneum including those arising from the liver, kidney and pancreas is common. A case of a retroperitoneal cystic lymphangioma occurring in a 41-year-old woman raising interesting diagnostic and management issues is reported. The patient presented with vague abdominal pain and persistent nausea. Radiological imaging demonstrated a large multiloculated thin-walled cyst involving the upper retroperitoneum. Surgical resection was complete, revealing a benign cavernous lymphangioma. The patient made a complete recovery and was disease-free 30 months postoperatively.