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Cystic Lymphangioma

Hygroma Cystic

Cystic lymphangioma is a benign and usually slow-growing tumor of the lymphatic system that is mainly diagnosed in the first few years of life. The head and neck area are the principal locations, but they may develop in virtually any part of the body. Symptoms depend on the site, but a slowly enlarging mass is frequently encountered. The diagnosis rests on imaging studies such as computed tomography (CT) and magnetic resonance imaging (MRI), whereas a biopsy may be necessary for confirmation.


Presentation

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].

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]
Chronic Infection
  • Acquired lymphangiomas, which are principally seen in middle-aged adults, are caused by a process of chronic lymphatic obstruction secondary to surgery, chronic infection, or radiation.[ncbi.nlm.nih.gov]
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]
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]
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]
Abdominal Mass
  • Lymphangioma was the final pathological diagnosis after laparotomy with complete resection of the cystic abdominal mass.[ncbi.nlm.nih.gov]
  • Omental cystic lymphangioma is an extremely rare abdominal mass caused by congenital malformation. An 8-year-old premenarchal girl reported abdominal pain.[ncbi.nlm.nih.gov]
  • Cystic lymphangiomas should always be included in the differential diagnosis of abdominal masses which present with mass effect signs and symptoms. Due to its rarity, it forms a diagnostic and therapeutic challenge for the clinician.[ncbi.nlm.nih.gov]
  • In this paper, we describe the advantages of MIS to treat newborn with abdominal masses.[ncbi.nlm.nih.gov]
  • Physical examination revealed an abdominal mass in 5 patients (55.5%). The CL was asymptomatic in four patients; the discovery of CL was performed preoperatively during an ultrasound for another pathology (n 3) or intraoperatively (n 1).[ncbi.nlm.nih.gov]
Abdominal Distension
  • 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]
  • In this paper, we describe 3 cases, that was admitted in our department, two with abdominal distension, pain, and an abdominopelvic mass; the other present an acute abdomen.[ncbi.nlm.nih.gov]
  • A 25-year-old female who presented with acute onset of abdominal distension and severe pelvic pain is presented.[ncbi.nlm.nih.gov]
  • We report on a 68-year-old woman who had experienced epigastric abdominal distension and nausea for over 1 year. Sonography revealed a cystic-solid mixed mass in the head of pancreas, with intrahepatic and extrahepatic duct dilatation.[ncbi.nlm.nih.gov]
  • This often leads to abdominal distension and discomfort. Lymphoedema It also known as lymphatic obstruction and causes localized fluid retention and acute swelling of the shoulders, neck, face, limbs, and throat.[hxbenefit.com]
Epigastric Pain
  • She was admitted to our clinic because of an enlargement of the cyst and epigastric pain after meals. She had a long history of heartburn after meals and had not undergone any treatment. She had no record of previous illnesses.[ncbi.nlm.nih.gov]
  • Case presentation A 38-year-old woman known for anxiety disorder developed constant epigastric pain without radiation toward the back.[wjso.biomedcentral.com]
Recurrent Abdominal Pain
  • A 35-year-old male patient was admitted to our hospital with recurrent abdominal pain and general weakness for 1 week.[ncbi.nlm.nih.gov]
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]
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]
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]
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]

Workup

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]
  • 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]
Pericardial Effusion
  • Echocardiography revealed massive pericardial effusion without tamponade.[ncbi.nlm.nih.gov]
Karyotype Abnormal
  • Etiology Karyotypic abnormalities are present in 25-70% of children with LM.[emedicine.com]
  • Greater volumes ( 75 mm 3 according to one study 9 ) are thought to correlate with increased karyotypic abnormality and more unfortunate fetal outcome 9-10. CT Commonly seen as a hypoattenuating ill-defined neck cystic mass.[radiopaedia.org]

Treatment

  • No recidive after 28 months in the average after treatment. CONCLUSIONS: The laparoscopic approach is the gold standard in the treatment of intraabdominal CL.We recommend complete surgical excision to avoid recurrence. Celsius.[ncbi.nlm.nih.gov]
  • We aim to present a rare case of small bowel symptomatic cystic lymphangioma in an adult patient, as well as its diagnosis and subsequent treatment.[ncbi.nlm.nih.gov]
  • She had a long history of heartburn after meals and had not undergone any treatment. She had no record of previous illnesses.[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]
  • 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]

Prognosis

  • Treatment is surgical and the prognosis is good.[ncbi.nlm.nih.gov]
  • 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]
  • 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]
  • 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]

Etiology

  • Although the etiology of omental lymphangioma remains largely unclear, these findings suggested strongly that obstruction of the lymphatic vessels after gastric resection for gastric carcinoma might be the most plausible cause.[ncbi.nlm.nih.gov]
  • Lymphatic malformation with blockage of the lymphatic flow is the most common etiology leading to the formation of lymphangiomas.[ncbi.nlm.nih.gov]
  • The etiology and management of adult retroperitoneal cysts are reviewed as well.[ncbi.nlm.nih.gov]
  • Descamps P, Jourdain O, Paillet C, Toutain A, Guichet A, Pourcelot D, Gold F, Castiel M, Body G: Etiology, prognosis and management of nuchal cystic hygroma: 25 new cases and literature review. Eur J Obstet Gynecol Reprod Biol 1997;71:3–10.[karger.com]

Epidemiology

  • 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]
Sex distribution
Age distribution

Pathophysiology

  • […] 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]
  • Chromosomal aneuploidy such as Turner syndrome or Down syndrome [5] were found in 40% of patients with cystic hygroma. [6] Pathophysiology [ edit ] In 1976, Whimster studied the pathogenesis of lymphangioma circumscriptum, finding lymphatic cisterns in[en.wikipedia.org]

Prevention

  • 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]
  • 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]
  • 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]
  • […] 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]

References

Article

  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.

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Last updated: 2019-07-11 21:17