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].

• Mediastinal 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]

• Localized Edema

  A lethal version of this condition is known as Cowchock Wapner Kurtz syndrome that, in addition to cystic hygroma, includes cleft palate and lymphedema, a condition of localized edema and tissue swelling caused by a compromised lymphatic system. [en.wikipedia.org]

• Mediastinal Disease

  Mediastinal disease is a frequent clinical presentation in children with Hodgkin lymphoma. It is usually due to mediastinal lymphadenopathy or involvement of the thymus gland. [ncbi.nlm.nih.gov]

• Persistent Cough

  A 2-month-old boy was referred to the hospital after developing a persistent cough. Chest X-ray showed a large cyst in the right lung. Under the preoperative diagnosis of bronchogenic cyst, he underwent right lower lobectomy at the age of 11 months. [ncbi.nlm.nih.gov]

• Pelvic Mass

  Physical examination showed an abdomino-pelvic mass. Preoperative studies including abdominal ultrasonography and computed tomography failed to determine the cause of the pain. Laparotomy found a giant cystic tumor of the small bowel mesentery. [ncbi.nlm.nih.gov]

  masses may have similar imaging appearances, and radiologic evaluation may be of limited diagnostic use. [indianjcancer.com]

  Multilocular cystadenoma of the prostate presenting as a giant pelvic mass. Br J Radiol. 2009;82:e200–e201 10. Smithers CJ, Fishman SJHolcomb GW III, Murphy P. Vascular anomalies. Ashcraft's Pediatric Surgery. 5th ed. [journals.lww.com]

• Bilious 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]

• Delayed Gastric Emptying

  The postoperative course was uneventful with slight delayed gastric emptying due to partial denervation of the lesser curvature of the stomach. The patient was discharged on eighth day after surgery without any postoperative complications. [ncbi.nlm.nih.gov]

• Cardiomegaly

  We report a case of asymptomatic chylopericardium in a young male who presented with cardiomegaly. Echocardiography revealed massive pericardial effusion without tamponade. [ncbi.nlm.nih.gov]

• Large Breast

  In reduction mammaplasty patients, plastic surgeons should consider cystic lymphangioma as a differential diagnosis of unilateral large breasts. [ncbi.nlm.nih.gov]

• 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]

  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]

  Review Article Introduction Neck masses are frequently encountered in pediatric medicine, and can present a diagnostic dilemma for the clinicians involved ( 1, 2 ). [doi.org]

• Neck Swelling

  They are an unusual cause of neck swelling but awareness of the lesions is necessary as they can be confused with thyroglossal duct cysts since they too are conventionally located adjacent to the hyoid bone in the midline. [doi.org]

• Drop Attacks

  This is the first reported case of late diagnosis of a cervical cystic lymphangioma revealed by recurrent drops attack. [ncbi.nlm.nih.gov]

• Lethargy

  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]

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]

  masses cystic hygroma vs occipital meningocoele Promoted articles (advertising) [radiopaedia.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]

  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]

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]

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

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

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 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]

Although the ovaries and uterus appeared to be distinct from this mass, a gyneocologic etiology could not be excluded. [pagepress.org]

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]

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]

A thin needle may be used to reduce the volume of the cystic hygroma to prevent facial deformities and airway obstruction. Close observation of the baby by a neonatologist after birth is recommended. [en.wikipedia.org]

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

[…] 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]

The entire growth must be removed to prevent it from coming back. In some cases, however, your doctor may not want to remove large cystic hygromas. [healthline.com]

1. Bhavsar T, Saeed-Vafa D, Harbison S, Inniss S. Retroperitoneal cystic lymphangioma in an adult: A case report and review of the literature. World J Gastrointest Pathophysiol. 2010;1(5):171-176.
2. Shahi M, Bagga PK, Mahajan NC. Cervical cystic lymphangioma in an adult, diagnosed on FNAC. J Cytol. 2009;26(4):164-165.
3. Yildirim E, Dural K, Kaplan T, Sakinci U. Cystic lymphangioma: Report of two atypical cases. Interact Cardiovasc Thorac Surg. 2004;3:63–65.
4. Chen CW, Hsu SD, Lin CH, Cheng MF, Yu JC. Cystic lymphangioma of the jejunal mesentery in an adult: A case report. World J Gastroenterol. 2005;11(32):5084-5086.
5. Joshi PS, Hongal B, Sanadi A. Cystic lymphangioma: A differential diagnosis. J Oral Maxillofac Pathol. 2015;19(3):393-395.
6. Pia F, Aluffi P, Olina M. Cystic lymphangioma in the head and neck region. Acta Otorhinolaryngol Ital. 1999;19:87–90.
7. Levy AD, Cantisani V, Miettinen M. Abdominal lymphangiomas: imaging features with pathologic correlation. AJR Am J Roentgenol. 2004;182 (6): 1485-91.
8. Correia FM, Seabra B, Rego A, Duarte R, Miranda J. Cystic lymphangioma of the mediastinum. J Bras Pneumol. 2008;34(11):982-984.