Abdominal Lymphangioma Research Articles

Cystic lymphangiomatous hamartoma masquerading as massive ascites

We report a 4-year-old boy presenting with a tense massive ascites and large hydrocele. History and physical examination were unremarkable. Routine laboratory studies were normal. Abdominal ultrasonography revealed massive ascites. Contrast CT was suggestive of a large cyst covering the entire peritoneal cavity. At laparotomy, a large cystic tumor was found extending into the scrotum through the left inguinal ring. Histopathologic examination diagnosed the tumor as a cystic lymphangiomatous hemartoma. Although abdominal lymphangiomas are seen in children, but presenting as massive ascites with hydrocele is very rare.

Abdominal lymphangioma in children: Report of three cases

Most cases of abdominal lymphangioma are asymptomatic. However, patients may occasionally present with acute abdomen because of an intestinal obstruction or peritonitis caused by infected cysts, hemorrhaging, and/or torsion. These conditions may differ based on the location of the lymphangioma and do not always require emergency surgery. This report presents two cases of abdominal lymphangioma derived from the omentum, which required emergency surgery because of severe abdominal distension and peritonitis caused by torsion, respectively. In contrast, another case of retroperitoneal lymphangioma presented with acute abdomen and underwent elective surgery after conservative therapy for peritonitis. Elective surgery is acceptable if the symptoms are controllable under conservative therapy. However, it is important not to overlook the possible lethal complications such as intestinal and/or urological obstruction, aggressive peritonitis, and torsion of the cyst, which require emergency surgery.

Diagnosis and treatment for abdominal lymphangioma: an analysis of 20 cases

Diagnosis and treatment for abdominal lymphangioma: an analysis of 20 cases

Small Bowel Lymphangioma

Purpose: Mesentric lymphangioma is a rare benign cystic tumor of lymphatics of the bowel. It is usually found in the first decade of life and has a female predominance in adults. It differs from other mesenteric and retroperitoneal cysts, as it is proliferative and invasive in nature. It is usually asymptomatic in adults and found on surgery or autopsy. It rarely presents as occult GI bleed and iron deficiency anemia. We present such a patient in whom capsule endoscopy was diagnostic. Methods: Our patient is a 36 year old female who presented with fatigue, shortness of breath, occassional diffuse abdominal pain, melena and dizziness for 6 months. She denied any nausea, vomiting, hematochezia, NSAID use or family h/o cancer. Labs revealed Hb of 3.7, Hct 12.5, MCV 60.4, Plt 461, WBC 7.2, Iron 2, Ferritin 3, TIBC 367 and positive FOBT x 3. After transfusion of 4 units PRBC, Hb improved to 9.8. Small bowel enteroscopy with biopsies revealed mild antral gastritis, H. pylori negative and normal villous architecture. Colonoscopy was completely normal. Abdominal CT and Small Bowel series showed luminal narrowing and thickening of small bowel loops in left upper quadrant. Patient was given a patency capsule which passed without any complications. Capsule endoscopy revealed a mass like lesion with yellowish – white discoloration with active bleeding in the small bowel. Laparoscopic resection of the lesion showed diffuse dilatation of the mucosal, submucosal and subserosal lymphatics consistent with small bowel lymphangioma. The post operative course was uneventful and patient was discharged. Results: Conclusion: Abdominal lymphangiomas are uncommon benign tumors. The mean age at presentation is 2.2 years with a male predominance. Etiology may be benign proliferation of ectopic lymphatics. 95% of lymphangiomas are found in the neck and axilla and very rarely found in the intestine. Clinical presentation include abdominal pain, distention, fever, and vomiting. There may be features of small bowel obstruction or volvulus. In an Australian study of 416 capsule endoscopies, 27 tumors were identified of which only 1 was a lymphangioma. Plain radiography may demonstrate small-bowel obstruction or noncalcified soft-tissue mass. CT and MRI may show multiloculated fluid-filled masses, thickening of the bowel wall and reveal size of the tumor, characteristics of the cyst wall and location. Capsule endoscopy may lead to earlier detection and treatment and an improved prognosis for patients with these neoplasms. The prognosis of lymphangiomas depends on location and extent of the lesion. Complete resection is the treatment of choice and has an excellent prognosis. The recurrence rate ranges from 0–13.6%. Malignant degeneration to a low-grade sarcoma is rare.

Several topics in diagnosis and treatment of abdominal lymphangioma

Several topics in diagnosis and treatment of abdominal lymphangioma

Small Intestinal B-Cell Lymphoma in a Patient With Lymphangiectasia Secondary to Abdominal Lymphangioma

Small Intestinal B-Cell Lymphoma in a Patient With Lymphangiectasia Secondary to Abdominal Lymphangioma

Prenatal ultrasonographic diagnosis of abdominal cystic lymphangioma: A case report

Abdominal lymphangioma is a rare tumor of the lymphatic vessels. A case of an abdominal cystic lymphangioma identified at 22 weeks of gestation is reported. Ultrasonographic monitoring showed a progressive increase of mass size during the gestation. Pregnancy was terminated at 38 weeks and the newborn was submitted to a laparotomy with resection of all cystic structures. At the present time the infant is three years old and is doing well.

Cystic Lymphangioma of the Gallbladder: Report of a Case

Abdominal lymphangioma is usually diagnosed within the first 2 years of life and is extremely rare in adults. The most common location of abdominal lymphangioma is the mesentery, but there are sporadic reports of its development in the gallbladder. A 66-year-old woman was found to have a cystic lesion near the gallbladder. Preoperative studies, including endoscopic ultrasonography, computed tomography, and magnetic resonance imaging, showed a tumor with multilocular cystic structure, originating in the gallbladder fossa. The patient underwent exploratory laparotomy, and the mass was resected en bloc with the gallbladder, as there was no evidence of malignancy on intraoperative ultrasonography. Macroscopically, the tumor was a multilocular cystic mass, 6 x 3 x 2 cm in size, with a rough, sponge-like appearance. Histologically, the cystic tumor was diagnosed as a lymphangioma, originating in the gallbladder. To our knowledge, only three other cases of a cystic lymphangioma originating in the gallbladder have been reported in the medical literature of the world.

Abdominal lymphangiomas in children: interest of the laparoscopic approach

Lymphangiomas are rare benign lesions of the lymphatic system. The most common symptoms are abdominal tumor or "acute abdomen" in children. The treatment of choice is complete surgical resection, but the recurrence rate with incomplete resection is high, and laparotomy exposes the patient to adhesions. The authors report their experience with the lymphangioma laparoscopic approach. This retrospective study examined 15 consecutive operations for lymphangiomas in children, ages 5 months to 14 years, treated during the 5-year period from 1999 to 2004. Six patients were treated using the primary laparotomy approach, and nine patients underwent the laparoscopic procedure, six successfully. Three conversions were necessary (1 case requiring partial colectomy, 1 retroperitoneal case with adherence on the aorta and vena cava, 1 case with partial volvulus). Morbidity included two cases of acute occlusion caused by adhesions after laparotomy. There was no recurrence of lymphangioma during a mean follow-up period of 35 months. The laparoscopy procedure could be used successfully for abdominal lymphangioma, even in an emergency. When the laparoscopic resection is impossible, laparotomy or sclerotherapy can be discussed.

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.

A huge Omental Lymphangioma with extention into Labia Majorae: A case report

BackgroundAbdominal cystic lymphangiomas are uncommon congenital benign tumors.Case presentationWe present a case of a 4 year old female child with a cystic lymphangioma arising from greater omentum and occupying whole of the abdomen and protruding through labia mejora. Ultrasonography and CT scan confirmed the diagnosis. Complete excision of the cyst along with omentectomy done with no clinical or radiological evidence of recurrence till 6 months.ConclusionDue to variable presentation of abdominal lymphangiomas, extensive imaging studies are necessary for evaluation and diagnosis. Complete surgical resection is a treatment of choice.

Fetal abdomino‐perineal lymphangioma: differential diagnosis and management

Fetal lymphangiomas are rare congenital anomalies of the lymphatic system most commonly presenting in the head and neck. Cystic abdominal lymphangiomas are more rare with only a few cases reported prenatally. We report a case of a prenatally detected abdomino-perineal lymphangioma that mimicked the more fatal prenatally detected sacrococcygeal teratoma (SCT), which resulted in one caregiver suggesting termination of the pregnancy. This case demonstrates the importance of carefully considering the differential diagnosis of fetal abdomino-perineal masses when counseling parents.

Abdominal Lymphangiomas in Adults

Abdominal lymphangiomas are rare benign cystic tumors that can become locally invasive and often require resection. They arise in all ages and have a variable presentation. We performed a retrospective review of a single institution surgical experience with this lesion in adults. The pathology prospective database was reviewed to identify patients with surgically resected abdominal lymphangiomas from January 1986 to May 2004. Retrospective review and follow-up was performed for each patient. The six patients with abdominal lymphangiomas ranged in age from 38 to 66 years. They presented with a variety of signs and symptoms. All underwent CT scan that demonstrated a cystic lesion, but in only one third was the diagnosis made preoperatively. Tumors were located in the retroperitoneum, small bowel mesentery, liver, and pancreas. Five of the six tumors were completely resected. Two of the six required resection of adjacent or involved organs. Follow-up ranged between 6 months and 18 years. All had symptomatic relief after resection, and no patient showed evidence of recurrence in this time period. Abdominal lymphangiomas are rare. The correct diagnosis often remains elusive until tissue is obtained. The treatment of choice is complete surgical resection. When completely resected, these lesions seem not to recur, and the overall prognosis is excellent.

TIE2 Gain-of-Function Mutation in a Patient with Pancreatic Lymphangioma Associated with Blue Rubber-Bleb Nevus Syndrome: Report of a Case

Abdominal lymphangioma is a rare tumor in adults. The most common location is the mesentery, but this tumor occasionally develops in the pancreas. We report a case of pancreatic lymphangioma associated with blue rubber-bleb nevus syndrome (BRBNS) in a Japanese woman. The pancreatic lymphangioma spread extensively throughout the retroperitoneum without causing any symptoms for more than 4 years after its histological diagnosis by laparoscopic biopsy. Multiple hemangiomas were also seen in the mucous membranes and on the skin. The hemangiomatosis was segregated in the dominant fashion in her family, and a germ-line gain-of-function mutation (Arg849Trp) in TIE2 gene was confirmed. To our knowledge, this is the first report of pancreatic lymphangioma occurring in association with BRBNS in a patient with genetic alteration. We describe the clinical features of this case and discuss a possible correlation between these two uncommon conditions.

Intra-abdominal lymphangioma.

The historical background, aetiology, clinical features, radiographic findings and treatment of abdominal lymphangiomas are reviewed. The condition may give rise to the acute surgical abdomen.

Colonic Lymphangiomatosis Associated with Protein-Losing Enteropathy

Most lymphangiomas are considered to be lymph vessel anomalies. They can occur anywhere in the body and only rarely affect the intestinal tract. However, as endoscopic examination has become more widespread, gastrointestinal lymphangiomas have been reported more frequently. The overwhelming majority of lymphangiomas are solitary; only several cases of multiple lymphangiomas of the colon have been reported so far (1‐3). Here we report multiple colonic and abdominal lymphangiomas in a young Korean woman who presented with protein-losing enteropathy.

Cystic Lymphangioma of the Small Bowel in a Woman at 15 Weeks?? Gestation

Lymphangiomas are uncommon benign tumors, usually encountered early in childhood. Here is described the case of a pregnant woman at 15 weeks' gestation with a symptomatic ileal small bowel lymphangioma that was approached laparoscopically. A limited number of laparoscopically managed cases of abdominal lymphangiomas have been reported. This is the first reported laparoscopically assisted resection of a small bowel cystic lymphangioma during pregnancy.

Intra‐abdominal and Retroperitoneal Lymphangiomas in Pediatric and Adult Patients

Intra-abdominal and retroperitoneal lymphangiomas are a rare, congenital malformations of the lymphatics, which are found predominantly in children. The aim of this study is to evaluate the clinical features of this tumor, highlighting the differences in adults and pediatric patients. We also evaluate the preoperative diagnosis, radiological features, surgical treatment, and outcome of this rare condition. Between 1990 and 2004, 14 patients who underwent surgical resection of an intra-abdominal lymphangioma were reviewed retrospectively. There were five pediatric patients between fetal age and 17 years of age and nine adults between 31 and 62 years of age. Overall, females outnumbered males in the series, with a male-to-female ratio of 3:4. However, males predominated in the pediatric age group with a male-to-female ratio of 1.5:1. The clinical presentation of children was more acute ranging from 3 days to 2 months. In adults, four patients were asymptomatic, and the remaining five had symptom duration ranging from 2 weeks to a year. The lymphangiomas occurred in the mesentery (n = 4), retroperitoneum (n = 4), omentum (n = 3), pancreas (n = 2), and spleen (n = 1). All the patients underwent total surgical resection with or without organ resection, and there were no recurrences at a median follow-up of 2 years (range; 3 months--13 years). This series demonstrates that abdominal lymphangiomas have a male preponderance and present more acutely in pediatric patients, whereas in adults, female patients predominate and the history is more chronic.

Intraabdominal cystic lymphangiomas obscured by marked superimposed reactive changes: clinicopathological analysis of a series

Cystic (or cavernous) lymphangiomas are uncommon tumors that most often occur in the head and neck, axilla, or groin of young children but are detected occasionally in adulthood at various other anatomic sites. When arising in the abdomen, cystic lymphangiomas may present with acute abdominal pain. We have encountered examples of mesenteric and retroperitoneal cystic lymphangiomas associated with such marked superimposed reactive and inflammatory changes that their lymphatic nature is obscured, a situation that is not widely recognized. To further characterize these lesions, 7 abdominal lymphangiomas associated with florid reactive changes were retrieved from the authors' consultation files. There were 5 female patients and 2 male patients (median age, 42 years; range, 1 month to 51 years). Five cases presented in adulthood. Tumor size ranged from 8 to 20 cm (median, 15 cm). Three tumors arose in the mesentery of the small intestine and 4 arose in the retroperitoneum (one of which also involved the posterior mediastinum). Three patients presented with a short history of abdominal pain. Radiological studies revealed large cystic or solid masses; clinical differential diagnoses included sarcoma (2 cases), enteric duplication cyst (2 cases), and cystic tumor not otherwise specified. Grossly, the tumors were generally multiloculated cystic masses associated with areas of fat necrosis and hemorrhage. The cysts often contained thick, gelatinous, or milky fluid. Histologically, all cases showed extensive areas of granulation tissue, most also including a floridly cellular reactive myofibroblastic proliferation, obscuring the lymphatic nature of the lesion. Two cases contained extensive areas of xanthogranulomatous inflammation. In foci where the underlying lesion could be discerned, the tumors were composed of cystically dilated lymphatic spaces, some of which were partially invested by a layer of smooth muscle and were associated with occasional lymphoid aggregates. The lymphatic spaces contained either clear fluid or large numbers of foamy macrophages. The lymphatic endothelial cells lining the cystic spaces were generally attenuated with no cytological atypia. One case showed features of a complex vascular malformation with a predominant component of cavernous lymphangioma. By immunohistochemistry, in all cases, the endothelial cells lining the dilated lymphatic spaces were positive for CD31 and D2-40, 4 of 7 were positive for CD34, and all were negative for keratin. Clinical follow-up information was available for 4 patients (median, 26 months; range, 22-36 months): 3 patients had no evidence of recurrence and 1 patient was asymptomatic with radiographic evidence of minimal persistent disease. In summary, some intraabdominal lymphangiomas have a tendency to induce marked reactive and inflammatory changes in the surrounding tissues, often obscuring their nature and occasionally leading to the clinical impression of a malignant tumor. Awareness of this unusual occurrence will lead to the correct diagnosis.

Abdominal lymphangiomas: imaging features with pathologic correlation.

Abdominal lymphangiomas: imaging features with pathologic correlation.