Described as rare tumors of the lymphatic system, lymphangiomas were initially divided into three main groups based on the size of lymphatic spaces and vessels - capillary (comprised of thin and small lymphatic vessels), cavernous (dilated channels) and cystic, which contains numerous lymphatic spaces with connective tissue and smooth muscles, but without connection to the proximal lymphatics [1] [2] [3] [4] [5]. The vast majority of lymphangiomas are diagnosed by the age of 2 (presumably due to pathological events during embryogenesis), whereas rare adult cases are mainly related to trauma or infections [1] [2] [6]. The most common locations are the head and neck area (particularly the posterior triangle), as well as the axilla, constituting 75% and 20% of patients, respectively [1] [2] [7]. However, virtually any site in the body can be a location of cystic lymphangiomas, including the abdomen (the retroperitoneum and the mesentery of the small bowel are the two main sites, but the pancreas, the spleen, the colon, the liver, and other organs have been described to be involved by lymphangiomas) and the mediastinum [1] [2] [7] [8] Even though the clinical course is often asymptomatic, a progressively enlarging slow-growing mass is the main sign of cystic lymphangiomas, particularly developing in the cervical region, but many symptoms have been reported depending on the location [1] [2]. Abdominal cystic lymphangiomas, comprising a small percentage of cases, can present with abdominal pain, nausea, vomiting, and loss of appetite [1] [4] [7]. Furthermore, descriptions of ascites, bleeding, or rupture of lymphangiomas in the retroperitoneum has been documented, mainly in association with tumor enlargement and subsequent compression of adjacent tissues [1] [4].

• Pain

  We describe a case of a 12-years old girl who was admitted in our hospital with abdominal distension and pain. Physical examination showed an abdomino-pelvic mass. [ncbi.nlm.nih.gov]

• Swelling

  Swellings can be quite large, and they can change over time, becoming larger or smaller depending on how much fluid they contain. [rch.org.au]

  We herewith present a rare case of cystic lymphangioma of the scrotum in an ado-lescent who presented with an incidental scrotal swelling with no other abnormality, where the diagnosis was suspected on scrotal ultrasonography. [ncbi.nlm.nih.gov]

• Lymphadenopathy

  It is usually due to mediastinal lymphadenopathy or involvement of the thymus gland. Cystic lymphangiomas are benign tumors of the lymphatic system, and less than 1% present as a solitary mediastinal mass. [ncbi.nlm.nih.gov]

  There was no evidence of lymphadenopathy on the CT scan. Furthermore, the level of the CA 19-9 tumor marker was within the normal range which made the diagnosis of malignancy less likely. Figure 1. Abdominal CT scan. [pancreas.imedpub.com]

  At physical examination was found with adequate nutritional status, without jugular engorgement or axillary lymphadenopathy; old surgical scars in anterior axillary line and third left intercostal space. [sciforschenonline.org]

  It is an aggressive malignancy and can invade adjacent structures such as the salivary glands and metastasize early in its course resulting in lymphadenopathy. [doi.org]

• Pathologist

  At histology they can be either circumscribed or locally infiltrative, and may pose diagnostic difficulties when the pathologist relies on morphology alone or is challenged on frozen section. [ncbi.nlm.nih.gov]

  Shekoufeh Hosseini (Senior Pathologist; Department of Pathology, Iran Mehr Hospital - Tehran, Iran) in helping us to prepare this case report. [pancreas.imedpub.com]

  Pediatricians, pediatric surgeons, neurosurgeons, ear-nose-throat specialists (otolaryngologists), speech pathologists, eye specialists (ophthalmologists) and other healthcare professionals may need to systematically and comprehensively plan an affect [rarediseases.org]

• Aspiration

  We present two cases of a rare pancreatic lesion that were diagnosed by endoscopic ultrasound with fine-needle aspiration. [ncbi.nlm.nih.gov]

  The patient was referred to the Pathology Department for fine needle aspiration cytology. Watery material came out as soon as the first needle prick was made. However, an attempt to aspirate with a syringe yielded no material. [mjdrdypu.org]

  Fine-needle aspiration cytology of lymphangioma of the parotid gland in an adult. Diagnostic Cytopathology, Vol. 24, Issue. 2, p. 126. [cambridge.org]

• Vomiting

  We herein describe a case of a 9-day-old female presented with a two-day history of bilious vomiting, feeding intolerance, weakness and lethargy. Preoperative studies were diagnostic of intestinal malrotation. [ncbi.nlm.nih.gov]

  Abdominal cystic lymphangiomas, comprising a small percentage of cases, can present with abdominal pain, nausea, vomiting, and loss of appetite. [symptoma.com]

  She gives a history of intermittent nausea and vomiting. Liver function test and complete blood count with differential were normal. [ccij-online.org]

• Nausea

  We report a case of a cystic lymphangioma arising from the lesser curvature of the stomach in a 16-year-old female who initially presented with abdominal pain, nausea, and emesis. [ncbi.nlm.nih.gov]

• Constipation

  Other associated manifestations were pallor, anorexia, fever and constipation. Plain x-ray of the abdomen showed a gasless space-occupying lesion displacing the bowel loops in seven patients. [jusurgery.com]

  […] strongly recommended even in asymptomatic cases because of the potential to grow, invade vital structures and develop life-threatening complications from associated volvulus. [3] Case Report A 2-month-old female child had history of abdominal fullness and constipation [jcnonweb.com]

  Lymphatic malformations affecting the gastrointestinal tract or pelvis can cause constipation, bladder obstruction, recurrent infection or protein loss. Lesions in bone can be associated with bone overgrowth or bone loss. [rarediseases.org]

• Diarrhea

  It may be clinically silent and discovered accidently during investigation or laparotomy for unrelated pathology or may mimic a variety of vague abdominal symptoms like abdominal discomfort, distension, diarrhea, anorexia, nausea and vomiting. [jusurgery.com]

• Heart Disease

  Mazatti L, Cacciari E; Italian Study Group for Turner Syndrome: Congenital heart disease in patients with Turner syndrome. J Pediatr 1998;133:688–692. [karger.com]

• Neck Mass

  We report a case of a female infant with a prenatal diagnosis of superior caval vein aneurysm presenting at birth with a neck mass that was found to be a cystic lymphangioma. [ncbi.nlm.nih.gov]

  Congenital cystic masses of the neck: radiologic-pathologic correlation. Radiographics 1999;19:121-46. [ Crossref ] [ PubMed ] Malik A, Odita J, Rodriguez J, Hardjasudarma M. Pediatric neck masses: a pictorial review for practicing radiologists. [doi.org]

  Differential diagnosis of neck masses. In: Flint PW, Haughey BH, Lund V, et al, eds. Cummings Otolaryngology: Head & Neck Surgery. 6th ed. Philadelphia, PA: Elsevier Saunders; 2015:chap 198. Tower RL, Camitta BM. Abnormalities of lymphatic vessels. [nlm.nih.gov]

  Guarisco JL (1991) Congenital head and neck masses in infants and children. Ear Nose Throat J 70:75–82 PubMed Google Scholar 6. Hancock BJ, St-Vil D, Luks FI, Di Lorenzo M, Blanchard H (1992) Complications of lymphangiomas in children. [link.springer.com]

• Neck Swelling

  Leukemia is a common childhood cancer which can present as a lymph node swelling in the neck. [doi.org]

• Confusion

  Although PCL is very rare in adult patients, it can cause confusion due to the presence of other cystic pathologies of the pancreas. Complete excision of the cyst is mandatory to prevent recurrences. [ncbi.nlm.nih.gov]

  Although retroperitoneal lymphangiomas may sometimes be asymptomatic, they usually present as a palpable abdominal mass, and are easily confused with other retroperitoneal cystic tumors including those arising from the liver, kidney and pancreas. [radiopaedia.org]

  It is very common to confuse them with venous lesions and thereby delay treatment and mismanagement, with the dire consequences observed in the clinical practice by delaying their surgical removal [8]. [sciforschenonline.org]

  The anterior mediastinal seats of our case tend toward confusion with other tumours, such as a specifically local congenital thymic cyst, a cyst or a bronchogenic teratoma. Similarly, a low anterior location would suggest a pleuropericardial cyst. [err.ersjournals.com]

The diagnosis of cystic lymphangiomas is made through a comprehensive clinical approach and implementation of appropriate imaging studies. With a properly performed physical examination, the physician can identify the key features of a cystic lymphangioma - a fluctuant, soft, and fixated mass that is non-tender on palpation [2]. To confirm the disease, however, imaging studies need to be employed. Ultrasonography may be used as a first-line method, revealing sharply delineated cystic septations [1] [5], whereas plain radiography of the chest shows a well-defined homogeneous mass [8]. More advanced imaging studies, CT and MRI, are able to clearly show the cystic lymphangioma and its defining characteristics (water-density appearing septate cysts, either unilocular or multilocular) [1] [6] [7]. Lymphangiography was mentioned as a useful tool as well for some types of lymphangiomas [3]. Some authors have addressed the beneficiary role of a preoperative biopsy and subsequent microscopic examination, but because of the risk for dissemination of the tumor content, this procedure is often avoided [1] [2]. Still, a fine-needle aspiration cytology (FNAC) might be used [2].

• Mediastinal Mass

  Cystic lymphangiomas are benign tumors of the lymphatic system, and less than 1% present as a solitary mediastinal mass. To our knowledge, there has been no report in the literature describing Hodgkin lymphoma arising from cystic lymphangioma. [ncbi.nlm.nih.gov]

  CT Commonly seen as a hypoattenuating ill-defined neck cystic mass. [radiopaedia.org]

  It is a benign tumor, generally occurring as a mediastinal mass of slow growth. Most patients are asymptomatic, being incidentally diagnosed after a routine chest radiograph. The best treatment consists of complete surgical resection. [journalpulmonology.org]

  The CT images showed a hypodense mediastinal mass, 3 cm in diameter, in contact with the left upper dorsal tracheal wall; the lesion had regular borders and spontaneous low density with minimal enhancement after administration of contrast medium ( Figure [qims.amegroups.com]

These malformations are rarely seen in adults, and the literature provides poor guidelines for treatment options that must be carefully applied to the facial region. [ncbi.nlm.nih.gov]

Treatment of Lymphatic Malformations with the mTOR Inhibitor Sirolimus: A Systematic Review. Lymphat Res Biol. 2018 Aug. 16 (4):330-339. [Medline]. [emedicine.com]

Cystic Lymphangioma Prognosis The prognosis for fetuses that are diagnosed with cystic lymphangioma late in the pregnancy is generally good. [knowcancer.com]

Prognosis after surgical removal is excellent. [ncbi.nlm.nih.gov]

Most fetuses with cystic hygromas have a poor prognosis although it may improve in utero on its own in a tiny proportion of cases. Spontaneous remission does not necessarily exclude an abnormal karyotype. [radiopaedia.org]

Prognosis - Abdominal cystic lymphangioma Treatment - Abdominal cystic lymphangioma Eneucleation is a good treatment whenever possible. Abdominal cystic lymphangioma can also be treated through surgery. [checkorphan.org]

The prognosis is excellent with its complete removal, since it is a benign tumor. [dovemed.com]

The etiology and management of adult retroperitoneal cysts are reviewed as well. [ncbi.nlm.nih.gov]

In these cases, the etiology is related to inadequate drainage of the lymphatic vessel into the venous system. Nuchal lymphangiomas are often associated with chromosome abnormalities. [nature.com]

Lymphatic malformation with blockage of the lymphatic flow is the most common etiology leading to the formation of lymphangiomas. [pancreas.imedpub.com]

This article was designed to study the epidemiological, diagnostic difficulties, and therapeutic principles of intra-abdominal cystic lymphangioma in adults. [ncbi.nlm.nih.gov]

Epidemiology The incidence of LM is estimated to be one case per 6000-16,000 live births. Most LMs (50-65%) are evident at birth, and 80-90% of them present by age 2 years. [emedicine.com]

[…] parents should receive genetic counseling because this condition can recur in subsequent pregnancies. [4] Complications after surgical removal of cystic hygroma include damage to the structures in the neck, infection, and return of the cystic hygroma. [9] Epidemiology [en.wikipedia.org]

[…] common presentations Standardized, two-column rapid retrieval template For each syndrome, from Aarskog to Zellweger Syndrome, you'll find a succinct "snapshot" of clinical information that includes: "At a Glance" Description Incidence Genetic Inheritance Pathophysiology [books.google.com]

Lymphangioma circumscriptum has a high recurrence rate after excision because of its deep component (see Pathophysiology ). Cavernous lymphangioma are also uncommon and usually arise during infancy. [emedicine.medscape.com]

Pathophysiology LMs are thought to arise from a combination of the following: Failure of lymphatics to connect to the venous system Abnormal budding of lymphatic tissue Sequestered lymphatic rests that retain their embryonic growth potential These lymphatic [emedicine.com]

[…] are present at birth, and up to 90% are detectable by 2 years of age. 8 Although more than 90% of lymphatic malformations are clearly congenital, later presentations may occur as a result of trauma, infection, neoplasms, or iatrogenic injuries. 1 The pathophysiology [mdmag.com]

Complete excision of the cyst is mandatory to prevent recurrences. In our case, no recurrence was detected after a two-year follow-up. [ncbi.nlm.nih.gov]

Symptoms - Abdominal cystic lymphangioma Causes - Abdominal cystic lymphangioma Prevention - Abdominal cystic lymphangioma Not supplied. [checkorphan.org]

Abstract Copyright © 2016, Iranian Journal of Cancer Prevention. [ijcancerprevention.com]

[…] into mediastinum Large mediastinal lesions may compress lungs, heart and nerves, but most lesions are asymptomatic and found on x-ray Rarely occur in lymph nodes ( Am J Surg Pathol 1992;16:335 ) Treatment Complete surgical excision is recommended to prevent [pathologyoutlines.com]

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8. Correia FM, Seabra B, Rego A, Duarte R, Miranda J. Cystic lymphangioma of the mediastinum. J Bras Pneumol. 2008;34(11):982-984.