Nigerian Journal of Clinical Practice

CASE REPORT
Year
: 2019  |  Volume : 22  |  Issue : 12  |  Page : 1778--1780

Generalized cystic lymphangiomatosis incidentally recognized in an asymptomatic adult: Peroperative, CT, MRI, and histopathological findings of a very rare case


KN Arda1, S Akay1, KT Kizilkanat2,  
1 Department of Radiology, University of Health Sciences, Gulhane Training and Research Hospital, Ankara, Turkey
2 Department of Gastrointestinal Surgery, ZTB Women's Health Research and Education Hospital, Ankara, Turkey

Correspondence Address:
Dr. S Akay
Department of Radiology, University of Health Sciences, Gulhane Training and Research Hospital, Etlik - 06018, Ankara
Turkey

Abstract

Generalized cystic lymphangiomatosis is an uncommon congenital lymphatic malformation that may affect soft and hard tissues as well as organs. It is rarely seen in adult patients. Osseous lesions appear as well-defined cystic lesions with a sclerotic rim without periosteal reaction or a soft tissue component. A nonenhancing lesion in fluid density appears to be one of the most common characteristics of abdominal diseases, and clinical features are directly related to the extent of the disease. In this report, we present peroperative, computed tomography (CT), magnetic resonance imaging (MRI), and histopathological findings of a very rare case of generalized cystic lymphangiomatosis recognized in adulthood.



How to cite this article:
Arda K N, Akay S, Kizilkanat K. Generalized cystic lymphangiomatosis incidentally recognized in an asymptomatic adult: Peroperative, CT, MRI, and histopathological findings of a very rare case.Niger J Clin Pract 2019;22:1778-1780


How to cite this URL:
Arda K N, Akay S, Kizilkanat K. Generalized cystic lymphangiomatosis incidentally recognized in an asymptomatic adult: Peroperative, CT, MRI, and histopathological findings of a very rare case. Niger J Clin Pract [serial online] 2019 [cited 2022 Sep 29 ];22:1778-1780
Available from: https://www.njcponline.com/text.asp?2019/22/12/1778/272211


Full Text



 Introduction



Generalized cystic lymphangiomatosis (GLA) is an uncommon congenital disorder of the lymphatic ducts that may involve soft tissues, bone structures, and various organs in the body.[1] Well-defined cystic lesions with a sclerotic rim without periosteal reaction or associated soft tissue mass are the most common presentation of osseous involvement.[1] The condition may involve mediastinum, lungs, liver, and spleen. Abdominal lymphangiomatosis is very rarely seen in adult patients.[1] Clinical features are directly related to the severity and the number of sites affected by the disease.[2]

In the current report, we present an extremely rare case of GLA in the abdomen recognized incidentally during a cesarean section and its peroperative, computed tomography (CT), and magnetic resonance imaging (MRI) results as well as histopathological findings.

 Case Report



A 29-year old pregnant female (gravida 2, para 1) was admitted to the obstetrics and gynecology department of a different hospital at term. The patient was accepted for a cesarean section because she had an uncomplicated cesarean section for her birth three years before. Before surgery, laboratory results were within normal limits. During the operation, white opaque liquid was seen at the incision and in the abdomen, and there were multiple white, cystic structures on the intestine walls [Figure 1]. A gastrointestinal surgeon was asked to join the operation.{Figure 1}

Frozen section biopsy revealed that the structures were all benign. A surgical drain was placed and the operation was terminated upon successful delivery of the baby. Lymphangiectasis was reported in histopathology results, and the patient was referred to our hospital for further evaluation.

Radiologic examinations were performed for further assessment. CT results revealed an enlarged spleen containing multiple well-circumscribed cystic lesions, which usually show no contrast enhancement. Perihepatic, paraaortic, and pelvic free fluid were seen. Multiple thin-walled cystic lesions were detected in the right iliac bone, sacrum, one of the ribs in the left cage, the T12 vertebral body, and some lumbar vertebrae. Extensive free fluid collections were observed in the perihepatic region and pelvic area [Figure 2]. Upon enhanced MRI examination, cystic lesions similar to those seen on CT were seen at thoracic and lumbar vertebral bodies and on the spleen. All of the lesions were hypointense on T1-weighted images and hyperintense on T2-weighted images and showed no enhancement after contrast administration [Figure 3].{Figure 2}{Figure 3}

Cytologic examination revealed many mature lymphocytes, a few histiocytes, eosinophils, and two centrally localized mesothelial cells in the cystic fluid. The findings were consistent with indicators for lymphangioma [Figure 4].{Figure 4}

Based on these findings, the patient was diagnosed with GLA both clinically and histopathologically and was referred to the oncology department of our hospital for further treatment.

As previously mentioned, the patient remained asymptomatic until adulthood, and no complication or white opaque liquid leakage from the incision site was observed during the cesarean section. We attributed this to the fact that there had not been much free fluid in the pelvic area at that time and it may have increased in the intervening three years.

 Discussion



Lymphangiomas are cystic malformations that constitute one of the rarest benign pathologies of the lymphatic system. This condition is commonly seen in children and adults are rarely affected.[3] When this malformation is identified in multiple locations and organs, it is considered “generalized lymphangiomatosis.”

The diffuse or multicentric proliferation of dilated lymphatic vessels resembling common lymphatic malformations is the main characteristic of GLA. Complex lymphatic anomaly is another term that has recently been suggested for intractable lymphatic disorders such as GLA and Gorham–Stout disease (GSD).[4] In 1955, Gorham and Stout[5] reported the main pathological characteristics of this disease. While GLA presents with lytic areas confined to the medullary cavity of the bones, GSD patients have cortical bone loss due to progressive osteolysis.[6].

Pelvic bones, femur, rib, vertebra, and skull are the most affected bones in GLA.[3],[6] Although osseous involvement occurs mostly as lytic lesions, a rare sclerotic variant has also been described.[7] Typical bone involvement is in the form of well-defined, round lytic lesions with sclerotic margins without periosteal reaction or a soft tissue component.[8]

GLA commonly presents at birth or in childhood. This condition has a wide range of presentations and may affect various sites, including bone, liver, spleen, mediastinum, lungs, and soft tissues. The number of affected sites and severity determines the disease's clinical course.[2]

Abdominal pain, gastrointestinal bleeding, and protein-losing enteropathy are typical clinical features of abdominal lymphangiomatosis, but it can be asymptomatic.[1] Cystic abdominal lesions such as pancreatic pseudocyst, abdominal tuberculosis, hydatid disease, or cystic malignancies are the main differential diagnoses of the lymphatic system.[9]

The current imaging modalities for evaluating lymphangiomatosis are CT and MRI. Lymphangiomas are seen as nonenhancing lesions in fluid density with thin, indistinct walls and septa on CT.[10] Typical lesions appear as well-defined, nonenhancing cystic structures.

MRI is widely used as a tool for the diagnosis of GLA and possesses several advantages. It offers a better characterization of lesion morphology and is able to specify lesion origin.[9] Existing studies show that lytic lesions of lymphangiomatosis typically appear hypointense on T1-weighted magnetic resonance images and hyperintense on T2-weighted images, while the rare sclerotic variant is seen as hypointense on both T1- and T2-weighted images.[7]

In conclusion, GLA is a disease that should not be disregarded in any pathology findings characterized by multiple cystic lesions involving bones and/or abdomen. Although it is generally seen in children, it can also be diagnosed in adult patients. Familiarity with the CT and MRI characteristics of these lesions enables more accurate diagnosis and can provide valuable guidance for the treatment plan.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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