Cystic lymphangioma of the lesser omentum in an adult patient.

INTRODUCTION: Lymphangiomas belong to the group of benign vascular tumors that originate in the lymphatic tissue. Up to 90% of cases manifest in children before the second year of life. In adults, their presence is very rare. In most cases, they are located in the head, neck and axilla. Intra-abdominal lymphangiomas are very rare and represent less than 1% of all cases. CASE REPORT: The authors present the case of a 64-year-old female patient diagnosed with an intra-abdominal cystic lesion following a routine examination. A CT scan of the abdomen confirmed a cystic lesion located in the lesser omentum between the left lobe of the liver and the lesser curvature of the stomach. The patient was scheduled for laparoscopic exstirpation of the lesion. Histological examination confirmed the clinical diagnosis of cystic lymphangioma of the lesser omentum. CONCLUSION: The etiopathogenesis of lymphangiomas remains unclear. Despite the fact that they are benign tumors, lymphangiomas tend to have an infiltrative pattern of growth, invading surrounding structures. The majority of cases are asymptomatic and the diagnosis is incidental. The gold standard in treatment remains complete surgical extirpation with microscopically negative margins.

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 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.

CYSTIC LYMPHANGIOMA ARISING FROM THE SMALL INTESTINE MESENTERY INCIDENTALLY FOUND DURING SURGERY FOR A LARGE OVARIAN TUMOR - A CASE REPORT.

The aim of this study was presenting significance of diagnostic process in doctor's daily routine. A 45-year-old patient for a planned laparotomy due to left ovarian cyst detected with a routine transvaginal ultrasound. She did not report any symptoms. Computer tomography of the abdomen and pelvis showed a cystic lesion with segmental wall thick¬ening, measuring 133 ☓ 83 ☓ 135 mm, adjacent to the left ovary and the uterus on the left side. At the laparotomy exploration, a giant cyst, ca. 20 cm in diameter was found above the uterus, on the left side, in the area between the lower and middle abdomen. The histological exam results showed lymphangioma of the small and large intestine mesentery. In this case, lymphangioma was found in a rare location, in the mesentery of the ileocecal valve. It did not present any signs and symptoms. CT used in the present case failed to identify the exact point of origin of the lesion.

Neoadjuvant Everolimus for Adult Giant Mesenteric Cystic Lymphangioma with mTOR Pathway Activation.

Cystic lymphangioma are rare benign vascular or lymphatic tumors, diagnosed mostly in newborns or children, that may become life-threatening because of local invasiveness. Surgical "en-bloc" resection with negative margins is the only curative treatment, but some patients are diagnosed with unresectable tumors. We describe the case of a young adult with giant unresectable mesenteric lymphangioma. Extensive pathological characterization as well as whole exome and transcriptome sequencing enabled us to identify mTOR pathway activation within endothelial tumor cells. The patient was treated with everolimus and experienced major partial response, leading to the surgical resection of the residual lesions. This case highlights the importance of molecular characterization of adult cystic lymphangioma for mTOR pathway activation because multidisciplinary therapeutic approaches, including neoadjuvant everolimus and secondary surgery, can lead to complete cure of this rare condition. KEY POINTS: The case of an adult patient diagnosed with giant unresectable mesenteric cystic lymphangioma, in which activation of the mTOR pathway was documented at both the pathological and transcriptomic levels, is reported. This patient showed major partial response to the mTOR inhibitor everolimus, which led to the successful resection of residual tumor lesions after 9 months of treatment. This report shows that mTOR targeting should be considered as neoadjuvant treatment in adult large cystic lymphangioma, as it can lead to complete surgery and cure of this rare condition.

A rare cause of intra-abdominal cysts: pancreatic cystic lymphangiomas.

Echenique et al. described a lymphangioma as a rare cystic neoplasm of the pancreas. We present a similar intra-abdominal lesion diagnosed by endoscopic ultrasound (EUS)-fine-needle aspiration (FNA) cytology.

Giant pancreatic cystic lymphangioma.

A giant cystic lymphangioma in the pancreatic body-tail was diagnosed as an incidental ultrasound mass in a 41-year-old patient, with a progressive size that had increased in the last year by about 20 cm size. An ultrasound guided fine needle puncture was performed and the result was a benign cystic lesion. Given the increase in size, a surgical intervention was decided. A retroperitoneal cystic tumor dependent on the posterior pancreatic wall was identified and a full laparoscopic resection with pancreas and spleen preservation was performed. The pathological report confirmed the diagnosis of benign cystic lymphangioma. The patient was discharged on the fifth postoperative day without any remarkable complications. After one year of follow-up, the patient remains asymptomatic.

Characteristics of adult abdominal cystic Lymphangioma: a single-center Chinese cohort of 12 cases.

BACKGROUND: Cystic lymphangioma is a rare, benign developmental disease, mostly affecting the cervical and axial regions. The clinical features of abdominal cystic lymphangioma (ACL) are reported among pediatric patients but are less well known in adults. The purpose of this study was to demonstrate the clinical characteristics of ACL in Chinese adults and describe our experience in treating this disease. METHODS: We conducted a single-center, non-interventional, retrospective study of 12 adult patients with ACL admitted to Peking Union Medical College Hospital in Beijing, China from November 1984 through August 2017. The demographic, clinical, laboratory, imaging, histopathologic, and therapeutic data were collected. RESULTS: Detailed information on seven males and five females was available. The mean age at diagnosis was 39.1 (SD 17.3) years. The mean duration of follow-up was 6.9 years. Six (50%) patients were asymptomatic, and abdominal pain was the leading symptom for three (25%) patients. The cysts were evaluated by ultrasound (n = 8, 67%), CT (n = 10, 83%), and MRI (n = 4, 33%). Therapeutic modalities include laparotomy (n = 6, 50%), laparoscopy (n = 4, 33%), and aspiration (n = 2, 17%), with variable outcomes. The mean postsurgical hospital stay was 6.8 days. Complete excision was accomplished in eight patients, and one recurrence was observed during follow-up. Of patients who underwent partial resection, one experienced disease relapse. CONCLUSIONS: This is the first report on an Asian cohort of adult patients with ACL. Typical imaging features could lead to timely diagnosis and treatment of ACL. Radical resection is recommended with a longer period of follow-up. The analysis of this cohort deepens our understanding of adult ACL.

Pediatric syndromes with noncraniofacial anomalies impacting the airways.

Syndromes with noncraniofacial abnormalities can be a real challenge in terms of airway management. The key to success is effective preparation, presence of personnel with expertise in difficult pediatric airway management, regular training and familiarity with difficult intubation equipment, and teamwork. Considering that there are a very large number of syndromes, with variable phenotypic expression, the management strategy of every case will be dictated by the anatomical and functional airway as assessed on physical examination and subsidiary examinations before induction of anesthesia.

Clinical image. Chylolymphatic mesenteric cyst in a 3-month old infant.

Chylolymphatic mesenteric cysts are extremely rare among children. Herein we report a case of a 3-month old infant that was admitted to the Emergency Department due to repeated vomiting. Preoperative ultrasonography demonstrated the presence of a thin-walled multiloculated cystic lesion in the right abdomen. Patient underwent then elective surgical excision. Histopathological examination documented the diagnosis of cystic lymphangioma type III, according to Lozanoff classification.

Cystic lymphangioma in the peripheral jejunal mesentery in an adult and excision with laparoscopic-assisted surgery: a case report.

BACKGROUND: Lymphangiomas are uncommon congenital malformations that present mainly in the head, neck, and axillar regions in pediatric patients. Mesenteric cystic lymphangiomas (MCLs), which occasionally present with substantial growth and the invasion of adjacent vital structures, are rarely reported in adults. We report a case of MCL in an adult who was treated with laparoscopic-assisted excision. CASE PRESENTATION: A 40-year-old Japanese man visited his family physician for prolonged periumbilical pain. Plain computed tomography (CT) showed a low-density mass in his left abdomen, and he was referred to our hospital 2 weeks later. His abdomen was flat and soft, and no mass was felt upon palpation. Routine laboratory data showed no abnormalities in the blood cell counts. The levels of tumor markers, such as carcinoembryonic antigen (CEA), carbohydrate antigen 19-9 (CA19-9), and cancer antigen 125 (CA125), were within normal ranges. Contrast-enhanced CT was performed, and a low-density mass was observed with an irregular outline and poor contrast, as well as involvement of the peripheral mesenteric artery and partial compression of the adjacent jejunum without dilatation of the oral side of the bowel. The patient was diagnosed with lymphatic cysts and observed for 1 month without symptom exacerbation. Follow-up CT showed no increase in the size of the mass but showed apparent invasion of the jejunal wall without bowel obstruction. Magnetic resonance imaging (MRI) showed intermediate intensity on T1-weighted imaging (T1WI) and high intensity on T2-weighted imaging (T2WI). The coronal view on T2WI clearly showed an accumulation of cystic lesions. We performed tumor excision with partial resection of the jejunum in a laparoscopic-assisted manner. Pathological examination showed multicystic lesions with an attenuated endothelial lining, surrounding rich adipose tissue and scattered smooth muscle fibers; the patient was diagnosed with MCL. Immunohistochemical assays supported this diagnosis. CONCLUSIONS: This is rare case of MCL presenting in an adult who underwent successful laparoscopic-assisted resection. Mesenteric lymphangioma (ML) should be considered in the differential diagnosis of patients with intraabdominal cysts. Radical excision is optimal, even when the patient is asymptomatic. Laparoscopic-assisted tumor resection is a suitable surgical method for treating MLs located in the peripheral mesentery.

[Lymphangioma of the penis].

Lymphangioma circumscriptum (LC) is a rare, benign condition, predominantly characterized by the malformation of lymphatic skin vessels. The most common sites include the oral mucosa, tongue, skin of the extremities and torso. Lymphangioma of the penis is rare disease. In the paper a clinical case of cystic lymphangioma of the penis, which was initially diagnosed as herpes and human papillomavirus infection, is described. Early diagnosis of skin tumors of anogenital area allows to prescribe a timely therapy. After the CO2-laser vaporization there was no recurrence during the 6-month follow-up period.

Cystic lymphangioma of the lesser omentum in a pregnant woman: a case report and review of the literature.

OBJECTIVE: Lymphangiomas are rare benign tumors which are generally seen in pediatric population and the etiopathogenesis has not yet been understood. They occasionally occur in the head and neck or axillary region with only 5% of them being located in the abdominal or mediastinal cavity. These tumors may be asymptomatic or may cause acute abdominal symptoms due to the location and extention. In the English literature, only 4 cases of lymphangioma were reported to have occurred in the pregnancy period. Herein, we report a case of cystic lymphangioma of the lesser omentum detected incidentally on the ultrasonogram of a 21 year-old, 26-week pregnant woman. The patient was followed up uneventfully during pregnancy. Caesarean section was performed due to transverse presentation of the fetus, and the tumor was completely resected during the same session. The patient is recurrence-free after 1 year of postoperative follow-up.

Failed Minimally Invasive Staged Treatment of a Giant Symptomatic Aortic Perigraft Hygroma after Open Aortic Repair.

BACKGROUND: Perigraft hygromas or seromas are an unusual finding and/or complication after open aortic repair. METHODS AND RESULTS: We present a case of an 82-year-old man with a previous urgent aortic bifurcated graft for abdominal aortic aneurysm rupture. He received several treatments due to abdominal compartment syndrome, requiring a Bogota Bag and colostomy derivation. He was finally discharged home and lost on follow-up. Eight years after this procedure, he presented to the urgency department with an abdominal mass and pain. Urgent computed tomography (CT) scan revealed a giant bilobed aortic sac, corresponding with a huge hygroma. A 3-stage minimally invasive procedure was scheduled due to hostile abdomen. Six months after successful treatment, patient came with fever and abdominal pain. He was diagnosed with graft infection and aortoenteric fistula and was treated with explantation and silver in situ repair. CONCLUSIONS: Aortic hygroma or seromas after open repair should be treated by open means whenever possible. Endovascular techniques could be a valid option in selected patients; however, further evidence is needed.

When Fever, Leukocytosis, and Right Lower Quadrant Pain Is Not Appendicitis.

Mesenteric cystic lymphangioma (MCL) is an uncommon, benign, slow-growing abdominal tumor that is derived from the lymphatic vessels (World J Gastroenterol. 2012;18:6328-6332, Radiographics. 1994;14:729-737). It is most often diagnosed in the head and neck of affected children. Rarely, a lymphangioma can develop within the small bowel (Pan Afr Med J. 2012;12:7). The clinical presentation of patients with an abdominal MCL can range from asymptomatic to acute abdominal pain (J Korean Surg Soc. 2012;83:102-106). We report a case of small bowel volvulus caused by an MCL in a 3-year-old child who presented to the pediatric emergency department with right lower quadrant pain. The child was thought to have a perforated appendicitis and was taken to the operating room where an MCL was identified and resected. This case illustrates the need to consider MCL when a patient presents to the emergency department with right lower quadrant pain.

Perigraft hygroma - a rare cause of post-EVAR aneurysm growth.

Development of perigraft hygromas following repair of abdominal aortic aneurysms is extremely rare. A case is presented of a patient who was found to have a large hygroma despite two re-interventions on a previous EVAR.

[Perirenal cystic lymphangioma in an adult: a case report and literature review].

Lymphangioma is a rare, benign mesenchymal neoplasm, which is characterized by numerous intercommunicating cystic spaces containing lymphatic fluid. It is considered a congenital disease resulting from the obstruction of regional lymph drainage during the developmental period. Lymphangioma frequently occurs in the cervical neck and axilla, also in the retroperitoneum, mediastinum, mesentery, omentum, colon, and pelvis, rarely in the perirenal space. These tumors usually present in childhood, but infrequently, these also present in adults. Patients often complain of hematuria, flank pain, or abdominal pain. Complications of lymphangioma have been reported to include infection, ruputure, or hemorrhage. There are three types of lymphangioma commonly identified: capillary, cavernous, and cystic. Cystic type is the one commonly found intra-abdominally or retroperitoneally, and may be uniloculated or multiloculated. All these perirenal tumors have a very low incidence, make it difficult to diagnose. Differential diagnosis must be performed with the primary renal lymphoma, urinoma, polycystic kidney, teratoma, both benign and malignant tumors, etc. Endoscopic ultrasound guided fine needle aspiration is recommended in some literatures, which may help make diagnosis and further guide subsequent therapeutic strategy. Regarding treatment, surgical excision can be performed via either laparotomy or laparoscopy. And injection of sclerosants into lympahgioma has been described in the literature in nonsurgical candidates. The optimal definitive treatment is total surgical excision. Despite being rare, the tumor has an excellent prognosis. Here, we report a case of a 48-year-old woman with a left renal mass found in an abdominal ultrasonography during a health checkup. In the case presented, abdominal ultrasonography and magnetic resonance urography (MRU) revealed an approximately 11.3 cm×10.6 cm×12.8 cm multilocular cystic mass in the left perirenal space. There was no history of bowel or bladder complaint, either previous illness episodes. Full blood count and kidney function tests were within normal limits. Laparoscopic surgical removal of the cyst was accomplished without incident. A benign cystic perirenal lymphangioma was diagnosed on histology and confirmed with immunohistochemical stains. One month after the surgery the ureteral stent was removed. The patient was free of disease after a 3-month follow-up period. We report the case and discuss the management of perirenal lymphangiomatosis with a literature review.