Lymphangioma of the pancreas: A case report

Abstract

Introduction

Lymphangiomas constitute a distinct subtype of benign lymphatic malformation. The occurrence of pancreatic involvement is exceedingly infrequent. Patients with this disease typically remain asymptomatic. Surgical resection serves as the primary therapeutic modality.

Case presentation

A female patient, aged 42, was revealed to have a mass located at the back of the pancreas and occupying the body of the pancreas during imaging examinations. As a result, she underwent laparoscopic surgery for resection.

Clinical discussion

Lymphangioma is a congenital aberration of the lymphatic vessels without malignant properties. Patients typically present without symptoms, and laparoscopic surgery is the primary treatment approach. We have focused our discussion on the etiology, diagnosis, and management of this condition.

Conclusion

Pancreatic lymphangioma, a clinical entity of rarity, often manifests with nonspecific symptoms. We believe that laparoscopic surgery is the preferred option for treating such diseases.

Highlights

• •Lymphangioma of the pancreas is an extremely rare case.
• •The complete clarification of pancreatic lymphangiomas using conventional imaging remains inconclusive.
• •Laparoscopic surgery is the preferred treatment option for this disease.

1. Introduction

Lymphangiomas constitute a distinct subtype of benign lymphatic malformation. The majority of lymphangiomas typically manifest in the cervical and axillary regions. The occurrence of pancreatic involvement is exceedingly infrequent. Pancreatic lymphangiomas are primarily induced by growth and developmental anomalies, alongside other iatrogenic factors. However, patients with this disease typically remain asymptomatic. This article presents a case inquiry of a 42-year-old female patient with pancreatic lymphangiomas following the identification of retroperitoneal space during imaging examinations.

The work has been reported in line with the SCARE 2023 criteria [1].

2. Case presentation

The 42-year-old female patient was admitted to the hospital on August 11, 2023, because of an enlarged mass in the pancreas on a review abdominal computed tomography (CT) scan with no adverse complaints. The patient had a previous history of diabetes mellitus and no other underlying conditions. The patient was diagnosed with right breast cancer one year ago and underwent a modified radical mastectomy. Since she had no relevant symptoms at that time, after our consultation, we suggested that she should recover for a while after she was discharged from the hospital after being cured of her breast cancer and then review the CT. We advised the patient to undergo surgery at that time, given the enlargement of the mass and the risk of possible cancer transformation. The laboratory tests and tumor markers of this patient were normal, and the abdominal CT showed an irregular cystic shadow with a low density in the hepatoportal region and the posterior aspect of the pancreatic head, measuring 8.6*6.7 cm (Fig. 1). The compression displaced the surrounding structures, and no enhancement was observed. Surgical resection was recommended because the retroperitoneal mass may be large. The patient agreed and accepted the surgical regimen. On August 17, 2023, laparoscopic resection was performed on the pancreatic mass. The intraoperative laparoscopic exploration revealed that the milky mass originated from the pancreas and protruded into the stomach and duodenum (Fig. 2). Its structure can be fully exposed. Because of the thin wall of the cyst, electrical damage caused a small rupture of the cyst when stripping the surrounding tissue. Relevant blood vessels and lymphatic vessels were ligated after aspiration, and the mass was resected entirely; we did a cystectomy, preserving the structure of the pancreas, and sent for a pathological examination. Postoperative symptomatic supportive treatment was performed, and the patient recovered well. Drainage fluid in the abdominal drain is clear and less than 10 ml. The pathological examination results revealed lymphangioma in the body of the pancreas with cyst development (Fig. 3). The patient was discharged from the hospital on the seventh day after surgery and came to the outpatient clinic for a follow-up three months later with no recurrence and no discomfort.

Fig. 1.

The preoperative abdominal CT scan identifies a mass located in the body of the pancreas (green arrow), with dimensions roughly estimated to be 8.6 ∗ 6.7 cm The pancreas is indicated by the yellow arrow.

Fig. 2.

Intraoperatively, the laparoscopic view reveals the horizontal part of the duodenum (white arrow) and a milky-white pancreatic lymphangioma (yellow arrow).

Fig. 3.

Microscopic examination reveals the lesion to be composed of spaces of varying sizes. The inner wall of these spaces is lined with a single layer of flat endothelial cells (The area indicated by white arrows). The spaces are filled with lymphatic fluid, within which lymphocytes are discernible.

3. Discussion

Lymphangioma is a congenital aberration of the lymphatic vessels without malignant properties. It is more accurately characterized by a developmental anomaly rather than a genuine tumor [2]. However, it remains unclear about the precise pathogenic mechanism of this disease. The isolation of lymphoid tissue during embryonic development in humans, together with its anomalous relationship to the venous plexus, may be the pathogenic cause of this disease. These abnormalities subsequently result in malformations of lymphatic vessels [3,4] and obstruction of lymphatic vessels due to exogenous abdominal trauma, surgery, radiation, inflammation, infection, and other factors [3,5]. Most lymphangiomas occur in the neck and axillary regions, whereas a mere proportion, approximately 1 %, exists in the mesenteric or retroperitoneal region [2,6,7]. Lymphangiomas can be histologically classified into three distinct subtypes, namely capillary, cavernous, and cystic subtypes, with the cystic subtype being the prevailing variant. Cystic lymphangiomas are highly prevalent [4,8]. Cystic lymphangiomas have smooth muscle and lymphatic tissues within the wall of the capsule and a lymphocyte group in the tubular cavity [4]. Immunohistochemistry indicates that the endothelial cells of cystic lymphangiomas express factor VIII-associated antigens CD31 and CD34, which are markers of the capillary and lymphatic endothelium [9]. Among them, pancreatic lymphangioma is an exceedingly uncommon variant, constituting roughly 1 % of retroperitoneal lymphangiomas and less than 0.5 % of pancreatic lesions [4,10]. Based on a literature review in this study, it was demonstrated that the initial instance of pancreatic lymphangioma was reported by Koch in 1913 [11]. Since then, pancreatic lymphangiomas have been reported in all age groups, and the prevalence of them is higher in females than in males [12]. Besides, most pancreatic lymphangiomas occur in the body of the pancreas rather than in the head of the pancreas [6,9].

Pancreatic lymphangiomas often manifest as a benign nature and exhibit a gradual growth trend. Patients commonly present with no distinctive symptoms and aberrant laboratory results [10,12]. In some patients, however, clinical manifestations may be detected due to a mass effect, and the size of the lesion is usually related to its location and occupying effect [12]. The size of the lesion and its location, as well as the occupying effect, usually determine the clinical presentation. Further, the lesion can compress other organs, resulting in various clinical manifestations. Common symptoms include stomach pain, abdominal fullness, and epigastric discomfort. However, it is worth noting that in exceptional circumstances, acute abdominal symptoms may arise due to such complications as lymphangioma rupture, hemorrhage, or infections [13]. In this study, the patient with pancreatic lymphangioma was admitted to the hospital and underwent relevant laboratory tests and imaging examinations. Combined with auxiliary examination methods, an appropriate treatment regimen was formulated for the patient, and laparoscopic surgery was successfully performed. The patient did not suffer from any obvious complications after surgery. After the diagnosis and treatment and relevant information were reviewed, no evident information can be observed for the diagnosis of pancreatic lymphangiomas. Lymphangioma can be detected in routine abdominal ultrasound, CT, and magnetic resonance imaging (MRI), but it cannot be completely differentiated from retroperitoneal masses from other sources with different natures [14].

Typically, the abdominal ultrasound can be selected as the initial imaging examination for patients. This examination can commonly reveal a multilocular lesion characterized by a homogeneous homogenate component and absence of notable echogenicity. Ultrasound imaging may exhibit echoes in the presence of potential secondary infection [15], the CT scan may reveal the presence of a discernible cystic mass exhibiting fluid attenuation, without any notable enhancement or calcified shadowing of the lesion [4]. Therefore, a CT scan is considered essential and optimal for the accurate identification of the location, dimension, and composition of lymphangiomas [14]. It has also been revealed that MRI is superior to CT in identifying whether a cystic mass is connected to the pancreatic duct or not [6]. In addition, Kawaguchi et al. concluded that ultrasound endoscopy-guided fine-needle aspiration was also valuable in the evaluation of pancreatic cystic lesions [2]. Although endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) is a feasible method in the diagnosis of lymphangiomas, it is not recommended for the diagnosis of recurrent lymphangiomas after puncture and drainage, and EUS-FNA may lead to such symptoms as bleeding and infection [2]. In the lack of pertinent test results, pancreatic lymphangiomas should be treated cautiously and distinguished from intraductal papillary mucinous neoplasm (IPMN), mucinous cystic neoplasm (MCN), and other conditions.

The complete clarification of pancreatic lymphangiomas using conventional imaging remains inconclusive. However, for such conditions, including MCN or IPMN, it is advisable to routinely conduct surgical interventions, contingent upon the patient's informed consent [14], and identify the status of the lymphangioma. Based on that, the therapeutic regimen to perform a complete resection of the mass or a partial resection of the associated pancreas can be formulated [16]. Meanwhile, it is required to ligate the main trunk and blood-supplying lymphatic vessels to avoid the development of coeliac ascites [17]. From the review in this study, laparoscopic resection emerges as the preferred treatment option [10,13,15]. Laparoscopic surgery has more obvious advantages than open surgery in terms of postoperative complications and prognosis. After the lymphangioma is removed, there is still a low probability of recurrence [13]. Throughout the treatment process, attention should be paid to the subsequent problems after treatment, so as to achieve a full recovery in the clinic.

4. Conclusion

Pancreatic lymphangioma is a rare clinical disease. Lymphangiomas are mostly benign and slow-growing, and their symptoms are nonspecific. Hence, the diagnosis of this disease should be made in combination with relevant imaging tests and histologic evaluation of lesions. We believe that laparoscopic surgery is the preferred option for treating such diseases, for which we look forward to further studies.

Consent

We obtained written informed consent from the patient for this publication. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Ethical approval

Ethical approval is not a requirement at our institution for reporting individual cases or case series.

Funding

No funding was obtained for this study.

Author contribution

Ting Jiang contributed to the initial draft, data organization, and image editing.

Xinglin Jin and Guangyi Li participated in manuscript review, subsequent article modifications, and supervision.

Guarantor

Ting Jiang.

Research registration number

This case report is not a first-in-man case.

Declaration of competing interest

All authors have no conflicts of interest to declare.