Lymphangioma

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Classification according to ICD-10
D18.1- Lymphangioma

0 = hygroma colli cysticum
1 = axilla
2 = inguinal
3 = retroperitoneal
8 = other locations (including mesenteric) 9 = unspecified location

ICD-10 online (WHO version 2019)

A lymphangioma ( English Lymphangioma , German also Lymphgeschwulst ) is a rare benign tumor disease ( hamartoma ) of the lymph vessels . The counterpart to lymphangioma is a hemangioma in the blood vessels .

Systematics

Lymphangiomas are divided into three classes:

  • Cavernous lymphangiomas ( Lymphangioma cavernosum )
Mostly on the face, armpits or arms and legs. Often discolored dark by bleeding.
  • Capillary lymphangiomas ( lymphangioma circumscriptum )
Small vesicles in the skin and mucous membrane area of ​​the mouth and lips, as well as genital and inguinal. Since the bubbles burst very easily, they wet.
In one or more places on the neck, neck, armpits or middle skin . The vessels are often connected to one another.

A fourth type, lymphangiomatous gigantism , which is similar to elephantiasis , is also occasionally described.

The lymphangiomatosis must be differentiated from the differential diagnosis.

Etiology and pathogenesis

The exact cause of the development of a lymphangioma is still unclear. May play inherited malformations of the lymphatic system a role. In the second month of pregnancy, an incorrect differentiation occurs in the mesoderm of the affected patient . This is how the lymphangiomas arise from veno-lymphatic pouches. The jugular vein ( jugular vein), the mesenteric root in the lower abdomen and the two pelvic veins ( iliac veins) are affected . The lymphangiomas manifest themselves accordingly in these body regions. No local connection can be established between the lymph vessels and the venous system, so that the lymph cannot drain into the veins. This in turn leads to an enlargement of the lymph vessels, a lymphangiectasia . From a histopathological point of view, the tumor itself consists of a multitude of cyst-like structures that are filled with a protein-like eosinophilic fluid, separated by delicate septa and lined with endothelia .

A lymphangioma usually occurs in early childhood. In 90% of the cases in the period immediately after birth up to the age of five. At birth, the lymphangiomas are already present in 90% of cases, but are noticed in only 65%.

The tumor usually manifests itself on the neck or neck (75%) or the armpits (20%). Other areas are less affected, but still possible. For example, the mediastinum , the pleura , the pericardium , the vulva , the penis, the gall bladder , the groin region , the bones , the pancreas , the ovaries and the abdomen have been described .

diagnosis

The imaging method of choice is sonography , in the case of lymphangiomas inside the body and especially for surgical planning, magnetic resonance imaging (MRI).

therapy

While hemangiomas often resolve spontaneously, this is not the case with lymphangiomas. The extirpation of the lymphangioma, i.e. the complete removal of the tumor, is the most common treatment method. If the removal is incomplete, a relapse always occurs . Only complete removal allows healing .

The laser therapy is often combined as a therapeutic alternative to surgery, applied. Several treatments are usually necessary. But the procedure is less stressful for the patient and leaves smaller scars .

With the exception of sclerotherapy with picibanil (OK-432), drug therapies are largely unsuccessful. Picibanil consists of a lyophilized and benzylpenicillin- treated special strain (Su) of Streptococcus pyogenes . Picibanil is injected multiple times into the lymphangioma. This treatment is an alternative to surgical extirpation when complete removal of the lymphangioma is associated with high morbidity .

forecast

The lymphangioma is a benign tumor. Complete removal ensures healing.

further reading

  • NR Tulasi et al: Lymphangioma circumscriptum. In: Int J Gynecol Cancer 14, 2004, pp. 564-566. PMID 15228436 (Review)
  • JE Losanoff et al: Mesenteric cystic lymphangioma. In: J Am Coll Surg 196, 2003, pp. 598-603. PMID 12691938 (Review)
  • HA Tanriverdi et al: Hygroma colli cysticum: prenatal diagnosis and prognosis. In: Am J Perinatol 18, 2001, pp. 15-20. PMID 11733855

Individual evidence

  1. ↑ a b c d R. Berchtold and M. Bartels: Berchtold surgery with student: Studentconsult. HP Bruch and O. Trentz (editors), Elsevier, Urban & Fischer Verlag, 2008, ISBN 3-437-44481-6 , pp. 1206-1207.
  2. ^ A b G. Gray et al: Cystic lymphangioma of the pancreas: CT and pathologic findings. In: Abdom Imaging 23, 1998, pp. 78-80. PMID 9437068
  3. ↑ P. Schnatterbeck and K. Drews: Cystic Lymphangiom. In: RöFa 12, 1999
  4. ↑ a b M. Khandelwal et al .: Abdominal lymphangioma masquerading as a pancreatic cystic neoplasm. In: J Clin Gastroenterol 20, 1995, pp. 142-144. PMID 7769196
  5. ↑ H. Ezold et al.: Cavernous lymphangiomas. In: Akt Dermatol 29, 2003, pp. 284-288. doi : 10.1055 / s-2003-41293
  6. ^ AT Vlastos et al.: Lymphangioma circumscriptum of the vulva: a review of the literature. In: Obstet Gynecol 101, 2003, pp. 946-954. PMID 12738156
  7. ↑ S. Gupta et al .: Lymphangioma circumscriptum of the penis mimicking venereal lesions. In: J Eur Acad Dermatol Venereol 17, 2003, pp. 598-600. PMID 12941108 (Review)
  8. ↑ JK Kim et al .: Gallbladder lymphangioma: a case report and review of the literature. In: World J Gastroenterol 13, 2007, pp. 320-323. PMID 17226918 (Review)
  9. ↑ D. Jain et al.: Lymphangioma of the ovary. In: J Obstet Gynaecol 29, 2009, pp. 260-261. PMID 19358048 (Review)
  10. ↑ CM Kullendorff et al: Cystic abdominal lymphangioma in children. Case report. In: Eur J Surg 159, 1993, pp. 499-501. PMID 8274560
  11. ↑ RB Colovic, NM Grubor, MT Micev, HD Atkinson, VI Rankovic, MM Jagodic: Cystic lymphangioma of the pancreas. In: World journal of gastroenterology. Volume 14, Number 44, November 2008, pp. 6873-6875, PMID 19058318 , PMC 2773887 (free full text) (review).
  12. ↑ K. Reinshagen and others: Treatment of congenital lymphangiomas with Picibanil. In: 125th Congress of the German Society for Surgery April 16, 2008
  13. ↑ M. Bloching et al.: Sclerotherapy of cystic masses in the neck area with OK-432. In: HNO 53, 2005, pp. 238-242. doi : 10.1007 / s00106-004-1102-4
  14. ^ R. Rautio et al .: Treatment of Lymphangiomas with OK-432 (Picibanil). In: Cardiovasc Intervent Radiol 26, 2003, pp. 31-36. doi : 10.1007 / s00270-002-1980-3
  15. ↑ Follow-up: Sclerotherapy of cystic masses in the neck area with picibanil (OK-432). In: 78th Annual Meeting of the German Society of Oto-Rhino-Laryngology, Head and Neck Surgery April 24, 2007

Web links

Wiktionary: Lymphangioma  - explanations of meanings, word origins, synonyms, translations