Intra-abdominal Lymphangiomas Research Articles

A Large Recurrent Retroperitoneal Lymphangioma in a Previously Healthy Adolescent Male: A Case Report

Lymphangiomas are uncommon benign lymphatic vessel tumors that can affect any area of the body served with the lymphatic system. Intra-abdominal lymphangiomas are considered rare, accounting for less than 5 percent of all lymphangioma cases. In this case report, we present a recurrent large retroperitoneal lymphangioma in a previously healthy 15-year-old boy. The patient presented to the Emergency Department with abdominal pain lasting for one week. The pain, described as diffuse, crampy, and non-radiating, gradually increased with time and was aggravated by movement. A large retroperitoneal fluid collection was found on the Computed Tomography (CT) scan. Subsequently, the patient underwent CT-guided drainage, at which 700ml of bloody fluid was extracted. After three months, the patient was readmitted to the hospital for a similar presentation of abdominal fullness and pain. Complete blood count analysis indicated an unremarkable profile, except for a left shift of 82.2% neutrophils and a white blood cell count of 11,300/mcl. A CT scan of the abdomen reported a large, thin-walled cystic lesion in the right hemi-abdomen with minimal thin internal septations, resulting in moderate right hydronephrosis/hydroureter.

Multiple intraabdominal lymphangiomas misdiagnosed as hydatid cysts: a case report and literature review

Multiple intraabdominal lymphangiomas misdiagnosed as hydatid cysts: a case report and literature review

Small Bowel Cystic Lymphangioma: An Uncommon Finding During Single-Balloon Enteroscopy

CASE REPORT Cystic lymphangiomas are rare, congenital, nonmalignant lesions, extremely rare in the gastrointestinal tract.1 We report the case of a 76-year-old man referred to our unit for abdominal pain and anemia. Colonoscopy showed hepatic flexure adenocarcinoma confirmed by a contrast-enhanced computed tomography scan, which also revealed a hypodense and intraluminal mass located in the distal jejunum. Therefore, single-balloon-assisted enteroscopy with an antegrade approach was performed. In the distal jejunum, a yellow, glossy, and polylobate polypoid lesion of 30 mm in diameter was found. The lesion showed a tender, smooth, and squeezable surface: Bite-on-bite biopsies were performed, and a large amount of chylous/purulent fluid leaked from the lesion, which gradually deflated (Video 1). Histopathological report was consistent with cystic lymphangioma. Afterward, the patient underwent right hemicolectomy and jejunal resection. Cystic lymphangiomas result from the failure of lymphatic channels to communicate with the main lymphatic system.1 Cysts may contain serous, chylous, bloody, or purulent fluid. Intra-abdominal lymphangiomas mostly occur in the mesentery, followed by the omentum, mesocolon, and retroperitoneum.2 Involvement of solid or cave organs is extremely rare.3 Lymphangiomas are generally asymptomatic or responsible for nonspecific symptoms, including abdominal pain, diarrhea, and bleeding; volvulus typically occurs in mesenteric small bowel lymphangiomas, requiring urgent surgery.2 Enteroscopy, colonoscopy, and endoscopic ultrasonography are useful tools for the diagnosis and management of abdominal lymphangiomas. For symptomatic cystic lesions or tumors larger than 20 mm, surgical resection is the treatment of choice.4 In selected patients, endoscopic resection is a feasible option.4 Lymphangiomas comprise 3% of benign small bowel tumors5; however, it is expected that the frequency of their detection will increase in the coming years given the augmented diffusion of both video capsule and enteroscopy. Therefore, it is important for small bowel endoscopists to be able to recognize these lesions to offer the best therapeutic options to their patients. {"href":"Single Video Player","role":"media-player-id","content-type":"play-in-place","position":"float","orientation":"portrait","label":"Video 1","caption":"Jejunal cystic lympangioma finding during single-balloon enteroscopy.","object-id":[{"pub-id-type":"doi","id":""},{"pub-id-type":"other","content-type":"media-stream-id","id":"1_4vtc2xhf"},{"pub-id-type":"other","content-type":"media-source","id":"Kaltura"}]} DISCLOSURES Author contributions: S. Cocca, G. Pontillo and G. Grande involved in drafting of the manuscript. G. Grande and G. Pontillo performed enteroscopy. S. Cocca, H. Bertani, S. Russo, A. Mussetto and R. Conigliaro performed critical revision of the manuscript. S. Cocca is the article guarantor. Financial disclosure: None to report. Informed consent was obtained for this case report.

Giant porta hepatis cystic lymphangioma mimicking intra-abdominal abscess

Background: Intra-abdominal lymphangiomas are very rare benign tumors in adults. Here we demonstrate a 22-year-old male who presented with abdominal pain and fevers. He underwent a CT of the abdomen and pelvis which showed multiple, large loculated fluid density lesions extending from the porta hepatis into the entire right hemi-abdomen and into the left upper quadrant. The largest cyst measured 10.4 cm by 1.9 by 26.4 cm. Due to ongoing fevers and concern for infection, the patient underwent percutaneous aspiration of the fluid for concern of intra-abdominal abscess. From the sampled fluid, cultures were negative for growth, cytology was negative for malignant cells, and cell count was within normal limits. The triglyceride level of the fluid was 2739 mg/dL. The patient had moderate relief, but the following day again developed abdominal distension, discomfort, and anorexia. Due to the symptomatic nature of the mass, the patient underwent an exploratory laparotomy with resection of the mass. 4.6 liters of chylous ascites were removed from within the cystic mass. The mass was noted to extend from the porta hepatis into the right retroperitoneum and extend to the left upper quadrant, abutting the pancreas and spleen. The pathology report was consistent with lymphangioma with marked acute fibrinopurulent inflammation and no evidence of malignancy. The patient had immediate relief postoperatively and recovered well from surgery.

Intralesional bleomycin injection treatment of intra-abdominal lymphangiomas presenting with acute abdomen in children.

We evaluated the results of urgent intralesional bleomycin injection (IBI) treatment of intra-abdominal lymphan-giomas (IAL) presenting with acute abdomen in children. The records of patients who underwent urgent IBI due to acutely presenting IAL between January 2013 and January 2020 were reviewed retrospectively in terms of age, presenting symptoms, cyst type, number of injections, pre- and post-treatment cyst volume, clinical response, complications, and follow-up. Six patients with a mean age of 4.3 years (2-13 years) were treated. Presenting symptoms were acute abdominal pain (n=4), abdominal distention (n=1), hypoproteinemia and chylous ascites (n=1). Lesions were of macrocystic type in four and macro and micro cystic in two patients. The median number of injections performed was 2 (1-11). Mean cyst volume reduced dramatically from 567 cm3 (range 117-1656) to 3.4 cm3 (range 0-13.8) after treatment (p=0.028). Treatment response was excellent in four patients with complete resolution of the cysts, while good in the remaining two. No early or late complications or recurrence was observed in a mean follow-up period of 40 months (16-56 months). IBI is a safe, fast, and easily applicable method with satisfactory results in the treatment of acutely presenting IAL. It may be recommended in primary as well as recurrent lesions.

S2216 A Rare Case of Symptomatic Colonic Lymphangioma

Introduction: Lymphangiomas are benign lymphatic lesions, most frequently identified in the head and neck. Colonic lymphangiomas occur but are rare and represent less than 1% of all lymphatic malformations. These lesions are often asymptomatic and discovered incidentally during routine colonoscopy. We present a case of a symptomatic colonic lymphangioma manifesting as a large mass requiring surgical resection. Case Description/Methods: A 42-year-old male presented with the complaint of lower abdominal pain. Associated symptoms included nausea with no vomiting, and he denied family history of gastrointestinal malignancies. At presentation, vital signs were stable and abdominal exam was benign. Laboratory studies were unremarkable, but a Computed Tomography (CT) abdomen/pelvis with contrast revealed a 6.6 x 7 x 6.6 cm colonic mass near the hepatic flexure. Follow up colonoscopy demonstrated a large, subepithelial-appearing mass in the ascending colon protruding more than 90% into the lumen. Retroflexed view revealed 2 x 2 cm ulceration on the proximal aspect of the lesion. Serum tumor markers including CEA, CA 19-9, and CA 125 were all within normal limits and additional CT imaging revealed no metastatic lesions. Biopsies resulted back demonstrating superficial portions of colonic mucosa with mild focal inflammation. However, surgical evaluation was advised given the mass size and the patient thus underwent an elective laparoscopic right hemicolectomy. Tissue samples were sent for evaluation and revealed lymphangiomatosis/lymphatic malformation/cystic lymphangioma centered in the colonic submucosa with extension into the subserosal tissue. No cellular atypia or malignancy was identified, and 24 lymph nodes were benign. These pathology findings were confirmed by expert review at a tertiary center, where it was noted that this case also lacked features of congenital lymphangiectasia. During post-operative follow-up, the patient was reported to be progressing well (Figure). Discussion: Lymphangiomas have an incidence of less than 0.20% and intra-abdominal lymphangiomas represent only a fraction of cases. Although most colonic lymphangiomas are asymptomatic and identified incidentally, they can lead to an array of symptoms and severe complications such as obstruction, intussusception, and perforation requiring surgical intervention if large. Although colonic lymphangiomas are rare, it is important to be aware of their rising incidence to help facilitate accurate and timely diagnosis to prevent complications.Figure 1.: A. Colonoscopy. Submucosal mass in the ascending colon involving more than 90% of the circumference. Figure B. CT axial image. Mass in the ascending colon/hepatic flexure. Figure C. CT coronal image. Mass in the ascending colon/hepatic flexure. Figure D. CT sagittal image. Mass in the ascending colon/hepatic flexure.

LYMPHANGIOMA OF A RARE SITE AND AT RARER AGE OF PRESENTATION: A CASE REPORT

Lymphangiomas are benign lesions characterized by the proliferation of lymphatic spaces, primarily diagnosed in children. It most often occur in the head, neck, axilla, or groin. Infrequently, intra-abdominal lymphangiomas are diagnosed in adults, primarily located in the mesentery. Author wish to discuss this case due to its rare site and age of presentation.

Giant mesenteric cystic lymphangioma: a rare cause of intra-abdominal catastrophe

Lymphangiomas are uncommon benign tumours and occur mainly in children with the most common sites being head and neck followed by the axilla and mediastinum. Intra-abdominal lymphangiomas are rare. Clinical presentation is diverse ranging from incidentally discovered abdominal mass to symptoms of acute abdomen. Life threatening complications are more likely to develop in children. We here present a rare case of giant mesenteric cystic lymphangioma causing intra-abdominal catastrophe in an adult.

An Unusual Case of Small Bowel Lymphangioma

Lymphangioma is a benign condition characterized by proliferation of lymphatic spaces. Usually these vascular tumors are seen mostly in children although some rare cases may be seen in adults. They usually occur in the head, neck or axillary regions. Intra-abdominal lymphangiomas are rare, comprising around only 3% of benign small bowel tumors. The most common gastroenterological lesions are usually located in the mesentery and account for less than 1% of the cases. We present the case of a 67-year- old man who presented to the emergency department based on a call back due to low hemoglobin values on routine labs. He reported increasing fatigue and generalized weakness over the previous two months. He denied melena or hematochezia. Past medical history was remarkable for asthma and hypertension. On physical examination, the patient presented normal vital signs. He was found with generalized paleness. Negative rectal exam. Laboratory data on admission were notable for microcytic anemia with hemoglobin level of 6.3g/dL, transferrin saturation of 4%, low ferritin level (8.8ng/mL), and normal BUN. One year prior to admission hemoglobin level was 16 g/dL. CT scan of the abdomen and pelvis was unremarkable. Colonoscopy was performed and showed one sub-centimeter polyp in the descending colon, not removed based on anemia. Esophagogastroduodenoscopy was unremarkable. Capsule endoscopy revealed a polypoid lesion in the distal jejunum which was further characterized by CT enterography as a large exophytic lesion arising from the mid-jejunum. A push enteroscopy did not reach the jejunal lesion. The patient underwent an exploratory laparotomy and small bowel resection. Pathology report described a jejunal specimen of small bowel which contained a 4 cm intraluminal rubbery jejunal mass consistent with a submucosal lymphangioma. Immunohistochemistry confirmed the reactive nature of the lymphoid infiltrate. Patient had an uneventful recovery. Submucosal jejunal lymphangiomas in an adult are rare. Accurate diagnosis of lymphangiomas may not be established preoperatively despite modern imaging studies. Although most often asymptomatic, small bowel lymphangiomas can present with abdominal pain, gross or occult GI bleeding, obstruction or microcytic anemia as in this patient. It is important to be aware of this rare tumor and possible presentations, as the optimal treatment is radical excision, since incomplete resection may lead to recurrence and further organ invasion.2512 Figure 1 No Caption available.

Small Bowel Lymphangioma: A Rare Cause of Small Bowel Bleeding

Lymphangiomas are benign lesions characterized by the proliferation of lymphatic spaces, primarily diagnosed in children. Infrequently, intra-abdominal lymphangiomas are diagnosed in adults, primarily located in the mesentery. Lymphangiomas are believed to be a primary malformation of the lymphatic architecture and are benign in nature. Lymphangiomas represent embryonic vascular malformations and exist in 3 types: circumscriptum, cavernous, and cystic. The cavernous type can be fast growing and ulcerate, and is the most likely subtype to cause gastrointestinal bleeding. Symptomatic small bowel lymphagniomas are a rare finding and only 15 cases have been described in the literature to date. A 93 year-old male with recently diagnosed atrial fibrillation and initiation of Xarelto presents with a 3-month history of intermittent melena. He presented to an outside hospital with a Hgb of 7.0. After unrevealing upper and lower endoscopies the patient had ongoing bleeding. He subsequently underwent capsule endoscopy, which demonstrated blood throughout the small intestine. He received 10 units of packed red blood cells prior to transfer to a tertiary care center. A push endoscopy was performed and revealed multiple jejunal sub-mucosal masses, the largest of which measured 2.5 cm in diameter and was actively bleeding. The bleeding lesion was biopsied and then successfully treated with argon plasma coagulation (APC).Histopathology revealed irregular dilated vascular spaces consistent with a lymphangioma. The bleeding resolved and the patient was discharged home. Among patients who present with gastrointestinal bleeding, 10% will not have a source identified on upper or lower endoscopy. A better understanding of small bowel pathology is critical to the effective treatment of these lesions. Lymphangiomas compromise approximately 3% of small bowel tumors. Although typically asymptomatic, small bowel lymphangiomas can cause a number of conditions including: GI bleeding, anemia, abdominal pain, volvulus and obstruction. Re-bleeding after treatment with hemoclipping and epinephrine injection is common. While complete resection is the definitive treatment, small bowel enteroscopy with APC treatment can be successfully performed to palliate bleeding from small bowel lymphangiomas in patients who are not surgical candidates with good long-term success.Figure 1

Peripancreatic cystic lymphangioma with secondary hemorrhage: A rare case report

Lymphangiomas are thought to be true neoplasms, hamartomas or lymphangiectasias. Intra-abdominal lymphangiomas are rare and occur most frequently in children. This is a case report of a 27-year-old female with recurrent episodes of the left-sided upper abdominal pain of 2 years duration. She gives a history of intermittent nausea and vomiting. Liver function test and complete blood count with differential were normal. During the initial stages of illness, the serum lipase was elevated; the serum amylase level was normal all through the entire period. The upper gastrointestinal endoscopy suggested hiatal hernia and mild duodenitis. Two computed tomography scans done at 5 months interval showed a hypodense lesion in the distal tail of pancreas with irregular margins. The size of lesions had decreased from 15 mm × 14 mm to 13.5 mm × 10 mm during this period. Endoscopic ultrasound showed ill-defined area in the distal tail of pancreas and pseudocyst was suspected. Magnetic resonance cholangiopancreatography after 2 months showed a cystic lesion in the tail of pancreas of size 11 mm × 10 mm. Due to increasing severity of pain and fainting spells, the patient was taken up for a distal pancreatectomy. The histopathologic examination confirmed a diagnosis of peripancreatic cystic lymphangioma with secondary hemorrhage. During the postoperative period, the drain amylase was high suggestive of grade A pancreatic fistula. Gradually, the levels decreased, the patient became stable and discharged after pneumococcal vaccination.

A Rare Presentation of Lymphangioma in a Patient With Hematochezia

Lymphangiomas are rare benign cysts caused by congenital malformation of the lymphatic system. The incidence of lymphangiomas is between 1.2:1,000 to 4:10,000 births per year. Amongst lymphangiomas 80-90% originate from the head and neck. Intra-abdominal lymphangiomas make up about 5% of lymphangiomas. There is no specific data on the incidence of colonic lesions. We present a case of a 38 year old female with no significant past medical history presenting with bright red blood per rectum. The patient had been having issues with chronic hematochezia since her teenage years, which had been attributed to hemorrhoids. The patient denied any early satiety, nausea, vomiting, unintentional weight loss, or black stools. Physical exam was unremarkable, except for a palpable mass on rectal exam. Laboratory evaluation revealed a microcytic anemia, but otherwise was within normal limits. She had never before undergone endoscopic evaluation. The patient subsequently underwent a full colonoscopy that revealed an extensive network of cyst like structures from just proximal to her dentate line to 15 cm. These lesions were examined radiographically, endoscopically, and via endoscopic ultrasound (EUS). EUS and MRI investigations demonstrated extent of disease down into the perirectal fat region, but with no invasion of adjacent structures. Biopsies of the lesion revealed multiple areas of dilated lymph vessels extending into the muscularis mucosae lined with endothelial cells, diagnostic of lymphangioma. The approach to treatment of lymphangiomas is varied, from conservative expectant course to sclerosant injections and aspiration, to surgical resection. There are reported cases of spontaneous regression, up to 15-20%. Recurrence rates of 50% are reported with surgery. Although very rare, there is a potential risk for malignant transformation. The patient ultimately elected for the expectant course. This case demonstrates a rare cause for hematochezia, which emphasizes the importance of a broad differential diagnosis, and the need to not discount patient's symptoms regardless of prior evaluations.Figure 1Figure 2

Cases of atypical lymphangiomas in children.

Background. Lymphatic malformations or lymphangiomas are rare benign hamartomas that result from maldevelopment of primitive lymphatic sacs. They are most frequently found in the neck and axilla, while intra-abdominal and mediastinal lymphangiomas are uncommon. These are primarily tumours of infancy and childhood and are successfully treated with surgical excision. Summary of Cases. Five cases of lymphangioma comprising three intra-abdominal lymphangiomas and two unilateral axillary lymphangiomas presenting at one institution in Trinidad W.I. between 2005 and 2012 were examined. The presentations, location, workup, treatment, and outcome of these patients were studied. Conclusion. This paper discusses a range of extracervical lymphangioma cases seen at San Fernando General Hospital, Trinidad W.I. We report three intra-abdominal cases and the most common clinical presentations were abdominal pain and distension. Also two axillary cases were reported, which presented as painless axillary masses. The major concerns for excision of axillary lymphangioma by parents and surgeons were cosmesis and feasibility of complete resection without disruption of developing breast tissue and axillary vessels. We believe that ultrasound scan is very good at detection of the lesion, while CT is better at determining tumour content and planning for the operation. It is our opinion that complete surgical excision can be achieved.

Small Bowel Lymphangioma

BackgroundLymphangiomas are uncommon benign tumors found mainly in children. Intra-abdominal lymphangiomas are rare, mostly located in the mesentery. Small bowel lymphangiomas are very rare. Patient and methodsA 38-year-old woman presented with recurrent melena and anemia. Bidirectional endoscopy was non-diagnostic. Balloon enteroscopy revealed a 1 cm actively oozing, soft, friable, polypoid lesion in the proximal-mid small bowel. The lesion was white-yellow with “strawberry” mucosal patterns. Endoscopic tattooing was performed and she underwent subsequent laparoscopic segmental small bowel resection. ResultsHistopathologic features were consistent with a cavernous lymphangioma. At last follow-up, the patient's gastrointestinal (GI) bleeding and anemia had resolved. ConclusionsSmall bowel lymphangiomas can cause gross or occult GI bleeding, anemia, abdominal pain, and/or obstruction. Endoscopists should be aware of this rare tumor and its unique endoscopic features. The optimal treatment is radical excision, since incomplete resection may lead to recurrence. Argon plasma coagulation or polypectomy have been used to achieve endoscopic ablation and palliation of GI bleeding.

Intra abdominal cystic lymphangioma in an adult

Lymphangiomas are benign abnormal lymphatic channels appearing most commonly in head and neck region in newborns and adults [5]. Intra abdominal lymphangiomas are rare in adult population [2]. They may present at rare and uncommon sites e.g. mesentery, omentum, mesocolon and retroperitoneum [9]. Clinical presentation is variable and misleading, hence complex imaging studies are required in their evaluation [6]. This paper reports a case of abdominal cystic lymphangioma in an adult male. We report this case because of its rarity and variable clinical presentations.

VEGF-C and VEGFR-3 in a series of lymphangiomas: Is superficial lymphangioma a true lymphangioma?

Lymphangiomas are commonly regarded as vascular malformations during embryonic development rather than as true neoplasms. VEGF-C and VEGFR-3 are known to be active in the formation of lymphangiomas. However, the significance of the disorders seems to be obscured by confusing different entities. In 114 lymphangiomas, we investigated the clinicopathological features and the expression of VEGF-C and VEGFR-3. The age of patients with lymphangioma circumscriptum or intraabdominal lymphangioma was significantly higher than in patients with cavernous lymphangioma and in patients with cystic hygroma. In cavernous lymphangioma, the age of female patients was significantly higher than in male patients. Five adult cystic hygromas were identified. VEGF-C was detected in 21 of 58 (36%) cavernous lymphangiomas, ten of 28 (36%) cystic hygromas, 0 of 12 (0%) lymphangioma circumscriptum, and four of ten (40%) intraabdominal lymphangiomas. VEGFR-3 was detected in 43 of 58 (72%) cavernous lymphangiomas, 20 of 28 (71%) cystic hygromas, six of 12 (50%) lymphangiomas circumscriptum, and seven of ten (70%) intraabdominal lymphangiomas. VEGF-C was absent from superficial lymphangiomas associated with cavernous lymphangiomas. In typical cases of cavernous lymphangioma, VEGF-C was strongly expressed, suggesting that these cases possessed proliferative activity. In cystic hygroma and intraabdominal lymphangioma, VEGF-C was limited in its distribution. Superficial lymphangiomas more likely represent from peripheral lymphatic dilatation rather than due to growth factor.

Cystic lymphangioma of the lesser sac presenting as acute appendicitis: A case report

Intra-abdominal lymphangiomas usually present by 2 years of age and are uncommon in adults. Cystic lymphagiomas arising from the lesser sac are even more uncommon. We report an unusual case of a lesser sac cystic lymphangioma presenting as acute appendicitis. A 21 year old female was admitted with pyrexia, right iliac fossa tenderness and an elevated C-reactive protein (CRP). At laparotomy, a large fluid filled cystic lesion was observed occupying the right side of the abdominal cavity. The lesion was excised in its entirety and histological diagnosis confirmed lymphangioma. The patient remains well with no evidence of recurrence 1 year post resection.

Intraabdominal lymphangioma: A case report

Discussion Intraabdominal cystic lymphangioma of the mesentery is a rare congenital lesion with a relatively low growth potential. It is typically found in young adults and presents with chronic features. In younger children, the lymphatic malformations are aggressive [2]. Lymphangiomas are prone to recurrence and infiltrating growth. These benign lesions usually present as painless abdominal distension or with complications like volvulus, infection, intracystic hemorrhage and intestinal obstruction. The preoperative diagnosis is difficult, without a high index of suspicion. Most of the cases can be diagnosed USG abdomen [3]. In some cases confirmation of diagnosis may require additional investigations like CT or magnetic resonance Introduction Lymphangiomas are the commonest tumours of infancy and 75% occur in the cervical region. 90% of all lymphangiomas manifest in the initial two years of life. However, intraabdominal lymphangiomas are rare, usually slow growing and manifest in early adulthood [1]. Mesenteric cystic lymphangioma is a rare cause of bowel obstruction and preoperative diagnosis is difficult due to silent clinical course.We present a case of intraabdominal lymphangioma in an infant which presented as a diagnostic dilemma.

Gallbladder lymphangioma: a case report and review of the literature

Lymphangiomas are benign neoplasms usually occurring in childhood and located in the head and neck. Intraabdominal lymphangiomas account for less than 5% of cases. The involvement of the gallbladder is rare. We report a case of a 29-year-old woman who presented with right upper quadrant pain that had persisted for 6 months. Imaging with ultrasound and magnetic resonance imaging (MRI) revealed a multiseptated lesion surrounding the gallbladder. The patient underwent an exploratory laporatomy, and the mass was resected en bloc with the gallbladder. Histological evaluation of the cystic mass revealed findings consistent with lymphangioma. The prognosis is generally good after complete surgical excision, as was the case for our patient.

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.