Giant retroperitoneal hemolymphangioma: case report and literature review

doi:10.21203/rs.3.rs-3816388/v1

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Case Report

Giant retroperitoneal hemolymphangioma: case report and literature review

https://doi.org/10.21203/rs.3.rs-3816388/v1

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Hemolymphangioma is a very rare benign tumor in clinical practice caused by abnormalities of the vasculature. Here, we report a patient with a giant retroperitoneal hemolymphangioma. A 37-year-old man was admitted to the hospital because of unprovoked upper abdominal distension and low back pain. Computed tomography (CT) and magnetic resonance imaging (MRI) suggested the possibility of a giant retroperitoneal lymphatic cyst. The patient underwent surgical treatment after adequate drainage. The postoperative pathological diagnosis was retroperitoneal hemolymphangioma. Its clinical features are often atypical, and it is easy to miss and misdiagnose. When the time of nuclear magnetic T1 is significantly reduced, the diagnosis of hemangiangiangioma should be considered. Therefore, we report this case in the hope of raising clinicians' awareness of the disease.

hemolymphangioma

retroperitoneal tumor

surgical excision

Hemolymphangioma is divided into primary and secondary. Primary hemolymphangioma is a congenital malformation of the vascular system, which may be related to obstruction of the venous lymphatic pathway between the abnormal vascular tissue and the systemic circulation. Secondary hemolymphangioma is usually caused by inadequate lymphatic drainage due to injury of lymphatic vessels caused by trauma or surgery[1]. It has been reported in pancreas, limbs, neonatal mediastinum, chest wall, anterior mediastinum, posterior mediastinum, pericardium, tongue, orbit, retroperitoneum, esophagus, cervix, adrenal gland, kidney, liver and spleen. However, there are few reports of retroperitoneal hemolymphangioma[1–5].In this article, we report a case of giant retroperitoneal hemolymphangioma, and discuss the clinical, imaging, and treatment options based on the published literature.

A 37-year-old male patient presented with upper abdominal distension and discomfort accompanied by low back pain 2 months prior without obvious inducement. He had no past history, family history or surgical history. He weighed 65 kg. On physical examination, the abdomen was distended, and a large abdominal mass was palpable. One month prior, the patient's symptoms worsened, and CT examination was performed at the local hospital, which was considered as a giant hepatic cyst. After enhanced CT and magnetic resonance imaging were performed in our hospital, the patient was considered as a left retroperitoneal cystic mass and a giant left perirenal lymphangioma. The lesion crawled along the left kidney and wrapped around the left kidney(Fig. 1).

In order to relieve compression symptoms and make a better diagnosis, we performed ultrasound-guided percutaneous nephrostomy, which drained a large amount of dark red fluid. Histopathological examination of the drainage fluid showed histiocytes and inflammatory cells under the microscope. On the fifth day after the operation, enhanced CT of the urinary system showed that the scope of the lesion was reduced, and the patient was discharged on the same day. Approximately 7000 ml of drainage fluid was drained by intermittent clamping after the operation. The patient underwent adequate drainage for two weeks before readmission, at which time she had lost approximately 25kg of body weight. During the operation, multiple cystic and solid masses on the surface of the kidney were found, and dark red blood fluid flowed out after the incision of the cyst wall. Multiple follicular masses and cord-like tissue were observed in the cyst. Part of the cord-like tissue was removed and subjected to pathology separately(Fig. 2). Pathology and further immunohistochemical analysis revealed hemolymphangioma, CD34 (vascular +), D2-40 (lymphatic +), Ki67 (2%+), CK (focal +)(Fig. 3). The patient was followed up for 3 months without recurrence.

Hemolymphangioma is a benign condition that may be a slowly growing cyst over a period of time or a tumor that grows aggressively but has no invasive capacity[6]. Therefore, hemangiolymangioma usually do not have typical clinical manifestations. In this case, the patient's symptoms of upper abdominal distension and low back pain may have been caused by compression from the large tumor. Preoperative diagnosis and evaluation by imaging are essential, and these lesions should be differentiated from ganglioneuroma, myelolipoma, epidermoid cyst, lymphangioma, and cystic teratoma. The typical appearance of hemolymphangioma is cystic or solid. The cystic part may be due to rupture and fusion of the vascular cavity, and the solid part may be residual compressed vascular tissue[7]. A significantly shortened T1-weighted MRI may indicate that the cystic mass contained old blood[8].

In this study, we used the PubMed database to review the literature up to December 2023, and there were only 3 reports of hemolymphangioma occurring in the retroperitoneal space. French scholars first reported a case of infectious retroperitoneal hemolymphangioma with venomusculoskeletal hypertrophy syndrome in 1983. We did not have the details of the case[9]. In 1987, Japanese researchers reported a case of giant retroperitoneal hemolymphangioma and discussed its appearance on MRI. In addition, factor VIII related antigen staining was also used to differentiate lymphatic vessels from blood vessels[8]. Another Japanese group reported a 68-year-old man with retroperitoneal hemolymphangioma in 2018[1].

As shown in Table 1, all four patients were male, three had large tumors, and the volume of tumors in our study was the largest. We speculate that because there are no special symptoms in the early stage of hemolymphangioma originating from the retroperitoneal space, patients will pay attention to this disease when the tumor volume increases which can lead to compression symptoms or signs of a large mass. Because hemolymphangioma is rare, various imaging methods are often needed for diagnosis and differential diagnosis before surgery, among which MRI is very helpful for determining the nature of the content of the mass. When the T1 time is significantly shortened, hemolymphangioma should be considered. Although hemolymphangioma is a benign tumor, surgical resection is recommended to avoid further aggressive tumor growth or recurrence, and surgical resection was performed for all 4 patients. The final diagnosis depends on the pathological results. Immunohistochemical staining for D2-40, CD31 and CD34 was often positive.

Table 1

Characteristics of four patients with retroperitoneal hemolymphangioma
	Sex	Age	Size	Preoperative diagnosis	Treatment	Prognosis
1	M	29year	22×9cm	none	tumor resection	none
2	M	17year	25×15×7cm	lymphangioma with bleeding	tumor resection	none
3	M	68year	40×32mm	liposarcoma or lymphoma	tumor resection	no recurrence
4	M	37year	20.0×16.3×31.3cm	lymphangioma	tumor resection	no recurrence

In summary, retroperitoneal hemolymphangioma is a rare lesion of blood vessels and lymphatic vessels. In the early stage, patients are often asymptomatic, and tumors are often found when they are large. It is difficult to differentiate it from lymphangioma via preoperative imaging. MRI is helpful for the diagnosis. Surgical treatment is the preferred treatment, and the long-term prognosis is good.

Consent for publication

Written informed consent has been obtained from the patient to publish this paper.

Ethics approval and consent to participate

Not applicable. Written informed consent has been obtained from the patient to publish this paper.

Competing interests

The authors declare no competing interests.

Funding

This research received no external funding.

Author Contribution

Conceptualization, W.S.; methodology, Y.W.; investigation, Y.W. and Y.L.; data curation and Y.L., Y.W.; writing, Y.W.; supervision, W.S.. All authors have read and agreed to the published version of the manuscript.

Acknowledgements

The authors thank doctors, nurses, and clinical staff who provided care for the patient.

Availability of data and materials

The data presented in this study are available on request from the corresponding author. The data are not publicly available due to consideration for the patients’privacy.

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Kanaitsuka T, Itani K, Shigeta H, Yamamura Y, Kogawa T, Yoshikawa T, Sugino S, Kanatsuna T, Kondou M, Takashina K: [A case report of giant retroperitoneal hemolymphangioma]. Nihon Naika Gakkai zasshi The Journal of the Japanese Society of Internal Medicine 1987, 76:1595-1603. https://doi.org/10.2169/naika.76.1595.
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No competing interests reported.

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Giant retroperitoneal hemolymphangioma: case report and literature review

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