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BACKGROUND: Lymphangioma is a rare cystic tumor that occurs across different locations. Retroperitoneal lymphangioma accounts for about 1% of all lymphangiomas. In this study, we report the clinicopathological features of retroperitoneal lymphangioma and describe our experience in treating this disease. METHODS: We collected clinical data from all patients who were pathologically diagnosed with retroperitoneal lymphangioma at Zhejiang Provincial People's Hospital, between June 2013 and August 2022. RESULTS: The 7 and 8 male and female patients analyzed herein had a mean age of 48.6 (SD 14.24) years at diagnosis. The mean duration of follow-up was 4.7 years. Among them, 66.67% were asymptomatic, with the rest manifesting abdominal pain, nausea, low back pain and elevated blood pressure as the main symptoms. Preoperative diagnosis and evaluation of cysts were mainly performed via computed tomography (CT) (n = 10, 66.67%) or magnetic resonance imaging (MRI) (n = 8, 53.33%). All patients were completely resected following surgery. Immunohistochemical analysis, performed on 6 patients, revealed that they were positive for D2-40. A total of 4, 4 and 3 patients were positive for CD31, CD34 and SMA, respectively. Moreover, the study cohort had an average postoperative hospital stay of 6.6 days. Follow up, after the end of the study, revealed no relapse in any of the 15 patients. CONCLUSIONS: Lymphangioma is a benign tumor of the lymphatic system. Although typical imaging features can be accurate for preoperative diagnosis, histological examination is crucial to final confirmation. Complete surgical resection is the best option to limit the risk of recurrence in cases with symptomatic lesions.

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Lymphangioma is a rare, benign tumor of the lymphatic system. It is believed to be a congenital malformation, when part of the lymphatic channels fail to connect to the main lymphatic system. Lymphangioma is a tumor of the pediatric age, with 50% of patients presenting at birth. The head and neck are the main affected sites (75%), while the retroperitoneal cavity is the least affected area, and comprises less than 1% of cases. Adult lymphangioma is an extremely rare tumor, and adult retroperitoneal lymphangioma (ARL) is even a rarer tumor. Over the last two decades, we have experienced a significant increase in reports published in the English literature discussing ARL. As reports have increased, several questions about previously known facts regarding this tumor arose: For years, it was known that ARL is usually an asymptomatic tumor which is incidentally found - is it a true claim? Is abdominal magnetic resonance imaging the radiological test of choice for diagnosis? What is the best therapeutic option? The main aim for this article is to review the current and old English literature concerning ARL, in order to collect data regarding demographic features, clinical presentation, imaging tests used for diagnosis, therapeutic options and follow-up. This in turn will give precise updated answers for the previous questions. In addition, it will raise awareness for the treating physician regarding the most effective approach for early diagnosis and best therapeutic option to be selected.

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

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Lymphatic malformation (LM) is the currently preferred term for what was previously known as lymphangioma. Retroperitoneal LMs are extremely rare, benign, cystic masses that arise from lymphatic vessels. They can be challenging to diagnose because they resemble other retroperitoneal cystic tumors. The development of treatment strategies for rare diseases, including retroperitoneal LM, requires the acquisition of new knowledge to enhance our understanding of the disease progression. Therefore, we present an update regarding fundamental and advanced issues associated with retroperitoneal LM. This review describes the epidemiology, histopathology, biomedicine, clinical manifestations, radiological features, differential diagnosis, and management of this lesion.

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Lymphangiomas are classified as lymphatic malformations, which are more common in children and rare in adults. It frequently occurs in the cervical and axillary regions and uncommonly in the retroperitoneum. A 39-year-old woman presented to our department for the investigation for a 55 mm asymptomatic mass in the right anterior adrenal cavity. Abdominal ultrasound showed a tumor containing cysts in the right anterior adrenal cavity. Contrast-enhanced computed tomography showed that the tumor was poorly contrasted and ill-defined. Magnetic resonance imaging suggested that the tumor contained a small amount of fat. The tumor tended to grow, and the possibility of malignant diseases such as liposarcoma could not be excluded. Therefore, surgical resection was performed. Since intraoperative findings showed that the tumor tightly invaded to the duodenum and pancreatic head, a pancreaticoduodenectomy was selected. The entire tumor was removed without exposing the tumor. Macroscopic findings indicated that the specimen was 55 mm in size, indistinctly demarcated, yellow-white in color, and polycystic. Histologically, lymphovascular proliferation was observed with infiltration of the pancreatic head and the duodenal muscle layer. The diagnosis of lymphangioma was finally made. There was no recurrence 2 years after surgery.

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Retroperitoneal lymphangiomas (RL) are rare benign cystic tumors of the lymphatic system, which account for 1% of all lymphangiomas, and till now less than 200 cases have been reported. We are presenting a 35 years old woman, who was admitted with symptoms of recurrent left lumbar pain. The preliminary diagnosis from CT scan was retroperitoneal cyst. The patient went under explorative laparotomy and whole cyst mass was removed from the surrounding structures using sharp and blunt dissection.

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Objetivo: Presentar el caso de una paciente con linfangioma cavernoso diagnosticado incidentalmente y tratado quirúrgicamente, primero por laparotomía y después, para su resolución final, mediante laparoscopia. Se discuten las ventajas de la cirugía miniinvasiva como método adyuvante en la resección del linfangioma residual después de una laparotomía extensa. Sede: Hospital de tercer nivel. Diseño: Presentación del caso y revisión de la literatura. Descripción del caso: Mujer de 43 años, en la cual se documentó masa quística aparentemente dependiente del ovario derecho, con marcadores tumorales para cáncer ovárico normales. Se programó para salpingoclasia y resección de quiste ovárico, por incisión Pfannenstiel, encontrando un quiste gigante de origen retroperitoneal, que se extendía desde el hueco pélvico hasta el abdomen superior. Fue extirpado casi en su totalidad, debido a la extensión de la tumoración, excepto en la porción pancreatoduodenal y retrohepática, ya que al intentar abordar esta zona, inició con sangrado transoperatorio y se desconocían las relaciones del tumor con estos órganos. Se difirió la resección de esta porción residual para complementar su estudio por imágenes e histopatología y llevarlo a cabo en un segundo tiempo quirúrgico. La tomografía postoperatoria no mostró dependencia pancreatoduodenal, biliar o vascular. Al mes, se programó para la resección laparoscópica del tumor residual. El reporte histopatológico fue linfangioma cavernoso en ambos casos. Conclusiones: El tratamiento para los linfangiomas retroperitoneales es la resección completa. La cirugía miniinvasiva es una alternativa útil, ventajosa y mejor aceptada por los pacientes en algunas reoperaciones planeadas, donde inicialmente se realizó cirugía abierta.

Objective: To present the case of a woman with cavernous lymphangioma incidentally diagnosed and surgically treated, first through laparotomy and later, for its final resolution, through laparoscopy. We discuss the advantages of mini-invasive surgery as an adjuvant method for the resection of a residual lymphangioma after extensive laparotomy. Setting: Third level health care hospital. Design: Case presentation and review of the literature. Case description: A 43-year-old woman, in whom a cystic mass, apparently depending on the right ovary was documented; tumor markers for ovarian cancer were normal. She was programmed to undergo salpingoplasty and resection of the ovarian cyst, through Pfannenstiel incision. During surgery a giant cyst of retroperitoneal origin was found, which extended from the pelvic hole to the upper abdomen. It was resected almost totally due to the extension of the tumor, except for the pancreatoduodenal and retrohepatic region, because, when attempting to approach this area, transoperative bleeding started and we did not know the relations of the tumor with these organs. The resection of this residual portion was deferred to be able to complement the diagnosis with imaging and histopathology studies and perform the resection at a second surgical time. Postoperative tomography revealed no pancreatoduodenal, biliary, or vascular dependence. After 1 month, the patient was programmed for laparoscopic resection of the residual tumor. The histopathological report corresponded to cavernous lymphangioma in both situations. Conclusions: Treatment of retroperitoneal lymphangiomas consists of complete resection. Mini-invasive surgery is a useful, valuable option and a better accepted alternative by patients in some planned re-operations where initially open surgery had been performed.

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Cystic retroperitoneal lymphangioma is an unfrequent pathology. Most cases are asymptomatic, and when symptomatic, they tend to present as abdominal pain and bowel obstruction secondary to compres-sion of the bowel loops. Treatment of choice is surgical excisión. We present a case of cystic retroperitoneal lymphangioma with adhesions to the second portion of duodenum and head of the pancreas. Once separated from the pancreas, it remained denudated. To avoid an eventual pancreatic fístula, Tachosil® was placed as sealing method. Postoperative course was uneventful.

El linfangioma quístico retroperitoneal es una patología de poca frecuencia. La mayoría de los casos son asintomáticos y cuando presentan síntomas, lo más frecuente es dolor abdominal u obstrucción intestinal por compresión de asas intestinales. Su tratamiento consiste en la extirpación completa de la lesión. Presentamos un caso de linfangioma quístico retroperitoneal adherido a 2a porción duodenal y cabeza pancreática. Al separarlo del tejido pancreático, quedó un borde cruento. Para evitar una fístula pancreática, se colocó una placa de Tachosil® a modo de sellante. No aparecieron complicaciones postoperatorias.

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Lymphangiomas are congenital malformations of the lymphatic system that account for about 5% of all benign tumors in infants and children.(1) The most common sites are the neck and axilla, which account for 95% of cases.(2) Abdominal cystic lymphangiomas are quite rare, and can arise from either the retroperitoneum, gastrointestinal tract, or the mesentery of the abdominal viscera.(3) The presenting symptoms are painless abdominal distension, a palpable mass, or secondary complications in the abdomen such as intestinal obstruction, volvulus, intestinal infarction, or bleeding.(4) Typically diagnosed during childhood, these tumors prompt surgical intervention. We describe an atypical case of an abdominal cystic lymphangioma, which did not manifest until adulthood, with atypical symptoms of a rapidly expanding and symptomatic mass.