[Skip to Content]
Access to paid content on this site is currently suspended due to excessive activity being detected from your IP address 54.161.128.52. Please contact the publisher to request reinstatement.
Sign In
Individual Sign In
Create an Account
Institutional Sign In
OpenAthens Shibboleth
[Skip to Content Landing]
Citations 0
Special Feature
April 01, 2005

Image of the Month—Diagnosis

Arch Surg. 2005;140(4):412. doi:10.1001/archsurg.140.4.412
Answer: Giant Multicystic Lymphangioma

Frozen section analysis of biopsy material could not clearly distinguish between lymphangioma, lymphangiosarcoma, teratoma, or the possibility of an inflammatory pseudotumor; a small blue-cell tumor, however, was excluded. Contents of the cysts appeared chylous. The final pathology analysis was consistent with a benign giant multicystic lymphangioma (or cystic hygroma). Mucin stains were negative; trichrome staining confirmed the presence of smooth muscle strands within the cyst walls.

Giant multicystic lymphangiomas are rare congenital malformations of the lymphatic system. These lesions result from a proliferation of dilated lymphatic spaces lined by thin endothelial layers.1 They are thought to develop when primary lymphatic sacs fail to join the lymphatic system during embryologic development.2,3 Lymphangiomas rarely present as intra-abdominal tumors; the majority of those that do are found in younger children. Lymphangiomas most commonly occur within the soft tissues of the head and neck, and they are also called cystic hygromas.4 Within the abdomen, the most common site is the small-bowel mesentery, followed by omentum, mesocolon, and retroperitoneum.5 Spontaneous regression of lymphangiomas appears to be rare, and the usual course is continued expansion. Rapid enlargement might also occur in association with infection, trauma, or erosion into a vessel and hemorrhage.2 Complete resection with negative microscopic margins might be impossible and might account for the overall 10% postoperative recurrence rate.6

Intestinal duplications are uncommon congenital abnormalities. Usually observed early in life, a small number of cases might remain undiagnosed until adulthood. Intestinal duplications share a common wall and blood supply with native bowel; some cysts carry ectopic gastric or pancreatic epithelium. The clinical presentation can vary depending on the location; possible complications include bleeding, abdominal pain, intussusception, or malignant degeneration. Computed tomography is useful in delineating surrounding structures. After the diagnosis is established, surgical correction is the treatment of choice, preferably with complete removal.7,8 A giant chylous duplication cyst has been described as well.9

Desmoplastic small round cell tumors are rare, highly aggressive malignant tumors found in adolescent males. The histologic appearance is that of a small blue-cell tumor; the cells carry a characteristic translocation, t(11;22)(p13;q12), resulting in a fusion between the Ewing sarcoma gene and the Wilms tumor gene. Most patients present with large masses in the abdomen or pelvis, which can be partly cystic. The overall survival rate is low, even after extensive resections; patients treated with aggressive chemotherapy followed by operative cytoreduction tend to have better outcomes.10 For this reason, the presence of a small blue-cell tumor should be excluded prior to committing to a resection of a large abdominal tumor in an adolescent.

Cysticercosis results from the larval infection with the pork tapeworm, Taenia solium. It is a potentially dangerous infection that usually manifests itself within the central nervous system or eye, but involvement of muscle or soft tissue, including extrahepatic intra-abdominal sites, have been reported.11,12 The multiloculated cysts might be calcified. Treatment with albendazole or praziquantel is preferred; operative therapy might be necessary to control specific, localized complications.

Box Section Ref ID

Submissions

The Editor welcomes contributions to the “Image of the Month.” Send manuscripts to Archives of Surgery, Johns Hopkins Medical Institutions, 720 Rutland Ave, Ross 759, Baltimore, MD 21205; (443) 287-0026; e-mail: archsurg@jama-archives.org. Articles and photographs accepted will bear the contributor’s name. Manuscript criteria and information are per the “Instructions for Authors” for Archives of Surgery. No abstract is needed, and the manuscript should be no more than 3 typewritten pages. There should be a brief introduction, 1 multiple-choice question with 4 possible answers, and the main text. No more than 2 photographs should be submitted. There is no charge for reproduction and printing of color illustrations.

Back to top
Article Information

Correspondence: Roderich E. Schwarz, MD, PhD, Cancer Institute of New Jersey, Department of Surgery, University of Medicine and Dentistry of New Jersey, Robert Wood Johnson Medical School, 195 Little Albany St, New Brunswick, NJ 08903-2681 (r.schwarz@umdnj.edu).

Accepted for Publication: June 15, 2003.

References
1.
Fenoglio-Preiser  CPascal  RPerzin  K Tumors of the intestines. Atlas of Tumor Pathology 3rd EditionVol 27 Washington, DC Armed Forces Institute of Pathology1990;494- 509
2.
Kennedy  TL Cystic hygroma-lymphangioma: a rare and still unclear entity. Laryngoscope 1989;991- 10
PubMedArticle
3.
 Cystic hygroma. Lancet 1990;335511- 512
PubMedArticle
4.
Enzinger  FWeiss  S Soft Tissue Tumors, 3rd Edition.  St Louis, Mo Mosby Yearbook1995;679- 689
5.
Pandit  SKRattan  KNBudhiraja  SSolanki  RS Cystic lymphangioma with special reference to rare sites. Indian J Pediatr 2000;67339- 341
PubMedArticle
6.
Scally  CMBlack  JH Cystic hygroma: massive recurrence in adult life. J Laryngol Otol 1990;104908- 910
PubMedArticle
7.
Holcomb  GW  IIIGheissari  AO'Neill  JA  JrShorter  NABishop  HC Surgical management of alimentary tract duplications. Ann Surg 1989;209167- 174
PubMedArticle
8.
Otter  MIMarks  CGCook  MG An unusual presentation of intestinal duplication with a literature review. Dig Dis Sci 1996;41627- 629
PubMedArticle
9.
Sebastian  JJFuentes  JBoldova  IGarcia  SCardiel  MJ Giant chylous cyst: an unusual presentation of intestinal duplication. Hepatogastroenterology 1996;43769- 770
PubMed
10.
Schwarz  REGerald  WLKushner  BHCoit  DGBrennan  MFLa Quaglia  MP Desmoplastic small round cell tumors: prognostic indicators and results of surgical management. Ann Surg Oncol 1998;5416- 422
PubMedArticle
11.
Garcia  HHDel Brutto  OH Taenia solium cysticercosis. Infect Dis Clin North Am 2000;1497- 119
PubMedArticle
12.
Luna  AWalling  AD Cysticercosis. Am Fam Physician 1988;37105- 108
PubMed
×